Biomedical subjects
Publications and source records attributed to G Alper.
Behçet's disease is a chronic, relapsing multisystem disorder, and nervous system involvement is one of the serious manifestations. Neuro-Behçet is rarely reported in children and may present with a wide variety of symptoms because the entire neuraxis may be affected. A case of cerebral vein thrombosis secondary to Behçet's disease is presented. The patient has recovered without any visual loss and had no complaints at 1-year follow-up.
The present study evaluated the effect of dietary vitamin E supplementation (1,000 mg/kg chow) on the alterations in vascular reactivity of streptozotocin-diabetic aorta of Wistar rats. After 12 weeks of treatment, thoracic aortic rings of rats were mounted in organ baths and contractile responses to phenylephrine and 5-hydroxytryptamine and relaxant responses to acetylcholine, calcium ionophore and sodium nitroprusside were assessed. Plasma vitamin E concentration as measured by HPLC was markedly decreased in diabetic rats and increased with dietary vitamin E supplementation. Induction of diabetes significantly impaired endothelium-dependent relaxations to acetylcholine and calcium ionophore in aortic rings, but did not change endothelium-independent relaxation to sodium nitroprusside. Vitamin E significantly improved the impaired endothelium-dependent relaxations, further it decreased the enhanced contractile response to phenylephrine and 5-hydroxytryptamine in diabetic rings. The mechanical denudation of endothelium or the chemical inhibition of endothelium-dependent relaxation with N(omega)-nitro-L-arginine methyl ester (100 micromol/l) significantly increased phenylephrine contractility in control rings and the rings of diabetic rats treated with vitamin E; such a difference was not observed in diabetic rats fed with normal diet. Liver and lung malondialdehyde concentrations, as an index of lipid peroxidation, were increased in diabetic rats and significantly decreased with vitamin E supplementation. It is concluded that dietary supplementation of vitamin E improved endothelial dysfunction in insulin-dependent model of uncontrolled diabetes, probably decreasing membranal lipid peroxidation.
The aim of this study was to document the magnetic resonance imaging (MRI) findings of cases with a history of severe neonatal indirect hyperbilirubinemia. Ten cases (eight cases with neurologic findings, two normal cases) with a history of severe neonatal indirect hyperbilirubinemia were studied. Neurologic findings and MRI results were described and correlated. Seven of eight cases with neurologic findings demonstrated symmetric and uniform increased T2 signal changes limited to globus pallidi. MRI scans of two cases without neurologic findings showed no abnormality. Severe neonatal indirect hyperbilirubinemia should be considered in the differential diagnosis of bilateral symmetric hyperintense signal changes in the globus pallidus on MRI. However, high levels of unconjugated bilirubin concentrations in the neonatal period may not always cause such lesions of globus pallidus on MRI despite the presence of neurologic findings.
A 4 year-old boy with mental retardation and seizures presented to the pediatric endocrinology clinic because of a history of hypoglycemia; a 16 month-old girl with developmental delay presented with bilateral breast tissue enlargement; in both, a diagnosis of Kabuki syndrome was made because of typical facial features, neurodevelopmental delay and other stigmata consistent with Kabuki syndrome. Kabuki syndrome is a mental retardation-malformation syndrome affecting multiple organ systems with a broad spectrum of abnormalities. The facial features of the syndrome are specific and independent of ethnic origin. In addition to presenting with endocrine problems, the patients reported here exhibit some novel findings such as congenital alopecia areata and hyperpigmented skin lesion. The diagnosis of Kabuki syndrome should be considered in patients with hypoglycemia or premature thelarche when associated with developmental delay and a peculiar facies.
This study examined the effectiveness of dialectical behavior therapy (DBT) in the treatment of people diagnosed with borderline personality disorder (BPD). The frequency of self-injurious behaviors of 15 women hospitalized on a DBT unit was traced over a 4-week period. Data on self-injurious behaviors and the use of the DBT approach were collected from a review of incident reports and patients' charts. The self-injurious behaviors decreased by almost 50%. In addition, four RNs, who were involved on the DBT unit both before and after the institution of the treatment approach, were interviewed. Semi-structured interviews were conducted to determine the nurses perceptions of the effectiveness of DBT. Their responses were uniformly positive. The most commonly occurring phrase was "it works."
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Reported are two patients presenting with both thrombocytopenia and sagittal sinus thrombosis. The first patient is a 42-month-old male with no identified thrombophilic risk factors who developed acute neurologic symptoms after an acute infection. The second patient is a 22-month-old female with no history of preceding infection but had a positive lupus anticoagulant test. She also developed deep venous thrombosis and was treated with intravenous heparin. Both patients are currently doing well without neurologic deficits. To the authors' knowledge the second patient is the youngest reported patient with cerebral vein thrombosis associated with thrombocytopenia and lupus anticoagulant. These observations call attention to the need for a thorough investigation of thrombophilic risk factors in pediatric patients with thrombotic complications.
The process of aging presents itself with various alterations in physiological events. Among many theories, the free radical (FR) theory of aging which reflects the FR damage to cellular components is accepted as one of the most important theories. Recently, the increases in catecholamine metabolism in aging have also attracted attention, and monoamine oxidase (MAO), a key enzyme in this process has been extensively studied. The aim of this study was to assess the role of FR species via MAO, a possible source of FRs, in physiological aging by determining the lipid peroxidation products (LPP) (malondialdehyde, diene conjugates) and antioxidant enzyme levels (superoxide dismutase (SOD) and catalase (CAT) in young (3 months old, n=10) and aging (16-18 months old, n=10) rat brain tissues of Swiss male albino rats. In the second part of the study, the same parameters were determined after the acute administration of MAO inhibitors (deprenyl and pargyline, 25 mg/kg i.p.) to investigate whether these agents have any beneficial effects in reducing oxidant stress via inhibition of MAO. In old rat brains, MAO activities showed a significant increase (P=0.000) in addition to an insignificant increase in LPP, while SOD (P=0.007) and CAT activities showed a decrease with advancing age. After the acute administration of both deprenyl and pargyline, a significant decrease in the MAO activities of both young (P=0.0002 for each) and aging rats (P=0.0002 for deprenyl and P=0.0001 for pargyline) were observed. It was noted that deprenyl causes a significant increase in CAT activity (P<0.05) but a significant decrease in SOD activity (P<0.05) in young rats, while it causes only a significant increase in SOD activity in aging rats (P<0.05). Both deprenyl and pargyline cause a significant decrease in conjugated diene levels of aging rats (P<0.05). These results confirm the role of catecholamine oxidation and MAO activity as one of the causative factors in increased oxidant stress during aging. By reducing the oxidant stress observed in aging brain, MAO inhibitors, especially deprenyl, may contribute to the control of the aging process.
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Benign macrocephaly of infancy is a common problem in the child neurology practice. The radiologic features of this entity are not well defined. In most of the previous studies, macrocephalic patients were evaluated by computed tomography. To define the radiologic characteristics of this entity, 20 children with macrocephaly with normal neurologic examinations were enrolled in the study. All the patients were evaluated by magnetic resonance imaging studies. Sixty-five percent of patients had enlargement of the subarachnoid space and 35% of patients had megalencephaly. None of the patients had subdural collections. The mean age of patients with enlargement of the subarachnoid space was found to be younger than those with megalencephaly. The cases with parental histories of macrocephaly demonstrated both enlargement of the subarachnoid space and megalencephaly. Our results suggest that the radiologic finding of benign macrocephaly can be both enlargement of the subarachnoid space and megalencephaly.
Mortality is high in patients with fulminant hepatic failure (FHF). Neurologic complications of encephalopathy and cerebral edema are major contributors to mortality. Orthotopic liver transplantation has improved survival in these patients. However, the complexity of medical and surgical problems in this patient population, coupled with a severe shortage of organs, requires careful patient selection. The aim of this study was to describe the neurologic outcome of children with FHF who developed radiologically apparent cerebral edema. The hospital and outpatient records and radiologic studies of 20 children with FHF admitted to Children's Hospital of Pittsburgh from 1981-1995 who developed encephalopathy and computed tomographic evidence of cerebral edema were reviewed. Fourteen patients died (70%), three were left with severe neurologic deficits (15%), and three were left with moderate deficits (15%). Survival was correlated with a lesser degree of coma. Histopathologic examination of eight brains demonstrated cerebral edema and widespread ischemic neuronal necrosis in all eight. The presence of radiographic cerebral edema in children with FHF is an objective measure that indicates a very poor prognosis. Termination of care is a reasonable option. Comprehensive monitoring of cerebral function and intracranial pressure is required in children with FHF. Orthotopic liver transplantation should be performed in children with severe and worsening encephalopathy before the development of radiographically apparent cerebral edema.
The process of aging presents itself with various alterations in physiological events. Although the turnover of catecholamines increases with aging, there is a lack of response to catecholamines in target tissues. One of the key enzymes in catecholamine metabolism is monoamine oxidase. It has been suggested that tissue and serum monoamine oxidase activities show pathological alterations in various diseases while physiological fluctuations can also be detected in normals. The aim of this study is to determine the sex and age related changes of platelet and serum monoamine oxidase in healthy volunteers. In this study, 75 healthy volunteers of different ages (21-80 a) and sexes (40 females, 35 males) were included. Serum and platelet monoamine oxidase determinations were performed spectrophotofluorometrically by Tufvesson's (Scand J Clin Lab Invest 1970; 26:151-4) and Kraml's (Biochem Pharmacol 1965; 14:1684-6) modified methods, respectively. While there was no significant difference in serum monoamine oxidase activities related to age and sex, platelet monoamine oxidase manifested a significant increase in females compared to males (p < 0.05) and the mean values in both sexes showed an increase with age (p < 0.001). The results of this study imply that platelet monoamine oxidase shows an age related increase which is more prominent in females.
Castleman syndrome (giant lymph node hyperplasia) is a rare, heterogeneous lymphoproliferative disorder of unknown etiology and pathogenesis. Most cases occur as mediastinal masses, although extrathoracic involvement including nodal and extranodal locations have been reported. The localized variants (solitary lesions) respond well to surgical excision. We report a 10-year-old boy who presented with headache, intermittent fever, and progressive weakness of his legs. MRI imaging showed an enhancing epidural mass with impingement on the spinal cord at the C6-T2 level. Other laboratory abnormalities included anemia, hypergammaglobulinemia, increased erythrocyte sedimentation rate, and cerebrospinal fluid pleocytosis with slightly increased cerebrospinal protein. The mass was partially resected and the histopathology showed lymphoplasmocytic infiltration compatible with Castleman syndrome. There was no evidence of malignancy. Castleman syndrome is the most likely diagnosis in the presence of the associated systemic findings, although the epidural site for lymphoplasmocytic inflammation is atypical.
We describe a case of listerial rhombencephalitis in a previously healthy 40-year-old man. The diagnosis was based on the clinical findings, results of cerebrospinal fluid analysis, blood culture, and MR imaging findings. The treatment was started before culture results were available, and the patient had a full clinical recovery.
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This study investigated hemodynamic and sedative effects of a single dose of the selective alpha-2 adrenoceptor agonist dexmedetoimidine (DMED) in isoflurane-anesthetized dogs. DMED (20 micrograms/kg i.v. 2-min infusion) was given to all dogs. In Group 1 the effects of DMED (time control; N = 10) were studied over 4 hr. In Group 2 (N = 11) glycopyrrolate (40 micrograms/kg initial dose followed by 20 micrograms/kg repeated every 30 min) was used to modulate the DMED-induced vagally mediated changes in heart rate. In Group 3 (N = 8), two doses of nifedipine were used to offset the DMED-induced increase in arterial blood pressure, low dose nifedipine = 10 micrograms/kg bolus followed by 2.5 micrograms/kg/min infusion for 20 min, high dose nifedipine = 20 micrograms/kg bolus followed by 5 micrograms/kg/min infusion for 20 min. DMED administration reduced isoflurane anesthetic requirements by 89% at 30 min and by 50% at 4 hr. Maximum increase in mean arterial blood pressure (MABP) +67 mm Hg occurred 1 min after DMED. MABP remained significantly elevated throughout the 4 hr studied (about +20%). Concomitant with the transient peak in MABP, heart rate (129 +/- 6 to 60 +/- 8 bpm) and cardiac output (3.5 +/- 0.3 to 0.9 +/- 0.1 l/min) decreased, whereas systemic vascular resistance (2460 +/- 210 to 14,700 +/- 1330 dynes.sec.cm-5) and left ventricular end diastolic pressure (4 +/- 1 to 27 +/- 4 mm Hg) increased.(ABSTRACT TRUNCATED AT 250 WORDS)
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