Patients with Primary Immunodeficiencies in Pediatric Intensive Care Unit: Outcomes and Mortality-Related Risk Factors

Purposes

The aims of this study were to review the frequency, characteristics, and the clinical course of primary immunodeficiency (PID) patients admitted to pediatric intensive care unit (PICU) and attempt to identify factors related with mortality that might predict a poor outcome.

Methods

We performed a retrospective review of children with PID aged 1 month to 18 years and admitted to PICU from January 2002 to January 2012 in our tertiary teaching children’s hospital.

Results

There were a total of 51 patients accounting for 71 admissions to the PICU. The most common diagnosis was severe combined immunodeficiency. Respiratory problems were the leading cause for admission. A total of 20 patients received hematopoietic stem cell transplantation. Immune reconstitution was achieved in 9 (45 %) patients and eight of them did survive. In all 56 % of all admission episodes resulted in survival. Risk factors for mortality included requirement of mechanical ventilation (P?<?.001), number of organ system failure (P?=?.013), need for renal replacement therapy (P?<?.001), use of inotropes (P?<?.001), higher Pediatric Logistic Organ Dysfunction (PELOD) score (P?=?.005), and length of PICU stay (P?<?.001).

Conclusions

This is the first study regarding the outcome and mortality-related risk factors for PID patients requiring PICU admission. We suggest that PICU management is as important as early diagnosis and treatment for these patients. Prediction of those at risk for poorer outcome might be beneficial for accurate intensive care management and survival.     

Internalized Stigma in Pediatric Psoriasis: A Comparative Multicenter Study

BackgroundInternalized stigma, adoption of negative attitudes and stereotypes of the society regarding persons'' illness, has not been studied previously in pediatric psoriasis patients.ObjectiveWe aimed to investigate the internalized stigma in pediatric psoriasis patients and to determine differences according to factors affecting internalized stigma compared to adult psoriasis patients.MethodsThis multicenter, cross-sectional, comparative study included 125 pediatric (55 female, 70 male; mean age±standard deviation [SD], 14.59±2.87 years) and 1,235 adult psoriasis patients (577 female, 658 male; mean age±SD, 43.3±13.7 years). Psoriasis Internalized Stigma Scale (PISS), Dermatology Life Quality Index (DLQI), Perceived Health Status (PHS), and the General Health Questionnaire (GHQ)-12 were the scales used in the study.ResultsThe mean PISS was 58.48±14.9 in pediatric group. When PISS subscales of groups were compared, the pediatric group had significantly higher stigma resistance (p=0.01) whereas adult group had higher scores of alienation (p=0.01) and stereotype endorsement (p=0.04). There was a strong correlation between mean values of PISS and DLQI (r=0.423, p=0.001). High internalized stigma scores had no relation to either the severity or localization of disease in pediatric group. However, poor PHS (p=0.007) and low-income levels (p=0.03) in both groups, and body mass index (r=0.181, p=0.04) in the pediatric group were related to high PISS scores.ConclusionInternalized stigma in pediatric patients is as high as adults and is related to poor quality of life, general health, and psychological illnesses. Unlike adults, internalized stigma was mainly determined by psoriasis per se, rather than disease severity or involvement of visible body parts, genitalia or folds.     

Immunohistochemical study on distribution of endocrine cells in gastrointestinal tract of flower fish （Pseudophoxinus antalyae）

INTRODUCTION Gastrointestinal hormones are secreted by endocrine cells which are distributed throughout the mucosa of the gastrointestinal tract. They play important roles in the overall regulation of digestive processes such as nutrient absorption, gut m…     

Comparison of Adhesive Properties of Five Different Prosthetic Materials Used in Hernioplasty

This experimental study was designed to assess and to compare intra-abdominal adhesions following the use of five commercially available prosthetic mesh grafts in the repair if abdominal wall defects. Sixty Wistar albino rats were randomly divided into six groups (n = 10). A 2 × 1 cm defect at abdominal wall was created and defects were closed either primarily or with one of the following prosthetic mesh grafts: monofilament polypropylene, polytetrafluoroethylene, sodium hyaluronate/carboxymethylcellulose-coated polypropylene, polypropylene/polyglactin 910 composite, or resorbable hydrophilic collagen-coated multifiber polyester. The severity of adhesions was graded, tensile strengths of adhesions were measured, and histopathological grades of inflammation and fibrosis were evaluated. Polypropylene mesh resulted in more adhesion formation in comparison to primary repair and other grafts used in this study, except polypropylene/polyglactin 910 composite mesh. In addition, the highest tensile strength of omental adhesions was detected in the polypropylene group (χ² = 26.249; p =. 0001). Polyester composite mesh caused the least adhesion formation among the groups. Sodium hyaluronate/carboxymethylcellulose-coated polypropylene and polyester composite meshes revealed the highest fibrosis scores (χ² = 50.776; p =. 0001). The highest inflammatory activity was detected in the polytetrafluoroethylene mesh group (χ² = 16.564; p =. 005). Thus, sodium hyaluronate/carboxymethylcellulose-coated polypropylene and polytetrafluoroethylene meshes following polyester composite mesh were the minimal adhesion-forming grafts in this study. Disadvantages of the polytetrafluoroethylene mesh were lower fibrotic activity and higher inflammatory reaction to the graft.     

Coronary lesion type, location, and characteristics of acute ST elevation myocardial infarction in young adults under 35 years of age

OBJECTIVE: In the present study, we attempted to analyze the coronary artery lesion characteristics of acute ST elevation myocardial infarction (STEMI) in young patients (aged less than 35 years). METHODS: We retrospectively surveyed 25 038 coronary angiography procedures, which were carried out at The Baskent University Adana Hospital from 1998 to present, to discover acute STEMI in young patients. We studied clinical risk factors and angiographic characteristics in 42 consecutive patients who underwent primary coronary angiography for acute STEMI. Control group (n=42) had no history of coronary artery disease and had angiographically proven normal coronary arteries. All patients were under 35 years of age. Angiographic features for STEMI group were collected and both groups were compared for coronary risk factors. RESULTS: Male sex was more prevalent in acute STEMI group when compared with control participants (83 vs. 59%, respectively; P=0.01). A significant difference was found in cigarette smoking (62 vs. 36%, respectively; P=0.007) and family history (33 vs. 16%, respectively; P=0.03) between the two groups. No statistical significance was observed between the groups in terms of hypertension, diabetes mellitus, total cholesterol, low-density lipoprotein cholesterol, and triglyceride levels. Mean high-density lipoprotein cholesterol level was 33+/-8 mg/dl in STEMI group and 39+/-12 mg/dl in control participants (P=0.02). Young patients with acute STEMI showed a preponderance of single-vessel disease (69%) and acute anterior STEMI (60%) owing to occluded left anterior descending artery (P<0.001). CONCLUSION: We observed risk factors such as family history, smoking, and low high-density lipoprotein cholesterol levels in young adults. Acute anterior STEMI owing to occluded left anterior descending artery was more frequent. Coronary atherosclerosis was characterized by higher presence of type B and proximal lesions. The handling selection was percutaneous coronary intervention in more than half of the patients.     

The comparison of the relaxant effects of two methoxylated flavones in rat aortic rings

The vascular effect of salvigenin (6-hydroxyapigenin 6,7,4' -trimethyl ether), a natural flavone, was investigated in comparison with another flavone, 6-hydroxyluteolin 6,7,3',4' -tetramethyl ether in rat aortic rings. Cumulative addition of their increasing concentrations (10(-9)-10(-4)M) produced graded relaxations on rings precontracted with noradrenaline (10(-6) M) and KCl (40 mM). The maximal relaxations induced by flavones were similar, however, based on their pEC50 values salvigenin displayed a higher potency than 6-hydroxyluteolin 6,7,3',4'-tetramethyl ether. Endothelium removal markedly reduced the relaxations to salvigenin while the responses to 6-hydroxyluteolin 6,7,3',4'-tetramethyl ether were partially affected. In addition, a significant decrease was observed in maximal responsiveness and sensitivity to flavones in the presence of L-NOARG, a NO synthase inhibitor. The cyclooxygenase inhibitor indomethacin significantly inhibited the relaxations to salvigenin, but not altered the responses to 6-hydroxyluteolin 6,7,3',4'-tetramethyl ether. Our results provide evidence that salvigenin is an effective flavone in causing vasorelaxation which appears to be mediated by endothelium derived NO and prostacyclin. Whereas, the other flavone, 6-hydroxyluteolin 6,7,3',4'-tetramethyl ether induced relaxant responses are partially endothelium, presumably NO mediated.     

Psychiatric symptoms and health-related quality of life in children and adolescents with psoriasis

Information about the relationship between psoriasis and psychiatric morbidity and quality of life in children and adolescents is limited. We aimed to examine the symptoms of depression and anxiety and health-related quality of life levels in children and adolescents with psoriasis. Forty-eight outpatients with psoriasis aged 8 to 18 years are included in this study. Child Depression Inventory (CDI), State-Trait Anxiety Inventories for Children (STAI-C) and Pediatric Quality of Life Inventory Parent and Child Versions (PedQL-P and C) were applied to both patient and control groups. Psoriasis symptom severity was measured by the Psoriasis Area Severity Index (PASI). Both study and control groups were divided into two age groups, child (8-12 yrs) and adolescent (13-18 yrs), to exclude the effect of puberty on psychological condition. The mean CDI score was higher, and PedQL-C psychosocial and total scores were lower in the children compared with controls. Duration of psoriasis had an increasing effect on physical-health and total scores of PedQL-C in the child group and all PedQL-C scores in the entire sample. Psoriasis severity showed a negative correlation with psychosocial and total scores of PedQL-P in the adolescent group and PedQL-P physical-health scores in the entire sample. Psoriasis is related to depression and impaired quality of life in children. The depressive symptoms in children with psoriasis should not be overlooked and psychiatric assessment of these children should be provided.