ABSTRACT
OBJECTIVE: Autoantibodies recognizing 3-hydroxy-3-methylglutaryl-coenzyme A reductase (HMGCR) are associated with statin exposure, the HLA allele DRB1*11:01, and necrotizing muscle biopsies in adult myositis patients. The aim of this study was to characterize the features of juvenile anti-HMGCR-positive myositis patients. METHODS: The sera of 440 juvenile myositis patients were screened for anti-HMGCR autoantibodies. Demographic and clinical features, responses to therapy, and HLA alleles were assessed. The features of anti-HMGCR-positive patients were compared to those of previously described adult patients with this autoantibody and to children with other myositis-specific autoantibodies (MSAs). RESULTS: Five of 440 patients (1.1%) were anti-HMGCR-positive; none had taken statin medications. Three patients had rashes characteristic of juvenile dermatomyositis and 2 patients had immune-mediated necrotizing myopathies. The median highest creatine kinase (CK) level of anti-HMGCR-positive subjects was 17,000 IU/liter. All patients had severe proximal muscle weakness, distal weakness, muscle atrophy, joint contractures, and arthralgias, which were all more prevalent in HMGCR-positive subjects compared to MSA-negative patients or those with other MSAs. Anti-HMGCR-positive patients had only partial responses to multiple immunosuppressive medications, and their disease often took a chronic course. The DRB1*07:01 allele was present in all 5 patients, compared to 26.25% of healthy controls (corrected P = 0.01); none of the 5 juvenile patients had DRB1*11:01. CONCLUSION: Compared to children with other MSAs, muscle disease appears to be more severe in those with anti-HMGCR autoantibodies. Like adults, children with anti-HMGCR autoantibodies have severe weakness and high CK levels. In contrast to adults, in anti-HMGCR-positive children, there is a strong association with HLA-DRB1*07:01.
Subject(s)
Acyl Coenzyme A/blood , Autoantibodies/blood , Myositis/blood , Myositis/diagnosis , Nerve Tissue Proteins/blood , RNA-Binding Proteins/blood , Severity of Illness Index , Adolescent , Biomarkers/blood , Child , Child, Preschool , Female , Humans , MaleABSTRACT
PURPOSE OF REVIEW: Episodes of acute illness associated with fever are common in children. Less common but also diagnostically challenging are episodes of prolonged fever in children. This article reports recent literature that has focused on the epidemiology, pathophysiology, clinical and laboratory evaluation, and treatment of both types of episodes. RECENT FINDINGS: A number of articles reviewed the epidemiology in several countries of bacteremia in children, including bacteremia caused by Streptococcus pneumoniae and Salmonella sp. Host susceptibility factors for bacterial illness, such as malnutrition, were reported. Diagnostic issues concerning bacteremia, including the technique of venipuncture and urine antigen testing, were of interest. A major study was published that reported a preventive approach through immunization to one type of bacteremia that caused by S. pneumoniae. Other studies addressed the issues of choice of therapy and duration of therapy in selected bacteremic disease, such as dental bacteremias and bacteremia, that were caused by Brucella melitensis. Lastly, several causes of prolonged fever (such as hemophagocytic syndrome and atypical cat scratch disease) were reported. SUMMARY: During the review period, studies addressed diverse issues related to acute and prolonged episodes of fever in children. There was a particular emphasis on the epidemiology, diagnosis, prevention, and treatment of bacteremic illness in children.
Subject(s)
Fever of Unknown Origin/diagnosis , Fever of Unknown Origin/therapy , Age Factors , Child , Child Nutrition Disorders/complications , Child, Preschool , Fever of Unknown Origin/etiology , Humans , Infant , Infant, NewbornABSTRACT
The etiology of pallidosis, a disease of strawberry identified more than 45 years ago, remains unknown. We report a putative agent of the disease, a virus belonging to the Crinivirus genus of the Closterovirideae family. A sensitive reverse transcription-polymerase chain reaction (RTPCR) test has been developed. Polyclonal antibodies that can be used to detect the virus in petiole tissue blots were developed using a recombinant virus coat protein. The nucleotide sequences of regions of the viral genome that encode the heat shock protein 70 homolog and the major coat protein were obtained. Alignments of the major coat protein show that the virus isolated from strawberry plants positive for pallidosis is most closely related to Cucumber yellows virus (syn. Beet pseudo-yellows virus) and Cucurbit yellow stunt disorder virus, members of the Crinivirus genus.
ABSTRACT
This section focuses on issues in infectious disease that are commonly encountered in pediatric office practices. Dr. McCarthy discusses recent literature regarding the evaluation and management of acute fevers without apparent source on clinical examination in infants and children and the evaluation of children with prolonged fevers of unknown origin. Drs. Klig and Chen (pp 121-126) review recent literature about lower respiratory infection in children. This section focuses on febrile children in whom a source of fever is not readily apparent on clinical examination. This issue is discussed in several contexts: recent developments concerning the epidemiology, pathophysiology, diagnostic approach, and therapy of febrile illnesses; children from 3 to 36 months of age with fever; infants younger than 90 days of age with fever; and children of any age with prolonged fever, usually lasting more than 7 to 10 days, for whom a diagnosis has not been established.
Subject(s)
Fever of Unknown Origin/diagnosis , Fever of Unknown Origin/etiology , Physical Examination , Adolescent , Child , Child, Preschool , Fever of Unknown Origin/therapy , Humans , Infant , Infant, NewbornABSTRACT
Although asthmatic children spend a significant amount of time in child care programs, little is known about child care program directors' knowledge about asthma and what factors influence directors' level of knowledge about asthma. A telephone interview was conducted with directors of a randomly selected sample of all licensed child care centers and group day care homes in Connecticut. Directors of most child care programs were willing to admit asthmatic children to their programs, but their knowledge about asthma needs to be improved. Directors who have served for long periods of time may be a target group for additional education about asthma.
Subject(s)
Asthma/therapy , Child Day Care Centers/organization & administration , Health Education/organization & administration , Health Knowledge, Attitudes, Practice , Needs Assessment , Adult , Child Welfare , Child, Preschool , Connecticut , Female , Health Care Surveys , Humans , Interpersonal Relations , Male , Program Evaluation , Risk Assessment , Statistics, Nonparametric , Surveys and QuestionnairesABSTRACT
A simple, nondestructive PCR-based screening method has been developed for identifying putative transgenic soft white winter wheat (Triticum aestivum L.) carrying the coat protein gene of wheat streak mosaic virus. Removal of the endosperm end of individual seed provided sufficient material for DNA extraction and PCR. DNA from seed is more free of the secondary, metabolites found in leaf tissue that can inhibit both PCR and restriction digests required for Southern analysis. The half-seed PCR assay has comparable accuracy to the leaf-tissue PCR assay and hence can be used as an accurate and rapid method for identifying transformed lines before planting. Germination of the remaining seed portion showed germination rates comparable to whole-seed controls. A slight delay in growth from the first-leaf through the first-tiller stage was observed in the half-seed-derived plants, as compared to plants grown from whole seed.
