MedPix® Patient Chart - Case No: 13973 :: - Review Images

A 26 day-old, Caucasian male with a history of GERD is admitted for failure to thrive. On admission history and physical exam he was noted to have hypertension.

Birth History: On admission, he was a 26 day-old, Caucasian male. Mom was G3 P3 and her pregnancy was complicated by preterm labor at 5 months, with the remainder of the pregnancy being uncomplicated. She was on bed rest for the last month of the pregnancy, but was able to carry the baby to term and had an uncomplicated SVD at 40 weeks. Mom received routine prenatal care throughout the pregnancy including 2 ultrasounds that were normal. She denied any history of smoking, drugs, or medications during her pregnancy. The patient was a full term infant with a birth weight of 7lbs 1 oz. He was discharged from the hospital on day #2 of life at the same weight.

He was 6lbs 9 oz at his one-week check-up and had developed issues with vomiting/GERD. As a result, his formula was changed and he was started on Zantac. However, the vomiting persisted, his continued to lose weight and was noted to tire with feeding- so he was admitted at 26 days-old for evaluation of failure to thrive.

PMH:
1. GERD
2. Failure to thrive

Surgical Hx: None.

Family Hx:
Dad: Childhood murmur that resolved
Mom: Healthy
Maternal Grandfather: HTN diagnosed as an adult
There is no FH of renal disease, dialysis requirement, HTN in childhood, congenital heart disease, or genetic diseases.
The patient has 1 and 3 year-old siblings that are healthy.

Medications:
- Zantac on admission.

Allergies: NKDA

Physical Exam:
Vitals on Admission: T: 37.1 C; P: 160, regular; R 44; BP: 127/77 in right arm. Manual BP’s were repeated while infant was sleeping using a Doppler and revealed a BP of 165-170/95-100 in the left arm, and 160-165/90-95 in the right arm.
SaO2 was 100% on RA. Height was 53cm. Weight: 3.05 kg.
General: Marasmic appearing infant with good a good active cry, interactive, resting comfortably in bed.
HEENT: Anterior fontanel open, flat, and soft.
CV: Heart RRR with a 2/6 systolic ejection murmur noted. Capillary refill brisk. Distal pulses symmetric and intact.
Lungs: Clear to auscultation bilaterally with good air entry. Normal work of breathing.
Abdomen: Soft, non-tender, non-distended. Bowel sounds normal. No palpable masses. No bruits heard over abdomen.
Genitals: Normal Tanner 1 male infant. Testes descended bilaterally. Circumcised.
Neurologic - Tone slightly low. Suck / moro / plantar / grasp reflex intact, but moro was slow.
Skin – Capillary refill < 2 seconds. No rashes, jaundice, petechiae or bruising. Minimal subcutaneous fat. No sacral dimple.
Musculoskeletal - No hip clicks or clunks, moves all extremities equally.

Laboratory Data:
Admission Labs at 1-month of age:
CBC: WBC# 14,600; Hgb 12.9(low); Hct 36.5(low); Plt# 536,000(high); MCV 102; RBC# 3.58 (low)

BMP: Sodium 133, Potassium 3.9(low), Chloride 94(low), CO2 31.7 (high), BUN 16, Creatinine 0.4, Calcium 10.7.

Urinalysis showed 100mg/dL of protein with a positive sulfasalicylic acid test.
Urine Organic Acids= Normal

Plasma Amino Acids= Normal
TSH: 4.107 (normal)
T4: 1.06 (normal)
Sweat Chloride: 11 (normal)
Plasma Aldosterone: 663 ng/dL (high) [6-179 ng/dL]
Plasma Renin Activity: 137 ng/mL/hr (high) [2.4-37.0 ng/mL/hr]
ACTH: 50 pg/mL (high) [5-46 pg/mL]
Cortisol: 3.6 (normal)
Urine normetanephrines: normal
Urine metanephrines: 741 (high) [ 83-523]
Urine total metanephrines: normal

Random urine protein: 88.1 (high) [1-14]
Random urine potassium: 32.9 (normal)
Random urine sodium: 10 (low) [20-110]
Random urine creatinine:10.7 (low) [30-125]

Pre-Operative Labs Before Angiography at 7-months of age:
Plasma Renin Activity: 32.3 ng/mL/hr (normal)
Plasma Aldosterone: 91 ng/dL (normal)

Intra-operative Labs (Angiography)at 7-months of age:
Pre-Captopril PRA from above the renal vein: 73.8 ng/mL/hr
Pre-Captopril PRA from below the renal veins: 46.5
Pre-Capropril PRA from the left renal vein: 93.0
Pre-Captopril PRA from the right renal vein: 40.4
---Patient given Captopril and 20 minutes was allowed to pass------
Post-Captopril PRA from above the renal vein: 171.5
Post-Captopril PRA from below the renal vein: 102.3
Post-Captopril PRA from the left renal vein: 206.1
Post-Captopril PRA from the right renal vein: 103.3

- Review Images for PT: 13973

1. Renal Ultrasound at 26 days-old:
Both kidneys were 5.3cm in length. There was no evidence of a renal mass on either kidney. Pelvocaliceal systems and ureters were normal without hydronephrosis or hydroureter. The abdominal aorta, IVC, and iliac arteries appeared normal. The bladder was normal.

2. 2D- Echocardiogram at 26 days-old:
Mild, concentric LVH but otherwise normal.

3. Digital Subtraction Angiogram (Aortogram) at the age of 7 months:
The abdominal aorta has a normal appearance. A single left renal artery is present at the L1-L2 level. The left main renal artery has a smooth appearance without beading or narrowing. The segmental, subsegmental, and arcuate arteries appear normal on the left. A small fetal lobule is present at the lower pole of the left kidney. Left renal vein flow is demonstrated to the IVC. There appears to be compensatory hypertrophy of the left kidney.

A single right renal artery is demonstrated from the aorta. The proximal right renal artery is quite narrow, with an additional focal narrowing present 1cm distal to the origin of the right renal artery. Mild poststenotic dilatation of the distal right renal artery is present. Delayed contrast flow is demonstrated through a small right kidney. The right subsegmental arteries are tortuous and irregular in appearance throughout the small right kidney.

4. Renal Ultrasound at 8-months-old (post-angiogram):
The right kidney is small and measures 4.3cm in length compared to the left kidney which measures 6.9 cm in length. On the previous ultrasound, both kidneys measured 5.3cm in length. The collecting systems and ureters are normal bilaterally. Renal cortical echogenicity is normal bilaterally. No renal masses are seen. The parenchyma of the right kidney is thinned compared to the previous exam.

5. DMSO Renography at 8.5 months-old:
Nuclear medicine renography shows no uptake in the right kidney.

- Review Images for PT: 13973

1. Fibromuscular dysplasia of the right renal artery
2. Congenital renal artery stenosis
3. Congenital right renal hypoplasia

- Review Images for PT: 13973

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