Iranian Journal of Pediatrics
Year:2003 | Volume:12 | Issue:4|Papers Count:20

Cases of the complete syndrome of testicular feminization were reported early in the 19th century, but it was in 1950 that Lawson recognized the etiology in a patient with amenorrhoea and failing pubic and axillary hair, which was unresponsive to testesterone therapy. The prevalence of androgen resistance is estimated to be between 1:20.000 and 1:64.000 men and the complete form is the third most common cause of primary amenorrhea. Testicular feminization is transmitted as X-linked recessive trait. In this article, we report on three siblings in a family with complete form of androgen insensitivity. The first case, the youngest of the siblings, was recognized by an inguinal hernia operation.The diagnosis was established by chromosomal analysis, hormonal assays, sonography, and pathological examination after laparatomy.

Non-steroid anti-inflammatory drugs (NSAIDs) are among the most commonly used drugs in rheumatic disorders. This group of drugs has been associated with various degrees of gastroduodenopathy (GD), which is due to inhibition of prostaglandin (PG) synthesis. There are several differences between their side effect in stomach and in duodenum, but all of these drugs have gastrointestinal side effect. Several studies on preventing NSAIDS GD have been performed in Europe and North America. There are several differences in safety profiles between various NSAIDs and NSAIDs GD prevention effect of cimetidine, ranitidine and omperazole. The incidence of NSAIDs GD was different depending on NSAID, and the patients" gentic base. The incidence of NSAIDs-induced gastric ulcers was higher, compared to Europe and North America., and that of duodenal ulcers was lower. The reason is the difference in basic acid output In this study the incidence of NSAIDs GD compared to that of Western patients was the same (gastric ulcer 18%, and duodenal ulcer 14%). The NSAIDs GD preventive effects of omperazole and H2 blocker (cimetidine, ranitidine), were significant when compared to placebo.

Non-steroid anti-inflammatory drugs (NSAIDs) are among the most commonly used drugs in rheumatic disorders. This group of drugs has been associated with various degrees of gastroduodenopathy (GD), which is due to inhibition of prostaglandin (PG) synthesis. There are several differences between their side effect in stomach and in duodenum, but all of these drugs have gastrointestinal side effect. Several studies on preventing NSAIDS GD have been performed in Europe and North America. There are several differences in safety profiles between various NSAIDs and NSAIDs GD prevention effect of cimetidine, ranitidine and omperazole. The incidence of NSAIDs GD was different depending on NSAID, and the patients" gentic base. The incidence of NSAIDs-induced gastric ulcers was higher, compared to Europe and North America., and that of duodenal ulcers was lower. The reason is the difference in basic acid output In this study the incidence of NSAIDs GD compared to that of Western patients was the same (gastric ulcer 18%, and duodenal ulcer 14%). The NSAIDs GD preventive effects of omperazole and H2 blocker (cimetidine, ranitidine), were significant when compared to placebo.

The worldwide application of vaccinations was very successful in eradication of a few diseases, but the other side of this was the problem of complications observed by many doses of the vaccines. In Iran there has been any documented investigation on the complications caused by vaccines. In our present study, we have studied the complications of DPT vaccination in 450 preschool children. 2-7 days after vaccine injection the children were examined for eventual general and local complications and the parents were asked for their observation on their children. We found no serious complications although 82.4 per cent of them showed general and 83 per cent local complications. Fever was the most frequently seen complication (73%), dizziness, loss of appetite, severe and prolonged crying were the next frequently observer complications. The most frequently encountered local complications consisted of pain at injection site (63 %) as well as swelling, erythema and induration. With increasing age, swelling and local erythema was seen more frequently (P<0.001) and induration less frequently (P<0.001). General complications, too, decreased with increasing age and times of vaccination remarkably. This was specially remarkable in the age group 2-7 years (5th injection).

Glucose-Galactose Malabsorption is a rare genetic disorder caused by a defect in glucose and galactose transport across the intestinal brush border. It is characterized by neonatal onset of sever watery, acidic diarrhea. In the past, it usually resulted in death within the first weeks of life. Now that the disease has been identified, children recover if glucose and galactose are withdrawn from their diet. In this article we report on two interesting cases of the disease consisting of one male and one female newborns with sever watery diarrhea beginning in the first days of life. These patients survived with appropriate glucose and galactose free formula.

Fifteen patients with stabilished diagnosis of ulcerative colitis are evaluated. Data was gathered in patients of pediatric endoscopy including: 1) patients history and phisycal examination, 2) endoscopic reports, and 3) pathologic reports of colon biopsies. The results showed that ulcerative colitis is seen equally in both sexes. The most common age in pediatric group is 11-15 years. The common presentations of ulcerative colitis in our study were diarrhea (80%) and hematochesia (86%), and abdominal pain (73%). Severe forms of ulcerative colitis were seen in 26.6% of cases. P-value of correlation of endoscopic and pathologic findings of ulcerative colitis in our cases was 0.08, which is not statisticaly significant, although very close to 0.05 (positive correlation). Except for one case of failure to thrive, extraintestinal findings were not seen in our study.

We report on a 12 year-old female with Criggler-Najjar syndrome Type I. She showed signs of kernicterus like relative decrease in IQ and speech disturbance. Total bilirubin was 29 and direct bilirubin 1 mg/dl. The first child of the parents, a male newborn, died because of high hyperbilirubinemia. One 10 year-old son is normal, and a 3 year-old daughter is also congenitally icteric. The liver of a 16-year old boy who died in an automobile accident was transplanted to her soon after his brain death. The immune system was depressed by methylprednisolone, i.v. cyclosporine, and Cell Cept. In an 18-months follow-up, she had normal liver and renal function as well as normal CBC. 14 months after transplantation, she had her first menstruation.

Diagnosis of breath-holding spells was made for all cases by medical history, physical examination, electroencephalogram, and laboratory findings. Piracetam or placebo was administered to patients on a randomized basis. Therapeutic dose of iron ( 3-6 mg/kg ) was administered in both groups of patients with iron deficiency and prophylactic dose of iron (1-2 mg/kg) was administered in children under 2 years of age. Piracetam in a dose of 40 mg/kg/day (0.15 cc/kg of 33.3 %suspention) and placebo in a dose of 0.15 cc/kg was administered once daily for a period of 2 months. Of the 32 children enrolled, 16 received piracetam and 16 recieved placebo. An overall control of breath-holding spells was observed in 87% of the patients in the group taking piracetam as compared with 100% in the group taking placebo. Complete control of spells was obsereved in 60.2% of piracetam group and 68.8 % of placebo group. Laboratory findings revealed no significant difference between the 2 groups. No significant difference was obsereved between the 2 groups in the control of breath holding spells (Chi2 =1.87, P=0.17).

Infantile spasms (IS) is an age specific epileptic syndrome. With respect to poor response of IS to conventional antiepileptic drugs, scientists tend to find new effective drugs. In this study, 50 infants with IS in the age group of 2-24 months, were divided randomly into two equal groups, treated with Sabril or ACTH and compared for clinical efficacy and drug side effects. After sixth week of treatment in Sabril group there was 28% complete remission and 40% reduction in seizure frequencies while in ACTH group there was 40.9% complete remission and 45.5% reduction in seizure frequencies, showing no significant difference between clinical responses in the two groups. Drug side effects were seen in 16% of patients in Sabril group and 81.8% of those in ACTH group. These differences were significant from statistical point of view. Unfortunately, it is impossible to evaluate visual field constriction, a probable irreversible side effect of Sabril. This justifies the use ACTH as the first line of treatment in IS.

In a descriptive, analytic study with randomly selected sample collection the relation between birth weights, head and arm circumference as well as Apgar score of the newborn with the age of mother was studied. The comparison of these data of the newborns of 130 primipara aged less than 18 years with that of 138 over age 35 years showed that - The mean value of the weight of the young mothers (3177.88 g) was 15 g less that that of older ones (3187.58 g). - Head and arm circumference of the newborns of older mothers were higher than that of younger ones. - Mean value of Apgar score after 1 minute of the neonates of younger mothers (8.6) was lower than that of older ones (8.7). - Mean value of Apgar score after 5 minutes of the neonates of younger mothers (9.9) was higher than that of older ones (9.84).

Ovulation induction drugs in the recent years and modern technologies in treatment of infertility resulted in remarkable increase of multiple births. The goal of the present study was to determine the frequency of multiple births and maternal -fetal as well as neonatal complications of multiple pregnancies in Mashad during a period of 5 years. Out of 19959 deliveries, 99.3 % were singletons, 0.67 % twins and 0.015 % triplets. The incidence of twins delivery was 6.7 per 1000 (1 in 150 cases). For triplets this was 0.15 per 1000 (1 in 6666). The majority of mothers aged 20-29 years, the youngest were 15-20 years old. The incidence of multiple births decreased with increasing parity and age over 29 years. Maternal complications included premature delivery (41.6 %), preeclampsy (11 %), hemorrhage (10.2 %), infection (4.3 %), and placental detachment (1.4 %). Fetal and neonatal complications were observed in 70 % and consisted of prematurity, fetal malformations (7.9%), stillbirth (6.1 %), and multiple birth mortality (13 %). A direct relation was noticed between mother"s age and neonate"s weight. Thus, multiple pregnancy must be seen as a high risk for mother and her newborn and should be managed carefully during pregnancy and in labor.

Screening is the search for asymptomatic illness in a defined population: it is usually performed for the purpose of treatment but sometimes done for counseling or research several programs, such as screening for in born error of metabolism(e.g, phenyl ketonuria, hypothyroidism) are counted the triumphs of contemporary of pediatrics. Screening programs are not always useful but disease can be selected for screening program that has some characters such as: 1. Disease must have a preclinic period 2. Existence of a good screening test 3. Availability of an effective treatment or prevention 4. Early diagnosis and treatment imply a better outcome

We studied hyperactivity syndrome and attention and concentration deficit in preschool children of kindergartens of Tehran. In this relation, etiological factors causing this behavioral disturbance have been also taken into consideration. This is a descriptive, analytic, cross-sectional study including 400 children together with their parents and teachers, performed in 2001. Four samples from every kindergarten were selected randomly. The incidence of hyperactivity and attention and concentration deficit was estimated to be 11%, of which 60% were male. 79% were first born children to their parents. Hyperactivity and deficit of attention and concentration were positively related to the marital position of the parents (divorced parents), occupational absence of parents from home more than 8 hours per day, illiteracy or low education and a history of psychiatric illness of the mother.