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BMC Nephrology Jan 2024Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is caused by mutations in the ubiquitin-activating enzyme1 (UBA1) gene and characterised by an...
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome presenting as recurrent aseptic peritonitis in a patient receiving peritoneal dialysis: a case report.
BACKGROUND
Vacuoles, E1 enzyme, X-linked, autoinflammatory, somatic (VEXAS) syndrome is caused by mutations in the ubiquitin-activating enzyme1 (UBA1) gene and characterised by an overlap between autoinflammatory and haematologic disorders.
CASE PRESENTATION
We reported a case of a 67-year-Japanese man receiving peritoneal dialysis (PD) who had recurrent aseptic peritonitis caused by the VEXAS syndrome. He presented with unexplained fevers, headache, abdominal pain, conjunctival hyperaemia, ocular pain, auricular pain, arthralgia, and inflammatory skin lesions. Laboratory investigations showed high serum C-reactive protein concentration and increased cell count in PD effluent. He was treated with antibiotics for PD-related peritonitis, but this was unsuccessful. Fluorine-18-fluorodeoxyglucose (FDG) positron emission tomography/computed tomography images demonstrated intense FDG uptake in his left superficial temporal artery, nasal septum, and bilateral auricles. The working diagnosis was giant cell arteritis, and he was treated with oral prednisolone (PSL) 15 mg daily with good response. However, he was unable to taper the dose to less than 10 mg daily because his symptoms flared up. Since Tocilizumab was initiated, he could taper PSL dose to 2 mg daily. Sanger sequencing of his peripheral blood sample showed a mutation of the UBA1 gene (c.122 T > C; p.Met41Thr). We made a final diagnosis of VEXAS syndrome. He suffered from flare of VEXAS syndrome at PSL of 1 mg daily with his cloudy PD effluent. PSL dose of 11 mg daily relieved the symptom within a few days.
CONCLUSIONS
It is crucial to recognise aseptic peritonitis as one of the symptoms of VEXAS syndrome and pay attention to the systemic findings in the patients.
Topics: Humans; Male; Abdominal Pain; Fluorodeoxyglucose F18; Mutation; Myelodysplastic Syndromes; Patients; Skin Diseases, Genetic; Vacuoles; Aged
PubMed: 38212709
DOI: 10.1186/s12882-024-03454-9 -
Acta Dermatovenerologica Croatica : ADC Nov 2023Dear Editor, Although some of my colleagues may find this surprising, as a neurologist, I have noticed many connections between dermatology and neurology. Neurological...
Dear Editor, Although some of my colleagues may find this surprising, as a neurologist, I have noticed many connections between dermatology and neurology. Neurological and dermatological signs and symptoms are common in many clinical entities, especially in the so-called phakomatoses or neurocutaneous syndromes (Von Recklinghausen's disease type 1 and 2, Bourneville-Pringle syndrome, Sturge-Weber syndrome, Von Hippel-Lindau syndrome, Louis-Bar syndrome) (1). The terms "neurodermatitis" and "neurodermatology" also confirm the above. Inspection is the basis of every clinical examination and an integral part of both dermatological and neurological propaedeutics. Therefore, I would like to remind your readers of Frank's sign, another link between dermatology and neurology. Frank's sign is a diagonal earlobe crease (DELC) that extends backwards from the tragus at a 45-degree angle across the lobule to the auricular edge of the ear (Figure 1). It has been described as a dermatological marker for atherosclerosis. Frank's sign is named after Dr. Sanders T. Frank, who observed this crease in 20 patients with coronary artery disease and published his findings in The New England Journal of Medicine in 1973 (2). Although this sign has been known for more than 50 years, it is still not routinely employed in clinical practice. Histopathological examination of DELC-positive earlobes revealed myoelastofibrosis in the arterial vessel at the base of the earlobe, indicating that DELC is not a coincidental finding but is directly related to atherosclerosis (3). Following the finding of DELC in patients with coronary artery disease, numerous studies have confirmed the presence of DELC in peripheral vascular disease as well as cerebrovascular disease. I encountered the description of this sign as a student in the textbook of Internal Medicine in 1991 (4). This sign was also the subject of research by Croatian authors. In 1998, Mirić et al. found that a positive Frank's sign carried a higher risk of heart attack (5,6). In 2008, Glavić et al. found a statistically significant association between Frank's sign and an increase in intima media thickness (IMT) of the common carotid artery as a surrogate marker of atherosclerosis, thus confirming the hypothesis that Frank's sign is an uncontrollable risk factor for cerebrovascular disease (such as gender or age) (7). In clinical practice, earlobe inspection should be considered an integral part of the physical examination. In the case of a positive Frank's sign, a color Doppler ultrasound examination of the neck arteries and a cardiologist's examination are recommended. The determination of Frank's sign can be used as a method of primary prevention for cardiovascular and cerebrovascular diseases.
Topics: Humans; Coronary Artery Disease; Ear, External; Carotid Intima-Media Thickness; Cerebrovascular Disorders; Atherosclerosis; Neurology
PubMed: 38006371
DOI: No ID Found -
Folia Morphologica Nov 2023The anatomy of the posterior auricular artery (PAA) is highly variable and relevant in various plastic and reconstructive procedures.
BACKGROUND
The anatomy of the posterior auricular artery (PAA) is highly variable and relevant in various plastic and reconstructive procedures.
MATERIALS AND METHODS
The results of 55 consecutive patients who underwent head and neck computed tomography angiography (CTA) were analyzed. A total of 78 hemifaces were evaluated. The analysis has been performed in 19 categories.
RESULTS
Median PAA length was found to be 47.59 mm (LQ = 32.75; HQ = 56.16). The median PAA diameter (at its origin) was established at 2.55 mm (LQ = 2.29; HQ = 2.90). Moreover, the median PAA cross-sectional area (at its origin) was set to be 3.22 mm (LQ = 2.49; HQ = 4.13). Sexual dimorphism regarding all of the measured parameters was also evaluated. Statistically significant differences (p ≤ 0.05) were found in 13 of the measured categories.
CONCLUSIONS
The present study demonstrated the complete anatomy of the PAA. The most frequent origin of the said artery was from the ECA, and its mean length was 45.07 mm; which did not differ between males and females significantly (p>0.05). Moreover, we have provided surgeons with tools to localize this artery pre- and intraoperatively using simple landmarks, namely the apex of the mastoid process and the center of the external acoustic meatus. The exact position of the origin of the PAA was also demonstrated by a heat map of the auricular region. Our findings have the potential to assist surgeons in developing a mental visualization of the arterial anatomy of the retroauricular region. This visualization can be instrumental in precisely identifying the location of the PAA during reconstructive surgeries, thereby minimizing complications and enhancing surgical outcomes.
PubMed: 37957932
DOI: 10.5603/fm.96993 -
Aesthetic Plastic Surgery Nov 2023There have been many reports on replantation of complete auricle amputation, but few reports on successful replantation of partial auricle amputation. The main reason is...
BACKGROUND
There have been many reports on replantation of complete auricle amputation, but few reports on successful replantation of partial auricle amputation. The main reason is that the diameter of blood vessels at the end of auricle is only 0.3 mm, and it is difficult to find suitable blood vessels, especially venous vessels. The purpose of this study was to investigate the method of revascularization after partial auricle amputation.
METHODS
Microvascular repairs were performed in an amputated segment with only identified artery vessels for anastomosis, and vein was unavailable for anastomosis. Postoperative acupuncture bloodletting and heparin compress treatments were planned.
RESULTS
Two patients with partial ear amputation were treated at our center between 2019 and 2021. All the amputated ear were replanted successfully. No blood transfusions and no infections were observed. A week later the replanted auricles were seen, blood flow established.
CONCLUSION
Microvascular repair should be considered as the best options in cases of auricular avulsion segment replantation. When no vein was available for anastomosis, only one artery repaired was feasible. Acupuncture bloodletting and heparin compress are the effective methods to treat vein congestion.
LEVEL OF EVIDENCE V
This journal requires that authors assign a level of evidence to each article. For a full description of these Evidence-Based Medicine ratings, please refer to the Table of Contents or the online Instructions to Authors www.springer.com/00266 .
PubMed: 37957395
DOI: 10.1007/s00266-023-03737-w -
Microsurgery Jan 2024
Topics: Humans; Free Tissue Flaps; Plastic Surgery Procedures; Head
PubMed: 37953651
DOI: 10.1002/micr.31132 -
BMC Cardiovascular Disorders Nov 2023The impact of sex hormones on right and left auricular contractile apparatus function is largely unknown. We evaluated the impact of sex hormones on left and right heart...
BACKGROUND
The impact of sex hormones on right and left auricular contractile apparatus function is largely unknown. We evaluated the impact of sex hormones on left and right heart contractility at the level of myocardial filaments harvested from left and right auricles during elective coronary artery bypass surgery.
METHODS
150 patients (132 male; 18 female) were enrolled. Preoperative testosterone and estradiol levels were measured with Immunoassay. Calcium induced force measurements were performed with left- and right auricular myofilaments in a skinned fiber model. Correlation analysis was used for comparison of force values and levels of sex hormones and their ratio.
RESULTS
Low testosterone was associated with higher top force values in right-sided myofilaments but not in left-sided myofilaments for both sexes (p = 0.000 in males, p = 0.001 in females). Low estradiol levels were associated with higher top force values in right-sided myofilaments (p 0.000) in females and only borderline significantly associated with higher top force values in males (p 0.056). In females, low estradiol levels correlated with higher top force values in left sided myofilaments (p 0.000). In males, higher Estradiol/Testosterone ratio (E/T ratio) was only associated with higher top force values from right auricular myofilaments (p 0.04) In contrast, in females higher E/T ratio was associated with lower right auricular myofilament top force values (p 0.03) and higher top force values in left-sided myofilaments (p 0.000).
CONCLUSIONS
This study shows that patients' comorbidities influence left and right sided contractility and may blur results concerning influence of sex hormones if not eliminated. A sex hormone dependent influence is obvious with different effects on the left and right ventricle. The E/T ratio and its impact on myofilament top force showed divergent results between genders, and may partially explain gender differences in patients with cardiovascular disease.
Topics: Humans; Male; Female; Myofibrils; Testosterone; Estradiol; Coronary Artery Bypass; Gonadal Steroid Hormones
PubMed: 37925416
DOI: 10.1186/s12872-023-03582-4 -
Journal de Medecine Vasculaire Sep 2023The therapeutic challenge in peripheral arterial occlusive disease (PAD) is often to increase walking distance, improve pain or heal a wound when PAD is symptomatic.... (Review)
Review
UNLABELLED
The therapeutic challenge in peripheral arterial occlusive disease (PAD) is often to increase walking distance, improve pain or heal a wound when PAD is symptomatic. Walking rehabilitation or surgical revascularization techniques are limited. Others strategies as alternatives and/or complementary treatments are needed. Among alternative options, Transcutaneous Electrical Nerve Stimulations (TENS) could be of interest, both for improved walking distance or pain reduction. The Transcutaneous Electrical Nerve Stimulation (TENS) is a non-pharmacological, mini-invasive technique involving transcutaneous electrical stimulation. However, there are other transcutaneous electrical nerve stimulation techniques based on the principle of vagus nerve stimulation with different mechanistics. Trans-auricular Vagus nerve stimulation (Ta-VNS) is another TENS technique (electrode on the external ear) which relies on the anti-inflammatory pathways of efferent and afferent vagal fibers. We propose here to review the literature of mini-invasive electrical stimulations, whatever the anatomical zone concerned, in PAD.
METHOD
The aim was to evaluate the use of non-invasive transcutaneous electrical stimulation therapies (regardless of location) in PAD of the lower limbs, whatever the disease grade. A review of the literature was carried out via a search of the MEDLINE/PubMed database from 1975 to 2023. The articles were selected via abstracts by checking (1) medical indications: PAD patients with claudication were retained, excluding neurological or venous claudication, PAD whatever the disease grade (intermittent claudication or critical limb ischemia [CLI]) and (2) non invasive electrical stimulations were considered (neuromuscular electrical stimulation and spinal cord stimulation were excluded) whatever the anatomical site. Non-electrical stimuli such as acupuncture and reflexotherapy were excluded.
RESULTS
Only 9 items were selected, including 7 studies with TENS treatment on the calf, one with trans-auricular vagus nerve stimulation and one with electro-acupuncture points of stimulation.
CONCLUSION
Even if the mechanisms involved are different, TENS on the calves or in the external ears show an improvement of walking distance in PAD patients with intermittent claudication. The results of the studies show few positive effects in arteriopathy but we should keep vigilant in the technics used since mechanisms are different and not fully understood. Electro-stimulation of the calf and external ear appears to be an easy-to-use and accessible therapeutic option, especially since some PAD patients are still failing to be released from pain, despite the rise of endovascular interventional techniques.
Topics: Humans; Animals; Cattle; Transcutaneous Electric Nerve Stimulation; Intermittent Claudication; Pain; Lower Extremity
PubMed: 37914456
DOI: 10.1016/j.jdmv.2023.10.001 -
Neurosurgical Review Oct 2023Ischemia-induced postoperative scalp necrosis in the superficial temporal artery (STA) region is known to occur after STA-middle cerebral artery anastomoses. However, no...
Ischemia-induced postoperative scalp necrosis in the superficial temporal artery (STA) region is known to occur after STA-middle cerebral artery anastomoses. However, no reports have evaluated the risk of postoperative scalp necrosis in the occipital artery (OA) region. This study examined the surgical procedures that pose a risk for postoperative scalp necrosis in the OA region following posterior cranial fossa surgery. Patients who underwent initial posterior fossa craniotomy at our institution from 2015 to 2022 were included. Clinical information was collected using medical records. Regarding surgical procedures, we evaluated the incision design and whether a supramuscular scalp flap was prepared. The supramuscular scalp flap was defined as a scalp flap dissected from the sternocleidomastoid and/or splenius capitis muscles. A total of 392 patients were included. Postoperative scalp necrosis occurred in 19 patients (4.8%). There were 296 patients with supramuscular scalp flaps, and supramuscular scalp flaps prepared in all 19 patients with postoperative necrosis. Comparing incision designs among patients with supramuscular scalp flap, a hockey stick-shaped scalp incision caused postoperative necrosis in 14 of 73 patients (19.1%), and the odds of postoperative scalp necrosis were higher with the hockey stick shape than with the retro-auricular C shape (adjusted odds ratio: 12.2, 95% confidence interval: 3.86-38.3, p = 0.00002). In all the cases, ischemia was considered to be the cause of postoperative necrosis. The incidence of postoperative necrosis is particularly high when a hockey stick-shaped scalp incision is combined with a supramuscular scalp flap.
Topics: Humans; Scalp; Cranial Fossa, Posterior; Middle Cerebral Artery; Necrosis; Ischemia
PubMed: 37864617
DOI: 10.1007/s10143-023-02189-9 -
International Journal of Surgery Case... Nov 2023Hemifacial microsomia (HFM) is a complex congenital facial anomaly characterized by a wide spectrum of clinical features, which encompass the facial skeleton and other...
INTRODUCTION AND IMPORTANCE
Hemifacial microsomia (HFM) is a complex congenital facial anomaly characterized by a wide spectrum of clinical features, which encompass the facial skeleton and other organ systems. Currently, there is no evidence to suggest an association between Hemifacial Microsomia and vascular malformations, whether of the vertebral or any other kind.
CASE PRESENTATION
Reporting a case of a 12-year-old male diagnosed with Hemifacial Microsomia (HFM) and left Microtia. The patient had previously undergone left auricle reconstruction; however, unfortunately, the flap resulted in necrosis. In our next step, we intend to proceed with further reconstruction. Before this, we plan to perform CT angiography to identify viable flap options for effectively closing the auricular defect. During this evaluation, we identified an anomaly structure in the vertebral vascularization.
CLINICAL DISCUSSION
During the CT angiography, we found a vascular malformation in the vertebral region. This anomaly manifested as tortuosity in the left vertebral vein, with the diameter on the left side being larger than that on the right. Additionally, the diameter of the left internal jugular artery was found to be smaller than its counterpart on the right. The maxillary artery of the left side was larger than the right. Notably, there was an absence of a submental artery on the left side, and a hypoplasia of the left angularis artery was observed.
CONCLUSION
Hemifacial microsomia could be associated with other malformations. Despite the fact that vertebral artery anomaly is not considered common anomaly in HFM, it is mandatory to perform CT angiography before reconstructive surgery, considering the possibility of massive bleeding during the operation.
PubMed: 37837667
DOI: 10.1016/j.ijscr.2023.108906 -
Journal of Neurological Surgery. Part... Oct 2023There are some cases where a radial artery (RA) graft is needed for a high-flow extracranial to intracranial (EC-IC) bypass as the external carotid artery (ECA)...
BACKGROUND
There are some cases where a radial artery (RA) graft is needed for a high-flow extracranial to intracranial (EC-IC) bypass as the external carotid artery (ECA) cannot be utilized as a donor artery. In this report, we describe two cases of extracranial vertebral artery (VA) to middle cerebral artery (MCA) high-flow bypass using an RA graft with an artificial vessel as an alternative bypass technique.
METHODS
The patient was placed supine with a head rotation of 80 degrees. After frontotemporal craniotomy, another C: -shaped skin incision was made at the retroauricular region and the V3 portion of the VA was exposed at the suboccipital triangle. Prior to attempting the high-flow bypass, the superficial temporal artery (STA) was anastomosed to the M4 portion of the MCA as an insurance bypass. The RA graft was anastomosed to the V3 portion of the VA that traveled under the periosteum at the supra-auricular region through an artificial vessel. After RA-M2 anastomosis, an alternative EC-IC bypass, the V3-RA-M2 bypass, was achieved.
RESULTS
Postoperative angiography demonstrated successful graft patency and no perioperative complications were observed in both cases.
CONCLUSIONS
In the cases where a high-flow bypass is required, the V3 portion of the VA is a suitable alternative proximal anastomosis site when the ECA is not a candidate donor. Furthermore, an artificial vessel shows satisfactory protection against graft complications.
PubMed: 37832591
DOI: 10.1055/s-0043-1775989