Púrpura fulminante postinfecciosa: a propósito de un caso / Postinfectious purpura fulminans: A case report

La púrpura fulminante adquirida postinfecciosa es una entidad aguda y grave, poco frecuente, caracterizada por necrosis cutánea asociada a coagulopatía intravascular diseminada (CID), en ausencia de infección activa o alteraciones previas de la coagulación. Afecta fundamentalmente a la población pediátrica y, en el 90 % de los casos, está precedida por un proceso infeccioso. El mecanismo fisiopatológico es un déficit transitorio de proteína S mediado por autoanticuerpos que favorece un estado de hipercoagulabilidad. Se presenta el caso de un varón de 8 años previamente sano, con lesiones cutáneas purpúricas características de púrpura fulminante asociada a CID en ausencia de sepsis. Se constató deficiencia plasmática transitoria de proteína S. Requirió tratamiento sustitutivo con plasma fresco congelado y anticoagulación; la evolución fue favorable. La actividad de la proteína S permaneció disminuida durante 2 meses.

Acquired postinfectious purpura fulminans is a rare, acute, and severe disease characterized by skin necrosis associated with disseminated intravascular coagulation (DIC) in the absence of active infection or previous coagulation disorders. It mainly affects the pediatric population and, in 90% of cases, it is preceded by an infectious process. The pathophysiological mechanism is a transient autoantibodymediated protein S deficiency that favors a hypercoagulable state. Here we describe the case of a previously healthy 8-year-old boy with purpuric skin lesions typical of purpura fulminans associated with DIC in the absence of sepsis. A transient plasma protein S deficiency was confirmed. He required replacement therapy with fresh frozen plasma and anticoagulation; he had a favorable course. Protein S activity remained decreased for 2 months.

IMMUNE THROMBOCYTOPENIC PURPURA: A HAEMATOLOGICAL DISORDER

Immune (idiopathic) thrombocytopenic purpura (ITP) is an autoantibody-mediated condition characterised by an unusually low level of platelets in the bloodstream. When thrombopoiesis was not occurring quickly enough to counteract the increased rate of platelet destruction, rapid antibody-mediated platelet destruction was initially thought to be the cause of ITP. However, recent research has concentrated on the creation of therapies that boost platelet production as it has emerged that insufficient or inadequate platelet production is also a factor in low platelet counts. ITP can be acute or chronic and affects both children and adults. Because the clinical manifestation of ITP can differ greatly from patient to patient, a thorough assessment of the signs and symptoms must be done in order to manage and treat ITP effectively. Due to the lack of data on clinical and laboratory characteristics, the diagnostic method for ITP now relies heavily on a process of exclusion. Obtaining the patient's medical history and conducting a physical examination are common diagnostic techniques used on both children and adults. Patients with suspected ITP have standard laboratory tests, such as a complete blood count and a peripheral blood smear. With various levels of success, a number of specialised laboratory assays have been created. There is still room to streamline and enhance the diagnostic procedure for detecting ITP.

Research progress of Hp infection and blood system related diseases / 重庆医学

Helicobacter Pylori(Hp)is a spiral bacterium that colonized on the surface of gastric muco-sal epithelium.It is the main cause of gastrointestinal diseases because human is the only natural host and can survive in gastric acid.In recent years,relevant clinical studies have shown that Hp infection is closely related to hematological diseases such as allergic purpura(HSP),immune thrombocytopenic purpura(ITP),iron de-ficiency anemia(IDA),megaloblastic anemia(MA),lymphoma,leukemia and so on.Therefore,for Hp infec-tion,early diagnosis and treatment are of great significance for improving the efficacy of hematological diseases.

Evaluation of ultrasonography combined with serum Gastrin-17 in differential diagnosis of abdominal IgA vasculitis in children / 国际检验医学杂志

Objective To study the value of ultrasonography combined with serum Gastrin-17 in differenti-al diagnosis of abdominal IgA vasculitis in children.Methods A total of 80 children with IgA vasculitis admit-ted to the hospital from June 2020 to December 2022 were selected,including 45 cases of abdominal IgA vascu-litis(observation group)and 35 cases of other types of IgA vasculitis(without gastrointestinal symptoms,control group).The ultrasonographic characteristics and Gastrin-17 level of abdominal IgA vasculitis were an-alyzed,and the relationship between Gastrin-17 level and purpura symptom score was analyzed.Receiver oper-ating characteristic(ROC)curve was used to analyze the diagnostic value of ultrasonography and Gastrin-17 for abdominal IgA vasculitis in children.Results The symptom score of purpura in the observation group was significantly higher than that in the control group(P<0.001),while the serum Gastrin-17 level in the obser-vation group was significantly lower than that in the control group.Pearson correlation analysis showed that serum Gastrin-17 level was negatively correlated with purpura symptom score(r=-0.758,P<0.001).Ul-trasound images showed"doughnut"-like changes in the intestinal wall,with different degrees of central-thick-ness thickening and reduced echo,mainly submucosal thickening.ROC curve analysis showed that the cut-off value of serum Gastrin-17 in the diagnosis of abdominal IgA vasculitis in children was 2.91 pmol/L,the area under the curve was 0.787(95%CI:0.685-0.888),the sensitivity and the specificity were 75.56%(34/45)and 74.29%(26/35),respectively.The sensitivity of ultrasound combined with Gastrin-17 in the diagnosis of abdominal IgA vasculitis in children was 97.78%(44/45),the negative prediction rate was 95.65%(22/23),and the accuracy rate was 82.50%(66/80),which was significantly higher than those of single diagnosis(P<0.05).Conclusion Serum Gastrin-17 level is low in children with abdominal IgA vasculitis,and ultrasound imaging shows"doughnut"-like changes in the intestinal wall and thickening of the submucosa.Combined de-tection of the two could effectively differentially diagnose abdominal IgA vasculitis in children.

Analysis of Vitamin D in Children with Henoch-Schonlein Purpura / 昆明医科大学学报

Objective To analyze the changes of vitamin D in children with Henoch-Schonlein purpura(HSP).Methods 130 children with HSP from Kunming Children's Hospital between July 2022-July 2023 were selected as the study subjects and 100 healthy children were selected during the same period as the control group.The blood samples were collected from the children with HSP and the healthy children.The content of vitamin D was measured by Kunming Kingmed Institute for Clinical Laboratory.Results The content of 25(OH)D in children with HSP was lower than that in healthy children,and the difference was statistically significant(P<0.01).The proportion of vitamin D insufficiency in children with HSP was higher than that in healthy children,and the difference was statistically significant(P<0.01).Conclusion The children with HSP are prone to vitamin D insufficiency.Vitamin D supplementation may provide a new method for the treatment of HSP.

Single-center controlled study for application of hemoperfusion and versus gammaglobulin for the treatment of Henoch-Sch?nlein purpura combined gastrointestinal bleeding in children / 中华实用儿科临床杂志

Objective:To compare the clinical efficacy and safety of hemoperfusion (HP) and gammaglobulin on the treatment of Henoch-Sch?nlein purpura (HSP) with gastrointestinal bleeding in children.Methods:Case-control study.A total of 39 HSP children combined with gastrointestinal bleeding diagnosed in the Department of Pediatric Nephrology, Rheumatology and Immunology, Shengjing Hospital of China Medical University from January 2015 to December 2019 were retrospectively recruited.They were divided into the HP group and the gammaglobulin group according to the therapeutic strategy.Clinical data were collected, and a 6-month follow-up survey was conducted for monitoring the relapse of gastrointestinal bleeding and the occurrence of kidney injury.The differences between groups were compared by Fisher′s exact test, two independent samples t-test, Mann-Whitney U-test, Kruskal-Wallis H-test, and One-Way ANOVA. Results:(1) There were 20 cases in the HP group and 19 cases were included in the gammaglobulin group.The gammaglobulin group was younger than the HP treatment group.(2) In addition to gastrointestinal bleeding, children in both groups had other clinical symptoms, such as abdominal pain, angioneurotic edema, and hematuria.(3)Comparison of laboratory indexes: Inflammatory indexes: white blood cell count (WBC), C-creative protein (CRP) and coagulation function indexes: fibrin degradation products (FDP), D-dimer (DD) were significantly elevated before treatment in the 2 groups, and there was no difference between the 2 groups ( P>0.05); WBC, CRP and FDP, DD declined in the 2 groups after treatment compared with the former, and there was no difference between the 2 groups ( P>0.05); (4) Comparison of clinical manifestations: when HP was applied with gammaglobulin in the treatment window within 3 d, the difference in the time of abdominal pain relief in the HP group was shorter than that of the gammaglobulin group [1.00(1.00, 1.00) d vs.2.00(1.75, 6.50) d, P=0.011]; comparing the time of gastrointestinal bleeding stopping when HP was applied with gammaglobulin comparison, the difference in gastrointestinal bleeding cessation time was not statistically significant ( P>0.05); (5) Comparison of hospitalization time: within 3 d application of HP compared with other window period hospitalization time were significantly reduced [(16.89±4.99) d than (19.20±2.39) d than (34.83±8.40) d, both P<0.05]; (6) Comparison of hospitalization costs: within 3 d application of HP compared with other window period hospitalization costs were significantly reduced [25 554.03 (22 168.61, 28 527.30) yuan than 33 619.48 (32 661.18, 36 971.47) yuan than 51 290.34 (34 163.04, 64 772.66) yuan, both P<0.05]; There were no statistically significant difference in the hospitalization time and hospitalization cost between and within the gammaglobulin group (all P>0.05); (7) Comparison of hormone dosages: the difference in the results of the initial dose of hormone use, pre-treatment dose of gammaglobulin/HP, and post-treatment dose of gammaglobulin/HP between the two groups of children was not statistically significant(all P>0.05). Safety profile was comparable between groups.The difference in hormone dosage before and after treatment within the gammaglobulin and HP treatment group was statistically different ( P<0.001). Conclusions:For children with severe HSP accompanied by gastrointestinal bleeding, early treatment with blood purification can rapidly relieve clinical symptoms and reduce the number of hospital days and hospitalization costs.For cases where blood purification is not available or suitable, gammaglobulin treatment is another option.

Literature Case Analysis of 44 Patients with Thrombocytopenia Induced by Clopidogrel / 医药导报

Objective To investigate the characteristics of clopidogrel-associated thrombocytopenia to provide references for clinically safe drug use.Methods The case reports of thrombocytopenia induced by clopidogrel published in PubMed,Embase,CNKI,Wanfang and VIP were searched from the establishment of each database to November 2022,and their occurrence was collated and analysed.Results A total of 44 cases from 43 articles were identified and included in the analysis.There were 30 males(68.2％)and 14 females(31.8％).Ages ranged from 37 to 88(65.0±11.4)years,of which 30(68.2％)were ≥60 years old.Thrombocytopenia was found from 8 h to 9 months after medication,of which 29 cases(65.9％)appeared within two weeks.There were 31 cases(70.5％)with severe thrombocytopenia and 38 cases(86.4％)with complications,of which 24 cases(63.2％)with bleeding and 19 cases(50.0％)with thrombotic thrombocytopenic purpura(TTP).The platelet countof41 cases(93.2％)returned to normal after drug withdrawal and symptomatic treatment,and 3 cases(6.8％)died finally.Conclusion Clopidogrel related thrombocytopenia is mainly severe thrombocytopenia,and often accompanied by bleeding or thrombotic thrombocytopenic purpura(TTP),but the overall outcome is good.Platelet count should be regularly monitored within the first two weeks after medication.Clopidogrel should be stopped and symptomatic treatment should be given in case of any abnormality.

Púrpura fulminante aguda concomitante con proceso infeccioso pulmonar

La púrpura fulminante o purpura fulminans es un síndrome de trombosis microvascular cutánea y necrosis hemorrágica de rápida evolución. Se presenta el caso de un paciente masculino, internado por patología infecciosa y evento cardiovascular agudo, que desarrolla púrpura fulminante por déficit de proteína C, relacionado a cuadro infeccioso concomitante.

Purpura fulminans is a rapidly evolving syndrome of cutaneous microvascular thrombosis and hemorrhagic necrosis. We present the case of a male patient, hospitalized for an infectious pathology and an acute cardiovascular event, who developed purpura fulminans due to protein C deficiency, related to a concomitant infectious condition.

Acquired thrombotic thrombocytopenic purpura in a patient with plasmodium vivax malaria: A case report

ABSTRACT Acquired thrombotic thrombocytopenic purpura (TTP) is a rare life-threatening disorder characterized by microangiopathic hemolytic anemia, severe thrombocytopenia, and organ damage. We present the case of a 71-year-old man initially diagnosed with malaria-like symptoms and displaying markers of microangiopathic hemolytic anemia, severe thrombocytopenia, renal injury, and neurological impairment. Despite antimalarial treatment, acquired TTP was suspected. Plasma exchange and immunosuppressive therapy led to clinical improvement, normalizing the platelet count and hemolytic profile. Diagnostic confirmation revealed significantly reduced ADAMTS13 levels. Following the proposed treatment, the patient's ADAMTS13 levels normalized. This case illustrates acquired TTP linked to uncomplicated Plasmodium vivax malaria.

Efecto de las vacunas inactivada y de ARNm contra la COVID-19 sobre la trombocitopenia en pacientes con trombocitopenia inmunitaria / Effect of inactivated and mRNA COVID-19 vaccines on thrombocytopenia in immune thrombocytopenia patients

Este estudio observacional retrospectivo tuvo como objetivo abordar los posibles efectos de las vacunas inactivada y de ARNm en pacientes con trombocitopenia inmunitaria relacionados con la exacerbación. Para definir exacerbación, se consideró una disminución de más del 30 por ciento en el recuento de plaquetas con respecto al valor basal o un recuento de plaquetas disminuido a menos de 30×109/L o el desarrollo de una nueva hemorragia. Cincuenta y nueve (hombres 30,5 por ciento, mujeres 69,5 por ciento) de 208 pacientes con trombocitopenia inmunitaria, se inscribieron en el estudio. La mediana de edad fue de 47 años (rango 18-86). Se realizó un total de 171 vacunaciones en 59 pacientes. El 38 por ciento y el 62 por ciento de los pacientes fueron vacunados con Sinovac® y BioNTech®, respectivamente. En total, 10 (16,9 por ciento) pacientes experimentaron una disminución del recuento de plaquetas por debajo de 30×109/L tras la vacunación. Durante el último año antes de la pandemia, 19 de la misma cohorte (32,2 por ciento) experimentaron dicha disminución. Después de la primera, segunda y la dosis de refuerzo de la vacunación, el 12,7 por ciento, 13,8 por ciento y 15 por ciento de los pacientes experimentaron exacerbaciones, respectivamente; las exacerbaciones con hemorragias leves fueron del 2,3 por ciento y todos los episodios hemorrágicos se trataron con éxito comenzando con esteroides o aumentando la dosis de esteroides. No se registró ninguna hemorragia grave o potencialmente mortal. Se documentó una diferencia estadística en la exacerbación en los pacientes vacunados con la vacuna de ARNm (p =0,041) sólo después de la primera dosis y los pacientes más jóvenes experimentaron una mayor tasa de exacerbación sin significación estadística (p=0,06) después de la primera dosis. En conclusión, tanto la vacuna de ARNm como la inactivada parecen ser seguras para los pacientes con trombocitopenia inmunitaria con complicaciones hemorrágicas poco frecuentes. Especialmente los pacientes más jóvenes y los vacunados con vacunas de ARNm deben ser objeto de un seguimiento estrecho durante 1-2 meses después de la vacunación para detectar trombocitopenia(AU)

This retrospective observational study was aimed to address the possible effects of inactivated and mRNA vaccines in immune thrombocytopenia patients related to exacerbation. To define exacerbation, more than 30percent decrease in platelet counts from baseline or platelet counts decreased to less than 30×109/L and/or development of new bleeding were considered. Fifty-nine (male 30.5percent, female 69.5percent) out of 208 immune thrombocytopenia patients, were enrolled in the study. The median age was 47 (range18-86). A total of 171 vaccinations were performed in 59 patients. Thirty-eight and 62percent of patients were vaccinated with Sinovac® and BioNTech®, respectively. Overall, 10 (16.9percent) patients experienced decrease in platelet count below 30×109/L after vaccination. During the last year before pandemic, 19 of the same cohort (32.2percent) experienced such decrease. After first, second and booster dose vaccinations, 12.7percent, 13.8percent and 15percent of patients experienced exacerbation respectively; exacerbation with minor bleeding was 2.3percent and all bleeding episodes were successfully treated by starting with steroid or increasing the steroid dose. We did not report any severe and life-threatening bleeding. A statistical difference in exacerbation was documented in patients vaccinated with mRNA vaccine (p =0.041) only after the first dose and younger patients experienced a higher rate of exacerbation without statistical significance (p=0.06) after the first dose. In conclusion, both mRNA and inactivated vaccines seem to be safe for immune thrombocytopenia patients with rare bleeding complications. Especially younger patients and those vaccinated with mRNA vaccines should be followed up closely for 1-2 months post vaccination for thrombocytopenia(AU)

Vasculitis por IgA del adulto. Caso clínico

La vasculitis por IgA, es la vasculitis más frecuente en pediatría. Puede presentarse en adultos, con una clínica y evolución diferente y un pronóstico más grave que en los niños, incluida la progresión a enfermedad renal terminal. La historia natural de la enfermedad y de la nefritis, ha sido poco estudiada en adultos; no se dispone de criterios diagnósticos universalmente aceptados y el tratamiento es controvertido, dada la ausencia de estudios controlados, randomizados que lo avalen. Se reporta el caso de un paciente que presentó un síndrome purpúrico petequial, microhematuria, proteinuria y una evolución rápida a la insuficiencia renal, de cuyo estudio etiológico surge el diagnóstico de vasculitis por IgA del adulto.

The IgA vasculitis is the most common vasculitis in Pediatrics. It can also present in adults but with a different clinical course and a worse prognosis, including the possibility of progression to end stage renal disease. The natural history of the disease and its nephritis have been scarcely studied in adults. There is no universal agreement in diagnostic criteria and the treatment is controversial given the absence of controlled randomized trials. We report the case of a patient who presented clinically with a petechial purpuric rash, microhematuria, proteinuria and rapid progression to renal failure that was diagnosed with IgA vasculitis in adult.

A vasculite por IgA é a vasculite mais comum em pediatria. Pode ocorrer em adultos, com apresentação e evolução clínica diferentes e prognóstico mais grave do que em crianças, incluindo progressão para doença renal terminal. A história natural da doença e da nefrite tem sido pouco estudada em adultos; Não existem critérios diagnósticos universalmente aceitos e o tratamento é controverso, dada a ausência de estudos controlados e randomizados que o apoiem. É relatado o caso de um paciente que apresentou síndrome purpúrica petequial, microhematúria, proteinúria e rápida evolução para insuficiência renal, de cujo estudo etiológico surge o diagnóstico de vasculite por IgA do adulto.

A case of concomitant connective tissue disorder and thrombotic thrombocytopenic purpura in an Indian middle aged female

Thrombotic thrombocytopenia (TTP) is a rare disease which is rarely present in adults. Adults usually have an acquired version of disease, associated with some underlying autoimmune disease. There has been paucity of literature about reports which shows the coexistence of connective tissue disorder in patients of acquired TTP. This is a case report of a female who presented with vague symptoms of breathlessness, abdominal pain and petechial rashes and was diagnosed as TTP, developed neurological complications but was stabilized by timely management through plasma exchanges and steroids.

Clinical, laboratory and treatment characteristics of patients with thrombotic thrombocytopenic purpura / Características clínicas, de laboratorio y tratamiento en pacientes con púrpura trombocitopénica trombótica

Abstract Introduction: thrombotic thrombocytopenic purpura (TTP) is a rare disease characterized by non-immune hemolytic anemia, thrombocytopenia and thrombotic microangiopathy. This study describes the clinical, laboratory and treatment characteristics of a series of patients with TTP, comparing them according to the presence or absence of associated illnesses. Materials and methods: a descriptive observational study of patients diagnosed with thrombotic thrombocytopenic purpura at a reference center in Medellín, Colombia, evaluated between 2012 and 2021. Results: a total of 19 patients were collected, with 80% female predominance; the most frequent clinical manifestations were neurological symptoms (73.6%), kidney problems (68.4%), gastrointestinal problems (52.6%) and fever (47.3%). It was associated with systemic lupus erythematosus (SLE) in 47.6% and was idiopathic in 31.5%. The mean hemoglobin was 7.7 gr/dL +/- 1.7, the median platelet count was 12 x 109 /L (8-29), and the mean lactate dehydrogenase (LDH) was 1,509 IU/L +/- 862. Altogether, 94.7% were classified as high probability according to the PLASMIC score, ADAMTS13 was measured in 42% and all received plasma exchange therapy. Clinical response was achieved in 78.9%, with refractoriness in 31.5% and 26.3% mortality; the comparison between idiopathic vs. non-idiopathic TTP showed lower kidney involvement (p=0.04) and higher LDH (p=0.02). Conclusion: the clinical presentation of TTP is notable for the predominance of neurological and gastrointestinal symptoms, marked elevation of lactate dehydrogenase and kidney injury, especially in the idiopathic type. We emphasize the need to measure ADAMTS13 activity in all patients prior to beginning plasma exchange or even in the first two sessions and look for SLE-like autoimmune disease. The higher mortality and refractoriness compared with other series presents the potential for improvement in timely diagnosis and availability of all the treatment schemes. (Acta Med Colomb 2022; 48. DOI:https://doi.org/10.36104/amc.2023.2760

Resumen Introducción: la púrpura trombocitopénica trombótica (PTT) es una enfermedad infrecuente, que se caracteriza por anemia hemolítica no inmune, trombocitopenia y microangiopatía trombótica. En este estudio se describen las características clínicas, de laboratorio y el tratamiento de una serie de pacientes con PTT comparando según la presencia o ausencia de enfermedad asociada. Material y métodos: estudio observacional descriptivo de pacientes con diagnóstico de púrpura trombocitopénica trombótica en un centro de referencia en Medellín (Colombia), evaluados entre 2012 y 2021. Resultados: se recolectaron 19 pacientes, con predominio de mujeres en 80%; las manifestaciones clínicas más frecuentes fueron síntomas neurológicos (73.6%), afectación renal (68.4%), gastrointestinales (52.6%) y fiebre (47.3%), se asoció a lupus eritematoso sistêmico (LES) en 47.6% e idiopático en 31.5%. La media de hemoglobina fue de 7.7 gr/dL ± 1.7, la mediana del recuento de plaquetas de 12 x 109 /L (8-29) y una media de lactato deshidrogenasa (LDH) de 1509 UI/L ± 862. Fueron clasificados como alta probabilidad por escala PLASMIC el 94.7%, se midió ADAMTS13 en 42% y todos recibieron terapia con recambio plasmático. La respuesta clínica se logró en 78.9%, con refractariedad en 31.5% y mortalidad 26.3%; en la comparación de PTT idiopática vs no idiopática se documentó una menor frecuencia de afectación renal (p=0.04) y mayor elevación de LDH (p=0.02). Conclusión: en la presentación clínica de PTT se destaca el predominio de los síntomas neu rológicos y gastrointestinales, la elevación marcada de lactato deshidrogenasa y la lesión renal en especial en el origen idiopático. Se recalca la necesidad de medir en todos los pacientes la actividad de ADAMTS13, previo al inicio de recambio plasmático o incluso en las primeras dos sesiones y buscar enfermedad autoinmune tipo LES. La mayor mortalidad y refractariedad comparada con otras series plantea la posibilidad de mejoras en el diagnóstico oportuno y la disponibilidad de todos los esquemas terapéuticos. (Acta Med Colomb 2022; 48. DOI:https://doi.org/10.36104/amc.2023.2760

Purpura fulminans-a rare complication post COVID-19 infection in children

Coronavirus disease 2019 (COVID-19) was officially declared as a pandemic in March 2020. COVID-19 infection and the post infectious sequalae has been widely researched since then. Post infectious inflammatory sequalae and its associated complications like toxic shock syndrome, Kawasaki like disease, macrophage activation syndrome, rather than the infection per se was found to be more of a concern in children, leading to more morbidity and mortality. Purpura fulminans was not a presentation reported post COVID-19 infection. Herein, we aim to describe a post COVID-19 infection patient who presented with purpura fulminans as a rare complication.

Síndrome de la bolsa de orina púrpura

Introducción: El síndrome de la bolsa orina púrpura es una condición llamativa, que rara vez se presenta en la práctica clínica. Aparece en el contexto de infecciones urinarias en ancianos, pluripatológicos, con sonda vesical y factores de riesgo asociados. Se produce por una reacción química entre la orina, el material plástico de la bolsa colectora y enzimas sulfatasas/fosfatasas de bacterias que generan el color violáceo característico. Objetivo: Reportar un caso con síndrome de la bolsa orina púrpura, como forma de presentación inusual de infección urinaria. Caso Clínico: Paciente femenina, de 76 años de edad, con antecedentes de constipación habitual, acudió a urgencias por pérdida del conocimiento y hemiparesia derecha. Se realizó tomografía axial computarizada de cráneo y se diagnosticó una enfermedad cerebrovascular. Como parte de la conducta se indicó sonda vesical y 14 días después apareció orina de color violeta en la bolsa colectora. Se diagnosticó infección urinaria por Escherichia coli y se trató con ceftriaxona. Se normalizó el color de la orina al tercer día de tratamiento, la paciente evolucionó de forma favorable. Conclusiones: Aunque se puede identificar con facilidad, sin requerir exámenes costosos, constituye un reto para los médicos que atienden a pacientes geriátricos. Conocer este trastorno es fundamental, porque, aunque es infrecuente, puede ser la única manifestación de infección urinaria en pacientes con cateterismo uretral.

Introduction: Purple urine bag syndrome is a striking condition that rarely occurs in clinical practice. It appears in the context of urinary infections in the elderly, with multiple pathologies, with a bladder catheter and associated risk factors. It is produced by a chemical reaction between urine, the plastic material of the collection bag and sulfatase/phosphatase enzymes from bacteria that generate the characteristic purple color. Objective: To report a case with purple urine bag syndrome as an unusual presentation of urinary tract infection. Clinical Case: Female patient, 76 years old, health history, usual constipation, attended at Emergency due to loss of consciousness and right hemiparesis. Computed axial tomography of the skull was performed and a cerebrovascular disease was diagnosed. As part of the conduct, a bladder catheter was indicated and after 14 days, purple urine appeared in the collection bag. Urinary infection due to Escherichia coli was diagnosed and treated with ceftriaxone, normalizing the color of the urine on the third day of treatment, with patient favorable evolution. Conclusions: Although it can be easily identified and without requiring costly tests, it is a challenge for physicians who care for geriatric patients. Knowing this disorder is essential because, although rare, it may be the only manifestation of urinary infection in patients with urethral catheterization.

Neonatal purpura fulminans: a rare disease with a sinister outcome

Neonatal purpura fulminans is a rare but frequently fatal disorder associated with high morbidity and mortality. Purpura fulminans describes a clinico-pathological entity of dermal microvascular thrombosis associated with disseminated intravascular coagulation (DIC) and perivascular hemorrhage occurring in the newborn period. It is usually congenital as a result of deficiency of protein C and S. it maybe be either idiopathic or acquired due to severe infection by gram negative organisms or Staphylococcus species. Here we present the case of a neonate born to an HIV reactive mother who presented to us at 6th day of life with desquamation of skin in periumbilical area which gradually progressed to involve the limbs, trunk as well as face. The neonate was managed with supportive therapy in ICU but despite intensive management, she expired at 10 days of life due to septicaemia and multi-organ failure.

Thrombocytopenia in pregnancy

Thrombocytopenia is defined as a platelet count of <150x109/l. It is the second most common hematological abnormality during pregnancy. We present a case series of thrombocytopenia in pregnancy. The aim of this study was to evaluate thrombocytopenia in pregnancy at tertiary care center, Bhopal and to identify, treat and assess the maternal complications and neonatal outcome. In a year, 10 cases were evaluated out of which the diagnosis of 3 cases was immune thrombocytopenic purpura, 3 cases of gestational thrombocytopenia, 3 cases of thrombocytopenia associated with hypertensive disorders of pregnancy and 1 case of HBV induced chronic liver disease associated with thrombocytopenia.

Safety and efficacy of romiplostim in children and adolescents with Immune thrombocytopenia: A systematic review

Abstract Objective To evaluate the efficacy and safety of romiplostim (thrombopoietin-receptor agonist) in the treatment of pediatric immune thrombocytopenia (ITP). Methods Searches were conducted in MEDLINE, EMBASE, LILACS, Cochrane Central Register of Controlled Trials and ClinicalTrials.gov (from January 2011 to August 2021). Randomized controlled trials (RCTs), double-blind, comparing romiplostim with a placebo in pediatric persistent or chronic ITP were included. The primary outcome was the overall response rate (platelets ≥ 50 × 109/L) in the absence of rescue therapy for at least two consecutive weeks. The secondary endpoints were the minimization of clinically significant bleeding and the necessity for rescue treatments and the maximization of safety (incidence of overall adverse events) and durable response (maintaining platelet counts for at least twelve weeks). Results Two double-blind randomized placebo-controlled trials (84 participants) were included in this systematic review. Our data showed that, compared to the placebo group, the proportion of patients achieving durable platelet response was significantly higher in the romiplostim group (p= 0.003, RR = 6.34, 95%CI = 1.89 - 21.23), as was the overall response in the romiplostim group (p= 0.002, RR = 3.62, 95%CI = 1.63 - 8.03). Significant bleeding incidents (p= 0.49), overall adverse events (p= 0.71) and the need for rescue treatment (p= 0.13) were not statistically different between the romiplostim and placebo groups. Conclusions Romiplostim might improve both durable and overall platelet response in children and adolescents with ITP, compared to a placebo. More clinical trials are needed to evaluate the efficacy and safety of romiplostim and to compare it with other second-line treatments that are being used in pediatric ITP.

Schõnlein-Henoch purpura in adults: Report of four cases

Schoenlein-Henoch purpura is a systemic small vessel vasculitis mediated by IgA-1 deposition in organs such as the skin, kidney, and gastrointestinal tract; it has been mainly described in children where it has a favourable prognosis. Although much rarer in adulthood it is associated with an increased risk of severe kidney involvement, gastrointestinal com-plications, and prolonged hospital stay. The therapeutic options are wide and vary according to the degree of involvement of the patient and the organ mainly affected.

La púrpura de Schönlein-Henoch es una vasculitis sistêmica de pequeno vaso mediada por depósito de IgA en órganos como la piel, el riñón y el tracto gastrointestinal. Se ha descrito principalmente en niños, grupo de población en el que tiene un pronóstico favorable. Si bien en la edad adulta es mucho menos frecuente, se asocia con un mayor riesgo de compromiso renal severo, complicaciones gastrointestinales y estancia hospitalaria prolongada. Las opciones terapêuticas son amplias y varían según el grado de compromiso del paciente y el órgano más afectado.

A rare presentation of common tropical fever, scrub typhus-purpura fulminans, lower limb arterial thrombosis and pulmonary artery thrombosis

Purpura fulminans (PF) is an acute emergency condition manifested as purpuric rash secondary to thrombosis of microvasculature. It is rapidly progressive, can cause thrombosis in large as well as small vessels and tissue infarction. Although it is commonly associated with Meningococcal and Streptococcal infections, here we report this case of PF associated with scrub typhus infection. Our patient presented with generalised body rash and progressed to multiorgan dysfunction. On evaluation, common causes of PF were ruled out and eventually patient came out to be IgM scrub typhus serology kit test positive. Lower limb angiography and pulmonary artery angiography revealed vascular thrombosis. The patient started on IV antibiotics, other supportive managements, anticoagulation. Later the patient improved clinically, skin rash resolved with excoriations but developed gangrenous changes in both lower limbs. Hence the uncommon presentation of scrub typhus infection as purpura fulminans needs early identification and effective treatment to achieve mortality and morbidity benefit.