COVID-19, glomerulonefritis ANCA y anti-membrana basal glomerular. Caso clínico / Association of COVID-19 and glomerulonephritis with ANCA and anti-glomerular basement membrane antibodies. Report of one case

Renal involvement in COVID-19 infection is varied and worsens its outcome and prognosis. However, the association of COVID-19 infection with glomerulonephritis is exceptional. We report a 46-year-old woman with COVID-19 who had an acute kidney injury and ANCA associated glomerulonephritis two weeks after the onset of the disease. The kidney biopsy showed a crescentic glomerulo-nephritis and the presence of anti-glomerular basement membrane antibodies (GBM-Abs). She was treated with steroids and oral cyclophosphamide with good response without requiring plasmapheresis. Plasma anti GBM-Abs were negative. This case suggests that the presence of anti-GBM-Abs in the kidney, was temporally related to COVID-19 pulmonary damage. The absence of plasma antibodies is probably due to transient production and glomerular adsorption, but with unknown pathogenic role.

Anca negative pauci-immune crescentic glomerulonephritis and mixed connective tissue disease: a case study / Glomerulonefrite crescêntica pauci-imune ANCA-negativa e doença mista do tecido conjuntivo: relato de um caso

Abstract One of the most common causes of rapidly progressive glomerulonephritis (RPGN) is pauci-immune crescentic glomerulonephritis (CrGN). In the majority of cases, this condition has a positive serologic marker, the anti-neutrophil cytoplasmic antibodies (ANCAs), but in approximately 10% there are no circulating ANCAs, and this subgroup has been known as the ANCA-negative pauci-immune CrGN. RPGN can be associated with systemic diseases, but there are only few case reports describing the association with mixed connective tissue disease (MCTD). The authors report a case of ANCA-negative CrGN associated with a MCTD.

Resumo Uma das causas mais comuns da glomerulonefrite rapidamente progressiva (GNRP) é a glomerulonefrite crescêntica (GNC) pauci-imune. Na maioria dos casos, a patologia apresenta um marcador sorológico positivo, o anticorpo anticitoplasma de neutrófilos (ANCA), mas em cerca de 10% dos pacientes não há ANCAs circulantes, perfazendo um subgrupo da patologia conhecido como GNC pauci-imune ANCA-negativa. A GNRP pode estar associada a doenças sistêmicas, mas são poucos os relatos de caso que descrevem sua associação com doença mista do tecido conjuntivo (DMTC). O presente artigo relata um caso de GNC ANCA-negativa associada a DMTC.

Endocarditis por bartonella asociada a glomerulonefritis y neurorretinitis / Bartonella endocarditis associated with glomerulonephritis and neuroretinitis

La endocarditis bacteriana con hemocultivo negativo constituye un dilema diagnóstico. Tanto Bartonella como Coxiella pueden causarla, con presentaciones clínicas similares que pueden simular una vasculitis sistémica no infecciosa. Sin embargo, difieren en el tipo y la duración del tratamiento, por lo que es fundamental identificar el agente etiológico. Presentamos un caso de endocarditis por Bartonella henselae asociada a glomerulonefritis y neurorretinitis, con hemocultivo negativo, anticuerpos anticitoplasma de neutrófilos y antiproteinasa 3 positivos, y serología positiva para Bartonella con reacción cruzada para Coxiella burnetti. El diagnóstico etiológico fue confirmado a posteriori mediante amplificación y secuenciación parcial del gen ribC a partir de tejido de la válvula cardíaca. El paciente recibió tratamiento antibiótico e inmunosupresor seguido de recambio valvular aórtico y presentó evolución favorable.

Blood-culture negative endocarditis is a diagnostic challenge. Both Bartonella and Coxiella can cause it with similar clinical presentations mimicking a systemic vasculitis. The identification of the etiologic agent is essential because they differ in treatment type and duration. We present a case of blood-culture negative endocarditis caused by Bartonella henselae, associated with glomerulonephritis and neuroretinitis, with negative blood culture, positive anti-neutrophil cytoplasmic and antiproteinase 3 antibodies. The serology was positive for Bartonella with cross-reactivity to Coxiella burnetti. The etiological diagnosis was achieved by polymerase chain reaction amplification and sequencing of a ribC gene fragment. The patient received antibiotic and immunosuppressive treatment followed by replacement of the aortic valve with favorable medium-term evolution.

Trastornos glomerulares en la práctica médica / Glomerulopathies in the medical practice

RESUMEN Las glomerulopatías agrupan varias nefropatías con lesiones fundamentalmente del corpúsculo renal y que se expresan principalmente por proteinuria, hematuria, edemas e hipertensión arterial. La presentación clínica varía en dependencia del tipo de enfermedad de que se trate. Constituye la causa más frecuente de enfermedad renal crónica en adultos jóvenes, por lo que su estudio resulta imprescindible sobre todo para el nivel primario de salud. El propósito fue actualizar consideraciones pertinentes sobre la conducta diagnóstica y terapéutica integral ante una glomerulopatía y valorar emisión de recomendaciones al respecto. Se realizó una búsqueda, análisis y síntesis de información a través de Bases de datos ScieLO Cuba, ScieLO regional, Pubmed, Cumed, Clinical Key en el período 2012-2017 con las palabras clave: síndrome nefrótico, glomerulonefritis, diagnóstico, terapéutica, atención integral. El abordaje en las glomerulopatías es integral, multidisciplinario e individualizado. En Cuba constituyen la cuarta causa de enfermedad renal crónica y predomina el síndrome nefrítico agudo postinfeccioso. El método clínico juega en ello un papel trascendental a la hora de reconocer y registrar sus aspectos clínicos, su etiología, su fisiopatología, y los exámenes complementarios que confirman su presencia o sus complicaciones, así como un tratamiento oportuno que garanticen el perfeccionamiento asistencial. El arma más poderosa ante el reto de los trastornos glomerulares es la visión integradora y con enfoque individual y social protagonizado por el médico ante este grupo de nefropatías en adultos.

ABSTRACT Glomerulopathies encompass a group of several renal disorders with lesions, mainly in the renal corpuscle, expressed in proteinuria, hematuria, edemas and arterial hypertension. Their clinical manifestations change in dependence of the kind of disease. They are the most frequent cause of chronic renal disease in young adults; therefore their study is very important above all in the health care primary level. The aim was updating pertinent considerations on the diagnostic behavior and comprehensive therapy in the case of glomerulopathy, and evaluating the emission of recommendations regarding to them. A search, analysis and synthesis of information was carried out in the databases ScieLO Cuba, ScieLO regional, Pubmed, Cumed, and Clinical Key in the period 2012-2017, using the key words nephrotic syndrome, glomerulonephritis, diagnosis, therapeutics, comprehensive care. The approach to glomerulopathies is comprehensive, multidisciplinary and individualized. They are the fourth cause of chronic renal disease; the acute post-infectious nephritic syndrome predominates. The clinical method plays a transcendental role at the moment of recognizing and registering their clinical characteristics, etiology and physiopathology, while complementary tests confirm their presence or complications, and therefore an opportune treatment guarantying the healthcare improvement. The most powerful weapon against the challenge of the glomerular disorders is the integrated vision with an individual and social approach led by the physician in the case of these nephropathies in adults.

Trasplante renal en bloque: a propósito de un caso / En bloc kidney transplantation: case report

En México la enfermedad renal crónica en la población pediátrica es un grave problema de salud pública. Las alternativas terapéuticas en niños con enfermedad renal crónica (ERC) son la diálisis y el trasplante renal (TxR), siendo esta última, la mejor opción terapéutica actual en niños con estadios terminal de la función renal. El objetivo de este manuscrito, fue reportar nuestra experiencia en el tratamiento perioperatorio de pacientes pediátricos sometidos a trasplante renal en bloque. Paciente de sexo femenino, 12 años de edad, originaria de Veracruz, México. Portadora de ERC estadio IV de KDOQI, secundaria a glomerulopatía. Fue programada para TxR en bloque de donante cadavérico pediátrico; el que se realizó bajo anestesia general balanceada, con ventilación mecánica controlada, isquemia fría de 17 hs, isquemia caliente de 30 min y un tiempo quirúrgico de 5 hs y 10 min. El tiempo anestésico total, fue de 6 hs. La paciente fue trasladada a la Unidad de Terapia Intensiva Pediátrica, con adecuada aceptación del injerto renal. Durante los siguientes siete meses, el curso clínico ha sido satisfactorio, con estudios ecotomográficos renales normales. En esta paciente la glomerulonefritis, le ocasionó que debutara a muy corta edad con una enfermedad renal crónica con terapia sustitutiva de hemodiálisis. El trasplante renal en bloque realizado, fue exitoso debido al manejo multidisciplinario que participa en el programa de trasplante renal

In Mexico, chronic kidney disease is a major public health problem in pediatric patients. The therapeutic options for chronic kidney disease (CKD) in children are dialysis and kidney transplant (KT); the latter constitutes the current treatment of choice for children suffering from end-stage renal disease. The aim of this study was to describe our experience of perioperative treatment of pediatric patients undergoing en bloc kidney transplant. Female patient, 12 years old, from Veracruz, Mexico, suffering from stage 4 CKD according to KDOQI criteria, secondary to glomerulonephritis. An en bloc kidney transplantarion from a pediatric deceased donor was performed; balanced general anesthesia with mechanical ventilation was used. Cold ischemia time was 17 hours and warm ischemia time was 30 minutes. The surgery lasted 5 hours and 10 minutes and the total anesthesia time was 6 hours. The patient was taken to the pediatric intensive care unit and showed an adequate renal graft acceptance. During the following seven months the clinical course was satisfactory and kidney echotomography showed normal results. Glomerulonephritis made this patient undergo hemodialysis replacement therapy due to CKD at a very early age. The en bloc kidney transplantation performed was successful thanks to the multidisciplinary management involved in the Kidney Transplant Program

Multiple causes of hyponatremia: a case report

Objective:To present a case with 4 different potential causes of hyponatremia

Clinical Presentation and Intervention: The patient presented with the following symptoms: nausea, vomiting, diarrhea, and dark urine after drinking large amounts of fluids that included alcohol and caffeine. Laboratory, microbiological, and morphological examinations revealed the existence of severe hyponatremia and acute poststreptococcal glomerulonephritis. The patient developed acute symptomatic seizures and coma. Gradual normalization of the sodium level led to a recovery of consciousness

Conclusion:Treatment with hypertonic sodium, fluid restriction, and antibiotics led to a complete recovery. In the case of multiple causes of hyponatremia, it is necessary to treat all causes

Microscopic polyangiitis with crescentic glomerulonephritis initially presenting as acute pancreatitis

No abstract available.

Glomerulonefritis difusa aguda postestreptocócica-psoriasis guttata. A propósito de un caso clínico / A case of acute diffuse poststreptococcal glomerulonephritis: guttate psoriasis

La glomerulonefritis asociada a la infección por estreptococo beta hemolítico del grupo A es la más conocida y es la causa más común de síndrome nefrítico en la edad pediátrica. La psoriasis es una enfermedad cutánea hereditaria eritematodescamativa poco frecuente, representa el 4,1% de las dermatosis que ocurren en niños menores de 16 años. Se presenta el caso de un adolescente de 12 años donde la infección por estreptococo beta hemolítico del grupo A de las vías respiratorias altas ocasionó glomerulonefritis difusa aguda, con expresión concomitante de psoriasis guttata. Se revisan los mecanismos inmunes en ambas patologías.

Glomerulonephritis associated with group A beta-hemolytic streptococcal infection is the best known and most common cause of nephritic syndrome in children. Psoriasis is often an erythematous hereditary skin disease. It represents 4.1% of the dermatoses children under 16 years. The study presents the case of a 12 year old patient where Group A beta-hemolytic streptococcal acute upper respiratory infection caused diffuse glomerulonephritis, with concomitant expression of guttate psoriasis. A review of immune mechanisms of both diseases is added.

Patterns of Glomerulonephritis in Uttarakhand State 13 Years Single Centre Experience at Teaching Institute.

Aims : Glomerular disease is considered to be a common cause of chronic kidney disease and its prevalence and histological pattern differ according to geographical area, race, age in different regions of the world. The present study was therefore conducted to study the pattern of different histological types of glomerulonephritis in this area of Uttarakhand region of India over an extended period of 13 years. Material and Methods : Retrospective study was conducted in the tertiary care centre of Uttarakhand state which included cases of different types of glomerulonephritis diagnosed on histopathology over a period of 13 years. Age, sex, clinical presentation, relevant investigations and the histopathological type of glomerulonephritis were noted for every case. Results : Histopathological examination due to various non-neoplastic renal diseases was done in total 218 cases and out of these glomerular diseases was diagnosed in 178 cases. Proteinurea and edema was the most common presentation. Primary glomerulonephritis was observed in 88.5% cases and secondary in 9.9%. Mesangioprolifrative glomerulonephritis was the most common primary glomerulonephritis and nephropathy due to amyloidosis was the most common type of secondary glomerulonephritis. Conclusion : The study concludes that primary glomerulonephritis is the most common renal disease with mesangioproliferative glomerulonephritis as the most common primary glomerulonephritis and amyloidosis nephropathy as the most common secondary glomerulonephritis which differs from other regions of world. The study may be useful to pathologists, nephrologists and health care providers to formulate a basic platform for effective diagnostic, therapeutic and research base for glomerular diseases so as to prevent its complications.

Síndrome nefrótico e injuria renal aguda con microangiopatía trombótica en mujer con enfermedad celíaca: Caso clínico / Nephrotic syndrome and acute kidney injury associated with celiac disease: Report of one case

Celiac disease may be associated with other autoimmune diseases and exceptionally with glomerulopathies and nephrotic syndrome. Associations have been reported with IgA nephropathy, membranoproliferative glomerulonephritis, membranous glomerulopathy and minimal change disease. We report a 63-year-old woman who simultaneously presented with massive nephrotic syndrome (proteinuria 46 g/day) and cachexia due to a malabsorption syndrome secondary to celiac disease. The course of her diseases was complicated with cardiomyopathy due to severe malnutrition, septic shock, acute kidney injury that required dialysis for seven weeks and severe hypertension. A renal biopsy showed a membranoproliferative pattern of injury secondary to a thrombotic microangiopathy and diffusepodocyte damage. Fouryears later, the patient was in good general health, the glomerular filtration rate was 30 ml/min/1.73m² and there was non-nephrotic proteinuria.

Progression of hepatic aspergillosis following second renal transplantation in a patient with recurrent glomerulonephritis.

Invasive aspergillosis is a serious complication in renal transplant recipients. Hepatic involvement, although seen in liver transplant recipients, has not been reported following renal transplantation. We describe here an interesting occurrence of hepatic Aspergillus infection in a renal transplant recipient. The infection responded to anti-fungal therapy, but there was re-activation following a second renal transplant. In addition, the patient had recurrence of the underlying membrano-proliferative glomerulonephritis following both transplants. The relevant existing literature relating to these problems has been reviewed.

Crescentic glomerulonephritis: A clinical and histomorphological analysis of 46 cases.

Background: Crescentic glomerulonephritis (CrGN), defined as crescents involving more than 50% of the glomeruli, includes pauci-immune, immune complex-mediated and anti-glomerular basement membrane disease. Objectives: The present study was aimed at evaluating the various clinical, biochemical and histological parameters in CrGN with respect to these categories and clinical outcome. Materials and Methods: Renal biopsies diagnosed as CrGN between Jan 2008 and Feb 2010 were included. Clinical and laboratory parameters were retrieved along with the therapeutic approach and clinical outcome, wherever available. Renal biopsy slides were evaluated for various glomerular, tubulo-interstitial and arteriolar features. Appropriate statistical tests were applied for significance. Results: A total of 46 cases of CrGN were included; majority (71.7%) of cases were pauci-immune (PI) while 28.3% were immune complex-mediated (IC). Among clinical features, gender ratio was significantly different between PI and IC groups (P = 0.006). The various histological parameters, including proportion of cellular crescents, tuft necrosis and Bowman's capsule rupture, were similar in both the groups. Four unusual associations, including idiopathic membranoproliferative glomerulonephritis (MPGN), multibacillary leprosy, acute lymphoblastic leukemia and C1q nephropathy were detected. Adequate follow-up information was available in 21 (46%) of the patients. Of these, 11 (52.4%) were dialysis-dependent at the last follow-up. Adult patients required renal replacement therapy more frequently than pediatric cases (P = 0.05). Presence of arteriolar fibrinoid necrosis also showed association with poor clinical outcome (P = 0.05). Conclusions: Crescentic glomerulonephritis remains one of the main causes of acute renal failure with histological diagnosis. Immunohistologic examination is essential for accurate classification into one of the three categories. This condition should be considered in rare causal associations like leprosy or MPGN with renal failure, to allow for timely performed renal biopsy and appropriate aggressive therapy.

Effect of RAAS Inhibition on the Incidence of Cancer and Cancer Mortality in Patients with Glomerulonephritis

Angiotensin II type 1 receptor blocker (ARB), which is frequently prescribed in patients with glomerulonephritis (GN), is suggested to increase the risk of cancer. We registered 3,288 patients with renal biopsy and analyzed the relationship between the use of renin-angiotensin-aldosterone system (RAAS) blockade and the incidence of cancer or cancer mortality. After renal biopsy, cancer developed in 33 patients with an incidence rate of 1.0% (95% of CI for incidence: 0.7%-1.3%). There was no difference in the cancer incidence among the groups according to the use of angiotensin-converting enzyme inhibitors (ACEI) or ARB: 1.2% in the None (23/1960), 0.7% in the ARB-only (5/748), 0.4% in the ACEI-only (1/247), and 1.2% in the ACEI-ARB (4/333) (P = 0.487) groups. The cancer mortality was 2.1%, 0.4%, 0.0%, and 0.3% in None, ACEI-only, ARB-only, and ACEI-ARB group, respectively (P < 0.001). The risk of cancer mortality in patients with ARB was only 0.124 (0.034-0.445) compared to that of non-users of ARB by Cox's hazard proportional analysis. In conclusion, prescription of ACEI or ARB in patients with GN does not increase cancer incidence and recipients of ARB show rather lower rates of all-cause mortality and cancer mortality.

Effect of RAAS Inhibition on the Incidence of Cancer and Cancer Mortality in Patients with Glomerulonephritis

Angiotensin II type 1 receptor blocker (ARB), which is frequently prescribed in patients with glomerulonephritis (GN), is suggested to increase the risk of cancer. We registered 3,288 patients with renal biopsy and analyzed the relationship between the use of renin-angiotensin-aldosterone system (RAAS) blockade and the incidence of cancer or cancer mortality. After renal biopsy, cancer developed in 33 patients with an incidence rate of 1.0% (95% of CI for incidence: 0.7%-1.3%). There was no difference in the cancer incidence among the groups according to the use of angiotensin-converting enzyme inhibitors (ACEI) or ARB: 1.2% in the None (23/1960), 0.7% in the ARB-only (5/748), 0.4% in the ACEI-only (1/247), and 1.2% in the ACEI-ARB (4/333) (P = 0.487) groups. The cancer mortality was 2.1%, 0.4%, 0.0%, and 0.3% in None, ACEI-only, ARB-only, and ACEI-ARB group, respectively (P < 0.001). The risk of cancer mortality in patients with ARB was only 0.124 (0.034-0.445) compared to that of non-users of ARB by Cox's hazard proportional analysis. In conclusion, prescription of ACEI or ARB in patients with GN does not increase cancer incidence and recipients of ARB show rather lower rates of all-cause mortality and cancer mortality.

Chronic renal failure in Jamaican children: an update (2001-2006) / Insuficiencia renal crónica en niños jamaicanos: actualización (2001-2006)

OBJECTIVE: This study evaluated the incidence, epidemiology, aetiology and outcome of chronic renal failure (CRF) in Jamaican children < 12 years old between 2001 and 2006. METHODS: The required data on all children who fulfilled inclusion criteria were obtained from their medical records at the University Hospital of the West Indies, Bustamante Hospital for Children and from practitioners in hospitals serving children islandwide. RESULTS: Eighteen new children (72.2% male) presented with CRF. The cumulative annual incidence was 4.61/million child population under age 12 years or 1.14/million total population. Congenital urological disease (44.5%) was the commonest cause of CRF, followed by glomerulonephritis (33.3%). Half of the cases of glomerulonephritis were secondary to HIV-associated nephropathy. Although all children with posterior urethral valves were diagnosed before age 6 months and promptly treated, renal failure present at birth proved irreversible. The mean age at diagnosis of CRF was 6.72 years. Ten children (55.6%) were already in CRF at first presentation with renal disease. Of these, the five with non-urological disease were already in End Stage Renal Disease (ESRD). Mortality was 44.4%. Five children died in ESRD without the benefit of dialysis. CONCLUSION: The incidence of CRF has increased from the 1985-2000 local study and is mainly due to urological pathology which progresses despite early diagnosis and treatment. Non-urological renal disease is presenting too late for therapeutic intervention. Greater public awareness of symptoms of renal disease is needed. Children's access to dialysis is unpredictable. A paediatric dialysis and transplantation programme is needed.

OBJETIVO: Este estudio evaluó la incidencia, epidemiología, etiología y resultado de la insuficiencia renal crónica (IRC) en niños jamaicanos menores de 12 años entre 2001 y 2006. MÉTODOS: Los datos requeridos sobre los niños que satisfacían los criterios de inclusión, fueron obtenidos a partir de sus historias clínicas en el Hospital Universitario de West Indies y en el Hospital Pediátrico Bustamante, así como a partir de médicos en hospitales que prestan atención a niños en todo el país. RESULTADOS: Dieciocho nuevos niños (72.2% varones) presentaron IRC. La incidencia acumulada anual fue de 4.61 por millón de población infantil menor de 12 años de edad o 1.14 por millón de población total. La enfermedad urológica congénita (44.5%) fue la causa más común de IRC, seguida por la glomerulonefritis (33.3%). La mitad de los casos de glomerulonefritis fueron secundarios a la nefropatía asociada al VIH. Aunque todos los niños con válvulas uretrales posteriores fueron diagnosticados antes de los 6 meses de edad y puestos bajos tratamiento, la insuficiencia renal presente al nacer resultó irreversible. La edad promedio al momento del diagnóstico de la IRC fue 6.72 años. Diez niños (55.6%) se hallaban ya con IRC desde que se les presentara inicialmente la enfermedad renal. De estos, los cinco con enfermedad no urológica se hallaban ya en la fase terminal de la enfermedad renal (ERT). La mortalidad fue de 44.4%. Cinco niños murieron en la ERT sin el beneficio de la diálisis. CONCLUSIÓN: La incidencia de la IRC ha aumentado desde el estudio local de 1985-2000, principalmente debido a patologías urológicas que progresan a pesar del diagnóstico precoz y el tratamiento. La enfermedad renal no urológica se está presentando demasiado tarde para una intervención terapéutica. Se requiere mayor conciencia pública de los síntomas de la enfermedad renal. El acceso de los niños a la diálisis es imprescindible. Hace falta una diálisis infantil y un programa de transplante.

Custo-efetividade do tratamento da infecção pelo vírus da hepatite C em candidatos a transplante renal submetidos à diálise / Cost-effectiveness of hepatitis C treatment in patients on dialysis awaiting renal transplantation

O estudo tem como objetivo geral avaliar a razão de custo-utilidade do tratamento da infecção pelo vírus da hepatite C (VHC) em pacientes dialisados, candidatos a transplante renal, tendo como esquemas terapêuticos alternativos o interferon-a em monoterapia; o interferon peguilado em monoterapia; o interferon-a em terapia combinada com ribavirina e o interferon peguilado em terapia combinada com ribavirina, comparando-os com o não-tratamento. A perspectiva do estudo foi a do Sistema Único de Saúde(SUS), que também serviu de base para estimar o impacto orçamentário da estratégia de tratamento mais custo efetiva. Para o alcance dos objetivos, foi construído um modelo de Makov para simulação de custos e resultados de cada estratégia avaliada. Para subsidiar o modelo, foi realizada uma revisão de literatura, a fim de definir os estados de saúde relacionados à infecção pelo vírus da hepatite C em transplantados e a probabilidade de transição entre os estados. Medidas de utilidade foram derivadas de consultas a especialistas. Os custos foram derivados da tabela de procedimentos do SUS. Os resultados do estudo demonstraram que o tratamento da infecção pelo VHC antes do transplante renal é mais custo-efetivo que o não tratamento, apontando o interferon-a como a melhor opção. O impacto orçamentário para adoção dessa estratégia pelo SUS corresponde a 0,3 por cento do valor despendido pelo SUS com terapia renal substitutiva ao longo do ano de 2007.

A Case of Light Chain Deposition Disease Involving Kidney and Bone Marrow with Microangiopathic Hemolytic Anemia / 대한진단검사의학회지

We report a case of light chain deposition disease in a 59-yr-old female showing deposition of monoclonal light chain in the kidney and bone marrow accompanied with a schistocytosis, the morphologic finding of microangiopathic hemolytic anemia. The immunofluorescence examination of the kidney revealed strongly stained kappa-light chain deposits on the glomerular mesangium and capillary wall, tubules, and vessel wall. The electron microscopy demonstrated electron-dense deposits on the glomerular basement membrane and mesangium. Anemia was observed with schistocytosis and Howell-Jolly body in the peripheral blood smears. The immunohistochemical examination of the bone marrow showed the presence of kappa-light chain deposits in scattered plasma cells and thickened vessel wall in the absence of a prominent plasma cell proliferation. Although an immunofixation electrophoresis failed to detect a monoclonal gammopathy, the presence of monoclonal protein could be identified by an abnormal kappa/lambda ratio on the serum free light chain analysis.

Acute hypocomplementemic post-infectious glomerulonephritis as a complication of sinus-related orbital cellulitis: case report / Glomerulonefrite pós-infecciosa hipocomplementêmica aguda como complicação de celulite orbitária relacionada aos seios paranasais: relato de caso

Group A beta-hemolytic streptococcus is the most common agent implicated in post-infectious acute glomerulonephritis. We report a case of acute poststreptococcal glomerulonephritis associated with sinus-related orbital abscess in an 11-year-old boy treated with surgical drainage and intravenous ceftriaxone and clindamycin. Twelve days after supportive measures, renal function was normalized. We also discuss this potentially severe nonsuppurative complication of orbital cellulitis caused by group A beta-hemolytic streptococcus.

Os estreptococos beta-hemolíticos do grupo A são os agentes mais comumente envolvidos na glomerulonefrite aguda pós-infecciosa. Relatamos um caso de glomerulonefrite pós-estreptocócica aguda associada a um abscesso orbitário secundário à sinusite, em menino de 11 anos de idade, o qual foi tratado com ceftriaxona e clindamicina endovenosas e drenagem cirúrgica. Doze dias após tratamento de suporte, a função renal se normalizou. Também discutimos a importância desta grave e potencial complicação não supurativa das celulites orbitárias causadas pelos estreptococos beta-hemolíticos do grupo A.

Comportamiento clínico y manejo de glomerulonefritis postestreptoccocica en niños egresados del HFVP en el período de enero 2006 a enero 2007 / Behavior and clinical management of glomerulonephritis postestreptoccocica in children discharged from the HFVP in the period from January 2006 to January 2007

El presente estudio fue realizado en el Hospital Fernando Velez Paiz en el período de enero 2006 a enero 2007. El principal objetivo fue el de conocer el comportamiento clínico y manejo de los niños con síndrome nefrítico egresados en el período de estudio. La muestra estuvo constituida por 48 pacientes, manejados en nuestro centro asistencial, fueron referidos únicamente 7 pacientes que presentaron complicaciones renales. La mayoría de los pacientes fueron del sexo masculino. La totalidad de estos pacientes presentaron como mínimo un foco infeccioso previo a su ingreso, predominantemente a nivel de faringe y piel. EL comportamiento clínico fue predominantemente clásico como edema, hipertensión y alteraciones del sedimento urinario como muestra de afectación renal...

unusual association between familial mediterranean fever and IgM nephropathy

To report a case with the diagnosis of IgM nephropathy and familial Mediterranean fever [FMF]. Clinical Presentation and Intervention: A 9-year-old boy was admitted to our hospital with recurrent abdominal pain since the age of 4 years. Laboratory investigations revealed a sedimentation rate of 88 mm/h, C-reactive protein: 83.2 mg/l [0-10 mg/l], white blood cell count: 12,700/mm3, fibrinogen: 622 mg/dl [200-400 mg/dl] and serum amyloid A: 186 mg/l [0-5.8 mg/l]. Urinalysis revealed +2 proteinuria. A 24-hour urinary protein excretion was 12 mg/m2/h. M694V homozygous mutation was identified in exon 10. Percutaneous renal biopsy showed mesangial cell proliferation and increased mesangial matrix in the glomeruli, without amyloid accumulation. Immunofluorescence study showed IgM [+1] and C1q [+1] deposits. Treatment with 1 mg/day colchicine was started. Six weeks later, proteinuria had disappeared and the patient was asymptomatic. This case illustrates the unusual association of FMF with non-amyloid glomerulopathy. Glomerular diseases such as IgM nephropathy may be seen as a manifestation of FMF