Generalized verrucosis in a patient with GATA2 deficiency.

Generalized verrucosis is a characteristic of several genetic and immunodeficiency disorders including epidermodysplasia verruciformis; warts, hypogammaglobulinaemia, infections and myelokathexis (WHIM) syndrome; warts, immunodeficiency, lymphoedema and anogenital dysplasia (WILD) syndrome; severe combined immune deficiency and HIV, among others. In recent years, it has been consistently recognized in patients with GATA2 deficiency, a novel immunodeficiency syndrome characterized by monocytopenia, B-cell and natural killer-cell lymphopenia, and a tendency to develop myeloid leukaemias and disseminated mycobacterial, human papillomavirus (HPV) and opportunistic fungal infections. Mutations in GATA2 cause haploinsufficiency and track in families as an autosomal dominant immunodeficiency. GATA2 is a transcription factor involved in early haematopoietic differentiation and lymphatic and vascular development. We describe a case of generalized verrucosis with HPV type 57 presenting in a young man with GATA2 deficiency. GATA2 deficiency is a novel dominant immunodeficiency that is often recognized later in life and should be considered in the differential diagnosis of patients with generalized verrucosis.

The association between epilation and corneal opacity among eyes with trachomatous trichiasis.

AIM: To examine the association between epilation and corneal opacity (CO) among trichiasis patients presenting for surgery. METHODS: Cross sectional data from the STAR trial were utilised. Patients presenting for trichiasis surgery in Wolayta Zone, Ethiopia, were evaluated for current trichiasis status. Number of inturned lashes, evidence of epilation, level of entropion, trichiasis duration, and CO were collected. The primary outcome was prevalence of CO, stratified by entropion and epilation status. RESULTS: Approximately 10% of eyes with mild entropion had CO, regardless of epilation status. Among eyes with moderate entropion, epilated eyes were less likely to have CO than non-epilated eyes (21% v 34% p = 0.002). The same association was seen in eyes with severe entropion: 43% of epilated eyes while 74% of non-epilated eyes had CO (p<0.0001). Presence of CO increased with age. Adjusted models showed a protective effect of epilation in eyes with moderate or severe entropion (OR: 0.51; 95% CI: 0.32 to 0.83 and OR: 0.24; 95% CI: 0.13 to 0.45, respectively). Among eyes with mild entropion there was no difference in the prevalence of CO comparing eyes that were epilated with those that were not epilated. CONCLUSION: Entropion was the most significant predictor of CO. Cross sectional associations suggest that epilation may not be helpful for eyes with mild entropion, but may offer protection against CO in eyes with moderate to severe entropion. Epilation should not be a substitute for trichiasis surgery, however, as 43% of eyes with severe entropion that were epilated still had CO.

Characteristics of trichiasis patients presenting for surgery in rural Ethiopia.

AIMS: To determine the characteristics of trichiasis patients presenting for surgery in Wolayta Zone of Ethiopia. METHODS: Patients referred for trichiasis surgery by community health agents were evaluated by trained integrated eye care workers (IECWs) for the presence of trichiasis, locations of inturned lashes, severity of trichiasis, corneal opacity, and visual acuity. RESULTS: 1635 individuals with trichiasis presented for surgery. 82% had bilateral trichiasis; 91% of patients reported trichiasis duration of >2 years. Epilation was practised by over three fourths of the study subjects. A high proportion of patients tested positive for ocular Chlamydia trachomatis at presentation. 17% had monocular blindness and 8% were binocularly blind. Corneal opacity was highly associated with the trichiasis duration and severity and visual loss was associated with corneal opacity. CONCLUSION: Severe trichiasis reflects the magnitude of the trachoma problem in Ethiopia. Visual impairment due to trichiasis is highly associated with disease severity and duration. Early intervention to correct trichiasis before it become severe is recommended to prevent visual impairment.

Interferon and ribavirin vs interferon alone in the re-treatment of chronic hepatitis C previously nonresponsive to interferon: A meta-analysis of randomized trials.

CONTEXT: Hepatitis C is the leading cause of chronic liver disease in the United States. Several trials have found that interferon and ribavirin combination therapy is more efficacious than interferon monotherapy for previously untreated patients and those who relapsed after prior interferon monotherapy, but its effectiveness for nonresponders to prior interferon monotherapy is unclear. OBJECTIVE: To assess the efficacy and safety of interferon and ribavirin vs interferon alone for treatment of patients with chronic hepatitis C who previously did not respond to interferon monotherapy. DATA SOURCES: A systematic search was performed using MEDLINE and the Science Citation Index for publications from 1966 to December 1999. A manual reference search and a manual review of relevant specialty journals also were performed, and input from clinical hepatology experts was sought. STUDY SELECTION: Included studies were randomized, controlled clinical trials comparing interferon and ribavirin with interferon alone and reporting virological and biochemical outcomes after a follow-up period. Of 50 identified studies, 12 trials (941 patients) were included in the analysis. DATA EXTRACTION: Two investigators reviewed trials independently for methods, inclusion and exclusion criteria, and outcomes. Disagreements were resolved by discussion. Abstracted data included study and patient characteristics and virological, biochemical, and histological outcomes. A quality evaluation questionnaire was used to score studies. DATA SYNTHESIS: The pooled virological response rate for combination therapy was 14% (95% confidence interval [CI], 11%-17%), with a risk difference favoring combination therapy of 7% (95% CI, 2%-13%). Use of interferon alfa-2a/2b and ribavirin, 1000 to 1200 mg/d, was associated with a pooled virological response rate of 18% and a risk difference of 16% (95% CI, 11%-21%). When interferon alfa-n/n3 and a lower dosage of ribavirin (600-800 mg/d) were used, the risk difference was 0% (95% CI, -7% to 7%). Combination therapy was associated with more adverse effects and an increased rate of discontinuation of treatment compared with interferon monotherapy. CONCLUSIONS: For chronic hepatitis C that is nonresponsive to prior interferon monotherapy, combination therapy is more effective than re-treatment with interferon alone. Response rates remain less than 20% even in the most responsive subgroups, demonstrating a need for better therapeutic options.