Periodontal Manifestation of Type Ib Glycogen Storage Disease: A Rare Case Report.

INTRODUCTION: Glycogen storage diseases (GSD) are genetic metabolic disorders of glycogen metabolism. There are >15 types based on the enzyme deficiency and the affected organ. Glycogen storage disease Type Ib is the only type associated with neutropenia and periodontitis. This type is caused by a deficiency of glucose-6-phosphate (G6P) translocase which prevents the transport of G6P across the endoplasmic reticulum. As a result, glycogen cannot be metabolized into glucose with its subsequent accumulation in tissues. The affected organs involved in Type Ib are the liver, kidney, and intestine. CASE PRESENTATION: A 5-year-old Jordanian boy from a consanguineous family referred to the periodontal clinic in February 2014 with an established diagnosis of GSD-Ib. The systemic manifestations include hepatomegaly, hypoglycemia, hyperprolactenemia, inflammatory bowel disease, osteoporosis, and neutropenia. Oral manifestations include severe gingival inflammation and recurrent oral ulceration disease. CONCLUSIONS: The clinical signs and symptoms of periodontal disease in GSD Type Ib are similar to those found in patients diagnosed with neutropenia. Future studies are needed to clarify whether severe generalized inflammation of the gingiva in children is part of the GSD Type Ib or is a separate entity caused by neutrophil dysfunction.

Aggressive Gingival Metastasis of Adenocarcinoma of Unknown Origin: Case Report.

A case of localized soft tissue growth located on the lingual aspect of the left mandibular gingiva is reported in a 61-year-old male patient in whom excisional biopsy of the lesion was performed. The histopathologic diagnosis was adenocarcinoma. Further clinical and biopsy examinations taken from several organs indicated the presence of metastatic adenocarcinomas in the brain, spleen, lymph nodes, and liver. The oral tumor recurred after repeated surgical removal. Based on histopathological examination and immunoprofiling analysis the primary tumor probably originated in the gastrointestinal tract.

Papillon-Lefèvre or Haim-Munk Syndrome? Report on Two Sisters in a Consanguineous Family.

INTRODUCTION: Papillon-Lefèvre (PLS) and Haim-Munk (HMS) syndromes are rare autosomal recessive type IV palmar plantar keratosis (PPK), which are clinically different from other types of PPK by their association with aggressive periodontitis and genetically by the mode of transmission. CASE PRESENTATION: Two Jordanian sisters, aged 10 and 13 years, from a consanguineous family were preliminarily diagnosed as having PLS by a pediatric dentist who referred them to the periodontal clinic in May 2010 because of the presence of loose teeth and to a dermatologist for the management of existing skin lesions. Physical examination of the two sisters revealed severe bilateral hyperkeratotic lesions on the palms and soles extending to the lateral and dorsal aspects; bilateral pes planus; scaly patches on the knees and elbows; overgrown fingernails and toenails that were curved inward; and range of movement limitations in the fingers. The patients were treated with topical emollients and keratolytic preparations containing salicylic acid for the skin lesions. Oral radiographs showed generalized severe bone loss around the remaining primary and permanent teeth. Oral treatment included dental prophylaxis, chlorhexidine mouthrinse, amoxicillin syrup, and extraction of several teeth. CONCLUSIONS: The two patients in this case report presented atypical symptoms of PLS. Mutational genetic analysis is proposed to confirm whether PLS and HMS are the same or two different symptoms.

Anatomical landmarks of maxillary bifurcated first premolars and their influence on periodontal diagnosis and treatment.

OBJECTIVE: To assess the anatomical landmarks of the roots of bifurcated maxillary first premolars and study their effect on the diagnosis and management of periodontal disease. METHODS: One hundred sixty-five maxillary first premolars were selected. The frequency of single-, two-rooted, and three-rooted premolars was assessed, but only the dual-rooted were used for the purpose of this study. For each tooth, the following measurements were obtained using a micrometer caliper: buccal and palatal root length, mesial and distal root trunk length, crown length, and width of the furcation entrance. The types of root trunk were classified according to the ratio of root trunk height to root length into types A, B and C. Root trunk types A, B and C are defined as root trunks involving the cervical third or less, up to half of the length of the root, or greater than the apical half of the root, respectively. The presence of any root grooves and concavities, as well as bifurational ridges, was assessed. The crown to root ratio was calculated. RESULTS: Of the 165 maxillary first premolar teeth retrieved, 100 (60.6%) were two-rooted, 62 (37.57%) were single-rooted, and three (1.81%) were triple-rooted. Type A root trunks comprised only 7% of the examined teeth, while types B and C had more or less comparable results (46% and 47% respectively). Type B was more common in distal root trunks while type C was dominant in mesial root trunks. Bifurcation ridges were observed in 37% of the teeth; the mean root trunk length was greater in teeth with bifurcation ridges than in teeth without (7.41 mm vs. 5.96 mm). Root grooves and concavities were found in 96% of the mesial aspects of the root, and in 57% of the palatal aspect of the buccal root. The mean width of the furcation entrance was 0.89 +/- 0.19 mm (range 0.39-1.28). The average crown to root ratio was 0.69:1. CONCLUSION: Awareness of root surface anatomical variations may help the practitioner when assessing the diagnosis, treatment plan and prognosis of periodontally involved two-rooted maxillary premolars.

Periodontal manifestation of leukocyte adhesion deficiency type I.

BACKGROUND: Leukocyte adhesion deficiency type I (LAD-I) is an autosomal recessive immunodeficiency disorder characterized by defects in the integrin receptors of white blood cells that lead to impaired adhesion and chemotaxis. Affected patients are susceptible to recurrent bacterial and fungal infections, impaired pus formation, delayed wound healing, and periodontitis. METHODS: A case of generalized advanced periodontal destruction of the permanent and deciduous dentition in a young Jordanian girl with a severe phenotype of LAD-I is presented in this report. The medical diagnosis was made based on the characteristic clinical and laboratory findings, in particular the total absence of CD18, CD11b, and CD11c as determined by flow cytometry sorting. RESULTS: Periodontal findings in this patient include the early onset of the disease, which affected the primary teeth and permanent dentitions, the intense redness and inflammation of the gingiva, and the rapid periodontal destruction that seems refractory to conventional non-surgical periodontal therapy. CONCLUSION: This report emphasizes the importance of the differential diagnosis of severe immunodeficiency disorders in children and adolescents and mandates the importance of combined care by medical and dental practitioners to prevent tooth loss and control oral infection.

Correlation and examiner agreement between a new method of plaque scoring and a popular established plaque index, modelled in vitro.

BACKGROUND, AIMS: Scoring of supragingival plaque is common to epidemiological studies, oral hygiene assessments and clinical trials of oral hygiene products. These clinical practices utilize indices such as the Turesky et al. (1970, Journal of Periodontology 41, pp. 41-43) modification [Turesky Plaque Index (TPI)] of the Quigley & Hein (1962, Journal of the American Dental Association 65, pp. 26-29) plaque index for evaluating plaque extension. Whilst this index is in common use by clinicians, there is potential for ambiguity in the interpretation of scores by different examiners. The aims of this study were to compare examiner agreement between a new method of plaque scoring (NMPS) and TPI, and to determine if there was a correlation between the two indices. METHODS: Simulated plaque was scored from eight study casts, each consisting of 14 teeth. The two indices were both scored twice from the study casts by eight examiners experienced in plaque scoring. The first readings obtained were analysed for a correlation between the indices and between-examiner variability. Within-examiner variability was assessed using both first and second readings of each index. RESULTS: A positive correlation was found between the two indices (r = 0.717). The NMPS was found to have better inter- and intra-examiner agreement than TPI. CONCLUSION: The NMPS was found to have less variability within and between examiners than TPI: evaluation of NMPS in a clinical setting would now appear warranted.