GUILLAIN-BARRÉOV SINDROM I ATIPIČNE VARIJANTE U DJECE: ISKUSTVO TERCIJARNOG CENTRA U REPUBLICI HRVATSKOJ

Aim: To depict heterogeneous clinical features of atypical Guillain-Barré syndrome (GBS) variants overlapping between different GBS types and subtypes. Methods: Retrospective analysis of data comprising neurological features, cerebrospinal fl uid (CSF) analysis, ganglioside antibody testing results, electromyography (EMG) fi ndings, brain and spinal magnetic resonance imaging (MRI) in all pediatric patients with GBS treated during a 10-year period at a tertiary center. Results: Twenty-three children were treated for GBS during the study period. Atypical variants were found in fi ve patients and included bifacial and severe pharyngocervicobrachial weakness of descending type, sixth nerve lesion accompanied with lower extremity paresthesias, sensory atactic neuropathy and facial nerve lesion, acute ptosis with mydriasis and incomplete Miller Fisher syndrome, and bilateral facial nerve paresis (one case each). Initial CSF analysis revealed mostly normal proteinlevel in atypical variants. MRI evaluation was normal in all atypical variants except for enhancement of the cervical nerve roots in a patient with pharyngocervicobrachial subtype. EMG performed in the fi rst two weeks showed prolonged distal latency and proximal conduction block in 3/5 patients, in elicitable nerves and axonal loss on upper extremities in a patient with pharyngocervicobrachial subtype, and absent F-waves and neural potentials in 3/5 patients. Slight decrease of motor conduction velocity was present in 2/5 patients in distal nerve segments. Antiganglioside antibodies were positive in 4/5 patients. Conclusion: Clinical manifestations of GBS are very variable, whereas atypical variants/overlaps are not so uncommon. This study supports the proposed hypothesis of continuous spectrum of GBS requiring reconsideration of the existing diagnostic criteria for classic GBS in pediatric population supported by recently proposed (published) diagnostic guidelines.Cilj: Prikazati heterogene kliničke značajke inačica atipičnog Guillain-Barréova sindroma (GBS) te preklapanje između različitih GBS tipova i podtipova. Metode: Retrospektivna analiza podataka koji uključuju neurološke značajke, analizu cerebrospinalne tekućine, rezultate ispitivanja antigangliozidnih protutijela, nalaze elektromiografi je (EMG), magnetsku rezonanciju mozga i kralježnice (MRI), svih pedijatrijskih bolesnika liječenih zbog GBS-a u tercijarnom centru u 10-godišnjem razdoblju. Rezultati: Liječeno je ukupno 23 djece zbog GBS-a. Atipične varijante pronađene su u pet bolesnika i uključivale su bifacijalnu i tešku faringo-cerviko-brahijalnu silaznu slabost, leziju šestog živca popraćenu parestezijama donjih ekstremiteta, senzoričku ataktičnu neuropatiju i leziju facijalnog živca, akutnu ptozu s midrijazom i nepotpunim Miller Fisherovim sindromom te bilateralnu parezu facijalnog živca (sve po jedan slučaj). Inicijalna analiza cerebrospinalne tekućine pokazala je većinom normalnu razinu proteina. Radiološka obrada je bila uredna u svim atipičnim varijantama osim u bolesnika s faringo-cerviko-brahijalnom varijantom gdje je nađen pojačan signal u korijenu živaca cervikalne kralježnice. EMG je prva dva tjedna bolesti pokazao produljenu distalnu latenciju i proksimalni blok u 3/5 bolesnika, gubitak aksona na gornjim ekstremitetima u bolesnika s faringo-cerviko-brahijalnim podtipom te odsutne F-valove i neuronske potencijale u 3/5 bolesnika. Blago smanjenje brzine provođenja u distalnim segmentima živaca bilo je prisutno u 2/5 bolesnika. Antigangliozidna protutijela bila su pozitivna u 4/5 bolesnika. Zaključak: Kliničke manifestacije GBS-a vrlo su varijabilne, a atipične varijante/preklapanja nisu rijetke. Ovo istraživanje podupire predloženu hipotezu o kontinuiranom spektru GBS-a koja zahtijeva preispitivanje postojećih dijagnostičkih kriterija za klasični GBS u dječjoj populaciji te uključenje kriterija za varijante GBS-a koji su nedavno predloženi i objavljeni

GUILLAIN-BARRÉOV SINDROM I ATIPIČNE VARIJANTE U DJECE: ISKUSTVO TERCIJARNOG CENTRA U REPUBLICI HRVATSKOJ

Aim: To depict heterogeneous clinical features of atypical Guillain-Barré syndrome (GBS) variants overlapping between different GBS types and subtypes. Methods: Retrospective analysis of data comprising neurological features, cerebrospinal fl uid (CSF) analysis, ganglioside antibody testing results, electromyography (EMG) fi ndings, brain and spinal magnetic resonance imaging (MRI) in all pediatric patients with GBS treated during a 10-year period at a tertiary center. Results: Twenty-three children were treated for GBS during the study period. Atypical variants were found in fi ve patients and included bifacial and severe pharyngocervicobrachial weakness of descending type, sixth nerve lesion accompanied with lower extremity paresthesias, sensory atactic neuropathy and facial nerve lesion, acute ptosis with mydriasis and incomplete Miller Fisher syndrome, and bilateral facial nerve paresis (one case each). Initial CSF analysis revealed mostly normal proteinlevel in atypical variants. MRI evaluation was normal in all atypical variants except for enhancement of the cervical nerve roots in a patient with pharyngocervicobrachial subtype. EMG performed in the fi rst two weeks showed prolonged distal latency and proximal conduction block in 3/5 patients, in elicitable nerves and axonal loss on upper extremities in a patient with pharyngocervicobrachial subtype, and absent F-waves and neural potentials in 3/5 patients. Slight decrease of motor conduction velocity was present in 2/5 patients in distal nerve segments. Antiganglioside antibodies were positive in 4/5 patients. Conclusion: Clinical manifestations of GBS are very variable, whereas atypical variants/overlaps are not so uncommon. This study supports the proposed hypothesis of continuous spectrum of GBS requiring reconsideration of the existing diagnostic criteria for classic GBS in pediatric population supported by recently proposed (published) diagnostic guidelines.Cilj: Prikazati heterogene kliničke značajke inačica atipičnog Guillain-Barréova sindroma (GBS) te preklapanje između različitih GBS tipova i podtipova. Metode: Retrospektivna analiza podataka koji uključuju neurološke značajke, analizu cerebrospinalne tekućine, rezultate ispitivanja antigangliozidnih protutijela, nalaze elektromiografi je (EMG), magnetsku rezonanciju mozga i kralježnice (MRI), svih pedijatrijskih bolesnika liječenih zbog GBS-a u tercijarnom centru u 10-godišnjem razdoblju. Rezultati: Liječeno je ukupno 23 djece zbog GBS-a. Atipične varijante pronađene su u pet bolesnika i uključivale su bifacijalnu i tešku faringo-cerviko-brahijalnu silaznu slabost, leziju šestog živca popraćenu parestezijama donjih ekstremiteta, senzoričku ataktičnu neuropatiju i leziju facijalnog živca, akutnu ptozu s midrijazom i nepotpunim Miller Fisherovim sindromom te bilateralnu parezu facijalnog živca (sve po jedan slučaj). Inicijalna analiza cerebrospinalne tekućine pokazala je većinom normalnu razinu proteina. Radiološka obrada je bila uredna u svim atipičnim varijantama osim u bolesnika s faringo-cerviko-brahijalnom varijantom gdje je nađen pojačan signal u korijenu živaca cervikalne kralježnice. EMG je prva dva tjedna bolesti pokazao produljenu distalnu latenciju i proksimalni blok u 3/5 bolesnika, gubitak aksona na gornjim ekstremitetima u bolesnika s faringo-cerviko-brahijalnim podtipom te odsutne F-valove i neuronske potencijale u 3/5 bolesnika. Blago smanjenje brzine provođenja u distalnim segmentima živaca bilo je prisutno u 2/5 bolesnika. Antigangliozidna protutijela bila su pozitivna u 4/5 bolesnika. Zaključak: Kliničke manifestacije GBS-a vrlo su varijabilne, a atipične varijante/preklapanja nisu rijetke. Ovo istraživanje podupire predloženu hipotezu o kontinuiranom spektru GBS-a koja zahtijeva preispitivanje postojećih dijagnostičkih kriterija za klasični GBS u dječjoj populaciji te uključenje kriterija za varijante GBS-a koji su nedavno predloženi i objavljeni

Još jedno lice infekcije Epstein-Barrovim virusom: akutni akalkulozni kolecistitis s kolestazom u prethodno zdrave petogodišnje djevojčice s primarnom infekcijom

Acute acalculous cholecystitis (AAC) is a rare disease, particularly in children. The clinical and laboratory presentation of AAC is variable. The diagnosis is based on radiological criteria. AAC is an atypical and rare complication of Epstein-Barr virus (EBV) infection, therefore we present a girl with AAC and cholestasis due to EBV primary infection. Conservative treatment and careful monitoring was followed by clinical, radiological and laboratory improvement, and finally complete recovery. Our aim was to highlight the importance of recognizing AAC as a differential diagnosis in children with abdominal pain and/or acute cholestasis.Akutni akalkulozni kolecistitis (AAK) je rijetka bolest, osobito u djece. Klinička i laboratorijska slika AAK-a je raznolika, a dijagnoza se temelji na radiološkim kriterijima. AAK je atipična i rijetka komplikacija infekcije Epstein-Barrovim virusom (EBV). Prikazujemo djevojčicu s AAK i kolestazom posljedično primarnoj infekciji EBV-om. Konzervativno liječenje praćeno je kliničkim, radiološkim i laboratorijskim poboljšanjem te na koncu potpunim oporavkom djevojčice. Cilj ovoga prikaza je naglasiti važnost prepoznavanja AAK-a kao diferencijalne dijagnoze u djece s bolovima u trbuhu i/ili akutnom kolestazom

The role of national registry in the care of children suffering from chronic infl ammatory bowel disease

Kroničnim upalnim bolestima crijeva u djece, za razliku od odraslih, svojstven je teži tijek i prošireniji oblik bolesti te negativan utjecaj na rast i razvoj. U posljednjih nekoliko desetljeća zamijećen je trend porasta incidencije kroničnih upalnih bolesti crijeva, najviše na račun Crohnove bolesti. Uzimajući u obzir sve navedeno, ali i činjenicu da se upalne bolesti crijeva u djece pojavljuju u razdoblju njihovog ubrzanog rasta i razvoja, svi pedijatrijski bolesnici iziskuju dugoročno praćenje koje je moguće jedino putem nacionalnog registra. U ovom preglednom članku raspravljamo o ulogama, ciljevima i metodama rada nacionalnih registara, koji imaju za cilj praćenje različitih varijabli u djece s kroničnim upalnim bolestima crijeva, ali naglašavamo i važnost osnivanja takvog registra u Republici Hrvatskoj.Unlike adults, infl ammatory bowel disease in children is marked by more extensive and severe course, aff ecting their future growth and development. In general, there is an increasing trend in the incidence of chronic infl ammatory bowel disease, mostly on the account of Crohn’s disease. Taking all these into consideration, but also the fact that infl ammatory bowel disease in children occurs in a period of rapid growth and development, all pediatric patients require long-term monitoring, which is only possible through national registry. In this review, we discuss the role of national registries, their aims and methods to achieve established goals, with special reference to the importance of establishing such a registry in Croatia

THE ROLE OF TRANSITION CLINIC IN THE HEALTH CARE OF ADOLESCENTS WITH CHRONIC INFLAMMATORY BOWEL DISEASE

Upalne bolesti crijeva jesu kronične bolesti probavnog sustava koje se u trećine bolesnika klinički očituju u prvih 18 godina života. Stjecanjem punoljetnosti pedijatrijski bolesnici prelaze u zdravstvenu skrb odraslih, koja od njih zahti­jeva samostalnost i odgovornost. Adolescenti s kroničnim bolestima često nisu spremni samostalno se brinuti za svoje zdravlje. Radi osiguranja kontinuiteta zdravstvene skrbi i premošćivanja dostupnosti specijalističko-konzilijarne skrbi u odrasloj dobi nametnula se potreba za jasno strukturiranim i organiziranim prijelazom koji se naziva tranzicijskom skrbi, gdje nadzor nad bolesnikom istodobno provode liječnici pedijatrijske i internističke struke radi osposobljavanja oboljelih adolescenata za samostalno preuzimanje kontrole, bez nadzora i pomoći roditelja. Takva je skrb organizirana u mnogim razvijenim zemljama za različite kronične bolesti, a ne samo za upalne bolesti crijeva. Namjena je ovoga preglednog članka prikazati dosadašnja iskustva u organizaciji tranzicijske skrbi u razvijenim zemljama Europe te obrazložiti na koji način i zašto se to namjerava učiniti i u Zagrebu.Inflammatory bowel disease (IBD) is a chronic disease of the digestive system that occurs in one third of cases in childhood and adolescence. In the majority of patients IBD persists and relapses more or less frequently during their whole life and therefore pediatric patients, at some point, are transferred to the adult health care where autonomy and ­responsibility are required. However, adolescents with inflammatory bowel disease are mostly insufficiently competent in respect to disease specific knowledge and self-management skills what can lead to poor disease outcome. Therefore, to ­enable adolescent patients continuing healthcare and bridging the gap between pediatric and adult services, gain independence and manage disease without parental assistance, structured and organized transition (transitional care) is required, whereby for a defined period of time health care is provided by both pediatricians and internal medicine specialists. That kind of care is carried out in many developed countries through transition clinics which have been organized for patients with a wide spectrum of chronic diseases, IBD included. The aim of this review is to investigate current organizational aspects of the transition care for adolescents with chronic diseases, namely IBD, and to present the published experience in the developed European countries. The final goal is to justify organization of the transition care for IBD patients in Zagreb and to provide methods of the implementation of this kind of care in Zagreb

Acute Bilateral Brain Infarction in Middle Cerebral Artery Territories

Naglo nastali bilateralni infarkti u područjima srednjih moždanih arterija javljaju se izrazito rijetko i često za posljedicu imaju smrtni ishod. Cilj je rada prikazati kliničku sliku bolesnice i pregled literature vezan uz naglo nastale bilateralne ishemijske moždane udare u irigacijskim područjima obiju srednjih moždanih arterija.Acute bilateral infarcts in the middle cerebral artery (lat. arteria cerebralis media - ACM) territories are very rare and can have a fatal outcome. The aim is to present the clinical picture of the patient, and the review of literature associated with bilateral stroke in middle cerebral artery territories

Long-term prognosis of patients with paediatric-onset infl ammatory bowel disease after transfer to adult care – a retrospective observational study

Cilj: Cilj je ove studije procijeniti i izvijestiti o dugoročnoj prognozi u bolesnika s kroničnim upalnim bolestima crijeva (IBD), koji su nastavili liječenje u zdravstvenoj skrbi odraslih. Metode: Pregledali smo bolesničku medicinsku dokumenaciju svih bolesnika koji su liječeni u tercijarnom pedijatrijskom medicinskom centru i koji su barem pet godina nastavili liječenje u adultnoj skrbi (medijan 12,4 godina). Rezultati: U razdoblju od 1993.-2003. u našem je centru liječeno 52-je bolesnika s IBD-om te su oni ili njihove obitelji bili dostupni za razgovor (13 žena, medijan dobi 29 godina; 20-ero oboljelih od Crohnove bolesti, sedmero od ulceroznog kolitisa (UC)). Ukupno je 23-je (85%) bolesnika nastavilo liječenje u internističkoj skrbi, dok njih četvero (15%) nikad nije bilo ni na jednom pregledu internista gastroenterologa (troje bolesnika oboljelo od UC). Ukupno je 19-ero (70%) bolesnika imalo relaps tijekom praćenja, dvoje (7,4%) bolesnika je umrlo, devetero (33%) je primilo biološku terapiju. Trećina bolesnika je kirurški liječena u odrasloj dobi. Zaključak: Ova studija potvrđuje važnost organizirane tranzicije iz pedijatrijske skrbi u zdravstvenu skrb odraslih.Aim: The aim of this study was to evaluate and report long-term prognosis of infl ammatory bowel disease (IBD) in patients transferred to adult care. Methods: We reviewed medical records of all patients treated in our tertiary medical centre and then on adult care for a minimum of 5 years (median 12.4 years). Results: During the study period, 52 IBD patients were treated in our centre and 27 patients/families were available for interview (13 of them women; median age 29 years; 20 with Crohn’s disease and 7 with ulcerative colitis (UC)). Twenty-three (85%) patients were transferred to adult care, whereas 4 (15%, 3 with UC) patients never consulted an adult gastroenterologist. Nineteen (70%) patients experienced a relapse, 2 (7.4%) died, and 9 (33%) received biological therapy in adult care. Of the surviving patients, 8 (32%) underwent surgery in adult age. Conclusion: This study confi rmed that, in the absence of organized transition to adult care, a number of patients would never transfer on their own, causing discontinuity of care

THE ROLE OF TRANSITION CLINIC IN THE HEALTH CARE OF ADOLESCENTS WITH CHRONIC INFLAMMATORY BOWEL DISEASE

Upalne bolesti crijeva jesu kronične bolesti probavnog sustava koje se u trećine bolesnika klinički očituju u prvih 18 godina života. Stjecanjem punoljetnosti pedijatrijski bolesnici prelaze u zdravstvenu skrb odraslih, koja od njih zahti­jeva samostalnost i odgovornost. Adolescenti s kroničnim bolestima često nisu spremni samostalno se brinuti za svoje zdravlje. Radi osiguranja kontinuiteta zdravstvene skrbi i premošćivanja dostupnosti specijalističko-konzilijarne skrbi u odrasloj dobi nametnula se potreba za jasno strukturiranim i organiziranim prijelazom koji se naziva tranzicijskom skrbi, gdje nadzor nad bolesnikom istodobno provode liječnici pedijatrijske i internističke struke radi osposobljavanja oboljelih adolescenata za samostalno preuzimanje kontrole, bez nadzora i pomoći roditelja. Takva je skrb organizirana u mnogim razvijenim zemljama za različite kronične bolesti, a ne samo za upalne bolesti crijeva. Namjena je ovoga preglednog članka prikazati dosadašnja iskustva u organizaciji tranzicijske skrbi u razvijenim zemljama Europe te obrazložiti na koji način i zašto se to namjerava učiniti i u Zagrebu.Inflammatory bowel disease (IBD) is a chronic disease of the digestive system that occurs in one third of cases in childhood and adolescence. In the majority of patients IBD persists and relapses more or less frequently during their whole life and therefore pediatric patients, at some point, are transferred to the adult health care where autonomy and ­responsibility are required. However, adolescents with inflammatory bowel disease are mostly insufficiently competent in respect to disease specific knowledge and self-management skills what can lead to poor disease outcome. Therefore, to ­enable adolescent patients continuing healthcare and bridging the gap between pediatric and adult services, gain independence and manage disease without parental assistance, structured and organized transition (transitional care) is required, whereby for a defined period of time health care is provided by both pediatricians and internal medicine specialists. That kind of care is carried out in many developed countries through transition clinics which have been organized for patients with a wide spectrum of chronic diseases, IBD included. The aim of this review is to investigate current organizational aspects of the transition care for adolescents with chronic diseases, namely IBD, and to present the published experience in the developed European countries. The final goal is to justify organization of the transition care for IBD patients in Zagreb and to provide methods of the implementation of this kind of care in Zagreb

Prevalence of Functional Gastrointestinal Disorders in Children and Adolescents in the Mediterranean Region of Europe

Little is known about the prevalence of functional gastrointestinal disorders (FGIDs) in children from the Mediterranean area of Europe. We aimed to assess the prevalence of FGIDs in children and adolescents in this region