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Objective To compare the clinical features of diffuse capillary endothelial proliferative Henoch-Schonlein purpura nephritis ( DEP-HSPN ) with or without crescent formation. Methods The clinical data of 110 children with DEP-HSPN admitted to Dalian Central Hospital and the First Affiliated Hospital of Dalian Medical University from January 2008 to January 2018 were analyzed retrospectively. Among them,24 cases were divided into non crescentic group and 86 cases into crescentic group. The clinical characteristics and prognosis of the two groups were statistically analyzed. Results The clinical manifestations of children without crescent body formation group were all type III. There were 35 cases of type III and 51 cases of type V in the crescent body formation group. Compared with non-crescent formation group,the proportion of gross hematuria(83. 7%(72／86) vs. 29. 2%(7／24),χ2＝10. 396),urine red blood cell count ((112. 4±20. 3)／HP vs. (45. 2±10. 6)／HP,t＝9. 697),24 h urine protein ((2471. 6 ±242. 0) mg／d vs. (1358. 5±109. 3) mg／d,t＝6. 372) and serum creatinine (( 44. 9 ± 9. 6) μmol／L vs. (32. 3±5. 2) μmol／L,t＝5. 390) increased significantly,the serum albumin (( 22. 8±3. 8) g／L vs. ( 35. 1 ±5. 7) g／L,t＝4. 806)decreased significantly (all P＜0. 05). Both groups had non-simple IgA deposits in the mesangial region. The proportion of complete remission and asymptomatic hematuria was 70. 8%( 17／24) and 29. 2%( 7／24) in the non-crescent group, 58. 1%( 50／86) and 41. 9%( 36／86) in the crescent group,respectively,with no significant difference ( χ2＝1. 330,1. 196, all P＞0. 05) . Conclusion When DEP-HSPN is accompanied by crescent formation, gross hematuria, urinary erythrocyte count and the proportion of massive proteinuria increase significantly. Combined immunosuppressive therapy in acute stage and long-term sequential treatment in remission stage can achieve good prognosis.
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Abtract:Purpose To analyze epidemiological characteristics and pathological types of 1 645 renal biopsies in Jiangsu province. Methods The reports of 1 654 percutaneous renal biopsies performed from January 2009 to June 2013 were retrospectively analysed . Results 1 597 out of 1 645 renal patients were successfully biopsied with a success rate of 97. 1%. Primary glomerular diseases ac-counted for 78. 56% of the total patients, secondary glomerular diseases 18. 71%. IgA nephropathy and mesangial proliferative lession accounted for high percent of primary glomerular diseases. Lupus nephritis was the most frequent pathologic type of secondary glomeru-lar diseases, followed by allergic purpura nephritis and diabetic nephropathy. Mesangial proliferative glomerulonephritis and hyperten-sive renal injury were more common in the Southern than in the Northern Jiangsu province, while acute tubular necrosis and allergic purpura nephritis were less in the Southern Jiangsu province. Conclusions Primary glomerular disease is still the most frequent glo-merular diseases in Jiangsu province, among which the IgA nephropathy was predominated. In secondary glomerular disease, lupus ne-phritis is the most frequent pathological type. The incidences of kidney diseases have geographical variation.
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Objective To investigate the association of HLA-DRB1 alleles in Han population of Shanxi childrcn with nephrotic syndrome of non-IgA mesangial proliferative glomerulonephritis (MsPGN). Methods HLA-DRB1 was performed by polymerase chain reaction-sequence specific primers technique, and twenty patients with nephrotic syndrome of non-IgA MsPGN were detected. Results Analysis of the fre- quencies of specific at the HLA-DRB1 loci revealed significantly higher frequencies of HLA-DRB1 * 11 al- leles among the nephrotic syndrome patients of non-IgA MsPGN comparing with controls (22. 50% vs 8.33%, x2= 9. 544, P = 0.002, CI = 1. 674-9.995, RR = 4.09). Nine patients with HLA-DRB1 * 11 all accompanied hematuria, hypertension or short renal insufficiency. Conclusion The results suggested that HLA-DRB1 * 11 alleles contribute to genetic susceptibility to nephritic syndrome of non-IgA MsPGN. The pa- tients with HLA-DRB1 *11 easy accompanied hematuria, hypertension or short renal insufficiency.
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The incidence of glomerulonephritis associated with malignancy is not common. Membranous glomerulonephritis associated with carcinomas and minimal change nephrotic syndrome with Hodgkin's disease has been occasionally reported. The pathogenesis of glomerular injury associated with malignancy is not well known. The IgA nephropathy associated with malignancy, though rare, has been reported. IgA nephropathy associated with acute myeloid leukemia, however, is yet to be reported. We hereby report a case of IgA nephropathy associated with acute myeloid leukemia (AML M2).
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IncidenceABSTRACT
The incidence of glomerulonephritis associated with malignancy is not common. Membranous glomerulonephritis associated with carcinomas and minimal change nephrotic syndrome with Hodgkin's disease has been occasionally reported. The pathogenesis of glomerular injury associated with malignancy is not well known. The IgA nephropathy associated with malignancy, though rare, has been reported. IgA nephropathy associated with acute myeloid leukemia, however, is yet to be reported. We hereby report a case of IgA nephropathy associated with acute myeloid leukemia (AML M2).
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IncidenceABSTRACT
BACKGROUND: Glomerular epithelial cell protein-1 (GLEPP1) and WT-1 expressed in mature visceral glomerular epithelial cell (VGEC) is required for maintenance of the mature status of VGEC. Nephrin protein is located at the filtration slit and regarded as a molecular component of the slit diaphragm. Alterations of these proteins in proteinuric diseases are not clearly defined. METHODS: We investigated the expression of GLEPP1, WT-1 and nephrin in 28 renal biopsies diagnosed with minimal change nephropathy (n=10), focal glomerulosclerosis (n=10) and membranous nephritis (n=8) by immunohistochemical staining. Normal control biopsies were obtained from six nephrectomy specimens. RESULTS: The patients consisted of 15 males and 13 females. The mean age was 40.7 years. Nephrotic range proteinuria (> or =3.5 g/day) was noted in 15 (54%) patients. GLEPP1 and nephrin expression were significantly decreased in patients as compared with those of the controls (p<0.05). The mean number of WT-1 expressing cells per glomerulus was also significantly decreased in patients as compared with those of the controls (p<0.05). However, there was no significant difference in the number of WT-1 expressing cells among the disease groups. CONCLUSIONS: These results suggest that the loss of biological markers of mature VGEC may play an important role in the pathogenesis of proteinuria.
Subject(s)
Female , Humans , Male , Biomarkers , Biopsy , Diaphragm , Epithelial Cells , Filtration , Glomerulosclerosis, Focal Segmental , Nephrectomy , Nephritis , Nephrosis, Lipoid , ProteinuriaABSTRACT
Thirty caaes diagnosed as lGA nephroparthyby kidney biopay were treated bassed on differential diagnosis of syndromes the cases were divided into several stages via lesion in the lung -kidney (initial stage )lesion in the spleen-kidney (stable atage )and lesion in the liver -kidney (stable atage)and relapsing stage, and were treated by Chinese medicaments accordingly. The results revealed that 10 cases were totally mitigated; 13 cases, markedly alleviated; 4 cases, ameliorated, with only 3 cases ineffective. The total effecitve rate was 90%.Thirty cases diagnosed as IgA nephropathy by kidney biopsy were treated based on differential diagnosis of syndromes. The cases were divided into several stages, viz. lesion in the lung -kidney ( initial stage) , lesion in the spleen - kidney (stable stage) and lesion in the liver -kidney (advanced stage) and relaps-