Xanthogranulomatous pyelonephritis in a pediatric patient / Pielonefrite xantogranulomatosa em paciente pediátrico

Abstract Introduction: Xanthogranulomatous pyelonephritis consists of a chronic infectious and inflammatory process of the renal parenchyma, a variant of chronic obstructive pyelonephritis. It is more prevalent in middle-aged adults, rare in pediatric patients, with less than 300 cases reported in children worldwide. Report: Preschooler, aged 2 years and 11 months, male, with 2 months of abdominal distention, increased temperature and intense pallor, associated with microcytic anemia refractory to the use of ferrous sulfate. 1 week before, he had a bulging in his left flank and a hard palpable mass there. Imaging exams (ultrasound and tomography) revealed an overall enlargement of the left kidney, destruction of the renal parenchyma and intense calyceal dilation, forming the "bear's paw" sign, with a staghorn calculus in the pelvis. He underwent treatment with antibiotic therapy and total nephrectomy, with a specimen sent for pathological examination. Discussion: a disease of uncertain incidence in the pediatric age group, xanthogranulomatous pyelonephritis is more prevalent in male children and affects mainly the left kidney, being frequently associated with the presence of stones. Clinically, it has nonspecific symptoms, the most common being abdominal distension and asthenia. Laboratory exams shows microcytic, leukocytosis, thrombocytosis and increased inflammation, pyuria, hematuria and proteinuria, in addition to bacterial growth in urine culture. The diagnosis is anatomopathological, but it can be hinted by contrasted CT scan, with the classical sign of the "bear's paw". Treatment may include nephrectomy and broad-spectrum antibiotic therapy.

Resumo Introdução: A pielonefrite xantogranulomatosa consiste em um processo infeccioso e inflamatório crônico do parênquima renal, variante da pielonefrite obstrutiva crônica. É mais prevalente em adultos na meia-idade, de ocorrência rara em pacientes pediátricos, com menos de 300 casos relatados em pediatria no mundo. Relato: Pré-escolar de 2 anos e 11 meses, do sexo masculino, com quadro de 2 meses de distensão e aumento da temperatura abdominal e palidez intensa, associado a anemia microcítica refratária ao uso de sulfato ferroso. Apresentava ainda, havia 1 semana da internação, abaulamento em região do flanco esquerdo e massa palpável, de consistência endurecida. Exames de imagem (ultrassonografia e tomografia) revelaram aumento global do rim esquerdo, destruição do parênquima renal e intensa dilatação calicial formando o sinal da "pata de urso", com presença de cálculo coraliforme em pelve. Submetido a tratamento com antibioticoterapia e nefrectomia total, com produto enviado para anatomopatológico. Discussão: Doença de incidência incerta na faixa pediátrica, a pielonefrite xantogranulomatosa é mais prevalente no sexo masculino na infância e acomete principalmente o rim esquerdo, estando frequentemente associada à presença de cálculos. Clinicamente apresenta-se como quadro arrastado de sintomas inespecíficos, sendo os mais comuns distensão abdominal e astenia. Laboratorialmente, apresenta-se com anemia microcítica, leucocitose, trombocitose e aumento de provas inflamatórias, piúria, hematúria e proteinúria, além de crescimento bacteriano em cultura de urina. O diagnóstico é anatomopatológico, porém pode ser sugerido pela tomografia computadorizada com contraste, que tem como sinal clássico a "pata de urso". O tratamento pode incluir nefrectomia e antibioticoterapia de amplo espectro.

Xanthogranulomatous pyelonephritis in a pediatric patient.

INTRODUCTION: Xanthogranulomatous pyelonephritis consists of a chronic infectious and inflammatory process of the renal parenchyma, a variant of chronic obstructive pyelonephritis. It is more prevalent in middle-aged adults, rare in pediatric patients, with less than 300 cases reported in children worldwide. REPORT: Preschooler, aged 2 years and 11 months, male, with 2 months of abdominal distention, increased temperature and intense pallor, associated with microcytic anemia refractory to the use of ferrous sulfate. 1 week before, he had a bulging in his left flank and a hard palpable mass there. Imaging exams (ultrasound and tomography) revealed an overall enlargement of the left kidney, destruction of the renal parenchyma and intense calyceal dilation, forming the "bear's paw" sign, with a staghorn calculus in the pelvis. He underwent treatment with antibiotic therapy and total nephrectomy, with a specimen sent for pathological examination. DISCUSSION: a disease of uncertain incidence in the pediatric age group, xanthogranulomatous pyelonephritis is more prevalent in male children and affects mainly the left kidney, being frequently associated with the presence of stones. Clinically, it has nonspecific symptoms, the most common being abdominal distension and asthenia. Laboratory exams shows microcytic, leukocytosis, thrombocytosis and increased inflammation, pyuria, hematuria and proteinuria, in addition to bacterial growth in urine culture. The diagnosis is anatomopathological, but it can be hinted by contrasted CT scan, with the classical sign of the "bear's paw". Treatment may include nephrectomy and broad-spectrum antibiotic therapy.

Myeloid sarcoma in the breast in a patient withacute myeloid leukemia: a case report

Myeloid sarcoma infiltration into the breast of patients with acute myeloid leukemia is rare. The present study reports the case of a 56-year-old woman diagnosed with AML and an incidental finding of a breast tumor. The nodule biopsy raised the suspicion of invasive lobular carcinoma and poorly differentiated angiosarcoma. Subsequent immunohistochemical study concluded the diagnosis of myeloid sarcoma. The varied image presentations, the lack of knowledge of clinical data and complementary propaedeutics, and the histopathological similarity with certain primary breast lesions make it difficult to discover secondary infiltration by myeloid sarcoma in this unusual site

Linfoma primário de efusão em mulher com síndrome da imunodeficiênciaadquirida (SIDA), derrame pleural e ascite: diagnóstico citológico e imunocitoquímico / Primary effusion lymphoma in women with acquired immunodeficiency syndrome (AIDS), pleural effusion, and ascites: cytological diagnosis and immunocytochemistry

O linfoma primário de efusão é tipo raro de forma não Hodgkin que se manifesta com derrame cavitário, em geral associado à imunodepressão. Associa-se à infecção pelo vírus herpes humano 8 (HHV-8), fato necessário para que seja confirmado seu diagnóstico.Este relato descreve o linfoma primário de efusão, diagnosticado por meio de citologia e imunocitoquímica, em paciente com manifestação não usual de comprometimento de duas cavidades serosas. Seu prognóstico é reservado e seu tratamento é limitado.

The primary effusion lymphoma is a rare type of non-Hodgkin form that manifests with cavity stroke, usually associated with immunosuppression. It is associated with infection with the human herpes virus 8 (HHV-8), a necessary fact to confirm the diagnosis. This report describes the primary effusion lymphoma, diagnosed by cytology and immunocytochemistry,in patients with the unusual manifestation of involvement of two serouscavities. Its prognosis is poor, and treatment is limited.

Botriomicose cutânea - relato de caso em Minas Gerais, Brasil / Skin botryomycosis - case report in Minas Gerais, Brazil

Botriomicose é doença infecciosa crônica granulomatosa e supurativa causada por bactérias Gram-positivas e Gram-negativas que formam grãos pseudomicóticos. As lesões cutâneas sãoplacas e tumores multinodulares com ulceração e formação de fístulas que podem eliminar grãos. A infecção acomete a pele, com possível disseminação para vísceras. É doença relativamente rara e seu diagnóstico pode ser alcançado por meio de biópsia das lesões e culturados grânulos nas secreções positivas para cocos Gram-positivos ou Gram-negativos. Esta descrição apresenta a botriomicose cutânea em brasileiro causada por Staphylococcus aureus.

Botryomycosis is a chronic infectious suppurative and granulomatous disease caused by Gram-positive and Gram-negative bacteria that form pseudo mycotic grains. The skin lesions are plaques and multinodular tumors with ulceration and formation of fistulae thatcan eliminate grains. The infection affects the skin, with possible dissemination to viscerae. It is relatively rare, and its diagnosis can be achieved through the biopsy of lesions and culture of granules from positive Gram-positive and Gram-negative cocci secretions. This description shows a case of skin botryomycosis in Brazil caused by Staphylococcus aureus.

Conservative treatment of uterine cervical adenocarcinoma in pregnancy.

Cancer of the cervix is the most common malignancy diagnosed during pregnancy, with an incidence of 1-10 cases per 10,000 pregnancies. The desire of patients to maintain pregnancy and subsequent fertility is a difficult target to be achieved and should be widely studied, since it depends on the stage of disease, gestational age at diagnosis, and the woman's desire to maintain pregnancy. We describe in this report the case of a pregnant woman with invasive cervical adenocarcinoma in stage IB1 (FIGO) initially treated with neoadjuvant chemotherapy, followed by radical surgery and cesarean section in the same surgical procedure.

Síndrome de ativação macrofágica em paciente com lúpus eritematoso sistêmico juvenil / Macrophage activation syndrome in a patient with juvenile systemic lupus erythematosus

A hemofagocitose reativa ou síndrome de ativação macrofágica (SAM) é uma complicação das doenças inflamatórias sistêmicas, causada por expansão de células T e macrófagos, com produção maciça de citocinas pró-inflamatórias, ocorrendo mais freqüentemente na artrite idiopática juvenil sistêmica e raramente no lúpus eritematoso sistêmico juvenil (LESJ). OBJETIVO: Relatar um caso de LESJ que evoluiu com SAM precipitada por infecção e infarto esplênico, com desfecho fatal. RELATO DE CASO: Uma menina de 7 anos, com diagnóstico de LESJ desde os 5 anos, evoluiu com artrite em atividade, alopecia intensa, citopenias, cefaléia, infecções respiratórias recorrentes e elevação intermitente de transaminases. Os anticorpos anti-DNA e anticardiolipina IgG e IgM foram identificados e a biópsia renal evidenciou glomerulonefrite lúpica de classe III. A paciente foi tratada com pulso de metilprednisolona, prednisona, azatioprina e hidroxicloroquina. Após dois anos, na vigência de pneumonia apresentou abdome agudo e convulsões, evoluindo para o choque hemorrágico fatal após esplenectomia, que evidenciou infarto esplênico e infiltração maciça por macrófagos hemofagocíticos CD163+. CONCLUSÃO: A revisão do desfecho sugere a SAM precipitada por infecção e sobreposta a atividade inflamatória do lúpus com febre persistente, citopenias, disfunção hepática, hepatomegalia e esplenomegalia, como efeitos do excesso de produção de citocinas. Os anticorpos anticardiolipina podem ter tido papel precipitante na coagulopatia, que resultou infarto esplênico e choque hemorrágico.

Reactive haemophagocytosis or macrophage activation syndrome (MAS) is a complication of systemic inflammatory disorders, caused by expansion of T cells and haemophagocytic macrophages, with cytokine overproduction. It has been described most often in systemic juvenile idiopathic arthritis and rarely in juvenile systemic lupus erythematosus (JSLE). OBJECTIVE: To report a JSLE case who developed MAS in association with spleen infarct triggered by infection, with fatal outcome. CASE REPORT: A 7-year old-girl diagnosed with lupus since age 5-y developed several episodes of arthritis flare, cytopenias, severe alopecia, headaches and recurrent episodes of respiratory infections with intermittently increased serum transaminases. Anti-DNA and anti-cardiolipin IgG and IgM were identified and Class III lupus glomerulonephritis was diagnosed by renal biopsy. The patient was treated with methylprednisolone pulses, prednisone, azatioprine and hydroxychloroquine. Last admitted due to pneumonia, she evolved into abdominal crisis and seizures, undergoing splenectomy and evolving into haemorragic shock with fatal outcome. A spleen infarct was found and anti-CD163 antibodies staining disclosed intense haemophagocytic macrophage infiltration. CONCLUSION: This outcome suggests infection-triggered MAS overlapping lupus flare with persistent fever, cytopenia, liver dysfunction, hepatomegaly and splenomegaly as cytokine excess driven effect. Anti-cardiolipin antibodies may also had a coagulopathy precipiting role.