scholarly journals Differences in Clinical Features and Diagnostic Strategies Between IgG4-Related Autoimmune Cholangitis and Cholangiocarcinoma

2021 ◽  
Vol 11 ◽  
Author(s):  
Ke Zhu ◽  
Jin Yang ◽  
Ying-zhen Chen ◽  
Xue-rong Zhang ◽  
Xian-huan Yu ◽  
...  

IgG4-related autoimmune cholangitis (IgG4-AIC) is often difficult to distinguish from cholangiocarcinoma (CCA). This study aimed to determine a practical clinical strategy for distinguishing between IgG4-AIC and CCA to avoid unnecessary surgical resection. We retrospectively collected and compared the clinicopathological data between IgG4-AIC and CCA patients, including the clinical, serological, and radiological characteristics, to follow up on these patients to investigate the prognosis. Among the 377 patients who received surgical resection for suspecting CCA at the Sun Yat-Sen Memorial Hospital between June 2004 and June 2014, 14 patients were diagnosed as IgG4-AIC through histochemistry after surgery. Immunohistochemistry revealed that IgG4 was up-regulated in the plasma cells of IgG4-AIC tissues in 13 out of 14 patients. The serum CA19-9 level was significantly lower than in the CCA group. Patients with IgG4-AIC can only see slight or no enhancement under the contrast enhancement CT scan, while there are no signs of ring-like or delayed enhancement that is unique to CCA. Thirteen patients were followed up, and the time was 12 to 92 months. Three of them were regularly treated with prednisone after surgery, and original symptoms disappeared. Our study demonstrated that the combination of imaging with serum CA19-9 could improve the preoperative diagnostic value and reduce the rate of unnecessary resection.

2019 ◽  
Vol 2 (1) ◽  
pp. 173-176
Author(s):  
Prajowl Shrestha ◽  
Ashesh Dhungana

Introduction: Saprophytic growth of Aspergillus species in preexisting lung cavities commonly presents with hemoptysis. Surgical resection is the preferred treatment for hemoptysis control in pulmonary aspergilloma. In patients, who are not candidates for surgical resection bronchoscopic Voriconazole instillation, is an effective option.Materials and Methods: In this retrospective study, data of patients presenting with active hemoptysis, radiological evidence of aspergilloma and those undergoing bronchoscopic Voriconazole instillation at National Academy of Medical Sciences from January 2018 to December 2018 were retrieved. Clinical details including the severity of hemoptysis, number, size and location of aspergilloma, number of Voriconazole instillation sessions and symptom control were assessed. Follow up CT scan after four to six sessions were also evaluated to compare the size of aspergilloma.Results: A total of 11 patients presented with aspergilloma and hemoptysis. Of these, five (45.4%) patients underwent at least four sessions of bronchoscopic Voriconazole instillation. Two patients required six sessions whereas one required nine sessions for hemoptysis control. The procedure was successful in all patients; however, one had a recurrence after 3 months and required bronchial artery embolisation. In four patients there was a reduction in the size of aspergilloma on follow up CT scan. The procedure was well tolerated by all the patients, except for a mild increase in cough in the immediate post-procedure period.Conclusions: Intrabronchial Voriconazole instillation is a safe and effective option for hemoptysis control in patients with pulmonary aspergilloma. However, the optimal dose, frequency, and duration of Voriconazole instillation need to be further evaluated.


2020 ◽  
pp. 014556132097192
Author(s):  
Guowei Lu ◽  
Qiang Zhang

Extramedullary plasmacytoma (EMP) is a malignant tumor formed by monoclonal abnormal proliferation of plasma cells, which is mainly characterized by localized masses and very rare in the larynx, particularly in the false vocal cord. We present a larynx EMP that arises from the false cord without systematic involvement. After surgical resection, the patient received adjuvant radiotherapy and was recurrence-free during the 8-month follow-up.


Author(s):  
S Wojcinski ◽  
A Farrokh ◽  
U Hille ◽  
E Hirschauer ◽  
W Schmidt ◽  
...  

2011 ◽  
Vol 50 (05) ◽  
pp. N57-N59
Author(s):  
S. Geiger ◽  
S. Horster ◽  
A. R. Haug ◽  
A. Hausmann ◽  
M. Schlemmer ◽  
...  

1996 ◽  
Vol 35 (04) ◽  
pp. 116-121 ◽  
Author(s):  
G. E Fueger ◽  
M. Vejda ◽  
R. M. Aigner

Summary Aim: To prevent orthopedic sequelae in acute hematogenous pyogenic osteomyelitis (AHPO) of infants early diagnosis, recognition of recurrence and effective therapy is needed. This retrospective study of 47 infants with bacteriologically confirmed AHPO concerned with an analysis of the diagnostic value of systemic serum parameters compared to bone scintigraphy (BSC). Methods: AHPO was characterized initially and during the course of disease by clinical findings, C-reactive protein (CRP), erythrocyte sedimentation rate (ESR), total and differential white blood cell (WBC) count, BSC, and plain radiography. Results: CRP was the most effective serum parameter for follow- up of disease. The first sign of BSC to signal adequate response to antibiotic treatment was the decrease or normalization of hyperperfusion. Escape from therapy or poor prognosis, even when the serum parameters were normalized, was signaled by the recurrence of focal hyperperfusion and the persistent or increasing local uptake ratios on the 3-h-image over 6 weeks during a course of antibiotic treatment. Conclusion: Antibiotic treatment masks the clinical presentation, and the radiographic findings, causes non-characteristic laboratory findings, but do not prevent the scintigraphic visualization; BSC and serum parameters used in the right completion are the most successful and efficient modalities for follow-up of AHPO. Maintenance of antibiotic therapy should be done until BSC findings have reverted to normal.


2020 ◽  
Author(s):  
Sissel Ravn ◽  
Henriette Vind Thaysen ◽  
Lene Seibaek ◽  
Victor Jilbert Verwaal ◽  
Lene Hjerrild Iversen

BACKGROUND Cancer survivors experience unmet needs during follow-up. Besides recurrence, a follow-up includes detection of late side effects, rehabilitation, palliation and individualized care. OBJECTIVE We aimed to describe the development and evaluate the feasibility of an intervention providing individualized cancer follow-up supported by electronic patient-reported outcomes (e-PRO). METHODS The study was carried out as an interventional study at a Surgical and a Gynecological Department offering complex cancer surgery and follow-up for advanced cancer. The e-PRO screened for a priori defined clinical important symptoms and needs providing individualized follow-up. We included following questionnaires in the e-PRO; the general European Organization for Research and Treatment of Cancer (EORTC) QLQ-C30 and the EORTC validated for colorectal and ovarian cancer patients. To support individualization, we included three prioritized issues of the patient’s preference in each e-PRO. The response-algorithm was aggregated based on the severity of the patient’s response. To ensure the sensitivity of the e-PRO, we performed semi-structured interviews with five patients. All clinicians (surgeons and gynecologists) performing the consultations reviewed the e-PRO. The evaluation was divided in two, 1)The feasibility was assessed by a)Patients’ response rate of the e-PRO, b)Number of follow-up visits documenting the use of e-PRO and c)Patients’ prioritized issues prior to the consultation(‘yes’ / ‘no’), and after the follow-up 2)Patients assessment of a)The need and purpose of the follow-up visit and b)the support provided during the follow-up visit. RESULTS In total, 187 patients were included in the study, of which 73%(n=136/187) patients responded to the e-PRO and were subjected to an individualized follow-up. The e-PRO was documented as applied in 79% of the follow-up visits. In total, 23% of the prioritized issues did not include a response. Stratified by time since surgery, significantly more patients did not fill out a prioritized issue had a follow-up >6 months since surgery. In total, 72 % follow-up visits were evaluated to be necessary in order to discuss the outcome of the CT scan, symptoms, and/or prioritized issues. Contrary, 19% of the follow-up visits were evaluated to be necessary only to discuss the result of the CT scan. A range from 19.3–56.3% of patients assessed the follow-up visit to provide support with respect to physical (42% of patients), mental (56%), sexual (19%) or dietary (27%) issues. Further, a range from 34–60% of the patients reported that they did not need support regarding physical (43% of patients), mental (34%), sexual (63%) or dietary (57%) issues. CONCLUSIONS An individualized follow-up based on e-PRO is feasible, and support most patients surgically treated for advanced cancer. However, results indicate that follow-up based on e-PRO may not be beneficial for all patients and circumstances. A thorough cost-benefit analysis may be warranted before implementation in routine clinic.


2021 ◽  
Vol 4 (Supplement_1) ◽  
pp. 234-236
Author(s):  
P Willems ◽  
J Hercun ◽  
C Vincent ◽  
F Alvarez

Abstract Background The natural history of primary sclerosing cholangitis (PSC) in children seems to differ from PSC in adults. However, studies on this matter have been limited by short follow-up periods and inconsistent classification of patients with autoimmune cholangitis (AIC) (or overlap syndrome). Consequently, it remains unclear if long-term outcomes are affected by the clinical phenotype. Aims The aims of this is study are to describe the long-term evolution of PSC and AIC in a pediatric cohort with extension of follow-up into adulthood and to evaluate the influence of phenotype on clinical outcomes. Methods This is a retrospective study of patients with AIC or PSC followed at CHU-Sainte-Justine, a pediatric referral center in Montreal. All charts between January 1998 and December 2019 were reviewed. Patients were classified as either AIC (duct disease on cholangiography with histological features of autoimmune hepatitis) or PSC (large or small duct disease on cholangiography and/or histology). Extension of follow-up after the age of 18 was done for patients followed at the Centre hospitalier de l’Université de Montréal. Clinical features at diagnosis, response to treatment at one year and liver-related outcomes were compared. Results 40 patients (27 PSC and 13 AIC) were followed for a median time of 71 months (range 2 to 347), with 52.5% followed into adulthood. 70% (28/40) had associated inflammatory bowel disease (IBD) (78% PSC vs 54% AIC; p=0.15). A similar proportion of patients had biopsy-proven significant fibrosis at diagnosis (45% PSC vs 67% AIC; p=0.23). Baseline liver tests were similar in both groups. At diagnosis, all patients were treated with ursodeoxycholic acid. Significantly more patients with AIC (77% AIC vs 30 % PSC; p=0.005) were initially treated with immunosuppressive drugs, without a significant difference in the use of Anti-TNF agents (0% AIC vs 15% PSC; p= 0.12). At one year, 55% (15/27) of patients in the PSC group had normal liver tests versus only 15% (2/13) in the AIC group (p=0.02). During follow-up, more liver-related events (cholangitis, liver transplant and cirrhosis) were reported in the AIC group (HR=3.7 (95% CI: 1.4–10), p=0.01). Abnormal liver tests at one year were a strong predictor of liver-related events during follow-up (HR=8.9(95% CI: 1.2–67.4), p=0.03), while having IBD was not (HR=0.48 (95% CI: 0.15–1.5), p=0.22). 5 patients required liver transplantation with no difference between both groups (8% CAI vs 15% CSP; p=0.53). Conclusions Pediatric patients with AIC and PSC show, at onset, similar stage of liver disease with comparable clinical and biochemical characteristics. However, patients with AIC receive more often immunosuppressive therapy and treatment response is less frequent. AIC is associated with more liver-related events and abnormal liver tests at one year are predictor of bad outcomes. Funding Agencies None


2020 ◽  
Vol 6 (1) ◽  
Author(s):  
Takuhisa Okada ◽  
Yasumitsu Hirano ◽  
Shintaro Ishikawa ◽  
Hiroka Kondo ◽  
Toshimasa Ishii ◽  
...  

Abstract Background Clear cell sarcoma-like tumor of the gastrointestinal tract (CCSLTGT) is extremely rare. It is a mesenchymal neoplasm that usually forms in the small intestine of adolescents and young adults, is prone to local recurrence and metastasis, and has a high mortality rate. We report a patient with CCSLTGT with lymph node- and liver metastases, who continues to survive 6 years after initial surgical resection. Case presentation A 38-year-old woman presented with lightheadedness. Laboratory analysis revealed anemia (hemoglobin, 6.7 g/dL), and enhanced computed tomography (CT) demonstrated a mass in the small intestine, about 6 cm in diameter, with swelling of 2 regional lymph nodes. Double-balloon small intestine endoscopic examination revealed a tumor accompanied by an ulcer; the biopsy findings suggested a primary cancer of the small intestine. She was admitted, and we then performed a laparotomy for partial resection of the small intestine with lymph node dissection. Pathologic examination revealed CCSLTGT with regional lymph node metastases. About 3 years later, follow-up CT revealed a single liver metastasis. Consequently, she underwent a laparoscopic partial liver resection. Histopathologic examination confirmed that the liver metastasis was consistent with CCSLTGT. It has now been 3 years without a recurrence. Conclusion Repeated radical surgical resection with close follow-up may be the only way to achieve long-term survival in patients with CCLSTGT.


Author(s):  
J. Hoevelmann ◽  
E. Muller ◽  
F. Azibani ◽  
S. Kraus ◽  
J. Cirota ◽  
...  

Abstract Introduction Peripartum cardiomyopathy (PPCM) is an important cause of pregnancy-associated heart failure worldwide. Although a significant number of women recover their left ventricular (LV) function within 12 months, some remain with persistently reduced systolic function. Methods Knowledge gaps exist on predictors of myocardial recovery in PPCM. N-terminal pro-brain natriuretic peptide (NT-proBNP) is the only clinically established biomarker with diagnostic value in PPCM. We aimed to establish whether NT-proBNP could serve as a predictor of LV recovery in PPCM, as measured by LV end-diastolic volume (LVEDD) and LV ejection fraction (LVEF). Results This study of 35 women with PPCM (mean age 30.0 ± 5.9 years) had a median NT-proBNP of 834.7 pg/ml (IQR 571.2–1840.5) at baseline. Within the first year of follow-up, 51.4% of the cohort recovered their LV dimensions (LVEDD < 55 mm) and systolic function (LVEF > 50%). Women without LV recovery presented with higher NT-proBNP at baseline. Multivariable regression analyses demonstrated that NT-proBNP of ≥ 900 pg/ml at the time of diagnosis was predictive of failure to recover LVEDD (OR 0.22, 95% CI 0.05–0.95, P = 0.043) or LVEF (OR 0.20 [95% CI 0.04–0.89], p = 0.035) at follow-up. Conclusions We have demonstrated that NT-proBNP has a prognostic value in predicting LV recovery of patients with PPCM. Patients with NT-proBNP of ≥ 900 pg/ml were less likely to show any improvement in LVEF or LVEDD. Our findings have implications for clinical practice as patients with higher NT-proBNP might require more aggressive therapy and more intensive follow-up. Point-of-care NT-proBNP for diagnosis and risk stratification warrants further investigation.


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