Poverty and health. Prospective evidence from the Alameda County Study

To examine the reasons for the association between socioeconomic status and poor health, the authors examined the nine-year mortality experience of a random sample of residents aged 35 and over in Oakland, California. Residents of a federally designated poverty area experienced higher age-, race-, and sex-adjusted mortality over the follow-up period compared with residents of nonpoverty areas (relative risk = 1.71, 95 per cent confidence interval 1.20-2.44). This increased risk of death persisted when there was multivariate adjustment for baseline health status, race, income, employment status, access to medical care, health insurance coverage, smoking, alcohol consumption, physical activity, body mass index, sleep patterns, social isolation, marital status, depression, and personal uncertainty. These results support the hypothesis that properties of the sociophysical environment may be important contributors to the association between low socioeconomic status and excess mortality, and that this contribution is independent of individual behaviors.     

Causes of B chromosome variant substitution in the grasshopper Eyprepocnemis plorans

We have analysed B chromosome frequency for three consecutive years, B transmission rate at population and individual levels, clutch size, egg fertility and embryo–adult viability in a natural population of the grasshopper Eyprepocnemis plorans containing two different B chromosome variants, i.e. B₂ and B₂₄, the second being derived from the first and having replaced it in nearby populations. From 2002 to 2003 the relative frequency of both variants changed, although the differences did not reach significance. A mother–offspring analysis showed no significant effect of any of the two B variants on clutch size, egg fertility or embryo–adult viability, but B₂₄ was more efficiently transmitted than B₂ through males from the 2002 season, which explains the observed frequency change. Controlled crosses, at individual level, showed significant drive through some females for B₂₄ but not for B₂, suggesting that this difference in transmission rate might also be important for the substitution process. The analysis of relative fitness for B₂ and B₂₄ carriers for all fitness components, as a whole, showed a significantly better performance of B₂₄-carrying individuals, suggesting that the cumulative effect of these slight differences might contribute to the replacement of B₂ by B₂₄. Electronic Supplementary Material Supplementary material is available for this article at .     

Occurrence of immunoblastic B-cell lymphoma in hairy cell leukemia

A 47-year-old man, was referred for evaluation of asymptomatic splenomegaly in September 1981, and a diagnosis of hairy cell leukemia (HCL) at the initial clinical stage was made. The patient remained asymptomatic until May 1985, when splenectomy was performed because of anemia and splenomegaly. Bone marrow and liver biopsy specimens showed diffuse infiltration by abnormal tartase resistant acid phosphatase (TRAP) positive lymphocytes with typical aspect of hairy cells. Four months later, he developed fever of unknown origin and, at laparotomy, diffuse retroperitoneal lymph node enlargement and metastatic liver nodules were seen. Lymph node and liver biopsy specimens showed diffuse infiltration by abnormal large lymphocytes, which bore monoclonal surface immunoglobulin M and light chain kappa. Only six cases of non-Hodgkin's lymphoma associated with HCL have been published to date. This report describes an additional case of immunoblastic B-cell lymphoma, preceded 4 years earlier by the diagnosis of HCL.     

The Peutz-Jeghers syndrome. Case reports.

Two additional cases of Peutz-Jeghers syndrome are described. One of them, a 19 year-old female, is a sporadic case, whereas in the other case, also a 19 year-old female, there are two members of the family with the Peutz-Jeghers syndrome. A review of some salient features of this entity is made. These include clinical presentation, histopathological features, malignant potential and treatment.     

Calciphylaxis – a topical overview

'Calciphylaxis', a calcification syndrome associated with ischaemic cutaneous necrosis, is acquired naturally in humans in disease states. It is a life and limb-threatening complication, usually observed in patients with renal disease and secondary hyperparathyroidism, but known to occur in the absence of renal or parathyroid disease. The reported mortality rate, which ranges from 60-80%, relates to wound infection, sepsis and organ failure. It is a small-vessel vasculopathy, which is estimated to occur in about 4% of haemodialysis patients. Clinically, violaceous, reticulate areas of cutaneous necrosis and eschar may be evident, particularly in the extremities. In addition to the clinical picture, a raised calcium phosphorous product, an elevated parathyroid hormone level, radiographic evidence of vessel and soft-tissue calcification and the finding of mural calcification affecting small arteries and arterioles on histopathology help to confirm the diagnosis of this entity which generally has a poor prognosis. A high index of suspicion and an active multidisciplinary management approach, with rigorous attention to wound care and prevention of sepsis, are vital in the management of these patients. In this overview, we discuss the pathophysiology, clinical features and associations, risk factors, diagnosis and management issues relating to calciphylaxis.     

Rapp-Hodgkin Syndrome with Pili Canaliculi

Abstract: A 20-year-old woman and her 12-year-old brother had hypohidrotic ectodermal dysplasia, cleft lip and palate, midfacial hypoplasia with narrow nose from the nasal bridge to the tip, narrow dysplastic nails, and conical teeth and hypodontia, and hypospadias and hypoplastic uvula in the boy. The woman had major underdevelopment of intellectual capacity. The most important hair anomalies in both siblings were sparse eyebrows, pili torti, and pili canaliculi. Some of the pili canaliculi had two canals (pili bicanaliculi), and the cross section for scanning electron microscopy had a quadrangular aspect. This is the seventh family reported with Rapp-Hodgkin ectodermal dysplasia.     

Mutations in the retinal guanylate cyclase (RETGC-1) gene in dominant cone-rod dystrophy

The dominant cone-rod dystrophy gene CORD6 has previously been mapped to within an 8 cM interval on chromosome 17p12-p13. The retinal- specific guanylate cyclase gene (RETGC-1), which maps to within this genetic interval and previously was implicated in Leber's congenital amaurosis, was screened for mutations within this family and in a panel of small families and individuals with various cone and cone- rod dystrophy phenotypes. A missense mutation (E837D) was identified in affected members of the CORD6 family, as well as a second missense mutation (R838C) in three other families with dominant cone-rod dystrophy. RETGC-1 is only the fourth gene to be implicated in cone-rod dystrophy and this is the first report of dominant mutations in this gene.