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Biomedical subjects

J F Bale

Publications and source records attributed to J F Bale.

At least 91 records · Page 5Linked to original sources
PubMed1981

Progressive neurological disease associated with chronic cytomegalovirus infection.

Cytomegalovirus (CMV), the most common infectious cause of nervous system damage in the fetus and newborn infant, generally has been thought to exert its teratogenic effect during early pregnancy, and it has been assumed that clinical or postmortem abnormalities reflect static insults incurred earlier. Over the past two years we have identified two infants who presented with various clinical signs suggesting CMV infection. Serial CT head scans showed mild hydrocephalus progressing to extensive loss of brain substance and hydrocephalus in one and a normal scan going on to polycystic encephalomalacia in the other. Both infants had urine or leukocyte cultures that were repeatedly positive for CMV. These data suggest that CMV can cause progressive central nervous system damage after birth. The findings may account for some unexplained brain malformations and for a portion of the neurodegenerative disorders occurring in babies who appear normal at birth.

Brain Diseases↗
PubMed1981

Brain abnormalities in infants with Potter syndrome (oligohydramnios tetrad).

We examined the brains of seven unrelated infants with Potter syndrome (oligohydramnios tetrad), a lethal neonatal disorder characterized by abnormal facies, lung hypoplasia, limb deformities, and classically, renal agenesis. All infants had defects of neuronal migration. The brains were small for gestational age in five of seven infants, and in four infants, the middle and inferior temporal gyri were incompletely demarcated. Cerebellar heterotopia occurred in five infants. All had abnormal hippocampi and abnormal lamination of the cerebral cortex. These neuropathologic abnormalities suggest that Potter syndrome may not be the result of a single toxic or infectious insult but may represent a polygenic inherited disorder.

Amniotic Fluid↗
PubMed1980

Cytomegalovirus and dual infection in infants.

In four unrelated infants without underlying immunodeficiency, dual infections with cytomegalovirus (CMV) and another microorganism developed. The patients included the following: (1) a 3-month-old girl with congenital CMV and perinatal cutaneous herpes simplex virus; (2) a 5-week-old girl with CMV and Pneumocystis carinii; (3) a 12-week-old girl with CMV and Haemophilus influenzae meningitis; and, (4) a 2 1/2-month-old girl with CMV and Escherichia coli meningitis. In all four cases, the patient's initial symptoms were referable not to CMV, but to the companion infecting organism. The diagnoses of CMV infection were made, respectively, by a high index of clinical suspicion in the first three cases and on the basis of a lucent parenchymal defect on computerized tomographic scan in the fourth patient. These cases provide additional evidence that CMV infection may predispose to secondary infection. We recommend that infants who have signs of infection and evidence of CNS abnormalities have cultures made for CMV. Both human CMV and experimental murine CMV infections have been associated with suppressed cellular and possibly humoral immunity.

Cytomegalovirus Infections↗
PubMed1980

Encephalopathy in young children with moderate chronic renal failure.

We studied three boys who had a similar encephalopathy develop during moderate chronic renal failure. The features included cerebellar system dysfunction, seizures, and intellectual and motor retardation. Extensive laboratory investigation failed to disclose an etiology other than chronic azotemia. These cases suggest that the developing nervous system is particularly sensitive to the effects of chronic azotemia. Early dialysis and/or transplantation should be considered in such children in an attempt to modify the serious nervous system abnormalities.

Brain Diseases↗
PubMed1980

CNS manifestations of the hemolytic-uremic syndrome. Relationship to metabolic alterations and prognosis.

To define the CNS manifestations of the hemolytic-uremic syndrome (HUS), the records of 61 consecutive episodes of HUS in 60 patients were reviewed. Major CNS signs (seizures and/or coma) occurred in 30 of the 61 episodes. Twenty-four children had seizures. Analysis of blood pressure and metabolic parameters or admission to the hospital did not predict which child would exhibit CNS signs. However, during the subsequent course of the illness, children with CNS dysfunction had lower minimum serum sodium concentrations and more severe azotemia and were more likely to require dialysis than children without CNS signs. Of the 61 episodes, there were three deaths, and three children suffered major CNS sequelae. Coma at the time of admission and elevated CSF protein levels were features associated with poor outcome.

Adolescent↗
PubMed1979

CT scans in Menkes disease.

The clinical courses and serial computerized tomography (CT) scans of four patients with Menkes disease are described. Although the initial clinical presentations were similar, head growth and serial CT scans showed striking individual differences. The CT scans varied from showing no abnormalities early in the disease to showing diffuse cortical atrophy, subdural accumulation of fluid, or multifocal areas of ischemic infarction. The pathologic findings in one patient showed only cerebral and cerebellar atrophy, whereas the findings in another patient showed areas of ischemic infarction, probably secondary to abnormal vessels. Menkes disease should be suspected in male infants with psychomotor deterioration and seizures, or when trauma is suspected from subdural hematoma and multiple fractures.

Brain↗
PubMed1977

Fatal histiocytic lymphoma of the brain associated with hyperimmunoglobulinemia-E and recurrent infections.

A 10-year-old girl with eczema, asthma, recurrent abscesses, middle ear infections and pneumonias developed a histiocytic lymphoma of the brain. Immunologic evaluation revealed hyperimmunoglobulinemia E, depressed neutrophil chemotaxis, absent delayed hypersensitivity skin test tests, low T-lymphocyte numbers and depressed in vitro lymphocyte responses. The occurrence in this patient of a histiocytic lymphoma, which is commonly associated with more classic immunodeficiency syndromes or immunosuppression for transplantation, suggests that individuals with hyperimmunoglobulinemia E and recurrent infections may also be at increased risk for developing unusual malignancies.

Brain Neoplasms↗
PubMed1977

Evaluation of nonspecific (alternative pathway) opsonic activity by neutrophil chemiluminescence.

The role of alternative complement pathway activation in protection against infection is not well understood. We have investigated nonspecific opsonic activity in human adult, term neonatal and premature serum using the technique of neutrophil chemiluminescence (cl) to measure particle uptake. Following phagocytosis, neutrophils become metabolically active, produce excited molecular oxygen and emit a burst of light which can be detected and quantitated in a liquid scintillation counter. In the present study, zymosan particles were preopsonized in human serum and added to control neutrophils. Particles opsonized in adult serum produced a marked peak in CL. Serial dilutions of the serum prior to opsonization yielded proportionally lower peaks in CL. Opsonic activity as measured by the CL procedure was completely blocked by the addition of chelating agents which remove calcium and magnesium ions and block both classic and alternative complement pathway activation. Addition of an excess of magnesium ions (needed for alternative pathway activation) to the reaction mixture partially restored the opsonic activity. Opsonic activity as measured by the CL procedure was significantly depressed in approximately two-thirds of term and premature infant sera tested. Deficient nonspecific opsonic activity was closely correlated with serum levels of C3PA. These studies suggest that the CL procedure may be of value in the investigation of nonspecific opsonins in human serum.

Adult↗
PubMedDate not supplied

Cytomegalovirus infection in newborns and their family members: polymerase chain reaction analysis of isolates.

Polymerase chain reaction (PCR) analysis with primers for the pp65, a-sequence, glycoprotein B, and major immediate early genes of human cytomegalovirus (CMV) was used to study five congenitally-infected infants and their CMV-infected family members. Family members excreting CMV included three mothers and two siblings. The PCR results indicated that the CMV strain excreted by each infant was indistinguishable from that excreted by the corresponding family member. By contrast, the molecular profiles of the CMV strains were distinct between families, indicating that the PCR algorithm described in this study is a useful method for analyzing CMV strains.

Antigens, Viral↗