Turkish J Pediatr
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Ichthyoses are divided into four groups according to clinical, histopathologic and genetic findings. Lamellar ichthyosis is one of them. The incidence of lamellar ichthyosis is believed to be approximately 1 per 100,000 to 300,000 live births. ⋯ There were no associated hair or neurological abnormalities. His clinical and histopathological findings were typical for isolated lamellar ichthyosis. Because of its rare occurrence, we report this case with a review of the literature.
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Two newborn infants with ichthyosis, one with lamellar ichthyosis and one with nonbullous ichthyosis form erythroderma, who presented at birth with a collodion baby appearance, were treated with acitretin (1 mg/kg/day). Clinical improvement was achieved shortly after treatment. The second case received oral retinoid for 3.5 months and was followed for nine months. ⋯ The tolerance to the drug was good. Side effects were not observed. It was concluded that early management of severe ichthyosis cases could prevent life-threatening events such as hyperthermia, disturbance in electrolyte and fluid balance, and infection.
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Apolipoprotein A-I (apo A-I) has been found to be decreased in adults with cirrhosis, but it has not been routinely used for prognostic purposes thus far. This study was performed to determine apo A-I levels in childhood cirrhosis and to establish some prognostic cut-off values. Apo A-I levels of 78 children with chronic liver disease, 38 of whom had cirrhosis as well, were studied. ⋯ Apo A-I value < 80 mg/dl had 84% specificity and 84% negative predictive value for the high risk group of Malatack's model. Similarly, Apo A-I value < 83 mg/dl had 95% specificity and 87% negative predictive value for the Child-Pugh C group. We concluded that Apo A-I is a sensitive and specific parameter of poor prognosis in childhood cirrhosis.
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In order to develop verbal communication skills, an infant nervous system needs sound stimuli, especially human speech, in the early and critical learning period of life. The maturation of hearing and language learning abilities of an infant is considered to be complete within the first two to three years of life. Therefore, detection of hearing impairment before the end of the critical language learning period is considered crucial if a child is expected to acquire his/her native language properly. ⋯ Even mild hearing loss may interfere with normal development of speech and language in infants, therefore, universal screening of infants for hearing loss as early as possible should be the goal. The purpose of this paper is to discuss the concept of the critical language learning period and its relevance to language acquisition. Furthermore, it is the aim of the paper to evaluate the available techniques for early identification of hearing impairment in general and the feasibility of the various techniques in Turkey.
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We report our clinical experience with the newly developed Amplatzer device in transcatheter closure of nine atrial septal defects (ASDs), one ventricular septal defect (VSD), and one patent arterial duct (PDA). Eleven patients with ASD (age range 2.5-18 years) selected according to the location and size of the defect by transesophageal echocardiography (TEE), a five-year-old patient with muscular VSD and a one-year-old patient with PDA were considered for transcatheter closure with Amplatzer devices. All procedures were performed under general anesthesia with fluoroscopic and TEE guidance, following a routine hemodynamic evaluation in the catheter laboratory. ⋯ None of the patients had major complications. Junctional rhythm developed in one patient, and another patient had frequent supraventricular extrasystoles. Amplatzer is an effective and safe device for transcatheter closure of ASD, VSD, or PDA, especially in pediatric patients.