2017年 · 第55卷第11期

In the past 10 years, important progress has been made in the clinical diagnosis, treatment and research of encephalitis, especially in the etiological diagnosis. The application of new technologies and the accumulation of new knowledge are profoundly changing the traditional clinical decision-making of encephalitis. New diagnostic technologies, including autoimmune encephalitis antibody lineage detection and pathogen identification based on next-generation sequencing technology, have achieved clinical application or are accelerating clinical translation, enabling more and more encephalitis patients to obtain timely and accurate etiological diagnosis, thus making it possible to obtain specific treatment^[1,2]。 At least half of cases of encephalitis 10 years ago could not be diagnosed as a cause-the so-called "most common encephalitis is encephalitis of unknown etiology". The discovery of anti-N-methyl-M-aspartate receptor (NMDAR) encephalitis in 2007 is undoubtedly one of the major milestones in the field of encephalitis^[3], marking a new period in the development of the field of encephalitis.

Steroid-resistant nephrotic syndrome (SRNS) is one of the common kidney diseases in children who can progress to end-stage renal disease. Because of its glucocorticoid resistance, difficult treatment and multiple complications, it is one of the important diseases that threaten children's life and health. In January 2010, Chinese Journal of Pediatrics published "Evidence-based Guidelines for Diagnosis and Treatment of Common Kidney Diseases in Children (Trial) (III): Guidelines for Diagnosis and Treatment of Steroid-Resistant Nephrotic Syndrome"^[1]It has played a positive role in standardizing the diagnosis and treatment of the disease. Due to the continuous publication of new research evidence in recent years, the study group revised the 2010 guidelines (i.e., these guidelines) in 2016 after comprehensively inquiring, analyzing and evaluating new research evidence, soliciting opinions from all parties and reaching a consensus through full discussion, aiming to help clinicians choose the current relatively good diagnosis and treatment methods for children with SRNS. This guide is mainly applicable to clinical pediatricians who have a certain professional foundation in pediatric nephrology and have received professional training or training in pediatric nephrology, especially to provide clinical reference for pediatric nephrologists. In clinical practice, physicians should refer to the principles of this guideline and individualize the treatment of children according to specific conditions.

In order to keep pace with the times and reflect the current best clinical practice evidence, the Nephrology Group of Pediatrics Branch of Chinese Medical Association revised the "Trial of Evidence-based Guidelines for Diagnosis and Treatment of Common Kidney Diseases in Children (III): Guidelines for Diagnosis and Treatment of Steroid-resistant Nephrotic Syndrome" issued in January 2010 through discussion and on the basis of analyzing and evaluating new progress and new evidence^[1]To provide guidance for better promoting and standardizing the diagnosis and treatment of children's kidney disease in China.

The child was a girl, 8 months old. Due to "fever for 12 days, intermittent convulsions for 8 days, and increased sleep for 6 days", he was seen in the Children's Hospital affiliated to the Capital Institute of Pediatrics in May 2017. Fever occurred 12 days before admission, with fever peaks twice/d, maximum body temperature 39.6 ℃, accompanied by mild cough, no convulsions, no vomiting, diarrhea, and no drowsiness. I went to a local hospital, and the blood routine showed that the white blood cells were roughly normal, and the proportion of lymphocytes was mainly. I was given ready-for-use traditional Chinese medicine oral administration for 4 days, and the peak temperature of heat was higher than before, with a peak of heat of 4 to 6 times/d a day. Eight days before admission, convulsions occurred once during high fever, manifested as refusal to respond to calls, staring at both eyes, trembling of the right lower limb, no closure of teeth, perioral cyanosis and incontinence, etc., which relieved spontaneously in a few seconds, and convulsions occurred again 1 h later, with the same manifestations as before, and relieved 1 min after sedation in the local hospital. Re-examination of blood routine showed that white blood cells were elevated, mainly neutral, and "ceftriaxone" was given intravenous drip for 2 days, but the fever did not improve. Six days before admission, drowsiness, less milk intake, and occasional irritability. Perfect lumbar puncture examination suggested that cerebrospinal fluid routine: nucleated cells 0; Cerebrospinal fluid protein 570 mg/L, normal range for sugar and chloride; Human immunoglobulin was given 5 g/d for 2 days. The child had the same sleepiness as before, intermittent irritability, intermittent convulsions, and the attack form was the same as before, accompanied by intermittent limb jitter.

acute necrotizing encephalopathy (ANE) is a kind of acute and explosive severe encephalopathy, which is clinically characterized by convulsions, disturbance of consciousness and symmetrical multifocal brain damage. The mortality rate is as high as 30%, and the survivors mostly leave neurological sequelae of different severity^[1]。 The disease is rare, globally sporadic, and is a group of clinical imaging syndromes of unknown etiology. Most scholars believe that sporadic cases are virus-related febrile diseases with rapid deterioration. In recent years, some cases have been found to be familial and recurrent, and their onset is closely related to RANBP2 gene. The recent research progress of this disease is summarized as follows.

Epilepsy and attention deficit disorder/hyperactivity (ADHD) often begin in childhood and have a significant negative impact on an individual's academic, interpersonal and self-awareness development^[1]。 About 1% of children suffer from epilepsy^[2]Studies have shown that the comorbidity rate of epilepsy and ADHD is 20% ~50%^[3,4,5,6]It has a more obvious negative impact on children's social ability and learning, so it is necessary to treat ADHD, a comorbidity of epilepsy^[7,8]。 Methylphenidate is a classic ADHD treatment drug. It belongs to a central stimulant. It increases the concentration of dopamine and norepinephrine in the synaptic space by inhibiting the central dopamine and norepinephrine transporters, thereby increasing catecholaminergic synaptic transmission^[9]。 Methylphenidate has been applied to comorbid ADHD in children with epilepsy^[10]However, methylphenidate has long been thought to lower the seizure threshold^[11,12]The point of view limits the use of the drug^[13,14,15]。 However, studies have also reported that methyl acetate is safe in children with well-controlled epilepsy and ADHD^[16,17]But there is no literature to give a clear answer to this question.

The significance and value of procalcitonin (PCT) as a marker of sepsis infectivity in intensive care unit (ICU) has been widely recognized by academia and widely used in clinical practice^[1,2,3]。 However, with the deepening of clinical research, PCT has been found to be elevated to varying degrees or even significantly in many children's non-bacterial infectious diseases, which has attracted increasing attention of scholars. This review discusses the clinical significance of PCT detection in non-bacterial infectious diseases in children.

acute promyelocytic leukemia (APL) is the M3 subtype of myeloid leukemia, which is characterized by abnormal proliferation of promyelocytes. 90% of APL have specific cytogenetic abnormality t (15; 17) (q22, q21), which forms PML-RARA fusion gene. The fusion protein encoded by APL can prevent cell differentiation and make a large number of abnormal promyelocytes accumulate in bone marrow. all-trans retinoic acid (ATRA) and arsenic trioxide (ATO) exert therapeutic effects by targeting PML-RARA fusion protein, and the CR (complete remission) rate can reach more than 90%^[1], making APL the best prognostic type of leukemia. t (15; 17) (q22, q21) translocation could not be detected in about 10% of cases by conventional cytogenetic techniques, and it was found that PML-RARA gene rearrangement was caused by complex and occult translocation in 70% of cases by molecular techniques^[2]Therefore, a combination of reverse transcription polymerase chain reaction (RT-PCR) and fluorescence in situ hybridization (FISH) is required to detect PML-RARA. However, RT-PCR and FISH may also have false negatives due to their own limitations, so more accurate techniques are needed to assist in diagnosis. next generation sequencing (NGS) has developed rapidly and been widely used because of its advantages such as speed, accuracy and rich information. It is of great significance in exploring the pathogenesis, accurate diagnosis, selection of treatment strategies, efficacy monitoring and prognosis judgment of APL. The genetic characteristics of APL and the application of NGS in APL are reviewed.

Multi-center clinical studies have the advantages of good representation of research objects and rapid enrollment, and the number of multi-center studies in China continues to rise. Although multi-center clinical research has its unique advantages, the difficulty of implementation cannot be ignored. For example, the rate of enrollment at different sites varies, resulting in cases where some sites have ended enrollment but there are still sites that have not completed enrollment. From the perspective of ensuring the safety of research objects, although ethics committees can play an active role, ethics committees are often established based on a single research structure, and cannot evaluate and monitor the data of other research centers.