Frequency of infective endocarditis among infants and children with Staphylococcus aureus bacteremia order accutane 5 mg with mastercard acne en la espalda. Comprehensive diagnostic strategy for bold cultures-negative endocarditis: a prospective study of 819 new cases purchase 5 mg accutane acne x ray. Two-dimensional echocardiographic assessment of infective endocarditis in children order accutane with american express acne 17 year old male. Role of echocardiography in evaluation of patients with Staphylococcus aureus bacteremia: experience in 103 patients. Penetration of the atrioventricular septum by spread of infection from aortic valve endocarditis: early diagnosis by transesophageal echocardiography and implications for surgical management. Infective endocarditis in children: clinical analysis and evaluation of two diagnostic criteria. Are the Duke criteria superior to the Beth Israel Criteria for the diagnosis of infective endocarditis in children? Value and limitations of the von Reyn, Duke, and modified Duke criteria for the diagnosis of infective endocarditis in children. Clinical Practice Guidelines by the Infectious Diseases Society of America for the treatment of methicillin resistant Staphylococcus aureus infections in adults and children. Guidelines from the American Heart Association: a guideline from the American Heart Association Rheumatic Fever, Endocarditis, and Kawasaki Disease Committee, Council on Cardiovascular Disease in the Young, and the Council on Clinical Cardiology, Council on Cardiovascular Surgery and Anesthesia, and the Quality of Care and Outcomes Research Interdisciplinary Working Group. Temporal trend in infective endocarditis in the context of prophylaxis guideline modifications: three successive population-based surveys. Incidence of infective endocarditis in England, 2000–13: a secular trend, interrupted time-series analysis. Although chest pain is common in patients presenting to pediatric cardiology clinics, myocardial ischemia is rarely ever the etiology. The list of etiologies leading to myocardial ischemia is potentially long and each diagnosis for the most part is uncommon. Unfortunately, many patients who suffer an ischemic episode do not present until after myocardial injury or infarction has already happened. The patient who presents with symptoms of chest pain before an event or in the middle of an event will be the focus of this particular chapter. A detailed history, clinical examination, and appropriate diagnostic testing should lead to the correct diagnosis and offer the ability to include or exclude myocardial ischemia from the differential diagnosis. For the purposes of this chapter we will not review atherosclerotic coronary artery disease, it is covered in Chapter 71. Definition Myocardial ischemia is an imbalance between myocardial oxygen supply and demand. Left untreated, it results in angina pectoris, myocardial stunning, myocardial hibernation, or under the most severe instances, acute coronary syndromes like myocardial infarctions. Myocardial ischemia can be caused by several mechanisms, including increased myocardial oxygen demand in the presence of a severe fixed stenosis, coronary spasm due to local release of vasoactive mediators, and transient thrombus formation. The determinants of myocardial ischemia are likely to differ in patients with unstable coronary syndromes as the underlying pathologic substrate usually consists of plaque rupture with a varying degree of intracoronary thrombus formation (1,3,4). In normal conditions, an uninterrupted flow of large quantities of oxygenated blood to the myocardium is critical to its normal function. During systole, this flow can be abolished or even reversed toward the epicardial vessels. The blood must flow from low-to-high intramyocardial pressure in order to meet the metabolic demands of each layer. Flow must be regulated in such a way that areas of high demand can immediately increase their blood supply. The myocardium extracts about 60% to 75% of oxygen from the blood that passes through it. Because of this high level of extraction, coronary sinus blood has low oxygen tension, generally around 25 to 35 mm Hg. This low level of oxygen tension requires that any increase in oxygen demand be met by an increase in blood flow rather than an increase in extraction (5,6). There are two main mechanisms by which myocardial ischemia can occur: (a) a reduction in myocardial supply of oxygen, and (b) an increase in myocardial oxygen demand. The first situation can occur as a result of reduced coronary blood flow or reduced oxygen content despite normal coronary flow. Reduced coronary blood flow can result from congenital malformations of the coronary arteries, acquired coronary diseases, and also postoperative states, especially after surgical reimplantation of the coronary arteries. Examples of reduced oxygen content in coronary blood include cyanotic heart diseases, severe anemia, and hemoglobinopathies (4,7). On the demand side, an increase in myocardial oxygen demand, that is, exercise, may lead to ischemia if there is a limitation on supply that under normal or steady state conditions is adequate but not sufficient during times of increased demand. Although this mechanism of ischemia may lead to an acute coronary syndrome, more commonly, patients suffer chronic chest pain during times of increased demand. The second mechanism can also occur in the presence of hypertrophic cardiomyopathy or vigorous exercises (8). History For the patient who presents with chest pain, a detailed history provides the most important information to include or exclude myocardial ischemia from the differential diagnosis. Myocardial ischemia causing chest pain has typical characteristics that when present requires further investigation. Chest pain is more likely ischemic in nature when it is associated with: Exertion more than at rest Dyspnea Diaphoresis Syncope and characterized as: Substernal pressure or burning rather than pain Pressure that radiates to neck or arm Fairly reproducible with similar activity Short lived 2 to 10 minutes as opposed to hours Other important historical features include how the pain is relieved. If a patient is able to continue to run and play and the pain goes away despite continuing activity, myocardial ischemia is less likely. A family history should screen for Marfan syndrome, other aortopathies, and for hypertrophic cardiomyopathy. A prior surgical history where the coronary arteries were manipulated or reimplanted is important in a patient with typical chest pain or ischemia. The surgical reports pertaining to the procedure and how the coronary arteries were either reimplanted or manipulated may be important to the current care of the patient and future testing that must be done. Differential Diagnosis The etiologies for nonatherosclerotic coronary artery disease and myocardial ischemia remain rare and for the most part are difficult to diagnose, require a high index of suspicion, and often involve advanced imaging studies. Most of the diagnoses are covered in other areas of this textbook and are detailed in those chapters. This group of diagnoses can be divided into those involving the coronary arteries directly and those that involve the myocardium leading to myocardial ischemia. They involve congenital anomalies of the coronary arteries and hypertrophic cardiomyopathy. Other diseases can present early in life with congestive heart failure, circulatory shock, or severe hypoxemia. All these factors can compromise coronary circulation and lead to myocardial ischemia. Congenital Anomalies of the Coronary Arteries The entire blood flow to the myocardium comes from two main coronary arteries that arise from the right and left aortic sinuses of Valsalva.

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One of the most sensitive discount accutane 40mg online skin care korea terbaik, but not solely cheap 30 mg accutane fast delivery acne boots, diagnostic features of Ebstein anomaly is the displacement of the septal leaflet hinge point cheap 20mg accutane with amex acne 5 weeks pregnant. In a normal heart the tricuspid septal leaflet hinge point is slightly apical to the anterior mitral valve leaflet hinge point (see Fig. The “Displacement Index” is measured in systole or diastole from the insertion point of the anterior mitral leaflet to the hinge point of the tricuspid septal leaflet (where it begins to delaminate). A 2 displacement index >8 mm/m , is one feature that supports a diagnosis of Ebstein anomaly (see Fig. This is difficult in the neonatal setting with elevated pulmonary vascular resistance (88,89). Approximately 10% of patients with Ebstein anomaly are diagnosed utilizing fetal echocardiography (75,76,77). Fetal tachyarrhythmias occur in Ebstein anomaly and can contribute to development of hydrops. This is likely secondary to the combination of the severity of the malformation and the attendant lung hypoplasia. Three-dimensional echocardiography may add incremental information in select patients with Ebstein anomaly. Nuances of Assessment of Tricuspid Valve Regurgitation in Ebstein Anomaly Tricuspid regurgitation may be difficult to accurately quantitate in patients with Ebstein anomaly (93,94). Echocardiographic factors that are typical of severe tricuspid regurgitation in patients with otherwise normal hearts, such as hepatic vein systolic reversal and a wide vena contracta are more rare in Ebstein anomaly despite massive tricuspid valve regurgitation. In addition, due to the anterior and apical displacement of the functional tricuspid valve orifice, the orientation of the regurgitant jets may not be completely visualized in the apical four-chamber view (Fig. The unusual direction of these jets warrants unique echocardiographic windows tailored to each patient (Fig. Quantification is challenging due to difficulty with orienting the plane of sound parallel to the regurgitant jet(s). In young patients, the subcostal windows may provide the best visualization of the tricuspid regurgitation jet(s). In older patients parasternal short axis or anteriorly angled apical views may provide similar information. If there is a single regurgitant jet the diameter of the vena contracta and the density of the signal can provide a convenient and reproducible method for defining tricuspid regurgitation. Conversely, a vena contracta width >8 mm represents severe tricuspid regurgitation. However, in Ebstein anomaly, multiple regurgitant jets are common and measuring the width of each vena contracta may not be useful. Many of the published society valve guidelines are not directly applicable to smaller patients with Ebstein anomaly. In smaller patients, vena contracta diameter <10% of the annulus diameter is considered mild. Vena contracta diameter >25% to 30% of the annulus dimension is classified as severe. The density of the tricuspid regurgitation spectral Doppler signal may also be useful. The tethering and multiple origins of regurgitant flow dramatically decrease the chance for successful monoleaflet repair, and a tricuspid valve replacement was performed. Although a monoleaflet repair was not possible, if this patient presented today the amount of leaflet tissue present suggests that a cone reconstruction would be possible, although each individual fenestration would need to be closed as a part of the repair. Nongeometric measures (such as the myocardial performance index) and qualitative grading of myocardial dysfunction are used in most clinical situations. The functional orifice is also the origin of a single broad jet of regurgitation (right, red arrow). This jet begins near the right ventricular outflow tract and is oriented in inferiorly and toward the diaphragm, near the inferior vena cava–right atrial connection. Echocardiography adequately displays all the anatomical and functional features needed to make clinical decisions in these patients. Cardiac magnetic resonance imaging and the assessment of Ebstein anomaly in adults. A rare circumstance for the use of cardiac catheterization is in the infant with associated critical pulmonary valve stenosis requiring balloon valvuloplasty. Cardiac catheterization is rarely needed in adolescents and adults with Ebstein anomaly. In that case, pulmonary valve competency and low pulmonary vascular resistance are prerequisites. Pulmonary arterial pressure and pulmonary arteriolar resistance usually are normal in patients with Ebstein anomaly. The need for inotropic support is rare but it is used in1 those patients with severe congestive cardiac failure. Several large centers have adopted a management strategy that all patients with Ebstein P. Neonates with Ebstein anomaly can be difficult to manage due to increased pulmonary vascular resistance. This leaves the patient extremely cyanotic due to right-to-left shunting across atrial level defects. The1 use of inhaled nitric oxide may also assist in the reduction of the pulmonary vascular resistance (95). As the pulmonary vascular resistance drops and antegrade flow across the pulmonary valve occurs, the prostaglandin infusion can be weaned. Cyanosis will steadily improve due to less tricuspid regurgitation and less right-to-left shunt at atrial level. In the rare cases of neonates with pulmonary valve regurgitation, a “circular circulation” is established which also increases cyanosis and these patients have a poor outcome. The pulmonary regurgitant stroke volume contributes to the tricuspid regurgitation. Principles of Surgical Management for Ebstein Anomaly The goals of surgery for Ebstein anomaly are: 1. However, those with the most severe forms with persistent cyanosis or important associated lesions will require intervention. Palliative tricuspid valve patch closure with a fenestration (Starnes procedure) in conjunction with a systemic-to-pulmonary artery shunt have been successful in neonates (96). However, early mortality in neonates requiring surgery for Ebstein anomaly remains high (25%). In that approach, the tricuspid valve is repaired and the atrial septum is partially closed. This technique utilizes a monocusp approach based on a satisfactory anterior leaflet (97). The complexity of the contemporary “cone reconstruction” (described below) limits its feasibility in most ill neonates.

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Homograft replacement of the aortic valve and root and Ross procedures (pulmonary autograft) have also been described in pediatric Takayasu case reports (40 purchase generic accutane pills skin care 2 in 1,41 discount generic accutane uk skin care uk,42 order accutane 5 mg on-line acne epiduo,43,75,77,78,80,82,113,114,151,153,176,177). Coronary artery reconstruction and patch angioplasty of stenotic coronary ostia may also be necessary (28,68,84,109,115,116,117,118,119,166,174). Several composite disease activity scores have been developed, and recently applied to children (27,28,29,86,141). Sustained remission was obtained in 79% at 1 year, but dropped to 29% by 5 years (93,124,141). However, disease modifying and biologic medicines appear to be improving outcomes. In a recent Indian cohort of 40 patients under the age of 16 years, 90% survival was noted at 5 years with management in 85% of patients with steroids and immunosuppression with mycophenolate mofetil, azathioprine, or methotrexate. Characteristic features include cutaneous manifestations (heliotrope rash, Gottron papules/rash), symmetrical proximal muscle weakness, and evidence of immune-mediated vasculopathy (periungual capillary changes with telangiectasia and dropouts). Clinical characteristics include proximal and distal muscle weakness, higher muscle enzymes, and more frequent cardiac events (102,127,184). Those with abnormal strain had higher scores of long-term organ damage (myositis damage index) as well as more early disease activity in the skin (28,58,113,122,132,133,194). Multisystem involvement can include mucocutaneous, articular, neurologic, urogenital, vascular, intestinal, and pulmonary manifestations (28,43,197,198). The prevailing hypothesis has been that an autoimmune response is induced in genetically predisposed individuals by a viral, bacterial, or environmental agent and/or an autoantigen, and the autoimmune response triggers vasculitis (114,134,197). Morbidity and mortality is increased in those with cardiac involvement (27,28,29,135,198). Treatment may include optimization of immunosuppression, colchicine, pericardiocentesis, and/or pericardiectomy, depending upon the clinical scenario and response to therapy (3,27,29,31,32,199,202,203,204,205,206). Vasculitis Vasculitis with resultant vessel dilation may involve any vascular bed, including pulmonary vessels, coronaries, and great arteries though it is rare in pediatrics. Similar to Kawasaki disease, coronaries with aneurysms may also develop stenosis over time (28,121,122,123,213,214). Echocardiography is the main imaging modality to assess the proximal coronaries and aortic root. Treatment of sinus of Valsalva aneurysm frequently requires aortic root replacement (222,223,226,227,228). Management of vasculitis should be produced by experts in pediatric rheumatology, and typically focuses on immunosuppression with steroids. In patients with active disease, coronary microvascular function has been reported to be impaired when evaluated by catheterization (232). Treatment of myocarditis is generally with high-dose steroids in addition to standard heart failure treatment. Intracardiac thrombosis has actually been described as the first manifestation of disease in some patients, particularly young men from the Mediterranean or Middle East (239,240). Echocardiographically, a thrombus typically appears as a diffuse, bright, thickened endocardial mass which must be distinguished clinically from vegetation or tumor (241). Intracardiac thrombosis most frequently involves the right ventricle; however, left ventricular and combined ventricular involvement also has been described (28,29,242,243,244,245,246). Pulmonary or cerebral embolism may occur depending upon the location of the thrombus and presence of a patent foramen ovale (56,57,121,122,123,126,130,240,242,243). Treatment of immobile intracardiac thrombi includes aspirin, warfarin, corticosteroids, and immunosuppressive agents. Mobile thrombi are treated with thrombolysis or surgical resection (122,123,126,127,129,130,240,242,243,244,245,246). These findings may be the result of myocarditis, inflammation of the conduction system, or vasculitis of the vessels to the conduction tissues (29,116,141,235,247,249). Scleroderma Introduction Scleroderma is a rare autoimmune collagen vascular disease characterized by fibrous thickening and hardening of the skin. Scleroderma Epidemiology Scleroderma is extremely rare in childhood; for example, the estimated incidence in the United Kingdom and Ireland was only 0. Cardiac pathology affects the conduction system and all cardiac layers from the endocardium to the pericardium in a pathognomonic patchy distribution (69,72,144,259,260). The initial inciting event is unknown, but sex, race, genetic predisposition, and environmental factors may play a role in its development. Other clinical criteria can include sclerodactyly, digital tip ulcers, gastroesophageal reflux or dysphagia, hypertension, arrhythmias or heart failure, pulmonary fibrosis or hypertensions, musculoskeletal changes such as arthritis, myositis, or tendon friction rubs, or neuropathy or carpal tunnel syndrome. Manifestations may include fibrosis of the conduction system, myocardium, and/or pericardium. Secondary cardiac findings may arise as a result of the pulmonary hypertension that may affect up to 80% of all patients (63,65,136,137,145,146,260,263,264,265,266,267,268,269). Palpitations and tachyarrhythmias have been reported in 23% to 35%, respectively, of all patients, and have been attributed to conduction system and myocardial fibrosis (147,270,271,272,273). Reported abnormalities on Holter surveillance include 32% to 42% with supraventricular tachycardia, 20% with premature ventricular contractions, 14% with conduction delay/block, and 10% to 28% with ventricular tachycardia (109,151,152,153,275,276,277). Therefore, Holter monitoring is recommended for those with symptoms and for routine surveillance (32,153,154,276,277). These abnormalities include left ventricular thinning and diastolic dysfunction and left and right ventricular systolic depression (29,73,76,128,129,158,285). Exercise testing may be useful in detecting subclinical or mild reductions in left ventricular systolic function by stress echocardiography with limited cardiac reserve (32,159,282,286). These medications may eventually play a role in primary prevention of the microvascular complications identified in these patients, though further research is necessary. Pericardial involvement does not typically respond to corticosteroid therapy and may necessitate surgical- or catheter-based intervention to relieve fluid (165,260,293). As well, these patients are at higher risk for atherosclerosis (28,168,296,297,298). This is likely secondary to the alveolar, interstitial, peribronchial, and pleural fibrosis (29,141,267). Pulmonary arterial hypertension may also be due to smooth muscle hypertrophy, intimal hyperplasia, vascular inflammation, and thrombosis (141,301,302). Those with pulmonary arterial hypertension have poor survival with a 1-year survival of 45% to 78% (129,144,264,306,307,308). The name “sarcoid” was derived by a Norwegian dermatologist in 1899, who likened the pathologic appearance of the nodular skin lesions to sarcoma cells (142,143,169,170,309). Other organ systems targeted include the cardiac, ocular, pulmonary, skin, endocrine, muscular, and nervous systems. It is reported to affect only 5 to 40 per 100,000 adults in the United States (69,72,144,311). While data in the pediatric populations are sparse, in Danish children the incidence is less than 1/100,000 (32,120,312). Sarcoid is more prevalent in African Americans and in Japan (31,34,109,172,173,174,311,313).

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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 purchase accutane 40 mg overnight delivery skin care myths. Increased prevalence of childhood obesity as a result of sedentary lifestyle and consumption of calorie-dense food predisposes for the early development of diabetes 20 mg accutane for sale skin care with retinol. Biochemistry revealed fasting plasma glucose of 190 mg/dl cheap accutane 10mg line acne keloidalis cure, 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.