Clinical characteristics of 86 children with Beh?et′s disease / 中华风湿病学杂志

Objective:To analyze the clinical characteristics of pediatric patients with Beh?et′s disease.Methods:The clinical characteristics of 86 newly diagnosed children with Beh?et′s disease admitted to the rheumatology department of Beijing Children′s Hospital from July 2015 to December 2020 were analyzed retrospectively. Statistical product and service solutions (SPSS) 26 was used for statistical analysis. The normal distribution of measurement data is expressed in Mean± SD, and the non normaldistribution of measurement data was expressed in median(minimum, maximum). The counting data was expressed in frequency (cases) and percentage. Results:There was no gender difference in the incidence of Beh?et′s disease in 86 children.The age of onset was 0.1~15.9 years, with an average of (7±4) years, and the age of diagnosis was 1.3~16.6 years, with an average of (10±4) years.The course of disease from onset to diagnosis was 0.5~168 months, with a median course of 21 months. Among 86 cases, 52 cases (60.5%) showed the most common oral ulcer at the onset, followed by 19 cases (22.1%) with fever. In terms of clinical manifestations: the most common clinical manifestation was oral ulcer in 82 cases (95.3%), followed by fever in 58 cases (67.4%), and gastrointestinal symptoms in 44 cases (51.2%). The common manifestation of digestive system involvement was abdominal pain and diarrhea. Ten cases (11.6%) had ocular symptoms, 13 cases (15.3%) had vascular involvement, and 3 cases (3.5%) had pulmonary involvement. Fourteen cases (16.2%) had family history. Fourty seven patients (54.7%) had elevated leukocyte, 65 patients (75.6%) had elevated CRP and 72 patients (83.7%) had elevated ESR.Conclusion:Beh?et′s disease in children is usually insidious in onset and infants may suffer from this disease. Oral ulcer is the most common clinical manifestation, followed by fever. For patients with fever of unknown cause, Beh?et′s disease should be noted. In terms of involvement of important organs, digestive tract involvement is more common in childhood, followed by large blood vessels and eyes.

Gene mutation analysis in a Chinese pedigree with autosomal dominant Waardenburg syndrome / 中华皮肤科杂志

Objective:To report a Chinese pedigree with autosomal dominant Waardenburg syndrome, and to identify causative gene mutations.Methods:Clinical data and peripheral blood samples were collected from the proband and her parents. Genomic DNA was extracted, gene mutations were detected through a next-generation skin-targeted sequencing panel, and Sanger sequencing was performed to verify causative mutations.Results:The proband clinically presented with irregular white patches on the abdomen and lower limbs, moderate to severe sensorineural deafness in the right ear, and iris heterochromia in both eyes. The proband′s mother presented with iris heterochromia in both eyes, epicanthus, early canities and thick eyebrows. In the family, both the proband and her mother were diagnosed with Waardenburg syndrome. A causative frameshift mutation c.976-977delinsT (p.Thr327Profs*54) was identified in both the proband and her mother, which caused the AG to TT base substitution at positions 976 - 977 in the coding region of exon 7 of the PAX3 gene, resulted in a frameshift from the amino acid position 327 to 54 in the PAX3 protein (threonine was substituted by proline at amino acid position 327). The proband′s father showed a normal phenotype, and his genetic test results were negative.Conclusion:The novel frameshift mutation c.976-977delinsT (p.Thr327Profs*54) in the PAX3 gene may contribute to the clinical phenotype of the patients with Waardenburg syndrome in the family.

A case of brain-lung-thyroid syndrome / 中南大学学报(医学版)

Brain-lung-thyroid syndrome is a rare autosomal dominant disorder. More than 100 cases have been reported worldwide, but few cases have been reported in China. In December 2018, a boy with brain-lung-thyroid syndrome, aged 3 years and 10 months, was admitted to Xiangya Hospital of Central South University due to repeated cough for more than 3 years. In infancy of the boy, psychomotor retardation, repeated cough, and hypothyroidism were found. Gene detection showed that there was c.927delc heterozygous variation in NKX2-1 gene (NM-001079668: exon3: c.927delC). The variation of this gene locus has not been reported in relevant literature so far, which indicates a new mutation. According to the above clinical manifestations and examination results, the boy was diagnosed as brain-lung-thyroid syndrome, which mainly characterized by nervous system disorders, accompanied by respiratory manifestations and hypothyroidism. The boy was treated with oral dopasehydrazine to relieve tremor and levothyroxine sodium tablets to relieve hypothyroidism. Anti-infection, atomization, rehabilitation training and other symptomatic supporting treatment were also administered. The boy's language and movement have improved, the thyroid hormone level is normal, and there are still repeated respiratory tract infections.

Effect of peer intervention by popular opinion leaders among young students who have sex with men / 中国学校卫生

Objective@#The proposal of this study is to determine the feasibility of using online social software to guide popular opinion leaders(POLs) among young students MSM (men who have sex with men, MSM) carrying out comprehensive AIDS prevention in young students MSM.@*Methods@#From November to December 2019 in Tianjin, 22 POLs who had previously experienced AIDS prevention education and training foucus on life skills were recruited. Though snowball sampling method, 100 POLs partners (friends, sexual partners, classmates, etc) were invited into the study. From January to March 2020, regular posters will be sent through the WeChat platform to guide POL to intervene their partners/sexual partners. The questionnaire surveys were conducted before and after the intervention.@*Results@#After the intervention, both the proportion of young students MSM had sex and with multiple sex partners decreased(χ2=12.12, 5.94, P<0.05). The comprehensive ability of AIDS prevention has improved significantly, the "National Eight Items" correct rate has risen from 90.0% to 99.0%(χ2=5.82, 7.56, P<0.05), and the "Youth Eight Items" correct rate has increased from 86.0% to 98.0%(P<0.01). Knowledge score increased from(11.08±1.89) to (11.84±1.27); Attitude score increased from (54.29±4.01) to (56.06±3.75), the behavior score increased from (52.12±4.78) to (55.08±4.25), skill score increased from (51.96±4.75) to (54.67±5.04), The comprehensive prevention ability score (percent) increased from (87.30±6.95) to (91.86±5.42)(t=-4.21, -3.51, -5.32, -5.16, -6.45, P<0.01).@*Conclusion@#Using Wechat platform to guide POL carrying out comprehensive AIDS prevention is effective for improving the comprehensive AIDS prevention ability of young students MSM.

Zollinger-ellison syndrome. case report

ABSTRACT Introduction: The Zollinger-Ellison syndrome (ZES) is a pathology caused by a neuroendocrine tumor, usually located in the pancreas or the duodenum, which is characterized by elevated levels of gastrin, resulting in an excessive production of gastric acid. Case presentation: A 42-year-old female patient with a history of longstanding peptic ulcer disease, who consulted due to persistent epigastric pain, melena and signs of peritoneal irritation. Perforated peptic ulcer was suspected, requiring emergency surgical intervention. Subsequently, a tumor lesion in the head of the pancreas was documented and managed with Whipple procedure. The pathology results reported a tumor suggestive of neuroendocrine neoplasm. Discussion: The Zollinger-Ellison syndrome occurs in 0.1 to 3 people per 1 000 000 inhabitants worldwide and is predominant in women between 20 and 50 years of age. It usually appears as a refractory acid-peptic disease or as a complication of gastric acid hypersecretion. Medical therapy is the standard management, being proton pump inhibitors (PPI) the most effective option. Surgery is recommended for sporadic ZES. Conclusions: ZES has a low incidence rate. It is rarely considered in the differential diagnosis of chronic epigastric pain and high clinical suspicion is required to achieve adequate management. This article is highly relevant as it presents a confirmed clinical case of ZES in Colombia, highlighting the importance of producing local scientific literature to improve the diagnosis and treatment of this pathology.

RESUMEN Introducción. El síndrome de Zollinger-Ellison (SZE) es una patología producida por un tumor neuroendocrino habitualmente localizado a nivel duodenal o pancreático, el cual produce niveles elevados de gastrina, derivando en hipersecreción de ácido gástrico. Presentación del caso. Paciente femenino de 42 años con antecedente de enfermedad ulceropéptica de larga data, quién consulta por epigastralgia persistente y deposiciones melénicas y presenta signos de irritación peritoneal. Se sospecha una ulcera péptica perforada, requiriendo intervención quirúrgica de urgencia. Posteriormente se documenta una lesión tumoral en la cabeza del páncreas, manejada con cirugía de Whipple; en el reporte de patología se detecta un tumor sugestivo de neoplasia neuroendocrina. Discusión. El SZE se presenta en 0.1-3 personas por cada 1 000 000 de habitantes a nivel mundial, predominantemente en mujeres entre 20 y 50 años de edad. Suele debutar como enfermedad ácido-péptica refractaria o por complicaciones de la hipersecreción gástrica. La terapia médica es el manejo estándar, siendo la más efectiva la que involucra inhibidores de la bomba de protones. En SZE esporádico está recomendada la cirugía. Conclusiones. El SZE tiene una incidencia baja, raramente se considera en el diagnóstico diferencial de epigastralgia crónica y se requiere alta sospecha clínica para lograr un manejo adecuado. Este artículo es valioso al presentar un caso clínico confirmado de SZE en Colombia, destacando la importancia de producir bibliografía científica local para mejorar el diagnóstico y tratamiento de esta patología.

Analysis of risk factors of nosocomial infection in hospitalized patients with myelodysplastic syndrome / 天津医药

@#Objective To investigate the characteristics and risk factors of nosocomial infection in patients with myelodysplastic syndrome (MDS), and to provide a theoretical basis for the prevention and treatment of nosocomial infection in such patients. Methods A total of 226 patients with MDS hospitalized in the Department of Hematology in our hospital from January 2016 to December 2017 were selected as subjects in this study. The retrospective investigation was used to analyze the characteristics and related risk factors in hospitalized patients with hospital infection. Results A total of 52 patients were found nosocomial infection during hospitalization, with an infection rate of 23.01%, 3 cases with double infections, and 2 cases with multiple infections. Among them, 28 cases were found pathogenic bacteria and 15 cases of Gram-negative bacteria (53.6%), 4 cases of blue positive bacteria (14.3%) and 9 cases of fungi (32.1%). Univariate analysis showed that MDS revised international prognostic score (IPSS-R) risk grouping, length of hospital stay, neutrophil count, duration of neutropenia, invasive procedure, albumin content, use of hormones and use of decitabine were closely related to nosocomial infection in hospitalized patients with MDS (P＜0.05). Multivariate Logistic analysis showed that patients with lower prognostic scores for disease IPSS-R, longer hospital stay, low albumin levels, longer duration of granulocytosis, and use of decitabine during hospitalization were the independent risk factors for nosocomial infection (P＜0.05). Conclusion Targeted measures are taken to address the risk of nosocomial infections in hospitalized patients with MDS, which shorten the hospital stay, and can reduce the incidence of nosocomial infection.

Wu Liangcun’s Analysis about Clinical Experience of Ovarian Cancer’s Treatment / 浙江中医药大学学报

Objective] To summarize national famous doctor of traditional Chinese medicine-Wu Liangcun ’s experience about the clinical treatment of ovarian cancer. [Method] By studying and following Professor Wu Liangcun to diagnose, this paper discusses Professor Wu ’s academic views and clinical experience of diagnosis and treatment of ovarian cancer respectively from the aspects such as etiology and pathogenesis, and treatment based on syndrome differentiation. It expounds the characteristics of his prescription and also gives proven cases. [Result]Professor Wu Liangcun proposes that the pathogenesis of ovarian cancer is mainly congenital deficiency and physical weakness resulted from cold coagulation, qi stagnation, blood stasis and phlegmatic hygrosis. All these remain a standstill, gather together and become a disease. He uses the syndrome differentiation method to divide the common ovarian cancer into four types:blood stasis and vital energy retardation, phlegm dampness condensation, deficiency of liver-yin and kidney-yin, asthenia of qi and blood. In the treatment, he organically combines the dialectics and the differentiation of disease; In the selection of drugs, he streamlines the prescription and the drug, which maximizes the efforts of the drugs and has good efforts in clinic.[Conclusion] Professor Wu Liangcun is good at making a diagnosis and giving treatment of ovarian cancer in combination with disease and syndrome, and he uses medicine flexibly. It has a very good effect and practical value for guiding clinic, and it should be promoted.

Study on micromegakaryocytes of patients with myelodysplastic syndrome by flow cytometry / 重庆医学

Objective To setup a measurement of human bone marrow micromegakaryocyte which based on CD41a and PI double‐labeled flow cytometric analysis ,and study the significance in the diagnosis of MDS .Methods In 42 cases of MDS patients , their bone marrow megakaryocytes were obtained by Percoll density gradient separation medium .The megakaryocyte glycoproteinⅡb/Ⅲa(CD41a)were marked with fluorescein isothiocyanate through its corresponding monoclonal antibody ,and their DNA were marked with PI .Then the megakaryocyte ploidy was analyzed by flow cytometry(FCM ) .Results The method for micromegakaryo‐cyte identification and analysis was established .In 42 patients with MDS ,the detection rate of micromegakaryocyte was 90 .5 per‐cent by FCM analysis ,but only 54 .8 percent by Wright‐Giemsa staining test and 64 .3 percent by immunohistochemistry ,the differ‐ence among them was statistically significant(χ2 = 13 .640 ,P= 0 .001) .The 42 patients with MDS were divided into two groups (low‐risk group and high‐risk group) .The detection rates of micromegakaryocyte were 81 .8 percent in low‐risk group and 100 per‐cent in high‐risk group separately by FCM analysis ,the difference was statistically significant(χ2 =4 .019 ,P=0 .045) .Conclusion The detection rate of micromegakaryocyte by FCM with CD41a and PI double marker is higher than that by cytochemical staining . The detection rate of micromegakaryocyte in the high‐risk group is higher than that of the low‐risk group ,which shows that the de‐tection of micromegakaryocyte is of great significance for MDS prognosis assessment .

Genetic and Clinical Aspects of Brugada Syndrome.

Death is always dreadful and the diseases those causes sudden death are universal threats in health concern. Brugada syndrom is a recently identified entity of arrhythmia and sudden cardiac death. This genetic and male dominant disorder is prevalent in Southeast Asian region. At least seven genes have been identified to associate with its occurrence though the detail pathophysiological mechanism is till to be resolved. The correlation of ion channel genes to Brugada syndrom is still dubious as the same genes also related to other cardiac diseases. Here we review the genetic aspects of Brugada syndrom with a breif overview of epidemiology, diagnosis and management system.

Contrast-Enhanced Ultrasound Defines Vascularization Pattern of Hamartomatous Colonic Polyps in Peutz-Jeghers Syndrome

The hamartomatous polyps in Peutz-Jeghers syndrome may have malignant potential. To differentiate between hamartomatous and adenomas polyps, vascular characterization can be assessed using noninvasive procedures, such as contrast-enhanced ultrasound (CEUS). The neo-angiogenic characteristics of colorectal adenomas and carcinomas are expressed as an anarchic vascular pattern observed on CEUS. Using CEUS in a patient with Peutz-Jeghers syndrome, we describe for the first time the vascularization of a hamartomatous colonic polyp that exhibits a hierarchy branching pattern.

Toxoplasmose gástrica em paciente infectado pelo vírus da imunodeficiência humana. Relato de caso / Gastric toxoplasmosis in patient infected with human immunodeficiency virus. Case report

A toxoplasmose é uma zoonose altamente disseminada. A maio­ria das infecções em imunocompetentes é assintomática. Porém, em pacientes imunodeprimidos, a infecção adquire um curso variável. Em pacientes com contagem de CD4 abaixo de 100 e que foram previamente expostos ao Toxoplasma gondii, pode haver reativação da doença em diversos tecidos. Envolvimento do trato gastrintestinal por Toxoplasma gondii é raramente relatado. Embora os sintomas gastrintestinais sejam comuns entre os pacientes com síndrome da imunodeficiência adquirida, a maioria é causada por infecções entéricas que não o Toxoplasma gondii. O objetivo deste estudo foi relatar um caso raro de toxoplasmose gástrica. Paciente do gênero feminino, 38 anos, com diagnóstico recente de vírus da imunodeficiência humana, iniciou sintomas gástricos inespecíficos como: epigastralgia, náuseas, vômitos e perda ponderal. O diagnóstico definitivo foi fechado com o estudo anatomopatológico da lesão na mucosa gástrica. Foi instituído tratamento para a toxoplasmose com clindamicina, pirimetamina e ácido folínico (devido à mielotoxicidade), com melhora parcial dos sintomas. Embora raro, a toxoplasmose gástrica deve entrar no diagnóstico diferencial de dor epigástrica em pacientes portadores da síndrome da imunodeficiência adquirida com contagem de CD4 baixa. Seu diagnóstico pre­suntivo pode ser dado pelo quadro clínico, mas o diagnóstico definitivo é obtido pela biópsia da lesão...

Toxoplasmosis is a highly disseminated zoonosis. Most infections are asymptomatic in immunocompetent patients. However, in immunocompromised patients, infection acquires a variable course. In patients with CD4 counts lower than 100 and who have been previously exposed to Toxoplasma gondii, there may be reactivation of the disease in various tissues. Involvement of the gastrointestinal tract by Toxoplasma gondii is rarely reported. Although gastrointestinal symptoms are common among patients with acquired immunodeficiency syndrome, most are caused by enteric infections other than Toxoplasma gondii. The aim of this study was to report a rare case of gastric toxoplasmosis. A 38-year-­old female patient, recently diagnosed with immunodeficiency human virus, presented with nonspecific gastric symptoms such as epigastric pain, nausea, vomiting and weight loss. The definitive diagnosis was reached with anatomopathological examination of gastric mucosa damage. She was treated for toxoplasmosis with clindamycin, pyrimethamine and folinic acid (due to myelotoxicity), with partial improvement of symptoms. Although rare, gastric toxoplasmosis should enter the differential diagnosis of epigastric pain in patients with acquired immunodeficiency syndrome with low CD4 count. Its presumptive diagnosis can be made on a clinical basis, but the definitive diagnosis is reached with biopsy...

Influence of glutamine on the outcome of acute lung injury / 中国基层医药

Objective To explore the influence of glutamine on the outcome of acute lung injury.Methods According to the digital table,70 patients with acute lung injury were randomly divided into the control group and glutamine treatment group(Gln group),35 cases in each group.After 7 days treatment,APACHE Ⅱ score Murray lung injury score,duration of mechanical ventilation and ICU stay time of the two groups were compared.Results After treatment,the APACHE Ⅱ score and the Murray lung injury score were also improved(all P ＜ 0.05).The APACHE Ⅱ score and the Murray lung injury score of the Gln group were (6.54 ± 2.31) points and (0.53 ±0.42) points,respectively,which were significantly lower than those of the control group [(10.43 ± 2.15) points and (1.53 ± 0.62)points] (t =3.15,2.17,all P ＜0.05).The duration of mechanical ventilation and ICU stay time of the Gln group were (6.17 ±4.53)d,(13.41 ± 8.74)d,respectively,which were significantly less than those of the control group [(11.57 ±5.25)d,(21.34 ±10.27)d] (t =2.78,4.62,all P＜0.05).Conclusion Glutamine treatment can improve the prognosis of patients with acute lung injury,and it is a parenteral nutrition drug for critically ill patients.

The multiple factors in ACI patients with different MODS scores / 中国综合临床

Objective To investigate the differences of multiple factors in acute cerebral infarction (ACI) patients with and without multiple organ dysfunction syndrome (MODS),as well as in ACI patients in different MODS score groups.Methods One hundred and fifty-seven ACI patients were divided into non-MODS group( without concurrent MODS group)and MODS group.The MODS group patients were further divided into four subgroups according to the scores,including 1 -6points,7 - 12 points,13 - 18 points and points over 19.All patients were measured for procalcitonin(PCT) and C-reactive protein(CRP).The National Institutes of Health stroke score( NHISS score),acute physiology and chronic health evaluation( APACHE Ⅱ score)and Watian water test score were calculated.The differences in age,gender,PCT,CRP,NHISS score,APACHE Ⅱ score,Watian water score,breathing support rate,eating rate and mortality rate between the two groups were compared.Results Non-MODS group,compared with the MODS group,was significantly younger( [72.11 ± 16.41 ] years vs.[ 77.88 ±17.67 ] years,t=2.451,P ＜ 0.05 ),and the difference in the ratio of male to female between groups was not significant (57/38 vs.34/28,x2 =0.414,P ＞ 0.05 ).Differed from MODS group,non-MODS group had significant lower PCT value ( 1.83 ± 0.51 vs.2.98 ± 0.71,P ＜ 0.01 ),CRP value ( [ 12.53 ± 7.12] mg/L vs.[69.89 ±43.83 ] mg/L,P ＜0.01 ),NHISS score(9.38 ±5.24 vs.21.35 ±7.47,P ＜0.01 ),APACHE Ⅱ score ( 11.63 ± 4.22 vs.30.92 ± 7.80,P ＜ 0.01 ),Watian water score ( 2.36 ± 0.98 vs.3.88 ± 1.09,P ＜ 0.01 ),breathing support rate ( 2.1％ vs.43.5％,P ＜ 0.01 ) and mortality rate ( 4.2％ vs.43.5％,P ＜ 0.01 ),but had remarkable higher eating rate(95.8％ vs.66.1％,P ＜0.01 ).Pairwised comparison among the four MODS score groups,the PCT,CRP,NHISS score,APACHE score,Watian water test,breathing support rate and mortality rate were significantly different(P ＜ 0.05) ;The differences in age between the 1 -6 points group and the other three groups was significant ( P ＜ 0.05 ).Conclusion Age,PCT,CRP,NHISS score,APACHE score,Watian water test score,breathing support rate,mortality rate of the high-score MODS groups were higher than those of MODS groups with low-score in ACI patients,while eating rate was lower than that of the low-score groups.

Status and demand on HIV/AIDS control among men who have sex with men in main districts of Chongqing / 第二军医大学学报

Objective: To survey the knowledge, attitude and behavior about HIV/AIDS among men who have sex with men (MSM) in the main districts of Chongqing and to analyze the current status and demand on HIV/AIDS control in the districts. Methods: Questionnaire survey was conducted among MSM, and qualitative interviews of MSM and some key informants, including the centers for disease control and prevention (CDC) staff, volunteers and the owners of bars or public bathrooms were done. Results: About 80% of MSM were younger than 30 years old and were unmarried. More than half of MSM had college degree or above. Most of them were students, office staff and entertainment staff, and about 25% of them were bisexual. Television and newspapers were the main pathways to obtain knowledge on HIV/AIDS; friends, free publicity materials, internet and consulting services were also ways to obtain related knowledge. It was found that in the recent six months, 6.5%(50/772) of MSM sold and 3.9%(30/772) bought sexual services, and about 17.1% (132/772) MSM had sex with women in the recent six months. The rates of condom use among MSM when having sex with men and women at every intercourse were 41.9%(271/647) and 29.5%(39/132), respectively. And 82.0%(633/772) of them had intervention service. MSM suggested that their privacy should be fully protected during the intervention, more attractive publicity materials should be provided, and more knowledge was needed on other sex transmitted diseases; the intervention ways should be diversified; and they needed the consultation of professional. The volunteers required more training and independent offices. The owners of entertainment argued that more efforts should be made to encourage the entertainment workers to promote intervention. Conclusion: MSM in the main district of Chongqing have many high risk factors of HIV/AIDS. Though various interventions have been given and some achievement has been made, more efforts are needed to protect the privacy of MSM population and to improve the intervention. And efforts should also be made to promote the initiation of the volunteers, entertainment owners and staff.

99Tcm-MDP whole body bone imaging in 25 patients with SAPHO syndrome / 中华核医学杂志

Objective To investigate the usefulness of 99Tcm-MDP whole body bone scintigraphy (WBBS) in patients with synovitis,acne,pustulosis,hyperostosis,osteitis (SAPHO) syndrome.Methods 99Tcm- MDP WBBS was performed in 25 patients (6 males,19 females,mean age =(55.1 ±9.8) years)with SAPHO syndrome.Bone lesions were classified into five categories:anterior chest wall,spine,mandible,sacroiliac joint,and limbs.The typical scintigraphic manifestations of SAPHO syndrome were summarized and compared to other radiological imaging data.Results Among 25 patients,32％ of cases (8/25)were associated with skin lesion; 48％ ( 12/25 ) were pathologically diagnosed with chronic nonspecific bone inflammation by bone biopsy.On 99Tcm-MDP WBBS,abnormal metabolic foci at anterior chest wall were found in all cases,most of which located in the sternocostoclavicular region (96％,24/25 ),including sternoclavicular joints (60％,15/25),first costosternal junctions (48％,12/25),and manubriosternal junctions (44％,11/25 ).Only 20％ of the patients (5/25) demonstrated the typical scintigraphic characteristic:“bull's head” sign.The second most frequent part was spine (44％,11/25).Appendicular skeleton was affected in 16％ (4/25) patients.WBBS also demonstrated additional skeletal lesions in 68％ (17/25 ) of the patients,mainly in first costosternal junctions (7 patients),sternoclavicular joints (6 patients),manubriosternal junctions (5 patients) and spine (4 patients).Conclusions Abnormal metabolic foci in sternocostoclavicular region and other imaging manifestations on 99Tcm- MDP WBBS can be used to diagnose,differentiate,and localize the insidious lesion and evaluate the lesion activity in patients with SAPHO syndrome.

99Tcm-MDP bone scan for the diagnosis of SAPHO syndrome / 中华核医学杂志

Objective To investigate the clinical value of bone scintigraphy for the diagnosis of synovitis, acne, pustulosis, hyperostosis and osteitis (SAPHO) syndrome. Methods The characteristics of SAPHO syndrome on 99Tcm-MDP imaging of 8 cases were retrospectively analyzed. Results The 99TcmMDP bone scan was positive for all patients. Seven cases were found lesions in the costoclavicular region, 3cases involved with unilateral sacroiliac joint, 2 cases involved with spine and 1 patient with extremity. Four lesions were found without relevant clinical symptoms. Conclusion Bone scintigraphy is useful for the diagnosis of SAPHO syndrome, especially for those lesions with no clinical symptoms.

Coronary artery bypass grafting for coronary disease due to Kawasaki disease / 中华胸心血管外科杂志

Objective To determine the efficacy of coronary artery bypass grafting (CABG) in children with coronary obstructive disease subsequent to Kawasaki disease. Methods Between Feb 2005 and Sep 2009, 6 children with ischemic heart disease due to Kawasaki disease comprised the study group. The age of patients at operation was ranged from 6 to 12 years with a mean of ( 8.0 ± 2.3 ) years. The preoperative EF was ranged from 0.33 to 0.71 with a mean of 0.57 ± 0.15. There was one case with moderate mitral valve regurgitation. Results All patients survived the procedures. The mean number of bypass graft was 2.0 ±0.6,with 4 internal thoracic arteries and 7 radial arteries. Mitral valve repair was employed in 1 case. The cardisc function ( NYHA and EF) was significantly improved postoperatively. Postoperative CTA showed no graft disease in all cases. Conclusion CABG using the arterial grafts can provide attractive mid-term results in patients with obstructive coronary arteries associated with Kawasaki disease.

The clinical indications for Extrocopereal membrane oxygenation in acute and sever cases / 中国综合临床

Objective To summarize the method and effects of extracorporeal membrane oxygenation (EC-MO) for eight patients with sever heart or(and) lung diseases,and to get some reliable advises by analysis of retro-spoctive indications,timing and result in ECMO therapy. Methods Eight patients,aging from 26 to 82 and weighted from 57-87 kg were observed. Vein-artery ECMO was used in all patients,with the flow in 40-70 ml/(kg·min) and the activated clotting time(ACT) kept at 160-200 s. Results The time of ECMO support varied from 9.5 h to 84.1 h;Among the eight patients,one didn't weaned from ECMO and died,two who were weaned died 38 h ,6 h af-ter ECMO;five patients weanned succsesefully from ECMO and discharged. Conclusions ECMO do well in treat-ment of severe cardiopulmonary failure, resuscitation and support post cardiosurgery,and the indication and timing performance are important to the final result.

Síndrome de Werdnig-Hoffman (atrofia muscular espinal de la infancia). Presentación de un caso y revisión de literatura / Werdnig-Hoffmann syndrome (spinal muscular atrophy of childhood). Case report and literature review

El síndrome de Werdnig-Hoffmann o atrofia muscular espinal (AME) de la infancia es una enfermedad de patrón autosómico recesivo de origen neuromuscular y degenerativo, poco prevalente en la población general, y que se caracteriza por la destrucción de las neuronas motoras del asta anterior de la médula espinal debido a alteraciones cromosómicas. La enfermedad no tiene tratamiento, es de mal pronóstico y, por lo general, culmina con la muerte del menor en los primeros años de vida por dificultad respiratoria, infecciones respiratorias o ambas. En el presente artículo se describe un caso de la enfermedad que se pone en evidencia con el árbol genealógico en el que se encuentra el patrón de herencia de la enfermedad, además de los síntomas y signos que la caracterizan. [Martínez SC, Lancheros EA, Rodríguez JC. Síndrome de Werdnig-Hoffmann (atrofia muscular espinal de la infancia). Presentación de un caso y revisión en la literatura. MedUNAB 2010; 13:116-122].

Werdnig-Hoffmann Syndrome or Spinal Muscular Atrophy (SMA) of childhood is a disease of autosomal recessive neuromuscular and degenerative origin, some prevalent in the general population, characterized by motor neurons destruction in anterior spinal cord due to chromosomal abnormalities. The disease has no treatment, had poor prognosis, and usually ends in child death in her/his first years of life caused by respiratory distress, respiratory infection, or both. This paper describes a case of SMA with and evident pedigree with a tipical inheritance pattern, and symptoms and signs that characterize this entity. [Martínez SC, Lancheros EA, Rodríguez JC. Werdnig-Hoffmann Syndrome (Spinal Muscular Atrophy). Case report and review. MedUNAB 2010; 13:116-122].