Publications by authors named "B T Hehn"
Bronchoscopic lung volume reduction is a procedure that involves placement of valves into the lung to intentionally cause atelectasis to help with perfusion-ventilation matching. There are strict exclusion criteria, such as hypercapnia, that prevent patients from qualifying for the procedure based on the early trials. We present a case of a patient that became a candidate for the procedure after utilizing AVAPS after BPAP failed to lower his PCO2 to qualify for the procedure.
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- VEXAS syndrome is a rare, recently identified autoinflammatory disease primarily affecting middle-aged men, with symptoms like skin lesions, fever, and joint pain.
- A case study of a man in his 40s presented with various symptoms such as testicular pain, a rash, and reduced blood cell counts, leading to extensive testing including a genetic analysis.
- Diagnosis is confirmed through identifying a mutation in the UBA-1 gene, and treatment typically involves corticosteroids and JAK inhibitors like ruxolitinib to manage symptoms.
With the advent of bronchoscopic lung-volume reduction (BLVR), this minimally invasive technique represents a new and effective way of managing the debilitating symptoms associated with severe centrilobular emphysema. Despite its vast potential in the management of this disease, there are still several potential risk factors associated with the procedure that may predispose the patient to increased morbidity. Our patient received four endobronchial valves in the right-upper lobe (RUL) and right-middle lobe (RML).
View Article and Find Full Text PDFThe signal peptide region of preproinsulin (PPI) contains epitopes targeted by HLA-A-restricted (HLA-A0201, A2402) cytotoxic T cells as part of the pathogenesis of β-cell destruction in type 1 diabetes. We extended the discovery of the PPI epitope to disease-associated and (risk) and and (protective) alleles, revealing that four of six alleles present epitopes derived from the signal peptide region. During cotranslational translocation of PPI, its signal peptide is cleaved and retained within the endoplasmic reticulum (ER) membrane, implying it is processed for immune recognition outside of the canonical proteasome-directed pathway.
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