Octreotide in Hennekam syndrome-associated intestinal lymphangiectasia.

A number of disorders have been described to cause protein losing enteropathy (PLE) in children. Primary intestinal lymphangiectasia (PIL) is one mechanism leading to PLE. Few syndromes are associated with PIL; Hennekam syndrome (HS) is one of them. The principal treatment for PIL is a high protein, low fat diet with medium chain triglycerides supplementation. Supportive therapy includes albumin infusion. Few publications have supported the use of octreotide to diminish protein loss and minimize hypoalbuminemia seen in PIL. There are no publications on the treatment of PIL with octreotide in patients with HS. We report two children with HS and PLE in which we used octreotide to decrease intestinal protein loss. In one patient, octreotide increased serum albumin to an acceptable level without further need for albumin infusions. The other patient responded more dramatically with near normal serum albumin levels and cessation of albumin infusions. In achieving a good response to octreotide in both patients, we add to the publications supporting the use of octreotide in PIL and suggest that octreotide should be tried in patients with PIL secondary to HS. To the best of our knowledge, this is the first case report on the use of octreotide in HS-associated PIL.

Angiodysplasia in a Child as a Cause of Lower GI Bleeding: Case Report and Literature Review.

Angiodysplasia or vascular ectasia of the colon is a rare but important cause of recurrent lower gastro-intestinal bleeding in children and should be kept in mind as a diagnostic possibility. This case reports of intermittent rectal bleeding with early presentation caused by angiodysplasia. Therefore, early diagnosis of this rare lesion is important to avoid a possible fatal outcome, and thus the pediatrician should be aware of this lesion as a rare cause of intestinal bleeding in children.

Invasive candidal laryngitis as a manifestation of cyclic neutropenia in an Omani infant.

Cyclic neutropenia is a congenital episodic defect in the development of neutrophils in the bone marrow. It is usually diagnosed late in infancy as it generally takes several cycles of neutropenia before the condition is suspected. These patients often have recurrent mild infectious episodes, but may develop life-threatening bacterial infections; however, they are unlikely to develop fungal infections as the neutropenia is usually self-limiting and of short duration. The authors report the case of an 8-month-old Omani female infant with cyclic neutropenia presenting as severe fungal (Candida) invasive laryngitis, needing life-saving tracheostomy and i.v. antifungal treatment.