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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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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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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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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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.

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 ABSTRACT

 Background: Combination therapy with interferon and ribavirin is the most effective treatment for chronic hepatitis C today. The aim of this study was to evaluate the efficacy and safety of thrice-weekly Heberon (interferon alfa-2b) in combination with ribavirin as first -line treatment of chronic hepatitis C.

 Methods: A total of97 treatment-naive patients received Heberon three million units thrice-weekly subcutaneously in combination with ribavirin for 12 months. Serum HCV RNA levels were measured before and during therapy and 6 months after the end of therapy. End-of-treatment and sustained virological responses was defmed as an undetectable HCV-RNA level at the end of treatment, and 6 months after treatment was completed (end of follow-up), respectively.

 Results: In an intent-to-treat analysis, HCV-RNA was undetectable at the end of treatment in 49.5% of patients. At the end of follow-up, sustained virological response was 36.1 %. Combination treatment was generally well tolerated. Six patients stopped therapy because of side effects: severe cytopenia (n=4), depression (n=1), and hyperthyroidism (n= 1 ). Common side effects of therapy include: Flu-like syndrome (85.6%), generalized alopecia (41.2% ), injection site inflammation (37.1% ), mood changes (36% ), anorexia (34%) and weight loss (32% ).

 Conclusion: Heberon as an IFN product in combination with ribavirin for treat-ment of patients with chronic hepatitis Cis relatively safe, feasible, and potentially efficacious. It has comparable results in achieving end-of-treatment and sustained virological responses in chronic hepatitis C.

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 ABSTRACT

 Background: The loop colostomy is one of the most popular techniques used as a protective maneuver for a distal anastomosis and/or temporary fecal diversion. We are introducing the use of a full thickness skin bridge under the large bowel instead of a glass rod which alleviates problems such as protrusion of the large bowel, retraction of the bowel into the abdomen after removing the rod and hindering proper application of a colostomy bag over the stoma.

 Methods: Seventeen patients needing double barrel colostomy for complete diversion of fecal material were selected using loop colostomy with skin bridge. Three patients had Fournier's gangrene and 14 had penetrating rectal injury. Omega loop colostomy with a full thickness skin bridge was performed for fecal diversion.

 Results: All of the 17 patients had gas passing and full passage of fecal material within 3 days postoperatively. No case of skin necrosis and stitch abscess was encountered. No parastomal hernia or large prolapse was noted until healing was completed and patients were discharged and after at least 8 weeks and in Fournier's gangrene somewhat longer, the loop colostomy was closed without the need for formal laparotomy and without any case of anastomotic leak.

 Conclusion: In this study we confirmed that diverting loop colostomy using a skin bridge is a safe, rapid and easy to manage colostomy technique which gives complete diversion similar to double barrel colostomy without the need of performing a laparotomy for closure of the colostomy.

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 Abstract

 Background: The aim of this study was to determine the auditory performance of congenitally deaf children and the effect of cochlear implantation (CI) on speech intelligibility.

 Methods: Aprospective study was undertaken on 47 children in a pediatric tertiary referral center for CI. All children were deaf prelingually and were younger than 8 years of age. They were followed up until 5 years after implantation. Auditory performance was assessed by using the categories of auditory performance (CAP) scale and speech intelligibility rating which evaluated the spontaneous speech of each child before and at frequent intervals for five years after implantation.

 Results: Pre-lingually deaf children showed significant improvement in auditory performance after implantation. Six months after implantation 91% of children had the ability to respond to speech sounds. At the end of year one, 96% of children could discriminate speech sounds and 84% of children who reached the three year interval could understand common phrases without lip-reading. After cochlear implantation, the difference between the speech intelligibility rating increased significantly each year for 3 years (p<0.05) and did not plateau up to 5 years after implantation. The changes in auditory performance and speech development were parallel.

 Conclusion: The results indicated the ability of cochlear implantations to significantly improve auditory receptive skills and subsequently speech development in young congenitally deaf children.

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 Abstract

 Alvelolar hydatid disease is caused by E. multilocularis. Human infection occurs at the larval stage of this small tape worm (1.2 to 4.5 mm). In this article we studied alveolar hydatid disease in a 50 year old woman who presented with a hepatic mass. Liver biopsy and histopathologic evaluation showed alvelolar hydatidosis of the liver. Then resection of the left lobe of liver was done, with medical treatment with oral Albendazole (15 mg/kg/12h) for 6 weeks with 2 week intervals. The drug was continued for 4 courses. 5 year follow up of the patient was satisfactory without any complaint.

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 Abstract

 Background: There are a large number of patients with penetrating abdominal trauma who have normal vital signs and negative abdominal examination when referred to trauma centers. Agreat deal of controversy exists between authorities about screening these patients for emergency explorative laparotomy. Many references have reported more than 90% sensitivity for DPL as a diagnostic method to determine whether intraabdominal injuries were present and emergent laparotomy is indicated or not. This study is for reassignment of this sensitivity according to our own evidence.

 Methods: All of the patients with abdominal stab wounds and normal vital signs plus negative abdominal examination who were referred to Shohada-e-Tajrish hospital between March 2004 to December 2005, underwent local wound exploration and those confirmed to have peritoneal penetration, underwent emergency laparotomy. In the operating room and prior to surgery, under general anesthesia, DPL was performed. Then DPL results were compared with laparotomy findings and DPL sensitivity was assigned.

 Results: Of the total number of 34 patients, 8 had a positive DPL and positive laparotomy 2 had a positive DPL and negative laparotomy 8 had negative DPL and positive laparotomy, and 16 patients had negative DPL and negative laparotomy.

 Conclusion: According to our study, DPL sensitivity is much less than mentioned in trauma texts (approximately 50%). So, it is not a valuable tool to discriminate between operative and conservative approaches in penetrating abdominal trauma. We suggest more sensitive modalities. Laparotomy is the most sensitive approach but at the price of a high negative laparotomy rate.

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  Abstract

  Background: Hydronephrosis is the most common congenital condition that is detected

  by prenatal ultrasonography. Moreover, the widespread use of prenatal ultrasonography

  results in an increased recognition of fetal hydronephrosis. Prenatal hydronephrosis

  is diagnosed at an incidence of 1:100 to 1:500 by ultrasonographic studies. The presence of hydronephrosis is not synonymous with obstruction. Obstruction signifies impairment of urinary flow, which if left untreated will cause progressive deterioration of renal function. Approximately 10-20% patients with obstruction show progression of hydronephrosis or worsening renal functions.

  Methods: The study population consisted of 72 patients who were referred to the Division of Pediatric Nephrology, Mofid Children's Hospital, Tehran, IRAN for evaluation

  and treatment of pyelonephritis. All patients underwent two-dimensional ultrasonography

  (2D US) of the urinary tract immediately afterward. Diagnosis of pyelonephritis has

  been based on clinical and paraclinical findings and abnormal 99mTc- demercaptosuccinic acid scan (DMSA scan). Glomerular filtration rate was in normal range in all of them. The children were classified in two groups as having normal kidney ultrasonography and abnormal ultrasonography. Fresh random urine samples were obtained on the admission time and at 48th hour of treatment. Urine samples were tested for N-acetyl-beta- D-glucosaminidase (NAG) (ELISA colorimetric, DIAZYME, USA) and creatinine. All of our patients were treated with same medication. We also evaluated our patients with voiding cyctoureterography (VCUG), renal scintigraphy and biochemical studies.

  Results: In this study 73.6% of the patients had normal ultrasonography and 26.4% abnormal ultrasonography. In patients with abnormal kidney ultrasonography, condition

  such as stasis, moderate to severe hydronephrosis, decreased cortical thickening and urinary stone were seen in 47.4%, 26.3%, 5.3% and 21.17%, respectively. The mean for urinary NAG/Creatinine before antibiotic therapy was 36.79 ± 42.24 U/g creatinine in patients with normal ultrasonography, and 46.22 ± 57.53 U/g creatinine in abnormal group. Patients with hydronephrosis had the highest level of urinary NAG (p-value<0.043) and patients with urinary stone, had the lowest level of urinary NAG (p-value<0.004).

  Conclusion: The urinary NAG was elevated in children with urinary tract abnormality

  with or without infection. Hense this protein could be a useful marker in prediction of hydronephrosis and its renal damage in pediatric group, although studies with greater numbers of patients are needed to establish this opinion.