Out of 330 adult Systemic Lupus Erythematosus (SLE) cases who attended the Rheumatic Care Centre, Government General Hospital, 59 children were analysed. There was no case with onset before the age of 5 years. There were 49 females and 110 males (M:F = 1:4.9). The initial manifestations were fever (67%), arthritis (61%), skin rash (59%) and lymphadenopathy (27.1%). There was no case of Raynaud's phenomenon. Only 10.1% of patients presented with thrombocytopenic purpura. In the cumulative clinical features, arthritis in 86.6%, fever in 79.8%, skin rash in 69.4%, lymphadenopathy in 61% and hepatosplenomegaly in 39.9% were observed. Renal involvement was seen in 49.1%, neuropsychiatric manifestations in 27.1%, pleuropulmonary in 22% and cardiac manifestations in 10.2%. Anaemia was seen in 50.8%, leukopenia in 18.4%, thrombocytopenia in 11.8%, ANA in 100%, anti-dsDNA in 92.3%, anti-Sm in 34.7%, anti-SSA in 38.5%, anti-SSB in 15.4%, ACL in 30.8%, low C3 in 50% and false positive VDRL in 3.3%. Death occurred in 8 children, 3 due to infection, 2 due to renal causes, 1 due to cardiac and 2 due to central nervous system involvement.

Adult↗

PubMedDate not supplied

Pregnancy-exaggerated galactosemia and congenital cataracts.

One child in a family and two children in another family had galactosemia and congenital cataract. Two of them had total soft cataracts while in one, cataract was less soft. In addition, they had mild lactosuria. The mothers of the affected children had significant lactosuria and mild galactosuria without cataracts. Fathers did not have galactosuria or lactosuria. Clinically unaffected siblings in one family had mild galactosuria and lactosuria. Pregnancy-exaggerated galactosemia was suspected in these two mothers who gave birth to children with congenital cataract. As an extension of this work, 5001 pregnant women were screened for galactose in urine just before the delivery of babies. Mild galactosuria was present in 54 (1.08%). Three children had congenital cataract and one had changes in posterior pole and cornea. Restriction of lactose by reducing intake of milk and milk products during pregnancy by mothers with galactosuria is recommended to avoid the birth of children with congenital cataract.

Adult↗

PubMedDate not supplied

Percutaneous transvenous mitral commissurotomy in patients with severe mitral stenosis and acute rheumatic fever.

There is a concern that percutaneous transvenous mitral commissurotomy (PTMC) performed during acute rheumatic fever (ARF) may result in adverse outcomes. We performed a retrospective study at a tertiary care center. Ten patients (ages 8-12 years) with severe symptomatic mitral stenosis and ARF who underwent PTMC (ARF group) were compared with 10 controls with similar mitral valve area (MVA). The procedure was successful in all patients. The median MVA increased from 0.7 to 1.5 cm2 following PTMC in the ARF group and from 0.7 to 1.8 cm2 in the control group [p = not significant (NS)]. The median MVA was 1.2 cm2 in the study group compared to 1.6 cm2 in the control group (p = 0.02) over a mean follow-up of 17.5 +/- 11.7 months. Echocardiographic restenosis occurred in 4 patients (40%) in the ARF group compared to 1 patient (10%) in the control group (p = NS). The improvement in the New York Heart Association functional class was maintained in 8 patients of the ARF group and 9 patients of the control group. PTMC can be performed in children with ARF without an increase in procedural complications. However, the restenosis rates appear to be higher.

Acute Disease↗

PubMedDate not supplied

Iatrogenic iliac artery rupture: emergency management by longer stent-graft on a shorter balloon.

Rupture of an iliac artery during percutaneous transluminal coronary angioplasty is a rare but potentially devastating complication. We report a case of iatrogenic external iliac artery rupture that was successfully treated by temporary balloon occlusion followed by endovascular stent graft placement in an unusual manner. Limited availability of the hardware necessitated the use of a longer bare stent graft mounted on a relatively shorter balloon.

Angioplasty, Balloon, Coronary↗

PubMedDate not supplied

Eales disease--an update.

Eales disease, first described by Henry Eales in 1880, remains an enigma. The disease, observed more commonly in the Indian subcontinent than in the rest of the world, occurs in young healthy adult males, initially presenting as retinal periphlebitis and later as retinal ischemia that may lead to vascular alterations and neovascularization. Recurrent vitreous hemorrhage with or without retinal detachment is the common sequelae. In recent years, immunological, molecular biological, and biochemical studies have indicated the role of human leukocyte antigen, retinal autoimmunity, mycobacterium tuberculosis genome, and free radical mediated damage in the etiopathogenesis of this disease. However, its etiology appears to be multifactorial. The management depends on the stage of the disease and consists of medical treatment with oral corticosteroids in the active inflammatory stage and laser photocoagulation in the advanced retinal ischemia and neovascularization stages. The results of vitreoretinal surgery have been found to be satisfactory in case of vitreous hemorrhage with or without retinal detachment.

Diagnosis, Differential↗

PubMedDate not supplied

Primary vitrectomy for combined rhegmatogenous retinal detachment and choroidal detachment with or without oral corticosteroids: a pilot study.

PURPOSE: The occurrence of choroidal detachment (CD) in eyes with primary rhegmatogenous retinal detachment (RRD) is relatively uncommon (2%-4.5%). Recent reports suggest that primary vitrectomy yields better anatomic success than scleral buckling. However, for these inflamed eyes with low intraocular pressure, the influence of preoperative oral steroids on reattachment rates has not been elucidated yet. METHODS: Twenty eyes with combined RRD and CD that underwent primary vitrectomy were randomized to receive oral steroids (for 1 week) or no oral steroids before surgery. RESULTS: Preoperative clinical data such as mean age, lens status, Snellen visual acuity, duration of macular detachment, CD (size and extent), and retinal detachment characteristics (e.g., extent, number of retinal breaks, atrophic or tractional retinal break, size of retinal break, and location of retinal break) were similarly distributed in both groups. Single-operation anatomic success was 81.8% (9/11) among those patients who received preoperative oral steroids and was 66.7% (6/9) among those who did not receive preoperative oral steroids. After reoperation, anatomic success was 100% in both groups. The mean follow-up was 20.1 months. CONCLUSION: The results suggest that administration of oral steroids before primary vitrectomy in eyes with combined RRD and CD improves reattachment rates.

Administration, Oral↗

PubMedDate not supplied

Inhibition of vascular endothelial cells by 1,4-phenylenebis (methylene)selenocyanate--a novel chemopreventive organoselenium compound.

Organoselenium compound 1,4-phenylenebis(methylene)selenocyanate (p-XSC) was investigated for its effects on endothelial cell proliferation in vitro and angiogenesis in vivo. The organoselenium compound, p-XSC, has been shown to prevent carcinogen-induced tumorigenesis in murine model systems with low toxicity. Since tumor growth and metastasis are dependent on angiogenesis, we investigated the effects of the organoselenium compound on this process. Human umbilical vein endothelial cells treated with p-XSC showed concentration dependent inhibition of protein synthesis and cell viability in vitro with a TCID50 value of 6 microM. Subsequently, we studied the effects of p-XSC on experimental angiogenesis. Addition of p-XSC to three-dimensional cultures inhibited endothelial cell tube formation. Furthermore, p-XSC treatment inhibited growth factor induced angiogenesis in chick chorioallantoic membrane assays and i.p. administration of p-XSC inhibited neovascularization induced by tumor cells implanted subcutaneously into athymic mice. These studies suggest that vascular endothelium is an additional target for the chemopreventive organoselenium compound p-XSC.

Animals↗

PubMedDate not supplied