Mesenchymal stem cell therapy in eosinophilic granulomatosis with polyangiitis-related lower limb gangrene: a case report.

BACKGROUND: Eosinophilic granulomatosis with polyangiitis (EGPA), a rare but life-threatening systemic vasculitis, is distinguished by marked eosinophilia and presents with diverse symptoms, including asthma, cutaneous purpura, ecchymosis, skin necrosis, cardiac lesions, peripheral neuropathy, and necrotizing vasculitis. The etiology of EGPA involves a complex interaction among humoral, adaptive, innate, and allergic immune responses. Standard treatment employs prolonged high-dose glucocorticoid therapy, which is critical for survival; however, some patients' symptoms cannot be relieved. CASE REPORT: This case report details the medical management of an 11-year-old patient with EGPA, who was at risk of bilateral lower limb amputation due to differential arterial occlusion and severe, necrotizing vasculitis-induced gangrene in both feet. Treatment modalities administered included systemic infusion of Umbilical Cord Mesenchymal Stem Cells (UC-MSCs), targeted gastrocnemius muscle injections, and application of a Placenta-Derived Mesenchymal Stem Cells (PD-MSCs) hydrogel. RESULTS: After receiving a four-month regimen of allogeneic mesenchymal stem cell therapy via intravenous and local administration, the patient showed normalized eosinophil counts, reestablished blood flow in the dorsal arteries, and marked improvement in foot ulcerations. CONCLUSION: Mesenchymal stem cell therapy is a promising option for severe EGPA cases refractory to glucocorticoids.

Mixed Cryoglobulinemia Syndrome Associated with Non-HCV B-Cell Lymphoproliferative Disorder Presenting with Gangrene and Peripheral Neuropathy.

Mixed cryoglobulinemia is a rare disorder characterized by gangrene, weakness, and arthralgias with variable organ involvement. It is often associated with hepatitis C, HIV, and immunological disorders. Diagnosis is based on clinical features and laboratory testing with serology detecting cryoglobulins. Our patient, a 64-year-old female, presented with weakness, fatigue, and discoloration of her fingers and toes. Physical examination showed upper- and lower-extremity skin changes with dry gangrene. Serology showed a non-hepatitis C status, positive cryoglobulin test with a positive rheumatoid factor, and monoclonal IgM-kappa, confirming the diagnosis of mixed cryoglobulinemia. She was treated with intravenous immunoglobulins, glucocorticoids, multiple cycles of rituximab, cyclophosphamide, and plasma exchange. Following a significant event of exacerbation and relapse requiring a below-knee amputation, this case report aims to raise awareness among clinicians to consider this as a rare cause of gangrene and peripheral neuropathy in an elderly adult.

Congenital Mesenteric Defect with Transmesenteric Hernia in Children: A Case Series.

ABSTRACT: Transmesentric hernias are a type of internal hernia, in which there is herniation of bowel loops through a defect in the mesentery. They present with a wide variety of symptoms without any specific radiological features, leading to a delay in diagnosis and high mortality rate. Here, we present a case series of four patients with this rare but fatal cause of small bowel obstruction. Three children presented to the emergency department with small bowel obstruction. The other baby was a preterm neonate with an antenatal scan showing small bowel obstruction. All children underwent emergency laparotomy and were found to have a mesenteric defect with herniation and gangrene of the small bowel. Resection anastomosis of the gangrenous segment and closure of the mesenteric defect were done. The differential for small bowel obstruction in children should include transmesentric hernia. Laboratory or imaging investigations are often inconclusive. Timely exploration can save lives in this rare but life-threatening condition.

Identification of risk factors and clinical outcomes for symmetric peripheral gangrene: A new scoring system using a common data model database.

Background: Symmetrical peripheral gangrene (SPG) is a destructive clinical condition where amputation is often the final treatment option. We aimed to identify the predictors of SPG using the common data model (CDM) and propose a new scoring system for predicting hospitalized patients at risk of developing SPG. Methods: A cohort of patients treated with intravenous noradrenaline, epinephrine, and vasopressin between 2011 and 2020 was retrospectively analyzed using the CDM database. The main outcome was amputation performed as a resuscitation measure. We investigated risk factors including demographic characteristics, comorbidities, and preoperative laboratory values. Based on demographic variables such as age and sex, a 1:10 propensity score matching (PSM) was performed. The odds ratio (OR) was calculated using logistic regression analysis. Results: Amputation was performed in 308 (0.4%) patients out of a cohort of 73,902 patients. Age, sex, hypertension, diabetes mellitus (DM), renal disease (RD), heart failure, anemia, hypercholesterolemia, peripheral vascular disease (PVD), and laboratory markers such as albumin, eosinophils, hematocrit, lymphocytes, monocytes, neutrophils, ESR, aPTT, creatinine, and BUN were statistically significant. Logistic regression analysis revealed statistically significant differences in DM (OR 5.51), RD (OR 2.90), PVD (OR 9.67), and cerebrovascular disease (CVD) (OR 0.49). Compared to the group without amputation, logistic regression analysis after matching the age and sex group with 1:10 PSM showed statistically significant results in DM (OR 3.59), RD (OR 2.59), PVD (OR 7.76), and CVD (OR 0.40). Conclusion: Early recognition of high-risk patients may help medical providers prevent severe outcomes, including amputation surgery.

A novel missense variant in CAT gene causing acatalasemia with gangrenous periodontitis (Takahara's disease).

OBJECTIVES: Acatalasemia is a very rare disorder characterized by gangrenous oral ulcerations and is caused by biallelic variants in the CAT gene which encodes the catalase enzyme that decomposes the hydrogen peroxide molecules to remove their toxic effect. We report two siblings from a consanguineous Egyptian family presenting with joint hyperlaxity, loose dentitions with gangrenous periodontitis, and early loss of teeth. STUDY DESIGN: The patients were clinically suspected to have the periodontal type of Ehlers-Danlos syndrome and thus genetic testing of C1S and C1R causative genes was carried out first by Sanger sequencing then exome sequencing (ES) was considered. RESULTS: No pathogenic variants were detected in C1S and C1R genes then ES revealed a new homozygous missense variant in the CAT gene segregating in the family, c .635 T > G (p.Met212Arg). CONCLUSION: We describe the first Egyptian cases with acatalasemia and expand the mutational spectrum of this rare disorder. Premature loss of teeth is an emerging finding in our cases and addresses the hazardous systemic manifestations associated with the disorder. The rarity of inherited orodental diseases renders the accurate diagnosis difficult and complicates the symptoms. Therefore, the use of advanced molecular technologies is highly advisable for early diagnosis and management of patients.

Vasopressor-Induced Peripheral Gangrene Secondary to COVID-19: A Case Report.

Lower-extremity amputations are a major concern for the current state and future of healthcare, with ischemia contributing significantly to this issue. This is an infrequent but serious complication of vasopressor use. Unfortunately, there is little research existing on the topic and even fewer guidelines on treatment options. Vasopressors are often used to treat life-threatening hypotension. However, ischemic changes associated with vasopressor use often do not present for several days. It is frequently recommended that patients undergo amputation when the first signs of ischemia occur. This approach, however, does not allow for the full process of demarcation, making it difficult to determine which portions of the lower extremity will remain viable. This is a case study of a 37-year-old man who developed severe complications from COVID-19 and was subsequently admitted. Because of a complicated hospital course, he required the use of vasopressor medication that subsequently led to the development of ischemic changes to his bilateral forefeet and hands. Through a prolonged period of demarcation and the collaboration of multiple specialties, the patient was able to salvage each limb and maintain his functionality.

Gallbladder torsion.

ABSTRACT: Gallbladder torsion is a rare cause of acute surgical abdomen. Early recognition and surgical intervention are important for reducing complications and improving postoperative patient outcomes, but standard imaging and laboratory evaluation typically are indistinguishable from those of acute cholecystitis. This article describes a patient with gangrenous cholecystitis secondary to torsion and summarizes recommendations for evaluation and management.

Critical peripheral ischemia: Higher prevalence in Indian systemic lupus erythematosus inception cohort for research (INSPIRE).

INTRODUCTION: There is limited literature on digital ischemia in systemic Lupus erythematosus (SLE). We report the prevalence, associations and outcome of digital infarcts and gangrene from the Indian SLE inception cohort (INSPIRE). METHODS: From the web-based database of INSPIRE, we extracted information for patients with 'Digital Infarct' and 'Digital gangrene' at enrolment into cohort, together considered as critical peripheral ischemia (CPI); all others were controls. We describe the associations of CPI with SLE clinical phenotype, autoantibodies, and disease activity at enrolment. We also report short term outcomes viz. Digital tissue loss and early mortality up to 6 months and recurrence of digital ischemic events in cases till date. RESULTS: Of 2503 SLE patients enrolled into the INSPIRE cohort, we identified 75 (2.9%) patients with CPI, 72 (96%) women and 6 (8%) children. Of them, 55 (73.3%) had digital gangrene and 21 (28%) patients had digital infarcts. Majority of digital gangrene resulted in amputation distal to terminal phalanx (63.6%). Multivariable analysis showed that pulmonary hypertension AOR [6.34 (1.99, 20.2)], coexistent thrombosis AOR [27.8 (15.7, 48.7)], triple antiphospholipid antibody positivity AOR [5.36 (1.67, 16.9)] and the presence of anti-Scl-70-antibody AOR [5.59 (1.86, 16.7)] were more likely while patients with class 3 or 4 lupus nephritis AOR [0.37 (0.15, 0.95)] and anti-nucleosome antibodies AOR [0.47 (0.23, 0.99)] were less likely to be associated with CPI. SLEDAI and short-term mortality were similar between cases and controls. CONCLUSIONS: CPI occurred in a higher proportion (2.9%) of SLE patients in the INSPIRE cohort as compared to earlier reports. Both prothrombotic state and vasculopathy contribute to its occurrence.

Can cannabinoids contribute to cholecystitis - a case of gangrenous acalculous cholecystitis.

SUMMARY: Cannabis legalisation continues to grow globally and its effects on the vascular system have been scrutinized.1 Cannabis has become recognised as a contributor to cardiovascular, cerebrovascular and peripheral vascular disease.2,3 This case report highlights the case of a young male patient presenting with atypical symptoms following cannabis use who developed gangrenous cholecystitis (GC) following vasospasm of his cystic artery. We believe that this is the first-ever case, shared with the anticipation of stimulating more research and prompting recognition of vascular events in this group of patients as our knowledge on the effects of cannabis continues to grow.

Editor's Choice - Association of Pedal Acceleration Time With Healing and Amputation Free Survival in Patients With Ulceration and Gangrene.

OBJECTIVE: Pedal acceleration time (PAT) is a novel non-invasive perfusion measurement that may be useful in the management of patients with ulceration and gangrene. The objective of this study was to report the association between PAT and wound healing, amputation free survival (AFS), and mortality at one year. METHODS: This prospective observational study reviewed all patients who underwent PAT after presentation with ulceration or gangrene from 1 January 2020 to 30 June 2022. PAT was defined as the time (in milliseconds) from the onset of systole to the peak of systole in the mid artery. The final PAT of a limb was used to assess outcomes (presenting PAT if no revascularisation, or post-revascularisation PAT). Wound healing, major limb amputation, and death at one year were collected. Healing was assessed with Fine-Gray competing risks model, AFS via logistic regression, and survival using Cox proportional hazards model. RESULTS: Overall, 265 patients (307 limbs) were included. The median patient age was 71 years and 74.0% (196/265) had diabetes mellitus. Patient demographics were similar among the final PAT category cohorts. Compared with a final PAT category 1, analysis of one year outcomes showed that the final PAT categories 2 - 4 had lower wound healing (category 2, hazard ratio [HR] 0.62, 95% confidence interval [CI] 0.43 - 0.9, p = .012; category 3, HR 0.21, 95% CI 0.08 - 0.58, p = .002; category 4, HR 0.12, 95% CI 0.04 - 0.34, p < .001), lower AFS (category 2, odds ratio [OR] 2.86, 95% CI 1.64 - 5.0, p < .001; category 3, OR 5.1, 95% CI 1.71 - 15.22, p = .003; category 4, OR 12.59, 95% CI 4.34 - 36.56, p < .001), and lower survival (category 2, HR 1.89, 95% CI 1.17 - 3.03, p =.009; category 3, HR 2.37, 95% CI 1.05 - 5.36, p = .039; category 4, HR 4.52, 95% CI 2.48 - 8.21, p < .001). CONCLUSION: The final PAT measurement is associated with wound healing, AFS, and death at one year. PAT may be a valuable tool to assess perfusion of the foot.

[Is this iatrogenic pathology or rare disease?] / Yatrogennaya patologiya ili redkaya bolezn'?

The article is devoted to the problem of diagnosis and treatment strategy of Buerger's disease rarely found in the expert and clinical practice, that is inflammatory disease of unknown etiology, affecting mainly small and medium arteries and veins of limbs. Vascular surgeons around the world have been solving this problem for many years, both in terms of timely diagnosis of this disease and its proper pathogenic treatment. The authors of the article described an expert case of Buerger's disease larvated course in 15-years-old girl, which primarily was mistakenly assessed by specialists as iatrogenic pathology of right forearm vessels in injecting aminazin solution that, according to the clinicians' opinion, led to dry gangrene formation of right wrist and its subsequent amputation. The results of the forensic histological study and retrospective analysis of all child's medical documents allowed to correctly diagnose this rare pathology but only on the stage of commission forensic medical examination in the framework of the initiated criminal proceeding against several leading medical organizations in Saint-Petersburg with a pediatric profile. The authors noted the role of routine medical manipulation in manifestation of larvated pathologic process in a teenage girl in addition to full health and well-being. The objective of present article is devoted to understanding this problem.

Digital ischemic necrosis in a patient with systematic lupus erythematosus patient after severe acute respiratory syndrome coronavirus 2 infection: a case report.

BACKGROUND: Patients with coronavirus disease 2019 have a high incidence of thrombosis that decreases after recovery. When coronavirus disease 2019 is accompanied by diseases prone to thrombosis, risk of post-infection thrombotic events may increase. CASE PRESENTATION: We report a case of digital ischemic gangrene in a 24-year-old Chinese female with systemic lupus erythematosus after recovery from coronavirus disease 2019. The pathogenesis was related to clinical characteristics of systemic lupus erythematosus, hypercoagulability caused by coronavirus disease 2019, and second-hit due to viral infection. CONCLUSION: Patients with autoimmune diseases should remain alert to autoimmune system disorders induced by severe acute respiratory syndrome coronavirus 2 and other viruses. Treatment for these patients should be strictly standardized, and appropriate anticoagulation methods should be selected to prevent thrombosis.

Re-do Hybrid Deep Venous Arterialization via the Popliteal Venous System: Conversion From a Deep Venous Arterialization to a Superficial Venous Arterialization.

Deep venous arterialization (DVA) is a final option for limb salvage in patients with end stage arterial anatomy. We report a 66-year-old dialysis dependent male with forefoot gangrene, Rutherford class 6 chronic limb ischemia, who required a redo endovascular DVA. On initial presentation an angiogram was demonstrated a desert foot with absent tibial runoff to his bilateral lower extremities. After discussion, patient elected to trial DVA in hope of avoiding a major amputation. A hybrid DVA was performed using a Pioneer Plus and .018″ Viabahn stents from the peroneal artery into the peroneal venous system; following this, the peroneal vein was anastomosed to the lesser saphenous vein via an open posterior approach at the ankle. 3 months later, a second DVA was performed by exposing the above knee popliteal artery and vein and creating an end-to-side anastomosis. Of note, the great saphenous vein was less than 2 mm in diameter and no arm vein was available due to history of prior fistulas in bilateral arms. Via the popliteal vein, the posterior tibial vein was selected and additional .018″ Viabahn stents were deployed from the malleolus to the P2 segment of the popliteal vein. Three months after the second hybrid DVA, the patient's forefoot had healed after split thickness skin grafting. Continued patency is noted of the re-do hybrid DVA with minimal calf edema. Newer creative strategies are required for "No Option Chronic Limb Ischemia" which is becoming more relevant in diabetic and dialysis dependent patients. This case illustrates the potential to convert a deep venous arterialization to a superficial venous arterialization for improved venous outflow and wound healing.

Digital Thermal Necrosis Resulting in Amputation After Removing a Tungsten Carbide Ring With a High-Speed Metal Burr: A Case Report.

CASE: A 41-year-old man removed a tungsten carbide ring from his left index finger by cutting it off with a high-speed metal burr. The patient presented two days later with a pink and perfused left index finger with circumferential dry gangrene along the area of the ring, active flexor and extensor tendon excursion, and decreased sensation distally. Within 24 hours, the wound developed into wet gangrene and diffuse cyanosis requiring amputation. CONCLUSION: After reviewing previously documented methods to remove tungsten carbide rings, the authors conclude clinicians should be cognizant of the potential complications associated with the use of a high-speed metal burr.

Digital Gangrene as a Paraneoplastic Manifestation of Peripheral T-cell Lymphoma Not Otherwise Specified Type.

BACKGROUND: Peripheral T cell lymphoma (PTCL), not otherwise specified (NOS) is a heterogenous group of predominantly nodal T cell lymphomas that generally presents with lymphadenopathy with or without extra nodal involvement. Acral vascular syndrome clinically presents as digital ischemia with Raynaud's phenomenon and acral cyanosis. Although, this condition is commonly associated with connective tissue disorder, smoking and vasculitis, its association with lymphoid malignancy is very rare. Here, we present a case report of a patient with digital gangrene of all toes and fingers as a presenting symptom of PTCL-NOS. CASE DESCRIPTION: A 62 year old male presented with digital ischemia associated with pain, low grade fever, loss of appetite and significant weight loss of 6 kilograms over a period of 3 months. On examination, he was found to have bilateral inguinal and axillary lymph nodes with gangrenous changes over toes and fingers but peripheral pulses were palpable. On evaluation he had anemia, elevated ESR and CRP. CT angiogram revealed thinned out digital arteries with multifocal areas of narrowing. Patient was screened for other causes of digital gangrene and was tested negative for ANCA, ANA, cryoglobulins and viral markers. Lymph node biopsy with IHC was suggestive of peripheral T-cell lymphoma-NOS and was started on CHOP regimen. Lymph nodes size decreased and gangrenous changes resolved. CONCLUSION: Though digital ischemia is a rare paraneoplastic presentation of lymphoma, it should be considered if there is a rapid progression of gangrene. Early initiation of chemotherapy may result in the reduction of further progression of digital gangrene and thus prevent permanent disability. In our patient, progression of gangrene was prevented even though it was an aggressive variant of T cell lymphoma.