Long-Term Follow-up Results of Renal Transplantation in Pediatric Patients With Focal Segmental Glomerulosclerosis: A Single-Center Experience.

INTRODUCTION AND AIM: Focal segmental glomerulosclerosis (FSGS) is a common cause of end-stage renal disease in children. We analyzed the long-term outcome of pediatric patients with FSGS undergoing renal transplantation. The objective of the study is to report the experience of a single center and determine the incidence of recurrence, rejection, graft loss, and related risk factors. MATERIALS AND METHOD: This retrospective cohort study was performed between 1991 and 2018. Thirty patients with a pathologic diagnosis of primary FSGS were included in the study. The patients were diagnosed with FSGS according to histologic features in biopsies. RESULTS: Twenty-one of the donors were deceased (70%) and 9 were alive (30%). FSGS recurred in only 2 patients. Graft loss occurred in 6 patients (20%). The causes of graft loss were chronic rejection in 4 patients and acute rejection in 2. Our graft survival rate was 100% at 1 year, 91% at 5 years, 80% at 10 years, 70% at 15 years, and 42% at 20 years. Five- and 10-year graft survival rates were 83% and 83% in living donors and 94% and 79% in deceased donors, respectively. According to Kaplan-Meier analysis, there was no statistically significant difference in terms of graft survival between living and deceased donors. CONCLUSION: This study, with its contribution to literature in terms of long follow-up of FSGS patients from childhood to adulthood, is important. However, further studies are required.

Inferior vena cava indices determine volume load in minimal lesion nephrotic syndrome.

The pathogenesis of edema in nephrotic syndrome has not been entirely understood. We investigated the value of the echographic parameters [inferior vena cava index (IVCI), inferior vena cava collapsibility index (IVCCI), and left atrium diameter (LAD)] to determine the volume load in children with minimal lesion nephrotic syndrome (MLNS). Twelve children with MLNS (seven boys, five girls) were included in this study. The patients were classified into three different stages (stage A: edematous; stage B: 50% decrease in weight gain; stage C: edema free) following measurement of their ideal weights. The ideal weight of patients in stage A was increased 13 +/- 7%. Serum total protein, albumin and urine sodium levels were found to be low in these patients. Plasma renin activity (PRA) and serum aldosterone levels in stage A were significantly different from those of the control group (P<0.05). PRA and serum aldosterone levels were not different from those of the control group in stage B (P>0.05). However, the increase in PRA was significant in stage C. Although a significant weight decrease was found in stages B and C, it had no effect on IVCI, LAD, and cardiothoracic index. We consider IVCI, IVCCI, and LAD measurements by echocardiography (ECHO) to be easy and reliable clinical methods for assessing the intravascular volume load in patients with MLNS.

Effect of enalapril on proteinuria, phosphaturia, and calciuria in insulin-dependent diabetes.

Elevated urinary calcium and phosphate excretion have been observed in children with insulin-dependent diabetes mellitus (IDDM). This may be related to a defect in tubular reabsorption. It is well known that converting enzyme inhibition decreases microalbuminuria and may prevent or retard diabetic nephropathy. We investigated whether enalapril also improves the defect in calcium and phosphate reabsorption. We studied 16 children and young adults (age 12-21 years) with IDDM and persistent microalbuminuria before and during 12 weeks of enalapril treatment. Before treatment microalbuminuria, urinary calcium excretion, and fractional tubular phosphorus reabsorption (TPR) were 153+/-53 microg/min, 5.5+/-0.9 mg/kg per day, and 71.4+/-3.6%, respectively. At the end of the 12th week, microalbuminuria had decreased to 20.3+/-7.9 microg/min and calcium excretion to 3.3+/-0.4 mg/kg per day (P<0.01), while the TPR increased to 80.1+/-3.8% (NS). The renal threshold phosphate concentration increased from 1.8+/-0.15 to 2.92+/-0.23 mg/dl (P<0.01). The fasting serum glucose and hemoglobin Alc levels did not change significantly during the study. Systolic and diastolic blood pressures were 120.4+/-2.2 / 79.3+/-1.4 mm Hg and 110.5+/-1.8 / 71.3+/-0.9 mm Hg before and after 12 weeks, respectively. We conclude that enalapril treatment improves not only microalbuminuria but also abnormal calcium and phosphate excretion in microalbuminuric children with IDDM.

The role of PAF and leukotrienes in bioincompatibility of cuprophane membranes in hemodialysis.

Inflammatory lipid mediators, PAF and leukotrienes (LTs), are thought to have an important role in biocompatibility in hemodialysis. PAF, LTB4 and LTC4 were studied both in controls (n: 12) and in 11 children on regular hemodialysis (150 minutes) with cuprophane dialyzers. Blood samples were collected initially (0'-precapillary), at first minute (1'-postcapillary) and at one hour after the hemodialysis sessions (210'-venous). Presence of LTs and high levels of PAF in 0' samples compared to levels in controls and significant increases in 1' samples suggested the alterations in PAF and LTs likely originated from the peripheral leukocyte activation. In 210' samples, PAF and LTs levels were decreased but still higher than the levels in 0' samples. This study suggested that PAF and LTB4 may be the control elements in biocompatibility in hemodialysis with cuprophane membranes, and demonstrated that the effects of activation last until the following session.

Plasma and urinary platelet activating factor concentrations and leukotriene releasing activity of leukocytes in steroid sensitive nephrotic syndrome of childhood.

Platelet activating factor (PAF) is synthesized and secreted by glomerular mesangial and endothelial cells. It increases glomerular basement membrane permeability and induces proteinuria. Leukotrienes (LT) are mediators released by either leukocytes or glomerular cells under the PAF effect. The possible role of PAF in steroid sensitive nephrotic syndrome (SSNS) of childhood was studied in 8 children with SSNS in the acute stage, 5 children in remission and 8 healthy controls. The PAF concentrations in urine and plasma were determined. Leukocytes were stimulated in vitro and the LT release in response to stimulation was determined. The urinary and plasma concentrations of PAF were significantly higher in the acute phase than in remission and in control patients. Children with SSNS were found to have peripheral leukocytes with increased LT releasing activity in vitro. These results are in accordance with clinical and experimental observations indicating that PAF originates in the kidney and plays a role in normal kidney physiology. Urinary PAF concentrations may be related to proteinuria because they were strongly correlated in the present study. Elevated plasma PAF concentrations in the acute stage of SSNS could result from either its secretion from the circulating leukocytes or decreased acetyl hidrolase activity needed for its hydrolysis in plasma. The increased LT release in vitro suggests that these cells might have been activated by PAF secreted from glomeruli. It is proposed that PAF and different LT in systemic and glomerular circulation are important mediators in childhood SSNS.

IgA nephropathy occurring in two siblings of three families.

There have been suggestions in the literature that IgA nephropathy may be familial. Genetic factors may influence the development of disease in an association between HLA antigens and IgA nephropathy. At the present time, no conclusion can be drawn. Here, two siblings with typical IgA nephropathy in three families are presented. A relation between HLA and IgA nephropathy was not detected in these family studies. The first family involved a 14-year-old girl and her brother, who at the age of 12 years were admitted to the hospital with macroscopic hematuria. All of the investigations, including serum IgA levels (104 mg/dl and 102 mg/dl respectively) showed normal kidneys with IgA deposition in the mesangium of the glomeruli. In the second family, a 15-year-old boy and his brother at nine years of age were admitted with macroscopic hematuria and gross hematuria, respectively. Laboratory investigations were normal. The serum IgA level (287 mg/dl) was normal in the first patient but the second was elevated at 485 mg/dl. IgA deposits were observed in the glomerular mesangium in these patients. The third family consisted of a 15-year-old boy and his nine-year-old sister who were both admitted with microscopic hematuria. Serum IgA levels (193 and 131 mg/dl, respectively) and laboratory investigations were normal. Renal biopsy specimens revealed C3 and IgA depositions in the glomerular mesangium of both siblings. In the first family the patients were HLA identical, while in the others the siblings were one-haplotype identical. Although IgA nephropathy and HLA antigens are strongly associated in the literature, we could not find this association.

Image-directed and color Doppler ultrasonography in the diagnosis of postbiopsy arteriovenous fistulas of native kidneys.

Using image-directed and color Doppler ultrasonography (ICDUS), we examined 65 patients with single kidney biopsy and diagnosed one arteriovenous fistula (AVF) in each of 8 kidneys. Three of them were associated with pseudoaneurysms. Three of the patients with AVF who presented with macrohematuria underwent angiography. Therapeutic percutaneous embolization was performed in 2 of them. The remaining 6 patients were followed up with ICDUS. All the lesions had disappeared at the end of a 6-month period. We conclude that ICDUS is an easy and noninvasive imaging technique in the diagnosis of postbiopsy native renal AVFs.