Most children who present with hypertensive crisis have a secondary cause for hypertension. This study describes the epidemiology and management of children with hypertensive crisis. A retrospective cohort study was done in a tertiary pediatric hospital from 2009 to 2015. Thirty-seven patients were treated for hypertensive crisis. Twelve (32.4%) patients were treated for hypertensive emergency. The majority of our patients (33 [89.1%]) had a secondary cause of hypertension. The most common identifiable cause of hypertension was a renal pathology (18/37 [48.6%]). Oral nifedipine (23 [62.1%]) was the most frequently used antihypertensive, followed by intravenous labetalol (8 [21.6%]). There were no mortalities or morbidities. Hypertensive crisis in children is likely secondary in nature. Oral nifedipine and intravenous labetalol are both effective treatments.

Background: HIDEA (hypotonia, hypoventilation, intellectual disability, dysautonomia, epilepsy and eye abnormalities) syndrome is a rare and novel disease. We describe a premature patient who required extensive work up for his hypoventilation with a diagnosis of HIDEA syndrome.Case Description: The patient was born to a pair of consanguineous parents at 32-week gestation. His intermittent bradypnoea requiring significant respiratory support during his postnatal clinical course was atypical for bronchopulmonary dysplasia and this required further extensive work up to look for a cause for his hypoventilation. A trio whole exon sequencing was done which identified homozygous variants in P4HTM, in keeping with the diagnosis of autosomal recessive HIDEA syndrome. He is currently doing well on BiPAP 18 cm H2O / 8 cm H2O, Rate 30 breaths per minute in room air and full nasogastric feeding. He also has cortical blindess and severe global developmental delay. Conclusion:Early diagnosis is crucial to optimise adequate ventilatory management including early tracheostomy as many require lifelong continuous or intermittent ventilation. This minimises the complications of chronic hypoxia and reduces mortality risk.

Background: HIDEA syndrome is a rare and novel disease characterised by hypotonia, hypoventilation, intellectual disability, epilepsy and eye abnormalities. Diagnosis is made by genetic testing with exclusion of other causes of hypoventilation. We present a case of a neonate born premature to a pair of consanguineous parents with an atypical course of bronchopulmonary dysplasia subsequently diagnosed with HIDEA syndrome. Conclusion: This is the first case report of HIDEA syndrome in South East Asia, broadening our understanding of the full phenotypic pattern of HIDEA syndrome. Patients with HIDEA syndrome are at risk of prematurity and hypothyroidism. Early diagnosis is crucial to optimise adequate ventilatory management including early tracheostomy as many require lifelong continuous or intermittent ventilation. This minimises the complications of chronic hypoxia and reduces mortality risk. HIDEA syndrome is an important differential diagnosis in the consideration of an infant who presents with hypoventilation.