Neonatal jaundice is associated with increased risks of congenital anomalies of the kidney and urinary tract and concomitant urinary tract infection.

The link between neonatal jaundice and urinary tract infection (UTI) remains debated, with congenital kidney and urinary tract anomalies (CAKUT) potentially playing a role. This population-based study aimed to analyze the correlations between neonatal jaundice, CAKUT, and concomitant UTI. The study cohort consisted of 2,078,122 live births from 2004 to 2014. We linked several population-based datasets in Taiwan to identify infants with unexplained neonatal jaundice and their mothers. The primary outcome was the rate of CAKUT occurring within 3 years after delivery, and the presence of concomitant UTI during neonatal jaundice hospitalization. Infants with neonatal jaundice had a significantly higher risk of CAKUT (adjusted odds ratio [aOR] 1.24, 95% confidence interval [CI] 1.11-1.39) during early childhood. Among the subtypes of CAKUT, obstructive uropathy, vesicoureteral reflux and other CAKUT were associated with an increased risk of neonatal jaundice. Infants who underwent intensive phototherapy, had a late diagnosis (> 14 days of postnatal age) or underwent a prolonged duration of phototherapy (> 3 days) exhibited a higher risk of concomitant UTI compared to other infants with jaundice. Our findings indicate a notable association between neonatal jaundice and increased risks of UTIs in the context of CAKUT. This study underscore the importance of vigilant monitoring and timely interventions for neonates presenting with jaundice, while acknowledging the complexity and variability in the progression of CAKUT and its potential connection to UTIs.

Associations between neonatal jaundice and autism spectrum disorder or attention deficit hyperactivity disorder: Nationwide population based cohort study.

BACKGROUND/PURPOSE: Neonatal jaundice might result brain insults. Both autistic spectrum disorder (ASD) and attention deficit hyperactivity disorder (ADHD) are developmental disorders, which might result from early brain injury at neonatal period. We aimed to explore the association between neonatal jaundice treated with phototherapy and the ASD or ADHD. METHODS: This retrospective nationwide population cohort study was based on a nationally representative database of Taiwan, and neonates born from 2004 to 2010 were enrolled. All eligible infants were divided into 4 groups, without jaundice, jaundice with no treatment, jaundice with simple phototherapy only and jaundice with intensive phototherapy or blood exchange transfusion (BET). Each infant was follow-up until the date of incident primary outcomes, death, or 7-year-old, whichever occurred first. Primary outcomes were ASD, ADHD. Using cox proportional hazard model to analyze their associations. RESULTS: In total, 118,222 infants with neonatal jaundice were enrolled, including diagnosed only (7260), simple phototherapy (82,990), intensive phototherapy or BET (27,972 infants). The cumulative incidences of ASD in each group was 0.57%, 0.81%, 0.77%, and 0.83%, respectively. The cumulative incidences of ADHD in each group was 2.83%, 4.04%, 3.52% and 3.48%, respectively. Jaundice groups were significantly associated with ASD, ADHD, or either one, even after all other extraneous maternal and neonatal variables were adjusted. After stratification, the associations were still existed in subgroup with birth weights ≥2500 grams and in male subgroup. CONCLUSION: Neonatal jaundice correlated with the ASD and ADHD. The associations were significant in infants of both sexes and with birth weights larger than 2500 grams.

Risk of autism spectrum disorder in children with a history of hospitalization for neonatal jaundice.

BACKGROUND: Limited research has focused explicitly on the association between neonatal jaundice and autism spectrum disorder (ASD), and inconclusive evidence exists in the literature within this framework. This study aimed specifically to investigate whether neonatal jaundice is a potential risk factor for ASD and whether there is a connection between the types of neonatal jaundice and the severity of ASD. METHODS: This study involved 119 children with ASD [90 males (75.6%), 29 females (24.4%), mean age: 45.39 ± 11.29 months] and 133 healthy controls [100 males (75.2%), 33 females (24.8%), mean age: 46.92 ± 11.42 months]. Psychiatric disorders were diagnosed through the Diagnostic and Statistical Manual of Mental Disorders criteria. Childhood Autism Rating Scale (CARS) was used to assess the screening and diagnosis of autism. A specially prepared personal information sheet was employed to investigate sociodemographic characteristics and birth and clinical histories. RESULTS: The rate of the history of jaundice and pathological jaundice requiring hospitalization and phototherapy were significantly higher in the ASD group compared to the controls. CARS total score and the mean scores of nearly all items were statistically higher in children with a history of pathological jaundice than those with a history of physiological jaundice. DISCUSSION: Neonatal jaundice, depends on its severity, seems to be one of the possible biological factors associated with subsequent development of and the severity of ASD. Establishing a causal relationship between neonatal jaundice and ASD by more comprehensive studies may contribute to alleviating of the severity of ASD for individuals at risk.

Long-term neurodevelopmental outcomes of significant neonatal jaundice in Taiwan from 2000-2003: a nationwide, population-based cohort study.

Newborns with significant neonatal jaundice (SNJ) would admit for evaluation and/or intervention due to an earlier or more rapid increase in bilirubin level. Bilirubin-induced neurological dysfunction in this population might be underestimated. We aimed to investigate the risk of long-term neurodevelopmental sequelae of SNJ in Taiwan. An SNJ 2000-2003 follow-up cohort consisting of 66,983 neonates was extracted from the nationwide, population-based health insurance database in Taiwan to survey the accumulative incidence of long-term (7-year) neurodevelopmental sequelae in comparison to a reference general-population neonate cohort of 12,579 individuals born in 2000. The SNJ follow-up cohort was furtherly categorized into subgroups according to interventions (phototherapy, intensive phototherapy, and exchange transfusion). The SNJ follow-up cohort exhibited significantly higher cumulative rates of long-term neurodevelopmental sequelae than did the reference cohort (P < 0.05). The risks of infantile cerebral palsy, hearing loss, and developmental delay in the SNJ follow-up cohort were between twice and three times of those in the reference cohort after adjusting for gender, comorbid perinatal disorders and urbanization levels. All intervention subgroups demonstrated higher risks for long-term neurodevelopmental sequelae than the reference cohort (P < 0.05) after adjustment. Patients with SNJ are at risk of developing neurodevelopmental disorders during their growth period. A scheduled follow-up protocol of physical and neurodevelopmental assessment during early childhood for these SNJ patients would potentially be helpful for the early detection of and intervention for neurodevelopmental disorders.

Neonatal Jaundice: The Other Side of the Coin in the Development of Allergy.

OBJECTIVE: The current study aims to assess the association between different characteristics of neonatal jaundice and common types of allergic diseases in childhood (as bronchial asthma, acute urticaria, and allergic rhinitis). STUDY DESIGN: A case-control study is conducted on 300 allergic children and 300 healthy children as a control group at Fayoum University Hospital. The study was conducted over a span of 2 years, from May 2016 to May 2018. Bronchial asthma, allergic rhinitis, and acute urticaria diagnoses were based on physician clinical examination using specific guidelines for each. For the data regarding children's demographic and maternal characteristics, a structured questionnaire was used. Regarding neonatal jaundice, data were collected from the patients' hospital records. RESULTS: Children with neonatal jaundice are more likely to develop allergy with 57% higher than neonates without jaundice. Early onset jaundice and treatment by phototherapy have statistically significant association with the development of allergic diseases. CONCLUSION: Different aspects of neonatal jaundice are associated with the development of common allergic diseases in children.

Three Novel Spectrin Variants in Jaundiced Neonates.

Various mutations in the genes encoding alpha spectrin (SPTA1) or beta spectrin (SPTB) are known to cause erythrocyte membrane disorders, sometimes associated with severe neonatal jaundice and anemia. We used a next-generation sequencing panel to evaluate 3 unrelated neonates who had puzzling cases of nonimmune hemolytic jaundice. In each case, we identified novel mutations in either SPTA1 or SPTB. Correlating erythrocyte morphology, clinical course, and computational analysis, we submit that each of the 3 variants is a probable pathogenic cause of the hereditary hemolytic conditions in these patients. We hope other pediatric practitioners caring for neonates with what appears to be idiopathic severe neonatal hyperbilirubinemia will look for spectrin variants as a possible cause, because additional cases with these specific variants along with this clinical phenotype are needed to confirm our postulate that these 3 cases are indeed pathogenic mutations.

Neonates with sickle cell disease are vulnerable to blue light phototherapy-induced oxidative stress and proinflammatory cytokine elevations.

Sickle cell disease is a frequent genetic anomaly characterized by altered molecular structure of hemoglobin resulting into crescent-like deformation of the red blood corpuscles. Neonatal jaundice is a frequent co-morbidity in sickle cell disease. Phototherapy induces isomerization of bilirubin rendering it extractable through urine and hence it is used as a routine treatment of neonatal jaundice. An exposure to light phototherapy as a treatment of neonatal jaundice induces oxidative stress. It is hypothesized that such exposure of neonates with sickle cell disease to the blue light phototherapy as a treatment of neonatal jaundice induces severe oxidative stress and increases the levels of proinflammatory cytokines. This hypothesis is supported with two case studies of sickle cell disease suffering neonates who were exposed to blue light phototherapy to treat jaundice. In both these cases, exposure to phototherapy induced oxidative stress (increased lipid peroxidation and superoxide dismutase, slight change in activity of catalase and GSH) and elevated the levels of proinflammatory cytokine (TNFα, IL-1, and IL-6) in the sickle cell disease suffering neonates. These observations warrant further investigations to determine the consequences and clinical significance of the blue phototherapy-induced oxidative and proinflammatory stress in Sickle cell disease suffering neonates exposed to phototherapy as a treatment of jaundice.

Albumin administration prevents neurological damage and death in a mouse model of severe neonatal hyperbilirubinemia.

Therapies to prevent severe neonatal unconjugated hyperbilirubinemia and kernicterus are phototherapy and, in unresponsive cases, exchange transfusion, which has significant morbidity and mortality risks. Neurotoxicity is caused by the fraction of unconjugated bilirubin not bound to albumin (free bilirubin, Bf). Human serum albumin (HSA) administration was suggested to increase plasma bilirubin-binding capacity. However, its clinical use is infrequent due to difficulties to address its potential preventive and curative benefits, and to the absence of reliable markers to monitor bilirubin neurotoxicity risk. We used a genetic mouse model of unconjugated hyperbilirubinemia showing severe neurological impairment and neonatal lethality. We treated mutant pups with repeated HSA administration since birth, without phototherapy application. Daily intraperitoneal HSA administration completely rescued neurological damage and lethality, depending on dosage and administration frequency. Albumin infusion increased plasma bilirubin-binding capacity, mobilizing bilirubin from tissues to plasma. This resulted in reduced plasma Bf, forebrain and cerebellum bilirubin levels. We showed that, in our experimental model, Bf is the best marker to determine the risk of developing neurological damage. These results support the potential use of albumin administration in severe acute hyperbilirubinemia conditions to prevent or treat bilirubin neurotoxicity in situations in which exchange transfusion may be required.

Neonatal jaundice and risks of childhood allergic diseases: a population-based cohort study.

BACKGROUND: Only a few studies have systemically analyzed the association between neonatal jaundice and childhood-onset allergic diseases. METHODS: From 2000 to 2007, 27,693 neonates with newly diagnosed neonatal jaundice and 55,367 matched nonneonatal jaundice cohorts were identified. The incidences and hazard ratios (HRs) of five allergic diseases, namely allergic conjunctivitis (AC), allergic rhinitis (AR), atopic dermatitis (AD), asthma, and urticaria, by the end of 2008 were calculated. RESULTS: The incidence density and HRs of the five allergic diseases were greater in the neonatal jaundice cohort than in the nonneonatal jaundice cohort, and the HRs declined modestly with age. The HRs for AR (HR = 2.51, 95% confidence interval (CI) = 2.43-2.59) and AD (HR = 2.51, 95% CI = 2.40-2.62) were the highest, and that for urticaria was the lowest (HR = 2.06, 95% CI = 1.94-2.19). The HRs of allergic diseases were substantially greater for boys and those requiring phototherapy. The HRs of the allergic diseases, except urticaria (HR = 2.49, 95% CI = 1.57-3.97), were not significantly different between the neonatal jaundice regardless of whether the patients received exchange transfusion. CONCLUSION: Neonatal jaundice is associated with the development of allergic diseases in early childhood.

Neonatal jaundice and increased risk of attention-deficit hyperactivity disorder: a population-based cohort study.

BACKGROUND: Previous studies have posited conflicting results regarding the relationship between neonatal jaundice and the subsequent risk of attention-deficit hyperactivity disorder (ADHD). We therefore performed a large population study with a defined neonatal jaundice cohort to investigate the incidence and risk of physician-diagnosed ADHD in Taiwan. METHODS: From 2000 to 2004, 24,950 neonatal jaundice cases and 69,964 matched nonjaundice controls were identified. At the end of 2008, the incidence rate and hazard ratios (HRs) of physician-diagnosed ADHD were calculated. RESULTS: The incidence of ADHD was 2.48-fold greater in the jaundice cohort than in the nonjaundice cohort (3.84 vs. 1.51 per 100,000 person-years) in the study period. The HR of ADHD was substantially greater for male, preterm, and low-birth-weight infants with neonatal jaundice. The risk of developing ADHD in the jaundice cohort was greater after a diagnosis of neonatal jaundice for more than 6 years (HR: 2.64; 95% confidence interval: 2.13-3.28). The risk of ADHD increased for neonates with higher serum bilirubin levels requiring phototherapy and with longer admission days. CONCLUSION: Neonates with jaundice are at high risk for developing physician-diagnosed ADHD during their growth period. A risk alert regarding neurologic consequences is urgently required after a neonatal jaundice diagnosis. Additional studies should be conducted to clarify the pathogenesis of these relationships.

Bullous skin lesions in a jaundiced infant after phototherapy: a case of congenital erythropoietic porphyria.

Congenital erythropoietic porphyria is a rare autosomal recessive disorder of porphyrin metabolism in which the genetic defect is the deficiency of uroporphyrinogen III cosynthase (UIIIC). Deficiency of this enzyme results in an accumulation of high amounts of uroporphyrin I in all tissues, leading to hemolytic anemia, splenomegaly, erythrodontia, bone fragility, exquisite photosensitivity, and mutilating skin lesions. We discuss a female infantile case who was admitted for jaundice; bullous lesions appeared on her trunk during phototherapy in the neonatal period. The skin biopsy findings were consistent with epidermolysis bullosa. Due to persistent hepatosplenomegaly and cholestasis, metabolic tests and liver biopsy were performed. During the follow-up, hemolytic anemia and red urine were detected. The levels of porphyrin metabolites were determined at high concentrations in plasma, stool and urine analysis, which were suggestive of congenital erythropoietic porphyria.

Neonatal jaundice is a risk factor for childhood allergic rhinitis: a retrospective cohort study.

BACKGROUND: This study evaluates the association between neonatal jaundice and childhood allergic rhinitis (AR). METHODS: Eleven thousand three hundred twenty-eight children were collected from the National Health Insurance Research Database in Taiwan. Their claims data were evaluated from birth to 10 years of age, and they were assigned to either the study (with neonatal jaundice) or the control (without neonatal jaundice) group. The diagnostic criteria for AR were at least three diagnoses of AR at outpatient services, one diagnosis of AR during an admission, or one diagnosis of AR in an emergency department. Mantel-Haenszel odds ratios (ORs) were calculated after adjustment for the following confounders: preterm/low birth weight, neonatal infection, other respiratory conditions, other birth conditions, and gender. AR rate, AR onset time, the use of oral antihistamines/nasal corticosteroids, outpatient visit frequency for AR, lower respiratory infection (LRI) rates, sinusitis/otitis media/conjunctivitis rates, and the effect of phototherapy were evaluated. RESULTS: After adjustment for the confounding factors, the rate of AR was higher in icteric children (OR, 1.46; 95% confidence interval, 1.24∼1.72). There was a higher incidence of AR in children <4 years old with icterus. The use of oral antihistamines, LRI rates, sinusitis rates, and otitis media rates were higher in the icteric children. There was no association between phototherapy and childhood AR. CONCLUSION: Neonatal jaundice increased the rate and complications of childhood AR in subjects aged up to 10 years and may be a risk factor for childhood AR.

[Clinical study of myocardial damage induced by neonatal jaundice in normal birth weight term infants].

OBJECTIVE: To clarify whether neonatal jaundice may cause myocardial damage to term infants with normal birth weight (BW). METHODS: Totally 178 term neonates admitted during March, 2004 to December, 2010 with normal BW were enrolled. Infants with antenatal or neonatal asphyxia, temperature abnormality, septicemia, antenatal viral infection, congenital dysmorphia, congenital heart disease, 21-trisomy, and polycythemia were excluded. There was no maternal complications during the pregnancy. Serum total bilirubin (TB), creatine kinase (CK), MB isoenzymes of creatine kinase (CK-MB), and cardiac troponin-I (cTnI) were measured. Patients with transcutaneous bilirubin level (TcB) ≥ 342 µmol/L (20 mg/dl) were in Group A (n = 32), and those with TcB below phototherapy level at matched time point were in Group B (n = 25). ECG, for correct Q-T intervals (QTc) and correct QT intervals dispersion (QTcd), and ECHO, for left ventricular ejection fraction (EF), the ratio of the peak velocity of early stage and advanced stage of diastolic phase at the mitral orifice (E/A), were applied to patients in Group A and B. SPSS 13.0 software was used for the data analysis. The coefficients of correlation among age in hours on admission (hr), TB, CK, CK-MB, CK-MB/CK, and cTnI were studied by multiple and partial correlation analysis. Data in Group A and B were compared by independent-samples Mann-Whitney U test (nonparametric method) or Student t-test. RESULTS: When the data were analyzed by multiple correlation, there were significant correlation between TB and cTnI, CK-MB, respectively (r = 0.212, -0.161, respectively, all P < 0.05). But, when the data were analyzed by partial correlation, there was no correlation between TB and cTnI, CK-MB, respectively (r' = 0.112, -0.112, respectively, all P > 0.05), negative correlation between hr and TB, cTnI, respectively (r' = -0.490, P = 0.000; r' = -0.162, P = 0.032). There was no significant difference in CK (Z = -1.384, P = 0.166), CK-MB (Z = -0.821, P = 0.412), cTnI (Z = -1.159, P = 0.246), QTc (t = 1.146, P = 0.257), QTcd (t = 1.342, P = 0.185), EF (t = 1.558, P = 0.125), E/A (t = -0.640, P = 0.525) between group A and B. There was significant difference in CK-MB/CK (Z = -3.187, P = 0.001) between group A and B with a lower value in group A [0.075 (0.032 - 0.102)] comparing to that in group B [0.160 (0.073 - 0.284)]. CONCLUSION: There is no sufficient evidence to support the hypothesis that neonatal jaundice may induce myocardial damage in normal birth weight term infants.

Morbidity of ABO haemolytic disease in the newborn.

BACKGROUND: Better understanding of the clinical characteristics of ABO haemolytic disease in neonates helps optimise care. OBJECTIVE: To assess the morbidity associated with maternal-neonatal ABO incompatibility. METHODS: Neonates with blood groups A or B born to mothers with blood group O with simultaneous rhesus blood factor compatibility were studied prospectively. Maternal and neonatal details, direct Coomb's test (DCT) on the cord blood, onset and progression of jaundice, and requirement and duration of phototherapy were studied. Neonates requiring phototherapy were considered to have significant hyperbilirubinaemia, and peripheral smear, reticulocyte count and haematocrit values were obtained. ABO haemolytic disease of the newborn (ABO HDN) is defined as a newborn with a positive DCT and/or laboratory evidence of haemolysis such as reticulocytosis and spherocytes on blood smear. RESULTS: Of 878 deliveries, 151 (17.3%) neonates were ABO incompatible with their mothers. The proportions who were O-A and O-B incompatible were 50.4% and 49.6%, respectively. Forty-six (30.4%) had significant hyperbilirubinaemia (119.7-256.5 mmol/L) and required phototherapy, 26 (34.2%) of them in the O-A group and 20 (26.6%) in the O-B group. None required exchange transfusion. Jaundice was detected within the first 24 hours in 47.8%. Of 46 newborns who required phototherapy, 25 (54.3%) had laboratory evidence of haemolysis. DCT was positive in 1.9% of ABO-incompatible newborns. There was no significant difference in the incidence and severity of haemolysis between the O-A and O-B-incompatible neonates. Neonates with haemolysis required phototherapy significantly earlier and for longer than neonates without haemolysis (P<0.001). CONCLUSIONS: ABO incompatibility was observed in 17.3% of pregnancies with almost equal O-A and O-B frequency. About a third of infants had significant hyperbilirubinaemia. There was no difference in severity between those with O-A and O-B HDN.

Prophylactic phototherapy for preventing jaundice in preterm or low birth weight infants.

BACKGROUND: Low birth weight and premature infants are at major risk for exaggerated hyperbilirubinaemia and jaundice that can lead to bilirubin encephalopathy. Phototherapy is the most common treatment for neonatal hyperbilirubinaemia and could be most effective in preventing the sequelae of hyperbilirubinaemia if initiated prophylactically. OBJECTIVES: To evaluate the efficacy and safety of prophylactic phototherapy for preterm (< 37 weeks gestational age) or low birth weight infants (birth weight < 2500 g). SEARCH METHODS: We searched the Cochrane Central Register of Controlled Trials (CENTRAL, The Cochrane Library, Issue 3) on 31 March 2011, MEDLINE (1950 to 31 March 2011), EMBASE (1980 to 31 March 2011) and CINAHL (1982 to 31 March 2011). SELECTION CRITERIA: Randomised controlled trials or quasi-randomised controlled studies evaluating the effects of prophylactic phototherapy for preterm or low birth weight infants. DATA COLLECTION AND ANALYSIS: Two authors independently obtained data from published articles. We performed fixed-effect meta-analysis for the outcomes: rate of exchange transfusion, cerebral palsy or other neurodevelopmental impairment, peak serum bilirubin level and all-cause mortality. MAIN RESULTS: Nine studies of 3449 participants were included. The rate of exchange transfusion was reduced in one study with liberal transfusion criteria (risk ratio (RR) 0.20; 95% confidence interval (CI) 0.13 to 0.31) but not in the other two more recent studies with stringent criteria (typical RR 0.66; 95% CI 0.19 to 2.28). There was no statistically significant difference in the rate of cerebral palsy (typical RR 0.96; 95% CI 0.50 to 1.85; two studies, 756 participants). However, one large study that reported on neurodevelopmental impairment (a composite outcome including cerebral palsy) found a slightly lower rate of neurodevelopmental impairment with prophylactic phototherapy (RR 0.85; 95% CI 0.74 to 0.99; 1804 participants). The prophylactic phototherapy group had lower peak bilirubin levels (mean difference (MD) -2.73; 95% CI -2.89 to -2.57; six studies, 2319 participants) and had fewer neonates with peak unconjugated serum bilirubin levels > 10 mg/dl (typical RR 0.27; 95% CI 0.22 to 0.33; three studies, 1090 participants) or peak unconjugated serum bilirubin levels > 15 mg/dl (typical RR 0.13; 95% CI 0.07 to 0.23; four studies, 1116 participants). There was no statistically significant difference in the rate of all-cause mortality between the two groups (typical RR 1.08; 95% CI 0.93 to 1.26; four studies, 3044 participants). AUTHORS' CONCLUSIONS: Prophylactic phototherapy helps to maintain a lower serum bilirubin concentration and may have an effect on the rate of exchange transfusion and the risk of neurodevelopmental impairment. However, further well-designed studies are needed to determine the efficacy and safety of prophylactic phototherapy on long-term outcomes including neurodevelopmental outcomes.

Neonatal jaundice is associated with a small increase in the risk of childhood type 1 diabetes: a meta-analysis of observational studies.

Published data on the role of neonatal jaundice as a risk factor for childhood type 1 diabetes mellitus is inconsistent. We aimed to review systematically, the evidence for an increased risk of type 1 diabetes in children diagnosed with neonatal jaundice. A comprehensive search of the published literature was performed to identify studies that had recorded the occurrence of neonatal jaundice in a group of children with type 1 diabetes and in a group of control children. Odds ratios (ORs) were extracted from reports or derived from tabulated data and then combined using a random effects meta-analysis. Data were available from 12 case-control studies and one retrospective cohort study. Overall, there was only weak evidence of an increase in the risk of type 1 diabetes in children who had neonatal jaundice (OR 1.14, 95% CI 0.99-1.32; P = 0.07), and there was some evidence of heterogeneity (I(2) = 53%, P = 0.01) mainly attributable to one study. An analysis restricted to studies not relying on parental recall showed a stronger, significant relationship (OR = 1.25, 95% CI 1.03-1.51; P = 0.02), although heterogeneity remained. This analysis found evidence of a small but statistically significant increase in childhood type 1 diabetes risk associated with neonatal jaundice but only for studies which used data from obstetric records. Jaundice caused by blood group incompatibility or requiring phototherapy may be associated with a greater increase in type 1 diabetes risk and deserves further study.

Vagal nerve stimulator placement for medically refractory seizures in a child treated with phrenic nerve pacing for congenital central hypoventilation syndrome.

Congenital central hypoventilation syndrome (CCHS) is a rare, idiopathic disorder characterized by a failure of automatic respiration. Abnormalities such as seizure disorder, failure to thrive, and Hirschsprung disease have been associated with CCHS. In this report, the authors discuss the use of vagal nerve stimulation (VNS) to treat a medically refractory seizure disorder in a child who had previously undergone placement of bilateral phrenic nerve stimulators for treatment of CCHS. Concomitant use of phrenic and vagal nerve stimulators has not previously been reported in the literature. No adverse reactions were noted with both devices working. Diaphragmatic pacing (DP) was clinically unaffected by VNS. The patient experienced a marked reduction in seizure frequency and severity following vagal nerve stimulator placement. Based on this case, the authors conclude that VNS is a potentially safe and efficacious treatment option for seizure disorder associated with CCHS in patients undergoing DP.

Hemolytic disease of the newborn associated with anti-Jra alloimmunization in a twin pregnancy: the first case report in Korea.

Jr(a) is a high-frequency antigen found in all ethnic groups. However, the clinical significance of the anti-Jr(a) antibody has remained controversial. Most studies have reported mild hemolytic disease of the newborn and fetus (HDNF) in Jr(a)-positive patients. Recently, fatal cases of HDNF have also been reported. We report the first case of HDNF caused by anti-Jr(a) alloimmunization in twins in Korea. A 33-yr-old nulliparous woman with no history of transfusion or amniocentesis was admitted at the 32nd week of gestation because of vaginal bleeding caused by placenta previa. Anti-Jr(a) antibodies were detected in a routine laboratory examination. An emergency cesarean section was performed at the 34th week of gestation, and 2 premature infant twins were delivered. Laboratory examination showed positive direct antiglobulin test and Jr(a+) phenotype in the red blood cells and the presence of anti-Jr(a) antibodies in the serum in both neonates. The infants underwent phototherapy for neonatal jaundice; this was followed by conservative management. They showed no further complications and were discharged on the 19th postpartum day. Preparative management to ensure the availability of Jr(a-) blood, via autologous donation, and close fetal monitoring must be performed even in cases of first pregnancy in Jr(a-) women.

Hemolytic Disease of the Newborn Associated with Anti-Jr(a) Alloimmunization in a Twin Pregnancy: The First Case Report in Korea / 대한진단검사의학회지

Jr(a) is a high-frequency antigen found in all ethnic groups. However, the clinical significance of the anti-Jr(a) antibody has remained controversial. Most studies have reported mild hemolytic disease of the newborn and fetus (HDNF) in Jr(a)-positive patients. Recently, fatal cases of HDNF have also been reported. We report the first case of HDNF caused by anti-Jr(a) alloimmunization in twins in Korea. A 33-yr-old nulliparous woman with no history of transfusion or amniocentesis was admitted at the 32nd week of gestation because of vaginal bleeding caused by placenta previa. Anti-Jr(a) antibodies were detected in a routine laboratory examination. An emergency cesarean section was performed at the 34th week of gestation, and 2 premature infant twins were delivered. Laboratory examination showed positive direct antiglobulin test and Jr(a+) phenotype in the red blood cells and the presence of anti-Jr(a) antibodies in the serum in both neonates. The infants underwent phototherapy for neonatal jaundice; this was followed by conservative management. They showed no further complications and were discharged on the 19th postpartum day. Preparative management to ensure the availability of Jr(a-) blood, via autologous donation, and close fetal monitoring must be performed even in cases of first pregnancy in Jr(a-) women.

Infants with severe neonatal jaundice in Lagos, Nigeria: incidence, correlates and hearing screening outcomes.

OBJECTIVES: To establish the incidence, correlates and hearing screening outcomes of infants with severe neonatal jaundice in Nigeria. METHODS: Community-based study in which all infants attending Bacille Calmette-Guérin immunisation clinics in inner-city Lagos were enrolled into a universal hearing screening programme during which correlates of severe neonatal jaundice (requiring phototherapy and/or exchange blood transfusion) were explored with multivariable logistic regression. RESULTS: Of the 5262 infants enrolled, only 48.7% were born in hospitals although almost all mothers (97.9%) attended antenatal clinics. 6.7% had a history of neonatal jaundice of whom 5.5% (95% CI:4.9-6.2) received phototherapy and 1.9% (95% CI:1.5-2.3) had an exchange blood transfusion. Factors independently associated with severe neonatal jaundice were maternal religion, occupation, use of herbal preparations during pregnancy, infant's gender, weight at screening, multiple gestation and place of birth. All but two infants with severe neonatal jaundice were exclusively breast-fed. Of those who failed the hearing tests, 17.3% were treated with phototherapy and 11.3% had an exchange blood transfusion. At least 8.9% of infants requiring phototherapy and 17.3% of those requiring exchange blood transfusion were at risk of sensorineural hearing loss. CONCLUSIONS: Severe neonatal jaundice is a significant condition associated with modifiable risk factors in this population. Policy initiatives for prevention, early detection followed by appropriate and timely intervention are urgently needed to reduce the disease burden.