| Minimal change disease (MCD) is the most common cause of thenephrotic syndrome (NS) in Children. It accounts for70%to90%of the NS inchildren who are younger than10yr and50%in older children. It is also animportant cause of the NS in adults of all ages, accounting for10%to37.4%ofcases. Although there are many data regarding the clinical and pathologicalfeatures, in pediatric patients, only a few series have examined these issues inadults. Relatively less is known about the presentation of MCD in adultpatients. Moreover, most series of adults with MCD have not been in Chinesepopulations, and it is unknown whether the features of the disease are thesame in differing parts of the world.The clinical and pathological data of adults who had biopsy-proven MCDand were followed at Ruijin Hospital affiliated to Shanghai Jiao Tong UniversitySchool of Medicine between January2009to October2013wereretrospectively reviewed. Between January2009and October2013,162adultpatients with MCD were identified. Among them,98patients was male and64female. The male and female ratio was1.53:1. The mean age was33.8±14.8years old (18~76years). The mean age at onset was33.114.7years. Theage at onset in male patients was earlier than that in female patients.18-30years old patients accounted for54.3%of all cases, Male patients mainlyconcentrated in the18-30years old, up to66.3%. The female patients with MCD mainly distributed in the18-30years old and31-40years old, accountingfor35.9%and31.25%. All162patients had different degrees of edema, greatamount of proteinuria, hypoalbuminemia and hyperlipidemia.47cases (29%)patients had different inducing factors. The most common cause was infection(61.7%). Only17.9%of the patients excreted protein in urine less than4g/24hand45.7%of the patients with urinary protein between4to8g/24h,36.4%with urinary protein>8g/24h.28.4%of patients presented microscopichematuria.17.3%of patients had serum creatinine higher than115umol/L.31.5%of the patients had MDRD-eGFR between60and90ml/min/1.73m2,and13.6%and8.0%of the patients with MDRD-eGFR between30and60ml/min/1.73m2and less than30ml/min/1.73m2.15.4%of the patients hadEPI-eGFR between60and90ml/min/1.73m2, while4.9%and8.0%ofpatients with EPI-eGFR between30to60ml/min/1.73m2and <30ml/min/1.73m2. The eGFR in patients aged60years old or higher was lessthan90ml/min/1.73m2, The proportion of renal involvement is higher with theage increased.10patients were accompanied with hypertension,2with type2diabetes. Among them,1patients had both diabetes and hypertension,Compared18-30to31-60years old age group, the incipient to relapse group,corticosteroid sensitive to corticosteroid dependence group, no statisticaldifference was observed in24h urine protein output, serum albumin, serumcreatinine level and so on.By light microscopy, global glomerular sclerosis was found in32.7%oftotal162renal tissue samples and renal interstitial fibrosis42.6%, renalinterstitial inflammatory cells infiltration55.6%, renal tubular atrophy45.7%and renal vascular lesions37.0%respectively. The frequency and severity ofall pathological changes except renal interstitial inflammatory cells infiltration were increased with the growth of age. The association of global glomerularsclerosis with age was not observed with age-adjusted.161samples had mildinterstitial fibrosis and renal tubular atrophy and1moderate.96.7%of sampleswith interstitial inflammatory cells infiltration showed mild severity and the othermoderate. The severity of interstitial inflammatiry cells infiltration in somesamples was higher than that of interstitial fibrosis and renal tubular atrophy.Acute tubular necrosis was found in eleven samples (6.8%). All11patientspresented acute renal impairment with serum creatinine418.5±265.8umol/L(143~956umol/L). Among them,2patients were younger than30years (2.3%),7between31and60years (11.5%) and2equal and older than60years(14.3%).90%(9/10) patients of MCD with hypertension had renal artory andartoriole lesions. There is no statistical difference in renal pathological changesbetween recipient and relapse group, corticosteroid sensitive and denpendentgroup.88.3%of samples showed no staining for IgA, IgG, IgM. C3, C4, c1qand fibrinogen. Low level mesangial staining for IgM (8.0%), IgA (7.4%) andC3(3%) were observed in several samples. Diffuse effacement of visceralepithelial cell foot processes was observed in all samples by electronmicroscopy.2samples had thin basement membrane disease at the sametime.In summary, this is a retrospective study in a large cohort of adult MCD inChina and their clinical and pathological characteristics are summarized. Wefound that MCD is more common in male patients than female patients. Theage at onset is eariler in male than female. Infection was the most commonpredisposing factor of MCD. A few patients have microscopic hematuria andhypertension. MCD can occur in patients with diabetes mellitus. The frequencyand severity of global glomerular sclerosis, renal interstitial fibrosis, renal tubular atrophy and renal vascular lestions increase with age growth. Renalvascular lesions are associated with both hypertension and age. Acute renalimpairment and acute tubular necrosis can occur in some patients, especiallyin elder aged60or more than60years. Thin basement membrane diseasecan be found in very few adult MCD patients. No obvious variation is observedin clinical and pathological parameters between the first onset and relapsegroup, corticosteroid sensitive and dependent group, which suggest that theresponse of corticosteroid treatment in adults MCD patients can not bepredicted by clinical and pathological parameters. |
