serial-number
stringlengths
6
9
pmcid
stringclasses
138 values
text
stringclasses
138 values
context
stringclasses
138 values
role
stringclasses
18 values
role-name
stringclasses
18 values
raw-initial-ground-truth
listlengths
0
18
test-1501
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
Endocrinology
ENDO
[ ". Her menstrual cycle was regular and she was menstruating one day after admission." ]
test-1502
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
Genitourinary-System
GU
[]
test-1503
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
Respiratory-System
RESP
[]
test-1504
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
Musculoskeletal-System
MSK
[]
test-1505
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
Eyes-Ears-Nose-Throat
EENT
[]
test-1506
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
Dermatology
DERM
[]
test-1507
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
Pregnancy
Pregnancy
[]
test-1508
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
Lymphatic-System
LYMPH
[]
test-1509
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
Age-at-Presentation
Age (at case presentation)
[ "a 24 year - old", "24 year - old-" ]
test-1510
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
Age-of-Onset
Age (of onset)
[]
test-1511
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
Confirmed-Diagnosis-IEM
Confirmed_Diagnosis(IEM)
[ "acute intermittent porphyria", "The diagnosis of AIP was based on the presence of high PBG in the urine ( test was done at another center ) ." ]
test-1512
5633257
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
{'Case presentation': 'We present a 24 year-old woman who developed acute intermittent porphyria five days after right hemi-colectomy. Her presentation included neuro-visceral and psychiatric manifestations, and severe hyponatremia. She received critical care symptomatic management including mechanical ventilation. The...
IEM-Treatment
IEM_Treatment
[ "high carbohydrate intake .", "regime of IV hematin ( heme arginate ) 4 mg / Kg body weight / day for three days .", "prophylactic IV hematin 4 mg / Kg body weight / day for three days" ]
test-1513
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Vitals-and-Hematology
Vitals_Hema
[ "polycythemia ( hemoglobin : 18.0 g / dL; red blood count : 627 × 10 4; hematocrit : 53.1 %", "height : 165 cm; weight : 79 kg; Body Mass Index : 29.0 kg / m²", "polycythemia also improved to a Hb level of 15.2 g / dL.", "height : 1.58 m; weight : 65 kg; body mass index : 23.9 kg / m²" ]
test-1514
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Gastrointestinal-System
GI
[]
test-1515
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Patient-History
History
[ "A 41 year old man was followed at the local clinic for polycythemia ( hemoglobin : 18.0 g / dL; red blood count : 627 × 10 4; hematocrit : 53.1 % ) and hypertension", "A 41 year old man was referred to Kobe City Medical Center West Hospital, Kobe City, Japan, because of 2 years of infertility", "female offspri...
test-1516
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Neurology
Neuro
[]
test-1517
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Laboratory-and-Imaging
Lab_Image
[ "polycythemia ( hemoglobin : 18.0 g / dL; red blood count : 627 × 10 4; hematocrit : 53.1 % )", "An abdominal CT scan for the examination of these conditions revealed the enlargement of the bilateral adrenal glands", "The laboratory tests revealed an extremely high level of ACTH ( 316.4 pg / mL ) and a relativ...
test-1518
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Cardiovascular-System
CVS
[ "hypertension" ]
test-1519
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Endocrinology
ENDO
[]
test-1520
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Genitourinary-System
GU
[ "Bilateral testicular tumors were identified in a scrotal ultrasound echo scan", "both testes had become slightly atrophied ( right : 14 mL; left : 12 mL ) ( performed with a Prader orchidmeter ) and no induration was palpated", "serum markers for testicular tumors were within the normal range and the testicula...
test-1521
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Respiratory-System
RESP
[]
test-1522
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Musculoskeletal-System
MSK
[]
test-1523
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Eyes-Ears-Nose-Throat
EENT
[]
test-1524
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Dermatology
DERM
[]
test-1525
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Pregnancy
Pregnancy
[]
test-1526
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Lymphatic-System
LYMPH
[]
test-1527
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Age-at-Presentation
Age (at case presentation)
[ "41 year old" ]
test-1528
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Age-of-Onset
Age (of onset)
[]
test-1529
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
Confirmed-Diagnosis-IEM
Confirmed_Diagnosis(IEM)
[ "Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility , accompanied by congenital adrenal hyperplasia ( CAH )" ]
test-1530
5768970
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
{'Cases': 'Testicular adrenal rest tumor ( TART ) is one of the possible causes of male infertility, accompanied by congenital adrenal hyperplasia ( CAH ). Here are reported two cases of TART s that were referred to Kobe City Medical Center West Hospital for the treatment of infertility and testicular tumors.', 'Case 2...
IEM-Treatment
IEM_Treatment
[ "After 6 months of low‐dose , daily oral glucocorticoid therapy ( 0.5 mg / d ) , the serum FSH , LH , testosterone , and estradiol levels returned to normal levels ( 9.2 IU / mL , 1.8 IU / mL , 0.8 ng / mL , and < 10 pg / mL , respectively ) . The polycythemia also improved to a Hb level of 15.2 g / dL.", "a low‐...
test-1531
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Vitals-and-Hematology
Vitals_Hema
[]
test-1532
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Gastrointestinal-System
GI
[]
test-1533
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Patient-History
History
[ "In 1989, a 21 - year - old male experienced visual loss after diving from a diving board", "Twenty years later, in April 2009, he presented again because of recent visual loss in the left eye" ]
test-1534
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Neurology
Neuro
[]
test-1535
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Laboratory-and-Imaging
Lab_Image
[ "Three biopsies from the flexural skin of the axilla and the neck did not reveal PXE related changes", "Analysis showed that our patient was homozygous for the R1141X - mutation in the ABCC6 - gene" ]
test-1536
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Cardiovascular-System
CVS
[]
test-1537
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Endocrinology
ENDO
[]
test-1538
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Genitourinary-System
GU
[]
test-1539
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Respiratory-System
RESP
[]
test-1540
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Musculoskeletal-System
MSK
[]
test-1541
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Eyes-Ears-Nose-Throat
EENT
[ "visual loss after diving from a diving board", "bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch ’s membrane were identified. The macula was not affected and vision returned to normal in both eyes", "recent visual loss in the left eye", "Visual acu...
test-1542
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Dermatology
DERM
[ "no typical plucked - chicken appearance, no papules, macules or other skin lesions. Three biopsies from the flexural skin of the axilla and the neck did not reveal PXE related changes" ]
test-1543
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Pregnancy
Pregnancy
[]
test-1544
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Lymphatic-System
LYMPH
[]
test-1545
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Age-at-Presentation
Age (at case presentation)
[ "21 - year - old" ]
test-1546
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Age-of-Onset
Age (of onset)
[]
test-1547
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
Confirmed-Diagnosis-IEM
Confirmed_Diagnosis(IEM)
[ "PXE" ]
test-1548
5015608
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
{'Case report': 'In 1989, a 21-year-old male experienced visual loss after diving from a diving board. Ocular examination was performed the same day, and revealed bilateral subretinal hemorrhages and angioid streaks. After clearing of the hemorrhages, more breaks of Bruch’s membrane were identified. The macula was not ...
IEM-Treatment
IEM_Treatment
[]
test-1549
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Vitals-and-Hematology
Vitals_Hema
[ "height and weight were 86 cm and 12.5 kg, respectively ( both were below the 3rd percentile, according to China 's 1995 urban 0–18 year - old male height percentiles scale", "height and weight were still below the 3rd percentile, at 116 cm and 20.1 kg, respectively", "his height and weight reached 121 cm and 2...
test-1550
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Gastrointestinal-System
GI
[ "showed a preference for carbohydrate intake from infancy, with a clear aversion to animal proteins and was prone to vomiting", "short stature and excessive thinness" ]
test-1551
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Patient-History
History
[ "The patient was born at 36 +4 weeks ’ gestation with a birth weight of 2500 g. Three days after birth, he was hospitalized for several days due to transient hypoglycemia. His developmental milestones were unremarkable before 1 year of age. Following this, his developmental milestones became delayed, for example, t...
test-1552
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Neurology
Neuro
[ "developmental milestones became delayed", "began to walk at 1 year and 6 months and his walk was slower than normal and clumsy", "A neurologic examination focusing on the motor and sensory nervous system and cerebellar function produced unremarkable findings", "a single episode of generalized tonic seizure",...
test-1553
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Laboratory-and-Imaging
Lab_Image
[ "Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.", "Laboratory tests revealed retardation of bone age ( equivalent to a 1 year old ) and mildly elevated total bilirubin ( 29.4 μmol / L, normal range : 2–24 μmol / L ), direct bilirubin ( 10.4 μmol / L...
test-1554
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Cardiovascular-System
CVS
[]
test-1555
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Endocrinology
ENDO
[ "retardation of bone age ( equivalent to a 1 year old )", "Blood glucose was slightly lower than normal ( 3.52 mmol / L, normal range : 3.9–6", "A growth hormone excitement test revealed low levels of growth hormone ( 0 minute : 0.76 ng / mL; 30 minutes : 4.5 ng / mL; 60 minutes : 6.3 ng / mL; 90 minutes : 4.1 ...
test-1556
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Genitourinary-System
GU
[]
test-1557
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Respiratory-System
RESP
[]
test-1558
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Musculoskeletal-System
MSK
[ "muscle strength of the low extremities was grade 4 ( Oxford Scale )", "His creatine kinase ( 564.4 IU / L, normal range : 55–190 IU / L ) and blood ammonia levels ( 62.3 mmol / L, normal range : 9–30 mmol / L ) were slightly elevated" ]
test-1559
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Eyes-Ears-Nose-Throat
EENT
[]
test-1560
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Dermatology
DERM
[]
test-1561
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Pregnancy
Pregnancy
[ "born at 36 +4 weeks ’ gestation with a birth weight of 2500 g. Three days after birth, he was hospitalized for several days due to transient hypoglycemia." ]
test-1562
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Lymphatic-System
LYMPH
[]
test-1563
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Age-at-Presentation
Age (at case presentation)
[ "10 years old" ]
test-1564
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Age-of-Onset
Age (of onset)
[ "1 year of age" ]
test-1565
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
Confirmed-Diagnosis-IEM
Confirmed_Diagnosis(IEM)
[ "argininemia with homozygous mutation ( c.32T > C ) of the ARG1 gene", "argininemia" ]
test-1566
5839826
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
{'Diagnoses:': 'Ultimately, the patient was diagnosed with argininemia with homozygous mutation (c.32T>C) of the ARG1 gene at 10 years old. Blood tests showed mildly elevated blood ammonia and creatine kinase, and persistently elevated bilirubin.', 'Case report': "The patient was born at 36 +4 weeks’ gestation with a b...
IEM-Treatment
IEM_Treatment
[ "Following the diagnosis , we recommended that the patient begins a low - protein diet ( 0.8 g / kg / day ) to decrease his blood arginine levels , as well as start taking citrulline ( 150–200 mg / kg / day ) ." ]
test-1567
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Vitals-and-Hematology
Vitals_Hema
[ "The patient 's height and body weight were 85.0 cm and 11.6 kg, respectively. Her weight - for - height Z - score ( −0.3 ) was in the 15th–50th percentile of the World Health Organization ( WHO ) growth standards", "Her body weight continuously declined from 11.6 kg to 10.5 kg, and her weight - for - height Z - ...
test-1568
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Gastrointestinal-System
GI
[ "swallowing difficulty", "symptoms of aspiration while swallowing and prolonged feeding time", "irregularly ingested a soft and blended diet", "She had difficulty in eating and she showed signs of aspiration ( e.g., coughing, gagging )" ]
test-1569
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Patient-History
History
[ "A 22 - month - old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control" ]
test-1570
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Neurology
Neuro
[ "chief complaints of spasticity and swallowing difficulty as well as difficulty with head control.", "She was able to stand while holding on to something at 8 months of age, which suggested normal development. However, developmental arrest occurred at 13 months of age. Spasticity and genu recurvatum were observed...
test-1571
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Laboratory-and-Imaging
Lab_Image
[ "Brain magnetic resonance imaging scans obtained at 15 months of age did not show delayed myelination or abnormal enhancement", "The patient underwent hexosaminidase assay in white blood cells, which showed increased hexosaminidase A ( % ) and decreased total hexosaminidase. The total hexosaminidase A & B and hex...
test-1572
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Cardiovascular-System
CVS
[]
test-1573
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Endocrinology
ENDO
[]
test-1574
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Genitourinary-System
GU
[]
test-1575
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Respiratory-System
RESP
[ "symptoms of aspiration while swallowing", "signs of aspiration ( e.g., coughing, gagging )" ]
test-1576
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Musculoskeletal-System
MSK
[ "genu recurvatum" ]
test-1577
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Eyes-Ears-Nose-Throat
EENT
[ "the patient did not show cherry red spot on fundoscopy" ]
test-1578
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Dermatology
DERM
[]
test-1579
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Pregnancy
Pregnancy
[ "born at full term through normal vaginal delivery" ]
test-1580
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Lymphatic-System
LYMPH
[]
test-1581
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Age-at-Presentation
Age (at case presentation)
[ "22 - month - old" ]
test-1582
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Age-of-Onset
Age (of onset)
[]
test-1583
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
Confirmed-Diagnosis-IEM
Confirmed_Diagnosis(IEM)
[ "Sandhoff disease", "the patient was diagnosed with Sandhoff disease ." ]
test-1584
5698679
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
{'CASE REPORT': "A 22-month-old girl diagnosed with Sandhoff disease was referred to our hospital with chief complaints of spasticity and swallowing difficulty as well as difficulty with head control. She was born at full term through normal vaginal delivery. She was able to stand while holding on to something at 8 mon...
IEM-Treatment
IEM_Treatment
[]
test-1585
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Vitals-and-Hematology
Vitals_Hema
[ "vital signs on admission showed uncontrolled hypertension ( BP = 148/118 mm Hg ) and tachycardia ( HR = 120 bpm )." ]
test-1586
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Gastrointestinal-System
GI
[ "history of recurrent abdominal pain", "nausea, vomiting, worsening of abdominal pain, and constipation", "unable to eat", "abdominal pain persisted", "abdominal pain resolved in less than 24 h" ]
test-1587
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Patient-History
History
[ "This is a 20 - year - old female with a 6 - month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days" ]
test-1588
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Neurology
Neuro
[ "two generalized tonic – clonic seizures", "she continued to be lethargic, and a stat EEG was requested, that showed LPDs + F over the right temporoparietal region", "She became profoundly lethargic and assumed a fetal position in bed. On neurological examination, there were left lower extremity clonus and a le...
test-1589
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Laboratory-and-Imaging
Lab_Image
[ "Head CT was normal, cerebral spinal fluid ( CSF ) was unremarkable, and comprehensive CSF profiles were negative, including PCR for Herpes Simplex virus, Varicella Zoster virus, Lyme disease, and Cryptococcus. The brain MRI showed PRES", "Urine, then serum and fecal porphyrins were requested. The results were as...
test-1590
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Cardiovascular-System
CVS
[]
test-1591
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Endocrinology
ENDO
[]
test-1592
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Genitourinary-System
GU
[ "acute pyelonephritis," ]
test-1593
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Respiratory-System
RESP
[]
test-1594
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Musculoskeletal-System
MSK
[]
test-1595
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Eyes-Ears-Nose-Throat
EENT
[]
test-1596
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Dermatology
DERM
[]
test-1597
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Pregnancy
Pregnancy
[]
test-1598
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Lymphatic-System
LYMPH
[]
test-1599
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Age-at-Presentation
Age (at case presentation)
[ "a 20 - year - old" ]
test-1600
5021915
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
{'Case presentation': "This is a 20-year-old female with a 6-month history of recurrent abdominal pain. She underwent cholecystectomy 3 months prior to admission but had no relief of her symptoms. She was admitted with acute pyelonephritis, nausea, vomiting, worsening of abdominal pain, and constipation for three days....
Age-of-Onset
Age (of onset)
[]