Alexander disease with serial MRS and a new mutation in the glial fibrillary acidic protein gene.
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Acidosis, Respiratory↗
Biomedical subjects
C Stack
Publications and source records attributed to C Stack.
Long-term outcome of patients with intraspinal neuroblastoma.
BACKGROUND: Chemotherapy, radiotherapy, and surgical decompression with laminectomy are effective therapeutic options in the treatment of cord compression from neuroblastoma (NB). We report the long-term outcome of patients with intraspinal NB treated with or without laminectomy at two large pediatric oncology centers. PROCEDURE: We reviewed the medical records and radiographs of 26 children with intraspinal NB treated at Children's Memorial Hospital in Chicago, Illinois, between 1985 and 1994 or at St. Jude Children's Research Hospital in Memphis, Tennessee, between 1967 and 1992. RESULTS: Twenty-four of the 26 patients are alive and disease-free (follow-up of 2-29 years; median, 10 years 2 months). Fifteen of the 23 patients with neurologic impairment underwent initial laminectomy. Nine of these 15 patients recovered neurologic function, including 3 patients who presented with paraplegia. Eleven of the 15 patients who underwent laminectomy have developed mild to severe spinal deformities. Eight patients with neurologic symptoms consequent to cord compression were treated with initial chemotherapy and/or surgery, but did not undergo laminectomy. Three patients with mild to moderate deficits recovered neurologic function. Four of 11 patients with intraspinal NB who did not undergo laminectomy have mild to severe scoliosis. CONCLUSIONS: A low incidence of neurologic recovery was seen in patients with long-standing severe cord compression regardless of treatment modality. For patients with partial neurologic deficits, recovery was seen in most patients following chemotherapy or surgical decompression with laminectomy. A higher incidence of spinal deformities was seen in the patients treated with initial laminectomy.
Child↗
Five-year follow-up of a prospective randomised multi-centre trial of weekly chemotherapy (CAPOMEt) versus cyclical chemotherapy (CHOP-Mtx) in the treatment of aggressive non-Hodgkin's lymphoma. Central Lymphoma Group.
BACKGROUND: Weekly alternating regimen known as CAPOMEt is compared to standard cyclical chemotherapy (CHOP-Mtx) in aggressive non-Hodgkin's lymphoma (NHL). PATIENTS AND METHODS: Three hundred and eighty-one patients with aggressive NHL were randomised to receive either cyclophosphamide, doxorubicin, vincristine, prednisone and methotrexate (CHOP-Mtx) on a cyclical basis or a weekly regimen incorporating the same drugs with the addition of etoposide (CAPOMEt). RESULTS: After pathological review, 281 patients were deemed eligible. At the census date of 31 March 1994, 158 patients were alive with a median follow up of 5.9 years (minimum 3.0 years). Analysis of all patients and eligible patients showed no significant treatment differences in the rates of complete remission (CR), failure free survival (FFS) or overall survival (OS) between the two arms. The actuarial median OS was 24 months for CAPOMEt compared with 31 months for CHOP-Mtx, with five-year actuarial survival rates of 37% and 43%, respectively. Myelosuppression was significantly more severe with CHOP-Mtx and neurotoxicity was much more common with CAPOMEt. CONCLUSION: Weekly CAPOMEt is equally effective as standard cyclical CHOP-Mtx treatment in aggressive NHL.
Adolescent↗
Amylaceous (polyglucosan) bodies in familial cerebral atrophy of early onset.
An 8-year-old girl and her 4-year-old sister presented with psychomotor retardation during the 1st year of life. This was followed by spasticity, seizures, and in the older patient, progressive loss of faculties and death. Computed tomographic and magnetic resonance imaging scans demonstrated progressive cerebral atrophy in both patients. Postmortem examination in the older sibling showed diffuse atrophy of the supragranular layers of the cerebral cortex, atrophy and gliosis of the white matter, and accumulation of numerous amylaceous (polyglucosan) bodies within the cytoplasm of cell processes. The inclusions were closely associated with atrophy of the parenchyma. The findings suggest that this disorder is different from other childhood diseases in which amylaceous bodies accumulate within the brain.
Adipose Tissue↗
Study design for epidemiologic studies with measurement error.
Exposure measurement error in epidemiological studies is recognized as a feature that must be considered because of the potential bias that can result in estimates of the exposure-disease association. Most of the work to date has focused on methods of analysis that adjust for the resultant bias, but the implications of this work to the design of epidemiologic studies is not as well understood. An overview of the issues involved in the use of methods for dealing with errors in exposure information is discussed along with some design options that have been proposed for providing information necessary for their use. Validation studies compare somewhat crude and inexpensive measures of exposure to a gold standard, and study designs that incorporate these into the overall plan can realize some advantages in terms of cost. In addition, repeated assessments of exposure can realize efficiency for measures of exposure that are unbiased. However, much work remains to be done in the development of efficient designs for studying disease aetiology and prevention.
Bias↗
Analgesia for children in intensive care. Assessing pain is fraught with difficulties.
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Analgesia↗
Respiratory compliance during sedation, anesthesia, and paralysis in infants and young children.
Although total respiratory compliance (Crs) has been shown to fall in adults on induction of halothane anesthesia, no successful paired studies have been reported in children. The multiple occlusion technique was used to measure Crs in 17 infants and young children during sedated sleep (CrsS) and shortly after, following induction of halothane anesthesia (CrsA). Crs fell in all but one infant after induction of anesthesia, with a mean fall of 34.7% (range 0-58%). This was accompanied by a reduction in tidal volume and increase in frequency in every case. In 7 of the 17 children, who were to be paralyzed for surgical purposes, Crs was also measured in this anesthetized-paralyzed state. When tidal volume administered during manual ventilation was similar to that observed during measurement of CrsA, Crs during this low-volume ventilation was similar to CrsA. When tidal volume was increased and Crs remeasured, there was a significant increase in every case, with the high-volume Crs within 10% of CrsS in all but one child, in whom there was a 31.4% increase with respect to CrsS. Changes in tidal volume accounted for approximately 50% of the variability in each state. These results demonstrate a highly significant fall in Crs in infants and young children after induction of halothane anesthesia. In addition it appears that this reduction in Crs can be reversed by paralyzing the child and manually ventilating with tidal volumes approximating those seen during sedation.
Anesthesia↗
Influence of tidal volume on respiratory compliance in anesthetized infants and young children.
Recent studies have suggested a close association between total respiratory compliance (Crs) and tidal volume in anesthetized paralyzed infants who are being artificially ventilated. To investigate this further, the multiple occlusion technique was used to measure Crs in 20 anesthetized infants and young children (aged 1-25 mo) before elective surgery. Measurements were made after intubation 1) during spontaneous breathing (SB), 2) after administration of a non-depolarizing muscle relaxant with tidal volume and frequency mimicking that during SB, and 3) with the child still paralyzed but tidal volume approximately double that during SB. Compared with values obtained during SB, there was no significant change in Crs after paralysis when ventilation matched the child's own pattern (P greater than 0.2). When ventilated with the larger tidal volumes, the infants showed a highly significant increase in Crs (mean 62%, range 14-158%, P less than 0.0001). These results may have implications not only for studies performed during anesthesia but also when infants were monitored in the intensive care setting. Values of Crs obtained in ventilated infants may reflect both the mechanical behavior of the respiratory system and the pattern of ventilation at the time of measurement.
Anesthesia↗
Superficial posterior compartment syndrome of the leg with deep venous compromise. A case report.
A 15-year-old boy was struck twice in the calf, developing what appeared initially to be phlebitis and deep venous compromise. Careful clinical examination and compartment pressure measurement revealed the presence of an isolated superficial posterior compartment syndrome. Fasciotomy provided prompt relief. Findings at surgery showed ischemic changes but no necrosis. Venous patency was demonstrated on a subsequent venogram. Recovery was complete. This is the first reported case of isolated traumatic superficial posterior compartment syndrome. It adds to numerous other reports and provides further evidence that compartment syndrome can occur in any myofascial compartment. The concurrent deep venous compromise, although physiologically expected because of pressure differentials, was graphically demonstrated for the first time as well. Its clinical significance is not clear.
Adolescent↗
Congenital generalized fibromatosis: a review of the literature and report of a case associated with porencephaly, hemiatrophy, and cutis marmorata telangiectatica congenita.
In congenital generalized fibromatosis (CGF), fibrous nodules involve the skin, bone, and viscera, and the mortality rate is as high as 80% because the visceral nodules can significantly obstruct and compress vital organs. If the fibromas involve only the skin and skeleton and not the viscera, the disease is known as congenital multiple fibromatosis, and the prognosis is excellent. In both conditions, which may be variants of the same disease process, the fibromas resolve completely and spontaneously. There are no previous reports in the literature of CGF associated with cutis marmorata telangiectatica congenita (CMTC), hemiatrophy, or porencephaly with hemiparesis, although CMTC has been seen in conjunction with hemiatrophy. These associations may be coincidental.
Atrophy↗