Context: Congenital bleeding disorders are abnormalities of hemostasis that may present with significant difficulties in diagnosis and management.Aims: This study was done to know various clinical presentations of congenital bleeding disorders and also to note the hematological changes associated with them.
Materials and methods:This was a hospital based study conducted at haematology unit, Department of Pathology, JJM Medical College, Davangere. The present study includes 110 cases referred to our department with history of bleeding disease were evaluated clinically and investigated appropriately to detect the underlying cause for the abnormal hemostatic function.Results: This clinico-hematological study of congenital bleeding disorders showed Glanzmann's thrombasthenia in 4 cases (3.64%). The remaining 106 cases are had diseases affecting clotting factor which included Hemophilia A -70 cases; Hemophilia B -19 cases; Factor VII deficiency -1 case; afibrinogenemia -1 case; Factor XIII deficiency -3 cases; vWD -11 cases and dysfibrinogenemia -1 case. Vascular disorders were not encountered. Majority of these cases presented with hemarthrosis and muscle hematoma. Plasma clotting tests (APTT) were prolonged in majority of cases, corrected with aged serum, adsorbed plasma, F VIII deficient plasma, F IX deficient plasma. Factor assay showed mild, moderate and severe forms.
Conclusion:In spite of various advanced diagnostic investigations, the basic hematological investigation remains first panel or step towards the approach to the diagnosis of congenital bleeding disorders.
: The Bethesda system of thyroid cytopathology (TBSRTC) established a standardized, category –based reporting system for thyroid fine needle aspiration cytology. The objective of this study was to analyse the thyroid cytology smears by TBSRTC, and to determine the distribution of diagnostic categories and subcategories, to analyse cytomophologic features and to correlate cytopathology with histopathology wherever surgery was done. In this study FNA of 248 patients of clinically palpable thyroid and deep-seated lesions were evaluated and categorised according to bethesda system of reporting thyroid cytopathology. 66 patients underwent surgical management in our hospital. Sensitivity, specificity, positive predictive value, negative predictive value and accuracy of FNA reported under bethesda system were obtained by comparing the cytological and histopathological diagnosis where ever possible. The distribution of various categories from 248 evaluated thyroid nodules is as follows: 2.82% ND/UNS, 83.87% Benign, 4.03% AUS/FLUS, 2.41% FN, 0.8% SFM and 6.04% malignant. Sensitivity, specificity, positive predictive value, negative predictive value were calculated. Simplicity, diagnostic accuracy and most of all cost effectiveness of FNA of thyroid has gained wide spread acceptance as first-line diagnostic test in the pre-operative evaluation of thyroid lesions. TBSRTC is an excellent reporting system for thyroid FNA. It also provides clear management guidelines to clinicians.
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