Humans ; Hypertension, Portal ; diagnosis ; etiology ; therapy ; Pancreatic Diseases ; complications

The medical records of six cases of nesidioblastosis were examined to determine the diagnostic approach, treatment, and neurologic sequelae. All six patients were male, and their ages at the onset of the disease ranged from one day to six months (mean 3.36 +/- 2.5 mo.). Initial clinical features were seizure, cyanosis, poor feeding, and apnea. Other subsequent symptoms were developmental delay, hyperactivity, and cold sweating. The Birth weight of the neonatal onset group was heavier than the postneonatal onset group (4.4 +/- 0.3 vs 3.26 +/- 0.04 kg). Before the diagnosis of hyperinsulinism, steroids of ACTH proved effective for seizure control. Initially, hyperinsulinemia (serum insulin greater than 10 microU/ml) was detected in four cases, but another two cases also showed hyperinsulinism by insulin/glucose(I/G) ratio greater than 0.3 during the fasting test. The glucagon response performed in 2 cases, showed normal and partial responses. Euglycemia was obtained by near total pancreatectomy (95% pancreatic resection)without malabsorption or persistent diabetes. In one case, nesidioblastoma coexisted with nesidioblastosis. Developmental delay was noted in three cases. In this group, the mean duration between symptom onset and operation was longer than the group without developmental delay (1.25 +/- 0.47 vs 0.38 +/- 0.19 yr).
Brain Damage, Chronic/*etiology ; Child, Preschool ; Developmental Disabilities/etiology ; Humans ; Hypoglycemia/blood/*etiology ; Infant ; Infant, Newborn ; Insulin/*blood ; Male ; Pancreatic Diseases/complications/*diagnosis/therapy ; Postoperative Complications/epidemiology

Abnormalities, Multiple ; genetics ; pathology ; Child, Preschool ; Chromosome Aberrations ; Diagnosis, Differential ; Diarrhea ; diagnosis ; etiology ; therapy ; Exocrine Pancreatic Insufficiency ; complications ; Fever ; diagnosis ; etiology ; Fingers ; abnormalities ; Humans ; Male ; Pancreatic Diseases ; etiology ; Syndrome

Anorexia nervosa, a syndrome most commonly affecting young women, is characterized by weight less than 85% of weight that is considered normal for that person's age and height, distorted body image, and fear of becoming obese, and its mortality is up to 9%. We present a case of a 33-year-old woman with a 9-year history of anorexia nervosa. She admitted to our institution with decreased mentality, and her body mass index was 11.5 kg/m2 of the time admission. Initial aminotransferase level was severely elevated, but it was normalized solely with improved nutrition and weight gain. Five and sixteen days after the admission urinary tract infection and elevation of pancreatic enzymes occurred. They were successfully treated with antibiotics and nutritional support. Fifty seven days after the admission, she discharged. We report a case of acute hepatitis and pancreatitis treated with nutritional rehabilitation in a patient with severe anorexia nervosa for the first time in Korea.
Acute Disease ; Adult ; Alanine Transaminase/analysis ; Anorexia Nervosa/complications/*diagnosis ; Aspartate Aminotransferases/analysis ; Body Mass Index ; Female ; Humans ; Lipase/analysis ; Liver Diseases/enzymology/*etiology/therapy ; Nutrition Therapy ; Pancreatic Diseases/enzymology/*etiology/therapy ; Weight Gain