Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0019209 (hepatomegaly)
 5,798 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This report reviews the manifestations in fifteen children of proved adenoviral pneumonia. Patients' ages ranged from 43 days to 4 years and 1 month. Twelve cases were younger than 2 years old. Adenoviral infections were proved by positive viral cultures or a four-fold increase of the complement fixation titer. Prolonged fever and cough were found in all cases. In 13 patients, respiratory distress occurred; 5 needed mechanical ventilation. Injected throats, conjunctivae and ear drums were common. Other clinical pictures included abdominal discomfort, hepatomegaly, skin rash, convulsion and bleeding tendency. Abnormal laboratory findings were mild anemia, leukopenia, thrombocytopenia, elevated erythrocyte sedimentation rate and C-reactive protein, impaired liver function test, and prolonged prothrombin time and partial thromboplastin time. Anemia (11 cases), leukopenia (7 cases) and elevated transaminases levels (7 cases) were more common than previously reported. All patients had para-hilar peribronchial infiltrates in chest roentgenography. Segmental atelectasis and compensated hyper-expansion were found frequently. Pleural effusion were noted in six of our cases. Air leak syndrome occurred in three patients who had received mechanical ventilation. Three of the 15 patients expired: one had a preceding measles infection, all had disseminated intravascular coagulopathy. For patients with antibiotic-resistant pneumonia, adenoviral studies should be done. Extrapulmonary manifestations, and some abnormal laboratory findings, i.e., mild anemia, leukopenia, impaired liver function are clues to adenoviral infections, while bleeding tendency can be regarded as a poor prognostic sign for children with adenoviral pneumonia.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Adenoviral pneumonia in children. 132 94

Two cases of atypical Kawasaki disease are reported. Case 1 was a five-month-old male infant admitted to this hospital with a 10-day's history of high fever. On examination, he appeared ill-looking and only hepatomegaly was noted. Laboratory studies showed leukocytosis, thrombocytosis, elevated ESR and pleocytosis in CSF. He was treated as sepsis with meningitis. Sudden death occurred on the eighth day of admission, and left coronary artery aneurysm with thrombosis was noted at autopsy. Case 2 was a four-month-old male infant referred to our hospital with fever and cervical lymphadenopathy of 11 day's duration, and unresponsive to antibiotics. Skin rash had developed after oxacillin injection. Echocardiogram, performed on the third day of admission, disclosed a 5-8 mm aneurysm of the left coronary artery and a 4 mm aneurysm of the right coronary artery. Before a specific diagnostic test for Kawasaki disease becomes available, we suggest that a possible diagnosis of Kawasaki disease and echocardiographic evaluation should be considered in case of (1) presence of partial criteria of Kawasaki disease with thrombocytosis; and/or (2) young infants with prolonged unexplained fever.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Atypical Kawasaki disease: report of two cases. 151 14

During the twenty-nine-year period from June 1959 to October 1988, 32 infants and children with histology-proved hepatoblastoma were seen at the Department of Pediatrics, National Taiwan University Hospital (NTUH). The age at diagnosis ranged from four days to six years, with a mean of one year and eight months. Most of the patients (87.5%) were under the age of three. There was no sex predilection (16 males, 16 females). The most common presenting signs were an upper abdominal mass (71.9%) and/or abdominal distention (62.5%). Physically, all the patients revealed hepatomegaly, with or without mass. Laboratory abnormalities included primarily thrombocytosis (64.3%), elevated serum aspartate aminotransferase (75.0%), serum alanine aminotransferase (62.5%), cholesterol (78.3%) and alphafetoprotein (92.3%). According to the classification of Ishak and Glunz, 56.3% of these belonged to the epithelial type; the others belonged to the mixed type. During the follow-up period, 6 patients with complete resection of the tumor remained alive from 2 years to 17 years and 4 months. Twenty-two patients died from 8 days to 16 months after diagnosis. Four patients were lost to follow-up. The two-year survival rate was 21.4% (6/28). Complete resection of the tumor was the key treatment for achieving survival.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Hepatoblastoma in infancy and childhood: a clinical and pathological study of 32 cases. 164 97

A 10-year-old boy with idiopathic scoliosis presented with exertional dyspnea, lower leg edema since two weeks prior to admission. Physical examination revealed neck venous engorgement, moist rales, mild hepatomegaly, ascites and lower leg edema in addition to the scoliosis. Chest x-ray films showed cardiomegaly, pulmonary congestion and pneumonic infiltration of both lower lobes with bilateral pleural effusion. Electrocardiograms showed right axis deviation and right atrial hypertrophy. Two-dimensional echocardiography revealed dilatation of the right heart chambers and pulmonary artery. He was treated initially with mechanical ventilation, antibiotics, bronchodilators, diuretics, vasodilators, and digoxin with improvement. Unfortunately the patient became comatose after an episode of cardiopulmonary arrest. He did not recover from this tragedy and was discharged against medical advice in comatose state. Scoliosis may be complicated with pneumonia and cor pulmonale in children. Early surgical correction of the scoliosis may prevent the development of cor pulmonale. Combined medical and surgical managements should be carried out aiming at scoliotic heart disease.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:[Idiopathic scoliosis and pneumonia complicated with cor pulmonale. Report of one case]. 227 31

A three-day-old female infant was transferred to the Pediatric Intensive Care Unit with chief presenting problems of progressive change of cyanosis and respiratory distress. Physical examination revealed tachypnea, acrocyanosis, hepatomegaly, undetectable pulse of extremities and oozing over the place of venous puncture. Chest roentgenograms revealed slight cardiomegaly; other X-rays were within normal limits. Complete electrocardiograms showed right axis deviation and right ventricular hypertrophy. Because of an impression of neonatal sepsis, the patient was put in an incubator with oxygen and antibiotics were given. Persistent anuria appeared associated with sighs of cardiac and renal failure; the ventilator was applied; dopamine and lasix were also given. Unfortunately, the cyanosis worsened progressive. Despite several attempts at resuscitations, the infant expired eight hours later. Pathology disclosed the heart size as normal; hypoplasia of ascending aorta as 0.4 cm in diameter; a PDA with 1 cm in diameter; a diminutive bean-sized left ventricle; hypertrophy of right ventricle and atresias of aortic and mitral valves. There was no evidence of septicemia.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:[Hypoplastic left heart syndrome due to aortic and mitral atresias: report of one case]. 263 9

To study the survival and prognostic factor of childhood acute lymphoblastic leukemia, 78 newly-diagnosed cases between January 1982 and June 1987 in National Taiwan University Hospital were reviewed and analyzed. They were stratified into two groups, i.e. standard-risk (SR) and high-risk (HR), according to their pre-treatment leukocyte count and age. Following induction therapy, 97% of the SR patients and 80% of the HR patients attained complete remission. In the SR group, the 2- and 3-year failure-free survival rates were 37% and 24%, with a median survival of 16 months. In the HR group, failure-free survival at the second and third year were 11% and 4%, respectively, with a median survival of 5.3 months. Three factors are strongly related to induction failure, i.e. high leukocyte counts (greater than 50*10(9)/1), massive hepatomegaly and large lymph nodes. Univariate analysis of failure-free survival showed six variables with significant detrimental effects on eventual outcomes, i.e. high leukocyte counts (greater than 50*10(9)/1), meningeal leukemia, marked lymphadenopathy, age younger than 2 years and older than 10 years, massive hepatomegaly (greater than 6 cm), and high LDH level (greater than 800 u/1). However, statistical survival models should also determine the joint effects of the prognostic factors so that the relative importance of each factor can be assessed. High initial leukocyte count, disclosed by multivariate analysis, was the single most important factor detrimental to the continuance of complete remission (P = 0.0004). Preliminary results also revealed poor compliance and early relapse in this study. Possible causes of early failure are discussed. Conceptual education for family members, as well as management with effective cytoreductive therapies are urgently needed.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:An analysis of risk factor and survival in childhood acute lymphoblastic leukemia. 263 11

Multiple myeloma is usually manifested with multiple destructive bone lesion, plasmacytosis and monoclonal protein. Here in we report a case of IgA (lambda) multiple myeloma with hepatomegaly and anemia but without bone lesions. Differential diagnostic points among lymphoma, amyloidosis, cancer and heavy chain disease are discussed.
Zhonghua Min Guo Wei Sheng Wu Ji Mian Yi Xue Za Zhi 1980 Dec
PMID:[A case report of variant form of multiple myeloma]. 697 40

A female infant, born at the gestational age of 29 weeks with a birthweight of 1,350 gm, developed progressive hepatomegaly at 10 days of age. Congestive heart failure gradually developed, and hepatic hemangioendothelioma was diagnosed at 1 month of age by open biopsy. Due to rapid enlargement of the tumor and progressive heart failure, steroid therapy was given from 36 days of age, including methylprednisolone 15 mg/kg/day for 3 days and 10 mg/kg/day for 4 days, then prednisolone 4 mg/kg/day for 20 days followed by tapering till 74 days of age. The tumor regressed gradually and was not detectable by sonography at 33 months of age. We suggest that, even in very-low-birthweight infants, the prognosis of hepatic hemangioendothelioma may be improved with aggressive therapy when symptoms develop during tumor progression, and that steroid should be the initial treatment.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Hepatic hemangioendothelioma: successful treatment with steroid in a very-low-birth-weight infant. 893 13

A 4-year-10-month-old boy with late infantile type neuronal ceroid lipofuscinosis was reported. He presented with progressive dementia, loss of visual acuity, gradual regression of speech and motor functions, and myoclonic jerks. A hyperactive deep tendon reflex was noted, but there was neither muscle weakness nor hepatomegaly. Serum lactate, pyruvate and ammonia levels were within normal limits. The funduscopic examination showed diffuse mottling of the retinal pigmented epithelium. The electroencephalogram showed irregular bilateral spike-and-waves or polyspike-and-waves and isolated focal spikes from the bilateral parieto-occipital regions. The wave forms of visual evoked potentials were flat. The electroretinogram was unrecordable. The somatosensory evoked potentials showed prolonged central conduction times from bilateral median nerves. The brainstem auditory evoked potentials were within normal limits. Diffuse cerebral and cerebellar atrophy were noted on magnetic resonance imaging. The diagnosis was confirmed by the electron-dense cytoplasmic inclusion bodies within the conjunctival squamous epithelial cells. No specific treatment was available. Regular anticonvulsants were not given during follow-up because seizures attacked him only on occasion. His visual acuity was progressively impaired. In addition to nearly total absence of speech, an inability to communicate and walk independently was also noted. The clinical features according to the subtypes, radiology, pathology, managements and prenatal diagnosis for this case are discussed.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Late infantile type neuronal ceroid lipofuscinosis: report of one case. 894 35

Choledochal cysts are malformations of the biliary ductal system, which rarely occur in infancy. In neonates and infants, choledochal cysts are congenital, may be associated with distal biliary atresia and typically presents with cholestatic jaundice and acholic stools characteristic of biliary obstruction. In older children and adults, it may be acquired in association with an anomalous pancreaticobiliary union and presents with any combination of intermittent jaundice, abdominal pain, and a palpable abdominal mass. Early detection of choledochal cysts is important in the prevention of the fatal complications of biliary obstruction. Here, we present two cases of congenital choledochal cyst in which the patients were 9 weeks old and 7 weeks old respectively. Both of them presented with jaundice, acholic stools, and hepatomegaly. Combined use of sonography, computed tomography and hepatobiliary scintigraphy demonstrated the presence of choledochal cysts. Thereafter, they underwent surgical excision of the cysts with Roux-en-Y reconstruction of the biliary tree; the results were successful.
Zhonghua Min Guo Xiao Er Ke Yi Xue Hui Za Zhi
PMID:Choledochal cyst in infancy: report of two cases. 929 33


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