Pediatric nephrology : journal of the International Pediatric Nephrology Association
-
A 5-month-old girl with distal renal tubular acidosis (RTA) and hyperammonaemia that had lasted for 12 days, despite metabolic acidosis correction, is presented in this report. The patient showed failure to thrive, poor feeding, hypotonia and vomiting crisis in absence of inborn errors of metabolism. Probably, hyperammonaemia was the result of an imbalance between the increased ammonia synthesis, in response to metabolic acidosis, and the impaired ammonia excretion, typical of distal RTA. Our case confirms that hyperammonaemia may be observed in distal RTA, mimicking an inborn error of metabolism, and it underlines that hyperammonaemia may persist several days after metabolic acidosis correction.
-
This prospective study, performed in 76 children with a urinary tract infection (UTI), evaluates the diagnostic value of procalcitonin (PCT) and proinflammatory cytokines (IL-1beta, IL-6 and TNF-alpha) in children with acute pyelonephritis documented by dimercaptosuccinic acid scintigraphy (DMSA). Renal parenchymal involvement was assessed by (99m )Tc-DMSA scintigraphy within 7 days of admission. The diagnosis of acute pyelonephritis was confirmed only in patients with reversible lesions on scintigraphy. ⋯ In acute pyelonephritis, serum PCT level was found to be significantly higher than it is in the lower UTI (p <0.001). Also, significantly higher serum proinflammatory cytokines (IL-1beta, IL-6 and TNF-alpha) levels were detected in those with acute pyelonephritis than those with lower UTI (p <0.001). We conclude that both serum PCT and proinflammatory cytokine levels may be used as accurate markers for diagnosis of acute pyelonephritis.
-
Randomized Controlled Trial Clinical Trial
Premedication during micturating cystourethrogram to achieve sedation and anxiolysis.
Micturating cystourethrogram (MCUG) is an imaging technique indicated in the diagnosis and follow-up of many diseases. We investigated the reliability and the efficacy of midazolam and chloral hydrate in sedation and anxiolysis during micturating cystourethrogram. Fifty-three children of similar ages (39 girls, 14 boys, mean age of 5.8+/-3.5 years) were randomized to midazolam (n=17), chloral hydrate (n=18) and control groups (n=18). ⋯ Bladder capacity and frequency of detection of residual urine were not statistically different between the three study groups (P>0.05). Vital signs did not change significantly in any child. Sedation with midazolam does not have adverse effects on the results of micturating cystourethrogram, while it reduces the discomfort in children undergoing this radiological technique.
-
Acute renal failure (ARF) requiring renal replacement therapy (RRT) has been associated with an excess risk of mortality in adult patients with septic shock, but it is unknown whether this is also applicable to pediatric patients. We therefore conducted a retrospective pilot study. All children presenting with septic shock between 1st January 1998 and 1st April 2004 were analyzed. ⋯ Mortality rates were significantly higher in patients with ARF (57.1% vs 6.7%; p=0.02). Pediatric patients with severe septic shock developing ARF have excess mortality compared to pediatric patients who do not develop ARF, although on diagnosis, severity of underlying disease and calculated risk of mortality were comparable. A multicenter trial is necessary to confirm these findings and to determine the contribution of ARF to pediatric sepsis mortality.
-
Chronic hypokalemia is known to induce renal cyst formation in some diseases including primary aldosteronism, distal renal tubular acidosis, Liddle disease and apparent mineralocorticoid excess syndrome. Although chronic hypokalemia is the main clinical feature of Bartter syndrome, renal cyst formation in this disease has never been reported. ⋯ Direct sequencing analysis of the chloride channel CLC-Kb gene identified a heterozygous nonsense mutation (W610X) in exon 16 indicating a diagnosis of Bartter syndrome type III. Although the precise mechanism underlying the development of renal cysts in our patient remains unclear, chronic hypokalemia and nephrocalcinosis may contribute to cyst development.