Gauchers disease in early infancy

The first case of Gauchers disease observed in the Philippines is reported in an infant of 10 monthsThe disease is characterized by storage of kerasin in the cells of the reticulo-endothelial apparatus resulting in marked splenomegaly and liver enlargementThe different types of lipoid storage diseases, diagnosis and treatment of Gauchers disease are discussed. (Summary)

Sheehan's Syndrome: UP-PGH experience from 1974-1984

Nineteen patients with a diagnosis of Sheehan's syndrome or postpartum hypopituitarism admitted to the UP-PGH Medical Center from 1974-1984 were reviewed. The history, signs and symptoms, and results of various laboratory examinations were assessed. Two patients had available postmortem reports Panhypopituitarism (89%) was more common than selective hypopituitarism. LSH producing cells seemed to be spared more often than the others. Hypothyroidism was seen in only 89% of the cases as compared to gonadal failure (100%), and cortisol insufficiency (100%). The pituitary involvement was not limited to the anterior pituitary but may involve the posterior pituitary and or the hypothalamus as suggested by the coexistence of manifestations of diabetes insipidus in 2 of our patients Most common cause of postpartum pituitary necrosis was obstetric hemorrhage but it could also be produced by any condition resulting in severe shock. The syndrome was usually heralded by failure of lactation and amenorrhea associated later with loss of axillary and public hair; genital, uterine, and breast atrophy; symptoms and signs of hypothyroidism and various degrees of adrenocortical insufficiency The different tests designed to show target organ failure did not show positivity in all patients. Among the tests which showed consistent results and thus were most helpful for diagnosis were determinations of: FSH, cortisol and T4 There is excellent prognosis for a productive life of patients with replacement of all target gland hormonal deficiencies. However, failure to diagnose it is disastrous for the patients. Continuation of treatment especially in the presence of stress, i.e. infection should be emphasized by the physicians. Regular follow-up at intervals of all patients with Sheehan's syndrome is necessarily recommended, (Summary)