Strategies for fixation of periprosthetic fragility fractures of the neck of femur below a well-functioning hip resurfacing arthroplasty: A case-series.

Introduction: Metal-on-metal Hip Resurfacing (HR) was performed in many young individuals as it conserved bone stock and had low wear rates, before it became less popular due to the detection of Adverse Reactions to Metal Debris. As such, many patients in the community have well-functioning HRs and as they age, the incidence of fragility fractures of the neck of femur around the existing implant is expected to increase. These fractures are amenable to surgical fixation as adequate bone stock remains in the head of the femur and the implants are well fixed. Case-series: We present a series of six cases which were treated by fixation using locked plates (3), dynamic hip screws (2) and cephalo-medullary nail (1). Four cases achieved clinical and radiographic union with good function. One case had a delayed union, though union was finally achieved at 23 months. One case had an early failure necessitating revision to a Total Hip Replacement after 6 weeks. Discussion: We highlight the geometrical principles of placing fixation devices under an HR femoral component. We have also conducted a literature search and present details of all case reports to date. Conclusion: Fragility per-trochanteric fractures under a well-fixed HR with good baseline function are amenable to fixation using a variety of methods including large screw devices that are commonly used in this location. Locked plates including variable angle locking designs should be kept available if needed.

The Early "Unnatural" History Following Surgical Repair of Ventricular Septal Defects.

INTRODUCTION: Surgical outcomes for simple ventricular septal defects (VSD) have been excellent in the past three decades. For this project, the timing of resolution of left-sided dilation and mitral regurgitation (MR) following VSD repair was assessed. METHODS: Echocardiographic data surrounding surgery of 42 consecutive children who underwent surgical patch repair of a VSD were reviewed. The echocardiograms were reviewed up to a mean of 12 months post-operatively (range 9 - 14 months). Quantitative data indexed to body surface area including left atrial (LA) volume, mitral valve annulus diameter, and left ventricular end-diastolic dimension (LVEDD) was analyzed. RESULTS: The majority of our pre-surgical cohort had only trace (44%) or no MR (31%), with a small proportion having mild (16%) or moderate MR (9%). No patients had moderate or greater MR following repair at follow-up. The median mitral valve annular Z-score was 1.8 (SD 1.6; range: -1.2 to 4.1) pre-operatively, improving to a 0.6 (range: -1.7 to 2.4; p < 0.001) at follow-up. LA dilation was present in 70% of patients, with a median LA volume Z-score of 1.1 (range: -2.6 to 15.5), decreasing to 13% median Z-score -1.2 (range: -3.5 to 2.9; p < 0.001) at follow-up. LV dilation was present in 81% of pre-operative patients with a median LVEDD Z-score of 3.0 (range: -2.0 to 7.9). There was significant improvement in qualitative assessment of LV enlargement (25%) with a median LVEDD Z-score of 0.5 (range: -2.1 to 2.9; p < 0.001) at follow-up. Discharge echocardiogram was performed at a mean of 5.7 days (range: 3 - 12 days) following surgery. CONCLUSIONS: Normalization of LA, mitral valve annulus, and LV size occurred within the first three months in the majority of patients, with significant changes occurring within the first post-operative week following surgical repair for VSD.

Preoperative Physiology, Imaging, and Management of Coarctation of Aorta in Children.

Coarctation of the aorta (CoA) is a narrowing of the proximal thoracic aorta typically located at the junction of the aorta with the ductus arteriosus. While it is a simple lesion to understand, considerable variation exists in the anatomy and pathophysiology, leading to varied clinical presentation, management options, and prognosis. On the one hand critical CoA manifests in the neonatal period as a duct-dependent lesion, while less severe forms of obstruction present later in childhood or adulthood as hypertension or incidentally noted precordial murmurs. While transthoracic echocardiography is usually adequate, older children and adults may need more advanced imaging modalities like computed tomography and magnetic resonance imaging prior to intervention. Depending on the type of lesion, management options currently available include surgery and percutaneous balloon angioplasty and stenting. Even after successful interventions, these patients need life-long surveillance for residual aortic obstruction and systemic hypertension with variable long-term clinical outcomes.

Phenotypic diversity and correlation with the genotypes of pseudohypoaldosteronism type 1.

Background Type I pseudohypoaldosteronism (PHA1) is a rare condition characterised by profound salt wasting, hyperkalaemia and metabolic acidosis due to renal tubular resistance to aldosterone (PHA1a) or defective sodium epithelial channels (PHA1b or systemic PHA). Our aim was to review the clinical presentation related to the genotype in patients with PHA1. Methods A questionnaire-based cross-sectional survey was undertaken through the British Society of Paediatric Endocrinology and Diabetes (BSPED) examining the clinical presentation and management of patients with genetically confirmed PHA1. We also reviewed previously reported patients where genotypic and phenotypic information were reported. Results Genetic confirmation was made in 12 patients with PHA1; four had PHA1a, including one novel mutation in NR3C2; eight had PHA1b, including three with novel mutations in SCNN1A and one novel mutation in SCNN1B. It was impossible to differentiate between types of PHA1 from early clinical presentation or the biochemical and hormonal profile. Patients presenting with missense mutations of SCNN1A and SCNN1B had a less marked rise in serum aldosterone suggesting preservation in sodium epithelial channel function. Conclusions We advocate early genetic testing in patients with presumed PHA1, given the challenges in differentiating between patients with PHA1a and PHA1b. Clinical course differs between patients with NR3C2 and SCNN1A mutations with a poorer prognosis in those with multisystem PHA. There were no obvious genotype-phenotype correlations between mutations on the same gene in our cohort and others, although a lower serum aldosterone may suggest a missense mutation in SCNN1 in patients with PHA1b.

Effect of Intravenous Phenobarbital on Left Ventricular Myocardial Contractility Determined by Echocardiography in Children.

INTRODUCTION: Animal studies and rare human studies have suggested a negative effect of barbiturates on cardiac function. Although intravenous (IV) phenobarbital is used routinely in children in the clinical setting, studies in children are lacking. We performed a study to evaluate effect of IV phenobarbital loading on myocardial systolic function of children. METHODS: In a prospective pilot study in children without congenital heart defects, the effect of IV phenobarbital was evaluated on the left ventricular systolic function measured by ejection fraction (EF) by Simpson's method via an echocardiogram. Any child less than 18 years of age who received IV loading dose of at least 20 mg/kg of phenobarbital given as an infusion over 20 - 30 minutes for various medical indications was eligible to take part in the study. Three measurements of EF by an echocardiogram were made: before loading dose, 30 minutes after completion of the loading dose, and prior to the first maintenance dose. Relevant clinical data were recorded, including vital signs, immediately prior to each echocardiogram. Change of function as measured by EF over time was analyzed using linear mixed modeling methods. For this study, significant change in blood pressure was defined as a drop of at least 20 mmHg in systolic blood pressure. RESULTS: Ten children (70% female, age range two days to 8.2 years) were enrolled. Three had hypotension with a drop of systolic blood pressure greater than 20 mmHg from baseline. On examining the trajectory of EF on each individual graphically, the left ventricular EF tended to fall immediately following phenobarbital therapy and return to baseline on re-evaluation. These trajectories were statistically significant for EF. CONCLUSIONS: Phenobarbital had a direct and transient depressant effect on systolic function of the myocardium in one third of the cases. The depression in EF appeared to be transient with return to baseline in less than 24 hours. We recommend close monitoring with anticipation of decreased function in children when using IV phenobarbital.

Anomalous aortic origin of the right coronary artery from the non-coronary sinus of Valsalva.

We report a rare case of anomalous aortic origin of the right coronary artery from the posterior/non-coronary sinus of Valsalva in a 9-year-old male diagnosed during the workup of premature ventricular contractions. The finding was initially noted on transthoracic echocardiogram and further confirmed with computed tomography. The anomalous coronary artery shows a wide ostium with no intramural or interarterial course.

Do medical students studying in the United Kingdom have an adequate factual knowledge of basic life support?

BACKGROUND: Healthcare professionals have a duty to maintain basic life support (BLS) skills. This study aims to evaluate medical students' factual knowledge of BLS and the training they receive. METHODS: A cross-sectional, closed-response questionnaire was distributed to the first- and fourth-year students studying at institutions in the United Kingdom. The paper questionnaire sought to quantify respondent's previous BLS training, factual knowledge of the BLS algorithm using five multiple choice questions (MCQs), and valuate their desire for further BLS training. Students received 1 point for each correctly identified answer to the 5 MCQ's. RESULTS: A total of 3,732 complete responses were received from 21 medical schools. Eighty percent (n=2,999) of students completed a BLS course as part of their undergraduate medical studies. There was a significant difference (P<0.001) in the percentage of the fourth-year students selecting the correct answer in all the MCQ's compared to the first-year students except in identifying the correct depth of compressions required during CPR (P=0.095). Overall 10.3% (95% CI 9.9% to 10.7%) of respondents correctly identified the answer to 5 MCQ's on BLS 9% of the first-year students (n=194) and 12% of the fourth-year students (n=190). On an institutional level the proportion of students answering all MCQ's correctly ranged from 2% to 54% at different universities. Eighty-one percent of students (n=3,031) wished for more BLS training in their curriculum. CONCLUSION: Factual knowledge of BLS is poor among medical students in the UK. There is a disparity in standards of knowledge across institutions and respondents indicating that they would like more training.

Early mortality and morbidity following a type II odontoid fracture in the elderly.

BACKGROUND: We aimed to analyse the rates of early and causes of death in patients aged over 65 years with a type II odontoid fracture. METHODS: A consecutive series of 93 patients with a type II fracture of the odontoid process was retrospectively identified. Data collected included patient demographics, co-morbidities, associated injuries, neurological injury, date of death and cause of death. Mean patient age was 81. Five patients (5%) were treated operatively while the rest were treated in a hard cervical collar. Five patients (5%) had an incomplete cervical cord injury secondary to the fracture. RESULTS: The rate of mortality at 30 days was 10% (9 patients) and at 90 days it was 16% (15 patients). Following multivariate analysis, the factors found to significantly increase the risk of 30-day mortality included increasing age, increasing injury severity score and leukaemia. Following univariate analysis the only factor found to increase the risk of 90-day mortality was advancing age. The commonest causes of death were pneumonia and ischaemic coronary disease. CONCLUSION: Our results suggest that this patient cohort is frail and at risk of early mortality. We suggest that their inpatient care be provided in close conjunction with elderly care physicians.

Innocent Heart Murmur.

Heart murmur is the most common reason for a referral to a pediatric cardiologist. Virtually all children have a heart murmur during their childhood. Less than 1% of murmurs are pathological in children. Innocent/functional heart murmur is the most common type of heart murmur. There are multiple theories proposed to identify etiology of innocent heart murmur with varying consensus, but everybody agrees that innocent heart murmur does not carry any morbidity or mortality risk. Even today, heart murmur is associated with high physician uncertainty and parental anxiety. Extensive cardiac evaluation for such a benign finding is also associated with high health care utilization and cost. This article attempts to review this long-known finding which continues to remain a diagnostic challenge.

Coarctation of Aorta in Children.

Coarctation of aorta (CoA) is a discrete narrowing in aorta causing obstruction to the flow of blood. It accounts for 6-8% of all congenital heart diseases. With advances in fetal echocardiography rate of prenatal diagnosis of coarctation of aorta has improved but it still remains a challenging diagnosis to make prenatally. Transthoracic echocardiography is mainstay of making initial diagnosis and routine follow-up. Cardiac magnetic resonance imaging (MRI) and computed tomography (CT) are great advanced imaging tools for two-dimensional and three-dimensional imaging of aortic arch in complex cases. Based on type of coarctation, size of patient, severity of lesion, and associated abnormalities various management options like surgical treatment, transcatheter balloon angioplasty and transcatheter stent implantation are available. There is significant improvement in long-term survival from pre-surgical era to post-surgical era. But, among the postsurgical era patients, the long-term survival has not significantly changed between older and contemporary cohort. Patients with coarctation of aorta need lifelong follow-up event after successful initial intervention.

Myocardial Ischemia with Cannabinoid Use in an Adolescent.

A 16-year-old male presented to the emergency department with chest pain after smoking a synthetic cannabinoid from a vape pen. He had rising troponin I levels, and his exercise stress echocardiogram showed distal apical and septal hypokinesis that resolved at six-month follow-up. This case report raises concern about cardiac ischemia related to synthetic cannabinoid abuse in the pediatric population in the current era of cannabis legalization.

Development of aortic coarctation following device closure of patent ductus arteriosus.

A patient who had transcatheter closure of a large patent ductus arteriosus in early infancy developed aortic coarctation during follow-up. Initially, balloon angioplasty and subsequent stent implantation successfully relieved the aortic obstruction. Avoidance of use of large devices in small babies and modification of current devices so that they do not cause aortic obstruction may result in better outcomes. Once aortic obstruction develops, transcatheter management is useful in relieving the obstruction.

Increased left main coronary artery dimensions in children with sickle cell disease.

For adults with sickle cell disease (SCD), cardiac changes are well described and may account for up to one-fourth of disease-related mortality. Much less is known about heart disease in pediatric SCD patients. This retrospective study analyzed a contemporary cohort of children with SCD followed at a single tertiary care center. The study aimed to evaluate the effect of SCD on left main coronary artery (LMCA) dimensions in the contemporary pediatric population. The echocardiography database was searched for studies of children with SCD from 2000 to 2009. The LMCA diameter was remeasured offline from digital images. Digital echocardiographic studies of 68 children (35 boys) 11.7 ± 4.6 years of age with SCD were analyzed. All the patients had normal systolic function as measured by fractional shortening. The left ventricular end-diastolic dimension (LVEDD) for 34 % of the pediatric SCD patients showed a dilated LV. In 24 % of the children, a LV mass index (LVMI) greater than 50 g/m(2.7) denoted LV hypertrophy. Application of sex-specific normal values showed that 60 % of the boys and 33 % of the girls had an increased LVMI. The LMCA diameter was enlarged in 43 % of the SCD children, including 26 % with an LMCA z-score higher than 3. This study found a higher incidence of LMCA dilation in a pediatric referral population with SCD. The findings also validated an increased LVMI in the contemporary SCD patient population, which was noted previously. This study adds the need to include assessment of coronary arteries in cardiac evaluation of SCD patients, and special attention should be paid to patients with a high-normal to high coronary z-score.

The characteristics and outcomes of parainfluenza virus infections in 200 patients with leukemia or recipients of hematopoietic stem cell transplantation.

Community respiratory viruses are significant causes of morbidity and mortality in patients with leukemia and hematopoietic stem cell transplant (HSCT) recipients. Data on characteristics and outcomes of parainfluenza virus (PIV) infections in these patients are limited. We reviewed the records of patients with leukemia and HSCT recipients who developed PIV infections to determine the characteristics and outcomes of such infections. We identified 200 patients with PIV infections, including 80 (40%) patients with leukemia and 120 (60%) recipients of HSCT. At presentation, most patients (70%) had an upper respiratory tract infection and the remaining patients (30%) had pneumonia. Neutropenia, APACHE II score more than 15, and respiratory coinfections were independent predictors of progression to pneumonia on multivariate analysis. Overall mortality rate was 9% at 30 days after diagnosis and 17% among patients who had PIV pneumonia, with no significant difference between patients with leukemia and HSCT recipients (16% vs 17%). On multivariate analysis, independent predictors of death were relapsed or refractory underlying malignancy, APACHE II score more than 15, and high-dose steroid use. Patients with leukemia and HSCT are at risk for serious PIV infections, including PIV pneumonia, with a significant mortality rate. We identified multiple risk factors for progression to pneumonia and death.