Subscribe to the gold package and get unlimited access to Shamra Academy
Register a new userA study of hyperimmunoglobulin E syndrome in Damascus University Children’s Hospital
دراسة متلازمة فرط الغلوبولين المناعي E عند الأطفال في مستشفى الأطفال الجامعي
2170
0 27
0
(
0
)
Created by
Shamra Editor
Ask ChatGPT about the research
تعد متلازمة فرط الغلوبولين المناعي E متلازمة جوب) من المتلازمات النادرة نسبياً و تتميز بحدوث التهابات متكررة في الجلد من دمامل و خراجات و طفح أكزيميائي، مترافقة مع أخماج تنفسية علوية راجعة من التهاب أذن وسطى، و التهاب جيوب و التهاب خشاء، و كذلك الأمر من أخماج تنفسية سفلية، مثل ذوات الرئة المتكررة التي كثيراً ما تختلط بتشكل القيلات الهوائية و الخراجات الرئوية التي يكون علاجها صعباً جداً. و من و تصنف إلى شكلين، الأول العلامات الواصمة للمتلازمة ترافقها مع ارتفاع شديد في مستوى الغلوبولين المناعي ذات الوراثة الجسدية السائدة، و الثاني وراثته جسدية متنحية. إلقاء الضوء على متلازمة جوب من ناحية التظاهرات السريرية و الموجودات المخبرية و مقارنتها بالأدب الطبي، دراسة الاختلاطات الجرثومية و العلاج بالمضادات الحيوية المختلقة. و نتائج تلك العلاجات، و رفع مستوى الشك السريري بالمتلازمة لدى الأطباء.
Hyperimmunoglobulin E (Job) syndrome is considered to be a relatively rare disease. Its characterized by recurrent infections in skin (eg. boils, abscesses), recurrent upper respiratory tract infections (eg. otitis media, sinusitis and mastoiditis), and lower respiratory infections (eg. recurrent pneumonia, which is often complicated by pneumatoceles and lung abscess), that are usually difficult to treat. The highly increased IgE levels are considered to be the hallmark of the disease. The syndrome has two types: one is inherited as an autosomal recessive, and the other, as an autosomal dominant trait. To highlight the clinical manifestations and laboratory findigs of Job disease, and to compare it with medical literature, to study the bacterial complications and differrent types of antibacterial treatments and treir results, and to increase the level of awareness among physicians.
References used
Rezai N. Primary Immunodeficiency Diseases, Definition, Diagnosis and Management. Tehran: Springer- Verlag Berlin Heidelberg; 2008
LaPine TR, Hill HR, Stiehm ER, TePas E. Hyperimmunoglobulin E syndrome. UpToDate. September 28, 2010. Available at: http://www.uptodate.com/contents/hyperimmunoglobulin-e-syndrome . Accessed April 17, 2011
Heimall J, Freeman A, Holland SM. Pathogenesis of hyper IgE syndrome. Clin Rev Allergy Immunol.. Feb 2010 ;38(1):32-38
rate research
Read More
Background& Objective: Selective IgA deficiency is the most common primary immunodeficiency in
humans. Its prevalence ranges from 1 in 100 to 1 in 1000 in among Arab populations. The majority of
patients with selective IgA deficiency are asymptomat
ic. The remaining present with recurrent upper and
lower respiratory tract infections, recurrent gastrointestinal infections, otitis media, sinusitis, celiac
disease, giardiasis, allergic disorders and some autoimmune diseases.
to highlight the clinical manifestations and laboratory findings of Selective IgA deficiency in our study,
and to compare it with medical literature. To study the bacterial complications and their prevalence.
Materials & Methods: A retrospective study was carried out in Damascus University Children’s Hospital
on sixteen child diagnosed as Selective IgA deficiency, in a period of the last ten years (from 2000 till
September 2010).
Results: most cases in our study were among males, failure to thrive was found in 75%,
hepatosplenomegaly in one third, the most prominent infectious complications were: recurrent pneumonia
(in 87.5% of cases), recurrent GI infections (in 75% of cases) and UTI’s in (50%)of cases. On the other
hand, the celiac disease was found in (25%) of our patients, which is much higher than described in
medical literature. In general, the infectious complications had a good response to antibacterial therapy,
and the IgA levels became normal in one patient, when he reached the age of eleven years.
Conclusion: there is no information in medical literature about the association of selective IgA deficiency
with hepatomegaly, splenomegaly, failure to thrive and an increased prevalence of urinary tract
infections, therefore, it is necessary to conduct researches about this association.
The hyper immuno-globuline E (IgE) syndrome with recurrent infections or
Job syndrome is a rare immunodeficiency characterized by recurrent skin
and pulmonary abscesses and extremely elevated levels of IgE in serum with
eczematous dermatitis. Asso
ciated facial and skeletal features have been
recognized but their frequency is unknown. The genetic basis of this
syndrome is poorly understood.
Visceral Leishmaniasis is still seen in developing country
To study the epidemiology of Visceral Leishmaniasis cases
hospitalized in Damascus University Children Hospital.
It was retro-spective study on (89) cases of Visceral
Leishmaniasis hospitalized between 1993 and 2005.
The aim of this study is to collect more information about
Congenital hypothyroidism (CH) in Syria due to it being one of the
most common causes of preventable mental retardation if detected
at early stage and treatment is preventable, and to the
absence of any
published statistics regarding it in our country, and to emphasize on
the necessity of applying a mandatory newborn screening program
for early diagnosis of congenital hypothyroidism to improvement of
child’s life, by investigating the adequacy of the clinical
determination the physician's experience to get an early diagnosis.
The empty sella syndrome (ESS) is an asymptomatic radiological syndrome,
affecting 6% of post-menopausal women and can be seen in all ages.
The results were similar with other studies in many respects, such as a wide
age spectrum including childre
n, and the occurrence of diabetes insipidus in
5.97%.
The results of our study were discordant with other studies with respect to
hyperprolactinemia which reached 58.2% in our study (in other studies did
not exceed 33.7%). No concurrent cases of secretory pituitary adenomas
were found (studies refer to an incidence of acromegaly and Cushing’s
syndrome in 2.6% of ESS for each). We did not record CSF rhinorrhea nor
were visual disturbances seen in our cohort. Patients received symptomatic
treatment and none were referred to surgery.
Log in to be able to interact and post comments
comments
Fetching comments
Sign in to be able to follow your search criteria
