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Hermaphroditism

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Hermaphroditism is a very rare condition in which the person has both ovarian and testicular tissue occurring together. The appearance of the external genitals varies greatly, and the persons have a problem for life. With more recent methods of studying chromosomal anomalies today, many have been found to have aberrations. In some it seems there has been a double fertilization of the ovum by two sperms, one giving female attributes and the other male ones, so producing a final ambiguous picture.

Hermaphroditism Treatment

This depends on how the child was originally brought up, and there is considerable feeling that whatever this has been should be continued. In recent years this has been an emotive topic.

Many teenage girls may fail to menstruate, or normal periods may suddenly cease. This may be due to excessive hormonal production preventing normal monthly ovulation, as well as obsessively over exercising. It may affect one in 20 among young ballet dancers and athletes.

Fortunately, when the vigorous sports are reduced, in most cases ovulation (and normal menstrual periods and the chances of pregnancy) returns to normal levels. This may be a worrying time for many young people. If continuing indefinitely (as with estrogen lack in the blood), calcium may be drawn from the bones, causing osteoporosis, with the high risk of fractures common in older postmenopausal women. It needs careful evaluation.

Two related conditions called female and male pseudohermaphroditism show that genetically a person with a female type chromosomal structure has varying degrees of masculinisation, and vice versa. In women, corticosteroids are used. In males, as there is a high risk of cancer developing in the testes, they may be removed, and plastic surgery carried out (with appropriate prosthetics). Testosterone is given to increase maleness.

Chronic Leukemia

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What is Chronic Leukemia?

This is also known as chronic granulocytic anaemia, chronic myeloid leukaemia or chronic myelocytic leukemia. These names are mentioned, for a patient with the disorder may have heard of these various terms.

It means that there is a malignant change in the bone marrow involving the granulocyte type of white blood cells.

The disease is rare in children and the very old. Most cases occur in those in the 30-60-years age group, and the sexes are affected equally. There are certain known causes. As with acute leukaemia, these include exposure to nuclear explosions, and in patients receiving radiotherapy for other disorders (most commonly for ankylosing spondylitis, a chronic painful and crippling back disorder). It is also seen in patients who were X-rayed repeatedly as a check for artificial pneumothorax, which was widely used for treatment of certain chest diseases in the past.

Chronic Leukemia Symptoms

The most common symptoms include fatigue and lassitude, feeling listless and weak, “off-color,” and abdominal swelling or frank pain due to enlargement of the spleen. There may be loss of weight, the symptoms of anaemia (see anaemia) or purpura (bruising).

Sometimes the disease is found by chance when a blood test is being carried out for some other purpose. Almost always there is enlargement of the spleen and this may be massive. Sometimes the lymph glands may be involved; this is not a good sign. Sometimes the skin and bones may be involved as well. The blood picture usually confirms the diagnosis and white-cell counts of 100 or more are common. (The normal figure is between 4.0 and 11.0 x 10 9/L.) All forms of developing white cells are seen, from the very immature to the well-developed. Often the platelet count is also raised.

Chronic Leukemia Treatment

At present the medication of choice is one called busulphan, given orally. Gradually the white-cell count drops. When this reaches the relatively normal figure of 10.0, maintenance doses are given.

Gradually this may be replaced with other forms of drug therapy, similar to those used for acute leukaemia. Busulphan has virtually replaced other forms of treatment that were popular in the past, including radiotherapy and other forms of chemotherapy.

A wide range of drugs has been used with varying degrees of success in leukaemia. Long-term, most fail, for the disease is often a fatal one. Many drugs, which appear to be promising at first, later prove to be less effective. Marrow transplants have been tried, and these also have proved successful in some patients, but often relapses have taken place as time advances. Patients with this disease will be under the care of experts in the field, and ideally attached to major public hospitals where the full range of facilities, at that time, will be made available. In short, despite our advances with modern drugs, technology, transplants and success in the field of acute cases, the outlook for older persons with leukaemia is still not good. Other aspects of the disease must be treated as supportive measures.

Antibiotics will be given to check any infection. Anaemia will be treated, and may require repeated blood transfusions. Platelets may be needed for the clotting abnormalities. With the early stages of treatment there is often a rise in the level of uric acid in the blood, and the drug al-allopurinol is often ordered. Otherwise, bouts of acute gout may complicate the picture as well as making life one of abject misery for the unfortunate patient.

Therapy has vastly improved the quality of life for these patients, but, sad to relate, it has not materially augmented the total life span. The disease is inevitably fatal. However, it is now possible for many to live relatively symptom-free lives for the two to three years that usually occur from the time of diagnosis to the time of death.

Hemolytic Anemia

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What is Hemolytic Anemia?

This type of anemia is due to a premature destruction of the red cells. Generally speaking it is fairly uncommon, and as care in the use of drugs looking out for situations in which it is likely to occur (such as Rh incompatibilities), and taking the necessary precautions increases, the risks are now far less than they were a few years ago. Apart from some of the usual symptoms of anemia (sometimes a feature. sometimes not, depending on the degree of anemia present), other typical symptoms are associated with it.

Hemolytic Anemia Symptoms

Jaundice (yellowing of the skin and whites of the eyes) can occur. This is due to the excessive breakdown of the red cells, and the production in large amounts of a chemical called bilirubin. The urine and feces may become severely pigmented also, as large amounts of bilirubin are excreted through these systems.

The blood picture (when examined under the microscope) shows typical changes. The bone marrow makes increasing efforts at stepping up red cell production to cope with the rapid rate of cell destruction. For this reason, red cells that have not matured properly are pumped into the general circulation.

These immature cells appear in profusion. The level of actual anemia depends on the body’s ability at balancing the rate of cell destruction with the rate of new cells being made available. In this way, the symptoms of typical red cell anemia will vary from case to case.

Also, abnormal by-products of red cell destruction are usually found in the urine. There is an extensive battery of tests available to help doctors decide which type of anemia is present, and to help pinpoint the probable cause. Most of the others are rarely seen by doctors in the ordinary routine of practice, and the majority is only diagnosed upon investigation in a large hospital equipped to deal with the full range of investigations.

From the practical point of view, it is essential that any of the telltale symptoms mentioned receive prompt medical attention. Your doctor will very quickly have you referred to the appropriate centers for total assessment and treatment. Treatment of these disorders has no place in home medicine, and trying simple home remedies and following the advice of well-meaning relatives and friends is a total waste of time and could be harmful in precluding vital medical attention.

Jaundice, an important symptom in this type of anemia, warrants immediate medical advice from a doctor.

Some of the more probable types of hemolytic anemia include:

Hemolytic Disease of the Newborn

The most likely situation in which this may occur is when an Rh-negative mother produces an Rh-positive infant, and the cells from the baby stimulate the mother to form anti-Rh (usually anti-D) antibodies.

These antibodies can then cross via the placenta and become attached to the baby’s red cells, causing their destruction (hemolysis).

Generally there is a history of a previous pregnancy or miscarriage in the mother, and during the birth of the first baby (who usually comes through unscathed) the release of fetal cells into the mother’s circulation takes place, and sets up the antibody production that will affect later babies.

Each subsequent baby will tend to be affected more severely It may be lethal to the baby, and a condition called hydrops foctolis can develop unless immediate steps are taken soon after birth. Formerly the only method of treatment was to give a prompt exchange transfusion to the baby. In this way, the diseased blood was removed, new blood replacing it completely.

It was a time-consuming and arduous undertaking and a marathon event for a newborn infant. However many lives were saved in this manner. In the late 1960s it was found that if the mother were given a special single injection of anti-D antibody within 7 2 hours of the birth of her Rh-positive infant, this effectively stopped production of the antibodies, and the risk to subsequent babies was greatly reduced.

As a new generation of mothers is growing up, and with routine blood tests being carried out before and at the time of birth (on the mother and infant), treatment is now effectively cutting back on this form of anemia. In time it will most likely disappear altogether.

However, a miscarriage, abortion or blood transfusion (with Rh-positive blood in an Rh-negative woman) may lead to similar complications later on in pregnancy.

Incompatible Blood Transfusion

The most obvious example of hemolytic anemia occurs when a patient is given the wrong blood during a transfusion. This is termed “incompatible” blood. In 1900 Karl Landsteiner showed that there were four main blood groups that could destroy incompatible red cells. For example, if a patient of group B is given group A blood, the group A cells will be destroyed by the anti-A in the recipient’s plasma. That is why great care is taken to type and cross-match blood before every blood transfusion. Only group 0 blood may be given in a dire emergency without cross-matching with relative safety, for it contains no antigens.

Symptoms that may occur when the wrong blood is given include acute hemolysis, the appearance of hemoglobin in the urine, fever and severe back pain, and frequently renal disorders. Most of the mistakes that occur causing this reaction have been found due to administrative errors at the hospital (wrong labels on bottles, failure to check labels correctly etc).

Hemolytic Anemia Due to Drugs and Chemicals

Certain drugs are well-known for their ability at reacting on the red cells and causing their premature destruction. This may occur almost at once, or in others it may occur about 10 days after administration of the drug, the first dose apparently sensitizing the system and later doses having an immune type reaction. But the result is the same, irrespective of the cause—red cells disintegrating and possibly causing a medical emergency.

Other Causes

A variety of other causes have been incriminated. Certain bacterial infections appear capable of producing bone-marrow depression and red-cell destruction.

Mechanical trauma of the red cells can cause their premature breakdown in others. Apparently healthy young men doing a lot of marching or running particularly on hard surfaces for prolonged periods of time, may damage the red cells in the blood circulating in their feet.

Hemoglobin is later passed in the urine a frightening experience. It is harmless and wearing rubber insoles in the boots should correct the problem.

Burns may have a similar effect, the red cells becoming directly damaged or else suffering damage as they flow through injured vessels. They subsequently tend to fragment and hemolyse. In these modern times, when Teflon is being used in cardiac surgery unless the prosthesis is completely covered with normal cells, red cell damage can occur similarly leading to cell destruction.

Other prosthetics can cause similar cell damage, a problem of modern surgery limited to the second half of the 20th century. It will probably increase as does the rate of surgery of this nature.

Enlarged Spleen

The spleen is an integral part of the reticulo-endothelial system of the body apparatus that deals with red cells once their useful life has come to an end. They are disposed of quietly, and their place taken over by the ever-proliferating number of new red cells produced in bone marrow.

However, if there is splenic enlargement from any reason, the organ may overreact and increase in its rate of destroying the red cells.

Many disorders can lead to splenic enlargement, even simple viral infections such as a glandular fever or viral hepatitis or any other mild viral infection. But some of the infections and conditions producing an enlarged spleen are rarer and more bizarre.

If it can be shown that normal red-cell production is taking place in the marrow and that destruction in the spleen is excessive, its removal could be the choice of treatment.

Acne Vulgaris

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What is Acne Vulgaris?

This very common skin inflammation affects chiefly adolescents of both sexes. Indeed, it is almost part of “growing up,” and often commences soon after the hormonal changes of puberty. It is usually at maximum intensity in the teen years, and frequently persists into the 20s and occasionally beyond. Often there is a family history of acne.

The tiny sebaceous glands in the skin do not function during childhood. But later, under hormonal stimulation, they commence to produce sebum, a whitish fluid discharged to the skin surface to spread evenly over it throughout life.

Sebaceous production frequently gets out of hand with a heaping up of sebum at the skin surface. A Whitehead is formed. The material oxidises and turns black and a blackhead or comedone occurs.

Often these discharge simply onto the skin (with a little pressure, or spontaneously). But some remain “closed” and, indeed, as further sebum is added from beneath, increase in size, creating a pustule. If this increases in size before discharge, a large, often painful, cyst is formed (see Sebaceous Cysts). This may penetrate into the deeper tissues of the skin.

Often the walls of the cyst break down the sebaceous of skin overproduce, causing small pustules whiteheads and local inflammation internally, and the highly irritating contents invade surrounding structures. This increases the irritation and discomfort.

It is easy for germs to invade, and uncomfortable and very tender swellings very much like boils occur. A major problem with acne is the tendency for the pustules and cysts to empty out, leaving unattractive scars and depressions. Acne usually occurs on the face, but it may extend to the neck, shoulders and back as well.

A germ called the Corynebacterium is often present in acne lesions. However, this is not contagious, and is not harmful. Doctors talk about acne in various grades according to its severity.

Grade I is mainly on the face and consists of comedones and an occasional small pustule. Grade II is characterised by an increase in the number of pustules, but they are chiefly on the face region. Grade III is composed chiefly of many small pustules, which have spread from the face to the shoulders, back and chest. Grade IV is a very advanced state that is even more extensive, and many penetrate down to the waist. Large cysts and painful swellings are common. This is commonly called Acne conglobata. Acne sometimes occurs suddenly in persons living in tropical climates.

Acne Vulgaris Treatment

Many treatments have been advocated over the years, which in itself is testimony to the fact that no universally satisfactory routine exists at present. However, simple home routines are worth trying before recourse to a doctor for professional advice. It must be remembered that the course of acne normally waxes and wanes so that an apparent improvement with a certain remedy does not necessarily testify to its efficacy in the long-term.

Girls may notice pimples are much worse in the week just prior to a menstrual period. Progesterone, the hormone being formed in the body, is often present at high levels and seems to aggravate it all. It is unfortunate. I might add that the less you think about your pimples, the better it is. Often problems from pimples are more in the mind than on the face. Flour and sugar-containing products and fatty foods rarely help, and could aggravate what may be a trying and persisting condition.

The simplest starting point is to wash the face, and all affected parts (it may extend to the shoulders, chest and back) with soap and hot water. Many blackheads can then be gently squeezed or eliminated with a “comedone extractor” (available from the pharmacist). Some say that squeezing is a bad idea, for it may spread infection. But when the face is hot they will often pop readily, eliminating the fatty material.

After this a lotion or cream may be applied. There are many different varieties, some containing sulphur and smelling vile. Salicylic acid and tar products, and sometimes corticosteroids are useful. A satisfactory application is benzoyl peroxide gel 5 per cent (Panoxyl Acne Gel).

This is rubbed in once or twice a day (probably best at bedtime) and is supposed to take oxygen to the cells below the surface and help clear the condition. Another product claimed to help is tretinoin, either in the form of a liquid or gel (Retin-A). This is a derivative of vitamin A, long believed to help acne, and the local application can often assist. Follow the directions on the packing slip, and keep out of bright sunshine, otherwise the skin becomes very red.

If acne is a persisting problem at puberty; it may need treatment by a skin specialist. If simple local measures are ineffective, it’s worth seeking medical advice from the doctor. Often the tetracycline antibiotics are used for adults. This may continue for many months or a year or even more. This seems to reduce sebum formation. The capsules must not be taken with food. Take three times daily between meals. A zinc tablet taken daily sometimes helps persisting cases. Occasionally a form of the oral contraceptive hormonal tablet is also effective.

Roaccutane is prescribed by skin specialists for severe cystic acne, but there are special precautions.

Here are some of the suggestions for a start:

  • Simple cleanliness. Wash the face and all affected parts with soap and hot water at least twice a day. Wash thoroughly, especially in the areas where grease tends to accumulate (in crevices such as thenasal folds). Ordinary soap is quite adequate. Medicated soaps containing hexachlorophene preparations may worsen the condition. Avoid greasy cleansing creams and other cosmetics.
  • Shampoo the scalp once or twice a week, especially if there is any sign of dandruff which indicates overactivity of the sebaceous glands. Selenium sulphide or a similar preparation is recommended; otherwise use an ordinary detergent based shampoo.
  • Gently squeeze out any blackheads. Some prefer a comedone extractor, a metal object with a hole in the centre. When the extractor is placed over the blackhead and pressure applied, the blackhead pops out into the hole in the instrument and leaves the skin surface if the blackhead is resistant to pressure, hot applications for a few minutes may reduce the resistance. Do not exert undue pressure.
  • Dab dry with a soft towel then apply a lotion. This is aimed at increasing the blood supply to the skin, and helping the upper skin layers to peel off gradually and so remove the acne lesions. Simple hot water or Epsom salt compresses are often used, or a sulphur-zinc lotion may be applied. Lots of different prescriptions are available.
  • This one has been used for many years, and often helps:
    • Sulphur (Ppt.)
    • Zinc sulphate
    • Sodium borate
    • Zinc oxide
    • Acetone: .30 parts
    • Camphor water, equal parts, made up to Rose water 120 parts

    This lotion is applied each night after the cleansing routine.

  • Many commercial preparations are readily available, and most of these are satisfactory for this same purpose. These include Eskamel and Acnederm. These may be applied at bedtime and removed in the morning.
  • Vitamin A tablets (50,000 in with each meal). This treatment is still claimed by some physicians to be of value. Others doubt it. It may be continued for three months.
  • Diet. A sensible, well-balanced diet is recommended. However, avoidance of chocolate in all forms seems to help a significant number of acne sufferers. (This includes chocolates as such, chocolate containing beverages such as cocoa and similar drinks and milkshakes, and cola drinks.) It’s wise to go lightly on excess carbohydrate foods, nuts, fried and fatty foods and alcoholic beverages. Some doctors suggest removing a certain set of foods from the diet for three weeks and awaiting results, then reintroducing them one by one to see if in fact they play a significant role in producing the disorder. (If so, it will be indicated by an upsurge of pimples when the offending items are reintroduced into the diet.) General measures. It is worthwhile correcting any basic physical derangements.
  • Correct indigestion; treat any underlying infection, malnutrition, anaemia or constipation. Emotional upsets should be avoided, and tensions and anxieties kept to a minimum.

If these simple measures fail to produce an improvement, it’s time to visit your family physician. There are several other courses of action available. They include the following:

  • Antibiotics. The broad-spectrum antibiotics (such as the tetracyclines and erythromycin) exert a beneficial effect on the lesions of acne for reasons not well understood. Often the severe forms of acne respond best. Dose given is variable but often a larger dose commences the routine, e.g. Tetracycline 1 or 2 x 250 mg capsules are given three times a day. It is essential that these are not taken with food, which destroys their action. Therefore they should be taken midmorning, midafternoon and at bedtime. Sometimes dose is increased from one, to two, and later to three capsules three times a day. It may be necessary to continue with the routine for six, nine, twelve months or even longer during the turbulent teen years. Results are almost always satisfactory. Side effects are rare.

    If one antibiotic does not produce the desired results, a switch to another type often will. Clindamycin is excellent, but may cause diarrhoea in some.

  • Oral contraceptives. The pill, which is merely the combination of two normally occurring hormones, often has a beneficial effect on acne, which in women is often worse just prior to menstruation. A pill low in progesterone is usually of greatest advantage. Other doctors sometimes find that low doses of stilboestrol for 10 days following a woman’s menstrual period give good results.
  • Injections of fluorinated steroids into very severe lesions are sometimes carried out by the doctor.
  • Oxygen-producing gels and lotions to be applied at night are often successful (e.g. Panoxyl Acne Gel or Lotion).
  • Retin-A lotion (a vitamin A preparation) applied to the affected spots is often successful. The directions must be carefully followed.
  • Local applications containing steroids and antibiotics in an emulsion base are often satisfactory in addition to these measures.
  • Often simple exposure to sunlight gives beneficial results. Ultraviolet light can also assist. In severe cases, X-radiation by a skin specialist can give excellent results when all other methods fail, but this is now rarely used.

Acne Vulgaris Scarring

A major problem with acne is the multiple scarring that causes embarrassment. Effective and vigorous treatment during the course of the disease, however, will often obviate major scarring.

  • Dermabrasion (the removal of the scar with a rapidly turning wire brush) often gave excellent results. However, as many patients were dissatisfied with the results because excessive pigmentation sometimes occurred at the margins, this has now fallen from favour. Some specialists apply carbon dioxide “slush” at fortnightly intervals. Others recommend the use of abrasive substances that are applied manually then washed off (e.g. Brasivol). Deep scars may require the attention of a plastic surgeon. However, the scar is often more in the mind of the patient than on the face, and careful thought should be given before such heroic ventures are embarked upon. They are expensive, too, despite national insurance schemes. Cosmetic surgery was never cheap.
  • Injection therapy. The use of collagen given by injection under the scar will often eliminate the depression. It is expensive therapy, usually carried out by the plastic surgeon or specially trained skin specialist. It is not a “forever cure,” but lasts anywhere from one to three years, when it may have to be repeated for continuity of appearance.
  • Severe pustular acne. The oral use of a powerful drug called isotretinoin (Roaccutance is the commercial name) may often have a dramatic beneficial effect in clearing severe acne with cysts and pus-affected, fluid-filled areas under the skin and which have not responded to any other treatment. It must be given under the supervision of a skin specialist. It is essential the person is not pregnant, for it is well-known to cause birth defects.

Otherwise, results are often dramatic. It is expensive, but this is often a small price to pay for a brand-new appearance.

Skin Disorders Related to Acne Vulgaris

  • Rosacea. This is a permanent flushing in the region of the forehead, nose and cheeks, and is common in people 20 years and over. Pustules may form and the skin over the nose often thickens, and minute superficial veins appear. Refer to section on Rosacea.

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