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Biomedical subjects

E B Charney

Publications and source records attributed to E B Charney.

At least 19 recordsLinked to original sources

Energy expenditure of children and adolescents with severe disabilities: a cerebral palsy model.

Spastic quadriplegic cerebral palsy (SQCP) is a severe disability that is associated with abnormal physical activity, body composition, and food intake and with frequent malnutrition. This study examined the pattern of dietary intake, anthropometry, and energy expenditure in a group of subjects with SQCP aged 2-18 y and a normal control group. The energy expenditure pattern was determined from resting energy expenditure (REE, n = 61 SQCP; n = 37 control group) by using indirect calorimetry and from total energy expenditure (TEE, n = 32 SQCP; n = 32 control group) by using the doubly labeled water method. Physical activity, including the chronic spasticity of SQCP, was estimated from the ratio of TEE to REE. Abnormal growth and body composition were common and dietary intake was markedly overreported in the children with SQCP. Children with SQCP were divided according to body fat stores determined by triceps-skinfold-thickness measurements. The children with low fat stores had a lower REE adjusted for fat-free mass compared with the SQCP and control groups with adequate fat stores. TEE was significantly lower for the SQCP group than for the control group. The ratio of TEE to REE, indicating energy for nonbasal needs, was significantly lower in the SQCP children than in the control group, with the adequately nourished SQCP children having lower ratios than the more poorly nourished SQCP group. The nonbasal energy expenditure, such as for physical activity and spasticity, of children with SQCP was low. The nutrition-related growth failure and abnormal pattern of REE are likely related to inadequate energy intake.

Adolescent↗

Body composition in children with spastic quadriplegic cerebral palsy.

OBJECTIVE: To determine the pattern of body composition and nutritional status in a group of prepubertal children with spastic quadriplegic cerebral palsy (SQCP) compared with healthy control children. STUDY DESIGN: Subjects were enrolled for this cross-sectional study from two tertiary care settings. One hundred thirty-six subjects with SQCP, 2 to 12 years of age, were evaluated by anthropometric measures, or by anthropometric and total body water (TBW) measures (n = 28), with 39 control subjects. RESULTS: Body composition and nutritional status indicators were significantly reduced in children with SQCP. Accretion of fat-free mass with age was smaller for children with SQCP. Calculation of body fat from two skin folds correlated best with measures of fat mass from TBW. CONCLUSION: Malnutrition is common in children with SQCP. Clinically available, serial anthropometric measures enable the clinician to identify malnourished children with SQCP.

Age Factors↗

Nutritional status and growth of children with diplegic or hemiplegic cerebral palsy.

The growth failure and nutritional status were assessed of 154 children with diplegic or hemiplegic cerebral palsy, aged between two and 17.4 years. Linear growth was significantly reduced compared with norms for healthy children. About 30 per cent of the sample were undernourished, indicated by bodyweight or depleted subcutaneous fat stores at the triceps skinfold site. 8 per cent were overly fat by triceps skinfold and 14 per cent overweight by bodyweight. 23 per cent of the children had stunted growth. Children in the youngest age-group were most at risk for poor nutritional status and delayed growth. These findings suggest that children with diplegic or hemiplegic cerebral palsy are at risk for a variety of abnormalities of growth and nutritional status, and that a growth and nutritional assessment should be conducted periodically so that under- or overnutrition can be corrected when identified.

Adolescent↗

Nutrition-related growth failure of children with quadriplegic cerebral palsy.

Growth failure and nutritional status were evaluated in 142 children with quadriplegic cerebral palsy, aged between two and 18 years. Linear growth was assessed by upper-arm and lower-leg lengths: both means were significantly reduced. Mean bodyweight and triceps skinfold thickness fat stores were reduced to 65 per cent medians and subcapsular skinfold fat stores to 81 per cent median. Muscle stores were the most preserved and reduced to 88 per cent median. Examination of the sample by age-group showed significant reductions in growth and nutrition status indicators, even at two to four years, except for muscle area. To determine the degree to which nutritional status affected linear growth, a set of two-step regression analyses was conducted. The linear growth measures were significantly correlated with the measures of nutritional status.

Adolescent↗

Ventriculitis in newborns with myelomeningocele.

It has frequently been cited that a delay in back closure of beyond age 48 hours in newborns with myelomeningocele is associated with an increased risk of ventriculitis. It has been suggested that antibiotic therapy prior to surgery might minimize this risk. We reviewed our experience with ventriculitis in newborns with myelomeningocele and its relationship to antibiotic usage and time of back closure. Of 186 newborns, ventriculitis developed in 13 (7%), and there was no observed difference by time of back closure, clinical severity of infant at birth, status of myelomeningocele sac at birth, or type of delivery. There was a significant association between antibiotic usage and ventriculitis among the infants with delayed surgery, of which only one (1%) of 73 receiving broad-spectrum antibiotic prophylactic therapy developed ventriculitis compared with five (19%) of the 27 who did not receive antibiotics. Our data support the suggestion that antibiotic treatment is effective in minimizing the risk of ventriculitis among infants with myelomeningocele undergoing surgery after 48 hours of age.

Anti-Bacterial Agents↗

Parental attitudes toward management of newborns with myelomeningocele.

The parents of 50 children with myelomeningocele completed a questionnaire assessing their attitudes toward the medical management of their newborn infants. Of the 47 mothers and 29 fathers interviewed, only 26 were satisfied with the information they had received at the delivery hospital, whereas 61 expressed satisfaction with the information given at the tertiary-care hospital. Satisfaction was significantly more common when parents had been involved in decisions about the management of their newborn infants. The people most commonly cited as being helpful in making decisions were physicians at the tertiary-care hospital, spouses and other family members. The most important factors in reaching a decision were the information received at the tertiary-care hospital and the parents' own personal philosophy and social values. Most parents felt that they should have the final decision about the medical management of their newborn infants.

Attitude to Health↗

Assessment of linear growth of children with cerebral palsy: use of alternative measures to height or length.

Various linear measurements were made of 100 children with cerebral palsy to evaluate the effects of various factors on growth, and to investigate the use of alternative measurements to height or recumbent length. Linear growth was more retarded in children with spastic quadriplegia than in those with less widespread spasticity, and there was a tendency for the older children to be more growth-retarded. Upper-arm and lower-leg lengths provided useful information about linear growth. Growth charts using these alternative measurements have been developed which can be used to assess linear growth of children with cerebral palsy when it is difficult or impossible to measure height or recumbent length because of joint contractures. These charts should improve the assessment of the nutritional status of children with cerebral palsy.

Anthropometry↗

Preliminary observations on maternal rating of health of children: data from three subspecialty clinics.

Although routinely used in health surveys, the factors affecting maternal perceptions of health in children, especially those with health problems, have received little examination. This question has been addressed using a telephone interview of families of 483 children followed in one of three clinics at Children's Hospital of Philadelphia: the Neonatal Follow-up Program, the Pediatric Rheumatology Center and the Spina Bifida Program. Limitations in activities of daily living due to the health of the child, medical care use and variables indicative of socio-economic disadvantage were associated with a maternal rating of child health as fair/poor in all groups. The relationship was strongest in the rheumatologic group and less strong in the other two, suggesting that mothers may distinguish between a chronic active disease in a previously healthy child and the disability following a neonatal event in conceptualizing health. The results add to our information about maternal perceptions in assessing child health and suggest ways in which such ratings may prove useful in assessing health and health services for children with health problems.

Activities of Daily Living↗

Management of Chiari II complications in infants with myelomeningocele.

Vocal cord paralysis with inspiratory stridor during infancy is a well-recognized complication of the Arnold-Chiari malformation associated with myelomeningocele. Management of these symptoms, however, remains controversial. The outcome in 19 infants with symptoms managed by us during 1978-1984 was therefore reviewed. Ten infants had stridor alone at presentation (clinical grade 1), four infants also had apnea (grade 2), and five infants also had associated cyanotic spells and dysphagia (grade 3). Of 14 ventricular shunt revisions performed, seven were associated with resolution: in five of eight infants with grade 1, two of four with grade 2, and none of two with grade 3 symptoms. Of the 10 posterior fossa decompressions performed, two were associated with clinical resolution: in one of four infants with grade 1, one of two with grade 2, and none of four with grade 3 symptoms. Mortality over 6 months following onset of symptoms was absent among infants with grade 1, one with grade 2, and three with grade 3 symptoms. We propose that differences in outcome among the three groups of infants reflect differences in pathologic processes within the brain stem. We speculate that infants with grade 2 or 3 symptoms have more extensive brain stem damage, such as hemorrhage, infarction, and necrosis. Use of our classification system may be helpful in further studies on intervention modalities in these infants.

Arnold-Chiari Malformation↗

Symptomatic Arnold-Chiari malformation: review of experience with 22 cases.

Twenty-two patients with closed myelomeningoceles, shunted hydrocephalus, and symptomatic Arnold-Chiari malformations were studied retrospectively. Seventeen of the 22 patients were aged 6 months or younger; five patients were aged 3 to 23 years. Patients in the younger group presented with stridor, apnea, and/or feeding difficulty; those in the older group presented with hemiparesis, quadriparesis, oscillopsia, nystagmus, or opisthotonos. Fourteen of the 17 younger patients underwent surgical decompression of the Arnold-Chiari malformation: 10 within 18 days following the onset of symptoms and four on Day 19 or later. Of the 10 infants with early treatment, five eventually died secondary to continued symptoms and five survived. Of those surviving, three were asymptomatic and two had continuing symptoms but were improved over their preoperative state. Among the four patients undergoing surgery later, two died, one had lessening of stridor, and one had complete relief of symptoms. Of the three infants not undergoing decompression, two died and one eventually became asymptomatic. All five of the older patients underwent decompression and all had complete resolution of their preoperative symptoms and signs. The authors conclude that while decompression of a symptomatic Arnold-Chiari malformation may be effective in children and adults, this treatment does not always improve the clinical condition of infants. This may be the result of ischemic/hypoxic effects on the infant's brain stem, which may not be organized normally at birth.

Adult↗

Assessing the impact of a child with spina bifida on the family.

This study examined the characteristics of families which may be more vulnerable than others to the impact of caring for a child with spina bifida. Morbidity and the use of health services were unexpectedly high. The impact on the family was related less to clinical diagnoses than to the characteristics of the child's functioning in the home and of the family. Major predictors of greater impact on the family were the number of the child's activities of daily living, parental perceptions of the child's health, low maternal educational attainment, low family income, the number of adults in the family, insurance status, the number of visits to a doctor in the month before the interview, and whether the adults in the family were employed. These results reinforce the need for assessments to include the child's function in the home, and for additional resources to help some families care for their child.

Adolescent↗

Fibrodysplasia ossificans progressiva presenting as osteomyelitis-like syndrome.

An 8-year-old boy presenting with fever, neck pain, and torticollis was eventually diagnosed as having fibrodysplasia ossificans progressiva (FOP). The initial bone scan was interpreted as being suggestive of vertebral osteomyelitis. Subsequent computerized tomographic studies (CT scan), however, demonstrated paravertebral soft-tissue calcification adjacent to, but not involving, intact cervical vertebrae. The acute process of FOP was thereby distinguished from that of osteomyelitis by use of CT scan.

Cervical Vertebrae↗

Medical management of bilateral renal malakoplakia.

Renal malakoplakia is reported in an 11 year old girl with myelomeningocele and associated neuropathic bladder. She is the first reported child to have survived bilateral renal malakoplakia with full recovery of renal function after medical management.

Child↗

Management of the newborn with myelomeningocele: time for a decision-making process.

The relationship between time of surgical intervention and eventual outcome was examined in 110 newborns with myelomeningocele. Numerous earlier reports have cited a significant increase in mortality and morbidity associated with delay of surgery beyond 48 hours. Within the study population of infants, 52 infants (47%) had "early" surgery within the first 48 hours of life, 32 infants (29%) had "delayed" surgery between 3 and 7 days of age, 12 infants (11%) had "late" surgery between 1 week and 10 months of age, and 14 infants (13%) never had surgery by parental decision. Survival rates were similar between those with early, delayed, or late surgery as 92%, 94%, and 100%, respectively, were alive at age 10 months. Also, no significant association existed between time of surgery and development of ventriculitis, developmental delay, or worsening of paralysis. From these observations, it is concluded that there is no urgency in surgical intervention for the initial management of newborns with myelomeningocele. Rather, there is time for comprehensive discussions, counseling, and emotional support for those parents in need of a decision-making process before establishing consent for or against surgical management of their newborn.

Bacterial Infections↗

Assessment of stature of children with myelomeningocele, and usefulness of arm-span measurement.

The lengths and arm spans of 99 children with myelomeningocele were measured. 52 of the children had short stature (less than the 3rd percentile): this occurred in 80 per cent of those with lesions at L3 or higher and in 43 per cent of those with L4-L5 lesions. Stature was unaffected among children with S1 or lower lesions. 54 per cent of all the children had an arm span wider than the anticipated range for length; of these, 69 per cent also had short stature. It is concluded that short stature is common among children with myelomeningocele, particularly those with the highest spinal lesions, and that arm span may be a more useful measure than length in evaluating growth velocity of these children.

Adolescent↗