Swyer-James-Macleod syndrome is a rare pulmonary disorder. It is characterized as a constrictive bronchiolitis resulting from loss of pulmonary vascularity secondary to a previous upper respiratory infection. We present the case of a 5-year-old boy who presented to the emergency department (ED) for acute asthma exacerbation. Radiographic imaging confirmed the diagnosis of Swyer-James-Macleod syndrome. The patient was admitted to the pediatric floor and treated with symptomatic management and discharged home after complete resolution of symptoms. Swyer-James syndrome is a rare disease that should be considered in individuals with recurrent pulmonary infections and hyperactive airway disease. Radiographic or computed tomography imaging is required in such patients for diagnosis. Swyer-James-Macleod syndrome is an extremely rare radiographic finding characterized by unilateral pulmonary hyperlucency. This diagnostic finding is a long-term sequela of bronchiolitis obliterans. Presentation of this syndrome is characterized by dyspnea, hemoptysis, reactive upper airway disease, and recurrent pulmonary infections. The etiology of this syndrome is diverse, as are its long-term effects. This rare disease causes a unique diagnostic challenge in the ED where the presenting sign is often an acute asthma exacerbation as witnessed in our patient. In the setting of both pediatric and adult patients, Swyer-James syndrome should be considered in patients with acute asthma exacerbation and a positive history of upper respiratory infections. A 5-year-old known asthmatic boy presented to the emergency department (ED) with chief complaints of wheezing and chest tightness of 1-day duration. Before presentation, the patient reported intermittent asthma for 2 weeks requiring daily use of albuterol. His symptoms worsened the day before presentation, when he began to cough and experience posttussive vomiting, chest tightness, and a tactile fever. The patient never became cyanotic or ashen. His symptoms did not improve after multiple albuterol treatments at home, after which he was brought to the ED. Medical history was positive for eczema and asthma. The patient reported allergies to egg, wheat, seafood, and animal dander. Family history was positive for asthma in both parents. There was no history of travel or sick contacts. Immunizations were up to date. At the ED, his temperature was 98.2°F; pulse, 148 beats per minute; respiratory rate, 18 breaths per minute; blood pressure, 112/79, with SaO 2 of 93% on room air. Physical examination revealed signs of moderate respiratory distress. Nasal flaring with supraclavicular, subcostal, and intercostal retractions were noted. Auscultation of the lungs revealed poor air entry with bilateral expiratory wheezing. The rest of the examination was unremarkable.

Typhoid fever and salmonellosis, which are caused by Salmonella typhi and typhimurium, respectively, are responsible for significant morbidity and mortality in both developed and developing countries. We model typhoid fever using mice infected with Salmonella typhimurium, which results in a systemic disease, whereby the outcome of infection is variable in different inbred strains of mice. This model recapitulates several clinical aspects of the human disease and allows the study of the host response to Salmonella typhimurium infection in vivo. Previous work in our laboratory has identified three loci (Ity, Ity2, and Ity3) in the wild-derived MOLF/Ei mice influencing survival after infection with Salmonella typhimurium. Fine mapping of the Ity3 locus indicated that two sub-loci contribute collectively to the susceptibility of B6.MOLF-Ity/Ity3 congenic mice to Salmonella infection. In the current paper, we provided further evidence supporting a role for Ncf2 (neutrophil cytosolic factor 2 a subunit of NADPH oxidase) as the gene underlying the Ity3.1 sub-locus. Gene expression profiling indicated that the Ity3.1 sub-locus defined a global gene expression signature with networks articulated around Ncf2. Furthermore, based on differential expression and complementation analysis using Selp (selectin-P) knock-out mice, Selp was identified as a strong candidate gene for the Ity3.2 sub-locus.

ObjectiveThe objective was to evaluate and improve accuracy of anticipatory counseling regarding neonatal intervention for prenatally diagnosed tetralogy of Fallot (TOF) by assessing new and previously published predictors of neonatal intervention.MethodsThis is a multi‐center, retrospective study from three centers of 112 fetal TOF patients undergoing third trimester fetal echocardiograms from 2004 to 2017. Additional cardiac defects requiring neonatal intervention were excluded. Fetal echocardiographic, clinical, and consultation data were compared between neonatal and late intervention. Optimal echocardiographic values were determined.ResultsTwenty‐six infants (23%) required neonatal intervention. Those infants had significantly different pulmonary valve (PV) z‐scores, PV:aortic valve (AoV) ratios, PV:AoV z‐score differences (absolute difference between z‐scores), and increased likelihood of abnormal ductal flow. Counseling during fetal echocardiogram regarding interventional timing was accurate for 50% needing neonatal intervention and 86% undergoing late intervention (P = .002). The best neonatal intervention predictors were PV:AoV ratio of <0.6 and counseling for neonatal intervention. PV:AoV z‐score difference ≥5 provided 89% negative predictive value for excluding patients from neonatal repair.ConclusionsThird trimester fetal echocardiograms can predict interventional timing. The best predictors of neonatal intervention are PV:AoV ratio <0.6, PV:AoV z‐score difference ≥5, and cardiologist counseling that neonatal intervention was likely.

HIV/AIDS patients often present with orogenital ulcers. In the immunocompromised patient diagnosis of these ulcers pose a challenge, as there is a myriad of etiologies. We present a case of an HIV/AIDS patient with recurrent orogenital aphthosis that was confirmed to have concomitant diagnosis of Behcet's disease. Proper awareness of the causes of these ulcers is essential for prompt and effective treatment. While rare causes may be at the bottom of a differential list in an immunocompetent host, when HIV/AIDS is involved these rare causes often percolate to the top.

The combination of Von Recklinghausen disease with a pheochromocytoma has a genetic linkage but is an exceedingly rare occurrence. Von Recklinghausen disease or neurofibromatosis type 1 (NF1) is a neurocutaneous disorder characterized by cafe-au-lait macules and multiple, soft tissue neurofibromas. While a pheochromocytoma is a neuroendocrine tumor characterized by intermittent hypertension. NF1 predisposes patients to pheochromocytomas; however, this occurrence is extremely rare. We describe the case of a young male with neurofibromatosis and a pheochromocytoma.