[Primary prevention of type 1 diabetes. Actual situation and perspectives]. / Pierwotna prewencja cukrzycy typu 1. Stan obecny i perspektywy.

The authors presented a review of the actual literature of primary prevention of type 1 diabetes. They stressed a dramatic increase of patients with newly diagnosed diabetes, which was called an epidemic of the XXI century. According to the recently published data, the increase of newly diagnosed cases was more rapid/faster than it was expected/anticipated, and the dramatic increase in the occurrence/prevalence of type 1 diabetes was observed particularly in children up to 5 years of age. Therefore, like it was mentioned by the authors of recently published reports, the necessity of an effective primary prevention of type 1 diabetes also increased. Many clinical trials of primary prevention of type 1 diabetes, in different clinical stages/phases, are currently being conducted. Scientific investigations about an effective primary prevention are now very important, seen as priority problems. Investigations in Poland, which started in the year 2002 and the end of them was established for the year 2017, are also performed as part of an international scientific program. The identification of subjects with a genetic risk of type 1 diabetes is now possible. Very interesting data of a therapy with t-regulatory cells (tregs), especially those received from the umbilical cord blood of newborns are worth emphasizing.

[Progress in diagnostics and therapy of type 1 and type 2 diabetes--part 2]. / Postepy w leczeniu i diagnostyce cukrzycy typu 1 i typu 2--czesc druga.

The report is the second part of a former publication. The authors presented a series of reports, especially from the last year, concerned with diagnostics and therapy of type 1 and type 2 diabetes. The progress of the diagnostics included especially new, more perfect glycaemic monitoring systems. The progress and future of the therapy with personal insulin pumps and different international recommendations for the therapy of type 1 diabetes with short-acting insulin analogues were presented. Also, the problem of the reports about the increased risk of cancer after the treatment with a long-acting insulin analogue glargine was discussed (Lantus). Further investigations of the effect of the therapy with a biphasic insulin and new possibilities of oral therapy in type 2 diabetes were mentioned. It was stressed that actually, a lot of proposals of new drugs and new reports are observed. These reports are, however, frequently conference news and not complete reports and the proposals require further examinations and observations for a confirmed appreciation of their efficacy and safety.

Progress in the treatment of diabetes type 1 and 2.

In the last years an increased incidence of diabetes was observed in the whole world. It was estimated that in the year 2030 there will be around 300 million patients with diabetes. Diabetes, especially not adequately treated, develops serious chronic complications. The main aim of the therapy in diabetes is, as we know, to achieve a stable normoglycemia, normal levels of HbA1c, the prevention or inhibition of the progression of late consequences of diabetes. In the paper the authors discuss new more perfect insulins which enable a better imitation of the physiological rhythm of insulin secretion, the therapy with personal insulin pumps, the more perfect equipment for the appreciation and monitoring of the metabolic control. The authors present also the actual data about the transplantation of the pancreas, the islets and recently the beta cells alone. They enumerate also the inclusion and exclusion criteria for transplantation. The gene therapy is mentioned. The present possibilities of the therapy of type 2 diabetes are presented. It is stressed that in the year 2006 for the first time an expert crew was appointed to elaborate a prevention and therapeutical program for diabetes in Poland. The program was accepted by the Ministry of Health for realisation in the years 2006-2008. The authors conclude that in the recent years an enormous progress in the prevention, diagnostics and therapy in diabetes has been achieved. However, the time which should elapse to the moment when the complete success would be achieved is dependent on the further scientific progress the intensity of investigations in the whole world.

[Incidentaloma in a 16 years old girl -- 2 year observation]. / Incydentaloma u 16 letniej dziewczynki -- obserwacja dwuletnia.

Adenomas of the hypophysis are tumors of the CNS which are on the third place in the frequency of appearance, which cause disturbances of hypophyseal function. In children incidentaloma is seldom observed. The authors present a 16-year-old girl who was admitted to the clinic because of amenorrhoea and an increased growth velocity during the last year. The MRI examination of the hypophysis proved a hypophyseal adenoma. Hormonal laboratory examinations do not show any hormonal activity of the observation. The girl's case is presented because the diagnosis of an incidentaloma is exceptionally rare in this age group.

[Pituitary response to GnRH analogue testing in girls with a polycystic ovary syndrome]. / Odpowiedz przysadki mózgowej w tescie z analogiem GnRH wsród dziewczat z zespolem policystycznych jajników.

UNLABELLED: THE AIM of our study was to estimate the gonadotropin level after GnRH analogue injection in girls with PCOS after suppression with dexamethasone. MATERIAL AND METHODS: 57 girls with hirsutism, mean age 15.9 years, were involved in the study. The research was performed in the early and middle follicular stage. Menstrual disorders were observed in 78% of them. The patients were divided into 3 groups: I -- with clinical and laboratory symptoms of PCOS (menstruation disorders, testosterone >65 ng/ml and/or LH/FSH >2; n=29), II -- with menstruation disorders and without elevated androgen level (n=15), III -- without menstruation disorders and without elevated androgen level (n=13). Basal blood samples were drawn at 8 a.m. GnRH analogue (Relefact LH-RH) 100 microg was then given subcutaneously and blood samples were drawn every 4 hours for 24 hours. RESULTS: Basal level of LH was the highest in group I (6,18+/-4,10 IU/l) in comparison with II (5.53+/-3.40 IU/l) and III (3.82+/-2.79 IU/l). After GnRH analogue administration mean LH concentration increased in all groups and peaked after 2 hours. Stimulated LH level was the highest in group I and differed statistically significantly from group III during the whole period of the test. The most significant difference occurred at 12 a.m. (p=0.003) and 10 a.m. (p=0.004). The FSH secretion in all tested groups was similar. It peaked, like LH, after 2 hours after GnRH analogue injection and decreased slightly during next 2 hours. A marked decrease was observed in the following period of time. CONCLUSIONS: 1. High and fast LH secretion responding to GnRH analogue indicates masculinization of the hypothalamo-pituitary axis in PCOS girls. 2. The hirsute girls without menstrual disturbances and hormonal abnormalities probably also have subtle masculinization of the pituitary response to stimulation by GnRH analogue.

Is breast reduction in puberty indicated? Retrospective observations of patients with a local hypersensitivity of estrogen and progesterone receptors.

The authors described two women in whom at the age of 14 and 15 diagnosed was gigantomasty and a hypersensitivity of the estrogen receptor. In both the patients at that time a significant increase of the estrogen and progesterone receptors in the breast glandular tissue was ascertained. The immunohistochemical investigation shows a significant higher sensitivity of the progesterone and estrogen receptors. In both the girls observed was gigantomasty with hyperlordosis, deformities of the chest and back, skin changes, orthopnoe. In both the patients a total mastectomy was performed. Both the girls had after surgery a normal menstruation, were married with normal sexual activity. One of the patients had a normal delivery two years ago.

[Retrospective comprehensive assessment of the treatment effect of children with growth hormone deficiency]. / Retrospektywna kompleksowa ocena efektów leczenia dzieci z somatotropinowa niedoczynnoscia przysadki.

The most often cause of hormonal microsomy is a growth hormone deficit on the consequence of hypofuncion of the pituitary anterior lobe (SNP). The aim of the study was a retrospective analysis of children with SNP treated with a synthetic growth hormone (rhGH). Analyzed was the growth velocity in dependence of the dose and developmental period of the patients. Additional analyzed was the influence of the relative body mass (BMI) index and the degree of growth hormone deficit on the result of the therapy with the recombined growth hormone. Analyzed was also the maturity of the bone age during the therapy with growth hormone. A statistical analysis was performed of the influence of the particular parameters on the growth velocity of the investigated patients on support on the one - or multifunctional variant analysis MANOVA. Observed was, that the growth velocity was the highest in children treated with the highest dose of growth hormone. A dependence between the degree of growth hormone deficit and the growth velocity during the therapy with growth hormone was not evidenced. Not shown was also difference in the growth velocity in dependence on the body mass index. Bone age after the therapy during the three years do not achieve the calendar age in the investigated children. The comparison of the therapeutic results in children in a different phase of maturity shows that the best growth velocity was achieved in children in whom the therapy was started before the age of six years. Therefore the beginning of the therapy with growth hormone was recommended at the earliest. The performed examination evidenced also, that children treated with present doses of growth hormone do not attain the provided height, therefore necessary is a further optimalisation of the therapy of SNP with higher doses of rhGH.

[The bone mineral density and the markers of the osseous circle in children with non-toxic parenchymatous or nodular goiter long-time treated with L-thyroxine]. / Ocena gestosci mineralnej tkanki kostnej oraz markerów obrotu kostnego u dzieci dlugotrwale leczonych syntetyczna lewotyroksyna z powodu nietoksycznego wola rozlanego i guzkowego.

The aim of the study was the appreciation of the influence of a therapy with L-thyroxine on the metabolism and density of the osseous tissue (BMD) in children with an euthyroid diffuse and nodular goiter. The examinations included 50 children (5 boys and 45 girls). Mean age of the investigated group: 17 years, time of therapy 2-5 years. The daily dose of L-thyroxine was not higher than 1-2 microg/kg body mass. All the children were in clinical and hormonal euthyrosis. The control group consisted of 50 healthy children (12 boys and 38 girls), mean age 16,16 years. A correlation between the age of the children, TSH level and the markers of the osseous circle was not observed. The mean level of TSH was statistical significant lower in the examined group. In the examined group the level of PTH and ICTP in the blood serum was (PTH: 35.83+/-8.34 pg/m vs 37.21+/-7.17 pg/ml); ICTP (8.7+/-3.87 microg/l vs 15.11+/-5.7 microg/l) was lower in the control group but the difference was statistical not significant (p=0.07). The mean concentration of PICP in the investigated group was significant lower in comparison with the control group. The mean level ICTP between the examined and control group was statistical significant (p<0.05). The mean concentration of osteocalcine (OC) in the treated with L-thyroxine was statistical not significant.

[Adrenal function in girls with hirsutism]. / Funkcja nadnerczy u dziewczat z hirsutyzmem.

UNLABELLED: The aim of our study was to estimate the adrenal function in hirsute girls. MATERIAL AND METHODS: 57 girls with hirsutism aged from 12 to 19 years, mean age 15.95 years, were involved into the study. The research was performed in early and middle follicular stage. Menstrual disorders were observed in 78% of them. Hirsutism was estimated with Ferriman-Gallwey scale (mean value 13+/-1.58), mean BMI was 22.7. The patients were divided into 3 groups: group 1 with clinical and laboratory symptoms of PCOS, n=29; group 2 with menstrual disorders and without elevated androgen level, n=15; group 3 without menstrual disorders and without elevated androgen level, n=13. RESULTS: 17OHP level was the highest in group I (1.17+/-0.58 ng/ml). Diurnal cortisol profile was regular in all patients. After Synacthen injection cortisol level rose in all groups to the similar values at 60 min. The same stimuli induced intensive 17OHP secretion in group 1 (2.42+/-2.02 ng/ml) at 30 min statistically higher than in group 2 (1.46+/-0.95 ng/ml), (p=0.045). None had 21-hydroxysase defect. There were positive correlation between levels of 17OHP and LH (r=0.38), 17OHP and T (r=0.39), 17OHP and LH/FSH (r=0.40) CONCLUSIONS: 17OHP level in patients with PCOS is significantly higher then in other hirsute girls. High 17OHP and normal cortisol level after Synacthen administration in PCOS girls point that activity of enzymes involved in 17OHP production is augmented.

Chronic autoimmune thyroid disease in children and adolescents in the years 1999-2004 in Lower Silesia, Poland.

The aim of the study was to analyze data related to chronic autoimmune thyroid disease at diagnosis and at follow-up of children and adolescents in Lower Silesia in the years 1999-2004. Age, gender, incidence of thyroid disease in the family, clinical presentation, hormonal findings, levels of thyroid antibodies, results of ultrasonography, and fine needle aspiration biopsy (FNAB) were recorded. 100 children, 10 boys and 90 girls, were included in the analysis. The mean age at diagnosis was 12.3+/-2.3 years and at last examination 14.9+/-1.9 years. At diagnosis, increased levels of TSH without overt hypothyroidism was observed in 26 children. In 11 children hyperthyroidism was detected whereas 63 children were euthyroid. An increased level of thyroid peroxidase antibodies was observed in 65% of the children. Ultrasonography was characteristic for Hashimoto's thyroiditis in all patients. Fine needle biopsy was performed when there were diagnostic difficulties (35% children). Thus, in all the children the diagnosis of Hashimoto's thyroiditis was ascertained either by high antibody titer or FNAB. Associated diseases were observed in 33% of the children. Thyroid disease in the family was present in 25% of the children. There was a gradual decline in the number of new cases presented from 1999 to 2004. The reason for this decline remains speculative.

Hyperprolactinemia in children during the peripubertal period--personal observations.

UNLABELLED: There can be many reasons for functional hyperprolactinemia, including polycystic ovary syndrome (PCOS), obesity, insulin resistance and hypoglycemia. Prolactinoma is also a frequent cause. This study included 12 patients (10 girls and 2 boys) aged 14-17 years with hyperprolactinemia. Six patients were treated for PCOS, two for type 1 diabetes mellitus, and one patient suffered from Prader-Willi syndrome. In all patients, TSH, fT3, fT4, FSH, LH, testosterone, and E2 levels were measured. MRI of the pituitary was performed. Prolactin was measured with the metoclopramid dynamic test (MTC). IRI, C-peptide, oral glucose test, and minor pelvis ultrasound examination were performed in patients with PCOS. Pituitary adenoma was diagnosed in four out of 12 patients. In the remaining eight patients, functional hyperprolactinemia was found. All of these patients remain under pharmacological treatment with positive clinical results. CONCLUSIONS: 1. It is necessary to determine prolactin in each girl with unexplained amenorrhea irrespective of galactorrhea. 2. In some patients with prolactinoma the basal prolactin levels may be in the normal range, but they are increased in the MTC test. 3. In girls with various disorders of the menstrual cycle it is necessary to determine the level of prolactin with a provocative test.

[Transplantation in diabetes type 1--current problems and perspectives]. / Przeszczepy w cukrzycy typu 1--aktualne problemy i perspektywy.

Diabetes type 1 is, as we know, a chronic progressive disease, which requires a substitutional therapy with insulin for the whole life. The cause is a definite destruction of the pancreatic beta cells. For many years there have been intensive investigations on the possibility to obtain a complete, persistent withdrawal of the symptoms. Substitution of the destroyed, not active cells, could take place after transplantation of the whole pancreas, transplantation of pancreatic islets or transplantation of stem cells. This is now the only method which may cause an independence from exogenous insulin, persistent normoglycemia, normal HbA1c level, without risk of hypoglycemia. Pancreas and islets transplantations, however, are connected till now with the necessity of an immunosuppressive therapy for the whole life, with the toxicity of the drugs, incidence of frequent infections and malignancy. Pancreas transplantation is a serious surgical intervention, connected with numerous risks and complications, considerably less risk appears in islet cell transplantations. Since 2000 exclusively islet cell transplantations have been performed. One of the leading centers is Edmonton, where professor Shapiro prepared the so called. Edmonton protocol which is characterized by using corticosteroid-free immunosuppressive drugs, islet cells from two or more donors, repeated till the attainment of insulin dependence. A problem now is that the islets are obtained from cadavers. Therefore intensive research is conducted for alternative sources of beta cells. At this moment it is mostly preferred for receiving a sufficient number of insulin producing cells to develop stem cells with a subsequent differentiation to insulin producing cells. The mentioned cells have an unlimited ability of reproduction, in this case also immunosuppressive therapy is not necessary. Alternative sources of beta cells are cells achieved on the genetic engineering, embryonic or adult somatic stem cells. It is however important to stress, that adult stem cells as insulin producing cells are not unequivocally identified. For obtaining better, permanent results after transplantation the following are important: optimalization of "islands growth" in the liver, prevention of the early inflammations, further development of highly selective, well tolerated, corticosteroid-free immunosuppressive drugs, identification of rejecting markers, induction of immunotolerance, micro- and macro-capsulation of the islets to protect the recipient against the immunological attack. Several multicenter studies in important scientific centers are opened, there is also Juvenile Research Foundation International. In spite of a permanent progress there are still many important problems to solve. It is necessary to institute further multicenter, international research to ascertain the effect of transplantation concerning the normalisation of glycemia, prevention or inhibition of the progress of diabetic complications and to prolong the life span in patients with type 1 diabetes after transplantation.

[Personal observations of puberty in girls after unilateral ovariectomy]. / Wlasne obserwacje dotyczace przebiegu okresu dojrzewania u dziewczat po jednostronnej owariektomii.

The authors present the course of puberty in three girls after surgery because of hormonally active ovarian tumours diagnosed and operated at the age of 3, 6 and 9 years, respectively. In the youngest patient with folliculoma ovari chemotherapy was also given, and girl No. 3 with luteinic capsule was treated for one year with decapeptyl because idiopathic precocious puberty was diagnosed. At present all three girls are in good physical condition with age-adequate development and puberty.

[Kallmann's syndrome in families]. / Rodzinne wystepowanie zespolu Kallmanna.

UNLABELLED: The authors present the incidence of Kallmann's syndrome in two families in four persons. In one family the syndrome was present in a boy and the brother of his mother. In the second family in siblings -- a boy and a girl. The two boys at preschool age underwent a surgery because of bilateral cryptorchism. In the first patient and his uncle additional hearing dysfunction and agenesis of the left kidney were diagnosed. In all the patients anosmia was diagnosed. All the patients receive a pharmacological treatment which improved significantly their clinical state, caused the development of tertiary sexual feature and an improvement of the psychological condition. CONCLUSIONS: 1. In patients with an abnormal development of the urethro-sexual organs a diagnosis for other disturbances or developmental defects is necessary. 2. In patients with an abnormal urethro-sexual development a permanent care of a psychologist is necessary.