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1.
Zhonghua Yi Xue Za Zhi ; 99(40): 3158-3163, 2019 Oct 29.
Article in Chinese | MEDLINE | ID: mdl-31694107

ABSTRACT

Objective: To assess the oncologic outcomes of radical nephroureterectomy (RNU) combined with adjuvant chemotherapy (ACT) in patients with high risk upper tract urothelial carcinoma (UTUC). Methods: From January 2014, all high-risk UTUC patients after RNU surgery were enrolled in this prospective comparative trial. And these patients were randomized to ACT group (Gemcitabine+Cisplatin three weeks regimen) and observing group. Cox proportional hazard modeling and Kaplan-Meier analysis were used to determine overall survival (OS), cancer specific survival (CSS) and disease-free survival (PFS) in the cohort. Results: The median follow-up duration was36 months (range: 6-54) in the ACT group (n=94) and 30 months (range: 6-54) in the observing group (n=82). Oncologic outcomes of RNU treated high-risk UTUC patients were improved much significantly by ACT: OS [P=0.0397, HR: 1.39(0.91-1.75)], CSS [P=0.0255, HR: 1.26(1.07-1.45)] and PFS [P=0.0033, HR: 3.78(3.13-4.55)]. The further analysis in lymph node positive cohort displayed that median times of oncologic events were prolonged in the ACT group compared with the observing group: OS (26.8mon vs 36.3mon, P=0.0255), CSS (28.2mon vs39.3mon, P=0.0197) and PFS (11.4mon vs 31.9mon, P=0.0018). Additionally in T3/4 cohort, the significant growth in the median times of OS (20.6mon vs 32.2mon, P=0.0183), CSS (21.9mon vs 38.4mon, P=0.0226) and PFS (13.9mon vs 36.3mon, P=0.0217) were observed in ACT group. Conclusion: ACT could play the important synergistic role in improving the OS, CSS and PFS of high-risk UTUC patients after RNU.


Subject(s)
Carcinoma, Transitional Cell , Nephroureterectomy , Carcinoma, Transitional Cell/drug therapy , Chemotherapy, Adjuvant , Humans , Nephrectomy , Prognosis , Prospective Studies , Treatment Outcome
2.
Zhonghua Yi Xue Za Zhi ; 99(16): 1237-1240, 2019 Apr 23.
Article in Chinese | MEDLINE | ID: mdl-31060163

ABSTRACT

Objective: To investigate the effect of the derepression of chemokine receptor-7 (CXCR7) in prostatic tissues from patients with Castration Resistant Prostate Cancer (CRPC) on the resistance to enzalutamide (Enza). Methods: During the period of January 2015 to December 2017 all CRPC cases who underwent radical radiotherapy or androgen deprivation therapy (ADT) were evaluated. After prostatic puncture biopsy, the tissues were treated for immunostaining with CXCR7. Cox proportional hazard modeling and Kaplan-Meier analysis were used to determine PSA Progression-Free Survival (PSAP-FS) and Clinical or Radiographic Progression-Free Survival (CRP-FS) in the cohort. At last, PSA response rates and progression outcomes in CXCR7 negative cases and CXCR7 positive cases were analyzed. Results: Total 39 CRPC patients were enrolled in this study. And 23 cases derepress CXCR7, 16 cases negatively express CXCR7. The median follow-up duration was 12 months (range: 6-18) in the cohort. Chi-square analysis confirmed that PSA response rates after Enza treatment were significantly associated with CXCR7 derepression (χ(2)=22.129, P=0.000 06). Compared with CXCR7 positive expression group, CXCR7 negative expression group displayed improved median PSAP-FS (4.4 mon vs 11.7 mon, P=0.040 8) and CRP-FS (5.2 mon vs 13.1 mon, P=0.036 2) after Enza treatment. Conclusion: Derepression of CXCR7 in CRPC patients may be associated with resistance to enzalutamide. This protein may be novel target for treatment of CRPC.


Subject(s)
Prostatic Neoplasms, Castration-Resistant , Receptors, CXCR/metabolism , Androgen Antagonists , Benzamides , Disease-Free Survival , Humans , Male , Nitriles , Phenylthiohydantoin/analogs & derivatives , Prostate-Specific Antigen
3.
Zhonghua Yi Xue Za Zhi ; 99(2): 115-119, 2019 Jan 08.
Article in Chinese | MEDLINE | ID: mdl-30669749

ABSTRACT

Objective: To assess the oncologic outcomes of radical nephroureterectomy (RUN) combined with adjuvant chemotherapy (ACT) in patients with high risk upper tract urothelial carcinoma (UTUC). Methods: One-hundred-thirty-four individuals with high-risk UTUC who underwent RUN with or without ACT were evaluated. Cox proportional hazard model and Kaplan-Meier analysis were used to determine overall and cancer specific survival in the cohort. Results: The median follow-up duration was 24 months (range: 6-36) in the RUN group (n=61) and 18 months (range: 6-36) in the RUN+ACT group (n=73). Median time of overall survival (OS) and cancer specific survival (CSS) showed much better in RUN+ACT group than in RUN group, but the differences were not reached the significant standard. The further analysis in lymph node positive cohort displayed that median times of oncologic events were prolonged in the RUN+ACT group compared with the RUN group: OS (30.1 mon vs 18.0 mon, P=0.083) and CSS (29.2 mon vs 18.6 mon, P=0.047). Additionally in T3/T4 cohort, the significant growth in the median times of OS (25.2 mon vs 12.6 mon, P=0.038) and CSS (31.3 mon vs 18.9 mon, P=0.044) were observed in combination treatment group. Conclusion: ACT could play the important synergistic role in improving the OS and CSS of RUN treated UTUC patients with lymph node-positive or stage of T3/T4.


Subject(s)
Nephroureterectomy , Urologic Neoplasms , Carcinoma, Transitional Cell , Chemotherapy, Adjuvant , Humans , Nephrectomy , Retrospective Studies , Treatment Outcome , Urologic Neoplasms/therapy
4.
Eur Rev Med Pharmacol Sci ; 22(14): 4720-4729, 2018 07.
Article in English | MEDLINE | ID: mdl-30058713

ABSTRACT

OBJECTIVE: Hydrazone compounds and their derivatives are a kind of special Schiff bases. The multiple hydrazone compounds and their derivatives have a variety of biological activities. This study aims to report the synthesis of diverse hydrazone derivatives and to explore the potent antitumor activities. MATERIALS AND METHODS: A series of aromatic heterocyclic sulfonyl hydrazones W1-W15 synthesized from hydrazine or acylhydrazine and aldehydes or ketones were estimated for their in vitro antitumor activities against human cancers. Through the spectral (FT-IR, 1H-NMR, MS) methods, the structure of the compounds was determined. Using MTT (3-(4,5-dimethyl-2-thiazolyl)-2,5-diphenyl-2-H-tetrazolium bromide) method, the effects of different concentrations of compounds on growth inhibition and viability of HepG-2 cells were detected. RESULTS: Compound W9 exhibited anti-proliferation activity with IC50 values of 63.91 µmol/L in HepG-2 cell line. In addition, mechanism studies indicated that compound W9 could distinctly prohibit the propagation of HepG-2 cells by arresting the cell cycle at G2/M and inducing apoptosis. Furthermore, we investigated the effectiveness of drug combination treatment with W9 and cis-platinum (cis-DDP) or 5-fluorouracil (5-FU) on HepG-2 cell line. CONCLUSIONS: Our results indicated that W9 with synergic treatment of 5-FU or cis-DDP shows better inhibitory cell growth. The combination of the two drugs blocks HepG-2 cells in the G2/M phase. The inhibitory effect of W9 on cell apoptosis was decreased with the increase of drug concentration.


Subject(s)
Antineoplastic Agents/chemical synthesis , Carcinoma, Hepatocellular/drug therapy , Hydrazones/chemical synthesis , Liver Neoplasms/drug therapy , Apoptosis/drug effects , Carcinoma, Hepatocellular/pathology , Cell Cycle/drug effects , Cell Proliferation/drug effects , Hep G2 Cells , Humans , Hydrazones/therapeutic use , Liver Neoplasms/pathology , Structure-Activity Relationship
5.
J Nanosci Nanotechnol ; 9(2): 1076-9, 2009 Feb.
Article in English | MEDLINE | ID: mdl-19441459

ABSTRACT

N-doped carbon nanotubes (CNTs) with ultra-large cavity have been synthesized by using a mixture of ZnO and graphite as catalyst in the floating catalyst method. The as-synthesized N-doped CNTs are very pure, and a striking characteristic structure is that the outer diameter is at least 10 times larger than the wall thickness. Moreover, electronic properties analysis reveals that the N-doped CNTs with ultra-large cavity have a reduced room temperature resistance compared with those of the common N-doped CNTs, which give an experimental prove for the previous theoretical predictions.

6.
Urology ; 58(3): 452-6, 2001 Sep.
Article in English | MEDLINE | ID: mdl-11549498

ABSTRACT

OBJECTIVES: To assess the value of magnetic resonance imaging (MRI) in the anatomic evaluation and management planning of complex congenital genitourinary anomalies. METHODS: Multiplanar T(1) and T(2)-weighted MR images were obtained in 6 pediatric patients with congenital genitourinary anomalies, including aphallia, diphallia, ectopic scrotum, and epispadias. The imaging studies were read by experienced radiologists and discussed with the urologic surgeons in a multidisciplinary conference. RESULTS: Each congenital anomaly was demonstrated in detail by MRI. The MR images of penile agenesis showed hypoplastic corpora cavernosa and a vestigial bulb. In patients with penile duplication, MRI was able to delineate the course of each corporal body and the varying degree of thickness of the tunica albuginea. For the patient with scrotal ectopia, detailed MR images excluded both the possibility of urethral and corporal duplications and the presence of viable testes in the ectopic scrotum. In the case of epispadias, MRI illustrated the precise spatial relationship between the erectile bodies and urethra. Additionally, MRI identified related aberrant pelvic organs and provided images of the external genital structures. CONCLUSIONS: MRI, by rendering excellent anatomic interpretation of complex genital anomalies and associated abnormal pelvic tissues, assists surgeons in conceptualizing the anomalous structures and contributes to their formulation of management approaches.


Subject(s)
Genitalia, Male/abnormalities , Magnetic Resonance Imaging/statistics & numerical data , Adult , Congenital Abnormalities/diagnosis , Epispadias/diagnosis , Humans , Infant , Male , Penis/abnormalities , Scrotum/abnormalities
7.
J Urol ; 165(5): 1580-4, 2001 May.
Article in English | MEDLINE | ID: mdl-11342921

ABSTRACT

PURPOSE: Involvement of the prostate by bladder cancer directly impacts survival, the risk of urethral recurrence, and treatment decisions concerning the timing of cystectomy and type of urinary diversion. Transurethral lateromontanal loop biopsies are proposed as the most accurate method for evaluating the prostatic urethra. Due to the potential clinical impact on individuals we assessed its accuracy in a large cohort. MATERIALS AND METHODS: Transurethral lateromontanal loop biopsies were performed in 246 of 416 male patients at our institution between 1989 and 1997. The predictive value and sensitivity of transurethral biopsy, patterns of recurrence, survival and clinical impact were assessed in a cohort with 10 years of followup. RESULTS: The sensitivity of transurethral biopsy for prostatic stromal invasion was 53%, specificity was 77%, positive predictive value was 45% and negative predictive value was 82%. At the 10-year followup 129 patients (52.4%) were dead, 85 (32%) had no evidence of disease, 16 (6.5%) had disease and 16 (6.5%) were lost to followup. Mean followup in patients at risk for urethral recurrence was 61.7 months (range 0.56 to 134.1, median 56.8). Delayed urethrectomy was performed in 15 of 235 cases (6.4%) at a mean of 15.2 months. Of the 246 patients 99 had prostatic disease at transurethral biopsy and/or cystectomy, including 11 (11%) with urethral recurrence. No patient required continent diversion takedown or died of urethral recurrence. CONCLUSIONS: Transurethral biopsy did not accurately determine prostatic involvement. Prostatic involvement at biopsy or cystectomy translated into a higher risk of urethral recurrence. However, it did not have significant clinical impact or affect survival and should not be an absolute contraindication to urethral diversion.


Subject(s)
Biopsy/methods , Prostate/pathology , Prostatic Neoplasms/diagnosis , Urinary Bladder Neoplasms/pathology , Adult , Aged , Aged, 80 and over , Cystectomy , Follow-Up Studies , Humans , Male , Middle Aged , Multivariate Analysis , Neoplasm Invasiveness , Predictive Value of Tests , Prostatic Neoplasms/pathology , Sensitivity and Specificity , Survival Rate , Urethra/surgery , Urethral Neoplasms/secondary , Urethral Neoplasms/surgery , Urinary Bladder Neoplasms/mortality , Urinary Bladder Neoplasms/surgery
8.
J Urol ; 163(5): 1577-83, 2000 May.
Article in English | MEDLINE | ID: mdl-10751891

ABSTRACT

PURPOSE: A decrease in the glycosaminoglycan (GAG) layer on the urothelium is believed to be one of the possible causes of interstitial cystitis. Consequently, GAG-like substances and hyaluronic acid (HA) have been prescribed for treating this condition. To delineate the possible role of GAG and HA in the interstitial cystitis disease process, we compared the urinary levels of total GAGs (sulfated + non-sulfated), sulfated GAGs and HA in interstitial cystitis patients and normal controls. We also examined different HA species present in the urine of interstitial cystitis patients. MATERIALS AND METHODS: The total GAG and sulfated GAG levels in urine specimens of normal individuals (n = 20) and interstitial cystitis patients (n = 25) were determined by utilizing the carbazole reaction assay and the Farndale method, respectively, and were expressed as microg./mg. creatinine. Urinary HA levels were measured by applying the HA test and were expressed as ng./mg. creatinine. Gel filtration column chromatography was used to examine the profile of urinary GAGs and HA species. RESULTS: Total urinary GAGs were 2.5 to 4-fold elevated in interstitial cystitis patients with moderate to severe symptoms (Group 2; 76.2 +/- 24.8) when compared with those in normal individuals (19.9 +/- 2.5) and patients with mild symptoms (Group 1; 30.4 +/- 5.1) (p <0.001). Three urinary GAG peaks were detected in both normal and interstitial patients. However, each GAG peak from interstitial cystitis patient urine was 3 to 5-fold higher than that from normal patient urine. The sulfated GAG levels, however, remained unchanged among normal individuals (1.4 +/- 0.22), Group 1 (2.2 +/- 0.96) and Group 2 (1.6 +/- 0.38) patients (p >0.05). Consequently, the ratio of total GAGs to sulfated GAGs was elevated 3 to 3.5-fold in Group 2 patients (49.9 +/- 13.9) in comparison to that in normal individuals (16.7 +/- 2.5) and group 1 patients (14.4 +/- 4.6) (p <0.001). Urinary HA levels were marginally elevated in Group 2 patients (821. 4 +/- 247.9) when compared with those in the normal group (337.3 +/- 106.1) and Group 1 patients (540.9 +/- 166.5). In addition, a distinct high molecular mass HA species was present only in Group 2 patients. CONCLUSIONS: The increased ratio of total GAGs to sulfated GAGs and marginally elevated HA levels in urine indicate that the GAG layer is altered in interstitial cystitis patients. However, these results are in contrast to the accepted concept that a reduction in urothelial GAGs causes interstitial cystitis. The high molecular mass HA species detected in patients with severe symptoms may play a role in the pathophysiology of this disease.


Subject(s)
Cystitis, Interstitial/urine , Glycosaminoglycans/urine , Hyaluronic Acid/urine , Adult , Humans , Middle Aged
9.
Blood ; 89(11): 4213-8, 1997 Jun 01.
Article in English | MEDLINE | ID: mdl-9166866

ABSTRACT

Three novel splice site mutations and two novel missense mutations were identified by molecular analysis of pyruvate kinase (PK) deficiency associated with hereditary nonspherocytic hemolytic anemia. A Nepalese PK variant, PK Kowloon, was found to have a homozygous transversion at the 5'-splice site of the seventh intervening sequence (IVS) of the L-type PK gene (Ivs7[+1]gt --> tt). Using a reverse transcription polymerase chain reaction (RT-PCR) assay, we showed that the R-type PK mRNA in the proband's reticulocytes included the seventh IVS between the seventh and eighth exon, introducing a stop codon 3 nucleotides downstream of the mutated site. Consequently, the translational product may lack 44% of the R-PK polypeptide. A transition at the last nucleotide of exon 9 (1269GCG --> GCA) was found in a Japanese PK variant, PK 'Kamata.' The mutation did not alter the amino acid sequence, but caused skipping of the ninth exonic sequence in the R-PK transcripts. As a result, the affected R-type PK lost 51 amino acid residues (373Met-423Ala del). A transversion at the splice acceptor site of the third IVS (Ivs 3[-2]ag --> tg) was identified in PK 'Aomori.' The mutation resulted in aberrant splicing at a cryptic splice site within exon 4, causing deletion of two codons in the aberrant R-PK transcript (95 Gly-96 Pro --> del). Both PK 'Kamata' and PK 'Aomori' had a missense mutation on the other allele, 1044AAG --> AAT (348Lys --> Asn) and 1075CGC --> TGC (359Arg --> Cys), respectively. Although both 348Lys and 359Arg were located in the sixth loop of A domain (beta/alpha)8 barrel, which has been shown to contain the substrate and cation binding sites, the degree of anemia was much more severe in PK 'Kamata' than PK 'Aomori,' possibly because the 51 amino acid deletion of PK 'Kamata' but the 2 amino-acid deletion of PK 'Aomori' may abolish PK catalytic activity.


Subject(s)
Anemia, Hemolytic, Congenital Nonspherocytic/genetics , Pyruvate Kinase/genetics , Child , Codon/genetics , Female , Frameshift Mutation , Gene Deletion , Humans , Infant , Pyruvate Kinase/deficiency
10.
Pathology ; 29(2): 234-7, 1997 May.
Article in English | MEDLINE | ID: mdl-9213350

ABSTRACT

Recent updated models of the coagulation mechanism suggest that factor VII/thromboplastin complex is the main initiator or trigger of coagulation. Factor VII activation of factor IX is likely to be an important activation pathway. Inhibition of factor VII by tissue factor pathway inhibitor may have a regulatory role in the initiation of coagulation. Defining factor VII's interactions in coagulation physiology may lead to answers for some clinical problems in both hemostasis and thrombosis. We describe a 15-year-old Chinese boy with factor VII deficiency and a factor VII level of 0.08 U/ml. His symptoms were recurrent epistaxis and moderate delayed bleeding post-dental extraction. Such specific symptoms have been reported previously in a study of 40 European patients. It is one diagnosis to consider if a patients main symptom is significant post-dental bleeding. It is possible that there is requirement for higher levels of factor VII at these anatomical sites making them the common sites for symptoms in patients with moderate deficiency. Case reports of rare clotting factor deficiencies will illustrate what may be important in vitro functions of clotting proteins. Therefore reporting should be encouraged, especially during review and reconsideration of models of the coagulation mechanism.


Subject(s)
Blood Coagulation/physiology , Factor VII Deficiency/diagnosis , Adolescent , Factor VII/physiology , Factor VII Deficiency/congenital , Humans , Male , Tooth Extraction/adverse effects
11.
Blood ; 84(10): 3505-9, 1994 Nov 15.
Article in English | MEDLINE | ID: mdl-7949104

ABSTRACT

We identified four distinct point mutations in homozygous pyruvate kinase (PK) variants in Japanese and Chinese patients with chronic nonspherocytic hemolytic anemia. All gene abnormalities were missense mutations that caused single amino acid substitutions. 1261A (Q421K) and 1436A (R436H), which were identified in PK Sendai and PK Shinshu, had been found in unrelated Japanese and Amish PK variants, respectively. The clinical severity and extremely low residual erythrocyte PK activity of PK Shinshu as well as of the Amish PK might be caused partly by aberrant splicing, because the 1436A mutation changes a nucleotide at the last nucleotide in the exon 10. Recently, we diagnosed a 42-year-old Japanese woman with chronic nonspherocytic hemolytic anemia as having a homozygous PK deficiency. DNA sequencing of the variant PK gene showed a homozygous missense mutation at 1403GCT-->GTT, resulting in a single amino acid substitution from 468la-->Val. The gene mutation is likely to impair the allostericity of this enzyme, speculated from the tertiary structure. A homozygous missense mutation in PK Hong Kong, a boy of a non-Han southern Chinese minority group, was identified in exon 7 of the human L-PK gene, 941ATT-->ACT, resulting in a single amino acid substitution from 314lle-->Thr. The R-PK activity is expected to be severely affected, because the mutated amino acid residue is located between the 313 Lys and the 315 Glu, which are very important for acid-base catalysis and magnesium binding, respectively. Both the R- and M2-type PK were shown by polyacrylamide gel electrophoresis of the PK Hong Kong erythrocyte lysate, and this is the first report of a homozygous individual whose erythrocytes contain the immature (M2)-type isozyme.


Subject(s)
Anemia, Hemolytic/genetics , Point Mutation , Pyruvate Kinase/genetics , Adult , Amino Acid Sequence , Anemia, Hemolytic/blood , Anemia, Hemolytic/enzymology , Base Sequence , DNA Primers , Erythrocytes/enzymology , Female , Homozygote , Hong Kong , Humans , Japan , Male , Molecular Sequence Data , Pedigree , Polymerase Chain Reaction , Pyruvate Kinase/blood
12.
J Hand Surg Br ; 19(5): 576-83, 1994 Oct.
Article in English | MEDLINE | ID: mdl-7822913

ABSTRACT

13 patients who sustained high-energy crush or blast injury of the carpal bones were reviewed after a mean follow-up period of 30 months. These complex injuries resulted in unusual disruptions of the distal carpal row and adjacent metacarpals. Frequent involvement of the carpometacarpal (CM) joints and violation of the proximal carpal row were also demonstrated. Nine were open injuries, with the majority accompanied by significant soft tissue damage. Treatment included either closed reduction or open reduction and Kirschner wire fixation, and soft tissue procedures as indicated. In this series, the majority of the open injuries gave unfavourable functional results despite adequate carpal alignment. Several cases had disastrous outcomes related to associated vascular injuries. Closed injuries, on the contrary, followed a relatively benign course. Nevertheless, decreased grip strength persisted in both groups for a long time. Patients with such a complex carpal injury should expect a less favourable prognosis due to the severe nature of the trauma.


Subject(s)
Hand Injuries/surgery , Joint Dislocations/surgery , Metacarpophalangeal Joint/surgery , Multiple Trauma/surgery , Soft Tissue Injuries/surgery , Wrist Injuries/surgery , Adolescent , Adult , Female , Follow-Up Studies , Hand Injuries/etiology , Humans , Internal Fixators , Joint Dislocations/etiology , Male , Metacarpophalangeal Joint/injuries , Middle Aged , Multiple Trauma/etiology , Postoperative Complications/physiopathology , Rupture , Soft Tissue Injuries/etiology , Surgical Procedures, Operative/methods , Treatment Outcome , Wrist Injuries/etiology
14.
J Trauma ; 35(1): 28-35, 1993 Jul.
Article in English | MEDLINE | ID: mdl-8331709

ABSTRACT

Twenty cases of carpal bone dislocation were encountered during a 7-year period, with an average of 27 months of follow-up. There were ten types of dislocation in this series; the most common type was transscaphoid perilunate dislocation which was seen in nine cases. In addition, there were two scaphoid subluxations; one volar lunate dislocation; one dorsal perilunate dislocation; one scaphoid perilunate dislocation; one hamate and pisiform dislocation; one transhamate pisiform dislocation; one trapezoid dislocation with dislocation of carpometacarpal joints two to five; one dislocation of the trapezium, trapezoid, and carpometacarpal joints two to four; and two trapezium periscapholunate dislocations. Methods of treatment included open reduction, closed reduction, proximal row carpectomy, total wrist arthrodesis, and excision of the lunate. In this series, the patterns of dislocation were different for crushing injuries and dorsiflexion injuries. The clinical results associated with the soft-tissue injuries of the ipsilateral hand were mostly caused by crushing forces. Although carpal instabilities were noted, there was no significant correlation between the clinical and roentgenographic results in some of our cases. Best results invariably relied on a stable anatomic reduction and an adequate period of immobilization. Poor results were demonstrated in the cases with incomplete initial reduction, secondary degenerative arthrosis, or nonunion.


Subject(s)
Carpal Bones/injuries , Joint Dislocations/surgery , Adult , Bone Wires , Carpal Bones/surgery , Casts, Surgical , Female , Follow-Up Studies , Humans , Joint Dislocations/complications , Male , Middle Aged , Postoperative Complications , Prognosis , Wounds, Nonpenetrating/surgery
15.
Am J Nephrol ; 13(2): 142-8, 1993.
Article in English | MEDLINE | ID: mdl-8342581

ABSTRACT

Thalassemia is a common genetic disorder among the South Chinese. To see if thalassemia would adversely affect the erythrocyte response to recombinant human erythropoietin (rHuEPO, Epogen) in dialysis patients, the response to rHuEPO in 4 dialysis patients with thalassemic traits (thal-t) was compared with that of 4 control patients who were matched for age, sex, mode of dialysis and baseline hemoglobin levels over a 6-month period. Patients with thal-t showed a reduced erythrocyte response to rHuEPO compared to control dialysis patients as reflected by a reduced reticulocyte index, a slower rise in hemoglobin or hematocrit levels, requirement of a higher cumulated dose of rHuEPO to achieve a target hemoglobin of 10 g/dl and a higher maintenance dose of rHuEPO. A dialysis patient with hemoglobin H disease (HbHD) was also studied. He failed to respond to rHuEPO despite that the dose was increased to 250 U/kg/week. In contrast, his matched control dialysis patient, despite a lower baseline hemoglobin level (6.1 versus 8.8 g/dl), was able to reach a target hemoglobin level of 10 g/dl by 6 weeks and could be maintained at this level with 50 U/kg/week. The patient with HbHD had splenomegaly and a higher baseline serum erythropoietin level, reticulocyte count, serum bilirubin, serum ferritin and serum iron saturation than control patients and patients with thal-t. It was concluded that thal-t reduces the erythrocyte response to rHuEPO in dialysis patients and that in the presence of active hemolysis and enhanced endogenous erythropoietin secretion, dialysis patients with HbHD are resistant to treatment with rHuEPO.


Subject(s)
Anemia/drug therapy , Erythropoietin/therapeutic use , Kidney Failure, Chronic/therapy , alpha-Thalassemia/complications , beta-Thalassemia/complications , Adult , Anemia/etiology , Female , Humans , Kidney Failure, Chronic/complications , Male , Recombinant Proteins/therapeutic use , Renal Dialysis
16.
J Paediatr Child Health ; 28(4): 334-6, 1992 Aug.
Article in English | MEDLINE | ID: mdl-1497965

ABSTRACT

Three cases of pyruvate kinase (PK) deficiency resulting in congenital haemolytic anaemia with transfusion dependency are described. These cases resulted from consanguineous marriages in non-Han Chinese and include a pair of twins. We believe this to be the first documentation of homozygous PK deficiency in the Hong Kong population. The diagnosis was masked due to transfusion dependency in each case stressing the need to take a sample of pretransfusion blood for PK enzyme assay, and for family studies, when this disorder is suspected.


Subject(s)
Erythrocytes/enzymology , Ethnicity/genetics , Homozygote , Pyruvate Kinase/deficiency , Anemia, Hemolytic/etiology , Blood Transfusion , Consanguinity , Diseases in Twins , Female , Hong Kong , Humans , Infant, Newborn , Male , Pyruvate Kinase/genetics
17.
Nephrol Dial Transplant ; 7(2): 137-42, 1992.
Article in English | MEDLINE | ID: mdl-1314974

ABSTRACT

Seventeen patients with post-renal transplant erythrocytosis and 17 non-erythrocytotic controls, matched in age, sex, serum creatinine and source of donor kidney, were studied to determine the role of erythropoietin, male sex hormones (testosterone, FSH, LH), and various patient risk factors in post-transplant erythrocytosis. Serum erythropoietin was significantly greater in erythrocytotic patients (35.6 +/- 5.7 mU/ml) than non-erythrocytotic patients (18.8 +/- 2.6 mU/ml) (P less than 0.05) and normal subjects (22.5 +/- 0.95 mU/ml) P less than 0.05). Serum testosterone was similar between the male study (13.2 +/- 6.2 nmol/l) and control (13.1 +/- 6.0 nmol/l) patients. This might be due to the greater basal LH in the male control subjects (13.9 +/- 11.7 IU/l versus 8.0 +/- 3.3 IU/l in erythrocytotic males, P = 0.084). Basal FSH in the male controls was greater than that in the study group (13.7 +/- 14 IU/l versus 6.8 +/- 2.9 IU/l, P = 0.067). Among the demographic risk factors, only the smoking history was important. There were more smokers among the erythrocytotic patients than controls (P = 0.051).


Subject(s)
Erythropoietin/blood , Kidney Transplantation/adverse effects , Polycythemia/etiology , Adult , Female , Follicle Stimulating Hormone/blood , Humans , Luteinizing Hormone/blood , Male , Polycythemia/blood , Risk Factors , Testosterone/blood
18.
Clin Lab Haematol ; 14(3): 269-70, 1992.
Article in English | MEDLINE | ID: mdl-1451411
19.
Clin Lab Haematol ; 14(1): 53-68, 1992.
Article in English | MEDLINE | ID: mdl-1600694

ABSTRACT

The performance of leucocyte analysis on the Coulter STKS (Coulter, Hialeah, FL, USA) was evaluated for accuracy, precision and reliability. The results were compared with those obtained from visual examination of a Romanowsky stained blood film together with the automated WBC-diff. from the Technicon H*1 (Technicon, Tarrytown, NY, USA). The relationship between the number of cells counted per WBC-diff. and the WBC count of the sample was established. Precision of the STKS WBC-diff. was acceptable on blood samples with normal and low WBC counts. Correlation with an 800 cell manual WBC-diff. (n = 104) was excellent (r = 0.97, 0.97, 0.83, 0.98 and 0.53 for neutrophils, lymphocytes, monocytes, eosinophils and basophils respectively). Blood specimens, collected into dipotassium EDTA, could be stored at 20-25 degrees C for at least 8 h with no significant effect on the STKS WBC-diff. In a study of 513 patient samples, the BLASTS suspect flag gave 5.4% false positives and zero false negatives, the VARIANT LYMPHS flag gave 1.5% false positives and 0.4% false negatives, and the IMM GRANS/BANDS flag gave 30.8% false positives and 2.3% false negatives. Several instrument and sample related problems were encountered during this study. Despite these limitations, the STKS can provide efficient 5 part WBC-diffs. and effective screening for WBC abnormalities.


Subject(s)
Leukocyte Count/instrumentation , Blood Preservation , Evaluation Studies as Topic , False Negative Reactions , False Positive Reactions , Humans , Sensitivity and Specificity
20.
J Trauma ; 31(1): 110-2, 1991 Jan.
Article in English | MEDLINE | ID: mdl-1986114

ABSTRACT

The majority of high-pressure injection injuries can produce serious damage to the hand. Nevertheless, the injury may follow a relatively benign course if the injected substance possesses a less harmful nature. Treatment for these injuries requires immediate and aggressive surgery in most circumstances, but conservative treatment may be justified in certain instances. During a 4-year period, eight cases of high-pressure injection injury were encountered. The types of injected material were: four from paint, and one each from grease, water, benzene, and hydraulic oil. Time is an important factor regarding the results, while the types of injected material modify the clinical courses. It is advisable that the etiology of high-pressure injection injury should be established initially, and this factor be taken into consideration in choosing treatment options.


Subject(s)
Hand Injuries/etiology , Accidents, Occupational , Adult , Hand Injuries/diagnostic imaging , Hand Injuries/therapy , Humans , Injections , Male , Oils , Paint , Pressure , Radiography , Water
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