Migraine in children-migraine syndrome in children of Vojvodina.

To assess the incidence of children with various types of migraine, an investigation was carried out from 1988 to 2008, on 30636 children (50.38% male, 49.62% female), in nine towns of the north province of Serbia. Migraine was reported in 8.63% children aged 3 to 17 (8.0% male, 9.6% female) as well as in 3.87% children aged 3-7 (4.2% boys, 3.57% girls). The proportion of children with either migraine or non-migraine headaches increased with their age, from 2.65% to 11.72% in boys, and from 2.71% to 15.86% in girls. Such increasing trend was also found for migraine with aura (from 1.8% to 32.7%). Children with migraine with aura showed their pick at the age of 9, while other migraine syndromes had the most frequent appearance at the age of 5 years. Migraine with aura accounted for 25.55%, migraine without aura for 67.21% and other migraine syndromes for 7.23% of investigated subjects with migraine. The average age of the migraine diagnosis was 5 years 1.8 months, while it was 4 years 11.4 months for migraine with aura, 5 years 7.2 months for migraine without aura and 3 years 7.2 months for other migraine syndromes. Migraine is much more frequent in second born children and in these from incomplete families. Migraine with aura appeared mostly in children coming from family with median socioeconomic welfare, while poor family welfare was predominant in children with migraine without aura. The length of breast-feeding influenced the appearance of the migraine in general, showing reciprocal influence of the length of breast-feeding on the appearance of migraine. Migraine was more often found (39.4%) among children who earlier joined nursery schools on a whole-day stay basis. Children with migraine have been diagnosed and treated by neuropaediatricians or neurologists in 55.4% cases (70.0% migraine with aura, 42.7% migraine without aura and 92.4% children with other migraine syndromes).

[A case report of pyridoxine-responsive homocystinuria]. / Slucaj piridoksinresponzivne homocistinurije.

INTRODUCTION: Homocystinuria is a rare, congenital, autosomal-recessive, metabolic disease biochemically characterized by homocysteinemia and homocystinuria and by multi-system clinical disorders. It is a biochemical abnormality of methionine metabolism caused either by transulfuration pathway disorders or by disorders of homocysteine remethilation into methionine and as such it can be a result of numerous specific and different genetic lesions. CASE REPORT: Homocystinuria is most commonly caused by deficiency of cystationine beta synthetase enzyme which catalyses the synthesis of cystathionine from homocysteine and serine in the methinione pathway. This results in accumulation of homocysteine and methionine in plasma and leads to excretion of excessive urinary homocysteine. Depending on specific property of the mutant enzyme molecule some patients respond to very high doses of pyridoxine with decreases of methionine and homocystine and some not. Pyridoxine responsiveness is based on the presence of small residual activity of cystathionine beta synthetase which is not present in nonresponsive patients. Homocystinuria due to cystathionine beta-synthase (CBS) deficiency is characterized by numerous different clinical abnormalities, but changes in four organ systems are dominant (eye, skeletal, central nervous and vascular system). CASE DESCRIPTION: Six years ago a six year-old boy was admitted to the hospital with vision problems. Ophthalmologic examination revealed a lens dislocation and because of many stigmates the child was sent to endocrinologist. The child had a marfanoid stature, with pectus carinatum and genu valgum, ataxic gait with motoric discoordination, muscle tone which ranged from hypotonia to hypertonia of extrapvramidal type and low intellectual abilities. A simple cyanide-nitroprusside test of patient's urine was positive suggesting homocystinuria. The diagnosis was established after plasma and urine amino acid analysis by HPLC. Concentration of homocystine and methionine were 52 mumol/l and 57 mumol/l in plasma and 249 mumol/du and 55 mumol/du in urine, respectively. After four months of treatment with pyridoxine there were not any significant changes in plasma homocysteine and methionine, but at the same time decrease in urine of these two amino acids were more than 2.5 times higher. This confirms that the patient has a pyridoxine-responsive type of homocystinuria and the dose was increased to 60 mg/day and 600 mg/day. This results in further decline of homocysteine and methionine in plasma and urine which persists up to now (for six years).

Predictive value of biological markers in etiopathogenesis of juvenile diabetes--reviewing the role of insulin antibodies.

The lack of complete concordance for diseases in monozygotic twins prevents application of genetic markers for a thorough identification of the subjects who will develop the type I diabetes. Furthermore, the impact of the environmental factors precipitating beta cells destruction in genetically sensitive subjects has not been completely enlightened yet. The identification of high risk markers for the development of diabetes is aimed at detection of the early immune response activation markers. Islet cell antibodies are the most valuable markers, whose presence can be discovered even up to 7-8 years prior to the onset of symptoms. They are found in 50-80% of the newly discovered insulin-dependent diabetics. Their prevalence in the general population is 0.5-2%. These are commonly concomitant with insulin antibodies, found in 20-40% of the newly discovered diabetics, as reported in the literature. In our circumstances it was possible to determine the insulin antibodies only. We have concluded that they appear in 13.6% of children with a newly discovered diabetes, presenting a significant marker for predicting the course of the disease.

[Meteorotropism in children with cerebral palsy]. / Meteorotropizam u dece s cerebralnom paralizom.

INTRODUCTION: Members of the Rehabilitation team and families of children with cerebral palsy are daily engaged in various activities with these children. Weather changes (the atmospheric pressure especially) on particular days, cause difficult performance of anticipated therapeutic exercises, occupational therapy, speech therapy, education, daily living etc. The aim of this study was to underline the possible connection between performing daily activities of children with cerebral palsy and weather changes. MATERIAL AND METHODS: There were 16 children with cerebral palsy, 2 to 14 years of age, 8 females and 8 males. Parents of these children got a questionnaire where they had to write down, on daily bases, one of the following: A--correct and easy to perform exercises, B--difficult to perform exercises, C--impossible to perform exercises--the child indisposed, for a period of three months. For the same period of time the atmospheric pressure changes and the temperature were recorded by a professional. RESULTS: Our findings are related to the relationship between the efficiency of physical treatment in children with cerebral palsy and meteorological phenomena. The weather changes did not influence about 75% of the patients. In the other group of patients the unsuccessful exercise was associated with periods of extensive cyclonic activity: 15 to 17 of March 1991 (the decrease of atmosperic pressure from 1010 to 999 mbara), 15 to 17 of April 1991 (the decrease of atmospheric pressure from 1000 to 987 mbara), 5 to 7 of May 1991 (the decrease of atmospheric pressure from 1000 to 987 mbara). DISCUSSION: Before and during cyclonic activity increases the concentration of the positive air iones which interferes with neuromuscular excitation and causes serotonin hyperproduction in the central nervous system, thus causing irritation and the lack of motivation for imposed demanods. CONCLUSION: About 23% of children with cerebral palsy show meteorotropism in terms of weather changes influencing the efficiency of exercises. The cyclonic activity has unfavourable influence on exercises, while the stable atmospheric pressure or anticyclonic activity has a favourable one in children with cerebral palsy who show meteorotropism. So, favourable and unfavourable days for therapeutic exercises can be clearly differed.

[Homocystinuria--biochemical, clinical and genetic aspects]. / Homocistinurija--biohemijski, klinicki i genetski aspekti.

Homocystinuria is a rare autosomal recessive disease characterized by homocystinuria and multisystemic clinical disorders. The term denotes a biochemical abnormality of methionine metabolism caused both by transsulfuration pathway disorders and remethylation of homocysteine into methionine, and as such it can be a result of numerous specific and different genetic lesions. Homocystinuria is most commonly caused by deficiency of cystathionine beta-synthase (CBS) activity (EC 4.2.1.22). In this lesion, depending on specific characteristics of mutant enzyme molecules, in regard to existence of residual activity, responsive and nonresponsive homocystinuria can be differed regarding clinical response to high doses of pyridoxine. Although there are numerous different clinical abnormalities, changes on four organ systems are dominant. The most common symptoms of homocystinuria include lens dislocation, vascular disorders, skeletal abnormalities and mental retardation. Laboratory findings are the first diagnostic procedure, while determination of enzymatic activity is a direct parameter for making diagnosis. Prenatal diagnosis and early detection are extremely important for the course and prognosis of the disease as they enable application of currently available therapy as soon as possible. The presently available therapy can, only in such cases, prevent occurrence of serious clinical symptoms, prevent their advancement to some extent or improve reversible clinical manifestations.

[Problems and perspectives in child health care]. / Problemi i perspektive zdravstvene zastite dece.

Health care protection of children in Vojvodina is of particular importance regarding the negative natural birth rate. In spite of difficult economic situation, health care of children in Vojvodina is permanently carried out and would be significantly better in quality if the education of subspecialized personnel, space facilities, technological innovations, computerization and continuous education were available. Introduction of microanalytic laboratory techniques is essential for monitoring of prematures. Propagation of natural nutrition is an obligation of pediatricians. Respiratory diseases are still on the top of morbidity pyramid but tuberculosis is evidently in increase. An important health care problem is the expansion of allergic diseases. The progressive incidence of insulin-dependent diabetes is also evident. The spheres of juvenile gynecological endocrinology and andrology are still underdeveloped and that also applies to adolescent medicine. Toxicology remains an ongoing problem in pediatrics due to an increased number of accidental poisoning. Pediatricians-gastroenterologists are lacking while paediatric hepatology should be brought into more advanced state. Bone marrow and stem cells transplantation is in the responsibility of the Centre for haematology and oncology. Nephrology department lacks children's haemodialysis, ultrasound biopsy of kidneys, urodynamic analyses and new staff facilities. The increased number of survivals in case of children with with sequeles inevitably asks for better development of rehabilitation, prolonged treatment and teamwork. Intensive care and therapy department requires new staff and high technology capacities. Development of children's surgery department inevitably means the reconstruction of space facilities, modern equipment and new subspecializations. Preventive outpatient service is performed through systematic examinations. ultrasound diagnostics of hips, auditive screening and educational program activities related to addiction and veneral diseases. Genetic health of the population is supremely covered by the Center for Medical Genetics, with the tendenca for development of molecular genetics. It is necessary to develop rehabilitation service, prolonged treatment and teamwork. Diagnostic of convulsive conditions should be advanced with more refined diagnostic methods.

[Bleeding in hemophilia as a function of meteorologic changes and the effect of tissue hormones]. / Krvarenje kod hemofilije u funkciji meteoroloskih promena i svetlu tkivnih hormona.

Hemophilia is a hereditary coagulopathy which has the characteristic of bleeding into various tissues, particularly joints and muscles, bearing the risk of early invalidity. A lot of bleedings in children with hemophilia occur without any recognizable trauma, spontaneously and during the night. By comparing the frequency of bleeding in hemophiliacs with meteorological phenomena, and by the one year material revision of the Hematology Department of the Institute for Child Care in Novi Sad (Yugoslavia) as well as the simultaneous recording of barometric pressure, it was found that the increase of atmospheric pressure was strongly associated with spontaneous bleedings in hemophiliacs. It is accompanied with a rise in negative air ions that when inhaled interact with platelets and decrease blood serotonin, thus blocking its vasoconstrictor effect and precipitating bleeding. Antiserotonin activity drugs can have a similar influence.

[The McCune-Albright syndrome with hyperthyroidism, acromegaly and hypophosphatemic-hyperphosphaturic rickets]. / McCune-Albrightov sindrom sa hipertireoidizmom, akromegalijom i hipofosfatemicno-hiperfosfaturicnim rahitisom.

A girl was described with an unusual combination of McCune-Albright's syndrome (polyostotic fibrotic dysplasia, hyperpigmentations on the skin and precocious puberty) with hyperthyroidism, hypophosphatemic-hyperphosphaturic rickets and acromegaly. Although the pathogenetic mechanism involved in the development of this endocrinopathy in this syndrome is not quite clear, the achieved results, as well as data of other researchers, suggest that the endocrinological disorders in this syndrome are the result of either an increased sensitivity of periphery endocrine organs or/and of an autonomous hyperfunction (similar to multiple endocrine adenomatosis).

[Menarche in schoolgirls in Novi Sad]. / Menarha kod ucenica u Novom Sadu.

Out of 947 examined schoolgirls aged 8.5-15 from the two schools in Novi Sad, 327 of them had menarche. Arithmetic mean value of the appearance of menarche was 12.25 years, the standard deviation being 0.91 years. Median was 12.59 years. The normal appearance of menarche (X +/- 2 SD) ranged from 10.4-14.1 years in our environment. According to X2 (Chi-square) test and asymmetry coefficient (beta 1) it was pointed out that the distribution of menarche in examined schoolgirls was normal and symmetric.

[Metabolism of (1-14C) cholesteryl oleate in the brain of the cat]. / Métabolisme de 1'(-1-14C) oléate de cholestéryle dans le cerveau du chat.

Following injection into the cerebral ventricles of conscious cats, cholesteryl oléate (-1-14C) was taken up, hydrolysed and included into a variety of brain lipids. The peak of uptake of cholesteryl oleate (-1-14C) into the brain tissue was obtained 4 h after its administration. The peak of hydrolysis occurred 48 h following intraventricular administration of cholesteryl oleate (-1-14C). Cholesterol esters and free fatty acids decreased, while phospholipids increased within 48 h following injection of labelled cholesteryl oleate. Small percentage of the radioactive material was found in triglycerides, monoglycerides and diglycerides. The radioactive material was found in the brain tissue surrounding the cerebral ventricles and in the cerebral cortex, but in varying amounts: the highest in the thalamus and in the caudate nucleus, while the smallest in the cerebral cortex. The labelled material in these structures increased within first 4 h after its intraventricular injection and thereafter slowly decreased, but by the end of 48 h it was still retained in appreciable amounts.

[Distribution, uptake and hydrolysis of cholesterol oleate (-1-14C) in brain tissue after its injection in the cerebral ventricles of the conscious cat]. / Distribution, captation et hydrolyse de (-1-14C) oléate de cholestéryle dans le tissu cérébral après injection dans les ventricules cérébraux du chat conscient.

Distribution, uptake and hydrolysys of cholesteryl oleate (-1-14C) in vivo in the cat's brain were investigated. Four hours following intraventricular injection of cholesteryl oleate (-1-14C), about one third of the injected radioactivity was taken up and about 50 % of that radioactive material was hydrolysed by brain tissue. Even more, the labelled material was found in the brain tissue surrounding the cerebral ventricles as well as in the cerebral cortex, but in varying amounts: the smallest amounts were found in the cerebral cortex, while the highest were in the hypothalamus. Radioactive material was also detected in the peripheral venous blood.