Facts on File Encyclopedia of Health and Medicine

early childhood, typically following an upper respi-
ratory viral INFECTIONsuch as a cold. As the damage
to the kidneys progresses, symptoms expand to
include ALBUMINURIA(excessive albumin or protein
in the urine) and HYPERTENSION(high BLOOD PRES-
SURE). About 80 percent of those who have Alport’s
syndrome develop neurosensory HEARING LOSSby
ADOLESCENCE, and 15 percent have irregularities in
the shape of the lens of the eye (lenticonus).
Symptoms in combination with family history
strongly suggest Alport’s syndrome, though kid-
ney biopsy is the definitive diagnostic tool. Kidney
biopsy, in which the urologist extracts a core of
kidney tissue using a large-gauge needle, allows
the him or her to determine the absence of any of
the three affected protein chains as well as to
assess the degree of damage already present. SKIN
biopsy can confirm X-linked Alport’s syndrome
because the skin in this type of the disorder lacks
the alpha-5 protein chain.

Treatment Options and Outlook

Treatment is primarily supportive and targets
symptoms such as hypertension, hearing loss, and
VISION IMPAIRMENT. For people who reach ESRD,
KIDNEY TRANSPLANTATIONoften offers a viable thera-
peutic course. With treatment, many people who
have Alport’s syndrome are able to enjoy the

lifestyles of their choosing well into the fifth or

sixth decade of life. The likelihood of RENAL FAILURE

increases with age, however.

Risk Factors and Preventive Measures

Because Alport’s syndrome is an inherited genetic

disorder, the only risks for this condition are the

causative gene mutations. There are no measures

to prevent the condition. Early treatment for con-

sequential health conditions and close medical

monitoring of kidney function help maintain opti-

mal health.

See also ALVEOLUS; GENETIC DISORDERS; GLOMERU-

LUS; INHERITANCE PATTERNS; MUTATION; RENAL DIALYSIS.

anuria The failure to produce URINE. Numerous

circumstances can result in anuria, from severe

DEHYDRATIONand severe HYPOTENSION(low BLOOD

PRESSURE) to END-STAGE RENAL DISEASE(ESRD) and

RENAL FAILURE. Anuria requires prompt medical

evaluation to determine and remedy the underly-

ing cause. Without such correction, waste byprod-

ucts accumulate to toxic levels and the body

cannot continue to function.

See also DYSURIA; ENURESIS; NOCTURIA; OLIGURIA;

URINARY INCONTINENCE.

azotemia See UREMIA.

azotemia 177