Ayşen Aydoğan

Hepatic Adenomatosis in a 7-Year-Old Child Treated Earlier With a Fontan Procedure

Patients who undergo a Fontan procedure experience some degree of liver disease. Hemodynamic changes such as central venous hypertension, depressed dynamic cardiac output, and late ventricular dysfunction combined with long-standing hypoxia preceding the Fontan procedure all are recognized risk factors for hepatic injury. The histopathologic changes associated with cardiac hepatopathy include chronic passive congestion, centrilobular necrosis, and cardiac cirrhosis. However, hepatic adenoma and hepatic adenomatosis (HA) are not well-known pathologies during the course of cardiac hepatopathy. This study focused on a 7-year-old girl with chronic hepatic changes and HA who had undergone a Fontan procedure. Hepatic adenomatosis was diagnosed on the basis of magnetic resonance imaging (MRI) and MRI-guided liver biopsy. To the best of the authors’ knowledge, this case involved the youngest patient with hepatocellular adenomatosis documented in the literature. It was a unique case because the patient experienced HA after a Fontan procedure.

The prevalence of anemia in adolescents: a study from Turkey.

A cross-sectional method was used to study a group of 400 high school students in Kocaeli, Turkey, aged 14 to 16 years, identified among 17,812 high school students. Students from 10 high schools were selected using a random sampling method. Whole blood counts were performed as a screening test for anemia. Serum ferritin levels and, when necessary, hemoglobin electrophoresis were determined for anemic students. Iron medication was prescribed for iron deficiency and genetic counseling was given to adolescents with thalassemia trait. Out of 338 participating students (mean age, 14.72±0.71 y), anemia (hemoglobin <12 g/dL for girls and <13 g/dL for boys) was detected in 17/174 girls (9.7%) and 6/164 boys (3.6%). Iron deficiency anemia was detected in 20/23 (86.9%) of anemic children [15/17 (88.2%) girls and 4/6 (66.6%) boys]. Of 23 students with anemia 2 had β-thalassemia trait and 2 had both iron deficiency, and β-thalassemia trait. Etiology of anemia could not be defined in 1 student. The prevalence of adolescent anemia in Kocaeli is almost equal to that in developed countries.

False positivity of FDG-PET during hemophagocytic lymphohistiocytosis in a child with Hodgkin lymphoma in remission.

To the Editor: We have read with interest the article by Levine et al entitled ‘‘Routine use of PET scans after completion of therapy in pediatric Hodgkin disease results in a high false positive rate.’’ The presence of nonmalignant factors, such as infection and inflammation, can make interpretation of positron emission tomography (PET) scans difficult in patients with Hodgkin lymphoma (HL) off therapy, particularly in pediatric patients. The therapeutic decisions should not be made without histologic confirmation. We reported a false-positive PET result, that is related to hemophagocytic lymphohistiocytosis (HLH) in a child with HL in remission. A 12-year-old boy was admitted to our clinic with pallor and fever. He had been diagnosed as stage IIA nodular lymphocyte predominant type HL, his primary tumor being in cervical and axillary regions. He received 2 courses of ABVD chemotherapy protocol and involved field radiotherapy (25Gy). After completion of therapy, no clinical or laboratory signs and symptoms, that may suggest a relapse of his tumor, have been noted for 3 years. In the last follow-up visit, pallor, fever, and hepatosplenomegaly were noted. There was not any lymphadenopathy. Complete blood count revealed pancytopenia (hemoglobin, 7.7 g/dL; white blood cells, 1800/mL; absolute neutrophil count, 700/mL; and platelets, 65,000/mL). Peripheral blood smear was normal except for hypochromia. Apart from hyperferritinemia (576 mg/L) and hypertriglyceridemia (265mg/dL), blood chemistry profile was normal. Thoracic and abdominal computed tomography (CT) and abdominal ultrasonogram, that were carried out to rule out an HL relapse, were normal except for hepatosplenomegaly. Bone marrow aspiration and biopsy findings were not suggestive of secondary leukemia, myelodysplastic syndrome, or HL involvement; but histiocytes containing red cells and neutrophils in their cytoplasm were noteworthy (Fig. 1). The patient was FIGURE 1. Histiocytes showing phagocytosis of normoblasts, neutrophils, and platelets in bone marrow aspirate (arrows: nuclei of histiocytes). Giemsa 100.

Transient peripheral leukocytosis in children with afebrile seizures.

The purpose of this study was (1) to demonstrate whether peripheral blood leukocytosis accompanies first afebrile seizures without bacterial infection, (2) to investigate the duration of leukocytosis, and (3) to assess the relationship between peripheral blood leukocytosis and seizure characteristics. Complete blood count was routinely obtained from all the patients. Blood and urine cultures were obtained from patients with leukocytosis. On the 24th hour of admission, a second complete blood count was obtained from patients with initial leukocytosis. Sixty-two children aged 4.0 ± 3.6 years (range, 6 months—13 years)—31 boys (50%) and 31 girls (50%)—enrolled in the study. The findings showed that peripheral blood leukocytosis was found in 8% of afebrile children without status epilepticus and 41.6% of afebrile children with status epilepticus. An interesting finding of the study was that peripheral blood leukocytosis accompanied by afebrile seizures subsided in 24 hours. Transient leukocytosis could be found in children with afebrile seizures without bacterial infection.

Left ventricular mechanics are affected in children with celiac disease: A study based on two‐dimensional speckle tracking echocardiography

An association between cardiomyopathy and celiac disease has been reported, but not frequently. We examined the effects on cardiac mechanics in children with celiac disease (CD) by two‐dimensional speckle tracking echocardiography (2DSTE).

Severe iron deficiency anemia due to late presentation of congenital diaphragmatic hernia in a toddler.

To the Editor, A 3-year-old female toddler was referred to our pediatric emergency unit with a 2-week history of fatigue, anorexia, progressive pallor, and vomiting. Medical history showed that iron deficiency anemia was diagnosed one year before and oral iron-sulfate was given. She also had a one year history of intermittent vomiting. Her diet seemed adequate in iron-rich foods. Chest X-ray and abdominal ultrasonographic examination performed in a medical center were normal. Complete blood count findings were as follows: Hb: 1.7 g/dL; WBC count: 8.0 x 109/L; ANC: 4.6 x 109/L; Hct: 6.9%; RBC count: 1.19 x 1012/L; RDW: 20.5; MCV: 58 fL; Plt count: 485 x 109/L; absolute reticulocyte count: 45.5 x 109/L (normal: 50-150 x 109/L). Peripheral blood smear showed hypochromic microcytic red cells, polychromasia, and nucleated red cells. Serum iron was 5 μg/dL, total iron binding capacity was 450 μg/dL, and ferritin was <1 ng/mL. She was hospitalized and given packed red cell transfusion. The following day she suddenly developed respiratory distress after breakfast. Chest X-ray showed a radiolucent lesion in the right paracardiac area (Figure). Congenital diaphragmatic hernia (CDH) was confirmed via barium gastrointestinal X-ray and computed tomography of the chest. During surgery the retrosternal diaphragmatic hernia sack was excised, and the stomach, intestines, and transverse colon were reduced to the abdominal cavity. Nissen fundoplication was also performed in an effort to prevent postoperative gastroesophageal reflux; however, another defect was noted on the left posterolateral side of the diaphragm. The defect was repaired and Thal anterior fundoplication was performed. The patient had both Morgagni and Bochdalek defects Figure X-ray of the thorax showing the radiolucent area with clear borders in the right paracardiac region. At 6 weeks post surgery the patient had no gastrointestinal symptoms, but her Hb dropped to 7.9 g/dL and fecal occult blood test findings were positive (Hct: 25.6%; RBC count: 3.63 x 1012/L; MCV: 70.5 fL; MCH: 21.9; MCHC: 31; RDW: 24; WBC count: 9.8 x 109/L; ANC: 4.3 x 109/L, Plt count: 453 x 109/L; absolute reticulocyte count: 11 x 109/L) [normal 50-150 x 109/L]). Peripheral blood smear showed hypochromic microcytic red cells. Esophagogastroduodenoscopy and esophageal biopsy showed erosions, gastroesophageal reflux, chronic inflammation, and hyperplasia of the epithelium. A proton pump inhibitor, domperidone, and anti-acid medications were started. Iron sucrose was administered twice weekly for 3 weeks and the Hb increased (Hb: 12.2 g/dL; Htc: 34.6%; RBC count: 4.76 x 109/L; MCV: 72fL; MCH: 25.6; MCHC: 35.1; RDW: 17.4; WBC count: 12.2 x 109/L; ANC: 8.2 x 109/L; Plt count: 348 x 109/L, absolute reticulocyte count: 85 x 109/L [normal: 50-150 x 109/L]; ferritin: 16 ng/mL). Blood smear showed normal red cells. At the 2-year follow-up the patient had no gastrointestinal symptoms or anemia. The coexistence of Morgagni and Bochdalek defects is rarely reported [1]. About 5%-30% of CDH cases present after the neonatal period, which poses a diagnostic challenge [2]. Such symptoms as vomiting and respiratory distress may be acute or intermittent—sometimes due to gastric volvulus or spontaneous reduction of the hernia to the abdomen. During asymptomatic periods imaging findings may be normal [3]. The presented patient had normal abdominal ultrasound and chest X-ray findings before referral to our department. The presented patient most probably had chronic occult blood loss from the gastrointestinal tract due to reflux esophagitis and mechanical trauma to the diaphragm. Sinaki et al. reported 2 patients—a 19 month-old and 6-year-old—that presented with gastrointestinal symptoms and an Hb of 5.8 g/dL and 6 g/dL, respectively [4]. Zaki et al. reported a 5-year-old patient with persistent anemia that did not respond to adequate hematinics and blood transfusion. The patient had abdominal pain, melena, an Hb of 4.8 g/dL, and multiple linear gastric erosions on the mucosal folds of the lesser curve of the stomach [5]. CDH must be included in the differential diagnosis of severe iron deficiency anemia in the absence of such obvious causes as nutritional deficiency, melena, hematochezia, and malabsorption. Physicians must be aware that a history of intermittent vomiting and/or sudden onset respiratory distress in young children are indications for imaging of the upper gastrointestinal tract and thorax.