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Target Concepts:
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Query: UMLS:C0010200 (
cough
)
23,843
document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)
Benign recurrent intrahepatic cholestasis
(
BRIC
) is a syndrome characterized by recurrent episodes of cholestasis with associated pruritus. The intensity and duration of cholestatic episodes, and the length of the intervening periods, vary unpredictably. We report the case of a patient with
BRIC
who was incapacitated by a severe intractable
cough
that accompanied marked pruritus during her second cholestatic episode. No cause for the
cough
was found, and it resolved spontaneously with amelioration of mild restrictive abnormalities of pulmonary function as the cholestasis subsided. Although
cough
has not been recognized as a complication of cholestasis, we postulate that it may occur either (i) as a result of direct stimulation of sensory nerves by circulating humoral substances related to the cholestasis that act either peripherally in the airways or centrally, or (ii) through stimulation of the vagus nerve in the liver, leading to
cough
that is mediated either centrally or by reflex.
...
PMID:Intractable cough and abnormal pulmonary function in benign recurrent intrahepatic cholestasis. 885 52
Benign recurrent intrahepatic cholestasis
(BRIC or Summerskill-Walshe-Tygstrup-syndrome) is a rare autosomal recessive form of liver disease, which usually becomes manifest in childhood. Characteristic are recurrent episodes of jaundice and itching of different duration. Number and duration of episodic attack and asymptomatic period develop individually. For diagnosis of BRIC following criteria are proposed: At least three episodes of severe jaundice and pruritus with biochemical evidence of cholestasis, normal intra-and extrahepatic bile ducts on cholangiography, absence of a factor known to produce intrahepatic cholestasis and symptom-free intervals of several months or years. Often the diagnosis of BRIC is made very late and patients have to suffer invasive investigations (explorative laparotomy). Because of the unknown pathophysiological mechanism there is no specific treatment. We report on a 53-year-old patient with jaundice, severe pruritus, vomiting, loss of hair and weight, extreme sleeplessness and intractable
cough
. At the onset of the attack an increase of serum bilirubin concentration and serum alkaline phosphatase was observed, whereas aspartate and alanine aminotransferase and gamma-glutamyltransferase were normal. Histological findings of liver biopsy revealed accumulation of bile plugs in bile canaliculi. The long-term follow-up of our patient confirms that the prognosis is good.
...
PMID:[Follow-up of benign recurrent intrahepatic cholestasis (Summerskill-Walshe-Tygstrup syndrome) over 46 years]. 965 5