Clinical Course Of Disease Research Articles

Geriatric Psoriasis: Evaluation of Clinical Features, Disease Course, and Treatment Modalities of Patients from a Tertiary Care Hospital

Introduction: Psoriasis is an inflammatory skin disease characterized by erythematous, scaly, and well-demarcated pruritic plaques on the extensor areas of the extremities. Psoriasis is one of the most common dermatological diseases in the geriatric population. Objective: The aim of this investigation is to evaluate the demographic data, clinical characteristics, and treatment responses of the geriatric population (≥60 with psoriasis at our hospital. Methods: In this study, we examined 221 geriatric patients diagnosed with psoriasis. We evaluated patients in 2 groups: early-onset (<60 years) and elderly-onset (≥60 years). Results: In our study, 50.7% of the patients were female, the average age was 65.9 years., and 76.4% of the patients were considered early-onset. There was no significant difference in terms of the initial body areas affected by the disease between the early-onset and elderly-onset groups; 45.7% of patients had nail psoriasis, while 27.1% had psoriatic arthritis. Nail involvement (p = 0.021) and the presence of psoriatic arthritis (p = 0.011) were more common in the early-onset group. Systemic treatment was predominantly initiated as the first treatment agent in the elderly-onset group (p = 0.014). Patients in the early-onset group received more cyclosporine treatment than the others (p=0.01). Conclusions: The geriatric population is associated with multiple comorbidities and polypharmacy, highlighting the importance of evaluating patients' medications and comorbidities when selecting a treatment agent for psoriasis.

'Diagnosis and Treatment of Hypersensitivity Pneumonitis' S2k Guideline of the German Respiratory Society and the German Society for Allergology and Clinical Immunology.

German recommendations for the diagnosis of hypersensitivity pneumonitis (HP), also known as extrinsic allergic alveolitis (EAA), were last published in 2007 [1]. The current S2k Guideline for the Diagnosis and Treatment of Hypersensitivity Pneumonitis (HP) replaces these diagnostic recommendations. They were supplemented by the aspect of chronic, and in particular of the chronic fibrotic phenotype of HP, and also, as first HP guideline, include treatment recommendations. Based on current scientific evidence and on expert opinion 12 consensus recommendations were developed. They include important statements summarizing the diagnostic process, the treatment indication and therapeutic strategies for patients with HP. Particular emphasis was placed on the different clinical courses (acute and chronic) and their characteristics (inflammatory and/or fibrotic pattern), which present differential diagnostic challenges and ultimately result in different treatment approaches. In addition to general information (diagnosis, classification, clinical disease course, epidemiology, pathogenesis, risk factors, prognosis and special aspects associated with occupational disease), the guideline will present the various clinical disease entities of HP, some in detail, others in tabular form. A major focus is on the various diagnostic steps and the different treatment approaches. The background information serves to provide a deeper understanding and inform the implementation of the recommendations. In particular, other current international guidelines for the diagnosis of HP as well as German guidelines for the diagnosis of Interstitial lung diseases (ILDs) (Leitlinien zur Diagnostik interstitieller Lungenerkrankungen) in general and for the pharmacotherapy of fibrotic ILDs were integrated and considered in this guideline [2-6].

Warrick score in rheumatoid-arthritis interstitial lung disease: a promising tool for assessing the extent and progression of lung involvement

BackgroundThe clinical manifestations and course of rheumatoid arthritis-associated interstitial lung disease (RA-ILD) exhibits considerable heterogeneity. In this study, we aimed to explore radiographic progression over a defined period, employing the Warrick score as a semi-quantitative measure in early RA-ILD, and to assess the associated risk factors for progression.MethodsRA-ILD patients underwent consecutive Warrick scoring based on initial high-resolution computed tomography (HRCT) at diagnosis and the first follow-up. Associations between Warrick scores, pulmonary function tests, and patient characteristics were analyzed. The ROC curve assessed the predictive performance of the Warrick score change rate for ILD progression, while multivariable logistic regression analysis identified risk factors for progression.ResultsSignificant correlations were found between Warrick scores and age at RA-ILD diagnosis, age at ILD diagnosis, and baseline DAS28-ESR. For the severity score, correlations were r = 0.359, r = 0.372, and r = 0.298 (p = 0.001, p < 0.001, p = 0.014, respectively); for the extent score, r = 0.364, r = 0.318, and r = 0.255 (p = 0.001, p = 0.005, p = 0.038, respectively); and for the total score, r = 0.376, r = 0.367, and r = 0.280 (p < 0.001, p = 0.001, p = 0.022, respectively). Annual changes in severity, extent, and total Warrick scores showed sensitivities of 91–97% and specificities of 98% for predicting progression over a 5-year follow-up. Cut-off values were 0.0278 for the severity score (AUC 0.954), 0.0227 for extent score (AUC 0.976), and 0.0694 for total score (AUC 0.946). Warrick severity, extent, and total scores increased significantly during follow-up. Age > 50 years (OR 7.7; p = 0.028) and baseline usual interstitial pneumonia (UIP) pattern (OR 3.1, p = 0.041) were identified as risk factors for progression.ConclusionsAdvanced age and UIP pattern were significant risk factors for progression. Warrick scoring may may help predict progression in RA-ILD, particularly through changes in severity, extent, and total scores. Due to the retrospective design and small sample size, further prospective studies with larger cohorts are needed to confirm these findings and validate Warrick scoring as a reliable marker for RA-ILD progression.

Early Motor Signs in Pathologically Verified Alzheimer's Disease and Lewy Body Disease.

The neuropathologies of Alzheimer's disease (AD) and Lewy body disease (LBD) commonly co-occur. Parkinsonism is the hallmark feature in LBD but it can be difficult to predict the presence of these co-pathologies early in the course of clinical disease. Timely diagnosis has crucial implications, especially with the advent of disease-modifying therapies. We sought to define early motor features that predict the ultimate neuropathological diagnoses of normal, AD, AD with concurrent LB pathology, and pure LB. We examined the associations between individuals' early motor features from their initial visit using the Unified Parkinson's Disease Rating Scale (UPDRS) Part III and their neuropathological diagnoses using the U.S. National Alzheimer's Coordinating Center (NACC) Database. We included data from participants with neuropathologically normal brains (n = 49), AD (n = 502), AD w/LB (n = 167), and pure LB (n = 51). Total UPDRS Part III scores were increasingly higher with purer LB pathology. Decreased facial expression at baseline differentiated those with AD w/LB pathology from those with AD. Participants having pure LB pathology more often had deficits in speech, facial expression, posture, gait, bradykinesia, and upper extremity rigidity relative to those with AD w/LB. Diminished facial expression significantly predicted the presence of LBs among those with concurrent AD pathology. Worse early speech, facial expression, posture, gait, bradykinesia, and upper extremity rigidity were suggestive of more pure LB pathology. These findings emphasize the utility of the neurological exam in the clinical assessment of persons with cognitive complaints as it can guide management.

P0349 Gasdermin D: A New Inflammatory Biomarker in Assessing Clinical Disease Activity in Crohn’s Disease

Abstract Background Crohn’s disease (CD) is a progressive, destructive, inflammatory bowel disease. Despite new treatment options in form of biologic and small-molecule therapy, a significant portion of CD patients require surgical interventions during disease course. Assessment of treatment response and disease activity is crucial in management of patients with CD. Gasdermin D (GSDMD) is a protein which belongs to the gasdermin protein family and plays a role in inflammatory cell death, pyroptosis. GSDMD activation has been shown to be related to inflammatory states in a variety of disorders, including inflammatory bowel diseases. Given the role of GSDMD in inflammation, we hypothesized that serum GSDMD levels of patients with CD may be linked to disease activity; thereby revealing a potential new inflammatory serum marker for assessing disease severity in CD patients in a non-invasive manner. Methods This prospective study was carried out at Antalya Training and Research Hospital, Turkey, between September 2022 and June 2024. 61 CD patients were enrolled in the study. Age, sex, body mass index (BMI), smoking status, time since CD diagnosis, treatment modality used at the time of enrollment, concurrent use of steroids, previous medical and surgical treatment history for CD, involvement site of CD (ileum only or ileocolonic involvement) and Harvey-Bradshaw Index (HBI) results were recorded at the day of blood sampling. Laboratory workout utilized in the study comprised WBC, ESR, CRP and GSDMD. Results Correlations between study parameters and GSDMD levels were investigated. GSDMD was found to be statistically significantly negatively correlated with age (r=-0.30, p=0.018). GSDMD was found to be statistically significantly positively correlated with CRP (r=0.26, p=0.041). Smoking was strongly correlated with increased GSDMD levels. Non-smokers (n=53) had a median serum GSDMD level of 3.03 ng/ml, compared to 8.29 in smokers (n=8) and this difference was statistically significant (p=0.001). GSDMD levels were found to be statistically significantly related to HBI (r=0.64, p=0.0001). Independent variables affecting serum GSDMD levels were investigated with multivariate logistic regression analysis. GSDMD levels were independently correlated with age (ß: -0,15, p=0.029), CRP (ß: -0,19, p=0.01) and HBI (ß: 0,95, p=0.000) of the patients. Conclusion This study is a pioneer study in revealing utility of serum GSDMD as a potential biomarker of clinical disease activity in patients with CD. Future studies will not only reveal more on the value of this biomarker as a tracker of clinical disease course, but also pave the path for a new potential therapeutic target in this burdensome disorder for both the patient and the healthcare team.

P0838 The clinical outcomes of Inflammatory Bowel Disease after liver transplantation for primary sclerosing cholangitis: a systematic review and meta-analysis

Abstract Background The clinical course of inflammatory bowel disease (IBD) associated with primary sclerosing cholangitis (PSC) after liver transplantation (LT) varies significantly across studies. This study aims to provide a comprehensive estimation of incidence rates (IR) for colorectal cancer (CRC) or high-grade dysplasia, bowel surgery, IBD exacerbation, and newly diagnosed IBD in PSC-IBD patients post-LT. Methods Online databases were searched from inception till January 2024 for studies reporting on patients who underwent LT for PSC with IBD diagnosed either before or after LT. The pooled IR were calculated with the "meta" package (R Studio [V.4.2.764] ) using a random effects model. Results Of 5,324 studies initially identified, 97 underwent full-text review, and 34 retrospective cohort studies were included in the meta-analysis. These cohorts encompassed 3,672 patients with LT for PSC, of whom 63% had concomitant IBD. The mean follow-up period across studies ranged widely from 2.46 years to 17.3 years. A pooled analysis of 21 studies reporting on colorectal neoplasia revealed an overall IR of CRC or high-grade dysplasia post-liver transplantation of 6.7 cases per 1,000 person-years (95% CI 4.3-9.0) (Figure 1A). Across 18 studies, 17.8% (n=195/1094) patients with concomitant IBD at the time of LT experienced clinical exacerbation, defined as worsening of IBD symptoms, increased inflammatory activity at endoscopy, or required optimization of IBD treatment post-transplantation. The pooled IR of clinical exacerbation was 34.1 cases per 1,000 person-years (95% CI 22.7-45.5), though heterogeneity was high (I² = 76%). Notably, meta-regression revealed a decline in IR of clinical exacerbation over time exclusively within European countries, with no observed change elsewhere. Additionally, 16 studies reported on newly diagnosed IBD in PSC patients without pre-existing IBD at LT, with pooled IR of 12.6 cases per 1,000 person-years (95% CI 9.7-15.5) (Figure 1B). The overall bowel surgery rate post-LT was 20.2 cases (95% CI 16.9-23.4) per 1,000 person-years within 23 studies. More specifically, pooled colectomy rates for neoplasia and therapy refractory IBD were 10.5 cases (95% CI 6.7-14.2) in 16 studies, and 13.8 cases (95% CI 9.4-18.3) per 1,000 person-years in 10 studies, respectively. Conclusion This meta-analysis provides a thorough assessment of IBD outcomes in PSC-IBD patients following liver transplantation, highlighting the considerable incidence rates of colorectal neoplasia and newly diagnosed IBD. Close clinical follow-up and regular endoscopic surveillance remain essential in PSC patients post-LT.

P0337 Incidentally Diagnosed Inflammatory Bowel Disease: Prevalence and Long-Term Outcomes

Abstract Background Incidentally diagnosed inflammatory bowel disease (IBD) can be found in asymptomatic individuals undergoing endoscopic examinations performed for various reasons such as colorectal cancer (CRC) screening. We aimed to characterize the prevalence of incidental IBD in individuals undergoing non-diagnostic colonoscopy and describe its clinical characteristics, disease course, and medium to long-term outcomes. Methods Retrospective cohort study including all asymptomatic adults who underwent colonoscopy for CRC screening or polyp surveillance between 2009 and 2023. Incidental IBD was considered any case with endoscopic and histologic findings suggestive of IBD. The endoscopic lesions considered were erythema, erosions, loss of vascular pattern, ulcerations, strictures, and pseudopolyps. On the other hand, the microscopic criteria were transmural inflammation, non-caseating granulomas, ulcers, crypt distortion and abscesses, cryptitis, Paneth cell metaplasia, and basal plasmacytosis. Clinical data and disease characteristics were collected. Tests for categorical variables were performed using the Fisher-Freeman-Halton Exact Test, while for quantitative variables the ANOVA and Kruskal-Wallis Test were utilized. Statistical analyses were conducted using R, with significance at p&amp;lt;0.05. Results Out of 40,573 colonoscopies, a total of 60 patients with incidental IBD were identified: 40 patients (67%) with Crohn’s disease (CD), 10 (18%) with ulcerative colitis (UC), and 10 (18%) with IBD-unclassified (IBD-U). Most CD patients presented with ileal (L1) involvement (98%) and inflammatory (B1) behavior (98%), while 60% of UC patients exhibited extensive (E3) disease. UC and IBD-U patients had lower hemoglobin values than those with CD (11.67 +/-1.97; 11,73 +/- 1.25; 13.74 +/- 1.93; respectively, p=0.019). Follow-up data was available from 27 patients (45%), after a mean follow-up of 27.9 +/- 34.7 months in CD patients, 20% (N=8) developed new-onset symptoms (diarrhea [n=3, 38%], abdominal pain [n=3, 37.5%], bloating [n=2, 25%], fatigue [n=1, 12.5%], weight loss [n=1, 13%]), while only one patient (10%) with UC reported symptoms (diarrhea and abdominal pain). A total of 5 patients (8%) initiated medical therapy, in most cases after symptom onset. Two patients (3%) with CD received oral corticosteroids, one (2%) with CD received sulfasalazine, and two patients (3%) with UC were started on oral mesalamine. No hospitalizations or surgeries were noted. Conclusion In our experience, the prevalence of incidental IBD was 0.2% with two-thirds receiving a diagnosis of ileal CD with inflammatory behavior. A low proportion of patients developed symptomatic disease (5% for CD and 2% for UC) after 2 years of follow-up and generally had milder disease course.

P0474 Five-Year Clinical Outcomes and Disease Course of Newly-Diagnosed Moderate-to-Severe Ulcerative Colitis: A Prospective Nationwide Multicentre MOSAIK Cohort Study in Korea

Abstract Background While the incidence of moderate-to-severe ulcerative colitis (UC) is rising in Asia, comprehensive data on its long-term disease course in Asian populations remain scarce. Methods The MOSAIK (MOderate-to-Severe ulcerAtive colItis in Korea) study is a prospective, multicentre, hospital-based, inception cohort that enrolled 353 patients newly diagnosed with moderate-to-severe UC between August 2014 and February 2017.1 Clinical outcomes and predictors of a disabling disease course were evaluated over 5 years of follow-up. Results Among 353 patients, the median age at UC diagnosis was 37.6 years (standard deviation, 15.2) with male predominance (58.9%, 208/353) (Table 1). Baseline disease activity was predominantly moderate (93.2%, 329/353) rather than severe (6.8%, 24/353), with median full Mayo Clinic Score (MCS) of 8 (Interquartile range [IQR], 7–9) and partial MCS of 6 (IQR, 5–7). Disease extent at diagnosis showed proctitis (10.7%, 37/353), left-sided colitis (46.7%, 162/353), extensive colitis (42.7%, 148/353). The median duration of follow-up was 4.9 years (IQR, 2.1–5.0), with 214 patients (60.6%) completing the 5 years (week 260). Treatment patterns during follow-up included: oral 5-aminosalicylates (96.3%, 340/353), topical 5-aminosalicylates (70.8%, 250/353), systemic corticosteroids (65.7%, 232/353), immunomodulators (38.5%, 136/353), biologics (20.1%, 71/353), and JAK inhibitors (1.7%, 6/353). At 5 years, the major disease outcomes were: relapse (62.9%), hospitalisation (26.9%), and proximal disease extension (29.5%). The colectomy rate was notably low (0.6%) with no mortality. A disabling disease course occurred in 171 patients (48.4%), with cumulative probabilities increasing from 41.0% at 1 year, 51.1% at 3 years, to 53.9% at 5 years. Baseline elevated white blood cell count was independently associated with a disabling disease course (adjusted hazard ratio, 1.062, 95% confidence interval, 1.004–1.123, P=0.0353). Using unsupervised clustering analysis (K-Means) of partial MCS, with missing data imputed using random forest, we identified 5 distinct disease trajectory patterns. These trajectories effectively discriminated clinical outcomes including relapse, persistent disease activity, hospitalisation, and disabling disease course (Figure 1). Conclusion In this Korean inception cohort, 48.4% of patients with moderate-to-severe UC revealed a disabling disease course over 5 years. Novel clustering analysis based on partial MCS identified 5 distinct disease trajectories that effectively predicted clinical outcomes, providing a potential framework for risk stratification. Funding This study was supported by Janssen Korea Ltd..

Features of the Clinical Course of Odontogenic Inflammatory Diseases of the Jaws and Soft Tissues in Drug Users

The objective: to analyze the features of the clinical course of odontogenic inflammatory diseases of the jaws and paramaxillary soft tissues in drug addicts. Materials and methods. 294 drug-addicted patients with odontogenic inflammatory diseases of the jaws and soft tissues were examined and treated. All patients underwent a generally accepted clinical and X-ray examinations at the first visit and during the course of treatment. Primary odontogenic inflammatory processes in the jaw were found in the patients, namely: periodontitis, periostitis and osteomyelitis. The reliability of the examination results was calculated according to Student’s criteria. Differences were considered significant at p &lt; 0.05. Results. Atypical clinical and radiological features of the course of periodontitis, periostitis, osteomyelitis, abscesses and phlegmons were found in the study participants, namely: progressive nature, significant prevalence of lesions, peculiar clinical symptoms. Unsatisfactory healing of post-extraction and postoperative wounds, very low effectiveness of the medical measures, relapse course, which should be taken into account during the treatment of this category of patients, were found in drug addicts. Conclusions. The developed schemes for the treatment of odontogenic purulent-inflammatory diseases of the soft tissues of the maxillofacial region and neck in drug-addicted persons contribute to the faster elimination of the purulent-inflammatory process (compared to traditional treatment), improve the healing of postoperative purulent wounds, and reduce the occurrence of local postoperative inflammatory complications in 3–4 times.

Autoimmune Thyroid Disease in Patients with Down Syndrome-Review.

Down syndrome develops due to the presence of supernumerary chromosome 21. This diagnosis is made in approximately 1:800 live births. The tendency to develop autoimmune disorders like idiopathic arthritis, celiac disease, diabetes mellitus type 1, vitiligo and autoimmune thyroid disease is strongly expressed in patients with Down syndrome. Autoimmune thyroid diseases consisting of Hashimoto's thyroiditis and Graves' disease are specifically prevalent in patients with Down syndrome. The aim of our study is to collect available data connecting the pathogenesis and clinical course of autoimmune thyroid diseases in patients with Down syndrome of different ages and compare them to control groups. According to published data, the incidence ratio of Hashimoto's thyroiditis diagnosis in patients with Down syndrome is elevated compared to in age-matched controls without this chromosomal aberration, similarly to Graves' disease risk, which is also increased in a group of patients with Down syndrome. What is more, both Hashimoto's thyroiditis and Graves' disease are diagnosed at an earlier age than in the healthy population and are not correlated with gender or a family history of autoimmune diseases.

Comparison of Pediatric- and Adult-Onset Mycosis Fungoides Patients in Terms of Clinical Features and Prognosis in a Large Series.

Studies comparing the clinical and prognostic differences between pediatric- and adult-onset mycosis fungoides (MF) are limited. To determine the impact of childhood-onset MF on clinical features and disease course in a large series. Consecutive MF patients seen in a single centre between 2007 and 2021 were categorized into 3 groups: (i) MF patients diagnosed in the pediatric ages (≤18 years) (pediatric group), (ii) MF patients with disease onset in the pediatric period and diagnosis in adulthood (lately diagnosed pediatric-onset group), and (iii) MF patients with disease onset in the adulthood period (>18 years) (adult-onset group). Demographics, clinical features, stage at diagnosis, and progression to advanced stages during follow-up were recorded and compared. Among 651 MF patients, 52 (8%) belonged to the pediatric group and 30 (4.6%) to the lately diagnosed pediatric-onset group. Pediatric MF presented with a combination of clinical variants in 48.1% of cases. While classical MF was the most common presentation in the 3 groups, hypopigmented (55.8%) and folliculotropic (17.3%) variants were more prevalent in the pediatric group compared to the others. Progression to the advanced stages was higher in the adult-onset MF group (12.1%) compared to the pediatric and lately diagnosed pediatric-onset MF groups (2.4%) (P = .014). This study highlights a relatively high prevalence of pediatric MF (8%) and lately diagnosed pediatric-onset MF (4.6%), often presenting with multiple and non-classical clinical variants. The higher rate of progression to advanced stages in the adult-onset group supports the relatively benign nature of MF in the pediatric-onset group.

Wound formation in patients with Rutherford category IV disease after endovascular therapy: rates and risk factors

BackgroundLower limb peripheral artery disease classified as Rutherford category IV, is characterized by lower limb ischemic pain both during exertion and at rest. This disease has an unclear course. We aimed to evaluate outcome predictors in this patient group after endovascular therapy. This single-center, retrospective, observational study included 234 consecutive patients (264 limbs), between April 2007 and December 2020. We investigated the disease clinical course after endovascular therapy. The primary endpoint was the wound formation rate 3 years after endovascular therapy.ResultsThe mean observation period was 48.2 ± 8.9 months. The patients (61.9% male; mean age, 76 ± 10 years) presented with diabetes (64.1%), and received hemodialysis with chronic kidney disease (35.0%) and ambulatory treatment (85.0%). The average ankle-brachial index before endovascular therapy was 0.69 ± 0.23. Skin perfusion pressure on the dorsal and plantar sides was 38 ± 13 mmHg and 36 ± 12 mmHg, respectively. The wound incidence rates at 1, 2, and 3 years after endovascular therapy were 8.3%, 11.4%, and 14.4%, respectively. Multivariate analysis revealed the following factors associated with wound formation: P2 in inframalleolar/pedal disease category in the Global Limb Anatomical Staging System (hazard ratio: 1.73, 95% confidence interval: 1.22–2.83, P = 0.01), non-ambulatory status (hazard ratio: 1.09, 95% confidence interval: 1.11–1.36, P = 0.02), intervention up to infrapopliteal lesion (hazard ratio: 1.55, 95% confidence interval: 1.17–2.46, P = 0.03), and patient with chronic kidney disease on hemodialysis (hazard ratio: 1.61, 95% confidence interval: 1.32–2.18, P = 0.03).ConclusionsThe 3-year incidence of wound onset in this study was 14.4%. Factors associated with this outcome included P2 in the Global Limb Anatomical Staging System, non-ambulatory status, intervention up to infrapopliteal lesion, and patient with chronic kidney disease on hemodialysis.

DIAGNOSTIC AND CLINICAL ASPECTS OF NORMOCALCEMIC PARATHYROID HYPERPLASIA AND NON-RENAL SECONDARY HYPERPARATHYROIDISM IN EXPOSED AND NON;EXPOSED TO IONIZING RADIATION INDIVIDUALS (LITERATURE REVIEW AND OWN RESEARCH DATA).

Parathyroids are the key regulators of calcium-phosphorus metabolism. By means of parathyroid hormone they respond to any changes in the serum level of calcium and phosphorus ions and determine the integrity of skeleton, affecting almost all systems and cells where calcium and phosphorus are involved in metabolism and/or signaling.Disorders of parathyroid function are associated with significant complications accompanying secondary hyperparathyroidism. Unfortunately, most medical practitioners and scientists underestimate the impact of parathyroid hormone excess and vitamin D deficiency. Usually, medical practitioners do not prescribe the assay of serum level of parathyroid hormone, which should be done as a screening test, so the disease remains unnoticed for a long time.In fact, the incidence of parathyroid disorders, namely hyperparathyroidism, is almost equal to the incidence of thyroid diseases and is more than 10 % for the entire population, depending on the observation groups (gender, age, exposure to ionizing radiation), as demonstrated in our previous studies. Thus, according to our data, the incidence of parathyroid hyperplasia is 24.3 % (with 50-70 % in children and adolescents) in the state of euparathyroidism, and incidence of normocalcemic hyperparathyroidism is up to 14.3 % in the average population of Ukraine. Today, we see much confusion in publications and available recommendations regarding diagnosis, pathogenesis, and course of parathyroid disease. It is often not taken into account that the strain in calcium-phosphorus system usually occurs due to the lack/deficiency of vitamin D, which is very common worldwide. Often a simple administration of vitamin D in optimal and individual dosages solves (prevents) the problem of hyperparathyroidism or reduces the aggressiveness of its course. Review of data from literary sources and results of own research on pathogenetic patterns and clinical course of parathyroid diseases was carried out, and it was determined that the lack/deficiency of vitamin D is the most common ground of development of hyperparathyroidism, which affects the formation of parathyroid hyperplasia or adenoma, and the process itself is of a gradual, staged clinical development.

Clinical Features and Disease Course of Hydroa Vacciniforme: A Systematic Review

Clinical Features and Disease Course of Hydroa Vacciniforme: A Systematic Review

A Novel MYH14 Variant Presenting as a New Phenotype of MYH14-Associated Neuromuscular Disorders-Clinicohistologic Findings and Review of the Literature.

Pathogenic variants in the nonmuscle myosin, MYH14, have been associated with several pathologic conditions including a complex phenotype with peripheral neuropathy, myopathy, hoarseness, and hearing loss. Since its first description in a large Korean kindred, this rare neuromuscular disorder has further been characterized in 1 American and 1 Canadian pedigree. Here, we describe a German patient with atypical MYH14-related neuromuscular disorder. The clinical phenotype included signs of a distal myopathy with early respiratory involvement and a prominent hoarseness and peripheral neuropathy. In contrast to previous reports, no relevant deafness was identified. Muscle biopsy indicated a vacuolated myopathy with excessive autophagy, whereas histology of the sural nerve showed signs of a mixed axonal-demyelinating neuropathy. Next-generation sequencing revealed a loss-of-function variant not identified so far in the MYH14 gene (c.4510del, p.[Arg1504Glyfs*10]). Because of rapid disease progression with respiratory failure, the patient died at the age of 52. We present a novel MYH14 variant resulting in a severe and rapidly progressive MYH14-associated phenotype with predominantly distal myopathy, early respiratory failure, dysphagia, hoarseness, and peripheral neuropathy, without hearing loss. This case expands the clinical spectrum of MYH14-related neuromuscular disorders by providing a new clinical phenotype and disease course and histopathologic features.

Spontaneous regression of head and neck squamous cell carcinoma: a systematic review and meta-analysis of case reports.

A systematic review of case reports in spontaneous regression of head and neck squamous cell carcinoma (SCC) was carried out to investigate the pattern and characteristics of this phenomenon. A systematic search of case studies of spontaneously regressed head and neck SCC was carried out in Ovid Embase, Ovid Medline and Pubmed. Methodological quality was assessed by ascertainment of diagnosis and overall details of reports. Outcomes included patient demographics, head and neck SCC characteristics and clinical course of disease. A total of 8 cases were included and 50 per cent (n = 4) of the reported cases were SCC of the vocal folds. All cases received a surgical biopsy and three cases had possible febrile episodes preceding regression. The mean length of time to regression was 4.3 months. Spontaneous regression in head and neck SCC is likely to be under-reported. A better understanding of how the host immune system can instigate an antitumour response will shed light on the development of novel treatments.

Spinal cord conduction disorders among neurological complications of degenerative dystrophic diseases of the cervical spine

Neurological complications of degenerative dystrophic diseases of the cervical spine depend on the involvement of functionally significant structures of the cervical spine in the pathological process. Current diagnostic techniques do not always accurately diagnose the involvement of these structures in the pathological process. This fact requires constant study and development of the novel methods for diagnosing degenerative dystrophic diseases of the cervical spine. In this regard, the purpose of the study is to identify various clinical and pathogenetic forms of the clinical course of degenerative dystrophic diseases of the cervical spine with the presence of manifestations of spinal cord conduction disorders based on the study of clinical symptoms and neuroimaging data of the cervical spine. The work is based on the study of clinical manifestations and magnetic resonance imaging of the cervical spine in the preoperative period in 457 patients with degenerative diseases of the cervical spine aged from 22 to 64 years. Among the patients, women predominated: 271 cases (59.3 %). Men made up 40.7 % (186 cases). Pathology in the form of a herniated intervertebral disc was detected at the levels of CV–CVI in 192 cases (42.0 %), CVI–CVII in 179 cases (39.2 %), CIV–CV in 45 cases (9.8 %), and CVII — ThI in 41 cases (9.0 %). Disc herniation at two levels simultaneously was diagnosed in 6.7 % (31 cases). The complex of patient examination included general clinical, neurological, and neuroimaging studies using MRI of the cervical spine. Damage to one of the functionally significant anatomical structures of the cervical spine occurred in 48.4 % (221 cases). Simultaneous involvement of two or more of these structures in the pathological process was diagnosed in 51.6 % (236 cases). Comparison of the neurological symptoms of the disease with magnetic resonance imaging data of the cervical spine makes it possible to diagnose five forms of degenerative dystrophic diseases of the cervical spine: 46.8 % — radicular, 1.5 % — myelic, 38.1 % — combined myelo-radicular, 7.5 % — combined radicular angiocerebral, and 6.1 % — combined myelo-radicular and angiocerebral. Notably, symptoms of spinal cord conduction disorders were identified in myelic, combined myelo-radicular, and combined myelo-radicular and angiocerebral forms. Due to the possible presence of a combination of spinal cord conduction disorders with symptoms of compression or irritation of the spinal cord roots and vertebral artery among neurological complications, the clinical manifestations of the disease acquire a significant polymorphism of symptoms, which is always associated with difficulties in diagnosing degenerative dystrophic diseases of the cervical spine. The identified clinical and radiological criteria for myelic, combined myelo-radicular, and combined myelo-radicular and angiocerebral forms of the course of this pathology of the cervical spine are decisive in the differential diagnosis with any other pathology of the spinal cord and for determining the feasibility of individual medical rehabilitation measures.

Psycho-Neuro-EndocrinE-Immunology Therapy of Cancer, Autoimmunity, Geriatric Disorders, Covid-19, and Hypertension.

Despite the great number of experimental investigations in the area of psycho-neuro-endocrine-immunology showing that endocrine, nervous, and immune systems cannot be in vivo physiologically separated, the diagnosis and therapies of the pathologies of these three functional biological systems continue to be separately performed from a clinical practice point of view. The separation between experimental and clinical medicine became dramatic after the discovery of more than 10 human molecules provided by anti-inflammatory and antitumor activity, completely devoid of any toxicity, which may be subdivided into three fundamental classes, consisting of the pineal indole, beta-carboline, and methoxy-kynuramine hormones. Moreover, human systemic diseases, including cancer, autoimmunity, and cardiovascular pathologies, despite their different pathogenesis and symptomatology, are commonly characterized by a progressive decline in the endogenous production of pineal hormones, endocannabinoids, and Ang 1-7, with a consequent inflammatory status and diminished natural resistance against cancer. Then the evaluation of the functional status of the pineal gland, the endocannabinoid system, and ACE2-Ang 1-7 axis should have to be included within the laboratory analyses for the systemic diseases. Finally, the correction of cancer- and autoimmunity-related neuroimmune and neuroendocrine alterations could influence the clinical course of systemic diseases. In fact, preliminary clinical results would demonstrate that the neuroimmune regimen with pineal hormones, cannabinoids, and Ang 1-7 may allow clinical benefits also in patients affected by systemic pathologies, including cancer, autoimmunity, and cardiovascular diseases, who did not respond to the standard therapies.

Obstructive sleep apnea in patients with fibrotic interstitial lung disease (non-idiopathic pulmonary fibrosis): what should be offered?

The frequency of obstructive sleep apnea (OSA) in patients with idiopathic pulmonary fibrosis (IPF) is high. The clinical course of non-IPF interstitial lung disease (ILD) can be similar to that of IPF. We sought to assess the frequency and predictors of OSA in patients with non-IPF fibrotic ILD, as well as the impact of positive airway pressure (PAP) therapy on the quality of life of such patients. This was a prospective study in which non-IPF fibrotic ILD patients underwent a home sleep apnea test. The patients with and without OSA were compared, and a multivariate logistic regression model was used to identify independent predictors of OSA. At 3 months after initiation of PAP therapy, we evaluated the participating patients for respiratory events, nocturnal hypoxemia, and changes in quality of life. Of a total of 50 patients, 50% were male, and 76% were diagnosed with OSA. The mean age was 67.8 ± 8.3 years. The patients with OSA had significantly lower TLC (p = 0.033) and awake SpO2 (p = 0.023) than did those without OSA. In the multivariate logistic regression model, SpO2 (OR = 0.46; p = 0.016) and TLC (OR = 0.95; p = 0.026) remained significantly associated with OSA risk. A total of 12 patients received PAP therapy. At 3 months after initiation of PAP therapy, 91.7% were well controlled, Epworth Sleepiness Scale scores decreased significantly (p = 0.006), and emotional well-being tended to improve (p = 0.068). PAP therapy corrected nocturnal hypoxemia in all patients. We found a high frequency of OSA in patients with non-IPF fibrotic ILD. A low TLC was an independent predictor of a higher risk of OSA. PAP therapy can correct nocturnal hypoxemia. There should be a low threshold for suspicion of OSA and initiation of PAP therapy in patients with non-IPF fibrotic ILD.

INVESTIGATING ETIOPATHOGENETIC FACTORS OF LEFT VENTRICULAR REMODELING AS THE UNDERLYING MECHANISM OF ISCHEMIC MITRAL REGURGITATION

The purpose of this study is to determine the etiopathogenetic features of the development of ischemic mitral regurgitation in patients with coronary heart disease by investigating the mechanisms of left ventricular remodeling. Materials and Methods. The study included 140 patients (99 male, 41 female) with ischemic mitral regurgitation who underwent myocardial revascularization and mitral valve reconstruction (either repair or replacement). The average age of participants was 65.9 ± 4.0 years, and all had a history of coronary heart disease and ischemic mitral regurgitation. All patients in the sample underwent operations for myocardial revascularization and mitral valve reconstruction (replacement). The data analyzed were obtained from primary medical documentation, including medical histories, patient medical charts, findings of physical and clinical examinations, laboratory and instrumental evaluations, as well as echocardiography findings. The data obtain were processed statistically. Results. This article presents an analysis of the etiopathogenetic features associated with the development of ischemic mitral regurgitation under the clinical course of coronary heart disease. The study examined several etiopathogenetic factors, including the presence of arterial hypertension, left ventricular hypertrophy, and the type of left ventricle remodeling. It was found that 39.1% of patients in the experimental group and 40.8% in the control group had hypertension (p ≥ 0.05). The most prevalent condition was grade II arterial hypertension, which occurred in 52.3% of the experimental group and 60.6% of the control group (p ≥ 0.05). When analyzing the occurrence rate of left ventricular hypertrophy, mild hypertrophy was significantly more common in the experimental group (44.9%) compared to the control group (26.8%) (p = 0.03, χ² = 4.27). Moderate left ventricular hypertrophy was found to be significantly higher in the control group (45.1%) compared to the experimental group (27.5%) (p = 0.04, χ² = 3.92). Eccentric hypertrophy of the left ventricle was prevalent in both groups, with values of 79.7% in the experimental group and 80.3% in the control group. In contrast, the frequency of concentric hypertrophy was higher in the control group (8.4%) compared to the experimental group (2.9%) (p ≥ 0.05). Conclusions: The study has established that the incidence of ischemic heart disease and ischemic mitral regurgitation is significantly associated with sex, with men being affected more frequently than women. Analysis of arterial hypertension revealed that 39.1% of patients in the experimental group and 40.8% in the control group were affected, with grade II arterial hypertension being the most common. Moderate and severe left ventricular hypertrophy, as well as concentric remodeling of the left ventricle, were characteristic of patients who underwent mitral valve replacement. These findings underscore the importance of left ventricular remodeling in determining the appropriate surgical treatment for mitral valve conditions.