Necrosis of the left buttock as a complication of umbilical catheterisation in neonatal resuscitation.
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Buttocks↗
Biomedical subjects
P G J Nikkels
Publications and source records attributed to P G J Nikkels.
Significance of donor anuria differs between monoamniotic and diamniotic twin-twin transfusion syndrome.
Development of severe twin-twin transfusion syndrome (TTTS) in diamniotic-monochorionic twins includes five stages of increasing severity, i.e. recipient polyhydramnios and donor oligohydramnios, donor anuria, abnormal umbilical flow velocities in either twin, hydrops in the recipient, and intrauterine fetal death (IUFD) in either or both twins. In a severe case of TTTS in monoamniotic twins we observed donor anuria to appear after hydrops in the recipient. We conclude that donor anuria is a late and serious symptom in monoamniotic TTTS.
Adult↗
Nitrotyrosine in brain tissue of neonates after perinatal asphyxia.
HYPOTHESIS: Nitrotyrosine, a reaction product of peroxynitrite and proteins, could be demonstrated in the postmortem examination of brain tissue of full-term neonates who had severe perinatal asphyxia. METHODS: The brain tissue of 22 full-term neonates who died after severe perinatal asphyxia was examined, including cerebral cortex, basal ganglia, thalamus, hippocampus, brain stem, olives and cerebellum. Median age at death was 52 h. Routine histopathological examination and additional immunohistological staining were carried out with anti-cysteine protease protein 32 antibodies to detect activated caspase 3, anti-nitrotyrosine antibodies to detect nitrotyrosine and anti-CD68 antibodies to detect activated microglia and macrophages, which might be associated with the production of nitric oxide. Staining was scored as none, weak (1-25% positive cells), moderate (26-75% positive cells) or severe (>75% positive cells). RESULTS: 14 patients showed global injury, 4 showed injury of the basal ganglia and thalamus, and 4 showed predominantly parasagittal brain injury. One neonate without perinatal asphyxia served as a control. Nitrotyrosine staining of neurones was shown in all neonates with asphyxia, mostly in the thalamus (70%) and inferior olives (68%). Total nitrotyrosine staining tended to be less in the base of the pons and inferior olives of neonates with parasagittal brain injury. Activated caspase 3 was found mostly in the thalamus (60%) and hippocampus (53%). Positive CD68 staining was mainly present in the thalamus (70% positive). CONCLUSION: Nitrotyrosine was found in brain tissue of full-term neonates, suggesting that nitric oxide toxicity might have a role in hypoxic-ischaemic brain injury at term. This may be relevant for neuroprotective strategies in full-term neonates with perinatal asphyxia.
Antigens, CD↗
Colour oscillations in arterioarterial anastomoses reflect natural differences in donor and recipient oxygenation and hematocrit.
Our aim was to show that the colour difference between brighter and darker red, occasionally observed as an oscillating boundary in the recipient and donor parts of an arterioarterial anastomosis in severe twin-twin transfusion syndrome (TTTS), is a consequence of natural differences in blood oxygenation and hematocrit developing between donor and recipient twins. As method we defined a theoretical model of the placenta with dimensions from pathology examination. From literature we determined the optical absorption and scattering properties of all tissue components, and hematocrit and oxygen saturation values for donor and recipient twins. From our placental model we simulated the spectrum of back-scattered light by standard Monte Carlo photon propagation computations and calculated the colour of chorionic arterial and venous blood vessels by applying the physics theory of colour perception. Our computations demonstrate that recipient arterial blood is somewhat brighter red than donor arterial blood. The strong colour differences seen after laser coagulation of all anastomoses but the arterioarterial were explained from an angiotensin II cut-off in the recipient due to obliteration of arteriovenous anastomoses, causing a temporary increase in recipient placental perfusion and hence in blood oxygenation. In conclusion, natural differences in recipient versus donor blood oxygen saturation and hematocrit in severe TTTS explain the observed colour differences between brighter and darker red observed in the recipient and donor parts of arterioarterial anastomoses.
Arterio-Arterial Fistula↗
Prenatal ultrasonographic prediction of the umbilical coiling index at birth and adverse pregnancy outcome.
OBJECTIVES: To evaluate whether the antenatal umbilical coiling index (aUCI) as measured by ultrasonography predicts the postnatal umbilical coiling index (pUCI) and adverse pregnancy outcome. METHODS: In a prospective study in 117 pregnancies, the aUCI was measured between 28 weeks and term by ultrasonography. The aUCI was calculated as the reciprocal value of the mean pitch of one complete coil. The pUCI was calculated as the number of coils divided by the cord length in cm. The correlation between aUCI and pUCI was assessed and likelihood ratios for adverse pregnancy outcome were calculated. RESULTS: We had complete data on 81 subjects. Mean aUCI +/- SD was 0.30 +/- 0.09 and mean pUCI +/- SD was 0.17 +/- 0.08. The correlation coefficient between aUCI and pUCI was 0.66, P < 0.001. Limits of agreement were 0-0.28 coils/cm. The positive likelihood ratio for small-for-gestational-age infants was 2.6 (95% confidence interval (CI) 0.6-11.6) for ultrasound hypocoiling, and 5.7 (95% CI 1.3-24.8) for ultrasound hypercoiling. The positive likelihood ratio for interventional delivery for non-reassuring fetal status was 1.2 (95% CI 0.2-9.0) for ultrasound hypocoiling, and 10.3 (95% CI 2.1-50.2) for ultrasound hypercoiling. CONCLUSIONS: Strong correlation coefficients comparing the aUCI and pUCI do not reflect agreement. Since the limits of agreement were almost as wide as the full range for the pUCI, the aUCI does not predict the pUCI with sufficient precision. Larger prospective studies are required to confirm the predictive potential of the aUCI for adverse pregnancy outcome.
Adult↗
Striking facial dysmorphisms and restricted thymic development in a fetus with a 6-megabase deletion of chromosome 14q.
During routine ultrasound screening at 12 weeks 5 days of gestation, a nuchal translucency of 7 mm, an omphalocele, and fetal hydrops were found and prompted chorionic villus sampling at 13 weeks 2 days. Chromosome analysis showed an unbalanced karyotype with an abnormal chromosome 14. The mother was a carrier of a translocation karyotype 46,XX,t(13;14) (q34;q32.2). In the fetus this gave rise to a partial trisomy 13q and partial monosomy 14q (fetal karyotype: 46,XX,der[14]t[13;14][q34;q32.2]). By Array-CGH on DNA extracted from a postmortem skin culture, a duplication of approximately 1.7 Mbp of the distal part of chromosome 13q34 and a deletion of approximately 6.0 Mbp of the distal part of chromosome 14q32.2 was demonstrated. Postmortem findings after termination of pregnancy at 14 weeks 6 days included, among others, a severe hypoplasia of the median part of the maxilla, no recognizable nose, a broad median palatoschisis, nonlobulated lungs, a horseshoe kidney with multicystic dysplasia, and decreased development of cortical cellularity in the thymus. These clinical manifestations and autopsy findings of the fetus are compared with those of previously published cases and the possible involvement in this pathology of the YY1 and JAG2 transcription factors and the BCL11b and SIVA-1 regulators of thymic development is discussed.
Abnormalities, Multiple↗
Complex cardiac defect with hypoplastic right ventricle in a fetus with valproate exposure.
We describe a fetus with a hypoplastic right ventricle detected by prenatal ultrasound examination. A possible causal relationship with prenatal valproate exposure is discussed.
Abortion, Induced↗
Concomitant anomalies in 100 children with unilateral multicystic kidney.
OBJECTIVES: To determine the incidence and type of associated urogenital anomalies in children with a unilateral multicystic kidney and to assess in children with nephrectomy the additional diagnostic value of cystoscopy and, in girls, of colposcopy. METHODS: This was a follow-up study of 100 fetuses with antenatally detected unilateral multicystic kidneys. After ultrasound confirmation of the diagnosis within a few days after birth voiding cystourethrography and isotope scan were performed in 83 of the surviving children to exclude vesicoureteral reflux and to establish renal function. Eighty-one children underwent nephrectomy and, prior to surgery, all underwent cystoscopy and girls also underwent colposcopy. RESULTS: Seventy-five children had one or more additional urogenital anomalies: 39 had anomalies of the contralateral kidney, 40 had anomalies of the ipsilateral kidney and 30 had one or more anomalies of the lower urogenital tract. With cystoscopy 54 anomalies of the genitourinary tract were detected in 48 children and with colposcopy three anomalies were detected in 35 girls. Eighty-one children had a nephrectomy or heminephrectomy and 33 of them needed other urological intervention. Thirteen fetuses died (mostly from agenesis of the contralateral kidney) and six infants had no surgery at all. CONCLUSION: Children with a unilateral multicystic kidney are at considerable risk of having other urogenital anomalies. When cystoscopy and colposcopy are added to routine investigations the rate of detection of anomalies is 75%, twice that reported in the literature.
Abnormalities, Multiple↗
Further evidence for an ischemic origin of perforation of the appendix in the neonatal period.
A full-term baby girl presented on the 14th day of life with an appendiceal abscess on a basis of appendicular perforation. Pathologic examination found focal transmural coagulation necrosis suggesting an ischemic origin for the perforation. It is argued that appendiceal perforation in the newborn period is a different disease entity than appendiceal perforation later in life. In the newborn, ischemia seems to be the leading pathogenetic factor, and neonatal appendiceal perforation seems, therefore, related to neonatal necrotizing enterocolitis.
Abscess↗
Distribution of glutamate transporters in the human placenta.
Glutamate metabolism is known to be important for growth and development of the human fetus. The glutamate transporters EAAT1, EAAT2 and EAAT3 are key components of the glutamate-glutamine cycle and responsible for active transport of glutamate over the cell membrane. The placenta is thought to regulate glutamate transport during fetal development. Glutamate transporters have been found in placentae of rats, but their distribution in the human placenta is unknown. Therefore, the distribution of glutamate transporters EAAT1, EAAT2 and EAAT3 were analysed in the human placenta during normal pregnancies ending between 8 and 40 weeks of gestation and in placentae of intrauterine growth restricted infants with gestational ages between 28 and 35 weeks of pregnancy. Using immunohistochemistry, EAAT1 expression was found in the syncytiotrophoblast layer, while EAAT2 was detected in the syncytiotrophoblast layer and in endothelial cells of about 5 per cent of all fetal blood vessels. EAAT3 was observed in the endothelium of the fetal blood vessels in all placentae examined. However, expression was also found in the syncytio- and the cytotrophoblast layer of the fetal villi at 8 weeks of gestational age. The expression patterns of EAAT1, EAAT2 and EAAT3 suggest involvement in active transport of glutamate between the fetal and maternal blood circulation. No differences were found in the distribution of the glutamate transporters between control and IUGR placentae. Our data show specific localization of EAAT1, EAAT2 and EAAT3 in the human placenta during development.
Amino Acid Transport System X-AG↗
Moyamoya and extracranial vascular involvement: fibromuscular dysplasia? A report of two children.
We present two patients with moyamoya syndrome and the unusual involvement of extracranial vessels. The first case illustrates the rare association between moyamoya and primary pulmonary hypertension. In the second patient, moyamoya was complicated by stenoses of vertebral, renal, and mesenteric arteries. In both cases, a systemic intima-proliferative disease, such as fibromuscular dysplasia (FMD), was suspected to be the cause of both intracranial and extracranial arterial disease.
Child↗
Intestinal function in glycogen storage disease type I.
Glycogen storage disease type I (GSD I) (McKusick 232200) is caused by inherited defects of the glucose-6-phosphatase complex. Patients with GSD Ia as well as patients with GSD lb may suffer from intermittent diarrhoea, which seems to worsen with age. The cause of this diarrhoea is unknown. This study describes the results of investigations of intestinal functions and morphology in patients with GSD Ia and GSD lb, which were performed to detect a common cause for chronic diarrhoea in GSD I. The following were investigated: faecal fat excretion, faecal alpha1-antitrypsin and faecal chymotrypsin, expiratory H2 concentrations, persorption of cornstarch in urine and colonic biopsies. With the investigations presented in this study, no common cause for diarrhoea in GSD I was found. In GSD lb loss of mucosal barrier function due to inflammation, documented by increased faecal alpha1-antitrypsin excretion (3.5-9.6 mg/g dry faeces) and inflammation in the colonic biopsies, seems to be the main cause. The inflammation is most likely related to disturbed neutrophil function, which is often found in GSD lb. Whether another cause is involved in GSD Ia and in GSD Ib, related to the disturbed function of glucose-6-phosphatase in the enterocyte, remains to be investigated.
Adolescent↗
Placental pathology in early onset pre-eclampsia and intra-uterine growth restriction in women with and without thrombophilia.
OBJECTIVE: The incidence of placental thrombotic lesions in early onset preeclampsia (PE) and/or intrauterine growth restriction (IUGR) were compared between women with and without thrombophilia or hyperhomocysteinemia. STUDY DESIGN: Matched case-control study. 183 women with a history of early onset PE and/or IUGR were tested for thrombophilia and hyperhomocysteinemia. From the 66 women with a thrombophilic factor the placental histological slides were available in 47 women. These were matched for maternal condition (PE and/or IUGR), gestational age at delivery, parity and maternal age, to 47 women with no thrombophilic factor. All slides were revised for lymphohistiocytic villitis, fetal thrombosis and fibrin depositions. RESULTS: There were no significant differences between the placentas of the matched groups with and without a thrombophilic factor. CONCLUSION: Placental thrombotic and inflammatory lesions associated with early onset PE and/or IUGR do not occur more often in women with compared to women without thrombophilia or hyperhomocysteinemia.
Case-Control Studies↗
The umbilical coiling index in normal pregnancy.
OBJECTIVE: To provide reference values for the umbilical coiling index in uncomplicated pregnancy. METHODS: Umbilical cords were collected from livebom singleton infants born after uncomplicated pregnancies. The umbilical coiling index (UCI) was calculated as the number of coils divided by the cord length in centimeters. The mean value (SD) for the UCI was calculated, and possible correlations of the UCI with maternal age, parity, gestational age at delivery, mode of delivery, sex and birth weight of the infant were examined. RESULTS: A total of 122 umbilical cords were included. The frequency distribution of the UCI was skewed to the right. The mean (SD) UCI was 0.17 (0.009) coils/cm. There were no significant correlations of the UCI with maternal age, parity, gestational age at delivery, mode of delivery, sex or birth weight of the infant. CONCLUSIONS: This is the first study to determine the UCI in a group exclusively consisting of uncomplicated pregnancies. The mean value that we found for the UCI may serve as the standard reference, allowing proper interpretation of umbilical coiling in complicated pregnancy.
Adult↗
Placental pathology in women with type 1 diabetes and in a control group with normal and large-for-gestational-age infants.
Unexplained intra-uterine fetal death is still a problem in diabetic pregnancies, especially in those with an LGA-infant. We hypothesized that in these pregnancies impaired placental function, in terms of abnormal placental weight and/or abnormal placental histology, may account for this phenomenon. To test this hypothesis, we assessed the relative placental weight and scored several histological abnormalities in 34 AGA- and 24 LGA-placentae of type 1 diabetic women and in 22 AGA- and 16 LGA-placentae of control women. Relative placental weight was comparable in the LGA-diabetic cases and in the control groups, but was significantly higher in the AGA-diabetic subgroup. Histological abnormalities such as the presence of nucleated fetal red blood cells, fibrinoid necrosis, villous immaturity and chorangiosis were observed more often in the diabetic placentae compared with the control placentae. These differences in histology were particularly observed when we compared both AGA-groups. LGA-control placentae showed a high incidence of histological abnormalities, almost comparable to the diabetic placentae. Only fibrinoid necrosis was significantly more common in the LGA-diabetic placentae. Three of the four cases of perinatal death/asphyxia in the diabetic group concerned an LGA-infant with a relative low placental weight. In conclusion, placentae of women with type 1 diabetes showed several abnormalities that can be associated with impaired functioning. The difference between AGA- and LGA-diabetic placentae was related to relative placental weight and our data suggest that an increase in relative weight may protect the fetus from asphyxia. Placentae from LGA-non-diabetic women showed several similarities to those of women with diabetes.
Adult↗
Monochorionic twins and twin-twin transfusion syndrome: the protective role of arterio-arterial anastomoses.
Unidirectional arterio-venous (AV) anastomoses often result in twin-twin transfusion syndrome (TTTS). Additional oppositely directed anastomoses may compensate for the circulatory imbalance and either prevent, delay the onset, or moderate the severity of TTTS. Intuitively, higher pressure gradient, oppositely-directed AV anastomoses (indicated as VA) would be expected to compensate better for TTTS than lower pressure gradient arterio-arterial (AA) anastomoses. However, clinical evidence suggests AA anastomoses compensate more efficaciously, because virtually all non-TTTS monochorionic twin placentas have AAs (84 per cent), contrary to TTTS placentas, where only 30 per cent have an AA. We sought to explain this observation by comparing the capabilities of various size VA and AA anastomoses to compensate for the effects of the primary AV. As study design we used a previously developed mathematical computer model of TTTS to determine ranges of anastomotic vascular resistances which cause varying fetal and amniotic fluid discordances. Anastomotic resistances were related with the radii of their feeding vessels, using fractal geometry modelling to mimic the placental vascular tree, and various assumptions regarding arterial blood flow. The results were as follows. An AA anastomosis of equal size as the feeding artery of an AV or VA has a significantly smaller resistance. The primary AV anastomosis may be compensated by both VA as well as AA anastomoses. However, VA transfusion adequately compensates AV flow only for a small range of VA to AV vascular radius ratios. In contrast, AA transfusion compensates the AV flow for a much wider range of AA to AV vascular radius ratios. In conclusion, the wider range of AA than VA radii for adequate compensation of the AV explains the finding that an AA protects more frequently than a VA of similar size against the manifestations of TTTS. These results may possibly allow future risk stratification of monochorionic twins by non-invasive sonographic assessment of the size and type of anastomoses.
Adult↗
Expression of Ro/SS-A and La/SS-B determined by immunohistochemistry in healthy, inflamed and autoimmune diseased human tissues: a generalized phenomenon.
OBJECTIVE: Autoantibodies to the ribonucleoproteins Ro/SS-A and La/SS-B are found in autoimmune diseases such as primary Sjögren's syndrome (pSS), systemic lupus erythematousus and rheumatoid arthritis. Increased and aberrant expression of Ro/SS-A and La/SS-B in target organs, which have been reported in the recent literature, might contribute to their antigenicity. However, data on the expression of Ro/SS-A and La/SS-B in other inflammatory conditions are scarce. MATERIALS AND METHODS: Using monoclonal antibodies against Ro/SS-A and La/SS-B, we studied the expression of these antigens in paraffin-embedded healthy tissue, aspecific inflamed tissue, the neonatal and adult cardiac conduction systems and labial salivary gland tissues of patients suspected of having pSS. RESULTS: In healthy tissues, the nuclei expressed both Ro/SS-A and La/SS-B. This expression was stronger in inflamed tissues. Nucleoli were negative and cytoplasmic expression was weaker than nuclear expression. No increased or aberrant expression of Ro/SS-A or La/SS-B was observed in either neonatal or adult atrioventricular nodes and bundle branches. More pSS patients showed high La/SS-B immunoreactivity levels in their labial salivary gland ductal cell nuclei than non-Sjögren's syndrome sicca patients. CONCLUSIONS: Ro/SS-A and La/SS-B expression is a generalized cell biological phenomenon and may be upregulated by increased cell activation both in aspecific and autoimmune-mediated inflammation. In pSS the high expression of La/SS-B in labial salivary, gland ductal cell nuclei might contribute to the local immune response.
Adult↗