Gestational alloimmune liver disease treated with exchange transfusion and intravenous immunoglobulin: A case study

2021 ◽  
pp. 103347
Author(s):  
Ga Young Park ◽  
Ji In Song ◽  
Sun Hyang Lee ◽  
Seak Hee Oh ◽  
Hyun Sook Hong ◽  
...  
Keyword(s):  
Liver Disease ◽  
Intravenous Immunoglobulin ◽  
Exchange Transfusion ◽  
Immunoglobulin A

Intravenous Immunoglobulin in the Management of Hemolytic Disease of the Newborn

2005 ◽  
Vol 24 (6) ◽  
pp. 17-24 ◽  
Author(s):  
Cynthia Mundy
Keyword(s):  
Side Effects ◽  
Intravenous Immunoglobulin ◽  
Exchange Transfusion ◽  
Clinical Presentation ◽  
Management Strategies ◽  
Additional Treatment ◽  
Blood Type ◽  
Hemolytic Disease ◽  
Current Management

Jaundice caused by hemolysis continues to challenge practitioners caring for infants in the NICU. Bilirubin levels can rise quickly in the first days of life, and interventions must be prompt to prevent side effects related to hyperbilirubinemia. Conventional treatments such as hydration and phototherapy are common, but new studies suggest that use of intravenous immunoglobulin (IVIG) as an additional treatment may prevent the need for exchange transfusion in some babies. This article presents a case study of an infant with blood-type incompatibility treated successfully with multiple doses of IVIG, discusses the pathophysiology and clinical presentation of hemolytic jaundice, and reviews current management strategies for this disease.

scholarly journals Efficacy of Intravenous Immunoglobulin/Exchange Transfusion Therapy on Gestational Alloimmune Liver Disease

2021 ◽  
Vol 9 ◽  
Author(s):  
Pai-Jui Yeh ◽  
Shiu-Feng Huang ◽  
Ming-Chou Chiang ◽  
Chao-Jan Wang ◽  
Ming-Wei Lai
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Liver Failure ◽  
Intravenous Immunoglobulin ◽  
Conventional Therapy ◽  
Exchange Transfusion ◽  
Elevated Serum ◽  
Transfusion Therapy ◽  
First Line Therapy ◽  
High Index Of Suspicion

Background: Gestational alloimmune liver disease (GALD) is a rare but critical cause of neonatal liver failure. After discovering the maternal–fetal alloimmune mechanism, intravenous immunoglobulin (IVIG) with or without exchange transfusion (ET) has gradually replaced antioxidant cocktails as the first-line therapy. Whether such therapy changes the outcome of neonates with GALD is yet to be defined.Method: We reported a pair of twins with discordant presentations, mild and self-limited in the older, whereas liver failure in the younger, who was successfully rescued by ET and IVIG. To investigate the outcome after therapeutic alteration, 39 cases between 2005 and 2020 from literature research were collected.Results: Half of the collected cases (47.1%) were preterm. Common presentations were ascites, jaundice, respiratory distress, hepatomegaly, and edema. Leading laboratory abnormalities were coagulopathy, hypoalbuminemia, and elevated serum ferritin. Salivary gland biopsy and magnetic resonance imaging detected extrahepatic siderosis in 70% (14/20) and 56% (14/25), respectively. IVIG, ET, and liver transplantation were performed in 19 (48.7%), 15 (38.5%), and 8 (20.5%) patients, respectively. The overall survival (OS) rate and native liver survival (NLS) rate were 64.1% (25/39) and 43.6% (17/39), respectively. Although the compiled results did not support a significant benefit, the OS and NLS were higher in the IVIG with/without ET group compared with those treated with conventional therapy [OS (70 vs. 57.9%) and NLS (55 vs. 31.6%), respectively].Conclusion: A high index of suspicion for GALD is crucial when facing a neonate with liver failure. Despite no significant influence on the outcome over conventional therapy in such a rare and detrimental disease, IVIG with or without ET can be worth trying before resorting to liver transplantation, which is resource-demanding and technique-challenging in small infants.

scholarly journals Neonatal hemochromatosis attributed to gestational alloimmune liver disease treated with intravenous immunoglobulin and exchange transfusion therapy: an evidence-based case report

2021 ◽  
Vol 61 (6) ◽  
pp. 350-5
Author(s):  
Adhi Teguh Perma Iskandar ◽  
Vini Jamarin ◽  
Kamajaya Mulyana
Keyword(s):  
Liver Transplantation ◽  
Liver Disease ◽  
Survival Rate ◽  
Iron Overload ◽  
Intravenous Immunoglobulin ◽  
Exchange Transfusion ◽  
Ivig Therapy ◽  
Definitive Treatment ◽  
Transfusion Therapy ◽  
Neonatal Hemochromatosis

Neonatal hemochromatosis (NH) is a rare fatal liver disease accompanied by hepatic and extrahepatic iron overload.1-3 Gestational alloimmune liver disease (GALD) is a materno-fetal alloimmune disorder and leading cause of NH.2,4,5 This condition allows an interplay between the maternal adaptive immune system and the fetus, resulting in an allograft to the mother. The mother becomes sensitized to an alloantigen expressed by the fetus and forms specific reactive antibodies. Immunoglobulin G (IgG) is transported through the placenta and attacks the fetal hepatocytes, resulting in severe loss of hepatocytes and fetal iron overload.3,6 Liver transplantation has been the only definitive treatment for NH for many years, with a survival rate of ±35%. Conventional therapy containing antioxidants and chelation agents reportedly have very poor success, with survival rate of only 10-20%. A new treatment paradigm involving intravenous immunoglobulin (IVIG) and exchange transfusion (ET) therapy has shown significant success in survival rate in NH, decreasing the need for liver transplantation.3,7,8 Here we present a case of NH caused by GALD and treated successfully with a combination of IVIG therapy and ET. We also aimed to evaluate the efficacy of IVIG and ET therapy for NH.

Membranoproliferative Glomerulonephritis and α1-Antitrypsin Deficiency in Children

PEDIATRICS ◽  
1983 ◽  
Vol 71 (1) ◽  
pp. 88-92
Author(s):  
C. Frederic Strife ◽  
George Hug ◽  
Gail Chuck ◽  
A. James McAdams ◽  
Charles A. Davis ◽  
...  
Keyword(s):  
Liver Disease ◽  
Membranoproliferative Glomerulonephritis ◽  
Immunoglobulin A ◽  
Clinical Signs ◽  
Type I ◽  
Complement Protein ◽  
Protein Levels ◽  
Antitrypsin Deficiency ◽  
Glomerular Deposits ◽  
At Deficiency

Two white girls had reduced serum concentration of α1-antitrypsin (α-AT), phenotype ZZ, and liver disease. Hepatocytes exhibited the microscopic criteria of α-AT deficiency. Hypocomplementemia, elevated circulating immune complexes (patient 1), clinical signs of renal disease, and the histologic findings of membranoproliferative glomerulonephritis (MPGN) type I developed. Immunoglobulin A (but not α-AT) was demonstrable immunologically as a component of glomerular deposits in patient 1. Among 53 patients with MPGN but without clinical signs of liver disease, none had Pi type Z. Among 23 patients with phenotype ZZ but without clinical signs of kidney disease, six had abnormal complement protein levels, but the pattern did not resemble that of idiopathic MPGN type I. These results are consistent with the conclusion that MPGN in the two patients reported here is a consequence of their chronic liver disease and is not directly related to the presence of the allelic α-AT variant PiZ.

Su1529 - Serum Immunoglobulin a Levels in Adult Patients with Alcoholic and Non-Alcoholic Fatty Liver Disease

2018 ◽  
Vol 154 (6) ◽  
pp. S-1168
Author(s):  
Evan Elias ◽  
Julia Uhanova ◽  
Kelly Kaita ◽  
Stephen Wong ◽  
David Peretz ◽  
...  
Keyword(s):  
Liver Disease ◽  
Fatty Liver ◽  
Fatty Liver Disease ◽  
Immunoglobulin A ◽  
Adult Patients ◽  
Serum Immunoglobulin ◽  
Alcoholic Fatty Liver ◽  
Alcoholic Fatty Liver Disease

scholarly journals Treatment of Kawasaki Disease with Intravenous Immunoglobulin: A Comparison of the 2GM/KG Regime with a 1 GM/KG Flexible Regime

2003 ◽  
Vol 53 (1) ◽  
pp. 180-180
Author(s):  
Rita Y T Sung ◽  
LY So ◽  
YM Ng ◽  
NC Fong ◽  
MC Yam ◽  
...  
Keyword(s):  
Kawasaki Disease ◽  
Intravenous Immunoglobulin ◽  
Immunoglobulin A

scholarly journals Utilizing Abdominal Sonography in the Diagnosis of Ascites Caused by Heart Failure: A Patient With Cirrhosis

2017 ◽  
Vol 34 (1) ◽  
pp. 50-52
Author(s):  
Spencer Knox ◽  
Mario Madruga ◽  
S. J. Carlan
Keyword(s):  
Heart Failure ◽  
Portal Hypertension ◽  
Liver Disease ◽  
Hepatitis C ◽  
Chronic Liver Disease ◽  
Ascitic Fluid ◽  
Chronic Liver ◽  
Imaging Method ◽  
Abdominal Sonography

Abdominal sonography is the most common imaging method used in the detection of ascites. In the presence of cirrhosis, the most likely etiology of ascites is portal hypertension, secondary to the chronic liver disease. A case study is presented of a male with hepatitis C cirrhosis, with symptoms of ascites, which was confirmed with abdominal sonography. Ascitic fluid obtained by paracentesis confirmed the etiology of the ascites was cardiogenic. Significant improvement was documented after heart failure protocol was implemented. Abdominal sonography can detect the presence of ascites with accuracy but lacks the accuracy to diagnose the precise cause.

Immunoglobulin A in the diagnosis of alcoholic liver disease

1988 ◽  
Vol 94 (2) ◽  
pp. 457-462 ◽  
Author(s):  
Albert van de Wiel ◽  
Jan van Hattum ◽  
Henk-Jan Schuurman ◽  
Louis Kater
Keyword(s):  
Liver Disease ◽  
Alcoholic Liver Disease ◽  
Immunoglobulin A
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