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Objective To analyze the clinical characteristics of 7 pediatric patients with C3 glomerulonephritis (C3-GN).Methods The clinical manifestations,pathological features,therapies,prognosis of patients from Jun.2006 to Nov.2011 were analyzed retrospectively.Results All of the patients were presented as acute nephritic syndrome,and 4 patients with macroscopic hematuria,other 3 cases with severe proteinuria.Five patients with eyelid edema.All the patients showed decreased level of serum complement C3,while serum complement C4 was normal.Investigations showed elevation of 24 h urine protein,5 patients with elevated antistreptolysin O titers.Three patients with renal dysfunction.Isolated C3 deposition in mesangial and endothelial areas(+ +-+ + +) was confirmed by immunofluorescence.Light microscope revealed membrane proliferative glomerulonephritis and mesangial proliferative glomerulonephritis.Electron microscope showed swelling and hyperplasia of endothelial cells without electron-dense deposition or podocyte foot fusion.Based on conventional treatment,administration of immunosuppressant was performed in 3 patients with severe pathological changes.After a follow-up of 2 months to 5 years,the prognosis seems to be benign.Conclusions Children with C3-GN are usually presented as acute nephritic syndrome,characterized by isolate C3 deposition in immunofluorescence.Electron microscope showed lesion of endothelial cells,while no electron-dense deposits in mesangial and endothelial areas.The mechanism may be associated with dysregulation of alternative complement pathway,and seems had a good short-term prognosis.
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From March 2009 to October 2009, three pediatric patients with parotid tumor were cured. Preoperative physical examination showed regional swelling in parotid area, the surface skin was in moderate reddish purple, the border was vague, and the swelling was inactive. The patients' IgE were significantly increased. B ultrasound examination demonstrated the focus was an isoecho with ringlike dark band around, which was concluded as bull's-eye sign. Magnetic resonance imaging (MRI) examination indicated a cystic mass between the skin and parotid. Preoperative diagnosis was eosinophilichyperplastic lymphogranuloma (Kimura's disease) and the granuloma was excised by operation. Pathological examination revealed the capillary vessel hyperplasia in local tissue with a plenty of eosinophils and lymphocytes infiltrating. The disease was confirmed. Although the disease is rare, the diagnosis still could be made by preoperative physical examination, laboratory and imaging examinations.
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Humans , Male , Angiolymphoid Hyperplasia with Eosinophilia , Magnetic Resonance Imaging , Parotid GlandABSTRACT
0.05).Compared with other immunopathologic types,IgA plus IgG plus IgM deposition type had higher proportion of histological grade Ⅲb-Ⅵ(P
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<p><b>OBJECTIVE</b>To investigate the interrelations among morphology, immunology, cytogenetics and clinical outcome in childhood acute leukemia with 11q23 abnormalities.</p><p><b>METHODS</b>Eighteen patients with 11q23 abnormalities, from 320 childhood acute leukemia patients, were retrospectively analysed for cell morphology, flow cytometry, immunophenotyping, R-banding karyotype as well as clinical features and prognosis. Twenty cases of childhood AL with normal karyotype during the same period were used as control.</p><p><b>RESULTS</b>The incidence of 11q23 abnormalities in our childhood acute leukemia patients was 5.63% including 14 acute lymphoblastic leukemia (ALL) and 4 acute myeloid leukemia (AML). Of 16 cases immunophenotypically tested, 13 expressed lymphoid antigens and 3 CD(34) and other myeloid antigens. Karyotype analysis disclosed the following abnormalities: t(4; 11)(q21; q23) in 6 cases, t(10; 11)(p13; q23) in 3, t(11; 19)(q23; p13) in one and del(11)(q23) in 6. The complete remission rate for these patients with 11q23 abnormalities was comparable to that of the control (72.2% vs 80.0%, P > 0.05), while the mortality rate in the former was significantly higher than that in the latter (61.1% vs 25.0%, P < 0.05).</p><p><b>CONCLUSIONS</b>11q23 abnormalities were mainly seen in childhood ALL and acute monocytic leukemia with unique prognostic features.</p>