Disseminated Intravascular Coagulation Associated With Hereditary Fructose Intolerance

Maggiore, Giuseppe

doi:10.1001/archpedi.1982.03970380081017

Cited by 3 publications

(6 citation statements)

References 5 publications

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“…In our literature review, we found very few cases reporting DIC occurring in HFI 6,10. In two such cases, DIC presented concurrently with severe liver disease in the setting of elevated transaminases and hepatomegaly.…”

Section: Discussionmentioning

confidence: 95%

“…The mechanism of DIC perpetuated by exposure to fructose occurring in this disorder is not well understood. Previous studies have described DIC occurring in HFI as likely resulting from acute hepatic necrosis causing intravascular coagulation,6 which complicates the ALF as it leads to hepatic coagulopathy perpetuating damage to the liver 10…”

Section: Discussionmentioning

confidence: 99%

“…6,7,9 In our literature review, we found very few cases reporting DIC occurring in HFI. 6,10 In two such cases, DIC presented concurrently with severe liver disease in the setting of elevated transaminases and hepatomegaly. The mechanism of DIC perpetuated by exposure to fructose occurring in this disorder is not well understood.…”

Section: Discussionmentioning

confidence: 99%

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An Infant With Hereditary Fructose Intolerance and a Novel Presentation of Disseminated Intravascular Coagulopathy Following Pyloromyotomy

Aldag

Fan

Marshall

et al. 2022

Journal of Pediatric Hematology/Oncology

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Hereditary fructose intolerance is a rare autosomal recessive metabolic disorder characterized by liver failure, renal tubulopathy, growth retardation, and occasionally death upon exposure to fructose. We present a 2-month-old male infant diagnosed with pyloric stenosis who developed disseminated intravascular coagulopathy following pyloromyotomy. Unexplained persistent coagulopathy, acute liver failure, and metabolic dysfunction led to whole-exome sequencing, which revealed compound heterozygous variants in ALDOB (p.Arg60Ter and p.Ala150Pro), diagnostic of hereditary fructose intolerance. Shortly after initiating a fructose-free diet, our patient had resolution of his coagulopathy, hepatic, and metabolic dysfunction.

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Section: Discussionmentioning

confidence: 95%

Section: Discussionmentioning

confidence: 99%

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An Infant With Hereditary Fructose Intolerance and a Novel Presentation of Disseminated Intravascular Coagulopathy Following Pyloromyotomy

Aldag

Fan

Marshall

et al. 2022

Journal of Pediatric Hematology/Oncology

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“…Independent of the fulminant hepatic failure, DIC has also been reported as an initial presenting feature. 579 Early recognition of hereditary fructose intolerance as the cause of DIC may be lifesaving. 579,580 DIC has also been associated with other meta bolic diseases of the liver, including Reye's syn drome 581 and Wilson's disease.…”

Section: Neonates and Infantsmentioning

confidence: 99%

“…579 Early recognition of hereditary fructose intolerance as the cause of DIC may be lifesaving. 579,580 DIC has also been associated with other meta bolic diseases of the liver, including Reye's syn drome 581 and Wilson's disease. 582 Homocystinuria (usually the result of the auto somal recessive deficiency of cystathione β -synthase) may result in damage to the vascular endothe lium and subendothelium and platelet-mediated intimal proliferation.…”

Section: Neonates and Infantsmentioning

confidence: 99%

Clinical Aspects of Disseminated Intravascular Coagulation: A Clinician's Point of View

Baker¹

1989

Semin Thromb Hemost

151

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DIC is a disorder of coagulation that is demonstrated to occur in many settings. In view of the fulminant nature of the disorder once it is fully activated, the potential for considerable morbidity and mortality, and the difficulty in treating this syndrome, the necessity for a thorough understanding of the disorder is clear. A high index of suspicion is absolutely essential; however, this requires a thorough knowledge of the medical and surgical disorders in which DIC is likely to occur. The presenting manifestations of DIC, the time course of development of the fulminant syndrome, and the specific complications of the disorder may be intrinsically related to the specific clinical characteristics of the underlying disease. Clinical evaluation of the patient must be comprehensive and consider not only the laboratory and clinical manifestations of DIC but the triggering illness. Without the ability to treat the underlying primary illness, controlling or abating the DIC may be difficult or impossible. This comprehensive review of the disease entities in which DIC is likely to occur and the associated clinical manifestations will, it is hoped, guide a more precise definition of the overall clinical picture that confronts the clinician when evaluating a patient with possible DIC.

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Haemophagocytosis in hereditary fructose intolerance: a diagnostic dilemma

Mandel

Gozal

Aizin

et al. 1990

J of Inher Metab Disea

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Disseminated Intravascular Coagulation Associated With Hereditary Fructose Intolerance

Cited by 3 publications

References 5 publications

An Infant With Hereditary Fructose Intolerance and a Novel Presentation of Disseminated Intravascular Coagulopathy Following Pyloromyotomy

An Infant With Hereditary Fructose Intolerance and a Novel Presentation of Disseminated Intravascular Coagulopathy Following Pyloromyotomy

Clinical Aspects of Disseminated Intravascular Coagulation: A Clinician's Point of View

Haemophagocytosis in hereditary fructose intolerance: a diagnostic dilemma

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