Unilateral Presentation of Pulmonary Alveolar Proteinosis

Pulmonary alveolar proteinosis (PAP) is a rare disorder, in which lipoproteinaceous material accumulates within the alveoli. We report a case of a 27-year-old male patient with acute worsening of breathlessness over the last 7�months and cough with desaturation up to 79% on room air. Contrast-enhanced computerized tomography of the thorax revealed unilateral diffuse crazy-paving pattern likely PAP. Transbronchial lung biopsy confirmed the diagnosis of PAP. The present case highlights the unusual presentation of PAP with unilateral involvement. To the best of our knowledge, this is the first reported case of unilateral PAP from India with a biopsy diagnosis and resolution with whole lung lavage.

Racial disparities in incidence & survival of Kaposi's sarcoma in the United States

Background & objectives: In the United States (US), Kaposi's sarcoma (KS) is usually seen in the patients affected by human immunodeficiency virus (HIV). The racial differences in the incidence rates and survival of patients with KS have been reported in the US. We undertook this study to analyse the disparities in the race-specific incidence rate and survival of KS patients of two different races in the US based on SEER (Surveillance, Epidemiology and End Results) database. Methods: Data on KS patients of African-American (AA) and non-Hispanic White (NHW) races who were diagnosed during 1973-2013 were extracted from SEER database to estimate the incidence rates and survival of KS patients. Results: A total of 18,388 NHWs and 3,455 AAs were diagnosed with KS. The age-adjusted incidence rate (AAIR) of KS in patients aged 20-44 yr was 3.8 times higher in AAs than in NHWs. The decline in AAIR of KS among NHWs started during 1989-1994 and preceded decline in the AAIR of AAs. After introduction of highly active antiretroviral therapy (HAART), the incidence continued to decline, but the decrease in the AAIR in AAs [annual percentage change (APC): ?6.2; 95% confidence interval (CI): ?8.8 to ?3.5] was slower than that in NHWs (APC: ?10.9; 95% CI: ?12.6 to ?9.1). The hazard ratio for all-cause mortality in KS patients of the AA race increased from 1.1 (95% CI: 1-1.2) in 1981-1995 to 1.55 (95% CI: 1.4-1.7) in 1996-2013 as compared to those of the NHW race. Interpretation & conclusions: Several significant racial disparities that emerged after HAART introduction in the incidence and survival of KS patients continued to persist, despite improvement in care of patients with HIV. Further studies need to be done to find out the underlying factors leading to these disparities.

Evaluation of Surrogate Markers for Insulin Resistance for Defining Metabolic Syndrome in Urban Indian Adolescents.

Objective: To compare parameters of insulin resistance, with special reference to McAuley index, in urban Indian adolescents, and to establish their cut-off values for defining metabolic syndrome. Design: Cross-sectional study. Setting: Schools located in four different geographical zones of Delhi, India. Participants: 695 apparently healthy adolescents grouped as normal weight (298), overweight (205) and obese (192). Outcome measures: Cut-off point for indices of insulin resistance was assessed by fasting insulin, insulin glucose ratio, and other methods (HOMA model, QUICKI, McAuley index) to define metabolic syndrome. Results: The McAuley index increased progressively from normal weight to obese adolescents in both sexes. McAuley index was significantly lower in adolescents with metabolic syndrome (5.36 ± 1.28 vs. 7.05 ± 1.88; P<0.001). McAuley index had the highest area under curve of receiver operator characteristics [0.82 (0.02)] as compared to other indices of insulin resistance. McAuley index of 6.23 had the highest specificity (88%) with sensitivity of 63.3% for diagnosing metabolic syndrome, whereas insulin glucose ratio had the highest sensitivity (79.7%) but low (55.5%) specificity. McAuley index was negatively correlated with height (r= -0.257, P=<0.001), weight (r= -0.537, P=<0.001), body mass index (r= -0.579, P<0.001), waist circumference (r= -0.542, p<0.001), and waist hip ratio (r= -0.268, P<0.001). Conclusions: Among various parameters of insulin resistance, McAuley index had the highest specificity, and insulin glucose ratio had the highest sensitivity in diagnosing metabolic syndrome in urban Indian adolescents.

Idiopathic granulomatous mastitis: a great diagnostic challenge.

Idiopathic granulomatous mastitis is a rare chronic inflammatory lesion of breast which presents as a spectrum of diseases and is diagnosed only histological. It is often a diagnosis of exclusion in most of the cases. Medical and surgical treatments have been tried with varied success rates and till today no standard protocol could be made in its management though excision remains the treatment of choice in refractory cases which is associated with morbidity more due to psychological factors. In the present case, medical treatment was tried in the initial phase followed by wide excision.

Assessment of lexical semantic judgment abilities in alcohol-dependent subjects: An fMRI study.

Neuropsychological studies have shown that alcohol dependence is associated with neurocognitive deficits in tasks requiring memory, perceptual motor skills, abstraction and problem solving, whereas language skills are relatively spared in alcoholics despite structural abnormalities in the language-related brain regions. To investigate the preserved mechanisms of language processing in alcohol-dependents, functional brain imaging was undertaken in healthy controls (n=18) and alcohol-dependents (n=16) while completing a lexical semantic judgment task in a 3 T MR scanner. Behavioural data indicated that alcohol-dependents took more time than controls for performing the task but there was no significant difference in their response accuracy. fMRI data analysis revealed that while performing the task, the alcoholics showed enhanced activations in left supramarginal gyrus, precuneus bilaterally, left angular gyrus, and left middle temporal gyrus as compared to control subjects. The extensive activations observed in alcoholics as compared to controls suggest that alcoholics recruit additional brain areas to meet the behavioural demands for equivalent task performance. The results are consistent with previous fMRI studies suggesting compensatory mechanisms for the execution of task for showing an equivalent performance or decreased neural efficiency of relevant brain networks. However, on direct comparison of the two groups, the results did not survive correction for multiple comparisons; therefore, the present findings need further exploration.

Glutamic acid decarboxylase (anti-GAD) & tissue transglutaminase (anti-TTG) antibodies in patients with thyroid autoimmunity.

Background & objectives: Several autoimmune disorders have been reported to be associated with autoimmune thyroiditis and may coexist with other organ-specific autoantibodies. The aim of the present study was to evaluate the presence of tissue transglutaminase (anti-TTG) and glutamic acid decarboxylase (anti-GAD) antibodies in patients suffering from autoimmune thyroiditis as diagnosed by anti-thyroid peroxidase (anti-TPO) antibodies, which may indicate high risk for developing celiac disease or type 1 diabetes mellitus. Methods: Five thousand children and 2800 adults were screening as part of a general health examination done on a voluntary basis in four different parts of Delhi. A total of 577 subjects positive for anti-TPO antibody constituted the cases. Equal number of age and sex matched anti-TPO antibody negative controls were randomly selected from the same cohort to form paired case control study. The cases and controls were further divided into two groups as follows: group-1 (children and adolescent <18 yr), group-2 (adults >18 yr). Serum samples of cases and controls were analysed for thyroid function test (FT3, FT4, and TSH), anti-TTG and anti-GAD antibodies. Results: A total of 1154 subjects (577 cases and 577 controls) were included in this study. Hypothyroidism was present in 40.2 per cent (232) cases compared to only 4.7 per cent (27) in controls (P<0.001). Anti-TTG and anti-GAD antibodies were present in 6.9 and 12.5 per cent subjects among cases compared to 3.5 per cent (P=0.015) and 4.3 per cent (P=0.001) in controls, respectively. Only anti-GAD antibody were significantly positive in cases among children and adolescents (P =0.0044) and adult (P=0.001) compared to controls. Levels of anti-TTG and anti-GAD antibodies increased with increasing titre of anti-TPO antibody. Interpretation & conclusions: Our findings showed high positivity of anti-GAD and anti-TTG antibodies among subjects with thyroid autoimmunity. It is, therefore, important to have high clinical index of suspicion for celiac disease or type 1 diabetes mellitus in patients with autoimmune thyroiditis.

Histopathologic changes following neoadjuvant chemotherapy in locally advanced breast cancer.

Aims: To compare the clinical and pathologic assessment of response to neoadjuvant chemotherapy and describe the various histopathologic changes observed. Materials and Methods: We studied a group of 40 patients with locally advanced breast cancer who had their initial workup in the form of clinico-imaging assessment of the size and pretreatment biopsy from the lesion. All the patients received two to six cycles of neoadjuvant chemotherapy, either cyclophosphamide 50 to 60 mg/m 2 IV, doxorubicin 40 to 50 mg/m 2 IV and 5-fluorouracil 500 to 800 mg/m 2 IV (CAF) or cyclophosphamide, epirubicin, and 5-fluorouracil (CEF). Clinical and pathologic assessment of response to chemotherapy was done based on the UICC guidelines. Result: Complete clinical response (cCR) was seen in 10% cases (4/40), thirty percent patients had (12/40) partial response and 60% (24/40) had stable disease after neoadjuvant chemotherapy. Pathologic complete response (pCR) with no evidence of viable tumor was observed in 20% patients (8/40). Fifteen patients (37.5%) showed partial response and 42.5% patients (17/40) had a stable disease. No patient progressed during the course of chemotherapy. Changes in the tumor type were observed following chemotherapy, most common being the mucinous change. Histologic changes like dyscohesion, shrinkage of tumor cells, elastosis, collagenization, necrosis, lymphocytic reaction, giant cell response are some of the common observations seen following treatment with neoadjuvant chemotherapy. Conclusion: Pathologic assessment of response to neoadjuvant chemotherapy is a better predictor than the clinical response. The chemotherapy drugs can be modified based on the response observed after 1-2 cycles of neoadjuvant, the response being based on both tumor and patient's responsiveness.

Bronchoscopic management of benign bronchial stenosis by electrocautery and balloon dilatation.

Benign bronchial stenosis is managed by surgical or bronchoscopic methods. Although surgical approach is definitive, it is technically demanding and is costlier than bronchoscopic treatment. Here, we report the case of a 27-year-old female patient with symptomatic benign bronchial stenosis of the left main bronchus. The stenosis was dilated successfully through a fibreoptic bronchoscope by electrocautery followed by balloon bronchoplasty and application of mitomycin-C. On follow up, there was no evidence of re-stenosis.

Response to growth hormone therapy in indian patients.

Objective. To analyse response to growth hormone therapy on Indian patients with short stature. Methods. Data were collected on 71 patients of short stature on GHT. All patients underwent clinical and hormonal evaluation. GHD was diagnosed in the presence of short stature (height SDS < 2) and peak GH levels < 10 ng/ml. Bone age was estimated using Tanner Whitehouse 3 method (TW3). Results. Primary GHD (73%) was the commonest diagnosis among patients on GHT, followed by organic GHD (12.6%), genetic syndromes (8.4%) and systemic diseases (5.4%). Mean chronological age at presentation was 10.07±3.26 years (median-11 years, range 3-15 years), mean height age was 6.98±2.82 years (median 7.5 years, range 1-13 years) and mean bone age (available for 55 patients) was 7.19±3.1 years (median 8.2 years, range 1.3 - 13 years). Patients with systemic diseases (6.75±3.5 years) presented earlier, compared to patients with GHD (10.27±3.16 years) and genetic syndromes (10.18±3.20 years) (p=0.349). Most of the patients on GHT were in the age group 9-15 years (60.6%). Mean height gain with GHT was 8.7±2.7 cm (median 8.3 cm, range 3.0-13cm) during 1st year then decreased to 6.9±2.4 cm (median 7.0 cm, range 3.0-12.5 cm) in the second year, and was maintained through the third year (mean 7.1±3.0 cm, median 7.0, range 3.0-13 cm). Among patients with GHD, those with primary deficiency had significantly better response to GHT in 1st year than secondary deficiency (9.0±2.65 vs 6.8±3.03 cm, p = 0.026). Response to GHT was negatively correlated with CA (r-0.27, p = 0.05), HA (r-0.47, p = 0.027) and BA (r-0.31, p=0.022) at presentation. Four patients (5.6%) developed hypothyroidism and one patient each developed disseminated tuberculosis and rickets. One patient of Turner's syndrome died of adrenal carcinoma. Short follow up and absence of measurement of IGF-1 and IGFBP3 were major limitations of this study. Conclusions. Response to GHT in Indian patients is comparable to western counterparts. Maximum height gain on GHT is during the first year than decreases in second year, but is maintained through third year. Patients with primary GHD had better response than secondary GHD. Response to GHT is negatively correlated with chronological, height and bone age at presentation.

Calotropis procera -induced keratitis.

Calotropis procera produces copious amounts of latex, which has been shown to possess several pharmacological properities. Its local application produces intense inflammatory response. In the 10 cases of Calotropis procera -induced keratitis reported here, the clinical picture showed corneal edema with striate keratopathy without any evidence of intraocular inflammation. The inflammation was reversed by the local application of steroid drops.

Cervical spine injuries in a teaching hospital of eastern region of Nepal: a clinico-epidemiological study.

Cervical spine injuries with neural deficits carry significant impact economically, socially and psychologically to the individual and to the society. Risk factors involved, mode of injuries, constraints of management and rehabilitation are different in developing countries. Total 149 patients of cervical spine injuries presented in B.P. Koirala Institute of Health Sciences, Dharan, Nepal were evaluated prospectively for three years. Demographic details, etiology of injury, method and time taken for transportation and treatment method and progression of recovery were recorded. Most commonly involved age group was 30-49 years (44%) with male to female ratio of 4:1. Fall related injury especially from trees was the commonest mode of injury (60%). Patients were transported to hospital without neck immobilization (81%) in a vehicle unsuitable for spinal injuries patient with average delay of two days of injury. 79% had neural deficits among which 42% are with quadriparesis, 31% are with quadriplegia. Associated extra spinal injuries were found in 9% patients. Average hospital stay was 31 days. C5 vertebra was the most commonly injured vertebra. Cervical spine injuries, which has major impact over patient and society is still not adequately addressed by medical and public health system of developing countries like Nepal. Incidence of spinal injuries and its devastating consequences can be reduced by appropriate preventive measures and management along with rehabilitation.

Human immunodeficiency virus infection in a child presenting as herpes zoster ophthalmicus.

Herpes zoster is mainly a disease of the elderly. Its occurrence in younger age should be viewed with suspicion. A 9-year-old boy presented with herpes zoster ophthalmicus. He had a history of abdominal surgery one and half years back during which he had received blood transfusion. A year following the surgery he developed general malaise and fever with progressive weight loss. He was treated by local doctors. Subsequently he developed eruptions of blisters around right eye for a duration of 8 days, with which he presented to the department of ophthalmology, Pt JNM Medical College, Raipur. On investigations he was found to have infected with human immunodeficiency virus. Systemic acyclovir along with antiretroviral treatment was started, to which he showed favourable response.

Elemental analysis of aerosol samples collected from an industrial and a non-industrial town of Punjab (India) using PIXE technique.

Chemical composition of the aerosols is an important aspect of aerosol monitoring. The adverse effects on human heath due to different elements in aerosols depend on their concentrations. A comparative study of aerosol concentration and composition from an industrial town Mandi-Gobindgarh and a nearby (25 km away) non-industrial and comparatively less polluted town Morinda, in state Punjab (India) was carried out. Aerosol samples were analyzed by Particle Induced X-ray Emission (PIXE) technique at the Institute of Physics, Bhubaneshwar. Elemental concentrations were found to be much higher in Mandi-Gobindgarh as compared to Morinda. However, the large deviations from the mean concentrations, particularly in Mandi-Gobindgarh is suggestive of highly varying day to day industrial activity and changing weather conditions. Elements such as S, Br and Pb were found higher in the PM2.5 (particulate matter with = 2.5 microm aerodynamic diameter), which are related to burning of coal and oil in furnaces in Mandi-Gobindgarh. The elements related to natural dust such as K, Ca, Ti, Mn, and Fe are mainly distributed in PMcf (particulate matter with aerodynamic diameter between 2.5 and 10 microm) fraction in both the towns. High concentrations of Ti, Cr, Mn, Fe and Zn in the PMcf fraction from Mandi-Gobindgarh are likely due to the industrial activity of Steel rolling mills.

Clinical Spectrum and Prognosis of Multiple Myeloma in Patients Younger than 30 Years : Is it Different from the Elderly

Multiple Myeloma (MM) is a disease of the elderly. The occurrence of MM in patients younger than thirty years is rare. Over the last ten years five patients with MM of less than thirty years of age were evaluated at our centre and which constituted 3.3% of all the MM cases. Three patients had initial extramedullary involvement. All patients responded to the initial planned therapy and were clinically disease free at the time of last follow-up. In the background of clinical and laboratory features, a brief review of literature was carried out and the prognosis of this subgroup has been discussed.

Biokinetics of 65Zn in liver and whole body and its bio-distribution in nickel treated protein deficient rats.

This study was designed to determine the effect of nickel treatment on biological half-lives of 65Zn in whole body and liver as well as on distribution of 65Zn in different organs of protein deficient rats. Nickel sulfate at a dose level of 800mg/l in drinking water was administrated to normal control as well as to protein deficient rats for 8 weeks. A significant increase was found in fast and slow components of biological half lives of 65Zn in whole body and only fast component in liver of protein deficient rats. Interestingly, slow component in whole body and fast component in liver of nickel treated protein deficient rats were not different from normal controls though they were significantly elevated in protein deficient rats. On the other hand, slow component of 65Zn was also not altered in nickel treated protein deficient rats, which however, was significantly decreased in nickel treated rats. Protein deficiency led to a marked elevation in per cent uptake of 65Zn in brain and caused significant depression in liver, kidney and intestine. However, uptake of 65Zn in brain showed a significant depression in nickel treated rats, whereas the uptake was elevated in brain in nickel treated protein deficient rats. In conclusion, protein deficient conditions seem to be playing a dominant role in context with the distribution of 65Zn in different organs when nickel is administered to protein deficient rats. However nickel alone is seen to cause adverse effect on the distribution of 65Zn.

Current perspective in insulin therapy in the management of diabetes mellitus.

Insulin therapy since its discovery in 1922 is effective in restoring normoglycaemia, suppressing ketogenesis, delaying or arresting diabetic complications. The commonest indication for regular insulin therapy in type-2 diabetes is oral hypoglycaemic agent (OHA) failure. Regular insulin therapy is also indicated in patients of diabetes associated with renal or hepatic disease where OHA is contra-indicated. Different types and species of insulin have different pharmacological properties. There are several methods of insulin administration. Efficacy of human insulin has been proved beyond doubt. However, there is no reason to believe that animal insulin is inferior to human insulin.