Clinical And Pathological Analysis Of 22 Children With Primary Immunoglobulin A Nephropathy

Background: IgAN is a renal biopsy shows deposits of IgA in the mesangial area and (or) capillary vessel of the glomerulus as diagnostic criteria. For Initially, people thought that it's a benign disease, but with a long-term follow-up observition ,its prognosis is not optimistic, part of these patients comes to ESRD with ten years, clinical manifestation may from asymptomatic hematuria to kidney failure, and histopathology may from no obvious renal lesion formation to cresent formation, glomerular sclerosis, renal tubular atrophy, tubelointerstitial fibrosis as almost all histologically primary glomerulonephritis. In recent years, along with our extensive renal biopsy of the technology, IgAN has become one of the most common primary glomerulonephritis, the high incidence of IgAN occurs on the period of youngs, so IgAN for children's study become more and more attention by people.Objective: Explore children's primary IgAN clinical manifestation and pathological features, analysis of the general situation, clinical manifestations, pathological features and relations between the prognosis, to find early the reason of prognosis mala, judge the condition synthetically, direct the clinical treament. Methods: Retrospectie analysis the clinical and laboratory datas of the patients in the hospital diagnosis of renal biopsy for IgAN puncture with complete datas from March 2007 to March 2010 in jilin university hospital. Record selected patients age, gender, urban or rural, the first onset to renal biopsy time, blood pressure, and onset of symptoms, complained of comorbid and whether incentives, past history and history. after admission, urea nitrogen, serum creatinine 24-hour urine protein quantitative, urine and every three under RBC count at high magnification, the serum immunoglobulins IgA, IgM, IgG, C3, C4, plasma albumin, total cholesterol, triacylglycerols glycerin, alpha apolipoprotein, fibrinogen content. According to clinical manifestations into isolation (gross hematuria and microscopic haematuria urine), isolated proteinuria type, persistent hematuria and proteinuria type, acute nephritis syndrome type, nephrotic syndrome, accelerated nephritis and chronic nephritis type. Pathology classification to the pathological grading standards in 1982 to renal damage into class I ~ V, reference Katafuchi points pathological parameters of glomerular, tubulointerstitium and pathological changes of blood vessels. According to sedimentary type into IgA, IgA + IgG, IgA+IgM, IgA+IgG+IgM and full of bright type, according to IgA deposits in renal tissue strength into light " + ", moderate" + +" and severe "+ + +". Comprehensive analysis of history, clinical manifestations and pathologic characteristics give individual treatment. Follow-up has divided prognosis into clinical cure, slightly abnormal urine test, active nephropathy and renal insufficiency. Analysis IgAN the characteristics of epidemiology, clinical manifestation, pathological features and prognosis and the relationship between them. Measurement data to x±s, measurement data with Z test comparison between mean, Fisher exact test between counting material inspection, P < 0.05 probability for the significant meaning.Results: 1.General situation: the incidence of IgAN is 25% on renal biopsy cases. the age is from 6-14 (average age 10.32±2.28), median age 11, diagnose time for five days to 3 years, with a median of 14.5 days. The ratio for boys and girls is 1.44, 63.6%live in rural and 36.4% in urban. 77.3% patients had triggers, most of them is respiratory infections. For simple macroscopic haematuria complained of 12 patients, 8 cases were hematuria with edema, microscopic hematuria in 2 cases. 1 case was hyperpressure and 3 cases (13.6%) indicates that exists abnormal renal function when the onset. 2.Clinical manifestations: clinical classification of hematuria and proteinuria 40.9% (9 cases), 8 cases(36.4%) were nephrotic syndrome. Isolation hematuria 2 cases and chronic nephritis in 2 cases, acute nephritis 1 case, there is no isolated proteinuria and accelerated nephritis type. 3.Pathological features and related factors: Pathological grade III 14 cases (63.6%), grade II 6 cases(27.3%) and grade IV in 2 cases (9.1%), no grade I and grade V.Glomerular total score 3 ~ 6 points, four points and five points was reported respectively by 45% and 36.4%, main show is segments or diffuse hyperplasia in mesangial area, partial visible crescent formation, hardening rare glomerular sclerosis. Renal tubular total score 0-2, 1point 11 cases (50.0%), 2 points 4 cases, main show is renal tubules and interstitial mild inflammatory cell, a few cases have renal tubular atrophy and interstitial fibrosis. Only 1 case has renal vessels for 1 point, hemal wall thickening mild. Immune pathological type IgA+IgM 10 patients (45.5%) and IgA+IgG+IgM 6 cases, 4 cases of IgA type, IgA+IgG 2 cases, no full bright type . In the renal tissue deposition strength IgA "+++"12 cases (54.5%), "+" 6 cases and "++" 4 cases. There is no obvious difference between pathologic stage to immunoglobulin sedimentary types and IgA in renal tissue deposition strength (P > 0.05). 24 hours urinary protein, quantitative fibrinogen and total cholesterol are higher in pathological grade III- IV than II. Serum albumin, plasma IgG are lower in pathological grade III- IV than II (P < 0.05). IgA + IgM type in hematuria and proteinuria type, IgA + IgG +IgM type in nephrotic syndrome type. Pathological grade II in hematuria and proteinuria type, chronic nephritic type. Level III pathology in hematuria and proteinuria type, nephrotic syndrome. Pathological grade IV at nephrotic syndrome (P < 0.05). There is no obvious difference between the renal tissue deposition IgA strength and clinical classification (P > 0.05). 4.Prognosis and related factors: follow-up time from 2 months to 3 years, with a median of 15 months. Mildly abnormal urine test 13 cases (59.1%), 11 cases of microscopic hematuria, 2 cases with mild proteinuria, completely cured in 8 cases, 1 case with active kidney disease, found no case of renal insufficiency. There is no obvious difference between children ages, to diagnose time, gender, urban or rural, whether incentives, onset form and prognosis (P > 0.05). In the renal tissue deposition IgA strength, immunoglobulin sedimentary types, pathology classification and prognosis of the difference was not statistically significant (P > 0.05).Conclusion: 1. IgAN clinical classification mainly for hematuria proteinuria and nephrotic syndrome. 2. IgAN pathological classification mainly for type III. Immune pathology mainly for type IgA + IgM. 3.Proteinuria increase mean heavy lesion histology pathology of kidney. 4. The clinical classification of type III mainly for hematuria proteinuria and nephrotic syndrome, type IV mainly for nephrotic syndrome. 5. The immune pathological of hematuria proteinuria mainly for type IgA + IgM, nephrotic syndrome mainly for type IgA + IgG + IgM.