Urinary uric acid excretion in term and premature infants

Objective : To clarify postnatal changes in urinary uric acid (UA) excretion in normal term infants and to examine the effects of prematurity or illness on the UA excretion.
Methodology : Measurements of urinary UA were performed in term and premature infants at the ages of 1 and 7 days and at 1 and 4 months, as well as at 7 months in term infants.
Results : Urinary UA levels were lowest on day 7 in term infants. The levels were highest on day 1 in premature infants and remained significantly higher compared to term babies during the first month of life. Respiratory failure requiring ventilation and oxygen supply resulted in further significant elevation of urinary UA in premature infants.
Conclusions : With the reference values obtained in the study reported here, urinary UA can now be used for the diagnosis and monitoring of inherited disorders of purine metabolism and for the assessment of oxygen radical insult to sick infants.     

Early onset of cecal perforation in neonatal,recto-sigmoid type Hirschsprung's disease

Hirschsprung's disease has been considered to cause intestinal perforation in rare cases. Even if a perforation occurs, the majority of cases are associated with the long-segment or total colonic type. Our case developed the perforation in the neonatal period in spite of being of the recto-sigmoidal type, and it affected the cecum. We do not have a good explanation for this condition. However, the pathological examination of the specimens of the perforated cecum revealed some necrosis (ulceration, subcutaneous hemorrhage, congestion and severe edema) which was considered to be caused by ischemia, secondary to a localized vascular accident in the wall of the distended intestine.     

雌激素受体基因多态性与儿童孤独症临床表型特征的关联研究

【目的】 探讨雌激素受体(estrogen receptor,ER)基因多态性与儿童孤独症临床表型特征的关系。 【方法】 应用TaqMan荧光探针Real-Time PCR的分析方法对127名孤独症患儿进行ERα的单核苷酸多态性片断rs11155819和ERβ的单核苷酸多态性片断rs1152582的基因型测定。用儿童孤独症评定量表(Children Autism Rating Scale,CARS)评价孤独症患儿临床表型特征。 【结果】 孤独症病情严重程度在rs11155819和rs1152582的不同基因型之间的差异无统计学意义(P>0.05)。rs11155819基因型为C/T、T/T的孤独症儿童在语言交流项得分的差异有统计学意义(P=0.048)。rs1152582基因型为C/C、G/G的孤独症儿童在非言语交流项、活动水平项得分的差异有统计学意义(P=0.035;P=0.030)。 【结论】 rs11155819基因型中,携带T/T的患儿比携带C/T的患儿有更明显的语言交流障碍。rs1152582基因型中,携带C/C的患儿比携带G/G的患儿有更明显的非言语交流障碍和活动异常。     

Clinical study of complicated urinary tract infection using ''The UTI Criteria (Draft Fourth Edition)'': Measurement methods for pyuria

OBJECTIVE: To evaluate the influences the change of the measurement method of pyuria from conventional centrifuged sediment to microchamber uncentrifuged urine for the results of evaluation of antimicrobial agents in clinical study against complicated urinary tract infections. From the viewpoint of international harmonization of judgement criteria, the recent method for counting white blood cells (WBC) in urine has changed from using uncentrifuged urine to using a microchamber in all countries. METHODS: Targeted diseases were non-catheterized complicated urinary tract infection, and cefcapene pivoxil hydrochloride or levofloxacin were used as antimicrobial drug. Pyuria was examined using the counting chamber method, a quantitative method using uncentrifuged urine with a microchamber, and the sedimentation method. RESULTS: Overall clinical efficacy in early evaluation by the two methods in measuring pyuria was evaluated as different in eight patients (7.3%). It was rated excellent in 63 (52.9%), moderate in 32 patients (26.9%) and poor in 24 (20.2%) with an efficacy rate of 79.8% using the counting chamber method, and excellent in 68 (57.1%), moderate in 27 (22.7%) and poor in 24 (20.2%) with an efficacy rate of 79.8% using the conventional sedimentation method CONCLUSION: No significant difference was seen between the two methods of WBC count in urine.     

High dose chemotherapy including paclitaxel (T-ICE) combined with peripheral blood stem cell transplantation for male germ cell tumor. Preliminary report

AIM: To evaluate the feasibility and usefulness of high dose chemotherapy including paclitaxel (T-ICE) combined with peripheral blood stem cell transplantation (PBSCT) for male germ cell tumor. METHODS: Five male patients with advanced germ cell tumor underwent 1-6 courses of high dose chemotherapy including paclitaxel (T-ICE; 175 mg/m2 of paclitaxel, 1250 mg/m2 of carboplatin, 1500 mg/m2 of etoposide and 7.5 g/m2 of ifosfamide) with PBSCT after 2-3 courses of induction chemotherapy (PEB or VIP). RESULTS: In all patients, serum marker levels decreased to within the normal range by T-ICE. Two patients underwent resection of residual tumor. In one patient, viable cancer cells were detected in resected lymph node tissue and adjuvant chemotherapy was then performed. Although the follow-up period was short (7-15.5 months), four of the five patients (80%) showed no evidence of recurrent disease. No significant differences in side-effects were noted between T-ICE and conventional high dose ICE, which was previously performed in 39 patients at the Division of Urology, Kobe University Graduate School of Medicine, Japan. CONCLUSIONS: High dose chemotherapy, including T-ICE, combined with PBSCT showed an almost identical degree of side-effects as seen in previous high dose chemotherapy without paclitaxel. Although 80% of the patients showed no evidence of disease so far, the efficacy of T-ICE should be evaluated with more patients and longer follow up.     

Clinical outcome of conservative therapy for stage T1, grade 3 transitional cell carcinoma of the bladder

BACKGROUND: The objective of this study was to retrospectively investigate the effectiveness of transurethral resection of bladder tumor (TURBT) and intravesical instillation therapy for stage T1, grade 3 (T1G3) transitional cell carcinoma (TCC) of the urinary bladder. METHODS: Between January 1995 and December 1997, 97 patients with T1G3 TCC of the urinary bladder were treated by TURBT and adjuvant intravesical instillation with bacillus Calmette-Guérin (BCG) or other anticancer agents. The recurrence-free survival rates were evaluated according to several clinicopathological factors. The cases that progressed to muscle invasive disease were also analysed. RESULTS: In this series, the median follow-up period was 25 months (range, 5- 41) after the initial TURBT. Intravesical recurrence was noted in 44 patients (45%), and the 1, 2, and 3 year recurrence-free survival rates were 72%, 58%, and 42%, respectively. Multivariate analyses revealed that the risk of intravesical recurrence was significantly higher for patients who did not receive BCG therapy, irrespective of age, gender, tumor size, multiplicity, pathological stage, concomitant carcinoma in situ, and lymphovascular involvement. Moreover, after a median of 10 months, disease progression occurred in seven patients (7%), of which only one patient was treated by BCG therapy after initial TURBT. CONCLUSION: These findings suggest that intravesical instillation with BCG combined with TURBT is an effective conservative treatment for T1G3 TCC of the bladder. Patients with negative prognostic factors should be treated by BCG rather than other anticancer agents after TURBT.     

STUDY OF AXONAL DYSTROPHY. I. PATHOLOGY OF THE NEUROPIL OF THE GRACILE AND THE CUNEATE NUCLEI IN AGEING AND OLD RATS: A STEREOLOGICAL STUDY

The changes with age in the neuropil of the gracile and the cuneate nuclei of rats were studied using stereological techniques, in relation to the occurrence of axonal dystrophy. The following were found: (1) significant difference in the volume fraction of presynaptic boutons between the gracile and the cuneate nuclei throughout the whole life span (17% and 13% respectively at 100 days of age); (2) progressive decrease in the volume fraction (34% decrease in the gracile nucleus between 100 and 800 days of age) and in the numerical density of presynaptic boutons, the decline being evident as soon as the animals reached maturity and before axonal dystrophy became manifest; (3) significant differences in the volume fractions of dendrites and of nerve cell bodies between the two nuclei throughout the whole life span of the animals, both being greater in the cuneate than in the gracile nucleus; an agerelated decrease in the volume fraction of dendrites was also suspected in the gracile nucleus; (4) progressive increase in the volume fraction of fibrous astrocytic processes (from 3% at 100 days to 10.5% at 800 days in the gracile nucleus); (5) the above described age-related changes of presynaptic boutons and fibrous astrocytic processes were significant only in the gracile nucleus, not in the cuneate. The loss of boutons in ageing gracile nuclei was partially reflected in the appearance of degenerating nerve fibres in ageing gracile tract in the rostral cervical cord. Involutional loss of boutons and dystrophic formation of spheroids both appear and progress closely related in time and space. It was suggested that this set of changes can be understood as one integrated whole in which axonal dystrophy may represent only one side of the coin. The question of the causal mechanism of axonal dystrophy still remains unanswered.     

Neonatal Dubin-Johnson Syndrome with Severe Cholestasis: Effective Phenobarbital Therapy

ABSTRACT. We described Dubin-Johnson syndrome (DJS) with severe cholestasis in a 20-day-old Japanese boy. Although neonatal DJS has been sporadically reported, DJS with severe cholestasis has not to our knowledge been described in the English literature. The ratio of urinary coproporphyrin isomer I to urinary total coproporphyrin in our patient was high (93%). Liver histology showed cytoplasmic pigment granules in the liver cells. Administration of phenobarbital (PB) significantly decreased the levels of bilirubin and bile acids in the serum. There was a significant elevation of 1β-hydroxylated bile acids in the urine. It is predicted that severe cholestasis in neonatal DJS may cause metabolic abnormalities in both bilirubin and bile acids transport.