Vitamin K Deficiency in Newborns: A Case Report in α-1-Antitrypsin Deficiency and a Review of Factors Predisposing to Hemorrhage

PEDIATRICS ◽  
1984 ◽  
Vol 73 (5) ◽  
pp. 712-716
Author(s):  
Nathaniel R. Payne ◽  
Duane K. Hasegawa
Keyword(s):  
Prothrombin Time ◽  
Vitamin K ◽  
Partial Thromboplastin Time ◽  
Signs And Symptoms ◽  
Serum Bilirubin Level ◽  
Intracerebral Hematoma ◽  
Cholestatic Liver Disease ◽  
Vitamin K Deficiency ◽  
Antitrypsin Deficiency ◽  
K Deficiency

A 4-week-old, breast-fed female infant appeared healthy until signs and symptoms of CNS deterioration suddenly occurred. At presentation the infant was found to have a left-sided parietal intracerebral hematoma, markedly prolonged prothrombin time, and partial thromboplastin time, normal platelet count, and jaundice with a total and direct serum bilirubin level of 5.4 mg/dL and 2.6 mg/dL, respectively. Vitamin K1 and fresh frozen plasma returned the prothrombin time and partial thromboplastin time to normal values within 18 hours, suggesting that the infant had severe vitamin K deficiency complicated by intracerebral hemorrhage. Evaluation of the infant's direct hyperbilirubinemia led to the diagnosis of homozygous (pi-type ZZ [PiZZ]) α-1-antitrypsin deficiency. The clinical circumstances predisposing to vitamin K deficiency in newborns and infants are discussed. Based on our observations in this case, we suggest that cholestatic liver disease should be suspected when unexplained vitamin K deficiency occurs in early infancy. The role of vitamin K in hemostasis and the laboratory diagnosis of vitamin K deficiency are discussed as they apply to the evaluation of hemorrhage in newborns and infants.

scholarly journals Vitamin K deficiency-induced spontaneous haemopericardium and cardiac tamponade in an infant with alpha-1 antitrypsin deficiency: a case report

2020 ◽  
Author(s):  
Christoph Bauer ◽  
Désirée Furthner ◽  
Eva Grohmann ◽  
Gerald Tulzer
Keyword(s):  
Cardiac Tamponade ◽  
Vitamin K ◽  
Cholestatic Liver Disease ◽  
Vitamin K Deficiency ◽  
Vitamin K Deficiency Bleeding ◽  
Young Infants ◽  
Antitrypsin Deficiency ◽  
Alpha 1 Antitrypsin Deficiency ◽  
K Deficiency ◽  
Alpha 1 Antitrypsin

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.

Vitamin K Deficiency

PEDIATRICS ◽  
1985 ◽  
Vol 75 (2) ◽  
pp. 373-374
Author(s):  
NATHANIEL R. PAYNE ◽  
DUANE K. HASEGAWA
Keyword(s):  
Liver Disease ◽  
High Risk ◽  
Vitamin K ◽  
Late Onset ◽  
Cholestatic Liver Disease ◽  
Hemorrhagic Disease ◽  
Vitamin K Deficiency ◽  
Antitrypsin Deficiency ◽  
K Deficiency

In Reply.— We agree with Aballi that a combination of factors placed our patient1 at high risk for vitamin K deficiency during her first month of life. These factors include decreased intestinal absorption of vitamin K due to the cholestatic liver disease of α-1-antitrypsin deficiency and a diet consisting solely of breast milk which is known to have little vitamin K. Aballi correctly points out the lack of data on the role of vitamin K in preventing late-onset hemorrhagic disease.

Factor VII Activity and Antigen in Haemophilia B Variants

1980 ◽  
Vol 43 (01) ◽  
pp. 016-019 ◽  
Author(s):  
M G Mazzucconi ◽  
R M Bertina ◽  
D Romoli ◽  
M Orlando ◽  
G Avvisati ◽  
...  
Keyword(s):  
Prothrombin Time ◽  
Vitamin K ◽  
Normal Values ◽  
Factor Ix ◽  
Factor Vii ◽  
Confidence Limits ◽  
Vitamin K Deficiency ◽  
Haemophilia B ◽  
K Deficiency ◽  
Mild Deficiency

SummaryTwenty three patients belonging to 18 different pedigrees of Haemophilia B were studied with regard to ox-brain prothrombin time and its correlation to factor VII.Eleven among them were B-negative (no detectable factor IX antigen), five were B-reduced (factor IX antigen detectable but below the normal values) and seven were B-positive (normal levels of factor IX antigen).Ox-brain prothrombin time was found prolonged (≥ x̄ + 2.5 SD:99% confidence limits) in nine patients. Factor VII Activity (VII: C) was found reduced in 1/11 B-negative, in 2/5 B-reduced and in 4/7 B-positive patients. Factor VII Antigen (VII: Ag) was found normal in all but one patient.The ratio VII:C/VII:Ag was abnormal in eight patients independently from the variant of Haemophilia B. The underlying defect which causes the prolongation of Ox-brain prothrombin time due to factor VII: C mild deficiency is heterogeneous. Age, a mild Vitamin K deficiency, the presence of an inhibitor of Factor VII activation and other unknown causes, may be responsible for this pattern.

Bleeding in Cystic Fibrosis?

PEDIATRICS ◽  
1984 ◽  
Vol 73 (6) ◽  
pp. 877-878
Author(s):  
PETER A. LANE ◽  
W. E. HATHAWAY
Keyword(s):  
Cystic Fibrosis ◽  
Biliary Atresia ◽  
Vitamin K ◽  
Blood Smear ◽  
Peripheral Blood Smear ◽  
Vitamin K Deficiency ◽  
Sweat Chloride ◽  
Antitrypsin Deficiency ◽  
K Deficiency ◽  
Fat Malabsorption

In Reply.— We agree with Rosenstein that the possibility of fat malabsorption should be considered in any infant with vitamin K deficiency. In addition to cystic fibrosis, biliary atresia,1 α-1-antitrypsin deficiency,2 and abetalipoproteinemia3 have all been seen in infancy with vitamin K deficient hemorrhage. Certainly, a sweat chloride test, fractionated bilirubin determination, and review of the peripheral blood smear for acanthocytes would be important in the evaluation of such patients. Our patient had no acanthocytes, a normal α-1-antitrypsin level, and no evidence of biliary atresia or cystic fibrosis at autopsy.

Evidence against Vitamin K Deficiency in Normal Neonates

1980 ◽  
Vol 44 (03) ◽  
pp. 159-160 ◽  
Author(s):  
R G Malia ◽  
F E Preston ◽  
V E Mitchell
Keyword(s):  
Prothrombin Time ◽  
Umbilical Cord ◽  
Vitamin K ◽  
Clotting Factors ◽  
Vitamin K Deficiency ◽  
Term Neonates ◽  
Prolonged Prothrombin Time ◽  
K Deficiency ◽  
Low Levels ◽  
Normal Neonates

SummaryUmbilical cord plasmas from 24 normal full-term neonates were studied. We confirmed the prolonged prothrombin time and low levels of Vitamin K dependent clotting factors previously described (1), but using a number of recently developed immunological and coagulation techniques we could find no evidence of vitamin K deficiency. We conclude that the decreased levels of vitamin K dependent clotting factors of normal neonates are the result of decreased synthesis by the immature liver and that it is questionable whether vitamin K prophylaxis is necessary for these infants.

Placental Transfer of Vitamin K1 and Its Implications in Fetal Hemostasis

1988 ◽  
Vol 60 (01) ◽  
pp. 039-043 ◽  
Author(s):  
L Mandelbrot ◽  
M Guillaumont ◽  
M Leclercq ◽  
J J Lefrère ◽  
D Gozin ◽  
...  
Keyword(s):  
Vitamin K ◽  
High Performance ◽  
Ultrasound Guidance ◽  
Placental Transfer ◽  
Vitamin K1 ◽  
Vitamin K Deficiency ◽  
Prothrombin Activity ◽  
Oral Supplementation ◽  
K Deficiency ◽  
The Mean

SummaryVitamin K status was evaluated using coagulation studies and/ or vitamin IQ assays in a total of 53 normal fetuses and 47 neonates. Second trimester fetal blood samples were obtained for prenatal diagnosis under ultrasound guidance. Endogenous vitamin K1 concentrations (determined by high performance liquid chromatography) were substantially lower than maternal levels. The mean maternal-fetal gradient was 14-fold at mid trimester and 18-fold at birth. Despite low vitamin K levels, descarboxy prothrombin, detected by a staphylocoagulase assay, was elevated in only a single fetus and a single neonate.After maternal oral supplementation with vitamin K1, cord vitamin K1 levels were boosted 30-fold at mid trimester and 60 fold at term, demonstrating placental transfer. However, these levels were substantially lower than corresponding supplemented maternal levels. Despite elevated vitamin K1 concentrations, supplemented fetuses and neonates showed no increase in total or coagulant prothrombin activity. These results suggest that the low prothrombin levels found during intrauterine life are not due to vitamin K deficiency.

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