Congenital cutaneous and subcutaneous nodule.
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Biomedical subjects
Publications and source records attributed to M L Levy.
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Radiation-induced tissue damage in the central nervous system is a well-known complication of interstitial brachytherapy for brain tumors, yet imaging correlates have historically been based upon computed tomographic scans. We now present magnetic resonance imaging (MRI) to correlate radiation changes after interstitial brachytherapy with the histopathology. The central nervous system of a 38-year-old man with a left frontal cerebral glioma diagnosed by stereotactic biopsy was treated with interstitial brachytherapy (iridium-192, 47 Gy), followed by limited-field irradiation (45 Gy). With progressive deterioration, a second biopsy demonstrated radiation changes. Despite aggressive medical management, the patient died 9 months after completion of radiation therapy. Postmortem evaluation compared MRI scans of the intact, fixed brain with the histopathology. Axial sections (10 mm) obtained by MRI scan and autopsy brain slices were cut in the identical plane. Neuroimaging and histopathological findings of the lesion correlated within 2 to 3 mm resolution. In the peripheral white matter, MRI scan did not indicate the extent of radiation effect histologically. We suggest that MRI has limited utility in assessing the extent of impact of radiation on surrounding brain.
We examined the relationships between specific magnetic resonance imaging features and certain gross and microscopic characteristics of meningiomas, including vascularity, gross texture (consistency), and venous sinus involvement. Magnetic resonance imaging scans, surgery reports, and the histopathological findings of tumors were examined retrospectively in 54 patients. Sinus involvement was accurately predicted on T1-weighted images in 9 of 10 cases (P = 0.001) and tumors with cystic changes in 3 of 3 cases. T1-weighted images were not useful for predicting vascularity unless actual flow voids could be visualized (five of six cases). There was no correlation between T1 signal intensity, tumor consistency, or histological findings. In tumors without detectable vascularity on T1-weighted images, hyperintensity relative to gray matter on T2-weighted images was correlated with increased vascularity (P = 0.004). Tumors with soft consistency (P = 0.007), cellular atypia, invasion, angioblastic, or melanocytic components were also hyperintense, compared with gray matter on T2-weighted images (P = 0.0266). Aggressive meningiomas were found to be more vascular (P = 0.045). No correlation was found between the degree of surrounding edema or contrast enhancement with histopathological findings, vascularity, or consistency.
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BACKGROUND: Chickenpox, the primary infection caused by the varicella-zoster virus, affects more than 3 million children a year in the United States. Although usually self-limited, chickenpox can cause prolonged discomfort and is associated with infrequent but serious complications. METHODS: To evaluate the effectiveness of acyclovir for the treatment of chickenpox, we conducted a multicenter, double-blind, placebo-controlled study involving 815 healthy children 2 to 12 years old who contracted chickenpox. Treatment with acyclovir was begun within the first 24 hours of rash and was administered by the oral route in a dose of 20 mg per kilogram of body weight four times daily for five days. RESULTS: The children treated with acyclovir had fewer varicella lesions than those given placebo (mean number, 294 vs 347; P less than 0.001), and a smaller proportion of them had more than 500 lesions (21 percent, as compared with 38 percent with placebo; P less than 0.001). In over 95 percent of the recipients of acyclovir no new lesions formed after day 3, whereas new lesions were forming in 20 percent of the placebo recipients on day 6 or later. The recipients of acyclovir also had accelerated progression to the crusted and healed stages, less itching, and fewer residual lesions after 28 days. In the children treated with acyclovir the duration of fever and constitutional symptoms was limited to three to four days, whereas in 20 percent of the children given placebo illness lasted more than four days. There was no significant difference between groups in the distribution of 11 disease complications (10 bacterial skin infections and 1 case of transient cerebellar ataxia). Acyclovir was well tolerated, and there was no significant difference between groups in the titers of antibodies against varicella-zoster virus. CONCLUSIONS: Acyclovir is a safe treatment that reduces the duration and severity of chickenpox in normal children when therapy is initiated during the first 24 hours of rash. Whether treatment with acyclovir can reduce the rare, serious complications of chickenpox remains uncertain.
Management of craniopharyngiomas and masses affecting the third ventricular chamber represents one of the most challenging problems confronting contemporary neurological surgeons. Given the devastating sequelae of surgical complications involved in approaches to the deep cerebral midline, surgical management requires a combination of sophisticated imaging, diagnostic pathology and surgical technique including ventricular microsurgery and stereotaxy and its attendant refinements. Surgical and non-surgical management is based upon the structural presentation of these masses as defined by detailed imaging studies. Operative objectives include histological definition, maximally feasible excision, cerebral spinal fluid diversion and relief of neurologic deficits created by masses affecting the third ventricular chamber.
A wide variety of disorders involving the scalp and hair exist that may be encountered in the pediatric and adolescent population. An approach to the differential diagnosis of many of these conditions can be considered once the patient is found to have either alopecia, scaling, or tumors of the scalp. Certainly other causes of scalp or hair disease exist but the more common disorders can be appropriately considered in this manner.
Cefaclor is an oral semisynthetic cephalosporin that is popular in the treatment of infections in children. This drug has been associated with a serum sickness--like reaction characterized by an urticarial eruption, pruritus, arthritis, and/or arthralgias. We report 12 additional cases of cefaclor-related serum sickness--like reactions in children. All patients improved after discontinuation of the drug and no long-term complications resulted.
A retrospective analysis was performed on all aneurysms operated on by one of us (SLG) from July 1980 to October 1988 to determine the factors that govern outcome from the intraoperative rupture of aneurysms. A total of 276 consecutive surgical procedures for 317 intracranial aneurysms produced 41 perioperative or intraoperative ruptures for analysis. Five cases were pre-exposure ruptures, 3 of which occurred during anesthetic induction. Four of these patients died, and 1 made a good recovery. Of the remaining 36 cases, outcome was analyzed in terms of the adjuncts used to deal with the intraoperative rupture. There was no statistically significant difference in outcome between those cases in which tamponade was used to control hemorrhage versus temporary clipping; however, those cases in which hypotension was used did less well than those in which it was not used. From October 1986 to October 1988, 108 operations for 132 aneurysms were performed without the use of induced hypotension. There were 16 intraoperative ruptures (14.8%). All 16 of these patients made a good recovery. In the group before 1986, of which there were 20 intraoperative ruptures (of 168 operations, 11.9%), 11 of those 20 patients suffered a permanent deficit or died. We conclude that hypotension may not be a necessary adjunct to the management of intraoperative rupture of aneurysms.
The effect of hypervolemic preload enhancement on cardiac performance was systematically analyzed in nine patients following aneurysmal subarachnoid hemorrhage. The patients ranged in age from 34 to 63 years, and none had a history of cardiac disease. Each patient underwent placement of a flow-directed balloon-tipped catheter and the following measurements were taken during hypervolemic therapy: pulmonary artery wedge pressure (PAWP), central venous pressure (CVP), cardiac index (CI), stroke volume index (SVI), and left ventricular stroke work index (LVSWI). After baseline measurements were recorded, hetastarch or plasmanate was infused intravenously at 300 cc/hr. Thermal output determination and pressures were measured every 15 minutes. The PAWP did not correlate in a statistically significant fashion with the CVP in the ranges recorded; however, a statistically significant correlation did exist between PAWP increases and increases in CI, SVI, and LVSWI (p less than 0.01). There was no statistical correlation between PAWP increases above 14 mm Hg and improvement in cardiac performance as evidenced by CI, SVI, and LVSWI measurements. It is concluded that CVP is an unreliable index of cardiac performance during hypervolemic therapy and that, in previously healthy individuals, a PAWP of 14 mm Hg is associated with maximum cardiac performance.
A case report of monostotic fibrous dysplasia of the clivus in a postadolescent woman is described. Although fibrous dysplasia of craniofacial structures is well documented, involvement of the clivus has not been reported. Diagnosis by clinical, radiographic, and histopathological features is detailed. Implications for the role of surgery and management are discussed.
Intestinal morphology and fluid and electrolyte transport were examined in a neonatal porcine model of cryptosporidiosis. Sections of jejunum, ileum, and colon were obtained for morphometric analysis on days 3, 6, 9, and 12 postinfection, and in vivo perfusion studies of jejunum and ileum were conducted on days 3 and 4 postinfection. The most severe morphologic lesion was seen in the ileum on day 3, and consisted of villous atrophy, crypt hyperplasia, and cellular infiltration. Villous surface area was reduced from 2.1 +/- 0.4 x 10(5) microns2 in control ileum to 0.8 +/- 0.1 x 10(5) microns2 in infected ileum, a result associated with enterocytes that were fewer in number and reduced in cross-sectional area. Conversely, the number of inflammatory cells in the lamina propria of the villus increased from 456 +/- 116 in control to 1014 +/- 187 in infected villus without a significant change in the volume of the lamina propria. At the height of infection, there was an approximate 1:2 ratio of both organisms and inflammatory cells to villous enterocytes. In contrast, organisms were not observed in the crypts, and the concentration of inflammatory cells in crypt lamina propria was unaltered. Disappearance of organisms and polymorphonuclear cells from the ileum was associated with restoration of normal structure and was complete by day 12. Although organisms were seen in the colon, the general architecture was not severely affected. On days 3 and 4 postinfection, there was a complete impairment of the glucose-stimulated Na and water absorption in both jejunum and ileum of infected pigs; however, absorption of electrolytes and water from a basic Ringer's solution, in the absence of glucose, was not significantly affected. These results are consistent with a malabsorptive diarrheal disease associated with the morphological damage and are very similar to those seen in enteric viral disease in pigs, except that the upper intestine is more severely affected in the latter.
A distinctive photodermatitis developed in 22 children who had been receiving naproxen for prolonged periods. The eruption was marked by erythema, vesiculation, or increased skin fragility characterized by easy scarring of sun-exposed skin. Results of biochemical studies for porphyria were normal, and other causes of photosensitivity were believed to be unlikely. Of the 22 patients, 21 had juvenile rheumatoid arthritis; one patient had systemic lupus erythematosus. Twenty of the patients had fair skin and blue eyes. In each case, all findings except scarring resolved when naproxen was discontinued. Attention must be paid to complaints suggesting photosensitivity in children receiving naproxen.
A case of pyoderma gangrenosum (PG) in a 14-year-old boy with acute myelogenous leukemia (AML) is described. The onset of pyoderma gangrenosum coincided with the relapse of AML. The lesions responded dramatically to treatment with oral prednisone despite the persistence of leukemia. Pyoderma gangrenosum should be included in the differential diagnosis of any nodular, pustular, or necrotic cutaneous eruption in children with leukemia.
The authors discuss a comprehensive management program for patients with cerebral vasospasm following subarachnoid hemorrhage in which therapeutic manipulation and meticulous monitoring of a number of physiologic determinants of cardiac function are employed. Ventricular preload is most significantly affected by hypervolemic therapy, but alternate methods of reversing ischemic neurologic deficits with various inatropic agents are discussed also.
PURPOSE: To describe and characterize the association of hereditary cutaneous lichen amyloidosis with multiple endocrine neoplasia type 2a (MEN 2a). DESIGN: Survey of a family for two diseases. SETTING: Evaluation of patients at a clinical research center. PATIENTS: Nineteen family members with MEN 2a. MEASUREMENTS AND MAIN RESULTS: In this family cutaneous lichen amyloidosis presented as multiple infiltrated papules overlying a well-demarcated plaque in the scapular area of the back (right or left). Immunohistochemical studies showed amyloid that stained for keratin but not calcitonin. Three family members had the characteristic skin lesion and also carried the gene for MEN 2a; two additional members carried the gene for MEN 2a, but did not manifest the observable skin changes associated with lichen amyloidosis. CONCLUSIONS: From the findings in this kindred and in another recently reported but unrelated family with an identical type of pruritic skin rash and MEN 2a, several conclusions can be drawn. First, the syndrome of cutaneous amyloidosis and MEN 2a is a clearly defined autosomal dominant hereditary syndrome. Second, the dermal amyloid appears to be caused by deposition of keratin-like peptides rather than by calcitonin-like peptides. Third, known families with hereditary lichen amyloidosis should be screened to determine the true frequency of this syndrome.
Multiple endocrine neoplasia type 2A (MEN 2A) is a rare hereditary disease transmitted in families as an autosomal dominant trait. We have identified a family in which the expression of a rare autosomal dominant form of cutaneous lichen amyloidosis appears to cosegregate with MEN 2A. In this family the skin lesion presented as multiple infiltrated papules overlying well demarcated plaques over the scapular area (right or left). Immunohistochemical studies demonstrated amyloid which stained for keratin but not calcitonin. A total of 19 members were screened. Three members of the family have the characteristic skin lesion and MEN 2A; two additional members have MEN 2A but have not manifested observable skin changes of lichen amyloidosis. Another unrelated Italian family with a similar type of pruritic skin rash and MEN 2A has been reported recently. Although the initial skin biopsies were negative for amyloidosis, subsequent biopsy established the association of MEN 2A with amyloidosis in this family also. When these kindreds are combined, several conclusions can be drawn. First, the syndrome of cutaneous amyloidosis and MEN 2A appears to be a clearly defined autosomal dominant hereditary syndrome. Whether this syndrome can be linked to chromosome 10 is not yet known. Second, the dermal amyloid appears to be caused by deposition of keratin-like peptides rather than calcitonin-like peptides. Third, we believe that patients with the hereditary form of cutaneous amyloid should be screened for medullary thyroid carcinoma to determine the true frequency of this syndrome.