Contents

Primary Immunodeficiencies in The Children's Hopital, Affiliate of Vilnius University Hospital Santariškių Klinikos 5 Neringa Stirbienė, Aušra Šiškutė, Ramunė Pasaulienė, Vilma Rutkauskaitė, Rimantė Cerkauskienė, Jelena Rascon

Five-Year Data Analysis of Patients with Connective Tissue Disease-Associated Pulmonary Arterial Hypertension in Pulmonary Hypertension Coordination Center 11 Martyna Kirklytė, Lina Gumbienė, Rita Rugienė

Causes of Central Venous Catheter Removal in Pediatric Oncology and Hematology 14 Dovilė Rimkutė, Vidūnas Daugelavicius, Sigitas Strupas, Jelena Rascon

Children's Tuberous Sclerosis Complex: Ten Years of Experience 20 Dovilė Jonuškaitė, Rūta Praninskienė, Augustina Jankauskienė, Rimantė Cerkauskienė

Analysis of The Genetic Structure of Monogenic Epilepsies Through Bioinformatic Approaches 26 Birutė Tumienė, Algirdas Utkus

The Causes of Children Dyslipidemias - Single Center Experience and Review of The Literature 31 Rimantė Cerkauskienė, Marija Jakutovič, Vilija Černiauskienė, Viktoras Sutkus, Saulenė Račkauskaitė, Gabrielė Rusinaitė, Augustina Jankauskienė, Valerija Jablonskienė

D-Lactic Acidosis - The Complication of Short Bowel Syndrome 37 Vaidotas Urbonas, Evelina Liukpetrytė, Oleg Aliancevič

Achromatopsia Due to a Homozygous Mutation in CNGB3 Gene: Description of a Familial Case 42 Rasa Strupaitė, Birutė Tumienė, Susanne Kohl, Algirdas Utkus

Von Hippel-Lindau Syndrome: Single Adrenal Pheochromocytoma Surgery and Adrenal Insufficiency Substitution Therapy During Pregnancy: Case Report and Literature Review 49 Žydrūnė Visockienė, Kristina Švaikevičienė, Gintarė Zelnytė, Rita Šulcaitė

Diagnostic Challenge: Two Cases of Necrotising Encephalopathy in Childhood 56 Dovilė Jonuškaitė, Rūta Praninskienė, Gintaras Michailovas, Rimantė Cerkauskienė, Eugen Boltshauser

Concomitant Thyroid Inflammatory Myofibroblastic and Follicular Tumors: A Rare Case Report 61 Romena Laukienė, Audra Padlipskaitė, Edvardas Žurauskas

Autoimmune Polyendocrine Syndrome with Recurrent Serositis 66 Paulius Kalibatas, Skaistė Sendžikaitė, Rita Sudikienė

Managing a Rare Airway Compromised Laryngeal Vascular Malformation with Nd:Yag Laser 69 Gintas Pošiūnas, Emilija Burokaitė

Central Nerve System Involvement in Granulomatosis with Polyangiitis 73 Vygantė Maskoliūnaitė, Dalia Miltinienė, Rita Rugienė