Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042963 (vomiting)
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The designation of "Infantile Bilateral Striatal Necrosis" (IBSN) was first given by Friede in 1975. However, this unusual condition was first described by Paterson and Carmichael in 1924. The disease is rare with uncertain etiology. The clinical picture includes choreoathetosis, abnormal eye movements, seizures and mental dullness. These circumstances often follow symptoms such as fever, vomiting and impaired consciousness. The final diagnosis is confirmed by pathological examination, which reveals symmetrical degeneration of bilateral basal ganglia. With present technology IBSN can be well demonstrated in the brain Ct scans or MRI scans nowadays. This article reports four cases with clinical manifestations which had appeared before the age of one year. Three cases had prodromal upper respiratory tract infection symptoms with vomiting, while seizure and impaired consciousness ensued. One case had several bouts of pneumonitis followed by seizures, impaired consciousness and abnormal eye movement. Brain sonogram of one of these cases showed hyper-echoic basal ganglia, while CT scans or MRI scans revealed symmetrical hypodensity or signal change over bilateral basal ganglia, respectively. All of these led to a bedridden life. These four cases are reported based on their clinical presentations and brain imaging findings, in spite of the absence of pathological confirmation. Some of the literature are also reviewed. To sum up, IBSN should be kept in mind in the differential diagnosis of symmetrical bilateral basal ganglia lesion after the exclusion of other disorders such as neurometabolic disorders, central nervous system infection, carbon monoxide intoxication, hypoxic-ischemic encephalopathy, tumors and cerebrovascular disorders etc.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:[Infantile bilateral necrosis of the striatum of corpus: report of four cases]. 151 18

A seven-month-old girl was admitted to the Pediatrics Department of Mackay Memorial Hospital with the following symptoms and signs: (1) high fever for more than five days; (2) injection of bilateral conjunctiva; (3) bright red lips with strawberry tongue; (4) edematous change of palms and soles, followed by digit desquamation; (5) an ill-defined, erythematous plaque on the scar of the BCG. Kawasaki disease was diagnosed, and high dose aspirin (100 mg/kg/day) and intravenous gamma-globulin (IVIG) (400 mg/kg/day) were given for four days. The patient was afebrile on the second day after IVIG infusion, and was discharged six days after admission. A small single daily dose of aspirin (10 mg/kg/day) was given after the afebrile days. Unfortunately, vomiting and consciousness disturbance were noted one day after discharge. Laboratory data showed elevated aspartate aminotransferase (AST), alanine aminotransferase (ALT) and ammonia. Hypoglycemia and prolonged PT and PTT were also noted. Reye syndrome was suspected, and the patient was admitted to the intensive care unit for further management. A liver biopsy gave findings consistent with Reye syndrome. In spite of intensive treatment, the infant expired on the second day after admission. In a review of the literature, no correlation between these two syndromes was found. This rare case is presented to warn that Reye syndrome may follow Kawasaki disease when aspirin has been prescribed at a high dose.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Kawasaki disease with Reye syndrome: report of one case. 162 54

Cases of penicillin-resistant pneumococcal meningitis have been reported in other countries since 1977, but never before reported in Taiwan. In 1990, two cases of the disease were diagnosed here. Case one was a two-year-old boy who had had fever and vomiting for several days prior to admission. Under the impression of meningitis, a spinal tap was done. The CSF yielded pneumococcus, which was misinterpreted as sensitive to penicillin. Penicillin (400,000 units/kg/day) was given parenterally without effect. On the 12th day after admission, another spinal tap still yielded pneumococcus. This time the sensitivity test was reread with great care, and then reported to be penicillin-resistant pneumococcus. Minimal inhibitory concentration (MIC) of penicillin was performed simultaneously and it revealed 0.1 microgram/ml. Vancomycin (60 mg/kg/day) was substituted for penicillin. The patient became afebrile two days later, and was discharged ten days later without sequelae. Case two, a five-month-old girl, was diagnosed to have meningitis because of fever, vomiting, tense fontanel and seizure on admission. After a spinal tap was done, she was put on ampicillin and cefotaxime. The fever subsided two days later. At that time, the CSF was reported to grow pneumococcus, again misread as sensitive to penicillin. The antibiotics was switched to penicillin, but fever recurred. The second spinal tap still yielded pneumococcus which was sensitive to penicillin but resitstant to oxacillin. Based on experience with the first case, penicillin was changed to vancomycin, and performed MIC immediately. The MIC was 1.0 microgram/ml. The patient became afebrile two days later, and was discharged in good condition after ten days of treatment.(ABSTRACT TRUNCATED AT 250 WORDS)
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:[Penicillin-resistant pneumococcal meningitis: report of two cases]. 177 62

During a 2-year period from January 1988 to December 1989, 125 patients (68 boys, 57 girls), aged 30 days to 9 years, were diagnosed as rotavirus gastroenteritis at this hospital. Diagnosis was made by identification of the rotavirus antigen in stool samples by latex agglutination assay. Ninety-nine (79.2%) of them were under 2 years of age. The seasonal peak in incidence was from January to March. The most common clinical characteristics were watery diarrhea (100%), followed by vomiting (68.8%), fever (68.0%), cough (42.4%), rhinorrhea (17.6%), convulsions (6.4%) and moderate to severe dehydration (1.6%). Fecal occult blood was positive in 4 patients and fecal leukocytes were positive in one patient. Stool cultures revealed concomitant infections with enteropathogenic Escherichia coli in 4 patients. Of the 106 patients who underwent serum electrolyte examinations, serum sodium concentrations ranged from 135-145 meq/L in 81.9% (86/106) and serum potassium concentrations ranged from 3.5-5.0 meq/L in 86.8% (92/106). Leukocyte counts greater than 15,000/mm3 were found in 10.8% (13/120) of the patients. All 125 patients recovered from the diarrheal illness on follow-up. Our results showed a different seasonal distribution of this disease from that of a previous observation between 1983-1984 in Taipei City and provides original clinical information on rotavirus gastroenteritis in children living in an area of Taipei County. Using the simple and rapid latex agglutination assay, we can make early diagnosis of rotavirus gastroenteritis. Thus, early treatment and early isolation of patients to prevent nosocomial infection among hospitalized patients is possible.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Rotavirus gastroenteritis in children: a clinical study of 125 patients in Hsin-Tien area. 206 88

A case of isovaleric acidemia appearing as diabetic ketoacidosis with acute encephalopathy and pancytopenia was reported. A three-year-old male patient, with mild psychomotor retardation, had recurrent bouts of acute encephalopathy and pancytopenia after episodes of upper respiratory infection. At admission, he had vomiting associated with dehydration, acidosis, ketonuria, coma and a pungent, rather unpleasant odor. Laboratory features included hyperglycemia, hyperammonemia, hyperamylasemia, hypocalcemia, neutropenia, thrombocytopenia and subsequent anemia. Urine organic acid profiles showed profuse amount of 3-beta-hydroxyisovaleric acid (295 mg/ml) and isovalerylglycine (616 mg/ml) by gas chromatography-mass spectrometry. Levels of amino acids in the serum and urine were normal. The patient received treatment with rehydration and insulin, with rapid improvement. After the acute illness, blood glucose levels returned to normal. The patient was doing well on a low-protein diet in recent 3 months.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Isovaleric acidemia: report of one case. 212 76

Nineteen children with clinical diagnoses of renal tubular acidosis were followed for periods ranging from 3 months to 20 years. Twelve patients had Type 1 renal tubular acidosis, five had Type 2, and two had Type 4. No sex predilection was found for any one of the types. Most patients had been diagnosed before 18 months of age, with failure to thrive the most common presentation. Tachypnea, polydipsia, polyuria, and vomiting were frequent symptoms. Some of these children had associated renal hypoplasia, vesicoureteral reflux, unilateral renal agenesis, glomerulocystic disease, adult polycystic kidney disease, and cyanotic congenital heart disease. Urinary anion gap may be useful for differential diagnosis of altered distal urinary acidification from other hyperchloremic metabolic acidosis. Furosemide test may need further investigation. Inability to raise urine to blood pCO2 gradient is helpful for diagnosis of Type 1 renal tubular acidosis. Hypokalemia, hypocalcemia, hypophosphatemia, decreased tubular reabsorption of phosphate, and hypercalciuria occurred in some patients. Complications included rickets in two, nephrocalcinosis in one, and episodic hematuria in one. There was relative bicarbonate wasting in children with Type 1 renal tubular acidosis, with a mean therapeutic bicarbonate requirement of 4.4 +/- 2.6 meq/kg/day. The mean bicarbonate dose for patients with Type 2 renal tubular acidosis was 8.3 +/- 2.6 meq/kg/day. Most children had good response to treatment with complete catch-up linear growth in 13, improved growth in 4, and continuing poor growth in 2. Two patients died during follow-up. Two other patients maintained normal growth without medication.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Renal tubular acidosis in childhood. 226 80

A 10-year-old mentally retarded girl was sent to our hospital due to generalized edema, vomiting and poor appetite for several days. Serum albumin level was low, but no proteinuria was detected. Her stool was bulky and foul. Stool examination for parasite with formalin-ether concentration method revealed negative result. Trypsin activity test of stool revealed low trypsin activity as compared with normal specimen. Daily fecal fat exceeded upper normal limit. The diagnosis of giardiasis was confirmed by duodenal juice examination. Intestinal histology revealed mild shortening of the villi with increased mononuclear cell infiltration in the lamina propria. The daily stool amount decreased markedly after treatment with metronidazole 250 mg three times a day for 7 days. The edema subsided during the treatment. Serum albumin bevel returned to normal after the treatment. Giardiasis with malabsorption syndrome has often been overlooked in Taiwan. It is advised that in case of malabsorption syndrome giardiasis should be included in the list of differential diagnosis.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:[Giardiasis with malabsorption syndrome: report of one case]. 227 67

During the past three and half years (Jan, 1984-Jun. 1987), 14 cases of ureteropelvic junction obstruction presented with abdominal pain were encountered in the Department of Pediatrics, Mackay Memorial Hospital. Eight cases were male and six female. Their ages ranged from 4 years old to 12 years old. The duration of abdominal pain lasted for several days in 5 cases, several months in 4, and several years in another 5 cases. The abdominal pain was usually recurrent. It occurred once every 2 to 6 months. The location of pain was predominantly in the left abdomen and the periumbilical area. Cramping was the most characteristic symptom. Besides abdominal pain, several cases also had vomiting or abdominal mass. Laboratory examination showed some cases to have microscopic hematuria and others pyuria. All were proved by renal ultrasonography to have varying degrees of hydronephrosis. Intravenous pyelography or retrograde pyelography also confirmed the diagnosis of ureteropelvic junction obstruction. In 9 of the 14 cases, surgical intervention was performed to correct abnormalities. Six cases were followed up regularly; all showed improvement, however, one developed ureteropelvic junction obstruction on the contralateral kidney one year later. The remaining five cases who did not receive surgical treatment continued to have recurrent abdominal pain.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:[Ureteropelvic junction obstruction presenting with abdominal pain: a analysis of fourteen cases]. 227 77

Cleaning naphtha, a product of Chinese Petroleum Corporation, is a complex hydrocarbon mixture which contains mainly aliphatic hydrocarbons. It is used as a cleaning fluid and solvent. Three toddlers mis-swallowed a mouth of cleaning naphtha accidentally. One developed multiple organ failure before death, another completely recovered without sequelae, and the other died very soon after mis-swallowing. Two fatal cases were both induced vomiting with aspiration immediately after mis-swallowing by their parents. Therefore, inducing vomiting should not be encouraged especially at home, and prevention of aspiration is very important in the management of such cases.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Mis-swallowing of cleaning naphtha: report of three cases. 227 78

Chylous ascites in neonates is an unusual and etiologically poor understood entity. Our first case was a female newborn who suffered from abdominal distension and recurrent vomiting after birth. The history, physical, laboratory, and radiologic evaluations were not diagnostic except the evidence of obvious ascites. Paracentesis was performed and ascitic fluid was obtained. She was later discharged on a strict low-fat medium-chain triglycerides formula. She was found to have continue increase in abdominal girth, poor growth and development, and respiratory distress in which led her to readmission at 8 months of age. Exploratory laparotomy was done in order to rule out an anatomical lesion in which may be obstructing the lymphatic flow; but no such lesion could be found. She expired at 1 year of age with chylothorax, chylopericardium and lobar pneumonia. The second case, a 37-day-old male baby, who was admitted because of right inguinal hernia. Milky ascitic fluid in the abdomen was incidentally found during herniorrhaphy. Analysis of the fluid revealed protein 1,616 mg/dl, glucose 487 mg/dl, and triglyceride 796 mg/dl. Culture of peritoneal fluid grew no bacteria. Other laboratory findings were: serum protein 4.8 mg/dl, and BUN 14 mg/dl. A plain film of abdomen and sonogram showed massive ascites. The infant was then put on Pregestimil with the hope that the medium-chain triglyceride formula would improve his condition. Since then the child's abdominal girth did not increase and he continued to growth and develop normally at 4 months follow up.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:[Neonatal chylous ascites: report of two cases]. 227 29


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