Oral findings in patient with lethal hypophosphatasia treated with enzyme replacement therapy

We report here a 3-year-old female diagnosed with perinatal HPP who had received enzyme replacement therapy starting from 1 day after birth. Oral and radiographic examinations revealed deep periodontal pockets, severe mobility, and dentinogenesis imperfecta with root formation defects, especially in the mandibular primary second molars, while the tooth germs of permanent teeth except for the first molars were unclear. Dental manifestations of lethal HPP following treatment with enzyme replacement therapy were markedly different from those of mild HPP.
Source: Pediatric Dental Journal - Category: Dentistry Source Type: research

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CONCLUSIONS AND CLINICAL RELEVANCE: Results suggested that identification of ≥ 4 B lines extending from the epicardium of the left ventricle into the lung field on pericardial LUS may be useful in the diagnosis of CPE in dogs with DMVD. Additional research is needed to determine whether pericardial LUS allows differentiation between CPE and pneumonia. PMID: 32101047 [PubMed - in process]
Source: American Journal of Veterinary Research - Category: Veterinary Research Authors: Tags: Am J Vet Res Source Type: research
Diffuse esophageal spasm (DES) causes symptoms such as dysphagia. However, DES is sometimes difficult to diagnose. DES can be diagnosed based on a characteristic image through esophageal radiography. As in the present case, DES is considered a good indication for peroral endoscopic myotomy. AbstractDiffuse esophageal spasm (DES) causes symptoms such as dysphagia. However, DES is sometimes difficult to diagnose. DES can be diagnosed based on a characteristic image through esophageal radiography. As in the present case, DES is considered a good indication for peroral endoscopic myotomy.
Source: Clinical Case Reports - Category: General Medicine Authors: Tags: CLINICAL IMAGE Source Type: research
ConclusionThe MED technique is highly effective for surgical resection of primary and recurrent maxillary IPs involving the anterior wall, providing complete access to the entire maxillary sinus. In many cases, the MED can obviate the need for an adjunctive sublabial or transseptal incision, while also providing excellent exposure for postoperative surveillance.
Source: International Forum of Allergy and Rhinology - Category: Allergy & Immunology Authors: Tags: ORIGINAL ARTICLE Source Type: research
Authors: Sanki T, Endo H, Tetsunaga T, Furumatsu T, Yamada K, Ozaki T Abstract We investigated the relationship between the initial contact status and stress shielding in total hip arthroplasty (THA) using fit-and-fill type straight-stem implants. In addition we evaluated the clinical and radiographic outcomes. Subjects were 100 hips of 94 patients who underwent THA and were followed-up for ≥10 years. Contact areas with the femoral cortical bone were investigated according to the zonal distribution of Gruen using postoperative CT images. Depending on the number of contact areas, the patients were classified into...
Source: Acta Medica Okayama - Category: General Medicine Tags: Acta Med Okayama Source Type: research
Conclusions: Alignment in the sagittal plane is a unique challenge facing surgeons for patients with AIS. Further research with an assessment of functional outcomes and longer follow-up is needed to more precisely guide treatment principles. Level of Evidence: Level IV.
Source: Journal of Spinal Disorders and Techniques - Category: Surgery Tags: Narrative Review Source Type: research
Conclusion: Interbody fusion and percutaneous reduction with MMED provides a minimally invasive procedure for lumbar spondylolisthesis, with sufficient decompression, reduction, fusion, and satisfactory clinical results.
Source: Journal of Spinal Disorders and Techniques - Category: Surgery Tags: Primary Research Source Type: research
Conclusions: Paraspinal muscles evaluated with MRI were more significantly associated with the pathology of ASD compared with appendicular skeletal muscle evaluations with dual-energy x-ray absorptiometry. The present study indicates that multifidus and erector muscles significantly influenced the maintenance of the pelvic alignment. Level of Evidence: Level III.
Source: Journal of Spinal Disorders and Techniques - Category: Surgery Tags: Primary Research Source Type: research
ConclusionIndividuals did not report an increase in knee pain, stiffness or disability in their ACL ‐deficient knee. Only disability was associated with worsening severity of radiographic OA in ACL‐deficient knees.
Source: International Journal of Rheumatic Diseases - Category: Rheumatology Authors: Tags: ORIGINAL ARTICLE Source Type: research
Nonsteroidal antiinflammatory drugs (NSAIDs) are the first ‐line pharmacotherapy for patients with axial spondyloarthritis (SpA). In recent years, treatment options have expanded with the availability of biologic agents, including tumor necrosis factor inhibitors and interleukin‐17 inhibitors. However, a treatment strategy that clearly prevents syndesmo phyte formation has not been established. Observational studies of patients with ankylosing spondylitis indicated potential disease‐modifying effects of NSAIDs, but two randomized trials came to different conclusions. More broadly, whether any of the currently availab...
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: New Perspectives in Rheumatology Source Type: research
ConclusionsIn contrast to the well ‐established association between black race and knee or hip OA, odds of hand OA are lower in black versus nonblack subjects, which is not mediated by known hand OA risk factors. Future mechanistic works are warranted to determine the mediating protective factors for hand OA among the black subject s.
Source: Arthritis and Rheumatology - Category: Rheumatology Authors: Tags: FULL LENGTH Source Type: research
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