Management of Harlequin Ichthyosis: A Brief Review of the Recent Literature.

Harlequin ichthyosis (HI) is a life-threatening genetic disorder that largely affects the skin of infants. HI is the most severe form of the autosomal recessive disorder known as ichthyosis. It is caused by mutations in the A12 cassette (lipid-transporter adenosine triphosphate-binding cassette A12). Neonates affected by this disease are born with specific morphological characteristics, the most prominent of which is the appearance of platelet keratotic scales separated by erythematous fissures. The facial features include eclabium, ectropion, a distinct flattened nose, and dysplastic ears. A common finding among those with HI is impaired skin barrier function. The purpose of the present narrative review is to assess the most recent literature regarding the management of HI. Emphasis is given to surgical management and consultation, to the indications for timing and surgical intervention, to the risks that are presented with surgery, and to the details of the surgical procedure itself. Management of HI requires a multidisciplinary team of experts, and specific guidelines are needed in order for the risks to be minimized and viability to be increased.

Renal Injuries after Cardiac Arrest: A Morphological Ultrastructural Study.

BACKGROUND: This study aims to investigate the probable lesions and injuries induced in the renal tissue after a cardiac arrest. The renal ischemia-reperfusion model in cardiac arrest describes the effects of ischemia in the kidneys, alongside a whole-body ischemia-reperfusion injury. This protocol excludes ischemic conditions caused by surgical vascular manipulation, venous injury or venous congestion. METHODS: For the experimental study, 24 swine were subjected to cardiac arrest. Seven minutes later, the cardiopulmonary resuscitation technique was performed for 5 min. Afterwards, advanced life support was provided. The resuscitated swine consisted one group and the non-resuscitated the other. Tissue samples were obtained from both groups for light and electron microscopy evaluation. RESULTS: Tissue lesions were observed in the tubules, parallel to destruction of the microvilli, reduction in the basal membrane invaginations, enlarged mitochondria, cellular vacuolization, cellular apoptosis and disorganization. In addition, fusion of the podocytes, destruction of the Bowman's capsule parietal epithelium and abnormal peripheral urinary space was observed. The damage appeared more extensive in the non-resuscitated swine group. CONCLUSIONS: Acute kidney injury is not the leading cause of death after cardiac arrest. However, evidence suggests that the kidney damage after a cardiac arrest should be highly considered in the prognosis of the patients' health outcome.

The adaptive immune response in cardiac arrest resuscitation induced ischemia reperfusion renal injury.

BACKGROUND: The present study aims to investigate, immunohistochemically, the role of the adaptive immune response in cardiac arrest/resuscitation-induced ischemia-reperfusion renal injury (IRI), namely to assess the presence of lymphocytes in renal tissue samples and the connection between the extent of the damage and the concentration of the lymphocytes by comparing the kidneys of non resuscitated swine with the kidneys of resuscitated swine. METHODS: Twenty four swine underwent cardiac arrest (CA) via a pacemaker wire. After 7 min, without any intervention, Cardiopulmonary Resuscitation, CPR, was commenced. Five min after CPR was commenced advanced life-support, ALS. Animals were divided into resuscitated animals and non resuscitated animals. Tissue samples obtained from the two groups for immunohistological study aiming to detect T-cells, B-cells and plasma cells using CD3 + , CD20 + , and CD138 + antibodies. RESULTS: There seems to be a strong concentration of T lymphocytes in the kidney tissues after ischemia of both non-resuscitated and resuscitated swine. B lymphocytes, also, appear to have infiltrated the ischemic kidneys of both animal groups; nevertheless, the contribution of T lymphocytes to the induction of injury remains greater. There is no strong evidence of correlation between the plasma cells and the damage. CONCLUSION: The adaptive immune response seems to have a strong association with kidney injury and acute tubular necrosis after cardiac arrest/ resuscitation-induced ischemia-reperfusion. However, the extent to which the adaptive immune cells are involved in the induction of renal injury remains uncertain and there are many questions about the mechanism of function of these cells, the answers of which require further studies.