Suna Emir

Subcutaneous fat necrosis of the newborn associated with anemia.

Abstract: Subcutaneous fat necrosis (SFN) of the newborn characteristically affects full‐term infants who have experienced perinatal distress, such as hypothermia, obstetric trauma, or asphyxia. We report a newborn who had pallor, deep breathing, and severe anemia immediately after birth. She developed SFN on the fourth postnatal day. Her condition improved after blood transfusions and the skin lesions resolved in 6 weeks. This appears to be the first report of SFN associated with anemia.

Gastroduodenal lesions and Helicobacter pylori in children with end-stage renal disease

Abstract Thirty-seven children with end-stage renal disease were evaluated for gastroduodenal lesions by upper gastrointestinal endoscopy between January 1993 and January 1998. The mean (±SD) age of the patients was 14.3±2.4 years (range 9–17 years). Endoscopic examination was abnormal in 17 patients (46%). The lesions were antral gastritis plus bulbitis (n=6), nodular bulbitis (n=4), antral gastritis (n=4), and duodenal ulcer (n=3). Fifteen patients had symptoms related to gastroduodenal disease, whereas 22 patients were asymptomatic at the time of endoscopic examination; 80% of the symptomatic and 23% of the asymptomatic patients had gastroduodenal lesions on endoscopy. Antral mucosal biopsy was taken from 26 of 37 children for the detection of Helicobacter pylori by the urease test. H. pylori was detected in 10 of 16 patients with gastroduodenal lesions (8 symptomatic, 2 asymptomatic). None of the patients with normal endoscopic examination were positive for H. pylori. Thus, we have demonstrated a significant number of gastroduodenal lesions and their frequent association with H. pylori in our pediatric renal transplant candidates. Our results emphasize the importance of gastrointestinal evaluation in these patients. Endoscopic examination should be considered in symptomatic patients and in areas where H. pylori is endemic.

Use of plerixafor for peripheral blood stem cell mobilization failure in children.

BACKGROUND Peripheral blood stem cell mobilization is usually performed following chemotherapy plus G-CSF in children. This standard approach may not be successful in some heavily pretreated patients undergoing mobilization. Plerixafor (AMD3100) has been used in adults as a second line mobilizing agent. Our aim is to analyze our experiences with plerixafor in children. METHODS We retrospectively evaluated three children who received plerixafor as a second line stem cell mobilizing agent in our department in the 2010-2012 period. Data including age, sex, diagnosis, previous chemotherapy, radiotherapy details, previous harvest attempts, adverse reaction, and harvest outcome were analyzed. RESULTS We used plerixafor in combination with G-CSF and chemotherapy or with only G-CSF seven times in three patients. All three patients were treated with different multiple chemotherapy regimens prior to stem cell harvest and failed earlier mobilization with chemotherapy plus G-CSF. The diagnoses were relapsed Hodgkin lymphoma in two and recurrent Ewings sarcoma in one patient. We used plerixafor in combination with G-CSF and chemotherapy or with only G-CSF seven times in three patients. The harvest was successful in four of seven attempts. No adverse reaction was observed in the patients. CONCLUSION The success rate is four out of seven attempts (57%) in our group. Although the data regarding the use of plerixafor in children is scarce, our experience also supports its use in poor mobilizer children. The use of plerixafor in children results in effective increases in peripheral stem cell counts and reduces the risk of mobilization failure.

A Rare Diagnosis In Children: Isolated Pulmonary Langerhans Cell Histiocytosis.

We read with great interest the article by Asilsoy et al. (1) about pulmonary Langerhans cell histiocytosis in children recently published in your journal. In this regard, we would like to give our contribution to the management of these cases. Langerhans cell histiocytosis (LCH) is a rare disorder in childhood and isolated pulmonary LCH is extremely rare in children, as it stated in the article (1, 2). The most common clinical symptoms suggesting pulmonary involvement of LCH are tachypnea, chest pain and chronic or persistent cough (3). We want to present a child case of isolated pulmonary LCH in this letter unlike the four cases with multisystem involvement presented in the study of Asilsoy et al. (1), and also we aim to draw attention to this diagnosis which should kept to in mind especially for the frequently encountered cases of chronic cough in clinical practice, and to emphasize the thoracic imaging in diagnosis of chronic cough cases even though completely normal physical examination. The case that we have managed was a previously healthy 14-year-old male who was admitted because of prolonged cough. On patient’s history, the cough began after complaints of fatigue and runny nose 1 month ago, then was accompanied by phlegm without fever. Any improvement in complaints was not observed despite non-specific antibiotherapy before admission. On physical examination, the patient had normal vital signs and all systemic examinations were normal. Hemoglobin, white blood cell count and platelets were in normal limits. Erythrocyte sedimentation rate was 31 mm/h (N: 0–20) and C-reactive protein was 2.98 mg/dL (N: 0–0.8) with normal blood biochemistry. Bilateral diffuse patchy infiltrates were located in the chest radiograph (Fig. 1A). On chest computed tomography, multiple cystic lesions with marked walls in different sizes in both lungs common in upper lobes and more pronounced in peripheral areas were observed, and several pieces of lymph nodes in both hilum of which the largest one 18 3 9 mm in size were detected (Fig. 1B). The majority of the possible diseases in differential diagnosis such as immunodeficiency syndromes, tuberculosis, respiratory viral agents and alpha-1 antitrypsin deficiency were excluded. In open lung biopsy for definitive diagnosis, LCH with the characteristic reniform CD1a, S100 and Langerin positive Langerhans cells were detected (Fig. 2A,B). Whole body bone survey, bone marrow biopsy, abdominal ultrasound and echocardiography were performed for other systemic involvements of LCH, and all were detected completely normal. The patient was considered as isolated pulmonary LCH, and the treatment was initiated.

Pulmonary arterial hypertension in a child with stage-IV neuroblastoma after autologous hematopoietic stem cell transplantation and review of the literature.

PH is a rare condition with high mortality rate after pediatric HSCT. As clinical presentation is non‐specific and may mimic other conditions, a high degree of suspicion is required for diagnosis. Here, we present a patient with stage‐IV neuroblastoma who developed PAH after autologous HSCT. After exclusion of other causes of PH, we regarded that this condition was secondary to HSCT.

Gastric Schwannoma Without Neurofibromatosis in a 16-Year-Old Adolescent.

BACKGROUND Schwannomas are benign, slowgrowing, encapsulated nerve-sheath tumors arising from Schwann cells. Gastric schwannomas occur more frequently in female patients in the fifth to the sixth decades of life and account for only 0.2% of all gastric tumors and 4% of all benign gastric neoplasms in adulthood. Schwannomas are rare in children without neurofibromatosis (NF).1–3 We describe a 16-year-old female patient diagnosed with gastric schwannoma.

Teratoid Wilms’ Tumor Exhibiting Extensive Squamous Differentiation

Teratoid Wilms’ tumor is a rare renal tumor. Herein, we report an unusual variant of such tumor which simulated renal teratoma because of abundant keratinized squamous epithelium within the tumor.

Anaplastic lymphoma kinase gene expression in small round cell tumors of childhood--a comparative ımmunohistochemical study.

The focus of this study was to investigate anaplastic lymphoma kinase (ALK) expression by immunohistochemistry using a highly specific antibody. Distribution and frequency of ALK expression may provide a clue for ALK inhibitor use in small round cell tumors of childhood. The study group involved 76 small round cell tumors of childhood, which composed of 11 rhabdomyosarcomas, 13 Wilms tumors, 7 Ewing sarcoma/primitive neuroectodermal tumors, 34 peripheral neuroblastic tumors, and 11 acute lymphoblastic lymphoma. Anaplastic lymphoma kinase protein expression in small round cell tumors of childhood is poorly described in the literature. The findings of our study highlight a potential and possible role of targeting ALK in pediatric solid tumors by using ALK immunohistochemistry. Anaplastic lymphoma kinase may also have an oncogenic role in rhabdomyosarcomas and peripheral neuroblastic tumors, and they may possibly be treated with ALK inhibitors. Anaplastic lymphoma kinase expression in Wilms tumors is not reported in the literature, previously. Our study evaluated ALK expression in Wilms tumor samples.

Total estimated effective doses from radiologic imaging modalities of children with cancer: a single center experience

BackgroundRecently, awareness of the cumulative radiation exposure for pediatric oncology patients has been increasing, together with increased survival rates and longer life expectancy. The aim of our study was to quantify the amount of ionising radiation from imaging modalities of pediatric oncology patients.MethodsEighty-eight patients who were diagnosed with childhood cancer and followed up for 5 years between 2004-2014 in our center were included in the study. Patients’ medical files were reviewed retrospectively for imaging history in the first 5 years after diagnosis. Total estimated effective doses from radiologic imaging modalities were determined. Also, the basic demographic data, histologic type, stage, and outcomes of disease were collected for all patients.ResultsThe individual total estimated effective doses ranged from 8.73 to 167 mSv, with a median of 62.92 mSv. Computed tomography was the greatest contributor of total effective doses. The doses ranged 21.45-113.20 mSv (median: 62.92 mSv) in Hodgkin lymphoma, 12.53-167.10 mSv (median: 52 mSv) in non-Hodgkin lymphoma, 4.13-172.98 mSv (median: 52 mSv) in neuroblastoma, 31-149.89 mSv (median: 63.10 mSv) in Wilms’ tumor, 11.50-73.72 mSv (median: 36.90 mSv) in germ cell tumor, 26.46-125.86 mSv (median: 80.90 mSv) in other solid tumor and 0.02-13.31 mSv (5.25 mSv) in brain tumor subgroup. Twenty-two children (25%) died with progressive disease during the 5-year follow-up period.ConclusionsSimilar to previous studies, the total estimated effective doses in children with cancer have been found various according to diagnosis, stage and clinical course. To clarify the harmfull effects of radiation burden, prospective studies should be conducted in children with cancer.

Squamous cell carcinoma associated with xeroderma pigmentosum: an unusual presentation with a tremendously huge mass over the face and paraneoplastic hypercalcemia-hyperleukocytosis

Emir S, Hacısalihoğlu Ş, Özyörük D, Kaçar D, Erdem A, Karakuş E. Squamous cell carcinoma associated with Xeroderma pigmentosum: an unusual presentation with a tremendously huge mass over the face and paraneoplastic hypercalcemia-hyperleukocytosis. Turk J Pediatr 2017; 59: 711-714. Xeroderma pigmentosum (XP) is a rare autosomal recessive disorder that results from genetic defects in DNA repair and manifests with a marked hypersensivity to ultraviolet rays. Children with X-P are at high risk of developing skin cancers. On the other hand, hypercalcemia-hyperleukocytosis is a rare paraneoplastic syndrome in children with cancer compared to adults. Here, we report a five-year-old female with X-P and squamous cell carcinoma (SCC). The patient presented with a necrotic, ulcerating huge mass sized 20x15x10 cm involving the right half of the face. She had a history of increased freckling over the face since the age of two years. Her other cutaneous findings are dryness of skin, photosensitivity, freckling and telengiectasis all over the body. A diagnosis of Xeroderma pigmentosum was made based on clinical features. She also had high fever, anemia, hyperleukocytosis, thrombocytosis and hypercalcemia. After pathological diagnosis of squamous cell carcinoma, she was treated with chemotherapy. All the symptoms and signs resolved dramatically with the initiation of chemotherapy. Our case is an example of early development of massive disfiguring SCC in children with undiagnosed and untreated X-P. Although we could not prove the paraneoplastic nature of hypercalcemia-hyperleukocytosis, dramatic response to the chemotherapy may be an evidence for paraneoplastic origin.