BACKGROUND: Minimal change nephropathy (MCD) is a common pathological type of nephrotic syndrome and is often associated with acute kidney injury (AKI). This study aimed to investigate the clinical characteristics and related factors of AKI in patients with MCD and nephrotic syndrome. METHODS: Patients from Chinese People's Liberation Army General Hospital who were diagnosed with pathological renal MCD with clinical manifestations of nephrotic syndrome were included from January 1, 2013 to December 31, 2017. Patients diagnosed with membranous nephropathy (MN) by renal biopsy from January 1, 2013 to December 31, 2017 are included as a control population. We retrospectively analyzed the clinical and pathological characteristics of patients as well as the percentages and clinical characteristics of AKI in different age groups. We assessed the correlation of pathological characteristics with serum creatinine using multivariate linear regression analysis. RESULTS: A total of 367 patients with MCD were included in the analysis, with a sex ratio of 1.46: 1 (male: female) and an age range of 6 to 77 years. Among all the patients, 109 developed AKI (29.7%), and of these patients, 85 were male (78.0%). In the 586 patients with MN, 27 (4.6%) patients developed AKI. The percentage of AKI in MCD patients was significantly higher than that in MN patients (χ2 = 41.063, P < 0.001). The percentage of AKI increased with age in the MCD patients. The percentage of AKI in patients aged 50 years or older was 52.9% (46/87), which was significantly higher than that [22.5% (63/280)] in patients under 50 years (χ2 = 6.347, P = 0.013). We observed statistically significant differences in age (43 [27, 59] years vs. 28 [20, 44] years, Z = 5.487, P < 0.001), male (78.0% vs. 51.4%, χ2 = 22.470, P < 0.001), serum albumin (19.9 ± 6.1 g/L vs. 21.5 ± 5.7 g/L, t = 2.376, P = 0.018), serum creatinine (129.5 [105.7, 171.1] μmol/L vs. 69.7 [57.7, 81.9] μmol/L, Z = 14.190, P < 0.001), serum urea (10.1 [6.2, 15.8] mmol/L vs. 4.7 [3.6, 6.4] mmol/L, Z = 10.545, P < 0.001), IgE (266.0 [86.7, 963.0] IU/ml vs. 142.0 [35.3, 516.5] IU/ml, Z = 2.742, P = 0.007), history of diabetes (6.4% vs. 1.2%, P = 0.009), and history of hypertension (23.9% vs. 5.1%, χ2 = 28.238, P < 0.001) between the AKI group and the non-AKI group. According to multivariate linear regression analysis, among the renal pathological features analyzed, renal tubular epithelial cell damage (β = 178.010, 95% CI: 147.888-208.132, P < 0.001) and renal interstitial edema (β = 28.833, 95% CI: 11.966-45.700, P = 0.001) correlated with serum creatinine values. CONCLUSIONS The percentage of AKI in MCD patients is significantly higher than that in MN patients. Patients over 50 years old are more likely to develop AKI. Renal tubular epithelial cell injury and renal interstitial edema may be the main pathological lesions that are associated with elevated serum creatinine in patients with MCD.
Acute Kidney Injury/etiology* ; Adolescent ; Adult ; Aged ; Child ; Cross-Sectional Studies ; Female ; Humans ; Kidney ; Male ; Middle Aged ; Nephrosis, Lipoid/complications* ; Nephrotic Syndrome/complications* ; Retrospective Studies ; Young Adult

A boy, aged 6 years and 11 months, was admitted due to nephrotic syndrome for 2 years, cough for 1 month, and shortness of breath for 15 days. The boy had a history of treatment with hormone and immunosuppressant. Chest CT after the onset of cough and shortness of breath showed diffuse ground-glass opacities in both lungs. Serum (1, 3)-beta-D glucan was tested positive, and the nucleic acid of cytomegalovirus was detected in respiratory secretions. After the anti-fungal and anti-viral treatment, the child improved temporarily but worsened again within a short period of time.
Child ; Cough/etiology* ; Cytomegalovirus Infections/therapy* ; Dyspnea/etiology* ; Extracorporeal Membrane Oxygenation ; Humans ; Male ; Nephrotic Syndrome/complications* ; Pneumonia, Pneumocystis/therapy* ; Respiratory Distress Syndrome/therapy*

To explore the risk factors of acute kidney injury(AKI)in adult primary nephrotic syndrome(PNS). Totally 185 patients with PNS were divided into AKI group(=51)and non-AKI group(=134).The demographic data and clinical and histological features at admission were compared between the two groups.The independent risk factors for AKI were evaluated by Logistics regression analysis. In 51 PNS patients with AKI,the common pathological types of AKI included minor glomerular abnormalities(29.4%),IgA nephropathy(25.5%),and membranous nephropathy(17.6%).The incidences of renal tubular casts and epithelial vacuoles in the AKI group were significantly higher than those in the non-AKI group(=0.004,=0.030).Males were more likely to suffer from AKI than females(=0.000).Patients in AKI group had significantly lower albumin level(=0.015)and higher levels of random urine protein,serum creatinine,uric acid,urea nitrogen,and triglyceride than non-AKI group(=0.030,=0.000,=0.000,=0.000,and =0.006),and polyserous and oliguria occurred more often in the AKI group(=0.000,=0.002).The AKI group had significantly higher incidences of high blood pressure and infections(=0.035,=0.000).Multivariate logistics regression analysis showed albumin(<25 g/L),serum creatinine(>96 μmol/L),urea nitrogen(≥6.8 mmol/L),uric acid(≥400 μmol/L),diabetes,infection,and renal tubular casts were the independent risk factors for AKI. AKI complicating PNS is associated with a variety of factors.Its independent risk factors include the levles of albumin,serum creatinine,urea nitrogen,and uric acid,diabetes,infections,and renal tubular casts.
Acute Kidney Injury ; etiology ; Adult ; Creatinine ; Female ; Humans ; Kidney ; Male ; Nephrotic Syndrome ; complications ; Risk Factors

To analyze the clinical characteristics of Maixuekang Capsules combined with traditional Chinese medicines in the treatment of patients with nephrotic syndrome,and provide references for improving rationality of clinical drug use. Based on the database of hospital information system(HIS) in 15 hospitals in China,the electrical medical records of the patients diagnosed as nephrotic syndrome and treated with Maixuekang Capsules were collected. Their diagnostic information and characteristics of combined traditional Chinese medicines were analyzed by using association rules. The results showed that 1 588 patients of nephrotic syndrome who used Maixuekang Capsules were often complicated with hypertension(863 cases,accounting for 7. 54%),anemia(551 cases,accounting for 4. 81%),and coronary heart disease(349 cases,accounting for 3. 05%). Maixuekang Capsules were mainly combined with Tabellae Rhei et Natrii Bicarbonatis,Baining Capsules,tanshinone,Ganmao Qingre Granule,Shuxuening Injection in treating nephrotic syndrome. The results indicated that in the real world,Maixuekang Capsules was mainly used in combination with traditional Chinese medicines such as blood-activating and stasis-removing agents,pathogens eliminating and supporting healthy Qi agents,digestants,anti-bacterial and anti-inflammatory agents,wind-dispersing and antipyretic agents for patients with nephropathy. By the pharmacological effect,it was suitable for nephropathy patients based on combined diagnosis. The association rules of combination were specific,and can provide reference for subsequent studies and rational clinical medication of traditional Chinese medicines.
Anemia ; complications ; Capsules ; China ; Coronary Disease ; complications ; Drugs, Chinese Herbal ; therapeutic use ; Humans ; Hypertension ; complications ; Medicine, Chinese Traditional ; Nephrotic Syndrome ; drug therapy

Acute Disease ; Adult ; Humans ; Iliac Artery ; Lower Extremity ; blood supply ; Male ; Nephrotic Syndrome ; complications ; Thrombosis ; etiology

OBJECTIVESchimke immuno-osseous dysplasia (SIOD), is an autosomal recessive inherited disease caused by SMARCAL1 (MIM:20606622) mutations, while in about half of the patients no any mutation in SMARCAL1 could be found. This disease involves multiple systems and is characterized by short and dissymmetric stature with spondyloepiphyseal dysplasia, progressive renal failure, lymphopenia with recurrent infections, and hyperpigmented macules. This study aimed to analyze SMARCAL1 gene of 2 unrelated suspected SIOD children, to make definite diagnosis, and find more SMARCAL1 mutation types of Chinese SIOD.

METHODTwo suspected Chinese Han male SIOD children who visited our hospital from 2008 to 2014, aged 3 y 6 m and 7 y 8 m, both were short and had spondyloepiphyseal dysplasia, progressive renal failure, lymphopenia with recurrent infections. After informed consent, they and their parents's DNA were extracted from blood. PCRs for all 16 exons of SMARCAL1 were performed and PCR products were purified by 2% gel electrophoresis and sequenced directly. Pathogenicity of missense variations was confirmed by SIFT and sequencing SMARCAL1 of fifty normal controls.

RESULT(1) Four gene variations were found in the two children: Two reported missense mutations c.1129G>C, p.Glu377Gln and c.1933C>T, p. Arg645Cys. Two splicing mutations c.1334+1G>A and c.2142-1 G>A were detected. (2) c.1129G>C, p.Glu377Gln were reported as a disease-causing mutations before, but it was an single nucleotide polymorphism (SNP) which was found in 15 of 50 normal controls. (3) Two novel splicing mutations were found in this study: c.1334+1G>A and c.2142-1 G>A.

CONCLUSION(1) We detected 3 disease-causing mutations in 2 SIOD children by SMARCAL1 gene analysis, while 2 splicing mutations were novel mutations. (2) c.1129G>C, p.Glu377Gln was a SNP but not a disease-causing mutation at least in Chinese population.

Arteriosclerosis ; complications ; genetics ; Base Sequence ; Child ; Child, Preschool ; DNA Helicases ; genetics ; Exons ; Humans ; Immunologic Deficiency Syndromes ; complications ; genetics ; Lymphopenia ; Male ; Mutation ; Mutation, Missense ; Nephrotic Syndrome ; complications ; genetics ; Osteochondrodysplasias ; complications ; congenital ; genetics ; Polymorphism, Single Nucleotide ; Pulmonary Embolism ; complications ; genetics ; Renal Insufficiency

OBJECTIVETo study the clinical characteristics of children with an initial onset of IgA nephropathy with nephrotic syndrome and compare them with children with primary nephrotic syndrome, in order to provide a theoretical basis for the differential diagnosis of the two diseases.

METHODSFifty children diagnosed with an initial onset of IgA nephropathy with nephrotic syndrome were included in this study. Seventy-two children diagnosed with an initial onset of primary nephrotic syndrome served as the control group. The clinical and laboratory examination characteristics were compared between the two groups.

RESULTSThe IgA nephropathy group had significantly higher incidence rates of gross haematuria, microscopic haematuria, hypertension, acute kidney injury, low serum high-density lipoprotein cholesterol, anemia, low serum complement C4, steroid resistance, and nephritis-type nephrotic syndrome and a significantly lower incidence of elevated serum IgE compared with the control group (P<0.05). There were significant differences in serum creatinine, serum uric acid, serum total cholesterol, serum high-density lipoprotein cholesterol, serum IgE, serum complement C4, and hemoglobin levels between the IgA nephropathy and the control groups (P<0.05). The thresholds of serum IgE (<131.2 IU/mL) and high-density lipoprotein cholesterol (<1.35 mmol/L) were reference parameters in the differential diagnosis of IgA nephropathy with nephrotic syndrome and primary nephrotic syndrome.

CONCLUSIONSChildren with IgA nephropathy presenting nephrotic syndrome manifest mainly as nephritis type and steroid-resistant type in the clinical classification. Cinical manifestations accompanied by serum levels of high-density lipoprotein cholesterol and IgE are helpful for differential diagnosis of IgA nephropathy presenting nephrotic syndrome and primary nephrotic syndrome.

Adolescent ; Child ; Child, Preschool ; Cholesterol, HDL ; blood ; Complement C4 ; analysis ; Female ; Glomerulonephritis, IGA ; blood ; complications ; Hematuria ; etiology ; Humans ; Immunoglobulin E ; blood ; Male ; Nephrotic Syndrome ; blood ; complications

Humans ; Male ; Myocardial Infarction ; complications ; Nephrotic Syndrome ; complications ; Young Adult

Blood Volume ; Child ; Edema ; etiology ; therapy ; Evidence-Based Medicine ; Hemofiltration ; methods ; Humans ; Nephrotic Syndrome ; complications ; therapy ; Renal Dialysis ; methods ; Severity of Illness Index

Adult ; Humans ; Intracranial Thrombosis ; diagnosis ; drug therapy ; etiology ; Male ; Methylprednisolone ; therapeutic use ; Nephrotic Syndrome ; complications ; drug therapy ; Venous Thrombosis ; diagnosis ; drug therapy ; etiology