Magnetic resonance imaging of orbital lymphangiomas.
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Biomedical subjects
Publications and source records attributed to R Weiss.
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Six cases of the McKusick-Kaufman syndrome (MKS), including two cases that were diagnosed prenatally, were studied. Review of the 54 previously described cases indicates that postaxial polydactyly and hydrometrocolpos in female patients are the hallmark features of this entity. Other manifestations, such as malformations of gastrointestinal, cardiovascular, and ophthalmic structures, occur less consistently. Affected children require careful medical follow-up. Recurrence of hydrometrocolpos following surgical repair may lead to serious sequelae, such as chronic renal failure. We believe that MKS is a distinct panethnic genetic entity, inherited in an autosomal recessive fashion, and that the diagnosis should be made only in female patients with hydrometrocolpos and polydactyly or in male patients with polydactyly who have an affected female relative.
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Previous experiments have shown that combination of herpes simplex virus (HSV) infection and simulated snuff-dipping in hamster buccal pouches enhances the development of micro-invasive squamous cell carcinoma in cheek pouch epithelium. The effect has now been determined of water-extractable components of snuff (snuff-extract) on the growth and the cell-lysing activity of HSV. Various dilutions of snuff-extract in tissue culture medium significantly inhibited the growth of HSV in Vero cell monolayers by inhibiting the viral DNA replication. Moreover, HSV was inactivated and its cell-lysing activity lost when it was incubated with snuff-extract in cell-free condition. Snuff also had a similar anti-herpetic effect in vivo; HSV infection of pouch tissues followed by simulated snuff-dipping resulted in significant inhibition of viral growth. Thus snuff interferes with the DNA synthesis and cytolytic activity of HSV in vitro and in vivo, and this in turn, may increase its oncogenic capacity.
Oral health problems were identified among 653 residents of 40 long-term care institutions in Vancouver, using a personal interview and a dental examination. Information on jaw function was analysed as part of this investigation to identify associations between the clinical signs of mandibular dysfunction and the complaints of the subjects, their occlusal instability, and the quality of their dentures. The sample population were predominantly female and over 75 years of age, and 42% had some natural teeth. Complaints of jaw dysfunction, usually sore muscles, were heard from 13% of the 488 subjects interviewed. The examination was performed on all of the subjects and revealed that 43% had unstable occlusal contacts, 37% were using loose or unstable dentures, and 20% had one or more clinical signs of jaw dysfunction. Joint noise was the most frequent sign detected and it was heard in a greater percentage of the women than men, while deviation of the mandible during mouth opening was observed with great frequency in the younger age groups. There was no apparent association found between the clinical signs of jaw dysfunction and the complaints of the subjects or the condition of their teeth or dentures. The study demonstrated that complaints and clinical signs of jaw dysfunction, with the exception of joint noises, were uncommon in this population.
In a stratified random sample of 41 long term care (LTC) facilities in Vancouver, 653 residents were chosen to investigate oral health needs and demands for treatment. All of the 603 dentists in the same area were questioned to assess their interest in attending the residents of the institutions. The information from each source was reviewed to identify factors influencing the oral health services to this predominantly elderly and medically compromised population. The majority (60%) of the residents were edentulous and they made infrequent demands on dentists. Two-thirds of those interviewed said that there was nothing wrong with their mouths, but most of those who were aware of a problem wanted it treated, preferably within the institution. They complained about loose or uncomfortable dentures most frequently, and many were dissatisfied with previous dental treatment. The oral mucosal lesions seen on examination were usually symptomless and associated with poor hygiene, while structurally defective dentures and deep carious lesions were not uncommon. The responding 334 dentists indicated that they enjoyed treating elderly patients, 19% had attended an LTC facility, usually to provide an emergency service, and 37% were willing to provide this service if asked. Interest, however, in the service was curtailed by pressures from private practice, concerns about inadequate training and the small demand and poor conditions in the facilities. Although the demand for treatment was not extensive from the residents, they did have problems that were not receiving care.
Three combination chemotherapy regimens each with or without the methanol-extracted residue of bacillus Calmette-Guérin (BCG) (MER) were compared for efficacy. After stratification for disease-free interval and dominant sites of disease, patients were randomized to either CMF (cyclophosphamide [CYC], 100 mg/m2 orally, days 1 through 14; methotrexate [MTX], 40 mg intravenously [IV], days 1 and 8; 5-fluorouracil [5-FU], 500 mg/m2 IV, days 1 and 8), or CAF (CYC, 100 mg/m2 orally, days 1 through 14; doxorubicin [DOX], 25 mg/m2 IV, days 1 and 8; 5-FU, 500 mg/m2 IV, days 1 and 8), or CAFVP (CAF as above plus vincristine [VCR], 1.0 mg/m2 IV, days 1 and 8; and prednisone [PRED], 40 mg/m2 orally, days 1 through 14). Nonspecific immunotherapy with MER was administered in five sites at 100 micrograms or at the lowest tenfold dilution that produced a 1-cm indurated lesion. A total of 432 patients were entered, but 37 were disqualified, leaving 395 evaluable for treatment results and toxicities. One hundred thirty-five evaluable patients were randomized to chemoimmunotherapy until October 28, 1978. One hundred twenty-six evaluable patients were randomized to chemotherapy alone in the same time period. For the entire study, a total of 260 evaluable patients were randomized to chemotherapy. Chemoimmunotherapy patients were compared with the initial 126 chemotherapy patients. Chemotherapy regimens were compared among all 260 patients. Patient characteristics were similar between regimens and between chemotherapy and chemoimmunotherapy treatment groups. For patients on chemotherapy plus MER, there was no significant differences between the regimens for response frequencies: 43%, 41%, and 32%, respectively for CMF, CAF, and CAFVP. The comparable chemotherapy alone group had 36%, 58%, and 63% response, respectively. The response rates, adjusted for chemotherapy regimen, were 52% and 38% (P = .02) for chemotherapy and chemoimmunotherapy, respectively. MER was associated with painful ulcers and fevers. Thus, MER produced toxicity without response or survival benefit and further randomization after October 28, 1978 was to chemotherapy alone. For 260 evaluable patients on chemotherapy alone, the complete (CR) and partial responses (PR) were 37%, 55%, and 58%, respectively for CMF, CAF, and CAFVP. These response rates for CAF and CAFVP were significantly better than CMF (P = .01 and P less than .01, respectively). These comparisons were consistent within subgroupings such as dominant sites of disease.(ABSTRACT TRUNCATED AT 400 WORDS)
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Myospherulosis was diagnosed in a mature pony. Several parent bodies containing many spherules were observed microscopically in biopsy material from an area of cellulitis. The spherules are altered red blood cells that form as the result of prolonged contact with necrotic fat or petrolatum-based ointments. These structures must be differentiated from fungal elements. The recommended treatment is surgical excision.
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At the end of 1984 a collaborative survey was carried out to determine the prevalence of infection with human T cell lymphotropic virus type III/lymphadenopathy associated virus (HTLV-III/LAV) and HTLV-I among 584 recipients of various blood products in Britain at that time. In 204 cases yearly point prevalence figures for infection were also obtained for 1978 to 1983. In 1984, 215 of 315 patients (68%) who had received commercial concentrate for haemophilia A were identified as positive for anti-HTLV-III/LAV as compared with only 18 of 166 patients (11%) given British concentrate alone for this disease. This difference was further emphasised by the yearly point prevalence rates: seroconversion began in 1980 among recipients of commercial concentrate, but not until 1983 did such an instance occur among recipients of British concentrate. Any conclusions must remain speculative, but possibly seropositivity among haemophiliacs may not carry so grave a prognosis as previously thought.
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Recurrent pyelonephritic episodes in children with normal contrast cystograms pose a difficult management problem, since the lack of demonstrable reflux adversely affects the treatment. A total of 10 children with clinical symptoms of pyelonephritis in whom normal contrast cystograms had been performed properly subsequently had significant degrees of reflux detected by isotope cystography. These findings indicate that isotope cystography should be included in the evaluation of patients with pyelonephritis in whom reflux is not confirmed by conventional contrast medium techniques.
Successful renal transplantation was accomplished in a patient with spina bifida and a neurogenic bladder, who previously had been continent after bladder neck reconstruction and institution of chronic intermittent bladder catheterization. Satisfactory bladder emptying and renal functional preservation have been maintained during a 4-year followup.
Sexually transmitted diseases have increased in both prevalence and public concern over recent years; however, current treatment of sexually transmitted diseases remains fragmented among several specialty groups. In this study, dermatologists, who historically were the leaders in the treatment of sexually transmitted disease, were surveyed to assess their training, practice activity, and attitudes toward sexually transmitted disease. An overwhelming majority of dermatologists and training program directors stated that most sexually transmitted diseases should be treated primarily by dermatologists. Factors that restricted dermatologists' involvement in sexually transmitted diseases included inadequate public awareness of dermatology's role in treating these diseases, the limited number of direct patient visits, and limited physician referrals. This study indicates that dermatologists want to increase their role in sexually transmitted disease and favor making the public aware of their interest and ability.
Thirty-one episodes of biopsy-proved acute rejection (R) in 28 patients maintained on cyclosporine did not respond to high-dose steroids and were treated with antilymphocyte globulin (ALG). Cyclosporine was discontinued in all but three during ALG administration. (A) Twenty-four patients received 26 courses of ALG within 90 days of transplant (11 1st R, 15 2nd or 3rd). Seven treatment courses were cut short due to infection (4), ongoing R (2) and a combination of infection and rejection (1). Only 1 of 7 has a functioning graft. Of the remaining 19 full ALG courses (17 patients) (8 1st R, 11 2nd or 3rd), 13 (11 patients) responded (7 1st R, 6 greater than 1st). The remaining 6 patients lost their grafts to ongoing acute rejection. (B) Five patients were treated after 6 months posttransplant; two responded but no grafts currently function. (C) Overall 7 patients developed systemic infection (7 viruses, 1 Candida) with 1 death, and 2 additional patients developed severe thrombocytopenia and leukopenia. Patients responding to their ALG course were restarted on cyclosporine. We conclude that ALG is not as effective in reversing steroid-resistant rejection in patients maintained on cyclosporine as it has been in patients maintained on azathioprine. However, more than 50% of steroid-resistant rejection episodes are reversed.
Thirty-seven patients with refractory ovarian carcinoma or other malignancies principally confined to the peritoneal cavity were treated with a combination ip chemotherapy regimen consisting of cisplatin (100 or 200 mg/m2), cytarabine (1,200 mg/m2), and bleomycin (15 or 2 U/m2) repeated at 28-day intervals. Sodium thiosulfate was simultaneously administered iv to protect against cisplatin-induced nephrotoxicity. While only one of 18 patients (6%) with bulky residual ovarian carcinoma experienced a partial remission, two of six evaluable patients with minimal residual disease experienced surgically defined complete remissions. Local abdominal pain was often severe with the higher dose of bleomycin and was occasionally a problem with the lower dose schedule. Fever was common (greater than 70% of courses), but was reduced in degree by pretreatment with steroids. We conclude that combination ip chemotherapy with this three-drug regimen can result in objective tumor regressions and surgically defined complete remissions in patients with minimal residual ovarian carcinoma who have failed to achieve a complete remission following cisplatin-based iv chemotherapy.