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1.
Gastric Cancer ; 2024 Jul 19.
Artículo en Inglés | MEDLINE | ID: mdl-39028419

RESUMEN

BACKGROUND: The significance of reinforcement of the duodenal stump with seromuscular sutures and the effectiveness of reinforced staplers in preventing duodenal stump leakage remain unclear. We aimed to explore the importance of duodenal stump reinforcement and determine the optimal reinforcement method for preventing duodenal stump leakage. METHODS: This retrospective cohort study was conducted between January 1, 2012 and December 31, 2021, with data analyzed between December 1, 2022 and September 30, 2023. This multicenter study across 57 institutes in Japan included 16,475 patients with gastric cancer who underwent radical gastrectomies. Elective open or minimally invasive (laparoscopic or robotic) gastrectomy was performed in patients with gastric cancer. RESULTS: Duodenal stump leakage occurred in 153 (0.93%) of 16,475 patients. The proportions of males, patients aged ≥ 75 years, and ≥ pN1 were higher in patients with duodenal stump leakage than in those without duodenal stump leakage. The incidence of duodenal stump leakage was significantly lower in the group treated with reinforcement by seromuscular sutures or using reinforced stapler than in the group without reinforcement (0.72% vs. 1.19%, p = 0.002). Duodenal stump leakage incidence was also significantly lower in high-volume institutions than in low-volume institutions (0.70% vs. 1.65%, p = 0.047). The rate of duodenal stump leakage-related mortality was 7.8% (12/153). In the multivariate analysis, preoperative asthma and duodenal invasion were identified as independent preoperative risk factors for duodenal stump leakage-related mortality. CONCLUSIONS: The duodenal stump should be reinforced to prevent duodenal stump leakage after radical gastrectomy in patients with gastric cancer.

2.
J Pediatr Gastroenterol Nutr ; 79(1): 48-53, 2024 Jul.
Artículo en Inglés | MEDLINE | ID: mdl-38356291

RESUMEN

Neonatal immune regulation transitions from fetal immunity and varies with maturation status, but its role in neonatal cow's milk protein allergy (CMPA) remains unknown. We studied the association between maturation status at birth and neonatal CMPA. Clinical and laboratory data of neonates presenting with CMPA symptoms were retrospectively collected from two tertiary hospitals. Patients were assessed according to gestational age at birth: preterm, late-preterm, and full-term. Fifty-five infants (26 females, 14 preterm, 15 late-preterm, and 26 full-term) were included; 44 were negative for milk-specific immunoglobulin E. Neonatal CMPA was common during moderately premature periods. Preterm infants exhibited longer latency from initial CM exposure to disease onset, lower incidence of bloody stool, and absence of elevated monocyte counts. However, immunoreactivity to CM antigens was retained in all infants. Neonatal CMPA features varied with infant maturation status at birth. Our results improve the understanding of intestinal immunity development, fetal/neonatal immune regulation, and CMPA pathogenesis.


Asunto(s)
Recien Nacido Prematuro , Hipersensibilidad a la Leche , Proteínas de la Leche , Estudios Retrospectivos , Hipersensibilidad a la Leche/inmunología , Humanos , Femenino , Recién Nacido , Masculino , Proteínas de la Leche/inmunología , Proteínas de la Leche/efectos adversos , Recien Nacido Prematuro/inmunología , Edad Gestacional , Inmunoglobulina E/sangre , Inmunoglobulina E/inmunología , Animales , Bovinos
3.
J Pediatr Hematol Oncol ; 45(2): 95-98, 2023 03 01.
Artículo en Inglés | MEDLINE | ID: mdl-35700350

RESUMEN

Multifocal lymphangioendotheliomatosis with thrombocytopenia (MLT) is a rare disease characterized by the presence of multiple cutaneous lesions and bleeding from the gastrointestinal tract with thrombocytopenia. Because of the varied phenotypes and rarity of MLT, a treatment strategy has not been standardized thus far. We describe a case of infantile MLT that did not respond to treatment with propranolol, prednisolone, or vincristine. We successfully treated the patient with everolimus, an inhibitor of the mammalian target of rapamycin. Our case provides the first evidence of the effectiveness of everolimus for the treatment of MLT.


Asunto(s)
Neoplasias Cutáneas , Trombocitopenia , Humanos , Everolimus , Piel/patología , Trombocitopenia/patología , Neoplasias Cutáneas/patología , Sirolimus
4.
Eur J Pediatr ; 182(4): 1555-1559, 2023 Apr.
Artículo en Inglés | MEDLINE | ID: mdl-36719478

RESUMEN

To investigate the development of diaphragmatic dysfunction in ventilated extremely preterm infants (EPI) using diaphragm ultrasound (DU). EPI of less than 28 weeks' gestational age who required mechanical ventilation within six hours of birth were included in this prospective, observational study. DU was performed once a day until four days of life. End-inspiratory and end-expiratory thicknesses of the diaphragm were measured, and the diaphragm thickening fraction was calculated. A total of 20 EPI were enrolled. After intubation, there was a progressive reduction in end-inspiratory thickness of the diaphragm from baseline to day 1 (P < 0.001), but not from day 1 to day 2 (P = 0.092), day 2 to day 3 (P = 1.0), or day 3 to day 4 (P = 1.0). There was also a significant reduction in the diaphragm thickening fraction from baseline to day 1 (P < 0.001), but not from day 1 to day 2 (P = 1.0), day 2 to day 3 (P = 1.0), or day 3 to day 4 (P = 1.0).    Conclusions: This study provides the first evidence of diaphragmatic dysfunction in ventilated EPI. We demonstrated a rapid progression of ventilator-induced diaphragmatic dysfunction, with a significant reduction in diaphragm thickness and thickening fraction within 24 h of ventilation. What is Known: • Over-assistance of the ventilator suppresses respiratory effort and induces diaphragm unloading, resulting in diaphragm atrophy or dysfunction. • Diaphragmatic dysfunction contributes to prolonged ventilator dependence and poor clinical outcomes. What is New: • Most extremely preterm infants develop diaphragmatic dysfunction after intubation within 24 hours. • Diaphragm thickness and contraction ability measured by ultrasound would be important indicators of worsening breathing or respiratory outcomes.


Asunto(s)
Diafragma , Recien Nacido Extremadamente Prematuro , Recién Nacido , Lactante , Humanos , Diafragma/diagnóstico por imagen , Estudios Prospectivos , Respiración Artificial/efectos adversos , Ventiladores Mecánicos
5.
Pediatr Int ; 64(1): e15006, 2022 Jan.
Artículo en Inglés | MEDLINE | ID: mdl-34585804

RESUMEN

BACKGROUND: The aim of this study was to explore and clarify the healthcare service utilization of children dependent on medical technology (CMT), and the parental health-related quality of life (HRQOL). METHODS: Participants recruited the primary caregivers of children with medical complexity (CMC), identified using an existing registry of raising CMC aged 1-20 years and receiving reimbursements for their home-based medical care at a children's hospital. We used an exploratory cross-sectional study design and sent questionnaires to 286 parents. To clarify the characteristics of CMT and families, we compared two groups on demographics of children's and families', service utilization and parental HRQOL. The participants were categorized into the CMT and children with chronic conditions (CCC) groups, based on whether they required ventilator, suctioning, or tube feeding; had tracheostomy, or gastrostomy; or had central line or clean intermittent catheterization. RESULTS: Of the 95 children selected, 31 were CMT. The participants' characteristics, service utilization, and the parents' HRQOL were compared between the two groups. Compared to CCC, CMT were more likely to be younger, more unstable, more demanding of their caregiver's time, caused greater reductions in their caregiver's sleep time, caused heavier caregiver burden, had higher rates of unexpected hospital admissions and emergency visits, required greater care coordination, and exhibited a higher health service utilization. Parents of CMT were found to have poor mental and physical health in HRQOL. CONCLUSIONS: Improving the outcomes of parents caring for CMT requires high quality healthcare services, especially respite care for the parents, and care coordination.


Asunto(s)
Padres , Calidad de Vida , Cuidadores , Niño , Enfermedad Crónica , Estudios Transversales , Humanos , Japón/epidemiología , Aceptación de la Atención de Salud , Tecnología
6.
Am J Perinatol ; 39(11): 1229-1235, 2022 08.
Artículo en Inglés | MEDLINE | ID: mdl-33374021

RESUMEN

OBJECTIVE: This study aimed to investigate the utility of lung ultrasound (LUS) with whole chest scanning for predicting respiratory outcomes in patients with bronchopulmonary dysplasia (BPD). STUDY DESIGN: We performed a prospective observational study. Preterm infants of less than 32 weeks' gestational age requiring oxygen therapy at 28 days of life were included. LUS was performed on day 28, at 36 weeks' postmenstrual age, and at the time of discharge. Each lung was divided into three regions by the anterior and posterior axillary lines and received an LUS score of 0 to 3 points; the total score was obtained by adding the six regional scores. The classification of BPD was determined based on the National Institute of Child and Human Development. The outcomes of this study were the development of moderate-to-severe BPD and the need for home oxygen therapy (HOT). RESULTS: We enrolled 87 patients; 39, 33, and 15 infants had mild, moderate, and severe BPD, respectively. The LUS score correlated with BPD severity and exhibited an improvement trend with time toward the point of discharge. LUS at 28 days of life predicted moderate-to-severe BPD with an area under the curve of 0.95 (95% confidence interval: 0.91-0.99) and HOT with an area under the curve of 0.95 (95% confidence interval: 0.81-1.0). CONCLUSION: LUS with whole chest scanning is useful for predicting respiratory outcomes in patients with BPD, as well as for understanding BPD severity or clinical improvement trends. KEY POINTS: · LUS predicts respiratory outcomes in patients with BPD.. · LUS indicates BPD severity.. · LUS can show clinical improvement with time..


Asunto(s)
Displasia Broncopulmonar , Niño , Edad Gestacional , Humanos , Lactante , Recién Nacido , Recien Nacido Prematuro , Pulmón , Oxígeno , Ultrasonografía
7.
Pediatr Int ; 63(2): 172-176, 2021 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-33012035

RESUMEN

BACKGROUND: In Japan, some cases of late-onset acute hemolysis in very low birthweight (VLBW) infants have been reported. These cases had common features but the cause of hemolysis was unknown. The incidence and prognosis of this disease are also unknown. However, there are only few reports of such hemolytic episodes in countries other than Japan. Thus, this study aimed to examine the incidence and clinical course of late-onset acute hemolysis and to establish it as a new disease concept. METHODS: A nationwide prospective survey was conducted from 2011 to 2015 as a rare disease surveillance project of the Japan Society for Neonatal Health and Development. RESULTS: Twenty-four cases were confirmed. The median (range) gestational age, birthweight, and onset of hemolytic episodes were 26 weeks and 2 days (23 weeks and 4 days-31 weeks and 2 days), 898 g (627-1,416 g), and 19 days after birth (9-33 days), respectively. Phototherapy, blood transfusion, and exchange transfusion were required in 22 (96%), 24 (100%), and 7 (29%) cases, respectively. During the observation period, no recurrence of the hemolytic episode occurred. All patients survived; however, one case developed kernicterus and suffered severe neurological sequelae. CONCLUSIONS: In this study, at least 1 out of 1,259 VLBW infants developed hemolysis at 9-33 days after birth in Japan. Owing to the risk of kernicterus, this disease should be recognized as among the important pathological conditions of VLBW infants, suggesting the need to manage jaundice and anemia until 5 weeks after birth.


Asunto(s)
Ictericia Neonatal , Kernicterus , Hemólisis , Humanos , Lactante , Recién Nacido , Recién Nacido de muy Bajo Peso , Estudios Prospectivos
8.
Pediatr Int ; 62(8): 932-936, 2020 Aug.
Artículo en Inglés | MEDLINE | ID: mdl-32239786

RESUMEN

BACKGROUND: Appropriate management of the endotracheal tube (ETT) insertion depth is important. The depth calculated using Tochen's formula is overestimated in extremely-low- birthweight infants, particularly those with a birthweight <750 g. Gestational age has been shown to be particularly useful in the Neonatal Resuscitation Program, 7th edition.5 However, a randomized trial for estimating the ETT insertion depth failed to show the advantage of using gestational age over birthweight.6 Therefore, we aimed to estimate the appropriate ETT insertion depth in neonates weighing <750 g. METHODS: This was a single-center, retrospective observational study including neonates weighing <750 g who required intubation. The appropriate depth was determined by adjusting the distance between the actual ETT position and the area from the first to the second thoracic vertebra on the radiograph. Correlations between gestational age and physique were investigated using Pearson's correlation coefficient. We examined small-for- gestational-age (SGA) infants and non-SGA infants separately. RESULTS: Forty neonates were enrolled in this study. The mean gestational age and birthweight were 26.3 weeks and 620 g respectively. Twenty infants were SGA. The ETT position was deep in 35 of 40 cases, with the strongest correlation between weight and ETT insertion depth. The correlation with gestational age was not observed in this study. CONCLUSIONS: Our study showed that the ideal ETT insertion depth at birth correlates with birthweight in neonates weighing <750 g. Therefore, determination by gestational age may not be feasible in populations with a high proportion of SGA infants.


Asunto(s)
Recien Nacido con Peso al Nacer Extremadamente Bajo , Intubación Intratraqueal/métodos , Antropometría/métodos , Peso al Nacer , Edad Gestacional , Humanos , Recién Nacido , Recién Nacido Pequeño para la Edad Gestacional , Unidades de Cuidado Intensivo Neonatal , Radiografía/métodos , Resucitación/métodos , Estudios Retrospectivos , Tráquea/diagnóstico por imagen
9.
Pediatr Int ; 62(2): 175-179, 2020 Feb.
Artículo en Inglés | MEDLINE | ID: mdl-31785124

RESUMEN

BACKGROUND: The purpose of this study was to investigate the usefulness of ultrasonography (US) for confirmation of endotracheal tube (ETT) placement during resuscitation in extremely low birthweight (ELBW) infants. METHODS: We conducted a retrospective review of the medical records of ELBW infants in whom ETT position was verified using US between June 2016 and September 2017. We investigated the backgrounds of the patients and US investigators, and the time required for the detection of exhaled carbon dioxide using the colorimetric method and US. RESULTS: Eleven ELBW infants were evaluated using US by four neonatologists. The median duration required to determine the ETT position by the colorimetric method and US were 11 s and 3 s, respectively. In six ELBW infants, we were able to verify the ETT position more rapidly using US than using the colorimetric method, and were able to perform prompt resuscitation. Unnecessary reintubations were avoided in three ELBW infants. CONCLUSION: Ultrasonography allowed the swift confirmation of the tracheal intubations. The colorimetric method yielded false negative results; in such cases, unnecessary reintubation could have been avoided if US was used. We assessed the mechanism of false negative results and performed appropriate resuscitation.


Asunto(s)
Recien Nacido con Peso al Nacer Extremadamente Bajo , Intubación Intratraqueal/métodos , Ultrasonografía Intervencional , Dióxido de Carbono/fisiología , Colorimetría , Humanos , Recién Nacido , Resucitación , Estudios Retrospectivos
10.
Child Care Health Dev ; 46(4): 436-444, 2020 07.
Artículo en Inglés | MEDLINE | ID: mdl-32246855

RESUMEN

BACKGROUND: Little is known about the provision of care coordination to children with medical complexity (CMC) and their families in Japan. The aim of this study was to describe provision of care coordination and explore the factors associated with quality of care coordination for Japanese CMC and their families. METHODS: We used an exploratory cross-sectional study design. Participants were recruited at a children's hospital located in one prefecture, Japan. Primary caregivers raising children aged between <1 and 20 years and receiving reimbursements for their home-based medical care at a children's hospital were eligible to participants in this study. The study examined the relationship between parents' ratings of care coordination as 'adequate,' 'inadequate' or 'not received' and characteristics of children, parents, and families. RESULTS: Ninety-nine parents caring for CMC were included in the analysis. Of those, 22.2% reported their child had a care coordinator. Caregivers were divided into three groups depending on the quality of care coordination: Group 1 reported adequate care coordination; Group 2 reported inadequate care coordination; and Group 3 had no need for care coordination. We compared the socio-demographics of children, parents and families, their service use and the health-related quality of life (HR-QOL) and caregiver burden scores of parents across the three groups. The parents' free time, access to timely care for children and service satisfaction were positively associated with adequate care coordination for CMC and their families. CONCLUSIONS: Providing adequate care coordination for CMC and their families is essential for access to timely services and more positive psychological health of parents. High quality care coordination for CMC and their families is urgently needed in Japan.


Asunto(s)
Cuidadores/psicología , Continuidad de la Atención al Paciente/organización & administración , Accesibilidad a los Servicios de Salud/organización & administración , Necesidades y Demandas de Servicios de Salud/organización & administración , Afecciones Crónicas Múltiples/terapia , Calidad de la Atención de Salud/organización & administración , Adolescente , Adulto , Niño , Preescolar , Estudios Transversales , Femenino , Servicios de Atención de Salud a Domicilio/organización & administración , Humanos , Lactante , Recién Nacido , Japón , Masculino , Persona de Mediana Edad , Factores Socioeconómicos , Adulto Joven
11.
Am J Med Genet A ; 179(3): 341-343, 2019 03.
Artículo en Inglés | MEDLINE | ID: mdl-30588754

RESUMEN

Schuurs-Hoeijmakers syndrome is a rare disease characterized by intellectual disability and dysmorphic facial features among various physical abnormalities due to PACS1 mutation. To date, 28 patients with a recurrent de novo PACS1 mutation (c.607C > T) have been reported, primarily in Western populations. Here, we describe two Japanese patients with Schuurs-Hoeijmakers syndrome with a recurrent PACS1 mutation. In addition to the typical clinical symptoms, each patient presented novel clinical phenotypes. One patient presented with involuntary movements and was treated with trihexyphenidyl hydrochloride. We hypothesized that the PACS1 mutation leads to an inherent dopaminergic insufficiency that underlies the developing symptoms along with the neurodevelopmental processes. The second patient was diagnosed with lipomyelomeningocele during an examination for severe constipation at the age of 2 years and 8 months. The diagnosis of lipomyelomeningocele in this patient was delayed due to the lack of cutaneous lesions. As the majority of patients with PACS1 mutation present constipation, underdiagnosis of lipomyelomeningocele is a possibility. As the phenotypic expansion of the patients with Schuurs-Hoeijmakers syndrome was not fully recognized, additional studies are needed to clarify the clinical spectrum.


Asunto(s)
Anomalías Múltiples/diagnóstico , Anomalías Múltiples/genética , Anomalías Craneofaciales/diagnóstico , Anomalías Craneofaciales/genética , Discapacidad Intelectual/diagnóstico , Discapacidad Intelectual/genética , Atrofia Muscular/diagnóstico , Atrofia Muscular/genética , Mutación , Fenotipo , Proteínas de Transporte Vesicular/genética , Encéfalo/anomalías , Encéfalo/diagnóstico por imagen , Preescolar , Electroencefalografía , Facies , Femenino , Humanos , Imagen por Resonancia Magnética , Síndrome
12.
Pediatr Diabetes ; 19(2): 243-250, 2018 03.
Artículo en Inglés | MEDLINE | ID: mdl-28597946

RESUMEN

BACKGROUND: Mutations in causative genes for neonatal diabetes or maturity-onset diabetes of the young have been identified in multiple patients with autoantibody-negative type 1 diabetes (T1D). OBJECTIVES: We aimed to clarify the prevalence and phenotypic characteristics of monogenic abnormalities among 89 children with autoantibody-negative insulin-requiring T1D. METHODS: Mutations in 30 genes were screened using next-generation sequencing, and copy-number alterations of 4 major causative genes were examined using multiplex-ligation-dependent probe amplification. We compared the clinical characteristics between mutation carriers and non-carriers. RESULTS: We identified 11 probable pathogenic substitutions (6 in INS , 2 in HNF1A , 2 in HNF4A , and 1 in HNF1B ) in 11 cases, but no copy-number abnormalities. Only 2 mutation carriers had affected parents. De novo occurrence was confirmed for 3 mutations. The non-carrier group, but not the carrier group, was enriched with susceptible HLA alleles. Mutation carriers exhibited comparable phenotypes to those of non-carriers, except for a relatively normal body mass index (BMI) at diagnosis. CONCLUSIONS: This study demonstrated significant genetic overlap between autoantibody-negative T1D and monogenic diabetes. Mutations in INS and HNF genes, but not those in GCK and other monogenic diabetes genes, likely play critical roles in children with insulin-requiring T1D. This study also suggests the relatively high de novo rates of INS and HNF mutations, and the etiological link between autoimmune abnormalities and T1D in the non-carrier group. Carriers of monogenic mutations show non-specific phenotypes among all T1D cases, although they are more likely to have a normal BMI at diagnosis than non-carriers.


Asunto(s)
Diabetes Mellitus Tipo 1/genética , Predisposición Genética a la Enfermedad , Factor Nuclear 1-alfa del Hepatocito/genética , Factor Nuclear 1-beta del Hepatocito/genética , Factor Nuclear 4 del Hepatocito/genética , Insulina/genética , Mutación , Niño , Preescolar , Estudios de Cohortes , Variaciones en el Número de Copia de ADN , Análisis Mutacional de ADN , Diabetes Mellitus Tipo 1/tratamiento farmacológico , Diabetes Mellitus Tipo 1/fisiopatología , Femenino , Estudios de Asociación Genética , Pruebas Genéticas , Factor Nuclear 1-alfa del Hepatocito/química , Factor Nuclear 1-beta del Hepatocito/química , Factor Nuclear 4 del Hepatocito/química , Heterocigoto , Humanos , Hipoglucemiantes/uso terapéutico , Insulina/química , Insulina/uso terapéutico , Japón , Masculino
13.
BMC Cancer ; 16: 354, 2016 06 06.
Artículo en Inglés | MEDLINE | ID: mdl-27268079

RESUMEN

BACKGROUND: It has been shown that epidermal growth factor receptor (EGFR) mutation status is associated with 5-fluorouracil (5-FU) sensitivity in non-small-cell lung cancer (NSCLC). However, the relationship between EGFR mutation status and dihydropyrimidine dehydrogenase (DPD), a 5-FU degrading enzyme, is unknown. METHODS: We elucidated the crosstalk among the EGFR signal cascade, the DPD gene (DPYD), and DPD protein expression via the transcription factor Sp1 and the effect of EGFR mutation status on the crosstalk. RESULTS: In the PC9 (exon19 E746-A750) study, EGF treatment induced up-regulation of both Sp1 and DPD; gefitinib, an EGFR-tyrosine kinase inhibitor (EGFR-TKI), and mithramycin A, a specific Sp-1 inhibitor, suppressed them. Among EGFR-mutated (PC9, HCC827; exon19 E746-A750 and H1975; exon21 L858R, T790M, gefitinib resistant) and -non-mutated (H1437, H1299) cell lines, EGF administration increased DPYD mRNA expression only in mutated cells (p < 0.05). Accordingly, gefitinib inhibited DPD protein expression only in PC9 and HCC827 cells, and mithramycin A inhibited it in EGFR-mutated cell lines, but not in wild-type. FU treatment decreased the level of cell viability more in gefitinib-treated EGFR-TKI sensitive cell lines. Further, combination treatment of FU and mithramycin A suppressed cell viability even in a gefitinib resistant cell line. CONCLUSIONS: The EGFR signal cascade regulates DPD expression via Sp1 in EGFR mutant cells. These results might be a step towards new therapies targeting Sp1 and DPD in NSCLC with different EGFR mutant status.


Asunto(s)
Carcinoma de Pulmón de Células no Pequeñas/genética , Dihidrouracilo Deshidrogenasa (NADP)/genética , Dihidrouracilo Deshidrogenasa (NADP)/metabolismo , Receptores ErbB/genética , Neoplasias Pulmonares/genética , Factor de Transcripción Sp1/metabolismo , Carcinoma de Pulmón de Células no Pequeñas/metabolismo , Línea Celular Tumoral , Supervivencia Celular/efectos de los fármacos , Resistencia a Antineoplásicos/efectos de los fármacos , Sinergismo Farmacológico , Factor de Crecimiento Epidérmico/farmacología , Fluorouracilo/farmacología , Gefitinib , Regulación Neoplásica de la Expresión Génica/efectos de los fármacos , Humanos , Mutación , Plicamicina/análogos & derivados , Plicamicina/farmacología , Quinazolinas/farmacología , Transducción de Señal
14.
Chemotherapy ; 61(2): 77-86, 2016.
Artículo en Inglés | MEDLINE | ID: mdl-26606244

RESUMEN

BACKGROUND: Postoperative 1-year administration of S-1, an oral derivative of 5-fluorouracil (5-FU), was shown to be feasible in lung cancer. The 5-year survival rates of postoperative patients treated with S-1 adjuvant chemotherapy and the prognostic impact of clinicopathological factors were examined. METHODS: The data of 50 patients with curatively resected pathological stage IB to IIIA non-small cell lung cancer, who were treated with S-1 postoperatively, were analyzed. The prognostic impacts of 22 clinicopathological factors including expressions of the 5-FU pathway enzymes were evaluated. A single-nucleotide polymorphism (SNP), i.e. 538G>A (rs17822931), of ABCC11/MRP8, which encodes a 5-FU excretion enzyme that is known as an earwax type determinant, was also evaluated. RESULTS: The 5-year overall and relapse-free survival rates were 72.5 and 67.5%, respectively. A performance status ≥ 1, lymphatic vessel invasion, blood vessel invasion, and the A/A type of SNP538, which is responsible for the dry earwax type, were significantly associated with shorter relapse-free survivals. In 34 patients who showed a relative performance of 70% or more for chemotherapy, multivariate survival analysis indicated significant hazard ratios only for the A/A type of SNP538 (p = 0.007). CONCLUSIONS: S-1 has sufficient power as adjuvant chemotherapy. However, its effect might be small in the dry earwax type patient group in an adjuvant setting.


Asunto(s)
Transportadoras de Casetes de Unión a ATP/genética , Carcinoma de Pulmón de Células no Pequeñas/tratamiento farmacológico , Neoplasias Pulmonares/tratamiento farmacológico , Ácido Oxónico/administración & dosificación , Polimorfismo de Nucleótido Simple , Tegafur/administración & dosificación , Transportadoras de Casetes de Unión a ATP/metabolismo , Administración Oral , Adulto , Anciano , Anciano de 80 o más Años , Antimetabolitos Antineoplásicos/administración & dosificación , Carcinoma de Pulmón de Células no Pequeñas/genética , Carcinoma de Pulmón de Células no Pequeñas/mortalidad , Carcinoma de Pulmón de Células no Pequeñas/cirugía , Quimioterapia Adyuvante , Supervivencia sin Enfermedad , Combinación de Medicamentos , Femenino , Humanos , Neoplasias Pulmonares/genética , Neoplasias Pulmonares/mortalidad , Neoplasias Pulmonares/cirugía , Masculino , Persona de Mediana Edad , Pronóstico , Análisis de Supervivencia
15.
Pediatr Int ; 58(3): 235-237, 2016 Mar.
Artículo en Inglés | MEDLINE | ID: mdl-26678362

RESUMEN

More than 1000 hemoglobin (Hb) variants have been identified. Hb Kirksey, a rare Hb variant involving mutation at codon 94 of α2-globin, is associated with low oxygen affinity. To our knowledge, there is no case report on Hb Kirksey in the literature, and, accordingly, the clinical features of patients with Hb Kirksey are currently unknown. We report here the case of a neonate who presented with asymptomatic low oxygen saturation on pulse oximetry (SpO2 ) just after birth, together with clinical analysis of the sister and cousin. Based on the presentations, Hb Kirksey does not seem to be associated with clinical abnormalities. Discrepancy between SpO2 and partial pressure of arterial oxygen (PaO2 ) detected on arterial blood gas analysis provided a clue to the diagnosis. Hb variants should be suspected and arterial blood gases should be measured in pediatric cases of unexplained low SpO2 .


Asunto(s)
Hemoglobinopatías/sangre , Hemoglobinas Anormales/análisis , Preescolar , Diagnóstico Diferencial , Femenino , Pruebas Genéticas , Hemoglobinopatías/diagnóstico , Hemoglobinopatías/genética , Hemoglobinas Anormales/genética , Humanos , Recién Nacido , Masculino , Oximetría , Oxígeno/sangre
16.
Tohoku J Exp Med ; 238(1): 9-16, 2016 01.
Artículo en Inglés | MEDLINE | ID: mdl-26656426

RESUMEN

Nogo-B, located in the endoplasmic reticulum, is an isoform belonging to the reticulon protein family, which is expressed specifically in cholangiocytes and non-parenchymal cells in the liver. Nogo-B expression is down-regulated with the progression of liver fibrosis, but its distinct function in liver malignancies has not been fully clarified. We have hypothesized that Nogo-B expression may be altered in intrahepatic cholangiocarcinoma (ICC), a relatively rare type of primary liver cancer with highly malignant behavior. The present study aimed to investigate the relationship between Nogo-B expression, assessed by immunohistochemical staining, and clinicopathological factors and prognosis in 34 ICC patients. Positive expression was observed in 19 (56%) of 34 ICC specimens: 6 patients (18%) with positivity levels of 1+ (positive cells in 10-50% of cancer cells) and 13 patients (38%) with 2+ (positive cells over 50%). Importantly, the remaining 15 patients (44%) were categorized as negative expression (Nogo-B-positive cells, less than 10%). Conversely, the mass-forming type of ICC tended to express Nogo-B with the degree of 2+ positivity, compared to the periductal infiltration type (p = 0.064), and the mass-forming type showed a better 5-year survival rate (66% vs. 5%) after hepatectomy (p < 0.05). However, the degree of positivity was not associated with tumor relapse rate, disease-free and overall survival, although each of the periductal infiltration type, intrahepatic metastasis, larger tumor size, and lower microvessel counts was associated with lower survival rates. We propose that Nogo-B expression is down-regulated in ICC, the implication of which, however, remains to be investigated.


Asunto(s)
Colangiocarcinoma/metabolismo , Regulación hacia Abajo , Proteínas de la Mielina/metabolismo , Anciano , Proliferación Celular , Colangiocarcinoma/patología , Colangiocarcinoma/cirugía , Femenino , Humanos , Masculino , Microvasos/metabolismo , Microvasos/patología , Recurrencia Local de Neoplasia/patología , Proteínas Nogo , Pronóstico , Tasa de Supervivencia
17.
Dig Surg ; 32(1): 32-8, 2015.
Artículo en Inglés | MEDLINE | ID: mdl-25678189

RESUMEN

BACKGROUND/AIMS: Oldest-old patients generally have several comorbidities, and laparoscopic-assisted colectomy (LAC) has not been performed on these patients. However, the surgical technique of LAC has improved, and its indications have been extended. The aim of this study was to evaluate the safety and effectiveness of LAC for patients over 85 years old. METHODS: Fifty-eight patients over 85 years old who underwent colectomy were retrospectively analyzed. The patients were divided into two groups (LAC group n = 15; open surgery group (Open group) n = 43), and clinicopathological features, surgical characteristics, and outcomes were compared. RESULTS: There were no significant differences in clinical background characteristics between the groups. The LAC group had longer operation time and greater lymph node dissection (both p < 0.01). Postoperatively, the use of analgesics (p = 0.01) was less and the start of oral liquid intake (p = 0.03) was faster in the LAC group. Postoperative complications occurred in 3 patients (20%) in the LAC group and 13 patients (30%) in the Open group (p = 0.66); delirium (n = 6) and sub-ileus (n = 4) developed only in the Open group. CONCLUSION: After LAC, elderly patients tended to have less postoperative pain and started oral liquid intake earlier. LAC can be safe and effective, preventing postoperative complications that occur specifically in oldest-old patients.


Asunto(s)
Neoplasias Colorrectales/cirugía , Factores de Edad , Anciano de 80 o más Años , Colectomía , Neoplasias Colorrectales/epidemiología , Comorbilidad , Femenino , Humanos , Japón , Laparoscopía , Masculino , Estudios Retrospectivos , Resultado del Tratamiento
18.
J Surg Res ; 192(2): 395-401, 2014 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-24974153

RESUMEN

BACKGROUND: Portal hypertension is a major risk factor for hepatic failure or bleeding in patients who have undergone hepatectomy, but it cannot be measured indirectly. We attempted to evaluate the intraoperative ultrasonography parameters that correlate with portal pressure (PP) in patients undergoing hepatectomy. METHODS: We examined 30 patients in whom PP was directly measured during surgery. The background liver conditions included chronic viral liver disease in seven patients, chemotherapy-associated steatohepatitis in four patients, fatty liver in one patient, hepatolithiasis in one patient, obstructive jaundice in one patient, and a normal liver in 16 patients. A multivariate logistic analysis and linear regression analysis were conducted to develop a predictive formula for PP. RESULTS: The mean PP was 10.4 ± 4.1 mm Hg. The PP tended to be increased in patients with chronic viral hepatitis. A univariate analysis identified the association of the six following parameters with PP: the platelet count and the maximum (max), minimum (min), endo-diastolic, peak-systolic, and mean velocity in the portal vein (PV) flow. Using multiple linear regression analysis, the predictive formula using the PV max and min was as follows: Y (estimated PP) = 18.235-0.120 × (PV max.[m/s])-0.364 × (PV min). The calculated PP (10.44 ± 2.61 mm Hg) was nearly the same as the actual PP (10.43 ± 4.07 mm Hg). However, there was no significant relationship between the calculated PP and the intraoperative blood loss and post hepatectomy morbidity. CONCLUSIONS: This formula, which uses ultrasonographic Doppler flow parameters, appears to be useful for predicting PP.


Asunto(s)
Hepatectomía , Hipertensión Portal/diagnóstico por imagen , Hipertensión Portal/cirugía , Monitoreo Intraoperatorio/métodos , Presión Portal/fisiología , Ultrasonografía Doppler/métodos , Adulto , Anciano , Anciano de 80 o más Años , Femenino , Hepatitis Crónica/diagnóstico por imagen , Hepatitis Crónica/fisiopatología , Hepatitis Crónica/cirugía , Hepatitis Viral Humana/diagnóstico por imagen , Hepatitis Viral Humana/fisiopatología , Hepatitis Viral Humana/cirugía , Humanos , Hipertensión Portal/fisiopatología , Modelos Lineales , Pruebas de Función Hepática , Masculino , Persona de Mediana Edad , Cuerpos Multivesiculares , Vena Porta/fisiopatología , Valor Predictivo de las Pruebas
19.
Hepatol Res ; 44(13): 1308-19, 2014 Dec.
Artículo en Inglés | MEDLINE | ID: mdl-24506195

RESUMEN

AIM: To evaluate hepatic fibrosis and tumor diagnosis preoperatively, we investigated the elasticity calculated by the new parameter of ultrasonography, acoustic radiation force impulse (ARFI). METHODS: We examined ARFI of the non-tumorous right and left lateral liver and in the tumor by push pulse of probe in 95 patients with hepatic malignancies undergoing hepatectomy. Measurement of ARFI as hepatic stiffness was indicated as the Vs (m/s). RESULTS: Measuring the Vs in the non-tumor region was achieved in the right liver in 99% and at the left lateral liver in 94%. The Vs in the right liver was significantly lower than in the left lateral liver, and the Vs of the liver tumor was significantly higher than in the non-tumorous liver. The Vs in the right and left lateral liver was correlated with the platelet count, aspartate aminotransferase, fibrotic indices and indocyanine green test. The Vs in the right liver was significantly correlated with the fibrotic marker or index. The Vs of liver cirrhosis and histological stage 4 in the right and left liver was significantly the highest compared to the others. The Vs in the right liver showed a high area under the receiver-operator curve value predicting histological fibrosis. The Vs in the right was significantly correlated with blood loss and postoperative complications, particularly uncontrolled ascites. CONCLUSION: Non-invasive ARFI imaging elastography is useful in evaluating impaired liver function or in the differential diagnosis of liver malignancies, highly hepatic fibrosis and in predicting posthepatectomy morbidity.

20.
Hepatogastroenterology ; 61(136): 2315-6, 2014.
Artículo en Inglés | MEDLINE | ID: mdl-25699373

RESUMEN

3-dimensional printed liver was constructed using 3D vascular imaging in a patient with intrahepatic cholangiocarcinoma who underwent major hepatectomy. The reproducibility of 3D modeling by the latest imaging has been clarified and future preoperative simulation should be adramatically changed.


Asunto(s)
Neoplasias de los Conductos Biliares/patología , Conductos Biliares Intrahepáticos/patología , Colangiocarcinoma/patología , Imagenología Tridimensional , Hígado/patología , Neoplasias de los Conductos Biliares/cirugía , Conductos Biliares Intrahepáticos/cirugía , Colangiocarcinoma/cirugía , Femenino , Humanos , Persona de Mediana Edad
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