Pediatrician! Do you know the symptoms of DRESS syndrome? A case report of a 4-year-old girl.

Drug Rash with Eosinophilia and Systemic Symptoms (DRESS) syndrome is a severe drug-induced hypersensitivity syndrome and may be observed after administration of many drugs. Clinical symptoms usually occur 2 to 8 weeks after drug introduction. Because DRESS syndrome is potentially life threatening, it is especially important to diagnose it early. Withdrawal of the drug which induced symptoms is the most important therapeutic option. DRESS syndrome appears mostly in adults. There are relatively few articles on the DRESS syndrome in children. The article presents a case of a 4-year-old girl with a life-threatening clinical course of DRESS syndrome with massive pulmonary involvement. The knowledge of DRESS syndrome clinical symptoms is essential for doctors of various specialties. It is especially important that general practitioners, pediatricians, and pediatric neurologists should be able to take this life-threatening syndrome into consideration for differential diagnosis.

Analysis of selected factors influencing seroma formation in breast cancer patients undergoing mastectomy.

INTRODUCTION: The aim of the work was to analyze the impact of selected factors on the incidence of seroma formation in breast cancer patients undergoing mastectomy. MATERIAL AND METHODS: One hundred and fifty breast cancer patients were prospectively enrolled in the study. All patients had mastectomy performed using the same operative technique with electrocoagulation. The amount of seroma formed after surgery and its duration were correlated with selected demographic, clinical and pathological parameters. RESULTS: The cumulative total seroma volume collected by the end of treatment was higher and the overall time of seroma treatment was longer in patients over the age of 60 years (p = 0.001 and p = 0.001 respectively). Duration of seroma was significantly longer in obese patients (p = 0.036). The cumulative total seroma volume collected by the end of treatment was higher and the overall time of seroma treatment was longer in patients who had over 130 ml of lymph drained during the first 24 postoperative hours (p < 0.001 and p = 0.001 respectively). Additionally, longer duration of seroma was observed in patients with pathological stage I and II according to TNM-UICC (p = 0.042) and in patients with ≥ 1200 g weight resected of mammary gland (p = 0.05). CONCLUSIONS: Age and obesity are important prognostic factors influencing seroma formation in breast cancer patients undergoing mastectomy. The amount of lymph formed during first postoperative day may have predictive value in assessing cumulative total seroma volume collected during treatment and its overall duration.

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.

Ultrasound assessment of thrombotic complications in pediatric patients with tunneled central venous catheters.

Central venous access consists in inserting a vascular catheter to the vena cava and placing its tip in the vicinity of the opening to the right atrium. In the patients of the Clinic of Pediatrics, Hematology and Oncology at the Academic Clinical Centre of the Medical University in Gdansk, such implantation procedures are conducted 40-50 times in a year using Broviac/Hickman catheters that are placed in the subclavian vein. In the Ultrasound and Biopsy Laboratory at the clinic mentioned above, approximately 200-250 examinations have been conducted since 2005 to assess the central venous access. Implantation of a catheter considerably increases the comfort of patients who require a long-term venous access. Nevertheless, it is an invasive procedure, burdened with a risk of numerous, early and late complications. The late complications are associated with implanted catheters and include catheter-related thrombosis. The aim of this paper was to present three patients of the Clinic of Pediatrics, Hematology and Oncology at the Academic Clinical Centre of the Medical University in Gdansk, in whom thrombotic complications occurred as a result of long-term central venous catheters. The paper also discusses the possibilities of using sonography in the assessment of such complications. In the presented patients, it was possible to determine the size and localization of a thrombus which enabled effective treatment in two cases. The pathomechanism of catheter-related thrombosis was explained and the risk factors of such complications were discussed. The attention was paid to the necessity of conducting ultrasound examinations in pediatric patients with inserted catheters as soon as the first symptoms of thrombosis appear. Based on own observations and despite the lack of validation of ultrasound imaging in the assessment of central catheters, we believe that this method is highly promising and can be recommended for the assessment of thrombotic complications in pediatric patients with central venous catheters.

Transient hyperglycaemia - an underestimated problem of paediatric oncohaematology.

INTRODUCTION: The majority of hyperglycaemic incidents in oncohaematological patients treated with glucocorticosteroids remain undiagnosed. The aim of our study was to work out a detailed protocol for the control of carbohydrate metabolism and to evaluate whether such a protocol can help in diagnosis of carbohydrate metabolism disturbances in oncohaematological paediatric patients. MATERIAL AND METHODS: A one hundred and twenty-eight children treated for proliferative diseases of the haematopoietic system and severe aplastic anaemia with therapeutic protocols including glucocorticosteroids were divided into two groups. Group I consisted of 70 children, whose blood glucose was evaluated on random occasions (retrospective analysis). Group II consisted of 58 children included in the programme of intensive carbohydrate metabolism control (prospective analysis). We compared the incidence of hyperglycaemia in both groups as well as the number of hyperglycaemic incidents per individual therapeutic protocol. RESULTS: A significantly higher incidence of transient hyperglycaemia was noted in oncohaematological patients in the programme of early carbohydrate metabolism disturbances diagnosis than in the other group (22.4% vs. 5.7% respectively; p = 0.008), especially in patients treated with the ALL IC-BFM 2002 protocol for the high risk group (arm A and B), the ALL-REZ BFM 2002 protocol, and in a heterogenous group of children (protocols ALCL 99, Euro-LB02, Interfant-06, WPSAA) (p = 0.042, 0.021 and 0.002, respectively). CONCLUSIONS: The improvement of transient hyperglycaemia detection may constitute the first step towards the reduction of unfavourable consequences of hyperglycaemia. Prospective studies are required to demonstrate the influence of normal carbohydrate metabolism on the frequency of infectious complications in this group.

A randomized, double-blind, placebo-controlled trial of preemptive analgesia with bupivacaine in patients undergoing mastectomy for carcinoma of the breast.

BACKGROUND: In this prospective, randomized, placebo-controlled, double-blinded clinical trial we tested the hypothesis that preemptive analgesia with bupivacaine applied in the area of the surgical incision in patients undergoing mastectomy for breast cancer would reduce post-operative acute pain and would reduce the amount of analgesics used during surgery and in the post-operative period. MATERIAL/METHODS: Participants were assigned into 1 of 2 groups--with bupivacaine applied in the area of surgical incision or with placebo. We assessed the intraoperative consumption of fentanyl, the postoperative consumption of morphine delivered using a PCA method, and the subjective pain intensity according to VAS score reported by patients in the early post-operative period. RESULTS: Out of 121 consecutive cases qualified for mastectomy, 112 women were allocated randomly to 1 of 2 groups--group A (bupivacaine) and group B (placebo). The final study group comprised 106 breast cancer cases. Between the groups, a statistically significant difference was observed with respect to: lower fentanyl consumption during surgery (p = 0.011), lower morphine (delivered by means of a PCA) consumption between the 4-12th postoperative hours (p = 0.02) and significantly lower pain intensity assessed according to VAS score at the 4th and 12th hours after surgery (p = 0.004 and p = 0.02 respectively) for the group A patients. CONCLUSIONS: Preemptive analgesia application in the form of infiltration of the area of planned surgical incisions with bupivacaine in breast cancer patients undergoing mastectomy decreases post-operative pain sensation, limits the amount of fentanyl used during surgery, and reduces the demand for opiates in the hours soon after surgery.

Candidemia in children after complex congenital heart defects surgery treated with caspofungin--our own experience and a review of literature.

BACKGROUND: Invasive fungal infections due to Candida species constitute an increasing clinical problem. There are no guidelines for the management of candidemia in children undergoing surgical procedures for congenital heart defects (CHD). The aim of the study was to draw attention to the problem of candidemia in children who are operated on due to congenital heart defects. MATERIAL/METHODS: We retrospectively analyzed medical documentation of 307 children with congenital heart defects treated in 1 clinical centre in Poland, from whom we selected those diagnosed with candidemia during the postoperative period. Next, we analyzed in detail the clinical course of invasive candidiasis in each individual and we performed an analysis of risk factors that lead to candidemia. RESULTS: In the analyzed period, among 307 children who underwent surgical procedures, we observed 2 cases of candidemia (0.65%) which were effectively cured with caspofungin. No adverse effects were observed after treatment with the drug. CONCLUSIONS: Candidemia in children who undergo surgical treatment of congenital heart defects is an important factor that can influence final clinical results. Caspofungin may be an effective therapeutic option when treating candidemia in children after extensive cardiosurgical procedures.

[Spontaneous splenic rupture in the course of infectious mononucleosis]. / Samoistne pekniecie sledziony w przebiegu mononukleozy zakaznej.

Spontaneous splenic rupture (SSR) in the course of infectious mononucleosis (IM) is a rare but potentially fatal complication. Mortality rate is relatively high, therefore emergency splenectomy is a life-saving intervention. In case of undergoing urgent operation there is no possibility to initiate proper prophylaxis of overwhelming infection. The humoral and cellular immunologic response impairment is a reason for life-threatening complications of splenectomised person. Asplenic children should receive infection prophylaxis immediately post splenectomy. We report two cases of splenic rupture inpatients with IM. The prevention of infection was initiated in both children. The mainstays of prophylaxis are: immunization, chemoprophylaxis and education. Complex information concerning asplenia-related subjects should be provided for patients and their parents.

[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.

[Anhydrotic ectodermal dysplasia as cause of recurrent hyperthermia in a 5 month old infant]. / Anhydrotyczna postac dysplazji ektodermalnej jako przyczyna nawracajacej goraczki u pieciomiesiecznego niemowlecia.

The X-linked anhydrotic ectodermal dysplasia is a rare disease in which defects in development of ectodermal derivatives are observed. This syndrome is clinically characterized by total or partial anodontia, characteristic physionomy and absent or reduced sweating. Recurrent fever was a clue to the disease diagnosis in 5 month old infant. The diagnosis was confirmed by the mutation of EDA exon 9.

[Non-Hodgkin lymphomas of the nasopharynx in children--diagnostic and therapeutic difficulties]. / Chloniaki nieziarnicze nosogardla u dzieci--trudnosci diagnostyczne i terapeutyczne.

UNLABELLED: About 7% of all childhood cancers comprise non-Hodgkin's lymphoma (NHL). NHL are heterogenous group of neoplasms deriving from lymphatic system (cell B and T). B-cell NHL characterize by high malignancy, but coincidentally good reaction for treatment. In about 20% primary tumour is localised within head and neck, and nasopharynx lymphomas comprise 10%. This location maintains the biggest diagnostic and therapeutic difficulties because of tumours of this site proliferate in the region of frequent infections postponing a proper diagnosis and the local control after complete treatment is difficult. AIM OF THE STUDY: The authors analyse clinical symptoms before diagnosis, the incidence of nasopharynx lymphomas, histopathological type of neoplasm, clinical stage, results of treatment. MATERIAL AND METHODS: The study includes 97 patients who were treated because of lymphomas between 1993-2002. The character of clinical symptoms and their duration, histopathological type of lymphomas, results of treatment were analysed. RESULTS: The primary nasopharynx location was assessed in 9 patients (9.3%). Sex: 7 boys, 2 girls, age: 2-17 years. The duration which elapsed from initial clinical symptoms to diagnosis was 2-10 weeks. The histopathological assessment in 6 children was Burkitt lymphoma and in 3 children--Burkitt-like lymphoma. Metastases: CNS--1 patient, bone marrow--1 patient, abdomen--1 patient. Treatment was performed according to LMB-89 protocol. RESULTS: First complete remission--7 patients; second complete remission--2 patients. CONCLUSIONS: Lymphomas of nasopharynx cause diagnostic problems because of their early stage pseudo-inflammatory manifestation. Special attention should be paid to perform imaging studies (MRI/CT), which are useful in the reaching the proper diagnosis. The radiologic evaluation of primary lesion is still difficult. In the doubtful cases, the surgery and histopathological examination are necessary.

[Vitamin B(12) deficiency anaemia in a 7.5 months old girl]. / Niedokrwistosc z niedoboru witaminy B(12) u 7.5-miesiecznej dziewczynki.

Vitamin B(12) stores at birth are adequate for infants until the end of the first year of life even if it 's concentration in maternal breast milk is low. However, there are some situations in which infants have depleted vitamin B(12) stores and in addition have a low dietary intake of cobalamin. Vitamin B(12) depletion occurs in infants who are exclusively breastfed by mothers who have unrecognized pernicious anemia or are strict vegetarians for many years. In those infants symptoms of bone marrow disfunction and impaired development of the central nervous system appear already in the first months of life. Unrecognized cobalamin deficiency may lead to serious neurologic consequences, and even to the death of the child. The authors present a case of a 7.5 month old girl admitted to the Department of Paediatrics, Haematology, Oncology and Endocrinology. Gdansk Medical University suspected of acute leukaemia. Based on a detailed diagnostic procedure a final diagnosis of vitamin B(12) deficiency anaemia was established. The child was exclusively breast-fed. Results of investigations into the reason for cobalamin deficiency in the patient s organism. Led to the diagnosis of pernicious anaemia in the mother. Such a suspicion had been made during pregnancy, but no continuation of investigations nor appropriate treatment were implemented. After treatment with vitamin B(12) supplements and modification of the diet the patient improved quickly and remarkably. A few months follow-up was enough to observe remarkable improvement of psychomotor development of this child.

[X-linked agammaglobulinemia (XLA) associated with agranulocytosis--case report]. / Agammaglobulinemia sprzezona z chromosomem X z towarzyszaca agranulocytoza--opis przypadku.

In this case study authors presented the clinical characteristics of X-linked agammaglobulinemia (XLA) associated with agranulocytosis diagnosed in a 2-year-old boy. Affected child lacked circulating mature B cells, presented low levels of serum immunoglobulins, but did not suffer from recurrent bacterial infections. XLA is a primary immunodeficiency caused by a defective tyrosine kinase (Btk) in B cells. Our patient and his mother have a mutation in the BTK gene, described as W281X. During therapy with intravenous gammaglobulin, the boy has not experienced agranulocytosis. It is important to consider a primary immunodeficiency diagnosis when a child presents agranulocytosis or neutropenia and a recurrent infectious disease.

[Recombinant erythropoietins--an alternative therapy to red cell blood transfusions in infants with hereditary spherocytosis]. / Zastosowanie erytropoetyny w leczeniu sferocytozy wrodzonej o ciezkim przebiegu klinicznym u niemowlecia--alternatywa dla transfuzji masy erytrocytarnej?

Hereditary spherocytosis is the most frequently occurring haemolytic anaemia. Some patients manifest clinical signs during the neonatal period and require transfusion. A case of hereditary spherocytosis in neonate is presented. The use of rhEPO during the first months may be an alternative therapy to red cell transfusion.

[LCHAD (long-chain 3-hydroxyacyl-CoA dehydrogenase) deficiency as a cause of sudden death of a three months old infant]. / Deficyt lchad (dehydrogenazy 3-hydroksyacylo-CoA dlugolancuchowych kwasów tluszczowych) jako przyczyna naglego zgonu trzymiesie czego niemowlecia.

LCHAD deficiency is a rare, autosomal recessive congenial defect of fatty acid oxidation. In this disorder high mortality is observed. In some cases patients die during the first episode of this disease, without the diagnosis of LCHAD. We report the case of a three months old boy, whose sudden death was the result of LCHAD deficiency.

[Two cases of congenital toxoplasmosis with a central nervous system damage--delayed diagnosis]. / Dlaczego nadal rozpoznajemy toksoplazmoze wrodzona z zajeciem osrodkowego ukladu nerwowego u noworodków? Opis dwoch przypadków.

OBJECTIVES: The intrauterine toxoplasma gondii infection is linked with a high risk of central nervous system/CNS/damage in the fetus. Why, despite the wide knowledge of this neurodegenerative disease prophylaxis and therapy, do we still meet neonates with the CNS damage? DESIGN, MATERIALS AND METHODS: Authors present two cases of congenital toxoplasmosis with the CNS involvement. RESULTS: One of the cases was identified as the internal hydrocephalus during a prenatal ultrasound examination but no further diagnostics tests were undertaken. The congenital toxoplasmosis and severe CNS damage was diagnosed post delivery. In the other case some clinical symptoms of the CNS infection appeared in the neonatal period. Now this patient is eight months old and presenting mild motor developmental delays. CONCLUSIONS: 1. There are many clinical symptoms of the congenital toxoplasmosis, that can occur in the prenatal/case 1/and postnatal/case 2/period. 2. No serologic tests for toxoplasmosis were performed in mothers during pregnancy, what delays treatment and diagnosis of sick children. 3. Fetus presenting the CNS anomalies identified during ultrasound examinations should be immediately diagnosed.