Microalbuminuria and renal morphology in the evaluation of subclinical lupus nephritis.

AIM AND METHOD: In an attempt to evaluate subclinical lupus nephropathy, we analyzed the clinical characteristics, determined the albumin excretion rate (AER) by radioimmunoassay and performed renal biopsy in 30 patients with systemic lupus erythematosus (SLE) who had no clinical signs of renal involvement (no urinary sediment abnormalities, absence of proteinuria, serum creatinine <1.3 mg/dl). All biopsies were classified according to a modified classification proposed by the WHO. RESULTS: Fifteen cases (50%) had mesangial glomerulonephritis (MGN) type IIb, 12 had MGN type IIa and 3 patients showed no changes on light microscopy (LM) or on immunofluorescence (IF) (type I). Anti-IgM-fluorescent deposits were found in 83% of the renal biopsies, being associated with less heavily stained deposits of IgG, IgA and C3. Patients with MGN type IIb showed lower mean age when compared to those of MGN type IIa (26.04 years vs. 36.3 years) (p<0.029); those patients also presented disease duration of 4.8 years and their mean AER was 39.9 microg/min. Six of the patients (6 of 15, 40%) showed positive anti-dsDNA antibodies, in contrast to patients with MGN type IIa who did not show positive anti-dsDNA antibodies (p<0.002). The group with abnormal AER presented lower mean age (p<0.029) and lower C3 levels (p<0.0098) when compared to the group with normal AER. CONCLUSION: The results suggest the high prevalence of MGN type IIb and IgM deposits on IF, despite the paucity of clinical and laboratory data on these patients. Furthermore, there is an association between MGN type IIb and positive anti-dsDNA antibodies and a relationship between abnormal AER and low C3 levels. The level of AER could not determine the presence or absence of renal disease on LM or IF in this population.     

Synthesis of 1-(3,4-dihydroxy-5-nitrophenyl)-2-phenyl-ethanone and derivatives as potent and long-acting peripheral inhibitors of catechol-O-methyltransferase.

A homologous series of novel nitro-catechol structures (7a-7e) were synthesized and tested as inhibitors of the enzyme catechol-O-methyltransferase (COMT). Increasing chain length was found to have significant impact on both brain penetration and duration of COMT inhibition in the rat. Of this series, compound 7b (1-(3,4-dihydroxy-5-nitrophenyl)-2-phenyl-ethanone) was found to exhibit the most potent and selective inhibition of peripheral COMT, with an inhibition profile more similar to entacapone 2 than tolcapone 1 (an equipotent peripheral and central inhibitor) but with much improved duration of action (7b, 70% inhibition and 2, 25% inhibition at 9 h after administration). The effects of structural modifications to 7b on COMT inhibitory profile were investigated, and it is concluded that the carbonyl group and preferably unsubstituted aromatic ring are essential features to maintain prolonged peripheral COMT inhibition. The introduction of the alpha-methylene group, the major structural difference between 7b and 1, would appear responsible for the observed enhancement in selectivity of peripheral COMT inhibition of 7b, which has more limited access to the brain than 1.     

Neurosarcoidosis: therapeutic success with methotrexate

Sarcoidosis affects the nervous system in 5% of cases. Treatment with corticosteroids is usually effective. We describe the case of a patient with neurosarcoidosis in which therapy with prednisone failed whereas the result of methotrexate administration was satisfactory.     

A 10-year median follow-up study after allogeneic stem cell transplantation for chronic myeloid leukemia in chronic phase from HLA-identical sibling donors.

We report long-term outcome in 102 patients with cCML transplanted from an HLA-identical sibling donor from 1982 to 1998. The conditioning regimen was based on cyclophosphamide associated with either total body irradiation (TBI) (37 patients) or with busulfan (63 patients). Graft-versus-host disease (GvHD) prophylaxis consisted of cyclosporin and methotrexate in the majority of the patients. Fifteen year overall survival was estimated at 53% (95% confidence interval (CI), 44-65) with a plateau after 2.5 years. Long-term survival was adversely affected by: longer time from chronic myeloid leukemia (CML) diagnosis to transplantation, older age at time of transplantation and GvHD (acute grade III-IV or chronic extensive). The main cause of death was infection, related to GvHD in 69% of patients. Splenectomy also significantly increased the risk of bacterial infection. 15-year relapse was estimated at 8% (95% CI, 0.1-14). Late malignancies occurred in seven patients, four of whom had an invasive cancer. Other frequent late complications included cataracts, psychological depression, osteonecrosis and hypothyroidism. These complications were more frequent following splenectomy, TBI and in patients with chronic extensive GvHD. We conclude that allogeneic transplantation with a related donor can cure more than half of CML patients in chronic phase, although physicians should be alert to long-term complications.     

Abnormally increased iron concentration in basal ganglia in Shy-Drager syndrome. MR imaging and autonomic study.

Report of an early case of Shy-Drager syndrome in a 67 year-old woman patient. Autonomic failure was diagnosed by functional evaluation as well as laboratory tests. MR imaging disclosed a prominent putamina hypodensity in T2-weighted images at high field strength due to iron increased depositing in this basal ganglia. MR imaging evidences confirm Shy-Drager syndrome diagnosis, and contributes for differential diagnosis of idiopathic hypotension (pure autonomic failure) in special in SDS early cases.     

Abdominal ultrasonography in hepatolenticular degeneration. A study of 33 patients

The ultrasonographic study of 33 patients with hepatolenticular degeneration showed the following main changes: disorders of the hepatic echotexture (29 cases), changes of the splenic dimensions (21), liver shrinkage (10), cholelithiasis (8), hepatomegaly and ascites (1). The disorders of liver echotexture exhibited different patterns, from slight to severe changes of the hepatic echogenicity, associated with anatomic distortions of the liver, such as alterations of outline and decrease of dimensions. Liver shrinkage was always accompanied by splenomegaly. The scarcity of hepatomegaly may be explained by the fact that the ultrasonography was performed after the onset of treatment with penicillamine, or by other factors still unknown, copper perhaps possessing a stronger fibrogenetic action than other hepatotoxic agents. Cholelithiasis was very frequent in females (6 of 13 patients), its incidence tending to increase with age. Regarding males there was no increase when the incidence was compared to the general population. The two sole males were young, an unusual finding in normal men.     

Ecthyma‐like phaeohyphomycosis caused by Cladosporium cladosporioides

A case of cutaneous phaeohyphomycosis caused by Cladosporium cladosporioides in a 50‐year‐old housewife is described. The clinical presentation was an ecthyma‐like crusted lesion on the back of her left hand. Scanning electron microscopy of the culture showed the conidiophores and the limoniform or ellipsoidal conidia, with a slightly verrucous surface. The lesion was removed surgically, with no relapses after 6‐month follow up.