A Case of Familial Intrahepatic Cholestasis

Hirooka, Minoru; Ohno, Tadasi

doi:10.1620/tjem.94.293

Cited by 26 publications

(8 citation statements)

References 16 publications

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“…In each of our cases, the liver was enlarged, greenish, regular, or already nodular (Case 5). These findings are identical to the other 10 published cases in which laparotomy (Clayton et al, 1969;Gray and Saunders, 1966;Hirooka and Ono, 1968;Williams et al, 1972. ) Histological examination of the liver in 4 cases showed portal fibrosis with a moderate number of round, inflammatory cells.…”

Section: Discussionsupporting

confidence: 90%

“…The clinical picture in these 5 children was identical to that of 16 published cases (Clayton et al, 1965(Clayton et al, , 1969Gray and Saunders, 1966;Hirooka and Ono, 1968;Linarelli et al, 1972;Schubert, 1965;Williams et al, 1972).…”

Section: Discussionsupporting

confidence: 78%

“…It presents with the features of familial cholestatic cirrhosis, with a gradually fatal course. Since 1965, other cases have been published (Clayton et al, 1969; Gray and Saunders, 1966;Hirooka and Ono, 1968;Linarelli, Williams, and Phillips, 1972;Williams et al, 1972. ) The present work relates the clinical and biological study and the course of development of 5 new cases belonging to 4 different families.…”

mentioning

confidence: 99%

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Severe familial intrahepatic cholestasis

Odièvre¹,

Gautier²,

Hadchouel³

et al. 1973

Archives of Disease in Childhood

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Section: Discussionsupporting

confidence: 90%

Section: Discussionsupporting

confidence: 78%

mentioning

confidence: 99%

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Severe familial intrahepatic cholestasis

Odièvre¹,

Gautier²,

Hadchouel³

et al. 1973

Archives of Disease in Childhood

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“…Ears were malformed in R II.3, and prominent in 5 other cases. Some of these features could be recognized in the apparently affected parent, especially in families M, R, and S. The eyes were in general rather deep set and the extremities were normal; no one had the prominent eyes and stubby fingers noted in cases of cholestasis by Juberg et al (1966) and Hirooka and Ohno (1968). 2 children (R II.3 and S II.2) had ectopic testes, and one parent (M I.1) had a round dorsal kyphos.…”

Section: Special Featuresmentioning

confidence: 87%

Arteriohepatic dysplasia: Familial pulmonary arterial stenosis with neonatal liver disease

Watson¹,

Miller²

1973

Archives of Disease in Childhood

228

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“…Although eight out of 18 reported cases belong to the Amish kindred (Clayton et al, 1969;Linarelli et al, 1972) the affection seems not limited to one race or family (Gray and Saunders, 1966;Juberg et al, 1966;Hirooka and Ohno, 1968;Williams et al, 1972). The purpose of this paper is to report the clinical and morphological findings in a patient of Moroccan origin.…”

mentioning

confidence: 89%

Progressive intrahepatic cholestasis (Byler's disease): case report.

Vos¹,

Wolf‐Peeters²,

Desmet³

et al. 1975

Gut

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943and craniotabts were present (Fig. 1). The liver was hard and palpable 2 cm below the costal margin. The spleen could not be felt. Over the two years of observation scratching and unexplained fever up to 38-39 'C were continuously noticed (Fig. 2). Subcutaneous haemorrhages with hypoprothrombinaemia occurred at the age of 5 and 25 months respectively. Up to the age of 1 year total serum bilirubin remained below 1 mg % (Fig. 3). The stools were intermittently gray or white and the urine contained urobilinogen and on some occasions bilirubin. Thin layer chromatography of ethyl anthranilate azopigment extracts of the duodenal fluid showed presence of the fl-compound and increase of the 'y-fraction which is suggestive for cholestasis: oto 13.1, oci P0, 0x2 09, 0t 333, ,B 6 1, y 27.9 and 8 47*7 % (Fevery et al., 1972). At the end of the first year, transaminases were moderately raised. Serum nucleotidase remained as low as 1*7-13 IU/l. At the age of 71 months, bile salts in the serum and duodenal content were 82 ,ug (normal value 1 gg) and 150 ,ug (normal value 3-5 mg) per ml respectively. On two more occasions, no bile salts were detectable in yellow stained duodenal fluid samples. The daily urinary excretion of bile salts was 24.7 mg of which chenodeoxycholic acid and cholic acid made up 12-6 % and 87-4 % respectively. No lithocholic acid could be detected. After the age of 1 year serum bile acids were studied only once and were 107 ,ug/ml. The percentage of sulfobromo-

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A Case of Familial Intrahepatic Cholestasis

Cited by 26 publications

References 16 publications

Severe familial intrahepatic cholestasis

Severe familial intrahepatic cholestasis

Arteriohepatic dysplasia: Familial pulmonary arterial stenosis with neonatal liver disease

Progressive intrahepatic cholestasis (Byler's disease): case report.

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