Case Report of MPO+ ANCA Vasculitis with Pauci-immune GN Associated with Invasive Ductal Carcinoma of the Breast.

BACKGROUND: Malignancy-associated vasculitis usually presents in the form of polyarteritis nodosa or leukocytoclastic vasculitis. However, ANCA vasculitis associated with malignancy is rare. Here, we present a case of MPO+ ANCA vasculitis with pauci-immune GN associated with invasive ductal carcinoma of the breast. CASE PRESENTATION: A 66-year-old female with a history of rheumatoid arthritis, Hashimoto's thyroiditis, and psoriasis presented with multiple joint pain, body aches, petechial rash, paresthesia and numbness, and deranged renal function a month after diagnosis of localized left breast invasive ductal carcinoma. Renal biopsy showed crescentic pauci-immune glomerulonephritis, and serology was positive for Perinuclear Antineutrophil Cytoplasmic Antibody (P-ANCA) and myeloperoxidase (MPO). The disease course was complicated by diverticulitis with peritonitis and intraperitoneal abscess collection, which required laparoscopic peritoneal lavage and additional interventional radiology-guided drainage of the abscess. We treated the patient successfully with steroids, rituximab, and mastectomy for left breast malignant lesions, resulting in the resolution of symptoms, normalization of inflammatory markers, and ANCA seroconversion. CONCLUSION: Treating ANCA-associated Vasculitis (AAV) in surgical emergencies like bowel perforation can be challenging. Individualized treatment strategy tailored to patients' acute needs is crucial. In this case, we considered malignancy-associated vasculitis and pursued treatment that fit the patient's clinical situation in a multidisciplinary approach.

Sarcomatoid Intrahepatic Cholangiocarcinoma: A Rare and Aggressive Primary Liver Cancer.

Sarcomatoid intrahepatic cholangiocarcinoma (S-iCCA) is a rare variant of primary liver cancer with a poor prognosis due to local aggressive expansion and frequent metastases. The pathogenesis remains unclear, but theories suggest epithelial-mesenchymal transition, biphasic differentiation of pluripotent stem cells, or sarcomatoid re-differentiation of immature multipotent carcinoma cells. Chronic hepatitis B and C, cirrhosis, and age above 40 are plausible contributors. Diagnosis of S-iCCA requires immunohistochemical evidence of both mesenchymal and epithelial molecular expression. Early detection and total resection are the current mainstay approach. We report a case of metastatic S-iCCA in a 53-year-old male with alcohol use disorder who underwent en bloc right hepatic lobectomy, right adrenalectomy, and cholecystectomy.

Immune Reconstitution Inflammatory Syndrome Presenting as Disseminated Kaposi Sarcoma.

We present a patient who was previously diagnosed with HIV and had multiple violaceous skin lesions at the time of his diagnosis. Following the initiation of antiretroviral therapy (ART), the number of lesions increased significantly and he developed shortness of breath, which prompted hospital admission for further workup. Biopsy of the skin lesions confirmed the diagnosis of Kaposi sarcoma (KS). Bronchoscopy with biopsy revealed KS lesions in his respiratory system. Imaging and biopsy confirmed KS invasion of lymph nodes. Due to widespread KS, he was diagnosed with immune reconstitution inflammatory syndrome (IRIS). Because of the lack of improvement on ART alone, he was started on chemotherapy, which decreased the size of existing skin lesions, stalled the development of new skin lesions, and led to symptom improvement. As a result of this case, we recommend that treatment teams have close follow-ups of patients started on ART and that they remain mindful of the possibility of IRIS. Disseminated KS may warrant a prompt response with chemotherapy to improve outcomes.

Cardiopulmonary Outcomes After the Nuss Procedure in Pectus Excavatum.

Background Pectus excavatum is the most common chest wall deformity. There is still controversy about cardiopulmonary limitations of this disease and benefits of surgical repair. This study evaluates the impact of pectus excavatum on the cardiopulmonary function of adult patients before and after a modified minimally invasive repair. Methods and Results In this retrospective cohort study, an electronic database was used to identify consecutive adult (aged ≥18 years) patients who underwent cardiopulmonary exercise testing before and after primary pectus excavatum repair at Mayo Clinic Arizona from 2011 to 2020. In total, 392 patients underwent preoperative cardiopulmonary exercise testing; abnormal oxygen consumption results were present in 68% of patients. Among them, 130 patients (68% men, mean age, 32.4±10.0 years) had post-repair evaluations. Post-repair tests were performed immediately before bar removal with a mean time between repair and post-repair testing of 3.4±0.7 years (range, 2.5-7.0). A significant improvement in cardiopulmonary outcomes (P<0.001 for all the comparisons) was seen in the post-repair evaluations, including an increase in maximum, and predicted rate of oxygen consumption, oxygen pulse, oxygen consumption at anaerobic threshold, and maximal ventilation. In a subanalysis of 39 patients who also underwent intraoperative transesophageal echocardiography at repair and at bar removal, a significant increase in right ventricle stroke volume was found (P<0.001). Conclusions Consistent improvements in cardiopulmonary function were seen for pectus excavatum adult patients undergoing surgery. These results strongly support the existence of adverse cardiopulmonary consequences from this disease as well as the benefits of surgical repair.

Sex Differences in Objective Measures of Adult Patients Presenting for Pectus Excavatum Repair.

BACKGROUND: Women have a reported incidence of pectus deformities four to five times less than men. Sex differences have not been well studied. METHODS: A retrospective review was performed of adult patients (aged 18 years or more) who underwent a pectus excavatum repair at Mayo Clinic in Arizona (January 1, 2010, to December 31, 2019). RESULTS: In total, 776 adults underwent pectus repair, with 30% being women. Women presented older (mean age 35 vs 32 years, P = .007) and more symptomatic. Despite this, women performed better on cardiopulmonary exercise testing (higher maximum oxygen consumption and oxygen pulse). Women had more severe deformities (Haller index 5.9 vs 4.3, P < .001). However, in 609 patients undergoing attempted primary minimally invasive pectus repair, intraoperative fractures/osteotomies occurred equally between men and women, with the majority occurring in patients 30 years of age or more (11.5% for age 30 or more, 1.7% for age less than 30; total 7%). Women were also less likely to require three bars for repair (12% vs 42%, P < .001). Hospital length of stay and postoperative complication rates were not significantly different. Postoperatively, women reported a greater daily intensity of pain, but only on the initial postoperative day did they use significantly more opioids than men. Cardiopulmonary exercise testing of 142 patients undergoing baseline and postoperative evaluation at bar removal showed equal and significant benefits in both sexes. CONCLUSIONS: Women presented for pectus excavatum repair at an older age and with greater symptoms and more severe symptoms. Despite this, women required fewer bars, and there were no significant differences in length of stay or complications. Cardiopulmonary benefits of repair were significant and equal for both women and men.

Cardiac Transplantation and Consecutive Minimally Invasive Pectus Excavatum Repair.

Pectus excavatum is a common chest wall deformity with inward deviation of sternum and accompanying ribs. The depression can cause symptomatic cardiac compression, although the cardiopulmonary impact remains controversial. We present 2 cases of cardiac transplantation followed by modified minimally invasive pectus excavatum repair due to the hemodynamic consequences of the pectus deformity.

Mild to moderate COVID-19 illness in adult outpatients: Characteristics, symptoms, and outcomes in the first 4 weeks of illness.

ABSTRACT: Most patients with coronavirus disease 2019 (COVID-19) have mild to moderate illness not requiring hospitalization. However, no study has detailed the evolution of symptoms in the first month of illness.At our institution, we conducted remote (telephone and video) visits for all adult outpatients diagnosed with COVID-19 within 24 h of a positive nasopharyngeal polymerase chain test for SARS-CoV-2. We repeated regular video visits at 7, 14, and 28 days after the positive test, retrospectively reviewed the prospective data collected in the remote visits, and constructed a week by week profile of clinical illness, through week 4 of illness.We reviewed the courses of 458 symptomatic patients diagnosed between March 12, 2020, and June 22, 2020, and characterized their weekly courses. Common initial symptoms included fever, headache, cough, and chest pain, which frequently persisted through week 3 or longer. Upper respiratory or gastrointestinal symptoms were much shorter lived, present primarily in week 1. Anosmia/ageusia peaked in weeks 2 to 3. Emergency department visits were frequent, with 128 visits in the 423 patients who were not hospitalized and 48 visits among the 35 outpatients (7.6%) who were eventually hospitalized (2 subsequently died). By the fourth week, 28.9% said their illness had completely resolved. After the 4-week follow up, 20 (4.7%) of the 423 nonhospitalized patients had further medical evaluation and management for subacute or chronic COVID-19 symptoms.Mild to moderate outpatient COVID-19 is a prolonged illness, with evolving symptoms commonly lasting into the fourth week of illness.

Efficacy of standard chest compressions in patients with Nuss bars.

BACKGROUND: The Nuss procedure temporarily places intrathoracic bars for repair of pectus excavatum (PE). The bars may impact excursion and compliance of the anterior chest wall while in place. Effective chest compressions during cardiopulmonary resuscitation (CPR) require depressing the anterior chest wall enough to compress the heart between sternum and spine. We assessed the force required to perform the American Heart Association's recommended chest compression depth after Nuss repair. METHODS: A lumped element elastic model was developed to simulate the relationship between chest compression forces and displacement with focus on the amount of force required to achieve a depth of 5 cm in the presence of 1-3 Nuss bars. Literature review was conducted for evidence supporting potential use of active abdominal compressions and decompression (AACD) as an alternative method of CPR. RESULTS: The presence of bars notably lowered compression depth by a minimum of 69% compared to a chest without bar(s). The model also demonstrated a dramatic increase (minimum of 226%) in compressive forces required to achieve recommended 5 cm depth. Literature review suggests AACD could be an alternative CPR in patients with Nuss bar(s). CONCLUSIONS: In our model, Nuss bars limited the ability to perform chest compressions due to increased force required to achieve a 5 cm compression. The greater the number of Nuss bars present the greater the force required. This may prevent effective CPR. Use of active abdominal compressions and decompressions should be studied further as an alternative resuscitation modality for patients after the Nuss procedure.