Characteristics and long-term prognosis of the autoimmune hepatitis/primary sclerosing cholangitis overlap syndrome.

Goals/background: Diagnosis, treatment, and prognosis of the overlap syndrome of autoimmune hepatitis (AIH) and primary sclerosing cholangitis (PSC) are controversial. Our aim was to assess the clinical characteristics and long-term prognosis of the AIH/PSC overlap syndrome.

Study: We reviewed the data of 16 patients seen in our center who fulfilled the diagnostic criteria of both diseases at some stage of their medical history.

Serologic markers compared with liver biopsy for monitoring disease activity in autoimmune hepatitis.

Goals/background: Disease activity and response to treatment in autoimmune hepatitis is assessed best by liver biopsy, which does not suit for regular disease monitoring. It is frequent clinical practice to follow disease by assessment of serologic markers. Here, we assessed the diagnostic fidelity of this clinical practice.

Severe exacerbations in children with mild asthma: characterizing a pediatric phenotype.

Background: NHLBI guidelines classify asthma in children as intermittent, mild persistent, moderate persistent, and severe persistent asthma based on baseline symptoms and pulmonary function. However, this may not capture the spectrum of asthma in children, since even mild baseline disease can have significant effects on quality of life. Our objective was to describe a population of children with mild asthma admitted to the ICU with severe exacerbations.

Simplified criteria for the diagnosis of autoimmune hepatitis.

Unlabelled: Diagnosis of autoimmune hepatitis (AIH) may be challenging. However, early diagnosis is important because immunosuppression is life-saving. Diagnostic criteria of the International Autoimmune Hepatitis Group (IAIHG) were complex and purely meant for scientific purposes.

Slow-responders to IV beta2-adrenergic agonist therapy: defining a novel phenotype in pediatric asthma.

Objectives: While aerosolized administration of beta(2)-adrenergic receptor (beta(2)-AR) agonists is the mainstay of treatment for pediatric asthma exacerbations, the efficacy of intravenous (IV) delivery is controversial. Failure to demonstrate improved outcomes with IV beta(2)-AR agonists may be due to phenotypic differences within this patient population. Our hypothesis is that children who respond more slowly to IV beta(2)-AR agonist therapy comprise a distinct phenotype.

Regulatory role of B cells in a murine model of allergic airway disease.

Mice sensitized to OVA and subjected to acute OVA aerosol exposures develop allergic airway disease (AAD). However, chronic continuous Ag exposure results in resolution of AAD and the development of local inhalational tolerance (LIT). Because we have previously observed the persistence of B cells in the bronchoalveolar lavage (BAL) and hilar lymph nodes (HLN) at the resolution stage of this model, we investigated the role of B cells in the modulation of AAD.

Subcutaneous late phase responses are augmented during local inhalational tolerance in a murine asthma model.

Acute exposure of sensitized mice to antigen elicits allergic airway disease (AAD) characterized by Th2 cytokine-dependent pulmonary eosinophilia, methacholine hyperresponsiveness and antigen-specific IgE elevation. However, chronic exposure induces a local inhalational tolerance (LIT), with resolution of the airway responses but persistent systemic IgE production. To further determine if systemic immunologic responses were maintained during LIT, we assessed subcutaneous late phase responses to ovalbumin in this model.

Oral Bromelain Attenuates Inflammation in an Ovalbumin-induced Murine Model of Asthma.

Bromelain, a widely used pineapple extract with cysteine protease activity, has been shown to have immunomodulatory effects in a variety of immune system models. The purpose of the present study was to determine the effects of orally administered bromelain in an ovalbumin (OVA)-induced murine model of acute allergic airway disease (AAD). To establish AAD, female C57BL/6J mice were sensitized with intraperitoneal (i.

Effect of a short course of prednisolone in infants with oxygen-dependent bronchopulmonary dysplasia.

Objective: The purpose of this work was to determine whether oral prednisolone is effective in weaning infants with bronchopulmonary dysplasia, after 36 weeks' postmenstrual age, off supplemental oxygen and to identify factors associated with successful weaning.

Methods: Data were abstracted from a standardized prospectively collected database at the John Dempsey Hospital NICU. Logistic regression and receiver operating curve analyses were used.

Accumulation of regulatory T cells in local draining lymph nodes of the lung correlates with spontaneous resolution of chronic asthma in a murine model.

Background: Mice sensitized to ovalbumin develop allergic airway disease (AAD) with short-term aerosol challenge; however, airway inflammation resolves with long-term aerosol challenge, referred to as local inhalational tolerance (LIT).

Methods: We sought to determine if resolution of airway inflammation correlated with increases in lymphocyte subsets in local lung compartments, including putative regulatory T cells.

Results: At the AAD stage, total numbers of T and B lymphocytes in bronchoalveolar lavage (BAL) were significantly increased above controls; however, at LIT, T and B lymphocytes were significantly reduced compared to AAD.

Maternal transmission of resistance to development of allergic airway disease.

Parental phenotype is known to influence the inheritance of atopic diseases, such as allergic asthma, with a maternal history being a more significant risk factor for progeny than paternal history. We hypothesized that recall Th1- or Th2-type immune responses during pregnancy would result in transfer of maternal factors that would differentially impact development of immune responsiveness in offspring. Following weaning, susceptibility and severity of allergic airway disease (a murine model of human asthma) was evaluated in progeny, disease being elicited by immunization with OVA-Al(OH)(3) and challenge with aerosolized OVA.

Endotracheal intubation and pediatric status asthmaticus: site of original care affects treatment.

Objectives: Status asthmaticus is a common cause of admission to a pediatric intensive care unit (PICU). Children unresponsive to medical therapies may require endotracheal intubation; however, this treatment carries significant risk, and thresholds for intubation vary. Our hypothesis was that children who sought care at community hospitals received less aggressive treatment and more frequent intubation than children who sought care at a children's hospital.

Evaluation of F-actin ELISA for the diagnosis of autoimmune hepatitis.

Objective: Antibodies to F-actin have been proposed to increase specificity in the diagnosis of autoimmune hepatitis (AIH). We compared the diagnostic value of a new F-actin enzyme-linked immunosorbent assay (ELISA) with the current gold standard of detection of smooth muscle antibodies by indirect immunofluorescence (SMA-IFT).

Methods: Archived sera of 47 patients with SMA positive AIH were tested with the F-actin ELISA and SMA-IFT.

Chronic inhaled corticosteroids do not affect the course of acute severe asthma exacerbations in children.

Chronic therapy with inhaled corticosteroids (ICS) suppresses airway inflammation and increases airway responsiveness to beta(2)-adrenergic receptor agonists. We hypothesized that the chronic use of ICS would be associated with shorter duration of hospitalization in severely ill children with status asthmaticus. An 8-year retrospective chart review was conducted of all children admitted to the ICU with status asthmaticus.

Childhood obesity increases duration of therapy during severe asthma exacerbations.

Objectives: Childhood obesity contributes to a wide array of medical conditions, including asthma. There is also increasing evidence in adult patients admitted to the intensive care unit (ICU) that obesity contributes to increased morbidity and to a prolonged length of stay. We hypothesized that obesity is associated with the need for increased duration of therapy in children admitted to the ICU with status asthmaticus.

Multiplex neuritis in a patient with autoimmune hepatitis: a case report.

A 37-year old woman presented with a 9-year history of hepatitis of unknown origin and aminotransferases within a 3-fold upper limit of normal. Autoimmune hepatitis (AIH) was diagnosed on the basis of elevated aminotransferases, soluble liver antigen/liver pancreas (SLA/LP) autoantibodies and characteristic histology. Immunosuppressive therapy led to rapid normalization of aminotransferases.

Interleukin-10 does not mediate inhalational tolerance in a chronic model of ovalbumin-induced allergic airway disease.

Objective: IL-10 is a potent anti-inflammatory cytokine, and IL-10-producing regulatory T cells are effective inhibitors of murine asthmatic responses. This study determined whether IL-10-dependent mechanisms mediated the local inhalational tolerance seen with chronic inhalational exposure to antigen.

Methods: Wildtype and IL-10(-/-) mice were sensitized with ovalbumin (OVA) and then challenged with daily OVA inhalations for 10 days or 6 weeks.

Tolerance induced by chronic inhaled antigen in a murine asthma model is not mediated by endotoxin.

Ovalbumin (OVA)-sensitized wildtype (WT) and endotoxin-resistant (ER) mice developed similar degrees of airways eosinophilia and serum OVA-specific IgE levels after acute aerosolized OVA challenge. WT mice demonstrated methacholine hyperreactivity, whereas ER mice showed no change in responsiveness. With chronic aerosolized OVA challenge, both WT and ER mice developed local tolerance, with resolution of airway eosinophilia but persistence of anti-OVA IgE in serum.

Noninvasive positive pressure ventilation for the treatment of status asthmaticus in children.

Background: Noninvasive positive pressure ventilation (NPPV) has been used safely and effectively to improve gas exchange and to treat respiratory failure in a variety of disease states. Although this technique has some benefits in the treatment of status asthmaticus in adults, the use of NPPV in pediatric patients with asthma has not been described.

Objective: To describe the use of NPPV in the treatment of pediatric status asthmaticus.

Octreotide hardens the pancreas.

Background And Aims: Leakage from pancreaticojejunostomy and development of pancreatic fistulas are the major postoperative complications in patients undergoing duodenopancreatectomy. The risk of developing these complications is higher when surgery is performed on a soft pancreas. A recent report suggests that octreotide hardens the pancreas when given intraoperatively.

Protocol-based titration of intravenous terbutaline decreases length of stay in pediatric status asthmaticus.

Although frequently used to treat status asthmaticus in children, intravenous (IV) terbutaline has not been shown to decrease hospital length of stay (LOS). We theorized that this lack of demonstrable benefit resulted from variations in dosing and titration, rather than the drug per se, and that intensive care unit (ICU) LOS would be shortened by the administration of terbutaline according to a protocol incorporating a quantitative assessment of severity of illness. We prospectively treated 20 consecutive children with status asthmaticus with IV terbutaline according to a protocol that titrated the dose based on a clinical asthma score, the Modified Pulmonary Index Score (MPIS).

Pregnancy in autoimmune hepatitis: outcome and risk factors.

Objective: Autoimmune hepatitis (AIH) may influence pregnancy outcome and pregnancy may affect AIH. We aimed at analyzing the disease course in pregnant AIH patients and at identifying disease-related risk factors for adverse pregnancy outcome.

Patients And Methods: AIH patients with at least one pregnancy were identified at four liver units.

The influence of pulmonary function testing on the management of asthma in children.

Objective: To assess how often in a single encounter that pulmonary function tests (PFTs) influenced management decisions in children with asthma, beyond what was obtained from history and physical examination alone.

Study Design: Children with asthma (n = 367, age 4 to 18 years) performed spirometry before clinical evaluation. Physicians and nurse practitioners in the outpatient pulmonary office evaluated the children and made initial treatment recommendations before reviewing the spirometry results.

Bromelain exerts anti-inflammatory effects in an ovalbumin-induced murine model of allergic airway disease.

Objective: Bromelain, a clinically used pineapple extract and natural product, has reported anti-inflammatory and immunomodulatory activities. The purpose of this study was to determine the effect of bromelain treatment in an ovalbumin (OVA)-induced murine model of allergic airway disease (AAD).

Methods: To establish AAD, mice were sensitized with intraperitoneal (i.