Recurrent or persistent salivary gland enlargement in children: When is it Sjögren's?

OBJECTIVES: To describe characteristic features in children with recurrent or persistent salivary gland enlargement and to propose a diagnostic algorithm with specific consideration for Sjögren's disease (SD). METHODS: In this single-center, prospective study, 45 patients < 18 years, with recurrent or persistent salivary gland enlargement of unknown etiology were enrolled from 2006 to 2019. We collected detailed clinical information to characterize this group of patients including specific details of their major salivary gland signs and symptoms. We compared clinical, laboratory and radiological parameters between 4 groups based on the results of labial salivary gland biopsy (LSGB) and between patients who met existing SD criteria or not. RESULTS: 44 patients, with a mean age of 6.8 years and female to male ratio 21:23 were observed over a mean of 3.8 years. Characteristics of salivary gland swelling episodes varied considerably between individuals, but the majority experienced ≤5 episodes per year, lasting ≤ 1 week, with swelling affecting either or both glands. Ocular and oral dryness symptoms were observed only in 25% and 59% patients, respectively. The majority were positive for ANA, but negative for SD-specific antibodies. A total of 75% patients fulfilled at least one of the existing SD criteria. CONCLUSION: SD is a major cause of recurrent salivary gland enlargement in children. For children meeting adult criteria, the diagnosis of SD is clear. However, for the many children without dryness symptoms, objective dryness, or SD-specific antibodies, further workup including a combination of salivary gland imaging and histopathological examination can help establish the diagnosis of SD.

[Malignant germ cell tumours. Multicenter prospective trial in Polish Pediatric Group for Solid Tumours (years 1998-2000)]. / Zlosliwe guzy germinalne u dzieci. Wieloosrodkowe badania prospektywne Polskiej Pediatrycznej Grupy Guzów Litych w latach 1998-2002.

UNLABELLED: Germ cell tumors constitute about 3% of all pediatric malignancies. Since 1998 the multicenter trial was initiated in Poland. MATERIAL AND METHODS: 95 children (aged from 1 month to 17 years--mean 9.2 years) were registered. There were 38 boys and 57 girls. Diagnosis was made on histopathological examination in 88% patients (pts) and in 12% was established on imaging and biochemical findings (elevated AFP). Mixed germ cell tumor and yolk sac tumor prevelaged. AFP was elevated in 72% pts; in 26% it was over 15.000. Primary tumor was localized in gonads (59%) and in sacrococcygeal region (30%). Following disease stages were identified: I and II--41% pts, III--34%, IV--25%. All patients were treated according to French TGM'95 protocol. 43 belonged to high risk and 52 to standard risk group. 77 children completed therapy, 15 continue treatment and 3 were lost from follow-up. RESULTS: Among children who were off therapy, 70 (91%) are alive in a complete remission (second remission in 3 cases). Survival in high risk group is 89%, while in standard risk group is 93%. Median time of follow-up is 31 months from the beginning of treatment and 25 months after completion of therapy. 7 children died; all had progressive disease. CONCLUSION: The outcome of malignant germ cell tumors treatment in Poland is favourable and comparable to results showed by other study groups in the world.

[Coexistence of Crohn disease and Wegener granulomatosis in a 15-year-old patient]. / Wspolwystepowanie choroby lesniowskiego-Crohna z Ziarniniakiem Wegenera U 15-letniej pacjentki.

Crohn disease is being diagnosed more and more frequently in children and teenagers. Clinical symptoms are mainly related to the gastrointestinal tract, however there are many reports in the literature about the coexistence of Crohn disease with other autoimmunological disorders such as celiac disease, autoimmune hypothyroidism, systemic lupus erythematosus and Wegener granulomatosis. We report a 15-year-old patient with Crohn disease who also developed Wegener granulomatosis. The presented case illustrates the difficulties in establishing the diagnosis when symptoms of the original disease are superimposed on symptoms of a different disorder.

[Analysis of risk factor treatment failures in therapeutic programme for malignant germ cell tumours in children. Multicentre prospective study of Polish Pediatric Group for Solid Tumours 1998--2006]. / Analiza czynników ryzyka niepowodzenia terapii w programie leczenia dzieci ze zlosliwymi guzami terminalnymi. Wieloosrodkowe badania prospektywne Polskiej Pediatrycznej Grupy Guzów Litych (PPGGL) w Latach 1998--2006.

AIMS: The aim of the study was the analysis of risk factors of therapeutic failures in children with malignant germ cell tumours treated within the multicentre programme of PPGGL from 1999--2006. MATERIALS AND METHODS: The investigated group included 18 (14.3%) patients, of 123 who have finished the treatment of malignant germ cell tumour, in whom no remission was obtained or relapse occurred. All the patients were treated according to the TGM 95 programme. Both clinical and morphological data of the group have been analysed. RESULTS: Among 18 patients with therapeutic failures 12 died. Two patients from the high risk group died of complications of the treatment--sepsis during neutropenia after chemotherapy and one after haemorrhage to the central nervous system. The other 9 died from progression of malignancy, 6 of them belonged to the high risk group. 10 (82%) of 12 patients who died had extragonadal location and in 11 (92%) the tumour was in stage III or IV of the disease. The most frequent histology in this group was mixed germ cell tumour with component of yolk sac tumour or carcinoma embrionale. 92% patients had elevated AFP, in 4 it was above 15000 ng/ml. In 11 (92%) patients primary chemoresistance was observed, and radical surgery was not possible for the reason of advanced stage of the disease. In 6 patients relapse occurred. In 3 patients testis was the primary location (I and II stage), in 3 patients the tumour was localized in the sacrococcygeal region (III and IV stage). All the patients are alive in remission after second line therapy, with 78 months (median) of follow-up. CONCLUSIONS: 1. The main risk factor for therapeutic failures in malignant germ cell tumours was primary chemoresistance in inoperable tumours of the sacrococcygeal region. 2. The mortality of treatment complications was low. 3. The relapse of cancer was not a risk factor for therapeutic failure due to the high probability of second remission 4. Therapeutic failures are mainly observed in patients with mixed germ cell tumour with components of yolk sac tumour or carcinoma embrionale. 5. Tumour chemoresistance should be considered an essential factor in identifying high risk patients.

[Pulmonary metastases in children with solid tumours--own experiences]. / Przerzuty do pluc w guzach litych u dzieci--obserwacje wlasne.

BACKGROUND: The most frequent reasons of relapses in solid tumours among children are lung metastases. AIM: Analysis of lung metastatic cases among children with solid tumours treated from 1995-2005. MATERIAL AND METHODS: 26 lung metastatic cases (17 males, 9 females) were analysed. At the moment of the diagnosis lung metastases were present in 19.2% of patients while in the rest (80.8%) occurred during and after treatment. The most often lung metastases were recognised in osteosarcoma (15-57.8%) and carcinoma embryonale (3-11.6%). Secondary metastases in lungs occurred within 4-48 months after the diagnosis. In 57.7% were bilateral. 36 thoracotomies (average 1.7/ a child) were performed. The after-surgery chemotherapy for tumour recurrence was introduced in each case. RESULTS: In the analysed group 14 (53.8%) children are alive with the overall survival time 8-120 months. The rest 12 (46.2%) are dead with the survival time 6-24 months. The statistically significant difference was found in comparison of complete surgery versus incomplete (p=0.02), no significance was found in primary or secondary metastases (p=0.27). Time of occurrence was statistically insignificant (p=0.26). CONCLUSIONS: The occurrence of metastases in children solid tumours worsened the prognosis. The active search for lung metastases at the moment of diagnosis, treatment and follow-up combined with complete surgery procedures may prolong survival. There is a need to find new methods of lung metastases treatment.

[Assessment of angiogenesis in children' s osteosarcoma]. / Ocena stopnia angiogenezy w miesaku kosciopochodnym u dzieci.

INTRODUCTION: Tumour angiogenesis is one of the most important hallmarks of cancer, which enables its development, progression and metastasizing. The extent of angiogenesis seems to be an essential prognostic factor in many solid tumours of children and adults. There have also been reports on the significance of angiogenesis in osteosarcoma. The most common methods to estimate angiogenesis is assessment of microvessel density (MVD) and vascular endothelial growth factor (VEGF) expression. AIM: The aim of the study was assessment of angiogenesis in osteosarcoma patients treated in our centre on the basis of MVD and VEGF expression. PATIENTS AND METHODS: Histopathological specimens of 16 patients with osteosarcoma aged 9-18 years (median 13.5), treated in Department of Paediatrics, Paediatric Gastroenterology and Paediatric Oncology of Medical University in Gdansk, between 1997-2004, were studied retrospectively. Immunochemistry was performed using anti CD34 monoclonal antibody and chromogen to highlight vessels, which were counted at 200 x magnification on 3 microscopic fields. In the same specimens VEGF expression was evaluated semiquantitatively using immunohistochemical method. Patients were divided into two groups depending on presence of metastases. The two parameters were also compared in patients who died and the survivors. RESULTS: 11 of 16 patients are alive, with time of follow up 19-100 months (median 52). Five children died. Mean vascular density ranged from 25 to 87 (46 +/- 16.5). No significant statistical difference in microvessel density between metastatic and non-metastatic patients was observed. Microvessel density in these groups is 46.8 +/- 22.7 and 45.2 +/-7.0 respectively. In the group of survivors MVD was 44.3 +/- 5.9, inpatients who died it was 47.1 +/- 21.0 showing no significant statistical difference. In all patients positive VEGF expression was seen. Only one patient presented low expression of VEGF, the rest had high or medium degree of VEGF expression. MVD in the group with high expression of VEGF was higher than in the group with low and medium expression of VEGF. It was 51.8 +/- 18.7 and 39.2 +/- 14.2. The difference was not significant. CONCLUSIONS: In the presented group of patients no differences in the extent of angiogenesis were seen in relation to treatment outcome or presence of metastases.

The "sugar" clear cell tumor of the lung-clinical presentation and diagnostic difficulties of an unusual lung tumor in youth.

A case of the "sugar" clear cell tumor of the lung in a 16-year-old boy is presented. The course of the disease with general symptoms, never reported before, highlights diagnostic difficulties of an extremely rare lung tumor in youth. The boy presented with daily spikes of unexplained high fever of 6 weeks' duration with features of hypochromic microcytic anemia, elevated erythrocyte sedimentation rate, C-reactive protein, alpha(2)- and beta-globulins, and elevated platelet count. The lung tumor was a yellow, circumscribed mass confined to the sixth segment of the left lung. Histological examination revealed the tumor composed of cells with clear cytoplasm with large content of glycogen, with no signs of necrosis, and immunoreactive for HMB-45, but not for cytokeratin, LCA, CD34, and CD68. The performed thoracotomy and segmentectomy were both diagnostic and curative.

[Bone mineral density in inflammatory bowel diseases in children]. / Mineralizacja kosci u dzieci z wrzodziejacym zapaleniem jelita gruego i choroba lesniowskiego-crohna.

OBJECTIVE: determination of bone mineral density in children treated because of inflammatory bowel diseases. MATERIAL AND METHODS: 42 patients were included: 21 with ulcerative colitis and 21 with Crohn's disease. The duration of illness was from 2.0-24.0 months. Glucocorticoid therapy was applied in 92.9% of patients with the duration from 4-1680 days. The cumulative doses of glucocorticoids were from 160 to 25900 mg. Bone mineral density (BMD) and z-score of L1-L4 were assessed by dual-energy X-ray absorptiometry (DEXA). The mean BMD of L1-L4 were measured in g/cm2 and compared with referential values for gender and age. Osteopenia (ope) mean z-score from -1 to -2 SD, osteoporosis (opo) < -2 SD were accepted. RESULTS: BMD values varied from 0.531 to 1.301 g/cm. Z-score values varied from 0.9 to -5.6 SD. Bone mineral disturbances occurred in 57.2% of cases and it was equally both in 28.6% of cases osteoporosis and osteopenia. In ulcerative colitis osteopenia was predominant (23.8%), while in Crohn's disease osteoporosis occurred more often (23.8%). There was no significance in the duration time of the disease and BMD and z-score. The significant difference was found in the duration of steroid therapy and z-score. No association was found among cumulative dose of steroids and z-score. No significant differences were found in BMD and z-score of lumbar spine in ulcerative colitis and Crohn's disease. CONCLUSIONS: 1. Bone mineral disturbances often complicate inflammatory bowel diseases in children. 2. The association among the duration time of steroid therapy and bone mineral density was confirmed. 3. No significant differences were found in bone mineral density among colitis ulcerasa and Crohn's disease cases.

[Tryptase activity in colon mucosal samples of children with inflammatory bowel disease]. / Aktywnosc tryptazy w bioptatach blony sluzowej jelita grubego u dzieci z przewleklym nieswoistym zapaleniem jelit.

INTRODUCTION: mast cells are dispersed in many tissues, especially in the digestive and respiratory system mucosal membranes. Tryptase is the most important proteinase released from mast cells after degranulation. It influences strongly the cells and tissues by activating the inflammatory process. THE AIM OF THE STUDY: was to assess the activity of tryptase in colon mucosa samples in children with inflammatory bowel diseases (IBD) and in children with bleedings from lower part of gastrointestinal tract (GTB), without inflammation. MATERIAL AND METHODS: a group of 30 children with IBD was analyzed in the study. IBD is formed by three disease entities: ulcerative colitis (UC) - 14 patients, Crohn's disease (CD) - 9 patients and non-specific colitis (NSC) - 7 patients. Moreover, a group of 18 children with bleeding from lower part of gastrointestinal tract was studied. The activity of tryptase in homogenates of colon mucosal samples was estimated fluoroimmunoenzymatically. RESULTS: the results of our analysis showed no statistically important difference between the mean activity of tryptase in groups of children with IBD and GTB (31442 +/- 1304 vs 31868 +/- 775 ug/l). The study of tryptase activities in different disease entities of IBD group showed, that its value in ulcerative colitis group was 31382 +/- 1170 ug/l, in Crohn's disease group it was 31536 +/- 1120 ug/l; in non-specific colitis group the tryptase activity was 32277 +/- 498 ug/l. The analysis with Kruskal-Wallis Anova test revealed that the differences are statistically significant (p = 0.034). In post hoc test the outstanding value is the tryptase activity in children with NSC. Activity of tryptase in colon in much higher than its activity in plasma (normal range 1-19 ug/l). CONCLUSIONS: the activity of tryptase in mucosal membrane samples is much higher than in blood. The extent of mast cells degranulation may be dependent on the form of IBD.

[Ovarian malignant tumours. Efficacy of germ cell and sex cord tumour treatment protocol in Poland]. / Nowotwory zlosliwe zlokalizowane w obrebie jajników. Ocena skutecznosci programu leczenia zlosliwych guzów germinalnych i guzów sznurów plciowych u dzieci w Polsce.

UNLABELLED: Approximately 1% of all malignant tumours among children are localized in the ovary. The majority belongs to germ cell tumours and occurs in the peripubertal period. AIM of the study was the evaluation of the efficacy of malignant ovarian germ cell tumour treatment programme in children. MATERIAL AND METHODS: Since 1998, 40 girls with malignant ovarian tumours were enrolled in the multicentre trial. Mixed germ cell tumours with yolk sac elements and dysgerminoma occurred the most often. Alfa-fetoprotein (AFP) was increased in almost one half of patients. Tumour exceeded the ovary margin in more than half the patients and 25% were qualified as high risk group. 38 children completed the treatment. All but one patient with neuroblastoma received TGM protocol (Tumeurs Germinates Malignes). A VBP regimen (vinblastine, bleomycin, cisplatin) was applied in 19 girls, VIP regimen (etoposide, ifosfamide, cisplatin) in 16, two received no chemotherapy. Due to delayed remission after first-line chemotherapy it was prolonged with ABK (adriamycine, bleomycine, carboplatin) in 3 patients, 1 megachemotherapy regimen with autologous bone marrow transplantation was realized, one patient received a 1.5 year long oral chemotherapy. All the children underwent surgery, 34 primary (56% complete), 12 secondary (75% complete). 8 children were operated twice. RESULTS: Among 34 children with germ cell tumours and 3 with sex cord tumours who completed the treatment all are alive in the first remission. 1 child with neuroblastoma localised in the ovary died due to recurrence. A median follow-up period was 42 months. CONCLUSIONS: The TGM protocol appears to be highly efficient in treatment of germ cell tumours even in advanced stages.

[Testicular malignant tumours. Efficacy of germ cell and sex cord tumours treatment protocol in Poland]. / Zlosliwe nowotwory jader. Ocena skutecznosci programu leczenia zlosliwych guzów germinalnych i guzów sznurów plciowych u dzieci w Polsce.

UNLABELLED: Approximately 2% of of all malignant tumours in boys are localised in the testis. Among them 80% are germ cell tumours with the malignant elements of yolk sac tumour. AIM of the study was evaluation of the efficacy of malignant testicular tumour treatment programme in children. MATERIAL AND METHODS: Since 1998 31 boys aged 1 month to 18 years (median 14 years) with malignant testicular tumours were enrolled in the multicentre trial. Patomorphologically clear yolk sac tumour (33%) and mixed germ cell tumour (42%) with the majority of yolk sac tumour component or carcinoma embryonale, occurred most often. Alfa-feto-protein was increased in 63% and choriogonadotropin in 26 patients. 61% patients had local clinical stage and the tumour was localized in the testis. In 39% patients tumour exceeded the testis margin. 4 patients were excluded from analysis as 3 are actually treated and 1 died on the second day of admittance to hospital. All patients received TGM 95 regimen (Tumeurs Germinales Malignes). Surgery (orchidectomy) was applied in 27 boys, 26 were primary (81% complete), 3 secondary (100% complete). 33% received no chemotherapy after surgery, in 41% VBP protocol (vinblastine, bleomycin, cisplatin) was given and in 26%o VIP protocol (ethoposide, ifosphamide, cisplatin). Two patients received also ABK (adriamycine, bleomycin, carboplatin). RESULTS: Among 26 children with germ cell tumours, 25 (96%) are alive, 23 (88%) are in first remission after completion of treatment. One child died due to central nervous system metastases. 2 children had local recurrence treated with chemotherapy or surgery with good result. Median follow-up is 45 months. CONCLUSIONS: TGM regimen is highly efficient in the treatment of malignant testicular tumours. Problems occur in cases of disseminated disease.

[Assessment of treatment results in children with malignant liver tumours in own experience]. / Ocena wyników leczenia dzieci z nowotworami zlosliwymi watroby na podstawie badan wlasnych.

AIM: The assessment of malignant liver tumours in children treated in our centre between years 1985-2004 has been made in order to analyze the prognostic factors and improvement in survival rate. MATERIAL AND METHODS: 17 patients with malignant liver tumours were followed-up. There were 10 (58,8%) patients with hepatoblastomas, 5 (29,4%) hepatocarcinomas, 1 (5,9%) undifferentiated embryonal sarcoma and 1 (5,9%) rhabdomyosarcoma. Primary metastatic disease was recognized in 3 cases as: hepatic vascular involvement, lungs, femoral bone and lymph nodes of liver hilus metastases. All patients underwent preoperative chemotherapy. Tumour resection was attempted in 13 (76,5%) cases; it was complete within adequate resection margins in 11 (64 7%). In 3 cases biliary fistulas occurred after surgery. Secondary metastases appeared in lungs, lymph nodes of liver hilus and central nervous system in 4 cases. RESULTS: Twelve patients are alive with median follow-up 34,0 mths, five died with median survival time 16,0 mths. Total excision of liver tumour had no statistical significance in lifetime prolongation (p =0,12). Survival rate was statistically longer in patients without metastatic disease (p=0,028). CONCLUSIONS: Complete surgical excision had no statistical significance in increasing survival time in liver tumour patients. Metastatic disease had statistical significance in shortening overall survival of patients with liver tumours. Unsatisfactory results in hepatocarcinoma treatment in children dramatically demonstrate the need for new treatment approaches.

[Superoxide dismutase in children with juvenile idiopathic arthritis]. / Dysmutaza ponadtlenkowa u dzieci z mlodzienczym idiopatycznym zapaleniem stawów.

UNLABELLED: The use or antioxidants as drugs that may control the inflammatory process recently has become widely studied in adults. One of the most important components or antioxidant barrier in humans is enzyme superoxide dismutase. Experimental treatment with superoxide dismutase proved to be effective in animals. THE AIM of the present study was to estimate the activity of superoxide dismutase (SOD) in patients with rheumatoid disease of childhood -- juvenile idiopathic arthritis and to analyse it in different clinical aspects of the disease including its activity, course and time of duration. MATERIAL AND METHODS: A group of 70 children with juvenile idiopathic arthritis, age from 3 to 18 years, patients of Rheumatologic Hospital in Sopot was examined in years 1996-2001. Juvenile idiopathic arthritis was diagnosed according to the International League Against Rheumatism classification (Durban 1997). The control group consisted of 29 healthy children age from 3 to 18 years. Superoxide dismutase activity was determined in full, heparinised blood with the use of spectrophotometric method or Randox. The study was accepted by the Local Committee of Ethics. RESULTS: A statistically significant increase in superoxide dismutase activity was found in group of children with juvenile idiopathic arthritis compared to the control group. In the polyarticular form of disease the activity of superoxide dismutase was also significantly higher than in the control group. CONCLUSIONS: In course of juvenile idiopathic arthritis, differently from rheumatoid arthritis of adults, superoxide dismutase activity seems to be stimulated in the majority of cases, so the treatment with exogenous superoxide dismutase may not be effective.

Abdominal inflammatory masses mimicking neoplasia in children-experience of two centers.

Despite progress in modern imaging, some inflammatory masses are difficult to distinguish clinically from neoplastic processes. In such cases the pathology report has a great distinctive value, but even then the final diagnosis may be difficult to reach. Eight patients with abdominal tumors of inflammatory origin were treated in two institutions, the Department of Pediatric Surgery of the Medical University of Gdansk, Poland, and Helios Center of Pediatric Surgery in Berlin, Germany, during the last 10 years. Four tumors were located in the pelvis, two in the liver, and two in the colonic mesentery. Five of them were inflammatory pseudotumors (two subclassified as inflammatory fibrosarcoma), one had nonspecific inflammatory changes, one was diagnosed as idiopathic retroperitoneal fibrosis, and one was diagnosed as bacillary angiomatosis. All patients underwent surgical tumor biopsy, excisional in four and incisional in four. All but two children underwent macroscopically complete tumor excision (four primarily, two secondarily). In one case the tumor resolved with antibiotherapy. Surgery in retroperitoneal masses was often extensive and associated with significant complications because of invasive tumor growth. In conclusion, intraabdominal inflammatory lesions may closely mimic neoplasia in children. Clinical doubts result in repeated biopsies, and for this reason excisional biopsy should be preferred. In some cases, when excisional biopsy is not feasible due to invasive growth of the tumor, delayed complete mass excision should follow, despite occasional significant morbidity. The etiology and exact nature of inflammatory pseudotumors are still obscure, and it is unknown whether they represent inflammatory lesions or true neoplasia.

[Role of parvovirus B19 as a causative agent for arthritis in children -- preliminary studies]. / Rola parvovirusa B19 jako czynnika etiologicznego artropatii u dzieci -- badania wstepne.

INTRODUCTION: Parvovirus B19 infection can be associated with arthritis in children and adults. The causative role of PB19 infection in arthritis and ulcerative synovitis would prove the etiology of juvenile idiopathic (jia) and reumatoid arthritis (ra). AIM: The aim of this study was the evaluation of the role of PB 19 infection in children's arthritis. MATERIAL AND METHODS: The group of 41 children with the diagnosis of jia, arthralgia, reactive arthritis and nodular erythema according to EULAR criteria was examined. In early stage of arthritis lasting less than 3 months, there were 22 and in prolonged period lasting more than 3 months there were 19 children. RESULTS: No evidence of PB19 genome was detected by PCR reaction in blood of the examined patients. The possible reasons of the obtained negative results in this study were discussed. CONCLUSIONS: PB19 research should be performed in more cases of children's arthritis, both using PCR and by serological methods.