Clinical course of psoriatic arthritis treated with biologics delineated with ultrasonography.

Psoriatic arthritis (PsA) is a chronic, inflammatory articular disease regarded as a specific subtype of psoriasis. Long-term assessment for PsA using ultrasonography has not yet been investigated. The present study was conducted to delineate the changes in articular lesions after the initiation of biologics using ultrasonography, and to provide the evidence of the utility of ultrasonography in long-term follow-up of PsA patients. We retrospectively recruited 17 Japanese PsA patients treated with biologics who met the classification criteria for psoriatic arthritis. Ultrasonographic images were recorded using a high-frequency linear 18 MHz probe through Doppler- and B-modes. Before the treatment with biologics, all examined patients (100%) had enthesitis and extensor tendinitis, while only six patients (35.3%) had loss of the fibrillar pattern of the tendon (LFP). There were significant changes over time in the numerical rating scale score for pain, and in the degree of ultrasonographic findings, including enthesitis, extensor tendinitis, and LFP. Also, there were significant changes over time between these ultrasonographic findings. The study identified the improvement course for a specific PsA lesion after the initiation of biologics. The improvement courses in enthesitis, extensor tendinitis, and LFP were found to differ from each other. These results may contribute to deeper understanding of the pathogenesis of PsA.

Prevalence and clinical profiles of primary sclerosing cholangitis in China: Data from electronic medical records and systematic literature retrieval.

BACKGROUND & AIMS: Epidemiology of primary sclerosing cholangitis (PSC) is lacking in China. We aimed to estimate the period prevalence and depict the clinical features of PSC in China. METHODS: We identified and included PSC cases between 2000 and 2023 from two sources: electronic medical records (EMR) and systematical literature retrieval (SLR). The period prevalence of PSC was estimated by the multiplier method. Rate ratios (RRs) for PSC prevalence in relation to macroeconomic indicators were calculated by the negative binomial regression model. RESULTS: A total of 1358 PSC cases were retrieved from 299 hospitals (162 from EMR and 1196 from SLR). Males accounted for 55.7 % of the PSC cases and 25.7 % had concomitant inflammatory bowel disease (IBD). The estimated period prevalence of PSC from 2000 to 2023 was 2.36 (95 % CI: 1.82, 3.34) per 100,000. Males had a numerically higher PSC prevalence than females (2.56, 95 % CI: 1.97, 3.63 vs. 2.14, 95 % CI: 1.65, 3.04 per 100,000). The highest prevalence of PSC was in East China at 4.87 (95 % CI: 3.44, 7.18) per 100,000, followed by North China at 2.94 (95 % CI: 2.33, 3.74) per 100,000, and the lowest in South China at 0.92 (95 % CI: 0.66, 1.30) per 100,000. Regional per capita GDP (RR 1.65, 95 % CI: 1.03, 2.65) and healthcare expenditure (RR 1.94, 95 % CI: 1.13, 3.38) were identified to be associated with PSC prevalence. CONCLUSION: Our study showed the estimated PSC prevalence varied within China, but was generally lower than that in Western countries.

A decade with anomic primary progressive aphasia.

Some patients with primary progressive aphasia (PPA) demonstrate only anomia. The lack of longitudinal observations of anomic PPA precluded us from determining whether progressive anomic aphasia was simply an early stage of semantic or logopenic variants, or a relatively independent variant. Herein, we report the 10-year clinical course of a patient with PPA who presented with pure anomic aphasia for 9 years. He is a right-handed man with anomia, who noticed word-finding difficulty at age 73. He was admitted to the hospital at age 77. On admission, the patient showed pure anomic aphasia with preserved other language function. Episodic memory and visuospatial function were preserved. Magnetic resonance imaging (MRI) revealed left temporal lobe atrophy. At 82 years of age, the patient presented with pure anomic aphasia. At 83 years old, he showed mild impairment in word comprehension and semantic memory, in addition to anomia. MRI demonstrated further atrophy in the bilateral anterior temporal lobes, predominantly on the left side. This case suggests the possibility of slowly progressive, late-onset anomic PPA, which could be differentiated from the early stage of semantic or logopenic variants.

Features of the clinical course of Autoimmune Encephalitis Associated with various antibodies.

Despite the increasing incidence of autoimmune encephalitis and the incomplete recovery observed in patients post-affliction, the issue of timely diagnosis remains unresolved. The primary objective of this study is identification the distinctive clinical presentation features evaluation the management strategies, and assess the outcomes of the disease in patients with various forms of autoimmune encephalitis. The research aims to contribute in a better understanding of the disease progression and facilitate the selection of optimal therapeutic interventions. A retrospective observational study enrolled 68 patients aged 18 years and older with verified autoimmune encephalitis who underwent treatment in state hospitals in Sofia, Bulgaria, from the beginning of 2014 to the end of 2022. The number of patients with pathology linked to antibodies against glycine receptors (Gly-R) was half as much, with 32 and 17 patients, respectively. The primary manifestations of autoimmune encephalitis included cognitive impairments observed in 51 patients, seizures occurring in 44 patients, and mood disorders observed in 22 patients. While the findings of imaging studies were nonspecific, hospitalizations for patients with this pathology, especially those with antibodies to CASPR2 and DPPX, were prolonged (114 and 232 days, respectively). In the vast majority of cases, incomplete recovery with residual symptoms was noted. Among the diverse forms of autoimmune encephalitis, the most prevalent is NMDA-R. Cognitive impairments predominate in the autoimmune encephalitis clinical presentation. Prolonged hospitalization periods and incomplete recovery of patients are characteristic features of autoimmune encephalitis, despite combined therapy involving intravenous administration of methylprednisolone and immunoglobulins.

A case study on anthrax in an eight-month old infant at Kyrgyz Republic.

Anthrax remains a threat, especially in countries like Kyrgyzstan with developed livestock farming. Despite preventive efforts, sporadic outbreaks endure on an annual basis, transmitted from infected animals to humans. Here, we report a severe anthrax case in an 8-month-old child known to be caused when a sick calf was slaughtered in the neighborhood without proper protocols, resulting in intra-family infection. This underscores the importance of swift diagnosis, treatment, preventive measures, and awareness of zoonotic infections, animal vaccination and adherence to sanitary and veterinary protocols.

Sex-Related Differences in the Phenotype and Course of Inflammatory Bowel Disease: SEXEII Study of ENEIDA.

BACKGROUND & AIMS: The impact of patient sex on the presentation of inflammatory bowel disease (IBD) has been poorly evaluated. Our aims were to assess potential disparities in IBD phenotype and progression between sexes. METHODS: We performed an observational multicenter study that included patients with Crohn's disease (CD) or ulcerative colitis from the Spanish ENEIDA registry. Data extraction was conducted in July 2021. RESULTS: A total of 51,595 patients with IBD were included, 52% were males and 25,947 had CD. The median follow-up period after diagnosis was 9 years in males and 10 years in females. In CD, female sex was an independent risk factor for medium disease onset (age, 17-40 y) (relative risk ratio, 1.45; 95% CI, 1.31-1.62), later disease onset (age, >40 y) (relative risk ratio, 1.55; 95% CI, 1.38-1.73), exclusive colonic involvement (odds ratio, 1.24; 95% CI, 1.14-1.34), inflammatory behavior (odds ratio, 1.14; 95% CI, 1.07-1.21), and extraintestinal manifestations (odds ratio, 1.48; 95% CI, 1.38-1.59). However, female sex was a protective factor for upper gastrointestinal involvement (odds ratio, 0.84; 95% CI, 0.79-0.90), penetrating behavior (odds ratio, 0.76; 95% CI, 0.70-0.82), perianal disease (odds ratio, 0.77; 95% CI, 0.71-0.82), and complications (odds ratio, 0.73; 95% CI, 0.66-0.80). In ulcerative colitis, female sex was an independent risk factor for extraintestinal manifestations (odds ratio, 1.48; 95% CI, 1.26-1.61). However, female sex was an independent protective factor for disease onset from age 40 onward (relative risk ratio, 0.76; 95% CI, 0.66-0.87), left-sided colonic involvement (relative risk ratio, 0.72; 95% CI, 0.67-0.78), extensive colonic involvement (relative risk ratio, 0.59; 95% CI, 0.55-0.64), and abdominal surgery (odds ratio, 0.78; 95% CI, 0.69-0.88). CONCLUSIONS: There is sexual dimorphism in IBD. The patient's sex should be taken into account in the clinical management of the disease.

Prior Appendectomy Is Associated With a Milder Clinical Course in Crohn's Disease: A Nationwide Population-based Cohort Study.

BACKGROUND: Appendectomy may affect the clinical course of Crohn's disease (CD), but rigorous evidence is sparse and contradicting. The aim of this study was to examine the association between appendectomy and the clinical course of CD. METHODS: All patients diagnosed with CD in Denmark in the period from 1977 to 2017 were identified from the Danish National Patient Registry. Patients with appendectomy were matched with up to 10 comparators with CD and no appendectomy; and rates of CD-related hospital admissions were compared between CD patients with and without appendectomy using incidence rate ratios (IRRs). We used stratified Cox regression analysis to calculate adjusted hazard ratios (aHRs) of initiating treatment with biologics or undergoing intestinal resections. RESULTS: In all, 21 189 CD patients (1936 with appendectomy and 19 253 without) were identified and followed for a median of 13.6 years. Crohn's disease patients who had undergone appendectomy experienced a lower rate of CD-related hospital admissions (appendectomy before CD: IRR = 0.83; 95% confidence interval [CI], 0.81-0.85; appendectomy after CD: IRR = 0.85; 95% CI, 0.81-0.88) compared with CD patients without appendectomy. For patients with appendectomy before CD diagnosis, the rate of initiating biologics was lower compared with CD patients with no appendectomy (aHR1-<5 years = 0.61; 95% CI, 0.46-0.81; aHR5-<10 years 0.47; 95% CI, 0.33-0.66; aHR10-20 years = 0.61; 95% CI, 0.47-0.79), as was the risk of undergoing colorectal resections (aHR1-<5 years = 0.94; 95% CI, 0.77-1.15; aHR5-<10 years 0.63; 95% CI, 0.47-0.85; aHR10-20 years = 0.75; 95% CI, 0.54-1.04). Rates of small bowel resections were comparable for CD patients with or without appendectomy prior to CD. Appendectomy performed after CD did not influence the rate of initiating treatment with biologics or undergoing intestinal resections. CONCLUSION: The clinical course of CD is milder for those who have previously undergone appendectomy.

In a large nationwide cohort study, patients with Crohn's disease who underwent appendectomy had a milder clinical course than those without appendectomy.

Usefulness of the Phalen Test and the Tinel Sign in the Prognosis and the Impact on Quality of Life of Patients with Carpal Tunnel Syndrome Undergoing Classical Open Carpal Tunnel Release.

Objective: To evaluate the usefulness of the Phalen test and the Tinel sign in the prognosis and the impact on quality of life in the clinical course of patients with carpal tunnel syndrome undergoing surgical treatment through the traditional open approach. Methods: The present is a cohort study on prognosis. We included 115 patients with high probability of receiving a clinical diagnosis of carpal tunnel syndrome with indication for surgical treatment. All patients underwent the Phalen test and Tinel sign and answered the Boston Carpal Tunnel Questionnaire before and after the surgical treatment. Results: The estimates for the probability of the time until remission of the Phalen test at 2, 4 and 16 weeks postoperatively were of 3.54% (95% confidence interval [95%CI]: 1.16%-8.17%), 0.88% (95%CI: 0.08%-4.38%) and 0.88% (95%CI: 0.08% to 4.38%) respectively, and, for the Tinel sign, they were of 12.39% (95%CI: 7.13%-19.18% ), 4.42% (95%CI : 1.65%-9.36%) and 2.65% (95%CI : 0.70%-6.94%) respectively. There was a reduction in the postoperative score on the Boston Carpal Tunnel Questionnaire of 1.8 points for symptom severity ( p < 0.001) and of 1.6 points for functional status ( p < 0.001). Conclusion: Phalen test remission was earlier than that of the Tinel sign, but, when performed as of the second postoperative week, they were prognostic factors favorable to the clinical course, with improved quality of life.

Exploring the Interplay between Complex Post-Traumatic Stress Disorder and Obsessive-Compulsive Disorder Severity: Implications for Clinical Practice.

Background and Objectives: Traumatic events adversely affect the clinical course of obsessive-compulsive disorder (OCD). Our study explores the correlation between prolonged interpersonal trauma and the severity of symptoms related to OCD and anxiety disorders. Materials and Methods: The study follows a cross-sectional and observational design, employing the International Trauma Questionnaire (ITQ) to examine areas linked to interpersonal trauma, the Hamilton Anxiety Rating Scale (HAM-A), and the Yale-Brown Obsessive-Compulsive Scale (Y-BOCS) to assess anxious and obsessive-compulsive symptoms, respectively. Descriptive analysis, analysis of variance (ANOVA), and logistic regression analyses were conducted. Results: We recruited 107 OCD-diagnosed patients, categorizing them into subgroups based on the presence or absence of complex post-traumatic stress disorder (cPTSD). The ANOVA revealed statistically significant differences between the two groups in the onset age of OCD (p = 0.083), psychiatric familial history (p = 0.023), HAM-A, and Y-BOCS (p < 0.0001). Logistic regression indicated a statistically significant association between the presence of cPTSD and Y-BOCS scores (p < 0.0001). Conclusions: The coexistence of cPTSD in OCD exacerbates obsessive-compulsive symptoms and increases the burden of anxiety. Further advancements in this field are crucial for mitigating the impact of early trauma on the trajectory of OCD and associated anxious symptoms.

Multisystemic Inflammation Influences Prognosis in Fulminant Lymphocytic Myocarditis.

BACKGROUND: Multisystem inflammatory syndrome (MIS) is a hyperinflammatory shock associated with cardiac dysfunction and severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. However, there are no reports on using MIS criteria, such as multisystemic inflammation (MSI) in fulminant myocarditis, without SARS-CoV-2 infection. This study investigated the differences in clinical characteristics and course between patients with fulminant lymphocytic myocarditis (FLM) plus MSI and those without MSI.Methods and Results: This multicenter retrospective cohort study included 273 patients with FLM registered in the JROAD-DPC database between April 2014 and March 2017. We evaluated the presence of MSI using criteria modified from previously reported MIS criteria and compared the characteristics and risk of mortality or heart transplantation between FLM patients with MSI and without MSI. Of the 273 patients with FLM, 107 (39%) were diagnosed with MSI. The MSI group was younger (44 vs. 57 years; P<0.0001) and had more females (50% vs. 36%; P=0.0236), a higher incidence of pericardial effusion (58% vs. 40%; P=0.0073), and a lower 90-day mortality rate (19% vs. 33%; P=0.0185) than the non-MSI group. The risk of mortality at 90 days was lower in FLM patients aged <50 years with MSI aged <50 years than in those without MSI (P=0.0463). CONCLUSIONS: These results suggest that MSI may influence the prognosis of FLM, especially in patients aged <50 years.

Navigating Uncharted Waters: Comparative Analysis of Clinical Progression and Outcomes in Vestibular Schwannoma Patients with Papilledema and without Hydrocephalus, Versus Those without Papilledema and Hydrocephalus: A Comprehensive Institutional Insight.

BACKGROUND: Papilledema's association with hydrocephalus (HCP)-linked larger vestibular schwannoma (VS) is established but cases lacking concurrent HCP require further investigation. METHODS: This retrospective comparative observational study, conducted from July 2018 to July 2023, examined 120 VS patients undergoing surgery. Patients were categorized into Group 1 (papilledema without HCP) and Group 2 (no papilledema or HCP), with comprehensive data analyzed. RESULTS: In this study, Group 1 (14 patients with papilledema) and Group 2 (106 patients without papilledema or HCP) were compared. Group 1 was younger (mean age 27.21 ± 11.73 years) than Group 2 (mean age 54.66 ± 11.44 years). Both groups had similar symptom durations and tumor detection times. Group 1 had increased vascularity (P = 0.001), elevated cisterna magna protein levels (P = 0.001), and a higher incidence of neurofibromatosis 2 (P = 0.003). They also experienced longer surgeries (P = 0.001) and more blood loss (P = 0.001), leading to extended postoperative complications. Group 2 showed improved postsurgery visual outcomes (P = 0.001), better Glasgow Outcome Scores (P = 0.001), enhanced facial nerve preservation (P = 0.002), and improved hearing on follow-up (P = 0.003). Logistic regression analysis highlighted prolonged surgery duration (P = 0.057) and papilledema (P = 0.0001) as significant factors influencing visual improvement. CONCLUSIONS: Patients with VS require preoperative fundoscopy evaluation due to potential visual loss and papilledema, even without HCP. Early treatment initiation enhances visual and hearing outcomes. Meticulous surgery is vital given the lesion's hypervascular nature and adherence to surrounding structures. Preoperative embolization may aid in preserving neurovascular structures. In developing countries with higher blindness rates, judicious noncontrast computed tomography brain evaluation is crucial for timely detection and treatment initiation of lesions like VS.

Prevalence and Clinical Impact of Immune-Mediated Inflammatory Diseases in Patients with Inflammatory Bowel Disease: Results from a Large Retrospective Observational Study.

(1) Background: Inflammatory bowel diseases (IBDs) are chronic inflammatory disorders involving innate and adaptive immune responses. Despite primarily affecting the gut, recent insights highlight systemic implications, expanding our understanding beyond intestinal boundaries. (2) Methods: This retrospective multicentric study explored the association of IBD and immune-mediated inflammatory diseases (IMIDs) and the impact of concurrent IMIDs on the course of IBD. Clinical data were collected from consecutive medical records of patients with IBD. For assessing the impact of concurrent IMIDs, a control group of IBD patients without associated IMIDs was considered. (3) Results: Of 6589 IBD patients, 6.8% exhibited concomitant IMIDs. Notably, 79.8% of these patients had an aggressive disease course. Psoriasis, atopic dermatitis, and type 1 diabetes mellitus prevalence were lower in the IBD population than in the general population. Conversely, multiple sclerosis, primary sclerosing cholangitis, and pyoderma gangrenosum were more prevalent in IBD patients. Among the patients with a concomitant IMID, 79.8% had an aggressive disease course vs. 8.1% in the control group (p < 0.001). (4) Conclusions: This study underscores the frequency of IMIDs in IBD patients and their association with a more aggressive disease course. The recognition of concurrent IMIDs is crucial for comprehensive patient management, influencing therapeutic decisions and potentially improving outcomes.

HBV reactivation and clinical resolution in an isolated anti-HBc-positive patient during immune checkpoint inhibition.

BACKGROUND: Immune checkpoint inhibitor (ICI)-related liver injury is a growing concern as ICIs are increasingly used in cancer treatment regimens. Interestingly, ICIs have exhibited antiviral effects among patients with chronic hepatitis B virus (HBV). However, the underlying mechanisms remain unclear, and clinical data on patients with previous HBV infection/exposure and isolated anti-HBV core antibodies (IAHBcs) are lacking. METHODS: We report a case illustrating the dual effects of ICIs in a patient experiencing panlobular hepatitis and concurrent HBV reactivation. FINDINGS: A 68-year-old male patient positive for IAHBcs was admitted with panlobular hepatitis and HBV reactivation after receiving systemic chemotherapy (several months before admission) and ICI treatment (4 weeks before admission) subsequent to metastatic primary lung cancer (NSCLC stage IV). This was followed by a rapid and significant decrease of HBV DNA viral load before and during antiviral treatment. CONCLUSIONS: This unique case sheds light on the dynamics of ICI therapy in IAHBc-positive patients experiencing HBV reactivation during chemotherapy and underscores the dual impact of ICIs. Moreover, it emphasizes the need for assessment of HBV serology and prophylaxis in IAHBc-positive patients undergoing chemotherapy and ICI treatment. FUNDING: R.T.C. was supported by the MGH Research Scholars Program.

Factors influencing the development of infantile traumatic brain injury with a biphasic clinical course and late reduced diffusion.

BACKGROUND: Infantile traumatic brain injury (TBI) with a biphasic clinical course and late reduced diffusion (TBIRD) has been reported as a type of TBI. However, it remains uncertain which pediatric patients with TBI develop TBIRD. METHODS: Patients with TBI who were admitted to our hospital and underwent magnetic resonance imaging (MRI) between December 2006 and October 2022 were included in this study. A diagnosis of TBIRD was made in patients with or suspected TBI, with initial symptoms being convulsions or disturbance of consciousness and late-onset subcortical reduced diffusion, the so-called bright tree appearance. Clinical features, neuroimaging (computed tomography (CT) and MRI) findings, laboratory data, and Tada score were retrospectively compared between TBIRD and non-TBIRD patients. Neurological prognosis was assessed using the Pediatric Cerebral Performance Category scale. RESULTS: Of 21 patients who met the inclusion criteria, a diagnosis of TBIRD was made in 7 patients (median age: 8 months). The factors contributing to TBIRD development were seizures lasting over 30 min as the initial symptom (5/7 in TBIRD vs. 0/14 in non-TBIRD), tracheal intubation during initial treatment (5/7 vs. 0/14), and brain parenchymal lesions on CT (3/7 vs. 0/14), suggesting that severe TBI may progress to TBIRD. The Tada score was more positive in patients with TBIRD (6/7) than in those without (0/14). CONCLUSIONS: It is important to monitor infant patients with severe TBI for the development of TBIRD. The Tada score can be a useful tool for TBIRD prediction.

Hypermagnesemia is associated with poor outcomes during hospitalization.

BACKGROUND: Hypermagnesemia is one of the vital electrolyte disturbances and is associated with such chronic conditions as cardiovascular, endocrinologic, renal diseases, and malignancy. AIM: This study evaluates the association between hypermagnesemia and clinical course in hospitalized patients. METHODS: This study was conducted at the University of Health Sciences Haseki Training and Research Hospital Internal Medicine Clinic. We evaluated a total of 3850 patients. 2130 patients have met the inclusion criteria were included in the study. Those who were discharged with healing were evaluated as having a good prognosis. Patients who died or were transferred to the intensive care unit (ICU) were defined as having a poor prognosis. We divided the patients' serum magnesium levels into four quartiles and examined the clinical course/conditions of the patients. RESULTS: Of 2130 patients, 1013 (51.9%) were female. The mean age of patients with poor prognoses (69.2 ± 14.9) was higher than those with good prognoses (59.7 ± 19.1). Hypermagnesemia (4th quartile) was detected in 61 (33.9%), and hypomagnesemia (1st quartile) was found in 42 (23.3%) patients out of 180 patients with poor clinical outcomes. It was statistically significant that hypermagnesemia was more common in patients with poor prognoses (p: 0.002). Chronic kidney disease (CKD) was diagnosed in 258 (53.3%) of 484 hypermagnesemia patients. Hypermagnesemia was found to be more common in patients with CKD, which was statistically significant (p: 0.003). CONCLUSIONS: Hypermagnesemia is associated with poor prognosis independent of comorbidities. Besides hypomagnesemia, hypermagnesemia should be considered a critical electrolyte imbalance.

Prognosis of Patients With Neuropathic Low Back-Related Leg Pain: An Exploratory Study Using Prospective Data From UK Primary Care.

This prospective cohort study investigates the prognosis of patients with neuropathic low back-related leg pain consulting in UK primary care. Data from 511 patients were collected using standardised baseline clinical examinations (including magnetic resonance imaging scan findings), self-report questionnaires at baseline, 4 months, 12 months, and 3 years. Cases of possible neuropathic pain (NP) and persistent-NP were identified using either of 2 definitions: 1) clinical diagnosis of sciatica, 2) self-report version of leeds assessment for neurological symptoms and signs (s-LANSS). Mixed-effects models compared pain intensity (highest of mean leg or mean back pain [0-10 Numerical Rating Scale]) over 3-years between persistent-NP versus non-persistent-NP based on 1) clinical diagnosis, 2) s-LANSS. Logistic regression examined associations between potential prognostic factors and persistent-NP at 4 months based on the 2 NP definitions. At 4-months, using both definitions: 1) approximately 4 out of 10 patients had persistent-NP, 2) mean pain intensity was higher for patients with persistent-NP at all follow-up points compared to those without, 3) only pain self-efficacy was significantly associated with persistent-NP (s-LANSS: OR .98, sciatica: .98), but it did not predict cases of persistent-NP in either multivariable model. Based on factors routinely collected from self-report and clinical examination, it was not possible to predict persistent-NP in this population. PERSPECTIVE: This study provides evidence that neuropathic back-related leg pain in patients consulting in primary care is not always persistent. Patients with persistent neuropathic pain had worse outcomes than those without. Neither leg pain intensity, pain self-efficacy nor MRI scan findings predicted cases of persistent neuropathic pain in this patient population.

Usefulness of the Phalen Test and the Tinel Sign in the Prognosis and the Impact on Quality of Life of Patients with Carpal Tunnel Syndrome Undergoing Classical Open Carpal Tunnel Release / Utilidade do teste de Phalen e do sinal de Tinel no prognóstico e o impacto na qualidade de vida de pacientes com síndrome do túnel do carpo submetidos a liberação aberta clássica do túnel do carpo

Abstract Objective: To evaluate the usefulness of the Phalen test and the Tinel sign in the prognosis and the impact on quality of life in the clinical course of patients with carpal tunnel syndrome undergoing surgical treatment through the traditional open approach. Methods: The present is a cohort study on prognosis. We included 115 patients with high probability of receiving a clinical diagnosis of carpal tunnel syndrome with indication for surgical treatment. All patients underwent the Phalen test and Tinel sign and answered the Boston Carpal Tunnel Questionnaire before and after the surgical treatment. Results: The estimates for the probability of the time until remission of the Phalen test at 2, 4 and 16 weeks postoperatively were of 3.54% (95% confidence interval [95% CI]: 1.16%-8.17%), 0.88% (95%CI: 0.08%-4.38%) and 0.88% (95%CI: 0.08% to 4.38%) respectively, and, for the Tinel sign, they were of 12.39% (95%CI: 7.13%-19.18%), 4.42% (95%CI : 1.65%-9.36%) and 2.65% (95%CI : 0.70%-6.94%) respectively. There was a reduction in the postoperative score on the Boston Carpal Tunnel Questionnaire of 1.8 points for symptom severity (p < 0.001) and of 1.6 points for functional status (p < 0.001). Conclusion: Phalen test remission was earlier than that of the Tinel sign, but, when performed as of the second postoperative week, they were prognostic factors favorable to the clinical course, with improved quality of life.

Resumo Objetivo: Avaliar a utilidade do teste de Phalen e do sinal de Tinel no prognóstico e o impacto na qualidade de vida no curso clínico de pacientes com síndrome do túnel do carpo submetidos ao tratamento cirúrgico por via aberta clássica. Métodos: Trata-se de um estudo de coorte sobre prognóstico. Foram incluídos 115 pacientes com alta probabilidade de diagnóstico clínico de síndrome do túnel do carpo com indicação de tratamento cirúrgico. Todos os pacientes foram submetidos ao teste de Phalen e ao sinal de Tinel, e responderam ao questionário de Boston antes e depois do tratamento cirúrgico. Resultados: As estimativas de probabilidade do tempo até a remissão do teste de Phalen em 2, 4 e 16 semanas pós-operatórias foram de 3,54% (intervalo de confiança de 95% [IC95%]:1,16%-8,17%), 0,88% (IC95%: 0,08%-4,38%) e 0,88% (IC95%: 0,08%-4,38%), respectivamente, e, do sinal de Tinel, foram de 12,39% (IC95%: 7,13%-19,18%), 4,42% (IC95%: 1,65%-9,36%) e 2,65% (IC95%: 0,70%-6,94%), respectivamente. Na pontuação pós-operatória no Questionário de Boston, houve redução de 1,8 ponto para a gravidade dos sintomas (p < 0,001), e de 1,6 ponto para o estado funcional (p < 0,001). Conclusão: A remissão do teste de Phalen foi mais precoce do que a do sinal de Tinel, mas, realizados a partir da segunda semana de evolução pós-operatória, esses testes foram fatores prognósticos favoráveis ao curso clínico, com melhora da qualidade de vida.

Clinical course of pathologically confirmed corticobasal degeneration and corticobasal syndrome.

The clinical presentation of corticobasal degeneration is diverse, while the background pathology of corticobasal syndrome is also heterogeneous. Therefore, predicting the pathological background of corticobasal syndrome is extremely difficult. Herein, we investigated the clinical findings and course in patients with pathologically, genetically and biochemically verified corticobasal degeneration and corticobasal syndrome with background pathology to determine findings suggestive of background disorder. Thirty-two patients were identified as having corticobasal degeneration. The median intervals from the initial symptoms to the onset of key milestones were as follows: gait disturbance, 0.0 year; behavioural changes, 1.0 year; falls, 2.0 years; cognitive impairment, 2.0 years; speech impairment, 2.5 years; supranuclear gaze palsy, 3.0 years; urinary incontinence, 3.0 years; and dysphagia, 5.0 years. The median survival time was 7.0 years; 50% of corticobasal degeneration was diagnosed as corticobasal degeneration/corticobasal syndrome at the final presentation. Background pathologies of corticobasal syndrome (n = 48) included corticobasal degeneration (33.3%), progressive supranuclear palsy (29.2%) and Alzheimer's disease (12.5%). The common course of corticobasal syndrome was initial gait disturbance and early fall. In addition, corticobasal degeneration-corticobasal syndrome manifested behavioural change (2.5 years) and cognitive impairment (3.0 years), as the patient with progressive supranuclear palsy-corticobasal syndrome developed speech impairment (1.0 years) and supranuclear gaze palsy (6.0 years). The Alzheimer's disease-corticobasal syndrome patients showed cognitive impairment (1.0 years). The frequency of frozen gait at onset was higher in the corticobasal degeneration-corticobasal syndrome group than in the progressive supranuclear palsy-corticobasal syndrome group [P = 0.005, odds ratio (95% confidence interval): 31.67 (1.46-685.34)]. Dysarthria at presentation was higher in progressive supranuclear palsy-corticobasal syndrome than in corticobasal degeneration-corticobasal syndrome [P = 0.047, 6.75 (1.16-39.20)]. Pyramidal sign at presentation and personality change during the entire course were higher in Alzheimer's disease-corticobasal syndrome than in progressive supranuclear palsy-corticobasal syndrome [P = 0.011, 27.44 (1.25-601.61), and P = 0.013, 40.00 (1.98-807.14), respectively]. In corticobasal syndrome, decision tree analysis revealed that 'freezing at onset' or 'no dysarthria at presentation and age at onset under 66 years in the case without freezing at onset' predicted corticobasal degeneration pathology with a sensitivity of 81.3% and specificity of 84.4%. 'Dysarthria at presentation and age at onset over 61 years' suggested progressive supranuclear palsy pathology, and 'pyramidal sign at presentation and personality change during the entire course' implied Alzheimer's disease pathology. In conclusion, frozen gait at onset, dysarthria, personality change and pyramidal signs may be useful clinical signs for predicting background pathologies in corticobasal syndrome.

[Association of polymorphic variants of hemostatic system genes with the course of COVID-19].

INTRODUCTION:   COVID-19 is characterized by a varied clinical course. The aim of the work was to identify associations of SNPs of hemostatic system genes with COVID-19. MATERIALS AND METHODS: DNA was isolated from patients (n=117) and healthy participants (n=104). All infected patients were divided into 3 groups, depending on disease severity assessment, which was appreciated by NEWS2. Another group consisted of participants, who had asymptomatic infection in the past. Determination of SNPs of the genes FGB (-455 G/A), FII (20210 G/A), FV (1691 G/A), FVII (10976 G/A), FXIIIA1 (103 G/T), ITGA2 (807 C/T), ITGB3 (1565 T/C), SERPINE1 (-675 5G/4G) were performed by PCR using the "Genetics of Hemostasis" kit ("DNA-Technology", Russia). RESULTS: In analyzed SNPs, no significant differences were detected between the group of infected patients and healthy participants. But significant association was revealed in gene SERPINE1 (-675 5G/4G), when patient groups, differing in the disease severity, were analyzed relative to the group of participants with asymptomatic infection (p=0.0381; p=0 .0066; p=0.0009). It was found, that as COVID-19 severity scores increased, the proportion of 5G allele of gene SERPINE1 decreased, and the proportion of the 4G allele increased (p=0.005; p=0.009; p=0.0005). Similar processes were observed for genotypes 5G/5G and 4G/4G. DISCUSSION: The gene SERPINE1 (-675 5G/4G) is associated with the severity of COVID-19. CONCLUSION: For the first time, it was discovered that 5G/5G genotype of gene SERPINE1 (-675 5G/4G) can be a marker of a milder course of COVID-19, and the 4G/4G genotype as a more severe one.

Evolución y factores de riesgo de mortalidad en una cohorte pediátrica con enfermedad renal crónica en Paraguay: estudio multicéntrico

Contexto: la enfermedad renal crónica (ERC) tiene una alta morbimortalidad y un alto costo de tratamiento. Entre sus causas principales en Pediatría se describen las anomalías congénitas del riñón y las vías urinarias (CAKUT, por sus siglas en inglés) Objetivo: determinar la evolución y los factores asociados a mortalidad de una cohorte pediátrica atendida en dos hospitales de referencia nacional, con el fin de elaborar estrategias para el manejo precoz y multidisciplinario de esta enfermedad y, de esa manera, optimizar los recursos para reducir la morbimortalidad de la enfermedad. Metodología: estudio de cohorte retrospectivo de pacientes de dos hospitales de referencia entre enero del 2000 y diciembre del 2020, en el cual se estudiaron: edad, sexo, seguro social, ingreso fijo, causa de la ERC, estadio de la ERC al ingreso y al final de estudio, requerimiento y tipo de diálisis, trasplante y óbito, donde el estadio de la ERC se clasificó según las guías K/DOQI (Kidney Disease Quality Iniciative). Por su parte, se relacionó óbito con edad, sexo, ingreso fijo, seguro social, estadio de ERC y requerimiento de diálisis. Se destaca que el análisis se realizó con el programa Epi Info (Atlanta), el cual usa estadística descriptiva y la prueba de chi cuadrado para establecer asociaciones con óbito a un nivel de significancia de 0,05. Resultados: se estudió a 187 pacientes de entre 2 y 17 años de edad, 96 mujeres y 91 varones, donde la mayoría eran mayores de10 años (44,9 %). Entre las causas de ERC más frecuentes se encuentran las anomalías congénitas (CAKUT) (54,5 %) y las indeterminadas (16,6 %); el estadio de ERC al ingreso estuvo repartido entre I (2,1 %), II (16 %), III (25,7 %), IV (8 %), y V (48,1 %); además, el 59,4 % requirió diálisis, el 87,3 % hemodiálisis, el 12,6 % diálisis peritoneal y 13,3 % recibieron un trasplante; por último, la mortalidad fue del 24,5 %, lo cual se asoció con el estadio de ERC al ingreso y el requerimiento de diálisis. Conclusiones: este estudio de cohorte demostró mejores resultados en la evolución de los niños con ERC diagnosticados en estadios tempranos. El diagnóstico y tratamiento precoces ayudan a mejorar la morbimortalidad.

Background: Chronic kidney disease (CKD) has high morbidity and mortality and high cost of treatment. Among the main causes are congenital anomalies of the kidney and urinary tract (CAKUT). Purpose: To determine the evolution and factors associated with mortality in a pediatric cohort attended in two national referral hospitals, in order to develop strategies for early and multidisciplinary management of this disease and, thus, optimize resources to reduce morbidity and mortality of the disease. Methodology: Retrospective cohort study of patients in two reference hospitals between January 2000 and December 2020. Age, sex, social security, fixed income, cause of CKD, CKD stage at admission and at the end of study, requirement and type of dialysis, transplant and death were studied. CKD stage was classified according to the K/DOQI (Kidney Disease Quality Initiative) guidelines. Death was related to age, sex, fixed income, social security, CKD stage, and dialysis requirement. Data analysis was performed with the Epi Info program (CDC, Atlanta), using descriptive statistics, and the chi-square test to establish associations with death at a significance level of 0.05. Results: 187 patients between 2 and 17 years of age 96 women and 91 men, were studied. Most of the patients were older than 10 years (44.9%). Among the most frequent causes of CKD are congenital anomalies (CAKUT) (54.5%) and undetermined (16,6%). CKD stage at admission was I (2,1%), II (16%), III (25,7%), IV (8%), and V (48.1%). 59.4% required dialysis, hemodialysis (87.3%), peritoneal dialysis (12.7%), 13.3% underwent kidney transplant. Mortality rate was 24.5%, which was associated with CKD stage at admission and dialysis requirement. Conclusions: This cohort study demonstrated better outcomes in children with CKD diagnoses at its early stages. The early diagnosis and treatment help to improve the morbidity and mortality.