Objectives: Phytic acid (IP6), a naturally occurring agent, has been previously reported as a potential alternative to ethylenediaminetetraacetic acid (EDTA). However, its effect on adhesion to sodium hypochlorite (NaOCl)-treated dentin and its interactions with NaOCl have not been previously reported. Thus, in this study, the effects of IP6 on resin adhesion to NaOCl-treated dentin and the failure mode were investigated and the interactions between the used agents were analyzed. Materials and Methods: Micro-tensile bond strength (µTBS) testing was performed until failure on dentin treated with either distilled water (control), 5% NaOCl, or 5% NaOCl followed with chelators: 17% EDTA for 1 minute or 1% IP6 for 30 seconds or 1 minute. The failed specimens were assessed under a scanning electron microscope. The reaction of NaOCl with EDTA or IP6 was analyzed in terms of temperature, pH, effervescence, and chlorine odor, and the effects of the resulting mixtures on the color of a stained paper were recorded. Results: The µTBS values of the control and NaOCl with chelator groups were not significantly different, but were all significantly higher than that of the group treated with NaOCl only. In the failure analysis, a distinctive feature was the presence of resin tags in samples conditioned with IP6 after treatment with NaOCl. The reaction of 1% IP6 with 5% NaOCl was less aggressive than the reaction of the latter with 17% EDTA. Conclusions IP6 reversed the adverse effects of NaOCl on resin-dentin adhesion without the chlorine-depleting effect of EDTA.

Objectives: Phytic acid (IP6), a naturally occurring agent, has been previously reported as a potential alternative to ethylenediaminetetraacetic acid (EDTA). However, its effect on adhesion to sodium hypochlorite (NaOCl)-treated dentin and its interactions with NaOCl have not been previously reported. Thus, in this study, the effects of IP6 on resin adhesion to NaOCl-treated dentin and the failure mode were investigated and the interactions between the used agents were analyzed. Materials and Methods: Micro-tensile bond strength (µTBS) testing was performed until failure on dentin treated with either distilled water (control), 5% NaOCl, or 5% NaOCl followed with chelators: 17% EDTA for 1 minute or 1% IP6 for 30 seconds or 1 minute. The failed specimens were assessed under a scanning electron microscope. The reaction of NaOCl with EDTA or IP6 was analyzed in terms of temperature, pH, effervescence, and chlorine odor, and the effects of the resulting mixtures on the color of a stained paper were recorded. Results: The µTBS values of the control and NaOCl with chelator groups were not significantly different, but were all significantly higher than that of the group treated with NaOCl only. In the failure analysis, a distinctive feature was the presence of resin tags in samples conditioned with IP6 after treatment with NaOCl. The reaction of 1% IP6 with 5% NaOCl was less aggressive than the reaction of the latter with 17% EDTA. Conclusions IP6 reversed the adverse effects of NaOCl on resin-dentin adhesion without the chlorine-depleting effect of EDTA.

OBJECTIVETo investigate the clinical and neurodevelopmental profiles of patients with biotinidase deficiency and to determine the efficacy of current therapy with respect to outcome.

METHODSSix patients aged from 3 months to 14 years with biotinidase deficiency were confirmed by urinary organic acid analysis with gas chromatography/mass spectrometry (GC/MS) and biotinidase assay on dried blood spots. Biotin was supplemented individually (10-40 mg/d). Their clinical features, laboratory findings, and treatment regimen were reviewed.

RESULTSAll the 6 patients presented with some extent of neurological abnormalities and dermatological lesions. Cases 1 - 3 had poor feeding, vomiting, seizures, mental retardation, and lethargy onset from their early infancy, with varied degree of anemia, ketosis, acidosis, and hypoglycemia. Case 2 exhibited eczema and dermatitis from his age of 7 months. Case 4 displayed motor deficit and ataxia after 6 months of age, and generalized pustular psoriasis when he was 8 months old. Cases 5 and 6 gradually showed muscle weakness and paraplegia at the age of 7 years and 5 years, respectively. Inflammatory demyelination changes of cervical cord were evident on magnetic resonance imaging in these two patients. Case 6 had progressive optic atrophy, eczema and alopecia. Remarkable elevations of urinary lactate, pyruvate, 3-OH-propionate, methylcitrate, propionylglycine, 3-OH-isovalerate, 3-methylcrontonylglycine were confirmed in cases 1, 2, 3 and 5. Slight increase of urinary lactate, pyruvate, and 3-methylcrontonylglycine was observed in cases 4 and 6. Biotinidase activities assayed on dried blood spots from all the patients were below 0.1 pmol/(min.3 mm) Biotin supplementation for all the patients, except for case 3 who was not treated, resulted in pronounced and rapid clinical and biochemical improvement. Cases 4 and 6 had residual neurological damage comprising ataxia and motor handicap of legs, due to prolonged disease course.

CONCLUSIONSBiotinidase deficiency intensively impairs nervous system and skin in the affected patients. Urinary organic acid analysis and blood biotinidase assay are crucial to the diagnosis. Early diagnosis and biotin supplementation can contribute significantly to the improvement of prognosis.

Adolescent ; Biotin ; administration & dosage ; therapeutic use ; Biotinidase Deficiency ; diagnosis ; drug therapy ; urine ; Child ; Child, Preschool ; Gas Chromatography-Mass Spectrometry ; Humans ; Infant ; Male ; Treatment Outcome