Diagnosis Of FS Research Articles

CT imaging features of lung ground-glass nodule patients with upgraded intraoperative frozen pathology

PurposeIntraoperative frozen section pathology (FS) is widely used to guide surgical strategies while the accuracy is relatively low. Underestimating the pathological condition may result in inadequate surgical margins. This study aims to identify CT imaging features related to upgraded FS and develop a predictive model.MethodsCollected data from 860 patients who underwent lung surgery from January to December 2019. We analyzed the consistency rate of FS and categorized the patients into three groups: Group 1 (n = 360) had both FS and Formalin-fixed Paraffin-embedded section (FP) as non-invasive adenocarcinoma (IAC); Group 2 (n = 128) had FS as non-IAC but FP as IAC; Group 3 (n = 372) had both FS and FP as IAC. Clinical baseline characteristics were compared and propensity score adjustment was used to mitigate the effects of these characteristics. Univariate analyses identified imaging features with inter-group differences. A multivariate analysis was conducted to screen independent risk factors for FS upgrade, after which a logistic regression prediction model was established and a receiver operating characteristic (ROC) curve was plotted.ResultsThe consistency rate of FS with FP was 84.19%. 26.67% of the patients with non-IAC FS diagnosis were upgraded to IAC. The predictive model’s Area Under Curve (AUC) is 0.785. Consolidation tumor ratio (CTR) ≤ 0.5 and smaller nodule diameter are associated with the underestimation of IAC in FS.ConclusionCT imaging has the capacity to effectively detect patients at risk of upstaging during FS.

P26 Felty’s syndrome as the first presentation of rheumatoid arthritis

Abstract Introduction Felty's syndrome (FS) is a rare, autoimmune disease occurring in < 1% of patients with rheumatoid arthritis (RA) and comprising a triad of persistent idiopathic neutropenia, splenomegaly and RA. In some affected individuals, FS may develop when the symptoms and signs of RA have subsided or are absent. In this case, FS may remain undiagnosed. In rare instances, the development of FS may precede the development of the clinical manifestation of RA. Case description A 73-year-old Caucasian gentleman presented in October 2017 with pancytopenia and splenomegaly. He was investigated by the haematology team with multiple bone marrow biopsies showing no underlying haematological causes for his pancytopenia. He was also investigated by the hepatologists for the possibility of liver cirrhosis as he had a heavy alcohol intake. His liver ultrasound showed fatty liver with splenomegaly, his fibro-scan showed fibrosis with no evidence of cirrhosis and his OGD showed no oesophageal varies. At this point, he was treated with blood transfusions, weekly erythropoietin and filgrastim as needed under the haematologists. In October 2022, he was admitted with febrile neutropenia and he gave a 20-year history of palindromic rheumatism with no evidence of synovitis or previous DMARD treatment. His rheumatoid factor (RF) was >120 iu/ml, anti-cyclic citrullinated peptide (ACCPA) was >340 u/ml and ANA by CIA was weakly positive (20.6 CU) with Hep-2 IFF being homogenous. ENA and dsDNA were negative. His hand X-ray showed evidence of osteoarthritis with no erosion. He subsequently had multiple admissions due to infections. CT chest, abdomen, and pelvis showed splenomegaly with portal vein dilatation suggestive of portal hypertension, normal liver appearance, and left lower lung bronchiectasis. Liver biopsy showed non-specific portal and sinusoidal inflammation, mild fibrosis, and architectural features suggestive of nodular regenerative hyperplasia indicative of FS. Furthermore, his HLA-DRB1*04 was positive. Together, these factors allowed us to diagnose FS. He was started on a tapered course of prednisolone, 30mg daily decreased by 10mg every two weeks, and methotrexate (10mg orally weekly) with careful blood monitoring. Two weeks later, he developed a mild rise in the ALT and alkaline phosphatase. Therefore, the methotrexate dose was reduced to 5mg weekly. Four weeks later, he reported feeling much better, with improvement in all his cell counts and liver function. Discussion While persistent idiopathic neutropenia and splenomegaly (present in 90% of FS patients) are salient features of FS, patients can also present with other extra-articular manifestations such as rheumatoid nodules (74%), hepatomegaly (68%), lymphadenopathy (42%), Sjögren's syndrome (48%), pulmonary fibrosis (50%), pleuritis (22%), peripheral neuropathy (14%) and leg ulcers (16%). Systemic symptoms include fever and weight loss. They also present with idiopathic non-cirrhotic portal hypertension, which can lead to variceal bleeding. The exact pathophysiology of FS is unclear, making the diagnosis and treatment of FS very challenging. A large multi-central cohort study found that FS is associated with HLA-DRB1*0401 and, in two other studies into the pathophysiology of FS, they found that 73% of patients with FS had IgG Anti-G-CSF and autoantibodies that bind to deaminated histones and neutrophil extracellular chromatin traps (NETs), leading to neutrophil sequestration. Therefore, treatment aims to control the underline RA, improve the neutrophil count, and prevent infection. The first challenge, in this case, was to confirm the diagnosis of FS in the absence of any obvious RA joint involvement and a history of heavy alcohol intake. Following an extensive investigation, we discussed his case at the Rare Disease MDT meeting and liaised with the haematology, hepatology, and histopathology teams to exclude the other differential diagnoses. The clues to the diagnosis of FS were the strongly positive RF and ACCPA, the positive HLA-DRB1*04, the liver biopsy, and the exclusion of other causes of neutropenia. The second challenge in this case was to safely start methotrexate, given his mild liver fibrosis and recurrent infections. With close blood test monitoring and follow-up, he achieved improvement in his cell count (WBC from 0.8 to 1.1x10 9/L, HB from 86 to 104 g/L, platelet from 70 to 91X109/L) with stable liver function. Key learning points Rheumatoid arthritis is a multisystem disease with variable presentations. Rarely, FS can occur in the absence of clinical manifestations of the erosive joint involvement of RA. In this situation, careful, thorough investigation to exclude other causes of neutropenia and prove the diagnosis of FS is imperative to justify immunosuppressive treatment. A multidisciplinary team working across specialties was pivotal in the safe diagnosis and treatment of this gentleman.

Sex/Gender Differences in the Language Profiles of Italian Children with Autism Spectrum Disorder: A Retrospective Study.

Sex/gender (S/G) differences in ASD language profiles have been poorly investigated. The present study aims to explore whether male (M) and female (F) children with ASD and with normal non-verbal cognitive abilities differ in their linguistic profiles. A sample of 76 Italian children with ASD (range: 4.9-8 years), including 50 Ms and 26 Fs, was retrospectively recruited. Language profiles were analyzed using standardized tests for the evaluation of receptive and expressive vocabulary as well as grammar. Grammatical comprehension was the most impaired domain compared to the other language measures in both M and F children. Comparing language profiles between S/G, Fs showed significantly better scores than Ms in grammatical production (p = 0.002), and Ms showed better active negative sentence comprehension (p = 0.035). Moreover, comparing the language profiles between Ms and Fs with a receptive disorder, Fs had significantly worse grammatical comprehension and better grammatical production than Ms. Even among children without a receptive disorder, Fs had significantly higher grammatical production scores. The S/G differences in language profile, particularly better expressive language in Fs than Ms, can partially contribute to the delayed ASD diagnosis or underdiagnosis of Fs without intellectual disability. Finally, the results document the importance of accurately investigating both expressive and receptive abilities in children with ASD.

Comparison of efficacy of intra articular steroid injection versus manipulation under anesthesia (MUA) in idiopathic frozen Shoulder.

Objective: To compare efficacy of intra-articular steroid (IAS) injection with manipulation under anesthesia (MUA) in idiopathic FS. Study Design: Randomized Control Trail. Setting: Department of Orthopedic, Independent University Hospital, (IMC) Faisalabad. Period: 1st January 2021 to 30th June 2021. Material & Methods: Diagnosis of FS was based upon detailed history and proper physical. Group A (n=50) included intra-articular injection patients and Group B (n=50) was patients receiving MUA. Pain was measured by VAS and disability was measured by Disability Index (SPADI) scale. Results: Mean age of sample was 37 ± 10.52 in group A and 39.14± 9.48 in group B. Age of patients ranges from 35 to 50 years. Female patients were greater in both the groups. Most of the patients have duration of disease for more than 1 month. The ratio of patients with right side disease was more than 50% in both the groups as compared to left side. In group A. there was a significant decrease in mean pain from 3.45 ± 1.12 to 2.16 ± 0.8 In group B, mean pain score decreased from 3.65 ± 0.88 to 2.35 ± 0.68 1. Overall group A was more effective as compared to group B but the results were not significant statistically. Conclusion: The intra-articular injection method was superior to MUA because it was easier, safer, and less expensive, and it produced early results.

Precentral gyrus and insula responses to stress vary with duration to diagnosis in functional seizures.

This study was undertaken to determine whether undiagnosed illness duration (time between functional seizures [FS] onset and diagnosis) is linked to differences in neural response and functional connectivity during processing of stressful experiences. Forty-nine participants with traumatic brain injury preceding the onset of FS confirmed by video-electroencephalography were recruited prospectively. Participants completed psychiatric symptom assessments before undergoing functional magnetic resonance imaging (fMRI) with an acute psychosocial stress task. Linear mixed effects (LME) analyses identified significant interactions between the factors of group (early vs. delayed diagnosis) and time lag to diagnosis on neural responses to stressful math performance and auditory feedback (corrected α=.05). Functional connectivity analysis utilized clusters from initial LME analyses as seed regions to determine significant interactions between these factors on network functional connectivity. Demographic and psychiatric symptom measures were similar between early (n=25) and delayed (n=24) groups. Responses to stressful math performance within the left anterior insula and functional connectivity between the anterior insula seed region and a precentral gyrus cluster were significantly negatively correlated with time lag to diagnosis for the early but not the delayed FS diagnosis group. There was no correlation between fMRI findings and psychiatric symptoms. This study indicates that aberrant left anterior insula activation and its functional connectivity to the precentral gyrus underlie differences in processing of stressful experiences in patients with delayed FS diagnosis. Follow-up comparisons suggest changes are associated with undiagnosed illness duration rather than psychiatric comorbidities and indicate a potential mechanistic association between neuropathophysiology, response to stressful experiences, and functional neuroanatomy in FS.

A Retrospective Study on Turnaround Time for Frozen Sections- A Tertiary Care Centre Experience from Southern India

Introduction: Intraoperative consultation by frozen sections is an integral and essential part of surgical pathology to provide critical real-time information and guide in further intraoperative management. Diagnostic accuracy and Turnaround Time (TAT) have been considered as essential parameters which decide its effectiveness. Although diagnostic accuracy has been studied extensively, very few studies have assessed TAT of frozen sections. Aim: To assess the TAT for frozen sections in a tertiary center and identifying the various practical factors determining it. Materials and Methods: This retrospective study was conducted in Department of General Pathology at Christian Medical college, Vellore, Tamil Nadu, India, on 615 frozen section samples obtained during the time period of June 2020 to June 2021. The TAT was defined as the time interval between receipt of sample and the time at which report was conveyed to the surgeon. The TAT for frozen sections should not exceed 30 minutes as per the criteria proposed by National Accreditation Board for Testing and Calibration Laboratories (NABL). Further details such as number of sites per frozen, number of pathologists involved, number of tissue blocks and slides made, requirement of deeper sections and special stains were documented and the factors influencing TAT were analysed based upon their subspecialty. Results: A total of 35,175 specimens were received during the study period, of which 615 cases had frozen sections. Out of 615, 16.9% had TAT of more than 30 minutes, however when only one tissue block was submitted (n=221), 90% were within TAT. The delay in TAT was likely to occur when more than two pathologists participated in the FS diagnosis, more number of sections/tissue blocks were required, the pathologist had to retrieve and review previous case material during the FS procedure, simultaneously receiving additional specimens and requirement of special stains. Conclusion: Turnaround time for frozen sections depends on several preanalytical and analytical reasons. An overall TAT that includes these factors cannot be taken as a standalone quality indicator of the laboratory. Therefore, a checklist that includes specimen receiving time, slide receiving time and time at which the diagnosis was conveyed to the surgeon needs to be documented to help understand where the delay happens. A periodic assessment of intraoperative frozen section TAT should be an integral part of an ongoing quality assurance program.

P-56 LOW ROLE OF NON-INVASIVE FIBROSIS ASSESSMENT USING FIB-4 AND APRI IN PATIENTS WITH AUTOIMMUNE HEPATITIS

The evaluation with non-invasive tests (NIT) of liver fibrosis is a more accessible method to estimate risk in patients with liver disease. The APRI and FIB-4 are scores that use easily accessible laboratory variables. They have been validated mainly in viral hepatitis and non-alcoholic fatty liver (NAFLD), but their usefulness in autoimmune hepatitis (HAI) has been little studied. To evaluate the usefulness of APRI and FIB-4 in the screening of significant fibrosis (SF) in patients with HAI. Observational, cross-sectional and retrospective study that includes liver biopsies performed between 2015-2018. The presence and degree of fibrosis were recorded according to the METAVIR scale; F3-F4 is considered FS. Histological diagnoses and clinical data were recorded. 93 HAI liver biopsies were analyzed; 80% women; average age 52 (18-82) years. Fibrosis present in 69% (F0: 29, F1: 12; F2: 10, F3: 15, F4: 27). FS at 45.2%. The diagnostic concordance (kappa index) of FS by biopsy and FIB-4 (> 3.25) was acceptable, but not for APRI (> 0.7). The ROC curve for APRI was only 0.58 and for FIB-4 0.75. With the cutoff of 0.7 the APRI had a sensitivity of 94%, but a specificity of only 10% and with the cutoff of 3.25 the FIB-4 had a sensitivity of 72% and specificity of 69%, for the diagnosis of FS. In HAI the usefulness of NIT fibrosis evaluation using APRI and FIB-4 was scarce. FIB-4 could be more useful, but liver biopsy remains important for staging and prognosis.

Ictal injury: Epilepsy vs. functional (psychogenic) seizures

ObjectiveThe aim of the current study was to compare the risk and also the types of ictal injuries in three groups of people with seizures [i.e., IGE vs. TLE vs. FS]. MethodsThis was a retrospective study. All patients with an electro-clinical diagnosis of IGE, TLE, or FS were recruited at the outpatient epilepsy clinic at Shiraz University of Medical Sciences, Shiraz, Iran, from 2008 until 2020. Age, sex, age at seizure onset, seizure type(s), and occurrence of ictal injury at any time since the onset of the seizures and its characteristics were registered routinely for all patients at the time of the first visit. ResultsOne thousand and one hundred seventy-four patients were studied (481 patients with IGE, 402 people with TLE, and 291 persons with FS). While the groups differed in their demographic and clinical characteristics, the rates of ictal injury did not differ significantly between the groups. Tongue injury was more frequently reported by patients with TLE compared with that by people with IGE or FS. Other types/locations of ictal injury were more or less reported by all three groups of the patients. ConclusionIctal injuries may happen with more or less similar rates among people with epilepsy (IGE and TLE) and those with FS. Ictal injury (rate, type, or location) should not be used as a marker for any specific diagnosis among people with seizures.

Современные возможности пренатальной и постнатальной диагностики синдрома Фринса: собственные наблюдения и обзор литературы

Fryns syndrome (FS, OMIM#194050) is a rare lethal autosomal recessive disorder with unknown genetic defect, manifested the distinct complex of the multiply congenital abnormalities (MCA), including diaphragmatic hernia. There was made the world literature overview for the FS phenotypic features, clinical and genealogical data of the published familial cases, ultrasound and morphological characteristics of affected fetuses. Aim . Analysis of the clinical, morphological and prenatal ultrasound data of 12 FS cases, detected in Belarus during 2009-2018 years, and families’ reproduction results. Material and methods . Genetic counseling of 10 families with affected outcome was performed. All the pregnants underwent the combined (biochemical and ultrasound) screening. Prenatal and postnatal cytogenetical studies (GTG-banding) and the morphological investigations of the 10 aborted fetuses and 2 deceased infants were fulfilled. Results and Discussion . All the patients presented typical FS phenotypic features correlated with the diagnostic criteria. The spectrum of prenatal findings diagnosed at 1-st trimester included nuchal skin enlargement/cystic hygroma (5 cases), at the 2-ed trimester – diaphragmatic hernia (11 cases), omphalocele (1), other malformations (4 fetuses). The facial dysmorphisms (coarse face) were detected in all the patients. Lungs hypoplasia was found in all cases, 5 patients additionally dysplayed other abnormalities: pulmonary sequestration, cystic-adenomatoid malformation, lobular aplasia. Characteristic phalanges and nails hypoplasia or aplasia were registered in 11 cases, 3 sibs presented rare malformations – polydactyly (hands) and ectrodactyly (feet). Associated malformations spectrum included cleft palate, brain abnormalities (ventricular dilatation, arhinencephaly), cardiac defects, omphalocele, Meckel diverticule, hydronephroses, renal cysts, bicornuate uterus and hypospadia. The familial case with 3 affected sibs demonstrated a similarity in main diagnostic criteria. The couples reproduction analysis illulustrated a serious failure. Outcome of 29 pregnancies were as follows: 12 – FS cases, 4 – miscarries at 1-st trimester, 11 – healthy offspring and 2 cases – pregnancies are going on currently (both fetuses without malformations). Conclusion . Clinical diagnosis of FS may be noted in patients (either fetuses or newborns), which display the conjunction of typical MCA pattern with a normal karyotype. Additional investigations must be used in order to exclude the associated abnormalities if diaphragmatic hernia has been detected in fetus. In our opinion polydactyly and ectrodactyly may be added to the FS phenotypic spectrum.

The predictors of acute bacterial meningitis among children presenting as first complex febrile seizure

The association between febrile seizure and bacterial meningitis is well established. Although FS induced by age related hyper-excitability of the brain to fever, determining the cause of the fever is critical in the evaluation of these children. It is therefore imperative to rule out BM prior to making the diagnosis of FS. However, the diagnosis of FS in certain subgroups of children with an apparent FS is a challenge: It is mandatory to look for signs of raised intracranial pressure i.e. bulging fontanel, papilloedema and signs of meningeal irritation to exclude meningitis. After approval from the institutional ethics committee, the study was initiated. The parents of children admitted to our institute who were fulfilling the inclusion criteria were informed about this study. Only those patients presenting as first complex febrile seizures are included in the study. Written informed consent from the parents of patients admitted in paediatric wards of Post Graduate Institute of Paediatric Sciences were taken. All cases of ABM had positive CRP compared to the 19% (n=22) cases without bacterial meningitis had positive CRP. Out of total 125 cases, 96.8% (n=122) had negative blood culture, 3.2% (n=4) had positive blood cultures and the blood culture was positive in 3 (33.3%) of ABM cases and 1 (0.8%) with NO ABM. This difference between positive blood culture and ABM was statistically significant.

2403 Adefovir-Induced Fanconi Syndrome: Missed Diagnosis and Misdiagnosis

INTRODUCTION: Nucleotide reverse transcriptase inhibitors (NRTIs), e.g., adefovir (ADV), are commonly used to treat chronic hepatitis B, which affects approximately 240 million worldwide and 2.2 million in the US. We report a case of ADV-induced Fanconi Syndrome (AIFS) in a patient who was undiagnosed for 5 y. CASE DESCRIPTION/METHODS: A 75 yo Spanish female with history of osteoporosis and CHB on ADV 10 mg/d for 15 y presents to the ED for bilateral hip pain. Over the past 2 y, her hip pain was refractory to steroid injections and complicated by traumatic bilateral hip and atraumatic clavicular fractures. One year ago she was diagnosed with osteoporosis and started on teriparatide (synthetic parathyroid hormone). Exam reveals bilateral hip tenderness and she is unable to bear weight. Labs reveal renal phosphorous wasting and glucosuria, proteinuria, hyperaminoaciduria and low fibroblast growth factor 23 confirm diagnosis of FS (Table 1). Bone scan is consistent with osteomalacia (Figure 1). Teriparatide is stopped to prevent worsening of osteomalacia. Lab review shows otherwise normal phosphorous prior to starting ADV (Table 2), and patient is diagnosed with AIFS. Phosphorous supplementation is started and ADV is changed to entacavir (ETV) due to decreased renal toxicity. Patient reports complete resolution of hip pain within 1 week, over which time labs also improve (Table 2). DISCUSSION: heavy metal or drug toxicity. Classic features include hyperphosphaturia, hyperuricosuria, hypercalciuria, modest proteinuria and glycosuria. In addition to hyperchloremic acidosis, rickets or osteomalacia are the predominant effects of FS. Osteomalacia can exist despite normal calcium and vitamin D levels. FS and osteomalacia affect 30% and 10% of patients on long-term NRTI therapy, respectively. Nephrotoxicity is reported at dosages of 10-120 mg. Risk factors for developing AIFS include old age, low BMI and >8 y treatment with ADV, all of which apply to this patient. While this patient's CMP was checked yearly, her metabolic derangements were overlooked for 5 y. Treatment of her osteoporosis likely worsened her underlying FS. This highlights the need for awareness of this clinically significant syndrome as a more timely diagnosis may have prevented fractures. While AIFS is well reported in Endocrine literature, it is not as widely reported in GI literature, which may contribute to this knowledge gap in Gastroenterologists. GI clinicians should consider FS in patients on longstanding ADV with low phosphorous.

Role of frozen section in the intraoperative management of ovarian masses

Abstract Objective To evaluate the performance and limitations of FS in the intraoperative evaluation of ovarian masses. Design Retrospective descriptive study. Methods The case records of patients presenting with ovarian masses who underwent surgery and intraoperative FS assessment between January 2009 and December 2012 were analyzed. Demographic and clinical data were reviewed. Data on FS analysis were compared with the final diagnosis on paraffin section. Results Sixty patients with ovarian masses undergoing surgery and FS were included. Four cases had the diagnosis at the time of FS deferred (6.6%). In the remaining 56 patients, the FS diagnoses were benign in 24 (40%), borderline in 9 (15%), and malignant in 23 (38.4%), whereas the final diagnosis was benign in 23 (38.4%), borderline in 11 (18.3%), and malignant in 26 (43.3%). The overall accuracy of intra-operative FS diagnosis was 95.5%. The sensitivity for FS diagnosis was 100% for benign, 72.7% for borderline and 88.4% for malignant category, whereas the specificity was 97.3%, 97.9%, and 100.0%, respectively. There were 4 cases with discordance between the FS diagnoses and the final diagnoses, all of which were under-diagnosed by FS. Conclusion Frozen section is a good tool for decision making at the time of ovarian surgery but does not always provide an immediate solution. However a large prospective study is recommended.

Diagnostic accuracy of frozen-section analysis of cancer-containing bladder transurethral resection specimens for the presence of muscularis propria invasion

AimsFrozen section (FS) consultation is generally an accurate diagnostic modality. At our institution, we are frequently asked to assess transurethral resection specimens (TURBT) at FS for muscularis propria (MP) invasion...

EpiFibro – a nationwide databank for fibromyalgia syndrome – the initial analysis of 500 women

IntroductionFibromyalgia syndrome (FS) is a common painful condition of the musculoskeletal system that is typically accompanied by several symptoms in other systems. In Brazil, the prevalence of FS is estimated at 2.5%. Here, we present the initial data from Epi-Fibro, a nationwide databank of FS patients seen in public and private settings. ObjectiveThe aims of this study were to assess how the diagnosis of FS was made, identify a set of clinical domains considered relevant by both clinicians and patients in cases of FS, analyse the impact of disease on patient quality of life, and compare the findings among patients of public and private services. MethodsBased on the results of questionnaires, we analysed data corresponding to the first 500 women in the database. Questionnaires pertaining to demographic and clinical data and the Fibromyalgia Impact Questionnaire (FIQ), which was translated and validated for Brazilian patients, were completed by the clinicians and/or patients. ResultsPreliminary analysis of the EpiFibro databank revealed that female FS patients in Brazil reported a high impact of disease, as measured by the FIQ, a high prevalence of associated symptoms, and a low degree of education (consistent with the public health care in Brazil used mainly by the underserved). In addition, most patients perceived their pain as diffuse from the onset of disease. ConclusionDepression and anxiety were seen as the main triggers of FM symptoms, but a significant proportion of the subjects perceived work strain as the initial trigger. We also observed a delay of a few years in seeking medical help and examination by a rheumatologist.

EpiFibro – um banco de dados nacional sobre a síndrome da fibromialgia – análise inicial de 500 mulheres

IntroductionFibromyalgia syndrome (FS) is a common painful condition of the musculoskeletal system that is typically accompanied by several symptoms in other systems. In Brazil, the prevalence of FS is estimated at 2.5%. Here, we present the initial data from Epi- Fibro, a nationwide databank of FS patients seen in public and private settings. ObjectiveThe aims of this study were to assess how the diagnosis of FS was made, identify a set of clinical domains considered relevant by both clinicians and patients in cases of FS, analyse the impact of disease on patient quality of life, and compare the findings among patients of public and private services. MethodsBased on the results of questionnaires, we analysed data corresponding to the first 500 women in the database. Questionnaires pertaining to demographic and clinical data and the Fibromyalgia Impact Questionnaire (FIQ), which was translated and validated for Brazilian patients, were completed by the clinicians and/or patients. ResultsPreliminary analysis of the EpiFibro databank revealed that female FS patients in Brazil reported a high impact of disease, as measured by the FIQ, a high prevalence of associated symptoms, and a low degree of education (consistent with the public health care in Brazil used mainly by the underserved). In addition, most patients perceived their pain as diffuse from the onset of disease. ConclusionDepression and anxiety were seen as the main triggers of FM symptoms, but a significant proportion of the subjects perceived work strain as the initial trigger. We also observed a delay of a few years in seeking medical help and examination by a rheumatologist.

Intraoperative frozen section assessment in the evaluation of axillary sentinel lymph node in breast cancer

INTRODUCTION: Intraoperative frozen section analysis has become a routine procedure to evaluate the status of axillary sentinel lymph nodes in breast cancer. OBJECTIVES: To evaluate the accuracy and sensitivity of FS in the detection of metastases in axillary sentinel lymph nodes and to investigate the predictive value of variables such as patients' age, tumor staging, histology, grade, and estrogen receptor expression. MATERIAL AND METHODS: We analyzed retrospectively the results of 177 FS procedures. The patients' age and tumor characteristics were organized in a database and the association with the presence of metastases was analyzed. RESULTS: Metastases were detected in 22 cases (12%). All macrometastases and one micrometastasis were detected by FS. Additional micrometastases were detected in post-operative analysis, from which five were determined by hematoxylin and eosin staining (H) and three by immunohistochemistry (IHC). FS diagnosis data proved to have an overall accuracy of 95%, sensitivity of 64%, and specificity of 100%. None of the analyzed variables showed significant association with lymph node metastases. CONCLUSION: Our results show that intraoperative FS is a highly accurate and sensitive method to detect macrometastases. However, it is inaccurate in the detection of micrometastases. The use of IHC improves the detection of micrometastases in postoperative analyses. The patient's age and tumor characteristics such as staging, histology, grade and estrogen receptor expression have low predictive value for lymph node metastasis in breast cancer.

Recruitment of infants with sickle cell anemia to a Phase III trial: Data from the BABY HUG study

BackgroundProtocol-eligible subjects may not be candidates for research participation or may decline. To determine factors that affected accrual, we evaluated enrollment in BABY HUG, a multi-center, randomized, placebo-controlled Phase III trial of hydroxyurea (HU) in infants with sickle cell anemia. MethodsAn anonymized registry of potential subjects served as the primary source of data. Study coordinators considered all infants less than age 18months with a hemoglobin FS diagnosis on newborn screening. Data included the number of potentially eligible subjects, whether parents were approached, and reasons for participating or declining. ResultsOf 1106 potential participants, 28% were not approached for reasons such as prior poor adherence to clinical care. Interested families expressed willingness to contribute to medical knowledge (51%), hope of being randomized to receive hydroxyurea (51%), and desire for closer clinical care (51%) as reasons for participating. Disease severity or the perception that their child was ill had less impact on willingness to participate (16%). Parents who declined cited fear of research (19%), transportation problems (14%), and the demanding nature of the study (25%). Ultimately, 234 (21%) gave informed consent, with little variability of acceptance rates among sites. Importantly, the number of subjects enrolled correlated with the number of families that were approached. Sites that excluded patients based on clinical/psychosocial biases were not more successful in recruiting than those who approached all eligible subjects. ConclusionLarge, demanding clinical trials require an adequate pool of potential participants. Approaching all potentially eligible patients without predetermined biases enhances success in recruitment.

Response to letter to the editor by Zenker? ?Diagnosis of FS should not be made until PKS is ruled out?

Response to letter to the editor by Zenker? ?Diagnosis of FS should not be made until PKS is ruled out?

IS IT JUVENILE RHEUMATOID ARTHRITIS OR FIBROMYALGIA

For the clinician evaluating adolescents with chronic musculoskeletal pain and fatigue, the distinctions between JRA and FS are clear based on physical examination findings. The two conditions can coexist. For the patient with an initial diagnosis of either JRA or FS whose clinical response to therapy is not in keeping with expectations or physical examination findings or whose clinical course worsens without explanation, reevaluation to determine if FS in the JRA patient has developed or JRA in the FS patient has emerged is warranted. Until clinicians have a better understanding of the intricacies of the neurohormonal and immunologic systems and how they affect somatic symptoms, they can continue to provide patients with a treatment plan based on current knowledge that should minimize patients' discomfort and allow them to have appropriately functional lives.

Experience with a Dynamic Inexpensive Video-Conferencing System for Frozen Section Telepathology

Aim: To evaluate the feasibility of an inexpensive, generally applicable video‐conferencing system for frozen section telepathology (TP). Methods: A commercially widely available PC‐based dynamic video‐conferencing system (PictureTel LIVE, model PCS 100) has been evaluated, using two, four and six ISDN channels (128–384 kilobits per second (kbs)) bandwidths. 129 frozen sections have been analyzed which were classified by TP as benign, uncertain (the remark probably benign, or probably malignant was allowed), malignant, or not acceptable image quality. The TP results were compared with the original frozen section diagnosis and final paraffin diagnosis. Results: Only 384 kbs (3 ISDN‐2 lines) resulted in acceptable speed and quality of microscope images, and synchronous image/speech transfer. In one of the frozen section cases (0.7%), TP image quality was classified as not acceptable, leaving 128 frozen sections for the analysis. Five of these cases were uncertain by TP, and also deferred by frozen section procedure (FS). One more benign and three malignant FS cases were classified as uncertain by TP. Three additional cases were uncertain by FS, but benign according to TP (in agreement with the final diagnosis). In one case, FS diagnosis was uncertain but TP was malignant (in agreement with the final diagnosis). Thus, test efficiency (i.e., cases with complete agreement) was 120/128 (93.8%, Kappa = 0.88) between FS and TP. Sensitivity was 93.5%, specificity 98.6%, positive and negative predictive values were 97.7% and 96.0%. Between TP and final diagnosis agreement was even higher. More importantly, there was not a single discrepancy as to benign‐malignant. Moreover, there was a clear learning effect: 5 of the 8 FS/TP discrepancies occurred in the first 42 cases (5/42=11.9%), the remaining 3 in the following 86 cases (3/86=3.5%). Discussion: The results are encouraging. However, TP evaluation is time‐consuming (5–15 min for one case instead of 2–4 min although speed went up with more experience) and is more tiring. The system has the following technical drawbacks: no possibility to point at objects or areas of interest in the life image at the other end, resolution (rarely) may become suboptimal (blocky), storage of images evaluated (which is essential for legal reasons) is not easy and no direct control of a remote motorized microscope. Yet, all users were positive about the system both for telepathology and personal contact by video‐conferencing. Conclusion: With a relatively simple videoconferencing system, accurate dynamic telepathology frozen section diagnosis can be obtained without false positive or negative results, although a limited number of uncertain cases will have to be accepted.