Difference in immunosuppressive effects of antibodies to ICAM-1 and LFA-1 between hepatic and cardiac allografts.
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Biomedical subjects
Publications and source records attributed to K Inoue.
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Pelizaeus-Merzbacher disease (PMD) is an X-linked dysmyelinating disorder caused by abnormalities in the proteolipid protein (PLP) gene, which is essential for oligodendrocyte differentiation and CNS myelin formation. Although linkage analysis has shown the homogeneity at the PLP locus in patients with PMD, exonic mutations in the PLP gene have been identified in only 10%-25% of all cases, which suggests the presence of other genetic aberrations, including gene duplication. In this study, we examined five families with PMD not carrying exonic mutations in PLP gene, using comparative multiplex PCR (CM-PCR) as a semiquantitative assay of gene dosage. PLP gene duplications were identified in four families by CM-PCR and confirmed in three families by densitometric RFLP analysis. Because a homologous myelin protein gene, PMP22, is duplicated in the majority of patients with Charcot-Marie-Tooth 1A, PLP gene overdosage may be a important genetic abnormality in PMD and affect myelin formation.
Two hundred thirty-five patients with small hepato-cellular carcinomas (HCCs) (mixed hepatocellular and cholangiocellular carcinoma included) measuring no more than 3 cm in diameter treated by surgical resection between 1980 and 1990 were reviewed retrospectively. Ninety-nine percent of the patients also had chronic hepatitis and 73% were diagnosed histologically as having liver cirrhosis. The operative and hospital mortality rates were 0.4% and 1.3%, respectively. The number of tumors, intrahepatic metastasis and vascular invasion were significant prognostic factors. Overall the 1-,3-and 5-year survival rates (operative deaths included) were 93.2%, 72. 7%, and 49.7%, and those of 144 patients treated in the late 80s (1986-1990) were 93.6%, 76.3%, and 52. 1%, respectively. These results were appreciably better than those for percutaneous ethanol injection therapy (PEIT) and transcatheter arterial embolization therapy (TAE) reported previously. In particular, the survival of the patient with single tumor demonstrated a clear advantage of surgery. The selection of treatment modalities is discussed, and our present standard strategy for surgical treatment is described.
We report a 63-year-old man with a history of diabetes mellitus for 23 years. Painful dysesthesia developed in his toes and trunk with weight loss of 2kg in two months, after the therapy for diabetes mellitus. Truncal painful dysesthesia was symmetrically distributed in the bilateral posterior and anterior T8-11 dermatomes, sparing the bilateral lateral tholacic areas. Electromyography showed denervation potentials in bilateral abdominal rectus muscles at the levels of Th8-10. Histopathological study of the biopsied right sural nerve revealed small fiber neuropathy. We suspected the truncal painful dysesthesia of this patient resulted from diabetic small fiber polyneuropathy, which was resistant to ordinary medical treatments such as non-steroidal anti-inflammatory drugs. Capsaicin cream containing 0.075% capsaicin, and lidocaine delivered by iontophoresis were both effective for his painful dysesthesia.
We report a 44-year-old man who had sudden-onset severe pain with ardor at the left occipital and nuchal region without trauma in July, 1992. Severe pain disappeared soon after, but dull pain continued intermittently. Four months later, he was referred to our hospital. Neurological examination showed no abnormalities. Cervical CT scan and myelography disclosed liquorrhea. Digital subtraction myelography confirmed cerebrospinal fluid leakage from the level of C1/2 disc. He underwent laminectomy of C1 and C2, and dural plasty on April 6, 1993 with subsequent disappearance of headache. We suppose that this headache was due to rupture of the root sleeve and compression to the root and dorsal root ganglia by outflowed liquor.
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A 23-year-old man was admitted to our hospital complaining of swelling and pain in the penis. Examination revealed fracture of the penis. The tear in the tunica albuginea was not palpable on physical examination of the penis, but magnetic resonance imaging (MRI) revealed the exact site of the tear in the tunica albuginea. MRI was very useful in localizing the site of fracture.
To study the pathological functions of anti-phospholipid (anti-PL) antibodies, we have analyzed their effect on platelet function. We identified an IgG anti-PL mAb, designated PSG3, which cross-reacted specifically with glycoprotein (GP) IIIa in human platelets and inhibited platelet aggregation. PSG3 bound also to certain polyanionic substances, such as double-stranded DNA, heparan sulfate, dextran sulfate and acetylated-LDL, but not to other polyanionic substances. The binding of PSG3 to GPIIIa was completely inhibited by heparan sulfate and dextran sulfate, indicating that PSG3 recognizes a particular array of negative charges expressed on both GPIIIa and the specified polyanionic substances. Since neither neuraminidase- nor endoglycopeptidase F-treatment of GPIIIa had any significant effect on the binding of PSG3, this array must be located within the amino acid sequence of GPIIIa but not in the carbohydrate moiety. Reduction of the disulfide bonds in GPIIIa greatly reduced its reactivity, suggesting that the negative charges in the epitope are arranged in a particular conformation. PSG3 inhibited platelet aggregation induced by either ADP or collagen, it also inhibited fibrinogen binding to activated platelets in a dose-dependent fashion. PSG3, however, did not inhibit the binding of GRGDSP peptide to activated platelets. These results suggest that the PSG3 epitope on GPIIIa contains a particular array of negative charges, and possibly affects the fibrinogen binding to GPIIb/IIIa complex necessary for platelet aggregation.
A 51-year-old woman with bilateral multiple pulmonary metastases of uterine leiomyoma was reported. The patient had undergone a hysterectomy for uterine myoma in 1976. In 1981, routine chest roentgenograms showed three nodular lesions in the right lung with well-defined margins. The patient received no treatment because she was asymptomatic. In January 1993, she consulted a gynecologist because of acute lower abdominal pain and an abdominal mass, and bilateral pulmonary tumors were found. The retroperitoneal solid tumor was extirpated in the first operation, and histological findings showed that it was a benign leiomyoma. The pulmonary tumors, extirpated in the second operation, were benign leiomyoma, also. The clinical course and histological findings suggested that this was a case of benign metastasizing leiomyoma. This disease is rare, 21 case have been reported in Japan.
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The ultrasonographic findings of 25 lesions in 23 patients with surgically proven ovarian masses were reviewed. There were 10 cystic teratomas, two simple cysts, two follicular cysts, two mucinous cystadenomas, two NHL, one corpus luteum cyst, one hydrosalpinx, one serous cystadenoma, one yolk sac carcinoma, one dysgerminoma, one embryonal carcinoma, and one mixed form (yolk sac carcinoma, choriocarcinoma). All patients were less than 15 years old. We classified all cases into four patterns: cystic, cystic with mural nodule, mixed, and solid. Eight lesions of the cystic pattern included two simple cysts, two follicular, cysts and one corpus luteum cyst. The other lesions were benign, too. Nine lesions with the cystic with mural nodule pattern consisted of eight cystic teratomas and one mucinous cystadenoma. All lesions were benign. The mixed pattern was seen in four lesions, half of which were malignant, i. e., one embryonal carcinoma and one yolk sac carcinoma. Four lesions with the solid pattern were all malignant masses: one dysgerminoma, two NHL and one mixed form. In this classification, the cystic and cystic with mural nodule patterns are benign, while mixed and solid patterns are highly suggestive of malignancy.
Cauda equina syndrome is a rare complication in the late stage of ankylosing spondylitis, for which approximately 60 cases have been reported in the literature. The cause of the syndrome is unclear, and there is no effective treatment. Recently lumboperitoneal shunt was reported to have been effective in two patients. In our study, we performed lumboperitoneal shunt in a patient and evaluated the condition after the operation compared with that preoperatively. Some alleviation of neurologic symptoms was observed for 6 months after operation. Histopathologic examination of the dural diverticulum revealed a residual change after old inflammation. Lumboperitoneal shunt was an effective surgical treatment for cauda equina syndrome in this patient with ankylosing spondylitis, but its effects were not extreme. Arachnoiditis is suggested to be involved in the pathogenesis of cauda equina syndrome.
A 69-year-old woman was admitted to our hospital complaining of urinary retention. On examination, the labia was found to be fused extensively, with a pinhole opening at the midline. The adhesions were separated under spinal anesthesia. One year after operation, there remains no recurrence. Etiological factor in labial adhesions are thought to include a combination of local inflammation and estrogen deficiency. In our case, the patient had hip joint disease. Hip joint disease is shown to influence the formation of adhesions by interfering with perineal hygiene and decreasing sexual activity.
Forty-three primary mediastinal tumors or cysts were surgically treated in 41 patients during a 10-year period. These tumors consisted of 20 thymic tumors, 10 neurogenic tumors, 5 teratomas, 3 lymphoid tumors, 2 congenital cysts, 2 mediastinal thyroid tumors, and 1 chondroma. There were 16 male and 25 female patients. The mean age was 44 years with a range of 6 to 79 years. Sixteen patients (39%) were symptomatic. There were 20 thymic tumors including 13 thymomas, 5 thymic cysts, and 2 hyperplasia with myastenia. Additional radiation therapy was recommended for all but stage I thymomas. Only 1 of the 10 neurogenic tumors was malignant. Eight teratomas were all cystic and matured. Early operative intervention is mandatory in these cases.