Juvenile dermatomyositis associated with autoantibodies to small ubiquitin-like modifier activating enzyme: a report of 4 cases from North India and a review of literature

Immunologic Research - Juvenile dermatomyositis (JDM) is the commonest inflammatory myositis in children. The clinical phenotype is often characterized by the presence of myositis-specific...     

Enzyme studies in GM2 gangliosidiosis,and their application in prenatal diagnosis

Assay of hexosaminidase A and B enzymes in four cases with developmental regression and cherry red spot on fundus examination confirmed that three cases had Tay-Sachs disease, and one case had Sandhoff disease. Prenatal diagnosis was carried out by hexosaminidase enzyme assay in amniotic fluid and cells in one family, and chorionic villus sample in the second family. The fetus was diagnosed to be unaffected in one, and affected in the other family. Assay of hexosaminidase A and B is useful for specific diagnosis of GM₂ gangliosidosis, and for prenatal diagnosis to reduce the burden of these disorders.     

Epidemiology of chronic suppurative otitis media and deafness in a rural area and developing an intervention strategy

Of 613 children evaluated in a village in Haryana 94 (15.3%) were observed to have chronic suppurative otitis media (CSOM). Fifty eight (61.7%) children had hearing impairment. CSOM contributed to 71.6% of the hearing impaired (58/81). On analysis of association of CSOM with literacy and socio-economic status of mothers, and age, sex, and upper respiratory tract infections (URI) in children positive correlation was observed only with URIs (P<0.001). Literacy and socio-economic status of the mothers did not correlate significantly with knowledge about treatment seeking, and ear cleaning practices, probably due to the narrow range of incomes and literacy levels. An intervention program consisting of play, demonstrations, health charts and slogans, and aural cleaning and antibiotic drops was introduced.     

A comparison of sampling and analytical methods for assessing occupational exposure to diesel exhaust in a railroad work environment

Methods of assessing occupational exposure to diesel exhaust were evaluated in a railroad work environment. The American Conference of Governmental Industrial Hygienists (ACGIH)-recommended elemental carbon and respirable combustible dust methods of sampling and analysis for assessing diesel exhaust were included in the study. A total of 215 personal and area samples were collected using both size-selective (nylon cyclone and Marple) and non-size-selective samplers. The results demonstrate that the elemental carbon method is suitable for the railroad environment and the respirable combustible dust method is not. All elemental carbon concentrations measured were below the proposed ACGIH Threshold Limit Value (TLV) of 0.15 mg/m3. The concentrations of oxides of nitrogen (nitric oxide and nitrogen dioxide) were also found to be below their respective TLVs. There is no correlation between elemental carbon or respirable combustible dust and the oxides of nitrogen. The elemental carbon as fraction of total carbon is about 13 percent, except for onboard locomotives where it is about 24 percent. Comparison of elemental carbon and respirable combustible dust measurements showed consistent relationships for most sampling locations, with respirable combustible dust concentrations 12 to 53 times higher than the elemental carbon levels.     

Management of ocular perforations resulting from peribulbar anaesthesia

PURPOSE: To analyze the clinical presentation and outcome of treatment for globe perforation secondary to peri-bulbar anaesthesia. METHODS: Eight patients (3 females and 5 males) aged 66-84 years were included in the study. Ocular perforations were suspected in 3 cases before or during surgery, in 4 cases diagnosis was established within one week and in one case at 3 weeks. Three patients underwent indirect argon laser photocoagulation to seal the retinal break, one patient had cryotherapy, 3 patients underwent a pars plana vitrectomy with fluid gas exchange and endo-laser; and one patient refused any further treatment. RESULTS: The final visual acuity after a mean follow up of 14 months was better than 6/9 in 2 patients, between 6/9-6/12 in 4 patients, and perception of light in 2 patients. CONCLUSION: If diagnosed early and treated adequately, a majority of patients with globe perforation during periocular anaesthetic could be saved.     

Ipsilateral rotational autokeratoplasty for the management of corneal opacities

PURPOSE/BACKGROUND: Penetrating keratoplasty is the logical solution for the management of corneal opacities. In situations such as in Papua New Guinea, where donor corneal tissue is scarce and corneal opacities are plenty, an alternative procedure for the management of corneal opacities in the form of ipsilateral rotational autokeratoplasty was considered. METHODS: In the present prospective study, ipsilateral rotational autokeratoplasty was performed in 17 eyes over a 2 year period in a general hospital.The patient's cornea was trephined eccentrically and the corneal opacity was dialed out of the visual axis and was replaced by clear peripheral cornea. RESULTS: Most opacities were leucomata (76.4%). The average size of the opacity was 5.1 mm and the corneal button size was 7 mm.A final visual acuity of 6/18 or better was obtained in 64.7% of cases (at 12 months). No significant postoperative complications were encountered. No complex formula was needed to calculate the size of the button and, by simply adding 3 mm to the pupillary diameter in standard illumination, one could make an estimation of the graft diameter. CONCLUSIONS: Rotational autokeratoplasty has a definite role in places where donor corneal tissue is scarce, in patients in whom long-term steroids are a risk or in situations where follow up of patients is difficult. Rejection is a theoretical impossibility, but late endothelial failure could occur, requiring regrafting. Rotational autokeratoplasty should be seriously considered as an alternative to conventional penetrating keratoplasty.     

Cognitive dysfunction in NIDDM: P3 event related evoked potential study

P3 component of the endogenous cerebral evoked response is a sophisticated, objective and quantitative approach to assess higher functions of the brain. This test was employed using auditory 'odd ball' paradigm to assess cognitive functions in thirty non insulin dependent diabetic patients (NIDDM) aged 43.6 +/- 9 yrs with poor blood glucose control. (HbAlc. 9.9 +/- 1.0%). The peak latencies of N2, P3 components of event related evoked potentials obtained in these patients were compared with 30 age and sex matched non diabetic healthy controls. Latencies of these potentials were: N2 = 248.0 +/- 36.3, P3 = 391.6 +/- 49.9 msec in NIDDM as compared to 220.6 +/- 26.4, 326.2 +/- 26.8 msec in controls and were highly significant (P < 0.001). The duration of disease, blood glucose level or the physical parameters of height, weight and blood pressure did not show any correlation with N2 or P3 latencies or amplitude. These findings provide an electrophysiological evidence of delayed cognition in poorly controlled NIDDM cases.     

Phase I clinical study of didemnin B

Didemnin B (NSC-325319), a new depsipeptide isolated from a Caribbean tunicate, has been evaluated in a clinical phase I study. The drug was administered in a schedule of a 4 weekly intravenous injection in a six-weeks cycle. Fifty-three patients received 71 evaluable cycles in an escalated dose ranging from 0.4 mg/m²/week to 2.5 mg/m²/week. No hematological toxicity was demonstrated at any dose level. Without prophylactic antiemetics nausea and vomiting was dose limiting at 1.2 mg/m²/week. Due to the use of Cremophor EL as a solvent, hypersensitivity reactions occurred in 9 patients. These reactions occurred following prior exposure to the drug and were commonly seen at the 3rd dose. They were not dose related but became more frequent at 1.5 mg/m²/week necessitating prophylactic treatment with H₁ and H₂ receptor blocking agents. Non-hematological toxicities included mild diarrhea, mucositis, anorexia, headaches, and local phlebitis. The dose- limiting toxicity was generalized weakness which became severe and disabling in 3 of 6 patients treated at 2.5 mg/m²/week. No objective responses were documented in 39 patients with evaluable disease. The recommended dose for phase II studies was 2.3 mg/m²/week × 4 4 in a 6-weeks cycle given with prophylactic antiemetics and H₁ and H₂ receptor blocking agents.