Clinical, histopathologic, and genetic features of pediatric primary myelofibrosis--an entity different from adults.

Primary myelofibrosis is a chronic myeloproliferative neoplasm characterized by cytopenias, leukoerythroblastosis, extramedullary hematopoiesis, hepatosplenomegaly and bone marrow fibrosis. Primary myelofibrosis is a rare disorder in adults; children are even less commonly affected by this entity, with the largest pediatric case series reporting on three patients. Most literature suggests spontaneous resolution of myelofibrosis without long term complications in the majority of affected children. We describe the clinical, pathologic, and molecular characteristics and outcomes of nineteen children with primary myelofibrosis treated in our center from 1984 to 2011. Most patients had cytopenia significant enough to require supportive therapy. No child developed malignant transformation and only five of the 19 children (26%) had spontaneous resolution of disease. Sequence analyses for JAK2V617F and MPLW515L mutations were performed on bone marrow samples from 17 and six patients, respectively, and the results were negative. In conclusion, analysis of this large series of pediatric patients with primary myelofibrosis demonstrates distinct clinical, hematologic, bone marrow, and molecular features from adult patients.

Clinical spectrum of acute otitis media complicating upper respiratory tract viral infection.

BACKGROUND: acute otitis media (AOM) often occurs as a complication of upper respiratory tract infection (URI). OBJECTIVE: to describe otoscopic findings during URI, the full clinical spectrum of AOM, and outcome of cases managed with watchful waiting. METHODS: : In a prospective study of 294 healthy children (6 months-3 years), characteristics of AOM complicating URI were studied. Otoscopic findings were categorized by tympanic membrane (TM) position, color, translucency, and mobility. Otoscopic score was assigned based on McCormick otoscopy scale (OS)-8 scale. RESULTS: during days 1 to 7 of URI, otoscopic findings at 1114 visits were consistent with AOM in 22%; myringitis (inflamed TM, no fluid) was diagnosed in 7%. In AOM episodes diagnosed within 28 days of URI onset, TM position was described as: nonbulging (19%), mild bulging (45%), bulging (29%), and TM perforation occurred in (6%). OS-8 scale showed mild TM inflammation (OS, 2-3) in 6%, moderate (OS, 4-5) in 59%, and severe (OS, 6-8) in 35%. In 54% of 126 bilateral AOM episodes, inflammation of both TMs was at different stages. Of 28 cases of nonsevere AOM managed with watchful waiting, 4 progressed and 3 later required an antibiotic. CONCLUSIONS: AOM is a spectrum of infection that may present at various stages, even in the same child with bilateral disease. During URI, otoscopic changes are observed from the first day of onset. Understanding the wide clinical spectrum of AOM is needed to help with future clinical trial design and development of a scoring system to establish treatment criteria that will minimize antibiotic use.

Effect of incubation temperature and androgens on dopaminergic activity in the leopard gecko, Eublepharis macularius.

Male leopard geckos that hatch from eggs incubated at a female-biased temperature (Tf) behave differently when compared with males hatching at a temperature which produces a male-biased sex ratio (Tm). We investigated the effect of incubation temperature and androgen implantation on aspects of the dopaminergic system of Tf and Tm males. Our data suggest that more dopamine (DA) is stored in the nucleus accumbens of naive Tf males compared with naïve Tm males when they encounter a receptive female conspecific across a barrier. No difference was measured in the preoptic area and the ventral tegmental area (VTA). This difference in intracellular DA levels in a motivation-related brain nucleus might be correlated with differences in sociosexual behavior observed between the two morphs. There were no differences in tyrosine hydroxylase (TH) expressing cell numbers in the VTA of cholesterol (CH)-implanted naive castrated Tf and Tm males. Only Tf males implanted with testosterone had significantly higher TH immunopositive cell numbers in the VTA compared with CH- and dihydrotestosterone-implanted Tf males. These data indicate that both the embryonic environment as well as the circulating hormonal milieu can modulate neurochemistry, which might in turn be a basis for individual variation in behavior.