Partial trisomy for short arm of chromosome 5

We present two unrelated cases of partial trisomy for the short arm of chromosome 5, the first such cases reported in Japan. The features are characterized by hypertelorism, low set ears, arachnodactyly, laryngostenosis, hypotonia and some cerebral malformation. The characteristic facial expression and arachnodactyly are the key features used to diagnose this disorder. A high-resolution chromosome banding technique showed that the karyotype of the first patient was 46, XX, inv dup(5) (p13.1 ← p15.3) de novo and that of the second patient was 46, XX, dir dup(5) (p13.3 ← p15.2) de novo. The similar symptoms in the two cases, despite the difference in karyotypes, were caused by duplication of 5p including segment 5p13. This would be a key site for this disorder.     

Analysis of the circumstances at the end of life in children with cancer: A single institution''s experience in Japan

BACKGROUND: With the aim of improving the quality of life of children with cancer, this study presents an analysis of one hospital's experience with terminal care. METHODS: Between 1994 and 2000, 28 children died after treatment for cancer at Hamamatsu University Hospital. The circumstances of their deaths were analyzed through medical records and interviews with 8 sets of bereaved parents. We compared results of this analysis with our previous data collected from 1978 to 1993. RESULTS: Of the 28 children, 11 had leukemia/lymphoma (LL group) and 17 had solid tumors (ST group). Six children (21.4%), all of whom were in the LL group, died of treatment-related complications. Twenty children (71.4%) died during terminal care: three (27.3%) were in the LL group and 17 (100%) in the ST group. Eleven children (39.3%) received terminal care at home and eight (28.6%) of these died at home. The number of children who received terminal care and died at home had increased in comparison with the previous period. Among problems with terminal care identified by parents were the lack of opportunity for the child to continue with education and an inadequate support system after the child's death. CONCLUSIONS: Some advances in the quality of life of the children were recognized. However, these advances were extended to a greater percentage of children in the ST group than in the LL group. The psychosocial problems faced by children and their families are now changing for the better.     

Macrocystic Serous Cystadenoma of the Pancreas

Abstract: The case of a 61 -year-old man with macrocystic serous cystadenoma of the pancreas is reported. Abdominal ultrasonography and computerized tomography showed a multilocular cystic lesion in the body of the pancreas. The major cysts ranged in size from more than 2.0 cm up to 6.0 crn. Endoscopic ultrasonography (EUS) showed a few small cysts in addition to the large cysts. The preoperative diagnosis was either mucinous cystic tumor or congenital cyst of the pancreas and tumor enucleation was performed. Histological examination revealed serous cystadenoma of the pancreas with macrocysts. Detecting small cysts with EUS may be useful for distinguishing this unusual tumor from mucinous cystic tumors.     

Kinetic analysis of propranolol-induced impairment of its own metabolism in rats

Abstract— The effect of repetitive oral administration of propranolol (100 mg kg^?1 day^?1, 5 days) on the kinetics of liver microsomal propranolol metabolism was investigated in the rat. V_max values of the high-affinity phase for biphasic kinetics of propranolol 4- and 5- hydroxylase activities were decreased by propranolol pretreatment, while those of the low-affinity phase were unchanged. The V_max value of monophasic 7-hydroxylase activity was also decreased. On the other hand, the V_max value of N-desisopropylase activity in the propranolol-treated rats was increased more than 2-fold compared with non-treated (control) rats, resulting in a change from monophasic in control rats to biphasic kinetics in propranolol-treated rats. These findings indicate that repetitive administration of propranolol selectively impairs a CYP2D isozyme that is involved in the high-affinity phases for propranolol ring-hydroxylations.     

The safety of a nitric oxide inhalation system with high frequency oscillatory ventilation

Nitric oxide (NO) inhalation and high frequency oscillatory ventilation (HFOV) has been indicated in infants with severe respiratory failure. The purpose of the present study was to evaluate the safety of an NO inhalation system with HFOV in terms of nitrogen dioxide (NO₂) production. The NO inhalation system consisted of a high frequency oscillatory ventilator, a neonatal circuit and a test lung. The NO concentration was changed from 0 to 19 p.p.m. At each level of NO, the oxygen (O₂) concentration was changed from 21 to 100%. The NO and NO₂ concentrations were measured with a chemiluminescence analyzer using a molybdenum converter. The NO₂ concentration was increased when either the O₂ or the NO concentration was increased. The interposition of the endotracheal tubes increased NO₂ concentrations at 4 p.p.m. NO. The high stroke volume and high mean airway pressure produced a significant increase in NO₂ production at 4 p.p.m. NO. The increase in NO₂ production was prevented by placing a one-way valve at the joint of the NO gas line to the inspired limb. It was concluded that the NO inhalation system with HFOV can be safely used when a one-way valve is placed at the joint of the NO gas line to the inspired limb and when inhaled NO is at a relatively low concentration.     

Diagnostic Accuracy of Laparoscopic Liver Biopsy in Chronic Liver Diseases,Comparison of Laparoscopic and US-Guided Liver Biopsy Results

Liver biopsies were carried out using three different needles, a Vim-Silverman needle 2.5 mm in outer caliber, an 18-Gauge (18G) Majima needle, and a 17-Gauge (17G) Majima needle. The biopsies were obtained from nearby locations on the liver surface under laparoscopic observation, to ascertain differences in histological diagnosis according to the size of the biopsy specimen. The biopsy specimens obtained with the Vim-Silverman needle were wider than those obtained with the other two needles. The agreement in histological diagnoses of the liver, obtained with the Vim-Silverman needle versus the 18G Majima needle, was 26.0%, while that between the Vim-Silverman needle and the 17G Majima needle was 40.0%. Histological diagnosis tended to be underestimated in small biopsy specimens in advanced chronic liver diseases. A questionnaire survey, conducted in 92 hospitals affiliated with Okayama University Medical School, revealed US-guided liver biopsy to be the practice of choice in 57 of 92 (62.0%) hospitals, and 18G needles were used in US-guided liver biopsy in 35 of 78 (45.2%) hospitals.     

SYNTHESIS OF SUBSTRATES SPECIFIC FOR HUMAN SPLEEN FIBRINOLYTIC PROTEINASE (SFP)

Two peptide anilides, Suc-Tyr-Leu-Val-pNA (III) and Ac-Ser-His-I.eu-Val-pNA (V) were synthesized by the conventional method with the object of obtaining specific substrates for human spleen fibrinolytic proteinase (SFP). The K_cat/K_m values for hydrolysis of HI and V by SFP were 22 64 7 and 2 700, respectively. On the contrary, the K_cat/K_m values for hydrolysis of III and V by porcine pancreatic elastase were 21 and 9, respectively.     

Experimental periodontal breakdown in the dog

abstract – This paper describes a method for inducing rapidly progressing and reproducible periodontal lesions around selected teeth in dogs. The experiments were performed on 10 beagles which were fed a diet favoring gross formation of plaque. Approximately 1 mm of the marginal alveolar bone around ₄P and P₄ was removed and a notch prepared in the root. A ligature of cotton floss was placed around ₄P and P₄ at the cemento-enamel junction. Radiographs and sections prepared 230 d later revealed that a progressive breakdown of the periodontal tissues had occurred. The average bone loss was 2.4 mm and the distance between the apical cells of the pocket epithelium and the notch was 854μm.     

Nucleotide sequence of a hepatitis B virus genome of subtype adw isolated from a Philippine: Comparison with the reported three genomes of the same subtype

The complete nucleotide sequence was determined for a hepatitis B virus genome of subtype adw (pFDW294) isolated and cloned from the plasma sample of a Philippino. The genome was 3221 base-pair long with a point mutation at the 1376th nucleotide that affected the coding capacity of the P and X genes. There was a wide range of sequence divergence among pFDW294 and the reported three genomes of the same subtype (1.1–9.9%), occurring more often in the pre-S region and the S gene than in the pre-C region and the C gene.     

Developmental Toxicity of Aspirin Prenatally Given to Rats: Assessment in Slc: Wistar-KY Rats

ABSTRACT  Slc:Wistar-KY rats were administered orally with 62.5, 125, 187.5 or 250mg/kg aspirin suspended with 0.5 % CMC-Na on days 9–11 of gestation (plug += day 0). Suppression of maternal weight gain and food consumption during treatment was observed at and over 187.5 mg/kg. At term, the fetal mortality increased at and over 187.5 mg/kg and the fetal weight was lowered at and over 125 mg/kg. Among 15 live fetuses at 250 mg/kg, 4 had external malformations. In the skeletal examination (double staining), skeletal anomalies increased at and over 187.5 mg/kg. The skeletal variations such as vertebral anomalies, fused costal cartilages and increased presacral vertebrae were often encountered and delayed ossification was also found at and over 125 mg/kg. The internal anomalies tended to increase at and over 187.5 mg/kg. The live birth rate was significantly lower at 187.5 mg/kg than that in controls, and all pups, except for 3 from a dam, died before weaning. At 125 mg/kg, the pivoting locomotion on day 7 post partum was poorer as compared with controls. The physical and functional development at 62.5 mg/kg was not changed. There were no significant effects on male offspring in the open-field, rotarod, under-water T-maze and avoidance learning tests. However, in the Biel T-maze test (9–10 weeks of age), the aspirin-treated groups showed more errors and the increased elapsed time on the 1st trial day than controls. These results indicate that aspirin may induce a slight learning defect on rat offspring even at the non-teratogenic dose and the Biel T-maze test is more sensitive than any other learning tests given in this study.