فهرست مطالب

  • Volume:4 Issue: 3, 2019
  • تاریخ انتشار: 1398/05/10
  • تعداد عناوین: 8
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  • هیپرکلسترولمی فامیلیال گزارش یک مورد
    شهلا رودپیما* صفحه 1
    هیپر کلسترولمی فامیلی یک بیماری شدید اتوزوم  غالب است. ما در این جا یک پسر 12 ساله مبتلا به فرم  هموزیگوت این بیماری را معرفی می کنیم .
    کلیدواژگان: بیماری قلبی عروقی، هیپر کلسترولمی فامیلی، فرم همو زیگوت
  • آیا تجویز بتامتازون در زایمان های زودرس 34 تا 36 هفته می تواند باعث بهبود نتایج نوزادی شود؟
    مریم ثبوته * صفحه 2
  • بررسی همخوانی تست پوستی توبرکولین و آزمون کوانتی فرون در کودکان دارای بیماری کلیوی کاندید دریافت داروهای سرکوبگر ایمنی در سال94- 1392در بیمارستان علی اصغر تهران
    فرانک داودزاده*، شیرین سیاح فر، رزیتا حسینی شمس آبادی، حسن اتوکش صفحه 3
    مقدمه
    با توجه به اینکه تاکنون بررسی های انجام شده بیشتر طیف سنی بزرگسالان را شامل شده است، این مطالعه با هدف بررسی هم خوانی دو روش TST و Quantiferon  در تشخیص عفونت با مایکوباکتریوم توبرکلوزیس در کودکان دارای بیماری کلیوی کاندید دریافت داروهای سرکوبگر ایمنی انجام شد.

    مواد و روش ها
    در این مطالعه ی مقطعی اینده نگر 31 کودک دارای بیماری کلیوی کاندید دریافت داروهای سرکو بگر ایمنی شامل بیماران  کاندید پیوند کلیه و سندرم نفروتیک از نظر LTBI بررسی شدند. در تمام بیماران ابتدا TST و سپس QFT  در یک آزمایشگاه مشخص انجام شد. در تمام بیماران معاینه بالینی و رادیوگرافی قفسه سینه به منظور رد کردن سل فعال انجام شد. در نهایت داده ها توسط نرم افزار آماری SPSS v. 16 مورد تجزیه و تحلیل قرار گرفت.

    نتایج
    در این مطالعه 31 کودک شامل 17 (54.8٪) نفر New Case سندرم نفروتیک و 14 (45.2٪) نفر کاندید پیوند کلیه با میانگین سنی (4SD= ) 6.86 سال، شامل 10 (32.3٪) نفر دختر و 21 (67.7٪) نفر پسر مورد بررسی قرار گرفتند. میانگین مدت ابتلا به سندرم نفروتیک در بیماران این گروه (SD= +)  1.3هفته بود. طول مدت ESRD در کودکان کاندید پیوند کلیه به طور متوسط (2.83SD=+ ) 6.35 سال بود و میانگین مدت دیالیز در این بیماران (1.25SD=+ ) 1.72 سال بود. تست QFT در تمامی بیماران هر دو گروه منفی بود و تست PPD در 1 (5.9٪) نفر از بیماران مبتلا به سندرم نفروتیک مثبت بود؛ که این اختلاف از نظر آماری معنی دار نبود (p Value= 0.35). ارزش اخباری منفی تست کوانتی فرون در برابر تست پوستی توبرکولین برابر 7/96% و میزان دقت تشخیصی آن نیز برابر 7/96% برآورد گردید.

    بحث و نتیجه گیری
    تست کوانتی فرون دارای ارزش اخباری منفی و دقت تشخیصی بالائی نسبت به تست TST است و لذا بین دو تست کوانتی فرون و PPD در قابلیت افتراق و تشخیص سل نهفته، ارجحیتی وجود ندارد. لذا، با توجه به مقرون به صرفه بودن تست TST، انجام تست کوانتی فرون ضرورتی نمی یابد.
    کلیدواژگان: تست پوستی توبرکولین، کوانتی فرون، مایکوباکتریوم توبرکلوزیس، سندرم نفروتیک، پیوند کلیه
  • مقایسه اثر موضعی شیر مادر با کلرهگزیدین و شیوه خشک نگه داشتن در کلونیزاسیون باکتریال بند ناف نوزادان نارس بستری در بخش مراقبت های ویژه نوزادان، یک مطالعه تصادفی شده دو سو کور
    مژگان یعقوبی* صفحه 5
    مقدمه
    نوزدان نارس از استعداد بالاتر نسبت به ابتلا به عفونت ها برخوردار بوده و بند ناف محیط مناسبی جهت رشد باکتری ها را فراهم می آورد. این مطالعه با هدف مقایسه اثر موضعی شیر مادر با کلرهگزیدین و شیوه خشک نگه داشتن در کلونیزاسیون باکتریال بند ناف نوزادان  نارس بستری در بخش مراقبت های ویژه نوزادان انجام شد.
    روش کار
    این کارازمایی بالینی شاهددار تصادفی شده دو سو کور بر روی نوزادان نارس بستری شده در بخش NICU بیمارستان ولی عصر (عج) بیرجند در سالهای 96-95 انجام پذیرفت. معیارهای ورود شامل سن حاملگی کمتر از 37 هفته، نیاز به بستری در بخش مرافبت های ویژه نوزادان بوده است. نوزادان به صورت تصادفی ساده با تخصیص 1:1:1 بعد از همسان سازی از نظر سن حاملگی و نوع زایمان در یکی از سه گروه خشک نگه داشتن، کلرهگزیدین و شیر وارد شدند. داده ها پس از جمع آوری توسط نرم افزار SPSS21، در سطح معناداری p کمتر مساوی 0.05 آنالیز شدند.
    نتایج
    در مجموع 25 نفر در هر گروه وارد شدند. گروه های مورد مطالعه از نظر جنسیت، روش زایمان، وزن نوزادان و سن حاملگی هنگام تولد همسان بودند. میانگین کلونیزاسیون تراکم باکتری قبل و بعد از مداخله در گروه شیر مادر 533.4±395.9 و  32.04±18.03، در گروه کلرهگزیدین 230.8±184.1 و 26.12± 11.05 و در روش خشک نگه داشتن 287.3±171.7 و 19.51±10.70 بوده است (هر سه 0.001>p). تعداد باکتری ها  کلونیزه شده در نوزادان گروه کلرهگزیدین قبل و بعد مداخله به ترتیب 52 و 20 درصد بوده است (0.008=p). همچنین در نوزادان گروه شیر مادر نیز تعداد باکتری ها  کلونیزه شده بعد از مداخله به طور معناداری کاهش یافت (به ترتیب 64 و 32 درصد با 0.02=p). در نوزادان گروه خشک کردن تعداد باکتری ها  کلونیزه شده قبل مداخله 64 درصد و بعد از مداخله 36 درصد بود که کاهش مشاهده شده معنادار بود (0.03=p) 
    نتیجه گیری
    در مجموع نتایج این مطالعه نشان دهنده موثر بودن هر سه روش خشک نگه داشتن، استفاده از کلر هگزیدین و همچنین استفاده از شیر در کنترل کلونیزاسیون باکتریایی در بند ناف نوزادان نارس بوده است هر چند که بین گروه های مختلف اختلافی وجود نداشته است.
    کلیدواژگان: نوزادان نارس، تولد پیش از موعد، امفالیت، کلونیزاسیون باکتریایی
  • یک سیستم امتیازدهی جدید برای طبقه بندی خطر ابتلا به ادم مغزی در کودکان مبتلا به کتواسیدوز دیابتی
    بهزاد حقیقی اسکی، علی منافی اناری، نازنین سارج لو*، فرهاد ابوالحسن چوب دار، رامین صائمی، رزیتا حسینی، داود امیرکاشانی صفحه 6
  • آنافیلاکسی به ایمونوگلوبین وریدی در یک بیمار CVID
    احمد بهرامی، مریم وفاپور* صفحه 6
  • مدیریت دیابت کتواسیدوز در کودکان
    بهزاد حقیقی اسکی، علی منافی اناری، رامین صائمی، رزیتا حسینی، داود امیرکاشانی، زهرا صراطی، نازنین سارج لو*، فرهاد ابوالحسن چوب دار صفحه 7
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  • Familial hypercholesterolemia: a case report
    Shahla Roodpeyma*, Sima Rafieyian, Reza Shakeri Page 1

    Familial hypercholesterolemia (FH) is a hereditary dislipidemia. Patients present with extremely high level of low-density lipoprotein cholesterol (LDL-C), which is due to mutation in the gene of LDL receptor. Homozygous patients (HoFH) whose incidence is 1 in 1.000.000 are at high risk of premature aortic valve stenosis, and coronary artery atherosclerosis. In homozygous individuals cardiovascular complications can occur in childhood. The current study presented a 12-y-old boy with HoFH who suffered from mild aortic stenosis, and right coronary artery atherosclerosis. The patient underwent a successful coronary artery stenting, and was discharged with pharmacologic therapy.
    Keywords: Cardiovascular disease, Familial hypercholesterolemia, Homozygous form
  • Does antenatal Betamethasone improve neonatal outcome in late preterm births?
    Fariba Almassinokiani, Maryam Sabouteh*, Fahimeh Soheilipour, Maryam Kashanian, Peyman Akbari, Nahid Rahimzadeh Page 2
    Introduction
    Preterm birth is a public health problem and late preterm birth (deliveries between 34-36 weeks of gestation) accounts for 75% of all preterm births. Antenatal Betamethasone can reduce the severity of respiratory distress in preterm infants and its effect is accepted in 24-34 weeks of gestation. Our goal was to determine the neonatal outcomes of Betamethasone prescription in late preterm births.

    Materials and methods
    In a prospective cohort study in a tertiary teaching hospital, the women at 34-36 weeks of gestation and at risk for imminent preterm delivery took one course of Bethametasone arbitrarily according to the on-call physician order (Betamethasone group) and the rate of neonatal respiratory distress and NICU admissions was assessed, and we compared the results with the results of late preterm deliveries without taken antenatal Betamethasone (Control group).

    Results
    We had 213 patients in control group and 187 in Betamethasone group. There was a significant difference between results, in two groups in 34 and 35 weeks deliveries. Frequency of need to respiratory support in Control group was 33.3% and in Betamthasone group was 9.6%. NICU admission in Control group was 33.8% and in Betamethasone group was 10.7% (P=0.00).

    Discussion and conclusion
    In 34 and 35 weeks of gestation, one antenatal Betamethasone course, even a single dose of Betamethasone has a significant effect on reduction of the respiratory distress and NICU admission rate.
    Keywords: Betamethasone, Neonatal Intensive Care Unit, Preterm Birth, Respiratory Distress Syndrome
  • concordance of the tuberculin skin test and quantiferon in child with kidney disease who candidate for immunosuppressive drugs in 1392-94 in aliasghar hospital
    Faranak Davoodzadeh*, Shirin Sayyahfar, Rozita Hosseinbadyy Shamsa, Hasan Otokesh Page 3
    Introduction
    Considering that prevoius investigation has included more adult age range, this study aimed to evaluate the agreement between TST and Quantiferon in kidney disease children who are candidate for immunosuppressive medications.

    Methods
    In this cross sectional study, 31 children with renal disease including nephrotic syndrome and renal transplant candidates nominated for immunosuppressive drugs were evaluated from the point of LTBI. These children underwere TST and QFT. Physical examination and chest x-ray was conducted in all patients who were positive for QFT or TST to rule out active TB. Finally, the data were entered into statistical software SPSS v. 16 and were analyzed.

    Results
    In this study, 31 children including 17 (54.8%) new case of nephrotic syndrome and 14 (45.2%) kidney transplant candidates with a mean age of 6.86 (SD= 4) years were evaluated from whom 10 (32.3%) were female and 21 (67.7%) were male. The average duration of nephrotic syndrome was 1.3(SD= 12.12) weeks. The average duration of ESRD in children undergoing kidney transplantation was 6.35 (SD= 2.83) years and the average duration of dialysis was 1.72 (SD= 1.25) years. QFT test was negative in all patients in both groups and PPD test was positive in 1 (5.9%) patients with nephrotic syndrome; the difference was not statistically significant (p Value = 0.35).

    Conclusion
    QFT test has a high negative predictive value as well as a high diagnostic accuracy compared with TST and thus QFT and TST have a similar diagnostic performance to assess TB, however due to lower cost-benefit of TST, this test is more preferred to QFT.
    Keywords: Tuberculin skin test, QuantiFERON-TB, Mycobacterium tuberculosis, Nephrotic syndrome, Kidney transplant
  • Management of Renal Abscess in 5 years old girl, A Case Report
    Mastaneh Moghtaderi*, Maryam Boroomand, Pooyah Hekmati Page 4

    Renal abscesses are rare in childhood. They have vague manifestations and  any delay in proper diagnosis and treatment could lead to morbidity and mortality. We present a 5 year old girl with multiple renal abscesses after pyelonephritis that lead to total nephrectomy due to multiple drug resistance.The renal abscess may have subtle symptoms. Early diagnosis and treatment strongly prevents undue mortality and morbidity. This case highlights the need of timed diagnosis and proper treatment.  There must be a clinical suspicion of renal abscess if treatments hve not enough effect on clinical condition of patient.
    Keywords: Management of Renal Abscess in 5 years old girlA Case Report
  • A Randomized, Double-Blind Trial of Topical Breast Milk, Chlorhexidine, and Dry Cord Care on Umbilical Cord Bacterial Colonization in Premature Infants in NICU
    Gholamreza Faal, Behjatossadat Bolandi, Mohammadhasan Namaei, Mozhgan Yaghoobi* Page 5
    Introduction
    The purpose of this study was to compare the topical effect of breast milk, chlorhexidine, and dry cord care on the umbilical cord bacterial colonization in premature infants hospitalized in a neonatal intensive care unit.
    Methodology
    This randomized controlled double-blinded trial was performed on premature infants admitted to the NICU of Valiasr Hospital, Birjand in 2016.  The infants were allocated to one of the three study groups of breast milk, chlorhexidine, dry cord care. The data were analyzed using the SPSS (v.21.0) at the level of p-value≤0.05.
    Results
    25 infants were included in each group matched for gender, type of delivery, birth weight, and gestational age. The density (p<0.001) and the number p<0.05) of bacterial colonization reduced after intervention in all groups, significantly. However, the was no difference between the groups.
    Conclusion
    The results of this study indicated the effect of the three procedures of dry cord care, application of chlorhexidine, as well as use of breast milk in the control of the umbilical cord bacterial colonization in preterm infants, although no difference was reported between the study groups.
    Keywords: Premature Infants, Preterm Birth, Omphalitis, Bacterial Colonization
  • A new scoring system for stratifying risk for cerebral edema in children suffering diabetic ketoacidosis
    Behzad Haghighi Aski, Ali Manafi Anari, Nazanin Sarejloo*, Farhad Abolhassan Choobdar, Ramin Saemi, Rozita Hoseini, Davoud Amirkashani Page 6
    Background
    Predicting adverse neurovascular complications due to diabetic ketoacidosis (DKA) particularly in children leads to more appropriately management of disease as well as achievement of acceptable patients' survival. Cerebral edema is a life-threatening adverse event following DKA responsible for high mortality rate. Recently, authors have focused introducing new risk assessment tools for predicting occurrence of brain edema to early prevention of nervous defects and losing consciousness following DKA. The present study aimed to present a new scoring system for predicting brain edema as a serious consequence of DKA among children.     
    Methods
    A retrospective chart review was conducted on 112 children with the definitive diagnosis of DKA who were admitted to Ali-e-Asghar Hospital, Tehran, between 2014 and 2017. The multivariable logistic regression modeling and analysis of the ROC curve were used to determine and stratify parameters predicting high risk groups for cerebral edema.
    Results
    In multivariable logistic regression model, using infant formula (OR = 4.153, p = 0.020), tachypnea on admission (OR = 3.342, p = 0.027), and raised serum creatinine (OR = 1.658, p = 0.026) could effectively predict the likelihood of brain edema following DKA in children. The children with three parameters were scored as 3, two of three parameters scored as 2, one of three parameters scored as 1, and none parameters scored as 0 considering as very high risk (with 8-fold risk for brain edema), high risk (with 5 to 6-fold risk for brain edema), moderate risk (with 2 to 4- fold risk for brain edema) and low risk for brain edema following DKA.
    Conclusion
    We presents a new scoring system for early identifying children prone to cerebral edema following DKA. This scoring system considers triad of history of using formula, tachypnea on admission and raised serum creatinine and thus can stratify DKA patients to low, moderate, high and very high risk for cerebral edema.
    Keywords: cerebral edema, diabetes
  • IVIG Anaphylaxis in a patient with Common variable immune deficiency disease (CVID).
    Ahmad Bahrami, Maryam Vafapour* Page 6
    Common variable immune deficiency disease (CVID) is one of the most common primary immunodeficiency disorders. It causes reduction of immunoglobulin levels and specific antibodies production and enhancement of recurrent and chronic infections risk, especially respiratory infections and gastrointestinal tracts. Here, we report a case of CVID in a 3 years old male with recurrent hospitalizations because of gastroenteritis and sinobronchitis infections that was treated as allergic disease for a long time but he does not improved. During receiving IVIG he had sever reaction and treated as anaphylaxis an admitted to ICU.
    Keywords: primary immunodeficiency disorders, CVID, immunoglobulin, IVIG
  • Management of Diabetic Ketoacidosis in Children
    Behzad Haghighi Aski, Ali Manafi Anari, Nazanin Sarejloo*, Farhad Abolhassan Choobdar, Ramin Saemi, Rozita Hoseini, Davod Amirkashani, Zahra Serati Page 7
    Diabetic ketoacidosis (DKA) is one of the leading causes of mortality in diabetic children. This study aimed to determine the frequency of mortality and complications of DKA during treatment in order to make the necessary modifications with regard to the common treatment problems.
    This was a cross sectional study, where patients were selected by examining the records of 112 children under the age of 15 who had been admitted to the pediatric intensive care unit of Ali Asghar Children's Hospital during 2014-2017 with DKA diagnosis. Required information were extracted from the records. Statistical analysis was performed using Chi-square (χ2) test, independent samples T-test and SPSS, Version 16. P value less than 0.05 was consider significant.
    The mean age of the children was 9.9 ± 3.15 years. In 66% of the patients, the first manifestation of diabetes was DKA. Regarding the type of serum therapy, hyponatremia was observed in 15%, hypernatremia in 4.6%, hyperkalemia in 4.5%, and hypokalemia in 15.2% of the patients during treatment. The mortality rate was 2.7% (3case). While other complication such as cerebral edema were reported in 20 cases (17.9%). Another important point in our study was that, among the initial markers, several clinical and laboratory markers had a high potential for predicting DKA-related mortality, including the need for intubation  at the time of admission, increased inflammatory factors, severe hyperglycemia, evidence of renal dysfunction, hypophosphatemia, and vitamin D deficiency. Also, in predicting cerebral edema, various base markers were identified as predictors that consist of low GCS on admission, tachypnea, increased level of inflammatory factors, increased BUN, severe metabolic acidosis, leukocytosis, thrombocytosis, hypophosphatemia, vitamin D deficiency and increased Hb A1C.
    Keywords: Diabetic Ketoacidosis