keppen–lubinsky syndrome keppen–lubinsky syndrome keppen–lubinsky syndrome is an extremely rare congenital disorder. the minimal clinical criteria for the keppen–lubinsky syndrome are as follows: normal growth parameters at birth, postnatal growth failure, peculiar face with an aged appearance (large prominent eyes, a narrow nasal bridge, a tented upper lip, a high palate, an open mouth), skin tightly adherent to facial bones, generalized lipodystrophy, microcephaly, and development delay. keppen-lubinsky syndrome is caused by mutation in the inwardly rectifying k+ channels encoded by kcnj6 gene. keppen–lubinsky syndromeother namesgeneralized lipodystrophy-progeroid features-severe intellectual disability syndromethis condition is inherited in an autosomal dominant manner