Bilirubin Encephalopathy Research Articles

Spectrum and outcome of neonatal emergencies seen in a free health-care program in South-Western Nigeria.

Neonatal emergencies contribute significantly to under-five morbidity and mortality in developing countries, partly due to poverty and limited access to quality healthcare in rural communities. The aim is to evaluate the spectrum, outcome, case fatality rates (CFRs), and prognostic factors of neonatal emergencies seen in the two free health-care facilities in Ondo State. This was a cross-sectional descriptive study of neonatal emergencies. Data were collected on eligible consecutive infants using a self-designed questionnaire. Neonatal emergencies were classified based on systemic involvement and underlying causes. Categorized data were expressed as percentages. Outcome and CFRs were presented pictorially. Inferential analysis was performed. The value of P < 0.05 was considered statistically significant. Altogether, 425 infants with neonatal emergencies were recruited, constituting 21.3% of the under-five medical emergencies managed during September 2014 to February 2015. The most frequent emergencies were neonatal sepsis (45.2%) and neurological emergencies, especially hypoxic ischemic encephalopathy (22.1%) and acute bilirubin encephalopathy (14.6%). Furthermore, 6.1% of the infants presented with disseminated intravascular coagulopathy. The outcome of the emergencies was as follows: 88.6% were discharged, 7.4% died, and 3.7% left against medical advice whereas 0.3% were referred for subspecialty services. The leading causes of death among the 32 mortalities in this survey were sepsis (39.5%), hypoxic ischemic encephalopathy (36.9%), and hypothermia (13.2%). Hypothermia, perinatal asphyxia, and hypoglycemia had the highest CFRs, 22.7%, 12.6%, and 11.8%, respectively. Neonatal encephalopathy and sepsis were the most common emergencies seen in the free health-care program. Furthermore, they accounted for a majority of the neonatal deaths. This should be taken into cognizance while designing local interventions for neonatal emergencies.

Exciting Results with Injection Darbepoietin Alfa and Pegfilgrastim in Patients with Decompensated Liver Cirrhosis

1.1.Introduction: Dysregulated erythropoietin (EPO) plasma levels may play a role in the patho physiology of liver cirrhosis. No report of Darbepoetin alpha and Pegylated Filgrastim use in Liver cirrhosis. 1.2.Aims and methods:To study the benefits of Darbepoetin and Pegfilgrastim in patients with de-compensated liver cirrhosis. Prospectively clinical data recorded since October 2014. Patients with active bleed, hepato renal syndrome, hepatoma, portal vessel thrombosis were excluded. Patients started on Injection Darbepoetin alpha 200 microgram and Injection Pegfilgrastim 6 mg subcutaneously every 15 days, total three visits of patients, then three month follow up planned. Improvement in clinical, laboratory parameters analyzed. Median calculated, Wilcoxon Signed-Rank Test applied to compare both groups. 1.3.Results and discussion:N=22 all male, 3 lost to follow up, aetiology of cirrhosis were Non-alcoholic Fatty Liver Disease 5, Hepatitis B Virus 3, Hepatitis C Virus 2 and Alcohol 12. Median age 59 years (range: 40 to 70). Improvement in Haemoglobin from 10.1 gram% (range 5.9-13.4 gm%) to 10.6 gm % (range: 7.5-13.7 gm%) p value 0.00374, Total leukocyte count from 5100/cu mm to 7100/cu mm p value 0.00214, Platelet count 90,000/cu mm to 146,000/cu mm, p value 0.00096, INR 1.7 (range 1.5-4.8) to 1.4 p value 0.00064, Albumin 2.4 gram/dl (range 1.6-2.9) to 2.5 gm/dl (range 1.8-3.5) P value 0.043, Child score from 10 to 8 P value 0.007, ascites score 2 to 1, P value 0.00222. No significant improvement in Serum creatinine, sodium, potassium, calcium, bilirubin, total protein and hepatic encephalopathy. High cost of medicine was the limiting factor. 1.4.Conclusion:Our study suggests that Darbepoetin Alpha and Pegfilgrastim is significantly effective in improving hematology, International normalized ratio, Albumin, ascites and Child score of liver cirrhosis patients.

Modulation of bilirubin neurotoxicity by the Abcb1 transporter in the Ugt1-/- lethal mouse model of neonatal hyperbilirubinemia.

Moderate neonatal jaundice is the most common clinical condition during newborn life. However, a combination of factors may result in acute hyperbilirubinemia, placing infants at risk of developing bilirubin encephalopathy and death by kernicterus. While most risk factors are known, the mechanisms acting to reduce susceptibility to bilirubin neurotoxicity remain unclear. The presence of modifier genes modulating the risk of developing bilirubin-induced brain damage is increasingly being recognised. The Abcb1 and Abcc1 members of the ABC family of transporters have been suggested to have an active role in exporting unconjugated bilirubin from the central nervous system into plasma. However, their role in reducing the risk of developing neurological damage and death during neonatal development is still unknown.To this end, we mated Abcb1a/b-/- and Abcc1-/- strains with Ugt1-/- mice, which develop severe neonatal hyperbilirubinemia. While about 60% of Ugt1-/- mice survived after temporary phototherapy, all Abcb1a/b-/-/Ugt1-/- mice died before postnatal day 21, showing higher cerebellar levels of unconjugated bilirubin. Interestingly, Abcc1 role appeared to be less important.In the cerebellum of Ugt1-/- mice, hyperbilirubinemia induced the expression of Car and Pxr nuclear receptors, known regulators of genes involved in the genotoxic response.We demonstrated a critical role of Abcb1 in protecting the cerebellum from bilirubin toxicity during neonatal development, the most clinically relevant phase for human babies, providing further understanding of the mechanisms regulating bilirubin neurotoxicity in vivo. Pharmacological treatments aimed to increase Abcb1 and Abcc1 expression, could represent a therapeutic option to reduce the risk of bilirubin neurotoxicity.

Clinical profile and outcome of newborns who underwent double volume exchange transfusion in the neonatal intensive care unit at B.P. Koirala Institute of Health Sciences, Nepal

Introduction: About 50% term and 80% preterm babies develop neonatal jaundice. Treatment to prevent bilirubin encephalopathy consists of phototherapy or exchange transfusion (ET). ET is not risk free, and mortality rates vary from 0.5 to 3.3%. Objective: To report our observations on efficacy, adverse events and factors contributing to mortality related with ET performed for the treatment of neonatal hyperbilirubinemia at a tertiary-care centre. Method : This retrospective study was conducted at the neonatology division of B.P. Koirala Institute of Health Sciences, Nepal. All neonates who required ET for jaundice from January 2013 to December 2014 were included. Results : Sixty four neonates underwent double volume ET. Predominant causes of hyperbilirubinaemia were ABO incompatibility (32.8%), Rh incompatibility (17.2%) and idiopathic (20.3%). There was a mean reduction in serum bilirubin levels to 13.1±3.82 mg/dl in the post-ET and 14.63±3.16 mg/dl in the 6 hour post-ET period. The common adverse events were hypocalcaemia in 50% and thrombocytopenia in 35.9% of neonates. Other events such as hypoglycaemia (17.2%), apnoea (18.8%) and mortality (3.1%) occurred following ET. Conclusions : ABO incompatibility was the commonest cause for ET. Most of the complications were transient. Mortality rate was 3.1% in this study.

Guidelines on transfusion for fetuses, neonates and older children.

Guidelines on transfusion for fetuses, neonates and older children.

Effect of c-Jun on hippocampal neuron cell apoptosis in neonatal mice injected with bilirubin encephalopathy

Objective To investigate the effect of c-Jun on hippocampal neuron apoptosis in neonatal mice with bilirubin encephalopathy. Methods Seven-day-old newborn SD rats were selected and injected bilirubin solution (10 g/L) into the cerebellum medulla oblongata pool.The bilirubin level in hippocampus was tested at 6 h and 12 h post-injection and neurons were labeled via Nissl staining; c-Jun amino-terminal kinase(JNK), c-Jun and p-c-Jun level in hippocampus were measured by Western blot and immunofluorescence staining.The expression le-vels of Caspase-3 and Caspase-6 were analyzed by enzyme-linked immunosondent assay and fluorescence spectrophotometry. Results Apart from a certain degree of abnormal behaviors following injection, a sharp rise of (44.00±13.00)%(P<0.05) and (96.86±9.00)%(P<0.01) in hippocampal bilirubin respectively at 6 h and 12 h post-injection.Hippocampal neuron lost and Nissl staining swallowed gradually after 24 h. At the time of 6 h following injecting, c-Jun amino-terminal kinase(JNK), c-Jun and p-c-Jun protein level in hippocampus was separately elevated by 1.4(2.41±0.15 vs 1.00±0.09), 0.43 (1.43±0.16 vs 1.00±0.09) and 1.16 (2.16±0.23 vs 1.00±0.09) fold(all P<0.05), compared with the control group.Immunofluorescent staining showed that c-Jun and p-c-Jun positive cells in hippocampus and cortex all increased after 6 h injection of bilirubin.At the same time, the activity of apoptosis-associated factors downstream Caspase-3 and Caspase-6 initially obviously raised at 12 h post-injection, respectively increased by 2.08 and 0.28 fold of control group after 24 h(all P<0.05). Conclusion The phosphorylation of c-Jun may act as an important activator of hippocampal neuron apoptosis in the bilirubin encephalopathy. Key words: c-Jun N-terminal kinase; c-Jun; Bilirubin encephalopathy; Caspase-3; Caspase-6

Spectrum of neonatal diseases requiring respiratory support in UBTH, Benin City, Edo State, Nigeria

Introduction: In Nigeria, eighty two percent of the three leading causes of neonatal mortality may require respiratory support for their management, yet this is unavailable.Objective: To review the spectrum of respiratory disorders that were ventilated, their outcomes and the contribution of such support to survivalMethods: A data base of cases managed in the unit is maintained and reviewed regularly for enhancement of quality of care. Such prospectively documented information on babies who received respiratory support over the period 1st January to 31st June 2014 were analysed.Results: Five hundred and seventy six babies were admitted. Fortynine (8, 5%) received ventilator support. These comprised preterm (77.6%) and term (22.4%) babies with mean gestational age of 33.2 weeks (range 28-41). The spectrum of disorders supported were: respiratory distress syndrome (RDS) 55.1%, asphyxia 20.4%, sepsis12.3%, acute bilirubin encephalopathy (ABE) 8.2%, transient tachypnoea of the new-born (TTNB) 2% and neonatal tetanus (NT) 2%. They were non- invasive support (NIVRS) 33(67.3%) for respiratory distress; invasive support (IVRS) 16(32.7%) for respiratory failure. NIVRS was mostly for RDS (88.9%); ABE (100%) received invasive support. Mortality was: NIVRS7/33(21.2%); IVRS 11/16(68.75 %). Respiratory dysfunction contributed 25.4% of the total neonatal mortality of 123 per thousand. Survival was 63.3%.Conclusion: The morbidities were: asphyxia, prematurity, sepsis, ABE, and tetanus. Ventilator support was non-invasive with bubble CPAP and invasive with mechanical ventilation through endotracheal intubation. Survival was improved. There is the urgent need to commence and sustain basic non -invasive respiratory support in all neonatal units in Nigeria.Keywords: Spectrum; Neonatal diseases; Respiratory support

Neurological impairment of children with history of prematurity and neonatal hyperbilirubinemia

Background In premature infants, the incidence of hyperbiliru-binemia is still high. Bilirubin encephalopathy can develop withoutmarked hyperbilirubinemia.Objective To know the incidence of neurological impairment inpremature with hyperbilirubinemia and the association betweenneurological impairment and serum unconjugated billirubin con-centration.Methods A retrospective study was conducted on 54 prematureswith history of hyperbilirubinemia and 54 without history of hyper-bilirubinemia born between 1997 and 1998 and discharged fromSanglah Hospital. Consecutive sampling was done. After univariateanalysis, multivariate analysis was used to identify the associationbetween serum unconjugated bilirubin concentration and neuro-logical impairment at the adjusted age of 318 months.Results There were statistically significant differences in mean ofage and neurological impairment between subjects with and with-out hyperbilirubinemia (p&lt;0.0001 and 0.026). In subjects with hy-perbilirubinemia, univariate analysis showed significant differencesin means of serum unconjugated bilirubin concentration, gesta-tional age, birth weight, and serum albumin concentration betweensubject who had neurological impairment and who had no neuro-logical impairment with p = 0.005; 0.001; 0.002; &lt;0.0001, respec-tively. Multivariate analysis found there were association betweenneurological impairment and serum unconjugated bilirubin concen-tration, gestational age, and serum albumin concentration withp&lt;0.0001; 0.004; and &lt;0.0001, respectively.Conclusion Neurological impairment in subject with hyperbiliru-binemia was greater than subject without hyperbilirubinemia. Se-rum unconjugated bilirubin concentration is one of three factorsthat associated with neurological impairment

Study of Cord Blood Bilirubin Levels in a Tertiary Care Centre of Kumaun Region (Uttarakhand) India

Jaundice in newborn is a very common problem. Neonatal hyper bilirubinemia (NH) may lead to kernicterus in otherwise healthy newborns. This can be easily prevented if excessive hyper bilirubinemia for age is promptly identified and appropriately treated. Newborns can be screened for severity of bilirubinemia before hospital discharge which may help in early detection of the newborns at risk for excessive hyper bilirubinemia during the first week of life. To determine the level of cord blood bilirubin in all healthy term new borns and asses its usefulness in predicting neonatal jaundice. Neonatal jaundice (NNJ) is an interesting, complicated and controversial clinical problem. It is a cause of concern for the parents as well as pediatricians. The concept of prediction of jaundice offers an attractive option to pick up babies at risk of neonatal hyper bilirubinemia. Physical examination is not a reliable measure of serum bilirubin. Under these circumstances it would be desirable to be able to predict the risk of jaundice, in order to implement early treatment and thereby minimize the risk of bilirubin dependent brain damage. Neonatal Hyper bilirubinemia has been defined as the bilirubin levels > 12.9 mg/dl in term babies and 15 mg/dl in preterm babies. Neonatal jaundice is visible manifestation in skin and sclera of elevated serum concentrations of bilirubin and this usually occurs in neonates if serum bilirubin level is >5 mg/dl. Most adults are jaundiced when total serum bilirubin (TSB) levels exceed 2.0 mg/dL. Kernicterus and near miss kernicterus are neonatal conditions that are associated with irreversible or reversible brain injury respectively. Concerns regarding jaundice have increased after reports of bilirubin encephalopathy occurring in healthy term infants without hemolysis. This study was conducted in 80 term newborns, a tertiary care center, Department of Pediatrics, Government medical college & Hospital, Haldwani Uttarakhand from July 2013 –Feb 2014. Serum bilirubin estimation was done in the Biochemistry department, Center lab of Government medical college, Haldwani (Formerly STM Haldwani) Uttarakhand. Serum bilirubin estimation was done by Diazo

Bilirubin-Induced Neurological Dysfunction: A Clinico-Radiological-Neurophysiological Correlation in 30 Consecutive Children.

The clinical expression of bilirubin-induced neurological dysfunction varies according to severity and location of the disease. Definitions have been proposed to describe different bilirubin-induced neurological dysfunction subtypes. Our objective was to describe the severity and clinico-radiological-neurophysiological correlation in 30 consecutive children with bilirubin-induced neurological dysfunction seen over a period of 5 years. Thirty children exposed to acute neonatal bilirubin encephalopathy were included in the study. The mean peak total serum bilirubin level was 625 μmol/L (range 480-900 μmol/L). Acoustic brainstem responses were abnormal in 73% (n = 22). Pallidal hyperintensity was observed on magnetic resonance imaging in 20 children. Peak total serum bilirubin levels correlated with motor severity (P = .03). Children with severe motor impairment were likely to manifest severe auditory neuropathy (P < .01). We found that in a resource-constrained setting, classical kernicterus was the most common bilirubin-induced neurological dysfunction subtype, and the majority of children had abnormal acoustic brainstem responses and magnetic resonance imaging.

A Hypothesis for Using Pathway Genetic Load Analysis for Understanding Complex Outcomes in Bilirubin Encephalopathy

Genetic-based susceptibility to bilirubin neurotoxicity and chronic bilirubin encephalopathy (kernicterus) is still poorly understood. Neonatal jaundice affects 60–80% of newborns, and considerable effort goes into preventing this relatively benign condition from escalating into the development of kernicterus making the incidence of this potentially devastating condition very rare in more developed countries. The current understanding of the genetic background of kernicterus is largely comprised of mutations related to alterations of bilirubin production, elimination, or both. Less is known about mutations that may predispose or protect against CNS bilirubin neurotoxicity. The lack of a monogenetic source for this risk of bilirubin neurotoxicity suggests that disease progression is dependent upon an overall decrease in the functionality of one or more essential genetically controlled metabolic pathways. In other words, a “load” is placed on key pathways in the form of multiple genetic variants that combine to create a vulnerable phenotype. The idea of epistatic interactions creating a pathway genetic load (PGL) that affects the response to a specific insult has been previously reported as a PGL score. We hypothesize that the PGL score can be used to investigate whether increased susceptibility to bilirubin-induced CNS damage in neonates is due to a mutational load being placed on key genetic pathways important to the central nervous system's response to bilirubin neurotoxicity. We propose a modification of the PGL score method that replaces the use of a canonical pathway with custom gene lists organized into three tiers with descending levels of evidence combined with the utilization of single nucleotide polymorphism (SNP) causality prediction methods. The PGL score has the potential to explain the genetic background of complex bilirubin induced neurological disorders (BIND) such as kernicterus and could be the key to understanding ranges of outcome severity in complex diseases. We anticipate that this method could be useful for improving the care of jaundiced newborns through its use as an at-risk screen. Importantly, this method would also be useful in uncovering basic knowledge about this and other polygenetic diseases whose genetic source is difficult to discern through traditional means such as a genome-wide association study.

Efficacy of prophylactic phototherapy for prevention of hyperbilirubinemia in very low birth weight newborns

Background: Jaundice is a common clinical condition in newborn occurring in approximately 60% of term and 80% of preterm infants. Unconjugated hyperbilirubinemia is universally common in all preterm infants especially in newborns with very low biLth weight. Low birth weight and premature infants are at major risk for exaggerated hyperbilirubinemia that can lead to bilirubin encephalopathy. Significant heterogeneity in the approach to the treatment of jaundiced neonates exists throughout the world. Phototherapy is the most common treatment for neonatal hyperbilirubinemia and could be most effective in preventing the sequelae of hyperbilirubinemia if initiated prophylactically. This randomized clinical trial has been proposed with the objective of assessing the efficacy of prophylactic photo therapy in preventing significant rise of unconjugated hyperbilirubinemia in premature neonates weighing less than 1500 gram and therefore to decrease the need for exchange transfusion and finally to reduce hospital stay due to hyperbilirubinemia. Methods: This randomized controlled clinical trial enrolled sixty newborns with birth weight less than 1500 gram. They were divided into two groups: 1) Prophylactic group, in whom phototherapy was started within 24 hours of birth and continued for 7 days and 2) Control group in whom therapeutic phototherapy was started considering serum bilirubin level and other clinical condi­tions as per institutional guidelines. Mean value of total serum bilirubin (TSB), duration of phototherapy, the need for exchange transfusion and duration of hospital stay in both groups were analyzed.Results: The maximum mean TSB level in prophylactic group was observed on 7th day and in control group it was observed on 3rd day of life. The total serum bilirubin levels were significantly lower in the 3rd and 5th days of life in the prophylactic group in comparison to control group (P value 0.001). Total serum bilirubin level exceeded therapeutic range in 6 (21 %) and 14 (50 %) newborns of the prophylactic group and control groups respectively (P value 0.026). No documented side effects of prophylactic photo­therapy was observed. Conclusion: The use of prophylactic photo therapy for infants weighing less than 1500 grn is effec­tive and sate when compared to the control group, considering satisfactory maintenance of low total serum bilimbin levels during first 7 days of life.

Influence of hyperbilirubinemia on neonatal sucking

BackgroundMothers of hyperbilirubinemic newborns frequently report to us that their infant is feeding poorly. As poor feeding in an extremely hyperbilirubinemic newborn may be an early sign of bilirubin encephalopathy, we hypothesized that neonatal hyperbilirubinemia would suppress the volume of feed ingested and diminish sucking parameters in comparison with minimally jaundiced neonates. ObjectiveTo determine whether hyperbilirubinemia does diminish feeding and sucking in neonates. Study designNeonates in a well-baby nursery with serum total bilirubin (STB) ≥15.0mg/dL were compared with those with transcutaneous bilirubin ≤10mg/dL. Neonur, a modification of Krohn's Nutritive Sucking Apparatus, was used to quantify sucking parameters. Measurements during a 5min feeding period included volume ingested (measured manually), number of sucks, average maximum sucking pressure, number of bursts, average burst duration, pause between bursts duration, number of sucks per burst, and average intersuck interval. Outcome measures were volume ingested and, presuming decreased volume, sucking parameter analysis would determine the component affected by hyperbilirubinemia. Results17 hyperbilirubinemic newborns (STB 17.8±1.6mg/dL) were compared with 24 controls, all with transcutaneous bilirubin <10.0mg/dL. The volume of feed ingested was similar between the hyperbilirubinemic newborns and controls (30.00 [20.00–42.50] ml vs. 25.00 [15.00–30.00] ml, p=0.2) (median [95% confidence interval]). No significant differences were noted in any of the other sucking parameters measured. ConclusionsAt concentrations of STB in the range of 15–20mg/dL, hyperbilirubinemia did not diminish feed volume or sucking parameters in term newborns. Poor feeding in moderately hyperbilirubinemic newborns cannot be attributed to the level of bilirubin per se.

The application value of aEEG and NBNA in the early evaluation on brain damage for full-term neonates with hyperbilirubinemia

Objective To investigate the application value of amplitude integrated electroencephalogram (aEEG) and neonatal behavioral neurological assessment(NBNA) in the early evaluation on brain damage for full-term neonates with hyperbilirubinemia. Methods From August 2015 to February 2016, 42 full-term neonates with hyperbilirubinemia were divided into two groups:subclinical bilirubin encephalopathy group and hyperbilirubinemia control group according to the results of magnetic resonance imaging(MRI), while 20 normal healthy full-term infants born in our hospital at the same time were chosen as normal control group. The study protocol was approved by the Ethical Review Board of Investigation in Human Being of Clinic Medical College of Yangzhou University. Informed consent was obtained from each parents.The serum level of total bilirubin(TB), the score of NBNA, the abnormality rate for brainstem auditory evoked potential (BAEP) and aEEG were compared among three groups,meanwhile the correlation for these parameters were assayed. Results (1)There were significant differences in the level of UCB ,the score of NBNA, the abnormality rate of BAEP and aEEG among different groups (F=20.16, 7.12,χ2=15.18,12.23,P 0.05). (2)Of the 7 mildly abnormal aEEG cases in the subclinical bilirubin encephalopathy group, 6 mildly abnormal and 1 moderately abnormal were found in BAEP test. All of the 9 severely abnormal aEEG cases in the subclinical bilirubin encephalopathy group were found 1 moderately abnormal and 8 severely/very severely abnormal of BAEP. By linear correlation analysis,the abnormality degree of aEEG was positively correlation with BAEP (γ=0.806,P=0.032). (3)22 cases of subclinical bilirubin encephalopathy group inclued 16 abnormal aEEG cases (72.73%) and 21abnormal aEEG with NBNA cases (95.45%) respectively,there were statistically significant differences (χ2=4.08,P<0.05). Conclusion aEEG combining with NBNA may be an reference indicator for early evaluation on brain damage in full-term neonates with hyperbilirubinemia. It has great clinical important value and can be worth popularizing. Key words: aEEG; NBNA; Neonate; Hyperbilirubinemia; Brain damage

Changes of electroencephalographic activities during fully automated simultaneous peripheral arteriovenous exchange transfusion in neonates with hyperbilirubinemia

Objective To analyze the changes of electroencephalographic activities during fully automated simultaneous peripheral arteriovenous exchange transfusion(ET) in neonates with hyperbilirubinemia. Methods A total of 45 neonates who suffered from severe hyperbilirubinemia and underwent fully automated simultaneous peripheral arteriovenous exchange transfusion were studied from March 2009 to March 2016 in Neonatal Intensive Care Unit of Guangzhou Women and Children's Medical Center, and 46 ETs were performed in 45 babies who were divided into 2 groups according to the severity of hyperbilirubinemia: the encephalopathy group and the none-encephalopathy group.Nineteen patients were in the encephalopathy group, in which 11 were male and 8 were female.The other 26 patients were in the none-encephalopathy group, in which 15 were male and 11 were female.Changes in amplitude integrated electroencephalogram(aEEG) during ETs were analyzed, including background activities, sleep-wake cycle (SWC) and seizures. Results Forty-five patients with hyperbilirubinemia underwent 46 fully automated simultaneous perip-heral arteriovenous ETs. As a result, total bilirubin dropped from (524.90±110.96) μmol/L before ETs to (245.62±78.97) μmol/L after ETs, with clearance rate of 53.2%. And indirect bilirubin dropped from(486.16±90.39) μmol/L before ETs to(222.19±79.49) μmol/L after ETs, with clearance rate of 54.3%. On the other hand, there was no significant difference in the changes of electroencephalographic activities during ETs, including background activities(χ2=0.16, P>0.05), SWC(χ2=0.71, P>0.05) and seizures(χ2=0.30, P>0.05). However, there were significant difference in suppressions on background activities between the encephalopathy group and the none-encephalopathy group(Fisher's exact test P=0.042), though there were no significant statistical differences in SWC or seizures between the 2 groups ( χ2=0.65, P>0.05; χ2=2.07, P>0.05, respectively). Conclusions In neonatal hyperbilirubinemia, fully automated simultaneous peripheral arteriovenous ET is safe and efficient without significant influence on electroencephalographic activities as a whole.However, background activities are more significantly depressed in infants of bilirubin encephalopathy than that of non-encephalopathy during ET. Key words: Fully automated simultaneous peripheral arteriovenous exchange transfusion; Amplitude integra-ted electroencephalogram; Hyperbilirubinemia; Infant, newborn

Congenital Thrombotic Thrombocytopenic Purpura: An Unusual Case of Microangiopathic Haemolytic Anaemia in a Newborn

We present an extremely rare case of hyperbilirubinemia with rapid progression leading to bilirubin encephalopathy in term neonate. Despite early recognition and intervention, death occurred as a total serum bilirubin reached 25 mg/dl. It was a case of Coomb’s negative microangiopathic haemolytic anaemia in a newborn period which is autosomal recessive inheritance i.e. Upshaw-Schulman Syndrome. (Congenital thrombotic thrombocytopenic purpura) characterised by numerous schistocytes on peripheral blood smear, thrombocytopenia , increased reticulocyte count, increased bilirubin and LDH level. This rare disease is often misdiagnosed especially in newborn baby. So we present this case not only for its variety but also for to create more awareness among pathologist and paediatrician as treatment protocol entirely differ.

Neonatal hyperbilirubinaemia

Neonatal hyperbilirubinaemia is an extremely common condition which continues to be a significant cause of readmission of neonates to hospitals. The aim of early recognition and appropriate treatment is to prevent bilirubin encephalopathy which can cause severe neurodisability. This article reviews the national guidelines issued by various organizations like American Academy of Paediatrics in USA and National Institute of Clinical Excellence in the UK which have attempted to standardize approaches to diagnosis and management. This review highlights how early detection with measurement of bilirubin as opposed to visual estimation and appropriate use of high intensity phototherapy based on recommended treatment threshold charts reduces the incidence of severe jaundice requiring exchange transfusion and how evaluation and support of new mothers and their babies in the community by trained nurses with access to transcutaneous bilirubinometers can reduce hospital referrals while providing safe care at home.

Heliotherapy for Neonatal Hyperbilirubinemia in Southwest, Nigeria: A Baseline Pre-Intervention Study.

BackgroundA novel filtered-sunlight phototherapy (FSPT) device has been demonstrated to be safe and efficacious for treating infants with neonatal jaundice in resource-constrained tropical settings. We set out to provide baseline data for evaluating the clinical impact of this device in a referral pediatric hospital.MethodsWe reviewed the medical records of infants admitted for neonatal hyperbilirubinemia in an inner-city Children’s Hospital in Lagos, between January 2012 and December 2014 to determine the pattern, treatment and outcomes during the pre-intervention period. Factors associated with adverse outcomes were identified through multivariable logistic regression.ResultsOf the 5,229 neonatal admissions over the period, a total of 1,153 (22.1%) were admitted for neonatal hyperbilirubinemia. Complete records for 1,118 infants were available for analysis. The incidence of acute bilirubin encephalopathy (ABE) and exchange transfusion (ET) were 17.0% (95% CI: 14.9%–19.3%) and 31.5% (95% CI: 28.8%–34.3%) respectively. A total of 61 (5.5%, 95% CI: 4.3%–6.9%) of the jaundiced infants died. Weight on admission, peak total serum bilirubin (TSB), sepsis and exposure to hemolytic products were predictive of ABE, while age on admission, peak TSB, ABO incompatibility and ABE were predictive of ET. Rhesus incompatibility, asphyxia, exposure to hemolytic substances and ABE were associated with elevated mortality risk, while ET was a protective factor. Lack of routine irradiance monitoring and steady energy supply were frequent challenges for conventional blue-light phototherapy.ConclusionsSevere hyperbilirubinemia is associated with high rates of ABE and ET in this setting, and remains a significant contributor to neonatal admissions and mortality. To be impactful, FSPT, complemented with improved diagnostic facilities, should effectively curtail jaundice-related adverse outcomes in this and comparable settings.

A decision-making tool for exchange transfusions in infants with severe hyperbilirubinemia in resource-limited settings.

Late presentation and ineffective phototherapy account for excessive rates of avoidable exchange transfusions (ETs) in many low- and middle-income countries. Several system-based constraints sometimes limit the ability to provide timely ETs for all infants at risk of kernicterus, thus necessitating a treatment triage to optimize available resources. This article proposes a practical priority-setting model for term and near-term infants requiring ET after the first 48 h of life. The proposed model combines plasma/serum bilirubin estimation, clinical signs of acute bilirubin encephalopathy and neurotoxicity risk factors for predicting the risk of kernicterus based on available evidence in the literature.

Prevalence of Bilirubin Encephalopathy in Calabar, South-South Nigeria: A Five-year Review Study

Background: Bilirubin encephalopathy is a clinical syndrome, associated with bilirubin toxicity in the central nervous system, resulting in chronic and permanent sequelae. It has been estimated that approximately 60% and 80% of term and preterm newborns develop jaundice in the first week of life, respectively. In the present study, we aimed to determine the prevalence, morbidity, and mortality of bilirubin encephalopathy in the neonatal unit of the University of Calabar Teaching Hospital, Calabar, Nigeria. Methods: In this retrospective, descriptive review, medical records of all newborns, diagnosed with bilirubin encephalopathy over the past five years (from January 2010 to December 2014), were studied. Information retrieved from the medical records included age, sex, presence of fever, duration of disease, place of delivery, causes of the disease, and selected treatments. Variables such as hospital discharge, discharge against medical advice, and mortality were also evaluated. Results: Out of 2,820 newborns, 21 (0.74%) cases were admitted on account of bilirubin encephalopathy. Among these affected cases, 17 (81%) were male and 4 (19%) were female (male-to-female ratio of 5:1). Based on the findings, 18 newborns (85.7%) had pyrexia, while 8 (38.1%) and 6 (28.6%) cases were hypertonic and hypotonic, respectively upon admission. Only 33.3% of deliveries took place in healthcare facilities. The established factors responsible for jaundice included infection, i.e., septicemia (n=15, 71.4%), ABO incompatibility (n=4, 19.1%), and glucose-6-phosphate-dehydrogenase (G6PD) deficiency (n=2, 9.5%). The mean maximum total bilirubin level in subjects was 321.3 μmol/L (range: 242.5–440.3 μmol/L). Also, mortality was reported in 4 (19%) out of 21 cases. Conclusion: Based on the findings, neonatal septicemia is associated with bilirubin encephalopathy. Therefore, identification and prompt treatment are of utmost importance in preventing the associated morbidity and mortality.