[Evaluation of final height in patients with pituitary growth hormone deficiency who were treated with growth hormone replacement]. / Ocena wzrostu koncowego pacjentów z somatotropinowa niedoczynnoscia przysadki, leczonych hormonem wzrostu.

BACKGROUND: Growth hormone (GH) therapy has been used for children with pituitary GH deficiency. It resulted in improving their height velocity and achieving an adult height in the normal range for the general population. OBJECTIVES: To evaluate the final height in childhood-onset growth hormone deficiency patients who had already completed treatment and were still GH deficient in adult life. MATERIAL AND METHODS: 21 children (12 boys and 9 girls) diagnosed as GH deficiency and treated with growth hormone to final height at doses of 0.17 mg/kg/week (0.5 IU/kg/week) subcutaneously for 7 days. There were 7 patients with isolated GHD and 14 with multiple pituitary hormone deficiencies. RESULTS: At the diagnosis peak serum GH concentrations were 2.8+/-2.8 mU/l in insulin tolerance test and 3.3+/-2.2 mU/l in clonidine test. Reconfirmation of the GH deficiency diagnosis after growth hormone treatment revealed a peak serum GH 1.77+/-1.2 mU/l in insulin tolerance test. Mean chronological age of the patients at the beginning of treatment was 10.29+/-3.57 years and was significantly higher in boys. Patients had completed a course of treatment in the chronological age of 17.85+/-1.97 years. Children began treatment with mean bone age 7.24+/-3.57 years and ended with 15+/-0.97 years. After the treatment a significant improvement in height was shown. Height SDS at the beginning of the treatment was -4.03+/-0.91 and -0.69+/-1.01 after the treatment. There was no difference between final height and target height (-0.54+/-0.93 SDS) in our patients. CONCLUSIONS: Children with pituitary growth hormone deficiency who were treated with growth hormone replacement achieve a final height in the normal range for the general population and their target height.

[Cardiovascular malformations in Turner syndrome]. / Wady ukladu sercowo-naczyniowego w zespole Turnera.

BACKGROUND: A literature review indicates that the prevalence of cardiovascular malformations in patients with Turner syndrome ranges from 17 to 47 percent. OBJECTIVES: The aim of the study was to evaluate the cardiovascular system in patients with Turner syndrome, controlled by the Pediatric Endocrinology Clinic of the Medical University of Gdansk. MATERIAL AND METHODS: The study included 55 females with Turner syndrome aged 3-20 years (mean age 9.6 years). The procedure included an interview, subject examination, chest X-rays and echocardiography. The echocardiography included M-mode measurements, two-dimensional evaluations from all standard planes, pulsed and continuous wave Doppler as well as color flow mapping. In selected cases, cardiologic examination embraced also cardiac catheterization, cineangiography and continuous 24-hour blood pressure measurement. RESULTS: Cardiovascular malformations were found in 18 out of 55 females (32.7%). Aortic coarctation was detected in 9 out of 18 patients (50%), bicuspid aortic valve in 33%, and aortic insufficiency in 22% of them. 78% of Turner subjects with cardiovascular malformations had monosomy (45,X). All females, except one, with aortic coarctation, had a 45,X karyotype. One aortic coarctation was diagnosed during prenatal examination. Before the last medical examination, 9 females had undergone a surgical treatment. In one case, the surgical treatment was preceded by a balloon angioplasty. Due to a recoarctation, one female was submitted to an additional balloon angioplasty. One female died during the post surgical period suffering from aortic wall injury. In four cases, despite successful surgeries, there still remains an arterial hypertension requiring pharmacological treatment. In 4 out of 18 cases (22%) isolated aortic valves defects were diagnosed, however, at present time they do not require any surgical treatment. In three cases, echocardiographic examination indicated a persistent left superior vena cava and aneurysm atrial septal defect with an intact septum. CONCLUSIONS: Due to a high prevalence of cardiovascular malformations, girls with Turner syndrome should be monitored by a pediatric cardiologist. In each case, female with Turner syndrome should be submitted to echocardiography. Also a careful control of the blood pressure is indicated.

[Endocrinologic problems in children with Down syndrome]. / Problemy endokrynologiczne u dzieci z zespolem Downa.

This "Endocrinologic health care guide" was prepared for individuals with Down syndrome. We hope this will serve as a reference for primary care physicians. We presented clinical characteristics, laboratory issues and treatment of children with Down syndrome and endocrinologic problems: abnormalities of thyroid function, obesity, diabetes mellitus, obesity, fertility control.

[Endocrinology complications of Langerhans cell histiocytosis]. / Endokrynologiczne powiklania histiocytozy.

The aim of presentation is an unusual case report of a 17 year old boy with short statured (SD (-)4), absent puberty and thyroid enlargement. Endocrine tests demonstrated a growth hormone and gonadotropin deficiency with diabetes insipidus. Magnetic resonance imaging showed a small pituitary gland and an absence of the posterior bright signal. Fine needle aspiration cytology of the focus localized in the left lobe of thyroid gland and nearest enlarged lymph nodes revealed diffuse infiltrate by the large histiocytosis confirming Langerhans cell histiocytosis like in the skin histological specimen biopsy. After chemotherapy we observed a regretion of focal changes in the thyroid.

[Influence of surgery and radiotherapy on growth and pubertal development in children treated for brain tumour]. / Wplyw zabiegu operacyjnego i radioterapii na tempo wzrastania i rozwoju dzieci z guzami osrodkowego ukladu nerwowego.

INTRODUCTION: The increasing number of childhood cancer survivors has resulted in a growing interest in the late effects, which depend on type of treatment. Frequently, a brain tumour and its therapy in children are endocrinologically devastating. AIM OF STUDY: The aim of study was to compare growth and pubertal development in children after brain tumour therapy treated or not treated with recombinant growth hormone (rGH). MATERIAL AND METHODS: 18 children were included in this study. Group I - (12/18) not treated with rGH, after total resection of brain tumour: craniopharyngeoma (8/12), astrocytoma (2/12) ependymoma (1/12), germinoma (1/12). Mean time of remission was 5,0yrs (+/- 0,9). Group II - (6/12) treated with rGH, after subtotal resection of craniopharyngeoma (4/6), ependymoma (1/6), medulloblastoma (1/6) and cranial irradiation with mean total doses 46,5 Gy (+/- 5,65). Children were qualified for rGH replacement according to deceleration of growth and lower growth hormone secretion (< 10 ng/ml) in stimulating tests. Mean time of remission was 6,5 yrs (+/- 2,41). Growth, height in centimeters converted to standard deviation score--SDS, body mass index (BMI), pubertal status and hormonal tests, were also evaluated. RESULTS: All patients were treated with surgery with no cranial irradiation in prepubertal age. 100% children of group I needed substitution because of secondary hypothyreosis, 83% due to secondary adrenal insufficiency and 53% of diabetes insipidus. Mean height after brain tumour surgical treatment in group I was - 1,24 SDS (+/- 0,85) and did not significantly change in the time of observation. Two girls needed hormonal substitution for hypogonadotropic hypogonadism. Mean BMI after total resection of brain tumour was 18,09 (+/- 4,20) and significantly increased to 23,73 (+/- 2,82). In group II - all children presented multihormonal pituitary insufficiency. Mean deviation score of height before rGH treatment was - 3,84 SDS (+/- 2,87) and after mean time of rGH therapy of 1,5 yrs (+/- 1,2) decreased to 2,6 (+/- 1,06). Mean BMI before treatment with rGH 18, 06 (+/- 4,4) increased to 22,41 (+ 0,74) in the time of observation and decreased to 18,5 (+/- 2,87) after 1,5 years (+/- 1,2) of rGH treatment. CONCLUSIONS: 1. Children treated with surgery for brain tumour need substitution for secondary hypothyroidism, part of then need treatment for secondary adrenal and gonadal insufficiency and diabetes incipidus. 2. Children who were treated with surgery and/or cranial irradiation developed multihormonal pituitary insufficiency, growth failure and replacement rGh therapy was needed. 3. Total resection of brain tumour without chemo- and radiotherapy did not impair growth in first years after surgery.

[Sudden death caused by myocarditis in a 14-year old boy with type I diabetes]. / Nagly zgon z powodu zapalenia miesnia serca u 14-letniego chlopca z cukrzyca typu I.

We report a case of diabetic ketoacidosis complicated by acute myocarditis which was confirmed by necroscopy. A 14 year boy was hospitalised with severe ketoacidosis. The patient levels of creatinine, transaminases were elevated, symptoms of hearth disease were complicated by dehydration and high temperature. An episode of upper respiratory viral infection before the onset of acute diabetes suggested that the patient died from viral myocarditis.

[Effect of three years obligatory iodine prophylaxis on the incidence of goiter in school children fron the seaside region of Poland]. / Wplyw trzyletniej obowiazkowej profilaktyki jodowej na czestosc wystepowania wola u dzieci szkolnych w regionie nadmorskim Polski.

UNLABELLED: In 1993 a mild iodine deficiency in the seaside region of Poland was revealed. In 1997 a mandatory iodine prophylaxis was introduced. The aim of the study was to determine the prevalence of goiter in schoolchildren aged 12-15 years from the seaside area after 3 years of obligatory iodine prophylaxis. The study comprised 628 children (291 boys and 337 girls). Thyroid size was estimated by palpation and by ultrasonography. Analysis of iodine excretion (Uroiod-Test Merck) was performed in 147 children. The goiter was found in 9% of children by palpation. The mean thyroid volume in 12 years old group was 9.3 +/- 5.1 ml, in 13 years old group 10.6 +/- 4.2 ml, 14 years old group 11.7 +/- 3.9 ml, 15 years 13.8 +/- 4.3 ml. The thyroid volume in girls was larger than that in boys. Thyroid volume above 97th percentile was assessed by ultrasound in 16% children (all of them from rural area). No goiter was found by palpation or ultrasound in children living in Gdansk Thyroid volume is closely correlated with iodine excretion, age and height. Urinary iodine excretion above 100 micrograms/l was stated in 68% children. CONCLUSIONS: Model of obligatory iodised salt prophylaxis is highly effective and is able to increase urinary iodine excretion and decrease of goiter prevalence. Ultrasound is especially recommended for the evaluation of thyroid volume in children from previously mild iodine deficiency areas.

[Difficulties in the diagnosis of thyroid cancer in 8-year-old boy]. / Trudnosci w rozpoznaniu raka gruczolu tarczowego u 8-letniego chlopca.

The 8-year-old boy with 13 months history of enlargement of right cervical lymph nodes is described in this paper. Infectious lymphadenopathy was initially diagnosed. He was treated with antibiotics without success. Fine-needle aspiration biopsy in one of the enlarged lymph nodes showed the cells of normal thyroid gland without any signs of atypical growth. USG and scintigraphy revealed normal thyroid gland without nodules; no extra thyroid glands were detected. In 13th month of the disease two enlarged (about 5 ml each), painful, rather hard cervical nodules were present. The thyroid gland was no enlarged and without any palpable nodules. Chest X-ray and second thyroid scintigraphy were normal. Ultrasound imaging of thyroid gland revealed hypoechogenic lesion 11 x 9 x 12 in diameter in the upper part of right thyroid lobe. The morphological findings of removed nodules allowed to make a diagnosis of carcinoma papillare psammomatosum glandulae thyroideae. Total thyroidectomy with removal of cervical ipsilateral lymph nodes was performed. The operation is to be followed by radioactive iodine ablation and thyroid hormone suppression. The authors analyzed the causes and difficulties in diagnosis.

[Growth velocity in children after chemo- and radiotherapy]. / Przebieg wzrastania dzieci dotknietych choroba nowotworowa.

UNLABELLED: The increasing number of childhood cancer survivors has resulted in growing interest in the late effects of chemo- and radiotherapy including growth also. THE AIM OF STUDY: The aim of study was to evaluate growth in children treated for acute lymphoblastic leukaemia (ALL) and Wilms' tumour who achieved complete continuous first remission following treatment. PATIENTS AND METHODS: 52 children included in this study: 30 treated for ALL - group I and 22 with Wilms' tumour - group II since 1986 to 1996. Group I- all children received prophylactic cranial irradiation at the total dose 12 and 18 Gy and chemotherapy according to therapeutic course BFM 83 and BFM 90. Group II - all children received abdomen irradiation with total dose 15-35 Gy, chemotherapy according to therapeutic course SIOP 9 and SIOP 92. We analysed growth velocity from the time of diagnosis to the time of examination. Results were reported as standard deviation score (SDS) to allow for comparison of patients of different age and sex. RESULTS: We observed significant growth deceleration in the first year of treatment and catch-up after 12 months from completion of therapy in both groups. The greatest reduction in yearly decrements in height SDS occurred in the first year after diagnosis. Patients of group I treated with prophylactic cranial irradiation with total dose 12 Gy presented significantly higher catch-up growth than treated with dose 18 Gy one year from completion of cancer therapy (p=0.001). Growth hormone deficiency in children of both groups was not observed. Bone age deceleration was retarded one year or more in both groups (group I p=0.025, group II p=0.001). CONCLUSIONS: 1) The chemotherapy and radiotherapy contribute to growth retardation in the first year of treatment in both groups 2) It seems that prophylactic cranial irradiation in children with ALL with total doses 12 and 18 Gy does not contribute to retardation of growth velocity after cancer treatment 3) Endocrine follow-up should be introduced in order to detect and treat complications as early as possible.

[Thyroid autoantibodies in children with recently diagnosed insulin-dependent diabetes mellitus]. / Przeciwciala przeciwtarczycowe u dzieci ze swiezo rozpoznana cukrzyca typu 1.

UNLABELLED: 119 newly diagnosed insulin dependent diabetes mellitus (IDDM) children - 59 girls and 60 boys aged 2-16 years were examined for the presence of thyroid autoantibodies (TA), T3, T4 and TSH levels. Estimate was repeated after 2 years with sonography of thyroid in children with the presence of TA. At onset of IDDM positive TA were found in 38 (32%) diabetic children - in 20 boys and 18 girls. The highest frequency of TA was stated in children aged 10-13 years. One boy with high TA was identified to have primary hypothyroidism caused by Hashimoto thyroiditis. In the remaining of children T3, T4 and TSH were in normal ranges and there was no significant difference between TA positive and TA negative group. In the examination after 2 years TA were found in 24% of children who were positive at onset. There were significant differences in T3 and T4 levels between children with positive TA at onset of IDDM and after 2 years. Thyroid volume by ultrasound was significantly higher in AT positive children (10.79+/-4.1 ml) than in ones who were TA negative 2 years later (7.99+/-4.3 ml). In half of TA positive children ultrasound changes were observed. CONCLUSIONS: High frequency of TA at onset of IDDM in children was observed. Careful follow-up examinations of thyroid function and ultrasound in that group are advised.

[Newborn mass screening for congenital hypothyroidism in Gdansk region]. / Badania przesiewowe noworodków w kierunku wrodzonej niedoczynnosci tarczycy w regionie gdanskim.

Newborn mass screening for congenital hypothyroidism was introduced in the Gdansk region in 1993. During the period 1993-2001 180708 newborns were screened. The prevalence of congenital hypothyroidism was 1:4203. The prevalence of transient hyperthyrotropinemia before implementation of the program of universal salt iodization was 0.016%, after that 0.003%.The request rate for repeated samples was 0.47% before and 0.24% after salt iodization.