Adult Presentation of Joubert Syndrome Presenting With Dysphagia: A Case Report.

Joubert syndrome (JS) is a rare autosomal recessive disease affecting the cilium, an intracellular organelle. It has a wide spectrum of presentations with the involvement of multiple genes. JS has multiple subtypes that are either pure JS or JS with other organ involvement such as the kidneys, liver, and others. However, all subtypes share the involvement of the cerebellar peduncles and the brainstem, which presents as "a molar tooth sign" on magnetic resonance imaging, hypotonia, and intellectual disability. It has a higher prevalence among children with few able to survive to adulthood. Unfortunately, survivors live with debilitating comorbidities. Here, we present the case of a 20-year-old patient who presented with a new onset of dysphagia that led to a diagnosis of JS.

Renovascular Compression by the Diaphragmatic Crus: A Case Report.

Renovascular hypertension (RVHT) is among the most prevalent causes of treatment-resistant hypertension. Mostly it is caused by renal artery stenosis (RAS). With atherosclerosis being the most common cause of RAS, RAS due to external compression by the diaphragmatic crus is rare. The treatment of rare causes requires individualization due to the differences between their etiologies. Herein, we present a case of an 18-year-old patient presenting with high blood pressure readings. On follow-up, he was diagnosed with hypertension. On further evaluation, the right diaphragmatic crus compressed the right renal artery. This case emphasizes medical management in patients with hypertension secondary to diaphragmatic crus compression, and radiological findings in such cases.

Technoeconomic data adopted for the development of a long-term electricity supply model for the Hashmite Kingdome of Jordan.

Electrical generation in Jordan currently relies on imported fossil fuels. In the past, most imported fossil fuels were subsidised by neighbouring countries through grants and aid. This has led to a regulated market, with subsidised low-cost electrical energy consumers, and the government being the sole buyer and seller of electricity. With the ageing of the national electrical infrastructure, political instability in the region, and lack of funds for direct investment, other options needed to be pursued. Long term Power Purchase Agreements (PPA) were granted to Independent Power Producers (IPP) to encourage investment in capacity and infrastructure. In addition, long-term fuel contracts were signed to secure steady flow of primary fuel sources. Over the past few years, renewable energy penetration has increased rapidly, but without proper planning or taking into consideration long term PPA and fuel contracts. Data in regard to the current infrastructure, renewable energy technology, signed energy commitments and system operation assumptions are described in this article, which may be used for modelling and analysis. The Data were collected from annual reports from the different energy related entities in Jordan.