Exacerbation of Neonatal Hemolysis and Impaired Renal Iron Handling in Heme Oxygenase 1-Deficient Mice.

In most mammals, neonatal intravascular hemolysis is a benign and moderate disorder that usually does not lead to anemia. During the neonatal period, kidneys play a key role in detoxification and recirculation of iron species released from red blood cells (RBC) and filtered out by glomeruli to the primary urine. Activity of heme oxygenase 1 (HO1), a heme-degrading enzyme localized in epithelial cells of proximal tubules, seems to be of critical importance for both processes. We show that, in HO1 knockout mouse newborns, hemolysis was prolonged despite a transient state and exacerbated, which led to temporal deterioration of RBC status. In neonates lacking HO1, functioning of renal molecular machinery responsible for iron reabsorption from the primary urine (megalin/cubilin complex) and its transfer to the blood (ferroportin) was either shifted in time or impaired, respectively. Those abnormalities resulted in iron loss from the body (excreted in urine) and in iron retention in the renal epithelium. We postulate that, as a consequence of these abnormalities, a tight systemic iron balance of HO1 knockout neonates may be temporarily affected.

Calcium bilirubinate sludge causes early onset of congenital biliary dilatation: a report of two cases.

BACKGROUND: Symptomatic congenital biliary dilatation (CBD) during early infancy is always characterized by cystic dilation of the common bile duct with a narrow segment connecting the pancreatic duct. CASE PRESENTATION: In two consecutive infants with a prenatal diagnosis of CBD, we found that biliary sludge had formed in the cyst upon the appearance of symptoms including acholic stool and hypertransaminasemia. Infrared absorption spectrometry revealed that the sludge consisted of calcium bilirubinate. CONCLUSION: We suggest that overproduction of bilirubin by neonatal hemolysis causes sedimentation of bilirubin calcium, resulting in obstruction of the narrow segment and development of symptoms.

A correction formula for neuron-specific enolase measurement in hemolyzed neonatal serum samples.

As a specific biomarker in neonatal hypoxic-ischemic encephalopathy (HIE), the measurement of neuron-specific enolase (NSE) has been advocated as a predictor of outcome in neurological injury. However, the measured levels of NSE may be influenced by hemolysis. In the current study, the change in the concentration of NSE in serum was measured by chemiluminescence prior to and following the addition of individual frozen and thawed red blood cells from 86 neonates that were collected within 24 h of birth. The changes in the concentration of NSE were compared with the changes in the concentration of hemoglobin (Hb), measured by the hemoglobin cyanide (HiCN) method, to establish a correction formula. The performance of the correction formula was evaluated by comparing the corrected concentration of NSE using the individual constants and the correction formula. The average individual constant of NSE from the 86 hemolyzed neonatal serum samples was 25.15±3.94 mg/g Hb. The concentration variation between NSE and Hb in neonatal sera could be described by the equation ΔNSEserum=1.8594+24.0670 xΔHbHiCN (r2=0.8045, P<0.001). There was no statistically significant difference in the NSE corrected results between the individual constant group and the correction formula group (Z=-1.645, P=0.100). The linear regression formula of Hb measured with the instrumental method compared with the HiCN method was Hbinstr=0.9816×HbHiCN+0.5596 (r2=0.9924, P<0.001). Based on these regression analyses, the correction formula for NSE in hemolyzed neonatal serum was determined as NSEcorr=NSEmeas-24.0670×HbHiCN-1.8594 or NSEcorr=NSEmeas-24.5181×Hbinstr+11.8609. In conclusion, hemolysis has a substantial influence on the accurate measurement of NSE in neonatal serum samples. For hemolyzed neonatal serum samples, correcting the NSE results using a correction formula is essential to evaluate the severity of neonatal hypoxic ischemic encephalopathy.

Diverse hematological phenotypes of ß-thalassemia carriers.

Most ß-thalassemia carriers have mild anemia, low mean corpuscular volume and mean corpuscular hemoglobin, and elevated hemoglobin α2 (HbA2 ). However, there is considerable variability resulting from coinheritance with α- and/or δ-globin gene mutations, dominant inheritance of ß-thalassemia mutations, highly unstable variant globin chains, large deletions removing part or all of the ß-globin gene cluster, loss of heterozygosity of the ß-globin gene cluster during development, or concomitant erythroid enzyme or membrane protein abnormalities. Recognition of the specific abnormality and correct diagnosis can allay anxiety and unnecessary investigation, help formulate treatment programs, and deliver appropriate genetic and family counseling.

Therapeutic effect of peripheral arteriovenous exchange combined with intravenous gamma globulin on neonatal hemolysis / 中国综合临床

Objective To observe the effect of peripheral arteriovenous exchange combined with intravenous gamma globulin in the treatment of neonatal hemolysis. Methods Seventy children with neonatal hemolysis admitted to the first affiliated Hospital of China Medical University from January 2013 to May 2018 and who met the indications for peripheral arteriovenous exchange were selected as the study subjects. The patients were divided into peripheral arteriovenous exchange group and ＂peripheral arteriovenous exchange+gamma globulin＂ group by random number table method,with 35 cases in each group. Baseline data of the two groups, changes of serum bilirubin before and after treatment, partial blood biochemical indicators, hospitalization time and jaundice regression time were observed. Results The levels of serum bilirubin ((241. 5±48. 1),(184. 6± 26. 3),(166. 3± 18. 5),(133. 5± 20. 8) μmol／L) in peripheral arteriovenous exchange + gamma globulin group were significantly lower than those in peripheral arteriovenous exchange group ((299. 3±32. 5),( 225. 7± 38. 9),(195. 4± 21. 1),( 173. 8± 35. 4) μmol／L) at 12,24,48 and 72 hours after treatment,the difference was significant (P＜0. 05). RBC in children in two groups after treatment was(4. 3±0. 8)×1012／L,(4. 2±1. 0)×1012／L vs. before(5. 2±1. 1)×1012／L,(6. 4±1. 3)×1012／L,Hb after treatment in both groups was (125. 8 ± 11. 2) g／L,( 124. 9 ± 10. 5) g／L vs. before ( 148. 9 ± 26. 5) g／L, (159. 3±14. 6) g／L and reticulocyte count after treatment in both groups were (7. 6±2. 1)%,(7. 3±1. 8)%vs. ( 5. 2 ± 1. 3)%, ( 3. 1 ± 0. 5)% were significantly improved, but the peripheral arteriovenous exchange+gamma globulin group was significantly better than the peripheral arteriovenous transfusion group, the difference was statistically significant ( all P＜0. 05) . The hospitalization time (10. 3±1. 9) d and jaundice regression time ( 8. 6 ± 0. 5) d in the peripheral arteriovenous exchange + gamma globulin group were significantly lower than those in the peripheral arteriovenous exchange group ((15. 5±2. 6) d,(10. 0±1. 1) d) . The difference was statistically significant ( t＝ 9. 553, 6. 855, P＜0. 05) . The children who had re-hemolytic after treatment in the peripheral arteriovenous exchange + gamma globulin group were significantly lower than the peripheral arteriovenous exchange group ( 5. 7%( 2／35) vs. 25. 7%(9／35)),the difference was statistically significant ( χ2 ＝ 5. 285, P ＝ 0. 022 ) . Conclusion Peripheral arteriovenous exchange combined with intravenous gamma globulin is effective in the treatment of neonatal hemolysis. It can significantly reduce serum bilirubin,improve blood biochemical parameters,shorten hospitalization time and jaundice regression time,and is safe and reliable.

Correlation analysis between blood typing test,irregular antibody screening of pregnant women and hemolytic disease of newborn / 中国基层医药

Objective To analyze the correlation between the blood typing test,irregular antibodies screening of pregnant women and hemolytic disease of newborn(HDN).Methods The ABO blood type and Rh(D)blood type of pregnant women were detected,and the irregular antibodies of pregnant women were screened.The blood type serol-ogy and hemolytic disease were detected in neonates with jaundice.Results In specimens of 2 032 pregnant women with type O blood,their husbands were non -O blood type,after delivery,152 neonates were diagnosed as HDN. Among 2 032 pregnant women with type non -O blood,only 5 neonates were diagnosed as HDN.There was significant difference between the two groups (χ2 =135.4,P <0.01).27 pregnant women with RhD negative type,their hus-bands were RhD positive,after delivery,4 neonates were diagnosed as HDN.Conclusion Pregnant women's blood type and irregular antibody are closely related to neonatal hemolysis disease.So pregnant women's blood type and irregular antibody should be regular project in pregnancy,and so as to prevent neonatal hemolytic anemia caused by blood type of feto -maternal incompatibility.

Effects of Gamma Globulin Combined with Phototherapy on Serum Prealbumin and Total Bile Acid of ABO Hemolytic Children / 中国药房

OBJECTIVE:To investigate the effects and safety of gamma globulin combined with phototherapy on serum preal-bumin(PAB)and total bile acid(TBA)of ABO hemolytic children. METHODS:A total of 90 ABO hemolytic children in our hos-pital during Feb. 2014-Sept. 2016 were selected as research objects and divided into observation group and control group according to random number table,with 45 cases in each group. Both groups were given routine active correction of hypoxia and pretreatment for possible hypoglycemia and hypothermia. Control group was additionally given phototherapy with wavelength of 425-475 nm. Ob-servation group was additionally given Human immunoglobulin for intravenous injection (pH4) 1.0g/kg was added to normal saline 10 mL,ivgtt,on the basis of control group and treated for 1 d. The time of jaundice regression,phototherapy duration and hospital-ization time were compared between 2 groups,and the levels of PAB and TBA were compared before and after treatment. The occur-rence of ADR was recorded in 2 groups. RESULTS:The time of jaundice regression,phototherapy duration and hospitalization time in observation group were significantly shorter than control group,with statistical significance(P<0.05). Before treatment,there was no statistical significance in the levels of PAB or TBA between 2 groups(P>0.05). After treatment,the level of PAB was increased significantly in 2 groups,while the level of TBA was decreased significantly;the observation group was significantly better than the control group,with statistical significance(P<0.05). There was no statistical significance in the incidence of ADR between 2 groups (P>0.05). CONCLUSIONS:Gamma globulin combined with phototherapy can significantly shorten the time of clinical symptom im-provement,increase serum level of PAB whlie decrease serum level of TBA in ABO hemolytic children,with good safety.

Relation between antibody titer in pregnant women with maternal-fetal ABO blood incompatibility and hemolytic disease of fetuses and newborns / 中华围产医学杂志

Objective To investigate the relationship between IgG antibody titer in pregnant women with maternal-fetal ABO blood incompatibility and hemolytic disease of fetuses and newborns.Methods From January 31 2009 to January 31 2010,1269 singleton pregnant women who were suspected to have maternal fetal ABO blood incompatibility in Department of Obstetrics and Gynecology,Southwest Hospital,Third Military University were collected.Anti-A or anti-B IgG titers of them were detected at 28-30 gestational age,and umbilical cord blood were taken when delivery and hemolytic disease of the newborn serological test were done to diagnose hemolytic disease of the newborn (HDN).The relationship between the titers and incidence of fetal or neonatal hemolytic disease was retrospectively analyzed by Kendall tau rank correlation.Results No IgG of anti-A or anti-B in serum were found in 58.4％ (741/1269) pregnant women,while the antibody titer of 5.1％ (65/1269) pregnant women were more than or equal to 1 ∶ 128.When they were tested again at 36 gestational week,the titer of 17 cases increased twice but lower than 1 ∶ 512.No signs of intrauterine hemolysis,such as edema,ascites and pleural effusion,were found.Three hundred and eighty neonates (29.9％,380/1269) were diagnosed as HDN.Among which,12 cases (3.2％,12/380) showed mild anemia and (or) jaundice within 24 hours after delivery.There was positive correlation between incidence of neonatal hemolysis and antibody titer(Tb=-0.293,P＜0.01).The incidence of HDN increased from 85.4％ (35/41) in women with antibody titer of 1 ∶ 128 to 5/5 inwomen with antibody titer at 1 ∶ 512 (x2=108.906,P＜0.01).Among 380 HDN neonates,322 cases were transferred to neonatal intensive care unit for phototherapy based comprehensive therapy,and two underwent exchange transfusion.All patients were cured.Conclusions The intrauterine hemolysis incidence of patients with suspected maternal-fetal ABO blood incompatibility is very low,and no special care is required during pregnancy.Anti-A or anti-B tests during pregnancy is helpful in early diagnosis and management of HDN.