Clinical Course Research Articles

Teaching Ethics Consultation Using a Tabletop Exercise.

Drawing on pedagogical tools utilized in clinical scenario simulation and emergency preparedness training, the authors describe an innovative method for teaching clinical ethics consultation skills, which they call a "tabletop" exercise. Implemented at the end of a clinical ethics intensive course, the tabletop enables learners to implement the knowledge and practice the skills they gained during the course. The authors highlight the pedagogical tools on which the tabletop exercise draws, describe the tabletop exercise itself, offer how to best operationalize such an exercise, reflect on the method's strengths and weaknesses, and provide insights for others who may want to implement their own tabletop for ethics consultation education.

SMARCA4 Deficient Gastric Carcinoma With Squamous Differentiation in a Young Patient With Aggressive Clinical Course

Inactivation of SMARCA4 (BRG1), a subunit of SWI/SNF complex, has been reported in malignancies from various sites, including the thorax, uterus, ovary, and gastrointestinal tract, and is usually associated with aggressive clinical course. These tumors have been reported primarily in elderly patients and on histology demonstrate high-grade morphology, often with rhabdoid differentiation. SMARCA4 loss is exceedingly rare in primary gastric cancers. Here, we present a unique example of SMARCA4 deficient gastric carcinoma in a 28-year-old male patient with molecular confirmation which showed a poorly differentiated histology and focal squamous differentiation. Despite multidisciplinary management, including FOLFOX chemotherapy, immunotherapy (nivolumab), and surgical resection, the patient succumbed to his disease within 2 years of diagnosis. This case report highlights the aggressive nature of SMARCA4-deficient carcinomas, which are often resistant to conventional chemotherapy, and underscores the importance of early diagnosis and exploring alternative therapeutic approaches for such malignancies.

Enteral tube administration of extemporaneously compounded suspensions of venetoclax according to an institutional medication use process.

Venetoclax is a potent oral oncology drug (OOD) frequently used to treat hematologic cancers due to its convenience and high efficacy. However, some patients cannot tolerate solid oral formulations, requiring a reformulated version of venetoclax for effective administration. Currently, there is limited information in the literature regarding the extemporaneous compounding of venetoclax. We present our institution's medication use process (MUP) for preparing compounded suspensions of venetoclax for patients with dysphagia who cannot tolerate solid oral formulations. This report has two main aims: to review our MUP from design to implementation, illustrated with real patient cases, and to present it as a strategy for administering venetoclax-based therapies to patients with dysphagia or feeding tubes. From February 2020 to January 2024, 17 patients received venetoclax through the standard MUP, with four developing tumor lysis syndrome (TLS). This study describes their clinical courses and evaluates their responses to the compounded suspensions, considering the uncertain effects of venetoclax prepared through this method. The four patients received venetoclax suspensions during hospitalization due to swallowing difficulties or nutritional support needs. We prepared extemporaneous venetoclax suspensions to ensure continuous therapy, thus avoiding treatment interruptions. TLS was managed using rasburicase and prophylactic allopurinol. The clinical outcomes indicate that the current MUP provides a practical approach to administering venetoclax for dysphagic patients. However, additional research is needed to determine if there are pharmacokinetic differences in the bioavailability of venetoclax between oral and enterally delivered formulations. Further studies will help inform best practices for patient care.

Unexpected Malrotation in Patients with Congenital Heart Disease Undergoing Gastrostomy Tube Placement: Is Routine Preoperative Upper Gastrointestinal Series Imaging Necessary?

Our aim is to determine the rate of unexpected malrotation identified on routine preoperative upper gastrointestinal (UGI) contrast study in infants with congenital heart disease (CHD) prior to gastrostomy tube (GT) placement and quantify any associated delay in care. We performed a retrospective review of infants with CHD who underwent GT placement following initial cardiac surgery at a single center between 2016 and 2021. Patients were identified in the electronic medical record.Demographic information, indications for GT placement, and clinical course were collected. Variables were compared using Mann-Whitney test, with significance set at p < 0.05. One hundred and thirty-one infants with CHD underwent GT placement after cardiac surgery; 124 (94.7%) underwent preoperative UGI of which 119 (95.2%) were normal.Five studies were read as "could not rule out malrotation" and one infant had malrotation on UGI. Median time from UGI to surgery was 3days. Median days from consult to GT placement was 3days among those who underwent UGI and 2days in those who did not (p = 0.34). Among infants with CHD, the rate of unknown malrotation is low.UGI contrast study prior to GT placement may be associated with surgical delay, unnecessary radiation exposure, and low value healthcare. If a child is tolerating gastric feeds prior to GT placement, routine UGI is unnecessary.

Sinus node dysfunction in children: different aetiologies, similar clinical course in two-centre experience.

This study aims to evaluate the clinical characteristics and outcomes of children diagnosed with sinus node dysfunction. This was a retrospective review of patients diagnosed with sinus node dysfunction in two tertiary paediatric cardiology centres in Turkey from January 2011 to June 2022. In all, 77 patients (50, 64.9% males) were included, with a mean age of 8.2 ± 6.3 years and a mean weight of 28.2 ± 18.8 kg. While age-incompatible bradycardia and pauses were the most common rhythm disturbances, syncope, presyncope, and dizziness (n:33, 43%) were the most frequent initial symptoms. Structural heart disease was present in 58 (75.3%) of the 77 patients, 47 (61%) of whom were congenital. The most commonly associated CHDs were transposition of the great arteries (n:8), atrial septal defect (n:7), and atrioventricular septal defect (n:5). Seven of them also had left atrial isomerism. The remaining 19 patients were isolated. Four patients had SCN5A mutation (two of them were siblings) and two of them had Emery-Dreifuss muscular dystrophy. Although sinus node dysfunction is rare in children, it has been diagnosed with increasing frequency with structural heart disease, especially in patients who have undergone corrective cardiac surgery related to atrial tissue. Since sinus node dysfunction can occur at any time postoperatively, these patients should be kept under constant control. If symptomatic sinus node dysfunction is confirmed, permanent pacing is an effective therapeutic modality.

Primary resistance to nivolumab plus ipilimumab therapy affects second-line treatment outcomes in patients with metastatic renal cell carcinoma.

Nivolumab plus ipilimumab (NIVO+IPI) has a long-term response rate of 30% for patients with metastatic renal cell carcinoma (mRCC). However, 20% of patients develop primary resistant disease (PRD) to NIVO+IPI and show poor survival outcomes. In this study, we aimed to evaluate the effect of PRD as a second-line treatment in patients with mRCC. The data used in this multi-institutional, retrospective cohort were collected between August 2015 and January 2023. In total, 189 patients with mRCC were treated with NIVO+IPI and then with a vascular endothelial growth factor receptor-tyrosine kinase inhibitor. Associations between PRD and progression-free survival of second-line treatment (PFS), progression-free survival 2 (PFS2), and overall survival (OS) were analyzed. The median age at NIVO+IPI initiation was 67 years in the male-dominant population (n = 140, 74.1%), and most patients had clear cell histology (n = 140, 74.1%). PRD was recorded in 42 (22.2%) of 189 patients during NIVO+IPI therapy. Patients who experienced PRD showed poor PFS (hazard ratio [HR], 1.788; 95% confidence interval [CI], 1.176-2.718; p = 0.007), PFS2 (HR, 4.127; 95% CI, 2.649-6.431; p < 0.001), and OS (HR, 3.330; 95% CI, 2.040-5.437; p < 0.001). Before starting second-line therapy, patients with PRD tended to have a poor performance status compared with non-PRD patients and a higher IMDC risk. Second-line drug therapy was not associated with treatment outcomes in patients with PRD. PRD in patients with mRCC receiving NIVO+IPI as first-line treatment was associated with poor clinical course, even with second-line therapy.

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.

Clinical factors to predict changes of esophagogastric varices after sustained viral response with direct-acting antiviral therapy.

The clinical course of esophagogastric varices (EGV) after sustained virological response (SVR) with direct-acting antiviral (DAA) therapy has not been clearly elucidated. The predictors for the worsening/improvement of EGV after SVR with DAA therapy were investigated. Of the cirrhosis patients who achieved SVR with DAA therapy, 328 patients who underwent endoscopic examinations both before and after DAA therapy were enrolled. The predictors of EGV worsening or improvement were investigated. Multivariate analysis identified a history of ascites retention, albumin at baseline, and MELD score at baseline as independent factors that contributed to EGV exacerbation. On multivariate analysis, two factors, BMI and platelet count, were related to EGV improvement. An integrated scoring system was created using these risk factors with or without weighting according to each hazard ratio, and the patients were divided into three groups. A scoring system with weighting of each factor appeared to be more useful, with fewer intermediate patients and more cases classified into the low-risk and high-risk groups. Esophagogastric varices after SVR have a varied clinical course. Using this scoring system that can accurately predict EGV outcomes in clinical settings, it may be feasible to establish a risk-based EGV surveillance plan following SVR.

Arbovirus in Solid Organ Transplants: A Narrative Review of the Literature.

The incidence of arbovirus infections has increased in recent decades. Other than dengue, chikungunya, and West Nile viruses, the data on arbovirus in solid organ transplant (SOT) are limited to case reports, and infections in renal transplant recipients account for most of the reported cases. Dengue and West Nile infections seem to be more severe with higher mortality in SOT patients than in the general population. Acute kidney injury is more frequent in patients with dengue and chikungunya although persistent arthralgia with the latter is less frequent. There is no clear relationship between arboviral infection and acute cellular rejection. Pre-transplant screening of donors should be implemented during increased arboviral activity but, despite donor screening and negative donor nucleic acid amplification test (NAT), donor derived infection can occur. NAT may be transiently positive. IgM tests lack specificity, and neutralizing antibody assays are more specific but not readily available. Other tests, such as immunohistochemistry, antigen tests, PCR, metagenomic assays, and viral culture, can also be performed. There are a few vaccines available against some arboviruses, but live vaccines should be avoided. Treatment is largely supportive. More data on arboviral infection in SOT are needed to understand its epidemiology and clinical course.

Partial Response to Treatment with ALK Inhibitor in a Patient With SQSTM1-ALK Fusion Positive Lung Adenocarcinoma.

Lung cancer is the second most common cancer worldwide and the leading cause of cancer deaths; non-small cell lung cancer (NSCLC) constitutes about 85% of lung cancer cases, with ALK fusions representing 3-6% of them. The SQSTM1-ALK fusion is a rare finding in NSCLC, accounting for only 1.1% of ALK rearrangements. We present a case of lung adenocarcinoma with documentation of SQSTM1-ALK fusion that showed a partial response to alectinib. This case details the clinical course of a 71-year-old, non-smoking woman with no significant medical history who presented with confusion, aphasia and multiple cerebral lesions detected on imaging. Further investigations revealed a stage IV lung adenocarcinoma with metastases to the brain and adrenal gland. Molecular profiling identified a rare SQSTM1-ALK fusion mutation alongside other genetic abnormalities, including low programmed death-ligand 1 expression and ROS1 kinase protein presence. Treatment with alectinib, initiated based on the identified ALK fusion, resulted in significant tumour regression in the lungs and complete resolution of the adrenal mass, as evidenced by follow-up imaging and clinical assessments. This case highlights the efficacy of alectinib in treating rare ALK fusion variants in and underscores the importance of comprehensive molecular profiling in guiding targeted therapy decisions. Recognition of rare ALK fusions This case highlights the importance of identifying rare ALK fusions, such as SQSTM1-ALK, in non-small cell lung cancer (NSCLC), which can guide personalised treatment strategies.Utility of advanced molecular diagnostics The use of next-generation sequencing alongside immunohistochemistry is crucial for accurately detecting ALK and ROS1 rearrangements, avoiding false positives and enabling the identification of druggable mutations.Impact of personalised medicine This case reinforces the value of personalised medicine in NSCLC, where molecular profiling can uncover unique genetic alterations, allowing for more tailored and potentially more effective treatments.

Tocilizumab (TCZ) for Giant Cell Arteritis: Clinical Outcomes Following Relapses and TCZ Discontinuation Due to Adverse Events.

Tocilizumab (TCZ) is effective for giant cell arteritis (GCA). However, little is known regarding treatment modification and clinical outcomes after unfavorable events such as GCA relapses or TCZ discontinuation due to adverse events (AEs). This multicenter retrospective study included patients with GCA who initiated TCZ from 2008 to 2021 at 5 Japanese hospitals. GCA relapses and TCZ-related AEs were monitored for 2 years after TCZ initiation. In patients with GCA relapses, subsequent clinical courses, including relapse symptoms and treatment modification, were followed for 90 days after the relapses. Similarly, patients who discontinued TCZ because of AEs were additionally followed until 1 year after the TCZ discontinuation to evaluate AEs, relapses, and treatment changes. Of 62 eligible patients, 10 patients (16%) relapsed after initiating TCZ therapy. Most relapses (8 of 10) occurred after extending TCZ intervals or discontinuing TCZ. Combinations of adjusting TCZ intervals, adjusting glucocorticoid (GC) dose, and/or adding or increasing methotrexate (MTX) therapy could manage the relapses without serious complications. In the entire cohort, AEs occurred in 28 patients (45%), and 8 patients (13%) discontinued TCZ because of AEs. After AE-related TCZ discontinuation, 6 patients attempted to taper GCs without other immunosuppressive therapy (IST), and 4 subsequently relapsed. In contrast, 2 patients who used other IST or biologic therapy could decrease GCs without relapses. Although GCA relapses can occur after initiating TCZ therapy, most relapses can be safely managed by adjusting TCZ, GC, and/or MTX doses. Adding IST or biologic treatments may potentially be related to preventing relapses when patients discontinue TCZ because of AEs.

The Clinical Significance of Interstitial Pneumonia with Autoimmune Features in Cryptogenic Organizing Pneumonia: A Prospective Multicenter Observational Study.

Background: There are cases of idiopathic interstitial pneumonias (IIPs) that do not meet the diagnostic criteria for connective tissue disease but have clinical features suggestive of autoimmune process. Interstitial pneumonia with autoimmune features (IPAF) was recently proposed as a research concept for these patients. Although several prospective studies on IPAF have been conducted, its clinical significance in cryptogenic organizing pneumonia (COP) remains unclear. Methods: Patients aged ≥20 years with suspected COP were prospectively enrolled between June 2018 and December 2022. Among the enrolled patients, those diagnosed with COP based on computed tomography (CT) and bronchoalveolar lavage (BAL) findings were compared between the IPAF and non-IPAF groups. Results: A total of 56 patients were enrolled in this study. Of these, 30 were diagnosed with COP and included in the analysis. Clinical and serological features were positive in two and six patients, respectively. Each feature was exclusive, and eight patients (26.7%) were diagnosed with IPAF. There were no differences between the IPAF and non-IPAF groups in terms of clinical features, including BAL findings, laboratory data, CT findings, and clinical course. During the one-year follow-up period, the frequency of COP exacerbation did not differ between the IPAF and non-IPAF groups, and no cases of systemic autoimmune disease or death occurred in either group. Conclusions: The COP characteristics of the IPAF and non-IPAF groups are similar in all aspects, and distinguishing between the two groups may be of little significance.

Dissecting the genomic traits and clinical course of secondary myelodysplastic syndrome following aplastic anaemia: A milestone.

Dissecting the genomic traits and clinical course of secondary myelodysplastic syndromefollowing aplastic anaemia is a milestone. The report by Li and colleagues investigates determinants of evolution to myelodysplastic syndrome and acute myeloid leukaemia in patients with aplastic anaemia and paroxysmal nocturnal haemoglobinuria with a specific focus on post-transplant outcomes. Commentary on: Li etal. Clinical and genetic profiles and outcomes of allogeneic haematopoietic stem cell transplantation in secondary myelodysplastic syndrome following aplastic anaemia. Br J Haematol 2024 (Online ahead of print). doi: 10.1111/bjh.19855.

"Treatable not curable": trade-offs in the use of treatment-oriented language with patients who have incurable cancer.

Treatment-oriented language is used by physicians to convey to patients that treatment is available for their cancer (eg, "our usual treatment for this is…," "we can treat this," "your cancer is still treatable"). For patients who have incurable cancer, especially for patients with a poor prognosis or who are at the end of life, it is important to understand how physicians conceptualize and use this "everyday" clinical language. We conducted a qualitative interview study with a multidisciplinary group of physicians (n = 30) who may care for patients with cancer at different points in their clinical course, from diagnosis to end of life. Physicians report a wide range of reasons for using treatment-oriented language in conversations with patients who have incurable cancer. However, physicians also reported concerns that this language can be ambiguous, can convey unintended positive prognostic information, and can shift attention away from important matters such as the non-curative nature of treatment or the inevitability of death. On the basis of these concerns, physicians should (1) consider whether their aims in using treatment-oriented language can be better achieved using other evidence-based communication strategies, and (2) recognize and proactively mitigate potential adverse effects of treatment-oriented language, which may manifest much later in the patient's clinical course.

The Many Faces of Guilt: A Review Mapping Unique and Overlapping Expressions in OCD and Depression.

Guilt, a complex emotional experience pervading many lives, takes on an intricate form when intertwined with psychiatric conditions. As a multifaceted concept, guilt represents a key diagnostic feature in depression and is an integral part of obsessive compulsive disorder (OCD). This systematic review aimed to synthesize empirical research on the varied dimensions of guilt across these two mental illnesses, where guilt is emphasized as a pathognomonic factor. A systematic computer-based literature search was conducted, using a rigorous set of eligibility criteria and specific keyword combinations to ensure relevant and exhaustive coverage of the topic. Following Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines, a comprehensive literature search spanning multiple databases identified 12 eligible studies on guilt in OCD and 8 in depression. The findings revealed 20 distinct guilt constructs measured across the two disorders. Notably, 5 unique guilt subtypes and 15 themes for OCD and 6 exclusively studied guilt for depression emerged, reflecting potential disorder-specific manifestations of guilt. Delineating such guilt profiles holds the potential to untangle the role of guilt in the onset, perpetuation, and clinical course of OCD and depression. This comprehensive mapping of guilt constructs provides an empirical foundation for elucidating disorder-specific pathways influenced by guilt, thereby informing the development of targeted psychological interventions tailored to the distinct guilt patterns underpinning these debilitating conditions.

The abundance of regulatory T cell subsets is associated with the clinical outcomes of sarcoidosis.

Some patients with sarcoidosis achieve spontaneous remission, whereas others repeatedly experience relapse. We examined differences in the clinical course of active sarcoidosis according to peripheral blood immunophenotypes before treatment. This retrospective study compared peripheral blood immunophenotypes between patients with active sarcoidosis (n=28) and healthy control subjects (n=10). Patients with sarcoidosis were divided into the spontaneous remission group without treatment (n=9), the non-relapsed group after treatment (n=13), and the relapsed group after treatment (n=6) and were compared for peripheral blood immunophenotypes and background characteristics at baseline. Patients with sarcoidosis showed increases in activated T helper (Th) 1 cells, activated Th17 cells, and regulatory T (Treg) cell subsets. The proportion of effector Treg cells was highest in the spontaneous remission group, and the proportion of non-suppressive Treg cells was highest in the relapsed group. No differences were observed in the proportions of other CD4+ T cell subsets. The cut-off values for predicting spontaneous remission and relapse were calculated for the effector Treg/non-suppressive Treg ratio. As a result, A ratio ≥1.469 predicted spontaneous remission (75%), while ≤0.722 predicted relapse (66.7%). Effector and non-suppressive Treg cell proportions before treatment may predict spontaneous remission and relapse in active sarcoidosis.

Clinical course of pediatric-onset Behçet's Disease in young adulthood.

Although Behçet's disease (BD) typically manifests in the second or third decade of life, initial symptoms may appear at a younger age. It may also take a longer time for the full disease phenotype to develop after the first symptom onset in pediatric patients. In this study we aimed to assess the clinical course of pediatric-onset BD in adulthood period. The files of 112 patients diagnosed with BD before the age of 18, selected from five tertiary clinics, were retrospectively examined. Patients with a follow-up of less than six months were excluded. The study comprised 93 patients with pediatric-onset BD, of whom 64.5% (n = 60) were male. The median age of diagnosis was 15 years (13-17). Major organ involvement was present in 49 (52.5%) patients. The most commonly affected organ was the eye (29%).Sixty-eight (73.1%) patients had follow-up data in adulthood. Fourty patients had only mucocutaneous manifestations in the pediatric period. During follow-up in adulthood, 15 (53.3% were male) had new major organ involvement with a mean of 10.1 (SD: 7.9) years after diagnosis. Twenty-eight patients (41.1%) experienced major organ involvement during the pediatric period. In adulthood follow-up, 12 (42.8%) developed new major organ involvement and/or relapse of the same organ. Eighteen (26.5%) of 68 pediatric-onset BD patients had new major organ involvement, and 9 (13.2%) had a relapse during adulthood follow-up. Our results showed that nearly one-third of pediatric BD patients have a new major organ involvement or a relapse in adulthood. Regular follow-up of pediatric BD patients in adulthood is essential to prevent long-term damage in this disease subset.

Dorsolateral Transdural Surgical Management of Spontaneous Intracranial Hypotension From Ventral Dural Cerebrospinal Fluid Leaks: Case Series and Technical Report

BACKGROUND and OBJECTIVES: Spontaneous intracranial hypotension is an uncommon but debilitating condition that commonly involves positional headaches and vertigo. One cause is cerebrospinal fluid (CSF) leakage from ventral dural defects from calcified spinal discs. Patients can undergo epidural blood or fibrin glue patches as treatment; however, in cases where the leak does not resolve, surgical repair of the dural tear may be warranted. We aim to describe a case series and technical notes for these patients. METHODS: Patients with spontaneous intracranial hypotension because of myelography-confirmed ventral dural defect from calcified thoracic disc, who were treated surgically between June 2020 and March 2024, were included in the study cohort. Demographic information, clinical course, and postoperative outcomes were collected. The surgical technique was also described based on an illustrative case. RESULTS: Twelve consecutive patients underwent surgery between June 2020 and March 2024 with postoperative records. The median age at the time of surgery was 41.5 years (range: 28-58 years). Male patients comprised 50% of the cohort. Dural defects were spread throughout the thoracic spine in the cohort. While all patients reported positional headaches, other symptoms included vertigo, pulsatile tinnitus, and neck pain. Median length of follow-up time was 6.5 weeks. There were no surgical complications or permanent neurologic deficits. All patients had radiologic cure of their leaks with absence of extradural CSF on postoperative MR imaging. Ten patients (83.3%) had complete resolution of spontaneous intracranial hypotension-related symptoms. Two patients did suffer from rebound headaches postoperatively. CONCLUSION: The dorsolateral transdural surgical approach is a safe and effective method to repair type 1 CSF leaks caused by ventral dural defects. Our case series of 12 patients did not involve any surgical or immediate postoperative complications, and all patients had radiologic resolution of their leaks with relief of positional headaches. Two patients experienced continued headaches after treatment.

Effects of dietary cellulose on clinical and gut microbiota recovery in dogs with uncomplicated acute diarrhea: a randomized prospective clinical trial.

To assess the impact of dietary fiber supplementation with cellulose on clinical course, fecal consistency, and intestinal microbiota composition in dogs with uncomplicated acute diarrhea (AD). From September 2022 to November 2023, a total of 19 dogs presenting with uncomplicated AD were included in this prospective, randomized, and double-blinded clinical trial. The time to resolution of diarrhea was evaluated via owner surveys and a fecal scoring chart. The client-owned dogs were randomly assigned to a cellulose group (CG) or control group. The intestinal microbiota was analyzed via quantitative PCR. A marginally significant, faster improvement in stool consistency on day 1 was observed in the CG (P = .09). All dogs improved clinically, with a median recovery time of 3.0 days in the CG and 3.2 days in the control group (range, 1 to 6 days in both groups). There was no significant difference regarding the Canine Acute Diarrhea Severity index or composition of the intestinal microbiota during the study. All dogs with uncomplicated AD exhibited rapid clinical improvement and recovery of the core intestinal microbiota within the first few days. Cellulose improved the fecal consistency in a subset of dogs, and intestinal dysbiosis was mild and self-limiting. The administration of dietary cellulose has the potential to accelerate improvements of stool consistency. Mild changes in pathobionts, such as an increased amount of Clostridium perfringens, are self-limiting; thus, antibiotic intervention is not warranted.