Periodontal status in two siblings with severe congenital neutropenia: diagnosis and mutational analysis of the cases

BACKGROUND: Severe congenital neutropenia (SCN), also known as Kostmann syndrome, was originally reported as an autosomal recessive disease of neutrophil production. The disease is characterized by a maturation arrest of neutrophil precursors at the promyelocytic stage of differentiation and by extremely low levels of mature neutrophils in peripheral blood. METHODS: A 6-year-old male presented with a complaint of gingival swelling and bleeding, and swelling at the left side of his face. Upon clinical examination, severe inflammation of all gingival tissues was apparent, and a periapical abscess with mobility was noted on the left mandibular second molar. Medical and dental histories revealed numerous recurrent bacterial infections associated with oral and non-oral tissues. His medical history with recurrent infections led us to evaluate his 3-year-old sister to determine the status of her oral health. Inflammation of her oral tissues and recurrent bacterial infections were apparent. Their consanguineous parents were in good health. To assist in identifying possible systemic diseases underlying the inflammatory situation in the siblings, consultations were requested from the Pediatric Hematology Department at Selcuk University and Pediatric Oncology Department at Gulhane Military Medical Academy. RESULTS: Based on absolute neutrophil count (< or =200/mm(3)) and bone marrow aspiration findings consistent with early maturation arrest in myelopoiesis, the cases were diagnosed as SCN. No chromosomal abnormality was detected upon cytogenetic examination. Sequencing analysis also revealed no mutation in the neutrophil elastase or growth factor independent-1 (GFI-1) genes in these patients. Severe periodontal disease, attachment loss, and mobility for over 50% of the deciduous teeth were noted. Within 6 months, the male sibling lost all of his deciduous teeth due to periapical and periodontal infections. His sister presented with tooth mobility for all mandibular incisors. Monthly visits, including scaling, polishing, and 0.2% chlorhexidine digluconate irrigation were performed to support their oral hygiene and to avoid recurrent oral infections. We have been able to stabilize these patients' periodontal conditions during a 2-year follow-up period. CONCLUSION: This case report emphasizes the role of periodontists and pediatric dentists in the diagnosis of diseases linked with neutrophil and other systemic disorders and highlights the need to optimize the health of oral tissues with regular appointments.     

Clinical, genetic, and biochemical findings in two siblings with Papillon-Lefèvre Syndrome

BACKGROUND: Papillon-Lefèvre Syndrome (PLS) is an autosomal recessive disease characterized by palmoplantar hyperkeratosis and severe periodontitis affecting both primary and secondary dentitions. Cathepsin C (CTSC) gene mutations are etiologic for PLS. The resultant loss of CTSC function is responsible for the severe periodontal destruction seen clinically. METHODS: A 4-year-old female (case 1) and her 10-year-old sister (case 2) presented with palmoplantar skin lesions, tooth mobility, and advanced periodontitis. Based on clinical findings, the cases were diagnosed with PLS. Mutational screening of the CTSC gene was conducted for the cases, and their clinically unaffected parents and brother. Biochemical analysis was performed for CTSC, cathepsin G (CTSG), and elastase activity in neutrophils for all members of the nuclear family. The initial treatment included oral hygiene instruction, scaling and root planing, and systemic amoxicillin-metronidazole therapy. RESULTS: CTSC mutational screening identified a c.415G>A transition mutation. In the homozygous state, this mutation was associated with an almost complete loss of activity of CTSC, CTSG, and elastase. Although monthly visits, including scaling, polishing, and 0.2% chlorhexidine digluconate irrigation were performed to stabilize the periodontal condition, case 1 lost all her primary teeth. In case 2, some of the permanent teeth could be maintained. CONCLUSIONS: This report describes two siblings with a cathepsin C gene mutation that is associated with the inactivity of cathepsin C and several neutrophil serine proteases. The failure of patients to respond to periodontal treatment is discussed in the context of these biological findings.     

Influence of previous provisional cementation on the bond strength between two definitive resin-based luting and dentin bonding agents and human dentin

This study evaluated the effect of two different types of provisional luting agents (RelyX Temp E, eugenol-based; RelyX Temp NE, eugenol-free) on the shear bond strengths between human dentin and two different resin-based luting systems (RelyXARC-Single Bond and Duo Link-One Step) after cementation with two different techniques (dual bonding and conventional technique). One hundred human molars were trimmed parallel to the original long axis, to expose flat dentin surfaces, and were divided into three groups. After related surface treatments for each specimen, the resin-based luting agent was applied in a silicone cylindrical mold (3.5 x 4 mm), placed on the bonding-agent-treated dentin surfaces and polymerized. In the control group (n = 20), the specimens were further divided into two groups (n = 10), and two different resin-based luting systems were immediately applied following the manufacturer's protocols: RelyX ARC-Single Bond (Group I C) and Duo Link-One Step (Group II C). In the provisionalization group (n = 40), the specimens were further divided into four subgroups of 10 specimens each (Group I N, I E and Group II N, II E). In Groups I N and II N, eugenol-free (RelyX NE), and in groups I E and II E, eugenol-based (RelyX E) provisional luting agents (PLA), were applied on the dentin surface. The dentin surfaces were cleaned with a flour-free pumice, and the resin-based luting systems RelyX ARC (Group I N and E) and Duo Link (Group II N and E) were applied. In the Dual bonding groups (n = 40), the specimens were divided into four subgroups of 10 specimens each (Group I ND, ED and Group II ND, ED). The specimens were treated with Single Bond (Groups I ND and ED) or One Step (Groups II ND and ED). After the dentin bonding agent treatment, RelyX Temp NE was applied to Groups I ND and II ND, and RelyX Temp E was applied to Groups I ED and II ED. The dentin surfaces were then cleaned as described in the provisionalization group, and the resin-based luting systems were applied: RelyX ARC-Single Bond (Group I ND and ED) and Duo Link-One Step (Group II ND and ED). After 1,000 thermal cycles between 5 degrees C and 55 degrees C, shear bond testing was conducted at a crosshead speed of 0.5 mm/minutes. One-way ANOVA, followed by a post hoc Tukey test (alpha = 0.05) was done. The dentin-resin-based luting system interfaces were evaluated under a scanning electron microscope. There was a significant reduction in the mean shear bond strength values of groups subjected to the provisionalization compared to the control and dual bonding technique groups (p < 0.05). The composition of provisional luting did not create a significant difference with regard to reducing shear bond strength values (p > 0.05). With regard to resin based luting systems, the shear bond strength values of the double-bond technique groups were not significantly different from the controls (p > 0.05).     

Clinical and biochemical effects of diode laser as an adjunct to nonsurgical treatment of chronic periodontitis: a randomized, controlled clinical trial

The aim of this randomized, parallel, controlled clinical trial was to examine the clinical and biochemical efficacy of diode laser as an adjunct to scaling and root planing (SRP). Thirty chronic periodontitis patients were randomly assigned into two groups to receive SRP alone (control) or SRP followed by diode laser (test). Plaque index, gingival index, bleeding on probing, probing depth, and clinical attachment level were measured at baseline and at 1, 3, and 6 months after treatment. The gingival crevicular fluid levels of interleukin-1β (IL-1β), interleukin-6 (IL-6), interleukin-8 (IL-8), matrix metalloproteinase-1 (MMP-1), matrix metalloproteinase-8 (MMP-8) and tissue inhibitor matrix metalloproteinase-1 (TIMP-1) were analyzed by enzyme-linked immunosorbent assay. Test group showed significantly a better outcome compared to the control group in full-mouth clinical parameters. MMP-1, MMP-8, and TIMP-1 showed significant differences between groups after treatment compared to baseline (p?<?0.05). The total amount of IL-1β, IL-6, MMP-1, MMP-8, and TIMP-1 decreased (p?<?0.05) and IL-8 increased after treatment in both test and control groups (p?<?0.05). Diode laser provided significant improvements in clinical parameters and MMP-8 was significantly impacted by the adjunctive laser treatment at first month providing an insight to how lasers can enhance the outcomes of the nonsurgical periodontal therapy.     

Morphometric study of the scapular free flap and the free rib osteomyocutaneous flap

The scapula free flap is often the first choice for reconstruction of bony defects of the facial skeleton. However, the vascularised rib as part of a free rib osteomyocutaneous flap may be a suitable second choice. We have investigated the morphology and clinical dimensions of the 7th rib and the scapula, and the ability of the available bone to carry dental implants. The age and sex of the cadaver, and the donor side, were also recorded. The dimensions of the scapulas and 7th ribs (n = 130 of each) from 65 cadavers were measured at 4 different points using osteometric methods. Examination showed that bone from the scapula and 7th rib were sufficient for placement of implants. The 7th rib gave reliable measurements for both height and width, and a consistent relation between compact and cancellous bone. Although the scapula provided adequate compact and cancellous bone, there were variations depending on the segment of bone chosen. Bones from male cadavers were more suitable for implantation. In both the scapula and the 7th rib ageing had a significant adverse effect in only one dimension. Most points of measurement have satisfactory bony dimensions for insertion of dental implants.     

The risk of venous thromboembolism associated with the factor V Leiden mutation and low B-vitamin status.

Venous thromboembolism (VTE) is a multi-factorial disease involving numerous genetic and environmental risk factors. In this study we investigated the occurrence and the risk associated with factor V Leiden, hyperhomocysteinemia and low folate and vitamin B12 levels in young patients with thrombosis. We studied 78 patients (33 females/45 males, mean age 33 years) with a history of thrombosis in a lower limb. Additionally, 98 healthy subjects (45 females/54 males, mean age 44 years) were included. Serum levels of homocysteine (Hcy), folate and vitamin B12 were assayed. Factor V Leiden and methylenetetrahydrofolate reductase (MTHFR) C677T mutations were investigated in all subjects. Factor V Leiden was highly prevalent in the patients (39% heterozygous, 10% homozygous vs. 6.3% heterozygous in controls). An increase in the risk of idiopathic VTE was associated with Hcy levels > 15.2 micromol/l (odds ratio, OR = 2.83), folate < 15.1 nmol/l (OR = 7.49) and vitamin B12 < 182 pmol/l (OR = 11.97). Low levels of folate or vitamin B12 were independently and strongly associated with the risk of VTE in a multivariate model (OR for idiopathic thrombosis = 16.44 and 10.76, respectively). Twenty patients (53%), carriers of factor V Leiden, had low levels of vitamin B12, compared to 28% of patients who were non-carriers of the mutation (p = 0.03). In contrast, none of the control carriers of the mutation had a low level of vitamin B12. The risk of VTE associated with lower levels of vitamin B12 and folate was stronger than that introduced by elevated Hcy levels. The increased risk of VTE, accompanied by factor V Leiden, may be related to confounding environmental factors.     

Comparison of the effects of lornoxicam versus diclofenac in pain management after cardiac surgery: A single-blind, randomized, active-controlled study

Background:

Inadequate pain management after cardiac surgery may result 10 in increased morbidity and length of hospital stay. Although opioids are the mainstay of postoperative analgesia, nonsteroidal anti-inflammatory drugs (NSAIDs) may be used instead to avoid the adverse effects (AEs) associated with opioids. Lornoxicam is a newly developed NSAID, the use of which is increasing. However, lornoxicam has not been studied for use in pain management after cardiac surgery.

Objective:

The objective of this study was to compare the efficacy and tolerability 10 of lornoxicam and diclofenac sodium, an NSAID well established for use in pain management after major surgery, in pain management after coronary artery bypass grafting (CABG).

Methods:

This single-blind, randomized, active-controlled study was conducted 10 at the Gaziantep University Hospital, Gaziantep, Turkey. Adult patients scheduled to undergo valve or CABG surgery for the first time were included. Patients were premedicated with diazepam 10 mg PO at 10 PM on the evening before surgery. General anesthesia was induced using fentanyl, midazolam, and propofol, and maintained using fentanyl and isoflurane in pure oxygen. After extubation and when they stated that they felt pain, patients were randomly assigned to 1 of 2 treatment groups: lornoxicam 8 mg IM q8h or diclofenac 75 mg IM q12h, for 48 hours. Meperidine 1 mg/kg IM was given for additional analgesia when needed (rescue medication). Pain relief was assessed using an I1-point visual analog scale (0 = no pain to 10 = worst pain imaginable) immediately before the first injection (baseline), and at 15 and 30 minutes and 1, 2, 3, 4, 6, 12, 18, 24, and 48 hours after the first injection. Sedation was assessed using a 5-point scale (0 = awake and alert to 4 = deep sedation) at the same time points. Tolerability was assessed by monitoring of AEs using patient interview and laboratory analyses.

Results:

Forty patients were enrolled in the study (30 men, 10 women; 10 mean [SD] age, 54.4 [11.1 ] years; 20 patients per treatment group). The demographic and clinical characteristics and mean baseline pain relief scores were statistically similar between the 2 treatment groups. The mean pain relief scores at 15 and 30 minutes were statistically similar to baseline values in the 2 treatment groups. However, the mean pain relief scores at ≥1 hour after the first injection were significantly lower compared with baseline values (both groups, P < 0.05 at time points ≥1 hour). No significant between-group differences in mean pain relief scores were found at any time point. The overall mean pain relief scores were statistically similar between the 2 treatment groups. The mean sedation scores were significantly higher at 30 minutes, 1 hour, and 2 hours after the first injection in the diclofenac group compared with the lornoxicam group (all, P < 0.05). No AEs were observed. The need for rescue medication was statistically similar between the 2 treatment groups (lornoxicam, 2 patients; diclofenac, 3 patients).

Conclusions:

In this study of adult patients who underwent CABG, the efficacy 10 of lornoxicam and diclofenac were similar in postoperative pain management. Both study drugs were well tolerated.