Amy DeFelice

Presentation of Pediatric Celiac Disease in the United States: Prominent Effect of Breastfeeding

Childhood celiac disease (CD) is considered rare in the United States. Consequently there are few data concerning its clinical presentation. A validated questionnaire was distributed to families of children with CD. One hundred forty-one children with biopsy-proven CD were included in the study. We found significant differences in the clinical spectrum of children based on their infant feeding history. Exclusively breastfed children were significantly less likely to report failure to thrive (69% vs 88%, p<0.05) and short stature (37% vs 62%, p<0.05), and had a higher rate of “atypical” symptoms (p<0.01). Breastfeeding alters the presentation and contributes to atypical presentations of CD and diagnostic delay. Pediatricians need to be aware of the diverse manifestations of celiac disease to reduce diagnostic delay.

Acute Hepatic Failure in a Child Treated With Lamotrigine

An 8-year-old boy with a known seizure disorder that was being treated with lamotrigine developed acute hepatic failure. The patient, who had been well previously, presented with jaundice, elevated liver enzymes, and a coagulopathy. After discontinuation of lamotrigine and aggressive resuscitation, the patient made an uneventful recovery. We believe that the hepatic failure in our patient was secondary to the use of lamotrigine. We recommend careful monitoring of liver function when lamotrigine is administered.

Use of neomycin as the prophylaxis against recurrent cholangitis after Kasai portoenterostomy

Ascending cholangitis after portoenterostomy for biliary atresia often leads to significant morbidity. The long-term prognosis also involves recurrent and frequently recalcitrant bouts of cholangitis. Attempts at prophylaxis with use of oral antibiotics are frequently unsuccessful. The authors describe three patients who, after successful portoenterostomies, were plagued with recurrent bouts of cholangitis, despite the use of a variety of antibiotics. The authors instituted oral neomycin as prophylaxis and nearly eliminated this serious complication.

Eikenella corrodens: A Rare Pathogen in a Polymicrobial Hepatic Abscess in an Adolescent

103/MMI (16.6 x 109/L), hemoglobin 8.0 g/dL (1.24 mmol/L), platelets 236 x 106/mm3 (236 x 109/L), and erythrocyte sedimentation rate 100/mm. Serum chemistry revealed: total bilirubin 1.0 mg/dL (18 pmol/L), albumin 2.8 g/dL (128 g/L), aspartate transaminase 179 U/L (12-38), alanine transaminase 94 U/L (7-41), alkaline phosphatase 269 U/L (33-96), gamma glutamylotranspeptidase 125 U/L (9-58), prothrombin time 14.2 sec and partial thromboplastin time 32.6

Essential fatty acid deficiency mimicking porphyria cutanea tarda in a patient with chronic cholestasis.

Essential fatty acid deficiency was documented in a 3-year-old boy with chronic cholestasis secondary to paucity of intrahepatic bile ducts (Alagilles syndrome). Dietary management had consisted almost exclusively of a proprietary formula with over 80% of the fat as medium-chain triglycerides. The bullous lesions involved mostly sun-exposed areas and were diagnosed initially as being compatible with acquired porphyria cutanea tarda. Improvement followed correction of the fatty acid abnormalities with a polyunsaturated fat supplement administered orally. We postulate that the association of fatty acid deficiency and abnormal vitamin E status contributed to skin damage, possibly involving photosensitizing compounds poorly cleared by the markedly cholestatic liver.

Obstruction of the roux limb after portoenterostomy for biliary atresia: A delayed complication

We report the case of a 5-year-old girl who underwent a Kasai portoenterostomy for extrahepatic biliary atresia. The conduit was exteriorized until 11 months of age. She was doing well, with stable portal hypertension until she suddenly developed jaundice, acholic stools, and bacteremia not responsive to a course of steroids and intravenous antibiotics. Suspecting obstruction at the site of the previously exteriorized anastomosis, a percutaneous cannulation of the conduit was performed. Catheterization of the conduit obstruction unkinked it and reestablished bile flow. She has remained anicteric with stable liver function.

A Comment on “Effect of Tactile Stimulation on Lingual Motor Function in Pediatric Lingual Dysphagia” (Dysphagia 20:311–324, 2005)

Nyla Claire Lamm, EdD, LCSW, Amy Defelice, MD, and Abba Cargan, MD Department of Pediatrics, Division of Gastroenterology and Nutrition, Columbia University College of Physicians and Surgeons; Department of Pediatrics, Division of Neurology, New York Columbia Presbyterian Children s Hospital; Department of Pediatrics, Division of Neurology, Columbia University College of Physicians and Surgeons; and New York Columbia Presbyterian Children s Hospital, CHN7-702, Pediatrics GI/Nutrition, 3959 Broadway, New York, NY, 10032, USA

Pylephlebitis, portal vein thrombosis, hepatic abscess; unusual complications in a teenager with Crohn's disease

Pylephlebitis, portal vein thrombosis and hepatic abscesses are unusual complications of Crohns disease. Our patient, a 17 year old male had been diagnosed with Crohns disease one year prior to the present illness. His clinical course had been uneventful, and his only medication was mesalamine 800 mg PO TID.