Margarida Guedes

A case of hepatopulmonary syndrome solved by mycophenolate mofetil (an inhibitor of angiogenesis and nitric oxide production)

The autoimmune lymphoproliferative syndrome (ALPS) is a rare, multisystemic disease, caused by an inherited defect in the Fas apoptotic pathway, characterized by a chronic non-malignant lymphoid accumulation and autoimmune manifestations. Lung, kidney, liver, and gut infiltration is described in severe, multisystemic cases; so far there has been no description of hepatopulmonary syndrome (HPS), for which orthotopic liver transplantation (OLT) is currently the only known effective treatment. A teenage boy, diagnosed with ALPS at 4years of age (lymph nodes enlargement, splenomegaly, immune cytopenias), was stable until 13years of age, when he developed insidious hypoxemia (PaO 2 =46.7mmHg). He was diagnosed with HPS on the basis of hypoxemia, non-cirrhotic liver disease with portal hypertension, and pulmonary vascular dilatation (intrapulmonary shunt=45%). He was treated with oxygen (maximum 6L/min), prednisolone and sirolimus. There was significant regression of all manifestations of ALPS, except for the pulmonary symptoms, therefore, after evaluation in referral centers in England, OLT was proposed. Since he was to undergo major surgery, sirolimus, which has wound-healing problems, was switched to mycophenolate mofetil (MMF). Following this change, we observed a huge improvement in pulmonary symptoms and reduction of oxygen needs. The intrapulmonary shunt decreased from 45% to 0% in less than a year, and it has not changed since (18months after complete normalization), on continued treatment with MMF. Indication for OLT was suspended. In the last year, lymphoid proliferation increased again, with huge splenomegaly, but no recurrence of HPS. The addition of sirolimus to MMF produced again a rapid resolution of lymphoid proliferation. The dramatic and unexpected regression of HPS may have been due to inhibition of angiogenesis and nitric oxide (NO) production by MMF (both important pathways/mediators in HPS pathogenesis). Therefore, we propose to perform clinical trials with MMF, and/or other angiogenesis and NO inhibitors, on a long-term treatment basis, to confirm their potential as a valid alternative to medical treatment of HPS.

Characterization of the mangrove plant community and associated sediment of Todos os Santos Bay, Bahia, Brazil

This work presents the phytosociological characteristics of Todos os Santos Bay mangrove communities in Brazil as well as the chemical composition of the leaves of the three mangrove species and their associated sediment. For the qualitative, phytosociological investigation, the contiguous parcel method was used at five sites associated with petroleum extraction (Site 4, Fontes Island), transportation (Site 2, Madre de Deus and 3, Pati Island), and refining (Site 1, Mataripe). Each site was divided into seven 10 x 10 m parcels. Results indicated that the Todos os Santos mangrove ecosystem has a fringe physiognomy with low bearing, containing the obligate species, Laguncularia racemosa, Rhizophora mangle, and Avicennia schaueriana, the former being the dominant species. The sediment presented a predominantly sandy texture and the average levels of the elements P, Ca, K, Mg, Na, Cu, Zn, Mn, and Fe were within the normal range of variation for this kind of environment. The chemical analysis of leaves revealed significant differences among the species, although Rhizophora mangle and Laguncularia racemosa demonstrated a great similarity in their average concentrations of the minerals N, P,K. Ca, Mg, S, Cu, Fe, Mn, and Zn. For the three species, the individuals collected at Mataripe (Site 1) site, presented significantly higher (p<0.05) concentration of the elements N, P,and Cu, when compared to the others sites, thus suggesting the influence of the oil refinery on mangrove plants.

Hidroxicloroquina na hemossiderose pulmonar idiopática – Caso clínico

Resumo Os autores apresentam o caso de uma crianca com hemossiderose pulmonar idiopatica grave, que, apos ter iniciado tratamento com hidroxicloroquina, apresentou alteracao do seu curso clinico, com melhoria significativa e duradoura. A eficacia desta terapeutica e salientada. Reportam ainda a ocorrencia de exacerbacao clinica, com hemoptise, apos administracao de vacina antigripal.

Pneumonite intersticial crónica

RESUMO A patologia pulmonar intersticial compreende um grupo de doencas cronicas caracterizadas por alteracoes das paredes alveolares e perda das unidades funcionais alveolocapilares. Sao doencas raras nas criancas, na sua maioria de causa desconhecida e revestindo-se habitualmente de uma elevada morbimortalidade, dada a pouca eficacia da terapeutica actualmente disponivel. Os autores descrevem o caso clinico de uma crianca de 3 anos, previamente saudavel, que no contexto de uma infeccao respiratoria desenvolve um quadro de sibilância e insuficiencia respiratoria grave, na investigacao do qual e diagnosticada uma pneumonite intersticial cronica. Foram tentadas diversas terapeuticas (corticoterapia sistemica, hidroxicloroquina, N-acetilcisteina) sem melhoria evidente. REV PORT PNEUMOL 2004; X (3): 243-251