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Biomedical subjects

M L Levy

Publications and source records attributed to M L Levy.

At least 91 records · Page 5Linked to original sources
PubMed1997

Surgical management of sagittal synostosis: a comparative analysis of strip craniectomy and calvarial vault remodeling.

Although surgical treatment of sagittal synostosis has become normative, the procedure of choice is still debated. This retrospective analysis, during a 10-year period (1986-1995) at Children's Hospital Los Angeles, compares the more conservative strip craniectomy (SC) procedure with a more extensive occipital reduction-biparietal widening calvarial vault remodeling (CVR) technique for the management of sagittal synostosis. SC was performed in 72 patients (61%) and CVR in 46 (39%). Patients undergoing CVR were older (mean 5.3 vs. 3.0 months, p < 0.01), the procedure time longer (mean 126 min vs. 72 min, p < 0.01), intraoperative blood loss greater (mean 243 vs. 54 cm3, p < 0.01), and the frequency of intraoperative blood transfusion increased (100 vs. 38%, p < 0.01; mean volume 357 vs. 51 cm3, p < 0.01) compared to patients undergoing SC. No significant intraoperative complications occurred in either group. The total time of hospitalization was similar (mean 4.4 vs. 3.8 days, p = 0.02). No neurological, hematological, or transfusion-related complications or deaths were encountered in either group. Cosmetic outcomes were significantly better in the CVR group with 79% rated as excellent compared to 41% in the SC group (p < 0.01). Fifty-nine percent of the SC patients manifested bony defects at last follow-up visit. Two SC patients required reoperation for poor cosmetic outcomes; CVR was successfully employed in both cases. These findings demonstrate that CVR is superior to SC in providing immediate and lasting correction of calvarial deformities secondary to sagittal synostosis. The optimal age at time of surgery is <6 months; however, CVR can be utilized throughout infancy, while SC becomes progressively less effective after 6 months of age. No significant complications were observed in either group, despite the increased operative manipulation and greater incidence of blood transfusion in the CVR patients.

Craniotomy↗
PubMed1997

Congenital alopecia areata.

Alopecia areata has never been documented in a newborn. Thus, it is generally assumed that alopecia areata is acquired only postnatally, and it is believed that the presence of an alopecia at birth virtually excludes its diagnosis. In this report we document a case of alopecia areata in a premature newborn.

Alopecia Areata↗
PubMed1997

Nutrition requirements in patients with toxic epidermal necrolysis.

Patients with toxic epidermal necrolysis, a severe, exfoliative skin disorder, have clinical features similar to those of partial-thickness burn patients. The literature suggests that they also have similar nutritional requirements. We report two patients diagnosed with toxic epidermal necrolysis on mechanical ventilation, in whom resting energy expenditure and respiratory quotient were measured by indirect calorimetry. The patients were treated using standard burn protocols. Nitrogen balance was calculated by measuring total urinary nitrogen in urine samples obtained over 24 hours. These measurements were done while the patients were on mechanical ventilation and receiving total parenteral nutrition. As in burn patients, early in their course the two patients had resting energy expenditure values twice that predicted. After 12 days of hospitalization, nitrogen balance was negative in patient 1 and positive in patient 2. Energy and protein requirements appear to have been related to the amount of body surface affected.

Calorimetry, Indirect↗
PubMed1997

Temporary occlusion of the middle cerebral artery in intracranial aneurysm surgery: time limitation and advantage of brain protection.

The risk of focal infarction secondary to the induced reversible arrest of local arterial flow during microsurgical dissection of middle cerebral artery (MCA) aneurysms was evaluated further to define the optimal approach to temporary arterial occlusion. To compare the effectiveness of potential brain-protection anesthetics, a group of patients treated with the intravenous agents propofol, etomidate, and pentobarbital, administered individually or in combination, was compared to a group treated with the inhalational agent isoflurane. Forty-nine consecutive MCA aneurysm surgeries involving the temporary clipping of the parent vessel were retrospectively reviewed. Thirty-eight patients received intravenous brain-protection (IVBP) anesthesia. Groups of patients with and without infarctions, and receiving and not receiving IVBP anesthesia, were compared based on the duration and nature of temporary arterial occlusion. Postoperative radiographic evidence of new infarction was used as the threshold for failure of occlusion tolerance. The overall infarction rate was 22.4% (11 of 49 patients), including 15.8% (six of 38 patients) in the IVBP group versus 45.5% (five of 11 patients) in the group that did not receive brain protection (NBP). In the NBP group, the mean duration of temporary occlusion was 3.9 +/- 2.2 minutes for patients without infarction versus 12.2 +/- 4.3 minutes for patients with focal infarction (p < 0.01). In contrast, the mean duration was 13.6 +/- 10.6 minutes for patients without infarction and 18.5 +/- 9.9 minutes for patients with infarction in the IVBP group. All patients (four of four) in the NBP group who underwent occlusion lasting 10 minutes or longer suffered an infarction versus five of 23 patients in the IVBP group (p < 0.0001). Patients with multiple aneurysms were found to be at increased risk of developing focal infarction, whereas those treated with intermittent temporary clip application were at decreased risk. It is concluded that patients in whom focal iatrogenic ischemia is induced during MCA aneurysm clip ligation have a significant advantage compared with those receiving isoflurane when they are given pentobarbital as the primary neuroprotective agent or when they receive propofol or etomidate titrated to achieve electroencephalographic burst suppression, particularly if more than 10 minutes of occlusion time is required. It is also concluded that 10 minutes is a general guideline for safe, temporary occlusion of the MCA. The use of intermittent temporary arterial occlusion and its use in patients with multiple aneurysms need further evaluation before specific recommendations can be made.

Anesthesia, Intravenous↗
PubMed1997

Methylmalonic acidemia, cobalamin C type, presenting with cutaneous manifestations.

BACKGROUND: Erosive dermatitis resembling the skin lesions of acrodermatitis enteropathica has been described in a number of aminoacidopathies and organic acidemias. In some, the dermatitis is a manifestation of untreated disease, while in others, including methylmalonic acidemia, skin lesions have been ascribed to nutritional deficiency due to therapeutic amino acid restrictions. OBSERVATIONS: We report 2 cases of methylmalonic acidemia presenting with cutaneous manifestations in the perinatal period before restrictive nutritional interventions. The cutaneous involvement consisted of cheilitis and diffuse erythema with erosions and desquamation. Methylmalonic acidemia, cobalamin C type, was subsequently diagnosed in both cases. CONCLUSIONS: An erosive, desquamating dermatitis with histopathologic characteristics resembling acrodermatitis enteropathica may be a presenting sign in cobalamin C methylmalonic acidemia, even in the absence of long-standing nutritional restrictions or deficiency.

Acrodermatitis↗
PubMed1997

Metered dose inhalers: current and future uses.

Respiratory disease poses an enormous burden on individuals and society. Asthma and chronic obstructive pulmonary disease (COPD), the diseases discussed here, contribute towards a substantial proportion of ill health and cost to the NHS and the economy. Most health professionals consider the inhaled route to be the best method of delivering drugs to the respiratory system. Four types of inhaled delivery system are in use: pressurised metered dose inhalers (pMDIs); dry powder devices; pMDIs and large volume spacers in combination; and nebulisers. In the UK pMDIs are the cheapest type of device on the market and are usually the method of choice for those patients able to use them. International agreement to phase out CFCs has led to the development of alternative propellants for pMDIs. The role of pMDIs is predicted to increase in the future. Demand for pMDIs to treat asthma is likely to increase as the introduction of clinical guidelines extends best practice, and their use to treat patients with COPD is increasing. Novel drugs for the respiratory system, including gene therapy and drugs for non-respiratory disorders, may well employ pMDIs as a delivery system in the future.

Anti-Asthmatic Agents↗
PubMed1996

Alzheimer disease and frontotemporal dementias. Behavioral distinctions.

BACKGROUND: Frontotemporal dementia (FTD) is a syndrome produced by lobar degeneration of the temporal and/or frontal lobes. OBJECTIVES: To quantify the behavioral disturbances of FTD and compare them with behavioral changes observed in Alzheimer disease (AD). DESIGN: Cross-sectional comparison of 2 groups defined by research diagnostic criteria and single photon emission computed tomography. Behaviors were assessed using a standardized rating scale-Neuropsychiatric Inventory. Groups were matched for dementia severity. SETTING: Patients were seen at 2 university-based outpatient dementia clinics and a Veterans Affairs medical center. PARTICIPANTS: Twenty-two patients with FTD and 30 patients with AD. RESULTS: Patients with FTD had significantly greater total Neuropsychiatric Inventory scores than patients with AD and exhibited more apathy, disinhibition, euphoria, and aberrant motor behavior. The Neuropsychiatric Inventory accurately assigned 77% of patients with FTD and 77% of patients with AD to the correct diagnostic group using disinhibition, apathy, and depression. Patients with FTD had higher levels of disinhibition and apathy with relatively lower levels of depression compared with patients with AD. CONCLUSIONS: The Neuropsychiatric Inventory provides a behavioral profile that differentiates patients with FTD from patients with AD. Patients with FTD are more behaviorally disturbed but are often less depressed than patients with AD relative to their level of apathy.

Aged↗
PubMed1996

Selection against mutant alleles in blood leukocytes is a consistent feature in Incontinentia Pigmenti type 2.

Incontinentia Pigmenti 2 (IP2) is an X-linked dominant disorder with male lethality. Affected females display a characteristic skin eruption that evolves through four classic stages, frequently accompanied by dental and retinal abnormalities. Non-random (skewed) X-inactivation in peripheral blood leukocytes and in fibroblasts has been observed in females with IP2; however, sample sizes have been small and methods of analysis varied. We have examined X-inactivation in a large group of multigenerational IP2 families, in smaller families, and in isolated cases. Ninety-eight percent of affected females in multigenerational IP2 pedigrees show completely skewed patterns of X-inactivation, while only approximately 10% of a normal control population is skewed. Results both in small families and in new mutation cases with subsequent segregation consistent with Xq28 linkage are similar. Isolated cases show a lower percentage (85%) of skewed affected individuals; this difference may be due to inaccurate clinical ascertainment. The parent of origin of new mutations could be determined in 15 families; paternal new mutations were twice as common as maternal. Fibroblast subclones from a biopsy at the boundary of a skin lesion in a newborn IP2 patient were isolated, and clones with either one or the other X active were identified, demonstrating that cells with the active disease-bearing X chromosome are still present in stage I skin lesions.

Adult↗
PubMed1996

C1-C2 fusion in children with atlantoaxial instability and spinal cord compression: technical note.

Children with atlantoaxial instability and subluxation that will not reduce with extension of the cervical spine often present with clinical and/or radiographic evidence of spinal cord compression at the C1-C2 level. If the patient requires a laminectomy at the C1 level, occipital fusion is necessitated with associated reduction in mobility and high incidence of nonunion, as compared with a C1-C2 fusion. Also, passing wires under the intact lamina at C1 can further compromise an already compressed cervical cord. To address the problems associated with either of the above options, we used a C1-C2 fusion with removal of the mid 2 cm of the lamina of C1. Another option is the application of a transarticular screw fixation of C1-C2, which does not require the posterior element of C1. The current study was performed before the introduction of this type of fixation. Over a 10-year period, we have used the described technique to treat 13 patients. After decompression and fusion at the C1 and C2 levels as described, all patients had resolution of their preoperative neurological symptoms and all have achieved a stable fusion. Partial removal of the lamina at C1 and then C1-C2 fusion are safe and effective procedures to treat children with atlantoaxial instability and spinal cord compression.

Adolescent↗
PubMed1996

Use of methylprednisolone as an adjunct in the management of patients with penetrating spinal cord injury: outcome analysis.

OBJECTIVE: Since the results of the Second National Acute Spinal Cord Injury Study were published in 1990, methylprednisolone has become a mainstay in the treatment of nonpenetrating spinal cord injury. Although potential significant relationships between the prompt administration of high-dose methylprednisolone after blunt spinal cord injury and outcome have recently been addressed, the relationship between the prompt administration of high-dose methylprednisolone after penetrating spinal cord injury and outcome remain unanswered. METHODS: To explore this relationship, we performed a retrospective nonrandomized study on a series of 252 patients with penetrating missile injuries to the spine who presented to our institution from March 1980 to July 1993. One hundred eighty-one patients (71%) were treated conventionally without adjunctive steroid therapy before 1990. Sixteen patients followed up during the 13-year study period received steroid protocols that were not consistent with the Second National Acute Spinal Cord Injury Study protocol and were excluded from the study. Since 1990, 55 patients (21%) were treated with intravenous methylprednisolone according to the Second National Acute Spinal Cord Injury Study protocol. All patients were subsequently transferred for rehabilitative care, and prospective evaluations of their neurological status were performed at admission and discharge. RESULTS: The study included 236 men and 16 women (mean age, 25.6 yr). The mean duration of stay for initial hospitalization was 94.6 days, and the mean duration of stay in rehabilitation was 78.6 days. Frankel scores were used to assess outcome (P < 0.05) and were assessed at admission and at the time of definitive discharge from the Spinal Cord Injury Care System. The hypothesis that methylprednisolone therapy significantly improves functional outcomes in patients with gunshot wound injuries to the spine was rejected. Only the total number of days in rehabilitation and the degree of neurological injury at admission contributed significantly to explaining outcome at discharge. CONCLUSION: The administration of methylprednisolone did not significantly improve functional outcomes in patients with gunshot wound injuries to the spine or increase the number of complications experienced by patients during their hospitalizations.

Adolescent↗
PubMed1996

Comparison of short courses of oral prednisolone and fluticasone propionate in the treatment of adults with acute exacerbations of asthma in primary care.

BACKGROUND: Oral corticosteroids used in short courses for acute asthma are regarded as safe, although the frequent use of these drugs may result in patients suffering from systemic side effects. It has become common practice for patients to increase their own inhaled corticosteroid intake when their asthma goes out of control, but it has never been established whether a high dose of inhaled corticosteroid can be as effective as a short course of oral corticosteroid in the treatment of acute exacerbations. METHODS: A multicentre, randomised, double blind, double dummy, parallel group study was undertaken to determine whether the introduction of a high dose of inhaled fluticasone propionate (2 mg daily) is as effective as a short reducing course of oral prednisolone (starting at 40 mg/day and reducing by 5 mg every other day) in the treatment of acute exacerbations of asthma not considered severe enough for admission to hospital but requiring treatment with oral corticosteroid. RESULTS: Four hundred and thirteen adult asthmatic subjects who presented to their general practitioner with an acute exacerbation of asthma were recruited in 47 general practices in the United Kingdom. Treatment failures, defined as a reduction in peak expiratory flow (PEF) to below 60% of the patient's best/predicted value on two consecutive occasions or persistent symptoms with no improvement on three consecutive days, occurred in 23% of patients who received oral prednisolone and 27% who received inhaled fluticasone propionate (difference in percentage of treatment failures 4.3, 95% CI -4.1 to 12.8, p = 0.31). In each group 48% were classified as treatment successes, defined as a 10% or greater increase in percentage best/predicted morning PEF. Both treatments were equally well tolerated. CONCLUSIONS: There is no evidence of a significant difference in efficacy between a reducing dose course of oral prednisolone and high dose inhaled fluticasone propionate in mild exacerbations of asthma which do not require admission to hospital.

Acute Disease↗
PubMed1996

Surgical treatment of 95 children with 102 intracranial arachnoid cysts.

Our review of 95 children with 102 intracranial arachnoid cysts treated surgically from 1976 to 1996 is presented. These patients are divided into two groups, those initially treated from 1976 to 1986 and those treated from 1987 to May 1996, to see whether any improvement in outcome had occurred. There were 31 patients (20 males/11 females) with 34 cysts treated from 1976 to 1986 and 64 patients (45 males/19 females) with 68 cysts treated from 1987 to 1996. The mean age at presentation for all cases was 4.9 years (range from 3 days to (7.8 years). The most common cyst location was the middle fossa. Treatment options for the management of intracranial arachnoid cysts include fenestration or shunting. We consider the avoidance of a shunt as a primary goal in the management of these patients. Accordingly, 82 (80%) of the cysts in our series were treated initially by fenestration. Fifty percent of those initially fenestrated from 1976 to 1986 required no further treatment as compared with 60% fenestrated from 1987 to 1996. The success rate of fenestration among those patients without associated hydrocephalus was significantly higher than in those with hydrocephalus; 73% without hydrocephalus required no additional treatment versus 32% with hydrocephalus. Seventeen percent of the patients treated from 1976 to 1986 required a subsequent ventriculoperitoneal shunt as compared with 22% treated from 1987 to 1996. Twenty-nine percent of those patients initially fenestrated from 1976 to 1986 required a subsequent cystoperitoneal shunt as compared with only 12% treated from 1987 to 1996. Although not statistically significant, the second group of patients had a lower rate of reoperation, fewer complications, and a better clinical outcome than the first group. We recommend that in patients without evidence of hydrocephalus, cyst fenestration be considered as the primary procedure, as 73% of the patients in the two series remain shunt free. In those patients with hydrocephalus, we still recommend cyst fenestration, but with a ventriculoperitoneal shunt inserted before fenestration if the hydrocephalus is marked or after fenestration if the hydrocephalus is progressive.

Adolescent↗
PubMed1996

Longitudinal assessment of symptoms of depression, agitation, and psychosis in 181 patients with Alzheimer's disease.

OBJECTIVE: The goal of this study was to define the recurrence or continuation of neuropsychiatric symptoms in patients with Alzheimer's disease who were observed serially for a 1-year period. METHOD: One hundred eighty-one patients with probable Alzheimer's disease were assessed five times at 3-month intervals with a standardized neuropsychiatric rating instrument. RESULTS: Recurrence rates of neuropsychiatric symptoms during the 1-year period were 85% for depression, 93% for agitation, and 95% for psychosis. Symptom frequency at any point in time underestimated the cumulative 1-year frequency. Recurrence rates were significantly greater among patients who had multiple symptoms. Women exhibited more symptoms than men. Patients in the oldest age group (76-87 years) had more psychosis, less depression and agitation, and slower cognitive decline. Psychosis was associated with more rapid cognitive decline, and agitation was associated with more rapid functional deterioration. CONCLUSIONS: These results indicate that once psychiatric symptoms are present in patients with Alzheimer's disease, they frequently recur. These symptoms vary with age, sex, and rate of illness progression.

Age Factors↗
PubMed1996

Surveillance imaging in children with primitive neuroectodermal tumors.

Controversy surrounds the benefits of routine surveillance magnetic resonance or computed tomographic imaging for monitoring children after resection of primitive neuroectodermal tumors. A recent study suggested that serial imaging studies detect only a small minority of tumor recurrences in patients with symptoms. The authors concluded that even in patients with recurrence documented by imaging, no patient with a recurrence survived long (average, 5 mo) and that surveillance scanning is of little clinical value in children with primitive neuroectodermal tumors. We reviewed our experience with 25 patients (28% of our total series) who presented to the Children's Hospital Los Angeles, Los Angeles, CA, from 1985 to 1993 with recurrent tumors after surgery and adjuvant therapy. Recurrent tumors were detected on routine imaging in 19 asymptomatic patients (76%) and in 6 symptomatic patients (24%). Recurrences were documented 15 months (mean) after the initial diagnosis in asymptomatic children and 5 months (mean) after the initial diagnosis in children with symptoms of recurrent tumor (P < or = 0.01). Asymptomatic patients with recurrence documented on serial imaging had prolonged survival when compared with those who were symptomatic (P < or = 0.05). The surviving patients with recurrence remained alive for more than 24 months after documentation of recurrence. Early detection of local tumor recurrence by surveillance scanning may provide a critical therapeutic window for successful treatment with aggressive or novel therapies.

Adolescent↗
PubMed1995

Graft-versus-host disease in children.

With the continued use of bone marrow transplantation in the treatment of malignancies, immunodeficiencies, and some metabolic diseases in children, it remains important for the dermatologist to be aware of the clinical and diagnostic features, and pathophysiology of Graft vs. Host disease. Specifically, the dermatologist should keep in mind that the skin is a primary site for involvement in this process. Recognition of the spectrum of cutaneous disease, its methods of laboratory diagnosis, and treatment should be known to those physicians caring for patients undergoing this type of therapy. A brief review of these and other aspects of Graft vs. Host disease is presented in this report.

Bone Marrow Transplantation↗
PubMed1995

Gonadal mosaicism for incontinentia pigmenti in a healthy male.

Incontinentia pigmenti (IP) is a genodermatosis that segregates as an X linked dominant trait with male lethality. The disease has been linked to Xq28 in a number of studies. A few affected males have been documented, most of whom have a 47,XXY karyotype. We report a family with two paternally related half sisters, each affected with IP. The father is healthy, clinically normal, and has a 46,XY normal male karyotype. Linkage analysis of 12 polymorphic markers (two X linked and 10 autosomal) confirms paternity. X inactivation studies with the human androgen receptor (HUMARA) indicate that the paternal X chromosome is inactivated preferentially in each girl, implying that this chromosome carries the IP mutation, and that the father is a gonadal mosaic for the IP mutation.

Abortion, Habitual↗