Causes of Chronic Granulomatous Disease

Chronic granulomatous disease is also known as fatal granulomatosis or childhood and progressive septic granulomatosis, according to the U.S. National Library of Medicine. Chronic granulomatous disease, or CGD, is a disorder that is inherited in which the immune system's cells called phagocytes do not work as they should. This can lead to constant infections.

Causes

• As part of the white blood cells that make up the immune system, phagocytes are cells that kill bacteria and foreign objects that enter the bloodstream. When these cells do not kill all the bacteria or fungi that enter the bloodstream, repeated infections that can last for extended periods of time are caused. These infections can range from small to large staph infections that take over the entire body and cause major damage that requires hospitalization.

Symptoms

• Skin abscesses with furuncles or pus, rectal abscesses as well as pneumonia is caused by recurrent bacteria. Swellings of lymph nodes in the neck as well as abscesses formed around the neck are common. Impetigo, a contagious skin infection that makes blisters and sores that itch, is also a common symptom of CGD. Dr. Lawrence Wolfe says that this disease normally reaches fatality rates in the 20s and 30s of the patient's years of life. Osteomyelitis and septicemia are also commonly found symptoms.

Cures

• According to Wolfe, more than two-thirds of all CDG cases are inherited from the X-linked defects, and 86 percent of these patients are male. Antimicrobial prophylaxism, which is an aggressive treatment for infections, can be given if the disease is found early on. Hematopoietic stem cell transplants, or HSCT, are done from a human leukocyte antigen, or HLA. A donor with the same blood type as the patient can be used to help cure this disease, yet the morbidity rate for the recipient is high enough that this treatment is often not undertaken.

Medications

• Bactrim or TMP-SMZ are given orally to help rid the body of bacterial infections without raising the number of fungal infections. If sulfa drugs are an allergy to the recipient, TMP or cephalosporin derivatives can be given daily. Ketoconazole can be given to help fight off the fungal infections that are not killed by Bactrim. To keep down toxicity levels in advanced cases, coriconazole and posaconazole should be given for fungal infections, although renal dysfunction is a side effect. In lung or liver abscesses, postoperative antibiotics should be given to help deal with the surgery to debulk the abscesses. High-dose interferon-gamma can help with infections, as well as IV antimicrobial therapies. The most favored treatment for all cases is low-dose corticosteroids, which can be given before biopsies are completed due to the high success rate.

Side Effects

• The side effects from the medications given to cure CGD range from headaches and nausea to double vision and dizziness. These side effects differ from medication to medication and from patient to patient. Not all patients taking the same medication will experience the same side effects, if any at all. For certain medications, a physician will need to monitor blood levels for liver function and kidney function if these medications are taken for an extended period of time.