ABSTRACT
Achalasia is very uncommon in children, and cases accompanied with alacrima and adrenal insufficiency is even more uncommon. When these three disorders are seen altogether, it is called triple A syndrome. It is inherited in an autosomal recessive manner and has potentially life-threatening sequelae. So, pediatricians should always consider the possibility of triple A syndrome when seeing children with achalasia. Neurological abnormalities such as autonomic neuropathy, peripheral neuropathy, sensory impairment and mental retardation occasionally accompany. We report a 2-year-old girl who presented with repeated vomiting, short stature and alacrima. Diagnosis of achalasia was made after perfoming esophagogram and endoscopy and was confirmed with esophageal manometry. After pneumatic dilatation, she became asymptomatic.
Subject(s)
Child , Child, Preschool , Female , Humans , Adrenal Insufficiency , Diagnosis , Dilatation , Endoscopy , Esophageal Achalasia , Intellectual Disability , Manometry , Peripheral Nervous System Diseases , VomitingABSTRACT
Angiodysplasia is an important vascular lesion of the gastointestinal tract , located at the site of ectasia of intestinal submucosal veins and overlying mucosal capillaries. It is a major cause of morbidity from gastrointestinal bleeding in old age, but rather rare in children. Its cause is still unknown, though is mostly considered to be acquired as a result of a degenerative process associated with aging. We experienced a case of colonic angiodysplasia in 14-year old boy, diagnosed by colonoscopy. The patient had a history of repeated hematochezia for a year. This report summarized our case and reviewed the literature.
Subject(s)
Child , Humans , Male , Aging , Angiodysplasia , Capillaries , Colon , Colonoscopy , Dilatation, Pathologic , Gastrointestinal Hemorrhage , Hemorrhage , VeinsABSTRACT
Segmental necrotizing jejunitis is characterized by severe abdominal pain of acute onset, bilious vomitings and foul smelling loose stools containing blood. Pathologic features include circumferential intestinal wall inflammation ranging from edema with minimal congestion to severe congestion, hemorrhage with necrosis, ulceration, and gangrene with perforation. Early diagnosis and suitable supportive measures prevent unnecessary laparatomy and complications. There was no report of this disease entity in children in Korea. We experienced a case of segmental necrotizing jejunitis with fever, abdominal pain and bloody stools, which was diagnosed by exploration and was treated successfully by antibiotics and supportive measures.