Warning: file_get_contents(https://eutils.ncbi.nlm.nih.gov/entrez/eutils/elink.fcgi?dbfrom=pubmed&id=27574996
&cmd=llinks): Failed to open stream: HTTP request failed! HTTP/1.1 429 Too Many Requests
in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 215
Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Deprecated: Implicit conversion from float 231.6 to int loses precision in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 534
Warning: imagejpeg(C:\Inetpub\vhosts\kidney.de\httpdocs\phplern\27574996
.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 J+Pediatr
2016 ; 178
(ä): 254-260.e4
Nephropedia Template TP
gab.com Text
Twit Text FOAVip
Twit Text #
English Wikipedia
Hypercalcemia in Patients with Williams-Beuren Syndrome
#MMPMID27574996
Sindhar S
; Lugo M
; Levin MD
; Danback JR
; Brink BD
; Yu E
; Dietzen DJ
; Clark AL
; Purgert CA
; Waxler JL
; Elder RW
; Pober BR
; Kozel BA
J Pediatr
2016[Nov]; 178
(ä): 254-260.e4
PMID27574996
show ga
OBJECTIVE: To evaluate the timing, trajectory, and implications of hypercalcemia
in Williams-Beuren syndrome (WBS) through a multicenter retrospective study.
STUDY DESIGN: Data on plasma calcium levels from 232 subjects with WBS aged
0-67.1 years were compared with that in controls and also with available
normative data. Association testing was used to identify relevant comorbidities.
RESULTS: On average, individuals with WBS had higher plasma calcium levels than
controls, but 86.7% of values were normal. Nonpediatric laboratories overreport
hypercalcemia in small children. When pediatric reference intervals were applied,
the occurrence of hypercalcemia dropped by 51% in infants and by 38% in toddlers.
Across all ages, 6.1% of the subjects had actionable hypercalcemia. In children,
actionable hypercalcemia was seen in those aged 5-25 months. In older
individuals, actionable hypercalcemia was often secondary to another disease
process. Evidence of dehydration, hypercalciuria, and nephrocalcinosis were
common in both groups. Future hypercalcemia could not be reliably predicted by
screening calcium levels. A subgroup analysis of 91 subjects found no
associations between hypercalcemia and cardiovascular disease, gastrointestinal
complaints, or renal anomalies. Analyses of electrogradiography data showed an
inverse correlation of calcium concentration with corrected QT interval, but no
acute life-threatening events were reported. CONCLUSIONS: Actionable
hypercalcemia in patients with WBS occurs infrequently. Although irritability and
lethargy were commonly reported, no mortality or acute life-threatening events
were associated with hypercalcemia and the only statistically associated
morbidities were dehydration, hypercalciuria, and nephrocalcinosis.