Endocrine manifestations of the rapid-onset obesity with hypoventilation, hypothalamic, autonomic dysregulation, and neural tumor syndrome in childhood.

CONTEXT: Rapid-onset obesity with hypoventilation, hypothalamic, autonomic dysregulation, and neural tumor (ROHHADNET) is a newly described syndrome that can cause cardiorespiratory arrests and death. It mimics several endocrine disorders or genetic obesity syndromes during early childhood and is associated with various forms of hypothalamic-pituitary endocrine dysfunctions that have not yet been fully investigated. OBJECTIVE: The current report aspires to facilitate the earlier recognition and appropriate treatment of the ROHHADNET syndrome when children present with various endocrine manifestations, such as early obesity, growth failure, pseudo-Cushing's syndrome, glucocorticoid insufficiency, congenital hypopituitarism, or adrenal tumors. A more widespread knowledge of the syndrome will help characterize its molecular origin. DESIGN: Endocrine studies were performed in six patients admitted for seemingly common early-onset obesity associated with growth failure in five of them. The six patients later showed distinctive features of the ROHHADNET syndrome. RESULTS: Abnormalities of the pituitary adrenal axis ranged from a true Cushing-like profile (one of six), to glucocorticoid deficiency with normal ACTH (two of six). Complete GH deficiency with low IGF-I was observed in four of six, hypogonadotropic hypogonadism in four of six, hyperprolactinemia in six of six, and various degrees of TSH/T(4) abnormalities in five of five patients. All had increased natremia without diabetes insipidus. Five children had unilateral macroscopic adrenal ganglioneuroma. Two patients died at 8.5 and 12 yr of age. CONCLUSIONS: Various hypothalamic-pituitary endocrine dysfunctions are associated with ROHHADNET, carrying a risk of misdiagnosis until other elements of the syndrome make it more easily recognizable. Given its severity, ROHHADNET syndrome should be considered in all cases of isolated, rapid, and early obesity.

[Shift and night work--is it a cancer risk factor?]. / Czy praca zmianowa i nocna jest czynnikiem ryzyka choroby nowotworowej?

This paper presents the results of studies on the effects of circadian rhythm disturbances and melatonin on the carcinogenic process. It is thought that melatonin synthesis mostly influences the generation of breast and colorectal cancers. However, its influence on the development of prostate, ovarian, and endometriosis cancers is also suggested. It should be remembered that many other important risk factors contribute to the pathogenesis of these diseases. It is essential for occupational medicine physicians to pay special attention to so called "oncological risk" in workers with long duration of employment in the shift-work system as this group of workers is also exposed to other cancer risk factors. Exogenous melatonin supplementation along with reduced effect of shift work is very important from the practical point of view.

[The inhibiting effect of phytoadaptogenic preparations from bioginseng, Eleutherococcus senticosus and Rhaponticum carthamoides on the development of nervous system tumors in rats induced by N-nitrosoethylurea]. / Tormoziashchii éffekt fitoadaptogennykh preparatov biozhen'shenia, éleuterokokka koliuchego i levzei saflovidnoi na razvitie opukholei nervnoi sistemy u krys, indutsirovannykh N-nitrozoétilmochevinoi.

The study was concerned with the inhibitory effect of officinal preparations of phytoadaptogenic drugs such as bioginseng and Eleutherococcus senticosus and Rhaponticum carthamoides root extracts on the carcinogenesis induced by transplacental administration of N-nitrosoethylurea. The phytodrugs were given orally over a year. The administration of the drugs was followed by longer survival of the rats and lower occurrence and/or multiplicity of tumors (mainly those of the central nervous system). The drugs were placed in the order of decreasing anticarcinogenic activity as follows: bioginseng, Rhaponticum carthamoides extract and Eleutherococcus senticosus extract.