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Patients with leaflet displacement fulfill a diagnostic criterion for Ebstein anomaly purchase tamsulosin 0.4 mg on line prostate cancer diet plan. In “tricuspid valve dysplasia” the leaflets are abnormal but not displaced (140) (Fig tamsulosin 0.4mg low price prostate cancer 10. Echocardiography confirms the diagnosis order 0.4 mg tamsulosin mastercard prostate health vitamins, determines the degree of tricuspid regurgitation, allows accurate evaluation of the tricuspid leaflets and subvalvar apparatus (displacement, tethering, dysplasia, etc. Tricuspid valve repair, rather than replacement, is the preferred treatment strategy when feasible, particularly in children. The tricuspid leaflets are thickened and chordae are shorter than normal (red arrow), restricting the motion of all three leaflets. Despite the restricted mobility, these leaflets are not adherent to the underlying myocardium and the apical displacement index, representing the offset of the mitral and tricuspid 2 valve septal insertions (white arrow), with only 6 mm/m. In 1905, William Osler, described the term “parchment heart,” but Henry Uhl reported the first case in 1952 (141). The septomarginal trabeculations and the papillary muscles of the tricuspid valve are normally muscularized (143). The absence of myocardium may be the result of primary nondevelopment of myocytes or a form of selective apoptosis. Congestive heart failure with associated peripheral edema, pleural effusions, and/or cyanosis are frequent signs and symptoms of Uhl anomaly. Echocardiography shows delaminated tricuspid leaflets that insert at the true anatomic annulus. Dysplastic, but nondisplaced, leaflets with severe valve regurgitation may be present. Treatment includes medical management of congestive heart failure, and drainage of pleural effusions. Indeed, cardiac transplantation may offer the best option for these rare patients (147). We also acknowledge the contributions of Dr Justin Horner to the Ebstein anomaly section and Dr Michael Epstein to the tricuspid atresia section. William Edwards and Gordon Danielson from Mayo Clinic whose contributions to our understanding of the anatomy, physiology, and surgical repair for patients with Ebstein anomaly cannot be understated. Prevalence, clinical presentation and natural history of patients with single ventricle. Tricuspid atresia associated with common arterial trunk and 22q11 chromosome deletion. A syndrome of tricuspid atresia in mice with a targeted mutation of the gene encoding Fog-2. Predictors of rhythm disturbances and subsequent morbidity after the Fontan operation. What is the clinical utility of routine cardiac catheterization before a Fontan operation? Doppler echocardiographic evaluation of pulmonary blood flow after the Fontan operation: the role of the lungs. Echocardiographic evaluation of the functionally univentricular heart after Fontan operation. Predictors of outcome after the Fontan operation: Is hypoplastic left heart syndrome still a factor? Improved early morbidity and mortality after the Fontan operation: the Mayo Clinic experience, 1987 to 1992. The Fontan procedure for tricuspid atresia: early and late results of a 25- year experience with 216 patients. Outcome and assessment after the modified Fontan procedure for hypoplastic left heart syndrome. Improving results of the modified Fontan operation in patients with heterotaxy syndrome. Predictors of early- and late-onset supraventricular tachyarrhythmias after the Fontan operation. Protein-losing enteropathy after Fontan operation for tricuspid atresia (imperforate tricuspid valve). Acquired combined immunodeficiency associated with protein losing enteropathy complicating Fontan operation. Protein-losing enteropathy after the Fontan operation: an international multicenter study. Clinical outcomes and improved survival in patients with protein losing enteropathy after the Fontan operation. Thromboembolic complications after Fontan procedures–the role of prophylactic anticoagulation. A multicenter, randomized trial comparing heparin/warfarin and acetylsalicylic acid as a primary thromboprophylaxis for 2 years after the Fontan procedure in children. Antithrombotic therapy in neonates and children: antithrombotic therapy and prevention of thrombosis, 9th ed: American College of Chest Physicians evidence-based clinical guidelines. Exercise tolerance and cardiorespiratory response to exercise after the Fontan operation for tricuspid atresia or functional single ventricle. Cardiorespiratory response to exercise after modified Fontan operation: determinants of performance. Cardiorespiratory response to exercise after the Fontan operation: a serial study. Influence of ventricular morphology on aerobic exercise capacity in patients after the Fontan operation. Lung function and aerobic capacity in adult patients following modified Fontan procedure. The precarious state of the liver after a Fontan operation: summary of a multidisciplinary symposium. Magnetic resonance elastography of liver: technique, analysis, and clinical applications. Uber einen sehr seltenen fall von insufficienz der valvula tricuspidalis, bedingt durch eine angeborene hochgradige misshildung derselben. Tricuspid valve disease with significant tricuspid insufficiency in the fetus: diagnosis and outcome. Correlation between echocardiographic and morphological investigations of lesions of the tricuspid valve diagnosed during fetal life. Total right ventricular exclusion procedure: an operation for isolated congestive right ventricular failure. Results of surgery for Ebstein anomaly: a multicenter study from the European Congenital Heart Surgeons Association.
You should evacuate all of your frefghters from the area to avoid any of them getting hurt or killed buy 0.2mg tamsulosin amex prostate bleeding. The explosion causes fooding around the initial area and also fattens a plant and several buildings in the nearby vicinity tamsulosin 0.4 mg without a prescription androgen hormone effects. You learn that the fre chief and 27 fre- fghters were killed discount 0.2 mg tamsulosin visa prostate biopsy alternatives, which leaves you with a huge shortage of trained frst respond- ers. In addition, you have no operational hospital in your city (Moore Memorial Public Library, 2007). The city manager needs to contact nearby cities with hospitals to manage anyone who has been injured in the fre or the blast. Search and rescue teams need to be formed to look for survivors in the buildings that were destroyed. The city manager will need to set up a makeshift hospital with as many medical volunteers as possible to assist with the wounded until those patients can be taken to a proper medical facility. Stage 8 of the Disaster The explosion killed hundreds of bystanders, pedestrians, and workers, and obliter- ated several buildings. The telephone operators have gone back on the job in this time of crisis and have telephoned a number of nearby agencies and municipalities for assistance. You have just received word that the military, local municipals, and medical centers are all sending personnel and resources to assist your city (Moore Memorial Public Library, 2007). The search and rescue eforts will need to be coordinated so that every survivor is found and treated. Coordinated communication will be essential between the other municipal frst respond- ers, your frst responders, and the military. The city manager will have to monitor how all of the eforts are being coordinated efectively. Medical facilities can easily become overloaded and medical supplies could quickly run out. Stage 9 of the Disaster Your city hall and chamber of commerce are now being used as makeshift medical centers. The military is setting up temporary housing and bulldozing as much of the debris as possible. The ship should be moved if possible and should be doused with a chemical fre retardant if available to the frst responders. What are the other risks that are in the port that should be addressed immedi- ately? You now know that 500 to 600 people have died and thousands more were wounded by the explosions and resulting fres (Moore Memorial Public Library, 2007). The plan of action should be to evacuate any people or ships that are still in the harbor. The city manager should now begin focusing on recovery of the bodies and getting any infrastructure repaired that will assist the frst responders in tackling fres or shoring up any buildings with structural damage. The city manager may want to con- sider bringing in engineers to determine the safety of some of the buildings that were hit with the explosions. In addition, pathologists are going to be needed to identify the bodies and families will need to be notifed. The American Red Cross should be contacted as well as the governor’s ofce to request assistance in cleaning up the disaster area. Key Issues Raised from the Case Study The main point that should be made in this case study concerns the issue of having proper rules and regulations in place to prevent a disaster from occurring. While it may be impossible to stop a fre from breaking out on a single ship, it is possible to limit the damage to that one particular vessel. In the Texas City incident, the disas- ter was compounded by the fact that the initial explosion destroyed or damaged infrastructure, killed or wounded individuals, and caused fres on other vessels that were laden with chemicals. It would behoove administrators to take note that when contending with certain industries, efective rules and regulations should be in place and reviewed on a regular basis. The ships that had volatile chemicals on board should not have been allowed near each other and should have been isolated from the harbor facilities until they were ready to completely load or of-load their cargo. A loading facility should be well away from other harbor facilities since a fre or explosion potentially puts those facilities at risk. Tere were three ships sunk and numerous people were hurt or killed in the ensuing fre and explosions. During the disaster, a city lost an entire corps of highly trained frefghters, which took years to replace. Until those personnel were replaced, the city had to rely more on outside help to battle any blazes that occurred within its municipality. Tere were also the additional costs of repairing any infra- structure that was damaged as well as the costs to salvage the ships to clear the port so that other ships could use the docking areas again. Items of Note The Texas City disaster had over 150 morticians working to take care of the bodies that were recovered as well as dental students being utilized to help with identifca- tion (Moore Memorial Public Library, 2007). Exxon Valdez, Alaska, 1989 Stage 1 of the Disaster You are the head of a state agency on the West Coast of the United States. It is March 24 when an oil tanker, the Exxon Valdez, has run aground at Prince William 134 ◾ Case Studies in Disaster Response and Emergency Management Sound. The accident has caused the oil tanker to release 42 million liters of oil into the Prince William Sound, which harbors several diferent types of animals and aquatic life (Andres, 1997). Second, the Exxon corporation should be ordered immediately to survey the ship and con- tain the oil that has not already leaked out of it. State wildlife employees should be deployed to the disaster area immediately to protect the indigenous wildlife as much as possible. For those animals or birds that have already been covered in oil, wildlife ofcials will need to clean the animals as well as possible. Contact veterinarians and request that they volunteer to assist the wildlife afected by the oil spill. Other state and fed- eral ofcials should be contacted to obtain resources to contain the oil spill as much as possible. Stage 2 of the Disaster The oil is spreading over an 8-mile area around the oil tanker and 1,300 miles of your coastline is now contaminated, and a large amount of wildlife is covered by the oil. The only action that can be taken is to repair the rupture in the ship to prevent more oil from leaking and to try to evacuate wildlife from the afected area. An appeal to volunteers and nonproft groups for help may bring some assistance in the cleanup of the area and the wildlife that is covered in oil. The situation needs to be closely moni- tored to see where the oil slick may be expanding to along the coastline. Federal, state, and local ofcials need to be kept informed on the extent of the disaster, resources that are currently needed, and the progress of the diferent eforts to contain the oil leakage. Key Issues Raised from the Case Study Unfortunately, there is very little that local administrators can do in a situation where it appears that industry standards for safety were less than ideal. However, administrators can mitigate foreseeable ecological disasters by working closely with government agencies that have jurisdiction over shipping and conduct spot checks on the sobriety of the crew on ships that are carrying potentially dangerous cargo.
Isotonic saline promptly corrects intravascular volume depletion and helps in restoring eukalemia purchase cheap tamsulosin line prostate oncology specialists nj. Once the child is hemodynamically stable and starts accepting oral feed order discount tamsulosin line prostate problems treatment, therapy with oral hydrocortisone (10–15 mg/m2 in divided doses) and fludrocortisone (100 μg twice daily) can be initiated along with oral salt supplementation (4–8 mmol/Kg) buy tamsulosin 0.2 mg cheap man health and environment. Newborns require higher doses of fludrocortisone as they are aldosterone resistant. Urogenital sinus differentiation is complete by 12th week of intrauterine life; hence, the androgen exposure prior to 12 weeks results in labioscrotal fusion along with clitoro- megaly, while exposure after 12 weeks results in isolated clitoromegaly. In addition, the severity of androgen excess and sensitivity to androgens also determine the extent of virilization (Fig. Posterior labial fusion is objectively assessed by the measurement of anogenital ratio, which is calculated by the distance between the anus and posterior four- chette divided by distance between the anus and base of phallus. In a developing embryo, the primitive Wolfﬁan and Mullerian ducts are attached to the cloaca. This is followed by differentiation of the cloaca into the urogenital sinus anteriorly (along with Wolfﬁan and Mullerian ducts) and rectum posteriorly. In females, regression of Wolfﬁan ducts occurs between 8 and 12 weeks; and the urogenital sinus starts differentiating into the lower part of the urinary bladder, urethra, and lower one-third of the vagina by 8–9 weeks; and two distinct openings, urethral and vaginal opening, are appreciable at perineum by 16–17 weeks. In males, regression of Mullerian ducts occurs between 7 and 11 weeks, and the urogenital sinus develops into the lower part of the urinary bladder, urethra, and prostate by 8–12 weeks (Fig. In the absence of androgens, the urogenital sinus differentiates into urethra and lower one-third of the vagina during organogenesis; however, on exposure to circulating androgens, the urogenital sinus differentiates into prostate and ure- thra. In a female embryo, the urogenital sinus differentiates into urethra and vagina with two distinct openings at the perineum. However, depending on the level and sensitivity to androgens, the site of Mullerian duct opening into the urogenital sinus can be located near the peri- neal surface (low vaginal conﬂuence) or away from it (high vaginal conﬂuence) (Fig. The differentiation between low- and high vaginal conﬂuence can be made by performing either genitoscopy or urogenitogram. This is important in determin- ing the nature and timing of genital reconstructive surgery. Children with low 10 Congenital Adrenal Hyperplasia 349 vaginal conﬂuence should undergo vaginoplasty and perineal reconstruction (with or without clitoroplasty) at an early age. The surgical reconstruction of high vaginal conﬂuence is technically more challenging, and the optimal timing for surgery in children with high vaginal conﬂuence is not deﬁned. Differentiation of external genitalia to male phenotype depends on the exposure to circulating androgens (predominantly dihydrotestosterone), whereas differentia- tion of Wolfﬁan structures is mediated by the paracrine action of androgens (pre- dominantly testosterone) from the testes. Post-natal exposure to androgens in a girl child will result in clitoral enlarge- ment, pubarche, acne, and deepening of voice. However, as the urogenital sinus has already completed differentiation, androgen exposure in the post-natal period does not result in genital virilization beyond Prader stage 1. This results in virilization of external genitalia in newborn girls and penile enlargement in newborn boys. This is because priming of pilosebaceous unit (and hence pubarche) require prolonged and persistent exposure to androgens as compared to virilization of the urogenital sinus which require only short-term exposure. Further, estimation of plasma renin activity also helps in moni- toring of a child on therapy. The greatest ben- eﬁt of neonatal screening is prevention of salt-wasting crises, especially in a male child who may otherwise be missed due to lack of genital ambiguity. In addition, neonatal screening helps in early and appro- priate gender assignment and allows timely initiation of therapy to prevent pro- gression of virilization and morbidity associated with surgical intervention. Therefore, cortisol levels in fetus dur- ing mid-gestation are approximately ten times lower than that found in maternal serum. This is accomplished by admin- istering dexamethasone to the mother as soon as the pregnancy is conﬁrmed. Dexamethasone inhibits fetal hypothalamo–pituitary–adrenocortical axis and thereby suppresses fetal adrenal androgen production and consequently prevents fetal virilization. Treatment with dexamethasone should be initiated as soon as pregnancy is con- ﬁrmed as exposure of female embryo to adrenal androgens between 7 and 12 weeks of intrauterine life results in virilization of the urogenital sinus and consequent genital ambiguity. Dexamethasone is administered at a dose of 20 μg/ Kg/day in divided doses with a maximum dose of 1. After genetic analysis by chori- onic villous sampling at 10–12 weeks, the need for further continuation of dexamethasone can be reconsidered. In a male fetus and unaffected female fetus, it is discontinued, while in an affected female fetus, dexamethasone is continued. Hence, to prevent genital ambiguity in one female child, seven children need to be treated unnecessarily. In addition, prenatal dexamethasone therapy is associated with adverse fetal and maternal outcomes. Prenatal therapy results in exposure of fetus to very high levels of glucocorticoids (approximately 60 times greater than levels in a normal fetus). The fetal risks associated with dexamethasone therapy include intrauterine growth retardation, orofacial congenital malforma- tions, impaired verbal working memory in childhood, and, possibly, future risk of cardiovascular events. The maternal risks include weight gain, edema, and, possibly, higher incidence of hypertension and hyperglycemia. In addition, oral salt (1–2 g/day) should also be administered during early infancy. High-dose glucocorticoids should be administered in these children during periods of stress like infection, trauma, or surgery. Genital reconstructive surgery should be considered at an appropriate age based on the severity of ambiguity. Hydrocortisone is preferred over other glucocorticoids like prednisolone and dexamethasone as it is physiological, has short duration of action, and exerts minimal detrimental effects on epiphyseal growth plate. It has been shown that at equivalent doses (in terms of glucocorticoid activity), prednisolone has 10–15-fold higher and dexamethasone 70–80-fold higher growth inhibitory effects, as compared to hydrocortisone. However, if the dose of hydrocortisone exceeds 20 mg/m2/day in infants and 15–17 mg/m2/day in adolescents, it may also exert growth-suppressive effects. Subclinical aldosterone deﬁciency results in chronic deple- tion of sodium and extracellular ﬂuid volume. Hence, therapy with ﬂudrocortisone results in lowering of glucocorticoid doses and improves growth potential (Fig. Growth velocity is a sensitive parameter for assessment of adequacy of therapy as decreased growth velocity suggests overtreatment, while increased growth velocity suggests undertreatment. Sample for biochemical evaluation should be taken in the morning between 0800 and 0900h without discontinuing the morning dose of hydrocortisone. Androstenedione and testoster- one should be maintained in age- and gender-speciﬁc normal range. However, serum testosterone has limited utility in men as it is predominantly produced from the testes. In addition, testicular ultrasonography, measurement of gonadotropins, and semen analysis in men and serum progesterone in women should be performed while planning fertility. In a developing embryo, adrenogonadal primordium differentiates into adrenal and gonadal tissue.