Diksha Shirodkar, Najya Nasrin, Safeena Ansari Suhara, Prakash M Saldanha
{"title":"伴侣:生长激素和其他垂体激素之间的相互作用","authors":"Diksha Shirodkar, Najya Nasrin, Safeena Ansari Suhara, Prakash M Saldanha","doi":"10.25259/kpj_3_2022","DOIUrl":null,"url":null,"abstract":"Short stature is defined as a height <2 SD from the mean height for a child of the same sex, ethnicity and chronological age. We present a case series of proportionate short stature whose associated endocrinological deficits left us intrigued. A 9 ½-year-old boy presented with poor growth-velocity (GV) for 5 years and central diabetes insipidus (on the treatment for 1 year). His height was 118 cm (−2.6 SD) and his weight was 20 kg (−2.03 SD). MRI brain showed hypoplastic anterior pituitary with absent bright spot and growth hormone (GH) dynamics proved GH deficiency. A 10-year-4-month-old girl presented with poor GV. Her height was 106 cm (−4.44 SD) and her weight was 15 kg (−3.74 SD). Targeted investigations revealed multiple pituitary hormone deficiencies (central hypothyroidism, secondary adrenal insufficiency and GH deficiency). MRI brain showed reduced pituitary height with ectopic posterior pituitary. GH therapy commenced only after coverage with hydrocortisone. A 1-year-old boy was admitted with failure to gain weight and height for 4 months of age. His length was 57 cm (−7.89 SD) and weight: was 4.6 kg (−5.86 SD) with immature facies, frontal bossing and midfacial hypoplasia. Low GH values at the time of critical sample (blood glucose = 36 mg/dl) revealed GH deficiency. MRI brain demonstrated a hypoplastic pituitary gland. All proportionate short-statured children without obvious dysmorphism need detailed evaluation. GH deficiency can present as a spectrum from isolated deficiency to multiple pituitary (anterior and posterior) deficiencies and so the order of correction of the deficiencies is equally important.","PeriodicalId":217083,"journal":{"name":"Karnataka Pediatric Journal","volume":"9 1","pages":"0"},"PeriodicalIF":0.0000,"publicationDate":"2022-06-20","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":"0","resultStr":"{\"title\":\"The company that one keeps: An interplay between growth hormone and other pituitary hormones\",\"authors\":\"Diksha Shirodkar, Najya Nasrin, Safeena Ansari Suhara, Prakash M Saldanha\",\"doi\":\"10.25259/kpj_3_2022\",\"DOIUrl\":null,\"url\":null,\"abstract\":\"Short stature is defined as a height <2 SD from the mean height for a child of the same sex, ethnicity and chronological age. We present a case series of proportionate short stature whose associated endocrinological deficits left us intrigued. A 9 ½-year-old boy presented with poor growth-velocity (GV) for 5 years and central diabetes insipidus (on the treatment for 1 year). His height was 118 cm (−2.6 SD) and his weight was 20 kg (−2.03 SD). MRI brain showed hypoplastic anterior pituitary with absent bright spot and growth hormone (GH) dynamics proved GH deficiency. A 10-year-4-month-old girl presented with poor GV. Her height was 106 cm (−4.44 SD) and her weight was 15 kg (−3.74 SD). Targeted investigations revealed multiple pituitary hormone deficiencies (central hypothyroidism, secondary adrenal insufficiency and GH deficiency). MRI brain showed reduced pituitary height with ectopic posterior pituitary. GH therapy commenced only after coverage with hydrocortisone. A 1-year-old boy was admitted with failure to gain weight and height for 4 months of age. His length was 57 cm (−7.89 SD) and weight: was 4.6 kg (−5.86 SD) with immature facies, frontal bossing and midfacial hypoplasia. Low GH values at the time of critical sample (blood glucose = 36 mg/dl) revealed GH deficiency. MRI brain demonstrated a hypoplastic pituitary gland. All proportionate short-statured children without obvious dysmorphism need detailed evaluation. GH deficiency can present as a spectrum from isolated deficiency to multiple pituitary (anterior and posterior) deficiencies and so the order of correction of the deficiencies is equally important.\",\"PeriodicalId\":217083,\"journal\":{\"name\":\"Karnataka Pediatric Journal\",\"volume\":\"9 1\",\"pages\":\"0\"},\"PeriodicalIF\":0.0000,\"publicationDate\":\"2022-06-20\",\"publicationTypes\":\"Journal Article\",\"fieldsOfStudy\":null,\"isOpenAccess\":false,\"openAccessPdf\":\"\",\"citationCount\":\"0\",\"resultStr\":null,\"platform\":\"Semanticscholar\",\"paperid\":null,\"PeriodicalName\":\"Karnataka Pediatric Journal\",\"FirstCategoryId\":\"1085\",\"ListUrlMain\":\"https://doi.org/10.25259/kpj_3_2022\",\"RegionNum\":0,\"RegionCategory\":null,\"ArticlePicture\":[],\"TitleCN\":null,\"AbstractTextCN\":null,\"PMCID\":null,\"EPubDate\":\"\",\"PubModel\":\"\",\"JCR\":\"\",\"JCRName\":\"\",\"Score\":null,\"Total\":0}","platform":"Semanticscholar","paperid":null,"PeriodicalName":"Karnataka Pediatric Journal","FirstCategoryId":"1085","ListUrlMain":"https://doi.org/10.25259/kpj_3_2022","RegionNum":0,"RegionCategory":null,"ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":null,"EPubDate":"","PubModel":"","JCR":"","JCRName":"","Score":null,"Total":0}
引用次数: 0
摘要
身高矮小的定义是身高与同性别、种族和实足年龄儿童的平均身高差小于2个标准差。我们提出了一个病例系列的比例矮小的身材,其相关的内分泌缺陷让我们很感兴趣。一个9岁半的男孩,表现为生长速度差(GV) 5年,中心性尿崩症(治疗1年)。身高118厘米(- 2.6 SD),体重20公斤(- 2.03 SD)。脑MRI示垂体前叶发育不全,无亮点,生长激素(GH)动态变化提示生长激素缺乏。一名10岁4个月大的女婴表现为GV差。身高106 cm (- 4.44 SD),体重15 kg (- 3.74 SD)。有针对性的调查发现多种垂体激素缺乏(中枢性甲状腺功能减退、继发性肾上腺功能不全和生长激素缺乏)。脑MRI显示垂体高度降低,垂体后叶异位。生长激素治疗仅在氢化可的松覆盖后开始。一名1岁的男孩被承认在4个月大的时候体重和身高都没有增加。体长57 cm (- 7.89 SD),体重4.6 kg (- 5.86 SD),发育不成熟,额部隆起,面中部发育不全。在临界样品(血糖= 36 mg/dl)时,GH值较低表明GH缺乏。MRI显示脑下垂体发育不全。所有没有明显畸形的比例矮小儿童都需要详细评估。生长激素缺乏可以表现为从孤立的缺乏到多发性垂体(前侧和后侧)缺乏,因此纠正缺陷的顺序同样重要。
The company that one keeps: An interplay between growth hormone and other pituitary hormones
Short stature is defined as a height <2 SD from the mean height for a child of the same sex, ethnicity and chronological age. We present a case series of proportionate short stature whose associated endocrinological deficits left us intrigued. A 9 ½-year-old boy presented with poor growth-velocity (GV) for 5 years and central diabetes insipidus (on the treatment for 1 year). His height was 118 cm (−2.6 SD) and his weight was 20 kg (−2.03 SD). MRI brain showed hypoplastic anterior pituitary with absent bright spot and growth hormone (GH) dynamics proved GH deficiency. A 10-year-4-month-old girl presented with poor GV. Her height was 106 cm (−4.44 SD) and her weight was 15 kg (−3.74 SD). Targeted investigations revealed multiple pituitary hormone deficiencies (central hypothyroidism, secondary adrenal insufficiency and GH deficiency). MRI brain showed reduced pituitary height with ectopic posterior pituitary. GH therapy commenced only after coverage with hydrocortisone. A 1-year-old boy was admitted with failure to gain weight and height for 4 months of age. His length was 57 cm (−7.89 SD) and weight: was 4.6 kg (−5.86 SD) with immature facies, frontal bossing and midfacial hypoplasia. Low GH values at the time of critical sample (blood glucose = 36 mg/dl) revealed GH deficiency. MRI brain demonstrated a hypoplastic pituitary gland. All proportionate short-statured children without obvious dysmorphism need detailed evaluation. GH deficiency can present as a spectrum from isolated deficiency to multiple pituitary (anterior and posterior) deficiencies and so the order of correction of the deficiencies is equally important.