Julije Meštrović

Esophagotracheal fistula after lithium disc battery ingestion successfully treated with hyperbaric oxygen therapy

We report a case of a 12-month-old child who acquired an esophagotracheal fistula of 5 mm in diameter after an ingested lithium disc battery impaction. Failure to recognize foreign body on chest X-rays resulted in a delay of 28 days in establishing the diagnosis. Feeding via nasogastric tube and hyperbaric oxygen treatment (HBOT) resulted in a complete closure of the fistula after 17 HBOT 60 min sessions at 2.2 bars.

Desfecho funcional de crianças tratadas em unidade de terapia intensiva

OBJETIVO: O desfecho de pacientes nao e somente determinado pelo indice de gravidade de doenca, mas tambem pelo impacto do estado pre-admissao de comorbidade dos pacientes. Portanto, este artigo buscou avaliar o desfecho de pacientes tratados em uma unidade de terapia intensiva pediatrica, com foco especial no grupo de criancas com doencas cronicas. METODOS: Os dados foram obtidos prospectivamente, e o desfecho foi avaliado segundo a escala Pediatric Overall Performance Category para 449 pacientes de uma unidade de terapia intensiva pediatrica do Split University Hospital. O desempenho funcional foi avaliado como o escore pre-admissao e o escore na alta hospitalar em pacientes com alteracoes neurodesenvolvimentais, com outras doencas cronicas e sem doenca cronica. RESULTADOS: O estado funcional a alta hospitalar foi significativamente dependente do estado funcional pre-admissao e da mortalidade prevista. Criancas com alteracoes neurodesenvolvimentais apresentaram escore basal significativamente pior e deterioracao de morbidade funcional na alta hospitalar significativamente menor, comparadas com criancas sem doenca cronica e com criancas com outras doencas cronicas. CONCLUSOES: A escala Pediatric Overall Performance Category demonstrou sua aplicabilidade em uma pequena unidade de terapia intensiva com uma populacao heterogenea de pacientes. Deve, portanto, ser considerada para avaliacao regular de qualidade de cuidados a saude como uma ferramenta simples e precisa. Ao contrario do que acontece com outros pacientes, o estado funcional de criancas com alteracoes neurodesenvolvimentais foi marcadamente influenciado por sua comorbidade. Seu estado pre-admissao foi pior do que o de outras criancas e, por isso, nao poderia estar significativamente deteriorado na alta hospitalar.

Neurodevelopmental disabilities and quality of life after intensive care treatment

Aim:  To analyze the quality of life after Pediatric Intensive Care Unit (PICU) treatment and compare the differences between quality of life in children who suffer chronic health conditions, and those who do not, post discharge from PICU.

Applicability of the Australian and New Zealand Paediatric Intensive Care Registry diagnostic codes and Paediatric Index of Mortality 2 scoring system in a Croatian paediatric intensive care unit

The severity of illness scoring systems enable benchmarking of standard of care among various paediatric intensive care units (PICU). The most accurate validation of a scoring system is the system’s evaluation in a population other than that from which the score is derived [3]. PICUs that collaborated in the Paediatric Index of Mortality (PIM2) study are large units, and most of them pertain to the Australian centralised paediatric intensive care service [5]. The PIM2 evaluates the condition of the patient at the first contact, during the first hour after arrival in the PICU. The score is calculated on linear regression equations from ten physiological and clinical data that can be routinely collected by trained medical staff. The Australian and New Zealand Paediatric Intensive Care Registry (ANZPIC Registry) diagnostic codes is a proposed diagnostic coding system for international use. The Registry codes the principal and up to five associated diagnoses [4]. We performed a prospective, observational study to evaluate the applicability of the ANZPIC Registry diagnostic codes, and validity of the PIM2 scoring system in a PICU diverse from those in which the two systems were created. The PICU of the Clinical Hospital Split is a regional, sevenbed, tertiary multidisciplinary unit of a public, university-affiliated hospital. Between June 2002 and July 2004, admission data were prospectively collected for 591 consecutively admitted patients aged £ 18 years, excluding preterm infants. PIM2-based mortality risk was calculated according to the equations published in the literature [5]. Diagnoses of all patients were classified according to the ANZPIC Registry diagnostic codes. The median age of our patients was 62 months (range 0.03–216 months). There were 89 (15%) elective admissions, and 168 patients (28.4%) were mechanically ventilated during the first hour. Median length of stay was 2 days. For 2.5% of patients, the exact principal diagnosis could not be selected from the ANZPIC Registry diagnostic codes. Among the 370 codes that create the system, 109 were eligible for our population of patients as principal diagnoses. The five most frequent diagnoses were: pneumonia or pneumonitis (8.6%), head trauma (8%), seizures (7.6%), laryngobronchoscopy (4.1%), and ingestion (4.1%). Out of 158 (26.7%) surgical patients, 46 (29.1%) were admitted for head trauma. Observed mortality was 35/591 (5.9%, 95% confidence interval (CI) 4.0 to 7.9%). All deaths were from the unscheduled admissions. The most frequent cause of death was head trauma (7/35, 20%). Predicted mortality was 35/591 (5.9%). Standardised mortality ratio (ratio of observed to expected deaths, SMR) was 1.00. Calibration, assessed by the Hosmer-Lemeshow goodness of fit test [2] in deciles of mortality risk is shown in Table 1. The Hosmer-Lemeshow goodness of fit test showed v=19.59, P =0.012. The sensitivity of the PIM2 was 65.7%, and the specificity was 99.3%. Predictive capability was assessed by calculating the area under the receiver operating characteristic curve (ROC). The predictive power for PIM2, expressed as area under the ROC curve, was 0.97 (95% CI 0.96 to 0.99). Results of previous external validation studies showed adequate performance of the PIM and prediction of death in heterogeneous groups of PICU patients [1,6]. The continuous improvement in paediatric intensive care requires the updating of existing mortality prediction models. Therefore, we conducted a study with the revised PIM scoring system. In spite of the relatively J. Mestrovic (&) Æ B. Polic Æ A. Omazic Æ L. Stricevic Paediatric Intensive Care Unit, Department of Paediatrics, Clinical Hospital Split, Spinciceva 1, 21000 Split, Croatia E-mail: julije.mestrovic@st.t-com.hr Tel.: +385-21-556686 Fax: +385-21-556590

Purpura fulminans successfully treated with hyperbaric oxygen--a report of 2 cases.

Purpura fulminans is a rare but serious postinfectious disorder of children. It appears during the recovery period after some common viral or bacteriologic infections. Its course is fulminant and cacracterized by appearance of diffuse hamorrhage with vasculitis, necrosis and gangrene of skin and subcutaneous tissue, toxemia and shock. The changes are, as rule, located symmetrically on lower limbs and gluteal regions. An ambiguity as to the usage of the term purpura fulminans exists in the literature. Skin manifestations during meningococcus or some other bacteriologic sepsis are also called purpura fulminans by some authors. Current tratment includes steroids, anticoagulants, protein C concentrate, blood derivates, and antibiotics. Since currently accepted therapeutic measures often fail, amputations of toes, fingers, and limbs are frequent. Fatal outcome was described in up to 90% of the cases. Only few paperas suggesting the efficiency of hyperbaric oxygen(HBO) in the treatment of purpura fulminans are available, reporting of a favorable outcome in 85% of the cases. Since purpura fulminans is not listed among the indications for HBO approved by the Undersea and Hyperbaric Medical Society, new data sugesting the efficiency of HBO in its treatment might be useful in clinical practise.

Life-threatening Valproate Overdose Successfully Treated with Haemodialysis

Life-threatening Valproate Overdose Successfully Treated with Haemodialysis Valproate (VPA) poisoning is an increasing clinical problem. The most common finding in VPA overdose is the depression of the central nervous system, which may progress to coma and death. This type of poisoning is difficult to treat, as no antidote exists. This report describes a case with a 16-year-old girl who poisoned herself with valproate. Initial treatment included naloxone, but she did not respond. She became comatose, with serum VPA concentration of 1320 μg mL-1. Three sessions of haemodialysis were performed, effectively eliminating VPA and decreasing the serum concentration. The patient regained consciousness and fully recovered. To our knowledge, this is the highest serum VPA concentration reported by now in children aged 16 or less. Haemodialysis has proved to be the treatment of choice for life-threatening acute VPA overdose in children. Uspješno liječenje teškog trovanja valproatom s pomoću hemodijalize Trovanje valproatom sve je češći klinički problem. Najčešći simptom nađen kod trovanja je depresija središnjega živčanog sustava koja može dovesti do kome i smrti. Trovanje se teško liječi jer antidot ne postoji. Prikazujemo slučaj 16-godišnje djevojke koja se otrovala valproatom. U početku je liječena naloksonom, ali nije došlo do poboljšanja. Postala je komatozna s koncentracijom valproata u serumu od 1320 μg mL-1. Provođenjem triju ciklusa hemodijalize serumska koncentracija valproata učinkovito je smanjena, nakon čega se razina svijesti vratila na normalu i djevojka se potpuno oporavila. Prema našim saznanjima, ovo je slučaj s najvišom izmjerenom serumskom koncentracijom valproata u djece od 16 godina ili manje. Na osnovi dobivenih rezultata preporučujemo uporabu hemodijalize u liječenju teških trovanja valproatom u djece.

Immune Modulation Therapy in a CRIM-Positive and IgG Antibody-Positive Infant with Pompe Disease Treated with Alglucosidase Alfa: A Case Report

Pompe disease is characterized by deficiency or absence of activity of the lysosomal enzyme acid alpha-glucosidase. As a result of ineffective metabolism, glycogen progressively accumulates in muscle tissues. Patients with an aggressive classic infantile-onset form generally rapidly die of cardiorespiratory failure. A cross-reactive immunological material (CRIM)-negative status is predictive of high anti-alglucosidase alfa antibody titers and usually a poor clinical outcome of enzyme replacement therapy (ERT). CRIM-positive patients can also develop robust antibody titers complicating therapeutic management.We successfully used an immune modulation therapy (IMT) protocol in a CRIM-positive infantile-onset patient with Pompe disease in whom infusions had to be temporarily discontinued because of safety concerns despite administration of pre-infusion medication. Prior to discontinuation, she had shown signs of clinical deterioration and continuous ventilation support through a tracheostomy was required. She was found to be positive for anti-alglucosidase alfa antibodies (1:6,400). IMT (rituximab, methotrexate and intravenous gamma globulin) was started, ERT was safely reintroduced during the IMT induction phase and, subsequently, the enzyme dose was increased, all without any complications. Antibodies disappeared, IMT was tapered and discontinued, and cadiomyopathy steadily improved. During 1 year of follow-up, she remained ventilator dependent and no gains in motor skills were noticed; motor functions will be closely monitored during sustained ERT.Although the reversal of clinical decline in our CRIM-positive and antibody-positive infant with Pompe disease cannot be solely attributed to IMT, our experiences with this protocol may be helpful to other physicians encountering comparable therapeutic dilemmas.

Effects of immune modulation therapy in the first Croatian infant diagnosed with Pompe disease: a 3-year follow-up study

SummaryPompe disease is a storage disorder characterized by deficient or absent activity of the enzyme acid alpha-glucosidase. As a result of ineffective metabolism, glycogen accumulates in muscle tissues. Patients with a classic infantile-onset form present by the first few months of life with hypertrophic cardiomyopathy and muscle weakness. If left untreated, these patients rapidly die of cardiorespiratory failure. A cross-reactive immunological material (CRIM)-negative status is predictive of high anti-alglucosidase alpha antibody titers. However, CRIM-positive patients also sometimes develop robust antibody titers. High antibody titers complicate therapeutic management, and those patients have a worse clinical outcome of enzyme replacement therapy (ERT).Four years ago, we successfully used an immune modulation therapy (IMT) protocol in a CRIM-positive infantile-onset patient with Pompe disease in whom ERT had to be discontinued because of severe infusion-associated reactions. She was found to be positive for anti-alglucosidase alpha antibodies. IMT (rituximab, methotrexate, and intravenous gammaglobulin) was started, and ERT was safely reintroduced during the IMT induction phase without any complications. Antibodies disappeared; IMT was tapered and discontinued; and cardiomyopathy steadily improved. During more than 3 years of follow-up, she remained ventilator dependent, and no gains in motor skills were noticed. The antibodies are still undetectable, and no adverse reactions associated with IMT had occurred. The cardiomyopathy is gradually increasing, but there is still ~ 50 % reduction as compared with the highest value measured. Although the reversal of clinical decline in our CRIM-positive and antibody-positive infant cannot be solely attributed to IMT, this protocol proved itself efficient and safe.ZusammenfassungDie Pompe’sche Krankheit ist eine Speicherkrankheit, die durch eine gestörte oder fehlende Aktivität des Enzyms saure alpha-Glucosidase gekennzeichnet ist. Als Folge des ineffizienten Stoffwechsels reichert sich Glykogen im Muskelgewebe an. Patienten mit der klassischen Form des Ausbruchs der Erkrankung im Kindesalter präsentieren sich bereits in den ersten Lebensmonaten mit einer hypertrophen Kardiomyopathie und Muskelschwäche. Unbehandelt sterben diese Patienten rasch am kardiorespiratorischen Versagen.Ein kreuzreaktiv immunologisches Material (CRIM) negativer Status sagt hohe anti-alpha Glucosidase Antikörper voraus. CRIM positive Patienten haben allerdings auch manchmal deutlich erhöhte Antikörper Titer. Hohe Antikörper Titer machen das therapeutische Management kompliziert: diese Patienten sprechen klinisch schlechter auf eine Enzymersatz Therapie an.Vor 4 Jahren verwendeten wir ein Protokoll einer immumodulatorischen Therapie (IMT) erfolgreich bei einer CRIM positiven Patientin mit Ausbruch der Pompe’schen Krankheit im Kindesalter, bei der die Enzymersatz-Therapie wegen schwerer infusions-assoziierter Reaktionen abgesetzt werden musste. Sie hatte anti-alpha Glucosidase Antikörper. Eine IMT bestehend aus Rituximab, Methotrexat, und intravenösem Gammaglobulin wurde begonnen. Während der IMT konnte die Enzymersatz Therapie wieder sicher ohne irgendwelche Komplikationen begonnen werden. Die Antikörper verschwanden und die IMT konnte ausgeschlichen beziehungsweise abgesetzt werden. Die Kardiomyopathie wurde kontinuierlich besser.Während mehr als 3 Jahren follow-up blieb die Patientin Respirator abhängig – es wurde keine Besserung der motorischen Fähigkeiten beobachtet. Die Antikörper sind weiterhin unter dem Detektionslimit. Die Kardiomyopathie wird nun langsam schlechter – ist aber noch immer 50 % besser im Vergleich zum schlechtesten im Verlauf gemessenen Wert.Obwohl die klinische Besserung unseres CRIM- und Antikörper-positiven Kindes nicht nur der IMT zugeschrieben werden kann, zeigte sich das Protokoll jedenfalls als wirksam und sicher.

LATE VENTRICULOPERITONEAL SHUNT INFECTION CAUSED BY SHEWANELLA ALGAE

We present a case of ventriculitis and peritonitis in a child with ventriculoperitoneal shunt, which occurred 5 years after the surgery. The infection developed after contact with seawater and began as otitis. For the first time, Shewanella algae, a marine microorganism, was identified as the cause of ventriculoperitoneal shunt infection.

Use of central venous catheters in children

The objective of this study was to evaluate the use of central venous catheters (CVCs) in the Pediatric intensive care unit (PICU) of Split University Hospital (SUH). We reviewed the records of all children that had CVCs and were hospitalized between January 2002 and March 2006. Patients were evaluated with respect to their age, gender, catheter type, indication for CVC insertion, site and side of the body of CVC insertion. The duration of catheter use and eventual complications were also taken into consideration. A total of 352 CVCs were inserted in 300 children. Patient age ranged from 0 to 18 years. The average catheter insertion time was 12.88 days. We noted 66 (18.8%) CVC-related complications. Complications related to CVCs insertion were malposition of catheter (5.4%) and pneumothorax (0.9%). Occlusion of CVCs (4.3%), catheter related-bloodstream infections (CRBI) (4.0%), dislodgment (3.7%) and catheter damage (0.6%) were complications associated with lenght of CVCs use. We conclude that central venous catheterization is a safe and efficient procedure with minimal complications in pediatric patients.