Browsing by Author "Dogan, G"

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    Clinical findings, child and mother psychosocial status in functional constipation
    Appak, YÇ; Sapmaz, SY; Dogan, G; Herdem, A; Özyurt, BC; Kasirga, E
    Background/Aims: Functional constipation (FC) is a common problem in childhood. In this study, we aimed to analyze the clinical and sociodemographic findings of patients with FC, parenting behaviors, and psychosocial states of children and parents. Materials and Methods: According to the Roma III diagnosis criteria, 32 patients with FC and 31 healthy controls were included. Patients' clinical and sociodemographic data set associated with constipation was determined. Strengths and Difficulties Questionnaire was used to screen the emotional and behavioral problems in children. To evaluate the parents and family, Beck Depression Inventory, State-Trait Anxiety Inventory, Parental Attitude Research Instrument were used. Results: Emotional and peer problems subscale scores, parental concerns as well as over-parenting attitude were found higher in patients. Significant difference was also observed between the groups in terms of mean score of authoritarian attitude dimensions. Attitude of hostility and rejection and marital discordance was found to be significantly high in patient families. Our study revealed a decrease in the constipation rate with the increasing education level of parents, higher rate of constipation in families with less income than expenses, and lower rate of working mothers in patients with constipation. Parents' depressive symptoms and anxiety level were determined to be considerably higher. Conclusion: A mother's low education level, low socioeconomic level, presence of psychological symptoms, and problems of parental attitude-primarily the authoritarian attitude-increase the risk of FC occurrence. Therefore, FC patients and their families should definitely undergo a psychosocial assessment.
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    Should we worry about the eyes of celiac patients?
    Dogan, G; Sen, S; Çavdar, E; Mayali, H; Özyurt, BC; Kurt, E; Kasirga, E
    Purpose: In this article, we evaluate subfoveal choroidal thickness in celiac patients with respect to adherence to the gluten-free diet and nonadherence to the gluten-free diet, comparing with age and sex matched healthy controls using spectral-domain optical coherence tomography. Materials and Methods: A case-control study among 42 celiac patients and 42 healthy participants was conducted in the Department of Pediatric Gastroenterology. Celiac patients of our policlinics compliant with spectral-domain optical coherence tomography examination enrolled in the study. Celiac patients had been asked verbally about their adherence to gluten-free diet, were evaluated according to negative or positive EmA and anti-TG2 for defining adherence, and were divided into two groups (adherence to gluten-free diet and nonadherence to gluten-free diet). Results: Subfoveal choroidal thickness was thinner in EmA (+) or anti-TG2 (+) eyes than EmA(-) or anti-TG2 (-) eyes in celiac patients, but it was not statistically significant. The mean subfoveal choroidal thickness values in eyes with celiac disease, whose diagnosis time was longer than 60 months, were thinner than shorter group. Longer duration of gluten-free diet was associated with adherence difficulty and thinner choroidal thickness (r = -0.15, p = 0.34). Adherence to gluten-free diet was 88.2% for children below the age of 60 months and 57.1% for children older than 60 months. Conclusion: In conclusion, in addition to other extraintestinal manifestations of celiac disease, diagnosis time longer than 60 months in pediatric celiac patients, nonadherence to the gluten-free diet, and antibody positivity should be focused on during ophthalmologic examination and choroid measurement.
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    Clinical significance of low transaminase levels in children with inflammatory bowel disease
    Cakir, M; Sag, E; Dogan, G; Unal, F; Kasirga, E
    BackgroundLow alanine aminotransaminase (LALT) levels may be seen in patients with inflammatory bowel disease (IBD), but there has been no study about the frequency and its clinical significance. We aimed to analyze the frequency of LALT, and its clinical significance in children with IBD.MethodsThe study included the 89 patients with IBD without hepatobiliary involvement. LALT was defined as ALT levels <5 U/L. Demographic and clinical findings and outcome of the patients with and without LALT were compared.ResultsLALT was found 47.1% of the patients. At initial examination, it was more common in female patients (92.3 vs. 41.3%, P<0.001) and patients with CD (57.7 vs. 30.2%, P=0.01). 75% of the patients with penetrating Crohn's disease (CD) had LALT (P=0.002). Hemoglobin (10.42.1 vs. 11.7 +/- 1.9g/dL, P=0.01), folic acid (5.2 +/- 3.3 vs. 8.6 +/- 5.9ng/mL, P=0.02) and serum albumin levels (3.6 +/- 0.8 vs. 4.7 +/- 5g/dL, P=0.002) were significantly low in patients with LALT. LALT was associted with the disease relapse within 2weeks in 12 of the 16 patients with LALT whereas it was seen in 16 of the 73 patients without LALT during the follow-up (75 vs. 21.9%, P<0.001). Additionally, steroid dependency was more common in patients with LALT during the follow-up (62.5 vs. 12.3%, P<0.001).ConclusionsLALT is common in children with IBD especially in CD and associated with low hemoglobin, albumin and folic acid levels. It may be a marker of relapse and steroid dependency.
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    The Association of Choledochal Cyst and Pancreatitis: A Case Report and Review of the Literature
    Appak, YÇ; Günsar, C; Dogan, G; Tarhan, S; Kasirga, E
    Choledochal cysts are relatively rare congenital anomalies which are characterized by dilatation of the extra-and/or intra-hepatic bile ducts. Pancreatic involvement before choledochal cyst operation might be a risk factor for postoperative pancreatitis. In this case report, we present a patient who had pancreatic enzyme elevation before choledochal cyst operation and who developed acute pancreatitis after surgery.
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    Evaluation of the Depression, Anxiety Levels and Attitudes of Mothers of Children with Celiac Disease
    Dogan, G; Sapmaz, SY; Appak, YC; Ozturk, M; Yigit, Y; Kasirga, E
    Objective: In celiac disease, a gluten-free diet is required for lifelong. Difficulties experienced by children with celiac disease can also negatively affect caregivers. The aim of this study is to evaluate the anxiety and depression of mothers having a child with celiac disease, and evaluate mothers' attitude towards their children. Method: Thirty-six children with celiac disease, their mothers, and 36 healthy controls were included in the study. The Parent Attitude Research Instrument, State-Trait Anxiety Inventory, and the Beck Depression Inventory were completed by all mothers. Results: The mothers of children with celiac disease had significantly higher scores in depression and state-trait anxiety than the mothers of the healthy children. Mothers of children with celiac disease had significantly higher scores in the attitude of overparenting, authoritarian attitude and attitude of hostility and rejection than the mothers of healthy children. Conclusion: This study revealed that having a child with celiac disease might have negative effects on mothers and their attitudes towards their children. Because of psychopathologic risks appropriate psychologic support should be provided for mothers.
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    A Rare Case of Ascites: Eosinophilic Gastroenteritis
    Dogan, G; Düzgün, F; Yilmaz, O; Ayhan, S; Gülen, H; Kasirga, E
    Eosinophilic gastroenteropathies are rare disorders. An 18-year-old girl presented to the hospital with abdominal pain and distension. Abdominal tenderness and grade 2 ascites, serious peripheral eosinophilia and high immunoglobulin E level were found. Laparoscopic antrum biopsy showed subserosal eosinophilic infiltration. Eosinophilic gastroenteropathy was suspected, diet elimination was given, no steroid treatment was used.
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    Relationship Between Duodenal Histopathology and Strong Positive Tissue Transglutaminase Antibodies in Children with Celiac Disease
    Dogan, G; Ayhan, S; Yilmaz, B; Appak, YÇ; Dündar, PE; Ecemis, T; Ünal, F; Kasirga, E
    Introduction: In celiac disease (CD) strong positive tissue transglutaminase antibody (TTGA) levels (> 100 U/A) have been shown to almost always indicate villous atrophy. The aim of this study is to determine the sufficiency of >= 100 U/A Ig A type TTGA levels for diagnosis of CD. Materials and Methods: Results from duodenum biopsy performed due to positive TTGA in 197 children were retrospectively examined. IgA TTGA levels had a positive value of > 18 U/A. Increases of 5 times or more than this threshold value (>= 100 U/A) are accepted as strong positivity. CD diagnosis was made according to ESPGHAN criteria. A modified Marsh stage >= 2 was accepted as significant for CD. Results: Of the cases, 129 were female (65.5%) and 68 were male (34.5%). Duodenum histopathology was compatible with Marsh 0 for 1 case (0.5%), Marsh 2 for 17 cases (8.6%), Marsh 3a for 41 (20.8%), Marsh 3b for 81 (41.4%) and Marsh 3c for 57 (28.9%). The TTGA levels of 64 of the 197 cases (32.5%) were >= 100 U/A. In cases with strong positivity for TTGA the duodenum histology was compatible with Marsh 3 (villous atrophy) for 63 and Marsh 0 (normal histology) for 1 case (type 1 diabetic and asymptomatic for CD). For Marsh 3c TTGA levels >= 100 U/A had a sensitivity of 85.96% (95% CI: 74.2-93.7%), specificity of 89.29% (95% CI: 82.9-93.8%), positive predictive value of 76.56% (95% CI: 64.3-86.2%) and negative predictive value of 93.9% (95% CI: 88.4-97.3%). Conclusions: This study showed that positive IgA TTGA levels (>= 100 U/A) were almost always accompanied by Marsh 3 duodenal histopathological changes. Diagnosis of CD without biopsy may miss certain accompanying diseases, however in some cases with advanced examinations CD may be diagnosed by pediatric gastroenterology specialists without endoscopy.
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    Retrospective evaluation of zygomycosis patients
    Cetin, CB; Dogan, G; Temiz, P; Tunger, O
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    Nutritional interventions improve quality of life of caregivers of children with neurodevelopmental disorders
    Ayca, S; Dogan, G; Sapmaz, SY; Dündar, PE; Kasirga, E; Polat, M
    Introduction: Children with neurodevelopmental disorders are at high risk for malnutrition. We aimed to investigate the impact of nutritional interventions to children with neurodevelopmental disorders and the quality of life of caregivers. Materials/Method: This is a prospective interventional study of 91 children with neurodevelopmental disorders. The children were separated into two groups: the intervention group and control group. The intervention group was selected from among children who had not been evaluated for nutrition and feeding problems by a pediatric gastroenterologist or dietician for the past one year. Children in the intervention group were called for follow-up visits and their nutritional intervention and anthropometric measurements were initiated by a pediatric gastroenterologist and dietician, at one month, three months, six months, and one year. The WHOQoL-BREF quality of life scale was completed by the caregivers of the children at baseline and at one year. Results: The intervention group had increased malnutrition (p < 0.001) and gastrointestinal system pathologies such as dysphagia (p < 0.001), constipation (p = 0.02), gastroesophageal reflux (p = 0.03) at baseline. After the nutritional intervention, 77.7% of the intervention group gained weight and 55.5% reached the target weight. The quality of life scale scores at baseline were lower among caregivers of the intervention group; however, they reached those of the control group after the nutritional intervention. Conclusion: Close multidisciplinary nutrition monitoring enables children with neurodevelopmental disorders to thrive appropriately and improves the quality of life of caregivers.
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    Neonatal Cholestasis as Initial Presentation of Portosystemic Shunt: A Case Report
    Dogan, G; Düzgün, F; Tarhan, S; Appak, YÇ; Kasrga, E
    Congenital intrahepatic portosystemic shunts are rare in children. Portosystemic venous malformations are characterized by extreme clinical variability. We report a full-term 33-day-old male infant presenting with neonatal jaundice. On physical examination, he had generalized icterus and the liver was palpable 3.5 cm below the right costal margin. He had no other symptoms. Laboratory tests showed AST 632 U/L, ALT 198 U/L, total bilirubin 12.1 mg/dL, conjugated bilirubin 10.2 mg/dL, alkaline phosphatase 753 U/L, GGT 47 U/L and glucose 67 U/L. Colour Doppler ultrasonography showed the left portal vein was more dilated than the right portal branch and communication with dilated left hepatic vein. There was no evidence of portal hypertension, heart failure, hepatopulmonary syndrome and encephalopathy during his hospital stay, so he was discharged from the pediatric department and his parents advised to attend monthly follow-up. Congenital portosystemic shunts are rarely observed in the childhood period. 1 Depending on anatomic characteristics they may be intrahepatic or extrahepatic. 2 Intrahepatic portosystemic shunts (PSS) are observed between the portal vein and hepatic vein or vena cava inferior. 3,4 Small shunts may close themselves before the age of 2 years. 5 With the increase in use of imaging methods, diagnosing PSS has become easier, with an increase in the number of cases reported. 6 Neonatal cholestatis is a frequent complication of PSS. 1 We present a case presenting with neonatal cholestasis diagnosed with congenital intrahepatic PSS.
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    ASSOCIATION OF MICRO RNA EXPRESSIONS WITH PEDIATRIC CELIAC CLINICAL FINDINGS
    Dogan, G; Boyacioglu, SO; Caliskan, M; Kasap, E; Ayhan, S; Kasirga, E
    There is a need to determine the relationship between the function of the immune system and miRNA expression in pediatric celiac disease (pCD). We aimed to describe the expression profiles of miRNAs in Turkish pCD patients based on the clinical and pathological findings. This study was conducted on 33 pCD patients and 33 pediatric control subjects with normal biopsy results. Four most common mutations (DQA1*05, DQB1*02, DQA1*03, DQB1*03:0.2) on HLA gene in pCD were screened. Paraffin -embedded biopsy tissue samples were used in miRNA isolations followed by cDNA synthesis. Expression of miRNAs were evaluated in the groups with qRT-PCR array -method. Significant underexpression of hsa-miR-194-5p gene was detected in pCD patients compared to the control group. The hsa-miR-194-5p gene was significantly underexpressed in anemic or short stature pCD patients compared to the control. The genes of hsa-miR-29b-3p, hsa-miR-30e-5p, and hsa-miR-146a-5p were significantly overexpressed in the patients with constipated celiac patients. Significant overexpression of hsa-miR146a-5p gene was detected in the Marsh2 and Marsh3a groups. The hsa-miR-29b-3p, hsa-miR-30e-5p, hsa-let-7a-5p, hsa-miR-27a-3p, hsa-miR141-3p, hsa-miR143-3p, and hsa-miR-146a-5p miRNA genes were significantly overexpressed in the Marsh3b group. Also, the hsa-miR-194-5p and hsa-miR-26a-5p genes were significantly underexpressed in the comparison of Marsh3c group to the control. These results suggest that miRNA expressions are likely to play a role in the pathogenesis of pCD. It is believed that the current results present valuable inferences that may help understand the genetic boundaries on pCD, which might be further supported by follow up studies on other miRNAs.
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    The effect of Celiac disease on cardiac functions and aortic elasticity parameters in children
    Alkan, F; Dogan, G; Kasirga, E; Coskun, S
    Aim: We aimed to investigate the effect of Celiac disease on myocardial functions and aortic elasticity parameters. Materials and Methods: Thirty children with Celiac disease and 30 healthy children were enrolled in the study. Both the groups were similar in terms of age and gender. Cardiac functions of all children in the patients and control group were evaluated using conventional transthoracic echocardiography and tissue Doppler imaging. Aortic strain, distensibility, and stiffness index were calculated by M-mode echocardiography. Results: The demographic findings, height, weight, and body mass index of cases were similar among two groups. No statistical difference was found between E wave velocity for conventional transthoracic echocardiography and tissue Doppler imaging measurements of the mitral valve; early diastolic flow peak velocity, A wave velocity; late diastolic flow peak velocity; and E/A ratio. Isovolumetric relaxation time and isovolumetric contraction time ratios were statistically different between the groups (p = 0.000, p = 0.000, p = 0.000). The myocardial performance index calculated according to the pulse Doppler measurement results was found to be statistically different between the groups (p = 0.000). There was no statistical difference between the groups in terms of aortic strain, distensibility, and stiffness index. Conclusion: In this study, both conventional transthoracic echocardiography and tissue Doppler imaging revealed the affection of the myocardial functions during systole and diastole in children with Celiac disease. Therefore, early follow-up and routine cardiac evaluation of celiac patients may be appropriate due to the increased risk of cardiac affection.
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    Measurement of Rectal Diameter and Anterior Wall Thickness by Ultrasonography in Children with Chronic Constipation
    Dogan, G; Keçeli, M; Yavuz, S; Topçu, A; Kasirga, E
    Background: Measurement of rectal diameter by ultrasonography helps the clinician in the diagnosis of chronic constipation in children for whom rectal examination cannot be performed. The aim of the study is to determine the rectal diameter and anterior wall thickness values in constipated and healthy children and to evaluate the feasibility of ultrasonography in the diagnosis of functional constipation in children who refuse digital rectal examination. Methods: One hundred forty constipated and 164 healthy children participated in the study. All patients were divided into 4 subgroups according to their ages (<= 3 years [group I], 3.1-6 years [group II], 6.1-12 years [group III], and >12 years [group IV]) and were referred to the radiology department. The measurement was made from above the symphysis pubis, under the ischial spine, and at the bladder neck. Anterior wall thickness measurement was performed. The measurements were recorded according to the presence or absence of fecal mass in the rectum. Results: Constipated children with fecal mass positive group III was found to have significant difference in all of the planes in rectal diameter measurement. Rectum anterior wall thickness measurement was found to be higher in constipated patients with fecal mass (+) compared to the control. Its measurements in constipated patients in group II, group III, and group IV with no fecal mass were found to be statistically higher than the control group. Conclusion: The measurement of rectal diameter and anterior wall thickness by ultrasonography as a noninvasive method was performed in children who did not want the digital rectal examination, and it may be useful in the diagnosis of constipation.
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    The Frequency of Lysosomal Acid Lipase Deficiency in Children With Unexplained Liver Disease
    Kuloglu, Z; Kansu, A; Selbuz, S; Kalayci, AG; Sahin, G; Kirsaclioglu, CT; Demirören, K; Dalgiç, B; Kasirga, E; Önal, Z; Islek, A; Eren, E; Hosnut, FÖ; Urganci, N; Yaman, A; Özkan, T; Bozbulut, E; Dogan, G; Eksi Bozbulut, N; Dogan, G; Durmaz Ugurcan, Ö; Usta, AM; Arslan, D; Akçam, M; Isik, IA; Ecevit, ÇÖ; Usta, Y; Özgür, T; Özçay, F; Balamtekin, N; Öztürk, Y; Balamtekin, N; Öztürk, Y; Cantez, S; Gülerman, F; Üstündag, GH; Emiroglu, HH; Karacabey, N; Comba, A; Erdemir, G; Aydogan, AU; Gökçe, S; Kuyum, P; Gülsan, M; Tosun, MS; Tokgöz, Y; Güven, B; Yüksekkaya, H; Tümgör, G; Eren, M; Baran, M; Gümüs, M; Canan, O; Kocamaz, H; Gerenli, N; Çakir, M; Agin, M; Hizli, S; Dogan, Y; Çeltik, Ç; Deveci, U; Balci Sezer, O
    Objectives: Evidence suggests that lysosomal acid lipase deficiency (LAL-D) is often underdiagnosed because symptoms may be nonspecific. We aimed to investigate the prevalence of LAL-D in children with unexplained liver disease and to identify demographic and clinical features with a prospective, multicenter, cross-sectional study. Methods: Patients (aged 3 months-18 years) who had unexplained transaminase elevation, unexplained hepatomegaly or hepatosplenomegaly, obesity-unrelated liver steatosis, biopsy-proven cryptogenic fibrosis and cirrhosis, or liver transplantation for cryptogenic cirrhosis were enrolled. A Web-based electronic data collection system was used. LAL activity (nmol/punch/h) was measured using the dried blood spot method and classified as LAL-D(<0.02), intermediate (0.02-0.37) or normal (>0.37). Asecond dried blood spot sample was obtained from patients with intermediate LAL activity for confirmation of the result. Results: A total of 810 children (median age 5.6 years) from 795 families were enrolled. The reasons for enrollment were unexplained transaminase elevation (62%), unexplained organomegaly (45%), obesity-unrelated liver steatosis (26%), cryptogenic fibrosis and cirrhosis (6%), and liver transplantation for cryptogenic cirrhosis (<1%). LAL activity was normal in 634 (78%) and intermediate in 174 (21%) patients. LAL-D was identified in 2 siblings aged 15 and 6 years born to unrelated parents. Dyslipidemia, liver steatosis, and mild increase in aminotransferases were common features in these patients. Moreover, the 15-year-old patient showed growth failure and microvesicular steatosis, portal inflammation, and bridging fibrosis in the liver biopsy. Based on 795 families, 2 siblings in the same family were identified as LAL-D cases, making the prevalence of LAL-D in this study population, 0.1% (0.125%-0.606%). In the repeated measurement (76/174), LAL activity remained at the intermediate level in 38 patients. Conclusions: Overall, the frequency of LAL-D patients in this study (0.1%) suggests that LAL-D seems to be rare even in the selected high-risk population.
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    HIV cascade of care in Turkey: data from the HIV-TR cohort
    Gokengin, D; Cimen, C; Cagatay, A; Gencer, S; Akalin, H; Ceran, N; Deveci, A; Ozdemir, HO; Erdinc, S; Dogan, G; Korten, V
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    Evaluation of the Knowledge, Attitudes and Behaviors of Pregnant Women on Tetanus Vaccination
    Çinar, G; Akdemir-Kalkan, I; Yilmaz-Karadag, F; Hocaoglu, M; Deniz, S; Isik, ME; Gülten, E; Erdem-Kivrak, E; Tüzün, T; Torun, A; Ünlü, G; Uygun-Kizmaz, Y; Akgül, F; Arslan, E; Dindar, K; Dogan, G; Sari, S; Bayindir, Z; Saricaoglu, EM; Kaplan-Atalay, E; Cavnar, AM; Haliloglu, EN
    Objective: Tetanus, a vaccine-preventable disease, still has the potential to threaten human health. Immunization, especially in pregnant women, is critical as it protects both mother and baby. This study aims to evaluate the tetanus immunization status of pregnant women, their approach to the vaccine, and the factors affecting these.Methods: A 19-item questionnaire was applied to pregnant women from all over Turkey who applied to the hospital for any reason and agreed to participate.Results: A total of 5000 pregnant women from seven geographical regions, aged between 15 and 44 (mean 28 +/- 5.7) and whose gestational age was between 8 and 40 weeks (mean 22.07 +/- 8.5), participated in the study. %88.2 of them did not get vaccinated in their current pregnancy, and %23.2 of them didn't plan to get vaccinated. When the reasons for not being vaccinated were questioned, %28.6 and %15.4 of them were afraid of side effects and harming the baby, respectively. In addition, %23.9 of them thought they didn't need to be vaccinated, and %7.1 of them stated that the vaccine didn't provide any protection. In univariate analysis, regions, age, gestational week, and the number of pregnancies resulting in delivery were found as factors that statistically significantly affected getting vaccinated, but in multivariate analysis, gestational week, education until secondary school, being followed by a family physician, history of more than three pregnancies, and being vaccinated in a previous pregnancy was found as an independent factor.Conclusions: Neonatal tetanus should be prevented not only because of its high mortality but also, the sequelae, and it can be prevented, minimized, or even completely eradicated by vaccination of pregnant women. For this purpose, every pregnant woman should be informed, and education and counseling support should be provided for vaccina-tion against tetanus.
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    Clinical characteristics, quality of life and risk factors for severity in palmoplantar pustulosis: a cross-sectional, multicentre study of 263 patients
    Solak, SS; Polat, AK; Kilic, S; Topal, IO; Saricaoglu, H; Karadag, AS; Canpolat, F; Kartal, SP; Deveci, BN; Kacar, N; Ekinci, AP; Guner, R; Polat, M; Dogan, G; Ozden, MG; Baskan, EB; Yavuz, GO; Adisen, E; Gulekon, A; Tanribilir, ME; Yilmaz, O; Karakas, AA; Ozturk, P; Balci, DD; Gonulal, M; Yondem, H; Ermertcan, AT; Sendur, N; Topyildiz, H; Korkmaz, S; Alpsoy, E
    Background Palmoplantar pustulosis (PPP) is a rare, chronic, inflammatory skin disease characterized by sterile pustules on palmar or plantar areas. Data on PPP are scarce. Aim To investigate the clinical characteristics and risk factors for disease severity in a large cohort of Turkish patients with PPP. Methods We conducted a cross-sectional, multicentre study of patients with PPP recruited from 21 tertiary centres across Turkey. Results In total, 263 patients (165 women, 98 men) were evaluated. Most patients (75.6%) were former or current smokers. The mean Palmoplantar Pustulosis Area and Severity Index (PPPASI) was 8.70 +/- 8.06 and the mean Dermatology Life Quality Index (DLQI) score was 6.87 +/- 6.08, and these scores were significantly correlated (r = 0.52, P < 0.001). Regression analysis showed that current smoking was significantly associated with increased PPPASI (P = 0.03). Coexisting psoriasis vulgaris (PsV) was reported by 70 (26.6%) patients. Male sex prevalence, PPP onset incidence, disease duration, DLQI, and prevalence of nail involvement and psoriatic arthritis (PsA) were significantly increased among patients with PPP with PsV. Of the 263 patients, 18 (6.8%) had paradoxical PPP induced by biologic therapy, and these patients had significantly increased mean DLQI and prevalence of PsA (r = 0.03, P = 0.001). Conclusion Our data suggest that smoking is a risk factor for both PPP development and disease severity. Patients with PPP with PsV present distinct clinical features and patients with biologic therapy-induced paradoxical PPP have reduced quality of life and are more likely to have PsA.
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    A snapshot of pediatric inpatients and outpatients with COVID-19: a point prevalence study from Turkey
    Yilmaz, D; Üstündag, G; Büyükçam, A; Sali, E; Çelik, Ü; Avcu, G; Belet, N; Taskin, EÇ; Demir, SÖ; Birbilen, AZ; Kiliç, Ö; Akcan, ÖM; Yilmaz, AT; Kocabas, BA; Hatipoglu, N; Karbuz, A; Çakir, D; Sütçü, M; Aygün, FD; Çelik, T; Sen, SB; Dalgiç, N; Ümit, Z; Kara, SS; Öncel, EK; Bolat, A; Çil, MK; Turan, C; Guzin, AÇ; Topal, S; Besli, GE; Dogan, G; Sahin, S; Akin, F; Bildirici, Y; Dayar, GT; Sari, EE; Isançli, DK; Kara, M; Önal, P; Aylaç, H; Lüleci, D; Yasar, B; Dede, E; Çaglar, A; Akova, S; Turgut, EA; Özkaya, PY; Gülmez, TK; Ulusoy, E; Duyu, M; Kara, Y; Çeliktas, H; Tekeli, O; Çaglar, F; Gül, D; Cebeci, SO; Battal, F; Bal, A; Aygün, E; Uysalol, M; Arslan, G; Özkavakli, A; Kizil, MC; Yazar, A; Aygün, F; Somer, A; Kuyucu, N; Dinleyici, EÇ; Kara, A
    This multi-center point prevalence study evaluated children who were diagnosed as having coronavirus disease 2019 (COVID19). On February 2nd, 2022, inpatients and outpatients infected with severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) were included in the study from 12 cities and 24 centers in Turkey. Of 8605 patients on February 2nd, 2022, in participating centers, 706 (8.2%) had COVID-19. The median age of the 706 patients was 92.50 months, 53.4% were female, and 76.7% were inpatients. The three most common symptoms of the patients with COVID-19 were fever (56.6%), cough (41.3%), and fatigue (27.5%). The three most common underlying chronic diseases (UCDs) were asthma (3.4%), neurologic disorders (3.3%), and obesity (2.6%). The SARS-CoV-2-related pneumoniae rate was 10.7%. The COVID-19 vaccination rate was 12.5% in all patients. Among patients aged over 12 years with access to the vaccine given by the Republic of Turkey Ministry of Health, the vaccination rate was 38.7%. Patients with UCDs presented with dyspnea and pneumoniae more frequently than those without UCDs (p < 0.001 for both). The rates of fever, diarrhea, and pneumoniae were higher in patients without COVID-19 vaccinations (p = 0.001, p = 0.012, and p = 0.027). Conclusion: To lessen the effects of the disease, all eligible children should receive the COVID-19 vaccine. The illness may specifically endanger children with UCDs.
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    The success of the Cystic Fibrosis Registry of Turkey for improvement of patient care
    Asfuroglu, P; Eyuboglu, TS; Aslan, AT; Gursoy, TR; Emiralioglu, N; Yalcin, E; Kiper, N; Sen, V; Sen, HS; Altintas, DU; Ozcan, D; Kilinc, AA; Cokugras, H; Baskan, AK; Yazan, H; Erenberk, U; Dogan, G; Unal, G; Yilmaz, AI; Keskin, O; Arik, E; Kucukosmanoglu, E; Irmak, I; Damadoglu, E; Ozturk, GK; Gulen, F; Basaran, AE; Bingol, A; Cekic, S; Sapan, N; Kilic, G; Harmanci, K; Kose, M; Ozdemir, A; Tugcu, GD; Polat, SE; Hangul, M; Ozcan, G; Aydin, ZGG; Yuksel, H; Topal, E; Ozdogan, S; Caltepe, G; Suleyman, A; Can, D; Ekren, PK; Bal, CM; Kilic, M; Cinel, G; Cobanoglu, N; Pekcan, S; Cakir, E; Ozcelik, U; Dogru, D
    Background Cystic fibrosis (CF) registries play an essential role in improving disease outcomes of people with CF. This study aimed to evaluate the association of newly established CF registry system in Turkey on follow-up, clinical, growth, treatment, and complications of people with this disease. Methods Age at diagnosis, current age, sex, z-scores of weight, height and body mass index (BMI), neonatal screening results, pulmonary function tests, history of meconium ileus, medications, presence of microorganisms, and follow-up were evaluated and compared to data of people with CF represented in both 2017 and 2019 registry data. Results There were 1170 people with CF in 2017 and 1637 in 2019 CF registry. Eight hundred and fourteen people were registered in both 2017 and 2019 of whom z-scores of heights and BMI were significantly higher in 2019 (p = 0.002, p =0.039, respectively). Inhaled hypertonic saline, bronchodilator, and azithromycin usages were significantly higher in 2019 (p =0.001, p = 0.001, p = 0.003, respectively). The percent predicted of forced expiratory volume in 1 sec and forced vital capacity were similar in 2017 and 2019 (88% and 89.5%, p = 0.248 and 84.5% and 87%, p =0.332, respectively). Liver diseases and osteoporosis were significantly higher, and pseudo-Bartter syndrome (PBS) was significantly lower in 2019 (p = 0.011, p = 0.001, p = 0.001, respectively). Conclusions The z-scores of height and BMI were higher, the use of medications that protect and improve lung functions was higher and incidence of PBS was lower in 2019. It was predicted that registry system increased the care of people with CF regarding their follow-up. The widespread use of national CF registry system across the country may be beneficial for the follow-up of people with CF.
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    Eligibility of Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) modulator therapies: cohort of cystic fibrosis registry of Türkiye
    Erdal, MA; Büyüksahin, HN; Sen, V; Kilinç, AA; Çokugras, H; Dogan, G; Yilmaz, AI; Ünal, G; Serbes, M; Altintas, DU; Arik, E; Keskin,Ö; Özaslan, MM; Karcioglu, O; Köse, M; Basaran, AE; Çakir, EP; Canitez, Y; Özdemir, A; Harmanci, K; Uytun, S; Polat, SE; Hangül, M; Yüksel, H; Özcan, G; Korkmaz, P; Kiliç, M; Aydin, ZGG; Çaltepe, G; Can, D; Dogru, S; Öztürk, GK; Süleyman, A; Topal, E; Özsezen, B; Hizal, M; Demirdögen, E; Ogun, H; Börekçi, S; Yazan, H; Sen, HS; Demir, AD; Çakir, E; Eyüboglu, TS; Emiralioglu, N; Pekcan, S; Özçelik, U; Dogru, D
    Background. Cystic Fibrosis Transmembrane Conductance Regulator (CFTR) variants are essential for determining eligibility for CFTR modulator drugs (CFTRms). In contrast to Europe and the USA, the treatment eligibility profile of cystic fibrosis (CF) patients in T & uuml;rkiye is not known. In this study we aimed to determine the eligibility of CF patients in T & uuml;rkiye for the CFTRms. Methods. The Cystic Fibrosis Registry of T & uuml;rkiye (CFrT) data was used to determine the age of patients in the year 2021 and the genetic variants they were carrying. Age- and CFTR-variant appropriate modulator therapies were determined using the Vertex (R) algorithm. Results. Among a total of 1930 registered patients, CTFR gene analysis was performed on a total of 1841 (95.4%) patients. Mutations were detected in one allele in 10.7% (198 patients), and in both alleles in 79% (1455 patients) of patients. A total of 855 patients (51.7% for whom at least 1 mutation was detected) were eligible for the drugs. The most appropriate drug among genotyped patients was found to be elexacaftor/tezacaftor/ivacaftor for 486 patients (26.4%), followed by ivacaftor for 327 patients (17.7%) and lumacaftor/ivacaftor for 42 patients (2%). Conclusions. Only half of patients registered in CFrT were eligible for CFTRms, which is a significant difference from the CFTR variant profile seen in USA and Europe. However, access to treatment is hampered for some patients whose genes are not analysed. Further studies in CF populations, where rare mutations are relatively more common, will contribute to the field of CFTR modulator treatments for such rare mutations.