Familial intrahepatic cholestatic jaundice in infancy.

Gray, O.P.; Saunders, Richard

doi:10.1136/adc.41.217.320

Cited by 52 publications

(19 citation statements)

References 9 publications

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“…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: 78%

mentioning

confidence: 99%

Severe familial intrahepatic cholestasis

Odièvre¹,

Gautier²,

Hadchouel³

et al. 1973

Archives of Disease in Childhood

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“…These conditions of our patient are considered to be similar to those of patients, which have been recently reported by Clayton et al (1965), Gray et al (1966) and Juberg et al (1966), except for some minor differences. …”

mentioning

confidence: 75%

A Case of Familial Intrahepatic Cholestasis

Hirooka

Ohno

1968

Tohoku J. Exp. Med.

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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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Familial intrahepatic cholestatic jaundice in infancy.

Cited by 52 publications

References 9 publications

Severe familial intrahepatic cholestasis

Severe familial intrahepatic cholestasis

A Case of Familial Intrahepatic Cholestasis

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

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