Data were collected of children admitted with ALF to 16 US pediatric liver transplant centers from 2008 to 2013 using the PHIS for a retrospective analysis of PALF trends. Patient data linked to the principal diagnosis code for acute necrosis of the liver (570.00) were analyzed for the following: demographics, regional differences, changes over time, pharmaceutical trends, procedural trends, associated diagnoses, and patient outcomes. In 52.5% of 583 patients who met the selection criteria for PALF, the etiology remained undetermined. Acetaminophen toxicity (18.7%) was the most common identifiable etiology, and hepatic encephalopathy (38.6%) was the most common complication. Mortality was lower than previously reported; 95.4% survived and 73.2% survived without a liver transplant. Acute respiratory failure (OR = 3.4, p = 0.035), acute kidney injury (OR = 3.6, p = 0.003), and cerebral edema (OR = 3.6, p = 0.02) were independently associated with increased risk of mortality. The use of N-acetylcysteine in non-acetaminophen-related ALF, the use of intracranial pressure monitoring, and the proportion of sepsis decreased significantly during the study period. The PHIS database can be a useful tool to study the future trends of PALF patients.

A 6-year-old Hispanic boy with 3 days of hemoptysis presented with hemoglobin 4.5 g/dL. He had 3 months of iron deficiency anemia unresponsive to iron supplementation, with no gastrointestinal symptoms. Chest x-ray demonstrated right lung infiltrates with small pleural effusion (Fig. 1A). Computed tomography of the chest revealed ground glass appearance of the entire right lung and left lower lobe (Fig. 1B). Bronchoalveolar lavage showed 60% hemosiderin-laden macrophages that along with the radiologic findings is diagnostic for idiopathic pulmonary hemosiderosis (1). Severe anemia was disproportionate to hemoptysis. Searching for other causes of anemia, we found elevated tissue transglutaminase (128 U/mL) and positive antiendomysial immunoglobulin A antibody. Esophagogastroduodenoscopy demonstrated edema and minimal scalloping of duodenum. Histology confirmed flattened villi with erosion and increased intraepithelial lymphocytes (Fig. 1C and D), consistent with celiac disease. After initiating a gluten-free diet, pulmonary symptoms resolved and both followup imaging and tissue transglutaminase normalized.The association of celiac disease and idiopathic pulmonary hemosiderosis has been defined as the Lane-Hamilton syndrome. Both the disorders are believed to be immunologically mediated (1,2). Because of the good prognosis with a gluten-free diet, hemoptysis associated with pulmonary hemosiderosis in the setting of disproportionately severe anemia should prompt the evaluation for celiac disease (3-5).

Supplemental Digital Content is Available in the Text.Preclinical experiments in mouse models of visceral pain show marked sex differences in hypersensitivity, behavioral manifestation of pain-related responses, and clinical progression of disease.