Renal architecture and function are abnormal in the majority of children with tyrosinemia. Liver transplantation improves renal function in about 50 % of patients, but abnormal renal size and architecture persist.
are the youngest with this affliction reported to date. Milk of calcium proximal to a staghom calculus has not been described previously. Gravity-dependent sonography was the most efficient and sensitive method of diagnosis. Follow-up of 1-16 years showed the formation and gradual increase in the quantity of UMC without evidence of gross calculus formation within the milk. In two patients, there was spontaneous, partial drainage of UMC.
Slowing and dampening of systole in the arterial network distal to stenosis is a well-known Doppler sign of severe arterial stenosis. To determine whether this sign is present in boys and girls with such stenosis, intrarenal Doppler curves (acceleration index [AI] and resistive index [RI]) were compared with findings on renal arteriograms in 20 boys and girls; the AI was also measured in 10 boys and girls without renal disease. Statistical analysis of AI and RI measurements was performed. Eleven of 32 renal arteries were normal. The normal AI was 4.0-7.0; in arteries with greater than 75% stenosis, the AI was 0.7-1.7. In five arteries studied after angioplasty, the AI had changed from 0.7-1.5 to 4.0-5.6 at the first posttreatment examination (performed 28 hours to 1 week after angioplasty), and it remained normal during the 3-year follow-up period. In kidneys with stenotic arteries, the RIs were lower (0.43-0.54) than in healthy subjects (0.56-0.63). Regression and correlation coefficients of AI and RI measurements were statistically significant, and discrimination between normal arteries and those with greater than 75% stenosis was excellent.
Ureteric jets were studied with conventional duplex Doppler (79 patients) and with color Doppler flow imaging (22 patients). Correlation with voiding cystourethrography (VCUG), performed ont he same day, was obtained in all patients. Thirty-six patients with normal VCUG results and normal renal and bladder ultrasound (US) studies served as the control group for 39 patients with vesicoureteric reflux (VUR) and 26 patients with urinary tract disorders other than reflux. The site of the ureteric orifice and duration, direction, and turbulence of the jets were recorded. Duration varied from 0.4 sec to 7.5 sec and depended largely on fluid intake. Duration varied in an individual patient by up to 2 sec from one jet to another. The direction of the normal jet was anteromedial and upward. Jets from refluxing ureters can appear normal. Severe renal parenchymal scarring reduced frequency and amplitude of the jets. Doppler analysis of the ureteric jet does not allow diagnosis or exclusion of VUR. Color Doppler was more sensitive in demonstrating ureteric jets than was gray-scale, real-time US and facilitated the study, but it was equally unable to help predict reflux.
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