The Association of Telangiectasias with Other Peripheral Vascular Lesions of Systemic Sclerosis.

Purpose: Systemic sclerosis (SSc) is a relatively rare collagenosis manifested as microvasculopathy, excessive cutaneous and visceral fibrosis in a background of autoimmune alteration. Autoimmune vasculopathy in SSc occurs early and begins with endothelial cell activation followed by blood vessel intimal proliferation in a context of defective angiogenesis. The alteration of peripheral micro and macrocirculation in SSc is evident through vascular lesions, such as Raynaud's phenomenon, telangiectasias, acrocyanosis, digital ulcers, gangrene, peripheral pulse deficiency. Our paper details the results of the study on the association between telangiectasias and other types of immune-mediated peripheral vascular lesions that can be identified in SSc. The presence of these peripheral vascular lesions can provide information about the magnitude of the peripheral vasculopathy. Patients and Methods: A total of 37 patients diagnosed with SSc, recruited from a university clinic in Bucharest between February 2019 and March 2020, were enrolled in an observational study. We evaluated the presence of telangiectasias, as a stigma of autoimmune microvasculopathy, and their association with other immune-mediated peripheral vascular lesions that may be present in SSc. Results: The presence of telangiectasias was identified in the absence, but especially in the presence of acrocyanosis and digital ulcerations, and patients with peripheral pulse deficiency almost always had telangiectasias. Less than a quarter of the patients with digital ulcers progressed unfavorably to gangrene, and only one required amputation, telangiectasias being present not only in the patient with amputation but in all patients with gangrene. Conclusion: We appreciate that telangiectasias may be the clinical expression of peripheral vasculopathy characteristic of SSc, they can often be present in association with other peripheral vascular lesions and may represent a valuable indicator for the gangrene risk of digital ulcerations in SSc.

Immunologic and nonimmunologic sclerodermal skin conditions - review.

Scleroderma-like cutaneous lesions have been found in many pathological conditions and they have the clinical appearance of sclerotic or scleroatrophic lesions. Affected skin biopsies described histopathological changes similar to those of scleroderma located strictly on the skin or those of systemic sclerosis. These skin lesions can be found in inflammatory diseases with autoimmune substrate (generalized morphea, chronic graft versus host disease, eosinophilic fasciitis), tissue storage diseases (scleredema, scleromyxedema, nephrogenyc systemic fibrosis, systemic amyloidosis), metabolic diseases (porphyrya cutanea tarda, phenylketonuria, hypothyroidism, scleredema diabeticorum), progeroid syndromes. Given the multiple etiologies of sclerodermal lesions, a correct differential diagnosis is necessary to establish the appropriate treatment.

Sweet Syndrome Associated with Myelodysplastic Syndrome-A Review of a Multidisciplinary Approach.

Sweet syndrome (SS) is a rare disease described as a febrile neutrophilic dermatosis with acute onset, the pathogenesis of which has not yet been elucidated. The syndrome is characterized by the sudden onset of erythematous infiltrated papules or plaques located on the upper body and is associated with fever, leukocytosis and neutrophilia. The lesions show a dense dermal infiltration with mature neutrophils. The condition is responsive to systemic steroids. The central nervous system, bones, muscles, eyes, ears, mouth, heart, lung, liver, kidneys, intestines, and spleen may be affected by SS as extracutaneous manifestations. More and more cases have been found to be associated with malignancies, particularly myelodysplastic syndrome, and, less frequently, other hematologic malignancies or solid tumors. Approximately 21% of patients with SS have an associated malignancy and up to 80% of MASS cases are associated with hematological diseases, predominantly myelodysplastic syndrome (MDS) or acute myeloid leukemia (AML). Myelodysplastic syndrome is a clonal disease of the bone marrow characterized by inefficient hematopoiesis, dysplasia of the bone marrow and peripheral cytopenias. Affected patients have a high risk of leukemic transformation. After analyzing later studies and current practical aspects regarding MDS-related SS, we suggest an algorithm for evaluating these patients.

Hearing Loss Secondary to Systemic Sclerosis Vasculopathy: Case Study with a Short Review.

Systemic sclerosis (SSc) is a collagenosis with a substrate of chronic inflammation, which is determined by autoimmunity. The pathogenesis of this disease involves microvasculopathy (small vessel pathology) followed by excessive cutaneous and visceral fibrosis. Although acoustic and vestibular impairment is not classified as being a secondary pathology of SSc, several studies have identified cases of SSc that associate hearing loss and especially vertigo and tinnitus. This paper presents data from the medical literature that have identified vestibular and auditory symptoms among patients with SSc, associating the clinical case presentation of a patient suffering from SSc, which is associated with hearing loss. The need for additional studies on larger groups of patients is underlined, in order to clarify the impact of vasculopathy and fibrosis on the acoustic and vestibular analyzer in patients with SSc.

CREST Syndrome in Systemic Sclerosis Patients - Is Dystrophic Calcinosis a Key Element to a Positive Diagnosis?

Introduction: CREST syndrome is a clinical entity associated with systemic sclerosis, which meets at least three of the five clinical features: calcinosis, Raynaud's phenomenon, esophageal dysmotility, sclerodactyly, and telangiectasia. Three of these clinical features (Raynaud's phenomenon, sclerodactyly and esophageal dysmotility) are often present in classical subsets of SSc: limited and diffuse, and their presence in association does not define CREST syndrome. Calcinosis seems to be less common in SSc and its association with other clinical features is characteristic of CREST syndrome. Therefore, it can be appreciated that calcinosis is the key element of CREST syndrome. Methods: This study included a number of 37 candidates with SSc, diagnosed with the help of the American College of Rheumatology (ACR)/European League Against Rheumatism (EULAR) 2013 criteria. Results and Discussions: These three elements (calcinosis, Raynaud's phenomenon, esophageal dysmotility) were recorded both in the limited subset of SSc, but especially in the subset of diffuse SSc, contrary to the data in the literature. Conclusion: We appreciate that CREST syndrome is a clinical entity that can overlap with both subsets of SSc. Given the divergent views of the authors on the classification of CREST syndrome, future studies may contribute to a reassessment of SSc classification.

Retrospective national cohort study of pregnancy outcomes for women with type 1 and type 2 diabetes mellitus in Republic of Ireland.

AIM: Report the outcomes of pregnant women with type 1 and type 2 diabetes and to identify modifiable and non-modifiable factors associated with poor outcomes. METHODS: Retrospective analysis of pregnancy preparedness, pregnancy care and outcomes in the Republic of Ireland from 2015 to 2020 and subsequent multivariate analysis. RESULTS: In total 1104 pregnancies were included. Less than one third attended pre-pregnancy care (PPC), mean first trimester haemoglobin A1c was 7.2 ± 3.6% (55.5 ± 15.7 mmol/mol) and 52% received pre-conceptual folic acid. Poor preparation translated into poorer pregnancy outcomes. Livebirth rates (80%) were comparable to the background population however stillbirth rates were 8.7/1000 (four times the national rate). Congenital anomalies occurred in 42.5/1000 births (1.5 times the background rate). More than half of infants were large for gestational age and 47% were admitted to critical care. Multivariate analyses showed strong associations between non-attendance at PPC, poor glycaemic control and critical care admission (adjusted odds ratio of 1.68 (1.48-1.96) and 1.61 (1.43-1.86), p < 0.05 respectively) for women with type 1 diabetes. Smoking and teratogenic medications were also associated with critical care admission and hypertensive disorders of pregnancy. CONCLUSION: Pregnancy outcomes in women with diabetes are suboptimal. Significant effort is needed to optimize the modifiable factors identified in this study.

Oral Mucositis Induced by Chemoradiotherapy in Head and Neck Cancer-A Short Review about the Therapeutic Management and the Benefits of Bee Honey.

Background and Objectives: Oral mucositis, a severe non-hematological complication, can be induced by chemoradiotherapy. It is associated with severe local dysfunction, severely affecting the patient's quality of life; it increases the risk of oral infections and interrupts oncological treatment, thus prolonging the duration and cost of hospitalization. Besides all of the agents used in the prevention and treatment of oral mucositis induced by oncological treatment, can there be found an easier one to administer, with an effective preparation, high addressability, both for adults and paediatric patients, without side effects, and at the same time cheap and easy to purchase? The aim of the present paper is to demonstrate the existence of this product, which is available to everyone, having multiple benefits. Materials and Methods: For the purpose of writing this article, materials were searched in electronic databases in between 2019 and 2021, taking into consideration papers where authors have demonstrated the effectiveness of this product through its topical or systemic use. Results: Numerous studies have highlighted the benefits of honey on oral mucositis. Through its analgesic, anti-inflammatory, anti-cancerous and antibacterial action, honey has proved to have a major impact on the patient's quality of life and nutritional status by promoting tissue epithelialization and healing of the chemoradiotherapy-induced lesions. Conclusions: Superior to many natural agents, bee honey can be successfully used in both preventing and treating oral mucositis. There are currently numerous studies supporting and recommending the use of bee honey in the management of this oncological toxicity.

Paraclinical Aspects in Systemic Sclerosis.

Systemic sclerosis (SSc) is a chronic inflammatory disease with an autoimmune substrate that affects the skin and a large number of internal organs. The chronic inflammatory process is sustained by a wide range of cytokines and chemokines, which are discharged by inflammatory cells, with fibrosis and nail bed vascular changes (disorganized vasculature architecture with microhemorrhages, megacapillaries and areas without capillaries). Confocal microscopy contributes to the understanding of the molecular mechanism involved in chronic inflammation and mainly targets the field of research. Coherent optical tomography, capillaroscopy, and skin biopsy are useful for the differential diagnosis of SSc with other sclerodermoid syndromes. The immunological profile is a classification criterion for SSc and directs the diagnosis to the two subsets of the disease. Multisystemic damage requires evaluation with the help of a set of investigations specific to each affected organ, such as: diffusing capacity for carbon monoxide, forced vital capacity, 6-minute walk test, high-resolution computed tomography standard and reduced sequential, cardiac ultrasound and right cardiac catheterization. The current possibilities of diagnosis, treatment and monitoring are permanently adapting to new medical discoveries.

Clinical, Imaging, Histological and Surgical Aspects Regarding Giant Paraovarian Cysts: A Systematic Review.

Paraovarian cysts (POCs) develop within the broad ligament of the uterus. POCs are considered to be giant when the threshold of 150 mm is exceeded. Clinical signs and symptoms occur as a consequence of the pressure effect on adjacent organs or due to complications. Abdominal ultrasonography, computed tomography or magnetic resonance imaging are useful imaging tools, but most often the exact origin of such voluminous cysts is revealed only by surgical exploration. The review aims to appraise and update the diagnostic, the histological aspects and the treatment of the giant POCs in rare cases. We carried out a systematic search in Medline-PubMed, Google Scholar and ResearchGate electronic databases. Twenty-seven papers fulfilling the selection criteria were included in the review. The data extracted included information about first author, year of publication, country, patient age, size and side of the POCs, symptoms, tumoral markers, imaging methods, preoperative diagnosis, surgical management and histopathological findings. Although not very numerous, all the studies highlighted the low incidence of giant POCs, the impossibility of establishing the origin of the cystic mass by clinical and imaging methods even with advanced technical tools and the low risk of torsion (11.1%). Despite the recognized benign nature of POCs, we found an unexpected high percent (25.9%) of borderline giant POCs. Surgical excision is the only treatment option. Ovarian-sparing surgery was performed in 85.1% of the cases, and minimally invasive techniques were applied in only 42.9% of the patients, which demonstrates the need of a high-level laparoscopic expertise. Knowledge of this pathology, its recognition as a possible etiology of an abdominopelvic cyst, and a higher awareness of the possibility of a borderline histology in giant POCs are required for the proper management of these particular cases.

Cystic Fibrosis-Related Diabetes Mellitus and Pregnancy: A Retrospective Study.

INTRODUCTION: Cystic fibrosis-related diabetes mellitus (CFRDM) is becoming a more common issue in pregnancy care as the life expectancy of females living with cystic fibrosis has improved, with an increasing number of pregnancies in this population. Despite the Republic of Ireland having the highest incidence of cystic fibrosis globally, there is limited Irish data on pregnancy outcomes for those with CFRDM. This study aimed to retrospectively review maternal and foetal outcomes of pregnancies affected by maternal CFRDM. METHODS: The patient records of all women with CFRDM who attended the National Maternity Hospital Dublin for obstetric care between 2015 and 2019 were retrospectively reviewed. RESULTS: A search of patient records identified 15 pregnancies in 12 women with CFRDM during the study period. CFRDM was diagnosed pre-conception in ten of the 15 pregnancies. Median neonatal weight at birth was lower in women with CFRDM diagnosed pre-conception compared to women diagnosed during pregnancy (2.8 vs. 3.02 kg). The median weight gain in women with CFRDM diagnosed pre-conception was 10.9 kg compared to 11.9 kg for those diagnosed during pregnancy. The majority of women (62.5%) with CFRDM diagnosed pre-conception delivered via caesarean section. Admission for CF exacerbations during pregnancy in women with CFRDM diagnosed pre-conception was very common (87.5%) compared with 75% of those diagnosed during their pregnancy. CONCLUSION: Women diagnosed with CFRDM were likely to require caesarean section, to be treated with insulin, and to be frequently admitted to hospital for CF exacerbations. Our review highlights the importance of good glucose control, stable cystic fibrosis before pregnancy and a multidisciplinary team approach.

[Spontaneous bacterial peritonitis: a severe complication of liver cirrhosis]. / Peritonita bacteriana spontana: complicatie severa a cirozei hepatice--studiu prospectiv.

UNLABELLED: Spontaneous bacterial peritonitis (SBP) is a frequent and severe complication of decompensated cirrhosis. SBP is defined as an infection of initially sterile ascitic fluid (AF) without a detectable, surgically treatable source of infection. AIM: To evaluate the prevalence, incidence, pathogens and clinical outcome of patients with liver cirrhosis and SBP. METHODS: A prospective study evaluated 34 episodes of SBP developed in 29 patients with cirrhosis and ascites admitted in hospital. SBP was diagnosed based on a polymorphonuclear cell count in ascitic fluid of > 250 cells/mm3 in the absence of data compatible with secondary peritonitis. RESULTS: The mean age of the group was 56.89 +/- 8.79 years, more frequently occurred in men 21 of 29 cases (72.41%). Microorganisms were isolated in 12 episodes (35.29%): Gram-negative bacteria in 8 (66.67%) and Gram-positive in 4 (33.33%). The first-line treatment failure and in-hospital mortality rates were 26.47% and 17.64%, respectively. The low in-hospital mortality seems to be related to earlier diagnosis and treatment. In the multivariate analysis, three significant independent predictive factors of in-hospital mortality were identified: a high Model of End-Stage Liver Disease (MELD) score, renal failure and SBP caused by extended-spectrum a-lactamase-producing organisms. The relapse rate of PBS was 21.73%. CONCLUSIONS: SBP remains to be a serious complication, especially in patients with a high MELD score. Ceftazidim represents a good choice in empirical treatment of SBP.

[Quality of interior air: biological contaminants and their effects on health; bioaerosols and gathering techniques]. / Calitatea aerului interior: contaminantii biologici si efectele asupra sanatatii; bioaerosolii si tehnici de prelevare.

Indoor Air Quality: biological contaminants and health effects; airborne organisms and sampling instruments. Biological contaminants include bacteria, molds, viruses, animal dander and cat saliva, house dust, mites, cockroaches and pollen. Symptoms of health problems caused by biological pollutants include sneezing, watery eyes, coughing, shortness of breath, dizziness, lethargy, fevers. Children, elderly people with breathing problems, allergies and lung diseases are particularly susceptible to disease-causing biological agents in the indoor air. It is convenient to consider microbiological samplers for collecting organisms in air as falling into several broad categories. Many popular microbiological air samplers use the principle of impaction to trap the organisms by impacting them directly on to agar. Further distinct groups are the impingers, which operate by impinging organisms into liquid.

[Gastrointestinal stromal tumors. Actual diagnosis and treatment]. / Tumorile stromale gastrointestinale diagnostic si tratament actual.

Gastrointestinal stromal tumors (GISTs) are currently defined as c-Kit-positive mesenchymal tumors with specific histological characteristics. CD117 (c-Kit protein) is a growth factor receptor with tyrosine kinase activity and product of the protooncogene c-Kit. The most frequent sign is gastrointestinal haemorrhage, which occurs in approximately one third of patients. The evaluations which are necessary for diagnosis include clinical examination, immunohistochemistry analysis, endoscopic examination, X-ray, abdominal CT scan, ultrasound, MRI, exploratory surgery, biopsy or fine-needle aspiration of the tumor. Current data suggest a major role of a new tyrosine kinase inhibitor, imatinib mesylate (Glivec), for patients with metastatic or advanced disease. Surgical resection is the treatment of choice and offers the only chance for cure.

[Colonic diverticulosis--current issues in etiopathogenesis, diagnosis and treatment]. / Diverticuloza colonica--aspecte actuale de etiopatogeneza, diagnostic si tratament.

Diverticular disease of the colon, which is an acquired disorder caused by mucosal herniation through the colonic wall, has been termed both a "disease of the 20th century" and a "disease of Western civilization" due to its increasing prevalence in modern times and its striking geographical variability. There are complex relations between colonic structure, motility, and dietary factors, and it is likely that all of these play a role in the pathogenesis to a greater or lesser degree. Although present in two thirds of the elderly population, the large majority of patients will remain entirely asymptomatic. Nonetheless, 20% of those affected may manifest clinical illness and complications. Barium exploration indicates the extent and severity of colonic diverticular disease. Colonoscopy is preferred in positive diagnosis of uncomplicated diverticulosis. Computed tomography is especially useful in complicated diverticular disease. The use of high fibre diets in the prevention and treatment of the symptoms of diverticular disease has now become commonplace. The morbidity and mortality associated with complications, demand that this condition should receive greater attention in terms of aetiology, prevention and management.