Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0020639 (hypoproteinemia)
 1,134 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A 61-year-old male stonecutter was in excellent health until December 1988 when he was admitted to Miyagikenritsu Semine Hospital with an abnormality on chest X-ray film. Chest X-ray film revealed a left anterior mass that was confirmed on chest CT scan and MRI. Biochemical examinations and serum protein electrophoresis demonstrated hypoproteinemia (5.4 g/dl) and hypogammaglobulinemia (7.9%, 0.43 g/dl). Lymphocyte subset analysis showed OKT4 epitope deficiency (OKT4 0.1% and Leu3a 28.4%), and decrease of CD4/CD8 ratio (0.65). An encapsulated anterior mediastinal mass and the thymus were resected on January 9, 1989, without difficulty. Histology revealed a thymoma with a spindle cell epithelial component. OKT4 epitope deficiency in a patient with Good's syndrome (thymoma with hypogammaglobulinemia) was our diagnosis. There was no change in the patient's serum immunoglobulin level after thymectomy. Fifteen cases of Good's syndrome have been reported in Japan, but this is the first report of OKT4 epitope deficiency with Good's syndrome in Japan.
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PMID:[OKT4 epitope deficiency in a patient with thymoma and hypogammaglobulinemia]. 170 Aug 38

A survival case of central pontine myelinolysis (CPM) is reported herein with a review of 22 MRI-analyzed CPM survivors in the literature. A 65-year-old male was struck on the forehead while in a traffic accident. He was almost fully conscious and neurologically free on admission. Laboratory data were normal except hyponatremia [122 mEq/l] and hypoproteinemia [5.8 g/dl]. 2 hours later he became drowsy and left hemiparesis. The CT scan showed right frontal cerebral hematoma leading to a remarkable midline shift. Immediately, aspiration of the hematoma was performed. On the next day, he returned to the initial neurological level. On the third day, however, he again became comatose followed by tetraparesis and ataxic respiration. While the CT scan at that time did not display any abnormality in the pons, the severe dysfunction of the brain stem occurred after the correction of hyponatremia strongly suggesting CPM. The serum Na levels were kept between 110 and 125 [mEq/l]. In addition, 375 mg of methylprednisolone had been daily administrated during and after a gradual correction of the hyponatremia, because glucocorticoid proved to be effective in the models of CPM. The neurological condition began to improve on the 14th day after admission. Eventually, he became conscious and independent in his daily activities, the time span being 8 months after the trauma. The trident MRI lesion in the basis pontis was 5 mm in diameter and much smaller than clinically expected. The 23 MRI-analyzed CPM survivors were reviewed in the literature including the present case. There were 7 males and 16 females, with a mean age of 48.5 years.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:[A case of central pontine myelinolysis with neurological recovery after administration of glucocorticoid]. 191 Sep 33

Neuromyelitis optica (NMO) associated with nephrotic syndrome and autoimmune-related pancytopenia has not been reported previously. We report herein a young woman who initially presented with bilateral blurring of vision and numbness in her hands. MRI disclosed multiple white matter lesions and a long cervical spinal cord lesion extending to the medulla oblongata. Serum aquaporin-4 antibody was positive and the patient was diagnosed with NMO. While in the hospital, she presented with hypoproteinemia and heavy proteinuria, meeting the diagnostic criteria of nephrotic syndrome. After high-dose methylprednisolone treatment, her vision improved significantly and urine protein quantity decreased. However, the patient subsequently developed severe pancytopenia with a positive Coombs' test. Thrombocytopenia finally led to uncontrollable gastrointestinal bleeding as the direct cause of the patient's death. This case illustrates the extremely rare condition of concurrence of NMO, nephrotic syndrome, and autoimmune pancytopenia in one patient, which suggests the involvement of organs beyond the central nervous system in NMO spectrum disorders.
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PMID:Neuromyelitis optica accompanied by nephrotic syndrome and autoimmune-related pancytopenia. 2723 48

Protein-losing enteropathy (PLE) is characterized by loss of serum proteins into the gastrointestinal tract. It may lead to hypoproteinemia and clinically present as protein deficiency edema, ascites, pleural or pericardial effusion and/or malnutrition. In most cases the site of protein loss is the small intestine. Here we present an unusual case of severe PLE in a 55-year old female with a one-year history of recurrent diarrhea, crampy abdominal pain, and peripheral edema. Endoscopy and MRI showed a diffuse inflammatory thickening of the sigmoid colon and the rectum. Surgical resection of the involved colon was performed and the symptoms were significantly resolved. The final histologic evaluation confirmed a diagnosis of a pseudomembranous colitis with cap polyposis-like features. Such a cause of PLE has never been described before.
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PMID:Protein-losing pseudomembranous colitis with cap polyposis-like features. 2852 19

A 17-year-old adolescent boy with a history of hypoproteinemia underwent Ga-NEB PET/MRI to evaluate possible lymphatic disorders suggested by FDG PET/CT imaging. The images revealed multiple lesions with increased Ga-NEB uptake and high signal on T2-weighted images, consistent with likely lymphatic disorder. Histopathological examination from biopsied specimen confirmed lymphangioma.
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PMID:Lymphangioma on 68Ga-NOTA-Evans Blue PET/MRI. 2974 95

Objective:To explore the early diagnosis, the treatment and theprognosis of invasive fungal rhinosinusitis.Method:By summarizing the clinical data of 18 patients, CT and MRI images, pathological diagnosis and follow-up results to analysis the early diagnosis and the treatment.Result:All 5 patients with invasive fungal sinusitis were infected with mucor, 3 of whom died of intracranial complications. Among 13 patients with chronic invasive fungal rhinosinusitis, 9 were aspergillus, 3 were mucor, and 1 was negative. There were 9 cases of diabetes, 1 cases of ankylosing spondylitis, 3 cases of hypoproteinemia, 1 cases of organ transplantation, and 1 cases of leukemia. There were 3 cases of death who unenforced the operation, 15 patients received enlarged endoscopic surgery, postoperative antifungal therapy. Followed up 1-3 years, relapse in 3 cases, 1 case of death.Conclusion:The diagnosis of FRS needs to be combined with clinical manifestations, imaging features and pathological findings. The treatment requires surgery to completely remove diseased tissue (enlarged sinus open surgery) combined with antifungal use in sufficient quantities.
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PMID:[Endoscopic sinus surgery for the treatment of invasive fungal sinusitis]. 2979 57