Evaluation of diagnosis and treatment results in children with Graves' disease with emphasis on the pubertal status of patients

This retrospective study evaluated the clinical and laboratory characteristics at presentation and treatment results of patients with Graves' disease (GD) with respect to pubertal status. Records of 143 patients (108 F, 35 M) were reviewed in a multicenter study. At diagnosis, 38% of patients were prepubertal. Anti-thyroid drugs (ATD) were used as initial therapy. There was no significant difference in clinical and laboratory characteristics at diagnosis, during treatment and adverse reaction to ATD with respect to pubertal status. Twenty patients (7 prepubertal, 13 pubertal) reached remission on ATD. Surgery was performed in seven and radioiodine (RAI) in four patients. Duration of treatment needed to achieve remission was longer in prepubertal (4.2 +/- 1.0 yr) than in pubertal patients (3.1 +/- 1.3 yr) (p = 0.02). The rate of remission was not different between prepubertal (25.9%) and pubertal patients (33.3%) (p = 0.59). ATD were associated with low remission rate in pediatric GD and required longer duration of therapy in prepubertal patients. For definitive treatment in older children, RAI could be evaluated as the initial therapy.     

A Novel Pressure Film Approach for Determining the Force Imparted by Clear Removable Thermoplastic Appliances

The force imparted by removable thermoplastic appliances (RTA) onto teeth has not been investigated in the past. This investigation was designed to explore a novel methodology to measure the magnitude and identify the pattern of this force. Eight patients with moderate malocclusion were selected. In each patient, the palatally mal-positioned upper first premolar was corrected by wearing a series of four ClearSmile RTA over a duration of 8 weeks. When constructing RTA, the ClearSmile Company was advised that the amount of movement to be programmed into each appliance was 0.5 mm. The Pressurex film was used to measure the pressure generated by ClearSmile RTA against the palatal surface of the upper first premolar for buccal tipping movement. Three measurements were conducted respectively upon the issue and retrieval of each appliance (after 2 weeks of wear), resulting in 24 pressure measurements for each patient. Digital imaging and spectrophotometry analysis were employed to quantify the stain intensity mounted by the pressure on the films. The irrelevant forces were subtracted out to allow an assessment of the force purely acting to buccally repositioning the tooth. The results revealed that (1) the mean force magnitude over 2 weeks of RTA wear was 1.12 N (SE = 0.72 N); (2) the higher force magnitude of 5.12 N (SE = 0.80 N) seen at the issue of the appliance declined drastically to -2.67 N at the time of retrieval. These findings suggest that ClearSmile RTA exerts a high level of force against the tooth to be moved at the initial stage followed by a rapid force diminish.     

Non-resective management of pineoblastoma.

The results of a non-resective treatment approach for pineoblastoma comprising stereotactic biopsy, cerebrospinal fluid diversion, and fractionated radiotherapy in six patients over a period of six years are presented. There were three male and three female patients, with a median age at diagnosis of 20 years. Magnetic resonance imaging of the spine, ventricular cerebrospinal fluid cytology, and tumour markers in cerebrospinal fluid were negative. Tumour response to initial radiotherapy was complete in three patients and partial in three patients. Recurrences were treated with interstitial irradiation with iodine-125 seeds in four instances, repeat radiotherapy when time elapsed was more than five years in one instance, with surgical resection in two instances, and chemotherapy in two instances. The diagnostic and therapeutic effectiveness of this management strategy is assessed. There were no complications related to surgical procedures. The median follow-up time was 48 months (range 14-70 months). Five patients were alive at 14, 45, 51, 57, and 70 months of follow-up. One patient died of disease at 28 months following diagnosis. The overall survival rate was 80 % +/- 17.89 % at 28 months. The results of this study suggest that this non-resective treatment approach is acceptable as an initial treatment alternative to radical surgical resection of pineoblastomas.     

Netherton Syndrome Associated with Growth Hormone Deficiency

Netherton syndrome (NS) is a rare autosomal recessive disorder characterized by ichthyosiform scaling, hair abnormalities, and variable atopic features. Mutations in the serine protease inhibitor Kazal type 5 (SPINK5) gene leading to lymphoepithelial Kazal‐type‐related inhibitor (LEKTI) deficiency cause NS. Growth retardation is a classic feature of NS, but growth hormone (GH) deficiency with subsequent response to GH therapy is not documented in the literature. It is proposed that a lack of inhibition of proteases due to a deficiency of LEKTI in the pituitary gland leads to the overprocessing of human GH in NS. Herein we report three patients with NS who had growth retardation associated with GH deficiency and responded well to GH therapy.     

Heterogeneity of X-linked recessive (spino)cerebellar ataxia with or without spastic diplegia

Ataxia with spastic diplegia was seen in seven males of a Turkish family, obviously transmitted as an X-linked recessive trait. The first clinical sign in infancy was nystagmus; ataxia and pyramidal signs were noted at age 2-3 years. Patients were never able to walk. Dysarthria, orthopedic impairment, and mild mental retardation appeared later as the disorder progressed. Death occurred in the 3rd or 4th decade from infectious diseases. The syndrome resembles X-linked spinocerebellar ataxia and X-linked spastic paraplegia in some aspects but is different if compared with previously published reports. Laboratory and neurophysiological studies showed no abnormalities. Various aspects of X-linked ataxia are discussed: genetic heterogeneity is apparent from observations reported.     

Agmatine Reverses Sub‐chronic Stress induced Nod‐like Receptor Protein 3 (NLRP3) Activation and Cytokine Response in Rats

The activation of Nod‐like receptor protein 3 (NLRP3) has lately been implicated in stress and depression as an initiator mechanism required for the production of interleukin (IL)‐1β and IL‐18. Agmatine, an endogenous polyamine widely distributed in mammalian brain, is a novel neurotransmitter/neuromodulator, with antistress, anxiolytic and antidepressant‐like effects. In this study, we examined the effect of exogenously administered agmatine on NLRP3 inflammasome pathway/cytokine responses in rats exposed to restraint stress for 7 days. The rats were divided into three groups: stress, stress+agmatine (40 mg/kg; i.p.) and control groups. Agmatine significantly down‐regulated the gene expressions of all stress‐induced NLRP3 inflammasome components (NLRP3, NF‐κB, PYCARD, caspase‐1, IL‐1β and IL‐18) in the hippocampus and prefrontal cortex (PFC) and reduced pro‐inflammatory cytokine levels not only in both brain regions, but also in serum. Stress‐reduced levels of IL‐4 and IL‐10, two major anti‐inflammatory cytokines, were restored back to normal by agmatine treatment in the PFC. The findings of the present study suggest that stress‐activated NLRP3 inflammasome and cytokine responses are reversed by an acute administration of agmatine. Whether antidepressant‐like effect of agmatine can somehow, at least partially, be mediated by the inhibition of NLRP3 inflammasome cascade and relevant inflammatory responses requires further studies in animal models of depression.     

In vitro inhibition of caries-like lesions with fluoride-releasing materials

The aim of this study was to compare the in vitro caries inhibition of various resin-based materials. Class V cavities were prepared in twenty-five freshly extracted human premolar teeth which were then restored with glass-ionomer cement (Chemfill II), compomer (Compoglass F, Dyract AP) and composite resin (Tetric Ceram and Z 100). The teeth were submerged in an acid gel for 6 weeks. Each specimen was sectioned. These sections were left in water for 24 hours, and then examined using polarized light microscopy. The lesion consisted of two parts, the outer surface lesion and the cavity wall lesion. There was no significant difference in the body depth of the outer lesion and in the depth of the wall lesion among teeth restored with Compoglass F, Dyract AP and Chemfill II (P > 0.05). There was a significant difference between those restored with Z 100 and Tetric Ceram (P < 0.05). The length of the wall lesion for the teeth restored with Chemfill II was significantly smaller than that in the remaining groups (P < 0.05). The length of the wall lesion for teeth restored with Tetric Ceram and Z 100 was significantly higher than in the remaining groups (P < 0.05). These results suggest that composite materials and compomer provide less caries inhibition than glass-ionomer cements.     

The biomechanical relationship between incisor and condylar guidances in deep bite and normal cases

The purpose of this study was to investigate the biomechanical relationship between the condylar and anterior guidances in deep bite malocclusion and control groups. The subjects consisted of 18 deep bite patients and 14 normal occlusions. A four-bar mechanism was described on the lateral cephalogram films and a computer program, for which the lengths of the links measured on the films were the inputs, was developed. The computer program was used to calculate the angles of rotation of the mandible and the condyle during contact of the mandibular anterior teeth with the lingual surface of the maxillary anterior teeth as the mandible moves forward. Comparison of the mean values of the angles of rotation of the mandible and condyle in the two groups has shown that the change in the angle of rotation of the condyle is statistically significant (P < 0.05). The angles of rotation of condyle in deep bite group were found larger than the angles of normal group. Correlation between the angles of rotation of the condyle and mandible has shown that they were significantly related. The cause of temporomandibular disorders may be attributed to the large angle of rotation of the condyle in deep incisal overbite.     

Relationship between arm span and height in postmenopausal osteoporotic women

Osteoporosis is a progressive systemic skeletal disease characterized by low bone density and microarchitectural deterioration of the bone. A minimum 3-cm difference between arm span and height makes up one of the criteria for suspecting osteoporosis. Therefore, it is easy to determine osteoporotic women by measuring the proportion of height to the arm span. The purposes of this study are to assess the relationship between arm span and height and to compare them in postmenopausal and young healthy women. This was a randomized-controlled, prospective study. There were two groups in this study. Group I included 70 postmenopausal osteoporotic women and group II had 70 healthy young women. Height, weight and arm span of the individuals were measured in all subjects. Bone mineral density and radiological examination of spine were also evaluated. Mean age of postmenopausal women was 64.4 ± 8.6 years and it was 27.3 ± 3.5 years in young healthy women. Mean height was 152 ± 5.1 and 161.5 ± 5.9 cm in group I and II, respectively. Mean arm span length was 159.6 ± 6.3 cm in postmenopausal women and 163.5 ± 6 cm in young healthy women. Mean arm span-height difference was significantly higher in postmenopausal women when compared to healthy young women (7.7 ± 3.6 and 2 ± 2.9 cm, respectively, P < 0.001). We suggest that arm span measurements can be used in the estimation of youth height and age-related loss in stature in postmenopausal women.