Joan DiMartino-Nardi

Presentation and 5-Year Follow-Up of Type 2 Diabetes mellitus in African-American and Caribbean-Hispanic Adolescents

Objective: We report the presentation and 5-year follow-up of 89 African-American (AA) and Caribbean-Hispanic (CH) youths with type 2 diabetes mellitus (T2DM) followed at the Montefiore Medical Center, Bronx, N.Y., USA, from 1990 to 2000. Methods: The medical records of 89 patients with T2DM diagnosed between 1990 and 2000 were reviewed. Results: Over a 10-year period, the number of pediatric patients less than 18 years of age diagnosed with T2DM at the Montefiore Medical Center increased tenfold. At presentation, the mean age was 14 ± 2.3 years, the mean body mass index (BMI) was 34.4 ± 9 kg/m2, the female/male ratio was 1.6:1, and all these patients were pubertal. Acanthosis nigricans was present in 89% of the patients, polyuria and polydipsia occurred in 48%, weight loss occurred in 22%, and nearly 30% of the patients were asymptomatic at diagnosis. Diabetic ketoacidosis occurred in 5 patients. By 5 years after diagnosis, 45% of the patients were able to maintain an HgbA1C <7% with oral medications (metformin and/or glipizide); 18% required insulin (<0.4 U/kg/day) in addition to oral medications, and 37% did not require any medication. The mean insulin level, BMI and HgbA1C at the time of diagnosis did not predict treatment requirements for 3 years after diagnosis. Conclusions: Because the incidence of T2DM is increasing in adolescents, the natural history and optimal therapy for adolescents with T2DM need to be established.

Bayley-Pinneau method of height prediction in girls with central precocious puberty: Correlation with adult height☆☆☆★

One of the sequelae of idiopathic central precocious puberty (ICPP) can be short adult stature. In this retrospective study adult height was normal in 90% of girls with untreated ICPP (mean, 161.4 +/- 7.7 cm). The height prediction made at the time of initial examination and the height age correlated with adult height. Therefore the initial height prediction can be useful in identifying those girls with ICPP at risk for short stature.

Transient congenital hypoparathyroidism: Resolution and recurrence in chromosome 22q11 deletion

Transient congenital hypoparathyroidism (TCHP), with spontaneous resolution in infancy and subsequent recurrence in childhood, has not been associated with a specific cause. We report three patients with TCHP, initially with severe but transient neonatal hypocalcemia. During childhood, recurrence of hypoparathyroidism and recognition of phenotypic features suggested a diagnosis of velocardiofacial syndrome (VCFS). Features specific for the DiGeorge syndrome, with known clinical and genetic overlap with VCFS, were not present except for hypoparathyroidism. Genetic analysis confirmed chromosome 22q11 deletion in each patient. TCHP may be the earliest specific finding in 22q11 deletion/VCFS subgroup, with other diagnostic features emerging in later childhood. Infants with resolved TCHP need continued observation of parathyroid sufficiency, genetic analysis, and examination for anomalies associated with chromosome 22q11 deletion.

Three-year follow-up of borderline congenital hypothyroidism

The purpose of this study was to determine whether children with borderline hypothyroidism in the neonatal period had persistent hypothyroidism after 3 years of levothyroxine replacement therapy. Fourteen term infants with slightly abnormal newborn screening results (thyroxine <10th percentile, thyroid stimulating hormone ¿TSH <40 microU/mL) were identified. The subsequent serum confirmatory TSH results of 12 subjects were modestly elevated (5.3 to 18.8 microU/mL, normal 0.6 to 4.6), whereas 2 subjects who had borderline confirmatory TSH (4.6 and 4.7 microU/mL) had abnormal TSH responses to thyrotropin releasing hormone testing. After 3 years of therapy, levothyroxine was discontinued in 13 patients, and repeat thyroid function tests were obtained 1 month later. Levothyroxine was not discontinued in one patient because he had an elevated random TSH (10 microU/mL) while receiving therapy. At 3 years of age, 13 patients had persistently abnormal thyroid function tests (TSH >4.6 microU/mL or a thyroid releasing hormone test result consistent with primary hypothyroidism), and levothyroxine was reinitiated. Only one patient had normal thyroid function studies. Although prospective studies are still lacking, we recommend levothyroxine replacement in newborns with borderline hypothyroidism.

Relationship between adiponectin and ambulatory blood pressure in obese adolescents.

Obesity is associated with elevated blood pressure (BP), insulin resistance, and altered plasma adiponectin levels; the relationship between the biochemical features of obesity and 24-h ambulatory blood pressure (24-h ABP) parameters in adolescents remains unknown. Anthropometric measurements and 24-h ABP monitoring were obtained on 41 obese adolescents with and without type 2 diabetes mellitus (T2DM). Serum adiponectin, high sensitivity C-reactive protein (hs-CRP), lipid profile, insulin, fasting glucose, liver enzymes, Hb A1c (HbA1c), and two random urine samples were obtained for creatinine and microalbumin measurements. The determinants of 24-h systolic (SBP) and diastolic (DBP) BP were examined using multivariate linear regression models with BP parameters as outcome variables. Forty-one obese adolescents were studied. Adiponectin levels were reduced and hs-CRP levels were elevated, and were inversely and significantly correlated (rho = −0.3, p = 0.05). ABP showed blunted nocturnal SBP dipping. Twenty-four hour SBP and DBP indexes were significantly (p < 0.05) and inversely correlated with adiponectin (rho = −0.4 and −0.42), respectively. In multivariate models, lower adiponectin level was independently associated with 24-h SBP and DBP. Adiponectin inversely correlate with ABP parameters in obese adolescents. Larger studies are needed to examine the relationship between adiponectin and mechanisms of BP regulation.

Pseudotumor cerebri in an infant after l -thyroxine therapy for transient neonatal hypothyroidism

Pseudotumor cerebri is generally a benign disorder. It has been reported to occur in hypothyroidism, particularly after the initiation of L-thyroxine replacement therapy. Previous case reports have involved children primarily in the peripubertal age range (approximately 8 to 13 years). We report here the development of pseudotumor cerebri in an infant who required treatment with L-thyroxine for transient neonatal hypothyroidism as a result of maternal thyroid-stimulating hormone receptor-blocking antibodies.

Carbohydrate metabolism in children receiving growth hormone for 5 years

Carbohydrate metabolism was evaluated by fasting and postprandial glucose, insulin, and hemoglobin (Hb)A1c levels in children with chronic renal insufficiency and various other growth disorders treated with growth hormone. Mean fasting and postprandial glucose remained unchanged throughout the 5-year study period in all four study groups. Median fasting insulin levels rose from lownormal levels into the normal range after 5 years of growth hormone. Average fasting insulin level after 5 years was 10 mU/l. Median postprandial insulin values also rose, yet remained within the normal range at the 5-year mark. Mean Hb A1c levels remained within low to middle end of the normal range in the patients with growth hormone deficiency, Turner syndrome, and idiopathic short stature. Mean Hb A1c levels at the 5 years were slightly elevated to 6.3% for the patients with chronic renal insufficiency.

Assessing Insulin Resistance: Application of a Fasting Glucose to Insulin Ratio in Growth Hormone-Treated Children

Background: Insulin resistance (IR) is an important risk factor for cardiovascular disease and type-2 diabetes mellitus. Therefore simple measures of IR have been proposed to screen the at-risk patient. A fasting serum glucose (mg/dl) to plasma insulin (µU/ml) ratio (FGIR) of <7 was recently suggested as a screening tool for IR in certain pediatric patients. Methods: To determine the utility of simple indicators of IR, the FGIR of <7 was applied to a group of patients with established risk for IR. The study group was comprised of non-growth hormone (GH)-deficient patients with Turner syndrome (TS, n = 92) and idiopathic short stature (ISS, n = 73) receiving GH. The occurrence of a FGIR of <7 in these cohorts was compared to data from previous publications. Results/Conclusions: The application of a FGIR of <7 confirmed a rise in IR with GH therapy in both groups as well as a higher occurrence in the TS group, rising from 22 to 48% between 12 and 24 months of GH therapy. We conclude that simple measures of IR such as the FGIR may be useful in screening and following patients at risk for IR.

Lack of clinical evidence of sodium retention in children with idiopathic short stature treated with recombinant growth hormone.

In adults patients, administration of human growth hormone and growth hormone synthesized by recombinant DNA technology (rGH) results in sodium and fluid retention and weight gain. This study was performed to determine whether rGH administration in children with idiopathic short stature (ISS) caused any clinical evidence of sodium retention. The parameters assessed included blood pressure, height, weight, plasma renin activity (PRA), aldosterone, and atrial natriuretic peptide (ANP). These were measured in nine treated children after 0, 3, 6, 9, and 12 months of growth hormone therapy; seven untreated children served as controls. After 12 months, the treated children had no significant increases in measurements of blood pressure, PRA, aldosterone, and ANP. Although treated children gained more weight than control patients, they also grew faster. Therefore, there was no significant difference in weight for height percentile for treated children when compared with normal controls. After 1 year of therapy, the administration of rGH to children with ISS does not result in any clinically significant evidence of sodium retention.

The Frequency of Elevated Blood Pressure in Obese Minority Youth

In this study, 167 obese persons were recruited (45 African Americans, 122 Caribbean Hispanic persons), with a mean age of 14.6±2.1 years, a mean body mass index (BMI) of 38±7.5 kg/m2, and mean BMI Z‐score of 2.47±0.36; 31 nonobese youth were recruited as controls (7 African Americans, 24 Caribbean Hispanic persons), with a mean age of 14.6±2.1 years, a mean BMI of 20±2.8 kg/m2, and a mean BMI Z‐score of −0.08±0.87. The objective was to assess the frequency of elevated blood pressure in obese minority youth. Weight, height, blood pressure (BP), and various biochemical markers were measured in each participant. Overall, 31% of the obese patients had elevated BP, compared with 3% of the control participants. Obese persons with elevated BP had significantly higher BMI, BMI Z‐scores, and hemoglobin A1c levels. The frequency of elevated BP and the degree of systolic BP elevation increased with increasing BMI Z‐score. Elevated BP was 10 times more frequent in obese minority youth, emphasizing the importance of screening for hypertension in this high‐risk population.