Recurrent episodes of febrile dyspnoea: hypersensitivity pneumonitis caused by a household ultrasonic humidifier.

Hypersensitivity pneumonitis is an immune-mediated interstitial lung disease that presents with respiratory symptoms, with or without systemic symptoms, following exposure to an identified or unidentified external factor. It can be caused by extrinsic factors including household items such as ultrasonic humidifiers.We present an intriguing case of a previously healthy 50-year-old man who displayed recurrent episodes of progressive dyspnoea and fever after repeated exposure to his household ultrasonic humidifier. He was treated with corticosteroids, followed by the removal of the humidifier, resulting in total recovery and absence of recurrence of further episodes.The clinical presentation of hypersensitivity pneumonitis can be dramatic, and the differential diagnosis is broad. The correct diagnosis is achieved by combining clinical, radiological and histopathological patterns. The key to finding the aetiology lies in a thorough history, with an important role for household investigations to identify the external factor.

The diagnostic performance of functional dopaminergic scintigraphic imaging in the diagnosis of dementia with Lewy bodies: an updated systematic review.

INTRODUCTION: Dopaminergic scintigraphic imaging is a cornerstone to support the diagnosis in dementia with Lewy bodies. To clarify the current state of knowledge on this imaging modality and its impact on clinical diagnosis, we performed an updated systematic review of the literature. METHODS: This systematic review was carried out according to PRISMA guidelines. A comprehensive computer literature search of PubMed/MEDLINE, EMBASE, and Cochrane Library databases for studies published through June 2022 was performed using the following search algorithm: (a) "Lewy body" [TI] OR "Lewy bodies" [TI] and (b) ("DaTscan" OR "ioflupane" OR "123ip" OR "123?ip" OR "123 ip" OR "123i-FP-CIT" OR "FPCIT" OR "FP-CIT" OR "beta?CIT" OR "beta CIT" OR "CIT?SPECT" OR "CIT SPECT" OR "Dat?scan*" OR "dat scan*" OR "dat?spect*" OR "SPECT"). Risk of bias and applicability concerns of the studies were evaluated using the QUADAS-2 tool. RESULTS: We performed a qualitative analysis of 59 studies. Of the 59 studies, 19 (32%) addressed the diagnostic performance of dopamine transporter imaging, 15 (25%) assessed the identification of dementia with Lewy bodies in the spectrum of Lewy body disease and 18 (31%) investigated the role of functional dopaminergic imaging in distinguishing dementia with Lewy bodies from other dementias. Dopamine transporter loss was correlated with clinical outcomes in 19 studies (32%) and with other functional imaging modalities in 15 studies (25%). Heterogeneous technical aspects were found among the studies through the use of various radioligands, the more prevalent being the [123I]N­ω­fluoropropyl­2ß­carbomethoxy­3ß­(4­iodophenyl) nortropane (123I-FP-CIT) in 54 studies (91.5%). Image analysis used visual analysis (9 studies, 15%), semi-quantitative analysis (29 studies, 49%), or a combination of both (16 studies, 27%). CONCLUSION: Our systematic review confirms the major role of dopaminergic scintigraphic imaging in the assessment of dementia with Lewy bodies. Early diagnosis could be facilitated by identifying the prodromes of dementia with Lewy bodies using dopaminergic scintigraphic imaging coupled with emphasis on clinical neuropsychiatric symptoms. Most published studies use a semi-quantitative analytical assessment of tracer uptake, while there are no studies using quantitative analytical methods to measure dopamine transporter loss. The superiority of a purely quantitative approach to assess dopaminergic transmission more accurately needs to be further clarified.

Patient Characteristics and Outcomes in Necrotizing Soft-tissue Infections: Results from a Prospective Cohort Study in a Tertiary Care Center Intensive Care Unit in South India.

Background: Necrotizing soft tissue infections (NSTIs) are life-threatening infections characterized by progressive destruction of muscle, fascia, and overlying subcutaneous tissue. Prospective studies in the field are few, and data from the Indian subcontinent are bleak. Prompt diagnosis and timely treatment are critical for optimal outcomes. The aims of this study are to provide detailed information on the clinical profile of patients with NSTIs and to identify predictors of mortality in order to pick up reversible factors that may improve outcomes. Materials and methods: This study was a prospective cohort study of adult patients with NSTIs in a tertiary center in South India. All patients who were admitted to the surgical intensive care unit (ICU) of the institute with a diagnosis of NSTI were screened and enrolled. All patients were managed according to the local protocol for treatment of NSTIs and intensive care support. Results: In our cohort of patients, simple and multiple logistic regression analysis showed that four factors, namely, AKIN stage 3, shock, need for mechanical ventilation for more than 3 days, and low serum albumin values were found to be significantly associated with higher mortality. Conclusion: The successful management of these patients calls for early diagnosis, resuscitation, surgical debridement, appropriate and timely antibiotics, and early ventilatory weaning before multi-organ failure associated with shock and AKI occurs. How to cite this article: Kurian GP, Korula PJ, Jacob JM, Desha AMK, Karuppusami R, Kandasamy S. Patient Characteristics and Outcomes in Necrotizing Soft-tissue Infections: Results from a Prospective Cohort Study in a Tertiary Care Center Intensive Care Unit in South India. Indian J Crit Care Med 2022;26(4):452-456.

Simethicone and N-acetyl cysteine combination as premedication before esophagogastroduodenoscopy: Double-blind randomized controlled trial.

Background and study aims Esophagogastroduodenoscopy (EGD), the most common method used for diagnosing upper gastrointestinal diseases, is often limited by the presence of foam and mucous. Thus, this study was designed to detect whether the combination of simethicone with N-acetyl cysteine (NAC) as premedication before EGD improves mucosal visualization. Patients and methods A total of 768 consenting patients were enrolled in this prospective, double-blind, randomized placebo-controlled trial in four groups (A: simethicone + N-acetyl cysteine; B: simethicone alone; C: NAC alone; and D: placebo). After 20 minutes of consuming the corresponding solution, EGD was done and multiple images were obtained from the esophagus, stomach, and duodenum. Based on the various images obtained, the study parameters were calculated. Statistical Analysis Software (SAS) was used to analyze the results using Kruskal-Wallis with the Bonferroni correction method. Results The study population consisted of 57 % men and the mean age was 44.18 years. Each group was randomized with 192 participants. Group A (combination of simethicone + NAC) premedication had the lowest total mucosal visibility score of 8.31, a significantly lower score for mucous/bubbles obscuring the vision, and less time to complete the procedure. Also, 81 % of the participants in group A did not require flushing to clear the mucous/bubbles. There were no side effects due to this premedication in any of the groups. Conclusions Using simethicone and NAC combined for premedication may improve the quality of EGD.

Vigorous achalasia: Zebra amongst horses.

Vigorous achalasia is an oesophageal disorder with clinical and radiological characteristics of classic achalasia and diffuse oesophageal spasm. It is a rarely reported variant. A 60-year-old gentleman presented with complaints of difficulty in swallowing, regurgitation and chest pain for the past 10 years. His symptoms persisted despite the use of proton pump inhibitors. On endoscopy and barium swallow, the diagnosis of vigorous achalasia was confirmed. It is a rare variant of classic achalasia usually misdiagnosed as diffuse oesophageal spasm.

Changes in Left Ventricular Mass and Cardiovascular Risk Factors after Kidney Transplantation.

Left ventricular hypertrophy (LVH), the most common structural cardiac complication, is the single most important cause for sudden cardiac death. There are no published data from India looking at the changes in left ventricular mass and cardiac dysfunction after kidney transplantation. We aimed to determine the changes in the left ventricular mass and other cardiovascular risk factors in kidney transplant recipients. This was a prospective observational study. All patients who underwent kidney transplantation at Amrita Institute of Medical Sciences and Research Centre, Kochi, Kerala, during the study period were included in the study. Measurement of clinical and biochemical parameters and echocardiography were done before, six months, and one year after transplantation. There was significant reduction in LV mass index (124.8 ± 39 vs. 102.2 ± 24.4 g/m2, P <0.001) and improvement in ejection fraction (57.8 ± 7 vs. 60.1 ± 1.9, P = 0.015) at the end of six months. There were significant differences in the mean hemoglobin, systolic, and diastolic blood pressures (P <0.001) during the study. There was also a significant reduction in the number of antihypertensive drugs required for blood pressure control. There was a significant reduction in LVH in the study group. There was also improvement in systolic and diastolic functions of the heart. There was also a significant improvement in blood pressure control both in terms of mean blood pressure levels as well as in terms of the number of anti-hypertensive drugs needed for blood pressure control. Renal transplantation ameliorates cardiovascular risk in renal transplant recipients.

Indian Subcontinent's First Bilateral Supracondylar Level Upper Limb Transplantation.

Introduction This is the first case of supracondylar level transplant from the Indian subcontinent, performed for a bilateral below elbow amputee. It has a completely different set of challenges for the transplant team, with a relatively shorter ischemia time window. The technical considerations for the same have been discussed in detail in this article. Materials and Methods The patient was a 19-year-old female who lost her both upper limbs at proximal forearm level due to severe crush injury following a road traffic accident. Insufficient bone length on either side necessitated a supracondylar level transplant. The preoperative workup included detailed clinical evaluation, biochemical, and psychological evaluation. The donor was a young brain-dead, male patient from a hospital, 30 minutes away. The donor and recipient preparations in this case were unique. The recipient's own elbow flexors and extensors were used while the elbow joint was from the donor. The specific challenges we faced during this procedure have been described in detail. Results The transplantation has been a complete technical success, with the patient rehabilitated back to her independent life style. This article describes only the technical considerations. The functional recovery aspect is part of an another soon to be published manuscript. Conclusion Supracondylar level arm-transplant requires a highly coordinated team effort with precise preoperative planning, along with meticulous attention to detail to achieve a successful outcome. In properly selected patients, it could be a life-changing procedure, worth all the effort.

Feasibility and Accuracy of a Nonmedical Research Person in Assimilation and Calculation of Acute Physiologic Assessment and Chronic Health Evaluation Scores in an Indian Intensive Care Unit.

BACKGROUND: The Physiologic Assessment and Chronic Health Evaluation (APACHE) score assimilation and calculation, as well as other demographic data collection, is inherent to research and nonresearch related needs of intensive care. There may be a role for well-trained nonmedical personnel to collect this vital material to enhance research and the standard of care in the Intensive Care Units (ICUs) in countries that are poorly funded and resourced in terms of medical personnel. AIMS: The aim of this study is to verify the interrater reliability of a trained nonmedical personnel and ICU trainee in the collection and calculation APACHE scores. MATERIALS AND METHODS: In a prospective study, two raters who were blinded, one a trained nonmedical ward clerk and another an ICU trainee, assimilated data and calculated APACHE scores for 60 consecutive patients admitted to two tertiary mixed ICUs (with a total of 19 beds). Primary outcomes were to assess interrater and interclass correlation as well as the agreement of scores between the two raters. RESULTS: There was an excellent correlation of APACHE scores (Kappa coefficient of 0.92) and Bland-Altman plot depicted overall good agreement with low bias between raters. CONCLUSIONS: A well-trained and supervised nonmedical research person can assimilate and calculate APACHE II scores with good agreement with an ICU trainee. This may help in deriving data from medically understaffed ICUs in India, thus promoting much-needed research from such ICUs.

Maintenance zinc therapy after initial penicillamine chelation to treat symptomatic hepatic Wilson's disease in resource constrained setting.

BACKGROUND: Experience with zinc in treating symptomatic hepatic Wilson's disease (WD) is limited. AIM: To study the efficacy of Penicillamine followed by zinc in treating symptomatic hepatic Wilson's disease. METHODS: We retrospectively analyzed case records of 31 symptomatic hepatic WD patients for whom disease severity scores (Child's, model for end-stage liver disease (MELD), Nazer's, and New Wilson Index (NWI) score) and 24-h urinary copper were compared at 3-time points-baseline at presentation, at transition from penicillamine to zinc and at end of follow up. RESULTS: Thirty-one patients (median age 11 [5-24] years) with symptomatic hepatic WD were studied; ten had associated neuropsychiatric manifestations of WD. Penicillamine was changed to zinc sulfate either due to financial constraints (28 patients) or due to adverse effects of penicillamine (3 patients). At presentation (baseline), six patients belonged to Child's class A, five to Child's B, and 17 to Child's C. Duration of initial penicillamine chelation therapy was 134 (2-320) weeks, and of subsequent zinc therapy was 363 (35-728) weeks. There was a significant improvement in liver function tests and disease severity scores (Child's, MELD, Nazer's, and NWI score) at the transition from penicillamine to zinc compared to baseline. This improvement was maintained until the end of study period with 90% survival at 10 (2-20) years. Fifteen of the 17 Child's C cirrhotic patients showed significant improvement in disease severity scores from baseline until end of follow up. CONCLUSIONS: Penicillamine followed by zinc may be a safe and effective treatment in resource-constrained setting for symptomatic hepatic WD patients in all grades of baseline disease severity. Some patients with decompensated cirrhosis due to WD may be managed with medical treatment, avoiding liver transplantation.

First two bilateral hand transplantations in India (Part 4): Immediate post-operative care, immunosuppression protocol and monitoring.

INTRODUCTION: Being able to counter immune-mediated rejection has for decades been the single largest obstacle for the progress of vascular composite allotransplantation (VCA). The human immune system performs the key role of differentiating the 'self ' from the 'non-self '. This, although is quintessential to eliminate or resist infections, also resists the acceptance of an allograft which it promptly recognises as 'non-self'. MATERIALS AND METHODS: Pre-operative evaluation of the recipient evaluation included immunological assessment in the form of panel reactive antibodies (PRA), human leucocyte antigen (HLA) typing, donor-specific antibody detection assays (DSA) and complement-dependent cytotoxicity assays (CDC). Induction immunosuppression was by thymoglobulin and the maintenance by the standard triple-drug therapy. RESULTS: Both the recipients were managed by the standard triple drug therapy and have had only minor episodes of rejections thus far which have been managed appropriately. DISCUSSION: Induction immunosuppression was by thymoglobulin and the maintenance by the standard triple-drug therapy. Various groups have tried various other formulations and regimes as well. CONCLUSION: A comprehensive plan has to be drawn up for immunological screening, selection and the post-operative immunosuppressant usage. The ultimate goal of these immunosuppression modalities is to achieve a state of donor-specific tolerance.

Schimidt Syndrome: An Unusual Cause of Hypercalcaemia.

Autoimmune polyglandular syndrome type 2 also known as Schmidt syndrome. It is a rare disorder involving a combination of Addison's disease with autoimmune thyroid disease with or without type 1 diabetes mellitus. In this case report one such patient with this rare syndrome is described who presented with hyperpigmentation of knuckles, palms and soles with significant weight loss for 2 months. At presentation she also had severe hypercalcaemia. Severe hypercalcaemia is rare and hypercalcaemia at the initial presentation of Addison's disease is also unusual. The mechanism of hypercalcaemia in addisons and management of this patient is discussed.

Cutaneous Hyperpigmentation in Megaloblastic Anemia: a Five Year Retrospective Review.

BACKGROUND: Cutaneous hyperpigmentation is an often overlooked clinical sign in megaloblastic anemia (MA) which has been sporadically reported in the literature. METHODS: We describe the bone marrow (BM) changes and clinicolaboratory characteristics of 25 of 198 adult cases (>16 years) with cutaneous hyperpigmentation who underwent BM evaluation for cytopenia (s). RESULTS: Twenty-one of 25 cases (84%) had MA, while MA without hyperpigmentation occurred only in 12 of remainder 173 cases (P<0.001). Knuckle pad hyperpigmentation (KP) was noted in 16 (64%) cases; whereas 9 (36%) had diffuse brownish black discoloration (DP) of the palms and/or soles. Eighteen of 25 (72%) cases had pancytopenia (13 with KP) and 7 of 25 (28%) had bicytopenia (3 with KP). In addition, five cases (20%) presented with pyrexia. Of the 17 cases where data available, eleven were B12 deficient [<190 pg/ml; eight had severe deficiency (<100 pg/ml); ref.; 190-800pg/ml], while 4 had pure folate deficiency (< 4.0 ng/ml; ref.; 4-20ng/ml); and remainder 2 had combined B12 and folate deficiency. Compared to those with diffuse pigmentation; KP group had lower Hb (69.6 ± 24.2 vs. 86.3 ± 33.9 g/L), higher MCV (106.1 ±12.6 vs. 99.2 ± 7.6 fL), lower platelet count (50.9 ± 29.3 vs. 69.6 ± 36.5 × 10(9)/L), and lower median B12 [100.0 (30.0 - 822.0) vs. 316.0 (142.0 - 1617.3) pg/ml] (P>0.05). In six cases where follow-up data were available, there was a significant reversal of hyperpigmentation at 12 weeks following parenteral cobalamin therapy. In all five cases with pyrexia, fever subsided after 24 to 72 hours following administration of parenteral cobalamin therapy. CONCLUSION: Cutaneous hyperpigmentation and cytopenia (s) are strongly associated with megaloblastic anemia. Knuckle pad hyperpigmentation is much more frequent than diffuse pigmentation of the palms and/or soles in such patents. A nonsignificant trend towards a greater degree of MA was found in cases with pigmentation of the knuckles.

Fibrosis in non-alcoholic fatty liver disease: correlation with simple blood indices and association with tumor necrosis factor-alpha polymorphisms.

AIM: The study was conducted with an aim to evaluate the clinico-pathological profile, the correlation of AST: ALT ratio and APRI with histological fibrosis, and the frequency of two specific polymorphisms (-238, -308) in the TNF alpha promoter region in patients with NAFLD. METHODS: The present study compared aspartate transaminase/alanine transaminase (AST/ALT) ratio and AST-to-platelet ratio index (APRI) with fibrosis score in 29 patients who underwent liver biopsy for NAFLD. Single nucleotide polymorphisms (SNP) in the tumor necrosis factor-alpha (TNF-alpha) promoter region at positions -308 and -238 were examined by polymerase chain reaction-restriction fragment length polymorphism. RESULTS: AST/ALT ratio correlated better than the APRI with liver fibrosis in patients with NAFLD (AUROC of 0.9 compared to 0.68). TNF-alpha promoter region SNPs were present in only a minority of patients, and did not correlate with fibrosis severity. CONCLUSIONS: AST/ALT ratio correlated well with liver fibrosis in Indian patients with NAFLD. The SNPs studied had no role in development of fibrosis in Indian patients with NAFLD.

Nail-patella syndrome-a novel mutation in the LMX1B gene.

Nail-patella syndrome (NPS) is an autosomal-dominant pleiotropic disorder characterized by dyplasia of finger nails, skeletal anomalies and frequently renal disease. In the reported case, genetic analysis revealed a new missense mutation in the homeodomain of LMX1B, presumed to abolish DNA binding (c.725T>C, p.Val242Ala). A missense mutation at codon 725 was identified, where thymine was replaced by cytosine which led to the replacement of valine by alanine at position 242. It was not detected in both parents. A 2005 study by Bongers et al. described a significant association between the presence of clinically relevant renal involvement in an NPS patient and a positive family history of nephropathy, which was lacking in our case.

Successful Pregnancy in Autoimmune Hepatitis/Primary Biliary Cirrhosis Overlap Syndrome: A Case Report.

Medical literature on pregnancy outcomes in hepatic overlap syndromes is limited. Autoimmune liver diseases are associated with sub-fertility and pregnancy is usually dissuaded in presence of portal hypertension and cirrhosis due to guarded prognosis for the pregnancy as well as the liver disease. We report a 24-year-old primigravida with autoimmune hepatitis/primary biliary cirrhosis overlap syndrome who had a successful pregnancy owing to intensive monitoring and meticulous treatment with drugs like immunosuppressants and ursodeoxycholic acid. She developed preterm prelabour rupture of membranes and delivered at 35 weeks without any major obstetric or hepatic complications.

Genetics of Wilson's disease: a clinical perspective.

Hepatic Wilson's disease is often a difficult diagnosis to confirm. This review examines the current role of genetic tests for Wilson's disease and is aimed at clinicians caring for patients with this disease. We discuss how genetic testing is carried out for Wilson's disease, indications for these tests, and genetic counseling for the family. In contrast to the advances in diagnosis of Wilson's disease by testing for ATP7B mutations, genotype-phenotype correlations are not yet sufficiently established. The non-Wilsonian copper overload syndromes causing cirrhosis in children are another important area for study. The review also identifies further areas for research into the genetics of Wilson's disease in India.

Cytokine gene polymorphisms in irritable bowel syndrome in Indian population--a pilot case control study.

AIM: This study aimed at evaluating the single nucleotide polymorphisms (SNPs) in five cytokine genes regulating inflammation at altogether 8 different loci and compared their frequencies in patients with Irritable bowel syndrome (IBS) versus healthy age and sex matched controls. METHODS: Peripheral blood was collected for DNA cytokine analysis from 23 patients with lBS and 20 healthy controls. The cytokine SNPs studied include TNF-alpha (-308G/A), TGF-alpha1 (codon10T/C, codon25G/C), IL-6 promoter (-1082A/G; -819T/C; -592A/C), IL-6 promoter (-174G/C), and IFN-alpha (+874T/A). RESULTS: There was a significant difference between a SNP in IL-b (-592A/C) among cases and controls. There was also a trend to significance as regards to IL-6 promoter (-174G/C). Frequencies of other SNPs were not significantly different between the two groups. CONCLUSION: This pilot study shows that there are polymorphism differences in cytokine genes between patients with lBS and healthy controls from India.

Idiopathic non-cirrhotic intrahepatic portal hypertension: common cause of cryptogenic intrahepatic portal hypertension in a Southern Indian tertiary hospital.

BACKGROUND AND AIM: Patients with intrahepatic portal hypertension and negative etiological work-up for liver disease are often labeled as having cryptogenic cirrhosis. The aim of this study was to evaluate causes of liver disease in patients with unexplained intrahepatic portal hypertension. METHODS: We retrospectively analyzed cause of liver disease in all patients with cryptogenic intrahepatic portal hypertension who underwent liver biopsies between June 2005 to June 2007 in our center. RESULTS: Five hundred and seventeen patients underwent liver biopsies of whom 227 had portal hypertension. Of these, the cause of liver disease could not be detected prior to liver biopsy in 62 patients. Causes of liver disease identified after liver biopsy in these 62 patients were: idiopathic non-cirrhotic intrahepatic portal hypertension (NCIPH) (30 patients, 48%), cirrhosis (14), fatty liver disease (7) and other causes (11). Initial presentations in idiopathic NCIPH patients were splenomegaly and anemia (18 patients), variceal bleed (9) and ascites (3). Median age (range) of patients at first presentation was 32 (15-57) years, and 19 were male. Majority (90%) were in Child's class A. Hepatic vein pressure gradient was <5 mmHg in 2 of 7 NCIPH patients tested. CONCLUSIONS: We identified 30 patients with idiopathic NCIPH at our center over the 2 year study period. The clinical presentation and investigations of NCIPH closely mimic cryptogenic cirrhosis. Idiopathic NCIPH should be considered as a differential diagnosis of cryptogenic cirrhosis in India.