CONGENITAL HEPATIC FIBROSIS WITH POLYCYSTIC KIDNEY DISEASE PRESENTED AS PORTAL HYPERTENSION IN A 7 YEAR OLD FEMALE CHILD – A RARE CASE REPORT

Syed Sardar Sulthana, Mallikarjuna M, Rajesh Goud S

Abstract


INTRODUCTION
Congenital hepatic fibrosis (CHF) is a rare, autosomal recessive
disorder, clinically characterized by hepatic fibrosis and portal
hypertension. CHF results from ductal plate malformation (DPM) of
the intrahepatic bile ducts. Four clinical forms can be observed: portal
hypertensive, cholangitic, mixed and latent. CHF is one of the
“fibropolycystic diseases” which also include several conditions with
a variety of intrahepatic bile duct dilatation and associated periportal
fibrosis such as Caroli disease, autosomal recessive and dom-inant
polycystic kidney disease (ARPKD or ADPKD), Ivemark, Jeune,
Joubert, Bardet-Biedl, Meckel-Gruber and Arima syndromes. Most of
them are accompanied by progressive cystic degeneration of the
kidneys. We present the case of a 7-year-old female patient with CHF
with POLYCYSTIC KIDNEY DISEASE and a review of the
literature.


Keywords


congenital hepatic fibrosis, portal hypertension, ductal plate malformation, esophageal varices.

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References


Gomez D, Haas B, Ahmed N, et al. Disaster preparedness of Canadian trauma centres: the perspective of medical directors of trauma. Can J Surg 2010;54:9-16.

Kaji A H .L Lewis R J Hospital disaster preparedness in Los Angel country California. Annals of Emergency Medicine 2004 0ct ;44(4)S33.

Chandorkar A G, Das J K, Jain A K, Satyanarayana P, Misra MC. Certificate course in hospital management- patient care and services New Delhi; National institute of health and family welfare New Delhi;2011

Gupta P khanna A, Majumdar S. Disaster management in flash floods Leh (Ladakh) A case study Indian J community Med . 2012;37(3)1985-190


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