Pneumatosis intestinalis after thoracic organ transplantation in children: A case series and review of the literature.

Pneumatosis intestinalis (PI) is a rare complication after thoracic organ transplantation. There are several theories for explaining the pathophysiology of this disease. In this paper, we highlight three cases of PI in a single pediatric center, one after lung transplantation and two after heart transplantation. Although the presentations differed, all cases improved with non-surgical therapies. There are not many articles in the pediatric literature about post-transplantation PI, and there are still many questions regarding the incidence, etiology, and treatment for this disease.

Bronchoscopic evaluation in childhood pulmonary tuberculosis: risk factors of airway involvement and contribution to the bacteriologic diagnosis.

We aimed to describe the risk factors of airway involvement and to investigate the contribution of bronchoscopy in the bacteriologic diagnosis of tuberculosis. Airway involvement was more often present in patients with resistance to tuberculosis therapy than in the patients having bronchoscopy performed at initial presentation. Addition of bronchoalveolar lavage to the diagnostic workup increased the mycobacteriologic yield statistically.

Intrinsic endobronchial obstructions in children from Turkey: evaluation of 2,555 flexible bronchoscopic procedures.

BACKGROUND: Endobronchial obstructions are rarely seen in children and are often misdiagnosed resulting in delay of definitive treatment. A variety of diseases can cause endobronchial obstructions in childhood, but data is limited as to the frequency, distribution and clinical characteristics of endobronchial obstructions diagnosed with flexible bronchoscopy (FB). OBJECTIVE: To document endobronchial obstructions detected by FB. METHODS: FB results from three pediatric pulmonology centers in Istanbul were evaluated. RESULTS: A total of 2,555 children underwent an FB procedure during the study period. Endobronchial obstructions were detected in 10% (n = 256) of the patients. Among FB in patients who had endobronchial obstructions, the four most common indications for bronchoscopy were persistent infiltrations (30%, n = 72), persistent wheezing (28%, n = 70), chronic cough (26%, n = 66) and atelectasis (23%, n = 59). The most common endobronchial obstructions detected in the patients were aspirated foreign bodies (35.9%, n = 92), endobronchial tuberculosis (31.6%, n = 81), mucous plugs occluding airway (16.7%, n = 43) and granulation scars (6%, n = 16). Other pathologies included hydatid cysts (n = 5), hemangiomas (n = 5), tumors (n = 5), submucosal nodules (n = 5) and polyps (n = 4). Endobronchial obstructions were most commonly located in the right bronchus (51%, n = 130) followed by the left bronchus (33%, n = 85), bilaterally (8%, n = 21) and trachea (8%, n = 20). CONCLUSIONS: Endobronchial obstructions can be caused by a number of different diseases which require various medical or surgical treatments. In the presence of clinical or radiological findings suggesting an endobronchial obstruction, FB should be performed promptly.

Upper airway abnormalities detected in children using flexible bronchoscopy.

OBJECTIVES: Rapid anatomical evaluation is essential to establish the severity of cases with upper respiratory obstruction and to define the degree of respiratory distress. Detailed airway endoscopy is required in most patients, not only for diagnosis, but also to treat the condition. In this study, as two of the largest paediatric pulmonology centres in Turkey, we reviewed the data of our bronchoscopy patients, and aimed to document the upper airway abnormalities that we detected during these procedures. PATIENTS AND METHODS: A retrospective analysis was made of the records of 1076 paediatric cases with pulmonary/airway disease who had undergone flexible bronchoscopy between 2007 and 2011. RESULTS: Upper airway malacia disorders were the most common (79.6%, n=259) bronchoscopic findings detected in the patients. The other most common pathologies were laryngeal edema (12.9%, n=42), external tracheal compression (12.3%, n=40), subglottic stenosis (4.0%, n=13), tracheal stenosis (2.8%, n=9), and vocal cord paralysis/irregularity (2.8%, n=9). The mean duration of symptoms was shortest in patients with vocal cord paralysis, and longest in patients with tracheal nodules (p<0.001). CONCLUSION: Paediatricians should keep in mind the possibility of malacia disorders and other congenital and acquired upper airway abnormalities in children with chronic respiratory problems. Diagnosis of underlying diseases, as soon as possible, permits the withdrawal of antibiotics or antiasthmatic drugs often used unnecessarily for long periods to treat these children.

Endobronchial findings of hydatid cyst disease: a report of five pediatric cases.

Hydatid disease is still an important public health problem throughout the world. Diagnosis of the disease is generally based on clinical and radiological findings. Evaluation of pulmonary disorders by flexible bronchoscopy (FOB) is a rapidly developing facility, but diagnostic and therapeutic FOB for pulmonary hydatid cysts is still controversial. This study examines the findings of endobronchial hydatid cyst disease in five pediatric patients from Turkey, and clinical experience about this subject is reviewed. All our patients presented with unusual symptoms of the disease, and for all of them, diagnosis had been delayed using current diagnostic methods. As a result of our experience, it can be reported that the endobronchial appearance of the hydatid cyst membrane is whitish-yellow, and it is difficult to differentiate it radiologically from some other common causes of endobronchial lesions in childhood, such as endobronchial tuberculosis, foreign body aspirations, mucous plaques, and granulation scars. The findings of these cases show that, hydatid cyst should also be kept in mind in differential diagnosis of endobronchial lesions. In the diagnosis of pulmonary hydatid cyst in children without typical clinical and radiological findings of the disease, FOB examination is a valuable diagnostic procedure.

A very rare cause of childhood paraparesis: primary intradural extramedullary spinal hydatid cyst.

Hydatid disease is caused by the larval form of Echinococcus. Lung and liver are the most commonly affected sites. Primary intradural extramedullary hydatid disease is extremely rare; a 13-year-old girl with primary intradural hydatid cyst who presented with symptoms of paraparesis is discussed in this article.

Cytoprotective effects of amifostine in the treatment of childhood malignancies.

BACKGROUND: Multi-systemic acute side effects occur, in response to intensive therapies that have been applied in childhood malignancies in recent years. Amifostine has rarely been used in the childhood cancers as a multisystemic protective agent for minimizing these side effects. PROCEDURE: In this study, the effectiveness of amifostine in combination with chemotherapy for childhood cancer treatment has been researched. Of 11 subjects (2.5 months-17 years) 4 subjects had leukemia, 4 had solid tumor, and 3 had lymphoma. For these 11 subjects, 29 chemotherapy courses were given in combination with amifostine, and 20 without amifostine. Their hematological, gastrointestinal and hepatic toxicity were evaluated according to the WHO toxicity criteria. Amifostine was given intravenously in a dose of 740 mg/m(2), one to three consecutive days depending on the chemotherapy regimen. RESULTS: The hemoglobin, leukocyte, and platelet levels of the two groups were not statistically different. However, when comparing the courses of the patients receiving the same medications at the same doses, in the group with amifostine, mean erythrocyte transfusion requirement was significantly reduced (P = 0.025). In 31% of the courses with amifostine and 50% of the courses without amifostine, febrile neutropenia developed. Gastrointestinal system and hepatic toxicity was significantly reduced in the courses with amifostine with respect to those without it (P = 0.001). Vomiting, hypotension and nausea were the only side effects related to amifostine. CONCLUSION: Use of amifostine during the treatment of childhood cancers with intensive chemotherapy and/or radiotherapy significantly reduced the erythrocyte transfusion requirements of the patients as well as gastrointestinal and hepatic toxicity.

Juvenile psoriatic arthritis carrying familial Mediterranean fever gene mutations in a 14-year-old Turkish girl.

Juvenile psoriatic arthritis (JPsA) is characterized by asymmetric arthritis of big and small joints, enthesitis, dactylitis, psoriatic skin lesions and nail pitting. Investigators agree that JPsA is a relatively common chronic arthropathy of childhood that differs clinically, serologically, and genetically from both juvenile idiopathic arthritis and juvenile ankylosing spondylitis. Familial Mediterranean fever (FMF) is a multisystemic autosomal recessive disease occasionally accompanied by sacroiliitis. This is characterized by recurrent self-limited attacks of fever and accompanying abdominal, chest and arthricular pain. We present a 14-year-old Turkish girl with JPsA and carrying FMF gene mutations. In this patient, JPsA was diagnosed according to her physical, laboratory and skin biopsy findings and a treatment with methotrexate and sulfasalazine was started. As an inadequate clinical and laboratory response was obtained after the first month of therapy, the patient was investigated for FMF, and was diagnosed by molecular analyses of related gene (E148Q heterozygous/V726A homozygous mutation) besides clinical findings. After 2 weeks of the colchicine treatment, symptoms of the patient regressed and acute phase reactants decreased. To our knowledge, this is the first case presenting with psoriatic arthritis and FMF gene mutations together and responds to colchicine, methotrexate and sulfasalazine dramatically in clinical and laboratory findings. This case has been presented to remind that cases with psoriatic arthritis may also carry mutations in the MEFV gene.