Gustatory function in persons with cerebral palsy.

Gustatory function is closely related to chewing and swallowing; however, there are currently no reports regarding gustatory function in persons with cerebral palsy (CP). The aim of this study was to compare the gustatory function between persons with CP and healthy controls. We investigated sweet, salty, sour and bitter tastes using the whole-mouth method and measured the electrogustometric thresholds in the chorda tympani nerve area. Twelve participants with CP (6 women and 6 men) for whom gustatory testing was possible at our facilities, and 17 age-matched controls (9 women and 8 men) were included. The mean age ± standard deviation was 58.6 ± 8.1 years and 58.5 ± 8.7 years in subjects with CP and controls, respectively. Taste detection and identification were significantly worse in persons with CP compared with the controls. Taste identification was more impaired than taste detection. At the highest concentration, taste identification was impossible 11 times out of 48 (12 persons × 4 kinds of tastes) in persons with CP but such a deficit was not observed in 68 attempts (17 persons × 4 kinds of tastes) involving controls. The electrogustometric thresholds were not significantly different between the groups. Gustatory function associated with chewing and swallowing is worse in persons with CP compared to the controls.

Prostaglandin E-major urinary metabolite as a noninvasive surrogate marker for infantile necrotizing enterocolitis.

BACKGROUND: Early definitive diagnosis of necrotizing enterocolitis (NEC) based on Bell's staging criteria is difficult because there are few observable changes on abdominal imaging and blood chemistry tests at the onset of the disease. PURPOSE: To investigate whether prostaglandin E-2 major urinary metabolite (PGE-MUM) can be a useful surrogate marker reflecting the disease state and severity of NEC in infants. METHODS: Infants were enrolled in this study between January 2014 and December 2016. NEC diagnosis was based on Bell's staging criteria > Stage II or necrotic bowel observed at surgery. After diagnosis, PGE-MUM level was measured and compared with that of the other disease and healthy infant groups. RESULTS: Median PGE-MUM value was highest in the NEC group (576 [65-3672] µg/g•Cre/BSA × 1000), followed by the other disease group (94 [57-296] µg/g•Cre/BSA × 1000) and the healthy infant group (19 [10-44] µg/g•Cre/BSA × 1000) (sensitivity: 92.3%, specificity: 81.5%, accuracy: 85.0%; p < 0.01). PGE-MUM level correlated with improved status of NEC, length of necrotic intestine, and Bell's staging criteria. CONCLUSIONS: PGE-MUM level may be a useful surrogate biomarker reflecting the disease state of NEC. The method of urine sample collection is also advantageous, being noninvasive for infants. This is the first study reporting PGE-MUM level in NEC. TYPE OF STUDY: Study of diagnostic test. LEVEL OF EVIDENCE: LEVEL II.

Management of extremely low birth weight neonates with bowel obstruction within 2 weeks after birth.

PURPOSE: The majority of bowel obstructions in extremely low birth weight (ELBW) neonates are meconium-related ileus (MRI). ELBW neonates with bowel obstruction may recover by conservative treatment, but some do not. Considering the high surgical morbidity rates, unnecessary surgery should be avoided. We sought to identify a reasonable treatment strategy under these conditions. METHODS: ELBW neonates who started to have bowel obstruction with an unclear cause within 14 days of age were enrolled. The study period was from January 2009 to August 2011. The enrolled patients had daily Gastrografin(®) enemas until 14 days of age or until the obstruction resolved. If the obstruction lasted beyond around 14 days of age, the patient underwent surgical intervention. The clinical data of the patients were collected and analyzed. RESULTS: Fourteen patients were enrolled. Twelve patients had MRI, which resolved within 14 days without surgery. Two patients with persistent obstruction underwent surgery, and they were found to have Hirschsprung's disease and ileal volvulus, respectively. CONCLUSION: For ELBW neonates with bowel obstruction of unclear etiology, the early and frequent administration of a Gastrografin(®) enema is reasonable. Surgery should be considered if the obstruction lasts beyond approximately 14 days after birth.

Long survival of congenital alveolar capillary dysplasia patient with NO inhalation and epoprostenol: effect of sildenafil, beraprost and bosentan.

The case is described herein of a patient with alveolar capillary dysplasia with double-outlet right ventricle and duodenal atresia who survived for a remarkably long time. The newborn girl was born at a gestational age of 36 weeks and weighed 1926 g. One min after delivery the Apgar score was 4. The patient had persistent pulmonary hypertension (PH) and needed nitric oxide inhalation and i.v. epoprostenol all through her life. Although other oral medications for PH were tried, they could not be used in practice because of gastrointestinal complications. The patient died on the 237 th day of life as a result of worsening PH associated with infection.

Pseudouridine at position 55 in tRNA controls the contents of other modified nucleotides for low-temperature adaptation in the extreme-thermophilic eubacterium Thermus thermophilus.

Pseudouridine at position 55 (Ψ55) in eubacterial tRNA is produced by TruB. To clarify the role of the Ψ55 modification, we constructed a truB gene disruptant (ΔtruB) strain of Thermus thermophilus which is an extreme-thermophilic eubacterium. Unexpectedly, the ΔtruB strain exhibited severe growth retardation at 50 °C. We assumed that these phenomena might be caused by lack of RNA chaperone activity of TruB, which was previously hypothetically proposed by others. To confirm this idea, we replaced the truB gene in the genome with mutant genes, which express TruB proteins with very weak or no enzymatic activity. However the growth retardation at 50 °C was not rescued by these mutant proteins. Nucleoside analysis revealed that Gm18, m(5)s(2)U54 and m(1)A58 in tRNA from the ΔtruB strain were abnormally increased. An in vitro assay using purified tRNA modification enzymes demonstrated that the Ψ55 modification has a negative effect on Gm18 formation by TrmH. These experimental results show that the Ψ55 modification is required for low-temperature adaptation to control other modified. (35)S-Met incorporation analysis showed that the protein synthesis activity of the ΔtruB strain was inferior to that of the wild-type strain and that the cold-shock proteins were absence in the ΔtruB cells at 50°C.

Effects of artichoke leaf extract on acute gastric mucosal injury in rats.

The present study was designed to clarify the effects of an ethanol extract of artichoke leaf on acute gastric mucosal injury in rats. Oral administration of artichoke leaf extract dose-dependently prevented absolute ethanol-induced (125-500 mg/kg) or restraint plus water immersion stress-induced gastric mucosal injury (1000-2000 mg/kg). The artichoke leaf extract contains 1% cynaropicrin and 0.8% chlorogenic acid as main components and 70% dextrin as a vehicle. Cynaropicrin at doses of 1/100 of artichoke leaf extract [ethanol-induced mucosal injury: 5 mg/kg, per os (p.o.); stress-induced mucosal injury: 20 mg/kg, p.o.] also prevented gastric mucosal injury in both animal models. However, dextrin and chlorogenic acid at doses contained in the leaf extract were ineffective in both models. When artichoke leaf extract was given orally to normal rats, it (500-2000 mg/kg, p.o.) dose-dependently increased gastric mucus content. In addition, it (125-500 mg/kg, p.o.) dose-dependently prevented the decrease in gastric mucus content by absolute ethanol. When the effects of artichoke leaf extract on basal gastric acid secretion in rats were evaluated, it (500-2000 mg/kg, p.o.) dose-dependently increased the volume of gastric juice in normal rats. However, it was ineffective in decreasing basal gastric acid secretion in normal rats. These results indicate that artichoke leaf extract is effective against acute gastritis and its beneficial effect is due to that of cynaropicrin. The gastric mucus-increasing action of artichoke leaf extract may be, at least in part, related to the anti-gastritic action of the extract.

Wavy line closure for revision of abdominal scars with suture marks in children.

Although various methods are available to treat scars, it is difficult to manage those with suture marks that look like fish-bones, mainly because a large amount of tissue between the suture marks must be discarded with the scar. We report the wavy line closure for revision of abdominal scars with suture marks in three children who were operated on for congenital abdominal diseases. The entire scar, including all the suture marks, was excised using an incision consisting of a pair of smoothly waved lines. This incision makes it possible to reduce the tension on the wound by preserving the normal skin between the suture marks, which is followed by fine scars. The resulting wave-shaped scar is less noticeable and more resistant to postoperative contracture than a straight scar.

Surgical correction for curly toe using open tenotomy of flexor digitorum brevis tendon.

Curly toe is a common congenital deformity characterized by flexion and varus deformity of the interphalangeal joints. Because this minor deformity is seldom accompanied with any symptoms, treatment strategy has rarely been discussed in detail in the literature. Eight toes in seven patients with curly toe were treated by open tenotomy of the medial slip of the flexor digitorum brevis tendon. If sufficient correction was not obtained, the collateral ligament and the volar plate of the proximal interphalangeal joint were dissected. The skin defect at the plantar base of the toe was covered using a local flap or a full-thickness skin graft. The median age at operation was 2 years 6 months (ranged from 8 months to 5 years 4 months). In all cases, contracture of the plantar skin at the base of the toe and tight FDB tendon were recognized to a variable degree. Postoperatively, overlapping of the affected toe was corrected in every case at a median follow-up of 2 years 9 months. However, flexion and/or varus deformity tended to remain to some degree in those patients with severe curly toe. Toes with moderate to severe deformity with overlapping beneath the adjacent toe are candidates for surgical correction, because spontaneous correction is unlikely and troublesome symptoms may occur as the child grows older. Surgical correction should be performed until 2-3 years of age. The postoperative result might be poor, if treated in the older age, because skeletal deformity is likely to occur. Open tenotomy of the FDB tendon is easy to perform, and toe function was seldom impaired.

Telangiectatic focal nodular hyperplasia of the liver in the perinatal period: case report.

We report a case of congenital telangiectatic focal nodular hyperplasia, a rare variant form of the disease. The patient was a 2-month-old boy whose parents noticed abdominal distention about 2 weeks after birth, and ultrasonogram revealed a large mass in the liver. He underwent right lobectomy, and gross findings showed an ill-defined mass without any central scar. Histologic findings demonstrated proliferating hepatocytes without atypia arranged in cords of 1- or 2-cell thickness with marked sinusoidal dilatation and extramedullary hematopoiesis. In addition, a significantly increased Ki-67 labeling index in the tumor compared with non-tumor liver cells, and cytogenetic analysis of 23 G-banded metaphase preparations revealed 3 abnormal karyotypes, suggesting hyperplastic or neoplastic features. To the best of our knowledge, the present case is only the third documented case of congenital telangiectatic focal nodular hyperplasia.

Successful resection of an infected duodenal duplication cyst after percutaneous cyst drainage: report of a case.

We report a case of a duodenal duplication cyst complicated by infection. An 8-year-old boy was brought to our hospital with severe abdominal pain and a fever of 39.8 degrees C. He had a 5-year history of occasional abdominal pain with vomiting. On examination, a cystic mass was felt under the liver. We performed percutaneous drainage of the cyst under a suspected diagnosis of a choledochal cyst complicated by acute biliary infection. A contrast study through the drainage tube and surgical exploration revealed a duodenal duplication cyst communicating with the second part of the duodenum. The patient had an uneventful recovery and remains well 5 years after surgery. To the best of our knowledge, this is the first documented case of an infected duodenal duplication cyst successfully treated by resection after percutaneous drainage.

A multicenter phase II study of docetaxel 60 mg/m2 as first-line chemotherapy in patients with advanced or recurrent breast cancer.

PURPOSE: Docetaxel is an active agent as first-line chemotherapy in patients with advanced breast cancer at a dosage of 100 mg/m2. However, the efficacy of this agent as a first-line drug when used at a lower dosage is unclear. This study was performed to evaluate the clinical efficacy and safety of 60 mg/m2 docetaxel for the treatment of breast cancer. PATIENTS AND METHODS: This study enrolled 23 patients with advanced and/or metastatic breast cancer, who had not been treated with an anthracycline or taxane previously. Treatment with docetaxel was continued in patients showing a response until there was evidence of disease progression or unacceptable toxicity. RESULTS: Among 20 fully evaluated patients, the overall response rate was 50.0% and the median time to progression was 31 weeks. The most commonly observed adverse events were neutropenia (78.2%) and fatigue (60.9%). Fluid retention occurred in only 8.7% of the patients. Adverse events did not cause discontinuation of the treatment. CONCLUSION: Docetaxel achieved good disease control with mild adverse events in first-line treatment at a dosage of 60 mg/m2.

[Clinical aspect of diffuse alveolar hemorrhage syndrome].

Thirteen cases of diffuse alveolar hemorrhage (DAH) were encountered in our Hospital between January 1996 and October 2001. Eight patients were men and five were women, their mean age being 59.5 +/- 19.2 years (range, 18-88 years). Three patients had systemic lupus erythematosus (SLE), three (23%) had polyarteritis nodosa (including microscopic PN), one (7.7%) had allergic granulomatous angitis, one (7.7%) had Goodpasture syndrome, one (7.7%) had MPO-ANCA-associated vasculitis, one (7.7%) had Behçet's disease, one (7.7%) had chronic heart failure caused by mitral stenosis, one (7.7%) had chronic renal failure (etiology unknown), and the last had no particular disorder. Nine episodes (69%) had occurred as complications of primary diseases, four (31%) as the first symptoms of underlying diseases. Prognosis was poor in the former cases but in the latter, the prognosis was relatively favorable.