Proactive patient safety: enhancing hospital readiness through simulation-based clinical systems testing and healthcare failure mode and effect analysis.

BACKGROUND: Recognizing and identifying latent safety threats (LSTs) before patient care commences is crucial, aiding leaders in ensuring hospital readiness and extending its impact beyond patient safety alone. This study evaluated the effectiveness of a combination of Simulation-based Clinical Systems Testing (SbCST) with Healthcare Failure Mode and Effect Analysis (HFMEA) with regard to mitigating LSTs within a newly constructed hospital. METHODS: Three phases of the combined SbCST and HFMEA approach were implemented across all hospital settings. The scenarios tested system functionalities, team responses, and resource availability. The threats thus identified were categorized into system-related issues, human issues, and resource issues, after which they were prioritized and addressed using mitigation strategies. Reassessment confirmed the effectiveness of these strategies before hospital commissioning. RESULTS: More than 76% of the LSTs were mitigated through the combined approach. System-related issues, such as nonfunctional communication devices and faulty elevators, were addressed by leadership. Human issues such as miscommunication and nonadherence to hospital policy led to improvements in interprofessional communication and teamwork. Resource issues, including missing equipment and risks of oxygen explosion, were addressed through procurement, maintenance, and staff training for equipment preparation. CONCLUSION: The SbCST and HFMEA were highly effective with regard to proactively identifying and mitigating LSTs across all aspects of hospital preparedness. This systematic and comprehensive approach offers a valuable tool for enhancing patient safety in new healthcare facilities, thereby potentially setting a new standard for proactive hazard identification and risk management in the context of healthcare construction and commissioning.

Evaluating a Modified Use of the Kaiser Permanente Early-onset Sepsis Risk Calculator to Reduce Antibiotic Exposure: a Retrospective Study.

BACKGROUND: Early-onset neonatal sepsis (EONS) remains an important disease entity due to very serious adverse outcomes if left untreated. Lack of diagnostic tools in identifying healthy from diseased neonates, and clinicians' fear of the missing positive-culture sepsis babies, or babies with clinical sepsis have led to overtreating and unnecessary antibiotic exposure. Kaiser Permanente EONS risk calculator is an internally validated tool that can predict EONS. This sepsis risk calculator (SRC) classifies neonates into three subgroups: (1) ill-appearing, (2) equivocal and (3) well-appearing. We propose a modification to this tool that aims to use it solely for well-appearing babies. This modification represents a more conservative approach to decrease antibiotic exposure and offers an alternative for those hesitant to fully implement this tool. METHODS: This is a dual-centre retrospective study where data were extracted from the electronic medical records. Our primary outcome was to validate the modified use of the SRC with a two-by-two table. Specificity, negative predictive value and expected antibiotic reduction were used to evaluate the tool's feasibility. RESULT: Among 770 babies suspected of EONS, the feasibility of the modified use was tested. The expected antibiotic exposure reduction rate on the modification was 40.4% overall. The proposed modification resulted in a specificity and negative predictive value of 99.28% (95% CI: 97.92% to 99.85%) and 99.5% (95% CI: 99% to 99.8%), respectively. CONCLUSION: The modified use of the sepsis risk calculator has shown that it can safely reduce antibiotic exposure in well-appearing babies. The modified use is used as a 'rule out' test that can identify very low risk of EONS babies, and safely minimise antibiotic exposure. Further prospective studies are needed to examine the efficacy of this use, and quality improvement projects are required to evaluate its applicability in different clinical settings.

Bardet-Biedl Syndrome Presenting With Bifid Epiglottis: A Case Report and Review of Literature.

Bifid epiglottis is a rare congenital laryngeal anomaly that is most commonly a syndromic rather than an isolated entity. It has been associated with specific syndromes, such as Pallister-Hall syndrome, Bardet-Biedl syndrome, and other related syndromes. Bardet-Biedl syndrome is a rare autosomal-recessive disorder characterized by hand and/or feet polydactyly, obesity, short stature, mental retardation, renal anomalies, and genital anomaly. Here we report a case involving a 25-year-old Saudi male patient who presented with hoarseness of voice since birth with no diurnal or diet association or other associated symptoms. On examination, he was noted to have craniofacial dysmorphism and polydactyly of the right hand and left foot. Fiberoptic nasopharyngolaryngoscopy (NPLS) revealed a laryngeal pedunculated rounded glottic mass and subglottic bulging with expiration and involuting with inspiration along with an abnormal-looking epiglottis having a separate cartilaginous framework with space in-between and bilateral mobile vocal cords. Computed tomography (CT) showed the vocal cord mass and a bifid epiglottis. Other investigations and labs were within normal range. The patient underwent vocal cord mass excision and soft tissue histopathology revealed a benign growth. On follow-up, the patient showed clinical improvement. In conclusion, this is a rare case of bifid epiglottis associated with Bardet-Biedl syndrome, which serves to highlight the significance of such anomalies in any syndromic patient presenting with airway symptoms. Our aim is to add more cases to the literature and to consider it as a differential diagnosis.

Outcomes of Cranioplasty: A Single-Center Experience.

Background Cranioplasty (CP) is a common cranial reconstructive procedure. It is performed after craniectomy due to various causes such as relieving increased intracranial pressure, infection, and tumor infiltration. Although CP is an easy procedure, it is associated with a high rate of complications. We aimed to retrospectively investigate the outcomes of CP at the King Abdulaziz Medical City, Ministry of National Guard Health Affairs, Jeddah (KAMC-J). Methods This is a retrospective observational study that included all patients who had CP (first time or redo) at KAMC-J from 1st January 2010 to 31st December 2020. Patients with congenital cranial malformation were excluded.  Result A total of 68 patients underwent CP. Of those, 23 (34%) had complications. The most common complication was infection (10.3%). Twelve of the 23 patients had major complications that necessitated reoperation. Of those 12, six underwent redo CP; three out of these six patients had further complications which were also managed surgically. On bivariate analysis, cranial defects over 50 cm² were associated with a higher rate of both infection and hydrocephalus (p=0.018) while the frontal site was associated with a higher rate of infection (p=0.014). Moreover, traumatic brain injury as an etiology was exclusively associated with post-cranioplasty hydrocephalus (p=0.03).  Conclusion Patients undergoing CP after craniectomy are prone to a considerably high rate of adverse outcomes. The overall rate of complications in this study was 34%, with an infection rate of 10.3% and a 1.5% mortality rate. Consistent with other studies, larger cranial defects as well as frontal sites have a higher rate of infection.

The Outcomes of Diffuse Large B-cell Lymphoma Patients with Synchronous and Early Central Nervous System Involvement: A Single-Center Experience.

BACKGROUND: Diffuse large B cell lymphoma (DLBCL) is the most commonly diagnosed subtype of non-Hodgkin's lymphoma (NHL). R-CHOP has significantly improved clinical outcomes in patients with DLBCL, however, its indication in the prevention of CNS relapse and recurrence is still inconsistent. Moreover, prophylactic methotrexate and/or cytarabine have been used prophylactically for DLBCL patients is at high risk of CNS relapse and to treat CNS DLBCL, however, their efficacy remains unclear. METHODS: The aim of our retrospective study was to determine the incidence of CNS in-volvement in patients with DLBCL and to describe its risk factors and survival outcomes. RESULTS: A total of 406 patients with DLBCL were identified, and 17 (4.2%) of DLBCL patients had CNS involvement i.e. 9 (2.2 %) at diagnosis and 8 (~2%) at relapse. The patients were younger, had advanced stage, high CNS-IPI, and had extra nodal involvement. Seven out of the 17 patients who survived received chemotherapy and a prophylactic methotrexate. Considering the CNS-IPI, of the 146 patients with high CNS-IPI at presentation, 18 received the prophylactic HDMTX and 3 (16.7%) of them had CNS relapse. Two (1.6%) out of 128 who did not receive the prophylactic HDMTX had CNS relapse. On the other hand, of the 223 patients with intermediate CNS-IPI, 25 received the prophylactic HDMTX and 2 (8%) of them had CNS relapse and in 198 patients who did not receive the prophylactic HDMTX, 2 (1.01%) had CNS relapse. The 5-year progression-free survival and overall survival rates for the entire cohort were 73% and 84%, respectively. The median OS for those who had CNS involvement was 17 months and the 2-year OS was 40%. CONCLUSION: CNS involvement in DLBCL has a poor prognosis, thus, aggressive CNS-directed therapy should be considered, especially in young patients.

Approach to Ankle Instability in Patients With a Negative Ankle MRI: A Case Series.

Objectives Injuries to the ankle ligaments are some of the most common musculoskeletal sports injuries. Ankle magnetic resonance imaging (MRI) is the standard diagnostic procedure in today's practice, but its reliability and validity remain controversial. The aim of this study was to explore the approach for patients with negative ankle MRI who continue to have symptoms of ankle instability despite conservative therapy. Methods A total of eight patients who were 14 years or older with negative ankle MRI who continue to have symptoms of ankle instability despite conservative therapy were admitted to our institution from January 1, 2015 to December 31, 2021. Results Eight patients with a mean age of 36, and a mean body mass index (BMI) of 37.7. All patients presented with ankle pain, locking, and giving way in variable severity. All the patients had a radiograph followed by an ankle MRI, which showed normal alignment of ankle joints without abnormality. Initially, all patients were treated conservatively but did not show any improvement. After that, they underwent an operation of lateral ankle ligament reconstruction by modified Brostrom technique, followed by casting and physiotherapy. The symptoms of ankle instability resolved in all patients. The ankle pain resolved completely in six patients, improved in one patient, and did not improve in one patient.  Conclusion Based on our results, we advocate reconstruction surgery by modified Brostrom technique for ligament repair in patients with clinical evidence of chronic ankle instability who have failed a trial of conservative management, even in the context of a normal ankle MRI.

A Multidisciplinary and a Comprehensive Approach to Reducing Fragility Fractures in Preterm Infants.

With advances in neonatal care, bone fractures prior to discharge from the hospital in preterm infants receiving contemporary neonatal care, are rare. Nevertheless, such fractures do occur in very low birth weight and extremely low birth weight infants who go on to develop metabolic bone disease of prematurity (MBDP), with or without secondary hyperparathyroidism. MBDP is a multifactorial disorder arising from the disruption of bone mass accrual due to premature birth, postnatal immobilisation, and loss of placental oestrogen resulting in bone loss, inadequate provision of bone minerals from enteral and parenteral nutrition, and medications that leach out bone minerals from the skeleton. All of these factors lead to skeletal demineralisation and a decrease in bone strength and an increased risk of fractures of the long bones and ribs. Secondary hyperparathyroidism resulting from phosphate supplements, or enteral/parenteral feeds with a calcium-to-phosphate ratio of < 1.3:1.0 leads to subperiosteal bone resorption, cortical thinning, and further skeletal weakening. Such fractures may occur from routine handling and procedures such as cannulation. Most fractures are asymptomatic and often come to light incidentally on radiographs performed for other indications. In 2015, we instituted a comprehensive and multidisciplinary Neonatal Bone Health Programme (NBHP), the purpose of which was to reduce fragility fractures in high-risk neonates, by optimising enteral and parenteral nutrition, including maintaining calcium-to-phosphate ratio ≥1.3:1, milligram to milligram, biochemical monitoring of MBDP, safe-handling of at-risk neonates, without compromising passive physiotherapy and skin-to-skin contact with parents. The at-risk infants in the programme had radiographs of the torso and limbs at 4 weeks and after 8 weeks from enrolment into the program or before discharge. Following the introduction of the NBHP, the bone fracture incidence reduced from 12.5% to zero over an 18-month period.

Elevated level of creatine phosphokinase in newborn: Clinical significance and association with congenital muscle diseases.

OBJECTIVES: To establish the normal Creatine phosphokinase (CPK) range in newborns of all 3 modes of delivery and prove that high CPK level in neonates is not specific a indicator for muscular pathology. METHODS: This is a prospective cohort study that is conducted in King Abdulaziz Medical City and King Abdullah Specialized Children Hospital in Riyadh and included 504 term neonates who were born between March 2021 and August 2021. Two hundred and fifty three were males and 251 were females. Data and consents were managed and collected using 2 coordinators. RESULTS: Duration of the second stage of labor, age on the first CPK test and fetal gestational age were significantly correlated with CPK values (r=0.197, r=-0.234, r=0.274, respectively). The normal ranges for each delivery type were 334 U/L-2667U/L in normal spontaneous vaginal delivery, 265U/L-1182U/L in elective cesarean section, and 223U/L-3082 U/L in emergency cesarean section. CONCLUSION: The CPK was elevated in all neonates in all 3 modes of deliveries. An elevated levels of CPK in neonates is not a specific indicator for any congenital muscular pathology.

Opioid-induced respiratory depression and risk factors in a tertiary hospital: A retrospective study.

Background: Opioids are potent analgesics used for the treatment of moderate to severe acute and chronic cancer and non-cancer pain. However, opioid usage may be limited by negative side effects, such as potentially life-threatening respiratory depression. Objectives: The aim of our study is to investigate the prevalence of opioid-induced respiratory depression (OIRD) and its predictors at King Abdulaziz Medical City in Jeddah (KAMC-JD). Method: This is a retrospective cross-sectional (chart review) study conducted from January 1, 2016, to December 31, 2020. Results: A total of 15,753 patients received opioids during admission to KAMC-JD, and only 144 (0.915%) of them received naloxone from January 1, 2016 to December 31, 2020. Only 91 patients (0.57%) developed opioid-induced respiratory depression (OIRD), which was more frequently reported among young and middle-aged adults. OIRD was significantly associated with receiving a daily morphine milligram equivalent (MME) dose of ≥150 MME and with having a low urea concentration at the baseline and at admission under surgery. Also, fentanyl use remained a significant risk factor for OIRD. Conclusion: In conclusion, monitoring patient receiving opioids with a daily MME dose of ≥150 MME, prescribed Fentanyl, low urea concentration at the baseline, and patients' admissions to the surgery department may mitigate the risk of developing OIRD.

The Practice of Pain Assessment and Management in a Tertiary Oncology Center.

Background Pain is one of the common and devastating symptoms that affects millions of cancer patients globally. Despite published guidelines and education on the assessment and management of cancer-related pain, underestimated or undertreated pain continues to be a considerable worldwide public health concern among cancer patients. In this study, we aimed to assess physicians' adherence to the World Health Organization (WHO) guidelines in the management and assessment of pain in oncology patients based on the available score of pain in the Princess Noorah Oncology Center (PNOC) at the King Abdulaziz Medical City in Jeddah. Methodology This cross-sectional, retrospective chart review study studied 451 patients (selected through computerized random sampling) who were admitted to the PNOC during the study period. Results The pain was assessed using the Brief Pain Inventory in almost all patients (n = 450, 99.8%). The pain was categorized as mild in 386 (85.6%) patients, moderate in 46 (10.2%) patients, and severe in 19 (4.2%) patients. Opioid prescriptions were significantly higher among patients with moderate (76.1%) and severe pain (89.5%) compared to those with mild pain (39.1%; p < 0.0001). Conclusions The practice of pain documentation for cancer patients was adequate as indicated by reporting the pain scores of 99.8% of inpatients. Patients with moderate and severe pain were more likely to receive opioids and a combination of opioids plus non-opioid analgesics, whereas the prescription of analgesics was predicted by experiencing moderate cancer pain.

Child mortality in Saudi Arabia: Time for action at all levels.

BACKGROUND AND OBJECTIVES: Although child mortality is declining in Saudi Arabia, new trends and causes are emerging. The objective of the study is to determine the causes of child death in a tertiary care hospital in Saudi Arabia and to identify its preventable causes and associated risk factors. METHODS: A modified UNICEF Multiple Indicator Cluster Survey (MICS) was used to analyze all deaths among children under the age of 18 which occurred at the King Abdullah Specialized Children's Hospital (KASCH) between 2010 and 2016. RESULTS: After reviewing all the death charts of 1138 children, the team determined that 15% (172) of all deaths could have been prevented and the preventability increased with age. Only 2% of the neonates died of preventable causes, while 53% of the children of 6 years of age or older died of preventable causes. The highest percentage of preventable deaths occurred in children aged 13-18 years (39.3%), followed by the age group of 6-12 years (32.4%) and the age group of 29 days to 5 years (13.9%). All 966 (85%) deaths from biological causes were considered to be unpreventable. Among the preventable causes, 142 (82.5%) had injuries and 30 (17.4%) were sudden unexpected infant death (SUID) with no documented autopsy or death scene investigation, and thus it was considered preventable by the researchers. The 5 major causes of deaths secondary to injuries were motor vehicle accidents (MVA) accounting for 86 deaths (60.6%), followed by drowning accounting for 19 deaths (13.4%), child maltreatment accounting for 13 deaths (9.2%), fire and weapon accounting for 12 deaths (8.5%), and finally home accident (fall, poisoning, suffocation) accounting for 12 deaths (8.5%). CONCLUSION: The State Child Death Reviews Board should thoroughly investigate deaths due to SUID and injuries by identifying the factors that contribute to the implementation of preventive strategies.

Framework for obstetrics and gynecology department change management in response to COVID-19 pandemic: A tertiary center experience.

Coronavirus (cov) disease 2019 pandemic caused by severe acute respiratory syndrome cov 2 has imposed significant demands on healthcare systems across the world. These demands were more significant on obstetrics and gynecology (obgyn) patients, who required services that had to continue despite the closure of other services. This paper describes the change management of an obgyn department at a tertiary health-care center. That experience resulted in a complete management shift in the institution and the formation of an infectious disease epidemic plan for respiratory infections. Description of the change management performed, difficulties encountered, and achievements obtained can assist other departments change management when they face similar situations.

Antimicrobial consumption in three pediatric and neonatal intensive care units in Saudi Arabia: 33-month surveillance study.

BACKGROUND: Despite their critical role in antimicrobial stewardship programs, data on antimicrobial consumption among the pediatric and neonatal population is limited internationally and lacking in Saudi Arabia. The current study was done as part of our antimicrobial stewardship activities. OBJECTIVES: To calculate overall and type-specific antimicrobial consumption. METHODS: A prospective surveillance study was conducted at King Abdulaziz Medical City, Riyadh, Saudi Arabia, between October 2012 and June 2015 in two pediatric and one neonatal intensive care units (ICUs). Consumption data were collected manually on a daily basis by infection control practitioners. Data were presented as days of therapy (DOT) per 1000 patient-days and as frequency of daily consumption. RESULTS: During the 33 months of the study, a total of 30,110 DOTs were monitored during 4921 admissions contributing 62,606 patient-days. Cephalosporins represented 38.0% of monitored antimicrobials in pediatric ICUs followed by vancomycin (21.9%), carbapenems (14.0%), aminoglycosides (8.8%), and piperacillin/tazobactam (8.8%). Their consumption rates were 265.1, 152.6, 97.6, 61.4, and 61.4 DOTs per 1000 patient-days (respectively). Aminoglycosides represented 45.4% of monitored antimicrobials in neonatal ICU followed by cephalosporins (30.4%) vancomycin (13.6%), and carbapenems (8.3%). Their consumption rates were 147.5, 98.7, 44.3, and 27 DOTs per 1000 patient-days (respectively). CONCLUSION: Cephalosporins are frequently used in pediatric ICU while aminoglycosides are frequently used in neonatal ICU. The local consumption of cephalosporins and carbapenems in both ICUs is probably higher than international levels. Such data can help in establishing and monitoring the functions of antimicrobial stewardship activities aiming to ensure judicious consumption of antimicrobials.

Neonatal Group B Streptococcal Infection in a Tertiary Care Hospital in Saudi Arabia: A 13-year Experience.

BACKGROUND: Group B streptococcus (GBS) is a leading cause of neonatal bacterial sepsis and meningitis globally. Studies concerning the incidence and burden of neonatal GBS disease in Saudi Arabia are lacking. This study determined the incidence and burden of GBS infection among neonates in association with maternal GBS screening. METHODS: A retrospective cohort chart review study included all neonatal GBS disease cases identified through microbiology lab records within the first 90 days of life in the hospital from January 2004 to December 2016. Charts were reviewed to collect maternal and neonatal characteristics using a standardized form. RESULTS: Over 13 years, of 108,609 live births, 55 GBS disease cases were identified (overall incidence, 0.51/1000 live births), 69.1% (n = 38) of those had early onset disease (EOD). The annual incidence in 2015 and 2016 was significantly higher than in any previous year (P < 0.0001), coinciding with the discontinuation of routine universal maternal GBS screening. Median age at presentation was 1 day (range, 0-54 days). We found that 67.3% (n = 37) of mothers were not screened antenatally, 72.9% (n = 27) of whom had neonates present with EOD. Neonates of unscreened mothers were more likely to have GBS disease (P = 0.01) and to present with EOD (P = 0.005). Urinary tract infection was the most common manifestation (47.3%, n = 26), followed by sepsis (43.6%, n = 24). Mortality rate was 3.6% (n = 2). CONCLUSIONS: The incidence of neonatal GBS infection in Saudi Arabia is similar to the worldwide incidence. Universal antenatal screening discontinuation was significantly associated with an increase in EOD incidence.

Intravitreal Ranibizumab Injection for the Treatment of Retinopathy of Prematurity.

OBJECTIVES: To evaluate the efficacy of a single injection of 0.3 mg intravitreal ranibizumab for the treatment of retinopathy of prematurity (ROP). METHODS: We conducted this retrospective case series study at King Abdul Aziz Medical City, Riyadh, Saudi Arabia. Seventy-four eyes of 37 preterm infants with ROP stage III with plus disease in zone I, posterior zone II, and aggressive posterior ROP received a single injection of 0.3 mg intravitreal ranibizumab. The favorable outcome measure was complete regression of the disease with normal vascularization of the retina of those infants. RESULTS: The gestational age of the 37 included cases was in the range of 23-28 weeks and their body weight at birth was between 510 and 1,235 g except for one case with 2,550 g under oxygen therapy <7days with severe hypoglycemia. All eyes showed a favorable response in terms of regression of plus disease from the first day after treatment, followed by regression of stage III retinopathy. All patients developed complete vascularization over variable periods of time. CONCLUSION: One injection of 0.3 mg intravitreal ranibizumab is effective in treating ROP stage III mainly in zones I and II.

Prospective study of early and late outcomes of extremely low birthweight in Central Saudi Arabia.

BACKGROUND: Survival of preterm neonates has steadily improved over the past five decades, due to changes in the neonatal intensive care. However, in Saudi Arabia, there are no written guidelines on the definition of the lower limit of viability, and there has been a call for such a limit. The aims of this study were: (1) to determine lower limits of viability and survival in extremely low birthweight (ELBW) infants, and (2) to determine incidence of neurodevelopmental and cognitive abnormalities within 3-6 years after birth. METHODS: Prospective study of all live inborn ELBW infants admitted to the neonatal unit of King Abdulaziz Medical City, Riyadh, Saudi Arabia, within 3 years [between January 1st, 2005 and December 31st, 2007] was conducted (n = 117). Data were collected on demographic and birth data, neonatal complications & interventions and death on discharge. Prospective follow up of all survivors was done, within 6 years after birth, to assess the outcome in terms of neurodevelopmental and cognitive abnormalities. Predictors of survival were determined using logistic regression model. Significance was considered at p-value ≤0.05. RESULTS: Of all ELBW infants, 41% died before discharge. Survival rate was directly correlated with gestational age (GA) and birthweight (p < 0.05). The 50% limits of viability were those at 25 weeks' gestation or with > 600 g. After adjusting for possible confounders, significant predictors of survival were birthweight (p = 0.001) and Apgar score (p < 0.001). The following impairments were reported during follow up of survivors: developmental delay (39.2%), cerebral palsy (36.2%), speech problems (33.3%), wasting (12.5%), intellectual disability (10%), visual problems (6.6%) and hyperactivity (5.6%). CONCLUSION: More than one-third of ELBW died before discharge from NICU, and two-thirds of survivors had one or more neurodevelopmental and/or cognitive abnormalities during their first 6 years of life. The 50% limits of viability of ELBW infants were those at week 25 of gestation or with a birthweight of more than 600 g. Birthweight could be considered as more valid than gestational age in the prediction of viability of ELBW infants. The process of care of ELBW infants in Saudi Arabia may need to be revisited taking these findings into consideration.

Expanded Newborn Screening Program in Saudi Arabia: Incidence of screened disorders.

AIM: To address the implementation of the National Newborn Screening Program (NBS) in Saudi Arabia and stratify the incidence of the screened disorders. METHODS: A retrospective study conducted between 1 August 2005 and 31 December 2012, total of 775 000 newborns were screened from 139 hospitals distributed among all regions of Saudi Arabia. The NBS Program screens for 16 disorders from a selective list of inborn errors of metabolism (IEM) and endocrine disorders. Heel prick dry blood spot samples were obtained from all newborns for biochemical and immunoassay testing. Recall screening testing was performed for Initial positive results and confirmed by specific biochemical assays. RESULTS: A total of 743 cases were identified giving an overall incidence of 1:1043. Frequently detected disorders nationwide were congenital hypothyroidism and congenital adrenal hyperplasia with an incidence of 1:7175 and 1:7908 correspondingly. The highest incidence among the IEM was propionic acidaemia with an incidence rate of 1:14 000. CONCLUSION: The article highlights the experience of the NBS Program in Saudi Arabia and providing data on specific regional incidences of all the screened disorders included in the programme; and showed that the incidence of these disorders is one of the highest reported so far world-wide.

Risk factors for isolated periventricular leukomalacia.

Periventricular leukomalacia, a major cause of neurologic disabilities in preterm infants, can be isolated or associated with intraventricular and periventricular hemorrhage. To determine the risk factors for isolated periventricular leukomalacia, we retrospectively studied the characteristics of all very low birth weight infants affected by isolated periventricular leukomalacia who were delivered over a 5-year period and compared them with a control group of very low birth weight infants, matched within 2 weeks for gestational age, with no central nervous system pathology, and born during the same period. In total, 20 affected infants were compared with 98 control infants. Neonatal sepsis caused by coagulase-negative Staphylococcus (P = 0.014) and neonatal seizure (P = 0.026) were associated with isolated periventricular leukomalacia only on univariate analysis. Three variables demonstrated statistically significant associations with isolated periventricular leukomalacia on both univariate and multivariate logistic regression analysis as independent risk factors: birth weight (odds ratio, 4.31; 95% confidence interval, 1.54-12.06; P = 0.005), early neonatal hypotension requiring combined inotropic therapy (odds ratio, 4.90; 95% confidence interval; 1.22-19.68, P = 0.025), and delayed surgical closure of hemodynamically significant patent ductus arteriosus beyond age 7 days (odds ratio, 1.20; 95% confidence interval, 1.06-1.35; P = 0.003).

CO(2) inhalation as a treatment for apnea of prematurity: a randomized double-blind controlled trial.

OBJECTIVE: To compare the effect of prolonged inhalation of a low concentration of CO(2) with theophylline for the treatment of apnea of prematurity. STUDY DESIGN: Prospective, randomized, double-blind controlled trial of 87 preterm infants with apnea of prematurity (27-32 weeks' gestational age) assigned to either theophylline plus 0.5 L/min of room air via nasal prongs or placebo plus 0.5 L/min with CO(2) (about 1% inhaled) by nasal prongs for 3 days. RESULTS: Apnea time significantly decreased in the theophylline group from 189±33 s/h (control) to 57±11, 50±9, and 61±13 (days 1-3) (P=.0001) and in the CO(2) group from 183±44 (control) to 101±26, 105±29, and 94±26 s/h (days 1-3) (P=.03). Seven infants in the CO(2) group but none in the theophylline group failed to complete the study due to severe apneas (P=.003). CONCLUSIONS: Because theophylline was more effective in reducing the number and severity of apneas, inhalation of low concentration of CO(2), as used in the present study, cannot be considered as an alternative to theophylline in the treatment of apnea of prematurity. The less effectiveness of CO(2) treatment may have been related to the variability of the delivery of CO(2).

Perioperative course of pulmonary hypertension in infants with congenital diaphragmatic hernia: impact on outcome following successful repair.

BACKGROUND: Pulmonary hypertension (PH) associated with congenital diaphragmatic hernia (CDH) remains a significant cause of morbidity and mortality. For improved outcomes, preoperative stabilization is commonly practiced to control the PH in infants with CDH. Some CDH infants who have been considered stabilized and ready for surgery have nevertheless developed significant PH after surgical repair. In fact, the markers and consequences of the preoperative stabilization are still unclear. Therefore, we examine the perioperative course of PH to evaluate the impact of preoperative PH severity on mortality and morbidity of infants who underwent surgical repair of CDH. METHODS: The medical charts of all newborns (n = 49) with CDH who were treated at our institution between January 2000 and December 2009 were reviewed. General management and perioperative data were evaluated for all infants. The ratio of estimated pulmonary artery pressure to systemic pressure (P/S ratio or PSR), based on echocardiographic data, was used to assess the PH severity during the perioperative period. RESULTS: The overall survival rate in our group of infants with CDH was 71.4%. Of the 49 infants with CDH, 9 (18.4%) died during the preoperative phase. Forty infants underwent CDH repair at a median age of 3.5 days (range, 1-46 days). Five of these infants (12.5%) subsequently deteriorated and died after surgery. Using receiver operating characteristic curve analysis, a PSR cutoff value before surgery of 0.9 predicted mortality in CDH infants with a sensitivity of 100% and specificity of 84% and with an area under the curve of 0.93 (P = .002). Accordingly, 2 groups of infants with distinct outcomes were identified, as follows: a low-PSR cohort (PSR ≤0.9) with a survival rate of 100% and a high-PSR cohort (PSR >0.9) with a survival rate of 50% (P = .001). The rate of pneumothorax and the frequency of use of several inotropic agents after surgery were significantly higher in the high-PSR group (P = .001 and .007, respectively). Compared with low-PSR infants, infants with high PSR were operated on later (P = .03) and were postoperatively ventilated longer (P = .01). During the entire perioperative period, significant differences in the PH severity were noted between the 2 PSR groups. During the first week of life, infants in the high-PSR group had significantly higher PSRs than those in the low-PSR group (P = .001); and similar tendencies continued to be significant between the 2 groups after CDH repair (P = .04). CONCLUSIONS: During the perioperative period, PH severity monitoring via the serial assessment of PSR is beneficial. Better outcomes were observed with a preoperative PSR less than or equal to 0.9, and this association needs to be confirmed by prospective study.