نشریه پژوهش در پزشکی
سال بیست و پنجم شماره 3 (پیاپی 99، پاییز 1380)

Myotonia chondrodystrophia, which is easier to diagnose than describe, is a rare congenital disorder inherited as an autosomal recessive. Myotonia is accompanied by chondrodystrophia and epiphyseal dysplasia. Affected individuals are presenting with growth retardation, pectus carinatum, sadly appearance of face, blepharophimosis, ear and dental abnormalities, puckered lips, and hypertrophic muscles. Articular contractures especially in elbow are existing and patients had problems in gait. EMG changes are usually used to differentiate it from congenital myotonia. Immune deficiency disorders are not noted and its etiology is still remained unknown. Therapeutic affaires are of less importance. Case report: we have presented an 8-year old boy referring due to facial deformity. His parents have stated that he had facial deformity and problems in gait since 6 years ago. His problem had progressive course. He had problems in sitting, standing up, and going up stairs, but he had never fallen down. His past medical history has revealed nothing. Now, he is studying in primary school and is a good student, too. His parents were not relevant. He is the second child and the other two children are normal. He has the following indices: Body weight: 21kg, height: 116cm, head circumference: 52 cm, chest: 80cm, BP: 100/60 mmHg, T:37.1 °c.

Couvade syndrome is a phenomenon that an expectant father waiting for childbirth experiences during his wife's pregnancy or postpartum periods in the form of somatic or psychological complaints. In rare cases, this syndrome may lead to the psychotic dimensions. In the present report, we have introduced a patient who had severe depression with psychotic feature, fallowing his wife's being hospitalized for childbirth. The content of his delusions was related to the childbirth and his wife's pregnancy. As regards to the occurrence of a psychotic depression syndrome, our diagnosis was psychotic couvades syndrome. Its very rare occurrence was the reason of presentation. Being admitted in the hospital, the patient was given haloperidol and amitiptyline. Then he was discharged after a successful recovery. Although this syndrome is very rare, more attention should be paid to the family mental health with much more emphasis on every member of family during the mother's pregnancy or postpartum periods.

Spinal cord injuries are among the most common problems originated in human societies. In our country this problem takes into account more seriously when considering the devotees of 8-year imposed war.

After filling the questionnaire and receiving necessary educations, paraplegic patients walked on a course like "figure of 8" with preferred speed for 5 minutes. Heart rate, blood pressure, and RPE were measured. Tests were achieved in three stages, each time with a different crutch. Axillary crutch, elbow crutch, and walker were used randomly. Energy cost was assessed using two novel indices PCI (Physiological Cost Index) and RPE.

10 paraplegic patients with mean age of 34±4.99 yrs, height of 167cm, and weight of 64.6kg had entered this study. All of them had spinal cord injuries, averagely occurred for 3.8±3.3yrs. Mean of PCI was measured 1.94, 1.58, and 1.65 beats/min for axillary crutch, elbow crutch, and walker, respectively. Mean of walking speed was higher with elbow crutch. Regarding the RPE, results have revealed that when patient has little balance, walker is the preferred device but in case of good balancing then elbow crutch is the device of choice. Patients with complete spinal cord injuries have shown lower PCI comparing with those who had incomplete injuries. This was proved in other studies, too. Meanwhile, walking speed in patients with complete injuries was 1.4 times as likely as those who had incomplete injuries.

Using crutches and exercising could lead to higher cardiovascular readiness that would be accompanied by lower PCI. Employing walking-aid devices in rehabilitation centers seeks more attention.

The present study was conducted with the aim of determining the prevalence of rheumatic heart disease (RHD) in individuals aged 6-20 yrs in Iran and comparing it with other countries. Primary and secondary prophylaxis could be a safe, cheap, and confident program for reducing the incidence of rheumatic fever (RF) and recurrence of RHD. RF and RHD are the most common cause of acquired heart disease in children and young adults of developing societies. Meanwhile, RHD is a serious long-term sequel of ARF and one of the leading causes of morbidity and mortality in adolescents and young adults.

320000 students, aged 6-20 yrs, had entered this descriptive-cross sectional study in Zanjan province since 1991 till 1993.

Mean age of the studied units was 11±6 yrs and M/F ratio was 3/2. Prevalence of RHD was revealed to be 0.36 per 1000.

Young age of individuals taking part in this study or satisfactory coverage of primary health cares in the above mentioned area could be some of the reasons for the low prevalence rate.

Early diagnosis of systemic fungeal infections like Aspergilosis is a main problem in patients, especially with malignancy, pulmonary TB, Asthma, and COPD. In this study pulmonary tuberculosis and chronic obstructive pulmonary disease are studied as the predisposing disorders for aspergilosis, and this was because of the high prevalence of these two disorders in Iran and high mortality in association with aspergilosis. 

Indirect hemagglutination assay (IHA) was used for measuring the antibody titers against aspergillus fumigatus,mainly of IgM type. Sheep red blood cells coated with aspergillus fumigatus antigens were used as the antigen suspension, for detection of the agglutination in titers between 1:10 to 1:1280. 58 patients, including 32 with TB and 26 with COPD had entered this study. For each patient in the case group, a person was matched as the control group.Obtained sera were kept frozen until the time for examinating all of them.

Test results for the TB group were so: 34% 1:1, 15.5% 1:10, 22% 1:20, 9% 1:40, 9% 1:80, and 9% 1:160. For the COPD group these results were achieved 38.5% 1:1, 27% 1:10, 4% 1:20, 19% 1:40, 7% 1:80, and 4% 1:160. From each control group one person had showed titer of 1:20, who had undergone further studies considering the probable previously aspergilosis. According to the kit instruction and by evaluation of cut off point, the positive titer was considred 1:20 or more. So, 50% of the patients with TB and 34% of the patients with COPD were considered positive.

Acquired deficiency in immune system among patients with TB was known responsible for special distribution of positive test titers, and IHA is proposed as a sensitive method,as well as cheap and easy, to be used in epidemiologic studies.

Bacterial endocarditis is a mysterious and dangerous disease in pediatrics. Even with aggressive antibiotic therapy 20-25% and in more serious cases 50-60% mortality rate has been reported.For this reason, this study was designed to evaluate the predisposing factors and conditions influencing the occurrence and the prognosis of this disease.

 This study of existing data type was performed on 40 patients aged under 14 years, who had definite diagnosis of bacterial endocarditis hospitalized in Medical Centers of Shahid Beheshti University of Medical Sciences since 1990 till 1995.

 60% of the cases were females.Congenital Heart Disease were the most common predisposing factor with prevalence of 60%, where VSD was by far the most common congenital disease predisposing to bacterial endocarditis with the prevalence of 66.6%. Blood culture was positive in 27.5% of the cases with the most common pathogens being Staphylococcus Aureus (36.5%) and Streptococcus Viridance (27.2%). ESR rate of above 20 was observed in 77.5%, Leukocyte count of above 10,000 was reported in 67.5% and CRP was positive in 55% of the cases. The most common" manifested symptoms were fever (97.5%) and heart murmurs 87.5%.

 Due to the relative high incidence of congenital heart diseases and their  predisposition to bacterial endocarditic, more attention should be paid to the prevention of  disease.

"Tehran prospective survey of lipid and sugar" is the first biggest epidemiologic study in Iran, conducted with the aim of determining the etiologic factors of cardiovascular disease in Tehran urban residents. This study, covering a vast majority of the population, was begun with society recognition and just like other prospective studies has paid more attention to social relations. Familiarizing the society with research objectives and encouraging them to active participation were among the most determining factors observed exactly in this survey. This was entrusted to the "group of social relations", composed of social workers and health assistants of East Health Services System. They have face to face communication with the studied society and have covered about 17000 cases within the first two years in Tehran 13th District. Despite being aware of weak points of therapeutic and health services and also employing previous experiments, we have faced numerous unpredictable problems. However, resolving these problems has brought us valuable experiences, steering us towards facilitated next steps. In this study we have taking advantage of voluntary "health assistants" and 3 Health Services Centers supervised by Shahid Beheshti University of Medical Sciences. Removing opposing tendencies towards public health services, spending adequate time for explaining and conducting the research, achieving strategies for cultural beliefs' concurrence, and finding ways of population excitement for participation were other activities performed in our study.

 Complete Heart Block (CHB) is one of the most important cardiac event emergencies.The present study was achieved to evaluate the variable types of CHB, associated with congenital heart disease, predominantly clinical manifestation, complication, treatment, outcome and mortality rate.

In a retrospective overview, 54 children with CHB admitted in Rajaie Heart Hospital were studied between 1979-1981 and 1991-1999. Data were collected by multiple-purpose questionnaires and then analyzed with descriptive statistic methods.

 Congenital, acquired- post surgical and non surgically CHB had been reported at about 63%, 26% and 11% of the cases, respectively. Meanwhile, acquired post surgical and congenital CHB were associated with congenital heart disease at 100% and 25% of cases respectively. Syncope and ECG findings were the most common manifested symptoms in congenital and acquired CHB. However, pacemaker malfunction and mortality rate were reported in 13% and 13% of the cases, respectively.

 Since CHB is a cardiac emergency and the most common cause of death in newborn is CHB, primary cares and immediate pacemaker implantation seems to be vital.

 Medical journal editors have impressible role in the publishing process. In the present study we have surveyed the attitudes and knowledge of Iranian medical journal editors towards standards of published medical research.

 51 editors of registered journals were invited, where 27 have taken part. A self-directed questionnaire according to the Vancouver group guidelines was distributed. It was composed of 28 questions in the form of 9 groups, including peer review, conflicts of interest, authorship, scientific fraud, duplicate publication, mass media, advertising, competing manuscripts based on the same study, internet. Then, editors' knowledge of Vancouver group guidelines was measured through (- 44) to (+ 44).

 23 editors and 4 executive managers with mean age of 47.3 ± 8.7 yrs had entered. More than half of them had Ph.D. degree and most of the journals have taking advantage of peer review systems, usually 2-3 peers. Articles were approved by the majority of the peers in 92.6% of the cases, however, 52% of the journals have employed statistical peer review. Most of the editors have believed that writing the primary manuscript and designing the method of study are the principles of authorship, even though, most of them have considered these according to the Vancouver group guidelines. 7(25.9%) journals had no advertising. 12 journals have been presenting in Internet, where, 81.5% of the editors asserted the benefits of having Internet sites for journals. Mean of knowledge score was 6.5 ± 7.5. where. 33.3% had score of 0, 44.4% and 22.2% had moderate and good scores, respectively.

 Results have revealed the importance of peer review process, but the editors' policies should be more clarified. Editors were more or less agreed with the Vancouver group guidelines. Other studies could be helpful in promoting editors' attitude and practice.

Hypothyroidism and associated mental disorders are prevalent in iodine deficient regions. Since the prevalence of hypothyroidism is unknown in mentally retarded patients in Iran the present study was carried out over 2003 patients hospitalized in Tehran institutes.

Following the physical examination and filling the questionnaire, blood samples were obtained for measuring T4, T3, and TSH. Radioimmunoassay technique was used for this purpose.

Among 2003 candidates, 295(17%) had goiter of grade lb, whereas grade II and grade III goiter were reported in 136(6.7%) and 16(0.8%) of the cases, respectively. T4 level was reported as below: 4.1- 4.5 ug/dl in 1.5%, 3.1- 4 in 1.7%, 2.1- 3 in 0.6%, and below 2.1u.g/dl in 0.1% of the mentally retarded patients. 53(2.65%) had TSH level of more than 5mU/l, where most of them (2.15%) had TSH level of 5-10 mU/1. TSH>10mU/l was recorded in 10 cases. 3 patients were received levothyroxine due to previously diagnosed hypothyroidism. 76(3.8%) cases were found to have mongolism, where 7(7.9%) of them had hypothyroidism.

Results have revealed the relatively high prevalence of hypothyroidism in mentally retarded patients. Controlling iodine deficiency disorders as well as screening hypothyroidism seems to be necessary for eliminating mental disorders.

 Along with the development of medical education and to achieve successful use of problem-based learning (PBL) practical policies and readiness are necessary. The present study was carried out over the general practice trainees with the aim of determining the effect of readiness (cognitive and affective) for self-directed learning (SDL) on PBL.

 50 general practice trainees had entered this experimental study, where they have been randomly distributed in two groups of case and control. Students in the case group were received related education. Cognitive and affective readiness were assessed by questionnaire. Data were analyzed by parametric (paired t-test, t-test...) and non-parametric (chi-square, Mann-Whitney U-test, Wilcoxon...) tests.

 Results have revealed low cognitive but nearly high affective readiness, however, non-significant difference was reported between the groups according to the level of readiness. There was significant difference between the score of "knowledge" and "tendency", before and after the education in the case group (p<0.05 and p<0.01, respectively. Meanwhile, significant difference was found between the groups considering the score of learning. (p<0.001)

 SDL is one of the basic principles of PBL. Bringing readiness for SDL before introducing PBL is highly recommended.

Respiratory Distress Syndrome, which was previously known as Hyaline Membrane Disease (HMD), is among the most common cause of immature neonatal death. The present study has discussed the disease history, pathophysiology, clinical manifestations, laboratory findings, radiologic findings, and differential diagnosis. Meanwhile, recent progresses achieved in the field of treatment using surfactant or modern techniques of mechanical ventilation were mentioned. Prevention and future perception were also discussed.