I. Myasthenia gravis clinical pathway standard inpatient procedure
(A) Applicable objects.
The first diagnosis of myasthenia gravis (ICD-10: G70, 0)
(B) Diagnostic basis.
According to the Clinical Diagnosis and Treatment Guide-Neurology Sub-volume (edited by the Chinese Medical Association, People’s Health Publishing House)
1. Clinical manifestations are mainly fluctuating weakness of the affected skeletal muscles, i.e., aggravated after activity and improved after rest, and may be “light in the morning and heavy at night”.
2, auxiliary examination: positive neostigmine test; EMG low frequency repetitive electrical stimulation attenuation of more than 10%, high frequency no increment; serum AChR antibody positive or negative.
3, clinical typing (Osserman): I oculomotor type, IIA mild generalized type, IIB moderate generalized type, III acute severe type, IV late severe type, V myasthenia gravis.
(iii) Choice of treatment plan.
According to the Clinical Diagnostic and Treatment Guide-Neurology Branch (edited by Chinese Medical Association, People’s Health Publishing House)
1.Cholinesterase inhibitors.
2.Adrenocorticotropic hormone: Applicable to all types of myasthenia gravis.
3.Other immunosuppressants: applicable to those who have poor hormone efficacy or cannot tolerate.
4.High-dose intravenous immunoglobulin: used in the critical phase, preparation for thymectomy or adjuvant treatment of refractory myasthenia gravis.
5.Plasma exchange: for the critical phase, preparation for thymectomy or adjuvant treatment of refractory myasthenia gravis.
6.Thymectomy: for generalized type patients with thymic hyperplasia, preferred for females; all types of patients with thymoma; generalized type with insignificant effect of drug treatment over 18 years old.
(D) Clinical pathway standard hospitalization days are 2-4 weeks.
(E) Entry pathway criteria.
1.The first diagnosis must be in accordance with ICD-10: G70, 0 myasthenia gravis disease code.
2. When the patient has other disease diagnoses at the same time, but does not require special treatment during hospitalization nor does it affect the implementation of the clinical pathway process for the first diagnosis, he/she can enter the pathway.
(F) Examination items during hospitalization.
1. Urgent examination items.
(1) routine blood, electrolytes (including blood glucose), and four items of coagulation.
2. Compulsory items.
(2) urine routine, stool routine, liver and kidney function, blood lipids, blood sedimentation, thyroid function, blood gas analysis (optional according to the condition), immune five + rheumatology three (immune complete set + anti-nuclear antibody profile), infectious disease screening (hepatitis B, C (hepatitis complete set), syphilis, AIDS, etc.), tumor indicators, etc.
(3) thymus CT (plain + enhanced), electrocardiogram, full chest X-ray;
(4) electromyography + nerve conduction velocity + heavy frequency electrical stimulation (low frequency, high frequency).
3.Optional items.
AChR antibody (and anti-presynaptic membrane antibody, connexin antibody) test.
4.Selective cranial and spinal MRI, MRA, etc. when needed for differential diagnosis.
(G) Selection of medication (treatment plan)
1.Cholinesterase inhibitors: bromipyridamole (dose from 60mg tid to 180mg qid according to the condition), etc.
2, adrenal glucocorticoids: (1) shock therapy (methylprednisolone 0, 5-1g IV qd after every three days to reduce the amount of half, to 60mg when changed to prednisone oral, and then still every three days to reduce half to stop.) ; (2) small dose incremental method (prednisone 20mg qod weekly increase of 10mg to 80mg qod or symptom improvement.) .
(3) Immunosuppressants: Azathioprine (50mg/d starting gradually to 100-200mg/d, total 10g for a course of treatment. (1g of cyclophosphamide (1g IV every 5 days, total 10g for a course of treatment.) Cyclosporine (4mg/(kg, d) divided into two doses, 3-12 months as a course of treatment.) , tacrolimus, etc.
4, high-dose intravenous immunoglobulin (0, 4g/(kg, d) IV, for 5 days.) .
5.Plasma purification therapy
5, (6) Symptomatic treatment and drugs related to the prevention and control of complications: calcium supplementation, potassium supplementation, gastric mucosal protective agents, etc.
(H) Discharge criteria.
1. Myasthenia gravis symptoms improve (those with relative scores of 0 and 2 or more. (The absolute and relative scores of myasthenia gravis are shown in the attached table).
2.Complications are effectively controlled.
(ix) Variation and cause analysis.
1, Co-infections (pulmonary, urinary, intestinal, etc.) during hospitalization, resulting in longer hospitalization and increased costs.
2, Patients using glucocorticoid shock therapy may experience short-term exacerbations, leading to prolonged hospitalization and increased costs.
3. Patients who develop myasthenia gravis crisis and are transferred to the appropriate clinical pathway.