Pediatric Nephrology

Pediatr Nephrol (1992) 6:556-558 9 IPNA 1992

Brief report Hypertension associated with diffuse small artery calcification: a ease report Elisabeth M. Hodsonl, Vincent F. Antic02, and Peter O'NeilP Departments of 1 Paediatrics, 2 Nuclear Medicine and Ultrasound, and s Anatomical Pathology, Westmead Hospital, Westmead, Sydney, New South Wales 2145, Australia Received November 18, 1991; received in revised form March 31, 1992; accepted April 2, 1992

Abstract. R e n a l ultrasound e x a m i n a t i o n , p e r f o r m e d foll o w i n g a urinary tract infection in a 4 . 5 - y e a r - o l d girl with triple X s y n d r o m e , s h o w e d m u l t i p l e e c h o g e n i c foci at the c o r t i c o m e d u l l a r y j u n c t i o n in b o t h k i d n e y s . She was a s y m p t o m a t i c but h a d h y p e r t e n s i o n with e c h o c a r d i o g r a p h i c evid e n c e o f left ventricular h y p e r t r o p h y . C o m p u t e r i s e d t o m o g r a p h i c scan r e v e a l e d foci o f calcification in the k i d n e y s , spleen and pancreas. O n b i o p s y c a l c i f i c a t i o n was f o u n d in the internal and external elastic l a m i n a e o f the superficial t e m p o r a l artery and in the internal elastic l a m i n a o f a renal arcuate artery. I n t i m a l fibrosis was m i l d and focal. N o other arterial c a l c i f i c a t i o n was d e m o n s t r a t e d r a d i o g r a p h i cally or b y ultrasound. B i o c h e m i c a l and h o r m o n a l profiles r e v e a l e d no a b n o r m a l i t y e x c e p t hypercalciuria. T h e aetio l o g y and p r o g n o s i s o f this c h i l d ' s c o n d i t i o n are u n k n o w n . A l t h o u g h similar u l t r a s o u n d and h i s t o l o g i c a l a p p e a r a n c e s h a v e b e e n d e s c r i b e d in p s e u d o x a n t h o m a e l a s f i c u m and in idiopathic arterial calcification o f infancy, there is no evid e n c e that the child has either o f these conditions.

Key words: H y p e r t e n s i o n - R e n a l u l t r a s o u n d - A r t e r i a l calcification - H y p e r c a l c i u r i a

Introduction A m o n g c h i l d r e n i n v e s t i g a t e d for r e n o v a s c u l a r h y p e r t e n sion, a few h a v e b e e n d e s c r i b e d with i n c r e a s e d e c h o g e n i c ity at the c o r t i c o m e d u l l a r y j u n c t i o n on renal ultrasound e x a m i n a t i o n , n o r m a l renal a n g i o g r a p h y and c a l c i f i c a t i o n in the internal elastic l a m i n a e o f superficial t e m p o r a l and renal i n t e r l o b u l a r arteries [ 1 - 3 ] . S i x c h i l d r e n h a d p s e u d o x a n t h o m a e l a s t i c u m ( P X E ) [3]. W e r e p o r t a child with

Correspondence to: E. M. Hodson

triple X s y n d r o m e w h o h a d h y p e r t e n s i o n and similar renal ultrasound and h i s t o l o g i c a l changes. In addition she h a d splenic and pancreatic calcification and hypercalciuria. She h a d no d e r m a t o l o g i c a l or fundal e v i d e n c e o f PXE.

Case report This previously well 4.5-year-old girl underwent renal ultrasound examination following a urinary tract infection. Both kidneys exhibited multiple echogenic foci in the region of the corticomedullary junction (Fig. 1). The echogenic foci extended between, but did not involve, the medullary pyramids, and a rim renal cortex of normal appearance was seen peripheral to the echogenic areas. Similar echogenic foce were seen in the spleen. The child had triple X syndrome (47 XXX) detected by amniocentesis and confirmed postuatally. She was the third child of non-consanguineous parents and was born at term, following a normal pregnancy which was not complicated by maternal hypertension or diabetes mellims. Resuscitation or placement of umbilical vessel catheters were not required. There was no family history of hypertension, renal disease or renal calculi. One sibling had died aged 6 weeks following surgery for interrupted aortic arch and tmncus arteriosus. The other sibling was well. The family was of English and Irish background, On examination, the child's height and weight were on the 75th percentile and her head circumference on the 50th percentile. Her blood pressure was persistently elevated at 135/90 mmHg. All her peripheral pulses were palpable and there were no bruits. No skin changes of PXE were noted and her fundi were normal. The remainder of the physical examination was normal. Investigations revealed: serum creatinine 42 gmol/1, calcium 2.55 mmol/1, phosphate 1.49 mmol/1, magnesium 0.87 mmol/1 and bicarbonate 26 mmol/1. Other serum electrolytes, liver enzymes, uric acid, glucose, cholesterol and triglycerides were within normal limits. Urinalysis showed no blood or protein and urine culture was negative. In the morning, when the child was ambulant, on a normal salt intake and on no medications, plasma renin activity was 970 fmol/1 per second (normal 100-1,500), plasma aldosterone 1,080 pmolfl (normal 80-1,040), plasma adrenaline 1.8 nmol/l (normal 0.1-6.3) and plasma noradrenaline 0.3 nmol/1 (normal 0-1.5). Intact parathyroid hormone was 14 pg/ml (normal 10-65), 25-hydroxyvitamin D 66 nmol/1 (normal 18-128) and 1,25-dihydroxyvitamin D 140 pmol/1 (normal 4 5 - 175). Urinary excretion of uric acid, oxalate and citrate were normal. The urinary amino acid pattern was normal and cystine, glycolic acid and glyceric acid were not increased. Urinary calcitlm excretion was increased at 0.24 mmol/kg per day (normal

Hypertension associated with diffuse small artery calcification: a case report.

Renal ultrasound examination, performed following a urinary tract infection in a 4.5-year-old girl with triple X syndrome, showed multiple echogenic f...
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