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AbstractThe aim of this letter to the editor was to highlight the importance of adding sources of support and conflicts of interests in the published articles so that they are adequately analysed for their quality by using AMSTAR‐2 tool.

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.

AbstractHajdu‐Cheney syndrome (HCS) also known as Cranio‐skeletal dysplasia is a rare genetic disorder of bone metabolism. It is mainly characterized by acro‐osteolysis and generalized osteoporosis. The other distinctive features include a dysmorphic face, short stature, aplasia of facial sinuses, and persistent cranial sutures. Although the condition begins to manifest since birth, the characteristic features become more prominent with age. This syndrome is usually recognized by dentists due to these craniofacial abnormalities. This case report aims to highlight a case of 6‐year‐old girl HCS who presented with aberrant facial features, premature exfoliation of teeth, unusual mobility of teeth and atypical root resorption in primary dentition.

AbstractIntroductionThe short‐term effects of chemotherapy and irradiation are well documented; however, there is paucity regarding their long‐term effects, especially in children and adolescents.Case DescriptionThis paper discusses the long‐term effects of chemotherapy and/or radiation received by the patients during their early childhood on the developing dentition. It comprises the compilation of 11 cases with alteration in the dental development screened from 138 cases of the childhood cancer patients who received the chemotherapy and/or radiation as a part of anticancer therapy.Results and ConclusionThe findings revealed that the age of initiation of anticancer therapy along with the synergistic effect of chemo‐irradiation, and the dose of radiation used were the principal determinants for the dental abnormalities. The root‐related abnormalities were found to be varied and more common as compared to the missing teeth and defects related to the tooth crown.

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.

ABSTRACTJalili syndrome (JS) (MIM#217080) is a rare autosomal recessive disorder with oculo‐dental malformations. The clinical phenotype is characterized by the presence of Cone‐Rod Dystrophy (CRD) and Amelogenesis Imperfecta (AI). Genetic mechanism entails a mutation in the CNNM4, a metal transporter gene located on Chromosome 2q11.2. A high fluoride concentration in groundwater has also been identified as an epigenetic factor in this syndrome. JS draws the attention of dentists due to its distinct oral manifestations. To the best of our knowledge, this is the first genetically confirmed pediatric case report from the Indian subcontinent emphasizing the clinical and radiographic features of this condition and its management in a 6‐year‐old child.