Fusiform dilatation of the internal carotid artery in childhood-onset craniopharyngioma: multicenter study on incidence and long-term outcome

Purpose

Fusiform dilatations of the internal carotid artery (FDCA) represent a vascular complication following surgery for suprasellar tumors in children. Incidence rate and long-term prognosis of FDCA in terms of survival rates, vascular complications, and quality of survival are unknown for patients with childhood-onset craniopharyngioma.

Methods

Magnetic resonance imaging (MRI) results of 583 patients with childhood-onset craniopharyngioma, recruited from 2001 to 2015 in the German Childhood Craniopharyngioma Registry, were reviewed for FDCA. Risk factors for FDCA and long-term outcome after FDCA were analyzed.

Results

Fourteen of 583 patients (2.4 %) developed FDCA based on reference assessment of MRI. FDCA occurred ipsilateral to the surgical approach and was not related to degree of resection, hypothalamic involvement, or irradiation. The median time interval between first detection of FDCA and initial surgery was 0.79 years (range 0.01–5.56 years). During a median follow-up of 6.47 years (range 1.2–21.9 years) after first detection of FDCA, no bleeding or cerebrovascular events were observed in any patient. Irradiation was not related to FDCA. Survival rates and functional capacity were similar in patients with and without FDCA. Clinically the FDCA was unapparent in all cases and not treated.

Conclusion

FDCA is a rare complication related to surgical treatment of childhood-onset craniopharyngioma without major impact on prognosis and clinical course of the disease.

Clinical trial number

KRANIOPHARYNGEOM 2000—NCT00258453; KRANIOPHARYNGEOM 2007—NCT01272622.

     

Low concordance between surgical and radiological assessment of degree of resection and treatment-related hypothalamic damage: results of KRANIOPHARYNGEOM 2007

Background

Assessment of presurgical hypothalamic involvement (psHI) and treatment-related hypothalamic damage (trHD) is relevant for the decision on risk-adapted treatment and rehabilitation strategies in craniopharyngioma.

Patients and methods

129 surgical reports of childhood-onset craniopharyngioma patients recruited 2007–2014 in KRANIOPHARYNGEOM 2007 were analyzed. Data on psHI were available based on surgeon’s (63%), reference neuroradiologist’s (95%), and local radiologist’s (23%) assessment. The surgical degree of resection (DoR) was assessed by neurosurgeon (95%), reference neuroradiologist (73%), and local radiologist (61%). TrHD was assessed by neurosurgeon (33%), by reference neuroradiologist (95%), and by local radiologist (2%). Neurosurgical center size was categorized based on patient load.

Results

Surgical assessments on psHI (n?=?78), DoR (n?=?89) and trHD (n?=?42) as documented in surgical reports could be compared with the assessment of respective parameters by reference neuroradiologist. Differences with regard to DoR (p?=?0.0001) and trHD (p?<?0.0001) were detectable between surgeon’s and reference neuroradiologist’s assessment, whereas psHI was assessed similarly. Concordance for DoR and trHD was observed in 48 and 62%, respectively. Surgeons estimated a higher rate of complete resections and a lower rate of trHD. Neuroradiological reference assessment of trHD had higher predictive value for hypothalamic sequelae then surgical assessment. Observed differences were not related to neurosurgical center size.

Conclusions

Observed differences between surgical and neuroradiological estimation of risk factors in craniopharyngioma support the necessity of neuroradiological reference review to assure standards of quality. This could be established by central internet-based neuroradiological review in KRANIOPHARYNGEOM 2007. Standardization of surgical reports including specific assessment of tumor/damage location is recommended.

     

Adipsic diabetes insipidus in adult patients

Introduction

Adipsic diabetes insipidus (ADI) is a very rare disorder, characterized by hypotonic polyuria due to arginine vasopressin (AVP) deficiency and failure to generate the sensation of thirst in response to hypernatraemia. As the sensation of thirst is the key homeostatic mechanism that prevents hypernatraemic dehydration in patients with untreated diabetes insipidus (DI), adipsia leads to failure to respond to aquaresis with appropriate fluid intake. This predisposes to the development of significant hypernatraemia, which is the typical biochemical manifestation of adipsic DI.

Methods

A literature search was performed to review the background, etiology, management and associated complications of this rare condition.

Results

ADI has been reported to occur in association with clipping of an anterior communicating artery aneurysm following subarachnoid haemorrhage, major hypothalamic surgery, traumatic brain injury and toluene exposure among other conditions. Management is very difficult and patients are prone to marked changes in plasma sodium concentration, in particular to the development of severe hypernatraemia. Associated hypothalamic disorders, such as severe obesity, sleep apnoea and thermoregulatory disorders are often observed in patients with ADI.

Conclusion

The management of ADI is challenging and is associated with significant morbidity and mortality. Prognosis is variable; hypothalamic complications lead to early death in some patients, but recent reports highlight the possibility of recovery of thirst.

     

Cancer Risk Communication with Low Health Literacy Patients: A Continuing Medical Education Program

BACKGROUND

Low health literacy (HL) is an important risk factor for cancer health disparities.

OBJECTIVE

Describe a continuing medical education (CME) program to teach primary care physicians (PCP) cancer risk communication and shared decision-making (SDM) with low HL patients and baseline skills assessment.

DESIGN

Cluster randomized controlled trial in five primary care clinics in New Orleans, LA.

PARTICIPANTS

Eighteen PCPs and 73 low HL patients overdue for cancer screening.

INTERVENTION

Primary care physicians completed unannounced standardized patient (SP) encounters at baseline. Intervention physicians received SP verbal feedback; academic detailing to review cancer screening guidelines, red flags for identifying low HL, and strategies for effective counseling; and web-based tutorial of SP comments and checklist items hyperlinked to reference articles/websites.

MAIN MEASURES

Baseline PCP self-rated proficiency, SP ratings of physician general cancer risk communication and SDM skills, patient perceived involvement in care.

RESULTS

Baseline assessments show physicians rated their proficiency in discussing cancer risks and eliciting patient preference for treatment/decision-making as “very good”. SPs rated physician exploration of perceived cancer susceptibility, screening barriers/motivators, checking understanding, explaining screening options and associated risks/benefits, and eliciting preferences for screening as “satisfactory”. Clinic patients rated their doctor’s facilitation of involvement in care and information exchange as “good”. However, they rated their participation in decision-making as “poor”.

DISCUSSION

The baseline skills assessment suggests a need for physician training in cancer risk communication and shared decision making for patients with low HL. We are determining the effectiveness of teaching methods, required resources and long-term feasibility for a CME program.

     

Disseminated nocardiosis caused by <Emphasis Type="Italic">Nocardia elegans</Emphasis>: a case report and review of the literature

Background

Disseminated nocardiosis is a rare disease mostly occurring in immunocompromised patients.

Methods

We report a case of disseminated nocardiosis in a diabetic patient with both pulmonary and cutaneous involvement. Nocardia elegans was isolated and identified using the 16s ribosomal RNA gene sequence data.

Results

Clinical improvement was observed within 3 months after initiation of antimicrobial treatment with oral doxycycline, trimethoprim-sulfamethoxazole and intravenous penicillin, but the patient died 5 months later after arbitrary discontinuation of the treatment.

Conclusions

This is the first case report of disseminated nocardiosis caused by Nocardia elegans in China.

     

Effects of a Video on Organ Donation Consent Among Primary Care Patients: A Randomized Controlled Trial

BACKGROUND

Low organ donation rates remain a major barrier to organ transplantation.

OBJECTIVE

We aimed to determine the effect of a video and patient cueing on organ donation consent among patients meeting with their primary care provider.

DESIGN

This was a randomized controlled trial between February 2013 and May 2014.

SETTING

The waiting rooms of 18 primary care clinics of a medical system in Cuyahoga County, Ohio.

PATIENTS

The study included 915 patients over 15.5 years of age who had not previously consented to organ donation.

INTERVENTIONS

Just prior to their clinical encounter, intervention patients (n?=?456) watched a 5-minute organ donation video on iPads and then choose a question regarding organ donation to ask their provider. Control patients (n?=?459) visited their provider per usual routine.

MAIN MEASURES

The primary outcome was the proportion of patients who consented for organ donation. Secondary outcomes included the proportion of patients who discussed organ donation with their provider and the proportion who were satisfied with the time spent with their provider during the clinical encounter.

KEY RESULTS

Intervention patients were more likely than control patients to consent to donate organs (22 % vs. 15 %, OR 1.50, 95%CI 1.10–2.13). Intervention patients were also more likely to have donation discussions with their provider (77 % vs. 18 %, OR 15.1, 95%CI 11.1–20.6). Intervention and control patients were similarly satisfied with the time they spent with their provider (83 % vs. 86 %, OR 0.87, 95%CI 0.61–1.25).

LIMITATION

How the observed increases in organ donation consent might translate into a greater organ supply is unclear.

CONCLUSION

Watching a brief video regarding organ donation and being cued to ask a primary care provider a question about donation resulted in more organ donation discussions and an increase in organ donation consent. Satisfaction with the time spent during the clinical encounter was not affected.

TRIAL REGISTRATION

clinicaltrials.gov Identifier: NCT01697137

     

Implementation of a Family Intervention for Individuals with Schizophrenia

BACKGROUND

Families are rarely included in clinical care despite research showing that family involvement has a positive effect on individuals with schizophrenia by reducing relapse, improving work functioning, and social adjustment.

OBJECTIVES

The VA QUERI study, EQUIP (Enhancing QUality of care In Psychosis), implemented family services for this population.

DESIGN

At two VA medical centers, veterans with schizophrenia and their clinicians were interviewed separately at baseline and 15 months. A family intervention was implemented, and a process evaluation of the implementation was conducted.

PARTICIPANTS

Veterans with schizophrenia (n?=?173) and their clinicians (n?=?29).

INTERVENTION

Consent to contact family was obtained, mailers to engage families were sent, families were prioritized as high need for family services, and staff volunteers were trained in a brief three-session family intervention.

MAIN RESULTS

Of those enrolled, 100 provided consent for family involvement. Seventy-three of the 100 were sent a mailer to engage them in care; none became involved. Clinicians were provided assessment data on their patients and notified of 50 patients needing family services. Of those 50, 6 families were already involved, 34 were never contacted, and 10 were contacted; 7 new families became involved in care. No families were referred to the family psychoeducational program.

CONCLUSIONS

Uptake of the family intervention failed due to barriers from all stakeholders. Families did not respond to the mailer, patients were concerned about privacy and burdening family, clinicians had misperceptions of family-patient contact, and organizations did not free up time or offer incentives to provide the service. If a full partnership with patients and families is to be achieved, these barriers will need to be addressed, and a family-friendly environment will need to be supported by clinicians and their organizations. Applicability to family involvement in other disorders is discussed.

     

Antithrombotic Therapy for ACS in Elderly Patients

Background

Patients over 75 account for more than one third of those presenting with myocardial infarction and more than 50% of intrahospital mortality. There are no specific guidelines for the management of acute coronary syndromes (ACS) in the elderly.

Setting

Although antithrombotic therapy seems to be effective and safe in such patients, it requires specific precautions and treatment adjustments because of the higher bleeding risk due to comorbidities such as renal function impairment and malnutrition.

Results

Scientific evidence concerning elderly patients is scarce as they are either excluded or underrepresented in most randomized trials. Overall, the antithrombotic therapy needs to be adapted to avoid complications, mainly bleeding complications, without compromising the effectiveness of the treatment in this high-risk population.

Conclusion

In the present paper, we review the current treatment strategies in ACS while focusing on data concerning the elderly, according to available data in pivotal trials and in both AHA/ACC and ESC guidelines.

     

Adenosine kinase deficiency: expanding the clinical spectrum and evaluating therapeutic options

Background

Adenosine kinase deficiency is a recently described defect affecting methionine metabolism with a severe clinical phenotype comprising mainly neurological and hepatic impairment and dysmorphism.

Methods

Clinical data of 11 additional patients from eight families with adenosine kinase deficiency were gathered through a retrospective questionnaire. Two liver biopsies of one patient were systematically evaluated.

Results

The main clinical symptoms are mild to severe liver dysfunction with neonatal onset, muscular hypotonia, global developmental retardation and dysmorphism (especially frontal bossing). Hepatic involvement is not a constant finding. Most patients have epilepsy and recurrent hypoglycemia due to hyperinsulinism. Major biochemical findings are intermittent hypermethioninemia, increased S-adenosylmethionine and S-adenosylhomocysteine in plasma and increased adenosine in urine. S-adenosylmethionine and S-adenosylhomocysteine are the most reliable biochemical markers. The major histological finding was pronounced microvesicular hepatic steatosis. Therapeutic trials with a methionine restricted diet indicate a potential beneficial effect on biochemical and clinical parameters in four patients and hyperinsulinism was responsive to diazoxide in two patients.

Conclusion

Adenosine kinase deficiency is a severe inborn error at the cross-road of methionine and adenosine metabolism that mainly causes dysmorphism, brain and liver symptoms, but also recurrent hypoglycemia. The clinical phenotype varies from an exclusively neurological to a multi-organ manifestation. Methionine-restricted diet should be considered as a therapeutic option.

     

Contemporary Perspectives in Durable Mechanical Circulatory Support: What Did We Learn in the Last 3 Years?

Purpose of Review

In this paper, we will review developments in the field of durable mechanical circulatory support over the past 3 years.

Recent Findings

The role of left ventricular assist device (LVAD) placement in non-inotrope-dependent ambulatory heart failure patients remains controversial in light of recent clinical trials. New devices are on the horizon for destination therapy in advanced heart failure patients. The concept of hemocompatibility and the calculation of hemocompatibility scores represent a novel approach to common adverse events.

Summary

Recent research in mechanical circulatory support has impacted our approach to durable LVAD therapy and set the stage for further advancements in the field.

     

Similar Sustained Virologic Response in Real-World and Clinical Trial Studies of Hepatitis C/Human Immunodeficiency Virus Coinfection

Background

Clinical trials evaluating efficacy of direct-acting antiviral (DAA) therapies demonstrate sustained virologic response (SVR) rates greater than 90% in patients infected with hepatitis C (HCV) and human immunodeficiency virus (HIV). However, generalizability of this data to real-world coinfected populations is unknown.

Aim

We aim to compare efficacy data from clinical trials to effectiveness data of real-world observational studies that evaluate oral interferon-free HCV treatment regimens in patients infected with HIV and HCV.

Methods

We included English-language studies on PubMed and MEDLINE databases from inception until October 2017. Eight clinical trials and 11 observational studies reporting on efficacy data and effectiveness data, respectively, of interferon-free oral DAA regimens in HCV/HIV coinfected patients, were included.

Results

Of patients in the eight clinical trials evaluated, 93.1% (1218/1308) achieved SVR12; of the 11 real-world observational studies, 90.8% (2269/2499) achieved SVR12. Relative risk between those treated in clinical trials versus observational studies was 0.98. Patients with genotype 1 infection, African-American patients, cirrhotic patients, and patients with prior HCV treatment experience had similar rates of SVR in real-world and clinical trial cohorts.

Conclusion

SVR among real-world HCV/HIV coinfected populations treated with DAA regimens is similar to SVR of patients studied in clinical trials. Historically negative predictors of achieving SVR during the era of interferon-based treatments, such as those with cirrhosis, prior HCV treatment failure, GT1 infection, and African-American race, are not associated with a significantly lower SVR in real-world populations treated with various DAA regimens.

     

Keystrokes,Mouse Clicks,and Gazing at the Computer: How Physician Interaction with the EHR Affects Patient Participation

Background

Evidence is mixed regarding how physicians' use of the electronic health record (EHR) affects communication in medical encounters.

Objective

To investigate whether the different ways physicians interact with the computer (mouse clicks, key strokes, and gaze) vary in their effects on patient participation in the consultation, physicians’ efforts to facilitate patient involvement, and silence.

Design

Cross-sectional, observational study of video and event recordings of primary care and specialty consultations.

Participants

Thirty-two physicians and 217 patients.

Main Measures

Predictor variables included measures of physician interaction with the EHR (mouse clicks, key strokes, gaze). Outcome measures included active patient participation (asking questions, stating preferences, expressing concerns), physician facilitation of patient involvement (partnership-building and supportive talk), and silence.

Key Results

Patients were less active participants in consultations in which physicians engaged in more keyboard activity (b?=??0.002, SE?=?0.001, p?=?0.02). More physician gaze at the computer was associated with more silence in the encounter (b?=?0.21, SE?=?0.09, p?=?0.02). Physicians’ facilitative communication, which predicted more active patient participation (b?=?0.65, SE?=?0.14, p?<?0.001), was not related to EHR activity measures.

Conclusions

Patients may be more reluctant to actively participate in medical encounters when physicians are more physically engaged with the computer (e.g., keyboard activity) than when their behavior is less demonstrative (e.g., gazing at EHR). Using easy to deploy communication tactics (e.g., asking about a patient’s thoughts and concerns, social conversation) while working on the computer can help physicians engage patients as well as maintain conversational flow.

     

Sex-Based Differences in Chronic Total Occlusion Management

?

Chronic total occlusions (CTOs) are an important and increasingly recognized subgroup of coronary lesions, documented in at least 30%, but up to 52% of patients with coronary artery disease (CAD) undergoing coronary angiography. Percutaneous coronary intervention (PCI) of these lesions is increasingly pursued, with excellent success rates.

Purpose of Review

It is known that gender differences exist in the presentation of CAD, as well as in clinical outcomes after routine PCI; however, it is not well described how these differences pertain to management of CTOs. This review summarizes the available data regarding sex-based differences in CTO management and outcomes.

Recent Findings

Women comprise approximately 20% of CTO registry and trial participants.

Summary

As has been demonstrated in PCI studies, women comprise a minority of patients in CTO PCI registries and trials. Sex-based differences exist in complication rates, collateral formation, and outcomes and need further evaluation in future studies.

     

Metachronous colorectal cancer

Aim

In this study, we present our patients with metachronous colorectal cancer.

Patients and methods

In the period between 1990 and 2009, 670 patients with colorectal cancer were treated.

Results

Metachronous cancer was developed in 4 (0.6%) patients. The time interval between index and metachronous cancer was 28 months to 22 years (mean 146 months).

Conclusion

Metachronous colorectal cancer is a potential risk that proves the necessity of postoperative colonoscopic control of all patients with colorectal cancer.

     

Prediction Model for Two-Year Risk of Opioid Overdose Among Patients Prescribed Chronic Opioid Therapy

Background

Naloxone is a life-saving opioid antagonist. Chronic pain guidelines recommend that physicians co-prescribe naloxone to patients at high risk for opioid overdose. However, clinical tools to efficiently identify patients who could benefit from naloxone are lacking.

Objective

To develop and validate an overdose predictive model which could be used in primary care settings to assess the need for naloxone.

Design

Retrospective cohort.

Setting

Derivation site was an integrated health system in Colorado; validation site was a safety-net health system in Colorado.

Participants

We developed a predictive model in a cohort of 42,828 patients taking chronic opioid therapy and externally validated the model in 10,708 patients.

Main Measures

Potential predictors and outcomes (nonfatal pharmaceutical and heroin overdoses) were extracted from electronic health records. Fatal overdose outcomes were identified from state vital records. To match the approximate shelf-life of naloxone, we used Cox proportional hazards regression to model the 2-year risk of overdose. Calibration and discrimination were assessed.

Key Results

A five-variable predictive model showed good calibration and discrimination (bootstrap-corrected c-statistic?=?0.73, 95% confidence interval [CI] 0.69–0.78) in the derivation site, with sensitivity of 66.1% and specificity of 66.6%. In the validation site, the model showed good discrimination (c-statistic?=?0.75, 95% CI 0.70–0.80) and less than ideal calibration, with sensitivity and specificity of 82.2% and 49.5%, respectively.

Conclusions

Among patients on chronic opioid therapy, the predictive model identified 66–82% of all subsequent opioid overdoses. This model is an efficient screening tool to identify patients who could benefit from naloxone to prevent overdose deaths. Population differences across the two sites limited calibration in the validation site.

     

Diagnostik bei Verdacht auf Systemerkrankungen

Background

Systemic diseases often affect the kidneys and are important differential diagnoses in nephrology. Connective tissue diseases and vasculitis are of major relevance as the degree of kidney involvement determines the clinical course of the disease and thereby limits the prognosis.

Aim

This review discusses relevant diagnostic steps in patients with systemic diseases.

Conclusion

The diagnostic procedure in patients with systemic autoimmune diseases is complex. The medical history should be taken, physical examination and specific laboratory tests should be tailored to the suspected diagnosis. Making an accurate and timely diagnosis has therapeutic implications and differential diagnoses should be carefully considered.

     

Placebo Rates in Randomized Controlled Trials of Pouchitis Therapy

Background

Approximately half of the patients with ulcerative colitis (UC) who undergo restorative proctocolectomy develop pouchitis within 10 years of surgery. Currently, there are no approved pouchitis treatments. It is important to quantify, and ultimately minimize, placebo rates to design and conduct efficient pouchitis trials.

Aims

To quantify the placebo rate observed in pouchitis randomized controlled trials (RCTs) in meta-analysis.

Methods

Embase, MEDLINE, and the Cochrane Library were searched from inception to November 3, 2017, for placebo-controlled RCTs enrolling adult UC patients with, or at risk for developing, pouchitis. A fixed-effect binomial-normal model was used to pool placebo rates on the log-odds (logit) scale. Proportions and 95% confidence intervals were reported. Outcomes of interest included development of pouchitis, induction of remission/response, and maintenance of remission/response. The Cochrane risk of bias tool was used to evaluate study quality.

Results

Twelve trials (five prevention, five induction, and two maintenance) enrolling a total of 229 placebo patients were eligible for inclusion. The pooled placebo rates for development of pouchitis and induction of response were 47% (95% CI 39–56%) and 24% (95% CI 14–37%), respectively. An insufficient number of trials prevented additional data pooling and meta-regression analysis and no consistent definitions of outcome were identified.

Conclusions

No consistent methods for measuring pouchitis disease activity or defining response and remission were identified, highlighting the need for standardized definitions of outcomes for use in pouchitis trials. Additional high-quality trials are required to evaluate existing and novel therapies in this area.

     

Evolving Landscape of Clinical Trials in Heart Failure: Patient Populations,Endpoint Selection,and Regions of Enrollment

Purpose of Review

Clinical trial design and execution are evolving as increasingly important considerations with respect to the success of heart failure trials. The current review highlights temporal trends in characteristics of heart failure clinical trials.

Recent Findings

Recent trials in heart failure have required longer recruitment phases, displayed inefficient enrollment rates, increased use of composite and nonfatal endpoints, undergone rapid globalization, and gradually increased focus on heart failure with preserved ejection fraction.

Summary

Understanding patterns and trends in clinical trial design and execution may inform future planning and conduct of trials of heart failure therapeutics.