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Constitutional Short Stature

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61. Cost-Conscious Growth-Promoting Treatment: When Discretion Is the Better Part of Value

of long-term growth hormone treatment of idiopathic short stature. J Clin Endocrinol Metab. 2005 Sep; 90(9): 5247–53. 26 Salehpour S, Alipour P, Razzaghy-Azar M, Ardeshirpour L, Shamshiri A, Monfared MF, et al. A double-blind, placebo-controlled comparison of letrozole to oxandrolone ef- fects upon growth and puberty of children with constitutional delay of puberty and idio- pathic short stature. Horm Res Paediatr. 2010; 74(6): 428–35. 27 Schroor EJ, van Weissenbruch MM, Knibbe P, Delemarre-van de (...) height is complicated by uncer- tainty about how best to measure its therapeutic effect. Cost-conscious growth promotion practice, however, is pos- sible and likely an emerging practical requisite as health care payers increasingly deny the medical necessity of and re- strict support for short stature treatment. The increase in de- nials is not surprising given the expansion and continued high cost of hGH treatment, debate about the value of such treatment, and universal need to restrain burgeoning

2018 Pediatric Endocrine Society

62. Increased N-methyl histamine with eosinophilia

of pollen allergy and mild asthma, none of which were active at the time of her visit. Skin testing to Northeastern aeroallergens was completely negative with good positive and negative controls, and her PFT’s were somewhat decreased, however, she was of very short stature and had scoliosis, at least some if which was related to her genetic abnormality. I sent her to pulmonary and her complete PFT’s were found to be unremarkable based on her body habitus. She has been diagnosed with sleep apnea (...) is, what’s with the n-methylhistamine when there is no evidence of either cutaneous or systemic mastocytosis? I’m not really sure what to do with the information I have. A: The short answer is I would not recommend any additional evaluation of the increase in urinary histamine and no other evidence of mastocytosis or other mast cell disorder. The most common cause of eosinophilia in my experience is secondary to medication so would review medications, particularly NSAIDs, to determine if any may

2020 American Academy of Allergy, Asthma & Immunology - Ask the Expert

63. Elosulfase alfa for treating mucopolysaccharidosis type Iva

paralysed or die. Bone and joint problems often cause chronic pain. Progressive hearing loss and frequent periods of diarrhoea can be debilitating and isolating. MPS IVa causes reduced life expectancy; the average life expectancy in people with this condition is about 25 years. The combination of symptoms in MPS IVa, including physical features and short stature, can cause considerable anxiety, depression and low self-esteem. Expensive wheelchairs and home adaptations carry a financial burden, which can (...) to the circumstances of the individual patient, in consultation with the patient and/or their carer or guardian. Commissioners and/or providers have a responsibility to provide the funding required to enable the guidance to be applied when individual health professionals and their patients wish to use it, in accordance with the NHS Constitution. They should do so in light of their duties to have due regard to the need to eliminate unlawful discrimination, to advance equality of opportunity and to reduce health

2016 National Institute for Health and Clinical Excellence - Highly specialised technology

64. Faltering growth: recognition and management of faltering growth in children.

short stature (see recommendation above) Rapid weight loss or severe undernutrition Features that cause safeguarding concerns (see the NICE guideline on ). Organisation of Care Ensure there is a pathway of care for infants and children where there are concerns about faltering growth or weight loss in the early days of life that: Clearly sets out the roles of healthcare professionals in primary and secondary care settings Establishes and makes clear the process for referral to and coordination (...) , based on the assessment, advise on food choices for infants and children that: Are appropriate to the child's developmental stage in terms of quantity, type and food texture Optimise energy and nutrient density. In infants or children who need a further increase in the nutrient density of their diet beyond that achieved through advice on food choices, consider: Short-term dietary fortification using energy-dense foods Referral to a paediatric dietitian. Advise the parents or carers of infants

2017 National Guideline Clearinghouse (partial archive)

65. Interventions Targeting Sensory Challenges in Children with Autism Spectrum Disorder - An Update

high risk of bias [ROB]). Populations, intervention approaches, and outcomes assessed varied across studies. Relative to usual care or other interventions, sensory integration–based approaches improved measures related to sensory and motor skills in the short term (3 RCTs with high, moderate, and low ROB and 1 high ROB retrospective cohort study). Environmental enrichment improved nonverbal cognitive skills in treated children compared with standard care in two small RCTs (low and moderate ROB (...) and high ROB) of interventions with sensory-related components (tactile stimulation exercises, weighted blankets) reported few significant differences between treatment groups. Conclusions. Some interventions targeting sensory challenges may produce modest short-term ( 70), both groups received active treatment that included either SI therapy or eclectic group therapy. 39 Treatment lasted for 8 to 10 months. Participants in the SI group improved significantly more than those in the control group

2017 Effective Health Care Program (AHRQ)

67. Obesity in Adolescents

in preventing short-term and long-term morbidity. The obstetrician–gynecologist who is knowledgeable about the behavioral and environmental factors that influence obesity may be better able to educate parents, guardians, and adolescents and advocate for programs that increase physical activity and improve nutrition. The obstetrician–gynecologist should be able to identify obese adolescents, particularly those at risk of comorbid conditions. They may have the opportunity to initiate behavioral counseling (...) , and psychologist or psychiatrist, should be used to select appropriate candidates for surgical intervention and provide postoperative support. Introduction Rates of obesity among adolescents in the United States have increased at a dramatic rate along with the prevalence of weight-related diseases. Because the obese female adolescent faces medical, psychologic, and reproductive health challenges, early intervention is imperative in preventing short-term and long-term morbidity. The obstetrician–gynecologist

2017 American College of Obstetricians and Gynecologists

69. European Society of Endocrinology Clinical practice guidelines for the care of girls and women with Turner syndrome

of the literature. These four questions related to the efficacy and most optimal treatment of short stature, infertility, hypertension, and hormonal replacement therapy. The guidelines project was initiated by the European Society of Endocrinology and the Pediatric Endocrine Society, in collaboration with the European Society for Paediatric Endocrinology, the Endocrine Society, the European Society of Human Reproduction and Embryology, the American Heart Association, the Society for Endocrinology (...) review of the literature. These four questions related to the efficacy and most optimal treatment of short stature, infertility, hypertension, and hormonal replacement therapy. The guidelines project was initiated by the European Society of Endocrinology and the Pediatric Endocrine Society, in collaboration with the European Society for Paediatric Endocrinology, the Endocrine Society, the European Society of Human Reproduction and Embryology, the American Heart Association, the Society

2017 European Society of Endocrinology

70. Clinical practice guidelines for the care of girls and women with Turner syndrome: proceedings from the 2016 Cincinnati International Turner Syndrome Meeting Full Text available with Trip Pro

systematic review of the literature. These four questions related to the efficacy and most optimal treatment of short stature, infertility, hypertension, and hormonal replacement therapy. The guidelines project was initiated by the European Society of Endocrinology and the Pediatric Endocrine Society, in collaboration with the European Society for Paediatric Endocrinology, the Endocrine Society, the European Society of Human Reproduction and Embryology, the American Heart Association, the Society (...) with a separate systematic review of the literature. These four questions related to the efficacy and most optimal treatment of short stature, infertility, hypertension, and hormonal replacement therapy. The guidelines project was initiated by the European Society of Endocrinology and the Pediatric Endocrine Society, in collaboration with the European Society for Paediatric Endocrinology, the Endocrine Society, the European Society of Human Reproduction and Embryology, the American Heart Association

2016 European Society of Human Reproduction and Embryology

71. Kanuma - sebelipase alfa

Time PV Process Validation qow Every-other-week qw Once weekly rhLAL Recombinant human lysosomal acid lipase SD Standard deviation SE Standard error SF-36 36 item Short Form Health Survey SMA Smooth muscle actin SmPC Summary of product characteristics SPF specific pathogen free TFHN Transfusion-free haemoglobin normalisation TPN Total parenteral nutrition UAS Unformulated active substance UFDF Ultrafiltration Diafiltration UK United Kingdom ULN Upper limit of normal US United States WFA Weight (...) , integration of a single copy of the transgene into Gallus gallus and the correct hLAL sequence. Of the transgene G1 generation, 3 males with the same genetic characteristics of the transgene were selected for generation of the G2 transgene progeny. Transgene G2 hens were the first animals to constitute the Production Line, after a match of the genetic characteristics with that of G1 hen had been reconfirmed for a proportional number of animals. The production line includes all hens of G2 and following

2015 European Medicines Agency - EPARs

72. Sivextro - tedizolid phosphate

tests for this kind of dosage form appearance, identification (IR and HPLC), assay (HPLC), degradation products (HPLC), uniformity of dosage units by weight variation (Ph. Eur.), loss on drying (Ph. Eur.), bacterial endotoxins (Ph. Eur.) and sterility (Ph. Eur.). Additional tests are carried out on the reconstituted solution: reconstitution time (visual), constituted solutions (clarity and degree of opalescence of liquids, degree of coloration of liquids, particulate contamination: visible particles (...) were also provided. Samples were tested for appearance, reconstitution time, constituted solutions pH, particulate matter (sub-visible particles), assay, degradation products, loss on drying and chiral purity, sterility and bacterial endotoxins. The stability data indicate that there were no significant trends or variability in the parameters tested. In addition, forced degradation studies (under heat, light, acid, base, and oxidation conditions) and a photostability study conducted in line

2015 European Medicines Agency - EPARs

73. Guideline on the management of premature ovarian insufficiency

and to keep it up-to-date, it cannot, however, guarantee the correctness, completeness, and accuracy of the guideline in every respect. In any event, these clinical practice guidelines do not necessarily represent the views of all clinicians that are member of ESHRE. The information provided in this document does not constitute business, medical or other professional advice, and is subject to change. 3 CONTENTS Introduction to the Guideline 5 Summary 7 Interpretation on the grades of recommendations 7 (...) be made to reduce the incidence of POI. Modifiable factors may include: ? gynaecological surgical practice ? lifestyle – smoking ? modified treatment regimens for malignant and chronic diseases. References Albright F, Smith P, Fraser R. A syndrome characterized by primary ovarian insufficiency and decreased stature. Am J Med Sci 1942;204: 625- 648. Alexandru Dan Corlan. Medline trend: automated yearly statistics of PubMed results for any query, 2004. . Retrieved 13 november 2015, from http

2015 European Society of Human Reproduction and Embryology

74. Genetics of Skin Cancer (PDQ®): Health Professional Version

, vertebral abnormalities (hemivertebrae, fusion or elongation of vertebral bodies), modeling defects of the hands and feet, or flame-shaped lucencies of hands and feet Bridging of sella turcica, vertebral abnormalities (hemivertebrae, fusion or elongation of vertebral bodies), modeling defects of the hands and feet Skeletal malformations (vertebral, short 4th metacarpals, postaxial polydactyly) (Rib abnormalities listed as major criterion; see above.) (Rib abnormalities listed as major criterion; see

2018 PDQ - NCI's Comprehensive Cancer Database

75. Childhood Cancer Genomics (PDQ®): Health Professional Version

constituted a favorable-risk group while patients with PTEN or RAS mutations, regardless of NOTCH1/FBXW7 status, have a significantly higher risk of treatment failure.[ , ] In the FRALLE study, 5-year cumulative incidence of relapse and disease-free survival (DFS) were 50% and 46% for patients with mutated NOTCH1 / FBXW7 and mutated PTEN / RAS versus 13% and 87% for patients with mutated NOTCH1 / FBXW7 and wild-type PTEN / RAS .[ ] The overall 5-year DFS in the FRALLE study was 73%, and additional

2018 PDQ - NCI's Comprehensive Cancer Database

76. Late Effects of Treatment for Childhood Cancer (PDQ®): Health Professional Version

:[ , , ] A short latency (<10 years from primary cancer diagnosis). The risk of t-MDS/AML plateaus after 10 to 15 years. Although the risk of subsequent leukemia remains significantly elevated beyond 15 years from primary diagnosis (standardized incidence ratio [SIR], 3.5; 95% CI, 1.9–6.0), these events are relatively rare, with an absolute excess risk of 0.02 cases per 1,000 person-years.[ ] An association with alkylating agents and/or topoisomerase II inhibitors. t-MDS/AML is a clonal disorder characterized

2018 PDQ - NCI's Comprehensive Cancer Database

77. Genetics of Breast and Gynecologic Cancers (PDQ®): Health Professional Version

of this summary for more information.) Oral contraceptives Oral contraceptives (OCs) may produce a slight increase in breast cancer risk among long-term users, but this appears to be a short-term effect. In a meta-analysis of data from 54 studies, the risk of breast cancer associated with OC use did not vary in relationship to a family history of breast cancer.[ ] OCs are sometimes recommended for ovarian cancer prevention in carriers of BRCA1 and BRCA2 pathogenic variants. (Refer to the section (...) tested for BRCA1/BRCA2 pathogenic variants.[ ] Short-term use of hormones for treatment of menopausal symptoms appears to confer little or no breast cancer risk.[ , ] The effect of HRT on breast cancer risk among carriers of BRCA1 or BRCA2 pathogenic variants has been studied in the context of bilateral risk-reducing oophorectomy, in which short-term replacement does not appear to reduce the protective effect of oophorectomy on breast cancer risk.[ ] (Refer to the section of this summary for more

2018 PDQ - NCI's Comprehensive Cancer Database

78. Amenorrhoea

) and luteinizing hormone (LH) If secondary sexual characteristics are absent, karyotyping in secondary care may be necessary. Short stature and high FSH and LH levels suggest Turner's syndrome. Short stature and low FSH and LH levels suggest an intracranial lesion for example, hydrocephalus. Normal height and high FSH and LH levels suggest ovarian failure (normal karyotype) or 46XY (abnormal karyotype). Normal height and low FSH and LH levels suggest constitutional delay, weight loss, anorexia nervosa (...) suggest an eating disorder or the ). Examine for: Features of Turner's syndrome (short stature, web neck, shield chest with widely spaced nipples, wide carrying angle, and scoliosis). Features of Cushing's syndrome (striae, buffalo hump, significant central obesity, easy bruising, hypertension, and proximal muscle weakness). Hirsutism and acne (suggesting PCOS, especially in those with a high BMI). See the CKS topic on for more information. Features of thyroid and other endocrine disease. See the CKS

2018 NICE Clinical Knowledge Summaries

79. Bites - human and animal

of age) [ ], and boys and men are more frequently affected [ ]. Older children experience more bites to the arms and legs, whereas younger children have an increased likelihood of being bitten on the head, face or neck because of their short stature and relatively large head compared with their body [ ; ; ]. There is an increased incidence of admissions related to dog bites in the summer months compared with winter [ ]. Around half of dog bites are reported as provoked [ ] and 90% were inflicted (...) if there is a report or appearance of an animal bite on a child who has been inadequately supervised. Refer for further assessment in line with local policy. Assess the person's . Basis for recommendation Basis for recommendation Documenting how and when the bite occurred This recommendation is based on expert opinion in review articles [ ; ; ]. Cat bites involve deep puncture wounds which can cause severe infections within a short time (median 12 hours), and an unprovoked animal bite increases suspicion of rabies

2018 NICE Clinical Knowledge Summaries

80. Faltering growth

children with faltering growth and: Symptoms or signs suggestive of an acute or chronic underlying condition. Rapid weight loss or severe under-nutrition. Safeguarding concerns. Slow linear growth or unexplained short stature. Management in primary care has failed. If weight loss is less than 10% of birth weight in the early days of life and assessment is normal: The parent/carer can be reassured that this is common and weight loss usually stops after about 3–4 days of life with most babies returning (...) not returned to their birth weight by 3 weeks of age: Refer to/discuss with paediatrics, with urgency dependant on the clinical situation, all infants: With symptoms or signs suggestive of an acute or chronic underlying condition. With rapid weight loss or severe under-nutrition. With safeguarding concerns — for further information see the CKS topic on . With slow linear growth or unexplained short stature. Who have not responded to management in primary care. Ensure that parents/carers have adequate

2018 NICE Clinical Knowledge Summaries

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