[Clinical diagnosis of group A streptococcal pharyngitis and progress in the application of scoring systems]. / A族链球菌咽炎的临床诊断及评分制的应用进展.

Pharyngitis can be caused by various pathogens, including viruses and bacteria. Group A streptococcus (GAS) is the most common bacterial cause of pharyngitis. However, distinguishing GAS pharyngitis from other types of upper respiratory tract infections is challenging in clinical settings. This often leads to empirical treatments and, consequently, the overuse of antimicrobial drugs. With the advancement of antimicrobial drug management and healthcare payment reform initiatives in China, reducing unnecessary testing and prescriptions of antimicrobial drugs is imperative. To promote standardized diagnosis and treatment of GAS pharyngitis, this article reviews various international guidelines on the clinical diagnosis and differential diagnosis of GAS pharyngitis, particularly focusing on clinical scoring systems guiding laboratory testing and antimicrobial treatment decisions for GAS pharyngitis and their application recommendations, providing a reference for domestic researchers and clinical practitioners.

Case Report: Infant-onset Degos disease with nervous system involvement and a literature review.

Degos disease also known as malignant atrophic papulosis (MAP), is an autoinflammatory disease that mainly affects small- to medium-sized arteries. Gastrointestinal and nervous system are most commonly affected systems. Herein, we reported a case of Degos disease with disease onset during infantile and had severe neurological involvement.

Lipid profiles in patients with juvenile idiopathic arthritis: a systematic literature review and meta-analysis.

OBJECTIVE: The purpose of this study was to comprehensively evaluate the lipid profiles in patients with juvenile idiopathic arthritis (JIA). METHODS: The literature and relevant reviews were searched for published clinical studies on the relationship between JIA and blood lipid levels. The Newcastle-Ottawa scale (NOS) was applied to evaluate the risk and methodological value of the included case‒control and cohort studies. Standardized mean differences (SMDs) and 95% confidence intervals were derived for all variables with adequate unprocessed data. This meta-analysis followed the Preferred Reporting Items for Systematic Reviews and Meta-analysis (PRISMA) guidelines. RESULTS: In total, 16 studies were incorporated through screening. The analysis findings revealed that the levels of very low-density lipoprotein cholesterol [SMD=-0.411, 95% CI (-0.774~-0.048), P = 0.026], high-density lipoprotein cholesterol [SMD=-0.528, 95% CI (-0.976~-0.079), P = 0.021], and apolipoprotein A1 [SMD=-1.050, 95% CI (-1.452~-0.647), P = 0.000] in JIA patients were statistically lower than those observed in healthy controls. The level of low-density lipoprotein cholesterol [SMD = 0.202, 95% CI (0.003 ~ 0.400), P = 0.046] was significantly higher in JIA patients than in healthy controls. In JIA patients, body mass index [SMD=-0.189, 95% CI (-0.690 ~ 0.311), P = 0.459], high-density lipoprotein [SMD =-1.235, 95% CI (-2.845 ~ 0.374), P = 0.133), low-density lipoprotein [SMD = 0.616, 95% CI (-0.813 ~ 2.046), P = 0.398), triglycerides (SMD = 0.278, 95% CI (-0.182 ~ 0.738), P = 0.236], total cholesterol [SMD=-0.073, 95% CI (-0.438 ~ 0.293), P = 0.696] and apolipoprotein B levels [SMD = 0.226, 95% CI (-0.133 ~ 0.585), P = 0.217] were not significantly different from those in healthy controls. CONCLUSIONS: The outcomes of this meta-analysis suggest that dyslipidemia is common in JIA patients compared to healthy controls. Patients with JIA have a significantly increased risk of atherosclerosis and cardiovascular disease later in life.

[Paying attention to the epidemic of group A Streptococcus infections in multiple European and American countries]. / å ³æ³¨æ¬§ç¾Žå¤šå›½Aæ—é“¾çƒèŒæ„ŸæŸ“ç–«æƒ .

At the end of 2022, the World Health Organization reported an increase in group A Streptococcus (GAS) infections, such as scarlet fever, in multiple countries. The outbreak primarily affected children under 10 years old, and the number of deaths was higher than anticipated, causing international concern. This paper reviews the current state of the GAS disease outbreak, its causes, and response measures. The authors aim to draw attention from clinical workers in China and increase their awareness and vigilance regarding this epidemic. Healthcare workers should be aware of the potential epidemiological changes in infectious diseases that may arise after the optimization of control measures for coronavirus disease 2019 to ensure children's health.

Research progress in drug therapy of juvenile idiopathic arthritis.

BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most common chronic rheumatic disease in children. With the gradual expansion of the incidence of JIA in the population, the pathogenesis and treatment of JIA were further explored and analyzed, and JIA has achieved some success in drug therapy. DATA SOURCES: A systemic literature search was conducted on PubMed, Cochrane Library, EMBASE, ISI Web of Science, the US National Institutes of Health Ongoing Trials Register, and the EU Clinical Trials Register. Through the searching of clinical trials of JIA in recent years, we summarized the progress of the clinical treatment of JIA. RESULTS: The main treatment drugs for JIA include non-steroidal anti-inflammatory drugs, glucocorticoids, disease-modifying antirheumatic drugs and biological agents. So far, a variety of biological agents targeting the cytokines and receptors involved in its pathogenesis have been gradually approved for JIA in many countries. The application of biological agents in JIA showed good efficacy and safety, bringing unprecedented experience to children and adolescents with JIA. CONCLUSIONS: The potential and advantages of biologic agents in the treatment of JIA are significant, and the application of biologic agents in the treatment of JIA will be more and more common.

Analysis of clinical manifestations and treatment in 26 children with fibrodysplasia ossificans progressiva in China.

BACKGROUND: Fibrodysplasia ossificans progressiva (FOP) is a rare and disabling heritable connective tissue disease that is difficult to treat. This study seeks to explore the clinical characteristics, clinical manifestations, treatment and prognosis of FOP to provide a clinical basis for its early diagnosis and treatment. METHODS: Twenty-six children with FOP were retrospectively analyzed in terms of their onset, clinical manifestations, auxiliary examinations and treatment. RESULTS: Among the 26 cases, the youngest age of manifestation of mass was 8 days after birth, and the average age was 3 years and 2 months. The peak age was 2-5 years old. Inflammatory mass and toe-finger deformity are the main early clinical manifestations of the disease. These inflammatory masses often lead to hard osteogenic deposits that initially mainly involve the central axis, such as the neck (22/26, 84.6%), back (20/26, 76.9%), and head (13/26, 50%). Toe-finger deformity mainly manifests as symmetrical great toe deformity, or short and deformed thumb and little finger. The diagnosis of FOP requires typical clinical manifestations or ACVR1 gene detection. The main therapeutic drugs for FOP include glucocorticoids and non-steroidal anti-inflammatory drugs. Although not compliant with the recommended medical management of FOP, in our clinical practice children with uncontrollable illness could be treated using a variety of immunosuppressive agents in combination. CONCLUSIONS: FOP is a rare autosomal dominant heritable disease. The main clinical manifestations observed in this study were recurrent inflammatory mass and toe-finger deformity. If the diagnosis and treatment are not performed in a timely manner, serious complications are likely to affect the prognosis. Therefore, early diagnosis and active treatment should be performed.

[Value of serum ferritin in measuring the activation and prognosis in systemic onset juvenile idiopathic arthritis].

OBJECTIVE: To explore the correlation of serum ferritin (SF) and systemic onset juvenile idiopathic arthritis (SOJIA) so as to determine the prognostic values of SF for SOJIA. METHODS: All samples were collected from 92 juvenile idiopathic arthritis (JIA) patients at Beijing Children's Hospital between February 2005 to September 2012. Their age range was 2-15 years. There were macrophage activation syndrome (MAS, n = 25), polyarticular JIA (n = 33) and oligoarticular JIA (n = 30). And 47 healthy children and another 30 with acute infective diseases were selected as control groups respectively. Blood samples were collected and SF was measured in different disease phases.Other parameters include leucocyte, hemoglobin, platelet, C-reactive protein and erythrocyte sedimentation rate.Statistics of SF level at different groups as well as at different disease phases were performed. RESULTS: The SF level of active phase SOJIA patients was significantly higher (P < 0.01) than that in Other groups. And its level in the active phase of SOJIA was significantly higher than that in patients during the recovery phase. The SF level in patients with MAS was significantly higher than that in those without MAS. CONCLUSION: Correlated with the course of SOJIA, the level of SF may judge the disease activity and predict the outcomes of SOJIA.

[Clinical efficacy of mycophenolate mofetil in the treatment of systemic-onset juvenile idiopathic arthritis].

OBJECTIVE: To evaluate the clinical efficacy of mycophenolate mofetil (MMF) in the treatment of systemic-onset juvenile idiopathic arthritis (SoJIA). METHODS: Thirty-five patients with a confirmed diagnosis of SoJIA who had received initial treatment were randomly divided into control (n=15), MMF1 (n=7) and MMF2 groups (n=13). The control group received conventional treatment, the MMF1 group received MMF after 2 weeks of conventional treatment that had not led to remission, and the MMF2 group received combination therapy with non-steroidal anti-inflammatory drugs, prednisone and MMF. Symptoms, signs, laboratory indices, and adverse events were observed after 2, 4, and 12 weeks of treatment, and follow-up was performed for 3-6 months. RESULTS: Before treatment, the MMF2 group had a significantly longer disease course than the control group (P<0.05). After 2 weeks of treatment, the MMF1 and MMF2 groups had a significantly lower prednisone dose and erythrocyte sedimentation rate (ESR) than the control group (P<0.05). The MMF1 group had significantly higher body temperature than the other two groups (P<0.05). After 4 weeks of treatment, the MMF1 group had a significantly lower prednisone dose and ESR than the control group (P<0.05). The MMF2 group had a significantly lower prednisone dose, body temperature (recovery to normal), white blood cell count, ESR and serum ferritin concentration than the control group (P<0.05). Body temperature was significantly lower in the MMF2 group than in the MMF1 group (P<0.05). No adverse events were observed in either the MMF1 or MMF2 groups during treatment. CONCLUSIONS: Combination therapy with MMF can lead to better control of the patient's condition, more rapid relief of clinical symptoms and reduced glucocorticoid dose. The therapy with MMF is safe in children.

[Effects of positive end expiratory pressure on pulmonary surfactant in patients undergoing one-lung ventilation].

OBJECTIVE: To evaluate the effect of positive end expiratory pressure on the bronchoalveolar lavage fluid (BALF) in pulmonary surfactant (PS) of patient during one lung ventilation anesthesia. METHODS: Patients undergoing Bullae resection were anesthetized with treatment (n = 12) or control (n = 12) combined anesthesia. Total phospholipid (TPL), saturated phosphatidylcholine (SatPC) and total protein (TP) in the bronchoalveolar lavage fluid (BALF) were measure. The ratio of SatPC/TPL% and SatPC/TP% represented the activity of PS. RESULTS: The thoracic surgery pulmonary surfactant significantly reduced, PEEP can prevent the decrease, but with decreased cardiac output. CONCLUSION: The end-expiratory pressure breathing can maintain the level of pulmonary surfactant to protect lung function.

[Detection of group B streptococcus in the cases died of neonatal pneumonia].

OBJECTIVE: From the 1970s, group B streptococci (GBS) have been widely recognized as an important pathogen in neonatal infectious disease, and it emerged as the leading cause of neonatal morbidity and mortality in the Western world. However, there are few data on the prevalence of neonatal GBS infections in China. The aim of this retrospective study was to estimate whether GBS is an important pathogen in severe neonatal pneumonia, and to develop a method for detection of GBS infections in fatal neonatal pneumonia. METHODS: A total of 234 neonatal cases (0 - 28 days) died in Beijing Children's Hospital from 1953 to 2004 were enrolled in this study. They were divided into two groups. Two hundred cases diagnosed as neonatal pneumonia were assigned to study group and the remaining 34 cases died of neonatal hemolysis or surgical operation without any confirmed infectious diseases were designated as control group. Formalin-fixed, paraffin-embedded lung tissues were used as source for total genomic DNA extraction. PCR and Southern blot analyses were applied to detect GBS specific cfb gene target sequence. And the clinical data of these cases were reviewed as well. RESULTS: In the study group, 52 cases were detected positive for GBS DNA by PCR (26%), 130 cases were positive by Southern blot (65%). In the control group, 1 case was detected positive GBS DNA by PCR (3%), and 6 cases were positive by Southern blot (18%). The positive rate was significantly lower in the control group than that in the study group (PCR, chi(2) = 8.82, P < 0.01; Southern blot, chi(2) = 26.77, P < 0.01). The positive rate in the neonates younger than 7 days (early-onset) was significantly higher than that in neonates older than 7 days (late-onset) (PCR: 37% vs. 13%, chi(2) = 15.537, P < 0.01; Southern blot: 72% vs. 52%, chi(2) = 4.37, P < 0.05). In the positive early-onset cases, 39% of whom were born prematurely (29/74). Out of the 200 cases, 75 had complete clinical data. Neither blood nor lung culture for GBS was performed in any of these cases. But risk factors were identified for 35 cases, such as premature delivery, low birth weight, premature rupture of the membrane and abnormal amniotic fluid. GBS was positive in all these cases. Severe apnea appeared to be a common symptom and was present in most of the early-onset GBS-positive cases, while cough and wheezing were found in most of the late-onset GBS-positive cases. In the control group, one PCR positive case was suffered from malignant teratoma. The other 5 positive cases confirmed by Southern blot were diagnosed as kernicterus, hepatoma, aproctia complicating with cysti-urethral fistula, neonatal physio logical bleeding and aproctia complicated with archo-perineal fistula. CONCLUSION: Group B Streptococcus is an important pathogen in fatal neonatal pneumonia, especially in early-onset cases. southern blot may be a sensitive method to detect GBS infection in archival tissues. In the clinical work, more attention should be paid to the neonates with GBS risk factors. And GBS detection and prevention in neonates should be put into clinical practice.

[Relationship between podocyte molecule's distribution and expression, foot process morphology and proteinuria].

OBJECTIVE: To investigate the association between nephrin, podocin and alpha-actinin of the glomerular podocyte molecules, the morphometric change of podocyte foot process and the development of proteinuria. METHODS: Puromycin aminonucleoside (PAN) nephrosis was established. Immunofluorescence staining, image analysis and real time quantitative PCR were employed to study the distribution and quantitation of glomerular expression of nephrin, podocin and alpha-actinin. Morphometric methods were applied to evaluate the morphology change of podocyte foot processes under electron microscopy. RESULTS: (1) Before the onset of proteinuria, 2 days after PAN injection, the podocyte foot process became swollen nephrin and podocin staining were changed into discontinuous pattern accompanied by the decrease of podocin staining intensity. The foot process became more swollen on day 5,and podocin intensity continued to decrease. Meanwhile, nephrin decreased significantly both in protein intensity and at mRNA level. (2) When heavy proteinuria [(130.8+/-30.7) mg/d, P=0.02] occurred, complete effacement of podocyte foot processes was revealed; both podocin and nephrin staining intensity decreased dramatically(P<0.01), and no linear staining could be observed; nephrin and podocin mRNA gained back. (3) During the recovery of proteinuria, the foot process morphology recovered stepwise; both the intensity of nephrin and podocin increased. When proteinuria disappeared, the podocyte foot process reappeared; podocin immunofluorescence intensity recovered whereas nephrin did not; the intensity of alpha-actinin increased significantly and the distribution changed too. (4) Podocyte foot process volume density correlated negatively with nephrin(r(nephrin)=-0.78, P=0.000 1) and podocin immunofluorescence intensity(r(podocin)=-0.76, P=0.000 1), respectively. CONCLUSION: Before the onset of proteinuria, the first response of podocyte is the swollen foot process, the distribution change of nephrin and podocin and the decreased podocin intensity. There was close relationship between nephrin, podocin protein level and distribution pattern with the development of proteinuria and podocyte foot process effacement, whereas no major role was found for mRNA of nephrin and podocin. Our results suggest that proteinuria might occur after series of events of nephrin and podocin distribution change, their protein and mRNA expression level decrease, and morphology change of podocyte foot process.