Search

Seventy years ago, the Swedish pediatrician Rolf Kostmann (1909-1982) was the first to report on a previous unknown lethal hereditary neutropenia in infants, Kostmann's disease. This essay presents the man behind the syndrome rather than focusing on the disease itself.

Pediatrics directly and indirectly played an important role in the history of the Nobel Prize in physiology or medicine. However, the history of the Nobel Prize and pediatrics goes beyond the actual laureates. Based on original files in the archive of the Nobel committee of physiology or medicine at the Karolinska Institute in Sweden, this overview aims to shed new light on why the international pioneers of pediatrics Abraham Jacobi (1830-1919) and Otto Heubner (1843-1926) were nominated but never received the prize in 1918. Moreover, Clemens von Pirquet (1874-1929), one of the founders of this journal in 1910 (previously known as Zeitschrift für Kinderheilkunde), also appears in the Nobel records during the first decades of the twentieth century, nominated by Heubner and others., Conclusion: We argue that studies of Nobel nominations give new opportunities to study not only the selection process for Nobel laureates, but also to explore which pioneers were seen as the most outstanding at a particular point in time and why. What is known? • Recent historical research suggests that Nobel Prize nominations can help to reconstruct trends in medicine over time. What is new? • This paper takes a new approach on the history of pediatrics and shows why the internationally famous pediatricians Abraham Jacobi, New York, and Otto Heubner, Berlin, were runners-up for the Nobel Prize hundred years ago.

Unlabelled: Barth syndrome (OMIM #302060) (BTHS) is an X-linked disorder of lipid metabolism characterized by skeletal myopathy, neutropenia, growth delay, and cardiomyopathy. It is caused by mutations in the tafazzin gene (TAZ), which lead to decreased production of an enzyme required to produce cardiolipin, a component of the inner mitochondrial membrane necessary for proper functioning of the electron transport chain. The most common initial presentation of BTHS is significant heart failure due to cardiomyopathy, which is the main cause of death in infancy or childhood. On the other hand, some patients have limited clinical features of BTHS. These patients may be overlooked or misdiagnosed with unclassified congenital myopathy, especially when heart failure is not clinically significant. However, these patients could also develop significant heart failure or life-threatening arrhythmias during or even after childhood. Heart failure in BTHS is often responsive to standard medical therapy, indicating early diagnosis is critical. Diagnostic clues of BTHS in the subclinical stage of heart failure include family histories, findings of lipid storage myopathy in the skeletal muscle biopsy, and elevated plasma brain natriuretic peptide levels. The genetic analysis of TAZ is the only confirmatory method for the diagnosis of BTHS., Conclusion: physicians should be aware of the possibility of this disease and carry out genetic studies when it is considered.

Attempts at the arterial switch operation predated the Senning and the Mustard venous switch procedures. No patients survived the earlier arterial switch methods. Therefore, from the 1960s to the 1980s, venous switch surgeries became the dominant treatment method for transposition of the great arteries. The Brazilian cardiac surgeon, Adib Jatene, reported the first successful arterial switch in 1975. The history of the earlier arterial switches performed in the 1950s and 1960s is not well known. This article discusses that early history.

John Locke (1632-1704) is primarily remembered for his highly influential philosophical works regarded as the engine of the Enlightenment. It is less well known that Locke also was a highly regarded and influential physician. In 1666, Locke performed a postmortem examination of an 18-month-old child who had physical signs of rickets. Locke, a medical student at this time, attributed rickets as the cause of death. However, Locke described and recognized severe cardiac abnormality and speculated that right-to-left interatrial shunting was part of rickets. Locke's clearly described clinical history and postmortem findings are more consistent with a congenital cardiac malformation, an Ebstein's anomaly, in addition to the rickets. Locke did not consider this case as other than rickets. His opinion was not challenged when the case report was re-presented in the past half century. This article forces a reevaluation of the 17th-century understanding of infant cardiovascular physiology and pathology: Locke clearly gives one of the earliest descriptions of right-to-left shunting through the patent foramen ovale. It is unfortunate that Locke apparently did not discuss his postmortem findings with his contemporary Richard Lower (1631-1691), whose celebrated masterpiece on the heart, Tractatus de Corde, was published in 1669.

Background: The Union of National European Paediatric Societies and Associations was founded in 1976 in Rotterdam. Thirty years later, the authors--former presidents and secretaries general--present a retrospective on the activities and achievements of UNEPSA. In 2006, 36 of 46 (78%) European countries, including some countries of the former Soviet Union, were members of UNEPSA. UNEPSA has created a forum for the mutual discussion of matters concerning paediatrics. UNEPSA is closely linked with the International Pediatric Association (IPA). It was never UNEPSA's ambition to promote a uniform European paediatrics. In the 30 years of its existence, it became clear that the diversity of paediatric care in different countries in Europe is extreme. During the "cold war", UNEPSA was able to cross boundaries between socialist and capitalist countries in Europe, and it was due to the activity of individual members of the UNEPSA council that clinical co-operation and research activities were initiated crossing many political borders. Annual meetings of national paediatric presidents focus on the most urgent problems of paediatric health care. "Europaediatrics" became the tri-annual congress for all general paediatricians and paediatric sub-specialists in Europe. The main research activities of UNEPSA concentrated on identifying the demography of primary, secondary and tertiary care paediatrics in Europe., Conclusion: UNEPSA is an active paediatric association representing more than three quarters of all European countries. After 30 years, it is still an expanding and vital instrument in improving the medical care of all children and the co-operation of their carers in Europe.

Ernst Moro was born on December 8, 1874, in Ljubljana, the capital of Slovenia, then part of the Austro-Hungarian empire and attended university in Graz, Austria. Pediatrics, initially regarded as a part of internal medicine, was in its early days as an independent field at universities in German-speaking Europe. The first Chair of Pediatrics had been established in Vienna, Austria in 1884. Ten years later Germany was granted its first Chair in Berlin. Escherich, who held the first Chair of Pediatrics at Graz, was seen as one of the most respected pediatricians in Europe. Therefore, he was invited in 1904 to represent pediatrics together with the American Abraham Jacobi at the International World Congress on Science at the Saint Louis World Exposition in the United States. The high mortality of nearly 25% in infancy was seen as one of the main problems. Escherich's scientific work had established him as the leading bacteriologist. Moro's pediatric career started in Escherich's laboratory with experimental research on the physiology of digestion in infants. In 1900 he presented the first bacteriological characterisation of Lactobacillus acidophilus. In 1906 he received his venia legendi for his work on the bacterial flora in physiological and pathological conditions of the infantile intestine. In 1908 Moro won international reputation for his simple percutaneous skin test for tuberculosis, which was widely used in many countries as the "Moro test" at least until the 1960s. He described a carrot soup and other dietary prescriptions as helpful for diarrhoeal disease. In 1911 he became Head of the Children's Hospital at Heidelberg. In 1918 he described some features peculiar to the first 3 months; according to him this trimenon should be regarded as an own entity like the newborn period. The most famous part of the paper was the description of a milestone in the infant's neurological development, the Umklammerungsreflex (embracing reflex). In 1919 Moro was promoted from associate professor to the first 'Ordinarius', thus founding the first Chair of Pediatrics at Heidelberg and marking the beginning of a scientifically most fruitful period of international collaboration. However, as his wife was of Jewish origin, Moro slowly withdrew from hospital service starting in 1933. After early retirement in 1936, he worked as a pediatrician at home until 1948.

The first issue of the Monatsschrift für Kinderheilkunde (Monthly Journal of Pediatrics) appeared in October 1903 with contributions by leading European pediatricians. The editor was Arthur Keller, who remained responsible until 1934. As a novelty, the journal contained detailed reviews of world-wide pediatric literature. After Keller's death in 1934, Georg Bessau became editor-in-chief for the next 10 years. The first issue after the second world war appeared in 1948/49 and was edited by Hans Kleinschmidt, Göttingen. In 1962 the editorial responsibility was transferred to Karl Heinz Schäfer, whom joined Hans Ewerbeck in 1970. The monthly journal written in German deals with informations for pediatricians in children's hospitals and in practices and with scientific contributions in all fields of pediatrics and related areas. The present editors are Flix Bläker and Werner Schröter. In 1949 it became the official organ of the Deutsche Gesellschaft für Kinderheilkunde (German Society of Pediatrics), endorsed by the impressum since 1956. In 1993 the subscription of the journal became part of the membership fee of the Deutsche Gesellschaft für Kinderheilkunde.

Otto Heubner can be counted among the fathers of pediatry. Beginning as an autodidact, he became pediatrist by chance, holding the first university chair in Germany (1884) exclusively devoted to pediatry; he needed no Dr. med. habil. thesis. Observing the little patients was his primary interest; for the infants treated in the hospital he introduced "aseptic environments". Heubners main scientific achievements were the treatment of infectious and stomach-intestinal diseases. He described "Endarteritis syphilitica" and found Meningococci in the cerebrospinal fluid. "Serious deficiency of digestion" (Coeliac disease) became known through his work. Together with his friend M. Rubner he created the notion of EQ (Nutrition Quotient). Also, he realized the functional effects of orthotic albuminuria. Heubners centers of activity were Leipzig and Berlin, where he had a large community of followers; his successor, recommended by him, was A. Czerny.

"80 years Department of Pediatrics at the University of Giessen (1912-1992)": Occasion to remember the development of this discipline obtaining only late independent Professorships and Clinics at German Universities. The reasons therefore are discussed.