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مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources
مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources
مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources
مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources
مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources
مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources
مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources
مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources
Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    1-9
Measures: 
  • Citations: 

    0
  • Views: 

    306
  • Downloads: 

    0
Abstract: 

Background/Objective: To evaluate the efficacy of lamivudine alone and in combination with interferon in patients with chronic hepatitis B who were nonresponsive to previous interferon therapy.Methods: Thirty-six adult patients, who were previously nonresponders to interferon, were randomly divided into two groups. Group I received lamivudine 100mg daily for nine months, and Group II received lamivudine 100mg daily for nine months in addition to interferon 5-10´106 u three times weekly for 6 months. Patients were followed up for another 6 months without receiving any medication.The end points were; normalization of liver enzymes, sustained negative DNA and reduction of at least 2 points in the necroinfiammatory scores of the liver biopsies on the basis of the modified Knodell HAl system.Results: In Group I, 55.6% had sustained negative DNA (P=0.001). The mean AST value of 75.22±64.14 ull before treatment decreased to 28.28±13.19 u/l after treatment (P=0.01). The mean ALT value of 104.22±92.96 u/l before treatment decreased to 31.22±20.10 u/l after treatment (P=0.005). The histological response was 38.8% (P=0.003). In Group 11, 38.9% had sustained negative viral DNA (0.004). The mean AST value of 67.29±53.07 prior to treatment decreased to 26.88±15.85 u/l after treatment (P=0.008). The mean ALT value of 98.24±75.92 u/l prior to treatment decreased to 28.06±21.53 u/l after treatment (P=0.002). The histological response rate was 27.7% (P=0.004). The P values were not significant comparing the post treatment AST, ALT, sustained negative HBV DNA and histological responses of the two groups.Conclusions: It is concluded that both groups had a relatively good response rate with no statistically significant difference between the two groups. The adverse effect was more prevalent in Group II. It is suggested that chronic hepatitis B nonresponders to interferon should be treated with lamivudine therapy alone.

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Author(s): 

SHOKRGOZAR M.A. | SHOKRI F.

Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    10-15
Measures: 
  • Citations: 

    0
  • Views: 

    394
  • Downloads: 

    0
Abstract: 

Background: A proportion of healthy adults and neonates do not respond to vaccination with hepatitis B surface antigen (HBsAg ). Re-vaccination induces a protective antibody response in a minor proportion of non-responders.Objective: To study the immunogenicity of primary and secondary vaccinations with recombinant hepatitis B vaccine in healthy adults, and to compare the results with those who obtained the vaccine only in the neonatal period. Methods: Ninety-one healthy adults were immunized with triple doses of recombinant hepatitis B vaccine given at 0, 1 and 6 months intervals. The responder individuals were arbitrarily classified into low and high responders, based on the anti-HBs antibody titer. The non-responder subjects were subsequently re-vaccinated with 2-5 additional doses of the vaccine. Anti-HBs antibody and HBs antigen were detected by a sandwich ELISA, and anti-HBc antibody was detected by a competitive ELISA.Results: A protective level of anti-HBs antibody (> 10 lU/L) was developed in 93.4% (85/91) of the vaccinees following primary vaccination. Only one of the non-responders (16.6%) seroconverted and developed low titer of anit-HBs antibody after administration of additional 3-5 vaccine doses.Conclusion: Our results suggest that unresponsiveness to hepatitis B virus (HBV) may be differentially regulated in adults as compared to neonates. 

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    22-27
Measures: 
  • Citations: 

    0
  • Views: 

    352
  • Downloads: 

    0
Abstract: 

Background: Tumor necrosis factor-alpha (TNF-a) is a determining factor in macrophage activation and direction of immunologic mechanisms to BCG. By regulating the rate of transcription, allelic polymorphism in the regulatory regions of TNF-a gene can affect the host's ability in BCG containment.Objective: To study the prevalence of G to A transition polymorphism at position -308 of the TNF-a gene in Iranian BCG vaccinees with lymphadenitis.Methods: In this study, we determined the polymorphism at position -308 relative to the transcription initiation site of TNF-a gene in 40 patients with BCG adenitis and 42 healthy age-matched infants without reactions by a method based on allele specific PCR. The results of PCR were confirmed by the SSCP method.Results: The frequency of TNFA2 allele in the patient and the control groups were 0.013 and 0.04, respectively. Statistical analysis showed no significant association of TNF-a promoter polymorphism with susceptibility to BCG adenitis. However, there was a threefold increase in the frequency of TNFA2 allele in the control subjects.Conclusion: We suggest that the lower frequency of TNFA2 allele in patients might have resulted in weaker immune response that allows bacterial burden and occurrence of lymphadenitis.

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Author(s): 

AMINIAN B. | JAMALI K.

Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    28-32
Measures: 
  • Citations: 

    0
  • Views: 

    261
  • Downloads: 

    0
Abstract: 

Background: Echocardiography is a valuable tool for detection of pulmonary hypertension (PHT) but little work has been done to compare the accuracy of this non-invasive method with invasive direct trans-catheter pulmonary artery pressure (PAP) measurement. The aim of this study is to compare the accuracy of different echocardiography methods in detection of PHT.Material and Methods: In a prospective double blind study 49 patients (female: 33 and male: 16) with an age range of 14-68 years (39:t 12) and various cardiovascular disorders were selected. Tricuspid regurgitation (TR) gradient was measured by continuous wave (CW) doppler, pulmonary artery acceleration time (PAT) by pulse wave doppler, and PA a-dip evaluated by M-mode echocardiography. Data analysis was performed by SPSS software.Results: Among 46 patients with measurable PAT, 22 had PAT < 100 ms, and 24 had values> 100 ms. In the group with PAT < 100 ms (n=22), 18 had trans-catheter measured mean pulmonary artery pressure (PAP) >20 mmHg while it was < 20 mmHg in four patients. Among 24 patients with PAT> 100 msec, in 10 patients measured mean pulmonary artery pressure (MPAP) was <20 mmHg, and in 14 patients, MPAP was >20 mmHg. (P=0.024, sensitivity: 60%, specificity: 75%). With respect to TR gradient (n=28), in 21 individuals trans-catheter measured MPAP was >20 mmHg; and in seven patients it was <20 mmHg (P < 0.0002, sensitivity: 93%, specificity: 98%). In 30 patients with demonstrable PA a-dip, it was present in 20 and absent in 10 patients. In the PA a-dip present group (n=20), 10 patients had MPAP >'20 mmHg, and in the rest of the patients MPAP was <20 mmHg. In the PA a-dip absent group (n = 10), seven patients had MPAP> 20 mmHg, and 3 patients had MPAP< 20 mmHg (P: 0.297, sensitivity: 58%, specificity: 23%). A proposed formula for estimation of PAP through TR gradient was suggested. Conclusion: This study shows that TR gradient is a non-invasive measurement for detection of PHT, with high grade of catheterization correlation, PAT is moderately specific, while M-mode PA a-dip measurement is neither sensitive nor specific.

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    33-36
Measures: 
  • Citations: 

    0
  • Views: 

    278
  • Downloads: 

    0
Abstract: 

Background: Primary atrial septal aneurysm (ASA) is a congenital malformation of the atrial septum which is characterized by its bulging into either atrium. Transesophageal echocardiography (TEE) has recently made it possible to detect ASA more easily and has clarified some of the previously obscure points regarding this infrequent entity. Objective: Our aim is to report the prevalence of ASA among 270 patients undergoing TEE in this center and to assess their clinical findings and associated cardiac disorders.Design: Cross sectional study.Setting: University Hospital.Methods: Over a 2-year period, 270 cases had TEE, of whom, 16 cases were diagnosed as having primary ASA. A protrusion of atrial septum ³10 mm beyond the plain of atrial septum and a base diameter of ³ 15 mm were considered diagnostic for ASA.Results: There were 8 female and 8 male patients with an age range of 19-67 years. The maximal protrusion of ASA ranged from 11-22 mm and the maximal base diameter ranged from 17-25 mm. Oscillating ASAs were present in only 3 cases and in the remaining it was bulging only toward the right atrium. Systolic click was detected in 10 cases, of whom 2 had double clicks. Two patients had documented embolic stroke by history and physical examination and brain CT scan. The most common transesophageal echocardiographic findings included ASD (26%), PFO (26%) and MVP (20%). Of interest was the presence of ostium primum defect in one of our ASD cases. Mild mitral regurgitation and AR were each detected in one case too. Three cases (20%) had no associated cardiac lesions. Conclusion: Primary ASAs are rare, however once present, they are frequently associated with ASD and PFO. In addition they may not only simulate MVP due to their ausculatatory findings but may actually be associated with it.They should be considered as a potential source for embolic stroke as well.

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    37-40
Measures: 
  • Citations: 

    0
  • Views: 

    266
  • Downloads: 

    0
Abstract: 

Background: Behcet uveitis ia a blinding disease with no standard treatment. New medications with less side effects are being evaluated for this disease.Objective: This study was done to evaluate the effect of sulfasalazine on uveitis in Behcet disease. Method: This study was conducted in Behcet Research Clinic of Shiraz, Nemazi Hospital. A clinical trial was carried out for 15 months to observe the efficacy of two therapeutic regimen; regimen I: sulfasalazine + low dose prednisolone (PO) and regimen II: cyclophosphamide + regular dose of Prednisolone (PO) in the treatment of uveitis in Behcet disease. 13 patients received regimen I and 22 patients received regimen H. After at least 3 months, 11 patients in the second group who had no response to regimen H were switched to regimen I and reevaluated for response to therapy. These patients were followed for at least 12 months after starting the trial and were followed every 3 weeks by a vitreoretinal specialist and a rheumatologist.The eye examination included visual acuity (V/A), slit lamp examination for reaction, indirect ophthalmoscopy, IOP and when needed fleurecin angiography (F/A) for suspected posterior segment leisions. Uveitis was classified according to the Uveitis Scoring System. Results: of 13 patients who were initially on regimen I, group A (n= 13)85% (11 patient) had good to moderateresponse during 2-8 weeks. Only 50% of the patients who were on regimen H group B (n=22) had good to moderate response.Therefore, the remaining 50% were selected as group C (n= 11) and were put on regimen I. all of This entire group had favorable response to medication.Conclusion: Sulfasalazine 1.5-3 g/day with low dose prednisolone (PO) (0.5 mg/Kg) could be a good substitute for the treatment of uveitis in Behcet disease.

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    41-47
Measures: 
  • Citations: 

    0
  • Views: 

    241
  • Downloads: 

    0
Abstract: 

Background: Head injury is a major worldwide health problem and may cause metabolic disruption toward increased energy expenditure and protein catabolism. In head-injured patients, inadequate nutritional support can cause impaired healing, increased tendency to infections, multiple organ failure and poor recovery and outcome.Objective: To compare the effects of parenteral-enteral nutrition versus enteral support alone on complications, recovery and final outcome.Methods: A prospective quasi-experimental, randomized, non-blinded study was performed on 66 purely head-injured patients (55 males and 11 females, aged 18-72 years) with 24-hour-admission peak Glasgow Coma Scale (GCS) scores of 4 to 10. Patients were randomly divided into two groups; Group 1 received total parenteral nutrition, gradually changing to enteral nutrition, and Group 2, received enteral nutrition alone. They were studied for 14 days in hospital and followed after 3 and 6 months post-injury by Glasgow Outcome Score (GOS).Results: GCSs on the 14th day and GOSs three months post-injury were significantly higher in Group 1 than Group 2 (p=0.023, p=0.039, respectively), but GOSs after 6 months were not significantly different. Coma duration and mortality rate revealed no statistically significant difference between the two groups. While a statistically significant difference was found in complication rate and the total WBC counts between the two groups, the lymphocyte count was significantly higher in Group 1 (p < 0.001). Anthropometric indices were not statistically significantly different, except for the percentage of body weight loss and the difference between the weights of the 1st and the 14th days, which were significantly higher (P < 0.001). Group 1 received a higher calorie intake (36.07±3.55 cal/kg/day vs. 30.86±5.42 cal/kg/day in Group 2, p<0.001), and had a higher cumulative calorie balance (83.38±6.36% vs. 67.49± 9.54%, p<0.001). Mean protein intake in Group 1 was 1.35±0.15 gm/kg/day, as compared to 0.98±0.18 gm/kg/day in Group 2 (p < 0.001). Conclusion: Head-injured patients can receive more calories and proteins via the parenteral-enteral route. Recovery occurs more rapidly in these patients with better early nutritional support, but long-term follow-up reveals no statistically significant difference in the effectiveness of each nutritional regimen on the final outcome.

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    48-50
Measures: 
  • Citations: 

    0
  • Views: 

    279
  • Downloads: 

    0
Abstract: 

Background: Tracheal defect repair is a challenging issue in surgery. A variety of prostheses have been used with little success.Objective: To investigate the suitability of aorta for tracheal repair.Methods: On the cervical trachea of 11 dogs we made a 5 cm long tracheal defect in the left posterior embranous portion. It was repaired by harvested infrarenal aorta and the aorta was replaced by Dacron graft. One dog died of heart failure, 8 were sacrificed 2 months and two 6 months after the procedure.Results: Gross and microscopic study showed patent lumen with excellent re-vascularization of the aortic wall and ciliated columnar epithelial growth endoluminally.Conclusion: Aortic graft is preferable to other substitutes because of its lower antigenicity, lower vascularities, as well as the absence of mucous secretions and peristalsis.

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Author(s): 

IZADPANAH A. | PAYRAVI S.A.A.

Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    51-54
Measures: 
  • Citations: 

    0
  • Views: 

    332
  • Downloads: 

    0
Abstract: 

Background/Objective: Postoperative peritoneal adhesion bands (PABs) are one of the most common complications of laparotomies. Approximately two - third of all intestinal obstructions are caused by adhesion bands.The use of methylene blue (MB) for prevention of these adhesions has been postulated on account of inhibitory effect of MB on oxygen for the transfer of electrons from flavo-enzymes primarily xantine oxidase.Methods: In this study 6 groups of guina pigs (n=20 in each group), laparotomy and induction of adhesion was performed in, then MB was administered intraperitoneally, at 0.5, 1,5, 10 or 20 mg/kg to experimental groups. Control group did not receive MB. After 2 weeks they were sacrificed and their PABs was graded by Nair classification.Results: MB at 0.5 mg/kg reduced the formation and severity of PABs significantly (p<0.005). However, at 1 and 5 mg/kg the PABs were not reduced (P<0.306 for G3 and P<0.219 for G4). At high doses of 10 and 20 mg/kg MB was lethal to 80% and 100% of the animals, respectively.Conclusion: In conclusion, MB might be able to prevent PABs at low dose(0.5 mg/kg) in guina pigs. However, at high doses (³10 mg/kg) it was lethal. 

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    55-61
Measures: 
  • Citations: 

    0
  • Views: 

    312
  • Downloads: 

    0
Abstract: 

Background: Sodium valproate, an anticonvulsant drug, has been reported to stimulate the replication of human immunodeficiency virus Type 1 and human cytomegalovirus.Objective: In view of the fact that epileptic patients receiving this drug may become infected with. different viruses, we investigated the effect of sodium valproate on the replication of other viruses especially those affecting neuronal tissues, such as measles virus. The viruses used in our studies include measles virus, poliovirus Type 1 and coxsackievirus B3.Methods: The replication of measles virus was studied by plaque assay and that for poliovirus Type 1 and coxsackievirus B3 was performed by quantal response method. Electron microscopy was used in evaluation of cell monolayers infected with measles virus.Results: The results obtained showed that sodium valproate stimulated both measles virus (p < 0.001) and poliovirus Type 1 replication (p<0.01) and had no effect on coxsackievirus B3 infectivity. Electron microscopic studies showed that measles virus nucleocapsids were more abundant in drug treated infected cells as compared with untreated infected control monolayers. The sparsity of intranuclear and intracytoplasmic inclusion bodies was also a prominent feature of cells exposed to the drug and infected with measles virus.Conclusion: The present preliminary investigation should be assessed by appropriate in vivo studies. Until then caution is to be exercised in prescribing sodium valproate to the patients with neurologic disorders due to infection with measles virus. This is particularly important with respect to the developing countries where measles infection is of higher incidence and severity. 

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    62-65
Measures: 
  • Citations: 

    0
  • Views: 

    287
  • Downloads: 

    0
Abstract: 

Objective: To study the course of events in rabbit eyes exposed to Dieffenbachia plant sap and effect of juice type on the severity of injury.Methods: Twenty-six rabbits (51 eyes) randomly divided into four groups and exposed to four types of juice (leaf juice, stalk juice, stem free running and stem squeezed extract). The eyes were examined in ambient light and by slit lamp, until the sixth week. For histopathologic examinations; the corneoscleral buttons were stained by hematoxylin and eosin and examined by light microscopy.Results: The most severe clinical manifestations were seen with the juice of stem. The most common findings in all groups were corneal epithelial erosions and needle shaped stromal crystals, that gradually disappeared spontaneously. Corneal edema was only seen in eight cases of stem juice exposure. It was the best factor related to developing corneal vascularization (p.v=0.0002). Permanent corneal vascularization and scarring was seen in four cases, all exposed to stem juice. Conclusion: Most of ocular injuries caused by dieffenbachia sap are reversible, except for corneal vascularization and scarring which is caused by the sap of mainstem and is highly associated with corneal edema, mandating extensive ophthalmic care upon exposure.

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    66-70
Measures: 
  • Citations: 

    0
  • Views: 

    604
  • Downloads: 

    0
Abstract: 

Background: The present study was designed to investigate the relationship between the Persian Adult Reading Test (PART) and current intellectual ability in a group of elderly with and with out memory problems. Objective: Reading short and irregular words is the last ability preserved in dementia and brain degenerative diseases This ability shows the premorbid IQ of patients with memory deficit.Methods: Seventy-five subjects aged above 55 years (23 males and 52 females), visitors of 3 available elderly community houses in Shiraz, Iran, were requested to take part in the study. Four measures of cognitive ability were utilized: 1- The verbal paired association learning task (a sub-test of Wechsler Memory Scale) with delay recall; 2- Computing a series of mental arithmetic; 3- A task of reading 50 Persian words (PART); 4- Finally, they were requested to give response to Raven Standard Progressive Matrices (RSPM) as a measure of general intellectual ability (i.e., IQ).Results: Positive and significant correlations between PART scores and RSPM (r=0.36 for total sample; r=0.46 for males; r=0.35 for females), and PART and mental arithmetic task (r= 0.36 for total sample, r=0.44 for males; r=0.33 for females) were obtained. There were no significant differences between elderly subjects suspected of having dementia and those without memory deficit in PART scores (p=0.36) while the two groups were significantly different in RSPM scores (P<0.0001).Conclusions: These findings suggest that PART could be valid for the estimation of premorbid intellectual ability in elderly people, but further study on clinically diagnosed subgroup of dementic patients is required.

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    71-73
Measures: 
  • Citations: 

    0
  • Views: 

    244
  • Downloads: 

    0
Abstract: 

The need for an easily available and cost-effective treatment for stress urinary incontinence (SUI) of women in the developing communities is a challenging issue for the urologist. We have attempted to determine the efficacy of periurethral autologous- fat injection in the treatment of female stress urinary incontinence (SUI).from October199 5to December 1998a total of 22 women, 10 with Type III SUI and 12 with Type I SUI underwent periurethral autologous fat injection. Pre-operative and follow-up evaluations consisted of filling out appropriate questionnaires, vaginal examination, stress test, subjective incontinence scoring, subjective estimation of the quantity of urinary leakage and urodynamic determination of abdominal leak point pressure (ALPP). Fifteen to 35 ml of fat was harvested from the anterior abdominal wall by liposuction and injected periurethrally under direct cystoscopic vision.Injection was done once in 20 patients and 2 patients received two injections. Follow-up period ranged from 13 to 50 months (mean 31 months). Out of 10 patients with Type III SUI, 4 (40%) were cured, 4 (40%) improved and 2 (20%) incurred failure, giving a total success rate of 80%. Out of 12 patients with Type I SUI only 1 (8.3%) was cured, 5 (41.7%) noticed improvement and 6 (50%) failed. The overall success rate in this group was 50%. The total success rate in the 22 women was 63.6 %. There were no major complications.Autologous fat can be a safe and cost-effective material for injection therapy of SUI. It was shown to be an effective alternative method in the treatment of Type III SUI in female, our results in Type I SUI were, however, disappointing.

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    74-76
Measures: 
  • Citations: 

    0
  • Views: 

    249
  • Downloads: 

    0
Abstract: 

Reference strains of Leishmania tropica and Leishmania major were recovered from cryopreservation on NNN medium, and adapted to serum-free brain-heart-infusion (BHI) medium. The cultures were centrifuged and supernatants were kept at -200°. The protein concentrations of the culture supernatants and extracts were determined and subjected to 20% SDS-PAGE with molecular weight markers. Presence of protein with MW of 63kDa was seen in samples of L. major and L. tropica with no proteins detected in the BHI-only medium. It is not known if the biological functions of the soluble 63 kDa protein are different from intact GP63 as this molecule has been a candidate for vaccine production.

Yearly Impact: مرکز اطلاعات علمی Scientific Information Database (SID) - Trusted Source for Research and Academic Resources

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Author(s): 

SALABIAN M.J.

Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    77-78
Measures: 
  • Citations: 

    0
  • Views: 

    295
  • Downloads: 

    0
Abstract: 

The disturbingly rising trend of pancreatic carcinoma (PC) has been an impetus for a growing number of chemotherapy trials in recent years. We report the preliminary results of a novel clinical evaluation of mitomycin + capecitabine in 11 patients with advanced Pc. An encouraging 63% overall response rate including two complete remissions and a limited manageable toxicity profile were noted.

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Author(s): 

IMANIEH M.H. | KARAMIZADEH Z.

Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    79-81
Measures: 
  • Citations: 

    0
  • Views: 

    225
  • Downloads: 

    0
Abstract: 

A 9-year-old boy with Wilson's disease and a combination of three uncommon clinical manifestations (cataract, gallstones and bone lesions) without significant CNS and liver involvement is described. The most common presentations of Wilson's disease are different types of liver involvement, CNS manifestations and hematologic disorders.Review of literature shows association of Wilson's with different presentations but to the best of our knowledge this patient is the first case of Wilson's disease with a combination of three uncommon features.Physicians should consider Wilson's disease as a differential diagnosis when encountering these features singly or in combination.

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    82-84
Measures: 
  • Citations: 

    0
  • Views: 

    258
  • Downloads: 

    0
Abstract: 

The prevalence of autoimmune disorders is increased in Down's syndrome. The most common autoimmune diseases associated with Down's syndrome, is related to the thyroid gland. Other autoimmune diseases including diabetes mellitus and hyperparathyroidism have also been reported. To our knowledge only two cases with insulin dependent diabetes mellitus and hyperthyroidism associated with Down's syndrome have been reported previously in the literature. The two reported cases had no ocular complications. We describe a 9 year old girl with Down's syndrome who developed diabetes mellitus six months prior to admission. Physical examination revealed bilateral exophthalmus, Mongolian face, a grade 3 goiter and moist skin. Laboratory data revealed blood sugar 731 mg/dl, TSH 0.01 mu/ml, T4 18.1 mg/dl and T3 481 ng/dl. She subsequently developed also cataracts. We suggest that children with Down's syndrome should be evaluated for endocrine diseases and ocular complications.

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Issue Info: 
  • Year: 

    2001
  • Volume: 

    26
  • Issue: 

    1-2
  • Pages: 

    85-87
Measures: 
  • Citations: 

    0
  • Views: 

    263
  • Downloads: 

    0
Abstract: 

A 15-year-old man was referred to our department with multiple lesions on the scalp, neck, abdomen, and back which -were proved to be basal cell carcinomas. The patient had a history of craniotomy at 3 years of age for exision of a cerebellar medulloblastoma, followed by cranioaxis irradiation post-operatively. On physical examination, hypertelorism, broad nose bridge and a slightly bulged out chest cage were noted. Chest X-ray revealed deformity of the chest wall. A diagnosis of nevoid basal cell carcinoma (NBCC) syndrome was made.

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