ABSTRACT
Introducción La púrpura fulminante (PF) es una complicación grave de la sepsis resultado de un conjunto de alteraciones caracterizadas por el desarrollo de lesiones hemorrágicas equimóticas y necrosis de la piel. Objetivo Analizar la eficacia y la seguridad de la aplicación tópica de un compuesto de ácidos grasos hiperoxigenados (AGHO) en la microcirculación de la PF en pacientes pediátricos afectados de sepsis. Material y métodos Se llevó a cabo un estudio prospectivo cuasiexperimental pretest-posttest de un solo grupo en una Unidad de Cuidados Intensivos Pediátrica (UCIP). Se incluyeron en el estudio pacientes con PF de 0 a 18 años. Para evaluar la efectividad del producto objeto de evaluación en la microcirculación de las lesiones por PF se determinaron los valores de oximetría somática antes y después de la aplicación de AGHO cada 4h durante los 3 primeros días de ingreso de los pacientes. Resultados Se reclutaron 4 pacientes, con una mediana de edad de 98 meses. Las lesiones purpúricas que se midieron estaban ubicadas, sobre todo, en ambos pies y manos y, en 2 pacientes, también en los maléolos laterales y gemelos de ambas extremidades inferiores. Se obtuvieron un total de 225 mediciones, observando unas puntuaciones medias preintervención de 71,17±15,65% versus los 73,68±14,83% postintervención. Se objetivó significación estadística (p<0,001) al comparar las mediciones pre- y postintervención. Conclusiones La aplicación precoz y continuada de AGHO en el manejo de la PF por sepsis es una práctica eficaz y segura en los casos de población pediátrica analizada. En más de la mitad de los episodios analizados se objetivó un aumento de microcirculación tisular tras la aplicación de los AGHO, sin eventos adversos. (AU)
Introduction Purpura fulminans (PF) is a serious complication of sepsis resulting from a set of alterations characterised by the development of ecchymotic haemorrhagic lesions and skin necrosis. Aim To analyse the efficacy and safety of the topical application of HOFA compound, in the cutaneous microcirculation of PF lesions in paediatric patients affected by sepsis. Material and methods A prospective quasi-experimental pre-test/post-test single-group conducted in a Paediatric Intensive Care Unit of a third level hospital was performed. Paediatric patients aged 0-18 years with sepsis were included. Somatic oximetry values were measured before and after application of HOFAs every 4hours over the first three days of the patients hospitalisation. Patient's socio-demographic and clinical variables and somatic oximetry by placing a sensor for measuring tissue perfusion on the area with PF were determined. Results Four patients were recruited, with a median age of 98 months. The purpuric lesions measured were mainly located on both feet and hands and, in two patients, also on the lateral malleoli and calves of both lower extremities. A total of 225 measurements were obtained, with mean pre-intervention scores of 71.17±15.65% versus 73.68±14.83% post-intervention. Statistical significance (p<0.001) was observed upon comparison of the pre- and post-intervention measurements. Conclusions Early and continued application of HOFAs in the management of sepsis-induced PF is an effective and safe practice in the cases analysed. In more than half of the episodes analysed, an increase in tissue microcirculation was observed after the application of HOFAs, with no adverse events. (AU)
Subject(s)
Humans , Infant, Newborn , Infant , Child, Preschool , Child , Adolescent , Purpura Fulminans/drug therapy , Pediatrics , Critical Care , Sepsis , Intensive Care Units , Prospective Studies , Non-Randomized Controlled Trials as TopicABSTRACT
This study used UPLC-TQ-MS technology to replicate a Henoch-Schonlein purpura(HSP) model in rats by administering warm drugs by gavage and injecting ovalbumin with Freund's complete adjuvant emulsion. The distribution differences and characteristics of eight major components(ferulic acid, caffeic acid, neochlorogenic acid, cryptochlorogenic acid, benzoyl oxypaeoniflorin, tracheloside, loganin, and paeoniflorin) in rat liver, lung, heart, spleen, and kidney tissues were determined after oral administration of the Liangxue Tuizi Mixture at a dose of 42 g·kg~(-1) in both normal physiological and HSP states at 0.5, 1, 2, 6, and 12 hours. The results showed that the distribution patterns of the eight components of Liangxue Tuizi Mixture in the tissues of normal and HSP model rats were different. The main component, paeoniflorin, in Moutan Cortex and Paeoniae Radix Alba had higher content in all tissues. The eight components were predominantly distributed in the liver, lung, and kidney tissues, followed by spleen and heart tissues.
Subject(s)
IgA Vasculitis , Rats , Animals , IgA Vasculitis/drug therapy , Monoterpenes , Administration, Oral , Liquid Chromatography-Mass SpectrometryABSTRACT
Vitamin C deficiency, otherwise known as scurvy, is a rare diagnosis among populations with adequate nutritional resources. We present a 37-year-old female patient with bilateral lower extremity edema, episodic anasarca, petechiae, and easy bruising who was diagnosed with scurvy. Given the clinical presentation, a broad differential was investigated with no findings suggestive of hematologic or cardiovascular pathology. Initial laboratory studies were unremarkable. Progression of cutaneous symptoms and subsequent laboratory findings demonstrating low vitamin C levels supported a diagnosis of scurvy. Classical symptoms of scurvy include mucocutaneous petechiae, poor wound healing, ecchymosis, hyperkeratosis, corkscrew hair, gingival swelling, and bleeding gums. Following standard enteral supplementation of vitamin C, repeat vitamin C levels failed to adequately respond with the patient remaining to be symptomatic. Given a lack of insufficient nutritional intake or known systemic illness, gastrointestinal malabsorptive etiology was suspected. Though rare in the United States, scurvy should be considered in patients with manifestations of a bleeding disorder. A gastrointestinal workup may be indicated if other nutritional deficiencies are identified, or a source of inadequate intake cannot be established.
ABSTRACT
This case report documents the unusual co-occurrence of immune thrombocytopena (ITP) and chickenpox in a 15-year-old girl. Initial symptoms included shortness of breath, chest pain, and heavy menstrual bleeding. Laboratory results revealed significant anemia and thrombocytopenia. Treatment involved blood transfusions, prednisolone, and iron supplementation. The patient's vesicular skin rash emerged 8 weeks later, prompting the combined diagnosis of ITP and chickenpox. Antiviral treatments, blood transfusions, and supportive care were used in the course of treatment, leading to full recovery. This case emphasizes the importance of prompt diagnosis, appropriate management, and regular follow-up for patients with both chickenpox and ITP. The coexistence of chickenpox and ITP poses a clinical challenge due to the complex interaction between the viral infection and the immune system. The exact mechanism linking these two conditions remains unclear, making it a baffling case that warrants investigation and further understanding. As low is the occurrence of hemorrhagic chickenpox, the presentation of simultaneous chicken pox with or following ITP was found to be rarer, and thus is this enigmatic case presented. Healthcare providers should remain vigilant about such co-occurrences to prevent complications. In order to improve treatment for instances with comparable clinical presentations and advance our collective knowledge, further study is required to better understand the mechanisms relating viral infections and ITP.
ABSTRACT
PURPOSE: Studies have suggested benefits from magnesium sulphate in thrombotic thrombocytopenic purpura (TTP). We aimed to measure the effects of magnesium sulphate supplementation on TTP recovery. METHODS: In this multicenter, randomised, double-blind, controlled, superiority study, we enrolled adults with a clinical diagnosis of TTP. Patients were randomly allocated to receive magnesium sulphate (6 g intravenously followed by a continuous infusion of 6 g/24 h for 3 days) or placebo, in addition to the standard treatment. The primary outcome was the median time to platelet normalisation (defined as a platelet count ≥ 150 G/L). Efficacy and safety were assessed by intention-to-treat. RESULTS: Overall, we enrolled 74 participants, including one who withdrew his/her consent. Seventy-three patients were further analyzed, 35 (48%) allocated to magnesium sulphate and 38 (52%) to placebo. The median time to platelet normalisation was 4 days (95% confidence interval [CI], 3-4) in the magnesium sulphate group and 4 days (95% CI 3-5) in the placebo group. The cause-specific hazard ratio of response was 0.93 (95% CI 0.58-1.48, p = 0.75). The number of patients with ≥ 1 serious adverse reactions was similar in the two groups. By day 90, four patients in the magnesium sulphate group and two patients in the placebo group had died (p = 0.42). The most frequent adverse event was low blood pressure occurring in 34% in the magnesium sulphate group and 29% in the placebo group (p = 0.80). CONCLUSION: Among patients with TTP, the addition of magnesium sulphate to the standard of care did not result in a significant improvement in time to platelet normalisation.
Subject(s)
Magnesium Sulfate , Purpura, Thrombotic Thrombocytopenic , Adult , Female , Humans , Male , Death , Double-Blind Method , Magnesium Sulfate/adverse effects , Platelet Count , Purpura, Thrombotic Thrombocytopenic/drug therapy , Treatment OutcomeABSTRACT
Introduction: Psychogenic purpura, also known as autoerythrocyte sensitization syndrome, is a rare condition which is characterized by spontaneous development of painful purpura, or ecchymoses. Skin lesions are usually preceded by stress and emotional trauma. It is usually a diagnosis of exclusion after ruling out history of trauma, drug intake and other bleeding disorders. Autoerythrocyte sensitization test (AEST) and a psychiatric evaluation helps in the diagnosis and treatment. Objective: To demonstrate the importance of AEST in diagnosing the patient of Gardner Diamond syndrome. Materials and Methods: Five suspected cases of autoerythrocyte sensitization syndrome underwent AEST after ruling out other causes of bleeding. Results: Four out of five patients were positive for AEST while one patient was negative. Psychiatric complaints were present in three patients. One patient was lost to follow up. Rest all patients responded well to vitamin C supplementation. Conclusion: Autoerythrocyte sensitization syndrome is a rare disorder and is a diagnosis of exclusion, so a thorough workup of the patient to rule out common causes of bruising is essential. A high index of suspicion on the clinician's part and a simple OPD-based AEST may help in the diagnosis. Psychiatric consultation is important to find out the stress factor and timely management.
ABSTRACT
Ultra-performance liquid chromatography-quadrupole time of fight/mass spectrometry(UPLC-Q-TOF-MS) and UNIFI were employed to rapidly determine the content of the components in Liangxue Tuizi Mixture. The targets of the active components and Henoch-Schönlein purpura(HSP) were obtained from SwissTargetPrediction, Online Mendelian Inheritance in Man(OMIM), and GeneCards. A "component-target-disease" network and a protein-protein interaction(PPI) network were constructed. Gene Ontology(GO) functional annotation and Kyoto Encyclopedia of Genes and Genomes(KEGG) pathway enrichment analysis were performed for the targets by Omishare. The interactions between the potential active components and the core targets were verified by molecular docking. Furthermore, rats were randomly assigned into a normal group, a model group, and low-, medium-, and high-dose Liangxue Tuizi Mixture groups. Non-targeted metabolomics was employed to screen the differential metabolites in the serum, analyze possible metabolic pathways, and construct the "component-target-differential metabolite" network. A total of 45 components of Liangxue Tuizi Mixture were identified, and 145 potential targets for the treatment of HSP were predicted. The main signaling pathways enriched included resistance to epidermal growth factor receptor tyrosine kinase inhibitors, phosphatidylinositol 3-kinase/protein kinase B(PI3K-AKT), and T cell receptor. The results of molecular docking showed that the active components in Liangxue Tuizi Mixture had strong binding ability with the key target proteins. A total of 13 differential metabolites in the serum were screened out, which shared 27 common targets with active components. The progression of HSP was related to metabolic abnormalities of glycerophospholipid and sphingolipid. The results indicate that the components in Liangxue Tuizi Mixture mainly treats HSP by regulating inflammation and immunity, providing a scientific basis for rational drug use in clinical practice.
Subject(s)
IgA Vasculitis , Animals , Rats , IgA Vasculitis/drug therapy , Network Pharmacology , Molecular Docking Simulation , Phosphatidylinositol 3-Kinases , MetabolomicsABSTRACT
Acquired thrombotic thrombocytopenic purpura (TTP) is a life-threatening disorder. N-Acetylcysteine (NAC) rapidly degrades ultra-large von Willebrand factor multimers by disrupting the disulfide bonds. We report a series of twelve consecutive patients diagnosed with acquired TTP successfully treated with high-dose NAC (150 mg/kg/day) in combination with plasma exchange and steroids. Eight patients also received rituximab. Two patients presented refractory TTP. All patients achieved a quick clinical response in a median time of 5.5 days after starting NAC and are alive after a median follow-up of 29 months. The treatment was feasible and well tolerated. These data provide further evidence of the potential benefit and safety of adding NAC to the standard of care.
Subject(s)
Purpura, Thrombotic Thrombocytopenic , Humans , Purpura, Thrombotic Thrombocytopenic/diagnosis , Purpura, Thrombotic Thrombocytopenic/drug therapy , ADAMTS13 Protein , Rituximab/therapeutic use , Plasma Exchange , Acetylcysteine/therapeutic useABSTRACT
Vitamin B12 deficiency-induced pseudo-thrombotic thrombocytopenic purpura (pseudo-TTP) is a rare condition. In reported literature, most cases were due to pernicious anemia (confirmed by the presence of anti-parietal cells or anti-intrinsic factor antibodies). Nutritional vitamin B12 deficiency causing pseudo-TTP is a much rarer entity. Differentiating thrombotic thrombocytopenic purpura (TTP) cases from pseudo-TTP (from any cause) should be done as soon as possible since the etiology, treatment, and outcome are different. Hematological findings from pseudo-TTP (when associated with vitamin B12 deficiency) respond to B12 replacement but do not respond to plasmapheresis. Neurological symptoms are one of the criteria for TTP, and altered mentation or psychosis in these cases is presumed secondary to either TTP or vitamin B12 deficiency. However, neurological symptoms are more characteristic of TTP rather than pseudo-TTP. In the rarer subsets of patients concerned with nutritional deficiency and neuropsychiatric symptoms, prompt consideration of concomitant vitamin B1 deficiency and Wernicke encephalopathy is essential. Immediate empiric treatment with high-dose IV thiamine should be started. If unrecognized and left untreated, thiamine deficiency can cause rapid progression to irreversible neurological symptoms, coma, and death, despite hematological improvement with B12 replacement. We report a rare case of concomitant vitamin B12 and vitamin B1 deficiency presenting with confusion, severe hemolytic anemia, acute renal failure, diarrhea, and thrombocytopenia mimicking TTP.
ABSTRACT
ObjectiveTo investigate the clinical efficacy of Niaoxue No.1 Prescription in treating Henoch-Schönlein purpura (HSP) nephritis with blood heat and stasis syndrome and its effect on urine erythrocyte, urine protein, blood neutrophils, and blood routine-derived indicators. MethodA multicenter, randomized controlled trial (RCT) was conducted involving 108 HSP nephritis patients from three hospitals. The patients were randomly divided into a control group (54 cases) and a treatment group (54 cases). The treatment group received Niaoxue No.1 prescription once daily, while the control group was treated with captopril and ferulic acid tablets. Both groups underwent a 4-week course of treatment. The urine erythrocyte, urine microalbumin (mAlb), urine sediment red blood cell count, traditional Chinese medicine (TCM) syndrome score, 24-hour urine protein, blood neutrophil count, neutrophil to lymphocyte ratio (NLR), platelet to lymphocyte ratio (PLR), lymphocyte to monocyte ratio (LMR), D-dimer, and immunoglobulin A were detected. The recurrence rate of HSP nephritis was followed up for 6 months. ResultThe total effective rates were 88.9% (48/54) in the treatment group and 70.4% (38/54) in the control group, and the treatment group was superior to the control group (χ2=5.708, P<0.05). Compared with the results before treatment, after 14 days of treatment, the TCM syndrome total score, urine erythrocyte, urine mAlb, and 24-hour urine protein in both groups significantly decreased (P<0.05,P<0.01), and the improvement was more significant in the treatment group than the control group (P<0.05). After 28 days of treatment, compared with the results before treatment, the TCM syndrome total score, urine erythrocyte, urine mAlb, urine sediment red blood cell count, D-dimer, and 24-hour urine protein in both groups significantly decreased (P<0.05,P<0.01), with the treatment group showing a more significant reduction in urine mAlb than the control group (P<0.05). On the 14th and 28th days of treatment, the neutrophil percentage and NLR were lower in the treatment group than in the control group (P<0.05), while there was no statistically significant difference in PLR and LMR. The recurrence rate of nephritis in both groups showed no statistically significant difference after a 6-month follow-up. ConclusionNiaoxue No.1 Prescription in the treatment of HSP nephritis with blood heat and stasis syndrome can significantly improve clinical symptoms, shorten the course of the disease, and reduce urine erythrocyte, urine mAlb, 24-hour urine protein, blood neutrophils, and NLR, thereby effectively alleviating the inflammatory state and reducing kidney damage in children with HSP nephritis.
ABSTRACT
Purpura fulminans (PF) is a rare and fatal complication of septic shock or diffuse intravascular coagulation (DIC) resulting in skin and soft tissue necrosis. PF can be caused by congenital or acquired protein C (PC) or protein S (PS) deficiency. The most common cause of PF in a neonate is sepsis. In our extremely low birth weight preterm case, due to PF that started in the right-hand fingers, examination was made and protein S deficiency was detected as well as MTHFR (A1298C) and Factor V Leiden (R506Q) homozygous mutations. While being unresponsive to fresh frozen plasma (FFP) and unfractionated heparin (UFH) therapy, we want to highlight the curative treatment with hyperbaric oxygen (HBOT), which has not previously been used in extremely low birth weight preterm infants for this purpose.
Subject(s)
Hyperbaric Oxygenation , Purpura Fulminans , Infant , Humans , Infant, Newborn , Purpura Fulminans/therapy , Purpura Fulminans/complications , Purpura Fulminans/genetics , Heparin , Infant, Extremely Low Birth Weight , Infant, PrematureABSTRACT
INTRODUCTION: Henoch-Schönlein purpura (HSP) is a disease commonly manifesting purpura, joint pain, and gastrointestinal symptoms. It can lead to glomerulonephritis (Henoch-Schönlein purpura nephritis, HSPN), which is directly associated with mortality and progression to chronic kidney disease (CKD). While HSP occurs more commonly in children, deadly outcomes occur at a higher rate in adult patients. Previous studies have not reported effective treatment of HSPN by Western or traditional medicine. Here, we report two cases of adult HSPN patients treated with the herbal medicine Jarotang (JRT, modified Sipjeondaebo-tang, modified SJDBT). CASE SUMMARY: Two female patients (Cases 1 and 2), who were 26 and 27 years old, respectively, came to visit us complaining mainly of cutaneous purpura. Both women were diagnosed with HSP, and the results of urinalysis indicated that the HSP had already progressed to renal involvement (3+ proteinuria with 3+ urine occult blood in case 1; 100-120 RBC/HPF with 2+ urine occult blood in Case 2). Both patients were given modified SJDBT in the name of JRT, with some herbs added to disperse and circulate stagnant qi, relieve indigestion, and clear heat. After treatment, patient 1 showed only a trace level of urine occult blood, with disappearance of purpura and proteinuria. Patient 2 showed complete remission of purpura and hematuria. CONCLUSIONS: Modified SJDBT, namely, JRT was effective in treating 2 cases of adulthood HSP and subsequent nephritis. This may be due to the ability of this therapy to replenish qi and blood and/or its immunological effect on T cells. The medication can serve as an effective cure for HSPN.
Subject(s)
Glomerulonephritis , IgA Vasculitis , Nephritis , Child , Humans , Female , Adult , IgA Vasculitis/complications , IgA Vasculitis/drug therapy , IgA Vasculitis/diagnosis , Nephritis/drug therapy , Nephritis/complications , Glomerulonephritis/drug therapy , Glomerulonephritis/complications , Proteinuria/complications , Proteinuria/urineABSTRACT
Background: Sheng-Xue-Xiao-Ban Capsule (SXXBC), as a classic Chinese traditional medicine comprised of natural indigo, cortex moutan, forsythia, herba agrimoniae, and licorice, exhibits a heat-clearing and detoxicating function, hemostasis, and stasis dissipation, which is widely applied to treat idiopathic thrombocytopenic purpura (ITP). However, report on ischemic colitis and pulmonary embolism induced by SXXBC therapy is never disclosed. We report the case of an ITP patient who received SXXBC for ascending platelets that then induced ischemic colitis and pulmonary embolism. Case Description: A 74-year-old female patient was admitted in June 2021 due to "bleeding in stool for 1 day," she was then re-admitted in July 2021 due to "repeated bleeding in stool for 2 days". Abdominal computed tomography (CT), colonoscopy, and a pathological examination suggested ischemic colitis according to the American College of Gastroenterology (ACG) clinical guidelines. Pulmonary artery CT angiography suggested pulmonary embolism reflected by multiple filling defects, and the patient presented with shortness of breath. It was noted that the patient had started taken SXXBC for ascending platelets 2 months before the onset of hematochezia. After the diagnosis of hematochezia was made, the patient received phenethylamine and carbazochrome for hemostasis, mesalazine enteric-coated tablets for anti-inflammation, and SXXBC was stopped. The hematochezia then ceased, and the ischemic colitis was attenuated. Afterwards, low-molecular-weight heparin was administered, followed by a 3-week treatment of rivaroxaban anticoagulant, which was taken orally after discharge. The pulmonary embolism was then obviously ameliorated. After excluding other causes, the patient was diagnosed with SXXBC-induced ischemic colitis complicated by pulmonary embolism. After conducting research, we came to the view that natural indigo, which is the main component of SXXBC, contributed to the patient's illness. Conclusions: Ischemic colitis complicated with pulmonary embolism are rare; however, close attention such as regular abdominal CT test needs to be paid and preventive steps such as anti-coagulant treatment could to be taken (if symptoms occur) when treating patients with SXXBC.
ABSTRACT
OBJECTIVE: To analyze and summarize the clinical features, diagnosis, and treatment of children with Henoch-Schönlein purpura (HSP) complicated by overt gastrointestinal bleeding (GI bleeding) for achieving early identification, prevention, and treatment in terms of severe GI bleeding. METHODS: A retrospective analysis was conducted on children with HSP complicated by overt GI bleeding who were admitted to the Department of Traditional Chinese Medicine of Beijing Children's Hospital from January 2017 to December 2019. According to the severity of GI bleeding, the patients were divided into mild bleeding group (61 cases) and moderate and severe bleeding group (38 cases). Inflammatory parameters, coagulation function, GI ultrasound findings, and clinical features were compared. Logistic regression analysis was used to determine the related variables affecting the severity of GI bleeding, and the ROC curve was used to determine the variable test efficacy. RESULTS: Onset in summer, wide distribution of skin rash with facial involvement, the elevation of D-dimer and high neutrophil-to-lymphocyte ratio (NLR) had significant effects on the severity of GI bleeding. ROC curve analysis showed that the optimal cut-off points of NLR and D-dimer for predicting severe GI bleeding in children with HSP were 10.56 and 0.89 mg/L, respectively. CONCLUSION: Facial rash may be a warning sign of GI bleeding. Enhanced monitoring of NLR and D-dimer is helpful for early recognition of GI bleeding as well as assessment of severity.
Subject(s)
IgA Vasculitis , Child , Humans , IgA Vasculitis/complications , IgA Vasculitis/diagnosis , Retrospective Studies , Gastrointestinal Hemorrhage/etiology , Lymphocytes , NeutrophilsABSTRACT
A DNA aptamer to the von Willebrand factor (VWF) A1 domain, TAGX-0004, inhibits the binding of VWF to platelets. Nucleic acid aptamers are single-stranded DNA or RNA molecule forming three-dimensional structures that are capable of specifically binding to proteins and are expected to contribute to alternative medicine. ARC1779, an aptamer targeting the VWF A1 domain, had been evaluated in a phase II clinical trial of patients with acquired thrombotic thrombocytopenic purpura (aTTP); however, its development was terminated. Caplacizumab, an anti-VWF A1 domain nanobody, is now increasingly employed as first-line therapy for the treatment of aTTP in Western countries. However, there have been reports regarding adverse bleeding events and the high cost of the treatment. In this study, the inhibitory effects of TAGX-0004 were compared with those of ARC1779 and caplacizumab on in vitro platelet aggregation and thrombus formation. TAGX-0004 had an excellent high affinity to the VWF A1 domain and superior efficacy, such as its potent inhibitory activity in in vitro platelet aggregation and thrombus formation. Therefore, it can potentially overcome the problems associated with caplacizumab and can be developed as a promising drug not only for aTTP treatment but also for the treatment of the various VWF-mediated thrombotic disorders.
Subject(s)
Purpura, Thrombotic Thrombocytopenic , Thrombosis , Blood Platelets , Humans , Platelet Aggregation Inhibitors/therapeutic use , Purpura, Thrombotic Thrombocytopenic/drug therapy , Thrombosis/drug therapy , von Willebrand Factor/therapeutic useABSTRACT
Thrombotic thrombocytopenic purpura (TTP) is a thrombotic microangiopathy, in which a severe deficiency of von Willebrand factor lyase results in thrombocytopenic clots that block blood vessels and eventually lead to terminal organ failure. Therapeutic plasma exchange is the cornerstone of TTP treatment which can greatly improves the survival rate of the patients. With the further exploration to the pathophysiological mechanism of TTP, other alternative therapies, new immunosuppressive agents, targeted antagonists, gene therapy and other emerging means gradually emerge, which are expected to further reduce the mortality and recurrence rate of the patients. In this review, the new developments in TTP treatment were summarized briefly.
Subject(s)
Purpura, Thrombotic Thrombocytopenic , ADAMTS13 Protein , Humans , Immunosuppressive Agents , Plasma Exchange , Purpura, Thrombotic Thrombocytopenic/therapy , von Willebrand FactorABSTRACT
PURPOSE: To review the published literature to determine the efficacy and safety of homeopathic agents or vitamins in reducing ecchymosis after oculofacial surgery or laser surgery. METHODS: A literature search was conducted in the PubMed database initially in December 2019 and updated in March 2020 to identify all studies in the English language literature on the use of homeopathic agents or vitamins in oculofacial procedures, including laser surgery. The search yielded 124 citations, and 11 articles met all inclusion criteria for this assessment. A panel methodologist then assigned a level of evidence rating for each study. Eleven studies met inclusion criteria; 9 were rated level I, and 2 were rated level III. RESULTS: The agents studied in the articles identified included oral or topical Arnica montana (AM), oral Melilotus extract, topical vitamin K oxide, and topical AM combined with Rhododendron tomentosum. Metrics to describe ecchymosis varied. In 7 controlled studies, perioperative AM provided no or negligible benefit versus placebo. In 2 studies, vitamin K cream was equivalent to placebo. One study of oral Melilotus extract had less ecchymosis compared with controls in paranasal and eyelid ecchymosis at postoperative day (POD) 7, but not at PODs 1 and 4. A lone cohort study of combined topical AM and R. tomentosum lacked objective metrics and adequate controls. No serious side effects from administration of homeopathic agents or vitamins were identified. CONCLUSIONS: The current literature does not support the use of AM, vitamin K oxide, R. tomentosum, or Melilotus extract for reducing ecchymosis after oculofacial surgery or pulsed dye laser surgery.
Subject(s)
Ecchymosis/drug therapy , Materia Medica/therapeutic use , Ophthalmologic Surgical Procedures/adverse effects , Plant Extracts/therapeutic use , Vitamin K/therapeutic use , Academies and Institutes/standards , Ecchymosis/etiology , Eyelid Diseases/surgery , Face/surgery , Humans , Ophthalmology/organization & administration , Paranasal Sinus Diseases/surgery , Technology Assessment, Biomedical , United StatesABSTRACT
Drug-induced thrombotic microangiopathy (DITMA) represents 10%-13% of all thrombotic microangiopathy (TMA) cases and about 20%-30% of secondary TMAs, just behind pregnancy-related and infection-related forms. Although the list of drugs potentially involved as causative for TMA are rapidly increasing, the scientific literature on DITMA is quite scarce (mostly as individual case reports or little case series), leading to poor knowledge of pathophysiological mechanisms and clinical management. In this review, we focused on these critical aspects regarding DITMA. We provided an updated list of TMA-associated drugs that we selected from a scientific literature review, including only those drugs with a definite or probable causal association with TMA. The list of drugs is heterogeneous and could help physicians from several different areas to be familiar with DITMA. We describe the clinical features of DITMA, presenting the full spectrum of clinical manifestations, from systemic to kidney-limited forms. We also analyze the association between signs/symptoms (i.e., malignant hypertension, thrombocytopenia) and specific DITMA causative drugs (i.e., interferon, ticlopidine). We highlighted their multiple different pathophysiological mechanisms, being frequently classified as immune-mediated (idiosyncratic) and dose-related/toxic. In particular, to clarify the role of the complement system and genetic deregulation of the related genes, we conducted a revision of the scientific literature searching for DITMA cases who underwent renal biopsy and/or genetic analysis for complement genes. We identified a complement deposition in renal biopsies in half of the patients (37/66; 57%), with some drugs associated with major deposits (i.e., gemcitabine and ramucirumab), particularly in capillary vessels (24/27; 88%), and other with absent deposits (tyrosine kinase inhibitors and intraocular anti-VEGF). We also found out that, differently from other secondary TMAs (such as pregnancy-related-TMA and malignant hypertension TMA), complement genetic pathological mutations are rarely involved in DITMA (2/122, 1.6%). These data suggest a variable non-genetic complement hyperactivation in DITMA, which probably depends on the causative drug involved. Finally, based on recent literature data, we proposed a treatment approach for DITMA, highlighting the importance of drug withdrawal and the role of therapeutic plasma-exchange (TPE), rituximab, and anti-complementary therapy.
ABSTRACT
BACKGROUND: Henoch-Schönlein purpura nephritis (HSPN) is listed as the most common secondary glomerular diseases among children. Approximately 15 to 20% of children eventually could develop into chronic renal failure. Chinese patent herbal medicine Huaiqihuang (HQH) has been widely used in children with HSPN. This study aimed to evaluate the effectiveness and safety of HQH for HSPN in children, so as to provide evidence for clinical use. METHODS: Randomized controlled trials (RCTs) on HQH for HSPN in children were searched in eight Chinese and English databases from their inception to December 2020. We included children with HSPN received HQH combined with conventional medicine. Cochrane "Risk of bias" tool was used to assess methodological quality, and "Grading of Recommendations Assessment, Development, and Evaluation (GRADE) approach" to summarize the certainty of evidence for main findings. Effect estimates were presented as risk ratio (RR), mean difference (MD) or standardized mean difference (SMD) with 95% confidence interval (CI) in meta-analyses using RevMan 5.3. Data not suitable for statistical pooling were synthesized qualitatively. RESULTS: In total seven RCTs were identified. Compared with conventional medicine alone, HQH plus conventional medicine showed the better effect in improving clinical cure rate (RR 1.58; 95%CI 1.17 to 2.14; n = 6) and total effective rate (RR 1.34; 1.16 to 1.54; n = 6); reducing urine sediment erythrocyte count (MD -9.23; - 10.76 to - 7.69; n = 3) and urine ß2 micro-globulin level (MD -0.09; - 0.12 to - 0.06; n = 2). No serious adverse event was recorded in all included trials. CONCLUSIONS: Limited evidence showed HQH combined with conventional medicine had a beneficial effect for children with HSPN, and the side effects were mild. HQH may be a promising complementary therapy. However, long term follow-up, high quality and multicenter RCTs are required to confirm the findings.
Subject(s)
Drugs, Chinese Herbal/therapeutic use , Herbal Medicine/methods , IgA Vasculitis/drug therapy , Nephritis/drug therapy , Child , Humans , Randomized Controlled Trials as TopicABSTRACT
BACKGROUND: Variations in the oral microbiota have been significantly correlated with the progress of autoimmune diseases, such as immunoglobulin A nephropathy and Henoch-Schönlein purpura (HSP). However, there is no report outlining the character of tongue coating microbiota variations in children with Henoch-Schönlein purpura nephritis (HSPN). METHOD: A total of 20 children with HSPN and 14 healthy controls were recruited for this research. Tongue coating samples of two groups were collected for 16S rRNA gene sequencing. The diversity, principal component analysis (PCA), nonmetric multidimensional scaling (NMDS), partial least squares discriminant analysis (PLS-DA), and linear discriminant analysis (LDA) effect size (LEfSe) were performed. Microbial function was assessed using the PICRUST. RESULTS: The ACE and Chao indices were greatly lower in the HSPN group than in the HG (P = 0.001). The Shannon and Simpson indices were dramatically reduced in children with HSPN compared with those in the healthy controls (P = 0.005). Bacteroidales, Selenomonadales, Lactobacillales, Fusobacteriales, Neisseriales, and Actinomycetales composed more than 80% of all sequences, while Bacteroidales was the most generous order in both groups. PCA, NMDS and PLS-DA showed a marked difference between the control and HSPN groups. LEfSe analysis showed alteration of tongue coating microbiota in the HSPN group. There were 30 metabolic functions significantly differed between the two groups. CONCLUSIONS: Children with HSPN have substantially various tongue coating microbiota compared to healthy controls. Even though this research does not indicate causality, it is beneficial to enhance the possibility for coming microbial-based treatments to enhance the clinical effects of HSPN in children.