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.

Echocardiographic evidence of left ventricular bands in infants and children.

M-mode and two-dimensional echocardiographic studies were carried out on 100 consecutive neonates, infants and children to evaluate the incidence of left ventricular chordae or bands. These fibromuscular structures were noted in 61 subjects and were seen to traverse both the long and short axes of the left ventricle. Although a single left ventricular band was usually noted, several subjects had multiple bands. These bands were seen in normal subjects and in patients with a wide variety of congenital cardiac lesions. Distortion of left ventricular geometry was noted in one subject. Identification of these linear echoes is essential to allow differentiation of left ventricular bands from true anatomic left ventricular outflow tract obstruction or septal thickening.

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.

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.

Red cell cotransport activity and sodium content in black men. Relationship to essential hypertension.

Furosemide-sensitive sodium and potassium cotransport and intracellular sodium content ([Na]i) were measured in erythrocytes (red blood cells, RBCs) from a population of 90 adult black men with and without essential hypertension (EH). The mean values for sodium cotransport activity, expressed as furosemide-sensitive Na efflux (mmol/liter RBC/hr), were not significantly different among the EH patients and two control groups, normotensive subjects with a positive history (N+) and those with a negative family history (N-) for hypertensive disease (EH: 154 +/- 123, n = 53; N+: 167 +/- 93, n = 12; and N-: 207 +/- 142, n = 20; all values are means +/- SD). The mean [Na]i 9.66 +/- 3.02 mmol/liter RBC (n = 56) for the EH group was greater than the mean value for the N- control group (7.96 +/- 1.97, n = 20; p less than 0.05). The N+ group also displayed a higher mean [Na]i (10.38 +/- 3.18, n = 12; N+ vs N- p less than 0.01). Although there was substantial overlapping of [Na]i values between the groups and no clear dividing line, the distribution curve of the [Na]i values in EH was skewed toward higher concentrations than in N-. Nevertheless, we must conclude that erythrocyte cotransport and [Na]i are not clinically useful in the identification of EH in black men.

Red blood cell Na+,K+-ATPase in men with newly diagnosed or previously treated essential hypertension.

Alterations of cellular function of Na+,K+-adenosine triphosphatase (ATPase; Na+-K+ pump) have been implicated in the pathophysiology of essential hypertension. Therefore, this aspect of red blood cell (RBC) Na metabolism was studied in black men with newly diagnosed, untreated essential hypertension (NEH) and a normotensive control group. RBC Na content, Na+-K+ pump number (ouabain binding sites), and pump activity were measured. No statistically significant differences were found between the two groups for any of these three parameters. However, a group of previously treated essential hypertensive subjects (PEH) who had been withdrawn from therapy in the preceding 6 weeks were also studied. This group differed significantly from the NEH subjects in regard to all RBC Na+-K+ pump parameters. Their RBC Na content (10.27 +/- 3.23 vs 7.77 +/- 2.52 mmol Na/LRBC; p = 0.006) was higher, and their Na+-K+ pump activity (166 +/- 50 vs 221 +/- 87 nmol inorganic phosphate/mg membrane protein/hr; p = 0.03) and Na+-K+ pump number (213 +/- 40 vs 284 +/- 85 binding sites/RBC; p = 0.001) were lower compared with those in NEH subjects. Although the PEH subjects were older and somewhat less hypertensive than their NEH counterparts, these factors were not found to influence the Na+-K+ pump parameters. These results indicate that chronic diuretic therapy of patients with essential hypertension is associated with a reduced number of RBC Na+-K+ pumps. Since RBCs are not considered target cells for diuretics, the effects of these drugs on RBC electrolyte metabolism may occur at the time of erythropoiesis by the production of RBCs with fewer Na+-K+ pumps.(ABSTRACT TRUNCATED AT 250 WORDS)

Perspective on carbamazepine-induced water intoxication: reversal by demeclocycline.

Carbamazepine (CBZ)-induced water intoxication occasionally limits its usefulness in refractory seizures and trigeminal neuralgia. Fluid restriction, CBZ dose reduction, or concomitant phenytoin therapy may be impractical or ineffective. Demeclocycline (7-chloro-6 demethyl tetracycline) (DMC) corrected the CBZ-induced water intoxication in a 51-year-old man with refractory complex partial seizures and a normal antidiuretic hormone (ADH) level. DMC inhibits ADH-sensitive adenylate cyclase activity in the renal collecting duct and may be useful in correcting the ADH-like or renal antidiuretic effect of CBZ.

Reversible coma with prolonged absence of pupillary and brainstem reflexes: an unusual response to a hypoxic-ischemic event in MS.

A quadriparetic man with multiple sclerosis became comatose with absent pupillary and brainstem reflexes for 48 hours following a respiratory arrest. A periodic electroencephalographic pattern was recorded 12 hours after arrest suggesting a poor prognosis. Despite the poor prognostic signs, clinical recovery to baseline occurred within 2 weeks. Reversible coma with absent pupillary and brainstem reflexes may occur in MS patients for prolonged periods and lead to potential errors in prognosis if not recognized.

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.

Familial parkinsonism, apathy, weight loss, and central hypoventilation: successful long-term management.

We report a third family with six members with features of the syndrome of familial parkinsonism, depression, weight loss, and central hypoventilation. Patients with this syndrome typically die suddenly, presumably from central respiratory failure. Following multiple respiratory arrests, one affected member was successfully managed with aggressive pulmonary care, tracheostomy, and intermittent home mechanical ventilation, which, combined with carbidopa/levodopa, allowed for a functional lifestyle with improvement in apathy, mobility, and nutritional status.

Physiological fitness measures and sensory and motor performance in aging.

This study investigated relationships between a multivariate index of physiological fitness and the degree of age-related sensory and motor performance deterioration observed in a group of 70 male subjects (age 63 +/- 12 years). Physiological fitness was determined by reducing a battery of resting pulmonary, hemodynamic and biochemical variables to a single score, the Index of Physiological Status (IPS). In addition, where possible, maximal oxygen uptake (VO2max) was predicted from submaximal values using a standard treadmill procedure. Both IPS and VO2 scores were significantly correlated with age (p less than .01). High scores on the IPS were associated with faster reaction times, improved hearing at high frequencies, greater phonatory control and improved lens accommodation. In contrast, VO2 measures were found to be insensitive to changes in sensory and motor performance. While all subjects were able to complete the tests required for the generation of the IPS, 33% of the participants were unable to complete the treadmill protocol required for estimation of VO2max. These data suggest that a simple noninvasive instrument for the evaluation of physiological fitness, which is applicable to almost all elderly subjects, may be more sensitive to age-related sensory and performance changes than more traditional exercise based measures which require the exclusion of less healthy subjects.

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.

Subclavian flap repair of coarctation of the aorta in neonates. Realization of growth potential?

The subclavian flap repair for coarctation of the aorta allows potential for growth by utilizing autogenous tissue. Although well documented in young children, its promise in the tiny neonate warrants further evaluation. Since August, 1979, 29 patients, including 24 infants, have undergone subclavian flap repair at the University of Maryland Hospital. Weights ranged from 1.4 to 5 kg (mean 3.2 kg). All patients less than 6 months old had associated intracardiac defects and were in severe congestive failure. Fifteen responded to preoperative prostaglandin infusions. The overall early mortality was 14%; among the neonates it was 21%; and among those operated upon within the first week of life, 33%. There was one intraoperative death among the eight patients who underwent simultaneous pulmonary artery banding. There were no deaths among patients older than 5 days at operation. Four of the five neonates who died had some variant of hypoplastic left heart syndrome, with severe stenosis or atresia of the systemic atrioventricular valve, critical aortic stenosis, or hypoplastic left ventricle. Twenty-two survivors continue to do well up to 3.7 years postoperatively (mean follow-up 26 months). At follow-up all patients are normotensive with brisk lower extremity pulses. Patients now weigh 1.3 to 6.9 (mean 2.3) times their operative weight, and only one patient has a measured arm-to-leg gradient greater than 10 mm Hg (mean gradient 3.7 mm Hg). Seven of the neonates have undergone repeat catheterization, and all had satisfactory growth of the subclavian flap segment of repair and no gradient. Two older patients (3 and 4 years old at operation) have undergone exercise testing 3.7 years after repair, with peak exercise gradients of only 7 and 15 mm Hg. We therefore continue to utilize this technique for the treatment of coarctation even in tiny neonates.

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.

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.

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.

Cardiologic perspectives of chest pain in childhood: a referral problem? To whom?

Discussed are the diagnostic and therapeutic issues of cardiac disease in the pediatric age group that have the potential to result in ischemic ventricular dysfunction. The discussion then turns to the nontraumatic thoracic, gastrointestinal, and psychogenic disturbances that are likely to produce symptoms of chest pain. In this context, the question of when to refer, when to reassure, when to begin a diagnostic evaluation, and when to institute longitudinal follow-up are addressed.

Structural failure of a Starr-Edwards aortic track valve.

Structural failure of a Model 2400 Starr-Edwards aortic track valve occurred suddenly, 4 years after implantation. At operation, the valve cage was removed from the descending aorta. Examination of the excised prosthesis disclosed minimal cloth wear and no evidence of infective growth; however, three struts were fractured above their insertion into the valve ring. To our knowledge, this type of valve malfunction has not been previously noted.

Use of computed axial tomography to diagnose vascular ring in an infant.

Computed axial tomography was performed in an infant with severe respiratory distress. A right aortic arch and paratracheal density was visualized, suggesting a vascular ring. Angiography confirmed a right aortic arch, mirror-image branching, and a left patent ductus arteriosus.

Myocarditis as a complication of infantile Chlamydia trachomatis pneumonitis.

The case is presented of an infant with chlamydia pneumonitis who developed clinical and electrocardiographic signs of myocarditis. No significant antiviral antibodies developed. Elevated antichlamydial IgG titers were demonstrated during the initial phase. These declined during convalescence. This report provides further evidence associating C. trachomatis with infectious myocarditis.