Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0013911 (emaciation)
 1,059 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Three cases of diencephalic syndrome, associated with brain tumors, are reported in this paper. Case 1. A 2-2/12-year old boy was initially admitted to our hospital because of failure to thrive which began at the age of three months. Physical examination revealed emaciation (weight, 7.8 kg), irritability and pallor without anemia. Horizontal nystagmus was seen. Laboratory studies were normal except for abnormally high plasma growth hormone (p-GH) which was incompletely suppressed by hyperglycemia (induced by glucose) and was not elevated by hypoglycemia (induced by insulin). A low grade astrocytoma of the optic nerve compression the hypothalamus was partially removed. After the operation followed by irradiation, p-GH returned to normal both in its basal level and in its reaction to insulin loading, then his gain of weight was accelerated. He was readmitted, however, at the age of 6 8/12 years with headache and vomiting. Since subtotal removal of the recurrent tumor and irradiation, preoperative symptoms have disappeared up to the present (7 9/12 years old). Case 2. A 3-9/12-year old girl was initially admitted because of failure to thrive since the age of 2 years. Examination on admission revealed emaciation (10.5 kg), irritability and right hemiparesis. Laboratory studies were normal except for high serum cholesterol (290 mg/dl). (p-HG was not measured) The patient had been well after the subtotal removal of the hypothalamic astrocytoma except occasional headache until the age of 6 years. She was readmitted at the age of 9 years with progressive emaciation.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[Diencephalic syndrome--report of three cases]. 671 44

Timely diagnosis of diencephalic syndrome is not often the case for patients presenting with failure to thrive (FTT) because of its rarity and lack of specific symptoms. Herein, we report two cases of diencephalic syndrome (2-year-old girl and 10-month-old boy) presenting with severe emaciation. Both patients had histories of poor weight gain for months despite having good appetites prior to diagnosis. Initial work-up did not reveal the diagnosis. Horizontal nystagmus was noted in both patients: by a neurologist in the first patient and by a family member in the second patient. MRI of the brain showed large suprasellar mass and pilocytic astrocytoma was confirmed by pathology in each case. The patients were started on appropriate chemotherapy with interval improvements in weight gain. These cases illustrate the importance of cranial imaging and consideration of diencephalic syndrome for children presenting with FTT despite normal or increased caloric intake.
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PMID:Diencephalic syndrome: a rare cause of failure to thrive. 2868 92