BACKGROUND: We report four pediatric subjects with Cushing\'s disease (CD) diagnosed in the Czech Republic. We focus on initial symptoms of Cushing\'s syndrome (CS) which can lead to early diagnosis, on typical symptoms of CS in children, their age and sex distribution, the mean length of symptoms prior to diagnosis, indication for examination, post-cure growth, sexual development and pituitary function in our four CD patients after transsphenoidal pituitary surgery (TSS). We describe the diagnostic process leading to confirmation of CD and we emphasize the biochemical and radiological diagnostic difficulties.
CONCLUSIONS: Pediatric CD has a number of features distinct from adult CD. Our retrospective analysis confirmed the presence of growth retardation and change in facial appearance with development of moon face as the first symptoms of CS. According to our observation, growth retardation is prior to development of moon face. The other typical symptoms frequently seen in pediatric patients are pseudo-precocious puberty in both sexes, hirsutism in pubertal girls due to excessive adrenal androgen secretion and pubertal delay. A corticotropin-releasing hormone (CRH) test and especially bilateral inferior petrosal sinus sampling for ACTH (BIPSS) contribute to confirming the diagnosis of CD and excluding ectopic ACTH syndrome in children with unvisible adenoma on pituitary magnetic resonance imaging (MRI).

UNASSIGNED: precocious puberty is defined as the development of sexual characteristics before 8 years of age in girls and 9 years of age in boys. The purpose of our study was to describe the clinical, paraclinical and etiological profile of precocious puberty.
UNASSIGNED: we conducted a retrospective descriptive study from 1999 to 2017 in the Pediatric Endocrinology Unit at the Rabat Children\'s Hospital.
UNASSIGNED: ninety-nine children were included in the study. The average age of girls was 4.25 ± 2.6 years, while that of boys was 3.6 ± 1.8 years. There was a female predominance (90%; 90 girls). The telltale signs in girls were dominated by breast development (77.77%). In boys, the most common reason for consultation was pubic hair (70%). Biologically, in central precocious puberty, the mean peak LH level after GnRH stimulation was high (17 IU/L) with a mean peak LH to peak FSH ratio of 1.30. The causes included: dissociated early puberty (60.60%), pathological early puberty (39.40%). In the latter, we noted a predominance of precocious pseudopuberty (58.98%). Concerning the central precocious puberty, idiopathic central precocious puberty was the most common etiology in girls (62.5%). In all boys, central nervous system lesion was found.
UNASSIGNED: our study confirms that central pathological precocious puberty is often related to a lesion of the central nervous system in boys, thus justifying systematic brain imaging.

BACKGROUND: Van Wyk Grumbach syndrome (VWGS) and Kocher-Debre Semelaigne syndrome (KDSS) are rare forms of pseudo-precocious puberty and myopathy in patients with longstanding untreated hypothyroidism. We present the case of an adolescent girl who developed pseudo-precocious puberty and myopathy caused by long-term untreated hypothyroidism.
METHODS: A 17-year-old female patient was referred to our outpatient clinic due to menstrual irregularities. She had muscle pain and multiple cystic ovaries in addition to clinical signs of severe congenital hypothyroidism. High levels of creatinine and creatinine kinase and accompanying muscle hypertrophy were present. After the initiation of L-thyroxine therapy, the symptoms were alleviated in a short time.
CONCLUSIONS: Congenital and acquired hypothyroidism should be considered in the differential diagnosis of pseudo-precocious puberty and myopathy that presents with muscle pain, muscle hypertrophy, and elevated creatinine kinase levels.

We report a rare case of virilizing cystic juvenile granulosa cell tumour of the ovary diagnosed by histopathological examination in a 17-year-old female presented with mass abdomen for two months, growing of the hairs on the face and abdomen and deepening of voice for one year.

Steroid cell tumor, not otherwise specified (NOS), are rare ovarian tumor, in addition, it is more rare in children. The majority of these tumors produce several steroid hormones, particularly testosterone. Estrogen also secreted by steroid cell tumor, NOS, but it is uncommon. Furthermore, hypertension is an infrequent sign in steroid cell tumor, NOS. An 8.5-yr-old girl with hypertension and frequent vaginal spotting visited at our clinic. On laboratory evaluation, secondary hypertension due to an elevated plasma renin level and isosexual pseudoprecocious puberty was diagnosed. Right solid ovarian mass was detected in radiologic tests. She underwent a right ooporectomy and it revealed renin and progesterone receptor positive steroid cell tumor, NOS. After operation, her blood pressure returned to normal level and vaginal bleeding disappeared. Even though this case is very rare, when hypertension coincides with virilization or feminization, a renin-secreting ovarian steroid cell tumor, NOS, should be considered.