Hypoglycémie hyperinsulinémique persistante du nouveau-né et du nourrisson

Persistent hyperinsulinemic hypoglycaemia of infancy (PHHI) is the most frequent cause of hypoglycaemia in infancy. Clinical presentation is heterogeneous, with variable onset of hypoglycaemia and response to diazoxide, and presence of sporadic or familial forms. Underlying histopathological lesions can be focal or diffuse. Focal lesions are characterised by focal hyperplasia of pancreatic islet-like cells, whereas diffuse lesions implicate the whole pancreas. The distinction between the two forms is important because surgical treatment and genetic counselling are radically different. Focal lesions correspond to somatic defects which are totally cured by limited pancreatic resection, whereas diffuse lesions require a subtotal pancreatectomy exposing to high risk of diabetes mellitus. Diffuse lesions are due to functional abnormalities involving several genes and different transmission forms. Recessively inherited PHHI have been attributed to homozygote mutations for the beta-cell sulfonylurea receptor (SUR1) or the inward-rectifying potassium-channel (Kir6.2) genes. Dominantly inherited PHHI can implicate the glucokinase gene, particularly when PHHI is associated with diabetes, the glutamate dehydrogenase gene when hyperammonaemia is associated, or another locus.     

Surgery in the nineties

The population of the United States is aging, and by 2020 it is estimated that 16 per cent of U.S. citizens will be over 65 years of age. Little has been published about the results of surgery in nonagenarians but mortality rates of 45 per cent are reported. Given recent improvements in perioperative care we reviewed the experience with major general surgical operative procedures in nonagenarians. We reviewed the charts of patients greater than or equal to 90 years of age who underwent general surgical procedures at UCLA Medical Center since 1986. No patients were excluded. Thirty-two patients were identified. Most (87.5%) patients had significant premorbid conditions. The most common diagnoses were cancer (12), incarcerated hernia (seven), trauma (three), colonic volvulus (two), and cholecystitis (two). Overall perioperative mortality was 9.4 per cent (3 of 32). Twenty-two surgeries (69%) were performed on an emergency basis, and all three deaths were in this group (13.6%). Overall morbidity rate was 57 per cent. Mean intensive care unit stay was 4.8 days. Most patients were discharged home. Our findings support the perioperative safety of elective general surgery in nonagenarians (0% mortality and 20% morbidity). We also found an acceptable risk (13.6% mortality and 68% morbidity) for emergency procedures despite significant comorbid conditions. Most of the patients had acceptable functional outcomes.     

Leiomyoma of the esophagus associated with bronchial obstruction owing to inflammatory pseudotumor in a child

Although relatively common in adults, leiomyoma of the esophagus is a rare disorder in children. A single case report describes the coexistence of both esophageal and bronchial leiomyoma in a child. The authors describe the diagnostic and treatment challenges encountered in a 2-year-old boy with coexisting inflammatory pseudotumor and esophageal leiomyoma presenting as massive atelectasis.     

利尿酸是打开血-迷路屏障的钥匙

目的观察在不同的时机应用利尿酸以暂时破坏耳蜗血管纹上皮是否能够开放血-迷路屏障,从而促使庆大霉素进入耳蜗或者从耳蜗排出.方法听神经动作电位测试技术,全耳蜗毛细胞计数定量观察技术和荧光偏振免疫法测定庆大霉素浓度的技术被用于以下两个不同目的的实验观察.(1)当庆大霉素血中浓度高于内耳液浓度时,应用利尿酸破坏蜗管外壁以促使更多的庆大霉素进入耳蜗以制备不同程度耳蜗损害的动物模型.(2)当庆大霉素内耳浓度高于血中浓度时,应用利尿酸损坏蜗管外壁以促使蓄积在耳蜗内的庆大霉素从内耳排出以达到挽救毛细胞的目的.结果1.当庆大霉素血中浓度高于内耳液浓度时,注射利尿酸可造成更多的毛细胞损害和听功能障碍,外淋巴中药物的峰值浓度和半衰期浓度也均比单独一次注射庆大霉素动物外淋巴中药物浓度增加一倍,说明同时注射利尿酸可促使更多的庆大霉素从血液进入耳蜗并造成更严重的毛细胞损害.2.当血液中庆大霉素排空之后,注射利尿酸可减少毛细胞数量的损失程度,同时发现延迟注射利尿酸组动物的听力损失程度比不经利尿酸处理动物组有所减轻,外淋巴中药物浓度也比不经利尿酸处理动物组降低一半,说明当GM在耳蜗内蓄积但血清中已经排空时注射EA有助于降低药物在耳蜗内的蓄积并挽救尚未被破坏的毛细胞.结论利尿酸可以做为打开血-迷路屏障的钥匙,但是应用利尿酸开放血-迷路屏障可以产生双向结果,其关键在于注射利尿酸的时机.     

Interpreting the improved outcome of patients with central nervous system metastases managed in clinical trials compared with standard hospital practice*

The aims were to determine the median survival and prognostic factors of patients with central nervous system (CNS) metastases managed with whole‐brain radiation therapy (WBRT), and to explore selection criteria in recently published clinical trials using aggressive interventions in CNS metastases. A retrospective audit was performed on patients managed with WBRT for CNS metastases. Potential prognostic factors were recorded and analysed for their association with survival duration. The proportion of patients with these factors was also compared with those of patients managed under three recently reported studies investigating aggressive interventions, such as radiosurgery and chemotherapy for CNS metastases. Seventy‐three patients were treated with WBRT for cerebral metastases over a 12‐month period. The median survival of the population was 3.4 months (95% confidence interval: 2.7–4.1), with 6‐ and 12‐month survival rates of 30 and 18%, respectively. Significant prognostic factors for prolonged median survival were Eastern Cooperative Oncology Group status 0–2 (P = 0.015), Medical Research Council neurological functional status 0–1 (P = 0.006), and Recursive Partitioning Analysis Class 2 versus Class 3 (P = 0.020). On multivariate analysis, younger patient age (P = 0.02) and better performance status (P < 0.01) were associated with improved outcome. When comparing these characteristics with selected published studies, our study cohort demonstrated a higher proportion of patients with poor performance status, a greater number of metastases per patient and a higher incidence of extracranial disease. This reflects the selected nature of patients in these published studies. Central nervous system metastases confer a poor prognosis and, for the majority of patients, aggressive interventions are unlikely to improve survival. The use of potentially toxic and expensive treatments should be reserved for those few in whom these studies have shown a potential benefit.     

Clinical Outcomes and Resource Use for Infants With Hypoplastic Left Heart Syndrome During Bidirectional Glenn: Summary From the Joint Council for Congenital Heart Disease National Pediatric Cardiology Quality Improvement Collaborative Registry

The National Pediatric Cardiology Quality Improvement Collaborative (NPC-QIC) registry captures information on interstage management of infants with hypoplastic left heart syndrome (HLHS). The purpose of this study was to identify interstage risk factors for increased resource use and adverse outcomes during bidirectional Glenn (BDG) hospitalization. All infants in the NPC-QIC registry (31 United States hospitals) undergoing BDG surgery were included (December 2009 to August 2010). Patient demographics, interstage variables, operative procedures, and complications were recorded. Days of hospitalization, ventilation, inotrope use, and complications were surrogates of resource use. Logistic regression analysis determined the associations between predictor variables and resource use. Of 162 infants, 105 (65 %) were males. At BDG, the median age was 155 days (range 78–128), mean weight-for-age z-score was ?1.6 ± 1.1, mean length-for-age z-score was ?1.5 ± 1.7, and mean preoperative oxygen saturation was 78 % ± 7 %. Caloric recommendations were met in 60 % of patients, and 85 % of patients participated in a home-surveillance program. Median days of intubation, inotrope use, and hospitalization were 1, 2, and 7, respectively. There were 4 post-BDG deaths and 55 complications. In multivariate analysis, lower weight-for-age z-score, female sex, and aortic atresia with mitral stenosis were associated with a higher risk of BDG complications. Meeting caloric recommendations before BDG was associated with fewer hospitalization days. Lower weight-for-age z-score was an independent and potentially modifiable risk factor for BDG complications. HLHS infants who met caloric recommendations before BDG had a lower duration of hospitalization at BDG. These data justify targeting nutrition in interstage strategies to improve outcomes and decrease costs for patients with HLHS.     

异一枝蒿酮酸的结构

从新疆一枝蒿(Artemisia rupestris L.)中分得一个新成分,命名为异一枝蒿酮酸(isorupestonic acid),根据光谱(IR,UV,MS.NMR),X-ray晶体衍射及CD谱分析,确定其结构及绝对构型。并经X-ray晶体衍射及CD谱分析修正了一枝蒿酮酸的绝对构型。     

Extending the two‐process model of burnout in child protection workers: The role of resilience in mediating burnout via organizational factors of control,values, fairness,reward, workload,and community relationships

Burnout has been disproportionally reported in child protection social work. This paper presents data from 162 child protection staff in Northern Ireland, assessed for burnout using the Maslach Burnout Inventory. Path models were estimated, based on an extension of the two‐process demands and values model (Leiter, 2008 ) to include additional measures of resilience using the Resilience Scale‐14, as well as perceived rewards and sense of community from the Areas of Work Life Scale (Leiter, 2008 ). Optimal model fit was achieved by modelling resilience as a mediator of the relationship between organizational factors of control and value congruence and burnout. Resilience also directly predicted emotional exhaustion (β = ?.23, p < .005) and personal accomplishment (β = .46, p < .001). Workload was the strongest direct predictor of emotional exhaustion (β = ?.54, p < .001). Adding perceived rewards to extend the two‐process model resulted in moderate associations with control (β = .44, p < .001), workload (β = .26, p < .005), fairness (β = .40, p < .001), and values (β = .32, p < .001). In the final model, resilience is modelled as both an outcome of some organizational factors whilst also making a unique direct contribution to explaining burnout alongside other organizational factors. Other pathways and mediating relationships are reported and further research directions discussed.     

Severe methemoglobinemia due to topical dapsone misuse in a teenage girl

Dapsone gel is a topical treatment for facial acne in adolescents and adults, and while systemic dapsone therapy is known to be associated with methemoglobinemia, once-daily topical dapsone has been well tolerated with few side effects in large randomized controlled trials. We describe the first reported case of severe methemoglobinemia in a healthy adolescent using daily topical dapsone. Although the medication was prescribed for facial use only, the patient reported topical use over her back and chest as well. This case illustrates the potential for significant systemic dapsone absorption even with daily topical dosing and demonstrates the need for clear anticipatory guidance to prevent the potential morbidity and mortality associated with methemoglobinemia from improper topical dapsone use.