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BACKGROUND: Cerebro-costo-mandibular syndrome (CCMS) is a rare congenital syndrome consisting of the main features of micrognathia and posterior rib gaps. Due to multiple abnormalities, patients almost have difficulty breathing with upper airway obstruction, decreased thoracic capacity, spina bifida, and scoliosis. CASE PRESENTATION: We describe a case of a late preterm neonate boy presenting with low Apgar, respiratory distress, and complicated orofacial anomalies that had a poor outcome. His radiographic findings showed mandibular hypoplasia (micrognathia), chest deformity, multiple posterior rib gap defects, and abnormal costotransverse articulation. Based on physical examination and radiologic findings, the diagnosis of CCMS confirmed for the patient. CONCLUSION: Physicians should always consider the diagnosis of CCMS in all infants with micrognathia and rib-gap defects. These infants need careful respiratory function monitoring. Early airway management improves growth and development. In addition, their physical and psychological development should be assessed regularly.
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BACKGROUND: Reduction of neonatal mortality rate can improve health and newborn status of the society. This study was done to evaluate the prevalence and pattern of causes for neonatal mortality in Gorgan, North of Iran. METHODS: this descriptive, cross-sectional study was carried out on all birth in the maternity Dezyani hospital in Gorgan, North of Iran during 1 September 2008 -31 March 2011. Causes of mortality were evaluated in newborns that admitted and died in the NICU. Died newborns were screened for primary and final causes of death. The final causes of the mortality in majority of neonates were extracted according to the International Coding of disease Ver10 (ICD10). RESULTS: The total number of births was14785. The leading primary obstetric causes of death were: spontaneous preterm labor 36(25.7%), fetal abnormality 31(22.1%), hypertensive disorders 21(15%), unexplained intrauterine death 21(15%), maternal disease 12 (8.6%), intra-uterine growth restriction (IUGR) 10 (7.1%), oligohydroamnios 5(3.6%) and ante partum hemorrhage 4 (2.9%). Mortality rate was 76.8% during the first week of life. General final causes of death were; pulmonary bleeding17.9%, septic shock16.8%, IVH15.8%, congenital anomalies and pnomotorax 13.7% in fourth grade. CONCLUSION: The risk factors, main associated reason and procedures for prevention of spontaneous preterm labor and fetal abnormality should be includes as the main themes in perinatal research. In addition, there should be emphasized on necessity of genetic consultations and health care before and during pregnancy.
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OBJECTIVE: Cleft lip with or without cleft palate is the most common orofacial congenital anomaly among live births. This study was carried out to determine the incidence rate of oral clefting in Gorgan, Northern Iran during 2004-2009. METHODS: This descriptive hospital-based study was performed on 35,009 live newborns in Dezyani Hospital in Gorgan, Northern Iran during 2004-2009. All newborns were screened for oral clefts. Data including birth date, gender, type of oral clefts, parents' consanguinity, parental ethnicity and presence of other congenital anomalies were recorded for analysis. RESULT: The overall incidence rate of oral clefts during this 6-year period was 1.05 per 1000, or 1 per 946 live births. The incidence of cleft lip and isolated cleft palate was 0.08 and 0.37 per 1,000 live births, respectively. The ratio for different cleft types was 1:7:4 (CL: CLP: CP). The incidence of oral clefting was 1.2 per 1,000 male births and 0.86 per 1,000 female births (RR=1.40; 95% CI: 0.73-2.71). According to parental ethnicity, the incidence of oral clefting was 0.7, 1.7 and 1.26 per 1,000 in Native Fars, Turkman and Sistani, respectively. The relative risk for oral clefting in Turkman to native Fars group was 2.56 (p<0.02). In this study, 56.7% of clefts were CL+P, 8.1% were CL and 35.1% of cases were CP. CP was more common among girls (54%) than among boys (46%) but CL was more common among boys. CONCLUSION: The results showed that the incidence of oral clefts in the study population as being 1.05 per 1,000 live births, which has increased from 0.97 per 1,000 live births reported in an earlier study in this area.
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OBJECTIVE: Neonatal sepsis (NS) is a common and life-threatening disorder in infants. Previous studies showed that interleukin-6 (IL-6) may be a valid non-invasive and rapid method for diagnosis of NS. We conducted this review to assess the validity of IL-6 for predicting NS. METHODS: This was a systematic review with meta-analysis. Embase, Medline and Web of Science databases were searched between January 1990 and December 2009. The search terms used were "cytokine", "neonate", "sepsis" and "interleukin-6". We used standard methods recommended for meta analyses of diagnostic test evaluations. The analysis was based on a summary ROC (SROC) curve. Meta-regression analysis was used to assess the effects of some confounding factors on the results of meta-analysis. Potential presence of publication bias was tested using funnel plots and the Egger test. FINDINGS: Meta-analysis was performed on 13 publications including 353 infants with sepsis and 691 control infants. The pooled sensitivity and specificity of IL-6 was 0.79 and 0.84, respectively. The maximum joint sensitivity and specificity (i.e., the Q value) in SROC curve was 0.82 and the area under curve (AUC) was 0.89 (95% CI: 0.84-0.94). Meta-regression analysis showed that the diagnostic accuracy of IL-6 was not affected by confounding variables. The evaluation of publication bias showed that the Egger test was not significant (P=0.07). CONCLUSION: IL-6 seems to be a valid marker for predicting NS. It may be considered for early diagnosis of sepsis in neonatal care units.