PEDIATRIC HEMATOLOGISTAT ISTANBUL: COAGULATION DISORDER DISEASE

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COAGULATION DISORDER DISEASE IN CHILDREN

Turkey Medicals Co-ordinator, “Expert on Hemophilia, a blood disease found in boys, we asked the questions you might be wondering to our Specialist Doctor,” a Pediatric Hematologistat Istanbul Istinye MedicalPark Hospital.

1- What is the origin of the word hemophilia?

Hemophilia disease, consisting of the words hemo= blood, bleeding, -fili= fond, prone, capable, is a coagulation disorder disease that, as its name suggests, is accompanied by bleeding.

2- Is this clotting disorder genetic?

It is the most common genetic clotting disorder in society. It shows a recessive transition due to the X chromosome, which means that only men get sick. Carrier women who do not show symptoms of the disease can pass the disease to their male children. And some of the girls are carriers, and some of them are strong. There is a family history in 80% of cases.

3- How many types of hemophilia are there?

There are types A, B and C of hemophilia. The missing clotting factor in the blood is different in each of them. Hemophilia A is the most common type and clotting factor VIII is missing. Hemophilia B is less common, there is a deficiency of factor IX. Hemophilia C is the rarest and factor XI is missing. The disease is classified according to the degree of deficiency of factors in the blood: severe factor deciency is mentioned if the factor level is below 1%, moderate factor deciency is mentioned if it is between 1-5%, and mild factor deciency is mentioned if it is above 5%.

4- Can you tell us about the symptoms?

According to the degree of the disease, the age and manner of occurrence of symptoms are variable. Usually, the baby does not give symptoms until he moves, falls and bumps start. If there is a severe factor deficiency, heavy bleeding occurs even with the slightest trauma. Fresh bleeding comes from small abrasions for a long time. Crawling, walking and bumps, as well as subcutaneous bleeding and bruising occur. Intra-articular bleeding is very important because it causes damage to the joints. If the factor level is below 1%, spontaneous bleeding into the intracranial, intramuscular and abdominal novae is possible. Until then, bleeding, injury did not happen by chance, bleeding from the penis is also a common picture after circumcision.

5- How is this disease diagnosed?

The amount of substances in the protein structure called coagulation factor in the blood is measured. The amount of factors VIII, IX and/or XI is measured.

6- How is he being treated?

It is possible to give the missing factor intravenously, such as serum. However, since the given factor will decrease after a while, the factor should be repeated. It is especially necessary to give a high amount of factors before the operation. Pediatric hematologist, psychologist, orthopedic and physical therapy specialist should be present during the monitoring of the hemophilia patient in Turkey.

Blood supplementation and prevention of iron deficiency are also essential in bleeding.

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President of Organ Transplant Center at MedicalPark Hospital Antalya

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.
Medically Reviewed by Professor Doctor Alper Demirbaş
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