Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0011168 (dysphagia)
 15,644 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

From January 1, 1981 to December 31, 1992, we experienced nine patients with childhood onset of dermatomyositis and polymyositis. The mean age of disease onset was 12 years (range 7 to 16 years). Seven of them fulfilled the criteria of dermatomyositis, the remaining two were polymyositis. Girls were more predominant than boys in 6:3 ratio. The clinical features included extremities muscle weakness, skin rash, periorbital swelling and dysphagia. Increased muscle enzymes including creatine phosphokinase (CPK) or lactic dehydrogenase (LDH) were all positive in nine patients. All of our nine patients were treated with prednisolone after the diagnosis was established. The duration of treatment ranged from 3 to 65 months (mean: 25.3 months). Two of the nine patients also received immunosuppressive agents, hydroxychloroquine and azathioprine respectively. At present six patients survive without treatment. Two patients continue with corticosteroid and immunosuppressive therapy. One patient died from primary peritonitis, six months after being diagnosed with JDMS. In conclusion our study shows there is a female dominance; monocyclic clinical course is more common; and the prognosis is good in general, in the cases of juvenile dermatomyositis and polymyositis.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Dermatomyositis and polymyositis in childhood. 794 27

Hallervorden-Spatz syndrome is generally considered to be an autosomal recessively hereditary disorder of unknown etiology. Some reported cases have been known to be sporadic. We present a boy who suffered from regressive developmental milestones since he was 2 years and 6 months old. He began to manifest tremors of the upper extremities, followed by unsteady gait, choreoathetosis, dystonia, dysarthria, and dysphagia at 4 years old, and subsequently became completely bedridden at 6 years old. Neurologically, opisthotonus, rigidity of extremities, dystonia, hyperreflexia, profound emaciation, and bilaterally positive Babinski signs were present. The brain magnetic resonance imaging (MRI) done at the age of 8 years revealed symmetrical low signal intensity over the bilateral globus pallidi in the T2-weighted images coexistent with an area of high signal intensity over the anteromedial portion, known as "eye of the tiger" sign. Another MRI, followed up two years later, did not show marked difference in signal abnormalities over the globus pallidi in the T2-weighted images as compared with that of the previous one. However, progressive neurological deterioration existed.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Clinical and MRI study of the Hallervorden-Spatz syndrome: long-term follow-up of one case. 794 31

Five episodes of fungemias are described; all had occurred in children with leukemia or lymphoma between January 1, 1978 and December 31, 1990. These fungemias comprised 3.4% of the total septicemias encountered during that period. Three episodes occurred during the induction phase and two during relapse. All patients had fever of varying degree and duration. In addition to steroids, all were receiving combination antibiotics before the fungemia had occurred. All patients had severe neutropenia lasting more than one week. Bacteremia preceded fungemia in four patients. Two episodes were diagnosed antemortem. The same species were isolated from other sites in three cases. Fever, chills and gastrointestinal symptoms were the most common clinical features; other symptoms included cough, dyspnea, oliguria and azotemia. One patient experienced skin lesion, dysphagia, hoarseness and hemiparesis. Only one patient survived. The prognosis from fungemia in leukemia and lymphoma patients is very poor. Empiric antifungal therapy is indicated in neutropenic patients who have recurrent or persistent fever despite one week of broad spectrum antibiotics. Early diagnosis and treatment will aid in improving the overall poor outcome of this disease.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Candida tropicalis fungemia in children with leukemia and lymphoma. 821 55

Seven patients were endoscopically diagnosed as having a fungal esophagitis with mycologic or histologic support for the diagnosis from 1979 to 1991 in the Department of Pediatrics, National Taiwan University Hospital. The major causative agent was Candida albicans. Other fungi isolated were Candida Krusei, Trichosporon cutaneum, Trichosporon beigelii, and Rhodotorula rubra, but they all resembled one another under endoscopic examination. The most common presenting symptom was hematemesis, and the lower part of the esophagus was more often involved. Only one patient was documented to have oral thrush. Most of the children did not present typical symptoms of esophagitis such as dysphagia or odynophagia, and they tended to be in more advanced stages of the disease when the diagnosis was made.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Fungal esophagitis in children. 829 55