Syndrome of inappropriate antidiuretic hormone secretion refractory to treatment in a newborn with alobar holoprosencephaly.

Alobar holoprosencephaly (AHP) is a serious malformation of the central nervous system characterized by complete failure of cleavage of the prosencephalon resulting in fusion of the cerebral hemispheres and a massive single ventricular midline fluid collection. Secretion of inappropriate antidiuretic hormone syndrome (SIADH) is a disorder of fluid-electrolyte balance caused by the inability to suppress the secretion of antidiuretic hormone (ADH) resulting in the development of hyponatremia. Coexistence of both of these entities has not been described in the literature. We report a newborn infant with AHP who had resistant SIADH refractory to treatment, to draw attention to the coexistence of these entities and to contribute to the literature with the management of this challenging condition.

Spontaneous intestinal perforation after oral ibuprofen treatment of patent ductus arteriosus in two very-low-birthweight infants.

AIM: To discuss intestinal side effects of ibuprofen in the treatment of patent ductus arteriosus, after having observed two cases of spontaneous intestinal perforation following ibuprofen treatment. METHODS: Clinical and laboratory records of two preterm infants, who developed intestinal perforation after ibuprofen administration, were evaluated. RESULTS: Gestational ages of infants were 29 wk (male) and 30 wk (female). Both infants developed intestinal perforations without signs of necrotizing enterocolitis. The perforations cured with Penrose drainage alone. CONCLUSION: Although ibuprofen is a reasonable treatment alternative to indomethacin, randomized controlled trials, which address potential adverse effects including spontaneous intestinal perforation, are needed.

Paranasal sinus computed tomographic findings of children with chronic cough.

OBJECTIVE: Chronic cough is a frequent problem in the practice of clinical pediatrics, and sinusitis is a common etiologic factor. In this study, our aim was to determine the prevalence of sinus abnormalities in pediatric patients presenting with chronic cough. METHODS: Forty-two patients, ranging from 3 to 16 years of age from both sexes completed the study. Paranasal sinuses were examined by coronal paranasal sinus limited computed tomographic scan. The CT scan findings were categorized as no disease, minimal, moderate and severe sinusitis. RESULTS: The most frequent symptoms after cough were rhinorrhea, sniffling, and halitosis. We found sinus abnormalities in 66.6% and no abnormality in 33.3% of the children. Age and sex were not important factors in sinus disease. There was no correlation between sinus findings and any of symptoms. The most common anatomical abnormalities were concha bullosa, paradoxical middle turbinate, Haller's cells, and deviated nasal septum. These abnormalities were not correlated with sinus disease. CONCLUSION: Paranasal sinus abnormalities are common among pediatric patients with chronic cough and imaging studies of the sinuses should be considered in these children.

Bernard-Soulier Syndrome Like Platelet Defect in a Patient with Noonan Syndrome; A Case Report.

Noonan's Syndrome (NS) is characterized by dismorphic facial features, short stature, short or webbed neck, congenital heart defects and testicular abnormalities. Various bleeding disorders in Noonan Syndrome have been reported. Bernard-Soulier Syndrome (BSS) is a rare congenital bleeding disorder characterized by thrombocytopenia and giant platelets. There is not any reported case of Noonan syndrome associated with BSS in literature. We report here a four-year-old male patient with Noonan Syndrome and BSS like platelet defect.

The frequency and etiology of anemia among children 6-16 years of age in the southeast region of Turkey.

The frequency and etiology of anemia were investigated in 2,913 children between six and 16 years of age in Sanliurfa, in the southeast region of Turkey. Anemia was found in 142 (7.8%) children in the 6-11 years of age group, and in 16 (1.5%) in the 12-16 years of age group; in total, in 158 (5.4%) children. Causes were iron deficiency in 93 (58.9%) children, beta-thalassemia heterozygosity in 10 (6.3%) children, chronic disease that causes anemia of inflammation in 30 (19.0%) children, and intestinal parasitic infections in 17 (10.8%) children. In eight (5.1%) children, the cause of anemia could not be determined. The study's results showed that iron deficiency anemia and chronic and parasitic disease are important problems in schoolchildren of Sanliurfa, while beta-thalassemia and hemoglobinopathies have less importance.

Juvenile rheumatoid arthritis presented with thrombocytopenia.

Leukopenia and thrombocytopenia are rare findings in systemic onset juvenile rheumatoid arthritis (S-JRA), and if present, bone marrow (BM) examination is necessary to exclude malignant diseases. We report here a 13.5-year-old boy with S-JRA who had severe thrombocytopenia and mild leukopenia, without arthritis, at the onset of the disease. BM was hypercellular with increased numbers of myeloid precursors and megakaryocytes. After treatment with acetylsalicylic acid, leukocyte and platelet counts returned to normal levels, and after two months chronic arthritis developed.

Altered anti-oxidant status and increased lipid peroxidation in marasmic children.

BACKGROUND: Protein energy malnutrition (PEM) is a common pediatric health problem in developing countries. Although the clinical features of PEM are well known, its pathophysiology is still unclear. Free radicals have been implicated in pathogenesis of PEM. In the present study, oxidant/anti-oxidant status in marasmus was investigated. METHODS: Red cell glutathione, glutathione peroxidase and superoxide dismutase and their related cofactors, serum selenium and copper, were studied in marasmic and control children. Serum lipid peroxidation was also evaluated to assess oxidative stress. RESULTS: The red cell glutathione levels and glutathione peroxidase activities were found to be significantly lower in the marasmic children than in the controls. Red cell superoxide dismutase (SOD) activity was not different between two groups. Serum selenium and copper concentrations were significantly lower in the marasmic children than in the control subjects. The malondialdehyde concentration, which is an index of lipid peroxidation, was significantly higher in the marasmic group compared with the controls. CONCLUSION: The anti-oxidant defense system was affected in marasmic children. Reduced anti-oxidant status and increased oxidative stress occurs in marasmic children.