Infantile polycystic kidney disease: autosomal recessive inheritance, are
associated with liver disease, although mainly seen in children, but can also
occur in older children and adults, a significant increase in the kidneys, to
maintain the normal shape, smooth surface, But fetal kidney leaf state more
obvious than the normal kidney, because the kidney cortex and medulla was
violated small cysts, so cut surface spongy or honeycomb-like, histology, renal
and kidney are most aligned at right angles to the surface a long cyst replaced
the film can be seen in a small number of normal glomeruli and convoluted
tubule, renal function tests show the cyst is a functional part of the nephron,
microdissection visible cyst expansion manifold, ureter and bladder normal
development, in severe cases, lead to poor bladder due to fetal oliguria
development,
According to the age of onset of symptoms, infantile polycystic kidney
disease can be divided into four clinical subtypes:
① perinatal type: As the baby abdominal distension, labor is often long and
not well seen in children with poor circulation and breathing difficulties,
showed typical Potter face, sometimes due to pulmonary hypoplasia,
pneumomediastinum and cyanosis, significant dyspnea, but soon died at birth or
after birth, some babies can reach huge kidneys birth, after birth uremia,
pyuria, hematuria and hypertension, perinatal may survive, 90% or more of these
babies renal kidney tissue cystic or dysplastic, intravenous urography kidney
does not develop, renal ultrasonography confirm large and polycystic kidneys,
urinary bladder urinary bladder angiography showed normal, no reflux, no
urethral obstruction, such as are born uremia the prognosis is poor, the high
percentage of stillborn, died within three months the majority of uremia are in
perinatal or infant after birth;
② neonatal form: Survivors in the neonatal period, often progressive uremia,
hypertension, and increased bilateral renal touched by kidney ultrasound cyst
wide range of diseases, about 60% of renal units abnormal development, more
after birth, died in the June uremia, some children through appropriate
restrictions on protein in the diet, the treatment of renal acidosis,
hyperphosphatemia, and high blood pressure can reduce the uremia, renal function
improved, sick children can survive to childhood, but the disease of the liver
gradually with age;
③ infantile: 25% to 50% of renal units have cystic kidney abnormal
development, clinical manifestations of growth retardation, and liver function
uremic childhood failure at birth and neonatal difficult type difference, but
infantile In the infancy no uremic;
④ childhood type: Since bilateral renal cystic disease of less than 10%,
while liver disease widely, so the performance of liver lesions in 10 to 20
years old, if only to do an autopsy incidentally found to have polycystic kidney
disease.
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