1,721,047 research outputs found
Infliximab and pediatric stricturing Crohn’s disease: a possibile alternative to surgery? Experience of seven cases.
No full textAbstract
INTRODUCTION: Infliximab (IFX) is one of the treatments of choice for the different phenotypes of pediatric Crohn's disease (CD). Although it was initially feared that anti-TNFα treatment might cause bowel stenosis, recent studies have validated the efficacy of IFX as an anti-stricturing agent.
AIM: To assess the efficacy of IFX treatment for pediatric stricturing CD.
PATIENTS AND METHODS: Data were obtained on pediatric patients treated at our tertiary level Pediatrics Department (years 2000-2010). Indications for IFX therapy included persistent disease activity (PCDAI > 20) unresponsive to corticosteroids and thiopurines. All patients treated with IFX underwent upper and lower intestinal endoscopy, abdominal ultrasound and magnetic resonance enterography.
CASE SERIES: Among 44 pediatric CD patients, 21 were treated with IFX. Seven of these cases had luminal strictures and in 6 patients the inflammatory strictures disappeared after treatment with IFX. One child with ileal fibrotic stenosis (MR) required a surgical resection.
CONCLUSION: Our data support the efficacy of IFX in pediatric CD, including the stricturing phenotype
The increasing and evolving pediatric IBD: a single centre focus on “old” and “new” patients.
No full textCholic acid therapy for inborn errors of primary bile acids synthesis
No full textInborn errors of primary bile acid synthesis are
rare genetic disorders that cause chronic liver
disease, steatorrhea and fat-soluble vitamins
deficiency in childhood. Absence of itching, normal
γGT and serum bile acids suggest the diagnosis,
confirmed by urinary mass spectrometry
and gene analysis. Oral cholic acid is a safe and
effective therapy for the most common defects
that if untreated may lead to early cirrhosis and
liver failure
Inside and outside the liver: hepatitis-associated aplastic anaemia (haaa) in three paediatric cases.
No full textBackground: Hepatitis-associated aplastic anemia (HAAA) is a
variant of aplastic anemia (AA) in which pancytopenia appears two
to three months after an acute hepatitis [1]. The etiology of this
syndrome is still uncompletely clarified: a role of various hepatitis and
non hepatitis viruses (i.e. CMV, EBV and Parvovirus B19) has been
detected; genetic predisposition and immune-mediated mechanisms
(including imbalance of the T cell immune system and the response
to immunosuppressive therapy) are also considered to have a pivotal
role.
Specific aim:We report three paediatric cases with HAAA who where
successfully treated with haematopoietic stem cell transplantation
(HSCT) or with administration of anti-lymphocyte globulins.
Case serie: (1) A formerly healthy 9 year old boy was admitted to
our Unit of Paediatric Gastroenterology and Hepatology for detection
of elevated transaminases and GGT at blood tests prescribed for
sudden appearance of hecchymoses and petechiae at his limbs,
feet and face. No trauma had occurred. In the previous two
months, asthenia had been referred. Blood tests showed pancytopenia
and repeated transfusions of immunoglobulines and platelets were
required. CRP-DNA for Parvovirus B19 was positive both on blood
and on bone marrow aspirate. Bone marrow aspirate and bone
biopsy confirmed AA. Given the absence of any recovery of the
medullar function within one month of follow-up, a HSCT was
successfully performed thank to the HLA compatibility of one sibling.
(2) A 12 year old boy was evaluated at our Unit for jaundice with
acute onset. A diagnosis of Type 1 Autoimmune Hepatitis (AIH 1)
was based on both serologic profile and liver biopsy. The genetic
screening for thiopurine methyltransferase excluded mutations, so a
treatment with azathioprine was began to withdrawal corticosteroids.
Pancytopenia was detected after two weeks of therapy. The CRPDNA
for Parvovirus B19, initially negative at the time of AIH
1 diagnosis, turned out to be positive on both peripheral blood
Oral Communications / Digestive and Liver Disease 44 Suppl. 4 (2012) S241–S257 S251
and bone marrow. Pancytopenia persisted with a worsening trend
until the appearance of clinical signs (major epistaxis, ecchymoses)
and repeated administrations of immunoglobulins, platelets and
erythrocytes were needed. A bone biopsy evidenced AA. HSCT
was finally required, and the existence of a HLA compatible twin
made it feasible. (3) A 3 year old boy was admitted at our Unit for
acute jaundice and detection of leucopenia, thrombocytopenia and
elevated transaminases. Infusions of platelets and erythrocytes were
required, given a progressive worsening of the bone marrow function.
A bone biopsy evidenced AA. An immunosuppressive treatment
with anti-lymphocyte globulins, cyclosporine, methylprednisolone
was administered, together with G-CSF. No infective causes were
detected. The bone marrow and liver function increased significantly
until a final recovery, so that no bone marrow transplantation was
needed.
Discussion: Diagnosis of HAAA includes clinical manifestations,
blood profiling, viral testing, immune functioning and bone marrow
examination. Patients presenting the features of HAAA are mostly
treated with HSCT from HLA matched donor. Immunosuppressive
therapy has a minor efficacy, as far as it is currently demonstrated.
Table
Pts Clinical onset AST/
ALT
(U/L)
GGT
(U/L)
Parvovirus
B19 DNA
PCR on
blood
and bone
marrow
Ig adm.
and blood
component
transfusions
Anti-lymphocyte
globulins and/or
G-CSF Adm.
Allogeneic
HSCT
Follow-up
since
diagnosis
Current
clinical
and
haemat.
outcome
Pt 1 Hecchymoses
and petechiae
at limbs,
feet and face
Asthenia
1284/
2949
111 Positive Yes No Yes 1 year Good
Pt 2 Jaundice
major
epistaxis
ecchymoses at
limbs
540/
780
94 Positive Yes No Yes 5 months Good
Pt 3 Jaundice 1357/
2322
230 No Yes Yes No 10 years Good
Pt: Patient, Adm.: Administration, Ig: Immunoglobulines, Transpl: Transplantation, Haemat.: Haematological,
HSCT: Haematopoietic Stem Cell Transplantation.
Reference(s)
[1] Rauff B, Idrees M, Shah SA, Butt S, Butt AM, Ali L, Hussain A, Irshad-
Ur-Rehman, Ali M. Hepatitis associated aplastic anemia: a review. Virol
J. 2011 Feb 28;8: 87
Going Beyond Counting First Authors in Author Co-citation Analysis
Full text linkThe present study examines one of the fundamental aspects of author co-citation analysis (ACA) - the way co-citation
counts are defined. Co-citation counting provides the data on which all subsequent statistical analyses and mappings
are based, and we compare ACA results based on two different types of co-citation counting - the traditional type that
only counts the first one among a cited work's authors on the one hand and a non-traditional type that takes into
account the first 5 authors of a cited work on the other hand. Results indicate that the picture produced through this non-traditional author co-citation counting contains more coherent author groups and is therefore considerably clearer. However, this picture represents fewer specialties in the research field being studied than that produced through the traditional first-author co-citation counting when the same number of top-ranked authors is selected and analyzed. Reasons for these effects are discussed
Variations on the Author
Full text link“Variations on the Author” discusses two of Eduardo Coutinho’s recent films (Um Dia na Vida, from 2010, and Últimas Conversas, posthumously released in 2015) and their contribution to the general question of documentary authorship. The director’s filmography is characterized by a consistent yet self-effacing form of authorial self-inscription: Coutinho often features as an interviewer that rather than express opinions propels discourses; an interviewer that is good at listening. This mode of self-inscription characterizes him as an author who is not expressive but who is nonetheless markedly present on the screen. In Um Dia na Vida, however, Coutinho is completely absent form the image, while Últimas Conversas, on the contrary, includes a confessional prologue that moves the director from the margins to the center of his films. This article examines the ways in which these works stand out in the filmography of a director who offers new insights into the notion of cinematic authorship
Appropriate Similarity Measures for Author Cocitation Analysis
Full text linkWe provide a number of new insights into the methodological discussion about author cocitation analysis. We first argue that the use of the Pearson correlation for measuring the similarity between authors’ cocitation profiles is not very satisfactory. We then discuss what kind of similarity measures may be used as an alternative to the Pearson correlation. We consider three similarity measures in particular. One is the well-known cosine. The other two similarity measures have not been used before in the bibliometric literature. Finally, we show by means of an example that our findings have a high practical relevance.information science;Pearson correlation;cosine;similarity measure;author cocitation analysis
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