Urinary loss of thyroid hormones: An issue to remember.

A 45-year-old woman suffering from primary hypothyroidism, previously well substituted with levothyroxine, was urgently referred from Primary Care to Endocrinology due to very elevated thyrotropin, free thyroxine at low limit of normality, very high cholesterol and generalised oedema. Hypothyroidism was suspected as the main aetiology of this clinical condition. A detailed examination showed nephrotic range proteinuria and the patient was finally diagnosed with lupus nephritis. Urinary loss of thyroid hormones, fundamentally linked to their transport proteins, in patients affected by nephrotic syndrome is sometimes a forgotten phenomenon and one which should be considered in patients with increased levothyroxine requirements. In this report, we present the details of this case and a brief review of the literature on this topic.

Catéter peritoneal tunelizado para el manejo de ascitis refractaria en paciente con síndrome nefrótico

Introducción: la ascitis refractaria puede ser una complicación frecuente en el síndrome nefrótico (SN), existen casos reportados del uso de un catéter tunelizado de diálisis peritoneal en pacientes con cirrosis o neoplasias abdominales. Se presenta el caso de un paciente con SN en quién se utilizó un catéter para diálisis peritoneal (DP) para manejo de la ascitis refractaria. Objetivo: mostrar que el catéter peritoneal puede ser considerado como una alternativa para el manejo de la ascitis refractaria en pacientes con síndrome nefrótico. Presentación del caso: paciente varón de 19 años, sin antecedentes patológicos, cursó con edema progresivo y alteración de la función renal. Se evidenció síndrome nefrótico con anasarca y evolucionó con empeoramiento de la función renal ingresando a hemodiálisis de soporte. Se realizó biopsia renal: podocitopatía, glomerulopatía colapsante. Se inició tratamiento con corticoterapia, mejorando la función renal hasta suspender la hemodiálisis, pero presentó ascitis refractaria al tratamiento médico, por lo que se realizó paracentesis evacuatoria en reiteradas ocasiones. Se decidió colocación de catéter peritoneal tunelizado para el manejo de la ascitis refractaria. La ascitis fue disminuyendo progresivamente hasta el retiro del catéter peritoneal. Discusión y conclusión: el uso de catéter tunelizado de diálisis peritoneal es una opción de manejo efectiva en casos de síndrome nefrótico con ascitis refractaria.

Introduction: Refractory ascites can be a frequent complication in nephrotic syndrome (NS), there are reported cases of the use of a tunneled peritoneal dialysis catheter in patients with cirrhosis or abdominal neoplasms. The case of a patient with NS is presented in whom used a peritoneal dialysis (PD) catheter to manage refractory ascites. Purpose: To show that the peritoneal catheter can be considered as an alternative for the management of refractory ascites in patients with nephrotic syndrome. Presentation of the case: A 19-year-old male patient, with no pathological history, presented progressive edema and impaired renal function. Nephrotic syndrome with anasarca was evidenced, and it evolved with worsening renal function, entering supportive hemodialysis. Renal biopsy was performed: podocytopathy, collapsing glomerulopathy. Corticosteroid treatment was started, improving renal function until hemodialysis was discontinued, but he presented ascites refractory to medical treatment, for which evacuatory paracentesis was performed repeatedly. It was decided to place a tunneled peritoneal catheter for the management of refractory ascites. Ascites gradually decreased until the peritoneal catheter was removed. Discussion and conclusion: The use of a tunneled peritoneal dialysis catheter is an effective management option in cases of nephrotic syndrome with refractory ascites.

Síndrome nefrótico secundario a preeclampsia: presentación, manejo y evolución clínica observados en 5 años de experiencia / Nephrotic syndrome due to preeclampsia: Presentation, management and clinical evolution observed in 5 years experience

Introducción: El síndrome nefrótico (SN) es una entidad rara durante el embarazo. En esta etapa, la preeclampsia (PE) severa es la principal causa. Nuestro objetivo fue describir la presentación clínica, las características analíticas, el manejo médico y la evolución de mujeres con SN por PE. Materiales y métodos: Estudio descriptivo, retrospectivo, realizado entre el 1 de enero de 2017 y el 1 de enero de 2022 (5años). Incluyó mujeres con embarazo ≥20semanas de edad gestacional (EG), internadas por trastorno hipertensivo del embarazo (THE), sin evidencia de daño renal previo a la gestación. Resultados: Entre 652 THE se identificaron 452 PE y 21 SN. La edad materna fue de 25±5,7 años, y la EG al diagnóstico, de 33,1±5,1 semanas. Todas presentaron edema facial y periféricos: 5 derrame pleural, 3 derrame pericárdico y 2 anasarca. La P24 fue de 6,17±2,34g (3,10-10,8), la albúmina sérica de 2,5±0,27g/dl (2,10-2,90) y el colesterol sérico de 281,4±21,7mg/dl (251-316). Hubo 13 que desarrollaron complicaciones maternas: daño renal agudo, edema pulmonar, miocardiopatía dilatada, eclampsia y síndrome HELLP. Todas permanecieron hipertensas en el posparto, requiriendo combinación de dos a tres fármacos antihipertensivos. En el posparto todas recibieron estatinas e inhibidores de la enzima convertidora de angiotensina (IECA) para el manejo de la proteinuria; ninguna desarrolló hiperkalemia o elevaciones de creatinina. La estancia hospitalaria fue de 10,4±3,7 días. Todas revirtieron los parámetros proteinúricos de rango nefrótico antes del alta. No se registraron muertes. Conclusión: La presentación incluyó desde edemas periféricos hasta compromiso seroso. La severidad de la proteinuria fue variable. El uso de IECA no precipitó hiperkalemia ni fallo renal. Las complicaciones maternas fueron frecuentes, pero no se observaron óbitos. (AU)

Introduction: Nephrotic syndrome (NS) is rare during pregnancy. The main cause is severe pre-eclampsia (PR). Our aim was to describe the clinical presentation, analytical features, medical management, and progress of women with NS due to PE. Materials and methods: A descriptive, retrospective study, conducted from 01/01/2017 to 01/01/2022 (5years). Women with a gestational age (GA) ≥20weeks were included in the study, hospitalised due to hypertensive disorders in pregnancy (HDP), with no evidence of kidney damage prior to gestation. Results: Of the 652 HDP, 452 PE and 21 NS were identified. Maternal age was 25±5.7 years, GA at diagnosis was 33.1±5.1 weeks. All the women had facial and peripheral oedema: 5 pleural effusion, 3 pericardial effusion, and 2 anasarca. Their p24 was 6.17±2.34grams (3.10-10.8), serum albumin 2.5±0.27g/dL (2.10-2.90), and serum cholesterol 281.4±21.7mg/dL (251-316). Thirteen developed maternal complications: acute kidney damage, pulmonary oedema, dilated cardiomyopathy, eclampsia, and HELLP syndrome. They all remained hypertensive postpartum, and required a combination of two to three antihypertensive drugs. They all received statins postpartum, and angiotensin converting enzyme (ACE) inhibitors to manage proteinuria. None developed hyperkalaemia or creatinine elevation. Hospital stay was 10.4±3.7days. All nephrotic range proteinuria parameters reversed prior to discharge. No deaths were recorded. Conclusion: Presentation ranged from peripheral oedema to serous involvement. Severity of proteinuria varied. Use of ACE inhibitors did not precipitate hyperkalaemia or kidney failure. Maternal complications were frequent, but no deaths were recorded. (AU)

Neurorretinitis bilateral y nefritis lúpica membranosa: dos manifestaciones infrecuentes en el lupus juvenil / Bilateral neuroretinitis and membranous lupus nephritis: 2 infrequent manifestations in juvenile lupus

Joven de 15 años, previamente sana, se presentó con dolor abdominal, vómitos, diarrea, eritema malar, edema palpebral y en miembros inferiores, artralgias, rigidez matinal y visión borrosa bilateral. Estudios de laboratorio y por imágenes junto con la clínica permitieron realizar el diagnóstico de síndrome nefrótico secundario a lupus eritematoso sistémico. Al examen oftalmológico se constató 8/10 de visión en ambos ojos y edema de papila bilateral con estrella macular parcial, hallazgos compatibles con una neurorretinitis bilateral. La biopsia renal estableció el diagnóstico de nefritis lúpica membranosa. Se inició tratamiento inmunosupresor, con mejoría clínica gradual. Si bien el lupus eritematoso sistémico con nefritis lúpica membranosa y neurorretinitis es una asociación muy infrecuente, frente a un paciente con neurorretinitis bilateral debemos considerar el lupus eritematoso sistémico dentro de los diagnósticos diferenciales (AU)

Fifteen-year-old female patient, previously healthy, referred to our center for presenting abdominal pain, vomiting, diarrhea, malar erythema, palpebral and lower limb edema, arthralgia, morning stiffness and bilateral blurred vision. Laboratory and imaging studies together with the clinic allowed the diagnosis of nephrotic syndrome secondary to systemic lupus erythematosus. Ophthalmology examination revealed a visual acuity of 8/10 in both eyes and bilateral disc edema with partial macular star, findings compatible with bilateral neuroretinitis. Renal biopsy established the diagnosis of membranous lupus nephritis. Immunosuppressive treatment was started, obtaining gradual clinical improvement. Although systemic lupus erythematosus with membranous lupus nephritis and neuroretinitis is a very infrequent association, when faced with a patient with bilateral neuroretinitis, we must consider systemic lupus erythematosus within the differential diagnoses (AU)

Proteinuria y síndrome nefrótico / Proteinuria and Nephrotic Syndrome

Introducción: El síndrome nefrótico es una patología que afecta el complejo glomerular del riñón, se caracteriza por una proteinuria mayor 3500 mg/d. De acuerdo a la respuesta de los esteroides se puede clasificar en síndrome nefrótico en esteroide resistente o esteroide sensible. Objetivo: Determinar la relación que existe entre la proteinuria y las variantes del síndrome nefrótico en adultos. Métodos: Se realizó un estudio descriptivo, retrospectivo, tipo serie de casos, con una población de 28 pacientes. Se recolectaron y se procesaron los datos a través del software Epi-Info 7,2TM; la frecuencia simple, la media estadística, prueba t de Student, y el coeficiente de correlación de Pearson. Resultados: En el análisis combinatorio de los fármacos adyuvantes para síndrome nefrótico, el grupo que utilizó antiproteinúricos pero no estatinas, demostró una diferencia estadísticamente significativa entre la proteinuria postratamiento media del grupo de síndrome nefrótico esteroideo resistente (6202 mg/d) vs síndrome nefrótico esteroideo sensible (65,9 mg/d) (valor de p 0,418). Existe una correlación negativa entre los niveles proteinuria postratamiento y el nivel de albúmina sérica postratamiento (r = - 0,7 valor de p < 0,00001). Conclusiones: Se demostró la ausencia de asociación entre la proteinuria inicial y las variantes de síndrome nefrótico esteroide sensible y esteroide resistente (valor de p = 0,8)(AU)

Introduction: Nephrotic syndrome is a pathology that affects the glomerular complex of the kidney, characterized by proteinuria greater than 3500 mg/d. According to the response to steroids, nephrotic syndrome can be classified as steroid-resistant or steroid-sensitive. Objective: To determine the relationship between proteinuria and the variants of the nephrotic syndrome in adults. Methods: A descriptive, retrospective, case series type study was carried out with a population of 28 patients. The data was collected and processed through Epi-Info 7.2TM software; simple frequency, statistical mean, student's t-test, and Pearson's correlation coefficient. Results: The statistically significant difference was obtained in the antiproteinuric and non-statin group, between the mean post-treatment proteinuria of the steroid resistant nephrotic syndrome group (6202 mg/d) in comparison to steroid sensitive nephrotic syndrome (65.9 mg/d) (p value 0.0418). There is negative correlation between post-treatment proteinuria levels and post-treatment serum albumin level (r= -0.7 p value <0.00001). Conclusions: The absence of association between initial proteinuria and steroid-sensitive and steroid-resistant variants of nephrotic syndrome was demonstrated (p value=0.8)(AU)

Bilateral neuroretinitis and membranous lupus nephritis: 2 infrequent manifestations in juvenile lupus.

Fifteen-year-old female patient, previously healthy, referred to our center for presenting abdominal pain, vomiting, diarrhea, malar erythema, palpebral and lower limb edema, arthralgia, morning stiffness and bilateral blurred vision. Laboratory and imaging studies together with the clinic allowed the diagnosis of nephrotic syndrome secondary to systemic lupus erythematosus. Ophthalmology examination revealed a visual acuity of 8/10 in both eyes and bilateral disc edema with partial macular star, findings compatible with bilateral neuroretinitis. Renal biopsy established the diagnosis of membranous lupus nephritis. Immunosuppressive treatment was started, obtaining gradual clinical improvement. Although systemic lupus erythematosus with membranous lupus nephritis and neuroretinitis is a very infrequent association, when faced with a patient with bilateral neuroretinitis, we must consider systemic lupus erythematosus within the differential diagnoses.

Documento de consenso del Grupo de Estudio de Enfermedades Glomerulares de la Sociedad Española de Nefrología (GLOSEN) para el diagnóstico y tratamiento de la nefritis lúpica / Consensus document of the Spanish Group for the Study of the Glomerular Diseases (GLOSEN) for the diagnosis and treatment of lupus nephritis

Un número importante de pacientes con lupus eritematoso sistémico (entre 20 a 60%, según diferentes series) desarrolla nefritis lúpica en el curso de su evolución, lo que influye directamente en su calidad de vida y pronóstico vital. En años recientes, el mayor conocimiento sobre la patogénesis del lupus sistémico y de la nefritis lúpica ha permitido avances relevantes en el abordaje diagnóstico y en el tratamiento de estos pacientes, lográndose desarrollar fármacos dirigidos específicamente a bloquear vías patogénicas claves de la enfermedad. Alentadoramente estos agentes inmunomoduladores han demostrado en ensayos clínicos aleatorizados, y bien ponderados, buena eficacia clínica a mediano plazo, definida como remisión de proteinuria y preservación de la función renal, con un aceptable perfil de seguridad y buena tolerabilidad del paciente. Todo esto ha permitido reducir el uso de corticoides y de otras terapias potencialmente más tóxicas, así como incrementar el uso de terapias combinadas. El presento documento de consenso realizado por el Grupo de Trabajo de Enfermedades Glomerulares de la Sociedad Española de Nefrología (GLOSEN) recoge de manera práctica y resumida, pero rigurosa, la mejor evidencia actual disponible acerca del diagnóstico, tratamiento y seguimiento del paciente con nefritis lúpica, incluyendo casos de situaciones especiales, con el objetivo principal de brindar información actualizada y recomendaciones clínicas bien fundamentadas a los médicos tratantes, para mejorar el enfoque diagnóstico y terapéutico a nuestro pacientes. (AU)

A significant number of patients with systemic lupus erythematosus (between 20% to 60% according to different reported series) develop lupus nephritis in the course of its evolution, which directly influences their quality of life and vital prognosis. In recent years, the greater knowledge about the pathogenesis of systemic lupus and lupus nephritis has allowed relevant advances in the diagnostic approach and treatment of these patients, achieving the development of drugs specifically aimed at blocking key pathogenic pathways of the disease. Encouragingly, these immunomodulatory agents have shown in well-powered, randomized clinical trials good clinical efficacy in the medium-term, defined as proteinuria remission and preservation of kidney function, with an acceptable safety profile and good patient tolerability. All this has made it possible to reduce the use of corticosteroids and other potentially more toxic therapies, as well as to increase the use of combined therapies. The present consensus document carried out by the Glomerular Diseases Working Group of the Spanish Society of Nephrology (GLOSEN), collects in a practical and summarized, but rigorous way, the best currently available evidence about the diagnosis, treatment, and follow-up of lupus nephritis patients, including cases of special situations, with the main objective of providing updated information and well-founded clinical recommendations to treating physicians, to improve the diagnostic and therapeutic approach to our patients. (AU)

[Nephrotic syndrome due to preeclampsia: Presentation, management and clinical evolution observed in 5 years experience]. / Síndrome nefrótico secundario a preeclampsia: presentación, manejo y evolución clínica observados en 5 años de experiencia.

INTRODUCTION: Nephrotic syndrome (NS) is rare during pregnancy. The main cause is severe pre-eclampsia (PR). Our aim was to describe the clinical presentation, analytical features, medical management, and progress of women with NS due to PE. MATERIALS AND METHODS: A descriptive, retrospective study, conducted from 01/01/2017 to 01/01/2022 (5years). Women with a gestational age (GA) ≥20weeks were included in the study, hospitalised due to hypertensive disorders in pregnancy (HDP), with no evidence of kidney damage prior to gestation. RESULTS: Of the 652 HDP, 452 PE and 21 NS were identified. Maternal age was 25±5.7 years, GA at diagnosis was 33.1±5.1 weeks. All the women had facial and peripheral oedema: 5 pleural effusion, 3 pericardial effusion, and 2 anasarca. Their p24 was 6.17±2.34grams (3.10-10.8), serum albumin 2.5±0.27g/dL (2.10-2.90), and serum cholesterol 281.4±21.7mg/dL (251-316). Thirteen developed maternal complications: acute kidney damage, pulmonary oedema, dilated cardiomyopathy, eclampsia, and HELLP syndrome. They all remained hypertensive postpartum, and required a combination of two to three antihypertensive drugs. They all received statins postpartum, and angiotensin converting enzyme (ACE) inhibitors to manage proteinuria. None developed hyperkalaemia or creatinine elevation. Hospital stay was 10.4±3.7days. All nephrotic range proteinuria parameters reversed prior to discharge. No deaths were recorded. CONCLUSION: Presentation ranged from peripheral oedema to serous involvement. Severity of proteinuria varied. Use of ACE inhibitors did not precipitate hyperkalaemia or kidney failure. Maternal complications were frequent, but no deaths were recorded.

Síndrome nefrótico por enfermedad de cambios mínimos asociado a enfermedad de Graves y uso de metimazol

Introduction: Graves' disease causes kidney injury through a series of multiple mechanisms, including the treatment for this condition. Nephrotic syndrome due to minimal change disease (MCD) is an unusual form of such kidney injury; the association between methimazole use and MCD is also rare. Case presentation: A 36-year-old woman with a history of Graves' disease in use of methimazole for several months, who presents edematous syndrome due to nephrotic syndrome associated with a KDIGO stage 3 acute kidney injury. Thionamide-induced hypothyroidism and the need of thyroid hormone replacement therapy was evidenced at the time of consultation. Based on a renal biopsy, the patient was diagnosed with MCD, her condition worsened as she experienced oliguria and hypervolemia, therefore, renal replacement therapy with hemodialysis was temporarily required. Methimazole administration was suspended, and treatment consisting of prednisolone administration and levothyroxine supplementation was started, achieving hemodialysis suspension, gradual improvement of proteinuria until remission and a full-maintained recovery of renal clearance. Radioiodine therapy was implemented as definitive treatment for Graves' disease, obtaining a successful outcome. Conclusions: Graves' disease and methimazole use are possible causes of minimal change disease; systemic corticosteroid therapy is a possible management. However, further basic, clinical and epidemiological research on this subject is required.

Introducción: La enfermedad de Graves genera daño renal por múltiples mecanismos, incluyendo sus tratamientos. El síndrome nefrótico por enfermedad de cambios mínimos, es una forma inusual de dicho daño renal en la enfermedad de Graves; la asociación de esta condición renal con el metimazol también es anómalo. Presentación del caso: Una mujer de 36 años con antecedente de enfermedad de Graves en uso de metimazol hacía meses, quien debutó con síndrome edematoso por síndrome nefrótico asociado a una lesión renal aguda KDIGO etapa 3. Se evidenció hipotiroidismo inducido por la tionamida con necesidad de suplencia al momento de la consulta. Asimismo, se realizó biopsia renal que concluyó en enfermedad de cambios mínimos. La paciente progresó a oliguria con hipervolemia sin respuesta a diurético del asa por lo que requirió terapia de reemplazo renal con hemodiálisis de forma transitoria. Se retiró el metimazol, se dio manejo con prednisolona y con suplencia tiroidea, lográndose el retiro de la hemodiálisis con mejoría gradual de la proteinuria hasta la remisión y recuperación de la depuración renal de forma plena y mantenida. Se dio manejo definitivo a la enfermedad de Graves con yodo radioactivo con éxito terapéutico. Discusión y conclusiones: La enfermedad de Graves y el metimazol son causas posibles de enfermedad de cambios mínimos; el tratamiento con corticoide sistémico se postula como una posible estrategia de manejo. Se requiere más investigación básica, clínica y epidemiológica en el tema.

Síndrome nefrótico como manifestación de microangiopatía trombótica secundaria al uso crónico de sunitinib / Nephrotic syndrome as a manifestation of thrombotic microangiopathy due to long-term use of sunitinib

El síndrome nefrótico en los pacientes con cáncer se puede asociar a su enfermedad de base o al tratamiento quimioterapéutico. El cáncer de órganos sólidos puede producir una glomerulonefritis membranosa que se manifiesta con síndrome nefrótico; otras presentaciones histológicas menos frecuentes son la glomeruloesclerosis focal y segmentaria y la enfermedad de cambios mínimos. Adicionalmente, los tratamientos quimioterapéuticos pueden causar toxicidad renal por afección de los pequeños vasos sanguíneos, los glomérulos, los túbulos y el intersticio. Los inhibidores de la tirosina quinasa como el sunitinib pueden causar daño endotelial y podocitario, produciendo una microangiopatía trombótica limitada a los riñones, que se manifiesta con proteinuria e hipertensión. Se presenta el caso de un hombre anciano con tumor del estroma gastrointestinal (GIST, por sus siglas en inglés) que fue tratado con sunitinib y como complicación presentó una microangiopatía trombótica manifestada con síndrome nefrótico e hipertensión de difícil control, que se controló al suspender este medicamento pero con desenlace fatal por su neoplasia maligna. (AU)

Nephrotic syndrome in patients with cancer may be related to the primary malignancy or chemotherapeutic therapy. Solid organ cancers may cause membranous glomerulonephritis manifesting with nephrotic syndrome; other less common histologic presentations include focal and segmental glomerulosclerosis and minimal change disease. In addition, chemotherapy agents can cause renal toxicity by affecting the small blood vessels, glomeruli, tubules, and interstitium. Tyrosine kinase inhibitors such as sunitinib may cause endothelial and podocyte damage leading to renal limited thrombotic microangiopathy, manifested by proteinuria and hypertension. We report a case of an elderly man with gastrointestinal stromal tumor (GIST) on treatment with sunitinib who had as a complication of a thrombotic microangiopathy manifested with nephrotic syndrome and difficult-to-control hypertension, which was controlled by stopping this drug but with a fatal outcome due to its malignant neoplasm. (AU)

Diagnóstico de amiloidosis renal AA mediante espectrometría de masas en un paciente con proceso inflamatorio crónico grave abdominal

Introducción la amiloidosis es una enfermedad rara, producto del plegamiento y depósito normal de proteínas en tejidos y órganos. Esta enfermedad puede tener un compromiso renal que se manifiesta con síndrome nefrótico y deterioro de la función renal y su etiología puede estar asociada a amiloidosis con compromiso sistémico, siendo la amiloidosis AL y la amiloidosis AA las más frecuentes, esta última está asociada a inflamación crónica grave de origen infecciosa o autoinmune. Para el diagnóstico es fundamental el estudio sistémico multidisciplinario (hematológico, cardiaco, autoinmune, infeccioso y neoplásico), y cuando hay compromiso renal: la biopsia con estudio completo de microscopía de luz, tinciones especiales incluyendo rojo congo, inmunofluorescencia y microscopía electrónica. Cuando no se logra establecer la causa, la espectrometría de masas es una ayuda crucial para el diagnóstico específico. Objetivo se presenta el caso de un paciente con un proceso inflamatorio crónico grave abdominal que evolucionó a síndrome nefrótico por amiloidosis AA, donde la espectrometría de masas ayudó a aclarar el diagnóstico. Presentación del caso se presenta el caso de un paciente con un proceso inflamatorio crónico grave abdominal que evolucionó a síndrome nefrótico por amiloidosis AA, donde la espectrometría de masas ayudó a aclarar el diagnóstico Discusión y conclusiones se considera que la espectrometría de masas es un estudio diagnóstico muy importante para establecer el diagnóstico etiológico de la amiloidosis cuando otros métodos no han logrado establecerlo.

Introduction Amyloidosis is a rare disease, resulting from the accumulation and deposition of insoluble proteins in tissues or organs. This disease may involve the kidney, resulting in nephrotic syndrome and renal failure. The amyloidosis has been associated with systemic involvement, with AL amyloidosis and AA amyloidosis being the most common. The last is associated with various inflammatory disorders as chronic infections and autoimmune diseases. A multidisciplinary approach is required to the diagnosis (hematologic, cardiac, autoimmune, infectious, neoplastic) and in cases of renal involvement, a kidney biopsy with complete study of light microscopy, special stains including congo red, immunofluorescence, electron microscopy is essential for diagnosis. In cases where the cause cannot be stablished, mass spectrometry is practical tool to the identification of the correct type of amyloidosis. Purpose Here, we present a patient with a chronic and severe abdominal inflammatory process that progressed to a nephrotic syndrome due to AA amyloidosis, in which mass spectrometry helped to clarify the diagnosis. Case presentation Here, we present a patient with a chronic and severe abdominal inflammatory process that progressed to a nephrotic syndrome due to AA amyloidosis, in which mass spectrometry helped to clarify the diagnosis Discussion and conclusion Mass spectrometry is considered a useful diagnostic test to confirm the etiology of amyloidosis, especially if other methods are insufficient to establish it.

Clinical and histopathological characteristics of diabetic patients with nephrotic proteinuria. A case series / Características clínicas e histopatológicas de pacientes diabéticos con proteinuria nefrótica. Una serie de casos

Abstract Objective: to describe the clinical and histopathological characteristics of diabetic patients with nephrotic-range proteinuria. Materials and methods: the kidney biopsies of diabetic patients with nephrotic proteinuria were reviewed. Descriptive analyses were performed along with a comparison of three groups according to the histopathological findings. Results: the medical charts of 19 patients from 2018 through 2020 were collected, most of whom (94.7%) were diagnosed with type 2 diabetes mellitus (DM), with an average age of 58 years, and an average duration of DM of 9.9 years (SD: ±7.3). The findings from biopsies performed throughout the years prior to data collection showed that 26.3% had diabetic nephropathy as the only finding, 31.6% had a nephropathy other than diabetic nephropathy, and 42.1% had findings of both diabetic and nondiabetic nephropathy. A comparison of the groups showed a significant difference in the duration of DM, which was greater in patients with diabetic nephropathy (16.4 vs. 5 vs. 9.5 years, respectively, p: 0.024). Conclusions: we present a case series of diabetic patients with nephrotic-range proteinuria in Colombia, showing that kidney biopsy lesions other than diabetic nephropathy may be a cause of proteinuria. We found that patients with a report of DN alone had a much longer duration of diabetes. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2231).

Resumen Objetivo: describir las características clínicas e histopatológicas de pacientes diabéticos con proteinuria en rango nefrótico. Material y métodos: se revisaron biopsias renales de pacientes diabéticos con proteinuria ne frótica, se realizaron análisis descriptivos y comparación entre tres grupos de acuerdo con hallazgos histopatológicos. Resultados: se recolectaron historias de 19 pacientes, entre los años 2018 y 2020, la mayoría (94.7%) con diagnóstico de diabetes mellitus (DM) tipo 2, edad promedio de 58 años, con un tiempo de evolución de la DM en promedio de 9.9 años (DE: ±7.3). En los hallazgos de la biopsia, practica das a lo largo de años anteriores a la recolección, se encontró que 26.3% tenían nefropatía diabética como único hallazgo, el 31.6% otra nefropatía diferente a la diabética y 42.1% hallazgos tanto de nefropatía diabética como no diabética. Al comparar los grupos se encontró diferencia significativa en el tiempo de evolución de la DM, siendo mayor en pacientes con nefropatía diabética (16.4 vs 5 vs 9.5 años respectivamente, p: 0.024). Conclusiones: se presenta una serie de casos de pacientes diabéticos con proteinuria en rango nefrótico en Colombia, mostrando que existen lesiones diferentes a la nefropatía diabética en la biopsia renal como posible causa de la proteinuria. Se encontró que los pacientes en quienes se reportó ND únicamente, tenían un tiempo de evolución de la diabetes mucho mayor. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2231).

Glomerulonefritis C3 en un paciente con síndrome de Alport: un caso raro

El síndrome de Alport es una entidad hereditaria que se produce principalmente por una mutación en los genes que codifican el colágeno tipo IV. Por otro lado, la glomerulonefritis C3 es una entidad rara que presenta un patrón de glomerulonefritis membranoproliferativa y su etiología se basa en un control anormal de la activación de la vía alternativa del complemento. A continuación se describe un caso de un paciente, de sexo masculino, que cursa con un síndrome nefrótico corticorresistente en el que se documenta un patrón de glomerulonefritis membranoproliferativa en la biopsia renal con depósitos de C3 en la inmunofluorescencia, asociada a una deleción heterocigota en el gen CFHR1 en el estudio genético de las proteínas reguladoras del complemento. Además, en el panel genético realizado por corticorresistencia se encuentra una variante COL4A5 asociada al síndrome de Alport ligado al X. Estas entidades pueden presentarse con un curso clínico diverso, pero al estar asociadas pueden acelerar la progresión a enfermedad renal crónica, por lo que se hace necesario hacer un seguimiento clínico más estricto.

Alport Syndrome is a hereditary entity that occurs mainly due to a mutation in the genes that encode type IV collagen. C3 glomerulonephritis is a rare entity with a pattern of membranoproliferative glomerulonephritis and its etiology is based on abnormal control of the activation of the alternative complement pathway. We describe a case of a male patient who presents with a corticosteroid nephrotic syndrome in which a pattern of membranoproliferative glomerulonephritis is documented in the renal biopsy with C3 deposits in the immunofluorescence, associated with a heterozygous deletion in the gene CFHR1 in the genetic study of complement regulatory proteins. Furthermore, a variant COL4A5 associated with X-linked Alport syndrome is found in the genetic panel for corticosteroid resistance. These entities can present with a diverse clinical course, but when associated they can accelerate progression to chronic kidney disease, which is why makes it necessary to do a more strict clinical follow-up.

Enfermedad de cambios mínimos cortico-resistente e hipotiroidismo. Reporte de caso / Steroid-resistant minimal change disease and hypothyroidism: Case report

Introducción: La enfermedad de cambios mínimos es una causa rara de síndrome nefrótico en el adulto, y su relación con el hipotiroidismo es más rara aún. Se considera que esta patología renal responde favorablemente al manejo con glucocorticoides y tiene una baja frecuencia de resistencia. Su abordaje hoy en día es objeto de investigación. Objetivo: Describir una rara etiología de síndrome nefrótico en el adulto con presentación, tratamiento y desenlace infrecuentes. Presentación del caso: Paciente femenino de 53 años quien inicia con síndrome nefrótico por enfermedad de cambios mínimos cortico-resistente y su asociación con un hipotiroidismo descontrolado, quien requiere manejo con rituximab y control de la enfermedad endocrinológica asociada, como enfermedad de base. Conclusiones: En este caso clínico se muestra como las enfermedades citadas pueden coexistir y el tratamiento en conjunto es necesario. El establecimiento de nuevas terapias en la población adulta como el rituximab podría mostrar beneficio, como en este caso. Sin embargo, aún existe la necesidad de estudios de mayor evidencia que validen firmemente la efectividad de los diferentes tratamientos en este tipo de pacientes.

Introduction: Minimal change disease is a rare cause of nephrotic syndrome in adults, and its association with hypothyroidism is even more exceptional. This renal pathology is considered to respond favorably to glucocorticoid management and has a low resistance frequency. How to approach this disease is currently under investigation. Objective: To describe a rare etiology of adult nephrotic syndrome with unusual presentation, treatment, and outcome. Case presentation: A 53 years-old female patient who initially experienced nephrotic syndrome due to steroid-resistant minimal change disease, which was also associated with uncontrolled hypothyroidism. She required management with rituximab and control of the associated endocrinological disease, which was considered as the underlying disease. Conclusions: This clinical case shows (i) how the two aforementioned diseases can coexist and (ii) that a joint treatment is necessary. Establishing new therapies may be beneficial for adult populations, such as the benefits seen in this case with the use of rituximab. However, further studies are needed to strongly validate the effectiveness of the different treatments for these types of patients.

Glomerulonefritis por cambios mínimos post vacuna COVID-19 AstraZeneca / Glomerulonephritis due to minimal change disease post COVID-19 vaccine AstraZeneca

RESUMEN Reportamos un caso de una mujer de 28 años, con síndrome nefrótico debido a glomerulonefritis por cambios mínimos 48horas después de la administración de la vacuna contra SARS-CoV2 de AstraZeneca. La paciente tuvo síndrome nefrótico idiopático en la infancia tratado empíricamente con corticoides y ciclosporina, en remisión completa desde los 9 años. Algunos reportes sugieren que determinadas enfermedades glomerulares podrían asociarse infrecuentemente a las vacunas.

ABSTRACT We report a case of a 28-year-old woman with minimal change disease secondary nephrotic syndrome 48 hours after the administration of the AstraZeneca SARS-CoV2 vaccine. The patient had suffered idiopathic nephrotic syndrome in childhood treated empirically with corticosteroids and cyclosporine, in complete remission from the age of 9. Some reports suggest that glomerular disease might appear infrequently associated to some vaccines.

Rituximab treatment in pediatric patients with steroid-dependent nephrotic syndrome: A tertiary hospital.

BACKGROUND: Corticosteroids have had a central role in the treatment of nephrotic syndrome. The management of these patients who become dependent to steroids is complex, involving different immunosuppressive drugs patterns. The monoclonal antibody anti CD20, Rituximab, is likely to have beneficial effects in cases of steroid-dependent nephrotic syndrome patients with no easy resolution, even when we cannot make a statement about the specific role in the impact. We bring our personal experience in pediatric patients treated with this medication during the last years, to provide a thorough overview and useful information about the role of Rituximab in this pathology. METHODS: Retrospective study in patients with steroid-dependent idiopathic nephrotic syndrome controlled in the division of Pediatric Nephrology of a Spanish tertiary hospital in those patients who had received at least one treatment cycle of Rituximab, at any moment along the evolution of the disease. RESULTS: The study involved 8 patients. All of them previously received immunosuppressive therapy. The Rituximab were administered as an intravenous infusion, in a dose of 375 mg/m2, and all doses were administered in a period during which the disease was in remission. The depletion of lymphocytes B (CD19, 0%) were confirmed after the first dose of Rituximab except for one, with a lymphocyte count of 1%. The period of depletion lasts 10,3 months (median; range 6,5-16 months), and only one of the patients registered a relapse of the disease in this period. A reduction of relapses suffered by patients has been shown after the treatment began (3,6 relapses/year in the previous year to the start of the treatment versus 0,1 relapses/year during the first year post-rituximab). The relapse-free survival in the first year reached 83,3% in patients who suffered more than one relapse (75% of patients), and without a relapse after the treatment began in 2 cases. One or more drugs could be removed in 87,5% of patients after the first cycle of rituximab. After the rituximab treatment, we reached a 96,5% decrease in the corticosteroids doses administered (28,5 mg/m2/day during the 3 months pre-treatment versus 1 mg/m2/day in the last 3 months of patient monitoring). Not a significant observed adverse effect attributed to the drug after the post-rituximab monitoring period (median 46,5 months, range 5-97 months). CONCLUSION: The favorable results reported after rituximab treatment in our patients seems to confirm the effectiveness of this drug in the steroid-dependent nephrotic syndrome, making that therapeutic option into consideration and legitimating the use of the drug in complex cases involving pediatric patients. Even so, it seems recommendable to design pertinent studies to clarify, among others, the optimum regimen of the treatment (dose, interval and cycles), clinical repercussion and potential adverse effects in long terms.

Tratamiento con rituximab en pacientes pediátricos con síndrome nefrótico córtico-dependiente. Experiencia en un hospital terciario / Rituximab treatment in pediatric patients with steroid-dependent nephrotic syndrome: a tertiary hospital

Introducción: La corticoterapia continúa siendo la piedra angular en el tratamiento del síndrome nefrótico. El manejo de los pacientes que desarrollan dependencia a esteroides es complejo, implicando distintas pautas de fármacos inmunosupresores. El rituximab, anticuerpo monoclonal anti-CD20, parece tener efectos beneficiosos en pacientes con síndrome nefrótico córtico-dependiente de difícil manejo clínico, si bien aún no está bien definido su papel en esta entidad. Con el fin de aportar información útil sobre el papel del rituximab en esta patología, presentamos nuestra experiencia personal en pacientes pediátricos tratados con este fármaco en los últimos años. Materiales y métodos: Estudio retrospectivo en pacientes con síndrome nefrótico idiopático córtico-dependiente controlados en la Sección de Nefrología Pediátrica de un hospital terciario español, y que habían recibido, al menos, un ciclo de tratamiento con rituximab durante cualquier momento de la evolución de la enfermedad. (AU)

Background: Corticosteroids have had a central role in the treatment of nephrotic syndrome. The management of these patients who become dependent to steroids is complex, involving different immunosuppressive drugs patterns. The monoclonal antibody anti CD20, Rituximab, is likely to have beneficial effects in cases of steroid-dependent nephrotic syndrome patients with no easy resolution, even when we cannot make a statement about the specific role in the impact. We bring our personal experience in pediatric patients treated with this medication during the last years, to provide a thorough overview and useful information about the role of Rituximab in this pathology. Methods: Retrospective study in patients with steroid-dependent idiopathic nephrotic syndrome controlled in the division of Pediatric Nephrology of a spanish tertiary hospital in those patients who had received at least one treatment cycle of Rituximab, at any moment along the evolution of the disease. (AU)

Nephrotic syndrome as a manifestation of thrombotic microangiopathy due to long-term use of sunitinib.

Nephrotic syndrome in patients with cancer may be related to the primary malignancy or chemotherapeutic therapy. Solid organ cancers may cause membranous glomerulonephritis which is manifested by nephrotic syndrome; other less common histologic presentations include focal and segmental glomerulosclerosis and minimal change disease. In addition, chemotherapy agents may cause renal toxicity by affecting the small blood vessels, glomeruli, tubules, and interstitium. Tyrosine kinase inhibitors such as sunitinib may cause endothelial and podocyte damage leading to thrombotic microangiopathy affecting only the kidney and manifested by proteinuria and hypertension. We report a case of an elderly man with gastrointestinal stromal tumor (GIST) on treatment with sunitinib who had as a complication a thrombotic microangiopathy manifested with nephrotic syndrome and a hypertension of difficult control, which was finally controlled by stopping this drug but had a fatal outcome due to its malignancy.

Síndrome nefrótico de inicio asociado a infección por SARS-COV-2 en un niño

Introducción: La infección por el SARS-COV-2 afecta en su evolución a diferentes órganos, entre ellos, el riñón. Objetivo: Examinar el inusual caso de un paciente afectado por COVID-19, que exhibió un cuadro clínico y humoral compatible con síndrome nefrótico. Presentación del caso: Paciente de un año con antecedente de buena salud, sin enfermedad renal previa, remitido al Hospital Pediátrico Cerro por manifestaciones respiratorias, edema marcado y test de antígeno positivo al SARS-COV-S. Al ingreso se constató un peso 14 kg, anasarca, tensión arterial de 100/60 mm Hg. En los análisis complementarios se comprobó PCR positivo al SARS-COV-2, hipoproteinemia, proteinuria e hipercolesterolemia. Ultrasonido abdominal y torácico mostraron ascitis y derrame pleural. Conclusiones: Paciente preescolar, ingresado por síndrome respiratorio e hidropígeno y PCR positivo al SARS-COV-2. Muestra los indicadores clínicos y humorales compatibles con síndrome nefrótico, con óptima respuesta al tratamiento esteroideo. Este caso podría constituir una coincidencia o una inusual forma de presentación de COVID-19 y ayudar, por tanto, a un mejor conocimiento del síndrome y del cuadro clínico inicial que pudiera originar este nuevo morbo.

Introduction: SARS-COV-2 infection affects different organs in its evolution, including the kidney. Objective: To examine the unusual case of a patient affected by COVID-19, who showed a clinical and humoral picture compatible with nephrotic syndrome. Case presentation: One-year-old patient with a history of good health, without previous kidney disease, referred to Cerro Pediatric Hospital due to respiratory manifestations, marked edema and positive to SARS-COV-S antigen test. On admission, a weight of 14 kg, anasarca, blood pressure of 100/60 mmHg were observed. In the complementary tests, positive PCR to SARS-COV-2, hypoproteinemia, proteinuria and hypercholesterolemia were verified. Abdominal and thoracic ultrasound showed ascites and pleural effusion. Conclusions: Preschool patient, admitted due to respiratory and hydropygenic syndrome and positive PCR to SARS-COV-2. The patient showed the clinical and humoral indicators compatible with nephrotic syndrome, with optimal response to steroid treatment. This case could constitute a coincidence or an unusual form of presentation of COVID-19 and therefore help a better knowledge of the syndrome and the initial clinical picture that could cause this new morbidity.

Endothelin-1 RS5370 gene polymorphism in primary nephrotic syndrome: A case-control study.

INTRODUCTION: Primary nephrotic syndrome (NS) is a common glomerular disease in children. We assessed the genotypes and frequency of the rs5370 allelic variant of the EDN1 gene in children with primary NS. PATIENTS AND METHODS: We conducted a case-control study in Mansoura University Children's Hospital, Egypt between December 2015 and January 2018. We recruited 50 patients with steroid-sensitive NS (SSNS) and 50 patients with steroid-resistant NS (SRNS) in addition to 100 healthy controls. The patients underwent clinical evaluations and tests including measurement of serum albumin, cholesterol, creatinine and urea levels and a 24-h urinary protein test. We used polymerase chain reaction methods to assess the genotypes of rs5370 variants of the EDN1 gene (GG, GT and TT) and alleles (T and G) in the groups under study. RESULTS: The most frequent genotype of the EDN1 gene at the locus of interest in the control group was the GT genotype (88%; P=.001) while the GG genotype was more frequent in the NS group compared to the control group (P=.02). We did not find statistically significant differences between the NS and control groups in regard to the EDN1 rs5370 alleles (P=.69). The GG genotype was more frequent in the SSNS group compared to the SRNS and control groups (P=.03). When we compared allele frequencies between the control, SSNS and SRNS groups, we did not find significant differences (P=.89). The GT genotype was associated with normal blood pressure in children with NS (P=.007), while the GG genotype was associated with hypertension (P<.001). We did not find statistically significant differences in renal histopathology or serum cholesterol levels based on the genotype. CONCLUSIONS: The GG genotype at the rs5370 locus of the EDN1 gene may be associated with an increased risk of primary NS and a better response to steroid therapy.