I Jornada de expertos en ictiosis

Hernández‐Martín, Ángela; Torrelo-Fernández, A.; Lucas-Laguna, R. de; Casco, Fernando; González‐Sarmiento, Rogelio; Vega, Ana; Pedreira-Massa, José Luis; Unamuno-Pérez, P. de; Larcher, Fernando; Arroyo, I.; Traupe, Heiko

doi:10.1016/j.ad.2012.11.018

Cited by 4 publications

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“…as ictiosis son trastornos hereditarios de la queratinización caracterizadas por la presencia de hiperqueratosis y/o descamación [1]. La palabra ictiosis deriva del griego ichthys, que significa pez, debido al efecto escamaso de la piel afectada [2].…”

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Silvia Zepeda Olivera

Olivera¹,

Santamaría²,

García³

et al. 2021

Rev. chil. anest.

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Introduction: Ichthyosis are hereditary disorders of keratinization which are characterized for the presence of hyperkeratosis and/or peeling. This disorder group can put in danger the life of the patient because of the severe alteration of the skin barrier, associated with a severe transdermic loss of water, different grades of hypothermia and a hypernatremic dehydration. Objective: Introducing the clinic case of an infant patient with the diagnosis of ichthyosis plus severe dehydration who is scheduled for placement of CVC. Clinical case: Male infant, 3 months old, with the diagnosis of ichthyosis and severe dehydration, scheduled for placement of CVC. Conclusions: Due to the urgency of our patient, who presented severe dehydration, an inhaled general anesthesia with sevoflurane and oxygen was decided, without instrumenting the airway which in these patients has the risk of being potentially difficult, from the placement of the face mask itself, until finding lesions within the oral cavity, ventilatory assistance should be maintained by the pediatric anesthesiologist, once venous access is achieved, the fluids restitution is performed with loads of 10 to 20 ml/kg of the patient's weight, hyperthermia was controlled with physical means.

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“…Las ictiosis pueden poner en peligro la vida del niño en las primeras semanas o meses de vida, debido a la severa alteración de la barrera cutánea. Los problemas sistémicos más frecuentemente asociados incluyen: intensa pérdida transepidérmica de agua, diferentes grados de hipotermia y deshidratación hipernatrémica [1].…”

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