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1.
Bone Rep ; 18: 101673, 2023 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-36968251

RESUMO

Pachydermoperiostosis (PDP) is a rare hereditary disease characterized by digital clubbing, pachydermia, and periostosis. We describe a Japanese male patient with PDP who was differentially diagnosed with acromegaly by identification of compound heterozygous variants in SLCO2A1. Recent studies have reported various clinical manifestations, as well as skeletal and dermal features, in patients with PDP. Genetic testing provided not only PDP diagnosis and differentiation from acromegaly, but also information about possible complications and comorbidities throughout life.

2.
Endocr J ; 70(4): 435-443, 2023 Apr 28.
Artigo em Inglês | MEDLINE | ID: mdl-36740254

RESUMO

Achondroplasia is a rare skeletal dysplasia characterized by rhizomelic short stature, whose prevalence is about 1 per 25,000 births. For some patients with achondroplasia, excess body weight is one of the major concerns due to an impaired linear growth. Epidemiological studies revealed a premature onset of cardiovascular or cerebrovascular events in achondroplasia. An association between obesity and cardiometabolic risk factors related to cardiovascular events remains unknown in patients with achondroplasia/hypochondroplasia. This cross-sectional study investigated anthropometric measurements, body compositions and cardiometabolic risk factors in pediatric patients with achondroplasia/hypochondroplasia. Thirty-two patients with achondroplasia and ten with hypochondroplasia aged between 1.9 and 18.7 years were enrolled in this study. Half of the participants presented at least one cardiometabolic abnormality. Elevated systolic blood pressure was the most common abnormality. None of the participants developed metabolic syndrome or type 2 diabetes mellitus. Body mass index-standard deviation score and hip/height ratio were strongly correlated with percent body fat assessed by dual energy X-ray absorptiometry although no significant association was found between anthropometric measurements or body fat mass and any cardiometabolic risk factors. No significant difference in body fat mass, as well as body mass index-standard deviation score and hip/height, was found between cardiometabolically normal group and cardiometabolically abnormal groups. These results suggest that not only weight gain and hip/height changes should be monitored but also individual cardiometabolic risk factors should be evaluated to avoid cardiometabolic events in the healthcare management of pediatric patients with achondroplasia/hypochondroplasia.


Assuntos
Acondroplasia , Doenças Cardiovasculares , Diabetes Mellitus Tipo 2 , Humanos , Criança , Adolescente , Lactente , Pré-Escolar , Estudos Transversais , Acondroplasia/complicações , Acondroplasia/epidemiologia , Índice de Massa Corporal , Doenças Cardiovasculares/epidemiologia , Doenças Cardiovasculares/etiologia , Fatores de Risco
3.
Sci Rep ; 12(1): 22116, 2022 12 21.
Artigo em Inglês | MEDLINE | ID: mdl-36543833

RESUMO

Mothers of children with chronic hepatitis C virus (HCV) infection experience anxiety about the health of their children. In this study we assessed an impact of treating children with chronic HCV infection on the psychological burden of their mothers. This was a multicenter, questionnaire survey conducted at six institutions in Japan. A newly-developed questionnaire for this study was used to assess changes in the mothers' various concerns regarding HCV infection and thoughts about their child's HCV infection. Responses at the time of diagnosis and at the time of the survey were compared between mothers of children who had received treatment and those without treatment. Responses were received from 36 of 37 eligible mothers (11 and 25, non-treatment and treatment groups, respectively). All children in treatment group had successfully eliminated the virus. Mothers in both groups were psychologically stressed in various ways, including concern about their child's health in the present and future at the time of diagnosis, concern about school, employment, and marriage, concern about the behavior of others towards them and infecting others with HCV, and feelings of guilt regarding their child. These concerns were significantly lower in the present compared to at the time of diagnosis in treatment group, and the rate of decrease was significantly higher in treatment group compared to non-treatment group. Successful treatment greatly reduced mothers' concerns about their children's HCV infection, indicating that treatment during childhood is beneficial from the perspective of the mothers' psychological burden.


Assuntos
Hepatite C Crônica , Feminino , Humanos , Criança , Mães/psicologia , Emoções , Ansiedade , Inquéritos e Questionários
4.
Oncogene ; 41(38): 4385-4396, 2022 09.
Artigo em Inglês | MEDLINE | ID: mdl-35970887

RESUMO

Tumor necrosis factor-related apoptosis-inducing ligand (TRAIL) induces cancer cell death and contributes to tumor rejection by cytotoxic lymphocytes in cancer immunosurveillance and immunotherapy. TRAIL and TRAIL receptor agonists have garnered wide popularity as promising agents for cancer therapy. We previously demonstrated that the loss of fucosylation in cancer cells impairs TRAIL sensitivity; however, the precise structures of the fucosylated glycans that regulate TRAIL sensitivity and their carrier molecules remain elusive. Herein, we observed that Lewis glycans among various fucosylated glycans positively regulate TRAIL-induced cell death. Specifically, Lewis glycans on lacto/neolacto glycosphingolipids, but not glycoproteins including TRAIL receptors, enhanced TRAIL-induced formation of the cytosolic caspase 8 complex, without affecting the formation of the membranous receptor complex. Furthermore, type I Lewis glycan expression in colon cancer cell lines and patient-derived cancer organoids was positively correlated with TRAIL sensitivity. These findings provide novel insights into the regulatory mechanism of TRAIL-induced cell death and facilitate the identification of novel predictive biomarkers for TRAIL-related cancer therapies in future.


Assuntos
Neoplasias , Receptores do Ligante Indutor de Apoptose Relacionado a TNF , Apoptose , Proteínas Reguladoras de Apoptose/metabolismo , Caspase 8/metabolismo , Glicoesfingolipídeos/farmacologia , Humanos , Ligantes , Glicoproteínas de Membrana/metabolismo , Neoplasias/tratamento farmacológico , Receptores do Ligante Indutor de Apoptose Relacionado a TNF/genética , Receptores do Ligante Indutor de Apoptose Relacionado a TNF/metabolismo , Ligante Indutor de Apoptose Relacionado a TNF/metabolismo , Ligante Indutor de Apoptose Relacionado a TNF/farmacologia , Fator de Necrose Tumoral alfa/metabolismo
5.
Sci Rep ; 12(1): 11205, 2022 07 01.
Artigo em Inglês | MEDLINE | ID: mdl-35778417

RESUMO

At present, noninvasive fibrosis markers are not available for the assessment of liver fibrosis in children with chronic hepatitis C. Sixty-three children with chronic hepatitis C were included. Changes in Wisteria floribunda agglutinin-positive Mac-2 binding protein (M2BPGi) levels were evaluated in l3 of 27 treatment-naive patients during the natural course of disease (median 4, range 3-6 years). Changes during treatment were evaluated in 27 of 36 patients for 4 (2-9) years of posttreatment follow-up. There were significant differences in the levels of M2BPGi between control group and HCV F0 group (P = 0.002) and between control group and HCV F1 group (P < 0.001). Receiver operating characteristic curve analysis showed that to discriminate stage F1 fibrosis from F0, the cut-off value was 0.95 for M2BPGi with a sensitivity of 52%, specificity of 90%, and area under the curve of 0.687. A substantial decrease in M2BPGi levels by treatment was shown from 0.98 ± 0.57 at pretreatment to 0.42 ± 0.15 at posttreatment (P < 0.001) in the 27 treated patients. Our study shows new findings that M2BPGi may be useful to predict the presence of a mild degree of fibrosis in children with chronic hepatitis C, and such mild fibrosis may be quickly resolved by treatment.


Assuntos
Hepatite C Crônica , Lectinas de Plantas , Receptores de N-Acetilglucosamina , Criança , Hepatite C Crônica/sangue , Humanos , Cirrose Hepática/sangue , Cirrose Hepática/diagnóstico , Glicoproteínas de Membrana/sangue , Lectinas de Plantas/sangue , Receptores de N-Acetilglucosamina/sangue
6.
Pediatr Int ; 64(1): e14962, 2022 Jan.
Artigo em Inglês | MEDLINE | ID: mdl-35224815

RESUMO

BACKGROUND: The first guidelines for care of pregnant women carrying the hepatitis C virus (HCV) and their infants were published in 2005 in Japan. Since then, evidence has gradually accumulated worldwide regarding the natural course and treatment of this condition and, especially in recent years, treatment for chronic hepatitis C in adult patients has made great progress. However, the clinical practice policy for children has not been standardized, and new clinical practice guidelines for children with mother-to-child (MTC) transmitted HCV infection have become necessary. METHODS: In the development of the current guideline, we requested cooperation from The Japanese Society for Pediatric Infectious Diseases, The Japan Society of Hepatology, and the Japan Society of Obstetrics and Gynecology. The committee members were recommended and approved by each society to participate in developing the guidelines. The guideline was also created in accordance with the Minds Guide for Practice Guideline Development. The statements were prepared by consensus-building using the Delphi method, based on the comprehensively searched academic papers and guidelines. These articles were retrieved through searching the PubMed, Cochrane Library, and the Igaku Chuo Zasshi databases. RESULTS: Eight clinical questions (CQs) with clinical statements were developed regarding etiology (CQs 1-3), diagnosis (CQs 4 and 5), and treatment (two CQs 6 and 7). In each statement, the consensus rate, evidence level, and recommendation level were determined. CONCLUSION: The guidelines will be helpful in the management of children with hepatitis C MTC transmission.


Assuntos
Gastroenterologia , Hepatite C , Adulto , Feminino , Hepacivirus , Hepatite C/diagnóstico , Hepatite C/tratamento farmacológico , Humanos , Lactente , Transmissão Vertical de Doenças Infecciosas/prevenção & controle , Mães , Gravidez
8.
Int J Clin Oncol ; 27(2): 281-300, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-35022887

RESUMO

The Japan Society of Clinical Oncology (JSCO) published the "JSCO Clinical Practice Guidelines 2017 for Fertility Preservation in Childhood, Adolescent, and Young Adult Cancer Patients" in 2017. This was the first guideline in cancer reproductive medicine in Japan. In the field of cancer reproductive medicine, close cooperation between an oncologist and a physician for reproductive medicine is important from before treatment initiation until long after treatment. The guideline takes into consideration disease specificity and provides opinions from the perspective of oncologists and specialists in reproductive medicine that are in line with the current state of the Japanese medical system. It is intended to serve as a reference for medical staff in both fields regarding the availability of fertility preservation therapy before the start of cancer treatment. Appropriate use of this guideline makes it easier to determine whether fertility preservation therapy is feasible and, ultimately, to improve survivorship in childhood, adolescent, and young adult cancer patients. In this article (Part 2), we describe details by organ/system and also for pediatric cancer.


Assuntos
Preservação da Fertilidade , Neoplasias , Oncologistas , Adolescente , Criança , Humanos , Japão , Oncologia , Neoplasias/terapia , Adulto Jovem
9.
Int J Clin Oncol ; 27(2): 265-280, 2022 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-34973107

RESUMO

In 2017, the Japan Society of Clinical Oncology (JSCO) published the JSCO Clinical Practice Guidelines 2017 for Fertility Preservation in Childhood, Adolescent, and Young Adult Cancer Patients. These were the first Japanese guidelines to address issues of oncofertility. In this field of medicine, sustained close cooperation between oncologists and reproductive specialists is essential from the diagnosis of cancer until many years after completion of cancer treatment. These JSCO guidelines were intended to guide multidisciplinary medical staff in considering the availability of fertility preservation options and to help them decide whether to provide fertility preservation to childhood, adolescent, and young adult cancer patients before treatment starts, with the ultimate goal of improving patient survivorship. The guidelines are presented as Parts 1 and 2. This article (Part 1) summarizes the goals of the guidelines and the methods used to develop them and provides an overview of fertility preservation across all oncology areas. It includes general remarks on the basic concepts surrounding fertility preservation and explanations of the impacts of cancer treatment on gonadal function by sex and treatment modality and of the options for protecting/preserving gonadal function and makes recommendations based on 4 clinical questions. Part 2 of these guidelines provides specific recommendations on fertility preservation in 8 types of cancer (gynecologic, breast, urologic, pediatric, hematologic, bone and soft tissue, brain, and digestive).


Assuntos
Preservação da Fertilidade , Neoplasias , Oncologistas , Adolescente , Criança , Feminino , Humanos , Japão , Oncologia , Neoplasias/terapia , Adulto Jovem
10.
J Pediatr Endocrinol Metab ; 34(11): 1411-1418, 2021 Nov 25.
Artigo em Inglês | MEDLINE | ID: mdl-34388329

RESUMO

OBJECTIVES: Iodine deficiency and excess both cause thyroid dysfunction. Few data describe the relationship between iodine status and outcomes of congenital hypothyroidism (CH) in iodine-sufficient areas. We investigated urinary iodine (UI) concentration and its relationship with the clinical course of CH. METHODS: We reviewed and retrospectively analyzed patients with positive newborn screening (NBS) for CH from January 2012 to June 2019 in Japan, obtaining UI and UI-urine creatinine ratio (UI/Cr), serum TSH, free T4, free T3 and thyroglobulin (Tg) at the first visit, TSH at NBS, levothyroxine (LT4) dose, and subsequent doses. A UI value of 100-299 µg/L was considered adequate. RESULTS: Forty-eight patients were included. Median UI and UI/Cr were 325 µg/L and 3,930 µg/gCr, respectively. UI was high (≥300 µg/L) in 26 (54%) and low (≤99 µg/L) in 11 (23%). LT4 was administered to 34 patients. Iodine status was not related to the need for treatment. We found a U-shaped relationship between Tg and UI/Cr. Patients with high Tg (≥400 ng/mL) and abnormal UI levels required significantly lower LT4 doses (≤20 µg/day) at three years of age. Even if they showed severe hypothyroidism initially, they did not need subsequent dose increments. CONCLUSIONS: Abnormal UI levels with Tg elevation were associated with lower LT4 dose requirements. The evaluation of iodine status and Tg concentrations were considered useful in patients suspected of CH.


Assuntos
Hipotireoidismo Congênito/diagnóstico , Iodo/urina , Tireoglobulina/urina , Biomarcadores/sangue , Biomarcadores/urina , Hipotireoidismo Congênito/urina , Feminino , Humanos , Recém-Nascido , Masculino , Triagem Neonatal , Tireotropina/sangue , Tiroxina/sangue , Tri-Iodotironina/sangue
11.
Clin Pediatr Endocrinol ; 30(3): 143-148, 2021.
Artigo em Inglês | MEDLINE | ID: mdl-34285457

RESUMO

Herein, we report two girls with a neonatal screening (NS)-negative 21-hydroxylase deficiency (21-OHD) requiring treatment with hydrocortisone due to virilization that developed in late childhood. Patient 1 was born prematurely on the 30th gestational week with normal external genitalia at birth. She passed the NS for 21-OHD. At 6 yr of age, she was referred to a hospital for evaluation of premature pubarche and clitoromegaly. Her diagnosis was central precocious puberty, and GnRH agonist was initiated. However, her symptoms did not improve despite treatment for over 4 years. She was then referred to our hospital where she was diagnosed with 21-OHD. Although she was started on hydrocortisone therapy, her adult height reached only 140 cm (-3.4 SD). Patient 2 was delivered at 37 weeks of gestation and passed the NS for 21-OHD. She was referred to a hospital because of premature pubarche at the age of 6 yr. She was diagnosed with 21-OHD, and hydrocortisone replacement therapy was initiated. Her present height at 13 yr of age is 148 cm (-1.3 SD). These cases reminded us that the possibility of 21-OHD should be considered when patients show premature pubarche or precocious puberty, even if they passed the NS test for 21-OHD.

12.
Clin Pediatr Endocrinol ; 29(2): 55-62, 2020.
Artigo em Inglês | MEDLINE | ID: mdl-32313373

RESUMO

Existing guidelines recommend long-term follow-up of childhood cancer survivors (CCS). However, in Japan, transitional care for CCS has not been established. To ascertain the current status in Japan, and to cultivate a better understanding, a questionnaire survey was conducted on transitional care in CCS, and adolescent and young adult (AYA) cancer survivors. Questionnaires were distributed to 183 councilors (137 institutions) of the Japanese Society for Pediatric Endocrinology. A total of 131 responses, representative of 174 councilors, were obtained. The response rate was 95%. Among the respondents, 91% had experience in medical care for cancer patients, while 63% had experience in transitional care; however, the number of patients referred to adult clinics was small. Further, 89% acknowledged the availability of adult endocrinologists who were willing to accept these patients; although their numbers were insufficient. Pediatric endocrinologists highlighted difficulties in medical examinations concerning infertility, obesity, pregnancy/delivery, and gonadal dysfunction, in that order. Staff and time shortages were listed as some of the challenges faced by medical staff, while multisystem morbidity was listed for patients. This nationwide questionnaire survey revealed that Japanese pediatric endocrinologists require cooperation between related departments and collaborative infrastructure to develop transitional care for cancer survivors.

13.
Yonago Acta Med ; 63(1): 55-62, 2020 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-32158334

RESUMO

BACKGROUND: Radical prostatectomy and radiotherapy are standard treatments for localized prostate cancer. When making decisions about treatment, it is important to not only consider medical information such as the patient's age, performance status, and complications, but also the impact on quality of life (QOL) after treatment.Our purpose was to compare health related quality of life (HRQOL) after robot-assisted laparoscopic radical prostatectomy (RARP) versus radiation therapy in Japanese patients with localized prostate cancer retrospectively. METHODS: Patients with localized prostate cancer receiving RARP or radiotherapy at Tottori University Hospital between October 2010 and December 2014 were enrolled in a retrospective observational study with follow-up for 24 months to December 2016. The Medical Outcome Study 8-Item Short-Form Health Survey was performed before treatment and 1, 3, 6, 12, and 24 months post-treatment. RESULTS: Complete responses to the questionnaire were obtained from 154/227 patients receiving RARP, 41/67 patients receiving intensity-modulated radiation therapy, 35/82 patients receiving low dose rate brachytherapy, and 18/28 patients given low dose rate brachytherapy plus external beam radiation therapy. The median physical component summary score of the Medical Outcome Study 8-Item Short-Form Health Survey was significantly lower at 1 month after prostatectomy than radiotherapy, but was similar for both treatments at 3 months, and was significantly higher at 6, 12 and 24 months after prostatectomy. The median mental component summary score was also significantly lower in the prostatectomy group at 1 month, but not from 3 months onwards. CONCLUSION: Our study suggested that HRQOL was inferior at 1 month after RARP, however, recovered at 3 months after RARP and was better than after radiotherapy at 6, 12, and 24 months.

14.
Biol Trace Elem Res ; 195(1): 71-81, 2020 May.
Artigo em Inglês | MEDLINE | ID: mdl-31392541

RESUMO

The essential trace element zinc maintains liver functions. Liver diseases can alter overall zinc concentrations, and hypozincemia is associated with various hepatic pathologies. Modulating systemic zinc through dietary supplementation is potentially useful for liver diseases. We evaluated the usefulness of zinc (NPC-02; acetate formulation) supplementation. We conducted two NPC-02 studies on zinc-deficient patients (serum zinc < 70 µg/dL). Study 1: double-blind, randomized, placebo-controlled trial on 57 subjects with chronic liver diseases comparing serum zinc in patients given NPC-02 (NPC-02 group) versus placebo (Placebo group). Study 2: dose adjustment study on 43 subjects with/without liver diseases to determine proportions maintaining serum zinc target (≥ 80 µg/dL but < 200 µg/dL). In study 1, NPC-02 subjects had higher serum zinc concentrations at week 8 than Placebo subjects (83.2 ± 20.2 and 61.3 ± 12.0, respectively; P < 0.0001), and more NPC-02 than Placebo subjects achieved the serum zinc target (15/27 vs. 1/26). In study 2, the NPC-02-induced serum zinc increase was dose-dependent in subjects both with and without liver diseases (r = 0.5143, P = 0.0022 and r = 0.5753, P = 0.0005, respectively). Interestingly, there was a marginally positive correlation between serum zinc and albumin levels in subjects with but not in those without liver diseases (r = 0.4028, P = 0.0631 and r = 0.1360, P = 0.5567, respectively). NPC-02 dose-dependently increases serum zinc in hypozincemic patients, regardless of liver disease. NPC-02 is a potentially effective therapy for liver cirrhosis, in which zinc deficiency is common. Clinical trial registry number: NCT02337569, NCT02321865.


Assuntos
Hepatopatias/sangue , Acetato de Zinco/sangue , Zinco/sangue , Idoso , Doença Crônica , Suplementos Nutricionais , Método Duplo-Cego , Feminino , Humanos , Hepatopatias/patologia , Masculino , Pessoa de Meia-Idade , Acetato de Zinco/administração & dosagem
15.
J Occup Health ; 61(6): 508-516, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31368154

RESUMO

OBJECTIVES: To determine whether restorative yoga is an effective method for relieving occupational stress among female nurses working the night shift in Japan. METHODS: From July 2017 to May 2018, 20 female nurses aged in their 20s to 30s who were working the night shift at a university hospital participated in a randomized crossover trial, consisting of a 1-hour guided group yoga session followed by 4 weeks of at-home practice and 4 weeks of usual stress relief methods. The level of stress was assessed by the Brief Job Stress Questionnaire of the Ministry of Health, Labour and Welfare before and after performing restorative yoga. Descriptive statistics were calculated and data were analyzed by Student's t test, one-way analysis of variance (with repeated measures), or Bonferroni's multiple comparison test. RESULTS: Vital signs showed no significant differences from before to after the group yoga session, and there was no change of weight after 4 weeks of at-home practice. The mean questionnaire score for "psychological and physical stress reactions" was significantly reduced after the group yoga session. In addition, the mean score was significantly lower after 4 weeks of at-home practice than before or after group yoga, or after 4 weeks of the usual stress relief methods. CONCLUSIONS: These findings suggest that restorative yoga may be effective for alleviating occupational stress among female nurses working the night shift.


Assuntos
Enfermeiras e Enfermeiros , Estresse Ocupacional/terapia , Jornada de Trabalho em Turnos , Yoga , Adulto , Estudos Cross-Over , Feminino , Hospitais Universitários , Humanos , Japão , Terapia de Relaxamento/métodos , Adulto Jovem
16.
J Pediatr Endocrinol Metab ; 32(6): 585-595, 2019 Jun 26.
Artigo em Inglês | MEDLINE | ID: mdl-31150358

RESUMO

Background In Japan, prophylactic thyroidectomy involves out-of-pocket expense. The American Thyroid Association (ATA) recommends prophylactic thyroidectomy for medullary thyroid carcinoma (MTC) during early childhood in patients with multiple endocrine neoplasia type 2 (MEN2). The ATA reports a high frequency of postoperative complications in childhood, which also influenced the delay of prophylactic thyroidectomy in Japan. Methods This retrospective study of multiple medical centers in Japan included individuals aged <20 years diagnosed with germline RET mutations between 1997 and 2017. The onset and onset possibility were defined based on confirmed lesions or calcitonin levels. The definition of risk and prophylactic thyroidectomy were based on the ATA 2015 revised guideline. Results Twenty-one patients with MEN2 were enrolled (highest risk, n = 5; high risk, n = 5; and moderate risk, n = 11). The cumulative incidence of the onset/onset possibility reached 50% at 5 and 8 years and 100% at 9 years and 17 years in high- and moderate-risk patients, respectively. Of 7 patients with MEN2A, 71% underwent prophylactic thyroidectomy. Only one 5-year-old patient (C634Y) had increased serum calcitonin level after prophylactic thyroidectomy in the MEN2A group. The only permanent complication, which did not occur in patients who underwent total thyroidectomy alone, was hypoparathyroidism (33% of patients). This permanent complication occurred with clinically developed MTC. No permanent postoperative complications occurred in patients aged 5-6 years. Conclusions Prophylactic thyroidectomy reduces recurrence and postoperative complications in pediatric patients with MEN2. Early thyroidectomy based on only calcitonin level could possibly reduce thyroidectomy delay.


Assuntos
Biomarcadores/análise , Neoplasia Endócrina Múltipla Tipo 2a/cirurgia , Recidiva Local de Neoplasia/cirurgia , Complicações Pós-Operatórias , Tireoidectomia/métodos , Adolescente , Calcitonina/metabolismo , Criança , Pré-Escolar , Feminino , Seguimentos , Mutação em Linhagem Germinativa , Humanos , Japão/epidemiologia , Masculino , Neoplasia Endócrina Múltipla Tipo 2a/epidemiologia , Neoplasia Endócrina Múltipla Tipo 2a/genética , Neoplasia Endócrina Múltipla Tipo 2a/patologia , Recidiva Local de Neoplasia/genética , Recidiva Local de Neoplasia/patologia , Prognóstico , Proteínas Proto-Oncogênicas c-ret/genética , Estudos Retrospectivos , Inquéritos e Questionários , Fatores de Tempo
17.
ESMO Open ; 4(2): e000459, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30962960

RESUMO

PURPOSE: Cancer treatment delay due to fertility preservation procedures is a barrier for patients with breast cancer who wish to preserve their fertility. This study aimed to describe the associations between fertility preservation and treatment delay in patients with breast cancer with reproductive concerns and assess the factors related to treatment delay. METHODS: Patients with primary breast cancer who visited the reproductive unit at our institution before cancer treatment between 2007 and 2015 were enrolled. The treatment delay cut-off was defined as follows: time to chemotherapy (TTC) >8 weeks for patients intending to receive neoadjuvant chemotherapy, TTC >12 weeks for patients intending to receive adjuvant chemotherapy, time to endocrine therapy (TTE) >12 weeks for patients intending to receive endocrine therapy without radiation therapy and TTE >20 weeks for patients intending to receive endocrine therapy after radiation therapy. Multivariable models were constructed to examine the factors of treatment delay. RESULTS: Overall, 212 patients met the inclusion criteria. Using the defined cut-offs, treatment delay was noted in 18% of the patients. Endocrine therapy was related to treatment delay (OR 4.49, 95% CI 1.02 to 19.7; p=0.05), but fertility preservation by artificial reproductive treatment (ART) was not. Pregnancy and delivery following treatment for breast cancer were achieved in 18 (19%) and 15 (16%) patients who underwent fertility preservation with ART. CONCLUSION: Fertility preservation with ART was not associated with treatment delay in patients with breast cancer who were referred to reproductive specialists before cancer treatment.

18.
Hepatol Res ; 49(3): 314-323, 2019 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-30156739

RESUMO

AIM: Bile acid biosynthesis is strictly regulated under physiological conditions. The expression of fibroblast growth factor (FGF) 19 is induced when bile acids bind to the farnesoid X receptor in the intestinal epithelium. Fibroblast growth factor 19 is then transported by the portal flow, causing transcriptional inhibition of cytochrome P450, family 7, subfamily A, polypeptide 1 (CYP7A1), a key enzyme in bile acid biosynthesis, through the extracellular signal-regulated kinase (ERK) pathway. However, the regulatory mechanisms of these signaling pathways in hepatocytes under chronic cholestasis remain unclear. We investigated the regulation of these signaling pathways in patients with biliary atresia (BA). METHODS: We analyzed the regulation of molecules in these signaling pathways using liver and serum samples from eight BA children and four non-cholestatic disease controls. RESULTS: CYP7A1 mRNA expression was not inhibited in BA microdissected hepatocyte-enriched tissue (HET) despite high serum bile acid concentrations. The FGF19 protein was synthesized in BA HET, and its serum concentration was elevated. Fibroblast growth factor receptor 4 was phosphorylated in BA livers. However, ERK phosphorylation was significantly reduced. We examined SPRY2 expression to determine how the ERK pathway was inactivated downstream of the FGF receptor; the expression was significantly increased in BA HET. CONCLUSIONS: This is the first study to measure the CYP7A1 mRNA levels in human BA HET. Fibroblast growth factor 19 was increased in BA hepatocytes. By focusing on its regulation in hepatocytes, we showed that the FGF19 pathway did not suppress bile acid synthesis, probably due to an altered mechanism involving upregulated SPRY2 in BA patients.

19.
J Pediatr Gastroenterol Nutr ; 67(3): 383-387, 2018 09.
Artigo em Inglês | MEDLINE | ID: mdl-29851770

RESUMO

OBJECTIVES: Duodenal tube test (DTT) is used as a preoperative screening to rule out biliary atresia (BA). In previous reports, DTT was assessed by the color of the duodenal fluid, but there were no quantitative criteria. The aim of this study was to examine the efficacy of DTT based on the total bile acid (TBA) concentration in duodenal fluid. METHODS: This is a single-center retrospective study of infants with cholestasis who underwent DTT from 2008 to 2016 at the Osaka University Hospital. The cut-off values of maximum TBA in duodenal fluid (dTBA), dTBA/serum TBA ratio (sTBA), and dTBA/serum gamma-glutamyl transpeptidase (sGGT) ratio were assessed for the accuracy in excluding BA. RESULTS: A total of 37 infants were included in this study; 16 infants with BA and 21 infants with other causes of intrahepatic cholestasis. dTBA demonstrated sensitivity of 100% and specificity of 90.5% with the cut-off value of 16.8 µmol/L. Specificity was further improved to 95.2% with dTBA/sTBA ratio (cut-off value: 0.088) and 100% with dTBA/sGGT ratio (cut-off value: 0.076 µmol/U). DTT could be performed 0.8 ±â€Š1.4 days after admission. Hypoglycemia was developed in 1 infant. CONCLUSIONS: DTT evaluated by dTBA, dTBA/sTBA ratio, and dTBA/sGGT ratio had high accuracy to rule out BA and could avoid unnecessary surgery in some infants.


Assuntos
Ácidos e Sais Biliares/metabolismo , Atresia Biliar/diagnóstico , Atresia Biliar/metabolismo , Colestase/metabolismo , Duodeno/metabolismo , Cuidados Pré-Operatórios/métodos , Biomarcadores/metabolismo , Feminino , Humanos , Lactente , Masculino , Reprodutibilidade dos Testes , Estudos Retrospectivos , Sensibilidade e Especificidade
20.
Support Care Cancer ; 26(10): 3447-3452, 2018 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-29681013

RESUMO

PURPOSE: The American Society of Clinical Oncology (ASCO) Clinical Practice guidelines recommend that physicians, nurses, social workers, and other health care providers should be prepared to discuss the risk of infertility with patients. We conducted an educational program for non-physician health care providers regarding fertility preservation and evaluated the effects of the educational program. METHODS: The 4-h educational program consisted of lectures about infertility as a potential risk of cancer treatment, fertility preservation, and psychosocial support. Knowledge, confidence, institutional change, and self-practice were assessed pre-program, immediately post-program, and 6 months post-program. RESULTS: Of 124 participants who joined the program, 74 completed and returned the follow-up survey 6 months after the program. Sixty-one percent of the participants were nurses, 27% were social workers, and 4% were psychologists. The scores for confidence and knowledge increased between pre- and immediate post-program periods (p < 0.01), and between pre- and 6-month post-program periods (p < 0.01). The knowledge score was 52, 76, and 71% at the 3 points respectively. The participants became more likely to disseminate fertility preservation counseling at their institutions (p < 0.01) and use informational resources (p < 0.01). Overall, self-practice and institutional support did not change. CONCLUSIONS: The study revealed that this educational program is applicable for non-physicians to learn about fertility preservation. The participants improved significantly in confidence and knowledge, but not in counseling skills.


Assuntos
Atitude do Pessoal de Saúde , Educação Continuada , Preservação da Fertilidade , Pessoal de Saúde/educação , Pessoal de Saúde/psicologia , Infertilidade/prevenção & controle , Neoplasias/terapia , Adulto , Aconselhamento/educação , Feminino , Preservação da Fertilidade/psicologia , Seguimentos , Conhecimentos, Atitudes e Prática em Saúde , Promoção da Saúde/métodos , Humanos , Masculino , Pessoa de Meia-Idade , Neoplasias/psicologia , Médicos , Inquéritos e Questionários
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