Craniofacial clefts are rare among facial anomalies with an incidence of 1.5 to 5 per 100,000 births, and 1 per 100 cases of cleft lip and palate. The Tessier No. 7 clefts are unusual lesions that result from failure of the embryonic mandibular and maxillary processes to properly fuse and form the corners of the mouth. We experienced a case of Tessier No. 7 craniofacial cleft in a 1 day-old female patient who presented with a macrostomia and auricular malformation. The diagnosis was established by clinical and radiographic findings. A brief review of literature was made.
Cleft Lip ; Diagnosis ; Female ; Humans ; Incidence ; Macrostomia ; Mouth ; Palate ; Parturition

Between 1986 and 1990, four children with recurrent CNS leukemia who had previous CNS prophylaxis therapy were treated with intermittent central nervous system irradiation and intrathecal chemotherapy (IIIC). There was no isolated CNS recurrence. One patient died form bone marrow relapse. Three patients are alive without evidence of disease for 3E3/12 year to 3E6/12 year after the diagnosis of recurrence of CNS leukemia. This experience suggests that IIIC may be an effective treatment for preventing the recurrence of CNS leukemia without any serious side effects.
Bone Marrow ; Central Nervous System* ; Child* ; Diagnosis ; Drug Therapy* ; Humans ; Leukemia* ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Recurrence

We performed this study to evaluate the therapeutic responses of bone marrow transplantation and immunosuppressive therapy for aplastic anemia in 103 cases (68 severe and 35 moderate). Among them, 15 cases with severe aplastic anemia having HLA-identical sibling donor underwent bone marrow transplantation, and cases(53 severe and 35 moderate cases)without available HLA-identical sibling donor had received immunosuppressive therapy using antilymphocye globulin and cyclosporin-A. The results were as follows; 1) For cases given bone marrow transplantation, successful engraftment was accomplished in all cases, but 2 cases showed rejection and 1 case died of thrombotic thrombocytopenic purpura (TTP), resulting 80% disease-free survival rate. acute graft-versus-host disease above grade II was noted in 2 cases and chronic graft-versus host disease was seen in other 2 cases. The most common pathogenic organism in post-transplantation infection was Herpes zoster (6 cases), and one each cases of Herpes simplex, candidiasis, measles and hemorrhagic cystitis developed. 2) Among 88 cases of receiving immunsouppressive therapy, complete response was seen in 26 cases (29.5%) and partial respones in 37 cases (42.0%), resulting 71.6% of overall response rate and recurrence rate was 19.5% (12 cases). In 53 severe cases, complete response was seen in 9 cases (17.0%) and partial response in 25 cases (47.2%), resulting 64.2% of overall response rate. In 35 moderate cases, complete response was seen in 17 cases (48.6%) and partial response in 12 cases(34.3%), overall response rate was 82.9%. All initial responses were seen within 6 months post-treatment period, and the time interval to initial response was 8 to 162 days (median; 53 days). There was no sex difference concerning response rate, however, partial response was predominant in male and more complete response in female. The complications of immunosuppressive therapy were thrombocytopenia (93.2%), fever (67.7%), hypertension (50.0%), skin eruption (45.8%), gingivitis (17.0%). In conclusion, our date suggest that bone marrow transplantation is preferred initial treatment modality to immunosuppressive therapy in severe aplastic anemia patient with HLA-identical sibling donor, and immunosuppression is a satisfactory alternative therapy for children with severe aplastic anemia in the absence of HLA-identical sibling and for whom with moderate aplastic anemia.
Anemia, Aplastic* ; Bone Marrow Transplantation ; Candidiasis ; Child ; Cystitis ; Disease-Free Survival ; Female ; Fever ; Gingivitis ; Graft vs Host Disease ; Herpes Simplex ; Herpes Zoster ; Humans ; Hypertension ; Immunosuppression ; Male ; Measles ; Purpura, Thrombotic Thrombocytopenic ; Recurrence ; Sex Characteristics ; Siblings ; Skin ; Thrombocytopenia ; Tissue Donors

Respiratory syncytial virus (RSV) is the major cause of lower respiratory tract infection in infants. Life-threatening RSV infection is often reported in young children and immunocompromised hosts. Since there is no report on ribavirin therapy for RSV pneumonia in pediatric cancer patients in Korea, we report one case of RSV pneumonia that developed in an infant with acute lymphoblastic leukemia (ALL). Despite administration of oral ribavirin and intravenous immunoglobulin, the patient's respiratory distress worsened and admission to an intensive care unit was necessary. Chest x-ray showed multifocal consolidation, pneumothorax, and pneumomediastinum. Treatment with aerosolized ribavirin led to significant clinical improvement. The role of aerosolized ribavirin is still controversial, but it might have a therapeutic potential for severe RSV pneumonia in children with leukemia.
Child ; Humans ; Immunocompromised Host ; Immunoglobulins ; Infant ; Intensive Care Units ; Korea ; Leukemia ; Mediastinal Emphysema ; Pneumonia ; Pneumothorax ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Respiratory Syncytial Viruses ; Respiratory Tract Infections ; Ribavirin ; Thorax

Paragonimiasis is a parasitic disease found in carnivorous animals which is caused by trematodes of the genus Paragonimus. Human infestation occurs by ingestion of raw or not incompletely cooked freshwater crab or crayfish with the metacercaria. Recently, the prevalence of human paragonimiasis has decreased markedly, however, about one hundred clinical cases have been diagnosed annually by antibody test in Korea. The symptoms, such as chronic cough, hemoptysis, and radiological findings are often confused with those of tuberculosis. Therefore, differential diagnosis between tuberculosis and paragonimiasis is important in Korea which has a high prevalence of tuberculosis. Recently, we experienced a case of pulmonary paragonimiasis in a 13yearold girl with hemoptysis and chest pain. Chest roentgenogram with subsequent tomography demonstrating multiple cavitary lesions and linear tubular densities. Strong positive was noted in intradermal skin test for Paragonimus westermani as well as in IgG antibody detection using ELISA method. Diagnosis was confirmed by stool with multiple P. westermani ova, which was treated with a two day course of praziquantel.
Animals ; Astacoidea ; Chest Pain ; Cough ; Diagnosis ; Diagnosis, Differential ; Eating ; Enzyme-Linked Immunosorbent Assay ; Female ; Fresh Water ; Hemoptysis ; Humans ; Immunoglobulin G ; Korea ; Ovum ; Paragonimiasis* ; Paragonimus ; Paragonimus westermani ; Parasitic Diseases ; Praziquantel ; Prevalence ; Skin Tests ; Thorax ; Tuberculosis

The aim of this study was to investigate the diphtheria-tetanus-pertussis antibody titers after antineoplastic treatment and to suggest an appropriate vaccination approach for pediatric hemato-oncologic patients. A total of 146 children with either malignancy in remission after cessation of therapy or bone marrow failure were recruited. All children had received routine immunization including diphtheria-tetanus-acellular pertussis vaccination before diagnosis of cancer. The serologic immunity to diphtheria, tetanus and pertussis was classified as: completely protective, partially protective, or non-protective. Non-protective serum antibody titer for diphtheria, tetanus and pertussis was detected in 6.2%, 11.6%, and 62.3% of patients, respectively, and partial protective serum antibody titer for diphtheria, tetanus and pertussis was seen in 37%, 28.1%, and 8.9% of patients. There was no significant correlation between the severity of immune defect and age, gender or underlying disease. Revaccination after antineoplastic therapy showed significantly higher levels of antibody for each vaccine antigen. Our data indicates that a large proportion of children lacked protective serum concentrations of antibodies against diphtheria, tetanus, and pertussis. This suggests that reimmunization of these patients is necessary after completion of antineoplastic treatment. Also, prospective studies should be undertaken with the aim of devising a common strategy of revaccination.
Adolescent ; Age Factors ; Antibodies, Bacterial/blood/immunology ; Antineoplastic Agents/therapeutic use ; Child ; Child, Preschool ; Diphtheria/immunology/prevention & control ; Diphtheria-Tetanus-acellular Pertussis Vaccines/*immunology ; Female ; Hematologic Neoplasms/*diagnosis/drug therapy ; Humans ; Immunization, Secondary ; Lymphoma/diagnosis/drug therapy ; Male ; Neuroblastoma/diagnosis/drug therapy ; Sex Factors ; Tetanus/immunology/prevention & control ; Whooping Cough/immunology/prevention & control

PURPOSE: In most cases, myelodysplastic syndrome(MDS) transforms into a more aggressive state or acute myelogenous leukemia; it's prognosis is very poor. It is believed that hematopoietic stem cell transplantation(HSCT) is the only curative treatment of MDS, but available data in children are very sparse. In this report, the short term outcome of HSCT in childhood MDS was analyzed. METHODS: Ten children with MDS(CMMoL 5, RAEB 3, RAEBt 2) underwent HSCT(HLA- matched sibling transplantation 4, HLA-matched unrelated transplantation 4, cord blood transplantation 1, HLA-mismatched familial transplantation 1) between November 1995 and January 2001 at St. Mary's Hospital. Median follow-up duration was 11 months. RESULTS: Engraftment was successful in all cases and 8 patients are alive without disease. Three cases of VOD were observed and improved without complication. Four cases of grade II and 1 case of grade III acute GVHD were observed and well controlled with treatment. Three patients relapsed after transplantation. One patient is alive without disease after cytoreduction with allogenic stem cell rescue and 2 patients died of relapse. CONCLUSION: HSCT is a curative strategy of MDS and the survival rate is relatively higher than that of adults. But there is an obvious need for more studies because of the small number of patients and the short duration of the follow-up.
Adult ; Anemia, Refractory, with Excess of Blasts ; Child ; Fetal Blood ; Follow-Up Studies ; Hematopoietic Stem Cell Transplantation* ; Hematopoietic Stem Cells* ; Humans ; Leukemia, Myeloid, Acute ; Myelodysplastic Syndromes* ; Prognosis ; Recurrence ; Siblings ; Stem Cells ; Survival Rate

Gastrointestinal system involvement is one of the principal complications seen in the recipients of hematopoietic stem cell transplantation (HSCT), and it is also a major cause of morbidity and death in these patients. The major gastrointestinal complications include typhlitis (neutropenic enterocolitis), pseudomembranous enterocolitis, viral enteritis, graft-versus-host disease, benign pneumatosis intestinalis, intestinal thrombotic microangiopathy, and post-transplantation lymphoproliferative disease. As these patients present with nonspecific abdominal symptoms, evaluation with using such imaging modalities as ultrasonography and CT is essential in order to assess the extent of gastrointestinal involvement and to diagnose these complications. We present here a pictorial review of the imaging features and other factors involved in the diagnosis of these gastrointestinal complications in pediatric HSCT recipients.
Child ; *Diagnostic Imaging ; Gastrointestinal Diseases/*diagnosis/*etiology ; Hematopoietic Stem Cell Transplantation/*adverse effects ; Humans

PURPOSE: This study aimed to determine the frequencies of CD4+CD25+ T cells in donor graft and peripheral blood CD4+CD25+ T cells in recipients after hematopoietic stem cell transplantation (HSCT) and their association with graft-versus-host disease (GVHD). METHODS: Seventeen children who underwent HSCT were investigated. CD4+CD25+ T cells in samples from donor grafts and recipient peripheral blood were assessed by flow cytometry at 1 and 3 months after transplantation. RESULTS: CD4+CD25+ T cell frequencies in the grafts showed no significant difference between patients with and without acute GVHD (0.90% vs. 1.06%, P=0.62). Absolute CD4+CD25+ T cell number in grafts were lower in patients with acute GVHD than in those without acute GVHD (6.18x10(5)/kg vs. 25.85x10(5)/kg, P=0.09). Patients without acute GVHD showed a significant decrease in peripheral blood CD4+CD25+ T cell percentage at 3 months compared to those at 1 month after HSCT (2.11% vs. 1.43%, P=0.028). However, in patients with acute GVHD, CD4+CD25+ T cell percentage at 3 months was not different from the corresponding percentage at 1 month after HSCT (2.47% vs. 2.30%, P=0.5). CONCLUSION: The effect of frequencies of CD4+CD25+ T cells in donor grafts on acute GVHD after HSCT could not be identified, and the majority of peripheral blood CD4+CD25+ T cells in patients who underwent HSCT may be activated T cells related to acute GVHD rather than regulatory T cells. Further studies with additional markers for regulatory T cells are needed to validate our results.
Cell Count ; Child ; Flow Cytometry ; Graft vs Host Disease ; Hematopoietic Stem Cell Transplantation ; Hematopoietic Stem Cells ; Humans ; T-Lymphocytes ; T-Lymphocytes, Regulatory ; Tissue Donors ; Transplants

Intracranial aspergillosis is a rare pathologic condition, difficult to treat and often fatal, which generally affects immuosuppressed patients. A case of brain abscess secondary to pulmonary localization in a child with acute lymphoblastic leukemia with a favorable outcome is described. A 4-year-old boy diagnosed with acute lymphoblastic leukemia was induced with vincristine, cyclophosphamide, daunorubicin, L-asparaginase and dexamethasone. On the second week of induction chemotherapy, he suffered febrile neutropenia (absolute neutrophil count below 100). Blood and sputum culture disclosed the presence of Aspergillus fumigatus, and chest X-ray examination showed thin-walled cavitation of infiltrates compatible with aspergilloma. The patient was treated with amphotericin B (1 mg/kg/day) and with G-CSF for neutropenia. Fever subsided a few days later and complete hematologic remission was attained on the sixth hospital week, during which antifungal treatment with amphotericin B was continued. Repeated blood and sputum cultures were sterile. On the fifty-sixth hospital day, the patient suffered from afebrile tonic seizure with right side weakness. CT scan of the brain showed multiple well-circumscribed, rim-enhancing round lesions in right frontal lobe and bilateral parieto-occipital area causing gross edema and displacement of the central structures. Itraconazole was added from eightieth hospital day and supportive care was provided for brain edema. After 2 weeks, there was marked clinical improvement, and the pulmonary aspergilloma had completely regressed on follow-up chest X-ray at the sixty-fifth hospital day. Follow-up brain CT scan at the sixty-eighth hospital day showed marked decrease in size, thickness of abscess wall, and brain edema. Patient also attained neurologic improvement. Amphotericin B therapy was continued for 9 weeks (cumulative dose 908 mg, 58 mg/kg) without discer-nable side effects and the patient was discharged on the ninety-sixth hospital day with improved condition. 6 months after detection of brain abscess, magnetic resonance image of the brain showed resolution of all brain lesions. To the best of our knowledge, this is the first reported case survived CNS aspergillosis in an immunocompromised setting in Korea which was successfully treated with medical therapy only. We present a case report with a brief review.
Abscess ; Amphotericin B ; Aspergillosis* ; Aspergillus fumigatus ; Brain ; Brain Abscess ; Brain Edema ; Child* ; Child, Preschool ; Cyclophosphamide ; Daunorubicin ; Dexamethasone ; Edema ; Febrile Neutropenia ; Fever ; Follow-Up Studies ; Frontal Lobe ; Granulocyte Colony-Stimulating Factor ; Humans ; Induction Chemotherapy ; Itraconazole ; Korea ; Male ; Neutropenia ; Neutrophils ; Precursor Cell Lymphoblastic Leukemia-Lymphoma* ; Seizures ; Sputum ; Thorax ; Tomography, X-Ray Computed ; Vincristine