PURPOSE: Heptaplatin (SKI-2053 R) is a new platinum analogue, with a better toxicity profile than cisplatin, and has antitumor activity even in cisplatin resistant cell lines. 5-fluoruracil (5-FU) has shown synergy with platinum compounds. This phase II trial was designed to determine the efficacy and toxicities of heptaplatin/ 5-FU (5-fluorouracil) for treating stomach cancer. MATERIALS AND METHODS: Thirty-two patients with advanced, measurable gastric adenocarcinomas were enrolled in this trial. The treatment consisted of heptaplatin, 400 mg/m2/day (1 hour IV infusion), on day 1 and 5-FU, 800 mg/m2/day (12 hours IV infusion), on days 1 to 5. The cycles were repeated every 3 weeks. RESULTS: Of the 26 evaluable patients, 9 had partial responses and 1a complete response (overall response rate, 38%; 95% confidence interval, 19~57%). The median response duration was 23 weeks (range: 4~60 weeks). The median time to progression was 26 weeks (range: 3~68 weeks). The grades III-IV toxicities were mostly hematological toxicities: leucopenia was observed in 11 patients (35%) and thrombocytopenia 4 (13%). No definite neuropathy was observed. Grade I-II nephropathy was also noted: grade I high BUN/creatinine levels occurred in 5 patients (16%), grade II proteinuria 2 (6%), grade I proteinuria 5 (16%). Neutropenic fever developed in 5 patients (16%) and 1 died of pneumonia in a neutropenic state. CONCLUSION: This study suggests that the regimen of Heptaplatin/5-FU should be effective and have a favorable toxicity profile for the patients suffering with advanced stomach cancer.
Adenocarcinoma ; Cell Line ; Cisplatin ; Drug Therapy ; Fever ; Fluorouracil ; Humans ; Leucovorin* ; Platinum ; Platinum Compounds ; Pneumonia ; Proteinuria ; Stomach Neoplasms* ; Stomach* ; Thrombocytopenia

PURPOSE: The aim of this study was conducted to investigate the mean nRBC count in very low births weight infants (VLBWIs) and to determine the usefulness of the nRBC as an independent prognostic factors of perinatal complications in VLBWIs. METHODS: This study was conducted on 112 VLBWIs who were hospitalized in the neonatal intensive care unit (NICU) of the author's hospital within the period from March 2003 to and May 2008. Based on the infants' nucleated red blood cells (nRBC) counts at birth, on the third day after birth, on the seventh day after birth, in the second week after birth, and in the fourth week after birth in the medical records, the correlation between nRBC or absolute nRBC counts with birth weight, gestational age, and other perinatal outcomes were retrospectively investigated. RESULTS: In VLBWIs, their mean nRBC and absolute nRBC counts were showing a gradual decrease after birth, and they were consisteantly kept at low values since one week after and inversely proportional to the birth weights. The mean nRBC counts based on the stage after birth showed a significant correlation with perinatal death, necrotizing enterocolitis, and severe intraventricular hemorrhage. CONCLUSION: The increase in the nRBC count showed a significant correlation with having a severe intraventricular hemorrhage, necrotizing enterocolitis, and perinatal death in VLBWIs. If an increase or no decrease in the nRBC count after birth is observed, newborn-infant care precautions should be required.
Birth Weight ; Enterocolitis, Necrotizing ; Erythrocytes ; Gestational Age ; Hemorrhage ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Infant, Very Low Birth Weight ; Intensive Care, Neonatal ; Medical Records ; Parturition ; Prognosis ; Reference Values ; Retrospective Studies

Neurofibromatosis type 1 (von Recklinghausen disease, NF1) involves the central and peripheral nervous systems as well as the skin, bone, endocrine, gastrointestinal and vascular systems. The gastrointestinal neurofibroma associated with NF1 has been infrequently reported. We report our experience with a 15-year-old boy who had a gastric plexiform neurofibroma with upper gastrointestinal bleeding and underwent a tumorectomy because of massive upper gastrointestinal bleeding. We conclude that gastrointestinal bleeding and anemia in the setting of NF1 mandates complete endoscopic examination of the digestive tract to rule out neurofibromas. Surgical resection is the standard treatment.
Adolescent ; Anemia ; Gastrointestinal Tract ; Hemorrhage* ; Humans ; Male ; Neurofibroma* ; Neurofibroma, Plexiform ; Neurofibromatoses ; Neurofibromatosis 1* ; Peripheral Nervous System ; Skin ; Stomach

Crohn disease (CD) is rare, but the incidence of CD has been increasing over the past ten years. We found two cases of CD, associated with myelodysplastic syndrome (MDS), for the first time in children. In the first patient, MDS was diagnosed at three years of age and CD developed later at eight years of age. The patient presented with recurrent abdominal pain, diarrhea, bloody stools and failure to thrive. Colonoscopy revealed cobble stone like mucosa and mass like lesions with superficial ulceration and inflammatory exudates, observed from the cecum to ascending colon. Ileo-cecal biopsy samples showed ulcers with skipped areas and lymphoid infiltrations. The patient was started on treatment with mesalazine and deflazacort, and symptoms remitted. In the second patient, MDS was diagnosed at nine years of age and CD developed at 13 years of age. This patient has recurrent hematochezia, abdominal pain, vomiting and fever. Colonoscopy revealed a large, deep indurative ulceration on the cecal side of the ileo-cecal valve. Ileocecectomy was done, and histology revealed ulceration with transmural inflammation and lymphoid aggregates. Symptoms improved after ileocecectomy.
Abdominal Pain ; Biopsy ; Cecum ; Child* ; Colon, Ascending ; Colonoscopy ; Crohn Disease* ; Diarrhea ; Exudates and Transudates ; Failure to Thrive ; Fever ; Gastrointestinal Hemorrhage ; Humans ; Incidence ; Inflammation ; Mesalamine ; Mucous Membrane ; Myelodysplastic Syndromes* ; Ulcer ; Vomiting

Tufting enteropathy is a rare autosomal recessive disorder presenting with early-onset severe intractable diarrhea. The epithelial cell adhesion molecule gene (EpCAM) has recently been identified as the gene responsible for tufting enteropathy. Based on histology, a diagnosis of tufting enteropathy was made in two Korean siblings. They developed chronic diarrhea and failure to thrive. They had a broad nasal bridge and micrognathia. Duodenal and colonic biopsies showed villous atrophy, disorganization of surface enterocytes, and focal crowding resembling tufts. Protracted diarrhea continued and so cyclic parenteral nutrition was supplied. The sister had juvenile rheumatoid arthritis. Mutation analysis of EpCAM identified two compound heterozygous mutations in these siblings: 1) a donor splicing site mutation in intron 5 (c.491+1G>A) and 2) a novel nonsense mutation in exon 3 (c.316A>T, Lys106X). Analysis of EpCAM will be useful for genetic counseling and prenatal diagnosis of tufting enteropathy.
Antigens, Neoplasm ; Arthritis, Juvenile Rheumatoid ; Atrophy ; Biopsy ; Cell Adhesion Molecules ; Codon, Nonsense ; Colon ; Crowding ; Diarrhea ; Enterocytes ; Epithelial Cells ; Exons ; Failure to Thrive ; Genetic Counseling ; Humans ; Introns ; Parenteral Nutrition ; Prenatal Diagnosis ; Siblings ; Tissue Donors

PURPOSE: Computational flow dynamic (CFD) study has not been widely applied in intracranial artery stenosis due to requirement of high resolution in identifying the small intracranial artery. We described a process in CFD study applied to symptomatic severe intracranial (M1) stenosis before and after stenting. MATERIALS AND METHODS: Reconstructed 3D angiography in STL format was transferred to Magics (Materialise NV, Leuven, Belgium) for smoothing of vessel surface and trimming of branch vessels and to HyperMesh (Altair Engineering Inc., Auckland, New Zealand) for generating tetra volume mesh from triangular surface-meshed 3D angiogram. Computational analysis of blood flow in the blood vessels was performed using the commercial finite element software ADINA Ver 8.5 (ADINA R & D, Inc., Lebanon, MA). The distribution of wall shear stress (WSS), peak velocity and pressure in a patient was analyzed before and after intracranial stenting. RESULTS: Computer simulation of wall shear stress, flow velocity and wall pressure before and after stenting could be demonstrated three dimensionally by video mode according to flow vs. time dimension. Such flow model was well correlated with angiographic finding related to maximum degree of stenosis. Change of WSS, peak velocity and pressure at the severe stenosis was demonstrated before and after stenting. There was no WSS after stenting in case without residual stenosis. CONCLUSION: Our study revealed that CFD analysis before and after intracranial stenting was feasible despite of limited vessel wall dimension and could reveal change of WSS as well as flow velocity and wall pressure.
Angiography ; Arteries ; Atherosclerosis ; Blood Vessels ; Cerebral Arteries ; Characidae ; Computer Simulation ; Constriction, Pathologic ; Glycosaminoglycans ; Humans ; Lebanon ; Magic ; Stents

Glycogen storage disease (GSD) and mucopolysaccharidosis (MPS) are both independently inherited disorders. GSD is a member of a group of genetic disorders involving enzymes responsible for the synthesis and degradation of glycogen. GSD leads to abnormal tissue concentrations of glycogen, primarily in the liver, muscle, or both. MPS is a member of a group of inherited lysosomal storage diseases, which result from a deficiency in specific enzymatic activities and the accumulation of partially degraded acid mucopolysaccharides. A case of a 16-month-old boy who presented with hepatomegaly is reported. The liver was four finger-breadth-palpable. A laboratory study showed slightly increased serum AST and ALT levels. The liver biopsy showed microscopic features compatible with GSD. The liver glycogen content was 9.3% which was increased in comparison with the reference limit, but the glucose-6-phosphatase activity was within the normal limit. These findings suggested GSD other than type I. Bony abnormalities on skeletal radiographs, including an anterior beak and hook-shaped vertebrae, were seen. The mucopolysaccharide concentration in the urine was increased and the plasma iduronate sulfatase activity was low, which fulfilled the diagnosis criteria for Hunter syndrome (MPS type II). To the best of the authors' knowledge, this is the first case of GSD and Hunter syndrome being identified at the same time.
Animals ; Beak ; Biopsy ; Glucose-6-Phosphatase ; Glycogen ; Glycogen Storage Disease ; Glycosaminoglycans ; Hepatomegaly ; Humans ; Iduronate Sulfatase ; Infant ; Liver ; Liver Glycogen ; Lysosomal Storage Diseases ; Mucopolysaccharidoses ; Mucopolysaccharidosis II ; Muscles ; Plasma ; Spine

Colorectal carcinomas are extremely rare in childhood and adolescence; however, the colon is the most common site of a gastrointestinal carcinoma. Mucin secreting adenocarcinomas with signet ring formation is the most common type of colon cancer identified in children. An 11-year-old boy had abdominal pain and weight loss for three months, back pain and left thigh pain for two months, and hematochezia for four days. Colonoscopy showed an annular mass in the sigmoid colon and the histopathology revealed a signet ring cell carcinoma. A metastatic signet ring cell carcinoma was suspected from the findings of the bone scan, and confirmed later by a left scalp mass incisional biopsy and a bone marrow biopsy. We report a case of a metastatic signet ring cell carcinoma of the colon in a child.
Abdominal Pain ; Adenocarcinoma ; Adolescent ; Back Pain ; Biopsy ; Bone Marrow ; Carcinoma, Signet Ring Cell* ; Child* ; Colon* ; Colon, Sigmoid ; Colonic Neoplasms ; Colonoscopy ; Colorectal Neoplasms ; Gastrointestinal Hemorrhage ; Humans ; Male ; Mucins ; Scalp ; Thigh ; Weight Loss