When is 'intersex' not intersex? A case of penile agenesis demonstrates how to distinguish non-endocrine disorders in neonates with genital anomaly.
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Biomedical subjects
Publications and source records attributed to P McDougall.
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BACKGROUND: The aim of the present study was to determine those factors which contribute to a poor outcome and to propose a management plan that is complementary to trauma systems in common use. METHODS: A prospective study of 110 consecutive patients with moderate head injury (post-resuscitation Glasgow Coma Scale (GCS) 9-13) was carried out. RESULTS: A total of 75% of the patients sustained multisystem trauma, generally of minor or moderate grade according to the Abbreviated Injury Scale (AIS). However, the death rate increased with the severity of the injury as measured by the Injury Severity Score (ISS). The initial cranial computed tomography (CT) scan was abnormal in 61% and no patient with a normal scan developed a delayed intracranial haematoma or neurological worsening. Those patients who developed a delayed intracerebral haematoma had a worse outcome. Sixteen patients underwent craniotomy for haematoma. The intracranial pressure (ICP) was measured selectively in 20 patients and exceeded 20 mmHg in half, requiring treatment. Nine patients died, four as a result of head injury and all those had an intracranial haematoma. As a group, those who died were older and had a higher ISS. CONCLUSIONS: A plan for care of patients with moderate head injury is proposed, complementary to the Early Management of Severe Trauma (EMST) protocol and the Neurosurgical Society of Australasia guidelines for neurotrauma management in rural and remote locations.
The article describes a successful model for clinical integration that has improved utilization rates, service levels, physician and staff satisfaction, and the financial performance of physician groups and health plans. The model for clinical integration provides processes of medical management, care management, and patient management that are designed to transform a traditionally fragmented delivery system into a more cohesive system where everyone is working toward a common objective with aligned incentives. Links are established among primary care physicians, specialists, and hospitals to create synergistic relationships and seamless, accessible care for members.
OBJECTIVES: To assess the accuracy of pulse oximetry under hypothermic conditions in neonates and infants undergoing cardiac surgery, and to assess the effect of probe site as well as probe site skin temperature on the reliability of pulse oximetry. DESIGN: Prospective Study. SETTING: Cardiac operating room and intensive care unit of children's hospital. PATIENTS: Twenty-five infants <3 months of age undergoing cardiac surgery with cardiopulmonary bypass. INTERVENTIONS: Pulse oximeter readings (Sp O2) from probes placed on the hand and foot were recorded at various skin temperatures and compared with hemoximeter oxygen saturations (Sa O2) obtained on simultaneously drawn arterial blood samples. Core temperature, arterial pressure and vasodilator use were recorded simultaneously. MEASUREMENTS AND MAIN RESULTS: Pulse oximetry bias (Sp O2 - Sa O2) increased to an unacceptable range (>+3% or <-3%) in 45.5% of the readings at foot probe site temperatures of <27 degrees. Pulse oximetry bias was within an acceptable range in 94.7% of the readings at temperatures >29 degrees. There was no significant difference between oximeter readings obtained from two probe sites (hand and foot). Administration of phenoxybenzamine improved the accuracy of pulse oximetry in ten infants at skin temperature of <27 degrees. CONCLUSIONS: Pulse oximetry readings in small infants are likely to be unreliable at skin temperatures of <27 degrees, irrespective of probe site. Intravenous phenoxybenzamine appeared to improve the accuracy of pulse oximetry at low temperatures.
Over the past decade, the survival rate of infants with congenital diaphragmatic hernia (CDH) treated in the intensive care unit of the Royal Children's Hospital, Melbourne, has remained unchanged at 56% +/- 6%. Newer forms of treatment, such as extracorporeal membrane oxygenation (ECMO), high-frequency oscillation, and surfactant and nitric oxide therapy, are now available. The exact role of these therapies in the management of infants with CDH has not been determined. This study examines five clinical parameters derived from an infant's best preoperative ventilatory and blood gas data in the first 24 hours of life. One hundred twenty-five CDH infants were admitted to the intensive care unit between January 1, 1981 and December 31, 1991. Criteria for inclusion in the study were (1) CDH diagnosed within 6 hours of delivery, (2) ventilation before repair, and (3) no associated lethal congenital abnormality. Of the 90 cases studied in detail, there were 38 deaths (42% mortality rate). All five parameters were analyzed by receiver operating curve analysis to determine the optimum value of each parameter in predicting survival. An oxygenation index (MAP x FIO2/PaO2) of less than 0.08 predicted a 94% chance of survival, with a sensitivity of 96% and a specificity of 95%. Similarly, a modified ventilation index (PIP x RR x CO2/1,000) of less than 40 predicted a 91% chance of survival, with a sensitivity of 94% and a specificity of 86%. By stratifying each criterion according to outcome, three groups of infants were identified according to their response to conventional therapy.(ABSTRACT TRUNCATED AT 250 WORDS)
This study reports the physical outcomes of 181 survivors of severe head injury [Glasgow Coma Score (GCS) 8 or less] following aggressive head injury management which included early triage, evacuation and resuscitation, the use of ventilation in the Intensive Care Unit and intracranial pressure (ICP) measurement and control. At the conclusion of the study period of 2 years after the head injury, 71% had achieved a Glasgow Outcome Score (GOS) of 1, 17% GOS 2, 10% GOS 3 and 2% GOS 4. Physical recovery continued during the 2 years. Locomotor independence was achieved in 93% despite a motor disorder in 59%. About 90% were considered independent for basic life functions, e.g. feeding, bathing, dressing or toileting. Speech disorders were present in 19%. A return to an occupation, either a job or studies, at or below the pre-morbid level was achieved in 68%. Most patients were cared for at home by family or relatives. The majority of patients make a reasonable physical recovery following severe head injury with independence in locomotion and basic life skills. It was our impression that the neuropsychological sequelae of cognitive and behavioural disorders were a major impediment to re-assimilation into society.
A prospective study of 315 consecutive patients with a severe head injury was undertaken to study factors contributing to mortality and morbidity, both in the pre-hospital and hospital phases. Entry criteria were a Glasgow Coma Scale (GCS) score of 8 or less after non-surgical resuscitation within 6 h of the injury, or a deterioration to that level within 48 h. Patients with gunshot wounds or who were dead on arrival were excluded. End points of the study were either death or at 6 months after the injury. Predictors of mortality were increasing age, the presence of hypotension, a low GCS, abnormal motor responses and pupillary non-reactivity. In the 167 patients in whom intracranial pressure (ICP) was measured, raised ICP and failure to respond to treatment for raised ICP also predicted mortality. Three CT predictors of mortality were the presence of cerebral oedema, intraventricular blood and the degree of midline shift. When analysed using logistic regression, the most accurate model (accuracy 84.4%) included increasing age, abnormal motor responses and the three CT indicators. Analysis of the data for 'good' (Glasgow Outcome Score (GOS) 1 and 2) vs 'poor' (GOS 3 and 4) survival at 6 months was also performed using logistic regression. The model which provided the most accurate prediction of poor outcome included age, hypotension and three different CT characteristics, subarachnoid blood, intracerebral haematoma or intracerebral contusion (accuracy 72.5%).
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At the Royal Children's Hospital, Melbourne, extracorporeal membrane oxygenation (ECMO) has been used in the treatment of newborn infants with life-threatening respiratory or cardiac failure since May 1989. The main indications for the use of ECMO are that the disease is reversible, the surviving infant is likely to be normal and there is an 80% likelihood of death without ECMO. Sixteen of 22 (73%) newborn infants have survived at least 6 months after ECMO. Fourteen of 16 (87.5%) infants receiving ECMO (who did not have a congenital diaphragmatic hernia) were functionally normal survivors; the other two infants died. Two of six infants with congenital diaphragmatic hernia who received ECMO were discharged and survived to have normal neurological and respiratory function at 6 month follow up. These results are similar to results from other centres internationally. It would appear that ECMO is a useful therapy for near-term newborn infants with otherwise fatal cardiorespiratory failure.
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The anti-inflammatory agent flufenamic acid was found to inhibit calcium uptake in isolated mitochondria at low concentrations (IC50 = 7.2 microM). Similar concentrations were required to promote the release of calcium from mitochondria preloaded with the cation (EC50 = 3.5 microM). Identical actions were found with diflunisal, mefenanamic acid and 2,4-dinitrophenol. It was concluded that flufenamic acid was affecting calcium movements across the mitochondrial membrane by virtue of its ability to uncouple oxidative phosphorylation.
Five patients with neonatal infective endocarditis were reviewed, two of whom survived. Infection was caused by Staphylococcus aureus in four and by Candida albicans in one. All cases of bacterial endocarditis had clinical signs of septicaemia, positive blood cultures, thrombocytopenia, microscopic haematuria, and heart murmurs. Three developed skin abscesses early in their illnesses. Three patients had two dimensional echocardiographic studies that showed bacterial vegetations. One of these studies was done before the heart murmur could be heard. We suggest that echocardiography in conjunction with the clinical picture described may help in making an early diagnosis of endocarditis in neonates.
Tolazoline Hydrochloride is a pulmonary vasodilator that is used for the treatment of pulmonary hypertension of the newborn. Two patients who were receiving Tolazoline by infusion developed duodenal ulceration and subsequent intestinal perforation. This undesirable side effect of Tolazoline may be prevented by the concomitant use of antacids to maintain gastric pH greater than 5.
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The anti-inflammatory agent diflunisal was found to induce a progressive loss of respiratory control in tightly coupled rat liver mitochondria, starting at low concentrations (3.3 microM). This loss of control was accompanied by a stimulation of state 4 respiration in the presence of either succinate or glutamate plus malate as the respiratory substrate. The inhibition of state 3 respiration by oligomycin was released by diflunisal. Mitochondrial ATP hydrolysis was stimulated by diflunisal over the same concentration range that affected state 4 respiration: the stimulation was inhibited by oligomycin. It was concluded that diflunisal was acting as an uncoupler of mitochondrial oxidative phosphorylation. An identical action was found in mitochondria isolated from the livers of mice, rabbits and guinea-pigs. Potencies similar to diflunisal were found with flufenamic acid and mefenamic acid, but other anti-inflammatory agents were either less potent or inactive.