In neurofibromatosis type-1 (NF-1), abnormal growth regulation may be related to the formation of multiple neurofibromas. We investigated the growth responses of neurofibroma-derived cells (NF cells) and control skin fibroblasts to various growth factors. The responses to platelet-derived growth factor (PDGF-BB) and transforming growth factor-beta 1 (TGF-beta 1) in NF cells were significantly greater than those in control fibroblasts. The increased response to PDGF-BB in NF cells was accompanied by an increased number of PDGF beta receptors, which was demonstrated by both 125I PDGF-BB binding assay and immunoblotting analysis. The increased response to TGF-beta 1 was assumed to be mediated through PDGF-like protein induction; TGF-beta 1-treated NF cells produced greater amounts of 36-kD PDGF-like protein than TGF-beta 1-treated control fibroblasts. These observations suggest that certain growth factors, e.g., PDGF-BB and TGF-beta, may play some role in the development of neurofibromas in NF-1.

Becaplermin↗

PubMed1994

Antihistone antibodies in systemic sclerosis. Association with pulmonary fibrosis.

OBJECTIVE: To determine the prevalence and clinical significance of antihistone antibodies (AHA) in systemic sclerosis (SSc). METHODS: Serum samples from patients with limited cutaneous SSc (n = 44), diffuse cutaneous SSc (dcSSc; n = 48), and other SSc-related disorders (n = 22) were examined by enzyme-linked immunosorbent assay and immunoblotting for AHA. RESULTS: AHA were demonstrated in 29% of the 92 SSc patients and in 44% of those with dcSSc. The presence of AHA correlated with severe pulmonary fibrosis in those with dcSSc. Immunoblotting revealed that the predominant antigen was histone H1. CONCLUSION: AHA might be a serologic indicator of the severity of pulmonary fibrosis in SSc.

Adult↗

PubMed1994

Diminished visceral adipose tissue in cancer cachexia.

To estimate the relationship between the visceral adipose tissue (AT) area and cancer cachexia, 13 cachectic patients (7 males, 6 females; age 65.2 +/- 11.0 years; body mass index 20.8 +/- 4.1 kg/m2) were examined by computed tomography (CT) scanning. Cachectic cancer patients who had a 10% decrease of body weight and died within 6 months because of gastrointestinal carcinoma had a significantly smaller visceral AT area than control subjects (mean +/- sd: 43.9 +/- 42.2 cm2 vs. 93.4 +/- 56.0 cm2, P < 0.05, P = 0.014). Otherwise, there were no significant differences between the visceral AT areas of cachectic cancer patients and those of cancer patients with resectable tumors treated by curative operation (mean +/- sd: 68.8 +/- 57.7 cm2) (NS, P = 0.206). There was, however, a tendency for cachectic cancer patients to have a smaller visceral AT area than those with resectable tumors. This result suggests that the visceral AT area is not preserved in the cachectic state associated with cancer.

Adipose Tissue↗

PubMed1994

Incidence of latent infection of Epstein-Barr virus in lung cancers--an analysis of EBER1 expression in lung cancers by in situ hybridization.

To evaluate the presence of Epstein-Barr virus (EBV) in lung cancers of Japanese patients, 81 lung cancers were examined using a highly sensitive in situ hybridization (ISH) method, employing an antisense oligonucleotide probe for EBV-encoded small nuclear RNA-1 (EBER). EBER1 expression was demonstrated in one poorly differentiated squamous cell carcinoma associated with marked lymphoid stroma (PDSCC-LS), two well differentiated adenocarcinomas, and two moderately differentiated squamous cell carcinomas, but was not detectable in other lung cancers, including small cell carcinomas. Unlike lymphoepithelioma-like undifferentiated carcinoma (LELC) of the lung, the PDSCC-LS consisted of poorly differentiated cells with distinct cell borders and nuclei with a coarse chromatin pattern and some prominent nucleoli. Most of the cancer cells expressed intense EBER1 signals. Although small to moderate numbers of cells positive for EBER1 were present in two adenocarcinomas and two squamous cell carcinomas, EBER1 signals varied in intensity and number in these four cases. Although polymerase chain reaction (PCR) and Southern blot hybridization with a 32P-labelled probe internal to the primers were conducted to detect the EBV genome in 24 lung cancers, including five EBER1-positive cases, the genome was found to be positive in the five cases with EBER1-positive staining, including the PDSCC-LS, two adenocarcinomas and two squamous cell carcinomas, but not in the other cases. This study indicates that the morphological features of EBV-associated lung cancers are not restricted to the typical LELC type.

Adenocarcinoma↗

PubMed1994

Production of interleukin-1 by primary cultured parenchymal liver cells (hepatocytes).

The production of interleukin-1 (IL-1) by cultured parenchymal liver cells was revealed by a biological assay with an IL-1-dependent cell line, Northern blot analysis, and in situ hybridization. Inhibition experiments on the IL-1 activity with anti IL-1 alpha antibody also support the presence of IL-1 alpha in the supernatant of cultured parenchymal liver cells. Based on these results, we discuss the possibility of IL-1 production by parenchymal liver cells in vivo.

Animals↗

PubMed1994

Rat T-cell receptor gamma chain sequences: identification of conserved gene segments and a unique chimeric constant region gene, C4L.

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Amino Acid Sequence↗

PubMed1994

Serum concentration of procollagen type I carboxyterminal propeptide in systemic sclerosis.

The serum level of procollagen type I carboxyterminal propeptide (P1CP), which has been used as an index of collagen synthesis in patients with various fibrotic diseases during the active stage, was measured using enzyme-linked immunosorbent assay in 61 patients with systemic sclerosis (SSc) and in 21 control subjects. The mean P1CP level in the SSc patients was significantly higher than in the normal controls (mean +/- SD, 326 +/- 319 vs 128 +/- 87 ng/ml; p < 0.005). In 36% of the SSc patients, the serum P1CP level was significantly elevated more than two standard deviations above the mean control value. The mean serum P1CP level in patients with diffuse SSc was significantly higher than in those with limited SSc (411 +/- 373 vs 255 +/- 199 ng/ml; p < 0.05). In addition, the SSc patients with elevated serum P1CP levels showed a significantly greater incidence of lung fibrosis and joint involvement than those with normal P1CP levels (p < 0.005 and p < 0.05, respectively). These results suggest that the serum P1CP level is a useful indicator of the severity of disease in SSc patients.

Adolescent↗

PubMed1994

Asymptomatic primary mucinous cystadenocarcinoma of the appendix with a large abdominal mass: report of a case.

A case of cystadenocarcinoma of the appendix with a large cystic lesion is reported. A 49-year-old man undergoing a routine ultrasonic scan was incidentally found to have an abdominal mass measuring some 30 cm in size. The clinical presentation was asymptomatic, and the patient underwent a laparotomy without ascertaining a diagnosis preoperatively. The lesion, which derived from the appendix, was removed and was found to be cystic and contained huge amounts of mucin. The histological findings revealed a well-differentiated cystadenocarcinoma of the appendix, and immunohistochemical staining of the epithelium and mucinous implants in the mass demonstrated a positive reaction for carcinogenic antigens, including carcinoembryonic antigen and carbohydrate antigen.

Abdominal Neoplasms↗

PubMed1994

Bronchomotor agents and hysteresis of collateral resistance in dog lobe.

The effect of smooth muscle tone on hysteresis of collateral channels was examined and compared with that of airways, in freshly excised dog lobes. A double lumen catheter was sealed into a branch off the main bronchus, and air (Vs) flowed through the outer lumen while the pressure in the segment (Ps) was measured by the inner lumen. Collateral conductance (Gcoll) was calculated as Gcoll = Vs/Ps. In another series of experiments a pleural capsule was used to measure either segmental airway flow (Vsaw) or capsule pressure (Pcap). Segmental airway conductance (Gsaw) was calculated as Gsaw = Vsaw/(Ps-Pcap). Hysteresis for Gcoll vs lung volume (VL) curves were almost absent in control and after isoproterenol inflation Gcoll was greater than deflation Gcoll at a given VL (clockwise history). Conversely, after histamine Gcoll vs VL curves showed a counterclockwise history. The behaviors of Gcoll and Gsaw against a bronchomotor agent were similar. We conclude that major collateral channels have muscular walls, and being that of an airway type.

Airway Resistance↗

PubMed1994

Vascular malformations of the brain in hereditary hemorrhagic telangiectasia (Rendu-Osler-Weber disease).

Six patients with vascular malformation of the brain in hereditary hemorrhagic telangiectasia (HHT) were reviewed to determine clinical and radiographic characteristics of these lesions. There were two patients with arteriovenous fistula (AVF), three with arteriovenous malformation (AVM), and one with multiple AVMs associated with AVF. Seizures were the most common presenting symptom (seen in three patients), and two of them had intracerebral hematomas (ICH). In the remainder, the malformations were incidentally found in the course of evaluation of other diseases. Their locations were variable, but the majority was superficially confined to the cerebral cortex. Arterial supply was from mostly one feeding artery that was a cortical branch of either anterior, middle, or posterior cerebral artery. In six of nine malformations, the venous drainage was through a superficial cerebral vein into either the superior sagittal sinus or transverse sinus. Direct surgery was done on two patients with ICH, artificial embolization on one, and stereotactic radiosurgery on one. The cerebral vascular malformations in HHT are not infrequent, and in particular the importance of computed tomography and cerebral angiography should be recognized in patients with pulmonary AVF associated with HHT.

Adolescent↗

PubMed1994

Anterior fossa dural arteriovenous malformation supplied by bilateral ethmoidal arteries.

Two cases of dural arteriovenous malformation (AVM) involving the base of the anterior cranial fossa are reported. The nidi in both cases were located in the region of the left cribriform plate and mainly supplied by the anterior ethmoidal arteries of both sides, draining through pial veins into either the cavernous sinus or the superior sagittal sinus. In the first case neurologic symptoms resulted from transient frontal lobe dysfunction presumably due to abnormal venous drainage, and the second case presented with subarachnoid hemorrhage caused by rupture of dural AVM at the base of the anterior fossa. Both cases were surgically managed and the AVMs were successfully obliterated. This unique subgroup of dural AVM in the anterior fossa is thoroughly reviewed in the literature, and the epidemiology, symptomatology, neuroradiology, surgical treatment, and associated vascular lesions are discussed.

Dura Mater↗

PubMed1994

Large pituitary adenoma of the sphenoid sinus and the nasopharynx: report of a case with ultrastructural evaluations.

A unique case of nonfunctioning pituitary adenoma exclusively involving the sphenoid sinus and the nasopharynx is reported. The computed tomography and magnetic resonance imaging (MRI) clearly depicted the presence of a large, soft mass in the sphenoid sinus and its extensive invasion to the sphenoid wing and the clivus. In particular MRI was found useful in delineating precise anatomic relationships between this sphenoid sinus tumor and the pituitary fossa. The sphenoid sinus tumor was partially resected by sublabial transnasal approach, and the intact dura mater of the base of the pituitary fossa was confirmed. Pathologic examinations including immunocytochemical and ultrastructural studies showed that the tumor was classified as a nonfunctioning acidophilic pituitary adenoma. Despite the endocrine-inactive tumor, the presence of small secretory granules in the cytoplasm demonstrated by electron microscopic studies was of significant importance in establishing the diagnosis. This rare tumor is reviewed in the literature in the context of nasopharyngeal extension of pituitary adenomas, and a possibility of ectopic occurrence and growth is also discussed in the presented case.

Adenoma↗

PubMed1994

Clinical characteristics associated with antihistone antibodies in patients with localized scleroderma.

BACKGROUND: Recently we demonstrated the presence of antihistone antibodies (AHA) in localized scleroderma. OBJECTIVE: Our purpose was to determine clinical characteristics associated with AHA in patients with localized scleroderma. METHODS: We examined 57 serum samples by an enzyme-linked immunosorbent assay in the following three subgroups: 15 patients with generalized morphea, 27 with linear scleroderma, and 15 with morphea. We classified the patients as having generalized morphea when they had four or more lesions on at least two areas of the body, irrespective of whether the lesions were of morphea or linear type. RESULTS: AHA were detected in 42% of patients with localized scleroderma (24 of 57), and in 87% of patients with generalized morphea (13 of 15). The presence of AHA strongly correlated with the number of morphea lesions, the total number of lesions, and the number of involved areas of the body. However, AHA did not correlate with the presence or number of linear lesions. The presence of AHA showed a 87% sensitivity (13 of 15 patients) and a 74% specificity (31 of 42 patients) for generalized morphea. CONCLUSION: Our data suggest that AHA are a serologic marker for generalized morphea and that the validity of our new classification for generalized morphea is supported by the high frequency of AHA detection.

Adolescent↗

PubMed1994

Increased levels of circulating intercellular adhesion molecule-1 in patients with localized scleroderma.

BACKGROUND: Intercellular adhesion molecule-1 (ICAM-1) is important in immune-mediated mechanisms, and its circulating form (cICAM-1) may be an indicator of immune activation. Localized scleroderma is accompanied by various immunologic abnormalities. OBJECTIVE: We investigated whether the serum level of cICAM-1 in patients with localized scleroderma was elevated and was correlated with the clinical or serologic features of this disease. METHODS: Serum cICAM-1 levels were determined by an enzyme-linked immunosorbent assay in 48 patients with localized scleroderma, in 20 patients with systemic sclerosis, and in 20 healthy control subjects. RESULTS: Serum levels of cICAM-1 were significantly higher in patients with localized scleroderma than in the healthy control subjects. These levels correlated with the number of lesions, the number of involved areas, levels of antihistone antibody IgM, and levels of soluble interleukin 2 receptor. CONCLUSION: The results suggest that immune activation may be a factor in localized scleroderma.

Adult↗