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1.
Medicine (Baltimore) ; 99(5): e18960, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-32000422

RESUMO

To investigate the changes in blood coagulability as measured by thromboelastography (TEG) in patients with nephrotic syndrome of different etiologies as well as in patients with venous thromboembolic events (VTE).From January 2013 to October 2017, patients who were diagnosed as idiopathic membranous nephropathy (IMN), minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) were enrolled into this retrospective study in which their clinical characteristics, including TEG variables, were investigated. According to the presence or absence of VTE, the patients with IMN were divided into 2 groups of VTE and non-VTE. The risk factors of VTE were analyzed with logistic regression.Significant differences in TEG parameters were found among the 3 groups of patients with R and K values lower, while the α-angle, maximum amplitude (MA) and confidence interval (CI) values higher, in the IMN group than those in the MCD and FSGS groups (P < .01). Multiple linear regression analysis indicated that the histologic subtype was an independent relevant factor of K time, angle, MA, and CI values. Multivariate logistic regression analysis revealed that serum albumin and CI value were independent risk factors of VTE (P < .05).The results showed that IMN patients may have higher whole blood coagulability than MCD and FSGS patients. The hypercoagulability in IMN patients may be attributed to platelet hyperactivity and the accelerated fibrin-platelet interaction. Hypoproteinemia and increased CI value were independent risk factors of VTE in IMN.


Assuntos
Síndrome Nefrótica/complicações , Tromboelastografia/métodos , Trombofilia/diagnóstico , Trombofilia/etiologia , Adulto , Feminino , Glomerulosclerose Segmentar e Focal/complicações , Humanos , Masculino , Pessoa de Meia-Idade , Nefrose Lipoide/complicações , Estudos Retrospectivos , Fatores de Risco
3.
Mol Genet Genomics ; 295(1): 135-142, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31520189

RESUMO

High-throughput techniques such as whole-exome sequencing (WES) show promise for the identification of candidate genes that underlie Mendelian diseases such as nephrotic syndrome (NS). These techniques have enabled the identification of a proportion of the approximately 54 genes associated with NS. However, the main pitfall of using WES in clinical and research practice is the identification of multiple variants, which hampers interpretation during downstream analysis. One useful strategy is to evaluate the co-inheritance of rare variants in affected family members. Here, we performed WES of a patient with steroid-resistant NS (SRNS) and intermittent microhematuria. Currently, 15 years after kidney transplantation, this patient presents normal kidney function. The patient was found to be homozygous for a rare MYO1E stop-gain variant, and was heterozygous for rare variants in NS-associated genes, COL4A4, KANK1, LAMB2, ANLN, E2F3, and APOL1. We evaluated the presence or absence of these variants in both parents and 11 siblings, three of whom exhibited a milder phenotype of the kidney disease. Analysis of variant segregation in the family, indicated the MYO1E stop-gain variant as the putative causal variant underlying the kidney disease in the patient and two of her affected sisters. Two secondary variants in COL4A4-identified in some other affected family members-require further functional studies to determine whether they play a role in the development of microhematuria in affected family members. Our data illustrate the difficulties in distinguishing the causal pathogenic variants from incidental findings after WES-based variant analysis, especially in heterogenous genetic conditions, such as NS.


Assuntos
Exoma/genética , Síndrome Nefrótica/genética , Adulto , Feminino , Variação Genética/genética , Heterozigoto , Homozigoto , Humanos , Rim/patologia , Masculino , Linhagem , Fenótipo , Sequenciamento Completo do Exoma/métodos , Adulto Jovem
4.
Int J Clin Pharmacol Ther ; 58(1): 1-9, 2020 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-31319907

RESUMO

OBJECTIVE: To establish a population pharmacokinetic (PopPK) model of cyclosporine A (CsA) in Chinese patients with nephrotic syndrome (NS) and to use the model to guide the adjustment of individualized dosage regimens. MATERIALS AND METHODS: 216 CsA therapeutic drug monitoring (TDM) concentration observations were collected from 127 Chinese patients with NS. The basic model was developed as a one-compartment PK model with first-order absorption and linear elimination. The first-order conditional estimation (FOCE) method was applied to establish the final model with covariates using NONMEM software. The final model was evaluated through internal validation including goodness-of-fit analysis and bootstrap method as well as external validation using 39 additional PK observations from 35 patients with NS. RESULTS: A PopPK model of CsA was established in Chinese NS patients with influence of body weight on clearance. The internal and external validation results showed that the final model was stable. CONCLUSION: The established population model adequately characterized the PK of CsA in Chinese patients and could support individualized medication during treatment of NS with CsA.


Assuntos
Ciclosporina/farmacocinética , Imunossupressores/farmacocinética , Síndrome Nefrótica/tratamento farmacológico , Grupo com Ancestrais do Continente Asiático , China , Humanos , Modelos Biológicos , Software
5.
Pan Afr Med J ; 34: 75, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31819791

RESUMO

We describe a patient who developed nephrotic syndrome in the setting of ovarian tumor. A kidney biopsy showed minimal change nephropathy (MCN). CT scan and MR imaging followed by surgery lead to diagnostic of ovarian dermoid cyst. Surgery combined with corticosteroids resulted in a complete remission of nephrotic syndrome with disappearance of proteinuria after 3 weeks. Ten other cases of ovarian tumor associated with glomerulopathy are reviewed. This is the second case of an ovarian teratoma associated with MCN. Accurate history, physical examination, laboratory data, and kidney biopsy are highlighted in establishing the correct diagnosis in such patients.


Assuntos
Nefrose Lipoide/diagnóstico , Síndrome Nefrótica/diagnóstico , Neoplasias Ovarianas/diagnóstico , Teratoma/diagnóstico , Feminino , Humanos , Imagem por Ressonância Magnética , Pessoa de Meia-Idade , Nefrose Lipoide/etiologia , Síndrome Nefrótica/etiologia , Neoplasias Ovarianas/complicações , Neoplasias Ovarianas/terapia , Proteinúria/etiologia , Indução de Remissão , Teratoma/complicações , Teratoma/terapia , Tomografia Computadorizada por Raios X
6.
Medicine (Baltimore) ; 98(49): e18247, 2019 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-31804353

RESUMO

RATIONALE: Patients with chronic Strongyloides stercoralis infection are usually asymptomatic; therefore, their condition is easily overlooked. In immunosuppressed patients, mortality is high because of disseminated infection and hyperinfection. This report describes a fatal S stercoralis hyperinfection in a patient with nephrotic syndrome after treatment with steroids. PATIENT CONCERNS: A 70-year-old male presented with a history of progressive edema, skin infection, persistent fever, cough, intermittent abdominal pain, and progressive respiratory failure after steroid treatment. DIAGNOSIS: Nephrotic syndrome; cellulitis; S stercoralis hyperinfection; Klebsiella pneumonia. INTERVENTIONS: During the first hospital admission, the patient was administered full-dose glucocorticoid and antibiotic therapy after suffering from cellulitis. During the second admission, he was diagnosed and treated for normal digestive discomfort and a bacterial infection. The patient had progressive respiratory failure and was placed on a ventilator. He was immediately treated with albendazole when S stercoralis was found in samples of his sputum and feces. OUTCOMES: The patient died despite treatment with albendazole and antibiotic therapy. LESSONS: It is essential to consider the possibility of S stercoralis infection in immunosuppressed patients with nephrotic syndrome. Given the lack of classic manifestations and high mortality rate of advanced disease, continuous monitoring, early diagnosis, and proper treatment are imperative.


Assuntos
Infecções por Klebsiella/diagnóstico , Síndrome Nefrótica/diagnóstico , Estrongiloidíase/diagnóstico , Idoso , Animais , Doença Crônica , Coinfecção , Diagnóstico Diferencial , Evolução Fatal , Humanos , Hospedeiro Imunocomprometido , Infecções por Klebsiella/tratamento farmacológico , Masculino , Síndrome Nefrótica/tratamento farmacológico , Strongyloides stercoralis , Estrongiloidíase/tratamento farmacológico
7.
Georgian Med News ; (294): 68-71, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31687952

RESUMO

The aim of our work was to determine the gene polymorphism of cytokines IL-1ß (-511) and IL-10 (-1082) in children with nephrotic syndrome. 20 patients with nephrotic syndrome were recruited into the study from 2017 to 2018 years in single center. Our study included children with levels of glomerular filtration rate >90 ml/min. Genetic polymorphism of IL-1ß (-511) and IL-10 (-1082) and serum IL1ß were evaluated. Analyzing the contents of IL-1ß in serum of children with nephrotic syndrome, we found that IL-1ß was significantly increased in children with steroid-resistant nephrotic syndrome and with progression of glomerulonephritis compared with remission and with healthy children (p<0.05). The presence of C/T genotype is associated with increased production of interleukin-1ß in serum, compared with children with genotype C/C (p<0.05). Checking the polymorphism of SNP -1082 of IL-10 we determined that in 50% of children with nephrotic syndrome there was G/A genotype, in 40% - G/G genotype, and genotype А/А was only in 10% of patients. A strong direct relationship between the level of IL-1ß in serum and C/T allelic polymorphism of the gene IL-1ß (-511) was found (r=+0,56) (p<0.05). Gene polymorphism of IL-1ß (-511) can be used as a marker of progression of glomerulonephritis, nephrotic syndrome but more studies are needed.


Assuntos
Interleucina-10/genética , Interleucina-1beta/genética , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/genética , Polimorfismo Genético/genética , Criança , Feminino , Predisposição Genética para Doença , Genótipo , Humanos , Interleucina-10/sangue , Interleucina-1beta/sangue , Masculino , Síndrome Nefrótica/imunologia
8.
Medicine (Baltimore) ; 98(46): e17999, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31725668

RESUMO

INTRODUCTION: Heavy and light chain amyloidosis is an extremely rare condition. There are few reports referring to the clinical impact of cardiac involvement in heavy and light chain amyloidosis, and the significance of myocardial impairment has not yet been completely explained. PATIENT CONCERNS: A 66-year-old Japanese man was admitted to our hospital presenting with nephrotic syndrome and congestive heart failure. DIAGNOSIS: Kidney and endoscopic gastric mucosal biopsy demonstrated congophilic hyalinization in most of the glomeruli and surrounding vessel walls, which were highly positive for immunoglobulin A and lambda. Finally, the patient was diagnosed as an atypical multiple myeloma with systemic heavy and light chain amyloidosis. INTERVENTIONS: The patient was referred to hematology for further treatment and was moved to another hospital for the administration of chemotherapy using melphalan and dexamethasone. OUTCOMES: The patient was still alive after 15-month follow-up from the initial diagnosis. CONCLUSION: Initial screening and follow-up for cardiac involvement are important for heavy and light chain amyloidosis. Further investigation for the prognosis of heavy and light chain amyloidosis is required to improve the strategies of diagnosis and treatment options for patients with this disease.


Assuntos
Amiloidose/complicações , Insuficiência Cardíaca/complicações , Cadeias Pesadas de Imunoglobulinas/sangue , Cadeias Leves de Imunoglobulina/sangue , Síndrome Nefrótica/complicações , Idoso , Amiloidose/patologia , Insuficiência Cardíaca/patologia , Humanos , Amiloidose de Cadeia Leve de Imunoglobulina/complicações , Amiloidose de Cadeia Leve de Imunoglobulina/patologia , Masculino , Síndrome Nefrótica/patologia
9.
Medicine (Baltimore) ; 98(48): e18117, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31770238

RESUMO

RATIONALE: To examine atypical manifestations of Kawasaki disease (KD) in children. BACKGROUND: Cardiovascular complications during acute KD are a major contributor to its mortality rate. It can involve the pericardium, the myocardium, the endocardium, and/or the coronary arteries; however, cardiomegaly and nephrotic syndrome (NS) during the acute stage of KD have seldom been reported. PATIENT CONCERNS: Two children, each with a fever lasting more than 5 days, were diagnosed with cardiomegaly using echocardiography in the early phase of Kawasaki disease (within 2 weeks). Case 1 was misdiagnosed with NS because of the proteinuria, hypoalbuminemia, and edema present at the onset of the disease. DIAGNOSES: A diagnosis of incomplete KD was based on a constellation of clinical manifestations and symptoms and was supported by laboratory results. INTERVENTIONS: Intravenous immunoglobulin (IVIG) and aspirin were administered, supplemented with and without supplemental steroid therapy (case dependent). OUTCOMES: The clinical manifestations and syndromes of the two cases were completely resolved and their heart size restored to normal within 2 weeks, with no evidence of coronary artery lesions (CAL). MAIN LESSONS: Physical findings and manifestations are atypical in incomplete KD. Cardiomegaly and nephrotic syndrome can be an early manifestation of KD; cardiomegaly, especially, should be recognized as a possible manifestation of the acute stage of KD. Furthermore, these symptoms can be rapidly relieved by treatment with IVIG, with or without supplemental steroid therapy.


Assuntos
Cardiomegalia/etiologia , Síndrome de Linfonodos Mucocutâneos/complicações , Síndrome Nefrótica/etiologia , Cardiomegalia/patologia , Criança , Feminino , Humanos , Lactente , Masculino , Síndrome de Linfonodos Mucocutâneos/patologia , Síndrome Nefrótica/patologia
10.
Medicine (Baltimore) ; 98(45): e17870, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31702653

RESUMO

RATIONALE: Bevacizumab-an inhibitor of vascular endothelial growth factor-is effective against various advanced cancers. However, it is associated with the development of hypertension and high-grade proteinuria during thrombotic microangiopathy of the kidney. In addition, there are several reports of immunoglobulin A deposition in the glomeruli, but the etiology is unclear. PATIENT CONCERNS: A 67-year-old Japanese man with metastatic rectal cancer underwent low anterior rectal resection, followed by treatment with bevacizumab and SOX (S-1 plus oxaliplatin). Six months later, the patient developed hematuria, nephrotic syndrome, and purpura. DIAGNOSES: Renal biopsy revealed endocapillary proliferative glomerulonephritis. Immunofluorescence analyses showed granular mesangial deposition of galactose-deficient immunoglobulin A1. Skin biopsy revealed leukocytoclastic vasculitis. INTERVENTIONS: We ceased bevacizumab treatment, while continuing the remaining chemotherapy regimen, as we suspected bevacizumab-induced nephropathy. OUTCOMES: Proteinuria and purpura improved immediately after cessation of bevacizumab. We identified this as a case of bevacizumab-induced immunoglobulin A vasculitis with nephritis. LESSONS: To our knowledge, this is the first case of bevacizumab-related immunoglobulin A vasculitis with nephritis, as evidenced by galactose-deficient immunoglobulin A1. When a patient's urine tests are abnormal during bevacizumab treatment, clinicians should consider not only thrombotic microangiopathy but also vasculitis.


Assuntos
Antineoplásicos Imunológicos/efeitos adversos , Bevacizumab/efeitos adversos , Glomerulonefrite por IGA/induzido quimicamente , Vasculite/induzido quimicamente , Idoso , Hematúria/induzido quimicamente , Humanos , Imunoglobulina A/efeitos dos fármacos , Masculino , Síndrome Nefrótica/induzido quimicamente , Púrpura/induzido quimicamente
12.
J Biol Regul Homeost Agents ; 33(5 Suppl. 1): 13-18, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31630708

RESUMO

Nephrotic Syndrome (NS) is a rare diseases (around 2-7 cases per 100.000 children per year) characterized by proteinuria ≥50 mg/kg/day (or ≥40 mg/m2/h) or a proteinuria/creatininuria ratio >2 (mg/mg); hypoalbuminaemia less than 25 g/l and edema. The protein leakage, with the consequent hypoalbunaemia and edema, due to podocyte alterations may be caused by genetic diseases, immunological mechanisms, infections, toxins or malignancy. However, most commonly the exact etiology is unknow. The idiopathic NS may be classified based on response to corticosteroid therapy or the hytological appearance. The first classification identifies steroid-resistant NS (no response after 4 weeks of steroid therapy); frequently relapsing NS (≥ 2 relapses in first 6 months or ≥4 relapses in 1-year); steroid dependent NS (relapses during steroid decalage or within 2 weeks from steroid therapy interruption). The hystological classification is based on light and electron microscopy after renal biopsy, which is indicated in case of onset disease before 1 year or after 12 years of age. Macroscopic hematuria: persistent hypertension and/or microscopic hematuria and/or low plasma C3 renal failure not related to hypovolemia; steroid resistence: secondary or relatedsyndromes NS. Minimal change disease (MCD) is the most common form of idiopahtic NS in children, with good response to steroid treatment, and it is characterized by normal glomerular appearance on light microscopy and evidence of podocyte foot alterations on electron microscopy, due to immunological related damage. Focal segmental glomerulosclerosis (FSGS) is described inidiopahtic NS, particularly in steroiddependent or steroid-resistant forms, and is characterized by evidence of focal glomerular damage with secondary sclerosis and adhesion with Bowman's capsule; the electron appearance is the same of MCD one. Recent authors hypotizethat the FSGS is an evolution of MCD. These 2 idiopathic NS forms may be expression of the same immunological disease, with 2 different severity grades; so they may be considered different moments of the same disease spectrum. Less common idiopathic NS forms are membrano proliferative glomerulonephritis; membranous nephropathy; IgM-nephropathy; C1q nephropathy and thin basement membrane disease (1, 2, 3).


Assuntos
Síndrome Nefrótica/imunologia , Criança , Glomerulosclerose Segmentar e Focal/patologia , Hematúria/patologia , Humanos , Podócitos , Proteinúria/patologia
14.
Zhonghua Yi Xue Yi Chuan Xue Za Zhi ; 36(10): 1022-1024, 2019 Oct 10.
Artigo em Chinês | MEDLINE | ID: mdl-31598951

RESUMO

OBJECTIVE: To explore the genetic basis for a fetus suspected for congenital nephrotic syndrome of Finland (CNF). METHODS: Genomic DNA was extracted from peripheral and umbilical cord blood samples derived from both parents and the fetus. Potential variants were detected by using next-generation sequencing. Suspected variants were confirmed by Sanger sequencing. RESULTS: The fetus was found to carry compound heterozygous variants c.1440+1G>A and c.925G>T of the NPHS1 gene, which were respectively inherited from its mother and father. CONCLUSION: Identification of the compound heterozygous NPHS1 variants has enabled diagnosis of CNF in the fetus and genetic counseling for the affected family.


Assuntos
Síndrome Nefrótica/congênito , Síndrome Nefrótica/diagnóstico , Feminino , Feto , Finlândia , Heterozigoto , Humanos , Proteínas de Membrana/genética , Gravidez , Diagnóstico Pré-Natal
15.
Turk J Pediatr ; 61(1): 111-116, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31559731

RESUMO

Düzova A, Gülhan B, Topaloglu R, Özaltin F, Cengiz AB, Yetimakman AF, Dogru D, Güçer S, Besbas N. BK virus associated nephropathy and severe pneumonia in a kidney transplanted adolescent with Schimke immune-osseous-dysplasia. Turk J Pediatr 2019; 61: 111-116. Patients with juvenile onset Schimke immune-osseous-dysplasia (SIOD) have less severe symptoms and can survive in the second and third decade of life. We present an 18 year-old adolescent with juvenile onset SIOD who was diagnosed after renal transplantation and developed BK virus associated nephropathy (BKVAN) and severe pneumonia during follow-up. The patient developed nephrotic syndrome, unresponsive to immunosuppressives, at the age of 8 years. He had a history of meningitis, short stature, microcephaly, prominent ears, and bilateral cryptorchidism. A renal transplantation was performed at the age of 15 years. During follow-up, he suffered from leucopenia, urinary tract infections, herpes labialis, and candida esophagitis. Sanger sequencing of SMARCAL1 revealed a missense mutation on exon 11 (R586W). A renal biopsy performed after a sharp increase in serum creatinine (without significant viremia) revealed BKVAN which responded to sirolimus monotherapy and cidofovir. Three months later, he suffered from productive cough and dyspnea with diffuse ground glass pulmonary infiltrates. His clinical situation deteriorated and non-invasive mechanical ventilation was started. Cidofovir (2 mg/kg) was re-started weekly for a possible BKV pneumonia with intravenous immunoglobulin. After 5 doses of cidofovir and intense antibiotic regime, his dyspnea resolved with stable graft functions. In our case; BKVAN, which developed without significant viremia, and possibly associated pneumonia were treated successfully with cidofovir and sirolimus monotherapy.


Assuntos
Síndrome Nefrótica/virologia , Pneumonia Viral/complicações , Infecções por Polyomavirus/complicações , Transplantados , Adolescente , Arteriosclerose/complicações , Vírus BK , DNA Helicases/genética , Humanos , Transplante de Rim , Masculino , Mutação de Sentido Incorreto , Síndrome Nefrótica/complicações , Osteocondrodisplasias/complicações , Embolia Pulmonar/complicações
16.
Nephrol Nurs J ; 46(4): 413-445, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-31490051

RESUMO

Pediatric patients with chronic kidney disease (CKD) have an increased risk of developing vaccine-preventable diseases due to reduced immunization coverage. Studies have demonstrated that reduced immunization coverage in this population is related to barriers, such as frequent hospitalization, lack of knowledge, and concerns about safety and efficacy. This article examines a nurse practitioner-led quality improvement project (QIP) conducted in an outpatient pediatric nephrology clinic. The QIP focused on educating pediatric providers related to age-appropriate immunizations for children with CKD or nephrotic syndrome, and those who are renal transplant candidates and recipients. A process is now in place to review immunization records upon initial visit and annually, and to notify primary care providers of current recommendations for this population.


Assuntos
Imunização/normas , Transplante de Rim , Síndrome Nefrótica , Insuficiência Renal Crônica , Criança , Humanos , Profissionais de Enfermagem Pediátrica , Guias de Prática Clínica como Assunto , Melhoria de Qualidade , Literatura de Revisão como Assunto
17.
Pol Merkur Lekarski ; 47(278): 72-75, 2019 Aug 30.
Artigo em Polonês | MEDLINE | ID: mdl-31473757

RESUMO

INTRODUCTION: Cerebral salt wasting syndrome (CSWS), characterized by natriuresis, polyuria, and hypovolemia, is a rare complication of central nervous system injury or disease. A CASE STUDY: 12-year-old girl was admitted with second attack of nephrotic syndrome (NS). On admission she presents with edema, blood pressure 110/60 mm Hg, proteinuria 145 mg/kg/24h, hypoalbuminemia (1.7 g/dL), GFR 94.4 mL/min/1.73m2, sodium 133 mmol/L. On 5th day the patient developed thrombosis of right subclavian and axillary vein and was treated with recombinant tissue plasminogen activator (0.3 mg/kg/h i.v.). 45 minutes after onset of the infusion severe headache appeared. Computed tomography revealed subarachnoid hemorrhage in a region of left occipital lobe and posterior 1/3 part of sickle of the brain. Control ultrasonography examination revealed resolution of the thrombus. No deficits were found on neurologic examination. Proteinuria subsided on 11th day of hospitalization. After the hemorrhage hypovolemia, hypotension (80/40 - 100/60 mm Hg, heart rate 100/min), polyuria, and pathologic natriuresis (up to 13.0 mmol/kg/24h) were observed. Cerebral salt wasting syndrome was recognized. The girl was supplemented with oral and intravenous sodium (up to 10 mmol/ kg/24h). In following days gradual decrease of diuresis and urinary sodium loss was observed. The patient was discharged home after 41 days with normal diuresis (1.5l/24h) and natriuresis (1.44 mmol/kg/24h). CONCLUSIONS: Treatment of thromboembolic complications in children with NS poses a risk of central nervous system bleeding. Serum sodium concentration and diuresis must be strictly monitored in patients with central nervous system lesion, especially in the course of nephrotic syndrome.


Assuntos
Hiponatremia , Síndrome Nefrótica , Hemorragia Subaracnóidea , Criança , Feminino , Humanos , Hiponatremia/complicações , Natriurese , Síndrome Nefrótica/complicações , Hemorragia Subaracnóidea/complicações , Síndrome , Ativador de Plasminogênio Tecidual , Equilíbrio Hidroeletrolítico
18.
Transplant Proc ; 51(7): 2283-2288, 2019 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-31400976

RESUMO

Congenital nephrotic syndrome (CNS) is a genetic disease that is present in the antenatal period or during the first 3 months of life. In this study, we aimed to compare growth parameters of patients with CNS who received kidney transplants and either (1) had a normal glomerular filtration rate (GFR) at the time of transplant or (2) chronic kidney disease (CKD) at the time of transplant. Patients with a diagnosis of CNS who had a minimum follow-up period of 6 months were evaluated retrospectively. Children at stages 4 or 5 CKD or patients receiving dialysis during the pretransplant period were defined as group 1; patients with normal GFR at the time of transplantation were classified as group 2. Short stature and low weight were defined as less than -2 standard deviation scores (SDS) for height and weight according to their age. A total of 17 patients were included in the study. Thirteen of 17 patients had NPHS1 gene mutations. Group 1 and group 2 consisted of 8 and 9 patients, respectively. Mean height SDS and mean weight SDS in group 2 were higher than group 1 in the pretransplant period (-4.34 ± 1.74 vs -2.84 ± 1.56; P = .011 and -3.54 ± 0.93 vs -1.83 ± 1.13; P = .008). In the post-transplant period, the significant difference in height SDS continued in favor of group 2 (-3.16 ± 1.11 vs -1.16 ± 0.87; P = .002). The short stature rate was 83% in group 1 and 72% in group 2 in the pretransplant period (P = .62), and 83% in group 1 and 27% in group 2 in the post-transplant period (P = .02). Early renal transplantation seems to be effective for optimal height gain in children with CNS.


Assuntos
Transtornos do Crescimento/etiologia , Transplante de Rim/estatística & dados numéricos , Síndrome Nefrótica/cirurgia , Complicações Pós-Operatórias/etiologia , Insuficiência Renal Crônica/cirurgia , Fatores de Tempo , Adolescente , Estatura , Peso Corporal , Criança , Pré-Escolar , Feminino , Taxa de Filtração Glomerular , Humanos , Transplante de Rim/métodos , Masculino , Síndrome Nefrótica/fisiopatologia , Período Pós-Operatório , Diálise Renal/estatística & dados numéricos , Insuficiência Renal Crônica/congênito , Insuficiência Renal Crônica/fisiopatologia , Estudos Retrospectivos
19.
Clin J Am Soc Nephrol ; 14(9): 1355-1362, 2019 Sep 06.
Artigo em Inglês | MEDLINE | ID: mdl-31416888

RESUMO

BACKGROUND AND OBJECTIVES: Nonsteroidal anti-inflammatory drugs (NSAIDs) have been associated with AKI. Their association with nephrotic syndrome has not been systematically studied. This study aimed to assess the risk of nephrotic syndrome associated with NSAID use. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: A matched case-control study was performed in the UK primary care database. Cases were patients with a first diagnosis of nephrotic syndrome and controls were those without nephrotic syndrome. NSAID exposure (grouped either based on cyclooxygenase enzyme selectivity and chemical groups) was classified as either current (use at the nephrotic syndrome diagnosis date and corresponding date in the control group), recent, or past use. Odds ratios (ORs) and 95% confidence intervals (95% CIs) were calculated using unconditional logistic regression analysis. RESULTS: We included 2620 cases and 10,454 controls. Compared with non-use, current use of 15-28 days and >28 days of conventional NSAIDs was associated with a higher relative risk of nephrotic syndrome: adjusted OR, 1.34; 95% CI, 1.06 to 1.70, and OR, 1.42; 95% CI, 0.79 to 2.55, respectively. Also, recent use (discontinuation 1-2 months before nephrotic syndrome diagnosis date; OR, 1.55; 95% CI, 1.11 to 2.15) and past use (discontinuation 2 months-2 years; OR, 1.24; 95% CI, 1.07 to 1.43), but not current use of <15 days (OR, 0.78; 95% CI, 0.46 to 1.31) nor past use (discontinuation >2 years; OR, 0.96; 95% CI, 0.85 to 1.09) were associated with a higher relative risk of nephrotic syndrome as well as past use of selective COX-2 inhibitors (discontinuation 2-24 months; OR, 1.24; 95% CI, 0.98 to 1.58). Categorization based on chemical groups showed that acetic acid and propionic acid derivatives were associated with a higher risk of nephrotic syndrome. CONCLUSIONS: The use of conventional NSAIDs was associated with a higher risk of nephrotic syndrome starting from at least 2 weeks of exposure, as well as for recent and past exposure up to 2 years before the diagnosis of nephrotic syndrome. This higher risk appeared mainly attributable to acetic acid and propionic acid derivatives.


Assuntos
Usuários de Drogas , Síndrome Nefrótica , Anti-Inflamatórios não Esteroides , Estudos de Casos e Controles , Inibidores de Ciclo-Oxigenase 2 , Humanos
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