ISSN: 2148-4902 | E-ISSN: 2536-4553
Northern Clinics of İstanbul - North Clin Istanb: 10 (3)
Volume: 10  Issue: 3 - 2023
EDITORIAL
1. Front Matter

Pages I - VIII

RESEARCH ARTICLE
2. Mentalization and emotion regulation abilities in parents of children with nocturnal enuresis and its relationship with perceived caregiver burden
Tuncay Toprak, Basar Ayribas, Recep Burak Degirmentepe, Mehmet Ozay Ozgur, Mehmet Yilmaz, Ayhan Verit
PMID: 37435288  PMCID: PMC10331249  doi: 10.14744/nci.2021.40225  Pages 281 - 288
OBJECTIVE: Various psychological factors play a role in the development of nocturnal enuresis (NE) which causes significant distress both on children and their parents. However, current studies cannot attribute a role to the psychiatric conditions that cause or result from NE. This study aims to reveal some psychiatric parameters related to the parents of patients with NE which may play a role in the etiopathogenesis of NE.
METHODS: Seventy-nine parents of primary 53 NE children and 78 parents of 44 healthy children were enrolled to the study. Parents of children with daytime voiding symptoms, additional comorbidity, or secondary enuresis were excluded from the study. Age- and sex-matched parents of healthy children with the absence of voiding symptoms were included as the control group. Parental Reflective Functioning (RF) Questionnaire, Interpersonal Emotion Regulation (ER) Questionnaire, and Zarit Caregiver Burden Scale were recorded to measure psychiatric conditions.
RESULTS: Parents of children with NE showed significantly poorer RF and ER abilities compared to the control group. Moreover, the perceived caregiver burden was also significantly higher in parents of NE patients. Correlation analyses also showed that RF and ER are negatively correlated with caregiver burden.
CONCLUSION: This study revealed that the parents of primary NE patients may have difficulty mentalizing and ER in interpersonal relationships. These difficulties may be a cause or a consequence of the NE. In addition, our findings showed that parents of NE patients perceive more caregiving burden. Therefore, it may be advisable for parents of NE patients to seek psychological counseling.

3. Relationship between insight level and clinical and familial features in pediatric obsessive-compulsive disorder
Dilsad Yildiz Miniksar, Busra Oz, Mikail Ozdemir, Tugba Yuksel
PMID: 37435285  PMCID: PMC10331248  doi: 10.14744/nci.2021.91979  Pages 289 - 297
OBJECTIVE: The aim of this study was to explore the relationship between insight level and clinical and familial psychiatric features of children with obsessive-compulsive disorder (OCD).
METHODS: Children’s Yale-Brown Obsessive-Compulsive Scale-Symptom Checklist, 11th item of the Children’s Yale-Brown Obsessive-Compulsive Scale, Wechsler Intelligence Scale for Children Revised Form, Affective Disorders and Schizophrenia for School Aged Children Present and Lifetime Version 1.0, and Structured Diagnostic Interview for Diagnostic and Statistical Manual of Mental Disorders-IV Axis I Disorders were applied to 92 pediatric OCD patients.
RESULTS: In this study, the prevalence of OCD in the first children of the family was high (41.3%), and low insight was significantly related with concomitant intellectual disability (p=0.003). The level of insight was high in patients with comorbid OCD spectrum disorders (p<0.001). Attention deficit and hyperactivity disorder (ADHD) was the most common psychiatric diagnosis accompanying OCD (19.5%). Among the obsession–compulsion subscales, the symmetry/hoarding was higher in males (p=0.046). OCD patients with a family history of major depressive disorder (MDD) had high ADHD comorbidity rates (p=0.038). In OCD patients, whose family had psychiatric disorders besides MDD and anxiety disorders, the diagnosis rate of intellectual disability was higher than other diagnoses (p<0.001).
CONCLUSION: The sociodemographic, clinical, and familial features of pediatric OCD patients cannot be adequately clarified if the patient has limited insight. Therefore, the insight of children with OCD should be considered a range or continuity.

4. Initial manifestations and risk factors for calcinosis in juvenile dermatomyositis: A retrospective multicenter study
Mustafa Cakan, Semanur Ozdel, Serife Gul Karadag, Kadir Ulu, Figen Cakmak, Gulcin Otar Yener, Kubra Ozturk, Esra Baglan, Hafize Emine Sonmez, Ferhat Demir, Betul Sozeri, Nuray Aktay Ayaz
PMID: 37435297  PMCID: PMC10331242  doi: 10.14744/nci.2021.11129  Pages 298 - 305
OBJECTIVE: This study aimed to look for the initial manifestations of juvenile dermatomyositis (JDM), give follow-up results, and search for risk factors for the development of calcinosis.
METHODS: The files of children with JDM diagnosed between 2005 and 2020 were reviewed retrospectively.
RESULTS: The study included 48 children, 33 girls and 15 boys. The mean age at the onset of the disease was 7.6±3.6 years. The median duration of follow-up was 35 (6–144) months. Twenty-nine patients (60.4%) had monocyclic, 7 (14.6%) patients had polycyclic, and 12 (25%) patients had chronic persistent disease course. At the time of enrollment, 35 (72.9%) patients were in remission, while 13 (27.1%) patients had active disease. Calcinosis developed in 11 patients (22.9%). Children having myalgia, livedo racemosa, skin hypopigmentation, lower alanine aminotransferase (ALT) levels, and higher physician visual analog scores at the time of diagnosis had a higher risk for calcinosis. Calcinosis was also more common in children with diagnostic delay and chronic persistent disease course. None of these parameters remained independent risk factors for calcinosis in multivariate logistic regression analysis.
CONCLUSION: The rate of mortality has decreased dramatically over decades in JDM, but the rate of calcinosis has not changed proportionately. Long duration of active, untreated disease is accepted as the main risk factor for calcinosis. We have seen that calcinosis was more common in children having myalgia, livedo racemosa, skin hypopigmentation, lower ALT levels, and higher physician visual analog scores at the time of diagnosis.

5. Are follistatin-like protein 1 and follistatin-like protein 3 associated with inflammatory processes in patients with familial Mediterranean fever?
Huseyin Kaplan, Mustafa Calis, Cevat Yazici, Inayet Gunturk, Isa Cuce, Abdurrahman Soner Senel
PMID: 37435280  PMCID: PMC10331238  doi: 10.14744/nci.2022.54189  Pages 306 - 313
OBJECTIVE: Follistatin-like protein 1 (FSTL-1) and follistatin-like protein 3 (FSTL-3) are glycoproteins whose associations with inflammatory cytokines were reported in previous studies. However, it is not yet known whether they have an effect on the pathogenesis of familial Mediterranean fever (FMF). We aimed to detect the FSTL-1 and FSTL-3 levels and to determine their relationship to the attack status and mutation types in patients with FMF.
METHODS: Fifty-six FMF patients and 22 healthy controls (HCs) were included in the study. Serum FSTL-1 and FSTL-3 levels were measured with the enzyme-linked immunosorbent assay method from collected serum samples. In addition, the MEditerranean FeVer (MEFV) gene mutation types of the patients were noted.
RESULTS: Serum FSTL-1 levels were significantly higher in FMF patients than in HCs (p=0.005). However, there was no significant difference in FSTL-1 levels between patients in the attack period (n=26) and in the attack-free period (n=30). FSTL-3 levels were similar between FMF patients and HCs or patients in the attack period and in the attack-free period. Furthermore, the MEFV mutation type and attack status had no significant effect on FSTL-1 and FSTL-3 levels (p>0.05).
CONCLUSION: Our results suggest that FSTL-1 may be associated with the pathogenesis of FMF, rather than FSTL-3. However, neither serum FSTL-1 nor FSTL-3 seems to be good markers to reflect inflammatory activity.

6. Fibulin1 and mesothelin expressions in pancreas ductal adenocarcinoma
Asude Aksoy, Gokhan Artas, Tuncay Kuloglu, Mustafa Koc
PMID: 37435286  PMCID: PMC10331239  doi: 10.14744/nci.2022.49260  Pages 314 - 321
OBJECTIVE: The balance between malignant tumor cells and the connective tissue surrounding them determines the aggressiveness of the tumor. We aimed to understand the effects of mesothelin (MSLN) and fibulin1 (FBLN1) expressions on survival in pancreas ductal adenocarcinoma (PDCA), and also whether these proteins have prognostic value for PDCA.
METHODS: Of 80 patients in total, 40 who underwent the Whipple procedure for diagnosed PDCA between 2009 and 2016, and 40 patients with diagnosed pancreatitis as the control group, were included in the present study. Immunohistochemically, MSLN, and FBLN1 expressions were evaluated retrospectively. We assessed the relationship between the degree of MSLN, FBLN1 expression, clinical-pathological features, and survival rates in PDCA cases.
RESULTS: The median follow-up duration was 11.4 (3–41) months. All of the patients for MSLN and FBLN1 were immune reactive. We detected a significant difference in MSLN expression between patients with PDCA and control groups, but not in FBLN1 expression. MSLN, FBLN1 expressions were categorized as lower-higher (L/H) groupings. There was no difference in the median overall survival (OS) of patients in the MSLN groups. The L-FBLN1 group had a median OS of 18 months (95% CI: 9.51–26.48) versus 14 months (95% CI: 13.021–14.97) in the H-FBLN1 group (interconnective tissue) (p=0.035). According to Kaplan–Meier analysis, L-FBLN1 expression in the tumor microenvironment was associated with longer survival in PDCA. The FBLN1 expression in the tumor microenvironment was shown to be significantly inversely related to OS (p=0.05).
CONCLUSION: The FBLN1 expression, which is in the tumor microenvironment of PDCA, may serve as a prognostic biomarker.

7. The validity and reliability of Turkish adaptation of the Fear-Avoidance Beliefs Questionnaire in patients with neck pain
Burcu Ozuberk, Ummuhan Bas Aslan, Gamze Ekici
PMID: 37435292  PMCID: PMC10331245  doi: 10.14744/nci.2022.48108  Pages 322 - 327
OBJECTIVE: “Fear-avoidance behavior” means that the individual avoids a specific activity or movement due to the perception that it may cause injury. Fear of movement associated with pain can reduce individuals’ adaptation to exercise programs. This situation may cause individuals to refrain from taking action and increase the existing limitation. Our aim is investigating of Fear-Avoidance Beliefs Questionnaire (FABQ) in patients with neck pain and creating a questionnaire option for clinicians and researchers to evaluate the fear-avoidance behavior in neck pain in Turkish.
METHODS: The sample of the research was comprised 175 patients between the ages of 18–65, who have a complaint of neck pain that lasted for at least 3 months. The test was applied on patients with neck pain and no treatment, with an interval of 2–7 days. The Visual Analog Scale (VAS), Neck Disability Index (NDI), and Nottingham Health Profile (NHP) were applied on the participants to evaluate the validity of the FABQ.
RESULTS: Accordingly, between FABQ and NHP (r=0.227), pain (NHPP) (r=0.214), emotional reactions (r=0.220), and physical activity (NHPPA) (r=0.243), a weak relationship was observed. A weak correlation was observed between physical activity (FABQ-PA) which are subscales of the FABQ questionnaire and NDI (r=0.210), NHPP (r=0.205), and NHPPA (r=0.267).
CONCLUSION: FABQ is a valid and reliable tool for patients with neck pain. In our study, a weak relationship was detected between FABQ, NDI, and NHP, as like VAS.

8. Anti-mutated citrulline vimentin antibodies may be higher in Ankylosing spondylitis, but may not have a role in diagnosing disease and may not be associated with disease severity
Nesrin Sen, Cahit Kacar, Sebahat Ozdem, Gulbahar Uzun, Mehmet Engin Tezcan
PMID: 37435295  PMCID: PMC10331240  doi: 10.14744/nci.2022.39225  Pages 328 - 334
OBJECTIVE: Ankylosing spondylitis (AS) is a rheumatologic disease with severe morbidity and mortality. Many studies in the literature showing that serum antibodies against anti-mutated citrullinated vimentin (anti-MCV ab) can be elevated in rheumatoid arthritis (RA) patients. However, there is little data in the literature about the levels of anti-MCV antibodies in AS patients. We designed the study to evaluate the role of anti-MCV antibody in the diagnosis of AS and to investigate whether it is associated with disease activity parameters.
METHODS: There were three separate groups in our study. The number of participants in these groups is 60 patients in the AS group, 60 patients in the RA group, and 50 healthy participants in the control group. The anti-MCV ab levels of the participants were measured by enzyme-like immune assay method. We compared anti-MCV levels between groups. We then evaluated its role in the diagnosis of AS and evaluated its relationship with disease activity parameters.
RESULTS: The anti-MCV antibody levels of both AS (p=0.006) and RA (p>0.001) patients were found to be significantly higher than controls. Anti-MCV antibody was higher than predefined threshold level (20 IU/mL) in 4 of 60 (6.7%) AS patients. Anti-MCV levels are similar in patients with or without a -acceptable symptom state (PASS). There is also no appropriate anti-MCV cutoff level with respect to PASS and a highly sensitive and specific level for diagnosis of AS.
CONCLUSION: Although AS patients has higher anti-MCV levels than controls, it may have a limited ability to AS diagnosis and to predict severity of the disease.

9. Oxidative stress, DNA damage, and inflammation in COVID-19 patients
Guven Bektemur, Kubra Bozali, Sahin Colak, Selman Aktas, Eray Metin Guler
PMID: 37435296  PMCID: PMC10331251  doi: 10.14744/nci.2022.00947  Pages 335 - 340
OBJECTIVE: Severe inflammation and oxidative stress seen in COVID-19 patients cause cumulative antiviral effects, and serious inflammation increases tissue, oxidative damage, and DNA damage. Therefore, in this study, oxidative stress, DNA damage, and inflammation biomarkers were investigated in patients diagnosed with COVID-19.
METHODS: In this study, blood samples were obtained from 150 Covid-19 patients diagnosed by polymerase chain reaction and 150 healthy volunteers with the same demographic characteristics. Total oxidant status (TOS), total antioxidant status (TAS), total thiol (TT), native thiol, and myeloperoxidase (MPO) activities were measured by photometric methods. The levels of tumor necrosis factor-alpha (TNF-α), interleukin 1 beta (IL-1β), and interleukin 6 (IL-6), which are inflammation markers, were measured by the ELISA method using commercial kits. The genotoxic effect was evaluated by Comet Assay.
RESULTS: The oxidative stress biomarkers; Disulfide, TOS, MPO, oxidative stress index, and IL-1β, IL-6, and TNF-α levels of inflammation biomarkers and the DNA damage in COVID-19 patients were increased (p<0.001), and the levels of TAS, TT, and NT In COVID-19 patients were decreased (p<0.001).
CONCLUSION: In COVID-19 patients, induced DNA damage, inflammation, and oxidative stress can guide the prognosis and treatment strategies of the disease.

10. Serum CXCL5 as a biomarker in multiple sclerosis and neuromyelitis optica spectrum disorder
Zerrin Karaaslan, Vuslat Yilmaz, Hande Yuceer, Elif Sanli, Halil Ibrahim Akcay, Murat Kurtuncu, Recai Turkoglu, Erdem Tuzun
PMID: 37435281  PMCID: PMC10331241  doi: 10.14744/nci.2022.77861  Pages 341 - 344
OBJECTIVE: Our aim was to determine whether serum C-X-C motif chemokine 5 (CXCL5) may serve as a diagnostic biomarker for relapsing-remitting multiple sclerosis (RRMS) as well as a marker that can be used to predict treatment response.
METHODS: CXCL5 levels were measured by ELISA in sera of 20 RRMS patients under fingolimod treatment, 10 neuromyelitis optica spectrum disorder (NMOSD) patients, 15 RRMS patients presenting predominantly with spinal cord and optic nerve attacks (MS-SCON), and 14 healthy controls.
RESULTS: Fingolimod treatment significantly reduced CXCL5 levels. CXCL5 levels were comparable among NMOSD and MSSCON patients.
CONCLUSION: Fingolimod might regulate the innate immune system. Serum CXCL5 measurement does not differentiate between RRMS and NMOSD.

11. Validation and reliability study of the Turkish version of the everyday cognition - 12 (T- ECog) scale
Nilgun Cinar, Fenise Selin Karali, Sevki Sahin, Miruna Florentina Ates, Sibel Karsidag
PMID: 37435290  PMCID: PMC10331235  doi: 10.14744/nci.2022.82084  Pages 345 - 352
OBJECTIVE: Assessing the activities of daily living (ADL) is important in cognitive impairment. The everyday cognition scale includes 12 items (ECog-12). It evaluates complex ADLs and executive functions. This scale can differentiate healthy elderly people from patients with mild cognitive impairment (MCI) as well as MCI from dementia patients. Our aim is to validate a Turkish version of ECog-12.
METHODS: The study group consisted of 40 healthy elders, 40 patients with Alzheimer’s disease (AD), and 40 patients with MCI. In addition to T-ECog-12, test - your memory- Turkish version (TYM-TR), Geriatric Dementia Scale (GDS), the Blessed orientation-memory-concentration (BOMC), and Katz ADL tests were administered to all participants for concurrent validity.
RESULTS: Cronbach’s alpha test showed excellent internal consistency (0.93). When T-ECog-12 was compared to the other tests, strong positive correlations were found between the GDS and BOMC; in addition, strong negative correlations were found between Katz ADL and TYM-TR scale. ECog-12 was found to be sensitive in differentiating healthy individuals from individuals with dementia (AD and MCI) (Area under the curve [AUC]=0.82, Cl=0.74–0.89). It was found to have low sensitivity in discriminating between MCI and healthy individuals (AUC=0.52, Cl=0.42–0.63).
CONCLUSION: T-ECog-12 was found to be reliable and valid for Turkish population. This scale is reliable and effective in diagnostic distinguishing healthy individuals from dementia.

12. Mannose-binding lectin (MBL) levels in children with Hashimoto’s thyroiditis
Ali Gul, Nihal Hatipoglu, Resul Yilmaz, Haci Omer Ates
PMID: 37435291  PMCID: PMC10331246  doi: 10.14744/nci.2022.22058  Pages 353 - 358
OBJECTIVE: Hashimoto’s thyroiditis (HT) was described many years ago, but the etiopathogenesis remains unclear. Mannose-binding lectin (MBL) initiates complement activation in the lectin pathway. We determined MBL levels in children with HT and the associations thereof with thyroid hormone and thyroid autoantibody levels.
METHODS: Thirty-nine patients with HT and 41 controls were enrolled from the pediatric outpatient clinics. Subjects were grouped according to their thyroid functions: Euthyroid, marked hypothyroidism and clinical/subclinical hyperthyroidism. MBL levels were compared among these groups. Serum MBL levels of the subjects were determined using MBL Human ELISA kit.
RESULTS: Serum MBL levels were studied in serum samples from the 80 subjects (48 (60.0%) females). MBL levels in HT and control groups were 50.787±34.718 and 50.593±44.28 ng/ml (p=0,983), respectively. In HT group, there was no significant difference in MBL levels between thyroid function groups (p=0.869). In addition, gender was not detected as a factor for serum MBL levels. Also we found negative correlation between WBC and serum MBL levels (r=-0.532; p=0.050). Otherwise there was no correlation between TSH, anti-TPO and anti-TG with serum MBL levels.
CONCLUSION: MBL levels did not decrease in HT patients. Further research is needed to elucidate more fully any role for MBL in the development of autoimmune thyroid disease.

13. Neuropathic pain in patients with post-COVID-19
Emine Burcu Comruk, Raikan Buyukavci, Erol Comruk, Semra Akturk, Yuksel Ersoy
PMID: 37435282  PMCID: PMC10331247  doi: 10.14744/nci.2022.31932  Pages 359 - 366
OBJECTIVE: In this study, we aimed to determine the continuing pain during the post-COVID-19 period the frequency of neuropathic pain in these patients, and the factors affecting the frequency.
METHODS: A total of 209 participants were included in the study who had COVID-19 disease (PCR-positive) aged 18–75 years. The demographic characteristics and COVID-19 severity data were recorded by questioning the patients. The musculoskeletal pain was also assessed using Visual Analog Scale (VAS) and the extended Nordic musculoskeletal system questionnaire (NMQ-E). In addition, the neuropathic components of pain were evaluated using the Leeds Assessment of neuropathic symptoms and Signs (LANSS) pain scale and the Pain-DETECT questionnaire (PDQ).
RESULTS: The mean time elapsed since COVID-19 was 5.76±2.95 months (min, 1; max, 12). Six patients (2.9%) had neuropathic pain according to the LANSS score, and 12 patients (5.7%) according to the PDQ score. The NMQ-E indicated that the most pain was detected in the back (20.1%), low back (15.3%), and knee (11.5%) regions during the post-COVID-19 period. According to both neuropathic pain scales; low back pain (p=0.001/0.001) and knee pain (p=0.001/0.01) were more common in patients with PDQ/LANSS neuropathic pain. Logistic regression analysis showed that there were significant associations between neuropathic pain and acute COVID-19 VAS score.
CONCLUSION: This study demonstrated that musculoskeletal pain was prominent mostly in the back, low back, and knee during the post-COVID-19 period. The incidence of neuropathic pain was 2.9%–5.7% depending on the evaluation parameters. Neuropathic pain is a finding that should be considered during the post-COVID-19 period.

14. Analysis of blood parameters and clinical features in women with pilonidal sinus disease: Is there a link between the disease and polycystic ovary syndrome?
Banu Yigit, Rumeysa Kevser Liman, Gulhan Kilicarslan
PMID: 37435284  PMCID: PMC10331243  doi: 10.14744/nci.2022.08784  Pages 367 - 377
OBJECTIVE: Pilonidal sinus disease (PSD) is a common disorder in the sacrococcygeal region and has a lower incidence in female as compared with male patients. The aim of this study is to evaluate clinical, hematological, biochemical, and hormonal parameters in women with PSD, and to determine whether the disease plays a major role in abnormalities of clinical and laboratory findings. This study also brings to the forefront the issue of the association between PSD and polycystic ovary syndrome (PCOS).
METHODS: The prospective single-center study included women with PSD, and an equal number of healthy women enrolled in the control group (50 women in each arm of the study). Medical history was taken from every patient, and blood tests were performed on all participants. Ultrasound imaging was performed to evaluate the ovaries.
RESULTS: Both groups were matched for age (p=0.124). The prevalence of obesity and dyslipidemia was significantly higher in women with PSD compared to controls (p=0.046, p=0.008, respectively). The right ovary volume was significantly higher in the study group than the control group (p=0.028). The study group had also significantly higher mean levels of neutrophil, C-peptide, and thyroid stimulating hormone (p=0.047, p=0.031, and p=0.048, respectively). The prevalence of PCOS was higher in patients with PSD, but the difference failed to reach statistical significance (32 vs. 22%, p=0.26).
CONCLUSION: Based on the findings of our study, some clinical and blood parameters differed significantly between women with and without PSD. Although the present study revealed that the prevalence of PCOS was not significantly different in women with or without PSD, more comprehensive and prospective studies are required.

15. Mean platelet volume as an effective biomarker for predicting high-risk patients of hereditary thrombophilia: A retrospective study
Hakan Keski
PMID: 37435289  PMCID: PMC10331236  doi: 10.14744/nci.2023.92331  Pages 378 - 384
OBJECTIVE: Literature shows evidence of the use of mean platelet volume (MPV) as a biomarker in thromboembolic conditions. It is recommended that genetic testing be performed selectively for hereditary thrombophilia. It might be useful to determine the priority of patients for genetic testing of hereditary thrombophilia through appropriate methods. We aimed to investigate the predictive value of MPV for high-risk patients of hereditary thrombophilia.
METHODS: The hematologic (MPV), biochemical (antithrombin III, protein S, protein C), molecular genetic test results (factor V Leiden [FVL], and prothrombin G20210A [PT]) obtained retrospectively from medical files of 263 patients categorized into high- versus low-risk for thrombophilia were statistically analyzed and the value of MPV in predicting high-risk patients was assessed by receiver operating characteristic (ROC) analysis.
RESULTS: The frequencies of high- versus low-risk patients were 45.2% and 54.8%, respectively. Significantly more high-risk patients (n=81) compared to low-risk patients had FVL (n=66) and PT mutations (n=80 vs. 34) (p<0.001). The MPV values in high-risk patients (mean=11.1 fl, range=7.8-13.6) were significantly higher than those in the low-risk patients (mean=8.6 fl, range=6-10.9) (p<0.001). The ROC curve analysis for MPV revealed a statistically significant area under the curve of 0.961 (95% confidence interval=0.931-0.981) at a cut-off point of 10.1 fl with a sensitivity of 89.1% and a specificity of 91.7% (p<0.001).
CONCLUSION: MPV might be used as an effective biomarker to screen and select patients for genetic thrombophilia testing. Large multicenter studies are needed for recommending the inclusion of MPV in future guidelines for hereditary thrombophilia.

CASE REPORT
16. Anti-N-methyl D-aspartate receptor encephalitis presenting with the new-onset refractory status epilepticus
Murat Alemdar, Turkan Acar, Sule Dalkilic
PMID: 37435283  PMCID: PMC10331237  doi: 10.14744/nci.2021.70431  Pages 385 - 389
New-onset refractory status epilepticus (NORSE) is a rare entity referring refractory status epilepticus (SE) in a patient without a history of epilepsy or an apparent cause. Herein, we report on a 31-year-old young female of anti-N-methyl D-aspartate (NMDA) receptor encephalitis admitted with NORSE. Her complaints began a week ago with a fever, meaningless movements, restlessness, and talking to herself. She had a history of operation for ovarian teratoma 10 years ago. Electrocardiography, hemogram, biochemistry, and neuroimaging were normal. Due to recurrent seizures despite intravenous diazepam infusions, phenytoin infusion was introduced, reducing the duration and frequency of seizures. Electroencephalogram (EEG) revealed a generalized slow background activity with low voltage and delta waves in left hemisphere derivatives without any epileptiform discharge. Autoimmune encephalitis panel revealed a positive anti-NMDAR receptor antibody. Intravenous immunoglobulins were given for 5 days. She was improved clinically and did not have a recurrent seizure. The history of our case emphasizes the importance of EEG and CSF antibody tests to reach the underlying etiology in patients presenting with refractory SE and neuropsychiatric symptoms of an unknown cause. Application of a proper treatment promptly with this approach could prevent the potential morbidity and mortality in these patients.

17. Acanthosis nigricans or terra firma-forme dermatosis? Three adolescent cases
Dua Cebeci, Filiz Cebeci Kahraman
PMID: 37435287  PMCID: PMC10331252  doi: 10.14744/nci.2022.56563  Pages 390 - 392
Terra firma-forme dermatosis (TFFD), also known as Duncan’ dirty dermatosis, is a keratinization disorder that is characterized by velvety, dark brown-blackish patches, and plaques and is not associated with systemic diseases. The lesions rarely show a verrucous or reticulate appearance. Especially, in children and adolescents, neck, face, torso, and ankles are the most frequently affected areas. TFFD is a condition that should be suspected in children and adolescents that cannot be cleaned with soap, especially if the neck area looks dirty. In this article, we report 3 cases with a diagnosis of TFFD resembling acanthosis nigricans. TTFD should be included in the differential diagnosis of adolescent cases presenting with hyperpigmented patches and plaques, especially in intertriginous areas such as the neck.

18. A pediatric case of Takayasu’s arteritis with antineutrophil cytoplasmic antibody-associated vasculitis triggered by COVID-19 infection
Sengul Caglayan, Gulcin Otar Yener, Kadir Ulu, Taner Coskuner, Meryem Guzel, Sevinc Kalin, Hakan Yazan, Seher Erdogan, Mustafa Cakan, Betul Sozeri
PMID: 37435294  PMCID: PMC10331244  doi: 10.14744/nci.2022.01878  Pages 393 - 397
Takayasu’s arteritis (TA) is a rare chronic granulomatous vasculitis characterized by large-vessel involvement. The aorta and its main branches are most commonly involved. Although pulmonary artery involvement is common, hemoptysis or respiratory findings are rarely seen. Herein, we present a case of TA who developed anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitis with diffuse alveolar hemorrhage after coronavirus disease 2019 (COVID-19) infection. A 17-year-old female patient with the diagnosis of TA presented with cough, bloody vomiting, and diarrhea. In follow-up, she developed tachypnea and dyspnea and was transferred to the pediatric intensive care unit. The findings on the chest computed tomography were compatible with acute COVID-19 infection, but the SARS-CoV2 reverse transcription-polymerase chain reaction test was negative, but SARS-CoV2 immunoglobulin (Ig) G and IgM antibody tests were positive. The patient was not vaccinated against COVID-19. The bronchoscopy showed bronchial mucosal fragility, bleeding foci, and mucosal bleeding. The broncoalveolar lavage hemosiderin-laden macrophages were seen in the histopathologic examination. The indirect immunofluorescence assay-ANCA test became 3 (+) with myeloperoxidase (MPO)-ANCA of 125 RU/ml (normal: <20). Cyclophosphamide and pulse steroid treatment were started. After immunosuppressive therapy, the patient condition improved and did not have hemoptysis again. The successful response was obtained by applying balloon angioplasty to the patient with bilateral renal artery stenosis. Types of post-COVID vasculitis include thromboembolic events, cutaneous vasculitis, Kawasaki-like vasculitis, myopericarditis, and ANCA associated vasculitis. It is thought that COVID-19 may impair immune tolerance and trigger autoimmunity with cross-reaction. To the best of our knowledge, the third pediatric case was reported with MPO-ANCA-positive COVID-associated ANCA vasculitis.

LETTER TO THE EDITOR
19. Oral pulsed high-dose dexamethasone therapy for rheumatic diseases: An alternative safe and effective scheme
Jozélio Freire de Carvalho, Thelma Skare
PMID: 37435293  PMCID: PMC10331250  doi: 10.14744/nci.2023.03704  Pages 398 - 400
Abstract |Full Text PDF

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