A clinical approach to parasellar lesions in the transition age.

Many reviews have summarised the pathology and management of the parasellar region in adult patients, although an analysis of these aspects in the transition years, from puberty onset to the age of peak bone mass, has been lacking. A comprehensive search of English-language original articles, published from 2000 to 2020, was conducted in the MEDLINE database (December 2019 to March 2020). We selected all studies regarding epidemiology, diagnosis and management of the following parasellar lesions: germinoma, craniopharyngioma, Langerhans cell histiocytosis, optic glioma, hypothalamic hamartoma, tuber cinereum hamartoma, cranial chordoma, Rathke cleft cyst, hypophysitis and hypothalamitis during the transition age from childhood to adulthood. In the present review, we provide an overview of the principal parasellar lesions occurring in the transition age. Symptoms are usually a result of the mass effect of the lesions on nearby structures, as well as anterior pituitary deficits. Diabetes insipidus occurs frequently in these patients. In this age group, pubertal developmental disorders may be more evident compared to other stages of life. Parasellar lesions in the transition age mostly include neoplastic lesions such as germinomas, hamartomas, optic gliomas, craniopharyngiomas Langerhans cell histiocytosis and chordomas, and rarely inflammatory lesions (hypophysitis, hypothalamitis). There are limited data on the management of parasellar lesions in the transition age. Endocrine evaluation is crucial for identifying conditions that require hormonal treatment so that they can be treated early to improve the quality of life of the individual patient in this complex age range. The clinical approach to parasellar lesions involves a multidisciplinary effort.

Unusual Glycemic Presentations in a Child with a Novel Heterozygous Intragenic INSR Deletion.

BACKGROUND: Mutations of the insulin receptor (INSR) gene lead to a wide spectrum of inherited insulin resistance (IR) syndromes. Among these, type A-IR, usually caused by dominant negative INSR mutations, generally presents peri-pubertally in girls. CASE: A 2.8-year-old girl was referred due to recurrent postprandial and fasting hypoglycemia. She had been born at full-term with birth weight 1.89 kg, and had developed transient neonatal diabetes. Examination showed satisfactory growth, reduced adipose tissue, acanthosis nigricans, and isolated thelarche. After 12 h of fasting, she developed hypoglycemia (glucose 2.8 mmol/L), with inappropriately raised plasma insulin concentration of 5.4 mU/L and suppressed fatty acids and ketone bodies. Oral glucose tolerance testing showed severely increased plasma insulin concentration (>300 mU/L) with hypoglycemia (glucose 1.6 mmol/L) at 2.5 h. She was initially managed on dietary modifications, cornstarch, and then trialed on acarbose for postprandial hyperinsulinemic hypoglycemia (PPHH) with some response. However, she was noted to have increased frequency of hyperglycemia after a couple of years of treatment. She was then switched to metformin and continued to have dietary carbohydrate modification including cornstarch that improved fasting tolerance, hyperglycemia, and postprandial hypoglycemia. Genetic testing identified heterozygous deletion of the last exon of the INSR gene, exon 22. CONCLUSION: We present a case of type A-IR, caused by a novel INSR deletion, presenting unusually early with transient neonatal diabetes, followed by episodes of hypoglycemia and hyperglycemia during later childhood. Early life presentations, including neonatal diabetes and PPHH, should lead to consideration of type A-IR.

Influence of anterior tooth alignment on peer perception in 8- to 10-year-old children.

The purpose of this research was to investigate whether anterior dental alignment in 8- to 10-year-old children influences the first impressions of their peers, and to verify the validity of the tested method. From colour photographs of two attractive children, one male and one female, Adobe Photoshop 5.0 was used to alter the images and create three versions of each photograph: one with good anterior dental alignment (OK smile), a second with proclination of the upper incisors (P-type smile), and finally one with moderate-to-severe anterior crowding (C-type smile). The six different photographs were shown to 121 subjects with mean age of 9.2 years (65 females and 56 males). Each subject was asked to view one photograph and subsequently respond to a questionnaire, the 'Smile perception questionnaire for children between the ages of 8 and 10' (SPQ 8-10), composed of 13 questions with graded responses. The responses for each photograph were analysed using linear regression analysis to determine the questionnaires validity as a whole and to investigate five area of common interest (honesty, intelligence, personal happiness, pleasantness, and extroversion). The results demonstrated that the questionnaire was reliable both from an internal coherence standpoint and from a test-retest reliability perspective. Data regarding the five areas of interest showed that 8- to 10-year-olds viewed their peers with well-aligned teeth more favourably as far as honesty, personal happiness, and intelligence were concerned. However, there was no statistically significant difference with regard to pleasantness and extroversion in children with harmonious, as opposed to crowded or proclined anterior teeth.