RESUMO
Both lipoprotein glomerulopathy (LPG) and fibrillary glomerulonephritis (FGN) are rare causes of end-stage renal disease (ESRD), and the literature concerning the outcome of kidney transplant in patients with LPG or FGN is scarce. We report a patient who suffered from ESRD with coexisting FGN and LPG and received deceased kidney transplant >10 years ago did not reveal any clinical features of disease recurrence during follow-up. Our case shows that the prognosis of patients with LPG component who received kidney transplant can be good. Kidney transplantation remains a viable therapeutic option for patients with ESRD secondary to FGN with LPG.
Assuntos
Neoplasias dos Nervos Cranianos/patologia , Células Epitelioides/patologia , Doenças do Nervo Facial/patologia , Neurilemoma/patologia , Neoplasias Parotídeas/patologia , Biomarcadores Tumorais/metabolismo , Colágeno Tipo IV/metabolismo , Neoplasias dos Nervos Cranianos/metabolismo , Neoplasias dos Nervos Cranianos/cirurgia , Células Epitelioides/metabolismo , Nervo Facial/metabolismo , Nervo Facial/patologia , Nervo Facial/cirurgia , Doenças do Nervo Facial/metabolismo , Doenças do Nervo Facial/cirurgia , Feminino , Humanos , Pessoa de Meia-Idade , Neurilemoma/metabolismo , Neurilemoma/cirurgia , Neoplasias Parotídeas/metabolismo , Neoplasias Parotídeas/cirurgia , Proteínas S100/metabolismoRESUMO
Lipoprotein glomerulopathy is a rare kidney disease in which lipoprotein thrombi are seen in the glomerular capillaries. Most of these patients are found in Japan and East Asian countries. The presenting symptoms include proteinuria, an abnormal plasma lipoprotein profile that resembles type III hyperlipoproteinaemia, and a marked increase in serum apolipoprotein E concentration. Previous studies have suggested that lipoprotein glomerulopathy might be related to APOE gene mutation. No effective therapeutic regimen has been established for lipoprotein glomerulopathy. We report the first case of biopsy-proven lipoprotein glomerulopathy in Hong Kong in a patient who presented with nephrotic syndrome and dyslipidaemia. DNA analysis revealed apolipoprotein E Kyoto together with a novel apolipoprotein E mutation, apolipoprotein E (Asp230Tyr) Hong Kong. There was significant improvement in the clinical parameters and resolution of symptoms after the introduction of statins. Further studies will be needed to clarify the role of apolipoprotein E Hong Kong and its interaction with apolipoprotein E Kyoto in the pathogenesis of lipoprotein glomerulopathy.
