Pycnodysostosis; A Rare Disease Case Report.

Pycnodysostosis is a rare disease with very few reported cased all over the world. It was first described in 1963 by Maroteaux and Lamy. It is also known as Toulouse-Lautrec syndrome, after a French artist, Henri de Toulouse Lautrec. The affected gene, CTSK, was first isolated in 1996. It is an autosomal recessive osteochondrodysplasia, characterized by disrupted function of osteoclasts. Incidence of this disease is 1.7 per 1 million births with a male to female ratio of 1:1 30% cases arise from consanguineous marriages.

Iatrogenic Cushing Syndrome And Hypertension In An Infant Girl Due To Use Of Nappy Rash Cream Containing A Potent Topical Steroid.

Eleven months old female patient presented to paediatric out patient with parents concerned about her facial swelling. Upon examination child's weight and height for age were normal on her percentiles, she had a cushingoid facies with plethoric cheeks (Figure-1,2) though generalized oedema was absent and there was centripetal obesity with some muscle wasting (Figure-3,4). Systemic examination was normal excluding blood pressure which was high for her age. Electrolytes and cortisol levels were normal. On further inquiry it was revealed that she had been using a nappy rash cream containing a potent steroid, i.e., fluticasone for 2 months and this was identified as a cause for her cushingoid features.

Synthesis and biological evaluation of curcumin-like diarylpentanoid analogues for anti-inflammatory, antioxidant and anti-tyrosinase activities.

A series of 46 curcumin related diarylpentanoid analogues were synthesized and evaluated for their anti-inflammatory, antioxidant and anti-tyrosinase activities. Among these compounds 2, 13 and 33 exhibited potent NO inhibitory effect on IFN-gamma/LPS-activated RAW 264.7 cells as compared to L-NAME and curcumin. However, these series of diarylpentanoid analogues were not significantly inhibiting NO scavenging, total radical scavenging and tyrosinase enzyme activities. The results revealed that the biological activity of these diarylpentanoid analogues is most likely due to their action mainly upon inflammatory mediator, inducible nitric oxide synthase (iNOS). The present results showed that compounds 2, 13 and 33 might serve as a useful starting point for the design of improved anti-inflammatory agents.

Randomized controlled trial of standard versus double dose cotrimoxazole for childhood pneumonia in Pakistan.

OBJECTIVE: Increasing concern over bacterial resistance to cotrimoxazole, which is recommended by WHO as a first-line drug for treating non-severe pneumonia, led to the suggestion that this might not be optimal therapy. However, changing to alternative antimicrobial agents, such as amoxicillin, is costly. We compared the clinical efficacy of twice-daily cotrimoxazole in standard versus double dosage for treating non-severe pneumonia in children. METHODS: A randomized controlled multicentre trial was implemented in seven hospital outpatient departments and two community health programmes. A total of 1143 children aged 2-59 months with non-severe pneumonia were randomly allocated to receive 4 mg trimethoprim plus 20 mg sulfamethoxazole/kg of body weight or 8 mg trimethoprim plus 40 mg sulfamethoxazole/kg of body weight orally twice-daily for 5 days Treatment failure occurred when a child required a change of therapy, died or was lost to follow-up. Children required a change of therapy if their condition worsened (they developed chest indrawing or danger signs) or if at 48 hours after enrollment, their clinical condition was the same (defined as having a respiratory rate that was 5 breaths/minute higher or lower than at the time of enrollment). FINDINGS: The results of 1134 children were analysed: 578 were assigned to the standard dose of cotrimoxazole and 556 to the double dose. Treatment failed in 112 children (19.4%) in the standard group and 118 (21.2%) in the double-dose group (relative risk 1.10; 95% confidence interval = 0.87-1.37). Using multivariate analysis we found that treatment was more likely to fail in children who were not given the medicine correctly (P = 0.001), in those younger than 12 months (P = 0.004), those who had used antibiotics previously (P = 0.002), those whose respiratory rate was > or =20 breaths/minute above the age-specific cut-off point (P = 0.006), and those from urban areas (P = 0.042). CONCLUSION: Both standard and double strength cotrimoxazole were equally effective in treating non-severe pneumonia. Close follow-up of patients is essential to prevent worsening of disease. Definitions of clinical failure need to be more specific. Surveillance in both rural and urban areas is essential in the development of treatment policies that are based on clinical outcomes.