Immune status in patients With inherited bone marrow failure syndromes / (Record no. 80270)
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| 000 -LEADER | |
|---|---|
| fixed length control field | 03607cam a2200349 a 4500 |
| 003 - CONTROL NUMBER IDENTIFIER | |
| control field | EG-GiCUC |
| 005 - DATE AND TIME OF LATEST TRANSACTION | |
| control field | 20250223032717.0 |
| 008 - FIXED-LENGTH DATA ELEMENTS--GENERAL INFORMATION | |
| fixed length control field | 210318s2020 ua dh f m 000 0 eng d |
| 040 ## - CATALOGING SOURCE | |
| Original cataloging agency | EG-GiCUC |
| Language of cataloging | eng |
| Transcribing agency | EG-GiCUC |
| 041 0# - LANGUAGE CODE | |
| Language code of text/sound track or separate title | eng |
| 049 ## - LOCAL HOLDINGS (OCLC) | |
| Holding library | Deposite |
| 097 ## - Thesis Degree | |
| Thesis Level | M.Sc |
| 099 ## - LOCAL FREE-TEXT CALL NUMBER (OCLC) | |
| Classification number | Cai01.11.28.M.Sc.2020.Ba.I |
| 100 0# - MAIN ENTRY--PERSONAL NAME | |
| Personal name | Bavly Barsoum Mikhael Ghattas |
| 245 10 - TITLE STATEMENT | |
| Title | Immune status in patients With inherited bone marrow failure syndromes / |
| Statement of responsibility, etc. | Bavly Barsoum Mikhael Ghattas ; Supervised Nermeen Mouftah Galal , Marwa Abdelhady Abdelsamad , Sohilla Lotfy Mohamed Abdelkader |
| 246 15 - VARYING FORM OF TITLE | |
| Title proper/short title | تقييم الحالة المناعية لمرضى متلازمة فشل النخاع الوراثى |
| 260 ## - PUBLICATION, DISTRIBUTION, ETC. | |
| Place of publication, distribution, etc. | Cairo : |
| Name of publisher, distributor, etc. | Bavly Barsoum Mikhael Ghattas , |
| Date of publication, distribution, etc. | 2020 |
| 300 ## - PHYSICAL DESCRIPTION | |
| Extent | 199 P . : |
| Other physical details | charts , facsmilies ; |
| Dimensions | 25cm |
| 502 ## - DISSERTATION NOTE | |
| Dissertation note | Thesis (M.Sc.) - Cairo University - Faculty of Medicine - Department of Pediatrics |
| 520 ## - SUMMARY, ETC. | |
| Summary, etc. | Background: Inherited bone marrow failure syndromes (IBMFS) comprise a heterogeneous group of rare cancer prone genetic disorders with hematologic and physical abnormalities. The major IBMFS are Fanconi anemia (FA), dyskeratosis congenita (DC), Diamond{u2013}Blackfan anemia (DBA), Shwachman{u2013}Diamond syndrome (SDS) and severe congenital neutropenia (SCN). The immune status of patients with an IBMFS is not entirely clear; however there are some suggestions that a few IBMFS patients exhibit various degrees of qualitative immune dysfunction. Recently, there are novel and rare PID syndromes have been associated with cytopenia secondary to bone marrow failure, but they are not always taken into consideration, some patients may be asymptomatic and may lack a history of severe infections. Phenotypic overlap may impair the correct diagnosis. Objective: This cross sectional analytical study aimed at evaluation of immune status of patients with inherited bone marrow failure syndromes. Methodology: The study was conducted on twenty one patients diagnosed with IBMFS, following up at hematology clinic at Cairo University Children{u2019}s hospital. The study included 17 patients diagnosed with Fanconi anemia and 4 patients diagnosed with Diamond-Blackfan anemia. Those patients were subjected to thorough history taking and clinical examination looking for special somatic features specific for IBMFS. Laboratory workup done in our study to evaluate immune status of IBMFS patients included; complete blood count (CBC) with differential leucocytic count, immunophenotyping of lymphocytes by Flow cytometry (CD3, CD4, CD8, CD19, CD56, B cell maturation, and naïve/memory T cells), as well as immunoglobulins assay (IgG, IgM, IgA levels). Results: Sino-pulmonary infections were the most frequently reported infections among our patients (52%), followed by recurrent oral moniliasis (19%) and gastrointestinal infections in (14%) of patients. Neutropenia was detected in 74% of (N= 15/21) with severity ranging from mild (24%) to severe neutropenia (36%), and all were FA patients. Lymphopenia could be detected in 57% of the patients (N=12/21). Low immunoglobulin levels for age were detected in 28.6% of the patients |
| 530 ## - ADDITIONAL PHYSICAL FORM AVAILABLE NOTE | |
| Additional physical form available note | Issued also as CD |
| 653 #4 - INDEX TERM--UNCONTROLLED | |
| Uncontrolled term | IBMFS |
| 653 #4 - INDEX TERM--UNCONTROLLED | |
| Uncontrolled term | Immune status |
| 653 #4 - INDEX TERM--UNCONTROLLED | |
| Uncontrolled term | Inherited bone marrow failure syndromes |
| 700 0# - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Marwa Abdelhady Abdelsamad , |
| Relator term | |
| 700 0# - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Nermeen Mouftah Galal , |
| Relator term | |
| 700 0# - ADDED ENTRY--PERSONAL NAME | |
| Personal name | Sohilla Lotfy Mohamed Abdelkader , |
| Relator term | |
| 856 ## - ELECTRONIC LOCATION AND ACCESS | |
| Uniform Resource Identifier | <a href="http://172.23.153.220/th.pdf">http://172.23.153.220/th.pdf</a> |
| 905 ## - LOCAL DATA ELEMENT E, LDE (RLIN) | |
| Cataloger | Amira |
| Reviser | Cataloger |
| 905 ## - LOCAL DATA ELEMENT E, LDE (RLIN) | |
| Cataloger | Nazla |
| Reviser | Revisor |
| 942 ## - ADDED ENTRY ELEMENTS (KOHA) | |
| Source of classification or shelving scheme | Dewey Decimal Classification |
| Koha item type | Thesis |
| Source of classification or shelving scheme | Not for loan | Home library | Current library | Date acquired | Full call number | Barcode | Date last seen | Koha item type | Copy number |
|---|---|---|---|---|---|---|---|---|---|
| Dewey Decimal Classification | المكتبة المركزبة الجديدة - جامعة القاهرة | قاعة الرسائل الجامعية - الدور الاول | 11.02.2024 | Cai01.11.28.M.Sc.2020.Ba.I | 01010110082959000 | 22.09.2023 | Thesis | ||
| Dewey Decimal Classification | المكتبة المركزبة الجديدة - جامعة القاهرة | مخـــزن الرســائل الجـــامعية - البدروم | 11.02.2024 | Cai01.11.28.M.Sc.2020.Ba.I | 01020110082959000 | 22.09.2023 | CD - Rom | 82959.CD |