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Sidney C. Smith, Jr., MD

• Professor of Medicine
• Division of Cardiology
• University of North Carolina School of Medicine
• Chapel Hill, North Carolina

Nowadays the Metabolic Therapy includes a number of variants diabetes test kit in india buy 100 mg januvia free shipping, each of them named after the doctor who used it diabetes type 2 blood sugar range purchase 100mg januvia mastercard. After 2 metabolic disorder ketosis discount januvia uk,500 years he revived concepts and thoughts that were already developed by the great Greek doctor Hippocrates of Cos diabetes insipidus definition cheap januvia 100mg visa, the founder of Western Medicine. Therefore, malignant tumours are essentially caused by chronic deficiencies in vitamins (their lack does not allow the repair of the genetic damage or the apoptosis-induced death of affected cells) and the treatment of these tumours must be based on the intake of high doses of vitamins, in order to cause the spontaneous suicide (apoptosis) of tumour cells. Some of these vitamins can be taken also intravenously to increase their accumulation on tumours. Under no circumstances should this therapy be used because it does not make sense to poison the body of a patient who is already seriously ill. Second principle: giving the tumour what kills it (but without damaging the patient). The first principle is based on the removal of Proteins (essential amino acids), vitamin B12, folic acid, Glucose and other substances, including hormonal substances, which can induce tumour cells to proliferate. Their use is extremely important as it allows rapid elimination of the toxins released by tumour masses (which are inflamed and therefore larger as a result of the immune response), thus reducing the pain deriving from the tumour masses themselves. This stage of the treatment is extremely important, as it allows the melting of the tumour mass over some months. In metabolic therapies very complex dietetic protocols are studied, but they all have a similar approach: frequent but small meals with low glycemic food. Famous is the scientific study conducted in 23 1995 on 153 patients suffering from malignant melanoma, which demonstrated percentages of remission much higher than those obtained with conventional therapies (40% of surviving patients as against 6% with Chemotherapy). In 1994, professor Binzel published the results obtained by treating his patients between 1974 and 1991. Out of 180 patients suffering from primary cancer (not metastasized and circumscribed to one single organ or tissue), 131 were still alive in 1991, when the report was published. In that year, 58 patients had been followed for 2-4 years, whereas 80 for 5-18 years. Out of the 42 patients who died in 1991, 23 died of cancer, 12 of unrelated causes and 7 of unknown causes. Among patients with metastasization, 32 out of 108 died of cancer, 6 of unrelated causes and 9 of unknown causes. Out of the 61 patients who were still alive in 1991, 30 had been followed for 2-4 years, 31 for 5-18 years. She studied many other diseases, above all Multiple Sclerosis, and documented more than 600 cases. The term commercially confidential used on some documents is a way of not letting people know about them. This goes against the recommendations of the Aarhus Convention, which is an agreement of the United Nations Economic Commission for Europe linking the environment to human rights. From these components we achieve our ability to defend our health, and therefore, to live as long as possible without disease, in good psycho-physical condition. In this sense, chronic-degenerative diseases such as arthrosis, osteoporosis, autoimmune diseases, tumours, heart diseases, diabetes, and neurological deficits (Alzheimers, Parkinsonisms, Multiple Sclerosis, etc) would be controlled more effectively. Food is divided as: 1) Carbohydrates 2) Proteins 3) Fats and/or Oils 4) Vitamins 27 Chapter 1. The daily requirement for an adult weighing 70 kilograms is about 2,000-2,500 Kilocalories. Note: for athletes, energy requirements can reach about 4,000-4,500 Kilocalories per day. In severe situations, such as for severe burns, they are significantly higher: about 6,000 Kilocalories per day. There are ready-made commercial sugars as well (white sugar, brown sugar, chocolate, glucose, mannose, ribose, galactose, etc. Indeed, fruit can give the following Kilocalories: 1 litre of well-whisked fresh fruit(^) (grapes and/or berries) gives about 800-900 Kilocalories, that equals: 750 cc milk (*), or: 70 grams cheese (*), or: 650 grams meat (*), or: 800-900 grams fish (*), or: 10 eggs (*). Raspberries (Rubus idaeus) 34 Oranges (Citrus aurantium) 34 Quinces (Cydonia oblonga) 34 Melon (Cucumis melo) 33 Natural Orange Juice (Citrus aurantium) 33 Carrots (Daucus carota) 33 Black Truffle 31 Sweet Soda 31 Spinachs (Spinacia oleracea) 30 Field Asparagus 29 (Asparagus officinalis, adscendens, racemosus). Cereals and legumes contain up to 7-8 essential amino acids, but the complete range of 9 essential amino acids is never contained in any of them. Besides, cooking traditions all over the world have always associated cereals to legumes as a sort of meat for the poor. This is not true: many patients manage to heal from very severe forms of chronic-degenerative diseases just by completely suspending the supply of all 9 essential amino acids for many months, obviously under the supervision of a doctor, in order not to have severe forms of protein malnutrition due to lack of food (see for example blood tests searching for Total Proteins, Albuminemia, Pre-albuminemia, etc. This explains why all mammals suckle their offspring until weaning, after which they stop feeding them with milk. When pH is low, that is acidic, our body will lose its alkalizing minerals while trying to restore the right biochemical balance (buffer system). Urine will noticeably have a strong smell of ammonia, and urination could even be painful, because of the caustic nature (highly basic pH) of the urine that is being eliminated. It is suggested to drink some acidulous fruit juice (blueberry, orange, lemon juice, etc) that will bring the solution back to normal and eliminate the pain. A strong smell of ammonia in urine could mean that our body is running out of alkalizing minerals. Of course, our body can find other stocks of alkalizing minerals such as calcium, sodium and magnesium, but by doing so these precious minerals will be taken from bones, later causing damage and causing, in the long term, arthrosis and osteoporosis. In turn, producing too much ammonia will cause in the long term a gradual but irreversible kidney chronic failure (demonstrated by the presence of proteins in urine). If our body does not have enough calcium and magnesium, it will take the required amounts of these minerals from bones, to guarantee adequate levels in blood. Then, our body will try and make up for this lack of calcium and magnesium by creating bony deposits that reduce movement and limit activities (arthrosis, arthritis). Magnesium and vitamin D (obtained thanks to sun exposure) are the safest solution to avoid such diseases. Restoring the biochemical conditions of the complex system in a young adult can take only a few months; on the contrary, in an elderly adult more than a year might be needed before pH (for example, salivary pH) goes back to being slightly alkaline. The loss of these good germs is due to eating too many proteins, rich in essential amino acids (all nine of them), in vitamin B12, and in glucose (simple sugar) that are freely available in the intestine. Glucose, and the presence of All Nine Essential Amino Acids, are the necessary source to develop the bad gut flora, that is, the one that causes putrefaction. The human intestine has a volume of about 6 litres and an enormous surface of about 400-600 square metres. From the throat to the anus, there are 150 very important lymphatic centres, where white blood cells (lymphocytes) maintain immune defences. This area is called intestinal lumen, it is very rich in good and bad germs, and it can be considered to be the most dangerous and crucial area of our body. In fact, the two lungs have a much more limited total surface (just 80 square metres). In an adult, the skin has a surface of no more than 2 square metres this immense intestinal surface, then, marks the difference between a healthy condition and disease. In vegetarians, 20-40% of fecal mass is made of good germs (enterobacteria, or symbiotic or saprotrophic germs). These germs, however, are present in all individuals in the higher part of the intestines (first and second part of the small intestine: duodenum and jejunum). The following are among the most important: Bifidobacterium bifidum, Lactobacillus acidophilus, Lactobacillus bulgaricus, Lactobacillus lactis, Lactobacillus rhamnosus; others: Edwardsiella, Citrobacter, Providencia, Arizona, Escherichia coli, Enterobacter, Serratia, Klebsiella, Pseudomonas, Shigella, Vibrio, Proteus, etc 36 Some subspecies of these germs are pathogenic (Vibrio colerae, Shigella dissenteriae, Pseudomonas aeruginosa). These bacteria are not damaged by a vegetarian diet, even though fruit, vegetables and spices are rich in germicidal, fungicide and parasiticidal substances.

Research has demonstrated that the early onset of sexual activity with others is usually accompanied by other risk behaviors diabetes signs when pregnant buy januvia 100 mg with mastercard, such as substance use diabetes mellitus zahnextraktion order 100mg januvia overnight delivery, school problems diabetes daily purchase januvia online now, and parent-teen conflict diabetes sliding scale definition buy januvia 100mg with amex. It is also highly associated with a history of physical and sexual abuse, both inside and outside the family. If an adolescent does become sexually active, these factors also influence the ability to engage in "safer sex" practices. In general, the earlier the age of sexual initiation the more likely there are associated risk factors and a history of significant childhood abuse. The initiation of sexual activity during later adolescence is more likely to represent a normative process with fewer associated risks. The latter interventions encourage abstinence as the safest choice but recognize that some adolescents will choose to be sexually active and should be provided the information and skills they need to make that activity as safe as possible. One of the most neglected areas related to adolescent sexuality has been that of sexual orientation. During puberty, approximately 3 to 10 percent of adolescents begin to recognize their lesbian or gay (homosexual) sexual orientation. An even greater percentage may be bisexual while a small minority is transgender, feeling as if they are one gender trapped in the body of the other gender. Sexual orientation and gender identity are not a choice and appear to be established by early childhood. Pediatrics now regards homosexuality and bisexuality as normal and healthy developmental outcomes. Certain segments of society regard a minority sexual orientation or transgender identity as pathologic or sinful. A small percentage run away from home, drop out of school, and turn to drugs, street-life, prostitution, or suicide as a means of escape. Health providers have a special responsibility to these disenfranchised youths to make sure that they have access to accurate information, appropriate health care, and supportive community services so they may develop into healthy and productive adults. A health provider has multiple roles in addressing issues of sexuality with adolescent patients, including those of screener, educator, counselor, and advocate. Research indicates, however, that many providers feel uncomfortable and unskilled in discussing sexuality with their adolescent patients. Therefore, providers must first examine their own comfort and attitudes about sexuality, particularly as these relate to adolescents, and reflect on how these attitudes affect their work with teenagers. As educators, providers are in an excellent position to provide accurate information and anticipatory guidance to teenagers and their families, not only about pubertal development but also about normative sexual development during the adolescent years. As counselor, the provider should encourage postponement of sexual activity with others until the adolescent has the physical, emotional and cognitive maturity to enter into relationships that are consensual and non-exploitative. The provider should counsel adolescent patients that healthy sexual relationships should be both honest and pleasurable, and that steps should be taken to prevent sexually transmitted infections and unintended pregnancy. At a community level, health providers are in an excellent position to participate in the development and delivery of comprehensive sexuality curricula in the schools and other community forums. They also can be strong advocates for the development of confidential, accessible and affordable reproductive services for teenagers and for policies that nurture and support the healthy sexual development of all adolescents. A 16-year-old boy reveals to you that he has become increasingly aware of his sexual attraction to other boys. Which is the most appropriate first response as a pediatrician to this revelation Reassure the boy that such feelings are normal and may or may not be indicative of a homosexual or bisexual orientation. True/False: the onset of sexual activity in older adolescents may have different antecedents, predictors and consequences than that in younger adolescents. In the field of pediatrics which of the following is considered abnormal in adolescent sexual development. American Academy of Pediatrics Committee on Psychosocial Aspects of Child and Family Health and Committee on Adolescence. The incidence of adolescent sexual activity, at least among in-school youth, appears to be declining. In addition, sexually active adolescents report fewer sexual partners and are more likely to use condoms than teenagers in the early 1990s. Same-sex attraction is considered a normal part of adolescent and adult sexual experience. It may or may not reflect a bisexual or homosexual orientation, either of which, like heterosexuality, is believed to be established in early childhood and represents a normal developmental outcome. The onset of sexual activity in younger adolescents is more likely to be associated with a history of negative life experiences and high-risk behaviors such as sexual abuse, substance use, parent-teen conflict and school problems. In older adolescents, the onset of sexual activity is often a more normative process. Pediatrics as a discipline recognizes that sexual experimentation, with oneself and others, is a normal part of adolescent development. More controversial are the issues of age of initiation of sexual activity and the nature of those activities. There is a wide spectrum of viewpoints within pediatrics, reflecting broader societal views, on these latter issues. Masturbation, homosexual orientation, and sexual fantasies and experimentation are considered a part of the spectrum of normal adolescent sexual development. She believes her menses have been "more or less" regular but she has never kept track. She states that when she has her menses, she has pain that is occasionally bad enough that she misses school. She states that she feels perfectly fine and refuses to have a pelvic exam performed. You explain carefully the need for a thorough gynecological exam and how the exam will be done. Clinical Course: After discussing contraceptive options with the patient, she decides on the combined oral contraceptive pill. One month later, you get a call from her mother who is upset when she sees her medical insurance statement which shows an itemized expense list which contains a pregnancy test, a gonorrhea culture, and a prescription claim for birth control pills. When dealing with the adolescent patient, it is important to remember that the adolescent is the patient, even if she is accompanied by a parent, usually the mother. She may be afraid that it will be painful, and will likely be embarrassed about undressing (1). In allaying such fears, it would be helpful to direct the majority of the initial discussion toward the adolescent and to explain the exam completely. Speaking to the mother alone is useful for obtaining family history that may be pertinent and for uncovering any concerns that she may have. Patients in this age range are often modest about the changes taking place in their bodies, and it is often best to leave it up to the girl whether she wants her mother present during the exam. It is important to speak to the patient alone at some point because she may have information that she is reluctant to reveal in the presence of her mother. When attempting to solicit information about her menstrual cycles, it is best to ask specific questions regarding the frequency of menstrual flow, length of menses, and the amount of blood lost. When assessing the amount of blood lost, the patient should be asked how long it takes to soak through a tampon or pad, if she ever has to awaken in the night to change a pad, or if she has to use both methods at once. It is not enough to simply note how many pads or tampons the patient uses in a day because she may change pads as soon as one is soiled, wait until it is soaked, or change it according to her class schedule which dictates when she can make it to the restroom. Estradiol promotes the formation of uterine endometrial glandular cells and stroma. Menarche occurs when estrogen levels are sufficient to stimulate proliferation of the uterine endometrium. Estradiol also stimulates the development of the follicle and its levels increase in puberty until ovulatory cycles are established. Following menarche, plasma estradiol levels range from 50-200 pg/ml during the follicular phase, while progesterone levels range from 200-2500 ng/dl (average 750) during the luteal phase (2). The majority of cycles within the first 2 years after menarche are anovulatory, and there tends to be great variance in cycle interval, duration of flow, and amount of blood loss. Cycle intervals may be as long as 6 months and continue to be irregular for the first 15 cycles (2,4,5,6).

A systematic review of the effects of hormone therapy on psychological functioning and quality of life in transgender individuals early symptoms diabetes in dogs generic 100mg januvia otc. Medical providers who feel comfortable making an assessment and diagnosis of gender dysphoria diabetes type 1 virus januvia 100 mg on line, as well as assessing for capacity to provide informed consent (able to understand risks diabetes diet pregnant proven 100mg januvia, benefits diabetes urine test purchase generic januvia on line, alternatives, unknowns, limitations, risks of no treatment) are able to initiate gender affirming hormones without a prior assessment or referral from a mental health provider. Qualifications of the prescribing provider Prescribing gender-affirming hormones is well within the scope of a range of medical providers, including primary care physicians, obstetricians-gynecologists, and endocrinologists, advanced practice nurses, and physician assistants. Most medications used in gender-affirming hormone therapy are commonly used substances with which most prescribers are already familiar due to their use in the management of menopause, contraception, hirsutism, male pattern baldness, prostatism, or abnormal uterine bleeding. Updated recommendations from the world professional association for transgender health standards of care. June 17, 2016 25 Guidelines for the Primary and Gender-Affirming Care of Transgender and Gender Nonbinary People 7. Estrogens the primary class of estrogen used for feminizing therapy is 17-beta estradiol, which is a bioidentical hormone in that it is chemically identical to that from a human ovary. Compounded estradiol valerate or cypionate for injection also exists, and may be an alternative in times of shortage or more cost effective for those who must pay cash for their prescriptions. Conjugated equine estrogens (Premarin) have been used in the past but are not recommended for a number of reasons, including inability to accurately measure blood levels and some suggestion of increased thrombogenicity and cardiovascular risk. Androgen blockers allow the use of lower estradiol dosing, in contrast to the supraphysiologic estrogen levels (and associated risks) previously used to affect pituitary gonadotropin suppression. Spironolactone is a potassium sparing diuretic, which in higher doses also has direct anti-androgen receptor activity as well as a suppressive effect on testosterone synthesis. Due to its diuretic effect, patients may experience self-limited polyuria, polydipsia, or orthostasis. Since these medications block neither the production nor action of testosterone, their antiandrogen effect is less than that encountered with full blockade. In the absence of estrogen replacement, some patients may have unpleasant symptoms of hot flashes and low mood or energy. Progestagens: There have been no well-designed studies of the role of progestagens in feminizing hormone regimens. In reality some patients may respond favorably to progestagens while others may find negative effects on mood. This class includes micronized bioidentical progesterone (Prometrium) as well as a number of synthetic progestins. The most commonly used synthetic progestin in the context of transgender care is the oral medroxyprogesterone acetate (Provera). First, the transgender women may be at lower risk of breast cancer than non-transgender women. The actual findings in the conjugated equine estrogen plus medroxyprogesterone group were an excess absolute risk per 10 000 person-years of 7 more cardiac events events, 8 more strokes, 8 more pulmonary emboli, and 8 more invasive breast cancers, with no change in overall mortality. Initial-low dosing for those who desire (or require due to medical history) a low dose or slow upward titration. Maximal effect does not necessarily require maximal dosing; as such maximal doses do not necessarily represent a target or ideal dose. As such an escalating regimen beginning with low dose estrogen only, and titrating up over several months, and then adding spironolactone may be an alternative approach,[17] consistent with management practices in children with delayed pubertal onset (Grading: T O W). It is recommended that providers discuss these considerations with patients before initiation of hormones in order to make an informed decision. However, estrogen levels in non-transgender women may not be associated with specific secondary sex characteristics. A general approach for titration would include increasing of both estrogen and antiandrogen dosing until the estrogen dose is in the female physiologic range. Once this has been achieved, titration efforts can focus on increasing androgen blockade. The drawback for this approach is that patients may begin to experience estrogenic side effects as described below. Some providers choose to omit the use of hormone level testing and only monitor for clinical progress or changes. Regardless of initial dosing scheme chosen, dosing may be titrated upwards over 3-6 months. While laboratory monitoring of hormone levels may seem complex, it is of similar difficulty to the monitoring of other similarly complex lab-monitored conditions managed by primary care providers, such as thyroid disorders, anticoagulation, or diabetes. As with other situations involving maintenance of hormone therapy (menopause, contraception), annual visits are sufficient for transgender women on a stable hormone regimen. Increased frequency of office visits may also be useful for patients with complex psychosocial situations to allow for the provision of ancillary or wraparound services. This is consistent with Endocrine Society recommendations that only total testosterone be monitored in non-transgender men being managed for testosterone deficiency, except in cases of borderline testosterone levels. However, since testosterone is of particular concern is insuring maximal feminization, the calculation of bioavailable testosterone in transgender women may still be of value. For example, a transgender woman who is still registered as male will result in lab reference ranges reported for a male; clearly these ranges are not applicable for a transgender woman using feminizing hormone therapy. In patients who have been using self-administered conjugated estrogens, or ethinyl estradiol, it is reasonable to check a total estrogens level, which may provide a more accurate estimate in these cases. Monitoring testosterone levels Testosterone levels can be difficult to measure in non-transgender men due to rapid fluctuations in levels, relating to pulsatile release of gonadotropins, with higher levels in the morning hours. When measuring hormone levels in patients using injected forms of estradiol, a mid-cycle level is often sufficient, however if the patient is experiencing cyclic symptoms such as migraines or mood swings, peak (1-2 days post injection) and trough levels of both estradiol and testosterone may reveal wide fluctuations in hormone levels over the dosing cycle; in these cases, consider changing to an oral or transdermal preparation, or reducing the injection interval (with concomitant reduction in dose, to maintain the same total dose administered over time). A single study suggests similar pharmacokinetics when estradiol is injected subcutaneously, rather than intramuscular. Several factors contribute to these differences, bone mass, muscle mass, number of myocytes, presence or lack of menstruation, and the erythropoetic effect of testosterone. Lower and upper limits of normal to use when interpreting selected lab tests in transgender women using feminizing hormone therapy Lab measure Lower Limit of normal Upper Limit of normal Creatinine Not defined Male value Hemoglobin/Hematocrit Female value Male value Alkaline Phosphatase Not defined Male value Individualized dosing based on patient centered goals Some patients may desire limited hormone effects or a mix of masculine and feminine sex characteristics. While manipulation of dosing regimens and choice of medication can allow patients June 17, 2016 34 Guidelines for the Primary and Gender-Affirming Care of Transgender and Gender Nonbinary People to achieve this goal, it is important to have a clear discussion with patients regarding expectations and unknowns. At the same time, response to hormone therapy is also individualized and measures such as breast growth are variable in both degree and time course. After an in depth and careful informed consent discussion, it is reasonable to prescribe estrogen using a harm reduction approach, with a preferred route of transdermal estrogen. This study found no correlation between sexual desire and testosterone levels in the transgender women, though a significant correlation was found between hormones and desire in non-transgender women. Post-gonadectomy: Since estrogen dosing should be based on physiologic female levels, no reduction in estrogen dosing is required after gonadectomy. Some patients may choose to use a lower dose, which is appropriate as long as dosing is adequate to maintain bone density. Due to higher levels of co-occurring conditions in older individuals, there may also be higher risk of adverse effects. Pituitary adenoma (prolactinoma) and galactorrhea: Prolactin elevations and growth of pituitary prolactinomas are theoretical risks associated with estrogen therapy; several cases have been reported. It is noted that some transgender women experience a minimal amount of galactorrhea early in their hormone therapy course. The presence of non-bloody minimal galactorrhea from more than one duct and/or bilateral is almost certainly physiologic and would not warrant further evaluation. Venous thromboembolism: Data from studies of menopausal women suggest no increased risk of venous thromboembolism with the use of transdermal estradiol. A report of 11 transgender women with a history of activated protein C resistance (the mechanism of action implicated in the hypercoaguable state associated with the Factor-V Lieden mutation) using transdermal estradiol without anticoagulation found no clotting events after a mean of 64 months of therapy. Routine screening for prothrombotic mutations is not recommended in the absence of risk factors. Testosterone has been associated with overall immune suppression, and autoimmune conditions are more common in non-transgender women than men. Oral or transdermal estrogen may be preferred to the potentially cyclic levels associated with injected estrogen. Mental health conditions: While hormones may contribute to mood disorders (such as in premenstrual dysphoric disorder or postpartum depression), there is no clear evidence that estrogen therapy is directly associated with the onset of or worsening of mental health conditions.

Syndromes

• Syringomyelia
• Pain
• Younger age
• Holes (necrosis) in the skin or tissues underneath
• Fever
• Pins, hairpins, metal zippers, and similar metallic items can distort the images.
• Head CT scan
• Nausea

Determining whether fluid will move into or out of the capillary is based on the net filtration pressure blood glucose numbers chart buy januvia 100mg amex. Diffusion limited a means that gas doesnt equilibrate by the time it reaches the end of the capillary diabetes education classes buy cheap januvia line. Perfusion limited is seen in healthy people diabetes mellitus child 100mg januvia, whereas diffusion limited occurs in those with emphysema diabetes mellitus type 2 discharge planning generic januvia 100 mg online, fibrosis, or when exercising. The question may come in the form of a definition, but it will most likely come in the form of a chart where you will have to calculate. If there is a mismatch, this indicates that there is a shunt and some degree of dead space in the same lung. A V/Q of 0 is indicative of a shunt (ie airway obstruction) A V/Q of is indicative of an obstruction of blood flow (ie physiological dead space). The metabolic pathways are very important; paying special attention to regulatory steps is crucial. While the details of biochemistry are not high-yield, the big picture as a whole is very high-yield material. There is no migration into or out of the population the frequency of different alleles in a population can be determines with the Punnett square, which can be linked mathematically to the with Hardy-Weinberg equation for equilibrium. The antibody or antigen that is added is linked to an enzyme, then a test solution is added to see if an intense color illuminates, indicating that there is a positive result. These proteins are then transferred to a membrane where they are probed using antibodies specific to the target protein. This technique allows for the detection of cellular control by determination of gene expression levels during differentiation and morphogenesis. The nucleotides belonging to the pyrimidine group are: Cytosine, Uracil, and Thymine. There are two types of chromatin, there is heterochromatin, which is condensed and transcriptionally inactive, and there is euchromatin, which is looser and transcriptionally active. Double-Strand Damage: Three mechanisms exist to repair double-stranded damage, they are: 1. Those afflicted with this disease are extremely sensitive to sunlight and have a significantly high risk for skin cancer. Symptoms include: photosensitivity, brittle hair and nails, scaly skin, protruding ears, physical and mental retardation, and a receding chin. The patient suffers from sensitivity to sunlight, have short-stature, and age prematurely. Phenylalanine builds up causing a myriad of severe symptoms the increase in phenylalanine leads to an increase in phenylketones (phenylpyruvate, phenylacetate, and phenyllactate) in the urine. This is an autosomal recessive condition, where the patient cannot produce melanin from tyrosine (tyrosinase deficiency) or from a defect in the tyrosine transporters. There is an increase in the risk of skin cancer due to the lack of protective melanin in the skin. The cause is a defect in the ability to break down the branched chain amino acids Leucine, Isoleucine, and Valine. Homogentisic acid (alkapton) thus accumulates in the blood and is excreted in the urine in large amounts, leading to blackening of the urine upon standing. Excessive amounts of homogentisic acid cause damage the cartilage, leading to severe arthralgias. In this case, cysteine will be essential and should be increased in the diet, while simultaneously decreasing the amount of methionine in the diet. This results in an excess of cystine in the urine, which can predispose the patient to kidney stones. Glycogenolysis occurs in the liver and muscle, and is stimulated by epinephrine and/or glucagon in response to low blood glucose levels. This phase consumes energy that is used to convert glucose into two 3-C sugar phosphates (G3P). Pyruvate dehydrogenase Hexokinase: Hexokinase is responsible for the first step of glycolysis in the muscles and brain. This complex consists of three enzymes that transform pyruvate (from glycolysis) into acetyl-CoA, through the process of pyruvate decarboxylation. There will be neurologic findings that can be managed by giving the patient amino acids that are purely ketogenic, such as Leucine and Lysine. In yeast, pyruvate is converted to ethanol in anaerobic conditions, in eukaryotes it is converted to lactate. There are four complexes that are embedded in the inner membrane, which are electrically connected by lipid-soluble electron carriers and water-soluble electron carriers. Two other electrons are passed across the protein reducing ubiquinone to quinol, and four protons are released from two ubiquinol molecules. Is with Cytochrome C oxidase, where four electrons are removed from four molecules of cytochrome c, and thus transferred to oxygen, thus producing two water molecules. Four more protons are transferred across the membrane, further contributing to the gradient. The process of creating glucose from pyruvate is quite costly compared to the amount of energy created by one molecule of glucose. Insulin is secreted by the cells of the pancreas in response to an elevated blood-glucose level. On the other side, glucagon is secreted by the cells of the pancreas in response to low levels of blood-glucose. Glucagon causes the liver to release glycogen which is broken down into glucose, and used to increase the amount of glucose running through the blood. The pharmacological basis of lowering cholesterol (statin drugs), is designed around the inhibition of this enzyme. The rate limiting enzyme is Acetyl-CoA Carboxylase, which does the following: Acetyl-CoA a Malonyl CoA this step is positively effected by with citrate, and negatively effected by with palmitoyl CoA. Other functions of apolipoproteins include acting as co-enzymes and as ligands between the lipoproteins and the tissues they supply. Lipoproteins: Because fatty acids alone have trouble being transported through aqueous compartments inside the cells, a mechanism must be in place to allow them to get to where they need to be, thus enters the lipoproteins. The basic structure of the lipoprotein is below: Lipoproteins are different based on the ratio of protein:lipids, as well as the particular apoproteins and lipids that they contain. Responsible for transportation of cholesterol from the peripheral tissues back to the liver. Transports and delivers both triglycerides and cholesterol to the liver, where they get degraded to low-density lipoproteins. Has a dual role as it supplies the peripheral tissues with triglycerides and supplies cholesterol to the liver. This leads to an accumulation of porphobilinogen in the cytosol, which causes a myriad of symptoms.

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