Familial syndrome with panhypopituitarism, hypoplasia of the hypophysis, and poorly developed sella turcica

家族性综合征伴全垂体功能减退、垂体发育不全和蝶鞍发育不良

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Abstract

Two sisters who died at the age of 2.5 years and 5 weeks are described. Both showed signs of panhypopituitarism. At necropsy, no hypophysis could be found in the first child and a rudimentary and partly ectopic hypophysis was found in the other. Both children had a flat, poorly developed sella turcica, and the sellar anomaly could be seen in skull x-rays. These patients represent a hereditary syndrome characterised by neonatal panhypopituitarism, hypoplasia of the pituitary gland, and flat sella turcica.

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