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Biomedical subjects

I J Hung

Publications and source records attributed to I J Hung.

At least 37 records · Page 2Linked to original sources

Hemin-induced membrane sulfhydryl oxidation: possible involvement of thiyl radicals.

Sublytic levels (microM) of hemin destabilized RBC membrane as indicated by ghost fragmentation pattern using a laser viscodiffractometer. Furthermore, electron microscopic study shows that 5 microM of hemin induced echinocytic transformation whereas higher hemin concentration (40 microM) induced spherocytic transformation. In addition, hemin oxidized sulfhydryl groups in a dose dependent fashion and Electron Spin Resonance study suggests that such oxidation may involve a thiyl radical. Moreover, sulfhydryl compounds enhanced hemin-induced lipid peroxidation. Desferroxamine could prevent hemin-induced sulfhydryl oxidation as well as hemin-induced decrease in membrane stability. In contrast, vitamin E could effectively prevent hemin-induced lipid peroxidation but could not prevent hemin-mediated membrane destabilization.

Deferoxamine↗

Sulphasalazine therapy in chronic uveitis of children with chronic arthritis.

Four children with chronic arthritis (3 juvenile rheumatoid arthritis and 1 juvenile ankylosing spondylitis) and poorly controlled chronic uveitis, were given sulphasalazine (SASP) therapy for a mean period of 3.3 years. Three patients showed an excellent response, as evidenced by a reduction of inflammatory cells in the anterior chamber of the eyes and improvement of visual acuity. The response occurred after a mean of 7.7 weeks. These data suggested SASP therapy may have a role in the treatment of chronic anterior uveitis in children with chronic arthritis.

Adolescent↗

Leukemia cutis as the initial manifestation of acute nonlymphocytic leukemia in a young child.

A 15-month-old boy was well except for asymptomatic, erythematous, wheal-like papuloplaques, macules, and nodules on his face and four extremities. It was misdiagnosed by a pediatrician and treated as urticaria for six months. Later, he was sent to our hospital for evaluation of prolonged fever. Acute nonlymphocytic leukemia (M5) with leukemia cutis was diagnosed by results of hematologic examination and examination of a skin biopsy specimen. After one course of chemotherapy, all of the skin lesions completely resolved and had not recurred. Five months after acute nonlymphocytic leukemia was diagnosed, bone marrow relapse and central nervous system involvement were noted.

Humans↗

Enhanced vesiculation exacerbates complement-dependent hemolysis in glucose-6-phosphate dehydrogenase deficient red blood cells.

Glucose-6-phosphate dehydrogenase (G6PD) deficient red blood cells (RBCs) are known to be more susceptible to oxidant-induced hemolysis. Erythrocytes from G6PD-deficient individuals are significantly more susceptible to Ca(2+)-induced vesiculation than normal control cells. The enhanced susceptibility of G6PD-deficient RBCs to Ca(2+)-induced vesiculation is not due to ATP depletion. The remnant G6PD-deficient RBCs following vesiculation are more sensitive to complement-mediated hemolysis than control normal RBCs. A strong positive correlation exists between the level of Ca(2+)-induced vesiculation and the extent of complement mediated hemolysis.

Calcium↗

Correlation of membrane lipid peroxidation with oxidation of hemoglobin variants: possibly related to the rates of hemin release.

Experiments were performed to delineate the biochemical mechanism of hemoglobin (Hb)-catalyzed lipid peroxidation in human red blood cells (RBCs). Using a modified Langmuir trough lipid monolayer technique, we found that oxidized Hb induced an increase in lipid monolayer surface pressure, suggesting that oxidized Hb readily releases its heme moiety into the lipid monolayer. To confirm our interpretation that oxidized Hb readily releases its heme moiety, we monitored the fluorescence of Hb tryptophan upon oxidation of Hb. We found an increase in Hb fluorescence in the aqueous phase of our monolayer system after the addition of H2O2. The increase in fluorescence should reflect the departure of heme from globin due to a decrease in fluorescent quenching effect by the heme moiety. The rate of increase in lipid monolayer surface pressure upon Hb oxidation differed from Hb to Hb with an order of Hb E > F > S > A. The ability of various Hbs to affect lipid peroxidation in the RBC membrane, as monitored by the parinaric acid oxidation technique, followed this same order. In addition, hemin was shown to be a more potent catalyst of lipid peroxidation in RBC membrane than nonheme irons.

Adult↗

Early-onset sepsis in children with acute lymphoblastic leukemia.

The presence of early-onset sepsis significantly affects the prognosis of childhood acute lymphoblastic leukemia (ALL). We retrospectively analyzed all the septic episodes that occurred within 30 days of diagnosis of ALL from January 1978 to June 1994. Within the study period, 51 early septic episodes occurred in 45 patients. This accounted for 36.2% of the total number of septic episodes (141) that occurred during this period. The rate of early-onset sepsis in childhood ALL was 11.3% (45/397). The patients ages ranged from 11 months to 14.7 years. Fortyeight episodes of sepsis were caused by single organisms and three were polymicrobic. Pseudomanas aeruginosa was the most common isolate, followed closely by Staphylococcus aureus, Escherichia coli, Streptococcus spp and Salmonella spp. The case fatality rate of early-onset sepsis was 35.6% (16/45). The average time between diagnosis and death was 5.2 days (median, 1 d). Sepsis caused by P. aeruginosa had the highest mortality, with a case fatality rate of 72.7% (8/11), followed by polymicrobial infections (66.7%, 2/3) and candidemia (66.7%, 2/3). In the adolescent age group, initial white blood cell count < 2.0 x 10(9)/L, and depressed immunoglobulin level (especially IgA) were risk factors for a fatal outcome. During the study period, which covered 16.5 years, the incidence of Gram-positive cocci, especially Streptococcus spp, increased significantly and the overall outcome associated with early-onset sepsis improved.

Adolescent↗

Enhanced susceptibility of erythrocytes deficient in glucose-6-phosphate dehydrogenase to alloxan/glutathione-induced decrease in red cell deformability.

It has been hypothesized that enhanced oxidant sensitivity of glucose-6-phosphate dehydrogenase (G6PD) deficient red cells(RBCs) is the underlying mechanism for drug- or chemical-induced hemolytic crises in G6PD-deficiency. To further test this hypothesis, we used an alloxan-glutathione system to mimic oxidative stress and see how oxidative damage might affect RBC deformability. RBC deformability, a major determinant of RBC survival in vivo, was monitored by a laser viscodiffractometer. Under our experimental conditions, GSH alone had very little effect on the deformability of either normal or G6PD-deficient RBCs. In contrast, alloxan alone induced a small but significant decrease in the deformability of either normal or G6PD-deficient RBCs. Interestingly, alloxan and GSH together induced a further decrease in the deformability of either normal or G6PD-deficient RBCs. The decrease in deformability in G6PD-deficient RBCs was much more profound than in normal RBCs. In addition, an alloxan-vitamin C system produced a similar deleterious effect on RBC deformability as that produced by the alloxan-GSH system. Appreciable amount of hydroxyl radicals was generated by both alloxan-GSH and alloxan-vitamin C systems as evidenced by the production of hydroxylated products of salicylate which was used as a radical trap. Moreover, salicylate could ameliorate the deleterious effect of the alloxan system on the deformability of RBCs. Taken together, our results demonstrated that G6PD-deficient RBCs were particularly susceptible to oxidant-induced damage leading to a dramatic decrease in their deformability and thus provided strong support for the hypothesis that enhanced oxidant sensitivity of G6PD-deficient RBCs is the underlying mechanism for accelerated destruction of these RBCs in vivo.

Alloxan↗

Acute pancreatitis: an early manifestation of systemic lupus erythematosus.

Acute pancreatitis is thought to be quite uncommon in patients with systemic lupus erythematosus (SLE), especially if it is the initial manifestation of the disease. We report a 13-year-old girl with SLE whose early clinical presentation was acute pancreatitis. The disease was not related to concomitant drug therapy. It was associated with elevated serum and urine amylase levels and pancreatic enlargement by ultrasound assessment. The clinical symptoms and signs subsided after prednisolone treatment.

Abdominal Pain↗

Childhood acute nonlymphocytic leukemia in 88 patients.

In this study, 88 newly diagnosed children with acute nonlymphocytic leukemia (ANLL) were retrospectively analyzed for their treatment outcome. The patients included 51 boys and 37 girls, aged from four months to 14 years 11 months (median, seven years one month). The treatment consisted of chemotherapy using protocols which were standard at the time of diagnosis (1978-1991). Cerebrospinal fluid was not routinely examined. Fever with neutropenia was treated with empiric combination antibiotics, and anemia and thrombocytopenia were treated with blood components. Non-compliance was frequent; it was estimated that only 35.6% of all patients followed the given instructions. For the overall group (n = 57), 50.9% achieved confirmed complete remission. Median remission duration was five months and survival time was 11 months. The probability of continuous remission at 24 months was 20.9%. For the compliant group (n = 31), 51.6% attained complete remission, with a median remission duration of 14 months. The probability of continuous remission at 24 months was 44.5%, and the median survival time was six months. Early death occurred in 21.1% of all patients, 38.7% of compliant patients and 85.7% of acute promyelocytic leukemia patients. This study demonstrates how supportive treatment, socioeconomic factors and chemotherapy interact and affect the treatment outcome of ANLL in children.

Adolescent↗

Herpes zoster in infancy after intrauterine exposure to varicella zoster virus: report of two cases.

Herpes zoster occurs very rarely in infancy. We report two infants who developed zoster-like lesions at three and seven months of age. They had no history of chickenpox after birth. Both mothers acquired varicella infection during their sixth month of pregnancy. The crops of vesicular rashes appeared in right L1-3 dermatomes in one patient and left V3, C2 dermatomes in the other. Laboratory findings demonstrated a four-fold rise in varicella zoster virus (VZV) IgG antibody in one patient and positive VZV IgM antibody in the other. Both infants received acyclovir treatment and the skin lesions healed rapidly without sequela. In infancy, herpes zoster may be the primary clinical manifestation of reactivation of latent VZV infection acquired transplacentally during intrauterine life.

Chickenpox↗

Management of yolk sac tumor of the testis in children.

Yolk sac tumor (YST) is the most common form of malignant testicular tumor in infancy and children. However, there is no general agreement on its treatment regimen due to its rare occurrence. From 1978 through 1990, 30 cases of YST of the testis were treated at Chang Gung Children's Hospital. Thirteen (43.3%) occurred in the right testis and 17 (56.7%) in the left. Age at operation ranged from six months to five years with a mean of 1.79 years. All patients received an orchidectomy as their initial treatment. Eighteen patients with stage I disease were treated by orchidectomy alone. Fourteen (77.8%) of them were free of disease, two patients who had recurrence with retroperitoneal metastasis were successfully treated with chemotherapy. The remaining two patients died of metastasis. Seven patients with stage II disease and five with stage III disease were treated with combination chemotherapy, irradiation or debulking operation following orchidectomy. Four stage II patients (57.1%) and one stage III patient (20%) showed no evidence of disease at the time of follow-up. It was concluded that for stage I disease, orchidectomy alone followed by strict monitoring of alpha- fetoprotein achieved an 89% disease-free survival rate. Retroperitoneal node dissection or routine chemotherapy added no benefit to survival. For stage II or III disease, chemotherapy or irradiation is needed to obtain a better outcome.

Child, Preschool↗

Treatment of malignant germ cell tumors of the ovary.

Thirty-five patients with ovarian germ cell malignancies presenting from 1982 to 1992 were studied. The pathologic diagnoses were immature teratoma in 11 patients, endodermal sinus tumor in eight, dysgerminoma in 12, and mixed germ cell tumor in four. The median age at presentation was 20 years. Postoperative multiple-agent chemotherapy was given to 28 patients. Five out of 35 (14%) patients died of malignancy, three with endodermal sinus tumors with stage Ia, Ic, and IIIc, respectively, one with stage IIIc dysgerminoma, and one with stage IIIc mixed germ cell tumor. The other 30 patients were alive without evidence of disease from 14 to 124 months, with a median follow-up of 52 months. All eleven patients with immature teratoma were alive with no evidence of disease at 20 to 104 months of follow-up. Four patients with stage I, grade 1 immature teratoma were successfully treated with surgery alone. The other seven patients were treated with adjuvant chemotherapy and were disease-free. One patient with stage IIIc dysgerminoma was treated with surgery and radiotherapy but died of disease. The other eleven patients were safely treated with surgery and adjuvant chemotherapy with vincristine, actinomycin-D and cyclophosphamide (VAC) or platinum-based chemotherapy. For the more virulent tumors such as endodermal sinus tumor and mixed germ cell tumor, platinum-based chemotherapy should be used exclusively in view of the high failure rate of the VAC regimen. Preservation of fertility for young patients with malignant ovarian germ cell tumors is possible in the early stages of disease and in selected cases of advanced disease.

Adolescent↗

The morbidity and mortality associated with childhood onset systemic lupus erythematosus.

From 1977 till 1991, the diagnosis of systemic lupus erythematosus was made on 137 children aged 18 years or under in Chang Gung Memorial Hospital. The medical records were reviewed and the clinical data were analysed with emphasis on the morbidity and mortality of this disease. The clinical and laboratory characteristics were similar to the findings from most other reports. Renal failure accounted for 8% of the initial presentation. The non-infectious complications were, in the order of frequencies, hypertension, avascular necrosis of femoral head, cataract, encephalopathy, and venous thrombosis. Sepsis, cutaneous infection and urinary tract infection were the frequently encountered infectious complications. The major causes of death in childhood onset systemic lupus erythematosus were sepsis (42%) and renal failure (30.7%). Forty patients were lost to follow-up. The 5-year survival rate, calculated by life-table, was 76.3%.

Adolescent↗

Clinical features of Wilms' tumor and treatment results.

We retrospectively reviewed 34 children treated for Wilms' tumors at Chang-Gung Memorial Hospital from September 1979 to April 1992. The clinical data were analyzed. Patients were treated with standard multimodal therapy; three patients received preoperative chemotherapy. The survival and relapse-free survival curves were calculated for the overall group and compared with prognostic variables. Patients included 20 boys and 14 girls; the median age at diagnosis was 2 years 1 month. Congenital anomalies, hypertension and gross hematuria were found in 11.8%, 17.9% and 17.9%, respectively. The tumors were on the right side in 19 patients, on the left side in 13, and bilateral in two. Tumor weights ranged from 95 g (following preoperative chemotherapy) to 3500 g. The tumor was histologically favorable in 29 cases (85.3%), anaplastic in two and clear cell sarcoma in three. The clinical stages of these patients were: stage 1, 10; stage II, five; stage III, 15, stage IV, two; and stage V, two. The median follow-up duration was 26 months (range, eight weeks to 12 years 10 months). Eight patients had a poor outcome and four patients died of disease. The probability of survival was 83.6% and the relapse-free survival was 69.4% at five years. Tumor histology and clinical stage were associated with statistically significant differences in outcome. The complications and patterns of relapse are discussed. The annual incidence rate of Wilms' tumor in Taiwan is estimated to be 2.7 per million children under the age of 15 years.

Adolescent↗

Type C Niemann-Pick disease: report of a Chinese case.

We report on a 12-year-old Chinese child with type C Niemann-Pick disease, who presented primarily with neurologic symptoms. He started to develop ataxia and dysarthria at the age of six years. Dementia, dysphagia, dystonia and seizures, in that sequence, followed within a couple of years. He was anarthric and bedridden five years after onset. Supranuclear vertical gaze palsy was found at the beginning of the illness. However, no hepatosplenomegaly or other physical abnormality was noted. Bone marrow aspirates revealed foamy storage cells and sea-blue histiocytes. However, sphingomyelinase activity in the cultured skin fibroblast was normal. The characteristic clinical presentations and typical pathologic and histochemical findings meet the diagnostic criteria of type C Niemann-Pick disease. We report the first Chinese case of type C Niemann-Pick disease and review 73 cases reported previously.

Child↗

Effect of inhaled beclomethasone dipropionate in the treatment of recurrent wheezing in infancy and early childhood.

Forty-one children, aged six to 36 months (mean, 23.3 months), with a past history of acute bronchiolitis with or without recurrent wheezing episodes were treated with either a beclomethasone dipropionate metered dose inhaler 150 microgram twice daily, or a placebo for 12 weeks. Aerosols were inhaled through an AeroChamber (Trudell, Canada) using a mask. The patients were followed up biweekly. The two groups were well matched in anthropometric data and frequency of wheezing prior to the study being undertaken. At the end of four to six weeks, the beclomethasone dipropionate treatment group showed a significant improvement in both wheezing and sleep patterns, and systemic steroid therapy was able to be tapered. No significant side effect could be ascribed to this treatment.

Acute Disease↗

Treatment of childhood acute lymphoblastic leukemia with protocol TCL-842 in Taiwan: the Taiwan Children's Cancer Study Group.

From March 1984 to May 1988, 212 children with acute lymphoblastic leukemia were enrolled on Protocol TCL-842. In all, 68 patients were classified as standard risk (SR), 56 as intermediate risk (IR), and 88 as high risk (HR) groups. Remission induction for all three groups consisted of vincristine (VCR), prednisolone (PRED) and L-asparaginase (L-Asp). One consolidation course with cyclophosphamide (CP) and cytarabine (AraC) was used for the SR and IR groups, and two courses were given to patients in the HR group. Central nervous system prophylaxis was randomized using either cranial irradiation 18 Gy + 5 intrathecal methotrexate (IT MTX) or triple IT with maintenance. Reinforcement cycles were employed periodically during maintenance therapy (basically 6-mercaptopurine+MTX) and varied among the three groups. Four-week oral PRED every 16 weeks was the sole reinforcement agent for SR. Two-week VCR+dexamethasone (DEX)+adriamycin CP cycles were used to reinforce IR and HR at different intervals. Five third-form cycles with VCR+DEX+AraC were used only for HR. Treatment was discontinued after three years in patients who achieved continuous complete remissions (CCR). Eight patients died during the induction phase and eight failed to achieve complete remission (CR). The CR rate for SR was 97%, for IR was 98% and for HR was 83.3%; the overall rate was 91.8%. As of 30 June 1991, 33 patients had dropped out, 12 had died during remission, and 52 had relapsed. Twenty-eight SR, 26 IR, and 29 HR patients remained in CCR with a median follow-up duration of 66 months (38-88 months).(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗