Demonstration of Somatostatin Production in Medullary Carcinoma of the Thyroid

The localization and release of somatostatin in seven casesof medullary carcinoma of the thyroid were investigated by meansof specific anti-somatostatin antibody. In all four patientsexamined high concentrations of plasma somatostatin (125–400pg/ml) and calcitonin (1,917–82,000 pg/ml) were shown.Primary and metastatic tumor tissues obtained from three patientscontained higher amounts of somatostatin (13.1–350.0 ng/gwet wt) than the surrounding tissues. Immunohistochemically,somatostatin-immuno-reactive (IR) cells and calcitonin-IR cellswere shown to compose the majority of tumor cells, leaving asmall number of nonreactive cells in primary and metastatictumors of the seven cases, but in general calcitonin-IR cellswere more numerous than somatostatin-IR cells. The numbers ofcalcitonin-IR and somatostatin-IR cells were not markedly differentfrom case to case, but their population density did not alwayscorrespond to the concentrations of plasma and tissue somatostatin.These data indicate that medullary carcinoma of the thyroidis one of the somatostatin-producing tumors.     

Lack of linkage between atopy and locus 11q13

Atopy as defined in terms of IgE responsiveness was reported to be controlled by a single gene in British families, and this concept was further supported by a significant linkage between atopy and restriction fragment length polymorphism (RFLP) detected by a DNA probe specific to chromosome 11q13. To confirm this observation in a Japanese population, segregation and linkage analyses were done in four large families. Although segregation patterns of atopy were in agreement with the pattern of autosomal dominant inheritance, there was no significant linkage between atopy and locus 11q13. Alterations in the definitions of atopy did not affect the results. These findings suggested the presence of heterogeneity in genetic elements of atopy, even though atopy may be determined mainly by a single dominant gene.     

A Case of Pleomorphic Adenoma of the Trachea

The extent of ultraviolet (UV) irradiation-induced DNA repairwas measured in bone marrow cells and peripheral lymphocytesof patients with refractory anemia with excess of blasts (RAEB).Bone marrow cells from RAEB, when exposed to a 2 J/m dose ofUV, exhibited 50% lower incorporation of tritiated thymidinethan those of control subjects. A similar finding was observedin the peripheral lymphocytes. These data suggest that bonemarrow cells and peripheral lymphocytes from RAEB are deficientin repair of UV-induced lesions by DNA. Furthermore, this impairedDNA repair efficiency in RAEB was not related to the presenceor absence of a karyotype abnormality.     

Epidemiological and clinical features of idiopathic pulmonary alveolar proteinosis in Japan

Abstract:   Idiopathic pulmonary alveolar proteinosis (IPAP) is a rare disease characterized by excessive amounts of lipoproteinaceous material in the alveolus. This report presents an interim analysis of nationwide epidemiological data from Japanese patients with pulmonary alveolar proteinosis, and the roles of serum markers for IPAP. (i) The nationwide demographic data from 166 Japanese patients with IPAP are shown. The female to male ratio was 1:2, and the average age was 51 ± 14 years old (age range: 15–79 years) at registration or diagnosis. A total of 30% of patients with IPAP have a poor clinical course. In total, 30% of patients were treated with whole lung lavage therapy (WLL). Under WLL, the patients significantly improved in the short term, but 40% of the patients who underwent WLL worsened again. A new strategy such as granulocyte-macrophage colony-stimulating factor (GM-CSF) therapy for intractable PAP is required. (ii) The correlation of serum KL-6, carcinoembryonic antigen, surfactant proteins D and A, and LDH with disease severity suggests their potential as disease markers. In contrast, serum anti-GM-CSF antibody did not correlate with disease severity, but is a specific marker for the diagnosis of IPAP. The combined measurements of the serum markers may well prove very useful for both the diagnosis and the management of IPAP patients.     

Early Recognition of Isolated Splenic Vein Thrombosis: Diagnostic Value of Endosonography

A 71-year-old man was followed-up for chronic pancreatitis and gastric mucosal cancer after endoscopic resection. Repeated endoscopic ultrasonography incidentally depicted isolated gastric varices and paramural collateral vessels, which had gone unrecognized on conventional endoscopy and abdominal ultrasonography. The features of subsequent angiography were consistent with isolated splenic vein thrombosis. Isolated splenic vein thrombosis, which often follows pancreatitis, is characterized by gastric varices and splenomegaly without portal hypertension. This condition has a poor medical prognosis due to possible variceal hemorrhage, if the condition is left undiagnosed. For patients with chronic pancreatitis, we should not only examine the pancreas but also check for gastric varices using endoscopic ultrasonography, which is a non-invasive yet sensitive procedure for uncovering this abnormality.     

Mechanism of pain in osteoid osteomas: an immunohistochemical study

Osteoid osteoma is a benign bone tumour characterized by pain which is relieved by non-steroidal anti-inflammatory drugs (NSAIDs), such as aspirin. To clarify the mechanism of the pain, five osteoid osteomas were studied immunohistochemically using polyclonal antibodies against prostaglandin E2 (PGE2), S-100 protein and protein gene product 9.5 (PGP 9.5). In all five cases, the pain had been relieved by NSAIDs. Nerve fibres positive for S-100 protein and PGP 9.5 were observed in the fibrous zone, especially close to the blood vessels, around the nidus in all the lesions and also within the nidus in three lesions. PGE2 immunoreactivity was variably positive in the nidus of three lesions. In one case a large number of actively proliferating osteoblasts reacted with this antibody. The other cases showed unevenly distributed PGE2 positivity which tended to be prominent in the plump osteoblasts. As control material we examined fibrous dysplasia (3 cases), osteosarcomas (3) and giant-cell tumours of bone (3). The plump osteoblastic tumour cells of three osteosarcomas and the bone-forming cells in two cases of fibrous dysplasia gave a positive reaction for PGE2. No S-100 or PGP 9.5 immunoreactive nerve fibres were seen in these lesions. It is concluded that the presence of nerve fibres alone might play a more important role in mediation of pain in osteoid osteomas than some effects of osteoblast-produced PGE2 on the nerves and proliferated blood vessels.