C. Ashton. University of New Mexico.

March 11 should be a fairly nice day in most parts of the country purchase sildalist online now; how- ever discount sildalist generic, you are coiled up in a blanket looking outside your ofce window as snow is falling at a very alarming rate generic sildalist 120mg without prescription. The scene is the same in at least three other states and transportation is coming to a standstill (Douglas, 2005). As director for health and human services it would be important to determine what groups of people are vulnerable to severe cold. Once these groups have been identifed, it is important to gather information on what resources are available and how those resources can be 99 100 ◾ Case Studies in Disaster Response and Emergency Management deployed. Motor vehicles are not available and you will have to fnd some other way to distribute any resources that are needed. You will need to fnd some other methods of communi- cating with the population, municipal and state leaders, other agencies, and frst responders. Tis means an administrator will have to know where to locate supplies and get agreements in place with local agencies in order to distribute the resources to the appropriate groups of people considered to be vulner- able to cold weather. In addition, supplies should be stockpiled in the way of food, fuel for heating, heaters, and water for diferent groups of people that may get isolated if roads are closed down and they are unable to get required resources. Additionally, an administrator will need to fnd alternative ways to get supplies to those types of isolated groups of people. Stage 2 of the Disaster As the day wears on, reports from the National Weather Service indicate that over 50 inches of snow has fallen throughout the day in diferent areas of the country (Douglas, 2005). Additionally, your communication infrastructure has faltered and fuel is now very limited (Brunner, 2007). You must see if you can get additional supplies brought in from states and areas of the country that are not afected by bad weather. The train would be a good source of transporta- tion to bring in large amounts of supplies quickly. Tis means that leaders in those areas of the country need to be communicated with and an agree- ment needs to be put in place to gather up the necessary food, water, and fuel for the residents in snowed-in communities. If possible, shipping may be another possibility to bring in large amounts of supplies from southern states. Additionally, you will need to have adequate resources to keep railroad tracks clear and waterways open for shipping. Temporary shelters need to be identi- fed for those that have no fuel or have no home. With electronic means of communica- tion being limited, using couriers in the form of horseback, ships, or trains * The frst automobile was invented in 1885 in Germany. Depending upon the technology of the region, infrastructure condi- tion, and availability of fuel, frst responders even in modern times may not have vehicles to utilize for disaster response operations. Case Studies: Other Natural Disasters ◾ 101 will be your main form of communication over any great distance. For com- municating locally, a series of couriers could be utilized to send messages to and from local municipality leaders. The resources will need to be dedicated to logistical eforts of keeping passages open and distributing goods, and a medical plan of action will need to be implemented for patients dealing with starvation and frostbite. Stage 3 of the Disaster It is now March 13 and your ofce is fooded with notifcations of people starving to death or freezing to death in their homes (Schmid, 2005). In addition, there are now several people who are being injured due to the fre stations being closed down due to the roads being completely closed, which is leaving fres going unchecked throughout the region (Brunner, 2007). At this point an administrator will need to take stock of the situation and determine what has been done to alleviate the food shortage situation and what should be done to alleviate the food shortage situation. Resources may have to be reallocated to get logistics where they need to be, and manpower will need to be allocated for hospital staf as well as getting the fre stations back in operational use. You will need to allocate resources toward logistic eforts and getting emergency management services in place. Manpower will be crucial in getting roads, railways, and ship channels clear to receive cargo for food, water, and fuel. Getting skilled personnel to man hospitals and fre stations will prove to be crucial during this phase of the crisis. It has been reported to you that 100 sailors have now died on 200 ships that have been frozen in place along the coastline (Douglas, 2005). The fuel has now been depleted and the unchecked fres have cost the region over $25 million in property damages alone (Brunner, 2007). Since so many ships are frozen in the harbor, the local administrators should provide shelter for sailors whose ships are stranded in the harbor and potentially use those individuals to provide logis- tics and clear transit areas. Additional personnel should be allocated to fght fres that are currently roaring unchecked. Your main focus should be to keep the logistical pathways clear; distribute food, water, and fuel to 102 ◾ Case Studies in Disaster Response and Emergency Management residents; and fght any fre that is currently active. Additionally, people who are without shelter or fuel should be evacuated to temporary shelters or even out of the region temporarily until logistics have improved. Key Issues Raised from the Case Study Having a good logistical plan in place and a reserve of fuel, food, and water can make the diference in surviving this disaster response situation. With adverse con- ditions, both the very young and very old are susceptible to becoming ill or dying as a result of extreme cold. Administrators need to keep these factors in mind when determining what parts of the population are the most vulnerable and where the resources should be sent as a priority. It is critical to have a fuel depot on hand for transportation and heating needs in case infrastruc- ture is damaged and people are isolated. Additionally, the communication infra- structure had no redundancy, leaving people without any means to communicate efectively with external entities. An administrator must make plans to use another manner of communication if the primary systems have failed. Items of Note The Great Blizzard of 1888 is still known as the worst snowstorm in American history. As a result of not having enough food, water, or fuel, a number of people died who would have been able to survive if the proper resources had been available. Lions of Tsavo, Africa, 1898 Stage 1 of the Disaster You are the director of engineering for a public works project to construct a vital bridge that is part of the railroad from Kenya to Uganda. On this project, the British government has spent 1 million pounds on rolling stock and labor from India alone (Monitor Reporter, 2012). The railway has been under construction since 1895, and the British government is anxious for you to get the bridge completed so that the rest of the railway can be completed ahead of any potential competition (Nairobi Chronicle, 2008). You have a design for the bridge Case Studies: Other Natural Disasters ◾ 103 and are about to begin work when you arrive at Tsavo to oversee the construction. After a week of being on-site, you receive word that two of your best workers have mysteriously disappeared. Some workers believe they had been killed by lions as they slept in their tents (Patterson, 1919).

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Patients with diabetes are predisposed for certain infections which include emphysematous pyelonephritis buy cheap sildalist line, emphysematous cholecystitis purchase 120mg sildalist with visa, malignant otitis externa purchase sildalist 120 mg online, rhino–orbito–cerebral mucormycosis, and liver abscess. The increased risk for these infections in patient with diabetes is due to glucotoxicity-mediated lazy leukocyte syndrome and impaired humoral and cellular immunity (Fig. Increased prevalence of childhood obesity as a result of sedentary lifestyle and consumption of calorie-dense food predisposes for the early development of diabetes. Biochemistry revealed fasting plasma glucose of 190 mg/dl, postpran- dial glucose 220 mg/dl, and HbA1c 8. He 12 Diabetes in the Young 415 should be carefully examined for other features of insulin resistance (double chin, skin tags, and central obesity), hypertension, and xanthelasmas. The index patient was advised to follow lifestyle modifcation and was initiated on metformin 1 g twice a day after meals. The monogenic forms of diabetes are rare and contribute only 1–2 % of individuals with diabetes. During adolescence and early adulthood, these individuals have normal fasting plasma glucose, but have hyperglycemia during oral glucose tolerance test. The important differentiating features between the two disorders are summarized in the table given below. Therefore, glucokinase is a key enzyme which regulates the rate of entry of glucose into the glycolytic pathway and its subsequent metabolism in β-cell. The most affected individuals are asymptomatic and are detected during screening (e. How do hepatocyte nuclear transcription factors regulate insulin secretion and glucose metabolism? Hepatocyte nuclear transcription factors are expressed not only in the liver but also in the pancreatic β-cells and urogenital tissues. These proteins regu- late tissue-specifc gene expression and thereby determine growth and development, as well as facilitate metabolic signaling in these organs. During embryogenesis, these transcription factors act in concert to promote islet devel- opment and regulate the expression of insulin gene, and genes-encoding pro- teins which are linked to insulin secretion. Diabetes is prevalent in approximately 60 % of individuals and occurs at an early age. These individuals are often diagnosed to have type 2 diabetes and started on oral antidiabetic drugs; however, most of these individual will require insulin within a few years. Exogenous insulin therapy results in decreased expression of β-cell autoantigens and may activate Treg cells and inhibit autoreactive T cells, thereby delaying the ongoing immunoinfamma- tory destruction of β-cells. Preservation of residual β-cell function helps to pre- vent wide swings in blood glucose and decrease the risk of hypoglycemia. Sulfonylureas are to be avoided as these drugs enhance the expression of autoan- tigens in β-cells and hasten the immunoinfammatory process. Metformin can be used in some patients who have features of insulin resistance, particularly in obese individuals. The alkaloids linamarin and lotaustralin present in cassava produce cyanide compounds which are detoxifed by sulfur-containing amino acids. These amino acids are defcient in individuals with malnutrition; therefore, accumulation of cyanogens result in chronic pancre- atitis. Increased secretion of a putative peptide termed as pancreatic stone protein has also been suggested for the development of pancreatic calcifcation. Microvascular complications are common; however, macrovascular complica- tions are rare. This dichotomy is possibly due to lack of hypertension and ath- erogenic lipid profle. Despite severe hyperglycemia, ketosis is less common because of the presence of residual β-cell function, loss of α-cell function (decreased glucagon), reduced availability of non-esterifed fatty acids due to lack of subcutaneous fat, and carnitine defciency associated with malnutrition. For glycemic control, majority 12 Diabetes in the Young 423 (85%) of patients require insulin therapy. Pancreatic enzyme supplements are recommended in patients with steatorrhea, and fat soluble vitamins should be adequately replenished. If pain is unbearable or nonresponsive to medical management, surgical interven- tion should be considered. Ketosis-prone diabetes refers to a heterogeneous group of disorders with pro- pensity to develop diabetic ketosis/ketoacidosis either at onset or during the course of disease. However, this classifcation adds confu- sion to the existing nomenclature of diabetes and has limited utility in clinical practice. The majority of patients were overweight/ obese (67 %) and had strong family history of diabetes (88 %). Evidence of islet autoimmunity was conspicuously absent, and β-cell function was relatively preserved in these patients. The mechanisms proposed include severe oxidative stress, and glucotoxicity-mediated β-cell dysfunction and insulin resistance. With intensive insulin therapy, glucotoxic- ity and oxidative stress are ameliorated which result in restoration of β-cell function and consequent insulin independence in majority of these patients during follow-up. Defective growth and development of β-cell during embryogenesis and in fetal life results in decreased insulin secretion and consequently hyperglyce- 12 Diabetes in the Young 425 mia. Normally, genes at 6q24 locus which are inherited from the mother undergo imprinting (silencing), whereas paternal alleles remain active and are responsible for growth and development of β-cell dur- ing intrauterine life. The resolution of diabetes in these children is pos- sibly attributed to partial defect in metabolic signaling pathway involved in glucose-mediated insulin secretion and progressive maturation of “glucose-β- cell axis” with increasing age. This result in opening up of voltage- dependent calcium channel and allow the entry of calcium from extracellular fuid into β-cell, thereby initiating the process of insulin release by exocyto- sis. This is attributed to sulfonylurea-mediated improvement in β-cell sensitivity to incretins. Lipodystrophic diabetes is a group of metabolic disorders characterized by gen- eralized or partial wasting/loss of adipose tissue mass, severe insulin resistance, hyperglycemia, hypertriglyceridemia, and hepatic steatosis. Previously, this entity was referred as lipoatrophic diabetes; however, this nomenclature was reframed to lipodystrophic diabetes later, as it refers to the presence of either lipoatrophy and/or lipohypertrophy in these individuals. Patients with general- ized lipodystrophy have global loss of fat mass, as opposed to patients with partial lipodystrophy who have loss of adipose tissue mass in upper half of the body with accumulation of fat in lower half of the body. Both excess and defcient adipose tissue mass are associated with insulin resistance and hyperglycemia. Decreased fat mass in patients with lipodystro- phic diabetes results in a state of leptin defciency, which in turn leads to decreased hepatic and peripheral insulin sensitivity via central mechanism through arcuate nucleus of hypothalamus. In addition, increased circulating free fatty acids as a result of lack of deposition as triglyceride at eutopic site (adipocytes) lead to lipotoxicity. Decreased secretion of insulin-sensitizing adipocytokines (adiponectin) due to paucity of adipocytes further contributes to insulin resistance. Lipodystrophic syndromes are classifed into congenital and acquired lipodys- trophic syndrome. Congenital lipodystrophy is a result of mutations in genes responsible for adipocyte differentiation and growth and development, whereas acquired lipodystrophy is usually drug-induced.

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The physical examination demonstrates a hyperactive precordium generic 120mg sildalist visa, an accentuated first sound effective 120 mg sildalist, and a second sound that may move with respiration but it is quite variable buy 120mgmg sildalist with amex. Because of elevated pulmonary artery pressures, the pulmonary closure sound is accentuated. A separate crescendo– decrescendo systolic ejection murmur is heard over the upper left sternal border as a result of increased pulmonary blood flow. As described earlier, assessment of the internal cardiac crux from the apical and subcostal four-chamber projections provides excellent detail of the size and locations of defects in both the atrial and ventricular septa (see Fig. Deliberate superior and inferior angulation of the probe will permit inspection of the cross section of all five valve leaflets. The valve is inspected from the inferior margin of the atrial septum to the superior margin of the ventricular septum (46). It is not divided into right and left components and has no attachments to the ventricular septum. Similar to the double-orifice valve, a single papillary muscle will reduce the effective valve area and complicate the surgical repair. The Concept of “Balance” Two-dimensional echocardiography is essential for determining the relative sizes of the ventricles. These patients frequently have severe coarctation of the aorta and aortic arch anomalies. Right versus left ventricular dominance, based on a classification scheme from Bharati and Lev: left ventricular dominance (left panels) and right ventricular dominance (right panels) are demonstrated. In the left ventricle dominant case, the common atrioventricular valve opens predominantly into the left ventricle. Conversely, in the right ventricle dominant case, the common atrioventricular valve opens predominantly into the right ventricle. Imaging is best performed from the subcostal sagittal plane to show an en face view of the common atrioventricular valve. With standard 2-D echocardiographic imaging, both ventricles are appreciated from the apical four-chamber view. Determining “balance” with echocardiographic imaging is important as it forms the basis for deciding single versus two-ventricle surgical repair. The clinician must be aware of several caveats that make interpretation of “ventricular balance” less straightforward. For example, the severity of valve malalignment may not necessarily correlate with the degree of ventricular hypoplasia. Finally, the presence of a large left-to-right shunt may cause severe right ventricular enlargement with bowing of the septum to the left. To overcome the false impression of left ventricular hypoplasia on 2-D echocardiography, van Son et al. A: Diastolic frame from the subcostal sagittal imaging plane showing balanced ventricles. Morphometric analysis of unbalanced common atrioventricular canal using two-dimensional echocardiography. Due to right ventricular volume overload, the septum bows toward the left ventricle. However, if one assumes normalization of septal position, the potential volume of the left ventricle after repair may be predicted. Predicting feasibility of biventricular repair of right-dominant unbalanced atrioventricular canal. However, despite use of all imaging assessments, practitioners must realize that none of the methods factor in patient/cardiac growth. Enlargement of the right atrium is suggested by increased convexity of the right heart border, and left atrial enlargement may produce a characteristic flattening of the left heart border. The pulmonary artery is prominent, and the pulmonary vascular markings are increased. In an older child, when pulmonary vascular obstructive disease is suspected, there is a role for determining pulmonary vascular resistance. Severe pulmonary vascular obstructive disease (pulmonary vascular resistance of 2 >10 U·m ) is rare but has been reported in infants <1 year of age. Cardiac catheterization reveals increased oxygen saturation at both the right atrial and the right ventricular levels. Pulmonary blood flow is increased as a result of left-to-right shunting at both atrial and ventricular sites, and the degree of shunting depends upon the relationship of pulmonary to systemic vascular resistance. The timing of surgical intervention must account for development of pulmonary vascular disease in these patients at an early age. Children with associated Down syndrome may require surgical intervention at an earlier age due to their propensity to develop pulmonary vascular obstructive changes. In contrast, splenic anomalies and abnormalities of sidedness (situs) are rare in patients with Down syndrome. Others (55,56) have suggested that children with Down syndrome have pulmonary parenchyma hypoplasia and develop pulmonary vascular obstructive disease earlier than patients with normal chromosomes. The hemodynamic assessment of children with Down syndrome must take into account that these patients may have chronic nasopharyngeal obstruction, relative hypoventilation, and sleep apnea. These factors contribute to carbon dioxide retention, relative hypoxia, and elevated pulmonary vascular resistance. Patients with Down syndrome have a higher ratio of pulmonary to systemic resistance than patients without Down syndrome (57). This difference resolves with administration of 100% oxygen, suggesting that apparent hypoxia and hypoventilation are factors that can be corrected during hemodynamic evaluation. Fixed and elevated pulmonary vascular resistance has been demonstrated in 11% of Down syndrome patients <1 year of age (57). These objectives can be accomplished by careful approximation of the edges of the valve cleft with interrupted nonabsorbable sutures. On occasion, it is necessary to add eccentric annuloplasty sutures, typically in the area of the commissures to correct persistent central leaks. The repair is completed by closure of the interatrial communication (usually with an autologous or bovine pericardial patch), avoiding injury to the conduction tissue (59). However, the morphologic concepts and surgical methods, favored by Carpentier (60) and Piccoli et al. The finding of surgical complete heart block has been uncommon and would require permanent pacemaker implantation. For the symptomatic infant, surgical options include palliative pulmonary artery banding or complete repair of the anomaly. In that series, there was one surgical death, and the remaining patients had excellent palliation. In the modern era, most centers perform complete repair in small infants who fail to thrive. Once the patch is sutured into place, the bridging leaflets are resuspended to the patch.

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However purchase sildalist 120mgmg without prescription, there have been no reports of anaphylaxis from the use of intravascular latex catheter balloons buy sildalist 120mg lowest price. For any patient with any history of latex allergy undergoing cardiac catheterization buy discount sildalist on line, standard institutional protocols for latex precautions should be observed. Acknowledgments The authors wish to acknowledge previous authors of this chapter, Drs. Textbook References That Exclusively Discuss Cardiac Catheterization, Angiography, and Intervention 1. Ultrasound-guided versus landmark-guided femoral vein access in pediatric cardiac catheterization. A randomized-controlled study of ultrasound prelocation vs anatomical landmark- guided cannulation of the internal jugular vein in infants and children. A randomized trial of ultrasound image-based skin surface marking versus real-time ultrasound-guided internal jugular vein catheterization in infants. Anatomic relationship between the internal jugular vein and the carotid artery in preschool children—an ultrasonographic study. Transhepatic therapeutic cardiac catheterization: a new option for the pediatric interventionalist. Transhepatic vascular access for diagnostic and interventional procedures: techniques, outcome, and complications. Feasibility of pulmonary artery pressure measurements in infants through aorto-pulmonary shunts using a micromanometer pressure wire. Assessment of pulmonary hypertension in the pediatric catheterization laboratory: current insights from the Magic registry. Hydraulic formula for calculation of the area of the stenotic mitral valve, other cardiac valves, and central circulatory shunts. Diagnostic reference levels and effective dose in paediatric cardiac catheterization. Visualization of the pulmonary arteries in pseudotruncus by pulmonary vein wedge angiography. Enlarged bronchial arteries after early repair of transposition of the great arteries. Adverse event rates in congenital cardiac catheterization - a multi-center experience. Hazards of cardiac catheterization in children with primary pulmonary vascular obstruction. The use of heparinization to prevent arterial thrombosis after percutaneous cardiac catheterization in children. Fibrinolytic therapy for femoral arterial thrombosis after cardiac catheterization in infants and children. Cheatham Introduction Catheter-based techniques, whether palliative or corrective, are the accepted therapy for many congenital cardiac defects. Interventional, or, better termed, therapeutic catheterizations, were initiated by Dotter and Judkins, who first reported the treatment of peripheral vascular lesions during a catheterization in 1964 (1), when they dilated a stenotic peripheral vessel through a cutdown on the vessel. The next major innovative accomplishment and the first intracardiac therapeutic catheterization procedure for pediatric congenital heart disease were the balloon atrial septostomy done by Rashkind and Miller in 1966 (2). That procedure really “set the stage” for all therapeutic catheterization procedures used today. In 1967, Porstmann and colleagues reported the first nonsurgical corrective procedure in the catheterization laboratory with their description of a technique for closure of a patent ductus (3). Even though their device has not found widespread use, it set the stage for future development of transcatheter devices. One of the largest contributions to interventional cardiology has probably been made by Gruentzig, a Swiss-native who in 1976 reported on dilation of peripheral vessels with noncompliant balloons. This initiated a rapid innovative spurt within the congenital cardiac community during which narrowed lesions at various locations were treated with balloon angioplasty, frequently initially in a noncontrolled fashion. Jean Kan reported the first successful transcatheter static balloon pulmonary valvuloplasty (6) and Dr. Charles Mullins introduced endovascular stents into the management of patients with congenital cardiac lesions (8), and the long list of innovations reached another milestone when Dr. Phillip Bonhoeffer, a German cardiologist working in France in 2000, performed the first transcatheter pulmonary valve replacement in a human (9). Transcatheter valve therapies and other interventional therapies to treat patients with structural heart disease have rapidly increased over the last few years. These therapies are not limited anymore to patients with congenital heart disease. In this section, the most important therapeutic catheterization procedures performed as of this writing are discussed. This chapter is not intended as a complete and exhaustive textbook of interventional techniques, but instead should give the reader a general overview of therapeutic catheterization. Acknowledgment We have used and expanded upon this chapter published in other editions of this textbook and therefore acknowledge the previous contributions made by Drs. It should be emphasized that not every pediatric cardiologist, or, for that matter, every center, should offer every therapeutic catheterization procedure. For any procedures to be performed at any particular institution, minimal specific skills are required, special techniques must be mastered and maintained, and a large inventory of specialized and expensive catheters and devices must be stocked to offer the patient an optimal procedure. Absence of appropriate qualifications and equipment can result in unnecessary risk to the patient without a reasonable chance of the therapeutic catheterization procedure being successfully accomplished. In fact, even if the patient is not acutely harmed by the attempt, it is important to be aware of the fact that the next procedure in a more appropriate setting might be compromised by a previously unsuccessful attempt. Adverse Events and Quality Improvement For many years, reporting of procedure-related adverse events was limited mostly to single-center retrospective experiences, often without any clearly and consistently applied criteria of what would be considered an adverse event, and how its severity should be defined (11,12,13). The data derived from these registries often provided the only prospective multicenter outcome data for many procedure types. This registry documented not insignificant rates of adverse events, 10% for diagnostic cases, and 20% for interventional procedures. Higher severity (level 3 to 5) adverse events occurred in 9% of interventional cases, and 5% of diagnostic cases. The incidence of life-threatening adverse events has been reported to be as high as 2. However, to accurately compare adverse event rates and outcome between institutions and operators, an adjustment for case mix and hemodynamic vulnerability is required. Following the definition of procedure-type risk groups, Bergersen and colleagues reported on hemodynamic variables associated P. The Interventional Armamentarium General Considerations The spectrum of transcatheter procedures available for the treatment of children and adults with congenital heart disease has rapidly increased over the last three decades. With rapid progress that is being made in the development of new and more refined equipment, the operator has an inherent responsibility to keep up-to-date with these development efforts and to avoid procedural failures in situations where the use of a different type of equipment may lead to a very different outcome. Even though many interventional meetings have a focus on new device developments, the choice of appropriate balloons, catheters, sheaths and wires is in many situations even more important for a successful outcome. It is beyond the scope of this discussion to describe all available balloon catheters, but the operator has to make a well- informed decision on which balloon to use, based on profile, rated maximum pressure, available lengths, and degree of compliance and adjust his/her choice to suit specifically the therapeutic intervention that is intended.