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Pediatric emergency medicine trisk 604

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Possible Unlikely Serologic criterion one or two with ≤1 minor criterion Maternal history of adequate treatment during pregnancy (penicillin-based regimen) + nonreactive serologic test for syphilis XR, radiographs; CNS, central nervous system; CSF, cerebrospinal fluid; RPR, rapid plasma reagin; VDRL, venereal disease research laboratory; FTA-ABS, fluorescent treponemal antibody absorption; TP-PA, Treponema pallidum particle agglutination; MHA-TP, microhemagglutination test for antibodies to Treponema pallidum Modified from Mascola L, Pelosi R, Blount JH, et al Congenital syphilis revisited Am J Dis Child 1985;139:575–580 e-TABLE 94.32 TREATMENT OF SYPHILIS Stage Primary, secondary, or early latent (infection within the last 12 mo) Treatment Penicillin G benzathine 50,000 units/kg (maximum: 2.4 million units) IM once Late latent (>1 yr since acquisition), Penicillin G benzathine 50,000 latent syphilis of unknown duration, units/kg (maximum: 2.4 million or tertiary syphilis units) IM weekly for wks Congenital syphilis or neurosyphilis Aqueous penicillin G 50,000 units/kg/dose IV every 12 hrs in 0–7 do, then every hrs in infants > days (maximum daily dose: 24 million units/day) for 10 days Alternative regimen for adults: Procaine penicillin G 24 million units IM daily with probenecid 500 mg four times daily for 10–14 days IM, intramuscular; IV, intravenous e-TABLE 94.33 STAGES OF SYPHILIS Stage Symptoms Time frame Primary Chancre: nontender, firm red lesion, often with associated nontender regional adenopathy As often found in the cervix or vagina, may not be noted by many women Due to disseminated spirochetemia Fever, influenza-like illness, generalized adenopathy, and dermal finding predominate in adults but are less prominent in young children These include condylomata lata and lesions on palms and soles (contact precautions should be used) Reactive serologic tests without symptoms 10–90 days after exposure Secondary Latent 2–10 wks after primary disease Early latent: 1 yr Tertiary Gummas: indolent localized dermal 3–10 yrs after secondary nodules caused by the host disease inflammatory reaction; in contrast to secondary syphilis, few spirochetes are found Neurosyphilis Paralytic dementia, meningitis, ≥5 yrs after primary seizures, gummas of the spinal disease cord, optic atrophy, or tabes dorsalis (myelopathy of the posterior columns of the spinal cord, resulting in loss of proprioception and vibration sensation) Cardiovascular Arteritis of the aorta and pulmonary 10–40 yrs after primary vessels that can result in valvular disease regurgitation or myocardial insufficiency CHAPTER 95 ■ METABOLIC EMERGENCIES BRET L BOSTWICK, JUSTIN R DAVIS GOALS OF EMERGENCY THERAPY Recognition and understanding of inborn errors of metabolism (IEMs) in the acutely ill child in the emergency department (ED) is critical for appropriate, and possibly lifesaving management Individually, metabolic diseases are rare, but collectively are common with the incidence approaching in every 800 to 2,500 newborns Goals of emergency care are to consider the possibility of IEM in the differential diagnosis of acutely ill, previously undiagnosed patients, and to identify, treat, and prevent acute metabolic derangements in patients with suspected or known IEMs, including the asymptomatic neonate with a positive newborn screening (NBS) KEY POINTS IEMs usually manifest in the neonatal period or infancy but can present at any age, even during adulthood Newborn screening (NBS) results may not be available in the first days to weeks of life: false negatives and false positives occur ED care does not require an extensive knowledge of individual metabolic diseases or biochemical pathways, but rather an understanding of the pathophysiology of categories of IEMs High index of suspicion is the most important element in making the diagnosis of metabolic disease Initial laboratory evaluation should include CBC, blood gas, glucose, ammonia, chemistries, uric acid, liver function studies, and urinalysis Successful emergency treatment of suspected and known IEMs depends on prompt institution of therapy to correct and prevent further metabolic derangement and is critical to prevent acute and long-term morbidity and mortality Specialists with expertise in inborn errors of metabolism should be consulted to guide diagnosis and management, and consideration should be given to consultation even if this requires referral outside of your facility UNKNOWN SUSPECTED IEM Goals of Treatment Recognizing the possibility of an IEM is critical for optimal management of the child with unknown IEM Immediate goals of treatment are to stabilize cardiopulmonary function, correct metabolic derangements, and avoid intake and/or endogenous production of potentially toxic substances Early consultation with an IEM specialist, including prior to transport of a child being transported from an outside facility, is advised to guide treatment and collection of appropriate specimens for diagnosis CLINICAL PEARLS AND PITFALLS ... L BOSTWICK, JUSTIN R DAVIS GOALS OF EMERGENCY THERAPY Recognition and understanding of inborn errors of metabolism (IEMs) in the acutely ill child in the emergency department (ED) is critical... collectively are common with the incidence approaching in every 800 to 2,500 newborns Goals of emergency care are to consider the possibility of IEM in the differential diagnosis of acutely ill,... gas, glucose, ammonia, chemistries, uric acid, liver function studies, and urinalysis Successful emergency treatment of suspected and known IEMs depends on prompt institution of therapy to correct

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