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Article ; Online: The role of nailfold capillaroscopy in pediatric patients with Kawasaki disease.

Sedaghat, Banafsheh / Raeeskarami, Sayed-Reza / Tahghighi, Fatemeh / Assari, Raheleh / Aghaei-Moghadam, Ehsan / Razavi-Khorasani, Niloofar / Najafizadeh, Seyed-Reza / Ziaee, Vahid

Reumatologia clinica

2023 Volume 19, Issue 5, Page(s) 255–259

Abstract: Background: Nailfold capillaroscopy has been used as a non-invasive diagnostic method for microvasculature evaluation in various rheumatological disorders. The present study aimed to determine the utility of nailfold capillaroscopy in the diagnosis of ... ...

Abstract	Background: Nailfold capillaroscopy has been used as a non-invasive diagnostic method for microvasculature evaluation in various rheumatological disorders. The present study aimed to determine the utility of nailfold capillaroscopy in the diagnosis of Kawasaki Disease (KD). Method: In this case-control study nailfold capillaroscopy was performed in 31 patients with KD and 30 healthy controls. All nailfold images were evaluated for capillary distribution and capillary morphology such as enlargement, tortuosity, and dilatation of the capillaries. Result: Abnormal capillaroscopic diameter was identified in 21 patients from the KD group and 4 patients in the control group. The most common abnormality in capillary diameter was irregular dilatation in 11 (35.4%) KD patients and in 4 people (13.3%) in the control group. Distortions of the normal capillary architecture was commonly seen in the KD group (n=8). A positive correlation was observed between coronary involvement and abnormal capillaroscopic results (r=.65, P<.03). The sensitivity and specificity of capillaroscopy for the diagnosis of KD were 84.0% (95%CI: 63.9-95.5%) and 72.2% (95%CI: 54.8-85.8%), respectively. The PPV and NPV of capillaroscopy for KD were 67.7% (95%CI: 48.6-83.3) and 86.7% (95% CI: 69.3-96.2), respectively. Conclusion: Capillary alterations are more common in KD patients compared to control group. Thus, nailfold capillaroscopy can be useful in detecting these alterations. Capillaroscopy is a sensitive test for detecting capillary alternations in KD patients. It could be used as a feasible diagnostic modality for evaluating microvascular damage in KD.
MeSH term(s)	Humans ; Child ; Microscopic Angioscopy/methods ; Case-Control Studies ; Mucocutaneous Lymph Node Syndrome/diagnostic imaging ; Nails/diagnostic imaging ; Nails/blood supply ; Sensitivity and Specificity
Language	English
Publishing date	2023-04-18
Publishing country	Spain
Document type	Journal Article
ISSN	2173-5743
ISSN (online)	2173-5743
DOI	10.1016/j.reumae.2022.08.004
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Article: Intravenous Methylprednisolone Pulse Therapy Versus Intravenous Immunoglobulin in the Prevention of Coronary Artery Disease in Children with Kawasaki Disease: A Randomized Controlled Trial.

Aslani, Nahid / Raeeskarami, Seyed-Reza / Aghaei-Moghadam, Ehsan / Tahghighi, Fatemeh / Assari, Raheleh / Sadeghi, Payman / Ziaee, Vahid

Cureus

2022 Volume 14, Issue 6, Page(s) e26252

Abstract: Background: Kawasaki disease (KD) is often complicated by coronary artery lesion (CAL), including dilatation or aneurysms. Intravenous immunoglobulin (IVIG) is used with aspirin to prevent CAL in KD.: Objective: Given that the primary treatment for ... ...

Abstract	Background: Kawasaki disease (KD) is often complicated by coronary artery lesion (CAL), including dilatation or aneurysms. Intravenous immunoglobulin (IVIG) is used with aspirin to prevent CAL in KD. Objective: Given that the primary treatment for other vasculitis is the use of corticosteroids, this study has been performed to evaluate the effect of intravenous methylprednisolone pulse (IVMP) therapy in preventing CAL in KD. Method: A randomized, single-blind clinical trial was conducted on 40 KD patients aged six months to five years. Patients were randomized into two groups according to the main treatment plan in addition to aspirin: case group (IVMP for three consecutive days and then oral prednisolone for three days) and control group (intravenous immunoglobulin 2 g/kg). Echocardiography was performed for all children at least three times, during the acute phase, two weeks, and two months later. Results: Data analysis at the end of the study was done on 40 patients (20 patients in each group). There were no significant differences in age and sex distribution, mean fever, and acute phase duration, as well as baseline echocardiography in the two groups. The frequency of CAL was 20% in the case group and 45% in the control group, after two weeks (p<0.05), but there was no significant difference between two groups in types of coronary artery lesion after two weeks and the frequency and severity of CAL after two months. Conclusion: IVMP as initial line therapy effectively control systemic and vascular inflammation and decrease coronary artery damage in KD.
Language	English
Publishing date	2022-06-23
Publishing country	United States
Document type	Journal Article
ZDB-ID	2747273-5
ISSN	2168-8184
ISSN	2168-8184
DOI	10.7759/cureus.26252
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Article ; Online: A case series of ten plus one deficiency of adenosine deaminase 2 (DADA2) patients in Iran.

Asna Ashari, Kosar / Aslani, Nahid / Parvaneh, Nima / Assari, Raheleh / Heidari, Morteza / Fathi, Mohammadreza / Tahghighi Sharabian, Fatemeh / Ronagh, Alireza / Shahrooei, Mohammad / Moafi, Alireza / Rezaei, Nima / Ziaee, Vahid

Pediatric rheumatology online journal

2023 Volume 21, Issue 1, Page(s) 55

Abstract: Background: Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive autoinflammatory disease caused by mutations in the ADA2 gene. DADA2 has a broad spectrum of clinical presentations. Apart from systemic manifestations, we can categorize ... ...

Abstract	Background: Deficiency of adenosine deaminase 2 (DADA2) is an autosomal recessive autoinflammatory disease caused by mutations in the ADA2 gene. DADA2 has a broad spectrum of clinical presentations. Apart from systemic manifestations, we can categorize most of the signs and symptoms of DADA2 into the three groups of vasculitis, hematologic abnormalities, and immunologic dysregulations. The most dominant vasculitis features are skin manifestations, mostly in the form of livedo racemosa/reticularis, and early onset ischemic or hemorrhagic strokes. Hypogammaglobulinemia that is found in many cases of DADA2 brings immunodeficiencies into the differential diagnosis. Cytopenia, pure red cell aplasia (PRCA), and bone marrow failure (BMF) are the hematologic abnormalities commonly found in DADA. Case presentation: We introduce eleven patients with DADA2 diagnosis, including two brothers and sisters, one set of twin sisters, and one father and his daughter and son. Ten patients (91%) had consanguineous parents. All the patients manifested livedo racemose/reticularis. Ten patients (91%) reported febrile episodes, and seven (64%) had experienced strokes. Only one patient had hypertension. Two of the patients (11%) presented decreased immunoglobulin levels. One of the patients presented with PRCA. Except for the PRCA patient with G321E mutation, all of our patients delivered G47R mutation, the most common mutation in DADA2 patients. Except for one patient who unfortunately passed away before the diagnosis was made and proper treatment was initiated, the other patients' symptoms are currently controlled; two of the patients presented with mild symptoms and are now being treated with colchicine, and the eight others responded well to anti-TNFs. The PRCA patient still suffers from hematologic abnormalities and is a candidate for a bone marrow transplant. Conclusions: Considering the manifestations and the differential diagnoses, DADA2 is not merely a rheumatologic disease, and introducing this disease to hematologists, neurologists, and immunologists is mandatory to initiate prompt and proper treatment. The efficacy of anti-TNFs in resolving the symptoms of DADA2 patients have been proven, but not for those with hematologic manifestations. Similarly, they were effective in controlling the symptoms of our cohort of patients, except for the one patient with cytopenia.
MeSH term(s)	Male ; Humans ; Adenosine Deaminase/genetics ; Iran ; Intercellular Signaling Peptides and Proteins ; Research
Chemical Substances	Adenosine Deaminase (EC 3.5.4.4) ; Intercellular Signaling Peptides and Proteins
Language	English
Publishing date	2023-06-13
Publishing country	England
Document type	Case Reports ; Journal Article
ZDB-ID	2279468-2
ISSN	1546-0096 ; 1546-0096
ISSN (online)	1546-0096
ISSN	1546-0096
DOI	10.1186/s12969-023-00838-3
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Article: The Comparison of Nailfold Capillaroscopy between Juvenile Systemic Lupus Erythematosus and Healthy Controls: Correlation with Laboratory and Clinical Parameters.

Raeeskarami, Seyed-Reza / Namazi, Navid / Assari, Raheleh / Najafizadeh, Seyed-Reza / Hassannejad, Zohreh / Ziaee, Vahid

International journal of vascular medicine

2020 Volume 2020, Page(s) 7631958

Abstract: Background: Nailfold capillaroscopy is a noninvasive technique to recognize peripheral microangiopathy, which is an important feature in systemic lupus erythematosus (SLE). The aims of the present study were to investigate the prevalence of nailfold ... ...

Abstract	Background: Nailfold capillaroscopy is a noninvasive technique to recognize peripheral microangiopathy, which is an important feature in systemic lupus erythematosus (SLE). The aims of the present study were to investigate the prevalence of nailfold capillaroscopy (NFC) changes in juvenile systemic lupus erythematosus (JSLE), find out patterns of these changes, and correlate findings with clinical and laboratory parameters. Methods: Forty-nine patients with SLE, all meeting the 1997 revised ACR criteria for SLE classification, and 30 healthy controls were included. A digital video camera was used to capture nailfold capillary images. Computerized image processing was used for analysis. Results: Different abnormal NFC changes were observed with abnormal morphology, the increased diameter and abnormal loop densities in 55.1%, 93.9%, and 26.5% of the patients, respectively. No statistically significant differences were depicted between capillaroscopy with age, gender, autoantibodies (APLs, anti-ds DNA), antiphospholipid antibody syndrome, thrombotic angiopathy, renal function tests (Bun, Cr), and abnormal urine analysis. However, a significant correlation was found between the branched pattern and the CNS involvement group ( Conclusions: Different abnormal NFC changes are quite common among patients with SLE, and nailfold capillaroscopy is an effective method to monitor such changes. Treatment strategies may change in the branched pattern of nailfold capillaroscopy due to CNS involvement.
Language	English
Publishing date	2020-04-27
Publishing country	United States
Document type	Journal Article
ZDB-ID	2573977-3
ISSN	2090-2832 ; 2090-2824
ISSN (online)	2090-2832
ISSN	2090-2824
DOI	10.1155/2020/7631958
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Article ; Online: Cherubism: report of three cases and literature review.

Mirmohammadsadeghi, Arash / Eshraghi, Bahram / Shahsanaei, Azadeh / Assari, Raheleh

Orbit (Amsterdam, Netherlands)

2015 Volume 34, Issue 1, Page(s) 33–37

Abstract: Purpose: To report 3 cases of cherubism, one of whom underwent surgery for orbital manifestations, and to provide a literature review.: Case reports: Our patients were normal at birth and developed painless enlarging of the cheeks and jaws when they ... ...

Abstract	Purpose: To report 3 cases of cherubism, one of whom underwent surgery for orbital manifestations, and to provide a literature review. Case reports: Our patients were normal at birth and developed painless enlarging of the cheeks and jaws when they were 4-5 years old. Ophthalmologic examinations showed mild proptosis, superior globe displacement and inferior scleral show in all cases. Cases 2 and 3 had lower lid skin discoloration. Computed tomography (CT) scans demonstrated bilateral multicystic lesions in the maxilla and mandible with cortical thinning in all cases. In Case 3, left eye hyperglobus and anisometropic amblyopia was seen. In this case, the CT scan showed a round, well-defined and homogeneous mass, involving the anterior and superior walls of the maxillary sinus on the left side, extending into inferior orbit. Debulking of the mass was performed at the surgery. The pathologic findings were compatible with the diagnosis of giant cell reparative granuloma. He returned 1 year after surgery with recurrence of the mass. Discussion: A few cases were reported in the literature with histopathologically proven orbital cherubism. To our knowledge, lower lid skin discoloration in Cases 2 and 3 and anisometropic amblyopia in case 3 were not described elsewhere in cherubism cases. We recommend that all cases with cherubism must be examined by an ophthalmologist to diagnose and treat possible orbital manifestations.
MeSH term(s)	Adult ; Cherubism/diagnosis ; Diagnosis, Differential ; Female ; Humans ; Male ; Orbital Diseases/diagnosis ; Tomography, X-Ray Computed ; Visual Acuity
Language	English
Publishing date	2015-02
Publishing country	England
Document type	Case Reports ; Journal Article ; Review
ZDB-ID	603157-2
ISSN	1744-5108 ; 0167-6830
ISSN (online)	1744-5108
ISSN	0167-6830
DOI	10.3109/01676830.2014.950287
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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: Dynamic Changes, Cut-Off Points, Sensitivity, and Specificity of Laboratory Data to Differentiate Macrophage Activation Syndrome from Active Disease

Raheleh Assari / Vahid Ziaee / Arash Mirmohammadsadeghi / Mohammad-Hassan Moradinejad

Disease Markers, Vol

2015 Volume 2015

Keywords	Medicine ; R ; Medicine (General) ; R5-920
Publishing date	2015-01-01T00:00:00Z
Publisher	Hindawi Publishing Corporation
Document type	Article ; Online
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Article: Pseudotumor Cerebri as the First Manifestation of Juvenile Systemic Lupus Erythematosus.

Raeeskarami, Seyed-Reza / Shahbaznejad, Leila / Assari, Raheleh / Aghighi, Yahya

Iranian journal of pediatrics

2016 Volume 26, Issue 5, Page(s) e5176

Abstract: Introduction: Headache is a common neuropsychiatric manifestation of juvenile systemic lupus erythematous (JSLE). Pseudotumor cerebri (PTC) is an uncommon cause of headache, presenting especially in active JSLE. In this paper, we report a case of missed ...

Abstract	Introduction: Headache is a common neuropsychiatric manifestation of juvenile systemic lupus erythematous (JSLE). Pseudotumor cerebri (PTC) is an uncommon cause of headache, presenting especially in active JSLE. In this paper, we report a case of missed intractable headache that was eventually diagnosed as PTC and presented as the first manifestation of JSLE. Case presentation: A 9-year-old girl with a history of progressive headache for four months, fever, fatigue, myalgia, arthralgia, small-joint arthritis of the hands, and recent diplopia was referred to our clinic. The diagnosis of PTC in the background of JSLE was made based on her history, physical examination, positive laboratory findings, and increased intracranial pressure, with normal neuroimaging. Treatment with high-dose prednisolone led to dramatic resolution of the headache. Conclusions: It seems that a complete neurologic examination in newly diagnosed SLE patients is mandatory, especially in the presence of any neuropsychiatric manifestations, such as headache.
Language	English
Publishing date	2016-07-12
Publishing country	Iran
Document type	Case Reports
ZDB-ID	2388780-1
ISSN	2008-2150 ; 2008-2142
ISSN (online)	2008-2150
ISSN	2008-2142
DOI	10.5812/ijp.5176
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Article ; Online: Periodic Fever and Neutrophilic Dermatosis

Raheleh Assari / Vahid Ziaee / Nima Parvaneh / Mohammad-Hassan Moradinejad

Case Reports in Immunology, Vol

Is It Sweet’s Syndrome?

2014 Volume 2014

Keywords	Immunologic diseases. Allergy ; RC581-607 ; Specialties of internal medicine ; RC581-951 ; Internal medicine ; RC31-1245 ; Medicine ; R
Language	English
Publishing date	2014-01-01T00:00:00Z
Publisher	Hindawi Publishing Corporation
Document type	Article ; Online
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Article ; Online: Dynamic Changes, Cut-Off Points, Sensitivity, and Specificity of Laboratory Data to Differentiate Macrophage Activation Syndrome from Active Disease.

Assari, Raheleh / Ziaee, Vahid / Mirmohammadsadeghi, Arash / Moradinejad, Mohammad-Hassan

Disease markers

2015 Volume 2015, Page(s) 424381

Abstract: Purpose: To compare the laboratory data and changes in these data between patients with MAS and patients with flare-up of the autoimmune diseases.: Methods: In a prospective study, the static laboratory data and dynamic changes in the selected data ... ...

Abstract	Purpose: To compare the laboratory data and changes in these data between patients with MAS and patients with flare-up of the autoimmune diseases. Methods: In a prospective study, the static laboratory data and dynamic changes in the selected data in 17 consecutive patients with MAS and 53 patients with active disease of SJIA, PJIA, Kawasaki disease, and SLE were compared. The ROC curve analysis was used to evaluate cut-off points, sensitivity, and specificity of the static and dynamic laboratory data to differentiate between MAS and active disease. Results: In the MAS group, the mean CRP3, ALT, AST, total bilirubin, ferritin, LDH, PT, PTT, and INR were significantly higher and the mean WBC2, PMN2, Lymph2, Hgb1, 2, 3, ESR2, serum albumin, and sodium were significantly lower than in control group. Some of the important cut-off points were PLT2 < 209000/microliter, AST > 38.5, ALT > 38, WBC < 8200 × 103/UL, ferritin > 5277 ng/mL. Conclusion: The dynamic changes in some laboratory data, especially PLT, can differentiate between MAS and active disease. The changes in WBC, PMN, and ESR and the levels of the liver enzymes may also be helpful in the early differentiation. Very high levels of ferritin may also help the diagnosis along with other clinical and laboratory signs.
MeSH term(s)	Adolescent ; Arthritis, Juvenile/blood ; Biomarkers/blood ; Blood Chemical Analysis/standards ; Case-Control Studies ; Child ; Child, Preschool ; Diagnosis, Differential ; Female ; Humans ; Infant ; Lupus Erythematosus, Systemic/blood ; Macrophage Activation Syndrome/blood ; Male ; Mucocutaneous Lymph Node Syndrome/blood ; Sensitivity and Specificity
Chemical Substances	Biomarkers
Language	English
Publishing date	2015-04-30
Publishing country	United States
Document type	Journal Article
ZDB-ID	604951-5
ISSN	1875-8630 ; 0278-0240
ISSN (online)	1875-8630
ISSN	0278-0240
DOI	10.1155/2015/424381
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Article ; Online: Dynamic Changes, Cut-Off Points, Sensitivity, and Specificity of Laboratory Data to Differentiate Macrophage Activation Syndrome from Active Disease

Raheleh Assari / Vahid Ziaee / Arash Mirmohammadsadeghi / Mohammad-Hassan Moradinejad

Disease Markers, Vol

2015 Volume 2015

Abstract: Purpose. To compare the laboratory data and changes in these data between patients with MAS and patients with flare-up of the autoimmune diseases. Methods. In a prospective study, the static laboratory data and dynamic changes in the selected data in 17 ... ...

Abstract	Purpose. To compare the laboratory data and changes in these data between patients with MAS and patients with flare-up of the autoimmune diseases. Methods. In a prospective study, the static laboratory data and dynamic changes in the selected data in 17 consecutive patients with MAS and 53 patients with active disease of SJIA, PJIA, Kawasaki disease, and SLE were compared. The ROC curve analysis was used to evaluate cut-off points, sensitivity, and specificity of the static and dynamic laboratory data to differentiate between MAS and active disease. Results. In the MAS group, the mean CRP3, ALT, AST, total bilirubin, ferritin, LDH, PT, PTT, and INR were significantly higher and the mean WBC2, PMN2, Lymph2, Hgb1, 2, 3, ESR2, serum albumin, and sodium were significantly lower than in control group. Some of the important cut-off points were PLT2 < 209000/microliter, AST > 38.5, ALT > 38, WBC < 8200 × 103/UL, ferritin > 5277 ng/mL. Conclusion. The dynamic changes in some laboratory data, especially PLT, can differentiate between MAS and active disease. The changes in WBC, PMN, and ESR and the levels of the liver enzymes may also be helpful in the early differentiation. Very high levels of ferritin may also help the diagnosis along with other clinical and laboratory signs.
Keywords	Medicine (General) ; R5-920
Subject code	610
Language	English
Publishing date	2015-01-01T00:00:00Z
Publisher	Hindawi Limited
Document type	Article ; Online
Database	BASE - Bielefeld Academic Search Engine (life sciences selection)

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