Хуудас 1 -аас 27 үр дүн
The paper presents the data of examination and treatment of children with pubertas precox (PP) resulting from hypothalamic hamartoma, a rare malformation of the brain. It analyzes the results of 27 cases that is one of the largest series of cases in the world literature. Of the greatest interest are
OBJECTIVE
Hypothalamic hamartoma (HH) is one of the most frequent causes of organic central precocious puberty (CPP). We compared the clinical presentation and the magnetic resonance images (MRI) of 19 patients with HH aged 5.7 +/- 4.1 (SD) years at the first endocrine evaluation. They had isolated
To date, in publications on hamartomas, precocious puberty and laughing seizures have been discussed, but behavioural and cognitive abnormalities have been neglected. Therefore, we report a 14-year-old girl with a proven hamartoma, in which abnormalities of behaviour and cognition played an
We report on the case of a 31-year-old woman who presented with localized unilateral hyperhidrosis on her lower right arm. Histological features showed a hamartoma of the eccrine sweat glands. Because she was afraid of being overweight, the patient took an appetite depressant. Under this
Four cases of lipomatous hamartoma of the interatrial septum (LHIAS) were diagnosed at autopsy, three in elderly women, two of whom were morbidly obese. One patient had signs of congestive cardiac failure, without other significant cardiopulmonary disease, which was thought to be a result of the
Clinical manifestations of the hypothalamic hamartoma-epilepsy syndrome (HH-ES) in adulthood are variable. Efficacy of therapeutic options and outcome are diverse.Retrospective study of adult patients diagnosed with a HH in magnetic resonance imaging and Intrahepatic cholangiocarcinoma is a rare type of biliary tract malignancy, seldom found resectable at diagnosis, the 5-year survival rate depending on the possibility of complete surgical excision. In most cases it is only accidentally found in the early stage. The report presents a case of a
Acquired scrotal giant muscular hamartoma is an uncommon benign lesion with fewer than 10 documented cases all over the world. It is characterized by a proliferation of dermal smooth muscle bundles of scrotum dartos fascia. The authors report a rare case of acquired scrotal giant muscular hamartoma,
Massive localized lymphedema (MLL) is an uncommon benign skin lesion typically presenting with prominent edema and vascular proliferation in the adipose tissue of lower limbs. When rarely occurring in scrotum, it instead is characterized by a striking proliferation of dermal smooth muscle bundles
BACKGROUND
Hypothalamic hamartoma (HH) represents the commonest cause of organic central precocious puberty (CPP). Follow-up of these patients in adulthood is scarce.
OBJECTIVE
To describe the anthropometric, metabolic, and reproductive parameters of patients with CPP due to HH before and after
A series of 17 patients aged from 9 months to 32 years with refractory epilepsy due to hypothalamic hamartoma were treated by total removal (one case) and disconnection (16 cases) between 1997 and 2002. The mean age at seizure onset was 16 months. Sixteen patients had gelastic seizures, 14 had
Hypothalamic hamartomas (HHs) are benign lesions that are often associated with central precocious puberty and may present with gelastic seizures. Treatment modalities for HH include medical therapy with long-term gonadotropin-releasing hormone analogs or resection. The authors report the case of a
OBJECTIVE
To study the surgical procedures and results on seizures, in 18 patients with refractory epilepsy due to hypothalamic hamartoma.
METHODS
Eighteen patients aged from 9 months to 32 years underwent surgery between 1997 and 2002. The mean age at seizure onset was 15.5 months. Seventeen
Although the GnRH agonist analogs have become an established treatment for precocious puberty, there have been few long term studies of reproductive function and general health after discontinuation of therapy. To this end, we compared peak LH and FSH after 100 microg sc GnRH, estradiol, mean
Oral-facial digital syndrome (OFDS) consists of a group of heterogeneous genetic disorders with different patterns of inheritance. These disorders share facial, oral and digital abnormalities. At least 11 types of this syndrome have been described (I-IX) and the association between OFDS type II and