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

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.

Risk Assessment For Readmission For Phototherapy in Neonates With ABO Hemolytic Disease

Background: ABO hemolytic disease of the newborn (ABO HDN) is a main risk factor for neonatal hyperbilirubinemia, which is one of the most common causes for readmission in neonates after discharge. Our objective is to assess the risk factors for readmission in neonates with ABO hemolytic disease for phototherapy.Methords: 291 neonates at gestational age ≥ 35 weeks were enrolled with the diagnosis of ABO hemolytic disease by collecting their clinical and laboratory data retrospectively. All these infants were born in Women’s Hospital School of Medicine Zhejiang University between 2018 and 2019. . Among these neonates, 36 cases were readmitted due to hyperbilirubinemia, which is defined as the study group, while the other 255 cases as the control group.Results: The study and control groups were similar on maternal and infants basic parameters (P> 0.05), as well as the complications of both infants and mothers (P> 0.05). However, we found significant differences in the concentration of initial total serum bilirubin, the onset age for phototherapy, the positive direct antiglobulin test (DAT) between two groups (P <0.05). Logistic regression analysis suggested that the age for onset phototherapy and the initial level of total serum bilirubinwere both independent risk factors for readmission in neonates with ABO hemolytic disease.Conclusions: For neonates with hyperbilirubinemia due to ABO HDN, positive direct antiglobulin test (DAT), small age for phototherapy and high initial level of bilirubin can increase the risk of readmission for phototherapy.

Severe Hemolytic Disease of Newborn Due to Alloimmune Anti-E Antibodies: A Case Report

Hemolytic disease in the newborn (HDN) as a cause of early jaundice is mostly due to Rh (D), ABO incompatibility, and rarely due to other minor blood group incompatibility. We report case of Rh anti-E isoimmunization presenting as significant unconjugated jaundice within the 24 h of life. Baby presented with severe jaundice and anemia on day 1 of life. Baby was treated with intensive phototherapy, double volume exchange transfusion (DVET), and intravenous immunoglobulins. On evaluation, both mother and baby had O positive (Rh) blood group; however, the infant showed evidence of severe hemolysis. Positive direct Coombs test (DCT) and 11 cell antibody panel showed anti-E antibodies. This case highlights the importance of early identification and evaluation of HDN in the absence of Rh(D) and ABO incompatibility and possibility of severe hemolysis in Rh anti-E isoimmunization needing DVET.