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H Wainwright

Publications and source records attributed to H Wainwright.

At least 19 recordsLinked to original sources
PubMed2006

Poikiloderma, tendon contracture and pulmonary fibrosis: a new autosomal dominant syndrome?

Members of two generations of a South African family have a unique syndrome comprising poikiloderma, tendon contractures and progressive pulmonary fibrosis. The condition is clinically important as the skin changes, which involve the face, have considerable cosmetic impact, while lung involvement is potentially lethal in adulthood. Skin manifestations which facilitate diagnosis include facial telangiectasia, mottled hypo- and hyperpigmentation, papules and epidermal atrophy. The scalp, facial and body hair are fine and scanty. The tendon contractures lead to progressive digital flexion deformities and abnormalities of the ankles and feet, with disturbance of gait. Pulmonary involvement manifests as progressive dyspnoea. Pedigree data are compatible with an autosomal dominant mode of transmission. Poikiloderma of Weary is characterized by linear sclerotic and fibrous bands and not tendon contractures and is not associated with potentially lethal pulmonary fibrosis. Rather than name this disorder a variant of Weary syndrome, it might be prudent to use as an umbrella title one composed by Weary himself: 'hereditary sclerosing poikiloderma' (HSP), under which variants such as HSP Weary type, HSP with cardiac involvement (aortic stenosis described as inconsistently associated with Weary syndrome) and HSP with tendon/pulmonary involvement (current family) may be classified. The manifestations in this family differ from other poikilodermata and, to the best of our knowledge, have not been previously documented.

Adult↗
PubMed2006

Four decades of conjoined twins at Red Cross Children's Hospital--lessons learned.

Conjoined twins represent a rare but fascinating congenital condition, the aetiology of which remains obscure. Over the past four decades, the paediatric surgeons at Red Cross Children's Hospital have been involved in the management of 46 pairs of conjoined twins, of which 33 have been symmetrical and 12 asymmetrical. Seventeen symmetrical twins have undergone separation with 22 children (65%) surviving; all of the live asymmetrical twins survived separation. We describe the important features of this unique cohort, outline our approach to management and present the results of this approach. We consider some of the ethical and moral dilemmas we have confronted, and discuss the prenatal diagnosis, obstetric implications and postnatal care of these children, including the relevant investigations and anaesthetic and surgical management. Specific aspects related to the cardiovascular system, hepatobiliary and gastrointestinal tracts, urogenital tract, central nervous system and musculoskeletal system are highlighted.

Adolescent↗
PubMed2005

Preterm prelabour rupture of the membranes before 28 weeks: better than feared outcome of expectant management in Africa.

OBJECTIVE: To document the prognosis after conservative management of patients with membrane rupture at gestations less than 28 weeks. STUDY DESIGN: Prospective observational study of 78 women with confirmed membrane rupture at less than 28 weeks gestation, managed conservatively. Antibiotics were given from the time of membrane rupture till delivery. Patients were delivered if clinical infection supervened, there was fetal compromise, spontaneous labour ensued or if the pregnancy continued to 34 completed weeks gestation. RESULTS: The mean gestational age at membrane rupture was 23.3+/-3.17 weeks (16.5-27.8) and the median 24 weeks. Mean latency period was 24.1+/-29.1 days (1.5-154) with a median of 12.5 days. Eight women (10%) delivered between 24 and 48 h, 25 (32%) within 7 days and 55 (70%) within 1 month. Of note is that 23 patients (30%) had latency periods of greater than 1 month. The mean gestational age at delivery was 26.7+/-3.92 weeks. Overall of the 78 women there were 81 fetuses delivered, of which 35 (43%) survived. Survival was related to latency period, birth weight and gestational age at delivery. Sixteen women (20%) developed chorioamnionitis. There was no increase in the incidence of clinical infection with increasing latency period. Compression limb abnormalities occurred in 17% of neonates and lung hypoplasia in 18%. CONCLUSION: Conservative management of patients with very preterm prelabour membrane rupture offers a survival rate of at least 40% with no serious complications in a study of 78 women.

Adolescent↗
PubMed2000

Evaluation of alternative methods of cervical cancer screening for resource-poor settings.

BACKGROUND: Noncytologic methods of screening for cervical carcinoma and its precursor lesions are needed for resource-poor settings in which cervical carcinoma continues to be an important cause of morbidity and mortality. METHODS: Two thousand nine hundred forty-four women ages 35-65 years were recruited from Cape Town, South Africa and screened using a combination of a Papanicolaou (Pap) smear, human papillomavirus (HPV) DNA testing, direct visual inspection after the application of a 5% acetic acid solution (DVI), and cervicography. Cervicography was considered primarily as a method with which to quality control the DVI examinations. Women with squamous intraepithelial lesions (SIL) or carcinoma on Pap smear, positive DVI examination (acetowhite lesion or cervical ulcer/growth), high levels of high risk HPV DNA (relative light units [RLU] > 10x positive control), or positive Cervigramtrade mark were referred for colposcopy and cervical biopsy. RESULTS: Pap smears were positive in 8.1% of all women screened and identified 65 (78%) of all cases of biopsy confirmed high grade disease (high grade SIL or invasive carcinoma). DVI and cervicography were classified as positive in 18.1% and 10.5%, respectively, of women screened and identified 58 (67%) and 46 (58%) of all cases of high grade disease, respectively. The results of HPV DNA testing varied depending on the cutoff value used to define a positive result. At the standard cutoff level (RLU > 1x positive control), 16.2% of women screened were classified as high risk HPV DNA positive, as were 63 women with high grade disease (73%). CONCLUSIONS: DVI and HPV DNA testing identified similar numbers of high grade SIL (cervical intraepithelial neoplasia Grade 2,3) and invasive carcinoma cases as Pap smears. However, both classify considerably more women without cervical disease as being test positive.

Adult↗
PubMed1998

Widespread axonal injury in gunshot wounds to the head using amyloid precursor protein as a marker.

In order to determine whether axonal injury (AI) is a factor in cases of penetrating head injury, the brains of 14 patients who died shortly after sustaining a fatal gunshot wound (GSW) to the head were examined, and the presence of AI determined using immunohistochemical staining for amyloid precursor protein (APP). The distribution of AI was mapped throughout the cerebral hemispheres and brain stem. AI was present in all cases in a diffuse distribution distant to the missle track with severe involvement of the brain stem in all cases. There was no axonal APP immunoreactivity in the direct region of the missle track at the point of primary axotomy. The APP-positive AI in these cases is likely to be a mixture of primary and secondary AI as APP immunostaining is unable to distinguish primary AI due to mechanical deformation from AI secondary to hypoxic-ischemic damage.

Adolescent↗
PubMed1997

Axonal injury in falls.

Amyloid precursor protein (APP) immunocytochemistry was used as a marker for axonal injury (AI) in a series of 16 cases of head trauma associated with fatal falls. Nine cases were falls from not more than the person's own height (falls from < or = own height) and seven cases were falls from a distance greater than the person's own height (falls from > own height). AI was recorded on a series of line diagrams of standard brain sections divided into 116 sectors. AI around focal lesions (infarcts, hemorrhages, contusions) was distinguished from nonfocal axonal injury that was distant from any focal area of damage. The percentage of sectors showing focal AI provided the Focal Axonal Injury Score (FAIS) and the percentage showing nonfocal AI the Non-Focal Axonal Injury Score (NFAIS). The FAIS is a measure of secondary AI and the NFAIS of diffuse axonal injury (DAI). The percentage of sectors involved with AI (focal and nonfocal) provided the cumulative Axonal Injury Score (AIS). A semiquantitative grading system was also used to assess the severity of axonal injury in each sector and the sum of the grades from all sectors was expressed as a percentage to provide the Axonal Injury Severity Score (AISS). Widespread AI was present in all cases irrespective of the height of the fall. AI was present in the midbrain (94%), pons (94%), corpus callosum (100%), central grey matter (100%), and cerebral hemispheric white matter (94%). AIS ranged from 10 to 94 in falls from < or = own height (mean 73) and from 38 to 92 in falls from > own height (mean 82). AISS ranged from 6 to 95 in falls from < or = own height (mean 65) and 28 to 95 in falls from > own height (mean 72). There was no statistically significant difference in AIS or AISS between the two groups. The extent and severity of AI cannot be predicted from biomechanical data, such as the height of the fall, as the total AI in a given case is a variable mixture of Nonfocal AI (DAI) and Focal AI arising by secondary mechanisms, and APP immunostaining is unable to distinguish primary from secondary AI. However, the combination of the Hypoxic-Ischemic Score (HIS) defined as the percentage of sectors showing any hypoxic-ischemic damage ranging from neuronal "red cell change" to infarction in conjunction with the FAIS and NFAIS provided a measure of the relative contribution of primary and secondary AI in a given brain.

Accidental Falls↗
PubMed1996

Intramuscular haemangioma of the chest wall. A case report.

Intramuscular haemangiomas of thoracic skeletal muscle are uncommon tumours. They are locally invasive and tend to recur if not completely and widely excised. This report illustrates the diagnosis and management of a 25-year-old man with an intramuscular haemangioma of the chest wall. A literature report on the aetiology and management of these tumours is also given.

Hemangioma↗
PubMed1995

Lipoma of corpus callosum associated with dysraphic lesions and trisomy 13.

We report on a further case of corpus callosal lipoma and frontal cranial defects. Most cases in the literature of corpus callosal lipoma in association with "dysraphic" lesions have been frontal in location. Malformation of the corpus callosum is said to be associated with 50% of these lipomas. Trisomy 13 was confirmed by the 13q14 cosmid probe on paraffin-embedded liver tissue.

Adult↗
PubMed1995

Topography of axonal injury as defined by amyloid precursor protein and the sector scoring method in mild and severe closed head injury.

Axonal injury (AI), as defined by amyloid precursor protein (APP) positive axonal swellings, was recorded on a series of line diagrams of standard brain sections divided into 116 sectors to provide an Axonal Injury Sector Score (AISS) ranging from 0 to 116. This sector scoring method of recording axonal damage and providing a topographic overview of AI was applied to a series of 6 mild head injury cases [Glasgow Coma Scale (GCS) 13-15] and six severe head injury cases (GCS 3-8). The AISS ranged from 4 to 107 overall and varied from 4 to 88 in the mildly injured group and 76 to 107 in the severe head injury group, supporting the concept that there is a spectrum of AI in traumatic head injury and that the AISS is a measure of the extent of AI. APP immunostaining demonstrated positive axonal swellings 1.75 h after head injury and analysis of the pattern of AI in the mild and severe head injury groups showed that axons were more vulnerable than blood vessels and that the axons in the corpus callosum and fornices were the most vulnerable of all.

Adolescent↗
PubMed1994

Staining of amyloid precursor protein to study axonal damage in mild head injury.

The most common definition of cerebral concussion is that of a transient loss of neurological function without macroscopic or microscopic abnormality in the brain. However, some patients have persistent symptoms and subtle neuropsychological deficits, particularly affecting memory. We have studied five patients aged 59-89 years who sustained mild concussive head injury and died of other causes (2-99 days post-injury). Immunostaining with an antibody to amyloid precursor protein, a marker of fast axonal transport, showed multifocal axonal injury in all five. All had axonal damage in the fornices, which are important in memory function.

Aged↗
PubMed1993

Polysplenia syndrome and congenital short pancreas.

We present a report of a stillborn female of mixed ancestry with congenitally short pancreas, polysplenia, congenital heart defect, normal bronchial branch pattern, and interruption of the inferior vena cava to add to the growing numbers (15) of this malformation complex in the literature. Additional manifestations were congenital hydrocephalus and absence of intestinal malrotation. A recent poster presentation at the Second International Workshop on Fetal Genetic Pathology in Montana by Drut et al. underlined the importance of reporting these cases and of the pathological examination required ["Abnormal Spleen Lobulation and Short Pancreas," in Blastogenesis-Normal and Abnormal. New York: Wiley-Liss, Inc. for the National Foundation-March of Dimes, 1993].

Abnormalities, Multiple↗
PubMed1993

Developmental anomalies in monozygous twins resembling the human homologue of the mouse mutant disorganization.

Human monozygous (Mz) twins are described with a pattern of abnormalities similar to those reported in heterozygous expression of the mouse mutant disorganisation gene (Ds). These include anomalies of the skeletal, gastrointestinal, genito-urinary and central nervous system. Concordance in Mz twins is suggestive of a genetic aetiology and lends further credence to the putative existence of a human homologue for Ds.

Abnormalities, Multiple↗
PubMed1991

Obliterative bronchiolitis: virus induced?

This report describes a 28-year-old patient with an acute onset of respiratory symptoms associated with clinical and roentgenographic features of an interstitial lung disease which proved fatal. The entity of bronchiolitis obliterans, which was diagnosed on transbronchial biopsy, is discussed, with particular emphasis on the postinfectious type.

Acute Disease↗
PubMed1989

GATAAG; a cis-control region binding an erythroid-specific nuclear factor with a role in globin and non-globin gene expression.

An erythroid-specific nuclear protein factor binds to a sequence motif (GATAAG) which is present in the promoter region of the mouse alpha and beta major globin genes, and in the erythroid-specific promoter of the human porphobilinogen deaminase (PBG-D) gene. The protein activity is conserved across species, being found in mouse erythroleukaemia (MEL) cells, chicken erythrocytes, the human erythroid K562 and KMOE cell lines, but not in a variety of non-erythroid mouse tissues or in HeLa cells. Functional analysis of this element in the alpha globin gene promoter by stable transfection experiments show that the GATAAG motif resides in a 68 bp sequence which has a stimulatory effect on transcription in mouse erythroleukaemia but not fibroblast cells. The GATAAG motif is conserved in the promoters and 3' enhancers of a variety of globin and non-globin genes implying that it is a cis-element involved in the tissue-specific up-regulation of several genes that are co-expressed during erythroid cell differentiation.

Animals↗
PubMed1978

Nutritional status of long-stay geriatric in-patients: effects of a food supplement (Complan).

In a detailed nutritional study of 50 long-stay elderly patients, intakes of vitamins C and D and nicotinic acid were found to be sub-optimal compared to D.H.S.S. recommendations. Whole-food supplementation did not increase calorie, fat or carbohydrate intake, although it did increase protein, vitamins and minerals. However, it did not correct the intake of vitamins C and D and of nicotinic acid to those recommended by D.H.S.S. Supplementation affected neither serum albumin nor transferrin. No positive correlation could be established between physical disabilities and total dietary intake among these patients. However, the appearance of the skin improved and skin thickness increased in the supplemented patients and the blood urea diminished in them also.

Aged↗
PubMedDate not supplied

Absence of the ductus venosus with direct connection between the umbilical vein and right atrium.

Abnormalities of the ductus venosus are rare. We present the case of a dichorionic, diamniotic twin pregnancy in which complete absence of the ductus venosus, with direct communication between the umbilical vein and right atrium, was detected in one infant on antenatal ultrasonography. Autopsy confirmed the aberrant course of the umbilical vein, which also had an abnormal histological structure. Associated congenital anomalies included an ostium secundum type atrial septal defect, absent inferior vena cava, single left pulmonary vein, stenosed right pulmonary artery, proliferation of the hepatic arterioles with reduction of portal venules, and a duplex ureter. The literature is reviewed and pathogenesis relevant to this case is discussed.

Abnormalities, Multiple↗
PubMedDate not supplied

Progress in the moral reasoning of baccalaureate nursing students between program entry and exit.

Changes in moral reasoning between entry into and exit from a baccalaureate nursing program and the relationship between student characteristics and moral reasoning at entry and exit were explored in this descriptive study. The moral reasoning of four cohorts of students was measured using the Defining Issues Test (DIT). Admission grade point average, prior college credits, and gender accounted for 10% of the variance in DIT P% scores at entry and 14% of the variance at exit from the program. Female students had significantly higher moral reasoning scores than men. Age did not contribute significantly to explaining DIT score variance. DIT P% scores at entry for all four cohorts were within the range of reported norms for college students. Exit scores for all four cohorts were between the normative means for undergraduate students and graduate students. DIT P% score gains between entry and exit were significant for all four cohorts. Students whose entry scores were in the lowest categories had the greatest mean gains.

Adult↗