TY  - JOUR
A1  - Schmidt-Hieber, M.
A1  - Silling, G.
A1  - Schalk, E.
A1  - Heinz, W.
A1  - Panse, J.
A1  - Penack, O.
A1  - Christopeit, M.
A1  - Buchheidt, D.
A1  - Meyding-Lamadé, U.
A1  - Hähnel, S.
A1  - Wolf, H. H.
A1  - Ruhnke, M.
A1  - Schwartz, S.
A1  - Maschmeyer, G.
T1  - CNS infections in patients with hematological disorders (including allogeneic stem-cell transplantation)-Guidelines of the Infectious Diseases Working Party (AGIHO) of the German Society of Hematology and Medical Oncology (DGHO)
JF  - Annals of Oncology
N2  - Infections of the central nervous system (CNS) are infrequently diagnosed in immunocompetent patients, but they do occur in a significant proportion of patients with hematological disorders. In particular, patients undergoing allogeneic hematopoietic stem-cell transplantation carry a high risk for CNS infections of up to 15%. Fungi and Toxoplasma gondii are the predominant causative agents. The diagnosis of CNS infections is based on neuroimaging, cerebrospinal fluid examination and biopsy of suspicious lesions in selected patients. However, identification of CNS infections in immunocompromised patients could represent a major challenge since metabolic disturbances, side-effects of antineoplastic or immunosuppressive drugs and CNS involvement of the underlying hematological disorder may mimic symptoms of a CNS infection. The prognosis of CNS infections is generally poor in these patients, albeit the introduction of novel substances (e.g. voriconazole) has improved the outcome in distinct patient subgroups. This guideline has been developed by the Infectious Diseases Working Party (AGIHO) of the German Society of Hematology and Medical Oncology (DGHO) with the contribution of a panel of 14 experts certified in internal medicine, hematology/oncology, infectious diseases, intensive care, neurology and neuroradiology. Grades of recommendation and levels of evidence were categorized by using novel criteria, as recently published by the European Society of Clinical Microbiology and Infectious Diseases.
KW  - Central nervous system
KW  - Polymerase chain raction
KW  - Herpes simplex encephalitis
KW  - Invasive fungal-infections
KW  - Clinical practice guidelines
KW  - Liposomal amphotericin-B
KW  - Immunocompromised patient
KW  - Progressive multifocal leukoencephalopathy
KW  - Varicella-zoster-virus
KW  - Diagnosis
KW  - Treatment
KW  - Bone-marrow-transplantation
KW  - Real-time PCR
KW  - Guideline
KW  - Central nervous system infection
Y1  - 2016
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-188210
VL  - 27
IS  - 7
ER  - 
TY  - JOUR
A1  - Politei, Juan M.
A1  - Bouhassira, Didier
A1  - Germain, Dominique P.
A1  - Goizet, Cyril
A1  - Guerrero-Sola, Antonio
A1  - Hilz, Max J.
A1  - Hutton, Elspeth J.
A1  - Karaa, Amel
A1  - Liuori, Rocco
A1  - Üceyler, Nurcan
A1  - Zeltzer, Lonnie K.
A1  - Burlina, Alessandro
T1  - Pain in fabry disease: practical recommendations for diagnosis and treatment
JF  - CNS Neuroscience & Therapeutics
N2  - Aims: 
Patients with Fabry disease (FD) characteristically develop peripheral neuropathy at an early age, with pain being a crucial symptom of underlying pathology. However, the diagnosis of pain is challenging due to the heterogeneous and nonspecific symptoms. Practical guidance on the diagnosis and management of pain in FD is needed. 

Methods: 
In 2014, experts met to discuss recent advances on this topic and update clinical guidance. 

Results: 
Emerging disease-specific tools, including FabryScan, Fabry-specific Pediatric Health and Pain Questionnaire, and Wurzburg Fabry Pain Questionnaire, and more general tools like the Total Symptom Score can aid diagnosis, characterization, and monitoring of pain in patients with FD. These tools can be complemented by more objective and quantifiable sensory testing. In male and female patients of any age, pain related to FD can be an early indication to start disease-specific enzyme replacement therapy before potentially irreversible organ damage to the kidneys, heart, or brain occurs. 

Conclusion: 
To improve treatment outcomes, pain should be diagnosed early in unrecognized or newly identified FD patients. Treatment should include: (a) enzyme replacement therapy controlling the progression of underlying pathology; (b) adjunctive, symptomatic pain management with analgesics for chronic neuropathic and acute nociceptive, and inflammatory or mixed pain; and (c) lifestyle modifications.
KW  - Enzyme replacement therapy
KW  - Small fiber dysfunction
KW  - System involvement
KW  - Outcome survey
KW  - Fabry disease
KW  - Randomized controlled-trial
KW  - Chronic neuropathic pain
KW  - Agalsidase beta
KW  - Screening questionnaire
KW  - Dose reduction
KW  - Adult patients
KW  - Diagnosis
KW  - Pain
KW  - Peripheral nervous system
Y1  - 2016
U6  - http://nbn-resolving.de/urn/resolver.pl?urn:nbn:de:bvb:20-opus-188127
VL  - 22
IS  - 7
ER  -