Two‐Piece Hollow Bulb Obturator for Postsurgical Partial Maxillectomy Defect in a Young Patient Revamping Lost Malar Prominence: A Clinical Report

The most frequent type of treatment for patients diagnosed with a malignant neoplasia of the oral cavity is surgical resection of the tumor. Ablative surgery may be followed by a reconstructive phase, in which the surgeon may choose between local flaps, nonvascularized bone grafts or free vascularized flaps to close the surgical site, depending on the general conditions of the patient. Esthetic and functional results are challenging to achieve for the prosthodontist, as variable amount of hard and soft tissues are removed. This report describes the fabrication of a two‐piece hollow obturator for a 19‐year‐old patient who underwent wide surgical excision of the osteosarcoma of the maxilla and was rehabilitated to function. In this case, the surgical site was covered with submental flap, and the second piece of the obturator provided fullness to the lost malar prominence.     

Utility of Sonoclot analysis and tranexamic acid in tetralogy of Fallot patients undergoing intracardiac repair

Sonoclot analysis is a point of care test to monitor the coagulation process, presenting a comprehensive evaluation of the clot formation and retraction as well as platelet function. This randomized double-blinded study was designed to investigate the utility of Sonoclot analysis in monitoring the coagulation profile as also the antifibrinolytic effects of tranexamic acid administered in patients with tetralogy of Fallot undergoing intracardiac repair. Eighty of a total 94 patients were randomly divided into two groups of 40 each. In the study group, TA was administered thrice at a dosage of 10 mg/kg, i.e. before CPB, on CPB and after CPB, whereas in the control group, placebo was administered at the same time intervals. Sonoclot analysis and D-dimer measurement were performed at baseline and following heparin neutralisation. An additional variable, DR?? (diminishing rate of clot strength at 15 min postmaximal clot strength), was calculated from the Sonoclot graph and was compared with d-dimer levels as a measure of fibrinolysis. The three Sonoclot variables, i.e. activated clotting time, clot rate and platelet function, were deranged at baseline in all the patients. Post-CPB, the change in these variables was not significant. ACT, clot rate and platelet function showed no significant (P > 0.05) difference in both the groups at both the time intervals. DR?? and d-dimer values were comparable at baseline in both the groups. However, a significant (P < 0.05) difference was seen in these variables in the control group as compared with the TA group following heparin neutralisation. To conclude, Sonoclot analysis is a useful, point of care method for the monitoring of coagulation and fibrinolysis in patients with tetralogy of Fallot undergoing intracardiac repair.     

Retrospective Study of Carfilzomib-Pomalidomide-Dexamethasone in Relapsed/Refractory Multiple Myeloma Patients in a Tertiary Care Hospital in India

Carfilzomib is a second-in class Proteosome Inhibitor and has been approved for Relapsed/Refractory Multiple Myeloma (RRMM). We retrospectively retrieved and analyzed data of KPd combination both biweekly and weekly regimens at our centre from 1 st August 2017 and 31 st May 2020. Sixty-nine patients were treated with KPd with median age of 58 years. Median prior lines of chemotherapy were 2(1-15). Twenty-eight (40.5%) patients underwent autoSCT. Median no. of cycles was 4(1-12) and 3(1-13) with median time to response of 4(2-12) and 2(2-6) months in biweekly and once weekly regimen cohorts respectively. At last follow-up, overall response rate (ORR) was 65.2%{CR-n = 10 (14.5%), VGPR-n = 19 (27.5%), PR-n = 16 (23.2%)} with n = 13(18.8%) patients had PD and relapse was observed in n = 24(34.8%). Thirty (43.4%) patients received maintenance therapy {n = 21(70%)} or autoSCT {n = 9(30%)}. Common toxicities were anemia {n = 11(15.9 %)}, thrombocytopenia (n = 15(21.7%) and neutropenia (n = 16 (23.2%)}, hypertension {n = 28(40.5%)}, peripheral neuropathy (grade1/2) {n = 15(21.7%)}, infections [n = 18(26%) {bacterial [n = 9(13%),viral n = 7(10.1%), fungal n = 8(11.6%)}]. At a median follow-up of 18 months, the estimated median PFS was 11.3 months (95%C.I. 8.3– 14.2) whereas the estimated median OS was 28 months (95%C.I. 20.4-35.5) for the entire cohort. Mortality rate of 2.5% and 10% in two cohorts respectively. Commonest cause of death was PD and sepsis. KPD is a well-tolerated regimen for RRMM, which can be a bridge to ASCT, however with significant side effects.     

Devastating salt‐wasting crisis in a four‐month‐old male child with congenital adrenal hyperplasia,highlighting the essence of neonatal screening

Congenital adrenal hyperplasia (CAH) is a rare condition usually referred to as a group of genetic disorders resulting due to a deficiency of steroid enzymes required by adrenal glands to produce cortisol and mineralocorticoid hormones. It has an autosomal recessive mode of inheritance and is further categorized into two types—Classic and Non‐Classic. Non‐Classic CAH is a more common milder form that presents late after puberty. Classic CAH, although more severe, is rare and detected at birth and is associated with the life‐threatening adrenal crisis in both sexes and virilization of the external genitalia in females (46, XX) patients, whereas in males, no overt abnormality of the external genitalia is present. We present a case of a four‐month‐old male child with the classic form of CAH who was brought with complaints of loose stools, projectile non bilious vomiting, decreased urine output, and failure to feed for 3 days. The child had a clinical presentation of salt wasting with hypoglycemia and hyperpigmentation of his genitalia. The USG findings revealed increased anteroposterior diameter of renal pelvis indicative of a growth in the suprarenal area. 17‐hydroxyprogesterone (17‐OHP) was found to be elevated confirming the diagnosis. He was treated with hydrocortisone with gradual improvement in his glucose and electrolytes. The patient was discharged home on replacement therapy consisting of oral prednisolone and fludrocortisone acetate and followed up as outpatient with significant improvement in the clinical findings. The fact that the child was not screened for CAH at birth led to the critical consequences of the disease in this case. To prevent life‐threatening adrenal crisis and help perform appropriate sex assignments for affected female patients, newborn screening (NBS) programs for the classical form of CAH should be made mandatory even in low‐ and middle‐income countries.     

Busulfan/melphalan/antithymocyte globulin followed by unrelated donor cord blood transplantation for treatment of infant leukemia and leukemia in young children: the Cord Blood Transplantation study (COBLT) experience.

A non-total body irradiation-containing preparative regimen was studied in young children (<4 years old) undergoing unrelated donor cord blood transplantation as part of the Cord Blood Transplantation trial for the treatment of acute lymphoblastic leukemia (n = 14), acute myeloid leukemia (n = 13), undifferentiated leukemia (n = 1), juvenile myelomonocytic leukemia (n = 2), and myelodysplastic syndromes (n = 2). Donor/recipient HLA matching based on low-/intermediate-resolution molecular typing for HLA-A and -B and high-resolution HLA-DRB1 typing was 5/6 or 6/6 (n = 21) or 4/6 (n = 11). The preparative therapy consisted of busulfan, melphalan, and antithymocyte globulin, with cyclosporine and corticosteroids for graft-versus-host disease (GVHD) prophylaxis. The median age was 1.6 years (range, 0.5-3.9 years), and the median weight was 10.5 kg (range, 5.8-19.5 kg). Cord blood grafts contained a median of 10.7 x 10 7 nucleated cells per kilogram (range, 4.6-29.2) and 2.6 x 10(5) CD34+ cells per kilogram (range, 0.7-8.3). The cumulative incidence (CINC) of neutrophil recovery (absolute neutrophil count >500/microL) at day 42 was 0.59 (95% confidence interval [CI], 0.44-0.78) at a median of 31 days (range, 23-55 days). The CINC and Kaplan-Meier estimates of platelet engraftment at day 180 were 0.53 (95% CI, 0.34-0.69) and 0.82 (95% CI, 0.61-1.00), respectively. CINC estimates of grade III/IV acute GVHD at day 100 and chronic GVHD at 1 year were 0.25 (95% CI, 0.09-0.41) and 0.26 (95% CI, 0.09-0.44), respectively. The CINC estimate of relapse was 0.31 (95% CI, 0.16-0.47) at 2 years. With a median follow-up of 27.8 months (range, 23.4-46.7 months), the probability of survival at 1 year was 0.47 (95% CI, 0.30-0.64). A preparative regimen containing a busulfan/melphalan/antithymocyte globulin preparative regimen is well tolerated in the setting of unrelated donor cord blood transplantation for childhood leukemia and can serve as a platform preparative regimen for intensifying host immunosuppression and antileukemic therapy to allow for improved engraftment and improved relapse-free survival.     

Par6alpha signaling controls glial-guided neuronal migration

Neuronal migrations along glial fibers provide a primary pathway for the formation of cortical laminae. To examine the mechanisms underlying glial-guided migration, we analyzed the dynamics of cytoskeletal and signaling components in living neurons. Migration involves the coordinated two-stroke movement of a perinuclear tubulin 'cage' and the centrosome, with the centrosome moving forward before nuclear translocation. Overexpression of mPar6alpha disrupts the perinuclear tubulin cage, retargets PKCzeta and gamma-tubulin away from the centrosome, and inhibits centrosomal motion and neuronal migration. Thus, we propose that during neuronal migration the centrosome acts to coordinate cytoskeletal dynamics in response to mPar6alpha-mediated signaling.     

Alkali-free bioactive glasses for bone tissue engineering: a preliminary investigation

An alkali-free series of bioactive glasses has been designed and developed in the glass system CaO-MgO-SiO₂-P₂O₅-CaF₂ along the diopside (CaMgSi₂O₆)-fluorapatite (Ca₅(PO₄)₃F)-tricalcium phosphate (3CaO·P₂O₅) join. The silicate network in all the investigated glasses is predominantly coordinated in Q² (Si) units, while phosphorus tends to remain in an orthophosphate (Q⁰) environment. The in vitro bioactivity analysis of glasses has been made by immersion of glass powders in simulated body fluid (SBF) while chemical degradation has been studied in Tris-HCl in accordance with ISO-10993-14. Some of the investigated glasses exhibit hydroxyapatite formation on their surface within 1-12 h of their immersion in SBF solution. The sintering and crystallization kinetics of glasses has been investigated by differential thermal analysis and hot-stage microscopy, respectively while the crystalline phase evolution in resultant glass-ceramics has been studied in the temperature range of 800-900 °C using powder X-ray diffraction and scanning electron microscopy. The alkaline phosphatase activity and osteogenic differentiation for glasses have been studied in vitro on sintered glass powder compacts using rat bone marrow mesenchymal stem cells. The as-designed glasses are ideal candidates for their potential applications in bone tissue engineering in the form of bioactive glasses as well as glass/glass-ceramic scaffolds.