Kidney transplantation in low- and middle-income countries: the Transplant Links experience.

Paediatric kidney failure is a global problem responsible for significant childhood morbidity and mortality. The gold-standard treatment is kidney transplantation. However, the availability of kidney transplantation remains limited in some low- and middle-income countries (LMICs). Transplant Links Community (TLC) is a UK-based charity that mentors units in LMICs wishing to start kidney transplantation; the ultimate goal is for these units to become self-sufficient. TLC provides this support through in-person training visits and skill transfer, plus direct mentorship from the UK that is maintained over many years. From such mentoring programmes, it is evident that there are numerous challenges in the initial establishment and long-term maintenance of kidney transplant services, with specific and unique barriers applying to setting up paediatric transplant programmes compared to their adult counterparts. This review summarises TLC's first-hand experience of developing paediatric kidney transplantation services in LMICs over the past 15 years, the challenges encountered, and the major ongoing barriers that must be addressed to facilitate further progress in delivering transplantation services to children globally.

Reference Range of Plasma Fatty Acids in North Indian Pregnant Population.

Reference values for Fatty Acids (FAs) are not well defined in the Indian population. Therefore, it is critical to establish FAs reference range for the healthy non-pregnant and pregnant Indian population. The present multi-centric, and cross-sectional study determines the 95% reference interval for FAs in an apparently pregnant Indian population and compare it to the healthy non-pregnant women. Physicians identified 164 reference individuals as healthy (56 non-pregnant and 108 pregnant) at various government and private hospitals of northern India. The 95th and 97.5th percentile reference limits were used to estimate the 95 percentile of the reference distribution. The reference ranges observed for Alpha-linolenic acid (0.29-0.42%; 0.36-0.58%), Docosahexaenoic-acid (3.38-4.23%; 3.8-4.55%), Eicosapentaenoic-acid (1.24-1.76%; 1.09-1.62%), Docosapentaenoic-acid-3 (0.61-0.69%; 0.65-0.76%), Linoleic-acid (18.44-20.75%; 19.51-21.88%), gamma-linolenic-acid (0.24-0.35%; 0.32-0.42%), Eicosatrienoic-acid (0.26-0.32%; 0.34-0.39%), Arachidonic-acid (9.29-11.02%; 10.02-11.56%), Docosatetraenoic-acid (0.62-0.89%; 0.79-1.09%), Docosapentaenoic-acid-6 (0.23-0.31%; 0.33-0.41%), Eicosatrienoic-acid (1.17-1.41%; 1.43-1.74%), Eicosenoic-acid (0.28-0.38%; 0.37-0.49%), Nervonic-acid (1.39-1.69%; 1.41-1.74%), Palmitoleic-acid (1.17-1.58%; 2-2.66%), Oleic-acid (19.8-22.26%; 19.68-22.94%), Myristic-acid (1.16-1.68%; 0.82-1.3%), Palmitic-acid (20.05-21.8%; 20.7-22.43%), Stearic-acid (11.34-12.56%; 10.29-11.02%), Arachidic-acid (0.17-0.2%; 0.18-0.23%), Lignoceric-acid (0.81-1.08%; 0.77-1.08%), trans-palmitoleic-acid (0.22-0.29%; 0.26-0.37%), trans-oleic-acid (0.55-0.72%; 0.68-0.84%), trans-linoleic-acid (0.38-0.54%; 0.42-0.59%) respectively for non-pregnant and pregnant women. Furthermore, total FAs were significantly (p ≤ 0:05) higher in women aged 31-45 years than in women aged 16-30 years. whereas, there was no significant change in total FAs profile based on omega-supplementation, diet category, preterm-birth history, and gestation period. Thus, the current study provides information about an individual who is deficient in FAs and the dose required to increase FA concentrations in the body.

Comparison of Maternal and Neonatal Outcomes among COVID-Positive Pregnant Women Affected in Second and Third Waves in a Dedicated COVID Hospital in Amritsar.

Background: With the emergence of new mutated variants of severe acute respiratory syndrome coronavirus 2 (SARS-COV-2), we have witnessed three waves of coronavirus disease (COVID) with varying severity, complication, and outcome in Punjab. The physiological changes of pregnancy make mother more vulnerable for severe infection. Current study is aimed at comparison of maternal and neonatal outcomes of COVID-positive pregnant women in second and third waves. Materials and Methods: This was a retrospective observational single-center study conducted at a dedicated COVID hospital in Punjab, India. Records of all COVID-positive pregnant women admitted from January to June 2021 and from January to February 2022 were reviewed. The demographic details, severity of symptoms, maternal and fetal complications, outcomes, and mortality were noted. Results: There were 220 COVID-positive pregnant patients in the second wave and 65 in third wave. The majority of patients belonged to the age group of 20-34 years (57.73% in the second wave and 70.77% in the third wave). Maternal deaths and severity of disease increased with increasing age of the mother, that is, 6.7% in <35 years and 13.95% in >35 years age group (RR = 2.058, P value = 0.1248). Also, the maternal deaths increased with increasing parity (RR = 2.00, P value = 0.2380). The majority of the study subjects were in the third trimester, with 77.73% in the second wave and 90.77% in the third wave. The majority of the patients in both the waves were asymptomatic or had mild symptoms. In the COVID second wave, 10.91% pregnant patients had moderate COVID symptoms and 8.18% had severe COVID symptoms, whereas none presented with moderate or severe symptoms in the third wave. Eighteen maternal deaths (8.18%) were seen during the COVID second wave, whereas no maternal death occurred in the third COVID wave. 100% of these deaths were because of COVID. Need of oxygen supplementation and intensive care unit admission had statistically significant association with maternal mortality. Conclusion: In the third COVID wave, the morbidity and mortality were significantly reduced. This could be a result of wide-spread vaccination, new strain of COVID, or both. In spite of this, the pregnancy complications such as pre-term birth, IUGR, and IUD were significant. Hence, pregnancies complicated by COVID should be considered as a high risk and closely monitored.

Quality of life in Hidradenitis Suppurativa (Acne Inversa): A scoping review.

Background: Hidradenitis Suppurativa (HS) is a chronic, relapsing, inflammatory skin condition which is physically, psychologically and socially disabling and often affects a patient's quality of life (QOL). There are numerous QOL tools used in dermatology. However, assessment of QOL in patients with HS is difficult due to the inability of generic QOL tools to specifically capture QOL in patients with HS. Numerous HS-specific QOL tools have been developed in recent years. It is important to identify evidence on full psychometric evaluation of these tools. Objectives: There has been a gradual increase in the use of generic and disease-specific QOL tools in the last few decades. The aim of this scoping review (SR) is to evaluate the most widely used generic QOL tools and HS-specific QOL tools to identify the psychometric evaluation of such tools. Methods: Design: An SR guided by Joanna Briggs Institute manual and Arskey O'Malley framework guidelines. Data extraction included the studies available on full psychometric evaluation of the most widely used dermatology generic QOL tools in HS and HS-specific QOL tools. Results: Ten papers were included in the review, eight papers demonstrated HS-specific QOL assessment tools. The psychometric properties of these tools were underpinned by reliability, validity and sensitivity measurement. Six disease-specific tools were identified in this SR. However, they all lack full psychometric evaluation. Conclusion: This review indicates that an extensive research in the field of QOL tools for HS is much needed. It is crucial to develop user-friendly and validate disease-specific tools to measure the real impact of disease on patients QOL. QOL instruments can evaluate the impact on life of an HS patient, thus helping improve intervention and management of disease. There is a necessity for more research into existing HS-specific QOL tools and they should be widely tested and fully validated.

Pediatric peritoneal dialysis training program and its relationship to peritonitis: a study of the International Pediatric Peritoneal Dialysis Network.

BACKGROUND: The guidelines for training of patients and caregivers to perform home peritoneal dialysis (PD) uniformly include recommendations pertaining to the prevention of peritonitis. The objective of this study conducted by the International Pediatric Peritoneal Dialysis Network (IPPN) was to investigate the training practices for pediatric PD and to evaluate the impact of these practices on the peritonitis and exit-site infection (ESI) rate. METHODS: A questionnaire regarding details of the PD program and training practices was distributed to IPPN member centers, while peritonitis and ESI rates were either derived from the IPPN registry or obtained directly from the centers. Poisson univariate and multivariate regression was used to determine the training-related peritonitis and ESI risk factors. RESULTS: Sixty-two of 137 centers responded. Information on peritonitis and ESI rates were available from fifty centers. Training was conducted by a PD nurse in 93.5% of centers, most commonly (50%) as an in-hospital program. The median total training time was 24 hours, with a formal assessment conducted in 88.7% and skills demonstration in 71% of centers. Home visits were performed by 58% of centers. Shorter (< 20 hours) training duration and lower number of training tools (both p < 0.02) were associated with higher peritonitis rate, after adjustment for proportion of treated infants and income of country of residence. CONCLUSIONS: An association between training duration and the number of training tools represent potentially modifiable risk factors to reduce peritonitis rates within the pediatric PD population. A higher resolution version of the Graphical abstract is available as Supplementary information.

Continuous Erector Spinae Plane Block in Paediatric VATS: A Case Series.

Decortication and stripping of infected pleura by video-assisted thoracoscopic surgery or thoracotomy is the treatment of choice in cases of empyema. The stripping is associated with intense post-operative pain. Erector spinae block is an excellent and safe alternative to thoracic epidural block. The experience in paediatric erector spinae plane block is very limited. We present our experience of continuous erector spinae block and one single-shot erector spinae plane block in paediatric video-assisted thoracoscopic surgeries. We had 5 patients aged 2-8 years with right-sided empyema, who were taken up for video-assisted thoracoscopic surgery decortication, and 2 patients aged 1-4 years with congenital diaphragmatic hernia (CDH) for video-assisted thoracoscopic surgery CDH repair. After induction and intubation, using high-frequency straight ultrasound probe, an erector spinae plane catheter was inserted and the local anaesthetic agent was administered. The patients were monitored for signs of effective analgesia. Post-extubation continuous erector spinae plane block was continued for 48 hours using bupivacaine and fentanyl. All patients had excellent postoperative analgesia for more than 48 hours. There were no side effects like motor block, nausea, vomiting, or respiratory depression. Continuous erector spinae plane block provides excellent analgesia in paediatric patients undergoing video-assisted thoracoscopic surgery, causing minimal side effects. Further, a prospective randomized control trial is suggested to establish the efficacy of this block in paediatric video-assisted thoracoscopic surgeries.

Bone Mineral Density and Vascular Calcification in Children and Young Adults With CKD 4 to 5 or on Dialysis.

Introduction: Older adults with chronic kidney disease (CKD) can have low bone mineral density (BMD) with concurrent vascular calcification. Mineral accrual by the growing skeleton may protect young people with CKD from extraosseous calcification. Our hypothesis was that children and young adults with increasing BMD do not develop vascular calcification. Methods: This was a multicenter longitudinal study in children and young people (5-30 years) with CKD stages 4 to 5 or on dialysis. BMD was assessed by tibial peripheral quantitative computed tomography (pQCT) and lumbar spine dual-energy X-ray absorptiometry (DXA). The following cardiovascular imaging tests were undertaken: cardiac computed tomography for coronary artery calcification (CAC), ultrasound for carotid intima media thickness z-score (cIMTz), pulse wave velocity z-score (PWVz), and carotid distensibility for arterial stiffness. All measures are presented as age-adjusted and sex-adjusted z-scores. Results: One hundred participants (median age 13.82 years) were assessed at baseline and 57 followed up after a median of 1.45 years. Trabecular BMD z-score (TrabBMDz) decreased (P = 0.01), and there was a nonsignificant decrease in cortical BMD z-score (CortBMDz) (P = 0.09). Median cIMTz and PWVz showed nonsignificant increase (P = 0.23 and P = 0.19, respectively). The annualized increase in TrabBMDz (ΔTrabBMDz) was an independent predictor of cIMTz increase (R 2 = 0.48, ß = 0.40, P = 0.03). Young people who demonstrated statural growth (n = 33) had lower ΔTrabBMDz and also attenuated vascular changes compared with those with static growth (n = 24). Conclusion: This hypothesis-generating study suggests that children and young adults with CKD or on dialysis may develop vascular calcification even as their BMD increases. A presumed buffering capacity of the growing skeleton may offer some protection against extraosseous calcification.

Enzyme-/metal-free quinoxaline assemblies: direct light-up detection of cholesterol in human serum.

Supramolecular assemblies of quinoxaline derivative (QxPyA) have been developed for turn-on detection of cholesterol in human serum and in aqueous media with a detection limit of 4 nM.

Development and standardization of processing technique for ready-to-use lab fermented Kanji mix using refractance window dried black carrot powder.

Black carrots are rich in bio-actives but underutilized owing to their short-term availability and perishable nature. Traditionally, black carrots have been used for the preparation of Kanji-a fermented non-dairy beverage prepared using natural fermentation by lactic acid bacteria and a few spices. This plant-based probiotic beverage has high antioxidant properties but there is a risk of contamination with pathogens due to uncontrolled fermentation during storage. To enhance the availability of this nutritious beverage throughout the year and to ensure the microbiological safety of the traditional fermented product, the present study was planned to optimize the process for controlled fermentation using freeze-dried lactic acid bacterial (LAB) culture and refractance window-dried black carrot powder. The physicochemical and microbiological profiles of LAB-fermented Kanji were analysed. The dried Kanji mix can be reconstituted into naturally fermented probiotic beverage with unique flavour and aroma along with ensured microbiological safety and enhanced commercial value.

The burden of subclinical cardiovascular disease in children and young adults with chronic kidney disease and on dialysis.

BACKGROUND: Cardiovascular disease (CVD) is a common cause of morbidity and mortality even in young people with chronic kidney disease (CKD). We examined structural and functional CV changes in patients ˂30 years of age with CKD Stages 4 and 5 and on dialysis. METHODS: A total of 79 children and 21 young adults underwent cardiac computed tomography for coronary artery calcification (CAC), ultrasound for carotid intima-media thickness (cIMT), carotid-femoral pulse wave velocity (cfPWV) and echocardiography. Differences in structural (CAC, cIMT z-score, left ventricular mass index) and functional (carotid distensibility z-score and cfPWV z-score) measures were examined between CKD Stages 4 and 5 and dialysis patients. RESULTS: Overall, the cIMT z-score was elevated [median 2.17 (interquartile range 1.14-2.86)] and 10 (10%) had CAC. A total of 16/23 (69.5%) patients with CKD Stages 4 and 5 and 68/77 (88.3%) on dialysis had at least one structural or functional CV abnormality. There was no difference in the prevalence of structural abnormalities in CKD or dialysis cohorts, but functional abnormalities were more prevalent in patients on dialysis (P < 0.05). The presence of more than one structural abnormality was associated with a 4.5-fold increased odds of more than one functional abnormality (95% confidence interval 1.3-16.6; P < 0.05). Patients with structural and functional abnormalities [cIMT z-score >2 standard deviation (SD) or distensibility <-2 SD) had less carotid dilatation (lumen:wall cross-sectional area ratio) compared with those with normal cIMT and distensibility. CONCLUSIONS: There is a high burden of subclinical CVD in young CKD patients, with a greater prevalence of functional abnormalities in dialysis compared with CKD patients. Longitudinal studies are required to test these hypothesis-generating data and define the trajectory of CV changes in CKD.

PPP2R5D-Related Neurodevelopmental Disorder or Developmental and Epileptic Encephalopathy?: A Novel Phenotypic Description and Review of Published Cases.

BACKGROUND: Protein phosphatase 2 regulatory subunit B' delta (PPP2R5D)-related neurodevelopmental disorder is caused by pathogenic variations in the PPP2R5D gene, product of which is involved in dephosphorylation. This is a rare disorder with description limited to case reports. Its phenotypic spectrum has expanded over the last decade. METHODS: We report a child with a developmental and epileptic encephalopathy phenotype with a pathogenic PPP2R5D variant. This phenotype has not been previously reported. We also reviewed the previously published reports of patients with this disorder. RESULTS: Including the index child, 28 cases (15 girls) were identified from nine relevant research items for analysis. All patients had developmental delay. History of seizures was observed in seven patients while macrocephaly was seen in nearly 80% of patients. Nonneurological manifestations were observed in 13 patients with the most common one being ophthalmological manifestations. The most common genetic variation was c.G592A (p.E198K). The common phenotypic associations of this variation were developmental delay, macrocephaly (11/15), and epilepsy (6/15). CONCLUSION: PPP2R5D gene variations should be suspected in children with developmental delay, autistic features, macrocephaly with or without epilepsy in the absence of any clear etiology. Dysmorphic features might provide a diagnostic clue. DEE phenotype may also be the presenting feature and might be an underreported entity.

Corrigendum to: May Measurement Month 2019: an analysis of blood pressure screening results from India.

[This corrects the article DOI: 10.1093/eurheartjsupp/suab047.].

May Measurement Month 2019: an analysis of blood pressure screening results from India.

AIMS: May Measurement Month is a global screening campaign to raise awareness regarding elevated blood pressure (BP). With the growing burden of hypertension, it is imperative to regularly assess the disease's prevalence, risk factors, and awareness levels in a country. The current prevalence of hypertension in India as per the National Family Health Survey Data stands at 25.3%. May Measurement Month mobilizes healthcare professionals and sensitizes them to regularly measure BP, and impart lifestyle modification advice to the community. It also complements the deficiency in screening programmes at a national and international level. METHODS AND RESULTS: May Measurement Month was carried out in May 2019 as an opportunistic screening campaign for adults (≥18 years). It was carried out by over 5000 trained volunteers across approximately 1000 screening sites (hospitals, public places, pharmacies, villages, and malls) in India. A total of 362 708 (57% males and 42.7% females) people were screened, among whom 68.1% had never measured their BP, and 29.4% (n = 106 522) were found to have hypertension. Of these, only 42.0% were on antihypertensive medication and 23.3% had controlled hypertension. CONCLUSION: Almost a third of the screened population had hypertension, and less than half of those with hypertension were aware of it or on treatment for it. Among those on antihypertensive drugs, BP was controlled in only half of them. These results support the need for greater impetus on BP screening initiatives to detect hypertension early in the community and prevent complications due to uncontrolled BP.