High-resolution loading tests in the study of genetic heterogeneity in gangliosidosis fibroblasts

Summary GM₁- and GM₂-gangliosides were isolated from brain and radio-labelled. The labelled moieties were localized by hydrolysis with lysosomal enzymes, followed by thin-layer chromatography of the products. High-resolution loading tests with labelled gangliosides were developed and found to differentiate infantile and juvenile forms of GM₁- and GM₂-gangliosidoses as well as the identification of B, O and AB types of GM₂-gangliosidosis.     

Adverse ocular effects associated with niacin therapy.

In a retrospective survey of patients taking medication for hyperlipidaemia, those taking niacin (nicotinic acid) were more likely (p < 0.05) to report sicca syndromes, blurred vision, eyelid oedema, and macular oedema compared with those who never took niacin. Additionally, 7% of those taking niacin discontinued the drug owing to adverse ocular side effects, while none of the other lipid lowering agents were found to cause these side effects (p = 0.016). Data from spontaneous reporting systems support a possible association of decreased vision, cystoid macular oedema, sicca-like symptoms, discoloration of the eyelids with or without periorbital or eyelid oedema, proptosis, loss of eyebrow or eyelashes, and superficial punctate keratitis with the use of niacin in high doses. Decreased vision may be marked, and if the drug is not discontinued, may progress to cystoid macular oedema. All ocular side effects listed above are reversible if the association with niacin is recognised and the drug is discontinued; both the incidence and severity of the ocular side effects seem to be dose dependent.     

Histology as a prognostic factor in early gastric cancer.

Of 431 patients with gastric cancer observed in our institution, 23 (5.3%) had early gastric cancer (EGC). Macroscopic presentation, histology, depth of invasion, and lymph node involvement were evaluated in all the cases. All patients underwent surgery and an intensive follow-up was performed. Five of the 23 patients progressed, and the risk factors were examined. Histology seemed to be the main prognostic factor in our study, since intestinal type of EGC was associated to a significantly better prognosis. Total gastrectomy is indicated in the proximal localization of EGC, and should perhaps be performed also in cases presenting undifferentiated histology.     

A prolonged outbreak of ornithosis in duck processors.

In 1985 an outbreak of ornithosis affected 13 of 80 (16%) workers in a duck-processing plant. New employees were three times more likely to become cases than established employees. The highest attack rate was in those on the production line. Following the outbreak, an occupational health scheme was set up to monitor the health of new recruits to the company. Serological evidence of recent infection was demonstrated in 18 of 37 (49%) new employees tested in the first 3 months of employment. Five (14%) also had clinical evidence of ornithosis. Veterinary investigation of the ducks demonstrated a high proportion with asymptomatic chlamydial infection. It is suggested that ornithosis may be more common in duck processors than is currently supposed. Strategies to reduce occupational risks are discussed.     

Pneumonia complicating abdominal sepsis: an experimental model of hematogenous contamination of the lung.

Pulmonary infection complicating intra-abdominal sepsis is a major clinical problem. An experimental model for intra-abdominal sepsis was created with implantation of gelatin capsules, containing 3 x 10(8) cfu E. coli strain no. 2554, in the peritoneal cavity of 20 rats (10 animals received and 10 did not receive antibiotic therapy with ceftriaxone) in order to verify the role of the primary site of infection in the pathogenesis of pneumonia. Ten rats were sacrificed to determine the relative pulmonary weight and 10 were submitted to simple laparotomy and insertion of a germ-free capsule (sham-operated group). In this group of animals there was only one death (10%). All the rats that received antibiotic therapy survived until sacrifice while all the rats that did not receive ceftriaxone died, 7 within the 2nd and 3 on the 6th postoperative day. Pneumonia and peritonitis developed only in the animals that did not receive ceftriaxone. Bacteriological findings of material obtained from peritoneal and pleural cavities revealed the same strain of E. coli used for the experiment, suggesting that bacteria involved in the pleuro-pulmonary infections may originate in the primary site of infection and that antibiotic therapy started at the moment of contamination, can prevent this major complication.     

Premalignant lesions and nonsquamous malignancy of the penis and carcinoma of the scrotum.

Premalignant lesions of the penis include cutaneous horn, balanitis xerotica obliterans, and leukoplakia. The true incidence of progression of each of these to squamous-cell carcinoma is unknown. Bowenoid papulosis, erythroplasia of Queyrat, and Bowen's disease are histologically identical to in situ carcinoma. Although the first is consistently benign, the latter two regularly evolve into invasive cancer. Malignant scrotal lesions include squamous-cell carcinoma, liposarcoma, leiomyosarcoma, basal-cell carcinoma, extramammary Paget's disease, erythroplasia of Queyrat, malignant melanoma, and metastases. Hemangioma can be confused with carcinoma.     

Expression of the Human Immunoglobulin Heavy Chain VH6 Gene Element by Fetal B Lymphocytes

Fetal B lymphocytes in mice and humans use a limited number of the available V_H gene segments. Mouse fetal B cells primarily utilize 3' V_H elements, suggesting that the localization of these elements determines their rearrangement frequency. The previously reported non-random usage of human V_H genes has been more difficult to explain. In this study the authors analysed the expression of the most proximal 3' human V_H element (V_H6) using a monoclonal antibody (JE-6). V_H6 expression was assessed in various B cell differentiation stages from fetal liver, bone marrow and spleen at 12–20 weeks of gestation. The authors demonstrate that the level of V_H6 expression does not exceed a stochastic usage frequency. This suggests that the localization of V_H6 does not significantly promote its expression during human fetal life, and that other factors must affect the usage of V_H genes during human fetal development.