Multiorgan Failure in Hemophagocytic Lymphohistiocytosis Secondary to Acute Monocytic Leukemia and Myocarditis in a Pediatric Patient.

Acute monocytic leukemia (AML), a subtype of acute myeloid leukemia, is a rare leukemia found in children. It occurs more frequently in adults over the age of 60. Myocarditis represents inflammation of the muscular layer of the heart, the myocardium causing weakening of the muscles that can lead to hemodynamic instability from a reduced ejection fraction. Myocarditis in the pediatric population is most commonly secondary to a viral or infectious etiology. Hemophagocytic lymphohistiocytosis (HLH) is a rare condition of immune dysregulation characterized by severe organ damage induced by an increased inflammatory response and uncontrolled T-cell and macrophage activation. In this case report, we examine a rare presentation of leukemic myocarditis in the presence of HLH, which displays an uncommon cause of an inflammatory state with several complicated concomitant diagnoses. Our patient developed severe multiorgan dysfunction involving liver and kidney failure that required prolonged critical care support, and the patient expired due to his multiorgan failure. We highlight the unusual clinical presentation of myocarditis in the setting of HLH and AML in this complicated pediatric patient and aim to improve outcomes of patients presenting similarly in the future.

The need for continuous quality assessment for providing optimal comprehensive care for patients with alpha-1 antitrypsin deficiency.

Background: Alpha-1-antitrypsin deficiency (AATD) is an orphan disease that mainly affecting the liver and the lung. This creates difficulties to ensure that comprehensive care is administered to both organ systems. Past assessments of care delivered to patients with AATD demonstrated that improvements are needed. For that reason, we reassessed a population of patients with AATD in a large health care system to see if past findings affected present care. Methods: We performed electronic health record (EHR) reviews on all patients with documented AATD and confirmed the diagnosis by evidence of genotyping. We then selected the patients with the ZZ genotype to review comprehensive care. We further compared the findings in patients treated by different specialists (allergy immunology, gastroenterology, and pulmonary). The data were captured and assessed by using a secure web application for building and managing online surveys and data bases. REDCap. Results: We found a total of 329 patients with diagnostic codes for AATD, of these, 203 patients had a confirmed abnormal genotype. Confirmed genotypes were MZ (n = 69), ZZ (n = 48), MS (n = 22), SZ (n = 22). Further focus was applied to the care of the ZZ population secondary to a predisposition to potential severe lung and liver disease. The findings suggest that care can be improved no matter which specialist cares for the patient. Conclusion: Our study demonstrated that all three subspecialty groups had room for improvement in providing care to patients with AATD. Our study further demonstrated the need for recurrent quality-assurance programs that may be aided by care suggestions built into the EHR.

Clinical Outcomes and Return to Sport After Arthroscopic Anterior, Posterior, and Combined Shoulder Stabilization.

BACKGROUND: Glenohumeral instability is a common abnormality, especially among athletes. Previous studies have evaluated outcomes after arthroscopic stabilization in patients with anterior or posterior shoulder instability but have not compared outcomes between groups. PURPOSE: To compare return-to-sport and other patient-reported outcomes in patients after primary arthroscopic anterior, posterior, and combined anterior and posterior shoulder stabilization. STUDY DESIGN: Cohort study; Level of evidence, 3. METHODS: Patients who underwent primary arthroscopic anterior, posterior, or combined anterior and posterior shoulder stabilization were contacted at a minimum 2-year follow-up. Patients completed a survey that consisted of return-to-sport outcomes as well as the Western Ontario Shoulder Instability Index (WOSI), Single Assessment Numeric Evaluation (SANE), American Shoulder and Elbow Sur'geons (ASES) score, and Shoulder Activity Scale. RESULTS: A total of 151 patients were successfully contacted (anterior: n = 81; posterior: n = 22; combined: n = 48) at a mean follow-up of 3.6 years. No significant differences were found between the groups with regard to age at the time of surgery or time to follow-up. No significant differences were found between the groups in terms of WOSI (anterior: 76; posterior: 70; combined: 78; P = .28), SANE (anterior: 87; posterior: 85; combined: 87; P = .79), ASES (anterior: 88; posterior: 83; combined: 91; P = .083), or Shoulder Activity Scale (anterior: 12.0; posterior: 12.5; combined: 12.5; P = .74) scores. No significant difference was found between the groups in terms of the rate of return to sport (anterior: 73%; posterior: 68%; combined: 75%; P = .84). CONCLUSION: Athletes undergoing arthroscopic stabilization of anterior, posterior, or combined shoulder instability can be expected to share a similar prognosis. High patient-reported outcome scores and moderate to high rates of return to sport were achieved by all groups.