FEATURES OF THE CLINICAL COURSE OF MEMBRANOUS NEPHROPATHY

Vestnik ◽  
2021 ◽  
pp. 155-157
Author(s):  
Л.П. Мамедова ◽  
А.К. Беисбекова
Keyword(s):  
Nephrotic Syndrome ◽  
Clinical Manifestation ◽  
Immune Complexes ◽  
Membranous Nephropathy ◽  
Clinical Course ◽  
The Body ◽  
High Cholesterol ◽  
Vascular Walls ◽  
Progressive Stage ◽  
Main Clinical Manifestation

Мембранозная нефропатия является иммунологически обусловленной не воспалительной гломерулопатией, имеет прогрессирующее стадийное течение и характеризуется дистрофическими и склеротическими процессами. Мембранозная нефропатия - это поражение почечных клубочков (основные функциональные единицы почек), возникающее вследствие оседания на стенках почечных капилляров иммунных комплексов. Это приводит к утолщению и дальнейшему расслоению базальных мембран и стенок сосудов в клубочках. Основным клиническим проявлением МН является нефротический синдром (отеки по всему телу, наличие белка в моче и высокий холестерин в крови). Membranous nephropathy is an immunologically determined non-inflammatory glomerulopathy, has a progressive stage course and is characterized by dystrophic and sclerotic processes. Membranous nephropathy is a lesion of the renal glomeruli (the main functional units of the kidneys) that occurs due to the settling of immune complexes on the walls of the renal capillaries. This leads to thickening and further delamination of the basal membranes and vascular walls in the glomeruli. The main clinical manifestation of MN is nephrotic syndrome (edema throughout the body, the presence of protein in the urine and high cholesterol in the blood).

Clinical case of membranous nephropathy in a patient with IgG4-related disease

2020 ◽  
Vol 24 (4) ◽  
pp. 110-121
Author(s):  
M. O. Pyatchenkov ◽  
O. A. Vorobyeva ◽  
A. N. Belskykh ◽  
M. V. Zakharov ◽  
M. Y. Dendrikova
Keyword(s):  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
The Body ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Pathologic Features ◽  
Wide Range ◽  
Serum Igg4

IgG4-related disease (IgG4-RD) currently is considered as a chronic fibroinflammatory immune-mediated multisystemic condition of unidentified etiology, which can imitate a wide range of malignant, infectious, rheumatologic, and other diseases. It can affect almost any organ system in the body synchronously or sequentially, but the most often affected are the pancreas, hepatobiliary tract, periorbital structures, salivary glands, kidneys, and lymph nodes. The most frequent renal manifestations of IgG4-RD is IgG4-related tubulointerstitial nephritis. Membranous nephropathy is the most common glomerular disease accompanied by IgG4-RD. Regardless of the organ localization, patients with IgG4-RD are characterized by elevated serum IgG4, but this laboratory abnormality is not specific and can be changed in other diseases. In all suspected cases of IgG4-RD the diagnosis should be confirmed by histological examination. Characteristic pathologic features include diffuse or focal lymphoplasmacytic infiltration with prominent IgG4+ plasma cells, storiform fibrosis, and obliterative phlebitis. Patients with IgG4- RD usually have an excellent clinical response to glucocorticoids, but relapse rates after steroid withdrawal are high, which may require additional use of immunosuppressants or rituximab. Due to the low prevalence and multitude of clinical manifestations the disease often remains underdiagnosed on time. This case report describes middle-aged patients with a history of chronic recurrent pancreatitis complicated by the nephrotic syndrome. Kidney biopsy showed membranous nephropathy and diagnosis IgG4-RD with multiorgan involvement was made. Partial remission was achieved on corticosteroid therapy. The presented case clearly demonstrates the difficulties of diagnosis and treatment of IgG4-RD. IgG4-related membranous nephropathy should be included in the differential diagnosis for patients with nephrotic syndrome accompanied by multiorgan dysfunction.

scholarly journals Murine Membranous Nephropathy: Immunization with α3(IV) Collagen Fragment Induces Subepithelial Immune Complexes and FcγR-Independent Nephrotic Syndrome

2012 ◽  
Vol 188 (7) ◽  
pp. 3268-3277 ◽  
Author(s):  
Jun-Jun Zhang ◽  
Mahdi Malekpour ◽  
Wentian Luo ◽  
Linna Ge ◽  
Florina Olaru ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Immune Complexes ◽  
Membranous Nephropathy ◽  
Collagen Fragment

ACE-2-Receptors of the Epidermis, Dermal Vascular Walls and Sebaceous Gland Cells: The Way of COVID-19 Entry into the Body?

2020 ◽  
Author(s):  
Stefan Bittmann
Keyword(s):  
Clinical Trials ◽  
Viral Infection ◽  
Sebaceous Gland ◽  
The Body ◽  
Viral Agent ◽  
Therapy Options ◽  
Vascular Walls ◽  
Fish Market ◽  
The World ◽  
The Usa

Since the outbreak near a fish market in Wuhan, China, in December 2019, researchers have been searching for an effective therapy to control the spreading of the new coronavirus SARS-CoV-2 and inhibit COVID-19 infection. Many countries like Italy, Spain, and the USA were ambushed by this viral agent. To date, more than 2.5 million people were infected with SARS-CoV-2. There is no clear answer, why SARS-CoV-2 infects so many people so fast. To date of April 2020, no effective drug has been found to treat this new severe viral infection. There are many therapy options under review and clinical trials were initiated to get clearer information, what kind of drug can help in this devastating and serious situation. The world has no time.

scholarly journals Membranous nephropathy associated with multicentric Castleman’s disease that was successfully treated with tocilizumab: a case report and review of the literature

2021 ◽  
Vol 22 (1) ◽  
Author(s):  
Ryosuke Saiki ◽  
Kan Katayama ◽  
Yosuke Hirabayashi ◽  
Keiko Oda ◽  
Mika Fujimoto ◽  
...  
Keyword(s):  
Membranous Nephropathy ◽  
Periodic Acid ◽  
Castleman’S Disease ◽  
Basement Membranes ◽  
The Body ◽  
Castleman's Disease ◽  
Multicentric Castleman’S Disease ◽  
High Zinc ◽  
Secondary Membranous Nephropathy ◽  
Multicentric Castleman's Disease

Abstract Background Multicentric Castleman’s disease is a life-threatening disorder involving a systemic inflammatory response and multiple organ failure caused by the overproduction of interleukin-6. Although renal complications of Castleman’s disease include AA amyloidosis, thrombotic microangiopathy, and membranoproliferative glomerulonephritis, membranous nephropathy is relatively rare. We experienced a case of secondary membranous nephropathy associated with Castleman’s disease. Case presentation The patient was a 43-year-old Japanese man who had shown a high zinc sulfate value in turbidity test, polyclonal hypergammaglobulinemia, anemia, and proteinuria. A physical examination revealed diffuse lymphadenopathy, an enlarged spleen and papulae of the body trunk. A skin biopsy of a papule on the patient’s back showed plasma cells in the perivascular area and he was diagnosed with multicentric Castleman’s disease, plasma cell variant. Kidney biopsy showed the appearance of bubbling in the glomerular basement membranes in Periodic acid methenamine silver stain and electron microscopy revealed electron dense deposits within and outside the glomerular basement membranes. Since immunofluorescence study showed predominant granular deposition of IgG1 and IgG2, he was diagnosed with secondary membranous nephropathy associated with Castleman’s disease. He was initially treated with prednisolone alone, however his biochemical abnormalities did not improve. After intravenous tocilizumab (700 mg every 2 weeks) was started, his C-reactive protein elevation, anemia, and polyclonal gammopathy improved. Furthermore, his urinary protein level declined from 1.58 g/gCr to 0.13 g/gCr. The prednisolone dose was gradually tapered, then discontinued. He has been stable without a recurrence of proteinuria for more than 6 months. Conclusions Tocilizumab might be a treatment option for secondary membranous nephropathy associated with Castleman’s disease.

Nephrotic syndrome secondary to alpha-1 antitrypsin deficiency

2021 ◽  
Vol 14 (3) ◽  
pp. e240288
Author(s):  
Gabriela F Santos ◽  
Paul Ellis ◽  
Daniela Farrugia ◽  
Alice M Turner
Keyword(s):  
Nephrotic Syndrome ◽  
Membranous Nephropathy ◽  
Clinical Investigation ◽  
Genetic Disorder ◽  
Clinical Manifestations ◽  
Protective Role ◽  
Caucasian Woman ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin

We report a 64-year-old caucasian woman diagnosed with membranous nephropathy secondary to alpha-1 antitrypsin deficiency (AATD). AATD is a rare autosomal codominant genetic disorder. Its clinical manifestations are mostly observed in the lungs, with early-onset emphysema. Nephropathy due to AATD is still very rare and only a few cohort studies have been reported. It has been recognised that alpha-1 antitrypsin has a protective role in the kidneys which enhances the possibility of development of kidney failure, such as nephrotic syndrome, in cases of AATD. Further clinical investigation is needed to understand the relationship between the development of nephropathy, namely membranous nephropathy, and AATD.

The clinical course and role of surgery in pediatric malignant peripheral nerve sheath tumors: a database study

2021 ◽  
pp. 1-8
Author(s):  
Victor M. Lu ◽  
Shelly Wang ◽  
David J. Daniels ◽  
Robert J. Spinner ◽  
Allan D. Levi ◽  
...  
Keyword(s):  
Overall Survival ◽  
Radiation Therapy ◽  
Peripheral Nerve ◽  
Pediatric Patients ◽  
Clinical Course ◽  
The Body ◽  
National Database ◽  
Nerve Sheath Tumors ◽  
Peripheral Nerve Sheath Tumors ◽  
Nerve Sheath

OBJECTIVE Malignant peripheral nerve sheath tumors (MPNSTs) are rare tumors found throughout the body, with their clinical course in children still not completely understood. Correspondingly, this study aimed to determine survival outcomes and specific clinical predictors of survival in this population from a large national database. METHODS All patients with MPNSTs aged ≤ 18 years in the US National Cancer Database (NCDB) between 2005 and 2016 were retrospectively reviewed. Data were summarized, and overall survival was modeled using Kaplan-Meier and Cox regression analyses. RESULTS A total of 251 pediatric patients with MPNSTs (132 [53%] females and 119 [47%] males) were identified; the mean age at diagnosis was 13.1 years (range 1–18 years). There were 84 (33%) MPNSTs located in the extremities, 127 (51%) were smaller than 1 cm, and 22 (9%) had metastasis at the time of diagnosis. In terms of treatment, surgery was pursued in 187 patients (74%), chemotherapy in 116 patients (46%), and radiation therapy in 129 patients (61%). The 5-year overall survival rate was estimated at 52% (95% CI 45%–59%), with a median survival of 64 months (range 36–136 months). Multivariate regression revealed that older age (HR 1.10, p < 0.01), metastases at the time of diagnosis (HR 2.14, p = 0.01), and undergoing biopsy only (HR 2.98, p < 0.01) significantly and independently predicted a shorter overall survival. Chemotherapy and radiation therapy were not statistically significant. CONCLUSIONS In this study, the authors found that older patient age, tumor metastases at the time of diagnosis, and undergoing only biopsy significantly and independently predicted poorer outcomes. Only approximately half of patients survived to 5 years. These results have shown a clear survival benefit in pursuing maximal safe resection in pediatric patients with MPNSTs. As such, judicious workup with meticulous resection by an expert team should be considered the standard of care for these tumors in children.

A CASE OF MEMBRANOUS NEPHROPATHY IN WHICH GASTRECTOMY FOR A GASTRIC CANCER LED TO A REMISSION OF NEPHROTIC SYNDROME

1997 ◽  
Vol 58 (8) ◽  
pp. 1864-1868 ◽  
Author(s):  
Kentaro EMOTO ◽  
Tadateru TAKAHASHI ◽  
Yoshitaka KATO ◽  
Takayuki NOMIMURA ◽  
Ichiro OMORI ◽  
...  
Keyword(s):  
Gastric Cancer ◽  
Nephrotic Syndrome ◽  
Membranous Nephropathy

scholarly journals Inguinal ulcerated sebaceous carcinoma: an unusual presentation

2013 ◽  
Vol 88 (6 suppl 1) ◽  
pp. 48-51 ◽  
Author(s):  
David Oschilewski Lucares ◽  
Fred Bernardes Filho ◽  
Hernando Vega ◽  
Bernard Kawa Kac ◽  
Maria Rita Pereira ◽  
...  
Keyword(s):  
Male Patient ◽  
Clinical Manifestation ◽  
Clinical Presentation ◽  
Sebaceous Carcinoma ◽  
The Body ◽  
Skin Tumor ◽  
Diagnostic Challenge ◽  
Elderly Male ◽  
Genital Ulcers ◽  
Clinical Description

Sebaceous carcinoma is a rare and aggressive skin tumor. It can be located in any area of the body, the most commonly involved area being the periorbital region. It does not entail a typical clinical presentation, which explains the often late diagnosis. The aim of this report is to outline the rarity of the disease and its atypical clinical description, since to this day, inguinal ulcers with clinical manifestation have not been reported. We present and discuss a case of sebaceous carcinoma with an unusual clinical presentation, in an elderly male patient. The precise approach to genital ulcers, as shown in this case, is a diagnostic challenge that requires a great deal of effort on the part of the clinician.

scholarly journals Factors affecting the course of body and kidney growth in infants with urolithiasis: A critical long-term evaluation

2016 ◽  
Vol 88 (4) ◽  
pp. 249 ◽  
Author(s):  
Kemal Sarica ◽  
Fatma Narter ◽  
Kubilay Sabuncu ◽  
Ahmet Akca ◽  
Utku Can ◽  
...  
Keyword(s):  
Breast Feeding ◽  
Clinical Course ◽  
Stone Disease ◽  
The Body ◽  
Renal Growth ◽  
Related Factors ◽  
Growth Status ◽  
Long Term Follow Up

Objective: To investigate the possible effects of dietary, patient and stone related factors on the clinical course of the stone disease as well as the body and renal growth status of the infants. Patients and Methods: A total of 50 children with an history of stone disease during infancy period were studied. Patient (anatomical abnormalities, urinary tract infection - UTI, associated morbidities), stone (obstruction, UTI and required interventions) and lastly dietary (duration of sole breast feeding, formula feeding) related factors which may affect the clinical course of the disease were all evaluated for their effects on the body and renal growth during long-term follow-up. Results: Mean age of the children was 2.40 ± 2.65 years. Our findings demonstrated that infants receiving longer period of breast feeding without formula addition seemed to have a higher rate of normal growth percentile values when compared with the other children. Again, higher frequency of UTI and stone attacks affected the growth status of the infants in a remarkable manner than the other cases. Our findings also demonstrated that thorough a close follow-up and appropriately taken measures; the possible growth retardation as well as renal growth problems could be avoided in children beginning to suffer from stone disease during infancy period. Conclusions: Duration of breast feeding, frequency of UTI, number of stone attacks and stone removal procedures are crucial factors for the clinical course of stone disease in infants that may affect the body as well as kidney growth during long-term follow-up.

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