Hematuria in patients with beta-thalassemia major

Our information about renal involvement in beta-thalassemia major is limited. Recently, few studies have reported proteinuria, hypercalcuria, phosphaturia, and oversecretion of tubular damage markers; however, hematuria has not yet been meticulously studied in these patients. We investigated hematuria in patients with beta-thalassemia major. Urinalysis was performed in 500 patients with beta-thalassemia major under a regular blood transfusion program. In those with hematuria [at least 3 to 5 erythrocytes per high-power field] a second urinalysis was done at the next transfusion time. The patients ranged in age from 6 months to 32 years. The male-female ratio was 1.05:1. Hematuria was detected in 55 [10.6%], including 9.8% of those younger than 20 years and 20.0% of those older than 20 years. Hematuria was persistent in 79.2% of the second urinalyses. Sixty-four percent of the patients with hematuria were females. A blood transfusion program had been started during the first year of life in 81% percent of the patients with hematuria. Sterile pyuria was detected in 4% and proteinuria in 16% of the patients with hematuria, while these figures in patients without hematuria were 2.1% [P=.56] and 1.4% [P=.002], respectively. We found that in patients with beta-thalassemia major, the risk of hematuria rises with age. Moreover, proteinuria seems to be more common in those with hematuria. Further studies are needed to ascertain the importance of these findings

Idiopathic nephrotic syndrome in Iranian children.

Of 231 children with nephrotic syndrome, 87% were steroid sensitive and 13% steroid resistant. Of patients with steroid sensitive nephrotic syndrome, 38.8% were non-relapsers, 34.8% frequent relapsers and 26.4% infrequent relapsers. Among those with steroid resistant nephrotic syndrome, 37.5% had focal segmental glomerulosclerosis with a high mortality rate of 44%

Acute glomerulonephritis in southern Iran

Acute post streptococcal glomerulonephritis [APSGN] is the most common type of in-patient glomerulonephritis [GN] in childhood. It has not been studied well in this region yet. Here, we report our experience with APSGN in a tertiary referral center during a five-year period. Hospital records of all 137 children who had been admitted to Nemazee hospital, between 2001 and 2006, with diagnosis of acute glomerulonephritis [AGN] were reviewed. All demographic, clinical, paraclinical data and consumed medications were obtained. Among 137 children diagnosed as AGN, 122 [89%] had APSGN. Other 15 [11%] children had membranoproliferative glomerulonephritis [n=4], mesangioproliferative glomerulonephritis [n=4], IgA nephropathy [n=2], lupus nephritis [n=2], rapidly progressive glomerulonephritis [n=2], and focal segmental glomerulosclerosis [n=1]. Mean [SD] age in children with APSGN was 8.5 [3.5] [range, 3.5-13] years, 117 [96%] children developed APSGN following a sore throat and 5 [4%] following an impetigo, with 95 [78%] during the cold seasons of the year. Periorbital edema was found in 97.5%, hypertension 75%, gross hematuria 72%, oliguria 37%, generalized edema 19%, azotemia [BUN > 20] 80%, and nephrotic-range proteinuria 24.5%. A high anti streptolysin-O [ASO] titer and a low C3 level was detected in 84% and 86%, respectively. There was dilutional anemia in 51.5%, hyponatremia in 27%, and hyperkalemia 14%. With regard to medications, 19 patients received only furosemide, 73 cases furosemide and nifidipine, and 10 patients furosemide, nifidipine, and another antihypertensive medication. Hypertensive encephalopathy occurred in 3 cases, but no mortality was reported during the study period. APSGN is the most common type of glomerulonephritis in this region. It follows sore throat in the majority of cases. It usually has an uneventful course

Association of distal renal tubular acidosis with hypercalcemia; report on three cases

Hypercalcemia is an endocrine emergency that should be diagnosed as soon as possible and managed carefully. For better management multiple causes of hypercalcemia must be taken into consideration. We observed three infants with hypercalcemia and distal renal tubular acidosis at the time of diagnosis during 5 years. The patients were referred with severe dehydration and failure to thrive. There was no reason for hypercalcemia found in these patients except distal renal tubular acidosis. So we suggest distal renal tubular acidosis as a cause for hypercalcemia

Antigen-specific antibody response in juvenile-onset SLE patients following routine immunization with tetanus toxoid

Infection is now the most common cause of morbidity in Systemic Lupus Erythematosus [SLE]. There is lack of information regarding the specific antibody formation in response to vaccines in young SLE patients. To determine the efficacy of anti-tetanus antibody response in young patients with SLE. Forty SLE patients with mean age of 14.1 years [range: 7-21] and 60 age and sex matched normal controls were enrolled in this study over a period of one year. Diagnosis was made according to the ACR criteria and disease activity was determined based on SLE Disease Activity Index [SLEDAI]. All patients and controls had received the complete schedule of tetanus vaccinations consisting of three primary doses and two boosters by the age of six. Serum immunoglobulins and anti-tetanus antibody titers were determined by Nephelometry and ELISA. Anti-tetanus antibody levels greater than 0.1 IU/ml have been suggested as protective. In all of the patients and controls anti-tetanus antibody titer was > 0.1 IU/ml. IgG, IgA, and IgM levels were in the normal range for their age. Mean disease activity score was 4.9 [range: 0-16]. There was no association between SLEDAI score and anti-tetanus antibody response. School age onset and immunosuppressive therapy does not seem to interfere with development of consistent immunity to tetanus vaccine in young SLE patients

Evaluation of microalbuminuria 4 to 6 years following type 1 diabetes in children

Diabetic nephropathy is one of the major complications and a leading cause of mortality and morbidity in diabetes mellitus. Microalbuminuria is the earliest sign of diabetic nephropathy and it is highly related to glycemic control. Progression of diabetic nephropathy is mostly asymptomatic until advanced stages of renal failure. In this study microalbuminuria and its correlation with duration of diabetes and quality of diabetes control [HbAIc level] is evaluated in 50 children with type 1 diabetes mellitus. Fifty children 4 to 6 years following the onset of type 1 diabetes, below 20 years of age, were enrolled in this study. Twenty four hrs urine was checked twice within 3 to 6 months period for microalbuminuria by nephelometry method and values >30 mg/24hrs were ponsidered abnormal. Also HbAIc level and FBS level assessed simultaneously. Mean FBS level during the years of diabetes and number of attacks of DKA were. Fifty children, 4 to 19 years old with mean age of 14.54 +/- 3.62 years, 28 [56%] males Completed the study. Nineteen [38%], 14 [28%] and 17 [34%] children enrolled in this study 4, 5, 6 after the onset of their diabetes respectively. At 1[st] evaluation microalbuminuria was detected in [26.3%], 4 [28.6%] and 6 [35.3%] children, 4, 5, 6 years after diabetes respectively. At 2[nd] evaluation these values were 4 [21.1%], 6 [42.9%] and 7 [41.2%] respectively. There was no Significant correlation between HbAIC level, FBS level, and mean FBS level during the years of diabetes in microalbuminuric and non-microalbuminuric children. Despite small sample size of this study, microalbuminuria was detected in children even 4 years after the onset of diabetes and its frequency increased in children with 5 and 6 years of diabetes. We recommend earlier than usual recommendations for microalbuminuria screening in diabetic children

Fungal peritonitis in Iranian children on continuous ambulatory peritoneal dialysis

Fungal peritonitis [FP], causing catheter obstruction, dialysis failure, and peritoneal dysfunction, is a rare but serious complication of peritoneal dialysis. In this study, the frequency and risk factors of FP are evaluated in children who underwent peritoneal dialysis. A retrospective multicenter study was performed at the 5 pediatric peritoneal dialysis centers in Iran from 1971 to 2006, and FP episodes among 93 children were reviewed. Risk ratios were calculated for the clinical and demographic variables to determine the risk factors of FP. Ninety-three children aged 39 months on average were included in study. Sixteen out of 155 episodes of peritonitis were fungi infections, all by Candida albicans. The risk of FP was higher in those with relapsing bacterial peritonitis [P = .009]. Also, all of the patients had received antibiotics within the 1 month prior to the development of FP. Catheters were removed in all patients after 1 to 7 days of developing FP. Six out of 12 patients had catheter obstruction and peritoneal loss after the treatment and 5 died due to infection. Fungal peritonitis, accompanied by high morbidity and mortality in children should be reduced by prevention of bacterial peritonitis. Early removal of catheter after recognition of FP should be considered