Methylmalonic acidemia mimicking diabetic ketoacidosis in an infant

Methylmalonic acidemia (MMA) is an inherited organic acidemia usually present with recurrent episodes of acute illness. A typical episode is ushered in with ketonuria and vomiting, followed by acidosis, dehydration, and lethargy, leading, in the absence of aggressive treatment, to coma and death. We report an infant with MMA presented with diabetes symptoms. A 13-month-old girl complained of polydipsia, diuresis, and loss of weight. She had clinical signs of diabetic ketoacidosis such as dehydration, deep sighing respiration, smell of ketones, lethargy, and vomiting. Laboratory analysis showed hyperglycemia with acidosis and ketonuria. She was treated with parenteral fluid, electrolyte, and insulin infusion. Two days after her discharge, after having a meal rich in protein, she was brought unconscious with hepatomegaly, severe acidosis, ketonuria, and mild hyperammonemia. The absence of hyperglycemia and the presence of neurologic findings suggested organic acidemia. MMA was diagnosed because of methylmalonic aciduria and elevated C3 carnitine esters. Cranial magnetic resonance imaging (MRI) showed increased uptake of radiocontrast material in the basal ganglia bilaterally. A homozygous mutation in exon 4 of the MMAA gene was found in mutation analysis and confirmed the diagnosis of cblA-deficient MMA. Neurologic regression was improved with treatment of low-protein diet, vitamin B12, and l-carnitine. In patients born to consanguineous parents who admit during infancy with severe acidosis refractory to treatment, organic acidemias should be kept in mind, even they have high blood glucose. The definitive diagnosis is important because it may allow a specific treatment and a favorable evolution to prevent the sequelae.     

Evaluation of bone metabolism and bone mass in patients with type-2 diabetes mellitus

The objectives of this study were to determine whether type-2 diabetes was associated with a higher bone mineral density (BMD) in men and women and to evaluate the differences in mineral metabolism between diabetic and normal subjects by using biochemical bone turnover markers. In this study, 52 patients (37 females/15 males) aged 41-64 with type-2 diabetes mellitus and 48 nondiabetic control subjects (34 females/14 males) were evaluated. In men, BMD was significantly higher in diabetics at the forearm (p <0.05), whereas in women tended to be higher at the hip (p=0.002). Serum osteocalcin (p<0.0001), bone alkaline phosphatase (BAP) (p<0.05) and carboxyterminal telopeptide (CTx) (p<0.05) were higher in the control group than in diabetics. In men, serum osteocalcin (p<0.05) and CTx (p<0.005) and, in women, serum osteocalcin (p<0.0001) and BAP (p<0.05) were lower in diabetic subjects. In conclusion, our findings suggest that although bone formation is decreased in type-2 diabetes, diabetic patients are not susceptible to bone resorption. This low bone turnover can slow the rate of bone loss and cause a higher bone density than expected for their age.     

Protective effect of melatonin on beta-cell damage in streptozotocin-induced diabetes in rats

The aim of the present study was the evaluation of possible protective effects of melatonin against beta-cell damage in streptozotocin-induced diabetes in rats. Malondialdehyde levels and glutathione peroxidase activity were measured in pancreatic homogenates. Pancreatic beta-cells were examined by immunohistochemical methods. Streptozotocin was injected intraperitoneally at a single dose of 60 mg/kg for induction of diabetes. Melatonin (200 microg/kg/day, ip) was injected for 3 days prior to administration of streptozotocin; these injections were continued until the end of the study (4 weeks). Streptozotocin induced a significant increase in malondialdehyde levels (p < 0.01) and a significant decrease in glutathione peroxidase activity (p < 0.05) in pancreatic tissue. Degeneration of islet cells and weak immunohistochemical staining of insulin was observed in diabetic rats. Treatment of diabetic rats with melatonin markedly reduced malondialdehyde production (p < 0.05) and increased glutathione peroxidase activity (p < 0.01) without affecting hyperglycemia. Increased staining of insulin and preservation of islet cells were apparent in the melatonin-treated diabetic rats. These data suggest that melatonin treatment has a therapeutic effect in diabetes by reduction of oxidative stress and preservation of pancreatic beta-cell integrity.     

Analysis of NKX3.1 expression in prostate cancer tissues and correlation with clinicopathologic features

This study aimed to analyze NKX3.1 expression in tissue samples of benign prostate hyperplasia (BPH) and in three different prostate cancer categories. The correlation of NKX3.1 expression with clinical and pathologic features of patients having undergone radical prostatectomy also was investigated. NKX3.1 expression was determined in tissue samples obtained from four different histopathological categories: (1) from patients treated with transurethral prostatectomy for BPH (n = 26), (2) localized prostate cancer patients subjected to radical prostatectomy (n = 38), (3) biopsy samples from prostate cancer patients who were metastatic at the initial admission (n = 10), and (4) tissue samples of prostate cancer patients administered antiandrogens, but who had undergone transurethral prostatectomy for infravesical obstruction (n = 11). Standard immunohistochemical staining was performed using an antiserum raised against recombinant human NKX3.1. Staining was seen in all categories of prostatic tissues. Immunohistochemistry staining scores were lower in prostate cancer patients. The staining scores were significantly higher in patients with BPH compared to metastatic or localized prostate cancer patients. Staining scores of patients with BPH and of those under antiandrogen therapy were similar. No significant correlation was found between NKX3.1 expression and tumor volume, Gleason sum scores, the presence of extraprostatic extension, tumor stage, or serum PSA. NKX3.1 expression is significantly decreased in prostate cancer patients when compared to BPH. However, the decline of NKX3.1 expression was not correlated with prostate cancer progression and was not associated with advanced stage. Thus, NKX3.1 expression is not a clinically valuable prognostic factor.     

EXAMINATION OF GUTTA-PERCHA CONES FOR MICROBIAL CONTAMINATION DURING CHEMICAL USE

Objective:

The aim of this study was to evaluate the degree of microbial contamination in packaged gutta-percha cones before and during use in clinical conditions.

Material and Methods:

Sealed packages of #15-40 gutta-percha cones were opened under aseptic laboratory conditions. Two gutta-percha cones from each size were randomly drawn and added to tubes containing glass beads and 750 μL of saline. The tubes were vortexed, serially diluted and samples of 250 μL were cultured on agar plates. The plates were incubated at 37°C for 3 days and colonies were counted. The initially sampled packages were distributed to 12 final year dental students. The packages were collected at the end of the first and the third clinical practice days and sampled as described above.

Results:

Baseline microbial counts did not exceed 3 CFU. At the end of the first and the third day, additional contamination was found in five and three of the packages, respectively. The ratio of contaminated packages at the first day and the third day was not significantly different (z-test; p > 0.05). The numbers of microorganisms cultured at the first day (8 ± 9.9 CFU) and the third day (4.5 ± 8.3 CFU) were not significantly different (Wilcoxon signed-rank test; p > 0.05). No significant correlation was found between the number of filled root canals and cultured microorganisms at either the first day (Spearman''s rho; r = 0.481, p = 0.113) or the third day (r = -0.034, p = 0.917).

Conclusions:

Gutta-percha cones taken directly from manufacturer''s sealed package harbored microorganisms. Clinical use of the packages has been found to be associated with additional contamination of the gutta-percha cones. The counts of cultured microorganisms did not correlate well with the number of filled root canals.     

Cardiac multidetector computed tomography (MDCT) of spontaneously closed ventricular septal defect

Purpose  

The authors present the findings of contrastenhanced electrocardiogram (ECG)-gated cardiac computed tomography (CT) in 18 patients with probably spontaneous closure of muscular ventricular septal defect (VSD).     

Sealer Penetration into Dentinal Tubules in the Presence or Absence of Smear Layer: A Confocal Laser Scanning Microscopic Study

Introduction

The aim of this study was to test the dentinal tubule penetration of AH26 (Dentsply DeTrey, Konstanz, Germany) and MTA Fillapex (Angelus, Londrina, PR, Brazil) in instrumented root canals obturated by using cold lateral compaction or warm vertical compaction techniques in either the presence or absence of the smear layer.

Methods

Forty-five extracted single-rooted human mandibular premolar teeth were used. The crowns were removed, and the root canals were instrumented by using the Self-Adjusting File (ReDent-Nova, Ra'anana, Israel) with continuous sodium hypochlorite (2.6%) irrigation. Final irrigation was either with 5% EDTA or with sodium hypochlorite. The canals were dried and obturated by using rhodamine B–labeled AH26 or MTA Fillapex in combination with the cold lateral compaction or the warm vertical compaction technique. After setting, the roots were sectioned horizontally at 4-, 8-, and 12-mm distances from the apical tip. On each section, sealer penetration in the dentinal tubules was measured by using confocal laser scanning microscopy.

Results

Regardless of the usage of EDTA, MTA Fillapex, compared with AH26, was associated with greater sealer penetration when used with the cold lateral compaction technique, and, conversely, AH26, compared with MTA Fillapex, was associated with greater sealer penetration when used with the warm vertical compaction technique (P < .05). Removal of the smear layer increased the penetration depth of MTA Fillapex used with the cold lateral compaction technique (P < .05); however, it had no significant effect on the penetration depth of AH26.

Conclusions

Greater sealer penetration could be achieved with either the MTA Fillapex–cold lateral compaction combination or with the AH26–warm vertical compaction combination. Smear layer removal was critical for the penetration of MTA Fillapex; however, the same did not hold for AH26.     

Novel Application of Internal-External Drainage Catheter as Biliary Stent for Percutaneous Transhepatic Treatment of Biliary Strictures in Living Donor Liver Transplantation Recipient Patients

ObjectivesAlthough endoscopic management is considered as the first-line treatment for biliary strictures, it may be challenging in living donor liver transplant recipients due to the complex nature of duct-to-duct reconstruction. In this study we present the use of a pigtail drainage catheter as a biliary stent to treat biliary strictures after a living donor liver transplant.MethodsTwenty-seven patients with biliary strictures were treated with our novel technique. In this technique, a pigtail catheter was trimmed into 3 parts (proximal, middle, and distal portions). A suture string was passed through the distal hole of the middle portion, which was then reversed and used as a stent while the proximal portion was used as a pusher. Following balloon dilation of the stenotic segment, the distal, reversed middle, and proximal portions were loaded over the guidewire. After proper placement of the stent, the retractor suture string, pusher, and guidewire were removed. The stent was removed during the third or fourth month of placement through endoscopic retrograde cholangiopancreatography (ERCP) in all patients.ResultsNo significant complications developed during the procedure or follow-up period. Ten patients required re-stenting by ERCP during the same session. The mean follow-up period was 2 years. Cholestase enzymes and bilirubin levels were within normal limits in all patients during follow-up.ConclusionStents derived from drainage catheter facilitate treatment of biliary strictures in patients not eligible for the retrograde approach. This stent is cheap, easy to implement, can be easily removed by ERCP, and re-stenting can be applicable in retrograde if needed.