Biomedical subjects

J F Bale

Publications and source records attributed to J F Bale.

At least 19 recordsLinked to original sources

PubMed2001

Early predictors of neurodevelopmental outcome in symptomatic congenital cytomegalovirus infection.

OBJECTIVE: To determine the ability of neonatal clinical, audiologic, and computed tomography (CT) findings to predict long-term neurodevelopmental outcome in children with symptomatic congenital cytomegalovirus (CMV) infection. METHODS: Longitudinal cohort study of children (n = 41) with symptomatic congenital CMV infection evaluated at birth and followed up with serial age-appropriate neurodevelopmental testing. The performance of birth characteristics as predictors of long-term outcome were determined, and clinical and CT scoring systems were developed and correlated with intellectual outcome. RESULTS: Microcephaly was the most specific predictor of mental retardation (100%; 95% CI 84.5-100) and major motor disability (92.3%; 95% CI 74.8-99). An abnormality detected by CT was the most sensitive predictor for mental retardation (100%; 95% CI 82.3-100) and motor disability (100%; 95% CI 78.2-100). A highly significant (P <.001) positive correlation was found between head size at birth and the intelligence/developmental quotient (IQ/DQ). Approximately 29% of children had an IQ/DQ >90. There was no association between sensorineural hearing loss at birth and cognitive outcome. However, children with sensorineural hearing loss on follow-up (congenital and late-onset) had a lower IQ/DQ (P =.006) than those with normal hearing. CONCLUSIONS: The presence of microcephaly at birth was the most specific predictor of poor cognitive outcome in children with symptomatic congenital CMV infection, whereas children with normal findings on head CT and head circumference proportional to weight exhibited a good cognitive outcome.

Cerebral Palsy↗

PubMed2000

Intrauterine cytomegalovirus infection and glycoprotein B genotypes.

Cytomegalovirus (CMV) strains display polymorphisms for the gene encoding glycoprotein B (gB; gpUL55). Recent data suggest that the gB genotype may influence the outcome of acquired CMV infections. To determine whether the gB genotype also contributes to the outcome of intrauterine infection, CMV strains were studied from 56 infants with culture-confirmed intrauterine CMV infections who were born in Iowa or Texas. CMV gB genotypes were compared with the neonatal clinical features and neurodevelopmental outcomes. Fifty-three strains (95%) could be assigned a gB genotype. The overall distribution of genotypes was as follows: type 1, 50%; type 2, 18%; type 3, 23%; and type 4, 4%. Strains with the gB 3 genotype were more common among the Iowa infants (P=.082). The gB 3 genotype was more common among infants with asymptomatic infections (P=.004), but geographic location and ascertainment biases may have accounted for these differences. The gB genotypes did not correlate with the neurodevelopmental outcome of intrauterine infection.

Cytomegalovirus↗

PubMed2000

Paroxysmal kinesigenic dyskinesia and infantile convulsions: clinical and linkage studies.

OBJECTIVE: To clinically characterize affected individuals in families with paroxysmal kinesigenic dyskinesia (PKD), examine the association with infantile convulsions, and confirm linkage to a pericentromeric chromosome 16 locus. BACKGROUND: PKD is characterized by frequent, recurrent attacks of involuntary movement or posturing in response to sudden movement, stress, or excitement. Recently, an autosomal dominant PKD locus on chromosome 16 was identified. METHODS: The authors studied 11 previously unreported families of diverse ethnic background with PKD with or without infantile convulsions and performed linkage analysis with markers spanning the chromosome 16 locus. Detailed clinical questionnaires and interviews were conducted with affected and unaffected family members. RESULTS: Clinical characterization and sampling of 95 individuals in 11 families revealed 44 individuals with paroxysmal dyskinesia, infantile convulsions, or both. Infantile convulsions were surprisingly common, occurring in 9 of 11 families. In only two individuals did generalized seizures occur in later childhood or adulthood. The authors defined a 26-cM region using linkage data in 11 families (maximum lod score 6.63 at theta = 0). Affected individuals in one family showed no evidence for a shared haplotype in this region, implying locus heterogeneity. CONCLUSIONS: Identification and characterization of the PKD/infantile convulsions gene will provide new insight into the pathophysiology of this disorder, which spans the phenotypic spectrum between epilepsy and movement disorder.

Adolescent↗

PubMed2000

Cysticercosis.

Cysticercosis, the consequence of ingesting viable eggs of the porcine tapeworm Taenia solium, currently remains one of the most common human parasitic conditions worldwide. Although preventable by the proper disposal of human wastes, cysticercosis of the central nervous system (neurocysticercosis) accounts for a substantial proportion of cases of epilepsy and hydrocephalus among children and adults in many developing countries. Cases also occur in nonendemic regions, reflecting patterns of immigration from highly endemic countries, especially Mexico and other areas of Latin America. Antiparasitic treatment during active infections, using albendazole or praziquantel, can eradicate the parasite, may lower the risk of late complications, and potentially reduces the morbidity of acute disease. Considerable controversy persists regarding the role of antiparasitic therapy in neurocysticercosis, however. Persons with active parenchymal or extraparenchymal disease, defined by the neuroradiographic appearance of lesions, can be treated with albendazole, 15 mg/kg/d divided into two daily doses for 8 days. Patients with parenchymal disease who do not respond to albendazole can receive a second course of albendazole or praziquantel, 50 mg/kg/d divided into three daily doses for 15 days. Concurrent administration of dexamethasone in standard doses is usually required during the first several days of antiparasitic therapy to minimize the inflammation and cerebral edema associated with death of the parasites. Patients with intraventricular cysts and hydrocephalus require shunting and surgical removal of cysticerci. By contrast, persons with inactive lesions and seizures as a consequence of remote infections typically require only symptomatic therapy with standard anticonvulsants.

Journal Article↗

PubMed1999

Cytomegalovirus transmission in child care homes.

BACKGROUND: Children attending child care centers have high rates of cytomegalovirus (CMV) excretion. Women exposed to such children have an increased risk of acquiring CMV infection, and primary infection places the offspring of such women at risk of congenital CMV infection. We studied family child care homes to determine if this child care alternative might represent a safe haven from CMV. METHODS: One hundred thirty-two women providing care in their homes were studied using a latex agglutination method to determine the rate of CMV seropositivity at baseline. Women who were seronegative for CMV were then sampled prospectively at 6-month intervals between March 1991 and August 1994 to determine the annual rate of CMV acquisition. A point prevalence of CMV excretion in family homes was determined by sampling 106 children from 25 randomly selected homes. Cytomegalovirus isolates were compared by molecular analysis using polymerase chain reaction-based methods to identify transmission. RESULTS: At baseline, 57.6% of the 132 providers were seropositive for CMV. Seropositive providers were more likely to be caring for toddlers (aged 1-2 years) (67% vs 46%; P=.02) and had worked in child care somewhat longer (median of 28.5 vs 21.5 months; P=.11). Using stepwise logistic regression, the strongest predictors of seropositivity at baseline were caring for children aged 1 to 2 years (odds ratio [OR] =2.37; P=.02) and number of months as a child care provider (OR= 1.17 for an increase of 24 months as provider; P=.08). Six or more years as a provider was highly associated with seropositivity (OR=3.27; P=.02). During follow-up, 5 of 51 seronegative providers seroconverted, yielding an annual infection rate of 6.8%. The point prevalence survey of children from the 25 homes (14 had seropositive providers) identified 8 CMV-excreting children. Three children in 1 home had indistinguishable isolates by polymerase chain reaction mapping. The provider seroconverted and excreted an isolate with a molecular profile indistinguishable from that of the children. CONCLUSIONS: The prevalence of CMV excretion is low among children attending child care homes (8% vs 15% in prior studies of child care centers; P=.07), and only 1 (20%) in 5 of the homes had CMV-excreting children. However, the overall CMV seroconversion rate of home child care providers was comparable to the rate observed among providers in child care centers. Families who use family home child care as an alternative to large child care centers are exposed to a low and unpredictable risk of CMV infection.

Adult↗

PubMed1999

Effect of long-term valproic acid administration on the efficiency of carnitine reabsorption in humans.

To elucidate the etiology of valproic acid-induced carnitine deficiency, we tested the hypothesis that long-term valproic acid administration decreases the rate of carnitine reabsorption. Thirteen healthy men participated in a 34-day protocol in which carnitine clearance was measured before and after 28 days of valproic acid administration. During valproic acid administration (days 6 to 33), plasma free and total carnitine concentrations decreased (18% and 12%, respectively, P<.05) by 16 days, but returned to pretreatment concentrations by 28 days. From day 14 to day 30, the rate of free carnitine excretion was 50% lower than at baseline (day 4, P<.05). Free and total carnitine clearance, indexed to the glomerular filtration rate, was lower after valproic acid administration (P<.01). Contrary to our hypothesis, after 28 days of valproic acid administration, the rate of carnitine reabsorption was enhanced independent of the glomerular filtration rate and filtered load. Changes in the plasma concentration, rate of excretion, and clearance were specific for carnitine and were not generalized in magnitude or direction to the other amino acids. We conclude that the kidney adapts to conserve carnitine during valproic acid administration and therefore does not cause valproic acid-induced carnitine depletion in adults.

Absorption↗

PubMed1999

Bilateral hippocampal encephalitis caused by enteroviral infection.

Nonpolio enteroviral encephalitis usually presents as a diffuse, generalized encephalitis. Focal cerebral involvement by nonpolioviruses is uncommon, and neuroradiologic studies in these cases are usually normal. The authors present a case of a 5-year-old male with an acute encephalitic illness and bilateral lesions of the hippocampi on magnetic resonance imaging. Enteroviral nucleic acids were detected in the cerebrospinal fluid by the reverse transcription polymerase chain reaction. The findings suggest that enteroviral infection should be considered in the differential diagnosis of acute bilateral hippocampal encephalitis in patients in whom polymerase chain reaction fails to demonstrate the presence of herpes simplex virus.

Child, Preschool↗

PubMed1999

Human herpesviruses and neurological disorders of childhood.

The human herpesviruses, a group of DNA viruses that includes herpes simplex viruses types 1 and 2, varicella zoster virus, cytomegalovirus, Epstein-Barr virus, and human herpes viruses types 6, 7, and 8, cause substantial neurological morbidity among infants and children. This review describes the biology of these viruses and summarizes the clinical manifestations and therapy of the diseases that result from childhood infection with these important pathogens.

Central Nervous System Viral Diseases↗

PubMed1998

Epidemiology of congenital cytomegalovirus infection: maternal risk factors and molecular analysis of cytomegalovirus strains.

To determine factors that influence the occurrence of congenital cytomegalovirus (CMV) infection, the authors surveyed prospectively 8,254 infants born in eastern Iowa between October 1989 and June 1994. The authors conducted a case-control study to identify maternal risk factors, matching each CMV-infected infant with three uninfected infants according to hospital and date of birth. CMV strains were compared by using the polymerase chain reaction (PCR) to identify common sources of infection. Of the 7,229 infants cultured successfully for CMV, 35 (0.48%) were congenitally infected. Mothers of CMV-infected infants were more likely to be single (odds ratio (OR) = 3.05, p = 0.016), to work in sales (OR = 4.93, p = 0.008), or to be students (OR = 5.01, p = 0.017). Conversely, women who worked in health-care professions were less likely to have a congenitally infected infant (OR = 0.14, p = 0.049). PCR analysis indicated 27 distinct strains of CMV, but two groups of infants (two infants per group) excreted strains with indistinguishable molecular patterns. One of these pairs of infants had older siblings who attended the same child-care center during their mothers' pregnancies. The authors concluded that demographic and occupational factors influenced the risk of giving birth to an infant with congenital CMV infection. Many distinct CMV strains were identified, suggesting that major point source outbreaks had not occurred. Nonetheless, point source acquisition of CMV from child-care environments did account for some cases of congenital CMV infection in eastern Iowa.

Adult↗

PubMed1998

Hemophilia morbidity, cognitive functioning, and academic achievement.

Data from the Hemophilia Growth and Development Study (HGDS) were used to evaluate the association between hemophilia morbidity, measured by abnormalities in coordination and gait (CG), and intellectual ability and academic achievement. The CG abnormalities observed in the HGDS participants (n = 333) were primarily due to hemophilia-related morbidity. Although HGDS participants performed within the average range for age on measures of intellectual ability, there were meaningful differences between CG outcomes at baseline and throughout the 4 years of study. Participants without CG abnormalities consistently achieved higher scores than those with CG abnormalities on Reading, Spelling, and Arithmetic subtests of the Wide Range Achievement Test-Revised. Our findings suggest that lowered achievement is related to the functional severity of hemophilia.

Adolescent↗

PubMed1998

Hemophilia growth and development study: relationships between neuropsychological, neurological, and MRI findings at baseline.

OBJECTIVE: To determine the effects of human immunodeficiency virus (HIV) infection on children's development by identifying neurological and environmental variables associated with neuropsychological measures of cognitive development in HIV-seronegative (HIV-) and HIV-seropositive (HIV+)children and adolescents with hemophilia. METHODS: Participants (N = 298; 60% HIV+) were males ages 7-19 years enrolled in the Hemophilia Growth and Development Study (HGDS). Least squares modeling was used to determine whether there was a difference at baseline in mean neuropsychological test scores by HIV status, age, and neurological baseline findings, adjusting for selected environmental and medical history variables. RESULTS: The participants were within age expectations for general intelligence. Variables associated with lowered neuropsychological performance included academic problems, coordination and/or gait abnormalities, parents' education, and previous head trauma. CONCLUSIONS: Hemophilia-related morbidity has a subtle adverse influence on cognitive performance. HIV infection was not associated with neuropsychological dysfunction in this group even when MRI abnormalities were present.

Adolescent↗

PubMed1997

Congenital lymphocytic choriomeningitis virus syndrome: a disease that mimics congenital toxoplasmosis or Cytomegalovirus infection.

OBJECTIVE: To describe the clinical characteristics of intrauterine infection with lymphocytic choriomeningitis (LCM) virus, an uncommonly recognized cause of congenital viral infection. PATIENTS: Three infants born in the midwestern United States in 1994 and 1995 with clinical features and serologic studies consistent with congenital LCM virus infection and cases of congenital infection identified by review of the medical literature between 1955 and 1996. RESULTS: Twenty-six infants with serologically confirmed congenital LCM virus infection were identified. Twenty-two infants were products of term gestations, and birth weights ranged from 2384 to 4400 g (median, 3520 g). Ocular abnormalities, macrocephaly, or microcephaly were the most commonly identified neonatal features. Twenty-one infants (88%) had chorioretinopathy, 10 (43%) had macrocephaly (head circumference >90th percentile) at birth, and 3 (13%) were microcephalic (head circumference <10th percentile). Macrocephaly and hydrocephalus developed postnatally in one of the latter infants. Hydrocephalus or intracranial calcifications were documented in five infants by computed tomography or magnetic resonance imaging. Nine infants (35%) died, and 10 (63%) of the 16 reported survivors had severe neurologic sequelae, consisting of spastic quadriparesis, seizures, visual loss, or mental retardation. One-half of the mothers reported illnesses compatible with LCM virus infection, and 25% reported exposures to rodents during their pregnancies. CONCLUSIONS: These cases suggest that congenital LCM virus infection could be an underrecognized cause of congenital infection among infants born in the United States. Because of the clinical similarities of these congenital infections, cases of congenital LCM virus infection can be confused with infections with cytomegalovirus or Toxoplasma gondii.

Apgar Score↗

PubMed1997

Longitudinal neurological follow-up of a group of HIV-seropositive and HIV-seronegative hemophiliacs: results from the hemophilia growth and development study.

BACKGROUND: Boys and young men with hemophilia treated with factor infusions before 1985 had a substantial risk of acquiring the human immunodeficiency virus (HIV) and the acquired immunodeficiency syndrome. This study was designed to assess the effects of HIV and hemophilia per se on neurological function in a large cohort of subjects with hemophilia, and to investigate the relationships between neurological disease and death during follow-up. METHODS: Three hundred thirty-three boys and young men (207 HIV seropositive and 126 HIV seronegative) were evaluated longitudinally in a multicenter, multidisciplinary study. Neurological history and examination were conducted at baseline and annually for 4 years. The relationship between neurological variables, HIV serostatus, CD4+ cell counts, and vital status at the conclusion of the study was examined using logistic regression models. RESULTS: The risks of nonhemophilia-associated muscle atrophy, behavior change, and gait disturbance increased with time in immune compromised HIV-seropositive subjects compared with HIV seronegative or immunologically stable HIV-seropositive subjects. The risk of behavior change in immune compromised HIV-seropositive hemophiliacs, for example, rose to 60% by year 4 versus 10% to 17% for the other study groups. Forty-five subjects (13.5%), all of whom were HIV seropositive, died by year 4. Subjects who died had had increased risks of hyperreflexia, nonhemophilia-associated muscle atrophy, and behavior change. CONCLUSIONS: These results indicate that immune compromised, HIV-seropositive hemophiliacs have high rates of neurological abnormalities over time and that neurological abnormalities were common among subjects who later died. By contrast, immunologically stable HIV-seropositive subjects did not differ from the HIV-seronegative participants. Hemophilia per se was associated with progressive abnormalities of gait, coordination, and motor function.

Adolescent↗

PubMed1997

Infections of the central nervous system in the newborn.

Safe, effective vaccines and potent antimicrobial agents have diminished substantially the morbidity and mortality associated with neonatal infections of the central nervous system (CNS), and new molecular methods, such as the polymerase chain reaction, enable clinicians to detect micro-organisms rapidly. Despite these advances, CNS infections remain an important cause of death and neurodevelopmental sequelae. This article summarizes current concepts regarding infections of the developing CNS.

Candidiasis↗

PubMed1996

Cytomegalovirus reinfection in young children.

OBJECTIVE: To determine the frequency of reinfection with new cytomegalovirus (CMV) strains in children in group child-care environments. METHODS: Ninety-two CMV strains isolated serially from children attending child care centers were analyzed. Strains were obtained from 1986 to 1994, from 37 children attending one of six centers in the area of Cedar Rapids and Iowa City, Iowa. The CMV isolates were analyzed by a polymerase chain reaction-based algorithm using primers for the a-sequence, glycoprotein B, and major immediate early (MIE) genes of human CMV. The a-sequence polymerase chain reaction products were compared on the basis of size, and products derived from glycoprotein B and MIE genes were compared according to restriction fragment length polymorphisms. RESULTS: Children were between 8 months and 5 years 7 months of age at the time of CMV isolation. The number of isolates ranged from 2 to 6 per child, and the intervals between the first and last CMV isolation ranged from 11 weeks to more than 3 years. At least 7 (19%) of the 37 children had evidence of infection with more than one CMV strain. In six of these children, reinfection with distinct strains was confirmed by analysis of the MIE gene products of sequential CMV strains. CONCLUSIONS: Children who attend child care centers, like adults who are immunosuppressed or have multiple sexual partners, are at risk of being reinfected with distinct CMV strains.

Algorithms↗

PubMed1996

Cadaver skin allografts and transmission of human cytomegalovirus to burn patients.

BACKGROUND: The potential role of cadaver skin as a vehicle for CMV transmission to burn patients has never been clearly defined. We sought to determine if a cytomegalovirus (CMV)-positive cadaver allograft transmits CMV infections to CMV-seronegative burn patients. STUDY DESIGN: All patients in this study were CMV seronegative on admission. They received CMV-seronegative blood products, and cadaver allografts for temporary wound closure and management without regard to the donor's CMV serum status (positive or negative). RESULTS: Of 493 patients admitted from 1989 to 1993, 22 were CMV seronegative on admission and required cadaver allografts for their burn wounds. Five (22.7 percent) of 22 patients seroconverted during hospitalization: one of five had CMV pneumonia develop, two had CMV viruria develop, and three had persistent fever, abnormal liver enzymes, and diarrhea not ascribable to bacterial or other viral agents. CONCLUSIONS: Cytomegalovirus infections result from using CMV-seropositive cadaver allografts on seronegative burn patients.

Adolescent↗

PubMed1995

Rapid epidemiologic characterization of cytomegalovirus strains from pediatric bone marrow transplant patients.

BACKGROUND: DNA amplification by the polymerase chain reaction (PCR) of human cytomegalovirus (CMV) nucleotide sequences recently has been reported for differentiation of CMV strains. DESIGN: Retrospective study. OBJECTIVE AND PATIENTS: Evaluate the strain patterns of 15 CMV-positive buffy coat specimens from five pediatric bone marrow transplant patients. SETTING: Pediatric bone marrow transplant unit. METHODS: We perform PCR using primers corresponding to two distinct regions of the CMV genome, the major immediate-early (MIE) region and the a-sequence region, with subsequent restriction enzyme analysis of the amplified products. RESULTS: Restriction enzyme analysis with Hae III and Hinf I of products amplified with nested PCR for the MIE region revealed distinguishable digestion patterns between patients but similar patterns for samples from each patient. All were distinct from the CMV Towne laboratory control strain. In contrast to these results, amplification of specimens with a-sequence primers, followed by restriction enzyme analysis, did not allow differentiation between all patients. CONCLUSION: Our results indicate that nested amplification directly from buffy coat specimens using primers for the CMV MIE gene allows rapid CMV strain characterization that is useful for laboratory quality control and epidemiological studies. Distinct CMV strains were found in each patient, suggesting horizontal transmission was not responsible for acquisition of CMV infection in these patients.

Adolescent↗

PubMed1995

The diagnosis of congenital infections: contemporary strategies.

Congenital infections remain an important source of neurologic, ophthalmologic, and audiologic disability for thousands of children throughout the world. This review summarizes the clinical features and describes contemporary approaches to the microbiologic diagnosis of congenital infections. In particular, this review emphasizes the important roles that molecular methods, especially the polymerase chain reaction, have in detecting the many infectious agents capable of damaging the developing nervous system.

Brain Damage, Chronic↗