Systemic lupus erythematosus (SLE) is a chronic autoimmune disease with unpredictable course and flares. The clinical manifestation can vary from mild to severe and life-threatening disease. Infection is the primary cause of mortality in hospitalized SLE patients. There is a paucity of evidence to support the co-management of SLE with major organ involvement and sepsis. We describe the clinical response of a 35-year-old male diagnosed with SLE; then, he developed severe sepsis and a flare of SLE with major organ involvement including lupus nephritis (LN), myocarditis, and neuropsychiatric systemic lupus erythematosus (NPSLE). Based on the patient’s condition, a treatment dilemma was encountered, and after a multidisciplinary meeting, the decision was made to use a combination of rituximab (RTX), intravenous immunoglobulin (IVIG), and pulse steroid. Shortly, the patient’s condition started to improve, and his symptoms were resolved. In conclusion, our clinical case suggests that combined RTX, IVIG, and pulse steroid seem to be effective and safe in achieving clinical response, thus representing a good choice for managing severe SLE flares in sepsis.
18 September 2022 in Advances in Medical Education and Practice
Purpose: Critical care knowledge constitutes an essential component in medicine. Unfortunately, ICU knowledge acquisition is limited in many medical schools. This study is intended to measure the knowledge background, gaps, and the confidence toward critical care concepts among final year medical students and interns at Umm Al-Qura University, Saudi Arabia.Methods: This was a cross-sectional study using a self-administered online questionnaire completed by final-year medical students and interns during January and February 2022. We collected data on demographics, academic year, critical care knowledge, competency, and satisfaction.Results: Two-hundred-twenty-one (221) responses were analyzed. The male gender was 54.3%. A small proportion (15.8%) identified the lowest acceptable oxygen saturation in a normal person. Around three-quarters of the participants did not feel competent about nasal airway suctioning and endotracheal tube suctioning (65.6% and 75.6%, respectively). Only 7.2% of the responder correctly identified the three most useful physiological observations pertinent to quick SOFA criteria. Regarding the training adequacy for identifying critically ill patients, 59.3% answered that they are inadequately trained. Regarding the satisfaction level of exposure to critical care, 54% of the sample responded that they need further improvement. Conclusion: Undergraduate students and interns have limited knowledge and confidence in critical care medicine. Thus, we highly recommend an early institution of a dedicated critical care training module in the medical school curriculum.
Hereditary hemochromatosis (HH) is a multisystem disease characterized by iron overload and various clinical presentations, including cirrhosis, diabetes mellitus, and heart failure. HH can be caused by the human homeostatic iron regulator (HFE) and non-HFE gene mutations. Aplastic anemia is a rare, life-threatening bone marrow failure in which fat replaces pluripotent stem cells, resulting in pancytopenia and hypoplasia of bone marrow. We present a case of a five-year-old-boy who initially presented with a large ecchymosis located at the right side of the chest and abdomen. These started suddenly after minor trauma. Later, he was diagnosed with idiopathic aplastic anemia and treated with immunosuppressive therapy (IST). As part of the workup for pancytopenia, we ordered whole exome sequencing (WES) and diagnosed the patient with autosomal recessive hereditary hemochromatosis (ARHH). The ARHH is caused by HFE pathogenic gene mutation variant (c.187C>G p homozygous genotype). After six months of IST, he still had persistent disease. Human leukocyte antigen (HLA) typing showed he has a sister who is a full match but also has ARHH. Because of this, a haploidentical hematopoietic stem cell transplantation (hHSCT) from the father was performed. The hHSCT had a successful outcome. We suggest that in children with idiopathic aplastic anemia, physicians should be aware of the possibility of co-existing hereditary hemochromatosis or secondary hemochromatosis. Serum ferritin and transferrin saturation should also be measured regularly in order to detect early hemochromatosis.