Original ArticleTreatment of Neonatal Hemochromatosis with Exchange Transfusion and Intravenous Immunoglobulin
Section snippets
Methods
The subjects of the study were 16 newborn infants with a clinical diagnosis of NH and liver failure. The Pediatric Acute Liver Failure (PALF; http://www.palfstudy.org) study group defines acute liver failure in young children as severe acute liver dysfunction accompanied by hepatic-based coagulopathy uncorrectable by parenteral vitamin K administration in the absence of sepsis-related disseminated intravascular coagulation.15 To meet the diagnostic criteria for coagulopathy in the absence of
Results
The 16 subjects were enrolled between 2004 and 2008 in 7 institutions in the United States and 1 each in The Netherlands and Australia. Table I provides basic clinical data including the results of laboratory testing and the clinical evidence of liver failure in the subjects. The gestational ages of the subjects ranged from 25 to 41 (median, 35) weeks and birth weights from 0.57 to 3.73 (median, 2.28) kg. The degree of coagulopathy qualified all subjects for the diagnosis of liver failure: the
Discussion
The approach to treating NH presented herein represents a major change from current care. Historically, NH has been difficult to treat, given the early onset of liver failure and the generally poor condition of affected infants. No medical therapy has proven effective, and data from recently published series suggest that only the application of liver transplantation can improve survival in NH.5, 13, 14 In the largest of these series, among 19 children with NH treated at King's College London,
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Cited by (129)
Disorders of the Liver
2023, Avery's Diseases of the NewbornDisorders of Iron Overload
2023, MacSween's Pathology of the Liver, Eighth EditionAdvances in medical management of acute liver failure in children: promoting native liver survival
2022, The Lancet Child and Adolescent HealthSuccessful pregnancy outcomes following intravenous immunoglobulin treatment in a woman with a previous fetal death in utero due to gestational alloimmune liver disease: A case report
2022, Case Reports in Women's HealthCitation Excerpt :Rodrigues et al. [5] concluded that chelation-antioxidant therapy in isolation did not improve the life expectancy of neonates with NH and that in their 19 examined cases, liver transplantation achieved the best long-term outcomes. Rand et al. [6] found that exchange transfusions and high doses of IVIg helped to avoid the need for liver transplantation. However, their follow-up time was on average only one year, which limits the strength of this conclusion.
Gestational alloimmune liver disease treated with exchange transfusion and intravenous immunoglobulin: A case study
2022, Transfusion and Apheresis ScienceCitation Excerpt :This hypothesis is based on the observation of the unusual pattern and high rate of recurrence of the disease in the offspring of women who have had one affected infant [4,5]. This principle led to the antenatal treatment of women at risk of having an affected offspring with high-dose intravenous immunoglobulin (IVIG) (to prevent the occurrence of the disease in subsequent pregnancies [2]. Further, a new treatment regimen with the same approach has been used for infants with postnatally established disease.
Neonatal Acute Liver Failure
2020, Clinics in Perinatology
The authors declare no conflicts of interest, real or perceived.