Autosomal recessive type I lissencephaly.

Lissencephaly (LIS) is a brain malformation manifested by a smooth cerebral surface, thickened cortical mantle and microscopic evidence of incomplete neuronal migration, excluding polymicrogyria and other cortical dysplasias. It is important to consider LIS in the diagnosis of developmental delay as many patients may be diagnosed as cerebral palsy. It may have familial occurrence and can occur in sibs of same family often leading to a diagnostic problem. Several lissencephaly syndromes have been described. Here a familial syndrome of lissencephaly is reported. Autosomal recessive inheritance is suggested by recurrence in sibs within the same family, but germ cell mosaicism for a dominant mutation cannot be excluded.

Kawasaki disease: are we missing the diagnosis?

We report 6 cases of Kawasaki disease (KD) diagnosed over a period of one year and review of all the cases reported from India. The diagnosis of KD was based on clinical criteria The mean age of patients was 6.83 years and mean duration of symptoms before diagnosis was 7.5 days. Apart from classical clinical features, elevated transaminases and blood urea along with free fluid in abdomen was present in one case each. Two patients had dilated coronaries that returned to normal on follow up. One patient developed headache and neck stiffness following treatment with intravenous gamma globulins. The outcome was excellent in all the cases.

Status epilepticus in Indian children in a tertiary care center.

OBJECTIVE: To study the clinical profile, immediate outcome and possible risk factors of SE in pediatric age group admitted to pediatric intensive care unit (PICU) in a tertiary care center. METHODS: A retrospective study of case records of 451 neuroemergency patients admitted in PICU in a tertiary care center between January 1993 to April 2000, out of which 30 patients had status epilepticus. They were evaluated for their clinical presentation, laboratory parameters, treatment profile and immediate outcome. RESULTS: The age group varied from 1 to 120 months with mean of 56.6+/- 46.5 months. Seventeen patients were less than 60 months. Sixteen patients (53.3%) presented with SE as first presentation without prior history of seizure activity. Nine patients died (30%) during hospital course. Seizure duration> 45 minutes (p-0.001) and presence of septic shock (p-0.001) were associated with significantly more mortality. CONCLUSION: There is a need to abort seizure activity at the earliest and this improves immediate outcome.

Standard precautions and post exposure prophylaxis for preventing infections.

In health care set up, risk of acquiring infection by both patients and health care worker (HCW) from each other is fairly high. Despite progress, hospital acquired infections (HAI) are a problem in both developed and developing countries and are an important cause of death. Many different microbes cause HAI in both patients and HCW; these include various commensals, pathogenic bacteria, viruses, parasites, and fungi. Among these HIV, hepatitis B, and hepatitis C are of major significance to HCW. 'Standard precautions' have now replaced the term 'universal precautions', and are designed to reduce the risk of transmission of microorganisms in health care set-up from both recognized and unrecognized sources. Ultimate aim is to reduce the risk of disease transmission in the healthcare setting, both to the patient and the provider, and thus reduce morbidity. This applies to all patients, regardless of their diagnosis and expands the coverage of universal precautions by recognizing that any body fluid may contain contagious and harmful microorganisms. This article reviews the standard precautions and discusses current guidelines on post exposure prophylaxis (PEP).

Adenoid cystic carcinoma of the lacrimal gland.

Adenoid cystic carcinoma (ACC) of the lacrimal gland is a rare tumor from the epithelial structure. It is rare in children. The authors report a case of ACC in a girl child. Extensive excision of the tumor is advised to prevent later recurrences. Local control can be better achieved by a combination of radiochemotherapy as ACC has been shown to recur event after 10 years.