A male infant with congenital thrombocytopenia, progressing to pancytopenia in the second year of life is presented. Other findings included microcephaly with cerebellar hypoplasia, growth failure of prenatal onset and severe psychomotor retardation. He died at 23 months of age from candida albicans septicemia. Laboratory studies and a postmortem examination failed to reveal any known etiology for his disorder, but parental consanguinity suggests a genetic basis with an autosomal recessive mode of inheritance. Høyeraal et al. have previously reported two brothers with similar clinical and laboratory findings. It is proposed that the condition of these three patients should be considered as a separate syndrome of congenital pancytopenia, distinguished from other congenital myeloid dysplasias by the extramedullary findings.

The role of hyperinsullnemia in the development of hypertension is not well understood, particularly insofar as both conditions relate to obesity. The present analysis examines the hypothesis that hyperinsulinemia, independent of obesity, precedes hypertension and natural blood pressure increases in women. The subjects were 50 -year-old women from a prospective population study in Gothenburg, Sweden. Fasting insulin levels were determined at baseline (1968)(1969) and were evaluated in relation to subsequent hypertension. Blood pressures were measured at the initial physical examination and at the 6-and 12-year follow-up examinations. The first analysis presented here (n=278) identified incident cases of hypertension during the 12-year follow-up period, whereas the second analysis (n=219) examined continuous changes in blood pressure. In both analyses, degree, type, and changes in obesity were considered as possible confounding factors. High fasting Insulin values were predictive of subsequent incidence of hypertension over the 12-year follow-up period. Subjects with insulin values above the 75th percentile experienced three times more hypertension than did those below the 25th percentile. There was also a significant association between insulin at baseline and increases in diastollc (but not systolic) blood pressure. The positive relations between fasting insulin, on one hand, and diastolic blood pressure changes and hypertension, on the other, could not be explained by confounding effects of body mass index, waist/hip ratio, or weight gain. These findings are consistent with the hypothesis that fasting insulin levels may be one predisposing factor in the etiology of hypertension. associations between hypertensive and hyperin-L. sulinemic states, the mechanisms of which are not clearly established.1 " 5 There is some evidence that elevated insulin levels might raise blood pressure by enhancing renal absorption of sodium.6 -8 It has also been proposed that insulin-induced changes in intracellular electrolyte balance might affect heart muscle and arteriole contractility, causing peripheral resistance and hypertension. 910 Alternatively, insulin may exert hypothalamic effects, with increased sympathetic activity raising heart rate and blood pressure.1112 Finally, rather than being linked by a causal pathway, insulin and blood pressure might be associated due to confounding by a third factor. Because obesity has been associated with both conditions, its possible explanatory role in the association between hyperinsulinemia and hypertension has been an additional topic of interest.13 -15 Two recent reports have suggested that the association between insulin resistance and hypertension may be specific to certain ethnic groups 16 or vary as a function of obesity. A few recent epidemiologic studies have studied this issue prospectivery, with mixed results. Salomaa et al 18 observed a small impairment of glucose tolerance in normotensive men before the onset of clinical hypertension. Skarfors et al 19 observed that fasti...

OBJECTIVE:To study blood pressure and pulse pressure longitudinally and their association with basal and change of body mass index (BMI) and waist to hip ratio (WHR). DESIGN: A prospective population study of 1462 women in Gothenburg, Sweden, aged 38 -60 y at baseline, with a longitudinal follow-up of 24 y. OUTCOME MEASURES: Incidence of hypertension, systolic and diastolic blood pressure, and pulse pressure at baseline and after 12 and 24 y of follow-up. RESULTS: Systolic and diastolic blood pressure as well as pulse pressure increased with age and turned down again at high age. BMI and WHR at baseline were each independently associated with baseline systolic and diastolic blood pressure, but only BMI with pulse pressure. However, baseline BMI and WHR were not associated with change of systolic, diastolic or pulse pressure during 12 or 24 y of follow-up. Increase in BMI during the follow-up period was associated with increase in systolic and diastolic blood pressure but not with increase in pulse pressure. There were no such associations with WHR changes which, were either unrelated or in one analysis inversely related with blood pressure changes. When considering incidence of hypertension during the first 12 y of follow-up, BMI and change in BMI were significant predictors, independent of WHR. CONCLUSION: Age, BMI and increments in BMI seem to be strong predictors for hypertension and increased systolic and diastolic blood pressure in women. In contrast, WHR plays a lesser and uncertain role in the development of hypertension in middle-aged women. Changes in BMI seem not to be accompanied by changes in pulse pressure during a long time follow-up.

Background: Heart failure with reduced ejection fraction (HFrEF) and chronic obstructive pulmonary disease (COPD) are relatively common conditions with similar symptoms of exercise intolerance and dyspnea. The aim of this study was to compare exercise capacity, ventilatory response, and breathing pattern in patient groups with either advanced HFrEF or COPD before and after exercise training. Methods: An observational study was conducted with parallel groups of 25 HFrEF and 25 COPD patients who took part in 6 wk of inpatient rehabilitation with exercise training. All patients underwent cardiopulmonary exercise tests at the start and end of the training, with resting arterial blood gas measurements. Results: The average peak oxygen uptake (V˙o2) was low at the start of the study but increased significantly after training in both groups, or by 2.2 ± 2.1 mL/kg/min in HFrEF patients and 1.2 ± 2.2 mL/kg/min in COPD patients. At ISO-V˙o2 (ie, same level of V˙o2 in pre- and post-exercise tests), carbon dioxide production (V˙co2) decreased after exercise training in both groups. Similarly, at ISO-V˙E (ie, same level of ventilation), breathing frequency (f) decreased and tidal volume (VT) increased, resulting in an improved breathing pattern (lower f/VT ratio) after training. Conclusion: The findings of this study show that exercise training in severely affected patient groups with HFrEF or COPD led to an increase in maximal exercise capacity, a more favorable breathing pattern, and a diminished V˙co2 during exercise. Therefore, comparisons of V˙co2 and breathing pattern at ISO-levels of V˙o2 or V˙E before and after training are valuable and underutilized outcome measures in treatment studies.

This study demonstrated that both a positive Albustix test and microproteinuria were associated with hypertension. Hypertension at baseline increased the risk for death during the follow-up period, while neither albuminuria, defined as a positive Albustix test, nor microproteinuria was associated with an impaired long-term prognosis with respect to renal function or survival in this cohort of Swedish middle-aged women during 24 years of follow-up. Microproteinuria in otherwise healthy normotensive or hypertensive women does not appear to impair the long-term prognosis.