Asunto(s)
Neurodegeneración Asociada a Pantotenato Quinasa/diagnóstico , Neurodegeneración Asociada a Pantotenato Quinasa/tratamiento farmacológico , Guías de Práctica Clínica como Asunto , Adolescente , Adulto , Niño , Humanos , Quelantes del Hierro/uso terapéutico , Neurodegeneración Asociada a Pantotenato Quinasa/genética , Ácido Pantoténico/uso terapéutico , Fosfotransferasas (Aceptor de Grupo Alcohol)/genéticaRESUMEN
Rhabdomyolysis is diagnosed with creatinine kinase (CK) elevation beyond 1000 U/L or ten times above the normal upper limit. Severe episodes can be fatal from electrolyte imbalance, acute renal failure, and disseminated intravascular coagulation. A 13-month-old child was admitted with a CK of 82,090 U/L in the setting of respiratory tract infection-related hyperthermia of 106.9° farenheit. His medical history was significant for prematurity, dystonia, and recurrent rhabdomyolysis. His home medications clonazepam, clonidine, and baclofen were continued upon admission. He exhibited uncontrolled dystonia despite treatment for dystonia. Therefore, sedative infusions and forced alkaline diuresis were begun to prevent heme pigment-induced renal injury. Despite these interventions, his CK peaked at 145,920 U/L, which is rarely reported in this age group. The patient also developed pulmonary edema despite diuresis and required mechanical ventilation. Sedative infusions were not enough for dystonia management, and he needed the addition of a neuromuscular blocking infusion. He finally responded to these interventions, and the CK normalized after a month. He required a month of mechanical ventilation and two and a half months of hospitalization and extensive rehabilitation. We were able to avert renal replacement therapy despite pulmonary edema and an estimated glomerular filtration rate nadir of 21 mL/min/1.73 m2 based on the bedside Schwartz formula. He made a complete recovery and was discharged home. His growth and development were satisfactory for two years after that event. His extensive diagnostic workup was negative. Unfortunately, he died from septic and cardiogenic shock with mild rhabdomyolysis two years later. Prompt recognition, early institution of appropriate therapies, identification of underlying disease, and triggering events are pivotal in rhabdomyolysis management. Evidence-based guidelines are needed in this context.
RESUMEN
Chronic pain is frequently experienced in adolescents; it affects functionality and requires interventions to decrease the impairments caused by pain. Cognitive behavioral therapy (CBT) has been analyzed in numerous studies that evaluated its effects on reducing the different types of chronic pain in children and adolescents. Interestingly, the outcome of CBT was initially focused on pain intensity, but, because there is no correspondence between children's pain intensity and level of disability, the ability to participate in school and social and recreational activities have been the primary focus of recent studies. There are innovative methods of CBT (such as the third generation of CBT) with and without the use of technology that facilitates the availability of this psychological treatment to adolescents with chronic pain, optimizing its accessibility and comprehensiveness, and maintaining its effectiveness. In the future, specific types of CBT could be specific to the diagnosis of chronic pain in the adolescent, sociodemographics, and other unique features. Parents of children with chronic pain are usually included in these programs, either as coaches in the intervention or as recipients of psychological therapies (including CBT) to optimize benefits. CBT has no adverse effect on chronic pain in adolescents, and there is no literature that makes reference to the effectiveness of CBT in preventing chronic pain in adolescents. A review of the role of CBT in chronic pain in adolescents via a PubMed database search was performed to identify the role of CBT in the management of chronic pain in adolescents.