Cílem standardu je stanovit základní diagnostické a především terapeutické postupy v péči o pacienty s vrozenou hemofilií a s inhibitorem koagulačního faktoru VIII, resp. faktoru IX. Úprava původního konsenzuálního doporučení vychází z vývoje poznání a reflektuje nová data i vstup nových léčiv, jež mění léčebné přístupy.

The aim of these guidelines is to set-up and standardize diagnostic as well as therapeutic approaches to patients suffering from congenital haemophilia and factor VIII or factor IX inhibitors. Update of the original consensual guidelines is based on evolution in our experience and reflects new available data as well as the advent of new drugs revolutionising our therapeutic approach.

• MeSH
• Surgical Procedures, Operative MeSH
• Factor VIII analysis   MeSH
• Hemophilia A * diagnosis   therapy   MeSH
• Blood Coagulation Factor Inhibitors blood   MeSH
• Humans MeSH
• Check Tag
• Humans MeSH
• Publication type
• Practice Guideline MeSH

Cílem standardu je stanovit základní diagnostické a především terapeutické postupy v péči o pacienty s hemofilií na základě aktualizovaných vědeckých poznatků. Úprava původního konsenzuálního doporučení odráží nová data i příchod nových léků a doporučuje optimální dávkování pro léčbu. Stručně je popsán systém péče o hemofilii v České republice.

The aim of these guidelines is to set-up and standardize diagnostic as well as therapeutic approaches to patients with haemophilia according to updated scientific evidence. Update of the original consensual guidelines reflects new available data plus advent of new drugs and recommends the optimal dosage for treatment. The system for haemophilia care in Czech Republic is briefly described.

• Keywords
• profylaxe,
• MeSH
• Hemophilia A * diagnosis   therapy   MeSH
• Hemophilia B * diagnosis   therapy   MeSH
• Humans MeSH
• Check Tag
• Humans MeSH
• Publication type
• Practice Guideline MeSH

Hemofilie A a B jsou vzácná vrozená krvácivá onemocnění. Díky dostupnosti koagulačních faktorů VIII a IX mohou být u pacientů s hemofilií bez inhibitoru bezpečně prováděny operační a invazivní výkony. Cílem těchto doporučení je shrnutí zkušeností a doporučení pro zajištění operací včetně optimální aktivity faktorů VIII a IX, laboratorního monitorování a doby trvání substituční léčby po chirurgických výkonech. Zvláštní pozornost je věnována postupu u pacientů léčených bispecifickou monoklonální protilátkou emicizumabem.

Haemophilia A and B are rare congenital bleeding disorders. Invasive procedures and surgery can be performed safely in patients with haemophilia without inhibitors due to the availability of coagulation factors VIII and IX. The aim of these guidelines is to summarize experience and recommendations for treatment including optimal factors VIII/IX levels during surgery, laboratory monitoring and replacement treatment duration after these procedures. A special attention is drawn to the procedures in patiens being treated by the bispecific monoclonal antibody emicizumab.

• Keywords
• emicizumab,
• MeSH
• Surgical Procedures, Operative MeSH
• Factor IX administration & dosage   therapeutic use   MeSH
• Factor VIII administration & dosage   therapeutic use   MeSH
• Hemophilia A * drug therapy   complications   MeSH
• Hemophilia B * drug therapy   complications   MeSH
• Hemorrhage prevention & control   MeSH
• Humans MeSH
• Antibodies, Bispecific administration & dosage   therapeutic use   MeSH
• Check Tag
• Humans MeSH
• Publication type
• Practice Guideline MeSH

Kočky mohou být častým zdrojem původců infekčních onemocnění. Jedním z onemocnění je bartonelóza, tzv. nemoc z kočičího škrábnutí (Cat Scratch Disease – CSD). Původcem jsou bakterie z rodu Bartonellaceae, které způsobují závažné uzlinové syndromy. V našem sdělení popisujeme klinické případy závažných lymfadenitid při bartenolóze a problematiku diferenciálně diagnostickou včetně možné onkologické etiologie, kterou musíme brát do úvahy u uzlinových syndromů u dětí.

Cats may be source of pathogens causing various infectious diseases, including bartonellosis, also called cat scratch disease – CSD. CSD is caused by the bacterium Bartonella henselae, and may be associated with severe lymphadenitis. We present case studies of severe lymphadenitis and discuss difficulties of differential diagnosis of lymph node enlargement in children.

• MeSH
• Bartonella henselae MeSH
• Diagnostic Imaging methods   MeSH
• Child MeSH
• Bartonella Infections * diagnosis   therapy   MeSH
• Cats microbiology   MeSH
• Lymphadenitis MeSH
• Magnetic Resonance Imaging methods   MeSH
• Zoonoses MeSH
• Check Tag
• Child MeSH
• Cats microbiology   MeSH
• Male MeSH
• Female MeSH
• Publication type
• Case Reports MeSH

BACKGROUND: The prevalence of autoimmune thyroiditis (AIT), as the most common autoimmune disease (AD) and papillary thyroid cancer (PTC) is steadily rising in children. The aim of this study was to determine the coexistence of other AD and thyroid carcinoma (TC) in AIT. METHODS: The cross-sectional study conducted at a tertiary center comprised AIT children (< 19 years). Data on age/sex, thyroid function and ultrasound, autoantibodies, associated AD, familial occurence of AD and the occurence of TC for each child were collected. RESULTS: In total, 231 eligible patients (77% females) were included. The most common onset (66%) was during adolescence. At onset, hypothyroidism was detected in 59.3%; hashitoxicosis in 1.3%. The positivity of both autoantibodies was present in 60.6%, the negativity was in 3,5%. We confirmed a high frequency (44.6%) of AD with AIT predominance in parents and/or grandparents of patients and in siblings (7.4%). 15.2% had at least 1 comorbid AD, of which type 1 diabetes mellitus was the most common (8.5%). Over a period of 7 years TC was diagnosed in 16 patients (mean age 13.5 years) with predominance of PTC in 15 (94%) patients. AIT had concurrently 69% patients. 56% of patients had metastases (89% in AIT subjects). An invasive PTC was present in 44% (86% in AIT subjects). CONCLUSIONS: The prevalence rate of AD in AIT and first-degree relatives is high, and several new associations have been reported. Providers should be aware of comorbidities and TC in AIT as this would help in early diagnoses and timely interventions.

• MeSH
• Autoimmune Diseases * diagnosis   MeSH
• Thyroiditis, Autoimmune * diagnosis   MeSH
• Child MeSH
• Genetic Predisposition to Disease MeSH
• Comorbidity MeSH
• Humans MeSH
• Adolescent MeSH
• Thyroid Neoplasms diagnosis   MeSH
• Carcinoma, Papillary MeSH
• Child, Preschool MeSH
• Prevalence MeSH
• Retrospective Studies MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Adolescent MeSH
• Male MeSH
• Child, Preschool MeSH
• Female MeSH
• Publication type
• Journal Article MeSH
• Research Support, Non-U.S. Gov't MeSH
• Geographicals
• Czech Republic MeSH

• MeSH
• Arginine Vasopressin administration & dosage   therapeutic use   MeSH
• Blood Coagulation Disorders, Inherited MeSH
• Diagnosis, Differential MeSH
• Factor VIII analysis   administration & dosage   pharmacokinetics   therapeutic use   MeSH
• Hemophilia A * diagnosis   etiology   drug therapy   MeSH
• Hemophilia B * diagnosis   etiology   drug therapy   MeSH
• Hemostatics MeSH
• Joints pathology   MeSH
• Coagulants MeSH
• Hemorrhage etiology   drug therapy   classification   MeSH
• Humans MeSH
• Prognosis MeSH
• Practice Guidelines as Topic MeSH
• Muscles pathology   MeSH
• Synovitis therapy   MeSH
• Tertiary Prevention MeSH
• Thrombosis therapy   MeSH
• Check Tag
• Humans MeSH

• MeSH
• Cyclophosphamide administration & dosage   therapeutic use   MeSH
• Diagnosis, Differential MeSH
• Factor VII administration & dosage   therapeutic use   MeSH
• Factor VIII analysis   MeSH
• Glucocorticoids MeSH
• Hemophilia A * diagnosis   epidemiology   MeSH
• Adrenal Cortex Hormones adverse effects   MeSH
• Immunologic Factors MeSH
• Immunosorbent Techniques MeSH
• Immunosuppression Therapy adverse effects   MeSH
• Coagulants MeSH
• Drug Therapy, Combination MeSH
• Pregnancy Complications MeSH
• Humans MeSH
• Prednisone administration & dosage   therapeutic use   MeSH
• Prothrombin administration & dosage   therapeutic use   MeSH
• Rituximab administration & dosage   therapeutic use   MeSH
• Practice Guidelines as Topic * MeSH
• Check Tag
• Humans MeSH

• MeSH
• Diagnosis, Differential MeSH
• Erythrocytes immunology   MeSH
• Humans MeSH
• Adolescent MeSH
• Psychophysiologic Disorders immunology   psychology   MeSH
• Purpura * immunology   psychology   MeSH
• Check Tag
• Humans MeSH
• Adolescent MeSH
• Female MeSH
• Publication type
• Case Reports MeSH

• MeSH
• Antifibrinolytic Agents therapeutic use   MeSH
• Deamino Arginine Vasopressin administration & dosage   MeSH
• Child MeSH
• Hemostatics administration & dosage   MeSH
• Humans MeSH
• von Willebrand Diseases * diagnosis   drug therapy   genetics   MeSH
• Check Tag
• Child MeSH
• Humans MeSH
• Male MeSH
• Publication type
• Case Reports MeSH