scholarly journals P0181 ORAL VITAMIN K PROPHYLAXIS DOES NOT PREVENT LATE HEMORRHAGIC DISEASE OF THE NEWBORN IN PIZZ ALPHA-1 ANTITRYPSIN DEFICIENCY.

2004 ◽  
Vol 39 (Supplement 1) ◽  
pp. S127
Author(s):  
R. M. L. De Bruyne ◽  
S. Van Biervliet ◽  
A. France ◽  
M. Van Winckel ◽  
A. Deprettere ◽  
...  
Author(s):  
Christoph Bauer ◽  
Désirée Furthner ◽  
Eva Grohmann ◽  
Gerald Tulzer

Abstract Background  Vitamin K deficiency bleeding is a life-threatening complication in early infancy. Exclusive breastfeeding and neonatal cholestasis syndromes, most notable α-1-antitrypsin deficiency, have been reported to be risk factors. Intracranial haemorrhage is most common. No association to haemopericardium has been reported before. Case summary  We report on an 11 weeks old at term-born infant, who presented with severe anaemia and signs of cardiogenic shock. Immediately echocardiography was done and depicted cardiac tamponade. Pericardiocentesis was performed and a significant amount of haemorrhagic fluid was removed. Further workup revealed deranged coagulation parameters, cholestatic liver disease, and reduced α-1-antitrypsin levels. Despite normal brain sonography, a small cerebral haemorrhage was detected on magnetic resonance imaging. A genetic test finally proofed homozygotic mutation of the SERPINA1-gene and confirmed the diagnosis of α-1-antitrypsin deficiency as the underlaying cause. After initial replacement of coagulation factors, erythrocytes and vitamin K, the infant recovered. Eighteen weeks after discharge, the infant is still on vitamin K supplementation. She did not have any further bleedings and no neurologic or developmental impairment. Discussion  Alpha-1-antitrypsin deficiency can lead to vitamin K deficiency in young infants even with adequate prophylaxis. Spontaneous haemorrhagic pericardial effusion was a new manifestation of vitamin K deficiency bleeding in our patient and should be considered and ruled out in young infants who present with acute anaemia and poor clinical condition.


2003 ◽  
Vol 25 (3) ◽  
pp. 274-275 ◽  
Author(s):  
Tansu Sipahi ◽  
Cengiz Kara ◽  
Betül Tavil ◽  
Ayşe Ïnci ◽  
Ayşegül Oksal

2006 ◽  
Vol 166 (10) ◽  
pp. 1081-1082 ◽  
Author(s):  
Sophie Huybrechts ◽  
Marek Wojciechowski ◽  
Sandra Poot ◽  
Patrick Van Reempts ◽  
José Ramet

PEDIATRICS ◽  
1993 ◽  
Vol 91 (5) ◽  
pp. 1001-1002
Author(s):  

Vitamin K deficiency may cause unexpected bleeding (0.25% to 1.7% incidence) during the first week of life in previously healthy-appearing neonates (classic hemorrhagic disease of the newborn [HDN]). The efficacy of neonatal vitamin K prophylaxis (either oral or parenteral) in the prevention of classic HDN is firmly established. It has been the standard of care since the recommendation by the Committee on Nutrition was adopted as policy by the American Academy of Pediatrics in 19611 Late HDN, a syndrome defined as unexpected bleeding due to severe vitamin K deficiency in infants aged 2 to 12 weeks, occurs primarily in exclusively breast-fed infants who have received no or inadequate neonatal vitamin K prophylaxis. In addition, infants who have intestinal malabsorption defects (cholestatic jaundice, cystic fibrosis, etc) may also present with late HDN. The rate of late HDN (often manifested by sudden central nervous system hemorrhage) ranges from 4.4 to 7.2 per 100 000 births based on reports from Europe and Asia. When a single dose of oral vitamin K has been used as neonatal prophylaxis, the rate has decreased to 1.4 to 6.4 per 100 000 births. Parenteral neonatal vitamin K prophylaxis prevents the development of late HDN, with the rare exception of infants with severe malabsorption syndromes. Oral regimens that have a similar efficacy as parenteral vitamin K in prevention of late HDN include the repeated administration of oral vitamin K1 (Germany) or K2 (Japan) at birth, 1 week, and 2 to 4 weeks. In 1990 Golding et al2 reported a study of a 1970 birth cohort in Britain in which they noted an unexpected association between childhood cancer and pethidine given in labor and the neonatal administration of vitamin K. Subsequently, Golding and others3 conducted a case-control study designed to examine the risk of cancer associated with intramuscular vitamin K administration among infants born in two hospitals in Avon between 1965 and 1987 and diagnosed with cancer between 1971 and 1989.


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