Adult ; Cyclosporine ; Glomerulosclerosis, Focal Segmental/drug therapy* ; Humans ; Leflunomide/therapeutic use* ; Nephrosis, Lipoid/drug therapy* ; Nephrotic Syndrome ; Steroids

Objective: To summary the clinical presentation and prognosis of primary nephrotic syndrome (PNS) in teenagers. Methods: The clinical data, renal pathological types and prognosis of 118 children over 10-year-old with PNS treated in the Department of Nephrology of the Children's Hospital Affiliated to Capital Institute of Pediatrics from January 2010 to December 2020 were retrospectively analyzed, with 408 children ≤10-year-old as control group synchronously. Chi-square test was used to compare the difference of clinical types, pathologic types, response to steroids and tubulointerstitial changes between the groups. The teenagers with steroid resistant nephrotic syndrome (SRNS) were divided into initial non-responder group and late non-responder group. Kaplan-Meier method was used to compare the difference of persistent proteinuria, and Fisher's exact test for the histological types. Results: There were 118 children >10-year-old, including 74 males and 44 females, with the onset age of 12.1 (10.8, 13.4) years; and 408 children ≤10-year-old with the onset age of 4.5 (3.2, 6.8) years. The proportion of SRNS was significantly higher in patients >10-year-old than those ≤10-year-old (24.6% (29/118) vs. 15.9% (65/408), χ²=4.66, P=0.031). There was no statistical difference in the pathological types between >10-year-old and ≤10-year-old (P>0.05), with minimal change disease the most common type (56.0% (14/25) vs. 60.5% (26/43)). The percentage of cases with renal tubulointerstitial lesions was significantly higher in children >10-year-old compared to those ≤10-year-old (60.0% (15/25) vs. 23.3% (10/43), χ²=9.18, P=0.002). There were 29 cases presented with SRNS in PNS over 10-year-old, including 19 initial non-responders and 10 late non-responders. Analyzed by Kaplan-Meier curve, it was shown that the percentage of persistent proteinuria after 6 months of immunosuppressive treatments was significantly higher in initial non-responders than those of the late non-responders ((22±10)% vs. 0, χ²=14.68, P<0.001); the percentage of minimal change disease was significantly higher in patients of late non-responders than those of the initial non-responders (5/6 vs. 3/13, P=0.041). Of the 63 >10-year-old with steroid-sensitive nephrotic syndrome followed up more than one year, 38 cases (60.3%) had relapse, and 14 cases (22.2%) were frequent relapse nephrotic syndrome and steroid dependent nephrotic syndrome. Among the 45 patients followed up over 18-year-old, 22 cases (48.9%) had recurrent proteinuria continued to adulthood, 3 cases of SRNS progressed to kidney insufficiency, and one of them developed into end stage kidney disease and was administrated with hemodialysis. Conclusions: Cases over 10-year-old with PNS tend to present with SRNS and renal tubulointerstitial lesions. They have a favorable prognosis, but are liable to relapse in adulthood.
Male ; Female ; Adolescent ; Child ; Humans ; Nephrotic Syndrome/pathology* ; Retrospective Studies ; Nephrosis, Lipoid/drug therapy* ; Prognosis ; Proteinuria/etiology* ; Recurrence

A 60 year-old woman was admitted with generalized edema. The patient had bronchogenic adenocarcinoma which was diagnosed 8 months ago, and treated with 3 cycles of etoposide and cisplatin and 6 cycles of paclitaxel and carboplatin. After completion of chemotherapeutic cycles, massive proteinuria (18, 018 mg/day) developed. Renal biopsy revealed minimal change disease, acute tubular necrosis and chronic interstitial nephritis. In spite of continuous chemotherapy, there was no evidence of remission of cancer lesion on a serial consecutive radiographic study. She quitted continuing chemotherapy, and alternative day high dose of prednisolone was initiated for minimal change disease. Proteinuria was decreased dramatically (180 mg/day) after 2 months, and did not recur during tapering of prednisolone. Although bone metastasis on the right femur was newly detected after 2 months, proteinuria did not develop. We experienced minimal change disease during chemotheraphy of bronchogenic adenocarcinoma, thus we report it with article review.
Adenocarcinoma* ; Biopsy ; Carboplatin ; Cisplatin ; Drug Therapy* ; Edema ; Etoposide ; Female ; Femur ; Humans ; Middle Aged ; Necrosis ; Neoplasm Metastasis ; Nephritis, Interstitial ; Nephrosis, Lipoid* ; Paclitaxel ; Prednisolone ; Proteinuria

We report treatment of a 38-year-old man with minimal change disease (MCD) who developed pneumatosis intestinalis (PI) during administration of immune-suppressive agents. His immunosuppressive medication had been tapered to 15 mg/day of prednisolone. MCD was steroid-resistant type. Abdominal examination and laboratory studies were not clinically remarkable. Radiologic findings were consistent with PI. Abnormal air accumulation was noted in the bowel, peritoneum, mediastinum and retroperitoneum. Conservative therapy with oxygen and metronidazole improved the PI symptoms. In 1993, a case of PI with nephrotic syndrome following steroid treatment was reported in Japan. However this is only the second case reported in the literature, and the first in English.
Adult ; Case Report ; Human ; Immunosuppressive Agents/*adverse effects ; Male ; Nephrosis, Lipoid/*drug therapy ; Pneumatosis Cystoides Intestinalis/*chemically induced ; Prednisolone/*adverse effects

We experienced a 22-year old patient with a documented history of minimal change nephrotic syndrome (MCNS), and a diagnosis of acute lymphoblastic leukemia (ALL) was then made for this patient. The patient received standard daily steroid therapy for the treatment of nephrotic syndrome. Cyclosporin A was administered because there was no clinical improvement with steroid therapy. Six years after the diagnosis of nephrotic syndrome, the patient was diagnosed with ALL. After chemotherapy for ALL, the patient was in complete remission and he showed clinical improvement of nephrotic syndrome. The hematological malignancies associated with nephrotic syndrome are mainly lymphoma and chronic lymphocytic leukemia. ALL has rarely been described in combination with nephrotic syndrome. Although the exact mechanism for development of ALL after nephrotic syndrome is unknown, at least two possibilities exist. First, the incidence of leukemia may be increased after immunosuppressive therapy, which may include cyclosporin A. Second, the underlying defect in T-lymphocyte function could account for both nephrotic syndrome and ALL. The possible mechanisms for such a relationship are discussed here along with a review of the relevant literature.
Cyclosporine ; Diagnosis ; Drug Therapy ; Hematologic Neoplasms ; Humans ; Incidence ; Leukemia ; Leukemia, Lymphocytic, Chronic, B-Cell ; Lymphoma ; Nephrosis, Lipoid* ; Nephrotic Syndrome ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; T-Lymphocytes ; Young Adult

OBJECTIVETo screen the differentially expressed proteins in the urine of children with steroid-sensitive and steroid-resistant minimal change nephrotic syndrome (SRINS and SSINS, respectively).

METHODSUrine samples were collected from 10 children with SRINS and 70 with SSINS as well as 30 healthy volunteers (control). Isoelectric focusing and two-dimensional electrophoresis in combination with matrix assisted laser desorption/ionization-time of flight (MALDI-TOF) mass spectrometry was performed for analysis of the urine proteins.

RESULTS AND CONCLUSIONIn the urine samples, 30 protein spots were identified to have differential expression between SRINS and SSINS. Further analysis of 14 protein spots identified 12 proteins expressing in SRINS, namely kinesin family member 27, PITPNB, bullous pemphigoid antigen, alpha-1 protease inhibitor, Zn-alpha-2GP, alpha-1B-glycoprotein, serum albumin precursor, haptoglobin precursor, kinesin like motor protein, IRAK4, cytoplasmic dynein and cytokeratin 9. Nine of these 12 proteins were up-regulated (U1-U3, U5, U7-U9, U11-U12) and 3 down-regulated (D4, D6, D10) in SRINS, suggesting that these proteins may serve as the potential therapeutic targets and as new diagnostic markers for steroid-resistant nephrotic syndrome.

Adolescent ; Case-Control Studies ; Child ; Child, Preschool ; Electrophoresis, Gel, Two-Dimensional ; Female ; Humans ; Male ; Nephrosis, Lipoid ; drug therapy ; urine ; Proteins ; chemistry ; Proteomics ; Steroids ; therapeutic use ; Urine ; chemistry

OBJECTIVE: To determine the factors associated with the eff ect of steroid on adult primary nephrotic syndrome. METHODS: The general information, laboratory examination and renal pathological type of 425 patients with primary nephrotic syndrome were retrospectively analyzed. RESULTS: Th ere were significant differences in the response to steroid among the pathological types of minimal change disease, focal segmental glomerulosclerosis and IgA nephropathy. Th e patients in the age of 14-24 years old showed the strongest response to steroid (P<0.05). Th e IgA level in the steroid resistance group was lower than that in the non-steroid resistance group (P<0.05). There was no significant difference in urine protein in 24 hour quantitation in the steroid resistance group between pre- and post-treatment (P>0.05), while there was significant difference in urine protein in 24 hour quantitation in the non-steroid resistance group between pre- and post-treatment (P<0.05). CONCLUSION Pathological types and ages of the patients are related to the steroid curative effect. The decrease in IgA probably affects the effect of steroid on primary nephrotic syndrome.
Adolescent ; Adult ; Glomerulonephritis, IGA ; drug therapy ; Glomerulosclerosis, Focal Segmental ; drug therapy ; Humans ; Kidney ; physiopathology ; Nephrosis, Lipoid ; drug therapy ; Nephrotic Syndrome ; drug therapy ; Proteinuria ; Retrospective Studies ; Steroids ; therapeutic use ; Urinalysis ; Young Adult

OBJECTIVETo study the significance of trace immunoglobulin M (IgM) deposits in glomerular mesangium in children with minimal change primary nephrotic syndrome (PNS).

METHODSOne hundred and six children who were clinically diagnosed with PNS and pathologically diagnosed with minimal change disease (MCD) and trace deposition of IgM in renal tissues were enrolled as subjects. Eighty-one PNS children with MCD but no deposition of immune complexes were used as the control group. The clinical characteristics and efficacies of glucocorticoids and immunosuppressants were retrospectively analyzed in the two groups. All patients were given full-dose prednisone by oral administration, and patients with glucocorticoid resistance or frequent relapses were additionally given immunosuppressants.

RESULTSThe incidence of glucocorticoid resistance in the IgM deposit group was significantly higher than that in the control group (27.2% vs 12.3%; P<0.05). The incidence of frequent relapses in the IgM deposit group was also significantly higher than that in the control group (48.1% vs 10.4%; P<0.05). The complete remission rate for glucocorticoid-resistant patients treated with prednisone combined with mycophenolate mofetil (MMF) was 68% and 62% respectively in the IgM deposit and control groups (P>0.05). The relapse frequency in patients with frequent relapses was significantly reduced in both groups after treatment with prednisone and MMF in combination (P<0.05).

CONCLUSIONSTrace deposition of IgM in renal tissues may be an important factor for glucocorticoid resistance and frequent relapses in PNS children with MCD. Prednisone combined with MMF may be a better choice in the treatment of patients with glucocorticoid resistance or frequent relapses.

Adolescent ; Child ; Child, Preschool ; Drug Resistance ; Female ; Glomerular Mesangium ; immunology ; Glucocorticoids ; therapeutic use ; Humans ; Immunoglobulin M ; analysis ; Immunosuppressive Agents ; therapeutic use ; Infant ; Male ; Nephrosis, Lipoid ; drug therapy ; immunology ; Retrospective Studies

The World Health Organization classifies lupus nephritis as class I to V or VI. However, a few cases of minimal change glomerulopathy have been reported in association with systemic lupus erythematosus (SLE). Mycophenolate mofetil has been shown to be effective for treatment of minimal change disease and lupus nephritis. A 24-year-old woman diagnosed with SLE five years prior to presentation complained of a mild generalized edema. The urinalysis showed microscopic hematuria and proteinuria. The assessed amount of total proteinuria was 1,618 mg/24 hours. A renal biopsy demonstrated diffuse fusion of the foot processes of podocytes on electron microscopy. Mycophenolate mofetil was started in addition to the maintenance medications of prednisolone 10 mg/day and hydroxychloroquine 400 mg/day. After six months of treatment, the microscopic hematuria and proteinuria resolved, and the total urine protein decreased to 100 mg/24 hours.
Antirheumatic Agents/therapeutic use ; Female ; Glucocorticoids/therapeutic use ; Humans ; Hydroxychloroquine/therapeutic use ; Immunosuppressive Agents/*therapeutic use ; Lupus Erythematosus, Systemic/complications/*pathology ; Mycophenolic Acid/*analogs & derivatives/therapeutic use ; Nephrosis, Lipoid/*drug therapy/etiology/pathology ; Prednisone/therapeutic use ; Young Adult

There are several reports to demonstrate that rifampicin, a major anti-tuberculosis agent, is associated with some adverse renal effects, with a few cases of rifampicin-induced minimal change disease (MCD). In the present case, a 68-year-old female presented with nausea, vomiting, foamy urine, general weakness and edema. She had been taking rifampicin for 4 weeks due to pleural tuberculosis. The patient had no proteinuria before the anti-tuberculosis agents were started, but urine tests upon admission showed heavy proteinuria with a 24-h urinary protein of 9.2 g/day, and serum creatinine, albumin, and total cholesterol levels were 1.36 mg/dL, 2.40 g/dL, and 283 mg/dL, respectively. MCD was diagnosed, and the patient achieved complete remission after cessation of rifampicin without undergoing steroid therapy.
Aged ; Antibiotics, Antitubercular/*adverse effects/therapeutic use ; Edema/etiology ; Female ; Humans ; Kidney Function Tests ; Kidney Glomerulus/pathology ; Nausea/etiology ; Nephrosis, Lipoid/*chemically induced/pathology ; Proteinuria ; Remission Induction ; Rifampin/*adverse effects/therapeutic use ; Treatment Outcome ; Tuberculosis, Pleural/*drug therapy