رحیم وکیلی
-استاد غدد واندوکرینولو‍ژی کودکان، دانشگاه علوم پزشکی مشهد، مشهد، ایران
[ 1 ] - استفاده از بی فسفونات‌ها در فیبرو دیسپلازی استخوان
مقدمه دیسپلازی فیبروز یک ضایعه اسکلتال خوش‌خیم است که یک یا چند استخوان را درگیر می‌کند. این اختلال یک بیماری خوش‌خیم مادرزادی است که هر دو جنس را یکسان درگیر می‌کند. این ضایعات ممکن است باعث دفورمیتی استخوان، شکستگی یا دردهای مزمن اسکلتی شود و یک عامل مساعد کننده برای شکستگی‌های پاتولوژیک است. معرفی بیمار در این مقاله دختری 12 ساله معرفی می‌شود، که از 2 سال قبل دچار درد دراستخوان فمور و لگنش ...
[ 2 ] - بررسی یافته های بالینی سندروم نونان در ایران
مقدمه :سندروم نونان توسط دکتر ژاکلین نونان در حدود چهل سال قبل، معرفی شده که با کوتاهی قد، چهره دیسمورفیک، اختلالات قلبی مشخص می شود. تنوع بسیاری در فنوتیپ این بیماران وجود دارد.شیوع این سندروم حدود 1در 1000_2500 تخمین زده می شود.ویژگی های اصلی بیماران مبتلا به این سندروم شامل چهره خاص ، بد شکلی های قفسه سینه و ستون فقرات،قد کوتاه، نقایص قلبی که اکثرا همراه با استنوز پولمونر و کاردیومیوپاتی هیپ...
[ 3 ] - ارتباط بین معیارهای رشد و سطح لیپیدهای سرم با میزان هموگلوبینگلیکه درکودکان و نوجوانان دیابتی
مقدمه دیابت نوع I شایعترین اختلال غددی متابولیکی در کودکان است که بـا عـوارض زیـادی منجملـه اخـتلال رشـد ممکن است همراه باشد. هدف از این مطالعه بررسی نقش دیابت روی رشد (وزن- قـد)، پارامترهـای رشـد و میـزان چربیهای خون است. روش کار در یک پژوهش مقطعی توصیفی 154 کودک دیابتی از مرداد 85 لغایت تیر 86 از نظـر معیارهـای رشـد وزنـی ، قدی، طول مدت بیماری، متوسط هموگلوبین گلیکه و لیپیدهای خون مورد ارزیاب...
[ 4 ] - Papillary Carcinoma Thyroid in a Nine-year-old Child: A Case Report
Thyroid enlargement and nodules are very rare in children, but when they occur, the chance of malignancy among these nodules is very high. Thyroid carcinoma is rare in childhood, but in the last two decades, its incidence has increased two-fold. A painless nodule in the neck is the most common presentation of the disease. Dysphagia, hoarseness, cervical lymphadenopathy, weight loss, and f...
[ 5 ] - 4-Hydroxybutyric Aciduria as a Rare Presentation of Global Developmental Delay in Children: Case Report of Two Different Patients
Succinic semialdehyde dehydrogenase (SSADH) deficiency or 4-Hydroxybutyric Aciduria is an autosomal recessive inherited disorder of amma-aminobutyric acid (GABA) degradation. It is characterized by developmental delay, infantile-onset hypotonia, cognitive impairment language deficit, and ataxia. Epilepsy, aggression, Hyperkinetic behavior, hallucinations, and sleep disturbances have been descri...
[ 6 ] - Lingual Thyroid: A Case Report and Literature Review
Thyroid ectopia is a dysgenesis of thyroid gland and Lingual position represents the most frequent ectopic location accounting up to 90% of ectopic cases. Hypothyroidism is commonly present because of absence of a normal thyroid gland in most instances. Primary hypothyroidism in juvenile population generally leads to retardation of linear growth and delay or even arrested puberty. We present a ...
[ 7 ] - Black Urine
A 2-year-old boy was born at term of healthy, non-consanguineous Iranian parents. His mother attended in the clinic with the history of sometimes discoloration of diapers after passing urine. She noticed that first at the age of one month with intensified in recent months. His Physical examination and growth parameters were normal. His mother denied taking any medication (sorbitol, nitrofuranto...
[ 8 ] - Immunization Coverage in WHO Regions: A Review Article
In 1974, the World Health Organization (WHO) established the Expanded Program on Immunization (EPI) to ensure that all children have access to routinely recommended vaccines. Since then, global coverage with the four core vaccines (Bacille calmette guérin vaccine [for protection against tuberculosis], Diphtheria-tetanus-pertussis vaccine [DTP], Polio vaccine, and Measles vaccine) has increase...
[ 9 ] - McCune-Albright Syndrome: A Case Report and Literature Review
McCune-Albright syndrome (MAS) is a rare, heterogenous, clinical condition caused by a rare genetic mutation. The disorder is more common in females and is characterized by a triad of cutaneous, bone and endocrine abnormalities. We describe a girl patient with MAS having precocious puberty and multiple cafe-au-lait macules and deforming polyostotic fibrous dysplasia of bone. Clinical presentat...
[ 10 ] - Maternal Knowledge and Attitude toward Exclusive Breast Milk Feeding (BMF) in the First 6 Months of Infant Life in Mashhad
Introduction: Breast milk is a complete food for growing children until 6 months of age, and mothers, as the most important child health care, play a decisive role in their growth. So promoting their attitude toward the benefits of breastfeeding ensures guarantee child health in the future. This study aimed to assess maternal knowledge and attitude of Mashhad toward exclusive BMF in the first ...
[ 11 ] - Congenital Rickets: Report of Four Cases
Introduction: Vitamin D deficiency and rickets continue to be health problems in developing countries and most of the infants with congenital rickets may present with hypocalcemic seizure. Case Report In this article, the report on four infants who presented with hypocalcemic seizures but subsequently were found to have congenital rickets is presented. All of them had hypocalcaemia and low le...
[ 12 ] - Assessment the Relationship Between Parents' Literacy Level with Children Growth in Mashhad: An Analytic Descriptive Study
Introduction: Present children are the investments of community in the future. Preparing children health which leads to the stability of community health, provided to accurate implementation of educational and health programs in the community and especially in mothers. So it is necessary to determine the relationship between parents' literacy with growth rate in children. Materials and Methods...
[ 13 ] - Precocious Puberty: An Unusual Presentation of Hypothyroidism
Hypothyroidism is usually associated with delayed pubertal development but in rare occasions precocious puberty may ensue which is seen in cases of prolonged and untreated hypothyroidism. This is also called the Van Wyk Grumbach syndrome. Here we present 4 cases of precocious puberty due to hypothyroidism.
[ 14 ] - Iron and Multivitamin Supplements in Children and its Association with Growth rate
Introduction: Vitamin deficiency and iron deficiency anemia are common nutritional problems, at least in children under 5. These materials shortage, especially in the first two years of life, impair physical and brain growth, reduces the child's learning ability, reduces body resistance against infections, behavioral changes, apathy and finally social and economic adverse consequences would be ...
[ 15 ] - Mutational analysis of ARSB gene in mucopolysaccharidosis type VI: identification of three novel mutations in Iranian patients
Objective(s): Mucopolysaccharidosis VI (MPS VI) or Maroteaux-Lamy syndrome is a rare metabolic disorder, resulting from the deficient activity of the lysosomal enzyme arylsulfatase B (ARSB). The enzymatic defect of ARSB leads to progressive lysosomal storage disorder and accumulation of glycosaminoglycan (GAG) dermatan sulfate (DS), which causes harmful effects on various organs and tissues an...
[ 16 ] - Type 1 diabetes and Ramadan fasting: A narrative review
Ramadan fasting is an important pillars of Islam .although patients and children expected from fasting during Ramadan, but every diabetic adolescents intends to fast should consult his diabetes management time, and intensive monitoring of blood glucose and urine ketones .close observation by endocrinologist, and weekly follow- up and highly individualized planning for each diabetic person is es...
[ 17 ] - Comparison of The Effect of Ketogenic Diet and Low Caloric Diet On Weight Loss in Iranian Obese and Overweight Children
Abstract Overweight and obesity has increased in prevalence over the last two decades in many developed and developing countries including Iran. The aim of this present study was to compare the weight reduction effects of ketogenic and low calorie diets on overweight and obese Iranian children. Seventy-six overweight or obese children aged 9-16 years recruited from outpatient Ghaem Hospital, Nu...
[ 18 ] - درمان با کلسیم خوراکی دوز بالا در بیماران مبتلا به ریکتز وابسته به ویتامین D نوع 2
سابقه و هدف: ریکتز وابسته به ویتامینD نوع 2(VDDR2= Vitamin D-Dependent Rickets Type 2) بیماری نادر ژنتیکی ناشی از موتاسیون در گیرنده ویتامین D بوده و منجر به مقاومت به اثرات بیولوژیک کلسیتریول خواهد شد. این بیماری معمولا بسته به نوع جهش، حتی به دوزهای بالای کلسیتریول مقاوم بوده و درمان موفق این بیماران مستلزم اصلاح هایپوکلسمی از طریق تجویز کلسیم با دوز بالا و حذف مسیر معیوب رسپتور است. تجویز ...
[ 19 ] - Mccune-Albright Syndrome: A Case Report Associated with Pamidronate Therapy and Literature Review
McCune-Albright Syndrome (MAS) is a rare sporadic disease characterized by bone fibrous dysplasia, Café au lait spots and a variable association of hyperfunction endocrine disorders. There is not any certain treatment available for this syndrome, and both physical and emotional disability in these patients is still a major concern for physicians. In present report we have described a 10- year-o...
[ 20 ] - Comparison of Novel Coronary Artery Disease Risk Factors between Obese and Normal Adolescent
Background: Coronary artery disease is considered as the most common cause of death in all societies including Iran. This study seeks to compare the new risk factors of coronary-artery diseases in obese adolescents and control group.Methods: In this cross-sectional study, amongst the obese adolescents registered in the nutrition clinic of Ghaem Hospital, 80 individuals were selected. As the con...
[ 21 ] - Gyrate Atrophy of the Choroid and Retina: A Case Report
Introduction Gyrate atrophy of the choroid and retina is a metabolic disorder, which is inherited in an autosomal recessive pattern. Although gyrate atrophy is rare, it is concerning as it results in blindness. It is characterized by hyperornithinemia, retinal atrophy, leads to progressive myopia and tunnel vision,...
[ 22 ] - Congenital Adrenal Hyperplasia and Schmid Metaphyseal Chondrodysplasia in a Child
Congenital adrenal hyperplasia (CAH) is a group of hereditary diseases, which are autosomal recessive. CAH occurs due to defect in one of the cortisol coding genes and often clinically presents itself with signs of androgen overproduction. In this article, we report a case of CAH and Schmid metaphyseal dysplasia. Our literature review indicated that this report is the first attempt on CYP11B1 a...
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