Emerging Data from a Newborn Hearing Screening Program in Sharjah, United Arab Emirates.

OBJECTIVES: Newborn hearing screening (NHS) plays a critical role in early identification of hearing loss and subsequent early habilitation. Active parental involvement influences the success of NHS, particularly the initial NHS and follow-up. The current study reports the results of an NHS program in a cohort of babies born in a tertiary care hospital in Sharjah, United Arab Emirates (UAE). Further, it explores a two-stage NHS model to reduce false responses, thereby alleviating parental anxiety. METHODS: Retrospective observational study was conducted for a period of five years from January 2017 to December 2020. NHS was done as a two-stage model. All the healthy newborn babies were screened using Automated Auditory Brainstem Response (AABR) by trained audiology professionals. Babies who failed the first NHS were followed up after two weeks. Further, babies that failed the follow-up NHS were sent for diagnostic hearing evaluation and intervention as necessary. RESULTS: A total of 1821 newborn babies were screened during the study period. Eighty-one percent of babies passed the initial NHS. Four hundred and twenty-three (23.22%) babies were referred on the first NHS and were followed up after 2 weeks. Among these babies, 7.03% (24) failed second NHS. Nine (37.50%) of the 24 babies were confirmed to have hearing loss in both ears. The incidence of hearing loss in our cohort was 4.94 per 1000. Confirmed hearing loss was statistically higher in boys than girls (p < 0.05). CONCLUSION: Current study was an attempt to report the emerging NHS data as part of the implementation of an NHS program. The study findings emphasize the need for a two-stage model of NHS to rule out false responses.

Knowledge and Attitudes of Parents towards Childhood Hearing Loss and Pediatric Hearing Services in Sharjah, United Arab Emirates.

The successful implementation of pediatric audiology services depends on parental engagement and support. It is essential to analyze the gaps in knowledge level and attitude of the parents in United Arab Emirates (UAE), towards pediatric hearing loss and pediatric audiology services. The present study aimed to assess the knowledge and attitude of parents in Sharjah, UAE.A cross-sectional survey was administered to 295 parents in a tertiary care hospital setting. The self-reported questionnaire consisted of 26 items. 34.2% of the parents ascertaining good knowledge and 65.8% reported poor knowledge regarding the various factors related to the childhood hearing loss. Further, 86.2% of parents reported positive attitudes regarding accessing pediatric audiology services. A significant association was found between age groups, educational status, and knowledge levels. The study highlights the poor knowledge demonstrated by parents in the UAE regarding hearing loss and its associated risk factors. Findings outline the critical need in the region to enhance parental awareness. More health promotion activities and community outreach campaigns are necessary to increase the uptake of pediatric audiology services in the region.

Burden and genotyping of rotavirus disease in the United Arab Emirates: a multicenter hospital-based surveillance.

Rotavirus (RV) is the most common etiological agent causing acute gastroenteritis (GE) in children aged <5 years. This cross-sectional, hospital-based surveillance study (NCT01201252) was designed to investigate RVGE disease burden. It was conducted from July 2009-July 2010 at 3 referral hospitals in the United Arab Emirates (UAE). Children who had been hospitalized for acute GE were enrolled with informed consent. Stool samples were tested for RV using enzyme immunoassay and RV-positive samples were further typed using reverse transcriptase-polymerase chain reaction and reverse hybridization to determine the G and P types. GE data were collected from medical charts and GE severity was assessed through clinical examination. Treatment and outcome were prospectively recorded. Among 6323 children hospitalized due to any reason, 771 (12.2%) presented acute GE and were enrolled, of whom 758 (98.3%) were included in the final analysis. Acute GE and RVGE accounted for 12.0% (758/6323) and 6.0% (381/6323) of all hospitalizations, respectively. RVGE accounted for 50.3% (381/758) of GE hospitalizations and predominantly affected, children younger than 2 years (66.1%; 252/381). The severity of GE before hospitalization was significantly associated with RV-positive status (P = 0.0031). The majority (>95%) of children received intravenous hydration during hospitalization. RVGE occurred throughout the year, with a subtle winter peak in February 2010 (63.6%; 56/88). G1WTP[8]WT was the most commonly detected RV strain (56.3%) in 268 analyzed samples. RV was a major cause of GE-hospitalizations in children under 5 years in the UAE; the highest number of RVGE cases was observed in children younger than 2 years.

Proteus syndrome and hypothyroidism. An unusual association.

We present a case of a 3½-year-old girl diagnosed as Proteus syndrome with severe cosmetic disfigurement-macrodactyly, hemi-hypertrophy of the face and limbs, megalencephaly, lymph edema of both hands and feet along with severe global developmental delay. She was found to have severe recalcitrant epilepsy and also primary hypothyroidism; the association of which is not mentioned in the previous literature.

Is sildenafil an effective therapy in the management of persistent pulmonary hypertension?

Persistent pulmonary hypertension of the newborn (PPHN) is a life-threatening neonatal pathology resulting from poor hemodynamic and respiratory transition to extra uterine life. Inhaled nitric oxide (iNO) is a current, commonly used treatment of PPHN. However, iNO is not available therapy in many developing countries and around 50% of infants with PPHN do not respond to iNO therapy. Sildenafil is a phosphodiesterase inhibitor type 5 (PDE5) that has been shown to selectively reduce pulmonary vascular resistance in both animal models and adult humans. Recent studies have found that in PPHN, administration of Sildenafil was associated with a significant increase in the oxygenation and a reduction in mortality with no clinically important side effects.

Nonoliguric hyperkalemia in neonates: a case-controlled study.

The objective of this study was to determine the incidence, risk factors, and morbidities associated with nonoliguric hyperkalemia (NOHK) in neonates. Infants were eligible for the study if they were born at Al Qassimi Hospital and fulfilled the diagnostic criteria of NOHK (serum potassium [SK] > or = 7 mmol/L during the first 72 hours of life with urinary output > or = 1 mL/kg/h). The next admitted infant with gestational age +/- 1 week and normal SK acted as control. Exclusion criteria were severe congenital malformation, renal failure, failure of adequate urinary collection, and early blood transfusion within the first 72 hours of life. Fluid intake and urinary output were monitored. Infants who developed hyperkalemia (SK > or = 6.5 mmol/L in two nonhemolysed venous or arterial blood samples) had serum potassium measured every 4 hours until it reached below 6 mmol/L. Hyperkalemia was identified between 6 and 36 hours of age in 45 infants (peak SK 7 to 9.3 mmol/L). During the time of the study, the prevalence of NOHK was 24% among extremely-low-birth-weight infants (with birth weight < 1000 g) who comprised 83% of those identified. Of infants with NOHK, 13% developed tachycardiac arrhythmia and 17% died. NOHK was significantly associated with fetal distress, early metabolic acidosis, early hyperglycemia, and absence of antenatal steroid administration. Hyperkalemic infants had significantly lower serum calcium and higher serum phosphorous, urea, and creatinine. Early polyuric episodes and high urinary output were also more common in hyperkalemic infants. NOHK affects mainly preterm infants. Electrolyte disturbance and increased serum urea and creatinine were associated with hyperkalemia. Infants with NOHK also had high incidence of cardiac arrhythmias and mortality.

Salbutamol versus cation-exchange resin (kayexalate) for the treatment of nonoliguric hyperkalemia in preterm infants.

Our objective was to compare the efficacy and safety of rectal cation-exchange resin (Kayexalate) versus salbutamol infusion for the treatment of nonoliguric hyperkalemia (NOHK) in preterm infants. Data of all neonates born with NOHK during the study period of 6 years and 8 months were recorded. Diagnostic criteria of NOHK included serum potassium (SK) concentration > or = 7 mmol/L during the first 72 hours of life with urine output > or = 1 mL/kg/hour. This before-after study was divided according to the date of admission; the first 15 patients were treated with Kayexalate enema 1 g/kg every 4 hours, and the remaining 30 patients were treated with intravenous salbutamol infusion as 4 mug/kg every 4 hours. Treatment discontinued when SK became < 6 mmol/L. SK was measured every 4 hours. Daily urine was collected. Fluid intake and output, serum electrolytes, urea, creatinine, and glucose concentrations were obtained in all infants every 12 hours. All infants were observed with a cardiorespiratory monitor and oxygen saturation and blood pressure measurements. Perinatal characteristics in both groups were comparable. Mean gestational age was 26 and 28 weeks for salbutamol and Kayexalate, respectively. The peak of SK ranged between 7 and 9.3 mmol/L in the Kayexalate group and between 7 and 8.7 mmol/L in the salbutamol group ( P = 0.64). At 12 hours of treatment, SK became normal in only 4 patients (26%) in the Kayexalate group compared with 18 patients (60%) in the salbutamol group ( P = 0.003). The number of doses of Kayexalate administration was significantly higher than the doses of salbutamol ( P = 0.003). No significant side effects were detected in the salbutamol-treated infants. In contrast, there were two cases of severe ventricular tachycardia and one case of intestinal obstruction in the cation-exchange resin group. We concluded that salbutamol infusion is more effective with faster action and safer than cation-exchange resin (Kayexalate) for the treatment of NOHK in preterm infants.

Does prophylactic phototherapy prevent hyperbilirubinemia in neonates with ABO incompatibility and positive Coombs' test?

OBJECTIVE: The objective of the study was to determine whether initiation of early phototherapy in positive direct Coombs' test (DCT) with ABO-incompatible newborns would prevent severe jaundice. STUDY DESIGN: A prospective controlled study was performed at Al Qassimi Hospital. Infants born at term and weighing >2000 g with ABO incompatibility and a positive DCT were included in the study. Within their first 4 hours of life and after parental consent, infants were enrolled into one of two groups: prophylactic phototherapy group, which received phototherapy during the first 24 hours of life (group I), or no prophylactic phototherapy, which represents the control group (group II). Selection of infants to either group was by 2-week alternative strategy. Blood group, complete blood count (CBC), reticulocyte count, blood smears, total serum bilirubin (TSB) and DCT were performed on cord blood of all neonates born to mothers with O-positive blood group. CBC, reticulocytes and TSB level were obtained in all enrolled infants at 12, 24, 48, 72, and 96 hours of life. RESULTS: During the study period, 242 newborns with positive DCT were enrolled. A total of 102 infants were allocated to the prophylactic phototherapy arm and 140 as controls. Prophylactic phototherapy was associated with a significant decrease in the TSB at 24 hours (p=0.002) and at 48 hours (p=0.003) but not later on. The total number of patients who had hyperbilirubinemia at any time during the first 96 hours was significantly less in the prophylactic group (17 vs 45--p=0.006). Prolonged hospital stay because of phototherapy was more frequent in the control group (p=0.03). CONCLUSION: Prophylactic phototherapy was associated with a significant reduction of TSB in the first 48 hours of life but not later on. Clinical benefits of this strategy could not be proven.

Clinical presentation of hypernatremic dehydration in exclusively breast-fed neonates.

OBJECTIVE: To identify the clinical presentation of dehydration related to failure of lactation in exclusively breast-fed term infants. METHOD: A prospective study was performed between January 2000 and June 2003 in Al Qassimi Hospital in the Emirate of Sharjah. Enrollment criteria included term neonates whose birth weight of > 2000 g with no underlying organic illness causing poor feeding admitted for clinical manifestations of dehydration with weight loss of > 10% during the first 2 weeks of life. The control group, a non-randomized sample included healthy full term neonates, seen in Sharjah maternal and child health care center at 4-7 days old for their routine Guthrie screening test. For each dehydrated neonate we took two neonates as controls. Mother's age, parity, length of pregnancy, any pathologic conditions, breastfeeding history and her level of knowledge of lactation was recorded. Neonatal information included mode of delivery, percentage of weight loss, clinical examination, and stool and urine output the previous day. Data was analyzed with Student 't' test and chi-square test. RESULTS: Out of 17208 live births, 29 neonates between the ages of 2-13 days were admitted with weight loss of between 12 and 29% (dehydrated group). 27 patients had hypernatremic dehydration with serum sodium level ranging from 150 to 195 mmol/l. Mean age of admission was 4.9 days. Reasons for admission were: signs of dehydration (55%); hyperthermia (55%); hypoglycemia (27%) and jaundice (59%). The control group included 58 healthy neonates. Their birth weight and age were comparable to those in the dehydrated group. In comparison with the control group, delivery by cesarean section (P< 0.0001), lower level of maternal breastfeeding knowledge (P=0.03), transient inadequate breast milk quantity (P=0.005) and nipple anomalies (P=0.001) was significantly more common in the dehydrated group. Fewer voidings of urine (< 6 times /day) and stool (< 3 times/day) in the previous 24 hours before admission was more frequently observed in the dehydrated group (P < 0.0001). CONCLUSION: Low level of maternal knowledge in lactation, cesarean section and failure of early postnatal follow up was associated with the neonatal dehydration. Decreased urine and stool frequency might be considered as a warning for failure of lactation.

Mesenteric cystic lymphangioma.

Abdominal cystic lymphangioma is a very rare congenital tumor of lymphatic origin. It usually appears in the pediatric age and frequently presents with non-specific symptoms and deceptive signs causing, at times, diagnostic dilemmas. Ultrasonography and computer tomography imaging are considered the diagnostic modalities of choice. Two cases of mesenteric cystic lymphangioma, one presenting as perforated appendicitis and the other as recurrent gastritis, are reported. Infection in the first and volvulus in the second case is behind the mode of presentation. The diagnostic approach and treatment are described, with emphasis on the operative tactic applied for upper jejunal resection. A high index of suspicion, accuracy and repeated physical examination and, most important, the liberal use of ultrasonography in all cases of unclear abdominal illness may contribute considerably to a correct diagnosis and decreased morbidity.

Epidemiology and outcome of "early-onset" vs "late-onset" necrotizing enterocolitis.

OBJECTIVE: We hypothesized that among neonates with necrotizing enterocolitis (NEC), important epidemiologic and outcome differences exist between those with early-onset vs. those with late-onset NEC. METHODS: We reviewed all records of neonates cared for in the King Fahad University Hospital during the past ten years who had the diagnosis of NEC. We separated cases into two groups depending on age at diagnosis. Specifically, we termed "early-onset" those cases diagnosed during the first seven days of life, and "late-onset" those diagnosed thereafter. We compared, in the two groups, gestational age, clinical signs at onset, laboratory data, surgical findings, complications, and mortality. RESULTS: From 1989 to 1999, 37 cases of stage IIa (or higher) NEC were diagnosed; 25 "early-onset" and 12 "late-onset". Neonates with "early-onset" NEC were more mature (35.4 +/- 2.5 weeks gestation) than those with "late-onset" (27.7 +/- 2.8 weeks, P=0.0001), were more likely to have feedings begun in the first 48 hours of life (P = 0.0002), and more likely to have feeding increments of >25 ml/kg/day (P=0.03). Neonates with "late-onset" NEC were more likely to present with vomiting (P=0.003) and apnea (P=0.001), and were more likely to have ileal rather than colonic necrotic lesions, short bowel syndrome, and mortality (P=0.03). CONCLUSION: During the past 10 years at the King Fahad University Hospital, cases of early- and late-onset NEC have had distinct epidemiologic and outcome features. Recognizing these differences may be useful in prognostication and counseling.