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Hepatosplenic gamma-delta (gammadelta) T-cell lymphoma is a rare but increasingly recognized lymphoid malignancy predominantly affecting young adult males. It is not well appreciated in the pediatric population. We report the third case of this aggressive lymphoma in a child as well as additional support for the consistency of the recently discovered cytogenetic abnormalities, isochromosome 7q and trisomy 8, which in this case were documented using fluorescence in situ hybridization (FISH) of a touch-preparation of the spleen.

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Children with special health care needs (CSHCN) use relatively high quantities of healthcare resources and have overall higher morbidity than the general pediatric population. Embedding clinical pharmacists into the Patient-Centered Medical Home (PCMH) to provide comprehensive medication management (CMM) through collaborative practice agreements (CPAs) for children, especially for CSHCN, can improve outcomes, enhance the experience of care for families, and reduce the cost of care. Potential network infrastructures for collaborative practice focused on CSHCN populations, common language and terminology for CMM, and clinical pharmacist workforce estimates are provided. Applying the results from the CMM in Primary Care grant, this paper outlines the following: (1) setting up collaborative practices for CMM between clinical pharmacists and pediatricians (primary care pediatricians and sub-specialties, such as pediatric clinical pharmacology); (2) proposing various models, organizational structures, design requirements, and shared electronic health record (EHR) needs; and (3) outlining consistent documentation of CMM by clinical pharmacists in CSHCN populations.

Genetic linkage studies have mapped the Huntington disease (HD) mutation to the distal region of the short arm of human chromosome 4. Analysis of recombination events in this region has produced contradictory locations for HD. One possible location is in the region distal to the D4S90 marker, which is located within 300 kilobases of the telomere. Other crossover events predict a more centromeric position for HD. Here we analyze the telomeric region of 4p in detail. Cloned DNA segments were derived from this region by utilizing a radiation-induced somatic cell hybrid as a source of DNA combined with preparative pulsed-field gel electrophoresis to enrich for the telomeric fraction. Additional DNA was obtained by using the cloned segments as multiple start points for cosmid walks. This strategy proved to be an effective method for cloning 250 kilobases of DNA in the region telomeric to D4S90. Hybridization analysis with the cloned DNA did not provide any evidence for the presence of rearrangements of 100 base pairs or greater in the DNA of individuals affected with HD. We also found no change in the size or structure of the 4p telomere in these samples.Huntington disease (HD) is a fatal neurodegenerative disorder of humans that is inherited as an autosomal dominant and highly penetrant trait (1). The disease usually begins between the ages of 30 and 45 years and progresses unremittingly for 15-20 years. Little is known about the biochemical basis of the disease. However, significant progress toward understanding HD has been achieved through genetic studies. In 1983, linkage analysis of two HD pedigrees mapped the mutation to the end of the short arm of chromosome 4, close to the polymorphic marker D4S10 (2). All subsequent families tested showed linkage to this region (3), suggesting that a single locus is mutated in all individuals affected by the disease. As HD also possesses a low mutation rate (1), the same mutation is generally believed to be present in all patients. This view is substantiated by genealogical studies that trace the disease to a single geographical origin (1) and by linkage disequilibrium studies that show that the disease probably arose from a single founder individual (4, 5). An additional feature of HD is that likely homozygotes show no more severe symptoms than heterozygotes, demonstrating that the disease is inherited in a completely dominant fashion (6).More recent meiotic linkage studies have mapped HD to the region between D4S10 and the 4p telomere (7) in a segment of DNA spanning -6000 kilobases (kb) (Fig. 1) FIG. 1. Possible locations for HD within the D4SJO-telomere region. The 6000-kb region extending from D4S10 to the 4p telomere is shown. The two likely positions for HD are indicated by the black bars; boundaries of the proximal location are presently defined by D4S10 on the centromeric side and marker A on the telomeric side, which represents the cluster of loci D4S96/D4S97/D4S115. identified, three suggest a telomeric location for the mutation (9, 11, 12). One of these c...