scholarly journals Pure extra-thoracic sarcoidosis: about 24 cases

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Donia Chebbi ◽  
Sameh Marzouk ◽  
Mouna Snoussi ◽  
Nessrine Regaieg ◽  
Nawrez Gouiaa ◽  
...  

Abstract Introduction: Pure extra-thoracic sarcoidosis is rare and poorly characterized in the literature. Herein we present features of pure extra-thoracic sarcoidosis, in comparison with sarcoidosis with intra-thoracic involvement, in a population from the south of Tunisia. Methods: Retrospective, descriptive and comparative study of the patients presenting systemic sarcoidosis and attending the Department of Internal Medicine at the Hedi Chaker University Hospital, Sfax, Tunisia, for 21 years, from January1996 to December2016. Results: Twenty-four patients presented pure extra-thoracic sarcoidosis (30% of the cases of systemic sarcoidosis). There was a female predominance (79%). The mean age was 50 years. The main features were polyadenopathies (10 patients:41,7%), cutaneous involvement (10 patients:41,6%), inflammatory polyarthralgia (8 cases:33.3%), general symptoms (6 patients:25%), uveitis (6cases:25%), cholestasis (3cases) and kidney involvement (2cases). Lymphopenia, hypercalcemia, and hypercalciuria were observed each one in 12.5% of the cases, and high angiotensin converting enzyme (ACE) level in 38,5% of the cases. The histological proof was required in all the patients. Statistically significant associations identified were mainly hepatic involvement with general symptoms (p=0.035), peripheral lymphadenopathies (p=0.035) and kidney damage (p=0.022), and cutaneous involvement with articular manifestations (p=0.032). Systemic corticosteroids were used in half of the cases, with a good outcome in 21 cases, and recurrence in two cases. One patient was lost to follow up. The comparison to the form with mediastino-pulmonary involvement showed less lymphadenopathies(p=0.001), less lymphopenia (p=0.006), and less frequent use of corticosteroids(p=0.044). Conclusion: Our series was characterized by the frequency of the pure extra-thoracic form and the diversity of the systemic manifestations.

Author(s):  
Tabitha Heller ◽  
Christof Kloos ◽  
Nicolle Mueller ◽  
Josef Roemelt ◽  
Christian Keinki ◽  
...  

AbstractBackgroundComplementary and alternative medicine (CAM) is used often by patients with different diseases. While some authors subsume religiousness and spirituality to CAM, others do not. The objective of the present study was to assess the prevalence and types of CAM usage as well as the participants' spirituality/religiousness in an outpatient department for endocrinology and metabolic diseases.MethodsAll individuals visiting the outpatient department at a German university hospital from April to June 2009 were offered a standardized questionnaire on the use of dietary supplements and alternative therapies as well as their religiousness/spirituality. Demographic and clinical data of 428 respondents were taken from the electronic health record.ResultsOf the respondents, 16.4% (n = 66) classified themselves to be religious/spiritual and 67.9% (n = 273) as not religious/spiritual. Women were more religious/spiritual than men (p = 0.02). 41.4% of the respondents used supplements and 27.4% additional therapies. The use of supplements and additional therapies was more frequent in people with higher religiousness/spirituality (p = 0.005 and p = 0.01,resp.) but there were no associations between religiousness/spirituality and the number of consultations, costs for drugs, appraisal of the physicians treatment methods, the perceived effectiveness of prescribed drugs, fear of late complications or of side effects.ConclusionsA higher religiousness/spirituality is associated with a more frequent use of supplements or additional therapies in individuals with endocrinopathies or metabolic diseases. As CAM has been shown to be associated with worse outcome, addressing religiousness/spirituality which stresses the responsibility of the person for his life might offer an additional resource and should be further studied.


2020 ◽  
Vol 79 (Suppl 1) ◽  
pp. 1595.1-1595
Author(s):  
F. M. Ortiz Sanjuan ◽  
C. Pávez Perales ◽  
E. Vicens Bernabeu ◽  
C. Alcañiz Escandell ◽  
I. Cánovas Olmos ◽  
...  

Background:Interstitial lung disease (ILD) is a frequent complication of systemic sclerosis (SSc) and is often progressive and has a poor prognosis. A restrictive ventilatory defect could suggest ILD either alone or in combination with pulmonary arterial hypertension.Nowadays, Early-SSc is well defined as preliminary stage of SSc. Patients who meet criteria for Early-SSc could benefit from an early diagnosis of pulmonary involvement.Objectives:Our aim was to assess the pulmonary function in patients diagnosed of Early SSc.Methods:Retrospective observational study of a wide and unselected series of patients diagnosed as Early-SSc from a single university hospital from 2012 to 2019. Patients were classified as Early-SSc following Le Roy criteria. Despite this, patients already did not meet 2013 ACR/EULAR classification criteria for SSc. We reviewed pulmonary function through conventional spirometry and diffusing capacity of lung for carbon monoxide (DLCO).Results:We included 56 patients with a mean age of 52.3±12.1 years (96.4% women; 3.6% men).At the diagnosis of Early-SSc, no one of our patients evidenced a restrictive ventilatory pattern. DLCO was below normal limits in 18 patients (32.1%). Small airway obstruction expressed according decreased maximal (mid-) expiratory flow (MMEF) 25-75 was present in 24 patients (42.8%).After a mean follow-up period of 38.3±2.4 months, 29 (51.8%) patients fulfilled 2013 ACR/EULAR criteria. The average time between diagnosis of Early-SSc and achieve SSc classification was 24.4±1.8 months. The remaining 27 patients continued classified as Early-SSc.An analysis of the subgroup of patients which progressed to SSc showed that DLCO was decreased in 15 of those 29 patients (51.7%) and 18 of 29 patients (62.1%) presented decreased MMEF 25-75. Comparing with the subgroup of patients which not progressed to SSc were significant differences (Decreased DLCO: 51.7% vs 11.1%; p=0.02 and decreased MMEF 25-75: 42.8% vs 22.2%; p=0.05).The analysis of pulmonary function of the subgroup of patients continued classified as Early-SSc after follow-up period did not show significative changes after follow-up.Conclusion:In our study, a third of the patients classified as Early-SSc presented at diagnosis abnormal values of DLCO and/or signs of small airway obstruction without the presence of a restrictive ventilatory pattern. Moreover, this pulmonary disfunction was significantly more frequent in patients who progressed to definitive SSc. Patients which remains classified as Early-SSc did not experience significative changes.Our results support the concept that pulmonary function was impaired in Early-SSc and that I should probably be considered for future Early-SSc classification criteria.Disclosure of Interests:None declared


Author(s):  
Rosana Carvalho Silva ◽  
Maria Augusta Montenegro ◽  
Carlos A.M. Guerreiro ◽  
Marilisa M. Guerreiro

ABSTRACT:Rationale:Clobazam has been used successfully in adults and children with partial epilepsy. The purpose of this study was to evaluate the safety and efficacy of clobazam as add-on therapy in children with epileptic encephalopathy.Methods:This was a retrospective study conducted at the pediatric epilepsy clinic of our university hospital. Children less than 18-years of age with epileptic encephalopathy were included in the study. Clobazam was introduced as add-on therapy, starting with 5 mg/Kg/day and increased in minimally effective doses, up to the maximum tolerated dose. Data were obtained from clinical files and follow-up visits.Results:Ninety-seven patients were included in the study (39 girls), aged between 1 and 17-years-old (mean = 9.9). Twenty-six patients had Lennox-Gastaut syndrome, seven had myoclonic astatic epilepsy, nine had West syndrome and, in 57 patients, the type of epileptic encephalopathy could not be determined. Clobazam dosage ranged from 5 to 60 mg/day (mean = 37.5 mg/day). Forty (41%) patients presented with adverse events, most of which were mild and transitory, and clobazam needed to be withdrawn in only 11 patients. Nine (9.2%) patients were seizure-free after clobazam adjunctive therapy. In 11 (11.3%) patients seizure improvement was >75%, in 16 (16.5%) it was >50%, in 17 (17.5%) improvement was <50% and in 44 (45.5%) there was no change in seizure frequency. Three patients were lost to follow-up. In 85% of the patients with seizure improvement, the results lasted for more than one year.Conclusion:Clobazam is safe and effective in the treatment of epileptic encephalopathies of childhood.


2020 ◽  
Vol 31 (4) ◽  
pp. 513-518
Author(s):  
Antti I Lehtomäki ◽  
Riikka M Nevalainen ◽  
Vesa J Toikkanen ◽  
Emilia S Pohja ◽  
Jaakko J Nieminen ◽  
...  

Abstract OBJECTIVES Patients with pleural infections frequently have several comorbidities and inferior long-term survival. We hypothesized that these patients represent a vulnerable cohort with high rates of hospitalization and frequent use of healthcare services. This study aims to ascertain the need for and causes of treatment episodes after pleural infections during long-term follow-up. METHODS Patients treated for pleural infections at Tampere University Hospital between January 2000 and December 2008 (n = 191, 81% males, median age 58 years) were included and compared to a demographically matched population-based random sample of 1910 controls. Seventy percent of the pleural infections were caused by pneumonias and 80% of the patients underwent surgery. Information regarding later in-hospital periods and emergency room and out-patient clinic visits, as well as survival data, was obtained from national registries and compared between patients and controls. RESULTS Patients treated for pleural infections had significantly higher rates of hospitalizations (8.19 vs 2.19), in-hospital days (88.5 vs 26.6), emergency room admissions (3.18 vs 1.45), out-patient clinic visits (41.1 vs 11.8) and procedures performed (1.26 vs 0.55) per 100 patient-months when compared to controls during 5-year follow-up, in addition to having increased mortality (30% vs 11%), P-value &lt;0.00001 each. Particularly, episodes due to respiratory and digestive diseases, malignancies and mental disorders were more frequent. The patients’ comorbidities, such as alcoholism or chronic pulmonary disease, were associated with more frequent use of healthcare services. CONCLUSIONS Patients treated for pleural infections have high rates of hospitalizations, emergency room admissions and out-patient clinic visits during follow-up.


2020 ◽  
Vol 13 (1) ◽  
pp. e227379
Author(s):  
Gustavo Ferrer ◽  
Alwiya Omar Saleh ◽  
Henry D Tazelaar ◽  
Andrea V Arrossi

Neurofibromatosis type 1 (NF1) is an autosomal dominant disorder with multiple systemic manifestations. Pulmonary involvement has been reported in the form of interstitial fibrosis, emphysema, pulmonary hypertension and thoracic neoplasm. We report a case of desquamative interstitial pneumonia in a non-smoker with NF1.


Author(s):  
Belén Atienza-Mateo ◽  
José Luis Martín-Varillas ◽  
Rosalía Demetrio-Pablo ◽  
Vanesa Calvo-Río ◽  
Raúl Fernández Ramón ◽  
...  

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Kirsten Davidse ◽  
Anne-Loes Staa ◽  
Karlijn Pellikaan ◽  
Wanda Geilvoet ◽  
Judith Eck ◽  
...  

Abstract Introduction Transition from paediatric to adult endocrinology is a challenge for adolescents, their families and their healthcare professionals. Previous studies show that up to 25% of young adults with endocrine disorders are lost to follow-up once they move out of paediatric care. This poses a health risk for young adults, as lack of medical treatment and surveillance can have both psycho-social and physical consequences. Apart from absenteeism from school or work, this can lead to serious and expensive medical complications like Addison crisis. Methods In 2019 we studied electronic medical records of 387 patients who were over 15 years old when they attended the paediatric endocrine outpatient clinic (OPC) of our medical centre in 2013-2014. We collected data from medical charts, the hospital digital agenda and medical correspondence. Results Of 387 adolescents, 161 (42%) did not need adult endocrine follow-up because paediatric endocrine care was only puberty- or growth-related. Forty-six patients did not enter regular transition because they 1) participated in a pilot to improve transition (N=10), 2) had intellectual disability (ID) and transferred to ID care (N=28), or 3) died (N=8, mostly cancer-related). Hundred-and-eighty patients entered regular transition: 49 (27%) to a regional hospital and 131 (73%) within our university hospital. Of these 131 patients, 33 (25%) were lost to follow up; in 24 of them (73%), the invitation for the adult OPC had never been sent. Loss to follow up occurred when three subsequent critical steps failed: 1) the adult endocrinologist had not received or read the paediatrician’s referral letter and/or had not invited the patient; 2) the paediatrician had not checked whether the appointment was really made and received by the patient and 3) the patients and/or caregivers had not alarmed the hospital when no invitation for an appointment was received. Conclusion We found a 25% dropout during transfer from paediatric to adult tertiary endocrine care. Starting the transition process early and in a structured manner, as well as assigning a transition coordinator, can prevent part of the dropouts. However, 73% of all dropouts appeared to be attributable to failure of practical, logistic steps. In order to prevent this part of the dropouts, we provide practical recommendations for all three parties involved: 1) the adult endocrinologist should carefully read paediatricians’ letters and check whether action is required (i.e. check whether an appointment is requested) 2) the paediatrician should ascertain whether the appointment is really made and received by the patient 3) the patients and/or caregivers should be instructed to alarm the hospital when they do not receive the appointment. These actions require relatively little effort and may prevent the part of drop-outs that is caused by logistic failures.


Respiration ◽  
2021 ◽  
pp. 1-7
Author(s):  
Keisuke Watanabe ◽  
Nobuyuki Horita ◽  
Yu Hara ◽  
Nobuaki Kobayashi ◽  
Takeshi Kaneko

<b><i>Backgrounds:</i></b> Recent studies have reported increased risks of adverse events from systemic corticosteroids even with only low-dose or short-term use. Some patients with asthma experience complications requiring systemic corticosteroids. However, few studies have examined issues associated with administration of systemic corticosteroids for reasons other than asthma among subjects with asthma. <b><i>Objectives:</i></b> We investigated patterns of systemic corticosteroid exposure for reasons other than asthma in subjects with asthma. <b><i>Method:</i></b> We retrospectively reviewed the records of adult subjects with asthma followed up for &#x3e;1 year at Yokohama City University Hospital from January 1, 2010, to December 31, 2019. We investigated patterns and reasons for systemic corticosteroid use during follow-up. In addition, factors related to systemic corticosteroid use for reasons likely other than asthma were investigated. <b><i>Results:</i></b> Among the 568 subjects with asthma analyzed, 326 (57.4%) had received systemic corticosteroids for some reason. Among those 326 patients, 120 (36.8%) had received systemic corticosteroids for reasons likely other than asthma. Multivariable analysis revealed rheumatoid arthritis, eosinophilic granulomatosis with polyangiitis, other collagen vascular diseases, chronic rhinosinusitis, and malignancy as positively associated with systemic corticosteroid exposure for reasons likely other than asthma in subjects with asthma. <b><i>Conclusions:</i></b> About 40% of systemic corticosteroid use in subjects with asthma was for reasons likely other than asthma. Clinicians should be aware of their asthma patients’ exposures to systemic corticosteroids for nonasthma reasons, to avoid missing adverse events or underestimating the severity of asthma, and to reduce systemic corticosteroid use.


2021 ◽  
Vol 8 (Supplement_1) ◽  
pp. S786-S786
Author(s):  
Zahid Talibi Alaoui ◽  
Mohammed Raiteb ◽  
Fatima Ihbibane ◽  
Nabila Soraa ◽  
noura Tassi

Abstract Background Tuberculosis is a health problem in Morocco, which is increasingly indicative of human immunodeficiency virus (HIV) infection. Objective To determine the epidemiological, clinical and paraclinical, therapeutic and evolutionary aspects of tuberculosis and HIV co-infection. Methods we report 135 cases co-infected with HIV and tuberculosis, collected by the infectious diseases department at the Mohammed VI University Hospital in Marrakech. This is a 12-year retrospective study (2007 to 2020) that involved all HIV-infected patients hospitalized for tuberculosis regardless of its location. Results The mean age of the patients was 40 years (17-73 years). A male predominance was noted in 69% of cases. In 74.6% of cases, tuberculosis was indicative of HIV infection. Nine patients were receiving antiretroviral (ARV) treatment at the time of the discovery of tuberculosis. There were 24% pulmonary tuberculosis, 25.3% extrapulmonary tuberculosis and 49% disseminated tuberculosis. Tuberculosis was confirmed in 31.7% of cases. At the time of tuberculosis diagnosis, the average CD4 count was 86 cells / mm. Quadruple therapy with isoniazid, rifampicin, pyrazinamide and ethambutol was started in 83% of patients. The average time to start ARVs was 7 weeks. All patients who received ARVs received a combination therapy comprising the combination of 2 nucleoside analogs and one non-nucleoside analog. At the end of our work, the evolution was favorable in 53% of cases, death occurred in 25% of cases, 18.6% of patients were lost to follow-up, two cases of failure and another of relapse. Immune restoration syndrome was noted in 8 cases. Drug toxicity was observed in 24.5% of patients, 73% of which was related to hepato-toxicity of antibacillary drugs. Conclusion Tuberculosis is the most common opportunistic infection in people with HIV. Despite the advent of highly active triple therapy, tuberculosis is still a major cause of death in HIV positive people. Disclosures All Authors: No reported disclosures


2020 ◽  
Vol 9 (3) ◽  
pp. 698
Author(s):  
Pauline Caillard ◽  
Cécile Vigneau ◽  
Jean-Michel Halimi ◽  
Marc Hazzan ◽  
Eric Thervet ◽  
...  

In patients presenting with anti-glomerular basement membrane (GBM) disease with advanced isolated kidney involvement, the benefit of intensive therapy remains controversial due to adverse events, particularly infection. We aim to describe the burden of severe infections (SI) (requiring hospitalization or intravenous antibiotics) and identify predictive factors of SI in a large cohort of patients with anti-GBM disease. Among the 201 patients (median [IQR] age, 53 [30–71] years) included, 74 had pulmonary involvement and 127 isolated glomerulonephritis. A total of 161 SI occurred in 116 patients during the first year after diagnosis. These infections occurred during the early stage of care (median [IQR] time, 13 [8–19] days after diagnosis) with mainly pulmonary (45%), catheter-associated bacteremia (22%) and urinary tract (21%) infections. In multivariable analysis, positive ANCA (HR [95% CI] 1.62 [1.07−2.44]; p = 0.02) and age at diagnosis (HR [95% CI] 1.10 [1.00–1.21]; p = 0.047) remained independently associated with SI. Age-adjusted severe infection during the first three months was associated with an increased three-year mortality rate (HR [95% CI] 3.13 [1.24–7.88]; p = 0.01). Thus, SI is a common early complication in anti-GBM disease, particularly in the elderly and those with positive anti-neutrophil cytoplasmic antibodies (ANCA). No significant association was observed between immunosuppressive strategy and occurrence of SI.


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