Taiwan yi xue hui za zhi. Journal of the Formosan Medical Association最新文献

Multiple myeloma has rarely been reported in patients with ankylosing spondylitis. We observed a patient with a 20-year history of ankylosing spondylitis, who subsequently developed IgA myeloma. This association may not be simply coincidental. It has been proposed that the protracted stimulation of immunocytes by inflammatory lesions on the mucosal surfaces of the gastrointestinal, respiratory, and biliary tracts, where lymphocytes are already committed to IgA production, may be implicated in the pathogenesis of IgA myeloma in some patients. Ankylosing spondylitis is a chronic inflammatory disease, probably resulting from the interaction of a genetic predisposition involving HLA-B27 with an environmental event such as enteric bacterial infection. We propose that ankylosing spondylitis and IgA myeloma occurring concomitantly in our patient implies a possible pathogenetic relationship. In ankylosing spondylitis, persistent reticuloendothelial stimulation, due to chronic subclinical gastrointestinal infection, may lead to IgA-producing plasma cell activation and proliferation, and subsequent IgA myeloma development.

Twenty-four tuberculoid (T)-type and 31 lepromatous (L)-type leprosy patients from Taiwan Provincial Lo-Sheng Leprosarium were enrolled in this study. Twenty-six age- and sex- matched normal subjects were also studied as a control group. The evaluation of their general and specific humoral immunity included B-cell subpopulations, 3 major classes of immunoglobulin (G, A and M) and antibodies in the IgG class against lepromin suspension and Bacillus Calmette-Guerin (BCG) sonicate. T-type patients showed a larger B-cell percentage than L-type patients (p less than 0.01). In general, patients with leprosy, both T and L types, had higher serum immunoglobulin levels than the control group. T-type patients showed greater antibody levels than the control group (p less than 0.05 for anti-lepromin and p less than 0.0001 for anti-BCG). L-type patients demonstrated a higher anti-BCG IgG level than the control group (p less than 0.0001). The level of anti-BCG IgG was more frequently above the cutoff level than that of anti-lepromin IgG in leprosy patients (p less than 0.01 for T, p less than 0.005 for L). In conclusion, humoral immunity is not impaired in leprosy patients. Discrepancies for T- and L-type patients among B-cell subpopulation, serum immunoglobulin levels and specific antibody levels reflect different aspects of cell-mediated immunity impairment. Though leprosy patients had elevated anti-BCG IgG levels, it is impossible to differentiate L- and T-type patients; specific antigens are needed for serodiagnosis of leprosy patients in Taiwan.

Oocyte retrieval for in vitro fertilization and embryo transfer (IVF/ET) through the cul-de-sac under the guidance of a vaginal sector scanner was performed on 12 patients and compared with the previous laparoscopical oocytes retrieval for in vitro fertilization. Using a transvaginal sector scanner with puncture guide line, the needle was introduced through the posterior fornix of the vagina into the ovary. Vaginal sonography obviously has more benefits than the conventional laparoscopic method as the patients can be rid of the risk of repeated laparoscopy, can have less anesthetic and it is not necessary to have CO2 pneumoperitoneum, etc. When this method is compared with the transvesical approach, the transvaginal method does not require a full bladder, and has a shorter distance between the transducer and the follicles so it can provide better patient acceptability and better sonographic images which can increase the accumulated success rate. The recovery rate of oocytes was 8.83 per cycle which equals a mean recovery rate of 84%. The clinical pregnancy rate was 25% per retrieval cycle and 33.3% per embryo transfer. The results were not worse than those by laparoscopy and the transvesical method. This procedure is less time-consuming and has less blood loss than the other methods. We believe that, after a period of progression, IVF/ET can be performed in an outpatient setting with decreased risks and expenses of laparoscopy and general anesthesia.

To assess whether ultrasonography is valuable in the diagnosis and management of patients with complete chest X-ray opacification of hemithorax, chest ultrasonography was conducted in 65 patients. There were 54 patients (83.1%) with massive pleural effusion with or without other pathology, six (9.2%) with tumors, two (3.1%) with organized fibrothorax, two (3.1%) with consolidation or atelectasis and one (1.5%) with pseudocyst of the pleura in the sonographic diagnosis. Sonography differentiated effusion from tumor, consolidation and fibrosis. The effusion showed a hypoechoic area with floating fibrin strands and changed shape on respiration. The tumor revealed a homogeneous iso- or hypoechoic mass with a sharp margin. Consolidation or atelectasis showed a lobar or segmental area of heterogeneous isoechoic density with air-bronchogram. The pleural showed an area of hypoechoic density without floating fibrin or a changed shape on respiration. In 24 (37%) patients we could demonstrate complex pathology addition to the pleural effusion. Twenty-one patients had a hidden tumor beneath the effusion. Three patients had consolidation or atelectasis with effusion. In 13 patients, aspiration biopsy was performed under ultrasonic guidance from the tumor and the histological diagnosis was confirmed in 12 patients. We conclude that ultrasonography is a very useful, noninvasive tool in the differential diagnosis of complete chest X-ray opacification of hemithorax. Complex pathology is also easily detected by sonography. Sonographic findings can also be used as a guideline for an appropriate approach to the patient's management.

From July 1978 to June 1987, non-Hodgkin's lymphoma was diagnosed in 667 patients at Chang Gung Memorial Hospital. Forty-eight (7%) patients presented primarily with involvement of Waldeyer's ring; their provisional clinical stage was I or II. The tonsils represented the most frequent site of involvement within Waldeyer's ring. A work-up showed 33.3% of patients with stage I, 50% with stage II, 8.3% with stage III and 8.3% with stage IV. All the patients had diffuse lymphomas. Diffuse large cell (46%) and immunoblastic lymphomas (23%) were the most common histologic subtypes. Eight patients received radiotherapy alone, 39 patients were treated with combination chemotherapy with or without local radiation and one patient was lost to follow-up. Eleven of 16 stage I (69%) and 18 of 23 stage II (78%) patients achieved a complete remission. The median survival for stage I patients was over 54 months and the median survival of stage II was 72 months. There was no significant difference between the stage I and II patients with lymphoma of Waldeyer's ring with respect to the complete remission rate and survival. Stage I patients had a relapse-free survival rate of 55% at 4 years, similar to that (49%) of stage II patients at 8 years. We recommend that chemotherapy be used as primary therapy for those patients with localized aggressive lymphoma of Waldeyer's ring.

The choice of a valved conduit or a valve in the right ventricular outflow tract remains a problem. We propose to reconstruct the pulmonary valve inside the patient's own pulmonary trunk by autologous pericardium in tri-semilunar fashion with concomitant formation of the sinus of Valsalva and commissure. This operation was carried out on a 7-year-old girl suffering from Fallot's tetralogy with an absent pulmonary valve. The ventricular septal defect was repaired and the pulmonary valve reconstructed as above. The postoperative cross-sectional echocardiogram, color Doppler echocardiogram and angiogram showed a pliable tri-semilunar valve across the pulmonary outflow tract without regurgitation. It is concluded that firstly, the sinus of Valsalva is indispensable for a stentless semilunar valve to be competent; secondly, reconstruction of the semilunar valve inside the pulmonary trunk by autologous pericardium is recommended for the absent pulmonary valve syndrome, but the long-term fate of this pericardial valve awaits further evaluation.

In November 1985, a 5-year-old Chinese girl presented to the Dermatological Department of Chang Gung Memorial Hospital, Taipei, having scaly erythematous plaque with mild itching on her right upper eyelid. Skin biopsy and fungal cultures were performed after failure of initial topical steroid therapy. The histopathology revealed many acute and chronic inflammatory cells infiltrating the dermis and H & E stain revealed some foamy vacuolated spores; P.A.S. and Gomori's methenamine stain also showed many spores and sporangia containing endospores. Lactophenol cotton blue and methylene blue wet mount preparations were made from the colony growing on Sabouraud's agar. Microscopically, these showed many round or oval spores and endospore-containing sporangia, corresponding with the histopathology. This microorganism grew as a milky white yeast-like colony on Sabouraud's dextrose agar, blood agar, EMB, Tween 80 cornmeal agar, chocolate agar, MacConkey agar and brain heart infusion with sheep RBC agar. On Pagano-Levin medium, the colony became deep red in color and in the thioglycollate broth tube culture, it was suspended on the upper layer as a whitish ring-form of granules. The microorganism showed no urease activity. In the assimilation tests, there were positive reactions to glucose, galactose, trehalose, fructose, mannose and glycerol, and negative reactions to maltose, xylose, raffinose, sucrose, lactose, cellabiose, n-propanol, etc. The electronmicroscopic examination of the colony revealed sporangium containing spores and characteristic dense body and plastids in the spores.(ABSTRACT TRUNCATED AT 250 WORDS)

Hypereosinophilic syndrome (HES) with Löffler's endomyocarditis is a rare disease with poor prognosis. Clinically, it mainly affects young adults, characterized by prolonged idiopathic blood eosinophilia, cardiopulmonary symptoms and hepatosplenomegaly. Pathological features consist of ventricular mural thrombi, marked fibrotic thickening of endocardium with scarring of myocardium, and multiorgan eosinophilic infiltration. We report a 2-year-old girl with idiopathic HES associated with Löffler's endomyocarditis. The girl was noted to have constitutional symptoms, cardiomegaly, and hepatosplenomegaly. Her total eosinophil count reached 150,000/microliters. She had no history of allergic diseases. The microbiological studies were negative. Echocardiography suggested thickened endocardium, mitral stenosis and regurgitation. In spite of intensive medical management, the girl died of refractory congestive heart failure. Autopsy revealed thick fresh mural thrombi involving the biventricular inflow tracts, inflammation and fibrosis of the endomyocardium, systemic thromboembolism and multiorgan eosinophilic infiltration. The pathology and pathogenesis of the disease are discussed and the literature is reviewed.

Epidemiology of gonococcal infection in Taiwan was investigated. Six hundred twelve isolates from 7 cities in 1983-1984 were examined for auxotyping by Hendry and Stewart's method, for serotyping by coagglutination of monoclonal antibodies with the antigenic specificity of gonococcal protein I molecules and for plasmid profile by the alkaline quick method. The results are described below: (1) Thirty-six auxotypes were found. Prototropic (Prototype) 45.4% (284/612) and Proline type (Pro) 38.7% (237/612) were the two dominant auxotypes. Arg- Hyp- Ura- which was thought to be correlated with disseminated gonococcal infection was not found. (2) Fifty-five percent penicillinase producing Neisseria gonorrheal (PPNG) were prototype and 48.8% non-PPNG were Pro. (3) Sixteen serotypes were identified among 56 strains. There was 17.8 (10/56 which belonged to the IA group and 82.2% (46/56) to the IB group. (4) Five molecular weight plasmids were found. They were 2.6 Mdal, 3.05 Mdal, 4.7 Mdal, 7.8 Mdal and 24.5 Mdal. (5) There were 7 plasmid profiles including 2.6 Mdal (26.4%); 2.6 Mdal +24.5 Mdal (23.2%); 2.6 Mdal+7.8 Mdal+24.5 Mdal (0.4%); 2.6 Mdal+4.7 Mdal (8.9%); 2.6 Mdal+4.7 Mdal+24.5 Mdal (38.2%); 2.6 Mdal+4.7 Mdal+24.5 Mdal (0.2%) and 2.6 Mdal+3.05 Mdal+24.5 Mdal (2.4%). (6) All the isolates harbored cryptic 2.6 Mdal plasmid. Eighty three percent of the PPNG and 48.6% of the non-PPNG harbored 24.5 Mdal plasmid. Twelve out of 305 PPNG isolates were "Toronto" type 3.05 Mdal plasmid. Others were "Asian" 4.7 Mdal plasmid. There were no "African" type 3.2 Mdal plasmids found.

We report 11 cases of acute graft-versus-host disease (GVHD); 10 occurred after bone marrow transplantation (BMT) and one following transfusion of nonirradiated whole blood in a patient with severe combined immunodeficiency (SCID). According to the Seattle Classification, 5 cases were of Grade III, 2 Grade II, and 4 Grade I. The skin rash developed between the 6th to 47th day (22 +/- 12 day) after transplantation or transfusion, and was usually manifested initially as erythematous macules or papules in the neck or upper chest. The rash resolved in scaling or became purpuric, pigmented or erythrodermic in severe cases. The grading of skin pathology correlated well with clinical severity of GVHD. Four of the five who had advanced GVHD (Grade III) died. In all 4 cases but one (the SCID case), the skin pathology showed extensive basal vacuolization and multiple dyskeratotic cells. None of the others with mild or focal basal vacuolization and few dyskeratotic cells progressed into advanced GVHD. Three of the 5 cases with advanced GVHD received an underdose of cyclosporin A. These results suggest that severe GVHD carries a poor prognosis and an adequate dose of cyclosporin A is important in alleviating the severity of GVHD and reducing the mortality. The sero-virologic study performed during the skin rash period was negative, suggesting that the skin rash following BMT is mainly caused by GVHD and always precedes intestinal or hepatic manifestations.