RESUMO
Mutations in the HNF-1ß gene have been found to be associated with renal cysts and diabetes syndrome (RCAD), also known as MODY5. The mutation is inherited in an autosomal dominant fashion, although sporadic mutations can be seen. Pediatric cases of HNF - 1ß mutations are more likely to present with renal involvement like renal failure or renal hypoplasia. In young individuals, the detection of renal abnormalities usually pre-date the diagnosis of diabetes with a mean age of 24 years. We report a 5 year old, end stage kidney disease patient with renal cysts and hypertriglyceridemia (in the absence of overt diabetes) with a known pathogenic mutation in the Hepatocyte Nuclear Factor-1ß (HNF-1ß) gene on chromosome 17q12. This case expands the clinical spectrum of HNF-1ß mutation disorders with a take home message, that end stage renal disease patients with unexplained hypertriglyceridemia (even in absence of diabetes mellitus) should alert a clinician for HNF-1ß mutational analysis.
RESUMO
BACKGROUND: Pediatric acute kidney injury (AKI) is a global health concern with an associated mortality risk disproportionately pronounced in resource-limited settings. There is a pertinent need to understand the epidemiology of pediatric AKI in vulnerable populations. Here, we proposed a prospective study to investigate the epidemiology and associated risk factors of "severe dialysis dependent AKI" in children among South Asian nations which would be the first and largest of its kind. METHODS: The ASPIRE study (part of PCRRT-ICONIC Foundation initiative) is a multi-center, prospective observational study conducted in South Asian countries. All children and adolescents ≤ 18 years of age who required dialysis for AKI in any of the collaborating medical centers were enrolled. Data collection was performed until one of the following endpoints was observed: (1) discharge, (2) death, and (3) discharge against medical advice. RESULTS: From 2019 to 2022, a total of 308 children with severe AKI were enrolled. The mean age was 6.17 years (63% males). Secondary AKI was more prevalent than primary AKI (67.2%), which predominantly occurred due to infections, dehydration, and nephrotoxins. Common causes of primary AKI were glomerulonephritis, hemolytic uremic syndrome, lupus nephritis, and obstructive uropathy. Shock, need for ventilation, and coagulopathy were commonly seen in children with severe AKI who needed dialysis. The foremost kidney replacement therapy used was peritoneal dialysis (60.7%). The mortality rate was 32.1%. CONCLUSIONS: Common causes of AKI in children in South Asia are preventable. Mortality is high among these children suffering from "severe dialysis dependent AKI." Targeted interventions to prevent and identify AKI early and initiate supportive care in less-resourced nations are needed.
RESUMO
OBJECTIVES: This study was aimed at the clinical and microbiological evaluation of the efficacy of subgingivally delivered Indian propolis extract as an adjunct to scaling and root planing (SRP) in the treatment of periodontitis. MATERIALS AND METHODS: Twenty patients diagnosed with chronic periodontitis presenting a minimum of two pockets (probing depth ≥5 mm) were selected. Sites were assigned randomly into control sites (n=20) which received SRP alone or test sites (n=20) which received SRP and locally delivered propolis. At selected sites, the clinical parameters were assessed and subgingival plaque samples were collected at baseline, 15 days and one month. The samples were cultured anerobically for periodontal pathogens. RESULTS: The results indicated that there was a significant improvement in both clinical and microbiological parameters (p<0.01) in the test sites compared to the control sites at the end of the study. CONCLUSION: Subgingival delivery of propolis showed promising results as an adjunct to SRP in patients with chronic periodontitis when assessed by clinical and microbiological parameters.