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Although reverse total shoulder arthroplasty (RTSA) has been shown to be effective for the treatment of cuff tear arthropathy (CTA), the patient's inability to accurately recall their preoperative shoulder condition could skew their perception of the effectiveness of the procedure. Identifying patients who are susceptible to notable recall bias before surgery can help surgeons counsel patients regarding expectations after surgery. The purpose of this study was to evaluate whether patients who undergo RTSA are susceptible to recall bias and, if so, which factors are associated with poor recollection.Patients who underwent RTSA for CTA by the senior author between September 2016 and September 2018 were identified. All patients completed the American Shoulder and Elbow Surgeons (ASES scores) Standardized Assessment Form at the time of preoperative assessment. Patients were contacted at a minimum of 24 months after surgery to retrospectively assess their preoperative condition.A total of 72 patients with a mean age of 72.2 ± 7.65 years completed a retrospective shoulder assessment at 28.3 ± 7.3 months postoperatively. Patient assessment of shoulder condition showed poor reliability (intraclass correlation coefficient = 0.453, confidence interval, 0.237-0.623). Greater preoperative shoulder ASES scores were associated with a greater difference between preoperative ASES scores and recall ASES scores (β = 0.275, P0.001).Patients who undergo RTSA for CTA are susceptible to clinically significant recall bias. Patients with better preoperative condition recall worse preoperative shoulder conditions compared with patients with worse preoperative conditions and are susceptible to a higher degree of recall bias. This patient population should be identified preoperatively and have notable counseling before and after surgery to help them better understand their disease burden and what to expect after surgical intervention.III, diagnostic cohort study.

Introduction Men who have sex with men continue to account for the majority of new HIV infections in the United States. Many of those with new infections are unaware that they have HIV. Preventative measures continue to be essential in reducing new infections, with pre‐exposure prophylaxis (PrEP) being widely recommended. Objectives The overall aim of this qualitative study is to explore the impact of stigma, patient–provider dynamics and patient perception of PrEP on men's engagement with PrEP in a primary care setting. Methods The Consensual Qualitative Research Methodology (Hill, 2012) was used to explore the experiences of 14 men receiving care for PrEP at a Family Medicine clinic in the Midwest. Semistructured interviews were conducted to allow for depth of understanding of individuals' experience. Results Four major domains were identified: motivation to pursue PrEP, barriers and adherence to care, beliefs about how PrEP is perceived by others and experiences discussing sexual health and PrEP with providers. Conclusion It is important to better understand factors contributing to the pursuit of and adherence to HIV prevention measures and HIV care. Further, health systems and providers are encouraged to consider opportunities in terms of how their practice can destigmatize PrEP use and offer a welcoming environment for those pursuing HIV prevention. Patient or Public Contribution Patients were involved in the study through their participation in semistructured interviews, which provided the data analysed for this study. There was no additional participation beyond the one‐time interview or follow‐up poststudy. Their interviews helped contribute to our better understanding of the needs and experiences of those receiving PrEP‐related care.

The degree of symptomatic disease and functional burden has been demonstrated to influence patient results and satisfaction in total hip and knee arthroplasty. Although the relationship between preoperative diagnosis and patient outcomes has been an area of study for reverse total shoulder arthroplasty (RTSA), the influence of the progression of cuff tear arthropathy (CTA) has not yet been examined. The purpose of this study was to evaluate whether preoperative radiographic disease burden and scapular geometry impact patient outcomes and satisfaction in a cohort of patients with CTA treated with RTSA.Eighty-six patients were treated for CTA with RTSA performed by the senior author (B.G.) between September 2016 and September 2018 and were enrolled in an institutional registry. At the time of initial evaluation, the baseline American Shoulder and Elbow Surgeons (ASES) score, patient demographic characteristics, history of shoulder surgery, and presence of pseudoparalysis were collected. Radiographs were obtained to evaluate the critical shoulder angle, acromial index, and progression of CTA as assessed by Hamada grading and the Seebauer classification. Patients were contacted to reassess the ASES score and their satisfaction with the improvement in their shoulder function.A total of 79 patients (91.6%) were available for evaluation at a minimum of 24 months of follow-up. Multivariate logistic regression modeling revealed that scapular geometry measurements (critical shoulder angle and acromial index) and the degree of CTA (Seebauer and Hamada classifications) were not associated with worse outcomes as assessed by the ASES score. However, degenerative changes as assessed by the Hamada grade (odds ratio, 0.13 [95% confidence interval, 0.02-0.86]; P = .03) and preoperative ASES score (odds ratio, 1.04 [95% confidence interval, 1.01-1.07]; P = .008) were independently associated with higher satisfaction at 24 months of follow-up.The results indicate that patients with greater CTA disease progression did not show differing outcomes after RTSA compared with patients with milder disease. In contrast, both poorer preoperative function and degenerative changes as assessed by the Hamada classification were associated with greater satisfaction after RTSA for CTA. Given the broad spectrum of disease in CTA, there is likely a corresponding range in patient expectations that requires further study to maximize patient satisfaction.

Background Psychological distress is common following a traumatic injury event. The Injured Trauma Survivor Screen (ITSS) was developed at a level 1 trauma center to assess for posttraumatic stress disorder (PTSD) and major depressive episode (MDE) following admission for a traumatic injury. The ITSS sensitivity and specificity were analyzed 1 to 3 and 6 to 9 months postinjury to test the validity across trauma centers. Method Four level 1 trauma centers from the East, Midwest, South, and West in the United States recruited 375 eligible adult inpatients (excluded participants included those with moderate or severe traumatic brain injury, whose injury was self-inflicted, were noncommunicative, or were non-English speaking). Baseline sample (White/Caucasian, 63.2%; male, 62.4%; mean (SD) age, 45 (17.11) years; injured by motor vehicle collision, 42.4%) measurements were conducted during index hospitalization. At first follow-up, 69.6% (n = 261) were retained; at second follow-up, 61.3% (n = 230) were retained. Measurements included the ITSS, PTSD Checklist for DSM-5, Center for Epidemiologic Studies Depression Scale-Revised, and Clinician-Administered PTSD Scaled for DSM 5. Results At follow-up 1, the ITSS PTSD subscale had a sensitivity of 75% and specificity of 78.8%, and the MDE subscale had a sensitivity of 80.4% and specificity of 65.6%. At follow-up 2, the PTSD subscale had a sensitivity of 72.7% and specificity of 83.1%, and the MDE subscale had a sensitivity of 76.1% and specificity of 68.3%. A combined risk group using two symptom based measures administered at baseline produced increased specificity. Conclusion The nine-item ITSS continues to be an efficient and effective risk screen for PTSD and MDE following traumatic injury requiring hospitalization. This multi-institutional validation study creates a solid foundation for further exploration of the generalizability of this screen's psychometric properties in distinct populations. Level of evidence Prognostic study, level III.

ImportanceFirearm violence is a public health crisis placing significant burden on individuals, communities, and health care systems. After firearm injury, there is increased risk of poor health, disability, and psychopathology. The newest 2022 guidelines from the American College of Surgeons Committee on Trauma require that all trauma centers screen for risk of psychopathology and provide referral to intervention. Yet, implementing these guidelines in ways that are responsive to the unique needs of communities and specific patient populations, such as after firearm violence, is challenging.ObservationsThe current review highlights important considerations and presents a model for trauma centers to provide comprehensive care to survivors of firearm injury. This model highlights the need to enhance standard practice to provide patient-centered, trauma-informed care, as well as integrate inpatient and outpatient psychological services to address psychosocial needs. Further, incorporation of violence prevention programming better addresses firearm injury as a public health concern.Conclusions and RelevanceUsing research to guide a framework for trauma centers in comprehensive care after firearm violence, we can prevent complications to physical and psychological recovery for this population. Health systems must acknowledge the socioecological context of firearm violence and provide more comprehensive care in the hospital and after discharge, to improve long-term recovery and serve as a means of tertiary prevention of firearm violence.

Surgery providers are integral to the treatment of patients with self-inflicted injuries. Patient disposition (eg, home, inpatient psychiatric treatment, rehabilitation) is important to long-term outcomes, but little is known about factors influencing disposition after discharge following traumatic self-inflicted injury. We tested whether patient or injury characteristics were associated with disposition after treatment for self-inflicted injury.National Trauma Data Bank query for self-inflicted injuries from 2010 to 2018.There were 77,731 patients treated for self-inflicted injuries during the study period. Discharge home was the most common disposition (45%), and those without insurance were less likely to discharge to inpatient psychiatric treatment than those with insurance. Racial minority patients were less likely to discharge to inpatient psychiatric treatment (18.9%) than nonminority patients (23.8%, P.001). Additionally, patients discharged to inpatient psychiatric treatment had significantly lower injury severity score (7.24 ± 7.5) than those who did not (8.69 ± 9.1, P.001).Racial/ethnic minority patients and those without insurance were significantly less likely to discharge to an inpatient psychiatric facility after treatment at a trauma center for self-inflicted injury. Future research is needed to evaluate the internal factors (eg, trauma center practices) and external factors (eg, inpatient psychiatric facilities not accepting patients with wound care needs) driving disposition variability.

Background Avoidance of trauma-related stimuli and unhelpful changes in thought patterns are integral to the development and maintenance of posttraumatic stress disorder (PTSD). Psychological inflexibility is a process that includes cognitive fusion (entanglement with unhelpful thoughts and taking one's thoughts as representing reality) and experiential avoidance (avoiding unpleasant thoughts, feelings, and memories). Prior research suggests that individual differences in psychological inflexibility may explain why some trauma-exposed youth develop PTSD. Additionally, decreased inflexibility may contribute to PTSD symptom improvement over the course of treatment, but no prior study has assessed this. We sought to shed light on this by evaluating, among adolescent psychiatric inpatients with PTSD, whether decreases in psychological inflexibility predicted PTSD symptom improvement from admission to discharge. Method Adolescents in an inpatient psychiatric unit completed a structured diagnostic interview at admission in addition to self-report measures of psychological inflexibility and PTSD symptom severity at admission and discharge. Analyses are conducted with adolescents with PTSD (n = 67). Results Greater reductions in psychological inflexibility during treatment predicted significantly greater decreases in PTSD symptom severity. Conclusion Reductions in psychological inflexibility appear to function as a mechanism of PTSD symptom improvement during treatment. Reducing psychological inflexibility may be a means by which PTSD treatments help trauma-exposed youth recover, and future research is needed to elucidate these findings.

RATIONALE: Ambient air pollution exposure has been linked to mortality from chronic cardiorespiratory diseases, while evidence on respiratory infections remains more limited. OBJECTIVES: We examined the association between long-term exposure to air pollution and pneumonia related mortality in adults in a pool of eight European cohorts. METHODS: Within the multicenter project ’Effects of Low-Level Air Pollution: A Study in Europe’ (ELAPSE), we pooled data from eight cohorts among six European countries. Annual mean residential concentrations in 2010 for fine particulate matter (PM(2.5)), nitrogen dioxide (NO(2)), black carbon (BC), and ozone (O(3)) were estimated using Europe-wide hybrid land use regression models. We applied stratified Cox proportional hazard models to investigate the associations between air pollution and pneumonia, influenza, and acute lower respiratory infections (ALRI) mortality. MEASUREMENTS AND MAIN RESULTS: Of 325,367 participants, 712 died from pneumonia and influenza combined, 682 from pneumonia, and 695 from ALRI during a mean follow-up of 19.5 years. NO(2) and BC were associated with 10-12% increases in pneumonia and influenza combined mortality, but 95% confidence intervals included unity [hazard ratios: 1.12 (0.99-1.26) per 10 µg/m(3) for NO(2); 1.10 (0.97-1.24) per 0.5 10(-5)m(-1) for BC]. Associations with pneumonia and ALRI mortality were almost identical. We detected effect modification suggesting stronger associations with NO(2) or BC in overweight, employed, or currently smoking participants compared to normal weight, unemployed, or non-smoking participants. CONCLUSIONS: Long-term exposure to combustion-related air pollutants NO(2) and BC may be associated with mortality from lower respiratory infections, but larger studies are needed to estimate these associations more precisely.

BackgroundIntimate partner violence (IPV) is a serious public health issue with a substantial burden on society. Screening and intervention practices vary widely and there are no standard guidelines. Our objective was to review research on current practices for IPV prevention in emergency departments and trauma centers in the USA and provide evidenced-based recommendations.MethodsAn evidence-based systematic review of the literature was conducted to address screening and intervention for IPV in adult trauma and emergency department patients. The Grading of Recommendations, Assessment, Development and Evaluations methodology was used to determine the quality of evidence. Studies were included if they addressed our prespecified population, intervention, control, and outcomes questions. Case reports, editorials, and abstracts were excluded from review.ResultsSeven studies met inclusion criteria. All seven were centered around screening for IPV; none addressed interventions when abuse was identified. Screening instruments varied across studies. Although it is unclear if one tool is more accurate than others, significantly more victims were identified when screening protocols were implemented compared with non-standardized approaches to identifying IPV victims.ConclusionOverall, there were very limited data addressing the topic of IPV screening and intervention in emergency medical settings, and the quality of the evidence was low. With likely low risk and a significant potential benefit, we conditionally recommend implementation of a screening protocol to identify victims of IPV in adults treated in the emergency department and trauma centers. Although the purpose of screening would ultimately be to provide resources for victims, no studies that assessed distinct interventions met our inclusion criteria. Therefore, we cannot make specific recommendations related to IPV interventions.PROSPERO registration numberCRD42020219517.

PURPOSE There is a need to address suicide among farmers in the United States and understand what contributes to suicide among American farmers. The purpose of this qualitative study is to analyze narrative data to uncover circumstances that were present in the lives of farmers who died by suicide. METHODS This study leverages data available in the National Violent Death Reporting System. For this study, we examined all suicides that occurred in Wisconsin between the years 2012 and 2016 and were extracted from the Wisconsin Violent Death Reporting System. Decedents were manually sorted by the "usual occupation" variable in order to identify farmers. FINDINGS During the study period, 73 farmers died, most of whom were White, non-Hispanic males. Four themes were identified in the thematic analysis: stymied by physical health issues, grief from loss of relationships, ready access to firearms in rural Wisconsin, and the burden of farming and the farm. CONCLUSIONS The findings from this study demonstrate how unique contextual factors lead to suicide among farmers. This study has implications for suicide prevention among farmers. Primarily, there is a need to address the accessibility of firearms, as their availability in a time of crisis has lethal consequences. However, suicide prevention programming that addresses access to lethal means must take the sociocultural context of rural America into consideration. These findings should also be considered as applied to health care providers that serve rural communities. Suicide risk assessment and management should consider stressors unique to farmers.

Previous research has identified experiential avoidance (EA) as related to a host of adolescent internalizing and externalizing problems, as well as borderline personality disorder, suggesting that it is a crosscutting factor for adolescent psychopathology. It remains unclear whether EA differs among adolescents with BPD compared to adolescents with other psychiatric disorders and healthy adolescents. The aims of this study were to 1) examine EA in adolescents with BPD compared to non-BPD inpatient adolescents and healthy adolescents, and 2) to evaluate whether EA has a unique relationship to borderline pathology over and above internalizing and externalizing. Self-report measures of BPD features, EA, and psychopathology were completed by 692 adolescents (64.5% female, M

In the United States, Black residents exposed to a traumatic event are at an increased risk of developing posttraumatic stress disorder (PTSD) and experiencing more severe symptoms compared to their non-Hispanic White counterparts. Although previous work has suggested a link between racial discrimination and PTSD symptoms, no studies have assessed this association in a sample of traumatic injury survivors. The current study investigated whether (a) past racial discrimination was associated with acute posttraumatic stress symptoms (PTSS) and (b) discrimination prospectively contributed to the prediction of future PTSD symptoms. African American and/or Black patients (N = 113) were recruited from an emergency department in southeastern Wisconsin. Patients in the acute postinjury phase (i.e., 2 weeks posttrauma) completed self-report measures, with PTSD symptoms assessed using the Clinician-Administered PTSD Scale at 6-month follow-up. Bivariate associations indicated past racial discrimination was significantly related to acute PTSS. A multiple regression analysis revealed that pretrauma exposure to racial discrimination significantly predicted PTSD symptoms at follow-up, even after controlling for age, gender, previous psychiatric diagnosis, social support, and lifetime trauma history. Our results suggest that experiences of racial discrimination add significant additional risk for PTSD symptom development following traumatic injury, R(2) = .16, F(6, 106) = 3.25, p = .006. Broadly, these findings add to the body of empirical evidence and personal testimonies of Black individuals in White-centric societies asserting that racial discrimination affects mental health and overall well-being and further highlight the recent call for racism to be classified as a public health crisis.

Purpose: To evaluate the impact of either Medicaid or private insurance on securing an appointment in an outpatient orthopaedic clinic and to determine waiting periods until an appointment as well as the relationship between population metrics and access to care. Methods: A total of 88 clinics were called. There were 2 fictitious patients, one with an anterior cruciate ligament (ACL) injury and the other with a medial meniscus injury, with each calling as having Medicaid or private insurance. Clinic responses were recorded for whether an appointment could be made, when it was scheduled, and with what provider. Results: A total of 32 of 88 (36.4%) of the clinics scheduled an appointment for the Medicaid patient reporting an ACL injury versus 71 of 88 (80.6%) of the clinics that scheduled an appointment for the same patient with private insurance. A total of 34 of 88 (38.6%) of the clinics scheduled an appointment for the Medicaid patient reporting a medial meniscus injury versus 71 of 88 (80.6%) of the clinics that scheduled an appointment for the same patient with private insurance. Mean waiting period for ACL patients with Medicaid was 8.6 days and 4.5 days for patients with private insurance, whereas medial meniscus patients with Medicaid was 7.7 days and 5.4 days for patients with private insurance. In total, 60 of the 66 (90.9%) patients with Medicaid who received an appointment were scheduled to see the orthopaedic surgeon (30 in both ACL and medial meniscus groups). In total, 126 (71.6%) patients with Medicaid and 34 (19.3%) patients with private insurance of the 176 encounters faced barriers to scheduling an appointment. Rural communities were associated with appointment acceptance for patients with Medicaid (P < .05), and patients with private insurance had successful appointment scheduling in all community types (P < .05). Conclusions: This study suggests that patients with Medicaid are less likely to receive orthopaedic care for multiple sports medicine injuries, are more likely to encounter barriers, and endure longer waiting periods. There are different patterns of insurance acceptance according to population metrics. Clinical Relevance: Serves as a baseline evaluation of the difference in access to health care that may be impacted by increases in Medicaid coverage and/or changes in government policies.

OBJECTIVE To investigate the associations between air pollution and mortality, focusing on associations below current European Union, United States, and World Health Organization standards and guidelines. DESIGN Pooled analysis of eight cohorts. SETTING Multicentre project Effects of Low-Level Air Pollution: A Study in Europe (ELAPSE) in six European countries. PARTICIPANTS 325 367 adults from the general population recruited mostly in the 1990s or 2000s with detailed lifestyle data. Stratified Cox proportional hazard models were used to analyse the associations between air pollution and mortality. Western Europe-wide land use regression models were used to characterise residential air pollution concentrations of ambient fine particulate matter (PM2.5), nitrogen dioxide, ozone, and black carbon. MAIN OUTCOME MEASURES Deaths due to natural causes and cause specific mortality. RESULTS Of 325 367 adults followed-up for an average of 19.5 years, 47 131 deaths were observed. Higher exposure to PM2.5, nitrogen dioxide, and black carbon was associated with significantly increased risk of almost all outcomes. An increase of 5 mu g/m(3) in PM2.5 was associated with 13% (95% confidence interval 10.6% to 15.5%) increase in natural deaths; the corresponding figure for a 10 mu g/m(3) increase in nitrogen dioxide was 8.6% (7% to 10.2%). Associations with PM2.5, nitrogen dioxide, and black carbon remained significant at low concentrations. For participants with exposures below the US standard of 12 mu g/m(3) an increase of 5 mu g/m(3) in PM2.5 was associated with 29.6% (14% to 47.4%) increase in natural deaths. CONCLUSIONS Our study contributes to the evidence that outdoor air pollution is associated with mortality even at low pollution levels below the current European and North American standards and WHO guideline values. These findings are therefore an important contribution to the debate about revision of air quality limits, guidelines, and standards, and future assessments by the Global Burden of Disease.

Drugging (i.e., administering someone a drug or alcohol without their knowledge or consent) is a problem with substantial consequences for college students. Although sexual minorities face greater rates of drugging risk factors (e.g., greater rates of binge drinking have been identified among sexual minorities), no prior study has examined the prevalence or risk of drugging in this population. We sought to (1) describe rates at which heterosexual and sexual minority college students (separated by gender) have been drugged and to assess (2) sexual minority status and (3) illicit drug use as risk factors for drugging victimization for male and female college students. Results revealed that, controlling for established drugging victimization risk factors, male sexual minority college students were 72.9% more likely to report drugging victimization than heterosexual males. No significant differences in rates of drugging victimization were found between sexual minority and heterosexual women. However, both genders had greater drugging victimization among students who engaged in illicit drug use, binge drinking, and Greek life membership. These findings suggest that initiatives to promote the well-being of college students (e.g., sexual assault prevention) should include drugging as a focus, with outreach particularly to women and sexual minority men.

This study examined the interactive effects of injunctive norm exposure and hostile and benevolent sexist attitudes on men's sexually aggressive responses during a behavioral analogue paradigm in which they interacted online with a bogus female partner. Heterosexual adult men (n = 201), recruited from an online sample, read fictional information regarding other men's approval of misogynistic, paternalistic, or egalitarian treatment of women, or non-gender-relevant control information. Through a media preference survey, men then learned that their female partner disliked sexual content in films, after which they had an opportunity to send her up to 120 sec' worth of either a sexually explicit or nonsexual film clip. Validating the online sexual aggression paradigm, men with a 1-year history of sexual assault exhibited more sexually aggressive responding during the film selection paradigm. Moreover, exposure to injunctive norm information produced a boomerang effect, such that men high in hostile sexist attitudes showed an increase in sexual aggression when confronted with paternalism and gender equality norms. Conversely, exposure to paternalism and gender equality norms suppressed the otherwise protective function of high benevolent sexism in reducing men's sexually aggressive tendencies. The implications of these results for social norms interventions are discussed. Aggr. Behav. 9999:1-14, 2015. © 2015 Wiley Periodicals, Inc. Language: en

Background: Patients with rotator cuff disease commonly complain of difficulty sleeping. Arthroscopic repair has been associated with improved sleep quality in many patients with rotator cuff tears; however, some individuals continue to suffer from sleep disturbance postoperatively. Purpose: To determine whether changes in sleep quality following rotator cuff repair are predicted by a patient’s narcotic use or ability to cope with stress (resilience). Study Design: Case series; Level of evidence, 4. Methods: A total of 48 patients undergoing arthroscopic rotator cuff repair were prospectively enrolled and completed the Connor-Davidson Resilience Scale (CD-RISC) preoperatively. The Pittsburgh Sleep Quality Index (PSQI) was administered preoperatively and at multiple intervals postoperatively for 6 months. Narcotic utilization was determined via a legal prescriber database. Pre- and postoperative sleep scores were compared using paired t tests and the McNemar test. Linear regression was used to determine whether narcotic use or CD-RISC score predicted changes in sleep quality. Results: An increased number of patients experienced good sleep at 6 months postoperatively ( P < .01). Mean ± SD nocturnal pain frequency improved from 2.5 ± 1.0 at baseline to 0.9 ± 1.1 at 6 months. CD-RISC score had a positive predictive value on changes in PSQI score ( R 2 = 0.09, P = .028) and nocturnal pain frequency ( R 2 = 0.08, P = .041) at 2 weeks. Narcotic use did not significantly predict changes in PSQI score or nocturnal pain frequency ( P > .05). Conclusion: Most patients with rotator cuff disease will experience improvement in sleep quality following arthroscopic repair. Patients demonstrated notable improvements in nocturnal pain frequency as soon as 6 weeks following surgery. CD-RISC resiliency scores had a significant positive predictive value on changes in sleep quality and nocturnal pain frequency at 2 weeks. Narcotic use was not associated with change in sleep quality.

MINTFIT is a joint project of the HafenCity University Hamburg (HCU), Hamburg University of Applied Sciences HAW), Hamburg University of Technology (TUHH) and Universität Hamburg (UHH) together with the Hamburg Ministry of Science, Research and Equalities (Behörde für Wissenschaft, Forschung und Gleichstellung, BWFG) to support high school students and others interested in STEM studies. The MINTFIT Math Test is a diagnostic online test which gives its participants the opportunity to check if their math skills are sufficient for a successful start into the first terms of a STEM degree programme.

Clinical and pathological features of two siblings of opposite sex with the Pena-Shokeir phenotype are reported. A detailed account of the prenatal and dysmorphological findings is given in one case. A broad range of deformations regarded as secondary to fetal hypokinesia was present, including a number of yet unreported findings. One case showed additional endocrine hyperplasia and left lung trilobation. Both siblings displayed extensive, highly similar CNS-abnormalities. The type and convergence of these malformations differ from previously reported cases and characterize a new familial subtype of the Pena-Shokeir phenotype.