The removal of ammonia from sanitary landfill leachate using a series of shallow waste stabilization ponds.

This study evaluated the efficiency of a shallow (0.5 m deep) waste stabilization pond series to remove high concentrations of ammonia from sanitary landfill leachate. The pond system was located at EXTRABES, Campina Grande, Paraiba, Northeast Brazil. The pond series was fed with sanitary landfill leachate transported by road tanker to the experimental site from the sanitary landfill of the City of Joao Pessoa, Paraiba. The ammoniacal-N surface loading on the first pond of the series was equivalent to 364 kg ha(-1) d(-1) and the COD surface loading equivalent to 3,690 kg ha(-1) d(-1). The maximum mean ammonia removal efficiency was 99.5% achieved by the third pond in the series which had an effluent concentration of 5.3 mg L(-1) ammoniacal-N for an accumulative HRT of 39.5 days. The removal process was mainly attributed to ammonia volatilization (stripping) from the pond surfaces as a result of high surface pH values and water temperatures of 22-26°C. Shallow pond systems would appear to be a promising technology for stripping ammonia from landfill leachate under tropical conditions.

The spectrum of pathogenic mutations in SPINK5 in 19 families with Netherton syndrome: implications for mutation detection and first case of prenatal diagnosis.

The Comèl-Netherton syndrome is an autosomal recessive multisystemic disorder characterized by localized or generalized congenital ichthyosis, hair shaft abnormalities, immune deficiency, and markedly elevated IgE levels. Life-threatening complications during infancy include temperature and electrolyte imbalance, recurrent infections, and failure to thrive. To study the clinical presentations of the Comèl-Netherton syndrome and its molecular cause, we ascertained 19 unrelated families of various ethnic backgrounds. Results of initial linkage studies mapped the Comèl-Netherton syndrome in 12 multiplex families to a 12 cM interval on 5q32, thus confirming genetic homogeneity of Comèl-Netherton syndrome across families of different origins. The Comèl-Netherton syndrome region harbors the SPINK5 gene, which encodes a multidomain serine protease inhibitor (LEKTI) predominantly expressed in epithelial and lymphoid tissues. Recently, recessive mutations in SPINK5 were identified in several Comèl-Netherton syndrome patients from consanguineous families. We used heteroduplex analysis followed by direct DNA sequencing to screen all 33 exons and flanking intronic sequences of SPINK5 in the affected individuals of our cohort. Mutation analysis revealed 17 distinct mutations, 15 of which were novel, segregating in 14 Comèl-Netherton syndrome families. The nucleotide changes included four non-sense mutations, eight small deletions or insertions leading to frameshift, and five splice site defects, all of which are expected to result in premature terminated or altered translation of SPINK5. Almost half of the mutations clustered between exons 2 and 8, including two recurrent mutations. Genotype-phenotype correlations suggested that homozygous nucleotide changes resulting in early truncation of LEKT1 are associated with a severe phenotype. For the first time, we used molecular data to perform prenatal testing, thus demonstrating the feasibility of molecular diagnosis in the Comèl-Netherton syndrome.

Phacomatosis pigmentovascularis with a selective IgA deficiency.

We report a 1-year-old boy with an extensive cutaneous vascular malformation, oculocutaneous pigmentation, and severe neurologic abnormalities from birth, as well as a selective IgA deficiency. Ultrastructural study demonstrated prominent endothelial cells in the luminal of the blood vessels. The diagnosis of phacomatosis pigmentovascularis type IIb seemed appropriate for this patient.

Benign cephalic histiocytosis: report of four cases.

We cared for four patients with benign cephalic histiocytosis, a self-healing non-X, nonlipid cutaneous histiocytosis of children. The age of onset of the disease was 5 to 9 months, with papules and erythematous macules involving the head (mainly the cheeks), and posterior spread to the trunk and limbs in three patients. Microscopic examination of skin biopsies revealed a histiocytic infiltrate in the superficial dermis that was S100 protein-negative by immunoperoxidase (PAP method). One patient showed comma-shaped bodies and desmosomelike junctions on electron microscopy. No Birbeck's granules were present. Benign cephalic histiocytosis is a self-limiting condition that requires no treatment.