Acta Medica Iranica
Volume:46 Issue: 3, May -June 2008

Glucose-6-phosphate dehydrogenase is an essential enzyme to cell growth. Its deficiency of enzyme plays an important role in senescence and death signaling. Also, it is actually the most common clinically important enzyme defect, not only in hematology, but also among all human known diseases. Clinical consequences of enzyme deficiency are: neonatal hyperbilirubinemia, acute hemolytic anemia, and chronic hemolytic anemia. The enzyme gene spans 18 kb on the X chromosome (xq28) and contains 13 exons. Its promoter is embedded in a CpG island that is conserved from mice to humans. The development of a number of PCR-based methods for the detection of known mutations in Glucose-6-phosphate dehydrogenase has made it possible to detect enzyme deficiency and identify the specific mutation responsible with relative ease. We will discuss the mentioned clinical manifestations of glucose-6-phosphate dehydrogenase deficiency, Genetics, biochemistry and pathophysiology of the enzyme in details using newer published data and present most of the studies in Iranian population.

Proprioception has been found to have a relation to subjective knee function and patients with patellofemoral pain syndrome (PFPS) seem to have larger deficits than asymptomatic individuals little is known about whether taping can restore defects in proprioception or by which mechanisms it can improve anterior knee pain. To determine the effect of patellar taping on knee joint proprioception a pre and post intervention repeated measures design was conducted on 25 male with PFPS (23.6 ± 3.04 years) and 25 healthy male (23.5 ± 3.1 years). Active angle reproduction, passive angle reproduction, and threshold to detection of passive movement tests were measured. Each of the tests was done under taped and no-taped conditions in 20 and 60 degree of knee flexion. We found significant difference between taped and no-taped conditions in active angle reproduction test for both groups (P < 0.05). The other tests did not show any significant difference (P > 0.05). Obtained results suggest that patellar taping may improve knee proprioception during active angle reproduction. Two groups could benefit from taping but further researches are needed to determine whether the present results are applicable to other situations or not.

Cutaneous leishmaniasis is endemic in 88 different countries. There are an estimated 1.5 million new cases each year, with over 90% occurring in Afghanistan, Algeria, Iran, Iraq, Saudi Arabia, Syria (Old World) and in Brazil and Peru (New World). Miltefosine is effective in vitro and in vivo against Leishmania species and it was demonstrated efficacy in animals via the oral route. This study is the first one for evaluating the effect of miltefosine on cutaneous leishmaniasis of L. major (MRHO/IR/75/ER) by in vivo and in vitro studies in the BALB/c mouse model. As it was shown, miltefosine has a better effect on reduction of size of lesion compared to Glucantime®, also it was not significant by statistical analysis. The results of this study show that miltefosine has a good activity against the proliferation of amastigotes of L. major. The results suggest that oral miltefosine might be a promising approach for developing new anti-Leishmanial drugs.

The oxidation of low-density lipoproteins and cell membrane lipids is believed to play an integral role in the development of fatty streak lesions, an initial step in coronary artery disease (CAD). Paraoxonase-1 (PON1) is an enzyme associated with the high-density lipoprotein (HDL) particle. PON1 protects LDL from oxidative modification by hydrolyzing lipid peroxides, suggestive of a role for PON1 in the development of CAD. The present study tested the hypothesis that Paraoxonase-1 promoter polymorphism T(-107)C could be a risk factor for severity of CAD in Iranian population. Paraoxonase-1 promoter genotypes were determined in 300 consecutive subjects (> 40 years old) who underwent coronary angiography (150 subjects with >50% stenosis served as cases [CAD+] and 150 subjects with < 20% stenosis served as controls [CAD-]). PON1 promoter genotypes were determined by PCR and BSTU1 restriction enzyme digestion. CAD+ Subjects did not show any significant differences in the distribution of PON1 promoter genotypes as compared to CAD- Subjects (P = 0.075). However the analysis of PON1 promoter genotypes distribution showed a higher percentage of (-107) TT among CAD+ compared with CAD- (P = 0.027). After controlling for other risk factors, the T(-107)C polymorphism had interaction with age (P = 0.012), but did not show any interaction with other risk factors such as BMI, gender, smoking, diabetes, level of HDL-C, LDL-C, triglyceride and Total cholesterol. These data suggest that the TT genotype may represent a genetic risk factor for Coronary artery disease in Iranian population.

Polycystic ovary syndrom (PCOS) is characterized by infertility, oligomenorrhea, and hyperandrogenism. Clomiphene citrate (CC), an antiestrogen, is first-line treatment for PCOS, if CC fails to induce ovulation, laparascopic electrocautery of the ovaries is offered. In this prospective controlled study, 52 women with clomiphen-resistant PCOS (group 1) and 46 women with regular menstrual cycles as a control group (group 2) were included. Hormonal profile and doppler blood flow changes within the ovarian stroma befor and after laparascopic ovarian electrocautery (LOE) in women with clomiphen- resistant polycystic ovary syndrome assessed and compared between groups. The doppler indices (pulsatility index and resistance index) of ovarian stromal blood flow were significantly lower in group 1 befor LOE than in group 2. The serum levels of testosteron (T) and LH were significantly reduced in group 1 after LOE compared with in group 1 befor LOE (P < 0.05). Doppler indices (pulsatility index and resistance index) of ovarian stromal blood flow were significantly increased after LOE. Laparascopic electrocautery reduced serum T and LH and reduced ovarian blood flow velocities, which may explain the reduction of ovarian hyperstimulation syndrome in women with PCOS after LOE. Laparascopic electrocautery of the ovaries is now accepted as the preferred first-line treatment of women with PCOS who are resistant to clomiphen citrate. All affected women could be considered for this treatment.

Although the increase in the number of core sutures during flexor tendon repair increases the tensile strength of the repair, both increased handling at the time of repair and increased bulk at the repair site have been hypothesized as affecting clinical outcomes by increasing gliding resistance. The purpose of our study was to assess whether increasing the number of core sutures from two strands to four strands in zone II flexor tendon repair could improve the clinical results. A total of 42 patients with 60 flexor tendon lacerations in zone II were enrolled in this study. The injured tendon randomly underwent surgical repair by using one of the 2-strand or 4-strand modified Strickland techniques. Postoperative rehabilitation was the active extension-rubber band flexion method of Kleinert in all patients. The average duration of follow up was 8 months. The mean total active motion was 155.16˚ in 2-strand group (excluding two ruptures) and 154.33˚ in 4-strand group (P > 0.05). Using Strickland's original score, this corresponds to excellent result in 83.3% and 86.6% of patients in 2-strand group and 4-strand group, respectively. Statistically, there was no significant difference between the clinical outcomes obtained in these groups. Concerning the tendon rupture after the repair, all of the ruptures (2 cases) occurred in the 2-strand group (P > 0.05). We concluded that both suture strength and gliding resistance have influence on the result of flexor tendon repair in zone II, and increasing the number of core sutures and the resulting suture strength may be negatively affected by increase in gliding resistance.

Concomitant coronary artery bypass surgery (CABG) in patients undergoing mitral valve replacement (MVR) has been shown to be an important risk factor for hospital mortality. We evaluated preoperative characteristics, postoperative complications, in-hospital mortality rate, and length of stay in hospital for patients undergoing concurrent CABG with MVR. Preoperative and postoperative clinical data from 175 patients undergoing concurrent CABG with MVR operation at Tehran Heart Center from 2002 through 2006 were collected and entered into a database. Information was obtained by clinical and case note review as well as detailed questionnaires to physicians and patients. Mean age of patients was 57.95 ± 10.54 years and 51.4% were male. Mean New York Heart Association (NYHA) score was 2.46 ± 0.84. Among studied patients, 18.3% and 2.9% underwent aortic and tricuspid valve replacement, respectively. In-hospital mortality was 6.9% and 96.0% of patients were hospitalized ≥14 days. History of congestive heart failure (P = 0.027) and postoperative brain stroke (P = 0.004) were independent predictors for in-hospital mortality. Exact considering of congestive heart failure and postoperative brain stroke related to in-hospital mortality in concurrent CABG with MVR operation are necessary.

Reconstruction of breast with transverse rectus abdominis myocutaneous (TRAM) flap is the standard for reconstruction of breast following mastectomy. In this article, authors report their experience with pedicled TRAM flap reconstruction of the breast. Records for the patients who had undergone breast reconstruction with pedicled TRAM flap were retrieved. Records of outpatient follow-ups were also obtained. Patient satisfaction with the outcome of surgery was assessed using a detailed questionnaire including a linear visual analogue scale ranging from zero (not satisfied) to ten (most satisfied). There were 61 reconstructions in 59 patients. In 42 (71.2%) cases a synthetic mesh and in 14 (23.7%) cases dermal graft was used for closure of the abdominal fascial defect. The mean hospital stay was 10.67 (1 - 72) days. Patients were followed up for a mean period of 621 days. The overall rates of complications were as follows: partial flap necrosis: 11 (18.6%), flap hematoma: 2 (3.4%), flap seroma: 7 (11.9%), flap wound infection: 7 (11.9%), abdominal wound hematoma: 9 (15.3%), abdominal wound seroma: 5 (8.5%), abdominal wound ischemia: 1 (1.7%), abdominal wound incisional hernia: 6 (10.2%), deep vein thrombosis: 1 (1.7%), complication requiring rehospitalization: 9 (15.3%), complication requiring reoperation: 8 (13.6%). There were no abdominal wound infection, no umbilical necrosis, and no pulmonary embolism. Aesthetic results were classified as excellent (62%), good (28%), fair (10%). The mean satisfaction score was 9.5 (range 6-10). Breast reconstruction with pedicled transverse rectus abdominis myocutaneous flap was associated with a low complication rate and a high level of patient satisfaction in our center.

Postsurgical hypothyroidism and its incidence has not been fully investigated. In this study, the incidence of hypothyroidism and its possible risk factors at Shariati Hospital was assessed. One hundred and two patients with benign thyroid diseases, who had undergone thyroidectomy, were investigated with regards to thyroid function profile during period of one year after the operation every three months with post-operative sample as the baseline. Hypothyroidism was developed in 36 patients (35.2%) on average 5 ± 3.2 months after surgery. Factors such as increased age, operation type, histopathologic type, underlying disease, lymphocytic infiltration and use of levothyroxine before surgery were associated with the increased incidence of hypothyroidism. It seems that use of indicators such as Grave's disease and lymphocytic infiltration in pathologic specimens should be helpful in projecting the potential occurrence of hypothyroidism in patients undergoing thyroidectomy.

Waste collection is a necessary activity all around the world and the removal of municipal solid waste is a job associated with a variety of biological, chemical, mechanical, physical, and psychosocial hazards. In our country, like many developing countries, municipal solid waste is collected manually and collection of household waste is also a job which requires repeated heavy physical activity such as lifting, carrying, pulling, and pushing. We performed this study to evaluate musculoskeletal disorders among municipal solid waste workers. We designed a cross sectional study. Our survey instrument for measurement of musculoskeletal symptoms was adapted from the Standardized Nordic Questionnaire that translated into Farsi language. A total of 65% (n=142) of participants reported that they had been troubled with musculoskeletal symptoms in one or more of the 9 defined body regions during the last 12 months. Prevalence of symptoms in low back, knees, shoulders, upper back and neck were 45, 29, 24, 23 and 22% respectively. Foreign workers reported more musculoskeletal symptoms in all body parts than Iranian workers. The differences between prevalence of symptoms between two groups were significant in all parts of body except knees. The study found that solid waste workers have more musculoskeletal disorders than general population. Meanwhile these symptoms were more common among foreign workers. The risk of disease was increased with the increasing years of working as solid waste worker and smoking. We didnt find relationship between musculoskeletal disorders and education or marriage status of workers.

Indirect immunofluorescence (IIF) has been used to identify and measure autoantibody levels in pemphigus vulgaris but data about relationship between clinical severity of disease and antibody titers by IIF have been conflicting. We conducted this cross-sectional study to correlate the severity of oral and/or cutaneous involvement in patients with pemphigus vulgaris based on Pemphigus Area and Activity Score with IIF titers. Sixty-one new pemphigus vulgaris patients were included in this study. Human prepuce was used as substrate for IIF and assessment of disease severity was based on Pemphigus Area and Activity Score. The mean±SD age was 44.04±30.46 years, with a range of 18 to 79 years. IIF was positive in 56 (91.8%) patients. There was a significant relationship between total disease score and IIF titers ((P<0.001). Also a significant relationship was found between skin score (P=0.04) and mucosal score (P=0.04) with IIF titers. Our results show that there is a significant relationship between disease activity based on Pemphigus Area and Activity Score and antibody titers by IIF. Further studies are recommended to determine the usefulness of this technique for monitoring disease.

Anaerobic bacteria are well known causes of sepsis in adults but there are few studies regarding their role in neonatal sepsis. In an attempt to define the incidence of neonatal anaerobic infections a prospective study was performed during one year period. A total number of 400 neonates under sepsis study were entered this investigation. Anaerobic as well as aerobic cultures were sent. The patients were subjected to comparison in two groups: anaerobic culture positive and anaerobic culture negative and this comparison were analyzed statistically. There were 7 neonates with positive anaerobic culture and 35 neonates with positive aerobic culture. A significant statistical relationship was found between anaerobic infections and abdominal distention and pneumonia. It is recommended for those neonates with abdominal distention and pneumonia refractory to antibiotic treatment to be started on antibiotics with anaerobic coverage.

Intracranial hemorrhage is one of the major causes of neonatal mortality and morbidity. It is the most severe cranial problem in that period. Those who survive would be affected by hydrocephalus, encephalomalacia, and finally brain atrophy. With accurate knowledge of risk factors, hemorrhage may be diagnosed earlier and the complications managed earlier. This study was performed in Neonatal Intensive Care Unit of Imam Khomeini Hospital. All the neonates less than 34 weeks of gestation were undergone intracranial sonography from Feb 2005 to Feb 2006. Sonography was performed via anterior fontanel with proper probe according to neonatal age. 113 neonates less than 34 weeks of gestation have been studied. Mean gestational age was 32 weeks. Mean neonatal weight were 1566 ± 734 grams. Intracranial hemorrhage was evident in 21% of them; 16.8% was grade 1, 0.9% grade 2, 2.7% grade 3, 0.9% grade 4. The mean weight of neonates with hemorrhage was 1504.11 grams. Intracranial hemorrhage had correlation with respiratory acidosis and pneumothorax. The latter was also correlated with hemorrhage grade. Supposing the safety and non-invasiveness of intacranial sonography, we suggest performing sonography in all premature neonates with low birth weight, and also in those neonates with pneumothorax and respiratory acidosis.

The systemic complications of blood donation are the first reasons why patients fail to return for further blood donation. This study was designed to determine the frequency of these complications and their associated risk factors among blood donors in Tehran. Also, we aimed to provide suitable methods to decrease the frequency of these adverse events, thereby eliminating the most important causes of withdrawal, while maintaining the health of the donors. This analytical descriptive cross-sectional study was performed on 554 blood donors who had donated blood from February 2005 through September 2005 in four fixed blood donation bases and four mobile blood collection buses. Each base was considered as a stratum, and a stratified random sampling proportional to size was done to select the donors. Results showed donor reaction rate to be 13.4%, the most common of which were blackout of vision (7%), dizziness (6.3%), fatigue (6.1%) and nausea (1.8%). There was no significant relationship between the incidence of these complications and type of base blood donation or fasting at the time of blood donation. Logistic Regression analysis showed that sex, condition of blood donor, exercise or walking, duration of donation, and practice to recommendation had significant effects on the odds ratio of systemic complication. Regarding the frequency values derived for the different systemic complications it can be concluded that attention to risk factors of these complications and their control can help encourage donors to become repeated donors as well as to prevent their withdrawal for further blood donation.

Breast cancer is the most common malignancy in women in many parts of the world. The risk factors for breast cancer cover a broad range of conditions, from age to complex genetic alterations. Present data is showing an increase in the annual incidence rates of disease and it is ranked as the first recorded malignancies among Iranian women after 1999. To study the association of reproductive and non reproductive factors with breast cancer among women with age group 30-75 years residing in urban area of Yazd city, Iran, this community based case-control study was carried out. A total of 160 subjects including 80 cases and 80 community controls were studied. Age was matched for both the case and control groups. Association of various kinds of risk factors with breast cancer was studied using odds ratios with 95% confidence intervals through both the univariate and multivariate analysis. The findings based on multivariate analysis showed that being employment in outdoor jobs, positive familial history of breast cancer amongst the first grades, early onset of the first menstruation cycle (≤ 13 ys.), delayed age of the first marriage (≥ 19 ys.), less number of live children (≤ 3) and less duration of breastfeeding (≤ 2 ys.) were significantly associated with breast cancer. However, consumption of OCPs was found to be a protective factor for breast cancer. Our study showed that the role of reproductive factors in the causation of breast cancer was more important than in other factors like socio-economic, psychological, behavior and nutritional factors.

In the present health care environment, cost-benefit analysis is extremely important. In this screening program, the minimal cost of screening dipstick urinalysis in 1601 asymptomatic school children was determined. The process of screening was similar to all the studies. The minimal cost utilizing 3 general physicians was calculated. Costs were determined by using current charge for supplies ordered to perform tests, charges for tests performed by a commercial laboratory, and the cost of a final evaluation by a pediatric nephrologist. Initial abnormal urinalysis was found in 4.7% (76/1601) of patients. Upon retesting 1.37% (22/1601) of patients were calculated to have a persistent abnormality. The calculated cost was 1/530/000 Rials (164.5 $) to initially screen all 1601 patients with a dipstick urinalysis or 850 Rials (0.09 $) per patient. The calculated cost to evaluate the 22 patients with any persistent abnormality on repeat dipstick urinalysis was 246/840 Rials (26.5 $) or 11.220 Rials (1.2 $) per patient. This is the calculated cost for a single screening of 1601 asymptomatic pediatric patients. Multiple screening dipstick urinalysis in asymptomatic pediatric are costly and should be discontinued. We purpose that a single screening dipstick urinalysis be obtained at school entry age, between 6 and 7 years old, in all asymptomatic children.

Pulmonary Langerhans Cell Histiocytosis (PLCH) is a rare idiopathic disorder that primarily affects young adult cigarette smokers. Affected patients often present with cough and dyspnea and about 20% of patients present with or later develop pneumothorax. It is striking that more than 90% of patients are smokers. We report a very unusual case of PLCH in a 20-year- old male patient with no smoking history in whom a life- threatening complication such as simultaneous bilateral pneumothorax was the presenting feature. The final diagnosis was made by open surgical biopsy and recurrent pneumothoraces necessitated surgical management with pleurodesis. We emphasize the early use of pleurodesis in managing patients with PLCH and spontaneous pneumothorax.

Congenital anterior tibiofemoral subluxation is an extremely rare disorder. All reported cases accompanied by other abnormalities and syndromes. A 16-year-old high school girl referred to us with bilateral anterior tibiofemoral subluxation as the knees were extended and reduced at more than 30 degrees flexion. Deformities were due to tightness of the iliotibial band and biceps femuris muscles and corrected by surgical release. Associated disorders included bilateral anterior shoulders dislocation, short metacarpals and metatarsals, and right calcaneuvalgus deformity.