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BACKGROUND: The incidence of pulmonary hypertension (PH) associated with bronchopulmonary dysplasia (BPD) has not been investigated in regional cohorts. The aim of this study was to clarify the incidence of PH associated with BPD in all very low birthweight infants (VLBWIs) born during the study period in Aichi Prefecture, Japan. METHODS: We conducted a retrospective observational cohort study of all VLBWIs born in Aichi Prefecture. The inclusion criteria were VLB, birth between 1 January 2015 and 31 December 2015, and admission to any neonatal intensive care unit in Aichi Prefecture. BPD28d and BPD36w were defined as the need for supplemental oxygen or any respiratory support at 28 days of age or 36 weeks of postmenstrual age (PMA). The primary outcome was the incidence of PH after 36 weeks' PMA (PH36w) in VLBWIs with BPD28d and BPD36w. The secondary outcomes were the clinical factors related to PH36w in BPD36w patients. Mann-Whitney U-test and Fisher's exact test were used for univariate analysis. Differences were considered statistically significant at P < 0.05. Risk ratio (RR) and 95% confidence interval (CI) were also evaluated. RESULTS: A total of 441 patients were analyzed. A total of 217 and 131 patients met the definition of BPD28d and BPD36w, respectively. Nine patients were diagnosed with PH36w (4.2% and 6.9% of the BPD28d and BPD36w patients, respectively). The presence of oligohydramnios (RR, 2.71; 95% CI: 1.55-4.73, P = 0.014) and sepsis (RR, 3.62; 95% CI: 1.51-8.63, P = 0.025) was significant in the PH36w patients. CONCLUSIONS: The incidence of PH36w was 4.2% and 6.9% in the BPD28d and BPD36w patients, respectively. Oligohydramnios and sepsis were significantly associated with PH36w in VLBWIs.
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Displasia Broncopulmonar , Hipertensión Pulmonar , Oligohidramnios , Sepsis , Displasia Broncopulmonar/complicaciones , Displasia Broncopulmonar/diagnóstico , Displasia Broncopulmonar/epidemiología , Estudios de Cohortes , Femenino , Edad Gestacional , Humanos , Hipertensión Pulmonar/epidemiología , Hipertensión Pulmonar/etiología , Lactante , Recién Nacido , Embarazo , Estudios RetrospectivosRESUMEN
Objective: This study aimed to report on 15 Japanese patients with acrodysostosis and pseudohypoparathyroidism (PHP) and analyze them using the newly proposed classification of the EuroPHP network to determine whether this classification system is suitable for Japanese patients. Design: We divided the patients into three groups based on hormone resistance, the number of fingers with short metacarpals, the existence of cone-shaped epiphyses and gene defects. Methods: We carried out clinical, radiological and genetic evaluations of two patients in group A (iPPSD5), six patients in group B (iPPDS4) and seven patients in group C (iPPSD2). Results: Group A consisted of two siblings without hormone resistance who had the most severe bone and physical developmental delays. PDE4D gene defects were detected in both cases. Group B consisted of six patients who showed hormone resistance without hypocalcemia. Short metacarpal bones with corn-shaped epiphyses were observed in all patients. In two cases, PRKAR1A gene defects were detected; however, their clinical and radiological features were not identical. The facial dysmorphism and developmental delay were less severe and PRKAR1A gene defects were detected in case B-3. Severe facial dysmorphism and deformity of metacarpal bones were observed, but no gene defect was detected in case B-1. Group C consisted of seven patients with PHP1a, four of whom had maternally inherited heterozygous inactivating mutations in one of the GNAS genes. The clinical and radiological features of the patients in group C were not identical either. Conclusions: The newly proposed classification is suitable for Japanese patients; however, heterogeneities still existed within groups B and C.
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BACKGROUND: Hydrops fetalis (HF) has a low survival rate, particularly in the case of preterm birth. In addition, the severity index of HF has not been fully investigated yet. The aim of this study was to clarify the prognostic factors of HF with pleural effusion. METHODS: All live-born HF patients with pleural effusion, except for chromosomal abnormality or complex congenital heart disease, born from 2009 to 2013 in Aichi Prefecture in Japan were included. Prenatal, perinatal, and postnatal information was obtained from the medical records and was retrospectively analyzed. RESULTS: Forty-one HF patients with pleural effusion were included, and 28 patients (68%) survived. On multivariate logistic stepwise analysis, gestational birth week (OR, 0.71; 95% CI: 0.52-0.96, P = 0.027) and standard deviation (SD) score of the birthweight (OR, 1.74; 95% CI: 1.01-2.99, P = 0.045) were significant factors for postnatal death. All patients with both ≥32 gestational weeks and <3.0 birthweight SD score survived. CONCLUSIONS: Combined with the gestational weeks data, birthweight SD score may be useful to estimate the prognosis of HF with pleural effusion.
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Hidropesía Fetal/diagnóstico , Enfermedades del Prematuro/diagnóstico , Derrame Pleural/diagnóstico , Femenino , Edad Gestacional , Humanos , Hidropesía Fetal/mortalidad , Recién Nacido , Recien Nacido Prematuro , Enfermedades del Prematuro/mortalidad , Modelos Logísticos , Masculino , Análisis Multivariante , Derrame Pleural/etiología , Derrame Pleural/mortalidad , Pronóstico , Estudios Retrospectivos , Índice de Severidad de la Enfermedad , Tasa de SupervivenciaRESUMEN
The number of long-term surviving stem cell transplant (SCT) recipients has increased steadily, and attention has now extended to the late complications of this procedure. The objective of this study was to investigate relationship among growth and endocrine functions in long-term adult survivors of childhood SCT. The inclusion criteria of this study were survival at least 5 yr after SCT and achievement of adult height. Fifty-four patients (39 males) fulfilled these criteria and were included in this study. Growth was mainly evaluated by height standard deviation score (SDS) and individual longitudinal growth curves. Among the 54 patients, those that received SCT before 10 yr of age showed significantly greater reductions in changes in height SDS (mean -1.75, range -4.80 to -0.10) compared with those that received SCT at or after 10 yr of age (mean -0.50, range -1.74 to 1.20; P<0.001). The mean loss of height for all patients who received SCT during childhood was estimated to be approximately 1 SDS/6.5 yr (r=0.517). Individual longitudinal growth curves indicated that a significant growth spurt was absent in severe short stature patients during the pubertal period without severe endocrine dysfunctions including GH deficiency. The incidence of growth disorder in long-term adult survivors depends on the age at SCT and whether they received radiation therapy. Life-long follow-up is necessary for survivors to detect, prevent and treat the late endocrine complications in SCT survivors.
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With a view to reducing pollution in the aquatic environment, estrone (E1) and 17beta-estradiol (E2) were tested for their absorbability in water onto activated carbons (ACs) with various pore-size distributions. In batch-type adsorption measurements, all adsorption isotherms obtained were found to fit a Freundlich equation. In case of eight different kinds of commercial AC in pure water, the amount adsorbed at equilibrium concentration of 1 microg/L was in the range of 25.6-73.5mg/g for E1 and 21.3-67.6 mg/g for E2. In case of pre-used ACs in water sand-filtered for use as drinking water, the amount adsorbed at 1 microg/L was in the range of 3.5-8.2mg/g for E2. In the case of two commercial ACs in river water and in effluent from secondary clarifier at municipal sewage treatment plant (MSTP), both originally containing E2, was in the range of 0.1-0.2 and 0.3-1 microg/g, respectively, at 1 ng/L. The difference of amount adsorbed onto AC was discussed in view of hydrophobicity of target chemicals, specific surface area and mean pore diameter of AC, and the difference in the absorbability and the ratios of co-present substances for the target compound.
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Carbono/química , Estradiol/aislamiento & purificación , Estrona/aislamiento & purificación , Purificación del Agua/métodos , Absorción , PorosidadRESUMEN
An increasing number of long-term surviving bone marrow transplant (BMT) recipients have recovered from their primary disease but are at risk of developing failure of endocrine organs. We investigated 147 patients who underwent allogeneic BMT. Thyroid function was evaluated by serial measurement of basal TSH and free T4 levels as well as by TRH provocative test. Thyroid ultrasound examination was performed for evaluation of thyroid tumor after BMT. Five patients were found to have overt thyroid dysfunction (hypothyroidism in four patients and hyperthyroidism in one patient). Twenty-three patients in the under 9-yr-old group at BMT and 16 patients in the over 10-yr-old group at BMT had subclinical compensated hypothyroidism. Younger age at BMT was the strongest factor for developing thyroid dysfunction, compared with older age (P < 0.001). Only in patients with subclinical compensated hypothyroidism did median basal and peak TSH increase to the upper half of the normal range by 8 yr after BMT and then returned slightly to the middle of the normal range spontaneously. These results suggest that thyroid dysfunction in long-term BMT survivors depends on age at BMT, with a greater risk among younger patients, indicating the need for life-long surveillance.
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Trasplante de Médula Ósea/efectos adversos , Enfermedades de la Tiroides/etiología , Enfermedades de la Tiroides/fisiopatología , Glándula Tiroides/fisiopatología , Adenoma/etiología , Adolescente , Niño , Femenino , Estudios de Seguimiento , Humanos , Hipertiroidismo/etiología , Hipotiroidismo/etiología , Incidencia , Masculino , Enfermedades de la Tiroides/diagnóstico por imagen , Enfermedades de la Tiroides/epidemiología , Pruebas de Función de la Tiroides , Glándula Tiroides/diagnóstico por imagen , Neoplasias de la Tiroides/etiología , UltrasonografíaRESUMEN
BACKGROUND: Changes in thyroid function among young patients who received bone marrow transplantation (BMT) were evaluated. METHODS: The study included 91 patients (50 males) who underwent BMT from 1985 to 1995 at the age of 0.6-21 years. Sixty patients had neoplastic disease such as leukemia or lymphoma, and the remainder had non-neoplastic diseases. Preconditioning regimen for BMT included 12 Gy of fractionated-total body irradiation (TBI) for patients with neoplastic disease and 3-8 Gy of irradiation for the remaining patients, in addition to chemotherapy. Evaluation of thyroid function was performed by serial assessment of basal serum FT4, FT3, TSH concentrations as well as by TRH test. RESULTS: No patient had overt hypothyroidism or elevated basal TSH concentrations (>10 mU/L). However, 6 (7%) of patients experienced exaggerated peak TSH response to TRH stimulation several years after BMT. In 33 patients whose thyroid status was evaluated before, within 3 months, and 1 year after BMT, serum FT3 concentrations as well as peak TSH response to TRH stimulation significantly decreased immediately after BMT (<3 months) and normalized within 1 year. However, serum FT4 concentrations did not change significantly. One patient developed primary hypothyroidism and another developed follicular adenoma of the thyroid 5 and 12 years after BMT, respectively. CONCLUSION: Short-term changes in thyroid function after BMT can indicate euthyroid sick syndrome rather than tertiary hypothyroidism. It must be noted that overt hypothyroidism may occur several years after BMT, hence long-term follow-up of thyroid function is warranted.
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Trasplante de Médula Ósea , Glándula Tiroides/fisiopatología , Adolescente , Adulto , Niño , Preescolar , Síndromes del Eutiroideo Enfermo/diagnóstico , Síndromes del Eutiroideo Enfermo/etiología , Femenino , Estudios de Seguimiento , Humanos , Hipotiroidismo/diagnóstico , Hipotiroidismo/etiología , Lactante , Masculino , Pruebas de Función de la Tiroides , Hormonas Tiroideas/sangre , Hormona Liberadora de Tirotropina , Factores de Tiempo , Acondicionamiento PretrasplanteRESUMEN
Treatment plan for neonates with borderline hypothyroidism (persistent hyperthyrotropinemia with normothyroxinemia) has not been established. In this study, changes in thyroid function after discontinuation of low-dose L-thyroxine (L-T4) supplement in infants with the condition were evaluated. Fourteen infants with hyperthyrotropinemia at neonatal screening had repeated hyperthyrotropinemia (> 8 mU/L) with normothyroxinemia. TSH response was exaggerated at TRH testing. The subjects were treated with low-dose L-T4 (3 to 9 microg/kg/day) for 2.2 to 6 years, and euthyroid status was maintained. After discontinuation of therapy, mild hyperthyrotropinemia persisted up to 24 months, while serum FT4 remained within the lower half of the normal range. TSH response to TRH stimulation, which tended to be exaggerated 1 month after discontinuation, became lower 6 to 12 months later. RAIU and thyroid scintigraphy were normal in all subjects. No patient developed hypothyroxinemia, although mild elevation of TSH lasted rather long after discontinuation of low-dose L-T4 therapy. Administration of L-T4 was not resumed provided the subjects were followed at regular interval. Further long-term investigation is needed to define whether re-administration is necessary or not.