Myasthenia gravis with alopecia totalis

Myasthenia gravis was diagnosed on the basis of a positive Tensilon test in a 4-year-old male child with alopecia totalis. His scalp hair and eyebrows had began to disappear at the age of 10 months. No other physical abnormalities such as motor paralysis, were seen except for left ptosis and alopecia totalis. His serum titer of anti-acetylcholine receptor antibody was elevated. No immune system abnormalities (C3, C4, CH50, C-reactive protein, antinuclear antibody or lymphocyte function disorders) were detected. Although alopecia areata and alopecia totalis are sometimes present in adults with autoimmune diseases and myasthenia gravis, this association is rare in children. The present case represents the youngest patient with myasthenia gravis associated with alopecia totalis.     

Positional relationships between the masticatory muscles and their innervating nerves with special reference to the lateral pterygoid and the midmedial and discotemporal muscle bundles of temporalis

For an accurate assessment of jaw movement, it is crucial to understand the comprehensive formation of the masticatory muscles with special reference to the relationship to the disc of the temporomandibular joint. Detailed dissection was performed on 26 head halves of 14 Japanese cadavers in order to obtain precise anatomical information of the positional relationships between the masticatory muscles and the branches of the mandibular nerve. After complete removal of the bony elements, the midmedial muscle bundle in all specimens and the discotemporal muscle bundle in 6 specimens, derivatives of the temporalis, which insert into the disc were observed. On the anterior area of the articular capsule and the disc of the temporomandibular joint, the upper head of the lateral pterygoid, the midmedial muscle bundle of temporalis and the discotemporal bundle of temporalis were attached mediolaterally, and in 3 specimens the posterosuperior margin of the zygomaticomandibularis was attached to the anterolateral area of the disc. It is suggested that these muscles and muscle bundles contribute to various mandibular movements. Although various patterns of the positional relationships between the muscles and muscle bundles and the their innervating nerves are observed in the present study, relative positional relationships of the muscles and muscle bundles and of nerves of the mandibular nerve are consistent. A possible scheme of the developmental formation of the masticatory muscles based on the findings of the positional relationships between the muscles and the nerves is presented.     

Topical Imiquimod as an Adjuvant to Laser Removal of Mature Tattoos in an Animal Model

BACKGROUND: Lasers are a commonly employed method of tattoo removal; however, numerous treatments are often needed and laser treatment may fail to eliminate the tattoo completely. It has been shown in animal studies that topical application of imiquimod cream, alone, fades tattoos. It is suspected that the combination of both imiquimod and laser treatment will result in enhanced tattoo pigment clearance. OBJECTIVE: The objective was to evaluate the efficacy of topical imiquimod cream as an adjuvant to laser removal of mature tattoos. METHODS: Fourteen albino guinea pigs were tattooed with black ink, then randomly assigned into two groups: one underwent sequential laser treatments with a Q-switched alexandrite laser in conjunction with triweekly applications of 5% imiquimod cream, while the other group underwent laser therapy alone. Subjects were evaluated with clinical photographs and skin biopsies after six laser treatment sessions. RESULTS: The combination laser and imiquimod treated group was clinically and histologically rated as having less pigment than the tattoos that were treated with laser alone (p=.012 and p=.047, respectively). Adjuvant imiquimod treatment had greater inflammation (p=.002) and fibrosis (p=.002) on posttreatment skin biopsies. CONCLUSION: Imiquimod appears to be a useful adjuvant to experimental laser tattoo removal in guinea pigs.     

Cardiovascular abnormalities in Noonan syndrome: The clinical findings and treatments

The clinical findings and treatment of cardiovascular abnormalities in 33 patients with Noonan syndrome are reviewed. Major cardiovascular abnormalities were pulmonary valvular stenosis in 17 patients (51.1%), hypertrophic cardiomyopathy (HCM) in 11 (33.3%), and atrial septal defect in 9 (27.3%). Dysplastic pulmonary valve was seen in 6 (35.3%). Balloon pulmonary valvuloplasty was performed for 4 patients with dysplastic pulmonary valves. Two patients were successfully treated. Surgical treatment was performed in 13 patients, 11 alive and 2 died. Two patients with HCM and arrhythmia died suddenly. In conclusion, balloon pulmonary valvuloplasty should be the initial palliation for dysplastic pulmonary valve in Noonan syndrome, and HCM is the risk factor for sudden death in Noonan syndrome.     

Urinary Excretion Ratio of Adrenaline to Noradrenaline in Patients with Pheochromocytoma

Clinically, patients with pheochromocytoma are divided intothree groups, adrenaline (A)- and noradrenaline (NA)-dominantand mixed type, according to their patterns of urinary excretionof A and NA. Our question was whether or not the dominance ofA or NA could convert to each other during the course of theillness. Serial determinations of A and NA in urine were carriedout in eight surgically verified cases of adrenal pheochromocytoma.Our results indicate that an individual patient has a fairlystable excretion ratio of A to NA for a relatively long period.Therefore, we propose that the ratio is determined at the beginningof tumor growth and is maintained throughout the illness.     

Pancreatic Internal Fistula to the Left Pleural Cavity

A case of recurrent pancreaticopleural effusion is presented. The pleural effusion with high enzyme and protein eouteuts resulted from a pancreatic internal fistula to the left pleural cavity. A sinus tract passing throngh esophageal hiatus was demonstrated by the endoscopic retrograde pancreatography (ERP). Surgical intervention afforded complete relief of abdominal pain and elimination of the pleural effusion.     

Efficacy and safety of hepatitis B vaccination in haemodialysis patients

Antibody response to vaccination with hepatitis B vaccine was evaluated in 39 haemodialysis patients. Three injections of 20 μg of the vaccine were given at time 0, 1 and 6 months. The seroconversion rate for the antibody to hepatitis B surface antigen (anti-HBs) was 2.6% at 1 month, 15.4% at 2 months, 33.3% at 4 months, 41.0% at 6 months and 59.0% at 7 months (males 53.6%, females 72.7%). Thus, with three injections, the seroconversion rate was significantly smaller and antibody titres were lower compared with 348 healthy control subjects. No correlation was observed between the anti-HBs seroconversion rate and lymphocyte subsets (OKT4/OKT8). When two additional injections were given at 9 and 10 months to 19 haemodialysis patients with an S/N ratio (the ratio of ct/min in the sample to mean ct/min in negative controls) smaller than 10 at 7 months, 16 patients (84.2%) developed anti-HBs and elevated antibody titres. Accordingly, the response rate of all haemodialysis patients 12 months after the first injection was elevated to 92.3% and was as high as that in normal subjects. At 24 months, the response rate had gradually declined to 64.1% accompanied with lowered antibody titres. There were no serious side effects.
From these results, it was concluded that the most effective dose and schedule for optimal hepatitis B immunization and booster injections should be decided in haemodialysis patients with low antibody titres.     

Vascular endothelial growth factor-C (VEGF-C) expression predicts lymph node metastasis of transitional cell carcinoma of the bladder

PURPOSE: It has been found that expression of vascular endothelial growth factor-C (VEGF-C) in several carcinomas is significantly associated with angiogenesis, lymphangiogenesis and regional lymph node metastasis. However, VEGF-C expression in bladder transitional cell carcinoma (TCC) has not yet been reported. To elucidate the role of VEGF-C in bladder TCC, we examined VEGF-C expression in bladder TCC and pelvic lymph node metastasis specimens obtained from patients who underwent radical cystectomy. METHODS: Eighty-seven patients who underwent radical cystectomy for clinically organ-confined TCC of the bladder were enrolled in the present study. No neoadjuvant treatments, except transurethral resection of the tumor, were given to these patients. The VEGF-C expressions of 87 bladder tumors and 20 pelvic lymph node metastasis specimens were examined immunohistochemically and the association between VEGF-C expression and clinicopathological factors, including angiogenesis as evaluated by microvessel density (MVD), was also examined. RESULTS: Vascular endothelial growth factor-C expression was found in the cytoplasm of tumor cells, but not in the normal transitional epithelium. Vascular endothelial growth factor-C expression was significantly associated with the pathological T stage (P = 0.0289), pelvic lymph node metastasis (P < 0.0001), lymphatic involvement (P = 0.0008), venous involvement (P = 0.0002) and high MVD (P = 0.0043). The multivariate analysis demonstrated that VEGF-C expression and high MVD in bladder TCC were independent risk factors influencing the pelvic lymph node metastasis. Moreover, the patients with VEGF-C-positive tumors had significantly poorer prognoses than those with the VEGF-C-negative tumors (P = 0.0087) in the univariate analysis. The multivariate analysis based on Cox proportional hazard model showed that the independent prognostic factors were patient age (P = 0.0132) and pelvic lymph node metastasis (P = 0.0333). CONCLUSION: The present study suggests that VEGF-C expression is an important predictive factor of pelvic lymph node metastasis in bladder cancer patients.     

Diagnosis of gastric adenoma versus early gastric cancer by Bromodeoxyuridine immunohistochemistry from gastric biopsy specimen

Differentiation between gastric adenomas and type IIa early gastric cancer by macroscopic and histologic examinations is often difficult. This study has shown that there are differences in the distribution of S-phase cells in these two types of tumour, and these differences can be exploited to differentiate between them. The distribution of S-phase cells was assessed by Bromodeoxyuridine (BrdU) immunohistochemistry using an in vitro BrdU-labelling method. Biopsy specimens were obtained from 27 lesions (12 adenomas and 15 type IIa early gastric cancers), and the distributions of BrdU-labelled cells in the tumour thickness as well as in the superficial layer of the tumour were determined. Significant difference was seen in the labelling of the two tumour types with regard to both these parameters. Using the criteria of BrdU immunohistochemisty, the accuracy of differentiation between gastric adenomas and cancers was 88.9%. Therefore, it is concluded in this study that in vitro BrdU labelling is a useful method of diagnosing gastric adenomas and cancers.     

Clinical evaluation of titration of hepatitis C virus core antibody and its subclasses

Abstract Titrations of anti-hepatitis C core (anti-HCc) immunoglobulin G (IgG) antibodies and its subclasses were studied in 90 patients with acute and chronic hepatitis C virus (HCV) infection, including 27 patients who underwent interferon (IFN) therapy. The positivity rates for each anti-HCc subclass were as follows: 95.2% for IgG1, 12.0% for IgG2, 69.9% for IgG3 and 19.3% for IgG4. The total anti-HCc IgG titre correlated well with the IgG1 titre, indicating that IgG1 was the main virus-specific IgG. Changes of IgG1 production mainly contributed to fluctuations of the anti-HCc IgG titre and corresponded well to positivity for HCV-RNA during and after IFN therapy. IgG3 was detected prior to IgG1 during the early phase of acute hepatitis in some cases and also appeared with relapse after IFN therapy. The serial assay of anti-HCc subclasses showed the patients' humoral immune response to HCV infection, and might be useful for evaluation of anti-viral immunity influenced by IFN therapy.