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Indications for catheter intervention include symptoms of fatigue and exercise intolerance buy super levitra 80mg cheap, symptoms which often are experienced with increased age buy cheap super levitra 80 mg on-line, even with stable stenosis buy super levitra 80 mg line. Severe pulmonary stenosis can be successfully treated by catheter-based balloon angioplasty. Surgical valvotomy is reserved for patients in whom balloon valvulo- plasty has been unsuccessful or for patients in whom multiple levels of obstruction are demonstrated. Critical pulmonary stenosis requires prompt initiation of prostaglandin infusion to maintain ductal patency and provide pulmonary blood flow. Following complete echocardiographic assessment, most neonates proceed to the cardiac catheterization laboratory for balloon valvuloplasty, after which the prostaglandin infusion is dis- continued. Occasionally, infundibular stenosis becomes apparent following balloon valvuloplasty, and a surgical Gore-tex shunt is required to maintain pulmonary blood flow. Though pulmonary valve patency has been established, many neonates continue to demonstrate moderate cyanosis, with SpO2 of 70–80%, which improves slowly over several months as the right ventricular compliance improves and decreases the degree of right to left atrial level shunt. An infant with a history of critical or severe pulmonary stenosis and pulmonary valvuloplasty requires pulse oximetry assessment at each visit. In the rare instance of isolated infundibular stenosis, patch widening of the right ventricular outflow tract and resection of the infundibular muscle are required. Treatment for supravalvular and branch pulmonary artery stenosis includes fre- quent medical observation. Catheter intervention is indicated following the onset and/or progression of symptoms. Surgical pericardial or prosthetic patch augmenta- tion is indicated for severe stenosis not amenable to catheter-based interventions. Treatment options for patients with diffuse peripheral pulmonary arterial obstruction syndromes (Noonan, Williams, Alagille, and Rubella) are limited and outcome is generally poor, particularly because lesions tend to be progressive. However, most patients undergo serial balloon angioplasty catheter interventions with the hope of modifying disease progression. Since the obstructions are fixed, pulmonary vasodilators such as nitric oxide, sildenafil, epoprostenol, or bosentan are ineffective. Patients with diffuse arteriopathy are at increased risk for sudden death with procedural sedation and anesthesia, and should therefore be referred for cardiology evaluation before any procedures or surgeries. In accordance with the most recent recommendations by the American Heart Association, subacute bacterial endocarditis prophylaxis is no longer indicated for 10 Pulmonary Stenosis 141 isolated pulmonary stenosis. If pulmonary stenosis is associated with a right-to-left atrial shunt, or if associated with surgical or transcatheter prosthetic material, then subacute bacterial endocarditis prophylaxis should be provided as long as there is a residual lesion. Case Scenarios Case 1A A 1-day-old infant born at 40 2/7 weeks’ gestation develops cyanosis without respiratory distress at 24 h of life. On examination, she is awake, cyanotic, and tachypneic with a respiratory rate in the 60’s. On auscultation, lung sounds are clear and heart tones are normal, without a click or a distinct P2 component. Discussion This history is typical of an infant with ductal-dependent pulmonary blood flow. The infant requires prompt initiation of prostaglandin infusion to maintain ductal patency. Oxygen administration does not improve the saturation because blood delivery to the lungs is compromised in the setting of obstructed pulmonary outflow and a closing ductus arteriosus. A chest radiograph, electrocardiogram, and echocardiogram can be performed to establish the diagnosis of critical pulmonary stenosis, following initia- tion of prostaglandin infusion. The differential diagnosis includes a variety of con- genital heart lesions which include severe or critical pulmonary stenosis such as tetralogy of Fallow with severe pulmonary stenosis. On the other hand, lesions with tricuspid or pulmonary atresia are unlikely to present in this fashion since these are ductal-dependent lesions, which would provide increase in pulmonary blood flow and restriction or closure of the ductus arteriosus would result in severe and life-threatening deterioration due to acute drop in blood flow to the lungs. Chest X-ray: In this infant, the cardiac silhouette is normal, without evidence of cardiac enlargement (Fig. Though many infants with critical pulmonary stenosis have right atrial enlargement and cardiomegaly on chest radiograph, the diagnosis can still be suggested in infants without cardiomegaly by noting the dark lung fields which occur as a result of reduced pulmonary blood flow. Echocardiography: An echocardiogram confirms the diagnosis of critical pul- monary stenosis with a patent ductus arteriosus supplying pulmonary blood flow to good-sized branch pulmonary arteries. The pulmonary vasculature is reduced suggestive of reduced pulmonary blood flow with no demonstrable flow across the valve. The right ventricle is hypertrophied with a small chamber size, and it contracts poorly. The interventricular septum bows into the left ventricle, suggesting the right ventricular pressure is greater than the left. Cardiac catheterization: The infant is taken to the cardiac catheterization labo- ratory, where a catheter is advanced from the right femoral vein to the right atrium and then manipulated into the right ventricle. The measured right ventricular sys- tolic pressure is 123 mmHg, compared with a systolic blood pressure of 74 mmHg. An angiogram is performed, which demonstrates a tiny “blow-hole” in the pulmo- nary valve, thereby distinguishing pulmonary valve stenosis from atresia. A guidewire is advanced from the femoral vein to the right atrium, and then manipulated across the tricuspid valve and the pulmonary valve, to the ductus arteriosus and down the descending aorta. The balloon is tracked over the guidewire and positioned across the pulmonary valve. A guidewire is advanced from the femoral vein to the right atrium, and then manipulated across the tricuspid valve and the pulmonary valve, to the ductus arteriosus and down the descending aorta. The balloon is tracked over the guidewire and positioned across the pulmonary valve. Note that as the balloon is inflated (a), the “waist” of the balloon disappears (white arrows) as it opens the valve and relieves the stenosis (b) Pulse oximetry at the start of the procedure was 80% in room air, with continuous prostaglandin infusion. The right ventricular systolic pressure is now down to 45 mmHg, compared with a systolic blood pressure of 68 mmHg. Since the last visit at 1 month of age, the infant has been feeding and acting normally. The precordium is hyperdynamic, and a thrill is pal- pable at the left upper sternal border. An audible click is present at the left upper sternal border, along with a 4/6 harsh ejection-quality (crescendo–decrescendo) mur- mur which radiates to the back and bilateral axillae. Discussion The pulmonary stenosis in this infant has progressed following the initial valvulo- plasty, and requires repeat valvuloplasty. Though valvular pulmonary stenosis usually improves with time, infants with critical pulmonary stenosis may experience initially progressive disease and require reintervention. Case 2 A 15-year-old girl with Williams syndrome has relocated from another city and presents for a required routine examination prior to enrollment at her new school. Her medical history is significant for a cardiology evaluation at the time of her genetic diagnosis as an infant, which was normal.

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Presentation and Diagnosis In addition to the typical signs and symptoms of pancreatitis cheap super levitra 80mg otc, such as moderate epigastric pain radiating to the back buy super levitra 80mg on line, vomiting order discount super levitra on line, tachycardia, fever, leukocytosis, and elevated amylase and lipase, patients with severe acute pancreatitis present with relatively greater abdominal tenderness, distension, and even symptoms of accompanying multiorgan failure (38). In these patients, the intensivist must maintain a high level of clinical suspicion for necrosis and possibly infection as well. Infection is estimated to develop in 30% to 70% of patients with necrotic pancreatitis (40). However, necrosis both with and without infection often manifest with similar clinical presentations because necrosis alone causes a systemic inflammatory response, and additional diagnostic data is generally needed to differentiate these (41). Enterococcus species are the organisms most frequently isolated, although many different pathogens including Candida spp. Treatment and Prophylaxis The distinction between sterile and infected necrotic pancreatitis is crucial, as the former may be handled medically when necrosis affects less than 30% of the organ, whereas the latter often demands surgical debridement (38). Recently, several studies have explored the potential of laparoscopy for infectious pancreatic necrosis, but this approach is rarely feasible in instances of extensive necrosis, and data is not yet sufficient to compare the safety and efficacy of 268 Wilson laparoscopic surgery versus laparotomy for this indication (43). Percutaneous drainage has a low success rate of just 32% and is generally insufficient management except in the case of a well-defined abscess, or one remote from the pancreas (41). Abdominal compartment syndrome has been noted in severe acute pancreatitis and decompression has been suggested for patients whose transvesical intra-abdominal pressure reaches 10 to 12 mm Hg (43). An appropriate antibiotic regimen for infected pancreatic necrosis is the second arm of a successful treatment plan: given the wide range of possible offending organisms, a Gram stain is recommended to tailor specific initial therapies prior to culture results. For gram-negative organisms, a single-agent carbapenem is effective; for gram-positives b-lactamase–resistant drugs, vancomycin, and even linezolid must considered. When yeast is identified, high-dose fluconazole or caspofungin should be sufficient. In any case, if infection develops despite antibiotic prophylaxis, a different class of drugs must be administered for treatment than was given for prophylaxis (44). Although current literature does not specifically favor any specific antibiotic as prophylaxis, it is nonetheless clear that microbial coverage must be broadly targeted. One- to two-week courses of cefuroxime, imipenem with cilastin, and ofloxacin with metronidazole have each been tried with success (42). An exhaustive list of these is beyond the scope of this chapter; however, the reader should be aware of the general possibilities. Fever, for instance, in the postoperative patient, is not always secondary to infection. Particularly relevant to the postsurgical patient are events such as atelectasis, myocardial infarction, stroke, hematoma formation, and even pulmonary embolism that may occasionally present with a fever component. Other causes that warrant deliberation include drug or transfusion reaction, malignancy, collagen vascular disease, endocrine causes such as hyperthyroidism, and less common etiologies such as disordered heat homeostasis secondary to an ischemic hypothalamic injury or even familial malignant hyperthermia. Furthermore, it is important to interpret radiological findings with an open mind. Again, high on the differential that must be considered is hematoma, and one may explore other diagnoses given the individual patient history. A myocardial infarction involving the inferior wall of the heart and lower lobe pneumonias, for instance, may present with abdominal pain and fever despite extra-abdominal origins. Approximately 40% of all organisms isolated by DeWaele and colleagues at Ghent University hospital were multidrug resistant. For example, a patient’s status post-aneurysm repair has the same likelihood of developing appendicitis as any member of the general population in the same age group. Therefore, the conscientious physician considers all possibilities appropriate for the patient’s complete history—not surgical history only—when constructing a thorough differential. Longitudinal outcomes of intra-abdominal infection complicated by critical illness. Daily organ-system failure for diagnosis of persistent intra-abdominal sepsis after postoperative peritonitis. Abdominal abscesses in patients having surgery: an application of Ga-67 scintigraphic and computed tomographic scanning. Postoperative enterococcal infection after treatment of complicated intra-abdominal sepsis. Determinants for successful percutaneous image-guided drainage of intra-abdominal abscess. Percutaneous postoperative intra-abdominal abscess drainage after elective colorectal surgery. Open management of the abdomen and planned reoperations in severe bacterial peritonitis. Planned reoperations and open management in critical intra-abdominal infections: prospective experience in 52 cases. Clostridium difficile-associated diarrhea: risk factors, diagnostic methods, and treatment. Ultrasound is not a useful screening tool for acute acalculous cholecystitis in critically ill trauma patients. Diagnosis, treatment and prophylaxis of spontaneous bacterial peritonitis: a consensus document. Review article: spontaneous bacterial peritonitis—diagnosis, treatment and prevention. Primary pneumococcal peritonitis in patients with cardiac ascites: report of 2 cases. Spontaneous bacterial peritonitis by campylobacter fetus in Budd- Chiari syndrome without liver cirrhosis. Abdominal compartment syndrome in patients with severe acute pancreatitis in early stage. Intraabdominal sepsis: newer interventional and antimicrobial therapies for infected necrotizing pancreatitis. Antibiotic therapy for prophylaxis against infection of pancreatic necrosis in acute pacreatitis. Accurate diagnosis of infarction of omentum and appendices epiploicae by computed tomography. Blood stream infections of abdominal origin in the intensive care unit: characteristics and determinants of death. Hjalmarson Division of Geographic Medicine and Infectious Diseases, Department of Medicine, Tufts Medical Center, Boston, Massachusetts, U. Gorbach Nutrition/Infection Unit, Department of Public Health and Family Medicine, Tufts University School of Medicine, and Division of Geographic Medicine and Infectious Diseases, Department of Medicine, Tufts Medical Center, Boston, Massachusetts, U. Staphylococcus aureus was the suspected pathogen since it was frequently recovered from patients stool culture samples. With increased use of cephalosporins in the 1980 to 2000, it became the antibiotic class most commonly associated with C.

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The frequency and severity of androgenetic alopecia is lower in women than in men but it still affects a sizeable proportion of the population cheap 80 mg super levitra mastercard. Two studies in Caucasian women in the United Kingdom and the United States reported prevalence rates of 3–6% in women aged under 30 order 80 mg super levitra with mastercard, increasing to 29–42% in women aged 70 and over (41 purchase super levitra 80 mg without a prescription,42). As in men, androgenetic alopecia is less common and appears to start later in life in Asian women, although nearly 25% of Korean women over 70 years of age show evidence of hair loss (39). There are no published data on the prevalence of androgenetic alopecia in African women although clinical experience suggests that its frequency is similar to that in other racial groups. Under normal circumstances it has no adverse effect on physical well-being apart from increasing the risk of chronic photodamage to unpro- tected scalp skin. Under exceptional conditions a full head of hair may also contribute to ther- moregulation. The French military surgeon Dominique-Jean Larrey observed that the bald men (and men without hats) were the first to die during the Russian campaign in the winter of 1812. Yet balding still has a powerful effect on the human psyche, to the extent that few men would choose to go bald were the choice available. However, for some men balding is important enough for them to seek treatment and, for a few, concern about hair loss reaches the level of a body dysmorphic disorder. Men in the latter group are important to recognize as treatment aimed at addressing the perceived hair problem is unlikely to be successful. A number of studies have shown that male balding has an adverse effect on quality of life (though this is almost inevitable in those seeking professional advice) (43). Nevertheless, balding is often seen as a trivial issue (mainly by non-sufferers) which may make men reluctant to approach their physician as they perceive, rightly or wrongly, that they will not receive a sympathetic response. In contrast to the pre- vailing attitude to male balding, however, society generally regards it as abnormal for women to lose their hair. Consequently the adverse effect of balding on quality of life tends be more severe in women than in men. As a group, women seeking medical advice for hair loss experi- ence more negative body-image feelings, more social anxiety, poorer self-esteem, and psycho- social well-being than control subjects with nonvisible skin disease, as well as dissatisfaction with their hair. In quality-of-life studies, individual responses were more related to self-percep- tion of hair loss than to objective or clinical ratings and those women most distressed by hair loss were more poorly adjusted and had a greater investment in their appearance (44,45). The physician needs to be alert and sensitive to these issues and needs counseling and psychothera- peutic skills that go beyond merely prescribing treatment. Diagnosis The diagnosis of androgenetic alopecia in men rarely causes difficulties. In cases presenting with general thinning, other causes of diffuse hair loss should be considered, particularly when the hair loss progresses quickly. This situation is perhaps most likely to be seen in teenage boys brought along by worried parents. The diagnosis of female androgenetic alopecia may be more challenging although it can usually be made on clinical grounds. Rapidly progressive hair loss with a strongly positive “tug test” should raise the possibility of diffuse alopecia areata. Loss of body hair, eyebrows, or eyelashes, and nail changes will support the diagnosis but it is sometimes necessary to obtain histology. Other causes of diffuse hair loss include systemic lupus erythematosus and thyroid disease and the relevant investigations should be performed where indicated by the overall clinical picture. Occasionally, scarring alopecia presents in a diffuse pattern and here a biopsy will usually be necessary. The most common clinical dilemma is the diagnosis of women pre- senting with chronic excessive hair shedding in whom hair density appears normal, often referred to as chronic telogen effluvium. If known causes of telogen effluvium are excluded, about 60% of these women show histological evidence of early androgenetic alopecia on biopsy (46). The cause of increased hair shedding in the remaining 40% is usually obscure, although it may simply be due to age-related shortening of the hair cycle. It should also be remembered that androgenetic alopecia may be a sign of hyperandrogen- ism. Some hyperandrogenic women show a male or, more com- monly, a partially male pattern of hair loss but, in the author’s experience, the pattern of hair loss is not a reliable indicator of androgen status. Investigations are unnecessary in women with typical androgenetic alopecia and no other evidence of androgen excess, although most authorities recommend checking a full blood count, serum ferritin and thyroid function. In the author’s experience, abnormalities of thyroid func- tion are not particularly common in women presenting with diffuse hair loss, possibly no more Androgenetic Alopecia 111 common than in the population at large, but formal data are lacking. Whether, and how far, to investigate women with evidence of hyperandrogenism depends on a variety of factors, such as the time course of the complaint, age of the patient, and the presence of associated features. The most important cause, albeit rare, is an androgen-secreting ovarian or adrenal tumor. A serum testosterone is a useful screening test; a level in excess of 5nmol/l should prompt further endocrinologic investigation. Counseling Those seeking medical advice for androgenetic alopecia, both male and female, fall roughly into three groups (which may overlap): (i) those who wish to ensure their hair loss is not a manifes- tation of an underlying serious disease, (ii) those who wish to be treated, and (iii) those with a body image problem. For all groups counseling should include an explanation of the nature of the condition and its natural history. For those interested in preventing further progression or improving their hair status, the treatment options, together with a realistic explanation of what can be achieved by treatment, will also need to be discussed. For those with a body dysmorphic disorder involvement of a clinical psychologist or psychiatrist should be explored. Hair loss is an emotional issue and sufferers are vulnerable to exploitation by the unscrupulous. Patients should be advised against parting with large sums of money on unproven and valueless remedies. Treatment Men At present only two medical treatments, minoxidil and finasteride are licensed for the treat- ment of male balding. Both drugs will stimulate some regrowth of hair in some men but are perhaps better regarded as preventative treatments. Neither will regrow hair on completely bald scalp and continued treatment is necessary to maintain the response. Both drugs have a good safety record, a consideration of paramount importance when treating hair-growth disor- ders. Minoxidil Minoxidil was licensed as an oral drug to treat hypertension in the early 1970s. It soon became apparent that a high proportion of those taking minoxidil tablets developed significant hypertri- chosis, a side effect that has almost eliminated its use as an anti-hypertensive agent. Following a report of increased hair growth on the scalp of a balding man taking minoxidil tablets (47), extem- poraneous formulations of minoxidil solution were developed for topical application in the treat- ment of hair loss (initial reports concentrated mainly on alopecia areata). Food and Drug Administration for the treatment of male balding and marketed by the Upjohn Company in 1986.

Without close monitoring of the diet however buy genuine super levitra, extremely low blood sugar can be fatal buy super levitra 80mg amex. Long- term complications can include kidney damage discount super levitra 80mg visa, brittle bones (osteoporosis), benign cysts on the ovaries (in women), and benign tumors of the liver (adenomas). Detection Population Rate* 45% African American 45% Ashkenazi Jewish 45% Eastern Asia 45% Finland 45% French Canadian or Cajun 45% Hispanic 45% Middle East 45% Native American 45% Northwestern Europe 45% Oceania 45% South Asia 45% Southeast Asia 45% Southern Europe * Detection rates shown are for genotyping. The lack of this enzyme means the body cannot properly break down glycogen, a stored form of sugar. As a result, glycogen cannot properly be used to energize the body and glycogen molecules accumulate in the body. This enlargement usually subsides with puberty, although there may be long term liver damage. Children with the disease may experience delayed growth, but usually reach normal adult height. A minority of people with the disease also have a mildly enlarged heart, though its function is usually normal. The onset of the disease may occur in adulthood, which typically corresponds with milder symptoms. This disease is much more common in Israeli Jews of North African descent, where 1 in 35 are carriers and 1 in 5,400 babies has the disease. The highest rate is found among people on the Faroe Islands of the North Atlantic, where 1 in 30 is a carrier and 1 in 3,600 babies is afected. Physicians will monitor the liver, heart, and muscles in afected people and recommend physical therapy when necessary to promote better movement. Physicians may recommend consuming corn starch, which breaks down slowly into simple sugars and may alleviate symptoms of low blood sugar between meals. Parents of infants should be particularly careful to monitor the child’s diet to avoid hypoglycemic seizures. The Counsyl Family Prep Screen - Disease Reference Book Page 122 of 287 Glycogen Storage Disease Type V Available Methodologies: targeted genotyping and sequencing. Detection Population Rate* <10% African American 80% Ashkenazi Jewish 64% Eastern Asia 80% Finland 80% French Canadian or Cajun <10% Hispanic <10% Middle East <10% Native American 80% Northwestern Europe <10% Oceania <10% South Asia <10% Southeast Asia 80% Southern Europe * Detection rates shown are for genotyping. This defciency prevents an afected person from turning glycogen, a stored form of sugar, into glucose, which can be used for energy. The severity of the symptoms can vary, but in many people symptoms do not get signifcantly worse over time. About a third of The Counsyl Family Prep Screen - Disease Reference Book Page 123 of 287 adults with the disease experience progressive muscle weakening later in life. One study showed the disease incidence to be 1 in 100,000 in the Dallas-Fort Worth area of Texas in the United States. Too much physical exertion will result in muscle breakdown and severe kidney problems. Detection Population Rate* <10% African American <10% Ashkenazi Jewish <10% Eastern Asia >99% Finland <10% French Canadian or Cajun <10% Hispanic <10% Middle East <10% Native American <10% Northwestern Europe <10% Oceania <10% South Asia <10% Southeast Asia <10% Southern Europe * Detection rates shown are for genotyping. Iron overload The body does not metabolize iron properly, leading to excess levels, particularly in the liver. Lactic acidosis Lactic acid builds up in the body, causing the blood to become too acidic. Here, researchers estimate that 1 in 47,000 babies are afected, meaning that roughly 1 in 110 Finnish people are carriers of the genetic mutation. There have been reports of several infants in the United Kingdom and Turkey with similar but not identical symptoms. Experimental treatments to reduce acidity and/or iron levels in the infants’ blood have not been shown to extend life beyond several months. The Counsyl Family Prep Screen - Disease Reference Book Page 126 of 287 Hb Beta Chain-Related Hemoglobinopathy Including Beta Thalassemia and Sickle Cell Disease Available Methodologies: targeted genotyping and sequencing. Detection Population Rate* 78% African American 83% Ashkenazi Jewish 86% Eastern Asia 83% Finland 83% French Canadian or Cajun <10% Hispanic 72% Middle East <10% Native American 83% Northwestern Europe <10% Oceania 86% South Asia 86% Southeast Asia 93% Southern Europe * Detection rates shown are for genotyping. Hb beta chain-related hemoglobinopathies are a group of inherited blood disorders that afect hemoglobin, the major component of red blood cells which carries oxygen throughout the body. People with Hb beta chain-related thalassemia do not produce enough beta protein—and in some cases do not produce it at all—resulting in a shortage of red blood cells (anemia). Without sufcient numbers of properly functioning red blood cells, the organs of the body do not receive enough oxygen. There are The Counsyl Family Prep Screen - Disease Reference Book Page 127 of 287 three main types of beta thalassemia. The lack of oxygen can cause him or her to be pale, listless, tired, and irritable. Without frequent blood transfusions, the condition can be life-threatening at an early age. Beta thalassemia intermedia, a less severe form of the condition, causes mild to moderate anemia and a wide spectrum of possible health problems. The types of symptoms are the same as with thalassemia major, including bone deformities and an enlarged spleen, though these are typically not as severe. People with thalassemia intermedia require fewer blood transfusions and use them to improve the quality of their lives. Sickle cell disease is a type of hemoglobinopathy caused by two Hb S mutations, or one copy of the Hb S mutation along with a beta thalassemia mutation. The sickled blood cells die prematurely, causing a person to feel weak and tired, a condition known as anemia. People with sickle cell anemia develop symptoms including anemia, repeated infections, shortness of breath, fatigue, jaundice, and bone pain starting in early childhood. These sickled cells also get stuck in small blood vessels, blocking blood fow and causing serious medical complications such as blood-starved organs or tissue deterioration. The most recognizable symptom is episodes of acute back, chest, or abdominal pain called "crises. Interactions between beta globin proteins and these mutations can alleviate or exacerbate the efects of the individual variants. Thalassemias are most common in people of Mediterranean descent, especially in those from Sardinia and Cyprus. In Cyprus, 1 in 7 people are carriers of beta thalassemia, a rate which prompted a successful government-run disease prevention program. The Counsyl Family Prep Screen - Disease Reference Book Page 128 of 287 Sickle cell disease is common in people from Africa, the Mediterranean, the Arabian Peninsula, India, South America, and Central America. In the African American population, approximately 1/10 people are carriers of sickle cell. Ethnic Group Carrier Rate Afected Rate Cypriot 1 in 7 1 in 170 Sardinian 1 in 8 1 in 240 Italian 1 in 31 1 in 3,700 Middle Eastern 1 in 34 1 in 4,500 Southeast Asian 1 in 35 1 in 4,800 East Asian 1 in 62 1 in 15,000 Indian 1 in 64 1 in 16,000 How is Hb Beta Chain-Related Hemoglobinopathy treated?

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