bluuebunny/crossref_metadata_2025 huggingface dataset:
Neurorazvojni ishodi i rezultati slikovnih pretraga mozga u petero djece s ranom novorođenačkom sepsom i meningoencefalitisom uzrokovanima beta-hemolitičkim streptokokom skupine B
Authors: Balažin Ana , Đaković Ivana , Bošnjak Vlatka Mejaški
Published: November 2024

Cilj je prikazati petero bolesnika koji su preboljeli meningoencefalitis u sklopu rane novorođenačke sepse uzrokovane beta-hemolitičkim streptokokom skupine B, s naglaskom na njihovim perinatalnim čimbenicima rizika, nalazima ultrazvuka i magnetske rezonancije mozga te neurorazvojnim ishodima. Iz arhiva Klinike za dječje bolesti Zagreb, Zavoda za neuropedijatriju retrospektivno suprikupljeni podatci za razdoblje 2008. - 2011. godine. Svi su bolesnici rođeni u terminu. Dvije majke su bile febrilne tijekom porođaja,a jedna je primila amoksicilin s klavulanskom kiselinom. Za četvero bolesnika nisu dostupni podatci o majčinoj kolonizaciji utrudnoći, ni o primjeni intrapartalne antibiotske profi lakse. Dvoje bolesnika je imalo blaže neurorazvojno odstupanje u vidu oštećenjarazvoja govora, odnosno blagog oštećenja razvoja motorike, s proširenjem moždanih komora i subarahnoidalnih prostora, odnosno leukomalacičnim cističnim lezijama frontalno u slikovnim nalazima. Troje bolesnika je imalo višestruko neurorazvojno odstupanje; dvoje pridruženo oštećenje vida, epilepsiju i hidrocefalus s ventrikuloperitonealnom drenažom, a bolesnik s najlošijim ishodom uz navedeno i oštećenje sluha. U slikovnim nalazima ovih bolesnika dominirale su brojne leukomalacične cistične lezije,uglavnom lokalizirane u telencefalonu, uz različit stupanj proširenja moždanih komora i stanjenja korpusa kalozuma. U nalazimaultrazvuka i magnetske rezonancije mozga prate se destruktivne lezije, s predilekcijom za područje telencefalona. Rana novorođenačkasepsa s meningitisom uzrokovana beta-hemolitičkim streptokokom skupine B ima loš ishod u donošene novorođenčadi; troje odpetero bolesnika imalo je teško višestruko neurorazvojno odstupanje. S obzirom na postojanje učinkovitih metoda prevencije oveteške bolesti u novorođenčadi, postoji potreba za donošenjem i sustavnim provođenjem nacionalne preventivne strategije.
Wilson’s disease
Authors: Relja M. , Miletić V.
Published: November 2024

Wilson’s disease (hepatolenticular degeneration), an autosomal recessive inherited disorder of copper metabolism, is one of the very few chronic neurologic diseases for which specific and effective treatment is available. The longer the recognition and diagnosis is delayed, the greater is the risk of permanent damage primarily to the liver and/or brain. On the contrary, early treatment may reverse even long-lasting symptoms. The disease is more frequent than it is usually believed. Unfortunately, a misdiagnosis appears early in the course of the disease. Diagnostic dilemmas have for the most part not been resolved by the identification and cloning of the WD gene. On the basis of the authors’ own long-term results in adult neurologic patients with Wilson’s disease, along with the published studies from the literature, the authors give a survey of the current knowledge about hepatolenticular degeneration.
Epilepsy and psychopathology in children and adolescents
Authors: Grgić M. , Perković M. , Butković S. Soldo
Published: November 2024

A group of 20 children and adolescents hospitalized and treated at the Department of Child and Adolescent Psychiatry, University Department of Psychiatry, Osijek University Hospital Center for epilepsy and psychiatric comorbidities were included in the study. The aim of the study was to investigate psychiatric comorbidity and cognitive characteristics of children with epilepsy and compare them according to gender. Diagnoses were based on the current ICD-10 classification criteria, whereas the following standardized psychological tests were used to determine cognitive characteristics of patients: Colored Progressive Matrices, Standard Progressive Matrices, Cognitive Nonverbal Test, Bender Motor Gestalt Test, Revised Beta Test, and Auditory Verbal Learning Test. Study results showed hyperkinetic disorder alone or in combination with conduct disorder to be the most common comorbidity in boys. On the other hand, the most common comorbidity diagnosed in girls was depressive disorder and anxiety disorder alone or combined with primary nocturnal enuresis. According to the results of psychological testing, most girls had average intellectual abilities; 69.23% of them showed signs of disorders of visuomotor coordination, whereas 61.54% showed signs of short-term memory impairment. Unlike girls, most boys (57.14%) had either borderline or subnormal intellectual abilities. All boys showed signs of disorders of visuomotor coordination and signs of short-term memory impairment. Early identification and treatment of comorbid psychiatric diagnoses are important components in determining optimal and comprehensive treatment of children with epilepsy.
Barth syndrome X-linked cardiomyopathy with neutropenia
Authors: Malčić I. , Vidaković M. , Šarić D. , Dilber D. , Kniewald H. , Bartoniček D.
Published: November 2024

We report on a child with Barth syndrome. The child had a positive family history (X-linked disease) with characteristic clinical symptoms (severe dilated cardiomyopathy, generalized myopathy with hypotonia) and typical biochemical findings, cyclic neutropenia and elevated urinary level of 3-methylglutaconic acid. Molecular analyses identified mutation in the second exon of TAZ gene, p.Arg94His (c.281G>A) mutation. The child is alive and requires cardiac medications: diuretics, ACE inhibitors, digoxin, β-blockers and anticoagulants.
A pediatric patient with familial mediterranean fever presented with vomiting
Authors: Arslan N. , Gencpınar P. , Makay B. , Bora E. , Ünsal E.
Published: November 2024

Familial Mediterranean fever is inherited as an autosomal recessive trait affecting mainly persons of Mediterranean descent, especially Arabs, Jews, Armenians and Turks. Fever, peritoneal and pleural inflammation, arthritis, erysipelas-like erythema, and arthralgia are well known features of familial Mediterranean fever. We herein report a three-year-old boy, who presented with vomiting and agitation attacks and was found to be a compound heterozygote for M694V/M680I mutation of the MEFV gene.
Subacute sclerosing panencephalitis
Authors: Anlar B.
Published: November 2024

Subacute sclerosing panencephalitis is a manifestation of persistent measles virus infection in the brain. It affects about 1/10 000 children who experience natural measles infection, but the risk is about 10 times higher if measles infection occurs below 1 year of age. Recent measles epidemics in central Europe will bring the possibility of this complication into consideration in the future. The diagnosis of subacute sclerosing panencephalitis should be considered in a child, adolescent or young adult with behavioral changes, myoclonia, forgetfulness, or deterioration of cognitive functions developing over weeks or months. Electroencephalogram is typical and measles antibody titers in the cerebrospinal fluid are diagnostic. Treatment includes immunomodulatory and antiviral agents, but results in relative remission or stabilization in less than 30% of patients. The fact that spontaneous remissions are also observed suggests host immunity as the main factor affecting outcome; therefore, supportive care and immunomodulatory agents constitute principal aspects of treatment.
Autoantibodies in encephalopathies
Authors: Vincent A.
Published: November 2024

There have been several advances in identifying antibody-mediated central nervous system diseases. These are associated with the presence of antibodies to neuronal or glial cell surface proteins, principally ion channels, receptors and associated proteins. Although first described in adults, they are now being identified also in children of all ages. These conditions are important to recognise because they often respond to immunotherapies.
Guidelines of the Croatian Society of Pediatric Neurology for the diagnosis of floppy infant
Authors: Barišić N. , Kuzmanić-Šamija R.
Published: November 2024

Floppy infant syndrome is a common diagnostic problem in newborns and infants. Clinical diagnosis is based on restriction of spontaneous motor activity, joint hypermobility and lack of resistance on passive movement examination. Floppy infant syndrome may be caused by the central nervous system, neuromuscular, connective tissue, endocrinological, chromosomal, genetic and metabolic disorders. The diagnostic work-up in many instances is often complex and requires multidisciplinary assessment. As the etiology is heterogeneous and diagnostic procedures are complex, rational approach to the diagnostic process is of utmost importance. In this paper, we present recommendations for the evaluation of children with hypotonia depending on the child’s age and clinical picture.
The Croatian Society of pediatric neurology guidelines for the diagnosis and therapy of febrile seizures
Authors: Šojat Lj. Cvitanović , Juraški R. Gjergja , Prpić I. , Barišić N.
Published: November 2024

Febrile seizures are occasional cerebral seizures that occur in 2%-5% of children aged 6-60 months. Despite progress in the understanding of febrile seizures pathophysiology and the development of its guidelines, there are still many variations in the interpretation of these guidelines and in the evaluation and treatment of children with febrile seizures in the Republic of Croatia. Therefore, the Croatian Society of Pediatric Neurology developed the guidelines for the rational diagnosis and therapy of febrile convulsions, which should be feasible in all hospitals in the Republic of Croatia. The purpose of these guidelines is to assist in making rational decisions for diagnosis and treatment and they do not represent the only possible protocol for all patients with this disorder. An additional specific objective is to emphasize the medical evidence based approach to the risk and benefit of continuous and intermittent prophylactic antiepileptic therapy, as well as to the use of antipyretics in children with febrile seizures. Despite the high prevalence and percentage of relapses of febrile seizures, long lasting effects are not known nor scientifically proven in the literature. The risk of developing epilepsy in these patients is low, although slightly higher than in the general population. There is no evidence that the prophylactic use of antiepileptic drugs in children with simple febrile seizures can reduce the incidence of epilepsy. There is no evidence that the use of intermittent diazepam prophylaxis of simple febrile seizures is rational and reasonable. Continuous prophylaxis with antiepileptic drugs or intermittent diazepam therapy may reduce the incidence of recurrent febrile seizures. However, the risk of side effects and potential toxicity of antiepileptic drugs beyond the already low risk of complications in children with simple febrile seizures does not recommend continuous or intermittent therapy with antiepileptic drugs in children with simple febrile seizures. Antipyretics have not been shown to be effective in preventing recurrence of febrile seizures.
Neurofibromatoses: I. Clinical recommendations for the diagnosis, treatment and multidisciplinary monitoring of children with neurofibromatosis type 1
Authors: Sabol Z. , Juraški R. Gjergja , Šižgorić M. Kovač , Sabol F. , Sabol Lj. Kipke , Bošnjak V. Mejaški , Šojat Lj. Cvitanović , Rešić B. , Sasso A. , Hafner K. , Dorešić J. Petrinović , Oršolić K. , Kapitanović S. , Barišić N.
Published: November 2024

Neurofibromatosis type 1 (MIM 162200) is one of the commonest autosomal dominant disorders in man. The NF1 gene is a large and complex gene localized on chromosome 17q11.2. Several different techniques of DNA analysis have been applied to look for specific mutations in NF1 gene. Clinical diagnosis is based on NIH NF1 criteria. For neurofibromatosis type 1 in children, the main aspects of interdisciplinary care are timely detecting and controlling of treatable complications. For longitudinal monitoring of the development of age-dependent clinical neurofibromatosis type 1 signs and complications, the recommended diagnostic and follow-up protocol for children with Neurofibromatosis type 1 is having an annual review of the following aspects: genetic counseling – at diagnosis; history and physical and neurological examination: general development (neuromotor/cognitive, speech/language function), dermatological (café au lait spots, freckling, cutaneous/subcutaneous and plexiform neurofibromas), skeletal (pseudarthrosis, scoliosis), ocular symptoms, growth measurement (short stature, macrocrania), pubertal development (delayed/precocious puberty), blood pressure check (renal artery stenosis) – yearly; cardiovascular examination (congenital heart disease) – at diagnosis; ophthalmological examination (visual acuity, funduscopy, visual fields, tonometry, ultrasound of eyes and orbit, search for Lisch nodules) – yearly; brain magnetic resonance imaging – at diagnosis >2 year, more frequently only if symptoms or signs are present; electroencephalogram – at diagnosis, later according to clinical indication; abdominal ultrasound (abdominal neurofibromas) – every 5 years; full spine magnetic resonance – at age 15 years; and molecular DNA-based testing (linkage analysis for familiar neurofibromatosis type 1, a pathogenic mutation in NF1 gene for sporadic cases). Children with neurofibromatosis type 1 should present on regular annual basis to a specialist familiar with neurofibromatosis in collaboration with their pediatrician or family practitioner. Treatment of complications of neurofibromatosis type 1 is symptomatic.
Neurofibromatoses: II linical recommendations for the diagnosis, treatment and multidisciplinary monitoring of patients with neurofibromatosis type 2 and schwannomatosis
Authors: Sabol Z. , Sabol F. , Šižgorić M. Kovač , Sabol Lj. Kipke , Klancir S. Bela , Gjergja Z. , Juraški R. Gjergja , Šojat Lj. Cvitanović , Krakar G. , Rešić B. , Sasso A. , Hafner K. , Oršolić K. , Marn B. , Dorešić J. Petrinović , Barišić N.
Published: November 2024

Neurofibromatosis type 2 is a neurocutaneous disease with an autosomal dominant pattern of inheritance. The NF2 gene is a tumor suppressor gene located on chromosome 22q12.1 and encodes the protein called merlin or schwannomin. Neurofibromatosis type 2 is characterized by the development of schwannomas of cranial nerves (usually bilateral involvement of the eighth nerve), spinal and peripheral nerves, multiple meningeomas, ependymomas, and ophthalmological changes (presenile cataract). The onset of neurofibromatosis type 2 symptoms occurs in the second and third decades of life. In about 10% and 18% of patients, they become evident at 10 and 15 years of age, respectively. There is currently good agreement on the criteria for the diagnosis of neurofibromatosis type 2 and follow-up protocols for neurofibromatosis type 2 affected/suspected and at risk subjects. Patients with neurofibromatosis type 2 should be periodically re-evaluated by a multidisciplinary medical team familiar with the disease. Longitudinal care includes the following: detailed medical history/genetic counseling/DNA analysis (at diagnosis), clinical assessment (dermatological, neurological, and ophthalmological (yearly); audiological tests with audiometry and brain stem auditory evoked response (BAER) (yearly); and brain and full spine magnetic resonance imaging (yearly). In first degree relatives of neurofibromatosis type 2 patients (at risk subjects), clinical monitoring includes the following: ophthalmological examination in the first two years of life (for asymptomatic congenital cataract); DNA analysis; clinical assessment (neurological, ophthalmological, and audiological) until the teens; and certainly brain and full spine magnetic resonance imaging at 15 and 30 years. If the results of these examinations are normal, follow-up can cease. The mainstay of treatment of NF2 complications is surgical removal of symptomatic tumors. Schwannomatosis is a third form of neurofibromatosis characterized by multiple schwannomas of cranial, spinal and peripheral nerves, but no bilateral vestibular schwannomas. The gene for schwannomatosis, called INI1/SMARCB1, is localized on chromosome 22 near the NF2 gene. In the pathogenesis of schwannomatosis, the most probable joint roles have INI1/SMACB1, NF2 gene, and other undetected genes. The disease begins to manifest itself clinically in young adulthood.
Psychiatric assessment of nocturnal enuresis in children
Authors: Grgić M. , Soldo-Butković S.
Published: November 2024

In ICD-10, nocturnal enuresis is defined as the repeated involuntary voiding of urine in bed after the age of 5 (or mental age of 4). The following subgroups of enuresis have been described: nocturnal, daily, and their combination. There are two types of enuresis: primary and secondary. Enuresis is defined as primary if the child has never had a 6-month period in which he or she was dry at night or secondary if the child begins wetting after a 6-month period of dryness. Recent studies indicate that nocturnal enuresis is best regarded as a group of conditions with different etiologies. The heterogeneity of primary nocturnal enuresis highlights the need for a differentiated approach to treatment, tailored to the suspected etiology in the individual child. This article reviews the psychiatric aspects of nocturnal enuresis: psychiatric classification, psychosocial factors, psychiatric comorbidity and psychiatric treatment.
Vancomycin-resistant Enterococcus faecium infections on pediatric wards
Authors: Bayraktar B. , Bar1ş A. Bayr1 , Bulut E. , Toksoy B. , Palanduz A. , Aktaş Z. , Kayacan Ç. Bal , Aslan T.
Published: November 2024

Nosocomial infections with vancomycin-resistant enterococci (VRE) have been reported with increasing frequency in recent years, and are associated with an increase in mortality. Isolates of VRE were first detected in our hospital between September 2006 and January 2007. In this study, we analyzed the phenotypic and genotypic characteristics underlying that resistance and clonal relationships amongst VRE isolated from six patients at pediatric wards. All isolates were identified as Enterococcus faecium and showed resistance to both vancomycin and teicoplanin by disk diffusion and E-test methods. The resistance patterns for other antimicrobials were similar, indicating that the isolates might belong to the same clone. The genes responsible for resistance were identified in the isolates by multiplex polymerase chain reaction (PCR). Four isolates from patients on the same pediatric ward showed the same random amplification of polymorphic DNA (RAPD) pattern, suggesting clonal dissemination of a single strain.
Does mental retardation affect masticatory efficiency in children with cerebral palsy?
Authors: Bakarčić D. , Lajnert V. , Cicvarić I. Vlašić , Jokić N. Ivančić , Hrvatin S. , Gržić R. , Prpić I.
Published: November 2024

The aim of the study was to assess the influence of cognitive abilities on the masticatory system functions in children with cerebral palsy. Forty-three children suffering from cerebral palsy and having different levels of cognitive impairment (aged 6-16 years) were evaluated with regard to their masticatory efficiency using a sieving test. Cognitive abilities were taken from medical documentation of the patients at University Department of Pediatrics, Rijeka University Hospital Center, Rijeka, Croatia. Study subjects were divided into two groups: children with severe and profound mental retardation (group 1) and children with no mental retardation or with mild mental retardation (group 2). There were no subjects with moderate mental retardation. Study results showed that masticatory efficiency values were lower in group 1 including children with severe and profound mental retardation as compared with group 2 that included children with no mental retardation or with mild mental retardation. Comparison of mastication efficiency index in children with cerebral palsy according to the grade of mental retardation yielded a statistically significant difference between the two groups (Z=92.50; p<0.001). It is concluded that the grade of mental retardation plays a major role in the level of masticatory efficiency.
Early detection of children at neurorisk and enrolment in habilitation programs
Authors: Bošnjak-Nađ K. , Mejaški-Bošnjak V. , Popović-Miočinović Lj. , Ahmetašević S. Gverić , Đaković I. , Mladin M. Čikara
Published: November 2024

Perinatal brain damage is the leading cause of poor neurodevelopmental outcome in children. About 10% of newborns classified as high-risk neonates are especially susceptible to brain injury. Perinatal brain damage is non-progressive, thus processes of maturation and brain plasticity accompanied by early medical intervention can lead to recovery of neuromotor function. Thus, early recognition, monitoring and habilitation are highly important in children at high neurorisk. The aim of this study was to present the project of detection and follow up of children at neurorisk and to show results of one-year follow up of children at high neurorisk as part of the pilot project of the Ministry of Health and Social Welfare named Follow Up of Children at Neurorisk. In comparison to the older Registry, study results pointed to the need of updating the survey questionnaire and program of follow up that were now implemented in the pilot project. Based on history data, clinical evaluation and early brain ultrasonography (US) findings, neonatologists classified children as low and high neurorisk at maternity ward, later sending completed questionnaire on neurorisk factors for each child by e-mail to regional center (children at high neurorisk) or county center (children at low neurorisk). Neonatologists also provided all necessary information on neurorisk factors, the need of early habilitation and date of first neuropediatric examination to the parents of children enrolled in the study. The extent of diagnostic procedures and clinical examinations depended on the risk category attributed to children. Children with deviant neurologic development were promptly included in habilitation procedures. The pilot study was performed from January 1, 2007 until August 31, 2008 and included data on 170 children at high neurorisk born at University Department of Gynaecology and Obstetrics, Zagreb University Hospital Center (Petrova). Neurodevelopmental outcome at the age of 12 months was normal in 107 and delayed in 47 children, whereas 16 children had some form of neurologic syndrome predictive for development of cerebral palsy; 75% of children with complex neuromotor disorder had pathological brain US findings; 38 children had visual disorders, and three children were blind; 14 children had abnormal electroencephalography (EEG) findings, two children suffered from West’s syndrome, and another two had febrile seizures. Data analysis of risk factors and neurodevelopmental outcome showed concordance with new achievements in the fields of perinatology and habilitation. Accordingly, early recognition, monitoring and habilitation are of utmost importance in children at high neurorisk.
Translational research in the diagnosis and therapy of hereditary muscular diseases and national registry of neuromuscular diseases
Authors: Barišić N. , Lehman I. , Grđan P.
Published: November 2024

Translational research enables transfer (translation) of basic laboratory, preclinical research to clinical practice, whereas results of clinical practice induce and direct further basic research. In the treatment of hereditary muscle diseases, pharmacological agents are used, while gene and stem cell therapies are still in the phase of clinical or preclinical trials. Standardization of models for testing therapeutic approaches, definition of certain diseases and definition of criteria for assessing therapeutic protocols and treatment efficiency represent a rational basis for clinical application of different therapeutic strategies. Completion of the European projects Translational Research in Europe – Assessment and Treatment of Neuromuscular Disorders (NMD-TREAT) and CARENMD has a very important role in the creation of uniform access to diagnostic and therapeutic procedures for patients with neuromuscular diseases. National registries have an important role in assessing the frequency, screening of patients, and planning of clinical trials of different therapeutic approaches including gene therapy, thereby contributing to improved quality of life and care of patients with neuromuscular diseases.
Friedreich’s ataxia
Authors: Hribljan M. Čačić , Jurin M.
Published: November 2024

Friedreich’s ataxia (FRDA) is the most common autosomal recessive ataxia. This neurodegenerative disease is caused by expansion of a GAA triplet repeat located within the first intron of the frataxin gene on chormosome 9q13. There is clear correlation between the size of expanded repeat and severity of the phenotype. Clinically, FRDA is characterized by early-onset progressive gait and limb ataxia, dysarthria, loss of vibration and proprioceptive sense, areflexia, abnormal eye movements and extensor plantar response. Cardiomyopathy, diabetes, scoliosis and pes cavus are other common systemic complications. Frataxin is a mitochondrial protein that plays a role in iron homeostasis and deficiency of frataxin results in mitochondrial iron accumulation, defects in specific mitochondrial enzymes and oxidative damage. Therefore, FRDA treatment options have been mostly directed at antioxidant protection against mitochondrial damage. Available evidence seem to suggest that patients with FRDA should be treated with idebenone because it may reduce cardiac hypertrophy and at higher doses also improves neurologic function, although large controlled clinical trials are still needed. Alternatively, gene-based strategies for the treatment of FRDA may involve development of small molecules increasing frataxin gene transcription.
Long-term follow-up and outcome of children with febrile seizures
Authors: Šojat Lj. Cvitanović , Malenica M. , Juraški R. Gjergja , Sabol Z. , Kužnik K. , Šojat T.
Published: November 2024

Febrile seizures (FS) precede the onset of various forms of epilepsy in 10%-15% of children. The risk of epilepsy in children with FS is 3% by the age of 7 years. Complex FS are associated with younger age at onset of epilepsy. One-third of patients with temporal lobe epilepsy have a previous history of prolonged febrile seizures. Retrospective data analysis of patients with FS treated at our Neuropediatric Unit and Outpatient Clinic in the last 20 years revealed 880 patients with FS: simple 81.1% and recurrent /complex 18.9%. Thirty-three children had subsequent non-febrile seizures. In 23 children, epilepsy occurred 1-5 years following FS: idiopathic generalized in five, partial idiopathic or cryptogenic extratemporal in seven, temporal in two, and symptomatic partial in three children. After FS, two children had epileptic encephalopathy, two had syndromes with molecular abnormalities, and one child had Fahr disease. One girl who presented for the first time with febrile status epilepticus had Sturge-Weber syndrome. Out of 23 children with epilepsy, 13 had previously had one FS episode and ten recurrent FS. Seven children had good control of epilepsy, while six children had normal intellectual development. Reviewing our 880 patients with FS revealed that 2.6% of them developed epilepsy and two of them had refractory idiopathic temporal lobe epilepsy. There was no case of hippocampal sclerosis in our children with FS and subsequent epilepsy.
Determinants of neonatal and functional status and physiotherapy intervention in children with cerebral palsy
Authors: Milaščević D. , Klaić I. , Petrak O. , Župetić K.
Published: November 2024

Assessment of neonatal and functional status for planning physiotherapy intervention in children with cerebral palsy is the main topic of this paper. „Surveillance of Cerebral Palsy in Europe“ (SCPE) classification system and Gross Motors Function Classification System (GMFCS) are the key determinants in planning physiotherapy intervention. Therefore, the aim of the study was to determine the indicators of general neonatal status, functional status, and physiotherapy intervention in children with different types of cerebral palsy, and to identify the possible correlations of these variables. The study included 98 children (56 male and 42 female) with cerebral palsy, all users of physiotherapy at Special Hospital for Children with Developmental Disabilities. Study results confirmed the sensitivity of GMFCS and its rather good agreement with SCPE classification. Significant correlation of functional status with complexity and intensity of therapy intervention is also evident from the results. This study confirmed good organization and dosing of therapy programs for children with different levels of functioning within the GMFCS at Special Hospital for Children with Developmental Disabilities in Zagreb.
Jeavons syndrome
Authors: Lujić L. , Đuranović V. , Fureš J. Sekelj , Krakar G. , Đaković I.
Published: November 2024

Eyelid myoclonia with absences or Jeavons syndrome is clinically characterized by rapid eyelid myoclonia with or without loss of consciousness. Seizures are provoked after eye closure with generalized discharges, mainly polyspikes in EEG and photosensitivity. Patients may occasionally have generalized tonic-clonic seizures. It is a lifelong disorder that begins in childhood with a striking female predominance. We report on three children (two female and one male) with eyelid myoclonia, observed at the age of 6 years in the boy and one girl, and at the age of 14 years in the older girl. In the youngest patient (the boy), the diagnosis was made after 2 months, in young girl after 2.5 years, and in older girl after 1.5 year of eyelid myoclonia persistence. The boy was referred to the hospital because of pathologic EEG and a „tic“ that manifested as eyelid myoclonia and occasional eyeball deviation, the young girl because of recurrent syncope and generalized epileptic changes in EEG, and the older girl because of syncope. Eyelid myoclonia after closure of the eyes followed by generalized epileptic discharges typical for Jeavons syndrome was confirmed by video EEG. All patients had normal neurologic status and intellectual development. The disease was stabilized by valproic acid and ethosuximide. In conclusion, proper knowledge of Jeavons syndrome is necessary to avoid inaccurate diagnosis of tics or other idiopathic generalized epileptic syndrome with absence type seizures.
Children with special health care needs in a paediatric emergency service
Authors: Meštrović J. , Sršen S. , Tudor K. Malić
Published: November 2024

Children with special health care needs fall ill more often, they are more likely to be treated in hospital, and account for half of all children who die in hospital. In our study 10.6% of children treated in the paediatric emergency service were children with special health needs. Most of them (72.9%) were examined due to complications of their basic illness. Children with special health care needs were admitted to hospital more often than others. Hospital treatment of children with special health care needs and technology dependent children require paediatric and other hospital services to be prepared for their admittance and treatment.
Caloric and nutritive value of kindergarten meals in Zagreb after the implementation of the new National nutritive recommendations and standards 2007.
Authors: Jagić V. , Bošnir J. , Racz A. , Jelušić S.
Published: November 2024

The aim of this study was to evaluate diet quality in 15 kindergartens (9 public and 6 private) in the City of Zagreb after the implementation of new National nutritive recommendations and standards for preschool children in kindergartens in December 2007. Standardized analytical methods were used to analyse in total 60 whole-day meals, sampled by a method of duplicate meals, four times per year during 2008. The results of energy and nutritive values of whole-day meals were compared to the recommendations of the new Nutrition Standards calculated using 75% daily values, that correspond to an average of 8-10 hours preschool child stay per day. The total mean energy value of whole-day meals was 1247.95 kJ (5223.86 kJ) or 103.99% of the recommended standard. The mean fat value was 101% of the recommended standard or 43.20 g/day. The mean amount of carbohydrates was 165.39 g/day, which was 102% of the recommended standard. An excessive mean protein value in whole-day meals (> +30% amounted of the recommended standard) was measured in both public and private kindergartens. No significant difference between public and private kindergartens was identified regarding any of the evaluated parameters. Preschool children’s diet and adaptation of healthy eating habits is a public health concern, so it is important to reduce their protein intake significantly in whole-day meals for medical, ecological and ethical reasons.
Anxiety and depression in paediatric patients with painful somatic complaints
Authors: Baraban D. , Kurtović A. , Zelić V. , Križanić V. , Buljubašić M.
Published: November 2024

The aim of this study was to examine the level and different aspects of anxiety and depression in children and youth with recurrent painful somatic complaints and (1) compare them to the norm values of a general population sample, (2) compare the patients with single complaints to patients with multiform somatic complaints and (3) to examine the proportion of clinically significant anxiety and depressive patients. The Fear and Anxiety Scale SKAD-62 and The Depression Scale SDD were applied to 160 children, ages 10 to 17, who were referred by paediatric specialists for psychology assessment under hospital or ambulatory treatment: 56 with headaches, 24 stomach pain sufferers, 40 chest pain sufferer and 39 children with multiple somatic complaints. The results showed that all patients had similar global levels of anxiety and depression as compared to the norm values. Clinically significant anxiety was observed in 22% patients and clinically significant depression in 4% patients. Most anxiety in patients was found in the chest pain group (35%) and less in those with headache (8.6%). Children with multiform complaints are as anxious as single complaint sufferers. A difference was found between clinical samples in different aspects of anxiety symptoms: those with headache were separation anxious, abdominal and chest pain sufferers were more anxiety sensitive, multiform complainers were more worried.
Nasogastric tube feeding in preterm infants
Authors: Kolundžić Z. , Klarić A. Šimić , Drkulec V. , Rajić M. Tomić , Tesari H.
Published: November 2024

Breastfeeding, which is enabled by the sucking reflex appearing at about 32 gestational weeks, provides all the relevant nutritive substances and protects the child during the neonatal period. Breastfeeding is the process of drinking fluid into the mouth by sucking power produced by moving the jowl, lips and tongue. Swallowing is the function of dislocating food and fluid through the mouth to the oesophagus and the stomach with a certain rhythm and speed. When the sucking reflex is absent in prematures, it is important to provide enteral nutrition, mostly by a nasogastric tube. Although it is impossible to avoid nasogastric tube feeding, it is necessary to apply all therapeutic possibilities to shorten the duration of tube use and to prevent the negative effects of this feeding procedure. Programs of tactile stimulation of the perioral structures and non-feeding sucking have a good effect on learning oral feeding, shorter tube feeding time and shorter hospitalization.
Less frequent causes of acute surgical pain in children - our experience in the past 10 years
Authors: Žganjer M. , Cigit I. , Žganjer V. , Car A. , Leko M.
Published: November 2024

Acute pain in children can occur for different reasons. The term acute abdomen is considered to be a set of symptoms that occur with a sudden illness of the body’s organs. According to the International Association for the Study of Pain, pain is defined as “an unpleasant sensory and emotional experience associated with actual or potential tissue damage, or described in terms of such damage.” Pain intensity can vary, starting from light, then severe, and finally agonizing and can occur as permanent or nonpermanent. It may be experienced as sharp, throbbing, agonizing, burning, stabbing or as a combination of these. We wanted to show a few patients with different problems and different clinical pictures that are not common in daily routine treatment. All patients had acute or chronic pain, and all were initially treated with medication. All of them required surgical treatment. When we have patients with abdominal pain we must always consider all the possibilities that could be causing the problems. There is often a diagnostic problem which is the cause of acute abdomen.
Attention deficit disorder/hyperkinetic disorder in children and adolescents
Authors: Juretić Z. , Petković Z. Bujas , Ercegović N.
Published: November 2024

Attention deficit/hyperactivity disorder (ADHD) is a neurobiological disorder characterized with three core symptoms: hyperkinesis, attention deficit and impulsivity and secondary symptoms like learning disorders, behavioural disturbances and low self esteem. Soft neurological signs are very often present. The aetiology of the disorder is not yet well known and there are probably more neurobiological and psychosocial aetiological factors. The prevalence is 3-10% of school-age children. There is a greater incidence in boys than in girls with the ratio 9:1 in clinical, and 4:1 in epidemiological samples. Comorbidity is a major problem among children with ADHD, and two thirds of them have at least one more diagnosed psychiatric disorder, most often a conduct disorder, oppositional defiant disorder and learning disorder, but also disorders of speech and communication, anxiety disorders, mood disorders, tic disorders such as Sy Tourette s. Comorbidity is very important because it makes the diagnostic process more complicated, and has implications for the course, prognosis and treatment. Treatment of ADHD is multidimensional and combines psychosocial and pharmacological interventions, and it should start as early as possible. Today, cognitive behavioural treatment and drug treatment are most important in therapy.
Urine neutrophil gelatinase associated lipocalin (uNGAL) as predictor of renal scarring
Authors: Yilmaz A. , Gedikbasi A. , Sevketoglu E. , Karyagar S. , Kiyak A. , Mulazimoglu M. , Aydogan G. , Hatipoglu S. , Ozpacaci T.
Published: November 2024

Urine Neutrophil gelatinase associated lipocalin (uNGAL) is a novel protein expressed in injured epithelia. The aim of our study was to assess whether uNGAL could represent a novel biomarker of renal scarring and to determine the optimal cut-off level for uNGAL to predict the presence of renal scars in children. Forty-four patients with renal scarring and 44 patients without renal scarring on dimercaptosuccinic acid scan were enrolled in the study. Serum urea and creatinine levels were normal in all children in the study. Urine NGAL was measured by ELISA. The mean uNGAL level was significantly higher in children with scars than in those without scars (14.92 ng/mL vs 5.71 ng/mL, p=0.001). According to receiver operating curve (ROC) analysis, the optimal cut-off level was 4ng/mL for uNGAL to predict renal scars. Using a cut-off 4ng/mL for uNGAL for prediction of scars, sensitivity and specificity were 68% and 68%, respectively. The positive and negative predictive values of this cut-off point were 68% and 68%, respectively. The area under the curve (AUC) was found to be 0.69 for uNGAL. In conclusion, the uNGAL level was higher in children with renal scars than in without scars. However, uNGAL has low sensitivity and specificity as a marker for prediction of renal scarring.
Dental erosion and associated risk factors among preschool children
Authors: Čuković-Bagić I. , Dumančić J. , Kujundžić-Tiljak M. , Škrinjarić T. , Salopek-Prpić H.
Published: November 2024

Dental erosion is the irreversible loss of dental hard tissue due to acid action. The aim of this study was to investigate the prevalence and severity of dental erosion in the primary dentition of preschool children, and to determine the relationship between dental erosion and dietary and oral hygiene habits as well as parental educational level. The subjects were 199 healthy children (aged 4-5 years) from two Zagreb kindergartens. Dental erosion of primary teeth was evaluated by a modified Smith and Knight Tooth Wear Index (1). Information on dietary and oral hygiene habits and parental educational level were obtained through a questionnaire. The results showed that 78% children had evidence of dental erosion. In 51.0% of children it was confined to the enamel, in 26.8% erosion extended into the dentine and in 1.0% it reached pulp. There was no significant difference between the sexes (p> 0.05). The questionnaire data indicated an association between dental erosion and consumption of sweet drinks, fruit, snacks and biscuits. There was no association with oral hygiene habits. With the higher educational level of the parents, the prevalence of erosion in children was lower. In conclusion, the prevalence of dental erosion in primary dentition is high, so the risk factors should be eliminated by proper diet and oral hygiene instructions, beginning from early childhood.
Conservative management of idiopathic chylothorax and chylopericardium in a preterm neonate
Authors: Polić B. , Meštrović J. , Markić J. , Stričević L. , Jurić I. , Radonić M.
Published: November 2024

Chylothorax is a rare condition characterized by accumulation of chyle within the pleural cavity. Idiopathic chylopericardium is extremely rare. It is characterized by chylous effusion in the pericardial cavity and clinical signs of cardiac tamponade. Conservative treatment of chylothorax is usually satisfactory, while treatment of chylopericardium needs to be surgical in the vast majority of patients. We present a unique case of idiopathic chylothorax and chylopericardium in a preterm neonate, successfully treated conservatively.
VEGH and HIF-1α expression in nephroblastoma correlates with anaplasia
Authors: Pajić A. , Džombeta T. , Cigrovski N. , Radulović P. , Čizmić A. , Stepan J. , Krušlin B.
Published: November 2024

HIF-1α (hypoxia inducible factor 1α) is a transcription factor that has a role in maintaining oxygen homeostasis. Increased expression of HIF-1α leads to a rise in amount of VEGF (vascular endothelial growth factor), thus inducing neovascularization. The aim of this study was to determine the expression of VEGF and HIF-1α in nephroblastoma and to compare it with the clinical and pathohistological characteristics of this tumour (dominant histological component, presence of anaplasia and necrosis, size of the tumour, age and sex of the patient). Expression of VEGF and HIF-1α was determined by immunohistochemistry of the tumour tissue. Monoclonal mouse anti-VEGF and polyclonal rabbit anti-HIF-1α antibodies were used. For immunoscoring of both markers the degree of positive staining was evaluated semiquantitatively. The χ²-test, which includes Spearman’s rank correlation and the ANOVA test were used for statistical analysis of data. Most of the tumours were positive for VEGF (38/40) and HIF-1α (37/40). Significant statistical correlation was found between HIF-1α and the presence of anaplasia (p=0.02986). A clear correlation between VEGF and tumour size was established (Spearman’s rank correlation: p=0.0027), showing strong positivity in larger tumours. Based on this study we can conclude that VEGF and HIF-1α are present in Wilms’ tumours, playing an important role in angiogenesis and tumour progression.
Exercise-related syncope in the young athletes
Authors: Herceg-Čavrak V. , Cvetko† Ž. , Batinica M.
Published: November 2024

Syncope in the young is a frequent occurrence, which is usually harmless. Syncope on exertion can be a sign of a life-threatening situation or even sudden death, hence diagnostic work-up is always necessary. Syncope during exertion is more serious than syncope which occurs immediately after exertion. Whenever it is associated with exertion, it is most frequently neurocardiogenic, but other forms, such as cardiac, nervous/reflex or metabolic, are not excluded. There is still no specific test with which the type of syncope can be determined with certainty, hence it is necessary to obtain a precise history and carry out a clinical examination, laboratory investigations and electrocardiogram (ECG). If cardiac syncope is suspected, additional diagnostic tests such as echocardiogram and stress test are necessary. With the collected data, decisions are made on further management and a judgement is made as to whether the child can continue to be involved in sport.
Gastroparesis in childhood: fiction or daily problem?
Authors: Palčevski G. , Perčić M.
Published: November 2024

Gastroparesis, or delayed gastric emptying without obstruction, is a chronic disorder caused by stomach pump failure. The prevalence and etiology of gastroparesis in children has not been extensively examined, though postviral gastroparesis (rotavirus) is thought to be the most com mon identifiable cause in children. Typical symptoms of gastroparesis are abdominal fullness or bloating, early satiety, epigastric pain and distress, heartburn, anorexia, nausea, vomiting and weight loss. Several methods have been proposed for quantification of gastric emptying: abdominal ultrasonography, radiographic contrast techniques, gastric emptying scintigraphy, electrogastrography and breath testing for gastroparesis. Initial therapy should be guided by underlying diseases, if existent. Treatment is based on psychological and dietary support and pharmacotherapy. Pharmacotherapy includes prokinetic and antiemetic agents. From a paediatric perspective, prokinetic erythromycin is more efficient, has mild and transient side effects after long-term, low-dose administration. Gastric electrical stimulation seems to be the most promising therapeutic option available at present.
Mowat-Wilson syndrome
Authors: Malčić I. , Vidaković M. , Kniewald H. , Dilber D. , Vuković J. , Dasović-Buljević A.
Published: November 2024

We report on two children with phenotypic characteristics of Mowat-Wilson syndrome associated with complex cardiac malformations. In molecular genetic analysis one of our patients had the expected mutation on the 2q22 chromosome. The purpose of this report is to point out the presence of a serious syndrome in paediatric cardiology, which certainly affects the final outcome of the disease. Diagnosis of Mowat-Wilson syndrome cannot be set without a detailed evaluation of the dysmorphic features and molecular genetic analysis. This approach demands more creativity and engagement of the paediatric cardiologist.