Near-fatal cocaine intoxication in an infant with thrombotic microangiopathy associated with multiple organ failure / Intoxicação grave por cocaína em criança com microangiopatia trombótica associada à falência múltipla de órgãos

ABSTRACT Objective: To report a pediatric case of drug-induced thrombotic microangiopathy caused by cocaine Case description: We report a nine-month-old patient who developed thrombotic microangiopathies after extreme cocaine intoxication, multiple organ dysfunction syndrome with hemodynamic dysfunction, anuric renal failure, liver failure, encephalopathy, and myocardial injury, corresponding phenotypically to thrombocytopenia-associated multiple organ failure. The patient received continuous venous hemofiltration and therapeutic plasma exchange, recovering satisfactorily. She was discharged after 30 days of hospitalization under the guidance of the childcare service, and was healthy after one year of follow-up. Toxicological samples confirmed high levels of cocaine and derivatives in blood, urine and hair. Comments: To our knowledge, this is the first reported pediatric case. There are particularities of cocaine intoxication pathophysiology that can trigger thrombotic microangiopathies because of vasoconstriction, direct endothelial injury, platelet activation, and increasing von Willebrand factor and fibrinogen levels. All of which results in a prothrombotic state, inflammatory dysregulation, and microvascular thrombi. The increasing use of cocaine, especially among young adults, puts children at high risk of toxicity, either by passive unintentional exposure, or abuse due to the increased availability in homes.

RESUMO Objetivo: Relatar um caso pediátrico de microangiopatia trombótica induzida por drogas causada por cocaína Descrição do caso: Relatamos uma paciente de nove meses de idade que desenvolveu microangiopatia trombótica após intoxicação extrema por cocaína, síndrome de disfunção de múltiplos órgãos com disfunção hemodinâmica, insuficiência renal anúrica, insuficiência hepática, encefalopatia e lesão miocárdica, correspondendo fenotipicamente à falência múltipla de órgãos associada à trombocitopenia. A paciente recebeu hemofiltração venosa contínua e plasmaférese terapêutica, recuperando-se satisfatoriamente. Recebeu alta após 30 dias de internação sob orientação do serviço de puericultura e estava saudável após um ano de seguimento. Amostras toxicológicas confirmaram altos níveis de cocaína e derivados no sangue, urina e cabelos. Comentários: Até onde sabemos, este é o primeiro caso pediátrico relatado. Existem particularidades da fisiopatologia da intoxicação por cocaína que podem desencadear a microangiopatia trombótica devido à vasoconstrição, lesão endotelial direta, ativação plaquetária e aumento do fator de von Willebrand e dos níveis de fibrinogênio. Tudo isso resulta em um estado pró-trombótico, desregulação inflamatória e trombos microvasculares. O uso crescente de cocaína, principalmente entre adultos jovens, coloca as crianças em alto risco de toxicidade, seja por exposição passiva não intencional ou abuso devido à maior disponibilidade nas residências.

Chinese Medicine Treats Diabetic Microangiopathy： A Review / 中国实验方剂学杂志

Diabetes and its complications are major public health issues of worldwide concern. Diabetic microangiopathy is a vascular complication of diabetes caused by blood stasis and deficiency， characterized by impaired microcirculation with hyaline deposits. Diabetic nephropathy， diabetic retinopathy， and diabetic neuropathy are the most common types of diabetic microangiopathy， which can be traced back to the pre-diabetes period and is aggravated by the dynamic evolution of diabetes. Therefore， early intervention is required. Anti-oxidative， anti-inflammatory， and microcirculation-improving drugs should be chosen to treat diabetic microangiopathy based on hypoglycemic， lipid-lowering， and hypotensive treatment in clinical practice. Diabetic microangiopathy belongs to the theoretical concept of "collateral disease" in traditional Chinese medicine （TCM）. The core of the treatment of diabetic microangiopathy with Chinese medicine is to protect "tertiary collateral vessels-microvascular"， and Chinese medicines with Qi-replenishing， Yin-nourishing， heat-clearing， and blood-activating effect are used for compatibility in Chinese medicine prescriptions. Based on the understanding and treatment principles of TCM and western medicine for diabetic microangiopathy， this review briefly summarized the research progress of commonly used prescriptions such as Renshen Baihutang， Yuye Tang， Simiao Yongantang， Gegen Qiliantang， Liuwei Dihuangwan， and modern Chinese medicine preparations for the treatment of diabetic microangiopathy. Moreover， the research progress of Chinese medicines including Ginseng Radix et Rhizoma， Astragali Radix， Rehmannia Radix， Lycii Fructus， Notoginseng Radix et Rhizoma， Salviea Miltiorrhizae Radix et Rhizoma， Lonicera Japonica Flos， and Puerariae Lobatae Radix were outlined. This review is expected to provide the clinical basis and theoretical guidance for the treatment of diabetic microangiopathy with Chinese medicine.

Progress on diagnosis and treatment of kidney transplantation-associated thrombotic microangiopathy / 器官移植

Thrombotic microangiopathy (TMA) is a severe complication after kidney transplantation, mainly characterized by thrombocytopenia, microvascular hemolytic anemia and acute kidney injury, which may lead to kidney allograft failure or even death of the recipients. With the increasing quantity of solid organ transplantation in China and deeper understanding of TMA, relevant in-depth studies have been gradually carried out. Kidney transplantation-associated TMA is characterized with different causes and clinical manifestations. Non-invasive specific detection approach is still lacking. The diagnosis of TMA mainly depends on renal biopsy. However, most TMA patients are complicated with significant thrombocytopenia. Hence, renal puncture is a risky procedure. It is difficult to make a definite diagnosis. For kidney transplantation-associated TMA, plasma exchange, intravenous immunoglobulin and withdrawal of potential risk drugs are commonly employed. Nevertheless, the overall prognosis is poor. In this article, the classification of TMA after kidney transplantation, diagnosis and treatment of kidney transplantation-associated TMA were reviewed, aiming to provide reference for clinical diagnosis and treatment of kidney transplantation-associated TMA.

Clinical characteristics and prognosis of anti-factor H antibody associated-atypical hemolytic uremic syndrome in children / 中华实用儿科临床杂志

Objective:To summarize the clinical data of anti-factor H antibody-associated atypical hemolytic uremic syndrome (aHUS) in children, and analyze the risk factors for disease recurrence and poor prognosis.Methods:A prospective cohort study was conducted on 52 children with anti-factor H antibody-associated aHUS in Beijing Children′s Hospital, Capital Medical University from November 2011 to November 2021.Patient information about the genetic background, clinical and renal pathological characteristics, treatment, and prognosis were collected.Then, the disease recurrence and prognosis were analyzed using the survival curve and Cox regression model. Results:In 52 children, there were 33 males and 19 females.The average age of onset for aHUS was 2.4-12.8 years, and 92.3%(48/52) of the children developed symptoms at the age of 4-12 years.The copy numbers of complement factor-H-related 1 (CFHR1) and complement factor-H-related 3 (CFHR3) genes were calculated in 42 children.Among the 42 cases, 18 cases (42.9%) had CFHR1 homozygous deletion, and 83.3% (15/18) of them also had CFHR3 homozygous deletion.All the patients were given plasma therapy.Besides, 76.9% (40/52) of the children were treated with immunosuppressive therapy (steroid and/or immunosuppressant) at the first onset of the disease.About 86.5%(45/52 cases) of the patients received immunosuppressive therapy in the course of disease, and the immunosuppressive treatment lasted for 6-20 months in total.The median follow-up time was 58 (28, 91) months.Among 52 patients, only 12 patients (23.1%) suffered disease recurrence.The relapse-free survival rate in children with CFHR1 homozygous deletion was significantly lower than that in children with non-homozygous deletion ( χ2=4.700, P=0.030). The relapse-free survival rate in children with CFHR1 and CFHR3 homozygous deletions was also significantly lower than that in other children ( χ2=4.181, P=0.041). At the end of the follow-up, 73.1%(38/52) of the children had normal renal function and no persistent proteinuria or hypertension.23.1%(12/52 cases) of the children had persistent proteinuria and/or hypertension.One child had Stage 3-4 chronic kidney disease, and 1 child was dialysis dependent. Conclusions:Anti-factor H antibody-associated aHUS is prone to occur in children aged between 4-12 years old, who respond well to plasma therapy and immunosuppressive therapy.Children with anti-factor H antibody-associated aHUS and CFHR1 and CFHR3 homozygous deletions have a high recurrence rate.Treatment with immunosuppressive therapy and assessment of the copy number of CFHR1 and CFHR3 genes in the early stage of the disease are important for preventing disease recurrence and improving prognosis.

Diagnosis and treatment strategies for solid organ transplant recipients with SARS-CoV-2 infection / 器官移植

Due to long-term use of immunosuppressive agents, solid organ transplant recipients (SOTR) belong to high-risk populations of multiple pathogenic infection, including SARS-CoV-2. In addition, SOTR are constantly complicated by chronic diseases, such as hypertension and diabetes mellitus, etc. After infected with SARS-CoV-2, the critically ill rate and fatality of SOTR are higher than those of the general population, which captivates widespread attention from experts in the field of organ transplantation. Omicrone variant is currently the significant pandemic strain worldwide, rapidly spreading to more than 100 countries worldwide and causing broad concern. According to the latest international guidelines on the diagnosis and treatment of SARS-CoV-2 infection and relevant expert consensus in China combined with current domestic situation of SARS-CoV-2 pandemic and China's "diagnosis and treatment regimen for SARS-CoV-2 infection (Trial Version 10)", the epidemiology, clinical manifestations and prognosis, diagnosis, clinical classification and treatment of SARS-CoV-2 infection were briefly reviewed.

Advances in Diagnosis and Treatment of Transplant-Associated Thrombotic Microangiopathy --Review / 中国实验血液学杂志

Transplantation-associated thrombotic microangiopathy (TA-TMA) is one of the serious complications mostly occurring within 100 days after hematopoietic stem cell transplantation (HSCT). Risk factors of TA-TMA include genetic predispositions, GVHD, and infections. The pathophysiological mechanisms of TA-TMA start with endothelial injury caused by complement activation, which leads to microvascular thrombosis, and microvascular hemolysis, ultimately resulting in multi-organ dysfunction. In recent years, the development of complement inhibitors has markedly improved the prognosis of TA-TMA patients. This review will give an update on risk factors, clinical manifestations, diagnosis, and treatment of TA-TMA, so as to provide references for clinical practice.

Microparticles: potential new contributors to the pathogenesis of systemic sclerosis?

Abstract Background Microparticles (MPs) are membrane-derived vesicles released from cells undergoing activation or apoptosis with diverse proinflammatory and prothrombotic activities, that have been implicated in the pathogenesis of systemic sclerosis (SSc). We aimed to evaluate the plasma levels of platelet-derived microparticles (PMPs), endothelial cell-derived microparticles (EMPs), and monocyte-derived microparticles (MMPs) in SSc patients, and the association between MPs and the clinical features of SSc. Methods In this cross-sectional study, 70 patients with SSc and 35 age- and sex-matched healthy controls were evaluated. Clinical and nailfold capillaroscopy (NFC) data were obtained from all patients. Plasma levels of PMPs (CD42+/31+), EMPs (CD105+), and MMPs (CD14+) were quantified by flow cytometry. Results Patients were mainly females (90%), with a mean age of 48.9 years old. PMP, EMP, and MMP levels were significantly increased in SSc patients compared to controls (79.2% ± 17.3% vs. 71.0% ± 19.8%, p = 0.033; 43.5% ± 8.7% vs. 37.8% ± 10.4%, p = 0.004; and 3.5% ± 1.3% vs. 1.1% ± 0.5%, p < 0.0001, respectively). PMP levels were significantly higher in patients with positive anti-topoisomerase-I antibodies (p = 0.030) and in patients with a disease duration > 3 years (p = 0.038). EMP levels were lower in patients with a higher modified Rodnan skin score (p = 0.015), and in those with an avascular score > 1.5 in NFC (p = 0.042). Conclusion The increased levels of PMPs, EMPs and MMPs in scleroderma patients might indicate a possible role for these agents in the pathogenesis of this challenging disease.

Development of an assessment scale of knowledge, attitude and practice on diabetic microangiopathy for primary care general practitioners using Delphi method / 中华全科医师杂志

Objective:To develop an assessment scale for measuring knowledge, attitude and practice of general practitioners in primary care towards diabetic microangiopathy.Methods:The knowledge-attitude-practice (KAP) theory was used as a framework to draw up the initial item pool based on related literature and guidelines during March to October 2022. Two rounds of Delphi consultation were conducted among 15 experts from general medicine and related fields. The positive coefficient, authority coefficient and coordination coefficient of experts were calculated, and the threshold table of indexes was screened, and the final assessment scale of KAP was developed after the two rounds of consultation.Results:The developed primary care general practitioners′ KAP assessment scale for diabetic microangiopathy consists of 3 primary indexes and 52 secondary indexes(25 of knowledge scale, 13 of attitude scale, 14 of practice scale). The positive coefficient of experts was 100.0% and the authority coefficient of experts was 0.89 in both rounds of consultation; and the coordination coefficient of experts was 0.319 and 0.322 for the first and second consultations, respectively (both P<0.05). Conclusion:A KAP assessment scale of diabetic microangiopathy for primary care general practitioners has been developed in this study. The expert positive coefficient, authority coefficient and expert coordination coefficient meet the requirements, which provides reference for evaluating the management ability of diabetic microangiopathy of primary care general practitioners.

Three shades of black - secondary thrombotic microangiopathy

Thrombotic microangiopathy is a group of disorders characterized by microangiopathic hemolytic anemia, thrombocytopenia and organ damage. Wide age distribution and the heterogeneity in presentation demand a deeper understanding into the pathogenesis of TMA. Primary TMA is distinct from TMA associated with secondary causes and remains clinically occult till a precipitating factor aggravates it. The extent and severity of renal damage caused by each of them is also distinct. The first alerting signal could be the presence of schistiocytes on peripheral smear and arteriolar thrombi on light microscopy. Thus in secondary TMA, identification of the underlying disorder is indispensible for targeted management.

Atypical hemolytic uremia syndrome in a young adult male

Atypical hemolytic uremia syndrome (aHUS) is a rare and life-threatening disease, characterized by the same triad of hemolytic anemia, thrombocytopenia, and renal failure as seen in HUS. It differs in its etiology, being caused by a dysregulation of the complement pathway rather than Shiga-like toxin-producing Escherichia coli. Prognosis is poor, with 50% of cases progressing to end-stage renal disease (ESRD) and 25% succumbing in the acute phase. The treatment of choice is therapeutic plasma exchange which can lower mortality. Monoclonal antibody drugs such as eculizumab, which suppress the dysregulated complement pathway, help to prevent complement-mediated kidney injury. We report the case of a young adult male who presented with thrombocytopenia and worsening acute kidney injury and was diagnosed with aHUS based on high lactic dehydrogenase, low complement C3, and haptoglobin, as well as renal biopsy showing thrombotic microangiopathy

Chronic gemcitabine-induced thrombotic microangiopathy in the kidneys / Microangiopatía trombótica crónica localizada en riñones asociada al uso de gemcitabina

Abstract Thrombotic microangiopathies (TMAs) are characterized by microvascular occlusion secondary to diffuse endothelial damage which produces inflammation, platelet aggregation and red blood cell destruction, causing ischemic injury to the affected organ. They are clinically characterized by Coombs-negative microangiopathic hemolytic anemia, and multiple organ damage (mainly of the kidneys, central nervous system, cardiovascular apparatus and gastrointestinal tract). They may occur systemically or locally, and they have multiple etiologies. In patients with cancer, determining the cause of thrombotic microangiopathy is a great diagnostic challenge, with the most frequent etiologies being active malignant neoplasms, disseminated intravascular coagulation, infections and antineoplastic drugs. We present the clinical case of a patient with unresectable pancreatic adenocarcinoma on chronic gemcitabine treatment, and highlight the importance of suspecting and distinguishing chemotherapy-induced TMAs from neoplasm-induced TMAs, as their prognosis and treatment are very different. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2249).

Resumen Las microangiopatías trombóticas (MAT) se caracterizan por la oclusión microvascular como consecuencia de una lesión endotelial difusa que produce inflamación, agregación plaquetaria y destrucción de glóbulos rojos, causando daño isquémico del órgano afectado. Se caracterizan clínicamente por anemia hemolítica microangiopática, Coombs negativo, daño multiorgánico (principalmente de riñones, sistema nervioso central, aparato cardiovascular y tracto gastrointestinal). Su presentación puede ser sistémica o localizada y sus etiologías son múltiples. En los pacientes con cáncer es un gran reto diagnóstico establecer la causa de la microangiopatía trombótica, siendo las etiologías más frecuentes la neoplasia maligna activa, la coagulación intravascular diseminada, infecciones y medicamentos antineoplásicos. Se presenta el caso clínico de una paciente con adenocarcinoma cáncer de páncreas irresecable, en manejo crónico con gemcitabina y se resalta la importancia de sospechar y distinguir la MAT inducida por quimioterapia, de la causada por la neoplasia ya que el pronóstico y tratamiento son muy diferentes. (Acta Med Colomb 2022; 47. DOI:https://doi.org/10.36104/amc.2022.2249).

Multitarget microangiopathy in a young healthy man with COVID-19 disease: A case report

A 41?year?old man presented to the emergency department complaining of decrease of vision in his left eye. Initial examination was consistent with retrobulbar optic neuritis, and an intravenous drip of methylprednisolone was started. On the third day, the fundus examination revealed the appearance of multiple Purtscher?like cotton?wool spots in the posterior pole and nasally to the optic disc, slight retinal whitening around the fovea, and cherry?red spot. The patient reported flu?like symptoms, and he tested positive at PCR (polymerase chain reaction) test for 2019?nCoV (2019 novel coronavirus) infection. Assuming possible 2019?nCoV?related vascular damage, we prescribed low?molecular?weight heparin. The lesions were regressing at follow?up, and we registered a complete visual recovery

Role of complement activation in the pathogenesis of severe cardiorenal injury in patients with primary malignant hypertension / 中华肾脏病杂志

Objective:To investigate the role of complement activation in the pathogenesis of primary malignant hypertension (MHT) with nephrosclerosis complicated with severe cardiorenal injury.Methods:Data of MHT patients with nephrosclerosis proven by biopsy from January 2010 to December 2020 in the Beijing Anzhen Hospital, Capital Medical University were retrospectively analyzed. The expressions of complement-related component C4d, C1q, complement factor H-related protein 5, C3c and C5b-9 were detected by immunohistochemical staining. According to whether the patients were complicated with acute heart failure (AHF) and/or acute kidney injury (AKI), they were divided into severe cardiorenal injury group and non-severe cardiorenal injury group. The differences of clinicopathological data between the two groups were compared. According to the degree of C4d deposition in renal tissues, patients were divided into C4d diffused deposition group and non-C4d diffused deposition group. The severity of cardiorenal injury and the pathological characteristics of thrombotic microangiopathy in renal tissues were compared between the two groups.Results:A total of 33 patients were enrolled in this study, of which 17 cases (51.5%) were complicated with severe cardiorenal injury; AHF occurred in 16 patients (48.5%), AKI occurred in 8 patients (26.7%), and AHF and AKI were combined in 7 patients (21.2%). Compared with non-severe cardiorenal injury group, patients in severe cardiorenal injury group had higher levels of baseline lactate dehydrogenase [326.0 (217.0, 366.0) IU/L vs 197.0 (165.0, 220.0) IU/L, Z=37.000, P=0.002] and hemoglobin [(143.6±24.0) g/L vs (106.4±24.7) g/L, t=38.500, P<0.001], lower levels of 12 h urinary incontinence osmolality [400.0 (342.5, 504.0) mmol/L vs 476.0 (432.3, 616.5) mmol/L, Z=72.000, P=0.021] and serum albumin [(36.2±9.4) g/L vs (43.2±6.2) g/L, t=6.423, P=0.017], and thicker left ventricular posterior wall [(14.0±2.1) mm vs (12.1±1.1) mm, t=6.552, P=0.018]. The immunohistochemical results of kidney tissue showed that the proportions of C4d and C5b-9 diffused deposition in severe cardiorenal injury group were significantly higher than those in non-severe cardiorenal injury group (5/16 vs 0/15, P=0.043; 12/16 vs 5/15, P=0.032). Compared with non-C4d diffused deposition group, C4d diffused deposition group had higher incidence of AHF (5/5 vs 10/26, P=0.018), poorer heart function, more severe ventricular remodeling, and shorter history of hypertension [2.0 (0, 12.0) months vs 48.0 (9.5, 84.0) months, Z=22.500, P=0.022]. Conclusions:The incidence of severe cardiorenal injury in MHT patients with nephrosclerosis is about 51.5%. The proportion of diffuse deposition of complement activated components in renal tissues in patients with severe cardiorenal injury is higher than that in patients with non-severe cardiorenal injury. Overactivation of complement may be involved in the pathogenic process of severe heart and kidney injury caused by MHT.

Hematopoietic stem cell transplantation associated thrombotic microangiopathy / 中国小儿急救医学

Hematopoietic stem cell transplantation related thrombotic microangiopathy (TA-TMA) is a clinical syndrome characterized by microvascular hemolytic anemia, thrombocytopenia and involvement of end organ.The pathogenesis of TA-TMA involves vascular endothelial cell injury and abnormal activation of complement system.The risk factors include conditioning regimens, graft-versus-host disease, immunosuppressants, infection and HLA compatibility.Timely diagnosis and early initiation of appropriate treatment are essential to prevent multiple organ dysfunction and eventual death.At present, the lack of clinical evidence of TA-TMA has led to the fact that its diagnostic criteria and treatment have not been unified.The diagnosis and treatment suggestions put forward by domestic and foreign experts are intended to help clinicians evaluate potential TA-TMA, establish diagnosis in time, and give reasonable treatment and management.

Brote de 4 casos leptospirosis de un grupo familiar en zona rural tropical de Colombia: un reporte de caso / Outbreak of 4 leptospirosis cases in a family group in tropical rural zone of Colombia: a case report

La leptospirosis es una zoonosis con manifestaciones clínicas causadas por espiroquetas patógenas del género Leptospira spp. Su curso puede ser desde enfermedad leve hasta un síndrome ictero-hemorrágico severo denominado enfermedad de Weil. Se estudió un brote epidemiológico constituido por una serie de cuatro casos de leptospirosis de severidad moderada a severa, ocurridos en una zona rural de clima tropical en los llanos orientales de Colombia, a 450 metros sobre el nivel del mar en una familia visitante en Puerto Lleras, Meta, Colombia. Probablemente secundario al consumo de aguas contaminadas por orina de roedores. Las pacientes fueron ingresadas bajo la sospecha de un síndrome ictérico de origen infeccioso con un falso positivo para antígeno de superficie de hepatitis B. Dos pacientes desarrollaron síndrome de Weil asociado a microangiopatía trombótica por lo que requirieron manejo en unidad de cuidados intensivos (UCI) sin embargo, una de ellas fallece y las otras dos pacientes desarrollan signos y síntomas moderados evidenciando un curso variable de la enfermedad. En nuestro país existe una sobre notificación de síndromes febriles, ictéricos e icterohemorragicos de diferentes etiologías y difícil diagnóstico por lo que la leptospirosis tiende a ser confundida o ignorada como diferencial en muchos casos.

Leptospirosis is a zoonosis with clinical manifestations caused by pathogenic spirochetes of the genus Leptospira spp. Its course can range from mild illness to a severe jaundice-hemorrhagic syndrome called Weil's disease. An epidemiological outbreak consisting of a series of four cases of leptospirosis of moderate to severe severity, which occurred in a rural area with a tropical climate in the eastern plains of Colombia, at 450 meters above sea level, was studied in a visiting family in Puerto Lleras, Meta, Colombia. Probably secondary to the consumption of water contaminated by rodent urine. The patients were admitted on suspicion of an infectious jaundice syndrome with a false positive for hepatitis B surface antigen. Two patients developed Weil's syndrome associated with thrombotic microangiopathy, requiring ICU management, however, one of them died and the other two patients develop moderate signs and symptoms showing a variable course of the disease. In our country there is an overreporting of febrile, jaundice and jaundice syndromes of different etiologies and difficult diagnosis, so that leptospirosis tends to be confused or ignored as differential in many cases.

Scleredema Diabeticorum / Escleredema Diabeticorum Scleredema Diabeticorum

Abstract Scleredema diabeticorum is one of the skin disorders associated with diabetes mellitus, characterized by thickening of the deep layers of the dermis, with excessive mucin and collagen deposition, clinically evidenced in hardening of the skin, especially in the upper half of the body. We describe the clinical case of an adult male diabetic who was seen for an indurated cervical lesion which was subsequently diagnosed histopathologically as scleredema diabeticorum. The interest in this case lies in the low prevalence of the condition and its association with poor metabolic control of diabetes. (Acta Med Colomb 2021; 46. DOI:https://doi.org/10.36104/amc.2021.1888).

Resumen Dentro de los trastornos cutáneos asociados a la diabetes mellitus, se describe el escleredema diabeticorum el cual se caracteriza por un engrosamiento de las capas profundas de la dermis con depósito excesivo de mucina y colágeno, que produce clínicamente endurecimiento de la piel, principalmente en la mitad superior del cuerpo. Se describe un caso clínico correspondiente a un hombre adulto diabético que consultó por la aparición de una lesión indurada en la región cervical con posterior diagnóstico histopatológico de escleroderma diabeticorum. El interés radica en la baja prevalencia de la condición y su asociación al pobre control metabólico de la diabetes. (Acta Med Colomb 2021; 46. DOI:https://doi.org/10.36104/amc.2021.1888).

Púrpura trombocitopénica trombótica en un paciente con infección por VIH / Thrombotic thrombocytopenic purpura, in a HIV-infected patient: case report

Resumen Las manifestaciones hematológicas de la infección por el VIH son frecuentes y variadas debido a su capacidad de afectar prácticamente todas las líneas celulares. Dentro de éstas, la púrpura trombocitopénica trombótica (PTT) es una de las entidades que constituyen las microangiopatías trombóticas. Se caracteriza por la presencia de trombocitopenia y anemia hemolítica microangiopática con alteración de la función renal. Actualmente, la co-existencia de estas dos entidades es poco frecuente debido a la terapia anti-retroviral de alta efectividad (TARV) Presentamos el caso de un paciente de 28 años, quien consultó por fiebre asociada a episodios de gingivorragia, palidez mucocutánea generalizada y debilidad progresiva. Los estudios evidenciaron una anemia y trombocitopenia grave. Se encontraron esquistocitos y microesferocitos en el frotis de sangre periférica con actividad de la enzima ADAMTS 13 disminuida (6,8%). Se confirmó el diagnóstico de una PTT como manifestación inicial de una infección por VIH. Se indicó manejo con plasmaféresis e inicio de TARV con buena respuesta.

Abstract Hematological manifestations for human immunodeficiency virus (HIV) infection are frequent and diverse due to its ability to affect almost all cell lines. Among these, thrombotic thrombocytopenic purpura (TTP) is one of the thrombotic microangiopathies syndromes, characterized by the presence of thrombocytopenia and microangiopathic hemolytic anemia with impaired renal function. Nowadays, the relationship between these two entities is rare given the current highly active antiretroviral therapy (HAART). We report the case of a 28-year-old patient, who presented with fever associated with gingival bleeding, generalized mucocutaneous pallor and progressive weakness. Routine investigations showed anemia and severe thrombocytopenia, schistocytes and micro spherocytes in peripheral blood smear. Required blood transfusion, with decreased ADAMTS 13 enzyme activity (6.8%). With these findings,TTP was diagnosed as the initial manifestation of the HIV infection. The patient received management with five sessions of plasmapheresis and HAART with subsequent improvement.

Clinical value of plasma miR-27 expression in patients with diabetic retinopathy / 国际眼科杂志(Guoji Yanke Zazhi)

@#AIM: To investigate the clinical value of microRNA(miRNA, miR)-27 expression in patients with diabetic retinopathy(DR).<p>METHODS: A total of DR 80 patients(DR group)treated between January 2019 and January 2020 were retrospectively reviewed. Meanwhile, 40 patients with simple type 2 diabetes mellitus(T2DM)(T2DM group)and 40 normal healthy persons(control group)were enrolled, and plasma RNA was extracted. Real time fluorescent quantitative reverse transcription polymerase chain reaction(RT-PCR)was adopted to determine plasma miR-27 expression, and enzyme-linked immunosorbent assay was performed to determine the vascular endothelial growth factor(VEGF)level. Plasma miR-27 and serum VEGF expression in different groups and in patients with different severities of DR was comparatively analyzed. Multivariate Logistic regression analysis was performed to screen factors influencing the expression of miR-27 in patients with DR, and Pearson correlation analysis of miR-27, serum VEGF and blood glucose indexes was conducted. Meanwhile, significance of miR-27 in pathogenesis of DR was summarized.<p>RESULTS: DR group had the highest plasma miR-27 and serum VEGF levels, followed by T2DM group, and then the control group(<i>P</i><0.05). Proliferative diabetic retinopathy(PDR)patients had higher levels of plasma miR-27, serum VEGF, fasting blood glucose and glycated hemoglobin than those with non-proliferative diabetic retinopathy(NPDR)(<i>P</i><0.05). It was found that course of disease(<i>OR</i>=3.206), fasting blood glucose(<i>OR</i>=2.570), glycated hemoglobin(<i>OR</i>=2.787), VEGF(<i>OR</i>=3.442)and severity of DR(<i>OR</i>=5.842)were influencing factors of plasma miR-27 expression in DR patients(<i>P</i><0.05). In DR patients, relative expression of plasma miR-27 was positively correlated with serum VEGF, fasting blood glucose and glycated hemoglobin(<i>r</i>=0.548, 0.398, 0.522, all <i>P</i><0.05).<p>CONCLUSION: DR patients have higher plasma miR-27 expression level than those with simple T2DM and normal healthy people. The duration of diabetes, fasting blood glucose, glycated hemoglobin and severity of DR all affect the expression of miR-27. Besides, miR-27 is positively correlated with serum VEGF, glycated hemoglobin and fasting blood glucose. It is speculated that miR-27 may mediate the pathogenesis and progression of DR by regulating glucose metabolism and promoting angiogenesis.

Efficacy of individualized rituximab as the rescue therapy for active lupus nephritis with acute kidney injury / 中华肾脏病杂志

Objective:To investigate the efficacy and safety of individualized rituximab rescue therapy for active lupus nephritis with acute kidney injury (AKI).Methods:The clinical data of lupus nephritis patients with AKI treated with rituximab at the Kidney Disease Center of the First Affiliated Hospital of Zhejiang University School of Medicine from April 2017 to June 2020 were collected, and the renal remission rate and adverse events after rituximab treatment were analyzed retrospectively. The Kaplan-Meier method was used to calculate the cumulative incidence of patients' remission.Results:There were 13 patients enrolled, including 8 females, and aged (35.23±15.92) years old. The urinary protein/creatinine ratio was (5.22±1.57) g/g before rituximab treatment. Four patients were on dialysis at admission, and 9 patients without dialysis had serum creatinine of (223.22±85.73) μmol/L. Eight patients were confirmed as proliferative lupus nephritis by renal biopsies, including 7 cases with crescent formation and 1 case with thrombotic microangiopathy (TMA), and the other 5 cases without renal biopsies were clinically diagnosed as TMA. The dose of rituximab was (815±516) mg (200-2 100 mg), and all the patients reached the state of peripheral blood B cells clearance (CD19 + B cell count was<5/μl). After the first treatment of rituximab, the median time to B-cell clearance was 21(15, 35) days, and 8 patients reached B-cell depletion (CD19 + B cell count was 0). The remission rate was 12/13 (two cases reached complete remission, and 10 cases reached partial remission). Three cases stopped dialysis, and 1 case (with glomerulosclerosis of 52.94%) entered maintaining dialysis. The relapse times in the maintenance remission period of 7 patients with refractory lupus nephritis declined significantly from (1.57±0.53) times in a median history of 60(20, 109) months to (0.43±0.79) times in a median history of 18(10, 23) months after the use of rituximab ( P=0.015). After using rituximab, the incidence of infection was 7/13. The median time from the use of rituximab to infection was 26(4, 44) days. Pulmonary infection (5/13) was the most common type and all infected patients recovered after anti-infection treatment. Conclusions:Rituximab can be used in the treatment of active lupus nephritis with AKI, especially in patients with crescent formation and TMA, but the infection should be paid close attention to and prevented.

Clinical characteristics and early recognition of thrombotic microangiopathy in children with systemic lupus erythematosus / 中国小儿急救医学

Objective:To analyze the clinical characteristics of children diagnosed with systemic lupus erythematosus(SLE)complicated with thrombotic microangiopathy(TMA)for early recognition.Methods:We retrospectively reviewed the clinical records of 14 SLE patients with TMA hospitalized at Shanghai Children′s Medical Center, Shanghai Jiaotong University School of Medicine from December 2005 to October 2020.Results:The incidence of TMA was 5.65%(14/248)of the hospitalized patients with SLE and 7.87%(14/178)of the hospitalized patients with lupus nephritis.Four patients were boys while ten patients were girls.One boy was six years old and other 13 patients were from 11 to 18 years old.Their SLEDAI scores ranged from 14 to 31, and all of them were severe activity.Renal biopsy of 11 patients during TMA course all revealed lupus nephritis(type Ⅳ, n=8; type Ⅳ+ Ⅴ, n=3). These 14 SLE children were diagnosed with TMA within 3 days to 2 months after admission.At the beginning of the hospitalization, only six patients had both anemia and thrombocytopenia, while eight patients only had moderate anemia.All of the patients had obvious hypocomplementemia.Especially in the patients with first onset of SLE without treatment, their serum levels of C3 were less than 0.17 g/L and C4 were less than 0.07 g/L.Moreover, glomerular filtration rates of these patients were lower than that in normal range.The follow-up time were 0.2-11.3 years(median time was 2.6 years). After treatment, six patients obtained complete remission, and five patients obtained partial remission.One patient had sudden death during the 4th plasmapheresis, and the other two patients deteriorated. Conclusion:Children with SLE and TMA are mostly in severe disease activity, and renal pathology is type Ⅳ lupus nephritis.The SLE children with anemia should be paid special attention to the level of serum complement whether they have thrombocytopenia or not.If the level of serum complements decrease obviously, glomerular filtration rates should be monitored closely and schistocytes should be searched repeatedly in the blood smears of the peripheral blood to facilitate the early recognition of TMA.