Slowly progress puberty

OBJECTIVE: There is a scarcity of data from randomised controlled trials on the association of growth hormone (GH) with gonadotrophin-releasing hormone agonists in idiopathic short stature (ISS), although this off-label use is common. We aimed to test whether delaying pubertal progression could increase near-adult height (NAH) in GH-treated patients with ISS. METHODS: Patients with ISS at puberty onset were randomised to GH with leuprorelin (combination, n  = 46) or GH alone ( n  = 45)...

BACKGROUND: Fibrous dysplasia is a slowly progressive benign fibro-osseous disorder that involves one or multiple bones with a unilateral distribution in most cases. It is a lesion of unknown etiology, uncertain pathogenesis, and diverse histopathology. Temporal bone involvement is the least frequently reported type, especially in children. We reviewed available articles regarding fibrous dysplasia with temporal bone involvement in children and added four patients aged 7 to 17 years who were diagnosed and treated in our institution from 2006 to 2017...

The prevailing view is that type 1 diabetes (T1D) develops as a consequence of a severe decline in β-cell mass resulting from T-cell-mediated autoimmunity; however, progression from islet autoantibody seroconversion to overt diabetes and finally to total loss of C-peptide production occurs in most affected individuals only slowly over many years or even decades. This slow disease progression should be viewed in relation to the total β-cell mass of only 0.2 to 1.5 g in adults without diabetes. Focal lesions of acute pancreatitis with accumulation of leukocytes, often located around the ducts, are frequently observed in people with recent-onset T1D, and most patients display extensive periductal fibrosis, the end stage of inflammation...

STUDY OBJECTIVE: To investigate the potential of serum anti-Müllerian hormone (AMH) and inhibin B (INHB) levels as markers for pubertal progression rate in girls with central precocious puberty (CPP). DESIGN, SETTING, PARTICIPANTS, INTERVENTIONS, AND MAIN OUTCOME MEASURES: A total of 148 girls were enrolled, including 65 girls with premature thelarche and 83 girls with CPP, grouped on the basis of the results of gonadotropin-releasing hormone stimulation tests. Girls with CPP underwent a 6-month follow-up, and were further divided into 2 subgroups: the progressive CPP (P-CPP) group (n = 55) and the slowly P-CPP (SP-CPP) group (n = 28)...

In vertebrates, male-typical sexual behavior (MSB) is largely controlled by gonadal androgens, however, the mechanism of this control is believed to vary among species. During immediate activation MSB is tightly correlated with circulating levels of androgens, while the organization of MSB by a hormonal event at a specific developmental period, early in ontogeny or during puberty, has been postulated in other lineages. Here, we put forward an alternative concept of "temporal organization". Under temporal organization longer exposure to circulating androgens is needed for the onset of MSB, which can continue for a long time after the levels of these hormones drop...

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...

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...

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...

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...

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...

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...

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...

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...

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...

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...

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...

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...

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...

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.

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...