Familial thyroxin-binding globulin excess with ichthyosis: A case report

PubMedID: 15214750Thyroxin (T4) binding globulin (TBG) the major thyroid hormone transport protein in humans. Congenital or acquired problems lead to TBG excess. Inheritance of TBG excess follows an X-linked pattern. A 21-month-old boy with ichthyosis was referred to the Pediatric Endocrinology Clinic with high levels of thyroid hormones (TT3=325 ng/dl, TT4 23 µg/dl, FT3=3.49 pg/dl, FT4=1.44 ng/dl, TSH=2.48 µIU/ml). He was clinically euthyroidic. Thyroid gland was normal in size and homogeneous. Thyroid autoantibodies were negative. TSH responded normally to thyroid releasing hormone (TRH) stimulus. TBG was elevated (56 µg/ml). Family investigation revealed high levels of TBG in mother grandfather, and an uncle. To our knowledge, no other TBG excess with ichthyosis has been reported in the literature

Serum IL-1, IL-2, TNF alpha and INF gamma levels of patients with type 1 diabetes mellitus and their siblings

WOS: 000184293800010PubMed ID: 12713258Type 1 diabetes mellitus (DM) develops as a result of autoimmune destruction of the pancreatic beta-cells. The aim of this study was to explore possible associations between serum levels of cytokines, IL-1, IL-2, TNFalpha and INFgamma and metabolic parameters in children with type 1 DM and their non-diabetic siblings to determine whether these cytokines could be indicators of disordered immune regulation. The study population consisted of 41 children with type 1 DM, 32 non-diabetic siblings, and 28 healthy controls. Children with DM were divided into three subgroups: 1) newly diagnosed patients with diabetic ketoacidosis (ND + DKA), 2) newly diagnosed patients without DKA (ND - DKA), and 3) previously diagnosed patients (PD). The highest serum IL-1alpha level was found in the ND - DKA group, which was significant compared to both the ND + DKA (p <0.05) and the siblings (S) (p <0.005). IL-2 levels were similar among all groups. The highest TNFalpha level was observed in the ND + DKA group, which was significant against the ND - DKA (p <0.05), PD (p <0.001), S (p <0.05), and: control (C) (p <0.005) groups. TNFalpha concentration in the PD group was significantly lower than those of S (p <0.005) and C (p <0.001) groups. The ND - DKA group had the highest INFgamma and this was statistically significant when compared with the S (p <0.005) and C (p <0.05) groups. Both the newly diabetics and all diabetics as a group had statistically significantly higher INFgamma levels than both the S (p <0.01 for both) and C (p <0.05 for both) groups. In the diabetics as a whole group, TNFalpha showed correlations with INFgamma (r = 0.370, p <0.05). IL-1 showed correlation with TNFalpha (r = 0.368, p <0.05) INFgamma (r = 0.796, p <0.001) and IL-2 (r = 0.862, p <0.001) in the all diabetics group. IL-2 was correlated with TNFalpha (r = 0.320, p <0.05) and INFalpha (r = 0.754, p <0.01) in the all diabetics group. In conclusion, our results suggest that proinflammatory cytokines TNFalpha, INFgamma, IL1alpha and IL-2 may play important roles alone or in combination in the pathogenesis of type 1 diabetes mellitus

Serum IL-1, IL-2, TNF? and INF? levels of patients with type 1 diabetes mellitus and their siblings

PubMedID: 12713258Type 1 diabetes mellitus (DM) develops as a result of autoimmune destruction of the pancreatic beta-cells. The aim of this study was to explore possible associations between serum levels of cytokines, IL-1, IL-2, TNF? and INF? and metabolic parameters in children with type 1 DM and their non-diabetic siblings to determine whether these cytokines could be indicators of disordered immune regulation. The study population consisted of 41 children with type 1 DM, 32 non-diabetic siblings, and 28 healthy controls. Children with DM were divided into three subgroups: 1) newly diagnosed patients with diabetic ketoacidosis (ND + DKA), 2) newly diagnosed patients without DKA (ND - DKA), and 3) previously diagnosed patients (PD). The highest serum IL-1? level was found in the ND - DKA group, which was significant compared to both the ND + DKA (p <0.05) and the siblings (S) (p <0.005). IL-2 levels were similar among all groups. The highest TNF? level was observed in the ND + DKA group, which was significant against the ND - DKA (p <0.05), PD (p <0.001), S (p <0.05), and control (C) (p <0.005) groups. TNF? concentration in the PD group was significantly lower than those of S (p<0.005) and C (p <0.001) groups. The ND - DKA group had the highest INF? and this was statistically significant when compared with the S (p <0.005) and C (p <0.05) groups. Both the newly diabetics and all diabetics as a group had statistically significantly higher INF? levels than both the S (p <0.01 for both) and C (p <0.05 for both) groups. In the diabetics as a whole group, TNF? showed correlations with INF? (r = 0.370, p <0.05). IL-1 showed correlation with TNF? (r = 0.368, p <0.05) INF? (r = 0.796, p <0.001) and IL-2 (r = 0.862, p <0.001) in the all diabetics group. IL-2 was correlated with TNF? (r = 0.320, p <0.05) and INF? (r = 0.754, p <0.01) in the all diabetics group. In conclusion, our results suggest that proinflammatory cytokines TNF?, INF?, IL-1? and IL-2 may play important roles alone or in combination in the pathogenesis of type 1 diabetes mellitus

Local Use Of Ankaferd Blood Clotter In Emergent Beating Heart Coronary Artery Bypass Grafting

Background: Severe beeding which requiring massive blood transfusion after emergent beating heart surgery is shown to be 1–3%. Therefore, complications and side effects of transfusion can be seen. The aim of this study was to investigate the effectiveness of Ankaferd blood clotter (ABC) as a new topical herbal blood clotter to decrease mediastinal bleeding in emergent beating heart CABG patients who medicated with clopidogrel and acetyl salisilic acite (ASA) prior to CABG surgery. Materials and Methods: 25 CABG patients received a high dose clopidogrel (600 mgr) and 300 mgr ASA have been included into the study (ABC group). 25 patients have also been included into the study for comparison (placebo group, PG). After the administration of protamine sulphate, a 10 ml of ABC solution has been sprayed to the surgical area including mediastinum and epicardial sac. We compared mediastinal drenaige, reoperation due to tamponade, and required blood and blood products in both groups. Results: The mean amount of bleeding after operation was 230 ml in ABC group, and 490 ml in CG (P=0.001). In ICU, bleeding in ABC group and CG was 410ml and 680ml, respectively (P=0.0022). The mean total bleeding from mediastinum was 530±280 mL and 990±440 mL In ABC and CG group, respectively (P=0.001). The amount of autotransfusion was as follows: 175 mL in ABC group, and 290 mL in CG (P=0.002). No patient needed the surgical revision in ABC group, but four patients (16%) from CG group because of cardiac tamponade. Seventeen patients from CG required blood transfusion due to low hematocrite level postoperatively. In CG, the mean hematocrite level was 17±2,3. Transfusion of fresh frozen plasma and platelets in ABC group and CG were as follows: 0.2 and 0 in ABC group, and 0.3and 0.4 in CG. Conclusion: Our study showed that the local use of 10 mL ABC reduces bleeding significantly. Therefore, transfusion requirements of PRBC, platelets, and total blood units in patients on clopidogrel and ASA undergoing emergent beating heart CABG. To provide cardiac tamponade because of excessive mediastinal bleeding and requirement of blood transfusion after emergent CABG patients who previously administered clopidogrel and ASA, we propose local use of ABC solution as a potent coagulant agent.PubMe