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What is Juene's syndrome is inherited as: (Repeat) and explain in detail?
Ans: A (AR) Ref: Robbins Pathologic Basis of Disease, 8th edition.Explanation:(Refer to explanation in 2011 Pediatrics Question) Jeune syndrome ( Familial asphyxiating thoracic dystrophy)Autosomal recessive disorderClassic manifestations in infancy includeDw arfism with short ribsShort limbsCharacteristic radiographic changes in ribs and pelvisSmall chestsRespiratory insufficiencyLung hypoplasia (Due to a restricted thoracic cage) Major cause of death in infancy.Renal and pancreatic insufficiency (in those w ho survive newborn period)Other Clinical FeaturesRespiratory distressChest deformity of varying degreeShort-limbed dwarfismFailure to thriveGastroenteritisRecurrent rectal prolapseDiarrheaCongestive cardiac failurePuffy face, andAnkle swelling