Chest
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Comparative Study
Heterogeneous Pulmonary Phenotypes Associated With Mutations in the Thyroid Transcription Factor Gene NKX2-1.
Mutations in the gene encoding thyroid transcription factor, NKX2-1, result in neurologic abnormalities, hypothyroidism, and neonatal respiratory distress syndrome (RDS) that together are known as the brain-thyroid-lung syndrome. To characterize the spectrum of associated pulmonary phenotypes, we identified individuals with mutations in NKX2-1 whose primary manifestation was respiratory disease. ⋯ Patients with mutations in NKX2-1 may present with pulmonary manifestations in the newborn period or during childhood when thyroid or neurologic abnormalities are not apparent. Surfactant dysfunction and, in more severe cases, disrupted lung development are likely mechanisms for the respiratory disease.
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Rhabdomyolysis is a well-known clinical syndrome of muscle injury associated with myoglobinuria, electrolyte abnormalities, and often acute kidney injury (AKI). The pathophysiology involves injury to the myocyte membrane and/or altered energy production that results in increased intracellular calcium concentrations and initiation of destructive processes. Myoglobin has been identified as the primary muscle constituent contributing to renal damage in rhabdomyolysis. ⋯ There is little evidence other than from animal studies, retrospective observational studies, and case series to support the routine use of bicarbonate-containing fluids, mannitol, and loop diuretics. Hyperkalemia and compartment syndrome are additional complications of rhabdomyolysis that must be treated effectively. A definite need exists for well-designed prospective studies to determine the optimal management of rhabdomyolysis.
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The advent of bronchial thermoplasty (BT) provides a novel therapeutic option for asthma refractory to traditional medical therapy. Insurance coverage poses significant frustration for centers performing BT. Although clinical research has provided evidence of the usefulness and long-term safety of BT, establishing "reasonability and necessity" remains a daunting challenge in securing private and governmental insurance coverage. ⋯ Based on increasing implementation of BT nationwide, the American Medical Association CPT Editorial Panel has assigned category 1 CPT codes for BT in their published 2013 professional edition. It is hoped that such a recommendation will reinforce the medical community's belief in the usefulness of BT and help facilitate decisions on insurance coverage. The ability to secure coverage for BT through physician advocacy and Centers for Medicare and Medicaid Services support will help move the treatment of refractory asthma forward.
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Comparative Study
Systemic BP and Heart Rate as Prognostic Indicators in Pulmonary Arterial Hypertension.
Heart rate (HR) and systolic BP (SBP) are significant multivariate predictors of survival in patients with pulmonary arterial hypertension (PAH) as part of a 19-element formula. To what extent HR and BP alone predict survival and future hospitalization in patients with PAH is unknown. ⋯ Changes in vital signs from enrollment to first follow-up were less predictive of mortality than the values of vital-sign parameters at either enrollment or first follow-up. HR, SBP, and SBP/HR at enrollment identified high-risk groups with survival differences of 5% to 7% and freedom from hospitalization differences of 9% to 11% vs lower-risk groups. SBP/HR defines the highest-risk group, including most of the high-risk patients defined by HR and SBP separately.
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Comparative Study
A new instrument to assess physician skill at thoracic ultrasound, including pleural effusion markup.
To reduce complications and increase success, thoracic ultrasound is recommended to guide all chest drainage procedures. Despite this, no tools currently exist to assess proceduralist training or competence. This study aims to validate an instrument to assess physician skill at performing thoracic ultrasound, including effusion markup, and examine its validity. ⋯ Procedural training on mannequins prior to unsupervised performance on patients is rapidly becoming the standard in medical education. This study has validated the UGSTAT, which can now be used to determine the adequacy of thoracic ultrasound training prior to clinical practice. It is likely that its role could be extended to live patients, providing a way to document ongoing procedural competence.