Adrenocortical status in infants and children with sepsis and septic shock

AbstractBackgroundThe benefit from corticosteroids remains controversial in sepsis and septic shock and the presence of adrenal insufficiency (AI) has been proposed to justify steroid use.AimTo determine adrenal state and its relation with outcome in critical children admitted with sepsis to PICU of Cairo University, Children Hospital.MethodsThirty cases with sepsis and septic shock were studied. Cortisol levels (CL) were estimated at baseline and after high-dose short ACTH stimulation in those patients and in 30 matched controls. Absolute AI was defined as basal CL<7μg/dl and peak CL<18μg/dl. Relative AI was diagnosed if cortisol increment after stimulation is <9μg/dl.ResultsOverall mortality of cases was 50%. The mean CL at baseline in cases was higher than that of controls (51.39μg/dl vs. 12.83μg/dl, p=0.000). The mean CL 60min after ACTH stimulation was higher than that of controls (73.38μg/dl vs. 32.80μg/dl, p=0.000). The median of %rise in cases was lower than that of controls (45.3% vs. 151.7%). There was a positive correlation between basal and post-stimulation cortisol with number of system failure, inotropic support duration, mechanical ventilation days, and CO2 level in blood. There was a negative correlation between basal and post stimulation cortisol with blood pH and HCO3.ConclusionRAI is common with severe sepsis/septic shock. It is associated with more inotropic support and has higher mortality. Studies are warranted to determine whether corticosteroid therapy has a survival benefit in children with RAI and catecholamine resistant septic shock

Fatal acute myocarditis and fulminant hepatic failure in an infant with pandemic human influenza A, H1N1 (2009) virus infection

We report the clinical presentation of a 10 month-old infant who succumbed with acute myocarditis and fulminant hepatic failure associated with a virologically confirmed human influenza A, H1N1 (2009) virus infection. To date, this is the first pediatric patient presenting with this fatal combination of complications during the current H1N1 pandemic. Therefore, we recommend meticulous assessment and follow up of the cardiac status, liver enzymes and coagulation profile in all pediatric patients with severe H1N1 influenza infection

Genetic polymorphism of ACE and the angiotensin II type1 receptor genes in children with chronic kidney disease

Abstract Aim and Methods We investigated the association between polymorphisms of the angiotensin converting enzyme-1 (ACE-1) and angiotensin II type one receptor (AT1RA1166C) genes and the causation of renal disease in 76 advanced chronic kidney disease (CKD) pediatric patients undergoing maintenance hemodialysis (MHD) or conservative treatment (CT). Serum ACE activity and creatine kinase-MB fraction (CK-MB) were measured in all groups. Left ventricular mass index (LVMI) was calculated according to echocardiographic measurements. Seventy healthy controls were also genotyped. Results The differences of D allele and DI genotype of ACE were found significant between MHD group and the controls (p = 0.0001). ACE-activity and LVMI were higher in MHD, while CK-MB was higher in CT patients than in all other groups. The combined genotype DD v/s ID+II comparison validated that DD genotype was a high risk genotype for hypertension .~89% of the DD CKD patients were found hypertensive in comparison to ~ 61% of patients of non DD genotype(p = 0.02). The MHD group showed an increased frequency of the C allele and CC genotype of the AT1RA1166C polymorphism (P = 0.0001). On multiple linear regression analysis, C-allele was independently associated with hypertension (P = 0.04). Conclusion ACE DD and AT1R A/C genotypes implicated possible roles in the hypertensive state and in renal damage among children with ESRD. This result might be useful in planning therapeutic strategies for individual patients.</p

Lack of association of CTLA-4 +49 A/G polymorphism with predisposition to type 1 diabetes in a cohort of Egyptian families

Background: Type 1 diabetes is one of the most common chronic childhood illnesses. Interplay between genetic susceptibility and environmental factors is thought to provide the fundamental element for the disease. Apart from the Major Histocompatibility locus which is the main contributor to risk susceptibility, more than 40 loci are recognized. One among these is the CTLA-4, however data from the literature are controversial. The aim of our study was to investigate the role of CTLA4 49 A/G as a risk susceptibility factor for the development of type 1 diabetes in a cohort of Egyptian families. Subjects and methods: This is a case control study including 88 Egyptian families with one or more index cases (<18 years). The control group comprised 369 healthy unrelated subjects with no family history of diabetes or autoimmune disease. Using PCR-RFLP methodology, CTLA4 49 A/G was analyzed in 738 samples representing 88 families (88 patients, 125 siblings and 156 parents) and 369 control. Results: The age of onset was 6 days-12.5 years with a mean of 5.3 ± 3.6 and a median of 5 years. The mode of presentation was classic symptoms in 51 and diabetic ketoacidosis in 37 cases. Twenty-two cases had a history of viral infection or exanthematous disease and four had associated autoimmune diseases. No significant differences were encountered between the different groups with regard to CTLA4 +49 A/G genotype or allele frequencies. Neither was there a relation between the various genotypes and age of onset or the mode of presentation. Conclusions: CTLA4 49 A/G polymorphism was not recognized as a risk susceptibility factor in our cohort. This may be attributed to the low co-incidence of autoimmune diseases. Up to our best knowledge, this is the first study involving families. We recommend that all studies performed on risk susceptibility to type 1 diabetes should include proper investigation for other autoimmune diseases to exclude their confounding effect on data analysis