Ayfer Gür Güven

Renal function after hematopoietic stem cell transplantation in children

The aim of this study was to assess glomerular and tubular renal function after HSCT in children in a prospective trial.

Mercury intoxication resulting from school barometers in three unrelated adolescents

Three adolescents with severe hypertension due to mercury intoxication are presented. Two of them had skin rash, signs and symptoms of central nervous system involvement, peripheral neuropathy and mild-to-moderate proteinuria in addition to hypertension. All three patients had a history of exposure to mercury, the source being broken barometers taken from school laboratories 2–4 months previously. Urine and blood mercury levels were consistent with mercury intoxication. The patients were treated with chelation therapy. One of them died; the others recovered over a period of 1–4 months. Conclusion:mercury intoxication should be considered in any child with signs and symptoms of hypertension, skin rash, peripheral neuropathy and behavioural changes. The parents and school administrators, as well as paediatricians, should be aware of the potential risks of mercury and should be encouraged to avoid mercury-containing devices in schools and households.

Evaluation of reproductive functions in male adolescents following renal transplantation

Abstract:  The aim of this study was to analyze the semen variables and hormone profiles in kidney transplanted male adolescents. Eight post‐pubertal male patients who underwent successful renal tx during the peripubertal period and who had ESRD during childhood were enrolled in the study. Patients who underwent tx before 14 yr old (group I) and patients who underwent tx after 14 yr old (group II) were evaluated separately. Semen was collected and analyzed. Serum levels of LH, FSH, and testosterone were measured and found to be normal in all patients except one. The mean age at the diagnosis of CKD was six yr and 13 yr in groups I and II, respectively. The mean age at the time of tx was 12 yr in the first and 17.8 yr in the second group. The patients in group I had received prednisone, cyclosporine A and azathioprine with a longer duration of time compared with patients in group II. Sperm counts (15.5 ± 15.7 vs. 82.3 ± 64.2 millions/mL) and sperm motilities (37.8 ± 30.9 vs. 57.8 ± 22.1%) were lower in group I than group II. Only one patient in group II had normal sperm parameters and azospermia was observed in one patient from group I. We conclude that the earlier onset and the longer duration of uremia, the more impairment of reproductive function. Also, it seems that duration of exposure to corticosteroids or cyclosporine combined with azathioprine contribute to sperm dysfunction in peripubertal transplanted boys.

Can serum cystatin C reflect the glomerular filtration rate accurately in pediatric patients under chemotherapeutic treatment? A comparative study with Tc-99m DTPA two-plasma sample method.

ObjectiveIt was assessed whether cystatin C (cysC) could be used as a marker of glomerular filtration rate (GFR) by considering the technetium-99m diethylenetriamine penta-acetate (Tc-99m DTPA)-two blood sample method (GFRTc-99m DTPA) as the reference in pediatric patients under chemotherapeutic treatment. MethodsThe chemotherapy group (CG) consisted of 31 patients (21 females, 10 males median age: 8.2 years; range: 2–16 years) who had been planned to receive allogenic hematopoietic stem cell transplantation. All patients in the CG received conditioning regimen (includes chemotherapy protocol) before hematopoietic stem cell transplantation. In addition, 21 patients (14 females, seven males median age: 9.5 years; range: 4–16 years) without any chemotherapy (nonchemotherapy group: nCG) were also prospectively investigated. Serum cysC, serum creatinine, GFRTc-99m DTPA, and GFR with a cysC-based formula (GFRcysC) were analyzed. Tubular function was also assessed. ResultsAlthough we found good correlation between GFRTc-99m DTPA and cysC (r = −0.78), GFRTc-99m DTPA and GFRcysC (r = 0.91), cysC and creatinine (r = 0.91) in nCG, the same correlations were poor in CG (r = −0.42, r = 0.43, r = 0.46, respectively). Tubular function was impaired after chemotherapy. Bias±1.96 SD values were −6±15.7 and −3±54.8 ml/min/1.73 m2 in nCG and CG, respectively. Precision was also better in nCG (10 ml/min/1.73 m2) than in CG (27.6 ml/min/1.73 m2). ConclusionSerum cysC and GFRcysC cannot reflect GFR accurately in pediatric patients under chemotherapeutic treatment. Tubular cell damage induced by chemotherapeutics could be a responsible factor through the impairment of tubular absorption and metabolism of cysC.

Value of the urine strip test in the early diagnosis of bacterial peritonitis

Abstract Background : In an effort to detect the presence of leukocytes in the peritoneal dialysate fluid (PDF) a urine dipstick may be practical for the early detection of peritonitis in peritoneal dialysis patients.

IgG and IgG subclasses deficiency in children undergoing continuous ambulatory peritoneal dialysis and its provocative factors.

Background : Low levels of serum IgG or IgG subclasses may be responsible for the defective peritoneal defense and for peritonitis attacks in continuous ambulatory peritoneal dialysis (CAPD) children. Malnutrition, peritoneal loss or frequent peritonitis may lead to IgG or IgG subclasses deficiency.

Case of Bartter Syndrome Presenting With Hypokalemic Periodic Paralysis

Hypokalemic periodic paralysis can occur secondarily to excessive potassium loss. Thyrotoxicosis, diuretic ingestions, hyperaldosteronism, barium poisoning, Gitelman syndrome, and Bartter syndrome are among the disorders causing secondary hypokalemic periodic paralysis. Clinical presentation of Bartter syndrome with hypokalemic periodic paralysis is rare. A 12-year-old boy was admitted to our hospital because of transient paralysis. He had been suffering from transient weakness attacks for 2 years and had had a total of 10 attacks, lasting 1 to 3 days. He had growth retardation, polyuria, and polydipsia. Laboratory examinations revealed hypokalemic alkalosis, normomagnesemia, hypercalciuria, and hyperaldosteronism. The clinical and laboratory findings were in accordance with Bartter syndrome. He has been followed up for 6 months and has suffered no further paralytic attacks under indomethacin therapy. This case highlights the importance of blood pH measurement in patients with hypokalemic periodic paralysis; it might prevent misdiagnosis and mismanagement in such diseases. (J Child Neurol 2006;21:255—256; DOI 10.2310/7010.2006.00049).

Severe lactic acidosis and nephrolithiasis in an infant--etiology?: type 1 glycogen storage disease (GSD).

A 4.5-month-old girl was admitted to the pediatric emergency room with dyspnea and tachypnea. Two weeks prior to admission the patient presented to a local hospital with restlessness and was diagnosed with a urinary tract infection; she later developed gastroenteritis. She was feeding with breast milk only. No history of increased susceptibility to infections was described. Her brother died because of sepsis and metabolic acidosis at the age of three months. There was also consanginuity between the parents. She exhibited Kussmaul breathing with a respiratory rate of 60/min, and tachycardia (176/min) without fever or any finding of heart failure. Height, weight and blood pressure percentiles were in the normal ranges. Marked hepatosplenomegaly was detected on physical examination. The eye examination was normal. Laboratory studies revealed blood pH of 7.05, pCO2 9.6 mm Hg, bicarbonate 2.4 mmol/L, base excess −25.1 mmol/L, sodium 143 mmol/L, potassium 3.9 mmol/L, chloride 94 mmol/L, anion gap 50.5 mmol/L, blood urea nitrogen 15 mg/dL, serum creatinine 0.44 mg/dL, total protein 4.6 g/dL and albumin 3.0 g/dL. Serum had lipemic appearance with a triglyceride level of 1988 mg/dL and cholesterol of 296 mg/dL, HDL 68 mg/dL; SGOT 570 U/L (0–50), SGPT 200 U/L (0–40), total bilirubin 0.15 mg/dL, calcium 9.7 mg/ dL, phosphorus 4.3 mg/dL, uric acid 17.9 mg/dL, ammonia 200 μg/dL, lactic acid 17.3 mmol/L (0.5–2.2), initial blood glucose 63 mg/dL (while hypoglycemia levels of 22 and 35 mg/dL glucose values were obtained during hospitalization period), hemoglobin 10.2 g/dl, WBC 12,200/mm with 56% lymphocyte, thrombocyte count 733,000 mm (control 133,000). Urine pH was 6, specific gravity 1.030; glucose, reducing substances and ketone bodies were negative. Urinary system ultrasonography demonstrated enlarged (80–81 mm) kidneys with normal echogenity and hyperdense areas of 7 mm diameter on left inferior region, 3 mm on middle calicea and 5 mm on right middle calicea; liver 115 mm, spleen 91 mm (Fig. 1). During follow-up she passed a stone, which revealed Weddellite (CaC2O4. 2.25 H20) upon infrared spectroscopy analysis. Amino acid/acylcarnitine profile tests by tandem mass spectrometry were found to be normal.

Renal functional reserve in insulin dependent diabetic children.

Abstract Background: Microalbuminuria has been shown to be predictive for clinical diabetic nephropathy. Renal functional reserve (RFR), as a response to protein loading in a short period of time, is a parameter to assess the ability of kidneys to increase the glomerular filtration rate (GFR). The aim of this study was to predict the early phase of diabetic nephropathy by measuring urinary albumin level and RFR capacity in patients with insulin‐dependent diabetes mellitus (IDDM).

Severe lactic acidosis and nephrolithiasis in an infant—etiology?: Answer

A 4.5-month-old girl was admitted to the pediatric emergency room with dyspnea and tachypnea. Two weeks prior to admission the patient presented to a local hospital with restlessness and was diagnosed with a urinary tract infection; she later developed gastroenteritis. She was feeding with breast milk only. No history of increased susceptibility to infections was described. Her brother died because of sepsis and metabolic acidosis at the age of three months. There was also consanginuity between the parents. She exhibited Kussmaul breathing with a respiratory rate of 60/min, and tachycardia (176/min) without fever or any finding of heart failure. Height, weight and blood pressure percentiles were in the normal ranges. Marked hepatosplenomegaly was detected on physical examination. The eye examination was normal. Laboratory studies revealed blood pH of 7.05, pCO2 9.6 mm Hg, bicarbonate 2.4 mmol/L, base excess −25.1 mmol/L, sodium 143 mmol/L, potassium 3.9 mmol/L, chloride 94 mmol/L, anion gap 50.5 mmol/L, blood urea nitrogen 15 mg/dL, serum creatinine 0.44 mg/dL, total protein 4.6 g/dL and albumin 3.0 g/dL. Serum had lipemic appearance with a triglyceride level of 1988 mg/dL and cholesterol of 296 mg/dL, HDL 68 mg/dL; SGOT 570 U/L (0–50), SGPT 200 U/L (0–40), total bilirubin 0.15 mg/dL, calcium 9.7 mg/ dL, phosphorus 4.3 mg/dL, uric acid 17.9 mg/dL, ammonia 200 μg/dL, lactic acid 17.3 mmol/L (0.5–2.2), initial blood glucose 63 mg/dL (while hypoglycemia levels of 22 and 35 mg/dL glucose values were obtained during hospitalization period), hemoglobin 10.2 g/dl, WBC 12,200/mm with 56% lymphocyte, thrombocyte count 733,000 mm (control 133,000). Urine pH was 6, specific gravity 1.030; glucose, reducing substances and ketone bodies were negative. Urinary system ultrasonography demonstrated enlarged (80–81 mm) kidneys with normal echogenity and hyperdense areas of 7 mm diameter on left inferior region, 3 mm on middle calicea and 5 mm on right middle calicea; liver 115 mm, spleen 91 mm (Fig. 1). During follow-up she passed a stone, which revealed Weddellite (CaC2O4. 2.25 H20) upon infrared spectroscopy analysis. Amino acid/acylcarnitine profile tests by tandem mass spectrometry were found to be normal.