An Unusual Presentation of Moyamoya Disease in a Pediatric Patient.

Moyamoya disease (MMD) is a chronic cerebro-vasculopathy that is extremely rare in the pediatric population. The main characteristic feature is the progressive stenosis in the internal carotid artery with or without the involvement of its main branches in the circle of Willis leading to ischemic stroke. Patients have clinical manifestations related to cerebral ischemia in the carotid branch territories, such as sensory impairment, hemiparesis, and aphasia/dysarthria. Herein, we report a case of MMD in a six-year-old Emirati female who presented with unusual manifestations of MMD in the form of headache, vomiting, and double vision and was diagnosed with MMD based on a brain MRI with angiography. To our knowledge, this is the first reported case of MMD in the United Arab Emirates.

Knowledge and Awareness Among Mothers Regarding Early Childhood Development: A Study From the United Arab Emirates.

Background and objective There is a dearth of studies on the knowledge and awareness among mothers on childhood development in the United Arab Emirates (UAE). Maternal knowledge of childhood development is a critical determinant of children's development and behavior. In light of this, we conducted this study to determine the level of maternal knowledge about childhood development. Methodology We employed a cross-sectional study design involving 200 mothers of all ages recruited using stratified random sampling. After obtaining informed consent, the participants were required to complete a questionnaire adapted from the "Ages and Stages" questionnaire, which covered demographics and items on developmental milestones. The questionnaire was validated and checked for reliability by using a focus group. Inferential statistics were used, and the association between the variables was analyzed using the Chi-squared test. Results Our findings indicated that the knowledge among mothers regarding child development is relatively low in the UAE. Two-thirds of the respondents were knowledgeable about gross motor skills (62% of the mothers knew the age at which a child could lift his/her head). Less than half of the mothers were well-informed about fine motor skills such as writing and drawing (44% of the mothers were aware of the age at which a child should be able to scribble on paper). The respondents demonstrated a lack of knowledge regarding children's speech and language skills. Regarding social skills, only 8% of the mothers were aware of the correct age at which a child should start to dress by himself/herself. Conclusion Although mothers in the UAE were knowledgeable about certain aspects of childhood development such as gross motor development, they were less knowledgeable regarding other aspects such as social and language skills. The gaps identified in our study highlight the need to implement effective health education programs so that mothers are better informed to help improve child development outcomes in the community.

Clinical spectrum, risk factors, and outcomes of children with laboratory-confirmed influenza infection managed in a single tertiary hospital: A 6-year retrospective cohort study.

BACKGROUND: Influenza is a highly contagious disease that causes severe illness each year. Data in the United Arab Emirates are scarce. OBJECTIVES: To study the seasonality, morbidity, mortality rate, and comorbidities associated with confirmed influenza infection in a tertiary hospital in Al-Ain city, UAE. METHODS: Retrospective study, from 2012 to 2017, of the electronic medical records in Tawam hospital, of children up to 15 years of age with laboratory-confirmed influenza infection. RESULTS: There were 1392 children, with the highest number in 2017 (n = 461, 33%). The incidence peaked between October and March. The infection was more common between 1 and 11 years of age (n = 948, 68%). The overall prevalence of influenza A (n = 1144, 82%) was higher than influenza B (n = 276, 19.8%). One-third of the patients required admission. The commonest underlying comorbidity was asthma (n = 170, 12%). The two commonest complications were pneumonia (n = 165, 12%) and acute otitis media (n = 82, 6%). CONCLUSION: Our findings serve as a benchmark for comparison with reports from other countries and need to be considered when reviewing the national vaccination program.

What is beyond Salmonella gastroenteritis? A case of acute pancreatitis complicating Salmonella infection in a child: a case report and literature review.

BACKGROUND: Salmonella infection presents itself in a wide variety of ways, ranging from mild self-limited illness to severe systemic disease with multiorgan involvement. Acute pancreatitis (AP) is a very rare complication that is associated with Salmonella infection, especially among the pediatric population. CASE PRESENTATION: A five-year-old boy presented with a two-day fever and experienced vomiting, diarrhea, and abdominal pain. The boy was admitted as a case of acute gastroenteritis, and Salmonella was found in his stool culture. The severity of his abdominal pain during his hospital stay indicated the possibility of AP. A clinical examination and blood workup were performed and showed significant elevation in amylase and lipase, which confirmed the diagnosis of AP. CONCLUSION: Although abdominal pain is a common presentation of Salmonella infection, the possibility of AP must be considered when the pain is severe and the characteristics of the pain are suggestive of AP. Herein, we report a case of AP complicating Salmonella infection in an immunocompetent child.

Epidemiological characteristics of children with coronavirus at a joint commission-accredited hospital in the United Arab Emirates.

OBJECTIVES: Our aim was to identify the epidemiological characteristics and transmission patterns of coronavirus (COVID-19) among pediatric patients in the multicultural and multiethnic city of Al Ain in the United Arab Emirates (UAE). METHOD: A retrospective study was conducted by abstracting data from the electronic medical records of pediatric patients with COVID-19 from two major public hospitals in Al Ain. The data of patients from birth through 16 years of the cases with COVID-19 confirmed by reverse transcription-polymerase chain reaction were obtained. In addition to the epidemiological characteristics, transmission patterns, comorbidities, index cases, travel history, and coinfection with other viruses were analyzed. Cohen's kappa was used to assess interrater reliability and descriptive data. Chi-square test was used to assess significant differences between the variables and was conducted using Statistical Product and Service Solutions software. RESULTS: We identified 298 (150 males, 148 females) laboratory-confirmed cases. The patients' median age was 7 years. Of these, 50% had parents who also tested positive. Most patients (86.9%) were healthy without any known medical problems. The coinfection rate was ~2%. CONCLUSION: The epidemiological characteristics of children with COVID-19 in Al Ain are similar to those observed internationally. Children of all ages appeared to be susceptible to COVID-19 and no significant sex or ethnicity differences were detected. Furthermore, this study provides strong evidence of human-to-human transmission.

Significance of platelet count in children admitted with bronchiolitis.

AIM: To determine the true prevalence of thrombocytosis in children less than 2 years of age with bronchiolitis, its association with risk factors, disease severity and thromboembolic complications. METHODS: A retrospective observational medical chart review of 305 infants aged two years or less hospitalized for bronchiolitis. Clinical outcomes included disease severity, duration of hospital stay, admission to pediatric intensive care unit, or death. They also included complications of thrombocytosis, including thromboembolic complications such as cerebrovascular accident, acute coronary syndrome, deep venous thrombosis, pulmonary embolus, mesenteric thrombosis and arterial thrombosis and also hemorrhagic complications such as bleeding (spontaneous hemorrhage in the skin, mucous membranes, gastrointestinal, respiratory, or genitourinary tracts). RESULTS: The median age was 4.7 mo and 179 were males (59%). Respiratory syncytial virus was isolated in 268 (84%), adenovirus in 23 (7%) and influenza virus A or B in 13 (4%). Thrombocytosis (platelet count > 500 × 109/L) occurred in 88 (29%; 95%CI: 24%-34%), more commonly in younger infants with the platelet count declining with age. There was no significant association with the duration of illness, temperature on admission, white blood cell count, serum C-reactive protein concentration, length of hospital stay or admission to the intensive care unit. No death, thrombotic or hemorrhagic events occurred. CONCLUSION: Thrombocytosis is common in children under two years of age admitted with bronchiolitis. It is not associated with disease severity or thromboembolic complications.

Hyponatraemia and neurological complications in children admitted with bronchiolitis.

BACKGROUND: Hyponatraemia occurs during bronchiolitis, sometimes with neurological manifestations. The prevalence of the latter differs widely and little is known about the time of occurrence and associated factors. This study was undertaken to investigate these complications. METHODS: This was a retrospective observational chart review of a cohort of 233 infants under 2 years of age admitted with bronchiolitis to a teaching hospital in the United Arab Emirates. RESULTS: Hyponatraemia (serum sodium <135 mmol/L) occurred in 105 infants (45%, 95% CI 38-51). Hyponatraemia was present on admission in 84 infants (80%) with 90% of cases occurring within 6 days of the onset of illness. It was mild (130-135) in 100 infants (95%) and severe (<130) in five (5%). It was not significantly associated with age, duration of illness before admission, viral aetiology, white cell count or serum C-reactive protein concentrations, or the volume of administered intravenous fluid or use of 0.18% sodium chloride (NaCl). Neurological manifestations occurred in a 29-day-old child with a serum sodium level of 123 mmol/L while receiving two-thirds intravenous maintenance fluids (0.18% NaCl). His developmental milestones remained normal on follow-up to the age of 5 years. CONCLUSION: Hyponatraemia is common in infants with bronchiolitis and occurs in the majority within 6 days of onset of symptoms. There was a significant association between the presence of fever (>38°C) on admission and the duration of hospitalisation.

Unusual Presentation of Gianotti-Crosti Syndrome due to Epstein-Barr Virus Infection.

Gianotti-Crosti syndrome (GCS) is viral exanthema of childhood. It typically presents with a symmetric erythematous papular and papulovesicular eruption. It has been classically associated with hepatitis B virus, as well as rarely with Epstein-Barr virus (EBV). We report a case of GCS related to EBV infection without the classical systemic symptoms in a five-year-old male patient.

Recessive mutations in EPG5 cause Vici syndrome, a multisystem disorder with defective autophagy.

Vici syndrome is a recessively inherited multisystem disorder characterized by callosal agenesis, cataracts, cardiomyopathy, combined immunodeficiency and hypopigmentation. To investigate the molecular basis of Vici syndrome, we carried out exome and Sanger sequence analysis in a cohort of 18 affected individuals. We identified recessive mutations in EPG5 (previously KIAA1632), indicating a causative role in Vici syndrome. EPG5 is the human homolog of the metazoan-specific autophagy gene epg-5, encoding a key autophagy regulator (ectopic P-granules autophagy protein 5) implicated in the formation of autolysosomes. Further studies showed a severe block in autophagosomal clearance in muscle and fibroblasts from individuals with mutant EPG5, resulting in the accumulation of autophagic cargo in autophagosomes. These findings position Vici syndrome as a paradigm of human multisystem disorders associated with defective autophagy and suggest a fundamental role of the autophagy pathway in the immune system and the anatomical and functional formation of organs such as the brain and heart.