Ankur Kumar Jindal

Healthy Donor Fecal Microbiota Transplantation in Steroid-Ineligible Severe Alcoholic Hepatitis: A Pilot Study

© 2017 by the AGA Institute 1542-3565/

Carvedilol delays the progression of small oesophageal varices in patients with cirrhosis: a randomised placebo-controlled trial

36.00 http://dx.doi.org/10.1016/j.cgh.2016.10.029 Patients with untreated severe alcoholic hepatitis (SAH) have 50% 28-day survival; much worse in the steroid-ineligible group. Gut dysbiosis is common and plays a role in the development and progression of alcoholic liver disease. We undertook to modulate gut microbiota in patients with SAH through healthy donor fecal microbiota transplantation (FMT) therapy.

Impact of family history of metabolic traits on severity of NASH related cirrhosis: a cross‐sectional study

Background and aims Carvedilol is effective in the primary prophylaxis for large oesophageal varices. We investigated its use in preventing progression of small to large oesophageal varices. Methods Consecutive cirrhotics with small oesophageal varices were prospectively randomised to either carvedilol (n=70) or placebo (n=70) and followed up for a minimum of 24 months. Endoscopy was done at baseline and six monthly intervals. Hepatic vein pressure gradient (HVPG) was measured at baseline and at 12 months. The primary endpoint was development of large varices. Results Baseline characteristics in two groups were comparable. The predominant aetiology of cirrhosis was non-alcoholic fatty liver disease in both the groups. The mean dose of carvedilol administered was 12±1.67 mg/day and the target heart rate achieved was 58±3 bpm. A higher proportion of patients in carvedilol group had non-progression to large varices than placebo (79.4% vs 61.4%; p=0.04); the mean time of non-progression to large varices was 20.8 months (95% CI 19.4 to 22.4) in carvedilol group and 18.7 months (95% CI 17.1 to 20.4) in placebo group (p=0.04). There was a modest reduction of HVPG at 1 year in carvedilol group (−8.64%) compared with placebo (+0.33%) (p=0.22). None of the patients in either group died of variceal bleeding or liver-related causes. No major adverse events were observed in either group. Conclusions Carvedilol is safe and effective in delaying the progression of small to large oesophageal varices in patients with cirrhosis. Trial registration number NCT01196507; post-results.

Diagnosis of Kawasaki disease

Familial aggregation of metabolic traits with fatty liver disease is well documented. However, there is scarcity of data regarding such association with non‐alcoholic steatohepatitis (NASH)‐related cirrhosis. This study was aimed to explore the association of family history of metabolic traits with severity of cirrhosis.

Efficacy of narrowband ultraviolet B phototherapy and levels of serum vitamin D3 in psoriasis: A prospective study

Kawasaki disease (KD) is a medium vessel vasculitis with predilection for coronary arteries. Due to lack of a reliable confirmatory laboratory test, the diagnosis of KD is based on a constellation of clinical findings that appear in a typical temporal sequence. These diagnostic criteria have been modified from time to time and the most recent guidelines have been proposed by the American Heart Association (AHA) in 2017. However, several children may have incomplete or atypical forms of KD and the diagnosis can often be difficult, especially in infants and young children. In this review, we have detailed the steps involved in arriving at a diagnosis of KD and also highlight the important role of echocardiography in diagnosis and management of children with KD.

Infectious and non-infectious complications in primary immunodeficiency disorders: an autopsy study from North India

Introduction: Narrowband ultraviolet B phototherapy (NBUVB) is safe and effective treatment for psoriasis. Vitamin D plays an important role in pathogenesis of psoriasis. It is known that psoriasis patients have low serum 25(OH)D levels, which increase after NBUVB. We assessed serum 25(OH)D levels, its correlation with Psoriasis Area and Severity Index (PASI), and the effect of NBUVB on 25(OH)D levels among Indian psoriasis patients. Materials and Methods: A prospective study comprising 30 adults with psoriasis with no major comorbidities (PASI > 10 and off-therapy >4 weeks) was conducted. PASI was estimated at baseline among patients and repeated after receiving 12 weeks of NBUVB therapy. Thirty age and gender-matched healthy controls were recruited to compare 25(OH)D levels at baseline and at 12 weeks. Patient demographic parameters, treatment dose, duration, side effects, and its impact on 25(OH)D levels and PASI were serially evaluated. Results: A total of 30 patients presenting with psoriasis and 30 healthy controls were enrolled in the study. Mean baseline PASI (M: F =19:11) among patients with mean age 36.8 (±7.7) years was 20.5 (±6.3) and all patients were either 25(OH)D deficient (n = 14) or insufficient (n = 16). Their baseline 25(OH)D levels were significantly lower than controls (25.93 nmol/L vs 47.54 nmol/L; P < 0.001). After NBUVB therapy (average cumulative dose 20.76 ± 7.1 J/cm2; average treatment sessions 32.57 ± 1.9), there was a significant improvement in PASI as well as 25(OH)D (P < 0.05). There was no correlation between the mean improvement in PASI and 25(OH)D after 12 weeks of therapy. Twelve (40%) patients had therapy-related side effects [pruritus (n = 8), erythema (n = 4)], none had major side effects. Conclusion: Improvement in PASI and serum 25(OH)D levels after NBUVB in psoriasis is significant but poorly correlated with each other. Vitamin D may not be the lone mediator of the therapeutic effects of NBUVB on psoriasis.

The emergence of Kawasaki disease in India and China

Background Primary immunodeficiency disorders (PID) include a wide spectrum of inherited disorders characterised by functional abnormalities of one or more components of the immune system. Recent updates from the genomic data have contributed significantly to its better understanding with identification of new entities. Diagnosis is always challenging due to their variable clinical presentation. With the evolution of molecular diagnosis, many of these children are being diagnosed early and offered appropriate therapy. However, in developing countries, early diagnosis is still not being made: as a result these patients succumb to their disease. Autopsy data on PID is notably lacking in the literature with histopathological evaluation of PID being limited to rare case reports. Objective To analyse the clinical, immunologic (including mutational) and morphologic features at autopsy in 10 proven and suspected cases of primary immunodeficiency disorders diagnosed at our Institute over the past decade. Methods Study includes a detailed clinico-pathological analysis of 10 proven and suspected cases of primary immunodeficiency disorders. Results A varied spectrum of infectious and non-infectious complications were identified in these cases of which fungal infections were found to be more frequent compared with viral or bacterial infections. Rare and novel morphological findings, like granulomatous involvement of the heart in a patient with chronic granulomatous disease, systemic amyloidosis in a teenage girl with X-linked agammaglobulinemia, are highlighted which is distinctly lacking in the literature. Conclusions The present study is perhaps the first autopsy series on PID. Even in the molecular era, such analysis is still important, as correlation of pathological features with clinical symptoms provides clues for a timely diagnosis and appropriate therapeutic intervention.

Severe Aspergillus Pneumonia and Pulmonary Artery Hypertension in a Child with Autosomal Recessive Chronic Granulomatous Disease and Selective IgA Deficiency

Kawasaki disease (KD) is recognized as a leading cause of acquired heart disease in children in developed countries. Although global in distribution, Japan records the highest incidence of KD in the world. Epidemiological reports from the two most populous countries in the world, namely China and India, indicate that KD is now being increasingly recognized. Whether this increased reporting is due to increased ascertainment, or is due to a true increase in incidence, remains a matter of conjecture. The diagnosis and management of KD in developing countries is a challenging proposition. In this review we highlight some of the difficulties faced by physicians in managing children with KD in resource-constrained settings.

Pulmonary presentation of Kawasaki disease—A diagnostic challenge

To the editor Chronic granulomatous disease (CGD) is a phagocyte disorder characterized by a defect in the neutrophil respiratory burst that predisposes to recurrent infections with catalase-positive microorganisms [1, 2]. In addition, these patients have inflammatory complications predominantly involving the gastrointestinal tract, lungs, and urinary tract [3, 4]. It is difficult to differentiate clinically the inflammatory lung disease from bacterial or fungal pneumonia. Selective IgA deficiency is the most common immunodeficiency disorder and it may also produce autoimmune manifestations [5, 6]. Co-occurrence of CGD and selective IgA deficiency is rare and seldom reported in literature. A 7-year-old boy, born of a non-consanguineous marriage, presented to us with a history of a cough, respiratory distress, and fever for 1 month. He had had two hospitalizations during the last 1 month for these complaints. In the past, at 6 years of age, he had an episode of dysentery, for which oral antimicrobials were prescribed. Three of his male siblings had died (at 3, 7, and 5 years of age, respectively) because of an unexplained acute respiratory illness. On examination, he had pallor, grade 2 clubbing and crepitations on auscultation of the chest. Rest of the examination was normal. Laboratory investigations revealed hemoglobin 112 g/L, white blood cell count 19 × 10 cells/L (differential count polymorphs 70%, lymphocytes 28%, and monocytes 2%), platelet counts 411 × 10/L, alanine aminotransferase (ALT) 414 U/L, aspartate aminotransferase (AST) 484 U/L, and human immunodeficiency virus (HIV) serology by enzyme-linked immunosorbent assay (ELISA) was non-reactive. Immunoglobulin profile revealed IgG 1710 mg/dL (normal range 540–1610), IgA <17 mg/dL (normal range 50–240), and IgM 141 mg/dL (normal range 50–180). Lymphocyte subset analysis revealed CD3+ T cells 70%, CD3+ CD4+ T cells 14%, CD19+ B cells 13%, and CD16+/56+ natural killer cells 6.3%. Nitroblue tetrazolium dye reduction test revealed no reduction of NBT dye by stimulated neutrophils and dihydrorhodamine 123 assay revealed markedly reduced stimulation index in the patient neutrophils as compared to control (1.08 in the patient and 159.5 in the control). Flow cytometric expression of the b558 protein on neutrophils was normal. DNA sequencing of the NCF-1 gene revealed a homozygous GT deletion in the exon 2 (Supplementary figure). Chest X-ray revealed ground-glass opacities in bilateral lung fields (Fig. 1a) and contrastenhanced computerized tomography (CECT) scan of the chest revealed diffuse ground-glass opacities, septal thickening, nodules, interspersed cysts, and dilatedmain pulmonary artery and right ventricle (Fig. 1b–d). Blood culture was sterile and culture from tracheal aspirate grew Aspergillus flavus. Gastric lavage for acid-fast bacilli (AFB) stain revealed no acid-fast bacilli and tuberculin skin test was non-reactive. Antinuclear antibody (ANA) test showed 2+ speckled pattern (on indirect immunofluorescence at 1:40 dilution) and anti-doubles t r a n d e d DNA an t i b o d y t i t e r w a s 4 0 IU /mL All authors have contributed equally to the preparation and editing of this manuscript.

Autoantibodies in children with juvenile dermatomyositis: A single centre experience from North-West India

Kawasaki disease (KD) is a multisystemic vasculitis with predominant mucocutaneous manifestations. Pulmonary involvement in KD is distinctly uncommon and is not commonly recognized. We describe our experience of managing children with KD wherein the initial presentation was predominantly pulmonary.