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e3 102 Both L, Collins S, de Zoysa A, White J, Mandal S, Efstratiou A Molecular and epidemiologic review of toxigenic diphtheria infections in England between 2007 and 2013 J Clin Microbiol 2015;53:567-572 103 Camello TCF, Pereira GA, Hirata Jr R, et al Diphtheria outbreak in Maranhao, Brazil: microbiological, clinical and epidemiological aspects Epidemiol Infect 2015;143:791-798 104 Hadfield TL, McEvoy P, Polotsky Y, et al The pathology of diphtheria J Infect Dis 2000;181(suppl 1):S10-S22 105 Overturf GD Corynebacterium diphtheria In: Long SS, Pickering LK, Prober CG, eds Principles and Practice of Pediatric Infectious Diseases New York: Churchhill Livingstone; 2003 106 Rosenberg RN, Prusiner SB, Dimauro S, Barchi RL The Molecular and Genetic Basis of Neurological Disease 2nd ed Boston: Butterworth-Heinemann; 1997 107 Elder GH, Hift RJ Treatment of acute porphyria Hosp Med 2001;62:422-425 108 Kuo HC, Huang CC, Chu CC, et al Neurological complications of acute intermittent porphyria Eur Neurol 2011;66: 247-252 109 Albers JW, FInk JK Porphyric neuropathy Muscle Nerve 2004;30:410-422 110 Sardh E, Harper P, Balwani M, et al Phase trial of an RNA interference therapy for acute intermittent porphyria N Engl J Med 2019;380:549-558 111 Brzustowicz L, Lehner T, Castilla L, et al Genetic mapping of chronic childhood-onset spinal muscular atrophy to chromosome 5q11.2-13.3 Nature 1990;344:540-541 112 Oskoui M, Levy G, Garland CJ, et al The changing natural history of spinal muscular atrophy type Neurology 2007;69:1931-1936 113 Talbot K Spinal muscular atrophy J Inherit Metab Dis 1999;22:545-554 114 Pearn JH The gene frequency of acute Werdnig-Hoffmann disease (SMA type 1) A total population survey in North-East England J Med Genet 1973;10:260-265 115 Iannaccone S, Browne R, Samaha F, et al Prospective study of spinal muscular atrophy before age years Pediatr Neurol 1993;9:187-193 116 Iannaccone S, Burghes A Spinal muscular atrophies Adv Neurol 2002;88:83-98 117 Thomas N, Dubowitz V The natural history of type I (severe) spinal muscular atrophy Neuromuscul Disord 1994;4:497-502 118 Gozal D Pulmonary manifestations of neuromuscular disease with special reference to Duchenne muscular dystrophy and spinal muscular atrophy Pediatr Pulmonol 2000;29:141-150 119 Zerres K, Rudnik-Schoneborn S, Forrest E, et al A collaborative study on the natural history of childhood and juvenile onset proximal spinal muscular atrophy (type II and III SMA): 569 patients J Neurol Sci 1997;146:67-72 120 Russman B, Buncher C, White M, et al Function changes in spinal muscular atrophy II and III Neurology 1996;47:973-976 121 McWilliam R, Gardner-Medwin D, Doyle D, et al Diaphragmatic paralysis due to spinal muscular atrophy Arch Dis Child 1985;60:145-149 122 Bosboom WM, Vrancken AF, van den Berg LH, et al Drug treatment for spinal muscular atrophy type I Cochrane Database Syst Rev 2009;1:CD006281 123 FDA New Release FDA Approves First Drug for Spinal Muscular Atrophy 2016 Available at: https://www.fda.gov/NewsEvents/ Newsroom/PressAnnouncements/ucm534611.htm 124 Finkel R, Mercuri E, Darras B, et al Nusinersin versus sham control in infantile-onset spinal muscular atrophy N Engl J Med 2017;337:1723-1732 125 Mercuri E, Darras B, Chiriboga C, et al Nusinersin versus sham control in later-onset spinal muscular atrophy N Engl J Med 2018;378:625-635 126 Mendell J, Al-Zaidy S, Shell R, et al Single-dose gene-replacement therapy for spinal muscular atrophy N Engl J Med 2017;377: 1713-1722 127 Feigin RD, Cherry JD Textbook of Pediatric Infectious Diseases Vol 2, 4th ed Philadelphia: WB Saunders; 1998 128 Thorsteinsson G Management of postpolio syndrome Mayo Clin Proc 1997;72:627-638 129 Gordon SM, Isada CM West Nile fever: lessons from the 2002 season Cleve Clin J Med 2003;70:449-454 130 Messacar K, Schreiner TL, Maloney JA, et al A cluster of acute flaccid paralysis and cranial nerve dysfunction temporarily associated with an outbreak of enterovirus D68 in children in Colorado, USA Lancet 2015;385:1662-1671 131 Ayscue P, Van Haren K, Sheriff H, et al Acute flaccid paralysis with anterior myelitis — California, June 2012 - June 2014 MMWR Morb Mortal Wkly Rep 2014;63:903-906 132 Zwiener R, Ginsburg C Organophosphate and carbamate poisoning in infants and children Pediatrics 1988;81:121-126 133 Morgan D Recognition and Management of Pesticide Poisonings Washington, DC: U.S Environmental Protection Agency Office of Pesticide Program; 1976 134 Mortensen M Management of acute childhood poisonings caused by selected insecticides and herbicides Pediatr Clin North Am 1986;33:421-445 135 Lifshitz M, Shahak E, Sofer S Carbamate and organophosphate poisoning in young children Pediatr Emerg Care 1999;15:102-103 136 Anand S, Singh S, Saikia UN, et al Cardiac abnormalities in acute organophosphate poisoning Clin Toxicol 2009;47:230-235 137 Lifshitz M, Rotenberg M, Sofer S, et al Carbamate poisoning and oxime treatment in children: a clinical and laboratory study Pediatrics 1994;93:652-655 138 Goswamy R, Chaudhuri A, Mahashur A Study of respiratory failure in organophosphate and carbamate poisoning Heart Lung 1994;23:466-472 139 Pawar KS, Bhoite RR, Pillay CP, et al Continuous pralidoxime infusion versus repeated bolus injection to treat organophosphorus pesticide poisoning: a randomised controlled trial Lancet 2006; 368:2136-2141 140 Peter JV, Moran JL, Graham P Oxime therapy and outcomes in human organophosphate poisoning: an evaluation using meta-analytic techniques Crit Care Med 2006;34:502-510 141 Tafuri J, Roberts J Organophosphate poisoning Ann Emerg Med 1987;16:193-202 142 Moses V, Mahendri NV, John G, et al Early hypocaloric enteral nutritional supplementation in acute organophosphate poisoning—a prospective randomized trial Clin Toxicol 2009;47:419-424 e4 Abstract: This chapter is devoted to the most common acute neuromuscular disorders encountered in the pediatric intensive care unit An organized approach to neuromuscular disorders requires recognition that neuromuscular diseases encompass the entire motor unit The motor unit consists of the anterior horn cell, which begins in the spinal cord and terminates in a motor nerve; the myelin associated with the nerve; the neuromuscular junction; and the muscle that the nerve innervates Any disruption of this pathway may produce weakness Neuropathies and myopathies may be distinguished by sensory abnormalities and the distribution of the weakness Neuromuscular junction defects often show variable weakness Etiologic possibilities are narrowed by considering the clinical presentation, family history, recent illness, travel, inciting factors, and the clinical course Key Words: Neuromuscular disorder, anterior horn cell, myelin, neuropathy, neuromuscular junction, myopathy, weakness 69 Acute Rehabilitation and Early Mobility in the Pediatric Intensive Care Unit HALLIE LENKER, YUN KIM, BETH WIECZOREK, AND SAPNA R KUDCHADKAR PEARLS • Increased survival from pediatric critical illness has been accompanied by an increase in both short- and long-term morbidities for children undergoing active neurocognitive development • The harms of bed rest and immobility far exceed the potential risks of early mobilization in many, if not most, critically ill patients • Early mobilization comprises a spectrum of activities that are tailored to each child’s individual illness acuity and premorbid baseline function • Creating a culture of mobility in the pediatric intensive care unit (PICU) requires a multicomponent approach to minimal but effective sedation, delirium prevention and management, optimizing sleep hygiene, and family engagement • The first step in optimizing acute rehabilitation in the PICU— including neurocognitive development, progression of the child’s physical function, and prevention of loss of skills—is engaging Increased survival from pediatric critical illness has been accompanied by an increase in both short- and long-term morbidities for children undergoing active neurocognitive development.1–3 A culture of immobility has been propagated by the practice of deep sedation for mechanically ventilated children due to a perception of increased safety and comfort.4 These practices have magnified the harms of prolonged pediatric intensive care unit (PICU) stay, including pressure ulcers, venous thromboembolism, and post– intensive care syndrome.5 Across the United States, 20% of all children admitted to the PICU longer than 72 hours are completely immobile; the youngest children and those with normal baseline function are less likely to receive acute rehabilitation.6 In the adult intensive care unit (ICU), early mobilization and acute rehabilitation have been shown to improve outcomes for survivors of critical illness, including improved muscle strength and decreased duration of mechanical ventilation.7–9 Children, with a longer potential lifespan after critical illness, have even more to gain from a comprehensive approach to acute rehabilitation An emerging body of evidence in pediatric critical care has unequivocally demonstrated that acute rehabilitation for children can be safe and feasible,10–14 with outcomes research evolving.15,16 These data underscore the principle that success in acute • • • • the rehabilitation team as early as possible in the admission regardless of patient acuity, ideally within the first 72 hours Key disciplines in the acute rehabilitation team include the physical therapist, occupational therapist, speech language pathologist, and physiatrist Education for the medical team, including bedside providers and family, is key so that patient-specific mobility goals can be performed throughout the day based on the rehabilitation team’s recommendations Positioning and mobilizing the critically ill patient is considered to be one of the most important ways of reducing morbidity from ventilator-associated pneumonia Augmentative and assistive communication includes any strategies, tools, and technologies that replace speech or writing, and is an important consideration to optimize a child’s ability to communicate and express emotions, wants, and needs rehabilitation requires a multicomponent and interdisciplinary approach with a focus on minimal but effective sedation, adequate analgesia, delirium prevention, and family engagement For example, a child who is delirious or agitated is not safe to mobilize, and a child who has not slept well during the night or who is in significant pain will not be able to optimally engage in rehabilitation during the day Furthermore, the foundation of successful early mobility programs in the PICU is engagement of the rehabilitation team, including physical and occupational therapy and speech language pathology regardless of patient acuity This chapter reviews foundational aspects of rehabilitation in the PICU setting and strategies for implementation of multicomponent rehabilitation programs for critically ill children Rehabilitation Team Members in the Pediatric Intensive Care Unit Rehabilitative therapists are key components of the interprofessional team in the PICU Their focus spans far beyond mobilization, centering on engagement in age-appropriate activities across the continuum of care in relation to the child’s medical 845 846 S E C T I O N V I Pediatric Critical Care: Neurologic PT Age-appropriate activities Endurance Normalization of sleep-wake cycle Gross motor Increased HOB OOB Positioning Pulmonary Ambulation Progressive mobilization includes the graded application of these activities OOB to chair Sit at edge of bed Turning Play Splinting • Fig 69.1 Activities of progressive mobilization. HOB, Head of bed; OOB, out of bed status These activities include optimal positioning in the bed or crib, providing developmental activities such as engagement with age-appropriate toys, getting out of the bed for meals, walking throughout the PICU, participating in school activities, or even riding a bike Early mobilization as a spectrum of activities has been demonstrated to be safe and feasible regardless of the acuity of PICU patients (Fig 69.1).12 Therefore, the rehabilitation team must work closely with the PICU medical team to determine the optimal activity level for each patient, each day Key rehabilitation team members in the acute care setting include the physical therapist (PT), occupational therapist (OT), and the speech-language pathologist (SLP) The rehab team may also include a physiatrist, who is a physician with a focus on medical interventions for a habilitative or rehabilitative approach during acute illness Habilitation is particularly important in the pediatric setting, defined as services to those who may not have ever developed a certain skill or functional ability In the PICU, the PT, OT, SLP, and physiatrist roles may overlap or collaborate in order to optimize a child’s current level of function and minimize the adverse effects of critical illness on development As in all settings, a therapist’s role is to analyze how the health condition is impacting the child’s physical function and ability to participate in functional or age-appropriate activities For example, play is a critically important for a child when considering the ICU physical and sensory environment and is integrated into the therapies of all rehabilitation team services Therefore, in this chapter, although one therapist may be discussed as having a role in a specific activity, the rehabilitative roles are often not mutually exclusive and may depend on the individual ICU and hospital culture (Fig 69.2) Role of Physical Therapy in the Pediatric Intensive Care Unit PTs are trained to diagnosis and treat individuals who have preexisting medical problems or newly acquired health-related conditions that limit their abilities to move and perform functional OT ADLs Swallowing Range of motion Sitting position SLP Phonation Language Cognition Feeding Communication Oral-sensorimotor Stimulation • Fig 69.2 Interrelationships of physical, occupational, and speech/ language therapies in the pediatric intensive care unit. ADLs, Activities of daily living; OOB, out of bed; OT, occupational therapist; PT, physical therapist; SLP, speech-language pathologist activities.17 PTs typically have a doctorate in physical therapy The role of the PT in the PICU is critical as parents report impaired functional abilities and acquisition of new morbidity months after discharge, which is associated with increased mortality following an ICU admission.1,18 PTs perform evaluations based on the child’s age, medical diagnosis, and medical status, and develop a plan using follow-up treatments to promote appropriate movement, reduce pain, and prevent loss of and promote the acquisition of developmentally appropriate function in order to prevent disability The PT’s role in the PICU may both overlap and differ from the PT’s approach during an inpatient floor stay versus in the community Analyzing the ICU environment and using mobility or developmental play to increase level of cognitive stimulation and physical activity for cardiac and pulmonary optimization is often the focus Education for healthcare providers and family is key so that patient-specific mobility goals can be performed throughout the day and the PT can facilitate progression of the patient’s function Optimal interdisciplinary communication is imperative Functional mobility interventions need to be carefully monitored, as a critically ill child can have minimal reserve Therefore, a thorough understanding of the patient’s medical status and changes in medication or pulmonary status should be discussed and taken into consideration daily for the mobility plan eTable 69.1 summarizes validated measures used by the PT to follow a child’s progress during the hospital stay Role of Occupational Therapy in the Pediatric Intensive Care Unit OTs focus on assisting people across their life span with the promotion of health and prevention of injury or loss of skills by adapting the environment or task to support the whole person.25 OTs typically receive masters or doctoral degrees and are registered OTs Due to the nature of an acute illness requiring PICU admission, the OT’s role is to enable children of all ages to progress toward or return to daily activities For infants, children, and adolescents, activities of daily living (ADLs) comprise engagement 846.e1 eTABLE Validated Tests for Measuring Physical Rehabilitation Outcomes in the Pediatric Intensive Care Unit 69.1 Test Age Norms System Measured Timed up and go (TUG)19 5–13 y Balance, gait mechanics 5–14 y Functional mobility, lower extremity strength 5–13 y Gait mechanics, endurance Timed floor to stand-normal (TFTS-N)20 Thirty-second walk test (30sWT) Six-minute walk test (6MWT) 22 21 3–18 y Endurance, pulmonary/cardiovascular WeeFIM II23 mo–12 y Gross motor function Gait speed24 1–10 y Gait mechanics ... junction; and the muscle that the nerve innervates Any disruption of this pathway may produce weakness Neuropathies and myopathies may be distinguished by sensory abnormalities and the distribution...e4 Abstract: This chapter is devoted to the most common acute neuromuscular disorders encountered in the pediatric... rehabilitation team as early as possible in the admission regardless of patient acuity, ideally within the first 72 hours Key disciplines in the acute rehabilitation team include the physical therapist,