Alagille syndrome: Experience of a tertiary care center in North India

Alagille syndrome (AGS) is an autosomal dominant disorder of chronic cholestasis characterized by paucity of interlobular bile ducts. The condition has been described only as isolated case reports in India. We describe clinical profile and outcome of nine subjects (six infants and three older children) with AGS. Cholestasis and characteristic facies were present in all, followed by congenital heart disease, vertebral anomalies, and posterior embryotoxon in seven, five, and four cases, respectively. Pruritus was the commonest symptom which was refractory to medical treatment in one third of cases. Two cases developed decompensated liver disease on follow up. High index of suspicion for this multisystemic condition is essential for correct diagnosis and management.     

Clinico-hematological profile of hereditary spherocytosis: experience from a tertiary care center in North India

Abstract

Hereditary spherocytosis (HS) is an inherited membranopathy characterized by phenotypic and genotypic heterogeneity. This study describes the clinico-hematological profile of 70 HS patients diagnosed at a tertiary care center in North India over a period of five years. Patients commonly presented with intermittent jaundice (82·9%), pallor (80%) and dark colored urine (11·4%). The common signs were splenomegaly (92·9%), hepatomegaly (50%), cholelithiasis or choledocholithiasis (36·8%) and hemolytic facies (10%). Family history was contributory in 28·6% patients. Blood transfusion (BT) requirement was present in 35·7% patients. Unconjugated and conjugated hyperbilirubinemia was seen in 89·1 and 10·9% patients respectively. At presentation, the hemoglobin ranged from 3–14 g/dl with a mean of 9·37 g/dl (SD2·43). Spherocytes were seen in 88·6% and incubated Osmotic fragility test (OFT) was positive in 88·2% patients. The Eosin-5-maleimide (EMA) flow cytometric test was done in 28 patients. Mean fluorescence intensiy (MFI) for normal subjects was 11861·5 (SD-883·51) and for confirmed HS patients was 7949·3 (SD1304·1). Taking the MFI range of 5341·1–10 557·5 for HS, eight cases of suspected HS/undiagnosed hemolytic anemia with a negative (n=5) or equivocal (n=3) incubated OFT were diagnosed as HS. An increase in HbF level was seen in 10 cases ranging from 2·1 to 17·7% with a mean of 5·66%, three of these had associated β thalassaemia trait. Twelve patients (17%) underwent splenectomy and 91% of them did not require any BT post-splenectomy. Among the patients treated conservatively 49% had persisting pallor and 16·3% had transfusion requirement.     

Dropout from care among HIV-infected patients enrolled in care at a tertiary HIV care center in Chennai,India

Long-term follow-up of persons infected with HIV infection is essential to optimize clinical outcomes. However, limited data exist on the rates of dropout (DO) from HIV care and factors associated with DO especially from resource-limited settings. We conducted a retrospective analysis of the data available at YRGCARE, a private HIV care provider in south India that has registered over 15,000 HIV-infected persons since its inception in 1993. We included 7995 patients who registered for care between 1 January 2004 and 31 December 2009. A dropout was defined as a person who registered for care during this period and had not been seen in the clinic for >1 year. Logistic regression was used to examine factors associated with DO from clinical care. The median age of the patients registered for care was 34 years; 66% were male and 83% were married. The overall DO rate was 38.1 per 100 person-years – the majority of the DOs occurred within 6 months from registration. In multivariate analyses, patients who were enrolled in clinical studies/projects entitling them to free medications and retention staff (Odds Ratio [OR]: 0.65) or were on antiretroviral therapy (ART; OR: 0.37) or had a CD4 > 350 at the last visit (OR: 0.20) were significantly less likely to DO from clinical care. We observed a high rate of DO from clinical care at this tertiary HIV clinic in Chennai, India. Making ART available free of charge in the private sector and providing incentives/benefits for attending clinic visits as is routinely done in clinical trials might help improve retention.     

Corynebacterium jeikeium bacteremia at a tertiary care center

Summary During a six-year period 23 patients with isolation ofCorynebacterium jeikeium (formerly known asCorynebacterium group JK) from one or more blood cultures at a university hospital were identified. Cases occurred sporadically without time- or ward-related clustering. Review of the cases showed that most infections were nosocomial, that most of the patients had underlying malignant disease, had a chronic intravascular catheter implanted, had been pretreated with antibiotics, and were neutropenic at the time the blood cultures were drawn. Patients with only one versus those with more than one positive blood culture differed in some important aspects. Patients with only one positive blood culture were less likely to have acute leukemia, had significantly higher neutrophil counts and a shorter duration of preceding antibiotic treatment, and all had other probable causes of infection and fever. The mortality also appeared to be lower in these patients. Despite the possibility of increasing frequency of blood cultures positive forC. jeikeium, severe infections due to this organism continue to be largely confined to neutropenic patients with hematologic malignancy.

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Corynebacterium jeikeium-Bakteriämien an einem Universitätskrankenhaus

Zusammenfassung In einer Universitätsklinik wurde während eines Zeitraumes von sechs Jahren bei 23 PatientenCorynebacterium jeikeium aus Blutkulturen isoliert.C. jeikeium-Bakteriämien traten weder zeitlich noch örtlich gehäuft auf. Die retrospektive Analyse der Patientendaten zeigte, daß in den meisten Fällen eine maligne hämatologische Grunderkrankung mit Neutropenie vorlag, ein zentral-venöser Katheter implantiert und eine antibiotische Therapie vorausgegangen war. Die Patientenpopulation mit nur einer positiven Blutkultur unterschied sich in wichtigen Aspekten von derjenigen mit mehreren positiven Blutkulturen. So waren Patienten mit nur einer positiven Blutkultur seltener an akuter Leukämie erkrankt, hatten signifikant höhere neutrophile Granulozytenkonzentrationen im peripheren Blut und waren zuvor über einen kürzeren Zeitraum antibiotisch behandelt worden. Weiterhin fanden sich in dieser Patientengruppe in allen Fällen andere mögliche Ursachen der Fieberepisoden. Auch die Mortalität war deutlich geringer. Trotz des zunehmend häufigeren Nachweises vonCorynebacterium jeikeium in Blutkulturen bleiben schwere Infektionen durch diesen Erreger weiterhin meist auf die Patientenpopulation mit hämatologischer Grunderkrankung und Neutropenie beschränkt.

     

Clinical profile of brucellosis from a tertiary care center in southern India

ObjectiveTo highlight the spectrum of clinical manifestations, labs, complications, treatment and outcome of brucellosis.MethodsRetrospective study was conducted in Kasturba Medical College, Manipal University, Karnataka, India which included 68 confirmed cases of brucellosis from January 2006- April 2010. Diagnosis of brucellosis was made by culturing the sera/body fluids by standard BACTEC method (or) by testing the sera for Brucella agglutinins using the standard agglutination test (SAT). A titer of 1:320 or more was considered as significant. SPSS 16 was used for statistical analysis and Microsoft Excel for graphical representation.ResultsOf the 68 patients, 46 (68%) were male and 22 (32%) were female patients with age distribution of 9–75 years. Forty four (64.7%) had history of contact with unpasteurized dairy products or infected animals. Symptoms included fever (68, 100%), myalgia (21, 31%), musculoskeletal symptoms (23, 34%), headache (16, 24%), gastrointestinal symptoms (19, 28%) and altered sensorium (3, 4%). Co-morbidities and associations included HIV positivity (2, 3%), type 2 diabetes mellitus (13, 19%), steroid therapy (3, 4%) and HBsAg positivity (8, 12%). Ten (15%) patients had cervical lymphadenopathy, 4(6%) had splenic enlargement, 6 (7%) had hepatomegaly, 19 (28%) had hepatosplenomegaly and 2(3%) got meningeal signs. Anaemia was observed in 39 (57.3%) cases, high erythrocyte sedimentation rate (ESR) was present in 55 (80.8%) cases, leucocytosis in 10(14.7%), leucopenia in 10(14.7%), thrombocytopenia in 23 (33.82%) and thrombocytosis in 2 (2.94%) cases.ConclusionsIn countries like India, where brucellosis and tuberculosis are endemic; rapid, sensitive and highly specific diagnostic methods are required to make early diagnosis and prevent resistance as there is an overlap in therapy.     

Ecology of blood stream infection and antibiotic resistance in intensive care unit at a tertiary care hospital in North India

ObjectiveTo analyse the prevalent microorganisms and their antimicrobial resistance among intensive care unit patients in a tertiary care centre in New Delhi.MethodsA retrospective study of all consecutive blood cultures from various intensive care unit patients in the hospital during four years (January 2008 to December 2011). Antibiotic consumption data in the intensive care units were also analysed during the same period.ResultsOut of the total 22,491 blood cultures processed, 2846 samples were positive and 3771 microorganisms were isolated. The blood culture positivity was estimated as 12.7% of which 67.5% were monomicrobial and 32.5% polymicrobial infections. Gram negative bacilli, Gram positive cocci, and fungi were isolated in 49%, 33%, and 18% cases, respectively. Coagulase negative staphylococcus was the commonest single isolate followed by Candida spp. A drastic shift in the distribution of Candida spp. towards nonalbicans along with high resistance to azole group of antifungals suggest echinocandins for the empiric therapy of candidemia. High penicillin resistance in Gram positive isolates suggest vancomycin, linezolid and tigecycline as the options for empiric therapy, whereas tigecycline and colistin are the only options remaining for highly resistant Gram negative isolates. Aminoglycosides were observed to have better sensitivity and reduced usage when compared with cephalosporins and β-lactam + β-lactam inhibitor combinations.ConclusionsHigh frequencies of multidrug resistant organisms were observed in intensive care units which is a warning as to use the only few effective antimicrobials wisely to reduce selective pressure on sensitive strains.     

Current status of inpatient HIV care at a tertiary care center in Nepal

The success of the WHO's '3 by 5' programme will depend on the ability of developing countries to provide antiretroviral therapy. The purpose of this study was to determine the current status of HIV care at a major teaching hospital in eastern Nepal. A retrospective cohort study was conducted of admitted HIV-infected patients at BPKIHS between 1993 and 2003, evaluating reasons for admission and the care received. There were 2, 2, 2, 3, 5 and 18 admissions, respectively, from 1998 to 2003. Only 4 were female patients. Two were children, both admitted in the last y studied. 10 admissions (31% of the total) were for opportunistic infections (OIs). Seven patients were prescribed OI prophylaxis, with incorrect dosages in 6. Four patients were prescribed antiretroviral therapy, all in inappropriate dosages or combinations. 13 patients were rapidly discharged without further care as soon as a diagnosis of HIV was made. Hospital admissions of HIV-infected individuals are increasing rapidly. Important segments of the HIV-infected population such as women and children are not receiving medical services. Most admissions are not due to OIs. The care of HIV-infected patients even at a major tertiary care teaching hospital in Nepal is sub-optimal.     

Clinicoradiological profile of interstitial lung diseases at a tertiary care center in Northern India: An eight-year study

Background and objectivesInterstitial lung diseases (ILDs) are a varied group of disorders that have been clubbed together due to some common attributes. The data is abundant from the developed world. However, the developing countries have gradually amassed the knowledge of these disorders and epidemiological data is still missing. We aimed to profile the ILD patients at our center.MethodsAll ILD patients above the age of 14 years were included in the study. A detailed history, examination, exercise capacity assessment, pulmonary function test and relevant serological investigations were done. Tissue was obtained wherever possible. Diagnosis was made after discussion from concerned specialists.ResultsAmong the total 884 subjects recruited, 54% were females with a mean age of 53 years. Most were residents of rural areas. Reticulations and traction bronchiectasis was seen in most subjects. Hypersensitivity pneumonitis was present in 35.9% and connective-tissue disease related ILD (CTD-ILD) in 30.9%.ConclusionMost common ILDs at our center are hypersensitivity pneumonitis and CTD-ILD. Reticulations and traction bronchiectasis are the most common findings on HRCT thorax.     

Clinical profile of insulinoma: analysis from a tertiary care referral center in India

Objective  

To describe the clinical presentation, localization techniques, surgical procedures and outcome in patients with insulinoma.     

Caspofungin for invasive candidiasis at a tertiary care medical center

Background

Caspofungin is emerging as first-line therapy for invasive candidiasis. Data on the use of caspofungin for treatment for invasive candidiasis are limited to clinical trials and case reports. We report a single-center experience with 104 consecutive courses of caspofungin for the treatment of invasive candidiasis to evaluate a real-world performance of this drug.

Methods

A retrospective chart review of patients receiving caspofungin at a tertiary care medical center was performed. Patient information and microbiologic data were abstracted from patient charts and electronic medical records.

Results

Of 241 patients receiving caspofungin for all indications, 122 (51%) had proven invasive candidiasis. There were 104 treatment courses for candidiasis in 99 patients available for review. Bloodstream (66%) and abdominal infections (25%) were the most common sites of infection. Most infections were non-albicans (80/104, 77%; including patients infected with more than one species). Clinical cure rates at the end of therapy were 83% (57/69) for bloodstream infections and 84% (22/26) for abdominal infections. Caspofungin did not clear candidiasis in 14 episodes (microbiologic cure rate 75%, 42/56).

Conclusions

The clinical use of caspofungin has been successful in the treatment of invasive candidiasis, even in patients with prior antifungal exposure. In this unselected review, caspofungin performed similarly as in clinical trials, and clinicians should consider caspofungin as first-line therapy for invasive candidiasis, particularly non-albicans species.     

Mortality in diabetes: a retrospective analysis from a tertiary care hospital in North India

Cause(s) of death in patients with diabetes mellitus (DM) admitted to a tertiary care hospital in North India was analysed from classified information in patients' death records and case file. Of the 306652 total admissions from 1991 to 1999, 21584 patients died (7.04%), 592 of whom (355 men and 237 women) had DM (2.7%). Information from 92 patients with diabetes could not be retrieved and six patients with hyperglycemia of short duration (two with gestational DM, three with post-transplant diabetes and one with stress hyperglycemia) were excluded. Of the 494 patients, 456 (92.3%) had T2DM and 38 (7.7%) had T1DM. Four hundred and forty patients had diabetes related mortality: infections (230, 46.5%), cardiovascular events (86, 17.4%), chronic renal failure (CRF) (48, 9.7%), stroke (30, 6%), diabetic ketoacidosis (DKA) (15, 3%), hyperosmolar coma (11, 2.2%), and hypoglycemia (5, 1%), while others (54 patients) had diabetes unrelated deaths. Cause of death could not be ascertained in six patients (1.2%). Death was attributed to a single cause in 301 (60.9%), to two causes in 175 (35.4%) and to three or more causes in 12 (2.4%) patients. Analysis of the cause of death in DM versus hospital in-patients in general, showed infection (P<0.02), coronary artery disease (CAD) (P<0.001), and CRF (P<0.001) to be more frequent in diabetes.     

Angiographic profile and outcomes in persistent non-valvular atrial fibrillation: A study from tertiary care center in North India

BackgroundThe relationship of atrial fibrillation (AF) with coronary artery disease (CAD) is well established, yet it is often missed. There is evidence of myocardial ischemia on stress imaging in AF patients in the absence of obstructive CAD. In this prospective cohort, we studied the angiographic profiles of non-valvular AF patients.MethodsThe study was a nonrandomized, prospective, single-center observational study of consecutive patients of persistent non-valvular AF. Patients symptomatic for AF despite optimal medical therapy for 3 months were recruited and all underwent coronary angiograms (CAG). Patients with prior history of CAD were excluded.ResultsA total of 70 patients were followed for a mean duration of 12 ± 1.4 months. The mean age of the study group was 66.07 (±11.49) years. Hypertension was the commonest comorbidity seen in 74% patients. Obstructive CAD was present in 32 (46%) patients, non-obstructive (<50% stenosis) CAD in 17 (24%) patients and normal coronaries in 21 (30%) patients. Overall 49 (70%) patients had evidence of CAD. Amongst patients without obstructive CAD, slow flow was seen in 16 (42%) patients. Lower baseline ejection fraction, lower haemoglobin & albumin levels and higher creatinine levels was associated with increased mortality. In patients without obstructive CAD, hospitalizations for fast ventricular rate were significantly increased in those having slow flow on CAG (p = 0.005).ConclusionsMajority (70%) of our patients had evidence of atherosclerotic CAD on CAG. A large proportion of patients without obstructive CAD had slow flow on CAG.     

Prevalence of celiac disease in nutritional anemia at a tertiary care center

Background

While anemia occurs in 80 % to 90 % of patients with celiac disease (CD), it may be the sole manifestation of CD. The prevalence of CD in Indian patients with nutritional anemia is not known.

Patients and Methods

Adolescent and adult patients presenting with nutritional anemia were prospectively screened for CD using IgA anti-tissue transglutaminase antibody (anti-tTG Ab) followed, if positive, by upper gastrointestinal endoscopy and duodenal biopsy.

Results

Ninety-six patients [mean?±?SD age 32.1?±?13.1 years and median duration of anemia 11 months (range 1 to 144 months)] were screened. Of these patients, 80 had iron deficiency anemia, 11 had megaloblastic anemia, and 5 had dimorphic anemia. Seventy-three patients were on hematinics and 36.4 % had received blood transfusions. Nineteen had a history of chronic diarrhea and the mean?±?SD duration of diarrhea in them was 9.7?±?35.8 months. IgA anti-tTG Ab was positive in 13 patients, of whom 12 agreed to undergo duodenal biopsy. Ten patients had villous atrophy (Marsh grade 3a in three, 3b in one, and 3c in six) and two did not. Thus, 10 patients with nutritional anemia (iron deficiency 9, vitamin B₁₂ deficiency 1) were diagnosed to have CD. On multivariate logistic regression, age, duration of symptoms, and presence of diarrhea were found to be the predictors of CD. All the patients with CD were put on gluten-free diet and with iron and vitamin supplementations and showed a significant improvement in hemoglobin concentration.

Conclusions

CD screening should be included in the work up of otherwise unexplained nutritional anemia.     

Survey of isolation practices at a tertiary care pediatric hospital

BACKGROUND: Although isolation precautions are an important aspect of hospital infection control, current rates of isolation in a pediatric hospital and rates of compliance with established precautions are unknown. We therefore initiated hospital-wide point prevalence studies to determine unit-specific rates of patient isolation and compliance with proper isolation requirements focusing on communication of isolation status and availability of personal protective equipment. In this report, we present data from the first 14 months of the study. METHODS: This was a prospective observational study. Twice monthly, between January 2004 and February 2005, infection control professionals reviewed the types and appropriateness of isolation of all hospitalized patients, except for those on the psychiatry unit. RESULTS: Seventeen percent of patients in the hospital during the study period were isolated, most frequently for community-acquired infections. Droplet isolation precautions were the most common form of isolation. Overall, only 74.6% of patients were isolated appropriately. The solid organ transplantation, hematology/oncology, and bone marrow transplantation units were those with the highest rates of inappropriate isolation. CONCLUSION: At our hospital, community-acquired infections, in particular respiratory infections, were the most common reasons for patient isolation. Monitoring of the appropriateness of isolation precautions offers the opportunity to reduce health care-related transmission of infection and identify specific target areas for improvement.     

Incidence of hepatocellular carcinoma among Indian patients with cirrhosis of liver: an experience from a tertiary care center in northern India.

BACKGROUND/AIM: Despite bearing the main burden of HCC, prospective studies from developing countries are lacking. This prospective observational study was designed to estimate the incidence of HCC among Indian patients with hepatic cirrhosis. METHODS: Between April 2001 and November 2004, we enrolled 301 patients with liver cirrhosis. Patients found to be free of HCC using baseline abdominal ultrasound, triple-phase computed tomography (TPCT) and serum alpha-fetoprotein (AFP) levels were followed up prospectively for detection of HCC using ultrasound and AFP every 6 months, and TPCT annually. RESULTS: Among the 194 patients (mean age [SD] 45.1 [+/-13.1] years; male:female 6.1:1.0) followed up, 154 had Child's A and 40 had Child's B disease. The causes of cirrhosis were: hepatitis B-71 (36.6%), hepatitis C-54 (27.8%), dual infection with hepatitis B and C-12 (6.2%) and others including autoimmune, alcoholic and cryptogenic cirrhosis 57 (29.4%). During a cumulative follow up period of 563.4 person-years, 9 cases of HCC were detected, with an incidence rate of 1.60 per 100 person-years. CONCLUSION: In our study, the incidence of HCC among patients with liver cirrhosis was intermediate, being lower than that in Japan but higher than that reported from Europe.