Faculty Bibliography

Osteomyelitis represents inflammation and infection of bone tissue by a pathogen. Acute osteomyelitis is more likely to be unifocal compared to a chronic process which tends to be multifocal and recurrent. Early diagnosis, aggressive appropriate antibiotic therapy and a multidisciplinary approach are essential for a satisfactory prognosis and improved outcome. We report an atypical case of acute multifocal methicillin-sensitive Staphylococcus aureus (MSSA) osteomyelitis.

BACKGROUND: Newborn screening (NBS) program is a simple and inexpensive method for early detection and treatment of over forty conditions as of 2005. Most cases of congenital hypothyroidism (CH) are sporadic and occurs in 1 in 3500 live births. Congenital adrenal hyperplasia (CAH) is a group of autosomal recessive disorders that occurs in 1 in 1600 live births. The detection of CH is determined by either low T4 and/or elevated TSH and the detection of CAH is determined by elevated 17OH progesterone on Guthrie filter paper. Infants who meet the above criteria on NBS for either condition must undergo confirmatory testing. OBJECTIVE: Is the incidence of CH and CAH at FHMC, a community hospital in northern Queens, New York serving a multiethnic population different from theincidence of CH and CAH in New York State (NYS)? DESIGN/METHODS: We reviewed records of the infants recalled and confirmed to have CH and CAH for 2000, 2001 and 2004 and compared with reported incidence of the same conditions in NYS. RESULTS: The total number of live births in NYS was 760,112 and in FHMC 6348 for the three years studied, accounting for 0.7 to 0.8% of total NYS live births. The population served by FHMC included 43% White, 35% Asian, 16% Hispanic, 3% Afro-American and 3% other. A total of 5 patients were identified to have CH in the three years studied, accounting for an incidence of 0.12 (p > 0.05). Three patients were identified to have CAH in 2004 or an incidence of 1.4 (p < 0.05). [Table 1]. CONCLUSIONS: The incidence rate of CH was similar to that of NYS. However, the incidence rate of CAH at FHMC was greater than that reported by NYS. This difference in incidence rate for CAH may be related to the ethnic composition of the population that FHMC serves. Pediatricians and pediatric endocrinologists must be cognizant of the signs and symptoms of CAH in certain populations known to have higher incidence for this condition. Further follow up of incidence rate for CAH at FHMC is indicated since CAH was added to the NBS in 2003

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), delta 4-androstenedione (delta 4A), 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, delta 4A 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.(ABSTRACT TRUNCATED AT 250 WORDS)

Eight patients with beta-thalassemia who were given long-term treatment with combined multiple transfusions and chelation therapy underwent adrenal testing. The six male and two female patients ranged in age from 7 to 19 years. Six of eight patients had delayed bone ages and height greater than 2.5 SDs below the mean. Of the six patients more than 13 years of age, two had clinical evidence of isolated adrenarche and only one had evidence of true puberty. Cortisol levels were similar in patients and controls at zero time (10.6 +/- 1.8 micrograms/dL [292 +/- 50 nmol/L] vs 10.8 +/- 1.4 micrograms/dL [298 +/- 39 nmol/L]) and at 60 minutes (26.6 +/- 2.5 micrograms/dL [734 +/- 69 nmol/L] vs 24.9 +/- 1.9 micrograms/dL [687 +/- 52 nmol/L]) after insulin hypoglycemia (all values are the mean +/- SE). During an eight-hour infusion of ACTH, cortisol responses in the patients with thalassemia were not significantly different from those of controls. Baseline levels of the adrenal androgens dehydroepiandrosterone (DHEA) and dehydroepiandrosterone sulfate (DHEA-S) were significantly lower in the subjects with thalassemia compared with controls of similar bone age and pubertal status. The prolonged ACTH infusion caused a significant increase in the DHEA level (79.2 +/- 14.7 ng/dL [2.74 +/- 0.51 nmol/L] vs 538.6 +/- 38.1 ng/dL [18.67 +/- 4.79 nmol/L]) and the DHEA-S level (37.5 +/- 10.8 micrograms/dL [1.02 +/- 0.29 mumol/L] vs 70.5 +/- 18.3 micrograms/dL [1.19 +/- 0.50 mumol/L]) in the patients. The patients' peak stimulated levels of DHEA-S were significantly lower than those of the controls, whereas peak levels of DHEA were similar in the patients and the controls. These results indicate that combined multiple transfusions and chelation therapy preserve the integrity of the ACTH-cortisol axis in patients with thalassemia. The reduced levels of adrenal androgens, short stature, and delayed puberty noted in our patients suggest, however, that alternative approaches to the therapy of thalassemia are needed