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von Willebrand factor (VWF) is a glycoprotein that plays a central role in the initiation of blood coagulation. VWF performs two important functions: it acts as a molecular bridge between platelets and as a carrier for coagulation factor VIII (FVIII). von Willebrand disease (VWD) and acquired von Willebrand syndrome (AVWS) are caused by the absence of and/or abnormality in VWF. The pathophysiology of VWD and AVWS is complex and, therefore, it is difficult to diagnose them by conducting the same laboratory tests in all patients. To develop useful monoclonal antibodies (mAbs) for the diagnosis of VWD and AVWS, rat mAbs against human VWF were generated. Immunoblotting analysis revealed that mAbs recognized the reduced and nonreduced VWF protein and endogenous VWF in normal human plasma. Furthermore, we developed a highly sensitive monoclonal antibody-based sandwich enzyme-linked immunosorbent assay technique. In conclusion, the development of VWF-specific mAbs would be useful in the diagnosis of VWD and AVWS.

An 11-year-old female with a congenitally malformed left hand, sickle-cell trait, asthma, and history of appendicitis was transferred from Zambia for evaluation and treatment of widespread suppurative and ulcerative skin lesions that typically appeared following trauma to her skin. The ulcers first presented 3 years earlier but had markedly worsened in the 9 months prior to transfer, spreading circumferentially on her extremities and abdomen at the site of an appendectomy. They were painful and did not resolve with multiple courses of intravenous (IV) antibiotics and close management by a Pediatric Infectious Disease specialist working for a non-governmental organization (NGO) in her home country. The child had participated annually in health screenings at the NGO and previously had been average weight-for-age. The NGO arranged for international transfer to our pediatric academic medical center. Upon presentation she was severely malnourished with lesions covering approximately 35% of her body. Initial workup found leukocytosis of 21 × 10(3) cells/μL (79% neutrophils), hemoglobin 6.1g/dL, and MCV 66 fl. Iron studies showed iron 18μg/dL, ferritin 55ng/mL, total iron binding capacity 222μg/dL, and transferrin saturation 8%. Inflammatory markers were elevated, CRP 20.1mg/dL, ESR 131mm/hr. A chest CT demonstrated bilateral pulmonary nodules, the largest in her left upper lobe measuring 2.4 × 2.0 × 1.9 cm. Our panel of experts reviews the evaluation and treatment of this patient with extensive suppurative and ulcerative skin lesions, severe malnutrition, hematological abnormalities and pulmonary nodules and the factors considered in offering charity care to international patients. TABLE OF CONTENTS SUMMARY: A previously healthy 11-year-old girl from southern Africa presents with widespread suppurative and ulcerative skin lesions that appear following trauma to her skin.

The association between Salmonella spp. and osteoarticular infections in pediatric patients with major sickle hemoglobinopathies has been well established. However, the contemporary microbiology of these infections in such patients is unknown. We conducted a retrospective review of medical records at Texas Children's Hospital in Houston from 2000 to 2018 to investigate this question. Fifty cases were identified. In 23 (46%) cases, a pathogen was identified. Salmonella was the most common pathogen isolated, accounting for 61% of culture-positive cases followed by Staphylococcus aureus (21.7%).