Evaluation of the reliability and validity of the Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) in paediatric cutaneous lupus among paediatric dermatologists and rheumatologists.

BACKGROUND: The Cutaneous Lupus Erythematosus Disease Area and Severity Index (CLASI) is a reliable outcome measure for cutaneous lupus erythematosus (CLE) in adults used in clinical trials. However, it has not been validated in children, limiting clinical trials for paediatric CLE. OBJECTIVES: This study aimed to validate the CLASI in paediatrics. METHODS: Eleven paediatric patients with CLE, six dermatologists and six rheumatologists participated. The physicians were trained to use the CLASI and Physician's Global Assessment (PGA), and individually rated all patients using both tools. Each physician reassessed two randomly selected patients. Within each physician group, the intraclass correlation coefficient (ICC) was calculated to assess the reliability of each measure. RESULTS: CLASI activity scores demonstrated excellent inter- and intrarater reliability (ICC > 0·90), while the PGA activity scores had good inter-rater reliability (ICC 0·73-0·77) among both specialties. PGA activity scores showed excellent (ICC 0·89) and good intrarater reliability (ICC 0·76) for dermatologists and rheumatologists, respectively. Limitations of this study include the small sample size of patients and potential recall bias during the physician rerating session. CONCLUSIONS: CLASI activity measurement showed excellent inter- and intrarater reliability in paediatric CLE and superiority over the PGA. These results demonstrate that the CLASI is a reliable and valid outcome instrument for paediatric CLE.

Early versus later onset childhood-onset systemic lupus erythematosus: Clinical features, treatment and outcome.

The objective of the study was to compare clinical features, treatment and disease outcome in patients with early versus later onset of childhood-onset systemic lupus erythematosus (cSLE). A retrospective matched cohort study of cSLE patients diagnosed between 1988 and 2008 and followed for a minimum of one year was conducted. Thirty-four pre-pubertal cSLE patients with disease onset prior to their 12th birthday were matched by ethnicity and year of diagnosis to 34 pubertal cSLE patients. The most common criteria at diagnosis in both groups were malar rash, arthritis, hematologic manifestations, and renal disease. After a mean follow-up of more than six years, a similar proportion of patients in the two groups were still prescribed corticosteroids (47% and 41%); patients in the early onset group required a significantly higher daily dose (0.6 mg/kg prednisone-equivalent versus 0.2 mg/kg, p < 0.05). There were no significant differences in organ involvement, disease activity and disease damage between the two groups, and severe complications occurred at similar rates. There were a greater number of admissions to the pediatric intensive care unit (PICU) in the early onset group (18 versus 5, p = 0.01), with time-to-event analysis demonstrating a significantly shorter disease duration from diagnosis to first PICU admission in the early onset group (p < 0.001). While a similar proportion of patients in the early and later onset groups required treatment with cyclophosphamide, patients in the early onset group received treatment earlier in their disease course (mean 13.7 versus 19.9 months, p < 0.001). Early onset cSLE leads to earlier and more frequent PICU admission, earlier use of cyclophosphamide, and higher corticosteroid dose at long-term follow-up.

Leukergy--a new diagnostic test for bone infection.

This new blood test for infection is based on the phenomenon of leukergy in which white cells agglomerate in the peripheral blood of patients with inflammatory diseases. It was used in 26 patients with proven bone or joint infection and was positive in 25. The leukergy test was more accurate than the ESR, white cell count or blood culture. The percentage of cells agglomerated correlated with the clinical severity of the infection and the test detected reactivation of the septic process better than the other haematological tests. It is a rapid and inexpensive method which is useful in the diagnosis and management of bone and joint infections.

Prostaglandin E2 in duodenal ulcer complications.

Prostaglandins are presumed to have cytoprotective properties and may play a role in the pathogenesis of duodenal ulcer and its complications. To evaluate this hypothesis, 35 patients with either duodenal ulcer bleeding (18 patients) or gastric outlet obstruction (17 patients) were investigated. Biopsies were taken from gastroduodenal tissues and secretions for prostaglandin E2 (PGE2) levels. These levels were compared to those taken from the same areas during a later endoscopy. A correlation was found between the severity of the clinical endoscopic findings and PGE2 levels. Increased levels of PGE2 were found in the quiescent phase and decreased levels found during the deteriorated phase. These differences of PGE2 levels were found to be of significant value (P less than 0.002). Furthermore, the patients in which the PGE2 levels were decreased at second endoscopy needed surgery. PGE2 may, thus, be a factor in duodenal ulcer pathogenesis and its complications, and be used as a prognostic marker and guide.

Surgical palliation combined with synchronous therapy in pancreatic carcinoma.

Palliative surgery has a role in nonresectable pancreatic carcinoma. We attempted to evaluate the efficacy of palliative surgery followed by synchronous therapy in such cases. A group of 92 patients was studied. Sixty-six patients (Group 1) with biliary obstruction underwent surgical biliary bypass. Twenty-six patients (Group 2) underwent explorative laparotomy only. Thirty patients (45.5%) in Group 1 and 10 patients (38.5%) in Group 2 received synchronous therapy consisting of 10 intravenous administrations of 5-fluorouracil (750 mg/m2) over 8 h, followed 8 h later by radiation with 400 rads repeated every 4 days. The mean survival for the entire group was 8.9 months. Those in Group 1 who received synchronous therapy had a mean survival of 13.5 months; those who did not 8.9 months (P less than 0.01). Patients in Group 2 who received synchronous therapy survived with a mean of 5.4 months, those who did not 2.7 months (P less than 0.01). Toxicity due to synchronous therapy was minimal. Of the 40 patients receiving synchronous therapy, 37 suffered from abdominal pains prior to initiation of therapy, 29 of which (78.4%) were free of pain upon completion of therapy. In nonresectable carcinoma of the pancreas, surgical palliation provides an acceptable survival. When combined with synchronous therapy, it results in prolonged survival and an ameliorated quality of life.

Prostaglandin E2 in pyloric stenosis.

Prostaglandins are presumed to have many cytoprotective properties that play a role in the pathogenesis of duodenal ulcer and its complications where decreased levels of prostaglandin E2 (PGE2) impair gastric motility, oppose ionic membrane influx, and enhance obstructive changes. These are just some of the mechanisms that may cause pyloric obstruction and may result from decreased PGE2 levels. To evaluate this hypothesis, 17 patients with duodenal ulcer complicated by pyloric stenosis were examined. Biopsy specimens were obtained from the duodenal bulb, ulcer margins, gastric antrum, fundus, and gastric secretions. Prostaglandin E2 levels were measured and compared with those taken from the same areas during a second endoscopy in a later quiescent or exacerbated phase. During the active phase of pyloric stenosis, decreased levels of PGE2 were found in the gastroduodenal tissues and secretions were compared with levels found during convalescence. These level differences were statistically significant. A correlation between the severity of the clinical and endoscopic findings and the PGE2 levels was found. A further decrease in PGE2 levels in the second endoscopy were indicative of the presence of scar tissue, representing an irreversible obstructive peptic disease.

Biliary colic treatment and acute cholecystitis prevention by prostaglandin inhibitor.

Sixty patients were treated in the emergency ward for biliary colic. Cholelithiasis was proven by ultrasonography. Twenty patients (group I) were treated by placebo. Twenty patients (group II) were treated by papaverine, and 20 patients were treated by diclofenac sodium (Voltaren) (group III). Twenty more patients (group IV) with low back pain (LBP) were treated with diclofenac sodium (Voltaren) as a control to assess the analgesic effect of Voltaren. Two interesting observations were made: Voltaren was proven more efficient for pain relief (P less than 0.002), and none of the patients treated with Voltaren were in need of hospitalization and immediate surgery. In comparison, nine patients of the other two groups progressed to acute cholecystitis and needed surgical intervention. The possible anticolic and anti-biliary inflammation properties and the indications for use of Voltaren are discussed.

Leukergy in inflammatory bowel disease.

In the phenomenon of leukergy, white blood cells agglomerate in peripheral blood slides. This agglomeration has been described in inflammatory infections of various causes. This study assesses this phenomenon in inflammatory bowel disease. A correlation was found between the severity of inflammatory bowel disease activity and the percentage of leukergy. Leukergy was found to parallel the clinical and endoscopic findings of inflammatory bowel disease. Furthermore, leukergy was found to be more accurate than white blood count and erythrocyte sedimentation rate. It is also found to accurately assess the course of the disease when clinical and other laboratory tests were masked by steroid and antibiotic administrations. Leukergy is a quick, inexpensive test that can easily be performed at the patient's bedside.

Prevention and treatment of urinary retention and infection after surgical treatment of the colon and rectum with alpha adrenergic blockers.

In a retrospective study, 153 male patients who underwent surgical treatment of the colon and rectum presented with complaints indicating obstruction of the prostate gland and clear indications for preoperative administration of alpha-blockers. Between the years 1982 and 1984, the alpha-blocker phenoxybenzamine hydrochloride (PB-HCl) was prohibited from use by the Ministry of Health because of suspicions of its carcinogenic properties. This enabled a comparative study between two groups of patients. Seventy-five patients (group 2a1) were prohibited from receiving PB-HCl. Urinary retention occurred in 54.7 per cent and urinary tract infection in 65.3 per cent. Seventy-eight patients (group 2a2) were administered PB-HCl. In this group of patients, urinary retention occurred in 19.2 per cent and urinary tract infection in 15.4 per cent. Forty-one patients with urinary retention of group 2a1 who were not administered PB-HCl were compared with 50 patients with urinary retention (group 3) who received PB-HCl. Spontaneous relief was observed in 21.9 and 76.0 per cent, respectively. PB-HCl was shown to be extremely effective, both as a preventative and therapeutic agent, when complaints indicating the prostate gland are present in patients undergoing surgical treatment of the colon and rectum.

Intrarectal bypass graft in low anterior resection and sigmoid obstruction--an experimental study.

The most common causes for morbidity and mortality in colorectal resections are anastomotic leaks. In low anterior resection, the incidence of anastomotic leakage ranges from 17 to 50%. With the use of the stapler technique, leakage incidence rate remains high and ranges from 10 to 25%. Colostomy formation and closures are associated with considerable morbidity and mortality. Due to the high incidence of anastomotic leakage rate in low anterior resection, and the additional complications of diverting colostomy formation and closure, the use of a rectal stent-intrarectal bypass graft has been instituted. This is carried out by means of a silastic graft, which prevents the fecal stream and gas pressure from coming into contact with the anastomotic site at the low rectum. The efficacy of intrarectal bypass graft was examined in two high-risk surgical situations, the first in very low anterior resection and the other, after early sigmoid obstruction. In both situations the intrarectal bypass graft provided for a safe anastomosis. Even when dehiscence and early obstructions occur, the tube may prevent leakage. This procedure presents effective practical implications which obviate the need for a proximal colostomy formation, thereby eliminating the physical and psychological stress that accompanies colostomies.

Hidden loop jejunostomy--an experimental model.

A hidden loop jejunostomy, the placement of a proximal small bowel loop under the skin of dogs, is described. A feeding tube was inserted in the loop at a later date, which enabled feeding for at least 6 weeks. This procedure was well tolerated by the 10 dogs involved in this experimental model. It should be considered as a possible surgical procedure at initial explorative laparotomy in patients with advanced cancer originating at the gastric cardia or esophagogastric junction.