RESUMO
The term Hoigné's syndrome denotes a mimicker of anaphylaxis, which occurs immediately after the parenteral administration of a drug and is likely caused by non-thrombotic pulmonary and systemic drug micro-embolization. It has so far been documented uniquely in case reports and small case series. Because this condition has never been systematically evaluated, we performed a structured literature review (pre-registered as CRD42023392962). The search was carried out in Excerpta Medica, National Library of Medicine, and Google Scholar. Cases with features consistent with anaphylaxis, urticaria, angioedema, asthma, syncope, anxiety, or panic attack triggered by needle phobia, and local anesthetic systemic toxicity were excluded. For the final analysis, we retained reports published between 1951 and 2021, which presented 247 patients with Hoigné's syndrome: 37 children and 211 adults with a male: female ratio of 2.1 : 1.0. The patients presented within 1 min after parenteral administration of a drug (intramuscular penicillin in 90 % of the cases) with chest discomfort, shortness of breath, fear of death, psychomotor agitation, and auditory or visual hallucinations and impairment. Recovery occurred within 30 min. The diagnosis of Hoigné's syndrome was also established in five patients 66-91 years of age with pre-existing cardiovascular or pulmonary diseases, who suddenly died after the administration of penicillin despite not exhibiting the aforementioned symptoms. It was therefore speculated that pulmonary drug micro-embolization induced a lethal cardiovascular compromise in these individuals. Histologic investigations supporting this hypothesis were performed in only one case. The diagnosis of Hoigné's pulmonary drug micro-embolization was established also in five patients with pre-existing cardiovascular or pulmonary diseases, who suddenly died after the administration of penicillin despite not exhibiting the afore mentioned symptoms. Histologic investigations supporting this hypothesis were performed in only one case. In conclusion, Hoigné's syndrome is an uncommon non-immune-mediated reaction. This report seeks to promote broader awareness and knowledge regarding this alarming mimicker of anaphylaxis. Diagnosis relies solely on clinical evaluation.
Assuntos
Anafilaxia , Humanos , Anafilaxia/diagnóstico , Anafilaxia/etiologia , Anafilaxia/terapia , Diagnóstico Diferencial , Masculino , Feminino , Idoso , Adulto , Idoso de 80 Anos ou mais , Síndrome , Criança , Penicilinas/efeitos adversos , Hipersensibilidade a Drogas/diagnóstico , Hipersensibilidade a Drogas/terapiaRESUMO
Finkelstein-Seidlmayer vasculitis, also called acute hemorrhagic edema of young children or infantile immunoglobulin A vasculitis, is habitually a benign skin-limited small vessel leukocytoclastic vasculitis that mainly affects infants 24 months or less of age. Since this disease is commonly triggered by an infection, an immune-mediated origin has been postulated. To better appreciate the possible underlying immune mechanism of this vasculitis, we addressed circulating autoimmune markers and vascular immune deposits in patients contained in the Acute Hemorrhagic Edema BIbliographic Database, which incorporates all original reports on Finkelstein-Seidlmayer vasculitis. A test for at least one circulating autoimmune marker or a vascular immune deposit was performed in 243 cases. Subunits of complement system C4 resulted pathologically reduced in 4.7% and C3 in 1.4%, rheumatoid factor was detected in 6.1%, and antinuclear antibodies in 1.9% of cases. Antineutrophil cytoplasmic antibodies were never demonstrated. Immunofluorescence studies were performed on 125 skin biopsy specimens and resulted positive for complement subunits in 46%, fibrinogen in 45%, immunoglobulin A in 25%, immunoglobulin M in 24%, immunoglobulin G in 13%, and immunoglobulin E in 4.2% of cases. Infants testing positive for vascular immunoglobulin A deposits did not present a higher prevalence of systemic involvement or recurrences, nor a longer disease duration. In conclusion, we detected a very low prevalence of circulating autoimmune marker positivity in Finkelstein-Seidlmayer patients. Available immunofluorescence data support the notion that immune factors play a relevant role in this vasculitis. Furthermore, vascular immunoglobulin A deposits seem not to play a crucial role in this disease.
Assuntos
Vasculite Leucocitoclástica Cutânea , Vasculite , Criança , Lactente , Humanos , Pré-Escolar , Vasculite/diagnóstico , Vasculite Leucocitoclástica Cutânea/diagnóstico , Imunoglobulina A , Imunoglobulina G , Hemorragia , EdemaRESUMO
OBJECTIVE: To investigate treatment modalities for children with extremity indwelling catheter (EIC)- or cardiac catheter-related arterial thrombosis. STUDY DESIGN: The treatment of consecutive cases of catheter-related arterial thrombosis (CAT) at our institution between 2002 and 2017 was analyzed retrospectively. RESULTS: A total of 242 CATs developed in 224 children. Of these, 125 (52%) were EIC-related and 117 (48%) were cardiac catheter-related. Treatment included heparin alone in 60 cases (25%), acetylsalicyclic acid (ASA) alone in 6 cases (2%), heparin followed by ASA in 171 cases (71%), heparin followed by vitamin K antagonist (VKA) in 4 cases (1.5%), and VKA alone in 1 case (0.5%). Complete resolution of CAT was observed in 173 cases (71.5%), partial resolution in 13 cases (5.4%), and no resolution in 56 cases (23.1%). No statistical significance in the resolution rate was observed between treatment groups (P = .23). In 66% of cases, complete resolution occurred at a median of 18 days (range, 4-44 days) with heparin alone. A switch from heparin to ASA in children with partial or no resolution of CAT did not increase the resolution rate at follow-up. CONCLUSIONS: Heparin is an efficient treatment modality for CAT in pediatric patients. Long-term, subsequent treatment with ASA does not increase the resolution rate.
Assuntos
Anticoagulantes/uso terapêutico , Cateterismo Cardíaco/efeitos adversos , Cateteres de Demora/efeitos adversos , Artéria Femoral , Fibrinolíticos/uso terapêutico , Artéria Ilíaca , Trombose/tratamento farmacológico , Adolescente , Aspirina/uso terapêutico , Criança , Pré-Escolar , Esquema de Medicação , Quimioterapia Combinada , Feminino , Seguimentos , Heparina/uso terapêutico , Humanos , Lactente , Recém-Nascido , Masculino , Estudos Retrospectivos , Trombose/etiologia , Resultado do Tratamento , Vitamina K/antagonistas & inibidoresRESUMO
Four individuals from two families presented with a multisystemic condition of suspected genetic origin that was diagnosed only after genome analysis. The main phenotypic features were immune system dysregulation (severe immunodeficiency with autoimmunity) and intellectual disability. The four individuals were found to be homozygous for a 4.4 Kb deletion removing exons 20-23 (NM_003291.4) of the TPP2 gene, predicting a frameshift with premature termination of the protein. The deletion was located on a shared chromosome 13 haplotype indicating a Swiss founder mutation. Tripeptidyl peptidase 2 (TPP2) is a protease involved in HLA/antigen complex processing and amino acid homeostasis. Biallelic variants in TPP2 have been described in 10 individuals with variable features including immune deficiency, autoimmune cytopenias, and intellectual disability or chronic sterile brain inflammation mimicking multiple sclerosis. Our observations further delineate this severe condition not yet included in the OMIM catalog. Timely recognition of TPP2 deficiency is crucial since (1) immune surveillance is needed and hematopoietic stem cell transplantation may be necessary, and (2) for provision of genetic counselling. Additionally, enzyme replacement therapy, as already established for TPP1 deficiency, might be an option in the future.
Assuntos
Aminopeptidases/genética , Doenças Autoimunes/genética , Dipeptidil Peptidases e Tripeptidil Peptidases/genética , Mutação da Fase de Leitura/genética , Síndromes de Imunodeficiência/genética , Serina Endopeptidases/genética , Adulto , Criança , Pré-Escolar , Éxons/genética , Feminino , Humanos , Masculino , Adulto JovemRESUMO
Children with sickle cell disease (SCD) require specific perioperative care, and clinical practice in this area remains poorly defined. We aimed to conduct a systematic, PRISMA-based review of the literature, available clinical guidelines and practice recommendations. We also aimed to extract any valuable information for the "best of available-evidence"-based prevention of perioperative adverse events in children with SCD, and highlight the most urgent priorities in clinical research. As data sources, US National Library of Medicine, Medline, National Guideline Clearinghouse, International Guideline Network, TRIP databases were searched for any content until January 2019. We also included institutional, consortia and expert group guidelines. Included were reports/guidelines in English, French, German, and Italian. Excluded were reports on obstetrical and fetal management. We identified 202 reports/guidelines fulfilling the criteria outlined above. A majority focused on visceral, cardiovascular and orthopedic surgery procedures, and only five were multicenter randomized controlled trials and two prospective randomized studies. After grading of the quality of the evidence, the extracted data was summarized into clinical recommendations for daily practice. Additionally, we designed a risk-grading algorithm to identify contexts likely to be associated with adverse outcomes. In conclusion, we provide a systematic PRISMA-based review of the existing literature and ancillary practice and delineate a set of clinical recommendations and priorities for research.
Assuntos
Anemia Falciforme/cirurgia , Assistência Perioperatória/métodos , Guias de Prática Clínica como Assunto , Criança , Humanos , Medição de RiscoRESUMO
BACKGROUND: Spinal epidural hematoma without significant trauma is a rare condition with potentially severe outcome. This case report and systematic review of the literature illustrates the clinical presentation, risk factors, evaluation, treatment and outcomes of spinal epidural hematoma without significant trauma in children. CASE PRESENTATION: We report one case of a 7-year-old girl who developed a neck pain after minor cervical sprain. MRI showed a right posterior epidural hematoma extending from C2/3 to T1. The hematoma was surgically evacuated, and the histopathology showed an arteriovenous malformation. Postoperative MRI showed complete evacuation of the hematoma and no residual vascular malformation. We report a second ASE with idiopathic spinal epidural hematoma of a 4½-year-old boy presenting with neck pain. MRI showed a right-sided latero-posterior subacute spinal epidural hematoma at C3-C5. Owing to the absence of any neurological deficit, the patient was treated conservatively. MRI at 3 months showed complete resolution of the hematoma. CONCLUSIONS: Spinal epidural hematoma without significant trauma in children is a rare condition. It may present with unspecific symptoms. Screening for bleeding diathesis is warranted and neuroradiologic follow-up is essential to rule out vascular malformation. Whereas most children have a favorable outcome, some do not recover, and neurological follow-up is required.
Assuntos
Malformações Arteriovenosas , Hematoma Epidural Espinal , Malformações Arteriovenosas/complicações , Criança , Pré-Escolar , Feminino , Hematoma Epidural Espinal/diagnóstico por imagem , Hematoma Epidural Espinal/etiologia , Humanos , Imageamento por Ressonância Magnética , Masculino , Cervicalgia/etiologiaRESUMO
Spinal epidural hematoma (SEH) is a rare condition leading to spinal cord compression after trauma, surgery, or other. In 40% of the cases, the cause is unknown or unidentified. Due to the absence of specific symptoms, the diagnosis is often delayed. The mainstay of treatment is urgent evacuation of the hematoma. The choice of the surgical technique is surgeon-dependent and ranges from simple decompression and hematoma evacuation to variable combinations of decompression and reconstruction of the posterior spinal arch. To our knowledge, we describe the youngest case in the literature of a thoracolumbar SEH in a newborn with hemophilia A which was evacuated by spinous process splitting laminoplasty (SPSL). SPSL was chosen to avoid damaging the primary ossification centers, preserve the paravertebral musculature, and evade the sequelae of multilevel laminectomies. In our opinion, this technique should be propagated in the pediatric population for accessing the posterior and posterolateral spinal canal.
Assuntos
Hematoma Epidural Craniano , Hematoma Epidural Espinal , Laminoplastia , Criança , Descompressão Cirúrgica , Hematoma Epidural Espinal/diagnóstico por imagem , Hematoma Epidural Espinal/etiologia , Hematoma Epidural Espinal/cirurgia , Humanos , Recém-Nascido , LaminectomiaRESUMO
OBJECTIVES: To investigate the long-term outcome of catheter-related arterial thrombosis in children. STUDY DESIGN: Data from clinical and radiologic long-term follow-up of infants with congenital heart disease developing arterial thrombosis following femoral catheterization are presented. RESULTS: Ninety-five infants with radiologically proven arterial thrombosis because of cardiac catheter (n = 52; 55%) or indwelling arterial catheter (n = 43; 45%) were followed for a median time of 23.5 months (IQR 13.3-47.3). Overall, radiologic complete thrombus resolution was observed in 64 (67%), partial resolution in 8 (9%), and no resolution in 23 (24%) infants. Complete resolution was significantly more frequent in infants with indwelling arterial catheter-related thrombosis compared with cardiac catheter-related thrombosis (P = .001). Patients with complete resolution had a significantly lower blood pressure difference and increased ankle-ankle index compared with patients with partial or no resolution (P < .0001). However, symptoms of claudication were present only in 1 case and clinical significant legs growth retardation (≥ 15 mm) was present in 1%. CONCLUSIONS: A significant percentage of persistent occlusion is present in children with arterial catheter-related thrombosis on long-term follow-up. In these children, the magnitude of leg growth retardation is small and possibly not clinically relevant. However, in children with congenital heart disease, the high prevalence of persistent arterial occlusion may hamper future diagnostic and/or interventional catheterization.
Assuntos
Cateterismo Cardíaco/efeitos adversos , Cateteres de Demora/efeitos adversos , Artéria Femoral/diagnóstico por imagem , Cardiopatias Congênitas , Artéria Ilíaca/diagnóstico por imagem , Trombose/etiologia , Índice Tornozelo-Braço , Arteriopatias Oclusivas/diagnóstico por imagem , Arteriopatias Oclusivas/etiologia , Pressão Sanguínea , Feminino , Seguimentos , Humanos , Lactente , Recém-Nascido , Masculino , Radiografia , Recuperação de Função Fisiológica , Trombose/diagnóstico por imagemRESUMO
Non-covalent (NP-1/3) and covalent (NP-A-1/3) pro-apoptotic SPION-Smac mimetic nano-conjugates antitumor agents are reported. The solution synthesis of key Smac mimetics, their support onto SPIONs through non-covalent adsorption (NP-1/3) or APTES-mediated covalent binding (NP-A-1/3), the analytical characterization of SPION-Smac mimetic conjugates, their target affinity in cell-free assays, and their cytotoxicity against tumor cells are thoroughly described.
Assuntos
Antineoplásicos/química , Antineoplásicos/farmacologia , Dextranos/química , Dextranos/farmacologia , Peptídeos e Proteínas de Sinalização Intracelular/química , Nanopartículas de Magnetita/química , Proteínas Mitocondriais/química , Apoptose/efeitos dos fármacos , Proteínas Reguladoras de Apoptose , Materiais Biomiméticos/química , Materiais Biomiméticos/farmacologia , Linhagem Celular Tumoral , Células HeLa , Humanos , Nanopartículas/administração & dosagem , Nanopartículas/química , Neoplasias/tratamento farmacológico , Neoplasias/patologia , Ligação ProteicaRESUMO
BACKGROUND: Little information is available on ureteral or vesical involvement in Henoch-Schönlein syndrome. To determine the features of this condition we performed a formal analysis of peer-reviewed scientific literature on this topic. METHODS: The US National Library of Medicine database was used as the data source. All articles published as full-length articles or letters were collected. Reports published in languages other than English, French, German, Italian or Spanish were not considered. RESULTS: We analyzed 32 reports describing 35 cases (24 male and 11 female subjects aged between 3.5 and 63, median 7.0 years) with ureteral (n = 30), vesical (n = 4), or both ureteral and vesical involvement (n = 1). The presentation included colicky abdominal pain, macroscopic hematuria (sometimes containing blood clots), urinary tract infection or urinary retention. The diagnosis of ureteral involvement was often fortuitous. Patients with vesical involvement were managed conservatively. However, the majority of those with ureteral involvement were managed surgically. CONCLUSIONS: Ureteral or vesical involvement is unusual and likely underappreciated in Henoch-Schönlein syndrome. Improved recognition and wider appreciation of this involvement can help to avoid associated morbidity. Management must be individualized for each patient. A multidisciplinary approach may be of value in planning medical treatment, surgical intervention, and follow-up.
Assuntos
Vasculite por IgA/complicações , Doenças Ureterais/etiologia , Doenças da Bexiga Urinária/etiologia , Adolescente , Adulto , Criança , Pré-Escolar , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome , Doenças Ureterais/epidemiologia , Doenças da Bexiga Urinária/epidemiologia , Adulto JovemRESUMO
In response to growing recognition that nonadherence prevents children, adolescents, and young adults from achieving the therapeutic benefits of anticoagulant medication, the International Society on Thrombosis and Haemostasis Scientific and Standardization Committee Subcommittee on Pediatric and Neonatal Thrombosis and Hemostasis convened a working party on medication adherence. The primary aim of this article was to synthesize recommendations from the larger adherence science literature to provide guidance regarding the classification, collection, and interpretation of anticoagulation adherence data. The secondary aim of this article was to evaluate the degree to which trials published from 2013 to 2023 adhered to these guidance recommendations. As less than half of all trials reported on adherence and none included all recommended elements, the proposed International Society on Thrombosis and Haemostasis Scientific and Standardization Committee guidance has the potential to enhance the rigor and reproducibility of pediatric anticoagulant research.
Assuntos
Anticoagulantes , Ensaios Clínicos como Assunto , Hemostasia , Adesão à Medicação , Trombose , Humanos , Anticoagulantes/uso terapêutico , Anticoagulantes/efeitos adversos , Criança , Trombose/prevenção & controle , Trombose/tratamento farmacológico , Trombose/etiologia , Adolescente , Recém-Nascido , Pré-Escolar , Hemostasia/efeitos dos fármacos , Lactente , Projetos de Pesquisa/normas , Fatores Etários , Fidelidade a DiretrizesRESUMO
BACKGROUND: Pediatric patients with acute lymphoblastic leukemia (ALL) are at highest risk of venous thromboembolism during the induction therapy (IT). These events are not predictable by conventional coagulation assays. OBJECTIVES: To investigate the utility of global coagulation assays (GCAs) for assessing the hemostatic state in children with ALL during IT. METHODS: We included children with ALL (n = 15) and healthy controls (n = 15). Analyses were performed at different time points during IT of the AIEOP-BFM protocols. In addition to prothrombotic biomarkers, natural anticoagulant proteins, and in vivo thrombin generation (TG) markers, ex vivo TG was measured using the gold standard calibrated automated thrombogram method, automated ST Genesia, and thrombodynamics analyzer (TD). The latter also provided measurement of fibrin clot formation. RESULTS: Different from conventional coagulation assays and in vivo TG markers, ex vivo GCAs detected increasing prothrombotic changes during IT. Particularly, TG measured with TD as expressed by endogenous thrombin potential was already significantly elevated at days 8 to 12 (P < .01) and continued to increase during IT compared with prior to beginning treatment, indicating a very early shift toward a procoagulant state. A similar pattern was observed for the rate of fibrin clot formation (stationary rate of clot growth: P < .01 at days 8-12). Remarkably, in patients developing thrombotic complications (n = 5), both GCAs, ST Genesia and TD, showed a significantly higher endogenous thrombin potential very early (already at days 8-12, P < .05), well before clinical manifestation. CONCLUSION: GCAs capture prothrombotic changes early during IT in ALL pediatric patients. If confirmed, this approach will allow tailoring thromboprophylaxis in children with ALL at highest risk for venous thromboembolism.
Assuntos
Coagulação Sanguínea , Leucemia-Linfoma Linfoblástico de Células Precursoras , Trombina , Tromboembolia Venosa , Humanos , Criança , Leucemia-Linfoma Linfoblástico de Células Precursoras/sangue , Leucemia-Linfoma Linfoblástico de Células Precursoras/complicações , Testes de Coagulação Sanguínea , Feminino , Masculino , Trombina/metabolismo , Pré-Escolar , Estudos de Casos e Controles , Adolescente , Tromboembolia Venosa/sangue , Tromboembolia Venosa/etiologia , Tromboembolia Venosa/diagnóstico , Fatores de Tempo , Valor Preditivo dos Testes , Fatores de Risco , Biomarcadores/sangue , Trombose/sangue , Trombose/etiologia , Trombose/diagnósticoRESUMO
OBJECTIVE: To investigate Port-A-Cath (PAC)-related thrombosis and postthrombotic syndrome (PTS) in children with cancer. STUDY DESIGN: The study population was a consecutive cohort of children diagnosed with cancer and a PAC implanted at diagnosis. Children were evaluated for the presence of PAC-related thrombosis by magnetic resonance venography and the presence of congenital prothrombotic risk factors and PTS. RESULTS: A total of 114 children (median age, 6.04 years) were included. Of these children, 48 (42%) were treated for solid tumors and 66 (58%) were treated for hematopoietic tumors, including 38 for acute lymphoblastic leukemia. At the time of magnetic resonance venography, 42 children (37%) had the PAC still in place, and 72 (63%) had the PAC removed. Overall, PACs were in place for a total of 324.92 PAC-years. PAC-related thrombosis was detected in 45 children (39.5%) with a current or previous PAC. Of these, 21 (47%) had a solid tumor, 14 (31%) had acute lymphoblastic leukemia, and 10 (22%) had another hematopoietic tumor. Younger age at diagnosis, female sex, duration of PAC use, and left-side PAC placement were independently associated with an increased risk of thrombosis, whereas asparaginase therapy and the presence of inherited prothrombotic risk factors were not. Mild PTS (ie, presence of prominent collateral vessels in the skin) was present in 5.6% of the children. CONCLUSION: PAC-related thrombosis is common in pediatric oncology patients. In some children, thrombotic complications can lead to the development of PTS.
Assuntos
Síndrome Pós-Trombótica/diagnóstico , Trombose/diagnóstico , Dispositivos de Acesso Vascular/efeitos adversos , Adolescente , Fatores Etários , Criança , Pré-Escolar , Estudos de Coortes , Feminino , Humanos , Lactente , Imageamento por Ressonância Magnética , Masculino , Neoplasias/complicações , Neoplasias/terapia , Flebografia , Síndrome Pós-Trombótica/etiologia , Fatores de Risco , Trombose/etiologiaRESUMO
BACKGROUND: The ISTH Scientific and Standardization Committee (SSC) Subcommittee on Pediatric/Neonatal Thrombosis and Hemostasis convened a working group on medication adherence to begin to understand the current state of clinical practice to inform priority areas for efforts to improve adherence for children, and adolescents and young adults (AYA) prescribed anticoagulants. OBJECTIVES: We sought to survey an international group of clinicians involved in anticoagulation management in children and/or AYA about perceptions of medication on health outcomes, clinical practice related to medication adherence, and barriers to assessing and improving medication adherence. METHODS: Clinicians involved in anticoagulation management in children and/or AYA were surveyed via REDCap® . Descriptive statistics were used to summarize demographic and clinical characteristics and responses to multiple choice and Likert-type questions. Free-text answers were coded based on the Behaviour Change Technique Taxonomy and the Expert Recommendations for Implement Change project. RESULTS AND CONCLUSIONS: There were 200 participants, 90% of whom were pediatric hematology/oncology physicians. Based on the results, which demonstrate that clinicians are concerned about impact of poor medication adherence and have limited resources to identify and improve adherence, the working group has identified next steps to further understand impact of medication adherence on anticoagulation-related health outcomes, address the need for validated measures to assess medication adherence for all anticoagulants prescribed to this population, and develop an intervention and implementation research agenda to improve outcomes.
Assuntos
Hemostasia , Trombose , Adolescente , Anticoagulantes/efeitos adversos , Criança , Comunicação , Humanos , Recém-Nascido , Adesão à Medicação , Padrões de Referência , Trombose/tratamento farmacológico , Trombose/prevenção & controle , Adulto JovemRESUMO
Clinically unsuspected venous thromboembolism (VTE) in children is defined as a VTE diagnosed via imaging test performed for surveillance (i.e., with an intent to identify clinically silent VTEs) or incidentally found (most often via imaging performed for evaluation of regional pathology unrelated to VTE) in the absence of any VTE-associated signs or symptoms. Our understanding of the clinical significance of these events in children is limited by a paucity of data on the epidemiology and outcomes of this complication. There is an urgent need for further research in this area to inform optimal management. Recognizing this knowledge gap, this Task Force has previously published a systematic review of the literature in this topic. We now provide guidance recommendations for standardization of definitions and identify future research needs on clinically unsuspected VTE in children. These recommendations will serve to enhance the quantity and quality of evidence on the topic and facilitate the design and execution of cooperative observational studies, and interventional trials of risk-stratified management approaches aimed at preventing and optimizing long-term outcomes of clinically unsuspected VTE in children.
Assuntos
Trombose , Tromboembolia Venosa , Anticoagulantes/uso terapêutico , Criança , Comunicação , Hemostasia , Humanos , Recém-Nascido , Trombose/complicações , Tromboembolia Venosa/diagnóstico , Tromboembolia Venosa/epidemiologia , Tromboembolia Venosa/etiologiaRESUMO
Caring for children and adolescents with disorders of hemostasis and thrombosis (HAT) has become more specialized and requires a unique skill set that many providers are not able to obtain in standard pediatric hematology/oncology/bone marrow transplant fellowship training programs. The influx of numerous therapeutic advances and increasing medical complexity has expanded the need for experienced HAT providers and subspecialty collaboration in the inpatient setting due to the nuances in the management of patients with HAT complications and concerns. While there are data highlighting the benefits of an inpatient hemostasis, thrombosis, and anticoagulation management service in adult hospitals, there are limited pediatric data supporting such programs. In this article, we summarize the current practices of various pediatric institutions in the inpatient management of HAT patients and provide a consensus opinion for the development of a pediatric inpatient HAT service at tertiary care referral centers.
Assuntos
Pacientes Internados , Trombose , Adolescente , Adulto , Criança , Comunicação , Consenso , Hemostasia , Hospitais Pediátricos , Humanos , Recém-Nascido , Encaminhamento e Consulta , Trombose/diagnóstico , Trombose/terapiaRESUMO
Acute hemorrhagic edema of young children is an uncommon but likely underestimated cutaneous leukocytoclastic vasculitis. The condition typically affects infants 6-24 months of age with a history of recent respiratory illness with or without course of antibiotics. The diagnosis is made in children, mostly nontoxic in appearance, presenting with nonpruritic, large, round, red to purpuric plaques predominantly over the cheeks, ears, and extremities, with relative sparing of the trunk, often with a target-like appearance, and edema of the distal extremities, ears, and face that is mostly non-pitting, indurative, and tender. In boys, the lesions sometimes involve the scrotum and, more rarely, the penis. Fever, typically of low grade, is often present. Involvement of body systems other than skin is uncommon, and spontaneous recovery usually occurs within 6-21 days without sequelae. In this condition, laboratory tests are non-contributory: total blood cell count is often normal, although leukocytosis and thrombocytosis are sometimes found, clotting studies are normal, erythrocyte sedimentation rate and C-reactive protein test are normal or slightly elevated, complement level is normal, autoantibodies are absent, and urinalysis is usually normal. Experienced physicians rapidly consider the possible diagnosis of acute hemorrhagic edema when presented with a nontoxic young child having large targetoid purpuric lesions and indurative swelling, which is non-pitting in character, and make the diagnosis either on the basis of clinical findings alone or supported by a skin biopsy study.
Assuntos
Edema/diagnóstico , Hemorragia/diagnóstico , Dermatopatias/diagnóstico , Vasculite/complicações , Doença Aguda , Pré-Escolar , Diagnóstico Diferencial , Edema/etiologia , Hemorragia/etiologia , Humanos , Lactente , Vasculite/diagnósticoRESUMO
A 2½-year-old male child and a 23-year-old woman with no clinical symptoms were investigated during routine consultations. Cation exchange high performance liquid chromatography (HPLC) revealed an additional peak eluting before Hb A. DNA sequencing showed a novel heterozygous mutation at codon 20 of the α1-globin gene. The hemoglobin (Hb) variant was named Hb Brugg. Analysis of oxygen affinity Hb and Hb stability did not show any changes compared to normal Hb constellation.