Learning lessons in emergency management: the 4th International Conference on Healthcare System Preparedness and Response to Emergencies and Disasters.

BACKGROUND: The International Preparedness & Response to Emergencies & Disasters (IPRED) conferences are conducted bi-annually in order to share insights and lessons learned from diverse crises. The aim of the article is to bring the IPRED conferences into better professional attention and to share the main insights that were presented in IPRED IV, which was held in January 2016. MAIN BODY: The major lessons learned included: Planning, regional/global collaboration and public-private cooperation should be implemented in developing novel technologies. International humanitarian action necessitates coordination between diverse actors concerning all potential threats. Leadership/coordination and decision-making capacities of emergency response leaders should be enhanced to ensure quality of care. Ethics in disaster management: Triage decisions must not discriminate against terrorists, even when attackers and victims are treated simultaneously. Resilience management: Establishing family and community networks increases resilience of individuals and society. Training programs & exercises must be evaluated considering cost-benefits. Human resources: Teams of experts should be transformed into expert teams. Communication: A common disaster-management language needs to be established. Social media is useful due to bi-directional communication. Civil-military cooperation should be established to facilitate a coordinated response including common terminologies and exercises. Animal sheltering: First responders and pet owners are jeopardized if animals are not included in emergency planning. Re-unification of animals with their owners should be included in response models. CONCLUSIONS: IPRED conferences provide a platform for sharing insights and lessons learned from diverse emergencies and disasters. The conferences offer a unique opportunity to share knowledge aimed at improving emergency preparedness, networking between various parties, and substantiates the knowledge and experience of all professionals who take part in the proceedings.

Impact of trastuzumab treatment beyond disease progression for advanced/metastatic breast cancer on survival - results from a prospective, observational study in Germany.

OBJECTIVES: Evidence suggests that continued trastuzumab therapy beyond progression (TBP) may provide additional survival benefit. Within the framework of an observational prospective study of patients with advanced/metastatic breast cancer receiving trastuzumab in routine clinical practice, we had the opportunity to examine the effect of TBP in a large population. PATIENTS AND METHODS: Among a total of 1843 trastuzumab-treated patients, a sub-cohort of 418 fulfilled the selection criteria for the TBP analysis: 261 continued trastuzumab and 157 discontinued. Logrank tests and Cox models were used to compare survival and identify prognostic factors. RESULTS: Survival from progression was significantly longer in those patients continuing trastuzumab treatment beyond disease progression (TBP: median 22.1 months; no TBP: median 14.9 months; HR = 0.64; P = 0.00021). In addition to TBP, a positive endocrine receptor status, a longer relapse-free interval, no visceral metastasis, no concomitant chemotherapy during first-line treatment, and first-line response were independently significant prognostic variables for longer survival on multivariate analysis. CONCLUSION: The hitherto limited evidence for TBP benefit from randomized studies was confirmed. While a number of strong disease-related predictors for survival after first progression could be identified, the positive effect of trastuzumab continuation retained statistical significance in a multivariate model.

Case report: pulmonary vein stenosis following RF ablation of paroxysmal atrial fibrillation: successful treatment with balloon dilation.

Paroxysmal atrial fibrillation and atrial tachycardia may originate from a focal source in one or multiple pulmonary veins. A focal origin facilitates a potential cure amendable to radiofrequency ablation. Herein we report the case of a 16 year old adolescent male with a tachycardia induced cardiomyopathy who presented with very frequent paroxysmal episodes of atrial fibrillation, atrial flutter and atrial tachycardia. The origin of the arrhythmia was mapped to the secondary branches of the left lower pulmonary vein using an octapolar micro-mapping catheter. Immediately following application of three radiofrequency lesions, angiography of the left lower pulmonary vein revealed a region of focal stenosis at the site of energy application, with delayed pulmonary venous emptying. Attempts to relieve any element of spasm using direct administration of nitroglycerin were unsuccessful. Three months later repeat catheterization revealed an unchanged region of tight anatomical stenosis. Balloon dilation of two stenotic areas resulted in dramatic relief of the obstruction and improved venous drainage. Recatheterization 6 months later revealed mild restenosis that was successfully redilated. Intracardiac ultrasound demonstrated focal constriction. Care should be exercised in attempting RF ablation in distal arborization sites of the pulmonary veins in children, because of the small caliber compared to adult subjects. Radiofrequency induced focal areas of stenosis may be amenable to balloon catheter dilation.

Ductus arteriosus in premature infants beyond the second week of life.

Persistent patency of the ductus arteriosus (PDA), common in premature infants, is associated with severe respiratory distress. The likelihood and significance of finding PDA in premature infants beyond the second week of life is unknown. We retrospectively analyzed all echocardiograms obtained between 1987 and 1992 on infants <35 weeks' gestational age. Of 446 echocardiograms 77 were obtained from infants >/=14 days. Of the 77 infants, 17 (22%) were found to have PDA (group 1) and the remainder did not (group 2). Forty-eight infants had been diagnosed as having PDA prior to 14 days of age. Of these infants, 16 were from group 1. Thus only 1/17 (6%) infants diagnosed as having PDA after 2 weeks did not have a history of PDA. The presence of PDA after 2 weeks did not relate to duration of oxygen therapy, ventilator therapy, or hospital stay. Furthermore, late closure of PDA in a subgroup of 11 infants did not appear to affect these parameters. It was concluded that premature infants beyond the second week of life are unlikely to have PDA if PDA had not been diagnosed during the first 14 days. Closure of PDA beyond the second week may not improve the infant's respiratory status.

Intermediate results of the extracardiac Fontan procedure.

BACKGROUND: Fourteen children (ages 2 to 14 years) and 1 adult (32 years) have undergone a modification of the Fontan procedure in which an extracardiac lateral tunnel or conduit is used in combination with staged or simultaneous bidirectional Glenn shunt(s). METHODS: Extracardiac lateral tunnels (n = 9) were constructed using a polytetrafluoroethylene patch (n = 7), pericardial patch (n = 1), or in situ pericardial flap (n = 1). Extracardiac lateral conduits (n = 6) were constructed using nonvalved homografts (n = 2) or polytetrafluoroethylene tube grafts (n = 4). Fenestrations were created in 4 patients (2 each in extracardiac lateral tunnel and extracardiac lateral conduit patients). Aortic cross-clamping was completely avoided in 12/15 patients (aortic cross-clamping in 2 patients for atrial septal defect enlargement and 1 for Damus-Kaye-Stansel procedure). RESULTS: There have been no operative deaths. Prolonged postoperative chest tube drainage (> 2 weeks) has been rare (n = 1). At follow-up (range, 6 to 54 months; mean, 27.5 months), all patients are in New York Heart Association class I or II and remain in normal sinus rhythm. Late protein-losing enteropathy was seen in 1 patient and was successfully treated by percutaneous creation of a stented fenestration from the extracardiac tunnel to the systemic atrium. Late catheterizations reveal unobstructed extracardiac lateral tunnel function and low pulmonary pressures (range, 11 to 13 mm Hg). Advantages of the extracardiac Fontan include (1) avoidance of aortic cross-clamping in most patients, (2) the hemodynamic benefits of total cavopulmonary connection, (3) avoidance of atriotomy and intraatrial suture lines, (4) preservation of sinus rhythm and no arrhythmias at 2 year follow-up, (5) drainage of the coronary sinus to low pressure atrium, (6) allowance for early/late fenestrations, (7) prevention of baffle leaks and intraatrial obstruction, and (8) allowance for growth (tunnel procedures only). CONCLUSIONS: We recommend this extracardiac procedure for all suitable patients undergoing surgical conversion to the Fontan circulation.

Pulmonary vein stenosis.

Pulmonary vein stenosis (PVS) is a rare disorder. Accurate diagnosis often requires anatomical examination. We report four children with pulmonary vein stenosis. Autopsy showed bilateral lesions in two patients who were thought clinically to have unilateral disease. A diagnosis of PVS was made at autopsy in the third case. Intimal and medial fibromuscular proliferation was noted in extrapulmonary and intrapulmonary veins. Some of the fibromuscular proliferation were eccentric, resembling organized thrombi. In one case a focal organizing thrombus was found in a clinically unobstructed but anatomically narrowed veno-atrial junction. In another case injection of contrast medium into the stenotic pulmonary vein (PV) showed anastomosis between PV and bronchial vessels as well as small pulmonary arteries. Bilateral hypertensive arteriopathy was observed in unilateral and bilateral PVS. Our histological finding of intrapulmonary venous lesions in the lobes in which PVS was not detected clinically suggests that during surgical correction of unilateral PVS multiple biopsies of the opposite lung may help to evaluate possible bilateral disease. Our study also suggests that thrombosis in a stenotic pulmonary vein may further compromise the lumen and contribute to the progression of pulmonary vein obstruction. The possible pathogenesis of bilateral pulmonary hypertensive arteriopathy in unilateral PVS also is discussed.

The extracardiac total cavopulmonary connection for definitive conversion to the Fontan circulation: summary of early experience and results.

Between July 1991 and March 1993, five children (ages 2 to 6 years) with complex congenital heart disease have undergone a new operation for conversion to the Fontan circulation. This procedure combines a bidirectional Glenn shunt with an extracardiac lateral tunnel (ELT) to carry systemic venous return to the pulmonary arteries (PAs). The ELT was constructed so that the circumference consists of Gore-Tex (2/3) and lateral epicardial atrial wall (1/3). The ELT can be performed with all varieties of single ventricle physiology, as in our patients with tricuspid atresia (n = 3), dextrocardia (n = 1), and situs inversus with levocardia (n = 1). PA reconstruction was required in four patients. At follow-up from 1 to 20 months, all patients are in New York Heart Association Class I and in normal sinus rhythm. Postoperative catheterization has revealed low PA pressures (< or = 12 +/- 1 mmHg) and angiography has shown excellent ELT function with brisk flow into the PAs bilaterally. All patients maintain an O2 saturation > 94% on room air. The advantages of this new extracardiac modification of Fontan's operation are: (1) aortic cross-clamping is not usually required; (2) incorporation of lateral atrial wall in ELT allows for growth while permitting construction of a fenestration or adjustable atrial septal defect in high risk patients; (3) absence of atriotomy and intraatrial suture lines may decrease late risk of arrhythmias; (4) early or late baffle leaks cannot occur; (5) intraatrial obstruction from the baffle cannot occur; (6) coronary sinus remains in low pressure atrium; and (7) hydrodynamic benefits of the total cavopulmonary connection are preserved. We recommend this procedure for patients undergoing surgical conversion to the Fontan circulation.

Cardiovascular reflex abnormalities in children and adolescents with diabetes mellitus.

OBJECTIVE: To assess the usefulness of specific cardiovascular reflex tests in childhood and to estimate the prevalence of cardiovascular reflex abnormalities among children with IDDM. In adults, abnormal cardiovascular reflexes are a frequent complication of diabetes, associated with increased morbidity and mortality. RESEARCH DESIGN AND METHODS: We measured heart-rate responses to deep breathing and standing in ambulatory children with and without IDDM between 6-19 yr of age. A subgroup of the IDDM patients was retested after 1 yr. RESULTS: We found the best techniques for detecting cardiovascular reflex abnormality in children were as follows: to record heart-rate responses to deep breathing either as the change in heart rate corrected for inspiratory heart rate or as the ratio of R-R intervals during expiration and inspiration; and to use the Maximum-minimum ratio for heart-rate responses to standing. HR-DBc was lower in diabetic than nondiabetic children (28.6 +/- 9.2% [n = 248] vs. 33.6 +/- 6.8% [n = 60]; P < 0.0005). Similarly, E:I was lower in children with IDDM than control subjects (1.42 +/- 0.19 [n = 248] vs. 1.52 +/- 0.15 [n = 60]; P < 0.0005). In the IDDM group, 21% of the children had abnormal HR-DBc or E:I responses. HR-STND M/m was lower in children with IDDM than control subjects (1.28 +/- 0.20 [n = 167] vs. 1.38 +/- 0.22 [n = 45]; P < 0.014). Among children with IDDM, 11.4% had abnormal HR-STND M/m responses. Overall, 29% of IDDM children tested abnormal in either HR-DBc or HR-STND M/m; 3% were abnormal in both tests. We found no correlation of HbA1c levels (n = 74) or duration of diabetes with either HR-DB, expiration to inspiration (n = 248), or HR-STND M/m (n = 167). In patients who were reevaluated after 1 yr we found a high correlation of the first and repeat HR-DBc tests (r = 0.47, n = 75, P < 0.0001), E:I (r = 0.53, n = 75, P < 0.0001), and HR-STND M/m (r = .49, n = 37, P < 0.002), but no evidence of an increased number of children with cardiovascular reflex abnormality. CONCLUSIONS: With easily performed HR-DB and HR-STND tests, we detected cardiovascular reflex abnormality in 29% of children with IDDM. We found no correlation of changes in HR-DB and HR-STND with HbA1c or duration of diabetes. These tests provide an objective clinical measurement to monitor autonomic neuropathy in children with diabetes.

Histologic, immunohistochemical and morphometric study of the mucosa of the total gastric antrum in patients with carcinoma of the stomach.

Examination of gastrin-immunoreactive G-cells, somatostatin-immunoreactive D-cells, enterochromaffin cells and 5-hydroxytryptamine-immunoreactive (5-HT-immunoreactive) cells of the completely mapped histologic antrum (70 to 100 tissue blocks) was done in 20 normal stomachs of persons between 17 and 94 years of age (from forensic autopsy). Results were compared with those of nine patients between 48 and 76 years of age with total gastrectomy for carcinoma of the proximal part of the stomach. Cell counts and morphometric examinations were performed. Results were summarized for the proximal (I), middle (II) and distal (III) one-third of the antrum and for the major (A) and minor (B) curvature side. In normal stomachs, the G-cell count was 2.52 percent of the total gland cell count in AI; 4.25 percent in AII and 4.77 percent in AIII. In BI, the numbers were 2.5 percent, in BII, 3.73 percent and 4.06 percent in BIII. The D-cell count was 0.47 percent in AI, 0.62 percent in AII and 0.58 percent in AIII. The numbers were 0.44 percent in BI, 0.51 percent in BII and 0.51 percent in BIII. In the antrum of the stomach with carcinoma, the G-cells revealed a non-significant 20 to 70 percent lower cell count, while the D-cell count was reduced insignificantly by as much as 35 percent in all areas. The 5-HT-immunoreactive cell count in normal stomachs is 0.25 percent in AI of the total gland cells, 0.32 percent in AII and 0.39 percent in AIII. In B, it shows numerically no difference to that of A. Contrary to the cell count in normal stomachs, the carcinoma antrum revealed a 200 to 400 percent increase in 5-HT-immunoreactive cell count, highly significant in every area of the antrum. Because 5-HT is known as a growth stimulant, especially for tumors, an increase in 5-HT-immunoreactive cells may be a factor that contributes to the initial histologic changes observed during the early phase of gastric tumor.

Extracardiac total cavopulmonary connection.

We report the case of a 6-year-old child who underwent definitive conversion to the Fontan circulation using a newly conceived operative technique. This new procedure allows the operation to be performed as a totally extracardiac operation and is based on the hydrodynamic principles of the total cavopulmonary connection.

Muscle involvement in the scleroderma syndromes.

Muscle involvement was identified in 14 patients with scleroderma or a connective tissue disease overlap syndrome with predominant features of scleroderma. Patients presented with symmetrical proximal weakness indistinguishable from other inflammatory myopathies. Creatine kinase and electromyography were useful to demonstrate muscle involvement. Muscle histopathology demonstrated primarily the vasculopathy of scleroderma or polymyositis in similar numbers of patients. Scleroderma vasculopathy and polymyositis generally occur without specificity to diffuse scleroderma, the calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, telangiectasia syndrome, or an overlap syndrome with arthritis. Polymyositis also occurs when the vasculopathy of scleroderma involves other organ systems.

Reflex epilepsy and nonketotic hyperglycemia in the elderly: a specific neuroendocrine syndrome.

We present five elderly patients with focal reflex or posture-induced seizures and nonketotic hyperglycemia (NKH). Each patient exhibited interictal focal findings, such as hemiparesis or hemisensory or aphasic deficits. With control of the hyperglycemia, the seizures stopped, and the neurologic deficits resolved. The syndrome of focal reflex epilepsy and neurologic deficits in the elderly is transient and almost invariably related to NKH, thus representing a specific neuroendocrine syndrome.

Endogenous inhibition of red blood cell Na,K-ATPase in essential and pregnancy-induced hypertension.

Digoxin-like inhibitors of Na,K-ATPase have been implicated in the pathophysiology of essential (EH) and pregnancy-induced hypertension (PIH). A technique that enhances dissociation of digoxin from red blood cells (RBC) was used to displace endogenous digoxin-like substances from RBCs. RBC membranes were preincubated in Na and ATP (Release) or Na,K,Mg and ATP (Retention) prior to measuring ATPase activity. Groups studied were: 39 men with EH and 34 controls plus 10 women with PIH and 17 normotensive controls. All displayed similar increases in Na,K-ATPase activity (24.0 +/- 7.9%) following Release. Plasma digoxin immunoreactivity (DI) was measured in pregnant women, m = 0.25 +/- 0.07 ng/ml. No DI was detected in nonpregnant women, but RBCs from these women demonstrated the same increase in Na,K-ATPase activity after Release. The 24% increase in activity achieved by Na and ATP preincubation can be reversed by adding K and Mg to the Release suspension. However, after RBC-bound digoxin is displaced by Release preincubation, addition of K and Mg cannot promote renewed binding and pump inhibition. Thus, the observed endogenous inhibition is not due to displacement of a digoxin-like substance but probably is related to alteration of the enzyme-membrane interaction. Furthermore, even though pregnant women demonstrate DI, an inhibitory substance with digoxin-like binding could not be recognized using this technique.

Task-specific organization of activity in human jaw muscles.

Coordination of jaw muscle activity for speech production sometimes has been modeled using nonspeech behaviors. This orientation has been especially true in representations of mandibular movement in which the synergy of jaw muscles for speech production has been suggested to be derived from the central pattern generator (CPG) for chewing. The present investigation compared the coordination of EMG activity in mandibular muscles over a range of speech and nonspeech tasks. Results of a cross-correlational analysis between EMG signals demonstrated that the muscle synergies of the mandibular system depend on task demands. Contrary to some of the models discussed, continuous speech production yielded activation patterns that were clearly not related to coordinative patterns generated by the chewing CPG.