Characteristics and outcomes of mechanically ventilated elderly patients in the absence of an end-of-life care policy: a retrospective study from Bahrain.

Background: Globally, the percentage of elderly patients has been increasing, leading to a higher demand for healthcare resources and intensive care. Bahrain has a majority Muslim population and Islam governs most policies, including end-of-life care. All patients at our institute receive full resuscitative measures regardless of the prognosis, leading to a high number of mechanically ventilated patients.

Sengers syndrome: six novel AGK mutations in seven new families and review of the phenotypic and mutational spectrum of 29 patients.

Background: Sengers syndrome is an autosomal recessive condition characterized by congenital cataract, hypertrophic cardiomyopathy, skeletal myopathy and lactic acidosis. Mutations in the acylglycerol kinase (AGK) gene have been recently described as the cause of Sengers syndrome in nine families.

Methods: We investigated the clinical and molecular features of Sengers syndrome in seven new families; five families with the severe and two with the milder form.

Ocular and skin manifestations in systemic pseudohypoaldosteronism.

Pseudohypoaldosteronism type-1 is a rare disorder characterised by end-organ resistance to aldosterone resulting in salt-losing crisis with hyponatraemic dehydration, hyperkalaemia and metabolic acidosis. We report two siblings with pseudohypoaldosteronism type-1 who presented early in neonatal period with hyponatraemia, severe hyperkalaemia and metabolic acidosis. Both babies had miliaria like skin rash which flared up during episodes of hyperkalaemia and hyponatraemia.