Life-Threatening Acute on Chronic Pulmonary Thromboembolism Requiring Extracorporeal Membrane Oxygenation

A 71-year-old female patient was admitted to the emergency department with sudden aggravation of chest pain and severe dyspnea. Computed tomography showed extensive pulmonary thromboembolism. Venoarterial extracorporeal membrane oxygenation (ECMO) was instituted due to sudden bradycardia and hypotension. An emergency operation was performed. However, chronic pulmonary thromboembolism combined with an acute pulmonary embolism was detected in the operating room. Embolectomy and endarterectomy were performed. ECMO was then discontinued. The patient was discharged on postoperative day 13 with warfarin for anticoagulation. The patient was followed up for 46 months as an outpatient without further thromboembolic events.

The Antitumor Effect of C-terminus of Hsp70-Interacting Protein via Degradation of c-Met in Small Cell Lung Cancer

BACKGROUND: The mesenchymal-epithelial transition factor (MET) receptor can be overexpressed in solid tumors, including small cell lung cancer (SCLC). However, the molecular mechanism regulating MET stability and turnover in SCLC remains undefined. One potential mechanism of MET regulation involves the C-terminus of Hsp70-interacting protein (CHIP), which targets heat shock protein 90-interacting proteins for ubiquitination and proteasomal degradation. In the present study, we investigated the functional effects of CHIP expression on MET regulation and the control of SCLC cell apoptosis and invasion. METHODS: To evaluate the expression of CHIP and c-Met, which is a protein that in humans is encoded by the MET gene (the MET proto-oncogene), we examined the expression pattern of c-Met and CHIP in SCLC cell lines by western blotting. To investigate whether CHIP overexpression reduced cell proliferation and invasive activity in SCLC cell lines, we transfected cells with CHIP and performed a cell viability assay and cellular apoptosis assays. RESULTS: We found an inverse relationship between the expression of CHIP and MET in SCLC cell lines (n=5). CHIP destabilized the endogenous MET receptor in SCLC cell lines, indicating an essential role for CHIP in the regulation of MET degradation. In addition, CHIP inhibited MET-dependent pathways, and invasion, cell growth, and apoptosis were reduced by CHIP overexpression in SCLC cell lines. CONCLUSION: CHIP is capable of regulating SCLC cell apoptosis and invasion by inhibiting MET-mediated cytoskeletal and cell survival pathways in NCI-H69 cells. CHIP suppresses MET-dependent signaling, and regulates MET-mediated SCLC motility.

Post-traumatic tricuspid regurgitation with anterior papillary muscle rupture, corrected by papillary muscle reimplantation / 고신대학교의과대학학술지

A 60-year-old male patient with blunt chest trauma was transferred to our facility because of unstable vital signs and pericardial effusion. These conditions occurred after orthopedic surgery to repair multiple left finger fractures at a local medical center. Trans-thoracic echocardiography showed severe tricuspid regurgitation and he underwent papillary muscle reimplantation and tricuspid annuloplasty open heart surgery for post-traumatic tricuspid regurgitation with anterior papillary muscle rupture. We report early surgical traumatic valve disease correction without complications.

Expression of the Brother of the Regulator of Imprinted Sites Gene in the Sputum of Patients with Lung Cancer

BACKGROUND: Brother of the regulator of imprinted sites (BORIS) is a putative new oncogene that is classified as a cancer germline gene; however, its role in the development of cancer is unclear. This study investigated the expression of BORIS in lung cancer and its clinical implications. METHODS: The expression of BORIS messenger ribonucleic acid (mRNA) in the sputum of 100 patients with lung cancer (50 with squamous cell carcinoma, 36 with adenocarcinoma, and 14 with small-cell carcinoma) was evaluated by reverse transcription polymerase chain reaction. RESULTS: The overall expression rate of BORIS in patients with lung cancer was 36.0%: 19 of 50 squamous cell carcinomas (38.0%), 13 of 36 adenocarcinomas (36.1%), and 4 of 14 (28.6%) small-cell carcinomas. There was no significant difference in the BORIS expression according to age, gender, or histologic type. However, the mRNA expression of BORIS was significantly related to the pathologic cancer stage (p=0.004) and lymph node metastasis (p=0.001). The expression of the melanoma antigen gene family A1-6 was not associated with the expression of BORIS. CONCLUSION: Our results suggest that the expression of BORIS might be a negative prognostic factor in lung cancers and implicate BORIS as a molecular target for immunotherapy.

Saccular Aneurysm of the External Jugular Vein: A Case Report

Saccular aneurysm of the external jugular vein presenting as a neck mass is very rare. We report the surgical treatment of an external jugular venous aneurysm in a 48-year-old female patient due to the cosmetic problem of neck engorgement, concomitant with thyroidectomy for cancer.

Surgical Removal of Endovascular Stent after Migration to the Right Ventricle Following Right Subclavian Vein Deployment for Treatment of Central Venous Stenosis

Central venous stenosis or occlusion occurs in 11-50% of hemodialysis patients with prior subclavian vein cannulation and ipsilateral fistula or shunt. Most patients are asymptomatic but some require treatment to reduce the risk of thrombosis and improve inadequate hemodialysis pressure. In these cases, endovascular intervention, including ballooning and stenting, is a feasible strategy for selected patents. We report an unusual case of a 40-year-old man on hemodialysis that underwent endovascular stenting to treat right subclavian vein stenosis and experienced stent migration to the right ventricle, requiring surgical removal.

Prevention of Lateral Synechia Formation with Silastic Sheet in the Endoscopic Sinus Surgery / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Lateral synechia formation between the middle turbinate and the lateral nasal wall is one of the most common complications in endoscopic sinus surgery. Unstable and floppy middle turbinate occurs not infrequently and it may make meticulous postoperative dressing difficult during healing period, and consequently increases the risk of synechia and recurrence of sinusitis. The aim of this study was to investigate whether insertion of the silastic sheet in the middle meatus can effectively prevent the synechia formation. SUBJECTS AND METHOD: Twenty eight patients (31 sides including 3 bilateral cases) with unstable and floppy middle turbinate occurred during endoscopic sinus surgery were enrolled in this study. The silastic sheet was designed in fan-shape and inserted between the middle turbinate and the lateral nasal wall. The silastic sheet was secured to the caudal septum and removed 2 to 3 weeks after the operation. We performed a follow-up study for 5 months and investigated the occurrence rate of synechia. RESULTS: The success rate of our method was 90.3% and synechia in the middle meatus developed in 3 of 31 sides (9.7%). Although synechia between the middle turbinate and nasal septum occurred in 4 sides, the patients did not complain of olfactory disturbance. CONCLUSION: The results of this study suggest that insertion of the silastic sheet in the middle meatus can be a useful method in the prevention of lateral synechia formation when unstable and floppy middle turbinate has developed during endoscopic sinus surgery.

The Expression of Vascular Endothelial Growth Factor(VEGF) and Survivin in Squamous Cell Carcinoma of Larynx / 대한이비인후과학회지

BACKGROUND AND OBJECTIVES: Angiogenesis and alteration of apoptosis are hypothesized to be the mechanisms of the growth of tumor. Vascular endothelial growth factor (VEGF) is considered to be the most primary factor prompting the angiogenesis in tumor tissue, which also holds the central position in the course of formation and metastasis of tumor and regulates expression of survivin. The survivin protein is the member of inhibitor of apoptosis protein (IAP) family which inhibits apoptosis. Recently, several authors reported survivin and VEGF expression was found in various cancer tissue and they are suggest to play on the important role in cancer development. The purpose of this study is to evaluate the expression of VEGF and survivin and to investigate their correlation with the clinical stage, nodal involvement, and histologic grade in the squamous cell carcinoma of larynx. SUBJECTS AND METHOD: Immunohistochemical staining for the paraffin sections by using a polyclonal antibody for VEGF and survivin by the standard avidin-biotin-peroxidase technique was performed in 19 cases with the squamouse cell carcinoma of larynx. The relationships between the expression of VEGF and survivin and clinicopathological characteristics were analyzed. RESULTS: Immunohistochemical analysis showed the expression of VEGF in 15 of 19 cases (78.9%) and survivin in 19 of 19 cases (100%). There was no correlation between the expression of VEGF and survivin and the clinical stage, nodal involvement, and histologic grade in the squamous cell carcinoma of larynx (p>0.05). But VEGF results in an increased expression of survivin (p<0.05). CONCLUSION: Further study will be needed to understand the relationships between the VEGF and survivin and squamous cell carcinoma of larynx.

Chimerism Analysis after Allogeneic Stem Cell Transplantation in Children by Genetic Polymorphism of Hypervariable Regions and Fluorescent in situ Hybridization for X-Chromosome / 대한소아혈액종양학회지

PURPOSE: In order to monitor the clinical outcome of pediatric allogeneic stem cell transplantation (SCT), serial evaluations of chimerism were performed to compare the risk of relapse or graft rejection between patients with complete chimerism (CC) and mixed chimerism (MC). METHODS: Between January, 1996 and April, 2004, 64 cases who underwent SCTs were prospectively enrolled. Serial genotyping of VNTR (variable number of tandem repeats) /STR (short tandem repeats) loci and/or X-chromosome-specific FISH (fluorescent in situ hybridization) were performed at regular intervals. RESULTS: The "informative loci" were found in all 64 patient/donor pairs. CC was persistently detected in 44 cases (68.7%), while MC was detected at least once in 20 (31.3%). In cases with malignancy (n=40), relapse was more frequently encountered in MC group (7/8) than in CC group (7/32) (P < .001), as was death (75% vs. 28%, P < .05). The Kaplan-Meier 5-year overall survival was higher in CC than in MC (69.1% vs. 16.6%; P < .05). In cases with non-malignancy, MC group showed higher rate of graft rejection than CC group (7/12 vs. 1/12, P < .01). Survival was not different between the two groups. The chimerism status was not influenced by sex, donor type, source of stem cells, and inclusion of radiation in conditioning. CONCLUSION: Detection and sequential assessment of MC might be an important tool to predict relapse of disease in malignant diseases as well as to portend graft rejection in non-malignant illnesses. Earlier intervention to circumvent those life-threatening complications should be pursued based on the chimerism analyses.

Pulmonary Epithelioid Hemangioendothelioma Association with Subcutaneous Metastasis: Surgical experience of one case / 대한흉부외과학회지

Epithelioid hemangioendothelioma (HE) is a very rare malignant tumor that is pathologically benign tumor originating from endothelial cell but clinically presents metastasis and recurrence. A 29-year-old asymptomatic man, preoperatively diagnosed as lung cancer in the left lower lung, underwent a lobectomy, a wedge lung resection of left upper lung, and partial resection of diaphragm. Left lower lobar lesion was confirmed as pulmonary epithelioid hemangioendothelioma, but the lesions of the left upper lung and diaphragm were remained calcified by spontaneous regression of HE. We report a case of subcutaneous metastasis that occurred two times at 10 months and 19 months after previous surgical treatment of pulmonary EH.

The Role of Gastric Resection for Advanced Gastric Cancer with Peritoneal Seeding

PURPOSE: Peritoneal seeding is the most common type of metastasis and recurrence in gastric cancer. Recently, some studies have reported the benefits of a noncurative gastrectomy for greatly advanced gastric cancer; nevertheless, there are many controversies. This study was performed to investigate the survival benefit of a noncurative gastrectomy for patients with greatly advanced gastric cancer with peritoneal seeding. METHODS: We retrospectively analyzed 286 gastric-cancer patients who had received operations and who had been proven to have peritoneal seeding without liver metastasis or other hematogenous distant metastasis between January 1990 and December 1997 at the Department of General Surgery, College of Medicine, University of Ulsan. RESULTS: The distribution of the degree of peritoneal seeding was P1 in 84 cases (29.4%), P2 in 56 cases (19.6%), and P3 in 146 cases (51.0%). The duration of median follow-up was 9 months (range: 0.4-83.9 months). A noncurative gastrectomy was performed in 121 cases (42.3%); out of them, a total gastrectomy was performed in 49 cases (40.5%), a distal gastrectomy in 70 cases (57.9%), and a proximal gastrectomy in 2 cases (1.6%). A noncurative gastrec tomy was done 51 of the P1 cases (60.7%), 23 of the P2 cases (41.1%), and 47 of the P3 cases (32.2%). D2 lymph-node dissection was performed in 168 cases (87.6%). Postoperative complications developed in 5 cases with a noncurative gastrectomy, and there was no operative mortality. The median survival times were 11.3 months in P1 cases, 10.5 months in P2 cases, and 6.6 months in P3 cases. The median survival times of noncurative gastrectomy, bypass, and expoloratory laparotomy cases were 11.5 months, 6.6 months, and 6.3 months, respectively; according to the degree of peritoneal seeding, they were 14.8 months, 7.1 months, and 5.3 months in P1 cases, 15.3 months, 8.2 months, and 12.5 months in P2 cases, and 7.6 months, 6.4 months, and 5.7 months in P3 cases, respectively. The difference in survival time between the resection and the nonresection groups had statistical significance regardless of the degree ofperitoneal seeding (p<0.05). In the multivariate analysis, the degree of peritoneal seeding (RR: 1.33) and gastric resection (RR: 1.52) were proven to be significant prognostic factors. CONCLUSION: A noncurative gastrectomy might lengthen the survival time in advanced gastric-cancer patients with peritoneal seeding.

The Effect of Intrathecal ACEA 1021, Competitive Glycine-site NMDA Receptor Antagonist on the Hyperalgesia Observed after Thermal Injury in the Rat / 대한마취과학회지

BACKGROUND: Previous reports have described NMDA antagonist reduced the nerve injury induced or inflammatory thermal hyperalgesia. One of the peculiarities of the NMDA receptor is the requirement for glycine as the co-agonist in order to be activated. Thus, the function of NMDA receptors can be modulated by ligands acting as agonists or antagonists at the glycine co-agonist site. ACEA 1021 has been recently characterized as a high potency competitive NMDA receptor/glycine site antagonist. This study evaluated the effects of spinally administered ACEA 1021 on the thermal hyperalgesia state induced by mild burn. METHODS: Rats were prepared with chronic spinal catheters. A thermal injury was induced after briefly anesthetizing with halothane, by applying the left hind paw to a thermal surface (52.5oC) for 45 sec. This exposure results in a mild erythema, but no blistering. Thermal escape latency of the hind paw was determined using an underglass thermal stimulus with which response latencies of the injured and uninjured (normal) paw could be obtained. In this work, ACEA 1021 was injected through intrathecal catheters in rats with mild burn injury on the right hindpaw, after then paw withdrawal latency was measured in both hindpaw every 30 minute for 3 hours. RESULTS: The intrathecal injection of ACEA 1021 (2.4-24 microgram) produced a dose dependent reversal of the hyperalgesia in the right hindpaw, but had no effect upon the response latency of the normal left hind paw even at the largest doses. The effects of intrathecal ACEA 1021 on the hyperalgesia reversed by intrathecal D-serine. CONCLUSIONS: Intrathecal ACEA 1021, competitive-glycine site NMDA receptor antagonist produce a dose-dependent inhibition and D-serine-sensitive reversal of the thermal hyperalgesia evoked mild burn injury. These results suggested that the glycine site of spinal NMDA receptor play an important role in the hyperalgesia induced by mild burn injury.

Report of a Child Donors Liver Transplanted into an Adult Recipient

Under normal circumstances, attempts are made to match the recipient of an orthotopic liver transplant with the best available donor in terms of organ and body size. Occasionally, it may happen that a child's liver is transplanted into an adult recipient, either because of the need for an urgent transplantation when only a small donor is available or because there is no child recipient transplantable with the graft. After a child-donor's liver was transplanted into an adult recipient, we had the opportunity to observe the change in the size of the allograft over time by using serial computed tomography scans to evaluate change in graft size. Reported here are the results of the small-for-size live transplantation at Asan Medical Center.

Bronchiolitis Obliterans and Generalized Vitiligo as Rare Presentations of Chronic Graft-versus-Host Disease / 대한소아혈액종양학회지

Chronic graft-versus-host disease(GVHD), found in 30~50% of long-term survivors after an HLA-identical sibling transplant, in a multi-organ syndrome resembling collagen vascular diseases. Although the skin is the most commonly involved organ, generalized vitiligo with poliosis has rarely been reported as a manifestation. Patient 1 underwent an allogeneic peripheral blood stem cell transplant when late graft failure developed after initial bone marrow transplant(BMT) for aplastic anemia. She suffered from Grade III GVHD involving gut and skin, and developed cough, wheeze and dyspnea along with decreased pulmonary function. The diagnosis of bronchiolitis obliterans in association with chronic GVHD was made, and treatment with steroid and hydroxychloroquine(HCQ) resulted in some improvement. Patient 2 showed de novo chronic GVHD after allogeneic BMT for aplastic anemia. The vitiligo with premature grayness of hair and eyelash was the manifestation of chronic GVHD. Steroid and HCQ treatment stabilized the skin manifestations. HCQ treatment, though a larger study is needed, seemed to be safe adjuvant in the management of chronic GVHD in children.

Prognostic Implication of MDR1 (Multidrug Resistance) Gene Expression in Childhood Acute Leukemia / 대한혈액학회지

BACKGROUND: The most important mechanism of drug resistance is related to the overexpression of P-glycoprotein which is encoded by the MDR1 gene. The expression of MDR1 and its clinical implication in childhood leukemias has not been extensively assessed. METHODS: To evaluate the expression of MDR1 gene and its prognostic implication on the remission induction rate, relapse rate and survival, we assayed MDR1 mRNA by reverse-transcriptase polymerase chain reaction (RT-PCR) from bone marrow samples of 23 childhood ALL and 17 AML patients. The prognostic factors were analyzed by logistic regression and Cox proportional hazards model. RESULTS: mdr1 mRNA was expressed in 51.5% of patients at diagnosis. MDR1 status did not influence the remission induction rate both in ALL and AML. The relapse rate was significantly higher in MDR1 positive patients than in negative patients (29.4% vs. 0.0%, P= 0.04). The cumulative relapse probability at 2 year was 60% vs 0% according to MDR1 status (P=0.006), suggesting the importance of MDR1 in the mechanism of relapse for childhood leukemias. The Kaplan-Meier 3-yr event-free survival (EFS) was 42.7% for MDR1 positive group, and 93.8% for negative patients (P=0.046). Analyses of prognostic factors showed that mdr1 mRNA expression was the sole prognostic factor predicting the poor EFS (X2, 6.217; P=0.013). CONCLUSION: These results suggest that RT-PCR for mdr1 mRNA expression is a readily feasible and useful method of assessing multidrug resistance. The expression of MDR1 was found to be the most important prognostic factor predicting the possibility of relapse and EFS in patients with childhood leukemia.

Prognostic Implication of MDR1 (Multidrug Resistance) Gene Expression in Childhood Acute Leukemia / 대한혈액학회지

BACKGROUND: The most important mechanism of drug resistance is related to the over-expression of P-glycoprotein which is encoded by the MDR1 gene. The expression of MDR1 and its clinical implication in childhood leukemias has not been extensively assessed. METHODS: To evaluate the expression of MDR1 gene and its prognostic implication on the remission induction rate, relapse rate and survival, we assayed mdr1 mRNA by reverse-transcriptase polymerase chain reaction (RT-PCR) from bone marrow samples of 23 childhood ALL and 17 AML patients. The prognostic factors were analyzed by logistic regression and Cox proportional hazards model. RESULTS: mdr1 mRNA was expressed in 51.5% of patients at diagnosis. MDR1 status did not influence the remission induction rate both in ALL and AML. The relapse rate was significantly higher in MDR1 positive patients than in negative patients (29.4% vs. 0.0%, p=0.04). The cumulative relapse probability at 2 year was 60% vs 0% according to MDR1 status (p=0.006), suggesting the importance of MDR1 in the mechanism of relapse for childhood leukemias. The Kaplan-Meier 3-yr event-free survival (EFS) was 42.7% for MDR1 positive group, and 93.8% for negative patients (p=0.046). Analyses of prognostic factors showed that mdr1 mRNA expression was the sole prognostic factor predicting the poor EFS(X 2, 6.217; p=0.013). CONCLUSION: These results suggest that RT-PCR for mdr1 mRNA expression is a readily feasible and useful method of assessing multidrug resistance. The expression of MDR1 was found to be the most important prognostic factor predicting the possibility of relapse and EFS in patients with childhood leukemia.

Dexamethasone Administration and Propofol Anesthesia Prevent Postoperative Nausea and Vomiting / 대한마취과학회지

BACKGROUND: Nausea, vomiting are among the most common postoperative complaints. We compared the effects of dexamethasone with or without propofol to the effects of conventional regimen consisting of enflurane-N2O in the prevention of postoperative nausea and vomiting. METHODS: Eighty healthy children, aged 3~14 yr, undergoing elective tonsillectomy were alldegrees Cated randomly to receive either 67% nitrous oxide and 1.5~2.0% enflurane or 67% nitrous oxide and propofol infusion 6~10 mg/kg/hr for maintenance of anesthesia. Dexamethasone 150 microgram/kg(maximum dose 8mg) or placebo was administered intrvenously(IV) in a double-blinded fashion before surgery. RESULTS: Dexamethasone reduced the overall incidence of vomiting from 45%(control) to 10%, Propofol also reduced from 45% to 15%. Combination of propofol anesthesia and dexamethasone administration reduced from 45% to 10%. CONCLUSIONS: Dexamethasone and propofol markedly decreased vomiting in healthy children after elective tonsillectomy.

High Dose of Amphotericin B in Intralipid Emulsion-based Delivery System in Immunocompromised Children with Invasive Fungal Infections

PURPOSE: Fungal infections are an important cause of morbidity and mortality in patients with hematologic malignancies. The therapy of choice in documented or suspected invasive fungal infections has been intravenous Amphotericin B (AmB). Adverse effects such as fever, chils, thrombophlebitis, nausea or vomiting are common. A more serious adverse effect is potential renal impairment. As AmB administration mixed with Intralipid (AmB/Intralipid) was reported to decrease AmB toxicity without a concomitant loss of antifungal efficacy, we studied the efficacy and side effects of long-term administration of AmB/Intralipid in leukemic children with invasive fungal diseases. METHODS: AmB/Intralipid was administered in seven leukemic children (male, 3; female, 4) who had invasive fungal infections between July 1994 and March 1997. RESULTS: AmB/Intralipid was administered at a mean concentration of 1.45mg/kg/day for a mean of 58.1 days with cumulative dose of 3.01g. Excluding 2 patients who succumbed to the underlying leukemia, 4 out of 5 remaining patients remained free of both fungal infection and leukemia. Chills associated with AmB/Intralipid were found 13 times in 4 patients. One patient could not continue the administration because of the chills on the 45th day of AmB/Intralipid. Renal and hepatic impairment greater than Grade II toxicity was found in each case, respectively. The other 6 patients showed mild elevation from the baseline, but remained within the normal limits. CONCLUSION: Long-term, high-dose AmB/Intralipid therapy can be safely and effectively used in immunocompromised children with invasive fungal infections.

Disseminated Fusarial Infections in Two Children with Acute Leukemia / 대한소아혈액종양학회지

Disseminated Fusarium infection has rarely been encountered in neutropenic cancer patients. The clinical features include fever, positive blood cultures, severe myalgias, disseminated ecthyma gangrenosum-like skin lesions, ocular symptoms and multi-organ-system involvement. The mortality rate reported so far exceeded 70% despite antifungal treatments. We report here the first two cases of disseminated fusarial infections in Korea. The first patient contracted pneumonia during severe neutropenic period following consolidation chemotherapy for acute myelogenous leukemia(AML). Fusarium oxyshrum was cultured from bronchoalveolar lavage fluid. The patient remains free of fungemia after successful antifungal treatment of high-dose amphotericin B and terbinafine. The second patient had relapsed AML and suffered from disseminated fusarial infection involving lungs with characteristic skin lesions. The patient succumbed to refractory leukemia and the fungal infection. A brief review of the literature ensues with the case report.

A Clinical Study of Fanconi's Anemia / 대한소아혈액종양학회지

BACKGROUND: Fanconi's anemia(FA) is an autosomal recessive disease characterized by aplastic anemia and congenital malformations. As up to 30% of patients have no physical stigmata, the modern diagnosis of FA rests on chromosomal breakage of patient's cells induced by chemical clastogens such as diepoxybutane(DEB) or mitomycin-C(MMC). METHODS: We reviewed the clinical manifestations, laboratory findings, diagnostic methods, treatment and outcome of 6 patients diagnosed to have a FA at the Chonnam University Hospital for the last 6 years. RESULTS: Six cases(16.2 %) were found to have FA among 37 aplastic children who were diagnosed during the same period. The mean age at diagnosis was 6.3 years which was the usual onset of hematologic findings. All patients had features of aplastic anemia, and had one or more anomalies, such as low birth weight, hyperpigmentation, cafeau-lait spots, mental retardation, developmental delay, peculiar face(broad nasal bases, epicanthal folds, micrognathia), polydactyly, microcephaly, short stature, and dislocation of hip. We found increased breaks in cultured cells with DEB and MMC in 5 cases tested. The median duration of follow-up was 30 months. Oxymetholone and prednisolone treatment was partially beneficial in three cases. Immunosuppressive treatment with ALG/ATG was not successful in two cases tried. Four cases are living now, without transfusion in three. Two patients were died of disseminated fungal infection and transplant-related problems, respectively. CONCLUSIONS: Fanconi's anemia should be sought carefully in any patients with aplastic anemia because the prognosis, treatment modality, and the approach to bone marrow transplantation are quite different when the hematologic disorder is inherited rather than acquired.