Exercise-Induced Pulmonary Hemorrhage in a Non-Athletic Child: Implications for Military Recruits.

Hemoptysis is a rare presenting symptom in pediatric and young adult patients with a highly variable outcome ranging from an isolated mild occurrence to severe illness and death. Exercise-induced pulmonary hemorrhage (EIPH) has several reports in adult literature but has not previously been reported in pediatric patients. A 12-year-old female with a history of trisomy X (47, XXX), obesity, depression, anxiety, and obstructive sleep apnea presented to the pediatric pulmonology clinic after several episodes of hemoptysis. Spirometry, imaging, and laboratory evaluation for autoimmune vasculitides and other causes associated with pediatric hemoptysis did not reveal an etiology for the hemoptysis. A combined bronchoscopy with pediatric and adult providers revealed no airway lesions or sources of bleeding. EIPH is a diagnosis of exclusion. This patient was diagnosed with EIPH and had spontaneous resolution with improved fitness. Many military training and service activities are similar to those reported with EIPH. Trainees with various levels of aerobic fitness are at risk of developing EIPH. The hemoptysis evaluation is important for military providers given the range of severity in presentations, even though it is a rare occurrence. In addition to a novel presentation of EIPH, this case demonstrates the value of collaboration between pediatric and adult specialists in the Military Health System (MHS). Military care providers should be aware of this rare phenomenon in service members and trainees who are at risk during maximal aerobic effort.

Increasing Mental Health Screening in Pediatric Subspecialty Clinics: An Improvement Project at Brooke Army Medical Center.

INTRODUCTION: Mental health screening allows for the early identification of patients at risk of mental health disorders such as anxiety and depression. The Defense Health Administration Procedures Manual 6025.01 established that patients older than 12 years of age should receive annual mental health screening assessing suicidality risk with a standardized screening tool. MATERIALS AND METHODS: To improve rates of mental health screening in this at-risk population, an A3 Lean approach to quality improvement was undertaken in the Pediatric Subspecialty Clinics at Brooke Army Medical Center. RESULTS: Baseline data of Patient Health Questionnaire-9 or General Anxiety Disorder-7 screening from pediatric pulmonology and cardiology clinics were 6.3%. Plan-Do-Study-Act quality improvement methods evaluated possible interventions to improve screening. A standard operating procedure (SOP) outlining scoring and interpretation recommendations was distributed in pediatric pulmonology and cardiology clinics. Using the Armed Forces Health Longitudinal Technology Application, a post-implementation chart review was conducted for 8 weeks. With the initiation of Military Health System Genesis, the SOP was adjusted and a root cause analysis was performed. The pediatric gastroenterology clinic cares for patients presenting with functional abdominal pain; therefore, the clinic was added to the second iteration to determine if anxiety or depression may play a role. Providers were educated on documentation and coding. A second post-implementation chart review was conducted for 8 weeks. Over two iterations, mental health screening of new adolescent patients in pediatric subspecialty clinics increased by 794% from baseline. Our providers screened 50% of the adolescent patients. CONCLUSIONS: With increased surveillance of an at-risk population, more adolescents will receive the standard of care. With refinement of the SOP and expansion to other subspecialties, this project has the potential to expand within Brooke Army Medical Center and other clinics in the Defense Health Administration.

Development and assessment of a low literacy, pictographic asthma action plan with clinical automation to enhance guideline-concordant care for children with asthma.

OBJECTIVE: Asthma is characterized by reversible pulmonary symptoms, frequent hospitalizations, poor quality of life, and varied treatment. Parents with low health literacy (HL) is linked to poor asthma outcomes in children. Recent practice updates recommended inhaled corticosteroids for the management of persistent asthma, but guideline-concordant care is suboptimal. Our aim was to develop and assess an Asthma Action Plan (AAP) that could serve as an individualized plan for low HL families and facilitate guideline-concordant care for clinicians. METHODS: We followed the National Institute of Health 5-step "Clear & Simple" approach to develop the Uniformed Services AAP. Our AAP included symptom pictographs (dyspnea, cough, sleep, activity) and guideline-concordant clinical automation tools. Caregivers assessed the pictograms for validity (transparency of ≥ 85%; translucency score ≥ 5; and ≥ 85% recall). Readability was assessed using 7 formulas. (<6th Grade was acceptable). Comprehensibility, design quality, and usefulness was assessed by caregivers using the Consumer Information Rating Form (CIRF) (>80% was acceptable). Understandability and actionability was assessed by medical librarians using the Patient Education Materials Assessment Tool-Printable (>80% was acceptable). Suitability was assessed by clinicians using the modified Suitability Assessment of Materials (SAM) instrument (>70% was superior). RESULTS: All 12 pictograms were validated (N = 118 respondents). Readability demonstrated a 4th grade level. Overall CIRF percentile score = 80.4%. Understandability and Actionability = 100%. Suitability score = 75%. CONCLUSIONS: Our AAP was formally endorsed by the Allergy & Asthma Network. The Uniformed Services AAP is a novel tool with embedded clinical automation that can address low HL and enhance guideline-concordant care.

Pediatric Hemoptysis without Bronchiectasis or Cardiac Disease: Etiology, Recurrence, and Mortality.

OBJECTIVES: To describe the etiologies of hemoptysis in patients without pre-existing bronchiectasis or cardiac disease; to assess odds of recurrent hemoptysis by diagnostic category; and to assess odds of mortality by diagnostic category. STUDY DESIGN: This retrospective case series included all patients with hemoptysis documented during an admission to Boston Children's Hospital from January 1, 2007 to June 1, 2017. Patients with bronchiectasis, congenital heart disease, primary pulmonary hypertension, bleeding above the glottis, hemoptysis before 38 weeks of corrected gestational age, hematemesis, foreign body, and trauma were excluded. Patients were also characterized by coagulation status. Primary outcomes were recurrent hemoptysis and death. Univariate analysis was performed to determine ORs for recurrence and death per diagnostic category with infection as the reference category. RESULTS: In total, 257 patients met study criteria and were analyzed. The most common causes of hemoptysis were infection (n = 122), neoplasm (n = 58), and other diagnoses (n = 49). Of the patients with infection, recurrence was 28% and all-cause mortality was 12%. Neoplasm had lower odds of recurrence (OR 0.3, P = .012) but higher odds of mortality (OR 15.8, P < .001). Thrombocytopenia had lower odds of recurrence (OR 0.2, P = .005) but higher odds of mortality (OR 5.9, P < .001). Patients with a tracheostomy had higher odds of recurrence (OR 6.3, P < .001), but lower odds of death (OR 0.4, P = .042). CONCLUSIONS: This study confirms that infection is the most common cause of hemoptysis in patients without severe underlying pulmonary or cardiac disease. Hemoptysis associated with neoplasm and/or thrombocytopenia confers mortality risk. Tracheostomy confers risk of recurrence. Future prospective research on diagnoses associated with hemoptysis is warranted.

Pulmonary hemorrhage in infancy: A 10-year single-center experience.

AIM: Pulmonary hemorrhage in infancy is rare, with challenges in determining its incidence, causes, and outcomes across diverse groups. Our aim was to better understand the incidence and identified causes. We further analyzed the subgroup of patients meeting criteria for acute idiopathic pulmonary hemorrhage of infancy (AIPHI) to determine recurrence, mortality, and treatment. METHODS: We performed a 10-year retrospective cohort study of infants with pulmonary hemorrhage in a large tertiary care center. One-hundred fifty-seven patients overall were identified. RESULTS: The most common diagnoses in infants with pulmonary hemorrhage were congenital heart disease (36.6%), prematurity/premature lung disease (34.6%), congenital or acquired lung disorders (15.0%), and congenital or acquired coagulopathies (13.7%). Nonaccidental trauma (NAT; n = 3) was also an important cause of pulmonary hemorrhage. All patients diagnosed with NAT had normal retinal examinations and skeletal surveys. Only four patients were identified with AIPHI. There was no mortality in this group of infants. One of four patients with AIPHI had a recurrence. Steroids were the consistent treatment for AIPHI, with a large range of treatment duration. CONCLUSION: Diagnostic studies should focus on identifying non-pulmonary sources of bleeding, infection, underlying lung disease, congenital heart defects, coagulopathies, infection, and NAT, as these were the most frequently identified causes of bleeding. NAT is not adequately identified with ophthalmology exam and skeletal survey. Overall, we found AIPHI to be a rare diagnosis. All of the patients with idiopathic hemorrhage received systemic steroids with varying doses and lengths of treatment.

Survival after lung transplantation of cystic fibrosis patients infected with Burkholderia dolosa (genomovar VI).

Cystic fibrosis (CF) with severe lung disease is a well-recognized indication for lung transplantation. Colonization with various organisms in CF patients may impact post-transplant morbidity and mortality. Burkholderia cepacia complex (BCC) is made up of distinct genomovars with significant morbidity and mortality associated with B. cenocepacia (genomovar III) following lung transplant. The outcomes of patients infected with genomovar B. dolosa (genomovar VI) have yet to be described in the literature. We performed a retrospective chart review of all cystic fibrosis patients colonized with B. dolosa from our center who underwent lung transplantation (n = 11) at various medical centers across the US between 2000 and 2014. Survival rates were 73%, 53%, and 30% for 1, 3, and 5 years, respectively. Median survival was 44 months (95% CI = 11.1-76.8). CF patients with B. dolosa that have undergone lung transplantation have decreased one-year survival when compared to all patients transplanted with cystic fibrosis. Conditional 5-year survival for B. dolosa-infected patients was 43% in patients that survived the first year post-transplant, suggesting that this first year is crucial in managing the infection. Importantly, the survival of the B. dolosa patients was higher than compared to previously reported survival rates of B. cenocepacia patients post-transplant.

Relationship of at Admission Lactate, Unmeasured Anions, and Chloride to the Outcome of Critically Ill Patients.

OBJECTIVES: To investigate the association between the concentration of the causative anions responsible for the main types of metabolic acidosis and the outcome. DESIGN: Prospective observational study. SETTING: Teaching ICU. PATIENTS: All patients admitted from January 2006 to December 2014. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Four thousand nine hundred one patients were admitted throughout the study period; 1,609 met criteria for metabolic acidosis and 145 had normal acid-base values. The association between at admission lactate, unmeasured anions, and chloride concentration with outcome was assessed by multivariate analysis in the whole cohort and in patients with metabolic acidosis. We also compared the mortality of patients with lactic, unmeasured anions, and hyperchloremic metabolic acidosis with that of patients without acid-base disorders. In the whole population, increased lactate and unmeasured anions were independently associated with increased mortality, even after adjusting for potential confounders (odds ratio [95% CI], 1.14 (1.08-1.20); p < 0.0001 and 1.04 (1.02-1.06); p < 0.0001, respectively). In patients with metabolic acidosis, the results were similar. Patients with lactic and unmeasured anions acidosis, but not those with hyperchloremic acidosis, had an increased mortality compared to patients without alterations (17.7%, 12.7%, 4.9%, and 5.8%, respectively; p < 0.05). CONCLUSIONS: In this large cohort of critically ill patients, increased concentrations of lactate and unmeasured anions, but not chloride, were associated with increased mortality. In addition, increased unmeasured anions were the leading cause of metabolic acidosis.

Similar Microcirculatory Alterations in Patients with Normodynamic and Hyperdynamic Septic Shock.

RATIONALE: In normodynamic septic shock, the quantitative assessment of sublingual microcirculation has shown decreases in perfused vascular density and red blood cell velocity. However, no studies have been performed in hyperdynamic septic shock. OBJECTIVES: To characterize the microcirculatory patterns and rule out the presence of fast red blood cell velocity in patients with hyperdynamic septic shock. METHODS: We prospectively evaluated the sublingual microcirculation in healthy volunteers (n = 20) and in patients with hyperdynamic (n = 20) and normodynamic (n = 20) septic shock. Hyperdynamic septic shock was defined by a cardiac index >4.0 L/min/m(2). The microcirculation was assessed with sidestream dark field imaging and AVA 3.0 software. MEASUREMENTS AND MAIN RESULTS: There were no differences in perfused vascular density, proportion of perfused vessels, or microvascular flow index between patients with hyperdynamic and normodynamic septic shock, but these variables were reduced compared with those of healthy volunteers, A similar pattern was observed in red blood cell velocity (912 ± 291, 968 ± 204, and 1303 ± 120 µm/s, respectively; P < 0.0001) and its coefficient of variation. In both types of septic shock, no microvessel had a red blood cell velocity higher than the 100th percentile value for healthy volunteers. CONCLUSIONS: Patients with hyperdynamic septic shock showed microcirculatory alterations similar to those of patients with normal cardiac output. Both groups of patients had reduced perfused vascular density and red blood cell velocity and increased flow heterogeneity compared with that of healthy subjects. Fast red blood cell velocity was not found, even in patients with high cardiac output. These results support the conclusion that microcirculatory function is frequently dissociated from systemic hemodynamic derangements in septic shock.

Valor prognóstico da disfunção ventricular diastólica em pacientes com sepse grave e choque séptico / Prognostic value of ventricular diastolic dysfunction in patients with severe sepsis and septic shock

RESUMO Objetivo: Avaliar a prevalência de disfunção miocárdica e seu valor prognóstico em pacientes com sepse grave e choque séptico. Métodos: Pacientes sépticos adultos, admitidos em uma unidade de terapia intensiva, foram estudados de forma prospectiva por meio de ecocardiografia transtorácica dentro das primeiras 48 horas após sua admissão e, então, entre o sétimo e o décimo dias. As variáveis ecográficas de função biventricular, inclusive a relação E/e', foram comparadas entre sobreviventes e não sobreviventes. Resultados: Foi realizado um total de 99 ecocardiogramas (53 na admissão e 46 entre os dias 7 e 10) em 53 pacientes com média de idade de 74 anos (desvio padrão de 13 anos). Estava presente disfunção sistólica em 14 (26%); disfunção diastólica foi observada em 42 (83%) pacientes; e ambos os tipos de disfunção estavam presentes em 12 (23%) pacientes. A relação E/e', ou índice de disfunção diastólica, foi o melhor preditor de mortalidade hospitalar segundo a área sob a curva ROC (0,71) e se constituiu em um preditor independente do desfecho, conforme determinado pela análise multivariada (odds ratio - OR = 1,36 [1,05 - 1,76]; p = 0,02). Conclusão: Em pacientes sépticos admitidos em uma unidade de terapia intensiva, a disfunção sistólica determinada ecograficamente não se associa com aumento da mortalidade. Em contraste, a disfunção diastólica foi um preditor independente do desfecho.

ABSTRACT Objectives: To evaluate the prevalence of myocardial dysfunction and its prognostic value in patients with severe sepsis and septic shock. Methods: Adult septic patients admitted to an intensive care unit were prospectively studied using transthoracic echocardiography within the first 48 hours after admission and thereafter on the 7th-10th days. Echocardiographic variables of biventricular function, including the E/e' ratio, were compared between survivors and non-survivors. Results: A total of 99 echocardiograms (53 at admission and 46 between days 7 - 10) were performed on 53 patients with a mean age of 74 (SD 13) years. Systolic and diastolic dysfunction was present in 14 (26%) and 42 (83%) patients, respectively, and both types of dysfunction were present in 12 (23%) patients. The E/e' ratio, an index of diastolic dysfunction, was the best predictor of hospital mortality according to the area under the ROC curve (0.71) and was an independent predictor of outcome, as determined by multivariate analysis (OR = 1.36 [1.05 - 1.76], p = 0.02). Conclusion: In septic patients admitted to an intensive care unit, echocardiographic systolic dysfunction is not associated with increased mortality. In contrast, diastolic dysfunction is an independent predictor of outcome.

Prognostic value of ventricular diastolic dysfunction in patients with severe sepsis and septic shock.

OBJECTIVES: To evaluate the prevalence of myocardial dysfunction and its prognostic value in patients with severe sepsis and septic shock. METHODS: Adult septic patients admitted to an intensive care unit were prospectively studied using transthoracic echocardiography within the first 48 hours after admission and thereafter on the 7th-10th days. Echocardiographic variables of biventricular function, including the E/e' ratio, were compared between survivors and non-survivors. RESULTS: A total of 99 echocardiograms (53 at admission and 46 between days 7 - 10) were performed on 53 patients with a mean age of 74 (SD 13) years. Systolic and diastolic dysfunction was present in 14 (26%) and 42 (83%) patients, respectively, and both types of dysfunction were present in 12 (23%) patients. The E/e' ratio, an index of diastolic dysfunction, was the best predictor of hospital mortality according to the area under the ROC curve (0.71) and was an independent predictor of outcome, as determined by multivariate analysis (OR = 1.36 [1.05 - 1.76], p = 0.02). CONCLUSION: In septic patients admitted to an intensive care unit, echocardiographic systolic dysfunction is not associated with increased mortality. In contrast, diastolic dysfunction is an independent predictor of outcome.

Visual diagnosis: 6-month-old boy with leg pain.

Pediatricians play an important role in diagnosing OI as a cause of fracture and may be asked to differentiate this uncommon genetic diagnosis from intentional trauma and other causes of fracture. Early referral to a pediatric endocrinologist, physical therapist, and orthopedic surgeon for the evaluation and treatment of low bone mass and recurrent fractures is important because early medical and surgical intervention may help to minimize the rate of future fractures, even within the first year of life. Continued follow-up with physical and occupational therapy, audiology testing, and regular evaluations of dental health are all essential for children with OI.

Lack of agreement between different observers and methods in the measurement of capillary refill time in healthy volunteers: an observational study.

OBJECTIVE: Peripheral perfusion abnormalities are relevant manifestations of shock. Capillary refill time is commonly used for their evaluation. However, the reproducibility of capillary refill time measurements and their correlation with other variables of peripheral perfusion, have not been comprehensively evaluated. Our goal was to determine, in healthy volunteers, the agreement between different methods of capillary refill time quantification and different observers, as well as their correlation with other markers of peripheral perfusion. Methods: We studied 63 healthy volunteers. Two observers measured capillary refill time by means of two methods, direct view (CRTchronome ter) and video analysis (CRTvideo). We also measured perfusion index (PI) derived from pulse plethysmography and finger pad temperature (T°peripheral). The agreement between observers and methods was assessed using the Bland and Altman method. Correlations were calculated using Pearson's correlation. A p-value<0.05 was considered significant. RESULTS: The 95% limits of agreement between the two observers were 1.9 sec for CRTchronometer and 1.7 sec for CRTvideo. The 95% limits of agreement between CRTchronometer and CRTvideo were 1.7 sec for observer 1 and 2.3 sec for observer 2. Measurements of CRTchronometer performed by the two observers were correlated with T°peripheral. Measurements of CRTvideo performed by the two observers were correlated with T°peripheral and perfusion index. CONCLUSION: In healthy volunteers, measurements of capillary refill time performed by either different observers or different methods showed poor agreement. Nevertheless, capillary refill time still reflected peripheral perfusion as shown by its correlation with objective variables of peripheral perfusion.

Falta de concordância entre diferentes observadores e métodos na mensuração do tempo de reenchimento capilar em voluntários saudáveis: estudo observacional / Lack of agreement between different observers and methods in the measurement of capillary refill time in healthy volunteers: an observational study

Objetivo: As anomalias da perfusão periférica são manifestações importantes do choque, sendo o tempo de reenchimento capilar comumente utilizado em sua avaliação. Entretanto, a reprodutibilidade das mensurações do tempo de reenchimento capilar e sua correlação com outras variáveis da perfusão periférica não foram avaliadas de forma abrangente. Nosso objetivo foi determinar, em voluntários saudáveis, a concordância entre diferentes métodos e diferentes observadores na quantificação do tempo de reenchimento capilar, assim como sua correlação com outros marcadores da perfusão periférica. Métodos: Estudamos 63 voluntários saudáveis. Dois observadores mediram o tempo de reenchimento capilar por meio de dois métodos distintos: visão direta (TRCcronômetro) e vídeo-análise (TRCvídeo). Medimos também o índice de perfusão derivado de pletismografia de pulso e a temperatura da polpa digital (Tºperiférica). A concordância entre os observadores e os métodos foi avaliada utilizando o método de Bland-Altman. As correlações foram calculadas utilizando a correlação de Pearson. Valor de p<0,05 foi considerado significante. Resultados: Os limites de concordância de 95% entre ambos os observadores foram de 1,9 segundo para TRCcronômetro e 1,7 segundo para TRCvídeo. Os limites de concordância de 95% entre TRCcronômetro e TRCvídeo foram de 1,7 segundo para o Observador 1 e 2,3 segundos para o Observador 2. As mensurações do TRCcronômetro realizadas pelos dois observadores se correlacionaram com a Tºperiférica. As mensurações do TRCvídeo realizadas pelos dois observadores se correlacionaram com a Tºperiférica e o índice de perfusão. ...

Objective: Peripheral perfusion abnormalities are relevant manifestations of shock. Capillary refill time is commonly used for their evaluation. However, the reproducibility of capillary refill time measurements and their correlation with other variables of peripheral perfusion, have not been comprehensively evaluated. Our goal was to determine, in healthy volunteers, the agreement between different methods of capillary refill time quantification and different observers, as well as their correlation with other markers of peripheral perfusion. Methods: We studied 63 healthy volunteers. Two observers measured capillary refill time by means of two methods, direct view (CRTchronometer) and video analysis (CRTvideo). We also measured perfusion index (PI) derived from pulse plethysmography and finger pad temperature (Tºperipheral). The agreement between observers and methods was assessed using the Bland and Altman method. Correlations were calculated using Pearson's correlation. A p-value<0.05 was considered significant. Results: The 95% limits of agreement between the two observers were 1.9 sec for CRTchronometer and 1.7 sec for CRTvideo. The 95% limits of agreement between CRTchronometer and CRTvideo were 1.7 sec for observer 1 and 2.3 sec for observer 2. Measurements of CRTchronometer performed by the two observers were correlated with Tºperipheral. Measurements of CRTvideo performed by the two observers were correlated with Tºperipheral and perfusion index. Conclusion: In healthy volunteers, measurements of capillary refill time performed by either different observers or different methods showed poor agreement. Nevertheless, capillary refill time still reflected peripheral perfusion as shown by its correlation with objective variables of peripheral perfusion. .