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Chairman of department: Professor D. M. Danks (on leave) Acting Chairman of department: Dr J. M. Court Stevenson Professor DAVID MILES DANKS

First Assistants JOHN MAURICE COURT IAN JAMES HOPKINS

DAVID ALEXANDER McCREDIE

First Assistants in Neonatal Paediatrics W I L L I A M HENRY KITCHEN JOHN HEDLEY DREW

Second Assistant ARNOLD LAWRENCE SMITH - part-time

Research Fellows HOWARD ERNEST W I L L I A M S - Senior Research Fellow - part-time ANGELA BRENTON - part-time

A L L A N CARMICHAEL

MARJORIE ELIZABETH DUNLOP JEFFREY M A N N - part-time DAVID BUXTON PITT - part-time ANNE LAVINIA RICKARDS - part-time ESTHER ROTENBERG

MARGARET RYAN - part-time

R O V A L C H I L D R E N ' S H O S P I T A L R E S E A R C H F O U N D A T I O N Director and Royal Children's Hospital Research Foundation Professor DONALD BROOK CHEEK

Director's Unit Research Fellows and Assistants JOAN EVELYN GRAYSTONE

A L A N BARRY HOLT MARGARET N I A L L KERIN O'DEA NICOLA PARTRIDGE HELEN WILMOT

Genetics Research Unit Head Professor D. M . DANKS

Research Fellows and Assistants RICHARD GRAHAM HAY COTTON - Senior Fellow JAMES CAMAKARIS

IAN GEORGE JENNINGS DEN1SE MARY KIRBY JOHN GRAHAM ROGERS PETER SCHLESINGER

DAVID OWEN SILLENCE - until Julv 1977 ROGER TRUSCOTT

R E S E A R C H T O P I C S IN P R O G R E S S

A. G E N E T I C S - Professor D. M . Danks

1. Purification and properties of phenylalanine hydroxylase from monkey and hitman liver

2. Search for phenylalanine hydroxylase cross-reacting-material in phenyl kctonuric liver and in cells in culture.

3. Phenylalanine hydroxylase deficient mutants induced in rat hepatoma cells.

4. Purification and properties of human dihydropteridine reductase.

5. Urinary pteridine excretion of phenylketonuria and in normal subjects.

6. Copper metabolism in mottled mice and in Menkes' syndrome.

7. Copper transport in human and murine cells in culture.

8. Methods of detection of inborn errors of metabolism.

9. Treatment of trimethylaminuria.

10. Ultra-structural studies in osteogenesis imperfecta.

11. Metabolic loading studies with stable isotopes.

12. Myelination in mottled mice.

13. A verbal-visual computer-based system of syndrome identification.

B. S T U D I E S IN G R O W T H A N D N U T R I T I O N - Professor D. B. Cheek 1. Assessment of cellular mass in Aboriginal children.

2. Trace metal levels in Aboriginals in North West Australia.

3. Diabetes in Australian Aborigines - a comparison of urban and traditional life-styles.

4. Growth peptides and their clinical application.

142

PAEDIATRICS 143 5. Hormonal control of skeletal muscle growth.

6. Growth following bowel resection.

7. Insulin action on arterial tissue in relation to diabetes and atheroma.

8. Mechanism of insulin supersensitivity accompanying reduced meal frequency.

9. Compensatory and adaptive growth.

10. Cellular growth studies in obesity, chronic asthma and anorexia nervosa.

C. S T U D I E S IN O B E S I T Y A N I ) IN D I A B E T E S - Dr J . M . C ourt 1. Development of foetal adipose tissue.

2. Insulin binding antibodies in diabetic children.

3. Investigation of insulin replacement in diabetic keto-acidosis.

4. Cholesterol metabolism in familial hypercholesterolemia.

5. Feeding habits and attitudes of mothers of obese and normally nourished infants.

D. S T U D I E S IN R E N A L P H Y S I O L O G Y AND D I S E A S E - Dr 1). A. McCredie 1. Action of parathormone on renin release.

2. Urinary kallikrein excretion in children of hypertensive parents.

E . C O M M U N I T Y P A E D I A T R I C N E E D S - Dr. H . E . Williams 1. Survey of community health needs in Brunswick.

F . N E O N A T A L P A E D I A T R I C S - Dr W. Kitchen: Dr J . Drew 1. Long term follow up of infants born lo mothers with low oestriol excretion.

2. Long term follow up of infants with a low birth weight.

3. Mortality and morbidity associated with breech delivery.

C . L I V E R D I S E A S E - Dr A. Smith

1. Studies of bile production and bacterial flora after portoenterostomy in extra hepatic billiary atresia.

2. Copper accumulation in chronic liver disease in childhood.

3. Antiproiease levels in infants with neonatal liver disease.

4. Other antiproleases in families with o, antitrypsin deficient infants.

P U B L I S H E D W O R K

C H A P T E R S IN B O O K S

1. DANKS. D. M. - Acute Neonatal Illness in Inborn Errors of Metabolism. Paediatric Emergencies. J. A.

Black ed. Butterworths, Kent.

2. DANKS, D. M . - Plan of Management for Newborn Babies in whom a Metabolic Disease is Anticipated or Suspected. Clinics in Perinatology Symposium on Early Detection and Management o f Inborn Errors. Mary G. Ampola ed. W. B. Saunders Co. Philadelphia. March 1976.

A R T I C L E S

3. BEISCHER, N. A , ABELL. D. A. and DREW, J. H. - Management of Foetal Growth Retardation.

Med. J. Aust.. 2: 641-646. 1977.

4. BURKE. }.. GLASGOW, E., McCREDIE, D. A. and POWELL. H. R. - Glomerulopathy in Cyanotic Congenital Heart Disease. Clin. Nephrology. 7. 38, 1977.

5. CHEEK, D. B.. HABICHT, J. P.. BERALL. J. and HOLT, A. B. - Protein-calories Malnutrition and the Significance of Cell Mass Relative lo Body Length. Am. J. of Clin. Nutrition. SO: 851-860. 1977.

6. CHEEK. D. B. - Human Growth - methods of approach. Aust. Paediatr. J. IS: 77-87, 1977.

7. CHEEK, D. B.. GRAYSTONE. J. E. and NIALL, M. - Factors Controlling Foetal Growth. Clin. Obstet and Cvn. 20: 925-942, 1977.

8. CHOO. K. H. and COTTON, R. I I . I I . - Genetics of the Phenylalanine Hydroxylase System I: Isolation or phenylalanine hvdroxvlase-deiicienl tyrosine auxotrophs from rat hepatoma cells. Somatic Cell Genetics 3:

457-470. 1977.

9. CORNELL, H.J. and WILMOT. H.C. - Partial Purificanl ofSomatomedin from Human Plasma. Ausi. J.

of Experimental Diol. and Med. Sc. 55: 435-440. 1977.

10. COTTON, R. G. H. - Thc Primary Molecular Defects in Phenylketonuria and its Variants, //if. J. Biochem.

S: 333. 1977.

11. COURT, J. M . and DUNLOP. M . - Development of Adipose Tissue in the Human Foetus. Proc. Nutr

12. COURT. J. M , JOHNS. NAN and WILSON, MARGERY - Obesilv in Childhood. Med. J. Ausi. I . 888- 891, 1977.

13. COURT, J. M . , JOHNS, NAN and WILSON, MARGERY - Obese Children and their Families: A Study of a Discussion Group. Awn. Paediair. J.. Vol. 13, 170-175, 1977.

14. DANKS, D. M . - Can we Remake Mankind? Search 8: 16, 1977.

15. DANKS, D. M . - Copper Transport and Utilization in Menkes' Syndrome and in Mottled Mice, Inorganic Perspective in Biol. and Med. / • "73-100, 1977.

16. DANKS, D. M . - Cost Benefits of Pre- and Postnatal Screening Programmes. Records ofthe Adelaide Children's Hospilal I : 408-416, 1977.

17. DANKS, D. M . - Management of Neonatal Obstructive Jaundice. Aust. Paediair. J. 13: 71. 1977.

18. DANKS, D. M. - New and Important Variants of Phenylketonuria, Paediair. Digest 19 (8): 9, 1977.

19. DANKS, D. M , CAMPBELL, P. E, JACK, J , ROGERS, J. and SMITH, A. L. - Studies on the Aetiology of Neonatal Hepatitis and Biliary Atresia. Arch. Dis. Childh. 52: 360-367, 1977.

20. DANKS, D. M , CAMPBELL, P. E., SMITH, A. L., ROGERS. J. - Thc Prognosis of Babies with Neonatal Hepatitis. Arch. Dis. Childh. 52: 368-372, 1977.

21. DANKS. D. M . — Inborn Errors of Connective Tissue Metabolism. Records of the Adelaide Children's Hospilal. I : 366-372, 1977.

22. DANKS, D. M , HAMMOND, J , SCHLESINGER, P, FAULL, K , BURKE, D , HALPERN, B. - Trimethylaminuria: Diet docs not always Control the Fishy Odor. New Engl. J. Med. 295: 963, 1976.

23. DREW. J. H., MOUNTAIN, K. R. and BEISCHER, N . A. - Spontaneous Improvement in Severity of Erythroblastosis with Advancing Parity, Aust. N.Z. J. Obsiei. Gvnaec. 17: 229-231, 1971.

24. DREW, J. H„ PARKINSON, P., WALSTAB, J. E. and BEISCHER. N. A. - Incidences and Types of Malformations in Newborn Infanls. Med. J. Aust. 26: 945-949, 1977.

25. DREW, J. H , SMITH, M . B. and KITCHEN, W. H. - Glucose-6-phosphate dehydrogenase Deficiency in Immigrant Greek Infants. J. Paediair. 90: 659-677, 1977.

26. FAULL. K. F , C A N , I , HALPERN, B., HAMMOND, J , I M , S, COTTON. R. G. H , DANKS, D. M„

FREEMAN, R. - Metabolic Studies on Two Patients with non-hepatic Tyrosinemia using Deuterated Tyrosine Loads, Pediatric Research I I : 631, 1977.

27. FORTUNE, DENYS W. and KITCHEN, W I L L I A M H. - Malformations in Infants of Very Low Birth Weight. Med. J. Aust. I : 239-242, 1977.

28. McCREDIE. D. A. - Vomiting and Diarrhoea in Young Children, Patient Management. Vol. I . pp. 53-61, 1977.

29. NIEDERWIESER, A , MATASOV1C, A , TIPPETT, P. and DANKS, D. M. - A new Sulfur Amino Acid, named Hawkinsin. Identified in a Babv with Transient Tyrosinemia and her Mother. Clin. Chim. Ada.

76: 345-356, 1977.

30. OBERKLAID, F. M , DANKS, D. M , MAYNE, V. and CAMPBELL, P. - Asphyxiating Thoracic Dysplasia. Clinical, Radiological and Pathological Information Regarding 10 Patients. Arch. Dis. Childh.

52: 758, 1977.

31. ROY, NEIL D. and KITCHEN, W. H , NETS: A New System for Neonatal Transport, Med. J. Aust. 2:

855-858, 1977.

32. SHEFFIELD, L. J , SCHLESINGER, P., FAULL, K , HALPERN, B. J , SCIIIER, G . M . , COTTON, R. G. H., HAMMOND, J. and DANKS, D. M . - Iminopeptiduria, Skin Ulceration and Edema in a Boy with Prolidase Deficiency. J. Pediat. 91: 578. 1977.

33. WARNE, G. +.. ALFORD, F. P, CHISHOLM, D.J. and COURT, J. - Glucagon and Diabetes. Complete Suppression of Glucagon by Insulin in Human Diabetes. Clinical Endocrinology. 6. 277-284, 1977.

34. WATSON, B. M , SCHLESINGER, P. and COTTON, R. G. H. - Dihydroxanthopterinuria in Phenylketonuria and Lethal Hyperphcnylalanincmia Patients, Clin. Chim. Acta 78: 4 1 7, 1977.

T H E S I S P A S S E D FOR H I G H E R D E G R E E Doctor of Philosophy

1. CARTWRIGHT. E. C. - Studies on Phenylanine Hydroxylase and Connective Tissue Disorders Using Cell Culture

THESES IN PROGRESS Doctor of Philosophy CHOO. H. K. - Cell Culture and Biochemical Studies on Phenylketonuria F1RGA1RA, F. — Studies on Human Dihvdropteridine Reductase GRAYSTONE, J. E. - Insulin as a Growth Hormone

McKERN, N. - Structure and Function of Serum Growth Hormones Related to Somatomedins, including Martin's Peptide

Doctor of Medicine

CARMICHAEl, A. — Epidemiology of Health and Illness in Young Families in Brunswick

SILLENCE, D. O. - Bone Dysplasias - Genetic and Ultrastructural Studies with Special Reference lo Osteogenesis Imperfecta

Master of Science

ALEXANDER. S. - Study of Altitudes lo Food and Ealing Habits of Mothers of Obese and Normally Nourished Infanls

BIRNER. R. — Chromosomal Location of Genes Coding for Human Immunoglobulin Production

PAEDIATRICS 145 FIELD, J. — Mechanism of Insulin Super-sensitivity Accompanying Reduced Meal Frequency

MANN, J. — Copper Metabolism of Copper Deficient Mouse Mutants in Relationship to Menkes' Syndrome MARTIN. G. — Effects of Increased Dietary Cholesterol Intake on Metabolism on Cholesterol Levels in Children with Familial IIypercholesterolaemia

WATSON, B. M. - Pterins in Human Urine

YAP, C. — Studies of Mammalian Phenylalanine Hydroxylase Master of Social Work RYAN, M. M. - Psycho-social Aspects of Low Birth Weight

S U B S T A N T I A L G R A N T S R E C E I V E D Grants have been recieved as follows: —

Apex Foundation for Research into Mental Retardation.

Australian Hospital and Health Services Commission.

Australian Kidney Foundation.

Clive and Vera Ramaciotti Foundation.

Department of Aboriginal Affairs.

National Health and Medical Research Council.

Percy Baxter Charitable Trust.

Royal Children's Hospital Research Foundation.

Royal Children's Hospital Research Grants Committee.