Assessing the role of tracheostomy placement in bronchopulmonary dysplasia with pulmonary hypertension.

OBJECTIVE: Bronchopulmonary dysplasia (BPD) is a common complication of preterm birth and is associated with abnormal vasculature that contributes to pulmonary hypertension (PH). We evaluated how a tracheostomy may alter PH in these patients. METHODS: A retrospective chart review over 15-years identified 17 patients with BPD and PH who underwent tracheostomy. Each patient had four echocardiograms re-reviewed and scored for tricuspid valve regurgitation velocity (TR), tricuspid annular plane systolic excursion (TAPSE), right atrial cross-sectional area (RACA), and left ventricle eccentricity indices (EI). RESULT: There was improvement in TR, TAPSE, RACA, and left ventricle EI indicating reduction in PH after tracheostomy. CONCLUSION: PH improves over time though role of tracheostomy in PH needs to be further defined. The EI may be a sensitive marker to follow over time in these patients.

When to panic about a panic attack: A challenging case of hypersensitivity pneumonitis.

The COVID-19 pandemic has created diagnostic difficulties with the increase in mental health illnesses that often present with nonspecific symptoms, like hypersensitivity pneumonitis. Hypersensitivity pneumonitis is a complex syndrome of varying triggers, onset, severity, and clinical manifestations that can be challenging to diagnose in many cases. Typical symptoms are nonspecific and can be attributed to other entities. There are no pediatric guidelines, which contributes to diagnostic difficulties and delays in treatment. It is particularly important to avoid diagnostic biases, have an index of suspicion for hypersensitivity pneumonitis, and to develop pediatric guidelines as outcomes are excellent when diagnosed and treated promptly. This article discusses hypersensitivity pneumonitis with a focus on the causes, pathogenesis, diagnostic approach, outcomes, and prognosis while using a case to illustrate the diagnostic difficulties worsened by the COVID-19 pandemic.

Carmi Syndrome in a Preterm Neonate: A Multidisciplinary Approach and Ethical Challenge.

Epidermolysis bullosa (EB) is characterized by blistering of the skin and mucosal erosions caused by hemidesmosomal abnormalities. EB is divided into 3 major subgroups depending on the particular location of tissue separation: EB simplex, dystrophic EB, and junctional EB. Junctional EB (JEB) can further be broken down into Herlitz, non-Herlitz, and JEB with pyloric atresia (Carmi syndrome) depending on genetic and histologic testing. When extensive, management of a patient with EB can be challenging due to not only cutaneous but also extracutaneous manifestations as well. Families and health care teams are often faced with difficult decisions in their infant's best interest. We report a case of a preterm neonate with Carmi syndrome and unique findings on immunofluorescence studies. The patient's course was complicated by multisystem involvement and ultimately death. A multidisciplinary approach was crucial in the light of diagnostic, therapeutic, and ethical challenges.