It is a great pleasure for me to see this work in print. As outlined by Dr. Traupe, at least 20 different types of ichthyosis have been identified to date. This book will effectively help to establish a correct diagnosis, as a basis for genetic counseling. Dr. Traupe proposes a new classification of the ichthyoses, based on reason able clinical criteria. He lets the reader share in his enormous knowledge and safely guides him through the difficult field of nomenclature. He describes the particular nature of these scaling disorders in a manner as simple as possible. During the last few years, research on ichthyosis has become a fascinating scientific challenge due to the introduction of novel investigative approaches. The main advantage of this book lies in the fact that the author successfully combines recent achievements made in lipid analysis, enzymology, electron microscopy, and molecular genetics. Heiko Traupe began his career in dermatology 11 years ago, as a resident in the Department of Dermatology in Munster. As we were both interested in the genetic aspects of skin diseases, this was the starting point of a most fruitful collaboration, which is still continuing. During our common work in Munster, Dr. Traupe was able, thanks to his unceasing clinical curiosity, to describe many novel aspects within the field of ichthyosis, and I took pride in coauthoring papers on new items such as cryptorchidism as a fea ture associated with X-linked recessive ichthyosis, autosomal dominant lamellar ichthyosis, and alopecia ichthyotica."

What is the Matter? 245 What is the Plan? 313 Why did it Happen? 325 Could it Happen Again? 326 Section 6 Skin Defects Introduction 333 Vascular Birthmarks 337 Pigmented Birthmarks 357 Partial Thickness Defects 369 Keratinisation Disorders 377 Blistering and Bullous Disorders 381 Neuroectodermal Dysplasias 387 Index 401 Introduction What is a normal newborn infant? To answer that question requires an experience large enough to know the many variations that are considered within a normal range. Some of these variations can be measured, such as weight and length. Others are less easily measured, but have come to be recognized as common variants such as molding of the head or temporary deformations of limbs caused by unusual intrauterine positions. One way to recognize deviations from normal that may require intervention is to have a long experience examining many infants. Another way is to enrich a finite experience by access to a superb collection of photographs taken over a long period by a careful observer. No one student can possibly see all the normal and abnormal variations in newborn infants in the time allotted in most curricula, or for that matter in a lifetime. We are dependent on shared information.

Ferment, a sign of progress in any scientific field, has previously been lacking in the area of cryptorchidism, where the only activity has been in improving operative methods. Now, however, profound changes in the care of boys with cryptorchidism are being brought about; ideas are arising from a fresh look at comparative anatomy, and histological and experimental observa tions are being supplemented by clinical tests made possible by new hormonal agents. The treatment of cryptorchidism begins with its recognition by the pediatri cian, who until now has shown little interest because of disappointing results from chorionic gonadotropin administration. As for the surgeon, his bent toward restoration of normal anatomical relationships has kept his attention focused on the development of better surgical technics to bring the recalci trant testis into the scrotum. Both specialists have avoided the primordial question of why the testis did not descend properly. If this were known they would treat the cause of cryptorchidism, and not be satisfied merely with trying to correct its end result. As one reads this book, one sees that in most patients cryptorchidism is not caused by some anatomical structure blocking the way or by some deformity of the testis interfering with the transport mechanism. Rather, deficiencies in the hormonal environment of the fetus retard the developmen tal sequences essential to the normal differentiation and descent of the testis. The clinical solution is thus to provide the deficient substances."

JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder, or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.

Although generally considered adult disorders, anxiety and depression are widespread among children and adolescents, affecting academic performance, social development, and long-term outcomes. They are also difficult to treat and, especially when they occur in tandem, tend to fly under the diagnostic radar.

"Anxiety and Depression in Children and Adolescents" offers a developmental psychology perspective for understanding and treating these complex disorders as they manifest in young people. Adding the school environment to well-known developmental contexts such as biology, genetics, social structures, and family, this significant volume provides a rich foundation for study and practice by analyzing the progression of pathology and the critical role of emotion regulation in anxiety disorders, depressive disorders, and in combination. Accurate diagnostic techniques, appropriate intervention methods, and empirically sound prevention strategies are given accessible, clinically relevant coverage. Illustrative case examples and an appendix of forms and checklists help make the book especially useful.

Featured in the text:

Developmental psychopathology of anxiety, anxiety disorders, depression, and mood disorders.

Differential diagnosis of the anxiety and depressive disorders.

Assessment measures for specific conditions.

Age-appropriate interventions for anxiety and depression, including CBT and pharmacotherapy.

Multitier school-based intervention and community programs.

Building resilience through prevention.

"Anxiety and Depression in Children and Adolescents" is an essential reference for practitioners, researchers, and graduate students in school and clinical child psychology, mental health and school counseling, family therapy, psychiatry, social work, and education.

"

The death of a friend, a colleague, a relative, or a patient can be a devastating experience, particularly when that death is. self deter mined. It is devastating to us as human beings to lose someone we cared about; it is devastating to us as professionals to wonder what we might have done that could have helped; and it is devastating to us as psychiatrists, because it makes us realize that for all we do know, there is still a great deal more that we do not know. When the person exhibiting suicidal behaviors or completing the act of self-murder (as the Germans call it) is a child or adolescent, the tragedy seems even greater. Completed suicide among the younger age groups (especially in the English-speaking and Scandinavian countries) has increased dramatically over the past three decades and is continuing to do so. Suicide is now, in fact, the second cause of death among adoles cents and young adults in the United States, according to the latest statistical reports. It is exceeded as a cause of death in these ages only by accidents (some of which are undoubtedly hidden suicides, probably more so among such younger persons), with suicide death the past few rates even rising above those from homicide during years."

Endocrine conditions requiring surgical intervention in the pediatric age group are uncommon. When diagnosed, they are the source of great in terest and, often, considerable debate. This is understandable, since few centers and even fewer individual surgeons can draw on vast experience of this subject. The great divergence of opinion regarding management is also understandable in that pediatric endocrine lesions often differ considerably from their adult counterparts in histology, natural history and response to treatment. Pediatric endocrine lesions are also, as a rule, less frequently malignant. In addition to the great strides made in surgical and anesthetic technique and operative monitoring, progress in four areas has substantially advanced the management of endocrine dis orders in the pediatric age group in the last decade: imaging, pathology, pharmacology and genetics. The new imaging tools, ultrasonography, computed tomography and magnetic resonance imaging, have added great diagnostic possibilities. More recent developments, such as radio nuclide imaging for the adrenal gland and the possibility of using tagged antibodies, promise to expand our imaging horizons even further. In the field of pathology, the develop ment of immunocytochemical markers (e. g. , monoclonal antibodies), the refinement in special stains and the continuous perfection of fine needle aspiration biopsies offer great new diagnostic as well as research capabil ities. Newer pharmacological agents, such as the alpha and beta blockers, the calcium channel blockers and thyroxine analogs, add a whole new level of safety to the management of the potentially lethal pheochromocytoma.

The death 'Of a child, 'One wh'Ose vulnerability and unfilled promise c'Omp'Ounds the tragedy 'Of any death, t'Ouches us deeply. When that death 'Occurs due t'O the generally unexpected and sudden loss 'Of brain functi'On, it is particularly P'Oignant. And yet, when death c'Omes, it sh'Ould be diag- nosed expediti'Ously. The professi'Onal resP'Onsibilities 'Of the physician require that he b'Oth rec- 'Ognize brain death and inf'Orm the family 'Of its occurrence. In additi'On, since recent advances in transplantati'On provide the P'Ossibility 'Of using the 'Organs 'Of a brain-dead child t'O save the lives 'Of 'Other children, the c'Oncepts 'Of beneficence an~ charity further c'Ompel physicians t'O facilitate such transplantati'Ons by inf'Orming the family about this P'Ossibility. Criteria 'Of brain death have been refined 'Over about 30 years since the term coma depasse (bey'Ond c'Oma) was coined by M'Ollaret and G'Oulon in 1957 (Kaufman and Lynn, 1986). The last maj'Or criteria ev'Olved 'On a nati'Onal level were th'Ose 'Of the President's C'Ommissi'On in 1981. H'Owever, studies t'O date have c'Oncentrated 'On adults, and relatively little work has been rep'Orted ab'Out devel'Oping brain death criteria f'Or the y'Oung. Indeed, the advis'Ors t'O the President's Com- missi'On suggested that there are significant-although n'Ot well defined~ifferences in the ability of the brains 'Of th'Ose under five years 'Of age t'O rec'Over from clinical states that W'Ould be accepted as indicative 'Of c'Omplete and irreversible l'OSS of brain functi'On in adults.

The twentieth century was the century of the development of morphological cerebral imaging by tomodensitometry (TDM) and Magnetic Resonance Imaging (MRI). In recent years new brain imaging methods were used in adults with neurological lesions, and more recently in adults with psychiatric disorders. Now it is also possible to use, most of these morphological and functional brain imaging methods in children.
This book presents the main morphological and functional brain imaging methods that we can use in the child. Two main applications are developped: physiopathological and therapeutical interest. The physiopathological approach is of a great interest, coupled with clinical evaluation in psychomotor disorders like hyperkinetic or Tourette syndrom, and in developmental disorders like autistic syndrom, mental retardation, Rett Syndrom ...

In just under three decades, the world has witnessed an enormous rise in obesity with a parallel growth in cardiometabolic disease risk factors characterized by insulin resistance, dyslipidemia, and hypertension, together known as the metabolic syndrome - conditions previously unheard of in children and adolescents. During this time, we have little knowledge of the global and cumulative detrimental health effects of childhood obesity. As obese children age, not only will their health be negatively affected, but infertility and pregnancy complications associated with the metabolic syndrome will affect generations to come. The work force will undoubtedly be affected because of increased sick days and decreased work productivity. Identifying children and adolescents at the earliest stages of chronic disease onset should be the goal of clinical practice, yet there is no clear guidance for defining the risk of metabolic syndrome or appropriate risk-factor thresholds in these groups. If children are identified early in the disease process, lifestyle and clinical interventions can be instituted when they are potentially more effective. Pediatric Metabolic Syndrome: Comprehensive Clinical Review and Related Health Issues approaches the pediatric metabolic syndrome by elucidating its effects on specific organ systems and by considering the problem through understanding the social, psychological and economic consequences of it. The Editors have recruited an invited group of esteemed experts in the field to provide the most timely and informative approaches on how to deal with this health crisis. Through educating our practitioners, our future researchers, our health and community organizations, our legislators and our families and children, we have the best chance at improving the health trajectory of the next generation.

Evidence-Based Neonatal Infections is expertly written by David Isaacs, an experienced author renowned for his knowledge in both pediatric infections and evidence-based medicine. It critically analyses the evidence for decision making in neonatal infections. Evidence-Based Neonatal Infections * The first evidence-based text on neonatal infections * Provides practical guidance where evidence is poor * Complements David Isaacs' Evidence-Based Pediatric Infectious Diseases (9781405148580) Practical and evidence-based, Evidence-Based Neonatal Infections is designed to help the clinician with day-to-day decisions on the care of newborn babies with possible, probable or proven infections. It considers clinical questions relevant to neonatologists, analysing the evidence carefully and providing recommendations for optimum management of neonatal infectious diseases, whilst reflecting on: * Efficacy and safety * Antibiotic resistance * Cost effectiveness * Adverse effects * Ethical considerations Evidence-Based Neonatal Infections provides a unique reference for neonatologists, pediatricians, trainees, specialist nurses; general practitioners, microbiologists, infection control doctors, and all staff in neonatal units.

This volume represents a collection of topics presented at the Tenth Annual Symposium of the Barrow Neurological Institute. No attempt is made to be comprehensive but rather an attempt is made to give adequate attention to the major important topics in this field. Neuro developmental biology, pathophysiology, and laboratory evaluation are discussed. Subjects include hypoxic ischemia, intraventricular hemorrhage, fetal neurosurgery, surgery of hydrocephalus, problems of apnea, ultrasound and CT imaging techniques, electrodiagnosis and ethical and economic issues. This volume will be of interest to neurologists and neurosurgeons in general, and especially to pediatric neurologists and neurosurgeons, and perinatologists. Richard A. Thompson, M. D. John R. Green, M. D. Stanley D. Johnsen, M. D. vii Contents Contributors v Preface vii Acknowledgments xi CHAPTER 1 Use of Fetal Neurosurgery for Experimental Studies of Structural and Functional Brain Development in Nonhuman Primates 1 Pasko Rakic and Patricia S. Goldman-Rakic CHAPTER 2 Pathogenesis of Perinatal Hypoxic-Ischemic Brain Damage 17 Robert C. Vannucci CHAPTER 3 Current Concepts of Pathophysiology of Perinatal Intraventricular Hemorrhage 41 Jan Goddard-Finegold CHAPTER 4 New Therapies for Asphyxia Neonatorum and Intraventricular Hemorrhage of the Premature 57 Gary W. Goldstein CHAPTER 5 Cranial Sonography and CT of the Infant 67 Diane S. Babcock ix x Contents CHAPTER 6 Cross-Sectional and Longitudinal Studies of Brain Stem Auditory-Evoked Potentials in High-Risk Infants 85 James J. Stockard, Janet E. Stockard, and Allen Merritt CHAPTER 7 Neurosurgery in Congenital Defects Other Than Hydrocephalus 111 Hector E."

Pediatric and Adolescent Osteosarcoma provides a historical review of the nature of osteosarcoma and the conflict that accompanied the introduction of adjuvant therapy for osteosarcoma culminating in accepted and prevailing methods of current therapy. It outlines concepts in Epidemiology and Etiology, and provides chapters on pathology and radiologic characteristics of osteosarcoma, surgical therapy tailored specifically for treatment of primary tumors in pediatric/adolescent age group, treatment of pulmonary and extra pulmonary metastases and complications, as well as the role of radiation therapy. The volume concludes with a review of differences and similarities in the management of osteosarcoma in adults as compared to pediatrics / adolescents and new laboratory and animal investigations currently in progress to develop effective diagnostic and therapeutic approaches to improve the outcome.

In essence the scope and intensive coverage of the book provides a historical perspective of the advances made over the past 30 years and emerging concepts and prospects for new diagnostic and therapeutic approaches. This is based upon past experiences and new discoveries. It also provides a unique opportunity for pediatric and adult medical oncologists, physicians in training, orthopedic surgeons, pathologists, radiologists, radiotherapists, oncology nurses and allied professionals involved in the care of pediatric/adolescent patients with osteosarcoma to become acquainted with prevailing methods of treatment and new and evolving concepts and developments.

Urticaria is one of the most common dermatological and allergological cutaneous reactions and, compared to other diseases, it is easily recognized by patients and physicians alike. Nevertheless, the disease is highly complex regarding its eliciting causes, its clinical manifestations and its therapy. Thus, a famous New York dermatologist once mentioned that he would rather have a lion than a patient with chronic urticaria walk into his office. This may seem surprising since, to the uninitiated, different types of urticaria look alike, and the pathomechanisms are rather well understood, with mast cells being almost invariably the main effector cells. In 1986, a monograph of the first editor (Prof. Czarnetzki, now with the married name Henz) appeared, giving a detailed and thorough review of the then current state of knowledge regarding all aspects of the disease. Since then, two updates of this book have appeared in the German language, with coworkers of the clinic of Prof. Henz helping in the revision of the various chapters of the old monograph, and with particular emphasis on practical aspects of the disease. The present book is mainly a translation of the second German edition, with only minor updates and with more citations from the literature since the 1986 monograph is no longer available for purchase.

Tachdjian's Procedures in Pediatric Orthopaedics is a brand new derivative resource from Tachdjian's Pediatric Orthopaedics, 5th Edition, the classic comprehensive offering on the diagnosis and treatment of pediatric musculoskeletal disorders and trauma. For over 40 years, Tachdjian's has been the trusted resource in pediatric orthopaedics, written by experts at Texas Scottish Rite Hospital for Children, a world-renowned pediatric hospital. This procedures-focused text includes more than 70 colorized, illustrated procedures, providing step-by-step surgical walkthroughs with accompanying intraoperative illustrations. This procedures-only text will appeal to general orthopaedic surgeons, pediatric orthopaedic surgeons, orthopaedic residents, and anyone who needs to quickly and easily learn new techniques or refresh their understanding of challenging procedures. 3 brand new procedures not included in Tachdjian's Pediatric Orthopaedics, 5th Edition: Ganz Periacetabular Osteotomy, Ponte Osteotomy, and Sacro-Iliac Screws. Instructional videos that demonstrate closed reduction of the left hip, pemberton osteotomy, salter osteotomy, and more. Expert Consult eBook version included with purchase. This enhanced eBook experience allows you to search all of the text, figures, images, and references from the book on a variety of devices.

In den vergangenen Jahren sind in der Medizinischen Universitatsklinik Wurzburg in Zusammenarbeit mit dem Hochschulinstitut fur Leibesiibungen die quantitativen Veranderungen hamodynamischer GraBen, ausgehend von Ruhebedingungen, bei karperlicher Belastung und in der daran anschlieBenden Erholungsphase kontinuier- lich verfolgt worden. Die Ergebnisse diescr Beobachtungen sind im einzelnen bereits in mehreren Veraffentlichungen mitgeteilt worden (SCHNEIDER, 1964, 1965; GATTEN- LOHNER, 1965, 1966; HisEL, 1965). Die vorliegende Arbeit steHt den Versuch dar, eine zusammenfassende Beurteilung uber das Verhalten der wichtigsten Kreislauf- graBen wie Herzminutenvolumen, Schlagvolumen, Frequenz, Blutdruck und Kreis- laufzeiten beim Sportler in den verschiedenen Phasen einer ergometrischen Belastung zu geben. Dabei wurde besonderes Gewicht auf das korrelative Verhalten dieser hamodynamischcn GraBen gelegt. II. Untersuchungsmaterial Das Untersuchungsgut umfaBte 72 Personen. Es handelte sich dabei um Mitglieder Wurzburger Sportvereine, um Sportstudenten der Universitat Wurzburg und um einige Bundeswehrsoldaten. Entsprechend der Spezialdisziplinen wurden die Sportler in 5 Gruppen eingeteilt: Gruppe I: Dauersportler Sie umfaBt 13 Mittel- und Langstreckler sowie einen StraBenradrennfahrer. Das Training erfolgte vorwiegend nach dem IntervaHprinzip. Diese Gruppe enthielt die besttrainiertesten Sportler. Gruppe II: Kurzstreckler Sie enthalt 12 Leichtathleten, deren Spezialdisziplin kein Ausdauertraining erforderte. Gruppe III: Unspezialisiertc Sportler In dieser Gruppe wurden Sportstudenten und einige gut trainierte Bundeswehr- angeharige zusammengefaGt. Diese Gruppe von 21 Personen konnte als weniger trainiert klassifiziert werden. Gruppe IV: Ruderer Bei diesen 5 Sportlern handelte es sich um die karperlich kraftigste Gruppe. Einer der Ruderer ist Mitglied des deutschen Weltmeisterschaftsachters.

Der Herzbeutel ist ein Stiefkind der Kardiologen. Seine Existenz wird dem Kliniker vor allem unter pathologischen Umstanden bewuBt, z. B. wenn bei der Pericarditis constrictiva oder bei der Herzbeuteltamponade schwere Storungen der Herzfunktion eintreten. Fiir den Morphologen ist es reizvoll, die Ergebnisse iiber die Funktion des Herzbeutels unter normalen und pathologischen Bedingungen auf der Grundlage einer funktionellen Betrachtung der Morphologie zu erortern. Dieser Versuch solI hier unternommen werden. II. Morpbologie des Herzbeutels 1. Einige makroskopische Befunde Der Herzbeutel umgibt allseitig das Herz als eine in Grenzen dehnbare, binde gewebige Kapsel, die durch den Perikardialspalt von der Herzoberflache getrennt ist. Einige Funktionen des Perikards werden schon aus seiner makroskopischen Betrachtung verstandlich. Der Herzbeutel ist als Scheidewand zwischen Herz und Lungen eingeschaltet und vermag deshalb das Dbergreifen entziindlicher Veranderungen von Lungen und Mediastinum auf das Herz zu verhindern odeI' zumindest zu erschweren. Das Perikard ist breit mit dem Zwerchfell verbunden. Besonders fest ist es in einem ventralen, schmalen, quergestellten Saum mit dem Diaphragma verwebt."

As 'seasoned campaigners' we offer our readers more than 60 joint practice years of commonsense experience on children and their prob lems. Child care is a large and fascinating part of general family practice. More than any other discipline it is a mix of understanding the wide range of normal and abnormal development, of skilful diagnosis and treatment of treatable conditions, of long-term care for handicapped children, and of organizing and carrying out prevention. F or all this and more the physician has to rely on sound knowledge and understanding of the child, parents, family, social and community conditions, available services and the likely natural history of the condition - and to dispense all this with humanity, sense and sensi bility. We have divided the book logically into 6 sections: (I) Factual background. (2) Universal problems of behaviour and development. (3) Common clinical disorders, so frequent and yet often so dif ficult to manage. (4) Social, family and community factors that create and influ ence many problems of childhood. (5) How to use available services and resources with discrimina tion and sensitivity. (6) The importance of understanding and managing the whole child. We have no single group of readers in mind. We hope that our views will be appreciated, for example, by parents, nurses, health visitors, general practitioners, community physicians and paedia tricians - in fact all who care for children."

The state of health care is reflected by perinatal and neonatal morbidity and mortality as well as by the frequencies of long-term neurologic and developmental disorders. Many factors, some without immediately rec ognizable significance to childbearing and many still unknown, undoubt edly contribute beneficially or adversely to the outcome of pregnancy. Knowledge concerning the impact of such factors on the fetus and sur viving infant is critical. Confounding analyses of pregnancy outcome, especially these past two or three decades, are the effects of newly un dertaken invasive or inactive therapeutic approaches coupled with the advent of high technology. Many innovations have been introduced with out serious efforts to evaluate their impact prospectively and objectively. The consequences of therapeutic misadventures characterized the past; it seems they have been replaced to a degree by some of the complications of applied technology. Examples abound: after overuse of oxygen was recognized to cause retrolental fibroplasia, its restriction led to an in crease in both neonatal death rates and neurologic damage in surviving infants. Administration of vitamin K to prevent neonatal hemorrhagic disease, particularly when given in what we now know as excessive dos age, occasionally resulted in kernicterus. Prophylactic sulfonamide use had a similar end result. More recent is the observation of bronchopul monary dysplasia as a complication of respirator therapy for hyaline membrane disease."

This volume is a second undertaking - following the proceedings of the first workshop published in 1982 under the title Genetic Basis of the Epilepsies -to review our current knowledge on the genetics of epilepsies. It also developed from a workshop held in Berlin in September 1986. It contains the papers presented by the speakers, but is revised to incorporate the results of the stimulating discussions. The main goals of the book are to stimulate communication across traditional disciplinary lines and further collaborative research, as well as to provide pediatricians, neurologists, and geneticists with a basic knowledge for genetic counselling while at the same time putting emphasis on the evidence of heterogeneity in this field. This book contains separate sections on population rates, family studies on selected syndromes, genetics of EEG characteristics, morbidity risk in offspring, and special methods and topics. Among the topics covered are the prospective evaluation of seizure morbidity in offspring of epileptic patients and the relationship of pharmacogenetics to epilepsy, two questions of current interest. The editors are greatly indebted to Mrs. Ilsebill Brookes, secretary of the Department of Neurology, Universitatsklinikum Rudolf Virchow, Free University Berlin, for her valuable assistance in planning and arranging the workshop. Major funding was provided by the Deutsche Forschungsgemeinschaft; Stiftung Michael; Freie Universitat Berlin; Boehringer, Ingelheim; Ciba-Geigy, Frankfurt; Desitin, Hamburg; Hoff mann-La Roche, Grenzach; Knoll, Ludwigshafen; Labaz, Munich; Nordmark, Uetersen; Parke-Davis, Munich, and Schering, Berlin. The editors are indebted to these institutions and firms for their generous support."

Disturbed children constitute a sizeable and varied percentage of the child population. Generally, two to three percent have severe, longstanding problems and 20 to 30 percent have current, more transient problems. While preventive efforts are needed, it also seems obvious that many children will continue to function in a disturbed and disturbing fashion and will continue to require vari ous kinds of therapeutic interventions. These interventions will mainly occur in school and community settings. This book attempts to present a picture of some fairly popular models of intervention and to consider important related issues. There are probably as many ways of coping with disturbed children as there are creative, caring persons attempting to do the coping. A growing literature exists on ways of conceptual izing and intervening with such children. It was believed by the editor that an organized body of information on these concepts and techniques would prove useful to student and practitioner. A danger with such collections is that if they mainly portray novel or atypi cal techniques, while informative, the book quickly becomes passe and of li mited value. This book's focus is on contemporary thought, but also on orien tations that have stood the test of some time and the stability of the under lying theory."

Sepsis is still the leading cause of death in surgical and general intensive care units. Although our knowledge of this complex syndrome has increased in recent years, it is still far from com- plete. New therapeutic approaches have been developed. Some did not fullfill the high expectations for their use in clinical prac- tice; others are still under clinical evaluation. is the aim of this monograph to offer basic information on the It etiology, definition, pathophysiology, prevention and therapy of sepsis and septic shock to both the clinician and the scientist. We believe that it presents an extensive overview that may help the clinician to better understand and treat sepsis and septic shock. We also hope that this work will give some directions for future research both in the clinical field as well as the laboratory. Jena, Berlin, Jerusalem, K.Reinhart February 1994 K.Eyrich C.Sprung TableofContents I.Definition/Prognosis Sepsis and Septic Shock: Update on Definitions (Jo-L.Vincent) 0 0 0 0 0 0 0 0 0 0 0 0 ******** 3 Clinical Manifestations and Prognostic Indicators in Sepsis (R.C.Bone) ...0 * * * * * * * * * * * * * * * * * * * 16 What Determines Prognosis in Sepsis? Evidence for a Comprehensive Individual Patient Risk Assessment Approach to the Design and Analysis of Clinical Trials (W.A.Knaus, D.P.Wagner, F.E.Harrell, Jr.

JIMD Reports publishes case and short research reports in the area of inherited metabolic disorders. Case reports highlight some unusual or previously unrecorded feature relevant to the disorder, or serve as an important reminder of clinical or biochemical features of a Mendelian disorder.