Lipoid pneumonia--a case of refractory pneumonia in a child treated with ketogenic diet.

Lipoid pneumonia (LP) is a chronic inflammation of the lung parenchyma with interstitial involvement due to the accumulation of endogenous or exogenous lipids. Exogenous LP (ELP) is associated with the aspiration or inhalation of oil present in food, oil-based medications or radiographic contrast media. The clinical manifestations of LP range from asymptomatic cases to severe pulmonary involvement, with respiratory failure and death, according to the quantity and duration of the aspiration. The diagnosis of exogenous lipoid pneumonia is based on a history of exposure to oil and the presence of lipid-laden macrophages on sputum or bronchoalveolar lavage (BAL) analysis. High-resolution computed tomography (HRCT) is the imaging technique of choice for evaluation of patients with suspected LP. The best therapeutic strategy is to remove the oil as early as possible through bronchoscopy with multiple BALs and interruption in the use of mineral oil. Steroid therapy remains controversial, and should be reserved for severe cases. We describe a case of LP due to oil aspiration in 3-year-old girl with intractable epilepsy on ketogenic diet. Diagnostic problems were due to non-specific symptoms that were mimicking serious infectious pneumonia. A high index of suspicion and precise medical history is required in cases of refractory pneumonia and fever unresponsive to conventional therapy. Gastroesophageal reflux and a risk of aspiration may be regarded as relative contraindications to the ketogenic diet. Conservative treatment, based on the use of oral steroids, proved to be an efficient therapeutic approach in this case.

Virtual simulations in planning intravascular treatment of aortic coarctation - a retrospective analysis.

Introduction: A number of studies on using both three-dimensional printing and virtual models in assessment of aortic coarctation have been published, yet none of them uses virtual modelling as a planning tool in a blind retrospective analysis. Aim: Assessment of virtual modelling and virtual reality in planning interventional treatment of aortic coarctation. Material and methods: The study involved computed tomography scans of 20 patients performed prior to interventional treatment of aortic coarctation, which were used to create a virtual three-dimensional model of the aorta in Materialise Mimics. A group of potential stents was modelled in Materialise 3-Matic and complete simulations were assessed in Mimics Viewer using a virtual reality headset in order to choose an optimal stent, which was later compared with the implanted one. Results: In 5 cases identical or very similar stents were proposed, in 12 cases simulations had slight, potentially avoidable misestimations either in stent length or diameter, and in 3 cases differences were more considerable. Overall, in 14 cases the location of the stent was concordant between the simulation and reality and in the remaining 6 cases the simulated stent was located lower than the actual one. Conclusions: The method of computer modelling provided a satisfactory success rate of predicting the possible stents to use during the procedure. Differences in chosen stents may have been caused by individual experience in interventional cardiology, the lack of availability of certain stents in the heart catheterization laboratory, the lack of information about the diameter of the vascular access and differences in dimensions measured on the model, tomography and angiography.

Inferior Vena Cava and Venous Outflow Reconstruction in Living Donor Liver Transplantation in Children: A Single-Center Retrospective Study and Literature Review.

BACKGROUND In this report, we present technical problems and solutions used in the reconstruction of the inferior vena cava and graft venous outflow during living donor liver transplantation (LDLT) in children. MATERIAL AND METHODS In 65 grafts out of 379 liver transplantations from living donors, reconstruction of multiple hepatic venous branches and/or IVC was necessary. In 4 cases, cryopreserved deceased donor venous grafts were used for the reconstruction of the IVC and/or HV. RESULTS Follow-up ranged from 2 months to 17.8 years (median 7.2 years). In 4 children, liver re-transplantation was required for a reason not related to venous outflow (biliary complications in 3 patients, graft insufficiency caused by small-for-size syndrome). Two patients died: 1 due to tumor recurrence and 1 due to multi-organ failure. Fifty-nine patients are alive with good liver function. One patient (1.5%) after deceased donor venous graft reconstruction showed symptoms of venous outflow obstruction, which was successfully treated with endovascular balloon angioplasty and stent placement. The remaining 59 transplanted patients do not show any signs of venous outflow obstruction. CONCLUSIONS In most cases, the reconstruction of multiple hepatic veins of living donor allografts can successfully be done with local venoplasty, while using cold-stored vein grafts may be helpful in selected cases of LDLT.

Invitation to participate in a multi-center study for validation of cerebral computed tomography angiography and computed tomography perfusion in the determination of cerebral circulatory arrest during brain death/death by neurological criteria diagnosis procedure in paediatric population below 12 years of age.

We would like to invite paediatric intensive care units (PICU) to join our multi-center trial concerning patient population < 12 y/o and aiming at: • validation of computed tomography angiography (CTA)/computed tomography perfusion (CTP) tests for brain death/death by neurological criteria (BD/DNC) diagnosis procedures, • validation of duplex Doppler insonation of extracranial segments of the internal cerebral arteries and the vertebral arteries for choosing an optimal time for CTA/CTP testing.

Hemodynamic failure as an indication to urgent liver transplantation in infants with giant hepatic hemangiomas or vascular malformations--report of four cases.

The aim of this study was to present acute hemodynamic failure as a rare indication for liver transplantation in neonates and infants with liver hemangiomatosis. We report four patients aged one to six months with giant liver hemangiomas, with huge arterio-venous shunting within these malformations. In three, many skin hemangiomas were found. All children developed right ventricular failure. In two, a trial of pharmacological reduction was attempted with corticosteroids and cyclophosphamide. In one patient, the arterio-venous fistulas were embolized without any improvement in hemodynamic status. Two children underwent rescue hepatic artery surgical ligation, which did not prevent heart and then multiorgan failure including liver failure. After unsuccessful conventional therapy, all infants were considered for urgent liver transplantation; in three cases, it was performed with a living-related donor, and in one case with a deceased donor. All patients are alive and well with the follow-up between nine and 37 months after transplantation. Liver transplantation should be considered as a rescue treatment in children with hepatic vascular malformations leading to hemodynamic insufficiency when conventional therapy is unsuccessful and multiorgan failure develops.

Non-resectable hepatic tumors in children - role of liver transplantation.

BACKGROUND: There is a group of children with primary hepatic tumors which can not be resected by conventional partial liver resection. Total hepatectomy followed by liver transplantation may be the only solution in such cases. Authors reviewed own experience with the liver transplantation for unresectable tumors in children and assessed the possible indications and role of transplantation in these patients. MATERIAL AND METHODS: Liver transplantation was performed in 17 children with unresectable hepatic tumors out of total number of 350 children transplanted. Hepatocarcinoma was present in 8 children, hepatoblastoma in 6 and benign giant hemangioma in 3. There was no other option for the treatment which would lead to the oncological cure of children with malignant tumors. All patients with giant hemangiomas were infants transplanted urgently due to circulatory and then multiorgan failure. RESULTS: Survival within whole group is 75.5% (13 of 17 pts), 3 children died of malignant tumor recurrence, one of other causes. All 3 children with benign tumors are alive and well. Actual follow-up is from 3 months to 7 years. CONCLUSIONS: Liver transplantation should be considered as option in the treatment of all children with unresectable hepatic tumors. With the careful and individual patient selection significant chances for survival can be achieved in this group of patients which would otherwise not survive with the conventional treatment.

[Bilateral papillary renal cell carcinoma in a patient post-renal transplantation]. / Obustronny rak "renal cell carcinoma" o typie brodawkowatym u pacjenta po transplantacji nerki.

Renal cancer accounts for about 2% of all cancers in general population. One of the risk factors is acquired cystic kidney disease developed in course of end- stage renal failure. About 2% of cases of renal cancer are associated with inherited syndromes. We present here the case of 18-year old boy with papillary bilateral renal cell carcinoma, which was found 3 years after renal transplantation, in the stage of graft failure, few days after graphtectomy, while looking for the reason of persistent fever.

Metastases of pancreatic neuroendocrine tumor to the liver as extremely rare indication for liver transplantation in children. Case report and review of the literature.

Neuroendocrine tumors (NET) are extremely rare in children (0.75 cases per 100,000 children and adolescents a year) and the majority of these tumors are benign or present low grade of malignancy. According to the American registry Surveillance, Epidemiology, and End Results (SEER) Program of the National Cancer Institute, less than 2% of all neuroendocrine tumors in children occur in the pancreas, making it a rare site for these tumors. The majority of them are found in children over 10years of age, especially those with malignant potential. Treatment of NET consists of different methods: surgery, somatostatin analogues and chemotherapy. Radical surgical resection remains the standard of treatment; however, it is not always feasible because of distant metastases. The authors present a case report of pancreatic NET with multiple metastases to the liver. The patient was treated with pancreatic resection and liver transplantation for liver metastases. Prior to liver transplantation, the patient was treated with somatostatin analogues, sunitinib and chemotherapy. Management of liver metastases with liver transplantation is discussed.

Treatment of undifferentiated embryonal sarcoma of the liver in children--single center experience.

BACKGROUND: Undifferentiated embryonal sarcoma of the liver (UESL) represents less than 5% of all malignant hepatic tumors in childhood. It is considered an aggressive neoplasm with an unfavorable prognosis. The aim of this paper is to present a single center experience in the treatment of children with UESL. MATERIALS AND METHODS: Ten children with UESL were treated between 1981 and 2012. Age at diagnosis ranged from 4 months to 17 years (median age, 6 years and 9 months). Surgery after neoadjuvant chemotherapy (CHT) was performed in 7 patients, and in 3 patients primary surgery was done. Adjuvant chemotherapy was administered in all 10 patients (CYVADIC, CAV, CAV/ETIF/IF+ADM, CDDP/PLADO). Right hemihepatectomy was performed in 1 patient, extended right hemihepatectomy in 6, and partial resection of the right lobe (segments V-VI, segment V) in 2 patients. One patient with unresectable tumor affecting both lobes was listed for liver transplantation (LTx). RESULTS: Follow-up from diagnosis ranged from 50 to 222 months (mean 138 months). Among 9 patients treated with partial liver resection, distant metastases/local recurrence was not observed in any, and disease-free survival in this group is 100% (9 patients alive). The patient that underwent liver transplantation died of multiorgan failure 4 months postoperatively. However, this patient was misdiagnosed as having hepatoblastoma (HBL) and received PLADO chemotherapy. The overall survival rate is 90%. CONCLUSION: Excellent results with long-term survival can be achieved in children with UESL with conventional therapy, including a combination of neoadjuvant and adjuvant chemotherapy and surgery, even in large extensively growing tumors.

Changing treatment and outcome of children with hepatoblastoma: analysis of a single center experience over the last 20 years.

BACKGROUND/PURPOSE: The aim of the study was to analyze changing management and survival of children with hepatoblastoma (HBL) treated in one center. MATERIALS AND METHODS: Over the last 20 years, 51 children with HBL were treated. Surgery was performed in 48 children (94.1%), conventional liver resection in 38 (of those, 2 received a rescue liver transplantation [LTx] for relapse), and total hepatectomy and primary LTx in 10 patients. The remaining 3 patients received only palliative treatment. Patient data were analyzed for survival with respect to PRETreatment EXTent of disease (PRETEXT), metastases, histopathology, conventional resection, and LTx. RESULTS: Survival of children with HBL treated with liver resection is 71% and 80% for primary LTx. Favorable prognostic factors for patient survival was tumor histology as epithelial-fetal subtype and mixed epithelial and mesenchymal type, without teratoid features, and good response to chemotherapy (necrosis, fibrosis). Unfavorable prognostic factors were small cells undifferentiated, transitional liver cell tumor, α-fetoprotein level above 1,000,000 IU/mL and below 100 IU/mL at diagnosis, lung metastases, and local recurrence after initial resection. Survival was related to PRETEXT stage. However, among patients with PRETEXT III and IV, LTx resulted in better survival. CONCLUSION: Liver transplantation is a good option for children with advanced HBL. Early referral of children with potentially unresectable tumors to centers where combined treatment (chemotherapy, surgery including LTx) is available is crucial.

Complications of umbilical vein catherisation. Case Report.

BACKGROUND: Umbilical vein catheterization is a relatively easy procedure performed routinely on the neonate intensive care units. It provides a fast central vein access, but some complications have been described in the literature. CASE REPORTS: We presented a case report of a premature infant (34 hbd) with extravasation of the parenteral nutrition and drugs to the liver after umbilical vein catheterization. Fever and increasing biochemical markers of infection were observed. USG revealed a heterogenic, well-limited space of 4 cm in diameter, located in the right lobe of the liver. CT excluded liver abscess. Considering neoplastic process or incorrect location of the catheter of the central vein, we performed liver biopsy. RESULTS: Cytological and biochemical analysis of the aspirated fluid revealed extravasation of parenteral nutrition to the liver. Our case confirms the necessity of controlling a proper location of the central catheter right after its insertion and during hospitalization.

[Results of the treatment of paediatric non-rhabdomyosarcoma. One centre experience]. / Wyniki leczenia miesaków tkanek miekkich nie-rhabdomyosarcoma w jednym osrodku.

UNLABELLED: Non-rhabdomyosarcoma soft tissue sarcomas (NR STS) are a rare group of neoplasms of mesenchymal origin. The incidence of these tumours in children is low and due to it's heterogeneity and different response to chemotherapy and radiotherapy, unified treatment methods have not yet been established. THE AIM of our study was to analyze methods and treatment results of patients with NR STS treated in our centre. MATERIALS AND METHODS: Between 1996 and 2004, 64 patients with NR-STS, aged 2.5-21.5 yrs, were treated in our institution. Treatment protocol included primary tumour resection or biopsy, induction (neoadjuvant) chemotherapy, local treatment: surgery and/or radiotherapy and adjuvant chemotherapy. Results of treatment were analyzed in relation to stage, tumour diameter, extent of surgery and response to chemotherapy. RESULTS: Out of 64 patients, 48 are alive (75%), with a median observation time 4 yrs 3 m. Sixteen patients died: 1 of treatment complications, the rest from basic disease. Four years overall (OS) and event free survival (EFS) are 75% and 64% respectively. Early stage, tumour size less than 5 cm in diameter, radical surgery, complete and very good response to induction chemotherapy had a significant influence on survival. CONCLUSIONS: Our results indicate that besides stage and tumour size, radical surgery played key role in the treatment of NRMSSTS and that radical resections were possible to perform after induction chemotherapy in 33% of patients with primarily unresectable tumours. High number of patients with stage IV disease at diagnosis, occurrence of distant relapses and good response to chemotherapy indicate the necessity for the use of chemotherapy in patients with NR STS.

[Focal nodular hyperplasia - own experiences]. / Ogniskowa hiperplazja guzkowa watroby- doswiadczenia wlasne.

INTRODUCTION: focal nodular hyperplasia (FNH) is a benign tumour of the liver, often discovered incidentally. It occurs mainly in women between 25-44 years old. Though it is rarefy reported in children the evidence has been growing in the recent years. THE AIM: of our work was to show our experience of focal nodular hyperplasia in pediatric cases. MATERIAL AND METHODS: 10 children, aged 6-17 years, were hospitalized in the Children's Memorial Health Institute between 2000 and 2005, with provisional diagnosis of focal nodular hyperplasia. In all children radiological examination was performed. In some cases laparotomy and operative liver biopsy was carried out. RESULTS: only in 3 children diagnosis of FNH was made from radiological examination. 6 children needed laparotomy and operative liver biopsy. Although operative resection of FNH lesions was carried out in some patients, the authors observed recurrence of hepatic lesions. CONCLUSIONS: our experiences confirm that nowadays the conservative approach should to be standard procedure in children with FNH. Only in doubtful cases laparotomy is recommended.

The effects of graft geometry on the patency of a systemic-to-pulmonary shunt: a computational fluid dynamics study.

The modified Blalock-Taussig shunt is a palliative operation for some congenital heart diseases. An artificial conduit with antithrombotic surface placed between the subclavian (or innominate) and the pulmonary artery supplies blood to the lungs in defects with decreased pulmonary flow. Clotting of the graft is the main cause of its failure. Stenosis of the arteries is also observed. The objective of the present study was to investigate the flow pattern in the graft to investigate the possibility that clotting is initiated by the stimulation of platelets by high shear stress, and the possible effect of the pathological wall shear stress on the stenosis formation. The model included the left subclavian artery (LSA), the left pulmonary artery (LPA), and the graft. The three-dimensional relative position and size of the arteries was obtained from a CT scan of real anatomy. Four different types of the graft with two different diameters (3 and 4 mm) and two different shapes (straight and curved) of the pipe, and one variable diameter pipe were inserted in the model. A pulsatile flow of 0.81 L/min on average was assumed at the inlet to LSA, and 80% of the flow was directed through the graft. Computer simulations demonstrated a complex flow pattern with eddies and low velocity regions in the arteries at the anastomoses with the graft in all five models. An eddy was also found inside the straight 4 mm graft. A high pathological shear rate was present within the graft, with higher values in the 3 mm grafts. The fractional volume with a high (>2500 L/s) shear rate was between 2.5% and 4.5%, and that with a very high (>7500 L/s) shear rate between 0 and 1.5% of the model volume, and depended on the graft geometry and the phase of the cardiac cycle. Pathologically high (>3.5 Pa) and pathologically low (<1.0 Pa) wall shear stress, which may induce neointimal growth, was found in LSA and LPA. We conclude that the activation of platelets by high shear rate is possible within the graft, followed by their subsequent aggregation in the eddies with a low flow rate. Flow-induced changes of the vessel wall thickness (stenosis) can also appear, especially in the pulmonary artery.

[Kawasaki disease in children--9 years experience]. / Choroba Kawasaki u dzieci--9 letnie doswiadczenie jednego osrodka.

PURPOSE: The aim of the study was assessment of the course and treatment of Kawasaki disease on the basis of own experience. MATERIAL AND METHODS: Between November 1995 and December 2004 Kawasaki disease (KD) was diagnosed in 30 patients, (20 boys, 10 girls). The median age in acute stage was 20 months (range 1-96 months). Patients were divided into 4 groups (gr) according to changes in coronary arteries (CA). Group 1 (N=13) without CA aneurysms (CAA), group II (N=5) with small (<5mm) CAA. Group III (N=5) with medium (5-8mm) CAA, group IV (N=7) with giant (>8mm) CAA. Echocardiographic examination was performed in all patients. In echocardiographic examination the following were observed: CAA and valvar insufficiency in 15 patients, left ventricle enlargement in 3 patients, pericardial effusion in 6 patients. The treatment was intravenous immunoglobulin and aspirin in all patients, steroid treatment in 2 patients methylprednisolone, in 2 patients prednisone, in l patient hydrocortisone. The anti-inflammatory treatment was started after 10 days of illness in 66.7% of patients. Thrombi in CAA were treated in 6 patients, the treatment was: in all tissue - type plasminogen activator iv and intracoronary in 2 patients; heparin in all; enoxaparine in 3 patients; abciximab in 2 patients; acenocumarol and aspirin in all. Coronary angiographies were performed in 2 patients in acute stage of disease for thrombolytic therapy and in 4 patients during follow-up. Myocardial perfusion was assessed in SPECT (Single Photon Emission Computed Tomography) in 6 patients. Two children with giant CAA died during the first 8 months of acute stage (6.7%), 2 patients we lost from follow-up. 26 patients are in follow-up. RESULTS: during anti-inflammatory treatment (immunoglobulin, aspirin, steroid treatment) loss of fever, normalization of inflammatory markers, and no side effect, were observed except for 1 patient treated with methylprednosolone with thrombi in CAA during treatment and no side effects during treatment. During the follow-up period CAA regressed in 5 patients (35.7%), CAA became smaller in 7 patients (50%), CAA were the same in 2 patients (14.3%). Changes in echocardiographic examination: valvar insufficiency, left ventricle enlargement, pericardial effusion, disappeared during follow-up. Perfusion defects were observed in 4 patients in SPECT. In coronary angiography changes were observed in coronary arteries in 4 patients. CONCLUSIONS: 1. Kawasaki disease was diagnosed in 66.7% children after 10 days of illness. 2. Coronary artery aneurysms were found in 56.6% treated children. 3. Regression of CAA was observed in 37.5% patients mainly with small CAA. They became smaller in 50% cases mainly with medium and giant CAA. 4. Mortality rate was 6.7% and concerned children with giant CAA; they died during the first 8 months of illness. 5. Thrombolytic treatment of thrombi in CAA (rt-PA, heparin, enoxaparine, abciximab, acenocumarol, aspirin) was safe and successful. 6. In all patients with medium and giant CAA in the acute stage, changes were observed in echocardiographic, angiographic examination and myocardial perfusion during follow-up.

Use of covered Cheatham-Platinum stents in aortic coarctation and recoarctation.

We inserted covered Cheatham-Platinum stents in 4 patients, ranging in age from 12 to 19 years, who weighed between 45 and 94 kg. All the patients had aortic coarctation, with surgical repair having been attempted previously in one, and with balloon dilation having been performed as the primary treatment in two, resulting in formation of aneurysms. The fourth patient had not received any treatment. The gradients were reduced from 10 to 40 mmHg before insertion of the stent to 0 to 5 mmHg after stenting. No complications were encountered. All the patients are well at an interval of 3 to 14 months after stenting.

Arterial hypertension with brachydactyly in a 15-year-old boy.

Autosomal dominant brachydactyly with hypertension is the only form of monogenic hypertension which is not sodium dependent. The disease is characterized by brachydactyly type E, short stature, arterial hypertension and aberrant loop of posterior inferior cerebellar artery (PICA) causing neurovascular conflict. So far the syndrome was described in one family in Turkey and two in Canada. We report a case of a 15-year-old boy who was admitted because of arterial hypertension 160/100 mmHg. He complained also of attacks of bilateral numbness of hands with deterioration of visual field. Examination revealed short stature (156 cm) and bone deformities of hands and feet consistent with brachydactyly type E. Ophthalmoscopy showed mild narrowing of retinal arteries. Serum electrolytes, blood gases, and renal function were normal. Renin activity and aldosterone concentrations were raised, and 24-h urinary excretion of catecholamines and urinary steroid profile were in normal range. Renal Doppler ultrasound was normal, but renal scintigraphy suggested vascular changes in the left kidney. Echocardiographic examination, besides mild left ventricular hypertrophy, was normal. Magnetic resonance angiography (angio-MR) revealed bilateral abnormal PICA loops and neurovascular conflict. Spiral angiotomography of renal arteries revealed narrow additional left renal artery. Both nonconsanguineous parents and younger brother were healthy, with normal height, without bone deformities, and had normal intracranial vessels. Amlodipine and metoprolol were given, and blood pressure lowered to 143/87. Adding rilmenidine gave no effect and enalapril was then added. It led to further improvement in blood pressure control. To our knowledge, this is the first pediatric description of a sporadic form of autosomal dominant brachydactyly with hypertension with abnormalities of brain and renal arteries.

Transitional liver cell tumors (TLCT) in older children and adolescents: a novel group of aggressive hepatic tumors expressing beta-catenin.

BACKGROUND: We encountered on seven malignant hepatocellular tumors developing in older children and adolescents. RESULTS: These tumors exhibit an unusual phenotype with respect to clinical presentation, histopathology, immunohistochemistry, and treatment response. As a working hypothesis, we suggest that these apparently novel, unusual, and aggressive tumors occurring in older children and adolescents may form a transition in the putative developmental pathway of hepatocarcinogenesis. CONCLUSION: We therefore propose the term, transitional liver cell tumors (TLCT), to denote these lesions.

Hemangioma of the liver in children: proliferating vascular tumor or congenital vascular malformation?

BACKGROUND: Hepatic vascular tumors (HVT) are the most common benign liver tumors present in infancy and childhood commonly associated with high output cardiac failure. Pediatric HVT usually are divided into infantile hemangioendothelioma (IHE), cavernous hemangioma (HC), and arteriovenous malformations (AVM). The aim of this study was to analyze clinical and histologic features of pediatric HVT in relation to treatment strategies. PROCEDURE: During last 12 years, 17 children have been treated because of HVT. The diagnosis of HVT was established in all on the basis of clinical and imaging data. A retrospective analysis of clinical records and histopathology divided 17 into: Group 1, neonates; and Group 2, infants and older children. RESULTS: Radiologic imaging revealed the vascular nature of the tumors in all patients. All nine from Group 1 were symptomatic from AVMs and seven were operated upon. Only one of eight children from Group 2 presented symptoms of AVM requiring surgery; four of five in this group had surgery because of the risk of malignancy. Within Group 1, a mixture of proliferating IHE with microscopic features of AVM was found in most. In three neonates with HVT immunologic and clinical features of cytomegalovirus (CMV) hepatitis were noted. In two Group 2 patients, pure HC was present and in another, the diagnosis of angiosarcoma was established after biopsy of a peritoneal metastasis. CONCLUSIONS: HVT in children demonstrate internal morphologic heterogeneity and an age-related behavior of the disease. We also confirm the proliferative nature of all hemangioendotheliomas (HEs) in children. Further studies on the tumorigenesis of these lesions are needed.