10 research outputs found
Nephrotic syndrome in a patient with Glycogen Storage Disease Type IXb.
Introduction: Glycogen storage disorder (GSD) IXb is characterized by liver and muscle involvement. We present a GSD IXb patient with an incidental union of nephrotic syndrome.
Case Report: A 4 year-old-patient was diagnosed with GSD IXb at 13 months of age with mildly elevated transaminases and hepatomegaly. During the follow-up period, there was no hypoglycemia. Development and growth were normal. In the last month, the onset of generalized edema was reported. Urinalysis showed a high protein level. He had low serum albumin, high serum triglycerides cholesterol. Complement levels were normal. The patient was diagnosed as minimal change disease with a renal biopsy. He was treated with oral prednisone.
Discussion: Minimal Change Disease is the most common cause of idiopathic nephrotic syndrome cases in children and the first step for therapy is the usage of corticosteroids. This is the first report of nephrotic syndrome associated with GSD IXb disease
Atypia and Differential Diagnosis in Cellular Blue Nevi: Clinicopathological Study of 21 Cases
WOS: 000367954000002PubMed ID: 25690862Objective: Cellular blue nevus differs from the classic blue nevus with characteristics such as large size, cellularity, intense pigmentation, and growing pattern with subcutaneous infiltration. It is a dermal melanocytic tumor that can be confused with melanoma due to the atypia it may contain. Material and Method: Hematoxylin-eosin and MIB-1 stained slides of 21 cases diagnosed between 2000-2014 were re-evaluated. In order to attract attention to this rare lesion, 21 cases are presented with the clinical and above-mentioned histopathological findings. Results: Thirteen (61.9%) cases were females and eight (38.1%) were male. The mean age was 25.4 (2-73). The most frequent localization was the sacral and gluteal region (11 cases). The mean diameter was 14.4 mm (4-60 mm). From the parameters defined to assess the atypia, ulceration was identified in four cases. Prominent cellularity and subcutaneous infiltration were seen in three and 16 cases, respectively. Mitosis was seen in six tumors. Immunohistochemically, MIB-1 was present in two cases as 3% and 2% respectively, while in others it was 1% or less. Although there is no precise definition for the "atypical cellular blue nevus", five patients were assessed as atypical cellular blue nevus (a case with infiltrative development of six cm tumor diameter, two cases with two mitosis and a MIB-1 index 3% and 2%, a case with one mitosis and confluent development and a case with one mitosis in addition to focal necrosis areas). No lymph node and/or distant metastasis was observed during follow-up. Conclusion: We think it is more important to rule out the possibility of conventional melanoma in cellular blue nevus with exaggerated morphological findings alongside low proliferative activity rather than to determine the atypia
Investigation of the Factors Affecting Allograft Kidney Functions: Results of 10 Years
12th Congress of the Turkish-Transplantation-Centers-Coordination-Association (TTCCA) -- OCT 18-21, 2018 -- Trabzon, TURKEYSen, Sait/0000-0002-1100-6657; Gokalp, Cenk/0000-0003-3909-7973WOS: 000500179300016PubMed: 31101175Introduction. Significant improvements in patient and graft survival and reductions in the frequency of acute rejection were obtained in the early period after renal transplantation, but this success was not sufficiently reflected in the long term. Allograft kidney losses in the long term remain a significant problem. in this study, we investigated the specific causes of graft losses in patients who had a good clinical course in the first year but developed graft loss in the long term. Methods. A total of 118 patients who underwent kidney transplantation in 2005 and 2006 in the Organ Transplantation Center of Ege University Medical Faculty Hospital were evaluated. the inclusion criteria were to be older than 18 years and have a serum creatinine value of <2 mg/dL at the 12th month after transplantation. Results. Sixty-one percent of the recipients were male, and the mean age at the time of transplantation was 34 +/- 11 years (18 to 61). We observed 29 graft losses during the mean follow-up period of 129 +/- 35 months (27 to 162). Three of the graft losses were death by functional graft. of the 26 patients with graft loss, 16 had chronic rejection, and 8 had recurrent glomerulonephritis. the relationship between nonimmune causes and graft loss was not detected. Conclusions. in conclusion, nonimmune factors may not be as important as we think in relatively young and healthier recipients. Chronic rejection and recurrent glomerulonephritis are the main causes of long-term graft loss of patients with good graft function at the end of the first year. Improvement of long-term survival will be possible with the prevention and effective treatment of these 2 problems.Turkish Transplantat Ctr Coordinat Asso
Relationship Between Severity of Renal Amyloid Deposition and Clinical Outcomes in Non-AA Amyloidosis
Objective: Renal involvement is a common manifestation of systemic amyloidosis. Amyloid load can be predicted by histopathological grading of amyloid deposits in renal biopsy specimens. This study aimed to determine the relationship of renal amyloid deposition grade with clinical manifestations and outcomes in patients with biopsy-proven renal non-AA amyloidosis. Methods: This retrospective cohort study included 74 subjects with renal non-AA amyloidosis (52 light chain amyloidosis and 22 unclassified amyloidosis). Baseline characteristics and follow-up data were recorded. Pattern and quantity of amyloid deposition in glomeruli, interstitium, vessels, and tubulointerstitial changes were scored. Renal Amyloid Prognostic Score was obtained by addition of all scores and divided into 3 grades (Renal Amyloid Prognostic Score grades I, II, III). Results: In light chain amyloidosis group, the median follow-up was 11 (4-45) months. The baseline estimated glomerular filtration rate was significantly lower among patients with Renal Amyloid Prognostic Score grade III. Death-censored Renal survival was significantly lower among patients with Renal Amyloid Prognostic Score grade III. Renal Amyloid Prognostic Score grade III was a significant predictor of lower renal survival. Patient survival was not significantly different according to Renal Amyloid Prognostic Score grade. Receiving autologous stem cell transplantation treatment was associated with better patient survival. The type of amyloid could not be determined in 22 patients. In this group, baseline estimated glomerular filtration rate was significantly lower in patients with Renal Amyloid Prognostic Score grade III. Conclusions: In patients with light chain amyloidosis, baseline renal function is associated with Renal Amyloid Prognostic Score grade. Renal survival is significantly lower in patients with the highest Renal Amyloid Prognostic Score grade. However, patient survival is not significantly different according to Renal Amyloid Prognostic Score grade
Small Cell Carcinomas of the Bladder Highly Express Somatostatin Receptor Type 2A: Impact on Prognosis and Treatment--A Multicenter Study of Urooncology Society, Turkey.
Small cell carcinoma (SmCC) is a rare and aggressive neuroendocrine carcinoma of the bladder. Neuroendocrine carcinomas expressing somatostatin receptors (SSTR) in other viscera such as lung, pancreas, and gastrointestinal system respond to therapy with somatostatin analogs. In the present study, expressions of SSTRs 1 to 5 including type 2A are investigated by immunohistochemistry (IHC) and their relationship with clinicopathologic factors was evaluated. Hundred primary bladder SmCC cases were collected from 12 centers in Turkey. Forty-three cases were pure SmCC. Other cases had mostly papillary urothelial carcinoma as a second component. The percentage of the SmCC component ranged from 5% to 100%. SSTR-2A expression was membranous, whereas the other receptors showed cytoplasmic staining. The percentages of positive cases for SSTR-1, SSTR-2A, SSTR-3, SSTR-4, and SSTR-5 were 4% (3/75), 61.4% (54/88), 2.4% (2/84), 24.4% (20/82), and 6.25% (5/80), respectively. The percentage of SmCC component was positively correlated with the percentage of SSTR-2A expression (P=0.003) while negatively correlated with patient age (P=0.032). SSTR-2A expression was correlated with survival as a bad prognostic factor (P=0.018). SSTR-1, SSTR-3, SSTR-4, and SSTR-5 expressions did not show any statistical significance with any parameter. In conclusion, although the limited number of cases with adequate term follow-up, SSTR-2A expression could be a prognostic factor and somatostatin analogs therapeutic candidate for SmCCs of the bladder as these tumors show high percentage of SSTR-2A expression