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IDCases 2024Adult-onset immunodeficiency syndrome is characterized by the presence of anti-interferon-gamma (IFN-γ) autoantibody and the distribution of infections. Here, we...
Adult-onset immunodeficiency syndrome is characterized by the presence of anti-interferon-gamma (IFN-γ) autoantibody and the distribution of infections. Here, we describe bacteremia in a Thai woman who also had anti-IFN-γ autoantibody. The patient was also suffering from osteomyelitis and a peri-orbital abscess. Her symptoms were completely eradicated after surgical intervention and the administration of appropriate antibiotics.
PubMed: 38298220
DOI: 10.1016/j.idcr.2024.e01926 -
Journal Francais D'ophtalmologie Mar 2024To investigate the long-term outcomes of COVID-19-associated rhino-orbito-cerebral mucormycosis (ROCM) patients.
PURPOSE
To investigate the long-term outcomes of COVID-19-associated rhino-orbito-cerebral mucormycosis (ROCM) patients.
METHODS
Retrospective, observational study including all COVID-19 patients who developed ROCM and were referred to our oculoplastic clinic.
RESULTS
Twenty-one patients with COVID-19-associated ROCM were included in this study. Twelve (57.1%) individuals were female with a mean age of 50.7±7.6 years (range 33-59), and nine (38.1%) were male with a mean age of 58.7±14.4 years (range 37-82). Corticosteroids were used in 85.7% of patients, and three patients received no systemic corticosteroids; 76.2% were diabetic and two of these developed new-onset diabetes mellitus (DM) after receiving corticosteroids during their treatment course. The average interval between COVID-19 and the development of ROCM in our subjects was 18.6 days (range 8-46 days). In our series of patients, decreasing vision, proptosis, and periorbital edema constituted the most prevalent presentation, seen in 52.4% of subjects. Endoscopic paranasal sinus debridement was performed a mean of 3.4 times in 95.2%, abscess drainage in one, and orbital exenteration in three (14.2%) patients. Orbital apex and bilateral paranasal sinus involvement were significantly associated with higher mortality, and the overall 18-month survival rate was 52.3%.
CONCLUSION
Based upon common factors among the COVID-19-associated ROCM patients, we presume that DM and drug-induced immunosuppression are two main factors, which may lead to a higher rate of ROCM infection in areas where fungal spores are more likely to be present, such as hospitals.
PubMed: 38696866
DOI: 10.1016/j.jfo.2024.104139 -
Journal of Clinical Immunology Jun 2024Moesin (MSN) deficiency is a recently reported combined immunodeficiency, and few cases have been reported to date. We describe a Chinese patient with a novel mutation...
PURPOSE
Moesin (MSN) deficiency is a recently reported combined immunodeficiency, and few cases have been reported to date. We describe a Chinese patient with a novel mutation causing MSN deficiency and a novel phenotype.
METHODS
Clinical and immunological data were collected. Whole-exome sequencing was performed to identify gene mutations. MSN protein expression and T cell proliferation and activation were determined by flow cytometry. Cell migration was confirmed with a Transwell assay. Autoantibody levels were analyzed using antigen microarrays.
RESULTS
The patient was a 10-year-old boy who presented with recurrent fever, oral ulcers and dermatomyositis-like symptoms, such as periorbital edema, facial swelling, elevated creatine kinase levels, and abnormal electromyography and muscle biopsy results. Epstein-Barr virus (EBV) DNA was detected in the serum, cells and tissues of this patient. He further developed nasal-type NK/T-cell lymphoma. A novel hemizygous mutation (c.68 A > G, p.N23S) in the MSN gene was found. The immunological phenotype of this patient included persistent decreases in T and B lymphocyte counts but normal immunoglobulin IgG levels. The patient had attenuated MSN protein expression and impaired T-cell proliferation and migration. The proportions of Tfh cells and CD21 B cells in the patient were higher than those in the controls. Moreover, 82 IgG and 102 IgM autoantibodies were more abundant in the patient than in the healthy controls.
CONCLUSIONS
The novel mutation N23S is pathogenic and leads to a severe clinical phenotype. EBV infection, tumor, and dermatomyositis-like autoimmune symptoms may be associated with MSN deficiency, further expanding the understanding of the disease.
Topics: Humans; Male; Epstein-Barr Virus Infections; Dermatomyositis; Child; Microfilament Proteins; Mutation; Herpesvirus 4, Human; Exome Sequencing; Immunologic Deficiency Syndromes; Autoantibodies; Phenotype; T-Lymphocytes
PubMed: 38922539
DOI: 10.1007/s10875-024-01755-0 -
The Cleft Palate-craniofacial Journal :... Mar 2024To evaluate the outcomes associated with two techniques of periorbital steroid administration in bilateral fronto-orbital advancement (FOA).
OBJECTIVE
To evaluate the outcomes associated with two techniques of periorbital steroid administration in bilateral fronto-orbital advancement (FOA).
DESIGN
Multi-institutional retrospective chart review.
SETTING
Two high volume, tertiary US craniofacial centers.
PATIENTS, PARTICIPANTS
Patients who underwent FOA between 2012 and 2021.
INTERVENTIONS
Patients were divided into three cohorts based on method of steroid administration. Groups GEL and INJ represent those who received steroids in the form of triamcinolone soaked gelfoam or direct injection of dilute triamcinolone to the frontal/periorbital region, respectively. Group NON did not receive any periorbital steroids.
MAIN OUTCOME MEASURE(S)
Peri-operative outcomes including hospital length of stay and complications were evaluated based on method of periorbital steroid administration. Variables predictive of infectious complications were assessed using stepwise logistic regression.
RESULTS
Four hundred and twelve patients were included in our sample (INJ:249, GEL:87, NON:76). Patients in the INJ group had a higher ASA class ( < .001) while patients in the NON group were significantly more likely to be syndromic ( < .001) and have multisuture craniosynostosis ( < .001). Rate of infectious complications for each cohort were NON: 2.6%, INJ: 4.4%, and GEL: 10.3%. There was no significant difference between groups in hospital length of stay ( = .654) or rate of post-operative infectious complications ( = .061). Increased ASA class ( = .021), increased length of stay ( = .016), and increased intraoperative narcotics ( = .011) were independent predictors of infectious complications.
CONCLUSIONS
We identified a dose-dependent relationship between periorbital steroids and rate of postoperative infections, with key contributions from ASA class, hospital length of stay, and dose of intraoperative narcotics.
PubMed: 38545721
DOI: 10.1177/10556656241241963 -
Cureus Jan 2024A 24-year-old female with a history of intravenous heroin use presented with two weeks of chills, myalgias, and cough and was found to be in acute hypoxemic respiratory...
A 24-year-old female with a history of intravenous heroin use presented with two weeks of chills, myalgias, and cough and was found to be in acute hypoxemic respiratory failure. Subsequent workup revealed the presence of bilateral septic pulmonary emboli and tricuspid valve endocarditis. Several weeks into her hospitalization, she developed periorbital edema and laboratory testing revealed she had developed acute renal failure and nephrotic range proteinuria. A renal biopsy confirmed the diagnosis of IgA-dominant Staphylococcus-associated glomerulonephritis (IgA-SAGN). Early recognition of this newly recognized variant of glomerulonephritis is paramount, as improper treatment may lead to catastrophic consequences.
PubMed: 38380208
DOI: 10.7759/cureus.52680 -
The Journal of International Medical... May 2024Fungal orbital cellulitis is usually seen in immunocompromised individuals, and opportunistic pathogens are the main etiology. We herein report a case of fungal orbital...
Fungal orbital cellulitis is usually seen in immunocompromised individuals, and opportunistic pathogens are the main etiology. We herein report a case of fungal orbital cellulitis due to in a patient with no history of trauma. A 48-year-old man presented to the emergency room of our hospital with a 2-week history of periorbital swelling, conjunctival hyperemia, and chemosis of his right eye. The visual acuity of his right eye was 6/20, and the intraocular pressure was 44 mmHg. The main clinical findings were proptosis of the right ocular globe with conjunctival hyperemia and a palpable infratemporal orbital mass. Laboratory testing failed to detect the presence of a pathogenic infection, and the lesions on computed tomography images resembled those of a malignant tumor of the orbit. The diagnosis was finally confirmed by postoperative pathological examination, and the patient responded favorably to debridement combined with antifungal therapy. Histopathological examination may help to reveal the nature of this disease. Surgical removal of inflammatory lesions can serve as an important diagnostic and treatment method for fungal orbital cellulitis.
Topics: Humans; Male; Middle Aged; Aspergillosis; Immunocompromised Host; Tomography, X-Ray Computed; Antifungal Agents; Orbital Cellulitis; Debridement; Eye Infections, Fungal
PubMed: 38757522
DOI: 10.1177/03000605241239857 -
Clinical Case Reports Jun 2024Immunosuppression from B-acute lymphoblastic leukemia (B-ALL) chemotherapy and a preceding COVID-19 infection may predispose patients to rare complications such as...
Co-occurrence of rhino-orbital mucormycosis and acute lymphoblastic leukemia post-COVID-19 infection in a young adolescent male: A case report from a low middle-income country.
KEY CLINICAL MESSAGE
Immunosuppression from B-acute lymphoblastic leukemia (B-ALL) chemotherapy and a preceding COVID-19 infection may predispose patients to rare complications such as rhino-orbital mucormycosis. Hence, a high index of suspicion should be maintained by physicians (and oncologists) if patients undergoing B-ALL treatment present with orofacial symptoms and ophthalmological manifestations such as peri-orbital swelling, ophthalmoplegia, and loss of vision, suggestive of infection.
ABSTRACT
Mucormycosis is a severe fungal infection that poses significant mortality and morbidity risks, particularly in immunocompromised individuals. We present a rare case of a 16-year-old patient with rhino-orbital mucormycosis following B-acute lymphoblastic leukemia (B-ALL) treatment and concurrent COVID-19 infection. We describe the clinical presentation, diagnosis, treatment, and outcome of this patient, and discuss the possible interactions and implications of these three conditions. A young 16-year-old male patient without significant clinical history was admitted with complaints of low-grade intermittent fever, fatigue, malaise, restlessness, and unexplained weight loss for the past 2 months. A bone marrow biopsy confirmed the diagnosis of B-ALL. Following the diagnosis of B-ALL, the patient underwent initiation of chemotherapy. Following the initial two cycles of chemotherapy, the patient experienced fever and cough and tested positive for COVID-19 infection. Nearly a week later, the patient presented to the chemotherapy emergency department with a clinical picture characterized by a fever up to 39°C associated with left facial swelling, severe headache, purulent rhinorrhea, and foreign body sensation in the ipsilateral nostril. The following day, erythema and left eyelid edema were observed, with ocular opening limitation. The diagnosis was confirmed based on the positive result of polymerase chain reaction for left-sided mucormycosis. Initial administration of liposomal and lipid amphotericin B at 1-1.5 mg/kg/d doses for 4-6 weeks was followed by surgical debridement of necrotic tissue on the left side of the face and nose. Subsequent ophthalmological examinations showed normal conditions of the left eye. The case underscores the importance of heightened clinical suspicion, early diagnosis through imaging and molecular techniques, aggressive multimodal therapy, and close interdisciplinary collaboration for improved outcomes in such rare and challenging clinical scenarios.
PubMed: 38799526
DOI: 10.1002/ccr3.8972 -
Medicine Apr 2024Trigeminal herpes zoster, which comprises 10% to 20% of cases of herpes zoster, often leads to severe pain in the ophthalmic branches. Current treatments, including drug...
INTRODUCTION
Trigeminal herpes zoster, which comprises 10% to 20% of cases of herpes zoster, often leads to severe pain in the ophthalmic branches. Current treatments, including drug therapy and minimally invasive interventions, have limitations; accordingly, there is a need to explore alternative approaches. This study aimed to evaluate the efficacy and safety of computerized tomography (CT)-guided pulsed radiofrequency of the sphenopalatine ganglion in patients with intractable trigeminal herpetic pain.
PATIENT CONCERNS
Three patients with intractable trigeminal ophthalmic zoster neuralgia were studied. All patients complained of bursts of headache, which occurred at least 10 times a day, usually in the periorbital and frontal regions. Conventional treatments, including oral medications and radiofrequency therapy targeting the trigeminal-semilunar ganglion and supraorbital nerve, could not sufficiently provide relief.
DIAGNOSIS
Two patients were diagnosed with herpes zoster in the ocular branch of the trigeminal nerve with conjunctivitis, while one patient was diagnosed with postherpetic neuralgia in the ocular branch of the trigeminal nerve.
INTERVENTIONS
This study employed a novel approach that involved CT-guided radiofrequency regulation of the pterygopalatine fossa sphenopalatine ganglion.
OUTCOMES
In all three patients, pain relief was achieved within 1 to 3 days after treatment. During the follow-up, one patient had pain recurrence; however, its severity was ≈ 40% lower than the pretreatment pain severity. The second patient had sustained and effective pain relief. However, the pain of the third patient worsened again after 2 months. The average follow-up duration was 3 months. None of the enrolled patients showed treatment-related adverse reactions or complications.
CONCLUSION
Our findings indicated that CT-guided radiofrequency regulation of the pterygopalatine fossa sphenopalatine ganglion was a safe and effective intervention for pain in patients with trigeminal ophthalmic zoster neuralgia, suggesting that it may be a therapeutic option if other treatments fail.
Topics: Humans; Herpes Zoster Ophthalmicus; Pulsed Radiofrequency Treatment; Neuralgia; Neuralgia, Postherpetic; Trigeminal Neuralgia; Herpes Zoster; Pain, Intractable; Treatment Outcome
PubMed: 38640323
DOI: 10.1097/MD.0000000000037884 -
Military Medicine Nov 2023Pott's puffy tumor (PPT) describes forehead swelling with associated frontal bone osteomyelitis and a subperiosteal abscess (SPA) requiring a high suspicion index for...
Pott's puffy tumor (PPT) describes forehead swelling with associated frontal bone osteomyelitis and a subperiosteal abscess (SPA) requiring a high suspicion index for optimal outcomes. PPT is a life-threatening complication of frontal sinusitis typically found in adolescents. Our case is one of the youngest in the literature. This report describes a 3-year-old patient who developed multifocal abscesses in the epidural space with frontal and orbital SPA, requiring surgical intervention. Additionally, her course was complicated by a superior sagittal venous thrombosis, a complication commonly associated with PPT. We present an unusual case of orbital SPA and aim to highlight a life-threatening pediatric condition that is often underrecognized.
Topics: Humans; Adolescent; Child; Female; Child, Preschool; Pott Puffy Tumor; Abscess; Frontal Sinusitis; Orbital Cellulitis; Edema
PubMed: 37489872
DOI: 10.1093/milmed/usad291 -
Ear, Nose, & Throat Journal Aug 2023Central retinal artery occlusion (CRAO) is an ophthalmic emergency and has poor visual prognosis. It is commonly found in elderly people and very rare in child. We...
Central retinal artery occlusion (CRAO) is an ophthalmic emergency and has poor visual prognosis. It is commonly found in elderly people and very rare in child. We reported an 8-year-old girl who suffered from acute sinusitis, periorbital swelling, and the visual acuity of her right eye was only light perception. She was diagnosed with CRAO, SPOA (subperiosteal orbital abscess), and acute sinusitis. Emergency treatments including surgery, antibiotics, glucocorticoids, intraocular-pressure-lowering drugs, and vasodilators were taken immediately in order to save the eyesight. The visual acuity of the right eye returned to 20/400. Conclusions: Severe intraorbital complications of acute sinusitis can lead to CRAO. Timely drainage, strong antibiotics, and glucocorticoids are the most effective methods for the treatments.
Topics: Humans; Child; Female; Aged; Abscess; Glucocorticoids; Orbital Cellulitis; Sinusitis; Retinal Artery Occlusion; Acute Disease; Anti-Bacterial Agents; Orbital Diseases
PubMed: 33975449
DOI: 10.1177/01455613211016731