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 15,856 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

This article reviews the pathophysiology of congenital defects and tumors that are of interest to the pediatric surgeon. Reliable information has been obtained through prenatal diagnosis of fetal anomalies and careful studies of fetal animal models. The topics covered include fetal diaphragmatic hernia, oligohydramnios-induced pulmonary hypoplasia, renal dysplasia, prune belly, gastrointestinal obstruction, biliary atresia, Wilms' tumor, and neuroblastoma. In addition, some recent experimental studies delineating the ability of the fetus to heal without scarring may have implications for all surgeons.
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PMID:The developmental pathophysiology of surgical disease. 806 34

The objective of this review is to discuss the current knowledge of fetal pulmonary hypoplasia and to summarize the clinical significance of the many ultrasound methods for predicting pulmonary hypoplasia in pregnancies complicated by oligohydramnios due to spontaneous rupture of the fetal membranes and in cases complicated by fetal congenital diaphragmatic hernia. We concluded that the presence or absence of polyhydramnios, fetal breathing movements, mediastinal shift, thoracic position of the stomach, fetal breathing-related nasal and oropharyngeal fluid flow, ductal flow velocity modulation, and gestational age at onset and severity of ventricular disproportion as useful markers for predicting fetal pulmonary hypoplasia is a productive area for continued research. All studies show that there is a clear association between most of these markers and pulmonary hypoplasia. However, these markers have not been studied together in a large number of cases, and comparisons between each of the markers is unknown.
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PMID:Ultrasound prediction of fetal pulmonary hypoplasia in pregnancies complicated by oligohydramnios and in cases of congenital diaphragmatic hernia: a review. 819 48

Three preterm infants are described who had respiratory failure immediately after birth. For each, historical factors (extrauterine abdominal pregnancy; ruptured membranes with oligohydramnios for 14 weeks; congenital diaphragmatic hernia) and the initial postnatal course were suggestive of pulmonary hypoplasia. However, with vigorous resuscitation each infant showed rapid improvement in pulmonary status for several hours. Two infants survived with no apparent pulmonary sequelae; the third had almost complete resolution of pulmonary disease but died from gastrointestinal complications. Thus some infants with historical and clinical indicators of severe pulmonary hypoplasia do not have hypoplastic lungs and respond favorably to resuscitation. The causes of the respiratory distress and of its rapid resolution are not known. On the basis of this experience, even infants with historical factors suggesting pulmonary hypoplasia and with postnatal respiratory distress should receive vigorous resuscitation.
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PMID:Transient severe respiratory distress mimicking pulmonary hypoplasia in preterm infants. 822 32

The hypothesis of this article is that growth of the fetal lung is stimulated by a pulmonary growth factor (PGF) produced by the kidneys, which is modulated by a feedback signal from the lungs, a pulmonary-derived renotropin (PDR). In the fetus with pulmonary hypoplasia (PH), the lungs may maximally stimulate this feedback loop to release more PDR, resulting in continual stimulation of the kidneys and renal enlargement. If such a schema plays a role in the pathophysiology of PH, newborn infants with congenital diaphragmatic hernia (CDH) or chronic amniotic fluid leak (CAFL) should have enlarged kidneys. To investigate this hypothesis, we created models of CDH in fetal lambs and CAFL in fetal rabbits, and then analyzed lung (Lu) and kidney (K) growth. When compared to controls, newborn CDH lambs had significantly smaller lungs and larger kidneys. The lungs were hypoplastic as defined by either decreased lung weight/body weight (LuW/BW), lung DNA/body weight (Lu DNA/BW), or lung total protein/body weight (LuTP/BW) (P < .01). Renal hyperplasia was confirmed by KW/BW, K DNA/BW (P < .01), and KTP/BW (P < .05). An inverse relationship between lung size and kidney size could be described by the equation KW/BW = 1.04 - 0.12 LuW/BW (r = -.75). The CAFL model in newborn rabbits produced severe oligohydramnios when compared with controls (P < .01). This resulted in fetuses with smaller lungs and larger kidneys as compared with those of controls. The lungs were significantly smaller and more hypoplastic than controls when compared by LuW (P < .01), LuW/BW (P < .01), Lu DNA/BW (P < .05), and Lu TP/BW (P < .01).(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Pathophysiology of congenital diaphragmatic hernia. IV: Renal hyperplasia is associated with pulmonary hypoplasia. 846 63

Gastrin-releasing peptide (GRP) is a developmentally regulated bioactive peptide believed to function as a pulmonary growth factor. It is produced by pulmonary neuroendocrine cells, found within the conducting and respiratory epithelium, as isolated cells and in clusters known as neuroepithelial bodies (NEBs). Deficient GRP expression has been reported in pulmonary hypoplasia (PH) associated with oligohydramnios and diaphragmatic hernia. To assess further the role of GRP in maldeveloped lung we reviewed the postmortem records and histologic lung sections, stained with H&E and anti-GRP antiserum, from 11 infants with anencephaly and 11 age-matched controls. Cells immunoreactive for GRP were quantified (isolated versus NEBs) in airways and airspaces per mm2 for a standard area. PH was present in five anencephalic infants. There was no difference in the total number of GRP-positive cells, number of NEBs, size of NEBs, or number of GRP-positive cells in airways or alveoli in either group regardless of lung development. A greater proportion of the GRP-positive cells was present in the airways in anencephalic infants with PH (58%) compared with anencephalic infants without PH (40%) (P = .018). There were no differences when comparing these groups with control infants and no differences in the density of airways in each of these groups. We conclude that deficient GRP expression is not a feature of lung hypoplasia in anencephalic infants. The altered distribution of GRP-positive cells in anencephalic infants with PH may be a reflection of the structural abnormalities or accompanying altered cellular maturity.
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PMID:Pulmonary gastrin-releasing peptide expression in anencephaly. 859 25

This study departed from a preconceived definition of VACTERL, including more than one of these six anomalies in the same infant: V (vertebral anomalies), A (anal atresia), C (congenital heart disease), TE (tracheoesophageal fistula or esophageal atresia), R (reno-urinary anomalies), and L (radial limb defect). Under this definition, 524 infants were ascertained by ECLAMC from almost 3,000,000 births examined from 1967 through 1990. Observed association rates among VACTERL components as well as between VACTERL and other defects were compared against randomly expected values obtained from 10,084 multiply malformed infants (casuistic method) from the same birth sample. Conclusions were: 1) Cardiac defects are not a part of VACTERL. 2) Single umbilical artery, ambiguous genitalia, abdominal wall defects, diaphragmatic hernia, and anomalies that are secondary to VACTERL components (intestinal and respiratory anomalies, and oligohydramnios sequence defects) are frequent enough to be considered an "extension" of VACTERL, and cardiac defects should be included in this category. 3) Neural tube defects are negatively associated with VACTERL which could not be explained by selection bias or any other operational artifact. High embryonic lethality or mutually exclusive pathogenetic mechanisms could be suitable explanations. 4) Results were not clear enough to determine whether VACTERL should be defined by at least two or three component defects.
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PMID:VACTERL association, epidemiologic definition and delineation. 882 30

Pulmonary hypoplasia(PH) commonly occurs in association with oligohydramnios and other congenital anomalies, especially congenital diaphragmatic hernia (CDH). Pulmonary hypoplasia is an important factor, as persistent pulmonary hypertension, in the prognosis of CDH. In some reports, there is a decrement of pulmonary surfactant in PH accompanying CDH. Recently, there are some reports that exogenous pulmonary surfactant therapy is effective in experimental animal model and neonatal respiratory distress with PH. We report a case of a 5 day-old male neonate, who had shown dyspnea and diagnosed as left pulmonary hypoplasia accompanying CDH. The CDH was surgically treated and the ipsilateral PH, with intratracheal administration of exogenous pulmonary surfactant postoperatively. After exogenous pulmonary surfactant application, the left lung volume was increased on chest roentgenogram and lung perfusion scan findings, and there was an improvement in oxygenation and clinical manifestations. We suggest that postoperative exogenous pulmonary surfactant replacement therapy is effective in the case of PH and further trials are needed to clarify the optimal dose and timing of supplementation of surfactant for treatment of infants with PH accompanying CDH.
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PMID:Exogenous pulmonary surfactant replacement therapy in a neonate with pulmonary hypoplasia accompanying congenital diaphragmatic hernia--a case report. 884 10

Transient neonatal diabetes mellitus is an uncommon disorder. Macroglossia in association with transient neonatal diabetes mellitus has been reported only twice before. We report the case of a 21-day-old male infant referred from a peripheral hospital for management of hyperglycemia. The mother was a 21-year-old primigravid in good health. There was no history of diabetes or drug or alcohol exposure. The pregnancy was complicated by intrauterine growth retardation and oligohydramnios from 30 weeks' gestation and the birth weight at 38 weeks' gestation was only 1480 gm. Physical examination revealed dysmorphic features and asymmetric growth retardation. The admission weight (1840 gm) and length (40.5 cm) were 5 SDs less than the mean and head circumference (32.5 cm) was 1 SD less than the mean. Dysmorphic features included macroglossia, large fontanelles, hypospadias, umbilical hernia, and bilateral inguinal hernias. Hyperglycemia had been noted on day 1 of life with an initial blood glucose value of 16 mmol/L (288 mg/dl). Despite treatment with regular insulin blood glucose control continued to be erratic. Therefore a regimen of daily NPH insulin was begun, which has a smoother action. Interestingly, from day 41 to day 47 the infant did not receive insulin and a crude control of the blood glucose was demonstrated. Peak levels of blood glucose in excess of 20 mmol/L (360 mg/dl) were followed by drops to levels less than 2 mmol/L (36 mg/dl) without insulin administration. This abnormal pattern of glucose control may represent poorly regulated release of endogenous insulin. However, because of unsatisfactory glucose levels administration of daily NPH insulin was reintroduced. The infant was discharged from the hospital on day 50 and administration of insulin was discontinued uneventfully at 9 months. At 1 year the hemoglobin A1c level was still normal and the infant's weight was at the 10th percentile. Macroglossia was less pronounced. Development showed mild delay in gross motor milestones.
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PMID:Transient neonatal diabetes mellitus and macroglossia. 886

Aside from recognized overgrowth syndromes, instances of visceromegaly are not uncommon at perinatal autopsy. The database of the University of Michigan Teratology Unit was screened for individual viscera exceeding the 90th centile for body and brain weight standards. The data were stratified for several maternal (hypertension, diabetes, obesity), gestational (chorioamnionitis, oligohydramnios, amniorrhaea, polyhydramnios), and fetal (body wall defect, cardiac malformation, renal malformation, diaphragmatic hernia, nonimmune hydrops, twin transfusion syndrome) characteristics and tested for statistically significant excessive numbers of heavy organs. The most striking associations were heavy adrenal glands and liver with chorioamnionitis, heavy heart with polyhydramnios and in the twin transfusion syndrome, and heavy heart and liver with nonimmune hydrops. Excessive brain weight for body weight had a number of correlations, each most likely reflecting growth restriction with sparing of brain growth.
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PMID:Correlates of prenatal visceromegaly. 978 3

Ultrasonic diagnosis is indispensable in perinatal medicine. The applications are; 1) diagnosis of early pregnancy, 2) diagnosis of fetal life, 3) diagnosis of pregnancy weeks by fetal crown rump length, 4) evaluation of fetal growth by biparietal diameter, femur length, abdominal size, or estimated fetal weight, 5) detection of early abnormalities in blighted ovum, fetal death, hydatidiform mole, ectopic pregnancy, etc., 6) diagnosis of fetal anomalies, e.g. anencephaly, hydrocephaly, neck hygraoma, diaphragmatic hernia, congenital heart disease, intestinal obstruction, renal anomalies, obstructive uropathy, etc., 7) diagnosis of fetal diseases, e.g. hydrops fetalis, 8) diagnosis of placenta previa, hydramnios, oligohydramnios, cord coiling, excessive cord twisting, 9) detection and prediction of fetal compromise by fetal and uterine blood flow velocity wave forms, 10) further precise diagnosis by transvaginal sonography, 11) interventional ultrasound in genetic diagnosis with amniocentesis and chorionic villi sampling, fetal blood sampling with cordocentesis, 12) detection of fetal hypoxia by ultrasonic fetal monitor, fetal actocardiogram, and ultrasonic Doppler flowmetry.
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PMID:Ultrasound in fetal diagnosis and therapy. 1012 69


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