Osteopetrosis: a rare cause of anemia

Normocytic anaemia is caused either by hypoproliferation of haemopoietic tissue or increased destruction of red cells. Osteopetrosis is a rare cause of anaemia. Infantile osteopetrosis (also called malignant osteopetrosis) is diagnosed early in life. But it is the adult osteopetrosis (also called benign osteopetrosis) which is diagnosed in late adolescence or adulthood that present as anaemia which is difficult to diagnose and treat. Approximately one half of patients are asymptomatic, and the diagnosis is made incidentally, often in late adolescence because radiologic abnormalities start appearing only in childhood. In other patients, the diagnosis is based on family history. Still other patients might present with osteomyelitis or fractures. We are presenting here an unusual case of osteopetrosis which was referred to us for the evaluation of anaemia

Radial Probe Endobronchial Ultrasound for Peripheral Pulmonary Lesions: Initial Experience in an Indian Tertiary Healthcare Centre

Introduction : Diagnosis of peripheral pulmonary nodules is confusing; therefore, an accurate and safe lung biopsy can prevent unnecessary invasive diagnostic procedures. This study soughtto study the diagnostic yield, sensitivity, specificity, and negative and positive predictive values (NPV and PPV) of radial probe endobronchial ultrasound (EBUS)-guided biopsy for peripheral pulmonary lesions. Materials and Methods: Patients referred to the Division of Pulmonary Medicine for evaluation of peripheral pulmonary lesions were subjected to radial probe EBUS-guided transbronchial lung biopsy under conscious sedation after reviewing positron emission tomography scan/computed tomography results. The obtained specimens were considered diagnostic when the cytological, histopathological, or microbiological diagnosis was consistent with the clinical presentations. Results: Totally, 14 procedures were performed on 13 patients with mean lesion size of 30.42 mm. Mean distance between the lesion and pleura was 1.17±0.68 cm, and the diagnostic yield of this technique was 78.57%. Furthermore, the sensitivity, specificity, and NPV were 70% (range: 34.75 to 93.33), 100% (range: 39.76 to 100), and 57.14% (range: 18.41 to 90.10), respectively. This procedure was not associated with any major complications. Conclusion: Radial probe EBUS with satisfactory diagnostic yield and low complication rate is a promising tool for early diagnosis of lung cancer

Ofuji's disease in an immunocompetent patient successfully treated with dapsone

Eosinophilic pustular folliculitis or Ofuji's disease is a non-infectious eosinophilic infiltration of hair follicles, which usually presents with itchy papules and pustules in a circinate configuration. We report this case of an immunocompetent patient with erythematous papules and plaques without macropustules diagnosed as eosinophilic pustular folliculitis—a rarely reported entity outside Japan. He was successfully treated with oral dapsone

First two bilateral hand transplantations in India (Part 4): Immediate post-operative care, immunosuppression protocol and monitoring

Introduction: Being able to counter immune-mediated rejection has for decades been the single largest obstacle for the progress of vascular composite allotransplantation (VCA). The human immune system performs the key role of differentiating the ‘self’ from the ‘non-self’. This, although is quintessential to eliminate or resist infections, also resists the acceptance of an allograft which it promptly recognises as ‘non-self’. Materials and Methods: Pre-operative evaluation of the recipient evaluation included immunological assessment in the form of panel reactive antibodies (PRA), human leucocyte antigen (HLA) typing, donor-specific antibody detection assays (DSA) and complement-dependent cytotoxicity assays (CDC). Induction immunosuppression was by thymoglobulin and the maintenance by the standard triple-drug therapy. Results: Both the recipients were managed by the standard triple drug therapy and have had only minor episodes of rejections thus far which have been managed appropriately. Discussion: Induction immunosuppression was by thymoglobulin and the maintenance by the standard triple-drug therapy. Various groups have tried various other formulations and regimes as well. Conclusion: A comprehensive plan has to be drawn up for immunological screening, selection and the post-operative immunosuppressant usage. The ultimate goal of these immunosuppression modalities is to achieve a state of donor-specific tolerance