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Late Effects of Treatment for Childhood Brain and Spinal Tumors
Published in David A. Walker, Giorgio Perilongo, Roger E. Taylor, Ian F. Pollack, Brain and Spinal Tumors of Childhood, 2020
Ralph Salloum, Katherine Baum, Melissa Gerstle, Helen Spoudeas, Susan R. Rose
Depot GnRH analog (GnRHa) therapy is used to treat early or rapidly progressing puberty, even before or during tumor therapy. The rate of progression of secondary sexual characteristics and bone age, predicted adult height, probability of menarche before age 9 years, and psychological factors, such as immaturity or developmental delays, should all be considered in the decision to initiate treatment.93 GnRHa therapy may optimize adult height potential by slowing bone maturation.97
Endocrinology and gonads
Published in Jagdish M. Gupta, John Beveridge, MCQs in Paediatrics, 2020
Jagdish M. Gupta, John Beveridge
11.3. Congenital hypothyroidismis less common than phenylketonuria.is reliably detected by biochemical investigation in the first week of life.is reliably detected by clinical examination in the first week of life.is commonly associated with other endocrine deficiencies.can cause delay in bone maturation.
Endocrinology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Mehul Dattani, Catherine Peters
This condition is usually detected on neonatal screening. In the UK, TSH screening occurs at 5–10 days of age; in other regions screening may be T4-based. Screening has resulted in a massive decline in cretinism, and few infants now manifest clinical evidence of severe thyroxine deficiency. Clinical features, when present, include an umbilical hernia, macroglossia, constipation, feeding problems, lethargy, respiratory distress, prolonged jaundice, hoarse cry, hypotonia, coarse facies (Fig. 13.41), growth failure, abundant hair, delayed closure of fontanelles, hypothermia and a dry, mottled skin. Retarded bone maturation with delayed epiphyseal ossification and epiphyseal dysgenesis are other features. These usually appear if the diagnosis has been missed for more than 6 weeks. Other congenital malformations are present in 7% of cases.
Emerging therapies for Achondroplasia: changing the rules of the game
Published in Expert Opinion on Emerging Drugs, 2021
Smitha Kumble, Ravi Savarirayan
The phase 3 data from this study was reported in 2020 [27] (ClinicalTrials.gov number, NCT03197766) and supported previous findings. In a randomized, double-blind, placebo-controlled, multicentre trial, 121 children with achondroplasia aged 5–18 years were assigned to receive once-daily subcutaneous vosoritide at a dose of 15 μg per kilogram vs a placebo. A mean difference in annualized growth velocity of 1.57 cm was reported in favor of Vosoritide with a similar mild side effect profile. The phase 3 extension study confirmed that improvements in growth velocity was sustained for up to 2 years and resulted in significant improvement in body proportions [28]. To date, no treatment-related serious adverse effects have been reported and no adverse effects on bone maturation have been observed [28].
A new compound heterozygous mutation in a female with 17α-hydroxylase/17,20-lyase deficiency, slipped capital femoral epiphysis, and adrenal myelolipoma
Published in Gynecological Endocrinology, 2019
Fan Yang, Yongting Zhao, Jie Lv, Xia Sheng, Lihong Wang
The patient also suffered from SCFE. SCFE is a common hip disease in adolescents but is rare in adults [19–22]. SCFE is associated with growth spurts, obesity, and endocrine disorders, such as growth hormone supplementation, hypothyroidism, hypogonadism, and panhypopituitarism. However, there is no report of SCFE in patients with CAH. In our case, due to the occurrence of SCFE, a low blood potassium level was found and 17-OHD was diagnosed in this patient after a systematic examination. Therefore, screening for endocrine diseases in adult SCFE patients is crucial. The etiology of SCFE is still unclear, and the role of endocrine disruption remains uncertain. Bone maturation is delayed because of hormone deficiency in adolescent patients. Therefore, the delayed closure of the growth plate results in instability of the growth plate, causing a higher incidence of SCFE [23]. Some other authors have indicated that hormone imbalances, such as hypogonadism, hypothyroidism, and hyperparathyroidism, lead to weak bone strength [22]; these mechanisms need to be further investigated. Surgical internal fixation combined with hormonal replacement therapy is recommended for SCFE patients caused by hormonal deficiency. This patient received internal fixation and estrogen/progestin cyclic therapy and recovered well.
Evaluation of the relationship between the Demirjian and Nolla methods and the pubertal growth spurt stage predicted by skeletal maturation indicators in Turkish children aged 10–15: investigation study
Published in Acta Odontologica Scandinavica, 2019
Sevcihan Günen Yılmaz, Abubekir Harorlı, Münevver Kılıç, İbrahim Şevki Bayrakdar
Along with the onset of the pubertal period, it is generally accepted that there is a positive relationship between sexual, skeletal and somatic maturation [6]; but this positive relationship may not be the same between dental and bone maturation [5]. Wrist radiography is often used to determine bone maturation. BA with hand–wrist radiography is assessed using the Greulich–Pyle atlas [19], Tanner–Whitehouse atlas [25] and age determination of forensic medicine atlas [20] in Turkey. Pubertal growth and skeletal maturation are evaluated according to the growth and development indices defined by Fishman [6] and Björk [26]. In this study, the Greulich–Pyle atlas was used to evaluate bone age and the Fishman method was used to evaluate skeletal maturation. A large number of studies evaluating bone maturation using hand–wrist radiography have been performed [1,3,11,12,27]. Panoramic radiography is a frequently used imaging technique in the clinical practice of dentistry and allows for the assessment of dental age by evaluating the calcification stages of the seven teeth in the left mandible, developed by Demirjian [15] and Nolla [16]. A number of studies employ dental age dating using these two methods [28–33].