ABSTRACT
We report the case of a 59-year-old female who developed facial edema together with hypoproteinemia. On the basis of (99m)Tc-human serum albumin scintigraphy and a1-antitrypsin clearance, she was diagnosed with protein-losing gastroenteropathy. Furthermore, she was diagnosed with Sjögren syndrome on the basis of eye and oral dryness, positive result with anti-SSA antibody, and salivary gland biopsy. Her symptoms improved with the use of immunosuppressive agents following steroid pulse therapy. Therefore, steroid pulse therapy and immunosuppressive agents should be considered as possible effective treatment strategies for protein-losing gastroenteropathy associated with autoimmune diseases.
ABSTRACT
We report here the case of an 83-year-old man who was treated for unconsciousness and hypoglycemia (39 mg/dL) accompanied by marked elevation of serum immunoreactive insulin (IRI) (4,760 µIU/mL). We diagnosed his condition as insulin autoimmune syndrome (IAS, Hirata disease) because of a high insulin autoantibody (IAA) titer (>90%: bound/total) and no history of exogenous insulin administration. Reactive hypoglycemia occurred due to immediate association followed by dissociation between insulin and insulin autoantibodies after glucose or food intake. An α-glucosidase inhibitor in combination with frequent small meals reduced the postprandial hyperglycemia (glucose spike) and ameliorated the reactive hypoglycemia.
Subject(s)
Autoantibodies/blood , Autoimmune Diseases/complications , Hypoglycemia/etiology , Hypoglycemia/immunology , Insulin Antibodies/blood , Insulin/blood , Insulin/immunology , Aged , Aged, 80 and over , Antigen-Antibody Reactions/immunology , Autoimmune Diseases/blood , Autoimmune Diseases/immunology , Blood Glucose , Glucose/administration & dosage , Humans , Hypoglycemia/blood , Kinetics , Male , Postprandial Period , Syndrome , Unconsciousness/blood , Unconsciousness/etiology , Unconsciousness/immunologyABSTRACT
Congenital portal systemic encephalopathy without liver cirrhosis and/or portal hypertension is rare. An 86-year-old man with senile dementia was admitted due to disturbance of consciousness. His serum ammonia level was high, but there was no evidence of liver cirrhosis or portal hypertension on laboratory tests and upper abdominal enhanced computed tomography (CT). However, on lower abdominal enhanced CT, a meso-caval shunt was found in the right lower abdomen. Superior mesenteric arteriography revealed a shunt flowing into the inferior vena cava via the right gonadal vein. The shunt was closed by balloon occluded retrograde transvenous obliteration, and dementia-like symptoms improved.