Multiple pterygium syndrome (escobar syndrome) with atrial septal defect.

The authors present the first case of multiple pterygium syndrome (OMIM # 265000) from Gujarat, a rare syndrome characterized by multiple pterygia, facial and skeletal anomalies. A 9-year-old female child born of consanguineous marriage, with features of arthrogryposis multiplex, multiple pterygia, hypoplastic genitalia and skeletal anomalies presented with pneumonia. A previously unreported association of Atrial Septal defect was discovered on routine 2D echocardiography, which is important for prognostication and follow-up.

A study of clinical profile of multidrug resistant typhoid fever in children.

Background: Salmonella typhi infection remains a serious problem in developing country. It has been estimated that approximately 12.5 million cases of typhoid fever occurs annually in the developing countries with 7.7 million cases in Asia alone. The disease is predominantly a disease of school age children and young adults and is reported to be a milder in infants and young children. Methods: A total of 150 children with clinical and/or laboratory diagnosis of typhoid fever admitted to the tertiary care level government hospital attached with the Government medical college in Pediatrics ward were reviewed during the months of JUNE to MAY for demographic data such as age, sex, clinical features, result of laboratory tests and antibiotic sensitivity in vivo. Results: There were 88 male and 62 female patients, from the age group ranging from 2 years to 12 years. Predominant symptoms were fever, abdominal pain , vomiting and headache. Hepatomegaly was almost twice as frequent as spleenomegaly. Common clinical signs of typhoid fever in adults such as relative bradycardia and rose spots were seldom documented in children. Fever, Toxic look, coated tongue and hepato-spleenomegaly were common clinical signs of clinical presentation in children. The positivity rate of WIDAL test and Blood Culture was 71.33% and 5.33% respectively. Hepatitis, Bronchitis and Encephalopathy were commonly observed complications of Multidrug resistant typhoid fever in this study. Antibiotics sensitivity in vivo revealed resistance rates of 78.12% for Ampicillin, 84.2% for Trimethoprim-Sulfamethoxazole (Co-trimoxazole), 19.5% for Ciprofloxacin, 14.28% for Ofloxacin and 20% for Cefotaxime. Conclusions : No resistance was detected against Cefixime and Ceftriaxone. Except the two patients died during the period of observation of this clinical study, all paediatrics patients survive from their illness completely.