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Abstract

A. Karabay Bayazit, A. Noyan, B. Aldudak, A. Özel, A. Anarat, Y. Kilinç, I. Sasmaz, E. Gali, R. Anarat and N. Dikmen

¹Department of Pediatric Nephrology, ²Department of Pediatrics,

³Department of Pediatric Hematology, Çukurova University School of Medicine, ⁴Antakya State Hospital, ⁵Biochemistry Laboratory, Adana Hospital, Baskent University, and ⁶Department of Biochemistry, Çukurova University School of Medicine, Balcali, Adana, Turkey
Background: Patients with sickle cell anemia have various forms of renal dysfunction. Subjects, materials and methods: 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. Results: 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-b-D-glucosaminidase/creatinine and urinary malondialdehyde/creatinine between patients and controls. Conclusion: 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.

Abstract

A.K. Bayazit, A. Noyan, N. Cengiz and A. Anarat

¹Department of Pediatric Nephrology, School of Medicine, Cukurova University, and ²Department of Pediatrics, Adana Hospital, Baskent University, Adana, Turkey
Aim: 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. Patients and methods: 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. Results: 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. Conclusion: 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.

Abstract

M. Buyukcelik, N. Cengiz, H. Dursun, M. Soran, A.K. Bayazit, A. Noyan and A. Anarat

¹Department of Pediatric Nephrology, Cukurova University, School of Medicine Adana, and ²Department of Pediatric Nephrology, Baskent University, Medical Faculty, Adana Research and Medical Training Hospital Center, Adana, Turkey
Aims: 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. Methods: 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. Results: 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. Conclusion: 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.Correspondence to:
M. Buyukcelik, MD Department of Pediatric Nephrology Cukurova University School of Medicine Balcali, Adana 01330, Turkey
Email: [email protected]

Abstract

B. Simsek, A. Noyan, A.K. Bayazit, M. Soran and A. Anarat

Department of Pediatric Nephrology, Cukurova University, School of Medicine, Balcali, Adana, Turkey
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.Correspondence to:
Dr. B. Simsek
Department of Pediatric Nephrology, Cukurova University, School of Medicine, Balcali, Adana, 01330 Turkey
Email: [email protected]

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