Long-term follow-up of children with ınflammatory bowel disease: Evaluation of 53 cases

Giriş: Bu çalışmada inflamatuvar barsak hastalığı (İBH) tanısı ile izlenen çocukların uzun dönem demografik, klinik, laboratuvar, tedaviye yanıt özelliklerinin belirlenmesi amaçlanmıştır. Gereç ve Yöntem: Çalışmaya 0-18 yaş aralığında İBH tanısı ile izlenmekte olan 53 olgu dahil edilmiştir. Hasta grubu; klinik, serolojik, endoskopik, histopatolojik ölçütlere göre İBH tanısı konulan hastaları içermiştir. Hastaların doğum tarihleri, özofagogastroduodenoskopi/kolonoskopi bulguları, tanı anındaki ve izlem sırasındaki laboratuvar tetkikleri, yakınmaları ve süreleri, daha önce almış olduğu ve şu an almakta olduğu tedaviler ve eşlik eden hastalık varlığı gözden geçirilmiştir. Tanı anındaki ve tedavi sonrasındaki boy ve vücut ağırlığı Z skorları hesaplanıp karşılaştırılmıştır. Hastaların fizik muayene bulguları ile ailede İBH ve otoimmün hastalık öyküleri sorgulanarak kayıt edilmiştir. Bulgular: Olgularımızın 18’i Crohn hastalığı (CH), 35’i ülseratif kolit (ÜK) tanısı ile izlenmekteydi. Erkek/kız oranı CH’de 3,5/1, ÜK’de 1,33/1 idi. On olgunun (%18,9) akrabalarından birisinde İBH tanılı başka bir hasta bulunmaktaydı ve bu olgularda yakınma başlama yaş ortalamasının ailesinde İBH olmayanlardan istatistiksel olarak anlamlı derecede düşük olduğunu saptadık (p=0,042). Olguların 20’sinin (%37,8) anne babaları arasında akrabalık olduğunu tespit ettik. Akrabalık saptanan 20 olgunun yakınma başlama yaş ortalamasının akrabalık olmayanlardan istatistiksel olarak anlamlı derecede düşük olduğunu gözlemledik (p=0,025). Yaşa göre ağırlık Z skoru %18,9 olguda -2’nin altındaydı ve bu olguların yedisi CH tanısı ile izlenmekteydi. Yaşa göre boy Z skoru %17 olguda -2’nin altındaydı ve bu olguların dokuzu yine CH tanısı ile takip edilmekteydi. Tanı anındaki beyaz küre sayılarının, eritrosit çökme hızının ve C-reaktif protein değerlerinin tedavi sonrasında istatistiksel olarak anlamlı derecede gerilediği gözlenmiştir (p<0,001). İlk basamak tedaviye direnç ÜK’de %14,3 iken CH’de %33,3 idi. Sonuç: Ailede İBH tanılı başka hastanın olması ve anne-baba arasında akrabalık olması gibi durumlarda etiyolojide genetik mutasyonların olabileceğini düşünerek hastalığın daha erken yaşta ortaya çıktığını saptadık. Büyüme geriliğinin, fizik muayene bulgularının ve laboratuvar göstergelerin tedaviyle normale gelmesi İBH’de aktif hasta izleminin değerini ortaya koymaktadır.Introduction: In this study it was aimed to determine the long-term demographic, clinical and laboratory characteristics, together with the responses to therapy, in children diagnosed with inflammatory bowel disease (IBD). Materials and Methods: Fifty-three cases, aged 0 to 18 years, followedup with the diagnosis of IBD were included in this study. The study group consisted of patients diagnosed as IBD according to clinical, serologic, endoscopic and histopathological criteria. Dates of birth, esophagogastroduodenoscopy/colonoscopy findings, laboratory results at the time of diagnosis and during follow-up, complaints and their durations, treatments received presently and previously and comorbid diseases were documented. Patients’ heights, weights and Z scores at the time of diagnosis and following treatment were documented, calculated and compared. Family history of IBD and autoimmune disorders were questioned and recorded together with physical examination findings. Results: Among our cases, 18 were followed up with the diagnosis of Crohn’s disease (CD) and 35 had the diagnosis of ulcerative colitis (UC). Male to female ratio was 3.5/1 in CD and 1.33/1 in UC. Ten cases (18.9%) had the history of having a relative with IBD in their families. Mean age for start of complaints of this group was statistically significantly lower than the group having no family history of IBD (p=0.042). Twenty of the cases (37.8%) had history of consanguinity between parents. Mean age for start of complaints of this group, whose parents were consanguine, was statistically significantly lower than the group with non-related parents (p=0.025). Weight-for-age Z-score was below -2 in 18.9% of cases and seven of them were diagnosed with CD. Height-forage Z-score was below -2 in 17% of cases and nine of them were also followed-up with the diagnosis of CD. The white blood cell count, erythrocyte sedimentation rate and C-reactive protein value at the time of diagnosis were statistically significantly decreased following treatment (p<0.001). Resistance to first-line treatment was 14.3% in UC and 33.3% in CD. Conclusions: We determined an earlier onset of IBD in cases having consanguineous parents or another relative with IBD, considering that genetic mutations may play a role in etiology of the disease. Reversal of growth retardation, physical examination findings and laboratory parameters by treatment reveals the value of active patient follow-up in IBD

Evaluation of malnutrition development risk in hospitalized children

Objectives: Many screening methods, such as the Screening Tool Risk on Nutritional Status and Growth (STRONGkids) and the Pediatric Yorkhill Malnutrition Score (PYMS), have been developed to detect malnutrition in pediatric patients. We aimed to explore the prevalence of malnutrition risk in hospitalized children via symptoms and identification of contributing factors, and to examine the efficacy of malnutrition screening tools for hospitalized children

Diagnosis and management of cow's milk protein allergy in Turkey: Region-specific recommendations by an expert-panel

Cow's milk protein allergy (CMPA) is the most common type of food-allergy in younger Cow's milk protein children. Prognosis is usually good, with most children developing tolerance before school age. allergy; Children may present with a wide spectrum of symptoms that range from mild to severe; skin Consensus report; reactions such as angioedema and urticaria and gastrointestinal symptoms are the most common Turkey presentations of CMPA. Approximately one-third of CMPA patients suffer from multiple food allergies; severe conditions such as anaphylactic shock (9%), eosinophilic esophagitis (4.7%), and food-protein induced enterocolitis (1%) may also develop in some children. Timely and accurate diagnosis and management is essential for proper growth and development of children with CMPA. In this expert consensus report, we aimed to adapt current understandings in the CMPA field to the specific conditions in Turkey and health system to help physicians with their day-to-day decision making.Danone Nutrici

The Role of Telehealth Services in Children with Cystic Fibrosis During Coronavirus Disease 2019 Outbreak

Objectives: This study aimed to monitor the health and nutritional status of pediatric cystic fibrosis (CF) patients via telehealth services during the novel coronavirus disease 2019 (COVID-19). Additional aims were to determine the level of anxiety in the patients and their caregivers and to determine the COVID-19 transmission status among CF patients

Inflammatory bowel disease and guillain barre syndrome in FCHO1 deficiency

To the Editor: FCH And Mu Domain Containing Endocytic Adaptor 1 (FCHO1) gene encodes a protein that plays a critical role in clathrin-mediated endocytosis, a biological process that maintains cellular functions in signaling, nutrient- and growth factor- uptake, and diferentiation [1–3]. Recently, biallelic mutations in FCHO1 were linked to a combined immunodefciency that is characterized by recurrent infections caused by bacteria, viruses, mycobacteria, fungi, T cell lymphopenia, and hypogammaglobulinemia [4, 5].Sidra Precision Medicine Progra

Genomic investigation into early onset protein-losing enteropathies: Therapeutic implications and insights into clusters of shared pathomechanisms

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