Hepatitis C treatment with triple therapy in a patient with hemophilia A

We report a case of successful treatment of chronic hepatitis C infection with telaprevir-based triple therapy in a patient with hemophilia A complicated by factor VIII inhibitor. A twenty-two years old male with hereditary hemophilia A and high-titer factor VIII inhibitor was taking maintenance doses of recombinant factor VIII. He visited our clinic for treatment of his chronic hepatitis C with the newly instituted protease inhibitor based therapy. He was diagnosed with hepatitis C genotype 1a at one year of age. He was initiated on telaprevir, ribavirin and peg-interferon for treatment of hepatitis C and qualified for response-guided therapy. He completed treatment at 24 wk with minimal adverse effects. Notably, after 4 wk of hepatitis C treatment, his factor VIII inhibitor screen was negative and the dose for recombinant factor VIII decreased by half of the initial dosing before he was treated for hepatitis C. We suspect that suppressing hepatitis C may help decrease factor VIII inhibitor level and the need for recombinant factor VIII.     

Delay in Seeking Medical Help in Patients with New‐Onset Erectile Dysfunction Remained High Over and Despite the PDE5 Era—An Ecological Study

IntroductionIt is common knowledge among researchers that erectile dysfunction (ED) is an important sentinel marker of cardiovascular and overall men's health.AimDetermine whether the delay of time between ED onset and seeking medical help (DSH), considered as a proxy of awareness of the importance of ED for overall men's health, has shortened during the phosphodiesterase type 5 inhibitors (PDE5) era.MethodsComplete data from 619 patients seeking first medical help for new‐onset ED as their primary disorder between July 2000 and July 2010 were analyzed (i.e., DSH, ED severity as defined by the International Index of Erectile Function‐erectile function [IIEF‐EF] domain score, patient's awareness of any PDE5, and Charlson Comorbidity Index [CCI]). Analysis of variance tested DSH throughout the 10‐year time frame. Cox regression models tested the association between predictors and DSH.Main Outcome MeasuresAssess if DSH has shortened throughout PDE5 era. Evaluate potential predictors of DSH.ResultsOverall, mean DSH was 30.2 months (median 12.0; range 5–300 months). DSH shortened throughout the analyzed 10‐year period (F = 1.918; P = 0.047), with a significant drop only from year 2009 (DSH up to year 2008 vs. from year 2009: 31.0 months [12.0] vs. 7.5 months [6.0], respectively; P < 0.001). Age, CCI, educational status, and ED severity did not significantly change over time. As a whole, 560 patients (90.5%) were aware of PDE5 at the time of their first office visit. PDE5 awareness emerged as an univarible and multivariable predictor of a shortened DSH. Conversely, DSH was not clearly associated with age, CCI, educational status, or ED severity.ConclusionDelay in seeking medical help in new‐onset ED patients remained high over the PDE5 era, with a significant drop only from the year 2009. PDE5 awareness emerged as an independent predictor of shortening of this delay. Salonia A, Ferrari M, Saccà A, Pellucchi F, Castagna G, Clementi MC, Matloob R, Briganti A, Rigatti P, and Montorsi F. Delay in seeking medical help in patients with new‐onset erectile dysfunction remained high over and despite the PDE5 era—An ecological study. J Sex Med 2012;9:3239–3246.     

A quantitative measure of handgrip myotonia in non‐dystrophic myotonia

Introduction: Non‐dystrophic myotonia (NDM) is characterized by myotonia without muscle wasting. A standardized quantitative myotonia assessment (QMA) is important for clinical trials. Methods: Myotonia was assessed in 91 individuals enrolled in a natural history study using a commercially available computerized handgrip myometer and automated software. Average peak force and 90% to 5% relaxation times were compared with historical normal controls studied with identical methods. Results: Thirty subjects had chloride channel mutations, 31 had sodium channel mutations, 6 had DM2 mutations, and 24 had no identified mutation. Chloride channel mutations were associated with prolonged first handgrip relaxation times and warm‐up on subsequent handgrips. Sodium channel mutations were associated with prolonged first handgrip relaxation times and paradoxical myotonia or warm‐up, depending on underlying mutations. DM2 subjects had normal relaxation times but decreased peak force. Sample size estimates are provided for clinical trial planning. Conclusion: QMA is an automated, non‐invasive technique for evaluating myotonia in NDM. Muscle Nerve 46: 482–489, 2012     

Histologic and electrophysiologic changes following subepineurial hematoma induction in rat sciatic nerve

The sciatic nerves of 34 rats were used to study the effects of intraneural, extrafascicular hematoma, exclusive of any other trauma. Electrodiagnostic, histologic, and electron microscopic techniques were used. The amount of nerve fiber damage was assessed using computerized planimetry. The effects of hematoma evacuation were also investigated. Changes consistent with partial denervation, such as prolongation of insertion activity and distal latency, and the presence of positive waves and fibrillations, were present in the blood-injected but not in the control groups. Degenerative nerve fiber changes localized to the area of hematoma were present in the blood-injected group and were significantly higher than those in the control group. Although there was a difference in the amount of nerve damage between the blood-injected and the hematoma-evacuated group (less damage), this difference was not statistically significant. These findings suggest that intraneural extrafascicular hematoma produces electrophysiologic and histologic evidence of axonal degeneration affecting nerve fibers in the vicinity of the hematoma. Early evacuation of the intraneural hematoma appears to slightly reduce the deleterious effects of the lesion.     

Eikenella corrodens in human mouth flora

The incidence of Eikenella corrodens in the human mouth was studied in 229 individuals. Six (2.6%) had positive cultures. The incidence of positive cultures for the saliva group was 0.6% and for the tooth-scraping group it was 8.2%. When 11 isolates were tested against 33 antimicrobials by disc-agar diffusion, 82% were susceptible to penicillin, cephalothin, and cefoperazone. Susceptibility to the remaining second- and third-generation cephalosporins studied was 100%. We suggest that the use of a second- or third-generation cephalosporin only is appropriate empiric therapy for human bite injuries.     

Should We Tailor the Clinical Management of Erectile Dysfunction According to Different Ages?

IntroductionThe impact of erectile dysfunction (ED) on patients’ sexual satisfaction and mood profile could differ across different ages.AimTo investigate the relationship between erectile function (EF), sexual satisfaction, and mood status among patients seeking medical help for ED.MethodsData from 765 patients presenting at a single center for ED were analyzed. Patients were categorized as young (≤50 years), middle-aged (>50 and ≤65 years), and old (>65 years) individuals and completed the International Index of Erectile Function (IIEF) and the Beck’s Inventory for Depression (BDI).Main Outcome MeasuresThe IIEF overall satisfaction and intercourse satisfaction domain scores and the BDI score were used to investigate sexual life satisfaction and depressive symptoms (defined as BDI > 11) across ages and according to ED severity. Linear and logistic regression analyses assessed the relationship between satisfaction scores and the risk of depressive symptoms with age and EF.ResultsMedian (interquartile range) age at first assessment for ED was 50 (38, 59) years. Compared with older men, young and middle-aged patients showed significantly higher IIEF-OS and IIEF– Intercourse Satisfaction scores for increasing IIEF-EF scores. Older men showed no difference in terms of satisfaction scores for mild ED and normal EF status. At linear regression analysis, both IIEF-EF and age were significantly associated with sexual satisfaction (all P < .0001). The interaction term between age and EF was also significant, suggesting that the older the patients, the higher the feeling of sexual satisfaction for the same EF status (P = .004). Overall, 25% of patients reported depressive symptoms. Logistic regression analysis showed a 40% risk of depressive symptoms for patients <45 years with severe ED compared to a risk <20% for a man >65 years of age with the same EF status.Clinical ImplicationsTreating older patients with mild ED may not lead to a further improvement in sexual satisfaction as compared with younger patients with the same ED severity. Younger ED patients suffer more from depressive symptoms compared with older men, regardless of ED severity, thus supporting the need for a comprehensive psychological counseling.Strength & LimitationsThe single-center design and the lack of the assessment of the impact of ED treatment are the main limits.ConclusionsThe clinical management of ED should be tailored according to different ages: younger patients deserve to be investigated and eventually treated for depressive symptoms. Older patients should be counseled for treatment when a sexual satisfaction improvement is expected.Capogrosso P, Ventimiglia E, Boeri L, et al. Should We Tailor the Clinical Management of Erectile Dysfunction According to Different Ages? J Sex Med 2019;16:999–1004.     

Microbiologic flora of human fingernails

The incidence of and nature of fingernail flora was studied in 20 patients. These patients underwent routine preoperative hand scrubbing in a clinical setting, without nail trimming or cleaning. Nineteen (95%) of 20 had a moderate to heavy growth of Staphylococcus epidermidis. These isolates were very susceptible to antibiotics. Thirteen (65%) of 20 patients had fungi (molds and yeast). In spite of adequate preoperative hand scrubbing, the fingernails were heavily contaminated. Such organisms may become pathogens and should not always be considered contaminants. This study also confirmed the importance of preoperative fingernail trimming and cleaning.     

Effects of a computerized working memory training program on working memory,attention, and academics in adolescents with severe LD and comorbid ADHD: a randomized controlled trial

Background: Youths with coexisting learning disabilities (LD) and attention deficit hyperactivity disorder (ADHD) are at risk for poor academic and social outcomes. The underlying cognitive deficits, such as poor working memory (WM), are not well targeted by current treatments for either LD or ADHD. Emerging evidence suggests that WM might be improved by intensive and adaptive computerized training, but it remains unclear whether this intervention would be effective for adolescents with severe LD and comorbid ADHD. Methods: A total of sixty 12‐ to 17‐year olds with LD/ADHD (52 male, 8 female, IQ > 80) were randomized to one of two computerized intervention programs: working memory training (Cogmed RM) or math training (Academy of Math) and evaluated before and 3 weeks after completion. The criterion measures of WM included auditory‐verbal and visual‐spatial tasks. Near and far transfer measures included indices of cognitive and behavioral attention and academic achievement. Results: Adolescents in the WM training group showed greater improvements in a subset of WM criterion measures compared with those in the math‐training group, but no training effects were observed on the near or far measures. Those who showed the most improvement on the WM training tasks at school were rated as less inattentive/hyperactive at home by parents. Conclusions: Results suggest that WM training may enhance some aspects of WM in youths with LD/ADHD, but further development of the training program is required to promote transfer effects to other domains of function.     

Arthrodesis of the spastic wrist.

The clinical and radiographic results of 9 patients (11 wrists) who had wrist arthrodeses for severe spastic flexion contracture were evaluated. The spasticity was due to cerebral palsy, traumatic head injury, and cerebrovascular accident. All wrist deformities were aesthetically unappealing and the patients or their caretakers had difficulty with hygiene or function. The subjective evaluation included overall satisfaction, hand hygiene, wrist deformity, functional improvement, and willingness to have surgery again given the same preoperative circumstances. A standardized hand function questionnaire was used to determine functional improvement following surgery. The objective evaluation included clinical evidence of fusion, skin condition, wrist position, and radiographic assessment. The average age of the patients was 22 years at the time of surgery and the average follow-up period was 32 months. All patients were satisfied with the results of the surgery and hygiene improved in all cases. None had palmar skin maceration or breakdown. All patients or their caretakers rated the overall appearance or wrist and hand deformity as improved and all but one patient would agree to have the surgery over again given the same preoperative circumstances. According to a 17-task hand function questionnaire, 8 of 9 patients (10 wrists) reported improved function after surgery. Face washing, propelling a wheelchair, and picking up both large and small objects were among the most frequently improved functions. Radiographic fusion was present in all cases. The average position of wrist fusion was 15 degrees flexion and the average amount of wrist correction was 85 degrees. Improved appearance, hygiene, and a certain degree of upper extremity function, regardless of cognitive abilities, can be expected following arthrodesis for severe spastic wrist deformity.     

A Canadian Perspective on the Treatment of Waldenström Macroglobulinemia

Waldenström macroglobulinemia (WM) is a slowly progressing B-cell non-Hodgkin lymphoma characterized by monoclonal IgM gammopathy in the blood and infiltration of the bone marrow by clonal lymphoplasmacytic cells. As an incurable disease, the goals for therapy for WM are to relieve symptoms, slow disease progression, prevent organ damage, and maintain quality of life. However, given the rarity of WM, clinical trials comparing treatments for WM are limited and there is no definitive standard of care. The selection of first-line WM therapy is thus based on patient factors, disease characteristics, and drug access, with bendamustine-rituximab and Bruton’s tyrosine kinase (BTK) inhibitor therapy considered preferred treatments. Other treatments such as proteasome inhibitor- or purine analogue-based therapy, alternative chemoimmunotherapy, and autologous stem cell transplantation are generally reserved for the relapsed setting but may be used in rare circumstances in earlier lines of therapy. This paper summarizes the efficacy and safety of these WM therapies and discusses considerations for treatment from a Canadian perspective.