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Doklady. Biochemistry and Biophysics Jun 2024The objectives of the study were to present the experience of diagnosis, management, and therapy with IL-1 inhibitors in patients with Schnitzler's syndrome (SchS)...
The objectives of the study were to present the experience of diagnosis, management, and therapy with IL-1 inhibitors in patients with Schnitzler's syndrome (SchS) according to a multicenter Russian cohort. An observational retrospective study for a 10-year period (2012-2022) involved 17 patients with SchS who were admitted to the hospital or were observed on an outpatient basis (eight women and nine men). The diagnosis of all of them corresponded to the Strasbourg diagnostic criteria. The age of patients ranged from 25 to 81 years (Me 53[46; 56]). The age at the time of the onset of the disease ranged from 20 to 72 years (Me 46[39; 54]), the duration of the disease before diagnosis ranged from 1 to 35 years (Me 6.5[3; 6]), in three patients it exceeded 10 years, in the rest it ranged from 1 to 8 years. Infectious and lymphoproliferative diseases, monogenic AIDs (CAPS, TRAPS, and HIDS) were excluded from all patients at the prehospital stage. The referral diagnosis for all of them was Still 's disease in adults. Clinical manifestations of the disease in all patients included fatigue, lethargy, fatigue, rash, and fever. In all patients, skin elements were urticular and were accompanied by itching in 6 (37.5%) patients. Bone pain was observed in 12 (70.6%) patients; arthralgias, in 16 (94.1%); arthritis, in 9 (52.9%); myalgia, in 7 (41.2%); and weight loss, in 4 (23.5%). Lymphadenopathy was detected in 6 (35.3%) patients; enlarged liver, in 6 (35.3%); pericarditis, in 4 (23.5%); angioedema, in 6 (35.3); redness and dryness in the eyes, in 3 (17.6%); sore throat, in 2 (11.8%); abdominal pain, in 1 (5.9%), distal polyneuropathy, in 2 (11.8%); paraesthesia, in 1 (5.9%); and chondritis of the auricles, in 1 (5.9%). Monoclonal gammopathy was detected in all patients with a secretion level of 2.9-15.1 g/L: IgMk (n = 10, 64.7%), less often IgMλ (n = 2), IgGk (n = 2), IgGλ (n = 1), and IgAλ (n = 1). Ben-Jones protein was not detected in any of them. All patients had an increased level of ESR and CRP. Before inclusion in the study, 16 patients received GCs (94.1%) with a temporary effect that disappeared with dose reduction or cancellation. Seven patients received cDMARDs, including methotrexate (5), hydroxychloroquine (2), and cyclophosphamide (1). All patients received NSAIDs and antihistamines, as well as biologics, including the anti-B-cell drug rituximab (1), monoclonal ABs to IgE omalizumab (2, 1 without effect and 1 with partial effect), IL-1i canakinumab (n = 10, 58.8%) subcutaneously once every 8 weeks, and anakinra (n = 4, 23.5%) subcutaneously daily. The duration of taking anakinra, which was prescribed in the test mode, ranged from 1 week to 2.5 months with a further switch to canakinumab in 3 patients. The duration of taking canakinumab at the time of analysis ranged from 7 months to 8 years. Against the background of treatment with IL-1i, 10 out of 11 (90.9%) patients received a complete response in terms of the clinical manifestations of the disease and a decrease in the level of ESR and CRP within a few days. In one patient, a partial response to the administration of anakinra was detected; however, after switching to canakinumab, the effect of treatment was finally lost. One patient received IL-6i for 8 months with an incomplete effect and a positive dynamics after switching to anakinra. Thus, anakinra was initially prescribed to four patients and changed to canakinumab in two of them; canakinumab was started as the first drug in seven patients. Treatment with anakinra was continued in two patients; with canakinumab, in nine patients. In one patient, due to the persistent absence of relapses, the interval between canakinumab injections was increased to 5 months without signs of reactivation; however, subsequently, against the background of stress and relapses of the disease, the intervals were reduced to 4 months. A healthy child was born by the same patient on the background of treatment. The tolerability of therapy was satisfactory in all patients, no SAEs were noted. SchS is a rare multifactorial/non-monogenic AID that should be differentiated from a number of rheumatic diseases and other AIDs. The onset in adulthood, the presence of recurrent urticarial rashes in combination with fever and other manifestations of a systemic inflammatory response are indications for examination for monoclonal secretion. The use of short- or long-acting IL-1i is a highly effective and safe option in the treatment of such patients.
PubMed: 38861148
DOI: 10.1134/S1607672924700923 -
Frontiers in Psychiatry 2024Catatonia is a complex neuropsychiatric syndrome involving a constellation of psychomotor disturbances including catalepsy, waxy flexibility, stupor, mutism, negativism,...
Catatonia is a complex neuropsychiatric syndrome involving a constellation of psychomotor disturbances including catalepsy, waxy flexibility, stupor, mutism, negativism, agitation, posturing, stereotypes, mannerisms, grimacing, echolalia, and echopraxia. Catatonia occurs in several conditions including psychotic, affective and neurodevelopmental disorders such as autism spectrum disorder (ASD). ASD is a neurodevelopmental disorder characterized by persistent deficits in communication, social interaction, restricted interests, repetitive behaviours and sensory sensitivities. Catatonia can occur in response to life stressors such as extreme fear or threat, interpersonal conflict, tragic events or following significant loss. Those with ASD may be particularly vulnerable to the negative impact of stressors and the link between catatonia and ASD is being increasingly recognized. The overlapping features of catatonia and ASD make it difficult to differentiate often resulting in delayed or missed diagnosis. Catatonia in ASD remains a significant clinical challenge; it is difficult to diagnose and can pose debilitating difficulties for those affected. Catatonia is a treatable condition and prompt recognition is vital in securing the best possible outcome. We report a complex and unique case of a 15-year-old boy who presented with severe cognitive and functional decline with a background history of significant bullying and deterioration in his mental state. This case posed a diagnostic conundrum leading to a diagnosis of underlying ASD, anxiety and trauma.
PubMed: 38859882
DOI: 10.3389/fpsyt.2024.1386949 -
Cureus May 2024Long-standing overt ventriculomegaly in adults (LOVA) is a kind of chronic hydrocephalus that has been reported to have started in infancy and is characterized by...
Long-standing overt ventriculomegaly in adults (LOVA) is a kind of chronic hydrocephalus that has been reported to have started in infancy and is characterized by severe ventriculomegaly and macrocephaly. It often manifests clinically in later adulthood. We describe the case of a 34-year-old male patient who had a history of chronic alcoholism and who had been complaining of headaches, disturbed gait, and frequent falls for three months when he arrived in a stupor at the emergency room. Massive ventriculomegaly with Evans' index of 0.40 was found during a head magnetic resonance imaging (MRI). The MRI results were more severe than the clinical manifestations. He was diagnosed with LOVA and treated with conservative hyperosmolar drugs, neuroprotective agents, and intravenous (IV) thiamine. The patient was discharged and consented to follow-up after a hospital stay of seven days.
PubMed: 38854239
DOI: 10.7759/cureus.59973 -
European Journal of Case Reports in... 2024Fournier's gangrene represents a life-threatening necrotising infection affecting the perineal region, while hidradenitis suppurativa is characterised by a chronic...
BACKGROUND
Fournier's gangrene represents a life-threatening necrotising infection affecting the perineal region, while hidradenitis suppurativa is characterised by a chronic inflammatory skin condition. The simultaneous occurrence of both conditions is exceedingly rare.
CASE DESCRIPTION
A 42-year-old female with a documented history of severe untreated hidradenitis suppurativa presented for shortness of breath, fever and lethargy, along with extensive wounds and skin breakdown involving the left axilla, perineum, lower back, lumbosacral region and bilateral gluteal areas, extending to the perineum. Upon presentation, the patient was in a state of septic shock, and a diagnosis of actively manifesting Fournier's gangrene was established at the site of the pre-existing hidradenitis suppurativa lesions. Despite the implementation of an aggressive multidisciplinary approach incorporating surgical interventions, antibiotic therapy and intensive care measures, the patient's condition deteriorated, culminating in septic shock, multi-organ failure and eventual demise. In this report, we discuss both clinical entities, their similarities and differences, and the possible mechanisms by which they may have co-occurred.
CONCLUSION
The co-existence of hidradenitis suppurativa and Fournier's gangrene poses unique challenges, given the rapid progression of Fournier's gangrene within the context of hidradenitis suppurativa, potentially suggesting the latter as a predisposing factor. This case underscores the importance of vigilant screening and management of hidradenitis suppurativa.
LEARNING POINTS
Clinicians should be aware of the potential association between hidradenitis suppurativa and Fournier's gangrene, especially in patients with shared risk factors.Both conditions present diagnostic and treatment challenges, emphasising the importance of a thorough differential diagnosis and a tailored selection of antibiotics.Proactive and continuous care is crucial in managing chronic diseases such as hidradenitis suppurativa to prevent severe complications, for example Fournier's gangrene.
PubMed: 38846649
DOI: 10.12890/2024_004493 -
The Journal of Neuropsychiatry and... Jun 2024
PubMed: 38835224
DOI: 10.1176/appi.neuropsych.20230194 -
Cureus May 2024Staphylococcus lugdunensis is a gram-positive, coagulase-negative organism, typically found in the normal skin flora, predominantly colonizing the perineal region. It...
Staphylococcus lugdunensis is a gram-positive, coagulase-negative organism, typically found in the normal skin flora, predominantly colonizing the perineal region. It has gained recognition as an opportunistic pathogen capable of causing severe infections. This manuscript presents a case study of a 75-year-old female with multiple comorbidities, including hypertension, hyperlipidemia, atrial fibrillation on Xarelto, type 2 diabetes mellitus, hypothyroidism, and a bioprosthetic aortic valve. The patient exhibited symptoms of fever, chills, and lethargy following a dog scratch that resulted in wounds on the left lower extremity. Despite initial negative findings in the drug screen and unremarkable workup for other infectious etiologies, the patient's clinical course revealed the presence of S. lugdunensis in the blood cultures. Timely intervention with broad-spectrum intravenous antibiotics and a six-week course of cefazolin led to significant improvement without recurrence. Staphylococcus lugdunensis, previously considered a relatively benign microorganism, has become a significant player in infectious diseases, particularly causing skin and soft tissue infections and infective endocarditis (IE). It is considered an aggressive pathogen, especially in chronic immunocompromised personnel, with a high potential for morbidity and mortality. S. lugdunensis was found to be the fourth most common cause of IE. The manuscript discusses the epidemiology, clinical presentation, and management of S. lugdunensis infections, emphasizing the importance of early recognition and treatment to prevent potentially fatal outcomes.
PubMed: 38832147
DOI: 10.7759/cureus.59648 -
Frontiers in Veterinary Science 2024Chylopericardium is a rare entity in veterinary medicine. In this report we document the development of chylopericardium in two dogs undergoing chronic hemodialysis. An...
Chylopericardium is a rare entity in veterinary medicine. In this report we document the development of chylopericardium in two dogs undergoing chronic hemodialysis. An 11-year-old female spayed Labrador retriever (Case 1) presented with acute coughing and lethargy 2 months following initial dialysis catheter placement and initiation of dialysis therapy for severe azotemia. Echocardiography demonstrated severe pericardial effusion and cardiac tamponade. Pericardial fluid analysis was consistent with chylous effusion. The dog underwent a subtotal pericardiectomy with thoracic duct ligation, and a PleuralPort™ was placed. The patient continued to receive outpatient hemodialysis therapy after pericardiectomy for several months until she died acutely at home. A 4-year-old male neutered Doberman (Case 2) was being treated for 2 months with outpatient hemodialysis for management of chronic kidney disease. On presentation for the 17th hemodialysis treatment, the patient had increased respiratory rate. Echocardiography demonstrated pleural and pericardial effusions, and fluid analysis in both cavities was consistent with chylous effusion. Use of tissue plasminogen activator (TPA), clot removal and replacement of the catheter was attempted; however pleural and pericardial effusion continued. The patient was euthanized after 25 hemodialysis sessions as owners elected not to pursue more procedures. In both cases, the cause of the chylopericardium was suspected to be secondary to catheter-associated thrombosis and/or stenosis based on multiple imaging modalities. Despite use of rivaroxaban and clopidogrel concurrently in each case, the chylous effusion persisted. This case report describes clinical details of a rare complication of long-term indwelling dialysis catheters in two dogs.
PubMed: 38831956
DOI: 10.3389/fvets.2024.1386710 -
Journal of Medical Virology Jun 2024There is a lack of evidence on the optimal administration of intravenous (IV) fluids in hospitalized adult dengue patients without compensated and hypotensive shock....
There is a lack of evidence on the optimal administration of intravenous (IV) fluids in hospitalized adult dengue patients without compensated and hypotensive shock. This study utilized a well-established cohort of dengue patients to compare risks of progressing to severe dengue (SD) over time for patients who were administered IV fluid versus others who were not. We included adult patients (n = 4781) who were hospitalized for dengue infection from 2005 to 2008. Cases were patients who developed SD (n = 689) and controls were patients who did not up until discharge (n = 4092). We estimated the hazard ratios (HRs) and risk of SD over time between groups administered different volumes of IV fluids versus the no IV fluid comparison group using Cox models with time-dependent covariates. The doubly-robust estimation approach was used to control for the propensity of fluid administration given clinical characteristics of patients. Subgroup analyses by age, sex, and dengue warning signs before IV fluid administration were conducted. High (>2000 mL/day) IV fluids volume was associated with a higher risk of development of SD for those who had warning signs (HR: 1.77 [1.05-2.97], p: 0.0713) and for those below 55 years old (HR: 1.53 [1.04-2.25], p: 0.0713). Low (<1000 mL/day) IV fluids volume was protective against SD for patients without warning signs (HR: 0.757 [0.578-0.990], p: 0.0883), no lethargy (HR: 0.770 [0.600-0.998], p: 0.0847), and females (HR: 0.711 [0.516-0.980], p: 0.0804). Over the course of hospitalization, there were no significant differences in IV fluid administration and SD risk in most subgroups, except in those who experienced lethargy and were administered IV fluid volume or quantity. Administering high volumes of IV fluids may be associated with an increased risk of SD during hospitalization for adult dengue patients without shock. Judicious use of IV fluids as supportive therapy is warranted.
Topics: Humans; Male; Female; Fluid Therapy; Adult; Middle Aged; Administration, Intravenous; Hospitalization; Severe Dengue; Young Adult; Dengue; Aged; Adolescent; Retrospective Studies
PubMed: 38828952
DOI: 10.1002/jmv.29726 -
Pediatrics in Review Jun 2024
Topics: Humans; Male; Lethargy; Infant; Diagnosis, Differential
PubMed: 38821898
DOI: 10.1542/pir.2022-005910 -
Knee Surgery, Sports Traumatology,... May 2024Arthroscopic shoulder surgery is an orthopaedic technique that involves the use of normal saline or hyperosmolar serums as irrigation. The mentioned operation is...
Arthroscopic shoulder surgery is an orthopaedic technique that involves the use of normal saline or hyperosmolar serums as irrigation. The mentioned operation is commonly regarded as a safe medical intervention. Occasionally, it may have serious repercussions for the patient. Fluid extravasation into muscle tissues and tissue injury and instability are possible consequences. This can be affected by the type and amount of serum used and the length of the surgery. The objective of this study was to document four cases of shoulder arthroscopy in which sterile distilled water, wrongly labelled as irrigation fluid, was utilized during the surgical procedure. Patients were readmitted a week after discharge due to acute kidney injury symptoms like fatigue and lethargy. All four patients were released after haemodynamic stability and normalization after haemodialyses. Due to the incident, serums should be closely monitored and labeled for the safety of patients. Additionally, distilled water as an irrigation solution in arthroscopic surgeries can harm patients. Although normal saline and hyperosmolar serums are unavailable, this remains true. LEVEL OF EVIDENCE: Level IV.
PubMed: 38819936
DOI: 10.1002/ksa.12285