Transient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report

Abstract Background In neonates, rhesus D alloimmunization despite anti-D immunoglobulin prophylaxis is rare and often unexplained. Rhesus D alloimmunization can lead to hemolytic disease of the newborn with anemia and unconjugated hyperbilirubinemia. In past reports, transient congenital hyperinsul...

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Autores principales:	Sandra Simony Tornoe Riis, Marianne Hoerby Joergensen, Kristina Fruerlund Rasmussen, Steffen Husby, Jane Preuss Hasselby, Lise Borgwardt, Klaus Brusgaard, Christina Ringmann Fagerberg, Henrik Thybo Christesen
Formato:	article
Lenguaje:	EN
Publicado:	BMC 2021
Materias:	Case report Newborn Rhesus immunization Transient congenital hyperinsulinism Conjugated hyperbilirubinemia Medicine R
Acceso en línea:	https://doaj.org/article/1cac8ce8fe0c48ba8ed6b0f26c0b0ce5
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spelling	oai:doaj.org-article:1cac8ce8fe0c48ba8ed6b0f26c0b0ce52021-11-28T12:37:20ZTransient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report10.1186/s13256-021-03167-91752-1947https://doaj.org/article/1cac8ce8fe0c48ba8ed6b0f26c0b0ce52021-11-01T00:00:00Zhttps://doi.org/10.1186/s13256-021-03167-9https://doaj.org/toc/1752-1947Abstract Background In neonates, rhesus D alloimmunization despite anti-D immunoglobulin prophylaxis is rare and often unexplained. Rhesus D alloimmunization can lead to hemolytic disease of the newborn with anemia and unconjugated hyperbilirubinemia. In past reports, transient congenital hyperinsulinism has been described as a rare complication of rhesus D alloimmunization. Our case report illustrates that rhesus D alloimmunization can result in a pseudosyndrome with severe congenital hyperinsulinism, anemia, and conjugated hyperbilirubinemia, despite correctly administered anti-D immunoglobulin prophylaxis. Case presentation We report of a 36-year-old, Caucasian gravida 1, para 1 mother with A RhD negative blood type who received routine antenatal anti-D immunoglobulin prophylaxis. Her full term newborn boy presented with severe congenital hyperinsulinism, anemia, and conjugated hyperbilirubinemia up to 295 µmol/L (ref. < 9), accounting for 64% of the total bilirubin. Syndromic congenital hyperinsulinism was suspected. Examinations showed a positive direct antiglobulin test, initially interpreted as caused by irregular antibodies; diffuse congenital hyperinsulinism by 18F-DOPA positron emission tomography/computed tomography scan; normal genetic analyses for congenital hyperinsulinism; mildly elevated liver enzymes; delayed, but present bile excretion by Tc99m-hepatobiliary iminodiacetic acid scintigraphy; and cholestasis and mild fibrosis by liver biopsy. The maternal anti-D titer was 1:16,000 day 20 postpartum. Y-chromosome material in the mother’s blood could not be identified. This could, however, not exclude late intrapartum fetomaternal hemorrhage as the cause of immunization. No causative genetic findings were deetrmined by trio whole exome sequencing. The child went into clinical remission after 5.5 months. Conclusion Our case demonstrates that rhesus D alloimmunization may present as a pseudosyndrome with transient congenital hyperinsulinism, anemia, and inspissated bile syndrome with conjugated hyperbilirubinaemia, despite anti-D immunoglobulin prophylaxis, possibly due to late fetomaternal hemorrhage.Sandra Simony Tornoe RiisMarianne Hoerby JoergensenKristina Fruerlund RasmussenSteffen HusbyJane Preuss HasselbyLise BorgwardtKlaus BrusgaardChristina Ringmann FagerbergHenrik Thybo ChristesenBMCarticleCase reportNewbornRhesus immunizationTransient congenital hyperinsulinismConjugated hyperbilirubinemiaMedicineRENJournal of Medical Case Reports, Vol 15, Iss 1, Pp 1-6 (2021)
institution	DOAJ
collection	DOAJ
language	EN
topic	Case report Newborn Rhesus immunization Transient congenital hyperinsulinism Conjugated hyperbilirubinemia Medicine R
spellingShingle	Case report Newborn Rhesus immunization Transient congenital hyperinsulinism Conjugated hyperbilirubinemia Medicine R Sandra Simony Tornoe Riis Marianne Hoerby Joergensen Kristina Fruerlund Rasmussen Steffen Husby Jane Preuss Hasselby Lise Borgwardt Klaus Brusgaard Christina Ringmann Fagerberg Henrik Thybo Christesen Transient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report
description	Abstract Background In neonates, rhesus D alloimmunization despite anti-D immunoglobulin prophylaxis is rare and often unexplained. Rhesus D alloimmunization can lead to hemolytic disease of the newborn with anemia and unconjugated hyperbilirubinemia. In past reports, transient congenital hyperinsulinism has been described as a rare complication of rhesus D alloimmunization. Our case report illustrates that rhesus D alloimmunization can result in a pseudosyndrome with severe congenital hyperinsulinism, anemia, and conjugated hyperbilirubinemia, despite correctly administered anti-D immunoglobulin prophylaxis. Case presentation We report of a 36-year-old, Caucasian gravida 1, para 1 mother with A RhD negative blood type who received routine antenatal anti-D immunoglobulin prophylaxis. Her full term newborn boy presented with severe congenital hyperinsulinism, anemia, and conjugated hyperbilirubinemia up to 295 µmol/L (ref. < 9), accounting for 64% of the total bilirubin. Syndromic congenital hyperinsulinism was suspected. Examinations showed a positive direct antiglobulin test, initially interpreted as caused by irregular antibodies; diffuse congenital hyperinsulinism by 18F-DOPA positron emission tomography/computed tomography scan; normal genetic analyses for congenital hyperinsulinism; mildly elevated liver enzymes; delayed, but present bile excretion by Tc99m-hepatobiliary iminodiacetic acid scintigraphy; and cholestasis and mild fibrosis by liver biopsy. The maternal anti-D titer was 1:16,000 day 20 postpartum. Y-chromosome material in the mother’s blood could not be identified. This could, however, not exclude late intrapartum fetomaternal hemorrhage as the cause of immunization. No causative genetic findings were deetrmined by trio whole exome sequencing. The child went into clinical remission after 5.5 months. Conclusion Our case demonstrates that rhesus D alloimmunization may present as a pseudosyndrome with transient congenital hyperinsulinism, anemia, and inspissated bile syndrome with conjugated hyperbilirubinaemia, despite anti-D immunoglobulin prophylaxis, possibly due to late fetomaternal hemorrhage.
format	article
author	Sandra Simony Tornoe Riis Marianne Hoerby Joergensen Kristina Fruerlund Rasmussen Steffen Husby Jane Preuss Hasselby Lise Borgwardt Klaus Brusgaard Christina Ringmann Fagerberg Henrik Thybo Christesen
author_facet	Sandra Simony Tornoe Riis Marianne Hoerby Joergensen Kristina Fruerlund Rasmussen Steffen Husby Jane Preuss Hasselby Lise Borgwardt Klaus Brusgaard Christina Ringmann Fagerberg Henrik Thybo Christesen
author_sort	Sandra Simony Tornoe Riis
title	Transient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report
title_short	Transient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report
title_full	Transient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report
title_fullStr	Transient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report
title_full_unstemmed	Transient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report
title_sort	transient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus d prophylaxis: a case report
publisher	BMC
publishDate	2021
url	https://doaj.org/article/1cac8ce8fe0c48ba8ed6b0f26c0b0ce5
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Transient congenital hyperinsulinism and hemolytic disease of a newborn despite rhesus D prophylaxis: a case report

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