Mitochondrial neurogastrointestinal encephalomyopathy associated with progressive hearing loss.

OBJECTIVE: We report a rare case of mitochondrial neurogastrointestinal encephalomyopathy with hearing loss. CASE REPORT: A 46-year-old woman presented with a three-year history of progressive, bilateral hearing loss and tinnitus. She had been suffering from unexplained abdominal pain and diarrhoea for 20 years. When first seen, her otoscopic findings were normal, and pure tone audiometry showed mild and moderate hearing loss in her right and left ears, respectively. She also had: bilateral ophthalmoparesis, neck and limb muscle weakness, and hypoactive deep tendon reflexes on neurological examination; diffuse leukoencephalopathy on magnetic resonance imaging of the brain; and markedly reduced leukocyte thymidine phosphorylase activity. On the basis of these findings, the patient was diagnosed with mitochondrial neurogastrointestinal encephalomyopathy. CONCLUSION: Mitochondrial neurogastrointestinal encephalomyopathy is an autosomal recessive disease caused by mutation of the thymidine phosphorylase gene, and is characterised by ophthalmoparesis, peripheral neuropathy, leukoencephalopathy, gastrointestinal symptoms and abnormal mitochondria in muscle cells. Current advances in genetic research may reveal a higher prevalence of mitochondrial disorders than had previously been thought. Otolaryngologists should be aware of mitochondrial neurogastrointestinal encephalomyopathy and other rare genetic disorders when managing patients with progressive hearing loss.

Myxofibrosarcoma of the neck.

We report a rare case of myxofibrosarcoma arising in the neck. A 55-year-old man presented with a two-year history of left-sided, painless, submandibular swelling. Computed tomography and magnetic resonance imaging (MRI) revealed an 80 x 35 mm, well defined, lobulated, submandibular tumour extending to the parapharyngeal space. The tumour showed uniformly low intensity and marked hyperintensity in T1- and T2-weighted MRI scans, respectively, and was scarcely enhanced by gadolinium. A tentative diagnosis of lymphangioma or plunging ranula was made, and the patient underwent local injection of OK-432, which proved to be ineffective. Resection of the tumour was then performed via a transcervical approach. The tumour was histopathologically and immunohistochemically diagnosed as a low-grade myxofibrosarcoma. The patient's post-operative clinical course was uneventful, and the patient was free of disease 27 months after surgery. The pathology, clinical characteristics and treatment of myxofibrosarcoma are bibliographically reviewed.

Evaluation of bedmaking-related airborne and surface methicillin-resistant Staphylococcus aureus contamination.

The number of airborne methicillin-resistant Staphylococcus aureus (MRSA) before, during and after bedmaking was investigated. Air was sampled with an Andersen air sampler in the rooms of 13 inpatients with MRSA infection or colonization. Sampling of surfaces, including floors and bedsheets, was performed by stamp methods. MRSA-containing particles were isolated on all the sampler stages-stage 1 (>7 microm diameter) to stage 6 (0.65-1.1 microm). The MRSA-containing particles were mostly 2-3 microm diameter before bedmaking and >5 microm during bedmaking. The number was significantly higher 15 min after bedmaking than during the resting period, but the differences in counts after 30 and 60 min were not significant. MRSA was detected on many surfaces. The results suggest that MRSA was recirculated in the air, especially after movement. To prevent airborne transmission, healthcare staff should exercise great care to disinfect inanimate environments. Further studies will be needed to confirm the level of MRSA contamination of air during bedmaking and establish measures for prevention of airborne transmission.

[Clinical significance of the Streptococcus milleri group in peritonsillar abscesses].

Few researchers have microbiologically studied peritonsillar abscesses in detail, and their results have been conflicting. Although Streptococcus pyogenes (Group A beta-streptococcus) is commonly considered an important pathogen in this infection, recent studies have demonstrated the recovery of many other streptococci mainly consisting of alpha-streptococci. Few studies have identified these streptococci at the species level, however. We studied details of bacteriology in 31 cases of peritonsillar abscess treated between 1991 and 2000. The Streptococcus milleri group was most frequently isolated (25.8%), followed by Eikenella corrodens (9.7%), Staphylococcus aureus (6.5%), and S. pyogenes (3.2%). The S. milleri group, consisting of 3 species of Streptococcus constellatus, S. intermedius, and S. anginosus, forms part of the normal flora most commonly found in the mouth, throat, gastrointestinal tract, and genital tract. These species have become known as an important pathogen in abscess disease but little attention has been paid to their role in peritonsillar abscesses. To adequately culture the S. milleri group, incubation in air containing carbon dioxide or in an anaerobic condition is required, and then the differentiation of the 3 species requires the biochemical reactivity tests. Since hemolytic patterns of the S. milleri group vary, we studied the population of alpha-, beta-, and gamma-hemolytic strains among 36 strains of this group. We found 32 (88.8%) to be alpha-hemolytic. Although not all alpha-hemolytic strains belong to the S. milleri group, a considerable number of this group could be missed among alpha-streptococci isolated from the peritonsillar abscess. As antibiotics began being used widely, normal flora such as the S. milleri group may have become an important pathogen in peritonsillar abscesses due to an imbalance between organisms and host defense.

[A case of chronic masticator space abscess].

Different neoplasms and infections are known to involve the masticator space, but pathological diagnosis and treatment of these lesions are not always simple due to anatomical complexity. We treated a 66-year-old man with an abscess in the nasopharyngeal masticator space. Physical and CT findings resembled those of neoplastic lesion because the onset dated back 5 years and the patient was lacking in notable signs of infection. Surgery through the maxillary sinus to the lesion enabled us to confirm its pathology and drain pus, with subsequent cure. We noted periodontal infection of the mandibular molars accompanied with osteomyelitis as a cause of this abscess, so infected molars were extracted 13 days after surgery. The infection had spread upward along the mastication muscles, resulting in an abscess in both the upper masseter muscle and the lower temporalis muscle. Based on a review of the literature, most abscesses in the masticator space originate from the mandibular molar, while the most impressive physical finding varied between the submandibular region and temporal fossa, as did its acute or chronic clinical course. Such clinical manifestations appear to reflect the pattern of infection spread along the muscles of mastication and a pattern involving adjacent spaces. We emphasize diagnostic significance when assessing findings for each mastication muscle and mandibular bone depicted using computed tomography, magnetic resonance imaging, and bone-scan technetium.

[A case of pulmonary plasma cell granuloma with a cavity].

Plasma cell granuloma of the lung is an uncommon and non-malignant neoplasm that may present difficulties in both diagnosis and management. We report a 52-year-old male who was admitted to our hospital due to fever and an abnormal shadow on chest roentgenography. Chest computed tomography revealed a tumor shadow with a cavity in the left upper lobe. However, neither bronchofiberscopy nor serum examinations suggested a diagnosis. Video-assisted thoracoscopic surgery (VATS) was performed and postoperative pathological examination of the resected specimen showed plasma cell granuloma. The postoperative course was uneventful. No recurrence was observed 10 months after the operation. As plasma cell granuloma of the lung is histologically benign, surgery should be performed to preserve the maximal residual lung with no lesion. VATS is the method of choice for treatment of pulmonary plasma cell granuloma.

Primary malignant fibrous histiocytoma of the ascending colon: report of a case.

We report herein an unusual case of primary malignant fibrous histiocytoma (MFH) of the ascending colon. A 47-year-old man was admitted to our hospital for further investigations following the discovery of a mass in the right lower quadrant of the abdomen during a medical checkup. Abdominal ultrasonography (US) and computed tomography (CT) demonstrated a mass extending to the right lateral side from the ascending colon. At laparotomy, a tumor was found originating in the ascending colon and infiltrating the right lateral peritoneum. A right hemicolectomy and partial peritoneal dissection were performed followed by an ileotransverse colostomy reconstruction. The resected specimen contained a tumor measuring 7 x 5 x 4 cm, the cut surface of which was yellowish white, and the mucosa of the colon was intact. Based on histological and immunohistochemical inspection, the tumor was diagnosed as MFH of the ascending colon. We reviewed the total 18 known cases of colorectal MFH documented in the literature including our case. After surgery, 4 of 17 patients died of local recurrence, all within 42 months, indicating that early and complete excision of tumor is essential to achieve cure.

Efficacy of combining donor-specific presensitization with a simultaneous single injection of tacrolimus on pulmonary allografts.

BACKGROUND: We investigated the effects of combining donor-specific presensitization with a simultaneous injection of tacrolimus by use of a fully allogeneic rat lung allograft model. METHODS: Lungs from Brown Norway donor rats were orthotopically transplanted into Lewis recipient rats. Seven days before transplantation, allograft recipients received a transfusion of donor splenocytes (1 x 10(8) cells, intravenously), tacrolimus (3 or 1.5 mg/kg, intramuscularly), or a combination. No posttransplantation immunosuppression was given. In the transplantation study, graft survival was evaluated, and histologic characteristics of acute rejection were graded. In the in vitro studies, mixed lymphocyte reaction assays were used to investigate the effects of pretreatment on immune response. RESULTS: The graft survival evaluation disclosed that untreated rats rejected the allografts at 4.6 +/- 0.2 days. Donor splenocyte transfusion alone accelerated the graft rejection (3.3 +/- 0.2 days). Tacrolimus (3 mg/kg) alone moderately improved the graft survival (8.7 +/- 0.6 days). When donor splenocyte transfusion was combined with tacrolimus, graft survival was significantly increased to 29.6 +/- 12.0 days. In a mixed lymphocyte reaction assay, peripheral blood lymphocytes obtained from animals pretreated with donor splenocyte transfusion alone seemed to be hyperresponsive against the donor lymphocytes. In contrast, donor splenocyte transfusion with tacrolimus significantly suppressed the proliferative response against the donor lymphocytes but not against third-party lymphocytes obtained from naive Wistar King A rats. CONCLUSION: These data demonstrate that donor-specific presensitization with a simultaneous single injection of tacrolimus prevented both sensitization and graft rejection and induced donor-specific unresponsiveness.

The effect of combination therapy with EPC-K1 and low-dose cyclosporine to pulmonary allograft after rat lung transplantation.

BACKGROUND: EPC-K1, a diester of alpha-tocopherol and ascorbic acid, has a hydroxyl radical scavenging effect and also has antiinflammatory properties through its phospholipase A2 inhibitory effect. With a view to decreasing the total dose of cyclosporine, the effect of a combination of EPC-K1 and cyclosporine on rejection was investigated by use of a rat orthotopic left lung transplantation model. METHODS: Orthotopic left lung transplantation was performed with brown Norway rats as donors and Lewis rats as recipients. Recipients were assigned to one of four experimental groups. Control group animals were given no immunosuppression. The EPC-K1 group received continuous intraperitoneal infusion of EPC-K1 (5 mg/kg/day) by osmotic pump on postoperative days (POD) 0 through 6. The cyclosporine group received cyclosporine (1.25 mg/kg/day) intramuscularly on POD 1 through 6. The EPC-K1 + cyclosporine group received both EPC-K1 and cyclosporine in the same manner as the EPC-K1 and cyclosporine groups. Recipients were killed on POD 7, and the transplanted lungs were examined histologically and graded in a blinded fashion (grade 0 to 4). The effect of EPC-K1 and cyclosporine treatment on the primary immune response was examined by mixed lymphocyte reaction (MLR) between brown Norway rat stimulator cells (treated with mitomycin) added to Lewis rat responder lymphocytes. RESULTS: Control group animals exhibited the severe destructive changes of grade 4 lung rejection. The EPC-K1 + cyclosporine group showed significantly less graft rejection compared with the EPC-K1 group and the cyclosporine group (p < 0.01). In MLR assay, the EPC-K1 + cyclosporine group (793 +/- 210 cpm) showed significantly suppressed lymphocyte proliferation compared with the control group (2188 +/- 360 cpm), EPC-K1 group (1869 +/- 541 cpm), and cyclosporine group (1873 +/- 326 cpm) (p < 0.01). CONCLUSION: EPC-K1 significantly improves effects of cyclosporine at lower doses both in preventing pulmonary allograft rejection and in suppressing lymphocyte proliferation in MLR.

[Treatment of 11 cases of pulmonary arteriovenous fistula].

We have experienced a total of 11 patients operated for pulmonary arteriovernous fistula (PAVF) from 1976 to 1993 consisting of three males and eight females with the average age of 35 years. To reserve the pulmonary function, limited resections were applied for all cases (partial resection in 8, fistula excision in 2, and segmentectomy in one case). The most recent case, a 36-year-old female who had two abnormal shadows on her routine chest X-ray, was diagnosed as PAVF by pulmonary arteriogram. She was treated by fistula excision in the right S8 lesion and embolization in the left S1 + 2 lesion. Because of its unsatisfactory natural course, PAVF should be treated aggressively by surgery and/or by embolization according to the size, shape and localization.