Marfanoid children: etiologic heterogeneity and cardiac findings

Article Abstract:

Children suspected to have Marfan's syndrome may have a distinct (''Marfanoid'') appearance. These characteristics include tall, slim body build; long arms, legs, fingers and toes; depressed breast bone or hollow chest; scoliosis (sideways curvature of the spine), and myopia (nearsightedness). Marfan's syndrome is an inherited disorder of the connective tissue of the body and has been related with significant heart abnormalities. These include enlargement of the aortic root, heart valve disorders (mitral prolapse, regurgitation and aortic regurgitation) and aortic dissection (separation of the layers of the wall of the aorta). However, Marfan's syndrome is not the only cause of Marfanoid appearance, and it may be difficult to diagnose, especially in young children. A retrospective study was undertaken to analyze the clinical and echocardiographic findings of children with Marfanoid appearance. Fifteen were diagnosed with Marfan's syndrome, two had possible Marfan's syndrome and three did not have Marfan's syndrome. The most common bone abnormality was hollow chest (16 of 20 patients). At initial evaluation, eight children (53 percent) with Marfan's syndrome had mitral valve regurgitation. Aortic root enlargement was identified by echocardiogram in 12 of the 15 patients (80 percent). No patient had a normal echocardiogram. During follow-up (minimum of six months) one patient developed aortic root enlargement and another developed prolapse of the mitral valve. It is suggested that echocardiography be used routinely in the diagnosis and follow-up of patients suspected of having Marfan syndrome. (Consumer Summary produced by Reliance Medical Information, Inc.)

Diagnosis, Echocardiography, Connective tissue diseases, Marfan syndrome

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Acute osteomyelitis in children: reassessment of etiologic agents and their clinical characteristics

Article Abstract:

Osteomyelitis, local or generalized infection of the bone and bone marrow, can occur in children. Although the usual cause of infection is Staphylococcus aureus, recently Haemophilus influenzae has been identified as a potential cause of osteomyelitis in young children. A study was undertaken of 135 children with osteomyelitis to identify the cause and characteristics of the condition. Half of the children were under the age of five, and children under the age of one had the highest rate of occurrence. The most common symptom was pain (94 percent of patients), and 85 percent of the children had elevation of temperature. The most common site was the thigh bone (39 patients) followed by the tibia (lower leg bone) in 20 patients. The bacterial cause of infection was S. aureus in 25 percent, H. influenzae type b in 12 percent, and Pseudomonas aeruginosa in 6 percent. H. influenzae occurred only in children under the age of three. Clinical and laboratory findings of these children were similar to those of children with osteomyelitis caused by different organisms. Patients with infection due to P. aeruginosa were all over the age of 9, and had penetrating injury to the foot. These children were mostly boys (83 percent) and often did not have fever (85 percent). Guidelines are presented for diagnostic work-up and treatment of osteomyelitis. (Consumer Summary produced by Reliance Medical Information, Inc.)

Research, Causes of, Demographic aspects, Bacterial infections, Osteomyelitis, Pediatric orthopedics

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Compliance with penicillin prophylaxis in patients with sickle cell disease

Article Abstract:

Patients with sickle cell disease do not seem to comply with a regimen of penicillin, and also do not report their non-compliance when asked. Sickle cell disease makes children vulnerable to infection by Streptococcus pneumoniae resulting in bacteremia and meningitis, which penicillin maintenance can prevent. Of 123 patients between 3 months and 24.2 years of age, 67.5% claimed compliance, but urine tests showed only 43.1% compliance. Compliance was greater in children under five, and in those families with private, rather than public insurance.

Usage, Infection, Prevention, Statistics, Pediatric diseases, Sickle cell anemia, Patient compliance, Penicillin, Penicillins, Streptococcus pneumoniae, Infection in children

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