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Article ; Online: Acquired factor VII deficiency in pediatric inflammatory bowel disease: Report of three cases.

Çakar, Sevim / Eren, Gülin / Karapınar, Tuba Hilkay / Ecevıt, Çiğdem Ömür / Bekem, Özlem

Arab journal of gastroenterology : the official publication of the Pan-Arab Association of Gastroenterology

2023 Volume 24, Issue 4, Page(s) 256–258

Abstract: Bleeding disorders can exacerbate gastrointestinal bleeding in inflammatory bowel disease (IBD) at the time of diagnosis or flares. Factor VII (FVII) deficiency is a life-threatening rare congenital bleeding disorder in childhood. This study describes ... ...

Abstract	Bleeding disorders can exacerbate gastrointestinal bleeding in inflammatory bowel disease (IBD) at the time of diagnosis or flares. Factor VII (FVII) deficiency is a life-threatening rare congenital bleeding disorder in childhood. This study describes three adolescent patients with IBD accompanied by acquired FVII deficiency. This is the first case series of patients with IBD accompanied by FVII deficiency. We hypothesized that inflammation, accelerated consumption, disease severity, and weight loss can cause decreased FVII activity in patients diagnosed with IBD. To control intestinal bleeding, we must keep in mind factor deficiencies in IBD.
MeSH term(s)	Adolescent ; Humans ; Child ; Factor VII Deficiency/complications ; Factor VII Deficiency/diagnosis ; Factor VII Deficiency/congenital ; Factor VIIa ; Gastrointestinal Hemorrhage/etiology ; Patient Acuity ; Inflammatory Bowel Diseases/complications
Chemical Substances	Factor VIIa (EC 3.4.21.21)
Language	English
Publishing date	2023-11-22
Publishing country	Egypt
Document type	Case Reports
ZDB-ID	2502114-X
ISSN	2090-2387 ; 1687-1979
ISSN (online)	2090-2387
ISSN	1687-1979
DOI	10.1016/j.ajg.2023.10.002
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Article: A Neonatal Case of Infantile Malignant Osteopetrosis Presenting with Thrombocytopenia and Hypotonicity: A Novel Mutation in Chloride Voltage-Gated Channel 7 Gene.

Odaman Al, Isik / Oymak, Yesim / Hazan, Filiz / Gursoy, Semra / Ozturk, Tulay / Bag, Ozlem / Gozmen, Salih / Karakaya, Nurgul / Karapinar, Tuba Hilkay

Sisli Etfal Hastanesi tip bulteni

2022 Volume 56, Issue 1, Page(s) 161–165

Abstract: Autosomal recessive osteopetrosis is also known as infantile malignant osteopetrosis (IMO). The clinical course is often serious and if left untreated, it is fatal in the 1st year of life. Diagnosis is challenging and often delayed or misdiagnosed. ... ...

Abstract	Autosomal recessive osteopetrosis is also known as infantile malignant osteopetrosis (IMO). The clinical course is often serious and if left untreated, it is fatal in the 1st year of life. Diagnosis is challenging and often delayed or misdiagnosed. Herein, we present an infant girl who was diagnosed with IMO during evaluations for her hypotonicity and thrombocytopenia. A novel mutation of the chloride voltage-gated channel 7 (CLCN7) gene was also reported. A 10-day-old female patient was referred to our hospital for evaluation of hypotonicity. Her physical examination was normal, other than hypotonicity. Laboratory analysis revealed thrombocytopenia and hypocalcemia. In the progress, while she was followed in outpatient clinic, hepatosplenomegaly was detected at the age of 3 months. IMO was suspected with the findings of hepatosplenomegaly, cytopenia, hypocalcemia, difficulty of obtaining bone marrow, peripheral smear findings, and hearing loss. The X-ray of the bones was consistent with IMO. A novel pathogenic homozygous c.1504>T (p.Arg502Trp) mutation in CLCN7 gene was revealed. IMO is a rare disorder and it is important to differentiate this entity for better clinical outcome. The presence of neurological and hematological findings, organomegaly, hearing loss, and vision disorders must attract attention to IMO.
Language	English
Publishing date	2022-03-28
Publishing country	Turkey
Document type	Case Reports
ZDB-ID	3026862-X
ISSN	1308-5123 ; 1302-7123
ISSN (online)	1308-5123
ISSN	1302-7123
DOI	10.14744/SEMB.2021.88964
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Article: The evaluation of Candida-related central line-related bloodstream infections in pediatric cancer patients: A pre- and post-intervention study.

Devrim, İlker / Celebi, Miray Yılmaz / Karakaya, Nurgul / Sahinkaya, Sahika / Acar, Sultan Okur / Cem, Ela / Demirağ, Bengü / Oruc, Yeliz / Kara, Aybuke Akaslan / Oymak, Yesim / Karapınar, Tuba Hilkay / Bayram, Nuri

Journal of infection prevention

2023 Volume 24, Issue 5, Page(s) 219–222

Abstract: Aim/objections: The purpose of this study was to assess the efficacy of a central line maintenance bundle in preventing catheter-related (implanted venous access devices, port) Candida bloodstream infections.: Methods: The study encompassed two ... ...

Abstract	Aim/objections: The purpose of this study was to assess the efficacy of a central line maintenance bundle in preventing catheter-related (implanted venous access devices, port) Candida bloodstream infections. Methods: The study encompassed two distinct time periods, namely, pre-bundle and bundle. The number of catheter-related bloodstream infections (CRBSI) episodes per catheter days for each timeframe was determined. Findings/results: Upon implementation of the central line bundle, the rate of CRBSI reduced significantly from 4.27 per 1000 central line days in the pre-bundle period to 1.0 per 1000 central line days in the bundle period ( Discussion: Using a central line bundle to avoid CRBSIs in pediatric cancer patients with ports led to a significant decrease in Candida species-related CRBSIs.
Language	English
Publishing date	2023-08-26
Publishing country	England
Document type	Journal Article
ZDB-ID	2595000-9
ISSN	1757-1782 ; 1757-1774
ISSN (online)	1757-1782
ISSN	1757-1774
DOI	10.1177/17571774231191334
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Article ; Online: Successful Management of l-Asparaginase-Associated Pancreatitis With Octreotide and Pegylated Asparaginase in 2 Patients With Acute Lymphoblastic Leukemia: Is There a Different Rare Warning Sign of Hypoglycemia for l-Asparaginase-Associated Pancreatitis?

Aydin Köker, Sultan / Oymak, Yeşim / Demirağ, Bengü / Karapinar, Tuba Hilkay / Koker, Alper / Genç, Sinan / Erdemir, Gülin / Vergin, Raziye Canan

Clinical therapeutics

2020 Volume 42, Issue 4, Page(s) e82–e86

Abstract: l-Asparaginase (l-Asp) is a critical component of chemotherapy for acute lymphoblastic leukemia (ALL). However, toxic effects associated with l-Asp, such as hepatic dysfunction, pancreatitis, hypercholesterolemia, and hyperglycemia, have occurred. In ... ...

Abstract	l-Asparaginase (l-Asp) is a critical component of chemotherapy for acute lymphoblastic leukemia (ALL). However, toxic effects associated with l-Asp, such as hepatic dysfunction, pancreatitis, hypercholesterolemia, and hyperglycemia, have occurred. In addition, acute pancreatitis is a significant life-threatening adverse event associated with ALL. We describe 2 patients with ALL who had l-Asp-associated pancreatitis (AAP), with one patient presenting with hyperglycemia and the other presenting with hypoglycemia during induction treatment. When octreotide was administered to both of these patients, the clinical findings and laboratory data were improved. AAP was not repeated after treatment with pegylated asparaginase. Although AAP has a high risk of mortality and morbidity in childhood, APP treatment with appropriate agents, such as octreotide, can be successful.
MeSH term(s)	Acute Disease ; Adolescent ; Antineoplastic Agents/adverse effects ; Asparaginase/adverse effects ; Asparaginase/therapeutic use ; Child, Preschool ; Female ; Humans ; Hypoglycemia/chemically induced ; Male ; Octreotide/adverse effects ; Pancreatitis/chemically induced ; Pancreatitis/drug therapy ; Polyethylene Glycols/therapeutic use ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy
Chemical Substances	Antineoplastic Agents ; Polyethylene Glycols (3WJQ0SDW1A) ; Asparaginase (EC 3.5.1.1) ; Octreotide (RWM8CCW8GP)
Language	English
Publishing date	2020-03-14
Publishing country	United States
Document type	Case Reports ; Journal Article
ZDB-ID	603113-4
ISSN	1879-114X ; 0149-2918
ISSN (online)	1879-114X
ISSN	0149-2918
DOI	10.1016/j.clinthera.2020.02.015
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Zs.A 1435: Show issues			Location: Je nach Verfügbarkeit (siehe Angabe bei Bestand) bis Jg. 1994: Bestellungen von Artikeln über das Online-Bestellformular Jg. 1995 - 2021: Lesesall (1.OG) ab Jg. 2022: Lesesaal (EG)
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Article ; Online: Acrocallosal Syndrome First Presenting with Acute Lymphoblastic Leukemia: A Rare Case Report.

Koker, Sultan Aydin / Hazan, Filiz / Oymak, Yeşim / Soydan, Ekin / Karapınar, Tuba Hilkay / Ay, Yılmaz / Demirağ, Bengü / Vergin, Raziye Canan

Neurology India

2019 Volume 67, Issue 5, Page(s) 1386–1387

MeSH term(s)	Acrocallosal Syndrome/complications ; Child, Preschool ; Humans ; Male ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/complications
Language	English
Publishing date	2019-11-19
Publishing country	India
Document type	Case Reports ; Letter
ZDB-ID	415522-1
ISSN	1998-4022 ; 0028-3886
ISSN (online)	1998-4022
ISSN	0028-3886
DOI	10.4103/0028-3886.271244
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Zs.A 698: Show issues

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Article ; Online: Assessment of clinical characteristics and treatment outcomes of pediatric patients with intracardiac thrombosis: a single-center experience.

Odaman Al, Işik / Oymak, Yeşim / Erdem, Melek / Tahta, Neryal / Okur Acar, Sultan / Mese, Timur / Yilmazer, Murat Muhtar / Gözmen, Salih / Zihni, Cuneyt / Calkavur, Sebnem / Karapinar, Tuba Hilkay

Blood coagulation & fibrinolysis : an international journal in haemostasis and thrombosis

2021 Volume 33, Issue 1, Page(s) 34–41

Abstract: The prevalence of intracardiac thrombus (ICT) is gradually increasing, though it is rare among children. Data related to the occurrence of ICT among children are limited, and treatment recommendations have been made utilizing adult guidelines. The ... ...

Abstract	The prevalence of intracardiac thrombus (ICT) is gradually increasing, though it is rare among children. Data related to the occurrence of ICT among children are limited, and treatment recommendations have been made utilizing adult guidelines. The primary objective of this study is to determine associated factors, management, and outcomes of intracardiac thrombosis in children. Between January 2013 and January 2020, patients diagnosed with ICT at the Pediatric Hematology-Oncology and Pediatric Cardiology departments in our hospital were included in the study. Demographic characteristics, clinical and laboratory findings, treatment protocols, and outcomes were analyzed retrospectively. The median age at diagnosis was 10.5 months (2 days to 14.5 years), and the median follow-up period was 6.5 months (1 month to 3.1 years). The most common primary diagnoses of the patients, in order of frequency, were heart disease (n: 8), metabolic disease (n: 3), prematurity and RDS (n: 3), burns (n: 2), pneumonia (n: 2), and asphyxia (n: 2). CVC was present in 19/23 of the patients. The reasons for CVC insertion were the need for plasmapheresis in one patient with a diagnosis of HUS and the need for well tolerated vascular access because of long-term hospitalization in others. LMWH was administered to all patients as first-line therapy. Complete response was achieved in 19 (79%) of 24 patients and 4 patients (16.6%) were unresponsive to medical treatment. It was found out that the thrombus location, type, sepsis, and hemoculture positivity, as well as the presence of CVC, had no impact on treatment response (chi-square P = 0.16, 0.12, 0.3, 0.49, 0.56). Moreover, no correlation was determined between thrombus size and treatment response (Mann Whitney U test P = 0.47). The mortality rate was determined to be 12.5% (3/24). Spontaneous occurrence of ICT is rare in childhood, without any underlying primary disease or associated factor. The presence of CVC, sepsis, and heart disease are factors associated with ICT. The success rate is increased with medical treatment. There was no significant difference in treatment response between the newborn and 1 month to 18-year-old patient group. It has been demonstrated that thrombus size, type, localization; sepsis, and hemoculture positivity had no impact on the treatment response.
MeSH term(s)	Adult ; Child ; Heart Diseases ; Heparin, Low-Molecular-Weight ; Humans ; Infant, Newborn ; Retrospective Studies ; Thrombosis ; Treatment Outcome
Chemical Substances	Heparin, Low-Molecular-Weight
Language	English
Publishing date	2021-11-03
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	1033551-1
ISSN	1473-5733 ; 0957-5235
ISSN (online)	1473-5733
ISSN	0957-5235
DOI	10.1097/MBC.0000000000001100
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Zs.A 2920: Show issues

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Article ; Online: Effect of pyridoxine plus pyridostigmine treatment on vincristine-induced peripheral neuropathy in pediatric patients with acute lymphoblastic leukemia: a single-center experience.

Aydin Köker, Sultan / Gözmen, Salih / Demirağ, Bengü / Ünalp, Aycan / Karapinar, Tuba Hilkay / Oymak, Yeşim / Gürbüz, Gürkan / Öner, Ecem İpek / Vergin, Raziye Canan

Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology

2021 Volume 42, Issue 9, Page(s) 3681–3686

Abstract: Background: Vincristine (VCR), which is a key component of chemotherapy, is important for survival. VCR is associated with a well-known side effect, including neurotoxicity.: Aims: The aim of this study was to evaluate the features of vincristine- ... ...

Abstract	Background: Vincristine (VCR), which is a key component of chemotherapy, is important for survival. VCR is associated with a well-known side effect, including neurotoxicity. Aims: The aim of this study was to evaluate the features of vincristine-induced peripheral neuropathy (VIPN) and the effectiveness of pyridoxine plus pyridostigmine therapy in children with acute lymphoblastic leukemia. Methods: The WHO and NCI CTCAE neurotoxicity scorings were used to evaluate VIPN at diagnosis, in the first month, and after the third month of the treatment. The clinical features of 23 patients having acute lymphoblastic leukemia with VIPN during the period of July 2013-February 2016 were prospectively evaluated. Results: The mean age was 72.8 ± 51.6 months, and 26.1%, 56.5%, and 17.4% were in standard, moderate, and high-risk groups, respectively. Neuropathy frequently occurred at induction (82.6%) and reinduction (17.4%) of the protocol. Drop foot (82.6%), leg pain (82.6%), and difficulty in walking (82.6%) were observed. The mean total cumulative dose of neuropathy occurrence was 5.6 ± 2.03 mg/m Conclusion: The WHO and NCI CTCAE scorings may be used for evaluating neuropathy at diagnosis and follow-up of neurotoxicity with treatment. Pyridoxine plus pyridostigmine therapy may be an effective option in the treatment of VIPN.
MeSH term(s)	Antineoplastic Agents, Phytogenic ; Child ; Child, Preschool ; Humans ; Infant ; Peripheral Nervous System Diseases/chemically induced ; Peripheral Nervous System Diseases/drug therapy ; Precursor Cell Lymphoblastic Leukemia-Lymphoma/drug therapy ; Pyridostigmine Bromide/therapeutic use ; Pyridoxine/therapeutic use ; Vincristine/adverse effects
Chemical Substances	Antineoplastic Agents, Phytogenic ; Vincristine (5J49Q6B70F) ; Pyridoxine (KV2JZ1BI6Z) ; Pyridostigmine Bromide (KVI301NA53)
Language	English
Publishing date	2021-01-13
Publishing country	Italy
Document type	Journal Article
ZDB-ID	2016546-8
ISSN	1590-3478 ; 1590-1874
ISSN (online)	1590-3478
ISSN	1590-1874
DOI	10.1007/s10072-020-04970-w
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Zs.A 1877: Show issues

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Article ; Online: Evaluation of renal effects of liposomal amphotericin B in children with malignancies with KDIGO and RIFLE criteria.

Devrim, Fatma / Çağlar, İlknur / Acar, Sultan Okur / Akkuş, Şeyma / Dinçel, Nida / Yılmaz, Ebru / Tahta, Neryal / Demirağ, Bengü / Karapınar, Tuba Hilkay / Gözmen, Salih / Oymak, Yeşim / Vergin, Canan / Bayram, Nuri / Devrim, İlker

Nephrologie & therapeutique

2021 Volume 17, Issue 7, Page(s) 507–511

Abstract: Background: Amphotericin B is a broad-spectrum antifungal agent and is the backbone of the treatment for medically important opportunistic fungal pathogens in children. This study aimed to compare the nephrotoxicity associated with L-AmB in children ... ...

Abstract	Background: Amphotericin B is a broad-spectrum antifungal agent and is the backbone of the treatment for medically important opportunistic fungal pathogens in children. This study aimed to compare the nephrotoxicity associated with L-AmB in children with acute lymphoblastic leukemia and acute myeloid leukemia. Materials and methods: A total of 112 pediatric acute lymphoblastic leukemia or acute myeloid leukemia patients who received treatment with L-AmB (Ambisome®) at the University of Health Sciences Dr Behcet Uz Children's Hospital over 7 years were included. The incidence of hypokalemia, decreased estimated glomerular filtration rate and presence of acute kidney injury was recorded. Results: The average L-AmB treatment duration was 17.1±15.0 days. Five patients (4.4%) of the patients had grade I acute renal injury according to KDIGO criteria and 16 patients (14.2%) had increased risk for kidney injury according to RIFLE criteria. There were no patients with eGFR decrease above 50% and no renal injury and failure were observed during L-AmB treatment. The rate of patients with hypokalemia in the pre-treatment was 17.9% and the post-L-AmB group was 50.0%. The rate of hypokalemia was higher in the post-treatment group (P=0.0015). Among the 112 patients, only two patients (1.7%) required cessation of L-AmB treatment due to resistant hypokalemia despite supplementation. Conclusions: Hypokalemia was more common compared to glomerulotoxicity and acute renal injury (according to KDIGO and RIFLE criteria) in pediatric leukemia patients treated with L-AmB. Hypokalemia developed in nearly half of the patients and the study shows the need for randomized controlled trials and strategies for hypokalemia associated with L-AmB treatment.
MeSH term(s)	Acute Kidney Injury/chemically induced ; Acute Kidney Injury/epidemiology ; Amphotericin B/adverse effects ; Child ; Humans ; Kidney ; Neoplasms ; Retrospective Studies
Chemical Substances	liposomal amphotericin B ; Amphotericin B (7XU7A7DROE)
Language	English
Publishing date	2021-09-15
Publishing country	France
Document type	Journal Article
ZDB-ID	2229575-6
ISSN	1872-9177 ; 1769-7255
ISSN (online)	1872-9177
ISSN	1769-7255
DOI	10.1016/j.nephro.2021.06.007
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Zs.A 6298: Show issues

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Article: Assessment of sleep in pediatric cancer patients.

İnce, Dilek / Demirağ, Bengü / Karapınar, Tuba Hilkay / Oymak, Yeşim / Ay, Yılmaz / Kaygusuz, Arife / Töret, Ersin / Vergin, Canan

The Turkish journal of pediatrics

2018 Volume 59, Issue 4, Page(s) 379–386

Abstract: İnce D, Demirağ B, Karapınar TH, Oymak Y, Ay Y, Kaygusuz A, Töret E, Vergin C. Assessment of sleep in pediatric cancer patients. Turk J Pediatr 2017; 59: 379-386. The purpose of the study is to describe sleep habits, assess the prevalence of sleep ... ...

Abstract	İnce D, Demirağ B, Karapınar TH, Oymak Y, Ay Y, Kaygusuz A, Töret E, Vergin C. Assessment of sleep in pediatric cancer patients. Turk J Pediatr 2017; 59: 379-386. The purpose of the study is to describe sleep habits, assess the prevalence of sleep disturbances in pediatric cancer patients and healthy controls, and to compare sleep patterns, sleep problems. One hundred-thirty-five patients and 190 healthy controls were evaluated. Healthy children matched for age, sex, economic status, parental education and family structure constituted the control group. Sleep was evaluated by using the Children`s Sleep Habits Questionnaire (CSHQ). Sleep problems were detected in half of patients. There were no significant differences in total sleep score and subscale scores between patients and controls. Solely the wake-time was found significantly different between patients and controls. Although our results indicated that neither childhood cancer survivors nor patients with cancer during treatment period had more sleep problems than their healthy peers, sleep problems were not uncommon in whole study group. This study underlines the need to screen, assess and manage sleep problems in children with diagnosis of cancer.
MeSH term(s)	Cancer Survivors/statistics & numerical data ; Child ; Child, Preschool ; Female ; Humans ; Male ; Neoplasms/complications ; Parents ; Prevalence ; Sleep ; Sleep Wake Disorders/epidemiology ; Sleep Wake Disorders/etiology ; Surveys and Questionnaires
Language	English
Publishing date	2018-04-06
Publishing country	Turkey
Document type	Journal Article
ZDB-ID	123487-0
ISSN	0041-4301
ISSN	0041-4301
DOI	10.24953/turkjped.2017.04.003
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Zs.B 988: Show issues

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Article: Children with chronic-refractory autoimmune cytopenias: a single center experience.

Karapınar, Tuba Hilkay / Durgun, Ersin / Oymak, Yeşim / Gülez, Nesrin / Ay, Yılmaz / Genel, Ferah / Gözmen, Salih / Serdaroğlu, Erkin / Köker, Sultan Aydın / Töret, Ersin / Vergin, Canan

The Turkish journal of pediatrics

2020 Volume 62, Issue 4, Page(s) 525–532

Abstract: Background and objectives: Autoimmune cytopenias are a group of heterogeneous disorders characterized by immune-mediated destruction of one or more hematopoietic lineage cells. The differential diagnosis of children with autoimmune cytopenias requires ... ...

Abstract	Background and objectives: Autoimmune cytopenias are a group of heterogeneous disorders characterized by immune-mediated destruction of one or more hematopoietic lineage cells. The differential diagnosis of children with autoimmune cytopenias requires much time and laboratory investigations. The aim of the present study was to evaluate the clinical course and significance of autoimmune cytopenias due to immunodeficiency or autoimmune diseases in children at a single children`s hospital. Method: Between February 1997 and September 2015, chronic/refractory autoimmune cytopenias patient data were evaluated retrospectively. Twenty-three patients were assessed in this study. Results: The median duration of following was 2.6 years (4 months-18.5 years). The median age of diagnosis was 3.1 years (6 months-16 years). A total of 13 patients (56.5%) had single-lineage and 10 (46.5%) had multilineage cytopenias. The most frequent single-lineage cytopenia was thrombocytopenia, followed by anemia. In 22 of the patients, cytopenias was detected before the primary diseases. All of the patients were treated with corticosteroids or intravenous immune globulin as first-line treatment. Ten patients (43.5%) needed second or further-line immunosuppressive therapies that patients diagnosed as systemic lupus erythematosus, hypogammaglobulinemia, or common variable immunodeficiency. A total of 8 patients (34.7%) recovered from autoimmune cytopenias after the treatment of primer disease. Cytopenias were continued in 14 patients. Conclusion: Cytopenia may be the first finding of an immunodeficiency or autoimmune disease and primary disease may be diagnosed in the clinical course. Taking the new targeted treatment options into consideration; early diagnosis is likely to become more important in the near-future in order to begin the treatment for the underlying disease as early as possible.
MeSH term(s)	Anemia ; Child ; Child, Preschool ; Humans ; Immunosuppressive Agents ; Leukopenia ; Retrospective Studies ; Thrombocytopenia
Chemical Substances	Immunosuppressive Agents
Language	English
Publishing date	2020-08-10
Publishing country	Turkey
Document type	Journal Article
ZDB-ID	123487-0
ISSN	0041-4301
ISSN	0041-4301
DOI	10.24953/turkjped.2020.04.001
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