Dyskeratosis Congenita Associated Non-Specific Interstitial Pneumonia

Dyskeratosis Congenita (DC) is a rare inherited disorder of ectodermal dysplasia. It consists of a classical mucocutaneous triad of abnormal skin pigmentation, nail dystrophy and leukoplakia. Pulmonary disease is seen in 10-15%. It is characterized by Idiopathic Pulmonary Fibrosis (IPF), or Idiopathic Familial Pulmonary Fibrosis (IFPF). Non-specific Interstitial Pneumonia (NSIP) has been reported rarely in children with DC and in an isolated adult patient. Our patient had classical clinical presentation of DC with pancytopenia and portal hypertension and clinic-radiological features of NSIP which is a rare association

Prevalence of Pre-Extensively Drug-Resistant Tuberculosis (Pre XDR-TB) and Extensively Drug-Resistant Tuberculosis (XDR-TB) among Pulmonary Multidrug Resistant Tuberculosis (MDR-TB) at a Tertiary Care Center in Mumbai

Background: India is a high burden country for Tuberculosis (TB). As per the World Health Organization (WHO) statistics, 24000 cases of Multi Drug Resistant (MDR) TB were diagnosed in India in 2014. MDR-TB patients consist of a heterogeneous cohort and management has its challenges. Aims and objectives: We studied the prevalence of PreExtensively Drug Resistant TB (Pre XDR-TB) and Extensively Drug Resistant TB (XDR-TB) among patients of pulmonary MDR-TB not previously exposed to second-line anti-tuberculous drugs and having baseline second-line Drug Susceptibility Testing (DST) against Fluoroquinolones (FQ) and Aminoglycosides (AM). Results: We included 227 patients. On the basis of the DST, patients were grouped into- 1) MDR-TB, 2) MDR-TB with FQ resistance {Pre XDR-TB (FQ)}, 3) MDR-TB with AM resistance {Pre XDR-TB (AM)} 4) XDR-TB. Of the 227 patients, 89 (39.2%) had MDR-TB, 127 (55.94%) had Pre XDR-TB (FQ), none had Pre XDR-TB (AM) and 11 (4.86%) had XDR-TB. Nine (4%) patients were human immunodeficiency (HIV) infected and 25(11%) had Diabetes Mellitus (DM). Conclusion: This study highlights the importance of baseline DST to FQ and AM in patients of diagnosed or suspected MDR-TB. We encountered a higher prevalence of Pre XDR-TB (FQ) which of concern in management of MDR-TB

TNMC-GAP (The New Modified Classification of Gender Age Physiology)- A novel risk assessment system in idiopathic pulmonary fibrosis

Background: Idiopathic pulmonary fibrosis (IPF) is a progressive idiopathic interstitial pneumonia. Gender-Age-Physiology (GAP) is the first prognostic staging system for IPF which predicts mortality. Disadvantage is the cumbersome diffusion lung capacity for carbon monoxide (DLCO) testing, one of the parameters in calculating GAP. Hence, we proposed The New Modified Classification of GAP (TNMC-GAP) index by replacing the DLCO parameter with the easy to perform and established prognostic test; 6 min-walk distance (6MWD). Aim: The aim of the study was to apply the TNMC-GAP index and compare with GAP index in IPF. Settings and Design: A retrospective observational study conducted at a tertiary care center after Ethics Committee permission. It was an independent subanalysis of IPF patient's data collected for National ILD India Registry. Statistical Analysis: The statistical analysis used mean, percentages, Chi-square test, and paired-t-test. Results: Seventy IPF (34 men and 36 women) patients were included in the study. Mean age was 62 years. Cough and dyspnea were the predominant symptoms. Most patients had comorbidities. Average partial pressure of oxygen on arterial blood gas (PaO2), forced vital capacity, and 6MWD were 68 mmHg, 1.42 L, 229 m, respectively. Patients, when classified as per GAP index, were distributed as 11, 26, and 33 against 16, 25, and 29 on applying the TNMC-GAP index in stages I, II, and III, respectively. At 3-year follow-up, 21 (30%) patients lived and 49 (70%) patients died. Of the deaths, 30 (43%), 42 (60%), and 49 (70%) had occurred at 1-, 2-, 3-year follow-up, respectively. Mortality prediction with TNMC-GAP and GAP yielded concordant results. We observed overall higher mortality. Cardiac comorbidity (P = 0.03), 6MWD <200 m (P = 0.0002), severe restriction (P = 0.0142), and moderate-to-severe pulmonary hypertension (P = 0.0068) were significant contributing factors for the same. Conclusion: The replacement of the DLCO parameter in the GAP with 6MWD yielded concordant results. Thus, TNMC-GAP serves a reliable replacement for GAP in resource-limited settings

A perplexing primary novel hilar lesion: Remember the pneumonic PPNHL!

A 60-year-old female presented with dyspnea, cough, and chest pain with a left hilar mass lesion. In our case, clinicoradiological correlation, bronchoscopy, and computed tomography-guided biopsy revealed the diagnosis of primary pulmonary non-Hodgkin's lymphoma (PPNHL) on histopathology and immunohistochemistry. We discuss the approach to hilar masses. PPNHL is a rare malignant lymphoma most common being mucosa-associated lymphoid tissue lymphoma. Various therapeutic options are available. The chemotherapy regimen consisting of CHOP (cyclophosphamide, doxorubicin, vincristine, prednisolone) is preferred

A retrospective observational study of liver abscess in children at tertiary care hospital of Ahmedabad city

In developing country like India, liver abscess in children is mostly pyogenic (PLA) in nature. This article aimed to analyze detail parameters of LA of admitted cases in tertiary care hospital of Ahmedabad city. Details of 32 patients From1 month to 14 years age groups were assessed retrospectively over periods of 2 years. The demographic profile, clinical presentation, laboratory and ultra sonographic, microbiological profile, management and outcome of patients were retrieved from case records. All cases had PLA with mean age 6 years and male predominance (64.28%). Under nutrition and enteric fever were only risk factors derived. Fever (93.75%) and abdominal pain (68.75%) were commonest presentation of LA. Leukocytosis found in 62.5% cases with neutrophilic predominance. Majority of LA were single (90.6%), in right lobe of liver (68.8%) with more than 50mm in size. Positivity rate of pus and blood culture in 12.5% and 15.62% cases respectively where staphylococcus aureus is commonest organism.17 cases (53.12%) require antibiotics treatment alone ,14 cases (43.75%) USG guided needler aspiration and 1 case (3%) open surgical drainage with 100%recovery rate. PLA is commonest in children and cryptogenic in origin. Management of LA required multidisciplinary approached include antibiotic and/or USG guided percutaneous needle aspiration