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

Author:

Sipponen P,Simila S,Collan Y,Autere T,Herva R

Publisher

BMJ

Subject

Pediatrics, Perinatology and Child Health

Reference19 articles.

1. The necropsy findings showed a severe hypophyseal anomaly in both of these sisters. A hypophysis could not be found in Case 1 who lived for 2*5 years. The fact that she lived this long might suggest that some hypophyseal tissue was present. Usually, patients with hypophyseal aplasia die soon after delivery, although there are some who have lived for years with intensive replacement therapy (Steiner and Boggs, 1965; Sadeghi-Nejad and Senior,1974

2. In addition to hypoplastic hypophysis, Case 2 showed clinical and laboratory evidence suggestive of polyendocrinopathy-primary hypocortisolism, slight primary hypothyroidism, and deficiency of HGH. Both patients suffered from severe hypoglycaemia and, in addition, atrophy of both thyroid and adrenal glands was revealed at necropsy. Clinical findings have been uniform in cases showing hypoplasia of the hypophysis (Blizzard and Alberts, 1956; Mosier, 1956; Ehrlich;and Stone, 1969, Johnson;American Journal of Obstetrics and Gynecology; or those in whom aplasia of the hypophysis was present,1957

3. Hypopituitarism, hypoadrenalism, and hypogonadism in the newborn infant;Blizzard, R.M.; Alberts, M.;Joutnal ofPediatrics,1956

4. An evaluation of seventy-five patients with hypopituitarism beginning in childhood;Brasel, A.; Wright, J.C.; Wilkins, L.; Blizzard, R.M.;American Journal of Medicine,1965

5. Congenital absence of the pituitary gland and its consequences;Brewer, D.B.;Journal of Pathology and Bacteriology,1957

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