Variant clinical courses in children with immune thrombocytopenic purpura: Sixteen year experience of a single medical center

Objective: Immune thrombocytopenic purpura (ITP) is the most common cause of acquired thrombocytopenia in children. The objective of this study was to evaluate the presenting features, variation in the clinical courses, initial response rate to therapy, and long-term outcome in patients with ITP. Materials and Methods: Three hundred and fifty out of 491 newly diagnosed patients with ITP between the initial diagnosis ages of 6 months to 16 years were included in this retrospective, descriptive study. Patients with acute vs chronic ITP, acute vs recurrent ITP and chronic vs recurrent ITP were compared in terms of age at diagnosis, gender, initial platelet count, response rate to initial therapy, long-term outcome, and total duration of follow-up. Results: The clinical courses of the patients were determined as acute, chronic and recurrent in 63.8%, 29.1%, and 7.1%, respectively. Platelet count >20x109/L and initial diagnosis age >10 years were found to increase the probability of chronic outcome by at least two-fold. Conclusion: It is concluded that ITP in childhood is a common disease with low morbidity and mortality. In addition to the acute and chronic form, a rare recurrent form, which accounts for about 4-7% of all ITP patients, should be considered

Çocukluk çağında poliarteritis nodoza: Olgu sunumu

Polyarteritis nodosa (PAN) is a'transmural fibrinoid necrotizing arteritis of small and medium-sized vessels characterized by kidney, skin, joint and peripheral nervous and gastrointestinal system involvement. This disease is rarely seen in childhood. We herein report a 14.5-year-old boy presented with skin lesions, myalgia, headache, abdominal pain and malign hypertension. Polyarteritis nodosa was diagnosed by renal angiography. the treatment was as follows: Corticosteroid (intravenous pulse metil prednisolone, 1 gr/day 3 day; PO prednisolone 60 mg/day 30 days; prednisolone 60 mg/every other day 60 days) and cyclophosphamide (2.5 mg/kg/day, PO, 10 weeks). the patient was treated successfully by this regime and has been followed with no complains for eight months. We reviewed the clinical features, differential diagnosis and therapeutic approachment of PAN.Poliarteritis nodoza (PAN) akut başlangıçtı, küçük ve orta çaplı arterlerin transmural fibrinoid nekrozu ile karakterli, sıklıkla deri, eklem, periferik sinirler, gastrointestinal sistem ve böbrek tutulumu ile giden sistemik bir vaskülittir. Genelde genç erişkin erkeklerde görülmekle birlikte çocukluk çağında da görülebilmektedir. Bu yazıda alt ekstremitelerde döküntü, miyalji, karın ağrısı ve baş ağrısı yakınmaları ile başvuran ve malign hipertansiyon saptanan 14.5 yaşında bir erkek olgu sunulmuştur. Poliarteritis nodoza tanısı renal anjiyografi ile konan ve steroid (intravenöz pulse metil prednizolon, 1 gr/gün 3 gün; PO prednizolon 60 mg/gün 30 gün; 60 mg/gün aşırı 60 gün) ve siklofosfamid (2.5 mg/kg/gün, PO, 10 hafta) ile tedavi edilen hasta 8 aydır sorunsuz izlenmektedir. Bu olgu eşliğinde çocukluk yaş grubunda nadir görülen PAN'da klinik, tanısal yaklaşım ve tedavi prensipleri tartışılmıştır

Congenital Complete Tracheal Ring in a Neonate: A Case Report

Abnormalities of the upper airway tract lead to congenital high airway obstruction and may complicate neonatal airway management in the delivery room. Congenital complete tracheal rings are a rare and unusual tracheal anomaly, usually presenting in the neonate or infant as respiratory distress. The clinical presentation can vary from almost asymptomatic patients to near-fatal airway obstruction. It may exist as an isolated entity, or in association with other congenital malformations, in particular, cardiac anomalies along with vascular rings and pulmonary slings. Other associated anomalies have also been reported, for example, chromosomal anomalies, malformation of other parts of the respiratory tract, esophagus and skeletal systems. Here, we report an extreme case of VACTERL/TACRD association presented with congenital complete tracheal ring, encephalocele, bilateral radial agenesis with absent thumbs, equinovalgus deformity on right foot, low-set ears and micrognathia

Neuronal apoptosis in the neonates born to preeclamptic mothers

Objective: Preeclampsia may result in uteroplacental insufficiency and chronic intrauterine fetal distress. The aim of this study is to address this issue investigating neuronal apoptosis in an experimental model of preeclampsia and to evaluate the neurological outcome of the perinatal asphyxia in the neonates born to preeclamptic mother