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.

The Influence of Living in Rural Areas on the Evolution and Management of Pediatric Ovarian Cystic Lesions: A Retrospective Study on a Cohort from South Eastern Romania.

Background: The incidence of ovarian cystic lesions (OCLs) in pediatric patients has been increasing in recent years. An early diagnosis is mandatory for a favourable prognosis but it depends on the primary medical care services and on the socioeconomic status of the patient. The present study aims at assessing the prevalence and the age-specific frequencies of pediatric OCLs, as well as identifying disparities between subjects in the urban and the rural areas, in order to explore the extent to which OCLs occurrence, diagnosis, evolution and treatment differ in the case of patients living in rural areas. Methods: A 3-year retrospective study was conducted between 2017 and 2019. All the female patients aged between 0 and 18 with OCLs ≥10 mm (N = 488), diagnosed and treated at "Sf.Ioan" Clinical Emergency Hospital for Children in Galati were included in the study. The Chi2 test was used for comparing the distributions of frequencies, and the t-Student test was used for comparing the means of any two normally distributed variables. The statistical significance threshold (p) was set at 0.05. Results: The prevalence of OCLs ≥10 mm was 14.85%, 47.13% of the subjects originating in rural area (p = 0.62). Different from the urban areas, the rural ones are characterised by a lower proportion of patients with BMI ≥25 (p = 0.002), larger OCLs mean size (p = 0.278), a more frequent complex aspect on ultrasonography (p = 0.01), and a smaller number of general physician referrals (p = 0.005). Moreover, a higher proportion of rural patients were intraoperatively diagnosed with OCLs (p = 0.044), had complicated OCLs (p = 0.012) and had their OCLs surgically treated OCLs (p < 0.01). Conclusion: Taking into consideration the socio-economic situation of south eastern Romania, patients living in rural areas have proven exposed to a higher risk of presenting with larger, complex and complicated OCLs, which most often require surgical treatment.

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.

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.

A Challenge of COVID-19: Associated Infective Endocarditis with Streptococcus gordonii in a Young Immunocompetent Patient.

The COVID-19 pandemic is a new challenge for the diagnosis and treatment of infective endocarditis (IE). Fever and other unspecific symptoms of coronaviral infection could be misleading or masking its manifestations. We present the case of a young patient admitted for persistent fever, profuse sweating, headache, articular pain, myalgias, and weight loss. She reported regression taste and smell disorders compared to a month earlier when diagnosed with moderate COVID-19 pneumonia. While the RT-PCR SARS-COV-2 test was positive, she was admitted to a COVID-19 ward. Investigations of febrile syndrome revealed two positive blood cultures with Streptococcus gordonii and the presence of vegetations on the aortic valve, supporting a certain diagnosis of IE. After six weeks of antibiotic treatment, the patient had clinical and biologic favorable outcomes. Streptococcus gordonii is a common commensal related to the dental biofilm, although there were no caries in our patient. The influence of COVID-19 infection on the human microbiome by modifying the virulence of some commensal germs may be a risk factor for IE pathogenesis on native valves and requires the vigilance of clinicians for suspicion of this disease.

AIDS Related Kaposi's Sarcoma: A 20-Year Experience in a Clinic from the South-East of Romania.

Kaposi's sarcoma (KS) was peculiarly described in the first notified cases of the acquired immunodeficiency syndrome as an opportunistic condition. However, the medical progress and the development of active antiretroviral therapy allowed the control of the HIV/AIDS epidemic, although the features of KS have changed throughout the past decades. The purpose of our study is to assess the epidemiological and clinical features of AIDS related KS in Romanian patients. A retrospective follow-up study was achieved in a single infectious diseases' clinic from Galati-Romania, between 2001 and 2021. Referring to 290 new HIV diagnosed cases from our clinic retained in care, the prevalence of KS was 3.4%. The main characteristics of patients with KS are a median age of 33, a predominance of males, prevalent severe systemic forms of diseases, frequent association of past or concomitant tuberculosis, and context of immune reconstruction syndrome. The mortality rate was 70%. KS has occurred in patients with delayed HIV diagnoses and inadequate adherence to therapy. Early recognition of both infections, the close monitoring of latent or symptomatic tuberculosis, improving the antiretroviral adherence and raising the access to oncologic procedures in Romanian HIV patients could improve their prognosis related to KS.

Emerging Infection with Elizabethkingia Meningoseptica in Neonate. A Case Report.

BACKGROUND: Elizabethkingia meningoseptica are Gram-negative rod bacteria which are commonly found in the environment. The bacteria have also been associated with nosocomial infections, having been isolated on contaminated medical equipment, especially in neonatal wards. CASE REPORT: Here, we present the case of a premature female infant born at 33 weeks' gestational age, with neonatal meningitis. The onset was marked by fever, in the 5th day of life, while in the Neonatal Intensive Care Unit. The patient was commenced on Gentamicin and Ampicillin, but her clinical condition worsened. Psychomotor agitation and food refusal developed in the 10th day of life, and a diagnosis of bacterial meningitis was made based on clinical and cerebrospinal fluid findings. A strain of Elizabethkingia meningoseptica sensitive to Vancomycin, Rifampicin and Clarithromycin was isolated from cerebrospinal fluid. First-line antibiotic therapy with Meropenem and Vancomycin was adjusted by replacing Meronem with Piperacillin/Tazobactam and Rifampicin. The patient's clinical condition improved, although some isolated febrile episodes were still present. The cerebrospinal fluid was normalized after 6 weeks of antibiotic treatment, although periventriculitis and tetraventricular hydrocephalus were revealed by imaging studies. Neurosurgical drainage was necessary. CONCLUSION: Elizabethkingia meningoseptica can cause severe infection, with high risk of mortality and neurological sequelae in neonates. Intensive care and multidisciplinary interventions are crucial for case management.

MRI differential diagnosis: bone metastases versus bone lesions due to malignant hemopathies.

Bone determinations are usually the first sign of disseminated cancers, whether is a hematological malignancy or other type of neoplasia. The aim of this paper is the possibility of differentiating the bone lesions from hematological malignancies by other malignancies that give bone metastases for the purpose to guide the clinician concerning causality of bone lesions. The research involved a retrospective study, which included 309 cases that were investigated by magnetic resonance imaging (MRI) at a segment of the spine, between 2010 and 2014, from which 137 were diagnosed with a form of hematological neoplasia, and the remaining had another form of cancer. Imaging aspect differs in these two study groups. Bone determinations due to malignant hemopathies (MH) were in general hypointense on T1-weighted sequences, iso- or hyperintense on T2-weighted sequences. On the other hand, bone metastases were hypo- or isointense on T1-weighted sequences, and had no specific signal intensity on T2-weighted sequences. In post-contrast images, all lesions showed contrast enhancement, with some differences. In terms of imagistic aspect, there are certain characteristics that can make a clear differentiation between bone determinations due to MH from the bone metastases, and some are found in the majority of the cases studied.

The use of Doppler ultrasonography for pre- and post-surgery monitoring of abdominal aortic aneurysm. Case report.

A 75 year old male patient was monitored for 3 years by Doppler Ultrasonography (US) for an abdominal aorta aneurysm (AAA). Because the aneurysm increased significantly, an aortic prosthesis was installed via an endovascular procedure. After one month of post-surgery monitoring, both Doppler US exam and contrast enhancement US (CEUS) suspected the presence of a leak at the level of the prosthesis. A new surgical procedure was scheduled and intraoperative arteriography confirmed an endoleak type II. Although not always able to specify the correct type of linkage, CEUS remains a reliable method for investigating the postoperative complications of AAA.

Parkinsonian Syndrome and Toxoplasmic Encephalitis.

Toxoplasmosis encephalitis in patients with human immunodeficiency virus may progress rapidly with a potentially fatal outcome. Less common neurological symptoms associated with this are Parkinsonism, focal dystonia, rubral tremor and hemichorea-hemiballismus syndrome. A 58 year old woman suddenly lost consciousness and was admitted to the emergency service. Her medical history was unremarkable, except for frequent headaches in the last year, recurrent herpes simplex skin lesions and an episode of urticaria. A computer tomography scan showed supra and infra-tentorial lesions on suggestive of cerebral toxoplasmosis. Both Toxoplasma gondii and HIV tests were positive. In the intensive care unit, antiparasitic and antiretroviral drugs were administered, and she recovered from the coma after six weeks but presented with tetraparesis, diplopia, and depression. The LCD4 count increased from 7 to 128/mm3. The neurological lesions slowly resolved over the next two months, although postural instability, rigidity, bradykinesia and predominantly left side tremor persisted. Mild improvement was achieved after the administration of levodopa. Associated Parkinsonian syndrome in HIV patients is a rare condition, explained by the location of the brain and basal ganglia lesions, and by the observed effect of Toxoplasma gondii which increases dopamine metabolism in neural cells. Early HIV diagnostic and treatment are necessary to prevent neurological disability.

Semiological characters and morphopathological-radiological correlations in duodenal malignancy.

Localized duodenal neoplasms are relatively rare entities, most often representing invasions of the duodenum by malignant masses developed in the duodeno-pancreatic region. The paper makes a more exact analysis of radiological semiology of duodenal localized tumors and seeks to determine through this analysis the role of radiological examinations in the support for the anatomopathologist. The study group included 17 cases of duodenal localized tumors, nine cases of which have been shown to be vaterian ampulloma and eight malignant tumors of the duodenum. All cases were CT (computed tomography) examined and two-thirds were performed conventional radiological examinations, prior to CT examination. Pre-operatively, all cases were evaluated endoscopically, and in three of them bile prostheses were made. CT examination protocol included a native acquisition and post-administration of intravenous contrast agent in both the arterial phase and in parenchymal and venous phase. The acquisition was made with 3 mm thin sections, subsequently coronal and sagittal plane reconstructions being made. The paper tries to establish possible correlations between the morphopathological aspect and the radio-imaging semiological characteristics of lesions.