The aim of this study was to compare the efficacy of three forms of cleansing enema for rigid proctosigmoidoscopy. One hundred fifty five patients referred for proctosigmoidoscopy to the Department of Surgery at the FEU-NRMF Medical Center were randomly assigned by simple random sampling into three groups to receive three forms of cleansing enema: monobasic sodium phosphate dibasic sodium phosphate (Fleet Enema ) for Group 1 (n=42), sorbitol and dioctyl sulfosuccinate ( Clyss Go) for Group 2 (n=38) and soap sud enema (SS Enema) for Group 3 (n=45). Quality of bowel preparation was graded as good, fair or poor. The cleansing enema was administered one hour prior to the procedure. It was readministered in case the patient did not have bowel movements. Another dose of the designated enema was given if the bowel preparation was poor. The reinfusion rate for the SS enema group was only 17 percent, significantly lower than the 45 percent of the Fleet Enema group (p=0.01), but not statistically different from the 39 percent of the Clyss Go group (p=0.05). The cost-effective analysis using direct costs showed SS enema was more cost- effective than Fleet Enema or Clyss Go enema. The SS enema was a good alternative for cleansing the bowel prior to rigid proctosigmoidoscopy. It had a low reinfusion rate and was more cost-effective than Fleet enema or Clyss Go. (Author)

Human ; Male ; Female ; Sigmoidoscopy ; Sodium Phosphate ; Dioctyl Sulfosuccinic Acid ; Soaps ; Sorbitol ; Enema ; Phosphates ; Defecation ; Intestines

PiT2 is an inorganic phosphate (Pi) transporter whose mutations are linked to primary familial brain calcification (PFBC). PiT2 mainly consists of two ProDom (PD) domains and a large intracellular loop region (loop7). The PD domains are crucial for the Pi transport, but the role of PiT2-loop7 remains unclear. In PFBC patients, mutations in PiT2-loop7 are mainly nonsense or frameshift mutations that probably cause PFBC due to C-PD1131 deletion. To date, six missense mutations have been identified in PiT2-loop7; however, the mechanisms by which these mutations cause PFBC are poorly understood. Here, we found that the p.T390A and p.S434W mutations in PiT2-loop7 decreased the Pi transport activity and cell surface levels of PiT2. Furthermore, we showed that these two mutations attenuated its membrane localization by affecting adenosine monophosphate-activated protein kinase (AMPK)- or protein kinase B (AKT)-mediated PiT2 phosphorylation. In contrast, the p.S121C and p.S601W mutations in the PD domains did not affect PiT2 phosphorylation but rather impaired its substrate-binding abilities. These results suggested that missense mutations in PiT2-loop7 can cause Pi dyshomeostasis by affecting the phosphorylation-regulated cell-surface localization of PiT2. This study helps understand the pathogenesis of PFBC caused by PiT2-loop7 missense mutations and indicates that increasing the phosphorylation levels of PiT2-loop7 could be a promising strategy for developing PFBC therapies.
Humans ; Cell Membrane ; Mutation, Missense ; Phosphates/metabolism* ; Sodium-Phosphate Cotransporter Proteins, Type III/genetics*

The purpose of this study was to compare the retention of complete cast crown over amalgam cores, composite resin cores, and cast gold cores when cemented with three different luting agents. Eighteen core specimens each of amalgam(Bestaloy, Dong Myung, Seoul, Korea), composite resin (Z100, 3M Dental product, st. Paul, Minn) and type N gold alloy (Ba 4, Heesung Engelhard Corp., Korea) were made in a customized milling stainless steel die. A wax pattern with a loop attached to occlusal surface was made for each core and a type 11 gold alloy casting was fabricated. The castings which had clinically acceptable marginal fit were used as test samples. The following luting cements were used to cement cast crowns on each core material : (1) zinc phosphate cement (Confi dental Products Co., USA) (2) glass-ionomer cement (Fuji Plus, GC Industrial Corp., Tokyo, Japan) (3) resin cement (Panavia 21, Kuraray Co., USA). All cements were mixed according to manufacturers instructions. A static load of 5kg was then applied for 10 minutes on the crowns. All specimens were stored in saline solution for 24 hours at 371C and thermocycled for 500 cycles. After storage and cycling, the tensile bond strengths were measured by using a universal testing machine (Instron Corp., Canton, Mass.) at a crosshead speed of 0.5mm/min. The results were as follows : 1. The retentive strength of resin cement was the highest of all three types of cement for resin core (p<0.05). 2. There was no statistical difference among the retentive strengths of three cements for amalgam core (p>0.05). 3. The retentive strength of resin cement was higher than that of zinc phosphate for cast core, but there was no difference between the retentive strength of glass ionomer cement and those of rein and zinc phosphate cement. 4. The retentive strength of the zinc phosphate cement for amalgam core was the highest of all type of cores.
Alloys ; Crowns* ; Dental Cements ; Glass Ionomer Cements ; Resin Cements ; Seoul ; Sodium Chloride ; Stainless Steel ; Zinc ; Zinc Phosphate Cement

OBJECTIVE: To study and compare the effects of different demineralization-inhibition methods on the shear bond strength (SBS) and fracture mode of an adhesive used to bond orthodontic brackets to demineralized enamel surfaces. METHODS: Eighty freshly extracted, human maxillary premolars were divided into 4 equal groups and demineralized over the course of 21 days. Brackets were bonded to the demineralized enamel of teeth in Group 1. In Group 2, bonding was performed following resin infiltration (ICON(R), DMG, Hamburg, Germany). Before bonding, pre-treatment with acidulated phosphate fluoride (APF) or solutions containing casein phosphopeptide-amorphous calcium phosphate with 2% neutral sodium fluoride (CPP-ACP/wF) was performed in Groups 3 and 4, respectively. The SBS values of the brackets were measured and recorded following mechanical shearing of the bracket from the tooth surface. The adhesive remnant index (ARI) scores were determined after the brackets failed. Statistical comparisons were performed using one-way ANOVA, Tukey's post-tests, and G-tests. RESULTS: Significant differences were found in some of the intergroup comparisons of the SBS values (F = 39.287, p < 0.001). No significant differences were found between the values for the APF-gel and control groups, whereas significantly higher SBS values were recorded for the resin-infiltrated and CPP-ACP/wF-treated groups. The ARI scores were also significantly different among the 4 groups (p < 0.001). CONCLUSIONS: Tooth surfaces exposed to resin infiltration and CPP-ACP/wF application showed higher debonding forces than the untreated, demineralized surfaces.
Acidulated Phosphate Fluoride ; Adhesives ; Bicuspid ; Calcium ; Calcium Phosphates ; Caseins ; Dental Enamel ; Humans ; Oral Hygiene ; Orthodontic Brackets ; Sodium Fluoride ; Tooth

Proximal renal tubular acidosis (RTA) is caused by a defect in bicarbonate (HCO₃⁻) reabsorption in the kidney proximal convoluted tubule. It usually manifests as normal anion-gap metabolic acidosis due to HCO₃⁻ wastage. In a normal kidney, the thick ascending limb of Henle’s loop and more distal nephron segments reclaim all of the HCO₃⁻ not absorbed by the proximal tubule. Bicarbonate wastage seen in type II RTA indicates that the proximal tubular defect is severe enough to overwhelm the capacity for HCO₃⁻ reabsorption beyond the proximal tubule. Proximal RTA can occur as an isolated syndrome or with other impairments in proximal tubular functions under the spectrum of Fanconi syndrome. Fanconi syndrome, which is characterized by a defect in proximal tubular reabsorption of glucose, amino acids, uric acid, phosphate, and HCO₃⁻, can occur due to inherited or acquired causes. Primary inherited Fanconi syndrome is caused by a mutation in the sodium-phosphate cotransporter (NaPₐ-II) in the proximal tubule. Recent studies have identified new causes of Fanconi syndrome due to mutations in the EHHADH and the HNF4A genes. Fanconi syndrome can also be one of many manifestations of various inherited systemic diseases, such as cystinosis. Many of the acquired causes of Fanconi syndrome with or without proximal RTA are drug-induced, with the list of causative agents increasing as newer drugs are introduced for clinical use, mainly in the oncology field.
Acidosis ; Acidosis, Renal Tubular ; Amino Acids ; Cystinosis ; Extremities ; Fanconi Syndrome ; Glucose ; Kidney ; Nephrons ; Sodium-Phosphate Cotransporter Proteins ; Uric Acid

The serum phosphorus level is maintained through a complex interplay between intestinal absorption, exchange intracellular and bone storage pools, and renal tubular reabsorption. The kidney plays a major role in regulation of phosphorus homeostasis by renal tubular reabsorption. Type IIa and type IIc Na+/Pi transporters are important renal Na+-dependent inorganic phosphate (Pi) transporters, which are expressed in the brush border membrane of proximal tubular cells. Both are regulated by dietary Pi intake, vitamin D, fibroblast growth factor 23 (FGF23) and parathyroid hormone. The expression of type IIa Na+/Pi transporter result from hypophosphatemia quickly. However, type IIc appears to act more slowly. Physiological and pathophysiological alteration in renal Pi reabsorption are related to altered brush-border membrane expression/content of the type II Na+/Pi cotransporter. Many studies of genetic and acquired renal phosphate wasting disorders have led to the identification of novel genes. Two novel Pi regulating genes, PHEX and FGF23, play a role in the pathophysiology of genetic and acquired renal phosphate wasting disorders and studies are underway to define their mechanism on renal Pi regulation. In recent studies, sodium-hydrogen exchanger regulatory factor 1 (NHERF1) is reported as another new regulator for Pi reabsorption mechanism.
Fibroblast Growth Factors ; Homeostasis ; Hypophosphatemia ; Intestinal Absorption ; Kidney ; Membranes ; Microvilli ; Parathyroid Hormone ; Phosphoproteins ; Phosphorus ; Sodium-Hydrogen Antiporter ; Sodium-Phosphate Cotransporter Proteins ; Vitamin D

Basal Ganglia ; pathology ; Calcinosis ; complications ; Female ; Humans ; Middle Aged ; Mutation ; Sodium-Phosphate Cotransporter Proteins, Type III ; genetics ; Supranuclear Palsy, Progressive ; etiology

OTC deficiency is an X-linked disorder in which the synthesis of urea is impaired. OTC catalyzes the synthesis of citrulline from carbamyl phosphate and ornithine. Complete or partial deficiencies of this enzyme may lead to Reye syndrome like picture such as encephalopathy, hepatic dysfunction, hyperammonemia, etc. We recently had a case that was presented as recurrent Reye syndrome, and was effectively treated with hemodialysis, arginine, sodium benzoate, etc. This report describes an experience in treating this condition with review of available literature.
Arginine ; Carbamyl Phosphate ; Citrulline ; Hepatic Encephalopathy ; Hyperammonemia ; Ornithine Carbamoyltransferase Deficiency Disease ; Ornithine Carbamoyltransferase* ; Ornithine* ; Renal Dialysis ; Reye Syndrome ; Sodium Benzoate ; Urea

OBJECTIVETo describe clinical and genetic feature in a Chinese family with familial idiopathic basal ganglia calcification 3 (IBGC-3) caused by a novel mutation in the SLC20A2 gene.

METHODSClinical data was collected from a family with familial IBGC-3. All of the family members underwent cerebral CT. Potential mutation of the SLC20A2 gene were screened in the proband, 5 symptomatic patients, 5 asymptomatic family members, and 100 healthy Chinese controls. Exon 8 of the SLC20A2 gene was cloned into plasmid and sequenced.

RESULTSThere were 6 symptomatic patients (3 males and 3 females) in an autosomal dominant pedigree. The patients manifested as juvenile-onset paroxysmal kinesigenic dyskinesia, in addition to pyramidal signs in proband. 5 patients alive had calcification in bilateral basal ganglia and subcortical areas. One asymptomatic member also had calcification in the brain; and 2 cases of asymptomatic young members had bilateral globus pallidus calcification. A novel c.1086delC mutation in SLC20A2 gene has been identified in proband and 7 family members with intracranial calcification. The deletion mutation was not found in 2 family members without intracranial calcification and healthy controls members. There is no clear relationship between clinical symptoms and the severity of calcification in cerebral CT.

CONCLUSIONFamilial idiopathic basal ganglia calcification caused by the SLC20A2 gene mutation can manifest as juvenile onset paroxysmal kinesigenic dyskinesia. Further study should be done to validate the unrelated relationships between the severity of calcification in IBGC 3 cranial CT and clinical symptoms.

Adolescent ; Adult ; Basal Ganglia Diseases ; genetics ; Calcinosis ; genetics ; Child ; Female ; Humans ; Male ; Mutation ; Neurodegenerative Diseases ; genetics ; Sodium-Phosphate Cotransporter Proteins, Type III ; genetics ; Tomography, X-Ray Computed

The aim of this study was to compare fluoride release and surface changes according to different orthodontic bracket adhesives the application of fluoride products. We used non-fluoridated composite resin Transbond fluoridated composite resins Blugloo and LightBond, resin-modified glass ionomer Rely XTM Luting 2, and conventional glass ionomer Fuji I®. Fluoride release of five orthodontic bracket adhesives and fluoride release ability after application of three fluoride products (1.23% acidulated phosphate fluoride gel, Tooth Mousse Plus®, Fluor Protector, and a toothbrush with sodium fluoride-containing toothpaste) were measured using a fluoride electrode that was connected to an ion analyzer. After 4 weeks of fluoride application, the surface roughness and surface morphology were examined using a surface roughness tester and field emission scanning electron microscopy. The amounts of fluoride release were observed not only on application of Tooth Mousse Plus® and Fluor Protector on resin-modified glass ionomer Rely XTM Luting 2 and Fuji I®, but also during tooth brushing using fluoride-containing toothpaste. After application of Tooth Mousse Plus®, except Transbond XT, the surface roughness increased, and all orthodontic adhesives showed a partial drop of micro-particle filler. On application of 1.23% acidulated phosphate fluoride gel on all orthodontic bracket adhesives, their surface roughness increased. To bond the orthodontic bracket, resin-modified glass ionomer Rely XTM Luting 2 and Fuji I® adhesives are highly recommended if the amount of fluoride release is considered to confer a preventative effect on dental caries, and among the fluoride products, Tooth Mousse Plus® and Fluor Protector are better than 1.23% acidulated phosphate fluoride gel, and these are expected to prevent dental caries even during tooth brushing with fluoride-containing toothpaste.
Acidulated Phosphate Fluoride ; Adhesives* ; Composite Resins ; Dental Caries ; Dental Cements ; Electrodes ; Fluorides* ; Glass ; Microscopy, Electron, Scanning ; Orthodontic Brackets* ; Sodium ; Surface Properties* ; Tooth ; Toothpastes