Transcranial Doppler Velocities among Sickle Cell Disease Patients in Steady State.

Transcranial Doppler (TCD) screening is an established tool to identify children with sickle cell disease at high risk of stroke. Our objective was to study TCD velocities among sickle cell disease patients while in a steady state. This cross-sectional study included 78 steady state sickle cell disease patients [31 Hb SS (ßS/ßS) (sickle cell anemia), 47 Hb S/ß-thalassemia (HBB: c.20A>T/ß-thal)], attending the Pediatric Hematology Clinic at Cairo University Children's Hospital, Cairo, Egypt. All patients underwent TCD velocity assessment as per the Stroke Prevention Trial in Sickle Cell Anemia (STOP) protocol. In our cohort, TCD velocities were comparable among Hb S/ß-thal vs. SS patients. Hemolysis indicators correlated significantly to TCD velocities in Hb S/ß-thal patients; positive correlation was found between total bilirubin level and right middle cerebral artery (MCA) and right distal internal carotid artery (dICA) TCD velocities (r = 0.428, p = 0.00, r = 0.360, p = 0.01), respectively as well as between reticulocyte count and right MCA, right dICA and right anterior cerebral artery (ACA) TCD velocities (r = 0.424, p = 0.01), (r = 0.40, p = 0.00), (r = 0.303, p = 0.04), respectively. On the other hand, statistically significant negative correlations were found between hemoglobin (Hb) level and right ACA, right dICA TCD velocities (r = -0.290, p = 0.05), (r = -0.324, p = 0.03). Although Hb F is considered an ameliorating factor for disease severity; hemolysis stands as an indicator of risk for TCD velocity elevation, and in turn, risk for stroke among sickle cell disease patients.

Modified technique for thermal radiofrequency ablation of Thoracic dorsal root ganglia under combined fluoroscopy and CT guidance: a randomized clinical trial.

BACKGROUND: This study is comparing thermal radiofrequency ablation (TRFA) of the thoracic dorsal root ganglia (TDRG) guided by Xper CT and fluoroscopy with the standard fluoroscopy. METHODS: This randomized clinical trial included 78 patients suffering from chronic refractory pain due to chest malignancies randomly allocated into one of two groups according to guidance of TRFA of TDRG. In CT guided group (n = 40) TRFA was done under integrated Xper CT-scan and fluoroscopy guidance, while it was done under fluoroscopy guidance only in standard group (n = 38). The primary outcome was pain intensity measured by visual analog scale (VAS) score, functional improvement and consumption of analgesics. The secondary outcome measures were patient global impression of changes (PGIC) and adverse effects. RESULTS: VAS scores decreased in the two groups compared to baseline values (p < 0.001) and were lower in CT guided group up to 12 weeks. Pregabalin and oxycodone consumption was higher in the standard group at 1, 4 and 12 weeks (p < 0.001). Functional improvement showed near significant difference between the two groups (P = 0.06 at week 1, 0.07 at week 4 respectively) while the difference was statistically significant at week 12 (P = 0.04). PGIC showed near significant difference only at week 1 (P = 0.07) while the per-patient adverse events were lower in CT guided group (p = 0.027). CONCLUSIONS: Integrated modality guidance with Xper CT-scan and fluoroscopy together with suprapedicular inferior transforaminal approach may improve efficacy and safety of TRFA of TDRG for the treatment of intractable chest pain in cancer patients. TRIAL REGISTRATION: The study was retrospectively registered at clinicaltrials.gov on 04/22/2018 (Registration No.: NCT03533413).

Role of transcranial Doppler in assessment of cerebral blood flow in full term neonates with extreme unconjugated hyperbilirubinemia.

PURPOSE: To evaluate the difference in cerebral blood flow in neonates with and without extreme unconjugated hyperbilirubinemia. METHODS: Transcranial Doppler parameters of 26 full term newborns with extreme unconjugated hyperbilirubinemia (UCH) were compared to 13 postnatal age and sex matched normal healthy neonates serving as controls. Resistance index (RI), pulsatility index (PI) and peak systolic velocity (PSV) were measured in the middle cerebral, internal carotid and posterior cerebral arteries on both sides by transcranial color Doppler ultrasound. RESULTS: An increase in cerebral blood flow (decreased RI, PI and increased PSV) was observed in the extreme unconjugated hyperbilirubinemia (UCH) group. There was positive correlation between total serum bilirubin level and peak systolic velocity and vice versa with resistivity and pulsatility indices. Eight neonates developed clinical features of acute bilirubin encephalopathy and showed significantly increased peak systolic velocity in the right middle cerebral artery compared to those with normal outcome. Resistivity index and pulsatility index were lower in patients managed by exchange transfusion compared to those managed with phototherapy. CONCLUSION: An increase in cerebral blood flow was observed in neonates with UCH compared to those without hyperbilirubinemia. By assessing the cerebral blood flow velocity, resistivity index (RI), and pulsatility index (PI) of particular intracranial arteries, the transcranial Doppler can identify the at-risk neonates, for development of neurological affliction in extreme unconjugated hyperbilirubinemia.

Prediction of extubation readiness using lung ultrasound in preterm infants.

We aimed to test the hypothesis that a lung ultrasound severity score (LUS) and assessment of left ventricular eccentricity index of the interventricular septum (LVEI) by focused heart ultrasound can predict extubation success in mechanically ventilated infants. We conducted a prospective study on premature infants less than 34 weeks' of gestation. LUS was performed on postnatal Days 3 and 7 by an investigator who was masked to infants' ventilator parameters. LVEI and pulmonary artery pressure (PAP) were measured at postnatal Day 3. A receiver operator curve was constructed to assess the ability to predict extubation success. Spearman correlation was performed between LVEI and PAP. A total of 104 studies were performed to 66 infants; of them 39 had mild and 65 had moderate-severe lung disease. LUS predicted extubation success with a sensitivity and a specificity of 91% and 69%, respectively. Area under the curve was 0.83 (CI: 0.75-0.91). LVEI did not differ between infants that succeeded and failed extubation. It correlated with PAP during systole (r = .66). We conclude that LUS predicts extubation success in mechanically ventilated preterm infants whereas LVEI correlates with high PAP.

ßS globin gene haplotype and the stroke risk among Egyptian children with sickle cell disease.

BACKGROUND AND AIM OF WORK: Sickle cell disease (SCD) is an inherited disease of the beta globin gene. The ßS globin gene haplotypes are Senegal, Benin, Bantu, Cameroon, Arab-Indian and atypical haplotypes. In SCD, stroke is a life-threatening event in both adults and children. In light of paucity of studies on ßS globin gene haplotypes in Egypt, we aimed to determine ßS globin gene haplotypes in children with SCD and study their impact on stroke risk. METHODS: Fifty-two SCD patients were included in the study, they were 26 males and 26 females with age range from 3 to 18 years old. The PCR-RFLP technique was used for the determination of ßS globin gene haplotypes. Transcranial Doppler (TCD) was done to identify patients at risk of stroke. RESULTS: Benin/Benin was the most prevalent haplotype detected in 50% followed by Benin/Bantu in 30.8% of studied patients. TCD study showed that 14/52 (26.9%) patients had abnormally high TCD flow velocities (TCD velocities ≥170 cm/s) and thus considered high stroke risk group, whereas 38/52 (73.1%) patients had TCD flow velocities <170 cm/s and are considered low stroke risk group. Stroke risk was not found to be associated with ßS globin gene haplotype (p = .532). CONCLUSION: This study provides a relevant contribution to our understanding of the anthropological and historical background of the population in Egypt where Benin haplotype is the commonest ßS globin gene haplotype and homozygous Benin/Benin is associated with higher stroke risk than other haplotypes.

Doppler ultrasound assessment of the splanchnic circulation in preterms with neonatal sepsis at risk for necrotizing enterocolitis.

OBJECTIVE: To evaluate the role of Doppler ultrasonography in the assessment of splanchnic circulation's hemodynamic changes in septic preterms at risk of necrotizing enterocolitis. METHODS: A total of 51 septic preterms were divided into two groups: 25 preterms with clinical signs of necrotizing enterocolitis (NEC) and 26 preterms with no clinical signs of NEC. Both groups were assessed with Doppler ultrasonography of the celiac and superior mesenteric arteries, and each septic preterm's peak systolic velocity (PSV), end-diastolic velocity (EDV), resistivity index (RI), and pulsatility index (PI) was calculated and recorded. RESULTS: These included a statistically significant lower PSV (p: 0.001) and a lower EDV (p: 0.001) in the superior mesenteric artery in the septic group with clinical signs of NEC in comparison with the septic group with no clinical signs of NEC. A statistically significant (p < 0.001) higher PSV celiac (CA)/PSV superior mesenteric (SMA) ratio was found for the group of septic preterms with clinical signs of NEC when compared to the other group. CONCLUSION: The study results showed that Doppler ultrasonography of the splanchnic circulation can be a tool for the early identification of NEC cases among septic preterms.

Plasma chitotriosidase and carotid intima-media thickness in children with sickle cell disease.

The relationship between chronic hemolysis with subsequent iron overload, inflammation, and premature atherosclerosis has been documented in hemolytic anemias, particularly ß-thalassemia. However, no such relationship has been established in sickle cell disease (SCD). We sought to evaluate SCD as a risk factor for early vascular insult by measuring carotid intima-media thickness (CIMT) and plasma chitotriosidase and to assess the role of the latter as a potential quantitative indicator of vascular inflammation and atherogenesis. Thirty SCD pediatric patients (3-18 years) and 30 matched controls were enrolled. Full clinical history, with hematological and biochemical parameters, was evaluated. CIMT and chitotriosidase activity were also assessed for all study participants. CIMT values were significantly higher in SCD patients (median 0.42; range 0.32-0.6 mm) compared to controls (0.36; 0.34-0.45 mm), P = 0.03. CIMT correlated positively with age (r = 0.460, P = 0.011), and total number of vascular incidents necessitating hospital admission (r = 0.439, P = 0.015). Similarly, chitotriosidase activity was significantly higher among SCD patients (median 59.6; range 7.3-512 nmol/ml plasma/h) compared to controls (32.7; 6.8-63.1 nmol/ml plasma/h), P < 0.001, and showed a positive correlation with serum ferritin (r = 0.517, P = 0.003) and CIMT (r = 0.535, P = 0.002). SCD children are at risk of developing premature atherogenic changes. Plasma chitotriosidase and CIMT may represent useful predictors of these changes.