Light chain deposition disease presenting as cholestatic jaundice: a case report

Light-chain deposition disease [LCDD] is characterized by tissue deposition of the immunoglobulin light chains in multiple organs. These deposits appear similar to amyloid on routine sections, but differ in their staining properties and ultrastructural appearance. The deposits of LCCD are non -Congophilic and do not exhibit a fibrillar ultrastructure; while, the proteinaceous substance seen in primary amyloidosis is Congo red positive and fibrillar. One of the most common organs to be involved in LCDD is the kidney. Earlier reports on cases of LCDD have mostly shown simultaneous liver and renal involvement, there are very few cases in the literature describing LCDD of the liver without renal involvement. This report describes a patient who presented with severe cholestatic jaundice and liver cell failure with normal renal function

Fraser syndrome in three consecutive siblings

Fraser syndrome [FS] is a rare disorder characterized by a combination of acrofacial and urogential malformations with or without cryptophthalmos. We report a newborn and its two elder siblings who had multiple congenital anomalies and clinico-radiological features consistent with FS