2 Basic Information Hemophilia A Clinical Pathway (version 20 19) The Notice of the General Office of the National Health Committee on Printing and Distributing the Clinical Pathway of Related Diseases (version 20 19) was issued by the General Office of the National Health Committee on February 29th, 20 19 (version 2019)
Notice of the General Office of the National Health and Wellness Committee on Printing and Distributing the Clinical Pathway of Diseases (20 19 Edition)
National Health Office Medical Letter [2019] No.933
Health and Health Committees of all provinces, autonomous regions, municipalities directly under the Central Government and Xinjiang Production and Construction Corps:
In order to further promote clinical pathway management, standardize clinical diagnosis and treatment behavior, and ensure medical quality and safety, our Committee revised the clinical pathway of 19, and formed 224 clinical pathways of diseases (version 20 19). It is hereby printed and distributed to you (which can be consulted in the column of medical administration on the website of the National Health and Wellness Committee) for the reference of health administrative departments at all levels and various medical institutions at all levels.
General Office of National Health and Wellness Committee
20 19 12.29
4 Clinical Paths Full text Hemophilia A Clinical Paths (version 20 19)
4. 1 1. Hemophilia A Clinical Pathway Standard Hospitalization Process 4. 1. 1 (1) The applicable object is hemophilia A (ICD 10: D66.x0 1).
4. 1.2 (II) The diagnosis basis is based on the Standard for Diagnosis and Treatment of Hematological Diseases (edited by Shen Lian and Zhao Yongqiang, Science Press 20 18, 4th edition) and Expert Consensus on Diagnosis and Treatment of Hemophilia in China (20 17 edition) [Thrombosis and Hemostasis Group, Hematology Branch, Chinese Medical Association] 38(05): 364370), Hemophilia (edited by Yang Renchi and Wang Hongli, Shanghai Science and Technology Press, 20 17, 2nd edition).
1. Almost all patients are male (female patients are homozygous, which is extremely rare), with or without family history, and those with family history conform to the X-linked recessive inheritance law.
2. Hemorrhage of joints and deep muscle tissue, delayed bleeding after trauma or operation are its characteristics, but spontaneous bleeding can also occur. Repeated bleeding shows joint deformity and pseudotumor.
3. Laboratory inspection
(1) The prothrombin time (PT), thrombin time (TT) and fibrinogen are normal, and the activated partial thromboplastin time (APTT) is prolonged, which can be corrected by normal fresh plasma and adsorbed plasma, but not by serum. Platelet count is normal.
(2) The activity of coagulation factor VIII (F VIII: C) decreased, with F VIII: C > 5% ~ 40% as light, 1% ~ 5% as medium and < 1% as heavy.
(3) vWF antigen is normal.
4. 1.3 (3) The choice of treatment scheme is based on hemophilia (edited by Yang Renchi and Wang Hongli, Shanghai Science and Technology Publishing House, 2nd edition), Expert Consensus on Diagnosis and Treatment of Hemophilia in China (2017th edition) [edited by Thrombosis and Hemostasis Group of Hematology Branch of Chinese Medical Association, China Hemophilia Collaboration Group]. 38(05): 364370] and Standard for Diagnosis and Treatment of Hematological Diseases (edited by Wang Jianxiang, Peking Union Medical College Press, 20 14).
1. Local hemostasis measures and precautions: including braking, local pressure dressing and placing ice packs, local application of hemostatic powder, thrombin or gelatin sponge, etc. Oral bleeding can contain tranexamic acid or 6 aminocaproic acid. Avoid intramuscular injection, trauma and surgery. If you need surgery, you need enough coagulation factor replacement therapy. Aspirin or other non-steroidal anti-inflammatory drugs and all drugs that may affect platelet aggregation are prohibited.
2. Alternative therapy
(1) F Ⅷ preparation: the first choice is gene recombinant F Ⅷ preparation or virus inactivated blood-borne F Ⅷ preparation. The half-life of F Ⅷ is 8 ~ 12 hours, and it is often required to be infused 2 ~ 3 times a day (in the case of excessive factor consumption, such as major surgery, it needs to be repeated 2 ~ 4 hours after the first infusion and 8 ~ 12 hours later).
(2) Cold precipitation: it contains coagulation factors such as F Ⅷ and fibrinogen, and F Ⅷ is 5 ~ 10 times higher than fresh plasma, which is used for unconditional use of F Ⅷ preparation.
(3) Fresh frozen plasma: contains all plasma proteins such as coagulation factors, and is only used for unconditional use of F Ⅷ preparation and cold precipitation.
3. Deamino -D- arginine vasopressin: For mild and partially moderate patients, it is suggested that before DDAVP infusion test, that is, after DDAVP 12h, F ⅷ is 2-3 times higher than the basic value, and it is considered effective if it is greater than 0.3IU/dl.
4. Low-dose adrenocortical hormone: it can improve capillary permeability, control hematuria and accelerate the absorption of acute hemoptysis, and can be used in combination with alternative therapy in the short term.
5. Anti-fibrinolytic drugs: 6- aminocaproic acid or tranexamic acid are commonly used, and those with gross hematuria are prohibited to avoid being used together with prothrombin complex.
4. 1.4 (4) The standard length of stay is 1.5 (5) The standard entry route is 1. The first diagnosis must conform to ICD 10: D66.x0 1 hemophilia A disease code.
2. Acute bleeding of joints, muscles, soft tissues or internal organs.
3. When the patient has other disease diagnoses at the same time, but does not need special treatment during hospitalization, and does not affect the implementation of the first-visit clinical pathway process, he can enter the pathway.
4. 1.6 (6) Items to be checked during hospitalization 1. Mandatory test items
(1) Blood routine, urine routine, stool routine+occult blood.
(2) Liver and kidney function, electrolyte, pre-transfusion examination, blood type, coagulation function and APTT calibration test.
(3)Fⅷ∶C .
(4) Detection of vWF ∶ Ag, VWF activity and F ⅸ ∶ C (previously undiagnosed).
2. According to the patient's condition, you can choose the examination items.
Determination of the titer of (1) f Ⅷ inhibitor.
(2)X-ray chest film, electrocardiogram, hematoma site, organ ultrasound, joint X-ray plain film or MRI, skull CT, etc.
4. 1.7 (7) Start time of treatment before admission. Patients diagnosed with hemophilia A will start treatment immediately after admission.
4. 1.8 (8) Treatment scheme and drug selection When hemophilia is in acute bleeding, F Ⅷ preparation should be infused immediately for alternative treatment to reduce the degree of functional damage of joints, tissues and organs.
The dosage of F Ⅷ preparation can be calculated according to the following formula: total amount of F Ⅷ ∶ C (required F Ⅷ ∶ C level%-current plasma F Ⅷ ∶ C level %)×0.5× patient's weight (kg).
The half-life of f Ⅷ is 8 ~ 12 hours. To keep F Ⅷ in the blood at a certain level, it is necessary to infuse/kloc-0 every 8 ~ 12 hours, preferably within 2 hours. See table 1 for specific alternative treatment schemes.
Table 1 Alternative Treatment Scheme
Bleeding site
Expected factor level (%)
F Ⅷ dose (international unit/kg body weight)
Course of treatment (days)
common
40~60
20~30
1~2
muscle
40~60
20~30
2~3
gastrointestinal tract
give rise to
80~ 100
40~50
7~ 14
maintain
50
25
Oral mucosa
30~50
15~25
Until the bleeding stops.
nosebleed
30~50
15~25
Until the bleeding stops.
haematuria
30~ 100
15~50
Until the bleeding stops.
Central nervous system
give rise to
80~ 100
40~50
1~7
maintain
50
25
8~2 1
retroperitoneal
50~ 100
25~50
7~ 10
Injury or surgery
50~ 100
25~50
Until the bleeding stops.
4. 1.9 (9) The standard bleeding symptoms after discharge improved or subsided.
4. 1. 10 (10) variation and cause analysis. Those who have the following conditions at the time of initial diagnosis or during diagnosis and treatment will exit this path:
1.f Ⅷ inhibitor was positive.
2. The patient is complicated with infection.
3. Life-threatening bleeding of important organs, such as pharyngeal bleeding, retroperitoneal bleeding, central nervous system bleeding, etc. , leading to confusion, decreased blood pressure and blood oxygen and other unstable vital signs.
4. Bleeding needs surgery.
4.2. Hemophilia A Clinical Pathway Table Applicant: Hemophilia A (ICD11:D66.X 01) was the first diagnosis.
Patient name:? Gender:? Age: outpatient number. : hospitalization number:
Hospitalization date: discharge date: standard hospitalization date: 10 day.
time
1 day hospitalization
The second day of hospitalization
owner
ask
examination
treat cordially
worker
work
□ Ask about medical history and physical examination.
□ Complete medical record writing.
□ Open the laboratory list.
-Preliminary diagnosis is determined by laboratory examination.
□ Symptom support therapy
-Inform the patient's condition, if necessary, inform the patient's family members that the condition is serious or critical, and sign the notice of serious or critical condition.
□ The patient's family signed the informed consent form for blood transfusion.
□ Senior physician rounds
□ Continue to complete the entrance examination.
□ Continue symptomatic support treatment.
□ Complete necessary consultations with relevant departments.
-to complete the superior doctor rounds and other medical records writing.
-explain the illness and matters needing attention to patients and their families.
heavy
main points
doctor
order
Long-term medical advice
□ Routine nursing care of hematological diseases
□ Primary health care
□ diet
-according to the condition of serious illness or critical notice.
□ Other doctor's advice
Temporary order
-Blood routine and classification, urine routine, stool routine+occult blood
□ Liver and kidney function, electrolyte, coagulation function, APTT calibration test, blood type, pre-transfusion examination, determination of Fⅷ:C and vWF:Ag, and determination of Fⅷ inhibitor titer (if possible).
X-ray chest film, electrocardiogram, hematoma or organ B-ultrasound, joint plain film, skull CT, MRI, etc.
□ transfusion of attention group F VIII or blood-borne F VIII preparation.
□ Cold precipitation
□ Fresh frozen plasma
□ adrenocortical hormone
□ Anti-fibrinolytic drugs
□ local hemostasis treatment
□ deamino -D- arginine vasopressin
□ Other doctor's advice
Long-term medical advice
□ Patients' previous basic drug treatment
□ Other doctor's advice
Temporary order
□ Coagulation analysis
□ transfusion of attention group F VIII or blood-borne F VIII preparation.
□ Cold precipitation
□ Fresh frozen plasma
□ deamino -D- arginine vasopressin
□ adrenocortical hormone
□ Anti-fibrinolytic drugs
□ local hemostasis treatment
□ Other doctor's advice
Main nursing care
work
-introduce ward environment, facilities and equipment.
□ Admission nursing evaluation
□ Education
□? Observe the changes of the patient's condition.
Disease variation
record
□ None □ Yes, reason:
1.
2.
□ None □ Yes, reason:
1.
2.
nurse
symbol
Certified doctor
symbol
time
Stay in hospital for 3 ~ 9 days
Hospitalization 10 days
(discharge date)
owner
ask
examination
treat cordially
worker
work
□ Senior physician rounds
□ Re-check the coagulation function and F Ⅷ: C.
□ Observe the changes of bleeding.
-Make differential diagnosis and definite diagnosis according to the results of physical examination, auxiliary examination and previous data.
□ Make differential diagnosis according to other examination results, and judge whether there are other diseases.
□ Start treatment.
□ Protect the function of important organs
-pay attention to the adverse reactions of blood products, symptomatic treatment.
□ Complete the course record.
-The superior doctors make rounds, evaluate, determine whether there are complications, and decide whether to leave the hospital.
□ Complete discharge records, home pages of medical records, discharge certificates, etc.
-explain to patients the matters needing attention after discharge, such as the time and place of returning to the hospital for follow-up, and the handling in case of emergency.
heavy
main points
doctor
order
Long-term medical advice (start treatment immediately after diagnosis)
□ transfusion of attention group F VIII or blood-borne F VIII preparation.
□ Cold precipitation
□ Fresh frozen plasma
□ deamino -D- arginine vasopressin
□ adrenocortical hormone
□ Anti-fibrinolytic drugs
□ Local hemostasis treatment and nursing care
□ Other doctor's advice
Temporary order
□ Review blood routine.
□ recheck blood biochemistry, coagulation function and F Ⅷ: C level.
□ Symptomatic support
□ Other doctor's advice
Unloading order
□ Take medicine after discharge
-Regular outpatient follow-up.
□ Monitor coagulation function.
Main nursing care
work
□ Observe the patient's condition changes.
-guide patients to go through the discharge formalities.
state of illness
change
record
□ None □ Yes, reason:
1.
2.
□ None □ Yes, reason:
1.
2.
nurse
symbol
Certified doctor
symbol
5 Clinical Pathway Hemophilia A Clinical Pathway (version 20 19). docx