It is the most common hereditary diseases of type I glycogen storage and, clinically, the most serious, where the cause of the disturbance is the absence of or diminishing of glucose-6-phosphatase of the liver, the intestinal mucosa, and the kidneys (2,3); leading to an accumulation of abnormally high amounts of glycogen in tissues like the liver and renal tubular cells, hence, through this mechanism producing hepatomegaly and nephromegaly (3,4), the first is well-described in both patients previously discussed and nephromegaly was noted in the younger patient.
The kidneys also show increase in size, as with the child in the case where nephromegaly was detected via ultrasound at 5 years of age; while the spleen and the heart are normal.
If we correlate clinical aspects with the cardinal symptoms of hypoglycemia associated to hepatomegaly and nephromegaly, we should consider a glycogen storage disease: Von Gierke's disease.
While CT scan findings of hydronephrosis, hydroureter, nephromegaly
, and perinephric stranding are helpful in diagnosing ureteral stones, they are not helpful in guiding the decision-making process for intervention.
Due to the incipient nephromegaly
, the diabetic kidney often looks bigger and 'Better' than the kidney with the same level of chronic, irreversible renal failure caused by other chronic renal diseases such as other glomerular diseases, hypertensive nephropathy or tubulointerstitial diseases.
Computed tomography indicated hepatosplenomegaly and a small pericardial effusion; ultrasonography indicated bilateral nephromegaly
; and echocardiography indicated a small pericardial effusion and an ejection fraction of 59%.
Among our study group the frequency expressed as percentages of various secondary signs of ureterolithiasis were hydroureter 25 (83.33 Percent), hydronephrosis of the affected kidney in 23 (76.67 Percent), nephromegaly 16 (53.33 Percent), periureteral edema 19 (63.33 Percent), perinephric stranding 21 (70 Percent), a difference in attenuation between the kidneys 25 (83.33 Percent) while soft tissue rim sign was present in 14 (46.67 Percent) patients.
Nephromegaly: An increase of more than 12 cm in the size of the right kidney and 12.2 cm in the size of the left kidney.
An ultrasound examination, abdominal computed tomographic scan, and magnetic resonance imaging were performed and revealed bilateral nephromegaly
due to bilateral solid masses with no obvious lymphadenopathy or vascular involvement.
includes the presence of either three major features (anterior abdominal wall defect, macroglossia,  or prepostnatal overgrowth) or two major plus three minor findings (ear creases and pits, facial nevus flammeus, neonatal hypoglycemia,  nephromegaly
,  or Hemihyperplasia, embryonal tumors, polyhydramnios).
Renal lymphangiomatosis: A rare cause of bilateral nephromegaly
. Australas Radiol 2003;47:184-7http://dx.doi.org/10.1046/j.0004-8461.2003.01149.x.