Long-term prognosis of idiopathic nephrotic syndrome in children.

BACKGROUND: To investigate the demographic, clinical and laboratory data of the children with idiopathic nephrotic syndrome (INS), and to determine prognostic factors that affect the clinical outcome of the patients. METHODS: Medical charts of 372 patients diagnosed to have INS and followed up at least 5 years between January 1990 and December 2008 were evaluated, respectively. After initial demographic, clinical and laboratory findings of the patients were documented, therapeutic protocols, prognosis and prognostic factors were investigated. RESULTS: 299 of the patients (80.4%) were steroid responsive and 73 (19.6%) were not. Focal segmental glomerulosclerosis (FSGS) was observed in 57%, minimal change disease (MCD) in 20.6% and diffuse mesengial proliferation in 21.9% renal biopsy materials. Steroid sensitivity was higher in patients with MCD and under the age of five years. Resistance to steroids was higher in children with FSGS. Complete remission was achieved in 96% of patients who were sensitive to steroids and in 46.6% who were resistant. 15% of patients who were steroid resistant developed chronic kidney disease (CKD). CONCLUSION: Intercurrent infections and response to steroid therapy are the most important factors affecting the prognosis of the disease.

The relation between treatment and prognosis of childhood membranoproliferative glomerulonephritis.

BACKGROUND: The prognostic factors, the outcome and the most favorable treatment regimen are not entirely known for children with membranoproliferative glomerulonephritis (MPGN). MPGN is a rarely observed disease more prevalent in adolescents, so we aimed to review the clinical and histological properties, treatments and the outcome of our patients who were diagnosed as MPGN. METHODS: Fifty-one children - diagnosed with MPGN - were selected from biopsy records in Dr. Sami Ulus Maternity and Children's Hospital Pediatric Nephrology Department from January 1999 to January 2011. A retrospective analysis was made of 33 regularly followed children. RESULTS: Thirty-three patients were identified, 13 female and 20 male. Their age groups at presentation ranged from 4 to 15 years. The following duration was 26-144 months (mean 74). Following the initial treatment, 20 (60%) patients achieved complete remission. Six patients with nephrotic syndrome and one with non-nephrotic proteinuria showed partial remission. The condition of one patient with nephrotic syndrome was unchanged with the persisting symptoms. The one patient with nephrotic syndrome and four others with non-nephrotic proteinuria did not respond to initial treatment as their renal functions decreased gradually. CONCLUSION: We concluded that only degree of tubulointerstitial damage on the initial biopsy is determinative for prognosis of childhood MPGN. If the patient receives high doses of steroid therapy in the early stages, their treatment is more likely to be successful. The effect of immunosuppressive treatment on MPGN is not clear.

Can sonographic peritoneal thickness be used to follow pediatric patients on peritoneal dialysis?

BACKGROUND: Peritoneal dialysis (PD) is an effective and successful therapy for end-stage renal disease (ESRD). However, PD does not have a life-long effectiveness, and peritoneal membrane failure is commonly observed in long-term PD patients. We hypothesized that ultrasonography could be used to follow these patients. METHODS: We recruited two patient groups (age range 3-18 years), of whom 20 had ESRD with ongoing PD for ≥24 months (study group) and 20 were pre-dialysis non-ESRD patients (control group). None of the patients had peritonitis during the preceding 3 months, and none had a history of abdominal surgery or malignancy. We measured the sonographic thickness of the parietal peritoneum and obtained Doppler indices of the superior mesenteric artery (SMA) by trans-abdominal ultrasonography. RESULTS: Peritoneal thickness as determined by sonography was significantly greater in the PD group than in the controls. The correlation between duration of PD and thickness of the peritoneal membrane was linear and statistically significant. We categorized all 20 patients as either rapid transporters or slow transporters for both creatinine and glucose. The peritoneal membranes of patients who were rapid transporters for both creatinine and glucose were significantly thicker than those of the slow transporters. No statistical difference was found between the Doppler indices of the SMA between the groups. CONCLUSION: Thickness of the parietal peritoneum as determined by sonography is associated with PD duration and transport characteristics. We conclude that ultrasonography is a non-invasive and practical method which can be useful for following PD patients.

An unusual case of ANA negative systemic lupus erythematosus presented with vasculitis, long-standing serositis and full-house nephropathy.

Systemic lupus erythematosus (SLE) is a chronic inflammatory autoimmune disease that may affect any organ of the body. We report here an unusual case of seronegative SLE presented as vasculitis with rash, lower gastrointestinal system bleeding and acute renal failure. The patient was a 13-year-old boy, with abdominal distention, pretibial edema, arthritis and petechia on bilateral ankles. He had deteriorated renal functions (creatinine 1.65 mg/dl), hypoalbuminemia (1.6 g/dl) and hypocomplementemia with nephrotic range proteinuria and hematuria. He developed pleural effusion and peritonitis. Serum ANA, anti dsDNA, p ANCA, c ANCA, anticardiolipin IgM and IgG titers were negative. A renal biopsy was performed which revealed diffuse proliferative glomerulonephritis with full-house staining pattern in immunofluorescent microscopic examination suggesting Class IV Lupus Nephritis. He was administered a total of six courses of monthly intravenous pulse methyl prednisolone, dipyridamole, oral cyclophosphamide followed by azothiopirine and oral prednisolone therapy. The renal functions and serum albumin levels turned normal but peritonitis persisted and disappeared after the third pulse steroid therapy. In conclusion, we presented this patient to remind the possibility of SLE in such seronegative patients with unusual findings in order to avoid the delay in the management of this disease with high mortality and morbidity if not treated. Full-house nephropathy is an important clue especially for the diagnosis of ANA negative SLE.

The association between urinary tract infection and idiopathic hypercalciuria in children.

BACKGROUND: Identifying the risk factors is important in prevention of urinary tract infections (UTIs) in children. The aim of this study is to evaluate the association of UTI and idiopathic hypercalciuria (IHC). METHODS: Two hundred and twenty-four children aged between 1 month and 16 years and diagnosed to have UTI were evaluated for urinary calcium excretion. The children were diagnosed to have IHC if their urinary calcium/creatinine ratios in at least two different spot urine samples were >0.6 between 0-1 year old and ≥0.21 over 1 year or daily calcium excretion >4 mg/kg. RESULTS: The frequency of IHC was found to be 16.7%. Family history of urolithiasis, parental consanguinity, presentation with abdominal pain, loss of appetite, and discomfort were found to be significantly higher in the IHC group. No association was found between IHC and the recurrence of UTI, presence of vesicoureteral reflux, renal scar formation, and the prognosis. CONCLUSIONS: IHC should be considered among the risk factors for UTI and should be investigated particularly in patients with family history of urinary stones and suggestive complaints of IHC.

An unusual pediatric case with neurofibromatosis and systemic lupus erythematosus.

Neurofibromatosis type 1 (NF1) is a relatively common autosomal dominant disorder affecting mainly ectodermal and mesodermal tissues. It is well known that patients with NF1 have an increased risk of developing benign and malignant tumors, but its association with autoimmune diseases has been rarely reported. Systemic lupus erythematosus is an autoimmune chronic inflammatory disease that has the potential to affect various organ systems. There are four cases with NF1 and SLE reported in the literature up to date. Here, we report a 9-year-old girl presenting with NF1 and SLE, and to our knowledge, this is the first childhood case in the literature.

Comparison of infants and children with urolithiasis: a large case series.

We evaluated the demographic features, etiologic risk factors, treatment strategies, and outcome of the infants and children with urolithiasis (UL). A retrospective multicenter study was conducted including 23 Pediatric Nephrology centers in Turkey. The medical records of 2513 children with UL were reviewed. One thousand, three hundred and four boys and 1209 girls (1.1:1) were reported. The mean age at diagnosis was 39.5 ± 35 months (0.4-231 months), and 1262 patients (50.2%) were in the first year of life (infants). Most of the cases with infantile UL were diagnosed incidentally. Microlithiasis (< 3 mm) was found in 794 patients (31.6%), and 64.5% of the patients with microlithiasis were infants. Stones were located in the pelvis-calyces in 63.2% (n: 1530) of the cases. The most common stone type was calcium oxalate (64.6%). Hypocitraturia was the most common metabolic risk factor (MRF) in children older than 12 months, but in infancy, hypercalciuria was more common. Fifty-five percent of the patients had received at least one medical treatment, mostly potassium citrate. At the end of a year's follow-up, most of the patients with microlithiasis (85%) showed spontaneous remission. The rate of spontaneous stone resolution in infants was higher than in children. Spontaneous remission rate was higher in cases with MRF ( - ) stones than in MRF ( +) stones. However, remission rate with medical treatment was higher in cases with MRF ( +) stones. This study represents the results of a large series of infants and children with UL and showed that there are several differences such as underlying metabolic and anatomic abnormalities, clinical course, and stone remission rates between infants and children with urinary stone disease.

Prevalence of MEFV gene mutations and their clinical correlations in Turkish children with Henoch-Schönlein purpura.

AIM: To investigate the frequency of MEFV mutations and their associations with the clinical and laboratory findings in children with Henoch-Schönlein purpura (HSP). METHODS: One hundred and seven children with HSP were investigated for 12 common MEFV mutations. RESULTS: Forty-seven patients (43.9%) were found to have one of the MEFV mutations. Eight patients (7.5%) were homozygous for one mutation, 33 (30.8%) were heterozygous for one and six (5.6%) were compound heterozygous for two mutations. There were no age and sex differences between patients with or without mutations. Scrotal involvement was statistically more frequent in patients with mutations. Leucocyte counts, erythrocyte sedimentation rates, serum C-reactive protein (CRP) concentrations, number of patients with increased CRP levels and number of patients with increased immunoglobulin A concentrations were found to be higher in patients with MEFV mutations. p.M694V was the most frequent mutation and was found to have effects on clinical and laboratory findings in children with HSP. Fifteen patients were started on colchicine with the diagnosis of familial Mediterranean fever (FMF). CONCLUSION: MEFV mutations are more frequent in HSP than in the general population, and mutation carriers may have more severe clinical findings with higher inflammatory response, suggesting a dysregulation of the inflammatory response because of defective gene encoding the protein pyrine. Investigation of these mutations may be beneficial to follow-up the susceptible patients more closely leading to early diagnosis and treatment of FMF.

Do immunosupressive patients really have a severe outcome with H1N1 virus infection?

Influenza virus is a common human pathogen that has the potential to cause widespread pandemics. The last pandemic began in April 2009 in CA, USA and killed about 14,000 people since then. The virus affects people at all ages, and school-aged children have the highest rates of infection. Chronic lung disease, immunosuppression and pregnancy are risk factors for seasonal influenza and pandemic influenza as well. Here, we report five immunosuppressive patients due to various diseases infected with H1N1 influenza and who were completely revealed after promptly treatment with oseltamivir.

Rare presentation of cystinosis mimicking Bartter's syndrome: reports of two patients and review of the literature.

We present here two girls with cystinosis initially diagnosed as Bartter syndrome. Both cases were admitted with hypokalemic, hypochloremic alkalosis. Their proximal tubular functions, ophthalmologic and bone marrow examinations were normal. They were started on therapies with the diagnosis of Bartter syndrome. The first patient developed signs of rickets, and the second patient was lost to follow-up and readmitted with chronic renal failure. On reevaluation cystine crystals were detected in cornea and bone marrow aspirates of both patients. We aimed to remind the rare presentation of cystinosis with metabolic alkalosis mimicking Bartter syndrome by these two cases and review the literature.

Is the urinary protein excretion pattern compatible with renal morphological findings in renal amyloidosis?

The aims of this study are to compare urinary protein excretion pattern with renal morphological findings and to find out whether urinary protein excretion pattern is a prognostic indicator of renal amyloidosis. Fifteen children with renal amyloidosis secondary to familial Mediterranean fever were included in the study. The patients were classified into three groups according to the degree of tubulointerstitial injury in renal biopsy (group 1, <25%; group 2, 25-50%; and group 3, >50%). In all patients, urinary protein electrophoresis were performed. Levels of urinary beta(2)-microglobulin, retinol binding protein, and beta.N-acetyl-D glucosaminidase were measured as markers for tubular injury, and urinary excretions of protein and albumin and plasma albumin levels were measured as markers of glomerular injury. While urinary excretions of protein and albumin and plasma albumin levels were not different between groups, higher urinary beta(2-)microglobulin and retinol binding protein values and lower creatinine clearance values were found in group 3 than in groups 1 and 2 (p < 0.05). We concluded that analysis of urinary protein excretion pattern is a non-invasive and reliable method to detect the degree of tubulointerstitial injury as the most important prognostic factor in renal amyloidosis and may be used to determine the changes during the follow-up period of the patients.

Oxidative stress in children with acute glomerulonephritis.

BACKGROUND: Oxidative stress has not been adequately investigated in acute glomerulonephritis (AGN); therefore, we aimed to evaluate the oxidative stress (OS) status in children with AGN both at acute and remission stages. PATIENTS AND METHODS: Seventeen children (mean +/- SEM, age 9.0 +/- 0.5 years) with AGN and 17 healthy controls were included. In addition to routine laboratory investigations, two blood samples were obtained from patients, at admission and after 6-10 weeks, to measure erythrocyte superoxide dismutase (SOD) activity and plasma malondialdehyde (MDA) level. RESULTS: Significantly elevated MDA levels (5.11 +/- 0.28 vs. 3.15 +/- 0.25 nmol/mL; p < 0.001) were found in acute stage of AGN compared with the controls; however there was no significant difference in SOD activities (3732 +/- 193 vs. 4035 +/- 142 U/gHb; p > 0.05) between acute stage-AGN and control subjects. Significantly elevated SOD activities (3985 +/- 195 U/gHb, p = 0.034) and decreased MDA levels (4.01 +/- 0.38 nmol/mL, p = 0.001) were found at remission stage when compared with the acute stage. MDA levels and SOD activities of remission phase were similar to those of controls (p > 0.05). A significantly positive correlation was found between MDA levels and SOD activities in remission period (r = 0.654, p = 0.004). Patients with and without impaired renal functions had similar MDA levels and SOD activities (p > 0.05). No significant correlation was found between glomerular filtration rates (GFR) and MDA levels (p > 0.05) and between GFR and SOD (p > 0.05) activities in acute stage-AGN. CONCLUSIONS: Oxidative stress may play important role in the pathogenesis of AGN and not be correlated with renal functions. Further research is needed to determine magnitude of OS and indications for antioxidants in other glomerulopathies.

Long-term efficacy and safety of quadruple therapy in childhood diffuse proliferative lupus nephritis.

In this study, we evaluated the frequency, clinical presentation, treatment protocols, prognostic factors, and outcome in children with diffuse proliferative lupus nephritis (DPLN). Between June 1990 and December 2004, 46 patients were diagnosed to have systemic lupus erythematosus (SLE), and 26 of them (56.5%) were found to have DPLN. Renal manifestations were present in 25 patients, and the majority of them presented with severe renal findings, such as nephrotic syndrome and renal failure. All patients were given a quadruple therapy protocol including 6-12 monthly courses of methyl prednisolone pulse therapy combined with oral prednisolone, oral cyclophosphamide, azathioprine, and dipyridamole. Nineteen of these patients were regularly followed up with a mean follow-up period of 5.9 years. Complete remission was achieved in 15 of 19 patients, and chronic renal failure developed in four patients. Renal survival rate was calculated to be 78.9% at the end of 5, 10, and 14 years. Although nephrotic range proteinuria, hypoalbuminemia, renal failure, and activity index above 12/24 at presentation seemed to be associated with poor prognosis, no significant difference could be found. Hypertension and chronicity index greater than 6/12 were found to be bad prognostic predictors. We concluded that satisfactory results were achieved with our quadruple therapy protocol; thus, more aggressive and expensive therapies can be avoided and preserved for more serious and persistent diseases.

Crescentic glomerulonephritis in children: a single centre experience.

BACKGROUND: Crescentic glomerulonephritis (CsGN) is characterized by crescents in 50% or more of glomeruli and clinically by a sudden and progressive decline in renal function. METHODS: We evaluated the etiology, clinical features, prognostic factors and long-term outcome of CsGN. Between January 2000 and December 2010, 45 children (26 girls, 19 boys) with biopsy-proven CsGN (>50% crescents) were investigated retrospectively. RESULTS: The mean age of the patients was 130.86±33.77 months. The mean duration of symptoms prior to diagnosis was 26±12 days (4-40 days). Most of the children had hypertension (62.2%), macroscopic hematuria (73.3%), oligoanuria (44.4%), edema (51.1%) and purpuric rash (40%) at presentation. The final clinical status of the patients was complete remission (n=21), partial remission (n=5) or chronic kidney disease (n=19). Adverse outcomes were significantly associated with a long duration between the onset of symptoms and treatment (P=0.038), the presence of oligoanuria (P=0.006), a severe decreased glomerular filtration rate (GFR <30 mL/min/1.73m²) and the need for dialysis (P=0.003) on admission, the ratio of crescents (>75%) (P=0.03), and the ratio of fibrous crescents (P=0.015). CONCLUSION: The outcome of CsGN in children continues to be poor, and it should be treated as a renal emergency.

A patient with Behçet's disease presenting with acute urinary retention.

Behçet's disease (BD) is a multisystemic inflammatory disorder of unknown etiology. Neurologic involvement is known to be the most devastating feature of BD. The frequency and types of neurologic involvement in the pediatric age group are not clear, and the available information is limited to case reports. Here, we report a BD patient who presented with urinary incontinence as the initial feature of spinal cord involvement.

A one-center experience with pediatric percutaneous renal biopsy and histopathology in Ankara, Turkey.

In this study we evaluated the indications, complications, and the spectrum of histopathological results of percutaneous renal needle biopsy (PRNB) performed in our clinic. Between June 1990 and December 2006, 679 PRNBs were performed on native kidneys of 614 children (304 boys, 310 girls) with a mean age of 10.4 years. Most frequent indications for PRNB were nephrotic syndrome (47%), hematuria, and/or proteinuria (15.9%), acute renal failure (14.6%) and complex renal manifestations (18.9%). The overall complication rate was 15.2%. The most common complications were perirenal hematoma (12.4%) and macroscopic hematuria (2.6%). The most frequent histopathological group of diseases were glomerulopathies; these were diagnosed in 376 patients (61.2%) and included membranoproliferative glomerulonephritis (11.1%), mesangial proliferation (10.7%), diffuse proliferative glomerulonephritis (7.7%), and focal segmental glomerulosclerosis (7.3%) as the most frequent. The second most frequent group of histopathology was manifestations secondary to systemic diseases; these were shown in 195 patients (31.8%). Amyloidosis (11.4%) and Henoch-Schönlein nephritis (9.9%) made the majority of this group. In conclusion, our study demonstrated that PRNB is a safe procedure with usually transient complications showing the most frequent renal diseases that cause diagnostic and therapeutic difficulties for pediatric nephrologists.

Acute renal failure due to rhabdomyolysis in a child with McArdle disease.

Rhabdomyolysis induced acute renal failure (ARF) is relatively rare in children. We report an 8-year-old boy with McArdle disease and rhabdomyolysis induced ARF after heavy muscle work. Physical examination revealed generalized tenderness on his extremities. Laboratory examinations showed acute renal failure due to myoglobinuria and revealed alanine transaminase 428 U/l, aspartate transaminase 1,400 U/l, blood urea nitrogen 119 mg/dl, creatinin 3.6 mg/dl, uric acid 13 mg/dl, and serum creatinine kinase (CK) 33,766 U/l. Hemodialysis was carried out for ARF. His clinical and laboratory findings improved and became normal in 2 weeks. Enzymatic analysis of the muscle biopsy showed a phosphorylase A level of 129 nmol/s/mg protein (normal: 200-600) and a phosphorylase A+B level of 385 nmol/s/mg protein (normal: 500-1500), which was compatible with glycogenosis type V. As McArdle disease rarely becomes symptomatic and ARF secondary to this condition is very rare, our case represents a rare clinical presentation.