The trichorhinophalangeal syndrome: study of 16 patients in one family.

The trichorhinophalangeal syndrome has recognizable clinical and radiographic features. In most patients, hand radiographs show typical cone-shaped epiphyses which affect predominantly the middle phalanges. Pattern profile analysis may help establish the diagnosis in the absence of typical clinical features of radiographic hand changes. The evolution of hip disease in young and older patients is illustrated and differs from what has been reported previously. Chest and spinal abnormalities, though not well known, are also features of this syndrome.     

Indolent nonfollicular lymphomas: characteristics, treatment, and outcome.

Indolent lymphomas are defined by their low aggressiveness at diagnosis compared with large-cell or T-cell lymphomas: small lymph nodes or blood involvement usually existing for months or years, no B symptoms, and good performance status. However, except in mucosa-associated lymphoid tissue (MALT) lymphoma, the disease is usually disseminated and this indolent presentation does not preclude a good outcome. Indolent lymphomas consist of follicular lymphoma, small lymphocytic lymphoma (SLL), lymphoplasmacytic lymphoma (LPL), marginal-zone lymphoma (MZL), and mantle-cell lymphoma (MCL) as defined in the World Health Organization (WHO) classification. A review of morphologic characteristics, clinical presentation, prognostic factors, and outcome is presented here for each of these entities. Despite the absence of good prospective or retrospective studies in the literature, some orientations for the treatment of these patients are given, as well as some ideas for future studies.     

Recent developments in sexually transmitted diseases: chancroid--epidemiology, diagnosis, and treatment

The most important recent developments concerning chancroid have been in its epidemiology (a tremendous increase in the incidence of the disease in the United States over the past several years), diagnosis (better culture media), and treatment (several newer and older drugs that have been shown to be effective against chancroid in recent studies).     

Splenic marginal-zone lymphoma: a distinct clinical and pathological entity

In the World Health Organization classification system, splenic marginal-zone lymphoma (splenic MZL) is described as an indolent B-cell lymphoma, which generally presents as splenomegaly with involvement of the bone marrow and peripheral blood. Presence of disease in peripheral lymph nodes and extranodal locations is uncommon. Splenic MZL is characterised by micronodular infiltration of the spleen with marginal-zone differentiation; the immunophenotype is usually IgM+ IgD+/- cytoplasmic-Ig-/+ pan B antigens+ CD5- CD10- CD23- CD43-/+ cyclin D1-; and the most common genetic abnormalities are deletions at 7q22-7q32. Most patients with splenic MZL live for a long time but classic prognostic factors cannot distinguish between patients who are likely to have good and poor outcomes. However, immunological events, such as haemolytic anaemia and immune thrombocytopenia, or the presence of a monoclonal component, are significantly associated with shorter survival. Splenectomy is considered the first-line treatment of choice for splenic MZL; it results in only partial remission, but responses are generally sufficient for correcting cytopenia, improving quality of life, and increasing survival.     

Synthesis and antiulcer activity of novel 5-(2-ethenyl substituted)-3(2H)-furanones.

In order to investigate new antiulcer agents, spizofurone 1 (AG-629) was fragmented and reassembled to generate 5-phenyl-2,2-dimethyl-3(2H)-furanone (bullatenone, 2). Because of the antiulcer activity of 2,5-phenyl-substituted 2,2-dimethyl-3(2H)-furanones (3-6) were made and shown to have poor activity. Insertion of an ethenyl link between the furanone and phenyl rings gave 5-(2-phenylethenyl)-2,2-dimethyl-3(2H)- furanone (7). This compound had better activity than 2. Compounds 8-41 were synthesized to evaluate the SAR in 5-(2-ethenyl substituted)-3(2H)-furanones. Electron-withdrawing substituents on the aromatic ring (8, 10, 19, and 20) gave 2-3-fold higher activity. Further increases in the activity were found when the phenyl ring was replaced by heterocyclic nuclei. Compounds that contained a thiophene (29), pyridine (24-26), or quinoline ring (32) had the best activity. Replacement of the methyl group on the furanone ring with a phenyl (34) or p-fluorophenyl (40) substituent in the 2-pyridine series gave compounds with activity that ranked with the best obtained in this study. The best compounds from the above SAR studies were evaluated in the ethanol-necrosis model for duration of cytoprotection action. Compounds 19, 24, and 29, which had the best duration of action, were tested with AG-629 in the acidified aspirin and indomethacin-induced lesion models. Only compound 24 had equivalent activity with AG-629 in both models.