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Biomedical subjects

I J Hung

Publications and source records attributed to I J Hung.

At least 55 records · Page 3Linked to original sources
PubMed1992

New observations in a child with angiofollicular lymph node hyperplasia (Castleman's disease) originated from the mesenteric root.

Localized plasma cell type Castleman's disease (CD) is an unusual pathologic entity. It is frequently associated with clinical and laboratory characteristics and rarely occurs in children. Total surgical excision results in cure in all aspects. To make early diagnosis of mesenteric CD is not easy, especially for children. An 11-year-old Taiwanese boy was recently evaluated for anemia and delayed growth. His clinical findings included a syndrome of severe hypochromic microcytic anemia, neutropenia, thrombocytosis, hypoferremia, hypergammaglobulinemia, and growth failure. Radiological examinations (abdominal ultrasound, small intestinal series, and computerized tomography) identified hepatosplenomegaly, nephromegaly, and huge masses in the middle abdomen with precaval, celiac, and paraaortic lymph nodal enlargement. However, detailed physical examination failed to detect a mass. At laparotomy a double-fist-sized confluent mass was found arising from the mesenteric root. Most masses were discrete and were excised individually. The pathologic diagnosis was plasma-cell type angiofollicular lymph node hyperplasia (Castleman's disease). Seven weeks after surgery, he had an episode of acute hepatitis B. Postoperatively, he exhibited a dramatic growth spurt; the hemoglobin, red blood cell indices, serum iron, and immunoglobulins returned to normal in 2 months. Neutropenia, which has not been previously related to mesenteric CD, was an unexpected finding in our case; however, it resolved spontaneously 3 months after the surgery, suggesting its causal relationship with the tumor.

Castleman Disease↗
PubMed1992

Sex-linked agammaglobulinemia (Bruton's disease). A case report.

X-linked agammaglobulinemia (Bruton's disease) is a rare disease characterized by marked decrease in all classes of immunoglobulins and absence of circulating B cells and plasma cells. The affected boys frequently present with recurrent respiratory tract infections after 6 months to 2 years of age. A combination regimen of intravenous human immunoglobulins and strong parenteral antibiotics remains the stone of treatment. Herein we report a one year and ten months old boy with Bruton's disease. He has had repeated infections of upper and lower respiratory tract since 6 months of age. Immunological studies revealed panhypogammaglobulinemia, nearly total absence of circulating B cells, negative isohemagglutinins and reversed CD4/CD8 ratio. Pedigree analysis suggests a sex-linked recessive inheritance. Intravenous immunoglobulins as well as parenteral antibiotics were administered to overcome pneumonia and purulent otitis media. He is currently undergoing co-trimethoxazole prophylaxis and periodic intravenous immunoglobulins infusion.

Agammaglobulinemia↗
PubMed1991

[Hematological data analysis in children with thalassemia trait or hemoglobin H disease in Taiwan].

In the past several years, we have collected 91 cases diagnosed as beta-thalassemia trait, 94 cases as alpha-thalassemia trait and 43 cases as hemoglobin H disease. The ages ranged from 6 months to 15 years. The hematological data were analyzed. We calculated the mean +/- SD for each item of the erythrocyte indices (RBC, Hgb, Hct, MCV, MCH, MCHC, +/- RDW) and the Hb electrophoretic results (Hb A, Hb A2, Hb F, Hb H & Bart's) in each group, and tabulated them. Then, comparisons were made between beta and alpha-thalassemia traits or between the thalassemia trait and hemoglobin H disease. We found that each of the mean values for Hgb, Hct, MCV and MCH for children with the beta-thalassemia trait was significantly lower than that for children with the alpha-thalassemia trait. The mean MCV value of the beta-thalassemia trait group was 60.7 +/- 3.4 fl, and 87% of the MCV values in this group ranged from 55 to 65 fl. The mean MCV value for the alpha-thalassemia trait group was 63.3 +/- 3.3 fl, and 56% of the MCV values in this group ranged from 60-65 fl. The MCV values for all of the 185 children studied with either the beta- or alpha-thalassemia trait ranged from 54 to 71 fl, and were far below the normal ranges for MCV values for each age-specific group. The mean percentage of deviation of MCV from normal was greater than that of Hgb in both the beta- and alpha-thalassemia trait groups. A comparison of those with beta-thalassemia trait 6 years old and under and those over 6 years showed that both the mean values of RBC and Hgb were higher in the older subgroup, while the mean MCV values did not show any significant differences between these 2 subgroups.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1991

Hodgkin's disease in children: a review of 21 cases.

The clinical data of 21 children with Hodgkin's disease were retrospectively analyzed to identify their characteristics. Our patients were exclusively boys, ranging in age from 2 years and 9 months to 13 years and 9 months (median 7 years and 10 months). A diagnosis could not be made until after the 2nd to 4th biopsy attempt in 9 patients, with a median time lapse of 5 months from initial biopsy. The primary manifestation was generally nodal enlargement, but also included idiopathic cholestasis and Coombs' positive hemolytic anemia. The disease stages of the patients at diagnosis were 2 stage I; 5 stage II; 10 stage III; 1 stage IV; and 3 not determined. The histologic subtypes were 12 nodular sclerosis, 5 mixed cellularity and 4 lymphocyte predominance. Nine patients had "B" symptoms. Seventy-one percent were associated with anemia and the majority were microcytic. There was a high prevalence of advanced disease (61%). The therapy plan was affected by treatment philosophy at the time, availability of anticancer drugs and the family's attitude toward primary treatment. The patients were initially treated with either radiotherapy alone, chemotherapy alone or combined modality regimens. Five patients were lost within 3 months of diagnosis. The remaining 16 patients were followed, with the longest duration being 9.5 years. Two patients died: 4 were lost after 5-12 months of follow-up, (2 with disease, 2 with no evidence of disease); and the remaining 10 were still being followed (from 2 months to 9 1/2 years). Among those still being followed, 6 of them had discontinued their therapy 8 months to 4 years 5 months earlier and none of them had evidence of tumor recurrence.

Adolescent↗
PubMed1989

Evaluation of methotrexate containing chemotherapeutic regimens in the treatment of childhood undifferentiated non-Hodgkin's lymphoma and B cell acute lymphoblastic leukemia.

From September 1983 to October 1988, 13 undifferentiated non-Hodgkin's lymphomas (NHL) of Burkitt's or non-Burkitt's type and 3 B cell acute lymphoblastic leukemias were treated with various multiagent chemotherapy regimens containing modest to high dose methotrexate (HDMTX) infusions. All were children between the ages 2 years 8 months and 14 years 1 month. The group included 13 boys and 3 girls. The lymphomas were located primarily in the head and neck, 5; abdomen, 7; and lymph nodes, 1. The clinical stages at diagnosis were stage I, 1; stage II, 6; stage III, 3; and stage IV, 3. The MTX infusion dosage ranged from 300 to 4,285 mg/M2, and the total cumulative dose per patient ranged from 750 to 30,168 mg/M2. Citrovorum Factor Rescue was given following all MTX infusions, except for 62 of the 300 mg/M2 infusions. The serum MTX levels were monitored following all HDMTX. The chemotherapy related toxicities were graded and analysed. The clinical characteristics, which might predispose to HDMTX-related toxicities, were identified and are discussed. Our data reveals the inpatient and interpatient variations in the kinetics of MTX. There were no drug-related deaths, and the overall outcome of the patients was satisfactory. We conclude that MTX infusion continues to play an important role in the current management of childhood B cell malignancies; however, obstacles still remain, especially for those with widespread B cell disease.

Adolescent↗
PubMed1987

Intra blood-cerebrospinal fluid-barrier detection of hepatitis B virus.

Hepatitis B, a major public health concern worldwide, is caused by hepatitis type B virus, a hepdnavirus that infects only human and certain nonhuman primates, and replicates strictly in hepatocytes. By using the techniques of slot and Southern blot DNA hybridization, and electron microscopy, the presence of HBV was identified in the cerebrospinal fluid of three affected individuals.

Blood-Brain Barrier↗
PubMed1984

Lymphadenopathic type of Kaposi's sarcoma presenting with generalized petechial hemorrhages. A case report from Taiwan.

An 8-year-old Taiwanese girl presenting with generalized petechial hemorrhages, severe thrombocytopenia and lymphadenopathy was found to have primary Kaposi's sarcoma of lymph node without cutaneous involvement. This patient is a first-documented case of lymphadenopathic type of Kaposi's sarcoma in Taiwan. The associated severe thrombocytopenia resulting in generalized petechial hemorrhages as the initial manifestation is also unique in this case.

Child↗
PubMed1983

Hereditary factor VII deficiency in a Chinese family.

Hereditary deficiency of factor VII is demonstrated in a Chinese family. The proposita was a 32-year-old female with bleeding diathesis consisting of spontaneous ecchymosis, menorrhagia and recurrent haemarthrosis. The prothrombin time was prolonged and the prothrombin and proconvertin test was 10% of normal. The activated partial thromboplastin time and the Stypven-cephalin clotting time were normal. The prolonged prothrombin time could be corrected by the addition of normal serum, but not by adsorbed normal or coumadin plasma. The factor VII level was 3.6% of normal. One of her brothers had bleeding symptoms and died at age 25, suggesting that factor VII deficiency might have been present. 13 of her family members had partial deficiency of factor VII with plasma levels ranging from 24 to 50%. These results suggest an autosomal recessive inheritance with a homozygous state occurring in the proposita and possibly in her brother, and a heterozygous state occurring in 13 of her family members. Our study marks an extensive survey of factor VII deficiency in an Oriental family.

Adult↗