The First Definition of Pulmonary Component of Hypereosinophilic Syndrome: Bronchial Casts.

Hypereosinophilic syndrome is a heterogeneous disease characterized by eosinophilic tissue inflammation and eosinophilia. Pulmonary involvement could be seen in up to 55% among children with hypereosinophilic syndrome. A 3-year-old boy with chronic hypereosinophilia and respiratory complaints was diagnosed with idiopathic hypereosinophilic syndrome. Atelectasis was detected in the radiological evaluation, and bronchial casts with eosinophilic structures were removed by bronchoscopy. Steroid, inhaled hypertonic saline, inhaled bronchodilator, inhaled corticosteroid, and leukotriene receptor antagonist were used for 1 year in the management of hypereosinophilic syndrome, and related eosinophilic casts and repetitive bronchoscopies were administered for removal of the casts. The patient was successfully managed with an inhaled N-acetyl cysteine treatment. In children, the long-term prognosis of hypereosinophilic syndrome is uncertain. Comprehensive diagnostic tests are required for the early diagnosis and management of pediatric hypereosinophilic syndrome. In the presented case, the rare occurrence of pulmonary involvement of hypereosinophilic syndrome in a 3 year-old-boy with recurrent hypereosinophilic casts and its management were discussed.

Assessment of the Nutritional Status, Bone Mineralization, and Anthropometrics of Children with Thalassemia Major.

OBJECTIVE: Children with thalassemia major (TM) are prone to growth failure and micronutrient deficiency. Thus, this study aimed to evaluate nutritional status, anthropometrics, and bone mineralization defects in patients with regular blood transfusion. METHODS: Data obtained were analyzed by evaluating laboratory tests, anthropometric measures, and bone mineral density. RESULTS: This study included 29 patients (62% male and 38% female) with a mean age of 12.26±4.74 years, mean pre-transfusion hemoglobin of 8.64±1.01 g/dL, and mean serum ferritin of 1158.6±556.8 ng/ mL. Vitamin D (72.4%), selenium (72.4%), and folate (37.9%) deficiencies were most frequent. Hypocalcemia was observed in 17.2%, hypomagnesemia in 3.5%, and decreased ceruloplasmin in 10.3% of patients. Folate was higher between 2 and 6 years old (p=0.028). Ceruloplasmin was higher between 6 and 10 years old (p=0.018). Selenium was significantly higher in patients with a ferritin of ≥1,500 (p=0.008). No significant ferritin-related differences were found in other micronutrients (p>0.05). Body mass index (BMI) were <5 percentile (p) in 31% of patient, whereas none was >95 p. Height in 24.5% and weight in 20.7% of patients were <3 p, whereas none with >97 p. BMI of patients aged 10-18 years was significantly higher (p=0.001). Anthropometric percentiles did not significantly differ in the mean serum ferritin and micronutrient levels. Hypoparathyroidism was observed in 13.8% and hypothyroidism in 3.5% of patients. Low bone density was detected in 14.8% (2 osteopenic and 2 osteoporotic) of patients. Bone mineral density did not significantly differ in the ferritin and micronutrient levels. CONCLUSIONS: Nutritional support and deficiency prevention are important to minimize the burden of complications and increase the life expectancy and quality in patients with TM.

Chylous ascites after lymphadenectomy in a Wilms` tumor patient.

Yörük MA, Erat-Nergiz M, Timur Ç, Canbolat-Ayhan A, Ergüven M. Chylous ascites after lymphadenectomy in a Wilms` tumor patient. Turk J Pediatr 2018; 60: 436-438. Wilms` tumor is the most common renal malignancy in children and the fourth most common childhood cancer. It accounts 6-7% of all childhood malignancies. Surgical resection is an important therapy option and transabdominal or transperitoneal resection with lymph node sampling is preferred. Development of chylous ascites following intraabdominal or retroperitoneal resection in pediatric age group generally results from extensive lymph node dissection, accidental ligation or interruption of lymphatic ducts. Diseases or conditions affecting abdominal and/or retroperitoneal lymph nodes may cause chylous ascites. Postoperative chylous ascites is associated with significant morbidity and may cause mechanic, nutritional and immunological complications. In the present study, a 16-month-old infant with Stage IV Wilms` tumor who developed chylous ascites after left nephrectomy and lymphadenectomy will be presented; chylous ascites treatment with enteral nutrition and surgical treatment approach for Wilms` tumor will be reviewed.

A retrospective analysis of complications observed in children with acute lymphoblastic leukemia during chemotherapy.

BACKGROUND: We aimed to evaluate the complications that we observed in children with acute lymphoblastic leukemia (ALL) during the remission induction, consolidation, and reinduction phases of chemotherapy retrospectively. METHODS: We analysed the clinical records of 128 patients with ALL who were diagnosed and treated in the Department of Pediatric Hematology of Istanbul Medeniyet University Goztepe Training Hospital between August 2009 and April 2014 to document the acute complication other than febrile neutropenia episodes, which developed during the induction, consolidation and reinduction phases of chemotherapy. RESULTS: We documented 279 complications. Of these, 53.05% were in males, 46.95% in females; 32.26% were in standard-risk, 45.52% in medium-risk, 22.22% in high-risk group of patients. Common documented events were pneumonia (25%), therapy-induced hyperglycemia (16.40%) therapy-related hepatitis (15.6%), generalized tonic-clonic seizures (14.8%), anaphylaxis to asparaginase (14.1%), hypertension (13.3%) varicella zoster virus (VZV) infection (13.3%), renal tubulopathy (12.5%). Time of complications was induction phase in 32.62%, consolidation in 19.35%, HR blocks in 18.28%, reinduction 29.75% during the phases. Mortality rate due to complications was 13.28%. CONCLUSIONS: Therapy-related complications can limit the survival rates in children with ALL. To minimize the treatment burden, even very rare complications must be considered and treated promptly with a multidisciplinary approach.

Myeloid sarcoma presenting with leukemoid reaction in a child treated for acute lymphoblastic leukemia.

Background. Myeloid sarcoma is an extramedullary neoplasm of immature myeloid cells. Our study reports a presentation of myeloid sarcoma which presented with severe leukemoid reaction as a secondary malignancy in a patient who was treated for acute lymphoblastic leukemia previously. The case emphasizes the difficulties in diagnosis of patients who do not have concomitant leukemia. Case Presentation. A 6-year-old girl who was treated for acute lymphoblastic leukemia previously presented with fatigue, paleness, and hepatosplenomegaly. Peripheral blood smear and bone marrow aspirate examination did not demonstrate any blasts in spite of severe leukemoid reaction with a white cell count 158000/mm(3). FDG/PET CT revealed slight uptake in cervical and supraclavicular lymph nodes. Excisional lymph node biopsy was performed from these lymph nodes and it showed myeloid sarcoma. Conclusion. Myeloid sarcoma can develop as a secondary malignancy in children who are treated for acute lymphoblastic leukemia. It can be associated with severe leukemoid reaction and diagnosis may be difficult if there is not concomitant leukemia. PET/CT is helpful in such cases.

Spontaneous intestinal perforation: an atypical presentation of neutropenic enterocolitis-a case report.

Background. Neutropenic enterocolitis is one of the most common gastrointestinal complications seen in patients who are receiving chemotherapy for leukemia. Severe neutropenia is the main underlying factor of this pathology. It is characterized by fever and abdominal pain. Case Presentation. Herein, we report a case of neutropenic enterocolitis which presented with intestinal perforation in an afebrile patient who was diagnosed with acute lymphoblastic leukemia and was receiving induction chemotherapy. Conclusion. We aimed to emphasize the importance of enterocolitis and increase awareness against such severe complications which could have unexpected presentations.