[Show abstract][Hide abstract] ABSTRACT: Pancreaticopleural fistula (PPF), a form of internal pancreatic fistula, is a rare complication of acute or chronic pancreatitis or pancreatic trauma.
We report two cases of PPF resulting in formation of pleural pancreatic pseudocysts. A 35-year-old male alcoholic patient with a history of recurrent episodes of acute pancreatitis was admitted due to a severe dyspnea. A CT scan showed a significant left pleural effusion with a total left lung atelectasis, compression of the mediastinum, and dislocation of the left diaphragm. A follow-up CT showed a fistula between the abdominal pancreatic pseudocyst and the left pleural cavity. The second case was a 13-year-old male patient, who was admitted for a splenic stump excision. Two weeks after the surgery the patient presented a massive pleural amylase-rich effusion. CT exam suggested a PPF, which was indirectly confirmed by a thoracoscopy.
PPF should be considered in cases of massive pleural effusion and encapsulated pleural fluid collections in patients with a history of acute pancreatitis and surgery involving pancreas.
[Show abstract][Hide abstract] ABSTRACT: In this study, H. pylori-infected and noninfected children with gastritis were compared to a control group with respect to circulating CD4(+) and CD8(+) T lymphocytes expressing activation and differentiation markers. Additionally, the lymphocyte phenotypes of children with gastritis were correlated with the gastric inflammation scores.
H. pylori infection status was assessed based on [¹³C]urea breath test, rapid urease test, and histology. Analysis of the lymphocyte surface molecule expression was carried out by triple-color flow cytometry.
The group of H. pylori-infected children showed an elevated proportion of peripheral B cells with CD19(low) , along with a twofold increase in the percentage of memory (CD45RO(+)) CD4(+) and CD8(+) T-cell subsets (p < .05). Moreover, a positive correlation between the age and the percentage of these subsets was seen (r = .38, p = .04 and r = .56, p < .01, respectively). Children with gastritis but without infection had a slightly increased percentage of CD8(+) T cells and CD56(+) NK cells, CD3(high) T cells and CD45RO(high) CD4(+) T-cell subsets (p < .05). Both H. pylori-infected and noninfected children with gastritis were characterized by an increased percentage of memory/effector CD4(+) T cells, the presence of NK cells with CD56(high), memory T-cell subset with CD4(high), and naive, memory, memory/effector, and effector T-cell subsets with CD8(high) (p < .05). Gastric inflammation scores correlated positively with the percentage of CD4(+) T lymphocytes in H. pylori-infected children (r = .42, p = .03). In noninfected children, gastric inflammation scores correlated positively with the percentage of B cells (r = .45, p = .04).
In H. pylori-negative children, gastritis was associated with an increased percentage of activated NK and T cells, and intermediate-differentiated peripheral blood CD4(+) T cells, which was more pronounced in H. pylori-positive children who also showed an increased B-cell response. However, increased inflammation was only associated with the elevation of CD4(+) T-cell percentage in H. pylori-positive children as well as B-cell percentage in H. pylori-negative children with gastritis.
[Show abstract][Hide abstract] ABSTRACT: Subcutaneous fat necrosis of the newborn is a rare, benign, self-limited hypodermatitis that affects full-term or postmature newborn with history of perinatal stress such as hypoxia, hypothermia or local pressure trauma. The main lesions appear as subucateneous nodules in adipose tissue. We present a case of a three-week old newborn born in spontaneous delivery in the 40Hbd in poor general condition (inborn pneumonia and perinatal hypoxia) which was admitted to the hospital due to subcutaneous tissue induration and skin discoloration above the lesion. The skin ultrasound examination showed foci of homogeneously hyperechogenic subcutaneous tissue thickened up to 6 mm. Further examinations did not reveal any abnormalities besides the slightly increased calcium concentration (1,69 mmol/l with normal range of 1,2 mmo/l). In order to diagnose the condition a tissue sample was collected. The histological examination revealed panniculitis with infiltration with mononuclear cells, e.g. macrophages as well as multinucleated giant cells with radial arrangement of needles-shaped clefts in cytoplasm. After treatment with corticosteroids resolution of all the lesions was noted.
Polish journal of pathology: official journal of the Polish Society of Pathologists 01/2010; 61(4):240-4. · 1.13 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Conjoined twins represent a rare case of embryonic failure. Siamese twins' final outcome is usually associated with poor prognosis due to complications, among which the inflammatory and septic disturbances are often present.
The article describes Siamese twins of craniopagus type. One of the twins died of heart and aortic failure after 48 hours of life. The decision regarding surgical separation then became an emergency. At the same time laboratory results for the surviving twin I indicated steadily worsening coagulation functions and processes, which we believed were caused by the death of twin II, as well as by systemic inflammatory response syndrome (SIRS).
Due to vascular conjunction between the twins' brains' circulatory systems the sequenced progress of coagulopathy was noticed in the surviving twin.
Medical science monitor: international medical journal of experimental and clinical research 01/2010; 16(1):CS1-5. · 1.43 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: One of the ways of diminishing risk of massive blood loss during liver surgery is the temporal vascular exclusion of the liver/Pringle maneuver. Scanty publications on the problem of liver resections in children are based on very limited series of patients and did not define save periods of time of temporal vascular exclusion of the liver during operation in this group of patients. They also did not specify the effects arising from prolonged acute hepatocytes ischemia in children. The aim of the study was the assessment of the influence of length of time of Pringle maneuver on the liver cells function in the perioperative period and the assessment of its influence on the postoperative course of treatment.
Patients according to the duration of Pringle maneuver were divided into 2 groups. Group I consisted of 18 patients, in whom the duration of Pringle maneuver was below 32 minutes. Group II consisted of 17 patients, in whom the duration of Pringle maneuver was between 32 and 60 minutes. Selection of the periods of duration of Pringle maneuver was dictated by mean and mediana value of its lasting. In patients from both groups values of the level of AspAT, ALAT LDH and INR were assessed in postoperative day 1, 2 and 3 as well as time of mechanical ventilation, length of stay in ICU, and the duration of hospitalization.
In group II of children operated with Pringle maneuver lasted between 32 and 60 minutes, statistically significant longer mean value of time of operation was indicated, as well as statistically significant higher mean value of biochemical parameters describing liver cells function, slow down dynamic of normalization of INR in the first 3 postoperative days and statistically significant higher mean value of length of time of hospitalization.
Extension of time of Pringle maneuver in children from 30 to 60 minutes may be connected with transient postoperative biochemical dysfunction of hepatocytes characterized by slow down dynamic of normalization of the level of liver enzymes as well as may results in extension of time of hospitalization connected with necessity of normalization of transient postoperative biochemical dysfunction of hepatocytes.
Polski merkuriusz lekarski: organ Polskiego Towarzystwa Lekarskiego 07/2009; 26(156):616-9.
[Show abstract][Hide abstract] ABSTRACT: Pediatric hemangiopericytoma (HPC) is an extremely rare vascular tumor with little data available on its clinical course and management.
Fourteen children with HPC registered in Polish Pediatric Rare Tumors and Polish Pediatric Soft-tissue Sarcomas Studies between 1992 and 2002 are reported.
Seven patients (F/M: 5/2, age 2-10 months) had infantile HPC, four of whom had primary tumors affecting superficial tissues of the trunk and upper limbs. No child had initial nodal or organ metastases. Primary excision (PE) was performed only in three patients with superficial lesions. They received no supplemental treatment. The remaining four children responded well to chemotherapy (CHT), entering complete remission after CHT alone (n= 2) or after delayed resection (n= 2). No relapses occurred and all patients were alive 48-146 months after treatment. Seven patients (F/M: 2/5, age 3.2-16.5 years) had adult-type HPC, five of whom had tumors localized in superficial tissues of the lower limbs or head/neck. All patients presented with locally advanced disease. PE was performed in five children (complete in four); all patients were alive at follow up of 40-127 months. Three patients died of recurrence after incomplete PE despite supplemental chemo- and radiotherapy.
Complete surgical excision remains the mainstay of treatment for both HPC types. In unresectable adult-type HPC adjuvant chemo- and radiotherapy should be administered in macro- and microscopic tumor residues, but the prognosis is poor despite supplemental treatment. High chemo-responsiveness of infantile-type HPC produces a favorable outcome even in cases of unresectable, life-threatening tumors.
Pediatrics International 03/2009; 51(1):19-24. DOI:10.1111/j.1442-200X.2008.02668.x · 0.73 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Partial vascular exclusion (Pringle maneuver) is one of the methods diminishing risk of massive intraoperative blood loss. Effects of Pringle maneuver were deeply explored in adults due to big series of operated patients but experiences with Pringle maneuver in children are very limited. The aim of the study was to estimate usefulness of Pringle maneuver in the limitation of intraoperative blood loss and the amount of intraoperative fluid therapy necessary during liver resections in children.
The group of 70 children operated because of liver tumors during the period of 20 years were studied. Patients were divided into 2 subgroups. Group I consisted of 35 patients without Pringle maneuver during liver resection. Group II was consisted of 35 children operated with Pringle maneuver. In all patients intraoperative diuresis and hemodynamic parameters of blood circulation were monitored and the blood loss and fluid therapy were assessed.
In group I operated on without Pringle maneuver statistically significant higher amount of blood transfusions were indicated and in group II statistically significant higher amount of colloids were indicated.
The results achieved documented that introduction of Pringle maneuver during liver resection in children allow to achieve statistically significant limitation of blood loss during operation but hemodynamic disorders during partial vascular exclusion (Pringle maneuver) requires transfusion of higher amounts of volume colloid fluids.
Polski merkuriusz lekarski: organ Polskiego Towarzystwa Lekarskiego 08/2008; 25(145):23-6.
[Show abstract][Hide abstract] ABSTRACT: Pathology diagnosis of chronic graft-versus-host-disease (GVHD) after allogeneic haematopoietic stem cell transplantation (allo-HSCT) is an important issue in clinical follow-up, in spite of frequent difficulties in interpretation., related to dynamic changes occurring in the skin during the disease, as well as to sequelae of basic disease and immunosuppressive therapy. Recently presented Consensus NIH (National Health Institute, Bethesda, USA) of histopathologic (HP) analysis is still complex and intrinsically divergent, thus clinically difficult to implement.
Analysis of clinical value of histological evaluation results of skin biopsy in children after allo-HSCT and its correlation with clinical status.
Ten skin biopsies were taken from 7 patients (4 boys, 3 girls, age 3-15 years) after allo-HSCT (6 MFD, 1 MMUD) and analyzed after hematoxylin/eosine and immunohistochemical (CD3, CD45T, CD20) staining. Pathology analysis was based on commonly accepted criteria enabling simple and unambiguous interpretation. Results were compared with clinical data and indications for immunosuppressive therapy.
It was found that reliable and coherent interpretation can be made when following parameters were taken into account: 1. in epithelium: the presence of apoptosis, archetypical changes and vacuolar degeneration in the basilar layer, presence of CD3/CD45 in the epidermis; 2. in the dermis: the extent of collagenization, presence of melanophages and lymphocyte infiltrations; 3. in the eccrine glands epithelium: eccrine glands atrophy and presence of lymphocytes.
A new scoring system of skin biopsy analysis in patients with chronic GVHD based on the modified NIH Consensus was proposed. The preliminary clinical value of histological results was assessed. Skin biopsy evaluation based on limited qualitative and quantitative analysis of lymphocyte infiltrates together with studies on intensity of apoptosis, collagenization and archetypical changes is a valuable diagnostic method complementary to clinical records, enabling easier undertaking of therapeutic decisions.
Medycyna wieku rozwojowego 02/2008; 12(4 Pt 2):1105-11.
[Show abstract][Hide abstract] ABSTRACT: We aimed to investigate the occurrence and types of pathogenic mutations in the RET gene in patients with MTC of the Central Poland population and in their relatives. DNA was extracted from the peripheral blood lymphocytes of a total of 330 persons, including 235 MTC patients and 95 of their unaffected kindred's. Exons 10, 11, 13, 14, 15 and 16 of the RET gene were amplified by PCR and sequenced. Sixty-seven people were found to carry pathogenic, germline mutations in the RET gene. In exon 10, C609F, C609R and C609Y (3 families), C618G, C618F (2 families), and C620G (4 families) mutations were identified. In exon 11, C634R (8 families) and C649L mutations (1 patient) were found. Five families carried Y791F mutation in exon 13. One patient with PTC revealed the presence of a Y791F mutation. In 3 families, exon 14 of the RET gene harbored the following mutations: V804L (1 patient), E819K (1 patient) and R844Q (1 patient). In 1 family, the S891A mutation was identified in exon 15, 3 families were found to carry mutations in exon16, R912P in 1 family and M918T in 2 families. In summary, of the 235 patients affected by MTC, 46 (19.6%) carried pathogenic RET gene mutations, 1 patient with RET mutation had kidney carcinoma, and 1 had PTC. The results show the occurrence of a variety of mutations prevalent in patients with MTC in the population of Central Poland. These results may contribute to a better diagnosis of medullary thyroid carcinoma.
Cancer Investigation 01/2008; 25(8):742-9. DOI:10.1080/07357900701518735 · 2.22 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: We sought to assess whether extensive surgical treatment, postsurgical radioiodine therapy, or both decrease the risk of locoregional recurrence (LR) after curative primary treatment in children and adolescents diagnosed with differentiated thyroid cancer (DTC) at age <or=18 y.
To determine the incidence of and identify predictive factors for thyroid bed recurrence (TBR) or lymph node recurrence (NR), we performed a chart review and retrospective multivariate Cox regression analysis on 235 patients with DTC diagnosed at age <or=18 y and managed with curative intent at our tertiary referral center from 1973 to 2002; 40 of these patients had distant metastases at diagnosis. We also determined overall and recurrence-free survival and generated curves for these variables using Kaplan-Meier and Cox univariate analysis.
During a median follow-up of 82 mo (range, 5-402 mo), no DTC-related deaths occurred, 203 (86%) children remained recurrence-free, and 32 (14%) children had LR, including TBR in 9 (28% of LR), NR in 20 (63% of LR), and both in 3 (9% of LR). Among patients treated with radical intent and showing no distant metastases, the most recent thyroglobulin level was <1 ng/mL in all but 4% of cases. The median time from the first surgery to LR was 37 mo (range, 9-280 mo). In multivariate analysis, significant risk factors for TBR were less than total thyroidectomy and lack of postsurgical radioiodine treatment (respective risk increases of 9.5 [P = 0.04] and 11 times [P = 0.03]). For NR, classic papillary histology, incomplete primary lymph node management (i.e., lack of modified lymphadenectomy of affected lymph nodes or lack of confirmation of disease-free nodes by intraoperative staging), and absence of adjuvant radioiodine therapy were independent significant predictive factors that increased the recurrence risk by 1.9 (P = 0.02), 3.3 (P = 0.02), and 3.2 (P = 0.02) times, respectively. Age or sex did not correlate with LR risk.
In DTC patients <or=18 y of age, extensive initial therapy-consisting of total thyroidectomy combined with modified lymphadenectomy performed in case of lymph node metastases and followed by radioiodine therapy--is associated with a substantial decrease of DTC LR risk.
Journal of Nuclear Medicine 06/2007; 48(6):879-88. DOI:10.2967/jnumed.106.035535 · 6.16 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Thyroglossal duct remnants (TGDR), most often cysts, are the most common type of developmental abnormalities of the thyroid gland. In about 1 to 2% of TGDR neoplastic transformation occurs. Papillary carcinoma of the thyroid may be encountered in over 90% of such cases. Two cases of primary papillary carcinoma of the thyroid in TGDR in young girls are presented. The diagnostic and therapeutic problems are shared, and up-to-date management guidelines in similar cases are discussed.
Endokrynologia Polska 01/2007; 58(2):164-7. · 0.99 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Differentiated thyroid carcinoma (DTC) in children presents different biological behavior in comparison to adults. Authors presents preliminary results of multicenter analysis concerning incidence, diagnostics and treatment of DTC in children.
The study is a retrospective analysis of 107 pediatric patients from 14 academic centers based on the data from 2000 to 2005 obtained by questionnaire in hospitals involved in the treatment of DTC in children.
Papillary thyroid cancer was diagnosed in 83 children, follicular thyroid cancer in 10 children and medullary thyroid cancer in 14 children. Incidence of DTC in children was estimated between 18 and 23 cases per year. The biggest group of patients consisted of children between 11 and 15 years of age, with girls to boys ratio 3.3 : 1. Clinically DTC in children presented most often as solitary thyroid nodule. Cervical lymphadenopathy was observed in 42% of patients. Intraoperative verification indicated metastatic nodes in 50% of children. Low stage DTC predominated (T1 in 36% and T2 in 26% of children). One step surgery was performed in 65% of children with DTC, two step surgery in 25% of patients. I131 therapy was undertaken in 80% of children. Lung metastases were indicated in post therapeutic studies in 14% of children with DTC. Prophylactic thyroidectomies were performed in 79% of children in the group of patients with MTC and RET gene mutations.
The necessity of introduction of unified therapeutic standard in children with DTC in Poland is underlined.
Endokrynologia Polska 02/2006; 57 Suppl A:75-81. · 0.99 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: The optimal surgical treatment of children with differentiated thyroid cancer remains an important point of discussion. Especially the need for completion operation is questioned in young patients. Our objective was to examine the rate of residual neoplastic disease after non radical initial operation.
From the 235 children diagnosed with differentiated thyroid cancer, 131 (56%) needed completion operation due to incomplete primary surgery. Completion operation involved thyroid bed, lymph nodes or both respectively in 91 (39%), 13 (6%) and 27 (11%) cases. Risk factors responsible for residual disease were evaluated by means of logistic regression analysis.
Residual disease was detected in 46 (35%) of reoperated children (25% in thyroid bed and 85% in lymph node of lateral neck compartment). Sex and age did not influence the risk of residual disease in thyroid bed or lymph nodes. Papillary type of cancer and multifocality increased risk of residual disease in thyroid bed respectively by the factor of 15 (95% CI: 2-125) and 2.3 (95% CI: 1.2-4.4). Infiltration of thyroid capsule did not correlate with the risk of residual disease. Lymph node metastases in primary operation increased risk of residual disease by the factor of 16 (95% CI: 1.2-245). Histopathology, multifocality of primary tumour or infiltration of lymph node capsule did not influence the risk of residual disease in lymph nodes of lateral neck compartment.
In children with differentiated thyroid cancer residual disease is diagnosed in about 1/3 of non radically operated cases. This high incidence justifies completion operations. The risk of residual disease is significantly increased in papillary thyroid cancer, multifocal tumours and cases with lymph node metastases.
Endokrynologia Polska 01/2006; 57(4):356-61. · 0.99 Impact Factor
[Show abstract][Hide abstract] ABSTRACT: Nephroblastoma is the most common kidney tumor in Polish children.
to present clinical material and outcome of 533 children with renal tumors.
500 pts with nephroblastoma and 33 of non-Wilms: CMN, RCC,CSSK, RTK and others tumors were registered, mean age 4.5 years between 1993 till 2002. Stage: CS I--148, CS II--191, CS III--114, CS IV--51, CS V--29 pts. All pts with nephroblastoma were treated according to the first national PPGGL 01-92 protocol with pre-operative chemotherapy (ACT, VCR) for CS I-III and ACT, VCR, DOX in pts of stage IV, over the age of 6 months. Pre-operative chemotherapy was done to 93.8% pts.
Radical nephrectomy post pre op chemotherapy was performed in 451 (98%) pts over 6 months and in 44 (8.2%) infants less than 6 months with nephroblastoma. Partial nephrectomy for unilateral tumor post preoperative chemotherapy was made in 6 (1.2%). In 26/29 (89.65%) of CS V nephroblastoma kidney sparing surgery was possible, and in 12 uni-lateral nephrectomy was performed. Surgical complications were mild and occurred only in 8.9% pts.
5-years overall survival of CS I pts (favorable and standard histology) is 93.48%, CS II--96.8%, CS III--84.4%, CS IV--67%, CS V--58%. The results of treatment of 33 pts with non-Wilms renal tumors have improved lately. 78.7% of our pts achieved 5-years overall survival.
The use of systemic neoadjuvant chemotherapy in all pts over 6 months according to the recommendation of SIOP Nephroblastoma protocol (01-92) produced tumor shrinkage, facilitated complete surgical nephrectomy, and was very advantageous in the treatment of renal tumors in children. The results of treatment of non-Wilms tumor have also improved thanks to introduction of new and more aggressive regimens of chemotherapy.
[Show abstract][Hide abstract] ABSTRACT: We encountered on seven malignant hepatocellular tumors developing in older children and adolescents.
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.
We therefore propose the term, transitional liver cell tumors (TLCT), to denote these lesions.
Medical and Pediatric Oncology 12/2002; 39(5):510-8. DOI:10.1002/mpo.10177
[Show abstract][Hide abstract] ABSTRACT: 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.
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.
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.
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.
Medical and Pediatric Oncology 12/2002; 39(5):524-9. DOI:10.1002/mpo.10179
[Show abstract][Hide abstract] ABSTRACT: The aim of the study was to assess the incidence, causes, diagnostic and treatment modalities, and outcome of vascular thrombosis after kidney transplantation in children. Between 1984 and 1995 we performed 176 kidney transplants in pediatric recipients aged 1 to 18 years. Vascular thrombosis followed 7 transplants, 4 were renal vein and 3 arterial thromboses. Venous thromboses occurred 2 to 12 days after transplantation. All of the patients with a renal vein thrombosis lost their grafts. Arterial thrombosis developed in 2 cases of double renal arteries which were separately anastomosed into the recipient vessels. One graft was lost, but the other was saved by thrombolytic therapy (streptokinase). One child experienced intrarenal segmental artery thrombosis during acute vascular rejection, which resolved following combined anti-rejection and thrombolytic (intra-arterial streptokinase) treatment with full recovery of graft function. In all, vascular thrombosis complicated 7 out of 176 transplants (4.0%), and was the cause of 5 graft losses (2.8%). The incidence of vascular thrombosis was not increased in grafts with vascular anomalies (3/34 v. 4/142; p>0.05, chi sq.). We conclude that acute tubular necrosis, rejection and unstable volemia may predispose to vascular thrombosis. In selected cases, early diagnosis of vascular thrombosis may enable graft salvage by surgical or thrombolytic treatment.