Brucellosis: A Rare Cause of Febrile Neutropenia in a Child.

We report a case of brucellosis-induced severe neutropenia in a 2-year-old girl who presented with a 2-week history of fever. On clinical examination, the patient was febrile with mild aphthous stomatitis. However, her general condition was stable, and systemic examination did not show involvement of any other organ. Laboratory test results revealed severe neutropenia, mild anemia, and an elevated serum C-reactive protein level. Flow cytometry of peripheral blood leukocytes revealed no malignancy, and blood film morphology was unremarkable except for mild microcytosis and hypochromia. Antineutrophil antibody and Coombs test results were negative. We administered intravenous cefuroxime; however, therapy was switched to meropenem plus clarithromycin because fever persisted for 5 days, despite treatment. On the 10th day after admission, Brucella serology tests showed positive results, and trimethoprim-sulfamethoxazole plus rifampicin therapy was prescribed for 8 weeks. The fever defervesced, and the child was discharged in a good state of health. Neutropenia persisted for several months but gradually resolved. Neutropenia, defined as an absolute neutrophil count (ANC) < 1.5 cells × 10 9 /L beyond the first year of life, is a benign transient condition associated with an intercurrent infection (usually viral illnesses or infections) in immunocompetent children. However, severe neutropenia (ANC < 0.5 × 10 9 /L) associated with fever necessitates hospitalization and administration of broad-spectrum antibiotics to avoid the high risk of sepsis, particularly in children. Brucellosis is rarely associated with hematologic abnormalities such as neutropenia. Early diagnosis of hematologic complications of brucellosis is essential for prompt initiation of specific and aggressive treatment.

Lack of telomere shortening with age in mouse resting zone chondrocytes.

BACKGROUND AND AIM: Telomeres are hexameric repeat sequences that flank eukaryotic chromosomes. The telomere hypothesis of cellular aging proposes that replication of normal somatic cells leads to progressive telomere shortening which induces replicative senescence. Previous studies suggest that growth plate chondrocytes have a finite proliferative capacity in vivo. We therefore hypothesized that telomere shortening in resting zone chondrocytes leads to replicative senescence. METHOD: To test this hypothesis we compared the telomere restriction fragment (TRF) length of Mus casteneus at 1, 4, 8, and 56 weeks of age. RESULTS AND CONCLUSIONS: We found that TRF length did not diminish measurably with age, suggesting that telomere shortening in resting zone chondrocytes is not the mechanism that limits proliferation of growth plate chondrocytes in vivo.

Short stature with normal growth hormone stimulation testing: lack of evidence for partial growth hormone deficiency or insensitivity.

OBJECTIVES: To test the hypothesis that children with short stature and peak stimulated GH (pGH) of 7-10 microg/l have partial GH deficiency and to test the hypothesis that short children with normal pGH but low IGF-I levels have partial GH deficiency or partial GH insensitivity. DESIGN AND PATIENTS: Retrospective analysis of the clinical and biochemical profiles of 76 children who underwent an evaluation for short stature (height < 5th percentile) that included two, sex steroid-primed GH stimulation tests. RESULTS: Patients with pGH < 7 microg/l (n = 14) differed significantly from those with pGH > 7 microg/l (n = 62), having greater midparental height (MPH) SDS, a greater disparity between height SDS and MPH SDS, and lower IGF-I SDS. Patients with pGH of 7-10 microg/l (n = 12) did not have characteristics intermediate between those with pGH < 7 microg/l and those with pGH > or = 10 microg/l, but instead resembled those with pGH > or = 10 microg/l. Patients with pGH > or = 7 microg/l, but low IGF-I (< -2 SDS) (n = 5), did not show characteristics intermediate between those with pGH < 7 microg/l and those with pGH > or = 7 microg/l and normal IGF-I. CONCLUSIONS: These data do not support either the hypothesis that children with pGH of 7-10 microg/l have partial GH deficiency or the hypothesis that children with normal pGH but subnormal IGF-I levels have partial GH deficiency or insensitivity.

No difference in pubertal growth and final height between treated hypogonadal and non-hypogonadal thalassemic patients.

BACKGROUND: Many factors can negatively affect growth in thalassemic patients, and hypogonadism has been considered as the main factor responsible for their pubertal growth failure. OBJECTIVE: To evaluate the influence of hypogonadism and its treatment on pubertal growth and final height in thalassemic patients. METHODS: We compared the growth of 28 hypogonadal thalassemic patients in whom puberty was induced to that of 25 patients in whom puberty occurred spontaneously. RESULTS: In both groups of patients we observed reduced peak height velocity (induced puberty: females 4.9 +/- 2.1, males 6.0 +/- 1.8 cm/year; spontaneous puberty: females 6.1 +/- 1.5, males 7.3 +/- 2.1 cm/year) and pubertal height gain (induced puberty: females 11.3 +/- 4.0, males 18.0 +/- 4.5 cm/year; spontaneous puberty: females 15.8 +/- 2.7, males 18.1 +/- 5.3 cm/year) and a short final height (induced puberty: females -1.8 +/- 0.7, males -2.1 +/- 1.0 SDS; spontaneous puberty: females -2.3 +/- 1.0, males -1.9 +/- 1.0 SDS). CONCLUSIONS: Poor pubertal growth is present in thalassemic patients regardless of hypogonadism. Other factors are responsible for the reduced growth spurt and the final short stature observed in these patients.

In boys with abnormal developmental tempo, maturation of the skeleton and the hypothalamic-pituitary-gonadal axis remains synchronous.

The primary mechanism that initiates puberty is unknown. One possible clue is that pubertal maturation often parallels skeletal maturation. Conditions that delay skeletal maturation also tend to delay the onset of puberty, whereas conditions that accelerate skeletal maturation tend to hasten the onset of puberty. To examine this relationship, we studied boys with congenital adrenal hyperplasia (n = 13) and familial male-limited precocious puberty (n = 22), two conditions that accelerate maturational tempo, and boys with idiopathic short stature (n = 18) in which maturational tempo is sometimes delayed. In all three conditions, the onset of central puberty generally occurred at an abnormal chronological age but a normal bone age. Boys with the greatest skeletal advancement began central puberty at the earliest age, whereas boys with the greatest skeletal delay began puberty at the latest age. Furthermore, the magnitude of the skeletal advancement or delay matched the magnitude of the pubertal advancement or delay. This synchrony between skeletal maturation and hypothalamic-pituitary-gonadal axis maturation was observed among patients within each condition and also between conditions. In contrast, the maturation of the hypothalamic-pituitary-gonadal axis did not remain synchronous with other maturational processes including weight, height, or body mass index. We conclude that in boys with abnormal developmental tempo, maturation of the skeleton and the hypothalamic-pituitary-gonadal axis remains synchronous. This synchrony is consistent with the hypothesis that in boys, skeletal maturation influences hypothalamic-pituitary-gonadal axis maturation.