Management of Pituitary Gigantism

ABSTRACT. True gigantism with overproduction of growth hormone (GH) and prolactin (PRL) was diagnosed in two boys, aged 13 years (case I) and 7 ½ years (case II). Both had shown increased growth rates since early childhood (from 4 years and 1 ½ years, respectively), but no skeletal acromegalic features were noted. However, both showed increased sweating and both had advanced pubic hair relative to testis volume. No other pituitary dysfunction was recorded. Case I underwent transsphenoidal surgery with only incomplete and temporary suppression of GH and PRL levels. However, in both patients bromocriptine administration promptly suppressed PRL levels. Following combined irradiation and bromocriptine treatment, GH also gradually normalized over a period of 2 years. Both boys are still on treatment, and both showed an increase in plasma GH concentrations when the dose of bromocriptine was reduced or discontinued, indicating that even 3 V2-5 years after irradiation therapy (and during continuous treatment with bromocriptine) the disease was controlled but not cured. However, in these two boys bromocriptine has proved effective in controlling the PRL/GH oversecretion.     

Ectopic Posterior Lobe of the Pituitary Gland and Intractable Nocturnal Enuresis in a Case with Pituitary Dwarfism

ABSTRACT. An 11 5/12-year-old girl with pituitary dwarfism had intractable nocturnal enuresis. Magnetic resonance imaging revealed pituitary stalk transection and the formation of an ectopic posterior lobe. The results of responses to dehydration, infusion of hyperosmolar NaCI solution, and l-desamino-8-D-arginine vasopressin showed that the nocturnal enuresis in the present case was due to a partial vasopressin deficiency. We suggest that the ectopic posterior lobe is one cause of nocturnal enuresis.     

Pituitary Enlargement, Hypertrichosis and Blunted Growth Hormone Secretion in Primary Hypothyroidism

ABSTRACT. Pituitary hyperplasia, hypertrichosis and blunted growth hormone (GH) secretion were observed in three children with untreated primary hypothyroidism. These abnormalities disappeared and improved after thyroid hormone therapy. The recognition of these associations may eliminate unnecessary surgery and GH therapy and lead to the choice of thyroid hormone replacement therapy.     

Increased Prolactin Level and Pituitary Adenoma as a Cause of Headache in two Patients with Sickle Cell Disease

In two patients with sickle cell disease who presented with headache pituitary adenoma and high levels of serum prolactin were found. Treatment with bromocriptine has controlled the headache. We believe that prolactin level determination should be part of the work-up of patients with sickle cell disease who complain of headache.     

矮小儿童下丘脑-垂体及其胰岛素样生长因子轴功能检查的意义

目的检测矮小儿童下丘脑-垂体及其胰岛素样生长因子(IGF-1)生长轴(GHRH-GH-IGF-1)功能,了解矮小儿童的发病因素及确定下丘脑-垂体及其IGF轴功能缺陷病因分类。方法矮小儿童30例。用统一印制的矮小儿童表格记录其临床特征。对矮小儿童进行甲状腺功能测定;用胰岛素 左旋多巴行生长激素(GH)刺激试验;放射免疫法测定血清IGF-1和血清胰岛素样生长因子结合蛋白-3(IGFBP-3)水平;同时行患儿骨龄、垂体增强MRI扫描、染色体核型分析、性激素测定。根据矮小症诊断标准和2004年Rosenfeld RG和GHRH-GH-IGF-1轴缺陷不同,将矮小儿童进行病因定位和分类。结果矮小儿童30例中,下丘脑-垂体及其IGF轴功能缺陷12例,占40%,其中肯定生长激素缺乏(GHD)4例,怀疑生长激素不敏感综合征2例,可疑GHD 6例。Turner′s综合征2例,占6.67%;体质性青春期延迟2例,占6.67%;特发性矮小14例,占46.6%。磁共振发现垂体微腺瘤2例;垂体发育不良12例。结论1.矮小儿童所占比例最大的为特发性矮小,其次为下丘脑-垂体及其IGF轴功能缺陷。2.IGF-1水平和IGFBP-3水平与生长激素刺激试验测定生长激素水平不一致,考虑存在生长激素抵抗和受体缺陷。3.矮小儿童可能存在先天性垂体发育异常,致使垂体分泌生长激素不足。     

Adrenal hypoplasia and pituitary agenesis in a normocephalic infant, with a review of the literature

Abstract A case of adrenal hypoplasia and pituitary agenesis in a normocephalic female infant is described. Twenty-eight other cases from the world literature were reviewed. There were 18 females and 11 males. More than one affected infant was described in five families, suggesting autosomal recessive inheritance. The mode of presentation, subsequent course, pathology and possible pathogenesis are discussed.     

Clinical significance of pulmonary nodules detected by CT and Not CXR in patients treated for favorable histology Wilms tumor on national Wilms tumor studies-4 and -5: a report from the Children's Oncology Group

Background

Metastatic lung disease in Wilms tumor (WT) patients was traditionally identified by chest radiograph (CXR). It is unclear whether patients with small lesions, detectable only by computed tomography (“CT‐only” lesions), require the more intensive therapy, including doxorubicin and lung irradiation, given to patients with metastases detectable by CXR.

Procedures

This study involved 417 patients with favorable histology WT and isolated lung metastases (detected by CXR or CT) who were registered on National Wilms tumor Study (NWTS)‐4 or ‐5. Outcomes by method of detection (CXR vs. CT‐only), use of lung radiation, and 2‐ or 3‐drug chemotherapy (dactinomycin and vincristine ± doxorubicin) were determined and compared using the log‐rank test.

Results

There were 231 patients with lung lesions detected by CXR and 186 by CT‐only. Of the patients with CT‐only nodules, 37 received only 2 drugs and 101 did not receive lung radiation. Five‐year event‐free survival (EFS) was greater for patients receiving three drugs (including doxorubicin) with or without lung radiation than for those receiving two drugs (80% vs. 56%; P = 0.004). There was no difference seen in 5‐year overall survival (OS) between the 3‐ and 2‐drug subsets (87% vs. 86%; P = 0.91). There were no significant differences in EFS (82% vs. 72%; P = 0.13) or OS (91% vs. 83%; P = 0.46) for patients with CT‐only nodules whether they received lung radiation or not.

Conclusions

Our results suggest that patients with CT‐only lung lesions may have improved EFS but not OS from the addition of doxorubicin but do not appear to benefit from pulmonary radiation. Pediatr Blood Cancer 2012;59:631–635. © 2012 Wiley Periodicals, Inc.     

生长激素缺乏症患儿垂体MRI检查的临床应用价值

目的 研究特发性生长激素缺乏症(GHD)儿童垂体MRI检查的临床应用价值,为进一步探索GHD病理机制提供临床依据.方法 选取100例2005-2007年内分泌专科就诊的GHD儿童乖体MRI资料,其中男74例,女26例;平均年龄为(8.82±3.68)岁.于SE序列T1WI头颅正中矢状及冠状面上观测垂体大小形态及信号特征,并比较其与临床的联系.结果 在10～15岁GHD患儿头颅MRI检查垂体矢状高径明显优于其他各径线(P<0.01);在联合垂体功能缺陷(MPHD)中垂体后叶异位(EPP)的发生率(92.3%)显著高于CHD(7.7%,P<0.01).结论 对GHD儿童应重视头颅MRI检查,其垂体形态、结构的阳性发现可有助于临床疾病的诊断及鉴别诊断,必要时应随访MRI,结合临床综合判断诊治及其预后.     

The Validity of Mixed Disorders of Conduct and Emotions in Children and Adolescents: A Research Note

In a study of differential validity, three diagnostic groups based on ICD-10 criteria were compared, namely children with emotional disorders, children with conduct disorders, and children with mixed disorders of conduct and emotions. The patients were taken from a consecutive series of child and adolescent psychiatric service attenders from 1988 to 1992 and represented the total entire subgroups of patients with the respective diagnoses. Based on standardized item sheets that were filled out by the clinicians who were responsible for the respective cases, the groups were compared with regard to age, sex ratio, family background, premorbid behavioral abnormalities, abnormal psychosocial situations, and treatment. It was found that children with mixed disorders of conduct and emotions share many characteristics with pure conduct disordered children. It is concluded that the former group is a subgroup of the latter, and there is little evidence in this study supporting the validity of a separate category of mixed disorders of conduct and emotions.     

High prevalence of eosinophilia in growth hormone-deficient children

BACKGROUND: To determine the clinical significance of eosinophilia in growth-hormone (GH)-deficient children, a clinical study consisting of 72 children and adolescents (mean age 9 years and 6 months at diagnosis) with GH deficiency (GHD) was undertaken. Patients were treated with GH, along with supplementation for the combined deficiency in patients with multiple hormone deficiency. METHODS: A complete blood count and hemogram with microscopic examination of a peripheral blood smear was performed. RESULTS: Before treatment, differential eosinophil counts exceeded 5% in 30 subjects (41.7%) and absolute eosinophil counts were >350 /microL in 27 subjects (37.5%). Growth hormone therapy did not significantly affect eosinophil counts. There was an inverse relationship between absolute eosinophil count and peak GH value in response to the L-dopa stimulation test (n=65; Rs=-0.252; P=0.044). CONCLUSIONS: For the diagnosis of GHD, one should take into account that GH response to L-dopa stimulation can be selectively blunted in patients with eosinophilia.     

CT and MRI in the diagnosis of tuberculosis

The advent of CT and MRI imaging in the last two decades has redefined the approach and analysis of various diseases including tuberculosis. Tuberculosis afflicts hollow and solid viscera. Genitourinary, hepatobiliary and adrenal tuberculosis is uncommon in children. CT and MRI have however shown several advantages over conventional radiology and other imaging modalities in early diagnosis and follow-up of tuberculosis in different parts of the body.     

CT and MRI in the diagnosis of tuberculosis

The advent of CT and MRI imaging in the last two decades has redefined the approach and analysis of various diseases including tuberculosis. Tuberculosis afflicts hollow and solid viscera. Genitourinary, Hepatobiliary and Adrenal tuberculosis is uncommon in children. CT and MRI have however shown several advantages over conventional radiology and other imaging modalities in early diagnosis and follow-up of tubeculosis in different parts of the body.     

成纤维细胞生长因子-7在除草醚诱导的先天性膈疝大鼠模型胎肺中的表达

目的研究成纤维细胞生长因子-7（FGF-7）在先天性膈疝（CDH）胎肺中的表达及探讨其在CDH肺发育不良中的作用。方法采用免疫组织化学和图像分析方法半定量地检测FGF-7在胎肺中的表达部位及表达相对含量。结果CDH肺发育不良，处于假腺体期。FGF-7在对照组显示强表达于支气管和细支气管上皮细胞，在除草醚（nitrofen）组中表达微弱，其表达的相对含量显著低于对照组，Nitrofen组中CDH组和noCDH组间差异没有显著性意义。FGF-7的表达与肺泡面积呈显著正相关性，与肺泡间隔呈显著负相关性。结论FGF-7在CDH胎肺中表达降低，且降低具有组织特异性；FGF-7可作为提示CDH胎肺成熟度的重要指标；FGF-7表达降低可能是CDH肺发育不良形成机制之一。     

A prospective study to evaluate the depictability of the hepatic veins on abdominal contrast-enhanced CT in small children

Background  The optimal conditions for abdominal contrast-enhanced (CE) CT, especially with respect to depiction of the hepatic veins (HVs), have not been well studied in small children. Objective  We compared the depiction of the HVs in small children using scan delay times of 50 s and 60 s and a power injector. The degree of contrast enhancement of the HVs according to body weight was also evaluated. Materials and methods  A total of 50 CT studies in 31 children ages 2 days to 3 years 4 months (median 12 months) with a body weight less than 15 kg were prospectively evaluated. Nonionic contrast medium (300 mgI/ml) at a dose of 2 ml/kg and an injection rate calculated as (body weight in kilograms × 0.1) ml/s was injected through a 24-gauge intravenous catheter. Scan delay time was randomly chosen at 50 s or 60 s (25 times each). Subjective assessment of HV depiction was performed and the difference in CT number between the HV and hepatic parenchyma (HV−HP value) was calculated. Results  In all cases, subjective assessment of HV depiction was good and the HV−HP value was greater than 20 HU. The mean±SD HV−HP value was 47.7±13.2 HU. There were no differences in the mean HV−HP values between scan delay times of 50 s and 60 s (P=0.58). Conclusion  For small children, this protocol for pediatric abdominal CECT resulted in good depiction of the HVs with no difference between scan delay times of 50 s and 60 s.     

Growth Response in Prepubertal Children with Idiopathic Growth Hormone Deficiency during the First Year of Treatment with Human Growth Hormone. Analysis of the Kabi International Growth Study.

ABSTRACT. In order that children with growth hormone deficiency (GHD) reach the goal of normal adult stature, treatment modalities need to be optimized. From the large database of patients enrolled in the Kabi International Growth Study (KIGS), 257 prepubertal patients with idiopathic GHD undergoing their first year of growth hormone (GH) substitution therapy were selected. A multiple regression analysis was performed to determine both auxiological factors characterizing the patients and the factors related to the chosen treatment modalities which are of significance for the observed magnitude of the growth response. Due to the structure of the data, pretreatment height velocity and bone age-derived auxiological data were not considered. It was observed that the magnitude of the growth response was inversely correlated with chronological age and relative height (HT SDS) at the start of GH treatment but was positively correlated with mid-parental height. The growth response was also positively correlated with the GH dose (IU/kg/week) and the frequency of GH injections per week. A regression equation using these five parameters was derived, allowing the growth response of these patients to be predicted. The extension of this analytical approach in the future will allow the treatment of patients with GHD to be tailored to individual requirements.     

A practical approach to the assessment of faltering growth in the infant and toddler

Faltering growth, previously called failure to thrive, in infants and toddlers is a common primary care presentation and reason for paediatric referral. This article suggests an approach to selection of cases for referral, the assessment and investigation required in secondary care and the identification of the less than 5% of cases with an underlying organic disease. A framework is suggested to aid advice to be given to parents with a child who has faltering growth due to inadequate nutritional intake for their energy requirements.     

Effect on lung growth of cervical cord section in the rabbit fetus

Experiments were performed to clarify the mechanism by which cervical cord transection retards lung growth in the fetal rabbit. In 10 sets of fetuses operated on at 24 1/2 days gestation and studied 3--4 days later, cord section at C1--C3 (high section) caused a significantly greater reduction in lung weight and lung DNA than cord section at C5--C8 (low section) as compared with control littermates. Comparison with the lungs of additional control fetuses removed at the time of operation showed that high section had reduced lung growth by 70% and low section had reduced growth by 40% relative to sham-operated controls. The hypoplastic lungs of the high-section group had poorly expanded, thick-walled terminal sacs, while those of the low section group more nearly resembled the controls. Fetal weights and weights of liver, kidneys, thymus and diaphragm did not differ significantly between the groups, but the hearts of the low-section group were unduly large. In a separate 6 sets of fetuses tracheal ligation at the time of high-cord section was found to result in large fluid-filled lungs with a normal DNA content. The results indicate that preservation of an upper motor neurone supply to the phrenic nucleus is of critical importance for fetal lung growth, and confirm the growth-promoting effects of liquid distension of the fetal lungs. We conclude that normal fetal lung growth depends on development and maintenance of a sophisticated form of function involving integration of respiratory movements and lung lipid secretion. This functional control of fetal lung growth has important implications for perinatal medicine.     

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目的探讨螺旋CT虚拟内诊镜(CTVE)在小儿气管、支气管异物诊断上的应用价值。方法回顾性分析东南大学医学院附属徐州医院1998年1月至2004年12月收治的121例术前经CTVE诊断为气管支气管异物,并行支气管镜探查术患儿的诊疗情况,并分析CTVE诊断在临床应用上的意义及诊断的阳性符合率。结果121例经CTVE诊断并行支气管镜探查证实并取除异物者115例,诊断符合率95.04%。结论CTVE检查是目前无创非侵入性诊断小儿气管支气管异物的较理想方法。