Multifocal lymphangioendotheliomatosis with thrombocytopenia is a rare disease characterized by multiple cutaneous and gastrointestinal (GI) vascular lesions and thrombocytopenia refractory to platelet and blood cell transfusions. GI bleeding can become life-threatening in this condition. We report a case of multifocal lymphangioendotheliomatosis with thrombocytopenia in a male infant with isolated GI involvement, diagnosed when he was 3 months old. The patient was managed with daily aminocaproic acid, octreotide drip, and corticosteroids for 13 months after diagnosis; he had complete resolution of symptoms by 2 years of age and showed adequate height and gain by 5 years of age. This case adds to the paucity of data in the literature pertaining to the disease’s phenotypic variability, long-term clinical course, and management of GI bleeding.

OBJECTIVE Erythromycin (ERY) is used in the treatment of gastroparesis; however, this medication is associated with serious side effects, such as cardiac arrhythmias and consequent cardiorespiratory arrest. Azithromycin (AZM) has been suggested as an alternative to ERY as a result of its improved safety profile. Intravenous ERY (1 mg/kg) is administered during antroduodenal motility studies to induce migrating motor complexes (MMCs), the presence or absence of which helps diagnose motility disorders like gastroparesis and gastrointestinal dysmotility. However, there are no pediatric studies comparing the effects of AZM and ERY on antroduodenal pressure profiles. The goal of this study is to determine if AZM is comparable in inducing MMCs in pediatric patients undergoing antroduodenal motility studies. METHODS We performed a retrospective chart analysis of gastric and small bowel manometric data in 2 adolescent patients, both age 15 years, who were given AZM (1 mg/kg) during antroduodenal motility studies. The pressure profiles obtained during motility studies were compared to those of patients of similar age and symptomology who were given the standard intravenous ERY dose during motility studies. We then compared the total duration of effect, mean amplitude of contractions, number of cycles per minute, and duration of highest antral and duodenal contractions. RESULTS Intravenous AZM induces migrating motor contractions in the stomach followed by contractions in the small intestine. The mean amplitude of the stomach contractions was 259 mm Hg in patients who received AZM vs 241 mm Hg in patients who received ERY. The mean amplitude of small intestinal MMCs was 68 mm Hg in patients who received AZI and 72 mm Hg in patients who received ERY. Additionally, the frequency and duration of stomach and small intestinal contractions were also similar in the 2 groups. CONCLUSIONS Intravenous AZM has similar prokinetic effects to intravenous ERY. Our study suggests that AZM is a suitable alternative to ERY in inducing MMCs without the concerning side effects related to ERY and may potentially be used in the management of gastroparesis and other small bowel motility disorders. However, larger prospective studies are required to better understand the long-term efficacy of AZM.

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A well-appearing, 11-year-old female with Down syndrome presented to the emergency department with back pain. The patient denied any history of vomiting or feeding intolerance. A physical examination was unremarkable; the abdomen was soft and nondistended. In addition to laboratory work, a single-view radiograph of the abdomen was obtained for further evaluation. The radiograph showed levoscoliosis, a normal bowel gas pattern, and an incidental finding of a radio-opaque foreign body in the area of the stomach (Fig 1). The patient and family denied previous knowledge of foreign body ingestion. The remainder of the workup done in the emergency department, including urinalysis, complete blood count, complete metabolic panel, amylase, and lipase, was normal. The patient was admitted to the hospitalist service for pain and perioperative management. Given the unknown type and timing of the foreign body ingestion, an esophagogastroduodenoscopy (EGD) was performed the next day. Although the patient was nil per os more than 8 hours before the procedure, the EGD showed a stomach full of food contents, a severely dilated pylorus, and a disintegrated penny in the first part of the duodenum. Question: What is the workup for suspected coin ingestion in children, and what are the recommendations for the removal of coins? Discussion Coins are the most common objects ingested by children in the United States. 1 The age of the child, position of the coin in the esophagus, and size of the coin determine the likelihood of spontaneous passage of the foreign body. Thirty percent of coins are cleared spontaneously. 2,3 Coins that are .23.5 mm in diameter, like American and Canadian quarters, are likely to become impacted whereas 60% of coins in the distal esophagus pass before endoscopic removal. 4,5 If the coin is in the esophagus and the patient shows signs of drooling, dysphagia, and respiratory compromise, then the coin must be removed urgently. A coin in the distal esophagus of an asymptomatic child can be removed within 24 hours. Generally, if the coin is in the gastric region and the patient shows no signs of gastric outlet obstruction, no emergent endoscopy is needed; a radiograph may be repeated 2 weeks after the time of ingestion. Coins in the intestine can be observed clinically unless the patient develops symptoms of obstruction, at which point the coin should be removed surgically. 4

Patients with Hemoglobin Questembert carry a Ser131Pro mutation in one of their alpha globin chains encoded by a TCT-CCT mutation of one of their alpha 2 globin gene. This mutation usually results in an unstable hemoglobin variant with mild anemia that is rarely symptomatic. Rarely these patients may show increased extravascular hemolysis during stress, due to oxidative membrane damage. Our patient is a 7 year old African American girl that came to medical attention due to severe anemia, reticulocytosis, absent haptoglobin levels, persistent jaundice and hyperbilirubinemia. Over a period of several years the patient progressed to develop marked splenomegaly with progressive anemia. Isoelectric focusing in this patient revealed Hb A, A2 and a variant "S" band which was less than 1% and thus not detectable by HPLC. We speculate that in the presence of limited alpha globin synthesis, due to a deletion of one α-2 gene and in the presence of a highly unstable alpha-2 variant, synthesis of wild-type alpha globin from the remaining alpa-1 gene is not sufficient to allow for normal alpha-globin/ beta-globin assembly. Furthermore the assembly of the alpha-globin Questembert into the hemoglobin tetramer is favored resulting in an extravascular hemolytic disorder. Disclosures No relevant conflicts of interest to declare.