G1 Persistent hypo-albuminemia in intestinal lymphangiectasia: a multi-therapy approach in a rare condition. A case report

SummaryA 7-month-old boy was referred to the Paediatric Gastroenterology Department with protracted diarrhoea, persistent hypo-albuminemia, electrolyte disbalance and failure to thrive. He had presented 2.5 months earlier with abdominal distention, pedal oedema, and generalized pallor. A mature cystic teratoma was found after a CT of the abdomen, followed by surgical resection. The histology was compatible with the above and the ascitic fluid had elements of a transudate. The initial oncologic investigations identified a low IgG serum level, with normal IgA and IgM, as well as low lymphocytes. Urine protein loss was out ruled. An oesophagus-gastro-duodenoscopy showed whitish mucosa in duodenum. Colonoscopy up to transverse colon was normal. The histology showed normal oesophagus. The stomach had vascular ectasia, mild oedema and congestions within the lamina propria. D1 normal. D2 small areas of the lamina propria appeared to be oedematous within the villous structures and a focal area of haemorrhage was noted. There was no obvious evidence of generalised dilated lacteals. The colonic biopsies were normal. He had a mildly raised faecal alfa-1-antitrypsin with normal faecal elastase. The initial management included Total Parental Nutrition and albumin infusions, followed by subcutaneous octreotide and tranexamic acid to reduce the albumin loss. After the patient’s albumin stabilized and gained weight, a low-fat diet with a MCT high-containing formula were started. At discharged the patient still required SC octreotide to keep normal level of serum albumin, which continued to receive for 12 months. After 1 year of treatment, the SC Octreotide was weaned off gradually until discontinued. The patient kept an adequate level of Albumin, his lymphopenia and hypogammaglobulinemia was resolved, and he thrived accordingly. He has been kept on low fat diet with high MCT formula to date. Accidentally, the patient ingested food with LCT (cross contamination) and developed profuse, auto-limited diarrhoea for 5 days without changes in his biochemistry. It was decided that the patient should remain on low fat diet for the foreseeable future.BackgroundProtein loosing enteropathy in children is a rare condition with major repercussions in nutrition and development. In early stages of life, congenital diarrhoea and cow’s milk allergy are the main causes. Coeliac disease and Cystic Fibrosis could also debut with such features. Intestinal lymphangiectasia, primary or secondary