2011年 · 第49卷第08期

Drug therapy is the control of epilepsyThe primary means of clinical onset, even when undergoing surgical treatment for drug-refractory epilepsyChildren still need to continue to take antiepileptic after surgeryantiepileptic drugs (AEDs). At the same time, with the continuous advent of new AEDs, on the basis of rational drug selection, 70% or even a higher proportion of epilepsyEpisodes are finally under complete and lasting control^[1,2]。 However, the adverse reactions of AEDs may become the direct cause of reducing the quality of life of children and even treatment failure. AEDS-related adverse reactions often have obvious age and individual differences. Physicians should be familiar with the main clinical characteristics of AEDs adverse reactions and their key countermeasures. Whenever a new AEDs is selected, parents should be informed of the possibility of relevant adverse reactions, and regular follow-up should be made to help them make correct judgment and treat it in time, so that more children can acquire epilepsy without or with minimal adverse reactions.Lasting, complete, or substantially complete control of the attack.

convulsion and convulsive epilepsy(epilepsy) status is an emergency condition often encountered in clinic. Every pediatrician should form a clear and complete concept and master the correct standardized treatment process, so as to be able to better deal with this acute and severe disease. Therefore, this article intends to focus on convulsions and convulsive epilepsyAll aspects of emergency treatment of persistent state are analyzed in combination with literature and clinical practice for the reference of pediatric colleagues.

Minimal residual disease (MRD) is a state in which a trace amount of leukemia cells remain in the body of children with acute lymphocytic leukemia (ALL) after treatment and reaching a complete clinical remission according to the currently established efficacy standard (less than 5% of primary or naive tumor cells in bone marrow, and the patient's condition is completely controlled). Is an important independent prognostic indicator of ALL^[1]It has been confirmed that the level of MRD in children is of great significance in the evaluation of efficacy, prognosis judgment and recurrence monitoring of ALL, as well as the purification of grafts during hematopoietic stem cell transplantation. Many foreign advanced treatment groups have adopted MRD levels for the risk stratification and prognosis assessment of ALL in children, and achieved good results^{[2,3,4,5,6,7,8]}。 At present, domestic and foreign childhood leukemia cooperative groups have taken the detection of MRD as one of the important risk factor evaluation indicators, so as to stratify the risk of MRD levels at the early and end of induction treatment, and incorporate adjustment treatment into the treatment plan^[9,10]To achieve maximum efficacy while reducing the toxicity of chemotherapy drugs, intensive treatment is given to children prone to relapse, and the survival rate and quality of life of children are improved.

EpilepsyIt is the most common chronic central nervous system disease in children, with about 50 million patients worldwide^[1]。 Drug therapy is the treatment of choice, and about 70% of patients can control their attacks with drug therapy^[2]。 There are many problems in the drug treatment process, such as: How to choose the appropriate individualized treatment dose? How to predict possible adverse drug reactions? How to explain the same epilepsyDifferent pharmacotherapeutic effects in children with syndrome? While many factors affect the course of treatment with drugs, genetic factors certainly play an important role in this^[3]。 pharmacogenetics is a discipline that studies the different effects of individual gene variations on drug metabolism, efficacy and adverse reactions. Its ultimate goal is to predict the efficacy of drugs and the risk of adverse reactions according to the individual's genotype before patients take drugs, so as to realize individualized medication. This paper mainly focuses on genetic variation to combat epilepsyThe effect of antiepileptic drugs (AEDs) on clinical efficacy and the impact on the risk of adverse reactions were reviewed.

Sepsis is a systemic inflammatory response whose symptoms are produced by the body's defenses rather than by invasive pathogens. Severe sepsis is on the basis of systemic inflammatory response syndrome (SIRS) caused by infection, complicated by organ dysfunction, tissue hypoperfusion, or hypotension^[1], whose characteristic pathophysiological changes include excessive systemic inflammatory response, activation of coagulation function, and microvascular dysfunction, which promote each other, leading to uncontrolled progression of sepsis^[2]。 More and more studies have confirmed that the activation and dysfunction of endothelial cells play an important role in their development. Recent studies on the functional status of endothelial cells in severe sepsis are summarized as follows.

Example 1Female, 10 months. Progressive abdominal swelling for 5 months. Physical examination: All abdominal swelling, fixed mass touched in the middle and upper abdomen, medium-hard texture, unclear boundary. Imaging showed a large mixed mass of high, medium and low density in the middle and upper abdomen, with smooth edges and clear boundaries. Cord-shaped, small massive high-density calcification and irregular low-density fat shadows were seen in it. Blood test: alpha-fetoprotein (AFP) 53.96 μ g/L. Intraoperatively, the mass was located under the serosa of the posterior wall of the stomach, with a size of about 22 cm ×18 cm ×18 cm, and was cystic-solid. The mass was completely dissected and removed. The giant examination showed that the surface of the mass was smooth, the section was cystic and solid, and mucus and bone tissue were visible in the sac. The microscopic tumor consists of three differentiated mature tissues derived from germ layer, among which more brain tissues are seen. Pathological diagnosis: mature teratoma of the posterior wall of the stomach. Postoperative serum AFP<7.02 μ g/L, no recurrence after 7 years follow-up.

The child was a male, 4 years and 7 months old. He was admitted to hospital for shortness of breath, multiple bone and joint pain, deformity for more than 2 years, and rash for more than 1 year. More than 2 years before admission, shortness of breath, no fever, cough, cyanosis, etc. Multiple joint pain throughout the body, involving both large and small joints, accompanied by skin itching, gradual swelling of both knee joints, especially on the right side, and gradual limitation of movement of the right knee joint. More than a year ago, the back of the left hand and the little fingers of both hands gradually swelled, and red maculopapular rash appeared, which merged into pieces, followed by herpes, accompanied by itching and no pain. After the herpes ruptured, there was yellow clear liquid, and scar formed after scabbing. Similar rashes appeared successively on the extension of the left elbow, the nose, the left ear and the chest wall. The anterior chest wall gradually bulges, and shortness of breath aggravates. I have sought medical treatment repeatedly, but the diagnosis is unclear and the treatment does not improve. More than 4 months before admission, X-rays of the joints and bones of the limbs were performed in the local hospital, which showed "asymmetric development of both upper and lower limbs, hypoplasia of the caudal vertebra, uneven density of the left radius, and disordered trabecular bone arrangement. Osteoporosis of the lower segment of the left humerus may be possible. Morphological and structural abnormalities of the right tibia, bone fibrous dysplasia? Obvious swelling around the right knee joint, reduced density of the patella, infection? Other? Lower femoral density decreased ". Chest X-ray showed" left pleural effusion, possible left lung infection, wedge deformation of thoracic 7 and 12 vertebrae ".

The child is a male, 3 years old and 8 months old, a rural native in Jiangsu Province, who has been living there since birth. On January 2, 2011, he was admitted to hospital with "cough for 2 days, aggravated with asthma for 1 day". One month before admission, he was diagnosed with nephrotic syndrome due to "edema of both lower limbs and scrotum", and was treated with oral prednisone and ready-for-use traditional Chinese medicine for 3 weeks. Seven days before the onset of this disease, more than a dozen chickens in his home and neighbors died in a short time. The child had a history of contact with poultry and pigs, dead birds and a history of eating sick chickens.

The so-called discipline has two meanings: First, it is the subject and branch of the knowledge system. The difference between it and professional is that it is biased in terms of knowledge system, whereas professional is biased in terms of social occupation field. Therefore, a specialty may require the synthesis of multiple disciplines, while a discipline can be applied in different areas of specialty. The second meaning of discipline is the functional unit of teaching and scientific research in colleges and universities, which is the relative definition of teachers' teaching and scientific research business^[1]。 The "discipline construction" in this paper refers to the academic promotion and development in a specific research field; "Development of clinical departments (including hospitals)" generally refers to various medical, preventive and related activities carried out by medical units to meet the needs of society. "Discipline construction" and "clinical department development" are internally related, but each has different connotations. Generally speaking, good discipline construction must be based on the development of excellent clinical departments, and the sustainability of clinical department development must be supported by discipline construction^[2]。 Chen Zhu, Minister of Health, proposed at the 2009 National Medical Administration Work Conference: Start the construction of key clinical specialties, create a number of nationally renowned key clinical specialties, and guide hospitals to take the road of connotation development. At the same time, he also believes that discipline construction promotes the connotation of public hospitals, gives full play to specialist characteristics on the basis of improving comprehensive ability, and solves the problem of diagnosis and treatment of difficult and severe cases. This meeting clearly put forward the initiation of the construction of key clinical specialties and the important role of discipline construction in it^[3]。

Wernicke's encephalopathy is an acute neurological disease caused by VitB1 deficiency, which can be fatal or have serious neurological sequelae untreated. After timely recognition and treatment with VitB1, the symptoms can be quickly relieved and the prognosis is good. According to foreign reports, the incidence (from retrospective autopsy data) in adult hospitalized children ranges from 0.5% to 2.8%. Among them, only 15% of the children had a definite diagnosis before their lives. Pediatric statistics are not available, and estimates are similar to adults. Adult Wernicke's encephalopathy typically has triple symptoms: psychiatric symptoms (such as confusion, coma, and even convulsions, etc.); Ocular symptoms (such as ophthalmoplegia, nystagmus, eyelid ptosis, etc.) and ataxia. Some children have hypothermia, aphonia and other symptoms. MRI or CT abnormalities during acute episodes are helpful in diagnosis. MRI often shows abnormal white matter signals around bilateral papillary bodies, tectum, aqueduct III and IV ventricles in the brain (T1W is low signal, T2W is high signal change).

For this case, which I have been involved in diagnosing and treating, I want to analyze it from a philosophical level. The case reported in this article, at the time of life-threatening, was internally caused by thiamine deficiency, and infection was a trigger and an external cause. Internal reason is the fundamental driving force and source of the development of things, it determines the direction of the development of things, and external reason can only play a role if it acts on the inside of things. In this case, long-term oral administration of "deep-sea fish oil", especially 2 capsules per day for children 1 month before illness, is also an external cause that cannot be ignored, which leads to and accelerates thiamine (vitamin B1) deficiency. In the development of this disease, both internal and external causes are important, but internal causes are the main, fundamental and the basis for change. When vitamin B1 was given according to clinical symptoms and typical head MRI findings, the liver function of the children returned to normal within 12 hours after treatment, the seizures were relieved, and the consciousness of the children improved 2 days after treatment. Whether the treatment is effective or not depends on whether it grasps the main contradiction at that time. If it grasps this main contradiction, all problems will be solved. Because a disease often has not only multiple symptoms and signs, but also a person can suffer from multiple diseases at the same time, it shows that children have multiple diseases. There must be a major contradiction among many kinds of contradictions. Whether we can grasp the main contradiction still needs us to constantly summarize, study and improve in our work.

Anti-epilepticantiepileptic drugs (AEDs) are a group of drugs approved by the State Food and Drug Administration (SFDA) to control epilepsyDrug for the attack. Clinically used AEDs include traditional AEDs and a variety of new AEDs marketed after the 1980s. Traditional AEDs commonly used in clinical practice include phenobarbital (PB), sodium valproate (VPA), carbamazepine (CZB), phenytoin (PHT), clonazepam (CZP), etc. None of the above traditional AEDs have strict age restrictions in the instructions. At present, new AEDs that are available and commonly used in the Chinese market include topiramate (TPM), oxcarbazepine (OXC), levetiracetam (LEV), lamotrigine (LTG), gabapentin (GBP) and zonisamide (ZNS), etc. Although the above-mentioned new AEDs have strict age restrictions in the instructions, after the advent of various new AEDs, there are cases of age indication external use at home and abroad, that is, off-label drugs (off-label). Off-label medication is when a medication is prescribed for use other than the SFDA-approved instructions. Off-label medications include medications beyond the age indication range and medications beyond the disease indication range.

EpilepsyIt is a kind of common disease of children's nervous system, with various causes and complex manifestations, so it is one of the most difficult diseases in clinical diagnosis. Non-epilepticnoepileptic seizures (NES) refer to various paroxysmal behavioral, motor, or sensory events that are not caused by abnormal electrical activity in the cerebral cortex^[1]。 Misdiagnosis of NES as epilepsyThe condition occurs frequently in clinical practice, causing pediatricians and epilepsySpecialist doctors attach importance to it, and now the relevant situation is reviewed and analyzed.

A male, 8 years old and 3 months old, was admitted to the hospital mainly for "persistent cough for 7 months". Seven months before admission, the child developed persistent cough without obvious trigger, white sticky sputum, large amount, accompanied by shortness of breath, occasional wheezing attacks, which aggravated when lying on the bed at night. At that time, there was no hemoptysis, fever, abdominal pain, diarrhea, and rash. The local hospital successively treated it according to "asthma and pneumonia", and gave antibiotics for 2 months and hormones for 1 week. Shortness of breath and wheezing improved, but cough and sputum did not improve. Chest X-ray and chest CT examination were performed 5 months and 4 months before admission, respectively. Considering bronchitis, intermittent intravenous drip of "cefoperazone sulbactam" and "azithromycin" and nebulized inhalation of "budesonide, ipratropium bromide and salbutamol" were treated for 3 months. The child's cough improved and sputum disappeared. Twenty days before admission, the child's cough worsened again, and sputum appeared again. He once sputtered out gray-yellow sticky sputum, without hemoptysis, and the routine blood test was roughly normal. He was intermittently treated with cephalosporin antibiotics and "azithromycin" for more than ten days, but his condition did not improve, and he appeared with low fever, with the highest body temperature of 37.8 ℃, in the afternoon and at night, without night sweats and fatigue. Three days before admission, the blood routine was reviewed for abnormal white blood cells, increased eosinophils, and chest X-ray showed aggravation of intrapulmonary lesions. He was admitted to the hospital for further diagnosis and treatment.