ABSTRACT
To establish the incidence of Nelson's syndrome in children treated with total bilateral adrenalectomy (TBA) for Cushing's disease, a survey was made of members of The Lawson Wilkins Pediatric Endocrine Society. Thirty-one patients aged 10 months to 16 years had been treated with TBA for Cushing's disease; one had been treated with ortho para prime isomer of dichlorodiphenyldichloroethane alone. Postadrenalectomy hyperpigmentation was reported in 18 patients. Sella enlargement was detected in eight patients (25%) after 1 to 5.5 years (mean, three years) post-TBA. Five of these patients have had documented pituitary adenomas to date. This incidence is higher than the adult figure of 10% to 16%.
Subject(s)
Adrenalectomy , Cushing Syndrome/surgery , Nelson Syndrome/epidemiology , Pituitary Neoplasms/epidemiology , Postoperative Complications/epidemiology , Adolescent , Child , Female , Follow-Up Studies , Humans , Male , Nelson Syndrome/diagnosis , Postoperative Complications/diagnosis , United StatesABSTRACT
Six girls, aged 5 to 15 years, presented with thyroid masses in otherwise nonpalpable thyroid glands and with normal serum thyroxine levels. Scintiscanning before and after TSH stimulation confirmed the presence of autonomous nodules in the four adolescents, of whom two had elevated T3 levels. Surgical exploration revealed adenomatous thyroid hyperplasia in three of the girls and papillary adenocarcinoma in the fourth. Scans in the other two girls revealed absence of the left lobe. One of them proved to have agenesis of the left lobe with enlargement of the right lobe because of lymphocytic thyroiditis. The other girl had an ectopic thyroid with chronic inflammation. A thorough diagnostic evaluation of single or multiple functioning thyroid masses in children and adolescents is essential in establishing the correct diagnosis. The possibility that carcinoma can occur in autonomous nodules as well as in hemiagenesis and ectopic thyroid tissue is discussed. An approach to the management of functioning thyroid masses in the pediatric age group is proposed.
Subject(s)
Thyroid Diseases/diagnosis , Thyroid Neoplasms/diagnosis , Adenocarcinoma, Papillary/diagnosis , Adolescent , Child , Child, Preschool , Female , Humans , Hyperplasia/diagnosis , Radionuclide Imaging/methods , Thyroid Function Tests/methods , Thyroid Gland/abnormalities , Thyroid Gland/pathology , Thyroid Neoplasms/surgery , Thyroiditis/diagnosisABSTRACT
Serum concentrations of unconjugated estrone, estradiol, and free estradiol, were determined in normal neonates, prepubertal children, adolescents, and adults. The values were compared with those obtained in children with premature thelarche and female sexual precocity. Unconjugated E1 and E2 fell rapidly, and the percentage of FE2 more gradually during the neonatal period and remained low prepubertally. During adolescence girls had greater increases in E1 and E2 while the percentage of FE2 was higher in boys. In premature thelarche only the FE2 was significantly increased. In sexual precocity E1, E2, and FE2 were elevated. Reference standards are provided in Tables I and II for use in the diagnosis of conditions with under- or overproduction of estrogens during growth and development.
Subject(s)
Estradiol/blood , Estrone/blood , Growth , Puberty, Precocious/blood , Adolescent , Adult , Age Factors , Child , Child, Preschool , Dialysis , Estradiol/analysis , Estrone/analysis , Female , Humans , Infant , Infant, Newborn , Male , Puberty , RadioimmunoassayABSTRACT
Fifty-two children with growth hormone (GH) deficiency were examined for factors that might influence development of hypoglycemia. Symptomatic and asymptomatic hypoglycemia occurred with equal frequency in children with isolated GH and multiple anterior pituitary deficiencies. Of 52 children, nine (17%) had symptomatic hypoglycemia and 14 (27%) had asymptomatic hypoglycemia. Symptomatic hypoglycemia was more frequent in children who were both young (less than 4 years of age) and lean (elevated height age/weight age [HA/WA] ratio). With HGH therapy, these children had decreases in HA/WA ratios and improvement in carbohydrate homeostasis. Insulin responses to oral glucose and intravenous arginine administration were substantially lowered in children with symptomatic hypoglycemia, A deficiency of gluconeogenic substrate or impairment of amino acid mobilization may be a factor in the development of hypoglycemia in hypopituitarism similar to that postulated for ketotic hypoglycemia.
Subject(s)
Hypoglycemia/etiology , Hypopituitarism/metabolism , Adolescent , Adrenal Cortex Function Tests , Adrenocorticotropic Hormone/deficiency , Age Factors , Body Weight , Carbohydrate Metabolism , Child , Child, Preschool , Female , Glucose Tolerance Test , Growth Hormone/deficiency , Humans , Hypoglycemia/diagnosis , Hypopituitarism/diagnosis , Infant , Male , Thyroid Function TestsABSTRACT
Subacute thyroiditis in a lateral, ectopic thyroid has been previously unreported. A 4 10/12-YEAR-OLD GIRL HAD AN ENLARGING MASS IN THE LEFT UPPER ANTERIOR NECK. Initially, the serum concentration of T4 was normal, T3 was elevated, and TSH was undetectable without response to TRH. RAI uptake was 1%. The data were consistent with subacute thyroiditis. Twelve weeks later the serum concentration of T4 was low and TSH was elevated; thyroid replacement therapy was given for 20 weeks. When this was discontinued, there was an initial increase and then a decrease in the TSH values accompanied by an increase in serum concentrations of T3 and T4 to normal during eight weeks. One must consider a lateral ectopic thyroid gland in the differential diagnosis of masses in the neck. Physicians must be aware that temporary hypothyroidism occurs during the course of subacute thyroiditis.
Subject(s)
Hypothyroidism/etiology , Pituitary Gland/physiopathology , Thyroid Gland/abnormalities , Thyroiditis/blood , Thyrotropin-Releasing Hormone/blood , Thyrotropin/blood , Thyroxine/blood , Triiodothyronine/blood , Acute Disease , Antibodies/analysis , Child, Preschool , Female , Humans , Hypothyroidism/blood , Hypothyroidism/drug therapy , Radioimmunoassay , Thyroid Function Tests , Thyroid Hormones/administration & dosage , Thyroid Hormones/therapeutic use , Thyroiditis/drug therapy , Thyroxine-Binding Proteins/bloodABSTRACT
Altered carbohydrate metabolism associated with fibrosarcomas and chondrosarcomas has been well-documented in past literature. This report describes abnormal carbohydrate metabolism in 2 osteosarcoma patients, and abnormalities in growth hormone and somatomedin serum levels. Experimental evidence is presented showing in vitro suppression of osteosarcoma tumor cell proliferation by 17 beta Estradiol. Estrogen inhibition of linear bone growth, cartilage proliferation, and somatomedin is discussed with reference to possible estrogen therapy in osteosarcoma.
Subject(s)
Carbohydrate Metabolism , Osteosarcoma/metabolism , Somatomedins/blood , Adolescent , Bone Development/drug effects , Cartilage/growth & development , Cells, Cultured , Estradiol/pharmacology , Female , Growth Hormone/blood , Humans , Male , Methotrexate/pharmacology , Osteosarcoma/drug therapy , Sex FactorsSubject(s)
Cortisone/therapeutic use , Addison Disease/drug therapy , Administration, Oral , Adolescent , Adrenocortical Hyperfunction/drug therapy , Age Factors , Asthma/drug therapy , Body Weight , Child , Child, Preschool , Cortisone/administration & dosage , Cortisone/adverse effects , Cushing Syndrome/chemically induced , Female , Glucocorticoids/adverse effects , Glucocorticoids/therapeutic use , Humans , Hydrocortisone/analogs & derivatives , Hydrocortisone/metabolism , Infant , Infant, Newborn , Male , Metyrapone , Prednisone/adverse effects , Time FactorsSubject(s)
Black People , Gonadotropins, Pituitary/blood , Puberty , Adolescent , Child , Female , Follicle Stimulating Hormone/blood , Humans , Luteinizing Hormone/blood , Male , Menstruation , Sex Characteristics , Sex Factors , White PeopleSubject(s)
17-Hydroxycorticosteroids/urine , Adrenal Glands/physiology , Aging , Growth , Hydrocortisone/urine , Adolescent , Adult , Age Factors , Body Composition , Body Surface Area , Body Weight , Child , Child, Preschool , Creatinine/urine , Female , Humans , Hydrocortisone/biosynthesis , Infant , Male , Middle Aged , Muscles , Statistics as TopicSubject(s)
Adrenal Insufficiency/enzymology , Chorionic Gonadotropin/pharmacology , Disorders of Sex Development/enzymology , Hydroxysteroid Dehydrogenases/metabolism , Metabolism, Inborn Errors/enzymology , Adolescent , Adrenal Insufficiency/diagnosis , Androstenedione/biosynthesis , Child , Child, Preschool , Dehydroepiandrosterone/biosynthesis , Dexamethasone/pharmacology , Disorders of Sex Development/diagnosis , Humans , Hydroxysteroid Dehydrogenases/deficiency , Infant , Male , Metabolism, Inborn Errors/diagnosis , Testis/drug effects , Testis/enzymology , Testosterone/biosynthesisSubject(s)
Estradiol/blood , Estrone/blood , Follicle Stimulating Hormone/blood , Luteinizing Hormone/blood , Puberty , Adolescent , Age Factors , Child , Female , Humans , Male , Radioimmunoassay , Sex FactorsABSTRACT
The patient was the fourth of affected male siblings. Cortisol (1.3 micrograms per cent), cortisone (9.6), and corticosterone sulfate (0.1) concentrations were low in cord blood. The larger amount of cortisone may have originated from maternal cortisol. Aldosterone was undetectable in cord blood, indicating lack of fetal secretion or maternofetal transfer. Unexpectedly normal concentrations of 11-deoxycorticosterone (DOC) sulfate in cord serum could represent maternal transfer of DOC, with subsequent fetal sulfurylation. Low estrone and estradiol concentrations in maternal and cord serum were consistent with absence of the fetal adrenals. Despite the low levels of the steroids, the propositus had a normal lecithin-sphingomyelin ratio at 38 weeks' gestation. Circulatory insufficiency developed within half an hour after birth and responded to gluco- and mineralocorticoid therapy. The three untreated siblings died between 14 and 67 hours of age. It is evident that early recognition of this condition may be lifesaving.
