Complete Remission Of Symptoms Research Articles

Blood Patch for Post-Surgical Cerebrospinal Fluid Leak: A Case Report

Intracranial hypotension syndrome may occur secondary to Cerebrospinal Fluid Leak (CSFL). Its etiology can be iatrogenic, traumatic or spontaneous. The most common cause is related to incidental or intentional punctures during anesthetic approaches. It is a rare complication during lumbar spine surgery. The clinical manifestations of this syndrome are characterized by orthostatic headache as the most frequent symptom, accompanied by a wide variety of manifestations, which frequently result in a disability for daily life activities and a delayed recovery. Diagnosis is made through a detailed medical history, the presence of symptoms and signs, and additional imaging studies. Initial treatment is conservative; however, using a hematic patch is recommended for a significant number of patients as the next step, reserving surgical treatment for cases that are not resolved with these measures. This paper presents the clinical case of a 56-year-old female patient diagnosed with CSF fistula following a surgical procedure, which is treated by applying a blood patch with complete remission of symptoms; similarly, a therapeutic algorithm is proposed for the diagnosis of CSFL.

Contrast Induced Encephalopathy: Case Report and Review of The Literature

Background: Contrast Induced Encephalopathy is a known but rare complication of endovascular procedures. Patients show neurologic symptoms mimicking a stroke and include visual disturbances, motor or sensory deficits, headache, seizures, memory loss, confusion, aphasia, and coma. Irreversible neurological symptoms are rare and fatal encephalopathy is even more so. Case Report: In this article we present a case of a 75-year-old female patient who showed neurological symptoms mimicking a stroke post cerebral Digital Subtraction Angiography that was done with Iohexol as a contrast agent, as a diagnostic work up to rule out a ruptured aneurysm. Further investigations showed no arterial spasms nor dissection. Symptoms reappeared after the second contrast administration but completely resolved after the administration of steroids and fluids. Conclusion: Contrast Induced Encephalopathy should be further investigated with imaging to rule out other thromboembolic or hemorrhagic causes. Treatment via the administration of steroids and fluids have shown to be effective with complete remission of symptoms.

Three cases of scombroid syndrome in Italy: clinical and preventive considerations.

Foodborne diseases are common sources of morbidity and mortality worldwide. Scombroid syndrome represents a particular condition since it is not directly related to the ingestion of spoiled food but is determined by high levels of histamine, a chemical mediator naturally produced within the human body under particular conditions. In these cases, histamine is formed as a result of the bacterial activity from histidine, an amino acid present at high levels in some fish species. The resulting symptomatology can range from mild symptoms such as headache and skin rash to more severe manifestations such as hypotension and coronary spasms. Reference regulations in Italy set maximum levels of histamine in food at 200 mg/kg. The cases described involve a family of three who, following the ingestion of a tuna dish, started to exhibit symptoms typical of an allergic reaction. In one case, hypotension, tachycardia, and electrocardiographic changes in the ST-tract suggestive of myocardial ischemia also appeared with negative myocardionecrosis enzyme dosage. All three cases experienced complete remission of symptoms in the absence of sequelae. Histamine concentrations in fish sampled three days later were 169 mg/kg. The cases described emphasize the importance of proper differential diagnosis as well as the importance of implementing specific controls in food hygiene.

Advances and new treatments are available for neuromuscular disorders and affect Quality of Life

Advances over the past two decades in the field of neuromuscular disorders have transformed the treatment landscape, bringing more genetic testing for screening, better laboratory analyses, new innovative therapies that target specific disease pathways and mechanisms, and a multidisciplinary approach to care. These advancements have led to more precise diagnoses and advances in the management of neuromuscular conditions, which can be life-altering for patients. This review examines how the emergence of new therapies impacts the quality of life (QoL) of parents and children with Duchenne muscular dystrophy and patients with late-onset glycogenosis type 2. Myasthenia Gravis (MG) is the most common neuromuscular transmission disorder. Despite the existence of few refractory cases, the goal of treatment is the complete remission of symptoms, achieved by thymectomy, immunosuppression, IVIG, or monoclonal antibody. Advances over the past two decades have been substantial both in mitochondrial disorders and for.MG patients that are benefitting from an expansion of treatments since more therapies are available.

Early and late outcomes of video thoracoscopic versus open approach for bronchiectasis lung resections.

To evaluate the early and late results of surgical treatment of patients with bronchiectasis, comparing the Video-Assisted Thoracic Surgery (VATS) vs. the open thoracotomy (OT). Observational retrospective study of patients who underwent surgery for bronchiectasis. Patients were divided into two groups according to surgical access OT/VATS. Variables collected included gender, age, preoperative symptoms, etiology, segments involved, FVC and FEV1, type of surgical resection, complications, mortality, and length of hospital stay. Late surgical results were classified as excellent, complete remission of symptoms; good, significative improvement; and poor, little/no improvement. 108 surgical resections (103 patients). OT group 54 patients (52.4%) vs. VATS 49 (47.6%). A high percentage of complications was observed, but no difference between the OT (29.6%) and VATS (24.5%) groups was found. Post-operative hospital stay was shorter in the VATS group (5.4 days) vs. the OT group (8.7 days (p=0.029). 75% of the patients had a late follow-up; the results were considered excellent in 71.4%, good in 26%, and poor in 2.6%. Regarding bronchiectasis distribution, an excellent percentage was obtained at 82.1% in patients with localized bronchiectasis and 47.5% with non-localized bronchiectasis, p=0.003. VATS leads to similar results regarding morbidity, compared to OT. However, VATS was related to shorter hospital stays, reflecting the early recovery. Late results were excellent in most patients, being better in patients with localized bronchiectasis. VATS should be considered a preferable approach for bronchiectasis lung resection whenever possible.

Management of Ekakushta (childhood psoriasis) through Ayurveda multimodal approach: A case report

Abstract Kushta Roga is a broad term used in Ayurveda to refer to various skin ailments. Ekakushta is a type of Kshudra Kushta caused by the Vata and Kapha Dosha. Its symptoms are quite similar to those of psoriasis, including Aswedanam (no sweating/dryness), Mahavastum (affecting a large region), and Matsyashakalopamam (scaling like fish skin). Corticosteroids are usually prescribed topically to treat psoriasis, although this only offers temporary relief. Ayurveda has effective treatment of Ekakushta using a variety of interventions; nevertheless, because of the disease’s complicated etiology, a multimodal approach is necessary. A 15-year-old girl with a moderate build has been complaining for the last 4–5 years about reddish-patchy skin rashes that cause pain, scaling, and itching all over her body. She also reported recurrent relapses. The symptoms pointed to the involvement of the Vata and Kapha Dosha, suggesting Ekakushta (psoriasis). Due to the disease’s tendency toward remission and recurrence, we undertook purgative therapy (Virechana Karma) for the purification of vitiated Dosha. The patient was administered Shamana medicines for Dosha pacification and a topical application following purifying therapy. A complete remission of symptoms was noted after Ayurvedic therapy, as documented by pre- and post-therapy photos of lesions. Furthermore, there were notable declines in the Psoriasis Area and Severity Index score (32.6 to 0), Adolescent Psoriasis Quality of life Psychosocial Impact (42 to 8 at the end of treatment), and Adolescent Psoriasis Quality of life Physical Symptoms and Treatment (17 to 3) after 6 months of treatment with no documented adverse drug reactions during treatment. Ekakushta (psoriasis) is extremely difficult to treat; nonetheless, a comprehensive pathogenesis assessment, appropriate purification, and pacifying therapy work well.

Comparison of clinical efficacy of open and single channel carpal tunnel release in the treatment of carpal tunnel syndrome

Background. Open or wrist arthroscopic release of carpal tunnel is a relatively safe and reliable method. However, there is still some debate as to which of these various surgical methods is superior. Therefore, this study provides more clinical evidence by comparing the clinical efficacy of open and endoscopic single-channel bowl release in the treatment of carpal tunnel syndrome. Methods. The study conducted a retrospective analysis from January 2010 to January 2021. A total of 105 patients met the inclusion criteria, including 56 patients in the open carpal tunnel Release (OCTR) group and 49 patients in the single channel carpal tunnel release (SCCTR) group. The preoperative, intraoperative, and postoperative symptom improvement and functional recovery of the patients were compared. Results. The results of this study showed that the surgical incision healed well in all patients and complete remission of entrapment symptoms. Compared with the OCTR group, the SCCTR group had reduced surgical and hospital duration, intraoperative blood loss, postoperative scar pain score, and time required to return to normal lifestyle, and the SCCTR group had a lower incidence of sympathetic dystrophy and significantly improved clinical symptoms. Conclusion. Single Channel Carpal Tunnel Release under Carpal Arthroscopy in the treatment of carpal tunnel syndrome can achieve reliable clinical efficacy. It can relieve symptoms such as hand numbness and improve hand function. Postoperative pain and other complications are low, and worthy of clinical promotion.

Late Arteriovenous Fistula after transurethral resection of the prostate: case report

This study describes a case of symptomatic arteriovenous fistula after Transurethral Resection of the Prostate diagnosed by urethrocystoscopy and confirmed by imaging tests. The patient presented lower urinary tract symptoms associated with hematuria twenty years after being submitted to a TURP. Arterial and venous tomography angiography of the pelvis visualized a hypervascularized lesion involving the membranous urethra and aneurysm of the right external iliac artery. The patient underwent embolization of bilateral prostatic arteries with good post-embolization recovery and complete remission of urinary symptoms. Arteriovenous fistula after transurethral resection of the prostate is a rare pathology that must be considered in the differential diagnosis of persistent hematuria and infravesical obstruction without significant prostatic enlargement. In cases like this, embolization has become a new therapeutic option with excellent obliteration rates and minimal morbidity.

JAK inhibitor treatment for inborn errors of JAK/STAT signaling: An ESID/EBMT-IEWP retrospective study

BackgroundInborn errors of immunity (IEI) with dysregulated JAK/STAT signaling present with variable manifestations of immune dysregulation and infections. Hematopoietic stem cell transplantation (HSCT) is potentially curative, but initially reported outcomes were poor. JAK inhibitors (JAKi) offer a targeted treatment option that may be an alternative or bridge to HSCT. However, data on their current use, treatment efficacy and adverse events (AE) are limited. ObjectiveWe evaluated the current off-label JAKi treatment experience for JAK/STAT IEI among ESID/EBMT-IEWP centers. MethodsMulticenter retrospective study on patients with a genetic disorder of hyperactive JAK/STAT signaling, who received JAKi treatment for at least 3 months. ResultsSixty-nine patients (72% children) were evaluated (45 STAT1-GOF, 21 STAT3-GOF, 1 STAT5B-GOF, 1 SOCS1-LOF, 1 JAK1-GOF). Ruxolitinib was the predominantly prescribed JAKi (80%). Overall, treatment resulted in improvement (partial or complete remission) of clinical symptoms in 87% of STAT1-GOF and in 90% of STAT3-GOF patients. We documented very heterogeneous dosing and monitoring regimens. The response rate and time to response varied across different diseases and manifestations. AE (i.e. infections and weight gain) were frequent (38% of patients), but mild (grade I-II) and transient in most patients. At last follow-up, 52/69 (74%) of patients are still receiving JAKi treatment, while 11 patients eventually underwent HSCT following previous JAKi bridging therapy with a 91% overall survival. ConclusionsOur study suggests that JAKi may be highly effective to treat symptomatic JAK/STAT-IEI patients. Prospective studies to define optimal JAKi dosing for the variable clinical presentations and age ranges should be pursued.

Management of congenital urogenital and perineal vascular malformations: correlation of clinical findings with diagnostic imaging for treatment decision.

Analysis of clinical and diagnostic findings in rare urogenital and perineal vascular malformations only occurring in 2-3 % of vascular anomalies with regard to clinical symptoms and treatment decisions. All 25 out of 537 patients presenting with congenital urogenital and perineal vascular malformations at our institution from 2014 to 2021 were included. Vascular anomaly classification, anatomical location, clinical symptoms at presentation, diagnostic imaging, and pain intensity were retrospectively assessed from the patient record and therapy management was evaluated. In total, 25 patients (10 females (40 %), 15 males (60 %)), aged 6 to 77 years were included. Diagnoses were: 10 (40 %) venous malformations (VMs), 5 (20 %) lymphatic malformations (LMs) and 10 (40 %) arteriovenous malformations (AVMs). Malformation manifestations were: 12 (32 %) lesser pelvis, 12 (32 %) external genitalia, and 13 (34 %) perineal/gluteal region. One AVM was located in the kidney. The leading clinical symptom was pain. The mean intensity was 6.0/10 for LM, 5.7/10 for VM, and 4.5/10 for AVM. Further major symptoms included physical impairment, local swelling, and skin discoloration. Bleeding complications or sexual dysfunction were rare findings. Patients with VM reported significantly more symptoms than patients with AVM (p = 0.0129). In 13 patients (52 %) minimally invasive therapy was indicated: 10 (77 %) sclerotherapies and 3 (23 %) transcatheter embolization procedures. Complete symptomatic remission was achieved in 9 (69 %) patients, partial response in 3 (23 %) patients, and 1 patient showed no clinical response to therapy. Follow-up appointments without the need for immediate minimally invasive therapy were significantly more common in patients with AVMs than in patients with VMs (p = 0.0198). To create a higher awareness of congenital urogenital and perineal vascular malformations. Awareness of this rare condition avoids misdiagnosis. Therapy decisions should be symptom-oriented. Emergency intervention is rarely required, even in fast-flow vascular malformations. · Venous malformations cause more symptoms with higher pain intensity than arteriovenous malformations.. · Diagnosis and adequate treatment can be hampered by a lack of awareness of the clinical presentation.. · Bleeding complications are rare, even in high-flow vascular malformations.. · Pain and physical impairment are the most commonly observed symptoms in these patients..

Functional Movement Disorder; Importance of Proper Diagnosis and Treatment: A Case Report

Background: Dystonia is a common movement disorder with a wide range of aetiologies. Delays in the identification and initiation of effective treatments should be minimized to improve patient pain and optimize outcomes. This case report aims to underscore the successful treatment of chronic dystonia with the use of mood-modifying serotonin and norepinephrine reuptake inhibitors (SNRI), and encourage clinicians to consider a diagnosis of functional (psychogenic) movement disorder in patients with dystonia that is refractory to usual treatment.
 Case Report: This case report describes a 40-year-old woman who presented to a chronic pain clinic for pain related to cervical dystonia with associated head tremor. Her symptoms were refractory to nearly a decade of quarterly botulinum toxin injections. Based on careful evaluation of the patient’s history, a normal neurological examination, increased Generalized Anxiety Disorder Scale (GAD-7), Patient Health Questionnaire-9 (PHQ-9), and Injustice Experiences Questionnaire (IEQ) scores, and unsuccessful symptom management with botulinum toxin A, a diagnosis of functional movement disorder (FMD) was made. Low-dose Cymbalta was initiated. The patient achieved near complete symptom remission and resolution of her chronic pain within 2 months and achieved near complete resolution in 2 years.
 Conclusion: A diagnosis of FMD should be considered in all patients with dystonia, but especially in patients who respond inadequately to botulinum toxin injections or other rehabilitation therapies. The treatment of comorbid psychiatric conditions can result in substantial benefits and remission from dystonia due to FMD.

Long-term clinical and radiological outcomes of endoscopic ultrasound-guided radiofrequency ablation of benign insulinomas.

In recent years, endoscopic ultrasound-guided radiofrequency ablation (EUS-RFA) has emerged as an alternative nonsurgical treatment for pancreatic neuroendocrine tumours. The aim of our study was to assess the long-term follow-up of patients treated with EUS-RFA for a sporadic insulinoma in our centre in terms of efficacy, safety and risk of recurrence. We retrospectively analysed the data of 11 patients with an insulinoma treated by EUS-RFA in our tertiary centre between June 2018 and April 2022. Clinical and biological, as well as imaging, follow-up was planned at 3, 6, 12 months and then annually. In our series, there were nine women and two men with a median age of 65 years. All tumours were sporadic, with a mean size of 11 mm. The procedure allowed an immediate and complete symptomatic and biological remission in all patients without notable complications. Complete radiological resolution of the tumour after ablation was observed in seven patients, and persistence of an asymptomatic tumour residue was observed in four patients. During the mean follow-up period of 26 months, two patients presented a significant but asymptomatic increase of the tumour residue; a second EUS-RFA session was performed in one patient and the other patient is being closely monitored. EUS-RFA treatment of benign insulinomas provides a long-term complete clinical resolution of hypoglycaemia. A long-term follow-up is essential if residual tumour persists after initial EUS-RFA treatment.

Evaluating the Effectiveness of Combination Antibiotic Therapy for Recurrent Cystitis in Women

Background: Investigating the Effectiveness of Combined Antibiotic Therapy for Recurrent Cystitis in Females. A study was conducted on twenty women with recurrent cystitis who received combination antibiotic therapy. The primary outcome measure for cystitis was the absence of all clinical and laboratory indicators of infection. The secondary objective was to assess symptom improvement related to cystitis. The findings revealed that, after using the combination antibiotic medication, cystitis symptoms disappeared completely in 18 out of the 20 women (90%), while 2 women (10%) experienced symptom improvement. According to these results, combination antibiotic therapy seems safe and effective for women with recurrent cystitis. Objective: The primary aim of this study was to evaluate the curative effects of combination antibiotic therapy in female repetitive cystitis (RC) patients who presented at the Main Gul Jehanzeb Hospital, Swat, during January 2018 and January 2019. Secondarily, we wanted to find out how well combination antibiotic therapy worked for female RC patients. Study Design: A retrospective study Duration and place of study: Main Gul Jehanzeb Hospital, Swat, from January 2018 to January 2019. Methods: A retrospective study of combination antibiotic therapy for curing recurrent cystitis in female patients was carried out at the Main Gul Jehanzeb Hospital, Swat, Pakistan. All women aged between 18 and 50 years who had a history of repeated cystitis, if they received medical treatment in the hospital's outpatient department during research periods and are outpatients now for one research visit, were included on this study. The drug cooperation mode of general practice co-operation network was used, while the principle of vaccination prevention was implemented to everyone for education against sexually transmitted diseases. In the clinic, clinical and laboratory diagnostics were utilized to make a comprehensive diagnosis and all cases were gave a standard questionnaire designed by research personnel. Phase 1 and Phase 2 of treatment: After a comprehensive medical history appraisal and physical examination, each patient received specific combination antibiotic therapy according to her diagnosis. The main purpose of our study was to obtain complete symptomatic remission of cystitis, as gauged by the absence of any clinical or laboratory evidence of infection. Additionally, a secondary object was to alleviate cystitis symptoms, or allay them to some extent. Results: In this study, 20 females who had RC were included. After receiving a combination of antibiotics, 18 of them (90%) had their cystitis symptoms completely eliminated, and the other 2 (10%) only had milder symptoms. Conclusion: This study has demonstrated that combined antibiotic therapy is safe and effective as a treatment for female recurrent cystitis. In recurrent cystitis, most women obtain relief from their symptoms using mixed antibiotics. Keywords:Recurrent Cystitis, Combination Antibiotic Therapy, Women

FRI509 A Girl With Trisomy 21 Presents With Van Wyk-Grumbach Syndrome

Abstract Disclosure: J. Hayes dorado: None. M. Calla Ayala: None. A. Colque Arias: None. K. Lanuza Gaite: None. M. Rossell Lopez: None. D. Frias Eid: None. Van Wyc-Grumbach syndrome (VWGS) is a rare presentation of long standing prepubertal hypothyroidism characterized by bilateral multicystic enlarged ovaries, vaginal bleeding and delayed bone age. It appeared that high TSH could produce FSH and LH like activity leading to multiple ovarian cysts. A 7-year-old girl with trisomy 21, presented with vaginal bleeding for 2 days prior to admission. Physical exam revealed bradycardia (68 bbm), apathy, sluggishness, as well as dry skin and eyelid oedema. She had no breast development nor pubic hair.Causes of vaginal bleeding such as trauma, possible abuse, foreign body insertion and urethral prolapse were excluded. There was no purpura or bleeding from other sites.The abdominal sonogram revealed bilateral ovarian cystic masses (V:17.29 cm3 and 16.48 cm3, right and left, respectively). Bone age was delayed almost 3 years behind her chronological age. Laboratory tests revealed TSH 860 μIU/mL, T4 0.71 μg/dL, FSH 8.12 mIU/mL, LH < 0.1 mIU/mL, estradiol 193 pg/mL, thyroid peroxidase Abs 184 IU/mL, thyroglobulin Abs 212 IU/mL, Β-hCG < 0.1mU/mL, confirming severe hypothyroidism. The girl was diagnosed with VWGS due to unrecognized chronic lymphocytic thyroiditis and LT4 treatment (75mug/OD) was initiated. The patient responded with complete remission of symptoms and regression of the ovarian cysts one month after initiation of treatment. It is very important to think of VWGS and investigate for thyroid status during the evaluation of ovarian cysts or isolated premature menarche. Early recognition can eliminate unnecessary surgery to remove ovarian cysts, as appropriate treatment with levothyroxine leads to complete remission of symptoms. Even when the diagnosis of VWGS is confirmed, practitioners must be watchful to consider surgical intervention in the presence of uncontrolled vaginal bleeding, hemodynamic instability, or failure of regression of ovarian cysts with exogenous thyroid hormone replacement. Presentation: Friday, June 16, 2023

The Predictors of Long COVID in Southeastern Italy.

Long COVID is now recognized as a common consequence of the SARS-CoV-2 infection, but we are still far from fully understanding its pathogenesis and predictive factors. Many pathophysiological factors have been studied, including ethnicity. To our knowledge, the risk factors for Long COVID have not been studied in Southeastern Italy. The aim of this study was to evaluate the predictive factors of Long COVID in a cohort of patients from Southeastern Italy. We conducted a retrospective longitudinal study, enrolling inpatients and outpatients diagnosed with COVID-19 from June 2021 to March 2022. A total of 436 subjects were evaluated in an outpatient setting 12 weeks after a SARS-CoV-2 infection, recording comorbidities, symptoms, therapy, and clinical information. Univariate and multivariate binomial logistic regression analyses were performed on different risk factors to define the probability of developing Long COVID. A total of 71.8% of patients (313) developed Long COVID, while the remaining 123 (28.3%) had a complete remission of symptoms 3 months after acute infection. During the acute phase of COVID-19, 68.3% of patients experienced respiratory failure and 81.4% received corticosteroid therapy. In a multivariate analysis, the female sex (SEX M ODD 0.513) and corticosteroids (ODD 2.25) were maintained as predictive values. From our data and in line with other studies, the female sex emerges as a risk factor for Long COVID in the population of Southeastern Italy. Corticosteroid therapy administered in the acute phase also appears to be associated with an increased risk of Long COVID. Although indications for the prescription of corticosteroid therapy in the acute phase were indicated by the presence of pneumonia complicated by respiratory insufficiency, there was an over-prescription of corticosteroid therapy in the real life of our cohort, with 64% of patients having respiratory insufficiency and 81% having corticosteroid therapy. We hypothesize that a synergistic link between viral infection and the side effects of corticosteroid therapy may arise in selected cases.

Dysregulation of humoral immunity, iron homeostasis, and lipid metabolism is associated with multiple sclerosis progression

BackgroundThough most patients with multiple sclerosis (MS) presented earlier on as a relapsing-remitting (RR) disease, disability progression eventually occurred. Uncovering the mechanisms underlying progression may facilitate the unmet need for developing therapies to prevent progression. Benign MS (BMS), a rare form of MS, is the opposite from secondary progressive MS (SPMS) in that it lacks disease progression defined as Expanded Disability Status Scale (EDSS) ≤3 after at least 15 years of disease onset. BMS is characterized by rare and mild relapses with complete remission of clinical symptoms (lower activity of the disease) and lack of progression. Our study aims to identify transcriptomic and immunological differences between BMS and SPMS to unravel the pathogenesis of disease progression. MethodsWe took multi-modal approaches with microarrays, flow cytometry, and lipidomics by three-way comparisons of patients with BMS vs. RRMS (low disease activity vs. moderate or severe activity), RRMS vs. SPMS (continued activity vs. complete transformation into progressive phase) as well as BMS vs. SPMS, matched for age and disease-duration (low disease activity and no progression vs. progression with or without activity). ResultsWe found that patients with RRMS and SPMS have a significantly higher percentage of B cells than those with BMS. BMS shows a different transcriptomic profile than SPMS. Many of the differentially expressed genes (DEGs) are involved in B cell-mediated immune responses. Additionally, long-chain fatty acids (LCFA), which can act as inflammatory mediators, are also altered in SPMS. Overall, our data suggest a role for the dysregulation of B cell differentiation and function, humoral immunity, and iron and lipid homeostasis in the pathogenesis of MS disease progression. ConclusionBMS has a unique transcriptomic and immunological profile compared to RRMS and SPMS. These differences will allow for personalized precision medicine and may ultimately lead to the discovery of new therapeutic targets for disease progression.

Therapy resistant urticaria as a long-term symptom of an incomplete Schnitzler syndrome

BackgroundRecurring therapy resistant hives, accompanied by IgM-gammopathy, fever and joint pain can indicate Schnitzler syndrome, a rare autoimmune disorder. There is currently no approved treatment, but complete remission of symptoms can be induced with IL-1 antagonists.Case presentationA patient with a history of chronic urticaria presented frequently at the outpatient clinic with severe hives and was treated unsuccessfully with antihistamines and omalizumab. After several years, additional symptoms such as joint pain, recurrent fever, and IgM-gammopathy developed. After the diagnostic criteria for Schnitzler syndrome were met, treatment with anakinra was initiated and resulted in an improvement of the symptoms. Shortly after the first injection, the patient developed large and painful erythematous lesions at the injection sites, leading to discontinuation of treatment and a rapid recurrence of symptoms. Subsequently, treatment with a longer-acting IL-1 antagonist (canakinumab) was initiated, resulting in a complete remission of symptoms.ConclusionThis case report demonstrates that patients with urticarial symptoms that are not relieved by typical treatments should prompt repeated reassessments of the diagnosis, even years later, because gammopathy and other diagnostic criteria for Schnitzler syndrome can occur with a delay.

Eosinophilic Esophagitis Histologic Scoring System: Correlation with Histologic, Endoscopic, and Symptomatic Disease and Clinical Use.

The eosinophilic esophagitis histologic scoring system (EoEHSS) was developed to enhance the diagnostic standard of peak eosinophil count (PEC) in evaluating disease activity in EoE. (1) Correlate the EoEHSS and PEC to measures of symptomatic and endoscopic disease activity, (2) Correlate EoEHSS grade and stage subcomponents to clinical, radiology, and endoscopic markers of fibrotic disease, (3) Evaluate EoEHSS remission in asymptomatic patients with PEC < 15 eosinophils per high powered field (eos/hpf). Secondary analysis of prospective cohort data of 22 patients with EoE that underwent dietary therapy and endoscopy at 3 time points. Active disease was defined by EoEHSS grade or stage > 0.125, symptomatic disease by EoE symptom activity index > 20, endoscopic disease by endoscopic reference score > 2, and histologic disease by PEC ≥ 15 eos/hpf. EoEHSS remission was defined by esophageal inflammation (EI) grade of 0-1, EI stage of 0, total grade ≤ 3, and total stage ≤ 3. EoEHSS grade and stage did not correlate with symptomatic disease but did with endoscopic and histologic disease. PEC showed similar correlation pattern. Abnormal grade and stage had strong sensitivity (87-100%) but poor specificity (11-36%) to detect symptomatic, endoscopic, and histologic disease activity. Lamina propria fibrosis was evaluated in 36% of biopsies and did not correlate with minimum esophageal diameter. Out of 14 patients who were in complete symptomatic, endoscopic, and histologic remission, 8 met criteria for EoEHSS remission. The positive and negative correlations of EoEHSS to specific measures of symptomatic, histologic, and endoscopic activity suggest that it provides complementary information in EoE.

Lesioni ossee simmetriche e drepanocitosi

An 8-year-old boy suffering from sickle cell disease (SCD) was admitted to emergency unit complaining of moderate-to-severe pain in his lower limbs. He received chronic transfusion therapy and hydroxyurea. Apparently, he had never experienced vaso-occlusive crises (VOC) before. At presentation he suffered pain in his left lower limb with mild functional limitation. Standard radiography was negative. Blood tests were consistent with chronic haemolytic anaemia, normal blood cell count and slight increase in inflammation markers. Blood cultures were negative. In the suspicion of a VOC, hydration and antalgic therapy with intravenous acetaminophen and ibuprofen were administered. Due to a poor clinical response, lower limbs MRI was performed and, interestingly, it revealed bilateral symmetrical tibial bone infarctions and analgesics were switched to tramadol in continuous infusion resulting in good pain control. He was discharged after nine days of hospitalization with complete remission of symptoms. &lt;br&gt; SCD is a spectrum of hereditary haemoglobinopathies identified by abnormal haemoglobin S (HbS) molecule. Painful crisis are the most frequent complications and VOCs virtually affect every patient with SCD. MRI can be useful to identify skeletal abnormalities, even in the pain free period and to distinguish acute infarction from acute osteomyelitis. When available, all SCD patients suffering from significant acute bone pain should undergo an MRI study of the painful region as the initial imaging investigation because, even in patients who have never experienced VOCs, there may already be bone lesions at the first painful crisis.

P35 CARDIAC MAGNETIC RESONANCE: PROMISING SOLUTION IN CLINICAL DILEMMA

Abstract There are many clinical conditions in which symptoms and signs can overlap, resulting in a real "clinical dilemma". Differential diagnosis is essential for identifying the pathology affecting a patient and consequently making the best therapeutic decisions. Herein we report a case of a young patient, with strong smoking habit, who looked for medical attention after experiencing chest pain, associated to upper airways inflammation. EKG revealed ischemic abnormalities, echocardiography showed a preserved ejection fraction (EF: 55%) and global cardiac contractility, although akinesia of the mid–segment of the inferolateral wall, and the presence of a mild inferolateral pericardial effusion. A concomitant increase in myocardial necrosis enzymes and inflammatory markers was observed in blood tests, but the coronarography showed undamaged coronary arteries. This clinical picture characterized by the young age of the patient, by the intercurrent inflammatory event, by the presence of pericardial rubbing on physical examination, by the evidence of echocardiographic end EKG anomalies in the absence of documentable epicardial lesions and by the complete symptomatic remission after anti – inflammatory therapy with acetylsalicylic acid and Colchicine was highly suggestive of pseudoinfarct–presenting myopericarditis. To confirm such diagnosis, cardiac magnetic resonance (CMR) was requested, unexpectedly, it showed oedema on the mid–basal inferolateral T2–weighted STIR sequences, but also microvascular obstruction (MVO) on early EGE contrastographic sequences mid–basal inferolateral site, and finally evidence of transmural fibrosis in the same site to the DE sequences resulting in pathognomonic lesions for acute ischemia in absence of epicardial coronaropathy. CMR imaging, which is able to analyse cardiac structure and function and simultaneously provide tissue characterization, has been an essential investigation for the diagnosis of MINOCA, as CMR can readily pinpoint many conditions responsible for myocardial damage and allow pathological discrimination of patients. Our patient‘s final diagnosis was “STEMI, MINOCA, and concomitant pericarditis” implying a consequent therapeutic modification, introducing DAPT. Making differential diagnosis in similar clinical conditions characterized by overlapping presentations, allows the use of personalized therapeutic strategies for each patient and better final results.