Unseen blindness, unheard deafness, and unrecorded death and disability: congenital rubella in Kumasi, Ghana

OBJECTIVES: Although rubella serosusceptibility among women of reproductive age in West Africa ranges from 10% to 30%, congenital rubella syndrome has not been reported. In Ghana, rubella immunization and serologic testing are unavailable. Our objectives were to identify congenital rubella syndrome cases, ascertain rubella antibody seroprevalence during pregnancy, and recommend strategies for congenital rubella syndrome surveillance. METHODS: Congenital rubella syndrome cases were identified through prospective surveillance and retrospective surveys of hospital records. A rubella serosurvey of pregnant urban and rural women was performed. RESULTS: Eighteen infants born within a 5-month period met the congenital rubella syndrome case definitions, coinciding with a 9-fold increase in presentation of infantile congenital cataract. The congenital rubella syndrome rate for this otherwise unrecorded rubella epidemic was conservatively estimated to be 0.8 per 1000 live births. A postepidemic rubella immunity rate of 92.6% was documented among 405 pregnant women; susceptibility was significantly associated with younger age (P = .000) and ethnicity (northern tribes, P = .024). CONCLUSIONS: Congenital rubella syndrome occurs in Ghana but is not reported. Information about congenital rubella syndrome and rubella in sub-Saharan Africa is needed to evaluate inclusion of rubella vaccine in proposed measles control campaigns.     

Progress in clinical neurosciences: Status epilepticus: a critical review of management options

Although generalized tonic-clonic status epilepticus (SE) is frequently seen, an evidence-based approach to management is limited by a lack of randomized clinical studies. Clinical practice, therefore, relies on a combination of expert recommendations, local hospital guidelines and dogma based on individual preference and past successes. This review explores selected and controversial aspects of SE in adults and provides a critical appraisal of currently recommended management strategies.     

Post-intubation pulmonary function test in Guillain-Barré syndrome

The value of daily postintubation respiratory function tests in predicting duration of ventilation in 37 mechanically ventilated patients with Guillain-Barré syndrome (GBS) was studied. Patients ventilated for less than 3 weeks were compared with those ventilated more than 3 weeks. Daily vital capacity and maximal inspiratory and expiratory pressures were summed to an integrated pulmonary function (PF) score. We calculated the PF ratio, which represents the PF score at day 12 after intubation divided by the PF score on the day of intubation. The PF ratio was greater than 1 in all 10 patients ventilated less than 3 weeks and was less than 1 in 19 of 27 patients ventilated for longer (P = 0.0001, Fisher exact test). The sensitivity of a PF ratio less than 1 for predicting duration of ventilation greater than 3 weeks was 70%; the specificity and positive predictive value were 100%. This study thus suggests that serial postintubation respiratory tests can provide a measure of respiratory status in patients with GBS. These parameters may help predict duration of ventilation and need for tracheostomy. If, at day 12, the PF ratio is less than 1, it is highly unlikely that patients will be weaned within 3 weeks, and tracheostomy should be performed. If the ratio is greater than 1, tracheostomy should be deferred, because a substantial proportion of these patients may be successfully weaned from the ventilator within 3 weeks.     

Long-term outcome in patients with Guillain-Barré syndrome requiring mechanical ventilation

OBJECTIVE: To analyze long-term recovery and predictors of outcome in patients with Guillain-Barré syndrome (GBS) requiring mechanical ventilation. METHODS: The clinical and electrophysiologic data of 114 patients with GBS admitted to the intensive care unit between 1976 and 1996 (60 mechanically ventilated, 54 nonventilated) were reviewed. Functional disability and predictors of outcome were determined at 1 year and at maximal recovery using the Hughes scale. Good outcome was defined as ability to ambulate without assistance; poor outcome was defined as inability to ambulate independently. RESULTS: Mechanical ventilation was required in 81% of patients with a poor outcome. Mortality was 20% in patients ventilated for GBS. However, ventilated patients who survived did well, with 79% eventually regaining independent ambulation. Nineteen percent of patients improved at least one functional grade beyond 1 year. Univariate predictors of poor maximal recovery in ventilated GBS patients were increased age (p < 0.001)), upper limb paralysis (p = 0.004), duration of ventilation (p = 0.006), and delay of more than 2 days to transfer to a tertiary center (p < 0.001). However, only age (OR 1.99, p = 0.004) and delayed transfer (OR 19.8, p = 0.002) were independently predictive of poor outcome on multivariate analysis. CONCLUSION: Mechanically ventilated patients constitute the majority of GBS patients with a poor outcome, and mortality remains substantial in this subgroup (20%). Although recovery from severe GBS may be prolonged, most survivors regain independent ambulation.     

Chemoprevention of spontaneous tumorigenesis in nullizygous p53- deficient mice by dehydroepiandrosterone and its analog 16alpha-fluoro- 5-androsten-17-one

Transgenic mice with both alleles of the p53 tumor suppressor gene product 'knocked out' by gene targeting are susceptible to early development of tumors, chiefly lymphomas and sarcomas. Compared with the control group, administration of dehydroepiandrosterone (DHEA) at 0.3% of the diet to male p53-deficient mice extended their lifespan by delaying death due to neoplasms (from 105 to 166 days on study, P = 0.002), primarily by suppressing lymphoblastic lymphoma (from 45 to 6% of neoplastic deaths, P = 0.010). Treatment with a synthetic DHEA analog, 16alpha-fluoro-5-androsten-17-one (compound 8354), at 0.15% of the diet also increased lifespan, to 140 days for mice that developed tumors (P = 0.037). The effects of these steroids on lifespan and tumor development did not appear to be strongly related to inhibition of food consumption and weight gain, in that a group pair-fed with control diet to the reduced food consumption of the DHEA-treated group developed and died of the same types of neoplasms at the same rate as the controls fed ad libitum. The chemopreventive effect of these steroids has been proposed to be due to suppression of DNA synthesis by inhibition of glucose 6-phosphate dehydrogenase, the rate-limiting enzyme of the pentose phosphate pathway. Although DHEA and its analog are strong non- competitive inhibitors of this enzyme in vitro, treatment with DHEA did not deplete cellular nucleotide pools in the liver, as would have been predicted. The chemopreventive effect of DHEA in this model may be due to steroid-induced thymic atrophy and suppression of T cell lymphoma, permitting these mice to survive long enough to develop tumors with longer latency.