Nutritional habits according to age and BMI of 6-17-year-old children from the urban municipality in Poland.

BACKGROUND: Balanced nutrition is crucial for adolescent's proper physical and mental development. Dietary habits change significantly with a child's development. Along with increasing age and the shift towards adolescence, unhealthy diet-related habits become more common. The objective of the survey study was to determine the differences in nutritional habits between children and adolescents according to their age and body mass index (BMI). METHODS: "Let's get the kids moving" campaign (pol. "Uruchamiamy dzieciaki") was launched in 2016. Within the campaign, the survey study was conducted in 2913 participants between 6 and 17 years old from primary and junior high schools in Wroclaw (Poland). The survey was anonymous, and its supplement was voluntary. Participants were divided into age groups. The study group of 2913 consisted of 29.8% of 6-9-year-olds, 32.7% of 10-12-year-olds, and 37.5% of 13-17-year-olds. Body mass index (BMI) was calculated and further interpreted as a BMI z-scores depending on children's age and gender. RESULTS: A total of 19.3% of participants consumed 3 meals a day or less. Children from the oldest age group (13-17) consumed statistically significantly fewer meals per day than younger children (p < 0.001). Children from the oldest age group (13-17) consumed breakfast statistically less often than children of age group 10-12 years (75.0% vs. 83.6%; p < 0.001) and children of age group 6-9 years (75.0% vs. 84.0%; p < 0.001). Severely thin children consumed breakfast significantly more often than overweight (85.8% vs. 76.3%; p = 0.004) and children with obesity (85.8% vs. 75.9%; p = 0.021). Children with obesity consumed vegetables significantly less often than severely thin (p < 0.008), thin (p < 0.001), and children with normal body weight (p < 0.007). The oldest children (13-17 years) consumed Coca-Cola and SSB (p < 0.001) and fruit-flavored beverages (p < 0.05) significantly more often than children from other age groups. Boys consumed carbonated beverages with added sugar significantly more often than girls (p < 0.01). CONCLUSIONS: Unhealthy diet-related behaviors in children and adolescents may promote overweight and obesity and should be targeted in health promotion programs. Special attention should be paid to 13-17-year-olds, as adolescents from this group made more unhealthy choices than younger children.

Pallister-Hall syndrome in a 2-years-old girl.

INTRODUCTION: Pallister-Hall syndrome (PHS) is a rare autosomal dominant syndrome characterized by polydactyly, bifid or shortened epiglottis, visceral anomalies, hypothalamic hamartoma often combined with hypopituitarism. PHS is characterized by significant variability in the expression of clinical symptoms. The clinical course ranges from mild with a good prognosis to severe and which can lead to death during the neonatal period. CASE REPORT: Two-years-old girl with facial dysmorphia, skeletal malformations of hand and feet and growth hormone deficiency. PHS was diagnosed on the basis of the presented symptoms and genetic tests. SUMMARY: Skeletal malformations, such as polydactyly or oligodactyly, are a markers which can be associated with endocrinological disorders. Quick and correct diagnosis would help in planning treatment during childhood and giving family counseling, including prenatal advice regarding the next pregnancy of the child's mother.

The assessment of skeletal status in young patients with Turner syndrome by 2 densitometric techniques: Phalangeal quantitative ultrasound and dual energy X-ray absorptiometry.

BACKGROUND: Studies using dual energy X-ray absorptiometry (DXA) demonstrate a reduction in bone mineral density (BMD) in children and adolescents with Turner syndrome (TS). However, these studies do not take into account changes in bone size, which influence BMD in the case of short-statured patients. Phalangeal quantitative ultrasound (phQUS) measurements have shown an ability to reveal changes due to skeletal growth, aging, and bone and mineral disorders. There is limited data on bone mineral status in girls with TS assessed by 2 different techniques, i.e., DXA and phQUS. OBJECTIVES: The aim of this study was to investigate the potential negative impact of TS on bone status and to assess whether densitometric values were related to former fractures. MATERIAL AND METHODS: In 43 TS girls aged 5-18 years, we evaluated bone status by 2 different densitometric techniques, DXA and phQUS. RESULTS: The mean lumbar spine areal bone mineral density (LS aBMD) Z-score was significantly lower than 0 (the hypothetical mean) compared to the reference population (p < 0.001). The mean LS aBMD height-adjusted Z-score did not differ significantly from 0. The amplitude-dependent speed of sound (Ad-SoS) Z-score was significantly lower than 0 compared with a Polish reference population. There were no significant differences between fractured and fracture-free patients as regards Ad-SoS Z-score and LS aBMD height-adjusted Z-score. CONCLUSIONS: Girls with TS have normal bone density adjusted for height, but significantly decreased phQUS values. Neither DXA nor phalangeal Ad-SoS can identify young TS patients with former fractures.

LPL gene mutation as the cause of severe hypertriglyceridemia in the course of ketoacidosis in a patient with newly diagnosed type 1 diabetes mellitus.

INTRODUCTION: Severe hypertriglyceridemia is a condition associated with extremely high triglycerides (TG) plasma concentrations exceeding 1000mg/dl. This condition may result in mutations in genes encoding lipoprotein lipase (LPL), apolipoprotein C2 (APOC2) and apolipoprotein A5 (APOA5) characterized by an autosomal recessive inheritance pattern. AIM: A case report of a patient in which clinical picture of type 1 diabetes mellitus (T1DM) was accompanied by diabetic ketoacidosis (DKA) and severe hypertriglyceridemia. CASE REPORT: A 2.5-year-old boy was admitted to the hospital with ketoacidosis (pH - 7.0, BE - 20mmol/l, HCO3 10mmol/l), glucose level of 850mg%, hyponatremia (Na 100mmol/l) and hyperlipidemia (TG 13493 mg/dl, TC 734 mg/dl). The administered treatment resulted in nearly normal glycemic values and lipid disturbances normalization. This child was diagnosed with a heterozygous mutation of the LPL gene. Currently with an intensive insulin therapy and correct metabolic control of type 1 diabetes mellitus (T1DM), this patient maintains a normal lipid profile. CONCLUSION: In patient with T1DM the diagnosis of severe hypertriglyceridemia in the course of ketoacidosis should be based on careful interpretation of laboratory tests results. Moreover genetic tests of the patient and his/her immediate relatives blood samples should be performed.

1H NMR-based metabolomics studies of urine reveal differences between type 1 diabetic patients with high and low HbAc1 values.

The aim of this study was to investigate relation between level of HbAc1 and concentration of metabolites in urine of T1D patients. To test this hypothesis the (1)H NMR (proton nuclear magnetic resonance) target analysis of crucial urine metabolites combined with chemometric approach were applied. Urine samples were collected from 30 children and teenagers aged 4-19 with T1D and 12 healthy children, aged 9, as control group. Patients were divided into two groups according to their level of glycated hemoglobin (HbA1c): below (L-T1D) and above 6.5% (H-T1D). The multivariate data analysis (OPLS-DA) was used to explore data and generate the models for selected groups of patients. Two tailed unpaired t-test was used for statistical analysis of quantified metabolites. Comparing to L-T1D patients, H-T1D group exhibited increased levels of alanine, pyruvate and branched amino acid valine that might be related with endogenous glucose production pathway from proteins as well as in the case of T2D. Application of (1)H NMR spectroscopy together with target analysis and chemometric tools based on urine metabolite concentration enable to monitor T1D patients. This methodology can be used as supporting tool for marker HbA1c analysis providing comprehensive information about T1D progression and treatment efficiency.

[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.

[History of diabetology in Lower Silesia]. / Rys historyczny diabetologii na Dolnym Slasku.

The authors presented the history of diabetology in the time before and after the 2nd world war, the outstanding diabetological scientists f.e. professor Minkowski, Stolte, Lublin. Further presented was the actual situation and development of the diabetology in Lower Silesia.

[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.

Panhypopituitary insufficiency in a patient with clinical diagnosis of Chitayat-Hall syndrome.

We report an 8-year-old proband with severe motor and intellectual disability presenting a variety of dysmorphic features such as microcephaly, prominent glabella (ridged metopic suture) and congenital distal limb contractures. As well as panhypopituitary insufficiency, brain defects, e.g. agenesis of corpus callosum, colpocephaly, and pachygyria as well as strabismus and tracheo-laryngeal hypoplasia, were diagnosed. Genetic examination revealed a normal karyotype and excluded Wolf-Hirschhorn syndrome and subtelomeric deletions. Chitayat-Hall syndrome was diagnosed based on clinical traits.

[The effect of one year therapy with recombinant human growth hormone (rhGH) on growth velocity, calcium-phosphorus metabolism, bone mineral density and changes in body composition in children with growth hormone deficiency (GHD)]. / Ocena szybkosci wzrastania i gospodarki wapniowo-fosforanowej, gestosci mineralnej kosci oraz zmiany skladu ciala u dzieci z somatotropinowa niedoczynnoscia przysadki w pierwszym roku leczenia rekombinowanym ludzkim hormonem wzrostu (rhGH).

INTRODUCTION: The most significant effect of growth hormone treatment is growth promotion. For adults the metabolic effect is the most important. This treatment has an influence on the forming of correct composition of body mass, on metabolism of osseous tissue and bone mineral density. AIM OF THE STUDY: Comparison between the rate of growth and estimation of calcium-phosphorus metabolism, bone mineral density and change of body composition in children with growth hormone deficiency during the first year of growth hormone treatment. MATERIAL AND METHODS: The treatment included 120 children and adolescents (85 boys and 35 girls) in age from 6 to 21.5 years old (the average age: 14.2+/-3.0) who were treated in Dept. of Endocrinology and Diabetology for Children and Adolescents during the years 2002-2006 as a result of growth hormone deficiency. Children suffering from panhypopituitarism and other diseases were excluded from the research. The following parameters were included in the analysis: age and sex, age at the start of treatment, the degree of growth hormone deficiency, concentration of calcium, magnesium and phosphates in blood serum, mineral density of bones (BMD), the concentration of osseous minerals, concentration of adipose tissue and lean body mass. All the children were treated with recombined human growth hormone (rhGH) in dose of 0.7 j/kg per week. No interruption of the treatment was noticed. RESULTS: Partial growth hormone deficiency (GHD) was diagnosed in the group of 71 children (52 boys and 19 girls), total GHD diagnosed in 49 cases (34 boys and 15 girls). Average age at the start of treatment was 11.7+/-2.9 years. During the first year of treatment the rate of growing increases from 3.9+/-1,1 cm per year before treatment to 8.72+/-2.27 cm per year (p<0.01) for the whole examined group, for girls from 3.8+/-1.2 cm per year to 8.77 cm per year (p<0.01), for boys from 3.2+/-1.3 per year to 8.58+/-2.20 cm per year (p<0.01). Average concentrations of calcium, magnesium and alkaline phosphatase (ALP) in blood serum remain in accordance with laboratory standards and were statistically not significant. In the majority of patients LBM increased significantly from 28 689+/-4423 g to 35 549.09+/-2968 g (p<0,05) and FM decreased from 21 070+/-4019,8 g (33,15+/-7,6%) to 19 075.37+/-4307,3 g (26.15+/-8.6%) (p<0.05). The alternations of the body composition in GH deficient children treated with rhGH occurred during the first year of therapy.

[Value of physical activity for proper bone mass and bone mineral density attaining in children and adolescents]. / Znaczenie aktywnosci fizycznej dla uzyskania odpowiedniej masy i gestosci mineralnej kosci u dzieci i mlodziezy.

Physical activity is an important factor for healthy life of the humans. Its significance regards mostly the developmental age, when natural mobility of the youth prones to the proper growing of the skeleton and is important in the prevention and therapy of many diseases. The advantageous effect of regular physical activity and different sport disciplines on bone mass and density is described. In the young age, puberty is an ideal moment for attaining the maximal bone mass and density gain due to physical exercising. The possible harmful effect of exaggerated physical activity has been shown. It is connected with hormonal disorders - secondary amenorrhea, delay of menarche, increased injuries and BMD loss together with significant body mass reduction.

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.

[Is arterial hypertension a significant problem in children and adolescents with type 1 diabetes?]. / Czy nadcisnienie tetnicze jest problemem u dzieci i mlodziezy z cukrzyca typu 1?

In the last two decades the problem of arterial hypertension in patients in the developmental age gained an increasing interest. The frequency of arterial hypertension in children was estimated at the level of 1-5% of the population. It was demonstrated that hypertension is observed in patients with type 1 diabetes over two-three times more frequently than in the general population. Arterial hypertension is a significant risk factor for cardio-vascular complications. The coexistence of diabetes type 1 and arterial hypertension predisposes to ischemic heart disease, stroke and premature death. Normal range of arterial tension for children was established during the Task Force on Blood Pressure Control study in children. Models depending on age and sex were created. Measurements above the 95 percentile for age and sex were referred to as significant hypertension and above the 97 percentile as heavy hypertension. For the development of arterial hypertension in patients with type 1 diabetes, which is the dominant type in children and adolescents, apart from the genetic predisposition, the coexistence of nephropathy is important. In children and adolescents almost exclusively secondary nephrogenic hypertension is observed, which develops usually 2 years after microproteinuria. Seldom in children and adolescents with type 1 diabetes essential hypertension or hypertension of other causes, as for example contraction of the nephrotic artery, may be observed. A particular form of arterial hypertension is lack of pressure decrease during the night, with a maintenance of the normal rhythm during the day. Recently the state called "pre-hypertension", considered as a precursor of hypertension and a predictor of excessive cardiovascular risk, has gained increasing interest. The pharmacological therapy of arterial hypertension in patients with diabetes type 1 may be taylored individually, depending on the degree and form of diabetes, and also on its late complications. In case of an unfavourable course dialysis may be considered and transplantation of the kidney, as hemodialysis in children with type 1 diabetes is connected with frequent complications. It is crucial to consider the transplantation of the kidney at early stages, when the creatinine level is above 5 mg/dl. It has been proved that the results of transplantation in patients with diabetes are similar to those in patients without diabetes.

[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.

[Precocious puberty and von Recklinghausen's disease]. / Przedwczesne dojrzewanie plciowe w przebiegu choroby Recklinghausena.

Von Recklinghausen's disease belongs to a group of neurocutaneous syndromes and is characterised by skin, nerve and bone abnormalities. We present a case of von Recklinghausen's disease and precocious puberty in 7-year-old boy. At the age of three café au lait spots on the skin and an incranial tumour situated near the optic chiasm--qualified as inoperable--were discovered. At the age of 7 first signs of precocious puberty appeared (pubic hair P3 and enlargement of the testes (15 ml) and penis). Laboratory measurements included: LH 7.5 mIU/ml, FSH 1.1 mIU/ml, testosterone 183 ng/ml, assessment of bone age: 9 years. The response to LHRH stimulation was characteristic for true precocious puberty (LH 15.9 mIU/ml and FSH 1.5 mIU/ml after 30 minutes). The MRI of the brain showed a tumour of the suprasellar region with compression of pituitary stalk. True precocious puberty was diagnosed. Treatment with Diphereline was introduced. At present the boy is 9 years old and has been treated with Diphereline for 16 months. The volume of the testicles has decreased to 7 ml and loss of pubic hair was noted. The MRI does not show any progression in tumour growth. The authors would like to underline the need of close observation of children with von Reclinghausen disease with regard to possibility of uncovering true precocious puberty which is critical for rapid diagnosis and introduction of correct treatment.

[The child from families with type 1 diabetes]. / Dziecko pochodzace z rodzin obciazonych cukrzyca typu 1.

Diabetes type 1 is observed in individuals with a genetic predisposition to the disease. Observed is a 3-5 fold risk for congenital defects, therefore diabetes type 1 is one of the highest known teratogenic risk factor. The main factor responsible for the development of congenital defects is hyperglycemia. Observed are congenital defects of the central nervous system, the bones, urinary and digestive tract. Characteristic is macrosomia. Observed are hypocalcemia, hypomagnesemia, polycythemia, hyperbilirubinemia, hypertrophic cardiomyopathy, respiratory disturbances. Children from families with diabetes type 1 are at high risk for the development of the disease in the newborn period, additional diseases. They must be in permanent medical control.

[Salt wasting syndrome caused by congenital, insufficient synthesis or aldosterone function--etiology, diagnosis and management]. / Zespól utraty soli spowodowany wrodzona, niedostateczna synteza lub funkcja aldosteronu -- etiologia, diagnostyka i postepowanie.

Salt wasting syndrome is caused by a congenital or acquired synthesis disorder or by the aldosterone function disorder. It manifests itself by ionic disorders where the sodium and chlorine level decrease with the simultaneous potassium retention. Synthesised aldosterone is in the glomerular zone of the adrenal cortex. Symptoms of dyselectrolitemia are not distinctive, they develop within a few first days of life. The suction aversion, apathy, lack of growth or progressing, body mass loss is being noticed. The most often cause of salt wasting syndrome is the congenital cortical adrenal hyperplasia (CAH) caused by 21-hydroxylase enzyme deficit. The classic form with and without salt wasting (SW), as well as non-classic form is distinguished. The therapy of SW form depends on Hydrocortisone and Cortineff administering. The other forms of salt wasting syndrome occur not so often and these are: aldosterone synthesis deficit, dehydrogenase 3beta-hydroxysteroid deficit, lipoid cortical hyperplasia, adrenal hypoplasia congenital (AHC), adrenoleukodystrophy and pseudohypoaldosteronism. The knowledge of the symptoms and causes of salt wasting syndrome allows for the proper therapeutic management and contributes to the regular psychophysical infantile development of the children.

[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.