Mucopolysaccharidoses in Calcutta

Seven hundred children referred to the clinic for mental retardation were screened for various inborn errors of metabolism. Eighteen were found to have mucopolysaccharidoses (MPS). Ten of these children were followed for a period of five years. Three of them died. Two of them had type 1 MPS, based on physical findings, biochemical investigations and deficient iduronidase activities of leucocytes. There were seven patients with MPS type II in this series. Two pairs were siblings. Skin cultures carried out in Dr Neufeld's Laboratory showed iduronate sulphatase deficiency. Three patients had type IV mucopolysaccharidoses. Two were siblings. In one patient leucocyte beta-galactosidase activity was about 1/8th of normal control. One patient had MPS type VI; her leucocytes had no detectable aryl sulfatase B. One patient had unclassifiable MPS. They had some clinical improvement as a result of parental care but the progression of the disease has remained unaffected.     

Antidiarrhoeal activity of seed extract of Albizzia lebbeck Benth

The antidiarrhoeal activity of the seed extract of Albizzia lebbeck (Benth.) was investigated employing conventional rodent models of diarrhoea, i.e. castor oil-induced diarrhoea, upper gastrointestinal transit (u.g.t.) and fluid secretion. It was found that the aqueous methanol extract of Albizzia lebbeck seeds (2.5-5 mg/kg i.p.) possessed antidiarrhoeal activity which strengthens the earlier use of the seeds in the treatment of diarrhoea and dysentery. The antidiarrhoeal dose of the extract was at least 10-30 times less than the LD(50) dose. The extract (2.5-5 mg/kg i.p.) potentiated the antidiarrhoeal activity of loperamide (1 mg/kg i.p.). Nalaxone (0.5 mg/kg i.p.) significantly inhibited the antidiarrhoeal activity of the extract as well as loperamide, thus indicating a role of the opioid system in the antidiarrhoeal activity of the extract.     

Chromosome Rearrangement with No Apparent Gene Mutation in Familial Adenomatous Polyposis and Hepatocellular Neoplasia

We have identified a constitutional inversion in chromosome 5 associated with familial adenomatous polyposis in three generations of a Mexican family. Two of three siblings developed hepatic neoplasia in infancy. The gene truncation assay failed to demonstrate a truncated protein in the segment harboring the adenomatous polyposis coli (APC) genes. Polymerase chain reaction (PCR) amplification of APC gene coding exons and sequencing of PCR products did not reveal any significant mutation. The data suggest that in this family, the phenotype may be the result of a “position effect.” Received May 21, 2001; accepted July 23, 2001.     

Prophylaxis of radiation-associated mucositis in conventionally treated patients with head and neck cancer: a double-blind, phase III, randomized, controlled trial evaluating the clinical efficacy of an antimicrobial lozenge using a validated mucositis scoring system.

PURPOSE: Mucositis occurs in almost all patients treated with radiotherapy for head and neck cancer. The aim of this multicenter, double-blind, prospective, randomized trial was to evaluate the clinical efficacy of an economically viable antimicrobial lozenge (bacitracin, clotrimazole, and gentamicin [BcoG]) in the alleviation of radiation-induced mucositis in patients with head and neck cancer. PATIENTS AND METHODS: One hundred thirty-seven eligible patients were randomized to treatment with either antimicrobial lozenge (69 patients) or placebo lozenge (68 patients). The primary end point of the study was the time to development of severe mucositis from the start of radiotherapy. Secondary end points included severity and duration of mucositis, pain measurement, radiation therapy interruption, and quality of life. Mucositis was scored using a validated mucositis scoring system. RESULTS: Toxicity profiles were similar between the two arms of the study. The median time to development of severe mucositis from the start of radiotherapy was 3.61 weeks on BCoG and 3.96 weeks on placebo (P =.61). There were no statistically significant differences between the arms in the extent of severe mucositis as measured by physician, in oral toxicities as recorded by patients, or in radiotherapy delays. CONCLUSION: This study was conducted on the basis of a pilot study that demonstrated the BCoG lozenge to be tolerable and microbiologically efficacious. A validated mucositis scoring system was used. However, in this group of patients treated with conventional radiotherapy, the lozenge did not impact significantly on the severity of mucositis. Whether such a lozenge would be beneficial in treatment situations where rate of severe mucositis is higher (ie, in patients treated with unconventional fractionation or with concomitant chemotherapy) is unknown.     

Paediatric adrenocortical neoplasia — a study of 25 cases

We reviewed our experience of 25 children with adrenocortical tumours from January 1980 to December 1994. Their ages ranged from 7 months to 15 years; there were 16 girls and 9 boys. Truncal obesity, moon facies, hypertension, and virilisation were the most common clinical features. Establishment of the diagnosis of Cushing's syndrome was accomplished by hormonal and radiological studies. Eighteen patients had adrenocortical carcinomas, but 2 of them refused operation; 7 had adrenocortical adenomas. Twenty-three patients were treated by surgery; 6 with carcinomas are still alive after periods of 6 months to 3 years. The results of treatment of adrenocortical carcinoma have been poor, but the prognosis is excellent for benign lesions.     

Immunostimulating agents against influenza virus infection in senescent rats

This study investigated the nonspecific immunomodulatory effects of Bacillus Calmette-Guerin (BCG), muramyl dipeptide (MDP) and ascorbic acid (vitamin C) on virus infection of the respiratory tract in Fischer-344 rats. Groups of young adult (12-16 months old) and aged (24-30 months old) rats were given BCG or MDP intranasally or vitamin C orally 6 weeks and again 3 days before an intranasal challenge with influenza virus A/Bangkok/H3N2 (10(6) 50% EID). Titers of hemagglutinin in lung homogenates and the presence of leukocytes in the respiratory tracts served as indices of infection. Lung macrophage functions of animals were determined by measuring random migration and phagocytic yeast cell killing in vitro. Clearance of Staphylococcus aureus from the respiratory tracts of the animals was also measured 4 hours after challenge. - Following BCG treatment, significantly fewer animals developed virus infection. MDP and vitamin C treatments also reduced the numbers of infected rats but did not differ significantly from the untreated control groups. BCG and MDP treatments significantly reduced the numbers of lung leukocytes in virus infected animals. Mean virus titers in the lung homogenates were significantly lower in all treatment groups by the third day of infection. Following all treatments, duration of virus infection was significantly shorter in the aged compared to the young adult groups. Lung macrophage functions increased in all treatment groups. The improvement of aged groups over their controls was greater than that of the young adults compared to their controls. BCG had the greatest protective effects in both the young adult and aged animals; MDP and vitamin C, in that order, were less effective.(ABSTRACT TRUNCATED AT 250 WORDS)