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.

Localized aggressive periodontitis in a patient with type 1 diabetes mellitus: a case report.

BACKGROUND: Poor metabolic control of diabetes mellitus (DM) has often been associated with the severity of periodontal disease. The aim of this report is to present a 9-year-old female with localized aggressive periodontitis who had a history of type 1 DM and the outcome of her treatment. METHODS: The patient had received medical, clinical, and radiographic periodontal examinations. Peripheral blood analysis was done as well. She had non-surgical periodontal treatment, and medical management of her diabetes was performed at the same time. She was followed longitudinally for 5 years. RESULTS: Medical examination revealed no pathological findings except for growth retardation. Laboratory tests showed that she had poor metabolic control, with 497 mg/dl fasting blood glucose and 15.6% HbA1c. The random migration and neutrophil chemotaxis were significantly reduced. Periodontal treatment and metabolic control of her diabetes resulted in significant improvement in her periodontal condition. No incipient periodontal breakdown was observed around the teeth after 5 years from baseline. CONCLUSIONS: This report proves the efficiency of periodontal therapy in the prevention of future periodontal breakdown in a systemically compromised patient. It seems that in certain individuals who are predisposed to the aggressive forms of periodontitis, clinical and medical examinations and intervention to the systemic condition, in combination with periodontal treatment, are important in the management of these individuals.

Flow-cytometric analysis of lymphocyte subsets and mCD14 expression in patients with various periodontitis categories.

BACKGROUND: Membrane-bound CD14 (mCD14) is expressed mainly on circulating monocytes and tissue macrophages. It is one of the receptors, which act at the recognition of lipopolysaccharides by host cells. Periodontopathic bacteria result in activation of cellular and humoral immune responses. AIM: The aim of the present study was to analyze the peripheral blood mCD14 concentrations as well as cell surface markers of lymphocyte subsets in periodontitis patients of various categories. MATERIALS AND METHODS: Peripheral blood samples were obtained from 22 early onset periodontitis (EOP), 10 adult periodontitis (AP) patients and 13 systemically and periodontally healthy control subjects. Three-color flow cytometry and a panel of relevant monoclonal antibodies were used to determine the percent expression of various cell surface markers on peripheral blood mononuclear cells (PBMCs). The results were tested statistically by one-way variance analysis and Newman Keuls test. RESULTS: No significant difference was observed between the study groups with regard to the relative counts of B-cells, T-cells, T-helper, T-cytotoxic/suppressor, activated T-cells and natural killer cells. EOP patients expressed significantly lower level of interleukin-2 receptor (IL-2R) when compared with AP patients (6.08% and 19.3% respectively) (p<0.05). The level of mCD14 in EOP patients (7.18%) was lower than that of AP patients (9.3%) and the control subjects (9.2%), but the differences were not statistically significant. CONCLUSIONS: The low level of IL-2R in the EOP group may be interpreted as an insufficient responsiveness to the periodontopathogens, which may be ultimately related with the more severe tissue destruction. Though not significant, the reduced expression of mCD14 in EOP group may also be related with the immune system deficiencies in these patients.

Phenotypic and functional analysis of peripheral blood mononuclear cells in generalised aggressive and chronic periodontitis patients.

BACKGROUND: The aetiology and pathogenesis of periodontal disease may involve dysfunctions in the cellular immune responses. The aim of the present study was to study the phenotypic and functional activities of peripheral blood mononuclear cells (PBMC) from 16 patients with generalised aggressive periodontitis (G-AP), 13 patients with chronic periodontitis (CP) and 20 periodontally healthy subjects (H). METHODS: The relative counts of CD3+, CD4+, CD8+ T cells, T cells exhibiting HLA DR antigen and interleukin-2 receptor, CD19+ B cells and natural killer cells were determined by two colour flow cytometry using monoclonal antibodies. Blastogenic response of PBMC to mitogen phytohemagglutinin (PHA) was assessed after 96 h incubation by measuring 3(H)-thymidine incorporation and the results were expressed as net counts per minute. Spontaneous PBMC proliferation was also evaluated in unstimulated culture and the results were reported as mean counts per minute. Comparisons of G-AP, CP and H groups were performed using the Kruskal-Wallis and Bonferroni-corrected Mann-Whitney U test. RESULTS: No significant differences in the relative counts of PBMC subsets were found between G-AP, CP and H groups (P > 0.05) with the exception of lower relative amount of CD3+ T cells found in the CP group compared with healthy subjects (P = 0.0048). Blastogenic response to PHA was significantly suppressed in G-AP and CP groups relative to that of H group (P < 0.02). However, there was no significant difference in the blastogenic response to PHA between G-AP and CP groups (P > 0.05). Spontaneous proliferative response of G-AP and CP groups was also significantly lower compared to that of H group (P < 0.02). Similarly, no significant difference in spontaneous PBMC response was observed between G-AP and CP groups (P > 0.05). CONCLUSIONS: Although G-AP and CP patients have similar amount of immune cells compared to healthy subjects, reduced functional activities of these cells may suggest the existence of defective cellular immune mechanism for the susceptibility to periodontal disease. One has to keep in mind that periodontal diseases have a genetic basis and the present functional analysis might not be connected to the actual genetic predisposition to the disease.

Does immunologic status predict peritonitis in children treated with CAPD?

The purpose of this study was to evaluate whether immunologic status could predict the development of peritonitis in children on continuous ambulatory peritoneal dialysis (CAPD). Twenty-one patients (12 boys, 9 girls) aged 10.1 +/- 4.32 years (range: 23 months to 14 years) were studied. The mean duration of CAPD treatment was 12.88 +/- 6.69 months (range: 2-22 months). Twelve healthy children (mean age 11.5 years) were selected as a control group. Lymphocyte subpopulations (CD3, CD4, CD8, CD19, NK, and IL-2R) were determined by double-color flow cytometry (Becton-Dickinson). Statistical evaluation was made by Student's t-test. CD4 (38.4 +/- 7.1% vs. 28.0 +/- 5.4%), CD4/CD8 ratio (1.30 +/- 0.4 vs. 0.96 +/- 0.3), and B lymphocyte (19.9 +/- 8.9% vs. 12.0 +/- 3.2%) levels were significantly higher in CAPD patients compared with controls. CAPD patients showed significantly lower natural killer (NK) cell values than controls (12.7 +/- 7.0% vs. 27.3 +/- 8.3%). Apart from CD19 values (21.9 +/- 10.4% vs. 12.0 +/- 3.2%) there were no significant differences between CAPD patients without infection and the control group in the laboratory parameters studied. On the other hand, CD3 (66.3 +/- 7.9% vs. 55.9 +/- 9.7%), CD4 (38.4 +/- 7.1% vs. 28.0 +/- 5.4%), and CD19 (6.3 +/- 3.3% vs. 12.0 +/- 3.2%) levels were significantly higher in CAPD patients with infection compared with the controls. CAPD patients with infection showed significantly lower NK activity (12.7 +/- 7.1% vs. 27.3 +/- 8.3%) than those in the control group. In conclusion, these results can explain the increased vulnerability to peritonitis in CAPD patients compared with healthy subjects. Additionally, immunologic status can predict the development of peritonitis in children treated with CAPD.

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.

Immunohistochemical characterization of lymphocyte subsets in chronic adult periodontitis.

It is well known that interactions between microbial dental plaque and the host immune system play a major role in the etiopathogenesis of periodontal disease. The aim of the present study was to analyze the phenotypic properties of gingival T lymphocytes and subsets in patients with chronic inflammatory adult periodontitis (AP) showing various degrees of inflammation and to relate the results to the immunopathogenesis of AP. Gingival biopsies were obtained from patients aged between 26 and 52 yr who were grouped according to gingival index scores (GI) of 1, 2, and 3. Using immunohistochemical techniques, T cells (CD2+), T-helper cells (CD4+) and T-suppressor cells (CD8+) were identified in three well-defined areas of the biopsy samples. Moreover, peripheral blood was collected from the same patients, and relative counts of B cells (CD19+), HLA-DR+ cells and IL-2R+ cells as well as CD3+, CD4+, CD8+ cells were determined using three color flow cytometry. While the blood results were found to be within the normal ranges, the relative counts of CD4+ cells showed statistically significant decreases as the GI score increased. Similarly, the CD4+/CD8+ ratio also decreased. Moreover, gingival T lymphocyte and subset counts appeared to be related to the severity of gingival inflammation. Particularly, CD4+ cells showed a significant increase with the GI score. Furthermore, the CD4+/CD8+ ratio beneath the pocket epithelium was apparently correlated with increasing GI score (p < 0.05). The cytotoxic effect of CD8+ cells seems to be more prominent at the local level while the suppressor effect is more active systematically. This means that the price of systemic protection appears to be local destruction.

The serum levels of soluble interleukin-2 receptor levels in patients with obstructive jaundice.

BACKGROUND/AIMS: Increased susceptibility to infection in patients with obstructive jaundice is well recognized. Depression of reticuloendothlial system phagocytic function and suppression of cellular immunity suggested by in vivo studies have been postulated as the cause. It has been shown that increased serum soluble interleukin-2 receptor (sIL-2R) levels are the marker of immune system activation, especially T cell activation. The purpose of this study was to evaluate cellular immune system activation by measuring serum sIL-2R levels in 18 patients with obstructive jaundice (11 with choledocholithiasis, 7 with malignant obstructive jaundice), 10 patients with liver cirrhosis and 10 healthy subjects. MATERIAL AND METHODS: Serum sIL-2R levels were measured by using ELISA (Boehringer Manheim). Lymphocyte subgroups were determined by flowcytometry. Serum immungolubulins (IgG, IgA, IgM) and autoantibodies such as antinuclear antibody, rheumatoid factor, anti-thyroglobulin and anti-microsomal antibody were measured. RESULTS: The levels of serum sIL-2R were found to be 47.1-121.2 (mean 77.3, SD +/- 20.1) pmol/l in healthy subjects, 82.8-199.2 (mean 150.9 +/- 32.2) pmol/L in patients with liver cirrhosis and 32.6-172.5 (mean 121.7 +/- 40.6) pmol/L in patients with obstructive jaundice. Serum sIL-2R levels were significantly higher in patients with liver cirrhosis or obstructive jaundice than in healthy subjects (p < 0.01 and p < 0.05 respectively). There is a significant difference in levels between patients with choledocholithiasis and with malignant obstructive jaundice (p < 0.01). Serum sIL-2R levels were measured higher in patients with liver cirrhosis than those in patients with obstructive jaundice (p < 0.059). CONCLUSIONS: In patients with obstructive jaundice, and to a lesser extent in those with liver cirrhosis, in vivo activation of immune system may be considered possible.

Lymphocyte subpopulations in children with vitamin D deficient rickets.

Recent studies have shown 1,25(OH)2D3-mediated modulation of the immune system. We examined lymphocyte subpopulations of 16 children with nutritional rickets. Most of the patients suffered more frequent infection episodes than the control group of 15 healthy children and low serum levels of 25OHD and 1,25(OH)2D, such as 38.2 +/- 8.6 ng/mL and 15.7 +/- 2.6 pg/mL respectively. This decrease correlated with a significant decrease in total T lymphocytes and an increase in B lymphocytes expressing surface IgA, IgM, IgG molecules. These results suggest that vitamin D plays an important role in the impaired functions of T lymphocytes which may lead to frequent infection episodes in nutritional rickets.