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Orofacial granulomatosis (OFG) is an uncommon condition with varying clinical presentation. Gingival enlargement in children could be due to a varied etiology. The present case report is of an adolescent female with initial presentation of generalized gingival enlargement, lip swelling and perioral discoloration without any known etiopathological factors or systemic involvement. Conservative excision of the enlargement was performed and histopathological examination revealed a non caseating granulomatous lesion. Diagnosis of orofacial granulomatosis in context to sarcoidosis was arrived after excluding other granulomatous diseases. Follow up after 18 months showed no recurrence and regression of lip swelling and perioral discoloration. Gingival enlargement can be considered as one of the presenting features of sarcoidosis., Competing Interests: The authors declare no conflict of interest., (©2022 The Author(s). Published by MRE Press.)

Autoimmune neutropenia is a self-limiting condition characterized by the presence of granulocyte-specific autoantibodies. This case report describes a 2-year-old female who presented to the Department of Pediatric Dentistry with a history of oral ulceration, complicated by high temperature, gingival hypertrophy and gingival hemorrhage. These symptoms had precipitated an admission to a district general hospital and diagnosis of viral tonsillitis. The severity of the presentation to the Pediatric Dental Department prompted a review of recent discharge blood investigations, which revealed a neutrophil count of 0.07×10 9 /L(1.0-8.5 ×10 9 /L) indicating severe neutropenia. Urgent referral to Pediatric Hematology resulted in same-day admission and a diagnosis of autoimmune neutropenia was established. The patient was managed with chlorhexidine mouthwash, prophylactic antimicrobial and antifungal medication and granulocyte colony stimulating factor (G-CSF). Neutropenia may present with features exclusive to the oral cavity. Recognition of atypical presentations such as oral ulceration with associated systemic illness may assist in early diagnosis.

Background: Renal transplant is the renal replacement therapy of choice for all patients of chronic kidney disease. The aim of this study was to analyse the trends of medical complications in renal transplant recipients at our centre., Methods: it is a prospective cross sectional descriptive study. All the patients undergoing renal transplant at Armed Forces Institute of Urology from September 2013 to September 2015 were included in the study. The patients were followed prospectively till March 2016 and a complete data about their complications and lab investigations was maintained.., Results: This study included a total of 63 patients with a mean duration of follow-up of 14.05 months (SD±4.45). Infective complications as a group are the commonest complication occurring in over 50% of cases followed by haematological complications (17.5%), new onset diabetes after transplant (15.9%) and transplant dysfunction (14.3%) Cardiovascular complications were seen in only 4.8% cases but with high mortality. Gingival hypertrophy was seen in 4.8% cases., Conclusions: Medical complications are common after renal transplant especially in the early post-operative period. The only way forward is early recognition and aggressive treatment, as delays can cost losses in the form of kidney function, life and higher health care cost.

Mucolipidosis type II (ML II, OMIM 252,500) is an autosomal recessive disorder clinically characterized by facial dysmorphia similar to Hurler syndrome and pronounced gingival hypertrophy. The disorder is caused by a defect in targeting acid hydrolases on the surface of lysosomes, which impede their entry and lead to accumulation of undigested substrates in lysosomes. The onset of the symptoms is usually in infancy, beginning in the 6th month of life. Early onset, at birth or even in utero, is a sign of severity and involves the specific dysmorphia as well as skeletal dysplasia related to hyperparathyroidism. We report on a severe neonatal form of this disorder revealed by respiratory distress with severe chest deformity. The dysmorphic syndrome, combining coarse features, pronounced gingival hypertrophy, with diffuse bone demineralization and secondary hyperparathyroidism associating significant elevation of parathyroid hormone and alkaline phosphatase with normal levels of vitamin D and calcium were characteristics of mucolipidosis type II. Recognizing this specific association of anomalies helps eliminate the differential diagnosis and establish appropriate diagnosis and care., (Copyright © 2015 Elsevier Masson SAS. All rights reserved.)

Urbach-Wiethe disease (lipoid proteinosis) is an autosomal recessive disorder that is characterized by a general thickening of the skin and mucous membranes. We report the case of a 22-year-old woman with lipoid proteinosis who presented with hoarseness, poor dentition, and skin lesions, and we discuss the management of this rare disease.

Acute myeloid leukemias (AMLs) are aggressive hematopoietic neoplasms that, if untreated, can lead to death within days. Owing to its high morbidity rate, early diagnosis and appropriate medical therapy is essential. Oral lesions may be the presenting feature of acute leukemias and are, therefore, important diagnostic indicators of the disease. Erythematous or cyanotic gingival hyperplasia with or without necrosis is reported to be the most consistent symptom leading to a diagnosis of acute leukemia that directs the patient to seek early dental consultation. This report refers to a patient with AML that was provisionally diagnosed in the dental hospital due to severe gingival enlargements.

Background: Human multipotent mesenchymal stromal cells (hMSCs) produce tumor necrosis factor (TNF)-α-stimulated protein 6 (TSG-6). TSG-6 modulates proinflammatory cytokine cascades and enhances tissue repair. This study tests the effects of recombinant human TSG-6 (rhTSG-6) on gingival wound healing within the first 2 days post-surgery., Methods: After gingival resection in 120 Sprague-Dawley rats, 2 µg rhTSG-6 in 5-µL phosphate-buffered saline (PBS) or the same volume of only PBS solution was injected into gingival tissue approximating the surgical wound. Control animals did not receive injections. Tissue biopsies and blood were collected at 1 to 2, 6 to 8, 24, and 48 hours post-surgery (n = 10 per group). Specimens were analyzed via histologic analysis and enzyme-linked immunosorbent assay (ELISA) for quantification and comparison of inflammatory markers interleukin (IL)-1β, IL-6, TNF-α, and myeloperoxidase (MPO). Wound photographs were taken for a double-masked clinical assessment at each time period. Weights were recorded for all animals pre- and post-surgery., Results: Animals injected with rhTSG-6 had significantly less severe clinical inflammation at 6 to 8 (P = 0.01228), 24 (P = 0.01675), and 48 (P = 0.0186) hours. Sham and control animals had more weight loss at 24 and 48 hours. Sham and control animals had more pronounced cellular infiltrate. rhTSG-6-treated animals had significantly less MPO (P = 0.027) at 24 hours and IL-1β (P = 0.027) at 24 and 48 hours. IL-6 showed a marginal significant difference at 6 to 8 hours, but there was no significant difference for TNF-α., Conclusion: rhTSG-6 reduced postoperative gingival inflammation by reducing levels of proinflammatory cytokines and cellular infiltrate and may offer significant promise as an anti-inflammatory agent for gingival surgery.

Juvenile hyaline fibromatosis is a rare, hereditary disease with distinct clinical and histopathological features. Clinically, it presents with gingival hypertrophy, pappulonodular skin lesions and joint contractures. Bone involvement is usually an uncommon finding. We report a case of a 2-year-old patient, daughter of consanguineous parents, who presented since the age of 2 months with impairment of mental development, multiple joint contractures, motion limitation and nodules on the scalp. The calvarian lesions were surgically removed, and histopathological examination concluded to juvenile hyaline fibromatosis.

Introduction: Gingival enlargement is a common form of periodontal tissue reaction to several irritating factors. The most common form is the drug related gingival hyperplasia--nevertheless the heredity gingival fibromatosis and hematological cases can also occur and might impose a challenge to periodontists. After a short literature summary three Case reports are presented. The first case is a drug related gingival overgrowth in a young kidney transplant women who took Cyclosporin-A. The excessive mass of fibrotic tissue was removed by a series of internal beveled incision and the oral and buccal gingival flaps were united with sutures. The healing was uneventful and during the follow up patient's compliance and oral hygiene was superb. The second case is a very severe antihypertensive drug related gingival overgrowth in a 62 years old man interfering with the closure of his lip and corrected with a combination of conventional gingivectomy and internal reverse beveled incision both and Ca-channel blockers. The third case is a 42 years old woman with chronic idiopathic hemolytic anemia who presented a sudden onset acute excessive generalized gingival enlargement accompanied with severe pain and fever. At admission she was suspect for leukemia. After obtaining biopsy samples and having negative histology the soft tissue mass was removed under general anesthesia with conventional gingivectomy technique, but after a couple of days the severe pain and gingival swelling recurred. With administering systemic corticosteroid therapy (32 mg Medrol), the gingiva healed in five days and the one year follow-up showed a stable hematological and periodontal status. Today the more conservative internal beveled incision is preferred over the conventional gingivectomy in the most cases because it provides a more predictable healing and better esthetics. The recurrence of the drug related gingival hyperplasia can be anticipated by meticulous postoperative individual oral hygiene and regular supportive therapy., Conclusion: The combined conservative and surgical therapy leads to predictable postoperative result even in very severe systematically motivated gingival enlargements, nevertheless the successful patients management needs good cooperation with medical doctors and with the patients themselves.

Introduction: Cowden syndrome is rare; oral symptoms are not always diagnosed. This case of Cowden syndrome was revealed by gingival hypertrophy., Observation: A 21-year-old female patient consulted for gingival hypertrophy and multiple papules in the mouth. She had a history of thyroid lobectomy due to a goiter. The gingival papillomatosis and the goiter suggested a Cowden syndrome. The diagnosis was confirmed clinically by facial skin papules., Discussion: Cutaneous and oral lesions are usually the first symptoms of the syndrome. Diffuse gingival papillomatosis may suggest a Cowden syndrome and should lead to screen for associated symptoms. A high-risk diagnosis of breast and thyroid cancer is associated to Cowden syndrome and the patient should have a yearly follow-up., (Copyright © 2013. Published by Elsevier Masson SAS.)

Oral cavity reflects the health status of an individual. Many systemic diseases have signs and symptoms that manifest in the oral cavity, which in most of the cases precede the systemic manifestations. These array of diseases also include acute leukemias which present as gingival hyperplasia as the most consistent symptom seeking dental consultation that can be easily confused with many other benign conditions that present as gingival enlargements. One such rare case of adult acute lymphoblastic leukemia is presented in this article.

Objective: Biliary atresia is a congenital disease treated by liver transplantation. Adults may have oral consequences of the medical therapy. Green teeth are oral manifestations of the induced hyperbilirubinemia. Gingival enlargement is associated with the immunosuppressive drug. This case report describes the successful treatment of an 18-year-old patient displaying severe green teeth and gingival enlargement., Method and Materials: The gingival enlargement was treated by reducing the pathogenic oral microflora through scaling and root planing of the teeth, gingival excision surgeries, and conversion from cyclosporin to tacrolimus., Results: Gingival enlargement and inflammation had completely disappeared after nonsurgical treatment for the maxilla and after surgical treatment for the mandible. The green coloration of the teeth was masked using composite restorations., Conclusion: This case report indicates that a patient's quality of life can be improved by a team approach combining pharmacologic and dental therapies.

Objective: To identify the risk factors for periodontal changes in adult patients during orthodontic treatment by evaluating the periodontal status of banded second molars using the gingival index (GI)., Material and Methods: The sample consisted of 100 adult patients divided into two groups: the Orthodontics group, undergoing corrective treatment with fixed appliances and bands cemented to the four second molars, and the Control group, with no prior history of orthodontic treatment, age and sex matched. Group GI values were compared using the Wilcoxon test. Additionally, a multivariate logistic regression was performed to study the risk factors for increases in the GI., Results: Individuals in the Orthodontics group showed a significantly higher GI than those in the Control group. Logistic regression revealed that among the risk factors found to increase GI, the following proved significant (in order of importance): plaque index, subgingival encroachment of the cervical margins of bands, probing depth, and length of orthodontic treatment., Conclusions: Banded second molars of adult patients during orthodontic treatment showed more clinical signs of gingival inflammation than those of untreated individuals. Moreover, major risk factors identified included the presence of plaque and the presence of subgingival band margins.

Background: Infantile systemic hyalinosis is a rare genetic disorder which involves accumulation of hyaline in the skin, bones, mucous membranes, and occasionally, also in internal organs. The major manifestations include painful articular contractures, cutaneous lesions (hyperpigmentation, subcutaneous nodules), malnutrition resulting from diarrhoea, gingival, labial and buccal hypertrophy., Case Report: The phenotype characteristics of infantile systemic hyalinosis (ISH) in a two year old boy were present. The characteristics of flattered occiput, limited limb movements and articular abnormalities of elbows and knees. Dental findings showed excessive gingival hypertrophy completely covering maxillary and mandibular teeth treatment. The gingival hypertrophy was surgically treated by gingivectomy under general anaesthesia. FOLLOWUP: The patient showed a full constellation of clinical manifestations of the disease. Despite the surgical intervention no improvement in oral hygiene was observed., Conclusions: Surgical treatment of the gingival hypertrophy was the treatment of choice.

Summary: We studied morphological changes at the maxillary first molar in a model rat for type 2 spontaneous diabetes mellitus (DM), the Goto-Kazizaki (GK) rat, vs. the normal 8-week-old Wistar rat. Serial frontal sections of the gingiva of the maxilla with the bone were prepared from the rats. Image analyses, performed on light micrographs of the hematoxylin-eosin stained specimens, allowed comparison of the thickness of the keratinized, granular, prickle, and basal layers. In addition, the cell population of the granular and prickle layers and the cross-sectional area of the connective tissue beneath the mucosal epithelium were examined. The thickness of the capillary of the maxillary first molar was determined by image analysis of scanning electron micrographs of microvascular corrosion cast specimens. We found that the thickness of the keratinized, granular, and prickle layers was significantly higher in the DM vs. normal group, as were the cell population of the granular and prickle layers. In contrast, the cross-sectional area of the connective tissue beneath the mucosal epithelium, and the thickness of the capillary were significantly lower in the DM vs. normal sections. Therefore, we consider that the DM-associated hyperglycemia causes hypertrophy of the mucosal epithelium, atrophy of the connective tissue beneath the mucosal epithelium, and microangiopathy of the capillary of the palatal gingiva of the maxillary first molar in the GK rat.

A 66-year-old female presented with gum bleeding and soreness. Her medical history was significant for delayed wound healing, which appeared to cause blindness in her right eye. A gingival incisional biopsy revealed replacement of fibrous connective tissue by an amorphous and eosinophilic material. Direct immunofluorescent staining for antibodies was negative. After the differential diagnosis of ligneous gingivitis (LG) was rendered, the activity and antigenic level of plasminogen was tested and found to be significantly decreased. Partial improvement was observed within 1 year following a regimen of scaling, gingival curettage, topical steroids, and improved oral hygiene. LG is a rare condition, occurring as a result of plasminogen deficiency and subsequent fibrin accumulation. It presents as pseudomembranous gingiva and might affect the eyes and other organs. Dentists should be familiar with this condition, since they might assist in a diagnosis of this disease, with significant morbidity often missed by medical professionals., (© 2011 Blackwell Publishing Asia Pty Ltd.)

We report one case of ligneous periodontitis, which is a clinical sign of hypoplasmino-genemia. It appears as massive, painless ulcerated gingival enlargements and alveolar bone destruction in the affected area. The course of the disease is progressive and typically ends with early loss of teeth. At present, no efficient treatment option seems to be available. To investigate the cause of the rapid bone destruction in this disease, gingival tissue specimens were taken from one patient and a healthy control patient to compare the function of fibroblastic cells. Our results showed that diseased fibroblasts (1) reorganized collagen lattices more rapidly than normal cells, (2) demonstrated a greater overall production of pro and active matrix metalloproteinase-2 (MMP-2) and increased activation of this protease, and (3) showed a more active phenotype than healthy fibroblastic cells. From these preliminary results, there seems to be increased MMP-2 production and activation, which might be one compensatory (but insufficient) mechanism for the decreased (plasmin-dependent) pericellular fibrinolysis in plasminogen-deficient patients. Further studies on this subject should evaluate the exact pathomechanism of plasminogen on this enzymatic bone and connective tissue destruction.

Introduction: In this study, we aim to compare the levels of matrix metalloproteinase 9 (MMP9) in the gingival crevicular fluid (GCF), as indicators for healing, in two groups of patients - operated with a classic periodontal surgical technique and the same technique but using a dental microscope., Materials: We included 14 patients with ages between 12 and 26 years, average 14±6.2 years. Eight patients were women and six men. All patients presented gingival hypertrophy because of the orthodontic treatment on the mandibular arch. We performed gingivectomy on one-half of the mandibular arch by classic periodontal surgery and on the other half of the mandibular arch by a microscope-assisted gingivectomy., Methods: In the hypertrophied gingiva, the expression of MMP9 was identified using immunohistochemical-staining techniques. For immunological determination of MMP9 in GCF we performed Elisa tests., Results: We found different levels in different moments of the healing process for the two hemiarcades., Conclusions: We consider that faster healing in case of microscope-assisted gingivectomy may be related to the expression of MMP-9 in the GCF.

Background: Leukemia is a fatal disease. The oral manifestations of the leukemias occur early in the course of the disease and these oral features can at times act as a diagnostic indicator. Saliva has been used as a diagnostic aid in a number of systemic diseases., Materials and Methods: In our study, samples of unstimulated saliva of 30 leukemia patients who were not on chemotherapy were collected and analyzed for salivary amylase and total protein. The oral manifestations and radiographic changes (OPG) were recorded. The correlation between the oral manifestations and the salivary components (salivary amylase and total protein) was assessed for prognostic significance., Results: In the present study when the mean values of salivary amylase (1280±754 U/ml) and total protein (647.2±320.7 mg%) were compared with that in control subjects. There was a statistically significant difference for amylase levels (P<.05). On intraoral examination the study subjects showed pallor, gingivitis, gingival enlargement, petechiae, and ecchymosis. On the OPG, the radiographic features included generalized rarefaction of bone (20%), thinning of lamina dura (3.4%), generalized alveolar crest bone resorption (30%), thinning of walls of alveolar crypts (6.7%), besides others, e.g., periapical abscess (10%)., Conclusions: The saliva of leukemic patients demonstrated obvious changes in composition. A rise in salivary amylase and total protein levels was evident, with the increase in amylase levels being statistically significant.

Leukemia is a hematological disorder arises from a hematopoietic stem cell characterized by a disordered differentiation and proliferation of neoplastic cells. Rapidly forming generalized gingival hyperplasia is usually the first sign of this disease (especially in acute forms). This case report describes a 54-year-old female who presented rapid gingival enlargement in only three weeks time, heralding the presence of acute monocytic leukemia (AML-FAB M5). The patient was immediately referred for hematologic management, but died five weeks later. Dentists and generalists should always be on guard to observe any oral manifestations that may lead to the early diagnostic of systemic diseases.

Traumatic lesions in primary teeth are frequent in pediatric patients and can cause problems both to the deciduous tooth and permanent successor. The impact strength on deciduous tooth can reach the growing permanent tooth, affecting its morphology, structure and position, or even hampering its proper development. This report describes an aesthetic-functional rehabilitation process in an 8 year 10 month old boy during a multidisciplinary treatment held at the Clinical Center of Dental Trauma in Primary Teeth of the Pediatric Dentistry of Dental College of University of São Paulo, Brazil. The patient presented bilateral posterior cross bite and the permanent left upper central incisor with ectopic eruption and enamel hypoplasy, preceded by avulsion of element 61, occurred when the patient was 1.6 years old. After diagnosis and treatment planning, a quick expansion of jaws was recommended with Hass-type rapid expander and orthodontic leveling with fixed braces. Due to the ectopic eruption, the gingival contour had been altered and hypertrophia was found, compromising aesthetics and avoiding local hygienic procedures. Gingivoplasty was carried out with high-intensity Diode Laser, followed by aesthetic restoration with compound resin. It was concluded that after deciduous teeth traumatism it is important that the patient undergoes clinic and radiographic assistance until the permanent teeth erupt so that an adequate multidisciplinary treatment can be offered to the patient.

Unlabelled: Gingival overgrowth occurs as a response to several drugs, in some systemic diseases, as a consequence of a genetic predisposition or could be idiopathic, the last being less studied. Independently of the etiological factor involved, histological changes are maintained and intensified by the presence of the bacterial plaque., Material and Methods: Fibrotic mucosa clinically diagnosed with idiopathic hypertrophy was included in paraffin and studied using usual histological stains and immunohistochemical techniques for vimentin, MMP-3 and TIMP-2., Results: Evaluation of histological changes revealed an unspecific pattern like the thickening of the epithelium, with acanthosis and acantholysis and massive deposition of connective tissue in the lamina propria. Keratinocytes from the areas with acantholysis express MMP-3. In the fibrous areas we noted few fibroblasts and a discrete expression of MMP-3. Extended areas of inflammatory tissue with numerous de novo capillaries were observed among the collagen bundles. Proinflammatory and endothelial cells expressed both MMP-3 and TIMP-2. The last antibody had an intense expression deeper in the epithelium. Our results were discussed in accordance with reference data., Conclusions: We conclude that the histological changes observed were not specific for the idiopathic gingival hypertrophy and the imbalance of tissue homeostasis was due to the interaction between keratinocytes and connective cells in the milieu of specific changes induced by the inflammatory conditions.

Gingival enlargement is a condition that commonly develops during orthodontic treatment. Orthodontic appliances are irritation and retention plaque factors holding up oral hygiene and control of gingival inflammation. Two cases of gingival hypertrophy in young Senegalese females undergoing orthodontic treatment with fixed appliances are described and treated by gingivectomy. This surgical procedure have led to morphological conditions of gingiva allowing better plaque control and the orthodontic treatment going on. Periodical controls in child and adolescent are required for healthy periodontium during orthodontic therapy. Collaboration between orthodontist and periodontist is one of the most important keys to successful treatment.

Gingival enlargement is a condition that commonly develops during orthodontic treatment. Orthodontic appliances are irritation and retention plaque factors holding up oral hygiene and control of gingival inflammation. This case-report shows localized gingival hypertrophy in young Senegalese female undergoing orthodontic treatment with fixed appliances. Modified Widman flap associated with osseous recontouring have led to morphological conditions of gingiva allowing better plaque control and orthodontic treatment going on. Comfort and controlled haemostasis after periodontal surgery make this procedure a good choice for treatment of gingival hypertrophy. Collaboration between orthodontist and periodontist is required for successful therapeutic.

Aim: To compare undisturbed plaque formation on teeth bonded with different types of orthodontic brackets with non-bonded control teeth, via a de novo plaque growth experiment over a 7-day period., Material and Methods: A randomized controlled trial with split-mouth design was set up enroling 16 dental students. Within each subject sites with Speed(S) and GAC(G), brackets and control sites were followed. Clinical periodontal parameters were recorded at baseline, on days 3 and 7. Microbiological samples were taken from the brackets and the teeth on days 3 and 7., Results: Both anaerobe and aerobe colony-forming units (CFU) were significantly higher in S-sites than in G-sites (p=0.0002, p=0.02). The shift from aerobic to anaerobic species was observed earlier in S-sites than in G-sites. The aerobe/anaerobe CFU ratio was significantly lower in S-sites than in G-sites (p=0.01). On day 3, the crevicular fluid flow was significantly higher in S-sites than in control sites (p=0.01). On day 7, S-sites and G-sites showed a significantly higher crevicular flow than control sites (both p<0.0001). More hypertrophy was seen in S- than in G- and control sites (p=0.05). No significant differences for bleeding on probing were observed., Conclusion: Bracket design can have a significant impact on bacterial load and on periodontal parameters.

With the advent of effective drug therapy, tuberculous lesions of the oral cavity have become so rare that they are frequently forgotten. Primary gingival tuberculosis is extremely rare and usually manifests as ulcer. We report the first case of primary tuberculosis manifesting as gingival enlargement, which was the only presenting sign of tuberculosis. Diagnosis was based on histopathology (hematoxin and eosin staining), complete blood count, polymerase chain reaction assay and immunologic investigation with the detection of antibodies against Mycobacterium tuberculosis. The possibility of gingival enlargement due to drugs, leukemia, fungus and sarcoidosis was ruled out. Antituberculous therapy over 6 months was followed by surgical excision of the residual enlargement under local anesthesia. After 1-year follow-up there was no recurrence of the disease. This case emphasizes the need for dentists to include tuberculosis in the differential diagnosis of gingival enlargement so that they may play a role in its early detection.

Lymphoblastic lymphoma-leukemia (LBLL) most commonly presents with mediastinal masses (50-75%), while pleural and pericardial effusion may also be present. Lymphadenopathy usually in the neck, axilla or supraclavicular regions, is considered as another typical presentation of the disease. This is a case report of a six-year-old boy with unusual huge enlargement of maxilla, mandible and soft palate as well as gingival hypertrophy which led to secondary respiratory and feeding difficulties. Morphologic and flowcytometric evaluation of bone marrow aspiration showed that it was a T cell type acute leukemia which may be due to dissemination of a lymphoblastic lymphoma and considered as a case of lymphoma-leukemia. After appropriate treatment, the symptoms of the patient relieved significantly and he is in complete remission for about one year.

Juvenile hyaline fibromatosis (JHF) is a rare systemic disease characterized by papulonodular skin lesions, gingival hyperplasia, joint contractures, and osteolytic lesions on long bones and the skull. It has recently been reported that the disease is caused by mutations in the gene encoding capillary morphogenesis protein-2 (CMG-2). To date, fewer than 60 cases have been published in the literature. Partial disease expression is common, but no cases featuring a solitary calvarial lesion have been reported. The authors discuss this 4-year-old boy with a solitary calvarial osteolytic lesion whose histopathological examination exhibited findings characteristic of JHF. Mutational analysis, however, revealed that there were no mutations in the CMG-2 gene. Two years after surgery, he was free of any complaints as well as gingival hyperplasia, joint contractures, and new skull or skin lesions. This patient's condition may represent clinical or genetic heterogeneity associated with JHF. Whether solitary lesions mimicking JHF can arise from somatic mutation of the CMG-2 gene remains to be proven.

Glycogen storage disease type Ib is a rare inherited metabolic disorder that is caused by a deficiency of glucose-6-phosphate translocase with consequent accumulation of glycogen. The purpose of this study is to report a case affected by glycogen storage disease type Ib in which unusual oral findings were evident and to review the pertinent literature. The disease presents with failure to thrive, hepatomegaly, hypoglycemia, hyperlacticacidemia, neutropenia, and neutrophilic dysfunction causing increased susceptibility to recurrent infections. Common intraoral manifestations are dental caries, gingivitis, periodontal disease, delayed dental maturation and eruption, oral bleeding diathesis, and oral ulcers. Conversely, unusual oral lesions were observed in this case as hyperplastic-hypertrophic gingiva and giant cell granulomatous epulis. The treatment with granulocyte colony-stimulating factor markedly increased the neutrophil counts and reduced the frequency of infections and inflammations. Proper evaluation of the patient's oral condition, a program of preventive measures, and suitable medical consultation are important to minimize and avoid long-term complications.

Hyalinosis cutis et mucosae (lipoid proteinosis, Urbach-Wiethe disease) is a rare syndrome with autosomal recessive inheritance. The disease is characterized by diffuse deposition of a hyalinelike substance in the dermis, submucosal connective tissue, and various internal organs. In this study, the patient demonstrated classic signs and symptoms of lipoid proteinosis except for gingival infiltration. Gingival infiltration is still an unexplainable feature of this disease. In the context of this case, the diagnostic significance of the microscopic findings of the gingival tissues and the possible factors playing a role in gingival hypertrophy, are discussed.

Unlabelled: There is little information on the oral health of children undergoing renal transplantation during the early transplant period., Methods: Twenty-four children undergoing renal transplantation aged 4-13.2 years and their matched controls were recruited. The dmfs, dmft, DMFS and DMFT, plaque, gingivitis and gingival enlargement scores were recorded. The oral microflora was sampled and cultured for S. mutans, Lactobacllus species and Candida species., Results: There was a significantly lower mean dmfs (0.3 +/- 0.9; P = 0.03), dmft (0.3 +/- 0.9; P = 0.03), DMFS (2.3 +/- 5.3; P = 0.01) and DMFT (1.5 +/- 2.6; P = 0.02), respectively, in the transplant group. There was a significantly greater mean plaque score (14.7 +/- 11) for the permanent dentition, at baseline only, compared with 90 days post-transplantation (9.4 +/- 10.4; P = 0.02). There was a significantly greater gingival enlargement score (1.8 +/- 1.4; P = 0.04) 90 days post-transplantation compared with baseline. The S. mutans and Lactobacillus counts were significantly lower both at baseline (P = 0.0001 and P = 0.004) and 90 days post-transplantation (P = 0.02; and P = 0.05), respectively, compared with the controls., Conclusions: The transplant children had less active dental disease than the controls although gingival enlargement needs careful monitoring.

Radiotherapy for the nasopharyngeal cancer patient with poor oral care may lead to severe deterioration of the dentition and may require multiple extractions. Although the use of an implant-retained overdenture can successfully restore the function and esthetics of edentulous patients, its use can be complicated by the tissue changes subsequent to head and neck irradiation. The difficulties in implant treatment planning for an edentulous patient with macroglossia and soft tissue changes following radiotherapy are discussed.