Newborn screening for sickle cell disease: when is an infant 'lost to follow-up'?

Article Abstract:

Many states now routinely screen newborns for sickle cell (SC) disease. Sickle cell disease is a serious, chronic anemic condition that occurs in people who are homozygous (have two genes) for hemoglobin S. This abnormal hemoglobin results in crescent-shaped red blood cells, causing crises of joint pain and fever with enlargement of the spleen, lethargy and weakness. Success of the screening program depends on prompt follow-up. Parents of babies identified must be notified immediately so that treatment and parent education and counseling can begin. In order to reduce illness and risk of death, this should occur before the baby reaches the age of four months. Contacting parents may be difficult, particularly in large urban areas. In an effort to define the problems encountered in achieving prompt parent notification and initiation of treatment, a study was undertaken of patients identified by routine newborn screening during a seven-month period. There were 25 babies identified to have SC disease; only nine of these babies (36 percent) arrived for their initial appointment. By the age of four months, only 64 percent of infants had been seen; at seven months one baby still had not been seen. All infants who had been initially seen were seen before they experienced any clinical complications. The authors suggest that institutions serving a large population at risk for SC disease designate one person to be contacted by the screening laboratory and to initiate the notification process. It is important that resources be made available to prevent a baby with SC disease from arriving at the emergency room, dead from a preventable complication of SC. (Consumer Summary produced by Reliance Medical Information, Inc.)

Care and treatment, Evaluation, Sickle cell anemia in children, Sickle cell anemia, Medical screening, Health screening

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Lumbar puncture frequency and cerebrospinal fluid analysis in the neonate

Article Abstract:

A newborn baby suspected of having generalized infection will often undergo lumbar puncture (LP). In this procedure a hollow needle is inserted into the subarachnoid space (the middle of three layers covering the spinal cord) of the lumbar (low back) portion of the spinal canal; cerebrospinal fluid can then be withdrawn and analyzed. Generalized infection can occur with or without meningitis (inflammation of the membranes covering the brain and spinal cord); meningitis is present in up to one third of newborns with generalized infection. Premature babies who show vague signs of infection may have lumbar puncture delayed and antibiotics begun empirically; or, they may undergo the procedure with its risks (trauma, infection). In special care nurseries the procedure may be performed by junior staff, not fully aware of its risks, who may not obtain enough fluid for all the required testing. A study was undertaken to determine the frequency and usefulness of LPs performed on newborns during their first week of life. There were 712 newborns who underwent 728 LPs. Eight patients had spinal fluid cultures positive for infection; only one patient had both blood culture positive for infection and clinical course for meningitis. When LP was performed later in life, 8 of 114 patients had positive cultures. These results suggest that routine use of LP in the first week of life may not be necessary when generalized infection is suspected but asymptomatic. (Consumer Summary produced by Reliance Medical Information, Inc.)

Methods, Infection, Neonatal intensive care, Meningitis, Spine, Lumbar puncture, Infection in children

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Early diagnosis of spastic diplegia, spastic hemiplegia, and quadriplegia

Article Abstract:

Cerebral palsy (CP), a disorder of the motor nerves, is caused by a birth injury or brain defect. There is a high rate of CP in children born of very low birth weights, namely those weighing less than 1500 grams or 3.3 pounds. There are three types of CP: spastic diplegia, affecting parts of both sides of the body; spastic hemiplegia, affecting one side of the body; and spastic quadriplegia, affecting the arms, legs and body. Diagnosis of CP during infancy is difficult because motor skills at that age are undeveloped. To determine factors that can predict the development of CP over a period of time, 108 nonhandicapped and 32 handicapped infants were studied. Values from the Bayley Mental and Motor Scales and the Movement Assessment of Infants Scale (MAIS), assessment tools for the prediction of CP, were obtained at four and 12 months of age. Children with all types of CP scored low on the Bayley Mental Scale at four months of age. Children with hemiplegia and quadriplegia also scored low on the Bayley Motor Scale. When compared with nonhandicapped children, children with hemiplegia and quadriplegia had scores placing them at higher risk. The MAIS tool was more sensitive in detecting motor problems in the four-month-olds with diplegia and hemiplegia, while the Bayley Motor scale was more efficient in detecting abnormalities in all three types of CP at 12 months. (Consumer Summary produced by Reliance Medical Information, Inc.)

Testing, Risk factors, Cerebral palsy, Child development, Movement disorders, Quadriplegia, Movement disorders in children, Bayley Scales of Infant Development, Hemiplegia, Paraplegia

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