Iranian Journal of Medical Sciences
Volume:32 Issue: 3, Sep 2007

Abstract This review summarizes current data on the pathomechanisms and new genetic findings of combined factor V and VIII deficiency (CF5F8D). Congenital haemorrhagic disorders characterized by deficiency of two clotting factors comprise an interesting group. Among dual coagulation disorders, CF5F8D is the most common type. For the first time combined factor V and VIII deficiency (F5F8D) was reported by Oeri et al in 1954. That is distinct from the coinheritance of both FV deficiency (parahaemophilia) and FVIII deficiency (haemophilia A) that has been reported in four families. Individuals who present with this phenotype have between 5 and 30% of normal plasma levels of FV and FVIII antigen and activity, whereas the level of other plasma proteins are not altered. Total numbers of affected individuals are less than 150 cases all over the world. At first it was assumed that deficiency of protein C inhibitor was a responsible cause, but further investigations revealed that it was due to mutations called ERGIC-53 and LMAN-1.

Abstract Serum lactate dehydrogenase (LDH) concentration is an indicator for tissue injury. It may be secreted locally in many conditions. For the first time, this study was performed to investigate the value of LDH level in bronchoalveolar lavage fluid (BALF) in differentiation of benign from ma-lignant single pulmonary nodules (SPNs) and to assess its relationship with serum LDH levels. This study was a prospective case-control clinical study. It included 59 patients with a SPN and 21 non-smoker healthy adult volunteers as controls. They underwent bronchoscopy with BAL, Transbronchial needle aspiration (TBNA), and transbronchial biopsy (TBB). Both total serum and BAL LDH levels were measured. The range of the BAL LDH levels in the control group was 4.60 -26 mIU/ml, in patients with benign nodule was 6 – 83 mIU/ml, and in those with malignant nodule was 33 -147 mIU/ml. Overall, the mean BALF LDH level was significantly higher in patients with a malignant pulmonary nodule (85.92 ± 28.31) as compared with that of either patients with a benign nodule (19.08 ± 18.35) (p<0.0001) or control group (12.16 ± 6.18) (p<0.0001). No significant difference between the absolute value of BAL LDH level in patients with benign pulmonary nodule and the control subjects was found (p=0.23). There was no correlation between BALF LDH and serum LDH level in patients with SPNs (p=0.595). BALF LDH levels are increased in patients with malignant SPN, but had no significant rise in benign solitary pulmonary nodules. This factor is useful in differentiating benign from malignant SPNs. A low BAL fluid LDH level in a patient with SPN who does not have a tissue diagnosis may be deemed acceptable for observation and follow up. This may save patients the need for operative procedures.

Abstract Acute disseminated encephalomyelitis (ADEM), which occurs spontaneously or after systemic viral infection and vaccination mostly affects children. This study aims at describing the epidemiologic, clinical, neuroimaging, laboratory features, treatment and outcome in children who diagnosed as having ADEM and admitted in a referral center in Tehran. This descriptive prospective study was conducted on patients with a diagnosis of ADEM over a period of 30 months, between Sep 2003 and Mar 2006, admitted to the neurology ward of Mofid Pediatric Hospital. All these patients were visited in regular follow-up every 6 months for two years. Eighteen patients with the mean age of 6 years (range 5 months - 12 years) were studied. No sex predominance was noted. Sixty-one percent had prodromal illness, and two patients had been vaccinated before the illness. Thirteen (72%) patients presented in fall or winter. Motor deficits and cranial nerve palsy were the most common features, which had occurred in 13 (72%) patients. Abnormal findings in cerebrospinal fluid evaluation were detected in 33% of the patients. Brain computed tomography was normal in all but one patient. Electroencephalograms done in nine patients were normal in 54%. Magnetic resonance imaging showed lesions were most commonly in the subcortical and periventricular areas (76%). Nine (50%) patients were treated with corticosteroids and intravenous immunoglobulin. The mortality rate was 5.5%, and the relapse occurred only in one case. In two-thirds of the patients, prognosis for complete recovery was excellent. Childhood ADEM is a benign condition, affecting both sexes equally. Recurrent infections have been its common cause in our center.

Abstract There always has been a question about the best age for cochlear implantation (CI) in prelingual deaf children. The age factor in the outcome of cochlear implantation in prelingual deaf children has been the subject of many studies. The aim of the present study was to find the effect of age at the time of implantation on hearing threshold of these children. One hundred and nine prelingual deaf children who had undergone CI were enrolled. The mean hearing threshold (HT) at octave intervals from 125 to 8000 Hz at different periods from the operation time were compared between those patients whose age at the time of implantation were less than 24 months and those whose age were equal or greater than 24 months. The mean age of patients at the time of implantation was 38.9 months and a half of them had less than 28 months. There was no difference in mean HT between the two age groups across different measurement periods. A multiple regression model showed that device type was the sole significant predictor of mean HT at the first and third months after implantation, where age replaced it at the sixth month. These results suggest that what had been found in other studies as a favorable effect of younger age at the time of implantation on speech perception cannot be explained by a better hearing, and there are other important factors including rehabilitation programs beginning at a younger age that may explain those findings.

Abstract The nutcracker syndrome represents a clinical condition caused by compression of the left renal vein (LRV) between the superior mesenteric artery (SMA) and the aorta. One of its manifestations is left-sided varicocele. The aim of this study is to determine the prevalence of nutcracker syndrome in patients with primary and recurrent high grade left-sided varicocele. Fifty patients with primary and recurrent high grade left-sided varicocele were enrolled in this study. Color Doppler ultra-sonography (US) of renal vessels was done and diameter of LRV and peak systolic velocity (PSV) were measured in renal hilum and in site of compression of LRV be-tween SMA and the aorta and then magnetic resonance angiography (MRA) of renal vessels was done. Thirty six patients (72%) had primary high grade varicocele and 14 patients (28%) had recurrent high grade varicocele. Twelve patients (33%) with primary high grade varicocele and seven patients (50%) with recurrent varicocele had evidences of NCS in color Doppler US and MRA. The differences of LRV diameter in NCS group and in patients without NCS were statistically significant (P<0.001). The differences of PSV in the LRV at the hilar portion in the NCS group and in the patients without NCS were not statistically significant. The differences of PSV in the LRV at the aortomesenteric portion in the NCS group and in the patients without NCS were statistically significant (P< 0.001). In all patients with NCS, MRA showed dilatation and compression of LRV at site of passage between aorta and SMA. NCS should be suspected in patients with high grade primary and recurrent varicocele.

Abstract The sternoclavicular joint (SCJ) is the only synovial articulation between the upper extremity and the trunk. This joint is one of the most frequently used joints, so osteoarthritis (OA) should be very common. However, there are few studies about OA in this joint. In this study, 48 sternoclavicular joints from the left and right joints of 23 cases and two left joints from two further cases were studied. Right and left sternoclavicular joints were removed together in an unselected sequential autopsy series. Ninety two blocks were prepared by cutting every SCJ to superior and inferior parts. Using histological staining, the articular surfaces of the SCJs were assessed microscopically. Contact radiography was done to help assessing the presence or absence of osteoarthritis. The features of normal joints were found in 18 blocks. Osteoarthritis changes were seen in 82 of the 92 blocks. There was no significant correlation between age and osteoarthritis. No differences in the degenerative changes were found between the sexes. Severe osteoarthritis changes were more common in the right SCJ and inferior part of the joint than in the left and superior parts respectively. The osteoarthritis changes were severe in 36.6% of cases and mild to moderate in 48.8%. OA was very common in these SCJs, which is not related to age and sex of the cases. There are clear changes in structure and glycan expression in the articular cartilage of the osteoarthritic sternoclavicular joint, which allow a distinction to be made between mild, moderate, and severe osteoarthritis and normal cartilage. Histochemical staining of the joints can lead to accurate diagnosis of this disease.

Abstract Morphological examinations of urinary erythrocytes can be of diagnostic value in initial evaluation of hematuria. Dysmorphic urinary red blood cells are known to indicate a glomerular origin of bleeding. We examined the clinical usefulness of this test in a population complained of hematuria by use of three different light microscopy, phase contrast microscopy, and Wright staining and compared their sensitivity and specificity. The study included 169 patients with hematuria (89 glomerular and 80 non-glomerular). The urine specimens were collected before invasive procedures such as biopsy and cystoscopy. In each urine sample, 100 urinary erythrocytes were examined. Statistical analysis was performed using Student''s t test, correlation coefficient, and x². Reliability parameters including sensitivity, specificity and predictive values of negative and positive tests were also evaluated. Dysmorphic red cells were recorded as acanthocytes, doughnut-like cells, yeast like cells with more than one blebs and ghost forms. Isomorphic erythrocytes had uniform size and shape. Significant difference was found in the number of urinary dysmorphic red cells between the two groups of patients. Statistical analysis showed that by using percentage of glomerular type erythrocytes and setting the cut–off at 20-25%, the specificity for three procedures was almost the same (≈ 97.5%). But sensitivity for light microscopy, phase contrast microscopy, and Wright staining was in different ranges as 70.7%, 89.8%, and 86.5% respectively. It was concluded that with some limitations, these simple, non-invasive techniques were useful in identifying the source of bleeding in the work up of hematuria by considering that sensitivity of the methods were in the order of phase contrast microscopy, Wright staining, and light microscopy.

Abstract Otitis media with effusion (OME) is the most frequent indication for surgery in children. Some surgeons prefer to use adenoidectomy and myringotomy with tympanostomy tubes insertion to prevent the sequelae of the disease and also more improvement of hearing. The aim of this study is to compare adenoidectomy-myringotomy with adenoidectomy-myringotomy plus tympanostomy tube in the management of OME. Thirty children with OME (age range 4-8 year) were randomly assigned to receive adenoidectomy with myringotomy in one ear or adenoidectomy, myringotomy plus tympanostomy tube insertion in the other. The patients were subjected to auditory examinations 1 and 6 months post operation to evaluate hearing levels. The results showed no significant difference in hearing level improvement between these two methods of surgery. It is concluded that adenoidectomy with myringotomy is sufficient for management of otitis media with effusion in children in short term.

Abstract Hexachlorobutadiene (HCBD) is a potent nephrotoxin in rodents, which can cause degeneration, necrosis and regeneration in renal tubular epithelial cells. It has been shown that safranal, the active ingredient of saffron, has a protective effect against ischemic injuries. The aim of this study was to examine the protective effect of safranal against HCBD-induced nephrotoxicity in rats. Thirty Wistar albino rats were randomly divided in five groups. The rats received a single dose of corn oil 1ml/kg (group1), HCBD 50mg/kg (group 2), or safranal at doses of 0.5, 0.25 and 0.1 ml/kg one hour before HCBD (50mg/kg) injection (groups 3-5). All injections were carried out intraperitoneally. Urine samples were collected one day before, and one day after injections. On day 3 the animals were sacrificed and both kidneys were removed. The right kidney was fixed in formalin for histological examination and the left kidney was homogenized for measuring malondialdehyde (MDA). Blood samples were taken by cardiac puncture and used for the measurement of urea, creatinine, glucose and protein concentrations. Blood urea concentration in HCBD treated group was significantly higher compared with group 3 (p<0.01) and groups 1 and 4 (p<0.001). There was no significant difference in urea concen-trations between group 5 and HCBD treated group. Urinary concentration of glucose was significantly higher in group 2, compared with groups 1, 3 and 4 (p<0.001) No significant differences were observed in urinary glucose concentrations between HCBD- and safranal (0.1ml/kg)-treated groups. Concentration of protein was also significantly higher in group 5 than those of other tested groups (p<0.001). Safranal at doses of 0.25 and 0.5ml/kg has a protective effect against HCBD-induced nephrotoxicity in rats.

Abstract Group B Streptococcus (GBS) is a major cause of severe systemic and local infections in newborns. This study was performed to evaluate the presence of GBS in pregnant women and their newborns in Tabriz, Iran. Vaginal specimens were collected from 965 women who were candidate for vaginal delivery for bacteriological study of group B streptococci. Several samples from various sites of the newborns'' body (ear canal, nose, navel, and groin) were also obtained to study the colonization rate in newborns after vaginal delivery. Identification of GBS strains was accomplished by bacteriological and serological tests. During the study period, 327 microorganisms were isolated from vaginal specimens of pregnant women from which 5.2% were Streptococcus agalactiae. Serotypes of S. agalactiae strains in our study were Ia (17.6%), Ib (13.4%), II (14.2%), III (9.5%), IV (8.2%), V (19.5%) and nontypable (17.6%). All of the newborns were followed up for eight weeks. Low carriage rate of GBS in vaginal canal of women in this area and probably lack of more virulent serotypes of GBS may explain the rarity of disease due to Streptococcus agalactiae in our region.

Abstract Ventriculoperitoneal (VP) shunt is one of the most common pediatric neurosurgical procedures. It has various complications that may have serious consequences such as shunt dysfunction, and unusual migration. Although, migration of shunt catheter to bladder, heart, umbilicus, rectum, pulmonary artery, and stomach has been reported, migration to vagina is a rare one. In this report we present a 16-month-old girl in whom VP shunt catheter was found in vagina. We will also describe the mechanism by which the VP shunt migrated to vagina.

Abstract Chondromas are benign tumors that may be found in many parts of the body. Among intracranial neoplasms, tumors of cartilaginous origin are rare. It has been shown that most of these tumors arise from the skull base. Their occurrence in other parts of the intracranial cavity is unusual. Chondroma of falx is a rare neoplasm. In this report we introduce a case of falcine chondroma arose from frontal midline area in a 17-year-old boy.

Abstract Teratomas are tumors originated from mature or immature tissues. Teratomas are made up of one embryonic layer or all three embryonic layers (endoderm, mesoderm, or ectoderm). Herein we report a 41-year-old man who presented with vertigo, vomiting, and tinnitus. After physical examination, laboratory evaluation and performing computed tomography, cholesteatoma was diagnosed, however, during radical mastoidectomy a cystic tumor was found. The result of pathology proved middle ear and mastoid teratoma.