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U. Querfeld, A. Anarat,
AK Bayazit,
AS Bakkaloglu,
Y. Bilginer,
S. Caliskan,
M. Civilibal,
A. Doyon,
A. Duzova,
D. Kracht,
M. Litwin,
A. Melk,
S. Mir,
B. Sozeri,
R. Shroff,
R. Zeller,
E. Wuhl,
F. Schaefer,
Study 4C. Grp
Clinical Journal of the American Society of Nephrology. 01/2010; 5(9):1642-1648.
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E. Wuhl,
A. Peco-Antic,
A. Teixera,
A. Zurowska,
M. Litwin,
S. Picca,
G. Laube,
A. Trivelli,
G. Ardissino,
S. Emre, [......],
T. Urasinski,
A. Jankauskiene, A. Anarat,
A. Bakkaloglu,
J. Gellermann,
D. Drozdz,
S. Mir,
O. Mehls,
F. Schaefer,
ESCAPE Network
Pediatric Nephrology. 01/2009; 24(9):1795-1795.
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E. Wuhl,
A. Trivelli,
S. Picca,
M. Litwin,
A. Peco-Antic,
A. Zurowska,
S. Testa,
A. Jankauskiene,
S. Emre,
A. Caldas-Afonso, [......],
K. Arbeiter,
T. Neuhaus,
J. Gellermann,
D. Drozdz,
M. Fischbach,
K. Moller,
M. Wigger,
L. Peruzzi,
O. Mehls,
F. Schaefer
New England Journal of Medicine. 01/2009; 361(17):1639-1650.
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ABSTRACT: Pleural effusions are seen relatively common in end-stage renal disease (ESRD) patients, on the other hand, hydrothorax secondary to pleuroperitoneal leak in continuous ambulatory peritoneal dialysis (CAPD) patients is rare. Since treatment modalities differ widely from that of other causes of pleural effusion seen in CAPD patients such as uremia, volume overload, congestive heart failure, infection and malignancy, pleuroperitoneal leak should always be kept in mind in the differential diagnosis and should be excluded urgently. To draw attention to this point, in this paper, 2 children on CAPD who developed a hydrothorax secondary to a pleuroperitoneal communication are presented with an overview of diagnostic tools and treatment modalities.
Clinical nephrology 09/2008; 70(2):178-80. · 1.17 Impact Factor
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M. Chinali,
G. Simone de,
MC Matteucci,
S. Picca,
A. Mastrostefano, A. Anarat,
S. Qaliskan,
N. Jeck,
TJ Neuhaus,
A. Peco-Antic,
L. Peruzzi,
S. Testa,
O. Mehls,
E. Wuhl,
F. Schaefer,
ESCAPE Trial Grp
Journal of the American Society of Nephrology. 01/2007; 18:593-598.
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ABSTRACT: We herein report the results of intravenous pulse cyclophosphamide (IVCP) therapy of 5 patients with steroid-resistant focal segmental glomerulosclerosis (FSGS). All patients had been treated with oral and intravenous pulse methylprednisolone and failed to respond to steroids from onset and were considered as primary steroid-resistant. Before starting IVCP, all patients were also treated with other immunosuppressive drugs with or without steroids, but none of them responded to such therapies and no patient had any NPSH2 gene mutations.
IVCP was given monthly at a dose of 500 mg/m2 for 6 months. At the end of 6 months, IVCP was discontinued in case there was no response. Otherwise, IVCP was continued for every 2 months. Oral prednisone was given concurrently at 60 mg/m2 daily for 6 weeks and then 40 mg/m2 on alternate days for 4 weeks. Prednisone was then tapered to 10 mg/m2 alternate days and continued during the therapy period.
Only 1 of these patients achieved remission after IVCP while 4 patients showed no response to IVCP. 2 patients who did not achieve remission progressed to end-stage renal disease (ESRD) and 2 others who had not been treated with cyclosporine before underwent cyclosporine therapy. None of our patients has suffered from adverse effects of IVCP.
We found that IVCP had a limited beneficial effect in treatment of steroid-resistant FSGS and it may be suggested that IVCP can be tried to treat steroid-resistant patients, also for patients with primary steroid resistance and those who do not respond to other immunosuppressive therapies.
Clinical nephrology 02/2006; 65(1):7-12. · 1.17 Impact Factor
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ABSTRACT: Beckwith-Wiedemann syndrome is a somatic overgrowth syndrome characterized by a variable incidence of congenital anomalies, including hemihypertrophy, omphalocele, macroglossia and renal malformations. We report a child with Beckwith-Wiedemann syndrome and posterior urethral valves. Urethral valve resection was successfully performed under general anesthesia after voiding cystourethrography. This is the first report of Beckwith-Wiedemann syndrome associated with posterior urethral valves.
Genetic counseling (Geneva, Switzerland) 02/2005; 16(1):41-4. · 0.50 Impact Factor
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ABSTRACT: The aim of the present study is to report our clinical experiences with MMF in problematic children with chronic glomerulonephritis resistant to corticosteroids and/or other immunosuppressive drugs.
Ten patients with chronic glomerulonephritis resistant to treatment with corticosteroids and other immunosuppressive drugs were treated with mycophenolate mofetil (MMF). Causes of chronic glomerulonephritis were mesangial proliferative glomerulonephritis (4), membranoproliferative glomerulonephritis (3), chronic sclerosing glomerulonephritis (1), focal segmental glomerulosclerosis (1), diffuse endo- and extracapillary proliferative glomerulonephritis (1). MMF 15 mg/kg was used in combination with low-dose corticosteroids and angiotensin-converting enzyme inhibitors.
During 24 weeks of MMF therapy, no significant changes were detected in mean serum creatinine, albumin and proteinuria. Severe leukopenia was seen in 1 patient. Additional adverse effects, including nausea and diarrhea, were observed in another patient when the dosage was increased to 20 mg/kg per day. During MMF treatment proteinuria decreased slightly without remission in 6 of 10 patients.
Further data and clinical trials are needed to evaluate the possible role of MMF in the treatment of chronic glomerulonephritis of similar etiologies in pediatric patients.
Clinical nephrology 02/2004; 61(1):25-9. · 1.17 Impact Factor
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ABSTRACT: The aim of this study was to investigate the role of nephrocalcin in childhood urolithiasis. Forty-one patients with urinary stones and 25 age- and sex-matched healthy controls were admitted to the study. Blood and timed urine samples were taken from both patient and control groups for biochemical analysis. Serum and urine creatinine (Cr) and urinary nephrocalcin (NC) were measured. NC excretion was expressed as a NC/Cr (mg/g) ratio. NC-PreA/Cr and NC-D/Cr ratios were found to be significantly higher in patients than in the control group. No statistically significant differences were found in NC-A/Cr, NC-B/Cr, NC-C/Cr ratios between the patient and control groups. The high NC-PreA/Cr ratio (p = 0.012) observed in stone-forming patients indicates that this ratio may also be an important stimulatory factor for urinary stone disease.
Nephron Physiology 02/2003; 94(4):p59-61. · 2.55 Impact Factor
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ABSTRACT: PURPOSE: The aim of this study is to compare the results of direct radionuclide cystography (DRNC) and voiding cystourethrography (VCUG) in a group of children with a high suspicion of vesicoureteral reflux (VUR).
METHODS: For this purpose, 25 children were studied with both VCUG and DRNC. Among 50 ureter units able to be compared 39 ureter units did not show any VUR on either study. Eleven ureter units (10 children) had VUR either on one study or on both (VCUG and DRNC). In the children who had VUR on either study, a dimercaptosuccinic acid scintigraphy (DMSA) was performed to determine their cortical function.
RESULTS: We identified the following four patterns: 1) Five ureter units (five children) read positive on DRNC who were negative on VCUG and four of these children had positive findings on DMSA; 2) Four ureter units (four children) read positive on VCUG who were negative on DRNC, and two of them had positive findings on DMSA; 3) Two ureters (one child) read positive in both studies and also had abnormal DMSA findings; 4) Thirty-nine ureter units read as negative on both studies.
CONCLUSION: Although the results of these two methods did not show a significant difference, DRNC offers a high sensitivity in the younger age group whereas VCUG seems to be more sensitive in the older age group. DRNC also offers continuous recording during the study, ease of assessment and lower radiation dose to the gonads, which makes it a preferable method for the initial diagnosis and follow-up of VUR.
Annals of Nuclear Medicine. 01/2003;
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ABSTRACT: Technetium-99m dimercaptosuccinic acid (Tc-99m DMSA) as a static renal agent is currently the most frequently used agent in the detection of renal scarring, and allows accurate calculation of differential renal function (DRF). But this agent has some disadvantages such as relatively higher radiation dose and time consumption.
METHODS: The purpose of this study was to evaluate the potential of summed image that obtained from parenchymal phase of the dynamic technetium-99m-N,N-ethylenedicysteine (Tc-99m EC) scintigraphy in the detection of renal parenchymal defects and in the estimation of DRF, and to compare the results of this method with those of Tc-99m DMSA scintigraphy. The uptake ratios of the kidney to body background were also calculated for these two methods. Twenty-nine children with various renal disorders underwent both static Tc-99m DMSA and dynamic Tc-99m EC scintigraphy. The cortical analysis of Tc-99m EC scintigraphy was performed on the summed image obtained from dynamic images using the time interval between the first 45-120 sec.
RESULTS: There was a very close correlation between these two methods with respect to DRF (r = 0.99). In the detection of renal parenchymal lesions, scintigraphy with Tc-99m DMSA detected more lesions, and the sensitivity and specificity of the summed Tc-99m EC images were calculated as 92.6% and 100%, respectively. In addition, the ratios of mean uptake values for Tc-99m DMSA and Tc-99m EC images were 7.59 +/- 2.17 and 2.95 +/- 0.91, respectively. This ratio of Tc-99m EC seems to be acceptable and allows good delineation of the kidneys. But, the main disadvantages of the summed Tc-99m EC images in comparison with static Tc-99m DMSA images are the use of only posterior projection that may be an important drawback in patients with abnormal kidney positions, lower image counts and higher pixel size because of dynamic acquisition.
CONCLUSION: These results show that summed Tc-99m EC images with an acceptable high image contrast provide an accurate DRF calculation in patients without abnormal kidney positions and allow the detection of most renal parenchymal abnormalities. However, Tc-99m DMSA scintigraphy remains the gold standard method because of its well known advantages.
Annals of Nuclear Medicine. 01/2003;
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ABSTRACT: Patients with sickle cell anemia have various forms of renal dysfunction.
The purpose of this study is to demonstrate the abnormalities of HbSS patients' renal function in childhood. Renal function studies were performed in 55 patients with homozygote sickle cell anemia and compared with 13 healthy children. The blood and timed urine samples were obtained for hematological and biochemical determinations.
Mean serum creatinine, sodium, phosphorus and calcium levels were not statistically different between patients and controls. Mean serum potassium and uric acid levels were significantly higher in patients than in controls. Mean tubular phosphate reabsorption (p < 0.001) and fractional excretion of potassium (p < 0.05) were lower in patients than in the control. There were no significant differences in fractional excretion of sodium and uric acid between patients and controls. Patients had significantly higher urine pH and significantly lower specific gravity and osmolality than controls. Also, there were no significant differences in urinary protein/ creatinine, urinary N-acetyl-beta-D-glucosaminidase/creatinine and urinary malondialdehyde/creatinine between patients and controls.
Thus, significant proximal tubular dysfunction is not a common feature but distal tubular abnormality is the most consistent renal functional derangement of patients with SCA in childhood.
Clinical nephrology 02/2002; 57(2):127-30. · 1.17 Impact Factor
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ABSTRACT: To investigate the effects of valproic acid (VPA) on renal tubular function, we examined 15 ambulatory children with epilepsy who received VPA for at least 6 months. None of the patients had mental retardation. Fourteen age- and sex-matched children were used as a control group. No statistically significant differences were found between patients and control subjects with respect to blood urea nitrogen (BUN), creatinine (Cr), uric acid, creatinine clearance (Ccr), tubular reabsorption of phosphorus (TRP), urinary Ca:creatinine ratio, urinary pH and mean urinary beta2-microglobulin concentrations (P>0.05). Protein and glucose in patient urine samples were negative. Urine microscopic examinations and amino acid chromatographies of patients were also normal. However, significant differences were found between patient and control groups with respect to mean urinary N-acetyl-beta-D-glucosamine:creatinine ratio (NAG:Cr) and mean urinary malondialdehyde:creatinine (MDA:Cr) ratio (P<0.05). In conclusion, ambulatory children with epilepsy taking VPA therapy may develop proximal renal tubular dysfunction. Although this finding is clini-cally insignificant, it should be kept in mind during VPA therapy.
Pediatric Nephrology 03/2001; 16(3):256-9. · 2.52 Impact Factor
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Nephron 01/2001; 86(4):517. · 13.26 Impact Factor
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ABSTRACT: In patients with beta-thalassemia major, the most important cause of mortality and morbidity is organ failure due to deposits of iron. In this study, the nature of the kidney injury and possible pathogenetic factors were investigated. Seventy children with beta-thalassemia major and 14 age and sex-matched healthy children were involved in the study. Blood and timed urine samples were obtained for hematological and biochemical tests. The mean values of blood urea nitrogen (BUN), serum creatinine, creatinine clearance, serum sodium, urine osmolality, fractional excretion of sodium, potassium, and uric acid were not statistically different between the groups. Serum levels of potassium, phosphorus, and uric acid and the urine volume, high urinary protein to creatinine (UP/Cr), urinary N-acetyl-beta-D-glucosaminidase to creatinine (UNAG/Cr), and urinary malondialdehyde to creatinine, (UMDA/Cr) and the tubular phosphate reabsorption (TRP) values were statistically different between two groups (P<0.05). Increased serum levels of potassium, phosphorus, and uric acid in the patient group were attributed to the rapid erythrocyte turnover. The presence of high UP/cr, UNAG/Cr and UMDA/Cr ratios shows that in these patients with proximal renal tubular damage may be secondary to oxidative lipid peroxidation mediated by the iron overload.
Pediatric Nephrology 12/2000; 15(1-2):109-12. · 2.52 Impact Factor
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ABSTRACT: In patients with β-thalassemia major, the most important cause of mortality and morbidity is organ failure due to deposits
of iron.. In this study, the nature of the kidney injury and possible pathogenetic factors were investigated. Seventy children
with β-thalassemia major and 14 age and sex-matched healthy children were involved in the study. Blood and timed urine samples
were obtained for hematological and biochemical tests. The mean values of blood urea nitrogen (BUN), serum creatinine, creatinine
clearance, serum sodium, urine osmolality, fractional excretion of sodium, potassium, and uric acid were not statistically
different between the groups. Serum levels of potassium, phosphorus, and uric acid and the urine volume, high urinary protein
to creatinine (UP/Cr), urinary N-acetyl-β-d-glucosaminidase to creatinine (UNAG/Cr), and urinary malondialdehyde to creatinine, (UMDA/Cr) and the tubular phosphate reabsorption (TRP) values were statistically different between two groups (P<0.05). Increased serum levels of potassium, phosphorus, and uric acid in the patient group were attributed to the rapid erythrocyte
turnover. The presence of high UP/Cr, UNAG/Cr and UMDA/Cr ratios shows that in these patients with proximal renal tubular damage may be secondary to oxidative lipid peroxidation mediated
by the iron overload.
Pediatric Nephrology 09/2000; 15(1):109-112. · 2.52 Impact Factor
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Nephron 01/1999; 80(4):484. · 13.26 Impact Factor
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ABSTRACT: The purpose of this study was to investigate the acute changes in endothelin (ET) levels immediately after hemodialysis and to determine whether these changes vary with the use of different membranes and hemodialysis solutions. Ten children were included in the study. Three different hemodialysis sessions were performed on all patients: session 1, acetate-based dialysate and polycarbonate membrane; session 2, bicarbonate-based dialysate and polycarbonate membrane; session 3, acetate-based dialysate and polysulfone membrane. In all cases blood samples were obtained before and after dialysis. Pre- and post-hemodialysis ET levels of the patients with acetate-based dialysate and polycarbonate membrane were 33.68 +/- 11.51 pg/ml and 28.27 +/- 12.85 pg/ml, respectively. The fall in ET levels after this session was statistically significant (P = 0.015). We did not observe a statistically significant change in ET levels in the other sessions. Post-dialysis mean arterial pressure values were significantly lower than the pre-dialysis values in all three dialysis sessions (P < 0.01). A positive correlation was observed between plasma ET levels and blood urea nitrogen and serum potassium; a negative correlation was observed between plasma ET levels and hematocrit.
Pediatric Nephrology 02/1998; 12(2):153-6. · 2.52 Impact Factor
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Nephron 01/1998; 78(3):356-7. · 13.26 Impact Factor
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ABSTRACT: We evaluated the clinical usefulness of 99Tcm-N,N-ethylenedicysteine (99Tcm-EC), a new renal tubular agent, in normal children and in children with various renal disorders and compared it with 99Tcm-mercaptoacetyltriglycine (99Tcm-MAG3). In a first group of children (Group 1), which included 15 patients and 4 normal children, both 99Tcm-EC and 99Tcm-MAG3 renal scintigraphy were performed within a period of 1-5 days. In a second group (Group 2), consisting of 12 patients, only 99Tcm-EC scintigraphy was performed. In the normal children, renograms, differential renal function (DRF) ratios and semi-quantitative parameters of the two agents were similar. For 99Tcm-EC and 99Tcm-MAG3, the mean (+/- S.D.) time to peak activity (Tmax) values were 3.2 +/- 0.5 and 3.1 +/- 0.4 min respectively, and the mean time from peak activity to 50% activity (T1/2) values were 6.3 +/- 0.5 and 6.4 +/- 0.4 min respectively. The mean parenchymal transit time index (PTTI) and mean whole-kidney transit time index (WKTTI) for 99Tcm-EC were 1.7 +/- 0.3 and 2.8 +/- 0.4 respectively, and for 99Tcm-MAG3 they were 1.8 +/- 0.2 and 3.0 +/- 0.3 min respectively. There were also no significant differences between these parameters for the two agents in children with various renal disorders, and the correlation coefficients (r) for DRF, Tmax, T1/2, PTTI and WKTTI were 0.99, 0.98, 0.94, 0.77 and 0.63 respectively. We conclude that 99Tcm-EC has excellent imaging characteristics in children, and even has some advantages over 99Tcm-MAG3. We suggest this agent can be used routinely with children.
Nuclear Medicine Communications 02/1997; 18(1):44-52. · 1.40 Impact Factor
