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Biomedical subjects

H E James

Publications and source records attributed to H E James.

At least 19 recordsLinked to original sources

The effect of intravenous fluid replacement on the response to mannitol in experimental cerebral edema: an analysis of intracranial pressure, serum osmolality, serum electrolytes, and brain water content.

Albino rabbits that had undergone a cryogenic insult over the left parieto-occipital cortex were analyzed for serum osmolality, serum electrolytes, brain water content, and intracranial pressure (ICP) following either a baseline infusion of intravenous (i.v.) fluid (45 mL total) for 3 hours or above-maintenance isotonic saline (73.5 +/- 12 mL or 90.5 +/- 1.5 mL) and mannitol therapy. The subgroups were compared amongst themselves and to sham-operated controls. Serum osmolality was elevated in the higher-dose mannitol subgroup compared with maintenance i.v. fluids subgroup (1 g/kg/h vs 1 g/kg/3 h; p < 0.05), accompanied by an insignificant reduction of serum sodium. A significant reduction in brain water in the injured left hemisphere was seen following high-dose mannitol in the subgroup that received less i.v. (maintenance) fluids than the group that received above-maintenance i.v. fluids (p < 0.025). No reduction in brain water was seen in the subgroup that received above-maintenance i.v. fluids (non-treated groups). Reduction of ICP was not found in the lower mannitol dose group. We conclude that the ability of mannitol to reduce cerebral edema is related to the total amount of i.v. fluid replacement. This implies that the amount of i.v. crystalloid fluid that is administered to patients with cerebral edema and raised ICP requiring mannitol for control needs to be carefully monitored.

Animals↗

The treatment of Candida albicans shunt infections.

Cerebrospinal fluid shunting procedures are performed for the treatment of hydrocephalus. Infection of ventriculoperitoneal shunts may create significant clinical management issues in these patients. The majority of these infections are bacterial, but occasionally a Candida albicans shunt infection may occur. We report two patients who acquired Candida albicans shunt infection and discuss their clinical presentation, management, and successful outcome. The treatment with or without removal of the shunt and the correct dosage and route of administration of the antifungal agents is not well documented. The dilemma of treatment of Candida albicans shunt infections in these patients and review of the limited literature on this subject are the subjects of this report.

Amphotericin B↗

Carboplatin is effective therapy for young children with progressive optic pathway tumors: a Pediatric Oncology Group phase II study.

The Pediatric Oncology Group conducted a phase II study to evaluate the activity of carboplatin in children 5 years or younger with progressive optic pathway tumors (OPTs). Of the 51 patients accrued to this study, 1 was not eligible because the child was older than 6 years. Fifty patients were eligible and had either neuro-imaging or symptomatic evidence of progressive OPTs. Twenty-one of 50 had evidence of neurofibromatosis type I (NF-1). Therapy consisted of carboplatin 560 mg/m2 at 4-week intervals. Patients with stable disease or better after two courses were continued on therapy for 18 months or until progressive disease. Of the 50 eligible children, 39 had stable disease or better, and 34 completed the 18-month therapy. Our data are sufficient to conclude that the proportion of objective responses (complete, partial, or minor response or stable disease) exceeded 30% (P < 0.00001), and the approximate 95% confidence interval estimate of the objective response rate was 0.665 to 0.895. Twenty-one patients went off protocol because of progressive disease. Fifteen patients progressed during the 18-month therapy, and 6 patients progressed after completing therapy. Six children died with progressive disease. Major toxicities were neutropenia and thrombocytopenia, and 3 children experienced allergic reactions. Carboplatin is active and safe for the treatment of young children with progressive OPTs. The addition of other potentially active drugs may further increase the event-free survival for these children.

Antineoplastic Agents↗

Pediatric head injury: what is unique and different.

Pediatric head injury has unique issues that make patient management and outcome different from that of adult head injury. The differences may be classified in various categories. The mechanism of injury may be that of accidental or non-accidental trauma, and in the latter, repeated secondary insults will affect patient management and outcome. Age related aspects will determine a greater or lesser degree of craniocervical junction injuries (disproportionate cranial size to trunk in infancy and early childhood). Other factors are potential underlying congenital anomalies, physiological factors (cerebrovascular reactivity and blood flow), differing support systems needed from that of adults for neuro imaging and specialized medical, nursing and allied health care support. Pediatric rehabilitation and educational needs and goals are different to that of adult head injury. Neurological recovery in non-accidental trauma is uniformly poor, when compared to accidental trauma. Pediatric head injury has unique requirements in early field management, hospital management, rehabilitation and educational needs, as well as community re-entry programmes.

Child, Preschool↗

Cutaneomeningospinal angiomatosis (Cobb syndrome) with tethered cord.

A newborn presented with a skin-covered lumbar mass with a subcutaneous hemangioma and on a magnetic resonance image (MRI) revealed a tethered spinal cord with a local mass. The mass had signal characteristics compatible with a lipoma. An initial diagnosis of a lipomeningocele with tethered cord was made, and the patient underwent surgical exploration and subtotal resection of the mass. A follow-up MRI revealed that the cord was still tethered, but an additional mass was present. The initial mass with signal characteristics of lipomatous tissue was accompanied by a low-signal mass in the lumbosacral canal, ventral to the cord, and bilateral enlargement of the foramina at the lumbosacral level. Because of a concern for an intraspinal tumor, a second operative intervention was performed. Multiple biopsies of the mass inside the spinal cord, the nerve roots and at the level of the foramina revealed angiomas that had similar pathology in all the specimens. A partial resection of the masses and a release of the tethered cord was performed by sectioning the thickened filum terminale. The diagnosis of Cobb's syndrome was made. The unique association of a tethered cord and the Cobb syndrome is reported here.

Abnormalities, Multiple↗

Suprasellar germinoma associated with Cushing's disease and diabetes insipidus in a child.

Cushing's disease is described in a child with a suprasellar germinoma. The patient presented with all of the classic stigmata of Cushing's disease as well as diabetes insipidus, but after surgical resection of the lesion was found to have pathology incompatible with an ACTH-secreting tumor. We believe that this is the first reported incidence of Cushing's disease associated with a suprasellar germinoma. The implications of this unusual association are discussed.

Adolescent↗

Spinal hamartomas: a distinct clinical entity.

OBJECT: Congenital spinal hamartomas are defined as tumors of well-differentiated mature elements situated in an abnormal location. In this report, the authors document the clinical and pathological features of spinal hamartomas in 10 patients. METHODS: Ten patients presented with midline dorsal malformations at birth, initially diagnosed as teratomas or myelomeningoceles. The locations of the masses were variable: two were located in the thoracic region, four at the thoracolumbar junction, two in the lumbar region, one at the lumbosacral junction, and one in the sacral region. The results of the neurological examination were normal in nine patients. All but one mass had intact skin and seven had palpable bone components. Neuroimaging studies revealed widening of the spinal canal, heterotopic bone located dorsally in some patients, and varying degrees of involvement of the intraspinal contents. During surgery, six patients were found to have involvement of the spinal cord or cauda equina. The pathological characteristics of the masses included three or more of the following: bone, cartilage, synovial membrane, urinary tract tissue, cyst wall, yellow or brown fat, and nerves. The well-differentiated cellular elements, which formed mature structures, along with the absence of primitive cellular components and neoplastic characteristics are more consistent with a diagnosis of hamartoma than teratoma. CONCLUSIONS: In this series, the authors describe a lesion that is overt on physical examination, yet can have occult spinal canal involvement. Complete neurosurgical evaluation is essential to provide appropriate treatment and prognosis.

Adipose Tissue↗

Ventriculoperitoneal shunts for hydrocephalus: a focus group discussion on the selection of shunt systems in pediatrics. A report of the Pediatric Neurosurgery Research Group meeting, December 1992.

As part of the Continuing Quality Improvement Study, phase I, 12 pediatric neurosurgeons participated in a discussion group to respond to the various issues related to selection of ventriculoperitoneal shunt systems in their practice. It was estimated that between then all the 12 participants performed approximately 1200 shunt procedures per year in the USA. The data were analyzed by grid and fish-bone charts as part of the data collection. All 12 participants indicated that they preferred a reservoir in the shunt system, 8 of them indicating that they preferred this for a manual test of the system. Three indicated that they did not routinely manipulate the system for testing. One participant stated that he placed a reservoir within the shunt system primarily to prevent migration of the ventricular catheter. Nine neurosurgeons indicated that they employed cranial valves in their shunt systems, and three indicated that they employed slit-end peritoneal catheters only. In reference to Delta valves, nine participants indicated that they employed them on occasions. In reference to pressure in the valve system, one participant employed high-pressure valves at all times, five employed only medium-pressure systems, and the remaining six always employed low-pressure valves. All 12 neurosurgeons stated that the incidence of symptomatic slit ventricle in their practices was between 1% and 5%, in their mind irrespective of the system used. It was concluded that any form of ventriculoperitoneal shunt, with or without valves, with slit-end valves or cranial valves seemed to work equally well in the hands of a dedicated pediatric neurological surgeon.(ABSTRACT TRUNCATED AT 250 WORDS)

Cerebrospinal Fluid Pressure↗

Cephalocele detection in utero: sonographic and clinical features.

Sonographic and clinical features of 26 fetal cephaloceles were reviewed retrospectively. The most frequent reason for referral was elevated maternal serum alpha-fetoprotein levels. The smallest lesion identified was 0.4 x 0.5 cm (frontal, at 20 weeks); the largest was 9.0 x 10.0 cm (frontal, at 27 weeks). Twenty-four of 26 cephaloceles were detected on prenatal ultrasound examination; in 13 of these, more than 50% of the intracranial contents were exteriorized. Fifteen of 24 cephaloceles detected prenatally had a sulcal pattern (identified between 16 and 36 weeks' gestation); five were solid without a sulcal pattern (identified between 13 and 21 weeks' gestation), three were cystic, and one underwent a change in appearance from solid at 21 weeks to cystic at 26 weeks. Other cranial features were evaluated and included visible skull defect, seen in 23/24 (96%), ventriculomegaly, in 6/26 (23%); microcephaly, in 12/24 (50%); beaked tectal plate, in 6/16 (38%); and flattened basiocciput, in 9/24 (38%). Of the 26 cases, 14 had normal amniotic fluid volume, five had oligohydramnios and seven had polyhydramnios. Fetuses with oligohydramnios had the highest incidence of concurrent fetal abnormalities; four of five fetuses (80%) with oligohydramnios had additional structural abnormalities. In the overall population, a very high incidence of other abnormalities was found; 17/26 (65%) cases showed additional abnormalities, some of which were not detected by ultrasound. Five fetuses had Meckel-Gruber syndrome and three had amniotic band syndrome. Only one of the 18 karyotypes obtained was abnormal (trisomy 18). Survival was very poor; only two of the eight who survived until birth are currently living.

Abnormalities, Multiple↗

Postoperative chemotherapy and delayed radiation in infants and very young children with choroid plexus carcinomas. The Pediatric Oncology Group.

Eight infants with choroid plexus carcinomas were treated with surgery, prolonged postoperative chemotherapy and delayed radiation. The results suggest that some infants with choroid plexus carcinomas can be successfully treated with multimodality therapy, even allowing children with less than a gross total resection to have prolonged disease-free intervals.

Antineoplastic Combined Chemotherapy Protocols↗

Syringomyelia of the distal spinal cord in children.

The syndrome of syringomyelia in children has been extensively described in the literature and is said to most commonly involve the cervical and thoracic spinal cord. We here present two children who had an unusual constellation of signs and symptoms, characterized by bowel and bladder disturbance in one, and in the other with intermittent paroxysmal severe pain and cramping and flexion of the lower extremities accompanied at times by episodes of incontinence. Both were found on magnetic resonance imaging (MRI) to have a syrinx localized to the most distal cord. This was not associated with Arnold-Chiari malformation, trauma, tumors or any other form of spinal dysraphism. Both patients underwent placement of a syringomyelia-peritoneal shunt with complete resolution of symptomatology. Postoperative MRI revealed a complete collapse of the syringomyelia cavity. In those children presenting with bowel or bladder disturbances, associated or not with intermittent pain, flexion attacks, or cramping in the lower extremities, the differential diagnosis of a syringomyelia of the distal cord should be part of the clinical considerations.

Child, Preschool↗

Human tails and associated spinal anomalies.

Human tails have been described intermittently in the literature, typically as isolated cases with varying forms of malformation. Attempts have been made to differentiate "true" tails from "tail-like" appearances. Unless identified, underlying occult spinal disorders, mass effect, and/or tethering of the spinal cord may lead to progressive neurologic damage. We report three patients with "tails" and the associated spinal anomalies.

Female↗

Distal spinal cord pathology in the VATER association.

Herein the authors report the pathology of six cases of VATER association and tethered spinal cord that underwent operative intervention for removal of lipomas of the conusfilum terminale and/or release of the tethered cord. In five of the six patients, the excised filum terminale consistently showed excessive mature adipose tissue, normal blood vessels, small myelinated nerve fibers, and fibrous connective tissue. One patient had mature lobulated adipose tissue and vessels only. It is concluded that patients with the VATER association who present with distal spinal cord pathology consistently have abnormal involution of the filum terminale, the hallmark being an abnormal collection of mature fat, thickening of the filum, and extension of the fat into the conus medullaris.

Abnormalities, Multiple↗

Cerebrospinal fluid shunt infection secondary to Escherichia coli bacteriuria.

A 10-year-old girl with a lumbosacral myelomeningocele, managed with clean intermittent catheterization, presented with headache, vomiting, and lethargy. The cerebrospinal fluid (CSF) and urine cultures revealed Escherichia coli, documented to be the same subtype. The organisms were subtyped and the E. coli from both the urine and CSF were noted to be of the same strain. Management consisted of intravenous antibiotics and ultimate replacement of the ventriculoperitoneal shunt. Children with myelodysplasia and CSF shunts should be carefully monitored in a multidisciplinary fashion to anticipate, correctly diagnose, and treat CSF shunt infections associated with bacteriuria.

Bacteriuria↗

Impairment in shifting attention in autistic and cerebellar patients.

MRI and autopsy evidence of early maldevelopment of cerebellar vermis and hemispheres in autism raise the question of how cerebellar maldevelopment contributes to the cognitive and social deficits characteristic of autism. Compared with normal controls, autistic patients and patients with acquired cerebellar lesions were similarly impaired in a task requiring rapid and accurate shifts of attention between auditory and visual stimuli. Neurophysiologic and behavioral evidence rules out motor dysfunction as the cause of this deficit. These findings are consistent with the proposal that in autism cerebellar maldevelopment may contribute to an inability to execute rapid attention shifts, which in turn undermines social and cognitive development, and also with the proposal that the human cerebellum is involved in the coordination of rapid attention shifts in a fashion analogous to its role in the coordination of movement.

Adolescent↗