Gastrointestinal System Involvement in Children suspected for COVID-19: A Single Tertiary Center Experience from Turkey

Abstract Background: Sarcoidosis is a systemic granulomatous disease of unknown etiology. Clinical cohort studies of different populations are important to understand the high variability in clinical presentation and disease course of sarcoidosis. The aim of the study is to evaluate clinical characteristics, including organ involvement, pulmonary function tests, and laboratory parameters, in a sarcoidosis cohort at the University of Minnesota. We compare the organ system involvement of this cohort with other available cohorts. Methods: We conducted a retrospective data collection and analysis of 187 subjects with biopsy-proven sarcoidosis seen at a tertiary center. Organ system involvement was determined using the WASOG sarcoidosis organ assessment instrument. Clinical phenotype groups were classified using the Genomic Research in Alpha-1 Antitrypsin Deficiency and Sarcoidosis criteria. Results: Mean subject age at diagnosis was 45.8 ± 12.4, with a higher proportion of males (55.1%), and a higher proportion of blacks (17.1%) compared to the racial distribution of Minnesota residents (5.95%). The majority (71.1%) of subjects required anti-inflammatory therapy for at least 1 month. Compared to the A Case Control Etiologic Study of Sarcoidosis cohort, there was a higher frequency of extra-thoracic lymph node (34.2% vs. 15.2%), eye (20.9% vs. 11.8%), liver (17.6% vs. 11.5%), spleen (20.9% vs. 6.7%), musculoskeletal (9.6% vs. 0.5%), and cardiac (10.7% vs. 2.3%) involvement in our cohort. A multisystem disease with at least five different organs involved was identified in 13.4% of subjects. A restrictive physiological pattern was observed in 21.6% of subjects, followed by an obstructive pattern in 17.3% and mixed obstructive and restrictive pattern in 2.2%. Almost half (49.2%) were Scadding stages II/III. Commonly employed disease activity markers, including soluble interleukin-2 receptor and angiotensin-converting enzyme, did not differ between treated and untreated groups. Conclusions: This cohort features a relatively high frequency of high-risk sarcoidosis phenotypes including cardiac and multiorgan disease. Commonly-utilized serum biomarkers do not identify subpopulations that require or do better with treatment. Findings from this study present further the high-variability nature of sarcoidosis and the need for a more reliable biomarker to predict and measure disease severity and outcomes for better clinical management for sarcoidosis patients.

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OUTCOME OF TREATING PEDIATRIC LANGERHANS HISTIOCYTOSIS WITH LCH III IN TERTIARY CENTER, SAUDI ARABIA

American Journal of Health, Medicine and Nursing Practice ◽

10.47672/ajhmn.667 ◽

2021 ◽

Vol 6 (1) ◽

pp. 38-50

Author(s):

Abrar Almohammadi ◽

Khalid Khalid ◽

Moussa Shehadah ◽

Abdullah Baothman

Keyword(s):

Saudi Arabia ◽

Langerhans Cell Histiocytosis ◽

Progressive Disease ◽

Langerhans Cell ◽

Single System ◽

Excellent Outcome ◽

System Involvement ◽

Tertiary Center ◽

Significant Difference ◽

Oncology Center

Background: Langerhans cell histiocytosis (LCH) is a rare disease affects any age and any organ; its presentations and outcome vary from self-healing lesions to life-threatening disseminated disease. This study evaluated the outcome of treating children diagnosed with Langerhans cell histiocytosis (LCH) at Oncology Center, Saudi Arabia. Methodology: Through a retrospective study design, the researchers reviewed the medical records and electronic files of all children (aged from 0-≤ 14 years) who had been diagnosed and treated for LCH at Princess Norah Oncology Center (PNOC), King Abdelaziz Medical City, Saudi Arabia, in the period from January 2000 to December 2019 (n=33). Findings: Males constituted (66.7%), with remarkable dominance of Saudis (93.9%). The median age at diagnosis was 28 months (IQR=49 months); (42.4%) were diagnosed before reaching their second birthday. Fourteen patients (42.4%) had multisystem (MS-LCH) involvement, of which 13 patients with risk organ (RO) (+) and one patient without risk organ (RO) (-). Most of the patients received LCH III protocols. Reactivation occurred in 11 patients (33.3%), and two deaths (6.1%) occurred in cases with MS (RO) (+) progressive disease. The overall survival was 93.9%; with no statistically significant difference in event free survival observed between patients with multisystem compared to single system involvement. Conclusion and recommendations: Excellent outcome of LCH is associated with single system involvement and worse outcome (reactivations, or morality) is determined by multi-organ involvement especially at younger age less than 24 months. Better understanding of pathophysiology and genetic molecular background could lead to a striking transformation to novel therapy that warrants a prospective clinical trial. A high mortality in patients with progressive disease demands an earlier aggressive salvage in such group. Prospective clinical trials are required for improved treatment strategies in these subgroups

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Interstitial Lung Disease in Systemic Sclerosis: A Single-Center Retrospective Analysis

Current Rheumatology Reviews ◽

10.2174/1573397117666210913104029 ◽

2021 ◽

Vol 17 ◽

Author(s):

Müçteba Enes Yayla ◽

Gülşah Balcı ◽

Murat Torgutalp ◽

Didem Şahin Eroğlu ◽

Ayşe Bahar Keleşoğlu Dinçer ◽

...

Keyword(s):

Computed Tomography ◽

Systemic Sclerosis ◽

Interstitial Lung Disease ◽

Lung Disease ◽

Interstitial Pneumonia ◽

Clinical Laboratory ◽

Chest Computed Tomography ◽

System Involvement ◽

Duration Of Disease ◽

Gastrointestinal System Involvement

Background: Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by microangiopathy, inflammation, fibrosis. Interstitial lung disease (ILD) is common among SSc patients. Objective: This study aims to define the clinical, laboratory and serologic characteristics of SSc patients with ILD and to present the frequency of chest computed tomography features. Methods: Two hundred twenty-six SSc patients who applied to the Rheumatology Department between January 2007 and August 2019 were retrospectively examined. A total of 100 SSc patients with ILD (44.2 %) were determined. Clinical, laboratory and serological features of SSc patients with and without ILD were compared. Results : Both groups had similar characteristics in terms of age and sex. The duration of disease (p=0.001) and follow-up time (p=0.001) were longer in SSc patients with ILD. Multivariable logistic regression analysis indicated that the duration of disease (OR: 1.06 [1.01-1.13], p=0.029), presence of gastrointestinal system involvement (OR: 3.29 [1.28-8.46], p=0.013) and anti-SCL70-positivity (OR: 6.04 [2.35-15.49], p<0.001) were associated with ILD. There was an inverse relationship between Anti-CENP-B positivity and the presence of ILD (p=0.001). The assessment regarding the chest computed tomography characteristics of interstitial pneumonia patterns were as follows: 82.5% non-specific interstitial pneumonia, 14.4% usual interstitial pneumonia and 2.1% desquamative interstitial pneumonia. The most frequent abnormal findings included ground glass opacification (88.7%), reticulation (64.9%), traction bronchiectasis (57.7%), septal thickening (52.6%) and honey combing (28.9%). Conclusion: We have shown that there is a relationship between anti-SCL70, disease duration, gastrointestinal system involvement and ILD in SSc patients.

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Ulcerative Lesions in Behcet's Disease

Ulcers ◽

10.1155/2012/146797 ◽

2012 ◽

Vol 2012 ◽

pp. 1-9

Author(s):

Ümit Türsen ◽

Belma Türsen

Keyword(s):

Behçet’S Disease ◽

Behcet’S Disease ◽

Behçet's Disease ◽

Definitive Diagnosis ◽

Necrotizing Vasculitis ◽

Behcet's Disease ◽

System Involvement ◽

Aphthous Ulcers ◽

Ulcerative Lesions ◽

Gastrointestinal System Involvement

Ulcerative lesions in Behcet’s disease (BD) are regarded as important manifestation for diagnosis. Various kinds of ulcerative lesions appear in patients with BD. They present as orogenital ulcers, necrotizing vasculitis and pyoderma gengrenosum. Gastrointestinal system involvement (Gis) in Behçet’s disease affects all areas from the esophagus to the anus. Most authors believe that the Gis manifestations of Behçet’s disease should be confined to aphthous ulcers, which can occur throughout the Gis tract. All patients with oro-genital and Gis ulcerations should be fully investigated to establish a definitive diagnosis and eliminate the possibility of an underlying BD.

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