Severe malabsorption in autoimmune polyendocrinopathy-candidosis-ectodermal dystrophy syndrome successfully treated with immunosuppression

A 15 year old boy with autoimmune polyendocrinopathy-candidosis-ectodermal dystrophy syndrome suffered recurrent episodes of severe intractable diarrhoea, steatorrhoea, and hypocalcaemia. The only treatment modality, which controlled the malabsorption syndrome, was immunosuppression with intravenous high dose methylprednisolone and oral methotrexate maintenance therapy.     

Cerebral venous sinus thrombosis

Cerebral venous sinus thrombosis is a challenging condition because of its variability of clinical symptoms and signs. It is very often unrecognised at initial presentation. All age groups can be affected. Large sinuses such as the superior sagittal sinus are most frequently involved. Extensive collateral circulation within the cerebral venous system allows for a significant degree of compensation in the early stages of thrombus formation. Systemic inflammatory diseases and inherited as well as acquired coagulation disorders are frequent causes, although in up to 30% of cases no underlying cause can be identified. The oral contraceptive pill appears to be an important additional risk factor. The spectrum of clinical presentations ranges from headache with papilloedema to focal deficit, seizures and coma. Magnetic resonance imaging with venography is the investigation of choice; computed tomography alone will miss a significant number of cases. It has now been conclusively shown that intravenous heparin is the first-line treatment for cerebral venous sinus thrombosis because of its efficacy, safety and feasability. Local thrombolysis may be indicated in cases of deterioration, despite adequate heparinisation. This should be followed by oral anticoagulation for 3-6 months. The prognosis of cerebral venous sinus thrombosis is generally favourable. A high index of clinical suspicion is needed to diagnose this uncommon condition so that appropriate treatment can be initiated.     

Toxic cardiogenic shock in a patient receiving weekly 24-h infusion of high-dose 5-fluorouracil and leucovorin

A 54-year-old man was treated with weekly 24-h infusion of high-dose 5-fluorouracil (2600 mg/m2) and leucovorin (100 mg/m2) for metastatic colon cancer. At first, he tolerated the treatment well and no significant toxicity was identified. After a total of eight courses of treatment, a stable disease was observed, but mild shortness of breath was found on occasion. The patient had no previous history of cardiac disease and the heart performance assessed by left ventricular ejection fraction before treatment was normal. Unfortunately, acute pulmonary edema with lethal cardiogenic shock occurred during the ninth course of treatment, in spite of intensive medical treatment. The chest X-ray showed extreme cardiomegaly. Repeated assessment of his heart function by echocardiogram and ventricular ejection fraction revealed a very poor cardiac performance. Toxic cardiogenic shock during weekly 24-h infusion of high-dose 5-fluorouracil and leucovorin is extremely rare. To the best of our knowledge, no case has been reported in the English literature. We report a case and the relevant literature about the incidence, clinical picture and possible pathophysiology on 5-fluorouracil-related cardioxicity is reviewed.      

Low dose oestrogen prophylaxis for recurrent urinary tract infections in elderly women

Objective To assess the efficacy of oral oestriol in the prevention of recurrent Urinary tract infections in elderly women.
Design Double-blind, randomised, parallel group, placebo controlled trial
Setting Urogynaecology Unit at King's College Hospital with some women recruited from the geriatric units of St. Pancras Hospital and Dulwich Hospital, London (UK).
Participants Seventy-two postmenopausal women older than 60 years of age (mean 73.2 years) suffering from recurrent urinary tract infections.
Intervention Oral oestriol (3 mg per day) or placebo for six months.
Main outcome measures Urinary tract infection rates.
Results The study was difficult to conduct because of its design and the age of the participants. Oral oestriol (3 mg per day) was not shown to be superior to placebo in the prevention of recurrent urinary tract infections, but both oestriol and placebo improved urinary symptoms during the trial.
Conclusion The power of the study might have been too low to detect a significant difference between the groups, or oral oestriol(3 mg per day) may have been either the wrong dose or the wrong route of administration for this indication.     

Ionic permeability of the opossum sciatic nerve perineurium, examined using electrophysiological and electron microscopic techniques

A parallel electrophysiological and electron microscopic study was used to assess the ionic permeability of the sciatic nerve perineurium of the opossum Monodelphis domestica. The electrophysiological method was used to monitor permeability to K(+), followed by combined electron microscopy and X-ray probe analysis to monitor permeability to the electron-dense tracer lanthanum. Isolated but intact nerves were mounted in a 'grease gap' chamber for extracellular measurement of DC potential and compound action potential (CAP). Challenge with 100 mM [K(+)] Ringer was used to assess the K(+) permeability of the perineurium, since a change in DC potential (DeltaDC) under these conditions reflected changes in the axonal resting membrane potential. There was no detectable change in DC potential or CAP to the first K(+) challenge (n=71 nerves) indicating negligible K(+) permeability under control conditions. The inflammatory mediators histamine 0.1-40 mg/ml (1. 3-130 mM), bradykinin (0.1-4.7 mM) and 5HT (serotonin) 0.1-5.0 mg/ml (0.5-23.5 mM) caused no measurable DeltaDC on subsequent challenge with 100 mM [K(+)] Ringer, indicating no effect on perineurial K(+) permeability. In nerves exposed to the bile salt sodium deoxycholate (DOC, 6 min, 4 mM), challenge with elevated K(+) Ringer caused a dose-dependent DeltaDC in the range 10-100 mM [K(+)] (1.67+/-0.17 mV in 100 mM [K(+)], n=20), indicating increased perineurial permeability caused by DOC, but the response was smaller than that previously reported for the frog perineurium. Lanthanum was observed in the outer layers of the perineurium, but was not seen to penetrate the endoneurium in any of the nerves examined (n=51), even after DOC application. This study shows that the combined electrophysiological and electron microscopic technique for monitoring ionic permeability can be applied to mammalian nerve, and suggests that the opossum perineurium is more resistant to tight junction opening by chemical modulators than is the frog perineurium.     

The effect of anticoagulation on antenatal ultrasound findings in pregnant women with thrombophilia.

OBJECTIVE: To assess whether treatment with heparin alters ultrasound findings in pregnant women with inherited thrombophilia. METHODS: This was a retrospective study of a cohort of patients referred for pregnancy complications who were found to have genetic thrombophilia. Ultrasounds were reviewed in treated and untreated pregnancies for the presence of growth restriction, oligohydramnios or abnormal Doppler results. RESULTS: There were a total of 178 pregnancies in 51 patients. The overall percentage of abnormal ultrasounds was significantly greater in the untreated compared with treated pregnancies (52.8% vs. 27.9%; p = 0.024.) Growth restriction and abnormal Doppler results were more common in untreated pregnancies. There was a significantly decreased risk of oligohydramnios with treatment (27.3% vs. 7%; p = 0.03). Overall outcomes were significantly improved with the use of anticoagulation ( p < 0.0001). CONCLUSIONS: Treatment markedly improves ultrasound parameters of growth, fluid and feto-placental blood flow in patients with thrombophilia. The presence of abnormalities despite treatment reinforces the need for close antenatal surveillance.     

Intra‐arterial blood pressure reading in intensive care unit patients in the lateral position

Background. Routine lateral turning of patients has become an accepted standard of care to prevent complications of immobility. The haemodynamic and oxygenation effects for patients in both lateral positions (45°) are still a matter of debate. We aimed to study the effect of these positions on blood pressure, heart rate and oxygenation in a general intensive care population. Design. Observational study. Method. Twenty stable intensive care unit patients had intra‐arterial blood pressure recordings in the supine and lateral positions with the correction of hydrostatic height compared with a fixed reference point (phlebostatic level). A multilevel model was used to analyse the data. Results. Mean arterial pressure readings in the lateral positions were, on average, 5 mmHg higher than in the supine position (p < 0·001). There were no significant differences between mean arterial pressure recordings in the left and right lateral position (p = 1·0). No important differences in oxygenation and heart rate were observed. After correction for covariates, the effects persisted. Conclusion. Our study demonstrated an increase, albeit small, in blood pressure in the lateral positions. No major differences between the left and right lateral position were found. No important differences in oxygenation and heart rate were observed. Relevance to clinical practice. Turning haemodynamically stable patients in the intensive care unit has no important effects on blood pressure measurements when continuous hydrostatic height correction is applied.     

Implementation and Evaluation of “Golden Hour” Practices in Infants Younger Than 33 Weeks' Gestation

Upon review of Vermont Oxford Network data, a Midwest level III neonatal intensive care unit noted increased levels of chronic lung disease (CLD) in infants born at less than 33 weeks gestation. Initial analysis of delivery room practices with these infants showed great variance based on each provider's experience and knowledge. In an effort to standardize processes and to reduce the level of CLD within this subpopulation of infants, providers adopted “golden hour” practices focused on providing respiratory support (use of an inspiratory hold, early continuous positive airway pressure, and intubation criteria), oxygen targeting, thermal regulation, and teamwork. Compliance was tracked via delivery room documentation. Although this is an ongoing quality improvement project, examination at 3 and 6 months postpractice shows increasing compliance with the golden-hour practices. Furthermore, after 6 months of using golden-hour practices, there is a decreased incidence of CLD.