Gastrointestinal System Involvement Research Articles

Disseminated tuberculosis leading to surgical emergency: A case report

Tuberculosis remains one of the most fatal diseases in the world, but treatable on early diagnosis. Disseminated tuberculosis can involve several organs and clinically present with a wide variety of signs and symptoms. Early diagnosis and timely initiation of proper treatment is of great importance in preventing the later complications of the disease. We report a case with disseminated tuberculosis who exhibited a wide spectrum of extra-pulmonary involvement. Simultaneous involvement of pulmonary, skeletal and gastrointestinal system leading to a surgical emergency is uncommon and managed successfully. This paper highlights the diverse clinical presentation of disseminated tuberculosis and the importance of early diagnosis and treatment.

Adult-onset blue rubber bleb nevus syndrome with cutaneous, gastrointestinal, renal, and central nervous system involvement

Adult-onset blue rubber bleb nevus syndrome with cutaneous, gastrointestinal, renal, and central nervous system involvement

Clinical analysis of methylmalonic acidemia and hyperhomocysteinemia with diffuse lung disease as an initial or main presentation

Objective: To improve the awareness of methylmalonic acidemia and hyperhomocysteinemia with diffuse lung disease as an initial or main presentation. Methods: A retrospective analysis of the clinical manifestations, radiological features, laboratory tests, genetic variations, treatments and prognoses was conducted in six children presented with diffuse lung disease and finally diagnosed with methylmalonic acidemia and hyperhomocysteinemia in Ward 2 of Department of Respiratory Diseases, Beijing Children's Hospital, from August 2017 to November 2018. Results: Six children were included in this study. Two children were male and four were female. The average age of onset was 28 months. The mean age at diagnosis was 34 months. The average interval from onset to diagnosis was 6 months. Four children who underwent genetic tests were found to have variants of gene MMACHC and diagnosed with CblC type. All children had respiratory symptoms and signs as initial or main presentation, which were tachypnea (5 cases), exercise intolerance (5 cases), cough (4 cases), cyanosis (4 cases), clubbing (4 cases), dyspnea (3 cases) and retractions (3 cases). Pulmonary arterial hypertension was found in all six children. Pericardial effusion (4 cases), kidney involvement (3 cases), nervous system involvement (3 cases), gastrointestinal system involvement (3 cases) and anemia (2 cases) also coexisted. The high resolution computed tomography (HRCT) features included dilated pulmonary artery (6 cases), ground-glass opacities (4 cases), diffuse poorly defined ground-glass centrilobular nodules (3 cases), pleural effusion (3 cases), thickening of interlobular septum (2 cases), etc. All children had an elevated concentration of methylmalonic acid in urine and homocysteine in plasma. Genetic tests were performed in four patients, and MMACHC genetic mutations were found in all of them. Clinical manifestations, HRCT features and pulmonary arterial hypertension turned better in five children after treatment. One patient who was not regularly followed-up died. Conclusions: Pulmonary involvement including diffuse lung disease and pulmonary arterial hypertension could coexist with methylmalonic acidemia and hyperhomocysteinemia, which may have respiratory symptoms and signs as the initial or main presentation. Characteristic HRCT features were found in some patients. Plasma homocysteine test is a quick method for screening the disease in children with diffuse lung disease and (or) pulmonary arterial hypertension. Both diffuse lung disease and pulmonary arterial hypertension may turn better after treatment.

Noninfectious Causes of Fever in 128 Patients with Systemic Lupus Erythematosus

This study aimed to explore the clinical characteristics of noninfectious fever in patients with systemic lupus erythematosus (SLE) and the underlying causes through multivariate analysis. Clinical data of 128 patients with SLE who were admitted to Affiliated Hospital of Nanjing University of TCM, Nanjing 210029, P.R. China from January 2015 to December 2017 were retrospectively analyzed. The following factors were closely associated with fever: patient age, treatment history, SLEDAI score, enlarged liver, spleen and lymph nodes, low hemoglobin, leukopenia, CRP, complement C3, albumin, anti-dsDNA antibody, glucocorticoids, and cyclophosphamide. Multivariate logistic regression analysis showed that factors, such as enlarged liver, spleen and lymph nodes, digestive system involvement, low hemoglobin, leukopenia, CRP, decreased albumin, anti-dsDNA antibody, glucocorticoids and cyclophosphamide, were closely associated with noninfectious fever in SLE. Noninfectious fever is a common clinical manifestation of SLE. Factors, such as enlarged liver, spleen and lymph nodes, digestive system involvement, low hemoglobin, leukopenia, CRP, decreased albumin, anti-dsDNA antibody, glucocorticoids, and cyclophosphamide, were independent risk factors for noninfectious fever in SLE.

HENOCH-SCHONLEIN PURPURA IN CHILDREN: A CROSS SECTIONAL STUDY

Background: Henoch-Schonlein purpura (HSP) is a leukocytoclastic vasculitis with small vessel involvement and mainly affects the skin as well as joints, the gastrointestinal system (GIS), kidneys, and, more rarely, other organs. Objective: The objective of this study was to evaluate the sociodemographic characteristics, and clinical and laboratory findings of patients diagnosed with HSP. Materials and Methods: This was a retrospective study done to find out the sociodemographic data, clinical, laboratory findings, and treatment information of patients diagnosed with HSP and was admitted to the Pediatric Clinic of a tertiary care hospital between January 1, 2008, and August 31, 2013. The data were obtained from the hospital’s data processing system. HSP cases were validated according to EULAR/PRINTO/PRES criteria. Mean standard deviation, median, lowest and highest, frequency, and ratio values were used in the descriptive statistics of the data. Results: The study included 85 patients between the ages of 2 and 16 years, wherein 49 patients (57.6%) were male and 36 (42.4%) were female. The mean age was 9.9±3.3 years and 53 patients (62.4%) were under 10 years of age. The most common precipitating factor was upper respiratory tract infections. Purpura was the only symptom observed in all the patients and joint involvement was the second most common symptom (60%). GIS involvement was observed in 46 patients (58.8%) and intussusception was observed in one patient. Nine patients (10.6%) had renal involvement with mild nephropathy. The most frequently observed laboratory findings were increased C-reactive protein (47%) and leukocytosis (31%). Conclusion: HSP is commonly seen in children and leads to life-threatening complications in a minority of patients. Whole patients with GIS and renal involvement should be examined and monitored to assess the severity of the disease and any complications.

Clinico-laboratory profile of central nervous system infection by scrub typhus at a tertiary care hospital

Background: Scrub typhus is an emerging epidemic in India. Its course can be complicated by involvement of Respiratory system, cardiovascular system, gastrointestinal system and central nervous system. Central nervous system involvement can lead to major morbidity and mortality.Methods: It is an observational study conducted in the Department of Pediatrics, Patna Medical College and Hospital, Patna from January 2016 to August 2018. All cases of AES who were serologically proven to be due to scrub typhus were clinically examined, signs and symptoms were noted, and complete blood counts were done. Lumbar puncture was done, and CSF was sent for analysis of cells, sugar and protein, IgM for scrub typhus in CSF, gram staining and CSF culture.Results: Seventy-eight cases were tested positive for scrub typhus. Incidence of scrub typhus among AES cases was 19.4% in the study. Among cases of Scrub typhus, a male predominance and sex ratio was 1.68. Age at presentation ranged from 2 months to 12 years with a mean age of 7.28 years. Most of the cases were seen in the months of September and October with a mini peak in the month of March. Fever and Altered consciousness were present in all the cases. Among laboratory investigations, thrombocytopenia was the most common feature followed by raised Transaminases. CSF Analysis shows mildly increased cell count with lymphocytic predominance and presence of few polymorphs, sugar mildly decreased, and protein mildly raised.Conclusions: Scrub typhus is an important causative agent of AES in the Eastern parts of India. It should be suspected in cases which present as AES with symptoms of hepatosplenomegaly, thrombocytopenia, and elevated liver enzymes in addition to dengue encephalitis which forms its close differential.

The clinical spectrum of Henoch-Schönlein purpura in children: a single-center study.

Henoch-Schönlein purpura (HSP) is the most common vasculitis of children. The aim of this study is to evaluate the demographic and clinic findings of patients with HSP and also to determine predictive factors for assessing the development of gastrointestinal system (GIS) and renal involvement. This study was performed prospectively among children with HSP who are under 18years of age and being followed-up in the Pediatric Rheumatology Unit of Health Sciences University Kanuni Sultan Süleyman Training and Research Hospital between January 2016 and January 2018. A total of 265 patients, 137 boys (51.7%) and 128 girls (48.3%), were involved to the study. The mean ± standard deviation of age at the diagnosis was 7.5 ± 3.2. The most common disease onset season was spring (31.7%). The rate of arthritis, GIS involvement, and renal involvement were 54%, 51.3%, and 29.1%, respectively. GIS bleeding was more frequent in males than females (p = 0.007). Boys over 7years of age had significantly more common GIS bleeding (p = 0.04). Intussusception, relapse, and serious GIS involvement requiring hospitalization and steroid treatment were highly associated with severe renal involvement. We demonstrated that patients suffering intussusception, relapse, and serious GIS involvement or requiring hospitalization and steroid treatment had tendency to present with severe renal involvement. Therefore, these patients should be followed up carefully for not overlooking renal involvement of HSP.

Behçet's disease in children: single-center experience.

Aim:In this study, it was aimed to summarize the demographics, and clinical and laboratory findings of children who were diagnosed as having Behçet’s disease, and also to determine the efficacy, duration, and adverse effects of the treatments.Material and Methods:The records of 34 patients who were diagnosed according to the International Behçet Study Group criteria between 1980 and 2013 in the Department of Pediatric Nephrology and Rheumatology, Hacettepe University Faculty of Medicine, were retrospectively reviewed and all demographic and clinical features were recorded. In the light of these data, the sex and age distribution, clinical and laboratory findings, most commonly preferred treatment approaches, efficacy of treatments, and adverse effects during treatment were analyzed.Results:Of the 34 children with Behçet’s disease, 18 (53%) were male and 16 (47%) were female, and the mean age was 11.18±3.34 years. There was no significant difference in age distribution of the male and female patients (p<0.05). In 97% (n=33) of the patients, the first symptom was recurrent oral aphthae. In order of frequency, the other mucocutaneous findings were pseudofoliculitis and pustular lesions (82%), genital ulcers (62%), and pathergy positivity (50%). System involvements in order of frequency were as follows: joint findings (38%), ocular findings (35%), vascular involvement (32%), neurologic involvement (18%), gastrointestinal involvement (5.8%), and pulmonary involvement (5.8%). Colchicine was the most commonly preferred drug (88%). Steroids were added to treatment in patients with skin involvement. Azathioprine was added in patients with uveitis. Anticoagulant therapy, cyclophosphamide, and anti-tumor necrosis factor-alpha were added in patients with vascular involvement. In patients with gastrointestinal system involvement, sulfasalazine was added to treatment. Diarrhea was the most common adverse effect in patients who used colchicine. In the patients who used steroid treatment, gastrointestinal symptoms such as unintentional weight gain, acne, and agitation were observed (17%). One patient who received interferon treatment had symptoms of depression and agitation.Conclusion:The aim of this study was to review the general characteristics of pediatric patients with Behçet’s disease and to emphasize the importance of early diagnosis and correct treatment in terms of mortality and morbidity.

Clinical Profile and Outcome of Dengue Fever in Multidisciplinary Intensive Care Unit of a Tertiary Level Hospital in India.

ABSTRACTBackgroundIndia is one of the seven identified countries in South-East Asia region regularly reporting dengue fever (DF)/dengue hemorrhagic fever (DHF) outbreaks. Even though the dengue prodrome and evolution of illness are most often similar in many patients, progress and outcome may differ significantly depending on the severity of illness as well as treatment instituted. We studied the clinical manifestations, outcome and factors predicting mortality of serology confirmed dengue fever cases admitted in Multidisciplinary Intensive Care Unit (MICU) of a high acuity healthcare facility in India.MethodologyAll patients with serology proven dengue fever admitted to MICU between 1st July 2015 and1st December 2015 were included in the study. Clinical presentation, laboratory findings, severity of illness scores and outcome were recorded.ResultsMajority of the patients (58.4%) belonged to 21–40 year age group. Hepatic (96.8%) followed by hematological (79.2%) involvement were the most common findings. CNS involvement observed among 27%. Survival to hospital discharge was 78.9%. Respiratory and gastrointestinal system involvement was associated with increased mortality. Acute respiratory distress syndrome (ARDS), acute kidney injury (AKI) and shock were the clinical syndromes associated with mortality. Serum lactate, aspartate transaminase (AST) and alanine transaminase (ALT) were significantly elevated among non survivors. Significant difference in sequential organ failure assessment (SOFA) and acute physiology and chronic health evaluation (APACHE) scores was also observed among survivors and non survivors.ConclusionOrgan system involvement and higher disease severity scores are strong predictors of mortality. High index of suspicion for atypical manifestations of dengue is warranted.How to cite this articlePadyana M, Karanth S, Vaidya S, Gopaldas JA. Clinical Profile and Outcome of Dengue Fever in Multidisciplinary Intensive Care Unit of a Tertiary Level Hospital in India. Indian J Crit Care Med 2019;23(6):270–273.

Gastrointestinal amyloidosis occurring in three different patterns: case series

Systemic amyloidosis is a rare disease characterized by extracellular accumulation of amyloid protein in one or more organs. In patients with systemic amyloidosis, the most frequently affected organs are kidney and heart, followed by the nervous system, soft tissues, and lungs. Small bowel and liver involvement are also frequent in systemic amyloidosis. Gastrointestinal (GI) findings are common, and the degree of organ involvement determines the symptoms. Patients usually have nonspecific findings such as abdominal pain, nausea, diarrhea, and dysphagia, which may delay the appropriate diagnosis. Liver involvement occurs in the majority of patients, but the symptoms typically do not happen unless a marked hepatic amyloid deposition occurs. Diagnosis is by tissue biopsy. Treatment and prognosis depend on the underlying disease. GI system involvement is a sign of poor prognosis. In this case series, five patients who were diagnosed with gastrointestinal system amyloidosis in our clinic are presented.

Disease Spectrum and Frequency of Illness in Pediatric Emergency: A Retrospective Analysis From Karachi, Pakistan.

Background: The National Institute of Child Health (NICH) is the largest tertiary care pediatric hospital operating in Karachi, Pakistan. Its emergency department (ED) is always occupied. However, the spectrum of illness in patients presenting to this ED has not been investigated in depth to identify the most common presentations and to develop effective management for treating patients.Methods: This retrospective study included all children visiting the pediatric ED of the NICH from January 2017 through December 2017. Newborns to children 14 years of age were included, for a total cohort of 188,803 patients. Sociodemographic data and clinical information were extracted from the medical record. Univariate analysis was performed to determine the frequency and percentage for all the variables.Results: The cohort consisted of 9% (n=16,952) neonates (0 to 1 month) and 91% (n=171,351) older children (>1 month to 14 years). Among the neonates, 36.6% presented as triage level 1. Sepsis was diagnosed in 23.8% of neonates, low birth weight/preterm in 18.4%, and respiratory distress/pneumonia in 15.2%. In infants and older children, diagnoses related to the respiratory system (37.3%), gastrointestinal system (16.4%), and multisystem involvement (15.9%) were the most common. During the evening shifts, 38.1% of patients were seen, and on weekends, 51.6% of patients were seen. Sunday was the busiest day in the ED.Conclusion: The tertiary care pediatric EDs in Pakistan have witnessed an increasing number of critical emergencies over time. Respiratory and gastrointestinal emergencies form the majority of the ED burden. A surge of patients is seen on the weekends and during the evening shifts. The spectrum of illnesses should be investigated via prospective, longitudinal studies in other pediatric EDs in Pakistan to understand the trends and to provide the foundation for developing nationwide recommendations for improving pediatric emergency care.

Management of systemic sclerosis: A dermatologist's approach

Systemic sclerosis (SSc) is an autoimmune connective tissue disease affecting the blood vessels, skin, and internal organs. The main pathogenic mechanisms are vascular dysregulation, tissue fibrosis due to collagen and extracellular protein deposition, and autoantibody production due to immune dysfunction. The treatment also targets these mechanisms. Various criteria including the American College of Rheumatology and the European League against Rheumatism are laid down for the clinical diagnosis of scleroderma, and treatment is tailored to the systems involved. In the initial stages of the SSc, immunosuppressants are more useful, whereas in the later stages, antifibrotic therapies are important. Vasospasm and endothelial dysfunction are seen in all stages of the disease, and hence, vascular therapies are needed throughout the course of the disease. Special care should be taken to rule out gastrointestinal, pulmonary, and renal system involvement if the primary treatment is by a dermatologist.

Exclusive abdominal lymphadenopathy as a rare presentation of sarcoidosis

Sarcoidosis is a systemic granulomatous disease. The disease’s immunopathogenic mechanism is the activation of a cell mediated immune response. Lung is the most affected organ, but up to 30% of patients can have extrapulmonary manifestations. Case report: A 21-year-old male presented with a history of nausea, vomiting after meals, epigastric and periumbilical pain for approximately 6 months. Hyporexia and loss of 35 kilograms in 7 months were present, in addition to intermittent low nocturnal fever. His computed tomography (CT) scan (Figure 1) of the abdomen showed the presence of multiple, enlarged mesenteric lymph nodes with homogenous shape, measuring up to 2.0 cm in diameter, extending from the upper abdomen to the pelvis. Histopathologic findings of this lymph nodes showed a sarcoid appearance (Figure 2). Lymphoma, tuberculosis and other infections were excluded through immunohistochemical analysis. Treatment with prednisone was started initially at a dose of 40mg and after 5 weeks the patient were asymptomatic. One year and three months after the onset of treatment, corticosteroid dose reduction was attempted 3 times, with all of them leading to disease relapses. Treatment was optimized with 60mg of prednisone (for 2 weeks) plus 100mg of azathioprine daily. Discussion: The prevalence of gastrointestinal system involvement with clinical manifestations is 0,1 to 0,9%. However, the incidence of subclinical involvement can be higher. Enlarged lymph nodes is present in approximately 30% of cases. Treatment is indicated in sarcoidosis for patients with damage organ function or symptomatic disease and the first-line therapy is corticosteroid.

Determination of Risk Factors in Children Diagnosed With Henoch-Schönlein Purpura.

This study aims to evaluate the frequency of skin, gastrointestinal, joint, and renal involvement in children with Henoch-Schönlein purpura and investigate the risk factors for organ/system involvement. The data of 186 patients (97 boys, 89 girls; mean age 7.4±2.8 years; range 1.5 to 16.5 years) who were followed-up with the diagnosis of Henoch-Schönlein purpura in our clinic between October 2011 and December 2013 were evaluated retrospectively. Logistic regression analysis revealed that the risk of gastrointestinal system involvement was significantly higher in patients with skin lesions in the upper extremities (p=0.002, odds ratio [OR]=3.2). The risk of joint involvement was significantly higher in girls (p=0.024, OR=2.18), in patients with soft tissue swelling (p=0.005, OR=2.63), and with low mean platelet volume levels (p=0.008, OR=4.07). The risk of renal involvement was significantly higher in girls (p=0.047, OR=2.7), in patients >10 years (p=0.001, OR=1.4), and in patients with elevated C-reactive protein levels (p=0.007, OR=6.57). Having skin lesions in the upper extremities is a risk factor for gastrointestinal system involvement. Female sex, soft tissue swelling, and low mean platelet volume levels are risk factors for joint involvement. Female sex, >10 years of age, and high C-reactive protein levels are risk factors for renal involvement. Renal involvement in Henoch-Schönlein purpura is independent of gastrointestinal system or joint involvement and very close follow-up is required in the acute period particularly in girls >10 years with high C-reactive protein levels.

Mononeuritis multiplex: an uncommon neurological manifestation of cytomegalovirus reactivation in an HIV-infected patient

BackgroundCytomegalovirus (CMV) reactivation with neurological involvement in patients with acquired immunodeficiency syndrome (AIDS) is increasingly rare since the introduction of antiretroviral therapy (ART). Manifestations include encephalitis, myelitis, polyradiculopathy and, less commonly, mononeuritis multiplex (MNM). We report a case of disseminated CMV disease with gastrointestinal and peripheral and central nervous system involvement in a patient with AIDS, manifesting primarily as MNM.Case presentationA 31-year old woman with AIDS presented with a clinical picture of MNM. Electromyography confirmed the clinical findings. CMV DNA was detected in cerebrospinal fluid (CSF) and blood. Gastrointestinal involvement was histologically documented. HIV RNA was also detected in CSF and brain MRI was consistent with HIV encephalopathy. A diagnosis of disseminated CMV disease (with esophagitis, colitis, encephalitis and MNM) and HIV encephalopathy was made. Treatment consisted of ganciclovir and foscarnet, followed by maintenance therapy with valganciclovir. Evolution was favorable and valganciclovir was stopped after sustained immune recovery following ART initiation.ConclusionWe discuss the diagnostic approach to CMV neurological disease, with a focus on MNM and CMV encephalitis. Combination therapy with ganciclovir and foscarnet should be considered for all forms of neurological involvement, although available data are scarce. Since there is significant overlap between CMV encephalitis and HIV encephalopathy, ART drugs with higher CSF penetration may have to be considered. ART and immune recovery are essential to improve outcomes.

Exclusive abdominal lymphadenopathy as a rare presentation of sarcoidosis

Introduction: Sarcoidosis is a systemic granulomatous disease. The disease’s immunopathogenic mechanism is the activation of a cell mediated immune response. Lung is the most affected organ, but up to 30% of patients can have extrapulmonary manifestations.Case report: A 21-year-old male presented with a history of nausea, vomiting after meals, epigastric and periumbilical pain for approximately 6 months. Hyporexia and loss of 35 kilograms in 7 months were present, in addition to intermittent low nocturnal fever. His computed tomography (CT) scan (Figure 1) of the abdomen showed the presence of multiple, enlarged mesenteric lymph nodes with homogenous shape, measuring up to 2.0 cm in diameter, extending from the upper abdomen to the pelvis. Histopathologic findings of these lymph nodes showed a sarcoid appearance (Figure 2). Lymphoma,tuberculosis and other infections were excluded through immunohistochemical analysis. Treatment with prednisone was started initially at a dose of 40mg a day and after 5 weeks the patient was asymptomatic. One year and three months after the onset of treatment, corticosteroid dose reduction was attempted 3 times, with all of them leading to disease relapses. Treatment was optimized with 60mg of prednisone (for 2 weeks) plus 100mg of azathioprine daily.Discussion: The prevalence of gastrointestinal system involvement with clinical manifestations is 0,1 to 0,9%. However, the incidence of subclinical involvement can be higher. Enlarged lymph nodes are present in approximately 30% of cases. Treatment is indicated in sarcoidosis for patients with damage organ function or symptomatic disease and the first-line therapy is corticosteroids

Pinpoint Localized Odynophagia (PLO) as a Specific Symptom of Pill-induced Oesophagitis (PIO) in the Evaluation of Acute Retrosternal Chest Pain

36 out of 100 cases of retrosternal chest pains are due to oesophageal pathologies, and Pill-induced Oesophagitis (PIO) is one of them. PIO can present as retrosternal chest pain associated with various Gastrointestinal (GI) symptoms and require a high index of suspicion. PIO is a clinical diagnosis; and oesophagogastroscopy is required for confirmation of the diagnosis, to find out complications of PIO and to rule out other oesophageal disorders. Our aims of the present study were to study clinical profile, risk factors and endoscopic features of PIO. We have done a cross-sectional study of 1000 patients with acute retrosternal chest pain, and all patients of suspected upper gastrointestinal system involvement were subjected to oesophagogastroscopy. Patients having a history of pill ingestion followed by retrosternal chest pain with GI symptoms of less than 10 days duration and having typical endoscopy findings like kissing ulcer, multiple small discrete ulcers or erosion of esophagus were diagnosed as PIO after excluding other oesophageal pathologies. Among 1000 retrosternal chest pain patients, 450(45%) cardiovascular, 255(25.5%) respiratory, 248(24.85%) upper GI and 47(0.47%) had other system involvement. Among 248 GI patients, the frequency of symptoms was as follows: Pinpoint localized odynophagia (8.46%), non-localised odynophagia (12.09%), nausea (62.09%), vomiting (44.35%), dysphagia (3.62%), dyspepsia (13.70%) and hematemesis (0.8%). PLO, dysphagia, and hematemesis were significant symptoms of PIO (p<0.05). Endoscopic findings suggestive of PIO such as kissing ulcer, multiple small discrete ulcers, oesophageal erosions were observed in 91.30%, 47.83%, and 34.78% patients, respectively. Involvement of the middle third of esophagus was present in 74.19% and the lower third in 25.81% patients. Most of the patients with PLO had kissing oesophageal ulcer seen on endoscopy (pvalue =0.0002). The habit of consuming pill with less than 100 ml of water and consumption of night pill dose 10 minutes or less before sleeping were observed as significant risk factors for PIO (p value<0.05). PLO is a newly established and highly specific symptom of PIO of our study and it matches with kissing ulcer of the esophagus by endoscopy.

Management of major organ involvement of Behçet’s syndrome: a systematic review for update of the EULAR recommendations

To assess the efficacy and safety of treatment modalities for major organ involvement of Behçet's syndrome (BS), in order to inform the update of the EULAR recommendations for the management of BS. A systematic literature review of all randomized controlled trials, controlled clinical trials, or open label trials assessing eye, vascular, nervous system or gastrointestinal system involvement of BS was performed. If controlled trials were not available for answering a specific research question, uncontrolled studies or case series were also included. We reviewed the titles and abstracts of 3927 references and 161 studies met our inclusion criteria. There were only nine randomized controlled trials. Observational studies with IFN-α and monoclonal anti-TNF antibodies showed beneficial results for refractory uveitis. Meta-analysis of case-control studies showed that immunosuppressives decreased the recurrence rate of deep vein thrombosis significantly whereas anticoagulants did not. CYC and high dose glucocorticoids decreased mortality in pulmonary arterial aneurysms and postoperative complications in peripheral artery aneurysms. Beneficial results for gastrointestinal involvement were obtained with 5-ASA derivatives and AZA as first line treatment and with thalidomide and/or monoclonal anti-TNF antibodies in refractory cases. Observational studies for nervous system involvement showed improved outcome with immunosuppressives and glucocorticoids. Meta-analysis of case-control studies showed an increased risk of developing nervous system involvement with ciclosporin-A. The majority of studies related to major organ involvement that informed the updated EULAR recommendations for the management of BS were observational studies.

Myotonic dystrophytype 1 – report of non-24-h sleep-wake disorder with excessive daytime sleepiness

ABSTRACTMyotonic dystrophy (MD) is a neuromuscular disease with myotonia, progressive weakness, and involvement of CNS, heart, and gastrointestinal system. Excessive daytime sleepiness (EDS) in myotonic dystrophy type 1 (MD1) is related to sleep breathing diseases, restless leg syndrome, periodic limb movements during sleep and narcoleptic-like phenotype. However, authors highlight a central dysfunction of sleep regulation.We describe a 26-year-old, female, MD1 patient with EDS. Sleep diary/actigraphy evidenced two different circadian periods with values of 1442 and 1522 min. Agomelatine, 50 mg at night, was prescribed with improvement of the circadian rhythm and complaints of sleepiness.The identification of unanticipated causes of EDS, such as circadian rhythm disorders permits an appropriated treatment. As we know, it is the first relate of non-24-h sleep-wake disorder in patient with MD1. Sleep diary and actigraphy could be good options to investigate sleep-wake cycle disorder in patients with MD and EDS.

Henoch-Schonlein Purpura in childhood: a tertiary care hospital experience in Turkey

Background: This study examines the clinical and laboratory features of children with Henoch-Schönlein purpura.Methods: A retrospective study was applied for the 32 Henoch-Schönlein purpura patients who were monitored between January 2014 and June 2017 at the Pediatric clinic of a tertiary care hospital. The diagnoses were made based on the HSP criteria of the American College of Rheumatology. Age, gender, clinical and laboratory findings of the patients were recorded.Results: The mean age of the 32 Henoch-Schönlein purpura patients was 9.29±3.29 years. Complaints at the time of admission were as follows: 93.8% of the patients (n=30) had rash; 40.6% (n=13) had abdominal pain; and 59.4% (n=19) had joint involvement and inability to walk. The results of fecal occult blood test examined during the admission were found as negative for the 75% (n=25) of patients and positive for the 19% (n=6) of patients. According to the urinary specimens, which were considered as the evidence of renal involvement at the applications, proteinuria was found negative for the 27 (8.4%) patients and positive for the 5 (1.6%) patients; hematuria was negative for the 29 (90.6%) children and positive for the 3 (%9.4) children. Antihistamine and non-steroidal anti-inflammatory were started as the treatment to the patients. During the follow-up, the 15.5% of patients had positive fecal blood tests although their test results were negative at the admission. During the clinical follow-up, the presence of proteinuria was found positive for the 6.3% of patients who had negative proteinuria test at their admissions.Conclusions: Henoch-Schönlein purpura is a childhood vasculitis that manifests itself with rash and joint findings. It can cause significant complications due to gastrointestinal and genitourinary system involvement.