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Childcare and Communicable Diseases

More than 20 million children <5 yr old attend a childcare facility. These facilities can include part-day or full-day programs at nursery schools or preschools and full-day programs based in either a licensed childcare center or another person's home. Regardless of the age at entry, children entering daycare are more prone to infections, largely from the exposure to greater numbers of children. Childcare facilities can be classified…

Infection Prevention and Control

Infection prevention and control ( IPC ) programs have an important role in pediatric medicine. To be fully effective, IPC programs require a functional infrastructure that addresses collaboration with the public health system, widespread immunizations, and use of appropriate techniques to prevent transmission of infection within the general population and within healthcare institutions. The national focus on preventing healthcare-associated infection (HAI) is exemplified by The Joint…

Immunization Practices

Immunization is one of the most beneficial and cost-effective disease-prevention measures available. As a result of effective and safe vaccines, smallpox has been eradicated, polio is close to worldwide eradication, and measles and rubella are no longer endemic in the United States. However, cases of vaccine-preventable diseases, including measles, mumps, and pertussis, continue to occur in the United States. Incidence of most vaccine-preventable diseases of childhood…

The Microbiome and Pediatric Health

From the time of birth, the human infant is exposed to a myriad of microbes found on the mother and in the surrounding environment. Microbes rapidly form assemblages across exposed areas of the body, including the skin and enteral tract. The microbial communities are called the microbiota and make a substantial impact on short- and long-term physiology, including immunologic and metabolic development and function. Together the…

Diagnostic Microbiology

Laboratory evidence to support the diagnosis of an infectious disease may be based on one or more of the following: direct examination of specimens using microscopic or antigen detection techniques, isolation of microorganisms in culture, serologic testing, host gene expression patterns, or molecular detection of an organism, resistance determinant, or virulence factor. Some additional roles of the clinical microbiology laboratory include performing antimicrobial susceptibility testing and…

Miscellaneous Conditions Associated With Arthritis

Relapsing Polychondritis Relapsing polychondritis (RP) is a rare condition characterized by episodic chondritis causing cartilage destruction and deformation of the ears (sparing the earlobes), nose, larynx, and tracheobronchial tree. Antibodies to matrillin-1 and collagen (type II, IX and XI) are present in approximately 60% of patients with RP, suggesting an autoimmune pathogenesis. Patients may experience arthritis, uveitis, and hearing loss resulting from inflammation near the auditory…

Musculoskeletal Pain Syndromes

Musculoskeletal pain is a frequent complaint of children presenting to general pediatricians and is the most common presenting problem of children referred to pediatric rheumatology clinics. Prevalence estimates of persistent musculoskeletal pain in community samples range from 10–30%. Although diseases such as juvenile idiopathic arthritis (JIA) and systemic lupus erythematosus (SLE) may manifest as persistent musculoskeletal pain, the majority of musculoskeletal pain complaints in children are…

Vasculitis Syndromes

Childhood vasculitis encompasses a broad spectrum of diseases that share inflammation of the blood vessels as the central pathophysiology. The pathogenesis of the vasculitides is generally idiopathic. Some forms of vasculitis are associated with infectious agents and medications, whereas others may occur in the setting of preexisting autoimmune disease. The pattern of vessel injury provides insight into the form of vasculitis and serves as a framework…

Kawasaki Disease

Kawasaki disease (KD), formerly known as mucocutaneous lymph node syndrome and infantile polyarteritis nodosa , is an acute febrile illness of childhood seen worldwide, with the highest incidence occurring in Asian children. KD is a systemic inflammatory disorder manifesting as a vasculitis with a predilection for the coronary arteries. Approximately 20–25% of untreated children develop coronary artery abnormalities (CAA) including aneurysms, whereas <5% of children treated…

Sarcoidosis

Sarcoidosis is a rare multisystem granulomatous disease of unknown etiology. The name is derived from a Greek word meaning “flesh-like condition,” in reference to the characteristic skin lesions. There appear to be 2 distinct, age-dependent patterns of disease among children with sarcoidosis. The clinical features in older children are similar to those in adults (pediatric-onset adult sarcoidosis), with frequent systemic features (fever, weight loss, malaise), pulmonary…

Amyloidosis

Amyloidosis comprises a group of diseases characterized by extracellular deposition of insoluble, fibrous amyloid proteins in various body tissues. Etiology Amyloidosis is a disease caused by protein misfolding. These misfolded proteins infiltrate, aggregate, and form insoluble fibrils that can affect the normal function of a number of vital organs. In the amyloidosis nomenclature, a distinction is made between amyloidosis that develops from mutations in the amyloid…

Hereditary Periodic Fever Syndromes and Other Systemic Autoinflammatory Diseases

The hereditary periodic fever syndromes are a group of monogenic diseases that present with recurrent bouts of fever and associated pleural and/or peritoneal inflammation, arthritis, and various types of skin rash. A number of identifiable disorders present with recurrent episodes of inflammation, although fevers may not be common feature. Therefore the term systemic autoinflammatory diseases is used to include all diseases that present with seemingly unprovoked…

Sjögren Syndrome

Sjögren syndrome is a chronic, inflammatory, autoimmune disease characterized by progressive lymphocytic and plasma cell infiltration of the exocrine glands, especially salivary and lacrimal, with potential for systemic manifestations. It is rare in children and predominantly affects middle-age women with classic symptoms of dry eyes ( keratoconjunctivitis sicca ) and dry mouth ( xerostomia ). Epidemiology Sjögren syndrome typically manifests at 35-45 yr of age, with 90%…

Behçet Disease

Behçet disease (BD) is classified as a primary variable vessel vasculitis , emphasizing the involvement of any size and type (arterial, venous) of blood vessel. BD is also recognized as an autoinflammatory disease. Originally described with recurrent oral ulcerations, uveitis, and skin abnormalities, the BD spectrum is much broader. Epidemiology Behçet disease has a high prevalence in countries along the Silk Road, extending from Japan to…

Scleroderma and Raynaud Phenomenon

Juvenile scleroderma encompasses a range of conditions unified by the presence of fibrosis of the skin. Juvenile scleroderma is divided into 2 major categories, juvenile localized scleroderma ( JLS , also known as morphea ), which is largely limited to the skin, and juvenile systemic sclerosis ( JSSc ), with multisystem organ involvement. Localized disease is the predominant type seen in pediatric populations (>95%), but systemic…

Juvenile Dermatomyositis

Juvenile dermatomyositis (JDM) is the most common inflammatory myositis in children, distinguished by proximal muscle weakness and a characteristic rash. Inflammatory cell infiltrates result in vascular inflammation, the underlying pathology in this disorder. Etiology Evidence suggests that the etiology of JDM is multifactorial, based on genetic predisposition and an unknown environmental trigger. Human leukocyte antigen (HLA) alleles such as B8, DRB1*0301, DQA1*0501, and DQA1*0301 are associated…

Systemic Lupus Erythematosus

Systemic lupus erythematosus (SLE) is a chronic autoimmune disease characterized by multisystem inflammation and the presence of circulating autoantibodies directed against self-antigens. SLE occurs in both children and adults, disproportionately affecting females of reproductive age. Although nearly every organ may be affected, most frequently involved are the skin, joints, kidneys, blood-forming cells, blood vessels, and central nervous system. Systemic signs of inflammation such as fever and…

Reactive and Postinfectious Arthritis

In addition to causing arthritis by means of direct microbial infection (i.e., septic arthritis; see Chapter 705 ), microbes activate innate and adaptive immune responses, which can lead to the generation and deposition of immune complexes as well as antibody or T cell–mediated cross-reactivity with self. In addition, microbes may influence the immune system in ways that promote immune-mediated inflammatory diseases such as systemic lupus erythematosus…

Ankylosing Spondylitis and Other Spondyloarthritides

The diseases collectively referred to as spondyloarthritides include ankylosing spondylitis (AS) , arthritis associated with inflammatory bowel disease (IBD) or psoriasis, and reactive arthritis following gastrointestinal (GI) or genitourinary (GU) infections ( Table 181.1 and Table 181.2 ). Spondyloarthritis is more common in adults, but all forms can present during childhood with varying symptoms and signs. Many children with spondyloarthritis are classified in the juvenile idiopathic…

Juvenile Idiopathic Arthritis

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children and one of the more common chronic illnesses of childhood. JIA represents a heterogeneous group of disorders sharing the clinical manifestation of arthritis. The etiology and pathogenesis of JIA are largely unknown, and the genetic component is complex, making clear distinction among various subtypes difficult. As a result, several classification schemes exist, each with…