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Dichlorodiphenyltrichloroethane (DDT) is a well-known insecticide that was introduced and widely used during World War II. In total more than 4.5 million tonnes DDT have been produced. Although its use and production stopped worldwide during the 1970s, it was reintroduced in the 2000s as a malaria vector control by the World Health Organization (WHO). DDT is toxic to animals and humans. Its main characteristics are its persistence, lipophilicity, and bioaccumulative potential. DDT and its metabolites are normally determined in organisms, sediments, or soil by gas chromatography combined with either electron capture detection or, preferably, mass spectrometry. Many interlaboratory studies have been carried out on the analysis of DDT and its metabolites, and certified reference materials are now available. DDT and its metabolites have been found in air, water, sediment, and biota from all over the world. As a consequence of global fractionation and cold condensation, DDT and its metabolites accumulate in the Arctic. Since the reintroduction of DDT against malaria in Africa in 2005, monitoring of DDT compounds is again highly relevant to detect changes in environmental levels of DDT. Such monitoring is particularly needed in Africa. The first indications show an increase of DDT in humans and wildlife.

Objective Propionic acidemia is one of the rare congenital neurometabolic disorders with autosomal recessive inheritance. This disorder is caused by a defect in the propionyl-CoA carboxylase enzyme and can be presented with life-threatening ketoacidosis, lethargy, failure to thrive, and developmental delay. Materials & Methods The patients diagnosed as having propionic acidemia in Neurology Department of Mofid Children’s Hospital in Tehran, Iran, between 2002 and 2012 were include in our study. This disorder was confirmed by clinical manifestations, neuroimaging findings, and neurometabolic assessment in the reference laboratory in Germany. Our study was conducted to define the sex, age, gender, past medical history, developmental status, clinical findings, and neuroimaging manifestations in 10 patients with propionic acidemia. Results Seventy percent of patients were offspring of consanguineous marriages. In this study, only one patient had microcephaly at birth, but at detection time, 30% of patients had head circumference and weight below the 3rd percentile. The patients were followed for approximately 5 years and this follow-up showed that the patients with early diagnosis had a more favorable clinical response. Neuroimaging findings included brain atrophy, white matter and globus pallidus involvement. Conclusion Finally we suggest that early diagnosis and treatment have an important role in the prevention of disease progression.

Several syndromes have been recognized with digital abnormality and CNS involvement such as oculodentodigital dysplasia (ODDD), Mohr syndrome and Joubert syndrome. We report a patient who was referred to us because of the neurological signs suspicious of metabolic disorders. This case was a 22-year-old woman whose problems began 4 years ago with shortening of memory, ataxia, abnormal gait and diplopia which progressed slowly. She consulted many neurologists and was on treatment with the suspicion of vasculitis, but no response was detected. She had severe short stature, hypoplasia of the middle and distal phalanges of the first, second and third fingers, clinodactyly, abnormal toes, abnormal enamel and missing teeth. She had no characteristic faces of ODDD and ophthalmological abnormality. Our patient might be a variant of ODDD or a new syndrome with somatic and neurologic signs.