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1.
BACKGROUND: Angiotensin receptor antagonists are effective in reducing proteinuria by an action independent of blood pressure. As a consequence, such agents retard progressive renal dysfunction in adults with chronic proteinuria. Long-term efficacy and tolerability data in children are unavailable. METHODS: Efficacy of losartan in reducing proteinuria and in preserving renal function was prospectively assessed in 52 consecutive children under 18 years of age with chronic proteinuric renal disorders, an initial creatinine clearance > or =25 mL/min/1.73 m(2), and a minimum of two or more follow-up visits. Thirty had proteinuria (P), and 22 had proteinuria combined with hypertension (P+H). Adverse effects were also evaluated. RESULTS: Proteinuria had persisted or increased during a mean interval of 8.5 months before initiation of losartan at a mean dosage of 0.8 mg/kg/d. Mean protein excretion before starting losartan was 2453 mg/m(2)/d and fell by 34% at a mean follow-up time of six weeks (visit I, P<.05), and between 64% and 67% at mean follow-up periods of 0.38, 0.71, and 2.48 years corresponding to visits II, III, and IV (all P<.001 compared with baseline). The proportion of children with protein excretion exceeding 40 mg/m(2)/h (nephrotic range proteinuria) or nephrotic syndrome (>3500 mg/1.73 m(2)/d) fell from 42% and 40% at the start, to 24% and 8%, respectively, at visit IV (P<.01). Mean creatinine clearance as well as serum potassium and total CO(2) levels remained unchanged during the time of follow-up. Reduction in proteinuria in the P subgroup alone correlated with lowering in diastolic blood pressure at visit II and with both diastolic and systolic blood pressure at visits III and IV (all P<.05); it was largely independent of reduction in blood pressure in the P+H subgroup. The concomitant use of immunosuppressive agents in 28 of the 52 children had an influence on proteinuria only at baseline and at visit I (P<.05). There was no significant change in height or body mass index Z scores. Thirteen children had adverse effects potentially ascribed to losartan; most of these either improved or resolved with dosage adjustment or resulted in its discontinuation in 9 of the 52 children (17%). CONCLUSION: Losartan therapy was associated with a marked and sustained reduction in proteinuria and in preservation of GFR in children with chronic proteinuric disorders. The association between proteinuria and systemic blood pressure reduction was complex: it was largely limited to the first year of losartan therapy and was more pronounced in the normotensive subgroup. Losartan was generally well tolerated.  相似文献   

2.
目的了解伴新月体形成的原发性IgA肾病(IgAN)患儿临床与病理特点。方法选择2000年1月至2011年1月在温州医学院附属育英儿童医院经肾活检确诊为原发性IgAN且病历资料完整的患儿78例,根据是否伴新月体形成分为伴新月体形成的IgAN组(C组)和不伴新月体形成的IgAN组(NC组),并比较两组结果。结果 78例原发性IgAN患儿中男性57例,女性21例;年龄平均(9.32±3.16)岁。C组33例(42.3%),NC组45例(57.7%)。与NC组相比,C组肾活检前病程更短[(2.15±4.06)个月对(9.87±19.09)个月,P<0.05],肉眼血尿发生率更高(30/33对25/45,P<0.01),24h尿蛋白定量更多[(93.08±82.75)mg/(kg·d)对(44.92±68.44)mg/(kg·d),P<0.05],且表现为大量蛋白尿者更多(19/31对10/42P<0.01),肾功能损害者较多(8/33对2/45,P<0.01)。在肾脏病理改变上,C组中-重度系膜增生、球囊粘连、毛细血管内皮增生均显著多于NC组(P均<0.01)。结论儿童伴新月体形成的原发性IgAN临床表现、病理改变较重,临床上应提高对此类型IgAN的认识,争取早期肾活检,早期诊断,积极治疗,在急性期控制疾病进展以改善预后。  相似文献   

3.
儿童新月体性IgA肾病临床与病理分析   总被引:2,自引:1,他引:2  
目的 了解儿童原发性新月体性IgA肾病的临床、病理和免疫病理的特征 方法 分析9例儿童原发 性新月体性IgA肾病患儿的临床、病理和免疫病理资料,进行疗效观察及随访 结果 临床表现为肾病综合征5例, 急进性肾炎3例,无症状性血尿和蛋白尿1例。尿蛋白均>2 g/d,其中>3 g/d者7例:有持续性肉眼血尿8例,其中>2 周者7例;肾功能减退苦8例,其中5例仅内生肌酐清除率(CCr)轻度下降者;伴高血压7例 9例肾病理均有不同程 度的弥漫性系膜增生和小管-间质病变,平均74.5%肾小球有新月体形成,半数病例可见球囊粘连、小球硬化、节段内 皮增生和间质灶状纤维化。免疫荧光无1例单纯IgA型,IgA M和IgA M G型占55.6%,4例呈"满堂亮"。8例经大剂 量甲基泼尼松龙冲击2~4个疗程治疗,肉眼血尿、高血压全部消失,肾功能恢复正常,蛋白尿有不同程度改善 7例随 访3~18个月,3例尿蛋白正常,2例轻度蛋白尿(<1 g/d),尿蛋白>3 g/d者2例 结论 本组儿童原发性新月体性IgA 肾病临床以肾病综合征为主,持续性肉眼血尿和大量蛋白尿突出,肾功能减退程度不一;肾小球、小管和间质均有急、 慢性病理改变,球囊粘连、小球硬化和间质纤维化易见,免疫病理以IgA合并IgM、IgG沉积为主,可见"满堂亮"现象 及时大剂量甲基泼尼松龙冲击治疗,短期疗效好。  相似文献   

4.
儿童紫癜性肾炎临床与病理相关性分析   总被引:6,自引:2,他引:4       下载免费PDF全文
目的:通过对95例紫癜性肾炎(HSPN)患儿临床表现及肾脏病理分析,阐明其临床及病理之间的联系。方法:对HSPN患儿进行临床分型及病理分级,对其进行综合分析。结果:①临床分型以肾病综合征型(27.4%)、蛋白尿+血尿型(24.2%)多见,病理分级以Ⅲb(42.1%)最多见;②尿检正常者可见肾脏病理改变。尿检正常型、孤立性血尿或蛋白尿型以及血尿和蛋白尿型病理改变差异无显著性(P>0.05);③孤立性血尿或蛋白尿型以及血尿和蛋白尿型病例,病程越长病理分级也越重(P<0.05);④免疫复合物沉积以IgA+IgG+IgM(58%)同时存在比例最高;病理分级越重,病程越短,IgA+IgG+IgM比例越高。结论:HSPN患儿临床表现为肾病综合征和肾炎型者病理改变相对较重,临床症状与病理不一定平行,尿检正常者病理改变也很明显,病程越长,病理改变呈加重趋势。免疫复合物沉积为IgA+IgG+IgM的病理改变相对较重。[中国当代儿科杂志,2007,9(2):129-132]  相似文献   

5.
Renal involvement in patients with congenital cyanotic heart disease.   总被引:2,自引:0,他引:2  
Patients with congenital cyanotic heart disease may develop a glomerulopathy with proteinuria and impaired renal function. In order to investigate this problem we conducted a study on 27 patients with uncorrected cyanotic heart disease who were between 1 day and 25 years old. As a consequence of hypoxaemia haematocrit was elevated to 57%. Proteinuria was above 150 mg/day/1.73 m2 body surface in 12 patients. Only one of 9 children under 10 years of age had pathological proteinuria presenting as isolated albuminuria. Seven out of 10 patients between 11 and 20 years had an elevated proteinuria with a glomerular pattern. Creatinine clearance was normal in these patients. All four patients above 20 years of age had a considerable glomerular proteinuria with a mean excretion of 5.7 g/24 h/1.73 m2 body surface. These patients suffered additionally from chronic cardiac failure and creatinine clearance was below the normal range. There was a clear relationship between pathological proteinuria and age of the patients and thus duration of hypoxaemia. Patients with pathological proteinuria had a significant higher erythrocyte count (7.3 +/- 1.3 vs 5.6 +/- 1.4 10(12)/l p less than 0.01) and a lower mean corpuscular haemoglobin. In summary, children with persistent congenital cyanotic heart disease have substantial risk of developing a glomerulopathy if the cyanosis remains unchanged for more than ten years.  相似文献   

6.
目的 报告1例由于服用丙基硫氧嘧啶(PTU)导致的抗中性粒细胞胞浆抗体(ANCA)阳性的血管炎。方法 根据病史、症状、血清学及肾活组织病理检查结果,结合文献进行分析。结果 12岁女性患儿,以肉眼血尿、蛋白尿起病,伴肾功能轻度受损,ANCA检查阳性,肾活检符合寡免疫小血管炎。结合发病前有服用PTU5年史而诊为PTU导致之NACA阳性小血管炎。经停用PTU,给予泼尼松、环磷酰胺治疗,随访3个月症状明显好转,ANCA-MPO滴度下降。结论 PTU可导致ANCA阳性的血管炎,及时诊治可改善预后。  相似文献   

7.
Most studies evaluating renal function post-renal transplantation in children have used serum creatinine (S(Cr)) or estimates of its clearance (C(SCH)). When renal function is impaired both S(Cr) and the C(SCH) overestimate glomerular filtration rate (GFR), especially during cyclosporine therapy. This study measured GFR in 64 children (age range: 4-19 years) with stable renal function who received renal allografts at the Childrens Medical Center of Dallas, 31 from live related donors (LRD) and 33 from cadaveric donors (CAD). 125I-iothalamate clearance (C(IO)) was used as the reference standard for measuring GFR. Data from 100 C(IO) studies, were analyzed and results reported as mean +/- S.E.M. C(IO) performed during the first year after renal transplantation in 23 children who received allografts from LRD was 72.4+/-5.5 ml/min per 1.73 m2 compared to 50.4+/-7.4 ml/min per 1.73 m2 in 18 children who received allografts from CAD (p<0.05). Beyond the first year post-renal transplantation there was no difference in C(IO) between LRD and CAD allografts. When S(Cr) was compared to C(IO), the relationship was nonlinear. C(IO) was also compared to the simultaneous estimation of creatinine clearance by C(SCH). The overestimation of GFR by C(SCH) was inversely proportional to the level of renal function. When renal function was normal or mildly reduced (C(IO) > 50 ml/min per 1.73 m2), C(SCH) closely approximated C(IO). When renal function was moderately to severely curtailed (C(IO) < or = 50 ml/min per 1.73 m2), C(SCH) overestimated C(IO) by 43.6+/-5.6%. The study concludes that in children with renal transplant: 1) C(IO) is higher in allografts from LRD compared to CAD kidneys only in the first 12 months following renal transplantation; 2) S(Cr) is a poor predictor of C(IO); and 3) C(SCH) consistently overestimates GFR children following renal transplantation unless renal function is normal or only mildly decreased.  相似文献   

8.
The association of renal disease and autoimmune thyroid disorders has been reported previously. Renal findings associated with autoimmune thyroiditis present more commonly as proteinuria ranging from mild to nephrotic levels. We report here two adolescent girls with hyperthyroidism associated with transient proteinuria correlated with thyroid hormone levels. They had positive antithyroid peroxidase and antithyroglobulin antibodies. Ultrasonographic and scintigraphic findings of the thyroid gland were consistent with Graves' disease in both. Their renal functions were normal except proteinuria (daily protein excretion of 13.5 mg/m2/h in patient 1 and 11 mg/m2/h in patient 2). When they became euthyroid on antithyroid treatment, proteinuria decreased without associated hematuria and/or hypertension. In conclusion, patients with autoimmune thyroid disease should be assessed for the possibility of proteinuria and the etiological investigation of proteinuria should include evaluation of thyroid functions.  相似文献   

9.
Fifty one children with IgA nephropathy verified at biopsy have been followed up clinically and functionally for 0.4-16.8 years from the onset of symptoms. Renal function was evaluated by determining the glomerular filtration rate (GFR) and effective renal plasma flow (ERPF) from the clearances of inulin and para-aminohippuric acid. Fifteen (29%) of the children had raised serum creatinine concentrations at the onset. Mean GFR was significantly lower than that of controls at the first investigation. During the follow up GFR and ERPF decreased and were significantly lower than in the controls after eight years of disease. The significant fall in renal function was found in children with proteinuria and especially in boys, in whom GFR and ERPF decreased from a mean (SEM) of 117 (5) and 616 (31) at 2.8 years to 97 (6) and 509 (36) ml/min/1.73 m2 at 7.5 years. Patients with raised serum creatinine concentrations at the onset had significantly lower GFRs, and patients with macroscopic haematuria at this time did not show decreased renal function at follow up. In conclusion, children with IgA nephropathy do not seem to have a benign clinical course. Boys with proteinuria show a significant decrease in renal function during follow up.  相似文献   

10.
蛋白尿与肾脏疾病进展密切相关,但也可能是儿童中出现的一种正常的一过性现象。尿蛋白>150 mg/(1.73 m2·d)或>4 mg/(m2·h)定义为尿蛋白阳性。既往的报道中,多个中心均发现,在无症状儿童中,亦有相当比例的蛋白尿者。按蛋白尿的来源,可分为肾小球蛋白尿、肾小管蛋白尿、分泌性蛋白尿、溢出性蛋白尿。蛋白尿按临床分类可分为暂时性蛋白尿、直立性蛋白尿、持续性无症状蛋白尿、原发或继发性肾小球疾病、原发或继发性肾小管间质性疾病。  相似文献   

11.
The urinary albumin to creatinine ratio (Ualb/Ucr) was compared with quantitative albumin excretion in normal subjects and patients with renal disease. Urinary albumin excretion varied from 3.4 to 4,699 mg/m2/day and Ualb/cr from 5.3 to 6,600 micrograms/mg; the correlation was highly significant (r = .979, p less than .001, n = 20). To characterize normal proteinuria using random urine samples, specimens were obtained from 279 healthy subjects (2 months - 62 years). Total protein, albumin and lysozyme were measured in all samples. Glomerular permeability and tubular function were assessed using the random Ualb/Ucr, the urinary albumin to protein ratio (Ualb/Up) and the urinary lysozyme to albumin ratio (Uly/Ualb). Ualb/Ucr was higher in children less than four years although no age-related differences were noted for Ualb/Up or Uly/Ualb. Furthermore, no differences were seen between males and females and normal reference values are provided. The results of this study support the use of Ualb/Ucr as an estimate of urinary albumin excretion and characterizes normal proteinuria using markers of both glomerular and tubular function.  相似文献   

12.
??Proteinuria is usually associated with progressive renal disease??but may sometimes be a benign transient finding. Abnormal proteinuria is defined as protein excretion of ??150 mg/1.73m2 per day or??4 mg/m2 per hour. Many studies in deferent centers have reported that quite a few asymptomatic children with proteinuria were detected. Excessive proteinuria can be classified as glomerular proteinuria??tubular proteinuria??secretory protein and overflow proteinuria by causes. It can also be classified as transient proteinuria??postural proteinuria??persistent asymptomatic proteinuria??primary/secondary glomerular proteinuria and primary/secondary tubular proteinuria by clinical symptoms.  相似文献   

13.
Acute pancreatitis (AP) rarely complicates the clinical course of systemic lupus erythematosus (SLE). AP as the initial manifestation of SLE is exceptional, but its outcome is often fatal. Corticosteroids have been suspected to play a role in the development of AP, but the therapeutic benefit seems to be far above the risk of exacerbation of pancreatic lesions. We report a 13-y-old girl presenting with arthralgia and malaise, followed by abdominal pain, generalized oedema and haemodynamic instability. Increased CRP (325 ng/ml), serum amylase (14,000 IU/l) and lipase (2500 IU/l) levels suggested AP. Acute anuric renal failure required haemodialysis. Multiorgan involvement suggested SLE, which was confirmed 3 d later by increased anti-ds-DNA levels. Three methylprednisolone pulses were administered promptly, followed by oral prednisone (1.5 mg/kg/d) and six pulses of cyclophosphamide (500 mg/1.73 m2/2 wk). Mycophenolate mofetil was introduced for long-term disease control. Amylase and lipase levels decreased over 4 wk. Renal function was normal after 3 wk and proteinuria negative after 6 wk.This case suggests that steroid pulse therapy should be promptly administered if clinical and biochemical investigations suggest SLE to be responsible for AP. Aggressive treatment may be life saving.  相似文献   

14.
Henoch-Schonlein Vasculitis (HSV) is systemic small vessel vasculitis involving the skin, kidney, joints, and gastrointestinal tract. The proportion of patients reported to have renal involvement varies between 20% and 80%. Rapidly progressive glomerulonephritis (RPGN)is rare syndrome in children, characterized by clinical features of glomerulonephritis (GN) and rapid loss of renal function. We present a severe kidney involvement in a 14 year old boy with HSV in who is carring MEFV mutation. A 14 year old boy had developed sudden onset of palpable purpuric rash on his extensor surfaces of lower extremities. He had elevated an erythrocyte sedimentation rate (ESR) (45 mm/h), C-reactive protein (3.74 mg/dl), serum urea 66 mg/dl, serum creatinine 1.8 mg/dl. Also, he had hypocomplementemia. Antinuclear antibody, anti ds DNA, antineutrophil cytoplasmic antibody, anticardiolipine antibodies were negative. Urinalysis revealed macroscopic hematuria and proteinuria with a 24-h urinary protein excretion of 55 mg/m2/h. The renal biopsy specimen showed crescentic and necrotizing glomerulonephritis. He had also M694V/E148Q compound heterozygote mutation. Clinical symptoms and renal failure resolved with intermittant hemodialysis and medical therapy.  相似文献   

15.

Background

This study involving 351 children who had undergone kidney biopsy secondary to persistent asymptomatic isolated hematuria was undertaken to assess histological diagnosis of the disease and its natural history and prognosis.

Methods

The patients were divided into two groups: 215 patients with asymptomatic isolated microhematuria (AIMH; proteinuria <0.1 g/day) and 136 patients with persistent asymptomatic microhematuria, recurrent macrohematuria and/or proteinuria (AMHP; proteinuria 0.1–0.25 g/day). After kidney biopsy, the patients were monitored for 2–10 years.

Results

Normal biopsies or minor abnormalities were more frequent in AIMH patients than those in AMHP patients, who exhibited IgA nephropathy more frequently. During the 2- to 10-year follow-up period, adverse renal events (i.e., development of proteinuria, hypertension, or impaired renal function) were observed in 13/215 (6.0%) patients with AIMH and 31/136 (22.8%) patients with AMHP (χ2=15.521, P<0.001).

Conclusions

Normal biopsies or minor abnormalities were more frequently observed in AIMH patients, whereas IgA nephropathy and adverse renal events were more frequent in AMHP. Microscopic hematuria, especially when accompanied by macroscopic hematuria and proteinuria, may represent an important risk factor for the development of chronic kidney disease.  相似文献   

16.
The clinical presentation, initial laboratory and renal biopsy findings, and course of focal segmental glomerulosclerosis (FSGS) were studied retrospectively in 57 children in order to compare findings in those with and without nephrotic syndrome and to establish factors of prognostic significance. All patients had proteinuria. Eleven patients were otherwise asymptomatic, and nephrotic syndrome did not develop (group 1); 14 patients had asymptomatic proteinuria, but nephrotic syndrome subsequently developed (group 2); 32 patients had nephrotic syndrome (group 3). There were no differences between these three groups with regard to sex, age, initial renal function, incidence of hypertension and hematuria, and pathologic findings. At the latest follow-up, five group 1 patients, six in group 2, and 14 in group 3 had chronic renal failure; the incidence was similar for those with asymptomatic proteinuria and those with nephrotic syndrome. The location of the sclerosis within the glomerulus proved to have prognostic significance. All 12 patients with peripheral FSGS maintained normal renal function, whereas in 25 of the 44 with hilar FSGS chronic renal failure developed.  相似文献   

17.
Li ZH  Wei M  Ding GF  Wang DH 《中华儿科杂志》2011,49(11):814-817
目的 总结新生儿木通中毒所致马兜铃酸肾病的临床特点和转归.方法 回顾性分析3例新生儿因木通所致马兜铃酸肾病的临床表现、治疗和转归情况.结果 3例患儿均在服用含有木通的中药后出现呕吐、腹泻及尿量减少,出现急性肾功能衰竭、肾小球及肾小管损害.实验室检查表现为高血钾、低血钠,肌酐、尿素氮增高及代谢性酸中毒.轻度肾小球损害,表现为蛋白尿及血β2微球蛋白增高.肾小管功能损害表现为碱性尿,尿β2微球蛋白增高,尿糖、尿酮体及尿氨基酸阳性.经过对症治疗后肾功能在3~4周恢复正常,5~8个月尿蛋白转阴,3个月~1年尿氨基酸转阴,9个月~3年尿糖转阴,5.0~5.5年尿pH值降至7.0.5.5 ~6.0年后停止服用枸橼酸合剂.3例患儿随访12年,前11年内3例患儿的血清肌酐均在正常范围内,但近期随访显示2例患儿的血清肌酐升高,例3的血清肌酐正常.3例患儿估算肾小球滤过率(eGFR)均有所降低,其中例1、2的eGFR低于90 ml/(min·1.73 m2),近6年的eGFR每年降低1.1 ml/(min·1.73 m2)及0.6 ml/(min·1.73 m2),1例患儿的eGFR较前无明显降低.3例患儿血气正常,尿常规阴性,但血、尿β2微球蛋白仍较高.尿N-乙酰β葡萄糖苷酶(NAG)降至正常后又有所增高.结论 新生儿马兜铃酸肾病可引起急性肾功能衰竭和肾小管功能损害,经过对症治疗肾功能可在短期内恢复正常,但肾小球滤过率呈缓慢下降趋势且肾小管损害可持续多年,需要长期随访.  相似文献   

18.
The clinical course of 43 children with IgA glomerulonephritis detected by mass urine screening was followed for a mean period of 8.1 years. Histological findings were graded according to the severity of glomerular and tubulointerstitial lesions. There was no correlation in the severity of histological grade and clinical outcome between subjects with microscopic hematuria and those with microscopic hematuria and proteinuria nor between those with and without one or more episodes of macroscopic hematuria during the follow-up period. None of the 35 children with proteinuria less than or equal to 1 g/m2/day had severe histological findings or developed renal impairment. In contrast, the 8 children with proteinuria greater than 1 g/m2/day had moderate and severe histological findings. Four of these 8 children developed hypertension or renal insufficiency during the follow-up period. Our study indicates that the outcome of screening detected IgA glomerulonephritis in children correlates with the level of proteinuria and the severity of renal pathology.  相似文献   

19.
We studied the efficacy of captopril, an angiotensin-converting enzyme inhibitor in treating persistent moderate or severe proteinuria in children with various glomerular diseases other than minimal-change nephrotic syndrome. Captopril was administered for 3 months to 15 normotensive and nonazotemic or mildly azotemic patients (12 boys, 3 girls) in whom corticosteroid and cytotoxic treatment had failed to induce remission. Urinary protein excretion decreased from 2873.14±1937.50 (mean ± s.e.m.) to 1684.71 ± 1463.13 mg/day (P < 0.05). The reduction in proteinuria was not related to a significant fall in systemic blood pressure or a change in renal function. Serum albumin did not rise and side effects due to captopril were not observed. We concluded that, in the short term, captopril can be used safely and effectively for decreasing the proteinuria of nephrotic children unresponsive to conventional therapy.  相似文献   

20.
OBJECTIVE: To estimate, semiquantitatively, the proteinuria of nephrotic patients by the use of the value of protein/creatinine ratio in single urine samples and determine its correlation with 24-hour proteinuria.METHODS: Analysis of 30 single urine samples and thirty 24-hour urine samples from 20 children with nephrosis followed up at the Division of Pediatric Nephrology of the University Hospital, Faculty of Medicine of Ribeir?o Preto, University of S?o Paulo. Proteinuria in single urine samples and 24-hour urine samples was measured by the turbidimetric method with 3% sulfosalicylic acid. Urinary creatinine concentration was measured by the method of Hare, modified by Haugen and Blegen, adapted to the microtechnique.RESULTS: An excellent correlation was observed between 24-hour proteinuria and the protein/creatinine ratio in single urine samples, by linear regression analysis before (r = 0.82; p < 0.001) and after logarithmic transformation (r = 0.93; p < 0.001). All patients with 24-hour proteinuria at physiological levels (less than 0.1 g/m(2)/day) had a protein/creatinine ratio of less than 0.1 (mg/mg) in single urine samples. All patients with nephrotic 24-hour proteinuria (more than 1.0 g/m(2)/day) had a protein/creatinine ratio of more than 1.0 (mg/mg). The patients with intermediate proteinuria (between 0.1 and 1.0 g/m(2)/day) had a protein/creatinine ratio distributed on the three levels.CONCLUSIONS: The protein/creatinine ratio in a single urine sample is a simple and reliable method for the evaluation of proteinuria and eliminates the errors due to inadequate 24-hour urine collection.  相似文献   

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