Long-term results of central venous access devices in children with haemophilia.

AIMS: Central venous access devices (CVADs) are often used to provide reliable venous access for factor VIII administration in children with haemophilia. This study investigates their long-term outcome. METHODS: A retrospective cohort study of 44 CVADs inserted into 31 children with haemophilia at a single centre between 1991 and 2006. RESULTS: Eight (18%) CVADs are still in place and working well. Fourteen (31.8%) were removed when the child was able to return to peripheral vascular access. Twenty-two (50%) were removed because of complications, most of these children needing a replacement CVAD. The median duration that the first CVAD was in place was 51 months. There were no life-threatening complications. CONCLUSIONS: CVADs function well in children with haemophilia for a long time, with a relatively low complication rate, and can tide a child over a difficult period for vascular access.

An audit of immunisation status of sickle cell patients in Coventry, UK.

Protection against infection is a key aim of any care programme for patients with sickle cell disorders, and is one of the stated objectives of the UK national neonatal screening programme. An audit of the immunisation status of the 58 sickle cell patients living in Coventry was carried out against UK national guidance (2006). Among 25 children (aged < or =16 years), 14 (56%) had complete immunisation against pneumococcus, Haemophilus influenzae type b (Hib) and meningococcus group C (MenC), with eight (32%) having some coverage, and three (12%) having none at all. Among 33 adults, only four patients (12%) had complete coverage, with up-to-date coverage against pneumococcus 21%, Hib 18% and MenC 15%. Current (ie, within the last year) immunisation against viral influenza was found in only 12% of adults and 8% of children. These rates are similar to those found in other UK studies. If the potential benefits of neonatal screening are to be realised, an effective immunisation programme is essential. There are key roles for haemoglobinopathy nurse specialists, and the proposed national haemoglobinopathy database, in improving the rates of immunisation coverage.

Auditing the new decentralised oral treatment regimens in Malawi.

SETTING: All 44 non-private hospitals in Malawi treating tuberculosis (TB) cases in which oral regimens were used allowing patients during the initial phase to receive directly observed treatment (DOT) from health centres or guardians at home. OBJECTIVES: A country-wide audit of the oral regimens to determine: 1) TB ward bed occupancy rates, 2) patient DOT options, 3) patients' knowledge of treatment and 4) treatment outcomes compared to those obtained with previous treatment regimens. DESIGN: Retrospective data collection using registers and treatment cards. Prospective interviews with patients. Inspections of TB wards. RESULTS: There were 1513 TB beds occupied by 807 (53%) TB patients. Over 50% of 4793 patients registered with different types of TB chose guardian-based DOT. For 266 patients with pulmonary TB the correct knowledge about total duration of treatment (45%), all three DOT options (62%) and the months for giving follow-up sputum (16%), was poor. There were differences in treatment outcomes between TB patients on oral compared with previous regimens. With oral regimens, rates of unknown outcome were high. CONCLUSION: Oral treatment regimens are associated with reduced bed occupancy rates on TB wards. However, rates of unknown outcome are increased, and TB control is therefore weakened.

Management and outcome of tuberculosis patients who fail treatment under routine programme conditions in Malawi.

SETTING: All 43 non-private hospitals (three central, 22 district and 18 mission) in Malawi that registered and treated TB cases between 1 July 1999 and 30 June 2000. OBJECTIVES: To determine 1) the number of new smear-positive PTB patients who failed treatment, 2) the management of patients who failed, 3) their treatment outcome and 4) culture and drug sensitivity results. DESIGN: Retrospective data collection using TB registers and laboratory culture and drug sensitivity registers. RESULTS: Ninety patients failed treatment, 60 (67%) at 5 months and 30 (33%) at the end of treatment. Sixty-four (71%) failure patients were registered and commenced on anti-tuberculosis treatment. Of these, 95% were registered in the same hospital as before, 89% were given a different TB registration number, 67% were correctly registered as 'failures' and 61% were treated within one month of failing the previous regimen. Forty-eight (75%) re-treated patients were cured. Only 31 (34%) of the 90 patients had sputum sent for culture and drug sensitivity testing. In 11 patients with cultures of M. tuberculosis, eight were fully sensitive and three had mono-resistance to isoniazid. CONCLUSION: While the outcome of failure patients who start retreatment is good, there are several programmatic deficiencies that need to be corrected.

A prospective study of neonatal inguinal herniotomy: the problem of the postoperative hydrocele.

Previous studies have reported an increased incidence of complications following neonatal inguinal herniotomy (IH) in boys. The incidence and natural history of postoperative hydrocele in such cases has not been described. A prospective follow-up study of a consecutive series of male infants weighing less than 3 kg at the time of IH was undertaken. Regular follow-up examinations were scheduled for at least 1 year. Thirty-eight boys weighing less than 3 kg underwent IH during an 18-month period. One subsequently died from complications of prematurity. Complete follow-up data were available for 29/37 (78%) patients, yielding a total of 46 IHs. There were 2 recurrent hernias (4%), 2 unequivocally atrophic testes (4%), and 1 iatrogenic testicular ascent (2%). Five ipsilateral hydroceles complicated the postoperative course of 4 boys (14% of patients, 11% of herniotomies). Two of these were explored, but in neither case was a recurrent/residual patent processus vaginalis found. One hydrocele was aspirated without recurrence, and the remaining 2 resolved spontaneously. The complication rate in small infants undergoing neonatal IH is significantly higher than in older boys. The presence of a hydrocele after neonatal IH may simply reflect the accumulation of fluid in the distal hernia sac and, provided there is no evidence of a recurrent inguinal hernia, an expectant approach is recommended.

The relentless spread of tuberculosis in Zambia--trends over the past 37 years (1964-2000).

OBJECTIVE: To review trends in the rates of tuberculosis (TB) case notifications over a 37-year period. DESIGN: A retrospective study of Ministry of Health records on TB notifications between 1 January 1964 and 31 December 2000. SETTING: Zambia, sub-Saharan Africa. METHODS: Retrospective analysis of case-notification data for TB of the Zambia Ministry of Health annual returns. OUTCOME MEASURES: Annual TB case-notification rates and trends over the past 37 years. RESULTS: TB case-notification data from 1964 to 2000 show a 12-fold increase over the past two decades, and apparent gains in controlling TB seen in the 1960s and 1970s have been reversed over the past two decades. A stable situation during the period 1964-1984 (case-notification rate remained around 100 per 100,000 population) was followed by an exponential increase since the mid-1980s. The absolute number of new TB cases increased from 8,246 in 1985 (124/100,000) to 38,863 (409/100,000) in 1996 and 52,000 (512/100,000) in 2000. Comparison of case-notification rates over the past 2 decades with neighbouring countries (Zimbabwe, Malawi and Tanzania) show that Zambia has one of the highest case-notification rates in the region. CONCLUSIONS: Zambia, like many countries in Africa, is in the midst of a serious TB epidemic and there are no signs that it is abating. This increase was most likely due to the impact of the HIV/AIDS epidemic and subsequent breakdown of TB services. Concerted donor-government efforts should invest appropriately in long-term plans for TB control.

Long-term survival after spontaneous rupture of a malignant rhabdoid tumor of the liver.

A 13-month-old girl presented with a large malignant rhabdoid liver tumor that ruptured soon after admission. Six years after an emergency right hepatectomy and subsequent chemotherapy (ifosfamide, vincristine, and actinomycin D), she remains well and disease free. Previously, these rare tumors invariably have been fatal and resistant to multimodal therapy. This is the first report of long-term survival of a patient with a malignant rhabdoid liver tumor.

Hirschsprung's disease: problems with transition-zone pull-through.

BACKGROUND/PURPOSE: It is generally accepted that if surgery for Hirschsprung's disease is to be successful, ganglionic bowel must be anastomosed to the lower rectum or anal canal. Above the aganglionic distal bowel lies a transition zone (TZ) where more subtle abnormalities of innervation are apparent. The significance of this transition zone in respect to the functional outcome of surgery has received little attention. The aim of this study was to identify the incidence of transition zone pull-through (TZPT) in a cohort of children who underwent surgery for Hirschsprung's disease, to identify the reasons why TZPTs occurred, and to identify the functional consequences. The authors report the long-term outcome of these children with emphasis on bowel function and the results of subsequent surgery. METHODS: A Retrospective study was conducted of children treated at a single institution from 1979 through 1994. TZPT patients were subject to detailed review of surgical records and histopathologic material. RESULTS: Thirteen children were identified with a TZPT. In 12 cases, histopathologic errors contributed to the TZPT: in 5 cases this was caused by single point biopsies missing an asymmetrical TZ, whereas in 7 cases the histopathologic features of the TZ were not recognized. In 1 case the TZPT was caused by surgical error. As a consequence of the TZPT 7 children underwent repeat pull-through. One child is fully continent, one has daytime fecal continence, and 2 others are incontinent. Two children have permanent stomas. One child is clean with antegrade colonic washouts. Repeat pull-throughs were not attempted in 6 children. Two children have achieved full continence, 2 have permanent stomas, 1 is clean with antegrade colonic washouts, and 1 child receives regular suppositories. CONCLUSIONS: Transition zone pull-throughs occurred because of a combination of surgical and histopathologic errors. The transition zone may follow an asymmetric course around the circumference of the bowel and may be missed if single-point extramucosal biopsy specimens are taken. Recognition of the subtle histologic features of the transition zone requires an experienced pathologist. The functional consequences of a TZPT are severe, with symptoms of constipation, diarrhea, and incontinence. The results of revisional pull-through were disappointing. Serious consideration should be given to alternative procedures such as the antegrade continence enema operation.

Factors on farms in eastern Australia associated with the development of tibiotarsal rotation in ostrich chicks.

OBJECTIVES: To determine the incidence of tibiotarsal rotation (TTR) in ostrich chicks and to identify factors on farms associated with the development of TTR. DESIGN: A cross-sectional study using a personal interview. PROCEDURE: During a single visit to 31 farms, data were collected about a defined cohort of chicks, and about farm-related factors that may be associated with the development of TTR. Farms were categorised for TTR status according to the proportion of the chick cohort that survived to 10 weeks of age without developing TTR. Chick performance was measured using descriptive epidemiological methods and univariable analyses were conducted to identify unconditional associations between TTR status and farm-related factors. RESULTS: Ninety-six of 931 ostrich chicks from 21 (68%) farms developed TTR during the first 10 weeks following hatch. Lower limb deformities were the most common cause of death in chicks between 3 and 10 weeks of age. On seven (23%) study farms, where less than 87% of young chicks survived to 10 weeks without developing this condition, TTR was considered a serious problem. Twelve farm-related factors were associated with farm TTR status, including eight chick-related variables (aspects of nutrition, pen design and management) and four farm-related variables (related to the number of veterinary visits, farm location, number of rainy days and the person most closely involved with chick raising. CONCLUSION: The results confirm a continuing problem of young chick wastage in eastern Australia. TTR was an important cause of mortality in farmed ostrich chicks during the first 10 weeks after hatch. The factors that producers could address to reduce the incidence of TTR include pen design, access to water and nutrition.

Molluscicidal activity in the seeds of Millettia thonningii (Leguminosae: Papilionoideae).

Seeds of the leguminous plant Millettia thonningii were shown to possess promising molluscicidal activity against Bulinus trunctatus. The size of the snails was an important determinant of their susceptibility to the molluscicide; specimens with shell lengths of 2 to 3 mm being more susceptible than snails 5 to 6 mm long.

Late results of valve replacement and factors influencing survival in patients with severe chronic mitral regurgitation.

Between July 1967 and September 1981 50 patients with isolated severe mitral regurgitation underwent mitral valve replacement. There were 29 males and 21 females (mean age 52 years). The aetiology of the valve lesion was rheumatic in 14 patients (mean age 42 years) and non-rheumatic in 36 patients (mean age 56 years). At the time of operation 36 patients (72%) were in class 3 or 4 of the New York Heart Association classification (mean duration of symptoms 20 months). Pre-operative ejection fraction was normal in only four patients (8%) and was below 0.50 in 27 patients (54%). There were two early deaths (4%) within one month of operation, and 17 late deaths (34%) during a follow-up period of four months to 10 years (mean 43 months). Actuarial analysis showed a 71% survival at five years and a 62% survival at 10 years after valve replacement. Of the 31 current survivors, 22 (71%) are in class 1 of the New York Heart Association classification, and all but two patients showed significant improvement in symptoms. Significant morbidity after operation occurred in 10 patients (20%) and was largely related to problems with anticoagulant control. Analysis of factors which may influence survival, showed that age greater than 55 years and parameters of left ventricular geometry, demonstrated by angiography, were the major determinants of survival.

Abnormal lymphocyte response to u.v. radiation in multiple skin cancer.

The lymphocyte response to u.v. radiation (254 nm) was investigated by two different methods in 29 unselected patients with multiple epidermal cancer. The u.v.-induced DNA synthesis was determined as the increase in incorporation of [3H]thymidine in irradiated cells compared with nonirradiated cells after incubation for 2 h. The u.v. tolerance was measured as the u.v. dose necessary for 50% reduction in phytohemagglutinin-stimulated lymphocyte proliferation. Patients with both squamous cell differentiated tumors and basal cell carcinomas had very high u.v.-induced DNA synthesis values (p less than 0.01, when compared with patients with basal cell carcinoma only and p less than 0.005, when compared with controls). The u.v. tolerance in patient lymphocytes was considerably lower than in control lymphocytes (p less than 0.001), with the lowest values occurring in patients with clinical sun intolerance (p = 0.05, when compared with the remaining patients). These investigations may be of predictive value in skin carcinogenesis.