A successful transition to sulfonylurea treatment in male infant with neonatal diabetes caused by the novel abcc8 gene mutation and three years follow-up.

Neonatal diabetes mellitus is a rare monogenic disease with incidence of 1/90,000 newborns. A case of two months aged male infant with life threatening diabetic ketoacidosis is presented with novel ABCC8 gene mutation (p.F577L), successful transition from insulin to sulfonylurea and follow-up of three years.

Primary pigmented nodular adrenocortical disease: literature review and case report of a 6-year-old boy.

Cushing's syndrome is rare in childhood and is usually caused by a pituitary adenoma. Primary hyperfunction of adrenal glands is less frequent, particularly primary pigmented nodular adrenocortical disease (PPNAD). It occurs usually in children and adolescents, with female preponderance, while Cushing's disease has increased frequency in prepubertal males. A case of a 6-year-old boy is presented with isolated non-familiar PPNAD. The clinical pattern involved Cushingoid appearance, hypertension, virilization and depressive mood. Laboratory analyses showed loss of circadian rhythm of cortisol, undetectable adrenocorticotropic hormone (ACTH) level, impaired fasting glucose, polycythemia and elevated white blood count (WBC). Radiology investigation revealed a slightly enlarged medial branch of the left adrenal gland and a normal right one, so a unilateral adrenalectomy was performed. Pathohistology described multiple dark brownish pigmented nodules of various sizes confined to the cortex. Contralateral adrenalectomy was done 3 months later. Follow-up of 3 years was uneventful, except for one adrenal crisis during an intercurrent respiratory illness.

Risk of the recurrent headache and migraine appearance within the family.

AIM: The aim of this research was to determine the risk for family appearance of the recurrent headache (non-migraine and migraine). METHODS: The research was conducted in Vojvodina, the Northern Province of Serbia. The population of Vojvodina is around 2 million people belonging to more than 20 different ethnic groups. During the 20-year period (1988-2008), 30,363 children aged 3 to 17 years were tested, independent of their place of birth. The presence of headaches similar to those tested was compared among all the members of the family within three generations. RESULTS: Positive family data of the recurrent headaches were detected among 98.6% children with migraine headaches, 64.7% children with non-migraine headaches, and 32.4% children without recurrent headaches. The relation among the members of the nuclear family (contingency quotient of 0.429) was significantly stronger than the relation to the members of wider family (contingency quotient of 0.338). CONCLUSIONS: The probability of a child having the migraine headache rather than the non-migraine one was very high for parents and high for father's mother, while it was not significant for mother's mother, mother's father and father's father, having similar recurrent headaches.

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).

[Risk of vaccination in children with epilepsy].

INTRODUCTION: The central nervous system, previously considered as "immune privileged", does exhibit features of inflammation in response to injury, infection or disease. We do not know its reactions on immunization. We do not know how common febrile seizures after vaccination are and if there are subgroups of children at higher risk. What is the long term outcome for children who had afebrile seizure after vaccination? Can the vaccine be a direct cause of a condition called an epileptic encephalopathy, where seizures damage the brain with the resulting epilepsy? DISCUSSION AND CONCLUSION: It should not be forgotten that "benign infective childhood diseases" can, and do, kill, and that vaccines are a public health intervention saving many millions of lives around the world. Parents as well as doctors have fear: whether vaccinations can cause convulsions, epilepsy or encephalopathy. Large studies of this issue have produced conflicting results, although the recent consensus is that the risk of vaccine-induced epilepsy and or encephalopathy, if it exists at all, is extremely low. It is necessary to establish a proposed immunization program for children at neurologically high risk and for children with epilepsy to protect them and the whole population from infectious diseases, children from immunization adverse events, and avoid possibilities of legal trial. It is necessary to know everything about the risks and benefits of immunizations for each child. For each child, the risks of the disease, and its squeal, must be compared with the vaccine's protective efficacy and potential adverse reactions. Vaccination is given preference in nearly all children with epilepsy.

[Cerebral palsy and epilepsy].

INTRODUCTION: Cerebral palsy is the most common cause of physical disability in early childhood. Epilepsy is known to have a high association with cerebral palsy. All types of epileptic seizures can be seen in patients with cerebral palsy. Complex partial and secondary generalized ones are the most frequent seizure types. In persons with cerebral palsy and mental retardation, the diagnosis of epilepsy presents unique difficulties. Generally they are not able to describe the epileptic events themselves, parents are not able to describe them without fear and persons trained in epilepsy witness the events only rarely. Some syndromes, such as infantile spasms. West and Lennox-Gastaut syndrome, are particularly frequent, whereas children with cerebral palsy are rarely free of epilepsy. It has been observed that epileptic seizures in children with cerebral palsy tend to have an earlier onset; they often appear in children with cerebral palsy and mental retardation; they are more severe in patients with a more severe degree of cerebral palsy. The overall outcome of seizures in children with cerebral palsy is poor, requiring prolonged course of antiepileptic medications, polytherapy with higher incidence of refractory seizures and hospital admissions for status epilepticus. The presence of a neurological deficit, as well as cerebral palsy, does not necessarily mean a poor prognosis after the discontinuation of antiepileptic drugs, but the risk of a relapse in persons with cerebral palsy is high. AIM: The objective of the paper was to show the relationship between cerebral palsy and epilepsy and to determine the occurrence, associated factors, nature and prognosis of epilepsy in children with cerebral palsy.

[Children migraine syndrome--definition and classification through the time].

INTRODUCTION: Even today, the migraine syndrome is not completeley defined knowing that it does not have either clear laboratory correlate or objectively defining marker. There is a great number of differential diagnostic references to define and classify migraine headaches; from Galen to ICHD-II (International Classification of Headache Disorders); from September 2003. Migraine syndrome represents a current problem of both paediatrics and children's neurology. The aim of this paper is to understand and define the migraine syndrome among children through the history of medical science, in order to enable the interpretation of the most acceptable defining and classification criteria of the children's migraine syndrome. DEFINITION AND CLASSIFICATION: Sensory, vegetative and affective phenomena of migraine, recognizable only among people, with striking quantitative and qualitative variations depending on the patient, define the migraine syndrome of children in general. There are no completely reliable principles or guidelines which would enable the accurate, precise and quick diagnosis, or differential diagnosis of children's migraine syndrome. Vahlquist is the first one to give special criteria to diagnose children's headache. His criteria were invalid because of insisting on headaches being unilateral. Classification criteria for migraine in children were given by Bille in 1962, Prensky in 1976, Deubner in 1977, Congdon and Forsythe in 1979, Tomasi in 1980, Sillanappa in 1982, Kurtz and Barlow in 1984, Hockaday in 1988. IHS classification was brought by consensus in 1988, and it was last modified in September 2003, when a consensus was reached about applying the ICHD-II International Classification of Headache Disorders. CONCLUSION: Being insufficiently defined and incompletely etiologically, clinically and therapeutically clear, children's migraine syndrome represents a striking example of interdisciplinary, scientific, health, practical and clinical entity. The degree of interest in children's migraine syndrome is directly dependent on the level of social and health standards within the society.

[Ohtahara syndrome--early infantile epileptic encephalopathy].

DEFINITION: Ohtahara syndrome (early infantile epileptic encephalopathy with suppression bursts), is the earliest developing form of epileptic encephalopathy. ETHIOLOGY: It considered to be a result of static structural developing brain damage. CLINICAL PICTURE: Variable seizures develop mostly within the first 10 days of life, but may occur during the first hour after delivery. The most frequently observed seizure type are epileptic spasms, which may be either generalized and symmetrical or lateralized .The tonic spasms may occur in clusters or singly, while awake and during sleep alike. The duration of spasms is up to 10 seconds, and the interval between spasms within cluster ranges from 9 to 15 seconds. In one third of cases, other seizure types include partial motor seizures or hemiconvulsions The disorder takes a progressively deteriorating course with increasing frequency of seizures and severe retardation of psychomotor development. DIAGNOSTIC WORKUP: In the initial stage of Ohtahara syndrome, interictal EEG shows a pattern of suppression-burst with high-voltage paroxysmal discharges separated by prolonged periods of nearly flat tracing that last for up to 18 seconds. PROGNOSIS AND TREATMENT: Half of the reported children having Ohtahara syndrome die in infancy. Anticonvulsant helps little in controlling the seizures and halting the deterioration of psychomotor development. Severe psychomotor retardation is the rule. With time, the disorder may evolve into West syndrome or partial epilepsy. Psychomotor development may be slightly better if the infants do not develop West and later Lennox-Gastaut syndrome.

[Predisposing factors of the migraine syndrome in children].

INTRODUCTION: The aim of this paper was to define characteristics which could be used to recognize groups of children at risk for migraine syndrome. MATERIAL AND METHODS: Predisposing factors of migraine syndrome were investigated by conducting a poll among 24.828 children aged 3-16, in Vojvodina, during the period between 1988-2004. RESULTS: For the purpose of defining recognizable characteristics of children with migraine syndrome, a comparison of children with migraine headaches, non-migraine headaches, and those without headaches was done among 24.828 children. Apart from direct heredity, the following factors should be accepted as predisposing factors of migraine syndrome: child's order of birth (second child), length of breast-feeding, age at which the ready-made industrial food was introduced, as well as the age at which the child started the whole-day stay (which is in inverse proportion to the risk of migraine attacks and the age of the first symptoms), particularities in behaviour pattern, family social status, atmosphere in the family, previous paroxysmal torticollis, episodic vertigo of unknown etiology, recurrent abdominal pain, and lack of cerebral hemisphere dominance. CONCLUSION: Children with migraine headaches have particularities, not clear enough for the time being, but recognition of which is necessary for early prevention of migraine syndrome in childhood.

Specific features of migraine syndrome in children.

The aim of the study was to define factors that can be used to distinguish migraine headaches from primary non-migraine headaches. Specific characteristics of headaches were analysed in 30,636 children aged 3-17; 18.97% had recurrent primary non-migraine headaches, whereas 8.63% had migraine headaches. Migraine attacks follow identical patterns (94.9%): occurring monthly (78.0%), occurring in morning hours (58.5%), lasting for several hours (45.1%) and ending after sleep (76.7%). Nausea, vomiting impulse and vomiting are basic present elements of migraine attacks in children. Canonical discriminate analysis defined the following statistically significant factors, which can distinguish migraine headaches from primary non-migraine headaches in children: relief after sleep (0.945), vomiting impulse (0.945), photophobia (0.523), nausea (0.379), phonophobia (0.354) and vomiting (0.330).