Outcome in ulcerative colitis after switch from adalimumab/golimumab to infliximab: A multicenter retrospective study

Background

Anti-TNF therapies infliximab (IFX), adalimumab (ADA), and golimumab (GOL) are approved for treating moderate to severe ulcerative colitis (UC). In UC, only the switch from IFX to ADA has been investigated, reaching no more than 10–43% remission rates at 12 months.

Enteropathy-associated intestinal t-cell lymphoma without celiac disease

Type II enteropathy-associated T-cell lymphoma (EATL) is an uncommon intestinal lymphoma. We report the case of a 73-year-old man with diarrhea and weight loss. Duodenal biopsy showed atrophy and infiltration of irregular lymphocytes. Immunohistochemistry was positive for CD3, CD8, and CD56 with monoclonal TCR rearrangement. The HLA-DQ genotype was DQ5/DQ9. The Epstein-Barr virus RNA test was negative. Before specific chemotherapy could be administered, the patient was admitted to hospital for a respiratory infection and died from a cause unrelated to his lymphoma. The differential diagnosis of CD56-positive lymphoproliferative processes include type II EATL, primary T-cell/natural killer-cell intestinal lymphoma and hepatosplenic T-cell lymphoma. The patient had CD8 y CD56+ markers that allowed type I EATL to be excluded. The HLA-DQ genotype did not correspond to celiac disease and the biopsy showed proliferation of lymphocytes with atypia. The primary intestinal T-cell/natural killer-cell lymphoma was characterized mainly by the absence of CD8 and monoclonal reassortment of the TCR present in this case.     

Fetal iron levels are regulated by maternal and fetal Hfe genotype and dietary iron

Background

Iron metabolism during pregnancy maintains fetal iron levels at the expense of the mother. The mechanism behind this regulation is still not clear despite recent advances. Here we examine the role of maternal and fetal Hfe, its downstream signaling molecule, hepcidin and dietary iron in the regulation of placental iron transfer.

Design and Methods

Hfe wild-type, knockout and heterozygote dams were fed iron deficient (12.5 ppm), adequate (50 ppm) and replete (150 ppm) iron diets and mated with heterozygote males to produce pups of all genotypes. Dams and pups were sacrificed at Day 18 of gestation; serum, placenta, body and liver iron parameters were measured. Protein and mRNA levels of various iron transporter genes were determined in duodenum, liver and placenta by Western blotting and real time PCR.

Results

Maternal liver iron levels were dependent on both dietary iron intake and Hfe genotype. Increasing iron levels in the maternal diet resulted in increased total iron in the fetus, primarily in the liver. However, fetuses of Hfe-knockout mothers showed further elevation of liver iron levels, concomitant with elevated expression of Tfr1, Dmt1 and Fpn in the placenta. Hfe-knockout fetuses that express low levels of liver hepcidin accumulated more iron in their liver than wild-type fetuses due to increased ferroportin levels in the placenta.

Conclusions

Maternal and fetal status, as well as dietary iron, is important in regulating iron transfer across placenta. Maternal Hfe regulates iron transfer by altering gene expression in the placenta. Fetal Hfe is important in regulating placental iron transfer by modulating fetal liver hepcidin expression.     

The role of peer support education model on the quality of life and self-care behaviors of patients with myocardial infarction

ObjectiveThis study aimed to assess the effect of peer education on the quality of life and self-care behaviors of patients with myocardial infarction.MethodsIn this clinical trial, 70 patients with myocardial infarction meeting the inclusion criteria were assigned to experimental (n = 35) or control (n = 35) groups using block randomization. Patients in the intervention group received two one-hour training sessions on the third day after myocardial infarction during the CCU stay along with routine care. Education intervention was performed by peers. The control group will follow routine care. All patients selected were assessed using McNews' quality of life questionnaire and Miller self-care questionnaire, respectively before the intervention and also one month after discharge. Chi-square and t-test were used to analyze the data.ResultsAfter the intervention, the mean of quality of life and the mean score of self-care behaviors in the experimental group were significantly higher compared to the control group.ConclusionsAccording to the results, to improve the quality of life and promote the self-care behaviors in such patients, using peer education along with healthcare professionals is recommended.Practice ImplicationThis patient education approach had a significant impact on quality of life and self-care behavior.     

A unique case of progressive hemifacial microsomia or Parry-Romberg syndrome associated with limb and brain anomalies with normal neurological findings: A review of the literature

In this report, we describe an unusual case of progressive hemifacial atrophy or Parry-Romberg syndrome in a 10-year-old girl with progressive hemifacial microsomia and limb anomalies who had brain magnetic resonance imaging (MRI) findings of white matter hyper-intensities. Patients typically present with neurological manifestations such as epilepsy, facial pain, and migraines and ophthalmological symptoms in conjunction with white matter lesions. The patient demonstrated normal cognition and psychomotor development despite the presence of white matter lesions in her frontal lobe that is commonly associated with neurological symptoms. This report brings attention to the complicated relationship between facial, limb and brain imaging findings in Parry-Romberg syndrome and differentiates it from hemifacial microsomia syndrome.     

The Prevalances and Patient Characteristics of Primary Immunodeficiency Diseases in Turkey—Two Centers Study

Purpose

Primary immunodeficiency diseases (PIDs) are inherited disorders of the immune system resulting in increased susceptibility to unusual infections and predisposition to autoimmunity and malignancies. The European Society for Immunodeficiencies (ESID) has developed an internet-based database for clinical and research data on patients with PID. This study aimed to provide a minimum estimate of the prevalence of each disorder and to determine the clinical characteristics and outcomes of patients with PID in Turkey.

Methods

Clinical features of 1435 patients with primary immunodeficiency disorders are registered in ESID Online Patient Registry by the Pediatric Immunology Departments of the Medical Faculties of Uludag University and Ege University Between 2004 and 2010. These two centers are the major contributors reporting PID patients to ESID database from Turkey.

Results

Predominantly antibody immunodeficiency (73.5 %) was the most common category followed by autoinflammatory disorders (13.3 %), other well defined immunodeficiencies (5.5 %), congenital defects of phagocyte number, function or both 3.5 %), combined T and B cell immunodeficiencies (2 %), defects in innate immunity (1 %), and diseases of immune dysregulation (0.7 %). Patients between 0 and 18 years of age constitued 94 % of total and the mean age was 9.2?±?6 years. The consanguinity rate within the registered patients was 14.3 % (188 of 1130 patients). The prevalance of all PID cases ascertained from the registry was 30.5/100.000. The major cause of the mortality was severe infection which was seen in forty-two of seventy five deceased patients. The highest mortality was observed in patients with severe combined immunodeficiencies and ataxia-telangiectasia.

Conclusion

Promoting the awareness of PID among the medical professionals and the general public is required if premature death and serious morbidity occurs due to late diagnosis of the wider spectrum of PID are to be avoided.     

Hepatitis A Immunity in the District of Aveiro (Portugal): An Eleven-Year Surveillance Study (2002–2012)

Hepatitis A is a common viral liver disease and brings serious health and economic problems as its epidemiologic pattern changes over time. National serosurveys from developed countries have indicated a decline in HAV (hepatitis A virus) seroprevalence over time due to the improvement of economic and sanitation levels. The hepatitis A virus (HAV) immunity rate was surveyed throughout an eleven-year period by sex and age group in Aveiro District. In this retrospective study, blood samples from patients of Aveiro District, in ambulatory regime, collected at the Clinical Analysis Laboratory Avelab between 2002 and 2012 were screened for the presence of antibodies against HAV antigen using a chemiluminescence immunoassay. The global immunity (positive total anti-HAV) was 60% and only 0.3% of the patients presented recent infection by HAV (positive IgM anti-HAV). The HAV immunity was age-dependent (p < 0.05), but no significant differences (p > 0.05) between sexes were observed. The immunity was similar throughout the study period (p > 0.05). The results of this study indicate that young people (especially under 25 years old) from District of Aveiro are susceptible to HAV infection, constituting a high risk group. The elderly should be also a concern in the future of Hepatitis A infection.