scholarly journals Clinical Presentation and Treatment Outcomes of Children and Adolescents With Pheochromocytoma and Paraganglioma in a Single Center in Korea

2021 ◽  
Vol 11 ◽  
Author(s):  
Hyojung Park ◽  
Min-Sun Kim ◽  
Jiwon Lee ◽  
Jung-Han Kim ◽  
Byong Chang Jeong ◽  
...  

PurposePheochromocytoma (PCC) and paraganglioma (PGL) (PPGL) are rare neuroendocrine tumors, and data on managing these conditions in children and adolescents are lacking. The objective of this study was to demonstrate the clinical presentation and treatment outcomes in children and adolescents with PPGL in a single tertiary care center in Korea.MethodsThis retrospective study included 23 patients diagnosed with PCC (n = 14) and PGL (n = 9) before the age of 21 at Samsung Medical Center (from June 1994 to June 2019). We describe age, gender, family history, clinical characteristics, laboratory findings, pathologic findings, therapeutic approaches, and treatment outcomes.ResultsOf the 23 patients, 14 had PCC and nine had PGL. The median age at diagnosis was 16.8 years (range, 6.8–20.8 years). The common presenting symptoms were hypertension (n = 10), headache (n = 9), palpitation (n = 4), and sweating (n = 4). The plasma or 24-hour urine catecholamine and/or metabolite concentrations were markedly elevated in 22 patients with PPGL, but were normal in one patient with carotid body PGL. All tumors were visualized on computed tomography. Genetic tests were performed in 15 patients, and seven patients showed mutations in RET (n = 3), SDHB (n = 3), and VHL (n = 1). All patients underwent surgery, and complete excision was performed successfully. Three patients with metastasis underwent postoperative adjuvant therapy.ConclusionThis study suggests that pediatric PPGL tends to be extra-adrenal and bilateral and shows a higher potential for genetic mutations. Considering the hereditary predisposition of pediatric PPGL, genetic screening tests are strongly recommended, and lifelong follow-up is needed to detect recurrence and metastasis. Further research with a larger sample size and routine genetic screening is needed to better understand the genetic conditions and long-term prognosis of PPGL.

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Henry P. Gottsch ◽  
Richard E. Berger ◽  
Claire C. Yang

Objective. The goal of this study is to describe comorbid characteristics in patients who have priapism, and their treatment outcomes.Methods. Chart review was undertaken on men who had a diagnosis of priapism from a tertiary medical center, from 2000–2010. Men with priapism due exclusively to the use of prescription erectile aids and medications were not included in the review.Results. We identified 79 patients with the priapism. The most common type of priapism was the low flow variant. High flow priapism was identified in 2 patients. The most common general comorbid condition associated with priapism was mental illness (including substance abuse), which was present in 56% of the patients. Neurogenic priapism accounted for 19% of the total priapism events. Psychopharmaceutical agents and recreational drugs were commonly associated with ischemic priapism. Acute complications of priapism treatment were not common, but long-term complications, especially erectile dysfunction, were frequent.Conclusions. We describe the characteristics and outcomes of a large group of patients with priapism. Our experience at a tertiary care center indicates that mental illness, including substance abuse disorders, is a highly prevalent comorbid condition in men who experience priapism. Consistent with previous reports, erectile dysfunction is the most common complication from priapism and its treatment, occurring in the majority of men.


2008 ◽  
Vol 2008 ◽  
pp. 1-5 ◽  
Author(s):  
Mirna H. Farhat ◽  
Ali I. Shamseddine ◽  
Kassem A. Barada

Introduction. Small bowel cancers are rare. Accumulation of data regarding their clinical presentation, pathologic features, prognostic factors, treatment modalities, and outcome is difficult.Methods. This is a retrospective study of the medical records of 33 patients with small bowel cancers treated at the American University of Beirut-Medical Center over a 20-year period.Results. The study included 25 males (76%) and 8 females (24%). Median age at presentation was 56 years. Most common symptoms were abdominal pain (66.7%) and weight loss (57.6%). Thirteen patients presented with abdominal emergencies (39.3%). Lymphoma was the most common malignant tumor (36.4%), followed by adenocarcinoma (33.3%), leiomyosarcoma (15.2%), gastrointestinal stromal tumors (12.1%), and neuroendocrine tumors (3.0%). Tumors were located in the duodenum in 30% of patients, jejunum in 33%, and ileum in 36%. Resectability rate was 72.7% and curative R0 resection was achieved in 54.1% (13/24) of patients. 5-year survival of the 33 patients was 24.2%.Conclusion. Small bowel cancers are difficult to diagnose because of the nonspecific symptoms. Most patients present with advanced disease and have poor prognosis. Adenocarcinoma and duodenal location have the worst 5-year survival in contrast to stromal tumors and those with ileal location which have the best survival.


Author(s):  
Akane Takamatsu ◽  
Hitoshi Honda ◽  
Tomoya Kojima ◽  
Kengo Murata ◽  
Hilary Babcock

Abstract Objective The COVID-19 vaccine may hold the key to ending the pandemic, but vaccine hesitancy is hindering the vaccination of healthcare personnel (HCP). Design Before-after trial Participants and setting Healthcare personnel at a 790-bed tertiary care center in Tokyo, Japan. Interventions A pre-vaccination questionnaire was administered to HCP to examine their perceptions of the COVID-19 vaccine. Then, a multifaceted intervention involving (1) distribution of informational leaflets to all HCP, (2) hospital-wide announcements encouraging vaccination, (3) a mandatory lecture, (4) an educational session about the vaccine for pregnant or breastfeeding HCP, and (5) allergy testing for HCP at risk of allergic reactions to the vaccine was implemented. A post-vaccination survey was also performed. Results Of 1,575 HCP eligible for enrollment, 1,224 (77.7%) responded to the questionnaire, 43.5% (n =533) expressed willingness to be vaccinated, 48.4% (n = 593) were uncertain, and 8.0% (n=98) expressed unwillingness to be vaccinated. The latter two groups were concerned about the vaccine’s safety rather than its efficacy. Post-intervention, the overall vaccination rate reached 89.7% (1,413/1,575), with 88.9% (614/691) of the pre-vaccination survey respondents who answered “unwilling” or “unsure” eventually receiving a vaccination. In the post-vaccination questionnaire, factors contributing to increased COVID-19 vaccination included information and endorsement of vaccination at the medical center (26.4%; 274/1,037). Conclusions The present, multifaceted intervention increased COVID-19 vaccinations among HCP at a Japanese hospital. Frequent support and provision of information were crucial for increasing the vaccination rate and may be applicable to the general population as well.


Author(s):  
Josee Paradis ◽  
Agnieszka Dzioba ◽  
Hamdy El-Hakim ◽  
Paul Hong ◽  
Frederick K. Kozak ◽  
...  

Abstract Background To evaluate the clinical presentation of choanal atresia (CA) in tertiary centers across Canada. Methods Multi-centre case series involving six tertiary care pediatric hospitals across Canada. Retrospective chart review of patients born between 1980 and 2010 diagnosed with CA at a participating center. Results The health charts of 215 patients (59.6% female) with CA were reviewed and included in this study. The mean age of patients at time of CA presentation was 0.4 months (range 0.1 to 7.2 months) for bilateral CA and 37.8 months (range 0.1 to 164.1 months) for unilateral cases. The most common presenting symptoms for bilateral CA in decreasing order were respiratory distress (96.4%), feeding difficulties (68.2%), and rhinorrhea (65.5%), and for unilateral cases in decreasing order were rhinorrhea (92.0%), feeding difficulties (24.7%), and respiratory distress (18.0%). For the majority of patients (73.2%), the obstruction comprised mixed bony and membranous tissue, with only 10.5% presenting with a purely membranous obstruction. Familial history of CA was confirmed in only 3.3% of cases. One half of patients with CA presented with one or more associated anomalies and 30.6% had a syndrome. Conclusions The present investigation is the first national multi-institutional study evaluating the clinical presentation of CA over three decades. The present cohort of CA patients presented with a breadth of co-morbidities with highly variable presentations, with bilateral cases being more severely affected than unilateral cases. Further investigation into hereditary linkages to CA development is warranted. Graphical abstract


Author(s):  
Rattanavalai Nitiyarom ◽  
Sirirat Charuvanij ◽  
Surachai Likasitwattanakul ◽  
Chaiwat Thanoophunchai ◽  
Wanee Wisuthsarewong

Background: Juvenile dermatomyositis is a rare condition, but it is the most common idiopathic inflammatory myopathy in pediatric patients. Aim: To study the clinical manifestations, investigations, treatment, clinical course, and outcomes of juvenile dermatomyositis in Thai children. Method: This retrospective study included juvenile dermatomyositis patients treated at Siriraj Hospital, a 2,300-bed national tertiary referral center in Bangkok, Thailand, from 1994 to 2019. Results: Thirty patients (22 females and 8 males) were included with a female to male ratio of 2.7:1. Median age at diagnosis was 5.1 years (range, 2.6-14.8 years). Median duration of illness before diagnosis was 6.5 months (range, 0.3-84.0 months). Acute and subacute onset occurred in the majority of patients. Presenting symptoms included muscle weakness in 27/30 (90%), skin rash in 26/30 (86.7%), muscle pain in 17/26 (65.4%), and arthralgia in 4/18 (22.2%) of patients. Dermatologic examination revealed Gottron’s rash, heliotrope rash, and periungual telangiectasia in 25/30 (83.3%), 21/30 (70.0%), and 15/24 (62.5%) of patients, respectively. Interestingly, scalp dermatitis was found in 8/21 (38.1%) of patients. The most commonly used treatment regimen in this series was a combination of prednisolone and methotrexate. During the median follow-up of 3.1 years (range, 0.0-18.5 years), only one-third of patients were seen to have monocyclic disease. Extraskeletal osteosarcoma at a previous lesion of calcinosis cutis was observed in one patient at 12 years after juvenile dermatomyositis onset. Limitations: This was a retrospective single-center study, and our results may not be generalizable to other healthcare settings. Prospective multicenter studies are needed to confirm the findings of this study. Conclusion: juvenile dermatomyositis usually poses a diagnostic and therapeutic challenge, which can be compounded by the ethnic variations in the clinical presentation, as observed in this study. Asian patients tend to present with acute or subacute onset of disease, and arthralgia and/or arthritis are less common than in Caucasian patients. Scalp dermatitis is not uncommon in pediatric juvenile dermatomyositis patients. An association between juvenile dermatomyositis and malignancy, though rare, can occur.


Author(s):  
Bhagirath Singh ◽  
Indira Subhadarshini Paul

<p class="abstract"><strong>Background:</strong> Pediatric dermatoses require a separate view from adult dermatoses as there are important differences in clinical presentation, treatment and prognosis. There is very little epidemiological study available on non-infectious childhood dermatoses in India. The aims of the study were to find the prevalence, clinical profile and various etiological factors associated with childhood non-infectious dermatoses and to determine the prevalence of most common non-infectious childhood dermatoses.</p><p class="abstract"><strong>Methods:</strong> This cross-sectional observational study conducted at tertiary care centre in Rajasthan, India. Children with age 13 years and below with clinical evidence of cutaneous disorders were studied. Parents who have not given consent for the study, acutely ill children, Children having infectious dermatoses (bacterial, fungal, viral, arthropods, parasitic and protozoal infection) were excluded from the study.<strong></strong></p><p class="abstract"><strong>Results:</strong> A total of 232 cases were studied which showed a female preponderance of 55.60%. Most of the skin diseases were seen in the 5 to 13 years (school children) age group. The most common dermatoses was eczematous (36.63%), among eczema atopic diathesis was the commonest (17;20%) followed by hypersensitivity dermatoses (21.12%), genetic disorders (13.36%), disorders of genetic disorders (7.4%), sweat gland disorders (8.18%), pigmentary disorders (4.31%), papulo squamous disorders (4.74%), Nevi (6.46%).</p><p class="abstract"><strong>Conclusions:</strong> Eczematous dermatoses were the most commonly noted in the study, followed by hypersensitivity dermatoses. Atopic diathesis was the commonest endogenous eczemas. Acne, insect bite reaction and miliaria were the other common dermatoses. There was no significant association of various dermatoses with systemic diseases in the study.</p>


2020 ◽  
Vol 99 (9) ◽  
pp. 569-576 ◽  
Author(s):  
Andrea Lovato ◽  
Cosimo de Filippis

Aim: Pharyngodynia, nasal congestion, rhinorrhea, smell, and taste dysfunctions could be the presenting symptoms of coronavirus disease 2019 (COVID-19) caused by severe acute respiratory syndrome coronavirus 2. The aim was to perform a systematic review of current evidences on clinical presentation of COVID-19, focusing on upper airway symptoms in order to help otolaryngologists identifying suspected cases. Methods: We searched PubMed and Web of Science electronic databases. Results: We included 5 retrospective clinical studies for a total of 1556 hospitalized patients with COVID-19, 57.5% were male and mean age was 49.1 years. Pooled data revealed that pharyngodynia was present in 12.4% of patients, nasal congestion in 3.7%, and rhinorrhea was rare. No reports on COVID-19 and olfactory/gustative disorders matched inclusion criteria but preliminary evidences suggested they could be present. Common symptoms were fever (85.6%), cough (68.7%), and fatigue (39.4%). Frequent comorbidities were hypertension (17.4%), diabetes (3.8%), and coronary heart disease (3.8%); 83% of patients had alterations on chest computed tomography that were bilateral in 89.5% of cases. Ground-glass opacity was the most common finding (50%). Lymphopenia (77.2%) and leucopenia (30.1%) were common. Critical cases with complications were 9%, intensive care unit admission was required in 7.3%, invasive ventilation in 3.4%, and mortality was 2.4%. Conclusion: Otolaryngologists should know that pharyngodynia, nasal congestion, olfactory, and gustative disorders could be the presenting symptoms of COVID-19. Clinical presentation together with radiological and laboratory findings could help to identify suspected cases.


2018 ◽  
Vol 5 (suppl_1) ◽  
pp. S63-S63
Author(s):  
Teppei Shimasaki ◽  
Yoona Rhee ◽  
Rachel D Yelin ◽  
Michelle Ariston ◽  
Stefanie Ollison ◽  
...  

Abstract Background Clinical culture results are sometimes used to estimate the burden of multidrug-resistant organisms (MDROs) in hospitals. The association between positive clinical culture results and prevalence of MDROs in the gut is incompletely understood. Methods Rectal swab or stool samples were collected daily from adult medical intensive care unit (MICU) patients and cultured for target MDROs using selective media between January 2017 and January 2018 at Rush University Medical Center, a 676-bed tertiary-care center in Chicago. Resistance mechanisms were confirmed by phenotypic methods and/or polymerase chain reaction. Clinical culture results during MICU stay were extracted from the hospital information system. Target MDROs included vancomycin-resistant Enterococci (VRE), carbapenem-resistant Enterobacteriaceae (CRE), extended-spectrum β-lactamase-producing Enterobacteriaceae (ESBL), carbapenem-resistant Pseudomonas aeruginosa (CRPA) and carbapenem-resistant Acinetobacter baumannii (CRAB). Patients with either a study or clinical culture positive for a target MDRO were analyzed. Results We collected 5,086 study samples from 1,661 unique admissions (1,419 patients) and included here data from 413 unique admissions (397 patients) with completed microbiologic analysis. Median (IQR) patient age was 65 (51–75) years and length of MICU stay was 3 (3–4) days. A total of 156 (37.8%) patients had a target MDRO detected from a study sample at any point; 57 (36.5%) patients had &gt;1 MDRO detected. Overall prevalence of these MDROs was found to be 22.5% VRE, 6.5% CRE, 19.8% ESBL, 4.4% CRPA, and 0.7% CRAB. New MDRO acquisition was observed in 58 (14.6%) patients (figure). Once a target MDRO was detected in a study sample, 82.2% of subsequent study samples were positive for that MDRO. Only 13 (5.8%) patients had a positive clinical culture for any target MDRO during their MICU stay (table). Conclusion Clinical cultures capture only the tip of the resistance iceberg and alone are insufficient to guide MDRO-targeted prevention strategies. Universal infection prevention measures are an alternative that may be preferred in settings where overall prevalence of MDROs is moderate or high and patients may be colonized with &gt;1 MDRO. Disclosures All authors: No reported disclosures.


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