Obesity and Sexual disorders-Primary and secondary hypogonadism, hermaphroditism and Menopause
TESTOSTERONE DEFICIENCY SYNDROMES
Testosterone has a role in the development of fetus and its absence will result in female phenotype. A lack of testosterone at the expected time of pubescence is manifest by delayed closure of epiphysis and eunuchoidal skeletal proportions, no deepening of the voice, delayed and scanty growth of pubic and axillary hair, absence of beard and mustache growth, small prostate, small penis and a nonpigmented, nonrugated scrotum, low testicular volume with absent spermatogenesis, poor muscular development, and usually, psychosocial immaturity. The patients are usually brought to the physician by their parents, who are worried about poor growth and development.
Primary hypogonadism (due to Leydig cell dysfunction. GnRHs in serum or urine are elevated because of decreased feedback at the pituitary – hypothalamic unit).
Secondary (due to disorders of the hypothalamic – pituitary unit. Pituitary and hypothalamic hypogonadism may be differentiated by appropriate testing with GnRH)
Leydig cell function is depressed in malnutrition, in renal failure, myotonic dystrophy, in chronic disease, to a variable extent with aging, and by certain toxins such as lead and alcohol.
This most frequent cause of primary hypogonadism is defined as the presence of one or more extra X chromosomes in at least one tissue.
The hallmark of the Klinefelter’s syndrome is the presence of small and firm testes, containing sclerosed tubules with only rare sertoli cells, and there is thus usually azoospermia. Eunochoidal habitus, gynecomastia, female distribution of body fat, particularly around the hips, and female distribution of pubic hair, lack of temporal recession of the hairline, arched palate, mental retardation constitute the typical findings. Many of these clinical findings may be absent and the disease manifests itself only by infertility or decreased fertility.
Laboratory studies include azoospermia, chromatin-positive buccal smear, and the elevated plasma FSH and LH. An XXY chromosomal pattern is most characteristic, but may be XXY/XX, XYY, XXYY, and multitude of others are known. Plasma testosterone levels are in the low range or slightly depressed.
Sertoli-cell-only syndrome (germinal aplasia).
These patients present as essentially normal men with slightly reduced testicular volume and infertility. There is an absence of germinal cells in the tubules. Plasma testosterone is normal, and elevated serum FSH concentrations. There are no chromosomal abnormalities in this syndrome, and the buccal smear is negative.
Noonan’s syndrome (male Turner’s)
The prepubertal male is hypogonadotropic. FSH stimulation of tubular development is the first evidence of pubescence. At the present, there is no reliable test to distinguish between delayed puberty and hypogonadotropic hypogonadism.
Kallman’s syndrome (hypogonadotropic hypogonadism).
It is the most frequent cause of secondary hypogonadism. It is inherited as an autsomal dominant with variable penetrance and is characterized by low FSH, LH levels, anosmia or hypoosmia, and the variable occurrence of short fourth metacarpals, syndactyly, midline skeletal defects, and mental retardation. Inadequate secretion of FSH and LH may occur as an isolated defect as well. In both cases the disease can be shown to be hypothalamic in origin, since repeated injections o GnRH will eventually elicit a normal gonadotropin response. Boys remain sexually prepubescent until either testosterone secretion is induced by HCG (chorionic gonadotropin) or androgen is given.
Isolated LH deficiency (fertile eunuch syndrome).
This is a rare syndrome, the boys having pubertal testicular size and some spermatogenesis in the absence of signs of androgen effect. The deficiency of LH is not complete and HCG will virilization and increased sperm counts.
Laboratory assessment of Leydig cell function
In the adult male, cessation or diminution of testosterone secretion is difficult to appreciate clinically. The symptoms of loss of libido and impotence are not specific for androgen lack and the most often occur in men with normal Leydig cell function. A decrease in frequency of shaving, early soft wrinkling of the face, and a softening of the testis may be present.
serum testosterone levels,
Patients with primary hypogonadism can be virilized with exogenous androgens (testosterone enanthate). They cannot become fertile and gonadotropin treatment need not be considered.
Patients with secondary hypogonadism can be treated by androgens and HCG.
Syndromes of androgen resistance.
Syndromes manifested by feminine habitus and the presence of testes, and is characterized by an absolute or varying degrees of resistance to androgen action as a result of absent or decreased amounts of intracellular receptor for dinydrotestosterone.
Patients present in their teenage years as girls with primary amenorrhea, this pseudohermaphroditism is inherited, with transmission as an X-linked recessive or autosomal dominant trait. In the most extreme form, testicular feminization, the women have well-developed breasts, absent pubic and axillary hair, normal external genitalia, a short blind vaginal pouch, an absent uterus, and testes(palpable “masses”) present either in the labial folds or inguinal canal. The tests have small tubules and lack germ cells.
Laboratory data are X, Y genotype, testosterone, and estradiol levels at the upper limits of the normal adult male range, high LH (due to androgen resistance), and usually normal FSH (due to persistent Sertoli cells).
Treatment of these patients consists of removal of the testes to obviate the increased risk of testicular neoplasm, and replacement of estrogens. Patients are irreversibly infertile.
Precocious puberty -
is activation of the hypothalamic-pituitary axis with a consequent enlargement and maturation of the gonads, and the development of the secondary sexual characteristics, adult serum testosterone levels, and spermatogenesis (the onset of sexual maturation before age 10 in males).
The incidence of true precocious puberty is greater in females (2:1), and about 80 % of female cases have no identifiable abnormality. In contrast, 60 % of male cases have underlying organic disease.
Boys exhibit facial, axillary, and pubic hair, penile growth, and increased masculinity. Linear growth is initially rapid in both sexes, but the adult height is shortened by premature closure of the epiphysis.
Laboratory evaluation should include skull x-rays, 24-h urinary 17-KS measurement, and the serum LH, FSH levels, CT scanning of the brain.
Treatment include therapy of the organic factor and suppressing of gonadotropins.
Sexual disorders in the female are often presented with menstrual abnormalities such as primary (Turner’s syndrome, congenital adrenal hypoplasia) and secondary amenorrea.
Turner’s syndrome (ovarian dysgenesis)is characterized by 45,X karyotype and chromatin-negative buccal swear, streak gonads, infertility, primary amenorrhea, short stature, sexual infantilism, a variety of phenotypic abnormalities (webbing of the neck, high-arched palate, low posterior hairline, low-set ears, cubitus valgys, chest deformities, shortening of metacarpal, metatarsal, and phalangeal bones, hypoplastic nails, pigmented nevi, small mandible, epicanthol folds, lymphedema of the hands and feet, tendency for keloid formation) plasma gonadotropins are usually elevated.
It is the presence of male and female gonads in the same individual.
The exact origin of true hermaphroditism is not known.
The external genitalia are ambiguous, with either male or female predominance. A penis with hypospadia and cryptorchism is often present. Breast development and menses occur in about 70 % of the patients ovulation and spermatogenesis are uncommon most patient are raised as males. 2/3 of patients are chromatin-positive, and the most common karyotype is 46,XX. Mosaic patterns such as XX/XY and XY/XXY are often found.
It is discontinuation of menstruation. It may be natural (results from age-related declining ovarian function and usually occurs between ages 40 and 50. As the ovary becomes atrophic and ceases to respond to gonadotropin stimulation, the few remaining follicles undergo atresia and urinary gonadotropin excretion increases sharply), premature (refers to cessation of ovarian function before the age 40, and must be distinguished from gonadal dysgenesis and hypopituitarism), artificial (follows ovariectomy, irradiation of the ovaries).
Menopause may be asymptomatic or symptoms primarily due to estrogen deficiency and autonomic nervous system responses may be severe and last a few months or year.
- vasomotor effects (hot flushes and sweating);
- psychological (anxiety, emotional lability, irritability);
- genitourinary (dyspareunia (senile vaginitis), vaginal infections, urgency of micturation);
- changes of the skin and hair (dryness, hair loss);
- cardiovascular disorders.
Treatment include nonmedicamentous (diet, physical exertions, massage), medicamentous (vitamins, sedative, calcium preparations), hormonal therapy (estrogens).
Obesity is characterized by excessive accumulation of body fat .
Obesity in not a condition for which a precise definition is particularly useful. Unlike many “real” diseases, obesity represents one arm of distribution curve of body fat or body weight, with no sharp cut-off point. Its importance lies in the many, often serious, complications to which obese people are subject. In these complications that warrant undertaking a treatment that is so often unsuccessful.
The cause of obesity is simple – consuming more calories than are expended as energy. However, we usually do not know why persons consume more calories than they expend.
1. Social factors (obesity is prevalent among lower-class people than among upper-class. Other social factors, particularly ethnic and religious are also closely linked to obesity, how these factors lead to obesity, or its control, has not been established, but differences in life style, dietary and exercise patterns, probably play a major role).
2. Sex (female have greater tendency to gain weight particularly at puberty and during pregnancy), age (at middle aged people have more tendency to become obese. Anyhow, obesity is present among all age groups).
3. Endocrine factors. (Certain diseases of endocrine glands are associated with obesity i.e. hypothyroidism, Cushing’s disease, hypogonadism.)
4. Psychological factor (many obese persons report that they overeat when emotionally upset, but many nonobese persons also overeat in such conditions. Two deviant eating patterns based on stress and emotional disturbance, however, may contribute to the obesity of a few patients. Bulemia is the sudden, compulsive ingestion of very large amounts of food in a very short time, usually followed by agitation, self-condemnation, and often by self-induced vomiting. The night-eating syndrome consists of morning anorexia, evening hyperphagia, and insomnia. Attempts at weight reduction in these 2 conditions are usually unsuccessful and may cause the patient unnecessary distress.)
5. Genetic factors (It is widely recognized that obesity runs in families: 80 % of the offspring of 2 obese parents are obese, compared with 40 % of the children of 1 obese parent and only 10 % of the offsprings of 2 nonobese parents.).
6. Physical activity. (Decreased physical activity in affluent societies is often sited as a major factor in the rise obesity.)
7. Development factors.(The increased adipose tissue mass in obesity can result from either an increase in size of fat cells (hypertrophic obesity), from an increase in the number of fat cells (hyperplastic obesity), or from an increase in both (hypertrophic-hyperplastic obesity). Most persons whose obesity began in adult life suffer from hypertrophic obesity. They lose weight solely by the decrease in the size of their fat cells; the number of fat cells does not change. Persons whose obesity began in childhood are more likely to suffer from hyperplastic obesity, usually of the combined hypertrophic-hyperplastic type. They may have up to 5 times as many fat cells as either persons of normal weight or those suffering from pure hypertrophic obesity. As a result, they may be able to reach a normal body weight only by marked depletion of the lipid content of each fat cell.)
8. Brain damage. (Brain damage, particularly to the hypothalamus, can lead to the obesity.)
Classification by Egorov
Classification due to stages of obesity
A. According to Brock’s index (N: weight = height – 100).
I. Weight excess < 30 %.
II. Weight excess 30 – 50 %.
III. Weight excess 50 – 100 %.
IV. Weight excess > 100 %.
B. According to Kettle’s index or body mass index .
Experts believe that a person's body mass index (BMI) is the most accurate measurement of body fat for children and adults.
Adults with a BMI greater than 30 are considered obese. Adults with a BMI between 25 and 29.9 are considered overweight.
Overweight-27,5 – 29,9
I. 30,0 – 34,9
II. 35,0 – 39,9
III. > 40,0
There are exceptions. For example, an athlete may have a higher BMI but not be overweight .
Classification due to deposition of fat tissue.
- lower type (gluteofemoralis).
- upper type (abdominal): Waist/hip ration
>1,0 in men
> 0.85 in women
>102 cm in men
> 82 cm in women
Both methods identify those with increased CVD risk;
Obese people come to the doctor not only complain about their fitness but also with complications (cardiovascular, pulmonary, orthopedic and others).
Clinical particularities of hypothalamic obesity.
1. Fast gain weight (20 – 30 kg during 1 – 2 years).
2. More frequent dysplastic localization of the fat.
3. The presence of the striae.
4. Symptoms associated with increased intracranial pressure and neurologic picture (somnolence, raised appetite and others).
5. Signs of hypothalamic dysfunction (palpitation, hyperhydrosis, hypertension).
have to be made between different types of obesity.
1. Genetic (family) factor.
2. Eating habits (ingestion of large amounts of food).
3. Slow progressing.
It can occur in the massively obese persons. Pressure on the thorax from the encompassing sheath of the fatty tissue combined with pressure on the diaphragm from below by large intra-abdominal accumulations may lead to reducing of the respiratory capacity, hypoventilation, retention of CO2leading to decreased effects of CO2 as respiratory stimulant and resultant hypoxia and somnolence
Barrakcer – Simmons’s disease (progressing lipodystrophia)
1. More frequent is in young women.
2. Atrophy of the subcutaneous adipose tissue in the region of face neck, thorax; increased quantity of adipose tissue in the lower part of body, thighs, legs (“riding-breeches” type).
3. Duration of the disease, as a rule, without any changes in nervous and endocrine system and patients have only cosmetic defect.
Dercum’s disease (generalized painful lipomatosis).
1. More frequent is in women in menopause.
2. There is localized, painful nodes (knots) in the subcutaneous adipose tissue. These nodes are painful, itch, the skin over nodes is red.
3. Patient can have normal weight or be obese.
4. Person has nervous changes (CNS asthenia, neuroses) and endocrine disturbances (decreasing of function of sexual glands).
Babinsky-Frelych’s disease (adipose-genital dystrophy).
1. More frequent is observed in boys.
2. Characterized by obesity (dysplastic type) and hypogenitalism (development of primary and secondary sexual signs is stopped: small sizes of scrotum, penis, may be criptorchism).
3. There is often lack in growth.
Postnatal neuroendocrine syndrome (PNES) .
1. Increasing of the weight during 3 – 12 months after abortion or labor (Kettle’s index usually is more than 30).
2. Subcutaneous adipose tissue is localized like in patients with Cushing’s syndrome.
3. Striae are present.
4. There is moderate hirsutism, tendency to hypertension and hyperglycemia.
5. Ovary unovulatory hypofunction is present. PNES may lead to endocrine sterility.
Laurence – Moon – Biedl syndrome.
1. Obesity, hypogenitalism like in patients with Babinsky-Frelych’s disease.
2. Decreased mental activity or debility.
3. Pigmental retinitis.
4. Bones or inner organs abnormalities (polydactylia, syndactylia and others)
Morganyi – Stuart – Morel’s syndrome.
1. More frequent in young women or in climacteric female.
2. Adipose tissue localized in the region of chin, abdomen (like apron) mammary glands (mastoptosis), skin is flabby, striae are absent.
3. Hirsutism is present (beard, moustache).
5. Diabetes mellitus.
6. Increased thickness of lamina interna of frontal bone.
The prognosis for obesity is poor, particularly for obese children, and the course tends to progress throughout the life. Obesity is a chronic condition resistant to treatment and prone to relapse. Most obese persons will not participate in outpatient treatment, and those who do will not lose a significant amount of weight. Most of those who do lose weight will regain it. These results are poor, not because of failure to implement any therapy of known effectiveness, but because no simple or generally effective therapy exists. The numerous people who try to reduce without medical assistance, on diets and advice from magazines, may have more success.
The basis of weight reduction in all treatment regimens is to establish a caloric deficit by reducing intake below output.
The simplest way to reduce caloric intake is with a low-calorie diet. Optimal long-term effects are achieved with a balanced diet containing readily available foods. For most people, the best reducing diet consists of their usual foods in amounts limited with the aid of standard tables of food values. Such a diet gives the best chance of long-term maintenance of the weight loss, although it is the most difficult diet to follow during weight reduction. Consequently, many people turn to novel or even bizarre diets, of which there are many. The effectiveness of these diets, if any, results, in large part, from monotony - nearly everyone will tire of almost any food if that is all they get to eat. Consequently, when they stop the diet and return to their usual fare, the incentives to overeat are increased. Fasting has had considerable vogue as a treatment for obesity, but it is now rarely used. Most patients promptly regain most of the weight they lose. Since fasting is not without complications, it should be carried out in a hospital.
Several recommendations. Patient has to:
1) eat 4 – 5 times a day, only in a direct time, not to eat between basic meal receptions;
2) eat only one portion;
3) limit a free liquid to 1,0 – 1,2 l/day;
4) not to eat with the aim of decreasing depression, not to eat “for a company”;
5) the total daily energy intake should be between 1600 – 800 Kcal.
It is frequently recommended in weight reduction regimens and its usefulness has probably been underestimated even by its proponents. Since caloric expenditure in most forms of physical activity is directly proportional to body weight, with the same amount of activity obese persons expend more calories than do those of normal weight.
Physical activity has to be: 1) regular; 2) bring only positive emotions; 3) it is better to work in a group of the patients.
Many preparations (amphetamines, fenfluramine, others) are used as anorectic drugs. Their efficacy and side effects seem comparable and their potential to abuse limited. However, to an even greater degree than after other conservative treatment, weight is regained after drug treatment and the use of appetite suppressants is currently out of favor.
We have to use medications in patients with endocrine and cerebral pathology: anti-inflammatory drugs (to treat encephalitis, arachnoiditis), bromcreptin, peritol (to treat hypothalamic and pituitary disorders) and others.
Physiotherapy. Massage, automassage, circulating shower-massage are very effective in the treatment of the patients.
Radical surgical treatment may offer some hope to persons with morbid obesity (100 % overweight) in whom all others treatments have failed.
For the dangerously overweight, it’s the only way to lose pounds and keep them off.
Morbid obesity leads to hypertension, type 2 diabetes, obstructive sleep apnea, depression, incontinence, arthritis and other significant health problems.
Worried about their health and tired of being socially isolated, some morbidly obese individuals turn to bariatric surgery. "Obesity is a complex problem, and successful treatment is based on a healthy diet, counseling, increased physical activity and, when indicated, surgery," says Raul J. Rosenthal, M.D., one of three bariatric surgeons at Cleveland Clinic Florida.
Limiting intake or altering digestion.
The are two types of bariatric surgery, both of which are effective for significant, long-term weight loss. Gastric (stomach) restrictive procedures limit food intake by decreasing the size of the stomach so that the sensation of "fullness" (or pain) occurs after a small amount of food is ingested. Malabsorptive procedures alter the digestion process, thus causing food to be poorly digested and incompletely absorbed. The excess is eliminated in the stool.
"Most patients who choose bariatric surgery have tried all kinds of diets with little success, and this is their last option. If they don’t have the surgery, they have a 50 to 100 percent chance of dying prematurely from obesity-related problems," says Dr. Rosenthal. "Our goal is to help patients live longer."
Getting around the stomach.
Several types of bariatric surgery have been developed, but the two favored by Dr. Rosenthal and his colleagues are adjustable gastric banding (also called gastric banding), a restrictive procedure, and proximal roux-en-y gastric bypass, a malabsorptive procedure that involves bypassing a large portion of the stomach.
Gastric banding involves no stapling or cutting of the stomach; rather an implantable hollow band is used to isolate a small portion of the upper stomach to make a pouch that serves as a "new" stomach. The pouch fills quickly with solid food and empties slowly to relieve hunger and produce a feeling of fullness. Overeating results in pain or vomiting.
The roux-en-y gastric bypass procedure is an alternative means of achieving malabsorption, and is the most commonly performed and successful malabsorptive surgery. It also involves creating a separate gastric pouch while leaving the rest of the stomach in place. Unlike bypass procedures that involve removal of the stomach, the roux-en-y approach poses fewer operative risks.
New approaches help reduce pain, shorten recuperation.
Traditional or "open" bariatric surgery requires a 6- to 8-inch incision and approximately four weeks of recuperation. Using minimally invasive surgical techniques in select patients, however, Dr. Rosenthal and colleagues can perform restrictive or malabsorptive bariatric procedures without opening the abdomen. The minimally invasive approach achieves results comparable to those associated with open surgery, but with less post-operative pain and swifter recovery. Patients who undergo minimally invasive bariatric surgery can return to work one week after undergoing gastric banding and two weeks after gastric bypass.
Malabsorptive bypass procedures such as roux-en-y bypass produce more weight loss than restrictive operations and are more effective in reversing the health problems associated with severe obesity. Patients who have malabsorptive operations generally lose two-thirds of their excess weight within 2 years. According to Dr. Rosenthal, gastric bypass procedures account for 90 percent of the bariatric operations performed today. "It has been done for 40 years, so it is well understood. Doing them laparoscopically, however, is rather new," he says.
Patients who undergo a gastric bypass feel full after eating about two tablespoons of food. Due to some malabsorption, nutritional supplements are necessary. In the three months following surgery, patients can expect to lose about 60 pounds. The weight loss generally continues for 1½ years before stabilizing.
Patients who undergo gastric banding can lose up to 60 pounds or more in one year. The duration of weight loss and number of pounds lost, however, varies by individual. Both gastric banding and bypass can help resolve underlying health problems common in the morbidly obese. For instance, they have been shown to cure type 2 diabetes and sleep apnea (a sleep and breathing disorder) and improve high blood pressure. "Only three weeks after surgery, most patients can stop taking medications for these problems," says Dr. Rosenthal. "More important, these patients experience a significant decrease in risk of premature death and a marked improvement in quality of life."
Risks are significant, but rare.
In gastric banding, complications include kinking of the stomach, leaking from the site of banding or band breakage, all of which require surgical intervention to resolve. Side effects of gastric bypass surgery include internal bleeding, pulmonary embolism and unidentified leakage. Such serious side effects, however, are rare, says Dr. Rosenthal; and the risk can be further reduced by seeking care from an experienced bariatric surgeon who offers a comprehensive program of care. "The surgeon should offer safe, proven procedures, a thorough preoperative workup, and a postoperative program consisting of nutritional consultation, support groups and mental health counseling. This helps ensure an optimal outcome," says Dr. Rosenthal.
Roux-en-Y stomach surgery for weight loss
The Roux-en-Y gastric bypass procedure involves creating a stomach pouch out of a small portion of the stomach and attaching it directly to the small intestine, bypassing a large part of the stomach and duodenum. Not only is the stomach pouch too small to hold large amounts of food, but by skipping the duodenum, fat absorption is substantially reduced.
Restrictive gastric operations, such as an adjustable gastric banding procedure, serve only to restrict and decrease food intake and do not interfere with the normal digestive process.
In this procedure, a hollow band made of special material is placed around the stomach near its upper end, creating the small pouch and a narrow passage into the larger remaining portion of the stomach. This small passage delays the emptying of food from the pouch and causes a feeling of fullness.
The band can be tightened or loosened over time to change the size of the passage. Initially, the pouch holds about 1 ounce of food and later expands to 2-3 ounces.
Adjustable gastric banding
Vertical banded gastroplasty
Restrictive gastric operations, such as vertical banded gastroplasty (VGB), serve only to restrict and decrease food intake and do not interfere with the normal digestive process.
In this procedure the upper stomach near the esophagus is stapled vertically to create a small pouch along the inner curve of the stomach. The outlet from the pouch to the rest of the stomach is restricted by a band made of special material. The band delays the emptying of food from the pouch, causing a feeling of fullness.
Biliopancreatic diversion (BPD )
Malabsorptive operations, such as biliopancreatic diversion (BPD), restrict both food intake and the amount of calories and nutrients the body absorbs.
In a BPD procedure, portions of the stomach are removed. The small pouch that remains is connected directly to the final segment of the small intestine, completely bypassing the upper part of the small intestines. A common channel remains in which bile and pancreatic digestive juices mix prior to entering the colon. Weight loss occurs since most of the calories and nutrients are routed into the colon where they are not absorbed.
1. The Merck Manual of Diagnosis and Therapy (fourteenth Edition)/ Robert Berkow and others. – published by Merck Sharp & Dohme Research Laboratories, 1982. – P. 987 – 992, 916 – 921. 990 – 997. 1032 – 1037, 1680 – 1681.
2. Endocrinology (A Logical Approach for Clinicians (Second Edition)). William Jubiz.-New York: WC Graw-Hill Book, 1985. - P. 21 – 33. 34 – 38,52 – 63. 416 - 497.
3. Manual of Endocrinology and Metabolism (Second Edition)/ Norman Lavin. – Little, Brown and Company.- Boston-New York-Toronto-London, 1994. - P. 55 – 56. 179 - 274.
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