Yersinia enterocolitica infection in a patient with sickle cell disease after exposure to chitterlings.

PURPOSE: We describe certain clinical, epidemiologic, and host-susceptibility features of Yersinia enterocolitica infection in the context of a patient with underlying risk factors. PATIENTS AND METHODS: A 10-year-old black girl with sickle cell disease receiving chelation therapy for iron overload resulting from chronic transfusion therapy was admitted with acute abdominal pain and fever. RESULTS: Upon hospital admission, differential diagnoses included enterocolitis, appendicitis, and vasoocclusive crisis. On the 6th hospital day, the patient's stool culture became positive for Y. enterocolitica. Household exposure to raw pork intestines (chitterlings) was the presumed source of the infection. Deferoxamine therapy was withheld, and antibiotic therapy was administered with subsequent clinical improvement. CONCLUSIONS: Y. enterocolitica infection should be considered as a cause of abdominal pain mimicking appendicitis in patients with underlying risk factors (including certain sickle cell patients). History of exposure to raw or undercooked pork products and appropriate cultures should be obtained. Deferoxamine therapy should be withheld in iron-overloaded patients presenting with such symptoms because deferoxamine and iron overload constitute independent risk factors for Yersinia infection. Such patients should be advised to avoid potential exposures to this pathogen.

Dermatitis as a presenting sign of cystic fibrosis.

BACKGROUND: Three percent to 13% of patients with cystic fibrosis present with protein-energy malnutrition that is characterized by hypoproteinemia, edema, and anemia and is associated with high morbidity and mortality. Cutaneous manifestations of malnutrition are rare in patients with cystic fibrosis and have been attributed to deficiencies of protein, zinc, and essential fatty acids. OBSERVATIONS: We describe five patients who presented with failure to thrive, hypoproteinemia, edema, and a cutaneous eruption before the onset of pulmonary symptoms and before the diagnosis of cystic fibrosis was made. The rash had a predilection for the extremities (lower > upper), perineum, and periorificial surfaces. In most cases, erythematous, scaling papules developed by 4 months of age and progressed within 1 to 3 months to extensive, desquamating plaques. Alopecia was variable, and mucous membrane or nail involvement was not observed. The rash was associated with malnutrition and resolved in all survivors within 10 days of providing pancreatic enzyme and nutritional supplementation. The pathogenesis of the rash is unclear, but it appears to stem from deficiencies of zinc, protein, and essential fatty acids and may be mediated by alterations in prostaglandin metabolism. CONCLUSIONS: Cystic fibrosis should be included in the differential diagnosis of the red, scaly infant, particularly when failure to thrive, hypoproteinemia, and edema are also present. Recognition of rash as a sign of cystic fibrosis complicated by protein-energy malnutrition will allow earlier diagnosis and treatment of these patients and may improve their outcome.