Cases reported "Vitamin K Deficiency"

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1/15. vitamin k deficiency with hemorrhage after kidney and combined kidney-pancreas transplantation.

    vitamin k deficiency is a common occurrence in the surgical and intensive care unit population, but its incidence in kidney and combined kidney-pancreas allograft recipients has not been described. We report four patients who received cadaveric kidney or combined kidney-pancreas allografts and subsequently developed significant bleeding associated with deficiency of vitamin K. Their coagulopathy promptly resolved with the parenteral administration of vitamin K. Treatment with vitamin K should be considered in kidney or combined kidney-pancreas allograft recipients with a prolonged prothrombin or partial thromboplastin time during the first postoperative week to avoid hemorrhagic complications.
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2/15. Novel mutation in the gamma-glutamyl carboxylase gene resulting in congenital combined deficiency of all vitamin K-dependent blood coagulation factors.

    A mutation in the gamma-glutamyl carboxylase gene leading to a combined congenital deficiency of all vitamin K-dependent coagulation factors was identified in a Lebanese boy. He is the first offspring of consanguineous parents and was homozygous for a unique point mutation in exon 11, resulting in the conversion of a tryptophan codon (TGG) to a serine codon (TCG) at amino acid residue 501. Oral vitamin K(1) administration resulted in resolution of the clinical symptoms. Screening of several family members on this mutation with an RFLP technique revealed 10 asymptomatic members who were heterozygous for the mutation, confirming the autosomal recessive pattern of inheritance of this disease. In 50 nonrelated normal subjects, the mutation was not found. This is the second time a missense mutation in the gamma-glutamyl carboxylase gene is described that has serious impact on normal hemostasis.
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3/15. Accidental administration of oxytocin to a premature infant.

    oxytocin has been used for several decades in close proximity to newborns, yet no published information is available regarding complications associated with its accidental administration to a newborn. We describe a case where oxytocin instead of vitamin K was accidentally administered intramuscularly to a premature infant shortly after birth. The patient described remained hemodynamically stable but developed transient hyponatremia as the sole biochemical abnormality.
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4/15. skin necrosis and protein c deficiency associated with vitamin K depletion in a patient with renal failure.

    skin necrosis similar to that induced by warfarin was seen in a patient who had never received the drug but who was vitamin K-deficient due to malnutrition and prolonged treatment with broad-spectrum antibiotics. He also had end-stage renal failure and was receiving prophylactic subcutaneous heparin therapy because of immobilization. His plasma protein C antigen level and, disproportionately, his plasma protein C functional activity were decreased. Both protein C values improved after vitamin K therapy, discontinuation of heparin, and initiation of hemodialysis. We surmise that skin necrosis occurred as a result of protein c deficiency caused by vitamin K depletion. Production of abnormal (descarboxy) protein C/protein s due to vitamin k deficiency and increased protein C inhibitory activity associated with renal failure and/or heparin administration may have contributed to the clinical picture. This rare but serious complication of a relatively common disorder, viz., vitamin k deficiency, reinforces the importance of vitamin K supplementation in malnourished patients who receive long-term antibiotic maintenance therapy.
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5/15. Bleeding disorder as the first symptom of biliary atresia.

    biliary atresia (BA) is occasionally diagnosed in infants whose first symptom is a bleeding disorder, such as intracranial bleeding, nasal bleeding or gastrointestinal bleeding. The authors describe 3 cases in which a bleeding disorder was the first symptom of BA. The presenting symptom was intracranial bleeding in a male on day 55 after birth, nasal bleeding in a female at 65 days, and gastrointestinal bleeding in a female at 25 days. Coagulation studies revealed a vitamin k deficiency in all patients. After the administration of vitamin K, the results of coagulation tests normalized and the bleeding tendency of the infants ceased. Subsequently, BA was suspected to be the cause of these bleeding disorders based on imaging findings. BA should therefore be considered in all infants with sudden onset of a tendency to bleed.
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6/15. Hemolytic uremic syndrome complicated by vitamin k deficiency.

    A 5-year-old child with hemolytic uremic syndrome developed bleeding due to vitamin k deficiency 9 days after the onset of a diarrheal prodrome. vitamin k deficiency was documented by rapid correction of the PT and PTT and cessation of bleeding following administration of vitamin K, as well as by the detection of noncarboxylated prothrombin in plasma. The case is instructive because it suggests that previously healthy older children who become acutely ill may develop vitamin k deficiency more rapidly than heretofore has been appreciated.
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7/15. Hazards of small amounts of heparin in a patient with subclinical vitamin k deficiency.

    The use of small amounts of a dilute solution of heparin (less than or equal to 100 IU) to keep indwelling intravenous needles or catheters patent for intermittent venous access either for intravenous therapy or timed blood sampling is a common clinical practice. It is considered safe since the amount of heparin required is much less than that required for heparinization. Herein, we describe a 13-yr-old patient with malabsorption who developed clinically significant bleeding shortly after a diagnostic test which required multiple small injections of heparin for intermittent venous access (total amount of heparin administered was 600 units over 5 hr). The coagulopathy was corrected by a single dose (10 mg) of parenteral vitamin K. As our patient had multiple risk factors for the development of vitamin k deficiency including malabsorption, decreased food intake, and antibiotic use, we postulate that the small amount of heparin precipitated the coagulopathy by increasing the antiprotease activity of antithrombin iii on abnormal factors X and II formed in the vitamin K deficient state. We would therefore recommend administration of vitamin K to patients who are at risk of developing vitamin k deficiency before using even small amounts of heparin.
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8/15. Severe deficiency of vitamin K dependent coagulation factors in an infant.

    A severe deficiency of vitamin K dependent coagulation factors presenting 25 days after birth in an apparently healthy breast-fed infant is reported. The administration of antibiotics to the lactating mother, and lack of a vitamin K supplement to the new-born baby were possible predisposing factors.
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9/15. Effects of N-methyl-thiotetrazole cephalosporin on haemostasis in patients with reduced serum vitamin K1 concentrations.

    Two patients with low random serum vitamin K1 concentrations but with normal prothrombin times and normal biological assays of the vitamin K dependent coagulation proteins were treated with an N-methyl-thiotetrazole cephalosporin (cefotetan) postoperatively. Four to six days later both patients developed a prolonged prothrombin time and a noticeable and specific lowering of the clotting activities of factors II, VII, IX and X, though the serum vitamin K1 concentrations remained unchanged. Crossed immunoelectrophoresis of prothrombin showed the appearance of a second peak corresponding to descarboxyprothrombin (PIVKA II). These abnormalities corrected after vitamin K administration. These data are consistent with the hypothesis that cephalosporins with an N-methyl-thiotetrazole side chain inhibit the hepatic utilisation of vitamin K but that this only causes hypoprothrombinaemia when liver reserves of vitamin K are low.
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10/15. association of congenital deficiency of multiple vitamin K-dependent coagulation factors and the phenotype of the warfarin embryopathy: clues to the mechanism of teratogenicity of coumarin derivatives.

    We have evaluated a boy who had excessive bleeding and bruising from birth and showed markedly prolonged prothrombin times, partially correctable by oral vitamin K administration. Additional laboratory studies demonstrated decreased activities of plasma factors II, VII, IX, and X; near normal levels of immunologically detected and calcium binding-independent prothrombin; undercarboxylation of prothrombin; excess circulating vitamin K epoxide; decreased excretion of carboxylated glutamic acid residues; and abnormal circulating osteocalcin. These results all are consistent with effects resulting from decreased posttranslational carboxylation secondary to an inborn deficiency of vitamin K epoxide reductase. This individual also had nasal hypoplasia, distal digital hypoplasia, and epiphyseal stippling on infant radiographs, all of which are virtually identical to features seen secondary to first-trimester exposure to coumarin derivatives. Therefore, by inference, the warfarin embryopathy is probably secondary to warfarin's primary pharmacologic effect (interference with vitamin K-dependent posttranslational carboxylation of glutamyl residues of various proteins) and may result from undercarboxylation of osteocalcin or other vitamin K-dependent bone proteins.
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