Mary A. Nevin

Tricuspid regurgitant jet velocity elevation and its relationship to lung function in pediatric sickle cell disease.

Concerns about the morbidity and mortality associated with tricuspid regurgitant jet velocity (TRJV) elevation, which may indicate pulmonary hypertension (PHT), in adults with sickle cell disease (SCD) have prompted growing interest in screening the pediatric sickle cell population. The goals of our study were to estimate the prevalence of TRJV elevation and determine its relationship to pulmonary function in children and young adults with SCD at baseline. Seventy‐eight subjects (10–24 years old) with SCD underwent prospective screening by Doppler echocardiogram (ECHO), complete lung function evaluation, and laboratory testing as part of standard care at steady state. Tricuspid regurgitation was quantifiable in 68/78 (87%) subjects and peak TRJV was ≥2.5 m/sec in 26/78 (33.3%) evaluated. The frequency of obstruction, restriction, or abnormal gas exchange found on lung function evaluation was not significantly different in subjects with and without TRJV elevation. However, significant inverse correlations were observed between TRJV and both % predicted forced vital capacity (FVC) (ru2009=u2009−0.29, Pu2009=u20090.022) and oxygen saturation (ru2009=u2009−0.26, Pu2009=u20090.036). When compared to subjects without TRJV elevation, subjects with TRJV elevation had significantly lower % predicted forced expiratory volume in 1 sec (FEV1) (78.9u2009±u200914.4 vs. 86.6u2009±u200913.0%, Pu2009=u20090.023), FVC (82.8u2009±u200914.1 vs. 90.7u2009±u200912.9%, Pu2009=u20090.017), and oxygen saturation (95.8u2009±u20093.2 vs. 97.5u2009±u20092.4%, Pu2009=u20090.016). We found that the combination of low hemoglobin and low % predicted FVC best predicted TRJV elevation (χ2u2009=u200917.05, Pu2009=u20090.001) in our cohort, correctly identifying 70% of cases and resulting in positive and negative predictive values of 60 and 74%, respectively. We conclude that in this young population with SCD, TRJV elevation that is not significantly associated with abnormal lung function is common at baseline. Pediatr Pulmonol. 2009; 44:281–289.

Functional capacity in children and young adults with sickle cell disease undergoing evaluation for cardiopulmonary disease

Although cardiopulmonary disease is associated with decreased functional capacity among adults with sickle cell disease (SCD), its impact on functional capacity in children with SCD is unknown. We evaluated 6‐min walk (6MW) distance in 77 children and young adults with SCD undergoing screening for cardiopulmonary disease. Of 30 subjects who also underwent cardiopulmonary exercise testing, we found evidence for decreased exercise capacity in a significant proportion. Exercise capacity was related to baseline degree of anemia and was significantly lower in subjects with a history of recurrent acute chest syndrome. We found that 6MW distance adjusted for weight and body surface area was shorter in subjects with restrictive lung disease but that only 6MW adjusted for weight remained significantly shorter when we controlled for baseline hemoglobin. Exercise capacity was not significantly different in subjects with and without cardiopulmonary disease. We conclude that restrictive lung disease is associated with shorter 6MW distances in children and young adults with SCD, but that variables associated with decreased exercise capacity, other than anemia, remain unclear. Our study underscores the importance of further delineating the direct pathophysiologic processes that contribute to decreased exercise capacity observed among individuals with SCD and cardiopulmonary disease. Am. J. Hematol., 2009.

Pediatric Obesity, Metabolic Syndrome, and Obstructive Sleep Apnea Syndrome

The prevalence of obesity in the pediatric population has dramatically increased in the last 30 years. While the adverse health effects of obesity have long been recognized in adults, many of these complications are now understood to begin in early childhood. Obese children and adolescents are significantly more likely than their peers of healthy weight to suffer from obstructive sleep apnea and metabolic syndrome. In turn, affected individuals may experience myriad serious clinical sequelae; neuro-cognitive, psychiatric, cardiovascular, and endocrinologic complications have each been extensively documented. Thus, the spectrum of obesity-related disease represents a serious but preventable threat to personal and family wellness; additionally, it is a source of considerable health care expenditure and represents a national and international health crisis. The optimal care of these patients will be best achieved through the pediatric health care providers timely recognition of these clinical problems and knowledge of appropriate intervention strategies.

Successful use of plasmapheresis for granulomatosis with polyangiitis presenting as diffuse alveolar hemorrhage.

Diffuse alveolar hemorrhage (DAH) is uncommon in pediatric patients and is a rare presenting sign of granulomatosis with polyangiitis (GPA). We present the case a 14‐year‐old girl who presented with respiratory failure secondary to DAH as the initial presenting sign of GPA. Her clinical course improved after initiation of plasmapheresis therapy and she is now in clinical remission. Pediatr Pulmonol. 2013; 48:614–616.

Respiratory Complications of the Gastrointestinal System

Symptoms which are relatable to the respiratory and gastrointestinal tract are commonly encountered in infancy and childhood. Due to the relative anatomic proximity and shared innervation of the airway and esophagus, disease or inflammation in the gastrointestinal tract may contribute to pathophysiologic changes in the airway and lungs. The manifestation of symptoms and clinical findings such as cough, wheezing, dyspnea, or hypoxemia may occur in primary gastrointestinal disease and may create diagnostic uncertainty for the care provider. An awareness of this potential for overlapping symptoms and for their physiologic basis is advocated.