Document Detail


Conjugated hyperbilirubinaemia as the first manifestation of mevalonic aciduria in a term newborn.
MedLine Citation:
PMID:  20027140     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
OBJECTIVES: To present clinical and laboratory findings in the case of a term newborn with conjugated hyperbilirubinaemia and to stress the importance of differential diagnosis. RESULTS: A term newborn delivered by caesarean section (birth weight 2550 g, birth length 47 cm, value of Apgar score 9/10) with good direct adaptation had on the first day of life increased levels of conjugated bilirubin (23 micromol/l), unconjugated bilirubin (55 micromol/l) and C-reactive protein 39.4 g/l. The diagnosis of mevalonic aciduria was confirmed by urine analysis (mevalonolactone 393 micromol/mmol crea, normal range <2.0 micromol/mmol crea; mevalonic acid 40.5 micromol/mmol crea, normal range <0.04 micromol/mmol crea). CONCLUSION: Mevalonic aciduria can be clinically distinguished based on symptoms of neurological involvement. It can also present itself with hepatosplenomegaly, lymphadenopathy, anaemia, leukocytosis, increased sedimentation rates and levels of C-reactive protein. In cases of conjugated hyperbilirubinaemia of unknown aetiology it is important to exclude mevalonic aciduria by urine investigation for organic acids.
Authors:
Ingrid Brucknerova; Darina Behulova; Claudia Sebova; Vladimir Bzduch; Mojmir Mach; Michal Dubovicky; Eduard Ujhazy
Publication Detail:
Type:  Case Reports; Journal Article; Research Support, Non-U.S. Gov't    
Journal Detail:
Title:  Neuro endocrinology letters     Volume:  30 Suppl 1     ISSN:  0172-780X     ISO Abbreviation:  Neuro Endocrinol. Lett.     Publication Date:  2009  
Date Detail:
Created Date:  2010-02-26     Completed Date:  2010-05-24     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  8008373     Medline TA:  Neuro Endocrinol Lett     Country:  Sweden    
Other Details:
Languages:  eng     Pagination:  29-31     Citation Subset:  IM    
Affiliation:
1st Department of Paediatrics, Medical School, Comenius University, Bratislava, Slovakia. osmium@centrum.sk
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MeSH Terms
Descriptor/Qualifier:
Diagnosis, Differential
Humans
Hyperbilirubinemia, Neonatal / diagnosis*,  etiology,  urine*
Infant, Newborn
Male
Mevalonate Kinase Deficiency / complications,  diagnosis*,  urine*

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