مجله علوم پزشکی رازی
سال پنجم شماره 1 (پیاپی 13، بهار و تابستان 1377)

Hutchinson - Gilford syndrome or progeria is a rare genetic disease with failure to thrive, deficiency of pubertal development and dwarfism. These patients die of premature cardiovascular disturbances and other complications. Characteristic clinical features are finely thin skin, small chin, defect in skin adnexals, prominance scalp tends to be further enhanced by frontal and perietet bossing and by hypopfasia of the face. The cartilage was visible, the lips were thin and there was cyanotic overcast about the nose and mouth with premature aging, and let droping of the shoulders were the important findings in diagnosis ot Hutchinson Gilford syndrome in this case.

Noonan syndrome is a genetic condition inherited by an autosomally dominant manner, characterised by congenital heart disease, short stature, abnormal facies and the somatic feature of Turner's syndrome, but a normal karyotype. Noonan syndrome affects approximately 1 in 1500 live births.
Congenital heart disease occurs in 35-50% of patients diagnosed with noonan syndrome. The most common cardiac lesions are pulmonary stenosis and hypertrophic cardiomyopathy. The frequency of renal anomalies in noonan syndrome is lower than that seen in Turner syndrome. However, splenomegaly with or without hepatomegaly occurs commonly. Ocular manifestations, coagulation factor deficiencies and abnormal bleeding and genital tract malformation are other features of this syndrome. If both parents had only possible or no signs of noonan syndrome, subsequent to the birth of the first child with noonan syndrome in a family, an empiric recurrence risk of 5% is predicted.
Successful treatment of short stature with growth hormone are reported. We report a 7-year old boy with noonan syndrome who presented with short stature, abnormal facies, congenital heart disease and bilateral undescended testis.

In order to find out the incidence and severity of intraventricular hemorrhage (lVH) in premature babies in Iran which probably has not been evaluated and reported so far and compare it with reports of other countries, a prospetive study was done over a two year period. All premature babies at or before 34 weeks gestation which were admitted in the newborn nursery of Ali Asghar Children Hospital were evaluated for IVH. Intraventricular hemorrhage was diagnosed by cranial sonography and autopsy. From 84 premature babies, 28 of them (33%) had IVH. In babies with birth weight of under 1000 grams 50%, in 1000-1500 grams 41% and in over 1500 grams 22% had IVH.ln babies under 29 weeks gestation the incidence of IVH was 56% and between 30-34 weeks 22% had IVH.
40% of babies with IVH had sever (grade 3 & 4), 40% had moderate (grade 2) and 20% had mild (grade 1) IVH.
The incidence and severity of IVH in our newborns are almost the same as developed countries.

A clinicoepidemiologic study has been done on patients with muliple sclerosis from 1991-1994 in Shafa Hospital in Kerman.
Diagnostic criteria were based on clinical findings and brain and spine MRI scanning. The patients were followed for 1-5 years. 60 patients were diagnosed of whom 30 were from Kerman city. Female to male ratio is 1.14/1. The patients had no family history for multiple sclerosis. The onset of disease was mostly in the third decade of life.
The most common clinical signs and symptoms were in this order: dizziness (81.7%), fatigability (75%), lower extremities spastic paresia (68.7%), sphincteric disorders (55%), ataxia (48.3%), visual loss (45%), Lhermitte sign (40%), diplopia (36.6%), moderate or sever psychiatric disorders(31. 7%), dysarticulation (11.7%) and seizure (5%).

Ort disease is a contagious viral infection, common in human and domestic animals.
Atter inoculation of virus to the skin a round target shape pustule appears. The virus is a DNA type that exists in perioral area of domestic animals such as sheep and goat. This is a descriptive study of 15012 patients older than 20 years old who were seen in dermatology center clinic in city of Kerman between the years of 1991-1996.
The incidence was 0.4%. Three forth of patients, 60 cases, were female and one forth were male. Most cases were in the age range 21-30 years old, and the rest in 51-60 years. The mean age was 40 years. Thumb and index finger were more involved than any other sites. Biopsy of skin lesions in 14 cases confirmed clinical diagnosis. Due to good prognosis and noninvasive nature of this condition no special treatmene is required, but occasionally in immune compromised patients application of 40% idoxouridine gel locally is helptut.

With the problems resulting from the rapid trend of population growth, the development of procedures capable of decreasing the population growth rate, to inhibit and stagnate this condition is a necessity.
It is obvios that without the contribution and sense of responsibility of men, population control programs and family planning will be unsuccessful. In the present study, attempts have been made to evaluate a newly synthesized analogue of diamino prymidines compounds with the chemical name of 2,4­diamono-5(3,4-dichlorophenyl)-6-isoproppyloxymethyl pyrimidine, (IPO) and trimethoprim (TRI) on male rats.
This study indicates that the compound without any toxic effects, important side effects or serum testosteron changes decreases the fertillity of rats. Also IPO significantly decreases the sperm motility (SM), percent of viable sperm, daily sperm production (DEP) and epidimal sperm reserve (ESR) but TRI decreases the SM and ESR.

Spontaneous g~stric perforation is a rare occurrence in the newborn. It occures most commonly during the first 5 days of life, especially in newborns subjected to severe stress or hypoxia.
The constellation of symptoms typically includes a sudden deterioration in clinical status between the second and fifth day of life, characterized by refusal to eat, vomiting, abdominal distention and respiratory distress. Free intraperitoneal air and fluid are demonstrable on plain X - Ray of the abdomen. Immediate surgical consultation should be sought.
In this article we are reporting a rare case of gastric perforation in a 2 day old newborn.

Today, the iive s of more than 3000000 children are protected by immun ization services in the from of EPI (expanded program of immunization).
This program helps ideal growth and development for mlliotis of children, but in spite of current EPI, preventable diseases are still able to take the lives innocent children.
Cause specific mortality rates of children, due to preventable diseases, show that immunization services alone are not the end of problem, because the important factors, such as giving priority to EPI, regular monitoring of immunization programs, doing practicable studies in this connection and cross sectional evaluations for recognition of strength and weak points of the program, have an important role in continuation of immunization program and keeping the immunization coverage at a favorable ieve/.
With due attention to EPI goals of year of 2000 (such as increasing of vaccine coverag throughout word, neonatal tetanus elimination and polio eradication) and also health policy makers universal efforts in this connection, we decided to review the sitution of immunization coverage of 12 to 24 month old children who are resident in Iran University of Medical Sciences (IUMS) region.
There are 5 districts in IUMS region (west of Tehran, Karadj, Shahriar, Savojbo/agh & Robatkarim).
In this study we calculated the number of clusters of each district on the basis of its poulation and finally 47 clusters (each cluster includes 7 cases) were studied. 53.8% of cases were female and 46.2% were male.
Age distribution of this group of children was normal (normal distribution mean age= 17.7, mode=17). Following results were obtained in this study:BCG vaccination coverage rate = 99.4% DPT.1 vaccination coverage rate = 97.6% DPT.2 vaccination coverage rate = 98.2% DPT.3 vaccination coverage rate = 98.6% DPT.4 vaccination coverage rate = 80.5%.
Drop out of DPT.1 to DPT.3 vaccination coverage = 1% Polio.O vaccination coverage rate = 99.7%.

In this study assesment of anthropometric indicators in children under 6 yr, in western region of Tehran, was evaluated during the month of sept. 1996.
The prevalence of failure in weight for height and height for age indicators was considered according to age groups.
The failure of weight for height (wasting) showed the highest prevalence among children in 48-59 month age group (21.29%).
The failure of height for age (stunting) showed the highest prevalence in under 12 month and 12-23 month age groups 19.2% and 17.3% respectively.

In order to evaluate the effect of breast feeding and rooming-in (R.I.) on the incidence of neonatal infections , this study was performed in Firoozgar Hospital - Tehran from 1982 through 7992.
This study was designed in order to review the birth records of neonates in three periods: 1, Pre-rooming- in (1982-1985),2, Partialy rooming-in (1985-1990) and 3 rooming-in period (7990-1992). In these three groups, prevalence of neonatal infection was analyzed.
The proportion of preterm births and cesarian section devliveries were significantly greather in the thired period.
The rate of neonatal infection was 2.3% in the first period, 2.7% in the sf!cond and 1.5% in the third period.
The incidence of fever was 0.6% in the first period, 0.57% in the second and 0.04% in the third period (only one neonate). Relative risk of fever found 12 times less in the third period.
On the whole the risk of neonatal infection in normal, fullterms, weighing 2500 gram or more was found to be 44% less in the third period, and no case of fever, skin rash and sepsis was found in this period.