Biomedical subjects
Publications and source records attributed to Makoto Yagi.
Leiomyosarcoma of the urinary bladder in childhood is uncommon. We reported bladder leiomyosarcoma in a 10-year-old boy. He had underwent the extirpation of the tumor and performed adjuvant chemotherapy in combination with radiotherapy according to the Intergroup Rhabdomyosarcoma Study-4 regimens. He had no evidence of recurrence of disease after 3 years.
TS-022, {4-[(1R, 2S, 3R, 5R)-5-Chloro-2-((S)-3-cyclohexyl-3-hydroxyprop-1-ynyl)-3-hydroxycyclopentyl] butylthio} acetic acid monohydrate, inhibits ADP-induced platelet aggregation, an effect significantly antagonized, as in the case of prostaglandin D(2) by the prostanoid DP(1) receptor antagonist (BW A868C). TS-022 is a prostanoid DP(1) receptor agonist, originally developed as a novel anti-pruritic drug for patients with atopic dermatitis. We examined the effects of TS-022 on experimental pruritus, cutaneous barrier disruption, and atopic dermatitis and in in vitro immune function tests. Topically applied TS-022 significantly suppressed scratching in skin-lesioned NC/Nga mice from a concentration of 2.5 nM, and this scratch-suppressive activity was significantly antagonized by BW A868C. Tacrolimus (FK-506) and dexamethasone, used as reference drugs for atopic dermatitis, also exhibited suppressive effects against scratching, but only at concentrations of 125 and 25,000 microM. TS-022 applied topically, once a day for 2 days, significantly accelerated repair of the cutaneous barrier disruption caused by mechanical scratching, from concentrations of 2.5 nM. This acceleration of repair of the disrupted cutaneous barrier by this drug was also significantly antagonized by BW A868C. FK-506 and dexamethasone showed no beneficial effects on the repair of the disrupted cutaneous barrier. Repeated topical application of 2.5 microM of TS-022 and 12.5 microM of FK-506 once a day for 6 weeks significantly improved the skin inflammation scores in the NC/Nga mice. In regard to the effects of TS-022 in vitro, the inhibitory activity of TS-022 against concanavalin A-induced cytokine production by splenocytes was marginal as compared with that of FK-506 or dexamethasone. These results suggest that the beneficial therapeutic effects of TS-022 in NC/Nga mice with atopic dermatitis are mediated by its suppressive effect on scratching and its effect of accelerating repair of the disrupted cutaneous barrier, both effects being attributable to its prostanoid DP(1) receptor agonistic activity.
Lung scintigraphy has been used to evaluate the degree of pulmonary hypoplasia in infants with congenital diaphragmatic hernia (CDH). However, the relationship between lung scintigraphy and long-term outcome of CDH remains unclear. The aim of this study is to determine whether lung scintigraphy correlates with long-term pulmonary morbidity and nutritional status in survivors of CDH. Consecutive 31 survivors of CDH were enrolled in this study. The initial scan was performed at 1-2 months when the patients were ready for discharge and the follow-up scan was performed following an approximately 1-year interval. The regional ventilation and perfusion were evaluated using (133)Xe-inhalation and intravenous (99m)Tc-MAA injection, respectively. The ventilation and perfusion of the ipsilateral lung was expressed as a percentage of that of the contralateral lung. Physical growth at 1 and 2 years, and pulmonary morbidity were reviewed from medical records. The ventilation and perfusion of the ipsilateral lung at the follow-up scan increased significantly from those at the initial scan. Ten patients had pulmonary morbidity. The ventilation and perfusion of the ipsilateral lung was significantly lower in the patients with pulmonary morbidity compared to the patients without pulmonary morbidity. The initial ventilation and perfusion of the ipsilateral lung were strongly correlated with body weight at 1 and 2 years (ventilation: R = 0.503, P < 0.01; perfusion: R = 0.760, P < 0.0001). These results suggest that lung scintigraphy is useful to predict long-term pulmonary morbidity and poor nutritional status in survivors of CDH.
PURPOSE: Children with neurologic and neuromuscular handicaps frequently have various symptoms related to gastroesophageal reflux (GER) disease. The long-term efficacy of antireflux surgery remains controversial in such children with GER. The clinical results of such patients who underwent laparoscopic fundoplication were examined in the current study. METHODS: Between 1997 and 2003, laparoscopic fundoplication was performed in 56 handicapped children (mean age, 6 years), and gastrostomy was performed concurrently in 52. The main symptoms were emesis/hematemesis in 40 and respiratory symptoms, including repeated respiratory infection and distress, in 31. RESULTS: There were no severe postoperative complications or operative mortality. Emesis/hematemesis was controlled adequately in those without recurrence. Respiratory symptoms were controlled unsuccessfully in 16 patients (52%), 8 of whom required further respiratory care including nasal airway tube, tracheostomy, and laryngotracheal separation. Recurrence of GER disease occurred in 10 patients, 7 of whom underwent a second Nissen fundoplication successfully. Thirteen died within the median follow-up period of 14 months. CONCLUSIONS: Laparoscopic fundoplication is effective in controlling emesis/hematemesis, but its efficacy is limited in terms of respiratory problems in handicapped children. Further refinements in diagnostic and treatment strategies are mandatory to improve the quality of life in such patients.
A subset of patients with esophageal atresia (EA) have an associated short esophagus and require an antireflux procedure. The Collis-Nissen procedure, which consists of a combination of Collis gastroplasty and Nissen fundoplication, is considered an option in such conditions. The long-term results of EA patients undergoing this procedure have rarely been reported. The results of the Collis-Nissen procedure were examined in four EA patients with follow-up for an average of 9 years. The Collis-Nissen procedure was conducted concurrently with segmental esophageal resection for an associated congenital esophageal stenosis close to the esophagogastric junction in two postoperative Gross type C patients (7 months, 2 years), as an antireflux operation in a postoperative Gross type A patient with an unreducible sliding hiatal hernia (10 years), and with primary repair of EA in a Gross type A patient with a long gap (4 months). There were no significant complications except minor anastomotic leakage and an anastomotic stenosis that required postoperative dilation. Significant reflux was negative, as shown by an upper gastrointestinal study and scintigraphy. Limited esophagitis just above the esophagogastric junction was observed in a Gross type A patient with a long mediastinal gastric segment. Swallow-related pressure reduction at the wrapping cuff was detected by manometric examination. The Collis-Nissen procedure is a useful option for treating EA patients who require esophageal lengthening and control of gastroesophageal reflux.
BACKGROUND/PURPOSE: The outcome of antenatally diagnosed congenital diaphragmatic hernia (CDH) has remained poor despite aggressive therapeutic strategies. Since 1996, the authors have used a new approach including early surgery and inhaled nitric oxide (iNO). The aim of this study is to determine whether early surgery in combination with iNO improves the clinical outcome of antenatally diagnosed CDH. METHODS: From 1988, 40 consecutive neonates with antenatally diagnosed CDH were admitted to the authors' hospital. Ten cases of fatal chromosomal anomalies or major cardiac anomalies were excluded from this study. From 1988 through 1995 (period 1: n = 13), delayed surgery was used in high-risk CDH. From 1996 through 2000 (period 2: n = 17), early surgery in combination with iNO was used. The severity of lung hypoplasia was evaluated using the fetal lung/thorax transverse area ratio (L/T). High-frequency oscillatory ventilation (HFOV) was used routinely during the study periods, and extracorporeal membrane oxygenation (ECMO) was used on basis of conventional entry criteria. The authors compared the clinical outcome, use of ECMO, and the L/T between the 2 periods retrospectively. RESULTS: Patients in the 2 periods were comparable in terms of birth weight, gestational age, and the L/T. The mean age at surgery was 3.1 +/- 4.9 days in period 1, and 0.8 +/- 1.1 days in period 2. Fewer infants in period 2 compared with period 1 were treated with ECMO (period 1, 62% v period 2, 6%; P <.01). There was significant difference in the survival rate between the 2 periods (period 1, 38% v period 2, 94%; P <.01). CONCLUSION: Our data suggest that early surgery and iNO improves the outcome and reduces the requirement of ECMO in the treatment of antenatally diagnosed CDH.
BACKGROUND/PURPOSE: There are few follow-up studies comparing posterior sagittal anorectoplasty (PSARP) with conventional procedures for patients with anorectal malformations (ARM). The authors have examined retrospectively postoperative anorectal function of patients with ARM treated with PSARP compared with those treated with conventional methods. METHODS: Anorectal function in 23 patients with high and intermediate type anorectal malformations (PSARP group), who underwent PSARP more than 4 years previously, were assessed by Kelly's clinical scoring system and objective studies. These results were compared with those in 14 cases (5 high and 9 intermediate type cases; control group), who underwent other conventional surgical procedures. RESULTS: Using Kelly's clinical scoring system, scores of the PSARP group compared with the control group were good in 48% versus 21%, fair in 48% versus 58%, and poor in 4% versus 21%, respectively. Barium enema studies suggested better anorectal sphincteric function in patients with high anorectal malformation in the PSARP group. Magnetic resonance imaging (MRI) studies showed more correct placement of the rectum through the striated muscle complex in the PSARP group at the I-line level. Manometric studies showed no difference in maximum resting pressure, anal canal length, and the incidence of anorectal reflex between the two groups. CONCLUSIONS: The favorable results of MRI and barium enema studies can be explained by direct visualization of the striated muscle complex with the aid of electrical stimulation as well as no harmful effects of amputation of the sphincter muscle in PSARP. However, manometric studies suggest anorectal function in patients with high and intermediate anorectal malformations is limited even after PSARP. Long-term postoperative follow-up with adequate bowel management is required for all patients with high or intermediate anorectal malformation.