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1.
Endocr J ; 69(8): 919-926, 2022 Aug 29.
Artigo em Inglês | MEDLINE | ID: mdl-35321987

RESUMO

Beckwith-Wiedemann syndrome (BWS) is infrequently associated with adrenocortical carcinoma (ACC) or non-hormone-producing adrenal cytomegaly, but we recently, encountered a single case of adrenal cytomegaly in a patient with BWS, which was difficult to distinguish from androgen-producing adrenocortical carcinoma (ACC). Here, we describe the case of a 4-month-old female who presented with clitoromegaly, hemihypertrophy, and an adrenal mass identified during the prenatal period. The mass was located in detected at the left suprarenal region and detected at 20 weeks of gestational age. At birth, she also presented with clitoromegaly and elevated serum levels of 17α-hydroxyprogesterone, dehydroepiandrosterone, and testosterone at birth and experienced hyper-insulinemic hypoglycemia, which improved following diazoxide therapy. We initially suspected androgen-producing ACC with metastasis and the left adrenal mass was resected accordingly when the patient reached 4 months of age. However, histological examination revealed adrenal cytomegaly. Genetic analysis revealed paternal uniparental disomy, and the patient was finally diagnosed as having BWS. Resection of the left adrenal gland restored the serum androgen levels to normal physiological levels without any recurrence. While it is reasonably well known that BWS is sometimes accompanied by virilization due to androgen-producing ACC, our findings are among the first to suggest that adrenal cytomegaly can also increase androgen hormone production. Thus, we propose that adrenal cytomegaly should be considered one of the differential diagnoses when accompanied with hyperandrogenism in BWS patients.


Assuntos
Neoplasias do Córtex Suprarrenal , Doenças das Glândulas Suprarrenais , Carcinoma Adrenocortical , Síndrome de Beckwith-Wiedemann , Androgênios , Feminino , Humanos , Lactente , Recém-Nascido , Gravidez , Dissomia Uniparental
2.
Clin Lab ; 67(1)2021 Jan 01.
Artigo em Inglês | MEDLINE | ID: mdl-33491421

RESUMO

BACKGROUND: Bacterial infections and some antibiotics show displacer effects on bilirubin-albumin binding and increase unbound bilirubin (UB) but not total bilirubin (TB) in serum. METHODS: A case study was conducted to show a successful treatment of hyperbilirubinemia by monitoring UB. RESULTS: In an extremely preterm infant with bloodstream bacterial infection caused by methicillin-resistant coagulase-negative staphylococci, 2 days after high-dose ampicillin and regular-dose amikacin were initiated, UB markedly increased, but TB did not. After vancomycin was substituted, UB decreased immediately with phototherapy and intravenous albumin infusion. CONCLUSIONS: When using antibiotics, the clinicians should be mindful regarding the displacer effect on bilirubin-albumin binding.


Assuntos
Infecções Bacterianas , Lactente Extremamente Prematuro , Bilirrubina , Humanos , Hiperbilirrubinemia/terapia , Lactente , Recém-Nascido , Fototerapia
3.
Pediatrics ; 146(6)2020 12.
Artigo em Inglês | MEDLINE | ID: mdl-33214331

RESUMO

OBJECTIVES: Our aim is to compare the efficacy and safety of high-flow nasal cannula (HFNC) against those of nasal continuous positive airway pressure (NCPAP) or nasal intermittent positive-pressure ventilation (NIPPV) after extubation in preterm infants. METHODS: This prospective, randomized, noninferiority trial was conducted in 6 tertiary NICUs. Infants born at <34 weeks who needed noninvasive ventilation after extubation were enrolled. We randomly assigned infants to an HFNC group when HFNC was used or to an NCPAP/NIPPV group when NCPAP or NIPPV was used. The primary outcome was treatment failure within 7 days after extubation. We then examined clinical aspects of treatment failure with HFNC use. RESULTS: In total, 176 and 196 infants were assigned to the HFNC and NCPAP/NIPPV groups, respectively. The HFNC group showed a significantly higher rate of treatment failure than that of the NCPAP/NIPPV group, with treatment failure occurring in 54 infants (31%) compared with 31 infants (16%) in the NCPAP/NIPPV group (risk difference, 14.9 percentage points; 95% confidence interval, 6.2-23.2). Histologic chorioamnionitis (P = .02), treated patent ductus arteriosus (P = .001), and corrected gestational age at the start of treatment (P = .007) were factors independently related to treatment failure with HFNC use. CONCLUSIONS: We found HFNC revealed a significantly higher rate of treatment failure than NCPAP or NIPPV after extubation in preterm infants. The independent factors associated with treatment failure with HFNC use were histologic chorioamnionitis, treated patent ductus arteriosus, and a younger corrected gestational age at the start of treatment.


Assuntos
Extubação , Pressão Positiva Contínua nas Vias Aéreas/instrumentação , Recém-Nascido Prematuro , Unidades de Terapia Intensiva Neonatal , Síndrome do Desconforto Respiratório do Recém-Nascido/terapia , Cânula , Desenho de Equipamento , Feminino , Seguimentos , Humanos , Recém-Nascido , Masculino , Estudos Prospectivos , Falha de Tratamento
4.
Pediatr Int ; 62(8): 926-931, 2020 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-32170965

RESUMO

BACKGROUND: The objective of the present study was to verify the speed and accuracy of fetal ultrasonic Doppler (fetal Doppler) in measuring heart rate of newborns at rest, including preterm, low-birthweight infants, and its efficacy during neonatal resuscitation, including cases of neonatal asphyxia. METHODS: A three-lead electrocardiogram and fetal Doppler were used to measure resting heart rates in 100 newborns, including 48 preterm, low-birthweight infants, at 0 to 72 h after birth. Times to display heart rate were compared between electrocardiogram and fetal Doppler by the Bland-Altman analysis and Wilcoxon signed-rank test. The time required for the fetal Doppler to measure heart rate during neonatal resuscitation was also assessed. RESULTS: In 100 newborns, the mean error of the resting heart rate in 1,293 measurement points was 0.07 beats/min. To display the heart rate, the fetal Doppler required a median time of 5 s, and electrocardiogram required a median time of 10 s (P < 0.001). During neonatal resuscitation, the heart rate was measured within 10 s in 18 of 21 cases (86%) and displayed with a median time of 5 s; this was measured in all neonatal asphyxia cases (9/9, 100%). CONCLUSIONS: Fetal Doppler can measure heart rate in newborns accurately and rapidly and is useful for evaluating heart rate not only at rest but also during neonatal resuscitation, especially in asphyxia.


Assuntos
Asfixia Neonatal/terapia , Eletrocardiografia/métodos , Frequência Cardíaca , Ressuscitação/métodos , Ultrassonografia Doppler/métodos , Feminino , Humanos , Recém-Nascido de Baixo Peso , Recém-Nascido , Recém-Nascido Prematuro , Masculino , Ultrassonografia Pré-Natal/métodos
6.
Pediatr Int ; 59(10): 1064-1068, 2017 Oct.
Artigo em Inglês | MEDLINE | ID: mdl-28714076

RESUMO

BACKGROUND: During neonatal resuscitation, careful oxygenation is needed. Pulse oximetry is recommended to evaluate the need for oxygenation, but it is not clear whether peripheral perfusion is adequate for the evaluation of arterial oxygen saturation (SpO2 ). Additionally, there has been no study on the changes in SpO2 immediately after birth in Japan, despite the indispensable need for definitive oxygenation criteria. METHODS: A prospective observational study was performed in neonates at gestational age 35-41 weeks. An SpO2 measurement probe was attached to the neonates immediately after birth at the right palm or wrist, and the perfusion index (PI), pulse rate, and SpO2 were measured until 10 min after birth. RESULTS: Sixty neonates were examined. Stable PI was obtained soon after birth, preceding SpO2 measurement. The median PI (%) was constant at approximately 1.3, and the median SpO2 at 2-10 min was 70%, 81%, 82%, 87%, 89%, 92%, 92%, 94%, and 95%, respectively. The current target value for SpO2 in the Neonatal Cardiopulmonary Resuscitation (NCPR) guideline in Japan is approximately the 25th percentile. CONCLUSION: PI is stable and sufficient in the early postnatal period, meaning that peripheral perfusion is adequate for the measurement of SpO2 . The current target SpO2 used in the NCPR guidelines is at approximately the 25th percentile and is thought to be sufficient for meeting oxygenation criteria.


Assuntos
Oximetria , Oxigênio/sangue , Biomarcadores/sangue , Feminino , Humanos , Recém-Nascido , Japão , Masculino , Estudos Prospectivos , Valores de Referência
7.
Artigo em Inglês | MEDLINE | ID: mdl-27914518

RESUMO

Term neonates have high delta-6 desaturase (D6D) activity, which is important for regulating polyunsaturated fatty acid's (PUFA) nutritional status. The aim was to investigate D6D activity in preterm infants and its postnatal changes. Forty-three appropriate-for-gestational-age infants were included. PUFA in red blood cells was analyzed at birth and at one, six, and 12 months of age. D6D activity was estimated by 20:3n-6/18:2n-6 ratio. At birth, preterm infants had D6D activity as high as that of term infants; D6D activity declined to about one-third at one month, then further decreased to about one-sixth at six months and remained stable until 12 months. The postnatal change in arachidonic acid exhibited a similar pattern to that of D6D activity; however, docosahexaenoic acid showed a transient decrease at one month and recovered to the cord blood level at six months. D6D may regulate PUFA profile in preterm infants, especially during the early postnatal period.


Assuntos
Ácidos Graxos Dessaturases/metabolismo , Ácidos Graxos Insaturados/sangue , Ácido Araquidônico/sangue , Ácidos Docosa-Hexaenoicos/sangue , Feminino , Regulação da Expressão Gênica no Desenvolvimento , Idade Gestacional , Humanos , Lactente , Recém-Nascido , Recém-Nascido Prematuro , Masculino
8.
J Clin Lipidol ; 9(5): 647-51, 2015.
Artigo em Inglês | MEDLINE | ID: mdl-26350810

RESUMO

BACKGROUND: Fetal organs require much lipid for growth, but the cord blood had low TG concentrations, compared to adult serum. We investigated the association between the concentration of apolipoprotein A-V (apoA-V) and lipid profile in cord blood and neonatal serum. OBJECTIVE: ApoA-V was identified as an important determinant of plasma triglyceride concentrations. We sought to determine the association between serum apoA-V concentrations and lipoprotein profile in preterm infants and its early postnatal change. METHODS: Sixty-three neonates (35 males and 28 females; 15 term and 48 preterm) were included. Serum lipoprotein profile and apoA-V concentrations were determined at birth and 1 month. RESULTS: Cord blood apoA-V concentrations in appropriate-for-gestational age infants were extremely low (13.1 ± 3.4 ng/mL in term infants, 4.4 ± 0.9 ng/mL in preterm infants) compared with adult values, and those of small-for-gestational age infants were further low (6.4 ± 4.2 ng/mL, 2.2 ± 1.3 ng/mL, respectively). During the first month, serum apoA-V concentration markedly increased, and the concentration of preterm appropriate-for-gestational age infants caught up, whereas that of preterm small-for-gestational age infants did not. At birth, apoA-V concentration positively correlated with gestational age (r = 0.354, P = .0069) but not with birth weight Z-score. ApoA-V concentration had a positive association with very low-density lipoprotein triglyceride concentrations (r = 0.646, P < .0001), and the relationships still remained at 1 month (r = 0.283, P = .0348). CONCLUSIONS: ApoA-V in neonates was unique in its serum concentration and in the association with lipoprotein profile.


Assuntos
Apolipoproteínas A/sangue , Recém-Nascido Prematuro/sangue , Apolipoproteína A-V , Feminino , Humanos , Recém-Nascido , Recém-Nascido Pequeno para a Idade Gestacional/sangue , Masculino
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