Abstract:
PATIENT REPORTS:We report on a male preterm infant (gestational age 31 weeks, birth weight 1420 g) and a male term infant (gestational age 38 weeks, birth weight 3680 g) with ornithine transcarbamylase (OTC) deficiency. After inconspicuous cardiopulmonary adjustment, both entered a state of metabolic crisis with respiratory insufficiency and ventilatory requirement at the 2nd and 4th day of life, respectively. Diagnosis of hyperammonemia (NH(3) > 1000 micromol/l) was followed by the detection of a plasma amino acid pattern that is typical for OTC-deficiency and an excessive orotic aciduria. Beside intravenous treatment (insulin-glucose-infusion, lipid infusion, sodium benzoate, arginine, L-carnitine), the preterm infant received an exchange transfusion and was supplied with central venous catheters, hemofiltration and hemodialysis. He died after severe disturbances of circulation and coagulation at the 14th day. The male term infant tolerated the effective hemofiltration and was dicharged home with specific therapy at day 26. CONCLUSIONS:Time of diagnosis and influence of additional risk factors are decisively for the prognosis of OTC-deficiency. The immediate aims of therapy (stabilization of vital functions, reduction of plasma ammonium, control of nutrition) can only be realized in cooperation between neonatology, division of metabolism, pediatric nephrology and pediatric surgery.
journal_name
Z Geburtshilfe Neonatoljournal_title
Zeitschrift fur Geburtshilfe und Neonatologieauthors
Hübler A,Seidel J,Patzer L,Bellstedt K,Schramm Ddoi
10.1055/s-2001-19056subject
Has Abstractpub_date
2001-11-01 00:00:00pages
236-41issue
6eissn
0948-2393issn
1439-1651journal_volume
205pub_type
杂志文章abstract::To date there has not been a systematic analysis of pregnant patients with an immigrant background and gestational diabetes in Germany, even though the number of these patients has been rising continuously since the 1980s. METHODS:The case number estimate for this prospective study targeted 160 patients with gestatio...
journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
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journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
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abstract:OBJECTIVE:To assess neonatal outcome and delivery mode in dichorionic twin delivery at term with a cephalic-presenting first twin. METHODS:A retrospective cohort study of 308 twin deliveries after 37 completed weeks of gestation with a cephalic-presenting first twin undertaken in one perinatal center with active manag...
journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
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journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
pub_type: 临床试验,杂志文章
doi:
更新日期:1999-12-01 00:00:00
abstract:OBJECTIVE:This study examines the recommendations of international guidelines on the use of antepartum and intrapartum cardiotocography. MATERIAL AND METHODS:The guidelines of Germany, Canada, UK, USA, Sri Lanka, Australia as well as FIGO have been compared. The recommendations on the use of cardiotocography were sepa...
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journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
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journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
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pub_type: 临床试验,杂志文章
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更新日期:2005-06-01 00:00:00
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pub_type: 杂志文章
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更新日期:2020-10-14 00:00:00
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journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
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journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
pub_type: 杂志文章
doi:
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journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
pub_type: 杂志文章
doi:
更新日期:1996-03-01 00:00:00
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journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
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更新日期:2006-10-01 00:00:00
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更新日期:2020-04-01 00:00:00
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journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
pub_type: 杂志文章
doi:
更新日期:1998-01-01 00:00:00
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journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
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更新日期:2014-12-01 00:00:00
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journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
pub_type: 杂志文章
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更新日期:2014-06-01 00:00:00
abstract::The early detection of HELLP syndrome (hemolysis, elevated liver enzymes, and low platelets) is the basic condition for immediate therapeutic management, which mainly leads to prompt delivery. The classical symptoms despite the typical laboratory evaluation (hemolysis, elevated liver enzymes, low platelets) are epigas...
journal_title:Zeitschrift fur Geburtshilfe und Neonatologie
pub_type: 杂志文章,评审
doi:
更新日期:1996-05-01 00:00:00