Brain tumors with symptomatic onset in the first two years of life

Eighty children who in the first 2 years of life had signs and symptoms relating to a cerebral neoplasm were studied over an 18-year period (1970-1987), the mean follow-up being 8.2 years. In each case age at onset, clinical presentation, tumor location and pathological diagnosis, extent of surgical resection, postoperative mortality, adjuvant therapy length of survival and quality of life were assessed. Supratentorial tumors (59%) were more common than infratentorial. The most frequent clinical presenting feature (70%) was increased intracranial pressure. Sixty-three patients (79%) were operated on and in all of these cases a histological diagnosis was obtained. Astrocytomas (41%) and medulloblastomas (20%) were the most common oncotypes. Surgical mortality was 17.4% and the 5-year survival rate was 54%. Quality of life was assessed for all long-term surviving patients using a specifically designed protocol. Normal physical and intellectual performances were found in 46% of cases, and all together 75% of the patients had sufficient autonomy in daily life. The prognosis is more closely related to tumor location and type of treatment than to histological diagnosis or age at onset.     

Causes of sudden unexpected cardiac death in the first two decades of life

Sudden, unexpected cardiac death in the age group 1 to 21 years usually is due to myocarditis, hypertrophic cardiomyopathy, aortic valvar stenosis, and coronary arterial abnormalities. The hearts of 70 patients <21 years of age who died suddenly were reviewed. Twenty patients were <1 year of age and 50 were 1 to 21 years old. The cardiac findings were compared with those in 68 age-matched controls with known cardiac disease who did not die suddenly. Significant cardiac abnormalities were present in 13 (65%) of the 20 infants; 10 (50%) had anomalies of the aortic origin of the coronary arteries. Among the 50 older patients, cardiac abnormalities were found in 40 (80%), among whom coronary arterial anomalies existed in 12 (24%). Anomalies of aortic origin more frequently involved the left main than the right coronary artery in both groups.     

Sleep ontogenesis revisited: a longitudinal 24-hour home polygraphic study on 15 normal infants during the first two years of life

In order to investigate the genetic basis of susceptibility to Henoch-Schoenlein purpura (HS), blood samples of 152 patients, 105 of whom had renal disease, were collected in a two-step study. The evaluation of DRB, DQB and DQA polymorphism was done by analysis of the restriction polymorphisms produced by TaqI enzyme. DRB1*07 was less frequent in patients than in the control group (gene frequency 0.09 and 0.18, respectively; P = 0.0023), whereas 64% of the patients were positive for DRB1*01 and/or DRB1*11 compared with 48% of the control group (P = 0.0069). Polymerase chain reaction-sequence-specific oligonucleotide (PCR-SSO) typing of DRB1*01- and DRB1*11-positive individuals did not show any deviation of frequencies of DRB1*01 subtypes between patients and controls, whereas among DRB1*11 subtypes DRB1*1104 was significantly increased in the patients (Pc = 0.033). The comparison between patients with renal disease and those without renal disease showed no significant differences in the frequency of the single DRB, DQB and DQA alleles. The study of restriction polymorphisms in the switch region of the constant genes alpha 1, alpha 2 and mu of the heavy chains of immunoglobulins, using the enzyme Sacl and a specific probe, did not show any difference between 44 patients and 54 controls. This study demonstrates that susceptibility to HS also has a genetic origin: on one hand, the presence of DRB1*01 or DRB1*11 makes disease onset easier; on the other hand, DRB1*07 could induce some resistance to the disease. It is suggested that, as well as for other diseases caused by an impaired immune response, single amino acids in a key position in the HLA-DRB molecule make it more or less easy to recognize some antigenic peptide, towards which an immune response leading to disease is triggered.     

Epilepsy surgery in the first three years of life

PURPOSE: Partial seizures in early postnatal life may be catastrophic and associated with poor long-term outcome. Epilepsy surgery can alleviate partial seizures in older children and adults, but there is little experience with surgical therapy in infancy apart from hemispheric epilepsy syndromes. METHODS: We analyzed the results of cortical resection to treat medically refractory partial epilepsy in 31 children (16 boys, 15 girls) aged <3 years (mean, 18.3 months). Subjects were included only if seizure relief was the primary indication for surgery. RESULTS: Follow-up of at least 1 year (mean, 4.6 years) in 26 patients revealed that 16 were seizure-free, 4 had >90% seizure reduction, and 6 had <90% reduction. There was no significant difference in seizure outcome between hemispherectomy/multilobar resections and lobar resections or temporal versus extratemporal resection. Seizure outcome was independent of the amount of cortex removed in nonlesional patients. Only the presence of a discrete lesion on preoperative neuroimaging correlated with a favorable outcome. Family perceptions of accelerated development in seizure-free patients were not confirmed on developmental assessment. CONCLUSIONS: We conclude that cortical resection often benefits very young children with catastrophic partial seizures, but does not guarantee enhanced neurological development. The location and extent of the excised cortex may not be critical as long as the entire epileptogenic region and lesion are removed.     

Water supplementation of infants in the first month of life

OBJECTIVE: To describe the prevalence of and risk factors associated with regular water supplementation of neonates. DESIGN: Evaluation of data from the Food and Drug Administration's Infant Feeding Practices Study, a panel study of US women of fairly high socioeconomic status who were followed up from late pregnancy through their infants' first year of life. The sample was drawn from a nationally distributed consumer mail panel. Each mother was asked whether she gave her neonate water at least 3 times per week. PARTICIPANTS: A total of 1677 mothers of infants who were neonates in April through November 1993. MAIN OUTCOME MEASURES: Percentages of mothers who gave their neonates water at least 3 times a week, considering infant feeding status, mother's education, and family income. RESULTS: About one fourth (24.7%) of the mothers reported giving their neonates water at least 3 times per week. Stratification by feeding practices and socioeconomic factors revealed that 41.6% of mothers who formula-fed their neonates, 47.4% of mothers with less than a high school education, and 35.4% of mothers with an annual family income less than $22,500 gave their neonates water at least 3 times per week. CONCLUSIONS: Water supplementation of neonates was a prevalent practice in this cohort of women. Feeding practices, maternal education, and family income were all significant risk factors associated with this behavior.     

Repair of ventricular septal defect in the first two years of life using profound hypothermia-circulatory arrest techniques

Ventricular septal defect repair had been performed in 57 infants ages 21 days to 21 months and under 10 kg in weight using profound hypothermia-circulatory arrest technics. Severe congestive heart failure was the indication for operation in all but two infants under 6 months of age, and in those under 3 months there was usually an associated moderate or large sized atrial septal defect or patent ductus arteriosus or a coarctation. In infants over 6 months controlled heart failure was accompanied by failure to thrive and often recurrent respiratory infections. The main indication for surgery in three infants was repeated severe respiratory infections and in 7 infants, ages 10-15 months, an elevation of pulmonary vascular resistance of 6 units M2 or more. There were two hospital deaths among the 49 infants without coarctation (ages 6 and 20 months) and two among the 8 with coarctation. Postoperative respiratory and other complications were uncommon. On late review there was no significant residual VSD amongst the 11 recatheterized patients. Psychometric studies in 19 children who had reached the age of three to four years gave no evidence of cerebral damage due to the circulatory arrest period. In view of these results palliative pulmonary artery banding is no longer performed for VSD in infancy unless there is a Swiss cheese septum or an associated severe coarctation.     

Cardiac valve replacement in the first 21 years of life

From 1969 through 1974, 25 patients under age 21 years had prosthetic cardiac vlave replacement at the Emory University Hospital. Twenty-nine valves were replaced in this group. Patients ranged in age from 2 to 21 years, with a mean of 14 years. Concomitant correction of associated cardiac lesions was required in 14 (56%). Five (21%) had had prior open valvuoplastic procedures. Operative mortality for the entire group was 12%, with an additional late mortality of 8%. There have been no early or late deaths in the last three years' experience. Best results are obtained when early correction of isolated valvular lesions is done as soon as clinical deterioration on vigorous medical management is recognized. The presence of multiple intrcardiac lesions or prior valve surgery does not preclude excellent results. Current indications for surgical correction and follow-up management are presented.     

Blood pressure levels of preterm infants in the first year of life

Systolic blood pressure was measured in 36 infants with median gestational age of 29 weeks (range 24-35 weeks) and birth weight of 1160 g (range 642-1500 g). Measurements were made at 1, 6 and 12 weeks, and subsequently at 12-weekly intervals during the first year of life. No infant developed chronic lung disease. Systolic blood pressure increased over the first 24 weeks (p < 0.001) to a mean value of 95 mmHg, but did not change significantly over the next 24 weeks. These data provide a reference range of blood pressure levels during the first year of life for infants born at an early gestational age.     

Intracranial meningiomas of the first two decades of life

Meningiomas arising in the first two decades of life are uncommon and their characteristics are controversial. Some authors believe meningiomas in younger patients occur in different locations, have more malignant histological features, and have a worse prognosis than those in adults. To address this controversy, the authors retrospectively reviewed 23 cases of meningiomas in patients under 21 years of age at diagnosis who were operated on at the University of Turin (1948 to 1990) or at the University of California, San Francisco (1970 to 1989). These tumors represented 2.9% of all tumors in this age group and 1.8% of all meningiomas during the study period at the two institutions. There were 14 males and nine females. The mean age at surgery was 13.3 +/- 5.6 years; nine cases occurred in the first decade and 14 in the second. The most common neurological symptoms were a focal neurological deficit (33%) and seizures (25%). Seventy percent of the tumors were supratentorial. A gross total resection was performed in 60% of the cases. Histologically, the majority (74%) of the tumors were meningothelial or mixed. An increased number of mitoses was observed in 33% of the tumors, focal necrosis in 29%, and invasion of adjacent brain in 14%; however, none of the tumors was classified as a Grade III (anaplastic) meningioma. All patients are alive without evidence of recurrent disease 3 to 22 years (mean +/- standard deviation: 10 +/- 7.3 years) after surgery. This study confirms the rarity of meningiomas of the first two decades of life and the absence of the female predominance associated with meningiomas in adults. The location and histological features of these tumors are similar to those in adults; they have a low recurrence rate, and the outcome and survival rate are excellent.     

Epidemiological features of brain tumors in the first 3 years of life

In contrast to CT and MRI, conventional B-scan echography is quick and easy to perform, cheap, and without side effects. The disadvantage is the limited field of view. This may result in a loss of information between the dynamic examination and the documentation. With SieScape a new technology was developed to address these problems. It allows one to obtain any imaging slices from the head and the neck. This technique is the first to allow a topographical orientation based on B-scan images. This study presents the application of SieScape, and demonstrates normal and abnormal findings. Initial experience with the new technology indicates that SieScape is an alternative to other methods such as CT and MRI.     

Development of feeding practices during the first 5 years of life

OBJECTIVE: To understand the transition from breast-and bottle-feeding to solid-feeding and factors that might affect the duration of breast- and bottle-feeding. DESIGN: Cohort followed up from birth with relatively well-educated, middle-class parents. SETTING: Community sample recruited from 3 suburban newborn nurseries (a teaching hospital, community hospital, and large health maintenance organization). PARTICIPANTS: One hundred ninety-one healthy full-term infants. MEASURES: Assessment of feeding practices through the ages of complete weaning from breast- and bottle-feeding. RESULTS: More than 90% of participants breast-fed for at least 2 weeks. Infants of older mothers were weaned from the breast later than infants of younger mothers. First-born infants were weaned from the breast earlier than later-born infants. Eighty-four percent of infants bottle-fed at some time during the first year of life. More than 40% of the cohort was still receiving bottles at 24 months of age, 16% at 36 months, and 8% at 48 months. The duration of breast- and bottle-feeding was related to maternal work status; mothers who returned to work during the first 3 months postpartum weaned sooner from the breast and later from the bottle than women who returned to work after 3 months postpartum. CONCLUSIONS: The frequency of late bottle-weaning in this well-educated, middle-class cohort was unexpected and was related to the timing of the mother's return to work. The impact of prolonged bottle-feeding on later growth and adiposity deserves further investigation.     

Morbidity during the first year of life in small for gestational age infants

OBJECTIVE: To study the effect of early postnatal dexamethasone (days 1-3) on the incidence and severity of chronic lung disease in preterm infants with respiratory distress syndrome. METHODS: A multicentre, randomised, placebo controlled, blinded study was carried out in 18 neonatal intensive care units in Israel. The primary outcome measure was survival to discharge without requirement for supplemental oxygen therapy beyond 28 days of life. The secondary outcome measures were requirement for mechanical ventilation at 3 and 7 days, duration of ventilation or oxygen therapy, need for subsequent steroids for established chronic lung disease and incidence of major morbidities. RESULTS: The study consisted of 248 infants (dexamethasone n = 132; placebo n = 116). No differences were found in the outcome variables except for a reduction in requirement for mechanical ventilation at age 3 days in treated infants (dexamethasone 44%, placebo 67%; P = 0.001). Gastrointestinal haemorrhage, hypertension, and hyperglycaemia were more common in treated infants, but no life threatening complications, such as gastrointestinal perforation, were encountered. CONCLUSIONS: These data do no support the routine use of early postnatal steroids, but may justify further study in a selected, high risk group of infants.     

Vitamin E and C levels in infants during the first year of life

The considerably lower vitamin E level found in cord blood and in newborns at birth than those found in the venous blood of mothers at delivery are not yet fully explained. In a group of 217 not selected newborns, we attempted to establish the relation between vitamin E and C levels at delivery and the changes during the first year of life. The mean serum vitamin E level rose from 0.37 mg/ml at 3 days to 0.80 mg/100 ml at 6 months and to 0.72 mg/100 ml at 12 months. On the other hand vitamin C mean levels lowered from 0.93 mg/100 ml in cord blood to 0.77 mg/100 ml at 6 months and to 0.73 mg/100 ml at 12 months. The rise of vitamin E values could be explained by the early use of infant solid foods with high vitamin and mineral content and by the increase of serum lipoproteins. Except at 3 days after delivery there were no individual values of serum vitamin E below the acceptable 0.35 mg/100 ml limit. However, serum vitamin C levels compatible with a moderate risk were very often observed, i.e., in 27.1% of infants at 6 months and in 30.5% at 1 year. Thus, vitamin E intake in infants was satisfactory with the usual diet but not vitamin C for which blood levels were not adequate. In view of these findings it appears necessary to evaluate periodically the vitamin E as well as vitamin C status in the infant population.     

Development of attention and distractibility in the first 4 years of life.

This observational study describes the early development of attention and distractibility. Under several conditions of distraction, 172 children at 10, 26, and 42 months of age played with toys. Attention to the toys was coded as casual, settled, or focused. All 3 levels of attention changed with age, with casual attention decreasing and focused attention increasing. The 10-month-olds were more distractible than the other children, even during focused attention. The infants were most distracted by the auditory-visual distractor, whereas the oldest children were most distracted by the visual distractor. Some 42-month-olds showed evidence of being more focused in the presence of distractors. Overall, the results point to a developmental transition in the processes underlying attention during play. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Neurodevelopmental outcome of infants with viral meningitis in the first three months of life

We prospectively evaluated the neurodevelopmental outcome of infants with documented viral meningitis to determine (1) whether deficits in physical growth, development, speech and language, hearing, or intelligence occur; and (2) if so, at what age these deficits can be detected. Sixteen infants with documented enteroviral meningitis under the age of 90 days and a control group of 13 patients matched for age, race, sex, and socioeconomic status were followed up prospectively for 3 years with annual evaluations, which included a developmental evaluation by a pediatric developmentalist, articulation and language tests by a speech-language pathologist (Sequenced Inventory of Communication Development, Receptive-Expressive Emergent Language Scale (REEL), Preschool Language Scale (PLS), Revised Peabody Picture Vocabulary Test (PPVT-R), Photo Articulation Test, audiometric screening), and intelligence tests by a psychometrist (Bayley Scales of Infant Development [BSID] and Stanford-Binet). No deficits were demonstrated in growth, development, hearing, BSID, articulation, and expressive language. Subtle but significant (P < 0.05) deficits were documented in the study group compared with the control group in the receptive component of the REEL, all subsections of the PLS, the PPVT-R, and the verbal comprehension/language-processing section (Factor II) of the Stanford-Binet. These differences could be reliably detected by 3 years of age. We conclude that viral meningitis in young infants may cause subtle deficits in language skills, particularly receptive language. We recommend that children who have had enteroviral meningitis during early infancy be monitored carefully for language development and, perhaps, receive increased language stimulation in the home prior to school entry in order to optimize their learning potential.     

Trajectories of life satisfaction in the first 5 years following traumatic brain injury.

Objectives: The trajectories of life satisfaction for 609 individuals who sustained a traumatic brain injury (TBI) were studied. Hierarchical linear modeling analysis examined individual level growth trends over the first 5 years following TBI using gender, functional independence, age, and time to estimate life satisfaction trajectories. Measures: Participants completed the Functional Independence Measure and the Life Satisfaction Inventory at years 1, 2, 4, and 5 after sustaining TBI. Results: Participants who reported higher functional independence at year 1 also had higher life satisfaction at year 1. Participants with lower functional independence across the 5-year period had life satisfaction trajectories that decreased at significantly greater rates than the individuals with more functional independence. The life satisfaction trajectory declined for the sample, but participants reporting lower cognitive and motor functional independence had significantly greater declines in life satisfaction trajectories. Age and gender were not significant factors in predicting life satisfaction trajectories following TBI. Implications: Individuals with greater cognitive and motor impairments following TBI are likely to experience significant declines in life satisfaction within 5 years of living with TBI. (PsycINFO Database Record (c) 2010 APA, all rights reserved)     

Systematic analysis of in-patients' circumstances and causes of death: a tool to improve quality of care

OBJECTIVES: To point out actions improving quality of care from a systematic analysis of causes and circumstances of death of hospital patients. DESIGN: All in-hospital patient deaths during 1993 and 1994. SETTING: Saint Luc University hospital. MAIN OUTCOME MEASURES: Immediate and original causes of death, other diagnoses or procedures, circumstances of death, place, members of hospital staff, family or friends with the patient, mention of "do not resuscitate", of unexpected death, of problems during the process of care and of autopsy performed. RESULTS: The mean age of the patients was 60 years. The proportion of men was 57%. The majority of patients died in their room. No family or friends, or members of hospital staff were observed for 17%. "Do no resuscitate" was mentioned for 38% of the patients and the proportion of unexpected death and autopsy was 15% and 28% respectively. The most frequent causes of death were malignant and cardiovascular diseases. At least one of the following diseases was mentioned on 14% of the forms; complications during care, septicaemia, pulmonary embolism, adverse effects of drugs or pressure ulcer. Mean length of hospital stay was 14.7 days. CONCLUSIONS: A systematic analysis of the causes and circumstances of death reveals the epidemiology of hospital deaths and highlights specific diseases, complications or circumstances that could be changed. This study creates the opportunity for possible further action, such as improvement in prevention of pulmonary embolism and support of the members of staff or the friends and family of the patient at the time of death.