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At least 1,153 records · Page 64Linked to original sources
PubMed2006

Pregnancy outcome in the Psammomys obesus gerbil on low- and high-energy diets.

INTRODUCTION: Diabetes mellitus (DM) during pregnancy is associated with an increased risk for poor reproduction and a high rate of congenital malformations. The gerbil Psammomys obesus is a unique model for nutritionally induced Type 2 DM (T2DM) that enabled us to study the outcome of uncontrolled T2DM during pregnancy. METHODS: Female Psammomys on low-energy (LE) or high energy (HE) diet were studied. The blood glucose levels and weights of pregnant animals were determined. The offspring from the different groups were followed-up to weaning. RESULTS: Most of the HE-diet animals were diabetic (77%). There were no differences in the pregnancy rates in animals on both diets (32.7% in HE vs. 38.3% in LE). Pregnancy of the HE-diet group was longer than the LE-diet group (26.7 vs. 26.1 days), and litter average was reduced (2.7 vs. 3.0). At birth, the offspring of the HE-diet dams weighed less (5.2 vs. 7.2 g) and had smaller crown rump length (4.0 vs. 4.6 cm) These offspring also presented a 1-3 days delay in neuro-developmental parameters (first turn over, hair appearance, eye-opening and response to noise). However, from the fourth week of life they became diabetic, and from the third week they weighed more than the LE offspring. CONCLUSION: HE-diet caused diabetes, maternal complications and altered reproduction in Psammomys animals. The offspring of diabetic Psammomys presented birth weight and length changes as well as developmental delay.

Animal Feed↗
PubMed1992

3-Methylglutaconic aciduria: a new variant.

3-Methylglutaconic aciduria has been described in two distinct syndromes. In one there was deficient 3-methylglutaconyl coenzyme A hydratase in fibroblast extracts where the only clinical manifestation was retarded speech development. In the second syndrome, the enzyme activity was normal but prominent neurological deterioration was noted. We describe two siblings with 3-methylglutaconic aciduria with normal enzyme activity who had choreoathetoid movements, optic atrophy, and mild developmental delay. The boy demonstrated developmental improvement in his second year of life, and his sister developed well, with normal school performance. These patients represent a new clinical variant of the second syndrome with a relatively favorable prognosis.

Acidosis↗
PubMed1986

Elevated lead levels in children with nonorganic failure to thrive.

Every child with failure to thrive has at least one organic medical disease: malnutrition. It is well documented that lead and other heavy metals are absorbed more readily in the presence of both malnutrition and iron deficiency anemia. Malnutrition and lead exposure tend to be found in the same population groups. Furthermore, lead poisoning is correlated with many of the identical intellectual and behavioral deficits demonstrated in children suffering from nonorganic failure to thrive. Because of these facts, whole blood lead levels were determined for 45 children with nonorganic failure to thrive and 45 age-, race-, and socioeconomically matched comparison subjects. Children with failure to thrive had a lead level of 22.67 +/- 10.29 (micrograms/dL (mean +/- SD); for control children, it was 14.33 +/- 5.42 (P less than .001). Children with failure to thrive were more frequently anemic (P less than .0001), a possible lead effect, and had higher free erythrocyte protoporphyrin levels. Children with failure to thrive were developmentally delayed on the Denver Developmental Screening Test (unblinded observation) with high failure rates in both language (P less than .001) and gross motor skills (P less than .02). Although failure on the Denver Developmental Screening Test within the failure to thrive group was not linearly correlated with lead level, any such effects may have been masked by the effects of malnutrition and failure to thrive per se. A number of authors have suggested that lead levels formerly thought to be inconsequential are clinically toxic.(ABSTRACT TRUNCATED AT 250 WORDS)

Body Weight↗
PubMed1989

Can developmental screening tests identify children who are developmentally at risk?

Developmental screening tests are in widespread use, but few reliable and valid tests are available. The most frequently used screening instrument for detecting young children who are at risk for developmental delays is the Denver Developmental Screening Test (DDST). Although the DDST has excellent test specificity, overreferring few children, results from more than a dozen studies of the DDST's concurrent and predictive validity fail to replicate the original validation and demonstrate a uniformly poor sensitivity, ie, a high proportion of underreferrals. Whether samples are stratified by age, risk, duration of time between predictor and outcome, or type of outcome measure used, these studies demonstrate that the DDST underrefers children at nearly a 2:1 ratio. Several other screening tests with more optimal psychometric properties are presented. It is urged that caution be exercised in using tests for predicting the risk of developmental problems in young children unless the tests have acceptable levels of sensitivity and specificity.

Child↗
PubMedDate not supplied

Neurodevelopmental outcome and respiratory morbidity for extracorporeal membrane oxygenation survivors at 1 year of age.

Outcome at 1 year was evaluated in 37 neonates treated with extracorporeal membrane oxygenation (ECMO) between June 1987 and March 1989. Thirty of 37 survived, and 7 of 30 showed major abnormalities in respiratory status, neurologic examination, or developmental status. Abnormal respiratory status was defined as need for supplemental oxygen, tracheostomy, or mechanical ventilation, and developmental delay was defined as developmental quotient < 70 on either the Mental or Psychomotor scale of the Bayley Scales of Infant Development. Five of seven children with major abnormalities were affected in more than one system. Adverse outcome was associated with the presence of congenital anomalies. Perinatal factors including Apgar scores, last pH before starting extracorporeal membrane oxygenation, and number of hours on ECMO did not show a significant correlation with Bayley scores. Computed tomography and electroencephalography of neonates showed no relationship to Bayley scores or neurologic examination. In conclusion, a small subgroup of patients accounts for the majority of abnormal findings at 1 year, and the presence of congenital anomalies appears to increase the risk for abnormal outcome.

Cohort Studies↗
PubMed1993

Infants of less than 1250 grams birth weight at Groote Schuur Hospital: outcome at 1 and 2 years of age.

A prospective 2-year follow-up study of infants with birth weights of less than 1250 g was undertaken at Groote Schuur Hospital Neonatal Intensive Care Unit. For a 12-month period beginning July 1988, all live infants born at Groote Schuur Hospital or referred to the Neonatal Intensive Care Unit were included in the study cohort. The aim of the study was to document the morbidity, mortality, and neurodevelopmental outcome of these infants to 2 years of age. Of 235 liveborn infants, 143 (61%) survived to discharge. One hundred twenty-six infants were born weighing less than 1000 g; 42% survived to discharge. One hundred nine infants weighed 1000 g or more at birth, and 83% survived to discharge. Better survival was documented for infants whose mothers attended antenatal care, who weighed more than 900 g, and who were of greater than 30 weeks' gestation. Eleven infants died in the first 6 months after discharge. One hundred six infants (83% of survivors) underwent Griffiths developmental testing and clinical assessment at 1 year of age. Ninety-six (91%) of these survivors were seen and tested at 2 years of age. Of the 106 infants assessed at 1 year of age, 6 infants had cerebral palsy, 6 were globally developmentally delayed without signs of cerebral palsy, and 1 infant showed significant motor delay with a normal developmental quotient. At 2 years of age 1 additional infant had cerebral palsy and 9 more infants are likely to be mentally retarded. At 2 years of age the major handicap rate was, therefore, 22%. Sixty-nine percent of surviving infants, and all but 1 of the infants with cerebral palsy, were underweight for gestational age at birth. There was a tendency for these underweight-for-gestational-age infants to score less well at 2 years of age. Infants who received ventilation and infants with a birth weight of less than 1000 g were not found to score less well than other infants in the cohort.

Birth Weight↗
PubMedDate not supplied

Congenital myotonic dystrophy: report of one case.

We report a laboratory-confirmed pediatric case of congenital myotonic dystrophy (CMD) in Taiwan. A 5-year-old girl presented in our neurological clinic with the chief complaints of developmental delay and slowness in learning. Clinical examination, intelligence tests, and developmental examinations revealed myopathic facial weakness, passivity and delayed cognitive development. Past history review showed neonatal hypotonia, feeding difficulty with cyanosis, and poor activity. Neonatal studies only revealed subgaleal hematoma and subarachnoid hemorrhage with mild degree right side intraventricular hemorrhage. Current neurological examination disclosed percussion myotonia and myotonia as poor muscle relaxation after forceful handgrip. CMD was confirmed by electromyography study and DNA analysis. The patient's mother, though minimally affected, had similar laboratory results. CMD affects children in both motor and cognitive function; the diagnosis should be entertained in developmentally delayed children with subtle neurological signs, even without strong family history.

Child, Preschool↗
PubMedDate not supplied

Alignment success following medical rectus recessions in normal and delayed children.

Others have suggested that developmentally delayed children, who are frequently esotropic, have a poor prognosis following esotropia surgery. To date, no comparison of success rates in normal and delayed children following similar surgery has been made. We compared our long-term results following graded bilateral medial rectus recessions in consecutive normal (n = 62) and delayed (n = 29) children. We defined success as alignment maintained within 10 delta of orthophoria. Survival curves were compared using the Mantel-Haenszel statistic. Delayed children had a significantly poorer outcome (p = .05) throughout follow up, which ranged from 12 to 120 months (mean = 24 months). Early in the period of study, we observed a large proportion of overcorrections among the delayed children. Subsequent modification in the amount of surgery performed appeared to improve the initial and long-term success in these children. We conclude that delayed children do have a poorer prognosis than normal children following medial rectus recession. This prognosis may be improved by more conservative amounts of surgery.

Adolescent↗
PubMed1991

The effect of intrauterine alcohol exposition in various durations on early cognitive development.

Fifty-three children exposed to alcohol of various duration were examined at 18 to 19 months of age. No significant difference was found in developmental outcome between non-exposed (n = 56) and during the I trimester exposed children (n = 21). Exposure until the III trimester (n = 19) or continuous exposure (n = 13) caused significantly lower scores in language and total mental assessment. The procentual numbers of children with possible developmental risk (failure or pass at acceptance limit) in various developmental fields grew with increasing duration of intrauterine alcohol exposure. Failures in gross or fine motor items differed significantly between the non-exposed and the two long exposed children but in language items only between the non-exposed and the children exposed until the III trimester. A high number of exposed children scored just the limit values indicating a milder form of developmental delay. In comparison with the developmental results of one year of age an increase in delay in cognitive development was seen. The incidences of other teratogenic effects (pre- or postnatal growth retardation, facial dysmorphia) of fetal alcohol exposure were highest in the continuous exposed group. Five (38%) of the continuous exposed children had typical fetal alcohol syndrome and only 1 (5%) of the children exposed until the III trimester.

Child, Preschool↗
PubMed2006

Clinical manifestations and neurodevelopmental outcome following an event of accidental intramuscular injection of atracurium in newborns.

UNLABELLED: Pediatric populations are at risk for medication errors that may be associated with mortality and disability. The purpose of this study was to describe the clinical manifestations of seven newborns following an event of accidental intramuscular injection of atracurium and to assess the impact on neurodevelopmental outcome. This study enrolled seven newborns who were accidentally administered 10 mg of atracurium, equivalent to 2.6-3.3 mg/kg, in a local perinatal clinic. Accident reports and hospital records were reviewed to obtain the history and medical data for the event. The survivors were prospectively examined for their growth, health and neurodevelopment until 24 months of age. All newborns showed immediate apnea and cyanosis requiring resuscitation after atracurium injection and presented with respiratory failure and flaccid paralysis on arrival for emergency medical services. One newborn was asystolic despite resuscitation and died of multiple organ failure. Of the five survivors available for follow-up, all achieved favorable growth outcomes. However, four showed mild to significant delay in development; and two manifested mild hypomyelination of cerebral white matter on the brain magnetic resonance imaging. CONCLUSION: Newborns accidentally injected with high doses of atracurium are at risk of death and neurodevelopmental delay. The serious clinical manifestations, developmental delay and cerebral hypomyelination were most likely due to insufficient immediate respiratory assistance following atracurium injection.

Atracurium↗
PubMedDate not supplied

Nonlissencephalic cortical dysplasias: correlation of imaging findings with clinical deficits.

PURPOSE: To establish correlations between MR patterns and clinical outcome in patients with nonlissencephalic cortical dysplasias. PATIENTS AND METHODS: MR and clinical data from 36 patients with cerebral cortical gyral anomalies (other than classical type I or type II lissencephaly) were retrospectively reviewed. RESULT: The five patients with diffuse cortical dysplasia, including two with congenital infections, had microcephaly and severe development delay from a very early age. Infantile spasms occurred in three of the five. Focal areas of cortical dysplasia were most common in the frontal lobes, but were seen in all areas of the brain. The most common MR appearances were 1) a thickened, irregularly bumpy cortex with shallow, wide sulci, and 2) a deep infolding of thickened cortex. The twelve patients with bilateral focal dysplasia had a high incidence of bilateral motor dysfunction (67%), delayed speech (67%), and generalized developmental delay (92%). When the dysplasia was unilateral, contralateral spastic hemiplegia or monoplegia was present in 14 of 19 patients (74%), but dysphasia was uncommon, even in patients with dysplasia in the frontal lobe of the dominant hemisphere. CONCLUSION: Surgical resection of focal areas of cortical dysplasia in patients with medically refractory seizures is becoming more common, and the neuroradiologist will play an increasingly important role in the initial diagnosis and delineation of these anomalies.

Adolescent↗
PubMedDate not supplied

Profiles of skill gain in delayed infants and young children.

Thirty-eight developmentally delayed preschool children were entered into a study to assess their developmental progress on skills checklists during a 3-month period. All children were receiving the South Glamorgan Home Advisory Service. Weekly tasks were set in a balanced manner. Children were assessed on twelve developmental checklists both prior to and following the study period. Results showed that in general children made greater progress in inspection/tracking and perceptual problem-solving skills than in motor, self-help and visuomotor areas. Separate analyses for Down's children and for children of different developmental levels indicated slightly altered developmental profiles. Results are discussed in terms of implications for early intervention programmes.

Child, Preschool↗
PubMed2001

The development of young children with retinoblastoma.

OBJECTIVES: To assess the health and development of children with retinoblastoma (RB), or cancer of the retina, and to determine if they are at greater risk for developmental delays than normal children. Specific aims were to determine if type of RB (unilateral vs bilateral), family history, and number of treatment types affected mental and motor development. DESIGN: Descriptive study based on medical record review and pediatric, psychological, and visual evaluations. SETTING: Major referral center for patients with RB and early intervention program in a voluntary urban hospital. SUBJECTS AND METHODS: Fifty-four children younger than 41 months with RB who attend an ophthalmology oncology clinic were recruited for study. Measures included demographic variables such as social class and race/ethnicity, and medical factors such as age at diagnosis (<18 months vs >18 months), type of RB (unilateral or bilateral), family history of RB, and number and types of treatments. All children received a pediatric examination that assessed physical growth and health; a behavioral test of visual acuity using Teller acuity cards; and the Bayley Scales of Infant Development II, a standardized test of mental and motor development. Children found to have delays were referred to intervention services to treat their specific areas of weakness. RESULTS: Three quarters of the children had had 1 eye enucleated; 51 of 54 had normal vision in at least 1 eye, and the other 3 had partial vision in 1 eye. Except for the RB, 46 children were largely normal in growth and health, and 8 had medical diagnoses that were unrelated to RB or its treatment. The average mental and motor development scores were in the normal range (91.4 +/- 16.3, and 91.1 +/- 13.4) and not significantly lower than the normal population. Twenty-six children were referred for early intervention services, and 21 of 26 were referred for services to improve their visuomotor coordination. Demographic variables were not associated with medical variables or outcome. Children with bilateral RB, in which both eyes are affected, performed significantly less well in motor development, received many more types of treatments, and were more likely to be referred for visuomotor therapy than children with unilateral RB. CONCLUSIONS: Children with RB generally function normally in terms of physical health and mental and motor development. However, they are more likely to show delays in visuomotor integration. Early developmental evaluations may improve the visuomotor development of children with visual impairment due to RB.

Analysis of Variance↗
PubMed1993

Mosaic tetrasomy 8p.

We report on a patient with mosaic tetrasomy 8p [46,XX/47,XX+i(8p)]. The patient has 2 fused vertebrae, abnormal ribs, congenital heart defects, agenesis of corpus callosum, hypotonia, and delayed development. The patient's developmental delays are most marked in receptive and expressive language skills, with more moderate delays on cognitive, sensorimotor, and motor skill testing. These findings are similar to those of the 3 previously reported patients with mosaic i(8p).

Abnormalities, Multiple↗
PubMed1986

Natural history of symptomatic partial ornithine transcarbamylase deficiency.

We reviewed the natural history and differential diagnosis of ornithine transcarbamylase deficiency (an X-linked inborn error of urea synthesis) in 13 symptomatic female heterozygotes. The patients presented as early as the first week of life or as late as the sixth year. The most common symptoms before diagnosis were nonspecific: episodic extreme irritability (100 percent), episodic vomiting and lethargy (100 percent), protein avoidance (92 percent), ataxia (77 percent), Stage II coma (46 percent), delayed physical growth (38 percent), developmental delay (38 percent), and seizures (23 percent). Including the proband, 42 percent of the female members of the 13 families studied had symptoms. The median interval between the onset of major symptoms (vomiting and lethargy, seizures, and coma) and diagnosis was 16 months (range, 1 to 142). Five patients had IQ scores below 70 at the time of diagnosis. We suggest that careful evaluation of the family history, the dietary history, the episodic nature of the nonspecific symptoms, the response of these symptoms to the withdrawal of protein, and their frequent onset at the time of weaning from breast milk will permit early diagnosis and might thereby reduce the risk of death or neurologic impairment in female patients with partial ornithine transcarbamylase deficiency.

Amino Acid Metabolism, Inborn Errors↗
PubMed2001

Febrile status epilepticus as the first presentation of cortical developmental malformation: report of 2 cases.

Cortical developmental malformations are an important cause for epilepsy. The epileptic disorder usually begins with sporadic seizures, followed by gradually increasing seizure frequency and culminating in epilepsia partialis continua. We report the de-novo occurrence of focal status epilepticus accompanying hyperpyrexia in two male children, aged 18 months and 4 years. One child had prior neuro-developmental delay, while in the other, developmental milestones were normal. In both instances, there were no seizures prior to the presenting neurological illness. Cerebrospinal fluid examination was normal in both the cases. Magnetic resonance imaging revealed extensive pachygyria in one patient and focal cortical thickening in the other. Therefore, cortical developmental malformation may be suspected as an underlying aetiology in children presenting with de-novo status epilepticus and hyperpyrexia.

Cerebral Cortex↗