172 Severe protein loss due to protein losing enteropathy or severe atopic dermatitis in an exclusively breastfed infant – a case report. (11th October 2021)

• Record Type: Journal Article
• Authors: Mašić, Mario; Hojsak, Iva; Niseteo, Tena; Ivković-Jureković, Irena; Navratil, Marta
• Is Part Of: Archives of disease in childhood. Volume 106(2021)Supplement 2
• Page Start: A73
• Page End: A73
• Publication Date: 2021-10-11
• Subjects: Children -- Diseases -- Periodicals; Infants -- Diseases -- Periodicals; 618.920005
• DOI: 10.1136/archdischild-2021-europaediatrics.172
• Languages: English
• ISSNs: 0003-9888

Abstract

Abstract : Protein loss is often the result of kidney or intestinal disease (protein losing enteropathy) and can cause a number of serious, potentially life-threatening complications such as hypotension, thrombocytosis, electrolyte imbalance and cerebellar ischemia. Recent research suggests an association between extremely severe atopic dermatitis (AD) and allergic enteropathy. Case Report: An exclusively breastfed 6-month-old infant was admitted to the Department of Gastroenterology of our hospital due to failure to thrive, electrolyte imbalance and severe AD (SCORing Atopic Dermatitis; SCORAD 40). On admission, the infant was in poor general condition, dehydrated, malnourished (body weight 4870 g, -3.98 z-score; body length 63 cm, -1.66 z-score), with exudative erythematous morphs scattered throughout the body. Initial laboratory results showed microcytic hypochromic anemia (Hb 110 g/L), hypoalbuminemia (16 g/L), hypogammaglobulinemia (IgG1 2g/L), thrombocytosis (1600×10e9/L), hyponatremia, high values of total immunoglobulin E (IgE) and eosinophilia. Due to the severe presentation and deeply disturbed values of laboratory parameters, extensive gastroenterological, immunological and allergological workup was performed. Polysensitization to a number of nutritional and inhalation allergens has been proven, and exclusive amino acid based formula has been introduced into the diet. During hospital course, patient developed superficial thrombophlebitis and methicillin-resistant Staphylococcus aureus (MRSA) bacteremia, treated with vancomycin, which was replaced with teicoplanin due to severe 'red man' syndrome. Given the initial presentation, hypersensitivity to numerous allergens, unclear primary cause of protein loss (due to enteropathy or severe AD) and burdened family history (father has Crohn's disease), esophagogastroduodenoscopy and ileocolonoscopy with small bowel biopsies were performed. In the small intestine biopsy sample, eosinophilia (> 30 eosinophils per HPF) was found without villous atrophy, and with neat calprotectin levels in stool sample. Due to severe hypogammaglobulinemia, skin infections, and bacteremia, the differential diagnosis included primary immune deficiency (STAT3 deficiency, DOCK8 deficiency, PGM3 deficiency, IPEX ), but all available immunological tests were unremarkable (immunophenotyping of peripheral blood lymphocytes, lymphocyte function test, respiratory burst, thymus ultrasound, and bone marrow morphology). Exclusive amino acid based formula diet was continued in infant, with topical corticosteroids under wet-dressing therapy, and intravenous immunoglobulin replacement therapy every four weeks. With the gradual improvement of the general condition and the regression of skin symptoms, introduction of solid foods started according to the findings of allergy testing. At 17 months of age, the patient gained weight (TM 12.5 kg, 0.61 z); his skin status is improving, although frequent use of topical corticosteroids is necessary. There were no infections, no anemia or thrombocytosis, and albumin and immunoglobulin supplementations were no longer required. Conclusion: The main mechanism of protein loss in infants with extremely severe atopic dermatitis is probably due to damaged skin, and partially to the eosinophilic inflammation of the small intestine. Immunoglobulin loss, potentiated by physiological or transient hypogammaglobulinemia in infants, poses a very high risk for severe, potentially life-threatening infections.