[Pulmonary artery sling as a cause of recurrent wheezing in children]. / Çocuklarda tekrarlayan hisiltinin bir nedeni olarak pulmoner arter sling.

Vascular rings are a group of aortic arch anomalies and usually presents with dispnea, stridor and feeding difficulties after birth. Pulmonary artery sling is created by anomalous origin of the left pulmonary artery from the posterior aspect of the right pulmonary artery. The anomalous left pulmonary artery compresses the lower trachea and right mainstem bronchus, producing upper airway symptoms. We presented a seven-month-old male patient who admitted to our clinic because of recurrent wheezing, stridor, and frequent lower respiratory tract infections.

Hemophagocytosis: the cause of anemia and thrombocytopenia in congenital syphilis.

Congenital syphilis is a rare, serious disease that continues to be a major health-care problem. The infected neonate may be asymptomatic or multiple-organ system involvement may occur. Anemia and thrombocytopenia are common hematological findings. Hemophagocytic lymphohistiocytosis (HLH) is a clinicopathological condition characterized by activation and uncontrolled nonmalignant proliferation of T lymphocytes and macrophages. The authors report an infant with congenital syphilis as a very rare cause of hemophagocytic syndrome. The reason for anemia and thrombocytopenia in congenital syphilis is not clear. The authors suggest that hemophagocytosis may play role in pathogenesis of cytopenia, particularly thrombocytopenia in patients with congenital syphilis.

[Jeune syndrome (asphyxiating thoracic dystrophy): a case report]. / Jeune sendromu (asfiktik torasik displazi): Olgu sunumu.

Jeune syndrome or asphyxiating thoracic dystrophy is a rare autosomal recessive skeletal dysplasia characterized by a small thorax, short-limbed dwarfism, renal and hepatic anomalies. It is estimated to occur in 1 per 100.000-130.000 live births. Radiological confirmation of diagnosis is essential. Prognosis of the disease is due to the severity of chest deformities and most of the patients are lost in their first year because of respiratory problems. We presented a 19-month-old female patient who admitted to our clinic because of recurrent lower respiratory tract infections and diagnosed as Jeune syndrome and reported due to its rarity.

Application of Flow Cytometry in the Early Diagnosis of Neonatal Sepsis.

GOALS: To raise awareness of the utility of flow cytometric detection of inflammatory markers in the early diagnosis of neonatal sepsis. PROCEDURES: In accordance with the Töllner scoring system, cases with ≥10 points are accepted as having "clinical sepsis" and cases with 0-4 points as having "no sepsis". The study group consisted of 50 newborns with clinical sepsis as well as a control group of 50 newborns without sepsis. In all cases, blood counts, C-reactive protein (CRP) levels, and procalcitonin (PCT) levels were recorded. Additionally, the "cluster of differentiation" (CD)64, CD11b, and CD62L adhesion molecules and the presence of the human leukocyte antigen HLA-DR on monocyte and neutrophil surfaces were examined by flow cytometry. RESULTS: The levels of acute-phase reactants CRP and PCT were significantly higher in the study group than in the control group (p<0.05). The cell adhesion molecules CD11b and CD64 and the human leukocyte antigen HLA-DR were significantly higher in the study group (p<0.05); CD62L levels were similar to those in the control group (p>0.05). Furthermore, receiver operating characteristic analysis indicated that neutrophil CD11b (nCD11b) is a diagnostic marker for neonatal sepsis (area under the curve [AUC]: 0.72, 95% confidence interval [CI]: 0.62-0.82, p<0.001). The sensitivity, specificity, and positive predictive value (PPV) for nCD11b were 72%, 68%, and 58.4%, respectively. Similarly, monocyte CD11b (mCD11b) positivity was found to be diagnostic (AUC 0.77, 95% CI: 0.68-0.87, p<0.001). The sensitivity and specificity for mCD11b were 72% and 68%, respectively. In addition, the sensitivity, specificity, and PPV for nHLA-DR were 62%, 60%, and 60.8%, respectively. CONCLUSION: In addition to acute-phase proteins, cell surface antigens such as CD11b, CD64, and HLA-DR should be used in routine investigations for the early diagnosis of neonatal sepsis. Such usage in combination with acute-phase reactants may enhance diagnostic accuracy.

Hamartoma of the larynx: an unusual cause of stridor.

BACKGROUND: Hamartoma of the larynx is a very rare lesion, and the number of reported cases is limited. Signs and symptoms include stridor, changes in voice, eating and respiratory complaints. Stridor is a sign of upper airway obstruction. Patients presenting with stridor and severe respiratory distress necessitate urgent otolaryngologic evaluation. CASE REPORT: Herein, we report a case of laryngeal hamartoma presenting with recurrent pneumonia and persistent stridor in a 7-month-old patient. He was admitted to hospital with the initial diagnosis of recurrent pneumonia and persistent stridor. Stridor was not responsive to cool mist, nebulised epinephrine or dexamethasone. Cervical computed tomography (CT) revealed a solid, nodular mass on the posterolateral wall of larynx. The mass was excised surgically. After surgical removal of the hamartoma, the child was relieved of the obstruction. CONCLUSION: We want to emphasise that patients presenting with persistent stridor and severe distress necessitate urgent otolaryngologic evaluation.