A retrospective study on the characteristics of renal pathological grades in HSPN children with mild to moderate proteinuria
Yan CaoTian ShenYongzhen LiLanjun ShuaiQiaoping ChenShuang-Hong MoCanlin LiXiaoyan LiYing WangXiaochuan Wu
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Objective To investigate the characteristics of renal pathological grades in Henoch–Schönlein purpura nephritis (HSPN) children with mild to moderate proteinuria and the correlation between pathological grade and severity of proteinuria among this population. Methods HSPN children who were presented with mild (150 mg <24 h urinary protein <25 mg/kg) to moderate (25 mg/kg ≤24 h urinary protein <50 mg/kg) proteinuria and performed renal biopsy without steroid ± immunosuppressant treatment in the Second Xiangya Hospital between January 2010 and March 2021 were involved. We retrospectively analyzed the correlation between age, disease course, degree of proteinuria, type of immunoglobulin deposits, C3 deposits in glomeruli and renal pathological grade. Results (1) 72 HSPN children including 46 boys and 26 girls were included, with a mean age of onset of 9.01 ± 2.65 years old. The majority of these patients (62.5%) had a disease course between 1 week to 1 month. 51 patients presented with mild proteinuria and 21 patients with moderate proteinuria. (2) Renal biopsy results showed that ISKDC Grade IIIa were both predominant in mild proteinuria group (25, 49%) and moderate proteinuria group (11, 52.4%). 32 patients had grade II (44.4%), 2 had grade IIIb (2.8%), 1 had grade IV (1.4%), and 1 had grade VI (1.4%). There was no correlation between age, disease course and renal pathological grade ( p > 0.05). (3) In patients with mild proteinuria ( n = 51), 27 (52.9%) HSPN children had a pathological grade ≥ grade III. In patients with moderate proteinuria ( n = 21), 13 (61.9%) HSPN children had grade ≥ III. There was no significant difference in the proportion of renal pathological grade between the 2 groups ( p > 0.05). (4) There was no significant correlation between glomerular C3 deposits or immunoglobulin deposit types and renal pathological grade ( p = 0.776 and p = 0.056 respectively). Conclusion In HSPN children with mild to moderate proteinuria, longer disease course or heavier urinary protein level is not completely parallel with higher renal pathological grade. ISKDC grade IIIa is the most common pathological grade. Clinicians should pay great attention to the renal injury in patients with mild to moderate proteinuria.A 61-year-old male had nephrotic syndrome in association with minimal renal amyloidosis. The amyloid deposits were inconspicuous and had been initially overlooked, and the biopsy specimen was thought to show minimal glomerular changes. Accordingly he was diagnosed as having minimal change nephrotic syndrome (MCNS). Thereafter, administration of 40 mg/day of prednisolone was started. A few weeks after treatment, proteinuria decreased, but did not disappear. Four years later, he was readmitted for the treatment of the nephrotic syndrome. The second biopsy at 65 years of age revealed typical renal amyloidosis by light microscopy. Congo red staining and electron microscopy confirmed the presence of amyloid deposits. The serum A protein (SAA) level was 328 micrograms/ml. Furthermore, the first biopsy specimen revealed minimal renal amyloidosis by Congo red staining. At the time, 40 mg/day of prednisolone proved ineffective for the proteinuria. However, after methylprednisolone pulse therapy, the proteinuria decreased and the nephrotic syndrome improved. After discharge, administration of 20 mg/day of prednisolone was maintained. One year later, the patient showed no evidence of recurrence of the nephrotic syndrome and the SAA level decreased (from 328 micrograms/ml to 74.4 micrograms/ml). Prednisolone proved to have a beneficial effect on the reduction of proteinuria and SAA levels. We strongly recommend careful examination for amyloid deposits in all kidney biopsy specimens with the appearance of MCNS on older patients whose proteinuria does not respond to the administration of prednisolone.
Prednisolone
Amyloid (mycology)
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Objectives To explore the dynamic changes of clinical features and pathological lesions in children with IgA nephropathy. Methods Clinical and pathological data of five children with IgA nephropathy were collected before and after each renal biopsy. Results Among 5 cases,4 children were biopsied twice, 1 child had 3 times. Basic clinical manifestations of 5 cases were persistent microscopic hematuria or intermittent gross hematuria with varied levels of proteinuria and decreased renal function. The basic renal pathology was mesangial proliferation. Repeated renal biopsies showed pathological progression. Conclusions Clinical features and renal pathological changes of IgA nephropathy were highly variable between cases. When severe relapse occurs,a repeat renal biopsy should be performed to provide information for further treatment. The indication for repeat renal biopsy was discussed.
Renal pathology
Gross hematuria
Mesangial proliferative glomerulonephritis
Microscopic hematuria
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Pathological and Clinical Analysis of Tubulointerstitial Lesions in 45 Cases with Nephrotic Syndrome
Objective:To study the occurrence of tubulointerstitial lesions (TIL) in nephrotic syndrome (NS). Methods:The bio-chemical tests about renal tubular function and the pathological changes of 45 cases with nephrotic syndrome were investi-gated. Results:Compared with patients with lupus nephritis, the patients with primary glomerular nephritis (PGN) had dif-ferent urine NAG,HCO_3 ~ - ,TA,NH_4~+ and TIL (P0.05) ;proteinuria of PGN was positively correlated to urine NAG (P 0.05),urine osmolar pressure (P0.05) ,TA and NH_4~+(P0.01) and TIL (P0.01);compared with renal tubular functions of younger patients, those of older ones ( 35y) were prone to be injuried. Conclusion: Lupus nephritis causes more serious TIL than PGN;the increase of proteinuria and age may promote the development of TIL.
Nephritis
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Methods for detection of proteinuria and its causes are reviewed. Recommendations are given concerning the diagnostic procedures after detection of pathological proteinuria. The nephrotic syndrome is discussed mainly under the aspect of predictability of the histo-pathological form of the glomerular lesions from clinical and biochemical parameters.
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Sub-nephrotic proteinuria as a clinical syndrome and an indication to renal biopsy has not much attention. In this systematic review, series from 24 studies have been researched. IgA nephropathy, MesPGN and MGN were respectively defined as the most likely diagnoses for patients undergoing renal biopsies due to sub-nephrotic proteinuria. Profound disparities were also detected with regard to the patients’ region of origin as well as their age subgroup. Due to the limitation of the number of series reporting data on sub-nephrotic proteinuria and associated pathological diagnoses, further data coming with the prospective reports are necessary to make more precise estimations.
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Abstract BACKGROUND AND AIMS Minimal change lesion (MCD) accounts for 10–15% of adult patients with nephrotic syndrome and is characterized by edema, nephrotic-ranged proteinuria (NP) and pathologic findings of nearly normal appearance of glomeruli on light microscopy, minimal staining of immunoglobins and extensive effacement of foot process of the podocyte. However, the fate of MCD with typical pathologic findings without nephrotic proteinuria (Non-NP) needs to be defined in more details. METHOD We enrolled 79 adult MCD patients with the first renal biopsy in a tertiary teaching hospital from May 2003 to June 2017. We did not include patients having any immunosuppressive treatment before renal biopsy, patients with inappropriate biopsy samples (<10 glomeruli/biopsy, any electron dense deposit, and light microscopic findings suggestive of secondary causes). Remission of proteinuria was defined as urine protein-to-creatinine ratio (UPCR) < 0.3 g/g creatinine and recurrence of proteinuria as UPCR ≥ 3.0 g/g creatinine. We compared the clinic-pathologic features of MCD with or without NP at admission for renal biopsy, the frequency of flare to nephrotic proteinuria and the renal failure which was defined as decrease of estimated glomerular filtration rate (GFR) more than 50% compared to GFR at renal biopsy, GFR less than 15 mL/min/1.73 m2 or progressed to end-stage renal disease during follow-up period. RESULTS There were 3 patients with UPCR < 0.3 g/g creatinine, 17 patients with UPCR 0.3–2.9 g/g creatinine and 59 patients with UPCR ≥ 3.0 g/g creatinine at admission for renal biopsy. Mean age at renal biopsy was 53.7 ± 19.2 (range:18.5–99.0) years, and there were 38 male patients (48.1%). Non-NP group had lower UPCR (1.36 ± 0.99 versus 10.2 ± 6.21 g/g creatinine, P < .001) and higher GFR (98 ± 27 versus 75 ± 36 mL/min/1.73 m2 P = .012) at renal biopsy, and lower frequency of acute kidney injury during follow-up period [1/20 (5.0%) versus 35/59 (59.3%) patients, P < .001]. NP group showed more severe foot process effacement and interstitial changes. Other clinic-pathologic findings were not different between groups. During follow-up period (63.2 ± 50.4 months), eight patients (40.0%) had recurrence of proteinuria and nine patients (45.0%) had steroid treatment in non-NP group. Among NP group, 55 patients (93.2%) had been treated by steroid. The peak dose of prednisolone for patients having steroid treatment was not different between groups (Non-NP versus NP: 0.92 ± 0.16 mg/kg/day versus 0.88 ± 0.17 mg/kg/day, P = .491). The remission rate by the first treatment of steroid was not different between groups (non-NP versus NP: 9/9 versus 50/55, P = 1.000). The recurrence of proteinuria was developed in 8/9 non-NP patients (88.9%) and in 25/50 (50.0%) NP patients after the first remission of proteinuria by steroid treatment (P = .282). Recurrence of proteinuria was developed 0.23 ± 0.33 times/year in Non-NP group and 0.25 ± 0.41 times/year in NP group (P = .830). At the last follow-up period, the final remission rate of proteinuria was not different [65.0% of non-NP patients (17/20) versus 76.5% of NP patients (45/59), P = .384], and the rate of renal failure was not different, neither [15.0% of non-NP patients (3/20) versus 27.1% of NP patients (16/59), P = .371]. CONCLUSION The adult MCD patients with non-nephrotic-ranged proteinuria at renal biopsy showed similar clinical outcomes compared with patients with nephrotic-ranged proteinuria. Therefore, the MCD pathology should be paid more attention regardless of the amount of proteinuria at renal biopsy.
Minimal change disease
Nephrology
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Objective To observe the pathological changes in children with frequently relapsing and refractory nephrotic syndrome.Methods 49 children with refractory nephrotic syndrome were repeated renal biopsy,and their pathology were compared.Results The 1st time of renal biopsy to repeated biopsy from 10 to 48 months,the mean interval was 20.6 months.At the time of the second biopsies,pathological changes in 12 cases and the pathologic change rate was 24.49%,pathological transformation of up to MCD(9 cases,75.0%),followed by MsPGN(3 cases,18.75%).FSGS,IgAN,MPGN and MN was not found in pathological transition.Conclusion These results suggest that the therapy management should be adjusted if the children with frequently relapsing and refractory nephrotic syndrome did not have a satisfactory clinical response.It is advocated that perform a repeat biopsy repeat biopsy as soon as possible if the effect is still poor,to keep abreast of pathological changes,adjustment and development of new treatment options.
Refractory (planetary science)
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Twenty-one nephrotic children, aged 2 to 15 years, were studied for their urinary N-acetyl-beta-glucosaminidase (NAG) and daily urinary total protein, in a total of 35 episodes. Among them, 18 patients had urinary NAG levels (77.3 +/- 112.1 u/g Cr) above normal mean + 2 SD for age, while 3 had normal levels during the nephrotic stage. No or poor correlation was found between urinary protein and NAG. In eight of these patients, urinary NAG levels during heavy proteinuria and after remission were evaluated. No significant change was detected. These observations suggest that urinary NAG excretion in nephrotic children is not caused by an increased glomerular permeability to macromolecules. Instead, the elevated urinary NAG may reflect the activity of associated renal tubular dysfunction or tubulointerstitial involvement in the nephrotic syndrome.
Idiopathic Nephrotic Syndrome
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Excretory system
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To elevate the clinical diagnosis and pathological compatibility of pediatric kidney diseases. Pathological changes from 101 cases of pediatric renal diseases were studied, covering 13 diseases entities, including glomerulo-nephritis, simple nephrotic syndrome, nephritic nephrotic syndrome etc. The technique of tru-cut biopsy under the guidance of B ultrasonogram was used in renal biopsy. The renal puncture biopsy was 100% satisfactory. Pathological changes involved 12 pathological types. MsPGN were the most commonly seen (39.6%), followed by MPGN and ICPGN (12.87% and 10.89%) respectively. It was found that children with similar clinical manifestation may possess different pathological changes, while the same pathological entity may present multiple clinical manifestation. The results suggest that renal biopsy pathological diagnosis is important in the diagnosis, treatment and prediction of prognosis in pediatric renal disease.
Renal pathology
Nephritic syndrome
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