RESUMO
Most cases of nephrotic syndrome following stem cell transplantation (SCT) occur 6 months after SCT. The patients are treated with immunosuppressive therapies; however, in some cases treatment is not effective. We used enalapril, an angiotensin-converting enzyme inhibitor (ACEI) and candesartan, an angiotensin II receptor blocker (ARB), for the control of proteinuria in a case of immunosuppressive treatment (IST)-resistant nephrotic syndrome. A 15-year-old boy with acute lymphoblastic leukemia underwent allogeneic peripheral blood SCT from a completely HLA-matched sibling after completion of a conditioning regimen composed of 12-Gy doses of total-body irradiation, 600 mg/m2 thiotepa, and 140 mg/m2 melphalan. Twenty-eight months after SCT, minimal-change nephrotic syndrome was diagnosed on the basis of biopsy findings. Although neither cyclosporine (trough level, 100-150 ng/mL) nor corticosteroid was effective, proteinuria disappeared 2 months after the beginning of treatment with tacrolimus (trough level, 13-20 ng/mL), and remission was maintained for 23 months. Nephrotic syndrome recurred, however, and was resistant to tacrolimus. Findings at the second renal biopsy revealed membranous nephropathy. An ARB (candesartan, 4 mg/ day) in combination with an ACEI (enalapril, 5 mg/day) was started. Proteinuria improved within 2 weeks. We suggest that ARB combined with ACEI can be used to control proteinuria in patients with IST-resistant nephrotic syndrome after SCT.
Assuntos
Inibidores da Enzima Conversora de Angiotensina/administração & dosagem , Anti-Hipertensivos/administração & dosagem , Benzimidazóis/administração & dosagem , Síndrome Nefrótica/tratamento farmacológico , Transplante de Células-Tronco , Tetrazóis/administração & dosagem , Adolescente , Compostos de Bifenilo , Resistência a Medicamentos/efeitos dos fármacos , Humanos , Imunossupressores/administração & dosagem , Masculino , Síndrome Nefrótica/etiologia , Síndrome Nefrótica/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Leucemia-Linfoma Linfoblástico de Células Precursoras/patologia , Leucemia-Linfoma Linfoblástico de Células Precursoras/terapia , Proteinúria/tratamento farmacológico , Proteinúria/etiologia , Proteinúria/patologia , Transplante de Células-Tronco/efeitos adversos , Fatores de Tempo , Transplante HomólogoRESUMO
A 9-month-old girl developed subacute limited adduction of the left eye, presenting with blepharoptosis. An orbital magnetic resonance imaging (MRI) 2 months after the onset revealed swelling of the left lateral rectus muscle, with increased intensity on T2-weighted images with fat saturation, which was enhanced with gadolinium. She was diagnosed with idiopathic orbital myositis based on history, physical examination, and MRI findings. Swelling of the left lateral rectus muscle was partially reduced by pulse steroid therapy. This is the first reported case of an infant orbital pseudotumor with clinical and MRI findings consistent with subacute orbital myositis. We propose that a fibrotic change of the orbital muscle may occur during a subacute course and would be incompletely responsive to steroid therapy.