Pediatric emergency medicine trisk 730
Bạn đang xem bản rút gọn của tài liệu. Xem và tải ngay bản đầy đủ của tài liệu tại đây (173.62 KB, 4 trang )
Copelovitch L, Warady BA, Furth SL. Insights from the chronic kidney
disease in children (CKiD) study. Clin J Am Soc Nephrol 2011;6:2047–
2053.
Hogg RJ, Furth S, LemLey KV, et al. National Kidney Foundation’s Kidney
Disease Outcomes Quality Initiative clinical practice guidelines for
chronic kidney disease in children and adolescents: evaluation,
classification, and stratification. Pediatrics 2003;111:1416–1421.
Schwartz GJ, Munoz A, Schneider MF, et al. New equations to estimate
GFR in children with CKD. J Am Soc Nephrol 2009;20:629–637.
Wuhl E, Schaefer F. Therapeutic strategies to slow chronic kidney disease
progression. Pediatr Nephrol 2008;23:705–716.
CHAPTER 101 ■ RHEUMATOLOGIC EMERGENCIES
THERESA M. BECKER, MELISSA HAZEN
GOALS OF EMERGENCY CARE
Pediatric rheumatologic conditions are rare and are typically chronic conditions with an
indolent onset rather than acute conditions likely to bring a child to the emergency
department (ED). Nonetheless, there are several reasons why children with
rheumatologic conditions may present to the ED. First, the majority of rheumatologic
conditions involve a myriad of signs and symptoms affecting many organ systems,
which may bring an exasperated family to the ED searching for an elusive diagnosis.
Second, arthritis, lupus, and vasculitis (especially Kawasaki disease [KD]) may have
acute and life-threatening complications that require rapid initiation of appropriate
therapy. Finally, the treatment of rheumatologic disorders is becoming more
sophisticated and more specialized, involving combinations of anti-inflammatory,
immunosuppressive, and biologic agents, with a wide spectrum of undesired effects.
Often a key challenge is differentiating the effects of underlying disease from the
effects of therapy. Thus, the goals of emergency care are the prompt recognition of
these conditions, and the expeditious use of medical therapy to treat the complications
of the diseases and the side effects of drug therapy.
KEY POINTS
Kawasaki disease requires treatment in the first 10 days of the illness in
order to achieve an optimal clinical outcome.
Many rheumatologic conditions are treated with medications that suppress
the immune system.
Stress doses of corticosteroids may be required for fever and other acute
illnesses.
Childhood vasculitis may affect any organ system and may present
indolently or acutely with life-threatening end-organ involvement.
Juvenile idiopathic arthritis subtypes are varied in their presentation and
associated with different articular and extra-articular complications.
Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation
syndrome (MAS) or reactive HLH should be considered in an ill child with
persistent fever, organomegaly, and neurologic symptoms with systemic
inflammation, cytopenias, and/or liver dysfunction.
RELATED CHAPTERS
Signs and Symptoms
Coma: Chapter 17
Edema: Chapter 25
Gastrointestinal Bleeding: Chapter 33
Hypertension: Chapter 37
Pain: Abdomen: Chapter 53
Seizures: Chapter 72
Medical, Surgical, and Trauma Emergencies
Cardiac Emergencies: Chapter 86
Gastrointestinal Emergencies: Chapter 91
Infectious Disease Emergencies: Chapter 94
Neurologic Emergencies: Chapter 97
Pulmonary Emergencies: Chapter 99
Renal and Electrolyte Emergencies: Chapter 100
Abdominal Emergencies: Chapter 116
Neurosurgical Emergencies: Chapter 122
Behavioral and Psychiatric Emergencies: Chapter 126
SYSTEMIC LUPUS ERYTHEMATOSUS
CLINICAL PEARLS AND PITFALLS
The most common initial symptoms are the gradual onset of fever, fatigue,
and generalized lymphadenopathy.
The clinical presentation is highly variable and may include multiple body
systems.
The classic malar rash is present in only one-half of pediatric patients at
presentation.
Infection is the major cause of mortality in childhood systemic lupus
erythematous (SLE) because of immune dysregulation inherent in the
disease and the immunosuppressant medications used to treat SLE.
Patients taking corticosteroids may require stress doses during acute febrile
illness.
Corticosteroids may mask the symptom of pain.
Current Evidence
SLE is a multisystem disease that is both pleomorphic in its presentation and variable
in its clinical course. In many ways, it is the quintessential autoimmune disease, with
antibodies to cellular constituents causing immune-mediated attack on various organs,
including the skin, joints, peripheral and central nervous system, kidneys, and serosal
surfaces. In children, the disease is more severe, with a higher incidence of renal and
neurologic involvement.
The classification system for SLE was revised in 2012, reflecting a harmonization
between the newer criteria of the Systemic Lupus International Collaborating Clinics
(SLICC) group and the criteria of the American College of Rheumatology (ACR),
which had been the standard classification system for decades. Table 101.1 lists the
ACR criteria and the updated revised SLICC criteria and definitions. This newer
classification differs from the 1997 ACR criteria in two significant ways. First, the
SLICC criteria were expanded to include 17 individual elements, rather than 11, thus
greatly expanding the breadth of the diagnosis. Secondly, the diagnosis rests on the
presence of one immunologic criterion as well as the presence of at least one clinical
criterion, rather than the previous format that relied on only clinical symptoms in some
cases. The SLICC criteria have improved sensitivity (97%) but decreased specificity
(84%) when compared to the 1997 ACR criteria.
