"Double eyes” sign of congenital bilateral dacryocystoceles

Dacryocystocele is caused by nasolacrimal duct obstruction and results in cystic dilatation of the proximal part of the nasolacrimal duct, which is located inferomedial to the orbit, leading to fluid accumulation. It is important to consider that persistent congenital bilateral dacryocystoceles may cause neonatal nasal obstruction resulting in respiratory difficulty, and large dacryocystoceles may require surgical drainage. Ultrasonography demonstrates that congenital bilateral dacryocystoceles and normal eyeballs prenatally resemble two pairs of cystic "lesions” of different sizes. We herein present a case of prenatally diagnosed isolated congenital bilateral dacryocystoceles and propose the new name of "double eyes” sign for this rare condition to create an impact on medical students and resident

タンノウ テキシュツジュツ デ チュウイ スベキ タンカン ソウコウ イジョウ Cystohepatic duct ノ 1レイ

　A 74-year-old man presented with epigastralgia and was diagnosed as having cholelithiasis. Endoscopic retrograde cholangiopancreatography (ERCP) initially visualized the cystic duct with the Heister valve from the common bile duct, and then two intra-hepatic biliary ducts of segment 5 (B5) were visualized from the neck of the gallbladder. There was a contrast medium filling defect in B5, which was considered to be due to an incarcerated stone. Magnetic resonance cholangiopancreatography (MRCP) and three-dimensional computed tomography (CT) cholangiography showed similar findings, suggesting that the patient had a biliary anomaly of the cystohepatic duct in which two intrahepatic bile ducts (B5) flowed into the neck of the gallbladder and a stone incarcerated in the neck of the gallbladder.　At surgery, during mobilization of the gallbladder, there was a thick string between the liver and the gallbladder, and this was considered to be the junction of B5 with the gallbladder. Therefore, the neck of the gallbladder was cut, and an incarcerated stone 10 mm in diameter was removed. Intraoperative cholangiography revealed that the cystohepatic ducts were preserved. The postoperative course was uneventful and there was no bile leakage or liver dysfunction.　Although cystohepatic duct is a rare biliary anomaly, the surgeon should be alert for its possible presence during cholecystectomy. When cholecystectomy is scheduled, more than one preoperative examination by three-dimensional CT cholangiography, MRCP or ERCP should be performed, and the surgeon should be careful not to overlook any biliary anomaly.　Keywords: cystohepatic duct, biliary duct anomaly, cholecystectomy, three-dimensional computed tomography, gallston

Direct Extraction of Polysaccharides from Moso Bamboo (Phylostachys heterocycla) Chips Using a Mixed Solvent System of an Amino Acid Ionic Liquid with Polar Aprotic Solvent

The cellulose-dissolving ability and some physical properties of mixed solvents of an amino acid IL, N-methyl-N-(2-methoxyethyl)pyrolidin-1-ium 2,6-diaminohexanoate ([P1ME][Lys]), with polar aprotic solvents, such as 1,3-dimethylimidazolidinone (DMI), N,N-dimethylformamide (DMF), dimethylsulfoxide (DMSO), and acetonitrile (CH3CN), have been investigated. The viscosity was significantly reduced by the increasing content of polar aprotic solvents, and a 1:1 mixture (molar ratio) of [P1ME][Lys] with DMF showed 91.5 cP which corresponded to less than 1/10 compared to that of the pure IL at 25 °C (1058 cP). The β values of the mixed solvents, which have the IL contents over 0.1, exhibited β-values similar to that of the pure IL. On the other hand, the π-value was dependent on the ratio of the IL content, and the pure IL had the highest π-value. We found that the mixed solvent of [P1ME][Lys] with DMF (1:1) easily dissolved the cellulose and the mixed solvent could be used to extract cellulose from moso bamboo (Phylostachys heterocycla) powder. The efficiency of the extraction of cellulose from the bamboo powder was significantly increased when a 1:1 mixture of the IL with a polar aprotic solvent was used as the extracting solvent at 60 °C; the extraction ratio of the 1:1 mixture (IL: DMF) reached twice that of the pure IL. We thus obtained cellulose in 18% (w/w) yield from the bamboo powder

A New X-Linked Mental Retardation Syndrome with Diplegia and Delayed Myelination

We report three boys (4, 6 and 8 years old) in a Japanese family with X-linked (XL) recessive severe mental retardation (MR), rigidospastic diplegia, mild athetotic movement of the upper limbs, delayed myelination and poor weight gain. Neurological manifestations were non-progressive. No deterioration of development, convulsion, cerebellar signs, dysarthria, pseudobulbar signs, or minor anomalies including facial dysmorphism or macro-orchidism were found. Ocular fundus was normal. The patients' mothers and one grandmother were clinically normal. Blood chemistry was within normal limits. Serum anti-human T-cell leukemia virus-I antibody titer was negative. Levels of plasma amino acids and serum very long chain fatty acids, and lysozomal enzyme activities from leukocytes were normal. Brain magnetic resonance imaging scans showed delayed myelination. Fragile X (FRAXA), fragile XE (FRAXE), proteolipid protein and L1 cell adhesion molecule (L1CAM) genes were normal. These findings were not consistent with previously reported 13 XLMR syndromes with paralysis. We conclude that this condition is a distinct and previously undescribed XLMR syndrome

Diagnostic value of serum EBV-DNA quantification and antibody to viral capsid antigen in nasopharyngeal carcinoma patients

医薬保健研究域医学系We compared the amount of serum Epstein-Barr virus DNA (EBV-DNA) detected in patients with nasopharyngeal carcinoma (NPC) in a high-incidence area, represented by Taiwan, and a low-incidence area, represented by Japan, using real-time quantitative PCR. The median serum EBV-DNA value in 41 Japanese NPC cases was 5450 copies/ml, and that in in 23 Taiwanese cases was 2125 copies/ml. The median serum EBV-DNA value in all 64 NPC cases was significantly higher than in control groups. Using receiver-operating-characteristic (ROC) curves, the sensitivity and specificity of EBV-DNA quantification were determined (cut-off point, 6.87 copies/ml; sensitivity, 0.855; specificity, 0.885) and compared with those of EBV-viral-capsid-antigen (VCA) titers; the results showed that EBV-DNA was a more sensitive and specific parameter than EBV-VCA titer. Then, we analyzed 19 NPC patients in whom recurrence developed (11 Japanese and 8 Taiwanese), and 26 NPC patients in continuous remission. Although there was no significant difference in EBV-DNA values between Japanese and Taiwanese patients, the value was significantly higher in the 19 patients with recurrence than in those in remission. ROC analysis again revealed a higher diagnostic value of EBV-DNA than EBV-VCA. These results suggest EBV-DNA is a more reliable tumor marker than EBV-VCA in both high-incidence and low-incidence areas of NPC

Pyridoxal in the Cerebrospinal Fluid May Be a Better Indicator of Vitamin B6–dependent Epilepsy Than Pyridoxal 5′-Phosphate

Background We aimed to demonstrate the biochemical characteristics of vitamin B6–dependent epilepsy, with a particular focus on pyridoxal 5′-phosphate and pyridoxal in the cerebrospinal fluid. Methods Using our laboratory database, we identified patients with vitamin B6–dependent epilepsy and extracted their data on the concentrations of pyridoxal 5′-phosphate, pyridoxal, pipecolic acid, α-aminoadipic semialdehyde, and monoamine neurotransmitters. We compared the biochemical characteristics of these patients with those of other epilepsy patients with low pyridoxal 5′-phosphate concentrations. Results We identified seven patients with pyridoxine-dependent epilepsy caused by an ALDH7A1 gene abnormality, two patients with pyridoxal 5′-phosphate homeostasis protein deficiency, and 28 patients with other epilepsies with low cerebrospinal fluid pyridoxal 5′-phosphate concentrations. Cerebrospinal fluid pyridoxal and pyridoxal 5′-phosphate concentrations were low in patients with vitamin B6–dependent epilepsy but cerebrospinal fluid pyridoxal concentrations were not reduced in most patients with other epilepsies with low cerebrospinal fluid pyridoxal 5′-phosphate concentrations. Increase in 3-O-methyldopa and 5-hydroxytryptophan was demonstrated in some patients with vitamin B6–dependent epilepsy, suggestive of pyridoxal 5′-phosphate deficiency in the brain. Conclusions Low cerebrospinal fluid pyridoxal concentrations may be a better indicator of pyridoxal 5′-phosphate deficiency in the brain in vitamin B6–dependent epilepsy than low cerebrospinal fluid pyridoxal 5′-phosphate concentrations. This finding is especially helpful in individuals with suspected pyridoxal 5′-phosphate homeostasis protein deficiency, which does not have known biomarkers