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Journal of the Belgian Society of... Nov 2020Nasu-Hakola disease (NHD) is characterized by multiple bone cysts in the appendicular skeleton and progressive presenile dementia.
Nasu-Hakola disease (NHD) is characterized by multiple bone cysts in the appendicular skeleton and progressive presenile dementia.
PubMed: 33336143
DOI: 10.5334/jbsr.2303 -
Veterinary Journal (London, England :... Oct 2021Nerve growth factor (NGF), a critical mediator of nociception, is a novel analgesic therapeutic target. Bedinvetmab, a canine monoclonal antibody (mAb), binds NGF and...
Nerve growth factor (NGF), a critical mediator of nociception, is a novel analgesic therapeutic target. Bedinvetmab, a canine monoclonal antibody (mAb), binds NGF and inhibits its interaction with tropomyosin receptor kinase A (trkA) and p75 neurotrophin receptor (p75) receptors. The objective of three integrated laboratory studies was to demonstrate the safety of bedinvetmab in adult laboratory Beagle dogs. Daily health, veterinary, clinical pathology, systemic exposure, and anti-drug antibody evaluations were performed. Study 1 additionally included electrocardiography, neurologic, and ophthalmic assessments, and radiographic monitoring of joints of the appendicular skeleton. Study 2 evaluated T-lymphocyte-dependent immune function. Study 3 evaluated the safety of short-term concurrent administration of carprofen, a nonsteroidal anti-inflammatory drug (NSAID), with bedinvetmab. Studies 1 and 3 included terminal pathology and histopathology evaluations. Study designs and procedures included directed complementary morphologic and functional evaluations of a literature- and in vitro-based list of potential safety issues related to the NGF signaling pathway and characteristics engineered into this mAb. Screening-level general procedures evaluated effects associated with mAbs that target and inhibit soluble agonist cytokines. There were no treatment-related adverse changes in clinical evaluations, clinical neurological and ophthalmic examinations, joints, immune morphology or function, and no effects of short-term concurrent NSAID usage. Treatment-emergent immunogenicity was not observed. Bedinvetmab (1 mg/kg SC monthly; 3× and 10× dose multiples) was well tolerated in normal laboratory Beagle dogs for 6 months and with 2 weeks' concurrent NSAID administration.
Topics: Animals; Antibodies, Monoclonal; Dogs; Laboratories; Nerve Growth Factor; Receptor, Nerve Growth Factor; Signal Transduction
PubMed: 34391918
DOI: 10.1016/j.tvjl.2021.105733 -
American Journal of Medical Genetics.... May 2020Ollier disease (OD) and Maffucci syndrome (MS) are characterized by multiple enchondromas. Patients with MS also have benign vascular overgrowths that become malignant...
Ollier disease (OD) and Maffucci syndrome (MS) are characterized by multiple enchondromas. Patients with MS also have benign vascular overgrowths that become malignant in 8.5% of cases. OD is characterized by multiple enchondromas, typically unilateral in distribution with a predilection for the appendicular skeleton. MS is characterized by multiple enchondromas bilaterally distributed in most of the cases. Both disorders feature multiple swellings on the extremity, deformity around the joints, limitations in joint mobility, scoliosis, bone shortening, leg-length discrepancy, gait disturbances, pain, loss of function, and pathological fractures. About 50% of patients with OD or MS develop a malignancy, such as chondrosarcoma, glioma, and ovarian juvenile granulosa cell tumor. To better understand the natural history of OD and MS, we reviewed 287 papers describing patients with OD and MS. We also created a survey that was distributed directly to 162 patients through Facebook. Here, we compare the review of the cases described in the literature to the survey's responses. The review of the literature showed that: the patients with OD are diagnosed at a younger age; the prevalence of chondrosarcomas among patients with OD or MS was ~30%; in four patients, vascular anomalies were identified in internal organs only; and, the prevalence of cancer among patients with OD or MS was ~50%. With these data, health care providers will better understand the natural history, severity, and prognosis of these diseases and the prevalence of malignancies in these patients. Here, we recommend new guidelines for the care of patients with OD and MS.
Topics: Adolescent; Adult; Child; Child, Preschool; Chondrosarcoma; Enchondromatosis; Female; Granulosa Cell Tumor; Humans; Infant; Infant, Newborn; Male; Middle Aged; Ovarian Neoplasms; Prognosis; Young Adult
PubMed: 32144835
DOI: 10.1002/ajmg.a.61530 -
Diagnostics (Basel, Switzerland) Jan 2022Osteomyelitis caused by spp. is a severe, but rare, clinical entity. However, clear guidelines regarding the most effective medical management have not yet been... (Review)
Review
BACKGROUND
Osteomyelitis caused by spp. is a severe, but rare, clinical entity. However, clear guidelines regarding the most effective medical management have not yet been established. The present study is a literature review of all such cases, in an effort to elucidate epidemiology, as well as the therapeutic management and the infection's outcome.
METHODS
A thorough review of all reports of osteomyelitis of the appendicular and the axial skeleton, without the skull and the spine, caused by spp. was undertaken. Data about demographics, imaging techniques facilitating diagnosis, causative , method of mold isolation, antifungal treatment (AFT), surgical treatment, as well as the infection's outcome were recorded and evaluated.
RESULTS
A total of 63 cases of osseous infection due to spp. were identified. The studied population's mean age was 37.9 years. The most commonly affected site was the rib cage (36.8%). Most hosts suffered immunosuppressive conditions (76.2%). Regarding imaging methods indicating diagnosis, computer tomography (CT) was performed in most cases (42.9%), followed by plain X-ray (41.3%) and magnetic resonance imaging (MRI) (34.9%). The most frequent isolated mold was (49.2%). Cultures and/or histopathology were used for definite diagnosis in all cases, while galactomannan antigen test was additionally used in seven cases (11.1%), polymerase chain reaction (PCR) in four cases (6.3%), and beta-d-glucan testing in three cases (4.8%). Regarding AFT, the preferred antifungal was voriconazole (61.9%). Most patients underwent surgical debridement (63.5%). The outcome was successful in 77.5%.
DISCUSSION
Osteomyelitis due to spp. represents a severe infection. The available data suggest that prolonged AFT in combination with surgical debridement is the preferred management of this infection, while identification of the responsible mold is of paramount importance.
PubMed: 35054368
DOI: 10.3390/diagnostics12010201