Traumatic Horner syndrome without anhidrosis.

In a patient with a traumatic avulsion of the arm, magnetic resonance imaging showed the exact site of the lesion that produced Horner syndrome with preservation of sweating on the face.

A binding protein to the DNase I hypersensitive site II in HLA-DR alpha gene was identified as NF90.

We previously observed that IFN gamma-inducible expression of the human MHC class II, HLA-DR alpha, gene was enhanced by treatment with 12-O-tetradecanoylphorbol-13-acetate (TPA) only in human monocytic leukemia THP-1 cells, but not in HeLa cells. In the HLA-DR alpha gene, three DNase I hypersensitive sites (DHS) are known to be present in the promoter region (DHS-I) and first intron (DHS-II and -III) and are assumed to be involved in HLA-DR alpha gene regulation. In this study, we found a binding factor which recognized a unique palindrome sequence (DHS-22) in the region of the DHS II site of the HLA-DR alpha gene in THP-1 cells and HeLa cells. The binding activity of this factor was decreased by TPA treatment in THP-1 cells, but not in HeLa cells. This binding activity was also detectable in nuclear extracts of bovine brains. Thus, we isolated the DHS-22 binding factor from bovine brain nuclear extracts and finally identified it as NF90 on the basis of molecular mass analysis of Lys-C-digested fragments and amino acid sequences of the two peptides of the trypsin-digested binding protein. The DHS-22 binding protein(s) in THP-1 cells is (are) further confirmed by reactivity to an antibody against NF90, and we have demonstrated that the GST fusion protein of NF90 interacts with DHS-22 by electrophoretic gel mobility shift assay (EMSA). The mRNA of NF90 was decreased by TPA treatment in THP-1 cells but not in HeLa cells. These results suggest that the binding of NF90 to the DNase I hypersensitive site II of HLA-DR alpha gene seems to negatively regulate HLA-DR alpha gene expression.

Functional outcome of anterior transposition of the vascularized ulnar nerve for cubital tunnel syndrome.

Anterior transposition of the ulnar nerve is a widely used treatment for cubital tunnel syndrome, but neurolysis performed at the time of surgery may impair the blood supply to the ulnar nerve. This study compared the results of intramuscular anterior transposition of the ulnar nerve with or without preserving the extrinsic vessels of the ulnar nerve in 35 patients. The postoperative nerve conduction velocity and the clinical results were better in the group in which the extrinsic vessels were presented.

Analysis of the TPA regulatory element in the genomic poly(ADP-ribose) synthetase gene in human leukemia U937 cells.

The human leukemia U937 cells differentiate into monocyte/macrophage-like cells when treated with 12-O-tetradecanoylphorbol-13-acetate (TPA). We observed that during this process, both protein and mRNA levels for PARS markedly decreased in U937 cells. Through deletion analysis of the PARS regulatory gene, we found that the sequence within the first intron region was responsible for the TPA-dependent repression. Electrophoretic mobility shift assays (EMSAs) and Southwestern blot analysis indicate that this element bound specifically to a nuclear protein. TPA treatment abolished the binding of the protein in U937 cells but not in HeLa cells. DNase I footprinting data show that the cis regulatory element is located between residues 328 and 383. We further examined the function of this cis element (BS207) in a basal promoter regulatory reporter construct and found that this cis element (BS207) functions as an enhancer via the binding of an unknown trans-acting factor. TPA treatment diminished the binding activity of the factor in U937 cells, resulting in a decrease in the enhanced activity to the basal level. These results suggest that abolishment of the binding of a special nuclear protein to the first intron of the PARS gene is related to the TPA-responsive downregulation of PARS in U937 cells.

Transactivation of transforming growth factor alpha gene by hepatitis B virus preS1.

Hepatitis B virus (HBV) causes acute and chronic liver injury, and integration of HBV DNA is considered to be an important pathogenic determinant for hepatocarcinogenesis and tumor development. Transforming growth factor alpha (TGF-alpha) drastically accelerates hepatocarcinogenesis when it is overexpressed in TGF-alpha transgenic mice (C. Jhappan et al., Cell, 61: 1137-1146, 1990). In HBV-infected patients, hepatocellular carcinoma (HCC) cells show elevated expression of TGF-alpha (C. C. Hsia et al., J. Med. Virol., 43: 216-221, 1994), the mechanism for which, however, has not been clarified yet. We here show that preS1, a part of the HBV large surface protein, carries a transcriptional transactivation domain and activates the transcription of the TGF-alpha gene by 2-fold in human HCC HuH6 cells. The responsive elements are restricted to the 315-bp segment of the proximal TGF-alpha promoter (-373 to -59). Furthermore, the expression of TGF-alpha was markedly increased in permanently preS1-producing HuH6 transformants. The crucial role for HBV preS1 in hepatocarcinogenesis and tumor development through transactivation of the TGF-alpha gene may give us new insight into the understanding of pathogenesis and therapy of viral hepatocarcinogenesis.

Correlation between HLA-DR expression and level of poly(ADP-ribose) synthetase in human thyroid carcinoma cells.

The expression of MHC class II molecules is normally restricted to antigen presenting cells. Aberrant expression of class II molecules, however, was detected in the thyrocytes of autoimmune thyroid diseases. We attempted to regulate the expression of HLA-DR molecules in thyroid carcinoma cells by expressing the exogenous poly(ADP-ribose) synthetase gene. We transfected a metal inducible expression plasmid capable of expressing poly(ADP-ribose) synthetase gene into thyroid carcinoma 8505C cells and the transformants, treated with metal and IFN-gamma, were separated by Magnetic Cell Separation. The activity of the synthetase was increased in the HLA-DR-enriched transformants as compared with that in control or the HLA-DR+ transformants. RNA blot analysis and flow cytometric analysis revealed that the IFN-gamma-inducible expression of HLA-DR molecules was depressed by the induction of exogenous poly(ADP-ribose) synthetase gene. This result indicates that HLA-DR expression was correlated with the level of poly(ADP-ribose) synthetase in human thyroid carcinoma cells. Furthermore we examined the level of poly(ADP-ribose) synthetase in patients with autoimmune thyroid diseases. We observed a significant decrease in poly(ADP-ribose) synthetase in the patients. Taken together with the previous observation, the decrease in poly(ADP-ribose) synthetase is closely linked to the aberrant expression of HLA-DR molecules in some autoimmune thyroid diseases.

Apophysitis of the acromion.

We treated three patients with apophysitis of the acromion. These patients were two male athletes 12 and 14 years of age, respectively, and one female athlete 13 years of age. They reported pain at the top of the shoulder during and after shoulder movement while playing sports but had no rest pain or disturbance of daily activities. Physical examination demonstrated marked local tenderness at the acromion and slight warmth. X-ray films showed sclerosis and irregularity of the secondary ossification center of the acromion. Bone scintigraphy carried out on one patient demonstrated increased uptake in that region. Conservative treatment was used for these patients. Recovery was gradual but satisfactory.

Brachial plexus stretching injuries: microcirculation of the brachial plexus.

This study was undertaken to investigate the pathogenesis of brachial plexus stretching injuries at an intensity level not severe enough to cause avulsion injury. While we performed traction on 64 forelegs of 32 rats, we evaluated changes in the blood flow in the extrinsic and intrinsic microvascular systems of the brachial plexus. While we laterally stretched the brachial plexus during 80 degree shoulder abduction, we measured the blood flow at the bifurcation of the brachial plexus and at the median nerve with the hydrogen washout technique. During weak traction the blood flow decreased markedly in the extrinsic system, causing an imbalance in the two systems. In the median nerve, however, no such imbalance occurred. On histologic examination the axon and myelin in the brachial plexus and the median nerve showed no morphologic change. However, in parts of the brachial plexus we noted hypertrophic connective tissue or granulomatous inflammation in tissue surrounding the extrinsic system. The extrinsic system's apparent susceptibility to injury by acute traction may be a factor in the pathogenesis of the brachial plexus stretching injuries.

Effect of poly(ADP-ribose) synthetase on the expression of major histocompatibility complex (MHC) class II genes.

We have studied the effect of poly(ADP-ribose) synthetase on the interferon-gamma (IFN-gamma)-inducible expression of major histocompatibility complex (MHC) class II molecules. We constructed an expression plasmid capable of expressing either a sense RNA (MT-ARS) or an antisense RNA (pAS-FL or pAS-5') for poly(ADP-ribose) synthetase. We transfected the plasmid into mouse or human macrophage tumor cells and examined the effect on the expression of MHC class II molecules. The IFN-gamma-inducible expression of MHC class II gene was considerably reduced in transformant clones (A-2, B-2), in which the synthetase was highly expressed, whereas the depletion of the synthetase due to the expression of antisense RNA for the synthetase amplified the expression of MHC class II molecules. The results indicate that the level of the synthetase critically regulates the IFN-gamma-inducible MHC class II molecules. Next, we analyzed DNase I hypersensitive sites (DHS) of mouse MHC class II, I-A beta gene and found two sites, one in the promoter region and the other one in the first intron. The DHS in first intron was less sensitive towards DNase I attack in transformant clones (A-2, B-2) in which the synthetase was synthesized in a large quantity. Thus we constructed two beta-galactosidase reporter genes, one (A beta 2.0kb-lac z) containing the promoter region to a part of the second exon of the class II gene, and the other (A beta pro-lac z) containing the promoter region of the class II gene alone. The expression of the reporter gene was analyzed by reverse transcriptase-polymerase chain reaction (RT-PCR) and found that the expression of A beta 2.0kb-lac z was suppressed in the transformant clones (A-2, B-2) relevant to control cells but the expression of A beta pro-lac z was the same level among those cells.(ABSTRACT TRUNCATED AT 250 WORDS)

[A case of degenerative type of progressive myoclonus epilepsy].

This report describes a patient with degenerative type of progressive myoclonus epilepsy (PME), who showed slowly progressive deterioration of the central nervous system; intellectual impairment, dysarthria, and involuntary movements, particularly action myoclonus and dystonia. The patient was a 19-year-old woman who had no hereditary factors. At the age of 4, she developed action myoclonus in the upper limbs bilaterally. Her condition became gradually worse, and at the age of 15, she was admitted to our hospital because of involuntary movement in the upper limbs. First physical examination revealed mild mental retardation, action myoclonus, dystonia, and delayed adolescence. As giant SEP characteristic of PME and Ramsay Hunt syndrome was found, she was tentatively diagnosed as having Ramsay Hunt syndrome without epilepsy, and delayed adolescence. Now, she is 19 years old, and unable to walk alone because of involuntary movements and paralysis. But she has not developed epilepsy. As she has not been compatible with progressive myoclonus epilepsy (PME) and progressive myoclonic ataxia (PMA) classified by Marseille Consensus Group, she has been diagnosed as having an atypical PME syndrome.

Factors contributing to cerebral hypomyelination in the growth hormone-deficient little mouse.

We attempted to delineate the events leading to hypomyelination in the brain of the little mouse, a promising murine model of isolated growth hormone deficiency. At 20 days of age, the mutant mouse brain weighed less than its normal counterpart, and this difference in brain weight persisted. Increase in CNPase activity was found to be suppressed in the cerebrum throughout the developmental stage, but not in the other parts of the brain. Differences in cerebral DNA content between the little and normal mice first became apparent on the 10th day of age. Thereafter, the rate of increase in the little brain consistently lagged behind the normal. [3H]Thymidine incorporation into the DNA fraction in vivo on the 7th day of age, when glial cell proliferation in the normal cerebrum is most active, was approximately half that of the controls in all parts of the little brain. These findings indicate that the hypomyelination of the mutant cerebrum might result from reduced oligodendroglial proliferation due to growth hormone deficiency.

[A study of an immunosuppressive acidic protein (IAP) in patients with a bone tumor].

We evaluated an immunosuppressive acidic protein (IAP) as a tumor marker in cases of a tumor of the bone (benign, 21; primary malignant, 26; metastatic carcinoma, 12). The IAP positive rate of a primary malignant tumor of the bone was 60%, and the mean was 591 + 59.4 micrograms/ml. This rate of a benign tumor of the bone was 10%, and the mean was 382 +/- 31.3 micrograms/ml. IAP may represent a tumor marker in malignant tumors of the bone.

Decrease in the level of poly(ADP-ribose) synthetase during nerve growth factor-promoted neurite outgrowth in rat pheochromocytoma PC12 cells.

We have studied the changes in the levels of the enzyme molecule and mRNA for poly(ADP-ribose) synthetase during nerve growth factor-promoted neurite outgrowth in rat pheochromocytoma PC12 cells. When the PC12 cells were cultured in the presence of nerve growth factor, the content of enzyme molecules decreased along with neurite outgrowth to 50% of the original amounts in 2 days and the content of mRNA for the enzyme also decreased to approximately 50% in 2 days. These results suggest that the decrease of the enzyme molecule may be due to depression of expression of the gene for synthetase during the process. Taken together with previous observations, the decrease of the synthetase seems to be required for some cellular differentiation.

Persistent hemarthrosis of the shoulder joint with a rotator-cuff tear in the elderly.

Three elderly patients developed persistent hemarthrosis of the shoulder joint without having suffered injury. Complete tears of the rotator cuff, attrition of the undersurface of the acromion, and instability were noted in the affected shoulders. Synovial fluids examined from two patients contained many alizarin red S-positive microspheroids. The synovium obtained at surgery from two patients showed hypervascularity, vasodilatation, and severe degenerative changes of collagenous tissues. The tendon of the m. supraspinatus showed infiltrations of multinucleated giant cells around calcified deposits consisting of hydroxyapatite crystals. Anatomical and mechanical derangements, and possible biological reactions following phagocytosis of hydroxyapatite crystals, may have contributed to the persistent hemorrhage in the shoulder joints.

Management of thoracic outlet syndrome.

The experience with transaxillary first-rib resection and scalenotomy reported herein shows these to be safe and effective treatments for patients with thoracic outlet syndrome (TOS). The former operation usually offers the best possibility for complete resection of the first rib and all anomalous congenital bands, and is curative in most cases, regardless of the mechanism of the compression. Scalenotomy, or Powers' operation, is also helpful, in particular for cases of TOS with a vertebral arterial insufficiency. Accurate diagnosis of TOS and selection for surgery after a detailed history and proper physical examination will result in gratifying relief of symptoms in almost all patients with TOS.

Enhancement of methyl mercury-induced lipid peroxidation by the addition of ascorbic acid.

Effects of mercuric chloride (HgCl2) and methylmercuric chloride (MMC) in the presence of ascorbic acid on lipid peroxidation in rabbit renal cortical mitochondria were studied. The addition of ascorbic acid showed no effect on HgCl2-induced lipid peroxidation, but ascorbic acid plus MMC were able to stimulate the lipid peroxidation markedly. This result suggests that in the presence of ascorbic acid MMC has a powerful stimulatory effect on the lipid peroxidation in mitochondria.