Editorial Manager(tm) for Journal of Pediatric Hematology and Oncology Manuscript Draft Manuscript Number: JPHO 06-270

Title: Renal Splenic Heterotopia with Extramedullary Hematopoiesis in a Thalassemic Patient, Simulating Renal Neoplasm :a case Report
Article Type: Clinical and Laboratory Observations Section/Category:

Keywords: Key words: Spleen-Renal- Thallasemia- Heterotopia- neoplasm
Corresponding Author: Dr mehran hiradfar, M.D.
Corresponding Author's Institution: Dr sheikh pediatric Hospital
First Author: Mehran Hiradfar, M.D.
Order of Authors: Mehran Hiradfar, M.D.; Mehran Hiradfar, M.D.; mehran hiradfar, M.D.; Nona Zabolinejad,
M.D.; abdolla Banihashem, M.D.; Mehran Hiradfar, M.D.; Nona Zabolinejad, M.D.; Abdollah Banihashem,
M.D.; mehran Hiradfar, M.D.; Nona Zabolinejad, M.D.; Abdollah Banihashem, M.D.
Manuscript Region of Origin: IRAN, ISLAMIC REPUBLIC OF

Abstract: Abstract
Intrarenal splenic tissue (splenorenal heterotopia)(SRH) is a rare condition . Simultaneous existence of SRH
and extramedullary hematopoiesis in a same patient can present as a mass of kidney .
We present an intresting case of splenorenal hetrotopia with EMH in a thallasemic child simulating as a
renal neoplasm .This is the first reported case in the right kidney.


Diagnostic Value of Ultrasound Findings in Mesenteric Lymphadenitis in Children with Acute Abdominal Pain

Backgrounds/Objective: The aim of this study was to compare abdominal sonographic features of patients with mesenteric lymphadenitis versus sonographic features of the asymptomatic children, in order to identify diagnost ic criteria for mesenteric lymphadenitis. Patients and Methods: Ultrasound data from 113 children with acute abdominal pain whose suspected sonographic diagnosis was mesenteric adenitis or ileitis was inspected in a child hospital in March 2004 to March 2005. The fina l diagnoses were established after patient management and follow-up. As for the control group, a hundred asymptomatic children were referred from health centers or kindergartens.

Results: No para-aortic lymphadenopathy was found in any of the subjects from the study and the control groups. The number of enlarge d mesenteric lymph nodes observed in symptomatic children was significantly greater than the figure in asymptomatic children (P 0.0001). The highest rate of sensitivity was seen with the presence of RLQ lymphadenopathy (94.7%) and the highest rate of specifici ty was seen with the presence of lymphadenopathy in two or three areas of LUQ or mi d-abdomen together; lymphadenopathies larger than 6mm in RLQ; presence of more than 4 lymph nodes (with any size) in a sonographic image from RLQ; or thickening of terminal il eum more than 8mm (92%-100%) in the diagnosis
of mesenteric lymphadenitis. Conclusion: In children with acute abdominal pain due to causes such as appendicitis which cannot be confirmed by ultrasound and the f indings consist of only enlarged mesenteric lymph nodes (≥4mm) and/or mural thickening of the terminal ileum ( ≥8mm), the probable diagnosis would be mesenteric adenitis-ileitis and surgery is not required; but close observation is recommended.

 

 

بررسي هيپوگناديسم در بيماران مبتلا به تالاسمي ماژور

شماره نود و يك ، سال چهل و نهم
بهار 1385

دكتر رضا رجبيان¹، دكتر رباب ابوترابي²، دكتر شكوفه بنكداران³، دكتر زهرا بديعي⁴،

دكتر محمد خواجه دلوئي⁵

تاريخ دريافت: 11/3/84 - تاريخ پذيرش: 24/1/84

خلاصه

مقدمه: گرچه درمان هاي جديد باعث طولاني شدن عمر و بهبود كيفيت زندگي بيماران مبتلا به تالاسمي شده است ولي هنوز مشكلات زيادي از جمله عوارض اندوكرين و به خصوص هيپوگناديسم در اين بيماران شايع است که به علت رسوب آهن در سلولهای گنادتروپ هيپوفيز ذکر گرديده است. هدف از اين مطالعه بررسي شيوع و شناسايي عوامل د خيل در ايجاد هيپوگناديسم در بيماران مبتلا به تالاسمي ماژور مي باشد.

روش کار: در اين مطالعه توصيفی 47 بيمار تالاسمي ماژور با سن بالاتر از 14 سال که در سال 1383 به بيمارستان دکتر شيخ مراجعه کرده بودند مورد مطالعه قرار گرفتند، جهت بررسی هيپوگناديسم. ميزانFSH, LH و استراديول در زنان و در مردان ميزان تستوسترون اندازه گيري و رابطه آنها با متغيرهائي شامل شروع و طول مدت دريافت ترانسفوزيون خون و طول مدت و دوز دسفرال دريافتي، سطح فريتين و يافته هاي باليني مربوط بررسی گرديد. مشخصات فردی، نتايج آزمايشگاهی در پرسشنامه ای ثبت گرديد. اطلاعات جمع آوری شده با استفاده از آمار توصيفی و آزمونهای تی و من ويتنی پردازش شد.

نتايج: ميانگين وزن اين بيماران 4/8±5/42 كيلوگرم و ميانگين قدي اين بيماران 2/10 ±151 سانتيمتر بود. فراواني كوتاهي قد و كم وزني1/68% و فراواني هيپوگناديسم در اين بيماران 8/63% بود. شيوع هيپوگناديسم هيپوگنادوتروفيك در جنس مذكر بيشتر بود (025/0=p) اما رابطه معني داري بين هيپوگناديسم با طول مدت ترانسفوزيون خون، زمان شروع و دوزدسفرال دريافتي و سطح فرتين وجود نداشت.

نتيجه گيري : عوارض اندوكرين بيماري تالاسمي متفاوت مي باشد و با توجه به يافته هاي فوق به نظر مي رسد عوامل ديگري غير از افزايش تجمع آهن در ايجاد عارضه هيپوگناديسم د خالت داشته باشد.

واژه های كليدي : تالاسمي ماژور، هيپوگناديسم، عوارض آندوکرين

 

Spring 2004; 47(83):79-89

QUANTITATIVE ASSESSMENT OF CARDIAC FUNCTION IN CHILDREN WITH BETA- THALASSEMIA MAJOR, USING THE DOPPLER MYOCARDIAL PERFORMANCE INDEX

MOTTAGHI MOGHADDAM H.,ALIZADEH B.,BANIHASHEM A.,AFZALNIA S

Introduction: Cardiac disease and early death due to myocardial injuries because of Iron overload and hemosiderosis are still the major problems in the management of patients with beta-thalassemia major (BTM). Valuable echocardiographic techniques have been limited in early detection of cardiac dysfunction in these patients. A new Doppler index combining systolic and diastolic time intervals (the Tei index) has been reported to be useful for assessment of global ventricular function in many cardiac diseases. The purpose of this study was to assess global ventricular function in children with BTM using Tei index and also to determine its correlation with hematologic parameters.
Material and Methods: This analytic cross sectional study was performed on 59 patient with Beta-Thalassemia Major during Jan.2003 until Feb.2004 in hematologic clinics of Imam Reza and Dr. Sheikh Hospitals. Complete physical examination and Doppler Echocardiographic study was done for all the patients and patients Tei indexes were compared with normal values in other studies statistically.
Results: The mean Tei index of left ventricule (LV) and right ventricule (RV) was 0.35±0.11 (mean±SD) and 0.28±0.12 (mean±SD) respectively. There was a significant difference between both right and left ventricular Tei index in-patients with BTM and healthy children. There was also a significant correlation between mean blood transfused volume per year and Tei index in both right (PV? 0.05) and left ventricule (PV? 0.05). We also find a significant difference between two groups of thalassemic patients according to their mean pre-transfused Hgb per year (Hgb? 9 g/dl and Hgb >9 g/lit) for left ventricular Tei index.
Conclusion: The Tei index is a simple, feasible and reproducible measure of global cardiac function in-patients with BTM with early myocardial dysfunction.