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Biomedical subjects

G Shortland

Publications and source records attributed to G Shortland.

9 recordsLinked to original sources
PubMed2001

Non-hepatic hyperammonaemia: an important, potentially reversible cause of encephalopathy.

The clinical syndrome of encephalopathy is most often encountered in the context of decompensated liver disease and the diagnosis is usually clear cut. Non-hepatic causes of encephalopathy are rarer and tend to present to a wide range of medical specialties with variable and episodic symptoms. Delay can result in the development of potentially life threatening complications, such as seizures and coma. Early recognition is vital. A history of similar episodes or clinical risk factors and early assessment of blood ammonia levels help establish the diagnosis. In addition to adequate supportive care, investigation of the underlying cause of the hyperammonaemia is essential and its reversal, where possible, will often result in complete recovery. Detection of an unborn error of metabolism should lead to the initiation of appropriate maintenance therapy and genetic counselling.

Adult↗
PubMed1999

Toxic shock syndrome secondary to a dental abscess.

A 9-year-old girl presented with arthralgia and myalgia which progressed to developing renal failure and overwhelming septic shock. The underlying cause was assumed to be a periodontal abscess from an upper right deciduous canine tooth. The pus from the abscess grew a toxic shock syndrome toxin 1-producing Staphylococcus aureus. This case illustrates the importance of an oral surgical review of patients presenting with features of toxic shock syndrome if the source of the infection is not immediately obvious.

Child↗
PubMed1998

Photosensitivity associated with the Smith-Lemli-Opitz syndrome.

A case of severe photosensitivity in a girl with the Smith-Lemli-Opitz syndrome is reported. Children with this recessively inherited metabolic disorder of cholesterol metabolism present with a variety of congenital abnormalities of the nervous system and internal organs in association with varying degrees of mental retardation. Photosensitivity is a feature which has previously only briefly been mentioned in the literature in association with this syndrome. However, more recently, it has become apparent that photosensitivity is not uncommon among children with the Smith-Lemli-Opitz syndrome, although the nature of the photosensitivity in these patients has remained undefined. Our patient has suffered from sunlight intolerance since early infancy, with redness and pruritus of sun-exposed skin developing within minutes of sun exposure. Monochromator ultraviolet (UV) radiation and visible light testing revealed an immediate and persistent reaction to low-dose UVA at 350 nm, and an abnormal erythemal response to visible light at 400 nm.

Child, Preschool↗
PubMed1994

Validation of a croup score and its use in triaging children with croup.

The Syracuse croup scoring system was validated in 165 children with croup who were admitted to an intensive therapy unit for assessment over a one year period. The unit served as a croup triage point for Cardiff and its environs. A score of > 5 was taken as an indication that a patient was at risk of upper airway obstruction and was used to support a triage decision by the junior hospital doctor to admit a patient to the intensive therapy unit. All patients with an initial score < or = 5 were considered safe for transfer to a general paediatric ward and none of these required subsequent admission to intensive care. This score was then tested on a further 134 children with croup, in order to identify those patients who required specialised monitoring, observation or treatment in intensive care. A score of > 5 gave a specificity of 100% and a sensitivity of 80%. Croup scoring continued after admission on the general paediatric wards. Two patients who were originally admitted to the intensive therapy unit with a score > 5 improved within 6 h and were transferred to the general ward with a score < or = 5. These children subsequently required readmission to the intensive therapy unit. Our tracheal intubation rate of 2% was low and may relate to the routine use of regular adrenaline nebulisation. We recommend this scoring system to other paediatric departments for initial triaging decisions and for documenting progress on the wards.

Child↗
PubMed1993

Are medical students missing out?

Increasing discontent, even boredom, shown by many clinical medical students is in marked contrast to the enthusiasm of school children interviewed for a place at medical school. The roots of the problem are sown in the pre-clinical years with too much teaching that is of little relevance to clinical practice and insufficient contact with clinical problems that are needed to keep the youthful flame of idealism alive. In clinical years disillusion continues with less time available for teaching by NHS colleagues because of clinical directorates and other administrative chores and a far from ideal balance between teaching and research activities amongst academic staff. To help enthusiasm return we recommend greater clinical involvement in the pre-clinical years, teaching to be better valued and teaching and examination methods reassessed. More self-directed learning, though desirable, will be difficult unless adequate resources are made available. At a time of increased pressure on all sides if we cannot meet the challenge of modern undergraduate clinical education disillusion amongst our students will continue with serious implications for the whole continuum of medical education.

Education, Medical, Undergraduate↗
PubMed1990

Serum immunoglobulins to endotoxin core glycolipid: establishment of normal concentrations.

Serum antibody to lipopolysaccharide core glycolipid was measured in normal children and in full term and premature (less than or equal to 1500 g) infants. Antilipopolysaccharide core glycolipid antibody was present in term infants and normal children, and reached adult titres by 15 years of age. The specific anti core glycolipid antibody was predominately of the IgG class. Preterm infants (less than 32 weeks' gestation) had significantly lower titres of antilipopolysaccharide core glycolipid than more mature preterm or term infants. The results suggest that administration of anticore glycolipid immunoglobulin may be beneficial in the treatment or prevention of Gram negative septicaemia in preterm and very young infants.

Adolescent↗
PubMed1990

Serum immunoglobulins to endotoxin core glycolipid: acute leukaemia and other cancers.

Circulating antibody to endotoxin core glycolipid and total serum immunoglobulin concentrations were measured in 86 children with cancer (54 with acute lymphoblastic leukaemia, four with acute myeloid leukaemia, and 28 with various solid tumours). Measurements were made before treatment in the group with acute lymphoblastic leukaemia as well as when patients were both on and off chemotherapy. In the other two groups measurements were made when patients were both on and off treatment. Significant reductions in endotoxin antibody and serum immunoglobulin concentrations were found only in patients with acute lymphoblastic leukaemia. In addition, there was a significant correlation between febrile episodes and the concentration of antibody to core glycolipid in the children with acute lymphoblastic leukaemia. These findings suggest that the use of prophylactic high titre endotoxin antibody may be of benefit to children with life threatening Gram negative infections who are receiving cytotoxic chemotherapy.

Child↗