[Benign recurrent intrahepatic cholestasis. Description of a clinical case].
We present the clinical case of an 8 years old boy affected by episodes of severe recurrent jaundice, preceded by intense itching with clinical and biochemical signs of cholestasis, diagnosed as benign recurrent intrahepatic cholestasis (B.R.I.C.), or Summerskill's syndrome. This was first described by this author in 1959. The syndrome appears as a rare form of cholestatic jaundice of unknown pathogenesis, which in 80% of cases shows up before the age of 20. Its clinical characteristics are episodes of severe jaundice preceded by intense itching with biochemical signs of cholestasis which rise with no apparent cause and which recover spontaneously and are intervalled by asymptomatic periods which last months or years. During this time there is also a regression of the chemical and histological evidence of cholestasis. The diagnosis of B.R.I.C. can be made after having excluded the other congenital or acquired causes of intrahepatic cholestasis according to the recurrent character of the jaundice and to the hepatic biopsy.
Bruni
L
L
Istituto di Clinica Pediatrica dell'Università, La Sapienza, Policlinico Umberto I di Roma, Italia.
Amato
R
R
Tozzi
M C
MC
Tarani
L
L
Vignetti
P
P
D002648:Child; D002780:Cholestasis, Intrahepatic / Q000175:diagnosis; D003937:Diagnosis, Differential; D006801:Humans; D008297:Male; D012008:Recurrence
Colestasi intraepatica ricorrente benigna. Descrizione di un caso clinico.