1/72. Late-form hemorrhagic disease of the newborn: a fatal case report with illustration of investigations that may assist in avoiding the mistaken diagnosis of child abuse.Hemorrhagic disease of the newborn (HDN) is usually a self-limiting hemorrhagic disorder of childhood that occurs as a result of vitamin k deficiency. It may be defined as early or late form depending on the time of onset related to birth. HDN is recognized as one of several bleeding disorders that can mimic the findings of nonaccidental head injury and may lead to a mistaken diagnosis of child abuse. We present a single fatal case of late-onset HDN with illustration of hematologic assays that can be performed to assist the pathologist in making the correct diagnosis of HDN.- - - - - - - - - - ranking = 1keywords = vitamin, deficiency (Clic here for more details about this article) |
2/72. 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.- - - - - - - - - - ranking = 3.4931885829002keywords = vitamin, deficiency (Clic here for more details about this article) |
3/72. Intracranial haemorrhage due to vitamin k deficiency following gastroenteritis in an infant.Breastfed infants who are not given supplemental vitamin K after birth may develop vitamin k deficiency following gastroenteritis. Severe intracranial haemorrhage may occur.- - - - - - - - - - ranking = 5.8356038056999keywords = vitamin, deficiency (Clic here for more details about this article) |
4/72. Intracranial hemorrhage in an infant owing to vitamin k deficiency despite prophylaxis.A 3-month-old male infant with intracranial hemorrhage attributable to a vitamin K deficiency is reported. Vitamin K2 was administered orally at birth and then at 5 days and I month of age. Oral antibiotics were also given 2 days before the onset of bleeding. Although the incidence of intracranial hemorrhage resulting from vitamin k deficiency has decreased since the introduction of vitamin K2 prophylaxis, spontaneous intracranial hemorrhages are still being reported in infants. We suggest that vitamin K prophylaxis is needed especially for breast-fed infants and for those undergoing antibiotic therapy.- - - - - - - - - - ranking = 7.6712076113999keywords = vitamin, deficiency (Clic here for more details about this article) |
5/72. Alpha1-antitrypsin deficiency with fatal intracranial hemorrhage in a newborn.A 4-week-old boy had a fatal intracranial hemorrhage resulting from vitamin k deficiency. The infant had received no vitamin K prophylaxis and was exclusively breastfed. At autopsy, examination of the liver showed cholestasis and fibrosis. dna was isolated from a blood spot on a Gutherie sample card obtained from the infant for routine metabolic screening. This dna was used for alpha1-antitrypsin genotyping studies. Genotyping studies identified homozygosity for the point mutation 9989G-->A, confirming a diagnosis of alpha1-antitrypsin deficiency (ZZ phenotype), and resulted in appropriate screening of siblings born after this child's death. Alpha1-antitrypsin deficiency should be considered in the differential diagnosis of infants with late hemorrhagic disease of the newborn. Use of blood from the metabolic screening card as a source of dna allowed confirmation of this diagnosis after the infant's death.- - - - - - - - - - ranking = 2.8219809715003keywords = vitamin, deficiency (Clic here for more details about this article) |
6/72. Severe acquired vitamin k deficiency: a hypothesis for rapid response to therapy.The potential mechanism underlying the rapid response to vitamin K replacement in acquired deficiency states is incompletely understood. To examine vitamin K metabolism, a 10-year-old boy with autoimmune enteropathy on oral vitamin K supplementation, who presented with profuse nosebleeds and calf tenderness, was evaluated. Laboratory analyses were consistent with severe vitamin k deficiency: vitamin K dependent protein (VKDP) levels < 5%, normal vitamin K epoxide level and depressed total prothrombin antigen (carboxylated and undercarboxyated forms). Intramuscular vitamin K (10 mg) was administered. Nine hours following therapy, VKDP levels corrected completely. Total prothrombin antigen increased indicating new prothrombin synthesis. However, the increase in the prothrombin-clotting assay far exceeded the increase in total prothrombin, supporting storage of undercarboxylated prothrombin in vitamin k deficiency states, with carboxylation and secretion after vitamin K replacement. Although this mechanism is known to occur in rodents, it has not been reported in humans. Our findings suggest a new potential mechanism of prothrombin metabolism in humans.- - - - - - - - - - ranking = 12.0136228342keywords = vitamin, deficiency (Clic here for more details about this article) |
7/72. protein c deficiency in microvascular head and neck reconstruction.OBJECTIVE: To determine the incidence of perioperative protein c deficiency in patients undergoing free flap reconstruction of cancer-related defects in the head and neck. STUDY DESIGN: Prospective case series. INTERVENTION: Ten patients underwent microvascular reconstruction after surgical therapy of carcinomas of the oral cavity or oropharynx. Coagulation studies were determined in all patients 72 hours after surgery. SETTING: Academic tertiary care medical center RESULTS: protein c deficiency was detected in 70% of patients. One free flap failure was attributed to protein c deficiency. CONCLUSIONS: Vitamin K-dependent clotting factors are frequently deficient during the postoperative period after major head and neck surgery, which may result in a state of hypercoagulability. protein c deficiency should be considered as a possible cause of free flap thrombosis in patients who undergo microvascular head and neck reconstruction.- - - - - - - - - - ranking = 1.3151695544005keywords = deficiency (Clic here for more details about this article) |
8/72. 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.- - - - - - - - - - ranking = 5.8356038056999keywords = vitamin, deficiency (Clic here for more details about this article) |
9/72. Parietal pseudofracture and spontaneous intracranial hemorrhage suggesting nonaccidental trauma: report of 2 cases.Massive intracranial hemorrhage, no history of trauma and radiographic findings that were initially interpreted as linear parietal fractures raised the possibility of nonaccidental trauma in 2 infants. Both had severe coagulopathy, 1 due to hemorrhagic disease of the newborn (vitamin k deficiency) and the other due to disseminated herpes simplex virus infection. Both infants died. At autopsy, the parietal bone abnormalities were not fractures, but proved to be an anomalous suture in 1 and a connective tissue fissure in the other.- - - - - - - - - - ranking = 1keywords = vitamin, deficiency (Clic here for more details about this article) |
10/72. calciphylaxis associated with cholangiocarcinoma treated with low-molecular-weight heparin and vitamin K.calciphylaxis is a rare disorder of small-vessel calcification and cutaneous infarction associated with chronic renal failure. Rare cases of calciphylaxis not associated with chronic renal failure have been reported with breast cancer, hyperparathyroidism, and alcoholic cirrhosis. To our knowledge, we report the first case of calciphylaxis without chronic renal failure associated with cholangiocarcinoma and the first attempt to treat calciphylaxis with vitamin K. A 56-year-old woman presented with necrotic leg ulceration. She was treated initially with low-molecular-weight heparin, with no effect. A coagulation work-up showed vitamin k deficiency. During vitamin K therapy, the patient had fulminant progression of the calciphylaxis. She died, and an autopsy showed metastatic cholangiocarcinoma. thrombosis and protein c deficiency have been implicated in the pathophysiology of calciphylaxis. Functional protein c deficiency may be one of several factors contributing to the development of calciphylaxis. Vitamin K therapy was ineffective in our patient and may have been detrimental.- - - - - - - - - - ranking = 6.3424152227998keywords = vitamin, deficiency (Clic here for more details about this article) |
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