Significance of Paneth Cells in Histologically Unremarkable Rectal Mucosa.

Paneth cell metaplasia of the rectal epithelium is a common histologic finding in patients with chronic inflammatory bowel disease. However, the clinical significance of isolated Paneth cells in otherwise unremarkable rectal mucosa has not been extensively examined. This study examined the frequency and clinical correlates of rectal Paneth cells in 245 biopsies obtained from patients between the ages of 2 weeks to 20 years in a pediatric tertiary care facility from 2010 to 2011.

Lymphocytic esophagitis in children.

Background: Lymphocytic esophagitis (LE) is a term recently suggested for the finding of >20 intraepithelial lymphocytes/high-power field in an esophageal biopsy with no more than a rare granulocyte. Two prior studies of LE suggested an association of LE with Crohn's disease (CD) in young patients, but there has been no systematic review of a large pediatric cohort to determine the prevalence and clinical associations of LE in children.

Methods: All esophageal biopsies performed at a tertiary care pediatric medical center in 2005 were identified (580 biopsies from 545 unique patients).

Ontogenic changes in lung cholesterol metabolism, lipid content, and histology in mice with Niemann-Pick type C disease.

Niemann-Pick Type C (NPC) disease is caused by a deficiency of either NPC1 or NPC2. Loss of function of either protein results in the progressive accumulation of unesterified cholesterol in every tissue leading to cell death and organ damage. Most literature on NPC disease focuses on neurological and liver manifestations.

Intraventricular rituximab and systemic chemotherapy for treatment of central nervous system post-transplant lymphoproliferative disorder after kidney transplantation.

PTLD of the CNS is a rare complication of solid organ transplantation, and there are only case reports/series available in the literature. Current literature suggests that CNS PTLD carries a worse prognosis than PTLD outside the CNS, and most are of B-cell lineage, predominantly monomorphic, and are associated with EBV infection. Because this disorder is so rare, there is no standard chemotherapy for pediatric patients with CNS PTLD and reported therapies for EBV-associated CNS PTLD are heterogeneous with mixed results.

The significance of small intestinal epithelium in gastric antral biopsies in children.

Intestinal metaplasia of the gastric antrum is common in adults with chronic gastritis and occurs in Helicobacter -associated gastritis in children. This study examined the frequency and clinical correlates of intestinal epithelium in 1690 consecutive antral biopsies obtained from children over a 2-year period in a tertiary pediatric care facility. Intestinal epithelium in gastric glands not associated with overlying villi was present in 22 (1.

Weekly cyclodextrin administration normalizes cholesterol metabolism in nearly every organ of the Niemann-Pick type C1 mouse and markedly prolongs life.

Niemann-Pick type C1 (NPC1) disease arises from a mutation inactivating NPC1 protein that normally moves unesterified cholesterol from the late endosomal/lysosomal complex of cells to the cytosolic compartment for processing. As a result, cholesterol accumulates in every tissue of the body causing liver, lung, and CNS disease. Treatment of the murine model of this disease, the npc1 mouse, s.

A subset of cranial fasciitis is associated with dysregulation of the Wnt/beta-catenin pathway.

Cranial fasciitis, an unusual fibroproliferative lesion that occurs in the scalp of infants, is considered a posttraumatic reactive process similar to nodular fasciitis. Its pathobiology has not been investigated. Over the last 15 years, we diagnosed cranial fasciitis in six children; in one case, the lesion recurred after 4 years.

Unsuspected, disseminated coccidioidomycosis without maternofetal morbidity diagnosed by placental examination: case report and review of the literature.

Historically, untreated disseminated coccidioidomycosis during pregnancy was thought to be associated with 100% maternal fatality and 50% fetal mortality and was the leading cause of maternal deaths in areas of endemicity. As recently as 1995, therapeutic abortions and early deliveries were advocated in certain contexts. This report describes an unrecognized case of disseminated coccidioidomycosis diagnosed at the time of placental examination in a woman who completed her pregnancy without significant maternofetal complications.

Extrahepatic biliary atresia demonstrates abnormal persistence of HES1 protein in neonatal biliary epithelium: an immunohistochemical study.

Extrahepatic biliary atresia (EHBA) is an important cause of conjugated hyperbilirubinemia in neonates. It is a progressive disease with a poor prognosis, requiring early surgical intervention to control morbidity and mortality. The exact pathogenesis of this disorder is not known, although genetic, infectious, toxic, and/or environmental factors are thought to play a role in the causation.

IFN-alpha induces early lethal lupus in preautoimmune (New Zealand Black x New Zealand White) F1 but not in BALB/c mice.

Recent studies indicate that IFN-alpha is involved in pathogenesis of systemic lupus erythematosus. However, direct proof that IFN-alpha is not only necessary, but also sufficient to induce lupus pathogenicity is lacking. In this study, we show that in vivo adenovector-mediated delivery of murine IFN-alpha results in preautoimmune (New Zealand Black (NZB) x New Zealand White (NZW))F(1), but not in normal, mice, in a rapid and severe disease with all characteristics of systemic lupus erythematosus.

Translocation (10;17)(q22;p13): a recurring translocation in clear cell sarcoma of kidney.

A clear cell sarcoma from the kidney of a 12-month-old male child manifested a balanced translocation, t(10;17)(q22;p13). This is the second report of this cytogenetic abnormality in renal clear cell sarcoma.

Spitz nevi and atypical Spitz nevi/tumors: a histologic and immunohistochemical analysis.

A subset of Spitz nevi poses substantial diagnostic difficulty, even among experts, due to its resemblance to malignant melanoma. These lesions are termed atypical Spitz nevi/tumors and there is currently a lack of objective criteria for predicting their biologic behavior. We compared the expression of Ki-67, p21, and fatty acid synthase by immunohistochemistry in 10 atypical Spitz nevi, 28 typical Spitz nevi, 19 compound melanocytic nevi and 18 invasive malignant melanomas.

Multilocular thymic cyst associated with mature mediastinal teratoma: a report of 2 cases.

Acquired thymic cysts are multilocular and show florid xanthomatous and myofibroblastic inflammation. They usually occur in association with mediastinal neoplasms, systemic autoimmune diseases, or trauma. We describe 2 cases (in a 12-year-old girl and an 11-year-old boy) of acquired thymic cysts occurring in association with cystic teratomas, an association that to our knowledge has not been described previously in the literature.

Adenovirus ascending cholangiohepatitis.

Three children, two with liver transplants and one with acquired human immunodeficiency virus (HIV) infection, presented with hepatitis accompanied by elevated gamma glutamyl transpeptidase. Biopsies revealed cholangiohepatitis caused by adenovirus infection. There was a progressive loss of interlobular bile ducts in two of the patients.

Prenatal dexamethasone programs hypertension and renal injury in the rat.

Dexamethasone is frequently administered to the developing fetus to accelerate pulmonary development. The purpose of the present study was to determine if prenatal dexamethasone programmed a progressive increase in blood pressure and renal injury in rats. Pregnant rats were given either vehicle or 2 daily intraperitoneal injections of dexamethasone (0.