What is this Condition?

Muscular dystrophy is actually a group of inherited disorders characterized by a progressive, symmetrical wasting away of skeletal muscles that occurs without causing pain or loss of feeling in the limbs. Paradoxically, these damaged muscles tend to enlarge because of connective tissue and fat deposition, giving a false impression of muscle strength.

So far, there is no cure for the condition, but there are four main types of muscular dystrophy, each with a different outcome. Duchenne type muscular dystrophy accounts for 50% of all cases. It generally strikes during early childhood and results in death by age 20. Becker’s muscular dystrophy is slower to develop, and people with it usually live into their 40s. The two last types (dystrophy of the face, shoulders, and arms and limb-girdle dystrophy) usually don’t shorten life expectancy.

What Causes it?

Muscular dystrophy is caused by various genes. The Duchenne and Becker types are carried by sex-linked genes affecting only men. Dystrophy of the face, shoulders, and arms and limb-girdle dystrophy are not sex-linked, so affect both sexes about equally.

What are its Symptoms?

Each type of muscular dystrophy causes progressive muscle deterioration, but incidence and severity varies in the following ways:

• The Duchenne type begins subtly, between ages 3 and 5. Children with this disorder have a waddling gait and difficulty climbing stairs, fall down often, can’t run properly, and their shoulder bones flare out (or “wing”) when they raise their arms.

Usually, a child with muscular dystrophy is confined to a wheel­chair by ages 9 to 12. Finally, progressive weakening of the heart muscle leads to death from sudden heart failure, respiratory failure, or infection.

• Although similar to the Duchenne type, the symptoms of Becker’s muscular dystrophy progress more slowly. Symptoms start around age 5, but children can still walk well beyond age 15 - sometimes into their 40s.

• Dystrophy of the face, shoulders, and arms is a slowly progressive and relatively harmless type that commonly occurs before age 10 but may develop during early adolescence. In the early stages, infants may not be able to suckle; children cant pucker their mouths or whistle or raise their arms above their heads. Affected children may have abnormal facial movements and a lack of facial movements when laughing or crying .

• Limb-girdle dystrophy follows a similarly slow course and often causes only slight disability. Usually, it begins between ages 6 and 10, causing muscle weakness first in the upper arm and pelvic muscles; then, the other common symptoms appear.

How is it Diagnosed?

The doctor examines the child, asks questions of the family, and orders certain tests. If another family member has muscular dystrophy, that persons symptoms usually tell a lot about the child’s future. If no one else has the disorder, tests of nerve activity in the affected muscles can reveal muscular dystrophy, and a muscle biopsy can show cell changes or cell fat and tissue deposits.

Medical centers with the most advanced immunologic and molecular biological techniques can accurately predict muscular dystrophy in the fetus. They can also test parents and relatives who may carry the genes for Duchenne and Becker’s muscular dystrophies.

How is it Treated?

No treatment has yet been found that can stop the progressive muscle deterioration of muscular dystrophy. However, orthopedic appliances, as well as exercise, physical therapy, and surgery to correct contractures, can help preserve the child or young adult’s mobility and independence for a time.

Family members who are carriers of muscular dystrophy should receive genetic counseling regarding the risk of transmitting this disease to their children.

What are these Conditions?

Folliculitis is a bacterial infection of the hair follicle that causes formation of a pustule - a collection of pus beneath the outer skin layer. The infection can be superficial or deep.

Folliculitis may also lead to the development of furuncles (furunculosis), commonly known as boils, or carbuncles (carbunculosis). The prognosis depends on the severity of the infection and on the person’s physical condition and ability to resist infection.

What Causes them?

The most common cause of folliculitis, boils, or carbunculosis is a bacterium called Staphylococcus aureus. Factors that increase a person’s risk of developing these conditions include an infected wound, poor hygiene, debilitation, diabetes, cosmetics that clog pores, tight clothes, friction, exposure to chemicals, and treatment of skin lesions with tar or with occlusive therapy, using steroids. Boils often follow folliculitis that’s exacerbated by irritation, pressure, friction, or perspiration. Persistent infection and boils may lead to carbunculosis.

What are their Symptoms?

Pustules of folliculitis usually appear on the scalp, arms, and legs in children; on the face of bearded men; and on the eyelids. Deep folliculitis may be painful.

Folliculitis may progress to hard, painful boils, which commonly develop on the neck, face, underarms, and buttocks. These boils enlarge for several days and then rupture, discharging pus and necrotic material. After they rupture, pain subsides, but redness and swelling may persist for days or weeks.

Carbunculosis is marked by extremely painful, deep abscesses that drain through many openings onto the skin surface, usually around several hair follicles. Fever and malaise may accompany these lesions.

How are they Diagnosed?

The presence of characteristic skin lesions confirms folliculitis, boils, or carbunculosis. A wound culture performed in a lab reveals the infecting organism. In carbunculosis, the person’s history reveals preexistent boils. A complete blood count may show an elevated number of white blood cells.

How are they Treated?

Treatment of folliculitis consists of cleaning the infected area thoroughly with soap and water; applying warm, wet compresses to promote drainage from the lesions; topical antibiotics, such as Bactroban ointment; and in extensive infection, systemic antibiotics (E-Mycin or Dynapen). Boils may also require incision and drainage of ripe lesions after application of warm, wet compresses, and topical antibiotics after drainage. Carbunculosis is treated with systemic antibiotics.

What is this Condition?

Pregnancy places special demands on carbohydrate metabolism and increases the body’s insulin requirement, even in a healthy mother. Thus, she may become prediabetic, or, if she’s diabetic, runs the risk of aggravating her preexisting condition.

The prognosis of the mother and fetus is good if the mother’s blood glucose (sugar) level is well controlled and ketosis and other complications are prevented. Infant morbidity and mortality depend on recognizing and successfully controlling low blood sugar, which may develop within hours after delivery.

What Causes it?

In diabetes, glucose is inadequately used by the body, either because insulin is not synthesized by the pancreas (as in insulin-dependent diabetes) or because body tissues resist the hormonal action of endogenous insulin (as in non-insulin-dependent diabetes). During pregnancy, the fetus relies on the mother’s glucose as a primary fuel source, but pregnancy triggers protective mechanisms that have anti-insulin effects: increased hormone production (placental lactogen, estrogen, and progesterone), which counteracts the effects of insulin; degradation of insulin by the placenta; and prolonged elevation of stress hormones (cortisol, epinephrine, and glucagon), which raises blood sugar levels.

In a normal pregnancy, an increase in anti-insulin factors is met by increased insulin production to maintain normal blood sugar levels. However, prediabetic or diabetic women can’t produce enough insulin to overcome the insulin antagonist mechanisms of pregnancy, or their tissues are insulin-resistant. As insulin requirements rise toward term, a prediabetic woman may develop gestational diabetes, requiring dietary management and, possibly, administration of insulin to achieve glucose control; an insulin-dependent woman may need to increase her insulin dosage.

What are the Symptoms?

Indications for diagnostic screening for maternal diabetes during pregnancy include obesity, excessive weight gain, excessive hunger or thirst, excessive urination, recurrent monilial infections, glucose in the urine, previous delivery of a large infant, excessive amniotic fluid, maternal high blood pressure, and a family history of diabetes.

Uncontrolled diabetes in a pregnant woman can cause stillbirth, fetal anomalies, premature delivery, and birth of an infant who is large or small for gestational age. Such infants are predisposed to severe episodes of low blood sugar shortly after birth and may also develop calcium deficiency, high levels of bilirubin in the blood, and respiratory distress syndrome.

How is it Diagnosed?

The prevalence of gestational diabetes makes careful screening for high blood sugar appropriate in all pregnancies in each trimester. Abnormal blood sugar levels measured in a fasting person or after she has eaten, signs and symptoms, and the person’s history suggest diabetes in women not previously diabetic.

A 3-hour glucose tolerance test confirms diabetes when two or more values are above normal.

Procedures to assess fetal status include stress and nonstress tests, ultrasound to determine fetal age and growth, and measurement of urine hormone levels.

How is it Treated?

Treatment of both newly diagnosed and established diabetes is designed to maintain the woman’s blood sugar levels within acceptable limits through dietary management and insulin administration. Most pregnant women with overt diabetes require hospitalization at the beginning of pregnancy to assess physical status, to check for heart and kidney disease, and to regulate diabetes.

Generally, the optimal time for delivery is between 37 and 39 weeks’ gestation. An insulin-dependent diabetic woman requires hospitalization before delivery because bed rest promotes optimal circulation to the fetus and improves uterine muscle tone. In addition, hospitalization permits frequent monitoring of blood sugar levels and prompt intervention if complications develop.

Depending on fetal status and maternal history, the obstetrician may induce labor or perform a cesarean delivery. During labor and delivery, the woman with diabetes will receive a continuous intravenous infusion of dextrose with regular insulin in water. Maternal and fetal status must be monitored closely throughout labor. The woman may benefit from half her prepregnancy dosage of insulin before a cesarean delivery. Her insulin requirement will fall markedly after delivery.