Malignant Pheochromocytomas/Paragangliomas and Ectopic Hormonal Secretion: A Case Series and Review of the Literature

Malignant pheochromocytomas (PCs) and paragangliomas (PGLs) are rare neuroendocrine neoplasms defined by the presence of distant metastases. There is currently a relatively paucity of data regarding the natural history of PCs/PGLs and the optimal approach to their treatment. We retrospectively analyzed the clinical, biochemical, imaging, genetic and histopathological characteristics of fourteen patients with metastatic PCs/PGLs diagnosed over 15 years, along with their response to treatment. Patients were followed-up for a median of six years (range: 1–14 years). Six patients had synchronous metastases and the remaining developed metastases after a median of four years (range 2–10 years). Genetic analysis of seven patients revealed that three harbored succinate dehydrogenase subunit B/D gene (SDHB/D) mutations. Hormonal hypersecretion occurred in 70% of patients; normetanephrine, either alone or with other concomitant hormones, was the most frequent secretory component. Patients were administered multiple first and subsequent treatments including surgery (n = 12), chemotherapy (n = 7), radionuclide therapy (n = 2) and radiopeptides (n = 5). Seven patients had stable disease, four had progressive disease and three died. Ectopic hormonal secretion is rare and commonly encountered in benign PCs. Ectopic secretion of interleukin-6 in one of our patients, prompted a literature review of ectopic hormonal secretion, particularly from metastatic PCs/PGLs. Only four cases of metastatic PC/PGLs with confirmed ectopic secretion of hormones or peptides have been described so far.

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Cyclic vomiting syndrome: a case series and review of the literature

Frontline Gastroenterology ◽

10.1136/flgastro-2016-100705 ◽

2016 ◽

Vol 9 (1) ◽

pp. 2-9 ◽

Cited By ~ 7

Author(s):

Jessica Shearer ◽

Pavit Luthra ◽

Alexander C Ford

Keyword(s):

Medical Records ◽

Tricyclic Antidepressants ◽

Case Series ◽

Response To Treatment ◽

Cyclic Vomiting Syndrome ◽

Review Of The Literature ◽

Duration Of Symptoms ◽

History Of ◽

Cannabis Smoking ◽

Cyclic Vomiting

ObjectiveCyclic vomiting syndrome (CVS) is under-recognised. Treatment is difficult as the pathophysiology is incompletely understood. We report our experience of treating patients with amitriptyline, and review the literature to summarise symptoms and associated features, epidemiology, potential pathophysiological mechanisms, differential diagnoses and treatment.DesignConsecutive adult patients with CVS were identified during a 5-year period from January 2010 until December 2015. Medical records were reviewed retrospectively, and age and sex of the patient, symptoms, associated features and response to treatment with amitriptyline were recorded.SettingA luminal gastroenterology clinic at a teaching hospital.ResultsSeventeen patients were identified (mean age 29.8 years, 13 (76.5%) female). Five had a history of cannabis use. Duration of symptoms prior to diagnosis ranged from 5 months to 15 years. Fourteen patients commenced amitriptyline, and in eight (57.1%) symptoms either ceased entirely or improved. Review of the literature suggested the prevalence of CVS was 0.5%. Symptoms are stereotypical, with acute episodes of nausea and vomiting, interspersed by periods when the patient is symptom-free. Proposed pathophysiologies include neuroendocrine dysfunction, mutations in mitochondrial DNA and re-intoxication effects from cannabis stored in fat tissues. Treatment during the acute phase is supportive, with rehydration, sedation and antiemetics. Prophylaxis to prevent future attacks with antihistamines, antimigraine drugs, antiepileptics and tricyclic antidepressants may be beneficial. Complete cessation of cannabis smoking should be advised.ConclusionsDiagnosis of CVS is often delayed in adults. Once identified, patients respond well to amitriptyline.

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Herpetic Whitlow of the Hand in Infants

Journal of Hand and Microsurgery ◽

10.1055/s-0040-1710199 ◽

2020 ◽

Author(s):

Mohammad M. Al-Qattan ◽

Nada G. AlQadri ◽

Ghada AlHayaza

Keyword(s):

Bacterial Infection ◽

Case Reports ◽

Case Series ◽

Individual Case ◽

Senior Author ◽

Secondary Bacterial Infection ◽

Clinical Appearance ◽

History Of ◽

Mode Of Transmission ◽

Time Of Presentation

Abstract Introduction Herpetic whitlows in infants are rare. Previous authors only reported individual case reports. We present a case series of six infants. Materials and Methods This is a retrospective study of six cases of herpetic whitlows in infants seen by the senior author (MMA) over the past 23 years (1995–2017 inclusive). The following data were collected: age, sex, digit involved in the hand, mode of transmission, time of presentation to the author, clinical appearance, presence of secondary bacterial infection, presence of other lesions outside the hand, method of diagnosis, treatment, and outcome. Results All six infants initially presented with classic multiple vesicles of the digital pulp. In all cases, there was a history of active herpes labialis in the mother. Incision and drainage or deroofing of the vesicles (for diagnostic purposes) resulted in secondary bacterial infection. Conclusion The current report is the first series in the literature on herpetic whitlows in infants. We stress on the mode of transmission (from the mother) and establishing the diagnosis clinically. In these cases, no need for obtaining viral cultures or polymerase chain reaction; and no medications are required. Once the vesicles are disrupted, secondary bacterial infection is frequent and a combination of oral acyclovir and intravenous antibiotics will be required.

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Dental Implant Placement in Patients with a History of Medications Related to Osteonecrosis of the Jaws: A Systematic Review

Journal of Oral Implantology ◽

10.1563/aaid-joi-d-19-00351 ◽

2020 ◽

Author(s):

Judd Sher ◽

Kate Kirkham-Ali ◽

Denny Luo ◽

Catherine Miller ◽

Dileep Sharma

Keyword(s):

Systematic Review ◽

At Risk ◽

Drug Therapy ◽

Dental Implant ◽

Case Series ◽

Cochrane Central Register ◽

Bisphosphonate Treatment ◽

Implant Placement ◽

Increased Risk ◽

History Of

The present systematic review evaluates the safety of placing dental implants in patients with a history of antiresorptive or antiangiogenic drug therapy. The Preferred Reporting Items for Systematic Reviews and Meta-analyses (PRISMA) guidelines were followed. PubMed, Cochrane Central Register of Controlled Trials, Scopus, Web of Science, and OpenGrey databases were used to search for clinical studies (English only) to July 16, 2019. Study quality was assessed regarding randomization, allocation sequence concealment, blinding, incomplete outcome data, selective outcome reporting, and other biases using a modified Newcastle-Ottawa scale and the Joanna Briggs Institute critical appraisal checklist for case series. A broad search strategy resulted in the identification of 7542 studies. There were 28 studies reporting on bisphosphonates (5 cohort, 6 case control, and 17 case series) and one study reporting on denosumab (case series) that met the inclusion criteria and were included in the qualitative synthesis. The quality assessment revealed an overall moderate quality of evidence among the studies. Results demonstrated that patients with a history of bisphosphonate treatment for osteoporosis are not at increased risk of implant failure in terms of osseointegration. However, all patients with a history of bisphosphonate treatment, whether taken orally for osteoporosis or intravenously for malignancy, appear to be at risk of ‘implant surgery-triggered’ MRONJ. In contrast, the risk of MRONJ in patients treated with denosumab for osteoporosis was found to be negligible. In conclusion, general and specialist dentists should exercise caution when planning dental implant therapy in patients with a history of bisphosphonate and denosumab drug therapy. Importantly, all patients with a history of bisphosphonates are at risk of MRONJ, necessitating this to be included in the informed consent obtained prior to implant placement. The James Cook University College of Medicine and Dentistry Honours program and the Australian Dental Research Foundation Colin Cormie Grant were the primary sources of funding for this systematic review.

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Criteria positive and criteria negative anaphylaxis, with a focus on undifferentiated somatoform idiopathic anaphylaxis: A review and case series

Allergy and Asthma Proceedings ◽

10.2500/aap.2020.41.200076 ◽

2020 ◽

Vol 41 (6) ◽

pp. 436-441 ◽

Cited By ~ 1

Author(s):

Daniel A. Rosloff ◽

Kunal Patel ◽

Paul J. Feustel ◽

Jocelyn Celestin

Keyword(s):

Diagnostic Criteria ◽

Statistical Significance ◽

Case Series ◽

Poor Response ◽

Response To Treatment ◽

Fisher Exact Test ◽

Subjective Symptoms ◽

Significant Difference ◽

Physical Findings ◽

Idiopathic Anaphylaxis

Background: Undifferentiated somatoform (US) idiopathic anaphylaxis (IA) is considered a psychogenic disorder characterized by a lack of observable physical findings and poor response to treatment. Although failure to diagnose true anaphylaxis can have disastrous consequences, identification of US-IA is crucial to limit unnecessary expenses and use of health care resources. Objective: To better define the presentation and understand the potential relationship between US-IA and underlying psychiatric comorbidities. Methods: We retrospectively reviewed 110 visits by 107 patients to our institution for evaluation and management of anaphylaxis over a 1-year period. The patients were classified as having either criteria positive (CP) or criteria negative (CN) anaphylaxis based on whether they met Second National Institute of Allergy and Infectious Disease/Food Allergy and Anaphylaxis Network Symposium criteria for the clinical diagnosis of anaphylaxis. Patient characteristics, including objective and subjective signs and symptoms, and the presence of psychiatric diagnoses were collected and analyzed. Statistical significance was assessed by using the Fisher exact test. A literature review of US-IA and other psychogenic forms of anaphylaxis was performed. Results: Patients with CP anaphylaxis were more likely to present with hypotension, wheezing, urticaria, and vomiting than were patients with CN anaphylaxis. The patients with CN anaphylaxis were more likely to present with subjective symptoms of sensory throat tightness or swelling compared with patients with CP anaphylaxis. No significant difference was detected in the prevalence of psychiatric conditions between the two groups. Conclusion: Patients who met previously established diagnostic criteria for anaphylaxis were more likely to present with objective physical findings than those who did not meet criteria for true anaphylaxis. CN patients who presented for treatment of anaphylaxis were more likely to present with subjective symptoms. Formal diagnostic criteria should be used by clinicians when evaluating patients with suspected anaphylaxis.

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Clinical Characteristics of Patients Diagnosed with Strongyloidiasis in a United States Urban Outpatient Dermatology Department: A Case Series

SKIN The Journal of Cutaneous Medicine ◽

10.25251/skin.1.3.8 ◽

2017 ◽

Vol 1 (3) ◽

pp. 156-160

Author(s):

Jacqueline Watchmaker ◽

Sean Legler ◽

Dianne De Leon ◽

Vanessa Pascoe ◽

Robert Stavert

Keyword(s):

United States ◽

Case Series ◽

The United States ◽

Screening Tools ◽

Dermatology Department ◽

Cutaneous Manifestations ◽

Serologic Testing ◽

Patient Reported ◽

History Of ◽

Endemic Areas

Background: Although considered a tropical disease, strongyloidiasis may be encountered in non-endemic regions, primarily amongst immigrants and travelers from endemic areas. Chronic strongyloides infection may be under-detected owing to its non-specific cutaneous presentation and the low sensitivity of commonly used screening tools. Methods: 18 consecutive patients with serologic evidence of strongyloides infestation who presented to a single urban, academic dermatology clinic between September 2013 and October 2016 were retrospectively included. Patient age, sex, country of origin, strongyloides serology titer, absolute eosinophil count, presenting cutaneous manifestations, and patient reported subjective outcome of pruritus after treatment were obtained via chart review. Results: Of the 18 patients, all had non-specific pruritic dermatoses, 36% had documented eosinophila and none were originally from the United States. A majority reported subjective improvement in their symptoms after treatment. Conclusion: Strongyloides infection and serologic testing should be considered in patients living in non-endemic regions presenting with pruritic dermatoses and with a history of exposure to an endemic area.Key Points:Chronic strongyloidiasis can be encountered in non-endemic areas and clinical manifestations are variableEosinophilia was not a reliable indicator of chronic infection in this case series Dermatologists should consider serologic testing for strongyloidiasis in patients with a history of exposure and unexplained pruritus

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Other Biological Approaches to OCD

10.1093/oxfordhb/9780195376210.013.0068 ◽

2012 ◽

Cited By ~ 1

Author(s):

Nicole C. R. McLaughlin ◽

Benjamin D. Greenberg

Keyword(s):

Deep Brain Stimulation ◽

Brain Stimulation ◽

Response To Treatment ◽

History Of ◽

Key Issues ◽

Term Management ◽

Deep Brain

Interest in psychiatric neurosurgery has waxed and waned since the 1930s. This chapter reviews the history of these methods, with a focus on OCD. This review of lesion procedures and deep brain stimulation includes neuropsychological and neuroimaging research in the context of putative neurocircuitry underlying symptoms and response to treatment. The chapter highlights how an abundance of caution is needed, as well as key issues in long-term management of patients so treated.

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Mixed neuroendocrine–non-neuroendocrine neoplasms of the gallbladder: a case report

Surgical Case Reports ◽

10.1186/s40792-021-01152-4 ◽

2021 ◽

Vol 7 (1) ◽

Author(s):

Tatsuki Ishikawa ◽

Katsunori Nakano ◽

Masafumi Osaka ◽

Kenichi Aratani ◽

Kadotani Yayoi ◽

...

Keyword(s):

System Analysis ◽

Cancer Control ◽

Staging System ◽

Neuroendocrine Neoplasms ◽

Ki 67 ◽

Regional Lymph Nodes ◽

Abdominal Ultrasonography ◽

Case Presentation ◽

History Of ◽

Lymph Nodes Dissection

Abstract Background Primary neuroendocrine tumors of the gallbladder (GB-NETs) are rare, accounting for 0.5% of all NETs and 2.1% of all gallbladder cancers. Among GB-NETs, mixed neuroendocrine–non-neuroendocrine neoplasms of the gallbladder (GB-MiNENs) are extremely rare. Case presentation We present the case of a 66-year-old woman who was referred to us for the management of a gallbladder tumor (incidentally found during abdominal ultrasonography indicated for gallbladder stones). The patient had no history of abdominal pain or fever, and the findings on a physical examination were unremarkable. Blood tests showed normal levels of tumor markers. Imaging studies revealed a mass of approximately 10 mm in diameter (with no invasion of the gallbladder bed) located at the fundus of the gallbladder. A gallbladder cancer was suspected. Therefore, an open whole-layer cholecystectomy with regional lymph nodes dissection was performed. The postoperative course was uneventful, and she was discharged on postoperative day 6. Pathological findings showed GB-MiNENs with invasion of the subserosal layer and no lymph node invasion (classified T2aN0M0 pStage IIA according to the Union for International Cancer Control, 8th edition staging system). Analysis of the neuroendocrine markers revealed positive chromogranin A and synaptophysin, and a Ki-67 index above 95%. Fourteen months after the operation, a local recurrence was detected, and she was referred to another hospital for chemotherapy. Conclusions GB-MiNENs are extremely aggressive tumors despite their tumor size. Optimal therapy should be chosen for each patient.

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Post-Infectious Guillain–Barré Syndrome Related to SARS-CoV-2 Infection: A Systematic Review

Life ◽

10.3390/life11020167 ◽

2021 ◽

Vol 11 (2) ◽

pp. 167

Author(s):

Pasquale Sansone ◽

Luca Gregorio Giaccari ◽

Caterina Aurilio ◽

Francesco Coppolino ◽

Valentina Esposito ◽

...

Keyword(s):

Single Case ◽

Case Reports ◽

Case Series ◽

Guillain Barre Syndrome ◽

Response To Treatment ◽

Comprehensive Overview ◽

Fisher Syndrome ◽

Immunomodulating Therapy ◽

Guillain Barré Syndrome ◽

Guillain Barré

Background. Guillain-Barré syndrome (GBS) is the most common cause of flaccid paralysis, with about 100,000 people developing the disorder every year worldwide. Recently, the incidence of GBS has increased during the severe acute respiratory syndrome coronavirus-2 (SARS-CoV-2) epidemics. We reviewed the literature to give a comprehensive overview of the demographic characteristics, clinical features, diagnostic investigations, and outcome of SARS-CoV-2-related GBS patients. Methods. Embase, MEDLINE, Google Scholar, and Cochrane Central Trials Register were systematically searched on 24 September 2020 for studies reporting on GBS secondary to COVID-19. Results. We identified 63 articles; we included 32 studies in our review. A total of 41 GBS cases with a confirmed or probable COVID-19 infection were reported: 26 of them were single case reports and 6 case series. Published studies on SARS-CoV-2-related GBS typically report a classic sensorimotor type of GBS often with a demyelinating electrophysiological subtype. Miller Fisher syndrome was reported in a quarter of the cases. In 78.1% of the cases, the response to immunomodulating therapy is favourable. The disease course is frequently severe and about one-third of the patients with SARS-CoV-2-associated GBS requires mechanical ventilation and Intensive Care Unit (ICU) admission. Rarely the outcome is poor or even fatal (10.8% of the cases). Conclusion. Clinical presentation, course, response to treatment, and outcome are similar in SARS-CoV-2-associated GBS and GBS due to other triggers.

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Vocal Fold Injection Augmentation for Post-Airway Reconstruction Dysphonia: A Case Series

Annals of Otology Rhinology & Laryngology ◽

10.1177/00034894211012594 ◽

2021 ◽

pp. 000348942110125

Author(s):

Mathieu Bergeron ◽

John Paul Giliberto ◽

Meredith E. Tabangin ◽

Alessandro de Alarcon

Keyword(s):

Vocal Fold ◽

Care Center ◽

Case Series ◽

Retrospective Chart Review ◽

Pediatric Care ◽

Voice Perception ◽

Perceptual Evaluation ◽

Airway Reconstruction ◽

Vocal Fold Injection ◽

History Of

Objectives: Post airway reconstruction dysphonia (PARD) is common and has a significant effect on the quality of life of patients. Vocal fold injection augmentation (VFIA) is one treatment that can be used to improve glottic insufficiency in some patients. The goal of this study was to characterize the use and outcomes of VFIA for PARD. Methods: Retrospective chart review from January 2007 to July 2018 at a tertiary pediatric care center. Consecutive patients with PARD who underwent VFIA, who had a preoperative voice evaluation and a follow-up evaluation within 3 months after VFIA (fat, carboxymethylcellulose gel, hyaluronic acid). Results: Thirty-four patients (20 female) underwent VFIA. The mean age at the time of the injection was 13.6 years (SD 6.1). Twenty patients (58.8%) had a history of prematurity and a mean of 1.8 open airway surgeries. After injection, 29/34 patients (85.3%) noted a subjective voice improvement. The baseline Consensus Auditory-Perceptual Evaluation of Voice (CAPE-V) overall severity score decreased by a mean of 5.7 (SD = 19.6) points, P = .12. Total pediatric Voice Handicap Index (pVHI) improved by 6.0 (SD = 19.5) points, from 57.4 (SD = 20.0) to 51.4 (SD = 17.2), P = .09. Functional pVHI subscore demonstrated a significant improvement, with a decrease of 3.4 (SD = 7.3) points, P = .02. All procedures were performed as an overnight observation and no complication occurred. Conclusion: Patients with PARD represent a complex subset of patients. VFIA is a straightforward intervention that may improve voice perception. Many patients reported subjective improvement despite minimal objective measurement. Further work is warranted to elucidate the role of injection in management of PARD

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Non-Coding RNAs as Biomarkers of Tumor Progression and Metastatic Spread in Epithelial Ovarian Cancer

Cancers ◽

10.3390/cancers13081839 ◽

2021 ◽

Vol 13 (8) ◽

pp. 1839

Author(s):

Karolina Seborova ◽

Radka Vaclavikova ◽

Lukas Rob ◽

Pavel Soucek ◽

Pavel Vodicka

Keyword(s):

Ovarian Cancer ◽

Tumor Progression ◽

Expression Profiles ◽

Gene Expression Profiles ◽

Distant Metastases ◽

Regulatory Elements ◽

Metastatic Spread ◽

Response To Treatment ◽

Primary Tumors ◽

Non Coding Rnas

Ovarian cancer is one of the most common causes of death among gynecological malignancies. Molecular changes occurring in the primary tumor lead to metastatic spread into the peritoneum and the formation of distant metastases. Identification of these changes helps to reveal the nature of metastases development and decipher early biomarkers of prognosis and disease progression. Comparing differences in gene expression profiles between primary tumors and metastases, together with disclosing their epigenetic regulation, provides interesting associations with progression and metastasizing. Regulatory elements from the non-coding RNA families such as microRNAs and long non-coding RNAs seem to participate in these processes and represent potential molecular biomarkers of patient prognosis. Progress in therapy individualization and its proper targeting also rely upon a better understanding of interactions among the above-listed factors. This review aims to summarize currently available findings of microRNAs and long non-coding RNAs linked with tumor progression and metastatic process in ovarian cancer. These biomolecules provide promising tools for monitoring the patient’s response to treatment, and further they serve as potential therapeutic targets of this deadly disease.

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