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Biomedical subjects

B A Kaufman

Publications and source records attributed to B A Kaufman.

At least 55 records · Page 3Linked to original sources
PubMed1994

Effects of selective dorsal rhizotomy for spastic diplegia on hip migration in cerebral palsy.

In spastic diplegia of cerebral palsy, migration of the femoral head beyond a lateral edge of the acetabulum is a common orthopedic deformity and requires surgical treatment. We investigated whether selective dorsal rhizotomy for spastic diplegia halts or exacerbates lateral hip migration. The Reimers migration percentage computed from preoperative and postoperative hip radiographs was used as an index of the severity of lateral hip migration in all 134 hips of 67 children examined. At the time of rhizotomy, 38 patients were between 2 and 4 years of age and 29 were between 5 and 11 years of age. The follow-up period ranged from 6 to 10 months in 20 patients and from 15 to 46 months in 47 patients. Overall, the MP remained unchanged in 75% decreased in 17% and increased in 7%; thus, 93% of all hips examined were stable radiographically. Although most patients experienced postoperative hip stability, there was a significant trend for patients with greater preoperative migration to show decreased postoperative migration. The preoperative ambulatory status of patients had no impact on hip stability after dorsal rhizotomy. By the last follow-up, only 1 patient had undergone orthopedic operations for a persistent hip deformity. The results suggest that in children with spastic diplegia, selective dorsal rhizotomy halts lateral hip migration in the great majority of cases.

Adolescent↗
PubMed1994

Disbanding a multidisciplinary clinic: effects on the health care of myelomeningocele patients.

A multidisciplinary clinic for the evaluation and care of children with central nervous system malformations was established at our hospital in 1963 and disbanded in 1988. Three years later, a retrospective telephone survey of myelomeningocele patients (n = 87) was used to identify the effect of clinic closing on the health and care of these patients. After the clinic was disbanded, 45-66% of myelomeningocele patients failed to have regular medical and specialty care, despite the continued availability of the same specialty care in the same location. The most significant correlation was that older patients had less frequent care; this failure in health care utilization was not related to the patients' insurance status. This lack of care is not benign. An increase in serious morbidity, including amputation and nephrectomy, was detected in those patients with the least follow-up, but not in the control group. A significant problem with coordination of the patients' care exists; neither the patient, the family, nor the local physician assumes the duties of coordinator of care. This review indicates that if a multidisciplinary clinic is disbanded, a large proportion of patients will fail to maintain adequate medical follow-up, and that significant morbidity can occur within several years. It is unrealistic to expect the continuation of effective care by merely maintaining the individual services of the disbanded clinic, without some coordinator of care.

Adolescent↗
PubMed1994

Preferential amplification of the paternal allele in neuroblastomas with N-myc amplification.

There is increasing evidence that imprinting plays an important role in influencing the parental origin of genes involved in cancer-specific rearrangements. In advanced stage neuroblastomas, both allelic loss of the short arm of chromosome 1 (1p) and amplification of the proto-oncogene N-myc are often seen. Therefore, we analyzed 22 human neuroblastomas with N-myc amplification to determine the parental origin of the N-myc allele that is amplified and the 1p allele that is deleted. We used at least three polymorphisms for both the 1p and the N-myc locus to analyze blood and tumor samples from neuroblastoma patients, as well as blood samples from their parents. We determined that the paternal allele of N-myc was amplified in 12 of 15 informative cases (P = 0.02), and the paternal allele on 1p was lost in 6 of 11 informative cases (P > 0.2). These results suggest that parental imprinting does not appear to affect the 1p allele that is deleted, at least in the cases that we have examined. However, imprinting has an important influence on determining the N-myc allele that is amplified in these tumors, suggesting that the paternal allele is either more highly expressed or more likely to undergo amplification.

Alleles↗
PubMed1993

Use of the single-strand conformation polymorphism technique to detect loss of heterozygosity in neuroblastoma.

Human neuroblastomas are characterized by cytogenetic and molecular analysis as frequently containing deletions of distal 1p. Loss of heterozygosity (LOH) studies have localized a region of shared deletion to 1p35-36.1. Using the single-strand conformation polymorphism (SSCP) technique, we developed polymorphic assays for two genes, the amiloride-sensitive Na+/H+ antiporter (APNH) and tumor necrosis factor receptor 2 (TNFR2) genes, which map to this region. We used these SSCPs to detect LOH in a panel of neuroblastomas. Allelic loss was readily detected in 8 of 39 informative tumors. The SSCP-derived LOH results were consistent with LOH results generated from a set of distal 1p probes that identify restriction fragment length polymorphisms (RFLPs). The APNH locus could be excluded from the region of consistent deletion, but the TNFR2 locus could not be excluded. We conclude that the SSCP technique is a precise and efficient method for detecting LOH in human neoplasia.

Alleles↗
PubMed1993

Ventriculoperitoneal shunt infections with gram-negative bacteria.

Infection causes major morbidity and mortality in patients with cerebrospinal fluid (CSF) shunts. The prognosis of CSF shunt infections caused by Gram-negative bacteria (GNB) has been thought to be particularly poor. The authors reviewed all GNB shunt infections treated at Children's Memorial Hospital from January 1986 to January 1990 (n = 23). Of these infections 20 (87%) occurred within 4 weeks after shunt revision (median, 10 days). The most frequent symptoms were fever, lethargy, and irritability; the illness was not severe in the majority of these patients. Escherichia coli was isolated from 12 of 23 patients (52%), Klebsiella pneumoniae from 5 (22%), and mixed GNB from 3 (13%) patients. Initial treatment always included immediate shunt removal, externalized ventricular drainage, and intravenous antibiotics. Extraventricular drainage revision and/or intraventricular antibiotics were required in four patients whose CSF cultures were persistently positive for GNB. At admission, these patients had CSF glucose levels of < 10 mg/dl and CSF positive for GNB by Gram's stain. The overall cure rate was 100%, and no recurrence was observed; however, a subsequent infection with a different organism developed in four patients. Only 2 of 19 patients (11%) who were followed up suffered apparent CNS damage. One patient died of unrelated causes shortly after treatment. Our findings indicate that 1) patients with GNB CSF shunt infections often appear relatively well at presentation; 2) CSF positive for GNB by Gram's stain and very low CSF glucose levels predict continued positive CSF cultures, despite appropriate antibiotic therapy; and 3) GNB CSF shunt infections can be successfully treated by prompt shunt removal, extraventricular drainage, and intravenous antibiotics.(ABSTRACT TRUNCATED AT 250 WORDS)

Anti-Bacterial Agents↗
PubMed1993

Selective lumbosacral dorsal rhizotomy immediately caudal to the conus medullaris for cerebral palsy spasticity.

We describe a variation of selective dorsal rhizotomy for spastic cerebral palsy that involves sectioning of the dorsal spinal roots immediately caudal to the conus medullaris. The operation entails an L1-L2 laminectomy, ultrasonographic localization of the conus medullaris, and partial deafferentation of the L1-S2 roots with electromyographic testing under an operating microscope. In 66 children with cerebral palsy, the operation reduced spasticity in the lower extremity without complications, e.g., motor weakness, neurogenic bladder, and sensory loss. It offers several important advantages over alternative techniques.

Cerebral Palsy↗
PubMed1993

Megakaryoblastic leukemia presenting as a temporal bone granulocytic sarcoma. Case report.

The unusual presentation of acute megakaryoblastic leukemia as a temporal bone granulocytic sarcoma in an infant without systemic manifestations of leukemia is reviewed. Leukemia should be considered in the differential diagnosis of skull and skull-based lesions since the appearance on neuroradiological imaging is not unique in this diagnosis. Surgical treatment, as in this case, is limited to obtaining tissue for diagnosis and draining the infection.

Child, Preschool↗
PubMed1992

Congenital spinal cord astrocytomas.

Congenital spinal cord astrocytomas have only rarely been reported. We report two additional cases and a review of the literature on these infrequent tumors. The congenital cord astrocytomas can have obscure initial presentations, including fever and irritability, leading to delays in diagnosis. Magnetic resonance imaging is the diagnostic procedure of choice, allowing noninvasive delineation of the lesion. The congenital tumors are similar in location and pathology to astrocytomas of the cord in older children. As in older patients, initial treatment should include attempted surgical extirpation, delaying or avoiding radiation therapy with its attendant risks. Reported follow-ups of the congenital astrocytomas suggest they will behave in a fashion similar to spinal cord astrocytomas in older children.

Astrocytoma↗
PubMed1992

Quadriplegia after chiropractic manipulation in an infant with congenital torticollis caused by a spinal cord astrocytoma.

An infant with congenital torticollis underwent chiropractic manipulation, and within a few hours had respiratory insufficiency, seizures, and quadriplegia. A holocord astrocytoma, with extensive acute necrosis believed to be a result of the neck manipulation, was found and resected. We believe that every child with torticollis, regardless of age, should undergo neurologic and radiologic evaluation before any form of physical treatment is instituted.

Astrocytoma↗
PubMed1990

Meningitis in the neurosurgical patient.

In the patient with a basilar skull fracture and CSF leak, the risk of meningitis is greatly increased. The diagnosis of both leak and infection can be obscured by the patient's other injuries, and requires aggressive investigation of symptoms that suggest infection. Although the diagnosis is made with CSF cultures, when clinically suspected, treatment should begin after appropriate cultures have been obtained. Treatment should be directed against the most likely organisms, Streptococcus pneumoniae, Haemophilus influenzae, and the other organisms common to the upper respiratory tract. There are no good indications for prophylactic antibiotic usage in patients with known CSF leaks. The patient with a shunt or other CNS prosthetic device may have various manifestations of infection, depending on the type of device and its termination. Frank meningitis or ventriculitis is not always present. Diagnosis requires direct culturing of the shunt milieu, with the most frequent isolates being staphylococcal species and gram-negative enteric bacilli. The most effective therapy, for both eradication of the infection and minimization of the duration and morbidity of therapy, involves removal of the infected shunt, external drainage during parenteral antibiotic therapy, and complete replacement of hardware at the time of internalization. The postsurgical patient will not develop meningitis very frequently, but like the posttrauma patient, concurrent factors can make the diagnosis difficult. Differentiating infectious from chemical meningitis must often be initially based on CSF cell counts and chemistries alone. Treatment to cover the most likely organism, staphylococcal species and respiratory flora, should be started before the culture results are finalized.(ABSTRACT TRUNCATED AT 250 WORDS)

Central Nervous System↗
PubMed1989

Herniation of the suprasellar visual system and third ventricle into empty sellae: morphologic and clinical considerations.

Intrasellar herniation of the optic nerve, optic chiasm, optic tract (suprasellar visual system, SVS), and anteroinferior third ventricle can occur into primary or secondary empty sellae. The anatomic part of this study evaluated the appearance of the SVS in subjects with normal sellae (n = 52), the patterns and prevalence of SVS herniation in enlarged primary empty sellae (n = 24), and the patterns of intrasellar herniation of the SVS in secondary empty sellae (n = 8). The clinical part of this study was to correlate the visual status with the anatomic patterns of the intrasellar herniated SVS. High-resolution MR and CT were used to define the anatomy. MR was superior to CT in all groups in defining accurately the SVS relationship to the sella turcica. In the normal group, the SVS invariably had a straight-line appearance formed by the optic nerve, optic chiasm, and floor of the third ventricle and was above the sella. The SVS was herniated in three of 24 enlarged primary empty sellae. A difference in the appearance of the hypothalamic and infundibular recesses in the primary empty sella group with SVS herniation (dilated recesses and formation of an obtuse angle) and in the secondary empty sella group with SVS herniation (nondilated recesses and formation of an acute angle) was observed. Visual disturbances in primary empty sellae with SVS herniation were present in two of three subjects. Visual disturbances may be absent or minimal in primary empty sellae and secondary empty sellae with herniation of the SVS. Progression of the symptoms--visual field defects, optic atrophy, and loss of vision--is not inevitable. There was no correlation between the severity of visual symptoms and the degree of herniation of the SVS in either the primary or secondary sellae. We found that intrasellar herniation of the SVS into a primary or secondary empty sella is well delineated with MR, and MR should facilitate decisions concerning surgery or therapy. Visual disturbances proved to be an unreliable indicator of herniation.

Adult↗
PubMed1988

Pituitary stalk agenesis: magnetic resonance imaging of 'ectopic posterior lobe' with surgical correlation.

A patient with anterior pituitary deficiencies, without diabetes insipidus, was found to have a retrochiasmatic mass in association with a large middle fossa cyst on CT and MRI scans, and no tissue in the sella ('empty sella'). At surgery, the pituitary stalk was absent. The retrochiasmatic mass was biopsied and shown to be consistent with pituitary stalk. Recent descriptions of MR visualization of an 'ectopic' posterior pituitary lobe are discussed in relation to this case of agenesis of the pituitary stalk and posterior lobe.

Adolescent↗
PubMed1987

Large pituitary gland adenomas evaluated with magnetic resonance imaging.

Magnetic resonance imaging (MRI), computed tomographic (CT) scanning, and angiography or digital subtraction angiography (DSA) were used preoperatively to evaluate 16 surgically proven cases of sellar tumor with extrasellar extension. There were 15 pituitary tumors and 1 schwannoma. The capabilities of MRI in evaluating these tumors were compared with CT scanning and angiography. Bone destruction and tumor calcification were better detected by CT scanning than by MRI. MRI was as effective as CT scanning in detecting a cyst or variation in tumor consistency. Neither MRI nor CT scanning was capable of distinguishing specific tumor types. In every case, MRI was superior to CT scanning for delineating spatial relationships of the tumor to the 3rd ventricle, the optic apparatus, adjacent brain, and parasellar vasculature. Vessel encasement by tumor was clearly seen on MRI when there was no direct indication of this on other studies. Cavernous sinus invasion was not demonstrated by CT scanning, but was indicated by MRI in 5 cases and was surgically confirmed in 3. MRI can provide more precise spatial information on extrasellar tumor extension. Vascular encasement and cavernous sinus invasion may be determined preoperatively. Treatment expectations and operative approaches can be guided by this information. When MRI is available, it is the test of choice for the preoperative evaluation of patients with suspected large pituitary gland (sellar region) tumors. Contrast-enhanced CT scanning and angiography can be used as supplementary studies to add information inherently unique to these techniques.

Adenoma↗
PubMed1984

Cervicomedullary junction tumor diagnosed by nuclear magnetic resonance scanning: case report.

A neurofibroma of the cervical medullary region was accurately delineated using nuclear magnetic resonance (NMR) scanning. The tumor and its compression of the spinal cord were so well visualized that evaluation by myelography and postmyelographic computed tomography (CT) was unnecessary. NMR scanning should be considered as a replacement for myelography with CT in the initial evaluation of cervicomedullary spinal cord tumors.

Adolescent↗