[Effect of Islet Cell Autoantibodies on clinical course of children with newly diagnosed type 1 diabetes mellitus: a one year follow up]

Background: Type 1 diabetes mellitus [T1DM] is an autoimmune disease identified by the presence of several autoantibodies to islet and/ or /beta-cell antigens, among which is islet cell autoantibodies [ICA]. The positivity of ICA in newly diagnosed patients with T1DM, may predict the course of the disease

Objectives: The purpose of this study was to identify the ICA positive patients with newly diagnosed T1DM and to verify how the ICA positivitycould influence the course of disease during one year follow up as regard clinical improvement[frequency of diabetic ketoacidosis [DKA] and insulin dose requirement] and glycemic ocontrol[glycosylated hemoglobin[HbAlc]level]

Methods: A prospective study was conducted over a period of twoyears at Assiut University Children Hospital; including 34 newly diagnosed T1DM children; 20 females and 14 males; the mean age was 9.14 +/- 3.52 yr. Work up for the patients included complete clinical assessment, CBC, liver andkidney function tests,lipid profile, ICA andHbAlclevels at first presentation. We followed up patients for one year with assessment of episodes of DKA, HbAlclevels quarterly, and the mean of insulin doses were calculated and assessed at the end of the study

Results: Out of 34 newly diagnosed T1DM children; 22 [64.7%] wereICA positive. The mean insulin requirement at the end offollow up was significantly greater in ICA positive group [P<0.04]. Out of 34patients, 13 [38.2%] presented by DKA as a first manifestation of T1DM, whileduring the follow up period there was no significant difference [P>0.05] between both ICA positive andnegative groups. The HbA lc levels were significantly greater in ICA positive group than those with negative results throughout the follow up period [P<0.02].Atthe time of presentation 27.3% and 63.6% of ICA positive cases had total cholesterol andLDL-Cholesterol levels which were significantly greater than IC Anegative cases [P<0.03 and P<0.001] respectively

Conclusion: Follow up of newly diagnosed children with T1DM with positive ICA had poor glycemic control [greater HbAlclevel], during the course of follow up, and higher insulin requirement at the end of follow up

Coagulation defects and platelets function in splenectomized and non-splenectomized beta-thalassemic children

The aim of this work is to evaluate the coagulation defects and platelet function in beta-thalassemic children before and after splenectomy. Also to evaluate the effect of L-carnitine therapy on the coagulation events and platelet function in these patients. The study included 56 beta-thalassemic children who were admitted at the Hematology Unit in the Pediatric University Hospital - Assiut with 20 sex and age matched children as controls. They were subjected to clinical examination and laboratory investigations in the form of: screening tests for coagulation: PT, APTT TT, PC and fibrinogen, natural anticoagulants studies like protein S, protein C and antithrombin III activity and platelet response to adinosindiphosphate [ADP], ristocetin [Rist], collagen and arachidonic acid [AA]. Nineteen children did splenectomy and these studies were done after 2 months of this manover. L-Carnitine therapy in a dose of 3 gm orally daily for 2 months was given to 20 non-splenectomized beta-thalassernic children and the previous follow up laboratorial studies were done. It was found that platelet response to ADP, Rist, collagen and AA was significantly decreased in non-splenoctomized patients and significantly increased in the splenectomized ones in comparison to controls. The coagulation screen tests showed significant defect in the form of prolonged PT, APTT, TT and low PC with decrease fibrinogen levels in both splenectomized and nonsplenecloinized beta-thalassemic children in comparison to controls with no significant difference between before and after splenectomy. Natural anticoagulants except protein S were significantly lower in both non-splenectomized and splenectomized beta-thalassemic children than those of the controls with no difference between before and after splenectomy. L-Carnitine therapy improved the platelet aggregation in the non splenectomized beta-thalasseinic children with no affection on the coagulation tests and natural anticoagulants. In conclusion, thalassemia major can be complicated by defective platelet aggregation and bleeding tendency. Splenectomy causes increased platelet aggregation which with the decreased levels of natural anticoagulants lead to the liability to thromboembolic complications. L-Carnitine might affect and improve platelet aggregation in non-splenectomized children but it could aggravate the thromboembolic complications in the non-splenectomized ones. Iron overload prevention with iron celation and antioxidants is recommended to prevent platelet aggregation and coagulation defects. Salicylates therapy to cases with thromboembolic complications might decrease thrombus formation and dangerous events