Familial exudative vitreoretinopathy (FEVR). Clinical profile and management

PURPOSE: To report our experience with the diagnosis and management of Familial Exudative Vitreoretinopathy (FEVR) in a predominantly older Indian population.. METHODS: This prospective interventional non-comparative case series included 38 patients of FEVR and their 23 family members. The diagnosis was established by clinical examination, fluorescein angiography and family screening. Prophylactic photocoagulation/cryotherapy or surgical treatment was done depending on the severity of the disease. RESULTS: The mean age of the patients was 23.6 years. The fundus/fluorescein angiographic findings in 116 eyes of our 61 patients (6 eyes phthisical) were as follows: forty eight (41.4%) eyes had only peripheral avascular zone, 8 (6.9%) eyes had peripheral new vessels, and 35 (30.1%) eyes had retinal detachments (RD)--10 (8.6%) exudative, 5 (4.3%) tractional and 20 (17.2%) rhegmatogenous. Prophylactic photocoagulation or cryotherapy was done in 34 eyes for retinal holes, local exudative detachments and bleeding new vessels. All the eyes retained stable vision over a mean follow-up of 16 months. Only 14 RDs were suitable for surgery: scleral buckling, vitrectomy or both. The reattachment rate was 85.7% (12 of 14) and the best-corrected visual acuity (BCVA) improved to 5/60 or better in 50% of these eyes over a 2-year follow-up. CONCLUSIONS: FEVR appears to be more common than reported. Timely diagnosis and intervention is essential in view of the lifelong progression of the disease, late exacerbations, frequent involvement of family members, and poor surgical results. A high index of suspicion, family screening and early prophylaxis are recommended to prevent avoidable blindness from this underdiagnosed disease.     

Asthma in children: Management practices among paediatricians and family physicians

OBJECTIVE:

To ascertain the variation in asthma management practices among paediatricians and family physicians to determine how to improve care.

DESIGN:

Questionnaire study of paediatricians and family physicians that focused on the use of beta₂-agonists, inhaled corticosteroids, patient asthma education, quantitative measurements of airflow and diagnostic investigations for asthma. Case scenarios were used in the questionnaire.

RESULTS:

The response rate was 66% (415 of 632) among paediatricians and 42% (1156 of 2750) among family physicians. In general, both groups followed consensus guidelines. There were some differences in management practices among paediatricians and family physicians. Paediatricians were more likely to develop an action plan and less likely to use xanthines or inhaled anticholinergic agents. However, family physicians were more likely to use spirometry or home peak expiratory flow rates to make a diagnosis of asthma.

CONCLUSION:

Family physicians and paediatricians require a different focus on educational interventions to improve the care of children with asthma.     

Pulmonary cystic hydatid disease in Ireland

Background Hydatid disease is rare in Ireland and its incidence and prevalence are unknown. Most cases are diagnosed by a combination of clinical findings, morphological features on imaging and by serological testing. Aims We describe an Irish case of pulmonary hydatid disease detected at bronchoscopy by bronchoalveolar lavage, and discuss the diagnosis and treatment of the disorder.     

Impact of beta-carotene supplementation through red palm

A 10-month long feeding trial was conducted to assess the impact of beta-carotene supplementation through red palm oil (RPO) with the focus on vitamin A status, morbidity status and acceptability of an RPO-incorporated noon-meal as a dietary supplement among two cohorts of 409 (experimental) and 346 (control) preschool children in two southern districts of rural Tamil Nadu, selected by stratified random sampling. Information was gathered on ocular symptoms of vitamin A deficiency and anthropometry, and blood samples were drawn at baseline and final rounds for estimation of serum beta-carotene, retinol and tocopherol. Data about Socioeconomic Status (SES) were collected once during the study period, while information on attendance, consumption and morbidity was recorded by preschool teachers. The results showed the following. 1. Significant improvement in the vitamin A status of children in terms of disappearance of Bitot's spots (50.0 per cent) in the experimental group vs. 28.0 per cent in the control group. 2. After feeding of RPO, incidence rate of new Bitot's spots cases was low at 2.13 in the experimental children vs. 4.78 in control children. 3. Marked improvement in the serum beta-carotene levels after 10 months of feeding. 4. RPO is acceptable to children as an edible grade oil as there is no perceptible difference in the consumption pattern between experimental and control children.     

Tuberculosis notifications in Australia, 1998

Since the inception of the National Mycobacterial Surveillance System (NMSS) in 1991, annual crude notification rates for tuberculosis have remained stable at between 5 and 6 per 100,000 population. In 1998, there was a total of 923 TB notifications in Australia of which 884 were new TB cases, and 39 relapsed cases. The corresponding annual crude notification rate for new and relapsed TB was 4.72 and 0.21 per 100,000 respectively. Seventy-seven percent of notifications that had a country of birth reported were overseas born. In keeping with trends observed over recent reporting years, the populations for which notified TB rates are highest include the overseas born from high prevalence countries and Indigenous Australians. The lowest rates of disease have continued to be reported in the non-Indigenous, Australian born population. Surveillance reports over the last seven years indicate that the rate of disease in this population is gradually declining.     

Sporovexins A-C and a new preussomerin analog: antibacterial and antifungal metabolites from the coprophilous fungus Sporormiella vexans

Sporovexins A-C (1-3) and 3'-O-desmethyl-1-epipreussomerin C (4) have been isolated from liquid cultures of the coprophilous fungus Sporormiella vexans (JS 306). The structures of these new metabolites were elucidated on the basis of MS and NMR analysis. Compounds 1 and 4 show antifungal activity against competitor fungi, as well as antibacterial effects.     

Expression of Fas and Fas ligand in Hodgkin's disease.

Fas and Fas ligand expression were investigated in twenty two cases of classical Hodgkin's disease (HD) by immunohistochemistry. While Reed-Sternberg (RS) cells in 7/22 (32%) cases expressed Fas ligand, reactive lymphoid cells expressed Fas ligand in only 2 (9%) cases. In 20/22 (91%) cases, the RS cells expressed Fas. A higher proportion of RS cells in the nodular sclerosis subtype expressed Fas as compared to the mixed cellularity subtype. In 18/22 (82%) cases, Fas expression was also noted in the reactive lymphoid cells. In eight cases, the reactive lymphoid cells were also analyzed by flow cytometry and a majority of them were CD4+CD45RO+. Most of these activated T-cells expressed Fas but were negative for Fas Ligand. To investigate the co-expression of Fas and Fas Ligand in the RS cells, six cases were subjected to Fas and Fas ligand immunostaining on consecutive sections. The co-expression was documented in the RS cells in four of six cases. These six cases with expression of both Fas and Fas ligand were investigated for the incidence of apoptosis. There was no statistically significant relationship between expression of Fas on reactive cells, expression of FasL on RS cells and the proportion of apoptotic reactive cells. In all these cases apoptosis was not observed in the RS cells. Thus Fas - FasL interactions may not lead to apoptosis of the RS cells.     

Non-Hodgkin's lymphoma of prostate: a rare site of primary extranodal presentation (a report of two cases)

We report two cases of primary extranodal lymphoma of the prostate, an unusual site for extranodal presentation. The clinical presentation and treatment is discussed.