Cystic fibrosis (CF) affects both males and females and people from all racial and ethnic groups. However, the disease is most common among Caucasians of Northern European descent. CF also is common among Latinos and American Indians, especially the Pueblo and Zuni. The disease is less common among African Americans and Asian Americans. More than 10 million Americans are carriers of a faulty CF gene. Many of them don't know that they're CF carriers. The signs and symptoms of cystic fibrosis (CF) vary from person to person and over time. Sometimes you'll have few symptoms. Other times, your symptoms may become more severe. One of the first signs of CF that parents may notice is that their baby's skin tastes salty when kissed, or the baby doesn't pass stool when first born. Most of the other signs and symptoms of CF happen later. They're related to how CF affects the respiratory, digestive, or reproductive systems of the body. Figure A shows the organs that cystic fibrosis can affect.

Figure B shows a cross-section of a normal airway. Figure C shows an airway with cystic fibrosis. The widened airway is blocked by thick, sticky mucus that contains blood and bacteria. People who have CF have thick, sticky mucus that builds up in their airways. This buildup of mucus makes it easier for bacteria to grow and cause infections. Infections can block the airways and cause frequent coughing that brings up thick sputum (spit) or mucus that's sometimes bloody. People who have CF tend to have lung infections caused by unusual germs that don't respond to standard antibiotics. For example, lung infections caused by bacteria called mucoid Pseudomonas are much more common in people who have CF than in those who don't. An infection caused by these bacteria may be a sign of CF. People who have CF have frequent bouts of sinusitis (si-nu-SI-tis), an infection of the sinuses. The sinuses are hollow air spaces around the eyes, nose, and forehead. Frequent bouts of bronchitis (bron-KI-tis) and pneumonia (nu-MO-ne-ah) also can occur.

These infections can cause long-term lung damage. As CF gets worse, you may have more serious problems, such as pneumothorax (noo-mo-THOR-aks) or bronchiectasis (brong-ke-EK-ta-sis). Some people who have CF also develop nasal polyps (growths in the nose) that may require surgery. In CF, mucus can block tubes, or ducts, in your pancreas (an organ in your abdomen). These blockages prevent enzymes from reaching your intestines. As a result, your intestines can't fully absorb fats and proteins. This can cause ongoing diarrhea or bulky, foul-smelling, greasy stools. Intestinal blockages also may occur, especially in newborns. Too much gas or severe constipation in the intestines may cause stomach pain and discomfort. A hallmark of CF in children is poor weight gain and growth. These children are unable to get enough nutrients from their food because of the lack of enzymes to help absorb fats and proteins. As CF gets worse, other problems may occur, such as: Men who have CF are infertile because they're born without a vas deferens.

The vas deferens is a tube that delivers sperm from the testes to the penis. Women who have CF may have a hard time getting pregnant because of mucus blocking the cervix or other CF complications. Other signs and symptoms of CF are related to an upset of the balance of minerals in your blood.Wedding Dresses For Sale In Qatar CF causes your sweat to become very salty. Bowie Knife Uk ShopAs a result, your body loses large amounts of salt when you sweat. Hitachi Digital Camera User ManualThis can cause dehydration (a lack of fluid in your body), increased heart rate, fatigue (tiredness), weakness, decreased blood pressure, heat stroke, and, rarely, death. CF also can cause clubbing and low bone density. Clubbing is the widening and rounding of the tips of your fingers and toes.

This sign develops late in CF because your lungs aren't moving enough oxygen into your bloodstream. Low bone density also tends to occur late in CF. It can lead to a bone-thinning disorder called osteoporosis (OS-te-o-po-RO-sis). Next: How is cystic fibrosis diagnosed?Pseudomonal infections can involve any part of the body. Pneumonia is observed in patients with immunosuppression and chronic lung disease. It can be acquired nosocomially in the intensive care unit (ICU) setting and is associated with positive-pressure ventilation and endotracheal tubes. The pneumonia may be primary, following aspiration of the organism from the upper respiratory tract, especially in patients on mechanical ventilation. Alternatively, it may occur as a result of bacteremic spread to the lungs. This is observed commonly in patients following chemotherapy-induced neutropenia. Bacteremic pneumonia occurs in patients with neutropenia following chemotherapy and in patients with AIDS. Chronic infection of the lower respiratory tract with P aeruginosa is prevalent among patients with cystic fibrosis.

These patients may present with chronic productive cough, anorexia, weight loss, wheezing, and tachypnea. Symptoms of pneumonia include fever, chills, severe dyspnea, cyanosis, productive cough, confusion, and other signs of a systemic inflammatory response. Bacteremia may be acquired via medical devices in hospitals and nursing homes, and the mortality rate remains greater than 10%. Signs and symptoms depend on the primary site of infection. P aeruginosa may infect native heart valves in individuals who abuse intravenous drugs and may infect prosthetic heart valves. Right-sided and left-sided valve infections may occur. Nonspecific symptoms include fever and malaise, with more specific symptoms depending on which cardiac valve is involved. Left-sided endocarditis typically presents with symptoms of congestive heart failure and those resulting from systemic spread of septic emboli. P aeruginosa infection can cause meningitis and brain abscess. Most infections follow an extension from a contiguous parameningeal structure, such as an ear, a mastoid, paranasal sinus surgery, or diagnostic procedures.

In some patients, the involvement of the CNS is due to hematogenous spread of the organism from infective endocarditis, pneumonia, or UTI. Patients present with fever, headache, and confusion. The onset may be fulminant or subacute, often depending on the immune status of the patient. In otitis externa (swimmer's ear), patients present with pain, pruritus, and ear discharge. The pain is worsened by traction on the pinna. Pseudomonas infection is a common cause of chronic otitis media. Malignant otitis externa is a manifestation of invasive infection predominantly observed in patients with uncontrolled diabetes. It begins as ordinary otitis externa that fails to respond to antibiotic therapy. Presenting symptoms are persistent pain, edema, and tenderness of the soft tissues of the ear, with a purulent discharge. Fever is uncommon, and some patients present with a facial nerve palsy. Extension of the infection to the temporal bone can result in osteomyelitis, and further extension can create cranial nerve palsies and possibly a CNS infection.

The cornea, aqueous humor, and vitreous humor comprise an immunocompromised environment, and Pseudomonas, when introduced, produces extracellular enzymes that cause a rapidly progressive and destructive lesion. P aeruginosa is a common cause of bacterial keratitis, scleral abscess, and endophthalmitis in adults and ophthalmia neonatorum in children. Predisposing conditions for corneal involvement are trauma, contact lens use, predisposing ocular conditions, exposure to an ICU environment, and AIDS. Corneal lesions can progress to endophthalmitis and orbital cellulitis. Symptoms are pain, redness, swelling, and impaired vision. The most common sites of involvement are the vertebral column, the pelvis, and the sternoclavicular joint. Infection may be blood-borne, as in individuals who abuse intravenous drugs or in patients with pelvic infections or UTI. Alternatively, the infection may be contiguous, related to penetrating trauma, surgery, or overlying soft tissue infections.

Patients at risk for pseudomonal bone and joint infections include those with puncture wounds to the foot, peripheral vascular disease, intravenous drug abuse, or diabetes mellitus. Vertebral osteomyelitis may involve the cervical spine, and patients present with neck or back pain lasting weeks to months. Occasionally, patients with complicated UTI may develop lumbosacral vertebral osteomyelitis. Patients with pyoarthrosis present with swelling and pain in the affected joint. Patients are persistently febrile. Pseudomonal infections can affect every portion of the GI tract. The disease is often underestimated but usually affects very young children and adults with hematologic malignancies and chemotherapy-induced neutropenia. Additionally, colonization of the GI tract is an important portal of entry for pseudomonal bacteremia in patients who are neutropenic. The spectrum of disease can range from very mild symptoms to severe necrotizing enterocolitis with significant morbidity and mortality.

Epidemics of pseudomonal diarrhea can occur in nurseries. Young infants may present with irritability, vomiting, diarrhea, and dehydration. The infection can cause enteritis, with patients presenting with prostration, headache, fever, and diarrhea (Shanghai fever). Pseudomonas typhlitis typically presents in patients with neutropenia resulting from acute leukemia, with a sudden onset of fever, abdominal distension, and worsening abdominal pain. Pseudomonal UTIs are usually hospital-acquired and are associated with catheterization, instrumentation, and surgery. These infections can involve the urinary tract through an ascending infection or through bacteremic spread. In addition, these infections are a frequent source of bacteremia. No specific characteristics distinguish this type of infection from other forms of UTI. Pseudomonas does not grow on dry skin, but it flourishes on moist skin. Green nail syndrome is a paronychial infection that can develop in individuals whose hands are frequently submerged in water.