RESUMO
Waardenburg syndrome (WS) is an autosomal dominant genetic disorder characterized by the absence of melanocytes, leading to distinctive pigmentary abnormalities and sensorineural hearing loss. This case report describes extremely rare concurrent anomalies in a preterm male infant diagnosed with WS type 1. The newborn, delivered prematurely at 35 weeks due to maternal complications, presented with multiple congenital anomalies and required immediate resuscitation. He exhibited hallmark features of WS, including a white forelock, dystopia canthorum, and bilateral sensorineural hearing loss. Genetic testing confirmed a PAX3 gene mutation. The infant experienced significant respiratory and feeding challenges, necessitating intensive care. Management included mechanical ventilation, surfactant therapy, phototherapy for hyperbilirubinemia, and broad-spectrum antibiotics for suspected sepsis. The cardiac assessment revealed multiple anomalies, such as a patent foramen ovale and left ventricular hypertrophy, while renal ultrasound identified multicystic dysplastic kidney and bilateral hydronephrosis. Multidisciplinary care facilitated the infant's stabilization, transition to oral feeding, and ongoing specialized care. WS type 1 is associated with mutations in the PAX3 gene and presents with diverse clinical manifestations. Although renal and cardiac anomalies are uncommon in WS, their presence in this case underscores the complexity of the syndrome. Early intervention for hearing impairment and genetic counseling are critical for optimal outcomes. This report highlights the importance of a comprehensive and interdisciplinary approach to managing infants with WS, addressing both typical and atypical manifestations. It is worth noting that effective management of WS in neonates requires prompt identification and treatment of associated complications.
RESUMO
AIM: This study aimed to assess the association between psychological reactions (e.g., stress, anxiety, and depression), resilience, and work engagement among Palestinian critical care nurses in the West Bank and examine the correlation of psychological reactions and resilience with work engagement. BACKGROUND: Work engagement is associated with psychological reactions and resilience, particularly among critical care nurses. There is a lack of studies on work engagement and these factors in Palestine. METHODS: A cross-sectional, descriptive correlational design was adopted. A convenience sample consisting of 273 critical care nurses from private and governmental hospitals was recruited to participate. Depression, Anxiety, Stress Scale-21 (DASS-21), Connor-Davidson Resilience Scale-25 (CDRS-25), Utrecht Work Engagement Scale-9 (UWES-9), and demographic data were used to collect data during the period from March 20 to May 20, 2023. RESULTS: Findings demonstrated that 53.9% of the nurses reported mild-to-moderate levels of depression, 49.8% reported moderate-to-severe levels of anxiety, and 49.1% reported moderate-to-severe levels of stress. Additionally, 57.5% and 52.7% of them had low resilience and work engagement, respectively. Moreover, work engagement negatively correlated with depression (r = -0.796, P < 0.01), anxiety (r = -0.654, P < 0.01), and stress (r = -0.796, P < 0.01), while positively correlated with resilience (r = 0.42, P < 0.01) and gender (r = 0.121, P < 0.05). Depression, anxiety, stress, resilience, and gender were the main predictors of work engagement. DISCUSSION: The majority of the nurses suffered from depression, anxiety, and stress. Additionally, more than half of the participants had low resilience and work engagement. Moreover, increased depression, anxiety, and stress were correlated with decreased work engagement, while high resilience and gender as being female positively correlated with high work engagement. CONCLUSION AND IMPLICATIONS FOR NURSING AND HEALTH POLICY: Policymakers and hospital administrators should develop interventions to improve critical care nurses' resilience and minimize psychological reactions, which have a significant influence on work engagement. Future studies should be conducted to examine the effectiveness of these interventions.
