Spectrum of Anemia in Indian children with Nephrotic Syndrome: a prospective observational study.

We aimed to estimate the prevalence of anemia in children with nephrotic syndrome (NS), determine its etiology, and correlate severity with disease duration and response to steroids. This was a prospective cohort study carried from 15th July 2019-14th July 2021 at the pediatric nephrology clinic, of a teaching hospital in India. We screened children aged 3 months-18 years with NS for eligibility. We excluded those suffering from chronic kidney disease and, on haematinics. All children underwent investigations for evaluation of nephrotic syndrome and anemia. To define the clinical phenotype of nephrotic syndrome, the patients were classified as infrequent relapsers, frequent relapsers, steroid dependent and steroid resistant NS as per ISPN guidelines. Children were followed up at least for a period of one year to define their response to steroids. A total of 125 children were finally analysed for all treatment outcomes. Of 125, 37 (30%) children presented with the first episode of NS. Remaining 88 were follow up cases of NS. Of 125 children, 41 (33%) were found to be anemic as per the WHO criteria. Iron deficiency anemia was found in 21 (51%) children. Steroid resistance was twice more prevalent in the anemic group compared to the non-anemic group, 7.3% vs 4.8% respectively, however this difference was not statistically significant, p = 0.65. Anemic group had a trend of higher no. of children receiving antihypertensives compared to non-anemics (38 (93%) vs. 67 (80%), p = 0.07. CONCLUSION: Iron deficiency anemia was the commonest cause of anemia and, anemia and need for anti-hypertensives to attain BP control and adequate proteinuria often coexisted in children suffering from nephrotic syndrome. WHAT IS KNOWN: • Anemia is a significant complication in children suffering from nephrotic syndrome. • Cause of anemia in nephrotic syndrome is multifactorial. WHAT IS NEW: • Iron deficiency anemia was the most common cause of anemia in Indian children with nephrotic syndrome. • Anemia and need for anti-hypertensives to attain adequate BP control and proteinuria often coexisted in children with nephrotic syndrome.

Acute disseminated encephalomyelitis due to a zoonotic co-infection.

Acute disseminated encephalomyelitis (ADEM) is a post-infectious, autoimmune, demyelinating neurological illness, usually attributed to infection with viruses. We describe a case of ADEM occurring in a child with Leptospira-Brucella co-infection. The 12-year-old girl developed a biphasic febrile illness with encephalopathy. On evaluation, she was found to have serological evidence of Brucella and Leptospira infections. Persistence of neurological symptoms after initiating treatment for the co-infection led us to do a magnetic resonance imaging scan of the brain which showed typical findings suggestive of ADEM. Patient responded appropriately to treatment of ADEM with glucocorticoids. The high prevalence of these zoonotic infections in developing countries, and the risk that these may lead to ADEM highlights the importance of detailed evaluation of such cases for proper treatment and better outcomes.

ADEM is a serious neurological disease which occurs as an uncommon complication of certain infections that lead to formation of antibodies which attack the cells of the nervous system. It usually occurs after viral infections, but we came across a 12-year-old girl with ADEM who tested positive for simultaneous infection with two different micro-organisms, both not viruses. These microbes, called Leptospira and Brucella, are common in developing countries and usually lead to infection in individuals in close contact with animals, or with consumption of infected, unpasteurized animal products. Neurological symptoms are uncommon in both infections. However, our case highlights that both infections can occur together and lead to serious neurological illness which needs proper evaluation and a different kind of treatment so that patient has better recovery.

Effect of fetal growth restriction on urinary podocalyxin levels at birth in preterm neonates.

BACKGROUND: Small-for-gestational-age (SGA) neonates are at a higher risk of adult-onset metabolic disorders because of fetal programming in the presence of growth restriction. Nephrogenesis may also be affected in fetal growth restriction. This study hypothesized that urinary podocalyxin levels, a marker of nephrogenesis, would be lower among preterm SGA neonates as compared to appropriate-for-gestational-age (AGA) controls. METHODS: This cross-sectional study enrolled gestation-matched SGA (n = 90) and AGA (n = 45) neonates born at 260-366 weeks of gestation. The SGA group comprised of 45 neonates with birth weight between 3rd and 10th centile and 45 neonates with birth weight <3rd centile. The primary outcome of the study was the difference in urinary podocalyxin levels between SGA and AGA neonates. Glomerular and tubular functions were also assessed. RESULTS: Urinary podocalyxin levels were similar in SGA and AGA neonates (ng/mg of creatinine; median [interquartile range]: 28.7 [4.8-70.2] vs. 18.7 [3.1-55.9]), P value 0.14). No correlation was observed between birth weight centile and urinary podocalyxin levels (r: -0.06). Glomerular filtration rate, fractional excretion of sodium, and serum ß-2-microglobulin levels were comparable across the study groups. CONCLUSIONS: Glomerular development as assessed by urinary podocalyxin levels and renal functions are comparable in SGA and AGA preterm neonates. IMPACT: Neonates born with fetal growth restriction are at a higher risk of adult-onset metabolic disorders because of fetal programming. This cross-sectional study investigated the effect of presence and severity of fetal growth restriction on glomerular development by measuring urinary podocalyxin levels in preterm infants. This study did not observe any effect of the presence or severity of fetal growth restriction on urinary podocalyxin levels and other markers of glomerular and renal tubular functions.

Recognizing and managing hereditary and acquired thrombotic thrombocytopenic purpura in infants and children.

We describe how infants and children with hereditary and acquired autoimmune thrombotic thrombocytopenic purpura (TTP) initially present and how they can be promptly diagnosed and effectively managed. These are uncommon disorders that are commonly misdiagnosed and can be rapidly fatal. TTP is caused by a severe deficiency of the plasma protease, A disintegrin and Metalloprotease with a ThromboSpondin type 1 motif, member 13 (ADAMTS13). Measurement of ADAMTS13 activity is becoming easily accessible. A common presentation of hereditary TTP is neonatal severe hemolysis and hyperbilirubinemia. However, the median age of diagnosis is not until 5.5 years. Plasma is effective treatment for exacerbations and for prophylaxis. Plasma may be replaced by recombinant ADAMTS13 when it becomes available. Acquired TTP is more frequent in older children, in whom it is more common in girls and is commonly associated with systemic lupus erythematosus. For acquired TTP, plasma exchange and immunosuppression are the current treatment for acute episodes; caplacizumab is now commonly used in adults and may replace plasma exchange.

RIN4 homologs from important crop species differentially regulate the Arabidopsis NB-LRR immune receptor, RPS2.

KEY MESSAGE: RIN4 homologs from important crop species differ in their ability to prevent ectopic activity of the nucleotide binding-leucine rich repeat resistance protein, RPS2. Pathogens deploy virulence effectors to perturb host processes. Plants utilize intracellular resistance (R) proteins to recognize pathogen effectors either by direct interaction or indirectly via effector-mediated perturbations of host components. RPM1-INTERACTING PROTEIN4 (RIN4) is a plant immune regulator that mediates the indirect activation of multiple, independently evolved R-proteins by multiple, unrelated effector proteins. One of these, RPS2 (RESISTANT TO P. SYRINGAE2), is activated upon cleavage of Arabidopsis (At)RIN4 by the Pseudomonas syringae effector AvrRpt2. To gain insight into the AvrRpt2-RIN4-RPS2 defense-activation module, we compared the function of AtRIN4 with RIN4 homologs present in a diverse range of plant species. We selected seven homologs containing conserved features of AtRIN4, including two NOI (Nitrate induced) domains, each containing a predicted cleavage site for AvrRpt2, and a C-terminal palmitoylation site predicted to mediate membrane tethering of the proteins. Palmitoylation-mediated tethering of AtRIN4 to the plasma membrane and cleavage by AvrRpt2 are required for suppression and activation of RPS2, respectively. While all seven homologs are localized at the plasma membrane, only four suppress RPS2 when transiently expressed in Nicotiana benthamiana. All seven homologs are cleaved by AvrRpt2 and, for those homologs that are able to suppress RPS2, cleavage relieves suppression of RPS2. Further, we demonstrate that the membrane-tethered, C-terminal AvrRpt2-generated cleavage fragment is sufficient for the suppression of RPS2. Lastly, we show that the membrane localization of RPS2 is unaffected by its suppression or activation status.

Psychopathological Aspects in Children with Epilepsy and Its Contributing Factors: A Cross-Sectional Study from India.

Background Children with epilepsy (CWE) are at high risk of psychopathological problems because of neurobiological, social, and treatment factors. Objectives This study was conducted to estimate the prevalence of psychopathological problems in CWE and their contributing factors. Methods This cross-sectional study was done in pediatric neurology clinic and outpatient department of a government medical college in Northern India. Children between the ages of 4 and 14 years with intelligence quotient > 70 were enrolled; for CWE, the criteria were antiepileptic drugs therapy for more than 6 months and for controls it was being free from any chronic illness. Childhood Psychopathology Measurement Schedule (CPMS) was used for assessing psychopathological problems. Results A total of 135 CWE and 70 controls were enrolled, groups were similar in respect of age, gender, socioeconomic status, and family history. CWE group had significantly high mean ± standard deviation CPMS scores (13.68 ± 10.57) as compared with controls (9.75 ± 7.97) ( p < 0.0001). These scores were particularly high in sectors of low intelligence, conduct disorder, psychotic symptoms, and depression. Academic performance was significantly poor in CWE (39%) versus controls (6%) ( p 0.042). Age of onset, duration, type, and etiology of epilepsy had no significant relation with CPMS scores. Polytherapy and treatment with valproate were associated with high CPMS scores ( p 0.005 and 0.045). Conclusion Psychopathological problems are frequently associated with epilepsy in children and antiepileptic drug therapy might contribute to it.

Infant with a parotid swelling: beware of the rare.

Salivary gland tumours are rare in children. We present the case of an infant who presented with a progressively increasing swelling of short duration in the parotid region accompanied by a small preauricular cutaneous haemangioma. Failing to comply with the commoner diagnoses like parotitis, help of imaging was taken that pointed towards the rarer diagnosis of parotid haemangioma. The child was started on oral propranolol and dramatic reduction in parotid and cutaneous swelling was seen after three months of therapy without any side effects.

Devising Carbon Nanotube, Green Tea, and Polyaniline Based Nanocomposite plus Investigating Its Rheological together with Bactericidal Efficacies.

Subsequently, engines are designed to operate at low viscosity engine oils. Low viscosity oils take less power from engines, bring down the internal drag, cut the fuel consumption, and ultimately improve the engine's efficiency. Considering this focus, an approach has been made to formulate a multiwalled carbon nanotube based green tea and polyaniline nanocomposite, that is, GT/MWCNT/PANI, and incorporate it in engine oil (base fluid). The objective was to reduce the viscosity of engine oil by examining the effects of the constant shear rate and varying shear rates on the viscosity of Castrol class 15W-40 engine oil. The investigation was performed at a constant temperature of 25 °C for a fixed volume fraction of 0.1% GT/MWCNT/PANI in engine oil on the experimental setup rheometer from Anton Paar Series. Primordial findings revealed that, at a constant shear rate of 100 s-1, engine oil viscosity was lowered from 0.221000 to 0.001402 Pa·s, that is, 99% reduction in viscosity of the engine oil, after incorporating the GT/MWCNT/PANI nanocomposite. Furthermore, a new correlation has been proposed considering the experimental and theoretical models with an average percentage error of 0.040%. Also, at varying shear rates, up to 90 s-1, the shear viscosity of nanofluid decreases significantly, leading to shear-thinning behavior of the nanofluid, while at a shear rate of >90 s-1, it shows Newtonian behavior. Besides, the ternary nanocomposite with 0.2 wt % GT/MWCNT/PANI also showed significant bactericidal effects with the zones of inhibition of 19, 18, and 15 mm against Gram-negative (Pseudomonas aeruginosa, Escherichia coli) and Gram-positive (Staphylococcus aureus) bacteria, respectively, as measured using the well diffusion method.

A rare case of pylephlebitis after colonic polypectomy.

Pylephlebitis or infective suppurative thrombosis of the portal mesenteric venous system is an uncommon condition that can potentially be deadly if not recognized and treated early. Although most commonly associated with pancreatitis and diverticulitis, any intra-abdominal or pelvic infection occurring in the region drained by the portal venous system can cause this rare entity. We report a case of a 75-year-old woman who developed post polypectomy pylephlebitis following colonoscopy, a condition rarely reported in the literature.

Isolated pulmonary valve endocarditis masquerading as community-acquired pneumonia.

Isolated pulmonary valve endocarditis in intravenous drug users is a rarely reported phenomenon. We present the case of a 25-year-old male with history of intravenous drug use who presented with respiratory symptoms after failing outpatient treatment for community-acquired pneumonia. Further investigations identified multiple lung lesions with early cavitation, concerning for septic pulmonary embolism on computerized tomography scan, positive blood cultures with methicillin-susceptible staphylococcus aureus, and isolated vegetation of the pulmonic valve on transthoracic echocardiography. The patient had a complete recovery after being treated medically with intravenous oxacillin for a total of 6 weeks.