Atypical presentation of acute post-infectious glomerulonephritis in patients with sickle cell disease: report of two cases.

BACKGROUND: Sickle cell disease (SCD) is a highly prevalent genetic disease worldwide. In the natural evolution of SCD, glomerular lesions can develop, presenting histopathological patterns of segmental or focal membranoproliferative glomerulosclerosis, with or without thrombotic microangiopathy. We report two cases of acute post-infectious glomerulonephritis (APIGN), with atypical presentations, in patients with SCD. CASE PRESENTATION: Case 1: An 18-year-old female with SCD presented with a 21-day history of progressive oedema, accompanied by dyspnoea, productive cough, fever, and chest pain. Blood tests showed the following: haemoglobin 6.1 g/dl; leucocytes 18,820 cells/mm3; and creatinine 0.49 mg/dl. A urine sample evidenced leucocyturia and haematuria. The 24-h proteinuria was 8.99 g, serum albumin level was 1.2 g/dl, low serum C3 levels and high levels of anti-streptolysin O. Renal biopsy was consistent with APIGN. The patient was treated with diuretic and anti-proteinuric agents, subsequently evolving to reversal of the renal alterations. Case 2: A 12-year-old male with SCD presented with a 20-day history of a non-productive cough and progressive oedema, together with hypertension. The serum creatinine concentration was 0.48 mg/dl. A urine sample evidenced leukocyturia and haematuria. The 24-h proteinuria was 12.5 g, and the serum albumin level was 2.6 g/dl. The levels of C3 and C4 were normal. Renal biopsy revealed APIGN. The patient was treated with diuretic and anti-proteinuric agents, subsequently evolving reversal of the renal alterations. CONCLUSIONS: The presentation of the two cases reported here are not typical of SCD-related kidney injury. Analysis of the renal biopsy specimens elucidated the diagnosis, affecting the prognosis, because that of APIGN is highly favourable, unlike that of nephrotic syndrome associated with SCD glomerulopathy.

Correção cirúrgica de aneurismas saculares de fístula arteriovenosa para hemodiálise utilizando a técnica de aneurismorrafia / Surgical repair of saccular aneurysms of an arteriovenous fistula for hemodialysis using aneurismorraphy technique

Uma das complicações mais frequentes da fístula arteriovenosa (FAV) para hemodiálise é o desenvolvimento de um aneurisma. A formação de aneurismas geralmente decorre de um enfraquecimento da parede venosa devido às repetidas punções, e sua rotura causa hemorragia intensa que pode levar à morte. Descrevemos o caso de uma paciente com dois aneurismas saculares de FAV, tratados através de aneurismorrafia, detalhando a técnica cirúrgica utilizada e a evolução pós-operatória. A técnica de rafia simples da parede constitui uma boa alternativa no reparo da parede da FAV quando o aneurisma é de curta extensão, evitando a colocação de um material protético e a confecção de duas anastomoses.

One of the most common complications of arteriovenous fistulas (AVF) is aneurysm formation due to weakening of the venous wall after repeated punctures. Its rupture causes severe bleeding that can lead to death. We report the case of a patient with two AVF saccular aneurysms treated by aneurysmorrhaphy. The technique and the postoperative follow-up are presented in detail. Aneurysmorrhaphy is a good option to repair the AVF wall when the aneurysmal segment is short, thus avoiding the placement of prosthetic grafts and the performance of two anastomoses.