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https://www.readbyqxmd.com/read/27747183/pectus-excavatum-from-a-pediatric-surgeon-s-perspective
#1
Donald Nuss, Robert J Obermeyer, Robert E Kelly
Historically, pectus excavatum (PE) was reported to be congenital, but in our experience only 22% are noticed in the first decade of life. Thus far, genetic studies support an autosomal recessive heritability, which coincides with only 40% of our patients having some positive family history, but is also contradictory given a constant sex ratio of 4:1 in favor of males. This inconsistency may be explained by the effect of more than one pectus disease-associated allele. Once the deformity is noticed, it tends to progress slowly until puberty, when rapid progression is often seen...
September 2016: Annals of Cardiothoracic Surgery
https://www.readbyqxmd.com/read/27199616/pubertal-maturation-compression-and-behavioral-impulsivity-among-boys-at-increased-risk-for-substance-use
#2
Charles W Mathias, Nora E Charles, Yuanyuan Liang, Ashley Acheson, Sarah L Lake, Stacy R Ryan, Rene L Olvera, Donald M Dougherty
OBJECTIVES: While early onset of puberty among girls has been related to substance use involvement and other adverse outcomes, less research has examined pubertal development and outcomes in boys. Further, research on puberty has not been conducted in the context of other risk factors for substance use involvement such as impulsivity. To address these gaps, this study characterized boys' pubertal development from preadolescence to mid-adolescence and related it to substance use risk and behavioral impulsivity...
June 2016: Addictive Disorders & Their Treatment
https://www.readbyqxmd.com/read/26339370/effects-of-hypoxia-and-its-relationship-with-apoptosis-stem-cells-and-angiogenesis-on-the-thymus-of-children-with-congenital-heart-defects-a-morphological-and-immunohistochemical-study
#3
A Bahar Ceyran, Serkan Şenol, Füsun Güzelmeriç, Eylem Tunçer, Aybala Tongut, Babürhan Özbek, Ömer Şavluk, Abdullah Aydın, Hakan Ceyran
INTRODUCTION: The thymus slowly involutes with age after puberty. Various stress conditions accelerate the involution of the thymus and cause changes in the histologic structure of the gland. OBJECTIVE: The present study performed histomorphological and immunohistochemical (IHC) evaluations of the thymus glands removed during surgical repair in patients with cyanotic or acyanotic congenital heart disease (CHD). The thymus glands in the hypoxic group were compared to those in the non-hypoxic group...
2015: International Journal of Clinical and Experimental Pathology
https://www.readbyqxmd.com/read/26156039/-surgical-treatment-of-scoliosis-in-childhood-and-adolescence-age-group-and-etiology-related-indications-and-choice-of-instrumentation
#4
J Matussek, A Benditz, E Dingeldey, F Völlner, D Boluki
The indications for a corrective surgical procedure for the complex 3-dimensional deformations of the spine collectively known under the term scoliosis, essentially depend on knowledge of the underlying etiology, the time of initial diagnosis in relation to the growth curve of the child and on considerations about the general operability of the patient. An early onset of scoliosis in childhood under defined diagnostic criteria is usually associated with a fast progression of spinal curvature and requires early surgical intervention during the growth period, while scoliosis in adolescence often allows a delayed surgical intervention until all conservative means have been taken into consideration...
July 2015: Der Orthopäde
https://www.readbyqxmd.com/read/25585547/ovarian-and-uterine-development-and-hormonal-feedback-mechanism-in-a-46-xx-patient-with-cyp19a1-deficiency-under-low-dose-estrogen-replacement
#5
Marie-Anne Burckhardt, Verena Obmann, Rainer Wolf, Marco Janner, Christa E Flück, Primus E Mullis
BACKGROUND: Aromatase deficiency may result in a complete block of estrogen synthesis because of the failure to convert androgens to estrogens. In females, this results in virilisation at birth, ovarian cysts in prepuberty and lack of pubertal development but virilisation, thereafter. OBJECTIVE AND METHODS: We studied the impact of oral 17β-estradiol treatment on ovarian and uterine development, and on LH/FSH and inhibin B during the long-term follow-up of a girl harboring compound heterozygote point mutations in the CYP19A1 gene...
May 2015: Gynecological Endocrinology
https://www.readbyqxmd.com/read/24536086/the-diagnostic-value-of-first-voided-urinary-lh-compared-with-gnrh-stimulated-gonadotropins-in-differentiating-slowly-progressive-from-rapidly-progressive-precocious-puberty-in-girls
#6
COMPARATIVE STUDY
Amnon Zung, Ella Burundukov, Mira Ulman, Tamar Glaser, Moshe Rosenberg, Malka Chen, Zvi Zadik
OBJECTIVE: Characterization of pubertal progression is required to prevent unnecessary intervention in unsustained or slowly progressive (SP) precocious puberty (PP), while delivering hormonal suppression in rapidly progressive (RP) PP. We aimed to assess the diagnostic value of first-voided urinary LH (ULH) compared with GNRH-stimulated gonadotropins in differentiating these forms of PP. METHODS: A total of 62 girls with PP underwent both GNRH stimulation and ULH assay...
May 2014: European Journal of Endocrinology
https://www.readbyqxmd.com/read/23725039/primary-hypothyroidism-with-growth-failure-and-pituitary-pseudotumor-in-a-13-year-old-female-a-case-report
#7
Noelle S Larson, Jordan E Pinsker
INTRODUCTION: Primary hypothyroidism is a well-known cause of poor linear growth in children. A rare finding with profound or long-standing disease is anterior pituitary enlargement (pituitary pseudotumor). This case highlights this uncommon finding, discusses clinical situations in which gradual dose escalation of levothyroxine may be advisable and reviews adjuvant therapies that have been previously attempted to improve final height in the setting of profound hypothyroidism. CASE PRESENTATION: We report the case of a 13-year-old Hispanic girl initially evaluated for poor linear growth and delayed puberty, and found to have pituitary enlargement secondary to profound primary hypothyroidism...
2013: Journal of Medical Case Reports
https://www.readbyqxmd.com/read/23483656/neuroinflammation-in-white-matter-tracts-of-cnp1-mutant-mice-amplified-by-a-minor-brain-injury
#8
Georg L Wieser, Ulrike C Gerwig, Bartosz Adamcio, Benoit Barrette, Klaus-Armin Nave, Hannelore Ehrenreich, Sandra Goebbels
Oligodendrocytes make myelin for rapid impulse propagation and contribute to the long-term survival of myelinated axons. The mechanisms by which oligodendroglial dysfunction(s) contribute to slowly progressive neurodegeneration are not well understood. Here, we demonstrate in Cnp1 mutant mice that secondary axonal degeneration in the subcortical white matter is associated with an age-dependent activation of both, innate and adaptive immune responses, including an expansion of infiltrating CD8+ T cells. While the detrimental role of lymphocytes in inherited myelin diseases is known, the role of activated microglia for the hypothetical cycle of inflammation/degeneration is unclear...
June 2013: Glia
https://www.readbyqxmd.com/read/23404027/condylar-hyperplasia
#9
Uma Shankar, Subhash Chandra, B H V Rama Krishnam Raju, G Anitha, K Venkata Srikanth, Afshan Laheji
Condylar hyperplasia (CH), as the name suggests, affects mandibular condyle producing overgrowth of condyle, which is characterized by a slowly progressive, usually unilateral enlargement of the mandible, facial asymmetry and deviation of chin to the unaffected side. The condition is known to be self-limiting, usually begins around puberty, but may not be recognized until later in life. This paper reports a case of severe facial asymmetry secondary to CH, which was successfully treated by high condylectomy only...
November 2012: Journal of Contemporary Dental Practice
https://www.readbyqxmd.com/read/21865362/testicular-failure-and-male-infertility-in-the-monogenic-mulibrey-nanism-disorder
#10
Susann Karlberg, Jorma Toppari, Niklas Karlberg, Mirja Nurmio, Riitta Karikoski, Hannu Jalanko, Marita Lipsanen-Nyman
CONTEXT: Few monogenic mutations causing human male infertility have been identified to date. OBJECTIVE: We studied pubertal development and fecundity in males with Mulibrey nanism (MUL) caused by mutations in the TRIM37 gene. DESIGN, SETTING, AND PATIENTS: Twenty-eight male MUL patients of the Finnish national cohort aged 8.7 to 50.0 yr (median age, 28.8) at the end of observation were followed for 10 yr beginning from 2000-2001. MAIN OUTCOME MEASURES: Clinical characteristics, reproductive hormone levels, semen quality, and testicular histology were assessed...
November 2011: Journal of Clinical Endocrinology and Metabolism
https://www.readbyqxmd.com/read/20886012/lh-dynamics-in-overweight-girls-with-premature-adrenarche-and-slowly-progressive-sexual-precocity
#11
Brian Bordini, Elizabeth Littlejohn, Robert L Rosenfield
Background. Excess adiposity and premature adrenarche (PA) are risk factors for the development of polycystic ovary syndrome (PCOS). Methods. Girls with slowly progressive precocious breast development, who were overweight and had PA (SPPOPA, 6.2-8.2 years, n = 5), overweight PA (6.6-10.8 years, n = 7), and overweight premenarcheal controls (OW-PUB, 10.6-12.8 years, n = 8) underwent hormonal sleep testing and GnRH agonist (GnRHag) and ACTH tests. Results. Despite an insignificant sleep-related increase in LH and prepubertal baseline hormone levels, SPPOPA peak LH and estradiol responses to GnRHag were intermediate between those of PA and OW-PUB, the LH being significantly different from both...
2010: International Journal of Pediatric Endocrinology
https://www.readbyqxmd.com/read/20203539/growth-and-endocrine-function-in-thalassemia-major-in-childhood-and-adolescence
#12
REVIEW
M Delvecchio, L Cavallo
BACKGROUND: Thalassemia major is an inherited hemoglobin disorder characterized by chronic anemia and iron overload due to transfusion therapy and gastrointestinal absorption. Iron overload causes most of the associated mortality and morbidity and frequently involves the endocrine glands. AIM: To review the most pertinent literature on the topic. METHODS: One hundred and twenty-three papers were evaluated. RESULTS: Disproportionate short stature is frequent and becomes more evident at puberty because of the lack of growth spurt...
January 2010: Journal of Endocrinological Investigation
https://www.readbyqxmd.com/read/19939538/-primary-essential-cutis-verticis-gyrata-case-report-and-literature-review
#13
REVIEW
P Dumas, V Medard de Chardon, T Balaguer, N Cardot-Leccia, J-P Lacour, E Lebreton
INTRODUCTION: Cutis verticis gyrata (CVG) is a rare and slowly progressive deformity of the scalp with thick gyrated skin folds and ridges which are similar to gyri of the brain cortex. Those folds can lead to local skin infections, to a social and cosmetic complain. CVG can be classified into two forms: primary (essential and non-essential) and secondary. To date, fifteen operated cases of primary essential CVG have been reported in the medical literature. CASE REPORT: We report the case of an 18 year-old male patient with a primary essential CVG...
June 2010: Annales de Chirurgie Plastique et Esthétique
https://www.readbyqxmd.com/read/19891915/lymphedema-praecox
#14
Carina Rizzo, Lisa M Gruson, Brent D Wainwright
A 57-year-old man presented with the post-pubertal onset of asymptomatic swelling of the left arm and legs that had been complicated by recurrent bouts of cellulitis. The presentation and disease course are consistent with lymphedema praecox, which is a subtype of primary lymphedema with onset at puberty and a slowly progressive course. The subtypes of lymphedema, pathogenesis, and treatment are reviewed.
2009: Dermatology Online Journal
https://www.readbyqxmd.com/read/19641297/growth-outcome-during-gnrh-agonist-treatments-for-slowly-progressive-central-precocious-puberty
#15
Francesco Massart, Giovanni Federico, Joshua Chuck Harrell, Giuseppe Saggese
BACKGROUND: Gonadotropin-releasing hormone agonists (GnRHa) represent the gold-standard treatment for central precocious puberty (CPP). In CPP children, GnRHa treatment slows bone age progression and preserves adult height (Ht) by suppressing sexual steroid secretion. In some patients, however, GnRHa induce an inappropriate growth deceleration impairing Ht outcome. Furthermore, slowly progressive CPP (spCPP) forms were reported which do not need GnRHa treatment. METHODS: We evaluated the growth outcome of 26 spCPP girls treated with triptorelin (TR) and 21 with leuprorelin acetate (LA) for 36...
2009: Neuroendocrinology
https://www.readbyqxmd.com/read/19339794/impact-of-aromatase-inhibitor-therapy-on-bone-turnover-cortical-bone-growth-and-vertebral-morphology-in-pre-and-peripubertal-boys-with-idiopathic-short-stature
#16
RANDOMIZED CONTROLLED TRIAL
Matti Hero, Outi Mäkitie, Heikki Kröger, Elina Nousiainen, Sanna Toiviainen-Salo, Leo Dunkel
In this randomized placebo-controlled study we examined the influence of aromatase inhibition on bone turnover, cortical bone growth, and vertebral body morphology in peripubertal boys. Thirty peripubertal boys with idiopathic short stature were treated with the aromatase inhibitor letrozole or placebo for 2 years. During treatment and posttreatment follow-up, dual-energy X-ray absorptiometry (DXA)-assessed bone mineral density, metacarpal index (MCI), and markers of bone turnover were examined. Vertebral morphology was examined by DXA after cessation of treatment...
2009: Hormone Research
https://www.readbyqxmd.com/read/19170953/reproductive-development-of-santa-in%C3%A3%C2%AAs-rams-during-the-first-year-of-life-body-and-testis-growth-testosterone-concentrations-sperm-parameters-age-at-puberty-and-seminal-plasma-proteins
#17
C E A Souza, A A Araújo, J T A Oliveira, A C Lima Souza, J N M Neiva, A A Moura
We have investigated the reproductive development of the tropically adapted Santa Inês ram, the most common hair sheep in Brazil. From 8 to 48 weeks of age, 16 animals were evaluated for body and testis growth, semen parameters, testosterone concentrations and seminal plasma proteins, using two-dimensional SDS-PAGE. Animals were weaned at 30 days and kept in feedlots thereafter, receiving hay, concentrate (18% of crude protein) and mineral supplement. Body weight increased from 12.3 +/- 0.7 to 54.3 +/- 1.6 kg between 8 and 48 weeks (p < 0...
August 2010: Reproduction in Domestic Animals, Zuchthygiene
https://www.readbyqxmd.com/read/19086040/utility-of-breast-ultrasonography-in-the-diagnostic-work-up-of-precocious-puberty-and-proposal-of-a-prognostic-index-for-identifying-girls-with-rapidly-progressive-central-precocious-puberty
#18
V Calcaterra, P Sampaolo, C Klersy, D Larizza, A Alfei, V Brizzi, F Beneventi, M Cisternino
OBJECTIVE: To determine the utility of breast ultrasono- graphy in the diagnostic work-up of precocious puberty and to create a prognostic index for early differentiation between non/slowly progressive or transient forms of precocious puberty and rapidly progressive central precocious puberty. METHODS: We recruited consecutively 60 girls with precocious pubertal development. In all the girls we evaluated Tanner stage, basal and gonadotropin-releasing hormone (GnRH)-stimulated follicle stimulating hormone (FSH) and luteinizing hormone (LH) levels, estradiol (E2) levels, and bone age, and performed pelvis and breast ultrasound examinations...
January 2009: Ultrasound in Obstetrics & Gynecology
https://www.readbyqxmd.com/read/18873866/esterases-of-testis-and-other-tissues
#19
C HUGGINS, S H MOULTON
The tissues most effective in the enzymatic hydrolysis of acyl esters of p-nitrophenol by tissue extracts of the rat and dog were the liver, lung, pancreas, renal cortex, and testis; in the dog tracheal and vesical mucosae were also esterase-rich and the lens had appreciable activity. Esterase was at low concentration in the tissues of the rat for 4 and 5 days after birth, but an increase to adult levels soon took place except in the testis where the rise was delayed until puberty. The esterase values of the blood of newborn children were also low...
August 1948: Journal of Experimental Medicine
https://www.readbyqxmd.com/read/18774024/-selective-dorsal-t1-t6-fusion-of-the-thoracic-spine-and-effects-on-thorax-growth-experimental-study-in-prepuberal-new-zealand-white-rabbits
#20
COMPARATIVE STUDY
F Canavese, A Dimeglio, M Granier, P Beraldo, F Bonnel, M Stebel, J-P Daures, B Canavese, F Cavalli
PURPOSE OF THE STUDY: The purpose of this study is to assess the consequences brought by selective dorsal arthrodesis of thoracic spine (T1-T6) to the growth of spine and thoracic volume in operated and sham-operated New Zealand White rabbits, between prepubertal age and the end of somatic growth, through the study of computerised tomography (CT) scans periodically carried out on them after arthrodesis surgery. MATERIAL AND METHODS: Nine female rabbits were subjected to surgery for selective dorsal arthrodesis of the upper thoracic spine and three were sham-operated...
September 2008: Revue de Chirurgie Orthopédique et Réparatrice de L'appareil Moteur
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