Fontan completion of a 10-year-old Kawashima patient with extensive arteriovenous malformations: consideration for a lobectomy.

A significant contributing factor to the progression of late cyanosis in individuals undergoing Kawashima operation is pulmonary arteriovenous malformations. Following the Fontan procedure, arteriovenous malformations may regress. However, in cases with extensive malformations causing severe cyanosis, lobectomy can also be a possible treatment approach. Thereby, we present our two-step treatment strategy in a late Fontan completion complicated by arteriovenous malformations in a Kawashima patient.

Diagnostic dilemma: osteopetrosis with superimposed rickets causing neonatal hypocalcemia.

Osteopetrosis is a rare genetic condition of reduced osteoclastic bone resorption which causes defective bone remodeling and skeletal sclerosis during growth, having effects on many organs and tissues. Mutation of T-cell immune regulator 1 (TCRG1) gene is the most common genetic defect leading to osteopetrosis, with poor prognosis. The autosomal recessive form presents in the infantile period (also known as malignant infantile osteopetrosis--MIOP), and is characterized by fractures, short stature, hepatosplenomegaly, compressive neuropathies, hypocalcemia and pancytopenia. Being a rare disease with non-specific clinical manifestations, the diagnosis is difficult and usually delayed. Rickets is a characteristic feature of MIOP which results from the defect in osteoclasts to provide a normal Ca/P balance resulting in the poor mineralization of the osteoid. Various treatment options have been suggested for osteopetrosis, but hematopoietic stem cell transplantation still remains the only curative treatment option presently. The authors report the case of a 46-day-old girl with late-onset neonatal hypocalcemia and rickets that was later diagnosed as osteopetrosis. This case report emphasizes that infantile osteopetrosis is an important cause of neonatal hypocalcemia. As irreversible complications develop within the first months of life, immediate diagnosis and early intervention are crucial and may be life-saving.

Predictors of extubation in the operating room after pediatric cardiac surgery: A single-center retrospective study.

Background: In this study, we aimed to evaluate the outcomes of our on-table extubation strategy in patients with congenital heart disease. Methods: Between April 2021 and November 2022, a total of 114 pediatric patients (58 males, 56 females; median age: 25.3 months; range, 57.5 to 4.4 months) who were operated for congenital heart diseases were retrospectively analyzed. The patients were evaluated according to the Society of Thoracic Surgeons-European Association for Cardio-Thoracic Surgery (STS-EACTS) scoring system. Perioperative patient data were analyzed and correlated with the extubation status. Results: Overall, 56% of the patients were extubated in the operating room. There was an association between fluid balance per unit body surface area, longer cardiopulmonary bypass and cross-clamp times and on-table extubation. Lactate value prior to extubation, STS-EACTS mortality category, estimated mortality, and estimated morbidity were statistically significant with regards to the achievability of extubation. Multivariate analysis revealed lactate value prior to extubation and estimated postoperative length of hospital stay to be significant factors affecting on-table extubation. There was a significant correlation between decreased length of intensive care unit and hospital stay and on-table extubation. Conclusion: The outcomes of our on-table extubation strategy for patients with congenital heart disease reveal the feasibility of this approach. Higher lactate and fluid balance/body surface area levels, longer cross-clamp and cardiopulmonary bypass durations, increased surgical complexity are indicators of a failure to perform on-table extubation. This strategy is also associated with shorter intensive care unit and hospital length of stays as an additional clinical benefit.

IL-5, IL-8 and MMP -9 levels in exhaled breath condensate of atopic and nonatopic asthmatic children.

RATIONALE: Asthma is a heterogeneous disease, and a great majority of pediatric patients with asthma demonstrate atopic characteristics and develop a Th2 type cytokine response. Nonatopic asthma, on the other hand, is seen more rarely. METHODS: In this study, levels of IL-5, IL-8 and MMP-9 were measured in exhaled breath condensate (EBC) of the subjects to demonstrate the extent of tissue damage as well as eosinophilic and neutrophilic inflammation in children with atopic and nonatopic asthma. A total of 37 children with atopic asthma and 37 children with nonatopic asthma were enrolled in the study. Patients who exhibited protease positive aeroallergen (House dust mite, mould mix, olea, grass mix) sensitivity in allergen skin prick test were included in the atopic asthma group. To evaluate the EBC, the fluid content of the breath was collected by having the patients exhale into an EBC device, after which the IL-5, IL-8 and MMP-9 levels were assayed using the ELISA method. RESULTS: The atopic asthmatics exhibited significantly higher IL-5 levels in their EBC samples than the nonatopic asthmatics (0.271 [0.198-0.489] pg/ml and 0.198 [0.125-0.344] pg/ml, respectively, p = 0.04), while no significant differences were observed in the levels of IL-8 and MMP-9 in the EBC samples of the atopic and nonatopic asthmatics. CONCLUSIONS: IL-5 levels, as a marker of eosinophilic inflammation, were demonstrated to be higher in the children with atopic asthma when compared to those with nonatopic asthma in EBC. The fact that no significant difference was apparent in the IL-8 levels between the groups suggests that it is the severity of the disease rather than the atopic state that plays an important role in IL-8 levels. Since no difference was recorded between the groups in terms of MMP-9 levels, lung damage in asthma sufferers seems to develop independent of atopia.

E-cadherin as an epithelial barrier protein in exhaled breath condensate.

Airway epithelium plays an important role as a physical barrier and a modulator of allergic response. Junctions between cells provide epithelial integrity and barrier function. The aim of this study was to investigate the influence of atopy on airway epithelial integrity in asthma and to measure E-cadherin levels in exhaled breath condensate as an indicator epithelial damage. A total of 74 patients with asthma (35 atopic and 39 non-atopic) and 39 healthy children were enrolled in this case-control study. Sociodemographic characteristics and asthma severity parameters in the last three-month period were recorded and pulmonary function tests were performed. Blood samples were obtained to measure serum immunoglobulin E (IgE) levels and peripheral blood eosinophil count, and exhaled breath condensate (EBC) was obtained to measure E-cadherin.EBC E-cadherin levels were significantly lower in the asthmatics when compared to non-atopic controls (0.109 (0.076) versus 0.191 (0.184) ng mL(-1) respectively, p = 0.01). Atopic and non-atopic asthmatic groups had lower EBC E-cadherin levels compared to the control group. (0.112 (0.060) ng ml(-1), 0.106 (0.089) ng ml(-1) and 0.191 (0.184) ng ml(-1), p = 0.02 and p < 0.01 respectively). However, EBC E-cadherin levels were not different between atopic and non-atopic asthmatics. The results of our study support the role of E-cadherin in the pathogenesis of asthma. However, the absence of difference in E-cadherin levels between atopic and non-atopic asthmatics suggests that allergic sensitization is not the primary factor for development of epithelial barrier dysfunction in asthma.