Survey of clinical practice: growth hormone use during illness.

Prior to the 1999 National Cooperative Growth Study (NCGS) meeting, a postal card survey was conducted of the NCGS investigators about their current practices regarding continuation of growth hormone (GH) therapy during intercurrent illnesses. The survey results were subsequently compared with responses to the same questions obtained from NCGS investigators who attended lectures at the NCGS meeting describing the physiology and effects of GH in critically ill patients. Comparing results from the two surveys, there were no observed differences in the responses with respect to practices relative to the intercurrent illnesses treated at home. The percentage of NCGS investigators who indicated that they would stop GH treatment during more serious illnesses was greater following attendance at the lectures.

The adrenal incidentaloma. A pediatric perspective.

In our commentary, we have reviewed the literature on adrenal incidentalomas and specifically have tried to relate it to the pediatric population. The studies of others have shown that adrenal tumors are very common in patients with CAH (homozygote and heterozygote) and that these tumors are very rarely malignant and therefore should not routinely be surgically removed. In fact, we suggest that only under very rare circumstances is surgical removal justified. Perhaps, in the future, biochemical markers will allow us to distinguish between adrenal adenomas and carcinomas. We have tried to give broad guidelines for the care of patients with CAH with adrenal tumors, but these are guidelines only--they are not laws chiseled in stone. Finally, as radiological imaging is done more frequently and becomes even better at finding small adrenal tumors, this general topic will become increasingly relevant. We hope our thoughts will lead to lively discussion and useful studies in this "newly discovered" medical dilemma.

Bromocriptine-unresponsive prolactin macroadenoma in a prepubertal female.

Macroprolactinomas occur rarely in prepubertal children. A prepubertal 11 10/12-year-old female with a macroprolactinoma presented with a diagnosis of anorexia nervosa. Findings included severe headaches, anorexia, weight loss, and growth failure. The initial serum prolactin level was 2,916 ng/ml. This value fell after beginning treatment with bromocriptine, but later rose despite a doubling of the dose. Transsphenoidal surgical resection was performed and postoperative radiation given. The use of bromocriptine, surgical resection, and radiation therapy in the treatment of macroprolactinomas are discussed.

Rapid adrenocortical recovery after short-term glucocorticoid therapy.

The recovery time of adrenocortical function was studied in 13 children receiving intermittent high-dosage glucocorticoid therapy (prednisone, 2 mg/kg/day) for one month out of every four months as treatment for acute leukemia. The data indicate that the adrenal gland's ability to respond to stress recovers rapidly from such treatment. The results of our study clearly show that routine supplementation with glucocorticoid therapy in these patients during periods of clinical stress occurring later than nine days after glucocorticoid therapy is not warranted.

H-Y antigen in XO/X,iso(X) mosaic Turner syndrome.

Androgen excess, found occasionally in gonadal dysgenesis, may be associated with hilus cell hyperplasia within the gonadal streaks. This report describes a phenotypic female with Turner syndrome, clitoromegaly, hilus cell hyperplasia, and a gonadal-to-peripheral venous step-down gradient for testosterone, androstenedione, and estradiol. Cytogenetic analysis of cells from peripheral blood and both streak gonads revealed a mosaic karyotype, 45,X/46,X,iso(X). The isochromosome was a nonfused dicentric type (pter leads to q2105 leads to pter). Despite absence of the Y chromosome, serologic analysis revealed the presence of H-Y antigen, thereby suggesting occult Y chromosomal function in the patient's gonads.

Low-dose intravenous insulin in the treatment of diabetic ketoacidosis.

Continuous slow intravenous infusion of insulin was used in 52 episodes of diabetic ketoacidosis. No complications of therapy, ie, hypoglycemia, induced hypokalemia, insulin resistance, or cerebral edema, were encountered. Potassium phosphate was given to 47 of the 52 patients. Sodium bicarbonate was administered to only one patient. The hyperglycemia frequently resolved more rapidly than the systemic acidosis; this was managed by adding glucose to the intravenous fluids when the blood sugar concentration decreased to approximately 250 mg/dL; insulin infusion, however, was continued until the acidosis was corrected (venous standard bicarbonate greater than 14 mEq/L). We have found this method of treatment to be safe and simple to administer, and we believe it is the preferred treatment of patients with diabetic ketoacidosis.

Low-dose intravenous insulin infusion in patients with diabetic ketoacidosis: biochemical effects in children.

Twenty-five episodes of diabetic ketoacidosis in 20 children were treated with continuous low-dose intravenous insulin infusion. Stable serum immunoreactive insulin concentrations were produced, along with prompt falls in glucose, beta-hydroxybutyrate, and glucagon levels, and a steadily increasing bicarbonate level. Neither hypokalemia nor hypophosphatemia developed. Elevated serum alanine concentrations were found during ketoacidosis in contrast to the lowered concentrations found in adults, and were correlated inversely with plasma glucagon concentrations. The treatment regimen described is safe, easy to use, efficacious, and resulted in prompt correction of the observed biochemical alterations in children with diabetic ketoacidosis.

Low-dose intravenous insulin: treatment of diabetic ketoacidosis in children (suitability for use in community hospitals).

A regimen is described to simplify current methods of ketoacidosis therapy by using only standard intravenous equipment and without requiring albumin administration. After an initial intravenous insulin bolus of 2 units, a dose of up to 3 units per hour was sufficient to bring patients out of ketoacidosis. No hypoglycemia, significant hypokalemia, or rapid fall in serum osmolality was detected. Patients showed steady increases in endogenous bicarbonate and were out of metabolic acidosis in 8.4 hours. This modified regimen appears to be a safe and easy method of treating diabetic ketoacidosis in children and is suited for use in community hospitals. Since the method is described for children over the age of three or those not in severe coma, consultation should be sought for any infant and for any child in severe coma or with a significant complication, such as severe infection.

Congenital hypothyroidism: clues to an early clinical diagnosis.

Congenital hypothyroidism is clinically difficult to diagnose early in life. A review of the common signs and symptoms of this treatable disease is presented, and a case diagnosed on the third day of life is discussed. Until neonatal thyroid screening is generally available it behooves all physicians caring for newborns and young infants to have a high index of suspicion for this disease.

Kwashiorkor not associated with poverty.

Four infants are described with kwashiorkor that was unassociated with poverty or infection as predisposing factors of their disease. The condition followed various dietary regimens deficient in protein of five weeks to seven months in duration. Features of this "sugar-bady" form of kwashiorkor included a low level of physician awareness of malnutrition as a diagnostic consideration, growth retardation, edema, muscle wasting, mental changes, and mild lymphocytosis. Initially, recovery was associated with rapid loss of weight and anemia and subsequently with weight gain and return to good health.

Theophylline poisoning.

Three cases of theophylline poisoning in young children, two of which occurred after erroneous administration of commonly prescribed pediatric drugs, are presented. The possible association of hyperglycemia and ketoacidosis with severe theophylline toxicity is discussed. Serial serum theophylline levels and half-life determinations for two patients suggest that, although serum half-lives (8.5 hours and 9.0 hours) may be slightly longer than average, drug disappearance followed first order kinetics even at extremely high erum theophylline levels.