Nevoid Basal cell carcinoma syndrome with medulloblastoma and meningioma--case report

A 35-year-old man presented with a rare case of nevoid basal cell carcinoma syndrome, or Gorlin's syndrome, associated with both medulloblastoma and meningioma, manifesting as visual field constriction due to multiple parasellar tumors. He had undergone resection of a medulloblastoma at the age of 1 year 9 months, followed by adjunctive irradiation with a total dose of 40 Gy. He presented with multiple subcutaneous nodules on his face and neck. Histological examination of biopsy specimens established the diagnosis of nevoid basal cell carcinoma syndrome. Tuberculum sellae meningioma was removed through a craniotomy, and his symptoms improved. Meningioma is known to occur in the field of therapeutic irradiation, so chemotherapy may be a better option for medulloblastoma associated with nevoid basal cell carcinoma syndrome.     

Impact of pre‐implant lower urinary tract symptoms on postoperative urinary morbidity after permanent prostate brachytherapy

Objectives: To assess the impact of baseline lower urinary tract symptoms on postoperative urinary morbidity in patients being treated for prostate cancer with 125‐I permanent prostate brachytherapy. Methods: A total of 104 prostate cancer patients were enrolled in this study. Their urinary morbidity was followed up using the International Prostate Symptom Score and Expanded Prostate Cancer Index Composite for 12 months or more after permanent prostate brachytherapy. Patients were classified into two groups based on their baseline International Prostate Symptom Score: the low International Prostate Symptom Score group (score ≤ 7) and the high International Prostate Symptom Score group (score ≥ 8). Urinary morbidity was estimated in each group based on the results of the International Prostate Symptom Score and Expanded Prostate Cancer Index Composite measured before permanent prostate brachytherapy, and at 1, 3, 6, 9 and 12 months after the end of all radiation therapy. Results: The overall mean total International Prostate Symptom Score, International Prostate Symptom Score quality of life score, and urinary‐related scores for Expanded Prostate Cancer Index Composite were significantly worse at 1 month after the end of treatment, but they improved gradually after the treatment and recovered to the baseline level within 12 months. Even in the high‐International Prostate Symptom Score group, the International Prostate Symptom Score and International Prostate Symptom Score Quality of Life score were significantly worse at 1–3 months after permanent prostate brachytherapy, and then recovered to the baseline level without prolongation. Although the urination‐related Expanded Prostate Cancer Index Composite score in the high‐International Prostate Symptom Score group was significantly worse at 1 month after permanent prostate brachytherapy in comparison with that in the low‐International Prostate Symptom Score group, it recovered to the baseline level without prolongation. Conclusions: The present findings suggest that the presence of lower urinary tract symptoms before implantation does not prolong urinary morbidity after permanent prostate brachytherapy.     

Strategy for prevention of hip fractures in patients with Parkinson’s disease

Hypovitaminosis D and K due to malnutrition or sunlight deprivation,increased bone resorption due to immobilization,low bone mineral density(BMD)and an increased risk of falls may contribute to an increased risk of hip fractures in patients with Parkinson’s disease.The purpose of the present study was to clarify the efficacy of interventions intended to prevent hip fractures in elderly patients with Parkinson’s disease.Pub Med was used to search the literature for randomized controlled trials(RCTs)regarding Parkinson’s disease and hip fractures.The inclusion criteria were 50 or more subjects per group and a study period of 1 year or longer.Five RCTs were identified and the relative risk and95%confidence interval were calculated for individual RCTs.Sunlight exposure increased serum hydroxyvitamin D[25(OH)D]concentration,improved motor function,decreased bone resorption and increased BMD.Alendronate or risedronate with vitamin D supplementation increased serum 25(OH)D concentration,strongly decreased bone resorption and increased BMD.Menatetrenone(vitamin K2)decreased serum undercarboxylated osteocalcin concentration,decreased bone resorption and increased BMD.Sunlight exposure(men and women),menatetrenone(women),alendronate and risedronate with vitamin D supplementation(women)significantly reduced the incidence of hip fractures.The respective RRs(95%confidence intervals)according to the intention-to-treat analysis were 0.27(0.08,0.96),0.13(0.02,0.97),0.29(0.10,0.85)and 0.20(0.06,0.68).Interventions,including sunlight exposure,menatetrenone and oral bisphosphonates with vitamin D supplementation,have a protective effect against hip fractures elderly patients with Parkinson’s disease.     

Falsely abnormally elevated blood trough concentration of tacrolimus measured by antibody-conjugated magnetic immunoassay in a renal transplant recipient: a case report

This report presents a falsely abnormally elevated blood trough concentration (C_t) of tacrolimus measured by antibody-conjugated magnetic immunoassay (ACMIA) methods in a renal transplant recipient. Because the C_t of tacrolimus was 78.5 ng/mL at day 2 after a 52-year-old man underwent renal transplantation, we stopped the tacrolimus extended-release formulation. However, because the abnormally elevated blood C_t continued in the range of 41.1-59.1 ng/mL, we then measured the tacrolimus concentration in a stored blood sample before renal transplantation, it was 43 ng/mL. Consequently, the day-7 blood sample was measured with both ACMIA and enzyme-linked immunoassay, showing C_t values of 42.8 ng/mL and 0.89 ng/mL, respectively. Because the abnormally elevated C_t was falsely measured by the ACMIA method, we restarted tacrolimus However, the calcineurin inhibitor was subsequently converted to cyclosporine at day 21 after renal transplantation. Although cyclosporine was also measured by ACMIA, there was not an abnormally elevated C_t. Subsequently, the tacrolimus concentration ratio in plasma and whole blood (P/B-tacrolimus concentration ratio) was measured by ACMIA in a posttacrolimus blood sample. The P/B-tacrolimus concentration ratio was 100%. In contrast, the P/B-tacrolimus concentration ratio was <30% in 2 control patients administered tacrolimus. It has been reported recently that there were cases showing falsely slightly elevated C_t of tacrolimus within the therapeutic range of concentrations. Therefore, we must be careful not to reduce the tacrolimus dose falsely. We consider confirmatory methods for a falsely abnormally elevated C_t of tacrolimus measured by ACMIA to (1) measure P/B-tacrolimus concentration ratio, (2) compare ACMIA with another measurement, and (3) evaluate a blood sample stored before tacrolimus administration.     

Urinary Cross-linked N-telopeptides of Type I Collagen Levels in Patients with Rheumatoid Arthritis

Osteoclastic activation rather than suppression of bone formation has been suggested to be the dominant process leading to bone loss in rheumatoid arthritis (RA). Although many studies have already shown the correlation of urinary pyridinoline (PYD) and deoxypyridinoline (DPD) levels with RA-related bone loss, urinary cross-linked N-telopeptides of type I collagen (NTx), a more specific marker of bone-derived type I collagen fragments in urine than urinary PYD and DPD in RA, has not been adequately studied. The purpose of the present study was to determine clinical factors that are associated with an increase in urinary NTx levels in patients with RA. One hundred and eighty-four patients with RA and 185 sex- and age-matched controls were enrolled in the study: 71 men, 37-68 years of age (RA: 31, controls: 40); 129 premenopausal women, 30-48 years of age (RA: 67, controls: 62), and 169 postmenopausal women, 48-69 years of age (RA: 86, controls: 83). The correlations of urinary NTx levels, measured by enzyme-linked immunosorbent assay with anatomic grade in the wrist, functional class, duration of disease, steroid use, modified health assessment questionnaire (HAQ) score for the upper and lower extremities, the levels of serum c-reactive protein and rheumatoid factor (RF), erythrocyte sedimentation rate, and/or years since menopause were examined by multiple regression analysis. Urinary NTx levels (nmol BCE/mmol Cr) did not differ significantly between men with RA and controls (53.2 +/- 29.6 vs 41.0 +/- 19.6, respectively), whereas urinary NTx levels were significantly higher in pre- and postmenopausal women with RA than in respective controls (premenopausal women: 57.1 +/- 36.6 vs 42.3 +/- 21.3, P <0.01; women: 76.2 +/- 27.3 vs 57.1 +/- 28.3, P <0.001). In men with RA, no clinical factors were significantly correlated with urinary NTx levels. In premenopausal women with RA, functional class, HAQ score for the upper extremities, and RF were significantly correlated with urinary NTx levels (all P <0.05); in postmenopausal women with RA, functional class and RF were significantly correlated with urinary NTx levels (both P <0.05). These findings suggest that urinary NTx levels were significantly higher only in women with RA than in age-matched controls, and a RA-related increase in urinary NTx levels may be associated with physical inactivity and disease activity.     

Treatment of Varicose Veins: An Assessment of Intraoperative and Postoperative Compression Sclerotherapy

Since thrombotic complications, such as superficial thrombophlebitis and subsequent skin pigmentation, are common after sclerotherapy, we conducted a study to evaluate whether combining sclerotherapy with ligation of varicose veins minimizes complications and what timing for sclerotherapy would be most beneficial-accompanying surgery or several weeks postsurgery. Surgical intervention and compression sclerotherapy were performed consecutively on 111 limbs (group A), and sclerotherapy was performed 28 days after surgical intervention on 87 limbs (group B). The volume of sclerosant used and the frequency of complications (thrombus formation and pigmentation) were analyzed. Plasma levels of thrombin-antithrombin III complex (TAT) and D-dimer (DD), as markers for activation of coagulation, were compared. In group A, the total volume of sclerosant used in patients with complications was significantly higher than that in patients without complications. The frequency of thrombus formation and of pigmentation was significantly lower (p <0.01) in group B (10% and 18%, respectively) than in group A (21% and 37%, respectively). The plasma levels of TAT 7 days after treatment were significantly lower in group B (3.4 +/- 1.2 mg/L) than in group A (4.9 +/- 1.1 mg/L). Performing compression sclerotherapy 28 days after surgical intervention is effective for reducing complications and a good alternative for patients with an underlying hypercoagulable state.     

Comparison of plasma levels of mature adrenomedullin and natriuretic peptide as markers of cardiac function in hemodialysis patients with coronary artery disease

BACKGROUND: It has been suggested that, like ANP and BNP, high plasma levels of mature adrenomedullin (mAM) indirectly reflect the severity of heart failure or renal failure. However, the relationship between mAM levels and hemodynamics and cardiac function has not been examined in hemodialysis (HD) patients with coronary artery disease (CAD). The best marker, among mAM, ANP and BNP, for left-ventricular function in those patients is also unclear. PATIENTS AND METHODS: Plasma levels of mAM, total AM (tAM), ANP and BNP were determined before HD in chronic HD patients with CAD (group 1; n = 17) and were compared with those of HD patients without cardiac disease (group 2; n = 22). We examined their relationship to hemodynamics and cardiac function in group 1 using data obtained by cardiac catheterization. RESULTS: Plasma levels of ANP and BNP were significantly higher in group 1 than in group 2, but there was no significant difference in plasma levels of mAM and tAM between the two patient groups. Plasma levels of both mAM and tAM significantly correlated with right atrial pressure (RAP), and only plasma tAM levels correlated with pulmonary artery pressure (PAP) and pulmonary artery wedge pressure (PAWP). However, no correlations were found between levels of the two forms of AM and ejection fraction (EF). In contrast, plasma ANP and BNP levels significantly correlated with both PAP and PAWP, and also with EF, although they did not correlate with RAP. The correlation of PAP and PAWP with ANP and BNP levels was closer than that with tAM levels. The most significant correlation was between BNP levels and EF (r = -0.756, p < 0.0001). CONCLUSIONS: Our results suggest that the mAM level may be less useful than natriuretic peptide levels as a marker of cardiac function in HD patients with CAD, and that the BNP level might be the best indicator of left-ventricular function. In addition, cardiac disease such as CAD may have a minor impact on mAM levels compared to renal failure.     

ABO-incompatible adult living donor liver transplantation for hepatocellular carcinoma

Living donor liver transplantation (LDLT) offers timely transplantation for patients with hepatocellular carcinoma (HCC). If ABO-incompatible LDLT is feasible, the need for pretransplantation treatment may be eliminated, which may reduce overall morbidity. In this article, we have described 8 adult HCC patients who successfully underwent LDLT from ABO-incompatible donors. Antirejection therapy included multiple preoperative plasmaphereses, splenectomy, and an immunosuppressive regimen with tacrolimus, methylprednisolone, and mycophenolate mofetil. The maintenance dose of immunosuppression did not differ from that of the ABO-identical cases. In addition, we also performed intrahepatic arterial infusion of prostaglandin E1. In 5 patients, we administered a single dose of rituximab, a chimeric CD20 monoclonal antibody. As a result of this treatment, 6/8 patients are still alive. Our experience has shown that it is possible to control antibody-mediated humoral rejection and other complications in adult ABO-incompatible LDLT.     

The frontal cortex is activated during learning of endoscopic procedures

Background

To date, several training and evaluation systems for endoscopic surgery have been developed, such as virtual-reality simulators and box trainers. However, despite current advances in these objective assessments, no functional brain studies during learning of endoscopic surgical skills have been carried out. In the present study, we investigated cortical activation using near-infrared spectroscopy (NIRS) during endoscopic surgical tasks.

Study design

A total of 21 right-handed subjects, comprising 4 surgical experts, 4 trainees, and 13 novices, participated in the study. Suturing and knot-tying tasks were performed in a box trainer. Cortical activation was assessed in all subjects by task-related changes in hemoglobin (Hb) oxygenation using NIRS.

Results

In surgical experts and novices with no experience of endoscopic surgical training, we found no changes in oxy-Hb, deoxy-Hb or total-Hb levels in any of the frontal channels. In surgical trainees and one novice with experience of endoscopic surgical training, we found significant increases in oxy-Hb and total-Hb levels in most of the frontal channels. There were significant differences in oxy-Hb and total-Hb levels in CH-19 between surgical experts and trainees (p = 0.02 for both), and between surgical trainees and novices with no experience of endoscopic surgical training (p = 0.008 for both). Furthermore, additional training increased oxy-Hb levels in the frontal cortex of novices with no experience of endoscopic surgical training but had no such effect on surgical experts.

Conclusions

The present data suggest that NIRS is a feasible tool for assessing brain activation during endoscopic surgical tasks, and may have a large impact on the future development of teaching, training, and assessment methods for endoscopic surgical skills.