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ABSTRACT: OBJECTIVE: To evaluate the clinical properties of patients with nutcracker syndrome (NCS). MATERIALS AND METHODS: The medical records of the patients with NCS were retrospectively investigated, and the patients' sex, age, presenting symptoms, urinalysis findings, associated diseases, and body mass indexes were recorded. RESULTS: A total of 23 nonconsanguineous patients (11 male and 12 female patients, mean age 141 ± 36 months). Two patients had posterior NCS. Seventeen patients presented with 1 or >1 symptom. Fatigue and lassitude (n = 1), flank pain (n = 8), abdominal pain (n = 7), varicocele (n = 2), and macroscopic hematuria (n = 9) were the presenting symptoms. Only 6 patients had received a diagnosis of NCS from the urinalysis findings, which revealed microscopic hematuria or non-nephrotic proteinuria. The urinalysis findings disclosed isolated proteinuria in 6, hematuria plus proteinuria in 8, and macroscopic hematuria in 9 patients. Orthostatic proteinuria was detected in 7 patients. In patients with regressed hematuria and proteinuria, the body mass index was markedly increased at the end of the follow-up period relative to at diagnosis (at diagnosis 16.9 ± 4.0 kg/m(2); at the end of the follow-up period 18.6 ± 4.6 kg/m(2); P = .028). CONCLUSION: The diagnosis of NCS should be considered in the presence of symptoms such as proteinuria and hematuria and should be absolutely ruled out before attempting renal biopsy. An apparent correlation between an increased body mass index and regression of symptoms was seen.
Urology 06/2013; · 2.43 Impact Factor
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ABSTRACT: OBJECTIVE: To evaluate the factors associated with compensatory hypertrophy in the functional kidneys of children. METHODS: The medical files of patients with a solitary functional kidney were reviewed retrospectively. Data regarding anthropometric measurements, functional renal length, functional renal length of standard deviation score (SDS) of functional kidney at diagnosis, and end of follow-up were obtained. Patients were divided into 2 groups, those with a unilateral kidney function of <10% (hypoplasia, dysplasia, and atrophy, group 1) and those with a solitary kidney (agenesis, and multicystic dysplastic kidney, group 2). RESULTS: A total of 126 patients (70 boys) were evaluated. Both the sizes of the functional kidney and functional kidney SDS values at diagnosis were greater in group 1 relative to group 2. At the end of the follow-up period, anthropometric values including functional kidney size were higher in group 2. Functional kidney size of 2 SDS above the normal was mostly predictive at age 17.5 months (odds ratio [OR] 5.06) and at a body height of 82 cm (OR 5.57). CONCLUSION: The most determining factors for renal length SDS values were age and height. Solitary kidneys complete compensatory hypertrophy by 17.5 months of age, and after that their growth continues in parallel to normal growth.
Urology 05/2013; · 2.43 Impact Factor
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ABSTRACT: Squamous metaplasia is a proliferative lesion characterized by the replacement of the transitional epithelium with stratified squamous cells. In the urinary system, it is mostly seen in the bladder. It can be nonkeratinized or keratinized. We report the cases of 2 adolescent girls with keratinizing metaplasia, 1 of whom presented with difficulty with indwelling catheterization and 1 with final terminal hematuria. The predisposing factors were recurrent urinary tract infection and additional catheterization in 1 of the patients. The diagnosis was confirmed by histologic examination in both patients. We report on these cases to draw attention to this rare entity in children.
Urology 05/2012; 80(1):207-9. · 2.43 Impact Factor
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Pediatric Nephrology 10/2011; 27(5):761-2, 763-4. · 2.52 Impact Factor
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Pediatric Nephrology 10/2011; · 2.52 Impact Factor
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Alper Soylu,
Halil Ateş,
Sultan Cingöz, Mehmet Türkmen,
Belde Kasap Demir,
Mehmet Tunca,
Meral Sakızlı,
Mustafa Cirit,
Rıfkı Ersoy,
Ayfer Ulgenalp,
Salih Kavukçu
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ABSTRACT: Amyloidosis is the major complication of familial Mediterranean fever (FMF). Toll-like receptors (TLR) are involved in the activation of an innate immune system TLR-2 and TLR-4 recognize lipoteichoic acid and lipopolysaccharides (LPS), respectively. While TLR-2 Arg753Gln polymorphism upregulates, TLR-4 Asp299Gly and Thre399Ile polymorphisms downregulate inflammation. We investigated the effect of these polymorphisms on the development of amyloidosis in FMF patients. We also investigated myeloid cell TLR-2 and TLR-4 expressions in these patients. We studied 26 FMF patients and 13 FMF patients with amyloidosis. TLR-2 Arg753Gln and TLR-4 Asp299Gly and Thr399Ile polymorphisms were analyzed with the polymerase chain reaction-restriction fragment length polymorphism method. Myeloid cell baseline TLR-2 and TLR-4 and LPS-induced TLR-4 expressions were evaluated. The TLR-2 and TLR-4 polymorphism rate was compared with the results of 100 healthy subjects in our previous study. In addition, 13 healthy controls were enrolled for leukocyte TLR-2 and TLR-4 expressions. Serum amyloid A (SAA) levels were measured in these 13 control cases and in FMF patients during attack-free periods. The frequency of TLR-2 Arg753Gln, TLR-4 Asp299Gly, and Thr399Ile polymorphisms in healthy controls in our previous study were 1%, 3%, and 2%, respectively. The frequency of these polymorphisms were not different in FMF patients (with or without amyloidosis) compared to the control group. Likewise, myeloid cell TLR-2 and TLR-4 expressions were not different among the controls and FMF patients. However, LPS-induced TLR-4 expression in granulocytes was more prominent in FMF patients. There was no correlation between TLR-2 and TLR-4 expressions and SAA levels. Neither myeloid cell TLR-2 and TLR-4 expressions nor TLR-2 Arg753Gln, TLR-4 Asp299Gly, and Thr399Ile polymorphisms seem to affect the development of secondary amyloidosis in FMF patients in our study population.
Inflammation 10/2011; 34(5):379-87. · 1.75 Impact Factor
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ABSTRACT: Lactococcus lactis cremoris infections are very rare in humans. It is recognized as a commensal organism of mucocutaneous surfaces of cattle, and is occasionally isolated from human mucocutaneous surfaces. We report a brain abscess caused by L. lactis cremoris in an immunocompetent child. A 19-month-old female patient was admitted with fever and vomiting. Brain computed tomography (CT) revealed brain abscess. L. lactis cremoris was isolated from culture of the abscess material. The patient was treated with pus drainage from brain abscess and antibiotics including vancomycin and meropenem. The patient recovered completely. To our knowledge, this is the first report of a L. lactis cremoris infection in children.
European Journal of Pediatrics 09/2011; 170(12):1603-5. · 1.88 Impact Factor
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ABSTRACT: The diagnosis of chyloperitoneum (CP) is based on the presence of high levels of triglycerides (TGs) in the dialysate. It is a rare complication of peritoneal dialysis (PD) and even rarer in neonates. We report here the case of CP in a 1700-g male baby delivered at the 30th gestational week due to posterior urethral valve and associated oligohydramnios. On postnatal day 2, the serum creatinine (Scr) was 1.6 mg/dL, and he was anuric. PD was instituted via a Tenckhoff catheter. At the end of the second week, after the initiation of enteral feeding, the ultrafiltrate became cloudy, with a leukocyte count of 900/mm(3). A treatment regimen consisting of intraperitoneal vancomycin and ceftazidime was then started. Five days later, the fluid became milky, with a TG level of 251 mg/dL. The patient was then placed on a diet based on medium-chain triglycerides and octreotide (1 microg/kg/h; increasing up to 2 microg/kg/h over 15 days). Although the TG and leukocyte levels decreased, the milky appearance persisted. PD was stopped for 2 days when the Scr decreased to 1.7 mg/dL. When it was resumed, the fluid was totally clear, with a TG level of 7 mg/dL. The infant was discharged with a nightly intermittent peritoneal dialysis program and has had no recurrence. In summary, we report a preterm infant who developed CP during PD and recovered following treatment that included diet modification, octreotide, and temporary discontinuation of the PD.
Pediatric Nephrology 11/2009; 25(2):363-6. · 2.52 Impact Factor
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ABSTRACT: Infections may trigger or aggravate glomerulonephritidis and renal vasculitis like Henoch Schonlein purpura (HSP). HSP is seen more frequently in patients with familial Mediterranean fever in which TLR-2 Arg753Gln polymorphism frequency is increased. Although renal involvement is the most important factor affecting the prognosis in HSP, it is not known which patients will have renal disease or why some patients have severe renal involvement while some others have mild renal disease. We investigated the role of TLR-2 and TLR-4 polymorphisms on the incidence and severity of renal involvement in HSP patients. We studied HSP patients with and without nephritis (n = 15 for each group) and healthy controls (n = 100). TLR-2 Arg753Gln and TLR-4 Asp299Gly/Thr399Ile polymorphisms were analyzed with polymerase chain reaction-restriction fragment length polymorphism method. The frequency of TLR-2 Arg753Gln, TLR-4 Asp299Gly, and Thr399Ile polymorphisms in healthy controls were 1, 3, and 2%, respectively. The frequencies of these polymorphisms were not different in HSP patients with or without nephritis compared to healthy controls. TLR-2 Arg753Gln, TLR-4 Asp299Gly, and Thr399Ile polymorphisms are not increased in HSP or HSP nephritis patients.
Rheumatology International 08/2009; 30(5):667-70. · 1.88 Impact Factor
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ABSTRACT: Linear scleroderma is the most common type of juvenile localized scleroderma, which usually involves the limbs. The linear lesions may extend through the dermis, subcutaneous tissue, muscles, and the underlying bone, leading to significant functional and cosmetic deformities. Renal involvement in juvenile localized scleroderma is a rare manifestation. Here we report two cases with juvenile linear scleroderma and unique renal manifestations, including unilateral renal arterial stenosis and immunoglobulin M nephropathy.
Pediatric Nephrology 06/2009; 24(10):2041-5. · 2.52 Impact Factor
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ABSTRACT: A 16-year-old boy presented with acute kidney injury (AKI) which was attributed to chronic heavy cola consumption. Habitual heavy cola ingestion might lead to hypokalemic rhabdomyolysis by its glycyrrhizin content. AKI has been described rarely in association with this clinical picture. It is important for physicians to keep heavy cola and other soft drink consumption in mind as a cause for hypokalemic rhabdomyolysis and AKI.
European Journal of Pediatrics 04/2009; 169(1):107-11. · 1.88 Impact Factor
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ABSTRACT: Familial Mediterranean fever (FMF) is an autosomal recessive disorder characterized by recurrent attacks of fever and polyserositis. It is the most frequent periodic fever syndrome. In FMF, sterile peritonitis, pleuritis and arthritis are frequently seen in addition to recurrent febrile attacks. Skin and muscle involvement is less common. Here, we report four patients presented with skin lesions or myalgia. Most striking findings in those patients are the absence of other major criteria for FMF and dominancy of skin lesions or myalgia. All four patients had MEFV gene mutations on both alleles. In patients with erysipelas-like lesions or erythema nodosum along with arthritis/arthralgia or recurrent myalgia, FMF should be kept in mind.
Clinical Rheumatology 03/2009; 28(7):857-60. · 2.00 Impact Factor
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ABSTRACT: Immunoglobulin A nephropathy (IgAN) is associated with mucosal IgA defect. Probiotics regulate specific and innate immunity. We evaluated the effect of Saccharomyces boulardii on experimental IgAN in mice. Four groups of BALB/c mice (eight for each) were formed. Group 1 was immunized by oral poliovirus vaccine (OPV) at 0, 14, and 28 days. Group 2 was also given S. boulardii in addition to OPV. Group 3 was given only S. boulardii, whereas group 4 received no treatment. At week 6, after urine and serum samples were obtained for urinalysis and serum creatinine and IgA measurements, all animals were sacrificed to get their kidneys for histopathological evaluation. Urinalysis and serum creatinine levels were normal in all groups. Serum IgA level was increased only in group 1. Whereas group 1 had mesangial proliferation, histology was normal in the other groups. Predominant IgA deposition was universal in group 1, whereas it was either not present or minimal in other groups. Three mice in group 1 also had C3 deposition, which was absent in other groups. Electron microscopy revealed mesangial proliferation, matrix expansion, focal glomerular basement membrane thickening and electron-dense deposits in group 1 only, whereas the other groups were normal. In conclusion, enteral S. boulardii prevented OPV-induced IgAN in mice.
Pediatric Nephrology 09/2008; 23(8):1287-91. · 2.52 Impact Factor
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ABSTRACT: We evaluated the effect of attack frequency, homozygosity for the M694V mutation and colchicine treatment on growth in children with familial Mediterranean fever (FMF). Prepubertal patients with FMF (19 M, 14 F) were evaluated retrospectively for height SDS, weight SDS and body mass index (BMI) before and after 46.2 +/- 39.8 months of colchicine therapy. Pretreatment attack frequency and acute phase markers at diagnosis were also recorded. While acute phase markers were not correlated to anthropometric variables, attack rate was negatively, albeit insignificantly, correlated to height and weight SDS. Height SDS did not change, while BMI showed a slight but significant increase during colchicine therapy (16.2 +/- 2.6 to 17.3 +/- 3.1 kg/m2, p = 0.035). Homozygosity for M694V did not affect time from the onset of symptoms to diagnosis, anthropometric variables and acute phase markers. In conclusion, pre-treatment attack rate and anthropometric development correlated negatively. Colchicine therapy improved BMI slightly, but significantly. Homozygosity for M694V had no effect on anthropometric development.
Journal of pediatric endocrinology & metabolism: JPEM 09/2008; 21(8):789-92. · 0.88 Impact Factor
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ABSTRACT: The aim of our study was to determine the effect of recombinant human erythropoietin (rhEPO) on cyclosporine (CsA) nephrotoxicity. Twenty-six female Wistar rats were injected with 15 mg/kg subcutaneous CsA and intraperitoneal saline/rhEPO for 28 days. Four groups were formed: Group 1 (n = 5), a control group; Group 2 (n = 7), CsA + saline; Group 3 (n = 7), CsA + low dose (20 U/kg per day) rhEPO; Group 4 (n = 7), CsA + high dose (100 U/kg per day) rhEPO. Body weights, creatinine clearance, urinary protein/creatinine, hematocrit, serum creatinine levels, histopathological parameters, apoptosis and lipid peroxidation tests were compared between the three groups. Body weights and renal functions were similar in Groups 2, 3 and 4 rats but significantly lower than the values found for the control group at the end of the study. The hematocrit was significantly different between the four groups, showing a positive association with the strength of the injected rhEPO doses. Tubular and arteriolar damage was significantly lower in Groups 3 and 4 rats than in Group 2 rats, while chronic changes were similar between the three groups. TUNEL-positive cells and thiobabarbituric acid reacting substance (TBARS) levels were significantly higher in Group 2 rats, whereas superoxide dismutase levels were significantly lower in Group 2 rats than in those of the other three groups. Low or high dose rhEPO had no significant protective effects on body weight, renal functions, chronic fibrotic changes, but both doses reduced tubular and arteriolar changes, apoptotis and oxidative stress.
Pediatric Nephrology 08/2008; 23(11):1991-9. · 2.52 Impact Factor
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ABSTRACT: We evaluated the predictors of renal scar in children with urinary tract infections (UTIs) having primary vesicoureteral reflux (VUR). Data of patients who were examined by dimercaptosuccinic acid (DMSA) scintigraphy between 1995 and 2005 were evaluated retrospectively. Gender, age, reflux grade, presence/development of scarring, breakthrough UTIs, and resolution of reflux, were recorded. The relation of gender, age and VUR grade to preformed scarring and the relation of gender, age, VUR grade, presence of preformed scarring, number of breakthrough UTIs and reflux resolution to new scarring were assessed. There were 138 patients [male/female (M/F) 53/85]. Multivariate analysis showed that male gender [odds ratio (OR) 2.5], age > or = 27 months in girls (OR 4.2) and grades IV-V reflux (OR 12.4) were independent indicators of renal scarring. On the other hand, only the presence of previous renal scarring was found to be an independent indicator for the development of new renal scar (OR 13.4). In conclusion, while the most predictive variables for the presence of renal scarring among children presenting with a UTI were male gender, age > or = 27 months in girls, and grades IV-V reflux, the best predictor of new scar formation was presence of previous renal scarring.
Pediatric Nephrology 07/2008; 23(12):2227-32. · 2.52 Impact Factor
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Seminars in arthritis and rheumatism 02/2008; 38(2):161-2. · 4.72 Impact Factor
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ABSTRACT: We investigated the question of whether IgM deposition causes any difference in the histomorphometry of children with mesangial proliferative glomerulonephritis (MePGN). We retrospectively compared the urinalysis, blood pressure measurements, and serum creatinine levels of children with IgM (+) and IgM (-) MePGN. For histopathological evaluation, the outlines of Bowman's capsules and glomerular tufts of 10 glomeruli were rimmed manually, and the surrounded areas were calculated as pixels. The cells at each tuft of the largest four glomeruli were counted. Bowman's capsular areas, glomerular tuft areas, glomerular cellularities, and the number of cells per tuft area were determined for each patient. There were 24 (M/F: 14/10) patients with MePGN. Twelve of them were IgM (+). Mean age at biopsy was 6.8+/-4.1 years. IgM (+) patients mostly presented with nephrotic syndrome (NS) (n=9) and IgM (-) patients mostly with macroscopic hematuria (n=7). None of the clinical or laboratory findings was statistically different between the groups (p>0.05) except increased NS frequency in IgM (+) patients (p: 0.038). The histomorphometric parameters were similar in both groups (p>0.05). IgM (+) patients with MePGN mostly presented with NS in our study, but their histomorphometric parameters demonstrated no significant difference.
Pathology - Research and Practice 01/2008; 204(3):149-53. · 1.21 Impact Factor
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ABSTRACT: Gut permeability to antigens is immature at birth, and while early administration of antigenic foods delays its maturation, breast-feeding accelerates it. We aimed to evaluate whether exposure to antigenic foods in early life is associated with a predisposition for immunoglobulin A nephropathy (IgAN). Three groups of children with IgAN (group 1), non-IgA glomerulopathies (group 2), and healthy controls (group 3) were formed. Parents filled out a questionnaire regarding gestational and postnatal ages, birth weight, and feeding by breast milk, formula, cow's milk, and complementary foods. All groups were similar for age, gender, birth weight, rate and duration of breast-feeding, and rate of formula feeding. Cow's milk consumption rate was higher in groups 1 and 2 than in group 3. Whereas introduction of formula was earlier in groups 1 and 2 than in group 3, feeding by cow's milk and weaning were earlier in group 1 than in the other groups. The respective best cutoff ages were 3.5 [odds ratio (OR) 28)], 3.75 (OR 5.7), and 5.5 (OR 10.5) months for formula, cow's milk, and complementary foods, respectively, for predicting the presence of IgAN. The results of this preliminary study indicate that early introduction of antigenic foods might increase the risk of future primary IgAN.
Pediatric Nephrology 08/2007; 22(7):1040-4. · 2.52 Impact Factor
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ABSTRACT: We aimed to determine the predictability of clinical and laboratory variables for vesicoureteral reflux (VUR) in children with urinary tract infection (UTI). Data of children with febrile UTI who underwent voiding cystoureterography between 2002 and 2005 were evaluated retrospectively for clinical (age, gender, fever > or = 38.5 degrees C, recurrent UTI), laboratory [leukocytosis, erythrocyte sedimentation rate (ESR), C-reactive protein (CRP), pyuria, serum creatinine (S(Cr))] and imaging (renal ultrasonography) variables. Children with VUR (group 1) vs. no VUR (group 2) and children with high-grade (III-V) VUR (group 3) vs. no or low-grade (I-II) VUR (group 4) were compared. Among 88 patients (24 male), 38 had VUR and 21 high-grade VUR. Fever > or = 38.5 degrees C was associated with VUR [odds ratio (OR): 7.5]. CRP level of 50 mg/l was the best cut-off level for predicting high-grade VUR (OR 15.5; discriminative ability 0.89 +/- 0.05). Performing voiding cystourethrography based on this CRP level would result in failure to notice 9% of patients with high-grade VUR, whereas 69% of children with no/low-grade VUR would be spared from this invasive test. In conclusion, fever > or = 38 degrees C and CRP > 50 mg/l seem to be potentially useful clinical predictors of VUR and high-grade VUR, respectively, in pediatric patients with UTI. Further validation of these findings could limit unnecessary voiding cystourethrography.
Pediatric Nephrology 06/2007; 22(6):844-8. · 2.52 Impact Factor
