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Biomedical subjects

J S Hahn

Publications and source records attributed to J S Hahn.

At least 55 records · Page 3Linked to original sources

Expression of Fas antigen in acute myeloid leukaemia is associated with therapeutic response to chemotherapy.

Flow cytometric immunofluorescent analysis was used to assess Fas antigen (CD95) expression in blasts obtained from the bone marrow of 30 patients with acute myeloid leukaemia. The percentage of positive cells in each sample was highly variable. Fas antigen expression did not correlate with age, FAB subtype, white blood cell counts, or CD34 expression. Low expression of Fas was associated with a low complete remission rate after induction chemotherapy (62.5% in cases with < 20% positive cells v 92.9% in cases with > or = 20% positive cells, P < 0.01). The main cause for not achieving remission was resistant disease. Our results suggest that the quantitation of Fas expression can be predictive of treatment outcome in acute myeloid leukaemia.

Acute Disease↗

Iron related indices in iron deficiency anemia of geriatric Korean patients.

The purpose of this study was to compare iron related indices in patients with iron deficiency anemia and chronic causative diseases between geriatric older than 65 years and adult, nongeriatric younger than 65 years groups. Iron deficiency anemia (IDA) cases with chronic disorders from Youngdong Severance hospital from June, 1991 to April, 1994, older than 65 years (17 cases), and younger than 65 years (29 cases) were analysed with iron related indices. Mean hemoglobin was 7.8 +/- 2.2 g/dl in geriatric IDA and 8.0 +/- 1.8 g/dl in adult IDA without significant difference. RDW value was 19.5 +/- 2.6 in geriatric IDA and 18.4 +/- 3.2 in adult IDA with no significant difference. Serum iron and transferrin saturation between geriatric IDA were 22.7 +/- 12.3 ug/dl, 6.7 +/- 4.1% and 28.6 +/- 16.6 ug/dl, 7.1 +/- 4.4% in adult IDA with no significant difference, but TIBC was significantly lower (P = 0.011) in geriatric IDA than in adult IDA patients (357.2 +/- 83.2, 413.6 +/- 54.0 ug/dl). In normal elderly people, serum ferritin was 152.5 +/- 95.4 ng/ml in male and 111.1 +/- 54.1 ng/ml in female with range 19.8 approximately 367.7 ng/ml in male and 11.7 approximately 238.7 ng/ml in female and was higher than that of normal adult in both sexes (147.0 +/- 108.0, 35.3 +/- 20.5 ng/ml) (P = 0.045). Serum ferritin in geriatric IDA was 13.8 +/- 11.8 ng/ml and 5.7 +/- 4.0 ng/ml in adult IDA with significant difference(P = 0.001). The Upper margin for geriatric IDA was 37 ng/ml with 95% confidence interval. In the diagnosis of geriatric IDA with causative diseases, we should consider that TIBC does not increase and the upper margin for serum ferritin is suggested to increases up to 37 ng/ml.

Aged↗

Brainstem multiple sclerosis in an 11-year-old child presenting as acute disseminated encephalomyelitis.

Multiple sclerosis and acute disseminated encephalomyelitis are demyelinating disorders of the central nervous system that can present initially as an acute focal demyelinating syndrome. We report an 11-year-old girl who initially presented with intractable vomiting and hypertension and later developed a subacute onset of focal neurologic (brainstem) signs. Magnetic resonance imaging (MRI) demonstrated a large solitary demyelinating lesion of the brain stem consistent with acute disseminated encephalomyelitis. Because of the morbidity associated with biopsy and its questionable value in the course of management of this patient, she was treated empirically with aggressive supportive measures and high-dose corticosteriod therapy. She had near full recovery, with only minimal neurologic sequelae. Six months later, she presented with similar focal neurologic signs, and a new lesion was found on MRI. Because of the separation of her two episodes in time and central nervous system location, a diagnosis of multiple sclerosis was made. Herein, we used this patient to illustrate the difficulty in distinguishing acute disseminated encephalomyelitis from multiple sclerosis in patients who present initially with an acute focal demyelinating syndrome.

Brain Stem↗

CD34 immunohistochemical staining of bone marrow biopsies in myelodysplastic syndromes.

Although it has been shown that the percentage of bone marrow blasts in myelodysplastic syndrome (MDS) constitute the only independent determinant of survival and progression to acute leukemia, the great variability in survival among patients with MDS of similar percentage of blasts has prompted us to investigate new objective, independent prognostic parameters for the selection of high-risk patients. It was suggested that CD34 antigen expression adversely affected the prognosis of acute myelogenous leukemia. However, no study has been published so far on clinical and prognostic significance of CD34 antigen expression in MDS. Bone marrow biopsies from 58 patients diagnosed as primary MDS were studied using QBEND/10, a monoclonal antibody which recognized the human progenitor CD34 antigen on routine aldehyde-fixed, paraffin-embedded samples. The high percentage of CD34-positive cells (above 3% of total bone marrow nucleated cells) was predominantly observed in cases with RAEB-T, CMML, and to a lesser degree in RAEB. But neither age, hemograms, bone marrow findings including percentage of blasts, ALIP, nor leukemic transformation correlated with the percentage of CD34-positive cells. The median actuarial survival time in the high positive group was significantly shorter (12.0 months) than that of the low group (30.0 months; p = 0.028). The high CD34 aggregate (> or = 3) was selectively found in cases with RAEB, RAEB-T, and CMML. The percentage of bone marrow blasts (p = 0.007) and ALIP (p = 0.030) significantly correlated with number of CD34 aggregates.(ABSTRACT TRUNCATED AT 250 WORDS)

Adult↗

Differential responses of CD34-positive acute myelogenous leukemic blasts to the costimulating effects of stem cell factor with GM-CSF and/or IL-3.

Stem cell factor (SCF), a c-kit ligand, has a preferential effect on the proliferation of several classes of immature hematopoietic progenitor cells in combination with GM-CSF or IL-3. To analyze the costimulatory role of SCF in leukemic growth, we investigated the effect of SCF in the presence of GM-CSF and/or IL-3 on isolated CD34-positive (CD34+) leukemic blasts from 15 patients with acute myelogenous leukemia (AML). Cultures of CD34+ cells from normal bone marrow were used as controls. When the proliferation of CD34+ AML blasts in the presence of GM-CSF and/or IL-3 were evaluated in vitro for the effects of SCF, two patterns emerged. In one pattern, CD34+ AML blasts responded with a significant increase in DNA synthesis and/or colony formation when SCF was used with GM-CSF and/or IL-3 relative to the growth with SCF alone; This result is consistent with those CD34+ bone marrow cells from normal donors. Six patients (40%) were included in this category. The addition of SCF as a single factor resulted in colony formation in all six of these cases. In the other pattern, nine of the patients (60%) had CD34+ leukemic cells whose growth with SCF plus either GM-CSF, IL-3, or GM-CSF+IL-3, was not significantly different from the growth noted in the presence of SCF alone. Among them seven cases that did not form colonies in response to SCF alone, and one case showing autocrine, background growth were included. In the six cases in which the costimulating effects of SCF were documented, CD34+ c-kit+ blasts comprised 50.5 +/- 18.7% of the CD34+ leukemic blasts-higher than 21.8 +/- 19.4% of cases in which the costimulating effect of SCF was not documented. In the cases showing high c-kit antigen expression (> or = 40%), SCF had a costimulatory effect in 71% (5/7) of the patients. In conclusion, our data indicate that CD34+ leukemic blasts from a good proportion of patients with AML did not respond to the costimulating effects of SCF in the presence of GM-CSF adn/or IL-3, in contrast to those CD34+ bone marrow cells from normal donors. The possible use of SCF for acute leukemia must await further cytogenetic and molecular studies, which should clarify the preferential costimulating role of SCF in normal hematopoiesis.

Antigens, CD↗

Primary lymphoma of the thyroid.

Primary lymphoma presenting in the thyroid gland is uncommon. A review of the Yonsei University Medical Center experience between 1982 and 1994 was performed retrospectively to assess the treatment outcome and prognostic factors. There were four females and one male, and the median age was 65 years. All 5 cases presented with a neck mass. Two of them had co-existing biopsy-proved Hashimoto's thyroiditis and three cases were each in a hypothyroid state. All cases with non-Hodgkin's disease were of intermediate grade. One case was in stage IE and four were in stage IIE. Three cases were treated with surgery alone and two cases with bulky inoperable stage IIB were treated with chemo-radiotherapy. Chemotherapy induced a complete response in one and a partial response in the other with minimal transient toxicity. As the questions regarding justification for extensive surgical intervention increase, combined chemo-radiotherapy can be suggested as an initial treatment even in stage I, and stage II thyroid lymphoma based on prognostic factor evaluation.

Aged↗

New antiepileptic drugs for the treatment of childhood epilepsies.

The Council on Scientific Affairs of the California Medical Association presents the following epitomes of progress in pediatrics. Each item, in the judgment of a panel of knowledgeable physicians, has recently become reasonably firmly established, both as to scientific fact and clinical importance. The items are presented in simple epitome, and an authoritative reference, both to the item itself and to the subject as a whole, is generally given for those who may be unfamiliar with a particular item. The purpose is to assist busy practitioners, students, researchers, and scholars to stay abreast of progress in medicine, whether in their own field of special interest or another. The epitomes included here were selected by the Advisory Panel to the Section on Pediatrics of the California Medical Association, and the summaries were prepared under the direction of Susan B. Conley, MD, and the panel.

Anticonvulsants↗

Unusual MRI findings after status epilepticus due to cat-scratch disease.

A 4-year-old girl with status epilepticus and severe encephalopathy associated with cat-scratch disease is described. She had prolonged seizures lasting more than 2 weeks and required treatment with pentobarbital coma. She developed unusual abnormalities on magnetic resonance imaging scans that involved the thalami and the cortex of the temporal, parietal, and occipital lobes. She has developed a persistent encephalopathy manifesting as visual disturbance and frequent partial seizures.

Brain Damage, Chronic↗

Disseminated multifocal herpes zoster leukoencephalitis and subcortical hemorrhage in an immunosuppressed child.

We describe a rare case of multifocal varicella-zoster leukoencephalitis in an immunosuppressed adolescent boy who developed a herpetiform rash on his groin and subsequently presented with a subacute encephalopathy, aphasia, and hemiparesis. Magnetic resonance imaging scan of the brain revealed multifocal bi-hemispheric target-like lesions predominantly in the white matter. A magnetic resonance imaging scan 2 weeks later showed a subcortical hemorrhage in the left insular region. He received long-term high-dose intravenous acyclovir and had significant improvement in his neurologic status.

Adolescent↗

GM-CSF and low-dose araC treatment of AML in prolonged hypoplasia with residual leukemic cells after induction chemotherapy.

We describe a case with acute myelogenous leukemia (AML; M2) who developed prolonged marrow hypoplasia with residual leukemic blasts and recurrent infections after induction chemotherapy. He was treated successfully with a sequential treatment of recombinant human granulocyte-macrophage colony-stimulating factor (rhGM-CSF) and low-dose cytosine arabinoside (LD AraC). To the best of our knowledge this is the first reported case of a successful treatment of a patient with AML, who showed prolonged markedly hypocellular bone marrow with significant residual leukemic cells after induction chemotherapy, with a sequential treatment of GM-CSF and LD AraC.

Bone Marrow Diseases↗

Electroencephalographic and neuroimaging findings in neonates undergoing extracorporeal membrane oxygenation.

Electroencephalograms (EEGs) were recorded on 36 infants who were treated with arteriovenous extracorporeal membrane oxygenation (ECMO) between 1986 and 1989. Twelve of 36 infants had EEGs prior to and during ECMO. Twenty-one infants who met ECMO criteria but were treated with mechanical ventilation only served as a comparison (COMP) group. Electrographic seizures occurred in 7 of the 36 (19%) ECMO patients and in 3 of the 21 (14%) COMP patients. Five of 7 ECMO infants had electrographic status epilepticus. Three infants developed electrographic seizures during ECMO in association with an acute cerebral injury. These patients did not have significant hemispheric predominance in the origin of electrographic seizures (2 right, 3 left and 2 bilateral). Repetitive or periodic discharges (RPD) with frequencies between 0.5 and 5 Hz were seen after starting therapy in 23 of the 36 (64%) ECMO patients and 15 of the 21 (71%) of the COMP group. Only in the ECMO patients did RPD arise significantly more frequently from the right hemisphere (13 right, 4 left and 6 bilateral; p = 0.015). Neuroimaging studies showed evidence of ischemic lesion in 8 of the 36 (22%) ECMO patients with significantly right-sided predominance (5 right, 1 left and 2 bilateral; p = .05). Cerebellar hemorrhages occurred in 3 ECMO patients. Ischemic lesions occurred in 3 of the 21 (14%) COMP patients, all occurring on the left side. In the ECMO group, severe outcome or death was significantly associated with the presence of electrographic seizures, status epilepticus, and suppression-burst pattern (p < 0.05).(ABSTRACT TRUNCATED AT 250 WORDS)

Brain Injuries↗

Liver involvement in multiple myeloma proven by peritoneoscopy--a case report.

Liver involvement in multiple myeloma has been known to be common in autopsied series. However, since its clinical significance is uncertain yet, invasive procedure confirming plasma cell infiltration of the liver has been rarely performed. We report a case with multiple myeloma which had plasma cell infiltration of a liver. A peritoneoscopic biopsy of the liver for the purpose of disclosing the nature of the aggravating liver function in a carrier of the hepatitis B virus showed infiltration of lymphoreticular cell which were identified later as lambda-light chain producing primitive plasma cells by immunohistochemical stain.

Adult↗

Detection of neonatal seizures through computerized EEG analysis.

Neonatal seizures are a symptom of central nervous system disturbances. Neonatal seizures may be identified by direct clinical observation by the majority of electrographic seizures are clinically silent or subtle. Electrographic seizures in the newborn consist of periodic or rhythmic discharges that are distinctively different from normal background cerebral activity. Utilizing these differences, we have developed a technique to identify electrographic seizure activity. In this study, autocorrelation analysis was used to distinguish seizures from background electrocerebral activity. Autocorrelation data were scored to quantify the periodicity using a newly developed scoring system. This method, Scored Autocorrelation Moment (SAM) analysis, successfully distinguished epochs of EEGs with seizures from those without (N = 117 epochs, 58 with seizure and 59 without). SAM analysis showed a sensitivity of 84% and a specificity of 98%. SAM analysis of EEG may provide a method for monitoring electrographic seizures in high-risk newborns.

Action Potentials↗

Surgical intervention in patients with aplastic anemia.

Eighteen surgical procedures have been performed on 14 cases of aplastic anemia (AA). Of the 10 major surgical procedures, 7 were emergency and 3 elective. The median duration from the diagnosis of AA to major surgery was 0.5 months (3 days-47.3 months), and the median survival after surgery was 12.3 months (4 days-38 months). The hematological status of AA at the time of major surgery were 3 in partial response (PR), 2 with no response (NR) and 5 at diagnosis, respectively; and those after major surgery were 2 with complete response (CR), 2 in PR, 1 with minimal response, and 2 in NR. Three postoperative complications were sepsis, pneumonia and atelectasis encountered in 2 cases. A total of 3 deaths were caused by infection and cancers. Considering the fact that surgery may not only control complications, but offer the opportunity to give effective therapy for AA and therefore improves chances for survival, it is strongly suggested that active surgical intervention should be performed if the patient's status is not terminal.

Adolescent↗

Intestinal neurofibromatosis in von Recklinghausen's disease: presenting as chronic anemia due to recurrent intestinal hemorrhage.

Neurofibromatosis (von Recklinghausen's disease) is a neuroectodermal disorder characterized by pigmentary changes of the skin (café-au-lait spots), cutaneous and visceral tumors (neurofibromas) and systemic abnormalities. The involvement of gastrointestinal tract in neurofibromatosis is not common. The most common symptoms, refer able to lesions in the gut, are hematemesis, melena and abdominal pain. We experienced a case of intestinal neurofibroma in von Recklinghausen's disease. The patient was a 39 year-old female who had suffered from chronic iron deficiency anemia and recurrent gastrointestinal hemorrhage due to two neurofibromas of jejunum for 3 years, which was diagnosed by superior mesenteric and ileal arteriogram and 99mTc pertechnetate-labelled RBC scan, and treated by segmental resection of jejunum with end to end anastomosis.

Adult↗