Functional hyposplenism is an important and underdiagnosed immunodeficiency condition in children.

AIM: Few studies have focused on paediatric hyposplenism/asplenism, in which splenic phagocytic activity is diminished or absent in an anatomically present spleen. This study aimed to evaluate clinical findings, laboratory tests and prognosis of children with functional hyposplenism/asplenism. METHODS: The study group comprised 74 children who had liver/spleen technetium-99m sulphur colloid scintigraphy from 2002 to 2008. Information collected included demographic features, background diseases, blood smear findings, indications for scintigraphy and outcome. Children with functional hyposplenism were followed until 2012. RESULTS: We found that 34 patients had functional hyposplenism/asplenism. The main indications for scintigraphy in the hyposplenic patients were persistent thrombocytosis and recurrent infections. Associated conditions included immunodeficiencies, autoimmune diseases, malignancies and genetic disorders. Main infections were sinopulmonary infections, bacteraemia and sepsis. The major pathogens were Streptococcus pneumoniae and Haemophilus influenza group A. There was no correlation between the presence of Howell-Jolly bodies in blood smear with clinical disease severity or scintigraphic findings. Repeated scintigraphy showed spontaneous normalisation in 40% of patients. CONCLUSION: Functional hyposplenism is an important and underdiagnosed immunodeficiency condition in children, associated with various clinical conditions including prolonged unexplained thrombocytosis, immune deficiency and autoimmunity. Technetium-99m sulphur colloid scintigraphy is the method of choice for evaluating splenic function.

Anaphylaxis in Israel: experience with 92 hospitalized children.

Little is known about the courses, causes, and clinical features of anaphylaxis in children outside the USA and Europe. Our objective was to evaluate the events of anaphylaxis in children admitted to the Schneider Children's Medical Center of Israel, a major tertiary facility, over a 12-year period. Ninety-two children with anaphylaxis (50 boys, 42 girls) aged 14 days to 18 yr (mean, 7.4 yr) were hospitalized during the study period. The event occurred at home in 52 children (56%), in a medical institution in 24 (26%), outdoors in 13 (15%), at school in 2 (2%), and in an unspecified location in 1 (1%). The main causes were foods (43%), mainly milk and nuts, medications (22%), and hymenoptera venom (11%); in five children, anaphylaxis occurred during general anesthesia, and in 5, the causative agent could not be determined. Food-induced anaphylaxis tended to occur in younger children. Forty-eight children (52%) had a history of atopy (mainly asthma). Hospital treatment consisted of corticosteroids (85%), antihistamines (75%), epinephrine (72%), and ß2 agonists (42%). Seven patients were admitted to intensive care units. There were no fatalities. EpiPen was used by only one of the 16 patients with more than one episode of anaphylaxis, indicating that patient and parent education in the application of the EpiPen needs to be improved.

Ganciclovir treatment in Ménétrier's disease.

UNLABELLED: A 2-y-old girl with severe edema, oliguria and hypoalbuminemia caused by protein-losing gastritis was diagnosed with cytomegalovirus-associated Ménétrier's disease. After almost two weeks, during which the patient required repeated albumin transfusions, she was treated with intravenous ganciclovir. Within five days her condition had improved, and no additional albumin replacement was needed. Complete recovery was observed after several weeks. CONCLUSION: In patients with severe Ménétrier's disease, a course of ganciclovir treatment may be of benefit and should be considered.