Biomaterial Database

Russell-Silver syndrome and hypopituitarism. Patient report and literature review. 1986

S B Cassidy, and O Blonder, and V W Courtney, and S K Ratzan, and D E Carey

Russell-Silver syndrome (RSS) is a sporadic form of prenatal onset dwarfism with typical facial features, variable asymmetry, and linear growth 3 to 4 SDs below the mean. Endocrinologic studies are usually normal; however, six cases of RSS with growth hormone deficiency have been reported, three of which had additional pituitary abnormalities. We describe another case, a 7-year-old girl with RSS and deficiencies of growth hormone, corticotropin, and thyroid-stimulating hormone. Replacement therapy including growth hormone resulted in an improved growth velocity, though twice the usual dose of growth hormone was required and short stature persisted. Since growth hormone secretion is usually normal in RSS, the existence of individuals with RSS phenotype and hypopituitarism including growth hormone deficiency suggests etiologic heterogeneity. We recommend that those individuals with RSS phenotype and a continuous significant decline in height velocity be investigated for pituitary abnormalities. Unusually high replacement doses of growth hormone may be required to overcome deficiency.

UI	MeSH Term	Description	Entries
D007003	Hypoglycemia	A syndrome of abnormally low BLOOD GLUCOSE level. Clinical hypoglycemia has diverse etiologies. Severe hypoglycemia eventually lead to glucose deprivation of the CENTRAL NERVOUS SYSTEM resulting in HUNGER; SWEATING; PARESTHESIA; impaired mental function; SEIZURES; COMA; and even DEATH.	Fasting Hypoglycemia,Postabsorptive Hypoglycemia,Postprandial Hypoglycemia,Reactive Hypoglycemia,Hypoglycemia, Fasting,Hypoglycemia, Postabsorptive,Hypoglycemia, Postprandial,Hypoglycemia, Reactive
D007018	Hypopituitarism	Diminution or cessation of secretion of one or more hormones from the anterior pituitary gland (including LH; FOLLICLE STIMULATING HORMONE; SOMATOTROPIN; and CORTICOTROPIN). This may result from surgical or radiation ablation, non-secretory PITUITARY NEOPLASMS, metastatic tumors, infarction, PITUITARY APOPLEXY, infiltrative or granulomatous processes, and other conditions.	Adenohypophyseal Hyposecretion,Anterior Pituitary Hyposecretion Syndrome,Sheehan Syndrome,Simmonds Disease,Hyposecretion Syndrome, Anterior Pituitary,Hyposecretion, Adenohypophyseal,Pituitary Insufficiency,Postpartum Hypopituitarism,Postpartum Panhypopituitarism,Postpartum Pituitary Insufficiency,Sheehan's Syndrome,Simmonds' Disease,Disease, Simmonds,Hypopituitarism, Postpartum,Insufficiency, Pituitary,Panhypopituitarism, Postpartum,Pituitary Insufficiency, Postpartum,Sheehans Syndrome,Simmond's Disease,Syndrome, Sheehan,Syndrome, Sheehan's
D007223	Infant	A child between 1 and 23 months of age.	Infants
D008297	Male		Males
D001827	Body Height	The distance from the sole to the crown of the head with body standing on a flat surface and fully extended.	Body Heights,Height, Body,Heights, Body
D002648	Child	A person 6 to 12 years of age. An individual 2 to 5 years old is CHILD, PRESCHOOL.	Children
D002675	Child, Preschool	A child between the ages of 2 and 5.	Children, Preschool,Preschool Child,Preschool Children
D003350	Cortodoxone	17,21-Dihydroxypregn-4-ene-3,20-dione. A 17-hydroxycorticosteroid with glucocorticoid and anti-inflammatory activities.	11-Deoxycortisol,11-Desoxycortisone,Cortexolone,11-Desoxycortisol,Reichstein's Substance S,11 Deoxycortisol,11 Desoxycortisol,11 Desoxycortisone,Reichstein Substance S,Reichsteins Substance S,Substance S, Reichstein's
D004392	Dwarfism	A genetic or pathological condition that is characterized by short stature and undersize. Abnormal skeletal growth usually results in an adult who is significantly below the average height.	Nanism
D005260	Female		Females