First Presentation (first + presentation)

Distribution by Scientific Domains
Distribution within Medical Sciences


Selected Abstracts


Some unusual type 2 reactions in leprosy

INTERNATIONAL JOURNAL OF DERMATOLOGY, Issue 2 2010
V. Ramesh MD
Background, Type 2 reactions with lepromatous leprosy (LL) not occurring during multi-drug therapy (MDT) have been reported. Methods, Three patients have been described, each representing a prototype, the first presenting as bullous erythema nodosum leprosum (ENL), second with ENL erupting after treatment for co-existing pulmonary tuberculosis and resembling immune reconstitution inflammatory syndrome, and a third patient with recurrent Sweets-syndrome like presentation who had taken incomplete MDT in the past for leprosy. In all, the diagnosis was established by demonstration of acid-fast bacilli (AFB) on slit-skin smears (SSS) and histopathology. Results & Conclusion, The fact that reactions can occur in patients with clinically inapparent LL, who are more likely to present in general hospitals, has been reemphasized to enhance awareness among physicians. First presentation of leprosy as ENL is probably precipitated by common antibiotics taken for other illnesses. Since reactional episodes can occur before, during and after MDT for leprosy and the clinical picture is not specific to any of them, it is important to ascertain the status of anti-leprosy therapy during these episodes and treat them accordingly. [source]


First presentation of myasthenia gravis

ANAESTHESIA, Issue 1 2006
M. Cheesman
No abstract is available for this article. [source]


Ethnic differences in the timely diagnosis of children with Type 1 diabetes mellitus in the Netherlands: clinical presentation at onset

DIABETIC MEDICINE, Issue 3 2007
J. J. N. Van Laar
Abstract Aims Little is known about ethnic differences in the timely diagnosis of Type 1 diabetes mellitus (Type 1 DM). This study aimed to assess ethnic inequalities in the timely diagnosis of Type 1 DM, as indicated by a more adverse clinical condition at onset. In addition, we assessed whether these differences could be explained by differences in socio-economic status. Methods From a national register, we selected 3128 children aged < 15 years with newly diagnosed Type 1 DM. Ethnic differences in serum glucose, blood pH, bicarbonate, presence of ketonuria, level of consciousness, hydration status, and diabetic ketoacidosis were assessed by logistic regression. A measure of socio-economic status based on postal codes was used as an explanatory variable. Results The risk of adverse clinical presentation was 1.5,2 times higher in non-Western immigrants than Dutch children, while Western immigrant children did not differ from Dutch children. Blood pH, bicarbonate level, and level of consciousness were lower in Turkish and Antillean children in particular. The adverse socio-economic position of immigrant children contributed very little to these differences in clinical presentation. Conclusions Non-Western children were likely to be sicker at first presentation of Type 1 DM, and thus diagnosis may have been delayed. These disparities were not accounted for by differences in socio-economic status. Possible explanations may be difficulties in recognition of symptoms, failure of GPs to take symptom reporting seriously and lack of awareness of the fact that Type 1 DM occurs more often in certain ethnic groups. [source]


Cytologic feature by squash preparation of pineal parenchyma tumor of intermediate differentiation

DIAGNOSTIC CYTOPATHOLOGY, Issue 10 2008
Keiji Shimada M.D., Ph.D.
Abstract Pineal parenchyma tumor of intermediate differentiation (PPTID) is a very rare intracranial tumor, and pathological investigation limited to immunohistological and ultrastructural analyses have been published to date. Although intraoperative cytology is one of the important approaches for initial diagnosis in brain tumors, no or little studies on cellular morphology of PPTID have been demonstrated due to its rarity. We report here cytological features of PPTID obtained from stereotactic surgical specimens in a case of 27-year-old female manifested by dizziness and diplopia. Brain MRI revealed an unhomogeneously enhanced, large-sized tumor (56 × 52 × 60 mm) mainly located in the pineal region expanding from the midbrain to superior portion of the cerebellum and the fourth ventricle. Squash cytology showed increased nucleocytoplasmic ratio, hyperchromatic nuclei, and small rosette-like cell cluster but cellular pleomorphism was mild to moderate and necrotic background was not observed. Histology showed high cellularity, moderate nuclear atypia, and small rosette formation but neither bizarre tumor cells nor necrosis was present. Mitotic counts were very low (less than 1 per 10 high-power fields) and the MIB-1 labeling index was relatively high (10.1%). Tumor cells were immunohistochemically positive for neural markers such as synaptophysin, neurospecific enolase but not for glial fibrillary acidic protein or S-100. In some parts, cells were strongly reactive for neurofilament protein. Taken together, we made a final diagnosis of PPTID. This is the first presentation of cytological analysis by squash preparation that gives an important clue to accurate diagnosis of pineal parenchymal tumor and to understand its malignant potential. Diagn. Cytopathol. 2008;36:749,753. © 2008 Wiley-Liss, Inc. [source]


Treatment retention in adolescent patients treated with methadone or buprenorphine for opioid dependence: a file review

DRUG AND ALCOHOL REVIEW, Issue 2 2006
JAMES BELL
Abstract The aim of this study was to compare retention and re-entry to treatment between adolescent subjects treated with methadone, those treated with buprenorphine, and those treated with symptomatic (non-opioid) medication only. We used a retrospective file review of all patients aged less than 18 at first presentation for treatment for opioid dependence. The study was conducted at the Langton Centre, Sydney, Australia, an agency specialising in the treatment of alcohol and other drug dependency. Sixty-one adolescents (age range 14,17 years at the time of commencing treatment); mean reported age of initiation of heroin use was 14 ± 1.3 years (range 11,16). Sixty-one per cent were female. The first episode of treatment was methadone maintenance in 20 subjects, buprenorphine in 25, symptomatic medication in 15; one patient underwent assessment only. These 61 subjects had a total of 112 episodes of treatment. Subjects treated with methadone had significantly longer retention in first treatment episode than subjects treated with buprenorphine (mean days 354 vs. 58, p<0.01 by Cox regression) and missed fewer days in the first month (mean 3 vs. 8 days, p<0.05 by t-test). Subsequent re-entry for further treatment occurred in 25% of subjects treated with methadone, 60% buprenorphine and 60% symptomatic medications. Time to reentry after first episode of buprenorphine treatment was significantly shorter than after methadone treatment (p < 0.05 by Kaplan-Meier test). Methadone maintenance appears to have been more effective than buprenorphine at preventing premature drop-out from treatment of adolescent heroin users. [source]


The development of the Recovery and Prevention of Psychosis Service in Melbourne, Australia

EARLY INTERVENTION IN PSYCHIATRY, Issue 2 2009
Brendan P. Murphy
Abstract Aim: To describe the establishment of a multicomponent, phase-specific, early intervention service for young people experiencing psychosis. Methods: The Recovery and Prevention of Psychosis Service commenced streamed clinical service delivery in November 2004, providing comprehensive case management for up to 3 years within Victoria's largest metropolitan health service. It delivers phase-oriented treatment focusing on early detection, recovery and relapse prevention, and minimizing disability and secondary comorbidity. The combined programme covers training and professional development, data collection and evaluation, specialist intervention services, group programme work and community development. Results: Of the first 151 clients, 70.2% were male, the average age at first presentation was 20.9 years, 15% were under 18 at first contact and 67% required inpatient admission at least once. Mean age at first contact was 20.84 years for those requiring inpatient services and 70% admitted were male. The average length of stay was 25.69 days and 23% were secluded, with an average of 2.1 seclusions. A large percentage of Recovery and Prevention of Psychosis Service clients (81%) required involuntary treatment, a significantly greater proportion of admitted patients were on Community Treatment Orders compared to those never admitted (22.5% cf. 4.1%; P = 0.04) and 92% of those admitted subsequently relapsed compared to 8% of those not admitted (P = 0.02). Conclusions: Recovery and Prevention of Psychosis Service is successfully developing a fully integrated first episode service. Recent developments include expanding the period of care up to 5 years for selected patients, the recruitment of a health promotions officer and planning for the development of a youth inpatient unit. [source]


Investigating moderate to severe paediatric trauma in the Auckland region

EMERGENCY MEDICINE AUSTRALASIA, Issue 2 2010
Louise Couch
Abstract Objective: To investigate differences between paediatric patients with moderate to severe trauma admitted from two paediatric ED, with respect to: demographics, patterns of presentation, mechanism of injury, injury severity scores (ISS), interventions and outcome. Method: Retrospective cohort study. Moderate to severe trauma was defined as ISS >9. Paediatric patients admitted to hospital via Starship Children's Emergency or KidzFirst ED, with trauma from 1 May 2003 to 30 April 2004, with ISS >9 were identified using multiple databases. The charts were reviewed and data collected included: demographics, hospital of first presentation, diagnoses, ISS, Paediatric trauma score (PTS), Glasgow coma score (GCS), ventilator hours, length of admission, survival and discharge destination. Descriptive statistics with 95% confidence intervals, Mann,Whitney U -test, ,2 -test and Fisher's exact test were used as appropriate. Results: A total of 393 children with moderate to severe trauma were identified using initial search strategies. Of these, 82 children met the inclusion and exclusion criteria for the study; 42 children were admitted via KidzFirst ED and 40 via Starship Children's ED. There was no statistically significant difference in ISS (P= 0.86), PTS (P= 0.11), GCS (P= 0.62), hours on a ventilator (P= 0.28) and length of stay (P= 0.87) between children admitted from Starship or KidzFirst ED. Conclusion: This study suggests that there are no differences in the numbers or severity of paediatric trauma patients admitted from the Starship and KidzFirst ED. This indicates triage is to the closest ED despite having a tertiary referral centre for paediatric trauma available in Auckland City. [source]


Early Recognition of Benign Partial Epilepsy in Infancy

EPILEPSIA, Issue 6 2000
Akihisa Okumura
Summary: Purpose: The aim of this study is to determine how precisely we can recognize the outcome in infants with epilepsy beginning in the first year of life. Methods: We performed a prospective 5-year follow-up study on 63 patients who developed epilepsy in the first year of life. We first judged that patients met the criteria of "possible benign partial epilepsy in infancy (BPEI)" on enrollment in this study. At 2 years of age, we reevaluated the seizure and developmental outcome in the patients who were diagnosed as having "possible BPEI." We finally judged that patients met the criteria of "definite BPEI" at age 5 years. "Possible BPEI" was defined as epilepsy meeting all the following conditions: (a) complex partial seizures and/or secondarily generalized seizures; (b) normal psychomotor development and neurologic findings before onset; (c) normal interictal electroencephalograms; (d) normal cranial computed tomography (CT) and magnetic resonance imaging (MRI) findings; and (e) no seizures during the first 4 weeks of life. "Definite BPEI" was defined as epilepsy meeting all the following criteria in addition to those of "possible BPEI": (a) normal psychomotor development beyond age 5 years, and (b) no seizures beyond age 2 years. Results: Thirty-two of the 63 patients met the inclusion criteria completely and were included in the "possible BPEI" group. Twenty-five of the 32 patients completed the 5-year follow-up. At age 2 years, four patients were excluded from the "possible BPEI" group because of seizure recurrence and/or delayed development. By age 5 years, one had a recurrence of seizures, and another exhibited mildly delayed psychomotor development. We finally diagnosed 19 patients as having "definite BPEI.""Definite BPEI" accounted for 76% of the patients diagnosed as having "possible BPEI" at the first presentation and 90% of those who met the conditions on reevaluation at age 2 years. Conclusions: Recognition of BPEI is possible, to some extent, at the first presentation, and reevaluation at age 2 years is useful for a more precise diagnosis. [source]


Gastric adenocarcinoma metastatic to the skin: a report

EUROPEAN JOURNAL OF CANCER CARE, Issue 2 2002
C. CHARALAMBOUS MBCHB, FRCS SURGICAL SENIOR HOUSE OFFICER
Metastases to the skin from internal tumours are uncommon, yet they may be the first presentation of such malignancies. They usually arise from the breast, lung and large bowel. Skin metastases from gastric adenocarcinoma are extremely rare. We report a case of a patient with gastric adenocarcinoma who at presentation had multiple, clinically benign-looking skin nodules. Biopsy of these revealed metastatic deposits. [source]


Evaluation of the genetic basis of phenotypic heterogeneity in north Indian patients with Thalassemia major

EUROPEAN JOURNAL OF HAEMATOLOGY, Issue 6 2010
Nidhi Sharma
Abstract Objectives: To assess the molecular basis of phenotypic heterogeneity in north Indian patients with thalassemia major (TM). Methods: To determine the clinical severity, 130 patients of TM were studied for the age of first presentation and frequency of blood transfusion. The type of beta mutations, Xmn,1G, polymorphism and G6PD Mediterranean mutation was characterized. Analysis of the phenotypic presentation and the genotype was performed. Results: Majority (83.8%) presented before 1 year of age (mean 8.8 months). The caste distribution showed 41.6% were Aroras and 32.3% were migrants from Pakistan. IVS1-5(G,C) was commonest (32.7%) and the common five Indian mutations comprised of 88.4% of alleles. The mean age of presentation with IVS1-5(G,C), Fr 8/9, (+G) 619-bp del and IVS1-1(G,T) homozygosity was 4.3, 6, 3.4 and 9.1 months respectively. Xmn,1G, status showed ,/, in 66.9%, +/, in 26.1% and +/+ in 6.9% patients. Xmn,1G,,/, presented before 1 year of age. The mean age of presentation with +/+ was 18.3 months. Six hemizygous boys and one heterozygous girl with G6PD Mediterranean were found (prevalence 5.3%). Eight patients could be reclassified as thalassemia intermedia on follow up. Conclusions: This study showed that majority of TM in north India present before 1 year of age and homozygous 619-bp deletion presents the earliest. The presence of Xmn-1G, polymorphism delays the presentation, is associated with the IVS 1-1 (G,T) and shows variable improvement with hydroxyurea therapy. Based on the results of genotyping, reevaluation of patients can improve the outcome in a few patients. [source]


Distinct kinds of novelty processing differentially increase extracellular dopamine in different brain regions

EUROPEAN JOURNAL OF NEUROSCIENCE, Issue 5 2006
Elvira De Leonibus
Abstract Behaviourally relevant novel stimuli are known to activate the mesocorticolimbic dopaminergic (DAergic) system. In this study we tested the reactivity of this system in response to distinct kinds of novelty processing. Using the in vivo microdialysis technique, we measured extracellular amounts of dopamine (DA) in different DAergic terminal regions during a social learning task in rats. In the first session (40 min) rats were exposed to two never previously encountered juveniles (i.e. unconditional novelty). Afterwards, the animals were divided into three groups: Control group was not exposed to any other stimulus; Discrimination group was exposed to one familiar and one new juvenile (i.e. novel stimulus discrimination); and Recognition group was re-exposed to the two familiar juveniles (i.e. familiarity recognition). In both the medial prefrontal cortex and the nucleus accumbens shell DA increased in response to the first presentation of the juveniles, showing that both structures are involved in processing unconditional social novelty. During the novel stimulus discrimination, we found no change in the prefrontal cortex, although DA increased in the accumbal shell in comparison with the group exposed to two familiar juveniles, showing that the shell is also involved in processing novel social stimulus discrimination. None of the stimuli presented affected DA in the accumbal core. This study provided the original evidence that DA in the various terminal regions is differentially coupled to distinct aspects of novelty processing. [source]


Merkel cell carcinoma with squamous and sarcomatous differentiation

JOURNAL OF CUTANEOUS PATHOLOGY, Issue 10 2008
June H. K. Hwang
Merkel cell carcinoma is an aggressive neuroendocrine tumor historically thought to arise from neural crest-derived cutaneous neuroendocrine cells. Recent evidence supports an epidermal origin. We present a case of Merkel cell carcinoma arising on the upper arm of a 94-year-old woman that had multiple morphologic patterns: small cells typical of Merkel cell carcinoma, malignant cells with squamous differentiation and malignant poorly differentiated spindle cells. Subsequent metastatic disease in regional lymph nodes showed only the small cells and the malignant spindle cells. To our knowledge, this is the first case of Merkel cell carcinoma showing these three patterns of differentiation at first presentation. This morphology raises the possibility that Merkel cell carcinomas may arise from epidermal stem cells that can differentiate along different lines. [source]


Peritoneal mesothelioma presenting as an acute surgical abdomen due to jejunal perforation

JOURNAL OF DIGESTIVE DISEASES, Issue 4 2007
Nikolaos S SALEMIS
BACKGROUND: Peritoneal mesothelioma is a rare disease associated with poor prognosis. Acute abdomen as the first presentation is an extremely rare occurrence. We report an exceptional case of a patient who was found to have a jejunal perforation due to infiltration of peritoneal mesothelioma. METHODS: A 62-year-old man was admitted with clinical signs of peritonitis. Computerized tomographic scans showed a mass distal to the ligament of Treitz, thickening of the mesentery and a small amount of ascites. RESULTS: Emergency laparotomy revealed a perforated tumor 15 cm distal to the ligament of Treitz and diffuse peritoneal disease. Segmental small bowel resection and suboptimal cytoreduction were performed. Histopathology and immunohistochemistry showed infiltration of malignant mesothelioma. During the postoperative period pleural mesothelioma was also diagnosed. Despite adjuvant chemotherapy, the patient died of disseminated progressive disease 7 months after surgery. CONCLUSIONS: Peritoneal mesothelioma is a rare malignancy with grim prognosis. Small bowel involvement is a poor prognostic indicator. Our case of a small bowel perforation due to direct infiltration by peritoneal mesothelioma appears to be the first reported in the English literature. [source]


Acute Myocardial Infarction Complicated by Early Onset of Heart Failure:

JOURNAL OF INTERVENTIONAL CARDIOLOGY, Issue 3 2003
Feasibility of Interhospital Transfer for Coronary Angioplasty., Safety
Objective: The objective of this study is to assess the feasibility and safety of interhospital transfer (within up to 60 minutes) for primary/rescue coronary angioplasty of patients with myocardial infarction (AMI) complicated by an early onset of acute heart failure (AHF) admitted to a community hospital without PCI facilities. Design and patients: From the multicenter randomized PRAGUE-1 study, a subgroup of 66 patients with AMI complicated by AHF on the first presentation to the community hospital were retrospectively analyzed. Group A patients(n = 21)were treated on site in community hospitals using thrombolysis (streptokinase), group B patients(n = 20)were transported with thrombolytic infusion to a PCI center for coronary angioplasty, and group C patients(n = 25)were immediately transported to a PCI center for primary angioplasty without thrombolysis. Results: No patient died during transportation. One group B patient developed ventricular fibrillation during transfer. The time delay from the onset of chest pain to reperfusion was >142 minutes, and 253 and 251 minutes in groups A, B, and C, respectively. Hospital stay (16 vs 11 vs 10 days,P = NS) was shorter in the angioplasty groups. Transported patients (groups B, C) displayed a significant decrease in heart failure progression within the first 24 hours after treatment (48% vs 15% vs 8%,P < 0.05). The combined end point, i.e., mortality + nonfatal reinfarction (43% vs 25% vs 8%,P < 0.05), was significantly less frequent in the coronary angioplasty group. Conclusions: Interhospital transfer for coronary angioplasty of patients with AMI complicated by an early onset of AHF is feasible and safe. Transport for angioplasty may even reduce the risk of heart failure progression and improve clinical outcome compared to immediate thrombolysis in the nearest community hospital. (J Interven Cardiol 2003;16:201,208) [source]


Systematic review: portal vein thrombosis in cirrhosis

ALIMENTARY PHARMACOLOGY & THERAPEUTICS, Issue 3 2010
E. A. TSOCHATZIS
Aliment Pharmacol Ther 31, 366-374 Summary Background, As current imaging techniques in cirrhosis allow detection of asymptomatic portal vein thrombosis during routine ultrasonography, more patients with cirrhosis are diagnosed with portal vein thrombosis. Although a consensus on noncirrhotic extra-hepatic portal vein thrombosis has been published, no such consensus exists for portal vein thrombosis with cirrhosis. Aim, To perform a systematic review of nonmalignant portal vein thrombosis in cirrhosis in terms of prevalence, pathogenesis, diagnosis, clinical course and management. Methods, Studies were identified by a search strategy using MEDLINE and EMBASE. Results, Portal vein thrombosis is encountered in 10,25% of cirrhotics. In terms of pathophysiology, cirrhosis is no longer considered a hypocoagulable state; rather than a bleeding risk in cirrhosis, various clinical studies support a thrombotic potential. Clinical findings of portal vein thrombosis in cirrhosis vary from asymptomatic disease to a life-threatening condition at first presentation. Optimal management of portal vein thrombosis in cirrhosis is currently not addressed in any consensus publication. Treatment strategies most often include the use of anticoagulation, while thrombectomy and transjugular intrahepatic portosystemic shunts are considered second-line options. Conclusions, Portal vein thrombosis in cirrhosis has many unresolved issues, which are often the critical problems clinicians encounter in their everyday practice. We propose a possible research agenda to address these unresolved issues. [source]


From the bench to the ,crib'-side: implications of scientific advances to paediatric neurogastroenterology and motility

NEUROGASTROENTEROLOGY & MOTILITY, Issue 4 2006
D. K. Chitkara
Abstract, Paediatric gastrointestinal motility disorders may present in the neonatal period as the result of a congenital insult that occurred during embryonic development or as a manifestation of an abnormal genetic background. Functional gastrointestinal and motility disorders may also be acquired and present during childhood as the first presentation of a condition that can persist or re-occur throughout adolescence and adulthood. These disorders can have a significant psychological and financial impact on the lives of the affected children and their families.1,2 Recently, enteric neuroscience research has advanced the understanding of the pathogenesis and treatment of uncommon congenital or developmental gastrointestinal motility disorders such as Hirschsprung disease and chronic intestinal pseudo-obstruction. In addition, research has contributed to improvements in the understanding of more prevalent functional gastrointestinal disorders in children, such as chronic constipation and functional abdominal pain syndromes. The purpose of this review is to highlight these advances with particular regard to the clinical impact they have in the understanding and management of disorders in the field of paediatric neurogastroenterology and motility. [source]


Bronchiolitis obliterans organizing pneumonia as an initial manifestation in systemic lupus erythematosus

PEDIATRIC PULMONOLOGY, Issue 3 2005
Hidetoshi Takada MD
Abstract Bronchiolitis obliterans organizing pneumonia (BOOP) is a rare complication of adult systemic lupus erythematosus (SLE). This is the first report of a pediatric patient with BOOP as an initial presentation of SLE. She had dyspnea, cough, arthralgia, and erythema on her face. Laboratory examinations revealed pancytopenia, low serum levels of complements, and positivity for anti-nuclear antibody, anti-double stranded DNA antibody, and anti-SM antibody. Her respiratory symptoms, pulmonary function tests, and radiologic findings showed significant improvement after treatment with oral prednisolone. Although it is a rare complication among the pleuro-pulmonary manifestations in SLE, BOOP can be the first presentation, even in pediatric patients. © 2005 Wiley-Liss, Inc. [source]


Fulminant hepatic failure: Wilson's disease or autoimmune hepatitis?

PEDIATRIC TRANSPLANTATION, Issue 1 2005
Implications for transplantation
Abstract:, Fulminant hepatic failure (FHF) accounts for 10,15% of pediatric liver transplants in the USA annually. Because the onset of FHF may be the first presentation of Wilson's disease (WD) and autoimmune hepatitis (AIH) in previously asymptomatic adolescents, determination of the etiology of FHF is critical as treatment and prognosis differ between these two entities. Patients with AIH may be salvaged by medical treatment. On the contrary, liver transplantation is currently the only life saving therapeutic option available for patients with WD who present with fulminant liver failure. To establish the diagnosis of WD and AIH in the setting of FHF remains challenging for diagnosticians and decisions regarding liver transplantation may be necessary before a diagnosis is firmly established. We report a previously asymptomatic patient who presented with FHF and clinical and laboratory features suggestive of both WD and AIH and who underwent successful therapeutic liver transplantation before the diagnosis of WD could be confirmed. [source]


Gray matter atrophy is related to long-term disability in multiple sclerosis

ANNALS OF NEUROLOGY, Issue 3 2008
Leonora K. Fisniku MRCP
Objective To determine the relation of gray matter (GM) and white matter (WM) brain volumes, and WM lesion load, with clinical outcomes 20 years after first presentation with clinically isolated syndrome suggestive of multiple sclerosis (MS). Methods Seventy-three patients were studied a mean of 20 years from first presentation with a clinically isolated syndrome (33 of whom developed relapsing-remitting MS and 11 secondary-progressive MS, with the rest experiencing no further definite neurological events), together with 25 healthy control subjects. GM and WM volumetric measures were obtained from three-dimensional T1-weighted brain magnetic resonance images using Statistical Parametric Mapping 2. Results Significant GM (p < 0.001) and WM atrophy (p = 0.001) was seen in MS patients compared with control subjects. There was significantly more GM, but not WM atrophy, in secondary-progressive MS versus relapsing-remitting MS (p = 0.003), and relapsing-remitting MS versus clinically isolated syndrome (p < 0.001). GM, but not WM, fraction correlated with expanded disability status scale (rs = ,0.48; p < 0.001) and MS Functional Composite scores (rs = 0.59; p < 0.001). WM lesion load correlated with GM (rs = ,0.63; p < 0.001), but not with WM fraction. Regression modeling indicated that the GM fraction explained more of the variability in clinical measures than did WM lesion load. Interpretation In MS patients with a relatively long and homogeneous disease duration, GM atrophy is more marked than WM atrophy, and reflects disease subtype and disability to a greater extent than WM atrophy or lesions. Ann Neurol 2008 [source]


Bone edema scored on magnetic resonance imaging scans of the dominant carpus at presentation predicts radiographic joint damage of the hands and feet six years later in patients with rheumatoid arthritis

ARTHRITIS & RHEUMATISM, Issue 7 2003
Fiona M. McQueen
Objective Magnetic resonance imaging (MRI) is capable of revealing synovitis and tendinitis in early rheumatoid arthritis (RA), as well as bone edema and erosion. These features are visible before radiographic joint damage occurs. We sought to examine whether MRI of one body region (the wrist) can be used to predict whole-body radiography scores reflecting joint damage at 6 years. Methods We conducted a 6-year prospective study of a cohort of patients who fulfilled the criteria for RA at presentation, using clinical parameters, radiographs, and MRI scans of the dominant wrist. Of the 42 patients enrolled at baseline, full MRI, radiographic, and clinical data were available for 31 at 6-year followup. MRI scans were scored by 2 radiologists, using a validated scoring system. Radiographs of the hands and feet were graded using the modified Sharp scoring method. MRI and radiography scores obtained at baseline and 6 years were compared, and baseline MRI scores were examined for their ability to predict radiographic outcome at 6 years. Results At 6 years, the total Sharp score correlated significantly with the total MRI score and the MRI erosion score (r = 0.81, P < 0.0001 and r = 0.79, P < 0.0001, respectively). The 6-year Sharp score also correlated with the baseline total MRI and MRI erosion scores (r = 0.56, P < 0.0001 and r = 0.33, P = 0.03, respectively). MRI synovitis and bone edema scores remained constant for the group as a whole over 6 years, but bone erosion scores progressed (P = 0.0001), consistent with radiographic deterioration. Erosions on 6-year MRI scans were frequently preceded by MRI bone edema at baseline (odds ratio 6.5, 95% confidence interval 2.78,18.1). Regression models indicated that the baseline MRI bone edema score was predictive of the 6-year total Sharp score (P = 0.01), as was the C-reactive protein (CRP) level (P = 0.0002). Neither shared epitope status nor swollen or tender joint counts predicted radiographic outcome in this cohort. A model incorporating baseline MRI scores for erosion, bone edema, synovitis, and tendinitis plus the CRP level and the erythrocyte sedimentation rate explained 59% of the variance in the 6-year total Sharp score (R2 = 0.59, adjusted R2 = 0.44). Conclusion MRI scans performed at the first presentation of RA can be used to help predict future radiographic damage, allowing disease-modifying therapy to be targeted to patients with aggressive disease. [source]


Long-term management of vulval lichen sclerosus in adult women

AUSTRALIAN AND NEW ZEALAND JOURNAL OF OBSTETRICS AND GYNAECOLOGY, Issue 2 2010
J. BRADFORD
Background:, Adult vulval lichen sclerosus (VLS) is usually a lifelong disease with an estimated remission rate after treatment of only 16% [Arch Dermatol 2004; 140 (6): 709]. Although superpotent topical corticosteroid (TCS) is the validated gold standard treatment to induce remission, little data are available on how remission should be maintained. Aims:, We present a retrospective chart review of 129 adult patients with VLS who have been under surveillance by the authors for a minimum duration of three years. Methods:, Remission was maintained in most patients with low-to-moderate potency TCS. All subjects' symptoms, signs, treatment regimes and response to treatment including compliance, symptom remission, disease progression with scarring, squamous cell carcinoma and side effects were recorded. Data were compared for the compliant and non-compliant groups. Fischer's exact test was used to identify significant differences. Results:, The mean age at presentation was 53.6 years and mean duration of follow-up was 6.2 years. Compliance was excellent: 84 (65%) of patients' self-reporting as being fully compliant. Symptom remission was achieved in 98% of compliant and 75% of non-compliant patients (P = 0.001) Progression of disease with scarring was not encountered in any of the compliant patients, but was seen in 35% of non-compliant patients (P = 0.0001). One patient had squamous cell carcinoma on first presentation. Carcinoma subsequently occurred in none of the compliant patients, and in five partly compliant patients (P = 0.004). Mild, reversible corticosteroid side effects were encountered in 7% of patients. Conclusions:, Long-term treatment of adult VLS with individualised regimes using moderate potency TCS is safe and effective. Patients require long-term follow-up. [source]


Metastatic pathways and time courses in the orderly progression of cutaneous melanoma

BRITISH JOURNAL OF DERMATOLOGY, Issue 1 2002
F. Meier
SummaryBackground,,It is known that two-thirds of patients who develop clinical metastases following treatment of a primary cutaneous melanoma initially present with locoregional metastases and one-third initially present with distant metastases. However, few reports in the literature give detailed figures on different metastatic pathways in cutaneous melanoma. Objectives,,The aim of the present study was to perform a detailed analysis of the different metastatic pathways, the time course of the development of metastases and the factors influencing them. Methods,,In a series of 3001 patients with primary cutaneous melanoma at first presentation, 466 subsequently developed metastasis and were followed-up over the long term at the University of Tuebingen, Germany between 1976 and 1996. Different pathways of metastatic spread were traced. Associated risk factors for the different pathways were assessed. Differences in survival probabilities were calculated by the Kaplan,Meier method and evaluated by the log-rank test. Results,,In 50·2% of the patients the first metastasis after treatment of the primary tumour developed in the regional lymph nodes. In the remaining half of the patient sample the first metastasis developed in the lymphatic drainage area in front of the regional lymph nodes, as satellite or in-transit metastases (21·7%) or as direct distant metastases (28·1%). Anatomical location, sex and tumour thickness were significant risk factors for the development of metastasis by different pathways. The most important risk factor appeared to be the location of the primary tumour. The median intervals elapsing before the first metastasis differed significantly between the different metastatic pathways. The direct distant metastases became manifest after a median period of 25 months, thus later than the direct regional lymph node metastases (median latency period, 16 months) and the direct satellite and in-transit metastases (median latency period, 17 months). In patients who developed distant metastases the period of development was independent of the metastatic route. The time at which the distant metastases developed was roughly the same (between 24 and 30 months after the detection of the primary tumour), irrespective of whether satellite or in-transit metastases, lymph node metastases or distant metastases were the first to occur. Conclusions,,The time course of the development of distant metastasis was more or less the same irrespective of the metastatic pathway; this suggests that in patients with in-transit or satellite metastasis or regional lymph node metastasis, haematogenic metastatic spread had already taken place. Thus, the diagnostic value of sentinel lymph node biopsy and the therapeutic benefit of elective lymph node dissection may be limited, as satellite and in-transit metastases or direct distant metastases will not be detected and haematogenous spread may already have taken place when the intervention is performed. [source]


The value of magnetic resonance cholangiopancreatography in predicting common bile duct stones in patients with gallstone disease,

BRITISH JOURNAL OF SURGERY (NOW INCLUDES EUROPEAN JOURNAL OF SURGERY), Issue 1 2003
B. Topal
Background: The application of available predictive scoring systems for the detection of common bile duct (CBD) stones has not reduced the number of patients who undergo unnecessary endoscopic retrograde cholangiopancreatography. The aim of this study was to create a predictive model for CBD stones and to assess the value of magnetic resonance cholangiopancreatography (MRCP) in prediction. Methods: In 1998, 366 patients with gallstone disease (118 males, 248 females; mean age 57 (range 8,84) years) underwent cholecystectomy. Statistical analysis was performed on patient data obtained at the time of first presentation. Results: CBD stones were demonstrated in 43 (12 per cent) of 366 patients. The predictive model for common duct stones included ultrasonography showing CBD stones or bile duct dilatation, age greater than 60 years, fever, serum alkaline phosphatase level above 670 units/l and serum amylase level above 95 units/l. In patients with a predicted probability greater than 5 per cent, CBD stones were present in 11 per cent, compared with 1 per cent in patients with a probability of 5 per cent or less. MRCP had an observed sensitivity of 95 per cent, specificity of 100 per cent, positive predictive value of 100 per cent and negative predictive value of 98 per cent. Conclusion: In patients with a predicted probability for CBD stones of more than 5 per cent, MRCP is recommended in order to confirm the presence or absence of stones and as guidance in further management. Copyright © 2003 British Journal of Surgery Society Ltd. Published by John Wiley & Sons Ltd [source]


Parathyroid hormone-related protein measured at the time of first visit is an indicator of bone metastases and survival in lung carcinoma patients with hypercalcemia

CANCER, Issue 8 2002
Akio Hiraki M.D., Ph.D.
Abstract BACKGROUND Parathyroid hormone-related protein (PTH-rP) is a major cause of tumor-induced hypercalcemia (TIH) and frequently is found to be elevated in serum of patients with TIH. In the current study, the authors examined the usefulness of PTH-rP measurement at the time of first presentation in the follow-up of lung carcinoma patients with TIH. METHODS The authors retrospectively studied 23 of 1149 lung carcinoma patients who were found to have TIH at the time of first presentation for the correlation between serum PTH-rP and the development of bone metastases and survival compared with lung carcinoma patients without TIH who were matched by gender, age, Eastern Cooperative Oncology Group performance status, histological type of tumor, and stage of the disease. RESULTS Twenty-three lung carcinoma patients with TIH demonstrated significantly increased serum levels of PTH-rP (mean ± standard error [SE], 84.1 ± 16.5 pmol/L) compared with control patients without TIH (mean ± SE, 36.2 ± 2.0 pmol/L) at the time of first presentation, (P < 0.001). In these hypercalcemic patients, patients whose serum PTH-rP was > 150 pmol/L (n = 16) were found to have a significantly increased rate of bone metastases (71.4% vs. 12.5%; P = 0.01) and decreased survival (median survival of 1.4 months vs. 5.4 months; P < 0.015) compared with patients whose serum PTH-rP was < 150 pmol/L (n = 7). CONCULUSIONS The data from the current study suggest that serum PTH-rP as determined at the time of first presentation is a useful indicator of not only hypercalcemia but also bone metastasis and eventual survival in patients with lung carcinoma. Cancer 2002;95:1706,13. © 2002 American Cancer Society. DOI 10.1002/cncr.10828 [source]


Comparison of Nebulized Epinephrine to Albuterol in Bronchiolitis

ACADEMIC EMERGENCY MEDICINE, Issue 4 2008
Paul Walsh MB
Abstract Objectives:, To compare the effect of nebulized racemic epinephrine to nebulized racemic albuterol on successful discharge from the emergency department (ED). Methods:, Children up to their 18th month of life presenting to two teaching hospital EDs with a clinical diagnosis of bronchiolitis who were ill enough to warrant treatment but did not need immediate intubation were eligible for this double-blind randomized controlled trial (RCT). Patients received either three doses of racemic albuterol or one dose of racemic epinephrine plus two saline nebulizers. Disposition was decided 2 hours after the first nebulizer. Successful discharge was defined as not requiring additional bronchodilators in the ED after study drug administration and not subsequently admitted within 72 hours. Adjusted relative risks (aRR) were estimated using the modified Poisson regression with successful discharge as the dependent variable and study drug and severity of illness as exposures. Secondary analysis was performed for patients aged less than 12 months and first presentation. Results:, The authors analyzed 703 patients; 352 patients were given albuterol and 351 epinephrine. A total of 173 in the albuterol group and 160 in the epinephrine group were successfully discharged (crude RR = 1.08, 95% confidence interval [CI] = 0.92 to 1.26). When adjusted for severity of illness, patients who received albuterol were significantly more likely than patients receiving epinephrine to be successfully discharged (aRR = 1.18, 95% CI = 1.02 to 1.36). This was also true among those with first presentation and in those less than 12 months of age. Conclusions:, In children up to the 18th month of life, ED treatment of bronchiolitis with nebulized racemic albuterol led to more successful discharges than nebulized epinephrine. [source]


Pulley anatomy for the radial side of the wrist

CLINICAL ANATOMY, Issue 4 2001
R. Simovitch
Abstract This is the first presentation in the literature of a radial tendon flexor pulley in the distal forearm adjacent to the flexor carpi radialis. The clinical significance is that in performing wrist tendon arthroplasty, this structure and, in close proximity, the sensory branch of the median nerve may be encountered. Whether cutting the pulley of the flexor carpi radialis is clinically significant in changing wrist biomechanics is unknown. Clin. Anat. 14:246,247, 2001. © 2001 Wiley-Liss, Inc. [source]


Maternal hypothyroidism in early and late gestation: effects on neonatal and obstetric outcome

CLINICAL ENDOCRINOLOGY, Issue 5 2005
Iskandar Idris
Summary Background, Maternal hypothyroidism may be associated with a variety of adverse neonatal and obstetric outcomes. Whether these outcomes are affected by maternal thyroid status at initial presentation or in late gestation specifically within a dedicated antenatal endocrine clinic remains unclear. The effects of thyroxine dose requirement during pregnancy and serum concentrations of TSH within such clinic settings are still not known. Objectives, We investigated these outcomes in patients with hypothyroidism during early and late gestation. TSH levels and thyroxine dose requirement during early and late gestation were also evaluated. Methods, We performed a retrospective study of data from 167 pregnancies managed in the antenatal endocrine clinic. Analysis of outcomes was linked to TSH at first presentation and in the third trimester. Outcome variables included: rate of caesarean section, pre-eclampsia, neonatal unit admission, neonatal weight and gestational age. Controlled TSH was defined as mothers with TSH between 0·1 and 2 with normal free thyroid hormone levels. Results, The caesarean section (CS) rates were higher in the study cohort (H) compared with the local (C) rate (H = 28·7%, C = 18%). The higher rate in our patient cohort was not due to a higher rate of emergency section nor to a lower threshold for performing elective caesarean section. The infant birthweight (IBW) from mothers with TSH > 5·5 (H1) and mothers with TSH between 0·1 and 5·5 at presentation (H2) was [median (range)] 3·38 (1·73,4·70) vs. 3·45 (1·36,4·76); P = ns. The prevalence of low-birthweight (LBW) infants (< 2·5 g) in groups H1 and H2 was 15% and 4·8%, respectively [odds ratio (OR) = 3·55, 95% confidence interval (95% CI) = 0·96,10·31]. IBW from mothers with TSH > 2 (H3) and mothers with controlled TSH in the third trimester (H4) were similar [3·38 (1·78,4·4) vs. 3·46 (1·36,4·76); P = ns]. The prevalence of LBW in groups H3 and H4 was 9% and 4·9%, respectively (OR = 1·95, 95% CI = 0·52,7·26). The median thyroxine dose (µg) increased significantly during pregnancy (first trimester: 100; second trimester: 125, P < 0·001; and third trimester: 150, P < 0·001) associated with appropriate suppression of TSH levels in the second and third trimesters. Rates of pre-eclampsia or admissions to neonatal units were negligible. Conclusion, Thyroxine dose requirement increases during pregnancy and thus close monitoring of thyroid function with appropriate adjustment of thyroxine dose to maintain a normal serum TSH level is necessary throughout gestation. Within a joint endocrine,obstetric clinic, maternal hypothyroidism at presentation and in the third trimester may increase the risk of low birthweight and the likelihood for caesarean section. The latter observation was not due to a higher rate of emergency caesarean section nor to a lower threshold for performing elective caesarean section. A larger study with adjustments made for the various confounders is required to confirm this observation. [source]


Characteristics of respiratory syncytial virus-related apnoea in three infants

ACTA PAEDIATRICA, Issue 6 2004
M Rayyan
Apnoea is a common sign in respiratory syncytial virus (RSV) infections in young infants and can be the first presentation of an acquired RSV infection. We describe polysomnographic recordings of three infants revealing prolonged RSV-related apnoea before RSV infection was diagnosed. The apnoeas were of central origin. The caregivers had not noted any apparent life-threatening events (ALTE) prior to the polysomnography. Cardiorespiratory monitoring after the acute infection did not reveal any further apnoeas. Conclusion: Central, prolonged apnoea can be the first sign of an acquired RSV infection in young infants in the absence of other respiratory symptoms and without any previous observation of apnoea by the caregivers. [source]


,When will I see you again?' Using local recurrence data to develop a regimen for routine surveillance in post-treatment head and neck cancer patients

CLINICAL OTOLARYNGOLOGY, Issue 6 2009
S.E. Lester
Objective:, To develop an evidence-based regimen for routine surveillance of post-treatment head and neck cancer patients. Design:, Review of 10 years of prospectively collected patient data. Main outcome measures:, Time of first presentation of ,new cancer event' (either first recurrence or second primary tumour). We did not evaluate whether or not the detected new cancer events were curable. Results:, Data from patients with primary squamous cell carcinoma of the larynx, oropharynx and hypopharynx were analysed. A total of 676 previously undiagnosed squamous cell carcinomas were recorded in these regions. In these patients there were 105 recurrences and 20 second primary cancers were recorded; 95th percentile of "time to a new cancer event" was calculated in years. These were for larynx 4.7 years, oropharynx 2.7 years, hypopharynx 2.3 years. The time to new cancer event was similar for early and late laryngeal cancers. Only 36 (47%) of the hypopharyngeal cancers were treated with curative intent and of these 36% had a previously undiagnosed cancer event. Conclusion:, Local data and published evidence support a follow-up duration of 7 years for laryngeal primaries and 3 years for both oropharyngeal and hypopharyngeal primaries. Late stage oropharyngeal cancers may require longer follow up than early cancers. Patients who continue to smoke may need longer follow up. A change in local follow-up protocol to this regimen would save 10 patient slots every week with no detriment to patient care. Clin. Otolaryngol. 2009, 34, 546,551. [source]


Conservative versus primary surgical treatment of acoustic neuromas: a comparison of rates of facial nerve and hearing preservation

CLINICAL OTOLARYNGOLOGY, Issue 3 2008
T.P.C. Martin
Objectives:, To determine whether patients with small to medium sized acoustic neuromas managed conservatively suffer reduced rates of facial nerve and hearing preservation when compared with patients undergoing primary surgery. Design:, An intention-to-treat comparison between patients managed conservatively at first presentation and those managed with primary surgery. Setting:, Tertiary referral neurotological centre in Birmingham, UK. Participants:, Data were drawn from our database of 487 sporadic acoustic neuromas managed from 1997 to present day. Main outcome measures:, Facial nerve status (assessed using the House-Brackmann system) was collated for all conservatively managed patients (n = 167) and compared with that expected when calculated from primarily surgically managed tumours of equivalent size (n = 121). A chi-square test was employed to test the statistical significance of any difference. Hearing preservation (maintenance of AAO-HNS Class) in patients presenting with Class A or B hearing was compared between conservatively managed patients and those with primary surgical management. Results:, Observed facial nerve preservation in conservatively managed patients was significantly better (P < 0.001) than expected when calculated from rates of facial nerve preservation in surgically managed patients. Hearing preservation was also significantly more successful in conservatively managed patients (Pearson chi-square: P < 0.000). Conclusions:, An initial period of conservative management is a safe and reasonable management policy in all acoustic neuromas up to 2 cm in size at the cerebello-pontine angle. Given that there are no failsafe ways of deciding potential for growth in acoustic neuromas, initial conservative management of these tumours offers superior hearing and facial nerve preservation when compared with primary surgical treatment. [source]