Chediak-Higashi syndrome in the intensive care unit

Chediak-Higashi Syndrome is a rare autosomal recessive disease characterized by recurrent infections, giant cytoplasmic granules and oculocutaneous albinism. We describe the clinical and laboratory findings of a patient with Chediak-Higashi syndrome who was diagnosed and treated in the intensive care unit because of bleeding tendency after surgery.     

Acyclovir induced coma in the intensive care unit

A 73-year-old man with multiorgan failure requiring mechanical ventilation and haemodialysis developed herpes labialis infection during his stay in the ICU. This was treated with enteral acyclovir. He developed persistent neurologic impairment soon after acyclovir administration, which, over the course of seven days, progressed to coma, the aetiology of which was unclear. The computed tomograph (CT) of the brain and the cerebrospinal fluid (CSF) examination was normal. The electroencephalogram (EEG) showed generalized slowing. The possibility of acyclovir neurotoxicity was considered and the drug was discontinued. Haemodialysis was instituted and the patient made a complete neurological recovery. We believe that this is the first reported case of coma due to enteral acyclovir.     

A neonate with long QT syndrome, refractory ventricular arrhythmias, and lidocaine toxicity

Long QT syndrome is characterized by electrocardiographic appearance of long QT intervals and propensity to polymorphic ventricular tachycardia. Aggressive anticipatory clinical management is required for a good outcome, especially in the symptomatic neonate. We present a neonate with a compound mutation with refractory ventricular tachycardia that necessitated multimodal pharmacotherapy with lidocaine, esmolol, and amiodarone along with ventricular pacing. Despite normal serum lidocaine levels, complex pharmacokinetic interactions resulted in presumed neurotoxicity due to lidocaine. This report discusses the implications and challenges of management of a neonate with compound long mutations.     

End-of-life care in the intensive care unit

The transition from active, invasive interventions to comfort care for critical care patients is often fraught with misunderstandings, conflict and moral distress. The most common issues that arise are ethical dilemmas around the equivalence of withholding and withdrawing life-sustaining treatment; the doctrine of double effect; the balance between paternalism and shared decision-making; legal challenges around best-interest decisions for patients that lack capacity; conflict resolution; and practical issues during the limitation of treatment. The aim of this article is to address commonly posed questions on these aspects of end-of-life care in the intensive care unit, using best available evidence, and provide practical guidance to critical care clinicians in the UK. With the help of case vignettes, we clarify the disassociation of withdrawing and/or withholding treatment from euthanasia; offer practical suggestions for the use of sedation and analgesia around the end of life, dissipating concerns about hastening death; and advocate for the inclusion of family in decision-making, when the patient does not have capacity. We propose a step-escalation approach in cases of family conflict and advocate for incorporation of communication skills during medical and nursing training.     

End-of-life care in the intensive care unit

End-of-life care in the intensive care unit (ICU) is an oxymoron. Intensive care units appeared in the 1980s only admitting patients for ‘intensive care’. Nowadays the ICU has become one of the few places in the hospital that can provide comfort care to the dying patient. For many doctors on ICU it remains a difficult and problematic area. Yet it is conceptually simple. The difficulty for the doctor is making the decision, for the patient and family, coming to terms with it. This article will focus on how this decision should be made and then on the care that should be provided for the patient. Many of the considerations in decision making are in the General Medical Council guidelines, Treatment and Care Towards the End of Life and this is essential reading before embarking of the process.     

Posthypoxic myoclonus (the Lance-Adams syndrome) in the intensive care unit

The neurological assessment of patients admitted to the intensive care unit after successful resuscitation from cardiopulmonary arrest may be difficult. We describe the cases of two patients who developed myoclonus within 24 hours of hypoxic respiratory and cardiac arrest. Initially, the clonic movements were thought to be generalised convulsions and were treated as such, until it became evident that the patients were aware and distressed. Posthypoxic myoclonus is a rare complication of successful cardiopulmonary resusitation. Recognition depends on the awareness that the syndrome exists, and is important so that correct therapy can be instituted. There may be important prognostic implications. Both our patients had normal intellectual recovery with moderate residual neurological disability from their movement disorder.     

Sepsis in the intensive care unit

Sepsis remains a major cause of mortality in intensive care. The past 10 years has seen a more uniform, worldwide approach to the management of sepsis, severe sepsis and septic shock. This has resulted in improved survival. It is important to recognize the early symptoms and signs of sepsis; the confused, hypoxic, hypotensive patient with pyrexia, tachycardia, tachypnoea and leucocytosis. Examination must include finding a source for infection and early drainage or debridement. Next take appropriate cultures, and give fluids and broad-spectrum antibiotics. If the picture does not improve over the next 6 hours step-up the treatment to include urine output, blood gases for base excess, lactate, haemoglobin, and glucose. These will guide the management of vasopressors, insulin, fluids, transfusion and bicarbonate. If the hypotension persists (septic shock) the patient should be moved to intensive care. Steroids should be added and additional inotropes. This should be instituted with 24 hours of the start of sepsis. Further advanced care may include mechanical ventilation which requires special consideration. Prevention by screening, stopping cross-infection and appropriate use of antibiotics remains the first priority.     

Sedation in the intensive care unit

Analgesics and sedatives are commonly prescribed in the ICU environment for patient comfort, however, recent studies have shown that these medications can themselves lead to adverse patient outcomes. Interventions that facilitate a total dose reduction in analgesic and sedative medications e.g. the use of nurse controlled protocol guided sedation, the combination of spontaneous awakening and breathing trials, and the use of short acting medications, are associated with improved outcomes such as decreased time of mechanical ventilation and ICU length of stay. This purpose of this review is to provide an overview of the pharmacology of commonly prescribed analgesics and sedatives, and to discuss the evidence regarding best prescribing practices of these medications, to facilitate early liberation from mechanical ventilation and to promote animation in critically ill patients.     

Gastroenterology in the intensive care unit

Hospitalized patients often have poor nutrition, and the metabolic demands of critical illness may exacerbate this. Gastrointestinal (GI) tract dysfunction may be as a result of surgery or contributed to by critical illness itself. This article describes the evidence behind feeding strategies, stress ulceration and the management of upper GI bleeding, selective gut decontamination.     

Education in the intensive care unit

Intensive care medicine is a newly formed specialty. Intensive care is characterized by a multidisciplinary activity focused on patients whose vital organs are compromised or who are at risk of multiorgan failure. Education in the intensive care unit is a complex activity where the educational and pedagogical process interacts with research, continuous improvement, professionalism, and bioethics. This model provides leadership and excellence in care with high standards of quality, security, solidarity and humanism.