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1.
Eur J Endocrinol ; 182(3): C9-C12, 2020 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-31972544

RESUMO

Primary adrenal insufficiency (PAI) in children is mostly due to genetic defects. The understanding of the molecular genetics of the causes of adrenal insufficiency in the pediatric population has made significant progress during the last years. It has been shown that inherited PAI can lead to certain clinical manifestations and health problems in children beyond the adrenals. Organ dysfunctions associated with different forms of PAI in children include a wide range of organs such as gonads, brain, heart, bone, growth, bone marrow, kidney, skin, parathyroid, and thyroid. Diagnosing the correct genetic cause of PAI in children is therefore crucial to adequately control long-term treatment and follow-up in such patients.


Assuntos
Doença de Addison/genética , Hiperplasia Suprarrenal Congênita/genética , Doença de Addison/complicações , Doença de Addison/diagnóstico , Doença de Addison/fisiopatologia , Hiperplasia Suprarrenal Congênita/complicações , Hiperplasia Suprarrenal Congênita/diagnóstico , Hiperplasia Suprarrenal Congênita/fisiopatologia , Doenças do Desenvolvimento Ósseo/etiologia , Doenças do Desenvolvimento Ósseo/genética , Doenças do Desenvolvimento Ósseo/fisiopatologia , Encefalopatias/etiologia , Encefalopatias/genética , Encefalopatias/fisiopatologia , Cardiomiopatias/etiologia , Cardiomiopatias/genética , Cardiomiopatias/fisiopatologia , Transtornos do Desenvolvimento Sexual/etiologia , Transtornos do Desenvolvimento Sexual/genética , Transtornos do Desenvolvimento Sexual/fisiopatologia , Transtornos do Crescimento/etiologia , Transtornos do Crescimento/genética , Transtornos do Crescimento/fisiopatologia , Humanos , Hipoadrenocorticismo Familiar/complicações , Hipoadrenocorticismo Familiar/diagnóstico , Hipoadrenocorticismo Familiar/genética , Hipoadrenocorticismo Familiar/fisiopatologia , Síndromes de Imunodeficiência/etiologia , Síndromes de Imunodeficiência/genética , Síndromes de Imunodeficiência/fisiopatologia , Técnicas de Diagnóstico Molecular , Síndrome Nefrótica/etiologia , Síndrome Nefrótica/genética , Síndrome Nefrótica/fisiopatologia , Dermatopatias/etiologia , Dermatopatias/genética , Dermatopatias/fisiopatologia
2.
Expert Rev Clin Pharmacol ; 13(2): 147-156, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-31847609

RESUMO

Introduction: Glucocorticoids (GCs) are the first-line therapy for patients with nephrotic syndrome (NS), a common glomerular disease, that cause complete remission in most of the cases. In response to the treatment, NS patients are divided into glucocorticoid-sensitive and -resistant. This variation is due to the differences in pharmacokinetics and pharmacodynamics of GCs in each patient that affect the response to the treatment modality. Since the genetic variations in drug-metabolizing enzymes and transporter proteins significantly impact the pharmacokinetics, efficacy and safety of the applied medications, this review highlights the basic mechanisms of genetic variations involved in GCs metabolism in drug-resistant NS patients.Areas covered: This review explains the pharmacogenetic variations that influence the profile of GCs responses and their pharmacokinetics in NS patients. Moreover, the epigenetic variations including histone modifications and miRNA gene regulation that have an influence on GCs responses will review. A comprehensive literature search was performed using different keywords to the reviewed topics.Expert opinion: The accumulative data suggest the importance of pharmacogenetic studies to develop personalized therapies and increase the GCs responsiveness in these patients. It is imperative to know that genetic testing does not give absolute answers to all existing questions in steroid resistance.


Assuntos
Glucocorticoides/administração & dosagem , Síndrome Nefrótica/tratamento farmacológico , Farmacogenética , Resistência a Medicamentos , Epigênese Genética , Testes Genéticos , Variação Genética , Glucocorticoides/farmacocinética , Glucocorticoides/farmacologia , Humanos , Síndrome Nefrótica/genética , Síndrome Nefrótica/fisiopatologia , Medicina de Precisão , Indução de Remissão
3.
Med Sci (Paris) ; 35(8-9): 659-666, 2019.
Artigo em Francês | MEDLINE | ID: mdl-31532378

RESUMO

The renal filtration is ensured by the kidney glomeruli selective for filtering the blood. The main actor of the glomerular filter is the podocyte whose interlaced pedicels bear protein complexes (nephrin, podocin, etc.) creating a molecular sieve (slit diaphragm) to achieve the filtration. Alterations of these podocytes lead to massive proteinuria, which characterizes the nephrotic syndrome. The idiopathic form is one of the most malignant and essentially comprises two entities: minimal change disease and focal segmental glomerulosclerosis. Many observations indicated that (1) immune cells are involved and that (2) there are several permeability factors in the blood that affect the morphology and function of podocytes (slit diaphragm with fractional foot processes fusion/effacement). Evidence for a permeability factor was chiefly derived from remission of proteinuria observed after implantation of a kidney with FSGS in healthy recipients or with other kidney diseases. Today, we are moving towards a multifactorial conception of the nephrotic syndrome where all these barely known factors could be associated according to a sequential kinetic mechanism that needs to be determined.


Assuntos
Células Sanguíneas/fisiologia , Proteínas Sanguíneas/fisiologia , Síndrome Nefrótica/etiologia , Proteínas Sanguíneas/efeitos adversos , Glomerulosclerose Segmentar e Focal/sangue , Glomerulosclerose Segmentar e Focal/complicações , Glomerulosclerose Segmentar e Focal/fisiopatologia , Humanos , Rim/patologia , Rim/fisiologia , Glomérulos Renais/patologia , Glomérulos Renais/fisiologia , Síndrome Nefrótica/sangue , Síndrome Nefrótica/fisiopatologia , Podócitos/patologia , Podócitos/fisiologia , Proteinúria/sangue , Proteinúria/complicações , Proteinúria/fisiopatologia , Fatores de Risco
4.
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
5.
Rev Assoc Med Bras (1992) ; 65(7): 988-992, 2019 Aug 05.
Artigo em Inglês | MEDLINE | ID: mdl-31389510

RESUMO

OBJECTIVE: The objective of this study was to investigate the effects of low triiodothyronine syndrome (LT3S) on platelet function and clotting factors in patients with nephrotic syndrome(NS). METHODS: Patients with primary nephrotic syndrome were divided into two groups, normal thyroid function (group A) and LT3S (group B), based on whether they had LT3S or not. Healthy subjects were selected as the control group (group C). Blood coagulation function was detected in each group. The platelet activation function (CD62P, CD63) was determined by flow cytometry. The platelet aggregation rate was detected by an optical method using adenosine diphosphate and arachidonic acid as inducers. RESULTS: The proportion of primary nephrotic syndrome with LT3S was 23.2% (69/298). Compared with group C, group A had higher CD62P and PAgTADP, and group B had higher CD62P, CD63, PAgTAA, and PAgTADP; the difference was statistically significant (all P < 0.05). There was no significant difference in renal pathology between group A and group B (X2 = 4.957, P = 0.421). Compared with group A, the 24-hour urine protein, CD63, PAgTAA, and PAgTADP were higher in group B, and APTT and Alb were lower. The difference was statistically significant (P < 0.05). Logistic regression analysis showed that LT3S was associated with CD36 (OR: 3.516; 95% CI: 1.742~8.186; P = 0.004) and PAgTAA (OR: 0.442; 95% CI: 1.001~1.251; P = 0.037). CONCLUSION: NS patients are prone to LT3S. Patients with LT3S may have abnormal platelet activation and increase of platelet aggregation.


Assuntos
Plaquetas/fisiologia , Síndromes do Eutireóideo Doente/sangue , Síndromes do Eutireóideo Doente/fisiopatologia , Síndrome Nefrótica/sangue , Síndrome Nefrótica/fisiopatologia , Tri-Iodotironina/sangue , Adulto , Feminino , Citometria de Fluxo , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome Nefrótica/complicações , Ativação Plaquetária/efeitos dos fármacos , Ativação Plaquetária/fisiologia , Agregação Plaquetária/efeitos dos fármacos , Agregação Plaquetária/fisiologia , Contagem de Plaquetas , Testes de Função Plaquetária , Valores de Referência , Análise de Regressão , Tri-Iodotironina/deficiência
6.
BMJ Case Rep ; 12(7)2019 Jul 11.
Artigo em Inglês | MEDLINE | ID: mdl-31300605

RESUMO

Acquired C1-inhibitor (C1-INH) deficiency is a rare and potentially life-threatening disorder, which presents with recurrent attacks of non-pitting oedema to the face, airway, limbs or gastrointestinal tract. It is often associated with underlying B-cell lymphoproliferative disorders. We describe a case of a 73-year-old man with acquired C1-INH deficiency who presented with nephrotic syndrome due to glomerular IgM deposition, secondary to an underlying secretory lymphoplasmacytic lymphoma. Both the acquired C1-INH deficiency and the nephrotic syndrome resolved when the underlying B-cell lymphoma was treated with rituximab and bendamustine, suggesting the underlying lymphoproliferative malignancy was driving both disorders.


Assuntos
Angioedemas Hereditários/diagnóstico , Antineoplásicos Alquilantes/uso terapêutico , Cloridrato de Bendamustina/uso terapêutico , Síndrome Nefrótica/diagnóstico , Rituximab/uso terapêutico , Macroglobulinemia de Waldenstrom/diagnóstico , Idoso , Angioedemas Hereditários/tratamento farmacológico , Angioedemas Hereditários/etiologia , Angioedemas Hereditários/fisiopatologia , Humanos , Masculino , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/etiologia , Síndrome Nefrótica/fisiopatologia , Qualidade de Vida , Retorno ao Trabalho , Resultado do Tratamento , Macroglobulinemia de Waldenstrom/complicações , Macroglobulinemia de Waldenstrom/tratamento farmacológico , Macroglobulinemia de Waldenstrom/fisiopatologia
8.
Medicina (Kaunas) ; 55(7)2019 Jul 11.
Artigo em Inglês | MEDLINE | ID: mdl-31336742

RESUMO

Acute kidney injury in the context of nephrotic syndrome is a serious and alarming clinical problem. Largely, acute kidney injury is a relatively frequent complication among patients with comorbidities while it has been independently associated with an increased risk of adverse outcomes, including death and chronic kidney disease. Nephrotic syndrome, without hematuria or with minimal hematuria, includes a list of certain glomerulopathies; minimal change disease, focal segmental glomerulosclerosis and membranous nephropathy. In the light of primary nephrotic syndrome, pathophysiology of acute kidney injury is differentiated by the nature of the primary disease and the severity of the nephrotic state. This review aims to explore the clinical circumstances and pathogenetic mechanisms of acute kidney injury in patients with nephrotic syndrome due to primary glomerulopathies, focusing on newer perceptions regarding the pathogenesis and management of this complicated condition, for the prompt recognition and timely initiation of appropriate treatment in order to restore renal function to its baseline level. Prompt recognition of the precise cause of acute kidney injury is crucial for renal recovery. Clinical characteristics, laboratory and serological findings along with histopathological findings, if required, will reveal the implicated pathway leading to individualized approach and management.


Assuntos
Lesão Renal Aguda/etiologia , Rim/patologia , Síndrome Nefrótica/complicações , Lesão Renal Aguda/epidemiologia , Lesão Renal Aguda/fisiopatologia , Anticoagulantes/efeitos adversos , Anticoagulantes/farmacologia , Anticoagulantes/uso terapêutico , Biópsia/instrumentação , Biópsia/métodos , Inibidores de Calcineurina/efeitos adversos , Inibidores de Calcineurina/uso terapêutico , Glomerulosclerose Segmentar e Focal/etiologia , Glomerulosclerose Segmentar e Focal/fisiopatologia , Humanos , Rim/metabolismo , Rim/fisiopatologia , Nefrite Intersticial/etiologia , Nefrite Intersticial/fisiopatologia , Síndrome Nefrótica/fisiopatologia , Veias Renais/patologia , Veias Renais/fisiopatologia , Trombose/etiologia , Trombose/fisiopatologia , Varfarina/efeitos adversos , Varfarina/farmacologia , Varfarina/uso terapêutico
9.
Indian J Pediatr ; 86(2): 180-182, 2019 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-30128632

RESUMO

A seven-months-old girl under treatment for pneumonia presented with generalized edema, decreased urinary output and was found to have hypertension, muco-cutaneous fungal infection and pulmonary hypertension. Investigations revealed that she had heavy proteinuria, hypertriglyceridemia, hypoalbuminemia and elevated levels of free T3 and T4 with suppression of TSH levels in the serum. A diagnosis of autoimmune thyroiditis (AT) in thyrotoxic phase was made on the basis of clinical presentation and presence of anti-TPO antibodies and reduced uptake in thyroid (technetium) scintigraphy. The child responded to carbimazole therapy and propranolol. The case is presented to remind pediatricians about the rare occurrence of auto-immune thyroiditis in infancy with rare complications such as nephrotic syndrome and pulmonary hypertension.


Assuntos
Síndrome Nefrótica/complicações , Tireoidite Autoimune/complicações , Carbimazol/uso terapêutico , Feminino , Doença de Hashimoto/complicações , Doença de Hashimoto/fisiopatologia , Humanos , Hipertireoidismo/complicações , Hipertireoidismo/fisiopatologia , Hipertrigliceridemia , Hipoalbuminemia , Lactente , Síndrome Nefrótica/fisiopatologia , Pneumonia/terapia , Propranolol/uso terapêutico , Tecnécio/sangue , Tireoidite Autoimune/tratamento farmacológico , Tireoidite Autoimune/fisiopatologia , Tireotropina , Tri-Iodotironina/sangue
11.
Artigo em Inglês | MEDLINE | ID: mdl-30539703

RESUMO

Idiopathic Edema (IE), is a syndrome involving real or perceived weight gain secondary to the pathological retention of fluid. This syndrome of generalized edema is almost solely reported in women. The diagnosis of IE is one of exclusion and requires a careful history, physical exam, and clinical suspicion. The aim of this article is to provide a comprehensive review of the available literature in order to attempt to define IE, identify the possible causes, review the proposed pathophysiology, and discuss potential treatment options.


Assuntos
Edema/etiologia , Edema/patologia , Animais , Edema/fisiopatologia , Edema/terapia , Humanos , Hiponatremia/etiologia , Hiponatremia/patologia , Hiponatremia/fisiopatologia , Hiponatremia/terapia , Linfedema/etiologia , Linfedema/patologia , Linfedema/fisiopatologia , Linfedema/terapia , Síndrome Nefrótica/etiologia , Síndrome Nefrótica/patologia , Síndrome Nefrótica/fisiopatologia , Síndrome Nefrótica/terapia , Síndromes da Apneia do Sono/etiologia , Síndromes da Apneia do Sono/patologia , Síndromes da Apneia do Sono/fisiopatologia , Síndromes da Apneia do Sono/terapia , Ganho de Peso
12.
BMC Nephrol ; 19(1): 302, 2018 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-30382824

RESUMO

BACKGROUND: Idiopathic nephrotic syndrome (INS) is the most common chronic glomerular disease in children. Approximately 80-90% of patients with childhood INS have steroid-sensitive nephrotic syndrome (SSNS), and can obtain remission with steroid therapy, while the remainder have steroid-resistant nephrotic syndrome (SRNS). Furthermore, approximately 50% of children with SSNS develop frequently-relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS). Children with FRNS/SDNS are usually treated with immunosuppressive agents such as cyclosporine, cyclophosphamide, or mizoribine in Japan. However, 10-20% of children receiving immunosuppressive agents still show frequent relapse and/or steroid dependence during or after treatment, which is defined as complicated FRNS/SDNS. Furthermore, 30% of SRNS patients who obtain remission after additional treatments such as cyclosporine also turn out to be complicated FRNS/SDNS. For such complicated FRNS/SDNS patients, rituximab (RTX) is currently used; however, recurrence after RTX treatment also remains an open issue. Because long-term use of existing immunosuppressive drugs has limitations, development of a novel treatment for maintenance therapy after RTX is desirable. Mycophenolate mofetil (MMF) is an immunosuppressive drug with fewer side effects than cyclosporine or cyclophosphamide. Importantly, recent studies have reported the efficacy of MMF in children with nephrotic syndrome. METHODS: We conduct a multicenter, double-blind, randomized, placebo-controlled trial to evaluate the efficacy and safety of MMF after RTX therapy in children with complicated FRNS/SDNS. Patients are allocated to either RTX plus MMF treatment group, or RTX plus placebo treatment group. For the former group, MMF is administered at a dose of 1000-1200 mg/m2/day (maximum 2 g/day) twice daily for 17 months after RTX treatment. The primary endpoint is time-to-treatment failure (development of frequent relapses, steroid dependence or steroid resistance). DISCUSSION: The results will provide important data on the use of MMF as maintenance therapy after RTX to prevent complicated FRNS/SDNS patients from declining into treatment failure. In future, MMF in conjunction with RTX treatment may permit increased duration of remission in 'complicated' FRNS/SDNS cases. TRIAL REGISTRATION: This trial was prospectively registered to UMIN Clinical Trials Registry on June 23, 2014 (UMIN Trial ID: UMIN000014347 ).


Assuntos
Fatores Imunológicos/administração & dosagem , Ácido Micofenólico/administração & dosagem , Síndrome Nefrótica/diagnóstico , Síndrome Nefrótica/tratamento farmacológico , Rituximab/administração & dosagem , Esteroides/administração & dosagem , Criança , Método Duplo-Cego , Quimioterapia Combinada , Humanos , Síndrome Nefrótica/fisiopatologia , Recidiva , Resultado do Tratamento
13.
Lancet Child Adolesc Health ; 2(12): 880-890, 2018 12.
Artigo em Inglês | MEDLINE | ID: mdl-30342869

RESUMO

More than 85% of children and adolescents (majority between 1-12 years old) with idiopathic nephrotic syndrome show complete remission of proteinuria following daily treatment with corticosteroids. Patients who do not show remission after 4 weeks' treatment with daily prednisolone are considered to have steroid-resistant nephrotic syndrome (SRNS). Renal histology in most patients shows presence of focal segmental glomerulosclerosis, minimal change disease, and (rarely) mesangioproliferative glomerulonephritis. A third of patients with SRNS show mutations in one of the key podocyte genes. The remaining cases of SRNS are probably caused by an undefined circulating factor. Treatment with calcineurin inhibitors (ciclosporin and tacrolimus) is the standard of care for patients with non-genetic SRNS, and approximately 70% of patients achieve a complete or partial remission and show satisfactory long-term outcome. Additional treatment with drugs that inhibit the renin-angiotensin axis is recommended for hypertension and for reducing remaining proteinuria. Patients with SRNS who do not respond to treatment with calcineurin inhibitors or other immunosuppressive drugs can show declining kidney function and are at risk for end-stage renal failure. Approximately a third of those who undergo renal transplantation show recurrent focal segmental glomerulosclerosis in the allograft and often respond to combined treatment with plasma exchange, rituximab, and intensified immunosuppression.


Assuntos
Resistência a Medicamentos/efeitos dos fármacos , Glucocorticoides/uso terapêutico , Imunossupressores/uso terapêutico , Síndrome Nefrótica/tratamento farmacológico , Prednisolona/uso terapêutico , Indução de Remissão/métodos , Adolescente , Criança , Pré-Escolar , Feminino , Humanos , Lactente , Transplante de Rim , Masculino , Síndrome Nefrótica/fisiopatologia , Estudos Observacionais como Assunto
14.
Am J Physiol Renal Physiol ; 315(5): F1336-F1344, 2018 11 01.
Artigo em Inglês | MEDLINE | ID: mdl-30110567

RESUMO

In many cells and tissues, including the glomerular filtration barrier, scaffold proteins are critical in optimizing signal transduction by enhancing structural stability and functionality of their ligands. Recently, mutations in scaffold protein membrane-associated guanylate kinase inverted 2 (MAGI-2) encoding gene were identified among the etiology of steroid-resistant nephrotic syndrome. MAGI-2 interacts with core proteins of multiple pathways, such as transforming growth factor-ß signaling, planar cell polarity pathway, and Wnt/ß-catenin signaling in podocyte and slit diaphragm. Through the interaction with its ligand, MAGI-2 modulates the regulation of apoptosis, cytoskeletal reorganization, and glomerular development. This review aims to summarize recent findings on the role of MAGI-2 and some other scaffold proteins, such as nephrin and synaptopodin, in the underlying mechanisms of glomerulopathy.


Assuntos
Proteínas de Transporte/metabolismo , Barreira de Filtração Glomerular/metabolismo , Taxa de Filtração Glomerular , Glomerulonefrite/metabolismo , Síndrome Nefrótica/metabolismo , Citoesqueleto de Actina/metabolismo , Citoesqueleto de Actina/patologia , Animais , Apoptose , Proteínas de Transporte/genética , Transição Epitelial-Mesenquimal , Predisposição Genética para Doença , Barreira de Filtração Glomerular/patologia , Barreira de Filtração Glomerular/fisiopatologia , Glomerulonefrite/genética , Glomerulonefrite/patologia , Glomerulonefrite/fisiopatologia , Humanos , Mutação , Síndrome Nefrótica/genética , Síndrome Nefrótica/patologia , Síndrome Nefrótica/fisiopatologia , Podócitos/metabolismo , Podócitos/patologia , Transdução de Sinais
15.
Drug Des Devel Ther ; 12: 2509-2518, 2018.
Artigo em Inglês | MEDLINE | ID: mdl-30147298

RESUMO

Objective: Chronic kidney disease (CKD) is characterized by progressive loss of renal functions. At present, there are only limited therapeutic strategies to slow down the progress of CKD and there is an urgent need to develop new therapeutic strategies to treat CKD patients. Numerous research evidence supports the potential role of EGCG in the renal protection of CKD. However, the clinical use is still limited due to the poor oral bioavailability. The aim of this study was to develop pH-sensitive polymeric nanoparticles of EGCG to improve this deficiency. Materials and methods: EGCG-loaded nanoparticles (EGCG NPs) were prepared by an improved emulsion evaporation method. The formulation prepared was in spherical with uniform sizes, high encapsulation efficiencies and drug loading. The therapeutic efficacy of EGCG NPs on chronic kidney disease was investigated on model of rat Nephrotic syndrome by measuring urinary protein excretion and kidney pathology score. Results: The mean particle size was found to be 91.3±0.8 nm and the encapsulation efficiency% and drug loading% of the formulation were 80.8%±1.6% and 6.3%±1.4%, respectively. The powder X-ray diffraction and differential scanning calorimetry of EGCG NPs showed that EGCG existed in amorphous form in NPs. The release of EGCG from NPs exhibited the lower burst release at pH 1.2 (<10%) and with the increase of pH value, the release of EGCG also gradually increased. During the observation period (24 hours), the total release amount was almost 68%. EGCG NPs could significantly modify the pharmacokinetic profile and increase the bioavailability of EGCG by more than 2.4-fold in comparison with the EGCG powder group. At the end of the fourth and sixth week, proteinuria excretion of nephrotic syndrome rats treated with EGCG NPs was significantly lower than those treated with EGCG powder, and kidney pathology scores in EGCG NPs treated rats were also significantly lower than EGCG powder treated rats. Conclusion: The results of pharmacodynamics showed that compared with EGCG powder treatment group, EGCG NPs treatment group had better efficacy and reduce kidney damage.


Assuntos
Catequina/análogos & derivados , Portadores de Fármacos , Rim/efeitos dos fármacos , Nanopartículas , Síndrome Nefrótica/tratamento farmacológico , Polímeros/química , Fármacos Renais/administração & dosagem , Administração Oral , Animais , Disponibilidade Biológica , Varredura Diferencial de Calorimetria , Catequina/administração & dosagem , Catequina/química , Catequina/farmacocinética , Cristalografia por Raios X , Modelos Animais de Doenças , Composição de Medicamentos , Liberação Controlada de Fármacos , Concentração de Íons de Hidrogênio , Rim/patologia , Rim/fisiopatologia , Microscopia Eletrônica de Transmissão , Síndrome Nefrótica/patologia , Síndrome Nefrótica/fisiopatologia , Síndrome Nefrótica/urina , Difração de Pó , Proteinúria/fisiopatologia , Proteinúria/prevenção & controle , Proteinúria/urina , Ratos Sprague-Dawley , Fármacos Renais/química , Fármacos Renais/farmacocinética , Solubilidade
16.
Nephrol Ther ; 14(7): 501-506, 2018 Dec.
Artigo em Francês | MEDLINE | ID: mdl-30150079

RESUMO

Idiopathic nephrotic syndrome represents up to 30% of adult glomerulopathies. However, its prognosis according to remission, relapse and renal failure remains unchanged since the 80s and prediction remains difficult. Physiopathology of adult idiopathic nephrotic syndrome is complex and multifactorial, including immunologic and environmental factors and a putative permeability-circulating factor, still unknown. In this point of view, we propose to summarize actual knowledge about idiopathic minimal change disease and focal and segmental glomerulosclerosis physiopathology.


Assuntos
Glomerulosclerose Segmentar e Focal/fisiopatologia , Nefrose Lipoide/fisiopatologia , Síndrome Nefrótica/fisiopatologia , Humanos , Rim/fisiopatologia
17.
BMJ Case Rep ; 20182018 Aug 27.
Artigo em Inglês | MEDLINE | ID: mdl-30150332

RESUMO

Diagnosing patients simply with heart failure or nephrotic syndrome is insufficient, and clinicians should always search for the underlying causes of these syndromes. Amyloidosis represents a rare group of diseases in which abnormal protein, namely amyloid fibrils, build up in various organs. Presentation depends on which organ systems are involved, and symptoms could include breathlessness associated with fluid overload suggestive of cardiac and/or renal involvement and diarrhoea and weight loss, suggestive of gastrointestinal involvement. The authors present a case of congestive cardiac failure and nephrotic range proteinuria in a patient with persistent fluid overload secondary toamyloid light-chain (AL) amyloidosis.


Assuntos
Amiloidose/diagnóstico , Protocolos de Quimioterapia Combinada Antineoplásica/administração & dosagem , Insuficiência Cardíaca/diagnóstico , Síndrome Nefrótica/diagnóstico , Idoso , Amiloidose/tratamento farmacológico , Amiloidose/fisiopatologia , Bortezomib , Ciclofosfamida , Dexametasona , Diagnóstico Diferencial , Ecocardiografia , Feminino , Insuficiência Cardíaca/etiologia , Insuficiência Cardíaca/fisiopatologia , Humanos , Síndrome Nefrótica/tratamento farmacológico , Síndrome Nefrótica/fisiopatologia , Diálise Renal , Resultado do Tratamento
20.
Nutrients ; 10(6)2018 May 27.
Artigo em Inglês | MEDLINE | ID: mdl-29861470

RESUMO

BACKGROUND: It was shown in animal models and adults that the epidermal growth factor (EGF) is involved in the pathophysiology of calcineurin inhibitor (CNI) induced renal magnesium loss. In children, however, the exact mechanism remains unclear, which was set as the purpose of the present study. METHODS: Children with nephrotic syndrome and renal transplant children treated with CNI (n = 50) and non-CNI treated children (n = 46) were included in this study. Urine and serum samples were collected at three time points to determine magnesium, creatinine, and EGF. The magnesium intake was calculated from a food frequency questionnaire. RESULTS: Serum Mg2+ and urinary EGF/creatinine were significantly lower in the CNI treated children, with significantly more CNI-treated children developing hypomagnesaemia. In the latter patients, the fractional excretion of magnesium (FE Mg2+) was significantly higher. Urinary EGF, age, renal function, and serum magnesium were independent predictors of the FE Mg2+. Only 29% of the children reached the recommended daily intake of magnesium. The magnesium intake did not differ between hypomagnesemic and normomagnesemic patients and was not a predictor of the FE Mg2+. CONCLUSIONS: In CNI-treated children who developed hypomagnesemia, the FE Mg2+ was increased. The urinary EGF concentration, age, and renal function are independent predictors of the FE Mg2+.


Assuntos
Inibidores de Calcineurina/efeitos adversos , Fator de Crescimento Epidérmico/urina , Falência Renal Crônica/tratamento farmacológico , Rim/efeitos dos fármacos , Deficiência de Magnésio/induzido quimicamente , Magnésio/urina , Síndrome Nefrótica/tratamento farmacológico , Adolescente , Adulto , Inibidores de Calcineurina/uso terapêutico , Criança , Fenômenos Fisiológicos da Nutrição Infantil , Pré-Escolar , Estudos de Coortes , Estudos Transversais , Feminino , Humanos , Imunossupressores/efeitos adversos , Imunossupressores/uso terapêutico , Rim/fisiopatologia , Falência Renal Crônica/metabolismo , Falência Renal Crônica/fisiopatologia , Falência Renal Crônica/cirurgia , Transplante de Rim/efeitos adversos , Estudos Longitudinais , Magnésio/sangue , Magnésio/uso terapêutico , Deficiência de Magnésio/etiologia , Deficiência de Magnésio/prevenção & controle , Masculino , Síndrome Nefrótica/sangue , Síndrome Nefrótica/fisiopatologia , Síndrome Nefrótica/urina , Eliminação Renal/efeitos dos fármacos , Adulto Jovem
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