Re: Renal Agenesis and Noncystic Renal Dysplasia - Hypovolemic shock??

From: James S. Smeltzer, MD (gaperina@mindspring.com)
Sun May 20 11:54:12 2001


Rich, the urine present and urine production in response to lasix and weight loss, vomiting indicate that the infant may have hypovolemic shock from intestinal obstruction or necrosis as the main problem. If K is LOW this is true. If K is high then intrinsic renal disease is more likely. An easy way to tell the difference is to look at the heart. If filling is lousy then baby is dry. If overfull then volume is overloaded, which I doubt given the history.

It is sort of urgent, as ATN is the last thing this baby needs to add to her problems.

Re: Gut malrotation and renal problem there are two X-Linked: X-linked Vater and Opitz G/BBB syndrome, ruled out by sex in this baby. Cat eye syndrome may have malrotation and renal dysplasia (Inv dup 22 q11), but other stigmata are prominent. Pallister-Hall syndrome (Mapped to 7p13) has many CNS problems and extremity abnormalities you do not mention. The laterality sequence is also sex-linked, and typically does not include renal problems. I cannot find a good fit for the malrotation renal aplasia with no other findings.

Good luck! Jim S

>----- Original Message -----
From: "Rich Dempsey RDMS/RVT " <ridem@msn.com> To: "Multiple recipients of list ULTRASOUND" <ultrasound@mail.medispecialty.com> Sent: Friday, May 18, 2001 10:18 PM Subject: Renal Agenesis and Noncystic Renal Dysplasia

> Scanned a 7 day old female infant,(born at 38 weeks prematurity) with a
> creatinine of 3.0, and metabolic acidosis (although the infant
> apparently has a malrotated gut and vomiting), and abmormal blood K
> levels. Lasix injection resulted in 70 cc of urine. They infant has
> lost 20% of its birthweight, presumably due to dehydration.
>
> I was unable to locate a right kidney, after an extensive search from
> the right adrenal gland down the psoas to the pelvis. The LEFT kidney
> was in the expected location, but measured only 2.5 cm, which is 2 SD
> below the mean of 4.5 cm for a newborn. Scanning (last resort) through
> the infant's back the left kidney, psoas, spine, and right psoas were
> vizualized. I felt that fused cross ectopia, etc was unlikely, although
> a nuclear renogram is the next planned test.
>
> There was no hydronephrosis, cortical or medullary cysts. The solitary
> kidney appeared mildly echogenic; there were hypoechoic areas within the
> renal cortex/medulla, however they were NOT arranged like neonatal renal
> pryramids. I don't believe that they were cysts though. MOST
> unfortunately I was unable to do Color Doppler for renal vein
> thrombosis,presence of a RRenArt, etc.
>
> There was a small amount of urine present in the normal appearing
> bladder, a uterus and (probably? both ovaries were briefly seen). This
> was a tough scan,done portably, on an irritable neonate.
>
> Anyone know of syndromes with these findings, or are the findings just
> part of the continuum stopping short of bilateral renal agenesis ?
> (Solitary dysplastic kidney).
>
> Reply: ridem@msn.com
>




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