Life expectancy in British Marfan syndrome populations

A total of 206 patients with Marfan syndrome were ascertained throughout genetic clinics in Wales and Scotland during the period 1970–1990. There were 45 deaths representing 22% of the cohort. Mean age at death was 45.3 ± 16.5 years. 50% median cumulative survival in the total cohort (n = 206) was 53 years for males and 72 years for females. Multivariate analysis confirmed severity as the best independent indicator of survival. These findings and survival curves will assist in the counselling of British families and individuals with Marfan syndrome.     

Does diabetes increase cell death in the heart?

Indian Journal of Thoracic and Cardiovascular Surgery -     

Verstoß einer privaten Krankenversicherung gegen das Rechtsberatungsgesetz durch Einzug abgetretener Patientenforderungen

Ohne Zusammenfassung     

Neutrophil dermatosis of the dorsal hands

Neutrophil dermatosis of the dorsal hands (NDDH) is a recently described skin manifestation regarded as a subset of acute febrile neutrophilic dermatotis (Sweet syndrome). We describe 5 cases with pustular and ulcerative plaques and/or bullae and vesicles of the dorsal hands. Three of the patients also had skin changes at sides other than the hands. Associated conditions were found in two patients, one patient treated with hemo-dialysis for chronic glomerulonephritis, and one patient had suffered from a streptococcal tonsillitis prior to the eruption. Two of the patients had fever, two had neutrophil leucocytosis in peripheral blood and two had elevated sedimentation rates. Histological findings showed signs of vasculitis in biopsies from two of the patients. NDDH is discussed on the basis of prior case reports concerning the subject, and it is concluded that Neutrophil dermatosis of the dorsal hands should be regarded as a localized variety of Sweet syndrome.     

Infant growth and aorta total lipid fatty acids

Abnormal fetal and infant growth have increasingly been correlated with adult onset cardiovascular disease. To date, there is little known about the lipid fatty acid profiles in infant cardiovascular tissue. Therefore, we analysed total lipid fatty acids from thoracic and abdominal aorta intima and media from 24 normally grown sudden infant death syndrome cases. Aorta from small for gestational age (n = 2), failure to thrive from birth (n = 3), and premature (n = 1) infants were also examined. Dihomo-gamma-linolenic acid (C20:3n-6) and oleic acid (C18:1n-9) concentrations were significantly lower in the thoracic than in the abdominal aorta. Similar dietary related differences were found in the subgroup (n = 15) of infants fed on formula milks. Both abdominal and thoracic intimal arachidonic (C20:4n-6) to dihomo-gamma-linolenic acid ratios were greater in the infants with retarded growth after birth than in their normally grown counterparts. Growth restriction in infancy might disrupt the normal accretion of vascular endothelial polyunsaturated fatty acids.