Childhood survival in Haiti: protective effect of measles vaccination

To evaluate the impact of measles vaccination on survival of children residing in a periurban slum in Haiti, a total-population survey was conducted 2.5 years after completion of a one-time study of the serologic response to measles vaccine administered in the same population. Pregnancy histories from the 16,400 women in the population revealed that 1499 children had been born during a 7-month interval that would have made them eligible for participation in the measles vaccine program. Of these children, 1381 (92.1%) survived to 9 months of age, the median age that measles vaccine had been administered. Seventy-three infants had died between 9 and 39 months of age. Mortality of infants who were seronegative before receiving measles vaccine was significantly lower (P = .0013) than that of unvaccinated infants (3/235 vs 70/1056, respectively). Other factors positively associated with survival between 9 and 39 months of age included socioeconomic status (P = .0002), maternal literacy (P = .0020), maternal knowledge and use of oral rehydration solution (P = .0002), and an interval of greater than 24 months to the birth of the next younger sibling (P = .0012). Multivariate stepwise logistic regression analysis was used to evaluate the independent association of measles vaccination by adjusting for other factors that also correlated with survival and that might have been associated with maternal seeking of vaccinations.(ABSTRACT TRUNCATED AT 250 WORDS)     

An ultrasonographic study of Peyronie's disease

A study was undertaken to determine the usefulness of ultrasonography as an investigative tool, and its role in deciding the management of Peyronie's disease. Fifteen patients with Peyronie's disease were studied by ultrasonography. The plaque could be demonstrated in all patients. The dimensions of the plaque varied from less than 1 cm to more than 7cm in length and 2-4mm in thickness. The disease was active in 26% of the patients, as indicated by the presence of hypoechoic areas around a central region of hyperechoism. Ultrasonogram was more accurate than clinical assessment in delineating the extent of lesions. In one-third of the patients, sonography demonstrated the plaques to be more extensive than had been detected by clinical examination. Calcification and activity of disease (which are clearly defined by ultrasonogram) are determining factors in the management of Peyronie's disease. This information allows the surgeon to select the modality of treatment, the timing of surgery and extent of excision. Thus, ultrasonography plays a vital role in the preliminary investigation and management of Peyronie's disease.     

Staged stent-assisted angioplasty for symptomatic intracranial vertebrobasilar artery stenosis

OBJECT: Medically refractory symptomatic vertebrobasilar atherosclerotic disease has a poor prognosis. Studies have shown that longer (> or = 10 mm), eccentric, high-grade (> 70%) stenoses portend increased procedure-related morbidity. The authors reviewed their experience to determine whether a staged procedure consisting of angioplasty followed by delayed (> or = 1 month later) repeated angioplasty and stent placement reduces the morbidity associated with endovascular treatment of symptomatic basilar and/or intracranial vertebral artery (VA) stenoses. METHODS: The authors retrospectively reviewed the medical records in a consecutive series of eight patients who underwent planned stent-assisted angioplasty for medically refractory, symptomatic atherosclerotic disease of the intracranial posterior circulation between February 1999 and January 2002. Staged stent-assisted angioplasty was planned for these patients because the extent and degree of stenosis of the VA and/or basilar artery (BA) lesion portended an excessive procedure-related risk. The degree of stenosis, recent onset of symptoms (unstable plaque), vessel tortuosity, and lesion length and morphological feaures were contributing factors in determining procedure-related risk. Patient records were analyzed for location and degree of stenosis, preprocedural regimen of antiplatelet and/or anticoagulation agents, devices used, procedure-related complications, and clinical and radiographic outcomes. Among the patients in whom staged stent-assisted angioplasty was planned, vessel dissection, which necessitated immediate stent placement, occurred during passage of the balloon in one of them. In a second patient, the stent could not be maneuvered through the tortuous VA. In a third patient, the VA and BAs remained widely patent after angioplasty alone, and therefore stent placement was not required. Significant complications among the eight patients included transient aphasia and hemiparesis in one and a groin hematoma that necessitated surgical intervention in another; there was no permanent neurological morbidity. The mean stenosis before treatment was 78%, which fell to 54% after angioplasty, and the mean residual stenosis after stent placement was 30%. At the last follow-up examination, none of the treated patients had further symptoms attributable to the treated stenosis. CONCLUSIONS: The novel combination of initial angioplasty followed by delayed endoluminal stent placement may reduce the neurological morbidity associated with endovascular treatment of long, high-grade stenotic lesions. Attempting to cross high-grade stenoses with higher-profile devices such as stents may result in an embolic shower. Furthermore, neointimal proliferation and scar formation after angioplasty result in a thickened fibrous layer, which may be protective during delayed stent deployment. Larger-scale studies involving multiple centers are needed to elucidate further the lesion morphological characteristics and patient population most likely to benefit from staged procedures.     

Major lower-extremity amputation: contemporary experience in a single Veterans Affairs institution

Our objective is to describe our current experience with major lower-extremity amputation secondary to vascular disease. We conducted a retrospective review of sequential amputations over a 3-year period at one Veterans Affairs institution. One hundred thirteen amputations were performed in 99 men (age 70 +/- 11 years). Seventy-five per cent were diabetic and 23 per cent were on dialysis. Fifty-six per cent were primary amputations. The final AKA/BKA (above-knee to below-knee amputation) ratio was 3:2 and was not related to prior bypass, ethnicity, or dialysis status (P > 0.5). Forty-three per cent of amputations were BKAs in diabetics versus 26 per cent in nondiabetics (P = 0.08). The in-hospital and 30-day mortality rates were 2.6 and 8 per cent and were not related to amputation level (P = 0.76). Forty per cent experienced postoperative complications that were most frequently wound related (22%). Wound complications were more frequent with BKA than AKA (P = 0.04). At an average follow-up of 10 +/- 8 months only 65 per cent were alive. Although 51 per cent were discharged to rehabilitation units only 26 per cent regularly wore a prosthesis with 23 per cent ambulating. BKA patients were more likely to ambulate than AKA (34% vs 9%; P = 0.001), and dialysis patients were less likely to ambulate than nondialysis patients (5% vs 25%; P < 0.02). During follow-up 17 per cent of patients discharged with an intact contralateral limb required amputation of that limb and 7 per cent had bypass surgery on that limb. Complication rates were higher in African Americans and Hispanics than in whites (59%, 45%, and 23%, respectively; P < 0.001), although mortality and ambulation rates were similar. Despite an acceptable perioperative mortality complication rates remain high especially in nonwhites. One-year mortality is high. Low rehabilitation rates especially in dialysis patients mandate further efforts in this regard. Vigilant follow-up of the contralateral limb is essential.     

Clinical evaluation of biomarkers in Gaucher disease

Novel or candidate biomarkers require thorough evaluation to establish their utility in a clinical setting. This paper describes an evaluation of several established enzyme markers of Gaucher disease and a newly-described chemokine, pulmonary and activation-regulated chemokine (PARC). The ability of the biomarkers to rank patients with Gaucher disease in order of disease severity and organ bulk, and to reflect changes in key clinical parameters in response to enzyme replacement therapy were evaluated. PARC concentrations were found to be reliably correlated with visceral disease and with key clinical responses to enzyme replacement in an unbiased manner. Unlike chitotriosidase and serum angiotensin-converting enzyme activity, genetic variation in serum PARC did not appear to influence its utility as a biomarker.
Conclusion: For each new candidate biomarker of lysosomal storage diseases, a similar clinical evaluation will be required, though the approach will need to be modified according to the clinical features and natural history of each disorder.     

Acute treatment with tamoxifen reduces ischemic damage following middle cerebral artery occlusion

Inhibitors of cell-swelling-activated anion channels, including the antiestrogenic compound tamoxifen (TAM), have been shown to attenuate the increase in excitatory amino acids (EAA) during ischemia. Since TAM enters the CNS we tested whether it provides protection from damage due to reversible middle cerebral artery occlusion (rMCAo) in rats. TAM (5 mg/kg, i.v.) infused 25 min before ischemia, potently reduced the total volume of the infarct from 328 +/- 34 mm3 to 41 +/- 21 mm3, a reduction of 87%, as measured by TTC staining. It was equally effective when infused starting at 1 h after reperfusion, i.e. 3 h after initiation of rMCAo. Protection of neurons was also found histologically. TAM had no effect on CBF as measured by hydrogen clearance. This appears to be the first report of a marked neuroprotective effect of TAM. Further studies are needed to determine whether its effects are due to inhibition of EAA release and/or other potential neuroprotective sites of action.     

Hirschprung's disease

Current evidence on the pathogenesis of Hirschprung's disease, then, favours the 'abnormal microenvironment' hypothesis wherein the developing and migrating normal neural crest cells confront a segmentally abnormal and hostile microenvironment in the colon. This hypothesis would account both for the congenital absence of ganglion cells in the wall of colon and also for the range of enteric neuronal abnormalities encountered including neuronal dysplasia, hypoganglionosis, and zonal aganglionosis. The abnormal constitution of the mesenchymal and basement membrane extracellular matrix in the affected segment of colon is presumably genetically determined and further understanding of the pathogenesis of this disorder will emerge as molecular geneticists characterise the specific genes and gene products associated with Hirschprung's disease. Advances in this field should permit gene probes to be developed to facilitate prenatal and postnatal diagnosis.