Cushing disease in pediatrics: an update

Concepción-Zavaleta Marcio José,Armas Cristian David,Quiroz-Aldave Juan Eduardo,García-Villasante Eilhart Jorge,Gariza-Solano Ana Cecilia,Durand-Vásquez María del Carmen,Concepción-Urteaga Luis Albert 대한소아내분비학회 2023 Annals of Pediatirc Endocrinology & Metabolism Vol.28 No.2

Cushing disease (CD) is the main cause of endogenous Cushing syndrome (CS) and is produced by an adrenocorticotropic hormone (ACTH)-producing pituitary adenoma. Its relevance in pediatrics is due to the retardation of both growth and developmental processes because of hypercortisolism. In childhood, the main features of CS are facial changes, rapid or exaggerated weight gain, hirsutism, virilization, and acne. Endogenous hypercortisolism should be established after exogenous CS has been ruled out based on 24-hour urinary free cortisol, midnight serum or salivary cortisol, and dexamethasone suppression test; after that, ACTH dependence should be established. The diagnosis should be confirmed by pathology. The goal of treatment is to normalize cortisol level and reverse the signs and symptoms. Treatment options include surgery, medication, radiotherapy, or combined therapy. CD represents a challenge for physicians owing to its multiple associated conditions involving growth and pubertal development; thus, it is important to achieve an early diagnosis and treatment in order to control hypercortisolism and improve the prognosis. Its rarity in pediatric patients has led physicians to have limited experience in its management. The objective of this narrative review is to summarize the current knowledge about the pathophysiology, diagnosis, and treatment of CD in the pediatric population.

Survival and Clinical Behavior of Hypertrophic Cardiomyopathy in a Latin American Cohort in Contrast to Cohorts from the Developed World

Nilda Espinola-Zavaleta,Antonio Vega,Diego Martínez Basto,Ana Cecilia Alcantar-Fernández,Veronica Guarner Lans,María Elena Soto 한국심초음파학회 2015 Journal of Cardiovascular Imaging (J Cardiovasc Im Vol.23 No.1

Background: Hypertrophic cardiomyopathy (HCM) is the most common hereditary heart disease with diverse phenotipyc,genetic expession and clinical presentations. The evolution of patients with HCM in Latin America has not been properly describedbeing the frequency, the long-term prognosis as well as the predominant phenotypic expression still unknown. The aim of thisstudy was to determine the survival rate of HCM patients having different phenotypes in a Mexican cohort of patients. Methods: Clinical and echocardiographic data obtained from 77 Mexican patients with recently diagnosed HCM wereanalyzed. The follow-up was of 12.5 years. Results: 96.1% of patients were in functional class I/II according to the New York Heart Association, 2.6% in class III and 1.3%in class IV. Only 3.9% of them went to surgery for myectomy. During the follow-up, 17 patients (22%) died: 4/9 (44%) had apicalHCM, 5/20 (25%) had obstructive septal asymmetric HCM, 6/35 (17%) had nonobstructive septal asymmetric HCM and 2/3(15%) had concentric HCM. The survival rate was worse for patients with apical HCM, followed by those with obstructive andnonobstructive septal asymmetric HCM and patients showing concentric HCM had the best survival rates. There is significantdifference in survival rates which declined in 65% in a 9 years-period. Log rank test showed significant differences (p < 0.002). Conclusion: The survival rate of patients with HCM was worse in those with apical variety. The majority of patients receivedmedical treatment. The indication for myectomy was below that observed in other international centers.

Prevalence of Congenital Heart Disease and Pulmonary Hypertension in Down’s Syndrome: An Echocardiographic Study

Nilda Espinola-Zavaleta,,María Elena Soto,Angel Romero-Gonzalez,Lidia del Carmen Gómez-Puente,Luis Muñoz-Castellanos,Aasha S. Gopal,Candace Keirns,Eulo Lupi-Herrera 한국심초음파학회 2015 Journal of Cardiovascular Imaging (J Cardiovasc Im Vol.23 No.2

Background: Down’s syndrome (DS) is a genetic anomaly, which undergoes increased morbidity and mortality when associatedwith congenital heart disease (CHD). The aims of the study were to determine the prevalence of CHD and pulmonary hypertension(PH) in DS. Methods: One hundred twenty-seven patients with DS living in Mexico City were evaluated by physical exam, electrocardiogramand echocardiogram. Results: CHD was found in 40%. In 80% (n = 102) PH was present [systolic pulmonary artery pressure (SPAP) of 47 ± 19mm Hg and mean pulmonary artery pressure (MPAP) of 32 ± 11 mm Hg]. Patients with CHD and PH were classified ashaving 1) no shunt (n = 18) with SPAP of 37 ± 9 mm Hg and MPAP of 25 ± 6 mm Hg and 2) with shunt (n = 26) with PASPof 57 ± 29 mm Hg and MPAP of 38 ± 19 mm Hg (p ≤ 0.001). In those without CHD or with CHD without shunt (n = 76), SPAPwas 37 ± 19 mm Hg and the MPAP 25 ± 6 mm Hg. The prevalence of PH in DS was 5.9% at one year and 15% at 10 years. Theodds ratio of PH in DS with CHD was 7.3 vs. 3 without CHD. Conclusion: DS has a high prevalence of CHD and PH. PH prevalence increases when it is associated with CHD. Thepathophysiology of PH in DS without CHD should be studied in the near future. Echocardiography is an indispensible tool forevaluation of DS.

A Bayesian cure rate model with dispersion induced by discrete frailty

Cancho, Vicente G.,Zavaleta, Katherine E.C.,Macera, Marcia A.C.,Suzuki, Adriano K.,Louzada, Francisco The Korean Statistical Society 2018 Communications for statistical applications and me Vol.25 No.5

In this paper, we propose extending proportional hazards frailty models to allow a discrete distribution for the frailty variable. Having zero frailty can be interpreted as being immune or cured. Thus, we develop a new survival model induced by discrete frailty with zero-inflated power series distribution, which can account for overdispersion. This proposal also allows for a realistic description of non-risk individuals, since individuals cured due to intrinsic factors (immunes) are modeled by a deterministic fraction of zero-risk while those cured due to an intervention are modeled by a random fraction. We put the proposed model in a Bayesian framework and use a Markov chain Monte Carlo algorithm for the computation of posterior distribution. A simulation study is conducted to assess the proposed model and the computation algorithm. We also discuss model selection based on pseudo-Bayes factors as well as developing case influence diagnostics for the joint posterior distribution through ${\psi}-divergence$ measures. The motivating cutaneous melanoma data is analyzed for illustration purposes.

Blue Maize Extract Improves Blood Pressure, Lipid Profiles, and Adipose Tissue in High-Sucrose Diet-Induced Metabolic Syndrome in Rats

Rosa Isela Guzman-Geronimo,Tania Margarita Alarcon-Zavaleta,Rosa Marıa Oliart-Ros,Jose Enrique Meza-Alvarado,Socorro Herrera-Meza,Jose Luis Chavez-Servia 한국식품영양과학회 2017 Journal of medicinal food Vol.20 No.2

The effect of blue maize extract in factors related to metabolic syndrome (MS) in Wistar rats was investigated. Total polyphenols, monomeric anthocyanins, and antioxidant activity were analyzed in blue maize. MS was induced in Wistar rats fed with high-sucrose (HS) diet for 12 weeks. During a period of 4 weeks, blue maize extract was administrated to HS groups fed with high-sucrose and high-cholesterol–high-sucrose (HS+C) diets. In the blue maize extract administered by orogastric cannulation, the levels of total polyphenols and anthocyanins were 9.97 and 2.92 mg/kg of weight, respectively. HS diet administered during a period of 12 weeks increased significantly systolic blood pressure, serum triglycerides, and decreased high-density lipoprotein cholesterol (HDL-C), alterations related to the MS. Abdominal adipose tissue was only increased in the HS + C group. Blue maize extract administration enhanced HDL-C and decreased systolic blood pressure, serum triglycerides, total cholesterol, and epididymal adipose tissue weight. The blue maize may represent a promising nutraceutical option for the treatment of MS.

Cell Wall Localization of Two DUF642 Proteins, BIIDXI and TEEBE, during Meloidogyne incognita Early Inoculation

Alexis Salazar-Iribe,Esther Zúñiga-Sánchez,Emma Zavaleta Mejía,Alicia Gamboa-deBuen 한국식물병리학회 2017 Plant Pathology Journal Vol.33 No.6

The root-knot nematode Meloidogyne incognita infects a variety of plants, including Arabidopsis thaliana. During migration, root-knot nematodes secrete different proteins to modify cell walls, which include pectolytic enzymes. However, the contribution of host cell wall proteins has not been described during this process. The function of two DUF642 cell wall proteins, BIIDXI (BDX, At4g32460) and TEEBE (TEB, At2g41800), in plant development could be related to the regulation of pectin methyl esterification status in the cell walls of different tissues. Accordingly, the expression of these two genes is up-regulated by auxin. BDX and TEB were highly induced during early M. incognita inoculation. Moreover, cell wall localization of the proteins was also induced. The cell wall localization of BDX and TEB DUF642 proteins during M. incognita early inoculation suggested that these two proteins could be involved in the regulation of the degree of pectin methylation during cell separation.

Molecular Characterization of Protease Producing Idiomarina Species Isolated from Peruvian Saline Environments

( Carol N. Flores-fernandez ),( Elizabeth Chavez-hidalgo ),( Marco Santos ),( Amparo I. Zavaleta ),( David R. Arahal ) 한국미생물생명공학회(구 한국산업미생물학회) 2019 한국미생물·생명공학회지 Vol.47 No.3

All Idiomarina species are isolated from saline environments; microorganisms in such extreme habitats develop metabolic adaptations and can produce compounds such as proteases with an industrial potential. ARDRA and 16S rRNA gene sequencing are established methods for performing phylogenetic analysis and taxonomic identification. However, 16S-23S ITS is more variable than the 16S rRNA gene within a genus, and is therefore, used as a marker to achieve a more precise identification. In this study, ten protease producing Idiomarina strains isolated from the Peruvian salterns were characterized using biochemical and molecular methods to determine their bacterial diversity and industrial potential. In addition, comparison between the length and nucleotide sequences of a 16S-23S ITS region allowed us to assess the inter and intraspecies variability. Based on the 16S rRNA gene, two species of Idiomarina were identified (I. zobellii and I. fontislapidosi). However, biochemical tests revealed that there were differences between the strains of the same species. Moreover, it was found that the ITS contains two tRNA genes, tRNA^{Ile (GAT)} and tRNA^{Ala (TGC)}, which are separated by an ISR of a variable size between strains of I. zobellii. In one strain of I. zobellii (PM21), we found nonconserved nucleotides that were previously not reported in the tRNA^Ala gene sequences of Idiomarina spp. Thus, based on the biochemical and molecular characteristics, we can conclude that protease producing Idiomarina strains have industrial potential; only two I. zobellii strains (PM48 and PM72) exhibited the same properties. The differences between the other strains could be explained by the presence of subspecies.

Molecular Characterization of Protease Producing Idiomarina Species Isolated from Peruvian Saline Environments

Flores-Fernandez, Carol N.,Chavez-Hidalgo, Elizabeth,Santos, Marco,Zavaleta, Amparo I.,Arahal, David R. The Korean Society for Microbiology and Biotechnol 2019 한국미생물·생명공학회지 Vol.47 No.3

All Idiomarina species are isolated from saline environments; microorganisms in such extreme habitats develop metabolic adaptations and can produce compounds such as proteases with an industrial potential. ARDRA and 16S rRNA gene sequencing are established methods for performing phylogenetic analysis and taxonomic identification. However, 16S-23S ITS is more variable than the 16S rRNA gene within a genus, and is therefore, used as a marker to achieve a more precise identification. In this study, ten protease producing Idiomarina strains isolated from the Peruvian salterns were characterized using biochemical and molecular methods to determine their bacterial diversity and industrial potential. In addition, comparison between the length and nucleotide sequences of a 16S-23S ITS region allowed us to assess the inter and intraspecies variability. Based on the 16S rRNA gene, two species of Idiomarina were identified (I. zobellii and I. fontislapidosi). However, biochemical tests revealed that there were differences between the strains of the same species. Moreover, it was found that the ITS contains two tRNA genes, $tRNA^{Ile(GAT)}$ and $tRNA^{Ala(TGC)}$, which are separated by an ISR of a variable size between strains of I. zobellii. In one strain of I. zobellii (PM21), we found nonconserved nucleotides that were previously not reported in the $tRNA^{Ala}$ gene sequences of Idiomarina spp. Thus, based on the biochemical and molecular characteristics, we can conclude that protease producing Idiomarina strains have industrial potential; only two I. zobellii strains (PM48 and PM72) exhibited the same properties. The differences between the other strains could be explained by the presence of subspecies.

Cell Wall Localization of Two DUF642 Proteins, BIIDXI and TEEBE, during Meloidogyne incognita Early Inoculation

Salazar-Iribe, Alexis,Zuniga-Sanchez, Esther,Mejia, Emma Zavaleta,Gamboa-deBuen, Alicia The Korean Society of Plant Pathology 2017 Plant Pathology Journal Vol.33 No.6

The root-knot nematode Meloidogyne incognita infects a variety of plants, including Arabidopsis thaliana. During migration, root-knot nematodes secrete different proteins to modify cell walls, which include pectolytic enzymes. However, the contribution of host cell wall proteins has not been described during this process. The function of two DUF642 cell wall proteins, BIIDXI (BDX, At4g32460) and TEEBE (TEB, At2g41800), in plant development could be related to the regulation of pectin methyl esterification status in the cell walls of different tissues. Accordingly, the expression of these two genes is up-regulated by auxin. BDX and TEB were highly induced during early M. incognita inoculation. Moreover, cell wall localization of the proteins was also induced. The cell wall localization of BDX and TEB DUF642 proteins during M. incognita early inoculation suggested that these two proteins could be involved in the regulation of the degree of pectin methylation during cell separation.