ABSTRACT
Objective @#To explore the prevalence of uremic pruritus (UP) in patients with maintenance hemodialy sis (MHD) in Anhui Province and its influential factors .@*Methods @#Patients with MHD were enrolled in 27 hemo dialysis centers in Anhui Province . Clinical data were compared .@*Results @#A total of 3 025 patients with MHD were included . The prevalence of UP was 63.3% , among them , mild UP 55.9% and moderate to severe UP 7.4% . The prevalence rates of UP in southern Anhui , central Anhui and northern Anhui were 75.4% , 63.6% and 57.9% . The prevalence of total UP in ≤30 years , 31 - 50 years , 51 - 70 years and ≥71 years was 53.5% ,59.8% , 65.4% and 65.9% . The prevalence of total UP and moderate to severe UP increased with age ( P < 0.01) . Age , age of dialysis , proportion of hypertension , 25(hydroxy) vitamin D3 [25(OH)D3 ] , proportion of low flux dialyzer usage and proportion of calcium phosphorus binder usage in UP group were higher than those in the group without UP. However , the levels of diastolic blood pressure , hemoglobin ( Hb) and hemodialysis filtration ratio in the UP group were lower than those in the non UP group (P < 0.05) . By comparison , the age , hyperten sion and diabetes of patients in moderate and severe UP group were higher than those in mild UP group , while the proportion of non calcium phosphorus binding was lower than that in mild UP group ( P < 0.05) . Binary Logistic regression analysis showed that high 25(OH)D3 was associated with a higher risk of UP in MHD patients , and high throughput dialyzer use was associated with a lower risk of UP in MHD patients ( P < 0.05) .@*Conclusion @#The prevalence rate of UP in maintenance hemodialysis patients in Anhui province is 63.3% . The prevalence of UP is the highest in southern Anhui , and the prevalence of total UP and moderate to severe UP increases with age . High 25(OH)D3 levels are a risk factor for UP in MHD patients , and the use of high throughput dialyzers can reduce the risk of UP in MHD patients .
ABSTRACT
El síndrome hemolítico urémico secundario a Streptococcus pneumoniae (SHU-Sp) es una complicación poco frecuente de las enfermedades invasoras por S. pneumoniae. Presenta una alta morbimortalidad, con requerimiento de transfusiones de glóbulos rojos y plaquetas, terapia de sustitución de la función renal de inicio precoz y más prolongada, así como mayores complicaciones a largo plazo, comparado con las formas secundarias a infección entérica por Escherichia coli productora de toxina Shiga. Presentamos el caso clínico de una preescolar de dos años, previamente sana, vacunada con tres dosis de PCV13, que desarrolló una insuficiencia renal aguda, anemia hemolítica y plaquetopenia, en el contexto de una neumonía con empiema y bacteriemia por S. pneumoniae.
Streptococcus pneumoniae associated hemolytic uremic syndrome (Sp-HUS) is an uncommon complication of invasive pneumococcal infections. Patients with Sp-HUS have a higher mortality and long term morbidity than those due to HUS from Shiga toxin-producing Escherichia coli infections (STEC-HUS). They often require more red blood cells and platelet transfusions, and early initiation of renal substitution therapy, presenting a higher rate of arterial hypertension and chronic renal disease in the long term, compared to STEC-HUS. We report a healthy 2 year-old infant, vaccinated with three doses PCV13, that developed acute renal failure, hemolytic anemia and thrombocytopenia in the course of a complicated pneumococcal pneumonia with empyema and bacteremia.
Subject(s)
Humans , Female , Child, Preschool , Pneumococcal Infections/complications , Hemolytic-Uremic Syndrome/etiology , Pneumococcal Infections/therapy , Pneumococcal Infections/diagnostic imaging , Streptococcus pneumoniae , Thrombocytopenia , Radiography, Thoracic , Renal Insufficiency , Hemolytic-Uremic Syndrome/therapy , Hemolytic-Uremic Syndrome/diagnostic imagingABSTRACT
Introducción. Existen limitados reportes epidemiológicos del síndrome urémico hemolítico (SUH) en Latinoamérica. Objetivo. Describir la frecuencia y características de las hospitalizaciones por SUH en niños de 0 a 14 años. Métodos. Se realizó un estudio descriptivo de análisis secundario con datos de hospitalizaciones por SUH de instituciones de salud de Perú en el periodo 2015-2022. Resultados. Se registraron 228 hospitalizaciones. El 52,2% fueron varones. El 88,6% fue menor de cinco años y el 11% menor de un año. Los departamentos con más hospitalizaciones fueron Lima y Arequipa con 133 y 46 casos. Las instituciones del Ministerio de Salud (MINSA) y del Seguro Social (EsSalud) reportaron el 42,5% y 27,6% de las hospitalizaciones. Del 2015 al 2022, la incidencia varió de 0,42 a 1,13 casos/100 000 niños menores de cinco años. Conclusiones. Las hospitalizaciones por SUH fueron más frecuentes en menores de cinco años y en las instituciones del MINSA.
Introduction. There are limited epidemiological reports of hemolytic uremic syndrome (HUS) in Latin America. Objective. To describe the frequency and characteristics of hospitalizations due to HUS in children aged 0 to 14 years. Methods. Descriptive study of secondary analysis was performed with data on hospitalizations due to HUS from healthcare institutions from Peru, 2015-2022. Results. Two hundred twenty-eight hospitalizations were registered, 52.2% were male, 88.6% were under five years old, and 11% were under one year old. The departments with more hospitalizations were Lima (133 cases) and Arequipa (46 cases). The institutions of the Ministry of Health (MINSA) and Social Security (EsSalud) reported 42.5% and 27.6% of hospitalizations. From 2015 to 2022, the incidence ranged from 0.42 to 1.13 cases/100,000 children under five years of age. Conclusions. Hospitalizations due to HUS were more frequent in children under five years of age and MINSA institutions.
ABSTRACT
ABSTRACT The covid-19 vaccine confers direct protection and reduces transmission rates of the virus and new variants. Vaccines from Pfizer/BioNTech and CoronaVac have been cleared for children in Brazil. They are safe, effective, and immunogenic. There are no known complications associated with the use of steroids or vaccines in pediatric patients with covid-19 and nephrotic syndrome. With or without immunosuppression, these patients are not at increased risk of severe covid-19, and steroids are safe for them. A milder form of covid-19 occurs in patients with chronic kidney disease without the need for hospitalization. The vaccine response may be reduced and/or the duration of antibodies after vaccination may be shorter than in the general population. However, considering risk of exposure, vaccination against covid-19 is recommended. It is believed that patients with hemolytic-uremic syndrome are at higher risk of severe covid-19. Vaccination is recommended, although specific data on the safety and efficacy of the covid-19 vaccine are limited. There is agreement that the benefits of induced immunity outweigh the risks of immunization. Vaccination against covid-19 is recommended for children and adolescents needing kidney transplantation or who have undergone transplantation. These patients present decreased immune response after vaccination, but immunization is recommended because the benefits outweigh the risks of vaccination. Current recommendations in Brazil stipulate the use of the messenger RNA vaccine. This paper aims to provide pediatric nephrologists with the latest knowledge about vaccination against covid-19 for children with kidney disease.
Resumo A vacina covid-19 confere proteção direta, reduz as taxas de transmissão do vírus e de novas variantes. No Brasil, estão liberadas para a população pediátrica as vacinas Pfizer/BioNTech e a CoronaVac, ambas seguras, eficazes e imunogênicas. Pacientes pediátricos com síndrome nefrótica e covid-19 têm curso clínico regular sem complicações relacionadas ao uso de esteroides ou vacinas. Esses pacientes, com ou sem imunossupressão, não apresentam maior risco de covid-19 grave e o tratamento com esteroides é seguro. Os pacientes com doença renal crônica têm covid-19 mais leve, sem necessidade de hospitalização. A resposta vacinal pode ser reduzida e/ou a duração dos anticorpos pós-vacinação pode ser menor do que na população geral. Entretanto, a vacina covid-19 está recomendada, considerando o risco de exposição. Acredita-se que pacientes com síndrome hemolítico-urêmica teriam maior risco de covid-19 grave. A vacina é recomendada, embora dados específicos sobre segurança e eficácia da vacina covid-19 sejam limitados. Há concordância que os benefícios da imunidade induzida superam quaisquer riscos da imunização. A vacina covid-19 é recomendada para crianças e adolescentes candidatos ao transplante renal ou já transplantados. Esses pacientes têm resposta imunológica reduzida após a vacina, entretanto ela é recomendada porque os benefícios superam qualquer risco dessa vacinação. A recomendação atual no Brasil é a vacina de tecnologia RNA mensageiro. O objetivo deste documento é levar aos nefrologistas pediátricos os conhecimentos mais recentes sobre a vacinação contra contra-19 em crianças com doenças renais.
ABSTRACT
RESUMEN El propósito del presente estudio fue describir las características clínicas, epidemiológicas, laboratoriales, tratamiento y seguimiento de pacientes con síndrome urémico hemolítico (SUH). Se revisaron las historias clínicas de los pacientes con SUH hospitalizados en el Instituto Nacional de Salud del Niño-Breña (INSN-B) de Lima, Perú. Se incluyeron a 83 pacientes. La mediana de edad fue de 22 meses. El 71,1% (n=59) registró uso previo de antibióticos. El 86,8% (n=72) tuvieron oligoanuria y el 74,6% (n=62) diarrea. Cinco cultivos fueron positivos (dos Escherichia coli enterohemorrágica). Cuarenta y nueve (59%) requirieron terapia de reemplazo renal. Ningún paciente falleció durante la hospitalización. Al año del seguimiento, siete pacientes presentaron nefropatía pos-SUH. En conclusión, en el INSN-B, la mediana de edad fue similar que años anteriores y hubo una mayor frecuencia de oligoanuria y terapia de reemplazo renal en comparación con reportes previos.
ABSTRACT This study aimed to describe the clinical-epidemiological, laboratory, treatment, and follow-up characteristics of patients with hemolytic uremic syndrome (HUS). The medical records of patients with HUS hospitalized at the Instituto Nacional de Salud del Niño-Breña (INSN-B) (Lima, Peru) were reviewed. We evaluated 83 patients. The median age was 22 months (interquartile range: 14 to 30 months). Of the sample, 71.1% (59) registered previous use of antibiotics. Seventy-two (86.8%) had oligoanuria and 62 (74.6%) had diarrhea. Five cultures were positive (two enterohaemorrhagic Escherichia coli). Forty-nine (59%) required renal replacement therapy. No patient died during hospitalization. At one year of follow-up, seven patients developed post-HUS nephropathy. In conclusion, in INSN-B, the median age was like previous years and there was a higher frequency of oligoanuria, and renal replacement therapy compared to previous reports.
Subject(s)
Humans , Male , Female , Infant, Newborn , Infant , Child, Preschool , ChildABSTRACT
Abstract Introduction: A better understanding of hemolytic-uremic syndrome (HUS) pathophysiology significantly changed its treatment and prognosis. The aim of this study is to characterize the clinical features, severity, management, and outcomes of HUS patients. Materials and Methods: Retrospective study of HUS patients admitted to a Pediatric Nephrology Unit between 1996 and 2020. Demographic and clinical data regarding etiology, severity, treatment strategies, and patient outcome were collected. Results: Twenty-nine patients with HUS were admitted to our unit, but four were excluded. Median age at diagnosis was two years (2 months - 17 years). Clinical manifestations included diarrhea, vomiting, oliguria, hypertension, and fever. During the acute phase, 14 patients (56%) required renal replacement therapy. Infectious etiology was identified in seven patients (five Escherichia coli and two Streptococcus pneumoniae). Since 2015, 2/7 patients were diagnosed with complement pathway dysregulation HUS and there were no cases of infectious etiology detected. Six of these patients received eculizumab. The global median follow-up was 6.5 years [3 months-19.8 years]. One patient died, seven had chronic kidney disease, four of whom underwent kidney transplantation, one relapsed, and seven had no sequelae. Conclusion: These results reflect the lack of infectious outbreaks in Portugal and the improvement on etiological identification since genetic testing was introduced. The majority of patients developed sequels and mortality was similar to that of other countries. HUS patients should be managed in centers with intensive care and pediatric nephrology with capacity for diagnosis, etiological investigation, and adequate treatment. Long-term follow-up is essential.
Resumo Introdução: Um melhor entendimento da fisiopatologia da síndrome hemolítico-urêmica (SHU) mudou significativamente seu tratamento e prognóstico. Este estudo teve como objetivo caracterizar condições clínicas, gravidade, manejo e desfechos de pacientes com SHU. Materiais e Métodos: Estudo retrospectivo de pacientes com SHU admitidos numa Unidade de Nefrologia Pediátrica entre 1996-2020. Foram coletados dados demográficos e clínicos sobre etiologia, gravidade, estratégias de tratamento, desfechos de pacientes. Resultados: 29 pacientes com SHU foram admitidos em nossa unidade, mas quatro foram excluídos. A idade mediana ao diagnóstico foi dois anos (2 meses-17 anos). Manifestações clínicas incluíram diarreia, vômitos, oligúria, hipertensão e febre. Durante a fase aguda, 14 pacientes (56%) necessitaram de terapia renal substitutiva. Identificou-se a etiologia infecciosa em sete pacientes (cinco Escherichia coli; dois Streptococcus pneumoniae). Desde 2015, 2/7 pacientes foram diagnosticados com SHU por desregulação da via do complemento e não foram detectados casos de etiologia infecciosa. Seis desses pacientes receberam eculizumab. A mediana global de acompanhamento foi 6,5 anos [3 meses-19,8 anos]. Um paciente faleceu, sete apresentaram doença renal crônica, sendo quatro submetidos a transplante renal, uma recidiva e sete sem sequelas. Conclusão: Estes resultados refletem a ausência de surtos infecciosos em Portugal e a melhoria na identificação etiológica desde que os testes genéticos foram introduzidos. A maioria dos pacientes desenvolveu sequelas e a mortalidade foi semelhante à de outros países. Pacientes com SHU devem ser manejados em centros com cuidados intensivos e nefrologia pediátrica com capacidade para diagnóstico, investigação etiológica e tratamento adequado. O acompanhamento alongo prazo é essencial.
ABSTRACT
El síndrome urémico hemolítico (SUH), descripto en 1955, se caracteriza por la tríada de anemia hemolítica no inmunomediada, trombocitopenia y lesión renal aguda. En su patogenia interviene la toxina Shiga, producida con mayor frecuencia por E. coli O157:H. Puede manifestarse a cualquier edad, aunque es infrecuente en adultos, y se desarrolla en forma esporádica o en brote. Se presenta con un cuadro de dolor abdominal, diarrea, fiebre y vómitos. Puede afectar el sistema nervioso central, pulmones, páncreas y corazón. En adultos, el síndrome evoluciona tras un período de incubación de 1 semana posterior a la diarrea y tiene alta morbimortalidad, a diferencia de los casos pediátricos. Presentamos el caso de una paciente adulta, que cursó internación por síndrome urémico hemolítico. (AU)
Hemolytic uremic syndrome (HUS), described in 1955, is characterized by the triad of non-immune mediated hemolytic anemia, thrombocytopenia, and acute kidney injury. Shiga toxin, produced most frequently by E coli O157:H, is involved in its pathogenesis. Hus can manifest at any age, although it is rare in adults and develops sporadically or in outbreaks. HUS presents with a picture of abdominal pain, diarrhea, fever and vomiting. It can affect the central nervous system, lungs, pancreas, and heart.In adults, the syndrome evolves after an incubation period of 1 week after diarrhea, with high morbidity and mortality, unlike pediatric cases.We present the case of an adult patient who was hospitalized for hemolytic uremic syndrome. (AU)
Subject(s)
Humans , Female , Middle Aged , Escherichia coli O157/isolation & purification , Escherichia coli Infections/complications , Hemolytic-Uremic Syndrome/pathology , Hemolytic-Uremic Syndrome/diagnostic imaging , Polymerase Chain Reaction , Diarrhea/etiology , Hemolytic-Uremic Syndrome/diet therapy , Hemolytic-Uremic Syndrome/blood , Hemolytic-Uremic Syndrome/therapy , Infusions, Parenteral , Kidney Function TestsABSTRACT
Chronic kidney disease (CKD) is a global health problem, and its incidence increases year by year. Studies have revealed that the progression of CKD into end-stage renal disease (ESRD) is related to its inability to effectively eliminate toxins due to decreased renal function. Additionally, intestinal microflora produces a large amount of gut-derived uremic toxins (GDUTs) during protein fermentation. The theory of gut-kidney axis holds that gut and kidney interact with each other, and CKD reduces the ability to remove uremic toxins (UTs), resulting in the accumulation of UTs in the blood. The accumulation of UTs also accelerates the deterioration of renal function, leading to a vicious circle. This paper focused on the sources of indoxyl sulfate and p-cresol sulfate in GDUTs and their mechanisms against CKD (such as inducing renal tubular cell death, oxidative stress and endothelial injury, promoting renal fibrosis and down-regulating renal protective protein) as well as the sources of trimethylamine oxide and its mechanisms against CKD (such as promoting renal fibrosis and inflammation). Moreover, starting from gut-kidney axis, this paper summarized the ways of diet and nutrition regulation, toxin adsorption, enhanced dialysis to increase the clearance, inhibiting the sources of gut-derived toxins and traditional Chinese medicine (TCM) therapy (TCM preparations and TCM active ingredients) to regulate intestinal microecology and reduce the generation of GDUTs, aiming to provide new therapeutic ideas for delaying the progression of CKD.
ABSTRACT
This research aimed to study the effect of Uremic Clearance Granules on chronic kidney disease in SD rats by using the methods of microbial functional genomics combined with metabolomics, and to preliminarily explore its mechanism. The SD rat model of chronic kidney disease was established by the adenine-induced method. After the model was successfully induced, the animals were randomly divided into a negative control group, a Uremic Clearance Granule treatment group, and a normal control group, with 8 rats in each group. After 4 weeks of administration, animal feces and serum were collected, and 16S rDNA sequencing technology was used to analyze the abundance, diversity, and function prediction of intestinal microorganisms. Liquid chromatography-mass spectrometry(LC-MS) technology was used to perform high-throughput sequencing to detect animal serum metabolites. The MetPA database was used to screen out potential biomarkers of chronic kidney disease in rats and conduct the enrichment analysis of metabolic pathways. Spearman's method was used to analyze the correlation between the two omics. The results showed that Uremic Clearance Granules effectively improved the body weight loss and renal function-related biochemical and appearance indicators in rats with chronic kidney disease. The results of 16S rDNA sequencing showed that Uremic Clearance Granules regulated the diversity and composition of the intestinal flora in rats with chronic kidney disease. The changes in the intestinal flora affected functional metabolic pathways such as amino acid biosynthesis and metabolism, lipid metabolism, and carbohydrate metabolism. The results of LC-MS showed that as compared with the negative control group, 15 metabolites were reversed in the Uremic Clearance Granule treatment group, among which 11 potential marker metabolites were significantly up-regulated and 4 potential marker metabolites were significantly down-regulated. Five amino acid metabolic pathways were mainly involved, which were significantly correlated with changes in the intestinal flora. Therefore, Uremic Clearance Granules can improve the renal function of rats with chronic kidney disease, and the mechanism may be related to its effect on the amino acid metabolism pathway by regulating the intestinal flora.
Subject(s)
Rats , Animals , Rats, Sprague-Dawley , Renal Insufficiency, Chronic/drug therapy , Metabolomics/methods , Gastrointestinal Microbiome , Amino AcidsABSTRACT
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.
ABSTRACT
Objective:A kinetic model of the clearance of protein-bound uremic toxins (PBUTs) in maintenance hemodialysis patients is established to evaluate the effects of adding sorbents with different sorption efficiency to dialysate on the clearance rate of PBUTs, and to predict the sorption efficiency of sorbents using the model.Methods:The kinetic model was established by integrating the parameters of plasma flow rate ( Qp), dialysate flow rate ( Qd), free plasma fraction of PBUTs ( f1), free dialysate fraction of PBUTs ( f2), mass transfer coefficient of dialyzer ( K) and surface area of dialysis membrane ( A), and using the mass balance equation and Fick's first law. The model was also used to evaluate the relationship between the clearance rate of different PBUTs and the parameters of dialyzer and the sorption efficiency of sorbents. Results:The kinetic model of PBUTs clearance (CL): ?CL=Qp1-f1-f2γφf1-f2γ,?γ=QpQd,?φ=eKAf1Qp-f2Qd. The model was used to analyze the dialysis parameters of indoxyl sulfate and p-cresol sulfate dialysis.The clearance rate of PBUTs increased with the decrease of its binding capacity to albumin in plasma and the increase of plasma flow rate in dialyzer, dialysate flow rate, mass transfer coefficient of dialyzer, surface area of dialysis membrane, and sorption capacity of sorbents in dialysate. The increasing trend of PBUTs clearance rate was particularly obvious after applying sorbents. Further analysis of the dialysate flow rate and the sorption efficiency of sorbents in the dialysate showed that the increase of the dialysate flow rate could make up for the difference of the sorption efficiency of sorbents. When the dialysate flow rate tended to be infinite, the sorption efficiency of sorbents in the dialysate had no effect on the clearance rate of PBUTs. Conclusion:Adding sorbents of PBUTs to the dialysate during dialysis can significantly improve the clearance rate of PBUTs, suggesting a promising clinical application value.
ABSTRACT
Atypical hemolytic uremic syndrome (aHUS) is characterized by non-immune microangiopathic hemolytic anemia, thrombocytopenia and acute kidney injury. IgA nephropathy (IgAN) is a common primary glomerular disease, while aHUS combined with IgAN is rare, and has been reported rarely in the past. This paper reported a case of children with aHUS combined with IgAN. In the acute stage, she was treated with plasma therapy and glucocorticoid. After remission, she was treated with glucocorticoid combined with immunosuppressants. During the follow-up period, aHUS did not recur, the renal function was normal, and urinary protein decreased to weakly positive.
ABSTRACT
Streptococcus pneumoniae-associated hemolytic uremic syndrome(SP-HUS) is a thrombotic microvascular disease caused by streptococcus pneumoniae infection, which is one of the causes renal failure in children.Clinical manifestations include thrombocytopenia, hemolytic anemia and acute renal failure.The neuraminidase and surface protein C substance of Streptococcus pneumoniae, immune factors and complement play important roles in the pathogenesis of SP-HUS.The complement inhibitors provides a new solution for the treatment of SP-HUS.However, few SP-HUS cases have been reported in China.This paper reviews the pathogenesis, clinical manifestations and treatment of SP-HUS to help clinicians better understand the disease, early recognition and intervention treatment.
ABSTRACT
Atypical hemolytic uremic syndrome (aHUS) is a rare acute and critical disease in childhood, which is easy to lead to acute kidney injury, and has a high mortality rate and chronic kidney disease incidence if not diagnosed and treated in time.According to the etiology, aHUS can be classified into hereditary and acquired.Anti-factor H antibody associated aHUS is acquired and mainly occurs in children aged 5 to 15 years.Anti-factor H antibody associated aHUS is strongly associated with homozygous deletion of the complement H related protein 1/3 (CFRH1/3) gene.In recent years, there have been significant advances in the etiology, genetics, and immunology of aHUS, especially the treatment of Eculizumab, which has greatly improved the prognosis of the disease.However, at present, there are still problems that need to be solved in the pathogenesis, diagnosis, treatment and prognosis of antibody-related aHUS, and this article will discuss the above content and put forward corresponding prospects to provide reference for clinical and scientific research.
ABSTRACT
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.
ABSTRACT
At present, clinical interventions for chronic kidney disease are very limited, and most patients rely on dialysis to sustain their lives for a long time. However, studies on the gut-kidney axis have shown that the gut microbiota is a potentially effective target for correcting or controlling chronic kidney disease. This study showed that berberine, a natural drug with low oral availability, significantly ameliorated chronic kidney disease by altering the composition of the gut microbiota and inhibiting the production of gut-derived uremic toxins, including p-cresol. Furthermore, berberine reduced the content of p-cresol sulfate in plasma mainly by lowering the abundance of g_Clostridium_sensu_stricto_1 and inhibiting the tyrosine-p-cresol pathway of the intestinal flora. Meanwhile, berberine increased the butyric acid producing bacteria and the butyric acid content in feces, while decreased the renal toxic trimethylamine N-oxide. These findings suggest that berberine may be a therapeutic drug with significant potential to ameliorate chronic kidney disease through the gut-kidney axis.
ABSTRACT
Introducción: el síndrome hemolítico urémico (SHU) es en muchos países, de las causas más frecuentes de insuficiencia renal aguda. La mayoría de los casos ocurre luego de un episodio de gastroenteritis aguda (GEA) por Escherichia coli productora de toxina Shiga (STEC). En Uruguay a pesar de ser una enfermedad de notificación obligatoria, existe subregistro. Objetivo: describir dos casos clínicos de SHU asociados a GEA con nexo epidemiológico. Casos clínicos: se trata de dos varones de 4 y 5 años, sanos. En los días previos, ingesta de carne en el mismo local comercial. Consultaron por dolor abdominal, deposiciones líquidas y vómitos reiterados. El niño de 4 años presentaba fiebre y deposiciones líquidas con sangre. El niño de 5 años dolor abdominal. El estado de hidratación y las constantes vitales eran normales en ambos. Fueron admitidos a cuidados moderados. A las 48 horas y a los 5 días, respectivamente, agregan palidez cutáneo-mucosa intensa, edemas y oliguria. Estudios complementarios: anemia, plaquetopenia e insuficiencia renal. Ingresaron a cuidados intensivos y se realizó diálisis peritoneal. La investigación de STEC fue negativa y la evolución favorable. Conclusiones: en menores de 5 años el SHU asociado a GEA es la forma de enfermedad más frecuente. En Uruguay predominan las cepas STEC no-O157. En estos casos no se pudo identificar el agente. La existencia de un nexo epidemiológico alerta sobre la necesidad de extremar los cuidados en la preparación y cocción de la carne. Debido a la asociación con una enfermedad prevalente, es necesario tener presente esta complicación para poder sospecharla e iniciar el tratamiento en forma precoz y oportuna.
Introduction: hemolytic uremic syndrome (HUS) is one of the most frequent causes of acute renal failure in many countries. Most cases occur after an episode of acute gastroenteritis (GEA) due to the Shiga toxin producing Escherichia Soli (STEC). In Uruguay, despite being a disease that requires mandatory notification, it is under reported. Objective: to describe two clinical cases of HUS associated with GEA with an epidemiological link. Clinical cases: these are two healthy boys aged 4 and 5 years. In the previous days, they reported meat intake in the same commercial premises. They consulted for abdominal pain, liquid stools and repeated vomiting. The 4 year old boy had a fever and bloody stools. The 5 year old boy had abdominal pain. They both showed normal hydration levels and vital signs. They were admitted to moderate care. At 48 hours and 5 days, respectively, they showed intense skin and mucosal paleness, edema and oliguria. Complementary studies: anemia, thrombocytopenia and renal failure. They were admitted to intensive care and peritoneal dialysis was performed. The STEC's investigation was negative and the evolution favorable. Conclusions: in children under 5 years of age, HUS associated with GEA is the most frequent form of the disease. In Uruguay, non-O157 STEC strains predominate. In these cases, the agent could not be identified. The existence of an epidemiological link warns us about the need for extreme care in the preparation and cooking of meat. Due to the association with a prevalent disease, it is necessary to keep this complication in mind in order to suspect it and initiate early and timely treatment.
Introdução: a síndrome hemolítico urêmica (SHU) é uma das causas mais frequentes de insuficiência renal aguda em muitos países. A maioria dos casos ocorre após um episódio de gastroenterite aguda (GEA) devido à Escherichia Coli, a toxina produtora de Shiga (STEC). No Uruguai, apesar de ser uma doença de notificação compulsória, há subnotificação. Objetivo: descrever dois casos clínicos de SHU associada à AGE com vínculo epidemiológico. Casos clínicos: dois meninos saudáveis com idades entre 4 e 5 anos. Nos dias anteriores, eles reportaram consumo de carne nos mesmos estabe- lecimentos comerciais. Eles consultaram para dor abdominal, fezes líquidas e vômitos repetidos. O menino de 4 anos teve febre e fezes com sangue. O menino de 5 anos teve dores abdominais. O estado de hidratação e os sinais vitais foram normais em ambos meninos. Foram internados em cuidados moderados. Às 48 horas e 5 dias, respectivamente, apresentaram aliás palidez intensa da pele e mucosas, edema e oligúria. Realizaramse estudos complementares: anemia, trombocitopenia e insuficiência renal. Eles foram internados em terapia intensiva e realizouse diálise peritoneal. A investigação do STEC foi negativa e a evolução favorável. Conclusões: em crianças menores de 5 anos, a SHU associada à GEA é a forma mais frequente da doença. No Uruguai, predominam cepas STEC não-O157. Nesses casos, o agente não pôde ser identificado. A existência de um nexo epidemiológico alerta para a necessidade de extremo cuidado no preparo e cozimento da carne. Devido à associação com doença prevalente, é necessário considerar essa complicação para suspeitar e iniciar o tratamento precoce e oportunamente.
Subject(s)
Humans , Male , Child, Preschool , Gastroenteritis/complications , Hemolytic-Uremic Syndrome/etiology , Vomiting , Abdominal Pain , Diarrhea , Fever , Red Meat/poisoning , Gastroenteritis/diagnosis , Gastroenteritis/therapy , Hemolytic-Uremic Syndrome/diagnosis , Hemolytic-Uremic Syndrome/therapyABSTRACT
Introducción: el síndrome hemolítico urémico atípico es una enfermedad severa y huérfana, la cual en su variedad atípica se presenta con manifestaciones clínicas extrarrenales y sistémicas. La presencia de afectación gastrointestinal es infrecuente, pero en los pacientes en los que se manifiesta el pronóstico desfavorable, dado que estos cursan con más recaídas y mayor mortalidad, por lo cual se hace indispensable que el personal de salud esté entrenado en detectar y reconocer las manifestaciones menos frecuentes de esta patología, para así impactar positivamente en el desenlace de estos pacientes. Objetivo: se busca ilustrar un caso singular en cuanto a la sintomatología presentada infrecuente por el paciente y sobre una etiología no descrita previamente en la literatura. Presentación del caso: se reporta el caso de un escolar masculino de 8 años que presentó fiebre, ictericia, dolor abdominal y lumbar, diarrea, hematemesis y hematuria, al cual se le diagnosticó síndrome hemolítico urémico atípico. El paciente presentó un rápido deterioro clínico con compromiso multiorgánico, documentándose hipertensión arterial y lesión renal aguda, que recibió manejo con cristaloides y diurético de asa sin mejoría, por lo que requirió inicio de terapia de reemplazo renal e incluso múltiple soporte transfusional; sin embargo, continuó sin mejoría clínica, por lo que se indicó inicio de anticuerpo monoclonal IgG humanizado recombinante con buenos resultados. Discusión y conclusión: este caso es un claro ejemplo de que la afectación extrarrenal y multiorgánica puede ser la manifestación principal de esta enfermedad, por lo que es importante que el clínico se encuentre sensibilizado y conozca los signos y los síntomas de la presentación atípica de esta patología, con el fin de evitar retrasos diagnósticos y terapéuticos. Asimismo, el abordaje etiológico es de suma importancia para brindar un pronóstico más preciso al paciente y su familia.
Introduction: Atypical hemolytic uremic syndrome is a severe and orphan disease, in its atypical variety courses with extrarenal clinical manifestations. The presence of gastrointestinal compromise is infrequent, but the prognosis is unfavorable, since they have more relapses and higher mortality. Therefore, it is important that physicians are trained in recognizing the rare manifestations of this pathology, in order to improve the outcome in these patients. Purpose: This case illustrates a unique case in terms of symptoms and etiology not previously described. Case presentation: We report the case of an 8-year-old male who presented with fever, jaundice, abdominal and lumbar pain, diarrhea, hematemesis, and hematuria. Atypical hemolytic uremic syndrome was diagnosed. He presented clinical deterioration with multiple organ involvement, documenting high blood pressure and acute kidney injury, who received management with crystalloids and diuretics without improvement, requiring renal replacement therapy and multiple transfusional support. However, there was still no clinical improvement, so the start of recombinant humanized IgG monoclonal antibody was indicated, with satisfactory outcomes. Discussion and conclusions: This case is a clear example that extrarenal and multi-organ involvement can be the main manifestation of this disease, so it is important that clinicians are aware of the clinical course that may develop a patient with atypical presentations, in order to avoid diagnostic and therapeutic delays. Likewise, the etiological approach is important in order to provide an accurate prognosis to the patient and his family.
ABSTRACT
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).
ABSTRACT
Resumen El síndrome urémico hemolítico (SUH) está caracterizado por microangiopatía trombótica, anemia hemolítica, trombocitopenia e insuficiencia renal aguda. Puede causar desde secuelas permanentes hasta muerte, principalmente en niños. En este trabajo, utilizando minería de textos (MT), se analizó el texto explícito e implícito de 16 192 artículos científicos originales sobre SUH indexados en la base de datos de Europe PMC. Los objetivos fueron examinar comportamientos, realizar seguimiento de tendencias, hacer predicciones y cruzar datos con otras fuentes de información. Para el análisis se utilizaron -entre otras herramientas infor máticas- flujos de trabajo (FT) especialmente desarrollados en la plataforma KNIME. La MT sobre las palabras de los resúmenes de las publicaciones permitió: detectar asociaciones no descritas entre eventos relacionados con SUH; extraer información subyacente; hacer agrupamientos temáticos mediante algoritmos no supervisados; realizar predicciones sobre el curso de las investigaciones asociadas al tema. Tanto el abordaje como los FT desarrollados para realizar Ciencia de Datos sobre SUH pueden aplicarse a otros temas biomédicos y a otras bases de datos científicos, permitiendo analizar aspectos relevantes en el campo de la salud humana para me jorar la investigación, la prevención y el tratamiento de múltiples enfermedades.
Abstract Hemolytic uremic syndrome (HUS) is characterized by thrombotic microangiopathy, hemolytic anemia, thrombocytopenia and acute renal failure. It can cause from permanent sequelae to death, mainly in children. In this work, using text mining (TM), we analyzed the explicit and implicit text of 16 192 original scientific articles on HUS indexed in the Europe PMC database. The objectives were to examine behaviors, track trends, and make predictions and cross-check data with other sources of information. For the analysis we used -among other computational tools- specially developed workflows (WF) in the KNIME platform. The TM on the words of the abstracts of the publications made it possible to: detect undescribed associations between events related to HUS; extract underly ing information; make thematic clustering using unsupervised algorithms; make forecasting about the course of research associated with the topic. Both the approach and the WFs developed to perform Data Science on HUS can be applied to other biomedical topics and other scientific databases, making it possible to analyze relevant aspects in the field of human health to improve research, prevention and treatment of multiples diseases.