Acute myeloblasts leukaemia without Philadelphia chromosome developing after interferon therapy for chronic myelocytic leukaemia with Philadelphia chromosome

Summary. We report a patient who developed Philadelphia chromosome negative acute myeloblastic leukaemia with trisomy 8 and trisomy 11 after receiving treatment with alkylating agents and interferon for chronic myelocytic leukaemia positive for Philadelphia chromosome. Leukaemic cells were positive for myeloperoxidase and expressed CD13, CD33 and DR; some expressed CD2, CD4 and CD34. The fluorescence in situ hybridization method revealed that bcr-abl fusion genes were absent from > 90% of the bone marrow cells. The major bcr rearrangement was not detected by Southern blot analysis. We conclude that the leukaemic cells negative for Philadelphia chromosome may have developed as a result of treatment with alkylating agents and interferon in the present case.     

Event-related changes of background electroencephalogram during contingent negative variation paradigm

Abstract The autoregressive model was applied to the background electroencephalogram during the tone(S1)-tone(S2)-respond contingent negative variation paradigm under eyes-open condition. The EEG data were obtained at the scalp sites of Fz, Cz, and Pz in seven normal males. During the first half of the interstimulus interval (ISI) of 2.5 s, Fz-dominant excitatory process was suggested in concordance with an orienting response to stimulus 1. In the second half of the ISI, Pz-dominant inhibitory process was considered. The stimulus 2 presentation and the required motor response promoted the Pz-dominant process.     

Structural studies on endothelin receptor subtype B specific agonist IRL 1620 (suc-[Glu9,Ala11,15]ET-1(8-21)) and its analogs with dipalmitoyl phosphatidylcholine vesicles by NMR spectroscopy

IRL 1620 {suc-[Glu⁹,Ala^11,15]ET-1(8-21)} is a potent and specific agonist for the ET_B receptor. Five analogs of IRL 1620 were synthesized in this study. These were all C-terminal linear peptides of endothelin 1 (ET-1) comprising 14 amino acid residues and exhibiting highly potent ET_B receptor binding affinities. The peptides consisted of three pairs and each component of the pairs differed from its partner in only the 18th residue, i.e. Asp was replaced by Gly. The replacements resulted in more than a 10-fold increase in affinity to the ET_A receptor. The structures of these peptides were investigated in the presence of phospholipid vesicles (dipalmitoyl phosphatidylcholine) by NMR spectroscopy. By the replacement of Asp by a less bulky Gly, the C-terminal tripeptide region folded back toward the helical region, making it shorter than the Asp-substituted peptide helical region. Such a folded conformational feature may explain the increased binding affinity to ET_A receptor.     

Suppression of Storms of Ventricular Tachycardia by Epicardial Ablation of Isolated Delayed Potential in Noncompaction Cardiomyopathy

A 65‐year‐old recipient of an implantable cardioverter defibrillator suffering from ventricular noncompaction developed storms of ventricular tachycardia (VT). Epicardial voltage mapping revealed the presence of a large low‐voltage area in the left ventricular apical and inferoposterior wall, and isolated delayed potential was recorded over 1.5 cm in the posterior border between low and normal myocardial voltage. Pacemapping at the delayed potential recording site produced two different QRS depending on pacing output strength, and these two QRS morphologies were similar to clinically documented VTs. During one of the VTs, a mid‐diastolic potential was recorded from the site with the delayed potential, and rapid pacing produced concealed entrainment. After epicardial radiofrequency ablation of the isolated delayed potential, VTs were noninducible and the VT storm was suppressed.     

Definitions of severely mentally and physically disabled children in Japan: Do the differences affect the prevalence rates of these children?

The prevalence rate of severely mentally and physically disabled children (SDC) aged 6–15 years in Okinawa prefecture on 1 May 1989 was 0.74/1000 (143/192 038) according to Oshima's classification, compared with 0.89/1000 (170/192 038) according to the Ministry of Education's classification with minor modifications. The number of children in region classes 1, 2, 3 and 4 of Oshima's classification for SDC were 100, 34, 6 and 3, respectively. The difference (n = 27) between the total numbers of SDC according to the two classifications was mainly because of 21 children categorized as ‘walking with support’ who were included as SDC according to the Ministry of Education's classification but not as SDC according to Oshima's classification. Only region class 1 of Oshima's classification corresponded with region class 25 of the Ministry of Education's classification. The results of the present study indicate that the differences between the two definitions of SDC affect the reported prevalence rates of SDC. Therefore, changing patterns in the prevalence of SDC should be assessed by serial surveys using the same method in each district.     

Thermal denaturation of tryptophan synthase α-subunit

Thermal denaturation for the wild-type of tryptophan synthase α-subunit from E. coli and one of its mutant proteins was followed by CD measurements at various pHs in the alkaline region and the results from van't Hoff analyses of the thermal denaturation curves were compared with those from calorimetry. Although the far-u.v. CD spectra of the thermally denatured proteins differed from those of the completely denatured states in 3.2 M guanidine hydrochloride, the titration curves by denaturants at higher temperatures were not sigmoidal but straight lines, indicating that the cooperative structure of the proteins has been completely destroyed by heating. The ratio of calorimetric enthalpy change to van't Hoff enthalpy change obtained from calorimetric study was unity, indicating that the thermal denaturation of the proteins was a two-state system. The unfolding heat capacity change (ΔCp) of the wild-type protein from van't Hoff analysis of the thermal denaturation curves by CD measurement was estimated to be 2.45 kcal/mol ± deg, which was similar to that from calorimetry. The values of unfolding enthalpy change at denaturation temperatures were lower by about 15 kcal/mol compared to those from calorimetry.     

Accidental poisoning of children in Japan: A report from the Japan Poison Information Center

The Japan Poison Information Center (JPIC) was founded only 6 years ago as a result of co-operation between the Ministry of Health and Welfare, the Japanese Association for Acute Medicine, the Japan Pediatric Society and other related medical organizations. The JPIC is the only poison information center admitted by the Ministry of Health and Welfare to provide toxicological information to medical personnel and the general public, and has two offices on duty in alternating 24 h shifts. Every year, JPIC receives about 30 000 inquiries. About 82% of these inquiries are from the general public and 84% of the patients are children 5 years and younger. We contrasted the data in the fiscal year 1991 with the data of the American Association of Poison Control Centers (AAPCC). Child poison exposure in Japan is characterized by a high exposure rate of children under 1 year of age to (mostly) household products. The JPIC also analyzed the cause of tobacco ingestion. It is considered that the Japanese lifestyle causes differences from those reported by AAPCC. We report the accidental poisoning of children in Japan.     

Serum free T4 and thyroid stimulating hormone levels in preterm infants and relationship between these levels and respiratory distress syndrome

BACKGROUND: There have been few studies of the thyroid stimulating hormone (TSH) surge in extremely low-birthweight (ELBW) infants, and the relationship between thyroid hormones and respiratory distress syndrome (RDS) has yet to be clarified. The present study sought to determine the serum levels of free T4 (fT4) and TSH in ELBW infants and to examine the relationship between these levels and the development of RDS. METHODS: The authors measured serum fT4 and TSH levels soon after birth in 449 preterm infants, who were born at 22-36 weeks of gestation, and determined the associations between these levels, the incidence of RDS, and the recognized clinical factors associated with RDS. RESULTS: Serum fT4 and TSH levels, and the fT4/TSH ratio, in the group at 22-24 weeks of gestation were significantly lower than those in the group at 28-36 weeks. The levels and ratio increased significantly with increasing gestational age. There were significant correlations between the serum fT4 level and the birthweight, Apgar score, and gender, and between the serum TSH level and the gestational age, mode of delivery, and birthweight. No significant relationship between the incidence of RDS and the serum levels of fT4 and TSH was observed. CONCLUSION: The authors' results suggest that the serum levels of fT4 and TSH in ELBW infants are very low, and that these levels are not correlated with the occurrence of RDS.     

Trans-mesosigmoid cutaneous ureterostomy

BACKGROUND: A new method was developed in order to create a single stoma cutaneous ureterostomy in which both ureters traverse the abdominal cavity and yet are buttressed by the mesosigmoid and covered by its visceral peritoneum. METHODS: The long mesenterium which is attached to the most mobile part of the sigmoid colon was used for the bilateral ureteral pathway. Tunnels for the ureteral path were made just underneath the visceral peritoneum on the bilateral side of the mesosigmoid. Through the tunnels both ureteral ends were brought from the retroperitoneal space to the mesenterocolonic junction (MCJ) and the MCJ is then approximated and sutured to the inside of the ureteral tract through the abdominal wall. The ureters brought outside the skin, are conjoined and sutured to the V skin flap. Eight patients who carried a high risk for operation and/or had a bladder tumor judged to be incurable underwent this cutaneous ureterostomy. RESULTS: All cases except one with low urinary output could be managed without catheter indwelling during the follow-up period. Three patients suffered from paralytic ileus and one required laparotomy for mechanical ileus during the short postoperative period. Postoperative excretory urography evaluated 14 kidneys during the follow-up period from 2 to 61 months and showed normal upper urinary tract in 11 and a mildly hydronephrotic tract in three. CONCLUSIONS: Transmesosigmoid cutaneous ureterostomy provides a single catheterless stoma even when the available ureters are relatively short. It appears to be a good method for supravesical urinary diversion when indicated.     

Alteration of esophageal pressure in sleep-disordered breathing

Abstract We investigated the alteration of esophageal pressure (Pes) in 10 patients with upper-airway sleep-disordered breathing (UASDB) and the relationship among Pes, breathing patterns and EEG arousals. Increased negative Pes without apnea or hypopnea, appeared not only in upper airway resistance syndrome but also in obstructive sleep apnea syndrome. This phenomenon produced frequent EEG microarousals leading to sleep fragmentation and daytime sleepiness. Moreover, increased negative Pes occasionally continued for more than 20 min without an EEG arousal, which might be considered to be one of the factors to cause complications of UASDB.