Kabuki syndrome (KS) is a rare multiple malformation disorder characterized by developmental delay, distinct facial anomalies, congenital heart defects, limb and skeletal anomalies, and short stature. Renal anomalies have been reported in a few cases of KS, but to our knowledge, hepatic anomalies have not. Here, we document two cases of KS requiring liver or kidney transplantation: one with severe hepatic and renal anomalies and one with severe renal anomalies. Both cases had the characteristic facial appearance of children with KS, postnatal growth deficiency, and developmental delay. At birth, case 1 presented with hypoglycemia, ileal perforation, right hydroureter, and hydronephrosis. The patient subsequently developed hyperbilirubinemia, hepatic abscess, and cholangitis. At age 8 months, he underwent a liver transplant. Hepatic pathology diagnosed neonatal sclerosing cholangitis. Case 2 presented with renal failure at age 6 years. Renal ultrasound study showed markedly dysplastic kidneys requiring transplantation. In addition to characteristic findings of KS, she had coronal synostosis and was shown to have immune deficiency and an autoimmune disorder manifesting as Hashimoto thyroiditis and vitiligo. We conclude: 1) severe hepatic and renal anomalies leading to organ failure can occur in KS; 2) patients with neonatal sclerosing cholangitis should be examined closely for features of KS; 3) coronal synostosis may occur in KS; and 4) immune deficiency and autoimmune disorder can be associated with KS.

Nipah virus (NiV) outbreak occurred in Kozhikode district, Kerala, India in 2018 with a case fatality rate of 91% (21/23). In 2019, a single case with full recovery occurred in Ernakulam district. We described the response and control measures by the Indian Council of Medical Research and Kerala State Government for the 2019 NiV outbreak. The establishment of Point of Care assays and monoclonal antibodies administration facility for early diagnosis, response and treatment, intensified contact tracing activities, bio-risk management and hospital infection control training of healthcare workers contributed to effective control and containment of NiV outbreak in Ernakulam.

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Donor deferral results in loss of potential, motivated blood donors, and thereby, availability of blood for needy patients. This study analyses the frequency and reasons for donor deferral, including high hemoglobin deferral, which is underreported in India. Methods: Retrospective analysis of the deferral record of whole blood donors from January 2014 to December 2018 was performed with respect to the pre-donation screening process at our center. Accordingly, the deferrals are categorized as stage1-evaluation of Donor History Questionnaire (DHQ), stage 2-medical examination, stage 3-hemoglobin (Hb) check using copper sulfate method or a gradually implemented quantitative hemoglobin analyzer, and stage 4-before phlebotomy. Donor demographic details, donation frequency, and deferral reasons were noted. Descriptive statistical analysis was performed using SPSS version 20 (IBM, USA). Results: Of 99,680 pre-donation screenings, 10.6% was deferred. The highest deferral (56.02%) was at stage 3 (based on hemoglobin level) contributed by low (52.45%) and high (3.75%) hemoglobin deferrals against cutoff of 12.5 to 18 g/dl. High Hb was noted only in male donors when screened by hemoglobin analyzer. Further, a steady fall in low hemoglobin but a rise in high hemoglobin deferral rate owing to the gradual implementation of hemoglobin analyzer over the study period was noted. The deferrals in stage 1, 2, and 4 were 29.64%, 13.97%, and 0.36%, respectively. Overall, the deferral rate was higher in females (49.88%), and in first-time (13.63%), and 18 to 25 yrs age-group (4.25%) donors for low Hb, underweight, and tattooing/ear piercing. Conclusion: Insights on donor deferral reasons promote proactive measures towards donor recruitment and retention. Further, donor hemoglobin screening by quantitative method, if followed uniformly by all blood centers across the country, will help identify the true prevalence of high hemoglobin in Indian blood donors and ensures donor safety.