Longitudinal Data in Patients with Niemann-Pick Type C Disease Under Combined High Intrathecal and Low Intravenous Dose of 2-hydroxylpropyl-β-cyclodextrin.

Niemann-Pick Type C disease (NPC) is a rare, incurable, autosomal-recessive, lysosomal storage disorder with protean and progressive neurovisceral manifestations characterized by accumulation of intracellular unesterified cholesterol. The investigational use of 2-hydroxypropyl-beta-cyclodextrin (HP-β-CD) in the treatment of NPC has shown promising results in improving life expectancy and reducing neurological damage in this patient population. This case report describes two children with the neurological form of NPC: a 5-year-old male patient in advanced stage of the disease and an 11-year-old female patient in moderately advanced stage.

The role of bulking agent in pile methane and carbon dioxide concentration during wastewater sludge windrow composting.

Wastewater sludge and wood chips were used as feedstock for the construction of two piles, Pile I ("PI") and Pile II ("PII"), at a ratio of 1:1 and 1:2 v/v, respectively. Each pile was originally 1.3-m high, 2.

Microbial characterization during composting of biowaste.

Windrow composting of source-separated biowaste was studied in a pilot plant in Crete, with regard to abiotic factors, gas concentration in the pile and succession of functional microbial groups. The pH, C/N ratio and VS content, as well as the O(2) and CO(2) concentration, correlated well with composting time, indicating typical composting behaviour. Most of the microbial groups examined exhibited their highest counts towards the end of the thermophilic phase, with declining trends thereafter.

Screening for faecal contamination in primary schools in Crete, Greece.

Background: Hygienic conditions in primary schools are a major concern for both governmental organizations and families. Particularly, the occurrence of faecal indicators on children's hands and various school surfaces has been associated with increased risk of diarrhoeal diseases. The presence of faecal streptococci on environmental surfaces and children's hands and the possible correlation with socio-economic factors were examined.

Constructed wetlands treating highway runoff in the central Mediterranean region.

Two free water surface (FWS) and two subsurface flow (SSF) pilot-size constructed wetlands treating highway runoff (HRO) were monitored over a period of two years (September 2005-August 2007). One FWS and one SSF were designed with a hydraulic retention time (HRT) of 12h, named FWS12 and SSF12, respectively, with each one capable of treating a maximum HRO of 12.6 m(3) d(-1).

Evaluating the use of electrical resistivity imaging technique for improving CH(4) and CO(2) emission rate estimations in landfills.

In order to improve the estimation of surface gas emissions in landfill, we evaluated a combination of geophysical and greenhouse gas measurement methodologies. Based on fifteen 2D electrical resistivity tomographies (ERTs), longitudinal cross section images of the buried waste layers were developed, identifying place and cross section size of organic waste (OW), organic waste saturated in leachates (SOW), low organic and non-organic waste. CH(4) and CO(2) emission measurements were then conducted using the static chamber technique at 5 surface points along two tomographies: (a) across a high-emitting area, ERT#2, where different amounts of relatively fresh OW and SOW were detected, and (b) across the oldest (at least eight years) cell in the landfill, ERT#6, with significant amounts of OW.