Medical Journal of The Islamic Republic of Iran (MJIRI)

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Two brothers with DIDMOAD syndrome are reported. The older brother has diabetes mellitus (type I), diabetes insipidus, optic atrophy, deafness and atonia of the urinary tract with severe symptoms such as diabetic ketoacidosis and frequent urinary tract infections. His younger brother had the same manifestations but with less severity. We report the findings of our two patients and compare them with the frequency of the symptoms in 100 patients from the literature.

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120 cases of Iranian soldiers complaining of hearing loss, were studied over a period of three years at the Ghaem Medical Center in Mashad,Iran. There was adequate information in III cases that their engagement in different types of explosions had caused their hearing impairment. The following aspects were evaluated: 1- Categorization of their hearing impairment. 2- The otoscopic findings. 3- The severity of the hearing impairment, the side of involvement and their relation to the type of explosion. This study while implying that war seems to be a major factor in causing hearing impairment, intends to clarify some of the cause and effect relations also. The hearing impairment caused by war can be of different types and severity and will be a real handicap. The abrupt loud impact noise and blast wave exposure are considered to be causes of hearing impairment. In ourseriesof III cases the different types of sensorineural hearing loss are more common than simple ruptured ear-drums.

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The new phase-II N-glucoside metabolites of butobarbitone and pentobarbitone are identified and quantified in human urine after single doses of these drugs. The identification and quantification of these metabolites were carried out by mass spectroscopy technique (accurate mass measurement and high voltage scanning). It was found that the butobarbitone N-glucoside comprised 26% of the dose with tl/2= 44h, while the pentobarbitone N-glucoside was 43% with t1/2= 40h.

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Metronidazole and furazolidone were compared for efficacy in 268 seven to 11 year old children infected with giardiasis. With metronidazole the cure rate was 82.3% and 80.0% in boys and girls respectively, while furazolidone showed an efficacy of74.4% and 71. 7% in the corresponding groups. The overall efficacy was 81.1 % with metronidazole and 73.3% with furazolidone. There were no significant differences between metronidazole and furazolidone efficacies in regard to the age groups or sexes.

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Isotherms of the binding of the anthracycIine antibiotic, adriamycin (adriblastin), to DNA histone complexes was studied by means of spectroscopic analysis. The results indicated that: (a) binding of adriamycin to histones reduced the interaction of histones with DNA, (b) binding of the drug to DNA did not change the binding affinity of histone to DNA and, (c) in the explored binding range of r

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The spontaneous regression of neuroblastoma to ganlioneuroma is a rare occurrence. Reviewing the literature, we found no more than 12casesreported.'•2.9.,o.'4.1s We report a case of metastatic neuroblastoma of multifocal skeletal and soft tissue areas with gross deformities which regressed to ganglioneuroma, with good prognosis.

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A young woman with no history of diabetes tried to commit suicide by injecting 2800 units of subcutaneous NPH insulin. She was transferred to Loghman Hospital within 12 hours. The main clinical symptom was continuous seizure activity which was resistant to all forms of routine drug therapy. The patient was treated with intravenous hypertonic glucose (50%) followed by continuous glucose (10%) infusion. The patient was discharged from the hospital after 8 days without any sequelae.

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Lead is known to be a potent neurotoxin that can cause seizure activity in humans and animals. Also, epileptic type convulsion is a known complication of hypothyroidism. This study was performed to determine the effect of propylthiouracil (PTU)-induced hypothyroidism and chronic lead exposure on pentylenetetrazol (PTZ)-induced kindling parameters. The test was carried out by using male albino rats weighing 180-230 g which were divided into four groups as follows: Group 1: rats which received distilled water for 25 days (control group) Group 2: rats pretreated with an orally administered lead acetate solution (0.05%) for 25 days Group 3: rats rendered hypothyroid with PTU solution (O.I%WIV) for 25 days and Group 4: rats co-treated with lead acetate solution (0.05% W IV) and PTU solution (0.1 % W IV) for 25 days. On the 26th day of the experiment PTZ (ip, 30 mg/kg) was injected once a day in order to induce seizure behaviors. Kindling parameters such as seizure latency, index, stage and frequency were evaluated. In animals, both lead acetate (0.05% WIV) and PTU (0.1 %WIV) pretreatment caused significant alterations in kindling parameters separately. But in animals pretreated with a combination of lead acetate and PTU, potentiation of kindling parameters was not observed compared to groups one and two. In conclusion, lead acetate and PTU may affect kindling parameters by different mechanisms.

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The goal of this study was to compare the effect of heater probe thermocoagulation for massive bleeding of peptic ulcers with a control group. Between March 1992 and August 1995 we used heater probe thermocoagulation endoscopically to treat 42 patients with active UGl bleeding or nonbleeding visible vessels at the base of ulcer craters within 2-3 hours of admission. We also selected 42 patients with active bleeding or non bleeding visible vessels who did not receive any endoscopic treatment but were instead treated conservatively as the control group. The energy applied to each of our patients in the heater probe group was 105±22.5 J (mean±SD). Rebleeding occurred within 2-5 days in 2 patients (4.7%) in the heater pro be group versus 9 patients (21.4 % ) in the control group (p= 0.05). Mean duration of admission in the heater probe group was 4.3±3.1 days versus 6.9±3.8 days in the control group which was comparable (p= 0.0027). There were no statistically significant differences between the two groups concerning transfusion requirement and mortality. Heater probe therapy was tolerated by the patients very well and no complications occurred. Heater probe thermocoagulation is an effective, safe and economical procedure for treating peptic ulcer bleeding.

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The effect of epinephrine on triacylglycerol secretion was investigated in isolated rat hepatocytes. The effect appeared at concentrations of more than 1 µM and reached a plateau at 10 µM. Epinephrine concentration for half of the maximal bioeffect (EC50) was about 1 µM. Epineplrrine at a concentration of 10 µM suppressed triacylglycerol secretion by 33% and increased its cellular content by approximately 18%. The total triacylglycerol content of the system (sum of the cell and the incubation medium) was constant at all concentrations of epinephrine. Time course experiments for triacylglycerol secretion exhibited relatively similar results on the basis of lipid analysis with or without lipid extractions. On the former basis triacylglycerol secretion versus time followed a linear relationship with a slope of 1.47 ± 0.11 µmole TG/3h/ g wet liver. Time course curves of cellular lipids revealed that cellular triacylglycerol and phospholipid contents in the presence of epinephrine were higher than the control at all time points, and the difference was constant during time. Furthermore, in the presence of glucose (20 mM) and oleate (0.25 mM), intracellular triacylglycerol content increased markedly (≥45%) whereas cellular phospholipid content remained constant. It is proposed that epinephrine exerts an inhibitory effect on VLDL secretion probably through blocking in the secretory pathway.

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The effects of alpha and beta-adrenergic stimulation on triacylglycerol secretion were investigated in isolated rat hepatocytes. Epinephrine within 3h of incubation suppressed triacylglycerol secretion by 35% and increased its cellular content by 18%. The inhibitory effect of epinephrine was abolished by inclusion of phentolamine and also prazosin but not with propranolol. Trifluoperazine concealed the inhibitory effect of epinephrine in a dose-dependent manner, whereas theobromine did not have any significant effect. The secretion of triacylglycerol was suppressed not only by the a-agonist phenylephrine but also by the β-agonist isoproterenol. Dibutyryl-cyclic AMP also inhibited secretion of triacylglycerol by approximately 30%. The results indicate that epinephrine suppressed triacylglycerol secretion via the α1-adrenoceptor whereas stimulation of beta-as well as alpha-adrenoceptors can exert a similar effect. Calcium-calmodulin dependent protein kinase may be involved in the down-regulation of VLDL secretion. The unexpected effect of isoproterenol has been discussed in relation to "dual signaling" and also the "store-dependent calcium entry" hypotheses.

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A retrospective study was done on 130 AML patients treated in Shariati and Imam Hospitals in Tehran from 1991 to 1997 to investigate the value of three post-remission methods of treatment. All patients who were in complete remission (CR) (Group I) had been treated with ARA-C (300 mg/m2/day continuous infusion for 5 days) and Daunorubicine (45 mg/m2/day for 3 days) as induction and early consolidation therapy. Forty patients were treated by additional similar chemotherapy as second consolidation and no further treatment was offered. Fifty patients (Group II) were treated by ARA-C (120 mg/m2 subcutaneously for 5 days), Etoposide (120 mg/m2 on day one), and Mitoxantrone (12 mg/m2 on day one) on each successive month as short-term miniconsolidation. Forty patients (Group III) were treated similarly to Group II until relapse for up to two years as long-term miniconsolidation. There was no difference in the three groups regarding mean age and other prognostic factors. Treatment related mortality and morbidity were also similar. Median duration of disease-free survival (DFS) was 36 (3.5-68 with 95% CI), 17 (12.5-21.5) and 19 (14.7-23.3) months respectively in these three groups. In a 14- month median observation there was no difference in DFS and overall survival (OS) among the three groups (p=0.7). We concluded that short- or long-term miniconsolidation chemotherapy compared to standard treatment does not improve DFS and OS in AML patients.

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Various biological bone g rafts have been used for osteogenes i s , osteoconduction and even osteoinduction, but due to systemic influences and local factors, the outcome of successful incorporation of a bone graft has not been satisfactory. This study was therefore conducted on 15 clinically healthy adult dogs between 1 to 3 years of age weighing 20 to 30 kg to evaluate the effect of bone cement and autogenous bone grafts. A piece of 3 cm complete mid-shaft right tibial cortical bone was removed in all animals. Then they were subsequently divided into three groups of 5 animals each. In group I the bone was fixed with a suitable intramedullary pin, whereas the gap was filled with harvested autogenous rib bone graft in group II animals. Bone cement was used to fill the gap in animals of group III for the first 30 days, then it was replaced with iliac bone chips and observation was made accordingly for 60 days in all animals of the three groups. After 60 days, before collection of callus samples, there was granulation and fibrocartilage tissue in groups I and II respectively, whereas a thick fibrous capsule or pseudomembrane layer was seen around bone cement on day 30 before removal of cement and on day 60 after replacement by iliac bone graft in group III animals. There were combinations of heavily impacted fibrous a n d fibrocartilagineous tissues with thick bundles of collagen fibers among hypertrophic chondroblasts in histomorphological cross section in group I animals, whereas active sequestra were present in group II animals. Fibrotic capsule, periosteal layer, lacunae, osteoblasts, osteocytes and well formed bone marrow with RBC and adipose tissue were noted in group III animals. It appears that the local reaction induced by bone cement leading to fibrotic capsule formation was quite helpful in delaying iliac bone graft resorption and enhancing osteogenic stimulation as to fill the gap with impacted callus with normal cortical structure. The application of bone cement in gap non:::union:::, crushed bone and fragmental fractures is therefore highly recommended.

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Up to 30% of Iranian adult multi-transfused thalassemic patients are infected with hepatitis C virus (HCV) which can intensify the progression of liver disease caused by iron overload in this group of patients. Our aim was to assess the biochemical and virological response of interferon alfa (INF-α) and its safety in thalassemic patients with chronic HCV infection. This trial was a single center, open label, single treatment prospective study of INF-a (Heberon alfa R, 3 MU, every other day) for a period of 12 months. 29 subjects, 13 to 56 years old (mean ± SD: 25.1 ± 10.4 years), whose serum HCV-RNA was positive and mean ALT remained greater than 1.5 times upper limit of normal in the last 6 months before the study were enrolled. A percutaneous liver biopsy was performed before treatment and all patients underwent monthly assessment for adverse events and monitoring of serum ALT. Qualitative serum HCV-RNA was obtained in months 3 and 6 and at the end of therapy. Pretreatment liver biopsy showed mild fibrosis in 33.3%, moderate fibrosis in 56.7% and cirrhosis in 10% of patients. Siderosis was severe in 14 patients (46.7%). Two nonsplenectomized patients discontinued INF becau'se of mild cytopenia, which resolved in less than one week after interruption of therapy. The following were some of the important adverse events observed during the study period: Flu syndrome in 29(100%), chills or fever>39°C in 14(48%), local pain in 14(48%), transient gastrointestinal symptoms in 13(44%), weakness in 5(17%), local induration in 3(10%) and edema in 2(7%) of the patients. By the end of 12 months of therapy, 15 patients out of 27 (55.6%) had a normal ALT and negative HCV-RNA (complete end-treatment response), they were followed up for a mean duration of 10.5 months (range: 6 to 22 months) and in 8 of them (53.3%) the condition relapsed (abnormal ALT with positive PCR). Viral clearance was a delayed event in our patients (29% by the end of month 3 and 63% by month 7) but ALT normalization occurred in 94% of responders by the end of month 3. Our experience indicates that the cure of HCV -related liver disease in thalassemic patients is not an unrealistic aim and may be achieved with a safe and inexpensive INF preparation (Heberon Alfa R) in a sizeable portion of cases. As opposed to non-thalassemic patients, in whom most viral responses happen in the first 3 months of therapy, in this group of thalassemic patients we found that maximum virologic response happened between 3 to 6 months of therapy. Although INF-a is an effective drug for initial treatment in thalassemic patients infected with HCV, its efficacy with the above dose and duration, for maintaining long term remission is under question.

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The authors describe a case of histiocyte-rich B-cell lymphoma (HR-BCL), a variant of diffuse large B-cell lymphoma, in a 51-year-old man. The patient presented with large axillary lymphadenopathy. Histopathologic and immunohistochemical examination of lymph node biopsy revealed diffuse effacement of the lymph node architecture by reactive histiocytes and neoplastic CD20 positive B cells. Reactive histiocytes were negative for CD15 and CD30 immunostaining. The final diagnosis was histiocyte-rich B-cell lymphoma (HR-BCL) and the patient was referred to the oncology clinic for treatment.

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Rotavirus nonstructural glycoprotein NSP4 can induce diarrhea in newborn mice. It has been suggested that NSP4 may be a key determinant for rotavirus pathogenesis and a target for vaccine development. In order to study the biological and morphological role of NSP4 a large amount of the purified protein and antibody against it are required. Simian rotavirus SA11 was propagated in BSCl cell, purified on cesium chloride gradients, and its genomic RNA was extracted. A cDNA from RNA segment 10 was synthesized and amplified by RT-PCR. cDNA fragment was cloned into plasmid vectors. The recombinant plasmid was characterized by restriction enzyme and sequencing. Construction of expression plasmid containing nsp4 gene was performed and expression of NSP4 was demonstrated by SDSPAGE, Western blot, ELISA and IF using polyclonal antibody against NSP4 from SA11 infected BSCI cells. A polyclonal antiserum against purified recombinant NSP4 was raised in rabbit which was reacted with NSP4 in BSCI cells infected with SA11 rotavirus. These results indicated successful expression of the full-length NSP4 in E.coli to produce antibody against it and to study its biological activities.

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An amplification polymerase chain reaction (PCR) test for the direct detection of Chlamydia trachomatis in urethral and endocervical swab specimens from symptomatic and asymptomatic women and men were compared to standard culture technique. During 6 months, 300 endocervical swab specimens from 205 asymptomatic women (64.4%) and 95 symptomatic women (31.6%), and 187 urethral swab specimens from 79 asymptomatic men (42.3%) and 108 symptomatic men (57.7%) attending the Gynecology Dept. and Genitourinary Clinic of Royal Victoria Hospital, Montreal, were collected. Processed specimens were cultured in McCoy cells and PCR was performed in a tube containing primer for C. trachomatis and internal control (IC). PCR products were detected by colorimetric and hybridization assay. Discrepant analysis for any specimens without unanimous results were performed by direct fluorescent antibody (DFA) or major outer membrane gene test (MOMP) with the 2SP medium sediment. In this study culture detected 13.1 % of asymptomatic and 33.6% of symptomatic infected women. By PCR, 16% of asymptomatic and 45.2% of symptomatic infected women exhibited positive results. By culture, 36.6% of asymptomatic and 45.3% of symptomatic men were positive, whereas 50.6% of asymptomatic and 51.8% of symptomatic men were positive by PCR. Sensitivity and specificity of PCR for asymptomatic and symptomatic women were 82.5% and 99.3%, and 89.5% and 97.8% respectively. Sensitivity and specificity of PCR for asymptomatic and symptomatic men were 93% and 100%, and 93.3% and 97.9% respectively. Sensitivity and specificity of culture for asymptomatic and symptomatic women and men were 67.5% and 100%, 66.6% and 100%,67.4% and 81.6%, and 100% and 100% respectively. The overall sensitivity and specificity of PCR and culture were 90% and 98%, and 75.6% and 100%. The internal control revealed that 3.9% of specimens were inhibitory, but when an aliquot of 10 fold dilution of these specimens was retested, 73.6% of them were non-inhibitory. In this study PCR exhibited higher sensitivity than culture for detection of C. trachomatis in both endocervical and urethral swab specimens and can be recommended for use in the c1inical laboratory.

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Familial colloid cyst of the third ventricle is very rare. This is one of the two largest families reported and the first in which all affected members are siblings. One asymptomatic sister was found by screening, emphasizing the value of screening. A brother and two sisters from a family consisting of three brothers and three sisters who were diagnosed as having colloid cyst of the third ventricle are presented. The index case like his sister underwent a tumor resection by middle frontal gyrus approach. Brain CT scan was performed for the other first degree family members for screening and colloid cyst was detected in another sister_ When two or more members of a family are affected, screening has an important value for detecting other asymptomatic members with colloid cyst of the third ventricle.

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Gastroesophageal-vul var leiomyomatosis is a very rare condition it is characterized by diffuse, ill-defined proliferation of smooth muscle in the esophagus and vulva. We present an interesting case of esophageal leiomyomatosis in a woman with a history of vulvar leiomyomatosis and a gall bladder full of stones. She was 18-years old and had a 4 year history of vulvar mass and clitoromegaly, with subclinical dysphagia. This paper represents the first reported simultaneous occurrence of these three pathologic entities in the English literature. Esophago-vulvar leiomyomatosis should be considered in a young patient with vulvar mass and long-standing dysphagia in whom a smooth, tapered esophageal narrowing on barium study and circumferential esophageal wall thickening on CT scan are seen. An esophagectomy combined with a reconstruction procedure is indicated.

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We evaluated the specific pattern of pre- and postoperative neurological signs and symptoms of cervical spondylotic myelopathy (CSM) to determine [mdings which had a predictive value for surgical outcome. Consecutive patients with CSM caused by osteophytic ridge or intervertebral disc herniation who underwent anterior cervical decompression and fusion in Loghman Hakim Hospital from 1999-2003 were prospectively enrolled. Patients were evaluated postoperatively by office visit. Outcome was assessed by objective neurological examination and scoring with multiple functional rating scales. Forty - three patients (30 male, 13 female) with a mean age of 48.8 years fulfilled our inclusion criteria. The most common preoperative symptoms were sensory deficit in distal upper limbs (88.4%), gait disturbance (69.8%) and sensory deficit of distal lower limbs (58.1 %). The most common signs were hyperreflexia (95.3%), Hoffman's sign (93%) and Babinski's sign (83.7%). Vertebral osteophyte and soft disc herniation were found in 86% and 14% of the patients, respectively. Overall functional improvement, evaluated by using a modification of the Japanese Orthopedic Association Scale was noted in 79.7% of the patients who had an abnormal scale preoperatively. Strength improved considerably and significantly after operation. However, less than half of the patients experienced functional improvement in the lower limbs, a discrepancy that was probably caused by persistent spasticity. Atrophy of the hand muscles, preoperative spastic gait and cord atrophy as shown in MRI were poor prognostic factors.