2017年 · 第55卷第09期

Under the organization of the Nephrology Group of the Pediatrics Branch of the Chinese Medical Association, and through the collective efforts of experts, we successively published seven evidence-based guidelines for the diagnosis and treatment of common childhood kidney diseases in the Chinese Journal of Pediatrics from 2009 to 2010^{[1,2,3,4,5,6,7]}(2009 Edition Guide for short) and interpretation^{[8,9,10,11,12,13,14]}。 When a third party invited by the Chinese Medical Association conducted AGREE II evaluation/evaluation on hundreds of evidence-based guidelines officially published in China, these guidelines were highly praised.

[Foreword]

[Foreword]

Primary IgA nephropathy (IgA nephropathy) is the most common glomerular disease worldwide and one of the main diseases leading to end-stage kidney disease. In order to standardize the diagnosis and treatment of this disease by pediatricians in China, the Nephrology Group of Pediatrics Branch of Chinese Medical Association organized relevant experts to revise and improve the guidelines in 2015 on the basis of the guidelines for the diagnosis and treatment of primary IgA nephropathy issued in 2010, and formed and published the "Evidence-based Guidelines for the Diagnosis and Treatment of Primary IgA Nephropathy (2016)" (hereinafter referred to as the "2016 Guidelines"). Here, it is intended to explain and explain the outstanding issues and some possible differences in the development of the 2016 Guidelines.

In 2009, the Pediatric Nephrology Group of Pediatrics Branch of Chinese Medical Association formulated the "Evidence-based Guideline for Diagnosis and Treatment of Purpura Nephritis in Children" based on the original diagnosis and treatment routine of purpura nephritis, referring to the latest research results at home and abroad, and according to the principle of evidence-based medicine^[1]It has played a positive role in standardizing the clinical diagnosis and treatment of purpura nephritis in children in China. Due to the continuous publication of new research evidence in recent years, the "Evidence-based Guidelines for the Diagnosis and Treatment of Henoch-Schonlein Nephritis (2016)" (hereinafter referred to as the 2016 Guidelines) published in this issue is based on the 2009 guidelines, through comprehensive literature inquiry, analysis and evaluation of new research evidence, solicitation of opinions from all parties and full discussion of the revised results after reaching consensus, aiming to help clinicians choose the current relatively good diagnosis and treatment methods for children with Henoch-Schonlein Nephritis. The main contents of the guide are presented.

A 10-year-old male was admitted to the Children's Hospital affiliated to Zhejiang University School of Medicine in September 2016 due to "fever for 9 days, cough for 7 days and abdominal pain for 1 day". The child developed fever 9 days ago, body temperature fluctuated around 39 ℃, no headache, vomiting, convulsions, cough 7 days ago, gradually worsening, initially dry cough, then accompanied by yellow phlegm, suspected of "acute pneumonia" and hospitalized in a local hospital and given anti-infective treatment, still with fever and cough. 1 d ago, he developed abdominal pain, located in the left upper abdomen, with persistent dull pain without radiating pain. For further treatment, he was transferred to our hospital.

Examples 1 and 2Identical twin sisters, 4 days old, were admitted to the Department of Neonatology of Pediatric Hospital affiliated to Fudan University in September 2016 due to "postpartum skin tumor-like changes for 4 days". They were the first child and the second child, respectively, with a gestational age of 36^＋1Weeks, birth weight 2.60, 2.45 kg, respectively, natural birth, history of premature rupture of membranes, 1, 5, 10 min Apgar score of 7, 8, 10 (consistent in both cases). After birth, two cases of erythema of different sizes appeared all over the body, which was tough in texture and protruding from the skin surface. Local bullae and crusts were seen on the skin, local bullae were accompanied by ulceration and exudation, and dense sarcoma-like growth was seen on the face. The clinical manifestations of the two children were basically the same. After birth, they were treated with "skin care and anti-infection" in the local hospital (details are unknown), but the skin lesions did not improve, so they were transferred from the local hospital to the neonatal intensive care unit of our hospital on the 4th day after birth for further multidisciplinary diagnosis and treatment. When her mother was young, she had a history of allergy to red papules on her skin after eating seafood. She ate more eggs and seafood during pregnancy, and her mother did not have allergic symptoms such as rash and erythema during pregnancy. His father and grandparents had no history of allergies.

Case 1 female, 3 years and 5 months old, Han nationality. He was seen in Guizhou Children's Hospital in March 2016 because of "repeated skin ecchymosis with epistaxis for more than 3 years". The mother of the child is pregnant 2 and gives birth 2. The child develops the disease 1 month after birth, and it is obvious after 6 months of age. After trauma or strenuous activity, the skin ecchymosis is aggravated, and there is epistaxis from time to time. Many visits to many hospitals inside and outside the province for anemia, normal platelet count, normal coagulation function, unknown diagnosis, 4 times of red blood cell transfusion treatment. My parents were married to my cousin, and my brother had a similar medical history. He died of blood in his stool at the age of 5. Mild anemia appearance, scattered bleeding spots and ecchymosis on the skin of the whole body, no swelling of liver and spleen lymph nodes, no abnormalities in heart, lung, abdomen and nervous system physical examination. Auxiliary examination after admission: white blood cells (WBC) 10.63×10⁹/L, neutrophils (N) 0.43, lymphocytes (L) 0.47, red blood cells (RBC) 2.36×10¹²/L, hemoglobin (HB) 67g/L, hematocrit (HCT) 0.20, platelet (PLT) 127×10⁹/L, percentage reticulocytes 3.6%, absolute value of reticulocytes 73.0×10⁹/L. Blood smear platelets are scattered and do not aggregate. There were no abnormalities in electrolyte, liver and kidney function. The bleeding time was prolonged for 25 min, the blood clot contracted poorly, and the coagulation function was normal; The positive rates of CD41 and CD61 were 0.7% and 1.3% respectively by flow cytometry. Genetic screening of hematological genetic diseases (2nd generation high-throughput sequencing, sent to Beijing Kangxu Medical Laboratory for detection), children with chromosome 17 ITGA2B gene exon 4 c.480C>G:p.S160R homozygous mutation; Both parents had heterozygous mutations at this site. Diagnosis of hereditary thrombocytasthenia, red blood cell transfusion to correct anemia, epistaxis stopped after platelet transfusion, hospitalization for 4 days improved and discharged, following the doctor's advice to avoid trauma after discharge. After 1 year of outpatient follow-up, the symptoms of skin and epistaxis were alleviated, and no hemorrhagic anemia occurred again.

A male child, 11 months old, was admitted to hospital due to "repeated cough and fever for 5 days". Five days before admission, the child developed fever after catching cold, and the body temperature fluctuated between 39 and 40℃, accompanied by cough, which was paroxysmal dry cough, without expectoration, convulsions, vomiting, diarrhea, etc. The next day after the onset of the disease, he went to the local hospital, considered "upper respiratory tract infection", and was given "viarabine" antiviral treatment and "amoxicillin clavulanate potassium" anti-infective treatment for 3 days. He still had repeated fever, and blood biochemical examination showed increased muscle enzymes, mainly creatine kinase (CK), CK 1 898.3 U/L (normal reference range 24~190 U/L). The personal history of the child, the past history is not special, the vaccine is given as planned, and the development is similar to that of children of the same age. One sister is in good health, her parents are not close relatives, and there is no special genetic history in the family.

membranoproliferative glomerulone-phritis (MPGN) is a pathological type of glomerular injury defined according to the histopathological characteristics of renal biopsy. It occurs in children and adolescents between 8 and 16 years old^[1]。 MPGN is a common pathological type of childhood nephrotic syndrome in Turkey and Nigeria. MPGN accounted for 3.3% and 2.8% of kidney transplantation and dialysis patients, respectively^[2]。 Typical pathological changes include proliferation of mesangial cells and stroma, remodeling of capillary walls (insertion of mesangial tissue and formation of "double track sign" of basement membrane), and lobular changes of glomerular capillary haptic components^[3]。 The main clinical manifestation is nephrotic syndrome, which may be accompanied by hematuria, hypertension and renal impairment, often accompanied by persistent hypocomplementemia^[4]。 In recent years, new understanding of the pathogenesis of MPGN has been gained, and great progress has been made in disease classification and treatment. Now we briefly discuss the re-understanding of MPGN.

Warfarin is a vitamin K antagonist, which acts by inhibiting the synthesis of coagulation factors II, VII, IX and X by hepatocytes, and inhibiting the carboxylation of protein C and protein S. Since being approved by the U.S. Food and Drug Administration (FDA) in 1954, warfarin is the most widely used oral anticoagulant worldwide. It is widely used for anticoagulant therapy in adults and children, with about 0.5% ~1.5% of the population receiving this drug every year. In the pediatric cardiovascular field, the use of warfarin anticoagulant therapy for some diseases has gradually become the standard treatment option. However, the clinical application of warfarin in pediatrics has not been long, and the experience and evidence-based basis of warfarin treatment in children are limited. At present, the implementation plan and management of anticoagulant therapy in children are mainly inferred based on adult case studies. The application and management of warfarin in childhood cardiovascular diseases are reviewed as follows.

Duchenne muscular dystrophy (DMD) is an X-linked recessive genetic disease with an incidence of 1/3 600 to 1/6 000 live-born male infants^[1]。 The typical clinical symptoms are slowly progressing muscle weakness with pseudohypertrophy of gastrocnemius muscle, loss of independent walking ability around the age of 12, and death of respiratory failure or heart failure around the age of 20^[1,2]。 DMD is caused by the loss of dystrophin function caused by DMD gene mutation. Anti-dystrophin is a skeletal protein in myocytes with a relative molecular mass of 427 000. It contains four domains: N-terminal domain, central rod region, cysteine-enriched region and C-terminal domain. Among them, the central rod region consists of 24 spectrin-like repeat regions and 4 hinge regions, accounting for 80% of the length of the whole protein chain^[3,4]。 There is no effective treatment for DMD. Clinically, glucocorticoids are mostly used to improve the symptoms of the disease, but they cannot change the final outcome of the disease. The study of myoblast transplantation in the treatment of DMD has stagnated due to poor myoblast migration, low survival rate and excessive immune rejection; The study of myogenic stem cells in the treatment of DMD also has problems such as unclear differentiation and regulation mechanism^[5,6]。 With the continuous development of gene therapy and the advancement of genome editing technology, starting from the root cause of disease-DMD gene has brought new possibilities for the treatment of diseases.

Clinical research papers need to be written according to a fixed format. The basic format of the text usually includes five parts: foreword, object and method, results, discussion and references. If statistical methods are used in the research process, the content of this section needs to be written in "Objects and Methods" with a separate subheading. "Statistical analysis method" is the key for readers to understand the process of research analysis and the method of generating core conclusions. Therefore, we should never copy it mechanically in the writing process, let alone excerpt it from different articles, but should reasonably express all the statistical processing methods involved in this research. There are also some necessary components in the writing process, which should generally include the following contents: sample size estimation, data management, statistical analysis software, statistical analysis set, statistical description, statistical inference and test level.