Aid and AIDS: a delicate cocktail

Development assistance targeting health overwhelmingly concentrates on HIV/AIDS. This column argues that that focus neglects critical demographic issues and degrades health infrastructure, particularly in Sub-Saharan Africa. The prime rule for AIDS aid should be “First, do no harm”.

What Drives Donor Funding in Population Assistance Programs?

The 1994 International Conference of Population and Development (ICPD) established goals for the expansion of population assistance. This global effort has so far not sufficiently been supported by donor funds. Dynamic panel estimation methods are used to see what lies behind the sharing of burdens and level of donor contributions. Panel data on expenditures for population and AIDS activities have been collected for 21 donor countries for the years 1996-2002. Donor countries are willing to contribute to the ICPD agenda, but those contributions depend heavily on national interests and preferences and to a lesser extent on the development state of less developed countries. Political opportunism in the timing of funds is not strong. With respect to the sharing of the ICPD burden within the group of OECD/DAC countries one can say that on an aggregated scale the burden of population assistance programs is in line with the ability to pay of donor countries. Differences in funding are more connected to other factors such as the size of governments, the state of development of a country and the dominant religions in donor countries

Ageing of a giant: a stochastic population forecast for China, 2006-2060

This paper presents a stochastic population forecast for China with a special emphasis on population ageing. The so-called scaled model for error was used to quantify the uncertainty attached to the population predictions. Data scarcity was a major problem in the specification of the expected error of the population forecast. Therefore, the error structures estimated for European countries were used with some modifications, taking into account the large size and heterogeneity of the Chinese population. The stochastic forecast confirms the expectation of extremely rapid population ageing during the first half of the twenty-first century in China. The old age dependency ratio (OADR) will certainly increase. Simply maintaining the current demographic rates (no international migration) would drive the OADR to 0.42 in 2060, four times the current level. Including expected declines in mortality and net outmigration in the projection would increase the median OADR in 2060 to 0.59, with a 80% prediction interval of [0.47, 0.75]. In particular, the oldest-old population will grow much faster than any other age group. This development has major implications for policy-making in China. Keywords: Stochastic population forecast Predictive distribution Uncertainty Scaled model for error China Population ageing Low fertility Mortality decline

A case of adult Pompe disease presenting with severe fatigue and selective involvement of type 1 muscle fibers

AbstractWe present a case of adult Pompe disease (acid maltase deficiency) with an uncommon clinical presentation characterized by severe fatigue and myalgia prior to the onset of limb girdle weakness. Remarkably, the muscle biopsy demonstrated selective involvement of type 1 muscle fibers. The cause and clinical effects of fiber type specific involvement are currently unknown, but the phenomenon might contribute to the clinical heterogeneity in Pompe disease and the variable response to enzyme replacement therapy

A biochemical and ultrastructural evaluation of the type 2 Gaucher mouse

Gaucher mice, created by targeted disruption of the glucocerebrosidase gene, are totally deficient in glucocerebrosidase and have a rapidly deteriorating clinical course analogous to the most severely affected type 2 human patients. An ultrastructural study of tissues from these mice revealed glucocerebroside accumulation in bone marrow, liver, spleen, and brain. This glycolipid had a characteristic elongated tubular structure and was contained in lysosomes, as demonstrated by colocalization with both ingested carbon particles and cathepsin D. In the central nervous system (CNS), glucocerebroside was diffusely stored in microglia cells and in brainstem and spinal cord neurons, but not in neurons of the cerebellum or cerebral cortex. This rostralcaudal pattern of neuronal lipid storage in these Gaucher mice replicates the pattern seen in type 2 human Gaucher patients and clearly demonstrates that glycosphingolipid catabolism and/or accumulation varies within different brain regions. Surprisingly, the cellular pathology of tissue from these Gaucher mice was relatively mild, and suggests that the early and rapid demise of both Gaucher mice and severely affected type 2 human neonates may be the result of both a neurotoxic metabolite, such as glucosylsphingosine, and other factors, such as skin water barrier dysfunction secondary to the absence of glucocerebrosidase activity

Survival and associated factors in 268 adults with Pompe disease prior to treatment with enzyme replacement therapy

Background: Pompe disease is a rare lysosomal storage disorder characterized by muscle weakness and wasting. The majority of adult patients have slowly progressive disease, which gradually impairs mobility and respiratory function and may lead to wheelchair and ventilator dependency. It is as yet unknown to what extent the disease reduces the life span of these patients. Our objective was to determine the survival of adults with P

Effects of a higher dose of alglucosidase alfa on ventilator-free survival and motor outcome in classic infantile Pompe disease: an open-label single-center study

Background: Though enzyme-replacement therapy (ERT) with alglucosidase alfa has significantly improved the prospects for patients with classic infantile Pompe disease, some 50 % of treated infants do not survive ventilator-free beyond the age of 3 years. We investigated whether higher and more frequent dosing of alglucosidase alfa improves outcome. Methods: Eight cross-reactive immun