A clinical and ultrasonographic study of risk factors for elbow injury in young baseball players.

PURPOSE: To determine the risk factors for elbow injury and its association with glenohumeral internal rotation deficit among young baseball players. METHODS: 229 baseball players aged 9 to 14 (mean, 11) years completed a self-administered questionnaire with items related to years of playing baseball, hours of training per weekday, days of training per week, and past and present experience of elbow pain. Two orthopaedic surgeons measured the range of motion of both shoulders and elbows. Another 2 orthopaedic surgeons performed ultrasonography to detect any elbow abnormality such as fragmentation of the medial epicondylar apophysis and osteochondritis dissecans of the capitellum. Using univariate and multivariable analyses, participants with or without elbow abnormality were compared to determine the risk factors for elbow abnormality. RESULTS: Elbow abnormality was detected in 100 of the participants and comprised osteochondritis dissecans of the capitellum (n=18) and fragmentation of the medial epicondylar apophysis (n=82). Elbow abnormality was associated with being a pitcher, past and present experience of elbow pain, loss of elbow extension, and the side-to-side internal rotation difference. The 100 participants with elbow abnormality were stratified into symptomatic (n=57) or asymptomatic (n=43) of elbow pain. Those with elbow abnormality and elbow pain was associated with being a pitcher. CONCLUSION: Being a pitcher was a risk factor for both elbow abnormality and elbow pain. Nonetheless, 43% of baseball players with elbow abnormality were asymptomatic. The use of ultrasonography was effective in detecting elbow abnormality and enabling early treatment.

Serum carcinoembryonic antigen (CEA) as a clinical marker in acquired idiopathic generalized anhidrosis: a close correlation between serum CEA level and disease activity.

BACKGROUND: Hypohidrosis/anhidrosis are congenital or acquired sweating impairments. Among them, acquired idiopathic generalized anhidrosis/hypohidrosis (AIGA) is the most common, and characterized by favourable response to systemic corticosteroid, however, no clinical markers for disease severity or activity have been developed. OBJECTIVE: Our aim was to verify the usefulness of serum carcinoembryonic antigen (CEA) level monitoring as a clinical marker for disease activity of AIGA. METHODS: Ten cases of AIGA diagnosed at Asahikawa Medical University, from 1980 to 2014 were included in the study. CEA and/or CEACAM1 expression level was analysed using immunohistochemistry and enzyme-linked immunosorbent assay. RESULT: CEA expression was restricted to the apical membrane of glandular cells in eccrine sweat glands in most of the three types of cases we examined [healthy control, patients with atopic dermatitis (AD) or urticaria]. However, CEA expression was detected diffusely and much more intensively in eight of the 10 AIGA cases included in this study. CEACAM1-expression was much more restricted on the apical membrane of glandular cells of both the AIGA cases and the other control subjects. While serum CEA levels increased in all five AIGA cases examined (5.8-43.2 ng/mL), it remained within normal limits in all control subjects: nine healthy individuals; 10 cases of AD; 10 cases of idiopathic urticaria; four cases of normohidrotic cholinergic urticaria (Mann-Whitney's U-test, P < 0.05). The increased serum CEA levels in AIGA decreased in conjunction with improved sweating during methyl prednisolone pulse therapy or repeated bathing. CONCLUSION: Serum CEA level may serve as a clinical marker for AIGA activity.

Myelopathy due to lumbar disc herniation in the presence of a tethered cord.

STUDY DESIGN: Single case report. OBJECTIVES: To present a case of lumbar disc herniation causing compression of a tethered cord that was successfully treated with lumbar decompression and fusion. BACKGROUND: A tethered cord is a rare pathology associated with a congenital spinal malformation, spinal dysraphism. Furthermore, myelopathy due to lumbar disc herniation in the presence of a tethered cord is extremely rare. METHODS: Single case report. RESULTS: A 43-year-old male with a history of spina bifida presented to our clinic for an evaluation of a progressive spastic gait disturbance and numbness in the lower limbs. A neurological examination revealed muscle weakness and pyramidal tract signs in the lower limbs. Magnetic resonance imaging of the lumbar spine showed disc herniation at L2-3 causing compression of a low-lying cord. Surgical intervention, including herniotomy via a posterolateral approach and instrumented posterolateral fusion, was performed, and a good outcome was achieved 1 year after the surgery. CONCLUSION: The potential for lumbar disc herniation in the presence of a tethered cord should be taken into account in the differential diagnosis of spinal pathologies causing spastic gait disturbances. Furthermore, posterior decompression and fusion is a useful treatment option in such cases.

Prognostic factors for cervical spondylotic amyotrophy: are signs of spinal cord involvement associated with the neurological prognosis?

OBJECTIVES: The purpose of this study was to clarify the prognostic factors for cervical spondylotic amyotrophy (CSA). METHODS: The authors retrospectively reviewed the medical records of 47 consecutive patients with CSA in whom the presence/absence of the pyramidal tract sign was noted. We analyzed whether the age, sex, presence of diabetes mellitus, medication (vitamin B12), type of the most atrophic and impaired muscle, the muscle strength at the presentation, the presence of the pyramidal tract sign, magnetic resonance imaging (MRI) findings, including the presence and number of T2 high signal intensity areas (T2 HIA) in the spinal cord and the conversion to surgery were associated with the recovery of muscle strength in the patients. In addition, we also investigated whether the duration of symptoms before surgery and the type of surgery were associated with the recovery of muscle strength in patients who required conversion to surgical treatment. RESULTS: The presence of T2 HIA on MRI (P=0.002), the number of T2 HIA on MRI (P=0.002) and conversion to surgery (P=0.015) were found to be significantly associated with a poorer recovery at the observational final follow-up. Further, the presence of the pyramidal tract sign (P=0.043) was significantly associated with a poor recovery at the final follow-up after surgery. CONCLUSION: The presence of a high signal intensity change on T2-weighted MRI and the pyramidal tract sign can be used as prognostic factors for patients with CSA.

The nuclear factor kappa B p50 subunit and cortactin as markers to distinguish between keratoacanthoma and well-differentiated squamous cell carcinoma.

BACKGROUND: Distinguishing keratoacanthoma (KA) from well-differentiated squamous cell carcinoma (SCC) is sometimes difficult. Recent evidence indicates that the nuclear factor kappa B p50 subunit (p50) and cortactin might be useful to distinguish between these two conditions. AIM: To verify whether p50 and cortactin are useful differentiation markers to distinguish between subungual KA and well-differentiated SCC. METHODS: Immunohistochemistry using p50, cortactin and Ki-67 was performed on 20 patients with KA and 20 patients with facial well-differentiated SC. Ki-67 staining was also evaluated and scored. RESULTS: Both p50 and cortactin had higher levels of expression in KA than in SCC. Both were localized to the basal-cell layer of KA, whereas they were scattered without polarity throughout the SCC lesions. Although the Ki-67 index was not significantly different between KA and SCC, the staining pattern also showed loss of polarity in SCC. CONCLUSION: p50 and cortactin might be useful makers to distinguish between KA and well-differentiated SCC.

Adult orbital xanthogranulomatous disease: adult-onset xanthogranuloma of periorbital location.

Adult orbital xanthogranulomatous disease (AOXGD) is a rare granulomatous disorder, which has four subtypes: adult-onset xanthogranuloma (AOX), adult-onset asthma with periocular xanthogranuloma, necrobiotic xanthogranuloma and Erdheim-Chester disease. We report a 42-year-old woman who presented with yellowish nonulcerative nodules on her eyelids. On histopathological examination of a nodule, mild degeneration of collagen fibres was seen, with surrounding infiltration of numerous foam cells and Touton giant cells in the deep dermis. Lymphoid follicles were seen in the reticular dermis. There was no apparent necrobiosis of collagen fibres. There were no clinical symptoms of asthma and no laboratory signs of paraproteinaemia during a follow-up of more than 5 years. We diagnosed this case as AOX, but further long-term follow-up would be required for the differentiation from the other AOXGDs. Dermatologists should be aware of these rare granulomatous disease conditions with ocular/orbital location, because they may cause ophthalmological complications.

Recalcitrant facial pemphigus vulgaris: correlation of skin lesions with the ratio of antidesmoglein antibodies 1 and 3.

Pemphigus vulgaris (PV) is an autoimmune bullous disease characterized by autoantibodies against desmogleins. We report a case of recalcitrant PV, which progressed from the mucosal to the mucocutaneous type, with a corresponding increase in anti-desmoglein (Dsg)1 and decrease in anti-Dsg3 antibody titres. Thus, the clinical features seemed to correlate with the ratio of anti-Dsg1 and 3. The patient also had anti-Dsg4 antibodies, which might be related to the nonscarring diffuse hair loss and marked facial involvement she also had. The patient did not respond to treatment with systemic steroid, ciclosporin, azathioprine, cyclophosphamide or double filtration plasmapheresis, and eventually died from fulminant thrombotic thrombocytopenic purpura of unknown cause.

Subungual keratoacanthoma: analysis of cell proliferation and copy number variation of oncogenes compared with periungual squamous cell carcinoma.

BACKGROUND: Subungual keratoacanthoma (SUKA) is a rare cutaneous tumour with several features distinct from ordinary KA. SUKA may not show spontaneous regression and sometimes grows progressively, resulting in phalangeal bone destruction. This makes its distinction from digital squamous cell carcinoma (SCC) difficult. Aim. To investigate differences in molecular expression between SUKA and digital SCC. METHODS: In addition to immunohistochemical analysis of Ki-67, one of the markers differentiating KA from SCC, we investigated the copy numbers of various oncogenes by multiplex ligation-dependent probe amplification (MLPA) using two cases of SUKA and three cases of periungual SCC. RESULTS: Ki-67 was moderately or strongly positive in SCC but negative in SUKA. The MLPA analysis showed that the nuclear factor (NF)κB1 and cortactin (CTTN; formerly known as EMS1) genes are amplified in SUKA but not in digital SCC. This increase in NFκB1 was confirmed by immunohistochemical analysis. CONCLUSION: NFκB1 could be a novel marker to differentiate between SUKA and SCC. Although this study was performed on limited numbers of patients with SUKA, MLPA analysis could be applied to differentiate other benign tumours from their malignant counterparts.

Serum cytokines and growth factor levels in Japanese patients with psoriasis.

BACKGROUND: Although the precise pathomechanism of psoriasis is still unknown, various cytokines and growth factors derived from T cells, dendritic cells or keratinocytes, are critically involved in this disease. There have been several studies determining the serum levels of cytokines in patients with psoriasis, but with conflicting results. The levels of various cytokines and growth factors were measured in the sera of patients with psoriasis and compared with those of healthy controls. The correlation with disease severity was also determined. METHODS: Sera were collected from 122 patients with psoriasis and 78 healthy controls for ELISA analysis to evaluate the levels of cytokines and growth factors. The severity of psoriasis was determined by the Psoriasis Area and Severity Index (PASI). RESULTS: Serum levels of tumour necrosis factor (TNF)-alpha, interferon (IFN)-gamma, interleukin (IL)-2, IL-6, IL-7, IL-8, IL-12, IL-17, IL-18 and vascular endothelial growth factor (VEGF) were significantly increased in patients with psoriasis compared with those of healthy controls. The serum levels of IL-2, soluble intercellular adhesion molecule-1, epidermal growth factor, hepatocyte growth factor and amphiregulin were not significantly different from those of healthy controls. Increased serum levels of TNF-alpha, IFN-gamma, IL-12, IL-17, IL-18 and VEGF correlated with PASI. Furthermore, these cytokine levels were decreased after psoriasis treatment. In contrast, serum levels of IL-10 were decreased in psoriasis and negatively correlated with PASI. DISCUSSION: Serum levels of TNF-alpha, IFN-gamma, IL2, IL-6, IL-7, IL-8, IL-12, IL-17, IL-18 and VEGF were positively correlated and that of IL-10 was negatively correlated with PASI in Japanese patients with psoriasis. These parameters might be useful for determining the disease activity of psoriasis.

Endoscopic submucosal dissection for colorectal tumors.

BACKGROUND: Endoscopic submucosal dissection (ESD) is a state-of-the-art method that enables resection of larger tumors than those resectable by conventional endoscopic mucosal resection (EMR). However, the individual role of each method in the treatment of colorectal tumors remains undetermined. OBJECTIVE AND METHODS: To consider the respective indications of ESD and EMR for colorectal tumors, we analyzed the results of the two treatments retrospectively. RESULTS: Tumors treated by ESD (44 tumors) were significantly larger, more often located in the rectum and more often coexistent with cancer than those treated by EMR (512 tumors). EMR was used in the majority of adenomas, and showed high rates of both one-piece resection (OPR) and complete resection (CR) for adenomas less than 20 mm. However, for adenomas and cancers greater or equal to 20 mm, the CR rate for EMR was significantly lower than that for ESD because of the incidence of OPR with a positive lateral margin (16% vs 0% with ESD vs EMR). Histopathology (cancer), size (> or =20 mm) and macroscopic type (laterally spreading tumors) were shown to be significant risk factors for that incidence. For tumors with these factors, ESD showed a higher CR rate than did EMR. However, ESD required longer operating times and tended to have a higher rate of perforation compared with EMR. ESD was aborted halfway in seven cases due to technical difficulties and perforation. CONCLUSION: ESD and EMR have different characteristics as treatment for colorectal tumors. Careful evaluation of the lesion and of the balance between benefits and risks are mandatory before selecting either of these treatments for colorectal tumors.

Morphological study of aneurysms at the junction of the superior cerebellar artery.

SUMMARY: Superior cerebellar artery (SCA) aneurysms sometimes involve the origin of the SCA making treatment difficult. We focused on the morphological characteristics of SCA aneurysms and adjacent vascular structures to apply clinical decision-making for the treatment strategy. Sixty-nine SCA aneurysms, including 34 ruptured and 35 unruptured ones, had been treated for over 12 years. Multiple aneurysms were associated in 30 patients. The pattern of the neck position of aneurysms was classified into three types: Type A: no SCA-involved type; Type B: half involved type with SCA originating from the aneurysmal neck; Type C: pure SCA aneurysm with all the neck mounting on SCA. Morphological and clinical analysis was done between ruptured and unruptured aneurysms and among the three types. There was no difference in patient profile between ruptured and unruptured aneurysms. The angle formed by the posterior cerebral artery and SCA on the aneurysm side was obtuse in 62 (90%) patients. From the morphological point of view the SCA-involved type (types B + C) was significantly more prevalent in ruptured aneurysms (77%). Bleb formation was particular in ruptured aneurysms. As for the treatment, the risk of SCA occlusion and incomplete and attempted operation was particularly high in cases with SCA-involved type. Although SCA aneurysms may grow due to the hemodynamic stress at the opened bifurcation between the PCA and SCA, the neck shifting to the origin of SCA, particularly in ruptured lesions,may suggest some other etiological mechanism. SCA-involved type aneurysms had a high treatment risk of SCA occlusion and tended to incomplete treatment to avoid such ischemic complications.

Hutchinson-Gilford progeria syndrome with severe skin calcinosis.

We describe a case of Hutchinson-Gilford progeria syndrome (HGPS) with long-term follow-up. A 1-month-old girl with marked sclerodermatous skin changes developed various symptoms of HGPS during follow-up. These included sclerotic skin, pigmentation, skin atrophy with translucent veins, wispy hair and alopecia, nail dystrophy and decreased sweating. Marked skin calcinosis was observed over almost the entire body, a symptom that has apparently been ignored in the literature. At 16 years old, the girl underwent surgery for a skull fracture and subdural haematoma, which was followed by chronic ulceration. Wet dressing with insulin-like growth factor was used with considerable effect. Mutation of the lamin A/C (LMNA) gene mutation, which encodes nuclear lamin A and C, has been reported to be the cause of HGPS. Our case showed the mutation G608G (GGC-->GGT), which resulted in a cryptic splice site and consequently in a truncated lamin A/C protein.

Endoscopic submucosal dissection for rectal tumors.

BACKGROUND AND STUDY AIM: Endoscopic submucosal dissection (ESD) has recently been developed for one-piece resection of gastric tumors. In order to improve patients' quality of life, it may be desirable to use the same technique for rectal tumors. METHODS: 35 consecutive patients with rectal tumors were enrolled. ESD was carried out using the same technique as for the stomach. The efficacy, technical feasibility, operation time, complications, and follow-up results were assessed. RESULTS: The mean size of the epithelial tumors was 26.2 +/- 14.0 mm, and the rates of one-piece resection and one-piece resection with tumor-free margins were 73.3% (22 of 30) and 70.0% (21 of 30), respectively. The median operation time was 70 min (range 8-360 min). All five carcinoid tumors were completely resected. No patient needed blood transfusion or had the complication of problematic bleeding. Perforation during ESD occurred in one patient (2.9%), who was managed with conservative medical treatment after endoscopic closure of the perforation. Excluding seven patients, who either underwent additional surgery or whose follow-up period was less than 1 year, all 23 patients with epithelial tumors were free of recurrence during a mean follow-up period of 25.7 months (range 12-53 months). CONCLUSIONS: ESD was thus found to be feasible for the treatment of rectal tumors, with promising results although the follow-up periods were short. ESD may therefore be indicated for rectal tumors which are not resectable en bloc by conventional procedures, in order to improve the patients' quality of life.

The current status of treatment strategies for cerebral aneurysms in nagoya university and affiliated hospitals based on a questionnaire survey.

SUMMARY: We investigated differences in the treatment strategies for ruptured aneurysms among 26 hospitals affiliated with Nagoya University and any changes in those strategies based on responses to a questionnaire.We also surveyed the data concerning patients with a ruptured aneurysm collected from our affiliated hospitals between 2001 and 2002. In half of the institutes, angiography is performed immediately after an urgent medical examination, there are only five hospitals (20%) which have a basic policy of terminating the angiography within three to six hours after onset. In half of the institutes, the timing of the treatment also depends on the location of the aneurysm. In particular, the treatment for vertebro-basilar aneurysms tends to be done the next day or later. Low-grade subarachnoid hemorrhage (SAH) patients with mild symptoms tended not to be given any sedative drugs (46%), while patients with SAH in some institutes were sedated without informed consent regardless of the severity. The main treatment method for most anterior circulation aneurysms was clipping. Concerning aneurysms located in the posterior circulation and the origin of the ophthalmic artery, clipping and coiling were equally selected. Almost all the hospitals (92%) responded that their treatment strategy had not changed even after the report of the International Subarachnoid Aneurysm Trial (ISAT). There is a great deal of difference in treatment strategies and indications among institutions. In particular, institutions without neuroendovascular interventionists (NETists) frequently persist in the conventional policy, making it urgently necessary to bring NETists up-to-date on the latest advance in endovascular treatment.

Effects of phthalate esters on the sensitization phase of contact hypersensitivity induced by fluorescein isothiocyanate.

BACKGROUND: Many different types of phthalate ester are used as plasticizers and are thus found in the air. There have been several studies that suggest an association between allergies and phthalate esters. We previously found that di-butyl phthalate (DBP) has an adjuvant effect in a mouse contact hypersensitivity model, in which fluorescein isothiocyanate (FITC) is involved as an immunogenic hapten. OBJECTIVE: We examined whether other phthalate esters enhance the process of sensitization to FITC by facilitating the trafficking of FITC-presenting dendritic cells or macrophages from skin sites to draining lymph nodes. METHODS: Mice were epicutaneously sensitized with FITC dissolved in acetone containing a phthalate ester. Sensitization was evaluated as ear swelling after a challenge with FITC. Draining lymph node cells obtained 24 h after skin sensitization were examined for FITC fluorescence by means of flow cytometry. FITC-positive cells were characterized with anti-CD11c and anti-CD11b by three-colour flow cytometry. RESULTS: When mice were sensitized with FITC in acetone containing DBP or di-n-propyl phthalate (DPP), strong enhancement of the ear-swelling response was observed. Di-methyl phthalate (DMP) and di-ethyl phthalate (DEP) were less effective but produced some enhancement. Consistent enhancement was not observed with di-(2-ethylhexyl) phthalate or di-isononyl phthalate. Upon sensitization in the presence of DBP or DPP, the number of FITC-positive dendritic cells (total CD11c+ as well as CD11c+/CD11b+) was increased in draining lymph nodes. As to the other four phthalate esters, there was no significant increase in the FITC-positive cell number in the draining lymph nodes. CONCLUSION: During the process of sensitization to FITC, DBP, and DPP exert strong adjuvant effects that are associated with enhancement of trafficking of antigen-presenting dendritic cells from the skin to draining lymph nodes.