Proteus syndrome: Clinical profile of six patients and review of literature.

OBJECTIVE: Proteus syndrome (PS) is characterized by patchy or segmental overgrowth and hyperplasia of multiple tissues and organs, along with susceptibility to development of tumors. Very few cases are reported in literature from developing countries. Due to certain overlapping features with other overgrowth syndromes, diagnosis is usually delayed. Our aim was to describe clinical profile of this rare condition in six patients. MATERIALS AND METHODS: Retrospective case sheet review of patients followed in a Pediatric Genetic and Metabolic clinic at a tertiary care institute of North India with a diagnosis of hemihypertrophy/overgrowth syndrome. RESULTS: Six cases presented with asymmetric overgrowth and peculiar features suggestive of PS were included in this study. Age at presentation was 2 months to 10 years; two were males and four were females. Hemihypertrophy was noticed in only one case at birth, and focal overgrowths in rest of other patients were seen later during childhood. CONCLUSION: Due to certain overlapping features with other overgrowth syndromes, diagnosis of PS is usually delayed. Pediatricians are the first persons who come across such patients and they should be aware about this rare condition.

Prevalence of vitamin D deficiency in apparently healthy children in north India.

BACKGROUND: The data on the prevalence of vitamin D deficiency (VDD) in apparently healthy children from India is limited. OBJECTIVE: To assess the prevalence of VDD in apparently healthy children. DESIGN: Prospective study. SETTING AND SUBJECTS: Apparently healthy children from the age groups of 3 months-12 years, from the upper socioeconomical status (USES), attending the outpatient department (OPD) of a private pediatric hospital in Chandigarh, India, for minor ailments were enrolled over a period of 6 months (March-August 2013). METHODS: Plasma levels of 25-hydroxyvitamin D [25(OH)D] were measured by competitive electrochemiluminescence immunoassay. RESULTS: In total, 338 children (188 boys, 150 girls) with mean age of 3.31 years were enrolled. The percentages of children with deficient, insufficient, and sufficient levels of 25(OH)D were 40.24%, 25.44%, and 34.32%, respectively. Clinical signs of VDD were seen in only 8.53% of the children. The mean (±SD) levels of 25(OH)D were 27.48 (15.99) ng/mL. On univariate analysis, deficient levels of 25(OH)D were associated with relatively younger age group, female sex, failure to thrive, exclusive breastfeeding, inadequate sun exposure, and no vitamin D supplements. CONCLUSION: A high prevalence of clinical and biochemical VDD was noted in apparently healthy children belonging to the USES.