Nephrotic syndrome in Trinidadian children

Fifty seven children with idiopathic nephrotic syndrome who were seen at two hospitals in Trinidad between 1989 and 1995 (median follow-up period, 38 months) were classified according to their response to glucocorticoids. 27 (47 percent) were two to six years old at presentation; 37 (65 percent) were of East Indian descent, 7 (12 percent) were of African descent, and 12 (21 percent) were of mixed race. 55 (96 percent) responded to glucocorticoids. Renal biopsies in 15 patients revealed membranoproliferative glomerulonephritis and membranous nephropathy in the two patients who had not responded to glucocorticoids. Ten patients showed mesangial hypercellularity, associated with immunoglobulin deposits in 7 cases. Age, presentation with nephrotic features, mesangial hypercellularity and immunoglobulin deposits did not predict for unresponsiveness to glucocorticoids. These findings may be explained by the predominance of East Indians in the study group.(AU)

Nephrotic syndrome in Trinidadian children

Fifty seven children with idiopathic nephrotic syndrome who were seen at two hospitals in Trinidad between 1989 and 1995 (median follow-up period, 38 months) were classified according to their response to glucocorticoids. 27 (47 percent) were two to six years old at presentation; 37 (65 percent) were of East Indian descent, 7 (12 percent) were of African descent, and 12 (21 percent) were of mixed race. 55 (96 percent) responded to glucocorticoids. Renal biopsies in 15 patients revealed membranoproliferative glomerulonephritis and membranous nephropathy in the two patients who had not responded to glucocorticoids. Ten patients showed mesangial hypercellularity, associated with immunoglobulin deposits in 7 cases. Age, presentation with nephrotic features, mesangial hypercellularity and immunoglobulin deposits did not predict for unresponsiveness to glucocorticoids. These findings may be explained by the predominance of East Indians in the study group.

SLE or HIV? Dilemma for the Caribbean clinician! - abstract

Systemic lupus erythematosus (SLE) is not an uncommon disease in the Caribbean. The clinical presentation though often characteristic can include a variety of non-specific symptoms. HIV infection, an increasing problem in the region, can mimic rheumatological diseases like SLE. This paper highlights the clinical and serological overlap of these two conditions. A 24-year old female presenting with fever, cough, weight loss, polyarthralgia and alopecia was treated for SLE. The possibility of AIDS was entertained. HIV antibody was positive by ELISA testing. Western Blot analysis was indeterminate with non-viral bands indicating a false positive ELISA test. The eventual outcome supported a final diagnosis of SLE. Four months after presentation, HIV antibody was negative by both ELISA and Western Blot analysis. This case prompted a retrospective analysis of medical records and a further three cases of SLE with false positive HIV tests by ELISA were identified. In a fourth patient with nephritis, the finding of a positive HIV by ELISA pre-empted therapeutic intervention. This patient succumbed. Ante-mortem blood samples were strongly positive for antinuclear factor and double-stranded DNA but negative for HIV by Western Blot. Caribbean clinicians should therefore be made aware of the possibility that HIV can mimic rheumatological disease and that rheumatological disease can result in false positive tests for HIV. The specific immunoblot techniques would be the investigation of choice in suspicious cases (AU)

An analysis of skin involvement in lupus erythematosus - abstract

Of 105 patients diagnosed as lupus erythematosus at the Port-of-Spain General Hospital between 1981 and 1986, 86 satisfied the ARA criteria for the classification of SLE, whilst 19 were clinically and histologically DLE. Fifty-eight SLE patients were of African origin, 24 of Asian origin and 4 were mixed. The ages of onset varied from 14 to 64 years, the majority presented in the 15 to 24-year age group. Of the SLE group, 55 percent had malar rash and 21 percent discoid lesions; this is higher than in the Jamaican study (32 percent and 14 percent respectively). Photosensitivity in the Trinidadian patients (20 percent) and the Jamaican patients (12 percent) was lower than that reported in temperate zones. These differences may be due to genetic and environmental factors. Direct immunofluorrescence (DIF) on 32 biopsies of non-sun esxposed (NSE) skin was characteristic of SLE in 80 percent cases compared to 30 percent by histology. Deposits of IgG, IgA and complement in the NSE skin are associated with a poor prognosis. IgM deposits were found in the NSE skin of 4 DLE patients; this probably represents a transitional phase to SLE, a benign state or a distinct subset of lupus erythematosus. This study confirms that DIF is a far more reliable and sensitive diagnostic tool than histology in the differential diagnosis of SLE and DLE, and in the early detection of SLE (AU)

Significant proteinuria: the Port-of-Spain General Hospital experience - abstract

Sixty-nine patients who had renal biopsies because of proteinuria of > 1 gm per day, oedema and normal sized kidneys were investigated. There were 58 adults and 11 children. Of the adults, 26 were male, and 32 female; of the 11 children, there were 10 males and 1 female. The modal age of the patients was 15 - 19 years. The majority of the patients (67 percent) had primary glomerular disease. The two most common histological patterns were minimal change and mesangio-capillary glomerulo-nephritis. Minimal-change nephritis was observed in 73 percent of children's kidney biopsies. The pattern was unlike that seen in tropical Africa. Mesangial-proliferative disease did not contribute remarkably (11 percent) to significant proteinuria. Membranous nephropathy, which usually accounts for the majority of idiopathic adult nephrotics in the developed countries, was rare in our Trinidadian series. Systemic lupus erythematous comprised the majority of patients (83 percent) who had secondary proteinuria. This may reflect a bias in the selection of patients for renal biopsy. Even though the incidence of the sickle-cell gene is high in Trinidad, sickle-cell disease did not contribute to the presence of nephropathy. The prognosis for patients with primary glomerular disease (21 percent mortality) was better than for thos with systemic disease (79 percent mortality). Our observations suggest that the pattern of proteinuria in Trinidad does not resemble that seen in tropical Africa but is some what similar to that observed in Jamaica (AU)

Immunopathological studies in a mixture of pemphigus and pemphigoid - abstract

This is the first report from the Commonwealth Caribbean of a mixture of two rare forms of vesiculobullous diseases. A mixture of both bullous pemphigoid and pemphigus vulgaris was present in a 61 year-old East Indian man. He presented at the Dermatology Clinic of the Port-of-Spain Hospital with vesiculobullous lesions of both the tense and flaccid types extending throughout his trunk, limbs and face. Skin biopsies showed both immunoglobulin and complement at the intercellular spaces of the epidermis of the flaccid bulla and at the basement membrane zone (BMZ) from the tense bulla supporting the existence of both pemphigus and pemphigoid but the fourth confirmed the above findings. This patient gave a favourable clinical response to corticosteroid therapy with the disappearance of pemphigus antibodies but the anti-BMZ antibodies (typical of pemphigoid) persisted, as expected. It is clear from these findings that immunopathological investigations are not only essential in the early diagnosis of these rare bullous forms but also in prognosis (AU)

Characterisation of mixed connective tissue disease - abstract

Of sixteen patients with MCTD, 10 62 percent) presented with the clinical features of systemic lupus erythematosus (SLE) and 6 (37 percent) with SLE-scleroderma. Ninety-four per cent had a speckled antinuclear factor pattern and 100 percent had IgG deposits in a speckled pattern within the epidermal cell nuclei of the skin. Seventy per cent of these patients had nRNP antibodies. These characteristics are more typical of the mixed connective tissue syndrome than of SLE or scleroderma. The low incidence of Raynaud's phenomenon in our group of patients (18 percent) with MCTD is probably due to the tropical climate. The IgM deposits in the skin of a significant number (67 percent) of the patients with the clinical picture of SLE-scleroderma probably represent an early stage in the development of the disease. The significance of high serum IgA in the clinically diagnosed SLE group is not understood and is now being further investigated. This study strongly suggests that laboratory investigations are of primary importance in distinguishing MCTD from SLE and other forms of collagen vascular diseases (AU)