Hyperthyroidism secondary to disseminated mucormycosis in a child with acute lymphoblastic leukemia: case report and a review of published reports.

Thyroiditis due to fungal infection is an extremely rare cause of hyperthyroidism. The most common etiological factor of thyroiditis is Aspergillus. Infections due to members of the Mucorales have been an increasing clinical problem in recent years, and the prognosis in generalized infections due to those fungi is usually very poor. No hyperthyroidism in a child with thyroiditis due to mucormycosis has been reported in the literature so far. We describe a clinical course of generalized mucormycosis with thyroid involvement in a 12-year-old girl treated for acute lymphoblastic leukemia. The child underwent a hyperthyroidism connected with thyroid involvement due to a fungal process. The diagnosis was based on the clinical signs, laboratory findings and typical ultrasound scan; however, later attempt to amplify the fungi DNA from the tissue block has failed. The child died because of multiorgan failure due to general fungal infection 49 days after the invasive fungal infection was diagnosed. The generalized mucormycosis is always connected with poor prognosis and the mortality is high.

Analysis of the impact of environmental and social factors, with a particular emphasis on education, on the level of metabolic control in type 1 diabetes in children.

BACKGROUND: Type 1 diabetes is a chronic, incurable childhood disease. Chronically uncontrolled diabetes is associated with eye, kidney, nerve, heart and blood vessel damage and function impairment. The aim of this study was to evaluate the impact of various social and environmental factors, with a particular emphasis on education, on the level of metabolic control in diabetes. MATERIAL AND METHODS: The survey research was conducted in 102 children aged 0-18 years, diagnosed with type 1 diabetes. Based on the HbA(1c ) level, patients were divided into: group A (63 patients with fairly well and moderately controlled type 1 diabetes mellitus) and group B (39 patients with metabolically uncontrolled type 1 diabetes mellitus). The impact of various environmental and social factors on the degree of metabolic control of type 1 diabetes was analysed. RESULTS: No effect of typical environmental and social factors, such as: place of residence, gender, parents' education and their professional activity, on the level of metabolic control of type 1 diabetes was found. However, groups A and B significantly differed in the level of knowledge about diabetes and its treatment, in the regularity of meals, in possessing a nutrition scale and in the self-assessed preparation for taking care and custody of a child with type 1 diabetes. CONCLUSIONS: 1. Children with type 1 diabetes and their parents require ongoing education about the disease and its treatment. 2. The regularity of meals and the use of a nutrition scale have considerable impact on the level of metabolic control of the disease.

[Pathological daytime sleepiness in an 11-year old girl receiving growth hormone treatment previously cured from craniopharyngioma]. / Napady patologicznej sennosci dziennej u 11-letniej dziewczynki leczonej ludzkim rekombinowanym hormonem wzrostu po zakonczonym leczeniu z powodu czaszkogardlaka.

UNLABELLED: An 11-year old girl with multihormonal pituitary deficiency previously cured from craniopharyngioma was admitted to the endocrinology Department because of pathological daytime sleepiness. At the age of 7 she had undergone brain tumor surgery with adjuvant radiotherapy (complete dose of 5400 cGy). She had been given replacement therapy of thyroid hormone, cortisol and adiuretin. At the age of 10 years she had started growth hormone (rGH) treatment. After a period of four months of rGH replacement therapy the girl's mother had observed symptoms of excessive daytime sleepiness with a tendency to escalation. MRI of the brain had been performed; no progression has been shown. After 10 months from the start of rGH replacement therapy was referred to the endocrinology department. On the basis of laboratory findings electrolyte and hormonal abnormalities were excluded. On physical examination, the girl manifested massive tonsillar hypertrophy. It was disclosed that she developed obstructive sleep apnea with the drop of oxygen saturation to 60%. The patient was qualified to adenotonsillectomy. There was a spectacular postoperative improvement observed with no future episodes of night apnea and daytime sleepiness. In our opinion, the rGH treatment in our patient induced hypertrophy of the tonsils and adenoid, which led to obstructive sleep apnea syndrome with compensatory daytime sleepiness. CONCLUSIONS: Obstructive sleep apnea syndrome with compensatory daytime sleepiness may occur in children on rGH replacement therapy. During rGH therapy children should be regularly examined by a laryngologist.

[The evaluation of the influence of growth hormone therapy on growing process and metabolic functions in patients after treatment of craniopharyngioma]. / Ocena wplywu terapii hormonem wzrostu na przebieg wzrastania i funkcje metaboliczne u pacjentów po leczeniu craniopharyngioma.

INTRODUCTION: Craniopharyngioma (CP) is a tumor, which damages pituitary function because of its localization. Panhypopituitarism (PHP) and excessive weight gain with lipid dysfunction are frequently observed. The growth hormone therapy (rGH) profits in the increase of growth rate and also may have metabolic effects like body weight reduction. AIM OF THE STUDY: The evaluation of benefits from rGH therapy in patients cured from CP. MATERIAL AND METHODS: 12 patients (7 boys and 5 girls) treated for CP with surgery; 3 of them also underwent radiotherapy. The mean age at examination time was 11.7 yrs; remission time 2.96 yrs; rGH therapy started on average 3.69 yrs after the surgery. Height (hSDS), weight, BMI were measured after the surgery, before and after 1 yr of rGH treatment. Height velocity (HV) was evaluated before and after 1 yr of rGH therapy. Pituitary GH-function was assessed. In addition, measurements of TSH, ACTH, LH, FSH. Cholesterol, LDL, HDL, triglycerides and HbA1c were estimated before and after one year of rGH therapy. RESULTS: All patients presented PHP. The GH-peak average 1.53 mIU/l; IGF-1 39.37 ng/ml; TSH 0.1 U/l; ACTH 17.48 pg/ml; LH 0.13 U/l; FSH 0.41 mIU/ml. HSDS after oncological treatment (OT) average -1.66 SD and decreased significantly until rGH therapy; weight after OT average 28.45 kg and until rGH therapy increased significantly; BMI after OT average 19.26 and increased significantly until rGH therapy as well. HV was on average 3.34 cm/yr until rGH started. After one year of rGH therapy hSDS and HV increased significantly; they average -1.65SD and 10.21 cm/yr respectively. BMI, HbA1c and LDL decreased significantly. During rGH therapy neither tumor recurrence nor severe side effects were observed. CONCLUSIONS: rGH therapy of patients cured from CP influences profitably not only growth rate, but also BMI reduction and the decrease in cholesterol LDL and HbA1c.

Difficulties or mistakes in diagnosing type 1 diabetes in children?--demographic factors influencing delayed diagnosis.

BACKGROUND: Diabetic ketoacidosis (DKA) development in children with new-onset type 1 diabetes (T1DM) is often the main consequence of delayed diagnosis. The aim of the study was to estimate the frequency of difficulties in T1DM diagnosis and to investigate if and how the demographic factors (gender, patient's age at presentation, family history of T1DM, level of maternal education, place of residence, and health service unit the patient called at) have any influence on diagnostic delays. SUBJECTS AND METHODS: Retrospective analysis of 474 children (243 boys-51.27% and 231 girls -48.73%) with new-onset T1DM aged below 17 yr and living in the Pomeranian region of Poland was carried out. The delay in diagnosis was recognized if the patient was not diagnosed on the first visit because of omission, wrong interpretation of main diabetic symptoms, exclusive treatment of additional signs, or concomitant diseases. RESULTS: Difficulties in diagnosing T1DM were found in 67 cases (14.13%) and they are the main cause of DKA development in these children (p = 0.00). Among the examined demographic factors, mainly the patient's age at presentation has a significant influence on diagnostic delays (p = 0.01), especially in children below 2 yr (p = 0.00). Most frequently family doctors were responsible for wrong preliminary diagnosis. CONCLUSIONS: Difficulties in diagnosing T1DM are a significant cause of DKA development in children with new-onset disease. Patient's age at presentation is the main risk factor of delayed diagnosis, especially in children below 2 yr. The increase in awareness among pediatricians concerning the possibility of T1DM development in children is needed.

Difficulties or mistakes in diagnosing type 1 diabetes mellitus in children? The consequences of delayed diagnosis.

INTRODUCTION: Delayed diagnosis is one of the causes of diabetic ketoacidosis (DKA) development in many children with new-onset of type 1 diabetes mellitus (T1DM). THE AIM: of the study was to analyse difficulties in diagnosing T1DM and establish if delayed recognition is the main risk factor of diabetic ketoacidosis (DKA) in children with newly diagnosed T1DM. MATERIALS AND METHODS: Retrospective analysis of 335 children with new-onset T1DM, aged below 17 years and living in the Pomeranian region of Poland, was carried out. The delay in diagnosis was recognized if the patient was not diagnosed on the first visit because of omission, wrong interpretation of symptoms, exclusive treatment of additional diabetic signs or concomitant diseases. RESULTS: Difficulties in diagnosing T1DM were found in 54 cases (16.12%). The initial diagnosis was wrongly categorized as respiratory system infection (46.3%), perineal candidiasis (16.6%), gastroenteritis (16.6%), urinary tract infection (11.1%), stomatitis (11.1%), appendicitis (3.7%). Duration of symptoms (mean 14 days) and glycated haemoglobin level did not significantly correspond with diagnostic delay. DKA was significantly more frequent in children with delayed diagnosis - 33 patients (61.11%) (p=0.0005). The infection preceding diagnosis of T1DM, more common among children with delayed recognition, did not affect the duration of symptoms or affect characteristicly more frequent DKA development in children with diagnostic delay. CONCLUSIONS: No significant associations between diagnostic delay and duration of diabetic symptoms as well as glycated haemoglobin level exclude possibility of delayed diagnosis because of slow development of diabetic symptoms. Significantly more frequent DKA development in children with delayed recognition, irrespective of infection preceding diagnosis, and no confirmation that younger patient's age predisposes to quick increase of DKA point out that the main risk factor for DKA development in the research group was diagnostic delay.

[Diagnosis and treatment of thyroid cancer in children in the multicenter analysis in Poland for PPGGL]. / Diagnostyka i leczenie raka tarczycy u dzieci w Polscew analizie wieloosrodkowej dla PPGGL.

INTRODUCTION: 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. MATERIAL AND METHODS: 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. RESULTS: 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. CONCLUSIONS: The necessity of introduction of unified therapeutic standard in children with DTC in Poland is underlined.

[Evaluation of final height in patients with pituitary growth hormone deficiency who were treated with growth hormone replacement]. / Ocena wzrostu koncowego pacjentów z somatotropinowa niedoczynnoscia przysadki, leczonych hormonem wzrostu.

BACKGROUND: Growth hormone (GH) therapy has been used for children with pituitary GH deficiency. It resulted in improving their height velocity and achieving an adult height in the normal range for the general population. OBJECTIVES: To evaluate the final height in childhood-onset growth hormone deficiency patients who had already completed treatment and were still GH deficient in adult life. MATERIAL AND METHODS: 21 children (12 boys and 9 girls) diagnosed as GH deficiency and treated with growth hormone to final height at doses of 0.17 mg/kg/week (0.5 IU/kg/week) subcutaneously for 7 days. There were 7 patients with isolated GHD and 14 with multiple pituitary hormone deficiencies. RESULTS: At the diagnosis peak serum GH concentrations were 2.8+/-2.8 mU/l in insulin tolerance test and 3.3+/-2.2 mU/l in clonidine test. Reconfirmation of the GH deficiency diagnosis after growth hormone treatment revealed a peak serum GH 1.77+/-1.2 mU/l in insulin tolerance test. Mean chronological age of the patients at the beginning of treatment was 10.29+/-3.57 years and was significantly higher in boys. Patients had completed a course of treatment in the chronological age of 17.85+/-1.97 years. Children began treatment with mean bone age 7.24+/-3.57 years and ended with 15+/-0.97 years. After the treatment a significant improvement in height was shown. Height SDS at the beginning of the treatment was -4.03+/-0.91 and -0.69+/-1.01 after the treatment. There was no difference between final height and target height (-0.54+/-0.93 SDS) in our patients. CONCLUSIONS: Children with pituitary growth hormone deficiency who were treated with growth hormone replacement achieve a final height in the normal range for the general population and their target height.

[Clinical characteristics and therapy outcome in children with stage IV Hodgkin's lymphoma--the experience of two oncological centres]. / Charakterystyka kliniczna i wyniki leczenia dzieci z choroba Hodgkina w IV stadium zaawansowania--doswiadczenia dwóch osrodków onkologicznych.

UNLABELLED: The cure rate in children with Hodgkin's disease (HD), at present time exceeds 90% but the prognosis in stage IV HD is much worse. THE AIM of the study was to analyze the initial symptoms, course and results of oncological therapy in children with stage IV of Hodgkin's disease. MATERIAL AND METHODS: The analyzed group comprised of 15 patients with IV stage HD (M/F: 11/4, mean age: 12 years), treated from January 1993 to March 2005, in two Polish centres of paediatric oncology in Gdansk and Lublin. The diagnosis and therapy were carried out according to the current protocols approved by the Polish Paediatric Leukaemia / Lymphoma Study Group (PPGBCh). RESULTS: Mean duration of initial symptoms was 4.5 months, with most children presenting general symptoms of HD. At diagnosis, the involvement of mediastinal and/or hilar lymph nodes was found in nine patients, lung infiltrations in six, involvement of the spleen, liver and bones in five, three and one patient, respectively. The nodular sclerosis histopathological type of HD predominated. Poor response to standard treatment was observed in five children. One patient received additional cycles of chemotherapy MVPP/B-DOPA, four children were administered the 2nd line chemotherapy Salvage 95. One boy with very poor response to the 1st and 2nd therapy lines additionally underwent megachemotherapy with peripheral blood stem cells transplantation. Radiotherapy was given to 13 children. 13 out of 15 children are alive and free of disease with mean follow-up duration of 6 years. In two of them late complications affecting hormonal status, cardio-pulmonary disorders and chronic B and C hepatitis were observed. Two children died including one admitted in a very severe condition, after long-lasting medical history who died of neutropenia-related sepsis. The second boy died 12 months after stem cell transplantation because of a second neoplasm--acute myeloblastic leukaemia. CONCLUSION: Chemo- and radiotherapy implemented according to protocols approved by the PPGBCh for children with stage IV HD, result in complete remission in most patients. Diagnosis made at earlier stages would result in giving less aggressive therapy, connected with a lower risk of durable late complications.

[Complications in children cured from Hodgkin's disease]. / Powiklania plucne u dzieci wyleczonych z choroby Hodgkina.

UNLABELLED: THE AIM of the study was to evaluate the incidence of pulmonary complications in children cured from Hodgkin's disease (HD). MATERIAL AND METHODS: 42 children with HD were treated in the Department of Paediatrics, Haematology, Oncology and Endocrinology, Medical University of Gdansk, between 1994 and 2004. Stages of HD: II--26 children, III--10, IV--6; general symptoms (group B) were present in 50% of patients. Mediastinal involvement was found in 33 children, lung parenchyma infiltration in seven and bronchi involvement in one. In 1/3 of these patients the localization of HD within the chest was massive and symptomatic with signs of the superior caval vein symptoms, cough, dyspnea and cardiac tamponade. The treatment was conducted according to the schemes of the Polish Paediatric Leukaemia /Lymphoma Study Group. Eleven patients required therapy modification including six, in whom the intense line II chemotherapy Salvage 95 was introduced. 29 patients received chest irradiation with doses between J 75 and 36.5 Gy. Pulmonary function was evaluated from the results of clinical examination, 1 chest radiography (CXR), computed tomography, spirometry and lung scintigraphy. RESULTS: Pulmonary complications occurring as fatigue and diminished physical effort tolerance was observed in only two children. Some of the remaining 40 patients demonstrated asymptomatic abnormalities in the analysed tests. Abnormalities in CXR (upper mediastinal fibrosis, postoperational changes within the diaphragm and pneumonitis) were found in six children, minor ventilation problems in spirometry--in 12 and decreased lung perfusion in five. The scintigraphic signs of lung embolisation were not observed in our material. Most of the pulmonary complications occurred in children with enlarged lymph notes located within the chest, especially these with bulky disease presenting with cardio-pulmonary symptoms. In this group of patients the chest irradiation was performed in all except four children, three patients were also administered aggressive salvage chemotherapy. CONCLUSION: The pulmonary complications in children after completed therapy of HD are not common and mainly asymptomatic and occur predominantly in patients with massive mediastinal and/or lung involvement at diagnosis. The issue needs further evaluation of a more numerous group of HD survivors and a longer follow-up.

[Cardiovascular malformations in Turner syndrome]. / Wady ukladu sercowo-naczyniowego w zespole Turnera.

BACKGROUND: A literature review indicates that the prevalence of cardiovascular malformations in patients with Turner syndrome ranges from 17 to 47 percent. OBJECTIVES: The aim of the study was to evaluate the cardiovascular system in patients with Turner syndrome, controlled by the Pediatric Endocrinology Clinic of the Medical University of Gdansk. MATERIAL AND METHODS: The study included 55 females with Turner syndrome aged 3-20 years (mean age 9.6 years). The procedure included an interview, subject examination, chest X-rays and echocardiography. The echocardiography included M-mode measurements, two-dimensional evaluations from all standard planes, pulsed and continuous wave Doppler as well as color flow mapping. In selected cases, cardiologic examination embraced also cardiac catheterization, cineangiography and continuous 24-hour blood pressure measurement. RESULTS: Cardiovascular malformations were found in 18 out of 55 females (32.7%). Aortic coarctation was detected in 9 out of 18 patients (50%), bicuspid aortic valve in 33%, and aortic insufficiency in 22% of them. 78% of Turner subjects with cardiovascular malformations had monosomy (45,X). All females, except one, with aortic coarctation, had a 45,X karyotype. One aortic coarctation was diagnosed during prenatal examination. Before the last medical examination, 9 females had undergone a surgical treatment. In one case, the surgical treatment was preceded by a balloon angioplasty. Due to a recoarctation, one female was submitted to an additional balloon angioplasty. One female died during the post surgical period suffering from aortic wall injury. In four cases, despite successful surgeries, there still remains an arterial hypertension requiring pharmacological treatment. In 4 out of 18 cases (22%) isolated aortic valves defects were diagnosed, however, at present time they do not require any surgical treatment. In three cases, echocardiographic examination indicated a persistent left superior vena cava and aneurysm atrial septal defect with an intact septum. CONCLUSIONS: Due to a high prevalence of cardiovascular malformations, girls with Turner syndrome should be monitored by a pediatric cardiologist. In each case, female with Turner syndrome should be submitted to echocardiography. Also a careful control of the blood pressure is indicated.

[Endocrinology complications of Langerhans cell histiocytosis]. / Endokrynologiczne powiklania histiocytozy.

The aim of presentation is an unusual case report of a 17 year old boy with short statured (SD (-)4), absent puberty and thyroid enlargement. Endocrine tests demonstrated a growth hormone and gonadotropin deficiency with diabetes insipidus. Magnetic resonance imaging showed a small pituitary gland and an absence of the posterior bright signal. Fine needle aspiration cytology of the focus localized in the left lobe of thyroid gland and nearest enlarged lymph nodes revealed diffuse infiltrate by the large histiocytosis confirming Langerhans cell histiocytosis like in the skin histological specimen biopsy. After chemotherapy we observed a regretion of focal changes in the thyroid.

[Malignant neoplasms localised in the parameningeal region in children treated at two Polish oncological centres-diagnostic and therapeutic dilemmas]. / Nowotwory zlosliwe o lokalizacji okolooponowej u dzieci w materiale dwóch osrodków onkologicznych--trudnosci diagnostyczne i terapeutyczne.

UNLABELLED: Malignant neoplasms localized in the parameningeal region include mainly soft tissue sarcomas (MTM), non-Hodgkin s lymphomas (NHL-B) and, less frequently, nasopharyngeal carcinomas. The aim of the study was to analyze diagnostic and therapeutic problems in children with parameniingeal neoplasms treated in Departments of Paediatric Oncology in Gdansk and Lublin between 1992 and 2004. MATERIAL AND METHODS: The study includes 32 patients (M/F: 23/9), aged 2 to 17 years, mean 6,3 years. In 17 children MTM was diagnosed: in nine NHL-B-cell and in six--nasopharyngeal carcinoma (lymphoepithelioma). The diagnosis of NHL-B and undifferentiated MTM were made in two children treated previously for NHL-nonB and retinoblastoma. Two cases of NHL appeared in a girl with ataxia-teleangiectasia syndrome. RESULTS: Initial symptoms lasted from 2 weeks to 24 months, mean 4,5 months for the whole group. In NHL patients mean period ofsymptoms was 4,5 weeks, in MTM- 5,5 months and in lynmphoepithelioma--7 months. Symptoms associated with the tumours localisation (snoring, breathing through the mouth, epistaxis, chronic purulent rhinitis, dysphagia and earache) predominated and were treated initially as upper respiratory tract infections. Cervical lymph nodes enlargement was observed in 30% children with MTM and 83% with lymphoepithelioma. Most of patients presented with highly advanced stages of neoplasms. MTM and NHL-B treatment was conducted according to the protocol approved by the Polish Paediatric Solid Tumours and Leukaemia/Lymphoma Studies Group. In patients with lymphoepithelioma different treatment schemes were administered, including chemo- and radiotherapy. Good response to therapy was found in 13/32 patients (41%). The group included 24% children with MTM (all with embryonic subtype), 56% with NHL-B and 67% with lymphoepithelioma. All these patients attained complete remission after standard line I therapy. But 13 children with MTM, four with NHL-B and two with lymphoepithelioma required more aggressive line II treatment because ofpoor response to therapy (NR) or relapse. Finally, 20 of 32 followed-up patients (62,5%) are in durable complete remission from 10 months to 11 years 4 months (mean 4 years) after therapy discontinuation. This group consists of all nine patients with NHL-B, 67% children with lymphoepithelioma and 41% with MTM. In six children (30%) persistent complications of oncological treatment occurred, including: hypoacusia, postradiation defect of the eye ball, postsurgical facial nerve palsy and cranio-nasal fistula complicated with pneumocephaly. A patient with MTM of maxillary sinus developed a second neoplasm 2 years after first therapy. This was glioblastoma multiforme located in the left parietal lobe (outside the radiation field). At present, the boy is in complete remission 2,5 years after treatment for the second tumour Among 32 children with parameningeal neoplasms 11 patients died (nine with MTM and two with lymphoepithelioma), all in the phase of disease progression (five NR and six after relapse). In two of them (with MTM) the direct cause of death was myelosupressive, gastrotoxic and infectious complications of antitumour therapy. One child still undergoes treatment for MTM relapse.

[Influence of surgery and radiotherapy on growth and pubertal development in children treated for brain tumour]. / Wplyw zabiegu operacyjnego i radioterapii na tempo wzrastania i rozwoju dzieci z guzami osrodkowego ukladu nerwowego.

INTRODUCTION: The increasing number of childhood cancer survivors has resulted in a growing interest in the late effects, which depend on type of treatment. Frequently, a brain tumour and its therapy in children are endocrinologically devastating. AIM OF STUDY: The aim of study was to compare growth and pubertal development in children after brain tumour therapy treated or not treated with recombinant growth hormone (rGH). MATERIAL AND METHODS: 18 children were included in this study. Group I - (12/18) not treated with rGH, after total resection of brain tumour: craniopharyngeoma (8/12), astrocytoma (2/12) ependymoma (1/12), germinoma (1/12). Mean time of remission was 5,0yrs (+/- 0,9). Group II - (6/12) treated with rGH, after subtotal resection of craniopharyngeoma (4/6), ependymoma (1/6), medulloblastoma (1/6) and cranial irradiation with mean total doses 46,5 Gy (+/- 5,65). Children were qualified for rGH replacement according to deceleration of growth and lower growth hormone secretion (< 10 ng/ml) in stimulating tests. Mean time of remission was 6,5 yrs (+/- 2,41). Growth, height in centimeters converted to standard deviation score--SDS, body mass index (BMI), pubertal status and hormonal tests, were also evaluated. RESULTS: All patients were treated with surgery with no cranial irradiation in prepubertal age. 100% children of group I needed substitution because of secondary hypothyreosis, 83% due to secondary adrenal insufficiency and 53% of diabetes insipidus. Mean height after brain tumour surgical treatment in group I was - 1,24 SDS (+/- 0,85) and did not significantly change in the time of observation. Two girls needed hormonal substitution for hypogonadotropic hypogonadism. Mean BMI after total resection of brain tumour was 18,09 (+/- 4,20) and significantly increased to 23,73 (+/- 2,82). In group II - all children presented multihormonal pituitary insufficiency. Mean deviation score of height before rGH treatment was - 3,84 SDS (+/- 2,87) and after mean time of rGH therapy of 1,5 yrs (+/- 1,2) decreased to 2,6 (+/- 1,06). Mean BMI before treatment with rGH 18, 06 (+/- 4,4) increased to 22,41 (+ 0,74) in the time of observation and decreased to 18,5 (+/- 2,87) after 1,5 years (+/- 1,2) of rGH treatment. CONCLUSIONS: 1. Children treated with surgery for brain tumour need substitution for secondary hypothyroidism, part of then need treatment for secondary adrenal and gonadal insufficiency and diabetes incipidus. 2. Children who were treated with surgery and/or cranial irradiation developed multihormonal pituitary insufficiency, growth failure and replacement rGh therapy was needed. 3. Total resection of brain tumour without chemo- and radiotherapy did not impair growth in first years after surgery.

[Late side-effects of treatment in patients with nephroblastoma]. / Odlegle nastepstwa leczenia przeciwnowotworowego u dzieci z rozpoznaniem nerczaka zarodkowego.

UNLABELLED: The aim of the study was to analyse late side-effects of the antineoplastic treatment in patients with diagnosed nephroblastoma. MATERIAL AND METHODS: In the years 1986-2002, 80 children were treated in the Gdansk Centre, for nephroblastoma, 13 of them followed SIOP-6, 25 were treated according to SIOP-9 and the remaining 42, SIOP 93-01 programme. RESULTS: 70 patients had long-term clinical remission. 10 patients died, 3 of them due to treatment complications (all were treated in accordance with SIOP 92-01: 1 - mycotic sepsis, 1 intra-operative hemorrhage and 1 - typhlitis), the other 7 due to disease progression (2 on the SIOP-6, 2 on the SIOP-9 and 3 on the SIOP 93-01 protocol). The late diagnosed complications of the treatment were: cardiomiopathy in 7 children, renal insufficiency in 1 case (the girl is presently after kidney transplant), tubulopathy in 4, proteinuria in 3, arterial 1 hypertension in 6, scoliosis in 9, chronic hepatitis (exclusively in children treated before 1994): type B in 11 (one girl has portal hypertension and esophageal varices), type C in 12 and both B and C in 5 children. Of the endocrinologic complications 4 patients were diagnosed with hipergonadotrophic hypogonadism. Secondary, neoplasms that were diagnosed several years from the completion of the nephroblastoma treatment, were the cause of death in 3 children (2 osteosarconma and 1 chondrosarconma). CONCLUSIONS: The advance in the treatment of neoplasia that has been achieved due to modern diagnostic and therapeutic procedures and the continual increase in the number of survivals obliges us the provide the patients with extensive specialistic and continuous medical care.

[Effect of three years obligatory iodine prophylaxis on the incidence of goiter in school children fron the seaside region of Poland]. / Wplyw trzyletniej obowiazkowej profilaktyki jodowej na czestosc wystepowania wola u dzieci szkolnych w regionie nadmorskim Polski.

UNLABELLED: In 1993 a mild iodine deficiency in the seaside region of Poland was revealed. In 1997 a mandatory iodine prophylaxis was introduced. The aim of the study was to determine the prevalence of goiter in schoolchildren aged 12-15 years from the seaside area after 3 years of obligatory iodine prophylaxis. The study comprised 628 children (291 boys and 337 girls). Thyroid size was estimated by palpation and by ultrasonography. Analysis of iodine excretion (Uroiod-Test Merck) was performed in 147 children. The goiter was found in 9% of children by palpation. The mean thyroid volume in 12 years old group was 9.3 +/- 5.1 ml, in 13 years old group 10.6 +/- 4.2 ml, 14 years old group 11.7 +/- 3.9 ml, 15 years 13.8 +/- 4.3 ml. The thyroid volume in girls was larger than that in boys. Thyroid volume above 97th percentile was assessed by ultrasound in 16% children (all of them from rural area). No goiter was found by palpation or ultrasound in children living in Gdansk Thyroid volume is closely correlated with iodine excretion, age and height. Urinary iodine excretion above 100 micrograms/l was stated in 68% children. CONCLUSIONS: Model of obligatory iodised salt prophylaxis is highly effective and is able to increase urinary iodine excretion and decrease of goiter prevalence. Ultrasound is especially recommended for the evaluation of thyroid volume in children from previously mild iodine deficiency areas.

[Difficulties in the diagnosis of thyroid cancer in 8-year-old boy]. / Trudnosci w rozpoznaniu raka gruczolu tarczowego u 8-letniego chlopca.

The 8-year-old boy with 13 months history of enlargement of right cervical lymph nodes is described in this paper. Infectious lymphadenopathy was initially diagnosed. He was treated with antibiotics without success. Fine-needle aspiration biopsy in one of the enlarged lymph nodes showed the cells of normal thyroid gland without any signs of atypical growth. USG and scintigraphy revealed normal thyroid gland without nodules; no extra thyroid glands were detected. In 13th month of the disease two enlarged (about 5 ml each), painful, rather hard cervical nodules were present. The thyroid gland was no enlarged and without any palpable nodules. Chest X-ray and second thyroid scintigraphy were normal. Ultrasound imaging of thyroid gland revealed hypoechogenic lesion 11 x 9 x 12 in diameter in the upper part of right thyroid lobe. The morphological findings of removed nodules allowed to make a diagnosis of carcinoma papillare psammomatosum glandulae thyroideae. Total thyroidectomy with removal of cervical ipsilateral lymph nodes was performed. The operation is to be followed by radioactive iodine ablation and thyroid hormone suppression. The authors analyzed the causes and difficulties in diagnosis.

[Growth velocity in children after chemo- and radiotherapy]. / Przebieg wzrastania dzieci dotknietych choroba nowotworowa.

UNLABELLED: The increasing number of childhood cancer survivors has resulted in growing interest in the late effects of chemo- and radiotherapy including growth also. THE AIM OF STUDY: The aim of study was to evaluate growth in children treated for acute lymphoblastic leukaemia (ALL) and Wilms' tumour who achieved complete continuous first remission following treatment. PATIENTS AND METHODS: 52 children included in this study: 30 treated for ALL - group I and 22 with Wilms' tumour - group II since 1986 to 1996. Group I- all children received prophylactic cranial irradiation at the total dose 12 and 18 Gy and chemotherapy according to therapeutic course BFM 83 and BFM 90. Group II - all children received abdomen irradiation with total dose 15-35 Gy, chemotherapy according to therapeutic course SIOP 9 and SIOP 92. We analysed growth velocity from the time of diagnosis to the time of examination. Results were reported as standard deviation score (SDS) to allow for comparison of patients of different age and sex. RESULTS: We observed significant growth deceleration in the first year of treatment and catch-up after 12 months from completion of therapy in both groups. The greatest reduction in yearly decrements in height SDS occurred in the first year after diagnosis. Patients of group I treated with prophylactic cranial irradiation with total dose 12 Gy presented significantly higher catch-up growth than treated with dose 18 Gy one year from completion of cancer therapy (p=0.001). Growth hormone deficiency in children of both groups was not observed. Bone age deceleration was retarded one year or more in both groups (group I p=0.025, group II p=0.001). CONCLUSIONS: 1) The chemotherapy and radiotherapy contribute to growth retardation in the first year of treatment in both groups 2) It seems that prophylactic cranial irradiation in children with ALL with total doses 12 and 18 Gy does not contribute to retardation of growth velocity after cancer treatment 3) Endocrine follow-up should be introduced in order to detect and treat complications as early as possible.

[Thyroid autoantibodies in children with recently diagnosed insulin-dependent diabetes mellitus]. / Przeciwciala przeciwtarczycowe u dzieci ze swiezo rozpoznana cukrzyca typu 1.

UNLABELLED: 119 newly diagnosed insulin dependent diabetes mellitus (IDDM) children - 59 girls and 60 boys aged 2-16 years were examined for the presence of thyroid autoantibodies (TA), T3, T4 and TSH levels. Estimate was repeated after 2 years with sonography of thyroid in children with the presence of TA. At onset of IDDM positive TA were found in 38 (32%) diabetic children - in 20 boys and 18 girls. The highest frequency of TA was stated in children aged 10-13 years. One boy with high TA was identified to have primary hypothyroidism caused by Hashimoto thyroiditis. In the remaining of children T3, T4 and TSH were in normal ranges and there was no significant difference between TA positive and TA negative group. In the examination after 2 years TA were found in 24% of children who were positive at onset. There were significant differences in T3 and T4 levels between children with positive TA at onset of IDDM and after 2 years. Thyroid volume by ultrasound was significantly higher in AT positive children (10.79+/-4.1 ml) than in ones who were TA negative 2 years later (7.99+/-4.3 ml). In half of TA positive children ultrasound changes were observed. CONCLUSIONS: High frequency of TA at onset of IDDM in children was observed. Careful follow-up examinations of thyroid function and ultrasound in that group are advised.

[Newborn mass screening for congenital hypothyroidism in Gdansk region]. / Badania przesiewowe noworodków w kierunku wrodzonej niedoczynnosci tarczycy w regionie gdanskim.

Newborn mass screening for congenital hypothyroidism was introduced in the Gdansk region in 1993. During the period 1993-2001 180708 newborns were screened. The prevalence of congenital hypothyroidism was 1:4203. The prevalence of transient hyperthyrotropinemia before implementation of the program of universal salt iodization was 0.016%, after that 0.003%.The request rate for repeated samples was 0.47% before and 0.24% after salt iodization.