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1.
Medicine (Baltimore) ; 99(5): e18857, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-32000387

RESUMEN

RATIONALE: IgG4-related disease (IgG4-RD) is a slowly progressing inflammatory disease that can involve multiple organ systems. There is considerable overlap between IgG4-RDs and anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV). Herein, we present an unusual case of IgG4-associated tubulointerstitial nephritis (IgG4-TIN) and ANCA-associated glomerulonephritis (ANCA-GN) co-occurring with C3 glomerulonephritis (C3GN). PATIENT CONCERNS: A 72-year-old male was admitted to hospital because of fever and fatigue. He was diagnosed with elevated serum creatinine and IgG4 levels, and was positive for ANCA. DIAGNOSIS: Initially, the pathology supported a diagnosis of IgG4-TIN and ANCA-GN; however, further examination revealed he also had C3GN. INTERVENTIONS: The patient was treated with methylprednisolone and cyclophosphamide and received regular follow-up care. OUTCOMES: After treatment, the patient no longer exhibited fever or fatigue and had no complications. The seven-month follow-up showed downward trends in IgG4 and MPO-ANCA levels and stable 24-hour urine protein, serum creatinine levels. LESSONS: Anti-neutrophil cytoplasmic antibody-associated glomerulonephritis and IgG4-associated tubulointerstitial nephritis with C3glomerulonephritis rarely occur simultaneously. Laboratory analysis and pathology are both needed to ensure diagnostic accuracy. However, in this case, the three diseases overlapped to such a large extent that achieving a definitive diagnosis was particularly challenging. Timely and accurate diagnosis is crucial for selecting the best treatment course and optimizing patient outcome.


Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Glomerulonefritis/diagnóstico , Nefritis Intersticial/diagnóstico , Anciano , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/tratamiento farmacológico , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/inmunología , Anticuerpos Anticitoplasma de Neutrófilos/inmunología , Complemento C3/inmunología , Ciclofosfamida/uso terapéutico , Glomerulonefritis/tratamiento farmacológico , Glomerulonefritis/inmunología , Glucocorticoides/uso terapéutico , Humanos , Inmunoglobulina G/inmunología , Inmunosupresores/uso terapéutico , Masculino , Metilprednisolona/uso terapéutico , Nefritis Intersticial/tratamiento farmacológico , Nefritis Intersticial/inmunología
2.
Medicine (Baltimore) ; 99(7): e19179, 2020 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-32049851

RESUMEN

RATIONALE: Polyglandular autoimmune syndromes (PAS) are a heterogeneous group of rare diseases characterized by the association of at least 2 organ-specific autoimmune disorders, concerning both the endocrine and nonendocrine organs. Type III is defined as the combination of autoimmune thyroid disease and other autoimmune conditions (other than Addison disease), and is divided into 4 subtypes. We describe a patient with Hashimoto thyroiditis, adult-onset Still disease, alopecia, vasculitis, antineutrophil cytoplasmic antibody (ANCA)-mediated crescentic glomerulonephritis, and hyperparathyroidism. Co-occurrence of these 5 diseases allowed us to diagnose PAS type IIIc. The rare combination of these different diseases has not been reported before. PATIENT CONCERNS: A 51-year-old woman was admitted in April, 2019 after the complaint of an enlarged thyroid. She was diagnosed with Hashimoto thyroiditis at the age of 36. At age 40, she was diagnosed with an adult-onset Still disease. Three months before admission, she experienced renal insufficiency. After admission, she was diagnosed with hyperparathyroidism. DIAGNOSIS: Renal biopsy revealed renal vasculitis and crescentic nephritis. Antineutrophil cytoplasmic autoantibody showed that human perinuclear ANCA and myeloperoxidase ANCA were positive. Therefore, the patient was diagnosed with vasculitis and ANCA-mediated crescentic glomerulonephritis. After admission, parathyroid single-photon emission computed tomography/computed tomography fusion image demonstrated the presence of hyperparathyroidism. INTERVENTIONS: The patient was treated with high-dose methylprednisolone pulse therapy (0.1 g/d) for vasculitis and ANCA-mediated crescentic glomerulonephritis, calcium and vitamin D3 (600 mg/d elemental calcium [calcium carbonate] and 2.5 µg/d active vitamin D3) for hyperparathyroidism, and levothyroxine sodium (50 ug/d) for Hashimoto thyroiditis. OUTCOMES: Up to now, serum thyroid-stimulating hormone, total triiodothyronine, total thyroxine, free triiodothyronine, and free thyroxine were within the normal ranges. Patient's renal function did not deteriorate. LESSONS: We report a patient with Hashimoto thyroiditis, adult-onset Still disease, alopecia, vasculitis, ANCA-mediated crescentic glomerulonephritis, and hyperparathyroidism, which is a very rare combination. We present this case as evidence for the coexistence of several different immune-mediated diseases in the clinical context of a PAS IIIc.


Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Glomerulonefritis/diagnóstico , Enfermedad de Hashimoto/diagnóstico por imagen , Poliendocrinopatías Autoinmunes/diagnóstico , Femenino , Glomerulonefritis/inmunología , Enfermedad de Hashimoto/complicaciones , Humanos , Persona de Mediana Edad , Poliendocrinopatías Autoinmunes/complicaciones
3.
Mymensingh Med J ; 29(1): 5-15, 2020 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-31915329

RESUMEN

Acute post-streptococcal glomerulonephritis (APSGN) is the commonest cause of acute glomerulonephritis (AGN), which usually present with gross hematuria, mild edema, oliguria, hypertension and varying degree of renal insufficiency. It is more common among the population of school going age where poverty, overcrowding and poor hygienic conditions are prevailing. This cross sectional observational study was aimed to know the socio-demographic variables, clinical profile and immediate outcome of AGN in hospitalized children and was conducted in the Pediatric department of Mymensingh Medical College Hospital (MMCH), Mymensingh, Bangladesh from November 2014 to April 2015. A detailed history was taken from the parents in each case with a written questionnaire. A written consent was also taken from the guardian of the including patients and also permission was taken from the ethical committee of MMCH. Thorough clinical examination and available relevant investigations were done in all patients. Progresses of the patient were monitored by daily clinical examinations and also by investigations. Data were analyzed by statistical package for social science (SPSS) windows version 18. Results were verified by doing standard test for significance. Among total 60 cases male was 58.3% & female was 41.7%. The common age group of presentation was between 7-12 years (73%), peak age of incidence was 7-9 years. Most of them came from low socioeconomic status (83.3%), 63.3% from rural area with average 5-6 member's family size. Most of the parents were illiterate. History of (H/O) skin infection was present in 35(58.3%) patients, 15(25%) had H/O sore throat, 15% did not give any H/O infection before presentation. Average duration of gap between infection and appearance of clinical feature was 7-14 days in 73.40%and 15-21 days was in 45.7% in case of sore throat & skin infection respectively. Almost all (95%) patients presented with puffiness of face, others presented with scanty micturition, gross hematuria, respiratory distress, fever, convulsion and altered sensorium. Edema (75%), hypertension (88.3%), pallor (38%), tachypnea (25%), tachycardia (26.7%) were the important clinical findings. Microscopic hematuria was present among 96.66% patients; low complement level was found in 85% cases. There is significant association between low socioeconomic statuses with more hospital stay. Only one patient died due to heart failure and 98.3% patient had complete recovery. Results of this study conclude that most of the patients came from rural illiterate family with low socioeconomic background. Skin infection is the commonest cause of acute glomerulonephritis. Edema, scanty micturation, hematuria and hypertension are the common mode of presentation. Heart failure and hypertensive encephalopathy are the common complication of AGN. Immediate prognosis of AGN was excellent.


Asunto(s)
Glomerulonefritis/diagnóstico , Tiempo de Internación/estadística & datos numéricos , Enfermedad Aguda , Bangladesh/epidemiología , Niño , Estudios Transversales , Femenino , Glomerulonefritis/epidemiología , Humanos , Incidencia , Masculino , Pediatría , Población Rural , Factores Socioeconómicos
6.
Medicine (Baltimore) ; 98(41): e17566, 2019 Oct.
Artículo en Inglés | MEDLINE | ID: mdl-31593141

RESUMEN

RATIONALE: Coexistence of Fabry disease and IgM nephropathy is rare. The varying severity and unapparent clinical manifestation of Fabry disease makes it difficult to recognize when coexisting with another more prevalent cause of nephropathy requiring electron microscopy and genetic testing to confirm their coexistence. PATIENT CONCERNS: A 54-year-old female presented with proteinuria without any clinical signs or family history of Fabry disease. DIAGNOSES: Immunostaining of the renal biopsy identified mesangial IgM deposition diagnosing it as IgM nephropathy. The light microscopy indicated prominent vacuolization of podocytes. Further examination of toluidine blue stained semi-thin sections and electron microscopy revealed blue bodies and myelin bodies in the cytoplasm of podocytes, respectively. Mutation analysis detected missense mutation establishing the diagnosis of coexisting Fabry disease. INTERVENTIONS: The patient was treated with angiotensin-converting enzyme inhibitors. Enzyme replacement therapy was not administered due to financial constraints. OUTCOMES: After 2 months of treatment the patient demonstrated urine protein to creatinine ratio of 0.21 g/g. LESSONS: Identifying coexistence of Fabry disease with other nephropathy requires meticulous pathologic investigations including electron microscopy especially when Fabry disease presents with atypical phenotype.


Asunto(s)
Enfermedad de Fabry/complicaciones , Glomerulonefritis/diagnóstico , Inmunoglobulina M/inmunología , Podocitos/ultraestructura , Inhibidores de la Enzima Convertidora de Angiotensina/uso terapéutico , Terapia de Reemplazo Enzimático/economía , Enfermedad de Fabry/diagnóstico , Enfermedad de Fabry/genética , Enfermedad de Fabry/patología , Femenino , Glomerulonefritis/complicaciones , Glomerulonefritis/tratamiento farmacológico , Glomerulonefritis/patología , Humanos , Microscopía Electrónica/métodos , Persona de Mediana Edad , Mutación Missense/genética , Podocitos/patología , Proteinuria/diagnóstico , Proteinuria/etiología , Resultado del Tratamiento
7.
Int J Mol Sci ; 20(20)2019 Oct 11.
Artículo en Inglés | MEDLINE | ID: mdl-31614644

RESUMEN

Glomerulonephritis (GN) represents a collection of kidney diseases characterized by inflammation within the renal glomeruli and small blood vessels. The lesions that occur in other nephron structures mainly result from the harmful effects of proteinuria. In recent years, an emphasis has been placed on gaining a better insight into the pathogenesis and pathophysiology of GN in order to facilitate diagnoses and provide efficient and targeted treatments of the disease. Owing to the advanced molecular and genetic diagnostic techniques available today, researchers have been able to elucidate that most cases of GN are determined by genetic risk factors and are associated with the abnormal functioning of the immune system (the immunologically mediated forms of GN). MicroRNAs (miRNAs) are a group of single-stranded, non-coding molecules, approximately 20 nucleotides in length, that act as regulatory factors in the post-transcriptional processes capable of regulating the expression of multiple genes. In this paper we present the available research aiming to determine effects of miRNAs on the development and progression of GN and discuss the potential role of miRNAs as new diagnostic markers and therapeutic targets.


Asunto(s)
Glomerulonefritis/diagnóstico , MicroARNs/genética , Biomarcadores de Tumor , Regulación de la Expresión Génica , Redes Reguladoras de Genes , Predisposición Genética a la Enfermedad , Glomerulonefritis/tratamiento farmacológico , Glomerulonefritis/genética , Humanos
8.
Zhonghua Nei Ke Za Zhi ; 58(10): 758-762, 2019 Oct 01.
Artículo en Chino | MEDLINE | ID: mdl-31594174

RESUMEN

Objective: To investigate the clinical characteristics of polyarteritis nodosa (PAN) patients with renal involvement. Methods: PAN patients admitted to the department of rheumatology, department of pediatrics, department of nephrology, general internal medicine department and department of vascular surgery at Peking Union Medical College Hospital from June 2012 to August 2018 were enrolled in this study and were divided into two groups according to renal involvement or not. The clinical characteristics were analyzed. Results: A total of 94 PAN patients were finally enrolled and 57 (60.64%) presented kidney manifestation. The mean age of onset was (37.76±17.40) years old and the interval from onset to diagnosis was 10 (0 to 240) months. Forty patients were misdiagnosed once or more times. In patients with renal involvement, 9 cases suffered from renal ischemia or infarction, 31 with microscopic haematuria, 26 with proteinuria, renal artery or its branch involved in 17 cases, renal vein thrombosis in 1 case, 4 cases with pyeloureterectasis, one case with renal fascia thickening, 33 cases with impaired renal function (serum creatinine>84 µmol/L) including creatinine>140 µmol/L in 10 patients. Renal artery branch stenosis was the most common presentation [9 cases (52.94%)] of renal vascular involvement, other abnormalities including nodular dilatation [4 cases (23.53%)], occlusion [3 cases (17.65%)]. There were significant differences (P<0.05) in the PAN patients with and without renal involvement in the following: age of onset [(33.72±16.13) years vs. (43.97±17.66) years, t(2)=2.901, P=0.005], weight loss(≥4kg since PAN onset) [25(43.86%) vs. 7(18.92%), χ(2)=6.216, P=0.013], elevation of diastolic blood pressure [22(38.60%) vs. 7(18.92%), χ(2)=4.072, P=0.044], acromegaly gangrene [18(31.58%) vs. 21(56.76%), χ(2)=5.859, P=0.015], and gastrointestinal artery involvement [20(35.09%) vs. 6(1.22%), χ(2)=3.993, P=0.046]. Laboratory parameters and the application of glucocorticoid and cyclophosphamide therapies were similar in two groups (all P>0.05). Conclusion: Young PAN patients are more likely to be associated with renal involvement, especially gastrointestinal arteries.


Asunto(s)
Arteritis/diagnóstico , Enfermedades Renales/etiología , Riñón/fisiopatología , Poliarteritis Nudosa/diagnóstico , Adulto , Anciano , Ciclofosfamida/uso terapéutico , Enfermedades Gastrointestinales , Glomerulonefritis/diagnóstico , Glucocorticoides/uso terapéutico , Humanos , Infarto , Enfermedades Renales/fisiopatología , Persona de Mediana Edad , Poliarteritis Nudosa/complicaciones , Poliarteritis Nudosa/tratamiento farmacológico , Adulto Joven
9.
J Ayub Med Coll Abbottabad ; 31(3): 308-313, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31535496

RESUMEN

BACKGROUND: C1q nephropathy (C1qN) is a rare glomerulopathy, with a very low prevalence world wide varying from 0.2 to 2.5%. Even though more than three decades have passed since this entity was first explained, still, it remains a dilemma for many due to the rarity of this lesion. This study was carried out principally to determine the clinical presentation, morphologic features and distribution of C1qN in our region based on renal biopsies studied by light microscopy (LM), and immunofluorescence (IF) so that this entity is better understood both by nephrologists and pathologists as no such study has ever been conducted in Pakistan to our knowledge. METHODS: It was a cross-sectional study carried out from 1st January 2012 to 30th December 2016 in Histopathology department, Shifa International Hospital. All cases diagnosed as C1q nephropathy were retrieved from the hospital's computerized database. Their clinical profiles, morphology and immunohistochemical profiles were studied.. RESULTS: Over this period a total of 31 cases were diagnosed with C1qN. Mean age of the patients was 32.09±18.66 years. The most common clinical presentation was nephrotic syndrome seen in 22 (71%) patients. The most frequent morphological pattern seen was minimal change disease (MCD) in 13 (41.9%) cases. All cases showed dominant 22 (71%) or codominant 9 (42.9%) mesangial±membranous C1q deposition. No correlation was found (p-value >0.05) between morphological pattern and clinical presentation of the disease or immunofluorescence findings. CONCLUSIONS: C1qN is a rare entity which is primarily diagnosed on the basis of immunofluorescence findings with a dominant or codominant fluorescent intensity for C1q. It is recommended that C1qN is sought for preferably with immunofluorescence staining of biopsies for immune reactants, especially for C1q. Studies from this part of the world are strongly recommended to predict clinical outcome and treatment options.


Asunto(s)
Glomerulonefritis , Enfermedades Raras , Adolescente , Adulto , Complemento C1q/análisis , Estudios Transversales , Mesangio Glomerular/patología , Glomerulonefritis/diagnóstico , Glomerulonefritis/epidemiología , Glomerulonefritis/fisiopatología , Humanos , Riñón/patología , Riñón/fisiopatología , Persona de Mediana Edad , Pakistán/epidemiología , Enfermedades Raras/diagnóstico , Enfermedades Raras/epidemiología , Enfermedades Raras/fisiopatología , Adulto Joven
10.
Internist (Berl) ; 60(11): 1201-1208, 2019 Nov.
Artículo en Alemán | MEDLINE | ID: mdl-31511906

RESUMEN

This article reports about a 73-year-old woman of Bosnian descent who presented with acute renal failure. A renal biopsy was diagnostic for a postinfect necrotizing and extracapillary proliferative glomerulonephritis. The patient reported a febrile infection fever 2 weeks previously. The diagnostics did not reveal any indications of an ongoing infection. The glomerulonephritis responded to treatment with systemic steroids. The patient was readmitted to hospital 6 weeeks later in a severely ill condition. A gastric biopsy revealed a Strongyloides stercoralis infestation. Due to the systemic steroid therapy the patient had developed a so-called hyperinfection syndrome and died despite treatment on the intensive care unit. This case illustrates the need for awareness of this rare parasitosis, particularly in patients from endemic areas. A likely causal relationship with the glomerulonephritis is discussed and an overview of the diagnostics, course of the disease and treatment of this parasitosis is given.


Asunto(s)
Lesión Renal Aguda/etiología , Glomerulonefritis/tratamiento farmacológico , Prednisolona/efectos adversos , Esteroides/efectos adversos , Strongyloides stercoralis/aislamiento & purificación , Estrongiloidiasis/diagnóstico , Anciano , Animales , Antiparasitarios/uso terapéutico , Resultado Fatal , Femenino , Glomerulonefritis/diagnóstico , Humanos , Ivermectina/uso terapéutico , Prednisolona/uso terapéutico , Esteroides/uso terapéutico , Estómago/microbiología , Estómago/patología , Estrongiloidiasis/complicaciones , Estrongiloidiasis/tratamiento farmacológico
11.
BMJ Case Rep ; 12(8)2019 Aug 01.
Artículo en Inglés | MEDLINE | ID: mdl-31371277

RESUMEN

A 70-year-old man presented with 1 month of haematuria and mild right-sided flank pain with no other symptoms. Diagnostic workup included serum studies which showed the presence of antimyeloperoxidase antibodies, a kidney biopsy which demonstrated necrotising crescentic glomerulonephritis with linear immunofluorescence of the basement membrane, and electron microscopy which exhibited thickening of the glomerular basement membrane. Incidentally, the patient was discovered to have a latent hepatitis B infection, which complicated immunosuppressive therapy. He was treated with a course of plasmapheresis and methylprednisolone, followed by entecavir for hepatitis B prophylaxis, and finally by rituximab. This case of glomerulonephritis was notable for its resemblance to the better known Goodpasture's disease. Typically, Goodpasture's syndrome exists on a spectrum from seronegative disease to double-positive disease that presents with both anti-glomerular basement membrane (anti-GBM) and cytoplasmic-antineutrophil cytoplasmic antibodies/antiproteinase 3 antibodies (c-ANCA/anti-PR3). However, this patient's glomerulonephritis was unique because he presented negative for anti-GBM antibodies and positive for perinuclear-antineutrophil cytoplasmic antibodies/antimyeloperoxidase antibodies (p-ANCA/anti-MPO).


Asunto(s)
Glomerulonefritis/diagnóstico , Enfermedades Pulmonares Intersticiales/diagnóstico , Peroxidasa/inmunología , Anciano , Anticuerpos Anticitoplasma de Neutrófilos/metabolismo , Autoanticuerpos/metabolismo , Diagnóstico Diferencial , Glomerulonefritis/inmunología , Glomerulonefritis/terapia , Guanina/análogos & derivados , Guanina/uso terapéutico , Humanos , Enfermedades Pulmonares Intersticiales/inmunología , Enfermedades Pulmonares Intersticiales/terapia , Masculino , Metilprednisolona/uso terapéutico , Microscopía Electrónica , Plasmaféresis , Rituximab/uso terapéutico , Resultado del Tratamiento
12.
BMJ Case Rep ; 12(8)2019 Aug 20.
Artículo en Inglés | MEDLINE | ID: mdl-31434674

RESUMEN

A 71-year-old man was admitted to our hospital with right lower abdominal pain. Blood analysis indicated severe inflammation, and abdominal CT revealed a pancreatic head tumour and multiple lung nodules. The level of a tumour marker was high. Pancreatic cancer with multiple lung metastases was suspected; however, because the mass was not detected via endoscopic ultrasonography, it was not biopsied. The serum creatinine level increased rapidly with a urine disorder, and myeloperoxidase-antineutrophil cytoplasmic antibody staining was positive. Severe rapidly progressive glomerulonephritis (RPGN) and microscopic polyangiitis were diagnosed, and high-dose glucocorticoid treatment was started. The patient's high fever returned to normal, and the serum creatinine level declined. Because the RPGN was severe, cyclophosphamide was administrated, and the glucocorticoid was tapered. The pancreatic tumour regressed, the lung nodules disappeared, and the tumour marker level normalised during the treatment. Renal function improved, and maintenance haemodialysis was avoided.


Asunto(s)
Glomerulonefritis/diagnóstico , Glucocorticoides/uso terapéutico , Neoplasias Pulmonares/diagnóstico , Poliangitis Microscópica/diagnóstico , Páncreas/patología , Neoplasias Pancreáticas/diagnóstico , Dolor Abdominal/diagnóstico por imagen , Anciano , Diagnóstico Diferencial , Glomerulonefritis/tratamiento farmacológico , Glomerulonefritis/fisiopatología , Humanos , Masculino , Poliangitis Microscópica/tratamiento farmacológico , Poliangitis Microscópica/fisiopatología , Páncreas/diagnóstico por imagen , Radiografía Abdominal , Resultado del Tratamiento
13.
Saudi J Kidney Dis Transpl ; 30(4): 803-811, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31464236

RESUMEN

Rheumatoid arthritis (RA) is accompanied by a variety of nephropathies. It is often difficult to distinguish between disease-associated and drug-associated renal diseases. Three hundred and seventy-six RA patients with renal involvement were included in our study; they were subjected to full history and clinical examination, kidney function, 24-h urinary protein, and kidney biopsy. All our patients were on methotrexate, low dose steroids, and nonsteroidal anti-inflammatory drugs, in addition to the previous medications. About 79.3%, 20.7%, 6.9%, and 5.9% of our patients were on leflunomide, hydroxychloroquine, etanercept, and infliximab, respectively. Renal presentation was in the form of nephrotic syndrome (33.5%), persistent subnephrotic proteinuria (12.2%), persistent proteinuria and recurrent hematuria (13.3%), acute nephritis (23.9), recurrent hematuria (7.4%), and creatinine >1.5 mg/dL (10.6%). Renal biopsies were glomerular amyloidosis (28.1%), mesangioproliferative (19.1%), membranous (6.1%), crescent (16.8%), focal segmental glomerulosclerosis (18.6%), and minimal changes (11.7%). There was a statistically significant difference in the incidence of membranous nephritis between patients who took leflunomide, and hydroxychloroquine and those did not. Etanercept in our study seems not to be related to any form of renal involvement, while infliximab is related to focal segmental sclerosis and amyloidosis of tubulointerstitial type. Kidney involvement in RA is not a rare complication. Any type of histopathological changes can be present, with amyloidosis on top of the list. Hydroxychloroquine and leflunomide are accused in membranous nephropathy. Infliximab is associated with focal segmental sclerosis and amyloidosis of tubulointerstial type, and etanercept appear to be safe as regards kidney affection.


Asunto(s)
Antiinflamatorios no Esteroideos/efectos adversos , Artritis Reumatoide/tratamiento farmacológico , Glomerulonefritis/inducido químicamente , Hospitales Universitarios , Inmunosupresores/efectos adversos , Riñón/efectos de los fármacos , Metotrexato/efectos adversos , Esteroides/efectos adversos , Adulto , Artritis Reumatoide/diagnóstico , Artritis Reumatoide/epidemiología , Egipto/epidemiología , Femenino , Glomerulonefritis/diagnóstico , Glomerulonefritis/epidemiología , Glomerulonefritis/fisiopatología , Humanos , Incidencia , Riñón/patología , Riñón/fisiopatología , Masculino , Persona de Mediana Edad , Medición de Riesgo , Factores de Riesgo
14.
J Korean Med Sci ; 34(24): e173, 2019 Jun 24.
Artículo en Inglés | MEDLINE | ID: mdl-31222986

RESUMEN

D-penicillamine has been reported to cause antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis presenting as rapidly progressive glomerulonephritis or pulmonary-renal syndrome mostly in adults. We report a pediatric case of D-penicillamine induced ANCA-associated vasculitis that manifests as a pulmonary-renal syndrome with a mild renal manifestation. A 13-year-old girl who has been taking D-penicillamine for five years under the diagnosis of Wilson disease visited the emergency room because of hemoptysis and dyspnea. She had diffuse pulmonary hemorrhage, microscopic hematuria, and proteinuria. Myeloperoxidase ANCA was positive, and a renal biopsy revealed pauci-immune crescentic glomerulonephritis. Under the diagnosis of D-penicillamine-induced ANCA-associated vasculitis, D-penicillamine was switched to trientine, and the patient was treated with plasmapheresis, glucocorticoid, cyclophosphamide, and mycophenolate mofetil. Pulmonary hemorrhage improved rapidly followed by the disappearance of the hematuria and proteinuria five months later.


Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Glomerulonefritis/diagnóstico , Hemorragia/diagnóstico , Enfermedades Pulmonares/diagnóstico , Penicilamina/efectos adversos , Adolescente , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/etiología , Femenino , Degeneración Hepatolenticular/tratamiento farmacológico , Humanos , Riñón/patología , Penicilamina/uso terapéutico , Tomografía Computarizada por Rayos X
15.
Klin Lab Diagn ; 64(5): 287-293, 2019.
Artículo en Ruso | MEDLINE | ID: mdl-31185152

RESUMEN

The paper presents data on the study of the content of cytokines (IL-1ß, RAIL-1ß, IL-2, IL-4, IL-10, IL-17A, TNF-, IFN-γ) in the morning urine using enzyme immunoassay in healthy individuals (n = 20) and in patients with acute glomerulonephritis (n = 93). The determination of cytokine levels in patients was carried out in the debut of the disease and 12 months after the onset of the disease. The obtained indicators of cytokine content in the urine are presented as absolute values in pg/ml and creatinine-normalized values calculated by the formula: cytokine level (pg/ml) / urine creatinine (µmol/ml). The study was made of changes in the content of cytokines in the urine of patients with glomerulonephritis with respect to a group of healthy individuals, as well as the dynamics of the content of cytokines in the urine during the 12-month observation period. The results of the study showed that the absolute values of cytokines in urine can distort the true picture of the cytokine profile of urine in renal pathology. Normalized values of the predominant number of pro- and anti-inflammatory cytokines (IL-1ß, IL-2, IL-8, IL-10, IL-17A and TNF-α) in patients with glomerulonephritis were significantly higher than the corresponding indicators of healthy individuals. The normalized values of cytokines were shown to be as more sensitive indicators than absolute values in the course of analyzing differences in the cytokine profile in patients with glomerulonephritis, depending on chronic and acute course of the disease. These indicators influenced the outcome of glomerulonephritis, assessed, as a rule, 12 months after the onset of the disease. Thus, the low levels of IL-1ß, IL-8 and IL-17А detected in the debut of the disease in combination with the high level of RAIL-1ß determined the chronization of glomerulonephritis. So, the creatinine-normalized cytokine levels in the urine expand the possibilities of using the evaluation of the cytokine profile of urine to establish changes in the cytokine content in the urine in renal pathology and predict the chronization of glomerulonephritis.


Asunto(s)
Citocinas/orina , Glomerulonefritis/diagnóstico , Riñón/fisiopatología , Creatinina , Glomerulonefritis/orina , Voluntarios Sanos , Humanos , Técnicas para Inmunoenzimas
16.
J Med Life ; 12(1): 49-55, 2019.
Artículo en Inglés | MEDLINE | ID: mdl-31123525

RESUMEN

Anti-Neutrophil Cytoplasmic Antibody (ANCA)-negative Rapidly Progressive Glomerulonephritis (RPGN) is a severe form of autoimmune renal injury with a bleak prognosis. A 60-year-old Indian woman was treated with classical homeopathy for ANCA-negative RPGN, and after one year of treatment, serum creatinine and other parameters indicating renal injury dropped steadily despite the withdrawal of immunosuppressive drugs; renal dialysis, which was conducted twice a week initially, was made rarer and stopped after one year. Classical homeopathy may be considered a potential therapeutic modality in severe pathologies. Controlled studies are required to establish further the extent to which classical homeopathy may relieve patients from procedures such as dialysis that cause considerable physical and economic discomfort.


Asunto(s)
Anticuerpos Anticitoplasma de Neutrófilos/metabolismo , Progresión de la Enfermedad , Glomerulonefritis/inmunología , Glomerulonefritis/patología , Homeopatía , Medicina de Precisión , Insuficiencia Renal/complicaciones , Femenino , Glomerulonefritis/complicaciones , Glomerulonefritis/diagnóstico , Humanos , Persona de Mediana Edad , Diálisis Renal
18.
Int J Mol Sci ; 20(9)2019 May 05.
Artículo en Inglés | MEDLINE | ID: mdl-31060307

RESUMEN

Glomerular hematuria is a cardinal symptom of renal disease. Glomerular hematuria may be classified as microhematuria or macrohematuria according to the number of red blood cells in urine. Recent evidence suggests a pathological role of persistent glomerular microhematuria in the progression of renal disease. Moreover, gross hematuria, or macrohematuria, promotes acute kidney injury (AKI), with subsequent impairment of renal function in a high proportion of patients. In this pathological context, hemoglobin, heme, or iron released from red blood cells in the urinary space may cause direct tubular cell injury, oxidative stress, pro-inflammatory cytokine production, and further monocyte/macrophage recruitment. The aim of this manuscript is to review the role of glomerular hematuria in kidney injury, the role of inflammation as cause and consequence of glomerular hematuria, and to discuss novel therapies to combat hematuria.


Asunto(s)
Glomerulonefritis/complicaciones , Glomerulonefritis/fisiopatología , Hematuria/etiología , Hematuria/orina , Glomérulos Renales/fisiopatología , Lesión Renal Aguda/etiología , Lesión Renal Aguda/fisiopatología , Animales , Biomarcadores , Glomerulonefritis/diagnóstico , Glomerulonefritis/etiología , Hematuria/diagnóstico , Humanos , Riñón/patología , Riñón/fisiopatología , Estrés Oxidativo , Fenotipo , Pronóstico
19.
Med. clín (Ed. impr.) ; 152(9): 361-367, mayo 2019. ilus, graf, tab
Artículo en Español | IBECS | ID: ibc-183662

RESUMEN

La glomerulopatía colapsante (GC) es una entidad poco frecuente como enfermedad glomerular. A pesar de ser considerada como una variante de la glomeruloesclerosis segmentaria y focal, lo cierto es que las lesiones podocitarias presentan rasgos diferenciales respecto a la glomeruloesclerosis segmentaria y focal típica, aspecto que le ha otorgado su clasificación como un tipo de podocitopatía. En la GC la lesión podocitaria se caracteriza típicamente por una desdiferenciación fenotípica, reflejada por la pérdida de la expresión de marcadores podocitarios maduros. La GC puede ser una enfermedad primaria o puede estar asociada a diversos factores causales que desarrollan una entidad histopatológica común. La expresividad clínica de la GC a menudo se caracteriza por la presencia de un síndrome nefrótico y un rápido deterioro de la funcional renal superior a otras variantes de la glomeruloesclerosis segmentaria y focal. El pronóstico de los pacientes afectados por una GC es una rápida progresión hacia la insuficiencia renal terminal con mala respuesta al tratamiento


Collapsing glomerulopathy (CG) is a rare entity as a glomerular disease. Although it has been considered as a variant of focal segmental glomerulosclerosis, the fact is that the podocyte lesions show different features with respect to the typical focal segmental glomerulosclerosis, an aspect that has been attributed to a type of podocytopathy. In CG, the podocyte lesion is typically characterised by a dysregulated podocyte phenotype, reflected by the loss of expression of mature podocyte markers. CG can be a primary disease or it can be associated with several causal factors that develop a common histopathological entity. The clinical expressiveness of CG is often characterised by the presence of a nephrotic syndrome and a rapid deterioration of the renal function than other variants of the focal segmental glomerulosclerosis. The prognosis of these patients is a rapid progression towards end-stage renal disease with poor response to treatment


Asunto(s)
Humanos , Glomerulonefritis/clasificación , Glomerulonefritis/diagnóstico , Glomerulonefritis/tratamiento farmacológico , Glomerulonefritis/etiología , Diagnóstico Diferencial , Pronóstico
20.
Ther Apher Dial ; 23(3): 253-260, 2019 Jun.
Artículo en Inglés | MEDLINE | ID: mdl-31033151

RESUMEN

Plasma exchange (PEX) can be an effective treatment in anti-neutrophil cytoplasmic antibody-associated vasculitis with severe renal damage; however, it is still controversial. Among cases of newly diagnosed AAV with rapidly progressive glomerulonephritis at our department from 2008 onward, 11 patients who received PEX (seven cases for severe renal damage [R-PEX] and four cases for lung hemorrhage [L-PEX]) were retrospectively analyzed. All cases of R-PEX were dependent on hemodialysis at the beginning of PEX and all received seven sessions of PEX (50 mL/kg or 1.3 plasma volume per exchange) within 2 weeks. All cases became dialysis-independent within 8 weeks, with 3- and 12-month cumulative renal survival rates of 100% and 80%, respectively. All cases of L-PEX retained their renal function. In rapidly developing, newly dialysis-dependent antibody-associated vasculitis with rapidly progressive glomerulonephritis patients with normal renal function before disease onset, standard PEX can be expected to induce sufficient renal recovery to establish dialysis independence.


Asunto(s)
Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/epidemiología , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/terapia , Glomerulonefritis/epidemiología , Glomerulonefritis/terapia , Intercambio Plasmático/métodos , Diálisis Renal/métodos , Centros Médicos Académicos , Anciano , Anciano de 80 o más Años , Vasculitis Asociada a Anticuerpos Citoplasmáticos Antineutrófilos/diagnóstico , Estudios de Cohortes , Comorbilidad , Progresión de la Enfermedad , Femenino , Estudios de Seguimiento , Glomerulonefritis/diagnóstico , Humanos , Japón , Pruebas de Función Renal , Masculino , Persona de Mediana Edad , Estudios Retrospectivos , Medición de Riesgo , Índice de Severidad de la Enfermedad , Tasa de Supervivencia , Resultado del Tratamiento
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