Indian Pediatrics 2001; 38: 1326-1327  

Reply

The association of infantile polycystic kidney disease and hepatic fibrosis is well documented. Meckel Gruber syndrome is characterized by encephalocele, polycystic kidney disease, polydactyly with or without cleft lip and palate(1-3). Hepatic fibrosis is not a feature of this syndrome although occasional case reports of hepatic fibrosis have been reported in conditions resembling this syndrome. Both infantile polycystic kidney disease and Meckel Gruber syndrome are inherited as autosomal recessive diseases. There could be some overlap between the features of these two conditions. The sentence referred by de Silva et al should have been stated as "occurrence of hepatic fibrosis in Meckel Gruber syndrome is unusal" in our article. However the case reported by us did not have polydactyly and cleft lip or palate but had telepes equinovarus. Thus the case reported by us is unusual.

Surendra Kumar,
Vishnu Bhat,
Department of Pathology and Pediatrics,
JIPMER, Pondicherry,
India.

1. Brown JK, Minas RA. Disroder of the central nervous system. In: Forfar and Arneil’s Text-book of Pediatrics, 5th edn. Eds. Campbell AGM, Macintosh N, New York, Churchill Livingstone, 1998; pp 641-846.

2. Shephard B, Kupke KG. Genetics of common problems presenting in the newborn. In: Avery’s Diseases of the Newborn, 7th edn. Eds. Taeusch HW, Ballard RA. Philadelphia, W.B. Saunders Co. 1998; pp 200-208.

3. Blyth H, Ockenden BG. Polycystic disease of kidneys and liver presenting in childhood. J Med Genet 1971; 8: 257-284.