[Evaluating the effects of Omega-3 on quality of life in children with cystic fibrosis]

Cystic fibrosis [CF] is an autosomal recessive disorder chiefly characterized by respiratory and gastrointestinal symptoms. This study investigates whether omega-3 fatty acid affects quality of life in children with CF. Materials and This was a single-blind, pilot study undertaken at the Cystic Fibrosis Center of Sarvar Children Hospital, Mashhad, Iran from March 2009 until September 2009. In this study, 11 patients aged 2-12 years were assigned to ingest 3 capsules of fish oil daily for 3 months. Each capsule contained fish oil [1 gr], eicosapentaenoic acid [180 mg] and docosahexaenoic acid [120 mg]. At the beginning of the study and after the treatment period, questionnaires were used to determine the quality of life of all patients, both totally and separately, in four domains [physical [PH], emotional [EM], social [SOC] and school functioning [SCH]]. Wilcoxon test compared the scores before and after treatment intervention. p < 0.05 was considered significant. The difference in total score of the Pediatric Quality Of Life Inventory TM 4.0 Short Form [PedsQLTM 4.0 SF15] before and after intervention showed a significant improvement [p = 0.02] in addition to the physical and social domains [p = 0.01 and p = 0.04, respectively], but was not significant in the other two domains. Fish oil, as a rich source of omega-3 fatty acid could have beneficial effects on improving quality of life in these patients. Other long-term studies including more patients might better clarify omega-3 effects on children with CF

Serum chemistry abnomalities in children with unprovoked seizures

Most children brought to the emergency department [ED] for evaluation of seizures undergo an extensive laboratory workup. Since results are usually negative, the value of such routine laboratory workups has been questioned. A group of children with unprovoked seizures was prospectively studied to determine the diagnostic values of routine serum chemistries and to identify risk factors predictive of abnormal findings. All patients evaluated at the ED of the Ghaem hospital during a consecutive 12 months period between Jan 2004 through Jan 2005 were studied. We collected data for patient's demographics, details of the history of present illness [including vomiting, diarrhea, apnea], medication use, past history of seizures, family history of seizures, metabolic disorders or other chronic medical illnesses, neonatal history and neurological examination as well as nutritional status, imaging and EEG results, type and time of seizure. The role of abnormal serum chemistries as a seizure trigger factor was assessed in patients with a history of seizure. A total of 210 patients [mean age 19.2 months] with unprovoked seizures were evaluated. Twenty- three serum abnormalities were noted in the patients [12 cases of hyponatremia, 7 of hypoglycemia, 4 of hypokalemia, 4 of uremia]. The incidence of abnormal serum biochemical values was higher in patients with a first seizure, younger patients, and those with gastrointestinal symptoms. According to the present study, one can conclude that in children younger than 2 years and having no structural CNS abnormality, electrolyte and glucose screening is recommended only for a first unprovoked seizure, when gastrointestinal symptoms or symptoms suggesting electrolyte disturbances are present

[Abetalipoproteinemia: report of two cases]

Abetalipoproteinemia is a rare disorder of lipoprotein metabolism, which causes low levels of cholesterol and absence or very low levels of LDL and VLDL. This rare autosomal recessive disorder is characterized by lipid malabsorption, spinocerebellar degeneration, acanthocytosis and retinopathy. In this study two cases of this disorder presented with severe malabsorption and abdominal distention, which mimicked celiac and fibrocystic diseases were reported

[Cerebro-oculo-nasal syndrome: a case report]

The most common congenital defect involving the face and jaws is cleft lip and palate. Exactly where they appear is determined by the locations at which fusion of the various facial processes failed to occur. The lip or the lip and palate together fail to close in approximately 1-1.5 in every 1,000 babies born. The presence of cleft lip and palate raises suspiscion to cerebro-oculo-nasal syndrome especially when accompanied by anophthalmia, mental retardation and hypoplasia of nares. In this article, one case of a 4-year-old boy with cerebro-oculo-nasal syndrome who had anophthalmia, median cleft lip and palate, a CNS defect and mental retardation has been reported. Discussion and Coclusion: Although anophthalmia is a rare congenital defect, its presence raises suspiscion to cerebro-oculo-nasal syndrome especially when accompanied by cleft lip and palate, mental retardation and hypoplasia of nares. Therefore, we can conclude that in patients with cleft of upper lip, hypoplasia of nares and anophthalmia, cerebro-oculo-nasal syndrome, must be considered. Since these abnormalities are also seen in Fraser syndrome the absence of digital and renal defects differentiates cerebro-oculo-nasal syndrome

Childhood vulvovaginitis

The aim of this study was to evaluate clinical findings, microscopic examination and culture of vaginal secretions, and response to treatment in prepubertal girls with vulvovaginitis. We studied prospectively over a period of about 6 years in a clinic for pediatric kidney and urinary tract diseases, 171 girls aged 2.5 -8 years with urogenital symptoms. Dysuria, erythema, itching, soreness, and vaginal discharge were frequently observed genital symptoms and signs. Pathogenic bacteria were isolated in 27% of cases, Streptococcus pyogenes being a common agent. Nonpathogenic enteric flora was isolated in about 43%. There was no growth of bacteria in 30%. Poor hygiene was an associated factor in those with nonpathogenic positive culture [p = 0.001]. Purulent vaginal discharge occurred more in cases with vulvovaginal pathogenic infection than in those with negative culture, and this difference was statistically significant [P<0.001]. Also there was significant difference in WBC count in vaginal smears of patients with pathogenic bacteria and of those who had no growth of pathogens [p<0.001]. Candida and sexually transmitted agents were not found in any of the girls. Labial fusion was not an uncommon abnormality. Simple measures to improve hygiene and use of local estrogen were effective in the patients with nonpathogenic and nonspecific etiology. Physical examination of genital area should be done in all girls with genitourinary symptoms. Antibiotic should be prescribed based on bacteriologic culture of vaginal secretion. Appropriate hygienic practices and local estrogen application is the most effective therapy in children with noninfectious vulvovaginitis

Diagnosis of coloretal polyps by hydrocolonic sonography in children with rectal bleeding

Because of the inherent danger and associated discomfort of invasive procedures such as colonoscopy or double contrast barium enema involving exposure to radiation, we studied the value of hydrocolonic sonography in the diagnosis of colorectal polyps in children with rectal bleeding. From March 2005 to January 2006, 46 children from 2.5-11 years of age presented with hematochezia were examined by means of hydrocolonic sonography and colonoscopy. On colonoscopy, 21 patients had normal results, 19 had polyps, 3 had proctitis, 2 had lymphonodular hyperplasia and 1 had anal fissure. Only 7 of 19 colorectal polyps were diagnosed by conventional abdominal sonography [37%], whereas hydrocolonic sonography permitted the diagnosis of 17 [89.5%] with a specificity equal to 92.5%. In comparison with colonoscopy, positive predictive value of hydrocolonic sonography was 89.4% and negative predictive value was 92.5%. Hydrocolonic sonography is a accurate and safe approach to evaluating children with rectal bleeding. Thus, it can be regarded as an appropriate replacement of barium enema

Effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome

To study the effect of oral garlic on arterial oxygen pressure in children with hepatopulmonary syndrome. Garlic powder in a capsule form was given to 15 children with hepatopulmonary syndrome [confirmed by contrast echocardiography] at the dosage of 1 g/1.73 m[2] per day. Patients were evaluated clinically and by arterial blood gas every four weeks. The garlic capsule was administered to 15 patients with hepatopulmonary syndrome. There were 10 boys and 5 girls with a mean age of 9.4 +/- 3.9 years. The underlying problems were biliary tract atresia [4 patients], autoimmune hepatitis [4 patients], cryptogenic cirrhosis [4 patients] and presinusoidal portal hypertension [3 patients]. Eight patients [53.3%] showed an increase of 10 mmHg in their mean arterial oxygen pressure. The baseline PaO2 was 65.6 +/- 12.1 mmHg in the responder group and 47.1 +/- 11.2 mmHg in non-responder group. At the end of treatment the mean PaO[2] in responders and non-responders was 92.2 +/- 7.75 mmHg and 47.5 +/- 11.87 mmHg, respectively [P < 0.01]. Garlic may increase oxygenation and improve dyspnea in children with hepatopulmonary syndrome