scholarly journals Hypophyseal Involvement in Immunoglobulin G4-Related Disease: A Retrospective Study from a Single Tertiary Center

2018 ◽  
Vol 2018 ◽  
pp. 1-9 ◽  
Author(s):  
Yang Liu ◽  
Linjie Wang ◽  
Wen Zhang ◽  
Hui Pan ◽  
Hongbo Yang ◽  
...  
Keyword(s):  
Medical Center ◽  
Immunosuppressive Agents ◽  
Clinical Manifestations ◽  
Surgical Excision ◽  
Pituitary Function ◽  
Pituitary Mass ◽  
Immunoglobulin G4 ◽  
Serum Igg4 ◽  
Tertiary Center

This study aims to outline the clinical features and outcomes of IgG4-related hypophysitis (IgG4-RH) patients in a tertiary medical center. We reviewed clinical manifestations and imaging and pituitary function tests at baseline, as well as during follow-up. Ten patients were included. The mean age at diagnosis of IgG4-RH was 48.4 (16.0–64.0) years. An average of 3 (0–9) extrapituitary organs were involved. Five patients had panhypopituitarism, three had only posterior hypopituitarism, one had only anterior hypopituitarism, and one had a normal pituitary function. One patient in our study had pituitary mass biopsy, lacking IgG4-positive cells despite lymphocyte infiltration forming an inflammatory pseudotumor. Five patients with a clinical course of IgG4-RH less than nine months and a whole course of IgG4-RD less than two years were managed with glucocorticoids, while three patients with a longer history were administered glucocorticoids plus immunosuppressive agents. One patient went through surgical excision, and one patient was lost to follow-up. All patients showed a prompt response clinically, but only three patients had normalized serum IgG4 levels. Two patients who took medications for less than six months relapsed. Conclusions. IgG4-RD is a broad disease, and all physicians involved have to be aware of the possibility of pituitary dysfunction. Younger patients should be expected. The histopathological feature of pituitary gland biopsy could be atypical. For patients with a longer history, the combination of GC and immunosuppressive agents is favorable. Early and adequate courses of treatment are crucial for the management of IgG4-RH. With GC and/or immunosuppressant treatment, however, pituitary function or diabetes insipidus did not improve considerably.

scholarly journals Immunoglobulin G4-related sclerosing cholangitis mimicking cholangiocarcinoma: a case report and literature review

2020 ◽  
Vol 48 (10) ◽  
pp. 030006052095921
Author(s):  
Cheng Xu ◽  
Yongmei Han
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Elevated Serum ◽  
Clinical Manifestations ◽  
Immunoglobulin G4 ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
History Of ◽  
The Common

Immunoglobulin G4 (IgG4)-related disease (IgG4-RD) is a novel clinical disease that is characterized by elevated serum IgG4 concentrations and tumefaction or tissue infiltrated by IgG4+ plasma cells. The clinical manifestations of IgG4-RD depend on the type of tissues affected. IgG4-related sclerosing cholangitis is a type of IgG4-RD. We report a patient who initially visited a local hospital with a 5-month history of jaundice. He was found to have a mass in the upper part of the common bile duct that mimicked cholangiocarcinoma. He underwent surgery in our hospital and was later diagnosed with IgG4-related sclerosing cholangitis. We administered prednisolone 40 mg once a day for treatment. Taking into account the possible side effects of moderate-dose hormone therapy, we also administered teprenone, potassium chloride, and calcium carbonate. The patient did not have any recurrence of symptoms or adverse drug reactions during follow-up.

scholarly journals AB1036 CLINICAL MANIFESTATIONS, CLINICAL COURSE, AND OUTCOMES OF IMMUNOGLOBULIN G4 RELATED DISEASE

2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1809.1-1809
Author(s):  
W. Katchamart ◽  
K. Phaopraphat ◽  
P. Ngamjanyaporn ◽  
P. Narongroeknawin ◽  
N. Kasitanon
Keyword(s):  
Disease Duration ◽  
Immunosuppressive Agents ◽  
Clinical Manifestations ◽  
Systemic Autoimmune Disease ◽  
Immunoglobulin G4 ◽  
Serum Igg4 Level ◽  
Related Disease ◽  
Igg4 Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

Background:Immunoglobulin G4 related disease (IgG4-RD) is an uncommon chronic systemic autoimmune disease, pathologically characterized by lymphoplasma cell, IgG4 plasma cell or storiform fibrosis infiltration with elevated serum IgG4 level. IgG4-RD is a new disease and not widely recognized.Objectives:The aim of this study was to describe clinical manifestations and outcomes of IgG4-RD in Thai patientsMethods:This multicenter retrospective cohort study included patients who aged ≥ 18 years and were diagnosed with IgG4-RD according to 2011 comprehensive or consensus diagnostic criteria, between 2000 and 2019 in four academic centers in Thailand. Baseline characteristic, laboratory and pathologic findings, treatments, and outcomes were systematically reviewed.Results:Of the 110 patients included, 71% were male with mean age (SD) of 59.6 (13.3) years and median disease duration (IQR) of 28.8 (14.6-53.5) months. Single organ involvement was observed in 60 patients (54.5%). The most common presenting organ involvement was the orbit (29%), followed by the salivary glands (19%), lacrimal glands (18%), bile duct (16%), and pancreas (11%). The most frequently affected organs were the orbits (34%), followed by the salivary glands (26%), lacrimal glands (20%), bile duct (19%), and lymph nodes (19%). Ninety-six percent (96%) had IgG4 level of more than 135 mg/dl at presentation. Most patients (92%) were treated with corticosteroid (CS) alone or in combination with immunosuppressive agents. Azathioprine (47%) and methotrexate (11%) were the most commonly used immunosuppressive agents. Additionally, 20% required surgery, and 6.4% underwent stent insertion. One-fourth (26%) were in remission with successfully CS tapering, while 37%, and 29% had complete, and partial response. Nevertheless, 22% relapse with median time to relapse (IQR) of 22.2 (12.8-41.1) months. Relapse was common in patients with orbital (p = 0.001) and lung (p= 0.007) involvement, and patients with longer disease duration (median 44.1 and 23.1 months, P=0.001), while serum IgG4 level was insignificantly higher in relapse group (median 1,085 vs. 850 mg/dL, p=0.28).Conclusion:IgG4-RD is a chronic systemic autoimmune disease with diverse manifestations, response to treatment, and outcomes. Most patients responded well to CS and immunosuppressive agents with notable relapse rate, while minority required surgery or mechanical intervention.References:[1]Wallace ZS, Zhang Y, Perugino CA, Naden R, Choi HK, Stone JH. Clinical phenotypes of IgG4-related Disease: an analysis of two international cross-sectional cohorts. Ann Rheum Dis. 2019;78(3):406-12.[2]Martinez-Valle F, Fernandez-Codina A, Pinal-Fernandez I, Orozco-Galvez O, Vilardell-Tarres M. IgG4- related disease: Evidence from six recent cohorts. Autoimmun Rev. 2017;16(2):168-72Acknowledgments:NoneDisclosure of Interests:None declared

scholarly journals Solitary Encapsulated Neurofibroma Not Associated with Neurofibromatosis-1 Affecting Tongue in a 73-Year-Old Female

2016 ◽  
Vol 2016 ◽  
pp. 1-4 ◽  
Author(s):  
Sk. Abdul Mahmud ◽  
Neha Shah ◽  
Moumita Chattaraj ◽  
Swagata Gayen
Keyword(s):  
Family History ◽  
Clinical Manifestations ◽  
Surgical Excision ◽  
Neurofibromatosis Type ◽  
Clinical Findings ◽  
Benign Tumors ◽  
History Of

Neurofibromas are benign tumors of nerve cell origin arising due to proliferation of Schwann cells and fibroblasts. They are usually asymptomatic and hence remain undiagnosed. They are commonly found on the skin and intraorally tongue is the most common site for their occurrence. Here, we present a unique case of solitary encapsulated neurofibroma in the oral cavity without any clinical manifestations or family history of Neurofibromatosis type 1 in a 73-year-old female patient who presented with a painless swelling on the tongue. The histopathologic findings closely mimicked benign fibrous histiocytoma. In our case, definitive diagnosis of neurofibroma was made based on clinical findings, family history, and histopathological and immunohistochemical evaluation. Through this case report we want to emphasize the role of biopsy and immunohistochemistry in arriving at a confirmatory diagnosis. The patient was treated by surgical excision and showed no signs of recurrence over a follow-up period of 12 months.

P-748 Diode laser hysteroscopic metroplasty for dysmorphic uterus: a pilot study

2021 ◽  
Vol 36 (Supplement_1) ◽  
Author(s):  
A Bilgory ◽  
E Shalom - Paz ◽  
Y Atzmon ◽  
N Aslih ◽  
D Estrada ◽  
...  
Keyword(s):  
Pilot Study ◽  
Diode Laser ◽  
Medical Center ◽  
Uterine Cavity ◽  
Reproductive Outcomes ◽  
Excessive Bleeding ◽  
Nulliparous Women ◽  
Tertiary Center ◽  
Adverse Pregnancy

Abstract Study question Whether diode laser hysteroscopic metroplasty for dysmorphic uterus is a safe and efficacious procedure and its effect on reproductive outcomes. Summary answer Diode laser hysteroscopic metroplasty is a safe and effective procedure for infertile women with dysmorphic uterus with comparable results to those reported in the literature. What is known already A T-shaped uterine anomaly is categorized by the ESHRE/ESGE consensus as dysmorphic uterus class U1a, characterized by an abnormal hypoplastic uterine cavity. A Y-shaped uterus is a dysmorphic uterus with a fundal subseptum. Dysmorphic uteri are associated with infertility, recurrent implantation failure (RIF), recurrent pregnancy loss (RPL), and adverse pregnancy outcomes. According to several studies, it seems that hysteroscopic metroplasty may improve the chances of conception and live birth. Previous studies described the procedure using bipolar systems, monopolar needle or scissors. The purpose is to achieve a uterine cavity of normal shape and volume by cutting the thickened lateral walls. Study design, size, duration This was a retrospective pilot study with a prospective follow-up. We retrospectively evaluated all cases operated between February 2018 to February 2020, at Hillel Yaffe Medical Center, Hadera, Israel. Reproductive outcomes for women who underwent the procedure were followed until September 2020. Pregnancies that were ongoing on September 2020 were followed until January 31st 2021. Participants/materials, setting, methods Nulliparous women with a diagnosis of infertility or RPL, who were diagnosed with dysmorphic uterus by three-dimensional ultrasound (3D-US) and underwent diode laser hysteroscopic metroplasty were included. All the metroplasties were done in one tertiary center by the same specialist. Reproductive outcomes were evaluated retrospectively and prospectively for a total follow-up time of 32 months. Reproductive performances before and after metroplasty were compared where possible. Main results and the role of chance Twenty-five women underwent diode laser hysteroscopic metroplasty for dysmorphic uterus in our institute. No perforations, excessive bleeding, or other complications were encountered during the procedures. Follow-up hysteroscopy and 3D-US were satisfactory in all cases 2 months after the metroplasty. A total of 15 nulliparous women returned to fertility treatments afterwards, among whom 9 conceived (60% pregnancy rate). Their infertility period before the procedure was 56.6 ± 36.1 months. The duration between the metroplasty to pregnancy was 5.2 ± 3.5 months. The rate of deliveries and ongoing pregnancies (pregnancies beyond 20 weeks of gestation) was 78% (7/9), with five successful liveborn deliveries and two ongoing pregnancies. All deliveries were between 36-37 weeks. The 10 women who were not treated by our infertility unit were contacted, among whom 6 discontinued their attempt to conceive. The other 4 conceived; three of them spontaneously. Among those 4 women,the rate of deliveries and ongoing pregnancies was 75%, with one term delivery and two ongoing pregnancies. Limitations, reasons for caution First, we included both T-shaped and Y-shaped uteri as both represent close versions of dysmorphic uteri, but in fact they differ. The subseptum might interfere with reproduction in a different mechanism. Second, the small and heterogeneous sample as well as the short duration of follow-up limit the conclusions. Wider implications of the findings We present the first application of diode laser in hysteroscopic metroplasty for dysmorphic uteri. This technique seems promising and our results are comparable with other series using different cutting devices. Only larger controlled trials with a longer follow-up can confirm the safety, efficacy, and impact on reproductive outcomes. Trial registration number Not Applicable

Contact Burns of the Hand

PEDIATRICS ◽  
1987 ◽  
Vol 80 (1) ◽  
pp. 18-21
Author(s):  
Richard Shugerman ◽  
Fred Rivara ◽  
Ruth Ann Parish ◽  
David Heimbach
Keyword(s):  
Inpatient Treatment ◽  
Visual Inspection ◽  
Medical Center ◽  
Surgical Excision ◽  
Area Burned ◽  
Major Burn ◽  
Acute Wound ◽  
Inpatient Basis ◽  
Burn Patient

Contact burns of the hand in children present difficult management questions. Because visual inspection of the acute wound often fails to distinguish major burns requiring inpatient treatment from minor burns amenable to outpatient therapy, we sought to identify characteristics of patients that would aid in decision making at the time the burn patient is seen. During the 5-year period, 1980 to 1984, 32 children less than 14 years of age were admitted to our medical center with contact burns of the hand. Patients were divided into two groups: those with major burns requiring ≥ seven days of hospitalization (n = 16) and those with minor burns requiring < seven days of hospitalization (n = 16). Compared with patients in the minor burn group, patients in the major burn group were hospitalized longer (16.9 ν 2.8 days), were more likely to require surgical excision and grafting (63% ν 0%), and had more extensive follow-up (5.3 ν 2.5 visits). There were no significant differences between the two groups with regard to percentage of area burned, age, sex, primary admission ν referral, and cause of burn. These data support the recommendation that all such burns be managed initially on an inpatient basis.

Abstract 12071: Serum Immunoglobulin G4 Level is an Independent Predictor of Major Adverse Cardiovascular Events After Percutaneous Coronary Intervention over Long-Term Follow-Up

Circulation ◽  
2015 ◽  
Vol 132 (suppl_3) ◽  
Author(s):  
Aiko Sakamoto ◽  
Nobukazu Ishizaka ◽  
Jiro Ando ◽  
Ryozo Nagai ◽  
Issei Komuro
Keyword(s):  
Coronary Intervention ◽  
Serum Levels ◽  
Major Adverse Cardiovascular Events ◽  
Immunoglobulin G4 ◽  
Serum Igg4 Level ◽  
Percutaneous Coronary ◽  
Serum Igg4 ◽  
Long Term Follow Up ◽  
Igg4 Level

Background: Inflammatory processes contribute to the development of atherosclerosis. However, it remains unclear whether immunoglobulin G4 (IgG4)-related immuno-inflammation may affect the progression of cardiovascular disease. We therefore analyzed the relationship between serum IgG4 level and future major adverse cardiovascular events (MACE) after percutaneous coronary intervention (PCI). Methods: We measured serum levels of IgG4 in 562 patients (mean age, 66.9 ± 10.3 years) who underwent coronary angiography from October 2005 to September 2008. Furthermore, a total of 108 patients who subsequently received first PCI were followed-up for a median period of 2721 days (interquartile range [IR] 1591-3183). MACE was defined as cardiac death, acute coronary syndrome, cerebral stroke, and target vessel revascularization. Results: Serum levels of IgG4 were significantly higher in patients with coronary artery disease (34.1 mg/dL, IR 18.7-58.9) than in those without (30.5 mg/dL, IR 15.8-50.3, P=0.045). Among patients who received PCI, 40 (37.0%) patients developed MACE during the follow-up period. Patients with serum IgG4 levels of median or greater (≥37.2 mg/dL) had a significantly higher risk of MACE than those with serum IgG4 levels below median (log-rank test, P=0.017). In Cox regression analysis using age, gender, estimated glomerular filtration rate (eGFR), hypertension, dyslipidemia, diabetes, smoking, and serum levels of high-sensitivity C-reactive protein (hsCRP) as covariates, serum IgG4 level was an independent predictor of MACE (hazard ratio 2.39 per 100 mg/dL increase, 95% confidence interval 1.41-4.07, P=0.001). Conclusions: Elevated serum IgG4 level at baseline significantly predicted MACE after PCI, as well as present coronary stenosis, over long-term follow-up even among patients without apparent IgG4-related disease. IgG4-related immuno-inflammation may play a certain role in the progression of coronary atherosclerosis.

scholarly journals Immunoglobulin G4 Sclerosing Cholangitis: An Unusual Cause of Obstructive Jaundice—Case Report and Literature Review

2018 ◽  
Vol 2018 ◽  
pp. 1-5
Author(s):  
Pragya Shrestha ◽  
Brian Le ◽  
Brent Wagner ◽  
William Pompella ◽  
Paras Karmacharya
Keyword(s):  
Sclerosing Cholangitis ◽  
Plasma Cells ◽  
Systemic Disease ◽  
Clinical Manifestations ◽  
Disease Process ◽  
High Serum ◽  
Diagnostic Challenge ◽  
Obliterative Phlebitis ◽  
Immunoglobulin G4 ◽  
Serum Igg4

IgG4-related sclerosing cholangitis (IgG4-SC) is one of the most common extra-pancreatic manifestation of IgG4-related disease (IgG4-RD) and is clinically distinct from primary sclerosing cholangitis (PSC). IgG4-RD is an increasingly recognized immune-mediated fibroinflammatory systemic disease, mostly affecting middle-aged and older male populations that can affect multiple organs. The presence of extra-biliary clinical manifestations of IgG4-RD, such as parotid and lacrimal swelling, lymphadenopathy, autoimmune pancreatitis, and retroperitoneal fibrosis, if present could provide important clues to diagnosis. High serum IgG4 levels, characteristic radiological (e.g., sausage-shaped pancreas or periaortitis) or biopsy findings (high percentage of IgG4+ plasma cells, lymphoplasmacytic infiltrate, storiform fibrosis, or obliterative phlebitis) in the setting of these features is diagnostic of this disease process. However, isolated IgG4-SC might be a diagnostic challenge, and the distinction is important as management of this disorder is vastly different from other causes of cholangitis such as PSC. Systemic corticosteroid therapy is the mainstay of therapy.

Laparoscopic Ovarian Transposition Before Pelvic Irradiation: A Saudi Tertiary Center Experience

2010 ◽  
Vol 20 (6) ◽  
pp. 1082-1086 ◽  
Author(s):  
Ismail A. Al-Badawi ◽  
Murad Al-Aker ◽  
Jamal AlSubhi ◽  
Hany Salem ◽  
Alaa Abduljabbar ◽  
...  
Keyword(s):  
Ovarian Function ◽  
Medical Center ◽  
Gynecologic Oncology ◽  
Follicle Stimulating Hormone ◽  
Premenopausal Women ◽  
Pelvic Irradiation ◽  
Ovarian Transposition ◽  
Tertiary Center ◽  
Stimulating Hormone

Objective:To report the authors' experience with laparoscopic ovarian transposition and ovarian function preservation in women who require pelvic irradiation as part of their cancer therapy.Design:Cohort study.Setting:The gynecologic oncology service in a referral tertiary/quaternary medical center in Saudi Arabia.Patients:Twenty-three premenopausal patients treated with radiotherapy for a pelvic malignancy.Interventions:Laparoscopic ovarian transposition to paracolic gutters with uterine conservation.Main Outcome Measures:Preservation of ovarian function assessed by patients' symptoms and serum follicle-stimulating hormone level.Results:Bilateral laparoscopic ovarian transposition was performed in 23 patients: 15 with cervical cancer, 4 with rectal cancer, 3 with Ewing sarcoma, and 1 with Hodgkin lymphoma. No immediate intraoperative or postoperative complications were observed. Three patients were lost to follow-up. Ovarian preservation was achieved in 13 (65%) of 20 patients. Seven patients with low follicle-stimulating hormone levels had regular uterine bleeding at follow-up.Conclusions:Laparoscopic ovarian transposition is a safe and effective procedure for preserving ovarian function. This procedure should be considered in all premenopausal women who need to undergo pelvic irradiation as part of their cancer treatment.

scholarly journals Over 10-Year Outcomes of Infantile-Onset Epilepsies

2021 ◽  
Vol 10 (3) ◽  
pp. 430
Author(s):  
Hyun-Jin Kim ◽  
Han Na Jang ◽  
Hyunji Ahn ◽  
Mi-Sun Yum ◽  
Tae-Sung Ko
Keyword(s):  
Medical Records ◽  
Medical Center ◽  
Neurological Deficits ◽  
Seizure Freedom ◽  
Neuropsychiatric Complications ◽  
Intractable Seizures ◽  
Tertiary Center ◽  
Long Term Follow Up ◽  
Asan Medical

Seizures in infancy have highly variable courses and underlying etiologies. However, there are only a few long-term follow-up studies regarding infantile-onset epilepsy. Therefore, we aimed to describe the clinical courses, seizure outcomes, and risk factors of infantile-onset epilepsy followed up for more than 10 years in a tertiary center. Methods: Data of the patients with epilepsy, diagnosed under the age of 12 months and followed up for more than 10 years, were retrieved from the electronic medical records of Asan Medical Center Children’s Hospital. The patients’ medical records were retrospectively reviewed, and clinical outcomes were assessed based on the duration of seizure freedom at the last follow-up. Results: Of the 146 patients, 103 (70.5%) entered at least one remission, of whom epilepsy was resolved in 46 (31.5%). Forty-nine (33.6%) were found to be intractable at last contact. Delayed development, neurological deficits, and later onset (>3 months) were significantly associated with intractable epilepsies (p < 0.01). Conclusions: This study demonstrated that many patients with infantile-onset epilepsy can experience seizure remission. However, in some cases, early onset epilepsy was highly associated with various comorbidities and intractable seizures. Therefore, appropriate diagnosis and treatment are necessary to prevent further neuropsychiatric complications.

scholarly journals Clinical Characteristics and Therapeutic Response of Immunoglobulin G4-Related Disease: a Retrospective Study of 127 Chinese Patients

2021 ◽  
Author(s):  
Wen An ◽  
Zhen Wu ◽  
Min Li ◽  
Haitian Yu ◽  
Xinyan Zhao ◽  
...  
Keyword(s):  
Risk Factors ◽  
Medical Therapy ◽  
Therapeutic Response ◽  
Classification Criteria ◽  
Chinese Patients ◽  
Immunoglobulin G4 ◽  
Related Disease ◽  
Serum Igg4 ◽  
Igg4 Level

Abstract Background & Aims: Immunoglobulin G4-related disease (IgG4-RD) is a multisystem fibroinflammatory condition. The aim of the present study was to characterize the clinical features and therapeutic response of patients with IgG4-RD and identify risk factors for disease relapse.Methods: We collected baseline data of eligible patients with IgG4-RD and analyzed clinical feature by interview and review of medical records. The patients who received medical therapy with at least 3 months follow-up were used to characterize the therapeutic response and identify risk factors for relapse. Result: Totally 127 IgG4-RD patients, including 92 males and 35 females, were included in the present study. The median age at diagnosis was 63.0 years, ranging from 23 to 86. The pancreas, bile duct and lymph nodes were the most frequently involved organs. The serum IgG4 level was elevated in 94.5% of the patients and was correlated with the number of organs involved. Patients classified as head and neck limited group were more likely to be female. Patients classified as pancreato-hepatobiliary group had highest aminotransferase, alkaline phosphatase (ALP), gamma-glutamyl transpeptidase (GGT), bilirubin level (all p＜0.05). Mikulicz syndrome and systemic involvement group had the highest IgG-RI score, serum globulin, IgG and IgG4 level. Among 92 patients who received medical therapy with at least 3 months follow-up,76 received glucocorticoids (GC) alone or in combination with immunosuppressive agents and 16 patients did not take GC. 74 out of the 76 patients (97.3%) achieved remission, with 59 of them remained in remission and 15 of them relapsed. Wheras16 patients did not take GC, among them, 6 patients achieved remission with one patient relapsed. On multivariate analysis, higher initial score of ACR/EULAR IgG4-RD Classification Criteria, GC monotherapy and GC withdrawal were independent predictors for relapse.Conclusion: Different phenotypes of IgG4-RD showed different demographic and serological features. The risk factors of relapse were GC withdrawal, GC monotherapy and higher score of ACR/EULAR IgG4-RD Classification Criteria.

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