Neonatal cholestasis as the presenting feature in cystic fibrosis.

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Neonatal cholestasis as the presenting feature in cystic fibrosis.

Between 1960 and 1994 cystic fibrosis was found in nine out of 1474 infants investigated for neonatal cholestasis. Four had delay in passing meconium. In all patients cholestatic jaundice was present during the first 48 hours and in three patients cholestasis was complete, mimicking biliary atresia. Serum cholesterol concentrations were normal in all but two children. Sweat chloride was repeate...

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Background: Appendicovesical fistula in patients suffering from cystic fibrosis is a rare condition. Although this situation is so rare it should be considered in the differential diagnosis, dealing with a patient with cystic fibrosis and chronic abdominal pain with no response to primary management. To the best of our knowledge, this is the third case of appendicovesical fistula in a patient w...

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Hemorrhagic Diathesis as the Presenting Symptom of Neonatal Cholestasis

A 4-week-old infant presented with a coagulation disorder resulting from a vitamin K deficiency. The vitamin K deficiency was caused by neonatal cholestasis due to biliary atresia. Jaundice, hepatomegaly and pale stools are the predominant presenting symptoms of biliary atresia, none of which were recognized in our patient before admission. However, the patient presented with bleeding caused by...

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Dubin-Johnson syndrome presenting with neonatal cholestasis.

We report a case of Dubin-Johnson syndrome presenting with neonatal cholestasis. Liver histology was studied during the neonatal period and at 6 years of age. Distinct brownish pigment granules in hepatocytes were noted. This case confirms that Dubin-Johnson syndrome is a cause of neonatal cholestasis.

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Growth failure is a common presentation of patients with pancreatic insufficient cystic fibrosis. However, full blown kwashiorkor is extremely rare. Cystic fibrosis is also considered to be rare in the South Asian population. This report describes a Sri Lankan infant with cystic fibrosis who presented with clinical features of severe kwashiorkor.

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ژورنال

عنوان ژورنال: Archives of Disease in Childhood

سال: 1996

ISSN: 0003-9888,1468-2044

DOI: 10.1136/adc.75.1.67