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Clinical Spectrum (clinical + spectrum)

Distribution by Scientific Domains

Medical Sciences	91%
Life Sciences	8%

Distribution within Medical Sciences

Neurology	23%
Dermatology	16%
Oncology & Radiotherapy	4%
14 Other Subdomains	41%

Selected Abstracts

Telithromycin-associated hepatotoxicity: Clinical spectrum and causality assessment of 42 cases,

HEPATOLOGY, Issue 1 2009
Allen D. Brinker
Telithromycin is the first of a new class of ketolide antibiotics with increased activity against penicillin-resistant and erythromycin-resistant pneumococci. This agent received approval by the United States Food and Drug Administration (FDA) in 2004 for treatment of upper and lower respiratory infections. Following market introduction, spontaneous reports of telithromycin-associated hepatotoxicity, including frank liver failure, were received. To address these reports, an ad hoc group with expertise in spontaneous adverse events reporting and experience in evaluating drug-induced liver injury was formed, including members of the FDA, other federal agencies, and academia. The primary objective of this group was to adjudicate case reports of hepatic toxicity for causal attribution to telithromycin. After an initial screening of all cases of liver injury associated with telithromycin reported to FDA as of April 2006 by one of the authors, 42 cases were comprehensively reviewed and adjudicated. Five cases included a severe outcome of either death (n = 4) or liver transplantation (n = 1); more than half were considered highly likely or probable in their causal association with telithromycin. Typical clinical features were: short latency (median, 10 days) and abrupt onset of fever, abdominal pain, and jaundice, sometimes with the presence of ascites even in cases that resolved. Concurrence in assignment of causality increased after agreement on definitions of categories and interactive discussions. Conclusion: Telithromycin is a rare cause of drug-induced liver injury that may have a distinctive clinical signature and associated high mortality rate. Consensus for attribution of liver injury to a selected drug exposure by individual experts can be aided by careful definition of terminology and discussion. (HEPATOLOGY 2009;49:250-257.) [source]

Clinical spectrum and histological analysis of 32 cases of specific cutaneous sarcoidosis

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 12 2006
Cristina Mangas
Background:, Sarcoidosis is a multisystem granulomatous disorder of unknown aetiology in which skin involvement is frequent. Objective:, To review histological characteristics of biopsies of specific cutaneous lesions of sarcoidosis and their relationship with clinical course. Patients and methods:, Biopsies from 32 patients with specific cutaneous sarcoidosis were reviewed. Histological findings and clinical characteristics of these patients were analysed. Results:, The initial clinical lesions of the patients were ten infiltrated nodule-plaques, eight papules, four maculopapular eruptions, five scar sarcoidosis, four subcutaneous nodules and one lupus pernio. Sarcoidal granulomas were located at dermis in 31 cases (74%) and at subcutaneous fat in 12 (28%) but only four were subcutaneous exclusively. Perivascular or periannexial distribution of granulomas was observed in eight cases (19%) and they had coalescence in 29 samples. The presence of foreign material was demonstrated in 11 cases (26%). Conclusions:, Clinical spectrum of specific lesions of cutaneous sarcoidosis showed a good correlation with granulomas localization in the biopsies. However, traditional classification of specific cutaneous sarcoidosis is often overlapping. On the other hand, foreign bodies and other atypical histological findings were more common than initially expected. [source]

Electrophysiological Changes In Diabetic Neuropathy: From Subclinical Alterations To Disabling Abnormalities

JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 3 2000
M. Baba
Clinical spectrum of diabetic neuropathy is variable; it may be asymptomatic, but once established, it becomes irreversible and disabling. Some investigators suggested that earliest change in diabetic nerve function is alteration in axonal excitability due to alterations in ion conductance of axon membrane, although these functional changes of ion channels necessarily cause permanent damage or degeneration of nerve fibers. Among various parameter of nerve conduction study in diabetics, prolonged F-wave latency in the peroneal and tibial nerve seems the commonest abnormality in asymptomatic patients. Decrease in amplitude of compound sensory action potential of sural nerve is another earlier abnormality, which is, then, accompanied by a fall in motor amplitude of peroneal and tibial nerves in advanced patients. In disabled patients no motor response is often elicited in the legs. Previous electrophysiological studies could not make clear if central axons were involved or not in diabetic neuropathy. Recently, our group has demonstrated that somatosensory central conduction from the spinal cord to the sensory cortex is delayed in diabetics as well as in the peripheral conduction, which might be partly responsible for the irreversible clinical presentation of diabetic neuropathy. [source]

Clinical spectrum of tuberculous pleural effusion in children

PEDIATRICS INTERNATIONAL, Issue 3 2007
CHIH-YUNG CHIU
Abstract Background: The aim of this study was to describe the clinical characteristics and potentially diagnostic specimens of pediatric patients with tuberculous pleural effusion (TPE) to make a prompt diagnosis. Methods: Children who had TPE from September 1997 to December 2003 were retrospectively reviewed at a tertiary pediatric facility in northern Taiwan. Results: There were seven boys and six girls and their ages ranged from 10 to 17 years (average, 14.6 years). Tuberculosis contact history was identified in only six patients (46%). Fever (12/92%), cough (9/69%) and malaise (6/46%) were the most common symptoms. Normal leukocyte count was found in 12 patients (92%). Chest radiograph review showed unilateral pleural effusion in 12 patients (92%) but parenchymal involvement was found in nine patients (69%). Most of the pleural fluid analysis showed a lymphocytic exudative effusion (5/6). The acid-fast bacilli (AFB) stain of sputum, gastric washing, and pleural aspirate was positive in six of 11 (55%), two of seven (29%), and one of five (20%) patients, respectively. Culture of sputum, gastric washing, and pleural aspirate yielded Mycobacterium tuberculosis in four of 11 (36%), two of seven (29%), and two of five (40%) patients, respectively. A total of 6 to 9 months of multiple-drug therapy for tuberculosis was successful without sequale. Conclusions: Tuberculous pleural effusion usually presents as an acute illness and should always be considered in the differential diagnosis for older children and adolescents with pneumonia. A normal leukocyte count with a lymphocytic exudative effusion may provide a clue to the correct diagnosis of TPE. Diagnostic specimen of sputum seems more effective and sensitive in childhood TPE, especially those having pulmonary involvement. [source]

Clinical spectrum in hospitalized children with echovirus type 13 infection

PEDIATRICS INTERNATIONAL, Issue 2 2005
Ken-Ichiro Kobayashi
AbstractBackground:,The aim of this study was to investigate the clinical spectrum of echovirus type 13 (E13) infection in hospitalized children. Methods:,From April to August 2002, prospective viral surveillance was performed for hospitalized patients (aged 10 days to 14 years) irrespective of their presenting symptoms and severity. Medical records of laboratory-confirmed echovirus 13 infection were reviewed. Results:,Of the 41 patients analyzed, the median age was 3.4 years and 30% of them were less than 1 year of age. The male:female ratio was 1.6:1. The main clinical features were non-specific febrile illness (nine patients), gastroenteritis (seven), bronchitis (seven), aseptic meningitis (16) and idiopathic thrombocytopenic purpura (two). Each age group had their representative symptoms: less than 1 year of age, non-specific febrile illness; from 1 to 6 years of age, enterocolitis and bronchitis; more than 6 years of age, aseptic meningitis. Conclusion:,The representative symptoms of E13 infection in hospitalized patients were variable but strongly associated with age distribution. It was of interest to note that two patients developed diopathic thrombocytopenic purpura along with the infection. [source]

Clinical spectrum of acute abdominal pain in Turkish pediatric patients: A prospective study

PEDIATRICS INTERNATIONAL, Issue 3 2004
Tülay Erkan
AbstractBackground:,The aim of the present study was to determine the prevalence, associated symptoms, and clinical outcomes of children with acute abdominal pain who had been admitted to an emergency department. Methods:,Children aged between 2 and 16 years who presented to the emergency department of Cerrahpa,a Medical School, Istanbul University between July 2001 and August 2002 with acute abdominal pain were enrolled in this study. A questionnaire was completed each patient admitted to our pediatric emergency unit for acute abdominal pain. Data collected included presenting signs and symptoms, the hospital follow up for all children who returned within 10 days, test results, and telephone follow up. Results:,The number of children referred to the emergency department was 7442, with 399 (5.4%) of these having acute abdominal pain. The mean age of the study population was 6.9 ± 3.5 years, and 201 of the patients were male. The five most prevalent diagnoses were: (i) upper respiratory tract infection and/or complicated with otitis media or sinusitis (23.7%); (ii) abdominal pain with uncertain etiology (15.4%); (iii) gastroenteritis (15.4%); (iv) constipation (9.4%); and (v) urinary tract infection (8%). The most common associated symptoms were decreased appetite, fever and emesis. Because of follow-up deficiency the progress of 28 patients was not obtained. Eighty-two children were referred to the department of pediatric surgery, but only 17 of 82 (20.7%) required surgical intervention (15 of these 17 for appendicitis). Eleven patients returned within 10 days for re-evaluation, but the initial diagnosis was not changed. The complaints of 57 patients with uncertain etiology were resolved within 2 days. Conclusions:,An acute complaint of abdominal pain was usually attributed to a self-limited disease. However, the percentage of surgical etiology is not negligible. [source]

Adverse drug reactions in medical intensive care unit of a tertiary care hospital,

PHARMACOEPIDEMIOLOGY AND DRUG SAFETY, Issue 7 2009
Lisha Joshua MBBS
Abstract Purpose Patients in the intensive care unit (ICU) have multiorgan dysfunction as well as altered pharmacokinetic parameters. Hence they are susceptible to adverse drug reactions (ADRs). The objective of the study is to assess the characteristics of ADRs among inpatients in the medical ICU and to compare the same with patients who have not experienced ADRs. Methods Prospective, observational study for a period of 1 year in medical ICU of a tertiary care hospital. Relevant data of patients with ADRS were analysed. Characteristics of patients with and without ADRs were compared. Results Of 728 patients admitted in medical ICU, 222 (28.4%) had ADRs. Multiple ADRs (38.7%) implicated by the same drug and serious ADRs (37%) were noticed. Renal/electrolyte system (21%) was most commonly involved. Clinical spectrum included acute renal failure (ARF, 11.4%), hepatic injuries (5.4%), haematological dysfunction (4.2%), seizures (3.3%), upper gastrointestinal bleed (3.3%) and cutaneous ADRs (3.3%). Antimicrobials (27%) were the commonly implicated drug class. The most commonly implicated drug was furosemide (6.8%). Infrequently reported ADRs included azithromycin-induced erythema multiforme, leflunamide-induced erythema multiforme and vasculitis, ceftazidime-induced seizures and ceftriaxone-induced hepatitis. Co-morbidity, polypharmacy and duration of stay were significantly higher in patients with ADRs compared to those who have not experienced ADRs. Three patients died. Conclusion High incidence of serious and multiple ADRs noticed. A wide clinical spectrum of ADRs and infrequently reported ADRs to newer drugs were also observed. Copyright © 2009 John Wiley & Sons, Ltd. [source]

On phenomenology and classification of hoarding: a review

ACTA PSYCHIATRICA SCANDINAVICA, Issue 5 2004
T. Maier
Objective:, Hoarding is a behavioural abnormity characterized by the excessive collection of poorly useable objects. It is described mainly in association with obsessive,compulsive disorders (OCDs) and in geriatric populations. Yet the literature on the phenomenon is heterogeneous and the notion obviously lacks a consistent definition. This review attempts to describe the psychopathological and clinical spectrum of hoarding and may contribute to clarify its classification. Method:, Systematic review and discussion of the literature on hoarding. Results:, Hoarding is a complex behavioural phenomenon associated with different mental disorders. The psychopathological structure is variously composed of elements of OCDs, impulse-control disorders, and ritualistic behaviour. Severe self-neglect is a possible consequence of hoarding. Conclusion:, Without further specifications the term hoarding is of limited heuristic value and cannot guide therapeutic interventions satisfactorily. The condition needs to be evaluated carefully in every particular case in relation to the aforementioned psychopathological concepts. [source]

Drug Resistance in Epilepsy: Putative Neurobiologic and Clinical Mechanisms

EPILEPSIA, Issue 6 2005
Dieter Schmidt
Summary:, Drug-resistant epilepsy with uncontrolled severe seizures despite state-of-the-art medical treatment continues to be a major clinical problem for up to one in three patients with epilepsy. Although drug resistance may emerge or remit in the course of epilepsy or its treatment, in most patients, drug resistance seems to be continuous and to occur de novo. Unfortunately, current antiepileptic drugs (AEDs) do not seem to prevent or to reverse drug resistance in most patients, but add-on therapy with novel AEDs is able to exert a modest seizure reduction in as many as 50% of patients in short-term clinical trials, and a few become seizure free during the trial. It is not known why and how epilepsy becomes drug resistant, while other patients with seemingly identical seizure types can achieve seizure control with medication. Several putative mechanisms underlying drug resistance in epilepsy have been identified in recent years. Based on experimental and clinical studies, two major neurobiologic theories have been put forward: (a) removal of AEDs from the epileptogenic tissue through excessive expression of multidrug transporters, and (b) reduced drug-target sensitivity in epileptogenic brain tissue. On the clinical side, genetic and clinical features and structural brain lesions have been associated with drug resistance in epilepsy. In this article, we review the laboratory and clinical evidence to date supporting the drug-transport and the drug-target hypotheses and provide directions for future research, to define more clearly the role of these hypotheses in the clinical spectrum of drug-resistant epilepsy. [source]

A 9-year review of dystonia from a movement disorders clinic in Singapore

EUROPEAN JOURNAL OF NEUROLOGY, Issue 1 2006
R. D. G. Jamora
The clinical features of dystonia have not been evaluated in Southeast Asia. We therefore investigated the clinical spectrum and characteristics of dystonia in Singapore, a multi-ethnic Southeast Asian country comprising 77% Chinese, 14% Malays, and 8% Indians. We identified all dystonia patients from the Movement Disorders database and Botulinum Toxin clinic between 1995 and November 2004. Their medical records were reviewed to verify the diagnosis of dystonia and obtain demographic and clinical data using a standardized data collection form. A total of 119 (73%) patients had primary dystonia whilst 45 (27%) had secondary dystonia. There were 77% Chinese, 9% Malays, and 8% Indians. The most common focal dystonia were cervical dystonia (47%), writer's cramp (32%), and blepharospasm (11%). There was no significant difference in the distribution of dystonia between the different races. Males were noted to have earlier onset of dystonia overall. There was a significant male predominance in primary dystonia overall (M:F 1.6:1, P = 0.008) and in the subgroup of focal dystonia (M:F 1.6:1, P = 0.037). This contrasts with previous studies that found a female predominance. The role of genetic, hormonal, and environmental factors and their interactions need to be investigated to better understand the gender differences in the occurrence of dystonia. [source]

PAX5/IGH rearrangement is a recurrent finding in a subset of aggressive B-NHL with complex chromosomal rearrangements,

GENES, CHROMOSOMES AND CANCER, Issue 2 2005
Bruce Poppe
We present an extensive characterization of 10 B-cell lymphomas with a t(9;14)(p13;q32). The presence of the PAX5/IGH gene rearrangement was demonstrated by fluorescence in situ hybridization (FISH) using a validated probe set, whereas complex karyotypic changes were reassessed by multiplex-FISH (M-FISH). Pathologic and clinical review revealed the presence of this rearrangement in 4 histiocyte-rich, T-cell-rich B-cell lymphomas (HRTR-BCLs) and 2 posttransplantation diffuse large B-cell lymphomas (PTLD-DLBCLs). In contrast to initial observations describing this translocation in lymphoplasmacytic lymphoma (LPL) and LPL-derived large B-cell lymphoma, our data showed a wide morphologic and clinical spectrum associated with the PAX5/IGH rearrangement, pointing to an association between this aberration and a subset of de novo DLBCLs presenting with advanced disease and adverse prognosis. In addition, the recurrent incidence of this rearrangement in both HRTR-BCL (4 cases) and PTLD-DLBCL (2 cases) was previously unrecognized and is intriguing. © 2005 Wiley-Liss, Inc. [source]

Systemic Lupus Erythematosus Presenting as Subacute Delirium in an 82-Year-Old Woman

JOURNAL OF AMERICAN GERIATRICS SOCIETY, Issue 4 2001
George A. Heckman MD
OBJECTIVES: To describe an older patient with delirium attributed to systemic lupus erythematosus (SLE) and to review the literature on neuropsychiatric manifestations of SLE in older people. DESIGN: Case report and literature review. MEDLINE search using terms systemic lupus erythematosus, neurologic, psychiatric, neuropsychiatric, autoantibodies (antinuclear antibody (ANA), antiphospholipid, anticardiolipin, anti-double stranded deoxyribonucleic acid (anti-dsDNA), anti-Smith), and elderly. Additional articles obtained from hand-searched references and through experts. SETTING: Hospital (case report). PARTICIPANTS: Case report and literature cases. MEASUREMENTS: None. RESULTS: SLE is increasingly diagnosed in older adults. Onset is insidious and diagnosis is delayed because of a different clinical spectrum and immunological profile than in younger adults. Autoantibodies have an important role in the pathogenesis of neuropsychiatric manifestations, while vasculitis is less common. Aggressive immunosuppressive therapy is typically indicated, although recent case reports suggest that lower doses may suffice. The American College of Rheumatology 1982 revised criteria may be inadequate to diagnose neuropsychiatric lupus in older persons. CONCLUSION: Neuropsychiatric symptoms can be prominent in older people, presenting features of SLE. This case illustrates the lowest dose of corticosteroids shown to be effective in an older patient with delirium due to SLE. [source]

Radiation-induced brain disorders in patients with pituitary tumours

JOURNAL OF MEDICAL IMAGING AND RADIATION ONCOLOGY, Issue 3 2004
A Bhansali
Summary Radiation-induced brain disorders (RIBD) are uncommon and they are grave sequelae of conventional radiotherapy. In the present report, we describe the clinical spectrum of RIBD in 11 patients who received post-surgery conventional megavoltage irradiation for residual pituitary tumours. Of these 11 patients (nine men, two women), seven had been treated for non-functioning pituitary tumours and four for somatotropinomas. At the time of irradiation the age of these patients ranged from 30 to 59 years (mean, 39.4 ± 8.3; median, 36) with a follow-up period of 6,96 months (mean, 18.3 ± 26.4; median, 11). The dose of radiation ranged from 45 to 90 Gy (mean, 51.3 ± 13.4; median, 45), which was given in 15,30 fractions (mean, 18.6 ± 5.0; median, 15) with 2.8 ± 0.3 Gy (median, 3) per fraction. The biological effective dose calculated for late complications in these patients ranged from 78.7 to 180 Gy (mean, 99.1 ± 27.5; median, 90). The lag time between tumour irradiation and the onset of symptoms ranged from 6 to 168 months (mean, 46.3 ± 57.0; median, 57). The clinical spectrum of RIBD included new-onset visual abnormalities in five, cerebral radionecrosis in the form of altered sensorium in four, generalized seizures in four, cognitive dysfunction in five, dementia in three and motor deficits in two patients. Magnetic resonance imaging (MRI)/CT of the brain was suggestive of radionecrosis in eight, cerebral oedema in three, cerebral atrophy in two and second neoplasia in one patient. Associated hormone deficiencies at presentation were hypogonadism in eight, hypoadrenalism in six, hypothyroidism in four and diabetes insipidus in one patient. Autopsy in two patients showed primitive neuroectodermal tumour (PNET) and brainstem radionecrosis in one, and a cystic lesion in the left frontal lobe following radionecrosis in the other. We conclude that RIBD have distinctive but varying clinical and radiological presentations. Diabetes insipidus and PNET as a second neoplastic disorder in adults following pituitary irradiation have not been reported previously. [source]

Risk factors and ultrasonographic profile of posterior fossa haemorrhages in preterm infants

JOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 4 2009
Arvind Sehgal
Aims: While preterm infants are known to be at risk of intracranial haemorrhages, advances in ultrasound imaging of preterm babies have facilitated recognition of presence of haemorrhages in the posterior fossa, which include cerebellar and Cisterna Magna haemorrhages. There are limited data on the profile and predisposing risk factors. The objective was to identify antenatal, intrapartum and post-natal risk factors for and to define the clinical spectrum. The study was designed as a retrospective case-control study in the setting of a tertiary level neonatal intensive care unit. Preterm babies ,30 weeks gestation age admitted between January 2005 and December 2006, with an ultrasound diagnosis of posterior fossa haemorrhage and an equal number of controls matched for gestation age, gender and month of birth with normal cranial scans were selected. Systematic chart and radiographic review was done. All cranial ultrasounds in both groups were reviewed. Results: Eighteen babies had documented posterior fossa haemorrhage (13 cerebellar, 5 isolated Cisterna Magna, 10 both), the median time of detection being 2.5 days. Eleven babies had either no or grade I/II supratentorial bleeds, while half of all cerebellar bleeds were bilateral. All haemorrhages were visualised from mastoid view and none from anterior fontanel. On univariate analysis, multiple gestations, lack of antenatal steroids, foetal heart rate abnormalities, need for volume expanders and cardiotrophins and sepsis were associated with a higher risk for having posterior fossa bleeds. Conclusions: Posterior fossa haemorrhages in preterm babies are being increasingly recognised. Antenatal, intrapartum and post-natal factors may predispose towards haemorrhages in the cerebellum or Cisterna Magna. [source]

Hepatitis C infection in children: A Melbourne perspective

JOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 4 2000
B Karim
Objective: To examine the clinical spectrum of hepatitis C virus (HCV) infected children in our care by determining presentation, mode of acquisition, degree of co-infection, biochemical evidence of persisting hepatitis and treatment outcome. Methodology: A retrospective review of the medical records of all children attending the Royal Children's Hospital, Melbourne, between 1990 and 1998, who had antibodies to HCV infection detected. Detailed clinical information, investigations and the results of treatment were extracted from the clinical notes. Results: A total of 94 children (age range 2 weeks to 19.7 years) were identified, of whom nine had passive transfer of maternal antibodies from HCV-positive mothers and were excluded from analysis. Sixty-seven children (79%) were infected by transfusion of blood or blood products. Perinatal transmission occurred in 11 children (13%), and six children (7%) had a history of i.v. drug abuse. The majority of children were asymptomatic at presentation. Of the 65 patients tested for HCV-ribonucleic acid, 43 (66%) were positive. Fifty-seven cases had serial alanine aminotransaminase (ALT) measurements over a mean of 28 months. Of these, 38 (67%) had an abnormal ALT. Ten cases (12%) were co-infected with hepatitis B virus, HIV or both. Of 12 patients treated with interferon, four responded with normalisation of ALT from 3 to 12 months post-commencement of therapy. Conclusions: Although HCV was largely an asymptomatic condition in our clinic population, more than half the patients had biochemical evidence of ongoing liver damage. Given the chronicity of this infection in the majority of patients and the long-term risks of cirrhosis and hepatocellular carcinoma, children with HCV infection represent a high-risk group worthy of regular follow up. [source]

Neurotoxicity of immunosuppressive drugs

LIVER TRANSPLANTATION, Issue 11 2001
Eelco F.M. Wijdicks MD
The clinical profile of neurotoxicity caused by immunosuppression has changed. When toxic levels are reached, both cyclosporine and tacrolimus may produce a clinical spectrum that varies from tremor and acute confusional state to status epilepticus and major speech or language abnormalities. Coma has become an unusual manifestation. Magnetic resonance imaging has been better defined, and abnormalities may be more widespread than those in the posterior lobes. These white matter lesions are caused by vasogenic edema, but may lead to apoptosis and cytotoxic edema if exposure is prolonged. Recent evidence suggests inhibition of a drug-efflux pump and dysfunction of the blood-brain barrier by enhanced nitric oxide production. [source]

Motor laterality asymmetry and nonmotor symptoms in Parkinson's disease,

MOVEMENT DISORDERS, Issue 1 2010
Esther Cubo
Abstract Background: In patients with Parkinson's disease (PD), asymmetric motor signs provide an interesting model to evaluate whether asymmetric nigrostriatal degeneration can affect neuropsychological function and other nonmotor symptoms (NMS). This study was designed to evaluate the predominant laterality of motor symptoms and its relationship with cognition and other NMS in idiopathic PD. Methods: Nationwide, longitudinal, and multicenter study (ELEP Registry) using outpatients with PD. Left PD (LPD) and right PD (RPD) was defined based on the motor signs on the SCOPA-motor scale. To include the clinical spectrum of asymmetric PD patients, we considered two groups of patients with mild-moderate and extreme asymmetry. Predominant LPD or RPD with mild-moderate versus extreme asymmetry were compared using the following scales: cognition, psychosis (Parkinson Psychosis Rating Scale), anxiety/depression, sleep (and autonomic dysfunction at baseline and 1 year later. Nonparametric tests were used for comparison. Results: One hundred forty-nine PD patients (74 RPD and 75 LPD) with mild-moderate asymmetry and 90 (47 RPD and 43 LPD) with extreme asymmetry and a mean age of 64.5 (10.4) years were included. Extreme RPD had higher Parkinson Psychosis Rating Scale scores over time (P = 0.005) compared with LPD, but no significant differences were observed between LPD and RPD in terms of other NMS. Conclusions: These findings suggest that damage to left-hemisphere plays a disproportionately greater role in PD-related psychosis over time. In contrast, motor laterality does not consistently affect other NMS, suggesting that NMS are related to a more widespread brain disorder. © 2009 Movement Disorder Society [source]

Benign hereditary chorea revisited: A journey to understanding

MOVEMENT DISORDERS, Issue 16 2007
Galit Kleiner-Fisman MD
Abstract Benign hereditary chorea (BHC) has been characterized as an autosomal dominant disorder manifesting nonprogressive chorea without dementia. However, there has been controversy regarding its existence. Diagnosis has been based solely on clinical criteria with many patients and families demonstrating "atypical" features and until recently, no diagnostic test was available for confirmation. Since 2002, mutations in the thyroid transcription factor (TITF-1) gene have been identified as resulting in some cases of BHC. Additionally, the clinical spectrum has expanded to include abnormalities in thyroid and lung with the putative mechanism of disease resulting from gene haploinsufficiency and reduced protein product. This review summarizes both a historical perspective and our current understanding of BHC. © 2007 Movement Disorder Society [source]

Sleep-related stridor due to dystonic vocal cord motion and neurogenic tachypnea/tachycardia in multiple system atrophy

MOVEMENT DISORDERS, Issue 5 2007
Roberto Vetrugno MD
Abstract Sleep-disordered breathing and sleep-related motor phenomena are part of the clinical spectrum of multiple system atrophy (MSA). Stridor has been attributed to denervation of laryngeal muscles or instead to dystonic vocal cord motion. We studied 3 patients with nocturnal stridor in the setting of MSA. All patients underwent nocturnal videopolysomnography (VPSG) with breathing and heart rate, O2 saturation and intra-esophageal pressure recordings, and simultaneous EMG recordings of the posterior cricoarytenoid, cricothyroid, and thyroarytenoid muscles and continuous vocal cord motion evaluation by means of fiberoptic laryngoscopy. VPSG/EMG and fiberoptic laryngoscopy documented normal vocal cord motion without denervation during wake and stridor only during sleep when hyperactivation of vocal cords adductors appeared in the absence of significant O2 desaturation. All patients had tachycardia and tachypnea and paradoxical breathing during sleep, erratic intercostalis and diaphragmatic EMG activity and Rem sleep behavior disorder. One of the patients had restless legs syndrome with periodic limb movement during sleep and excessive fragmentary hypnic myoclonus. In conclusion, our patients with MSA had nocturnal stridor due to sleep-related laryngeal dystonia. Stridor was associated with other abnormal sleep-related respiratory and motor disorders, suggesting an impairment of homeostatic brainstem integration in MSA. © 2007 Movement Disorder Society [source]

The Unified Parkinson's Disease Rating Scale (UPDRS): Status and recommendations

MOVEMENT DISORDERS, Issue 7 2003
Article first published online: 18 MAR 200
Abstract The Movement Disorder Society Task Force for Rating Scales for Parkinson's Disease prepared a critique of the Unified Parkinson's Disease Rating Scale (UPDRS). Strengths of the UPDRS include its wide utilization, its application across the clinical spectrum of PD, its nearly comprehensive coverage of motor symptoms, and its clinimetric properties, including reliability and validity. Weaknesses include several ambiguities in the written text, inadequate instructions for raters, some metric flaws, and the absence of screening questions on several important non-motor aspects of PD. The Task Force recommends that the MDS sponsor the development of a new version of the UPDRS and encourage efforts to establish its clinimetric properties, especially addressing the need to define a Minimal Clinically Relevant Difference and a Minimal Clinically Relevant Incremental Difference, as well as testing its correlation with the current UPDRS. If developed, the new scale should be culturally unbiased and be tested in different racial, gender, and age-groups. Future goals should include the definition of UPDRS scores with confidence intervals that correlate with clinically pertinent designations, "minimal," "mild," "moderate," and "severe" PD. Whereas the presence of non-motor components of PD can be identified with screening questions, a new version of the UPDRS should include an official appendix that includes other, more detailed, and optionally used scales to determine severity of these impairments. © 2003 Movement Disorder Society [source]

Early detection of acute kidney injury: Emerging new biomarkers (Review Article)

NEPHROLOGY, Issue 2 2008
ZOLTAN H ENDRE
SUMMARY: Acute kidney injury (AKI) has recently become the preferred term to describe the syndrome of acute renal failure (ARF) with ,failure' or ,ARF' restricted to patients who have AKI and need renal replacement therapy.1 This allows capture of the broader clinical spectrum of modest reductions in creatinine, which are themselves known to be associated with major increases in both short- and long-term mortality risk.2,5 It is hoped that this change in nomenclature will facilitate an expansion of our understanding of the underlying pathophysiology and also facilitate definitions of AKI, which allow comparisons among clinical trials of patients with similar duration and severity of illness. This review will cover the need for early detection of AKI and the role of urinary and plasma biomarkers, including enzymuria. The primary message is that use of existing criteria to diagnose AKI, namely elevation of the serum creatinine with or without oliguria, results in identification that is too late to allow successful intervention. New biomarkers are essential to change the dire prognosis of this common condition. [source]

Fanconi's syndrome and subsequent progressive renal failure caused by a Chinese herb containing aristolochic acid

NEPHROLOGY, Issue 3 2004
SANGHO LEE
SUMMARY: Chinese herb nephropathy contains a variety of clinical features of progressive renal failure (indicated by studies conducted in Belgium) to the variant type of Fanconi's syndrome. Fanconi's syndrome has mostly been reported in Asian countries, and is characterized by proximal tubular dysfunction and slower progression to end-stage renal disease (ESRD); it also often revealed a reversible clinical course. We describe a 43-year-old woman who presented with polyuria and polydipsia caused by Fanconi's syndrome. The cause of Fanconi's syndrome was not identified because the patient denied the intake of the Chinese herbal mixture at first. Fanconi's syndrome seemed to be reversible in its early stage, but it rapidly progressed to renal failure after 3 months, despite the interruption of Chinese mixture use. A renal biopsy revealed typical findings of aristolochic acid-induced nephropathy. Aristolochic acids were also detected in the Chinese herbs that were consumed. This case highlights the variety of the clinical spectrum of aristolochic acid induced nephropathy (AAN). We emphasize that AAN should be suspected in all patients with Fanconi's syndrome, even if patients deny the intake of any Chinese herbal preparation. [source]

PL1 Subepithelial bullous diseases , topic overview

ORAL DISEASES, Issue 2006
M Mravak-Stipeti
Subepithelial bullous diseases comprise the group of mucocutaneous autoimmune blistering diseases characterized by subepithelial separation and the deposition of immunoglobulin and complement against several antigens along the basement membrane zone (BMZ). This result in spectrum of diseases that affect skin, oral mucosa, and other mucosal membranes and include bullous pemphigoid (BP), mucous membrane (cicatricial) pemphigoid (MMP), linear IgA disease (LAD), and chronic bullous dermatosis of childhood (CBDC). The most common clinical features are oral erosions, desquamative gingivitis and conjunctival fibrosis, as well as skin lesions, predominantly in older female population. The heterogeneity of clinical presentation and diversity of target autoantigens have contributed to difficulties in characterizing this condition immunologically. In addition to the clinical presentation and a subepithelial vesicle or bullae on routine histologic analysis, the diagnosis is based on direct and indirect immunofluorescence studies. The nature of the disease is determined by the target antigens in the epithelium and BMZ such as antigen 180 (BP180), antigen 230 (BP230), laminin 5, and beta 4 integrin. Circulating IgG and IgA antibodies bind to different epitopes of BP180. The use of salt-split skin substrate enables differentiation between epidermal and dermal 'binders'. Since the antigen and the antibody titer appear to have direct relationships with the disease severity, and a combination of clinical finding and antibody titer provides valuable prognostic data, these investigations should be carried out routinely. Clinicians should recognize clinical spectrum of SBD, the histopathologic and immunopathologic characteristics, the differential diagnosis, the treatment, and the natural history of the disease. Involvement of oral medicine specialists, dermatologists, ophthalmologists, otolaryngologists and gastroenterologists contribute to early diagnosis and will aid in providing SBD patients with the highest quality of care. [source]

Hyperferritinemia and iron overload in type 1 Gaucher disease,

AMERICAN JOURNAL OF HEMATOLOGY, Issue 7 2010
Philip Stein
Hyperferritinemia occurs in Gaucher disease but its clinical spectrum or its association with systemic iron overload and HFE mutations are not known. In 114 patients with Type 1 Gaucher disease, we determined serum ferritin, transferrin saturation and HFE genotype. The results were correlated with the extent of hepatosplenomegaly, overall Gaucher disease severity score index, and response to enzyme replacement therapy. In a subset of patients with radiological and/or laboratory evidence of systemic iron overload, liver biopsy was performed. There was a mean 3.7-fold elevation of serum ferritin over the upper limit of normal (ULN). Prior splenectomy was associated with most severe hyperferritinemia compared to patients with intact spleen (6.53 × ULN vs. 2.69 × ULN, P = 0.003). HFE genotyping revealed two patients homozygous for H63D mutation and 30% of patients heterozygote carriers of H63D mutation; no patients harbored C282Y mutation; there was no correlation of ferritin with HFE genotype. Ferritin level correlated with liver volume (Pearson correlation coefficient = 0.254, P = 0.035) and it was negatively correlated with hemoglobin (r = ,0.315, P = 0.004); there was no relationship with other indicators of Gaucher disease activity. Enzyme replacement therapy (ERT) resulted in amelioration of hyperferritinemia: 707 ± 898 ng/ml vs. 301 ± 310 ng/ml (P = 0.001), transferrin saturation remained normal. Three patients were suspected of clinical iron overload, confirmed on liver biopsy. Iron accumulation was variably noted in hepatocytes and Kupffer cells. There is a high prevalence of hyperferritinemia in Type 1 Gaucher disease that is associated with indicators of disease severity, reversed by ERT and is not related to HFE mutations. Am. J. Hematol. 2010. © 2010 Wiley-Liss, Inc. [source]

Chronic Recurrent Multifocal Osteomyelitis (CRMO) and Synovitis, Acne, Pustulosis, Hyperostosis, and Osteitis (SAPHO) Syndrome with Associated Neutrophilic Dermatoses: A Report of Seven Cases and Review of the Literature

PEDIATRIC DERMATOLOGY, Issue 5 2009
Brook E. Tlougan M.D.
Classically, patients present with swelling, pain, and impaired mobility of the affected area, with skin lesions developing concurrently or in the future. Bone biopsy reveals inflammatory changes consistent with infectious osteomyelitis, but cultures and histologic staining invariably fail to identify an infectious source. Patients are refractory to antibiotic therapy, but dramatically respond to systemic steroids and may need to be maintained on low-dose steroids to prevent relapse. Numerous authors have suggested that CRMO and synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome lie along the same clinical spectrum. In fact some believe that CRMO is the pediatric presentation of SAPHO. The two syndromes share numerous characteristics, including osteitis, a unifocal or multifocal presentation, hyperostosis, and pustulosis, which all occur in a generally healthy individual. Our seven patients, five of whom were diagnosed with CRMO, and two of whom were diagnosed with SAPHO syndrome further strengthen the idea that CRMO and SAPHO syndrome do indeed lie along the same clinical spectrum. In addition, we include two rare cases of pediatric Sweet's syndrome with evidence of pathergy. [source]

Adams,Oliver Syndrome: A Sporadic Occurrence With Minimal Disease Expression

PEDIATRIC DERMATOLOGY, Issue 1 2008
TARUN NARANG M.D.
We report a child with the sporadic form of the disease who had minimal disease expression, illustrating the wide clinical spectrum of the syndrome. [source]

The Challenge of asthma in adolescence

PEDIATRIC PULMONOLOGY, Issue 8 2007
Diletta de Benedictis MD
Abstract The adolescents with asthma are a distinct group of patients with different problems and needs compared to children and adults. Specific issues of asthma in adolescence are the variability of the clinical spectrum, the presence of particular risk factors for the persistence of symptoms, underdiagnosis and undertreatment of the disease. Refusal of the sick role, denial of symptoms, carelessness about dangerous inhalation exposure, erratic self-medication, overexertion without taking precautions against exercise-induced asthma, and a poor relationship between patients, their families, and often doctors are the main obstacles to successful management of asthma in this critical age. There are also major problems of compliance for these patients. The goal of optimal quality of life will be achieved only if the physician thoroughly understands the adolescent's needs and provides optimal care. Pediatr Pulmonol. 2007, 42:683,692. © 2007 Wiley-Liss, Inc. [source]

Complications of varicella in healthy children in Izmir, Turkey

PEDIATRICS INTERNATIONAL, Issue 3 2005
Güldane Koturoglu
AbstractBackground:,The purpose of the paper was to evaluate the indications of hospital admissions and complications of varicella infection in immunologically healthy children. Methods:,Between 1997 and 2001, patient records of children hospitalized due to varicella infection were reviewed. Incidence and clinical spectrum of complications and their distribution related to age and seasonal variations were analyzed. Results:,A total of 178 immunocompetent children were hospitalized for varicella complications during the study period. This resulted in a crude incidence of 6.3/100 000 population at risk. All hospital admissions were due to accompanying complications. The majority of complications occurred in preschool-age children with a median age of 3 years. No gender predominance was found. The most frequent complications were infectious complications, which were observed in 79 children (44%). Superinfections of the skin were present in 24 patients. Pneumonia was observed in 59 children: 49 had bacterial, 10 had viral pneumonia. Pyogenic arthritis was seen in two children and one had concomitant osteomyelitis. Group A ,-hemolytic streptococci were recovered from two patients with invasive bacterial infections. A total of 68 (38%) neurologic complications were observed. Cerebellar ataxia was present in 24, encephalitis was present in 17. Infectious complications occurred more frequently in younger children (median age: 2 years), whereas neurologic complications occurred at an older age (median age: 6 years). Hematologic complications were seen in nine children. There was a seasonal distribution of complications with a peak in January. Conclusion:,Complications of varicella requiring hospitalization in immunocompetent children are more frequent than previously thought. [source]