BACKGROUND According to the World Health Organization analgesic ladder,cancer-related pain generally begins with pharmacotherapy in a stepwise approach.Nevertheless,some patients continue to experience poorly controll...BACKGROUND According to the World Health Organization analgesic ladder,cancer-related pain generally begins with pharmacotherapy in a stepwise approach.Nevertheless,some patients continue to experience poorly controlled pain despite medications,particularly when considering adverse effects and self-care quality.Percutaneous cervical cordotomy is an alternative interventional procedure for unremitting unilateral intractable cancer-related pain.CASE SUMMARY The patient was diagnosed with lung cancer with destruction of the brachial plexus and ribs.For 2 mo,the patient experienced progressive severe weakness and pain in the right upper extremity.Notably,the pain intensity reached an extreme level,particularly when lying supine,even under heavy sedation.This heightened pain response posed a significant challenge;as a result,the patient was unable to undergo further evaluation through magnetic resonance imaging.Ultimately,he underwent percutaneous cervical cordotomy for symptom relief,resulting in complete resolution of right arm pain.After a 3-mo follow-up,the pain did not recur,and only a flurbiprofen local patch was required for mild scapular tightness.CONCLUSION Cordotomy,under careful patient selection,appears to enhance the quality of life of patients with unilateral cancerrelated pain.展开更多
BACKGROUND Coronary artery disease(CAD)is a leading cause of global cardiovascular morta-lity.Refractory angina pectoris,a manifestation of CAD,requires effective drug treatments.Pericarpium Trichosanthis injection,a ...BACKGROUND Coronary artery disease(CAD)is a leading cause of global cardiovascular morta-lity.Refractory angina pectoris,a manifestation of CAD,requires effective drug treatments.Pericarpium Trichosanthis injection,a traditional Chinese medicine,improves cardiovascular symptoms,while nicorandil alleviates spasms and angina.Both have potential in treating CAD.AIM To investigate the therapeutic effects of combining Pericarpium Trichosanthis injection and nicorandil in elderly patients suffering from refractory angina caused by coronary heart disease.METHODS A retrospective analysis was conducted on the data of 130 patients diagnosed with refractory coronary heart disease.Based on the different treatment regimens administered during hospitalization,the patients were divided into a control group(58 cases)and a study group(72 cases).The control group received conven-tional treatment,which included aspirin,statins,and nitrate vasodilators.In addition to the conventional medication,the study group received a combination treatment of Pericarpium Trichosanthis injection and nicorandil.RESULTS After treatment,the study group showed significantly higher left ventricular ejection fraction and cardiac output,and lower brain natriuretic peptide and C-reactive protein levels compared to the control group.The study group also exhibited improvements in angina,quality of life,exercise endurance,and lipid profiles.Multivariate logistic regression analysis revealed a relationship of lipid levels and heart function with the combined treatment.Some patients in the study group experienced headaches during treatment,but no significant adverse reactions were observed.Follow-up showed that the treatment was well-tolerated,with no drug-related adverse reactions detected.CONCLUSION Combination of Pericarpium Trichosanthis injection and nicorandil is more effective than conventional treatment in improving symptoms and heart function in elderly patients with refractory angina pectoris.展开更多
Objective To study the feasibility of developing botanical drugs to treat intractable diseases and play an important role in dealing with major public health crises.Methods From January 1990 to May 2021,a bibliographi...Objective To study the feasibility of developing botanical drugs to treat intractable diseases and play an important role in dealing with major public health crises.Methods From January 1990 to May 2021,a bibliographic search was carried out on the use of botanical drugs,rare disease drugs,related registration management policies and regulations in PubMed and CNKI.The following keywords were searched in the database:Rare disease policies and regulations,orphan drugs,botanical drugs for intractable diseases,botanical drugs for the treatment of new coronary pneumonia,traditional Chinese medicine,and emergency guidelines for major public health crisis.Other data were obtained from“Chinese Pharmacopoeia”and relevant Chinese government websites for sorting and analysis.Results and Conclusion Based on 39 Chinese corresponding policies and regulations,challenges and opportunities of developing and researching drugs for treating rare diseases were found out after the analysis and comparison.Based on the study of national policies on drugs for rare diseases,the priority review and approval procedures in the drug registration,as well as China’s emergency guidelines and policies for major public health events,some problems in the use of drugs for rare diseases are found out.Therefore,it is recommended to actively adopt the property rights protection system,explore the folk prescriptions of traditional Chinese medicine and the potential of hospital preparations,and the registration review strategy of giving priority to the use of botanical drugs for rare diseases.Thus,the international status of botanical drugs for rare disease and the influence of responding to major public health events can be enhanced.展开更多
The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morpho...The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morphological aberrations of the majority of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type II is the most frequent type. All types of congenital dyserythropoietic anemias distinctly share a high incidence of iron loading. Iron accumulation occurs even in untransfused patients and can result in heart failure and liver cirrhosis. We have reported about a patient who presented with liver cirrhosis and intractable ascites caused by congenital dyserythropoietic anemia type II. Her clinical course was further complicated by the development of autoimmune hemolytic anemia. Splenectomy was eventually performed which achieved complete resolution of ascites, increase of hemoglobin concentration and abrogation of transfusion requirements.展开更多
Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare cholestatic liver disease. Such liver disease can get worse by female hormone disorder. Albumin dialysis or Molecular Adsorbent Recirculating Syst...Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare cholestatic liver disease. Such liver disease can get worse by female hormone disorder. Albumin dialysis or Molecular Adsorbent Recirculating System (MARS) has been reported to reverse severe cholestasis-linked pruritus. Here, we report the first use of MARS during a spontaneous pregnancy and its successful outcome in a patient with PFIC3 and intractable pruritus. Albumin dialysis could be considered as a pregnancy-saving procedure in pregnant women with severe cholestasis and refractory pruritus.展开更多
Gastric varices(GV) are one of the most common complications for patients with portal hypertension. Currently, histoacryl injection is recommended as the initial treatment for bleeding of GV, and this injection has be...Gastric varices(GV) are one of the most common complications for patients with portal hypertension. Currently, histoacryl injection is recommended as the initial treatment for bleeding of GV, and this injection has been confirmed to be highly effective for most patients in many studies. However, this treatment might be ineffective for some types of GV, such as splenic vein thrombosis-related localized portal hypertension(also called left-sided, sinistral, or regional portal hypertension). Herein, we report a case of repeated pancreatitis-induced complete splenic vein thrombosis that led to intractable gastric variceal bleeding, which was treated by splenectomy. We present detailed radiological and pathological data and blood rheology analysis(the splenic artery- after a short gastric vein or stomach vein- gastric coronary vein- portal vein). The pathophysiology can be explained by the abnormal direction of blood flow in this patient. To our knowledge, this is the first reported case for which detailed patho-logy and blood rheology data are available.展开更多
Antiepileptic drugs are the preferred treatment approach for epileptic patients. However, informal treatment is important for intractable epilepsy. In this study, 500 epileptic patients were recruited from the General...Antiepileptic drugs are the preferred treatment approach for epileptic patients. However, informal treatment is important for intractable epilepsy. In this study, 500 epileptic patients were recruited from the General Hospital of Beijing Military Area Command of Chinese PLA during the period of October 2009 to January 2012. These involved patients that had been medically treated for at least 1 year. Information on the initial treatment and changes to treatment regimens for each patient was collected through questionnaires. The survey results showed that 52.3% of the epileptic patients searched for treatment after the first seizure, and the mean numbers of seizures was 12.8; 59.8% of the epileptic patients were diagnosed at the first visit, and the mean onset time was 17 months after the first seizure. After diagnosis, patients were treated for an average of 20 days, and the median time was 1 day. Formal anti-epileptic drugs were selected as the first treatment regimen by 67.8% of patients, and 77.5% of these drugs were monotherapies. The mean and median numbers of seizure were respectively 36.9 and 3.0 times before the first regimen was changed. The regimen was changed within the first 6 months by 46.6% of patients, and after the first and second years of treatment, the proportions increased to 54.0% and 71.8%, respectively. In total, 78.5% of the regi- mens were changed to informal treatments. The informal treatment of epilepsy in China is common, being initiated by either patients or physicians. Enhancing epileptic treatment services in hospital, improving physicians' professional quality, and strengthening health propaganda may promote the normalization of drug treatment of epilepsy in China.展开更多
INTRODUCTIONOf the three cardinal manifestations of chronic pancreatitis-pain,diabetes mellitus and steatorrhea,it is pain thatbrings the patient to the physician and is the most difficultto manage.The intractabale pa...INTRODUCTIONOf the three cardinal manifestations of chronic pancreatitis-pain,diabetes mellitus and steatorrhea,it is pain thatbrings the patient to the physician and is the most difficultto manage.The intractabale pain that is quite展开更多
Takayasu’s arteritis(TA)and Crohn’s disease(CD)are two rare autoimmune disorders;however some reports describe the presence of both diseases in the same patient.This finding has suggested the possibility that both d...Takayasu’s arteritis(TA)and Crohn’s disease(CD)are two rare autoimmune disorders;however some reports describe the presence of both diseases in the same patient.This finding has suggested the possibility that both diseases could share some common etiologic origin.We describe a case of a 13-year-old male affected by CD characterized by fever,diarrhea,weight loss,abdominal pain and elevation of inflammatory markers.Clinical and histological features from colonic specimens were consistent with CD.Treatment with steroids and azathioprine was started,however disease flared every time steroids were tapered.One year later,while still on treatment,he came back to our attention for dyspnea at rest and at night,tiredness and weakness.At physical examination a diastolic heart murmur was found as well as a left carotid artery bruit.A transthoracic echocardiography showed mild aortic valve insufficiency,left ventricular hypertrophy and a dilated ascending aorta with same findings at the aortic arch.A computed tomography scan showed abdominal aortathickening,dilated thoracic aorta and the presence of a thoracic aortic aneurysm.TA associated with CD was diagnosed and medical treatment with cyclophosphamide,steroids and aminosalicylic acid was started,with good clinical response at 6 mo follow-up.We discuss the presence of possible common causes for the two diseases and the importance of differential diagnosis in those patients characterized for intractable disease.展开更多
A peritoneovenous shunt has become one of the most effi cient procedures for intractable ascites due to liver cirrhosis.A case of intractable ascites due to hepatic lymphorrhea after hepatectomy for hepatocellular car...A peritoneovenous shunt has become one of the most effi cient procedures for intractable ascites due to liver cirrhosis.A case of intractable ascites due to hepatic lymphorrhea after hepatectomy for hepatocellular carcinoma that was successfully treated by the placement of a peritoneovenous shunt is presented.A 72-year-old Japanese man underwent partial resection of the liver for hepatocellular carcinoma associated with hepatitis C viral infection.After hepatectomy,a considerable amount of ascites ranging from 800-4600 mL per day persisted despite conservative therapy,including numerous infusions of albumin and plasma protein fraction and administration of diuretics.Since the patient's general condition deteriorated,based on the diagnosis of intractable hepatic lymphorrhea,a subcutaneous peritoneovenous shunt was inserted.The patient's postoperative course was uneventful and the ascites decreased rapidly,with serum total protein and albumin levels and hepatic function improving accordingly.For intractable ascites due to hepatic lymphorrhea after hepatectomy,we recommend the placement of a peritoneovenous shunt as a procedure that can provide immediate effectiveness without increased surgical risk.展开更多
A 66yearold gentleman presented for a cardiovascular evaluation secondary to chest discomfort,right knee pain,and intractable hiccups.The patient also had type 2 diabetes mellitus,hypercholesterolemia,aortic atheroscl...A 66yearold gentleman presented for a cardiovascular evaluation secondary to chest discomfort,right knee pain,and intractable hiccups.The patient also had type 2 diabetes mellitus,hypercholesterolemia,aortic atherosclerosis,and ascending aortic aneurysm.His family history was also significant for premature coronary artery disease in the father.The patient had been compliant with his medical therapy,which included subcutaneous 200 units/mL insulin degludec,18 mg/3 mL subcutaneous liraglutide,daily oral 500 mg metformin hydrochloride,daily oral 100 mg sitagliptin,daily oral 12.5 mg zolpidem,daily oral 5 mg tadalafil,and 20 mg tablet rosuvastatin daily.展开更多
Neurodermatitis,a commonly seen,intractable dermal disease,is characterizedby severe paroxysmal itching,lichenoidchanges and other symptoms of chronic in-flammation of skin.Most patients
BACKGROUND: Increased expression of multidrug resistance 1 (MDR1) mRNA in peripheral blood of patients with intractable epilepsy is not due to epilepsy drugs, but epilepsy behavior. Monitoring MDR1 expression in pe...BACKGROUND: Increased expression of multidrug resistance 1 (MDR1) mRNA in peripheral blood of patients with intractable epilepsy is not due to epilepsy drugs, but epilepsy behavior. Monitoring MDR1 expression in peripheral blood is a target for MDR1 gene evaluation. OBJECTIVE: To investigate the influence of antiepileptic drugs and seizures on MDR expression in intractable epilepsy, and to analyze the genetic polymorphisms of C3435T in the MDRl gene. DESIGN, TIME AND SETTING: Factorial designs and comparative observations at the experimental center of the Affiliated Hospital of Qingdao Medical College, Qingdao University between October 2003 and October 2004. PARTICIPANTS: A total of 120 subjects were recruited from the epilepsy clinical department of the Affiliated Hospital of Qingdao Medical College. Four groups (n = 30) were classified according to statistical factorial design: intractable epilepsy, treatment response, no treatment, and normal control groups. METHODS: One-step semi-quantitative reverse-transcription polymerase chain reaction technology was used to test expressions of the MDR1 gene in 120 subjects. C3435T polymorphisms in intractable epilepsy group and normal control groups were analyzed by polymerase chain reaction-restriction fragment length polymorphism. MAIN OUTCOME MEASURES: Expression of MDR1 mRNA in the four groups, and C3435T genetic polymorphisms in intractable epilepsy and normal control groups. RESULTS: MDRl gene expression was increased in the intractable epilepsy group, due to the factor seizures, but not the antiepileptic drugs. However, the interaction between the two factors was not statistically significant. Of the 30 subjects in the intractable epilepsy group, the following genotypes were exhibited: 3 (10%) C/C genotype, 9 (30%) C/T genotype, and 18 (60%) T/T genotype at the site of C3435T, while 4 (13%), 10 (33%), and 16 (53%) subjects were determined to express these genotypes in the normal control group, respectively. C and T allele frequency were 25% and 75% in the intractable epilepsy group, and 30% and 70% in the normal control group, respectively. However, there was no statistical difference between the groups. CONCLUSION: Results demonstrated that seizures, not antiepileptic drugs, induced MDR1 gene expression in intractable epilepsy. Genetic polymorphisms of C3435T in the MDR1 gene did not contribute to the development of multidrug resistance in patients with intractable epilepsy.展开更多
BACKGROUND: The biochemical hypothesis of dopamine hyperfunction in the brain can explain the pathological mechanisms of schizophrenia. Surgery is performed based on limbic system circuit theory correspondence to abo...BACKGROUND: The biochemical hypothesis of dopamine hyperfunction in the brain can explain the pathological mechanisms of schizophrenia. Surgery is performed based on limbic system circuit theory correspondence to above-mentioned hypothesis. Stereotactic surgery for the treatment of mental disorders is related to stereotactic surgery that influences the Papez circuit. OBJECTIVE: To observe the effects of stereotactic multi-target limbic leucotomy on the improvements in memory, intelligence and psychiatric symptoms in the treatment of intractable psychiatric disease. DESIGN: Self-control case analysis and follow-up of therapeutic effects. SETTING: Department of Neurosurgery, First Hospital, Hebei Medical University. PARTICIPANTS: Thirty patients with intractable psychiatric disease, who received stereotactic surgery in the Department of Neurosurgery, First Hospital, Hebei Medical University between July 2002 and August 2005, were included in this study. The patients, 21 males and 9 females, all met the diagnostic criteria of intractable psychiatric disease, determined by the national psychosurgery cooperation team in 1998. Informed consents for surgery and clinical follow-up exams were obtained from patients and/or patients' relatives (guardians). METHODS: In 30 patients with intractable psychiatric disease, limbic leucotomy was performed by stereotactic technique. Multi-target radiofrequency hyperthermia was performed in the intracranial amygdaloid nucleus, anterior limb of internal capsule, callosal gyrus, among other regions. The therapeutic effects of patients were evaluated by Brief Psychiatric Rating Scale (BPRS) before surgery, and 6 months, 1, and 3 years after surgery. The Wechsler Adult Intelligence Scale (WAIS) and Clinical Memory Scale (CMS) were used to assess memory and intelligence before and after surgery. MAIN OUTCOME MEASURES: Memory, intelligence, and psychiatric symptoms of patients before and after operation. RESULTS: Thirty patients were included in the final analysis. (1) Memory and intelligence changes of patients: CMS and WAIS showed there were no significant differences in the changes of brain functions before and after operation (P 〉 0.05). (2) Improved degree of symptoms: 5 patients were cured, 8 patients significantly improved, 8 patients improved, 3 patients had no changes, and 6 patients had recurrent symptoms. CONCLUSION: Stereotactic multi-target limbic leucotomy can improve the symptoms of patients with intractable psychiatric disease. Follow-up results showed that memory and intelligence were not noticeably improved after surgery.展开更多
BACKGROUND Implant vagus nerve stimulation is an adjunctive treatment for intractable epilepsy when patients are not suitable for resective surgery.AIM To identify the safety and efficacy of vagus nerve stimulation in...BACKGROUND Implant vagus nerve stimulation is an adjunctive treatment for intractable epilepsy when patients are not suitable for resective surgery.AIM To identify the safety and efficacy of vagus nerve stimulation in children with intractable epilepsy and analyze the effects on different epilepsy syndromes.METHODS Eligible children with intractable epilepsy were admitted to the study.We collected data from preoperative assessments as the baseline.During the followup time,we recorded the process of seizures(frequency,duration,and seizure type),the changes of drugs or parameters,the complications,etc.The mean reduction rate of seizures,response rate,and McHugh scale were chosen as the outcomes.RESULTS A total of 213 patients were implanted with Tsinghua Pins vagus nerve stimulators,and the average age was 6.6 years.In the follow-up time of postoperative 3 mo,6 mo,12 mo,18 mo,and 24 mo,the average reduction rate was 30.2%,49.5%,56.3%,59.4%,and 63.2%,while the response rate was 21.8%,62.5%,57.1%,69.2%,and 70.7%.In addition,implanted vagus nerve stimulation had different effects on epilepsy syndromes.The reduction rate of West syndrome increased from 36.4%(postoperative 6 m)to 74.3%(postoperative 24 m).The reduction rate of Lennox-Gastaut syndrome improved from 25.4%to 73.1%in 24 mo.The chi-square test of the five efficacy grades showed P<0.05.The comparison between the 3-mo follow-up and the 6-mo follow-up showed P<0.05,and the comparison between the 6-mo follow-up and the 24-mo follow-up showed P>0.05.CONCLUSION Vagus nerve stimulation is safe and effective in children with intractable epilepsy,and the seizure reduction occurred in a time-dependent manner.Moreover,patients with West syndrome may get the most benefits.展开更多
Background: The effect of Vagus nerve stimulation (VNS) therapy following major resective surgeries has been reported. However, the effect of VNS therapy following multiple-subpial-transections (MST) has not been repo...Background: The effect of Vagus nerve stimulation (VNS) therapy following major resective surgeries has been reported. However, the effect of VNS therapy following multiple-subpial-transections (MST) has not been reported. The objective of this paper is to examine the beneficial effect of VNS therapy following MST. Methods: There are 22 patients aged 10 - 55 years. Male/female distribution is 11/11 and follow-up is 24 - 148 months (median of 120 months). Seizure foci were bilateral in 9 patients, multi-lobar (unilateral) in 12 patients and single-lobar in 1 patient. MST was performed over broad areas in and around the seizure foci. VNS implantation was done when the response to MST procedure was poor (1 patients), or there was recurrence of seizures (21 patients). Interval between MST and VNS implanttation varied from one month to three years (median of 2 years). Results: Thirteen patients (59%) are seizure free (Engel’ Class I), 8 (36.5%) have greater than 90% reduction in seizure frequency (Class II), and 1 (4.5%) has between 50% - 90% reduction in seizure frequency (Class III). Conclusion: The results show that VNS therapy produced meaningful improvement in seizure outcome in all patients with extra-temporal seizures that had inadequate response to MST.展开更多
Synaptic vesicle protein 2A(SV2A) involvement has been reported in the animal models of epilepsy and in human intractable epilepsy. The difference between pharmacosensitive epilepsy and pharmacoresistant epilepsy re...Synaptic vesicle protein 2A(SV2A) involvement has been reported in the animal models of epilepsy and in human intractable epilepsy. The difference between pharmacosensitive epilepsy and pharmacoresistant epilepsy remains poorly understood. The present study aimed to observe the hippocampus SV2 A protein expression in amygdale-kindling pharmacoresistant epileptic rats. The pharmacosensitive epileptic rats served as control. Amygdaloid-kindling model of epilepsy was established in 100 healthy adult male Sprague-Dawley rats. The kindled rat model of epilepsy was used to select pharmacoresistance by testing their seizure response to phenytoin and phenobarbital. The selected pharmacoresistant rats were assigned to a pharmacoresistant epileptic group(PRE group). Another 12 pharmacosensitive epileptic rats(PSE group) served as control. Immunohistochemistry,real-time PCR and Western blotting were used to determine SV2 A expression in the hippocampus tissue samples from both the PRE and the PSE rats. Immunohistochemistry staining showed that SV2 A was mainly accumulated in the cytoplasm of the neurons,as well as along their dendrites throughout all subfields of the hippocampus. Immunoreactive staining level of SV2A-positive cells was 0.483±0.304 in the PRE group and 0.866±0.090 in the PSE group(P〈0.05). Real-time PCR analysis demonstrated that 2-ΔΔCt value of SV2 A m RNA was 0.30±0.43 in the PRE group and 0.76±0.18 in the PSE group(P〈0.05). Western blotting analysis obtained the similar findings(0.27±0.21 versus 1.12±0.21,P〈0.05). PRE rats displayed a significant decrease of SV2 A in the brain. SV2 A may be associated with the pathogenesis of intractable epilepsy of the amygdaloid-kindling rats.展开更多
Neuropathic pain,a type of pain arising after direct damage or disease of the nervous system,is often intractable and challenges the search of effective therapeutic strategies.In particular,neuropathic pain is a very ...Neuropathic pain,a type of pain arising after direct damage or disease of the nervous system,is often intractable and challenges the search of effective therapeutic strategies.In particular,neuropathic pain is a very frequent sequel of spinal cord injury(SCI)and a decisive contributor to decreased quality of life.展开更多
This study looked into the efficacy of a modified titration protocol of intratympanic gentamicin injection(ITG) in the patients with unilateral intractable Ménière's disease(MD). Modified titration prot...This study looked into the efficacy of a modified titration protocol of intratympanic gentamicin injection(ITG) in the patients with unilateral intractable Ménière's disease(MD). Modified titration protocol of ITG at a low dose(20 mg/m L) was administered to 10 patients with definite unilateral intractable MD. After initial first two fixed ITGs on weekly basis,the patients might or might not be given any more injections,depending on the appearance of unilateral vestibular loss(UVL). ITG was terminated if the patients satisfied the criteria of UVL. All patients were followed-up for at least two years. The effects of ITG on the vertigo attack,functional level scores and postural balance were evaluated. Of the 10 cases,8 showed the sign of UVL after receiving initial two ITGs and were not given any more intratympanic injections,and the other 2 patients were administered three ITGs. A two-year follow-up revealed that complete and substantial vertigo control was achieved in 9 cases,and limited vertigo control in 1 patient. Hearing level was lowered in 2 patients. The posture stability and functional level scores were improved. Our study showed that the modified titration protocol of ITG at a low dose could effectively control vertigo in patients with unilateral intractable MD.展开更多
Neurodermatitis, a commonly seen, intractable dermal disease. is characterized by severe paroxysmal itching, lichenoid changes and other symptoms of chronic inflammation of skin. Most patients are accompanied by neura...Neurodermatitis, a commonly seen, intractable dermal disease. is characterized by severe paroxysmal itching, lichenoid changes and other symptoms of chronic inflammation of skin. Most patients are accompanied by neurasthenic symptoms. The predilection sites of the disease, generally speaking. include the neck, the nuchal region, the popliteal fossa, the sacral region. the ventral surface of the abdomen, the perineum, etc. Neurodermatitis may展开更多
文摘BACKGROUND According to the World Health Organization analgesic ladder,cancer-related pain generally begins with pharmacotherapy in a stepwise approach.Nevertheless,some patients continue to experience poorly controlled pain despite medications,particularly when considering adverse effects and self-care quality.Percutaneous cervical cordotomy is an alternative interventional procedure for unremitting unilateral intractable cancer-related pain.CASE SUMMARY The patient was diagnosed with lung cancer with destruction of the brachial plexus and ribs.For 2 mo,the patient experienced progressive severe weakness and pain in the right upper extremity.Notably,the pain intensity reached an extreme level,particularly when lying supine,even under heavy sedation.This heightened pain response posed a significant challenge;as a result,the patient was unable to undergo further evaluation through magnetic resonance imaging.Ultimately,he underwent percutaneous cervical cordotomy for symptom relief,resulting in complete resolution of right arm pain.After a 3-mo follow-up,the pain did not recur,and only a flurbiprofen local patch was required for mild scapular tightness.CONCLUSION Cordotomy,under careful patient selection,appears to enhance the quality of life of patients with unilateral cancerrelated pain.
文摘BACKGROUND Coronary artery disease(CAD)is a leading cause of global cardiovascular morta-lity.Refractory angina pectoris,a manifestation of CAD,requires effective drug treatments.Pericarpium Trichosanthis injection,a traditional Chinese medicine,improves cardiovascular symptoms,while nicorandil alleviates spasms and angina.Both have potential in treating CAD.AIM To investigate the therapeutic effects of combining Pericarpium Trichosanthis injection and nicorandil in elderly patients suffering from refractory angina caused by coronary heart disease.METHODS A retrospective analysis was conducted on the data of 130 patients diagnosed with refractory coronary heart disease.Based on the different treatment regimens administered during hospitalization,the patients were divided into a control group(58 cases)and a study group(72 cases).The control group received conven-tional treatment,which included aspirin,statins,and nitrate vasodilators.In addition to the conventional medication,the study group received a combination treatment of Pericarpium Trichosanthis injection and nicorandil.RESULTS After treatment,the study group showed significantly higher left ventricular ejection fraction and cardiac output,and lower brain natriuretic peptide and C-reactive protein levels compared to the control group.The study group also exhibited improvements in angina,quality of life,exercise endurance,and lipid profiles.Multivariate logistic regression analysis revealed a relationship of lipid levels and heart function with the combined treatment.Some patients in the study group experienced headaches during treatment,but no significant adverse reactions were observed.Follow-up showed that the treatment was well-tolerated,with no drug-related adverse reactions detected.CONCLUSION Combination of Pericarpium Trichosanthis injection and nicorandil is more effective than conventional treatment in improving symptoms and heart function in elderly patients with refractory angina pectoris.
文摘Objective To study the feasibility of developing botanical drugs to treat intractable diseases and play an important role in dealing with major public health crises.Methods From January 1990 to May 2021,a bibliographic search was carried out on the use of botanical drugs,rare disease drugs,related registration management policies and regulations in PubMed and CNKI.The following keywords were searched in the database:Rare disease policies and regulations,orphan drugs,botanical drugs for intractable diseases,botanical drugs for the treatment of new coronary pneumonia,traditional Chinese medicine,and emergency guidelines for major public health crisis.Other data were obtained from“Chinese Pharmacopoeia”and relevant Chinese government websites for sorting and analysis.Results and Conclusion Based on 39 Chinese corresponding policies and regulations,challenges and opportunities of developing and researching drugs for treating rare diseases were found out after the analysis and comparison.Based on the study of national policies on drugs for rare diseases,the priority review and approval procedures in the drug registration,as well as China’s emergency guidelines and policies for major public health events,some problems in the use of drugs for rare diseases are found out.Therefore,it is recommended to actively adopt the property rights protection system,explore the folk prescriptions of traditional Chinese medicine and the potential of hospital preparations,and the registration review strategy of giving priority to the use of botanical drugs for rare diseases.Thus,the international status of botanical drugs for rare disease and the influence of responding to major public health events can be enhanced.
文摘The congenital dyserythropoietic anemias comprise a group of rare hereditary disorders of erythropoiesis, characterized by ineffective erythropoiesis as the predominant mechanism of anemia and by characteristic morphological aberrations of the majority of erythroblasts in the bone marrow. Congenital dyserythropoietic anemia type II is the most frequent type. All types of congenital dyserythropoietic anemias distinctly share a high incidence of iron loading. Iron accumulation occurs even in untransfused patients and can result in heart failure and liver cirrhosis. We have reported about a patient who presented with liver cirrhosis and intractable ascites caused by congenital dyserythropoietic anemia type II. Her clinical course was further complicated by the development of autoimmune hemolytic anemia. Splenectomy was eventually performed which achieved complete resolution of ascites, increase of hemoglobin concentration and abrogation of transfusion requirements.
文摘Progressive familial intrahepatic cholestasis type 3 (PFIC3) is a rare cholestatic liver disease. Such liver disease can get worse by female hormone disorder. Albumin dialysis or Molecular Adsorbent Recirculating System (MARS) has been reported to reverse severe cholestasis-linked pruritus. Here, we report the first use of MARS during a spontaneous pregnancy and its successful outcome in a patient with PFIC3 and intractable pruritus. Albumin dialysis could be considered as a pregnancy-saving procedure in pregnant women with severe cholestasis and refractory pruritus.
基金Supported by National Natural Science Foundation of China,No.81401993(to Tang SH)
文摘Gastric varices(GV) are one of the most common complications for patients with portal hypertension. Currently, histoacryl injection is recommended as the initial treatment for bleeding of GV, and this injection has been confirmed to be highly effective for most patients in many studies. However, this treatment might be ineffective for some types of GV, such as splenic vein thrombosis-related localized portal hypertension(also called left-sided, sinistral, or regional portal hypertension). Herein, we report a case of repeated pancreatitis-induced complete splenic vein thrombosis that led to intractable gastric variceal bleeding, which was treated by splenectomy. We present detailed radiological and pathological data and blood rheology analysis(the splenic artery- after a short gastric vein or stomach vein- gastric coronary vein- portal vein). The pathophysiology can be explained by the abnormal direction of blood flow in this patient. To our knowledge, this is the first reported case for which detailed patho-logy and blood rheology data are available.
基金supported by Beijing Natural Science Foundation,No.4132017
文摘Antiepileptic drugs are the preferred treatment approach for epileptic patients. However, informal treatment is important for intractable epilepsy. In this study, 500 epileptic patients were recruited from the General Hospital of Beijing Military Area Command of Chinese PLA during the period of October 2009 to January 2012. These involved patients that had been medically treated for at least 1 year. Information on the initial treatment and changes to treatment regimens for each patient was collected through questionnaires. The survey results showed that 52.3% of the epileptic patients searched for treatment after the first seizure, and the mean numbers of seizures was 12.8; 59.8% of the epileptic patients were diagnosed at the first visit, and the mean onset time was 17 months after the first seizure. After diagnosis, patients were treated for an average of 20 days, and the median time was 1 day. Formal anti-epileptic drugs were selected as the first treatment regimen by 67.8% of patients, and 77.5% of these drugs were monotherapies. The mean and median numbers of seizure were respectively 36.9 and 3.0 times before the first regimen was changed. The regimen was changed within the first 6 months by 46.6% of patients, and after the first and second years of treatment, the proportions increased to 54.0% and 71.8%, respectively. In total, 78.5% of the regi- mens were changed to informal treatments. The informal treatment of epilepsy in China is common, being initiated by either patients or physicians. Enhancing epileptic treatment services in hospital, improving physicians' professional quality, and strengthening health propaganda may promote the normalization of drug treatment of epilepsy in China.
文摘INTRODUCTIONOf the three cardinal manifestations of chronic pancreatitis-pain,diabetes mellitus and steatorrhea,it is pain thatbrings the patient to the physician and is the most difficultto manage.The intractabale pain that is quite
文摘Takayasu’s arteritis(TA)and Crohn’s disease(CD)are two rare autoimmune disorders;however some reports describe the presence of both diseases in the same patient.This finding has suggested the possibility that both diseases could share some common etiologic origin.We describe a case of a 13-year-old male affected by CD characterized by fever,diarrhea,weight loss,abdominal pain and elevation of inflammatory markers.Clinical and histological features from colonic specimens were consistent with CD.Treatment with steroids and azathioprine was started,however disease flared every time steroids were tapered.One year later,while still on treatment,he came back to our attention for dyspnea at rest and at night,tiredness and weakness.At physical examination a diastolic heart murmur was found as well as a left carotid artery bruit.A transthoracic echocardiography showed mild aortic valve insufficiency,left ventricular hypertrophy and a dilated ascending aorta with same findings at the aortic arch.A computed tomography scan showed abdominal aortathickening,dilated thoracic aorta and the presence of a thoracic aortic aneurysm.TA associated with CD was diagnosed and medical treatment with cyclophosphamide,steroids and aminosalicylic acid was started,with good clinical response at 6 mo follow-up.We discuss the presence of possible common causes for the two diseases and the importance of differential diagnosis in those patients characterized for intractable disease.
文摘A peritoneovenous shunt has become one of the most effi cient procedures for intractable ascites due to liver cirrhosis.A case of intractable ascites due to hepatic lymphorrhea after hepatectomy for hepatocellular carcinoma that was successfully treated by the placement of a peritoneovenous shunt is presented.A 72-year-old Japanese man underwent partial resection of the liver for hepatocellular carcinoma associated with hepatitis C viral infection.After hepatectomy,a considerable amount of ascites ranging from 800-4600 mL per day persisted despite conservative therapy,including numerous infusions of albumin and plasma protein fraction and administration of diuretics.Since the patient's general condition deteriorated,based on the diagnosis of intractable hepatic lymphorrhea,a subcutaneous peritoneovenous shunt was inserted.The patient's postoperative course was uneventful and the ascites decreased rapidly,with serum total protein and albumin levels and hepatic function improving accordingly.For intractable ascites due to hepatic lymphorrhea after hepatectomy,we recommend the placement of a peritoneovenous shunt as a procedure that can provide immediate effectiveness without increased surgical risk.
文摘A 66yearold gentleman presented for a cardiovascular evaluation secondary to chest discomfort,right knee pain,and intractable hiccups.The patient also had type 2 diabetes mellitus,hypercholesterolemia,aortic atherosclerosis,and ascending aortic aneurysm.His family history was also significant for premature coronary artery disease in the father.The patient had been compliant with his medical therapy,which included subcutaneous 200 units/mL insulin degludec,18 mg/3 mL subcutaneous liraglutide,daily oral 500 mg metformin hydrochloride,daily oral 100 mg sitagliptin,daily oral 12.5 mg zolpidem,daily oral 5 mg tadalafil,and 20 mg tablet rosuvastatin daily.
文摘Neurodermatitis,a commonly seen,intractable dermal disease,is characterizedby severe paroxysmal itching,lichenoidchanges and other symptoms of chronic in-flammation of skin.Most patients
文摘BACKGROUND: Increased expression of multidrug resistance 1 (MDR1) mRNA in peripheral blood of patients with intractable epilepsy is not due to epilepsy drugs, but epilepsy behavior. Monitoring MDR1 expression in peripheral blood is a target for MDR1 gene evaluation. OBJECTIVE: To investigate the influence of antiepileptic drugs and seizures on MDR expression in intractable epilepsy, and to analyze the genetic polymorphisms of C3435T in the MDRl gene. DESIGN, TIME AND SETTING: Factorial designs and comparative observations at the experimental center of the Affiliated Hospital of Qingdao Medical College, Qingdao University between October 2003 and October 2004. PARTICIPANTS: A total of 120 subjects were recruited from the epilepsy clinical department of the Affiliated Hospital of Qingdao Medical College. Four groups (n = 30) were classified according to statistical factorial design: intractable epilepsy, treatment response, no treatment, and normal control groups. METHODS: One-step semi-quantitative reverse-transcription polymerase chain reaction technology was used to test expressions of the MDR1 gene in 120 subjects. C3435T polymorphisms in intractable epilepsy group and normal control groups were analyzed by polymerase chain reaction-restriction fragment length polymorphism. MAIN OUTCOME MEASURES: Expression of MDR1 mRNA in the four groups, and C3435T genetic polymorphisms in intractable epilepsy and normal control groups. RESULTS: MDRl gene expression was increased in the intractable epilepsy group, due to the factor seizures, but not the antiepileptic drugs. However, the interaction between the two factors was not statistically significant. Of the 30 subjects in the intractable epilepsy group, the following genotypes were exhibited: 3 (10%) C/C genotype, 9 (30%) C/T genotype, and 18 (60%) T/T genotype at the site of C3435T, while 4 (13%), 10 (33%), and 16 (53%) subjects were determined to express these genotypes in the normal control group, respectively. C and T allele frequency were 25% and 75% in the intractable epilepsy group, and 30% and 70% in the normal control group, respectively. However, there was no statistical difference between the groups. CONCLUSION: Results demonstrated that seizures, not antiepileptic drugs, induced MDR1 gene expression in intractable epilepsy. Genetic polymorphisms of C3435T in the MDR1 gene did not contribute to the development of multidrug resistance in patients with intractable epilepsy.
文摘BACKGROUND: The biochemical hypothesis of dopamine hyperfunction in the brain can explain the pathological mechanisms of schizophrenia. Surgery is performed based on limbic system circuit theory correspondence to above-mentioned hypothesis. Stereotactic surgery for the treatment of mental disorders is related to stereotactic surgery that influences the Papez circuit. OBJECTIVE: To observe the effects of stereotactic multi-target limbic leucotomy on the improvements in memory, intelligence and psychiatric symptoms in the treatment of intractable psychiatric disease. DESIGN: Self-control case analysis and follow-up of therapeutic effects. SETTING: Department of Neurosurgery, First Hospital, Hebei Medical University. PARTICIPANTS: Thirty patients with intractable psychiatric disease, who received stereotactic surgery in the Department of Neurosurgery, First Hospital, Hebei Medical University between July 2002 and August 2005, were included in this study. The patients, 21 males and 9 females, all met the diagnostic criteria of intractable psychiatric disease, determined by the national psychosurgery cooperation team in 1998. Informed consents for surgery and clinical follow-up exams were obtained from patients and/or patients' relatives (guardians). METHODS: In 30 patients with intractable psychiatric disease, limbic leucotomy was performed by stereotactic technique. Multi-target radiofrequency hyperthermia was performed in the intracranial amygdaloid nucleus, anterior limb of internal capsule, callosal gyrus, among other regions. The therapeutic effects of patients were evaluated by Brief Psychiatric Rating Scale (BPRS) before surgery, and 6 months, 1, and 3 years after surgery. The Wechsler Adult Intelligence Scale (WAIS) and Clinical Memory Scale (CMS) were used to assess memory and intelligence before and after surgery. MAIN OUTCOME MEASURES: Memory, intelligence, and psychiatric symptoms of patients before and after operation. RESULTS: Thirty patients were included in the final analysis. (1) Memory and intelligence changes of patients: CMS and WAIS showed there were no significant differences in the changes of brain functions before and after operation (P 〉 0.05). (2) Improved degree of symptoms: 5 patients were cured, 8 patients significantly improved, 8 patients improved, 3 patients had no changes, and 6 patients had recurrent symptoms. CONCLUSION: Stereotactic multi-target limbic leucotomy can improve the symptoms of patients with intractable psychiatric disease. Follow-up results showed that memory and intelligence were not noticeably improved after surgery.
文摘BACKGROUND Implant vagus nerve stimulation is an adjunctive treatment for intractable epilepsy when patients are not suitable for resective surgery.AIM To identify the safety and efficacy of vagus nerve stimulation in children with intractable epilepsy and analyze the effects on different epilepsy syndromes.METHODS Eligible children with intractable epilepsy were admitted to the study.We collected data from preoperative assessments as the baseline.During the followup time,we recorded the process of seizures(frequency,duration,and seizure type),the changes of drugs or parameters,the complications,etc.The mean reduction rate of seizures,response rate,and McHugh scale were chosen as the outcomes.RESULTS A total of 213 patients were implanted with Tsinghua Pins vagus nerve stimulators,and the average age was 6.6 years.In the follow-up time of postoperative 3 mo,6 mo,12 mo,18 mo,and 24 mo,the average reduction rate was 30.2%,49.5%,56.3%,59.4%,and 63.2%,while the response rate was 21.8%,62.5%,57.1%,69.2%,and 70.7%.In addition,implanted vagus nerve stimulation had different effects on epilepsy syndromes.The reduction rate of West syndrome increased from 36.4%(postoperative 6 m)to 74.3%(postoperative 24 m).The reduction rate of Lennox-Gastaut syndrome improved from 25.4%to 73.1%in 24 mo.The chi-square test of the five efficacy grades showed P<0.05.The comparison between the 3-mo follow-up and the 6-mo follow-up showed P<0.05,and the comparison between the 6-mo follow-up and the 24-mo follow-up showed P>0.05.CONCLUSION Vagus nerve stimulation is safe and effective in children with intractable epilepsy,and the seizure reduction occurred in a time-dependent manner.Moreover,patients with West syndrome may get the most benefits.
文摘Background: The effect of Vagus nerve stimulation (VNS) therapy following major resective surgeries has been reported. However, the effect of VNS therapy following multiple-subpial-transections (MST) has not been reported. The objective of this paper is to examine the beneficial effect of VNS therapy following MST. Methods: There are 22 patients aged 10 - 55 years. Male/female distribution is 11/11 and follow-up is 24 - 148 months (median of 120 months). Seizure foci were bilateral in 9 patients, multi-lobar (unilateral) in 12 patients and single-lobar in 1 patient. MST was performed over broad areas in and around the seizure foci. VNS implantation was done when the response to MST procedure was poor (1 patients), or there was recurrence of seizures (21 patients). Interval between MST and VNS implanttation varied from one month to three years (median of 2 years). Results: Thirteen patients (59%) are seizure free (Engel’ Class I), 8 (36.5%) have greater than 90% reduction in seizure frequency (Class II), and 1 (4.5%) has between 50% - 90% reduction in seizure frequency (Class III). Conclusion: The results show that VNS therapy produced meaningful improvement in seizure outcome in all patients with extra-temporal seizures that had inadequate response to MST.
基金supported by grants from National Natural Science Foundation of China(No.81241129/H0913)Guizhou Province Governor Special Funds(No.1065-09)and Guizhou High-level Personnel Scientific Funds(No.TZJF-2010-054)
文摘Synaptic vesicle protein 2A(SV2A) involvement has been reported in the animal models of epilepsy and in human intractable epilepsy. The difference between pharmacosensitive epilepsy and pharmacoresistant epilepsy remains poorly understood. The present study aimed to observe the hippocampus SV2 A protein expression in amygdale-kindling pharmacoresistant epileptic rats. The pharmacosensitive epileptic rats served as control. Amygdaloid-kindling model of epilepsy was established in 100 healthy adult male Sprague-Dawley rats. The kindled rat model of epilepsy was used to select pharmacoresistance by testing their seizure response to phenytoin and phenobarbital. The selected pharmacoresistant rats were assigned to a pharmacoresistant epileptic group(PRE group). Another 12 pharmacosensitive epileptic rats(PSE group) served as control. Immunohistochemistry,real-time PCR and Western blotting were used to determine SV2 A expression in the hippocampus tissue samples from both the PRE and the PSE rats. Immunohistochemistry staining showed that SV2 A was mainly accumulated in the cytoplasm of the neurons,as well as along their dendrites throughout all subfields of the hippocampus. Immunoreactive staining level of SV2A-positive cells was 0.483±0.304 in the PRE group and 0.866±0.090 in the PSE group(P〈0.05). Real-time PCR analysis demonstrated that 2-ΔΔCt value of SV2 A m RNA was 0.30±0.43 in the PRE group and 0.76±0.18 in the PSE group(P〈0.05). Western blotting analysis obtained the similar findings(0.27±0.21 versus 1.12±0.21,P〈0.05). PRE rats displayed a significant decrease of SV2 A in the brain. SV2 A may be associated with the pathogenesis of intractable epilepsy of the amygdaloid-kindling rats.
基金partly supported by grants from CONICET(PIP201-101-00576)Fundación RenéBarón
文摘Neuropathic pain,a type of pain arising after direct damage or disease of the nervous system,is often intractable and challenges the search of effective therapeutic strategies.In particular,neuropathic pain is a very frequent sequel of spinal cord injury(SCI)and a decisive contributor to decreased quality of life.
基金supported by the National Science&Technology Pillar Program during the 12th Five-year Plan of China(No.2012BAI12B02)the 11th Five-year Plan of China(No.2007BAI18B13)National Natural Science Foundation of China(No.30872865)
文摘This study looked into the efficacy of a modified titration protocol of intratympanic gentamicin injection(ITG) in the patients with unilateral intractable Ménière's disease(MD). Modified titration protocol of ITG at a low dose(20 mg/m L) was administered to 10 patients with definite unilateral intractable MD. After initial first two fixed ITGs on weekly basis,the patients might or might not be given any more injections,depending on the appearance of unilateral vestibular loss(UVL). ITG was terminated if the patients satisfied the criteria of UVL. All patients were followed-up for at least two years. The effects of ITG on the vertigo attack,functional level scores and postural balance were evaluated. Of the 10 cases,8 showed the sign of UVL after receiving initial two ITGs and were not given any more intratympanic injections,and the other 2 patients were administered three ITGs. A two-year follow-up revealed that complete and substantial vertigo control was achieved in 9 cases,and limited vertigo control in 1 patient. Hearing level was lowered in 2 patients. The posture stability and functional level scores were improved. Our study showed that the modified titration protocol of ITG at a low dose could effectively control vertigo in patients with unilateral intractable MD.
文摘Neurodermatitis, a commonly seen, intractable dermal disease. is characterized by severe paroxysmal itching, lichenoid changes and other symptoms of chronic inflammation of skin. Most patients are accompanied by neurasthenic symptoms. The predilection sites of the disease, generally speaking. include the neck, the nuchal region, the popliteal fossa, the sacral region. the ventral surface of the abdomen, the perineum, etc. Neurodermatitis may