Hidradenocarcinoma: A Case Series From the Scripps Clinic With a Systematic Review of the Literature.

BACKGROUND: Hidradenocarcinoma (HAC) is a rare adnexal carcinoma. To the best of the authors' knowledge, there are no published systematic reviews on HAC. OBJECTIVE: To incorporate a case series from the authors' institution and systematically integrate reported information to provide a reference tool for optimization of diagnosis and management. METHODS: A comprehensive MEDLINE search was conducted from database inception to 2021 using Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines. This yielded 225 studies with 165 cases of HAC. References of included articles were also searched. In addition, 9 patients with HAC were identified from the authors' institution over the past 10 years. RESULTS: The mean age of HAC presentation is 60 years with a slight male predilection (60%). The head and neck is the most commonly affected region. Over 36% of cases either presented with metastatic disease or went on to metastasize. The most common treatment type was wide local excision, followed by Mohs micrographic surgery. CONCLUSION: Early detection with accurate histologic interpretation is prudent in all cases of HAC. Wide local excision is the current first-line treatment. However, Mohs micrographic surgery offers complete marginal analysis with evidence of reduced risk of metastasis and better outcomes compared with wide local excision. Currently, there are no National Comprehensive Cancer Network guidelines for the treatment of HAC, and consensus guidelines are limited to tumor and nodal metastasis staging provided by the American Joint Committee on Cancer, eighth edition. Thus, this case series and systematic review integrates important aspects of diagnosis, workup, and management of HAC.

Delayed stabilisation of dynamically unstable syndesmotic injuries results in worse functional outcomes.

PURPOSE: Patients with primarily ligamentous injuries of the distal tibiofibular joint comprise up to 12% of all ankle sprains. Patients frequently present late after a syndesmosis injury and delayed treatment potentially leads to pain, prolonged disability and arthritis in the long term. This study aimed to assess clinical outcomes in patients who required syndesmosis fixation in the presence of arthroscopically proven instability, the hypothesis being that a delay to treatment would be associated with worse function. METHOD: A retrospective cohort study was performed of patients with dynamic instability requiring fixation between the years of 2010-2016. The procedures were performed by two foot and ankle fellowship trained orthopaedic surgeons, over three hospital sites. Patients were classified into three groups based on the time since injury to surgery, acute syndesmotic injury (< 6 weeks), sub-acute (6 weeks-6 months) and chronic syndesmotic injury (> 6 months). Functional scores were retrospectively collected using the Foot and Ankle Outcome Score (FAOS). RESULTS: Compared to patients with acute injuries, those with chronic injuries had significantly lower FAOS subscales (p < 0.001), with the greatest difference in quality of life (- 20.7, 95% CI - 31.6 to - 9.8, p = 0.012). There was a mean follow-up of 4.3 years. Although the average FAOS subscales in those with sub-acute injuries were lower than in those with acute injuries, the difference was not statistically significant. CONCLUSION: The results of this study suggest that delayed surgical stabilisation (> 6 months) is associated with significantly worse clinical function, and thus timely identification and early referral of those patients with potentially unstable syndesmotic injuries is recommended. LEVEL OF EVIDENCE: Level III.

Immunoreactive trypsinogen levels in newborn screened infants with an inconclusive diagnosis of cystic fibrosis.

BACKGROUND: Newborn screening (NBS) for cystic fibrosis (CF) not only identifies infants with a diagnosis of CF, but also those with an uncertain diagnosis of cystic fibrosis (CF), i.e. CF transmembrane conductance regulator (CFTR)-related metabolic syndrome (CRMS) or CF screen positive inconclusive diagnosis (CFSPID). These infants have an uncertain long-term outcome and it is currently unclear around time of diagnosis, which infants are at higher risk of later fulfilling a CF diagnosis. In this study, we hypothesised that immunoreactive trypsinogen (IRT) levels, used in NBS as a marker of pancreatic disease and function, may reflect the degree of CFTR dysfunction in each individual and therefore would help to identify those with CRMS/CSPID who are later at risk for meeting the criteria of CF. METHODS: In this longitudinal, prospective study, infants with CRMS/CFSPID and CF were recruited and followed in 9 CF clinics (Canada and Italy). We compared NBS IRT levels between CF and CRMS/CFSPID, and between children with CRMS/CFSPID→CF and CRMS/CFSPID→CRMS/CFSPID during the period of June 2007 to April 2016. RESULTS: Ninety eight CRMS/CFSPID and 120 CF subjects were enrolled. During the study period, 14 (14.3%) CRMS/CFSPID subjects fulfilled the diagnostic criteria for CF (CRMS/CFSPID→CF), while the diagnosis remained uncertain (CRMS/CFSPID→ CRMS/CFSPID) in 84 (85.7%) subjects. Significantly higher NBS IRT concentrations (ng/ml) were present in CF than CRMS/CFPSID (median (interquartile range): 143.8 (99.8-206.2) vs. 75.0 (61.0-105.9); P < 0.0001). Infants with CRMS/CFSPID→CF (n = 14) had significantly higher NBS IRT concentrations (ng/ml) than CRMS/CFSPID→ CRMS/CFSPID (n = 83) (median (interquartile range): 108.9 (72.3-126.8) vs. 73.7(60.0-96.0); P = 0.02). CONCLUSIONS: Amongst infants who tested positive on NBS for CF, there is a gradation of elevated NBS IRT concentrations. Infants with CF have higher NBS IRT levels than CRMS/CFPSID, and higher NBS IRT concentrations were present in infants with CRMS/CFSPID→CF than CRMS/CFSPID→ CRMS/CFSPID. NBS IRT concentrations, in concert with other factors, may have the potential to predict the likelihood of CF amongst infants with CRMS/CFSPID.

Shave removal for videogame controller-induced knuckle pads.

Videogame controller-induced knuckle pads may present in a strikingly unique distribution. Successful paring without recurrence can be achieved when combined with removal of the original insult.

Nebulized hypertonic saline in the treatment of viral bronchiolitis in infants.

OBJECTIVE: To investigate the use of nebulized 3% hypertonic saline (HS) for treating viral bronchiolitis in moderately ill hospitalized infants by a prospective, randomized, double-blinded, controlled, multicenter trial. STUDY DESIGN: A total of 96 infants (mean age, 4.7 months; range, 0.3 to 18 months) admitted to the hospital for treatment of viral bronchiolitis were recruited from 3 regional pediatric centers over 3 bronchiolitis seasons (December 2003 to May 2006). Patients were randomized to receive, in a double-blind fashion, repeated doses of nebulized 3% HS (treatment group) or 0.9% normal saline (NS; control group), in addition to routine therapy ordered by the attending physician. The principal outcome measure was hospital length of stay (LOS). RESULTS: On an intention-to-treat basis, the infants in the HS group had a clinically relevant 26% reduction in LOS to 2.6 +/- 1.9 days, compared with 3.5 +/- 2.9 days in the NS group (P = .05). The treatment was well tolerated, with no adverse effects attributable to the use of HS. CONCLUSIONS: The use of nebulized 3% HS is a safe, inexpensive, and effective treatment for infants hospitalized with moderately severe viral bronchiolitis.

Changes in pediatric tracheostomy tubes exposed to home dishwashing.

OBJECTIVE: Determine the effects of household dishwashing on Tracheostomy Tube safety. INTRODUCTION: Tracheostomy tubes accumulate biofilms, which may limit their lifespan. Frequent cleaning of the tubes is a method for biofilm prevention. Cleaning practices vary widely. Some families prefer dishwashing of tubes, but its effects are currently unknown. We hypothesize that dishwashing has no significant effect on the physical properties of tracheostomy tubes and can be recommended as a safe way to clean tracheostomy tubes. METHODS: Twenty 4.0 Shiley™ pediatric tracheostomy tubes were randomly assigned into dishwashed (DW) and non-dishwashed (NDW) groups, 10/group. DW tubes were subjected to 12 wash cycles. Each tube's hardness along with the surface spectra were analyzed to assess for chemical composition changes. Three cannula samples from each group were also randomly assessed with scanning-electron microscopy and scored by blinded examiners to assess for changes in surface heterogeneity. RESULTS: Hardness testing revealed a statistically significant difference (p = 0.0009) between the NDW and the DW group indicating increased fragility in the dishwashed tubes. Spectral analysis revealed loss of plasticizers, indicating decreased flexibility. Blinded electron microscopy scoring revealed increased surface heterogeneity in the DW group (p = 0.00007). CONCLUSION: A significant decrease in tube hardness and increased surface heterogeneity were found with dishwashing. The spectral analysis demonstrated increasing fragility. We believe these effects could potentially lead to decreased mechanical safety. With increased surface heterogeneity there is a greater potential for biofilm formation. At this time, dishwashing cannot be recommended as a tracheostomy tube cleaning method.

ARBITER: Arterial Biology for the Investigation of the Treatment Effects of Reducing Cholesterol: a randomized trial comparing the effects of atorvastatin and pravastatin on carotid intima medial thickness.

BACKGROUND: Whether marked LDL reduction to levels well below 100 mg/dL would further reduce the burden of cardiovascular disease is controversial. We compared the effects of 2 statins with widely differing potencies for LDL reduction (pravastatin 40 mg/d and atorvastatin 80 mg/d) on carotid intima-media thickness (CIMT). METHODS AND RESULTS: This was a single-center, randomized, clinical trial of 161 patients (mean age, 60 years; 71.4% male; 46% with known cardiovascular disease) that met National Cholesterol Education Program (NCEP) II criteria for lipid-lowering therapy. The effects of atorvastatin (80 mg/d; n=79) and pravastatin (40 mg/d; n=82) on CIMT were compared using blinded, serial assessments of the far wall of the distal common carotid artery. Baseline CIMT and other characteristics were similar between study groups. As anticipated, atorvastatin was substantially more potent for LDL reduction after 12 months: in the atorvastatin group, LDL cholesterol was 76+/-23 mg/dL after 12 months (-48.5%); LDL cholesterol was 110+/-30 mg/dL in the pravastatin group (-27.2%; P<0.001). Atorvastatin induced progressive CIMT regression over 12 months (change in CIMT, -0.034+/-0.021 mm), whereas CIMT was stable in the pravastatin group (change of 0.025+/- 0.017 mm; P=0.03). CONCLUSIONS: Marked LDL reduction (<100 mg/dL) with a high-potency statin provides superior efficacy for atherosclerosis regression at 1 year. This early effect on CIMT, a surrogate for clinical benefit, suggests that marked LDL reduction with synthetic statins may provide enhanced reduction in clinical coronary event rates.

Exercise testing in Wolff-Parkinson-White syndrome: case report with ECG and literature review.

ECG changes during exercise stress testing, such as false-positive ST-segment depression and disappearance of the delta wave, are reported in patients with the Wolff-Parkinson-White (WPW) pattern. We present a case of exercise testing in a 53-year-old man with WPW syndrome with ischemic-appearing ECG changes and normal nuclear stress perfusion study findings who was thought to be at clinically low risk for having significant coronary disease. A literature review is discussed. Although ST-segment depression typical for ischemia occurs in half of the patients in whom WPW syndrome is reported, exercise testing is still an important tool in their evaluation. Data other than ECG response can be interpreted in the context of clinical history and physical examination findings to stratify the risk of coronary disease. Complete and sudden disappearance of the delta wave has been seen during exercise in 20% of patients with WPW syndrome and can identify those who are at low risk for sudden arrhythmic death.

Importance of transesophageal echocardiography in the evaluation of Staphylococcus aureus bacteremia.

BACKGROUND AND AIM OF THE STUDY: Staphylococcus aureus is a leading cause of bacteremia and is often associated with endocarditis. The diagnosis of endocarditis may be missed when relying on clinical risk prediction, and this has led others to recommend transesophageal echocardiography (TEE) for diagnosis in most cases of S. aureus bacteremia (SAB). The study aim was to determine the likelihood of finding vegetations on TEE in patients with SAB in a suburban teaching hospital setting, and to identify risk factors predictive of vegetation on TEE. METHODS: All cases of SAB at Walter Reed Army Medical Center between January 2000 and May 2003 were evaluated. The prevalence of vegetations was determined in those cases selected for TEE. Potential risk factors for endocarditis were analyzed by review of medical records. RESULTS: A total of 176 patients had documented SAB during the time frame of the study, and 64 of these had TEE performed. Among the latter patients, 14% had a previously unidentified vegetation discovered by TEE. Patients with vegetation on TEE were as likely as those without vegetation to have nosocomial bacteremia, an alternate source of infection, and lack of valvular disease by prior surface echocardiography. Patients with a vegetation were significantly older (mean age 68.4+/-10.9 versus 54.6+/-19.6 years; p = 0.04). CONCLUSION: TEE identified a significant number of vegetations resulting from SAB. The clinical risk profile and transthoracic echocardiography did not reliably exclude vegetation. These findings support the liberal use of TEE for the diagnosis of SAB.

Inconclusive diagnosis of cystic fibrosis after newborn screening.

OBJECTIVES: To prospectively study infants with an inconclusive diagnosis of cystic fibrosis (CF) identified by newborn screening (NBS; "CF screen positive, inconclusive diagnosis" [CFSPID]) for disease manifestations. METHODS: Infants with CFSPID and CF based on NBS from 8 CF centers were prospectively evaluated and monitored. Genotype, phenotype, repeat sweat test, serum trypsinogen, and microbiology data were compared between subjects with CF and CFSPID and between subjects with CFSPID who did (CFSPID→CF) and did not (CFSPID→CFSPID) fulfill the criteria for CF during the first 3 years of life. RESULTS: Eighty-two subjects with CFSPID and 80 subjects with CF were enrolled. The ratio of CFSPID to CF ranged from 1:1.4 to 1:2.9 in different centers. CFTR mutation rates did not differ between groups; 96% of subjects with CFSPID and 93% of subjects with CF had 2 mutations. Subjects with CFSPID had significantly lower NBS immunoreactive trypsinogen (median [interquartile range]:77 [61-106] vs 144 [105-199] µg/L; P < .0001) than did subjects with CF. Pseudomonas aeruginosa and Stenotrophomonas maltophilia were isolated in 12% and 5%, respectively, of subjects with CFSPID. CF was diagnosed in 9 of 82 (11%) subjects with CFSPID (genotype and abnormal sweat chloride = 3; genotype alone = 4; abnormal sweat chloride only = 2). Sweat chloride was abnormal in CFSPID→CF patients at a mean (SD) age of 21.3 (13.8) months. CFSPID→CF patients had significantly higher serial sweat chloride (P < .0001) and serum trypsinogen (P = .009) levels than did CFSPID→CFSPID patients. CONCLUSIONS: A proportion of infants with CFSPID will be diagnosed with CF within the first 3 years. These findings underscore the need for clinical monitoring, repeat sweat testing at age 2 to 3 years, and extensive genotyping.

Effect of atorvastatin and pravastatin on serum C-reactive protein.

BACKGROUND: Extra-lipid effects of statins, such as anti-inflammatory actions, may contribute to their clinical benefit. These effects, with important implications for the concept of a statin "class effect," may be drug specific or may be related to the extent of lipid lowering. METHODS: We randomized 130 patients to treatment with either atorvastatin (80 mg daily, n = 63) or pravastatin (40 mg daily, n = 67), and measured serum lipids, C-reactive protein, and fibrinogen at baseline and after 3 months of therapy. RESULTS: Mean C-reactive protein (CRP) levels were significantly reduced in both groups, with a 36% reduction in the atorvastatin group (0.39 +/- 0.36 to 0.25 +/- 0.27, P =.001) and a 22% reduction observed in the pravastatin group (0.40 +/- 0.33 to 0.31 +/- 0.32, P =.003). A reduced or unchanged CRP level was seen in 67.2% of pravastatin-treated patients (45/67) and 73% of atorvastatin- treated patients (46/63) (P =.47). There was no difference between drugs in either the absolute or relative reductions in CRP levels. However, whereas the reduction of CRP with pravastatin was unrelated to the degree of low-density lipoprotein reduction (r = -.05, P =.69), atorvastatin-induced CRP reductions correlated directly to the change in low-density lipoprotein-C (r =.33, P =.009). CONCLUSIONS: High-dose atorvastatin and pravastatin both reduce CRP levels. However, whereas pravastatin's effect on CRP is independent of lipid-lowering efficacy, these data suggest that lipid-dependent mechanisms are, at least in part, active in atorvastatin-treated patients.

Usefulness of lowering low-density lipoprotein cholesterol to <70 mg/dl and usefulness of C-reactive protein in patient selection.

C-reactive protein levels may identify patients likely to benefit from lowering low-density lipoprotein (LDL) cholesterol to ultra-low levels. We find that above-average C-reactive protein with statin therapy predicts failure of carotid intima-media thickness regression in those with currently defined optimal LDL cholesterol (<100 mg/dl) but not if LDL cholesterol is <70 mg/dl.

Meta-analysis of randomized clinical trials on the usefulness of acetylcysteine for prevention of contrast nephropathy.

Multiple small studies of oral N-acetylcysteine for prevention of contrast nephropathy have been performed, demonstrating variable efficacy. We performed a meta-analysis of the randomized clinical trials to clarify the degree of benefit.

Marked low-density lipoprotein cholesterol reduction below current national cholesterol education program targets provides the greatest reduction in carotid atherosclerosis.

BACKGROUND: Current National Cholesterol Education Program (NCEP) guidelines recognize low-density lipoprotein cholesterol (LDL-C) below 100 mg/dl as an optimal level. Evidence supporting this is scant. Both LDL-C and C reactive protein (CRP) are known correlates of atherosclerosis progression. HYPOTHESIS: We examined the effect of final LDL-C and CRP obtained with statin therapy on carotid intima-media thickness (CIMT), a valid surrogate for clinical benefit of lipid-lowering therapies. METHODS: In a randomized, single-center trial, 161 patients were assigned to statin therapy of different potencies (pravastatin 40 mg, n = 82; atorvastatin 80 mg, n = 79). The effects on CIMT were assessed in relationship to LDL-C and CRP levels obtained after 12 months of therapy. RESULTS: Changes in CIMT were directly related to the final LDL-C level obtained on statin therapy after 12 months (R = 0.219, p = 0.015). Carotid intima-media thickness regression was seen in 61% of the subjects in the lowest quartile of final LDL-C (< 70 mg/dl) versus 29% of the subjects with the highest quartile of final LDL-C (> or = 114 mg/dl, p = 0.008). No threshold value was seen, with more favorable effects on absolute change in CIMT with lower values of LDL-C (decrease in CIMT of 0.06 +/- 0.17 mm in the lowest quartile compared with an increase of 0.06 +/- 0.09 in the highest quartile of LDL-C, p = 0.008). On-treatment LDL and CRP concentrations both below the group median values were associated with the greatest likelihood of CIMT regression. CONCLUSIONS: Regression of carotid atherosclerosis is directly related to the absolute LDL-C level on statin therapy. The greatest regression was obtained with an LDL-C < 70 mg/dl, supporting marked LDL-C reduction to levels below current NCEP guidelines.

Effect of inhaled hypertonic saline on hospital admission rate in children with viral bronchiolitis: a randomized trial.

OBJECTIVE: We sought to determine whether inhaled 3% hypertonic saline (HS) reduces admission to hospital in ambulatory children with moderately severe viral bronchiolitis. Secondary objectives compared changes in respiratory scores before and after treatment and assessed the need for unscheduled medical intervention within 7 days. METHODS: Children under the age of 2 years presenting with moderately severe viral bronchiolitis to the emergency department of 4 general hospitals from November 2008 to March 2009 were randomly assigned to receive 3 consecutive 4-mL doses of nebulized 3% HS (treatment group) or 0.9% normal saline (NS; control group) in a double blind fashion, each coadministered with 1 mg salbutamol. Outcome measures included the difference in hospital admission rate and changes in respiratory distress scores. RESULTS: A total of 81 children (mean age 8.9 mo, range 0.7-22 mo) were assessed over 88 visits on an intention-to-treat basis. No statistically significant differences were found between treatment groups. Children in the HS group had a nonsignificant trend toward greater improvement compared with NS controls with a same-day admission rate of 18% (95% confidence interval [CI] 9%-32%) versus 27% (95% CI 16%-42%), respectively. Respiratory Assessment Change Scores (RACS) favoured the HS group over NS controls (mean RACS 4.7 [95% CI 3.6-5.8] v. 3.7 [95% CI 2.5-4.9], respectively), although the CIs overlap and these differences were not statistically significant. CONCLUSION: The short-term use of nebulized 3% HS did not result in any statistically significant benefits, although a nonsignificant trend toward a decrease in admission rate and improvement in respiratory distress was found. A larger study would be required to determine whether these trends arise from a clinically relevant treatment effect.