The Effect of Prenatal and Postnatal Treatment with Intravenous Immunoglobulin on Severity of Neonatal Hemochromatosis: The Tale of Two Brothers (Case Report)

Samedi, Veronica Mugarab; Ryan, Michelle; Awad, Essa Hamdan Al; Elsharkawy, Adel

doi:10.1055/s-0041-1731311

AJP Rep

2021

DOI: 10.1055/s-0041-1731311

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The Effect of Prenatal and Postnatal Treatment with Intravenous Immunoglobulin on Severity of Neonatal Hemochromatosis: The Tale of Two Brothers (Case Report)

Veronica Mugarab Samedi

Michelle Ryan

Essa Hamdan Al Awad

et al.

Abstract: Background Neonatal hemochromatosis (NH) is a rare condition that was the main reason for liver transplantation in infants. With the realization that NH results from the fetal complement-mediated liver injury, intravenous immunoglobulins (IVIG) were successfully introduced for the treatment. Case Presentation We present two cases of NH from the same family to illustrate the role of antenatal treatment with IVIG in alleviation and possible prevention of this serious morbidity. Conclusion A p… Show more

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Phenytoin/phenobarbital/levetiracetam

2021

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Phenytoin/phenobarbital/levetiracetamGeneralized tonic-clonic seizures: case report A 3-days-old neonate exhibited a lack of efficacy after administering phenytoin, phenobarbital, and levetiracetam for generalised tonic-clonic seizures [routes and dosages not stated].The neonate was born at 29 weeks of gestation via emergency caesarian section. He was intubated and provided a surfactant for respiratory distress. During the following 24 hours, he was kept on ventilator support and eventually developed hypoglycaemia, which was treated with a glucose. The newborn experienced severe hypotension, thrombocytopenia, and increasing hypoxaemia on his second day of life. His ampicillin and gentamicin [gentamycin] therapy started. Next day his health worsened, and he experienced increased abdominal distension with ileus-like symptoms; his liver function deteriorated abruptly; and coagulation tests revealed disseminated intravascular coagulation, despite therapy with fresh frozen plasma, unspecified cryoprecipitate, platelets, and vitamin K. He underwent exploration laparotomy and revealed minor ascites as well as several haemorrhages within the intestinal wall. He experienced generalised tonic-clonic seizures at the age of three days, which were resistant to phenytoin, phenobarbital, and levetiracetam treatment. Despite extensive resuscitation, his haemodynamic instability persisted, and he died at the age of four days. A liver autopsy led to the diagnosis of neonatal haemochromatosis.

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Phenytoin/phenobarbital/levetiracetam

2021

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Neonatal Hemochromatosis: Systematic Review of Prenatal Ultrasound Findings—Is There a Place for MRI in the Diagnostic Process?

Staicu

Popa-Stănilă

Albu

et al. 2023

JCM

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Neonatal hemochromatosis (NH) is an uncommon, severe disorder that results in fetal loss or neonatal death due to liver failure. NH is currently regarded as the phenotypic expression of gestational alloimmune liver disease (GALD). The diagnosis of NH-GALD is rarely prenatally established. In addition to providing a systematic review of the prenatal features that are identifiable using ultrasound (US) and MRI, we suggest a prenatal diagnosis algorithm for use in suspected NH during the first affected pregnancy. From a total of 586 database entries identified in PubMed, Google Scholar, and ResearchGate, we selected 18 studies published from 1993 to 2021 that reported maternal medical and obstetric history, prenatal ultrasound findings, and postpartum outcomes. We investigated the ultrasound and MRI features of these studies, along with the outcome due to this condition. A total of 74 cases were identified. The main reported prenatal US finding was fetal growth restriction (FGR) (33%), followed by oligohydramnios (13%) and hydrops fetalis (13%), with 13% cases described as uneventful. Other rare prenatal findings were fetal anemia, ascites, and abnormal fetal liver and spleen. Most pregnancies ended with fetal/perinatal death or therapeutic interruption of pregnancy. Favorable evolution with treatment (ensanguine transfusion and intravenous immunoglobulin (IVIG)) was reported for only 7% of fetuses. Using T2-weighted MRI, fetal extrahepatic siderosis confirmed prenatally in two cases and postnatally in 11 cases. IVIG treatment throughout subsequent pregnancies was found to significantly improve fetal prognosis. MRI should be indicated in selected cases of oligohydramnios, fetal hydrops, fetal hepatomegaly, ascites, or unexplained FGR or anemia after ruling out all other more frequently encountered conditions. MRI can be used to detect iron overload in the liver and extrahepatic siderosis.

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The Effect of Prenatal and Postnatal Treatment with Intravenous Immunoglobulin on Severity of Neonatal Hemochromatosis: The Tale of Two Brothers (Case Report)

Cited by 2 publications

References 12 publications

Phenytoin/phenobarbital/levetiracetam

Phenytoin/phenobarbital/levetiracetam

Neonatal Hemochromatosis: Systematic Review of Prenatal Ultrasound Findings—Is There a Place for MRI in the Diagnostic Process?

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