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An 18-yr-old 46,XY man with primary hypogonadism and a microphallus is described whose Leydig cells appear to be partially insensitive to gonadotropin action. The external genitalia were well differentiated though abnormally small. The mean +/- SE baseline plasma testosterone (T) level was 62 +/- 3.9 ng/dl, and androstenedione was 34.5 +/- 7.3 ng/dl. Plasma levels of dehydroepiandrosterone, dehydroepiandrosterone sulfate, 17-hydroxyprogesterone, 17-hydroxypregnenolone, corticosterone, deoxycorticosterone, and 17 beta-estradiol were all normal. After the im administration of hCG, plasma T increased insignificantly from 71 to 78 ng/dl, and androstenedione increased from 22 to 47 ng/dl; there was no significant change in the levels of precursor steroids. The mean +/- SE serum FSH level was 17.4 +/- 3.6 mIU/ml, and LH was 15.4 +/- 1.1 mIU/ml (normal, 5-20); both responded briskly to iv GnRH. Exogenous T therapy resulted in normal virilization, whereas therapy with hCG was ineffectual. Testicular biopsy revealed Leydig cells in normal numbers, some spermatogenesis, and thickened tubular basement membranes. In vitro binding studies using [125I]hCG were performed with testicular homogenates from the patient and three normal subjects. With 7.4 fmol labeled hCG, the specific binding (mean +/- SD), expressed as femtomoles of hCG per mg protein, was 1.16 +/- 0.44 compared to 2.49 +/- 0.41 in normal subjects (P less than 0.05). These data demonstrate partial resistance to hCG and suggest that the defect in Leydig cell function may be at the LH receptor or postreceptor level.

Recent data suggest that adolescent individuals with growth hormone (GH) deficiency have subnormal levels of adrenal androgens (AA). In order to determine the developmental pattern of AA in GH deficiency and to assess whether AA levels can help identify children with GH deficiency, we measured plasma concentrations of dehydroepiandrosterone (DHEA), DHEA sulfate (DHEA-S), Δ⁴-androstenedione (Δ⁴A), and cortisol in the basal state and during prolonged adrenocorticotropin (ACTH) infusion (8 h) in a group of 34 individuals, 26 males and 8 females, with short stature. Their chronological ages (CA) ranged from 1.75 to 17.5 years (median 10.35 years). The subjects were grouped into two categories according to the results of pituitary testing: group 1 = short, non-GH-deficient (n = 16), and group 2 = GH-deficient, ACTH-sufficient (n = 18). Patients in groups 1 and 2 had similar bone ages (BA: 7.2 ± 0.7 vs. 7.5 ± 1.0 years) and Z scores for height ( 3.0 ± 0.2 vs. – 3.2 ± 0.3 units) and height velocity (-2.5 ± 0.4 vs. -2.6 ± 0.2 units). For both groups there were significant increases from basal to peak levels for DHEA, DHEA-S, Δ⁴A and cortisol following prolonged ACTH infusion. Although both basal and peak levels of DHEA-S overlapped in groups 1 and 2 for all CA and BA, levels in group 2 tended to be lower, especially for BA greater than 10 years. Analysis of covariance showed a significant difference in the slopes of the regression lines of DHEA-S between groups 1 and 2 (basal vs. CA: F 4.9, p < 0.05; basal vs. BA: F 9.7, p < 0.005; peak vs. BA; F 8.7, p < 0.01). There were no differences noted between groups 1 and 2 for DHEA and Δ⁴ A, either in the basal state or after ACTH stimulation. Stimulated values of cortisol were similar for groups 1 and 2 (1,180 ± 40 vs. 1,300 ± 70 nmol/l). We conclude that, although differences in the overall pattern of DHEA-S between endocrinologically normal short individuals and those with GH deficiency were significant, measurements of AA in the age group studied are not helpful in the differential diagnosis of children with short stature.

Nonketotic hyperglycemic chorea-ballism (NKHCB), also known as diabetic striato-pathy (DS) by some, is a rare complication of diabetes mellitus and uncommon in children. We report a case of a 10 11/12-year-old boy of Asian descent with uncontrolled type 1 diabetes mellitus (T1DM), Hashimoto's thyroiditis, and multiple food allergies presenting with bilateral chorea-ballism. His brain magnetic resonance imaging revealed developmental venous anomaly in right parietal lobe and right cerebellum, no focal lesions or abnormal enhancements. Choreiform movements resolved with correction of hyperglycemia. Children and adolescents with a movement disorder should be evaluated for diabetes mellitus, especially with increasing prevalence and insidious nature of T2DM associated with obesity.

Severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) causing coronavirus disease 2019 pandemic in 2020 remains a major public health challenge until mass vaccination. The number of SARS-CoV-2 positive children aged 0-17 years has been increasing as older adults are vaccinated. Infected children tend to have less severe illness compared with adults, have predominantly respiratory or GI symptoms, or no symptoms. Children have an increased risk for multisystem inflammatory syndrome in children (MIS-C), which is unique. Neuropsychological complications of COVID-19 remain uncommon. Case reports and data from series exist. We report a case of tremors as sequelae of SARS-CoV-2 infection in a non-verbal adolescent with autism spectrum disorder (ASD).

Objectives To compare clinical characteristics and outcomes of infants born to COVID-19 to non COVID-19 mothers at delivery in a community hospital in Queens, New York. Methods Case-control study conducted March 15 to June 15, 2020. Cases were infants born to mothers with laboratory-confirmed COVID-19 infection at delivery. The infant of non COVID-19 mother born before and after each case were selected as controls. Results Of 695 deliveries, 62 (8.9%) infants were born to COVID-19 mothers; 124 controls were selected. Among cases, 18.3% were preterm compared to 8.1% in controls (p=0.04). In preterm cases, birth weight was not significantly different between groups. However, there was a significantly higher proportion of neonatal intensive care unit (NICU) admissions, need for respiratory support, suspected sepsis, hyperbilirubinemia, feeding intolerance and longer length of stay (LOS) in preterm cases. Among term cases, birth weight and adverse outcomes were not significantly different between cases and controls except for more feeding intolerance in cases. All infants born to COVID-19 mothers were COVID-19 negative at 24 and 48 h of life. No infants expired during birth hospitalization. Conclusions Significantly, more infants of COVID-19 mothers were premature compared to controls. Preterm cases were more likely to have adverse outcomes despite having similar birth weight and gestational age. These differences were not seen among full term infants. Health care providers should anticipate the need for NICU care when a COVID-19 mother presents in labor.

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