This is a real-world medical entrance exam question, please give the true answer based on the question and selection. Topic name: Mendelian Disorders: Single-Gene Defects, Subject name: Pathology
[{'content': "Juene's syndrome is inherited as: (Repeat) \nSelect and explain. \nA. AR, B. AD, C. XR, D. XD", 'role': 'user'}, {'content': 'Answer: A \nExplanation: 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', 'role': 'assistant'}]