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Biomedical subjects

P A McKeever

Publications and source records attributed to P A McKeever.

12 recordsLinked to original sources

Focal nodular hyperplasia in a child with hemihypertrophy and multiple cutaneous vascular malformations.

A case of focal nodular hyperplasia of the liver occurring in a 9-year-old girl with musculoskeletal hemihypertrophy and multiple cutaneous capillary haemangiomata is described. The child presented because of limb length discrepancy and was found to have a large mass in the liver. Imaging showed a mass of similar characteristics to normal liver tissue. Prominent vascular supply to the liver was also seen. We present this case to emphasize the important diagnosis of focal nodular hyperplasia, which may occur in syndromic form in children with typical cutaneous and skeletal manifestations.

Child↗

Pathological complications of non-survivors of newborn extracorporeal membrane oxygenation.

The pathology was reviewed of the early deaths identified from the first 50 neonates treated with extracorporeal membrane oxygenation (ECMO) during its introduction to the UK. Fifteen neonates died during or shortly after ECMO between August 1989 and June 1992. Data on 12 are presented (three did not have a postmortem examination). The clinical diagnoses at referral for ECMO were as follows: persistent pulmonary hypertension of the newborn (six infants), primary congenital pneumonia (one infant), community acquired pneumonia (two infants), birth asphyxia (one infant), respiratory distress syndrome (one infant), and meconium aspiration syndrome (one infant). In our group, at necropsy, five had significant haemorrhage (three intracranial, one pulmonary, one pericardial and intraventricular). Three of five infants with evidence of haemorrhage also had signs of sepsis. Six infants had evidence at necropsy of systemic sepsis, five showed evidence of severe anoxic brain injury, and four infants had cerebellar haemorrhages. Three infants had evidence of myocardial ischaemia. It is difficult to discriminate between the relative influence of the primary diagnosis, the mode of treatment, and the severity of presentation in the genesis of this pathology. It is likely that the extent and severity of some of the findings represent a pathological progression that would have been interrupted by the death of the patient, had ECMO not been instituted.

Acute Kidney Injury↗

New syndrome with features overlapping the Baller-Gerold and Roberts syndromes.

A male fetus is described with multiple congenital abnormalities including craniosynostosis and bilateral radial aplasia. There are many similarities to the case recently reported by Imaizumi and Kuroki (Am J Med Genet 41: 162-163). These cases may represent a new syndrome with overlapping features of the Baller-Gerold and Roberts syndromes.

Abnormalities, Multiple↗

Lethal olivopontoneocerebellar hypoplasia with dysmorphic features in sibs.

This report describes the clinical and neuropathological features in male and female sibs who died shortly after birth as a result of frequent convulsions and lack of spontaneous respiratory effect. Both sibs had a prominent occiput with mild contractures and the female also had overlapping fingers and rockerbottom feet. The genetic and neuropathological findings were consistent with a diagnosis of an autosomal recessive form of olivopontoneocerebellar hypoplasia/atrophy.

Abnormalities, Multiple↗

Smith-Lemli-Opitz syndrome. II: A disorder of the fetal adrenals?

Two cases of Smith-Lemli-Opitz syndrome type II are presented. During the late stages of both pregnancies maternal oestriol levels were unrecordable and there was evidence of suppression of maternal adrenal function. We speculate on the existence of a primary defect in the fetal adrenals.

Abnormalities, Multiple↗

Prenatal diagnosis of the megacystis-microcolon-intestinal hypoperistalsis syndrome.

The ultrasonographic and necropsy findings in a male fetus with the megacystis-microcolon-intestinal hypoperistalsis syndrome are reported. The presence of vacuolation and degeneration in smooth muscle of bowel and bladder wall supports a previous suggestion that the macroscopic findings in this syndrome are the consequence of an underlying visceral myopathy. The unusual degree of severity of the findings in this fetus may explain the marked skewing of the sex ratio observed in affected liveborn infants.

Colon↗

Fetal ovarian cysts: a report of five cases.

Large fetal ovarian cysts are rare; however, widespread use of antenatal ultrasound examination has led to an increased detection rate and surgical removal. A case presenting with bowel obstruction is described along with four other cases that presented in the last 24 years. Previously reported series are reviewed and compared. It is stressed that complications are very rare and hasty surgical management of cases detected by ultrasound scanning is not warranted.

Adult↗

Prevention of spread of echovirus 6 in a special care baby unit.

A case of fatal neonatal infection with enteric cytopathogenic human orphan virus (echovirus) type 6 is presented. The measures taken to prevent further spread of infection with special reference to the use of human normal immunoglobulin are described.

Cross Infection↗

Fetal and placental pathology in two cases of maternal varicella infection.

We describe the fetal and placental pathology in two cases of maternal varicella infection. Varicella in pregnancy is uncommon, and although sporadic cases of fetal involvement are described very little regard has been paid to placental features. Only one previous report describes the changes in both the fetus and its associated placenta. In both our cases the placenta showed chronic villitis with with granuloma formation, and occasional cells with nuclear viral-like inclusions were detected. Cells with similar inclusions were found in the lung interstitium, and foci of dystrophic calcification were present in the liver.

Adult↗