xuè yǒu bìng Alín chuáng lù jìng (2019nián bǎn )
2 Basic InformationThe Clinical Pathway for Hemophilia A (2019 Edition) was published by the National Health and Health Commission General Office of the National Health Commission on December 29, 2019 "Notice of the General Office of the National Health Commission on the Issuance of Clinical Paths for Relevant Diseases (2019 Edition)" (National Health Office Medical Letter 〔2019〕 No. 933) was issued for the reference and use of the health administrative departments at all levels and all types of medical institutions at all levels.
3 Issue noticeNotice of the General Office of the National Health Commission on the issuance of clinical paths for relevant diseases (2019 version)
Medical letter of the State Health Office [2019] No. 933
Health commissions of provinces, autonomous regions, municipalities directly under the central government and the Xinjiang Production and Construction Corps:
In order to further promote the management of clinical paths and standardize the In order to further promote the management of clinical path, standardize clinical diagnosis and treatment behavior, and protect medical quality and safety, the Commission organized the revision of the clinical path of the relevant diseases in 19 disciplines, forming 224 clinical paths (2019 version). It is now issued to you (available in the medical administration and management section of the National Health and Health Commission website) for the reference and use of health administration departments at all levels and medical institutions of all types at all levels.
General Office of the National Health and Wellness Commission
December 29, 2019
4 Full text of the clinical pathwayClinical pathway for hemophilia A (2019 version)
4.1 I. Standard hospitalization process of the clinical pathway for hemophilia A. 4.1.1 (a) Applicable objectsThe first diagnosis is hemophilia A (ICD10: D66.x01).
4.1.2 (ii) Diagnostic basisAccording to the Diagnostic and Therapeutic Efficacy Criteria for Hematological Diseases (edited by Shen Ti and Zhao Yongqiang, Science Publishing House, 2018, 4th edition), Hemophilia Diagnosis and Treatment of Chinese Experts*** Knowledge (2017 edition) [Thrombosis and Hemostasis Group of the Hematology Branch of the Chinese Medical Association, China Hemophilia Collaborative Group, edited by the Chinese Journal of Hematology, 2017,38( 05): 364370], Hemophilia (edited by Yang Renchi and Wang Hongli, Shanghai Science and Technology Press, 2017, 2nd edition).
1. Patients are almost always male (female patients are pureblooded and extremely rare), with or without a family history, and those with a family history conform to the X-linked recessive inheritance pattern.
2. Bleeding from joints and deep muscle tissues, delayed bleeding after trauma or surgery, but also spontaneous bleeding. Repeated bleeding can be seen in joint deformities and pseudotumors.
3. Laboratory tests
(1) Prothrombin time (PT), prothrombin time (TT) and fibrinogen quantification are normal, and activated partial thromboplastin time (APTT) is prolonged, which can be corrected by normal fresh plasma and adsorbed plasma, but not by serum. Platelet count was normal.
(2) Reduced coagulation factor VIII activity (FⅧ:C), FⅧ:C>5% to 40% for light, 1% to 5% for medium, <1% for heavy.
(3) Normal vascular hemophilic factor (vWF) antigen.
4.1.3 (3) Selection of treatment regimenAccording to Hemophilia (edited by Yang Renchi and Wang Hongli, Shanghai Science and Technology Press, 2017, 2nd edition), Hemophilia Diagnosis and Treatment of Chinese Experts*** Knowledge (2017 edition) [Thrombosis and Hemostasis Group of the Hematology Branch of the Chinese Medical Association, China Hemophilia Collaborative Group, edited by the Chinese Journal of Hematology, 2017,38 (05): 364370] and "Hematological Disease Diagnosis and Treatment Standards" (Wang Jianxiang, ed., Peking Union Medical College of China Press, 2014).
1. Local hemostatic measures and precautions: including braking, local compression bandage and placement of ice packs, local application of hemostatic powder, thrombin or gelatin sponge. Bleeding from the mouth can contain tranexamic acid or 6 aminocaproic acid. Avoid intramuscular injections, trauma, and surgery; if surgery is necessary, adequate clotting factor replacement therapy is required. Avoid aspirin or other non-steroidal anti-inflammatory drugs and all drugs that may affect platelet aggregation.
2. Replacement therapy
(1) F VIII preparation: preferred recombinant F VIII preparation or viral inactivation of blood-borne F VIII preparation. F VIII half-life of 8 ~ 12 hours, often need to be infused 2 ~ 3 times a day (for the factor may be consumed in excess, such as major surgery, the first infusion needs to be repeated 2 ~ 4 hours after the first, and then repeat the next 8 ~ 12 hours).
(2) cold precipitate: containing F Ⅷ, fibrinogen and other coagulation factors, F Ⅷ is 5 to 10 times higher than fresh plasma, for those who are unconditional to use F Ⅷ preparation.
(3) Fresh frozen plasma: contains all the coagulation factors and other plasma proteins, only for unconditional use of F Ⅷ preparation and cold precipitation.
3. Deamino-D-arginine vasopressin: used in mild and some intermediate patients, it is recommended that a DDAVP infusion test be performed prior to use, that is, after 12h of DDAVP F VIII increased by 2 to 3 times compared with the basal value, and greater than 0.3 IU/dl is considered effective.
4. Small-dose adrenocorticotropic hormone: it can improve capillary permeability, and is effective in controlling hematuria and accelerating the absorption of acute joint hemosiderosis, and can be used in combination with replacement therapy for a short period of time.
5. Antifibrinolytic drugs: commonly used 6-aminohexanoic acid or tranexamic acid, with naked eye hematuria is prohibited, and avoid the simultaneous use of thromboplastin complex.
4.1.4 (iv) Standard hospitalization is within 10 days 4.1.5 (v) Entry pathway criteria1. The first diagnosis must be consistent with the ICD10: D66.x01 Hemophilia A disease code.
2. Manifestation of acute bleeding in joints, muscles, soft tissues or internal organs.
3. Pathways may be entered when the patient also has other disease diagnoses that do not require special management during hospitalization and do not interfere with the implementation of the clinical pathway process for the first diagnosis.
4.1.6 (F) Checkups during hospitalization1. Necessary checkups
(1) Blood routine, urine routine, stool routine + occult blood.
(2) Liver and kidney function, electrolytes, pre-transfusion examination, blood type, coagulation function, APTT correction test.
(3) FⅧ:C.
(4) vWF:Ag, vWF activity, FⅨ:C test (for those who have not been diagnosed before).
2. Tests that can be selected according to the patient's condition
(1) Measurement of the titer of FⅧ inhibitor.
(2) X-ray chest radiograph, electrocardiogram, hematoma site, organ ultrasound, joint X-ray flat film or MRI, head CT, and so on.
4.1.7 (vii) Start of treatmentStarted immediately after admission for those with a clear diagnosis of hemophilia A prior to admission.
4.1.8 (viii) Treatment regimen and choice of medicationIn acute hemophilia bleeding, F VIII preparation should be infused immediately, and replacement therapy should be carried out in order to reduce the degree of damage to joints, tissues, and organs.
The dose of F VIII preparation can be calculated according to the following formula: the total amount of F VIII:C needed (% of desired F VIII:C level - % of current plasma F VIII:C level) × 0.5 × patient's body weight (kg).
The half-life of FⅧ is 8 to 12 hours, and to maintain FⅧ in the blood at a certain level, it is necessary to infuse it every 8 to 12 hours, preferably within 2 hours. The specific alternative treatment regimen is shown in Table 1.
Table 1 Alternative treatment regimen
Site of bleeding
Desired factor level (%)
FⅧ dose (IU/kg body weight)
Duration of therapy (days)
Joints
40 to 60
20 to 30
1
Muscle
40 to 60
20 to 30
2 to 3
Gastrointestinal
Initial
80 to 100
40 to 50
7 to 14
Maintenance
50
< p> 25
Oral mucosa
30 to 50
15 to 25
Until bleeding subsides
Epistaxis
30 to 50
15 to 25
Until bleeding subsides
Hematuria
30 to 100
15 to 50
Until bleeding subsides
CNS
Initial
80 to 100
40 to 50
1 to 7
Maintenance
50
25
8 to 21
Peritoneal After
50 to 100
25 to 50
7 to 10
Injury or surgery
50 to 100
25 to 50
Until the bleeding has subsided
4.1.9 (ix) Discharge CriteriaImprovement or subsidence of bleeding symptoms.
4.1.10 (X) Analysis of variations and causesExit from this pathway if any of the following conditions are present at the time of initial diagnosis or during the course of the consultation:
1. Positive for FⅧ inhibitors.
2. Patients with co-infections.
3. Life-threatening hemorrhage in vital organs, such as pharyngeal hemorrhage, retroperitoneal hemorrhage, central nervous system hemorrhage, etc., with blurring of consciousness, drop in blood pressure, blood oxygen and other unstable vital signs.
4. Bleeding requiring surgical intervention.
4.2 II. Hemophilia A Clinical Pathway FormApplicable to: first diagnosis of hemophilia A (ICD11: D66.x01)
Patient name: ? Gender: ? Age: Outpatient No.: Inpatient No.
Date of hospitalization: Month/year Discharge date: Month/year Standard hospitalization day: 10 days
Time
Hospitalization day 1
Hospitalization day 2
Main
Diagnosis
Therapy
Work
Work
□ Ask for medical history and physical examination
□ Complete the medical record
□ Write a laboratory checklist
□ Combine laboratory tests to determine a preliminary diagnosis
□ Symptomatic supportive treatment
□ Inform the patient of the condition of the patient, and, if necessary, inform the patient's family of the seriousness of the illness or the criticality of the notification and sign the notification of the seriousness of the illness or criticality of the notification letter
□ Sign an informed consent form for blood transfusion by the patient's family
Sign an informed consent form for blood transfusion by the patient's family
□ Superior physician's examination
□ Continue to complete the admission examination
□ Continue the symptomatic supportive treatment
□ Completion of necessary consultation with relevant departments
□ Completion of medical record writing, such as records of superior physician's examination
□ Explaining the condition of the patient and his family members as well as precautions to be taken
Points to note
□ Inform the patient and his family about the condition and the precautions to be taken. >
Points
Medical
Testimonials
Long-term medical advice
□ Nursing routines for hematology
□ First level of care
□ Diet
□ Notification of serious or critical condition depending on the condition
□ Other medical orders
Provisional medical orders
□ Routine and classification of blood and urine routine, Stool routine + occult blood
□ Liver and kidney function, electrolytes, coagulation function, APTT correction test, blood type, pre-transfusion examination, FⅧ:C and vWF:Ag assay, FIX:C, and FⅧ inhibitor titer assay, if possible
□ X-ray chest radiographs, electrocardiograms, ultrasound of hematomas or organs, arthroplasty, head CT, MRI, etc.
□ Infusion of Genetically recombinant FⅧ or blood-derived FⅧ preparation
□ Cold precipitation
□ Fresh frozen plasma
□ Adrenocorticotropic hormone
□ Anti-fibrinolytic drugs
□ Topical hemostatic therapy
□ Deamino-D-arginine vasopressin
□ Other medical advice
Long-term medical advice
□ Patient's previous underlying medication
□ Other medical advice
Temporary medical advice
□ Coagulation analysis
□ Transfusion of genetically recombinant F VIII or blood-borne F VIII preparation
□ Cold precipitation
□ Fresh frozen plasma
□ Deamino-D-Arginine vasopressin
□ De-amino-D-Arginine vasopressin
□ adrenocorticotropic hormones
□ antifibrinolytic drugs
□ local hemostatic therapy
□ other medical advice
Primary nursing care
Work
□ introduction to the ward environment, facilities, and equipment
□ Admission nursing assessment
□ Advocacy
□? Observation of patient's condition
Variation of condition
Record
□No□Yes, reason:
1.
2.
□No□Yes, reason:
1.
2.
Nurse
Signature
Physician
Signature
Nurse
Signature
Signature
Physician
Signature
Time
Hospitalization day 3-9
Hospitalization day 10
(Discharge day)
Main
Diagnostic
Therapeutic
Work
□ Supervisory physician's checkup
□ Review coagulation function
□ Review of coagulation function, FⅧ:C
□ Observation of bleeding changes
□ Differential diagnosis and determination of diagnosis based on physical examination, results of auxiliary examinations and past data
□ Differential diagnosis based on other examination results and determination of whether there is a combination of other diseases
□ Initiation of treatment
□ Protection of vital organ function
□ Observe the adverse reactions of blood products and treat them symptomatically
□ Complete the record of the course of the disease
□ Supervisory physician's check-up, conduct assessment, determine whether there is any complication situation, and clarify whether to discharge
□ Complete the record of discharge, the first page of the case, and the certificate of discharge, etc.
□ Explain the patient about the precautions to be taken after discharge, such as the time and place of returning to hospital for follow-up consultation, Emergencies
Important
Points
Medical
Testament
Long-term medical advice (diagnosis is clear and immediately start treatment)
□ Infusion of recombinant F Ⅷ or blood-borne F Ⅷ preparation
□ Cold precipitation
□ Fresh frozen plasma
□ Demineralized-D-arginine vasopressor. -D-arginine vasopressin
□ adrenocorticotropic hormone
□ antifibrinolytic medication
□ topical hemostatic therapy and care
□ other medical orders
provisional medical orders
□ review of blood routine
□ review of blood biochemistry, coagulation function, FⅧ:C level
□ Symptomatic support
□ Other medical advice
Discharge medical advice
□ Discharge with medication
□ Regular outpatient follow-up
□ Monitoring of coagulation function
Primary nursing care
Work
□ Observation of patient's condition change
□ Instruct the patient for discharge procedures
Condition
Variation
Record
□No□Yes, Reason:
1.
2.
□No□Yes, Reason:
1.
2.
Nurse
Signature
< p>Physician
Signature
5 Clinical Pathway
Hemophilia A Clinical Pathway (2019 Edition).docx