Application of the procedural consolidation concept to surgical treatment of children with epidermolysis bullosa: a retrospective analysis

AIM: To assess the efficacy of the procedural consolidation concept (PCC) at reducing the number of sessions of general anesthesia necessary for treating children with epidermolysis bullosa (EB). ----- METHODS: We examined the records of children treated at Children's Hospital of Zagreb between April 1999 and December 2007. Children treated before the introduction of PCC in January 2005 (n=39) and after (n=48) were analyzed in order to determine the effect of PCC on the occurrence of complications, days of hospitalization, and number of hospitalizations. ----- RESULTS: During the study period, 53 patients underwent 220 sessions of general anesthesia for a total of 743 surgical interventions per session. Before the introduction of PCC (n=39 patients, 83 sessions), the median number of interventions per session was 2 (range 1-5), and after the introduction of PCC (n=48 patients, 137 sessions) it was 4 (range 3-7, P<0.001). After the introduction of PCC, the median number of complications per anesthesia session increased from 2 (range 0-10) to 3 (range 0-10) (P=0.027), but the median number of complications per surgical procedure decreased from 1 (range 0-10) to 0.6 (range 0-2.5) (P<0.001). PCC lengthened each anesthesia session from a median of 65 minutes (range 35-655) to 95 minutes (range 50-405), (P<0.001). Total length of hospitalization was similar before (median 1, range 1-4) and after (median 1, range 1-3) introduction of PCC (P=0.169). The number of hospitalization days per procedure was 3 times lower after the introduction of PCC (median 0.3, range 0.2-3) than before (median 1, range 0.75-1.7) (P<0.001). ----- CONCLUSION: PCC should be considered an option in the surgical treatment of children with EB

THE PARADIGM OF A SECOND OPINION IN THE FIELD OF PAEDIATRIC MEDICINE

Možda će roditelji bolesnog djeteta prije započetog liječenja izraziti želju da drugi liječnik pregleda postavljenu dijagnozu i plan liječenja. Drugo mišljenje omogućava roditeljima da budu sigurniji u točnost postavljene dijagnoze, ali ono ujedno olakšava razmatranje i donošenje odluke o najboljoj opciji tretmana. Neki su liječnici “konzervativniji”, neki “agresivniji”. Kad se od roditelja očekuje da odluče o tretmanu svoga djeteta, kliničar treba razjasniti nesigurnosti i evenutalne dvojbe. Prije ili tijekom liječenja roditelje je potrebno ohrabriti i podržati njihovu želju za dobivanjem drugog mišljenja - bez obzira na liječnički stav o nužnosti takvog postupanja. Načini dobivanja drugog stručnog mišljenja su različiti. Djelokrug tog područja nije u potpunosti rasvijetljen. Također nije jasno jamči li taj postupak pouzdanost. Ipak, drugo mišljenje katkad može značiti razliku između života i smrti.Before starting treatment, the parents of a sick child may want another doctor to review the diagnosis and treatment plan. Getting a second opinion allows them not only to confirm the diagnosis, but also to gain a different perspective on the child’s treatment options. Some doctors are more conservative and others more aggressive. When parents are invited to make choices their clinicians need to explain about clinical uncertainty and how individual values and preferences may relate to treatment decisions for their child. The options should be communicated in a clear manner, differences in opinion should be acknowledged and the doctor\u27s own preference stated. Parents need to be encouraged and supported to make their decision—whether or not it reflects their doctor\u27s preference. There are a number of ways to find specialists to consult for a second opinion: The scope of this phenomenon is not well understood . Also it is not clear whether it is warranted or not. However sometimes second opinions can mean the difference between life and death

Innovative advantages of the Zagreb surgical protocol of hand reconstruction in patients with epidermolysis bullosa

Kirurško liječenje deformiteta šaka bolesnika s buloznom epidermolizom, unatoč stalnomu napretku znanstvenih spoznaja, još je i danas obilježeno brojnim prijeporima. Cilj istraživanja je na temelju medicinske dokumentacije utvrditi karakteristike, specifičnosti i ishode svih bolesnika s buloznom epidermolizom koji su kirurški liječeni zbog deformiteta šake u Zagrebu u razdoblju od 1994. do 2009. godine. Zagrebački kirurški protokol rekonstrukcije (ZSRP) deformiteta šake u bolesnika s buloznom epidermolizom (EB) jedinstven je alat standardiziranih postupaka i jasno definiranog algoritma. Analizirani su značajke i ishodi u bolesnika s buloznom epidermolizom skupine bolesnika liječenih u razdoblju od 1994. do 2001. sa skupinom bolesnika koji su kirurški liječeni od 2002. do 2009. godine (s follow upom od 2 godine poslije termina operacije) s obzirom na to da su od 2002. godine u kiruršku praksu uvedene nove smjernice s brojnim modifikacijama postupka (ZSRP). Primjena Zagrebačkog kirurškog protokola rekonstrukcije deformiteta šake u bolesnika s buloznom epidermolizom sa svojim inovativnim sastavnicama ima praktične prednosti u usporedbi s prethodno primjenjivanim kirurškim postupcima zbog svoje veće učinkovitosti uz redukciju ukupnih troškova liječenja. Uzimanjem presatka kože po metodi PRST postiže se brže cijeljenje donorskog područja, uz smanjenje rizika očekivanih komplikacija u usporedbi s klasičnim tehnikama uzimanja STSG presatka kože dermatomom ili elektrodermatomom. Prema našem dosadašnjem 20-godišnjem iskustvu primjerena kirurška korekcija deformiteta šaka u bolesnika s EB-om omogućava uspješno višekratno ponavljanje kirurškog postupka koji je indiciran kao posljedica očekivane progresije lokalnog statusa zbog prirode same bolesti.Surgical treatment of the deformity in a handful of patients with epidermolysis bullosa (EB) is still marked by numerous controversies today despite constant advancement of scientific knowledge. The aim of the study was to determine, on the basis of medical documentation, the characteristics, specificities and outcomes of all EB patients surgically treated in Zagreb from 1994 to 2009. The Zagreb Surgical Reconstruction Protocol (ZSRP) of hand deformity in EB patients is a unique tool of standardized procedures and a clearly defined algorithm. Characteristics and outcomes were compared between EB patients treated from 1994 to 2001 and EB patients treated surgically from 2002 to 2009 (with 2-year follow-up after surgery), given that new guidelines with numerous modifications to the procedure (ZSRP) had been introduced into surgical practice from 2002. Using ZSRP for reconstruction of hand deformity in EB patients with its innovative components was found to have practical advantages compared to the previously applied surgical procedures because of its higher efficiency while reducing the overall cost of treatment. This method resulted in faster healing of the donor area, reducing the risk of expected complications compared to the conventional split-thickness skin grafting techniques with a dermatome or electrodermatome. According to our 20-year experience, appropriate surgical correction of hand deformity in EB patients enables successful repeated surgery, which is indicated as a result of the expected progression of local status due to the nature of the disease itself

Fibrin u tkivnom inženjerstvu kože – proizvodnja i kliničko iskustvo

The purpose of this study was to create a fibrin-based human skin substitute in vitro with epidermal and dermal component and to assess its healing potential in deep partial and full thickness burns. Fibrin scaffolds were prepared from commercial fibrin glue kits. Human fibroblasts were cultured in fibrin gel. Human keratinocytes were seeded on the top of the gel. Viability of cells was determined fluorimetrically. Scanning electron microscope and immunocytochemistry analysis of cultured cells were performed. After hydrosurgical preparation of deep burn necrotic tissue, wound bed was prepared for skin substitutes. Progress of healing was documented using visual estimation and photos. Scanning electron microscope images showed good cell attachment and colony spreading of keratinocytes and fibroblasts on fibrin scaffold. Immunofluorescent staining of cell cultures on fibrin scaffold showed expression of vimentin, a marker of fibroblast cells, cytokeratin 19, a marker of epithelial stem cells, as well as involucrin, a marker of differentiated keratinocytes. Clinical results clearly showed that appearance of the skin did not differ significantly from the areas of transplanted skin using split-thickness skin graft techniques. In conclusion, using these fibrin-cultured autografts on massive full-thickness burn resulted in good healing.Cilj ovoga istraživanja bio je kreirati in vitro nadomjestak ljudske kože s epidermalnim i dermalnim dijelom. Pritom smo koristili fibrinski gel kao nosač stanica. Procijenili smo učinak nadomjestka kože na cijeljenje opeklina drugog i trećeg stupnja. Fibrinski gel načinjen je od komercijalnog kirurškog fibrinskog ljepila. Ljudski fibroblasti kultivirani su u samom fibrinskom nosaču. Ljudski keratinociti zasijani su na gornju površinu nosača. Vijabilnost uzgojenih stanica određena je fluorimetrijski. Nadomjestak kože analiziran je elektronskim skenirajućim mikroskopom. Napravljena je imunocitokemijska analiza kultiviranih stanica. Skenirajući mikroskop pokazao je dobro prianjanje i proliferaciju stanica kože na nosaču. Imunocitokemijska analiza pokazala je prisutnost vimentina, biljega fibroblastnih stanica; citokeratina 19, biljega epitelnih matičnih stanica; te involukrina, biljega diferenciranih keratinocita. Klinička primjena ovih nadomjestaka kože pokazala je slične rezulatate cijeljenja kao i dijelovi rana koji su prekriveni autolognim presatcima kože djelomične debljine. Zaključujemo da nadomjestci kože bazirani na fibrinskom nosaču imaju potencijala u liječenju teških opeklina

EWING SARCOMA OF THE RIB – CASE REPORT

Ewingov sarkom je jako maligni koštani tumor koji je prvi put u literaturi opisao J a m e s E w i n g 1921. godine (1). Ewingov sarkom se obično javlja u dječjoj ili ranoj adolescentnoj dobi, s najvećom učestalošću između 10. i 20. godine života, premda se može javiti i kod mlađe i starije životne dobi. Najučestalije se javlje u području zdjelice, natkoljenične i nadlaktične kosti te rebara. Ewingov sarkom je po učestalosti pojavljivanja drugi tumor koštanog tkiva kod djece i najmaligniji koštani tumor dječje dobi. Učestalost obolijevanja je 0.3 bolesnika na 1.000.000 djece mlađe od 3 godine i 4.6 djece na 1.000.000 mladih ljudi između 15 i 19 godina života. Prosječna učestalost obolijevanja je ispod 2 bolesnika na 1.000.000 djece na godinu. Odnos muške i ženske djece koja obolijevaju je 1.5:1 (2-6). Preživljavanje kod lokaliziranog tumora je 60-70% oboljelih, 30% preživljavanje je s metastazama po plućima te preživljavanje manje od 10% je s metastazama na drugim mjestima (7, 8). Prikazujemo osmogodišnju djevojčicu s Ewingovim sarkomom na rebru.Ewing sarcoma, a highly malignant primary bone tumor, was first described by James Ewing in 1921 (1). Ewing sarcoma usually presents in childhood or early adulthood, with its peak between 10 and 20 years of age, although it can occur in younger children and older adults. The most common areas in which it occurs are the pelvis, the femur, the humerus and the ribs. Ewing sarcoma is the second most common malignant bone tumor in young patients and it is the most lethal bone tumor. Tumor occurrence ranges from 0.3 cases per 1 000 000 children younger than 3 years to as high as 4.6 cases per 1 000 000 adolescents aged 15-19 years. The annual incidence rate averages less than 2 cases per 1 000 000 children. The male-to-female ratio is 1.5:1 (2-6). Survival rate is 60-70% with low-risk, in a localized tumor, intermediate risk (30%) when the tumor has spread to lungs and advanced risk (less than 10%) when the tumor has spread further to bone, bone marrow and/or other tissue (7, 8). We present an 8 year-old girl with Ewing sarcoma of the rib

EWING SARCOMA OF THE RIB – CASE REPORT

Ewingov sarkom je jako maligni koštani tumor koji je prvi put u literaturi opisao J a m e s E w i n g 1921. godine (1). Ewingov sarkom se obično javlja u dječjoj ili ranoj adolescentnoj dobi, s najvećom učestalošću između 10. i 20. godine života, premda se može javiti i kod mlađe i starije životne dobi. Najučestalije se javlje u području zdjelice, natkoljenične i nadlaktične kosti te rebara. Ewingov sarkom je po učestalosti pojavljivanja drugi tumor koštanog tkiva kod djece i najmaligniji koštani tumor dječje dobi. Učestalost obolijevanja je 0.3 bolesnika na 1.000.000 djece mlađe od 3 godine i 4.6 djece na 1.000.000 mladih ljudi između 15 i 19 godina života. Prosječna učestalost obolijevanja je ispod 2 bolesnika na 1.000.000 djece na godinu. Odnos muške i ženske djece koja obolijevaju je 1.5:1 (2-6). Preživljavanje kod lokaliziranog tumora je 60-70% oboljelih, 30% preživljavanje je s metastazama po plućima te preživljavanje manje od 10% je s metastazama na drugim mjestima (7, 8). Prikazujemo osmogodišnju djevojčicu s Ewingovim sarkomom na rebru.Ewing sarcoma, a highly malignant primary bone tumor, was first described by James Ewing in 1921 (1). Ewing sarcoma usually presents in childhood or early adulthood, with its peak between 10 and 20 years of age, although it can occur in younger children and older adults. The most common areas in which it occurs are the pelvis, the femur, the humerus and the ribs. Ewing sarcoma is the second most common malignant bone tumor in young patients and it is the most lethal bone tumor. Tumor occurrence ranges from 0.3 cases per 1 000 000 children younger than 3 years to as high as 4.6 cases per 1 000 000 adolescents aged 15-19 years. The annual incidence rate averages less than 2 cases per 1 000 000 children. The male-to-female ratio is 1.5:1 (2-6). Survival rate is 60-70% with low-risk, in a localized tumor, intermediate risk (30%) when the tumor has spread to lungs and advanced risk (less than 10%) when the tumor has spread further to bone, bone marrow and/or other tissue (7, 8). We present an 8 year-old girl with Ewing sarcoma of the rib

THE IMPACT OF EDUCATION ON CHRONIC WOUND CARE IMPROVEMENT

Inherited epidermolysis bullosa is a group of diseases characterized by skin/mucous membrane fragility and development of blisters and erosions after insignificant mechanical trauma. It is a multisystemic disease with complications occurring on numerous organs other than the skin. As there is no cure for these diseases, treatment consists of early recognition and therapy of complications, quality wound care and skin protection. Optimal wound treatment depends primarily on the type of the disease, localization and type of wounds. Apart from good skin care, treatment of these patients requires intensive supportive therapy in which various specialists must be involved.Although not fully understood, close relationship between health and education ensures unambiguous health and quality of life advantages to educated individuals. Education ensures different thinking and decision making processes and man is enabled to receive information from the external world. Even though the process of education and learning still relies on banking principles and coping of common knowledge, modern and technological society drives the system as well as education opportunities towards the new learning sources. In the developed world, the impact of chronic wounds on health systems is fairly perceived, as well as chronic wound treatment and education. Our health system still neglects the significant impact of chronic wounds on social and economic, individual and community well-being. Recognizing the importance of chronic wounds and implementation of a developed educational system gives us the potential for improving care for chronic wounds, and thus to substantially improve the quality of life of patients. Furthermore, consequent reduction of unnecessary health costs could reallocate substantial resources to other points of interest

A 10-Year Experience in the Treatment of Intraabdominal Cerebrospinal Fluid Pseudocysts

The aim of this retrospective study was to assess a ten-year experience in the treatment of rare complications of ventriculoperitoneal shunting – intraabdominal cerebrospinal fluid pseudocysts. At this time there are no data about incidence, clinical course and treatment of these complications in Croatia. Cerebrospinal fluid (CSF) abdominal pseudocyst is an uncommon but important complication of ventriculoperitoneal shunts. Retrospective data were obtained from 5 children with abdominal CSF pseudocysts, treated between 1996 and 2007. The incidence of intraabdominal CSF pseudocysts in our study is 2.9%. All patients were girls ranged in age from 4 to 12 years old (mean 8.8 years). In most cases etiology of hydrocephalus was congenital, idiopathic. Abdominal pain and distension were the most frequent clinical finding (4/5). Although infection has been reported as responsible for pseudocyst formation, we did not found it in our series. Laparotomy with cyst wall excision and catheter replacement was performed in 2/5 cases, and only cyst fluid aspiration with catheter replacement in 3/5 cases. Recurrence of the abdominal cyst was observed in one girl who was in terminal stadium of anaplastic ependymoma. It is our opinion that only catheter replacement and cyst fluid evacuation, as one of the treatment modalities, may be successful, even in large CSF intraperitoneal pseudocysts

THE PARADIGM OF A SECOND OPINION IN THE FIELD OF PAEDIATRIC MEDICINE

Možda će roditelji bolesnog djeteta prije započetog liječenja izraziti želju da drugi liječnik pregleda postavljenu dijagnozu i plan liječenja. Drugo mišljenje omogućava roditeljima da budu sigurniji u točnost postavljene dijagnoze, ali ono ujedno olakšava razmatranje i donošenje odluke o najboljoj opciji tretmana. Neki su liječnici “konzervativniji”, neki “agresivniji”. Kad se od roditelja očekuje da odluče o tretmanu svoga djeteta, kliničar treba razjasniti nesigurnosti i evenutalne dvojbe. Prije ili tijekom liječenja roditelje je potrebno ohrabriti i podržati njihovu želju za dobivanjem drugog mišljenja - bez obzira na liječnički stav o nužnosti takvog postupanja. Načini dobivanja drugog stručnog mišljenja su različiti. Djelokrug tog područja nije u potpunosti rasvijetljen. Također nije jasno jamči li taj postupak pouzdanost. Ipak, drugo mišljenje katkad može značiti razliku između života i smrti.Before starting treatment, the parents of a sick child may want another doctor to review the diagnosis and treatment plan. Getting a second opinion allows them not only to confirm the diagnosis, but also to gain a different perspective on the child’s treatment options. Some doctors are more conservative and others more aggressive. When parents are invited to make choices their clinicians need to explain about clinical uncertainty and how individual values and preferences may relate to treatment decisions for their child. The options should be communicated in a clear manner, differences in opinion should be acknowledged and the doctor\u27s own preference stated. Parents need to be encouraged and supported to make their decision—whether or not it reflects their doctor\u27s preference. There are a number of ways to find specialists to consult for a second opinion: The scope of this phenomenon is not well understood . Also it is not clear whether it is warranted or not. However sometimes second opinions can mean the difference between life and death

RAPID COMMUNICATION Treatment of rectal prolapse in children with cow milk injection sclerotherapy: 30-year experience

contributed equally to this wor