Colquhounia root tablet in the treatment of idiopathic membranous nephropathy with subnephrotic proteinuria.

PURPOSE: Idiopathic membranous nephropathy (IMN) is the most frequent global cause of nephrotic syndrome in non-diabetic people. In clinical practice, An effective and mild treatment for IMN patients with subnephrotic proteinuria has been adopted. Colquhounia root tablet (CRT) is a traditional Chinese medicine that is widely used in China to treat glomerulopathies. In this study, the effectiveness and safety of CRT in the treatment of IMN with subnephrotic proteinuria have been determined by reviewing the clinical records of 44 patients with IMN. METHODS: Retrospective analysis of IMN patients with subnephrotic proteinuria treated with CRT in combination with ACEI/ARB or ACEI/ARB alone. The remission rate (complete or partial remission) was the main outcome observed, and proteinuria, estimated glomerular filtration rate (eGFR), serum albumin levels, and adverse effects were the secondary outcomes. RESULTS: This clinical trial included 44 patients, and the overall remission rates at months 6, 9, and 12 after treatment were 68.2% versus 27.3% (p = 0.016), 72.7% versus 36.4% (p = 0.015), and 77.3% versus 36.4% (p = 0.006) in the treatment and control groups, respectively. The application of CRT treatment was an independent predictor of proteinuria remission (p = 0.024). In addition, in patients who were positive for phospholipase A2 receptor (PLA2R) antibodies, the overall remission rate was higher in the treatment group than in the control group after 9 months of treatment (75% versus 23.08%, p = 0.017). CONCLUSION: This retrospective study illustrates that, based on supportive therapy, CRT could be effective in the treatment of IMN with subnephrotic proteinuria with a good safety profile at the same time.

Membranous nephropathy caused by dimercaptosuccinic acid in a patient with Wilson's disease: a case report and literature review.

BACKGROUND: Dimercaptosuccinic acid (DMSA) therapy is a kind of chelation therapy for patients with Wilson 's disease (WD). While there have been reports of side effects associated with DMSA, the development of membranous nephropathy as a result of this therapy is uncommon. CASE PRESENTATION: We present a case of a 19-year-old male patient with Wilson's disease who experienced proteinuria while receiving long-term DMSA treatment. Further evaluation revealed abnormally low levels of serum ceruloplasmin and serum albumin, as well as a 24-hour urinary protein excretion of 4599.98 mg/24 h. A renal biopsy confirmed the presence of membranous nephropathy. After ruling out other potential causes, we determined that the patient's membranous nephropathy was likely caused by DMSA. Following treatment with glucocorticoids, there was a significant reduction in proteinuria. CONCLUSION: This case highlights the possibility of DMSA-induced membranous nephropathy and the importance of considering this diagnosis in patients receiving DMSA treatment. Given the widespread use of DMSA in the treatment of Wilson's disease, further research is needed to fully understand the potential role of this drug in the development of membranous nephropathy.

Chinese medicinal herbs for idiopathic membranous nephropathy in adults with nephrotic syndrome: A systematic review of effectiveness and safety.

ABSTRACT: To assess the benefits and harms of Chinese medicinal herbs formulae for the treatment of idiopathic membranous nephropathy in adult patients with primary nephrotic syndrome.Only randomized controlled trials were included. We searched the Cochrane Central Register of Controlled Trials database, PubMed, EMBASE, Chinese National Knowledge Internet, Chinese Biomedicine Database, and VIP. All studies were analyzed using the criteria of the Cochrane Handbook and were assessed in terms of quality and the risk of bias. Review Manager ver. 5.3.5 software was used for the data analysis, and GRADE profiler software was employed to evaluate quality.Two studies were included (n = 126 Chinese participants). We found that compared with against conventional treatment, one Chinese medicinal herbs formula plus conventional treatment reduced 24-hours urinary total protein (mean differences -3.16 g/24 h, 95% confidence intervals -4.03 to -2.29), and two Chinese medicinal herbs formulae increased serum albumin levels (mean differences 3.18 g/L, 95% confidence intervals 1.12 to 5.52; I2 = 0%).Chinese medicinal herbs formulae may reduce 24-hours urinary total protein and increase serum levels of albumin. However, larger and multicenter studies with high methodological quality are still needed.

[Dystrophic Calcinosis Cutis: a rare fearsome issue of Chronic Kidney Disease].

Disorders of calcium-phosphate-parathormone balance, are very important issues in ESRD patients, that may lead to severe complications, as dystrophic calcinosis cutis, a rare disease, caused by calcium salt deposits in cutaneous or subcutaneous tissues and many organs. We present the case of a 47 years old woman, in ESRD due to membranous glomerulopathy, treated by peritoneal dialysis, who, after 7 months of dialysis, developed painful masses on second finger and fifth metacarpus of the right hand. Laboratory and instrumental data showed hyperparathyroidism with a parathyroid mass consistent with adenoma. Increasing of therapy with phosphate binders and cinacalcet only, was not effective to solve cutaneous masses, that were biopsied. Histological exam revealed deposition of amorphic material with calcific component, consistent with cutaneous dystrophic calcinosis. We further increased dialysis and therapy and we observed complete regression of masses in 2 months.

[A Case of Advanced Transverse Colon Cancer with Nephrotic Syndrome Treated with Curative Resection and Complete Adjuvant Chemotherapy].

A 74-year-old woman was diagnosed as having transverse colon cancer after diagnosis of nephrotic syndrome caused by membranous nephropathy. Although she had hypoproteinemia and hypoalbuminemia, we judged that she had no major nutritional problem. In previous, similar case reports, the use of human serum albumin and fresh-frozen plasma was suggested to be important to avoid complications in the perioperative period. Thus, we used the same in our patient in the perioperative period. In addition, we paid special attention to perioperative nutrition management and used total parenteral nutrition in perioperative period. We performed laparoscopic assisted right hemicolectomy. On the 15th day after the surgical resection, the patient was discharged without any problems. We considered that postoperative adjuvant chemotherapy with XELOX (CapeOX)should be performed because the TNM pathological stage was pStage III b. Regarding adjuvant chemotherapy for gastrointestinal cancer with nephrotic syndrome, no previous reports detailed the indications for postoperative adjuvant chemotherapy. Upon introduction of adjuvant chemotherapy, we determined adaptation in accordance with the general adaptation criteria. While observing the patient's progress with a nephrologist, we safely completed the scheduled 8 courses adjuvant chemotherapy.

Characteristics of patients diagnosed with renal vein thrombosis and glomerulopathy: a case series.

BACKGROUND: Few contemporary studies have evaluated the clinical characteristics of patients with biopsy-proven glomerulopathy diagnosed with renal vein thrombosis (RVT). METHODS: Retrospective case series study within an integrated health system in a 12-year period (January 1, 2000 through December 31, 2011) investigating clinical characteristics of all adult patients who underwent native or transplant kidney biopsy and also had a diagnosis of RVT. Patient characteristics, diagnostic studies, and outcomes were evaluated. RESULTS: Among 3763 eligible patients, 17 had imaging confirmed RVT. Of these, 15 had membranous nephropathy (idiopathic or secondary to autoimmune disease). Although the biopsy population included primary and secondary glomerular disease patients, all 17 RVT patients had severe nephrotic syndrome and profound hypoalbuminemia with mean (SD) of albumin: 1.5 g/dL (0.66). CONCLUSION: Clinically significant RVT in patients with glomerulopathy appears to be a rather rare entity, occurring predominantly in patients with severe nephrotic syndrome due to idiopathic membranous nephropathy and membranous nephropathy secondary to autoimmune disease.

Clinicopathological features and prognosis of Kimura's disease with renal involvement in Chinese patients.

AIMS: Kimura's disease (KD) with renal involvement is a rare disease. Optimal treatments are still not well established. It is necessary to analyze clinicopathological features, treatment responses, and prognosis for improving KD diagnosis and treatment. MATERIALS AND METHODS: Clinicopathological data, treatment responses, and prognosis were collected and analyzed retrospectively. RESULTS: The patients consisted of 27 males and 2 females, with an average age of 35.5 ± 15.1 (13 - 61) years. 27 exhibited proteinuria ranging from 0.730 to 14.1 g/24 h (5.98 ± 3.40 g/24 h). Hypertension, renal insufficiency (serum creatinine (Scr) > 1.24 mg/dL), and microhematuria occurred in 4 (13.8%), 11 (37.9%), and 13 (44.8%) cases, respectively. Light microscopy (LM) identified mesangium proliferation, minimal change, focal and segmental glomerulosclerosis (FSGS), membranous glomerulonephritis, membranoproliferative glomerulonephritis (MPGN), and acute tubular necrosis in 14, 8, 3, 2, 1, and 1 cases, respectively. All were treated with Tripterygium wilfordii (TW), prednisone, leflunomide (LEF), tacrolimus (FK506), myophenolate mofetil (MMF), or renin-angiotensin system blockers (RASI). 26 patients were followed up for 1.60 - 108.7 months (39.6 ± 28.7). After treatments, urinary red blood cells (RBC) decreased in all. The amount of 24-hour urinary protein (24-hUPE) decreased in 24 patients. 22 reached complete remission (CR), 4 partial remissions (PR). The patients who did not relapse were younger than those who relapsed. CONCLUSIONS: KD with renal involvement occurs predominantly among 35 - 50 year old Chinese patients with male predilection. The most common features are proteinuria, hypertension, micro hematuria with minimal change, and mesangial proliferative glomerulonephritis. Most were responsive to treatment, but could relapse. Gender, age, and hypertension are associated with KD recurrence. The prognosis is good mostly.

Carlos II: Del hechizo a su patología génito-urinaria / Charles II: From spell to genitourinary pathology

OBJETIVO: Tratar de desentrañar la compleja patología del último rey de la dinastía de los Austrias, Carlos II, apodado El Hechizado, dentro de la cual la urogenital fue preponderante, impidiéndole cumplir con una de las finalidades de la institución monárquica cual es la transmisión a un descendiente y produciéndole una serie de complicaciones que le llevaron al fallecimiento.MÉTODOS: Revisamos las obras en las cuales se describe la vida del Rey, haciendo hincapié en sus antecedentes consanguíneos, en las dudas sobre su sexo en el momento del nacimiento, sus procesos patológicos, la repercusión en las Cortes Europeas, pero sobre todo en sus matrimonios y en la incapacidad para generar un heredero. Resaltamos como, siguiendo el pensamiento de la sociedad española del siglo XVII, hizo pensar que se encontraba hechizado. Sobrenombre con el que pasó a la historia.RESULTADO: Deducimos que pudo presentar un hipospadias posterior que junto con la monorquia y testículo atrófico, hace pensar que presentó un estado intersexual con genitales ambiguos. Su fenotipo físico inclina más hacia un hermafroditismo verdadero y sobre todo un varón XX, que hacia un síndrome de Klinefelter que ha sido el más atribuido. Es probable su asociación con un síndrome X frágil. Monorreno congénito muy posiblemente, su muerte se debió a una insuficiencia renal crónica producida por una glomerulopatía o una nefropatía intersticial a consecuencia de una litiasis renal más infecciones del tracto urinario recidivantes.CONCLUSIONES: Fruto de una reiterada política matrimonial endogámica, feneció en 1700 la dinastía de los Habsburgo en España encarnada en Carlos II, un monarca pluripatológico que sólo se libraría de especulaciones si se efectuaran estudios cromosómicos y genéticos de sus restos presentes en el monasterio de El Escorial(AU)

OBJECTIVES: We attempt to unravel the complex condition of the last king of the Hapsburg dynasty in Spain, Charles II, called The Bewitched, in whom a genitourinary disorder was preponderant, preventing him from fulfilling one of the objectives of the monarchial institution, engendering a heir, and causing a series of complications that led to his death. METHODS: We review the works describing the life of the King, with special emphasis on his bloodline, the doubts about his sex at birth, his pathological processes, the repercussion among European Courts, but above all on his marriages and the inability to engender an heir. We also emphasize the thought of 17th century Spanish society which led to the belief that he was bewitched. The nickname he passed into history with.RESULT: It was deduced that he could have presented posterior hypospadias which, together with monorchism and atrophic testicle, led to the belief that he presen-ted an intersexual state with ambiguous genitals. The physical phenotype leans more towards true hermaphro-ditism and above all a XX male, rather than the more often attributed Klinefelter’s syndrome. This is probably also associated with a fragile X syndrome. Very possibly congenital monorenal, death was due to chronic kidney failure caused by glomerulopathy or interstitial nephro-pathy as a consequence of renal lithiasis plus recurrent infections of the urinary tract.CONCLUSIONS: As a result of a reiterated endogamic matrimonial policy, the Hapsburg dynasty died out in Spain in 1700, represented by Charles II, a pluripatho-logical king who can only be freed from speculation by chromosomal and genetic studies of his remains buried in El Escorial monaster(AU)

[Experimental study on treatment of chronic renal failure with yishen qudu capsule].

OBJECTIVE: To study the mechanism of action of Yishen Qudu capsule (YSQDC) in experimental rat model of chronic renal failure (CRF). METHODS: Membranous nephropathy model was induced by cationized bovine serum albumin intravenous administration in rats for 7 weeks and developed to CRF. The model was then treated with YSQDC of large or small dose by gastrogavage for 5 weeks. Oxidated starch was used in the control group. RESULTS: YSQDC could reduce proteinuria, serum creatinine, blood urea nitrogen, total cholesterol, triglyceride and serum circulating immune complex (P < 0.01 or P < 0.05), it also reduced the kidney coefficient, glomerular cells and tubular protein cast significantly (all P < 0.01). The strength of immune fluorescence weakened remarkably (P < 0.01). The pathological lesions improved significantly after treatment under light and electron-microscopic examination. CONCLUSION: YSQDC could improve renal function significantly and inhibit the progression of CRF.

Possible associations of rectal carcinoma with Schistosoma japonicum infection and membranous nephropathy: a case report with a review.

We report the first case of rectal carcinoma associated with S. japonicum and membranous nephropathy. A 57-year-old Japanese man noticed narrowing of his feces. He had lived in Yamanashi prefecture, an endemic area of S. japonicum. He had suffered from nephrotic syndrome for about 1 year. Barium enema study showed a severe stricture in the upper rectum and biopsy specimens from the tumor demonstrated well differentiated adenocarcinoma and many ova of S. japonicum. Sonography of the liver showed a network pattern and a linear high echoic area. Low anterior resection with incisional biopsy of the liver and the right kidney was performed. Histopathological findings showed well differentiated adenocarcinoma and schistosomal ova. The total number of ova in the resected colon amounted to 15,133, consisting of 2243 inside and 12,890 outside the carcinoma. The nearer to the carcinoma the area was, the higher was the density of ova. The findings of light microscopy and electron microscopy of the biopsy specimen from the kidney were compatible with membranous nephropathy (stage II). This case suggests that schistosomal ova have some effect on carcinogenesis and nephrotic syndrome. In patients with nephrotic syndrome of unknown cause, especially in inhabitants of endemic areas of S. japonicum, gastrointestinal malignancy should be ruled out as an etiological factor. Sigmoidoscopy would be useful for colorectal carcinoma surveillance in S. japonicum patients.

Micro-angiopathic haemolysis, thrombocytopenia and nephrotic syndrome associated with membranous nephropathy in a Vietnamese boy.

The unique association of idiopathic diffuse membranous nephropathy and micro-angiopathic haemolytic anaemia and thrombocytopenia is described. A 7 year old Vietnamese boy with a 1-month history of anaemia resistant to oral iron supplements presented with acute onset of nephrotic syndrome. Investigations revealed a micro-angiopathic haemolytic anaemia and thrombocytopenia. There was no associated oliguria or uraemia. Diffuse membranous nephropathy was diagnosed by renal biopsy. Apart from a fourfold rise in enterovirus titres, no underlying cause for the haematological or glomerular abnormalities was found. There was an apparent, partial haematological response to fresh frozen plasma infusions, but not to Vitamin E.