Surgical treatment of cardiac achalasia in children is still the main line of treatment with a success rate of 70-80%. Balloon dilatation is less widely used due to inappropriate size of balloons. The authors report on their experience in 11 children with cardiac achalasia over the last 7 years using balloon dilatation as the treatment of choice, 8 boys and 3 girls with ages ranging from 1.5-14 years (average 7.5 years) were investigated. One family (brother and sister) presented with no glucocorticoid deficiency or other anomalies, one patient had mental retardation, the rest had no associated anomalies. All patients presented with vomiting, 7 with dysphagia, 6 with loss of weight, 5 with recurrent chest infection and 2 with retrosternal pain. Radiological diagnosis was accurate in all patients, endoscopy with biopsy were done to confirm diagnosis and exclude other pathology, manometry yielded positive results in 4 patients. Dilatation was done under general anesthesia with fluoroscopic control, balloons were used over a guide wire (balloon sizes were 18-35 mm). Seven patients had 2 sessions and 4 had 3 sessions with radiological follow-up after the second dilatation. Follow-up ranged from 2-7 years: excellent results were achieved in 8 patients (72.7%) with disappearance of symptoms and marked radiologic improvement, 2 still have mild symptoms with overall success (90.9%), one had mild gastroesophageal reflux, controlled medically, and one had mild dysphagia but his status was improved compared to that before dilatation. One patient had recurrent dysphagia necessitating cardiomyotomy (9.1%). Results were not related to age or sex. The authors recommend balloon dilatation in children with cardiac achalasia as the treatment of choice or even as the only feasible treatment.

There have been limited cases linking SARS-CoV-2 infection with the development of reversible cerebral vasoconstriction syndrome (RCVS). We hereby report a rare case of RCVS in the setting of mild SARS-CoV-2 respiratory infection successfully treated with nimodipine and aspirin. SARS-CoV-2 attacks the ACE2-receptors, which are expressed in various body organs including the lungs, kidneys, and blood vessels. Vasoconstriction can result from down-regulation of the ACE2-receptors that can lead to sympathetic hypertonia of the cerebral blood vessel walls and/or over-activation of the renin-angiotensin axis.

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Mucopolysaccharidosis (MPS) is a group of rare disorders that are characterized by intracellular accumulation of glycosaminoglycans with subsequent cellular and organ dysfunction. In the Middle East, especially Saudi Arabia, higher prevalence of MPS type I was observed compared to reported rates from European countries and the United States (U.S). The present work was developed as a part of the Saudi MPS Group’s efforts to address the current situation of MPS type I in Saudi Arabia and to reach a national consensus in the management of MPS type I. The first “Management of MPS Type I Advisory Board” meeting was held in Riyadh on May 2, 2019, to reflect the expert opinions regarding different aspects of MPS type I and develop this manuscript; eight consultants from different specialties (medical genetics, pediatric rheumatology, and pediatric endocrinology), representing six Saudi institutions, in addition to a global expert in genetics participated in the meeting.

Background: Screening approved medications to identify therapeutics for drug repurposing is an effective tactic, and a deep research into off label drug use (OLDU) is required. Unfortunately, OLDU has not been extensively studied in Middle East. Our study aimed to evaluate the extent of OLDU in Saudi Arabia.Methods: Retrospective study carried out during 12 month period at six tertiary hospitals in Saudi Arabia. Each prescription was evaluated as unlicensed or OLDU based on the product information or based on Food and drug Administration (FDA) approval.Results: A total of 288 prescriptions were analyzed, where the reasons for off-label prescribing were OLDU by indication (94.42%), OLDU by different age group (2.09%), and other reasons represented (3.48%). Adults/geriatrics (≥18 years) received (89.05%) of the orders, and children (1-11 years) received (7.78%) of the orders. Both adolescents (12-18 years) and neonates (1-29 days) received (1.42%) of the orders per each category, while infants (1 month-1 year) received (0.36%) of the orders. The therapeutic classes most often prescribed were antidepressants (21.88%), antidiabetics (17.71%), and atypical antipsychotics (10.06%).Conclusions: Off-label prescribing was found in most adults/geriatrics suffering from depression, and diabetes mellitus. The most common reason for off-label prescription was off-label by indication. The results call for the need to conduct more studies with larger sample size, do more investigations on the OLDU in the whole kingdom, and develop policy for OLDU across hospitals.