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Biomedical subjects

H J Hoffman

Publications and source records attributed to H J Hoffman.

At least 37 records · Page 2Linked to original sources

Relation between very low birth weight and developmental delay among preschool children without disabilities.

The authors examined the relation between very low birth weight (VLBW: < 1,500 g) and possible developmental delay (DELAY) in the absence of frank developmental disability among young children. The prevalence of DELAY in a population-based cohort (Missouri resident births born from December 1989 through March 1991) of singleton VLBW children (n = 367) was compared with the prevalence of DELAY among both moderately low birth weight (MLBW: 1,500-2,499 g; n = 553) and normal birth weight (NBW: > or = 2,500 g; n = 555) singleton control children. DELAY was defined by nine measures of performance on the Denver Developmental Screening Test II at a median adjusted age of 15 months (range: 9-34 months). Subjects were asymptomatic for disabling conditions at developmental follow-up. Apparently well VLBW children were consistently at greater risk for both moderate and severe measures of DELAY and for DELAY across four functional areas than were either the MLBW (adjusted odds ratios: 1.4-2.7) or NBW children (adjusted odds ratios: 2.1-6.3). The greatest prevalence of DELAY tended to be among appropriate-for-gestational age VLBW children who were also the most premature. This study supports developmental follow-up of nondisabled VLBW children because of the significantly elevated risk for DELAY among apparently normal infants.

Alcohol Drinking↗

Sudden infant death syndrome and smoking in the United States and Sweden.

The association between sudden infant death syndrome (SIDS) and maternal smoking was compared between the United States and Sweden-two countries with different health care and social support programs and degrees of sociocultural heterogeneity. For 1990-1991 among the five US race/ethnic groups studied, SIDS rates ranged from a high of 3.0 infant deaths per 1,000 live births for American Indians to a low of 0.8 for Hispanics and Asian and Pacific Islanders. The SIDS rate for Sweden (using 1983-1992 data) was 0.9. The strong association between maternal smoking and SIDS persisted after controlling for maternal age and live birth order. Adjusted odds ratios ranged from 1.6 to 2.5 for mothers who smoked 1-9 cigarettes per day during pregnancy (compared with nonsmokers) and from 2.3 to 3.8 for mothers who smoked 10 or more cigarettes per day during pregnancy. Although birth weight had a strong independent effect on SIDS, the addition of birth weight to the models lowered the odds ratios for maternal smoking only slightly, suggesting that the effect of smoking on SIDS is not mediated through birth weight. SIDS rates increased with the amount smoked for all US race/ethnic groups and for Sweden. Smoking is one of the most important preventable risk factors for SIDS, and smoking prevention/intervention programs have the potential to substantially lower SIDS rates in the United States and Sweden and presumably elsewhere as well.

Adolescent↗

Coexistence of hemimegalencephaly and chronic encephalitis. Detection of cytomegalovirus by the polymerase chain reaction.

We report the extraordinary association of hemimegalencephaly with chronic encephalitis and cytomegalovirus (CMV) positivity in a 5-month-old infant with intractable seizures and a left hemisphere resection. Microscopy revealed a severe neuronal migration disorder (NMD) with fusion of gyri, marked disarray of neuronal lamination, neuronal gigantism and extensive neuronal heterotopias. Also widespread were microglial nodules, gliosis and nodular calcifications and some foci of frank necrosis with calcification. Occasional perivascular and leptomentingeal lymphocytic infiltrates were present. No viral inclusions were identifiable. Polymerase chain reaction on multiple specimens showed unequivocal CMV positivity. In intrauterine CMV infection. NMDs such as polymicrogyria are well recognized, but the association of hemimegalencephaly with CMV infection has not previously been described. Our finding of chronic encephalitis with CMV positivity and hemimegalencephaly in the same patient raises questions about the role of CMV in the etiopathogenesis of the NMD.

Brain↗

Intracranial and spinal metastases from a ganglioglioma with unusual cytogenetic abnormalities in a patient with complex partial seizures.

We describe an unusual clinical presentation of a ganglioglioma in a patient with complex partial seizures. The patient underwent a right temporal lobectomy with subtotal tumor resection at age 15 years, followed by a complete resection 1 year later. Follow-up MRI scan a year later documented recurrence and leptomeningeal dissemination. Another biopsy was performed. Pathological examination revealed similar histology in all three resections, with a ganglioglioma showing no evidence of anaplasia. The tumor exhibited a number of karyotypic abnormalities, notably, a paracentric inversion of chromosome 7. In summary, despite lacking anaplastic features by conventional histological criteria, this ganglioglioma showed an unsusual karyotype and demonstrated radiological evidence of widespread dissemination.

Adolescent↗

Moyamoya disease and syndrome.

Moyamoya disease was first described by Suzuki in 1963. The surgical management of Moyamoya disease began in the mid 1970s. At our institution we began operating on Moyamoya disease in 1979 and between 1979 and 1995 we have treated 30 children with Moyamoya disease and Moyamoya syndrome. Of these 21 children have an excellent outcome. Two children continued to have symptoms and both responded to an encephalo-myo-synangiosis (EMS) procedure, five children had a good outcome but are left with a significant neurologic deficit, one child remains in a poor state and one child had an excellent outcome but then died of his Fanconi's anemia 7 years after his encephalo-duro-arterio-synangiosis (EDAS) procedure.

Adolescent↗

Positive epileptiform discharges in children with neuronal migration disorders.

Most epileptiform abnormalities show a negative polarity on EEG. Focal positive spike waves have rarely been identified in seizure disorders and are generally associated with physiological and neurological impairment. Results of EEG, computed tomography, MRI, and pathologic studies of 15 children with focal neuronal migration disorders who underwent surgery for refractory localization-related epilepsy were compared to examine the association between positive discharges and other findings. Subjects were studied both ictally and interictally by scalp EEG with the International 10-20 system and zygomatic or sphenoidal electrodes, and video EEG telemetry. The 5 children with positive discharges were significantly more likely to develop hemiparesis during the preoperative period (P < or = .025). Correlations were observed between positive discharges and lesions apparent on MRI situated around the rolandic fissure (P < or = .025). Children with positive discharges had a significantly less favorable outcome after surgical treatment (P < or = .025). Positive epilepti-form discharges in children with neuronal migration disorders may signal a more dysfunctional cortex leading to a focal neurological deficit or a more extended lesion than is detected on MRI. This would explain the less favorable outcome of seizures after surgery, since the epileptogenic areas and neuronal migration lesions cannot be completely resected.

Brain↗

Management and outcomes of posterior fossa subdural hematomas in neonates.

OBJECTIVE: To review and analyze a contemporary series of 15 neonates who were treated for posterior fossa subdural hematomas (PFSDHs) during the era of computed tomography and magnetic resonance imaging. METHODS: A retrospective chart review identified all neonates with PFSDHs for whom neurosurgical consultations were obtained for treatment planning. RESULTS: There were nine male and six female patients. The mean gestational age was 39 weeks. Nine of the 15 mothers of the patients were primiparous. Instrument-assisted delivery (forceps and/or vacuum extractor) was undertaken for seven patients. The mean birth weight of the infants was 3165 g (range, 2160-3930 g). The mean 5-minute Apgar score was 7.5. Symptoms of PFSDH developed within the first 24 hours of life in 13 neonates. The predominant symptoms and signs were failure to thrive, irritability, seizures, apnea, and bradycardia. Lumbar punctures to rule out central nervous system sepsis were performed in six neonates. Hemograms revealed that six neonates were anemic with low hemoglobins, five had low platelets, and four had abnormal prothrombin and/or partial thromboplastin times at the time of diagnosis. Computed tomography established the diagnosis of PFSDH in all cases. Magnetic resonance imaging was performed for two neonates. The median time to diagnosis by imaging studies was 10 hours after birth. Surgical evacuation of the PFSDHs was performed in eight neonates. Seven neonates were followed conservatively with serial imaging studies. There was no mortality in either treatment group. Follow-up ranged from 2 to 10 years, with a mean of 4.5 years. Functional outcome assessment revealed that seven neonates were neurodevelopmentally normal, three were mildly delayed, two were moderately delayed, and three were profoundly delayed. In addition to traumatic causes of the PFSDHs, three neonates were observed to have coagulation disturbances at birth and one was observed at follow-up to have a posterior fossa medulloblastoma that had bled at birth. CONCLUSION: PFSDHs are rare but important lesions to diagnose early in the neonatal period. Surgery can be life-saving when performed in a timely manner for signs and symptoms of brain stem dysfunction. A search for an underlying cause predisposing to a PFSDH may, on occasion, reveal a coagulation disturbance or a neoplasm that will require additional therapeutic considerations.

Birth Injuries↗

Optic chiasmatic-hypothalamic glioma.

Optic chiasmatic-hypothalamic gliomas (OCHGs) have been considered benign tumors and self-limiting in growth potential because of their histological appearance. Unfortunately, most clinical series have reported significant morbidity and mortality especially with the more extensive, posteriorly positioned tumors. The biological behavior of OCHGs is age-dependent, with patients younger than five years and older than 20 years typically having tumors that exhibit aggressive growth. There are no specific pathological features to help differentiate the clinical behavior of such tumors. The emergence of modern imaging techniques, including magnetic resonance imaging (MRI), has facilitated the monitoring of the natural history of the disease and the determination of the effects of therapy. Most patients with OCHGs survive for many years. While the natural history of an OCHG for any individual may be indeterminate, enough data are now available from large series to make recommendations for treatment. Our current treatment policy for patients with OCHGs in the context of NF-I without visual failure is a conservative one involving CSF shunting for hydrocephalus if present and medical therapy for endocrinologic dysfunction. Patients with or without NF-I with visual deterioration or progressive neurological deficits and a rapidly expanding suprasellar mass lesion are treated surgically. After tumor resection, patients whose vision is significantly compromised or who show progression of their disease on serial neuroimaging scans receive chemotherapy. If chemotherapy proves ineffective in disease stabilization, then considerations of radiation therapy are given to children over five years old.

Child, Preschool↗

Combined utility of functional MRI, cortical mapping, and frameless stereotaxy in the resection of lesions in eloquent areas of brain in children.

We studied 16 children with lesions in the eloquent brain to determine if the amalgamation of information from functional magnetic resonance imaging (fMRI), frameless stereotaxy, and direct cortical mapping and recording could facilitate the excision of these lesions while minimizing potential neurological deficits. The mean age of the children was 10 years. Fourteen children presented with seizures. All lesions were located in or near eloquent cerebral cortex. fMRI was successful in all patients in delineating the relationship between the lesion and regions of task-activated cortex. The ISG wand was utilized in all cases for scalp and bone flap placement, and for intraoperative localization of the lesion. Direct cortical stimulation or recording of phase reversals with somatosensory evoked potentials helped delineate the central sulcus and language cortex in patients with lesions near the motor or language cortex. Intraoperative electrocorticography (ECoG) was utilized in all patients who presented with seizures to guide the extent of resection of the epileptiform cortex. Ten children had benign cerebral neoplasms, nine of which were totally resected. The other diagnoses included vascular malformations, Sturge-Weber, tuberous sclerosis, Rasmussen's encephalitis, and primitive neuroectodermal tumor. Only 1 patient with a left Rolandic AVM developed a new neurological deficit postoperatively. Thirteen of fourteen patients who presented with seizure disorders were rendered either seizure free or improved in terms of seizure control postoperatively. Follow-up has ranged from 12 to 18 months, with a mean follow-up of 15 months. We conclude that the techniques of fMRI, frameless stereotaxy, direct cortical stimulation and recording can be utilized in sequence to accurately localize intracerebral lesions in eloquent brain, and to reduce the morbidity of resecting these lesions in children.

Adolescent↗

Intrauterine growth pattern by the tendency to repeat small-for-gestational-age births in successive pregnancies.

BACKGROUND: Fetuses of women who repeat small-for-gestational-age births in successive pregnancies may have a different intrauterine growth pattern than SGA birth of non-repeater mothers. Also repeated SGA births may grow differently depending on whether the tendency to repeat is due to some external factors such as cigarette smoking ("false repeaters") or due to genetic or intrinsic factors ("true repeaters"). MATERIAL AND METHODS: Fetal growth were compared in a "nested case-control" study within a longitudinal (cohort) study, comparing three types of SGA births, 23 of "true repeater" mothers, 46 of "false repeater" mothers and 65 of non-repeater mothers, and these were compared with 1017 non-SGA births. Fetal growth was compared using a regression analysis based on repeated measurements (four for each woman). RESULTS: For mean abdominal diameter the "true repeater" SGA births grew more slowly towards the end of pregnancy. However, the growth curves show only minor differences between the three types of SGA births, but the patterns are grossly different from the growth of non-SGA births (controls). CONCLUSION: The intrauterine growth retardation starts early in pregnancy, and is not strikingly different between births of repeater and non-repeater mothers.

Case-Control Studies↗

The relationship between maternal characteristics and fetal and neonatal anthropometric measurements in women delivering at term: a summary.

BACKGROUND: We wanted to determine the relationship between a number of maternal characteristics and various fetal and neonatal anthropometric measurements determined by ultrasound and at birth. METHODS: A total of 1205 term singleton maternal-infant pairs were studied. Various ultrasound measurements obtained at 18, 24, 30 and 36 weeks' gestation and neonatal anthropometric measurements obtained at birth were studied in relationship to various maternal characteristics using univariate and multivariate techniques. RESULTS: Black race, female sex, cigarette smoking, drug use, having a previous low birthweight infant, maternal hypertension and being short or thin or failing to gain weight each resulted in a birthweight decrease of 100 to 300 g. The effect of each of these characteristics on each ultrasound measurement, the timing of the effect, and its ultimate effect on neonatal anthropometric measurements are described. CONCLUSION: The data presented in this paper provide a more complete understanding of the relationship between maternal characteristics, infant sex, and various fetal ultrasound and neonatal measurements.

Alabama↗

Psychosocial factors and small-for-gestational-age infants among parous Scandinavian women.

BACKGROUND: We wanted to analyze the association between small-for-gestational-age (SGA) births, defined as a newborn with a birthweight below the 15th percentile-for-gestational age, and socioeconomic and psychosocial risk factors. METHODS: Information on social background, psychological status, and life events was collected prospectively by use of questionnaires in the second and third trimester of pregnancy. The respondents were 1552 women who expected their second or third child and took part in a Scandinavian multicenter study of fetal growth and perinatal outcome. RESULTS: No significant differences were found in relational stress, state and trait anxiety, depression, and physical strain between SGA and non-SGA births, whereas smoking around time of conception and low prepregnant body mass were significant SGA birth predictors. Maternal and paternal education of nine years or less increased the SGA birth risk (RR 1.46 (95% CL 1.12; 1.92) and RR 1.34 (95% CL 1.01; 1.79), respectively. The increased risk from a low maternal education was still significant when body mass and low paternal education were controlled, but not after adjustment for maternal smoking. A protective effect of paternal, but not maternal, education of 12 years or more was also observed and retained its effect when maternal smoking and body mass were controlled. CONCLUSION: In this seemingly homogeneous Scandinavian population, parental education and maternal body proportion and life style influenced the prevalence of small-for-gestational-age births. Relational stress, anxiety, depression, and physical strain did not influence birth outcome.

Educational Status↗

Abbreviated scale for the assessment of psychosocial status in pregnancy: development and evaluation.

BACKGROUND: Data from five existing psychosocial scales were used to develop an abbreviated scale for the assessment of psychosocial status during pregnancy. METHODS: Scales were self-administered by 842 black and 381 white low-income multiparous women at risk for poor pregnancy outcome. Trait anxiety (Speilberger), self-esteem (Rosenberg), mastery (Pearlin), and depression (CES-D) were assessed at 24-26 weeks' gestation; subjective stress (Schar) was assessed at 30-32 weeks' gestation. The 59 pooled items were examined for redundancy and the discernment of primary factors using principal factor analysis. Regression analysis was used to determine if the resulting abbreviated scale (28 items) would provide information similar to that obtained with the 59 item pool (full scale) in predicting gestational age (GA), birth weight (BW), fetal growth restriction (FGR), and preterm delivery (PTD). RESULTS: The abbreviated scale was highly correlated (r = 0.97) with the 59-item pool and the six factors isolated were generally compatible with the major characteristics assessed by the five original scales. The distribution of FGR and PTD by scale quartile was similar for the abbreviated and the combined scales. Logistic regression analysis of scores for all women revealed that poor (high) scores on both the full (p = 0.0151) and the abbreviated scales (p = 0.0131) were positively associated with FGR, but not with PTD. In linear regression analysis poor (high) scores on both the full (p = 0.0024) and the abbreviated scale (p = 0.0019) were negatively related to BW, but not to GA. When data for black and white women were examined separately, the two scales provided comparable information. CONCLUSIONS: The abbreviated psychosocial scale provided information similar to that obtained with 59 pooled items in predicting GA, BW, FGR, and PTD.

Adult↗

Prediction of fetal growth based on maternal serum concentrations of human chorionic gonadotropin, human placental lactogen and estriol.

BACKGROUND: The purpose was to determine the usefulness of maternal serum concentrations of human chorionic gonadotropin (hCG), human placental lactogen (hPL) and estriol as predictors of fetal growth. METHOD: From a large cohort serum obtained serially at 17, 25, 33 and 37 weeks of gestation were analyzed for randomly selected pregnancies resulting in small for gestational age (SGA, n = 102) and non-SGA (n = 112) infants. RESULTS: There were no significant correlations between birthweight ratio (ratio of birthweight to mean weight for gestational age) and hCG, but between birthweight ratio on one hand and estriol for all stages of pregnancy (r = 0.19-0.38, p < 0.01 - p < 0.001) and hCL except at 33 weeks (r = 0.11-0.40, p ns-p < 0.001) on the other. There were statistically significant, but small median differences and substantial overlaps between the SGA and non-SGA infants for hCG at 17 and 37 weeks, for hPL at 17, 33 and 37 weeks, and for estriol at all the stages of pregnancy. The sensitivity and positive predictive value of low hormone concentrations (below the 10th percentile) in predicting the birth of an SGA infant were in the range of 6-26% and 17-39%, respectively. The corresponding specificity and prediction of a non-SGA infant from normal levels were 91-93% and 85-88%. CONCLUSIONS: HPL and estriol, but not hCG concentrations, are positively related to the size of the fetus, but the relationships are too weak to be of predictive value in an unselected population.

Adult↗