Calciphylaxis: An Unusual and Silent Usher for an Underlying Carcinoma.

Calciphylaxis is an uncommon but serious and potentially life-threatening painful condition that is characterized by cutaneous vascular calcification and ischemia. It is typically seen in patients with end-stage renal disease but has been known to occur less commonly in patients with normal renal function. A rare but established etiology of non-uremic calciphylaxis is malignancy. We have thus far been successful in documenting an association between calciphylaxis and certain solid tumor types. The objective of this clinical case study is to better define a possible association between calciphylaxis and underlying malignancy.

Frequency of iron deficiency anemia in type 2 diabetes - Insights from tertiary diabetes care centres across India.

AIM: To study the frequency of iron deficiency anemia (IDA) in individuals with type 2 diabetes mellitus (T2DM) seen at tertiary diabetes care centres across India. METHODS: This is a retrospective study (January 1, 2017-December 31, 2019), which included 1137 individuals with T2DM, aged ≥18 years, for whom data on glycemic, lipid and haematological parameters were available. Anthropometric measurements were done using standardized techniques. Biochemical investigations included fasting plasma glucose[FPG], post prandial plasma glucose, HbA1c, lipids and serum ferritin and iron wherever feasible. RESULTS: Of the 1137 individuals included for the study, 117 (10.3%) were categorized as no 'iron deficiency' (ID) group [normal hemoglobin: male ≥13 g/dl, female ≥12 g/dl and normal serum ferritin ≥70 µg/L], 123 (10.8%) as ID group [normal hemoglobin and low serum ferritin <70 µg/L)], 447 (39.3%) as IDA group [low haemoglobin: male <13 g/dl, female <12 g/dl and low serum ferritin] and 450 (39.6%) as 'anemia of chronic disease' (ACD) group [low hemoglobin and normal serum ferritin]. The percentage of women having ID (57.7%) and IDA (65.3%) was significantly higher than their male counterparts. ID was most prevalent (61.7%) in the individuals with duration of diabetes <5 years whereas ACD was most prevalent (50.5%) in individuals with long standing diabetes (>10 years). Independent risk factors for IDA were female gender (OR 3.3,95% CI:1.75-6.23, p < 0.001), duration of diabetes (OR 1.05, 95% CI 1.01-1.11, p = 0.028) and FPG (OR 1.01, 95% CI 0.99-1.00, p = 0.018). CONCLUSIONS: There is a need of identifying and monitoring iron status and anemia in patients with T2DM.

Management of Children and Adolescents with Wilson Disease and Neurological Worsening Following D-Penicillamine Therapy: A Single Centre Experience.

Background: Most centers in developing countries prefer chelation therapy with D-penicillamine for the management of Wilson's disease (WD) because of its easy availability and affordability. Neurological worsening following treatment with D-penicillamine is not uncommon. However, there is a paucity of Indian data on the incidence of neurological worsening in children and adolescents with WD following chelation therapy. Our study objectives were to identify the prevalence of neurological worsening in children and adolescents with WD following chelation with D-penicillamine therapy and to describe the management options and outcomes in these patients. Materials and Methods: In this retrospective chart review, children and adolescents with an established diagnosis of WD from 2010 to 2020 were identified from the hospital electronic database. Among these patients, data of children and adolescents with neurological worsening following D-penicillamine therapy were extracted and analyzed. Results: Neurological worsening was observed in 27/122 (22.1%) children and adolescents with WD on chelation therapy with D-penicillamine. Fifteen patients with neurological worsening following D-penicillamine therapy were managed with zinc monotherapy. Four patients were managed with a combination therapy of zinc and trientine. Five patients were treated with trientine monotherapy. Re-challenging with D-penicillamine at a lower dose followed by a slow dose escalation was attempted in three patients. Gradual clinical and functional status improvement was observed in 24 cases while one patient succumbed to pneumonia. Conclusion: Children and adolescents with WD who had neurological worsening on D-penicillamine therapy may be managed with trientine. Zinc monotherapy with copper restricted diet was also found to be effective in non-affordable patients.

Scrub Typhus: A Spatial and Temporal Analysis from South India.

Scrub typhus is a zoonotic rickettsial disease caused by the bacterium Orientia tsutsugamushi. The non-specificity of presentation, low index of suspicion and the poor availability of diagnostic tests often lead to delayed diagnosis and significant morbidity and mortality. Temperature, humidity, rainfall and Normalized Difference Vegetation Index (NDVI) on the spatio-temporal clustering of scrub typhus cases in children in three contiguous administrative districts in South India over 5 years were studied. A total of 419 children were diagnosed with scrub typhus during the study period. A surge of children with scrub typhus was noted when the NVDI ranged between 0.6 and 0.8 µm. Temperature, humidity and rainfall had a major role in the incidence of scrub typhus.

Pancreatic, hepatobiliary, and gastrointestinal manifestations of children with cystic fibrosis: A 10-year experience from a tertiary care center in southern India.

OBJECTIVES: To describe the demography and spectrum of pancreatic, hepatobiliary, and gastrointestinal (GI) manifestations in children with cystic fibrosis (CF) from the Indian subcontinent. METHODS: In this retrospective study, relevant information from the database of all children with CF below 18 years of age was collected and analyzed. RESULTS: Among the total 109 children, 58 (53%) were from the southern states of India. The most common manifestation was pancreatic insufficiency (PI) in 85 (83%) children. Those with PI presented at an earlier age (1.8 vs. 6.9 years). Cirrhosis with portal hypertension was documented in only one patient and meconium ileus in three (2.8%). There was significant malnutrition in the PI cohort with a mean weight-for-age Z-score of - 3.17 ± 1.79 at diagnosis. Twenty-one (19%) patients had died during the follow-up and 18 (90%) of them had PI. There was no difference in the prevalence of selected pulmonary manifestations in the PI and pancreatic sufficient (PS) groups. Among children with PI, 78 were screened for ΔF508 mutation, 16 (21%) were homozygous, and 17 (22%) were heterozygous. In the PS group, only 2 (14%) were heterozygous for ΔF508 mutation. The median duration of follow-up of the patients was 1.8 (1.5) years. CONCLUSION: PI is the most common GI manifestation of children with CF and is associated with severe malnutrition and poor outcome. Timely identification and management of the comorbidities involving the digestive system are essential for better growth and quality of life in these children.

Practice Patterns and Outcome of Extracorporeal Membrane Oxygenation Therapy for Severe Acute Respiratory Distress Syndrome in Indian ICUs.

INTRODUCTION: Extracorporeal membrane oxygenation (ECMO) is increasingly used in managing patients with severe acute respiratory distress syndrome (ARDS). The aim of the study is to describe the practice of ECMO and evaluate the outcomes in patients with severe ARDS in Indian intensive care units (ICUs). METHODS: Data of 39 patients with severe ARDS managed with ECMO in two tertiary centers between 2012 and 2018 were retrospectively studied. RESULTS: The mean age of the patient was 44.6 ± 13.5 years and 51% were female. Primary ARDS due to viral pneumonia was the common indication for ECMO. Mean APACHE II and Murray scores were 32.3 ± 7.8 and 3.64 ± 0.21, respectively. Prone ventilation and/or inhaled nitric oxide were used in 69.3% of the patients prior to ECMO therapy. Among 39 patients, 38 patients were managed with venovenous ECMO and 1 patient was managed with venoarterial ECMO. Average ECMO duration was 9.4 ± 6.9 days. Among the 17 (43.5%) patients successfully weaned off ECMO, 15 (38.5%) survived to discharge home. The average ICU and hospital length of stay were 18.9 ± 15.5 and 20.6 ± 16.6 days, respectively. While, sepsis was the common complication noted in 19 (49%) patients, bleeding and thrombotic complications were also noted in six and two patients, respectively. CONCLUSION: In conclusion, ECMO support was used as rescue therapy in severe ARDS with a survival rate of 39%. Sepsis was the common complication of ECMO followed by bleeding and thrombosis. HOW TO CITE THIS ARTICLE: Mariappan R, Kumar M, Ramakrishnan N, Mani AK, Kumar S, Chandrasekaran V. Practice Patterns and Outcome of Extracorporeal Membrane Oxygenation Therapy for Severe Acute Respiratory Distress Syndrome in Indian ICUs. Indian J Crit Care Med 2021;25(11):1263-1268.

Infant Dengue a 10-Year Experience from a Tertiary Center in South India.

Dengue remains a major problem in the tropics. Several Asian countries have reported an increasing trend in the proportion of infants with dengue fever. However, most studies are limited to case reports or small case series from isolated outbreaks. We planned this study to look at clinico-laboratory profile, outcome, and predictors of severity in a large cohort of infants over a decade. Electronic medical records of infants admitted at a tertiary center of South India, with laboratory confirmed dengue infection between 2009 and 2019 were reviewed. Diagnosis was based on detection of NS-1 antigen and/or immunoglobulin M antibody against DENV(dengue virus) or positive DENV RNA polymerase chain reaction in infants presenting with acute febrile illness and clinical features consistent with dengue. Of 395 children with dengue admitted during study period, 99 (25%) were infants. A cyclical incidence pattern was noted, with higher cases in alternate years. Fever (99%) was most common, followed by gastrointestinal symptoms (vomiting, diarrhea-28%) and upper respiratory symptoms (cough, coryza-22%). Fifty-three infants had severe dengue, and 39 had shock. Fourteen children had multiorgan dysfunction syndrome, and 13 died. Infants with severe dengue were older than those with nonsevere disease, had lower serum albumin and greater frequency of severe thrombocytopenia, and had coagulopathy. On multivariable analysis, low serum albumin predicted development of severe dengue [P = 0.003, odds ratio 12.4 (95% confidence interval: 2.42-63.7)]. Dengue in infants may be challenging to recognize because of its undifferentiated presentation, with gastrointestinal and upper respiratory symptoms that are similar to other viral illness. Severe dengue is common in this sample, and lower serum albumin at presentation was predictive of severe disease.

Effect of malnutrition in infants with cystic fibrosis in India: An underestimated danger.

AIMS: To assess the clinical profile and nutritional status of infants with cystic fibrosis (CF) and track their nutritional outcomes with treatment. MATERIALS AND METHODS: This retrospective study was conducted in a tertiary-care institute in South India. Demographic and clinical information were collected. The nutritional status and treatment outcome was assessed by Z-scores for weight-for-age (WAZ), length-for-age (LAZ), and weight-for-length (WLZ) at diagnosis and follow-up. RESULTS: Nineteen infants with CF had mean follow-up duration of 9.7 ± 8.7 months. There was a mean delay of 2.9 ± 2.1 months from symptom onset to diagnosis, by which time infants were severely malnourished (mean WAZ -4.68 ± 1.8). Pneumonia, summer dehydration with electrolyte abnormalities (42.1%), and a combination of anemia, hypoalbuminemia, and malnutrition (42.1%) were the predominant features. Significant weight loss had been recorded in undiagnosed infants by second month of life before symptom onset. At follow-up, there was a remarkable improvement in WAZ (P 0.001), but not LAZ and WLZ. There was a high mortality rate of 37% in these infants. CONCLUSIONS: Malnutrition is a significant morbidity in infants with CF in India. There was significant improvement of WAZ with treatment, but it lagged behind the recommended targets. There is an opportunity for identification of CF infants at the time of vaccination at six and ten weeks of age, by the primary care physician and pediatrician. Screening of young infants having failure to thrive in the immunization clinic may be a strategy for early diagnosis of infants with severe CF phenotype.

Management of Shallow Vestibule with Reduced Attached Gingiva in Fixed Prosthetic Intervention.

Shallow vestibule has long been considered a deterring factor in the use of removable dental prosthetics. The need for management in fixed prosthetic replacement is not widely discussed. Adequate attached gingiva is essential for continued proper oral hygiene. Muscular and fibrous traction leads to gingival recession, which can cause marginal leakage in a fixed prosthetic restoration. In the long-term, this causes the suprastructure to fail and, ultimately, the restoration also fails. Therefore, shallow vestibule with reduced attached gingiva should be identified in the diagnostic stage and should be effectively managed prior to restoration of lost tooth structure. This case report discusses the management of a shallow vestibule in a LeFort I fracture with emphasis on a fixed prosthetic replacement.

Extracorporeal Membrane Oxygenation in Drug Overdose: A Clinical Case Series.

Overdose of cardiovascular medications such as beta blockers and calcium channel blockers cause impaired cardiac contractility, vasoplegia, and/or rhythm disturbances. In addition to conventional management of limiting absorption, increasing elimination and hemodynamic support intravenous (IV) calcium infusion, hyperinsulinemia-euglycemia therapy, glucagon infusion, and IV lipid emulsion have been tried. Extracorporeal circulatory assist device support has been reported as a rescue therapy in overdose refractory to maximal medical therapy. We report three patients with cardiovascular medication overdose presenting with profound cardiovascular instability refractory to medical therapy. Venoarterial extracorporeal membrane oxygenation support (VA ECMO) was initiated to provide hemodynamic support. Despite the occurrence of device-associated complications, the outcome was good and all patients survived. VA ECMO may be considered in patients with severe refractory shock due to cardiotoxic medication overdose.