Mustafa Koyun

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.

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).

Assessment of left ventricular function by tissue Doppler echocardiography in pediatric chronic kidney disease

Abstract Background: Cardiovascular (CV) disease remains the most common cause of mortality in chronic kidney disease (CKD). Methods: In this cross-sectional study, 43 pediatric patients with CKD were divided into two groups according to their estimated glomerular filtration rate (eGFR): groups 1 and 2 (eGR; 29–75 and 15–29 mL/min/1.73 m2, respectively). M – mode, conventional pulsed wave Doppler (cPWD) echocardiography and tissue Doppler imaging (TDI) were performed in all patients and 16 healthy controls. Maximal early (E wave) and late (A wave) diastolic flow velocities were assessed by cPWD. Using TDI, the early (E′) and late (A′) diastolic filling velocities were recorded. Early and late diastoles were evaluated using E′ values and E/E′ ratios, respectively. Results: Left ventricular hypertrophy (LVH) was determined in 19/43 (44.2%) patients. The E/E′ ratio was significantly higher in group 2 than in group 1 and controls. E/E′ was found to be positively correlated with left ventricular mass (LVM) index, and negatively with hemoglobin (Hb) levels. Low Hb levels were only independent predictor of E/E′ (p = 0.001, β: −0.470, 95% CI: −0.764; −0.196). E′ ratio was significantly lower in both patient groups compared to the controls. Conclusions: LVH and diastolic dysfunction are already present in early stages of CKD. Treatment of risk factors, such as anemia, is important to improve the clinical outcome.

Epstein-Barr virus infection in children with renal transplantation: 17 years experience at a single center

Abstract Objectives: The aim of this study was to detect the frequency, time of occurrence, management and outcome of Epstein–Barr virus (EBV) infection and related complications in pediatric renal transplant recipients. Methods: Pediatric renal allograft recipients transplanted between August 1994 and December 2011 at our hospital was evaluated retrospectively. The patients were divided into two groups; Groups 1 and 2 were composed of patients transplanted before and after November 2007, respectively, when plasma EBV DNA levels were periodically measured. Results: The study included 166 children, 89 (53.6%) boys, with a mean age of 12.2 ± 3.8 years. Prior to transplantation, 144 patients (86.7%) were EBV seropositive. Within a median follow-up period of 36 months, 11 of 22 seronegative children (50%) developed primary EBV infection. EBV reactivation was observed in 23 of 144 children (15.9%). Two patients with primary infection developed post-transplant lymphoproliferative disorder, one of whom died. Elevated serum creatinine levels or graft loss were not observed in any patient with EBV reactivation. Conclusions: EBV DNA monitoring by PCR in high-risk pediatric renal transplant recipients will provide early diagnosis and treatment of EBV infections.

Magnesium excretion and hypomagnesemia in pediatric renal transplant recipients

Abstract Background: We investigated magnesium excretion and rate of hypomagnesemia in pediatric renal transplant recipients. Method: The medical records of 114 pediatric renal transplant recipients were retrospectively evaluated. After exclusion of 23 patients, 91 patients were included in the study. We recorded serum magnesium levels at the time of measurement of urine magnesium wasting. Results: Mean serum magnesium levels were 1.73 ± 0.22 mg/dL and 38 of the patients (41%) had hypomagnesemia. There was a negative correlation between serum magnesium levels and estimated glomerular filtration rate and serum tacrolimus trough level (r = −0.215, p = 0.040 and r = −0.409, p = 0.000, respectively). Also, there was a statistically significant positive correlation between serum magnesium levels and transplantation duration (r = 0.249, p = 0.017). Mean fractional magnesium excretion was 5.9 ± 3.7% and 59 patients (65%) had high magnesium excretion. There was a significant negative correlation between fractional magnesium excretion and estimated glomerular filtration rate (r = −0.432, p = 0.001). There was a significant positive correlation between fractional magnesium excretion and serum creatinine (r = 0.379 p = 0.003). Conclusion: Patients with higher tacrolimus trough blood levels, lower glomerular filtration rate and at early posttransplant period had risk of hypomagnesemia.

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.

Urinary system obstruction in a preterm infant: Answers

1. The ultrasound (US) scan showed that both kidneys were large for his age (left kidney 72 mm and right kidney 73 mm), and it was difficult to differentiate cortex and medulla. Bilateral hydronephrosis, graded according to the Society for Fetal Urology, was grade 3, which means that the renal pelvis dilated beyond the sinus and calyces were uniformly dilated, with a renal pelvic anteroposterior diameter of 10 mm. Multiple echogenic particles within both renal pelvises without an acoustic shadow, compatible with bilateral renal fungus balls, were seen (Fig. 1). An antegrade pyelography revealed no passage of contrast agent to the ureter, due to obstruction of fungus balls (Fig. 2). The reason for acute kidney injury (AKI) in our case was bilateral urinary system obstruction caused by fungus balls. Both urine and blood cultures were positive for Candida albicans. A urine specimen taken from the renal pelvis via nephrostomy was also positive for Candida albicans. The patient had a history of intubation, central catheterization, total parenteral nutrition, longterm and broad-spectrum antibiotic usage in addition to prematurity and low birth weight, all of which are risk factors for the development of renal candidiasis. 2. The patient was administered intravenous furosemide and sodium bicarbonate at the emergency service; however, lung auscultation signs did not improve and his metabolic acidosis was resistant to medical treatment at the second hour following admission. So, it was decided to initiate renal replacement therapy. A peritoneal dialysis catheter was placed and dialysis was performed in the intensive care unit. At hour 16 following initiation of peritoneal dialysis, his rales completely disappeared and the acidosis was corrected. Due to the presence of bilateral urinary system obstruction, bilateral nephrostomy catheters were placed in order to provide urinary drainage, after which renal failure resolved rapidly.

Early Infantile Galactosialidosis Presenting with an Unusual Renal Involvement

Galactosialidosis is a rare lysosomal storage disease associated with deficiencies of beta-galactosidase and neurominidase. In this report, we present a 9-month-old early infantile Galactosialidosis infant with renal involvement. In the literature only isolated cases of Galactosialidosis with IgA nepropathy, renal insufficiency and renal transplantation reported. To the best of our knowledge, the patient is the first case reported in the literature in which steroid resistant nephrotic syndrome has been found in a Galactosialidosis patient.

A case report: hepatic posttransplant lymphoproliferative disorder in a non-liver transplant patient.

Posttransplant lymphoproliferative disorder (PTLD) is the most common malignancy in children after solid organ transplantation. We present a patient, who developed Epstein-Barr virus (EBV)-related PTLD in the liver after renal transplantation. A 10-year-old EBV-seronegative boy with cystinosis underwent a living related preemptive renal transplantation. He received antiviral prophylaxis with valacyclovir. At 5.5 months posttransplantation he displayed a primary EBV infection with an high fever, hepatosplenomegaly, monocytosis, and positive EBV DNA levels. Two months there after, a hypoechoic nodular 20-mm lesion in the left lobe of liver was detected on abdominal ultrasonography, performed because of anorexia and weight loss. EBV-DNA copy number was 7820 copies per milliliter. Liver biopsy showed a diffuse large B-cell lymphoma that was compatible with PTLD. We stopped all immunosupressive agents other than prednisolone. Chemotherapy consisting of two courses of cyclophosphamide, vincristine, prednisolone, and adriamycin was followed by rituximab. Within 2 months, the lesion resolved and within 18 months, he was free of disease.