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EASY BRUISING CAN BE A HARBINGER OF HEMOPHILIA
Assoc. stating that hemophilia is a chronic disease that should be followed for life, Assoc. Dr. “Hemophilia is a deficiency of factor VIII and factor IX, which act as a clotting protein in the blood. Girls are carriers, boys are sick. A third of the patients are admitted and diagnosed with a complaint of prolonged bleeding after circumcision. Hemophilia should come to mind in patients with easy bruising on the body, intramuscular and intra-articular bleeding, prolonged bleeding in the form of leakage from the place where blood is taken, prolonged menstrual bleeding, intracranial bleeding,” he said.
MedicalPark Hospital Istanbul Pediatric Hematology and Oncology Specialist Assoc. Dr. made important statements within the scope of World Hemophilia Day on April 17. Assoc. Dr. stated that hemophilia is a deficiency of factor VIII and factor IX, which act as a clotting protein in the blood, and said, “There are two types of hemophilia A and hemophilia B. What is missing in hemophilia A is factor VIII. It accounts for 85 percent of all hemophilia patients. In hemophilia B, on the other hand, factor IX deficiency is present and accounts for 15 percent of patients. Hemophilia is a hereditary (congenital) disease. It is inherited as X-linked recessive. In other words, girls are carriers, and boys are sick. On the other hand, mutations acquired later can also cause hemophilia disease. Its frequency in society is 1 in 5 thousand boys for hemophilia A and 1 in 30 thousand boys for hemophilia B,” he said.
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PAY ATTENTION TO PROLONGED BLEEDING AFTER CIRCUMCISION
Assoc. indicating that the clinical findings are similar in hemophilia A and B. Dr., it went like this:
“Joint and intramuscular hemorrhages are the most common findings. A third of the patients are admitted and diagnosed with complaints of prolonged bleeding after circumcision. The disease is classified as severe (factor level < 1 percent), moderate (1-5 percent) and mild (5-40 percent) according to the level of factor level in the blood. Clinical findings, on the other hand, vary depending on the patient’s age and factor level. The findings are more serious in severe hemophilia and occur during the neonatal period and early childhood. Patients present with intra-articular bleeding during the period of crawling and walking. In mild hemophilia, on the other hand, the symptoms appear at a later age, in the form of bleeding after a severe trauma or surgical procedure. The diagnosis of the disease is made by looking at the blood factor level in suspected people. Hemophilia should come to mind in patients with easy bruising, intramuscular and intra-articular bleeding, prolonged bleeding in the form of oozing from the place where blood is taken, unexpected bleeding after circumcision, prolonged menstrual bleeding, intracranial bleeding in muscle. Family history should definitely be questioned when making a diagnosis. Early diagnosis is especially lifesaving in patients with severe hemophilia.”
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PREVENTIVE TREATMENT IS NECESSARY WITHOUT SERIOUS BLEEDING
Assoc. Dr. stated that the basis of the treatment is to replace the missing factors and gave the following information:
“Currently, there are plasma-derived and recombinantly produced factor concentrates available. Preventive treatment should be started in patients with severe hemophilia before serious bleeding develops. Preventive treatment is individualized according to the type of hemophilia, the patient’s weight, bleeding frequency and severity, and factor level. It usually starts as 1 per week and increases to 3 per week. The aim of this treatment is to keep the patient’s blood factor level above 1 percent and prevent serious bleeding. Another form of treatment is factor replacement therapy, which is applied in case the patient has bleeding, which is called ‘bleeding’. Factor concentrates are administered to patients by intravenous infusion. Hemophilia is a chronic disease that should be followed for life. In this process, it is very important to inform the patient and his family and to comply with the treatment. Patients should be followed up by professional specialists from many disciplines such as hematology, physical therapy, orthopedic physician, physiotherapist, psychologist. Patients should carry ID cards containing the name of the disease, the degree of weight, the concentration of factors used, the monitoring center and the information of the physician. The aim of the treatment is to prevent life-threatening, sudden intramuscular, intracranial hemorrhages, as well as to identify and solve muscle chronic problems that patients may face during the transition from infancy to early childhood, school period, adolescence and adulthood in the long term. Recurrent intra-articular bleeding significantly affects the quality of life of patients in the short and long term.”
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IT CAN LEAD TO DISABILITY AND PSYCHOLOGICAL PROBLEMS IN THE LONG TERM
Assoc. who drew attention to the fact that repeated bleeding can lead to disability in the long term. Dr. “Inflammation occurs in the joint as a result of repeated bleeding and causes pain, swelling, movement restriction in the short term; in the long term, it causes loss of joint movement ability, that is, disability. Such disabilities, on the other hand, also cause a decrease in physical activity in patients and osteoporosis, obesity, sociological and psychological problems. Physical activity is very important in every age group and should be supported. Thus, nerve and development are supported and joint range of motion increases. The most recommended sports activities are walking and swimming. Vaccinations of patients in early childhood are carried out in accordance with the vaccination calendar. However, subcutaneous application should be preferred instead of intramuscular application. If intramuscular administration is mandatory, it should coincide with the same day as preventive factor therapy and pressure should be applied to the area where the vaccine was administered after vaccination. For patients during the school period, the school administration and teachers should be informed about the treatment of the disease and emergency situations. The family and the teacher should be in cooperation and the child should be supported psychosocially during this period. Adolescence is the period when compliance with treatment is most impaired. Patients may disrupt their treatment for reasons such as boredom caused by the chronic process, bleeding episodes are not as frequent as before, and the desire to be independent. In recent years, in addition to classical factor applications, long-half-life factors, bisypecific antibody (Emicizumab) and non-factor therapies have been developed that have a similar effect to factor VIII. Emicizumab has not yet received approval for use in our country. Gene therapy studies are ongoing,” he said.
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Turkey's world-renowned organ transplant specialist. Dr. Demirbaş has 104 international publications and 102 national publications.
Physician's Resume:
Born on August 7, 1963 in Çorum, Prof. Dr. Alper Demirbaş has been continuing his work as the President of MedicalPark Antalya Hospital Organ Transplantation Center since 2008.
Prof. who performed the first tissue incompatible kidney transplant in Turkey, the first blood type incompatible kidney transplant, the first kidney-pancreas transplant program and the first cadaveric donor and live donor liver transplant in Antalya. Dr. As of August 2016, Alper Demirbaş has performed 4900 kidney transplants, 500 liver transplants and 95 pancreas transplants.
In addition to being the chairman of 6 national congresses, he has also been an invited speaker at 12 international and 65 national scientific congresses. Dr. Alper Demirbaş was married and the father of 1 girl and 1 boy.
Awards:
Eczacibasi Medical Award of 2002, Akdeniz University Service Award of 2005, Izder Medical Man of the Year Award of 2006, BÖHAK Medical Man of the Year Award of 2007, Sabah Mediterranean Newspaper Scientist of the Year Award of 2007, ANTIKAD Scientist of the Year Award of 2009, Social Ethics Association Award of 2010, Işık University Medical Man of the Year Award of 2015, VTV Antalya's Brand Value Award of 2015.
Certificates:
Doctor of Medicine Degree Hacettepe University Faculty of Medicine Ankara, General Surgeon Ministry of Health Turkey EKFMG (0-477-343-8), University of Miami School of Medicine Member of Multiple Organ Transplant, ASTS Multiorgan Transplant Scholarship. Lecturer at Kyoto University. Lecturer at University of Essen, Research assistant at the University of Cambridge .
Professional Members:
American Society of Transplant Surgeons, American Transplantation Society Nominated, Middle East and Southern Africa Council Transplantation Society 2007, International Liver Transplantation Association, Turkish Transplantation Association, Turkish Society of Surgery, Turkish Hepatobiliary Surgery Association.
Disclaimer:
Our website contents consist of articles approved by our Web and Medical Editorial Board with the contributions of our physicians. Our contents are prepared only for informational purposes for public benefit. Be sure to consult your doctor for diagnosis and treatment.