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1.
Article in Chinese | WPRIM | ID: wpr-970890

ABSTRACT

OBJECTIVE@#To assess the association of rs55829688 and rs75315904 polymorphisms of the lncRNA-GAS5 gene with susceptibility to systemic lupus erythematosus (SLE) in Guangxi population.@*METHODS@#Peripheral venous blood samples were collected from the SLE group and control group. Following extraction of genomic DNA, SNPscan and Sanger sequencing were carried out to determine the genotypes for the rs55829688 and rs75315904 loci of the lncRNA-GAS5 gene.@*RESULTS@#No difference was found between the two groups with regard to the genotypic frequencies for rs55829688 and rs75315904 (P > 0.05). However, the frequencies of C allele of rs55829688 between the two groups was significantly different (P < 0.05). In the SLE group, the frequencies of C allele and CT+CC genotype for rs55829688 among SLE patients with nephritis were significantly lower than those of SLE patients without nephritis (P < 0.05). In addition, haplotype analysis showed that the frequency of rs55829688 C/rs75315904 A allele in the SLE group was lower than that of the control group (P < 0.05).@*CONCLUSION@#In Guangxi population, the carrier status of rs55829688 C allele of the lncRNA-GAS5 gene may reduce the risk of SLE and its complicated nephritis, and the rs55829688 C/rs75315904 A haplotype may reduce the risk for SLE.


Subject(s)
Humans , Case-Control Studies , China/epidemiology , Gene Frequency , Genetic Predisposition to Disease , Genotype , Lupus Erythematosus, Systemic/genetics , Nephritis , Polymorphism, Single Nucleotide , RNA, Long Noncoding/genetics
2.
Article in Chinese | WPRIM | ID: wpr-1009829

ABSTRACT

OBJECTIVES@#To investigate the clinical characteristics, pathology, and prognosis of children with diffuse endocapillary proliferative Henoch-Schönlein purpura nephritis (DEP-HSPN).@*METHODS@#A retrospective analysis was performed on the clinical, pathological, and prognosis data of 44 children with DEP-HSPN and 765 children without DEP-HSPN. The children with DEP-HSPN were diagnosed by renal biopsy in Jiangxi Provincial Children's Hospital from January 2006 to December 2021.@*RESULTS@#Among the 809 children with purpura nephritis, 44 (5.4%) had DEP-HSPN, with a mean age of (8±3) years, and there were 29 boys (65.9%) and 15 girls (34.1%). Compared with the non-DEP-HSPN group, the DEP-HSPN group had a significantly shorter time from onset to renal biopsy and a significantly higher proportion of children with respiratory infection or gross hematuria, and most children had nephrotic syndrome. The DEP-HSPN group had significantly higher levels of 24-hour urinary protein, urinary protein grading, microscopic hematuria grading, serum creatinine, and blood urea nitrogen and significantly lower levels of serum albumin and complement C3 (P<0.05). The DEP-HSPN group had a higher pathological grading, with predominant deposition of IgA in the mesangial area and capillary loops, and higher activity scores in the modified semi-quantitative scoring system (P<0.05). The Kaplan-Meier survival analysis showed that there was no significant difference in the renal complete remission rate between the two groups (P>0.05).@*CONCLUSIONS@#Children with DEP-HSPN have a rapid onset, severe clinical manifestations and pathological grading, and high activity scores in the modified semi-quantitative scoring system. However, most of the children with DEP-HSPN have a good prognosis, with a comparable renal complete remission rate to the children without DEP-HSPN.


Subject(s)
Male , Female , Humans , Child , Child, Preschool , Hematuria , IgA Vasculitis , Retrospective Studies , Prognosis , Nephritis
3.
Article in Chinese | WPRIM | ID: wpr-1009856

ABSTRACT

OBJECTIVES@#To investigate the difference in the therapeutic effect of mycophenolate mofetil (MMF) or cyclophosphamide (CTX) in children with Henoch-Schönlein purpura nephritis (HSPN) of different age groups.@*METHODS@#A retrospective analysis was conducted on the clinical data of 135 children with HSPN who were treated with MMF or CTX in the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics, from October 2018 to October 2020. According to the immunosuppressant used, they were divided into two groups: MMF group and CTX group, and according to the age, each group was further divided into two subgroups: ≤12 years and >12 years, producing four groups, i.e, the ≤12 years MMF subgroup (n=30), the >12 years MMF subgroup (n=15), the ≤12 years CTX subgroup (n=71), and the >12 years CTX subgroup (n=19). All children were followed up for at least 12 months, and the above groups were compared in terms of clinical outcomes and the incidence rate of adverse reactions.@*RESULTS@#There was no significant difference in the complete response rate between the MMF group and the CTX group after 3, 6, and 12 months of treatment (P>0.05). There were no significant difference in the complete response rate and the incidence rate of adverse reactions between the >12 years MMF subgroup and the ≤12 years MMF subgroup at 3, 6, and 12 months of treatment (P>0.05). The >12 years CTX subgroup had a significantly lower complete response rate than the ≤12 years CTX subgroup at 6 and 12 months of treatment (P<0.05). The >12 years CTX subgroup had a significantly higher incidence rate of adverse reactions than the >12 years MMF subgroup (P<0.05).@*CONCLUSIONS@#The efficacy and adverse reactions of MMF are not associated with age, but the efficacy of CTX is affected by age, with a higher incidence rate of adverse reactions. CTX should be selected with caution for children with HSPN aged >12 years.


Subject(s)
Child , Humans , Mycophenolic Acid/adverse effects , IgA Vasculitis/drug therapy , Retrospective Studies , Cyclophosphamide/adverse effects , Immunosuppressive Agents/adverse effects , Vasculitis/drug therapy , Nephritis/complications
4.
Medicina UPB ; 41(1): 80-84, mar. 2022.
Article in Spanish | LILACS, COLNAL | ID: biblio-1362715

ABSTRACT

El envenenamiento debido a picadora de escorpiones es reportado en diferentes países de Suramérica, en algunos, como México, es un problema de salud pública. En Colombia el accidente por escorpiones no es de reporte obligatorio, por lo que hay subregistro de casos. Los accidentes por este tipo de animales ponzoñosos se asocian a manifestaciones locales, y en casos de gravedad moderada a severa, a problemas cardiovasculares y neurológicos. Sin embargo, es importante reconocer manifestaciones que, aunque reportadas con menos frecuencia, implican gran morbilidad, como la pancreatitis y las alteraciones renales, entre las que está la nefritis. Se reporta el caso de un varón de 46 años, que presentó un accidente por Tytus, con nefritis intersticial. Este es el primer reporte de esta complicación en Colombia. Nuestro objetivo principal es resaltar la importancia de una pronta identificación de casos y un oportuno inicio del tratamiento por parte del personal de la salud, que permitan disminuir la morbilidad y mortalidad de estos pacientes.


Scorpion poisoning is reported in different countries in South America, especially in Mexico, where it has become a public health issue. In Colombia this type of accident is not mandatory to report, therefore, there is underreporting of cases. Accidents caused by these poisonous animals are mainly associated with local manifestations and, in cases of moderate to severe severity, to cardiovascular and neurological problems. Nevertheless, it is important to recognize other manifestations which, despite being reported less frequently, are associated with high morbidity such as pancreatitis and renal alterations, among which is nephritis. The case of a 46-year-old male patient is reported, who had an accident due to Tytus and presented interstitial nephritis. This is the first report of this complication in Colombia, and based on this, the main objective is to highlight the importance of prompt identification and treatment by health personnel, in order to reduce morbidity and mortality in these patients.


O envenenamento devido a picadora de escorpiões é reportado em diferentes países da América Latina, em alguns, como México, é um problema de saúde pública. Na Colômbiao acidente por escorpiões não é de reporte obrigatório, porque há sub-registro de casos. Os acidentes por este tipo de animais peçonhentos se associam a manifestações locais, e em casos de gravidade moderada a severa, a problemas cardiovasculares e neurológicos. Entretanto, é importante reconhecer manifestações que, embora reportadas com menos frequência, implicam grande morbilidade, como a pancreatite e as alterações renais, entre as que está a nefrite. Se reporta o caso de um homem de 46 anos, que apresentou um acidente por Tytus, com nefrite intersticial. Este é o primeiro reporte desta complicação na Colômbia. Nosso objetivo principal é ressaltar a importância de uma breve identificação de casos e um oportuno início do tratamento por parte do pessoal da saúde, que permitam diminuir a morbilidade e mortalidade destes pacientes.


Subject(s)
Humans , Animals , Scorpions , Acute Kidney Injury , Scorpion Stings , Animals, Poisonous , Nephritis
5.
Acta Medica Philippina ; : 72-76, 2022.
Article in English | WPRIM | ID: wpr-988169

ABSTRACT

@#We report two Filipino women with systemic lupus erythematosus (SLE) who developed diffuse alveolar hemorrhage (DAH), a rare, life-threatening complication associated with a high mortality rate. DAH should be suspected in patients with SLE presenting with new pulmonary infiltrates, a decline in hemoglobin, hemoptysis, dyspnea, and persistent desaturation. The first patient is 23 years old and was diagnosed with SLE 8 years ago; initially presenting with malar rash, oral ulcers, nephritis, and positive antinuclear antibodies (ANA). She had a poorly controlled disease and was admitted for facial and bipedal edema due to lupus nephritis. She was given 1 gram of methylprednisolone intravenously (IV) for three consecutive days. She then became tachypneic producing bloody secretions, with desaturation and sudden decline in hemoglobin. She was given cyclophosphamide 1 gram IV and referred for plasmapheresis but eventually succumbed. The second patient is 56 years old with generalized body weakness. Laboratory workup showed nephritis, anemia, ANA, low C3, and high anti-dsDNA titers. Pulse methylprednisolone 1000 mg was initiated. However, there was new-onset hemoptysis and desaturation and the patient was intubated. Bronchoscopy revealed diffuse bleeding on the right middle lobe and she eventually expired. Both patients with active SLE nephritis presented in this study died within days of DAH diagnosis. Hence, aside from early recognition to improve outcomes we should anticipate its possible occurrence in patients with high disease activity.


Subject(s)
Lupus Erythematosus, Systemic , Cyclophosphamide , Nephritis , Methylprednisolone
6.
J. bras. nefrol ; 43(2): 283-287, Apr.-June 2021. tab, graf
Article in English, Portuguese | LILACS | ID: biblio-1286937

ABSTRACT

ABSTRACT A wide spectrum of renal complications can occur with acute and chronic use of cocaine. Most cases are related to rhabdomyolysis, but other mechanisms are malignant hypertension, renal ischemia, and rapidly progressive glomerulonephritis (RPGN) associated-ANCA vasculitis. In recent years, the use of cocaine adulterated with levamisole has been associated with ANCA vasculitis and pauci-immune RPGN. RPGN is clinically manifested as a nephritic syndrome with a rapid and progressive decline in renal function, and its histopathological finding is the presence of crescents in more than 50% of the glomeruli. We report a case of a 38-year-old man chronic user of cocaine, alcohol, and cigarettes who had red urine, oliguria, swollen legs and eyelids, as well as the uremic symptoms anorexia, emesis, and mental confusion. He was admitted with acute kidney injury and performed six hemodialysis sessions during the first 16 days of hospitalization and then was transferred to a tertiary hospital for diagnostic investigation. Tests of ANF (antinuclear factor), ANCA, anti-DNA, serology for hepatitis B, C, and HIV virus were negative. A renal percutaneous biopsy revealed crescentic glomerulonephritis with mild tubular atrophy. The patient underwent pulse therapy with methylprednisolone (for 3 days) and cyclophosphamide. Then he maintained daily prednisone and monthly intravenous cyclophosphamide and evolved with progressive improvement of renal function.


RESUMO Um amplo espectro de complicações renais pode ocorrer com o uso agudo e crônico de cocaína. A maioria dos casos está relacionada à rabdomiólise, mas outros mecanismos são hipertensão maligna, isquemia renal e glomerulonefrite rapidamente progressiva (GNRP) associada à vasculite por ANCA. Nos últimos anos, o uso de cocaína adulterada com levamisol tem sido associado à vasculite por ANCA e GNRP pauci-imune. A GNRP se manifesta clinicamente como uma síndrome nefrítica, com um declínio rápido e progressivo da função renal, e seu achado histopatológico é a presença de crescentes em mais de 50% dos glomérulos. Relatamos um caso de um homem de 38 anos, usuário crônico de cocaína, álcool e cigarros que apresentava urina vermelha, oligúria, pernas e pálpebras inchadas, além dos sintomas urêmicos, anorexia, êmese e confusão mental. Foi internado com lesão renal aguda e realizou seis sessões de hemodiálise durante os primeiros 16 dias de internação, e depois foi transferido para um hospital terciário para investigação diagnóstica. Os testes de ANF (fator antinuclear), ANCA, anti-DNA, sorologia para vírus da hepatite B, C e HIV foram negativos. Uma biópsia percutânea renal revelou glomerulonefrite crescente, com leve atrofia tubular. O paciente foi submetido à pulsoterapia com metilprednisolona (por 3 dias) e ciclofosfamida. Em seguida, ele manteve a prednisona diária e a ciclofosfamida intravenosa mensal e evoluiu com melhora progressiva da função renal.


Subject(s)
Humans , Male , Adult , Cocaine , Acute Kidney Injury/chemically induced , Glomerulonephritis , Nephritis , Antibodies, Antineutrophil Cytoplasmic , Kidney
8.
Article in Chinese | WPRIM | ID: wpr-879808

ABSTRACT

OBJECTIVE@#To study the clinical effect and mechanism of total glucosides of paeony (TGP) in the adjuvant therapy for children with Henoch-Schönlein purpura nephritis (HSPN).@*METHODS@#Sixty-four HSPN children with moderate proteinuria were divided into a TGP treatment group (@*RESULTS@#Compared with the healthy children before treatment, the children with HSPN had higher proportion of Tfh cells and expression levels of IL-21 and IL-4 (@*CONCLUSIONS@#TGP has a marked clinical effect in the treatment of HSPN and can reduce the inflammatory response of the kidney and exert a protective effect on the kidney by inhibiting the proliferation of Tfh cells and downregulating the expression of IL-21 and IL-4 in plasma.


Subject(s)
Child , Humans , Glucosides/therapeutic use , Nephritis , Paeonia , Prospective Studies , IgA Vasculitis/drug therapy
9.
Article in Chinese | WPRIM | ID: wpr-879857

ABSTRACT

OBJECTIVE@#To study the efficacy and safety of mycophenolate mofetil (MMF) versus cyclophosphamide (CTX) in the treatment of children with Henoch-Schönlein purpura nephritis (HSPN) and nephrotic-range proteinuria.@*METHODS@#A prospective clinical trial was conducted in 68 pediatric patients who were admitted to the Department of Nephrology, Children's Hospital Affiliated to Capital Institute of Pediatrics and who were diagnosed with HSPN and nephrotic-range proteinuria from August 2016 to November 2019. The patients were randomly divided into two groups:MMF treatment (@*RESULTS@#At months 3, 6, and 12 of treatment, there was no significant difference in the complete remission rate and the response rate between the MMF treament and CTX treatment groups (@*CONCLUSIONS@#MMF and CTX have similar efficacy and safety in the treatment of HSPN children with nephrotic-range proteinuria.


Subject(s)
Child , Humans , Cyclophosphamide/adverse effects , Immunosuppressive Agents/adverse effects , Mycophenolic Acid/adverse effects , Nephritis/drug therapy , Prospective Studies , Proteinuria/etiology , IgA Vasculitis/drug therapy , Retrospective Studies
10.
Biociencias ; 15(1): 59-72, jun.2020.
Article in Spanish | LILACS, COLNAL | ID: biblio-1122950

ABSTRACT

Objective: To identify if the absence of the GSTM1 and / or GSTT1 genes is correlated with the response to cyclophosphamide and the presence of anti DNA in a patient with SLE. Methodology: An correlational cross study that included 46 patients with a diagnosis of systemic lupus erythematosus. (LES), who attended the rheumatology clinic of the Venezuelan Institute of Social Security- Central Hospital "Dr. Miguel Pérez Carreño ", during the period June 2016 - June 2017. Patients with SLE were classified according to the presence or absence of renal involvement (lupus nephritis). Each individual was extracted 5 mL of peripheral blood, from which the genomic DNA was extracted. The presence or absence of the GSTM1 and GSTT1 genes was determined using the multiplex polymerase chain reaction method with specific primers. Results: In the studied population (n = 46), 95.7% were women and only 4.3% men. The average age was 31 years, being mostly young women of childbearing age. The treatment of patients included the use of chloroquine (78%), mycophenolate (69.6%) and corticoids (38.7%). The GST genes were present, the GSTM1 gene with a frequency.


Objetivo: Identificar si la ausencia de los genes GSTM1 y/o GSTT1 está correlacionada con la respuesta a ciclofosfamida y con la presencia de anti DNA en paciente con LES. Metodología: Estudio transversal correlacional, que incluyó 46 pacientes con diagnóstico de lupus eritematoso sistémico (LES), que acudieron a la consulta de reumatología del Instituto Venezolano del Seguro Sociales- Hospital Central "Dr. Miguel Pérez Carreño", durante el período septiembre 2016 ­ junio de 2017. Los pacientes con LES se clasificaron de acuerdo a la presencia o no de afectación renal (nefritis lúpica). A cada individuo se le extrajo 5 mL de sangre periférica, a partir de la cual se extrajo el ADN genómico. La presencia o ausencia de los genes GSTM1 y GSTT1 se determinó utilizando el método de reacción en cadena de la polimerasa múltiplex con iniciadores específicos. Resultados: En la población estudiada (n= 46), 95,7% eran mujeres y sólo 4,3% hombres. La edad media fue de 31 años, siendo en su mayoría mujeres jóvenes en edad fértil. El tratamiento de los pacientes, comprendió el uso de cloroquina (78%), micofenolato (69,6 %) y corticoides (38,7%). Los genes GST estuvieron presentes, el gen GSTM1 con una frecuencia del 100 % y GSTT1 del 93,5%. Solo un 6,5% de los pacientes no presentaron el gen GSTT1. Conclusión: No se observó una correlación entre la presencia y/o ausencia de los genes GSTM1 y GSTT1 con la respuesta al tratamiento con Ciclofosfamida ni a la presencia de anti-DNA


Subject(s)
Humans , Polymorphism, Genetic , DNA , Mediation Analysis , Nephritis
11.
Adv Rheumatol ; 60: 05, 2020. tab
Article in English | LILACS | ID: biblio-1088640

ABSTRACT

Abstract Background: Nephritis occurs frequently in systemic lupus erythematosus (SLE) and may worsen disease morbidity and mortality. Knowing all characteristics of this manifestation helps to a prompt recognition and treatment. Aim: To compare the differences in clinical data, serological profile and treatment response of nephritis of early and late onset. Methods: Retrospective study of 71 SLE patients with biopsy proven nephritis divided in early nephritis group (diagnosis of nephritis in the first 5 years of the disease) and late nephritis (diagnosis of nephritis after 5 years). Epidemiological, serological, clinical and treatment data were collected from charts and compared. Results: In this sample, 70. 4% had early onset nephritis and 29.6% had late onset. No differences were noted in epidemiological, clinical, serological profile, SLICC and SLEDAI, except that late onset nephritis patients were older at nephritis diagnosis (p = 0.01). Regarding renal biopsy classification, C3 and C4 levels, serum creatinine, 24 h proteinuria and response rate to treatment the two groups were similar (p = NS). Patients with early onset had lower levels of hemoglobin at nephritis onset than those of late onset (p = 0.02). Conclusions: Most of SLE patients had nephritis in the first 5 years of disease. No major differences were noted when disease profile or treatment outcome of early and late onset nephritis were compared.(AU)


Subject(s)
Humans , Lupus Erythematosus, Systemic/physiopathology , Nephritis/diagnosis , Prognosis , Retrospective Studies , Delayed Diagnosis
12.
Med. UIS ; 32(1): 33-37, ene.-jun. 2019. graf
Article in Spanish | LILACS | ID: biblio-1040393

ABSTRACT

Resumen La apendicitis aguda en pediatría ocurre generalmente en escolares y adolescentes, siendo su principal complicación el absceso intraabdominal. Por su parte, la nefronía lobar aguda es una infección localizada del parénquima renal, de la cual no existen casos reportados de ésta como secundaria a apendicitis no perforada. Se presenta el caso de una preadolescente, quien seis días después de una apendicectomía consulta por dolor abdominal, vómito y fiebre. Ingresa con signos de respuesta inflamatoria sistémica, por lo que se inicia terapia antibiótica, y luego de tres días sin mejoría clínica, se realiza una ecografía abdominal que evidencia una lesión renal derecha sugestiva de nefronía lobar aguda y un absceso hepático del segmento VI y VII. Se inicia terapia antibiótica de amplio espectro con adecuada respuesta clínica. La nefronía lobar aguda debe tenerse en cuenta entre las complicaciones de la apendicitis, dado su comportamiento agresivo, rápida progresión a sepsis y frecuente asociación a cicatrices renales. MÉD.UIS.2019;32(1):33-7.


Abstract Acute appendicitis in pediatrics presented in scholar and adolescent ages have as its main complication an intraabdominal abscess. Moreover, acute lobar nephronia is a localized infection in the renal parenchyma. There are no cases reported about nephronia as a consequence of acute non-perforated appendicitis. This case describes a preadolescent presenting abdominal pain, vomit and fever, six days after acute appendicitis. She was admitted with signs of systemic inflammatory response syndrome, so antibiotic therapy was started. After three days there was no clinical improvement, whereby an abdominal ultrasound was performed, which reported injury in the right kidney suggestive of acute lobar nephronia and an hepatic abscess of VI and VII segments. Broad spectrum of antibiotic therapy was started with adequate clinic response. Acute lobar nephronia should be considered when studying acute appendicitis complications, given it's aggressive behavior, accelerated progression to sepsis and frequent association with renal scarring. MÉD.UIS.2019;32(1):33-7.


Subject(s)
Humans , Female , Child , Nephritis , Pediatrics , Appendectomy , Postoperative Complications , Intraabdominal Infections
13.
Hig. Aliment. (Online) ; 33(288/289): 2987-2991, abr.-maio 2019. tab, graf
Article in Portuguese | LILACS, VETINDEX | ID: biblio-1482499

ABSTRACT

O objetivo do estudo foi mensurar as principais causas de condenações viscerais e seus prejuízos econômicos em um matadouro frigorífico localizado na cidade de Corumbá - MS. Foi realizada a quantificação do número de cabeças e órgãos condenados, identificação das principais lesões e estimativa dos prejuízos econômicos causados pelas mesmas. Foram abatidos 21.097 animais, dos quais 13.215 vísceras foram condenadas. Os órgãos com maiores índices de condenações foram os pulmões (47,73%), seguido dos rins (30,2%) e fígados (12,24%). As lesões de maior incidência de condenações foram enfisema pulmonar (27,63%), nefrite (17,43) e aspiração ruminal (13,43%). As condenações levaram a um prejuízo de R$ 73.484,19 em um ano. O fígado foi o órgão responsável por mais da metade das perdas econômicas (56,59%).


Subject(s)
Pulmonary Emphysema , Wounds and Injuries , Abattoirs , Nephritis , Pneumonia, Aspiration , Refrigeration/veterinary , Viscera , Sanitary Inspection
14.
Childhood Kidney Diseases ; : 128-133, 2019.
Article in English | WPRIM | ID: wpr-785571

ABSTRACT

Henoch-Schönlein purpura (HSP) is a systemic vasculitis characterized by purpura, arthritis, abdominal pain, and nephritis. Gastrointestinal involvement can manifest as pain, intussusception, intestinal bleeding, and intestinal perforation. We report a case of fulminant HSP at an age of eight in 1994, with multiple complications of intra-thoracic bleeding, massive intestinal perforation, nephritis, and various skin rashes. The brisk bleeding findings of intestinal on Technetium-99m-labeled red blood cell scan (99mTc RBC scan) were well matched to those of the emergency laparotomy and the resected intestine. The patient's abdominal conditions improved gradually but nodular skin eruptions developed newly apart from improving preexisting lower limb rashes and the urine findings continued abnormal, so skin and kidney biopsy were done for the diagnosis. After cyclosporine therapy, skin eruptions and urine findings returned to normal gradually. On a follow-up after 25 years in 2019, the patient is 33-year-old, healthy without any abnormality on blood chemistries and urine examination.


Subject(s)
Adult , Humans , Abdominal Pain , Arthritis , Biopsy , Cyclosporine , Diagnosis , Emergencies , Erythrocytes , Exanthema , Follow-Up Studies , Hemorrhage , Intestinal Perforation , Intestines , Intussusception , Kidney , Laparotomy , Lower Extremity , Nephritis , Purpura , Skin , Systemic Vasculitis
15.
Article in English | WPRIM | ID: wpr-786124

ABSTRACT

Anti–glomerular basement membrane (GBM) nephritis is characterized by circulating anti-GBM antibodies and crescentic glomerulonephritis (GN) with deposition of IgG along the GBM. In a limited number of cases, glomerular immune complexes have been identified in anti-GBM nephritis. A 38-year-old female presented azotemia, hematuria, and proteinuria without any pulmonary symptoms. A renal biopsy showed crescentic GN with linear IgG deposition along the GBM and mesangial IgA deposition. The patient was diagnosed as concurrent anti-GBM nephritis and IgA nephropathy. Therapies with pulse methylprednisolone and cyclophosphamide administration were effective. Concurrent cases of both anti-GBM nephritis and IgA nephropathy are rare among cases of anti-GBM diseases with deposition of immune complexes. This rare case of concurrent anti-GBM nephritis and IgA nephropathy with literature review is noteworthy.


Subject(s)
Adult , Female , Humans , Anti-Glomerular Basement Membrane Disease , Antibodies , Antigen-Antibody Complex , Azotemia , Basement Membrane , Biopsy , Cyclophosphamide , Glomerulonephritis , Glomerulonephritis, IGA , Hematuria , Immunoglobulin A , Immunoglobulin G , Methylprednisolone , Nephritis , Proteinuria
16.
Article in Chinese | WPRIM | ID: wpr-775075

ABSTRACT

OBJECTIVE@#To study the clinical effect and safety of double filtration plasmapheresis (DFPP) combined with double pulse therapy with methylprednisolone (MP) and cyclophosphamide (CTX) in the treatment of children with severe Henoch-Schönlein purpura nephritis (HSPN).@*METHODS@#A total of 60 children with severe HSPN who were admitted to the hospital from January 2014 to March 2018 were enrolled and were randomly divided into an observation group and a control group (n=30 each). In addition to routine treatment, the children in the control group were given MP+CTX pulse therapy. Those in the observation group were given DFPP treatment in addition to the treatment in the control group, with three courses of treatment in total. After three courses of treatment, the two groups were compared in terms of 24-hour urinary protein, urinary microproteins, renal function parameters, adverse reactions, and clinical outcome.@*RESULTS@#After three courses of treatment, the observation group had significantly greater reductions in 24-hour urinary protein, urinary albumin, urinary immunoglobulin G, urinary β2-microglobulin, serum creatinine, and blood urea nitrogen than the control group (P0.05).@*CONCLUSIONS@#Compared with MP+CTX pulse therapy alone in the treatment of severe HSPN in children, DFPP combined with MP+CTX pulse therapy can further alleviate renal injury and improve clinical outcome and does not increase the incidence rate of adverse reactions.


Subject(s)
Child , Humans , Glucocorticoids , Immunosuppressive Agents , Nephritis , Plasmapheresis , IgA Vasculitis
17.
Article in English | WPRIM | ID: wpr-763262

ABSTRACT

Renal cortical necrosis (RCN) is patchy or diffuse ischemic destruction of the renal cortex caused by significantly reduced renal arterial perfusion. It is a rare cause of acute kidney injury (AKI) and is associated with high mortality. Here, we review the case of RCN in a 15-year-old boy who developed AKI. A 15-year-old boy was referred to our hospital from a local hospital due to a sharp decrease in his renal function. He presented with acute flank pain, nausea with vomiting, and oliguria for the past two days. He had taken a single dose of antihistamine for nasal congestion. At our hospital, his peak blood pressure was 148/83 mmHg and he had a high body mass index of 32.9 kg/m². The laboratory data showed a blood urea nitrogen (BUN) of 28.4 mg/dL, a creatinine of 4.26 mg/dL, and a glomerular filtration rate estimated from the serum cystatin C of 20.2 mL/min/1.73m². Proteinuria (spot urine protein to creatinine ratio 1.66) with pyuria was observed. Kidney sonography showed parenchymal swelling and increased renal echogenicity. Due to rapidly progressing nephritis, steroid pulse therapy (750 mg/IV) was done on the second day of his admission and the patient showed complete recovery with normal renal function. However, the kidney biopsy findings revealed renal cortical hemorrhagic necrosis. Multifocal, relatively well-circumscribed, hemorrhagic necrotic areas (about 25%) were detected in the tubulointerstitium. Although RCN is an unusual cause of AKI, especially in children, pediatricians should consider the possibility of RCN when evaluating patients with rapidly decreasing renal function.


Subject(s)
Adolescent , Child , Humans , Male , Acute Kidney Injury , Biopsy , Blood Pressure , Blood Urea Nitrogen , Body Mass Index , Creatinine , Cystatin C , Estrogens, Conjugated (USP) , Flank Pain , Glomerular Filtration Rate , Kidney , Kidney Cortex Necrosis , Mortality , Nausea , Necrosis , Nephritis , Obesity , Oliguria , Perfusion , Proteinuria , Pyuria , Vomiting
18.
Article in English | WPRIM | ID: wpr-760199

ABSTRACT

Alport syndrome (ATS) is an inherited glomerular disease caused by mutations in one of the type IV collagen novel chains (α3, α4, and α5). ATS is characterized by persistent microscopic hematuria that starts during infancy, eventually leading to either progressive nephritis or end-stage renal disease. There are 3 known genetic forms of ATS, namely X-linked ATS, autosomal recessive ATS, and autosomal dominant ATS. About 80% of patients with ATS have X-linked ATS, which is caused by mutations in the type IV collagen α5 chain gene, COL4A5. Although an 80% mutation detection rate is observed in men with X-linked ATS, some difficulties do exist in the genetic diagnosis of ATS. Most mutations are point mutations without hotspots in the COL4A3, COL4A4, and COL4A5 genes. Further, there are insufficient data on the detection of COL4A3 and COL4A4 mutations for their comparison between patients with autosomal recessive or dominant ATS. Therefore, diagnosis of ATS in female patients with no apparent family history can be challenging. Therefore, in this study, we used whole-exome sequencing (WES) to identify mutations in type IV collagen in 2 girls with glomerular basement membrane structural changes suspected to be associated with ATS; these patients had no relevant family history. Our results revealed de novo c.4688G>A (p.Arg1563Gln) and c.2714G>A (p.Gly905Asp) mutations in COL4A5. Therefore, we suggest that WES is an effective approach to obtain genetic information in ATS, particularly in female patients without a relevant family history, to detect unexpected DNA variations.


Subject(s)
Child , Female , Humans , Male , Collagen Type IV , Diagnosis , DNA , Exome , Glomerular Basement Membrane , Hematuria , Kidney Failure, Chronic , Korea , Nephritis , Nephritis, Hereditary , Point Mutation
19.
Braz. j. med. biol. res ; 52(7): e8222, 2019. graf
Article in English | LILACS | ID: biblio-1011591

ABSTRACT

Monoclonal gammopathy of renal significance (MGRS) can present with different morphologic features and lead to kidney failure. The Henoch-Schönlein purpura nephritis (HSPN) that cannot be relieved by treatment with glucocorticoid and immunosuppressive agents suggests the presence of monoclonal gammopathy in adult patients. The present study reports on a single case of HSPN associated with IgA-κMGRS. The patient who suffered from recurrent skin purpura for 6 months and nephrotic syndrome for 2 months was admitted to our hospital. Bone marrow biopsy showed monoclonal gammopathy of undetermined significance. Kidney biopsy indicated a Henoch-Schönlein purpura nephritis (HSPN, ISKDC classified as type III) with positive staining with κ-light chain in the glomeruli and renal tubular epithelial cells. Furthermore, skin biopsy showed leukocytoclastic vasculitis and negative staining for Congo red and light chain. Given both the renal and cutaneous involvement, the patient was considered to have HSPN associated with IgA-κMGRS. The patient experienced an exacerbation in his purpura-like lesions and clinical status after treatment with glucocorticoid and immunosuppressive agents. Consequently, the patient was put on a regimen that included dexamethasone (20 mg on the 1st, 4th, 8th, and 11th days of each month, iv) and bortezomib (2.4 mg on the 1st, 4th, 8th, and 11th days of each month, iv). Eight weeks after treatment, he had complete resolution of his cutaneous purpura and his biochemical parameters improved. The latent presence of MGRS in cases of HSPN should be considered in adult patients. Increased cognizance and correct treatment options could improve patient outcomes.


Subject(s)
Humans , Male , Middle Aged , Paraproteinemias/etiology , IgA Vasculitis/complications , Nephritis/complications , Paraproteinemias/pathology , Paraproteinemias/drug therapy , IgA Vasculitis/pathology , IgA Vasculitis/drug therapy , Glucocorticoids/administration & dosage , Immunosuppressive Agents/administration & dosage , Nephritis/pathology , Nephritis/drug therapy
20.
Adv Rheumatol ; 59: 21, 2019. tab, graf
Article in English | LILACS | ID: biblio-1088627

ABSTRACT

Abstract Background: The Henoch-Schonlein Purpura (HSP) or IgA vasculitis is the most common vasculitis of childhood and may occur with renal involvement, with hematuria and / or proteinuria, and may cause severe and non-reversible sequelae. Objectives: To establish the profile of patients with renal involvement due to IgA vasculitisand to describe our experience with the use of azathioprine to treat patients with nephritis. Methods: Clinical data were retrospectively collected from medical records of patients with IgA vasculitiswho attended the pediatric rheumatology unit between 1995 and 2017. Patients were separated into two groups based on whether or notthey weretreated with non-glucocorticoid immunosuppressants. Results: From the178 patients with IgA vasculitis, nephritis was found in67 patients (37.6%), 13 of whom receivedtreatment with non-glucocorticoid immunosuppressants. Ten patients responded well to azathioprine and 1 patient to cyclosporine. Forty patients received oral glucocorticoids, whilst 16received intravenous glucocorticoids. Conclusion: Azathioprine may be beneficial in the treatment of IgA vasculitis with renal involvement.


Subject(s)
Humans , IgA Vasculitis/physiopathology , Azathioprine/therapeutic use , Vasculitis/physiopathology , Nephritis/drug therapy , Health Profile
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