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Thrombotic Thrombocytopenic Purpura

Thrombotic Thrombocytopenic Purpura: Thrombocytopenic purpura is a disease in which patient develops small areas of hemorrhage or bleeding on skin or in other body areas due to decreased no. of platelets  which are responsible for blood clotting after an injury or trauma to blood vessels.

 Thrombocytic purpura is of two types
1. Idiopathic, thrombocytopenic purpura.
2. Thrombotic thrombocytopenic purpura.

In idiopathic form cause is unknown but some autoimmune process results in it causation. In thrombotic form, patients have tendency to develop thrombi within the vasculature, which consume the available platelets as a result, over all platelet concentration in blood decreases resulting in bleeding.

Thrombus is tiny mass, clot or plaque within the blood vessel, which consists of platelets. patients with this condition bleed easily after minor trauma, nose and gum bleeds are common, there are bruises on skin, fever, malaise and fatigue are also present, symptoms of organ failure and ischemia depending on organs involved are also present like if brain is involved seizures, paralysis, confused mental state unconsciousness or even coma may develop.

Treatment strategies include exchange blood transfusions, immunosuppressive drugs, transfusions of mega units of platelets and symptomatic treatment according to cause. 

Thrombosis: is a state in which abnormal coagulation of blood occurs inside the blood vessels resulting in their occlusion.

Normally blood remains in uncoagulated state in the body by various, after an injury to blood vessel as in case of trauma, bleeding stops by formation of blood clot or plug which consists of platelets and many other cells, but when this process starts abnormally inside the body it results in formation of thrombus or blood clot with partial or complete occlusion of blood vessel.

Thrombus when dislodged tends to move in circulation and called embolus. Emboli can travel to heart and lung vasculature and cause life threatening conditions by impairing blood flow to these vital organs.

Even sudden death can occur without symptoms, emoli result in tissue ischemia or cessation of blood supply and results in development of an area of tissue damage and tissue death called infarct and process called infarction.

There are three major factors responsible for thrombosis:
1. Hypercoagulability or thrombophillia.
2. Injury to blood vessel as in surgeries or trauma
3. Abnormal blood flow inside the blood vessel called turbulence.

Symptoms depend on the area involved, like in venous thrombosis of leg veins pain, swelling  and  redness over legs  are present, thrombosis of heart vasculature results in heart attacks, renal vein thrombosis results in high blood pressure, emboli in lungs cause dyspnea or shortness of breath, cyanosis of body and even sudden death.

In this page, we are going to discuss about thrombotic thrombocytopenic purpura and types of thrombotic thrombocytopenic purpura, symptoms, diagnosis and treatment in Urdu and Roman Hindi for the people of Pakistan and India.
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Thrombotic Thrombocytopenic Purpura In Hindi
Purpura aik damvi marz hai jis mein khoon aur aruq khoon mein fatoor ronuma hojata hai chunanchay jild kay nichay jariyane khoon kay sabab jisam par qarmazi rang kay dhabay par jatay hain ragon kay sath waly bafaton mein khoon kay risnay ka amal aur is kay bad jild mein sarayat karjata hai aur chotay chotay surkh dagh dhabbay zahir hotay hain chontay zakhmon say bhi khoon rista hai.

Yeh bimari kam hoti hai laikin is mein halat khatarnak hoti hai plateless ki tabahi hai thrombi sito piniea ki shikayat hojati hai is ki alamat mein flodpar pura bikhar dimaghi nuqs aur hemo litic animea hai surkh cells toot photo ka shikar hojatay hain cogolshan screen normal hoti hai TTP pregnancy,manah hamal dawayon ka istemal waram waghairah say bhi hota hai.

Treatment
Ilaj plazma ki tabdili say munsalik hai is mein cyoprecipitate depleted ya solvent detergent treated is istemal hota hai.

Jab plateless ki miqdar 50x10/L say barh jai to mariz ko aspirin ki halki khurak 75 mili gram rozana dain.

Inherited Coagulation Disorders
Coagulation wirasat mein bhi miltay hain aur doosron say bhi lag jatay hain morusi kam hotay hain aur amuman kisi aik factor ki kami hoti hai hasil kardah coagulations lagatar hotay rehtay hain khandati coagulation bleeding bohot kam hoti hai.

Hemophilia-A
Hemophilia-A mein factor viii-c ka level kam hojata hai laikin factor VWF normal hota hai yeh marz 500 mein say aik ko hoti hai aur sirf mardon ki abadi mein hoti hai agar koee mariza beta janay to is ko 50% bimari ka chance hota hai aur aik beti 50% chance bimar hosakti hai hemophilia wali betiyan bap ki taraf say mutasirah hoti hain aur betay normal hotay hain taqriban pachas fisad mutasira khandati nuqs ulta hota hai muntaqli ki sharah bohot unchi hoti hai taqriban 1/3 hissa cancer mutafariq hotay hain in mein haemophiliaee khandani nahin hota.

Clinical Features
Alamat ka inehsar factor viii-c kay level par hai. Bimar thross am halat hai is say jor ki shakl bigar jati hai aur langra pan paida hosakta hai agar munasib ilaj na kiya jai muscles mein bleeding bhi aam hai is liye intra muscular injections say bacha jai.

1 say 5% levels mein shaded bleeding hoti hai yeh zakham ki wajah say hoti hai is terha kay waqiyat aksar hotay hain.

5% say upper kay levels mein mamooli bimari hoti hai aur yeh zakhan ya surgery kay bad hotay hain yeh not karna chahiye kay aisay mariz jin ko haemophiliae hojai.

Agar hemostatis fail hojai to bleeding ziyadah hosakti hai haemophiliae kay mariszon ki amwat cerebral hammer hage kay fail honay ki wajah hoti hain.

Treatment
1.    Mariz mein bleeding kam karnay kay liye factor VIII warid mein dakhil karain.
2.    Jab bleeding shaded hoto factor VIII_C ka level 50% tak barhana chahiye
3.   Bari surgery ki ho to factor VIII-C ko 100% tak barhain aur 50% say upper barqarar rakhain dain takay matlooba level barqarar rakha jasakay laikin surgery kay waqt laga tar diya jata hai factor VIII-C ko gharailu refrigerator mein rakha jai 4 darji centi grade par is say mariz ko jaldi bawaqt zaroorat dainay mein asani hogi.

Ziyadah mutasirah marizon ko prophylaxis haftay mein tin bar bachpan say hi shurooh karnay chahiyan takay jor ko hamaisha kay nuqsan say bachaya ja sakay.

Synthetic vasopressin agar warid mein diya jai to factor VIII-C aur factor VIII ka level barabar hojata hai is say khoon mein paida honewali mushkilat say bacha ja sakta hai yeh mamooli bleeding aur surgery mufid rehta hai laikin heamophiliaee ki soorat mein bay asar hai haemophiliaee kay marizon ko ziyadah nigehdasht kay wards mein rakha jai takay in ki ziyadah hifazat ki ja sakay.

Complications
Taqriban 10% haemophiliae kay marizon mein factor VIII-C ki anti bodies hoti hain yeh inhibitors shaded mutasira marizon mein jin ka factor VIII-C ka pata na ho bohot jald barhtay hain aisay marizon ka ilaj mushkil hojata hai aur factor VIII ki bhari khurak bhi factor VIII-C kay level ko barha nahin sakay gi factor IX concentrate mein activated factor honay ki wajah say doosray factor ko bypass karkay building mein kami kar sakta hai recombinant factor VIIa mein bhi bypass ki khasiyat hoti hai woh bhi in hibitors say mutafiq hojata hai.

Khandani maloomat ikhatti karnay aur coagulation factors kay liye koshishon say females mein carrier status ke tayun karna shurooh ho chukka hai females mein carrier aam tor par factor VIII 50% tak hota hai laikin sahih qimat mukhtalif hoti hai khas kar lionization ki wajah say agar factor VIII-C  ka level 50% ho to carrier ki paimaish aitemad say hosakti hai ya phir factor VWF kay moqay level say kam ho carrier ka janchna molecular jenetic kay istemal kay zariye hosakta hai  yeh ya to factor VIII-C mein jean bilwasta muntaqli say ya polymarfinz DNA ko factor VIII jean mein istemal karnay say antenatol ki pehchan chorionic villus say hasil kardah fatal tissue ka tajziya karnay say hosakti hai.

Hemophilia B
Haemophiliae B factor IX ki kami say hoti hai is ki alamat wohi hain jo haemophiliae ki hain laikin is ki sharah 30000 admiyon mein say aik hai is ka jean 34 kilo bases par chota hota hai. aur factor ki adhi zindagi 18 ghantay hoti hai haemophiliaee B ka ilaj factor IX ki concentration say hota hai.

Von Will brand's Disease
Is bimari mein plateless ki function naqis hoti hai factor VIII-C ki kami hoti hai yeh donon factor VWF mein kami ya fatur ki wajah say hai VWF factor VIII-C ko p lazma mein barqarar rakhnay key liye aham kirdar ada karta hai VWF jean kromosom 12 par waqay hoti hai aur is ki latadad muntaqli imtiaz kiya gaya hai VWD ki tin halatain hoti hain.

Type 1 mein mamooli kami hoti hai aur morusi hoti hai.
Type 2 high molecular wazan mein kami.
Type 3 mukamal tor par khandani hoti hai aur marizon mein VWD aur factor VIII-C ka level maloom kiya jata hai.

Clinical Features
Yeh mukhtalif hoti hain type 1 aur 2 kay marizon mein mamooli alamat nazar ati hain mamooli surgery aur phoray say bleeding hoti hai hamar thruses nahin hotay.

Type 3 kay marizon mein bleeding shaded hoti hai yeh leeding jor aur muscles mein daikhay mein aaee hai.

Treatment
Ilaj ka inehsar mariz kay marz ki shidat par hai aur yeh mamooli heamophilia kay ilaj ki terha hai bleeding ko roknay kay liye factor VIII ka vonwillebrand factor kay concentratrate istemal karain yeh surgery kay moqay par type 3 mein istemal karain.

Disseminated Intravascular Coagulation
Khoon munjamad karnay walay madon kay choray ki wajah say khoon ki shariyanon mein bohot ziyadah fibes phailay hotay hain is ki wajah endotheliat ko nuqsan pohonchnay ki wajah say ya platelet ki ziyadti hoti hai.

Safaid khoon kay zarat ki tamil khas kar monosites jo tissue factor ko phailatay hain aur cytokines ka chorna DIC kay barhanay mein ahsam kirdar ada kartay hain.plateless ki khapat istemal aur coagulation factors aur fibers ki tehlil  fiber ki degredation  paidawar kartay hain jo fibran ki poly merazilation karkay coagulation nuqa ka bais banti hai in tabdiliyon ka natija ibtedaee thrombosis kay majmuay ka bais banti hain aur bleeding paida karti hain.

Causes Of Dic
Is ka asbab mandaja zain hain. Mariz ko musbat bimari hosakti hai septiceamia aur trauma hosakta hai mariz ko hemalatic dakhil karnay kay radde amal aur sanp kay katnay ki wajah say yeh marz hosakti hai paidaishi bimariyon ki wajah hosakti hai malaria aur jigar ki bimari bhi is ka sabab banti hai.

Clinical Features
Fatoor zahir nazar asakta hai mariz ka aksar bimar hota hai mariz ko bleeding bhi nahin hoti aur bohot ziyadah hambrage bhi hota hai khoon mun say ,nak say aur zakhmi shariyanon say nikal sakta hai aur til kay dhabbai ho bakasrat phailay huway hotay hain.

Diagnosis
Mariz ki zahiri halat say tashkhis hosakti hai. Bleeding ki film surkh cells bikhray huway nazar aain gay. Mariz ko shaded qisam ka thrumbositopenia hoga.

Treatment
Mariz ki zahiri bimari ka ilaj karna chahiye aisay mariz jin ki bleeding nahin hoti. Khoon kay hajam tissue kar barqarar asli satah par rakhna zaroori hai. Jin marizon ko bleeding horahi ho in ko pallets ka dakhil karna aur surkh cells dakhil karna behtar sabit hota hai heparin intra vescular coagulation ko roknay kay liye istemal ki jai fiber nolisis kay in say hibitors istemal na kiye jain. Makhsoos marizon kay liye antithrombin aur protein C istemal ki jasakti hain.
 
Thrombotic Thrombocytopenic Purpura In Urdu

Thrombotic Thrombocytopenic Purpura In Urdu  

 
Thrombotic Thrombocytopenic Purpura In Pakistan And India
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Page Created: 10-Mar-12 07:24 Last Update: 10-Mar-12 07:44
 
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