Síndrome hemofagocítico en un trasplantado renal con síndrome de Alport / Hemophagocytic syndrome after kidney transplant in a patient with hereditary nephritis. Report of one case

We report a 28-year-old mole with a hereditary nephritis (Alport Syndrome) on hemodialysis for 5 years, who received a kidney graft from a deceased donor. Cyclosporine (CsA), mycophenolate mofetil (MMF) and steroids were prescribed. In thepostoperativeperiod thepatient had thrombophlebitis and diarrhea. A CTsean showed splenomegaly, ascites, bilateral pleural effusion and bowel edema. Laboratory showed hypoalbuminemia, increased C reactive protein (CRP) and panhypogam-maglobulinemia. At day 32 after transplantation, an acute rejection (Banff II b) was diagnosed and treated with methylprednisolone, replacing CsA by tacrolimus. The acute rejection was controlled but six days later, high fever, pancytopenia and hyperferritinemia appeared. A bone marrow smear showed numerous histiocytes and hemophagocytosis. Hemophagocytic syndrome was diagnosed. MMF and tacrolimus were withdrawn and CsA was reinstituted. Fever fell quickly, CPR normalized at 24 hours and white blood cell count at 72 hours. Days later, the concentrations of albumin, immunoglobulins and hematological parameters normalized. The patient was discharged on day 57 after admission in good condition.

Alport syndrome: significance of gingival biopsy in the initial diagnosis and periodontal evaluation after renal transplantation

Alport Syndrome (AS) is an important hereditary disorder affecting the glomerular basement membrane. Diagnosis of AS is based on the presence of hematuric nephropathy, renal failure, hearing loss, ocular abnormalities and changes in the glomerular basement membrane of the lamina densa. The aims of this case report were to show the changes in the gingival tissues in a patient with AS under therapy with cyclosporin-A after renal transplantation and to discuss the possible role of type IV collagen in gingival basal lamina as an alternative approach for the diagnosis of AS. A 20-year-old male patient with AS underwent periodontal therapy including a series of gingivectomy surgeries. Gingival samples obtained during the second surgery were examined histopathologically and by transmission electron microscopy for further pathological examination. Gingivectomy procedures have been performed every 6 months over the last 4 years. The excessive and fibrous gingival enlargements resulted in migration of the anterior teeth, but no alveolar bone loss occurred. This is the first report to demonstrate the possible changes in the gingival tissues caused by AS. It is suggested that gingival biopsy can be an initial diagnostic tool instead of renal or skin biopsies. Proper dental and periodontal care and regular visits to the dentist could provide limited gingival hyperplasia to patients with AS.

Síndrome de Alport: relato de um caso com recuperaçäo total / Alport syndrome: report of a case of total recovery

Os autores relatam um caso de paciente do sexo masculino, 27 anos, com síndrome de. Alport. Anteriormente ao tratamento cirúrgico do déficit visual por facoemulsificaçäo è implante de lente intra-ocular, o paciente tinha sido submetido a transplante renal com recuperaçäo total da funçäo do órgäo. Acuidade visual do paciente após o tratamento foi de 20120 em ambos os olhos. Descrevem sucintamente a etiopatogenia, sinais clínicos e critérios diagnósticos da síndrome.

Enfermedad antimembrana basal glomerular en un paciente trasplantado renal con enfermedad de Alport / Anti glomerular basement membrane disease in a renal transplant patient with Alport syndrome

Se comunica un caso de enfermedad anti membrana basal glomerular (anti MBG) en un paciente com síndrome de Alport que recibió un trasplante de riñón cadavérico. Luego de alcanzar una función renal normal al mês del trasplante, deterioró progresivamente la función a partir del 3er mês y la punción biopsia renal mostró formación de semilunas y depósitos lineales de inmunoglobulinas. El estudio del suero demostró anticuerpos contra la cadena alfa 5 del colágeno tipo IV y recibió tratamiento con plasmaféresis, lográndose estabilización funcional durante un año. Al cabo de dicho lapso una infección respiratoria requirió interrupción de la inmunosupresión y el paciente debió reingresar al programa de hemodiálisis crónica. Se discuten los posibles mecanismos que condicionaron la especificidad de los anticuerpos circulantes en este caso, ya que difiere de la prevalente en la enfermedad anti MBG idiopática, en la que los anticuerpos circulantes están habitualmente dirigidos contra la cadena alfa 3.