Cystic renal lymphangiectasia presenting as renal insufficiency in childhood

Cystic renal lymphangiectasia is an unusual cause of cystic renal disease in childhood. We present a case of bilateral cystic renal lymphangiectasia in a 7-year-old boy who presented with asymptomatic renal insufficiency and anemia with decreased erythropoietin production. The clinical features of this condition and the diagnostic approach are reviewed. Although rare, this disorder should be considered in the differential diagnosis of cystic renal disease. Received: 2 September 1999 / Revised: 26 April 2000 / Accepted: 27 April 2000     

End-stage renal disease in two pediatric patients with Fechtner syndrome

Fechtner syndrome, a disease in the spectrum of the hereditary nephridites, is a macrothrombocytopenia associated with sensorineural hearing loss, cataracts, nephritis, and characteristic leukocyte inclusions. Renal biopsy findings are consistent with those of Alport syndrome, and the associated renal disease is said to be unusual before mid to late adulthood. Here, we review the available literature on this disease and report two African-American pediatric patients with Fechtner syndrome who rapidly progressed to end-stage renal disease during adolescence. We conclude that chronic renal failure can occur at a young age in patients with Fechtner syndrome, with a possible relation to race/ethnicity. Fechtner syndrome, or other variants of Alport syndrome, need to be considered in patients presenting with proteinuria and thrombocytopenia. Received: 13 July 1998 / Revised: 2 June 1999 / Accepted: 3 June 1999     

End-stage renal disease and its treatment in Venezuela

In Venezuela there are 3234 new cases (132 per million population [pmp]) requiring renal replacement therapy each year, and only 40% of these are admitted to the different modalities of dialysis. In the year 2000, there were 195 patients pmp in chronic hemodialysis (4700 patients). Diabetes, glomerular diseases, and hypertension account for more than 60% of the patients in chronic dialysis. Gross mortality in hemodialysis is around 20%, and cardiovascular causes are the primary cause of death (39.5%). Hospital admission in the dialysis patients amounts to 4.6 days/patient/year. Rehabilitation is inadequate. Only 45% of the dialysis patients report normal home or work activities. Transplantation in Venezuela has a general graft survival rate of 83% at 1 year (90% for living related grafts) and 50% (64% for living related grafts) at 10 years. Future tendencies include emphasis in preventive strategies, including early detection and treatment of diabetes and hypertension, as well as efforts to increase the rate of renal transplantation.     

Hepatitis C infection in children and adolescents with end-stage renal disease

The prevalence of hepatitis C virus (HCV) infection and the risk factors associated with its transmission are described in a contemporary cohort of 55 children and adolescents with end-stage renal disease (ESRD). Thirty-seven patients were on dialysis or had been transplanted (ESRD) and 18 had chronic renal failure (CRF) but had not yet received dialysis. Seven (19%) tested positive for HCV by enzyme-linked immunosorbent assay (ELISA), polymerase chain reaction (PCR), or both. None of the children with CRF were infected. HCV infection was associated with length of time on dialysis, but not with age, gender, race, or units of blood transfused. These data corroborate earlier reports and confirm that children with ESRD continue to have a high prevalence of HCV. It is also shown for the first time that elevated transaminases should not be employed to predict HCV infection in this cohort, as all affected children had normal serum levels. Because of unique characteristics in this cohort, both ELISA and PCR are required to maximize HCV diagnostic sensitivity. Although HCV remains an important consideration in pediatric ESRD, the present study shows that recent advances in clinical practice have eliminated one of the major ways in which it was previously being transmitted. Received: 30 July 2001 / Revised: 2 January 2002 / Accepted: 4 January 2002     

Growth failure,risk of hospitalization and death for children with end-stage renal disease

Growth failure remains a significant problem for children with chronic renal insufficiency and end-stage renal disease (ESRD). We examined whether growth failure is associated with more-frequent hospitalizations or higher mortality in children with kidney disease. We studied data on prevalent United States pediatric patients with ESRD in 1990 who were followed through 1995. Patients were categorized according to the standard deviation score (SDS) of their incremental growth during 1990: severe (<–3 SDS), moderate growth failure (>–3 and <–2 SDS), and normal growth (>–2 SDS). Among 1,112 prevalent pediatric dialysis and transplant patients (<17 years, Tanner I–IV), those with severe and moderate growth failure had higher hospitalization rates {relative risk (RR) 1.14 [95% confidence interval (CI) 1.1, 1.2] and 1.24 [95% CI 1.2, 1.3]} respectively than those with normal growth after adjustment for age, gender, race, cause and duration of ESRD, and treatment modality (dialysis or transplant) in 1990. Kaplan-Meier survival analysis showed 5-year survival of 85% and 90% for patients with severe and moderate growth failure, respectively, compared with 96% for patients with normal growth (P<0.001, log-rank). Cox proportional hazards analysis revealed that those with severe (RR 2.9, 95% CI 1.6, 5.3) and moderate growth failure (RR 2.01, 95% CI 1.1, 3.6) had an increased risk of death compared with youths with normal growth, after adjustment. A higher proportion of deaths in the severe and moderate growth failure groups were attributed to infectious causes (22% and 18.7%, respectively) than in the normal growth group (15.6%). We conclude that growth failure is associated with a more-complicated clinical course and increased risk of death for children with kidney failure. Received: 15 August 2001 / Revised: 14 January 2002 / Accepted: 15 January 2002     

Towards guidelines for dialysis in children with end-stage renal disease

There are few data describing the current practices of treatment selection for children with end-stage renal disease (ESRD). In an effort to establish a consensus among Spanish pediatric nephrologists for inclusion and exclusion criteria for renal replacement therapy in children with ESRD, in 1995 we surveyed members of the Spanish Pediatric Nephrology Association. Although only 43% of members responded, pediatric nephrologists and bioethicists studied the results and compiled a list of ten guidelines for treatment of children with ESRD. The proposed guidelines are meant to be a starting point for further discussion. An emphasis on flexibility, individual case assessment, and consideration of the best interests of the patient must remain central to any treatment plan. Decision making should ideally be shared by parents, professionals, the child, when appropriate, and ethics committees, as necessary. Received: 9 February 1999 / Revised: 21 June 1999 / Accepted: 28 June 1999     

Chronic dialysis in children and adolescents

 The 1996 annual report of the North American Pediatric Renal Transplant Cooperative Study (NAPRTCS) summarizes data submitted from 130 centers on 2,208 patients in whom 2,787 independent courses of dialysis were performed between 1 January 1992 and 16 January 1996. Approximately two-thirds of the dialysis population were maintained on peritoneal dialysis (PD), with automated PD remaining the preferred modality. There were 964 episodes of peritonitis in 1,018 patient years, yielding an overall peritonitis rate of 1 episode every 13 patient months. More PD patients attended school full time than hemodialysis (HD) patients at baseline (77% vs. 45%), which continued at 6, 12, and 24 months of followup. There were fewer Hispanic patients who were full-time students, whether on HD or PD, compared with white or black patients; 18% of Hispanic patients did not attend school, even though they were medically capable. The majority of dialysis courses terminated due to transplantation (54%), with change in dialysis modality the next most-common reason (28%). Early dialysis termination for any reason was seen more often in HD than PD (40% vs. 23% at 6 months), but by 24 months similar percentages of PD and HD courses had been terminated (75% HD, 72% PD). The most-common PD access was a Tenckhoff catheter with a single cuff, a straight tunnel and lateral exit site. The majority of HD accesses were external percutaneous catheters, with the sublcavian vein the most-common site. Erythropoietin was administered in 93% of HD and PD patients at 24 months. Received: 27 January 1998 / Revised: 16 July 1998 / Accepted: 22 July 1998     

Nephrotic syndrome and end-stage renal disease with WT1 mutation detected at 3 years

We report a boy who presented at 3 years with nephrotic syndrome and end-stage renal failure. Although histopathological findings showed end-stage kidney, isolated diffuse mesangial sclerosis (IDMS) was suspected because of his clinical course, and was confirmed by the presence of WT1 (Wilms tumor suppressor gene) mutation. He did not have ambiguous genitalia or Wilms tumor. The karyotype was 46:XY. A constitutional mutation in exon 7 (953G→A, 312Arg→Gin) was detected. A few cases of male IDMS, associated with WT1 mutations, have been reported. We believe that investigation for the WT1 mutation should be performed not only in Denys-Drash syndrome and IDMS, but also in end-stage renal disease with unexplained nephrotic syndrome of early onset. WT1 mutation-associated nephrotic syndrome has an increased risk of Wilms tumor. Careful ultrasound evaluations or bilateral nephrectomies are indicated. Received: 24 November 1998 / Revised: 5 February 1999 / Accepted: 9 February 1999     

End-stage renal disease secondary to renal malignancy: Epidemiologic trends and survival outcomes

Objectives

Loss of renal parenchyma after surgery may contribute to chronic kidney disease; however, the long-term consequences of chronic kidney disease may differ by cause. We analyzed the outcomes of patients with end-stage renal disease (ESRD) based on various medical and surgical causes.

Cystic renal cell carcinoma,suspected because of lack of regression of renal cysts after renal transplantation in a dialysis patient with acquired renal cystic disease

A 34-year-old man who had been on dialysis for about 6 years, and had acquired renal cystic disease, received a renal transplantation. However, in spite of the success of the transplantation, one area without cyst regression was observed in the original kidney. Therefore, carcinoma was suspected and nephrectomy was performed. Histology revealed cystic renal cell carcinoma (RCC). No case of cystic RCC occurring in a dialysis patient has previously been reported. Cystic RCC should be suspected in a cystic mass in the original kidney which does not regress after successful renal transplantation. Received: August 23, 2002 / Accepted: November 25, 2002 Acknowledgments We thank Dr. Shigehiko Sato, Department of Internal Medicine, Tonami General Hospital, who referred this patient to us. Correspondence to:I. Ishikawa     

Lymphosuction – a new treatment modality for chronic filarial lymphedema

The surgical treatment of filaritic lymphedema despite various well reasoned attempts in the past to improve function and contour has to date defied cure. The technique of blunt suction lipectomy has been applied in eight cases and it appears to be well tolerated with encouraging short term results. The final results were accepted favourably by the patients because of smaller incisions, instant improvement in contour, reduction in weight of limb in addition to simple and minimal postoperative care required. Based on our findings, lymphosuction can be a useful treatment modality in mild grades of filarial lymphedema, though further long term clinical observations and studies are required to define its exact indications and limitations. Severe cases require lymphosuction plus excisional debulking. Received: 17 January 1996 / Accepted: 15 July 1997     

Exercise-induced acute renal failure in a patient with renal hypouricemia

We describe a case of exercise-induced acute renal failure (ARF) in a patient with hypouricemia. Following recovery from ARF, the patient’s serum urate concentration was 0.6–0.9 mg/dl, and the ratio of urate clearance to creatinine clearance (C _ua/C _Cr) was 41.9%–56.6%. There was no change in the C _ua/C _Cr following the administration of pyrazinamide or probenecid, suggesting defects of tubular urate/anion exchangers. Because the renal biopsy revealed acute tubular necrosis without uric acid crystals, the ARF of this patient might be due to oxygen free radicals resulting from exercise stress and hypouricemia. Received: 15 March 1999 / Revised: 10 September 1999 / Accepted: 14 September 1999     

Ulcerative colitis in a renal transplant patient with previous Goodpasture disease

A renal transplant was performed in a 6-year-old boy who developed end stage renal disease (ESRD) after presenting with antiglomerular basement membrane (anti-GBM) disease. At 10 years of age he developed ulcerative colitis while being immunosuppressed with cyclosporin, prednisone, and azothioprine. He had a pancolectomy, and at 14 years has no symptoms of ulcerative colitis or anti-GBM disease. HLA typing revealed that he was homozygous for HLA DR2. The co-occurrence of anti-GBM disease and ulcerative colitis has not previously been described. Although there is no known common etiology for these two autoimmune diseases, we propose that the patient’s homozygosity at HLA DR2 may have predisposed him to both. Received: 21 September 2000 / Revised: 26 January 2001 / Accepted: 1 February 2001     

Vascular access in elderly patients with end-stage renal disease

During the last few years, the number of elderly patients with end-stage renal disease (ESRD) has been increasing worldwide. Establishment of a viable vascular access is of primary importance in these patients. This review discusses the advantages and disadvantages of the available vascular access modalities [namely arteriovenous (AV) fistulae, AV grafts, and central venous catheters (CVCs)] in elderly ESRD patients. AV fistulae seem to be superior when compared with other vascular access alternatives with respect to patency, morbidity and mortality rates. On the other hand, due to the age-related advanced atherosclerosis in the elderly, higher failure rates for AV fistulae in this age group have been described. Two controversial issues, namely the higher infection and thrombosis rates in elderly ESRD patients, are also discussed. Current evidence suggests that old age should not comprise a drawback when selecting the appropriate vascular access modality (AV fistula, AV graft or CVC) for the performance of hemodialysis. The possible vascular access options in elderly ESRD patients should not be different from younger individuals.     

Chronic renal failure in pediatrics 1996

 The Nephrology Branch of the Chilean Pediatric Society has greatly influenced the development of government health plans regarding the management and care of patients under 18 years with chronic renal failure (CRF). In order to assess the status of children with CRF in Chile up to 1996, a questionnaire was sent to all pediatric nephrologists in charge of those children. The total sample was of 227 patients under 18 years, giving a national prevalence of 42.5 and an incidence of 5.7 per million inhabitants; of these patients, 50.7% were male, 58.6% over 10 years and 15% younger than 5 years. The most frequent etiologies of CRF were: obstructive uropathy, 18.1%; hypo/dysplasia, 16.7%; reflux nephropathy, 16.7%; and glomerulopathies, 16.3%. Although 48% of patients were on conservative medical treatment, 42.2% of these were in end-stage renal disease, 22.9% were on dialysis, and 29.1% had undergone renal transplantation. Of the dialysis group, 75% were on peritoneal dialysis. Of the transplanted children, 78.8% had normal renal function, but 16.7% returned to dialysis. Three-year graft survival and patient survival were 68% and 94%, respectively. Received: 19 January 1998 / Revised: 6 July 1998 / Accepted: 16 July 1998     

Metabolic alkalosis in a hemodialysis patient after ingestion of a large amount of an antacid medication

A maintenance hemodialysis patient developed metabolic alkalosis in the absence of vomiting or nasogastric suction. The cause of the metabolic alkalosis was ingestion of an exogenous alkali in the form of Bromoseltzer. The metabolic alkalosis improved with hemodialysis using a low-bicarbonate bath.     

Therapeutic camping for children with end-stage renal disease

Therapeutic camping experiences for children with end-stage renal disease (ESRD) have proliferated in the United States and abroad. This report is based on the results of a survey designed to accumulate data on the development and implementation of 20 such camps. Children attending camp ranged in age from 1 year to 19 years. Single disease-specific camps were most common, while camps for children with a variety of chronic illnesses, including ESRD, and mainstream camps were also conducted. Facilities were available for hemodialysis and continuous ambulatory peritoneal dialysis, but not automated peritoneal dialysis, in the majority of surveryed camps. Dialysis nurses, pediatric nephrologists, dietitians and social workers were the medical personnel that most frequently participated in the camps. On average, 32 dialysis/transplant patient campers (range 6–100) attended camp for a 1-week session. Therapeutic camping experiences for children with ESRD are extremely successful and attempts to increase the availability of similar camps should be encouraged.Presented in part at the 13th Annual Conference on Peritoneal Dialysis, 7–9 March 1993, San Diego, California, USA.     

End-stage renal disease of the Tunisian child: epidemiology,etiologies, and outcome

From December 1989 to December 1993, 90 children under 15 years were admitted to our department for end-stage renal disease; 9 children were less than 5 years and 28 were aged between 5 and 10 years. The sex ratio (M/F) of the children was 2.1. The estimated incidence of pediatric end-stage renal disease in Tunisia is 7 new cases per year and per million child population under 15 years. The chief etiologies of end-stage renal disease are glomerulonephritis (19%), hereditary nephropathies (29%), and malformative uropathies (13%); 26% of the end-stage renal diseases are of unknown etiologies. Our findings, compared with the European data, show a particularly high frequency of primary hyperoxaluria (13.5%) and an unusual proportion of male subjects. Received April 13, 1995; received in revised form and accepted January 4, 1996     

Clinical guidelines and hospital discharges of children with acute urinary tract infections

 In order to evaluate the effect of the introduction of recent similar guidelines on the treatment of acute urinary tract infection (UTI) in children, and possible changes in its epidemiology, we analyzed the records of hospital discharge for acute UTI under the age of 15 years in England and Wales between 1979 and 1993 and in Finland between 1978 and 1994. Cases were defined by the ICD9 diagnostic codes 590.1 (acute pyelonephritis) and 599.0 (UTI, site not specified) for males and females according to three age groups (0–4, 5–9, and 10–14 years). We also compared the registry data on kidney transplants due to end-stage renal disease caused by recurrent pyelonephritis in the United Kingdom and Finland. In England the rate of attack of symptomatic UTI per 1,000 girls under 15 years increased from 0.74 (95% confidence interval 0.71–0.76) in 1987 to 1.32 (1.29–1.35) in 1993 (P<0.001, test for trend). The respective figures for Finnish girls were 1.74 (1.62–1.86) in 1987 and 1.62 (1.51–1.74) in 1993 (P=0.72). In English boys, the increase in the attack rate was from 0.38 (0.36–0.40) in 1987 to 0.70 (0.68–0.73) in 1993 (P<0.001). In Finnish boys the respective figures were 0.74 (0.66–0.82) in 1987 and 0.88 (0.80–0.97) in 1993 (P<0.02). The observed increases in the attack rates of UTI most probably relate to increased referral of acute UTI patients to hospitals for the recommended imaging studies rather than changing occurrence. Publication of guidelines for treatment of UTI in children, consolidating more-general awareness, may have contributed to this. The mean annual numbers of kidney transplants in the United Kingdom and Finland during 1989–1995 due to end-stage renal disease caused by pyelonephritis were of similar magnitude, i.e., 1.9 (1.6–2.3) transplants per million inhabitants in the United Kingdom and 2.8 (1.5–4.7) transplants per million inhabitants in Finland. The decreasing trend in these figures in both countries, although statistically significant only in the United Kingdom (P<0.05, test for trend), suggests improved long-term outcome of these patients induced by better diagnosis and treatment of pyelonephritis and the diseases related to it, such as congenital malformations. According to our data, valid clinical guidelines are effective in changing clinical practice. Received: 1 September 1997 / Revised: 29 April 1998 / Accepted: 29 April 1998