Pregnancy onset congenital thrombotic thrombocytopenic purpura (Upshaw-Schulman syndrome) mimicking HELLP syndrome: a case report

Objective Thrombotic thrombocytopenic purpura is a thrombotic microangiopathic condition characterized by hemolytic anemia, thrombocytopenia, neurologic abnormalities, fever and renal dysfunction. Thrombotic microangiopathies such as preeclampsia and HELLP syndrome are pregnancy-specific, whereas others such as thrombotic thrombocytopenic purpura (TTP) and hemolytic uremic syndrome are not. In this report, we present a case at which we identified a novel mutation which led to a significant reduction of ADAMTS13 activity. Case(s) A nulliparous pregnant woman of 32-year-old presenting with epigastric pain, hypertension and low platelet count was first suspected of HELLP syndrome, but was diagnosed with congenital TTP after delivery. Conclusion HELLP syndrome co-existed with undiagnosed TTP in this case. We strive to have sufficient awareness in order to distinguish these two pathologies from each other on an antenatal basis, because the causes of the managements are entirely different.

Diagnostic utility of protein excretion in 24-hour urine and the protein to creatinine ratio for adverse perinatal outcomes and time of delivery

Objective Our study aimed to evaluate the perinatal and neonatal outcomes of hypertensive pregnant women with or without proteinuria. We compared the predictivity of spot urinary protein to creatinine (P/C) ratio and 24-hour protein excretions on outcomes. Methods We retrospectively enrolled 230 pregnant women with a new diagnosis of hypertension between 20 and 37 weeks of gestation. We divided the patients into two groups according to the protein level determined by 24-hour urine collection and P/C ratio. The presence and level of proteinuria, its relationship with the P/C ratio, and the relationship between these findings and perinatal outcomes were evaluated. Results Gestational age at delivery weeks and latency period (duration between diagnosis of hypertension and delivery) were significantly earlier in pregnant women with ≥300 mg/24-h and P/C ratio ≥0.3. Adverse neonatal outcomes were significant in patients with proteinuria ≥300 mg/24-hour and P/C ratio ≥0.3. Urinary protein levels in 24-hour urine were significantly higher in pregnant women with P/C ratio ≥0.3 and a significantly positive correlation was found between 24-h proteinuria and P/C (r=0.382, p<0.001). Conclusion Our study demonstrated that a protein loss of ≥300 mg in 24-h and a P/C ratio in spot urine ≥0.3 in hypertensive pregnant women is associated with adverse perinatal outcomes. Furthermore, we have identified that proteinuria ≥300 mg/day and spot urine P/C ratio ≥0.3 in hypertensive pregnant women make them prone to early delivery risk.

The implications for advanced maternal age on pregnancy complications and adverse neonatal outcomes

Objective This study aimed to determine the effect of advanced maternal age (AMA) on maternal and neonatal outcomes in pregnant women aged ≥35 years compared with patients aged 30–34 years. Also, we aimed to analyze the risk estimates of potential confounders to identify whether these variables contributed to the development of adverse pregnancy outcomes or not. Methods This retrospective cohort study included 2284 pregnant women aged ≥35 years at the time of delivery who was delivered in a tertiary referral hospital from January 1, 2016, to December 31, 2020. We further classified these women into two subgroups: 35–39 years as early AMA (EAMA), and ≥40 years as very AMA (VAMA). Pregnancy complications and adverse neonatal outcomes were recorded. Results Compared to younger women, pregnant AMA women had significantly higher risks of complicated pregnancies, including a higher risk of gestational diabetes mellitus (GDM, p<0.001), polyhydramnios (p<0.001), cesarean section (p<0.001), stillbirths (p<0.001), major fetal abnormality (p<0.001), preterm delivery (p<0.001), lower birth weight (p<0.001), lower 5-minute Apgar scores (p<0.001), lower umbilical artery blood pH values (p<0.001), neonatal intensive care unit (NICU) admission (p<0.001), and length of NICU stay (p<0.001). Conclusion We found a strong and significant association between VAMA and adverse pregnancy outcomes, including an increased risk of GDM, polyhydramnios, cesarean section, and adverse neonatal outcomes, including a higher risk of stillbirths, preterm delivery, lower birth weight, lower 5-minute Apgar scores, and NICU admission.

Conditions that increase the risk of cesarean-related blood transfusions: a single-center cohort study

Objective This study aimed to define the conditions that increase the possibility of receiving a blood transfusion in patients who had a cesarean section. Methods This study was conducted between January 2016 – May 2020 in a university hospital located in Konya, Turkey. Pregnant women undergoing cesarean section were included. Of 4303 eligible patients, 188 women were the transfused group and 4115 women were the non-transfused group. Logistic regression analysis was performed for potential confounding factors. Results A total of 4303 eligible patients were evaluated in this study. There were 4115 patients (95.6%) in the non-transfused group. The transfused group consisted of 188 patients (4.4%). The probability of transfusion was higher in pregnant women with placenta previa, placenta accreta spectrum, thrombocytopenia, preoperative anemia, macrosomia above 4500 g, and multiple gestations [adjusted OR values (95% CI); 10.58 (range 4.75–23.57), 7.75 (range 3.22–18.61), 7.85 (range 3.46–17.79), 5.71 (range 4.21–7.74), 4.22 (range 1.21-14.67) and 2.10 (range 1.18-3.72), respectively]. There was no increase in the possibility of transfusion in 4000–4500 gram macrosomia, uterine fibroids, preeclampsia, premature rupture of membranes, previous cesarean sections and gestational diabetes mellitus. Conclusion Placenta previa, placenta accreta spectrum, thrombocytopenia, preoperative anemia, macrosomia above 4500 g and multiple gestations increase the possibility of transfusion. Perioperative blood preparation is vital in such patients. Prevention of anemia during pregnancy is critical in reducing transfusions.

Discordant umbilical arteries in the umbilical cord entanglement: a light microscope study

Objective The present study aimed to examine the histopathological diagnosis of the umbilical artery discordance in cases with single or multiple umbilical cord entanglement and pregnancy outcomes. Methods The vascular structure of the umbilical cord, histopathological findings of the placenta and obstetric outcomes were retrospectively examined in 50 cases. The cases were divided into two groups by the number of cord entanglement (single-multiple) and their histopathological findings and neonatal Apgar scores were assessed. Results Out of 50 cases, 38 (76%) had single and 12 (24%) had multiple cord entanglement. In 50 cases, the mean gestational age was 39.16±1.06 weeks, neonatal Apgar scores were 8.7±0.58 at 1 minute and 9.64±0.56 at 5 minute. No statistically significant difference was detected between single and multiple groups in terms of gestational age (p=0.79), 1-minute Apgar score (p=0.832) and 5-minute Apgar score (p=0.656). In histopathological examination, the diameters of umbilical arteries 1 and 2 were found to be 0.11±0.12, 0.09±0.05 µm, respectively in the single group (p=0.756) and 0.13±0.14, 0.06±0.02 µm, respectively in the multiple group (p=0.131). When the umbilical arterial diameters were compared by group, the diameter of the umbilical artery 2 was detected 0.09±0.05 µm in the single and 0.06±0.02 µm in the multiple group and statistically significant difference was detected (p=0.037). Out of 50 cases, placental hypoxia finding was detected as chorangiosis only in 10 cases (2 multiple, 8 single). Conclusion Umbilical artery discordance was detected in cases with multiple umbilical cord entanglement. However, poor pregnancy outcome was not observed in any of the cases. When multiple cord entanglement is seen during obstetric examination, umbilical artery discordance must be remembered and investigated, and also maternal-fetal condition should be considered.

Urinary uric acid to creatinine ratio as a marker of perinatal asphyxia and its correlation with arterial blood gas values

Objective Perinatal asphyxia is a leading cause of neonatal morbidity and mortality in developing countries. Lack of facilities like arterial blood gas analysis in resource limited settings warrants cost effective methods to support the diagnosis of asphyxia. The study objectives were to evaluate the utility of urinary uric acid to creatinine ratio (UA/Cr ratio) as a marker of perinatal asphyxia and to ascertain its correlation with cord blood arterial blood gas values. Methods It was a prospective comparative study where cases and controls were of asphyxiated neonates and normal neonates respectively delivered in a tertiary care medical college hospital from April 2019 to September 2019. Urinary UA/Cr ratio and its correlation with Apgar score was determined. The ability to predict asphyxia was estimated by ROC curve and p<0.05 was considered as statistically significant. Results Data from 38 asphyxiated and 38 normal neonates were analyzed. The mean urinary UA/Cr ratio was higher in the asphyxiated babies. There was negative correlation between urinary UA/Cr ratio and pH, pO2, Apgar scores and positive correlation with pCO2. The urinary UA/Cr ratio had excellent predictive validity for perinatal asphyxia determined by ROC curve. The urinary uric acid /creatinine ratio had sensitivity of 92.11% and specificity was 92.11%. Conclusion Urinary uric acid to creatinine ratio correlated well with the cord blood arterial blood gas values and the Apgar scores. This study showed that there is a significant increase in the urinary UA/Cr ratio in asphyxiated neonates and it can be used as a biochemical marker of perinatal asphyxia.

The relationship between umbilical cord measurements and newborn outcomes

Objective This study investigated the relationship between umbilical vein diameter and cord length and fetal outcome in low-risk pregnancies (fetuses appropriate for gestational age [AGA]). Methods A prospective cohort study of 39 singleton pregnant women aged 19–44 years at between 38+0 and 41+6 weeks of gestation was conducted. Case demographics, umbilical vein diameter measured by prenatal ultrasound, postnatal birth weight, gender, 1- and 5-minute Apgar scores, blood gas analysis, and umbilical cord length were recorded. Fetuses with a fetal weight in the 10–90th percentile according to week of gestation were accepted as AGA. Results The mean age of the pregnant women was 27.5±5.3 years. 33% (13/39) of the pregnant women were nulliparous. There was no statistically significant correlation between umbilical vein diameter and other variables in correlation analysis (p>0.050). Umbilical cord length and umbilical vein lactate level were found to have a statistically negative and significant correlation (r=-0.418; p=0.015); however, no other pregnancy outcomes were found to have a significant correlation. There was no statistically significant difference between the median values of umbilical vein diameter and cord length by gender (p=0.076 and 0.181, respectively). Conclusion In conclusion, this study found no relationship between umbilical vein diameter and cord length and fetal weight and pregnancy outcome in low-risk 38.0–41.6-week pregnancies (AGA fetuses). However, the obtained results still need to be confirmed by larger series.

Morphometric analysis of duodenum in human fetus

Objective The mucosa of the duodenum is lined by finger-like villi. In adults, the villus height to width ratio is about 4–5:1. The aim of this study is to evaluate the villus height to width ratio in the human fetus. Methods Eighty-one human fetal autopsies were included in the study. Three random villi were measured with the X and Y-axes. The statistical analysis was performed by Pearson correlation, Kolmogorov-Smirnov, chi-square, linear regression tests, and Student’s t-test. Results There were 29 female and 52 male fetuses. The age range was between 12 and 40 weeks of gestation. Of 81 fetuses, 2 were in the first trimester, 73 in the second trimester, and 6 in the third trimester. The mean villus height to width ratios in the first, second, and third trimesters were 2.75±0.37, 3.21±0.17, and 3.76±0.6, respectively. There was no correlation between the week of gestation and duodenum villus height to width ratio (p=0.080). The mean villus height to width ratios among females and males were 3.62±1.28 and 3.02±0.84, respectively (p=0.014). Conclusion In our study, villi were blunter and shorter in fetuses than in adults but not in children regardless of weeks of gestation compared to the literature. The gradual elongation of villi after birth may be attributed to environmental factors.

Prenatal and neonatal outcomes of pregnancies diagnosed with fetal single umbilical artery

Objective To investigate the associated anomalies and outcomes of fetuses diagnosed as having a single umbilical artery (SUA) which were reported inconsistently in previous studies. Methods The data of 82 pregnancies with fetal SUA, 35 of which were complex, and 47 isolated SUA (iSUA) and 100 pregnancies with fetal double umbilical arteries (DUA) between June 2018 and July 2020 were retrieved. We compared the maternal characteristics, and pregnancy and fetal outcomes of the three groups (iSUA, SUA, and DUA). Results Of 82 fetuses with SUA, 35 had 64 major structural abnormalities. 20 of these 35 fetuses (57.1%) had cardiovascular malformations, 12 (34.2%) had central nervous, 10 (28.5%) had genitourinary, and eight (22.8%) had gastrointestinal system malformations. Isolated SUA was present in SUA. Compared with the 100 DUA fetuses, SUA was a risk for intrauterine growth restriction (IUGR), preterm delivery, Apgar scores of <7, and admission to the neonatal intensive care unit. Having fetal chromosomal or structural abnormalities, was a risk for amnion fluid abnormality, pregnancy termination, intrauterine fetal death, early neonatal death, and a low live birth ratio in SUA cases. Conclusion SUA has an increased rate of fetal structural and chromosomal abnormalities. Among them, the most detected one is cardiac and the second most common one is central nervous system malformations. Pregnancies with fetal SUA have increased risk for IUGR, preterm delivery, low Apgar scores, and admission to the neonatal intensive care unit. The presence of additional structural or chromosomal malformations increases the rate of these adverse pregnancy risks. Thus, these cases warrant dedicated fetal ultrasonographic organ screening and close prenatal follow-up.