Clinical and Laboratory Profile of Macrophage Activation Syndrome in Kawasaki Disease: A Single Centre Cross-Sectional Study.

OBJECTIVE: To study the prevalence of macrophage activation syndrome (MAS) in children with Kawasaki disease (KD) and to devise a classification tree for predicting MAS in early KD based on easily available clinical and laboratory information using artificial intelligence (AI) technology. METHODS: A cross-sectional observational study was conducted (March 2020 - October 2021) during which hospitalized children aged 1-18 years with KD were consecutively enrolled. Those with a positive RTPCR test or IgM/IgG serology for COVID-19 were excluded. The clinical and laboratory profiles of children with and without MAS were studied. A multivariable logistic regression (LR) model was developed utilizing backward elimination method to determine the relationship between select candidate predictor variables and MAS in patients with KD. A classification tree was created based on these using artificial intelligence algorithms. RESULTS: Sixty-two children were diagnosed with KD during the study period, of these, 42 children with KD were included; 14 (33.3 %) were diagnosed with MAS. The median (IQR) duration of fever (days) was significantly more in MAS than those without MAS [7 (5, 15) vs 5 (5, 9), P < 0.05]. Serum albumin (g/dL) was significantly lower in those with MAS [2.3 (2.2, 2.7) vs 2.8 (2.3, 3.1), P = 0.03]. The classification tree constructed using the AI-based algorithm predicted that in children with KD who had myocardial dysfunction, serum albumin < 2.8 g/dL and fever > 6 days duration at admission had an increased likelihood of developing MAS. In children without myocardial dysfunction, alanine transaminase (ALT) levels > 70 U/L and fever > 5 days were equally predictive of MAS. CONCLUSION: Nearly one-third of the children with KD had MAS. Clinicians should consider screening all children with KD for MAS at admission. A classification tree based on the presence of myocardial dysfunction, duration of fever > 6 days, ALT levels and hypoalbuminemia can identify MAS in the course of KD.

Short-Chain Fatty Acid (SCFA) as a Connecting Link between Microbiota and Gut-Lung Axis-A Potential Therapeutic Intervention to Improve Lung Health.

The microbiome is an integral part of the human gut, and it plays a crucial role in the development of the immune system and homeostasis. Apart from the gut microbiome, the airway microbial community also forms a distinct and crucial part of the human microbiota. Furthermore, several studies indicate the existence of communication between the gut microbiome and their metabolites with the lung airways, called "gut-lung axis". Perturbations in gut microbiota composition, termed dysbiosis, can have acute and chronic effects on the pathophysiology of lung diseases. Microbes and their metabolites in lung stimulate various innate immune pathways, which modulate the expression of the inflammatory genes in pulmonary leukocytes. For instance, gut microbiota-derived metabolites such as short-chain fatty acids can suppress lung inflammation through the activation of G protein-coupled receptors (free fatty acid receptors) and can also inhibit histone deacetylase, which in turn influences the severity of acute and chronic respiratory diseases. Thus, modulation of the gut microbiome composition through probiotic/prebiotic usage and fecal microbiota transplantation can lead to alterations in lung homeostasis and immunity. The resulting manipulation of immune cells function through microbiota and their key metabolites paves the way for the development of novel therapeutic strategies in improving the lung health of individuals affected with various lung diseases including SARS-CoV-2. This review will shed light upon the mechanistic aspect of immune system programming through gut and lung microbiota and exploration of the relationship between gut-lung microbiome and also highlight the therapeutic potential of gut microbiota-derived metabolites in the management of respiratory diseases.

Serum Calprotectin Levels in Different Subtypes of Juvenile Idiopathic Arthritis (JIA) and Its Correlation with Quantitative CRP and JADAS-27.

OBJECTIVE: To study the serum calprotectin levels in children with juvenile idiopathic arthritis (JIA) and correlate it with C-reactive protein (CRP) and the Juvenile Arthritis Disease Activity Score-27 (JADAS-27). METHODS: This was a cross-sectional observational study done between November 2017 and March 2019. Fifty treatment-naive children, aged 1 to 18 y with the diagnosis of JIA as per the International League of Associations of Rheumatology (ILAR) criteria were enrolled. Assessment of disease activity was done according to the Juvenile Arthritis Disease Activity Score (JADAS-27). Determination of serum calprotectin and CRP levels was done by immunoassay. The correlation between calprotectin levels with CRP and JADAS-27 was calculated. RESULTS: Of the 50 patients with JIA included in the study, there were 18 female and 32 male children. The median age of presentation to the hospital was 9 y (IQR 5.82-13). The median JADAS-27 was 14 (IQR 6, 20.25). The median serum calprotectin level was 45,375 ng/mL (IQR 30,725, 52,270; range 8,560-63,160 ng/mL). The median CRP was 35.4 mg/L (IQR 3.48, 80.3; range 0.02 and 107.4 mg/L). The levels of calprotectin in different JIA subtypes were not statistically different using Kruskal-Wallis test. The study also demonstrated a positive correlation between serum calprotectin with CRP and the JADAS-27 (r = 0.418). CONCLUSION: The calprotectin levels in JIA were significantly higher than those reported in the literature irrespective of the subtype. Serum calprotectin positively correlated with CRP and JADAS-27 in children with JIA.

Relation of Endothelial Progenitor Cells and Carotid Intima Media Thickness in Indian Rheumatoid Arthritis Patients -A Cross Sectional Study.

Carotid intima media thickness (CIMT), a surrogate marker for atherosclerosis, has been used to predict cardiovascular (CV) risk. Recently, endothelial progenitor cells (EPCs) have also garnered interest in its prediction. However a handful of studies, exploring this concept, have come out with disputed results. Notably, there are no Indian studies on this topic as well. Present study evaluates the relation of EPC with CIMT as a prelude to assess the capability of EPC as a marker for CV risk. MATERIAL: This cross sectional pilot study enrolled 30 RA patients fulfilling the inclusion and exclusion criteria. Endothelial progenitor cells (CD34+ and CD45-) and CIMT were determined by flow cytometry and by a single radiologist respectively. The association between EPCs and CIMT was determined with the help of the Spearman correlation coefficient. A p-value < 0.05 was considered statistically significant. OBSERVATION: The mean age of patients was 35.07 ± 10.09 years and the majority (83.3%) were females. The mean (SD) of EPC (%) and CIMT (mm) were 1.05 (0.98) and 0.60 (0.08) respectively. The correlation coefficient between CIMT and EPC was 0.76 (p = <0.001). CONCLUSION: There was a significant positive correlation between EPCs and CIMT. CD34 positive and CD45 negative cells represent circulating endothelial cells rather than hematopoietic progenitor cells. Hence, increased levels of CD34+ and CD45-EPCs in RA patients may highlight atherosclerosis/endothelial damage and may be utilized for risk stratification just as higher CIMTs.

Comparison of clinical and laboratory profile of survivors and non-survivors of SARS-CoV-2-related multisystem inflammatory syndrome of childhood in India: An observational study.

AIM: The SARS-CoV-2 pandemic is characterised by multiple reports of paediatric multisystem inflammatory disease or multisystem inflammatory syndrome in children (MIS-C) with Kawasaki disease-like features often complicated by myocarditis, shock and macrophage activation syndrome. Certain clinical and laboratory markers may be used to identify high risk cases. METHODS: All sequentially admitted patients hospitalised between April 2020 and October 2020, who met the WHO case definition for MIS-C were included. Data included patient demographic information, presenting symptoms, organ dysfunction and laboratory parameters. SARS-CoV-2 infection was diagnosed by nasopharyngeal swab real-time reverse transcription-polymerase chain reaction and/or rapid antibody test for SARS-CoV-2 as recommended. The clinical and laboratory criteria were compared in the survival and non-survival groups. RESULTS: A total of 29 patients with MIS-C were treated during the study period. There were 21 survivors and 8 non-survivors. The non-survivors had more neurocognitive and respiratory symptoms along with increased incidence of myocarditis compared with survivors. The serum levels of CPK-MB, D-dimer, ferritin and triglyceride were significantly raised in non-survivors as compared to survivors. CONCLUSION: The non-survivor group had higher CPK and greater proportion of children with troponin-T elevation indicating higher incidence of myocardial injury and necrosis. The D-dimer, ferritin and triglyceride were also higher in the mortality group, indicating the greater extent of inflammatory damage in this group.

Splenic infarct with scrub typhus: a rare presentation.

Scrub typhus has a variety of clinical presentations ranging from asymptomatic to fever with chills, myalgias, hepatitis, gastric ulcerations and pancreatitis, all being attributed to disseminated vasculitis, with splenic infarction being a rare presentation. A 26-year man, a resident of north India, presented with an acute febrile illness and abdominal pain, computed tomography scan of the abdomen was suggestive of a splenic infarct. After ruling out other aetiology, a positive IgM (ELISA) for scrub typhus led to treatment with oral doxycycline, following which the patient was discharged symptom-free. Rarely can splenic infarction be attributable to scrub typhus; so far, five such cases have been reported in the published literature.

Pulmonary manifestations of leptospirosis.

Leptospirosis has a spectrum of presentation which ranges from mild disease to a severe form comprising of jaundice and renal failure. Involvement of the lung can vary from subtle clinical features to deadly pulmonary hemorrhage and acute respiratory distress syndrome. Of late, it has been identified that leptospirosis can present atypically with predominant pulmonary manifestations. This can delay diagnosis making and hence optimum treatment. The purpose of this review is to bring together all the reported pulmonary manifestations of leptospirosis and the recent trends in the management.

Dengue fever-like illnesses: how different are they from each other?

In tropical countries and possibly elsewhere, dengue fever can be confused with other common tropical infections like enteric fever, leptospirosis, typhus fever, malaria, etc. Many of these illnesses can present in significant numbers after rains, and because of similar early presentations, can cause confusion in decision-making. With global warming, these diseases can assume significant proportions even in non-endemic areas. Identifying these illnesses in a non-immune returning traveller is equally challenging. Recognition of these diseases is important to diagnose them and treat them early, in order to avoid potentially fatal complications. This review is an attempt to highlight important clinical and laboratory differences among dengue fever-like illnesses.

Hemoptysis with a classical radiological sign.

There can be various causes for hemoptysis. We present here a case of hemoptysis in which the chest X-ray helped us to arrive at the diagnosis because of a classical sign. This highlights the need of identifying this finding for prompt recognition of the cause of hemoptysis.

Diffuse lymphomatous infiltration of kidney presenting as renal tubular acidosis and hypokalemic paralysis: case report.

We report the case of a 22-year-old woman who presented with acute onset flaccid quadriparesis. Physical examination showed mild pallor with cervical and axillary lymphadenopathy, hepatomegaly, and bilateral smooth enlarged kidneys. Neurological examination revealed lower motor neuron muscle weakness in all the four limbs with hyporeflexia and normal sensory examination. Laboratory investigations showed anemia, severe hypokalemia, and metabolic acidosis. Urinalysis showed a specific gravity of 1.010, pH of 7.0, with a positive urine anion gap. Ultrasound revealed hepatosplenomegaly with bilateral enlarged smooth kidneys. Renal biopsy was consistent with the diagnosis of non-Hodgkin lymphoma (B cell type). Metabolic acidosis, alkaline urine, and severe hypokalemia due to excessive urinary loss in our patient were suggestive of distal renal tubular acidosis. Renal involvement in lymphoma is usually subclinical and clinically overt renal disease is rare. Diffuse lymphomatous infiltration of the kidneys may cause tubular dysfunction and present with hypokalemic paralysis.

Atypical manifestations of dengue.

As the spread of dengue and dengue haemorrhagic fever is increasing, atypical manifestations are also on the rise, although they may be under reported because of lack of awareness. This review compiles descriptions of atypical manifestations of dengue, such as dengue encephalitis, dengue myocarditis, dengue hepatitis and dengue cholecystitis.

Chyluria with proteinuria or filarial nephropathy? An enigma.

Lymphatic filariasis is endemic in India. Out of 128 million infected individuals worldwide, India accounts for 48 million cases [Manson's Tropical Diseases, 21st Ed. p 1488]. Filariasis can have protean manifestations, but Tropical pulmonary eosinophilia and chyluria are unusual manifestations reported mainly from South Asian countries [Manson's Tropical Diseases, 21st Ed. p 1494]. Chyluria occurs only in 2% of filarial afflicted patients in the filarial belt [Diamond E, Schapira HE. Chyluria--a review of literature. Urology 1985;26(5): 427-31]. Lymphatic filariasis presenting as chyluria may be equally rare. Predominant chyluria with no overt lymphatic filariasis remains an enigma.