scholarly journals DOP79 Primary hypogammaglobulinemia with IBD-like features: An ECCO CONFER Multicenter Case Series

2021 ◽  
Vol 15 (Supplement_1) ◽  
pp. S111-S111
Author(s):  
A Albshesh ◽  
P Eder ◽  
D G Ribaldone ◽  
B Oldenburg ◽  
N K De Boer ◽  
...  
Keyword(s):  
Case Reports ◽  
Case Series ◽  
Iga Deficiency ◽  
Clinical Feature ◽  
Aminosalicylic Acid ◽  
Igg Subclass Deficiency ◽  
Selective Iga Deficiency ◽  
Appropriate Treatment ◽  
One Year

Abstract Background The most commonly recognized clinical feature of hypogammaglobulinemia is recurrent infections with high prevalence of gastrointestinal manifestations. In some cases, clinical and endoscopic features are indistinguishable from those of inflammatory bowel disease (IBD). Methods This was a multicenter case series performed as a part of the Collaborative Network of Exceptionally Rare case reports (CONFER) project. Results This report includes 25 patients with primary hypogammaglobinemia and IBD-like features [20 males and 5 females, mean age 50 years (±21.7 SD)]. Crohn’s disease-like features were noted in 22 patients, three patients had ulcerative colitis-like features. The diagnosis of hypogammaglobulinemia preceded IBD-like features diagnosis in 20 patients (mean of 7.7 years prior, range 0.3–35 years), and followed IBD-like features appearance in 5 cases (mean of one year after, 0.4- 9.1 years). Hypogammaglobinemia etiologies were common variable immunodeficiency (72%), Agammaglobulinemia (8%), selective IgA-deficiency (8%), Goods syndrome (8%), IgG subclass deficiency with IgA deficiency (4%). In addition to antibiotics and intravenous immunoglobulin (IVIG) as a treatment for hypogammaglobinemia, fifteen patients received IBD treatment during the follow-up period, of whom two were on 5-aminosalicylic acid, five on corticosteroids, three on immunomodulatory, four on anti-tumor necrosis factor, and one on vedolizumab. By the end of the follow-up [35.5 months (Interquartile range 18–75)], 20 of 25 (80%) patients were in clinical remission. Conclusion This case series illustrates a strong male and CD-like features predilection. The diagnosis of IBD-like features mainly occurs after that of hypogammaglobulinemia, the majority of cases successfully recovered after appropriate treatment.

scholarly journals Pediatric Extraspinal Sacrococcygeal Ependymoma: Report of Two Cases and Literature Review

Diagnostics ◽  
2021 ◽  
Vol 11 (9) ◽  
pp. 1680
Author(s):  
Francesco Fabozzi ◽  
Silvia Ceccanti ◽  
Antonella Cacchione ◽  
Giovanna Stefania Colafati ◽  
Andrea Carai ◽  
...  
Keyword(s):  
Literature Review ◽  
Case Reports ◽  
Case Series ◽  
Surgical Excision ◽  
Central Canal ◽  
Ependymal Cells ◽  
The Past ◽  
First Case ◽  
One Year

Primary central nervous system (CNS) tumors represent the most common solid tumors in childhood. Ependymomas arise from ependymal cells lining the wall of ventricles or central canal of spinal cord and their occurrence outside the CNS is extremely rare, published in the literature as case reports or small case series. We present two cases of extra-CNS myxopapillary ependymomas treated at our institution in the past three years; both cases originate in the sacrococcygeal region and were initially misdiagnosed as epidermoid cyst and germ cell tumor, respectively. The first case, which arose in a 9-year-old girl, was treated with a surgical excision in two stages, due to the non-radical manner of the first operation; no recurrence was observed after two years of follow-up. The other case was a 12-year-old boy who was treated with a complete resection and showed no evidence of recurrence at one-year follow-up. In this paper, we report our experience in treating an extremely rare disease that lacks a standardized approach to diagnosis, treatment and follow-up; in addition, we perform a literature review of the past 35 years.

Mechanical thrombectomy in pediatric stroke: systematic review, individual patient data meta-analysis, and case series

2019 ◽  
Vol 24 (5) ◽  
pp. 558-571 ◽  
Author(s):  
Kartik Bhatia ◽  
Hans Kortman ◽  
Christopher Blair ◽  
Geoffrey Parker ◽  
David Brunacci ◽  
...  
Keyword(s):  
Systematic Review ◽  
Ischemic Stroke ◽  
Acute Ischemic Stroke ◽  
Mechanical Thrombectomy ◽  
Case Reports ◽  
Meta Analysis ◽  
Case Series ◽  
Neurological Outcomes

OBJECTIVEThe role of mechanical thrombectomy in pediatric acute ischemic stroke is uncertain, despite extensive evidence of benefit in adults. The existing literature consists of several recent small single-arm cohort studies, as well as multiple prior small case series and case reports. Published reports of pediatric cases have increased markedly since 2015, after the publication of the positive trials in adults. The recent AHA/ASA Scientific Statement on this issue was informed predominantly by pre-2015 case reports and identified several knowledge gaps, including how young a child may undergo thrombectomy. A repeat systematic review and meta-analysis is warranted to help guide therapeutic decisions and address gaps in knowledge.METHODSUsing PRISMA-IPD guidelines, the authors performed a systematic review of the literature from 1999 to April 2019 and individual patient data meta-analysis, with 2 independent reviewers. An additional series of 3 cases in adolescent males from one of the authors’ centers was also included. The primary outcomes were the rate of good long-term (mRS score 0–2 at final follow-up) and short-term (reduction in NIHSS score by ≥ 8 points or NIHSS score 0–1 at up to 24 hours post-thrombectomy) neurological outcomes following mechanical thrombectomy for acute ischemic stroke in patients < 18 years of age. The secondary outcome was the rate of successful angiographic recanalization (mTICI score 2b/3).RESULTSThe authors’ review yielded 113 cases of mechanical thrombectomy in 110 pediatric patients. Although complete follow-up data are not available for all patients, 87 of 96 (90.6%) had good long-term neurological outcomes (mRS score 0–2), 55 of 79 (69.6%) had good short-term neurological outcomes, and 86 of 98 (87.8%) had successful angiographic recanalization (mTICI score 2b/3). Death occurred in 2 patients and symptomatic intracranial hemorrhage in 1 patient. Sixteen published thrombectomy cases were identified in children < 5 years of age.CONCLUSIONSMechanical thrombectomy may be considered for acute ischemic stroke due to large vessel occlusion (ICA terminus, M1, basilar artery) in patients aged 1–18 years (Level C evidence; Class IIb recommendation). The existing evidence base is likely affected by selection and publication bias. A prospective multinational registry is recommended as the next investigative step.

Highly selective diversion with proactive leakage management after low anterior resection for rectal cancer

2021 ◽  
Author(s):  
K Talboom ◽  
I Vogel ◽  
R D Blok ◽  
S X Roodbeen ◽  
C Y Ponsioen ◽  
...  
Keyword(s):  
Low Anterior Resection ◽  
Case Series ◽  
Fecal Diversion ◽  
Single Center ◽  
Single Centre ◽  
Diverting Stoma ◽  
Faecal Diversion ◽  
One Year ◽  
Diversion Rate

Abstract In this single center case series with nine percent primary diversion, 86 of 94 patients alive and with complete follow-up at one year had a functioning anastomosis. Seventy-five of the initial 99 patients never had a stoma. Meaning: Highly selective fecal diversion in combination with proactive leakage management, low anastomoses can be preserved safely, and the majority of patients will be spared all disadvantages of a diverting stoma. In this single-centre case series, with a primary diversion rate of 9 per cent, 86 of 94 patients who were alive and had complete follow-up at 1 year had a functioning anastomosis. Seventy-five of the initial 99 patients never had a stoma. The results indicate that, with highly selective faecal diversion in combination with proactive leakage management, low anastomoses can be preserved safely, and the majority of patients will be spared the disadvantages of a diverting stoma.

scholarly journals Role of Radiotherapy in Recurrent Intra-Abdominal Yolk Sac Tumor

2021 ◽  
pp. 1010-1018
Author(s):  
Marhendra Satria Utama ◽  
Andi Kurniadi ◽  
A.A. Citra Yunda Prahastiwi ◽  
Antony A. Adibrata
Keyword(s):  
Case Reports ◽  
Yolk Sac ◽  
Yolk Sac Tumor ◽  
Appropriate Treatment ◽  
Curative Radiotherapy ◽  
Chief Complaints ◽  
Abdominal Mri ◽  
Malignant Germ Cell Tumor

Yolk sac tumor (YST) is a rare malignant germ cell tumor with no appropriate treatment strategy to date. However, patients are treated on a case-to-case basis as per various case reports that have been published. Here, we present a case of 27-year-old female patient who presented to us with chief complaints of severe abdominal pain associated with leucorrhea. She previously had a similar pain episode, which was then evaluated by a multidisciplinary team. She was diagnosed with YST. After that, she underwent 6 cycles of chemotherapy, but there was no improvement. Then the medical oncologist referred her to performed radiotherapy. Then, the radiation oncologist decided to give her curative radiotherapy of 3D-CRT. After completing her sessions, she felt better and clinically improving. After that, she was discharged and scheduled a follow-up visit for first evaluation. At her follow-up visit, she was feeling well, and we decided to have an abdominal MRI.

One-year Follow-Up Results with Hydrogel Implant in Therapy of Hallux Rigidus: Case Series with 44 Patients

2021 ◽  
Author(s):  
Christoph Zanzinger ◽  
Norbert Harrasser ◽  
Oliver Gottschalk ◽  
Patrick Dolp ◽  
Florian Hinterwimmer ◽  
...  
Keyword(s):  
Learning Algorithm ◽  
Case Series ◽  
Metatarsophalangeal Joint ◽  
Hallux Rigidus ◽  
Therapy Failure ◽  
Retrospective Case ◽  
Aofas Score ◽  
Medium Level ◽  
One Year

Abstract Background The Cartiva implant (CI) is being increasingly used in the surgical therapy of hallux rigidus. Despite a growing number of studies, numerous questions regarding patient selection remain unanswered. Patients and Methods As part of a retrospective case series with prospective follow-up (average follow-up period: 12 months), a total of 44 patients (male/female = 16/28; mean age at the time of surgery: 55.4 years) with 44 CI were analysed (VAS, EFAS-, AOFAS-score). Using a correlation analysis and a machine learning algorithm, risk factors for therapy failure were investigated. Results The overall survival rate of the CI was 93% at 12 months. The VAS, EFAS and AOFAS scores showed a significant improvement in comparison to the preoperative condition. The mobility of the metatarsophalangeal joint showed no increase. Patients with a medium osteoarthritis grade and a medium level of clinical restraint showed the greatest improvement in relation to their preoperative condition. Conclusion The CI can be seen as an effective therapy for hallux rigidus. Nonetheless, realistic patient expectations must be communicated.

Long-Term Follow-Up after Radiotherapy of Hidradenitis Suppurativa

Dermatology ◽  
2021 ◽  
pp. 1-7
Author(s):  
Jurr Boer
Keyword(s):  
Total Dose ◽  
Hidradenitis Suppurativa ◽  
Early Stage ◽  
Case Reports ◽  
Case Series ◽  
Postal Survey ◽  
Long Term Follow Up ◽  
Late Treatment

<b><i>Background:</i></b> Patients with hidradenitis suppurativa (HS) are still often disappointed with the current treatments offered and there is a clear demand for more effective options. Since the late 1990s there has been a revival in the use of radiotherapy (RT) for different benign diseases, including HS. During the past 20 years one case series and some scattered case reports have described promising results of RT. <b><i>Objectives:</i></b> To evaluate the long-term efficacy of RT in early-stage HS. <b><i>Methods:</i></b> A postal survey-based long-term follow-up with simple factual questions of partly retrospective and partly contemporary characteristics was performed. Sixty-four patients (96 axillae), diagnosed with mild to moderate HS were irradiated with a orthovoltage unit with 100 kV, 3 mm Al or 200 kV, 0.5 Cu filtering, respectively. Four to six biweekly fractional doses ranging from 0.75 to 1 Gy up to a total dose of 6 Gy in one series, and in chronic cases followed by four daily fractions of 2 Gy up to a total dose of 14 Gy, were given. Late treatment toxicity and the rate of remission of the disease were evaluated. <b><i>Results:</i></b> The overall response rate of the survey was 64.1% with 40.6% (26/64) valid, complete questionnaires. In total, 40 axillae were irradiated in these 26 patients. After a median follow-up of 40 years (range 32–52) complete remission of the lesions occurred in 34 of the 40 sites (85%). None of the 26 patients with 40 irradiated sites reported adverse effects at the time of the survey. <b><i>Conclusions:</i></b> RT appears to be an effective treatment for early and mild HS in the majority of patients. In this case series, no side effects were reported after a median follow-up period of 40 years.

Malignant thyroid teratoma: an integrated analysis of case series and case reports

2021 ◽  
Author(s):  
Huy Gia Vuong ◽  
Truong P.x. Nguyen ◽  
Hanh T.t. Ngo ◽  
Lewis Hassell ◽  
Kennichi Kakudo
Keyword(s):  
Clinical Course ◽  
Case Reports ◽  
Case Series ◽  
Seer Program ◽  
Integrated Analysis ◽  
Continuous Variables ◽  
Chi Square ◽  
Large Tumor ◽  
Exact Test

Malignant thyroid teratoma (MTT) is a very rare thyroid malignancy. These neoplasms have been reported only in case reports and small-sized case series so far. In this study, we searched for MTTs in the Surveillance, Epidemiology, and End Result (SEER) program during 1975-2016. Subsequently, we incorporated the SEER data with published MTT cases in the literature to analyze the characteristics and prognostic factors of MTTs. Integrated data were analyzed using Chi-square or Fisher’s exact test for categorical covariates, and t-test or Mann-Whitney test for continuous variables. We included 28 studies with 36 MTT cases and found additional 8 cases from the SEER program for final analyses. Our results showed that MTT is typically seen in adult females. These neoplasms were associated with an aggressive clinical course with high rates of extrathyroidal extension (80%) and nodal involvement (62%). During follow-up, the development of recurrence and metastases were common (42% and 46%, respectively), and one-third of patients died at the last follow-up. Large tumor size (p = 0.022) and the presence of metastases during follow-up (p = 0.008) were associated with a higher mortality rate. In conclusion, our study demonstrated the characteristic features of MTT patients and outlined some parameters associated with a negative outcome which could help clinicians better predict the clinical course of these neoplasms.

scholarly journals A rare case of metachronous neuroendocrine tumor after a colorectal adenocarcinoma: qualitative critical review of synchronous and metachronous gastrointestinal NET

2020 ◽  
Author(s):  
Francesco Lancellotti ◽  
Luigi Solinas ◽  
Davide Telesco ◽  
Andrea Sagnotta ◽  
Augusto Belardi ◽  
...  
Keyword(s):  
Neuroendocrine Tumor ◽  
Colorectal Adenocarcinoma ◽  
Retrospective Studies ◽  
Case Reports ◽  
English Literature ◽  
Case Series ◽  
Population Based ◽  
Review Articles ◽  
Gastrointestinal Neuroendocrine Tumor

Abstract Gastrointestinal neuroendocrine tumor (NET) associated with a metachronous intestinal adenocarcinoma is rare. We report the case of a 71-year-old man with an ileal NET. Patient has previously undergone a left colectomy for sigmoid cancer. We report a complete review both of the metachronous and synchronous NET. A comprehensive systematic literature search in PubMed, EMBASE, and MEDLINE identified a total of 35 relevant studies. This study includes an analysis of review articles, case reports, case series, retrospective studies and population-based studies. In the English literature to date, there are 21 case reports (19 synchronous cases and 2 metachronous cases), 3 case series and 3 review articles, and less than 10 retrospective studies or population-based studies. A total of 31 patients in 24 articles were included in the study: 28 patients with a synchronous gastrointestinal NET and colorectal adenocarcinoma and 3 patients with metachronous gastrointestinal NET and colorectal adenocarcinoma. The incidence of synchronous cancer (particularly for colorectal and gastric cancer) with a gastrointestinal NET ranges from 10 to 50%, while for the metachronous ones it is still unclear. This is the third metachronous case report and the first descriptive case of gastrointestinal NET diagnosed 2 years after a colorectal adenocarcinoma. An endoscopic follow-up program for gastrointestinal NET patients and/or for first-degree relatives of NET patients appears recommendable.

scholarly journals Autotransplantation of mature impacted tooth to a fresh molar socket using a 3D replica and guided bone regeneration: two years retrospective case series

2019 ◽  
Author(s):  
Ye Wu ◽  
Jiaming Chen ◽  
Fuping Xie ◽  
Huanhuan Liu ◽  
Gang Niu ◽  
...  
Keyword(s):  
Clinical Outcome ◽  
Bone Regeneration ◽  
Case Series ◽  
Guided Bone Regeneration ◽  
Third Molars ◽  
Retrospective Case ◽  
Impacted Tooth ◽  
Probing Depth ◽  
One Year

Abstract Background: The aim of this study was to evaluate the clinical outcome of autotransplantation of mature third molars to fresh molar extraction sockets using 3D replicas. Methods: Ten patients underwent teeth autotransplantation with or without GBR. We observed the mobility, percussion, radiography examination, the probing depth and the masticatory function of the transplanted teeth during two years following up, which were transplanted into fresh molar sockets by using 3D replicas, and GBR when it is necessary. Results: The average extra-oral time of donor tooth had been shortened to 1.65 min when used the 3D replica. Some probing depth of the transplanted tooth were deeper than 3 mm at 4 or 5 weeks temporarily. And one patient felt slight sensitive when chewing with soft food at 4 weeks, then disappeared. The clinical examination of the autotransplantation teeth during one year follow-up showed no sign of failure. Conclusions: The tooth autotransplantation using 3D replica with or without GBR is an effective method which can reduce the extra-oral time of the donor teeth and may result in less failure.

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