Transparency Ecosystem for Research and Journals in Medicine (TERM) working group summarized the essential recommendations that should be considered to review and publish a high-quality guideline. These recommendations from editors and reviewers included 10 components of essential requirements: systematic review of existing relevant guidelines, guideline registration, guideline protocol, stakeholders, conflicts of interest, clinical questions, systematic reviews, recommendation consensus, guideline reporting and external review. TERM working group abbreviates them as PAGE (essential requirements for Publishing clinical prActice GuidelinEs), and recommends guideline authors, editors, and peer reviewers to use them for high-quality guidelines.
Humans ; Practice Guidelines as Topic

Objective:To investigate the factors affecting phenotypes in the patients of methylmalonic acidemia combined with homocysteinemia cblC type with MMACHC c. 609G>A homologous variant. Methods:A retrospective study on the clinical manifestations, complications, treatment, and outcome in 164patients of cblC type with MMACHC c. 609G>A homologous variant was conducted.The patients were diagnosed by biochemical and genetic analysisfrom January 1998 to December 2020. Results:Among the 164 patients, 2 cases were prenatally diagnosed and began treatment after birth. They are 3 and 12 years old with normal physical and mental development. Twenty-one cases were diagnosed by newborn screening. Among them, 15 cases had with normal development. They were treated fromthe age of two weeks at the asymptomatic period. Six cases began treatment aged 1 to 3 months after onset. Their development was delayed. One hundred and forty-one cases were clinically diagnosed. Their onset age ranges from a few minutes after birth to 6 years old. 110 cases had early-onset (78.0%). 31 cases had late-onset (22.0%). Five of them died. 24 patients lost to follow-up. Of the 141 clinically diagnosed patients, 130 (92.2%) with psychomotor retardation, 69 (48.9%) with epilepsy, 39 (27.7%) with anemia, 30 (21.3%) had visual impairment, 27 (19.1%) had hydrocephalus, 26 (18.4%) had feeding difficulties, 7 (5.0%) with liver damage, and 5 (3.5%) with metabolic syndrome. The frequency of hydrocephalus and seizures was significantly higher in the early-onset group. The urinary methylmalonic acid increased significantly in the patients with epilepsy. During the long-term follow-up, the level of plasma total homocysteine in the seizure-uncontrolled group was significantly higher than that in the seizure-controlled group, the difference had a statistical significance ( P<0.05). Conclusion:Most of the patients with MMACHC c. 609G>A homozygous variant had early-onset disease, with a high mortality and disability rate. If not treated in time, it will lead to neurological damage, resulting in epilepsy, mental retardation, hydrocephalus, and multiple organ damage. Pre-symptomatic diagnosis and treatment are crucial to prevent irreversible neurological damage. Neonatal screening and prenatal diagnosis are important to improve the outcome of the patients.

Netrin-1 may protect and repair the damage caused by cerebral infarction, in terms of inhibiting apoptosis and inflammatory, and promoting angiogenesis and axon regeneration, etc. Netrin-1 may associate with the pathogenesis and outcome of cerebral infarction. The application of Netrin-1 in clinic needs more researches.

Objective: Most of the studies on the herb Chuanxiong Rhizoma (CR) have focused on the L-arginine-nitric oxide (NO) pathway, but the nitrate-nitrite-NO (NO

BACKGROUND: Psoriasis is a chronic inflammatory skin disease, affecting about 0.6% of the Chinese population. Many patients are not well controlled by conventional treatments, thus there is need for new treatment regimens. In this study, we assessed the efficacy and safety of secukinumab in Chinese patients with moderate to severe plaque psoriasis. METHODS: This study was a 52-week, multicentre, randomized, double-blind, placebo-controlled, parallel-group, Phase 3 trial. A sub-population of study participants (≥18 years) of Chinese ethnicity were randomized to receive subcutaneous injections of 300 or 150 mg secukinumab, or placebo. The co-primary endpoints were psoriasis area severity index (PASI) 75 and Investigator's Global Assessment (IGA) 0/1 at Week 12. RESULTS: A total of 441 Chinese patients were enrolled in this study. Co-primary outcomes were achieved; 300 and 150 mg secukinumab were superior to placebo as shown in the proportion of patients that achieved PASI 75 (97.7% and 87.2% vs. 3.7%, respectively; P < 0.001), and IGA 0/1 (82.3% and 69.7% vs. 2.7%; P < 0.001) at Week 12. Treatment efficacy was maintained until Week 52. There was no increase in overall adverse events with secukinumab relative to placebo throughout the 52-week period. CONCLUSION: Secukinumab is highly effective and well tolerated in Chinese patients with moderate to severe plaque psoriasis. TRIAL REGISTRATION ClinicalTrials.gov, NCT03066609; https://clinicaltrials.gov/ct2/show/record/NCT03066609.
Antibodies, Monoclonal/therapeutic use* ; Antibodies, Monoclonal, Humanized ; China ; Double-Blind Method ; Humans ; Psoriasis/drug therapy* ; Severity of Illness Index ; Treatment Outcome

Background: Nickel-induced allergic contact dermatitis (Ni-ACD) is a global health problem. More detailed knowledge on the skin uptake of haptens is required. This study aimed to investigate the penetration process and distribution of nickel in skin tissues with late phase and early phase of Ni-ACD to understand the mechanisms of metal allergy. Methods: Forty Hartley guinea pigs were divided into four groups according to the NiSO₄ sensitizing concentration and the NiSO₄ challenged concentration: the 5% NiSO₄-group, 5% to 10% (sensitization-challenge; late phase group); 10% NiSO₄-group, 10% to 10% (sensitization-challenge; early-phase group); and the positive and negative controls. Pathological biopsies were performed on each group. The depth profile of nickel element concentration in the skin of guinea pigs was detected by synchrotron radiation micro X-ray fluorescence spectroscopy (SR-μ-XRF) and micro X-ray absorption near-edge spectroscopy (μ-XANES). Results: In each section, the nickel element concentration in both the 5% NiSO₄-group and 10% NiSO₄-group was significantly higher than that in the negative control group. In the upper 300-μm section of skin for the early phase group, the nickel element concentration was significantly higher than that in the lower section of skin. In deeper sections (<200 μm) of skin, the concentration of nickel in the early phase group was approximately equal to that in the late phase group. The curve of the late phase group was flat, which means that the nickel element concentration was distributed uniformly by SR-μ-XRF. According to the XANES data for the 10% NiSO₄ metal salt solution, structural changes occurred in the skin model sample, indicating that nickel was not present in the Ni²⁺ aqueous ionic state but in the nickel-binding protein. Conclusions This study showed that the distribution of the nickel element concentration in ACD skin tissue was different between the early phase and late phase groups. The nickel element was not present in the Ni²⁺ aqueous ionic state but bound with certain proteins to form a complex in the stratum corneum in ACD model tissue.

Objective To investigate the clinical and antenatal sonographic characteristics of placenta previa accreta after cesarean section. Methods The data of 21 inpatients diagnosed as placenta previa accreta after cesarean section in PUMC Hospital from 2006 to 2016 were retrospectively reviewed. The clinical and ultrasound features were recorded and compared among three placental accreta groups,including placenta accrete group(n=5),increta group(n=12),and percreta group(n=4). The relationship between the placental thickness at the uterine anterior lower segment level and the blood loss of the following cesarean section was tested. Results Of 21 patients,placenta previa was diagnosed by ultrasound in 20 cases(95.2%) and placenta previa accreta was diagnosed in 9 cases(42.9%). Antenatal ultrasound findings included following signs:loss of "clear zone"(15/18,83.3%),myometrial thinning(12/18,66.7%),abnormal placental lacunae(12/19,63.2%),bladder wall interruption(2/18,11.1%),and uterovesical hypervascularity(4/9,44.4%). Myometrial thinning(J-T=64.000,P=0.036),abnormal placental lacunae(J-T=74.500,P=0.032) and the placental thickness at the uterine anterior lower segment level(U=83.000,P=0.010) showed significant difference among different placenta accreta groups. Placental thickness at the uterine anterior lower segment level showed linear correlation with the blood loss of the following cesarean section(r=0.669,P=0.002). The blood loss of the following cesarean section showed significant difference among different placenta accreta groups(U=118.500,P=0.000). Conclusions The clinical and sonographic manifestations of placenta previa accreta after cesarean section show a spectrum of demographic characteristics. The measurement of thickness of placenta at the anterior lower segment may help the evaluation of the clinical prognosis of this special pathology.

OBJECTIVETo investigate the severity related influencing factor and treatment strategy of severe acute pancreatitis with early organ dysfunction.

METHODSFrom July 2007 to December 2008, 167 patients with severe acute pancreatitis were treated in the Surgical Department of Ruijin Hospital. The relationships between the happening of early organ dysfunction and outcome of the patients were observed, with operative or nonoperative treatment strategy.

RESULTSAmong 167 patients, 68 patients have early organ dysfunction, in which 39 with single organ dysfunction and 29 with multiple organ dysfunction. The early organ dysfunction were involved in 47.1% in cardiovascular system, 35.3% in lung and 29.4% in kidney. Aging (P < 0.05) and higher APACHE II score (P < 0.05) predicted a poor prognosis, which were benefit from early operation.

CONCLUSIONSThe mortality of the patients with SAP is related to age, and the degree of organ dysfunction as well. In the first phase of the disease, the selection of operation depends on the trends and the degree of early organ dysfunction before infected necrosis happens, with the aid of SOFA score as a scale.

Acute Disease ; Aging ; Humans ; Multiple Organ Failure ; Pancreatitis ; diagnosis ; Prognosis

OBJECTIVETo investigate the strategy of controlling fluid resuscitation for severe acute pancreatitis (SAP) in acute phase.

METHODSFrom March 2001 to January 2006, 83 patients meeting for experimental criteria were included in this clinical trial. They were divided into early fluid expansion group (Group I, within 24 h after admission, 21 patients), middle fluid expansion group (Group II, within 25 - 48 h, 35 patients) and late fluid expansion group (Group III, within 49 - 72 h, 27 patients). Parameters of treatment of fluid therapy within 4 d after admission were observed. Serum lactic level was measured on admission and on meeting for criteria of fluid expansion. APACHEII scores, operation rate within 2 weeks, rate of mechanical ventilation, rate of ACS and survival rate were observed.

RESULTSTime interval for meeting fluid expansion criteria in Group I, Group II, Group III was (13 +/- 6) h, (38 +/- 5) h and (61 +/- 8) h, respectively. And there was statistical significance among them (P < 0.05). HCT (%) in Group I (33 +/- 6)% was lower than that of Group II (40 +/- 6)% and Group III (42 +/- 11)% significantly (P < 0.01) at the first day after admission; and there was no statistical significance between Group II and Group III. The amount of crystal and colloid infused in Group I (4014 +/- 2887) ml and (1220 +/- 705) ml at the day of admission was more than those of Group II (2366 +/- 1959) ml and (821 +/- 600) ml and Group III (2615 +/- 1574) ml and (701 +/- 585) ml (P < 0.01); but there was not different between Group II and Group III (P > 0.05). The ratio of colloid and crystal in Group III at the day of admission was lower than those of Group I and Group II (P < 0.05). The total amount of fluid infused was not different among 3 groups for the 4 d (P > 0.05). And infusion rate at the day of admission in Group I was more rapid than those of Group II and Group III (P < 0.05); and there was no difference between Group II and Group III (P > 0.05). The total amount of fluid sequestration in Group II for the 4 d was lower than those of Group I and Group III (P < 0.05); and there was no statistical significance between Group I and Group III (P > 0.05). At the first to the third day after admission APACHEII scores in Group I were higher than those of Group II and Group III (P < 0.05); and at the second and third day, APACHEII scores in Group III were higher than those of Group II (P < 0.05). Rate of mechanical ventilation in Group I (85.7%) was higher than those of Group II (37.1%) and group III (63.0%) (P < 0.05); and rate of ACS was most lowest in Group II (37.1%) (P < 0.05). Survival rate in Group I (38.1%) was lower than those of Group II (85.7%) and Group III (66.7%) (P < 0.05); and Group III was lower than that of Group II (P = 0.075).

CONCLUSIONSWithin 72 h after onset of the disease, survival rate is improved significantly through controlling fluid resuscitation and prevention of body fluid sequestration.

APACHE ; Adult ; Aged ; Female ; Fluid Therapy ; methods ; Humans ; Male ; Middle Aged ; Pancreatitis, Acute Necrotizing ; mortality ; pathology ; therapy ; Resuscitation ; methods ; Severity of Illness Index ; Survival Rate ; Treatment Outcome

OBJECTIVETo investigate the relationship between the clinical character and therapeutic strategy and prognosis in severe acute pancreatitis.

METHODSFrom January 2001 to December 2005, 783 patients with SAP were treated. Therapeutic strategy was selected based on the preliminary scheme for diagnosis and treatment of severe acute pancreatitis by pancreatic surgery society of CMA. All the patients were divided into biliary group and non-biliary group, while 375 patients in biliary group, with 182 patients treated operatively and 193 patients treated nonoperatively; and 408 patients in non-biliary group, with 147 patients treated operatively and 261 patients treated nonoperatively.

RESULTSThere were 698 survivals, the overall survival rate was 89.1%. 357 survivals in the biliary SAP group, the survival rate was 95.0%, in which 171 survivals from operation treated cases, with the survival rate of 94.0%, and 186 survivals from non-operation treated cases, with the survival rate of 96.4%; 341 survivals in the non-biliary SAP group, the survival rate was 84.0%, in which 110 survivals from operation treated cases, with the survival rate of 74.8%, and 231 survivals from non-operation treated cases, with the survival rate of 88.5%. 48.3% patients of the survival group had organ dysfunction, and 18.3% patients had multiple organ dysfunctions, while 100% patients of the death group had organ dysfunction, and 97.6% patients had multiple organ dysfunction. Respiratory dysfunction was found to be the most common cause totally followed by nerve system dysfunction and shock, with the rates of 26.3%, 11.7% and 10.3%, respectively. Respiratory dysfunction, renal dysfunction and cardiac dysfunction are most commonly in death group, with the rate of 94.1%, 60.0% and 60.0%, respectively. The rate of fungi infection in the survival group and death group were 8.9% and 37.6%. The rates of alimentary tract fistula in the survival and death group were 0.9% and 14.1%, respectively.

CONCLUSIONSThe therapy aiming at the cause for biliary SAP and the operation aiming at infected pancreatic necrosis is helpful to improve curative rate; MODS is the main cause of death in severe acute pancreatitis. Respiratory dysfunction, renal dysfunction and cardiac dysfunction are high risk factors.

Female ; Humans ; Male ; Middle Aged ; Pancreatitis, Acute Necrotizing ; diagnosis ; mortality ; therapy ; Prognosis ; Retrospective Studies ; Survival Rate