Medical Journal Of the Islamic Republic of Iran
Volume:18 Issue: 2, Summer 2004

Medullary thyroid carcinoma (MTC) occurs both sporadically and in the autosoma1 dominantly inherited multiple endocrine neoplasia (MEN) type 2 syndromes. The distinction between true sporadic MTC and a new mutation familial case is importantfor future clinical management of both the patient and family. The susceptibility gene forhereditary MTC is the RET proto-oncogene. DNA analysis for germline mutations ofthe RET proto-oncogene was performed in a series of 24 patients with MTC [apparentlysporadic MTC (20 cases), familial MTC (2 cases), MEN 2A (one case) and MEN 2B (one case)] to determine whether they were true sporadic cases or hereditary forms. Genomic DNA was amplified using polymerase chain reaction (PCR) and oligonucleotide primers for exons 10 & 11. The PCR products were examined by restriction enzymes analysis to detect the mutations. One of the 20 patients with apparent sporadic MTC had exon 10 mutation (Cys-620 Arg); and exon 11 mutation (Cys- 634 Trp) was also found in the index case with MEN 2A. No mutation was detected in the other patients. Three of six evaluated members of the MEN 2A patient had the same mutation. We conclude that routine application of RET proto-oncogene testing should be included in all cases of apparent sporadic MTC.

Oxidation of low density lipoprotein (LDL) particles plays a key role in the etiology of atherosclerosis and coronary artery disease (CAD). Oxidative stress enhances the likelihood of LDL oxidation and atherosclerotic plaque development. Paraoxonase (PONI) is an enzyme associated with HDL that metabolizes organophosphates and has antioxidant activity. In order to investigate the relationship between oxidative stress and the onset ofcoronary artery disease (CAD), total ferric reducing antioxidant power (FRAP)-as an index of antioxidant capacity of plasma- and the activity of PON 1 were measured in 80 patients over 65 and 80 patients less than 55 years old as late and early-onset CAD groups respectively. Plasma lipids were also determined. Patients with early-onset CAD had significantly lower serum levels of HDL-C (p<0.05) and higher LDL-C/HDL-C (p<0.01) than the late-onset group. This may imply the significance of HDL at the onset of CAD. There was no difference in serum levels of TG, TC, LDL-C, PON 1 activity and FRAP values between the two groups.The FRAP value was significantly lower than the reference range for healthy subjects in our laboratory. Although the FRAP value is lower in normal elderly people compared to the younger subjects, there was no difference between the two groups. This indicatesthat in young CAD patients, oxidative stress may be more important than in the elderly subjects and should be monitored in conjunction with routine lipid measurements.

Mitral valve prolapse (MVP) is a relatively frequent valvular abnormality. In both children and adults with MVP, an increased incidence of ventricular arrhythmias has been reported. QT dispersion, defined as the difference in duration between the longest QT interval and the shortest one, for a given set of electrocardiographic leads has been proposed as a sign of regional difference in cardiac repolarization and as a marker of increased risk of ventricular arrhythmias. This study was designed to compare the corrected QT dispersion (QTD) in children with isolated uncomplicated prolapse with a normal age-and sex-matched group of children. Twenty children with MVP (mean±SD age: 8 ±5 years) and thirty normal children enrolled in the study. Corrected QT dispersion was manually calculated on their surface electrocardiogram in all of them. QTD was 3 9± 14.7 and 40± 15.2 milliseconds respectively. An unpaired Student t -test was performed. There was no significant difference between the mean value of the two groups (p value.

Electroconvulsive therapy (ECT) is an efficient treatment for several neuropsychiatric disorders; however a large number of patients develop memory impairment after ECT. Different studies both on animals and human suggest that vasopressin has positive effects on memory and improves cognitive functions. In this randomized, double-blind controlled clinical trial, 50 patients with psychiatric disorders who were candidate for ECT were studied. In the control group (20 patients) who received normal saline, ECT resulted in impairment in several aspects of memory such as: immediate memory, short tenn memory, visual memory, associate learning and memory quality. The sub-scores for most of the sub-fractional aspects of memory by WMS tests were decreased significantly by the 2nd and the 3rd tests (p<0.00 I for most of the measured parameters). In the case group (30 patients), each patient received 60 Mg/day (in 3 doses) desmopressin as intranasal spray; during the ECT treatment most of the measured parameters were increased significantly by the 2nd and the 3rd WMS tests. There were significant differences between the two groups for most memory scores (p<0.000 I). The data demonstrate that desmopressin has protective and facilitating effects on memory impairment after ECT. Therefore, we conclude that desmopressin may be effective in the prevention of memory disorders by facilitating effects on memory and learning processes and may be used in patients who receive ECT for neuropsychiatric disorders.

In order to determine the role of different anti-phospholipid antibodies as an etiologic factor in recurrent pregnancy failure, a prospective study was done on one-hundred and thirty-eight women who had unexplained recurrent pregnancy loss (group I) with one-hundred well-matched women with normal reproductive outcome allocated as control group (GIl). Sera from 138 patients and 100 controls were analyzed for anticardiolipin antibody (ACLA) and lupus anticoagulant (LA). ACLA was measured by Elisa and LA by activated PTT. Sixteen women (1l. 6%) had positive ACLA in group I, while 3 (3%) of group II were positive for this antibody (p= 0.0 157 and odds ratio = 4.24). LA was positive in 12(8.7%) of group I and 3(3%) of group II, but the difference was not significant (p= 0.074, odds ratio=3.08). Overall 24 women (17.4%) were positive for one of the mentioned antibodies (p= 0.00055, OR= 6.81). Four patients were positive for both antibodies. This study emphasizes the relationship between antiphospholipid syndrome and recurrent pregnancy failure.

To determine the frequency of mumps infection in children hospitalized with aseptic meningoencephalitis (ME), as well as its correlation with parotiditis, this case-series study was conducted in the pediatric ward ofRasool Akram Hospital in Tehran, Iran, from 1 999 to 2001. T he blood samples of these patients were tested for specific IgM antibody for mumps by Elisa method. The results of this study showed that 62. 7% of children with ME were IgM positive, and the remaining (37. 3%) were IgM negative. There was a significant correlation between positive IgM and the presence of parotiditis. However, no correlation was noted between age, sex and season with IGM positivity except for CSF changes (aseptic meningitis) between the two groups. It can be concluded that the presence of neural symptoms (especially aseptic meningitis) in children less than 7 years of age with parotiditis could indicate mumps ME, especially in the spring. Due to a higher percentage of unvaccinated young persons «15yr old) in Iran, probably the incidence rate of mumps infection and its sequelae are higher compared with its incidence in developed countries before massive vaccination. Therefore with massive vaccination ofIranian young people «20yr old), the costs of mumps infection and its sequelae will decrease.

Inverted papillomas of the nose and paranasal sinuses are uncommon neoplasms, characterized by their tendency to occur and by their association with malignancy. In the past decade there has been a trend toward the use of endoscopic surgical techniques in the management of these tumors. This article presents the results of a prospective study of24 patients with inverted papilloma resected by the endoscopic approach. 21 patients had unilateral disease and three patients had bilateral involvement. None of the patients had orbital or cranial extension. No association with malignancy was seen. Following endoscopic surgery, four patients had recurrences that required surgery. No complication occured in any of the patients. The results of endoscopic surgery for inverted papilloma is much better than nonendoscopic transnasal approaches and compares favorably with radical extranasal approaches. Endoscopic surgery is an effective surgical option for inverted papilloma removal.

In this prospective descriptive study, all patients with acute lower limb ischemia who presented to Shohada Tajrish Hospital (STH) between July 200 1 and July 2003 were analyzed and followed for 6 months. The total population of 109 patients, with no significant difference in sexual distribution had a mean age of 64± 16 years. 23% had AF and 31 % had a history of MI, 'but in the ECG of 30%, no pathologic finding had been detected. Pain and coolness were the two most prevalent symptoms. Only onethird of the patients.had normal sensory and motor examination in their limb and the rest had different degrees of impairment. More than 70% of the patients arrived here after 12 hours from the onset of the symptoms. Fasciotomy was done in 29%, and amputation in 23.5%. The mortality rate was 12.2%. Surgical site infection occurred in 11 % and] 4.1 % underwent re-embolectomy. There was no pseudoaneurysms. The time interval from the onset, sensory and motor findings, and calf tenderness affected the prognosis. Prognosis was better in smokers. The iatrogenic group which consisted of 13.7% of our patient population had the best prognosis (no mortality or morbidity). 4% of the patients who had first undergone embolectomy in other centers, all lost their limbs. In cases of late embolectomies, venotomy and heparin flush has lowered the amputation level without increasing mortality. Early referral by a cardiologist, receiving heparin rapidly and smoking are among the factors which improve prognosis.

Early infantile epileptic encephalopathy or EIEE (Ohtahara syndrome; OS) is a kind of intractable seizure that begins in neonatal age with sudden onset of tonic spasms in series or single suppression-burst S-B in EEG.I Imaging shows anatomic defects such as migration disorders and generalized atrophy" with essentially normal metabolic tests. The seizures often change to West's syndrome (WS) or Lennox G austaut syndrome (LG S) pattern.3 Ohtahara et a1. first described this syndrome in 1976.3 We observed ten cases of this syndrome during a period of I 2 months. 70% of the patients were male. The mean age at the time of onset of seizures was 8 days; the mean age at the time of admission was 6.8 months. In 20% of cases the first seizures were generalized, in 80% tonic. EEG at the time of referring showed suppression-burst S-B in 50%, hypsarrhythmia in another and in 60% of them there were scattered sharp, spike or spike and wave in one or both sides. The drugs that have been used in mUltiple therapy were clonazepam 50%, prednisone 60%, sodium valproate 30%, nitrazepam 20%, vigabatrin 30% and acetazolamide in 10%. Response to treatment was good in 70% and moderate in 30%. At the end of the course all patients showed severe mental and motor retardation. Prognosis was shown to be poor in these patients.

Bacterial expression systems provide economic and logistic advantages in manufacturing proteins for human therapeutic purposes. However, most such proteins accumulate in insoluble biologicaIIy inactive form when overexpressed in bacterial cells. This is the case while attempting to produce recombinant human granulocyte-macrophage colony stimulating factor (hGM-CSF) in E. coli. In this report, different strong promoters are used to compare the yield of expression when the protein is expressed as insoluble and soluble forms. The results obtained indicated that the level of expression was independent of the nature of promoter used. Moreover experimental data presented here suggest that the fusion system which directs hGM-CSF to the periplasmic space not only can prevent inclusion body formation but also improve the level of expression significantly.

Telomerase activation is one of the main pathways to immortalize cancer cells. In many kinds of cancer cells, this special reverse transcriptase stabilizes and elongates telomeres and prevents telomere erosion that naturally occurs in every cell division. Esophageal cancer is the fifth most frequent cause of cancer death worldwide, and is highly associated with alcohol, smoking, cultural habits, and environmental factors. Telomerase has been suggested as a tumor marker and a molecular target for drug design in several kinds of cancers. In this work telomerase activation was inspected among Iranian patients with Esophageal Squamous Cell Carcinoma (ESCC), and detected in 90% of samples of different stages. This may be an indication that telomerase activation happens in an initial step in the development of ESCC. Although there is no correlation between telomerase activity and the progress of ESCC, it could be considered as a good tumor marker in ESCC. Telomerase activity tests are suggested for screening purposes in high risk areas for ESCC, which can be easily done on a small amount of scrapped samples of esophageal mucosa. It is also possible that ESCC results from incomplete differentiation or a failure in telomerase gene switching off that nonnally occurs during the differentiation of esophageal epithelial cells.

Our previous studies have demonstrated the relaxant, anticholinergic (functional antagonism), antihistaminic, and inhibitory effect of calcium channels of Nigella sativa on guinea pig tracheal chains. To investigate the other mechanism responsible for the relaxant effect of this plant, the stimulatory effect of Nigella sativa on J32-adrenergic receptors in tracheal chains of the guinea pig was examined in this study. The 􀁫2-Adrenergic effects of macerated and aqueous extracts from Nigella sativa, 50 nM propranolol, and saline were tested by performing the cumulative Log concentration-response curves of isoprenaline induced relaxation of precontracted isolated guinea pig tracheal chains with three different conditions including: non-incubated (group 1, n=8); incubated with I).lM chlorpheniramine, and 1).lM atropine (group 2, n=5). The effective concentration of isoprenaline, causing 50% of maximum response (ECso)' maximum response and the slope of isoprenaline curves obtained in the presence of extracts, and propranolol were compared with those of saline. The results showed clear leftward shifts in isoprenaline curves obtained in the presence of both macerated and aqueous extracts compared with that of saline in group 2. The EC50 obtained in the presence of aqueous extract in group 2 was significantly lower than that of saline (p<0.05). The maximum response obtained in the presence of aqueous extract in group 2 was non-significantly greater than that of saline. However, in group 1 experiments there was no significant difference between EC50 and maximum responses obtained in the presence of two extracts and saline, although isoprenaline curves obtained in the presence of both macerated and aqueous extracts showed clear rightward shifts compared to that of saline. Isoprenaline curves were obtained in the presence of propranolol in both groups of experiments. The EC50 obtained in the presence of propranolol was significantly greater than that obtained in the presence of saline and extracts in both groups of experiments (p<0.00 1 for all cases). The results of this study indicated a stimulatory effect of aqueous extract and a possible stimulatory effect of macerated extract from Nigella sativa on β2- adrenoceptors.

The life cycle of the American trypanosome, Trypanosoma rangeli, in its invertebrate host, Rhodnius prolixus, is completed by invasion into the insect salivary glands. As a prerequisite, there is an initial recognition of the salivary gland receptors for the adhesion/invasion by T rangeli. Therefore, the purpose of this study was to investigate the putative receptor molecules on either the salivary gland or parasites surfaces, which mediate the adhesion/invasion. Initially, the possible agglutination activity ofT rangeli epimastigotes against blood cell was examined. Then, in order to investigate the role of any receptor ligands on the surface of T rangeli in adhesion/invasion of the parasite, GlcNAc was preferentially used to isolate a ligand protein. A 60kDa protein bound to the GlcNAc column and was isolated from T rangeli (Tr-60) and the purity of this protein was confirmed by reverse phase HPLC. Tr-60 also exhibited a potent parasite inhibitory effect indicating that this protein (most probably a glycoprotein) may act as a ligand receptor. In vivo, the development and invasion of T rangeli into the salivary gland was not interfered with by the presence of a sugar inhibitor of the R. prolixus hemolymph lectin

Virulence of the opportunistic yeast, Candida albieans, involves the interplay of many complex changes including the yeast-hyphae transition, which mainly involves protein changes. Cell wall mannoproteins are found to be the main cause of adherence of C. albieans to epithel ial cells in the first step of an infection process. In the present study, cell wall mannoproteins of intact yeast were purified using a simple treatment of yeast with mercaptoethanol and sodium dodecyl sulfate followed by Concanavalin A chromatography. Both electrophoretic analysis of the column effluent and Western blot analysis using polyclonal and monoclonal antibodies showed the presence of mannoproteins with molecular weight in the range of30-50 kDa. Dot blot analysis of the purified antigen with the polyclonal and monoclonal antibodies prepared in this study showed that outer membrane mannoprotein antigens were obtained successfully following the above simple purification strategy

A 33-year-old female-a known case of Behcet's disease- was admitted to the hospital due to fever, dyspnea and hemoptysis. One week before admission, she had developed right leg swelling. The history suggested either deep vein thrombosis (DVT) and pulmonary emboli or pulmonary artery vasculitis. Diagnostic tests were requested and the necessary treatment was started accordingly.

There are occasional reports of using the appendix to replace sections of the ureters, and to our knowledge, only a couple of them were in the left side. We used the appendix as a ureteral substitute in two cases in technically difficult parts, the upper left ureter and the first part of the right ureter. Follow-ups of8 years and 6 months in right and left side cases, respectively, are impressive with maintenance of patency and improving renal function. We believe that the use of the appendix is a valuable alternative to substitute all segments of both ureters, with good results.

Paecilomyces sp, is a saprophytic fungus which has rarely been associated with human disease. We report the first case of invasive subcutaneous infection caused by this fungus in a 78-year-old female from Damghan, Iran, with diabetes and chronic renal failure. A biopsy provided an initial diagnosis of the mold in tissue. Multiple positive fungal cultures which were obtained from the biopsied tissue were subsequently identified by microscopic and macroscopic characteristics to be Paecilomyces sp. This case was successfully treated by oral ketoconazole (200 mg/day).

In this article, the authors introduce a case of Caroli's Disease (CD) according to the results of ultrasonography (U.S), abdominal computed tomography (CT scan) and hepatic biopsy that has been manifested in the neonatal period. In the routine examination of the neonate, abdominal protrusion was noticed. Then milk intoterance, inability of meconium passage and vomiting developed, during workup, a diagnosis of CD was confirmed. Although CD is rare in the neonatal period, it should be considered in the differential diagnosis of abdominal protrusion.