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עמוד בית
Fri, 22.11.24

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October 2015
Jalaa Zarroug MD, Graham R.V. Hughes MD FRCP and Christopher J. Edwards MD FRCP
September 2015
Shannon L. Castle MD, Osnat Zmora MD, Stephanie Papillon MD, Dan Levin MD and James E. Stein MD

Background: Gastric bezoars in children are infrequent. Most are trichobezoars. Surgical intervention is sometimes necessary.

Objectives: To describe the clinical findings and radiological workup, as well as treatment and outcome of patients with complicated gastric bezoars who underwent surgery in our institution.

Methods: We conducted a retrospective review of all cases of surgery for gastric bezoars performed in our institution between 2000 and 2010. Data collected included gender and age of the patients, composition and extent of the bezoar, presenting signs and symptoms, imaging studies used, performance of endoscopy, and surgical approach. Outcome was measured by the presence of postoperative complications.

Results: We identified seven patients with gastric bezoars who underwent surgery. All were females aged 4–19 years. Six had trichobezoars and one had a mass composed of latex gloves. Presenting symptoms included abdominal pain, vomiting, weight loss, and halitosis. All patients had a palpable epigastric mass. A large variety of imaging modalities was used. Endoscopic removal was attempted in three patients but failed, and the laparoscopic approach was attempted in one patient and failed. All patients eventually underwent laparotomy with gastrotomy and recovered without complications. 

Conclusions: The presence of gastric bezoars should be suspected in any child with unexplained abdominal pain, vomiting, weight loss, or halitosis, or with a palpable abdominal mass, especially in girls. A variety of imaging modalities can aid in diagnosis. Endoscopic removal might be attempted, although failure of this approach is frequent and must prompt surgical intervention, preferably laparotomy and gastrotomy, which has an excellent outcome.

 

August 2015
Keren Mahlab-Guri MD, Ilan Asher MD, Tanir Allweis MD, Judith Diment MD, Zev M. Sthoeger MD and Eliezer Mavor MD

Background: Granulomatous lobular mastitis (GLM) is a rare disorder that can clinically mimic breast carcinoma. The recommendation for diagnosis and treatment of GLM has not yet been established. 

Objectives: To assess a series of GLM patients, including their clinical presentation, diagnosis, treatment and outcome. 

Methods: We retrospectively analyzed the clinical data and treatment of 17 female patients with biopsy-proven GLM. Breast tissue was obtained by a core needle biopsy (15 patients) or open biopsy (2 patients). Images were reviewed by an experienced radiologist.

Results: The mean age of the patients at diagnosis was 44.6 ± 12.6 years. Five patients (29%) presented with bilateral disease, and seven (41%) presented with a mass, suggesting the initial diagnosis of breast carcinoma. Treatment comprised observation alone (23%), antibiotics (58.8%) and/or corticosteroids (with or without methotrexate) (35%). At the end of the study 70.6% of the patients demonstrated complete remission. None of the patients developed any systemic (granulomatous) disease or breast carcinoma during the follow-up period (4.7 ± 3.8 years). 

Conclusions: Core needle biopsy is mandatory for the diagnosis of GLM and the exclusion of breast carcinoma. The recommended treatment modalities are observation alone or corticosteroids; surgery should be avoided. GLM is a benign disease with a high rate of resolution and complete remission.

 

Rafael S. Carel MD DrPH, Inna Brodsky MPH and Giora Pillar MD MPH

Background: Obstructive sleep apnea (OSA) is a common health problem with an estimated prevalence of 4% among men, many of whom are undiagnosed and untreated. 

Objectives: To compare demographic characteristics, health profiles, risk factors, and disease severity in Arab and Jewish men with OSA syndrome.

Methods: In this cross-sectional study we retrospectively analyzed clinical data from the medical files of men ≥ 22 years old who were referred to the Rambam Medical Center sleep clinic during the period 2001–2009 with a suspected diagnosis of OSA. OSA severity was measured using the apnea-hypopnea index (AHI). Categorical variables were compared using the chi-square test. Relations between OSA severity and a set of independent risk factors were assessed by linear regression analysis.

Results: A total of 207 men were included (39 Arabs, 19%; 168 Jews, 81%). Arab participants were younger than their Jewish counterparts (45.5 ± 8.9 years vs. 49.8 ± 11.8, P = 0.04) and their body mass index (BMI) was higher (33.1 ± 5.1 vs. 30.0 ± 4.4, P = 0.001). OSA severity (AHI score) was higher among Arab men, with low, medium and high severity scores seen in 10%, 33% and 56% of Arab men vs 35%, 29% and 37% of Jewish men, respectively [T(198)=2.39, P = 0.02]. Mean blood oxygen saturation was comparable.

Conclusions: Arab men presenting for evaluation of sleep apnea harbored more severe OSA symptoms, were younger, and had higher BMI compared to Jewish men. Since OSA syndrome evolves for several years until it becomes severe, these findings suggest that Arab men seek medical assistance later than Jewish men with OSA.

 

June 2015
Barak Benjamin MD, Roy Zaltzman MD PhD, Baruch Shpitz MD, Carlos R. Gordon MD DSc and Shmuel Avital MD
March 2015
Itay A. Sternberg MD, Benjamin F. Katz MD, Lauren Baldinger DO, Roy Mano MD, Gal E. Keren Paz MD, Melanie Bernstein BA, Oguz Akin MD, Paul Russo MD and Christoph Karlo MD

Abstract

Background: Renal hemangiomas are rare benign tumors seldom distinguished from malignant tumors preoperatively.

Objectives: To describe the Memorial Sloan-Kettering Cancer Center (MSKCC) experience with diagnosing and treating renal hemangiomas, and to explore possible clinical and radiologic features that can aid in diagnosing renal hemangiomas preoperatively.

Methods: Patients with renal hemangiomas treated at MSKCC were identified in our prospectively collected renal tumor database. Descriptive statistics were used to describe the patient characteristics and the tumor characteristics. All available preoperative imaging studies were reviewed to assess common findings and explore possible characteristics distinguishing benign hemangiomas from malignant renal tumors preoperatively.

Results: Of 6341 patients in our database 15 were identified. Eleven (73%) were males, median age at diagnosis was 53.3 years, and the affected side was evenly distributed. All but two patients were treated surgically. The mean decrease in estimated glomerular filtration rate (eGFR) after surgery was 36.3%; one patient had an abnormal presurgical eGFR and only two patients had a normal eGFR after surgery. We could not identify radiographic features that would make preoperative diagnosis certain, but we did identify features characteristic of hepatic hemangiomas that were also present in some of the renal hemangiomas.

Conclusions: Most renal hemangiomas cannot be distinguished from other common renal cortical tumors preoperatively. In select cases a renal biopsy can identify this benign lesion and the deleterious effects of extirpative surgery can be avoided.

January 2015
Maria A. Martínez-Godínez MSc MD1, Maria P. Cruz-Domínguez DSc, Luis J. Jara MD, Aarón Domínguez-López DSc, Rosa A. Jarillo-Luna DSc, Olga Vera-Lastra MD, Daniel H. Montes-Cortes DSc, Rafael Campos-Rodríguez DSc, Dulce M. López-Sánchez MSc, Cesar M. Mejía-Barradas DSc, Enrique E Castelán-Chávez MSc and Angel Miliar-García DSc

Background: The activated NLRP3 inflammasome is associated with the etiology of fibrotic diseases. The role of inflammasomes in SSc is still poorly understood.

Objectives: To determine the expression of NLRP3 (nucleotide-binding domain, leucine-rich-repeat-containing family, pyrin domain-containing 3) in the skin of patients with systemic sclerosis (SSc) and its relationship with pro-inflammatory cytokines and vascular mediators expression.

Methods: Skin biopsies were taken from 42 patients with either limited or diffuse SSc (21 lcSSc and 21 dcSSc), and from 13 healthy individuals. Using real-time polymerase chain reaction (PCR), the relative expression of caspase-1, IL-1β, IL-18, IL-33, TGF-β, ET-1, iNOS and eNOS genes, were measured. The location of NLRP3 and IL-1β were also determined by immunohistochemistry. Clinical characteristics were evaluated.

Results: The mean age of the patients was 49.3 ± 12.9 (lcSSc), 44.6 ±1 3.8 (dcSSc), and 45 ± 14.1 (healthy individuals). Compared to healthy individuals, the skin of both subtypes of SSc showed a significant increase (P < 0.05) in NLRP3, caspase-1, IL-1β, IL-18 and ET-1. Samples of lcSSc also showed a significant increase of eNOS (P < 0.029), iNOS (P < 0.04) and TGF-β (P < 0.05). Dermal fibrosis evaluated by modified Rodnan skin score (MRSS) had significant correlation with NLRP3, IL-1β, IL-18, and ET-1. Immunohistochemical analysis showed stronger staining of NLRP3 and IL-1β cytoplasmic expression in the keratinizing squamous epithelium of skin from SSc patients compared to controls.

Conclusions: This study identified NLRP3 over-expression in skin of patients with SSc. Skin thickness correlates positively with the NLRP3 inflammasome gene expression and with the vascular mediator and pro-fibrotic ET-1, suggesting that NLRP3 inflammasome plays a role in the pathophysiology of skin fibrosis in human SSc.

November 2014
Evgeni Brotfain MD, Alexander Zlotnik MD PhD, Andrei Schwartz MD, Amit Frenkel MD, Leonid Koyfman MD, Shaun E. Gruenbaum MD and Moti Klein MD

Background: Optimal oxygen supply is the cornerstone of the management of critically ill patients after extubation, especially in patients at high risk for extubation failure. In recent years, high flow oxygen system devices have offered an appropriate alternative to standard oxygen therapy devices such as conventional face masks and nasal prongs.

Objectives: To assess the clinical effects of high flow nasal cannula (HFNC) compared with standard oxygen face masks in Intensive Care Unit (ICU) patients after extubation.

Methods: We retrospectively analyzed 67 consecutive ventilated critical care patients in the ICU over a period of 1 year. The patients were allocated to two treatment groups: HFNC (34 patients, group 1) and non-rebreathing oxygen face mask (NRB) (33 patients, group 2). Vital respiratory and hemodynamic parameters were assessed prior to extubation and 6 hours after extubation. The primary clinical outcomes measured were improvement in oxygenation, ventilation-free days, re-intubation, ICU length of stay, and mortality.

Results: The two groups demonstrated similar hemodynamic patterns before and after extubation. The respiratory rate was slightly elevated in both groups after extubation with no differences observed between groups. There were no statistically significant clinical differences in PaCO2. However, the use of HFNC resulted in improved PaO2/FiO2 post-extubation (P < 0.05). There were more ventilator-free days in the HFNC group (P < 0.05) and fewer patients required re-intubation (1 vs. 6). There were no differences in ICU length of stay or mortality.

Conclusion: This study demonstrated better oxygenation for patients treated with HFNC compared with NRB after extubation. HFNC may be more effective than standard oxygen supply devices for oxygenation in the post-extubation period.

October 2014
Elisabetta Borella MD, Lavinia Palma MD, Margherita Zen MD, Silvano Bettio MD, Linda Nalotto MD, Mariele Gatto MD, Marta Domeneghetti MD, Luca Iaccarino MD, Leonardo Punzi and Andrea Doria MD
Autoinflammatory (AIF) and autoimmune (AIM) diseases are chronic immune disorders characterized by dysregulation of the immune system. Most AIF diseases are monogenic diseases which lead to hyperactivation of the inflammasome and release of pro-inflammatory cytokines, such as interleukin-1 (IL-1) and IL-18, resulting in tissue inflammation. Besides, the main feature of autoimmune diseases is the loss of tolerance of the adaptive immune cells against self antigens. Most AIF diseases are polygenic and numerous immune pathogens are involved in organ damage. The involvement of some AIF-associated mechanisms in AIM diseases, i.e., the activation of the inflammasome and the role of IL-1, was recently recognized. Moreover, some single nucleotide polymorphisms of the inflammasome genes have been proven to be involved in the development of AIF-related inflammatory features in autoimmune patients. These observations raise the possibility of using some anti-inflammatory drugs, like IL-1 antagonists, in autoimmune diseases with autoinflammatory features. 
Serena Colafrancesco MD, Roberta Priori MD PhD, Cristiano Alessandri MD, Elisa Astorri MD PhD, Carlo Perricone MD, Miri Blank PhD, Nancy Agmon-Levin MD, Yehuda Shoenfeld MD FRCP MaACR and Guido Valesini MD
August 2014
Matti Eskelinen MD PhD, Tuomas Selander MSc, Pertti Lipponen MD PhD and Petri Juvonen MD PhD

Background: The primary diagnosis of functional dyspepsia (FD) is made on the basis of typical symptoms and by excluding organic gastrointestinal diseases that cause dyspeptic symptoms. However, there is difficulty reaching a diagnosis in FD.

Objectives: To assess the efficiency of the Usefulness Index (UI) test and history-taking in diagnosing FD.

Methods: A study on acute abdominal pain conducted by the World Organizati­on of Gast­roentero­logy Research Committee (OMGE) included 1333 patients presenting with acute abdo­minal pain. The clinical history-taking variables (n=23) for each pa­tient were recorded in detail using a prede­fined structured data collection sheet, and the collected data were compared with the final diagnoses.

Results: The most signifi­cant clinical history-taking variables of FD in univa­riate analysis were risk ratio (RR): location of pain at diagnosis (RR = 5.7), location of initial pain (RR = 6.5), previous similar pain (RR = 4.0), duration of pain (RR = 2.9), previous abdominal surgery (RR = 4.1), previous abdominal diseases (RR = 4.0), and previous indigestion (RR = 3.1). T­he sensi­tivity of the physicians’ initial de­cisi­on in detecting FD was 0.44, speci­fi­city 0.99 and effi­ciency 0.98; UI was 0.19 and RR 195.3. In the stepwise multivariate logistic regression analysis, the independent predictors of FD were the physicians’ initial decision (RR = 266.4), location of initial pain (RR = 3.4), duration of pain (RR = 3.1), previous abdominal surgery (RR = 3.7), previous indigestion (RR = 2.2) and vomiting (RR = 2.0).

Conclusions: The patients with upper abdominal pain initially and a previous history of abdominal surgery and indigestion tended to be at risk for FD. In these patients the UI test could help the clinician differentiate FD from other diagnoses of acute abdominal pain.

May 2014
Ilan Ben-Zvi MD and Avi Livneh MD
Familial Mediterranean fever (FMF) is a genetic autoinflammatory disease characterized by spontaneous short attacks of fever, elevated acute-phase reactants, and serositis. Approximately 5%–10% of FMF patients do not respond to colchicine treatment and another 5% are intolerant to colchicine because of side effects. Recently, following the discovery of the inflammasome and recognition of the importance of interleukin-1β (IL-1β) as the major cytokine involved in the pathogenesis of FMF, IL-1β blockade has been suggested and tried sporadically to treat FMF, with good results. To date, case reports and small case series involving colchicine-resistant FMF patients and showing high efficacy of IL-1β blockade have been reported. At the Israel Center for FMF at the Sheba Medical Center the first double-blind randomized placebo-controlled trial of anakinra in FMF patients who are resistant or intolerant to colchicines is underway. In this report we discuss the mechanism of colchicine resistance in FMF patients, the data in the literature on IL1β blockade in these patients, and the anakinra trial inclusion criteria and study protocol.

Donato Rigante MD PhD, Aurora Rossodivita MD PhD and Luca Cantarini MD PhD
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