Globally, acute malnutrition triggers more than 50% of childhood mortality in children under 5 years old, which implies that about 3.5 million children die of malnutrition each year. Prior to the advent of ready-to-use therapeutic food (RUTF), the management of acute malnutrition was limited to hospitals, resulting in low coverage rates with high mortality, as malnourished cases were indentified at later stages often plagued with complications. However, current availability of RUTF has enabled malnourished children to be treated at communities. Further, because RUTF is dehydrated and sealed, it has the added advantage of a lower risk of bacterial contamination, thereby prolonging its storage life at room temperature. Recent data indicate that Community Management of Acute Malnutrition (CMAM) is as cost effective as other high-impact public health measures such as oral rehydration therapy for acute diarrheal diseases, vitamin A supplementation, and antibiotic treatment for acute respiratory infections. Despite the high efficacy of CMAM programs, CMAM still draws insufficient attention for global implementation, suggesting that CMAM programs should be integrated into local or regional routine health systems. Knowledge gaps requiring further research include: the definition of practical screening criteria for malnourished children at communities, the need for systematic antibiotic therapy during malnutrition treatment, and the dietary management of severe malnutrition in children below 6 months of age.
McCune-Albright syndrome (MAS) is a sporadic disease characterized by café-au-lait spots, polyostotic fibrous dysplasia and hyperfunctional endocrinopathies. To elucidate the mechanism of skin pigmentation, melanocytes, keratinocytes and fibroblasts were primary cultured from the café-au-lait spot of a MAS patient. Then, mutational analysis and morphologic evaluation were performed. Also, cAMP level and tyrosinase gene expression in cultured cells were determined. Only Gsα mutation was found in affected melanocytes and the cAMP level in affected melanocytes was higher than that of normal melanocytes. The mRNA expression of tyrosinase gene was increased in the affected melanocytes. This study suggests that skin pigmentation of MAS results from activating mutation of Gsα in melanocytes and the mechanism involves the c-AMP-mediated tyrosinase gene activation.
The syndrome of protracted diarrhea (PD) includes several diseases with diverse etiologies. This study was conducted to characterize the spectrum of causes, clinical manifestations, and the outcomes of PD. A retrospective analysis of the clinical and pathological findings was performed on 25 patients with diarrhea starting within the first 2 yr of life and a requirement of parenteral nutrition (PN). According to the intestinal histopathology, patients were classified into four groups: immune enteropathy (12 cases), lymphangiectasia (6 cases), epithelial dysplasia (5 cases), and unclassified (2 cases). All patients with epithelial dysplasia had earlier onset of diarrhea and longer duration of PN than those in the other groups. Three patients (12%) had an evidence of a familial condition. Five patients (three with microvillous inclusion disease and two with immune enteropathy) died. Sixteen patients recovered, and three (two with primary lymphangiectasia and one with microvillous inclusion disease) still had diarrhea. One patient underwent intestinal transplantation for tufting enteropathy. In conclusion, infants with PD should be referred to specialized centers where advanced diagnostic and therapeutic facilities are available, because histological analysis is critical for the diagnosis of PD, and PN or intestinal transplantation is the only therapeutic option in a subset of cases.
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