Heparin is one of the ultimate weapons for clinical anticoagulation. Since the discovery of heparin in 1916, this miraculous extract has continued to play a major role in the field of human medicine. In particular, the use of heparin is extremely common in cardiac surgery and cardiopulmonary bypass. Unlike drugs such as antibiotics, heparin itself is one of the mucopolysaccharide sulfates that the body naturally secretes to control the rate of blood clotting. Therefore, heparin does not become ineffective due to overuse and drug resistance. If there is a patient who is not sensitive to heparin, then the situation becomes very tricky.

This situation becomes even more troublesome especially when a patient with pulmonary embolism develops heparin insensitivity. Coumarin anticoagulants such as warfarin are too slow to act, and generally clinical practice requires a "pre-start" of anticoagulation with heparin, followed by switching to warfarin to achieve the desired effect. Warfarin alone is obviously not fast enough in the current situation.

As the pinnacle of Chinese medicine, the doctors at Union Medical College Hospital are basically the top figures. Even if you are a doctor of emergency department inpatients, you can make a judgment as soon as you notice an abnormality. Yuan Ping'an almost didn't put much effort into it, and decided to ask the hematology department to have a consultation - Li Songzhao's condition was indeed a little abnormal.

It took only a few minutes for the hematologist to arrive at the emergency department. After getting the patient's coagulation test report and listening to Sun Lien's description, the doctor in the Department of Hematology immediately made a recommendation. "Check his F-XIII clotting factor right away. Let the comrades in the laboratory department work overtime, and this one has to run an ELISA test. ”

Yuan Ping'an frowned and said, "F-XIII?" He suddenly seemed to understand something, and nodded in agreement to the inspection request. After the doctor from the Department of Hematology left, he suddenly asked Sun Lien, "I see that Liuxiangyuan said that Dr. Sun is a good hand at making diagnoses. He pointed to Li Songzhao, who was lying on the bed with a pale face, and asked, "Then this patient, what kind of disease do you think he has?"

Sun Lien has been on duty all day today, and after being pinched for a long time on the plane, he landed and had nowhere to rest, and he went to the Internet café all night and found a serious illness. After this series of things, my brain has long been wooden. He deliberately shirked and said, "There are too many big bulls in Xiehe, so it won't be my turn to make a big deal here." You can judge, the most I can do is to visit. ”

"It seems that Dr. Sun is reluctant to deal with an emergency doctor like me, who is a halfway monk. Yuan Ping'an sprinkled his resentment about missing out on Liu Pingchuan on Sun Lien's head, and even took Ningyuan Medical College to him for hating, "Or is Xiehe no longer important in the eyes of your hospital, and even the nature of the case is not discussed?"

Sun Lien has been training for two months, and he has seen many kinds of people in the emergency room, but this is the first time he has seen such an aggressive peer. He frowned and said, "Doctor Yuan, I'm still just a regular trainee, and I don't have a medical practitioner's qualification certificate, so I can't carry out medical activities here." ”

"It's just a discussion. Yuan Ping'an spread his hands, communication between doctors is a common thing. Even if it looks like he is bullying people, Yuan Ping'an just wants to humiliate the little Guipei in front of him, who was blown as a house in the world. Tell him with practical actions that medical behavior relies on professional knowledge and a large number of case experience, rather than some juggling diagnosis. In his opinion, Sun Lien is just a guy with good luck. The disease diagnosed by Sun Lien is not a particularly rare disease, but the symptoms of the patient are relatively unique.

And Li Songzhao's disease may be a real rare disease. The hematology doctor ordered an ELISA test, which already indicated the direction of the specialist's suspicions. This disease is so rare that if Yuan Ping'an hadn't studied ELISA technology in his graduate school and heard about this detection method in the mouth of the instructor, he wouldn't even know about the existence of this disease.

AHXIII。 The full name is "Autoimmune Haemorrhaphilia Factor X.III.", and no more than 120 cases have been reported worldwide, of which 51 cases have been reported in Japan. So far in China, only Beijing Concord and Shanghai Ruijin have confirmed 5 cases.

Yuan Ping'an almost laughed out loud, I'm afraid that even your instructor has never seen this rare disease! How can you diagnose a disease that you can't even know?

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Sun Lien looked at Li Songzhao lying on the hospital bed, feeling a little anxious. He didn't think that Concord would be unable to diagnose Li Songzhao's condition, and Yuan Ping'an's behavior was naturally causing problems for himself. Sun Lien was anxious that when the symptoms of pulmonary embolism were clear, Yuan Ping'an was still in the mood to give himself a problem, but he did not take any treatment measures.

"If you have a diagnosis, you should treat him first. Sun Lien sighed, "Communication can be done after the patient's condition has stabilized." ”

"He's in a pretty stable condition. ELISA tests can take about three hours to produce results. Until the results are available, other treatments are meaningless. Yuan Ping'an spread his hands indifferently, "But if Dr. Sun can make a diagnosis as soon as possible, maybe we will have a more suitable treatment!"

Sun Lien looked at Yuan Ping'an, and suddenly felt annoyed. "I'm not a doctor to be in the limelight and attract attention!" he looked at Yuan Ping'an and threw out such a sentence coldly. Then put his backpack behind the duty desk, and the brocade flag of "superb medical skills and wonderful hand rejuvenation" was square. Hang his coat on the clothes rack, pick up a white coat and put it on his body, "Since Dr. Yuan wants to communicate, then let's have a good exchange!"

Sun Lien's confidence in daring to argue with the emergency doctors of Xiehe comes partly from the status bar, and the other part comes from the ideal of the profession of doctors. Race with the patient's condition? Now is not the 19th century when the game was about who had the faster amputation!

Putting on a white coat is equivalent to a soldier putting on armor, as if a general is wearing a battle robe. Sun Lien's eyes changed suddenly, and he walked towards Li Songzhao on the hospital bed.

Report. Sun Lien didn't even look at Yuan Ping'an, and directly put his hand under Yuan Ping'an's nose, "If you want to communicate, at least you have to take out the inspection report and the like, right?"

seems to have an arrogant attitude, but Sun Lien actually didn't want to express his dissatisfaction in such a place. He was just simply observing the status bar on Li Songzhao's head.

"Li Songzhao, male, aged, pulmonary embolism, deep vein thrombosis of the lower extremities, stasis ulcer of the left leg, gangrene of the thumb of the left foot, ACT time 0S, positive anti-FX.III.-A antibody. ”

The status bar has changed. And the most worrying part of the change is that the state of high blood sugar disappears. The diagnosis of diabetes mellitus is confirmed by a fasting blood glucose level of more than 7.0 mmol/L and a blood glucose level of more than 11.1 mmol/L two hours after a meal. The disappearance of hyperglycemia indicates that the hyperglycemia content in Li Songzhao's body should only be a transient hyperglycemia.

The gangrene on his foot was not diabetic foot! Sun Lien immediately understood the point. If it wasn't diabetic foot, then the gangrene on his foot was probably a symptom of an undiagnosed condition.

So, what does ACT time 0S mean?

ACT, Activated ClottingTimeofWholeBlood. It is generally used to test the anticoagulation effect of heparin. THE REFERENCE VALUE IS ABOUT 70~130S. After using 8,000 units of heparin, Li Songzhao's ACT time should have reached at least 400S. The data of 0S proves that Yuan Ping'an's diagnosis direction is not wrong, and Li Songzhao is indeed insensitive to heparin.

"Here's the report. was thinking about the direction of diagnosis, and Yuan Ping'an also cooperated with the previous test report in Sun Lien's hands. "Dr. Sun can tell me if you need anything. He smiled a little complacently, "Although our Xiehe is not a big emergency center, the corresponding examination equipment is relatively complete. ”

Sun Lien took the checklist and took a general look. Then I continued to ponder the content of the diagnosis.

Anti-FXIII-A antibody is positive, this indicator is Sun Lien's most clueless. And his intuition told him that this should be the key to the whole diagnosis. But this indicator ...... He hadn't even heard of it once.

"Doctor Sun, do you need any other examination methods?" Yuan Ping'an kept asking beside Sun Lien like a noisy toad, "We can all provide it." ”

Sun Lien didn't hear his proposal at all, but was thinking about another direction.

How exactly does heparin stop blood clotting, and why does Li Songzhao show symptoms of insensitivity to heparin?

Heparin can promote thrombin failure, inhibit platelet aggregation, and stimulate the release of anticoagulant substances and fibrinolytic substances from vascular endothelial cells. The heparin insensitivity shown by Li Songzhao is likely to be a problem with one of these three aspects.

No, if there is a problem with the speed of thrombin failure, without heparin intervention, all the blood in Li Songzhao's blood vessels will turn into blood tofu. It wouldn't be that there was a problem with platelet agglutination, he had a big gangrene on his foot, and if there was a problem with platelet agglutination, he would have died of excessive bleeding when the gangrene had just formed.

If these two directions are ruled out, then the cause can only be the anticoagulant released by the vascular endothelial cells, or the fibrinolytic substance is problematic.

Considering that the ATC data was still extended to 0S under the influence of heparin, the anticoagulant substance should still be secreted, and the fibrinolytic substance ...... Sun Lien nodded lightly, it's you!

It should be that Li Songzhao's blood has insufficient fibrin, or there is a problem with fibrin activation...... No, it's the antibodies that control fibrin that have a problem! Sun Lien suddenly realized that this is the meaning of the anti-FXIII-A antibody positive! FXIII factor is the fibrin stabilization factor in the blood.

Li Songzhao developed an autoimmune disease in his body, which caused antibodies to attack factor FXIII in his blood. Because of the lack of fibrin stabilizing factor, Li Songzhao is insensitive to heparin!8)