Pulmonary alveolar proteinosis a rare disease.

Pulmonary alveolar proteinosis is a rare lung disease in which abnormal accumulation of surfactant occurs with in the alveoli, interfering with gas exchange. PAP can occur in a primary form or secondarily in the settings of malignancy , pulmonary infection, or environmental exposure to dusts or chemicals. Rare familial forms have also been recognized suggesting a genetic component . we are going to present a rare case of pulmonary alveolar proteinosis.

Gunthers with haemolytic anaemia extremly rare: A case report.

Congenital erythropoietic porphyria (CEP), also called Gunthers is the rarest of the porphyrias, with a prevalence estimated at 1 in 1,000,000 or less. Only approximately 200 cases of CEP have been reported till now worldwide. CEP affects males and females equally, and occurs in all ethnic groups. Clinically it is a subacute to chronic type of porphyria, defect is expressed in infancy and clinical features such as extreme cutaneous photosensitivity, blistering, scarring, hyper and hypo pigmentation of photo exposed parts. Haemolytic anaemia with splenomegaly and retarded growth may also be present. Due to its rarity we are presenting Congenital erythropoietic porphyria with haemolytic anaemia.

Remmiting seronegative symmetrical synovitis with pitting edema (RS3PE) a rare Disease.

Remitting Seronegative Symmetrical Synovitis with Pitting Edema is a rare clinical entity described as an acute onset polyarthritis with edema affecting geriatric population particularly males. The etiopathogenesis of disease is still elusive but clinical, radiological and immunological characteristics separate it from the more common disease like Rheumatoid arthritis and Polymyalgia rheumatica. Disease has an excellent prognosis with low dose steroids and patients undergo long-term remission even after withdrawal of drugs. We hereby describe a case of 90 year old female presented to us with polyarthritis and pitting edema of hands that we diagnosed as RS3PE.

Role of serum HsCRP as prognostic markers in septicemia in elderly population.

Background: Presence of elevated serum HsCRP is strongly associated with morbidity and mortality in diverse populations with sepsis and is a potentially useful biomarker to risk stratify patients with severe sepsis. Aims & Objective: We aimed to study whether the serum HsCRP level at presentation in elderly patients with sepsis correlate with stage of sepsis and ultimate outcome. Material and Methods: In a single centre prospective, observational study conducted in geriatric ward of tertiary care centre, 200 elderly patients (age>60 yrs) with sepsis were included. The primary outcome was 14 days in hospital mortality. Risk factor variable was venous serum HsCRP (mg/l) at presentation. Results: Mortality at 14 days in elderly patients with sepsis was 20%. Mean serum HsCRP in survivor group was 33.42 ± 21.56 while 57.28 ± 25.31 in mortality group (p value <0.001). The mean serum HsCRP level in survivor and non survivors was 32.49 ± 21.45 and 48.05 ± 16.40 in sepsis, 33.61 ± 25.45 and 67.71 ± 21.49 in severe sepsis, 46.62 ± 10.55 and 73.82 ± 32.87 in MODS while 49.0 ± 26.72 and 101.5 ± 9.19 in septic shock group. Conclusion: We conclude that initial high serum HsCRP level was associated with increased mortality independent of etiology of sepsis, it also correlate well with severity of sepsis and serum HsCRP level were high in those with septic shock.