Left Pulmonary Agenesis with Right Lung Bronchiectasis in an Adult.

Pulmonary agenesis is a rare congenital anomaly characterized by the absence of pulmonary parenchyma and vasculature. Bilateral pulmonary agenesis is incompatible with extrauterine life. Unilateral agenesis is often associated with other congenital cardiovascular, genitourinary and gastrointestinal malformations. Right lung agenesis is more frequently associated with congenital anomalies and has poor prognosis as compared to left lung agenesis. Diagnosis is often made in childhood but can be delayed, if the clinician is not aware about this entity. Chest radiograph in unilateral lung agenesis shows opaque hemithorax and these patients are often confused with other common causes of opaque hemithorax like collapse, pleural effusion and diaphragmatic hernia. We report a case of left lung agenesis with right lung bronchiectasis in a middle-aged adult who was treated for tuberculous pleural effusion and was referred to our institute for persistent symptoms despite treatment.

Benign Recurrent Intrahepatic Cholestasis in a Young Adult.

Benign Recurrent Intrahepatic Cholestasis (BRIC) is a rare genetic disorder characterized by recurrent episodes of cholestatic jaundice. The initial episode of jaundice generally occurs before second decade of life and can persist for several weeks to months before resolving spontaneously. It is a benign disease and even after repeated episodes of jaundice, fibrosis of liver cell does not occur. We had a young adult patient who was having recurrent episodes of cholestatic jaundice with intervening symptom free period for last 20 years. He had first episode of jaundice at the age of eight and since then had several similar episodes. Diagnosis was made by classical clinical presentation and histopathological findings. We intend to report this case due to rarity of this disease in India.

Leukemoid Reaction in Chikungunya Fever.

Chikungunya is a viral illness caused by an arbovirus which is transmitted by Aedes mosquito. Fever and polyarthralgia are hallmark of this viral illness. Viral infections are generally associated with leucopenia and bacterial infections with leukocytosis. Leukemoid Reaction (LR) is defined by reactive increase in leukocyte count of more than 50,000/cu mm with increase in mature leukocytes on peripheral blood. Leukocytosis is common in Chikungunya but leukemoid reaction has not been reported in medical literature. Our patient presented with high grade fever and symmetrical polyarthritis. Blood investigation showed Leukemoid reaction and after extensive work up a diagnosis of chikungunya was made.

Intravenous Immunoglobulin Responsive Persistent Thrombocytopenia after Dengue Haemorrhagic Fever.

Dengue outbreak is common in Indian subcontinent and causes significant morbidity and mortality. Year 2015 has witnessed yet another Dengue epidemic in northern India and the number of cases this year is maximum in a decade. Dengue infection is a viral disease and there are 4 different serotypes DENV1, DENV2, DENV3 and DENV4. This year DENV2 and DENV4 have been isolated from most of the patients. Thrombocytopenia is hallmark of dengue infection and generally recovers within ten days of onset of symptoms. We report a case of dengue haemorrhagic fever in which thrombocytopenia persisted for almost a month and improved after Intravenous immunoglobulin (IVIG) administration. This is the first case where IVIG has been successfully used for treating persisting thrombocytopenia after dengue infection.

Gullo's Syndrome: A Case Report.

Benign Pancreatic Hyperenzymemia (BPH) or Gullo's Syndrome is a new entity with only few reported cases till date. It is characterized by persistently elevated pancreatic enzymes without any clinical or pathological evidence of pancreatic disease. Gullo's syndrome is a diagnosis of exclusion and clinician should be aware of various other conditions which can cause elevation of pancreatic enzymes. There are no reported cases of Gullo's syndrome from Indian subcontinent till date. A 42-year-old lady presented to us with complaints of fever and cough for which she was evaluated and diagnosed to be having left upper zone pneumonia. However, her routine investigations showed persistently elevated serum amylase and lipase levels. She was extensively worked up for pancreatic hyperenzymemia but no pancreatic disease was detected. She was followed up for a period of one year and raised levels of serum lipase and amylase persisted even after a year.

Prevalence of thyroid dysfunction and its correlation with CD4 count in newly-diagnosed HIV-positive adults--a cross-sectional study.

Prevalence of subclinical hypothyroidism in HIV-positive patients is reported to be high in those with severe immune deficiency. However, there is paucity of literature in newly-diagnosed HIV-positive population. Our aim was to estimate the prevalence of thyroid dysfunction and study its correlation with CD4 count in this population. In this cross-sectional study, patients presenting to the antiretroviral therapy clinic were screened with thyroid function tests, including thyroid stimulating hormone, free triiodothyronine, free thyroxine, and anti-thyroid peroxidase antibody levels at the time of diagnosis. Two hundred and twenty-five HIV-positive and an equal number of healthy volunteers were enrolled. The mean (SD) CD4 count in the study group was 147.1 (84) and 70.7% had advanced immune deficiency with CD4 count <200 cells/µL. The overall prevalence of thyroid dysfunction was 75.5% in the study group and 16% in the control group. Subclinical hypothyroidism was the commonest abnormality noted in almost 53%. Significant correlation was observed between CD4 count and thyroid stimulating hormone, free triiodothyronine, and free thyroxine levels (r = -0.86, r = 0.77, and r = 0.84, respectively, p < 0.0001 for all). The present study demonstrated high prevalence of thyroid dysfunction in HIV-positive patients. The dysfunction is subclinical in most cases and correlates well with declining CD4 counts.

Alport syndrome: a rare cause of uraemia.

Alport syndrome (AS) is a heterogeneous basement membrane disease characterised by haematuria with progressive hereditary nephritis, high-frequency sensorineural hearing loss (SNHL) and pathognomonic ocular lesions. It is one of the spectra of diseases representing hereditary nephritis, which inevitably leads to end-stage renal disease (ESRD). Microscopic or frank haematuria persistent from childhood constitutes the clinical clue for its early recognition. It occurs as a result of genetically inherited or de novo mutations in type IV collagen genes. The most common mode of inheritance is X-linked and men are more severely affected. We report a case of a young woman, in her fourth decade of life presenting with overt nephropathy, having persistent haematuria associated with SNHL and lenticonus with dot and fleck retinopathy on detailed clinical examination, diagnosed as a previously undetected case of Alport syndrome.

A Study of Clinical, Microbiological, and Echocardiographic Profile of Patients of Infective Endocarditis.

Infective endocarditis, a great masquerader, is a clinical entity which may present with a myriad of manifestations. Its changing epidemiological profile has been studied in the previous decades in both the developed and the developing nations. In this study, we strived to uphold the evolving clinical profile and its outcome from a government tertiary care hospital in Northern India. It was a descriptive, cross-sectional, observational study conducted over two years' period involving 44 patients diagnosed with definite infective endocarditis, according to modified Dukes' criteria. Demographic, clinical, microbiological, and echocardiographic data were analysed. Mean age of patients was 31 years. Rheumatic heart disease with regurgitant lesions was the commonest risk factor. Dyspnea and fever were the predominant symptom, and pallor and heart failure the commonest sign. Cultures were positive in 52% with Staphylococcus, the major isolate. Transesophageal echocardiography fared better than transthoracic one to define the vegetations. Mortality is reported in 4.5%. Prolonged duration of fever, pallor, hematuria, proteinuria, rheumatoid factor positivity, and large vegetations proved to be poor prognostic variables. Culture positive endocarditis, with persistent bacteremia, had higher incidence of acute renal failure. Right sided endocarditis was frequent in congenital lesions or IV drug user, whereas left sided endocarditis mostly presented with atrial fibrillation.

Guillain-Barre syndrome in an adult patient with Henoch-Schonlein purpura.

A 42-year-old man presented with pain in the abdomen, massive haematemesis and rashes over the body and development of bilateral lower limb weakness the next day. The patient was later diagnosed with Guillain-Barre syndrome with Henoch-Schonlein purpura. He was treated with intravenous immunoglobulins. At 3 months of follow-up and rehabilitation, patient showed complete recovery from weakness.

Pituitary macroadenoma: a rare cause of thyrotoxic hypokalaemic periodic paralysis.

Thyrotoxic hypokalaemic periodic palsy (THPP) is a well-recognised but under-diagnosed complication of hyperthyroidism and is commonly seen in Asian males. Patients usually present fully conscious with acute onset of severe motor weakness. Baseline investigation reveals severe hypokalaemia due to Na(+)/K(+) ATPase overactivity causing a massive influx of intracellular potassium ions. The most common cause of THPP identified in the medical literature is Graves' disease. We report an interesting and unusual case of THPP due to previously undiagnosed hyperthyroidism secondary to a pituitary macroadenoma. The patient was consequently found to have a tumour secreting gonadotropin and thyrotropin.

Etiology and outcome determinants of intracerebral hemorrhage in a south Indian population, A hospital-based study.

BACKGROUND: There is paucity of methodologically sound published studies on intracerebral hemorrhage (ICH) from India, on pub med/embase search. AIMS: To explore etiology of ICH and correlate the causes, location, and size of hemorrhage to clinical outcome. MATERIALS AND METHODS: A hospital-based descriptive study from South Indian eastern coastal town of Puducherry; 60 consecutive subjects aged > 12 years, predominantly of inbred Tamil population, with head CT evidence of intracerebral hemorrhage not associated with trauma and brain tumors, were recruited. Outcome at three months was measured using Glasgow Outcome scale, NIHSS and mortality. SPSS v 19 was used for statistical analysis. RESULTS: Commonest etiological factor was hypertension, followed by bleeding diathesis, thrombolysis for myocardial infarction, and cortical vein thrombosis. Most frequent locations of hematoma were basal ganglia, thalamus, internal capsule, and cerebral and cerebellar parenchyma. Hematoma volume correlated significantly with systolic and mean arterial pressure but not with diastolic blood pressure. Poor outcome was correlated to size (P < 0.05) and intraventricular extension of hematoma (P < 0.05), and to systolic, diastolic and mean arterial pressure, but not to age, gender, smoking, alcoholism, ischemic heart disease, and blood sugar level. Among diabetic patients with ICH, the size of hematoma (P = 0.04) and severity of coma (P = 0.01) at admission were significantly worse compared to the non-diabetic, but not the outcome at three months [Glasgow outcome scale or mortality (P = 0.94 and 0.14)]. CONCLUSIONS: The location of hemorrhage and correlation with outcome agreed with the patterns described for the non-white races in prior reports. Independence of outcome to diabetic status despite a more severe initial presentation may indicate importance of good care, even in high risk groups.

Common carotid intima-media thickness in acute ischemic stroke: A case control study.

Stroke is the third leading cause of mortality worldwide. Combined carotid intima-media thickness (CIMT) is a marker of atherosclerosis and is also a predictor for ischemic stroke. We determined the frequency of CIMT in patients with acute ischemic stroke and in matched controls and also the risk factors for CIMT. Sixty patients with ischemic stroke diagnosed by computer tomography (CT) scan and 50 controls matched by age, gender, diabetes, and hypertension were studied. Subjects in both groups underwent carotid duplex scanning (ACUSON 128 x P/10 machine) with a 7.5 MHz linear superficial array probe in B-mode to determine the CIMT and presence of plaques. The mean age in the patient group was 62 years and 63.3% were males. The average CIMT in the patient group was 0.798 mm and it was 0.6 mm in the control group (P < 0.0001). Patients with carotid plaque had significantly increased IMT (0.95 +/- 0.22) when compared to patients without plaques (0.71 +/- 0.12) (P < 0.001). When the differences in mean IMT were compared among the different age groups in the patient group, there was significance (P < 0.05). In this study the CIMT was independently associated with increasing age and with the presence of carotid plaques.

Acute demyelinating encephalitis due to Abrus precatorius poisoning--complete recovery after steroid therapy.

INTRODUCTION: Poisoning from Abrus precatorius is attributed to a toxalbumin (abrin) that acts by inhibiting protein synthesis and rarely can cause immuno-mediated demyelination. We report a case of abrin poisoning with demyelination. CASE REPORT: A 19-year-old man presented with a history of ingesting crushed Abrus precatorius seeds following a family quarrel. He developed vomiting, abdominal pain, and bloody diarrhea, followed by a seizure and an altered sensorium. Magnetic resonance imaging (MRI) of the brain showed demyelination in the bilateral-medial temporal lobes. The patient was treated with supportive care, and intravenous methylprednisolone followed by oral prednisone, and recovered fully. DISCUSSION: Abrin is an immuno-modulator that may cause immune-mediated demyelination. We report the clinical course of a patient with demyelination after abrin poisoning, treated with corticosteroids, and document his clinical recovery. CONCLUSION: Demyelination is a rare complication of Abrus precatorius poisoning. In our case, the demyelination was demonstrated by MRI. Although our patient appeared to recover completely following methylprednisolone therapy, the suggestion that methylprednisolone or other corticosteroids might be useful in treating this demyelination needs experimental verification and clinical validation before concluding that it is a beneficial therapy.