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April-June 2022 Volume 68 | Issue 2
Page Nos. 63-123
Online since Monday, April 25, 2022
Accessed 50,126 times.
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EDITORIAL |
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Blau syndrome: An under-reported condition in India?  |
p. 63 |
A Agarwal, S Karande DOI:10.4103/jpgm.jpgm_1016_21 PMID:35295039 |
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EDITORIAL COMMENTARIES |
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Glomerulonephritis registry: Need of the hour |
p. 68 |
T Jamale DOI:10.4103/jpgm.JPGM_343_21 PMID:35381753 |
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Tyrosine kinase domain mutations in chronic myelogenous leukemia patients: Indian perspective |
p. 70 |
K Ghosh DOI:10.4103/0022-3859.343150 PMID:35417998 |
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ORIGINAL ARTICLES |
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Who will guard the guardians? Cross-sectional study on prevalence of psychiatric morbidity, quality of life, and coping skills in caregivers of children with thalassemia major |
p. 72 |
AY Angane, KS Kadam, GS Ghorpade, VB Unnithan DOI:10.4103/jpgm.JPGM_1128_20 PMID:34708694
Background: Patients of thalassemia major require frequent hospitalization. Caregivers are more affected than the patient themselves as they better appreciate the magnitude of illness and treatment, resulting in increased risks for psychiatric illness.
Aims and Objectives: The purpose of the study was to assess the prevalence of psychiatric morbidity in the caregivers of patients with thalassemia major. The study also examined the quality of life of the caregivers, their coping strategies, and its association with sociodemographic variables.
Methodology: A cross-sectional study with 100 caregivers, recruited by convenience sampling technique, attending the thalassemia daycare center, was carried out over 12 months in a tertiary care hospital. They were administered a semistructured proforma along with General Health Questionnaire 12 (GHQ 12), WHO-Quality of Life-BREF (WHO-QOL-BREF), and Coping Inventory for Stressful Situations 21 scale. The GHQ 12 was used for screening and those scoring three or more underwent a clinical psychiatric interview. Those who were diagnosed with psychopathology were ascribed diagnosis as per ICD-10. Descriptive analysis was done. Associations were studied using Fischer's exact test. Comparison of quality of life with blood transfusion variables was done using Mann–Whitney U test.
Results: The prevalence of psychiatric morbidity amongst the caregivers was found to be 35% with depressive episode (22%) being the most common. Psychiatric morbidity was found to have a significant association with both, the frequency (P = 0.037) and total number of blood transfusions (P = 0.012). Coping was found to have a strong association with psychiatric morbidity (P = 0.001) and employment (P = 0.009).
Conclusions: Caregivers of children with thalassemia major face psychological burden like depression or anxiety, for which treatment is not sought. Improved psychological health of the caregivers will ensure better care of the child and guarantee better adherence to the treatment.
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Short term outcome of patients attending a renal-immunology clinic in central India |
p. 78 |
M Atlani, NK Kapoor, D Joshi, R Sutar, T Sharma, A Joshi DOI:10.4103/jpgm.JPGM_1320_20 PMID:35259787
Background: Glomerular diseases (GDs) and other renal immunologic diseases are an important cause of morbidity and mortality. Providing a single point of service in collaboration with various specialists at a renal immunology clinic for such patients is not novel, but outcomes have not been reported. Here, we report the short-term outcome of Indian patients attending our clinic.
Methods: This single-center prospective cohort study enrolled biopsy-proven immunologically-mediated adults with renal diseases between April 2018 and December 2019, and followed them for six months. The primary end point for the analysis was an incidence of end-stage renal disease (ESRD) or loss of >50% estimated glomerular filtration rate (eGFR) and patient survival at six months. Secondary endpoints were the rate of complete or partial remission, and impact of demographic factors.
Results: Ninety two patients underwent renal biopsy for suspected immunological renal diseases. Fourteen (15.2%) cases were excluded for nonimmune etiologies, whereas 78 (84.7%) confirmed cases of immune etiology were included. Most common primary GD (n = 51) (93.5%) was membranous nephropathy (n = 20) (25.6%), whereas lupus nephritis was the most common (n = 8) (29.6%) secondary GD. Overall, 10 (12.8%) patients reached renal endpoint of ESRD or >50% fall in eGFR. Focal segmental glomerulosclerosis (FSGS) (27%) patients had worst renal outcome. Patient survival was 94.8%. Thirty patients (38.4%) achieved complete, whereas 24 each (30.7%) achieved partial remission and remained resistant to disease specific therapies, respectively. Univariate analysis identified hypertension, severity of hypertension, and resistance to achieve proteinuria remission as significantly associated (P < 0.001) factors with poor renal outcome.
Conclusions: The present study shows that short term renal outcome of Indian patients with renal immune diseases remains poor. FSGS remains the GD with the worst renal outcome. Hypertension, its severity, failure to achieve proteinuria remission were significantly associated with poor renal outcomes.
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Efficacy and safety of hydroxychloroquine for managing glycemia in type-2 diabetes: A systematic review and meta-analysis |
p. 85 |
D Dutta, R Jindal, D Mehta, M Kumar, M Sharma DOI:10.4103/jpgm.JPGM_301_21 PMID:35466661
Aims: No Cochrane meta-analysis with grading of evidence is available on use of hydroxychloroquine (HCQ) in type-2 diabetes (T2DM). This meta-analysis evaluated the efficacy and safety of HCQ in T2DM.
Methods: Electronic databases were searched using a Boolean search strategy: ((hydroxychloroquine) OR (chloroquine*)) AND ((diabetes) OR (“diabetes mellitus”) OR (glycemia) OR (glucose) OR (insulin)) for studies evaluating hydroxychloroquine for glycemic control in T2DM. The primary outcome was a change in glycated haemoglobin (HbA1c). The secondary outcomes were changes in other glycemic/lipid parameters and adverse effects.
Results: Data from 11 randomized controlled trials (RCTs) (3 having placebo as controls [passive controls] and 8 having anti-diabetes medications as controls [active controls]) involving 2,723 patients having a median follow-up of 24 weeks were analyzed. About 54.54% of the RCTs were of poor quality as evaluated by the Jadad scale. The performance bias and detection bias were at high risk in 63.64% of the RCTs. The HbA1c reduction with HCQ was marginally better compared to the active (mean differences [MD]-0.17% [95%, CI:-0.30–-0.04;P=0.009;I2=89%; very low certainty of evidence, VLCE]), and passive (MD-1.35% [95%CI:-2.10–-0.59;P=0.005;I2=74%]) controls. A reduction in fasting glucose (MD-16.63mg/dL[95%, CI: -25.99 – -7.28mg/dL;P<0.001;I2=97%;VLCE]) and post-prandial glucose [MD -8.41mg/dL (95%CI: -14.71 – -2.12mg/dL;P=0.009;I2=87%;VLCE]), appeared better with HCQ compared to active controls. The total adverse events (risk ratio [RR]0.93 [95% CI:0.68–1.28]; P=0.65;I2=66%) were not different with HCQ compared to the controls.
Conclusion: The routine use of HCQ in T2DM cannot be recommended based on the current evidence.
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BRIEF REPORT |
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Tyrosine kinase domain mutations in chronic myelogenous leukemia patients: A single center experience |
p. 93 |
KB Bommannan, S Naseem, J Binota, N Varma, P Malhotra, S Varma DOI:10.4103/jpgm.JPGM_781_20 PMID:34747876
Introduction: Despite the impressive responses achieved with tyrosine kinase inhibitor (TKI) therapy, treatment resistance develops in 16–33% of patients of chronic myelogenous leukemia (CML). Of the BCR-ABL1 dependent mechanisms, mutations in the tyrosine kinase domain (TKD) are the commonest cause of resistance. Material and Methods: Allele specific oligonucleotide - polymerase chain reaction (ASO-PCR) was done for testing the six common TKD mutations, T315I, G250E, E255K, M244V, M351T, and Y253F. Results and Conclusion: TKD mutation study was done on 83 patients. Of these 44 (53%) were positive for one or more mutations. On analyzing specific mutations, E255K was the commonest mutation seen in 24 (29%) cases, followed by T315I in 23(28%) cases. Y253F mutation was not seen in the present study sample. In the present cohort of 83 patients, 29 (35%) cases were positive for single mutation, 12 (14%) had two mutations and 3 (4%) had three mutations.
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RESEARCH LETTER |
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Esophageal squamous cell cancer in Plummer-Vinson syndrome: Is lichen planus a missing link? |
p. 98 |
P Mohan, M Munisamy, K Senthamizh Selvan, A Hamide DOI:10.4103/jpgm.jpgm_375_21 PMID:35417996
This retrospective study analyzed the occurrence of esophageal squamous cell cancer (ESCC) in a cohort of patients with Plummer-Vinson syndrome (PVS) and mucosal lichen planus (LP). ESCC occurred in 6.2% cases of PVS, more than half of whom had associated oral LP. Mucosal LP and PVS together may increase the predisposition to ESCC.
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GRAND ROUND CASE |
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Post-trabeculectomy early bleb localization mimicking a 'ring of steel' |
p. 100 |
S Choudhary, M Kaur, SS Pandav, S Kaushik DOI:10.4103/jpgm.jpgm_738_21 PMID:35322658
A 54-year-old-man underwent trabeculectomy with mitomycin-C (0.02%). At 4-week follow-up, there was an elevated, well-circumscribed bleb with raised intra-ocular pressure (IOP) of 32-mmHg, with a classic “ring of steel” appearance, typically seen in a tenon's cyst. A sub-tenon needle revision re-established aqueous flow. Two weeks later, the IOP was 8-mmHg without any anti-glaucoma medications. A diffuse well formed bleb was noted. Careful ocular examination helps to identify early post-operative localized bleb that may appear like a “ring of steel” but can be managed by needling, unlike a tenon's cyst, which would require more extensive intervention.
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ADR REPORT |
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Drug reaction with eosinophilia and systemic symptoms syndrome related to piperacillin-tazobactam use |
p. 102 |
M Bai, V Govindaraj, R Kottaisamy, N Vijayarangam DOI:10.4103/jpgm.JPGM_1226_20 PMID:35466662
Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a severe, idiosyncratic reaction to a drug which presents after a prolonged latency period. Although it most commonly occurs with aromatic anticonvulsants, antibiotics are also occasionally implicated. A 50-year-old male was admitted for left pyopneumothorax. He was started on intravenous piperacillin-tazobactam (Pip/Taz) and clindamycin. After 10 days of treatment, he developed high grade fever with maculopapular rashes with areas of scaling. He had elevated WBC counts with eosinophils of 21% and raised serum transaminases. After excluding other possible etiologies for febrile illness, a possibility of DRESS was considered. Naranjo scale, used for causality assessment, yielded a total score of 6, pointing toward probable adverse drug reaction. Also, the patient had 6 out of the 7 inclusion criteria for DRESS as per European Registry of Severe Cutaneous Adverse Reaction (RegiScar) scoring. Pip/Taz was found to be causative drug and was discontinued. He was conservatively managed with antipyretics and topical steroids. Fever subsided the day after stopping Pip/Taz and his rashes resolved gradually. In conclusion the possibility of antibiotics-induced DRESS should be considered and high index of vigilance is advised.
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CASE REPORTS |
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Villous adenoma of the prostatic urethra |
p. 106 |
SY Ho, N Rosli, LY Lim DOI:10.4103/jpgm.jpgm_811_21 PMID:35381754
Primary villous adenoma originating from the urinary tract is an infrequent entity. We present a rare case of villous adenoma arising from a prostatic urethra with no sign of malignant transformation. Villous adenoma should be considered as one of the differential diagnoses of urethral lesions, especially if it has similar magnetic resonance imaging features as its colonic counterpart. Due to its potential for malignant transformation, its complete resection is mandatory.
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Hypoglycemic hemiparesis as stroke mimic with transient splenial lesion and internal capsule involvement: A reversible clinico-radiological concurrence |
p. 109 |
M Sharma, B Menon, G Manam DOI:10.4103/jpgm.jpgm_817_21 PMID:35466663
Hypoglycemia presents with a spectrum of neurological manifestations ranging from lightheadedness to confusion and coma. We report here the case of a 61-year-old woman with right hemiparesis presenting within the window period for stroke thrombolysis. MRI brain showed diffusion restriction in posterior limb of left internal capsule and splenium. Patient had documented hypoglycemia of 38 mg/dL. Patient's hemiparesis resolved after glucose correction, and radiological findings completely resolved after 10 days. We present this case to highlight the rare radiological finding of simultaneous internal capsule and splenium involvement in hypoglycemic hemiparesis and the importance to rule out stroke mimics to avoid unwanted thrombolysis.
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Sialadenoma papilliferum of a minor salivary gland with transformation to mucoepidermoid carcinoma |
p. 112 |
N Sood, B Arya, R Gupta, S Gupta DOI:10.4103/jpgm.jpgm_912_21 PMID:35381752
Sialadenoma papilliferum (SP) is a rare benign tumor usually occurring in the minor salivary glands. Only two cases of malignant transformation of SP and an occasional case of dysplasia in SP have been reported in the literature till now. We report a case of SP of a minor salivary gland in a middle-aged male where the exophytic component retained the histomorphological features of SP while the endophytic component displayed characteristics of a mucoepidermoid carcinoma. Malignant change in sialadenoma papilliferum is an extremely rare phenomenon that requires further research and documentation. Careful evaluation of morphologic features and reporting of more such cases is required to delineate the clinical behavior of this unusual tumor.
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CASE SNIPPETS |
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Long standing untreated multifocal cutaneous tuberculosis with development of breast carcinoma |
p. 115 |
S Srihari, P S S Ranugha, V Shastry, JB Betkerur DOI:10.4103/jpgm.JPGM_144_21 PMID:35417995 |
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Ascending aortic dissecting aneurysm with rupture into the right atrium associated with COVID-19 |
p. 117 |
D Kamanakeri, A Anoop, S Harikrishnan, A Gopalakrishnan DOI:10.4103/jpgm.jpgm_903_21 PMID:35417997 |
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Aortic aneurysm causing compressive myelopathy |
p. 120 |
SK Bhoi, S Naik DOI:10.4103/jpgm.jpgm_409_21 PMID:35418000 |
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Splenodiaphragmatic interposition of the splenic flexure of colon |
p. 122 |
VK S Nalamolu, A Chatterjee DOI:10.4103/jpgm.jpgm_2_22 PMID:35466664 |
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