Diminished activity of a chemotactic inhibitor in synovial fluids from patients with familial Mediterranean fever

Synovial fluids from patients with osteoarthritis contain a chemotactic inhibitor that acts by antagonizing the complement-derived chemotactic anaphylotoxin, C5a. The activity of this inhibitor in synovial fluids from patients with several forms of inflammatory arthritis (rheumatoid arthritis, systemic lupus erythematosus, ankylosing spondylitis, and gout) were comparable to the activity present in osteoarthritic synovial fluids. In contrast, levels of inhibitory activity in synovial fluids from 9 patients with familial Mediterranean fever were decreased to less than 20% of those found in osteoarthritis fluids. The possibility was considered that the diminished inhibitory activity in fluids from patients with familial Mediterranean fever plays a part in the pathogenesis of the inflammatory attacks characteristic of this disease.     

Current strategies for retarding progression of renal disease

Physiological insights gained in the 1980s into mechanisms of disease progression in experimental chronic nephropathies established the basis for therapeutic interventions to retard the progression of chronic renal disease in humans. In the 1990s, several large-scale clinical trials have confirmed the renoprotective effects of angiotensin-converting enzyme inhibitors in diabetic and nondiabetic nephropathies. Other studies have afforded strong support for the efficacy of dietary protein restriction in certain settings and underscored the importance of blood pressure control in proteinuric individuals. These interventions form the core of current strategies designed to preserve kidney function in patients with chronic renal disease.     

The impact of chorionicity on umbilical cord acid–base values in twin gestations

Objective: To evaluate the impact of chorionicity on inter-twin differences in acid–base status at birth.

Methods: Records for twin pregnancies delivered at ??24 weeks' gestation from 1 January 1990 to 31 June 2000 were reviewed. Collected data included maternal demographics, gestational age, fetal presentation, anesthesia, delivery mode, inter-twin interval, umbilical artery (UA) and venous (UV) acid–base values, Apgar scores and birth weights. The influence of chorionicity on umbilical cord biochemistry was evaluated. (p?<?0.05 was considered significant.)

Results: Analysis was carried out in 87 twin pairs (29 monochorionic, MC; and 58 dichorionic, DC). MC and DC twins were similar in maternal age (25.5 vs. 28.2 years), estimated gestational age (33.7 vs. 33.6 weeks), Cesarean delivery (55.2 vs. 52.6%), delivery interval (10 v s.5?min) and respective birth weights (twin A,1882 vs. 1981; and twin B,1828 vs. 1872?g). MC first twins had a higher UA pH (7.31?±?0.05 vs. 7.26?±?0.08; p?=?0.0005) than DC first twins. MC first and second twins had higher UA and UV bicarbonate levels than their DC counterparts (ΔpH?=?21.7?±?5.1 vs. 18.5?±?3.1?mmol/l and 22.0?±?3.5 vs. 19.6?±?2.5?mmol/l, respectively; p?=?0.003). MC twins were more discordant in UA pH than DC twins (ΔpH?=?0.043?±?0.09 vs. 0.003?±?0.07; p?=?0.009). MC and DC twins had a similar venous pH (ΔpH?=?0.01?±?0.06 vs. 0.02?±?0.06; p?=?0.5).

Conclusions: There is a significant association between placental chorionicity and umbilical cord biochemistry in twins. Although it is possible that the mechanism of this finding is related to placental angioarchitecture, it is unlikely to be a result of simple mixing of blood volumes between twins. The physiology of underlying processes requires further study.     

Level of agreement on postoperative complications after one-stage hypospadias correction comparing medical records and parent reports

PurposeTo analyze agreement on postoperative complications after hypospadias surgery according to medical records and parents' reports.Materials & methodsIn this retrospective cohort study, data were collected from 409 children who received an initial one-stage hypospadias correction in the Radboudumc, The Netherlands.Postoperative complications according to medical records were compared with parent-reported complications in an online questionnaire. Main complications studied were wound-related complications, urinary tract infections, fistulas, stenosis, and prepuce-related complications. Agreement was determined by Cohen's kappa coefficient.ResultsSlightly less complications were mentioned in medical records (37%) compared to parents' reports (42%). Overall agreement was moderate (κ = 0.50, 95% confidence interval (CI):0.41–0.59), but poor for some specific complications. Agreement was higher for complications that needed reoperation compared to when no reoperation was performed (κ = 0.53, 95% CI: 0.43–0.62 and κ = 0.18, 95% CI: 0.06–0.31) and for patients with recent surgery (< 5 years before questionnaire completion) compared to less recent surgeries (κ = 0.69, 95% CI: 0.55–0.84 and κ = 0.43, 95% CI: 0.33–0.54).ConclusionsAgreement on complications according to medical records and parents' reports was poor to moderate, but better after reoperation and more recent surgery. Some complications mentioned in medical records were missing from parents' reports and the other way around. Better agreement will give physicians and parents a more reliable view on postoperative outcome after hypospadias surgery.Type of studyDiagnostic test.Level of evidenceLevel III.     

Multiple transverse folds in the gastric antrum

Fine transverse folds of the esophagus are well described as a transient motor phenomenon seen in patients with or without gastroesophageal reflux. They appear to be due to contraction of the muscularis mucosa and are thought to have little, if any, significance. We have observed similar transverse folds in the gastric antrum during double-contrast upper gastrointestinal series in five patients. In contrast to the transient nature of the folds in the esophagus, these gastric folds were more persistent. They were seen in multiple spot images with the gastric antrum distended or partially collapsed and even during peristalsis. In follow-up studies, the folds were repeatedly demonstrated in two patients. Two patients had associated gastric polyps with histologic evidence of chronic gastritis; however, the antral mucosa appeared normal on endoscopy. The remaining three patients had no associated gastric disease. The pathophysiologic significance of these folds is yet to be determined; however, they appear to be clinically insignificant.     

Severe aplastic anemia: a prospective study of the effect of early marrow transplantation on acute mortality

A prospective randomized trial of therapy for severe aplastic anemia was designed to compare early bone marrow transplantation with conventional treatments. All patients with a sibling matched at the major histocompatibility region were transplanted. Transplantation was performed with 17-100 (median 33) days of original diagnosis. Conventional treatments included transfusion support with or without androgens. Twenty-four of 36 patients intered on the transplant arm are alive after 4-20 (median 9) mo with full marrow reconstitution. Only two are limited by chronic graft-versus-host disease. In contrast only 12 of 31 conventionally treated patients are alive. Six of these survivors have improved, five incompletely. The 19 nontransplant deaths have occurred within 1-11 (median 3) mo of diagnosis. Compared to nontransplant regimens, early transplantation more effectively restores normal marrow function and decreases the acute mortality of severe marrow aplasia (p = 0.006). Pending longer follow-up, early marrow transplantation appears to be the most effective available treatment for severe aplastic anemia.     

The genotype and phenotype of T cell and non-T, non-B acute lymphoblastic leukemia

We used Southern blot analysis to investigate the T cell receptor and immunoglobulin (Ig) genes in tumor DNA derived from 13 patients with acute lymphoblastic leukemia (ALL). Rearrangement of both alleles of the T cell receptor beta chain gene was demonstrated in the three individuals with T cell ALL, while the Ig genes remained in germline configuration. In contrast, in nine of ten patients with non-T, non-B ALL (seven common ALL antigen [CALLA]-positive), Ig genes were rearranged while the T cell receptor genes were intact: the noteworthy exception was a CALLA-positive individual in whom one allele of the T cell receptor was rearranged and in whom a minor rearranged Ig band was detected as well. This exception indicates that CALLA-positive non-T, non-B ALL includes proliferations that have undergone an initial genetic step toward T cell differentiation. With probes now available for both the Ig and T cell receptor genes, analysis of genomic tumor DNA is useful in all variants of ALL.     

Ovarian mucinous cystadenocarcinoma with virilization

BACKGROUND: Ovarian neoplasms, other than sex cord--stromal tumors, are rare causes of hyperandrogenism. Only two cases of primary mucinous carcinomas associated with virilization have been reported. CASE: A 50-year-old female was referred to our clinic with a large pelvic mass. On examination she had significant facial hirsutism, clitoromegaly, and male pattern pubic hair growth. Serum levels of testosterone and dihydroepiandrosterone sulfate were elevated. A 30-cm, multilocular, solid and cystic, left ovarian mass was resected. Histology revealed moderately to poorly differentiated mucinous cystadenocarcinoma. The ovarian stroma contained florid proliferation of luteinized cells. The right ovary showed cortical stromal hyperplasia. Abnormal hormone values normalized 10 days postoperatively. CONCLUSION: We report a rare case of mucinous cystadenocarcinoma with virilization, review the literature, and discuss the mechanisms of hormone production by these tumors.     

Body fat distribution measured with CT: correlations in healthy subjects, patients with anorexia nervosa, and patients with Cushing syndrome

Computed tomography (CT) was used to study fat distribution in three groups of women of comparable age: 39 healthy volunteers, 15 patients with anorexia nervosa, and seven with Cushing syndrome. Patients with anorexia nervosa had a fivefold decrease in subcutaneous fat and only a twofold decrease in intraabdominal fat compared with the values for the volunteers. Patients with Cushing syndrome had less than a twofold increase in subcutaneous fat and greater than a fivefold increase in intraabdominal fat compared with values for the healthy subjects. These findings suggest that fat in different body compartments responds differently to disease processes and that CT can be used to measure these changes.