A Saudi Hospital's Experience in the Management of Well-Appearing Neonates at Increased Risk for Early-Onset Bacterial Sepsis.

BACKGROUND: Early-onset neonatal bacterial sepsis (EOS) is a serious medical condition where pathogenic bacterial species are isolated from the blood of newborns within the first 72 hours of life. Neonatal healthcare providers face challenges in managing well-appearing newborns born at 35 weeks gestational age or more who are at an increased risk of developing EOS. The American Academy of Pediatrics (AAP) has recommended three approaches for managing EOS. One of these approaches includes enhanced observation to observe the progression of the newborn's clinical condition within the first 48 hours after birth. The AAP recommends that birth centers should adopt institutional approaches that are tailored to their specific local resources and structures. It recommends that the chosen approach is evaluated to identify infrequent negative outcomes and to confirm its effectiveness. AIMS: To report our experience in managing EOS in newborns born at 35 weeks gestation or later with an increased risk for EOS. METHODS: This was a review of electronic medical records from the past five years. We included a sample of newborns born at or after 35 weeks gestational age who were at increased risk of EOS and appeared to be healthy. We implemented universal antenatal culture-based screening for Group B streptococcus (GBS). We followed the recommendations of the AAP in 2012 to manage these newborns. We performed a complete blood count (CBC) with differential and C-reactive protein (CRP) tests to predict EOS. We also considered the newborns symptomatic if they displayed any clinical signs of EOS. RESULTS: A total of 806 newborns were included in the study, out of which 27 (3.3%) of them had symptoms of EOS, while the remaining 782 newborns appeared healthy. Predictive blood tests were performed on 281 (34.9%) of the newborns, out of which 126 (44.8%) of them had a positive test result. However, blood cultures were obtained from 134 (16.6%) of the total cohort. Intravenous antibiotics were administered to 33 (4.1%) of the newborns. All symptomatic newborns had a positive predictive blood test result, and two of them had culture-proven EOS. Blood cultures obtained from the remaining 107 asymptomatic newborns were negative. In this context, 140 newborns needed to be pricked for positive predictive blood tests to predict one case of EOS. However, if the positive predictive blood tests were only performed on symptomatic newborns, then only 14 newborns would need to be pricked to predict one case of EOS. CONCLUSION: Based on the present study, it is advised to follow the current AAP recommendation against predicting EOS by solely relying on CBC with differential or CRP. The study suggests that the enhanced observation approach is a more sensible option for managing EOS, but this needs to be confirmed in a larger study.

A Saudi Girl With Co-occurring CHD1 (Pilarowski-Bjornsson Syndrome) and ASH1L Gene Variants.

Pilarowski-Bjornsson Syndrome (PBS) is a recently identified and rare genetic disorder. PBS is caused by missense variants in the CHD1 gene, a chromatin remodeler and helicase DNA-binding protein. In this report, we present the first case of PBS in Saudi Arabia. The patient exhibits a phenotype and genotype that are consistent with previously reported cases of PBS. Notably, this case is unique due to the coexisting presence of an absent, small, and homeotic disks protein 1 homolog like a histone lysine methyltransferase (ASH1L) variant and developmental dissociation. The ASH1L variant may contribute to the developmental dissociation observed in the patient. Furthermore, since the patient is female, this case contributes to the female-skewed distribution of PBS, although the exact cause of this phenomenon requires further investigation. This report highlights the importance of identifying and characterizing rare genetic disorders such as PBS. Understanding the genetic basis of these disorders can lead to improved diagnosis, treatment, and management strategies. Continued research on the genetic and molecular mechanisms underlying PBS and related disorders is crucial for advancing our knowledge and developing effective therapies.

Cord Platelet Count of Full-Term Neonates in Relation to ABO Incompatibility and Glucose-6-Phosphate Dehydrogenase Levels: A Retrospective Cohort Study.

Background The immunoglobulin G of mothers with O blood type may sensitize the platelets of their neonates with A (O-A incompatibility) or B (O-B incompatibility) blood type. As the expression and antigenicity of the B antigen on platelets is less than that of the A antigens, we have hypothesized that platelet count is higher in the O-B incompatibility group compared to the O-A incompatibility group. There is controversy about whether glucose-6-phosphate dehydrogenase (G6PD) deficiency, without evidence of hemolysis, is associated with a lower platelet count than G6PD-normal. Aim To assess whether platelet count is higher in the O-B than in the O-A incompatible neonates and whether it correlates with their G6PD levels. Methods This study was a retrospective cohort study on a sample of 835 healthy neonates born at ≥38 weeks gestation who were either A or B blood types with mothers that carried the blood type O Rh-positive. The platelet count (thousand per microliter) from umbilical cord venous blood (UCVB) was used. A G6PD level of 11.0 units/gram of hemoglobin (U/g Hb) was considered the lower reference limit. G6PD deficiency was defined as a G6PD level of <3.3 U/g Hb in both sexes. Intermediate G6PD deficiency in females was described as a G6PD level of 3.3-8.8 U/g Hb. Results The mean UCVB platelet count was higher in female neonates compared to male neonates (n=389, 283±65 versus n=446, 272±73, p=0.01). The mean UCVB platelet count was higher in the O-B incompatibility group in both male (n=114, 291±82 versus n=103, 266±63) and female neonates (n=83, 303±66 versus n=81, 278±58) with G6PD levels of >8.8 U/g Hb. There was a positive weak correlation between UCVB platelet counts and G6PD levels only in O-B incompatible female neonates (n=176, r=0.23, p=0.002). The partitioning and combined 95% reference intervals (RIs) of the UCVB platelet count were presented. Conclusion The platelet count was higher in the O-B incompatibility group compared to the O-A incompatibility group, but only when the G6PD level was >8.8 U/g Hb. A correlation between UCVB platelet count and G6PD levels was found only among O-B incompatible female neonates. These findings may have an important implication in estimating RIs of the UCVB platelet count, however, they need to be confirmed and explored in future research.

Congenital Pseudarthrosis of the Clavicle: A Case Report and a Hypothesis for the Right-Side Predominance.

Congenital pseudarthrosis of the clavicle (CPC) is rare. It predominantly affects the right side for an unknown reason. Most of the reported cases are diagnosed outside the neonatal period. Only two CPC cases have been reported in Saudi Arabia, where both were diagnosed during childhood. Here, we present the case of a Saudi male newborn with right-sided CPC. The diagnosis was made shortly after birth because of the uneventful cesarean delivery and painless clavicular lump. Fetuses prefer keeping their head in a right lateral position which may be a plausible explanation for the right-side predominancy in the CPC.

Late-onset neonatal sepsis in Arab states in the Gulf region: two-year prospective study.

OBJECTIVES: This study aimed to investigate the incidence of late-onset sepsis (LOS) in neonatal intensive care units (NICUs) in Arab states in the Gulf region and to describe the main causative organisms and their antibiotic resistance. METHODS: This observational prospective cohort study was conducted over a 2-year period in five NICUs in Kuwait, Saudi Arabia, and the United Arab Emirates. LOS was defined as the growth of a single potentially pathogenic organism from blood or cerebrospinal fluid in infants >3days of age with clinical and laboratory findings consistent with infection. RESULTS: Seven hundred and eighty-five cases of LOS occurred among 67 474 live births. The overall incidence of LOS was 11.63 (95% confidence interval (CI) 10.84-12.47) per 1000 live births, or 56.14 (95% CI 52.38-60.08) per 1000 admissions. Coagulase-negative staphylococci and Klebsiella spp were the most common organisms, causing 272 (34.65%) and 179 (22.80%) of LOS cases, respectively. No evidence of a seasonal variation in the incidence of Klebsiella spp or in the incidence of all Gram-negative organisms was found. More than half of the Klebsiella spp were resistant to third-generation cephalosporins. CONCLUSION: LOS poses a major burden in this area, which could be due to the increasing care of premature babies. Gram-negative organisms, particularly Klebsiella spp, are having an increasing role in LOS in this region, with high levels of resistance to third-generation cephalosporins. NICUs in the area should create a platform through which to share experience in reducing neonatal sepsis and contribute to a common antibiotic stewardship program.

Culture-proven early-onset neonatal sepsis in Arab states in the Gulf region: two-year prospective study.

OBJECTIVE: To investigate the incidence and the pattern of causative organisms of culture-proven early-onset sepsis (EOS) in Arab states in the Gulf region. METHODS: Five neonatal care units participated in this 2-year prospective study in Kuwait, the United Arab Emirates, and Saudi Arabia. Data were collected prospectively using a standardized data collection form. EOS was defined as the growth of a single potentially pathogenic organism from blood or cerebrospinal fluid in infants within 72h of birth, with clinical and laboratory findings consistent with infection. RESULTS: Out of 67 474 live births, 102 cases of EOS occurred. The overall incidence of EOS was 1.5 (95% confidence interval 1.2-1.8) per 1000 live-births, ranging from 2.64 per 1000 live-births in Kuwait to 0.40 per 1000 live-births in King Abdulaziz Hospital in Saudi Arabia. The most common causative organism of EOS was group B Streptococcus (GBS; 60.0%), followed by Escherichia coli (13%). The incidence of invasive GBS disease was 0.90 per 1000 live-births overall and ranged from 1.4 per 1000 live-births in Kuwait to 0.6 per 1000 live-births in Dubai Hospital. CONCLUSIONS: The incidence of EOS and the patterns of the causative organisms in the Arab states in the Gulf region are similar to those in developed countries before the era of intrapartum antibiotic prophylaxis. Efforts should be made to improve intrapartum antibiotic prophylaxis in the Arab state setting, which could avert large numbers of GBS infections.

Associations between spontaneous preterm birth and maternal-newborn ABO blood phenotype pairs.

OBJECTIVE: To study whether spontaneous preterm birth (SPB) is associated with maternal-newborn ABO blood phenotype pairs. METHODS: We conducted a retrospective case-control study in the Department of Pediatrics, King Abdulaziz Hospital, Al-Ahsa, Kingdom of Saudi Arabia. A total of 631 live singleton SPBs (less than 37 weeks) between August 2005 and May 2011 formed the case group. A total of 2,204 live singleton term births (greater than or equal to 37 weeks) between May 2008 and April 2009 formed the control group. We extracted data on the mothers and their newborns from our neonatal electronic database and delivery room log book. We extracted ABO blood phenotypes using Cerner's Lab Information Software. We used a Chi square test to study the association between SPB and maternal-newborn ABO pairs. We used a combination of maternal-newborn A-A, B-B, AB-AB, and O-O pairs as the reference group. We used a binary logistic regression analysis to adjust for 6 established risk factors for SPB. RESULTS: Spontaneous preterm birth was associated with only maternal-newborn pairs B-A (odds ratio: 2.67, 95% confidence interval: 1.35-5.24, p=0.003) and AB-B (odds ratio: 1.97, 95% confidence interval: 1.04-3.74, p=0.04). Both associations remained significant in the regression analysis. CONCLUSION: Spontaneous preterm birth is associated with maternal-newborn B-A and AB-B pairs. This finding requires further confirmatory and exploratory study as it could reduce SPBs.

Saudi mothers' preferences about breaking bad news concerning newborns: a structured verbal questionnaire.

BACKGROUND: Breaking bad news (BBN) to parents whose newborn has a major disease is an ethical dilemma. In Saudi Arabia, BBN about newborns is performed according to the parental preferences that have been reported from non-Arabic/non-Islamic countries. Saudi mothers' preferences about BBN have not yet been studied. Therefore, we aimed to elicit the preferences of Saudi mothers about BBN concerning newborns. METHODS: We selected a convenience sample of 402 Saudi mothers, aged 18-50 years, who had no previous experience with BBN. We selected them via a simple number-randomization scheme from the premises of a level III Saudi hospital between October of 2009 and January of 2011. We used a hypothetical situation (BBN about trisomy 21) to elicit their preferences about BBN concerning newborns via a structured verbal questionnaire composed of 12 multiple-choice questions. We expressed their preferences as percentages (95% confidence interval), and we used the Kendall's W test (W) to assess the degree of agreement in preferences. RESULTS: The Saudi mothers preferred that BBN be conducted with both parents together (64% [60-69]), albeit with weak levels of agreement (W = 0.29). They showed moderate agreement in their preferences that BBN should be conducted early (79% [75-83], W = 0.48), in detail (81% [77-85], W = 0.52), in person (88% [85-91], W = 0.58), and in a quiet setting (86% [83-90], W = 0.53). With extremely weak agreement, they preferred to have a known person present for support during BBN (56% [51-61], W = 0.01), to have close bodily contact with their babies (66% [61-70], W = 0.10), and to have no another patients present (64% [59-68], W = 0.08). They showed moderate levels of agreement in their desires to detail, in advance, their preferences about process of BBN by giving a reversible, written informed consent that could be utilized for guidance, if needed (80% [76-84], W = 0.36). CONCLUSIONS: In our experience, Saudi mothers' preferences about BBN concerning newborns are varied, suggesting that a "one-size-fits-all" approach is inappropriate. A reversible, written informed consent detailing their preferences about BBN that would be kept in their medical records and utilized for guidance, if needed, may be the best solution, given this level of diversity. These findings merit further study.

Hyperbilirubinemia in glucose-6-phosphate dehydrogenase-deficient male newborns in Al-Ahsa, Saudi Arabia.

OBJECTIVE: To study the clinical and laboratory characteristics of hyperbilirubinemia in glucose-6-phosphate dehydrogenase (G6PD)-deficient male newborns from Al-Ahsa area (Ahsais). METHODS: The medical records of inborn male infants at King Abdulaziz Hospital (KAH) in Al-Ahsa area, Kingdom of Saudi Arabia from May 2008 through April 2009 were reviewed. Inclusion criteria were healthy non-polycythemic G6PD-deficient Ahsai males born at less than or equal to 37 weeks gestation, weighing less than or equal to 2.5 kg, with no other cause of hyperbilirubinemia, and were sampled for a total serum bilirubin (TSB) within the first 48 hours of life. Hyperbilirubinemics were compared with non-hyperbilirubinemic newborns. RESULTS: Among the 93 G6PD-deficient newborns that met the inclusion criteria, 67 were hyperbilirubinemic and required phototherapy, and 13 of them required re-phototherapy. Phototherapy was started at 11 +/- 4 (mean +/- SD) hours of life, and for a total duration of 42 +/- 28 hours. Hyperbilirubinemics had statistically significant higher levels of both hematocrit (53 +/- 6 versus 49 +/- 8%, p=0.02) and hemoglobin (176 +/- 18 versus 166 +/- 21 g/L, p=0.04), and lower reticulocyte percentage (4.3 +/- 0.7 versus 5.2 +/- 1.0 %, p=0.02), when compared to non-hyperbilirubinemic newborns. CONCLUSION: Hyperbilirubinemia in G6PD-deficient Ahsai male newborns was characterized by higher levels of both hematocrit and hemoglobin levels, and lower reticulocyte percentage compared to their non-hyperbilirubinemic counterpart. This hyperbilirubinemia required early phototherapy and re-phototherapy. Appropriate follow up should be made available to those high-risk newborns. Further research is needed to understand the exact mechanism of hyperbilirubinemia in G6PD-deficient newborns.

Pulmonary interstitial emphysema and continuous positive airway pressure in a premature infant.

Pulmonary interstitial emphysema PIE is a recognized sequel of high pressure mechanical ventilation. Nevertheless, with the improvement in perinatal medical care, PIE started to be seen in spontaneously breathing infants. We present a 29-week-old girl who developed PIE on the first day of life, while she was on nasal continuous positive airway pressure, and was successfully managed with high frequency oscillatory ventilation. There is a paucity of these cases in the literature. We report on the occurrence of this entity, early reorganization, and the possible associated or causation factors.