outcome of childhood onset full house nephropathy

n/

Prevalence and potential relevance of hyperuricemia in pediatric kidney transplant recipients—a CERTAIN registry analysis

Background: Asymptomatic hyperuricemia is frequently observed in pediatric kidney transplant recipients; symptomatic hyperuricemia, however, is a rare complication. Only few data are available in this patient population. We, therefore, investigated the prevalence of hyperuricemia and its association with kidney transplant function and blood pressure in a multicenter cohort of pediatric kidney transplant recipients. Methods: This is a retrospective, observational multicenter registry study. All pediatric kidney transplant recipients in the CERTAIN database with at least one documented serum uric acid level and a follow-up of 5 years posttransplant were eligible. We identified 151 patients with 395 measurements of serum uric acid. We calculated the prevalence of hyperuricemia, analyzed potential risk factors and clinical consequences such as elevated blood pressure and reduced estimated glomerular filtration rate (eGFR). Statistical analysis was performed using IBM SPSS Statistics 26. Results: One hundred and ten of 395 (27.8%) serum uric acid levels were above 416 µmol/L (7.0 mg/dL), defined as the upper limit of normal. Univariate analysis showed a significant (p = .026) inverse association of serum uric acid with eGFR overtime. There was no significant association of serum uric acid concentrations with body mass index (z-score), blood pressure (z-score), or sex. No episodes of gout were documented. Conclusion: This study shows that hyperuricemia is present in a considerable number of patients sometime after pediatric kidney transplantation and is associated with lower eGFR. Whether hyperuricemia contributes to faster decline of graft function or to the overall cardiovascular risk of these patients remains to be elucidated. Keywords: gout; long-term outcome; pediatric kidney transplantation; uric acid; uric acid-lowering therapy

Years of life that could be saved from prevention of hepatocellular carcinoma

BACKGROUND: Hepatocellular carcinoma (HCC) causes premature death and loss of life expectancy worldwide. Its primary and secondary prevention can result in a significant number of years of life saved. AIM: To assess how many years of life are lost after HCC diagnosis. METHODS: Data from 5346 patients with first HCC diagnosis were used to estimate lifespan and number of years of life lost after tumour onset, using a semi-parametric extrapolation having as reference an age-, sex- and year-of-onset-matched population derived from national life tables. RESULTS: Between 1986 and 2014, HCC lead to an average of 11.5 years-of-life lost for each patient. The youngest age-quartile group (18-61 years) had the highest number of years-of-life lost, representing approximately 41% of the overall benefit obtainable from prevention. Advancements in HCC management have progressively reduced the number of years-of-life lost from 12.6 years in 1986-1999, to 10.7 in 2000-2006 and 7.4 years in 2007-2014. Currently, an HCC diagnosis when a single tumour <2 cm results in 3.7 years-of-life lost while the diagnosis when a single tumour 65 2 cm or 2/3 nodules still within the Milan criteria, results in 5.0 years-of-life lost, representing the loss of only approximately 5.5% and 7.2%, respectively, of the entire lifespan from birth. CONCLUSIONS: Hepatocellular carcinoma occurrence results in the loss of a considerable number of years-of-life, especially for younger patients. In recent years, the increased possibility of effectively treating this tumour has improved life expectancy, thus reducing years-of-life lost

Blood pressure measurement in children: which method? which is the gold standard

The burden of hypertension has become increasingly prevalent in children. Hypertension that begins in childhood can carry on into adulthood, therefore early detection, accurate diagnosis and effective therapy of high blood pressure may improve long-term outcomes of children and adolescents. As far as pediatric hypertension is concerned, doubts still persist about the right instruments, modalities and standards of reference that should be used in routine practice. Due to the dynamic process of growth and development, many physiological parameters undergo intensive change with age. Therefore, in children, the definition of hypertension can not rely on a single blood pressure level but should be based on age- and height-specific percentiles. In this review, we introduce the nephrologist to the correct definition of high blood pressure in children. Moreover, we specifically address the main characteristics of different modalities for blood pressure measurement in children, focusing on practical aspects. The latest international guidelines and appropriate standards of reference for office, ambulatory and home blood pressure data collection are presented. As clinicians are being faced with a greater number of children with hypertension, they should be aware of these peculiarities

Un approccio metodologico alla cultura materiale nei siti abbandonati della collina torinese. Il caso di Mombello

Nell'ambito del rogetto di catalogazione dei villaggi abbandonati sulla collina torinese, durante l'ampliamento del territorio comunale tra XI e XIII secolo, si analizza, ance dal punto di vista archeologico, il caso di un villaggio scomparso

Comamonas testosteroni-associated peritonitis in a pediatric peritoneal dialysis patient

Comamonas testosteroni (C. testosteroni) has been rarely observed as an infectious agent in clinical practice. Few reports described its potential pathogenicity in bloodstream and abdominal infections. Here, we report our experience in the treatment of a C. testosteroni-associated peritonitis in a four-year-old girl receiving chronic peritoneal dialysis (PD). The organism was shown to be highly susceptible to appropriate antibiotic therapy. Infection responded promptly and the patient was managed conservatively without withdrawal from PD

Duplex system ureterocele in infants: Should we reconsider the indications for secondary surgery after endoscopic puncture or partial nephrectomy?

OBJECTIVE: We assessed outcomes and need for secondary surgery after primary trans-urethral puncture (TUP) or upper pole partial nephrectomy (UPPN) in duplex system ureterocele (DSU) patients undergoing management that disregards vesicoureteral reflux and upper pole function. SUBJECTS AND METHODS: Between 2003 and 2010, 41 DSU <1 year underwent TUP (n = 32) or UPPN (n = 9). Postoperatively, additional investigations and surgery were limited to cases showing persistent hydroureteronephrosis or developing recurrent febrile urinary tract infections (UTI). Outcome parameters included upper tract decompression, UTI after decompression, continence status, and secondary surgery rate. Preoperative variables were compared between patients who required secondary surgery and those who did not. RESULTS: Additional surgery was required for persistent hydroureteronephrosis in 20% of cases after TUP vs none after UPPN. After decompression, 4 female patients developed recurrent febrile UTI and 2 required additional surgeries. No case suffered from urinary incontinence. After a median (range) follow-up of 46 (17-102) months, TUP or UPPN was the only surgery required in 32 (78%) cases irrespective of preoperative variables. CONCLUSION: UPPN seems more effective than TUP in decompressing severely dilated urinary tracts. After decompression, disregarding VUR status and upper pole function, TUP or UPPN is the only procedure required in 80% of DSU cases, regardless of preoperative variables. Copyright \ua9 2012 Journal of Pediatric Urology Company. Published by Elsevier Ltd. All rights reserved