Leukocyte and plasma N-laurylsphingosine deacylase (ceramidase) in Farber disease

Severe deficiency of acid ceramidase activity (4-5% of normal) was demonstrated in cultured skin fibroblasts, leukocytes and plasma from a 1-year-old boy who was diagnosed as being affected with Farber disease. Determination of ceramidase activity in plasma was achieved by a highly sensitive assay employing a ceramide substrate containing radiolabeled C12 N-acyl moiety (N-lauryl). The enzyme activity in the parents' leukocytes and plasma was found to be reduced to 18-47% of the respective normal values, and that determined in a plasma specimen from a patient with I-cell disease was about 4 times elevated above the normal level.     

Acute ammonium dichromate poisoning in a 2 year-old child

Hexavalent chromium compounds are most commonly used in printing, dyeing, plastics and rayon manufacturing. Poisoning in children by ammonium dichromate, an odorless and bright orange-red crystal, are rarely reported. Acute poisoning will result in death due to multi-organ failure. The target organs that are affected by this poison are the respiratory system, kidneys, liver, eyes and skin. On ingestion, initially there is a relative lack of severe symptoms and signs. Hence, the delay in seeking medical attention could lead to the increased rate of mortality. In this case study, we report the ingestion of ammonium dichromate by a child. Despite appropriate management, such as hepatic supportive measures and plasma transfusion, the toxicity progressed to multi-organ failure and death.     

Detection of Multi Drug Resistant Bacteria in Retail Fish Market Water Samples of Vashi,Navi Mumbai

Proceedings of the National Academy of Sciences, India Section B: Biological Sciences - Occurrence of faecal indicator bacteria (FIB) is being reported regularly by various researchers. But there...     

Fast neutron therapy for malignant astrocytomas

The treatment of supratentorial malignant gliomas has continued to be a major problem for neurosurgeons and oncologists. Post-operative conventional radiotherapy is known to prolong survival and to enhance the quality of life. Local persistence of tumor kills the majority of patients. Fast neutron irradiation is being utilized to treat malignant gliomas based on its reputed radiobiological advantages for treatment of large tumors and the encouraging preliminary reports showing tumor eradication in post-radiation biopsy and autopsy specimens. This paper reviews the results of fast neutron irradiation alone and in combination with photons and hypoxic cell sensitizers. Survival comparisons do not show any superiority for neutrons compared to conventional radiation. However, post-neutron radiation tissue samples have shown less agressive and minimal residual tumor in many instances. At the same time radiation necrosis has emerged as a significant problem. In summary, even though neutron irradiation can eradicate malignant gliomas a therapeutic window has yet to be identified. Address for offprints: Neutron Therapy Facility, MS-301, P.O. Box 500, Batavia, IL 60510, USA     

Prenatal diagnosis of I-cell disease in the first and second trimesters

First trimester prenatal diagnosis of I-cell disease (1 case) was based on demonstration of profound deficiency of N-acetylglucosamine 1-phosphotransferase in chorionic villi and in cultured trophoblasts derived from the chorionic villus specimen. Deficiency of this enzyme in cultured amniotic fluid cells obtained via amniocentesis was the basis for prenatal diagnosis of I-cell disease in the second trimester (2 cases). In both procedures, the diagnosis was corroborated by the finding of intracellular deficiency and extracellular elevation of multiple lysosomal enzymes in the fetal cell cultures (trophoblasts and amniotic fluid cells), as well as a significant increase in several lysosomal enzyme activities in the maternal serum.