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Article
Bilinorm administration combined with Phototherapy in the management of neonatal jaundice: a hospital based clinical trial

Author: Numan N. Hameed
Journal: Journal of the Faculty of Medicine مجلة كلية الطب ISSN: 00419419 Year: 2010 Volume: 52 Issue: 4 Pages: 395-397
Publisher: Baghdad University جامعة بغداد

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Abstract

Background: Neonatal jaundice (NJ) is a common problem worldwide and frequent in Iraq . Several reports were published on prevalence, distribution, causes and treatment of NJ.This clinical trial was carried out to demonstrate the effect of combinations of Castrol oil, riboflavin and magnesium in mechanical elimination of bilirubin after enhancing hepatic excretion by phototherapy.Patients & methods: This clinical trial included a total of 61 significantly jaundiced neonates who were admitted to the special care baby unit of Children Welfare Teaching Hospital, medical city complex, Baghdad, Iraq, during the period June 1st to Dec.31st 2007. Phototherapy was applied alone in 30 neonates (group 1) and phototherapy with Bilinorm in 31 neonates (group 2),(Bilinorm oral gel constituted of riboflavin sodium phosphate, magnesium oxide and castor oil). Student’s t test was used to examine the differences in variables between the two groups.
Results: There were no significant differences between the two groups in body weight, age of onset of jaundice, age on admission, and TSB on admission (p =0.9,0.4, 0.4, 0.4, respectively). A significant difference in total decrease in TSB was noticed between group II (7.2 ± 2.4) and group I (5.9 ± 1.8)(p = 0.02). Days of hospitalization were significantly lower in group II (2.4 ± 0.8 days) than in group I (3.3 ± 1.5 days) (p = 0.009), and hours of phototherapy were significantly lower in group II (47.4 ± 19.7 hours) than in group I (66.8 ± 27.4 hours) (p= 0.002).
Conclusions: There was a statistically significant difference regarding total decrease in TSB, days of hospitalization and hours of phototherapy when both Bilinorm and phototherapy were used than when phototherapy was used alone. So mechanical elimination of bilirubin from intestinal lumen may enhance the effect of phototherapy.

Keywords

Bilinorm --- Phototherapy --- neonatal --- jaundice.


Article
OBSTRUCTIVE JAUNDICE IN BASRAH

Authors: Abutalib Bader Abdullah --- Zeki A Al-Faddagh
Journal: Basrah Journal of Surgery مجلة البصرة الجراحية ISSN: 16833589 / ONLINE 2409501X Year: 2011 Volume: 17 Issue: 2 Pages: 45-57
Publisher: Basrah University جامعة البصرة

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Abstract

Obstructive Jaundice is a common surgical problem presenting to hospitals as it resulted from many etiological factors like choledocholithiasis or periampullary tumors especially CA head of pancreas. According to the difference in these etiological factors and their progress, symptoms and signs vary in different patients. Diagnostic tools like US, CT scan, MRCP, ERCP and others vary in their ability in diagnosing the main etiology and the operative procedures also differs according to the etiologies, ranging from least invasive like ERCP to very sophisticated procedures like Whipple's procedure for CA head of pancreas. Many factors may affect the morbidity and mortality like the age of patients, presenting etiology and the presence of associated comorbid diseases. This study aimed to demonstrate the main etiological factors of obstructive jaundice in Basrah and the commonest presenting symptoms and signs. Also to study the most applicable investigations and compare their results according to their accuracy in diagnosing the etiology, and to study the most common surgical intervention applied to relieve the obstruction in obstructive jaundice and hospital morbidity and mortality. Both retrospective and prospective study was done in Basrah between January 2006 and December 2009, 243 patients with obstructive jaundice were included in this study from the main general hospitals and private hospitals in Basrah. Data were collected about the presenting clinical features, the diagnostic techniques, operative procedures and the causes of in hospital mortality and morbidity and were analyzed so that a complete picture of these details can be assessed for obstructive jaundice in Basrah. The study shows no significant difference between male and female in obstructive jaundice. The majority of cases found in the age group 50-59 years. Most common etiology was choledocholithiasis. The most frequent applied investigation was the liver function test which was done to all patients. Imaging techniques were applied variably with the US was the most applied while MRCP and ERCP were the least; however, the accuracy was higher with the latter two techniques. Intervention depends on the main etiology: for the choledocholithiasis, most common operation was CBD exploration, for CA head of pancreas the most common operation done was bypass procedure and for complicated hepato-biliary hydatid disease the CBD exploration with T-tube was the common. The post operative morbidity was 20.07% mostly due to respiratory complications, while mortality was 9.86 % mostly due to sepsis. In conclusion, the most common cause of obstructive jaundice in Basrah is choledocholithiasis and CA head of pancreas comes second. ERCP and MRCP are the least applied imaging techniques in diagnosis of obstructive jaundice. The threshold for their application was very high. The least applied intervention to treat obstructive jaundice was the therapeutic ERCP, while the most common was open surgical procedures.

Keywords

OBSTRUCTIVE --- JAUNDICE --- BASRAH


Article
Unconjugated Neonatal Hyperbilirubinemia: Evaluation and Treatment
تَقْييم و مُعالَجَة فَرْطُ بِيِلِيروبينِ الدَّمِ اللامُقْتَرِن الولادي

Authors: Nahla I. Al-Gabban د.نهلة الكبان --- Haider Nadhim Abd د.حيدر ناظم --- Essam Ahmed Abd د. عصام احمد عبد الله
Journal: IRAQI JOURNALOF COMMUNITY MEDICINE المجلة العراقية لطب المجتمع ISSN: 16845382 Year: 2010 Volume: 23 Issue: 3 Pages: 165-168
Publisher: Al-Mustansyriah University الجامعة المستنصرية

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Abstract

Background: neonatal jaundice is a common problem with a lot of mistakes that happen during its management, requiring the doctors and medical staff to follow a certain guidelines for dealing with this disease to obtain the best results.Objective: (1) to study some of the causes of neonatal unconjugated hyperbilirubinemia. 2) To discuss the causes and proper line of therapy.(3) To discuss the complications associated with the management of this problem. Methods: The study included 100 neonates aged 0-14 days with hyperbilirubinemia who were admitted to the Central Teaching Hospital for Children in Baghdad during the period from 1st of February – 1st of June2008. Term infants with TSB ≥ 22mg/dl were treated by exchange transfusion and phototherapy (Group A, 38 patients). Those with TSB level from 13-22mg/dl were treated by phototherapy only (Group B, 62 patients). These decisions were taken mostly according to the TSB level, gestational age and body weight.Results: Twenty five percent of cases had hemolytic cause (Rh-isoimmunization, 13%; ABO-incompatibility, 10%; G6PD-deficiency, 2%). Other causes include: pretermaturty, 33%; sepsis, 8%; congenital CMV infection, 1%; and there were 33 (33%) infants had no evidence of hemolysis or other serious problems. There were 2 deaths in our study after exchange transfusion due to septicemia. The mortality rate after exchange transfusion in the study was 4.4% (from total number of exchange transfusion processes). Conclusions: There appears to be lack of a protocol for the management of cases of neonatal unconjugated hyperbilirubinemia. The cause of hyperbilirubinemia is usually overlooked.Key words: Neonate, hyperbilirubinemia, Jaundice.

خلفية: اليرقان الولادي هو مشكلة شائعة مع الكثير من الأخطاء التي تحدث أثناء العلاج مما يتطلب من الأطباء والكادر التمريضي تتبع دلائل ارشادية خاصة لغرض التعامل مع هذا المرض للحصول على أحسن النتائج. الهدف:(1) هو لدراسة بعض اسباب اليرقان الولادي اللامقترن في مستشفياتنا.(2) لمناقشة الخط الصحيح للعلاج.(3) لمناقشة الممارسة اليومية في مستشفيات الأطفال , والمضاعفات المترافقة مع التعامل مع هذه المشكلة.الطريقة: دراسة استباقية شملت100 وليد مصابين باليرقان الولادي كانت أعمارهم بين 0-14 يوما ادخلوا إلى مستشفى الطفل المركزي التعليمي في بغداد.الولدان الناضجين والذين تبلغ نسبة البيليروبين لديهم أكثر من أو يساوي 22ملغم/100 مل عولجوا بعملية تبديل الدم مع علاج ضوئي ( 38 مريض).أولئك الأطفال ألكاملي النمو والذين تراوحت النسبة لديهم من 13-22ملغم/100 مل عولجوا بالعلاج الضوئي فقط(62 مريض). أخذت هذه القرارات على الأغلب بالاعتماد على نسبة البيليروبين,العمر ألحملي للجنين,والوزن. النتائج: 25٪ من الحالات كانوا يعانون من تحلل الدم (تَمْنيعٌ راهائيٌّ إِسْوِيّ13%, تَنافُرُ الزُّمَرِ الدَّمَوِيَّةِ10% ونقص إنزيم الباقلاء2%).والأسباب الأخرى تشمل أنتان دموي 8٪, الخداج 33٪, والتهابات خلقية ( ولادية) 1٪ , و كان هناك 33 (33%) رضيع لا توجد ليهم أي علامة تدل على وجود تحلل في الدم أو أية مشاكل أخرى.كانت الوفيات في دراستنا 2 بعد عملية تبديل الدم نتيجة الأنتان الدموي.بلغت نسبة الوفيات بعد تبديل الدم في دراستنا 4.4٪ (من العدد الكلي لعمليات تبديل الدم). الاستنتاج: يبدو أن هناك فقدان لسياسة منتظمة و قياسية في علاج حالات اليرقان الولادي اللا المقترن. إن سبب اليرقان عادة يتغافل عنه.


Article
The Role of Magnetic Resonance Imaging and Magnetic Resonance Cholangiopancreatography in Patients with Obstructive Jaundice
استخدام الرنين المغناطيسي وتلوين القنوات الصفراوية لدى مرضى اليرقان الانسدادي

Authors: Najeeb S. Jabbo د. نجيب صليوة جبو --- Ayad Mahmood Subhi د. اياد محمود صبحي --- Ra'ad H. AL-Kayat د. رعد حفظي الخياط
Journal: IRAQI JOURNALOF COMMUNITY MEDICINE المجلة العراقية لطب المجتمع ISSN: 16845382 Year: 2006 Volume: 19 Issue: 3 Pages: 253-258
Publisher: Al-Mustansyriah University الجامعة المستنصرية

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Abstract:Objectives: The aim of this study is to evaluate the sensitivity, specificity and diagnostic accuracy of magnetic resonance cholangio-pancreatography in patients with obstructive jaundice after comparing it with the final diagnosis after surgical exploration and histopathological study. Methods: This study was held at Al-Yarmouk Teaching Hospital where patients with obstructive jaundice were investigated with magnetic resonance cholangio-pancreatography followed by surgical exploration and confirmation of diagnosis. Comparison of both results was carried out.Results: There were 42 patients, 26 females and 16 males. Their mean age was 59 years. 17 patients had common bile duct stones, 17 had malignancy, 3 had hydatid cyst, 2 had benign stricture and one patient had Mirrizi`s syndrome, chronic liver disease and biloma for each. After comparison with the final diagnosis after surgical exploration, the sensitivity, specificity and diagnostic accuracy of magnetic resonance cholangio-pancreatography were 100%, 92% and 95.2% respectively for those with common bile duct stones, while they were 94.1%, 100% and 97.6% respectively for those with malignancy.Conclusion: Magnetic resonance cholangio-pancreatography is of high diagnostic accuracy when used for patients with obstructive jaundice of different causes. In addition to its characters of being a non-invasive technique that need neither specific preparation nor anesthesia. We recommend using it on a wider scale in our country.Keywords: Obstructive jaundice, diagnosis, MRCP

الملخص:الهدف: هدف هذه الدراسة هو تقييم حساسية ومدى الوثوق ودقة الشخيص لجهاز الرنين المغناطيسي في مستشفى اليرموك التعليمي في حالات اليرقان الأنسدادي وبعد المقارنة مع التشخيص النهائي بواسطة التداخل الجراحي مع أو بدون الفحص النسيجي.الطرق: اجريت هذه الدراسة التتبعية لحالات اليرقان الأنسدادي في مستشفى اليرموك التعليمي. أجري لجميع المرضى فحص الرنين المغناطيسي ثم تم متابعتهم بالأستكشاف الجراحي وتثبيت التشخيص بالتحليل النسيجي أو اعتمادا على الملاحظات الجراحية. النتائج: شملت الدراسة 42 مريضا ( 25 من الأناث و 16 من الذكور)0 معدل العمر كان 59 سنة0 عدد المرضى المصابين بالأورام الخبيثة وحصى قناة الصفراء 17 مريضا لكل منهم, 3 مصابين بالأكياس المائية ومريضين مصابين بتضيق قناة الصفراء0 بعد التحقق من التشخيص اعتمادا على نتائج الأجراء الجراحي كانت حساسية الفحص بجهاز الرنين المغناطيسي 100 % في حالات الحصى و9401 % في حالات الأورام0 أما دقة التشخيص فكانت95,2 % في حالات الحصى و97,6 % في حالات الأورام.الأستنتاج: أن الفحص بواسطة الرنين المغناطيسي لحالات اليرقان الأنسدادي ذو حساسية ودقة عالية وخالية من المضاعفات. بالأضافة الى أنها لا تحتاج الى التخدير ولا اجراء تحضيرات خاصة.


Article
NEONATAL JAUNDICE WITH URINARY TRACT INFECTION

Authors: Shatha H. Ali شذى حسين علي --- Deia K. Khalaf ضياء خلف --- Sinan A. Ibrahim سنان ابراهيم
Journal: IRAQI JOURNAL OF MEDICAL SCIENCES المجلة العراقية للعلوم الطبية ISSN: P16816579,E22244719 Year: 2016 Volume: 14 Issue: 1 Pages: 45-49
Publisher: Al-Nahrain University جامعة النهرين

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Background:Urinary tract infection is a common and serious clinical problem in newborns. Renal scarring, hypertension, and even kidney failure can be prevented by early diagnosis and treatment of urinary tract infection. Jaundice is an important and sometimes the presenting feature of urinary tract infection.Objectives:To evaluate the frequency and bacterial profile of urinary tract infection in full term and preterm newborns with hyperbilirubinemia in the first two week of life, and the relation with some demographic parameters.Methods:Seventy two jaundiced neonates were studied. Hematocrit, white blood cell count, reticulocyte count, blood group and Rh, bilirubin (direct and total), Coombs test, and glucose 6-phosphate dehydrogenase level were evaluated. Septic screen and thyroid function test, urinalysis and culture for all patients.Results:Twenty two (30%) out of 72 complain from urinary tract infection. Majority of them were full term 15 (68%), and Body weight > 2.5 Kg 12 (54%), fifteen delivered by normal vaginal delivery (68%). Breast feeding was recorded in 12 (54%), total serum bilirubin level above 20 mg/dl was found in 19 (86%). Gestational age, Normal Vaginal Delivery, Type of feeding, and total serum bilirubin level more than 20 mg/dl had significant correlation with urinary tract infection in jaundiced neonates, while birth weight was not significant. Commonest isolated bacteria was Escherichia coli in 11 (50%), staphylococcus infection in 2 cases (9.1%) have significant correlation with male gender. Ultrasound result was only positive in one (4.5%) neonate and showed pelvi-calceal obstruction.Conclusion:Urinary tract infection is common among jaundiced neonates particularly in males. Escherichia coli is the commonest causative bacteria. Gestational age, normal vaginal delivery, type of feeding, and total serum bilirubin level more than 20 mg/dl had significant correlation with urinary tract infection in jaundiced neonates, while birth weight was not significant.Key word:Neonatal jaundice, hyperbilirubinemia, UTI


Article
The Study of the effect of serum zinc level in the mothers and Neonates on neonatal jaundice in al-diwanyia
دراسة تأثير مستوى الزنك في المصل لدى الأمهات وحديثي الولادة على اليرقان الولادي في الديوانية

Author: Nasma N. Al-Hajjiah نسمة ناجي الحجية
Journal: Al-Qadisiyah Medical Journal مجلة القادسية الطبية ISSN: 18170153 Year: 2018 Volume: 14 Issue: 25 Pages: 88-102
Publisher: Al-Qadisiyah University جامعة القادسية

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Abstract

Zinc has a role in normal growth and development during pregnancy, childhood, and is needed for the proper sense of taste and smell. Studies on animals, adolescents and low birth weight neonates showed that oral zinc salt intake decreased serum bilirubin level, probably through inhibition of the bilirubin enterohepatic circulation.Objective :To study the effect of serum zinc level of the newborn and their mothers on neonatal jaundice.Patient and methodsA case control study included a total 130, 65 with jaundice and 65 healthy neonates with their mothers. The study was carried out from May to October 2016 at ALhussein hospital for children in Al-Diwanyia city. For all neonates serum zinc, total serum bilirubin, hematocrit, blood group typing and Rhesus were carried out. Serum zinc for the mothers also was measured .Results :Serum zinc in jaundiced neonates was significantly lower than non jaundiced neonates (P<0.05). One hundred and nine mothers out of 130 mothers have low serum zinc. In multipara, zinc deficiency was more frequent in the mothers of both groups and P value (0.69).There was no significant difference was in mean serum zinc level when gestational age was taken into consideration (full term versus pre-term) (P>0.05).Conclusions :This study showed that serum concentration of zinc in neonates with jaundice was significantly lower than that in healthy control.

يلعب الزنك دورًا في النمو والتطور الطبيعي أثناء الحمل والطفولة ، ويلزمه الشعور بالشعور بالذوق والشم. أظهرت الدراسات التي أجريت على الحيوانات والمراهقين وحديثي الولادة الذين يعانون من انخفاض الوزن عند الولادة أن تناول ملح الزنك عن طريق الفم قلل من مستوى البيليروبين في المصل ، ربما من خلال تثبيط الدورة الدموية المعوية للكبد.هدف :لدراسة تأثير مستوى الزنك في المصل من حديثي الولادة وأمهاتهم على اليرقان الوليدي.المريض والأساليبوشملت دراسة مراقبة الحالات ما مجموعه 130 ، 65 مع اليرقان و 65 الولدان الأصحاء مع أمهاتهم. أجريت الدراسة في الفترة من مايو إلى أكتوبر 2016 في مستشفى الحسين للأطفال في مدينة الديوانية. لجميع حديثي الولادة الزنك المصل ، نفذت مجموع البيليروبين في الدم ، الهيماتوكريت ، وكتابة فصيلة الدم وريسوس بها. كما تم قياس الزنك المصل للأمهات.النتائج :كان مصل الزنك عند الولدان المصابين باليرقان أقل بكثير من الولدان غير المصابين باليرقان (P <0.05). مائة وتسع أمهات من بين 130 أمهات لديهن نسبة منخفضة من الزنك في الدم. في النتوءات المتعددة ، كان نقص الزنك أكثر شيوعًا في أمهات المجموعتين وقيمة P (0.69). لم يكن هناك اختلاف كبير في متوسط ​​مستوى الزنك في المصل عند أخذ عمر الحمل في الاعتبار (المدى الكامل مقابل ما قبل الأجل) (P> 0.05).الاستنتاجات:أظهرت هذه الدراسة أن تركيز مصل الزنك في الولدان المصابين باليرقان كان أقل بكثير من تركيزه في التحكم الصحي.


Article
Cholestatic jaundice in a sample of Iraqi Infants(A hospital based study)

Authors: Lana A. Mohammad --- Bayan A. Al-Badri --- Sawsan I. Al-Azzawi سوسن العزاوي
Journal: Journal of the Faculty of Medicine مجلة كلية الطب ISSN: 00419419 Year: 2011 Volume: 53 Issue: 1 Pages: 6-10
Publisher: Baghdad University جامعة بغداد

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Background: Cholestatic Jaundice is a dilemma facing not only the primary care provider but also the specialist pediatricians in our country.Subjects & methods: Analysis of 50 cases of cholestatic jaundice aged 3 weeks- 18 months were carried out over 18 months in the Gastroenterology & Hepatology unit in the Children Welfare Teaching Hospital, Medical City, and Baghdad. Clinical, biochemical, radiological and histopathological results were recorded.Results: Fifty patients with Cholestatic Jaundice, 28 males &22 females, were evaluated. The main causes of Cholestatic Jaundice were Biliary Atresia in 22 cases (44%)(the mean age of presentation was 4.1 months ), and Neonatal Hepatitis Syndrome of different etiology in 17 cases (34%) (The mean age of presentation was 5.9 months).Galactosemia was the most common cause of metabolic liver disease and CMV was the commonest of the intrauterine viral infections. Two cases of choledocal cyst (4%) and no definite cause was found in 9 cases (18%)
Conclusion: Biliary atresia was the most common cause of cholestasis in this study of extra-hepatic type. Different stages of cirrhosis were found in almost all cases of biliary Atresia because of delayed presentation. Clay color stool, hepatomegaly, high alkaline phosphatase & high cholesterol level favor atresia cases. Ultrasonography & liver biopsy prove to be very valuable tools in the differentiation between Biliary Atresia & Neonatal Hepatitis Syndrome.Key Words: Cholestatic Jaundice, Infants, Biliary Atresia.


Article
The Role of Ultrasound and Magnetic Resonance Imaging in the Diagnosis of Obstructive Jaundice

Author: Safa Al-Obaidi
Journal: Iraqi Academic Scientific Journal المجلة العراقية للاختصاصات الطبية ISSN: 16088360 Year: 2007 Volume: 6 Issue: 1 Pages: 7-17
Publisher: The Iraqi Borad for Medical Specialization المجلس العراقي للاختصاصات الطبية

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ABSTRACT:BACKGROUND:Evaluation of jaundiced patients should include proper history and examination, laboratory investigation and imaging investigations (non invasive like US, CT and MRI or invasive like ERCP and PTC).AIM OF STUDY:The aim of this prospective study is to evaluate the role of US and MRI-MRCP in patients with obstructive jaundice in clinical practice.METHODS:This is a prospective study performed on 80 patients (42 female and 38 male) with an average age of 53 years presented with obstructive jaundice for whom abdominal ultrasound (US) and magnetic resonance imaging (MRI) and magnetic resonance cholangiopancreatography (MRCP) performed in the departments of radiology in Al-Kadhemiyyah teaching hospital and Specialized surgical hospital and Baghdad teaching hospital from October 2003 to October 2005. The final diagnosis was found by endoscopic retrograde cholangiopancreatography (ERCP) and or surgery and confirmed by histopathology.RESULTS:The most common cause of obstructive jaundice in our study was tumors (41.25%) followed by common bile duct stones (36.25%) then benign strictures (13.75%), hydatid cyst (6.25%) & finally choledochal cyst (2.5%). In this study, MRI-MRCP could differentiate surgical from medical jaundice in all cases, while US could differentiate surgical from medical jaundice in 91.25% of cases. MRI-MRCP correctly defines the level of obstruction in all cases (100%).While US correctly define the level of obstruction in only 86.2 % of the total cases. MRI-MRCP correctly suggests the most possible cause of obstruction in 96.25% of cases. While US correctly suggests the most possible cause in only 36.2 %.CONCLUSION:So that US, as a screening modality is useful to confirm or exclude biliary dilatation & to choose patients for MRCP examination. MRI-MRCP is a useful non-invasive and essential method in the preoperative evaluation of patients with obstructive jaundice. In addition MRI-MRCP was superior to US or ERCP in studying the extent & staging of malignant lesions

Keywords

Ultrasound --- MRI --- MRCP --- Obstructive jaundice.


Article
Estimation of some trace elements in serum of children with jaundice
تقدير العناصر النادرة فى الاطفال المصابين باليرقان

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The objective of this study was to measure the alterations in serum trace elements copper (Cu), chromium (Cr) and zinc (Zn) in children with jaundice and compare them with the results of healthy individuals by using AAS. Significant low serum levels of Zn and high level of serum Cu and chromium were observed in children with jaundice as compared with normal healthy controls.. The study showed that Cu, Zn and Cr concentrations in serum of jaundice were (175.6±47,5 μg /dL ,95±67μg /dL and 7.98±0.54 μg /dL)respectively. In healthy individuals these concentrations were 109.3±13.72 μg /dL,106.55±11.7 μg /dL and3.8 ±0.48 μg /dL) respectively.

موضوع هده الدراسة هو قياس التغيرات فىمستوى العناصر النادرة (الزنك,الكروم والنحاس )فىمصول الاطفال المصابين باليرقان و مقارنتهابالنتا ئج للاطفال الاصحاء باستخدام تقنية الامتصا ص الدرى حيث لوحظ انخفاض ملحوظ فى مستويات الزنك وارتفاع ملحوظ فى مستويات النحاس والكروم فى الاطفال المصابين باليرقان عند مقارنتها بالمصول لطبيعية حيث اوضحت ا الدراسة بان مستويات النحاس ,الزنك والكروم كانت (175.6±75.5 μg/dl) ,( 95± 67 μg/dl),(7.98±0.54 μg/dl) على التوالى فى مصول الاطفال المصابين باليرقان اما فىمصول الاطفال الاصحاءفقد كانت القيم (106.55±11.7μg/dl) (3.8±0.48 μg/dl), (109±13.72 μg/dl) على التوالى.


Article
Wilson’s Disease in Children (Clinical Presentations & Diagnostic Difficulties) (Three years experience in Children Welfare Teaching Hospital- Baghdad

Authors: Sawsan I.I. Al-Azzawi --- Dheia Hassan Al-Baldawi --- Auday Yasin Abbas
Journal: Iraqi Academic Scientific Journal المجلة العراقية للاختصاصات الطبية ISSN: 16088360 Year: 2012 Volume: 11 Issue: 4 Pages: 437-442
Publisher: The Iraqi Borad for Medical Specialization المجلس العراقي للاختصاصات الطبية

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Abstract

ABSTRACT:BACKGROUND:Wilson’s disease (WD) is rare but curable hereditary metabolic disease presents in childhood in different ways which make the diagnosis difficult and delayed.OBJECTIVE:To evaluate all cases of WD, modes of presentations, available diagnostic methods, treatment and follow up in a sample of Iraqi children.PATIENTS AND METHODS:Clinical presentations and management of 24 patients with WD were studied after the exclusion of other chronic liver diseases by thorough investigation. Low ceruloplasmin level and high copper excretion in urine pre and post challenge with penicillamine, in addition to the presence of Kayser Fleischer rings were the main stay of the diagnosis. The diversity of the results with the response to treatment and side effects of drugs used were registered over 3 years period in the GIT & Hepatology unit and consultation clinic at the Children Welfare Teaching Hospital, Medical city, Baghdad in the period from the 1st of May 2003 till the 1st of may 2006.RESULTS:From the twenty four patients with WD included in the study, we had 17 patients (70.8%) with hepatic manifestations (4 with acute hepatitis, 3 with fulminant hepatic failure and 10 as chronic liver diseases). Only two patients (8.3%) had neurological symptoms. One patient had hepatic manifestation + evidence of hemolysis (4.1%) and 4 (16.7%) asymptomatic siblings. The mean age of presentation was less in hepatic than the neurologic presentation (7.5 and 9.2 years respectively). The time interval between the symptoms and the diagnosis was more in the neurological than hepatic cases (95 vs. 42 days). Low ceruloplasmin found in 70.1% & Kayser-Fleischer ring present in 47.1% of hepatic cases and in all neurological cases. Family history was positive in 3 patients of the index cases. On follow up after treatment one child had bone marrow depression and two patients had thrombocytopenia. One patient died (4.2%) with fulminant hepatic failure, ascites and encephalopathy.CONCLUSION:Acute hepatitis, chronic liver affection, fulminant hepatic encephalopathy acute hemolytic anemia may be the presentation of Wilson disease in children. They also may exhibit a neurological manifestation as tremor and ataxia. At least two of the three diagnostic criterion plus index of suspicion, family history of affected sibling or death in the family of a jaundiced child raises the possibility of WD after exclusion of other chronic liver diseases by investigations.

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