Robert Rapaport

Prediction of neonatal hyperthyroidism in infants born to mothers with Graves disease.

OBJECTIVEnTo determine whether determinations of thyrotropin-receptor antibody (TRAb) levels in newborn infants of women with Graves disease would predict which infants will have hyperthyroidism.nnnMETHODSnThe TRAb levels, assayed in the sera of 14 infants born to 14 women with Graves disease, were measured sequentially in the infants with hyperthyroidism during the course of antithyroid medication therapy.nnnRESULTSnSeven infants had TRAb values less than 0.15 and remained euthyroid. In seven infants whose initial TRAb values were more than 0.25 (range, 0.48 to 0.88), clinical and biochemical signs of hyperthyroidism developed. The infants were treated with antithyroid medication until day 57 to day 123 of life. Therapy was discontinued when the infants were free of symptoms and when serum thyroxine and triiodothyronine and free thyroxine levels remained normal during therapy with decreasing doses of antithyroid medication. When the medication was discontinued, TRAb values were less than 0.20.nnnCONCLUSIONSnInfants born to mothers with Graves disease with initial TRAb values less than 0.15 remained euthyroid. The TRAb values greater than 0.25 were associated with the development of neonatal hyperthyroidism. During treatment of neonatal hyperthyroidism, TRAb values less than 0.20 may be helpful in deciding when to withdraw antithyroid medication.

Suppression of immune function in growth hormone-deficient children during treatment with human growth hormone*

Inasmuch as growth hormone is known to interact with the immune system, we studied immune functions including immunoglobulins, cell surface markers, mitogen responses, and polymorphonuclear cell function in eight children with growth hormone deficiency, ages 1 to 17 years, before and during treatment with human growth hormone for 12 to 16 months. Before treatment immune functions were normal in all children. Treatment with human growth hormone did not significantly affect serum immunoglobulins, polymorphonuclear cell function, or percent T cells. However, percent B cells decreased to subnormal levels in seven of seven patients. T helper/suppressor ratios decreased in all patients, to subnormal values in seven of eight patients; and mitogen responses decreased to below normal in all. The decline of percent B cells was transient in all patients, of T helper/suppressor ratios in seven of eight, and mitogen responses in five of eight patients. In vitro incubation of lymphocytes with growth hormone resulted in no changes in cell surface markers or mitogen responses. Although the depression of immune functions resulted in no increased rate of infections during the observation period, we do not know the possible effects of prolonged treatment and therefore caution against the indiscriminate use of human growth hormone. The effects of biosynthetically obtained growth hormone on immune function remain to be determined.

Vitamin D Deficiency Rickets: Reports of Its Demise Are Exaggerated

ciency is unusual in our society. The few exceptions are mainly food faddists, persons who are confined to their homes or have a malabsorption syndrome, and those receiving drugs... &dquo;6 It appears, however, that, to paraphrase Mark Twain, the demise of vitamin D deficiency rickets is greatly exaggerated. In a recent 3year period we diagnosed vitamin D deficiency rickets in seven black infants. We believe, therefore, that it is necessary to reemphasize the continued existence of this totally preventable disease, even in the United States in the 1990s.

Effects of human growth hormone on immune functions: In vitro studies on cells of normal and growth hormone-deficient children

We have studied the in vitro effects of human growth hormone on cell surface markers and mitogenic responses of peripheral blood lymphocytes (PBL) of normal and growth hormone-deficient children before, during and after treatment with growth hormone. Growth hormone resulted in a decrease in B cell expression but it did not affect expression of T cell subsets. Growth hormone depressed the proliferation of PBL of normal and untreated growth hormone-deficient children. The proliferative responses to phytohemagglutinin (PHA) versus PHA with growth hormone were not statistically different, though the responses of most normal and on treatment children were diminished by the addition of growth hormone. PBL derived from growth hormone-deficient children during treatment with human growth hormone exhibited significantly greater spontaneous proliferation then the PBL of normal children. Growth hormone further significantly enhanced their proliferation. PHA and PHA with growth hormone resulted in significantly greater proliferation of these patients PBL when compared to those of normal children. We demonstrated that human growth hormone had substantial in vitro effects on immune functions. These effects, some of which depend on the treatment status of the children, may need to be considered in the clinical use of human growth hormone.

Late rise of thyroid stimulating hormone in ill newborns.

OBJECTIVESnTo determine the frequency and characteristics of late rise of thyroid stimulating hormone (LRT) among ill newborns.nnnINFANTS AND METHODSnData were retrospectively analyzed from infants in intensive care settings with abnormal thyroid tests over 13 months. Thyroid tests were performed by filter paper if neonatal intensive care >4 weeks or serum if clinically indicated. LRT was defined as thyroid stimulating hormone (TSH) >10 microIU/ml after normal TSH on initial newborn screen.nnnRESULTSnLRT was identified in 13 infants. Of 736 admissions to the neonatal intensive care unit (NICU), 10 (1.4%) had LRT. Excluding 3/10 with diagnosis at <1 week of age the frequency is 0.95%. Three additional cases occurred in other ICUs. TSH elevation resolved in 6/13 (group A, TSH 10.6-20.6 microIU/ml) and persisted in 7/13 necessitating treatment (group B, TSH 10.5-1326 microIU/ml). 7/13 had birth weights <1500 g. 11/13 had gestational ages <37 weeks. LRT was associated with surgery, sepsis workup, dopamine, and gastrointestinal disorders.nnnCONCLUSIONSnLRT was not infrequent in ill newborns. Most were premature and half were not very low birth weight. We recommend monitoring of thyroid function by serum specimen in ill newborns with prolonged ICU care regardless of birth weight.

Relationship of growth hormone deficiency and leukemia

The use of growth hormone (GH) has been implicated as a possible risk factor for leukemia. We present data from six patients that support a working hypothesis that an increased risk of leukemia may exist in patients with GH deficiency not related to exogenous use of GH.

Type 1 and type 2 diabetes mellitus in childhood in the United States: practice patterns by pediatric endocrinologists.

AIMnTo determine the relative frequency of type 2 diabetes mellitus (DM) in the US, and to assess diabetes practice patterns in the US.nnnMETHODnA questionnaire regarding pediatric diabetes practice patterns was distributed to the members of the Lawson Wilkins Pediatric Endocrine Society in 1999. Only one member of each practice group was requested to respond. Responses received through early 2000 were analyzed.nnnRESULTSnOne hundred and twenty-six practices representing 45% of the members of the Society responded. 11.9% of pediatric patients with DM were considered to have type 2 DM. On average 53 new patients with DM were seen each year. The average practice consisted of 2.5 physicians, 1.5 nurse educators, 1.3 dieticians, 1.0 social workers and 0.5 nurse practitioners. Management practices comply by and large with the recommendations of the American Diabetes Association and reflect a trend toward more intensive treatment and monitoring.nnnCONCLUSIONnType 2 DM was seen in 11.9% of patients. Most diabetes practices in the US utilize a team approach to the management of youth with DM.

New-Onset IDDM Presenting With Diabetic Ketoacidosis in a Pregnant Adolescent

OBJECTIVE To describe the presentation of insulin-dependent diabetes mellitus(IDDM) as ketoacidosis during pregnancy in a teenager. CASE A 14-year-old pregnant girl presented with ketoacidosis (bicarbonate 14 nM, 14 meq/l, pH 7.27, glucose 67 mM, 1,208 mg/dl) during the last month of pregnancy with a fetal demise. Two years of follow-up has confirmed that she has IDDM. CONCLUSIONS Diabetes presenting in pregnant adolescents is likely due to IDDM. Immediate insulin therapy and proper education about managing diabetes should be initiated to hopefully prevent the outcome described in this patient.

Primary Empty Sella Syndrome in Childhood: Association with Precocious Puberty

Primary empty sella syndrome has been considered an infrequent finding in childhood. One hundred and twelve cases have been reported in children ages 0.7 to 18 years. The frequency of primary empty sella ranged from 1% to 58%. Endocrine abnormalities were described in nearly all , children while visual abnormalities were noted in only 6%. Growth hormone deficiency was the single most common hormonal dysfunction noted in children with primary empty sella. Precocious puberty has been rarely reported in association with primary empty sella. We report the case of a 7-1/2 year old girl with gonadotropin dependent central precocious puberty and a partially empty sella who had no other hypothalamic-pituitary dysfunction. We suggest that pubertal abnormalities be included among the endocrine disorders potentially associated with the primary empty sella syndrome.

NON-INSULIN DEPENDENT DIABETES MELLITUS IN A PREPUBERTAL CHILD WITH PRADER-WILLI SYNDROME

Diabetes mellitus has only rarely been reported in prepubertal children with Prader-Willi syndrome. All reported children have required insulin therapy. We report the development of a previously unrecognized association of non-insulin dependent diabetes mellitus in an obese 6 year-old child with Prader-Willi syndrome. She has never developed ketosis or acidosis, and she has been treated with oral hypoglycemic medication.