Suchergebnisse

AbstractBackgroundKlippel–Fiel syndrome (KFS) is a rare congenital skeletal disorder characterized clinically by presence of a triad of short neck, limited neck mobility (due to fused cervical vertebrae) and low posterior hair line. It was first described by Maurice Klippel and Andre Feil in 1912. Various skeletal and non‐skeletal anomalies may be seen in association with KFS.Case PresentationThis report aims to highlight orofacial manifestations of a 16‐year‐old male patient with KFS along with a rare presentation of bilateral osteoarthritic changes in the temporomandibular joint. The treatment planning and execution for such a case has also been described.ConclusionBilateral osteoarthritic changes of temporomandibular joint have been rarely reported in KFS. This report emphasizes that early diagnosis of various associated anomalies and timely intervention through an interdisciplinary approach is very essential in the management of patients with KFS.

AbstractGriscelli syndrome (GS) is an autosomal‐recessive disorder of the vesicle transport and membrane trafficking system first identified by Griscelli et al in 1978. The three types of GS have specific genetic defects and systemic manifestations apart from classic partial pigmentary dilution, resulting in hypopigmentation of skin and silvery hair. GS‐II occurs due to a defect in the Rab27a gene and is characterized by primary immune deficiency along with accelerated phases of a hemophagocytic lymphohistiocytosis (HLH) crisis. This rare disorder has been widely studied for dermatological, hematological, and neurological manifestations; however, the oral features and presentations have not been elucidated in detail. This report presents a case of a 4‐year‐old male with known mutation c.550C > T or p.R184X mutation (ENST00000396307) in Rab27a with oral features.

ABSTRACTLeukocyte adhesion defect I is a rare disorder (1:1,000,000) caused by diminished expression of CD‐18 β2 integrins on leukocytes leading to abnormal adhesion, migration, and chemotaxis. Clinical manifestations include delayed separation of umbilical cord, omphalitis, recurrent severe infections, impaired wound healing, persistent oral ulcers, and severe periodontitis in primary and permanent dentition.A 5‐year‐old girl, second‐born child to parents with consanguinity, presented with pain and mobility of lower teeth. There was history of recurrent infections and multiple hospital admissions with CD18 level‐3% and frame shift mutation in ITGB2, on 21q22.3. There were scars on hands and feet. Oral examination revealed multiple missing teeth and periodontitis in primary dentition. Oral prophylaxis and palliative treatments were performed with periodic follow‐ups.Interdisciplinary care is ubiquitous for patients with immune deficiencies. Early consultation with pediatric dentists and exploration of medical history is essential for diagnosis and treatment of rare diseases.

Intro -- Preface -- Acknowledgments -- Contents -- Chapter 1: Hospital Designing and Planning -- 1.1 Introduction -- 1.2 Need of Hospital Planning -- 1.3 Hospital Planning Process -- 1.3.1 Hospital Planning Team -- 1.3.2 Hospital Consultant -- 1.3.3 Techniques Adopted by the Planning Team -- 1.3.4 Feasibility Analysis Done by the Hospital Planning Team -- 1.4 Size of the Hospital -- 1.5 Site Selection -- 1.6 Hospital Architect -- 1.7 Preparation of Detailed Architectural Report -- 1.7.1 Major Departments of a General Hospital -- 1.8 Identifying Equipment Needs -- 1.8.1 Acquiring New Equipment -- 1.8.2 Addition of Equipment -- 1.8.3 Replacement of Old or Obsolete Equipment -- 1.8.4 Calculation of Requirement of Equipment -- 1.8.5 Collection of Information -- 1.8.6 Product Evaluation and Specification Audit -- 1.9 Equipping a Hospital -- 1.9.1 Plants and Equipment Required in the Hospital -- 1.10 Interiors and Graphics -- 1.10.1 Graphic Art and Design -- 1.10.2 Types of Graphics -- 1.10.3 Use of Decorative Colors in Hospitals -- The Cultural Meanings of Colors in a Healthcare Setting -- Psychological Impacts of Colorations in the Healthcare Environment -- Colors as a Signage System in Hospitals -- International and NABH-Accepted Medical Institution Signage Criteria -- Chromotherapy -- Color and Health -- 1.11 Construction -- 1.12 Commissioning -- 1.12.1 The Commissioning Team -- 1.12.2 Scheduling the Sequence of the Services -- 1.12.3 Categorization of Services [13] -- Shake-Down Period -- References -- Chapter 2: Importance of Hospital Management -- 2.1 Healthcare -- 2.2 Healthcare Industry -- 2.3 Classification of Healthcare Industry -- 2.3.1 Based on Funding and Governance -- Public Sector in Healthcare -- Private Sector in Healthcare -- 2.3.2 Based on Service Delivery -- Healthcare Providers -- Healthcare Financiers -- Life Sciences.

AbstractMoebius syndrome (MS) is a rare congenital neuromuscular disorder characterized by weakness or paralysis (palsy) of abducens and facial nerves, or other cranial nerves which may be affected. Diagnosis, treatment, and dental management of MS patients are focused on treating manifestations like malocclusion, while catering to associated extraoral (neurologic, dermatologic, ocular) complications, aiming to improve their quality of life. Here, we report the case of a 9‐year‐old female patient with MS who underwent orthodontic camouflage using combined orthopedic‐orthodontic therapy using a high‐pull chin cup and fixed orthodontic appliance to improve skeletal mal‐relation and facial appearance. The outcome displayed great improvement in function and better esthetics, improving not only the patient's but also the family's quality of life. A year's follow‐up showed successful maintenance of the achieved results. A multidisciplinary approach in MS not only helps in overcoming the treatment challenges but also provides great psychosocial benefits to these patients.

AbstractObjectiveTo assess and analyze the global status of parental responses to feeding problems in patients with cleft lip and palate (CLP) and to provide recommendations for future research.DesignA priori protocol was formulated according to PRISMA guidelines and registered. The predefined search strategy was used in established search engines up to July 27, 2020 without any restriction of language or year of publication. Studies evaluating the parental responses to feeding problems in patients with CLP were included. The data extraction was performed with a self‐designed form, and risk of bias (ROB) was assessed.ResultsOut of 27 identified papers, eight studies could be included. Most were from Europe, conducted between 1994 and 2020, and had utilized valid and reliable questionnaires. The included studies showed moderate or low ROB. The majority of the studies reported parental dissatisfaction with the knowledge provided. The presence of anxiety and low self‐esteem among parents was also highlighted.ConclusionsThere was general consensus regarding lack of medical advice and support for CLP families, along with the presence of anxiety and low self‐esteem among parents. Well‐designed studies from different regions of the world must be conducted in the future for an accurate global picture to be presented.