Evaluation of the Crithidia assay to distinguish between auto-immune chronic active hepatitis and systemic lupus erythematosus

The aim of this study was to determine if the Crithidia luciliae assay for auto-antibodies to double-stranded DNA, often positive in systemic lupus erythematosus, is always negative in auto-immune chronic active hepatitis (CAH) as has recently been suggested. Twenty-five patients were identified as having auto-immune CAH. Mean duration of follow-up was 10.5 years. Antinuclear antibodies were detected in 92%, smooth muscle antibodies in 76% and antimitochondrial antibodies in 16%. Antibodies to double-stranded DNA were detected by the Crithidia assay in four patients (16%). Two of these patients had positive tests on only one occasion and no features of systemic lupus erythematosus. In the other two the assay was persistently positive. During follow-up both developed arthritis and serositis but the liver lesion remained the dominant clinical feature. It was concluded that there is significant serological overlap between auto-immune CAH and systemic lupus erythematosus making the Crithidia assay unreliable in distinguishing between them.     

Generalized elastosis perforans serpiginosa in Down''s syndrome

Elastosis perforans serpiginosa is a rare disorder of epidermal perforation characterized by the extrusion of dermal elastic tissue through the epidermis. Its aetiology is unknown, but there is histological and biochemical evidence of an abnormality of elastic tissue. Three forms of elastosis perforans serpiginosa exist. It may be either idiopathic, iatrogenic, or, in approximately one quarter of cases, associated with certain genetically determined disorders of connective tissue. Cutaneous lesions appear between the ages of 6 and 20 years, and persist for 6 months to 5 years. They may be confined to one anatomic area, or less frequently are disseminated. We review the case of a 28-year-old woman with recent onset of unusually extensive elastosis perforans serpiginosa with co-existing Down's syndrome and (secondary) sclerosing cholangitis.     

A Subset of Patients With Recurrent Spontaneous Abortion Is Deficient in Transforming Growth Factor β-2-Producing “Suppressor Cells” in Uterine Tissue Near the Placental Attachment Site

PROBLEM : To determine if patients with unexplained recurrent miscarriage have a deficiency of decidual immunosuppressor cells that produce transforming growth factor β type 2, as has been found in mice with abortion due to rejection and/or trophoblast failure. METHODS : Decidual biopsy specimens were taken as near to the placental attachment site as possible under ultrasound guidance from first trimester legal termination (control) patients with recurrent miscarriage and non-viable pregnancy, and from patients with sporadic missed abortion. The tissue was tested for TGFβ-2⁺ suppressor cells by in situ hybridization, immunohistochemistry, and analysis of supernatants. RESULTS : TGFβ-2-related suppressor molecules similar but not identical to those identified in pregnant mice were released by decidual lymphoid cells. Fifty percent of 14 recurrent miscarriage patients showed a lack of suppressor cells and 59% were subnormal in comparison to 20 controls and 5 sporadic miscarriage patients, where 80–85% of the patients had detectable suppressor cells. CONCLUSIONS : Suppressor cell deficiency is compatible with a role for rejection and/or trophoblast failure in some patients with recurrent miscarriage. Presence of suppressor cells in most patients with missed abortion (4/5) is compatible with an alternative cause of fetal death, similar to findings reported in genetic fetal death mice.     

Chylous ascites in cirrhosis: A case report and review of the literature

Chylous ascites is an uncommon clinical entity which results from the accumulation of fat, predominantly chylomicrons, in the ascitic fluid. Conventional treatment methods are unsatisfactory. A patient is reported with chylous ascites associated with cirrhosis and portal hypertension in whom the ascites, the renal insufficiency and the fluid and electrolyte disturbances were corrected by the insertion of a Denver peritoneovenous shunt.     

The effect of growth hormone on growth and blood urea levels in children with chronic renal failure

It has been claimed that low protein diets slow deterioration of chronic renal failure (CRF) by reducing renal solute load. The anabolic effect of recombinant human growth hormone (rhGH) also has potential to reduce renal solute load and thereby slow progression of renal failure. The aim of this study was to determine the effect of rhGH on growth, renal solute load and renal function in children with CRF.
Seven prepubertal children, aged 2-14 years, with moderately severe CRF (creatinine clearance 7.7-23.4 mL/min per 1.73 m²) were treated with daily subcutaneous rhGH, 1 U/kg per week for 10-12 months. As expected, mean height velocity standard deviation scores (SDS) increased, from - 2.87 before treatment to + 3.39 on rhGH, and mean height increased from - 3.1 to - 2.4 SDS. Serum urea concentrations decreased in most patients during the first month of growth hormone treatment from a mean of 20.0pm7.7 mmol/L to 14.8pm5.8 mmol/L ( P = 0.006). The serum urea then returned to pretreatment levels over the next few months.
In the 12 months before treatment with growth hormone, mean creatinine clearance decreased from 19.3 mL/min per 1.73 m² to 16.7 mL/min per 1.73 m². In the next 12 months on rhGH mean creatinine clearance decreased further to 13.5 mL/min per 1.73 m². Therefore the rate of deterioration of renal function was unaffected during treatment with growth hormone. Initial treatment with rhGH is associated with decrease in serum urea concentrations in children with CRF, probably mediated by stimulation of anabolic incorporation of dietary nitrogen into body protein. Despite this reduction in renal solute load, renal function deterioration continued unchanged in most children.