ABSTRACT
Gaucher's disease is a rare lysosomal storage disorder. Excess accumulation of glucosylceramide predominantly occurs in abdominal viscera. Cardiac involvement is rare, though they generally represent as restrictive cardiomyopathy and pericarditis. Our case, a 19-year old boy initially presented with hepatosplenomegaly but later diagnosed with haemorrhagic pericardial effusion. Enzyme supplementation gives successful outcome but our case was managed symptomatically with pericardiocentesis.
Subject(s)
Gaucher Disease/complications , Hemorrhage/etiology , Pericardial Effusion/etiology , Gaucher Disease/diagnosis , Hemorrhage/therapy , Hepatomegaly/etiology , Humans , Male , Pericardial Effusion/therapy , Pericardiocentesis , Splenomegaly/etiology , Young AdultABSTRACT
Remitting seronegative symmetrical synovitis with pitting edema (RS3PE) is a rare entity mainly found in elderly males. It is characterized by pitting edema mainly of dorsum of both hands giving a "boxing glove hand" appearance; rarely involving feet also, acute in onset, negative rheumatoid factor and a good response to low dose corticosteroid therapy. Clinically it almost resembles a case of polymyalgia rheumatica, late onset rheumatoid arthritis or other seronegative spondyloarthropathy.Though there are multiple underlying factors causing this rare entity but it has very close associations with many malignancies.So far its association with solid tumours and hematological malignancies has been reported. Phyllodes tumour of breast shows wide spectrum of activity from a benign condition to a locally aggressive and sometimes metastatic tumour.One fourth of the cases recur after definitive treatment.Our case represent an unusual association with recurrent phyllodes tumour of breast with RS3PE.
Subject(s)
Breast Neoplasms/complications , Neoplasm Recurrence, Local/complications , Phyllodes Tumor/complications , Adult , Breast Neoplasms/pathology , Breast Neoplasms/surgery , Edema/etiology , Female , Foot , Hand , Humans , Neoplasm Recurrence, Local/drug therapy , Phyllodes Tumor/pathology , Phyllodes Tumor/surgery , Synovitis/etiologyABSTRACT
Tuberculosis is one of the leading causes of development of Immune reconstitution inflammatory syndrome (IRIS) in HIV patients receiving antiretroviral therapy (ART). OBJECTIVE: To determine the incidence of IRIS in HIV-TB coinfected patients, and to find out the possible risk factors associated with IRIS. MATERIALS AND METHODS: Study commenced with 96 patients adhered to standard antitubercular therapy (ATT) and ART without defaultering, and followed up for six months. RESULT: The mean (± SD) CD4 count and CD4 percentage at baseline was 59.16 (± 24.63) per mm³ and 4.59 percent (± 1.73) respectively. Only 18.75 percent developed IRIS after 57.05 (± 14.12) days of initiation of ART. Extrapulmonary tuberculosis was the most significant factor associated with IRIS (83.33 percent) than those without IRIS (44.87 percent) (p = 0.0032). Specifically, tubercular lymphadenitis (38.88 percent, p = 0.0364) and disseminated tuberculosis (33.33 percent, p = 0.0217) were significantly associated with IRIS. The other risk factors associated with appearance of IRIS were higher CD4 count (p = 0.0212) at three months after initiation of ART and increment of CD4 count (p = 0.0063) and CD4 percentage (p = 0.0016) during this period. The major manifestations of IRIS were fever (40 percent), followed by lymphadenitis (38 percent). The mortality rate in IRIS was not higher than those without IRIS. CONCLUSION: Patients with extrapulmonary tuberculosis, especially tubercular lymphadenitis, were more likely to develop IRIS and fever was associated in most of them. Higher increment of CD4 count may indicate development of IRIS in presence of new or worsening tuberculosis lesion.
Subject(s)
Adult , Female , Humans , Male , Middle Aged , AIDS-Related Opportunistic Infections/drug therapy , Anti-Retroviral Agents/adverse effects , Antitubercular Agents/adverse effects , Immune Reconstitution Inflammatory Syndrome/chemically induced , Tuberculosis, Pulmonary/drug therapy , AIDS-Related Opportunistic Infections/epidemiology , Anti-Retroviral Agents/therapeutic use , Antitubercular Agents/therapeutic use , Cross-Sectional Studies , Incidence , Immune Reconstitution Inflammatory Syndrome/diagnosis , Immune Reconstitution Inflammatory Syndrome/epidemiology , India/epidemiology , Risk Factors , Tuberculosis, Pulmonary/epidemiologyABSTRACT
UNLABELLED: Tuberculosis is one of the leading causes of development of Immune reconstitution inflammatory syndrome (IRIS) in HIV patients receiving antiretroviral therapy (ART). OBJECTIVE: To determine the incidence of IRIS in HIV-TB coinfected patients, and to find out the possible risk factors associated with IRIS. MATERIALS AND METHODS: Study commenced with 96 patients adhered to standard antitubercular therapy (ATT) and ART without defaulting, and followed up for six months. RESULT: The mean (± SD) CD4 count and CD4 percentage at baseline was 59.16 (± 24.63) per mm³ and 4.59% (± 1.73) respectively. Only 18.75% developed IRIS after 57.05 (± 14.12) days of initiation of ART. Extrapulmonary tuberculosis was the most significant factor associated with IRIS (83.33%) than those without IRIS (44.87%) (p = 0.0032). Specifically, tubercular lymphadenitis (38.88%, p = 0.0364) and disseminated tuberculosis (33.33%, p = 0.0217) were significantly associated with IRIS. The other risk factors associated with appearance of IRIS were higher CD4 count (p = 0.0212) at three months after initiation of ART and increment of CD4 count (p = 0.0063) and CD4 percentage (p = 0.0016) during this period. The major manifestations of IRIS were fever (40%), followed by lymphadenitis (38%). The mortality rate in IRIS was not higher than those without IRIS. CONCLUSION: Patients with extrapulmonary tuberculosis, especially tubercular lymphadenitis, were more likely to develop IRIS and fever was associated in most of them. Higher increment of CD4 count may indicate development of IRIS in presence of new or worsening tuberculosis lesion.
