Takayasu arteritis: differential diagnosis in a teenager with severe acute kidney injury - a case report.

Takayasu arteritis (TA) is a chronic granulomatous inflammatory condition of unknown cause that involves large vessels - particularly the aorta and its branches - such as the carotid, coronary, pulmonary, and renal arteries. The left subclavian artery is the most frequently involved vessel. Stenosis of the renal artery has been reported in 23-31% of the cases and may result in malignant hypertension, ischemic renal disease, decompensated heart failure, and premature death. Involvement of both renal arteries is uncommon. Early onset anuria and acute kidney injury are rare and have been reported only in a few cases in the literature. This report describes the case of a 15-year-old female with constitutional symptoms evolving for a year, combined with headache, nausea, and vomiting, in addition to frequent visits to emergency services and insufficient clinical examination. The patient worsened significantly six months after the onset of symptoms and developed acute pulmonary edema, oliguria, acute kidney injury, and difficult-to-control hypertension, at which point she was admitted for intensive care and hemodialysis. Initial ultrasound examination showed she had normal kidneys and stenosis-free renal arteries. The patient was still anuric after 30 days of hospitalization. A biopsy was performed and revealed her kidneys were normal. Computed tomography angiography scans of the abdominal aorta presented evidence of occlusion of both renal arteries. The patient met the diagnostic criteria for Takayasu arteritis and had a severe complication rarely described in the literature: stenosis of the two renal arteries during the acute stage of ischemic renal disease.

Development of a risk score for earlier diagnosis of chronic kidney disease in children.

OBJECTIVE: To develop a clinical score for the early identification of chronic kidney disease (CKD) in children and adolescents. The early diagnosis of CKD in childhood allows the adoption of measures to slow the progression of the disease, thereby reducing morbidity and mortality. Nevertheless, the diagnosis is often made too late for proper patient management. STUDY DESIGN: We preformed a case-control study of a multicenter Brazilian sample of 752 pediatric patients; the study cases (n = 376) were CKD patients with a median estimated GFR of 37 (IQR = 22 to 57) ml/min/1.73 m2. The control group (n = 376) comprised age-, gender- and center-matched children who were followed for nonrenal diseases. Potential risk factors were investigated through a standard questionnaire that included symptoms, medical history, and a clinical examination. Two multivariable models (A and B) were fitted to assess predictors of the diagnosis of CKD. RESULTS: In model A, 9 variables were associated with CKD diagnosis: antenatal ultrasound with urinary malformation, recurrent urinary tract infection, polyuria, abnormal urine stream, nocturia, growth curve flattening, history of hypertension, foamy urine and edema (c-statistic = 0.938). Model B had the same variables as model A, except for the addition of the history of admission during the neonatal period and the exclusion of antenatal ultrasound variables (c-statistic = 0.927). CONCLUSIONS: The present scores may serve as a warning sign for CKD diagnosis in children among professionals working in the primary care setting where the symptoms associated with a risk of CKD may be overlooked.

Rare case of nephrotic syndrome: Schimke syndrome.

Schimke syndrome corresponds to dysplasia of bone and immunity, associated with progressive renal disease secondary to nephrotic syndrome cortico-resistant, with possible other abnormalities such as hypothyroidism and blond marrow aplasia. It is a rare genetic disorder, with few reports in the literature. The most frequent renal involvement is nephrotic syndrome with focal segmental glomerulosclerosis and progressive renal failure. The objective of this study was to report a case of Schimke syndrome, diagnostic investigation and management of the case.

Resumo A síndrome Schimke corresponde à displasia imuno-óssea, associada à doença renal progressiva secundária à síndrome nefrótica córtico-resistente, podendo haver outras anormalidades como hipotireoidismo e aplasia de medula óssea. Trata-se de uma patologia genética rara, com poucos relatos na literatura. O acometimento renal mais frequente é uma síndrome nefrótica por glomeruloesclerose segmentar e focal e falência renal progressiva. O objetivo deste estudo foi relatar um caso de síndrome de Schimke, investigação diagnóstica e condução do caso.

Takayasu arteritis: differential diagnosis in a teenager with severe acute kidney injury - a case report / Arterite de Takayasu: diagnóstico diferencial em adolescente com lesão renal aguda grave - relato de caso

ABSTRACT Takayasu arteritis (TA) is a chronic granulomatous inflammatory condition of unknown cause that involves large vessels - particularly the aorta and its branches - such as the carotid, coronary, pulmonary, and renal arteries. The left subclavian artery is the most frequently involved vessel. Stenosis of the renal artery has been reported in 23-31% of the cases and may result in malignant hypertension, ischemic renal disease, decompensated heart failure, and premature death. Involvement of both renal arteries is uncommon. Early onset anuria and acute kidney injury are rare and have been reported only in a few cases in the literature. This report describes the case of a 15-year-old female with constitutional symptoms evolving for a year, combined with headache, nausea, and vomiting, in addition to frequent visits to emergency services and insufficient clinical examination. The patient worsened significantly six months after the onset of symptoms and developed acute pulmonary edema, oliguria, acute kidney injury, and difficult-to-control hypertension, at which point she was admitted for intensive care and hemodialysis. Initial ultrasound examination showed she had normal kidneys and stenosis-free renal arteries. The patient was still anuric after 30 days of hospitalization. A biopsy was performed and revealed her kidneys were normal. Computed tomography angiography scans of the abdominal aorta presented evidence of occlusion of both renal arteries. The patient met the diagnostic criteria for Takayasu arteritis and had a severe complication rarely described in the literature: stenosis of the two renal arteries during the acute stage of ischemic renal disease.

RESUMO A Arterite de Takayasu (AT) é uma doença inflamatória crônica, granulomatosa, de causa desconhecida, que afeta grandes vasos, principalmente a aorta e seus ramos, incluindo artérias carótidas, coronárias, pulmonares e renais, sendo a artéria subclávia esquerda o vaso mais acometido. A estenose da artéria renal é relatada em 23-31% dos casos e pode resultar em hipertensão maligna, insuficiência renal por isquemia, descompensação cardíaca e morte prematura. O acometimento bilateral de artérias renais é incomum, sendo rara a presença de anúria súbita e lesão renal aguda como sintoma inicial da doença, com poucos relatos na literatura. O caso reporta uma adolescente de 15 anos com sintomas constitucionais durante um ano de evolução, associados a problemas como cefaleia, náuseas e vômitos, com idas frequentes a serviços de emergência, sem adequada investigação clínica. Após 6 meses do início dos sintomas, a paciente evoluiu de forma grave, com quadro de edema agudo de pulmão, oligúria, lesão renal aguda e hipertensão arterial de difícil controle, sendo necessário suporte em Unidade de Terapia Intensiva e hemodiálise. A ultrassonografia inicial mostrava rins normais e artérias renais sem sinais de estenose. Após 30 dias de internamento, paciente permanecia anúrica, sendo realizada biópsia renal que se mostrou dentro dos padrões da normalidade. Angiotomografia de aorta abdominal evidenciou oclusão bilateral de artérias renais. A paciente descrita fechou critérios diagnósticos para arterite de Takayasu e manifestou uma complicação grave pouco descrita na literatura: estenose bilateral de artérias renais, ainda na fase aguda da nefropatia isquêmica.

Rare case of nephrotic syndrome: Schimke syndrome / Caso raro de síndrome nefrótica: síndrome de Schimke

Abstract Schimke syndrome corresponds to dysplasia of bone and immunity, associated with progressive renal disease secondary to nephrotic syndrome cortico-resistant, with possible other abnormalities such as hypothyroidism and blond marrow aplasia. It is a rare genetic disorder, with few reports in the literature. The most frequent renal involvement is nephrotic syndrome with focal segmental glomerulosclerosis and progressive renal failure. The objective of this study was to report a case of Schimke syndrome, diagnostic investigation and management of the case.

Resumo A síndrome Schimke corresponde à displasia imuno-óssea, associada à doença renal progressiva secundária à síndrome nefrótica córtico-resistente, podendo haver outras anormalidades como hipotireoidismo e aplasia de medula óssea. Trata-se de uma patologia genética rara, com poucos relatos na literatura. O acometimento renal mais frequente é uma síndrome nefrótica por glomeruloesclerose segmentar e focal e falência renal progressiva. O objetivo deste estudo foi relatar um caso de síndrome de Schimke, investigação diagnóstica e condução do caso.