Primary hyperparathyroidism in childhood and adolescence

Objective : The aim of this study was to determine the nature of the presentation and pathology of primary hyperparathyroidism in children and adolescents and to compare these findings with adults.
Methodology : Data were obtained from the Thyroid Surgery Database at Royal North Shore Hospital on all children and adolescents undergoing parathyroid surgery. These results were then compared to data obtained from the last 100 consecutive adult parathyroidectomies.
Results : A total of eight younger patients underwent parathyroidectomy including three children (0–12 years) and five adolescents (13–18 years). A common presentation in the younger age group was hypercalcaemic crisis (50%) with a serum calcium >3.5 mmol/L and clinical signs of calcium intoxication. This compared to the adult age group where only 8% presented with crisis ( P <0.05). Eighty-eight per cent of the younger patients complained of abdominal symptoms compared to only 1% of adults. Seven of eight young patients had sporadic hyperparathyroidism.
Conclusions : Primary hyperparathyroidism, although uncommon in the younger age group, is still most commonly associated with sporadic tumours. Familial syndromes do not constitute a large percentage of patients. Younger patients are more prone to present late with abdominal symptoms, toxicity and hypercalcaemic crisis, presumably due to delayed diagnosis of an uncommon condition in this age group.     

Asthma in late adolescence – farm childhood is protective and the prevalence increase has levelled off

Wennergren G, Ekerljung L, Alm B, Eriksson J, Lötvall J, Lundbäck B. Asthma in late adolescence – farm childhood is protective and the prevalence increase has levelled off.
Pediatr Allergy Immunol 2010: 21: 806–813.
© 2010 John Wiley & Sons A/S While the prevalence of and risk factors for asthma in childhood have been studied extensively, the data for late adolescence are more sparse. The aim of this study was to provide up‐to‐date information on the prevalence of and risk factors for asthma in the transitional period between childhood and adulthood. A secondary aim was to analyze whether the increase in asthma prevalence has levelled off. A large‐scale, detailed postal questionnaire focusing on asthma and respiratory symptoms, as well as possible risk factors, was mailed to 30 000 randomly selected subjects aged 16–75 in Gothenburg and the surrounding western Sweden region. The present analyses are based on the responses from 1261 subjects aged 16–20 (560 men and 701 women). The prevalence of physician‐diagnosed asthma was 9.5%, while 9.6% reported the use of asthma medicine. In the multivariate analysis, the strongest risk factors for physician‐diagnosed asthma and other asthma variables were heredity for asthma and heredity for allergy, particularly if they occurred together. Growing up on a farm significantly reduced the prevalence of physician‐diagnosed asthma and the likelihood of using asthma medication, OR 0.1 (95% CI 0.02–0.95). Smoking increased the risk of recurrent wheeze, long‐standing cough, and sputum production. In conclusion, the prevalence of physician‐diagnosed asthma and the use of asthma medication in the 16‐ to 20‐yr age group support the notion that the increase in asthma prevalence seen between the 1950s and the 1990s has now levelled off. In line with the hygiene hypothesis, a farm childhood significantly reduced the likelihood of asthma. The adverse effects of smoking could already be seen at this young age.     

Williams syndrome: features in late childhood and adolescence

Nine children with the Williams syndrome were evaluated for physical, neurodevelopmental, and behavioral characteristics to record the natural history of this disorder. The study subjects, who ranged in age from 10 years to 20 years, generally showed lower than expected cognitive functioning with four of the nine functioning in the severely retarded range. However, all the children showed uneven developmental profiles, compared to measured IQ, with reading abilities exceeding the expected level and visual-motor skills deficient for overall performance expectations. All but one child had evidence of supravalvular aortic stenosis on echocardiography, but there was little morbidity from cardiovascular disease in this group of patients. Although all had grown at or below the fifth percentile in early childhood, seven now were above the fifth percentile for height. Personality attributes that characterize younger children with Williams syndrome persisted in this group of older children.     

Psychocutaneous disorders in childhood and adolescence

The adverse impact of skin diseases on an individual's physical and emotional well-being is well recognised, particularly in children as it is reflected in their growth and development. Conversely, the psychological and psychosocial issues that a child or young person may face, can manifest as a skin problem. Cutaneous manifestations of an underlying primary psychiatric problem are a poorly recognised entity amongst paediatricians. Limited knowledge and experience in this realm increases diagnostic and management challenges. The discipline of Psychodermatology attempts to address these problems by relating skin disease to intrinsic and extrinsic mental health factors. This review summarises the current literature on Psychocutaneous conditions with expert opinion from the authors own experience. The importance of a holistic approach in a multidisciplinary setting to manage this vulnerable group of children and young people cannot be over-emphasised.     

The radiographic features of meningiomas in childhood and adolescence

Meningiomas, although rare in the first two decades of life, must be considered in the differential diagnosis of intracranial neoplasms in this age group. Their radiographic appearance in children is sufficiently characteristic to suggest the diagnosis. Although their appearance differs little from that of adult meningiomas, childhood meningiomas are characterized by an increased frequency of calcification and higher incidence of location in the posterior fossa and within the ventricles.     

Benign paroxysmal migraine variants of infancy and childhood: Transitions and clinical features

Introduction

Migraine variant disorders of childhood include benign paroxysmal torticollis of infancy (BPTI) and benign paroxysmal vertigo of childhood (BPVC). This study aimed to review our experience with BPTI and BPVC and determine the incidence of children transitioning between each of these disorders and to vestibular migraine (VM).

Conservative surgical approach for thyroid and lymph-node involvement in papillary thyroid carcinoma of childhood and adolescence

BACKGROUND: Prior to 1990s, papillary thyroid carcinomas (PTCs) in childhood/adolescence underwent a standard therapeutic approach (total thyroidectomy plus elective neck dissection, followed by radioactive iodine (RAI) ablation), with an overall survival of about 100%. The aim of this study is to outline the possibility of a conservative approach (hemithyroidectomy plus selective neck dissection of clinically involved nodes, followed by TSH-suppressive therapy) in a selected group of patients. PROCEDURE: From 1968 to 2001, 42 pediatric PTC patients were treated at our institution. Absence of distant metastases and a tumor clinically limited to one lobe were both present in 28 cases that underwent a radical (20 cases) or a conservative (8 cases) surgical approach at the thyroid level. At cervical node level, 10 patients underwent a radical and 32 a conservative surgical approach. Clinicopathologic features at onset, type of therapy (radical vs. conservative), post-operative complications, and outcome till May 31, 2004 were recorded. The impact of the type of surgery on outcome was evaluated. RESULTS: Overall and progression-free survival (PFS) curves were found to be independent of the type of therapy (radical vs. conservative) in subgroups of patients matched for extent of disease at onset. Post-operative complications occurred only with radical surgical approaches. CONCLUSIONS: Childhood and adolescence PTCs show a high rate of spread but an excellent outcome independent of the type of therapy (radical vs. conservative). Taking into account the marked responsiveness to TSH-suppression and the complications after radical therapy, in selected cases, a conservative approach should be considered, reserving more aggressive therapies in case of metastases or relapse.     

运动神经元存活基因与儿童期脊肌萎缩症临床特征研究

目的 探讨运动神经元存活基因与儿童期脊肌萎缩症临床特征的关系。方法 回顾性分析38例疑诊为儿童期脊肌萎缩症(SMA)病例的临床资料特点及其相应的基因诊断结果。结果 不同型儿童期脊肌萎缩症临床各有特点,主要是与病情轻重和起病年龄有关。运动神经元存活基因(SMN)缺失分析(外显子7和外显子8或单纯外显子7)显示缺失为35例(92．10％),它们与疾病有关与临床分型无关。结论 运动神经元存活基因缺失的检测对脊肌萎缩症的确诊有意义,但不能协助临床分型。