[Are preventive guidelines for kidney failure observed in patients treated with ACEI/ARA II?].
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Biomedical subjects
Publications and source records attributed to F López García.
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The NMR structures of the recombinant human prion protein, hPrP(23-230), and two C-terminal fragments, hPrP(90-230) and hPrP(121-230), include a globular domain extending from residues 125-228, for which a detailed structure was obtained, and an N-terminal flexibly disordered "tail." The globular domain contains three alpha-helices comprising the residues 144-154, 173-194, and 200-228 and a short anti-parallel beta-sheet comprising the residues 128-131 and 161-164. Within the globular domain, three polypeptide segments show increased structural disorder: i.e., a loop of residues 167-171, the residues 187-194 at the end of helix 2, and the residues 219-228 in the C-terminal part of helix 3. The local conformational state of the polypeptide segments 187-193 in helix 2 and 219-226 in helix 3 is measurably influenced by the length of the N-terminal tail, with the helical states being most highly populated in hPrP(23-230). When compared with the previously reported structures of the murine and Syrian hamster prion proteins, the length of helix 3 coincides more closely with that in the Syrian hamster protein whereas the disordered loop 167-171 is shared with murine PrP. These species variations of local structure are in a surface area of the cellular form of PrP that has previously been implicated in intermolecular interactions related both to the species barrier for infectious transmission of prion disease and to immune reactions.
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Pulmonary lymphangioleiomyomatosis (LAM) is a rare, serious, chronic disease whose etiology is unknown and which affects young women almost exclusively. It produces typical clinical and radiological characteristics. Diagnosis is confirmed on finding histological evidence of irregular proliferation of the smooth muscle cells of the lungs. Treatment is not usually effective and there is rapid worsening of the pulmonary function leading to serious respiratory failure which often results in the patients death. We present two cases of LAM in women, one of whom was 33 and the other 38. In the first case the patient had all the pulmonary findings described in the literature, while the second also had bilateral renal angiomyolipomas. We discuss the histological, radiological and clinical characteristics and evolution of both cases, as well as the treatment given. We also review the literature. It is concluded that LAM should be suspected in young women who have dyspnea, cough and/or hemoptysis and an interstitial radiological pattern, especially when associated with a pleural effusion or pneumothorax.
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