Nemaline Myopathy in a Hypotonic Neonate: Diagnostic Approach for Early Detection and Management.

Neonatal hypotonia presents with low muscle tone and an array of symptoms that vary depending on the etiology. The differential diagnosis for this condition is complex. It is crucial to exclude life-threatening causes before following a diagnostic algorithm and performing additional tests. Given the wide range of clinical symptoms and etiologies for neonatal hypotonia, rapid genetic testing has the potential to expedite diagnosis, reduce invasive testing such as muscle biopsy, reduce hospital stays, and guide condition management.  A four-week-old girl was admitted to the emergency department (ED) with a one-day history of lethargy, poor feeding, congestion, cough, and hypoxemia. Given positive rhino-enterovirus testing and high inflammatory markers, antibiotics were administered. Imaging, venous blood gas, and blood cultures were negative, and the patient was admitted to the pediatric intensive care unit (PICU) for hypoxemia. After speech-language pathology (SLP) and occupational therapy (OT) evaluation, weak orofacial muscles and feeding issues resulted in a nasogastric tube placement. A swallow study revealed decreased pharyngeal contraction and post-swallow liquid residue. Laryngoscopy showed mild laryngomalacia and dysphagia with aspiration. Genetic testing identified an ACTA1 mutation and confirmed nemaline myopathy (NM). The patient's oxygen levels dropped further during sleep, resulting in diagnoses of severe obstructive and moderate-severe central sleep apnea. Treatment included oxygen therapy, SLP, physical therapy, albuterol, and cough assists. After discharge, the patient was frequently re-admitted with chronic respiratory failure and bronchiolitis and later had gastrostomy and tracheostomy tubes inserted.  This specific case highlights the importance of implementing a diagnostic algorithm for neonatal hypotonia. It is also important for physicians, especially emergency medicine (EM) providers, to first exclude infection, sepsis, and cardiac and respiratory organ failure before looking into other tests. Then, physicians should evaluate for more rare etiologies. In this patient's case, the hypotonia was due to a rare genetic disease, nemaline myopathy, and a multidisciplinary approach was used for this patient's care.

Bottle mix-up: Ethanol intoxication in an infant.

Alcohol intoxication is a common ingestion in pediatrics with close to 10,000 reports to poison control centers annually. Hypoglycemia, neurological depression (ataxia, coma, nystagmus, etc.) and unstable vitals (hypothermia, hypotension, bradycardia, and respiratory depression) are common presentations. The patient is a 3 month old female who was brought into the Emergency Department (ED) for one day of decreased oral intake and inconsolability. Vital signs were reassuring. Physical exam revealed gaze preference to the right with inability to look left, dysconjugate gaze, and hypotonia. Work-up including CT of the head, and urinalysis was unremarkable. Urine drug screen was found to be positive for ethanol with follow up serum ethanol at 162 mg/dL. With conservative management the patient returned to her baseline. On follow-up with her pediatrician, it was elicited that the mother inadvertently used a water bottle of vodka to mix the patient's formula. This case adds to the paucity of literature of abnormal presentations of alcohol intoxication in an infant.

Endovascular Embolization of a Perforated Deep Femoral Artery in a 15-Year-Old Boy.

Trauma patients who are hemodynamically unstable or have certain signs of vascular injury should have immediate surgical exploration. For less severe signs of vascular injury, current literature states that endovascular intervention is optimal. This case presents the opportunity to review how signs of vascular injury were considered along with other diagnostic tools to inform decision-making after a penetrating stab wound injury to an extremity.  A 15-year-old male presented to the emergency department (ED) as a trauma activation after being stabbed in the left thigh. The patient had an approximately 5 cm long laceration over the lateral superior aspect of his left thigh with visible subcutaneous tissue and biceps femoris muscle upon probing. He had an initial blood pressure of 101/61 mm Hg. Shortly after the tourniquet was removed, the patient developed brisk bleeding from the wound and his blood pressure decreased to 88/55 mm Hg. He was taken expediently to computed tomography (CT) for an angiogram of the lower extremity which showed active bleeding from a posterior peripheral branch arising from the deep femoral artery in the posterolateral thigh. Interventional radiology performed intravascular embolization, and hemostasis was achieved. The patient was admitted for observation and then discharged 17 hours after admission without ambulatory difficulty. We present a case of penetrating extremity trauma (PET) where the patient had a presentation with mixed hard signs and soft signs of vascular injury. The patient responded well to endovascular embolization. Early detection and intervention in PET are critical in minimizing blood loss and ischemia to distal structures. While following professional organization guidelines can help guide care, a collaborative approach by multiple specialty care teams is critical in providing optimal care in PET.

Classic Presentation of Catecholaminergic Polymorphic Ventricular Tachycardia: A Case Report.

Syncope is a common reason for children and adolescents to seek care in the emergency department. Often syncopal episodes are benign and most commonly due to a vasovagal event. Occasionally an underlying cardiac arrhythmia is responsible. We present a case report of a 17-year-old male who collapsed during an emotional event and went into cardiac arrest. Emergency department evaluation including imaging, laboratory studies, and EKG indicated the cause of cardiac arrest was likely a primary cardiac arrhythmia. An initial clinical diagnosis of catecholaminergic polymorphic ventricular tachycardia (CPVT) was made based on symptom onset during an emotional event, family history of sudden cardiac death, patient age, past episodes of chest pain and palpitations, absence of structural heart defect, and lack of EKG changes after the return of spontaneous circulation (ROSC). The diagnosis was later confirmed with genetic testing. The patient was started on a beta-blocker and a subcutaneous implantable cardioverter-defibrillator (S-ICD, Boston Scientific, Marlborough, MA) was placed. Given the rarity of this condition, this diagnosis is often missed, which contributes to increased mortality rates. In children and young adults presenting with syncope without clear etiology in the presence of high-risk features, further evaluation should be performed including referral to cardiology to rule out chronic cardiac arrhythmias.

Ovarian Torsion and Its Remediation in a Three-Year-Old Girl.

Ovarian torsion is a rare, emergent occurrence seen in the premenarchal population. If detected promptly, ovarian torsion can be treated via detorsion. We present a case of a three-year-old girl whose ovary spontaneously torsed and was corrected via ovarian detorsion. The patient presented with sudden-onset abdominal pain and emesis; a transabdominal ultrasound with Doppler was performed, which led to the diagnosis of ovarian torsion. The patient was directly taken into surgery for correction, after which she quickly recovered and was subsequently discharged. The choice of ovarian detorsion to protect fertility in pediatric patients is supported by this case and by the related literature. The key to safeguarding fertility in these patients lies in rapid detection, which remains a challenge in the pediatric population. By raising widespread awareness of the use of Doppler ultrasound as well as symptom presentation, the protection of fertility in cases of pediatric ovarian torsion can be improved.

Accuracy and Implications of Hemorrhagic Stroke Recognition by Emergency Medical Services.

Background Hemorrhagic stroke is a medical emergency that requires rapid identification and treatment. Despite playing a critical role in the emergency response to hemorrhagic stroke patients, a minimal amount is known about the quality of emergency medical services (EMS) care for this condition. The objectives of this study were to quantify EMS hemorrhagic stroke recognition, identify predictors of accurate EMS recognition, and examine associations between EMS recognition, quality of prehospital care, and patient outcomes. Methods: Consecutive EMS-transported hemorrhagic strokes were identified from medical records at 4 primary stroke centers. Data regarding prehospital care were abstracted from EMS records and linked to in-hospital data. Clinical predictors of accurate EMS recognition were examined using logistic regression. EMS performance measure compliance and hospital outcomes were also compared among EMS recognized and unrecognized hemorrhagic strokes. Results: Over 24 months, EMS-transported 188 hemorrhagic stroke patients; 108 (57.4%) were recognized by EMS. Recognized cases had higher rates of stroke scale documentation (84.3% vs. 20.0%, p < 0.001); multivariable logistic regression confirmed a strong independent relationship between stroke scale documentation and recognition (adjusted OR 15.1 [5.6 to 40.7]). Recognized cases also had shorter on-scene times (15.5 vs. 21 min, p < 0.001) and door-to-computed tomography (DTCT) acquisition times (20 vs. 47 min, p < 0.001). Conclusions: Among EMS-transported hemorrhagic stroke cases, stroke screen documentation was strongly associated with EMS stroke recognition, which was in turn associated with higher quality of EMS care and faster computed tomography (CT) scans upon emergency department arrival.

BET 2: Is there an association between iodine, shellfish and contrast agent allergies?

A shortcut review was carried out to establish whether having a shellfish or iodine allergy changed the risk of allergic reaction in patients receiving radiocontrast medium more than other concomitant allergies. One relevant paper (a systematic review) was identified for inclusion using the reported search strategy. The author, date and country of publication; group studied; study type; relevant outcomes; results and study weaknesses of this paper are tabulated. It is concluded that there is no evidence that allergy to shellfish or iodine alters the risk of reaction to intravenous contrast more than any other allergies.

Educating Paramedics on the Finger-to-Nose Test Improves Recognition of Posterior Stroke.

Background and Purpose- Emergency medical services (EMS) stroke recognition facilitates rapid care, however, prehospital stroke screening tools rely on signs that are often absent in posterior circulation strokes. We hypothesized that addition of the finger-to-nose (FTN) test to the Cincinnati Prehospital Stroke Scale would improve EMS posterior stroke recognition. Methods- In this controlled before and after study of consecutive EMS transported posterior ischemic strokes, paramedics in a single EMS agency received in-person training in the use of the FTN test. Paramedics at 2 other local EMS agencies served as controls. We compared the change in posterior stroke recognition, door-to-CT times, and alteplase delivery between the FTN (intervention) and control agencies. Results- Over 21 months, 51 posterior circulation strokes were transported by the FTN agency and 88 in the control agencies. Following training, posterior stroke recognition improved from 46% to 74% (P=0.039) in the FTN agency, whereas there was no change in the control agencies (32% before versus 39% after, P=0.467). Mean door-to-CT time in the FTN agency also improved following training (62-41 minutes, P=0.037) but not in the control agencies (58-61 minutes, P=0.771). There was no difference in alteplase delivery. Conclusions- Paramedics trained in the FTN test were more likely to identify posterior stroke. If future studies confirm these findings, such training may expedite the care of posterior stroke patients transported by EMS.

Brief Educational Intervention Improves Emergency Medical Services Stroke Recognition.

Background and Purpose- Recognition of stroke symptoms and hospital prenotification by emergency medical services (EMS) facilitate rapid stroke treatment; however, one-third of patients with stroke are unrecognized by EMS. To promote stroke recognition and quality measure compliant prehospital stroke care, we deployed a 30-minute online EMS educational module coupled with a performance feedback system in a single Michigan county. Methods- During a 24-month study period, a registry of consecutive EMS-transported suspected or unrecognized stroke cases was utilized to perform an interrupted time series analysis of the impact of the EMS education and feedback intervention. For each agency, we compared EMS stroke recognition and quality measure compliance rates, as well as emergency department performance and hospital outcomes during 12 preintervention months with performance in the remaining study months. Results- A total of 1805 EMS-transported cases met inclusion criteria; 1235 (68.4%) of these had ischemic or hemorrhagic strokes or transient ischemic attacks. There were no trends toward improvement in any outcome before the intervention. After the intervention, the EMS stroke recognition rate increased from 63.8% to 69.5% ( P=0.037). Prenotification increased from 60.9% to 77.3% ( P<0.001). Among patients with ischemic stroke/transient ischemic attack, there was a trend toward higher rates of tPA (tissue-type plasminogen activator) delivery (13.9%-17.7%; P=0.096) and a significant increase in tPA delivery within 45 minutes (5.7%-8.9%; P=0.042) after intervention. However, improvements in EMS recognition were limited to the first 3 months following intervention. Conclusions- A brief educational intervention was associated with improved EMS stroke recognition, hospital prenotification, and faster tPA delivery. Gains were primarily observed immediately following education and were not sustained through provision of performance feedback to paramedics.

Does Non-Targeted Community CPR Training Increase Bystander CPR Frequency?

OBJECTIVE: Only 37% of out-of-hospital cardiac arrests (OHCA) receive bystander Cardiopulmonary resuscitation (CPR) in Kent County, MI. In May 2014, prehospital providers offered one-time, point-of-contact compression-only CPR training to 2,253 passersby at 7 public locations in Grand Rapids, Michigan. To assess the impact of this intervention, we compared bystander CPR frequency and clinical outcomes in regions surrounding training sites before and after the intervention, adjusting for prehospital covariates. We aimed to assess the effect of this broad, non-targeted intervention on bystander CPR frequency, type of CPR utilized, and clinical outcomes. We also tested for differences in geospatial variation of bystander CPR and clinical outcomes clustered around training sites. METHODS: Retrospective, observational, before-after study of adult, EMS-treated OHCA in Kent County from January 1, 2010 to December 31, 2015. We generated a 5-kilometer radius surrounding each training site to estimate any geospatial influence that training sites might have on bystander CPR frequency in nearby OHCA cases. Chi-squared, Fisher's exact, and t-tests assessed differences in subject features. Difference-in-differences analysis with generalized estimating equation (GEE) modeling assessed bystander CPR frequency, adjusting for training site, covariates (age, sex, witnessed, shockable rhythm, public location), and clustering around training sites. Similar modeling tested for changes in bystander CPR type, return of spontaneous circulation (ROSC), survival to hospital discharge, and cerebral performance category (CPC) of 1-2 at hospital discharge. RESULTS: We included 899 cases before and 587 cases post-intervention. Overall, we observed no increase in the frequency of bystander CPR or favorable clinical outcomes. We did observe an increase in compression-only CPR, but this was paradoxically restricted to OHCA cases falling outside radii around training sites. In adjusted modeling, the bystander CPR training intervention was not associated with bystander CPR frequency (ß -0.002; 95% CI -0.16, 0.15), compression-only CPR (ß -0.06; 95% CI -0.15, 0.02), ROSC (ß -0.06; 95% CI -0.21, 0.25), survival (ß -0.02; 95% CI -0.11, 0.06), or favorable neurologic outcome (ß -0.01; 95% CI -0.07, 0.09). CONCLUSIONS: We observed no impact in bystander CPR performance or outcomes from a blanket, non-targeted approach to community CPR education. The effect of targeted CPR education in locales with known low bystander CPR rates should be tested in this region.

Emergency Dispatcher Stroke Recognition: Associations with Downstream Care.

OBJECTIVE: As the first point of contact for patients activating emergency medical services (EMS), emergency dispatchers have the earliest opportunity to recognize stroke. We sought to quantify dispatcher stroke recognition and its relationships with EMS stroke recognition and response speed. METHODS: We assembled a cohort of consecutive EMS-transported patients with a dispatcher suspected stroke or a hospital discharge diagnosis of stroke or transient ischemic attack (TIA). Dispatcher sensitivity and positive predictive value (PPV) for stroke recognition were calculated. Multivariable logistic regression analysis was used to determine predictors of dispatcher recognition and relationships between dispatcher recognition and downstream care. RESULTS: During a 12-month period, 601 patients met inclusion criteria. Dispatchers suspected stroke in 229/324 (sensitivity = 70.7% [65.5 to 75.4%]) confirmed stroke/TIA cases and correctly assigned a suspected stroke label in 229/506 cases (PPV = 45.3% [41.0 to 49.6%]). Dispatchers had higher odds of recognizing ischemic strokes (aOR 3.4 [1.4 to 8.5]) and lower odds of recognizing patients with visual deficits (aOR = 0.4 [0.2 to 0.9]) or vomiting (aOR = 0.3 [0.1 to 0.9]). Dispatcher suspected stroke cases received more on-scene stroke screens (79.0% vs. 54.7%, p < 0.0001) and were more often recognized by EMS as strokes (77.7% vs. 57.9%, p = 0.0005). Dispatcher recognition was independently associated with EMS stroke recognition (aOR = 3.8 [1.9 to 7.7]), but not with transportation times, door-to-CT times, or t-PA delivery. CONCLUSIONS: Emergency dispatcher stroke recognition is associated with higher rates of on-scene stroke scale performance and EMS ischemic stroke recognition but not with reduced transport times, door-to-CT times, or t-PA treatment.

Bystander Cardiopulmonary Resuscitation Is Clustered and Associated With Neighborhood Socioeconomic Characteristics: A Geospatial Analysis of Kent County, Michigan.

OBJECTIVES: Geographic clustering of bystander cardiopulmonary resuscitation (CPR) is associated with demographic and socioeconomic features of the community where out-of-hospital cardiac arrest (OHCA) occurred, although this association remains largely untested in rural areas. With a significant rural component and relative racial homogeneity, Kent County, Michigan, provides a unique setting to externally validate or identify new community features associated with bystander CPR. Using a large, countywide data set, we tested for geographic clustering of bystander CPR and its associations with community socioeconomic features. METHODS: Secondary analysis of adult OHCA subjects (2010-2015) in the Cardiac Arrest Registry to Enhance Survival (CARES) data set for Kent County, Michigan. After linking geocoded OHCA cases to U.S. census data, we used Moran's I-test to assess for spatial autocorrelation of population-weighted cardiac arrest rate by census block group. Getis-Ord Gi statistic assessed for spatial clustering of bystander CPR and mixed-effects hierarchical logistic regression estimated adjusted associations between community features and bystander CPR. RESULTS: Of 1,592 subjects, 1,465 met inclusion criteria. Geospatial analysis revealed significant clustering of OHCA in more populated/urban areas. Conversely, bystander CPR was less likely in these areas (99% confidence) and more likely in suburban and rural areas (99% confidence). Adjusting for clinical, demographic, and socioeconomic covariates, bystander CPR was associated with public location (odds ratio [OR] = 1.19; 95% confidence interval [CI] = 1.03-1.39), initially shockable rhythms (OR = 1.48; 95% CI = 1.12-1.96), and those in urban neighborhoods (OR = 0.54; 95% CI = 0.38-0.77). CONCLUSIONS: Out-of-hospital cardiac arrest and bystander CPR are geographically clustered in Kent County, Michigan, but bystander CPR is inversely associated with urban designation. These results offer new insight into bystander CPR patterns in mixed urban and rural regions and afford the opportunity for targeted community CPR education in areas of low bystander CPR prevalence.

Emergency Medical Dispatchers Can Obtain Accurate Pediatric Weights from 9-1-1 Callers.

BACKGROUND: Prehospital pediatric drug dosing errors affect 56,000 U.S. children annually. An accurate weight is the first step in accurate dosing. To date, the accuracy of Emergency Medical Dispatcher (EMD) obtained weights has not been evaluated. We hypothesized that EMD could obtain accurate pediatric weights. METHODS: We used a convenience sample of patients 12 years and younger that were transported by EMS to one children's hospital. EMD obtained patient weight (DW) from the 9-1-1 caller. Paramedics reported their estimate of the patient's weight on arrival to the hospital (PW). The DW and PW were compared to the hospital scale weight (HW) for accuracy. RESULTS: A total of 197 patients were included. Parent/guardians were the most frequent 9-1-1 callers (74%). The most frequent method utilized by paramedics to obtain patient weight was to ask a family member. For 0-2 year olds, the mean differences between HW and DW/PW were 0.239kg (SD 3.117)/ -0.374 (SD 2.528). For 3-7 year olds, the mean differences between HW and DW/PW were 0.041kg (SD 4.684)/1.007 (SD 2.466). For 8-11 year olds the mean difference between HW and DW/PW was 2.768 kg (SD 10.926)/ 1.919 (SD 6.909). CONCLUSION: EMD were able to obtain pediatric patient weights with relative accuracy for patients 0-7 year old. Using this EMD-obtained weight to carry out a drug dose calculation would be unlikely to result in a clinically significant dose error in the vast majority of cases. Communicating an EMD-obtained weight to EMS crews en route to a pediatric patient offers additional preparation time for drug calculations, which could improve accuracy.