Prenatal diagnosis of granular cell tumor

Congenital granular cell tumor [GCT] is a relatively rare intraoral benign tumor, approximately 200 cases of which have been reported in the neonatal period worldwide. The newborn infant may have feeding problems and respiratory difficulties due to airway obstruction. This lesion may be diagnosed by prenatal ultrasonography and simple resection is mostly required. We report a case of an adult type of GCT in a newly born infant, who presented with an intraoral protruding mass with a prenatal diagnosis. This article describes the prenatal course, clinical, and pathological characteristics, and management of the GCT

Apparent life-threatening events in neonatal period: clinical manifestations and diagnostic challenges in a pediatric referral center

Apparent Life-Threatening Events [ALTEs] is an episode that is frightening to the observer and is characterized by some combination of apnea, color change, altered muscle tone, choking, and gagging. This study was designed to evaluate and follow up neonates who presented with clinical manifestation of an ALTE in a year. In this prospective observational study, all of the neonates with episode of ALTE who were admitted to the Children's Medical Center [CMC] in Tehran, from June 15[th] 2010 to May 14[th] 2011 were enrolled in the study. Data from patients consisting of history, physical examinations, and paraclinical findings were recorded in a checklist and all followed up 3 to 6 months after discharge. During the study period 18 neonates were admitted due to ALTE episode[s] with mean age of 15 +/- 13 days. Nine [50%] neonates had previous attacks of ALTE. The most frequent complaint was cyanosis in 12 [67%] and apnea in 8 [44%] patients. In 10 [56%] the event lasted less than one minute, 13 [72%] were awake, 17 [95%] in supine position and 13 [72%] on their parent's lap. Primary antagonistic impression on admission was sepsis in 11 [61%] and concomitant seizure in 5 [28%]. The most common final diagnosis according to repeated physical examinations, result of paraclinical investigations and follow up was sepsis 4 [22%] and aspiration 9 [50%]. ALTE recurred in none of the neonates during follow up. The rate of ALTE seems to be higher than in this study owing to high incidence of recurrent ALTE. Although most of these attacks regress spontaneously, more attention should be paid for the underlying diseases

Prevalence of portal vein thrombosis following umbilical catheterization in neonatal period

Portal venous thrombosis [PVT] is one of the most common causes of extrahepatic portal hypertension in children that may be occurring following umbilical venous cauterization or omphalities during neonatal period. We investigated the effects of umbilical cauterization during neonatal period on portal vein thrombosis. This study investigated the frequency of thrombosis of portal vein in term or preterm infants following umbilical catheterization by color doppler ultrasound. Fifty neonates who had age range [3.6+ 8days] and birth weights 1250- 4230 gram were recruited for this study and umbilical venous catheters were placed on neonates. All parents of these neonates signed a consent form. Color doppler ultrasound sonography of the portal vein was performed by two expert radiologists within 3 days following umbilical cauterization and serially for at least a period of 6 months intervals up to 3 years [6 times for each case]. Ten children were excluded from the study because of lack of follow up. Forty children completed the study. From total of 40 subjects, portal vein thromboses were detected in two children [1 girl, 1 boy] with age 2.5 and 1.5 years respectively. In these two children, other clinical signs of portal vein thrombosis manifested such as esophageal and stomach varices and splenomegaly confirmed by endoscopy. In all the other children, the physical examinations and ultrasounds were normal during 6 stages. It is recommended that care be exercised during cauterization placement in order to prevent thrombosis of the portal vein from occurring

Staged repair of giant recurrent omphalocele and gastroschesis "camel-litter method"-A new technique

The aim of this article is to present a new Technique of giant omphaloceles repair in neonatal period and also later in life in patients that the primary repair has been failed. From 1999 to 2006, seven consecutive children [male/female ratio 0.4] with giant omphalocele [n=6] and Gastroschesis [n=2] were underwent this new operation in our center. In this technique, there were two operations. The mean of hospital stay was 38 days [range, 23-42 days], and full enteral feeding was achieved on the 8 to 25 postoperative day [Mean, 14 day]. The final closure, in all patients was achieved between the 14 to 32 days after the first operation [Mean, 21 day]. Mechanical ventilation was necessary for the mean of 5 days [range, 2-8 days]. All patients are alive and have no complication due to the operation [1 month-7 years]. Giant omphalocele and Gastroschesis can be safely repaired. The placement of an intraperitoneal tissue expander and traction of abdominal muscles can create the needed space for closure in several weeks in patients with giant omphalocele/ Gastroschesis

relationship between thyroxine level and short term clinical outcome among sick newborn infants

Premature and critically sick infants frequently experience several interventions, including blood transfusions, parentral nutrition, and prescriptions during hospitalization that could affect the result of thyroid function test. This study aims to investigate the correlation between thyroxine level and clinical short term outcome among the newborn infants in the neonatal intensive care unit [NICU]. We assessed serum levels of thyroxine and thyroid stimulating hormone of 99 neonates who were admitted in the NICU from September 1[st] 2004 to March 30[th] 2005. Number of patients with low thyroxin level [less than 6.5 micro g/dl] was determined and the relation between serum total thyroxine level and birth weight, gestational age, duration of hospitalization, clinical diagnosis, and final outcome was investigated. Short term outcome was considered as duration of hospitalization and discharge alive from hospital. Prevalence of hypothyroxinemia was 26 percent. Later assessment of thyroxine level within 3 weeks revealed normal level of this parameter [8.12 micro g/dl +/- 1.36]. Patients with lower gestational age and lower birth weight had lower thyroxine level [7.15 micro g/dl +/- 2.56, and P=0.03, 6.72 micro g/dl +/- 3.03, and P=0.08]. Low thyroxine level was not associated with adverse short-term clinical outcome [mortality rates; 3[11%] and 9[12%], and duration of hospitalization among 17.7 +/- 9.8 vs 16.7 +/- 13.0 in patients with hypothyroxinemia and low thyroxine level respectively]. Hypothyroxinemia has considerable prevalence in neonatal intensive care setting and is related with lower birth weight and gestational age. Whether thyroxin levels are a marker or mediator of short term clinical outcome remains to be determined by further studies

Role and structure of morning report in children's teaching hospitals in Iran

Morning report is an integral component of medical training programs. It is conducted as "evidence based" or "problem based". It takes an efficient time of all members of the medical team in teaching hospitals, it seems necessary to evaluate its role in the education. Because of the importance of morning report in education, we evaluated the current and ideal conditions of morning report according to the opinions of medical teams in teaching children's hospitals. A cross- sectional descriptive study conducted in three children's teaching hospitals in Tehran in 2005. The opinion and perception of 358 participants, including faculties, residents, fellows, interns, and medical students, were collected by a questionnaire regarding the importance and structure of morning report. The data were presented as frequency and percentage. 78% of respondents expected a high educational role for morning report. Although 317 [88.54%] had a regular attendance in morning report, only 34.1% were satisfied from current condition. The majority believed that faculty had better to lead the sessions, and voted for case presentation to be selected by senior resident on call, despite the prominent current leadership of the faculty. Most of the participants [88.6%] preferred complicated and unusual cases for presentation. Current morning reports predominantly based on the presentation of the interesting or complicated cases were admitted on the previous day. A few number of cases were reintroduced after achieving the final diagnosis. In addition out-patients and those under observation in emergency room were usually ignored in the meetings. Regarding the educational role of morning report, there is a far distance between the present and ideal condition. Unattractiveness of presentations and poor participation in discussion might have negative impact on achieving the goals

Two consecutive stillbirths with multiple intestinal atresias in a 30-year-old female

Multiple duodenal and jejuno-ileal atresias are extremely uncommon.. The familial occurrence of multiple intestinal atresias is even more unusual. Also fetal death with isolated intestinal atresia is very rare, and the combination of recurrent familial intestinal atresias and intra-uterine fetal death has not been reported previously. We report on two cases of intra-uterine fetal death in a 30-year-old woman who is consanguineous with her husband and they have only one boy who carries chromosomal abnormality. To best of our knowledge this is the first reported case of two consecutive pregnancies with multiple intestinal atresias leading to intrauterine fetal death. Multiple and recurrent intestinal atresias are extremely uncommon. These rare cases may contribute to genetic mapping for intestinal atresias

Retinopathy of prematurity: Single versus multiple-birth pregnancies

To compare the frequency and severity of retinopathy of prematurity [ROP] among singleton and multiple-birth neonates referred to Farabi Eye Hospital, Tehran-Iran. In this retrospective study, records of 99 consecutive neonates from multiplegestation pregnancies including 68 twins, 26 triplets and 5 quadruplets who were screened for ROP from 2002 to 2004 were reviewed. The frequency, severity and risk factors for ROP were determined and compared to a group of singletons who were matched in terms of gender, birth weight [BW], gestational age [GA], oxygen therapy, respiratory distress syndrome, blood transfusion, sepsis and phototherapy. ROP was present in 12.1% of multiple-birth neonates as compared to 15.1% of singletons [P=0.53]. Threshold ROP was present in 6.1% of multiple-birth neonates versus 7.1% of singletons [P=0.62]. ROP was detected in 60% of quadruplets versus 9.6% of twins and triplets; threshold disease was observed in 40% of quadruplets as compared to 4.2% of twins and triplets [P<0.03]. However, considering the effect of BW and GA, logistic regression analysis revealed no statistically significant difference in the frequency and severity of ROP among subgroups of multiple-gestation pregnancies. There was no significant difference between multiple-birth neonates and matched singletons in terms of frequency and severity of ROP. Any apparent higher rate may be due to independent risk factors such as low birth weight and gestational age rather than multiple pregnancies per se. Screening for ROP in multiple gestation births may be conducted according to standard protocols applied for singletons

Neonatal mortality risk assessment in a neonatal intensive care unit [NICU]

This study aims to assess the utility of a scoring system as predictor of neonatal mortality rate among the neonates admitted within one year to the neonatal intensive care unit [NICU] of the Children's Medical Center in Tehran, Iran. Data were gathered from 213 newborns admitted to the NICU from September 2003 to August 2004. In addition to demographic data, Apgar scores at 1 minute and 5 minutes, history and duration of previous hospital ization, initial diagnosis and final diagnosis, and scoring system by using the score for the neonatal acute physiology-perinatal extension II [SNAP-PE II] were carried out within 12 hours after admission to the NICU. All of the parameters were prospectively applied to the admitted newborns. The exclusion criteria were discharge or death in less than 24 hours after NICU admission. 198 newborn infants met the inclusion criteria. The mean and standard deviation [SD] of the variables including postnatal age, birth weight, SNAP, and finally Apgar scores at 1 minute and 5 minutes of neonates under this study were 7.6 [0.5] days, 2479.8 [29.4] grams, 21.6 [1.1], 7.47 [0.08], and 7.71 [0.06], respectively. Twenty five of the 198 patients died [12.6%]. Gestational age [p=0.03], birth weight [P=0.02], Apgar score at 5 minutes [0.001], and SNAP-PE II [P=0.04] were significantly related to the mortality rate. By Analyzing through logistic regression to evaluate the predictive value of these variables in relation to the risk of mortality, it was shown that only SNAP-PE II and Apgar score at 5 minutes could significantly predict the neonatal mortality. According to this study SNAP-PE II and Apgar score at 5 minutes can be used to predict mortality among the NICU patients. SNAP-PE II score had the best performance in predicting mortality in this study. More studies with larger samples are suggested to evaluate all of the above-mentioned parameters among neonates who are admitted to NICUs countrywide

Angiotensin converting enzyme inhibitors and aortic arch obstructive malformations.

We describe two newborn infants with aortic arch obstructive malformations who became anuric after initiation of captopril. Since angiotensin converting enzyme inhibitors can alter renal blood flow by reduction in angiotensin II and blocking autoregulation phenomenon, it is important to use them with great caution in neonates with aortic arch obstructive malformations, while monitoring their renal function closely.

Recombinant erythropoietin and blood transfusion in very low birth weight infants

Very low birth weight infants [<1500 g] frequently require blood transfusions because of repeated blood sampling accompanied by anemia of prematurity. In an attempt to identify the effect of human recombinant erythropoietin to decrease the requirement for blood transfusions, erythropoietin was administered to 24 preterm infants less than 1500 g prospectively from September 1999 till December 2000.Data about the characteristics of the population, the severity of diseases, and treatment with erythropoietin, clinical diagnosis, initial and subsequent hemoglobin, volume of blood loss, and the number of blood transfusions were recorded. These results were compared with data from the recorded information of 49 infants who did not receive erythropoietin during those past 2 years. There were no differences between the 2 groups with regard to the gestational age, birth weight, clinical diagnosis, severity of the illness, primary causes of admission, and initial hematologic parameters such as hemoglobin, hematocrit and reticulocytes. Erythropoietin was administered in a dose of 200 IU/kg three times weekly for 6-8 weeks accompanied with iron supplement 6 mg/ kg/day. Transfusions were administered according to protocol.There was no significant difference between the number of blood transfusion among these 2 groups [p= 0.07]. However, transfusions in the erythropoietin treated group were fewer in comparison to the other group [1.9 +/- 1.6 to 3.2 +/- 1.1]. No difference was observed between final hemoglobin and hematocrit levels among the two groups [10.3 +/- 0.9 vs. 10.4 +/- 0.7 and 33.7 +/- 2.3 vs. 32.2 +/- 2.2].Very low birth weight infants receive frequent blood transfusions but a reduction in transfusion requirements was not apparent after administration of erythropoietin and iron in preterm infants in this study. However, the lack of impact on transfusion requirements fails to support routine use of erythropoietin