Search PubMed⌕ Search

Biomedical subjects

J Calleja

Publications and source records attributed to J Calleja.

At least 19 recordsLinked to original sources
PubMed2006

Necrotizing leukoencephalopathy associated with nonconvulsive status epilepticus and periodic short-interval diffuse discharges: a clinicopathological study.

We describe the clinical, neuroimaging and neuropathological features of an immunocompromised patient diagnosed as having refractory nonconvulsive status epilepticus (NCSE), and whose consecutive electroencephalograms (EEGs) revealed persistent periodic short-interval diffuse discharges (PSIDDs). Prominent subcortical white matter lesions in keeping with the diagnosis of multifocal necrotizing leukoencephalopathy may be neuropathological substrate of NCSE with persistent PSIDDs.

Brain↗
PubMed2003

Autonomic neuropathy in end-stage cirrhotic patients and evolution after liver transplantation.

Autonomic neuropathy (AN), which is frequently observed in cirrhosis patients, has been associated with a higher mortality. We have prospectively evaluated the prevalence of AN, its relationship with the degree of liver dysfunction and circulatory disturbances, and the evolution of AN after liver transplantation (LT) in 62 end-stage liver cirrhosis patients. AN was evaluated by seven cardiovascular tests assessing sympathetic or parasympathetic function before and 6 months after LT. Patients were classified as showing absent (A), early (E), or definite dysfunction (D). AN appeared in 67.7% of cases (E: 24.2%, D: 43.5%) without relation to liver disease etiology. Parasympathetic dysfunction was more prevalent than sympathetic dysfunction (59.7% vs. 20.9%). AN was significantly related to Child-Pugh score. Hyperdynamic circulation was more marked in the D than the A group as shown by a greater cardiac output (CO)(9 vs. 7.3 L/min) and a lower peripheral resistance (SVR)(666 vs. 866 dyn.s.cm(-5)). Moreover, AN scores significantly correlated with CO and SVR. Overall the prevalence of AN decreased 6 months after LT (67.7% vs 48%) due to a significant reduction in definite AN (43.5 vs. 14.8%; P<.05). AN improved in 70% of cases after LT. Sympathetic dysfunction remained in only one patient. We conclude that AN is frequent in liver transplant candidates; its severity is associated with the degree of liver failure. Systemic circulatory disturbances seem to correlate with the severity of AN. AN is clearly improved by LT. The evaluation of AN may contribute to a better selection of LT recipients.

Autonomic Nervous System Diseases↗
PubMed2003

Liver transplantation for fulminant and subfulminant hepatic failure.

AIMS: The aim of this study was to assess the long-term course and outcome after liver transplantation (LTX) for fulminant/subfulminant hepatic failure (FSHF) to determine which factors relate to outcome. PATIENTS AND METHODS: Between April 1990 and October 2002, 30 adult patients with FSHF underwent LTX. King's College criteria were used to decide which patients needed LTX. Pretransplantation parameters (age, sex, degree of hepatic encephalopathy, etiology, and time between onset of symptoms and LTX) were examined as risk factors for LTX outcome. RESULTS: Mean age at LTX was 40.4+/-13.9 years and 46.7% were men. The most frequent causes of FSHF were virus B in 23.3%, autoimmune hepatitis in 23.3%, and cryptogenic in 20%. Fifty percent of the patients with a survival longer than 15 days suffered episodes of acute rejection; chronic rejection occurred in 25%. One- and five-year patient and allograft survival rates for FSHF were 56.3% and 54.7%, respectively. Autoimmune hepatitis was the only factor associated with better posttransplantation outcome, although there were no differences in posttransplant course. Patient survival rates increased during the study period. During the first 5 years (1990-1995) the survival rates were 53.3% (1-year and 5-year), whereas they were 60% at 1 and 5 years in the second interval (1996-2002). CONCLUSIONS: The mortality rate of FSHF is high during the first year post-LTX. LTX for FSHF of autoimmune etiology showed better outcomes with increasing patient survival rates during the study period.

Adolescent↗
PubMed2003

Liver transplantation for autoimmune hepatitis: fulminant versus chronic hepatitis presentation.

AIMS: The aim of this study was to analyse long-term outcomes of patients with liver transplantation for autoimmune hepatitis and to determine if fulminant/subfulminant hepatic failure (FSHF) at presentation was a predictor of outcome after ortothopic liver transplantation. PATIENTS AND METHODS: Between April 1990 and October 2002, 18 patients with autoimmune hepatitis underwent 21 liver transplants. Three patients were excluded because of coexisting causes of hepatitis. Seven patients had FSHF and eight patients had chronic disease. The initial immunosuppressive regimen was cyclosporine based in 80%, and all but one patient received steroids or azathioprine. RESULTS: Mean age at time of transplant was 44.2+/-15.5 years. Patients were followed for 38.9+/-29.6 months. Five patients (33.3%) had seven episodes of acute rejection (two steroid-resistant). Three patients developed chronic rejection. One patient displayed histologically proven recurrent autoimmune hepatitis. Actuarial patient and graft survival rates at 1 and 5 years were 80% and 56% and 78.6% and 51%, respectively. No differences in the clinical characteristics of the patients, rates of acute or chronic rejection episodes, end biliary or arterial complications were observed between FSHF and chronic autoimmune hepatitis. The study suggests a better survival for autoimmune FSHF (P=.003). CONCLUSIONS: Liver transplant is indicated for patients displaying autoimmune chronic liver disease and FSHF with similar clinical courses at however, patients with FSHF at presentation had better survivals.

Acute Disease↗
PubMed2003

Autonomic neuropathy is associated with hemodynamic instability during human liver transplantation.

INTRODUCTION: End-stage liver disease is frequently associated with autonomic neuropathy (AN). The hemodynamic changes during liver transplantation (LT) require an adequate autonomic response to maintain cardiovascular stability. PATIENTS AND METHODS: Forty-one patients undergoing LT were evaluated for the influence of AN on the evolution after LT. AN was previously evaluated by seven cardiovascular tests assessing sympathetic (Sy) or parasympathetic (P) function. Patients were classified as absent (A), early (E), or definite dysfunction (D). A hemodynamic study was performed before and after vascular clampings. The analysis included the duration of LT, transfusion requirements, intra-operative artenal hypotensive episodes, incidence of postreperfusion syndrome (PRS), cardiac arrhythmias and vasoactive drug requirements. RESULTS: The hyperdynamic circulation worsened during surgery in D patients, as shown by a significantly increased cardiac output and a significantly decreased systemic vascular resistance. The incidence of PRS was greater in the AN group. Arterial hypotension during the neohepatic period was more frequent among patients with AN, more frequently requiring vasoconstrictor and inotropic therapy. CONCLUSIONS: AN is associated with hemodynamic impairment and with increased vasoactive drug requirements during liver transplantation, probably associated with impaired reflex vasoconstrictor responses to surgical manipulations and changes of blood volume. AN may be associated with a greater surgical risk during LT. Preoperative evaluation of AN may select a high-risk population of LT recipients.

Adult↗
PubMed2003

Liver transplantation for hilar cholangiocarcinoma: Spanish experience.

INTRODUCTION: Palliative treatment for nondisseminated irresectable hilar cholangiocarcinoma (HCC) carries a 0% 5-year survival rate. The role of orthotopic liver transplantation (OLT) in these patients is controversial because the survival rate is lower than that for other indications for transplantation and the lack of available donor organs. The aim of this paper was to review the Spanish experience in OLT for HCC and identify prognostic factors for survival. METHODS: We retrospectively reviewed 36 patients undergoing OLT for HCC over 13 years. RESULTS: The actuarial survival rate at 1, 3, and 5 years was 82%, 53%, and 30%, respectively. The main cause of death was tumor recurrence (53%). In the univariate analysis, the factors for a poor prognosis were vascular invasion (P<.001) namely 0% survival at 3 years when present versus 63% and 35% at 3 and 5 years, respectively, when it was not; and stages III to IVA (P<.05), namely 15% survival at 5 years versus 47% for stages I to II. Lymph node and perineural invasion also reduce survival. In the multivariate analysis, the factors for poor prognosis included vascular invasion (P<.01) and stages III to IVA (P<.01). CONCLUSION: OLT for nondisseminated irresectable HCC has higher survival rates at 3 and 5 years than palliative treatments, especially with initial stage tumors, which means that more information is needed to better select cholangiocarcinoma patients for transplantation.

Bile Duct Neoplasms↗
PubMed2003

Liver transplantation for peripheral cholangiocarcinoma: Spanish experience.

INTRODUCTION: Palliative treatment for nondisseminated unresectable peripheral cholangiocarcinoma (PCC) carries a 0% 5-year survival rate. The role of orthotopic liver transplantation (OLT) in these patients is controversial because the survival rate is lower than with other indications for transplantation and the lack of available donor organs. The aim of this paper was to review the Spanish experience in OLT for PCC to identify prognostic factors for survival. METHODS: We retrospectively reviewed 23 patients undergoing OLT in Spain for PCC over a period of 13 years. RESULTS: The actuarial survival rates were 77%, 65%, and 42% at 1, 3, and 5 years, respectively. The main cause of death was tumor recurrence (35%). Prognotic factors for an adverse outcome were pTNM classification (P<.05) in the univariate analysis and perineural invasion (P<.05) and stages III or IVA (P<.05) in the multivariate analysis. CONCLUSIONS: OLT for nondisseminated irresectable PCC displays higher survival rates at 3 and 5 years than palliative treatments, especially for tumors in the initial stages, which means that more information is needed to help better select PCC patients for transplantation.

Bile Duct Neoplasms↗
PubMed2000

Clinico-electrophysiological correlation of extensor digitorum brevis muscle atrophy in children with charcot-marie-tooth disease 1A duplication.

The purpose of the study is to describe the electrophysiologic abnormalities accounting for the appearance and progression of extensor digitorum brevis (EDB) muscle atrophy in Charcot-Marie-Tooth-disease type 1A (CMT-1A) children. Twelve children with CMT-1A duplication were serially evaluated. Initial ages of clinico-electrophysiological exams ranged from 1 month to 4 years (mean: 2 years) and final ages from 6 to 23 years (mean: 13). All subjects had two or more electrophysiological studies of the peroneal nerve. EDB atrophy was observed in two out of 12 (17%) patients by the age of 5, in eight out of ten (80%) examined between 5 and 9 years, and in all eight (100%) patients who had reached the second decade at the end. Nerve conduction maturation was systematically abnormal, but by age of 5 the mean values of nerve conduction parameters of peroneal nerve did not significantly differ from those in older patients. Compound muscle action potential (CMAP) amplitudes of EDB were reduced in 42% of cases initially and 100% upon last exam. Furthermore, a constant finding throughout the study was progressive attenuation of CMAPs, these becoming unobtainable in four cases. EDB muscle atrophy in CMT-1A children is an age-dependent sign which is accounted for by gradual reduction of the distal peroneal nerve CMAP amplitudes.

Action Potentials↗
PubMed2000

Peripheral motor and sensory nerve conduction studies in normal infants and children.

OBJECTIVE: There are few data on electrophysiological data of motor and sensory fibres during nerve maturation. The aim of this study is to investigate the evolution of nerve conduction in the upper and lower limbs during the first years of life. METHODS: The study comprised 92 normal infants and children aged from 1 week to 6 years. Using surface electrodes, the investigation included the following data: (1) motor conduction velocity (MCV), corrected distal motor latency (DML) to a standard distance, and F-waves of the median, ulnar, peroneal and tibial nerves; (2) sensory conduction velocity (SCV) of the median and tibial nerves; and (3) amplitude and morphology of the muscle and sensory action potentials. RESULTS: Maximal MCV and SCV in the neonatal period was about half of adults; there was a steep conduction increase during the first year of life, adult values being reached around age 4. In the neonatal period corrected DML was greater than in adults with a further decrease during the first year. F-wave latencies also decreased during the first year with increase at the end of the study. CONCLUSIONS: This study corroborates the fact that 'maturation' of MCV and SCV occurs during the first 5 years of life, especially in the former. Evolution of DML is accounted for using correction. F-wave latency changes are explained both by an increase in MCV, and extremity growth.

Action Potentials↗
PubMed2000

Continuous facial myokymia in multiple sclerosis: treatment with botulinum toxin.

Continuous facial myokymia (CFM) is an involuntary undulating, vermicular movement that spreads across facial muscles and is associated with a characteristic electromyographic pattern. It is an infrequent clinical sign that almost always occurs in intrinsic brainstem lesions, particularly in multiple sclerosis (MS). It is usually present for only a few weeks, but it may persist for long periods of time being very troublesome for patients. We report 2 cases with MS and continuous hemifacial myokymia persisting for up to 1 month which disappeared after injection of botulinum toxin. Botulinum toxin A (BTX-A) has been used successfully to treat a variety of focal dystonias and occasionally in orbicularis myokymia, but its use has not been reported in continuous hemifacial myokymia. BTX-A appears to be effective and safe for treating persistent facial myokymia in MS patients.

Adult↗
PubMed1999

Castleman's disease associated with chronic inflammatory demyelinating polyradiculoneuropathy: a clinical and electrophysiological follow-up study.

Castleman's disease (CD), or angiofollicular lymph node hyperplasia, is a rare lymphoproliferative disorder that can be associated with peripheral neuropathy. We report the long-term follow-up of a patient with a chronic inflammatory demyelinating polyradiculoneuropathy complicating the mediastinal form of classic CD who improved notably with immunosuppressive therapy. Our findings suggest that serial electrophysiological studies may be useful for monitoring treatment efficacy.

Aged↗
PubMed1999

Presence of a nitric oxide synthase inhibitor in the graft efflux during reperfusion in human liver transplantation.

Involvement of the nitric oxide (NO) system in complications following human orthotopic liver transplants (OLT) has been reported, but the contribution of the graft to the modulation of the NO system during reperfusion in normal OLT has not been characterized. We have studied the contribution of the graft efflux to the modulation of the NO system in 20 consecutive OLT. We evaluated its effects on isolated vascular reactivity of the rabbit and on rat cultured macrophages stimulated with lipopolysaccharide (LPS). In none of the donor liver biopsies was expression of inducible NO synthase (iNOS) activity by Northern or Western blot analysis found. Graft efflux after the onset of liver reperfusion, but not pre-transplant patient plasma, reversibly inhibited the acetylcholine-induced relaxation of norepinephrine-contracted rabbit aortic rings. Moreover, graft efflux reversibly inhibited NO production in rat macrophages treated with LPS, as evidenced by both a decrease in nitrite plus nitrate formation and a decrease in the production of [14C]citrulline from [14C]arginine. Addition of a 10% dilution of graft efflux to cultured rat macrophages incubated with LPS increased iNOS mRNA levels, suggesting direct inhibition of the enzyme but not of its expression. These results cannot be ascribed to the depletion of arginine the iNOS substrate since they can be reproduced even in the presence of an excess (10 mM) of exogenously added arginine. No correlation was found between the iNOS inhibitory activity in each sample and the corresponding clinical parameters related to either the graft function after the OLT or the existence of post-reperfusion syndrome. Our results indicate the existence of a soluble factor in the graft efflux from human OLT that reversibly and unspecifically inhibits NOS activity. Its involvement in the physiology and/or pathology of human liver diseases deserves further study.

Acetylcholine↗
PubMed1999

[Arterial complications after liver transplantation: early and late forms].

Arterial complications after liver transplantation (OLT) was related with high morbimortality rates, with variable consequences on patient and graft survival. 234 patients who underwent 270 liver transplantation between april of 1990 and december 1996 have been evaluated. Arterial complications were detected in 34 patients (12.5%), hepatic artery stenosis in 10 patients (29.4%), thrombosis in 24 (70.6%) and aneurysm in 2 (5.9%). It was considered early artery complications those diagnosed at the first month after OLT, and late complications the latest ones. Diagnosis was realized by Doppler sonography in all cases, and confirmed by celiac arteriography in those with Doppler abnormal findings. Clinical appearance of early artery complications were as acute liver failure (61.1%) or graft disfunction (38.8%). On the other hand, late complications were diagnosed incidentally in 46% of the patients due a programmed Doppler follow up. Actuarial survival at 24 months was higher in patients with late arterial complication (50 vs 30%; p < 0.01). Therapeutic measures applied were retransplantation, surgical artery reconstruction and angioplasty. Early complications required more frequently retransplantation or surgical reconstruction. Risk factors related with artery complications development were analyzed, but not significant differences between both types of arterial complications were found. It is concluded that programmed follow up by Doppler or arteriography is necessary to make an earlier diagnose and to detect silent arterial complications.

Adult↗
PubMed1998

Abdominal epilepsy in an adolescent with bilateral perisylvian polymicrogyria.

PURPOSE: We report a patient with recurrent episodes of severe periumbilical pain accompanied by headache, pallor, dizziness, and visual hallucinations who was subsequently diagnosed as having abdominal epilepsy and a bilateral sylvian cortical malformation. METHODS AND RESULTS: During an EEG examination, the patient had a simple partial seizure, manifested as intense abdominal pain. The ictal EEG showed a focal electrographic seizure arising from the left frontotemporal region. Computed tomography scan was normal; however, magnetic resonance imaging (MRI) revealed asymmetric lesions involving both sylvian fissures compatible with polymicrogyria. Onset of treatment with sodium valproate was followed by a significant reduction of seizures. CONCLUSIONS: Abdominal epilepsy should be suspected in adolescents or adults with paroxysmal episodes of abdominal pain associated with migraine-like symptoms. EEG and MRI investigations are indicated. A developmental brain disorder should be considered as a possible etiology.

Abdominal Pain↗
PubMed1998

Charcot-Marie-Tooth disease type 1A with 17p duplication in infancy and early childhood: a longitudinal clinical and electrophysiologic study.

OBJECTIVE: We describe longitudinal clinical and electrophysiologic evaluation of Charcot-Marie-Tooth disease type 1A (CMT-1A) in infancy and early childhood. BACKGROUND: The clinical picture and electrophysiologic evaluation of CMT-1A during the age of nerve conduction maturation have not been documented. DESIGN/METHODS: Twenty at-risk children from six unrelated CMT-1A families were examined in the first 5 years of life. Initial ages were 1 month to 4 years (mean, 1.5 years) and final ages 4 to 19 years (mean, 9 years). All subjects had two or more motor and sensory conduction velocities (MCV and SCV), corrected distal motor latencies (DML), and F-waves. RESULTS: Twelve children were affected. Initially, two of these (17%) had symptoms, whereas five (42%) were symptomatic at the end. Numbers of abnormal examinations at the beginning was six (50%) and at conclusion was 10 (83%). None of the patients were disabled. From 2 years of age, all affected children had abnormal MCV, SVC, F-waves, and DML. Prolonged DML was already present in the first months of life and preceded slowing of MCV in three cases. CONCLUSION: The electrophysiologic studies were concordant with the presence or absence of the CMT-1A DNA duplication. In most CMT-1A patients, symptoms appear in early childhood, although the florid clinical picture does not occur until the second decade of life. Serial electrophysiologic studies can detect the CMT-1A gene carrier in infancy.

Adolescent↗