Otitis media und ihre Komplikationen

Acute suppurative otitis media is one of the most common infections in childhood, caused by Streptococcus pneumonia, Haemophilus influencae, Staphylococcus or Pneumococcus infection via the Eustachian tube. Occasionally it can be blood-borne during viral or bacterial infections. With the improvement of tubal function as the child grows, the incidence of acute AOM decreases. Acute suppurative otitis media is a severe inflammation of the middle ear mucosa accompanied by severe pain, fever, reddening and bulging of the tympanic membrane which may perforate spontaneously. The course can be variable, but usually resolves without consequences. Diagnosis is made on the basis of otoscopic findings. It is important to promptly recognize possible complications such as mastoiditis, sinus thrombosis and brain abscess, which are rare but threatening situations. Treatment of acute suppurative otitis media consists of the application of topical decongestants, analgesics, antipyretics and antibiotics. In some cases paracentesis and adenoidectomy might be helpful. In the case of complications a surgical procedure is mandatory. As a residuum of acute otitis media a permanent perforation of the eardrum may result in chronic otitis media. Rarely, squamous epithelium may grow through the perforation into the middle ear and thus give rise to a cholesteatoma.     

Positionspapier zur Begrenzung lebenserhaltender Therapie im Kindes- und Jugendalter

“To preserve life, heal (or prevent) disease, reduce suffering“ are the words of the Hippocratic oath – the basis on which medical decisions are made and actions taken. Nowadays, the first and third of these requirements conflict increasingly in the context of life-support as a result of constant technological developments on an ever wider scale. The question must always be asked as to what serves the well-being of the patient, or even what threatens it. Thus, it is not possible to formulate recommendations and guidelines on how to proceed in a certain situation, but rather it is up to the individual to decide when it is more in the interests of the patient to refrain from life-support therapy in favour of a therapy aimed purely at relieving suffering. The physician or nursing staff must be aware that there are situations in which the death of the patient is consciously accepted and considered as the better option than continuing to live. Any decision taken against the preservation of life in the interests of the patient is always a question of choosing “the lesser of two evils”.     

Cannabisintoxikation bei einem 17-Monate alten Jungen

Cannabis intoxication is seen time and again. We report on a young boy who was somnolent and had a generalized hypotonia that lasted over 3 days. After exclusion of infection, trauma, or metabolic disturbance, these symptoms were found to be due to ingestion of hashish.     

Neonatale Ovarialzysten

The detection rate of neonatal ovarian cysts has markedly increased with the widespread use of ultrasonography during pregnancy. Up to now the decision for an invasive therapy has depended primarily on the size of the cyst. Recent insights into the spontaneous postnatal course of ovarian cysts within the 1st year of life have made it possible to prefer a less invasive procedure or at least to postpone any invasive treatment beyond the neonatal period. Having studied the literature published to date, we recommend the following approach: (1) When ovarian cysts are so large that they distend the fetal abdomen to the extent of dystocia of labor primary cesarian section or prenatal aspiration in utero shortly before the time of birth should be performed. (2) All ovarian cysts that are symptomatic in the neonatal period require immediate surgical intervention, preferably by laparoscopy. (3) In the case of ovarian cysts that are asymptomatic in the neonatal period the approach needs to be more tailored to the individual case. (a) Asymptomatic simple cysts with a diameter of more than 5 cm and with no tendency to regress spontaneously should be operated on within the neonatal period, preferably by laparoscopy. (b) In the case of any other asymptomatic neonatal ovarian cyst a wait-and-see policy with ultrasound monitoring can be adopted and continued at least until the end of the neonatal period.     

Postoperative Blutung trotz „normaler“ Gerinnungsdiagnostik?

We report the case of an unusually severe bleeding episode following adenotomy in an infant. Preoperative laboratory coagulation tests (aPTT, PT) were normal. We diagnosed type-1 von Willebrand disease (VWD) on the basis of abnormal history of the patient and her family and low von Willebrand factor values. The multimeric pattern was normal. We also give a summary of the current classification, diagnosis and therapy of VWD.     

Akute unilaterale Mydriasis

We report on a seven-year-old boy who presented with acute unilateral mydriasis after playing with Angel’s Trumpet, a common ornamental plant belonging to the Deadly Nightshade family. This plant contains tropane alkaloids which can cause anticholinergic symptoms following contact with or ingestion of parts of the plant. In this case the correct diagnosis was quickly established by patient history and thorough clinical examination. As expected, the symptoms resolved completely within 3 days. This case emphasizes the importance of a thorough history and clinical examination, which helped in this case to establish the correct diagnosis quickly and without the use of more expensive diagnostic procedures (e.g., computed tomography of the brain).     

Zellen, Feten, Menschenwürde

The goal of prenatal diagnosis includes the identification of severe fetal abnormalities. It inherently involves the option of abortion and as such is generally accepted by society as a means of primary prevention. In contrast, discarding of in vitro fertilized oocytes and cultured embryonic stem cells is controversial because the principle of human dignity is said to extend to human life at its very beginning. Considerable conceptual and ethical difficulties result from this definition of human life. These difficulties can be overcome by separating human life from the concept of a“person,” i.e., from traits such as self-consciousness, language, reasoning, self-determination, and so on. Human life can be biologically defined by its genotypic uniqueness. In this way, it can be awarded human dignity requiring basic respect in the context of respect for life as such. In contrast, a person is a developing being and can be protected by graded declaratory acts. The principle of restraining the basic right to life, such as in self-defense or war, is well entrenched in basic constitutional law.     

Eislaufunfälle bei Kindern

Background

Ice skating is among the most popular winter sports in Austria. Consequently, it is also one of the main reasons for winter sport accidents. The aim of the presented project was to analyse ice-skating injuries in children in order to identify possibilities for prevention.

Methods

In the period covered, 103 children were admitted to our clinic after ice-skating injuries. Their clinical records were analysed. Furthermore, all patients were sent a standardised questionnaire to document etiology, safety equipment, transport to hospital, and skating experience in years.

Results

The main causes of accidents were collisions and falls. Most accidents occurred within the first 30 min of skating, and significantly more accidents happened on natural rather than artificial ice (p<0.01). Fractures were seen in 33%, and the other patients sustained lacerations, contusions, distorsions, or head injuries. Only 12.5% of all patients were using safety equipment.

Conclusion

To prevent accidents in the future, more children should wear protective gear, and they should be encouraged to skate on artificial rinks with good ice quality. Additionally, physical exercises before skating could perhaps reduce the number of accidents in the first minutes.     

Pränatal diagnostizierte zystisch-hämorrhagische Raumforderung der Nebenniere

Prenatal cystic-hemorrhagic masses in the adrenal gland are rare. Neuroblastoma is the main differential diagnosis. Histology should always be performed. These masses are regularly combined with the Beckwith-Wiedemann syndrome. A case of a newborn with cystic abdominal mass detected prenatally in the fifth month of gestation is reported. The tumor was surgically removed. Histology revealed cystic-hemorrhagic cortical hyperplasia of the adrenal gland not associated to Beckwith-Wiedemann syndrome.     

Hyperthyroxinämie mit kardialer Symptomatik

Resistance to thyroid hormone (RTH) is a rare autosomal dominant inherited disease, which is usually caused by mutations in the TRβ-gene. The clinical presentation of RTH is highly variable and patients can show signs of euthyroidism, hypothyroidism and/or hyperthyroidism. We report on a 12¹¹/₁₂ year old girl who was admitted to hospital with tachycardia. Laboratory tests showed high levels of serum free T3 and free T4 in the presence of non-suppressed TSH concentrations. Further investigations, including molecular genetic tests, were then performed and revealed a rare case of thyroid hormone resistance. Our case report demonstrates that patients with RTH may manifest only cardiovascular symptoms.     

Vorgehen bei Säuglingen mit Verdacht auf Kuhmilchproteinallergie

This article presents recommendations for the diagnosis and treatment for infants with a suspected allergy to cow’s milk protein (KMPA) and replaces earlier publicized statements on this topic. Children with KMPA should receive a medically necessary diet, which permits normal growth and health. Non-indicated diets or unnecessarily long-lasting diets should be avoided because they negatively affect the quality of life of the children and their families and cause unnecessary costs. The prognosis for KMPA in infants and babies is good. Approximately 75% of affected children develop tolerance by 2 years old and 90% by school age.     

Pseudopubertas praecox

Germ cell tumors in childhood are rare and present as a heterogeneous group of tumors, typically situated in the midline. They originate in the central nervous system (CNS) in only about 20% of cases. We report on a 4-year-old boy with precocious puberty as an isolated symptom of a secreting germ cell tumor of the CNS.     

Risikomanagement in pädiatrischen Kliniken

Iatrogenic injuries as a consequence of medical errors are a serious problem in medicine. Critical incident reporting systems (CIRS) aim to reduce these errors and near-misses. By collecting data to identify and analyse risk factors, prevention strategies can be planned and instituted. In a multicentre study in 12 paediatric hospitals in northern Germany, CIRS were implemented, and data on the type and characteristics of errors and near-misses were collected. From 1,299 reports, 1,829 risk constellations were identified. The majority were attributed to medication errors (35%), failure to follow standards (24%), and errors due to incorrect documentation (15%). Our data show typical risks for paediatric hospital patients without measuring the real incidence of medical errors and near-misses. Based on the reports, risk management strategies were developed to reduce the risk of similar errors and adverse events. The study also shows that CIRS are realizable in paediatric wards and that they help improve patient safety.     

Schnelle und sensitive Keuchhustendiagnostik mit der Polymerasekettenreaktion

Zusammenfassung Fragestellung: Es sollte geprüft werden, ob der Einsatz der Polymerasekettenreaktion in der Keuchhustendiagnostik wesentliche Vorteile gegenüber den traditionellen Nachweismethoden erbringen kann, insbesondere, ob die PCR eine Erh?hung der Sensitivit?t und eine Verkürzung der Nachweisdauer bewirkt. Methode: PCR und kultureller Nachweis von Bordetella pertussis wurden anhand der Untersuchung von 201 Nasenabstrichen miteinander verglichen und die jeweiligen Ergebnisse in Bezug zum klinischen Bild gesetzt. Ergebnisse: Obgleich nicht alle klinischen Daten zur Verfügung standen, konnte gezeigt werden, da? die Pertussisdiagnostik mittels PCR der traditionellen mikrobiologischen Anzüchtung in allen Belangen überlegen ist. Die PCR verkürzt nicht nur die Nachweisdauer von 5–7 auf 1 Tag, sie weist auch wesentlich mehr apparente Infektionen nach als die Kultur. Schlu?folgerungen: Insbesondere im Frühstadium einer Keuchhustenerkrankung und bei milden oder atypischen Krankheitsbildern erweist sich die PCR im Vergleich zur Kultur als sensitivere und schnellere Methode zum Nachweis von Bordetella pertussis.      

Thrombotisch-thrombozytopenische Purpura mit akuter Niereninsuffizienz bei einem Knaben mit Lupus-ähnlicher Systemerkrankung

Zusammenfassung Ein 13⁹/₁₂ Jahre alter Knabe entwickelte 3 Monate nach Beginn einer Lupus-?hnlichen Systemerkrankung unter Prednisontherapie ein akutes Nierenversagen mit h?molytischer An?mie und Thrombozytopenie. Die Nierenbiopsie zeigte eine thrombotische Mikroangiopathie. Unter Behandlung mit Plasmaaustausch, immunsuppressiver Therapie und H?modialyse normalisierten sich die h?matologischen Befunde, und die Nierenfunktion besserte sich. Die Cyclophosphamidtherapie wurde über 2¹/₂ Jahre fortgesetzt. Drei Jahre nach dem akuten Nierenversagen ist der Junge unter antihypertensiver Behandlung symptomfrei und hat eine normale glomerul?re Filtrationsrate. Diskussion. Die Assoziation eines systemischen Lupus erythematodes mit einer thrombotisch-thrombozytopenischen Purpura ist in der Literatur bisher nur selten und fast nur bei Erwachsenen beschrieben worden; sie hat eine hohe Mortalit?t. Der dargestellte Fall demonstriert den Erfolg einer frühzeitigen aggressiven Therapie.      

Aicardi-Goutières-Syndrom

Aicardi-Goutières syndrome (AGS) is a rare severe progressive encephalopathy which starts in early infancy. Hallmarks include calcifications of the basal ganglia, chronic lymphopleocytosis and abnormal interferon-α elevations in cerebrospinal fluid (CSF). The disorder mimicks intrauterine infections, but is a genetic disorder, mostly inherited by an autosomal recessive trait with a variable genetic and clinical spectrum. In clinical and laboratory presentation, there is an overlap to infantile systemic lupus erythematosus. Four different genes have been identified to date, others are assumed. They code for nucleases, which are involved in cellular repair mechanisms by cleaving RNA-DNA particles. Mutations cause, via enzyme inactivation, a chronic overstimulation of the innate immune system, leading to increased CSF interferon-α production. Prenatal diagnosis is available for known mutations.     

Diagnose und Behandlung des Ornithintranscarbamylase(OTC)-Mangels

Zusammenfassung Der Ornithintranscarbamylasemangel ist der h?ufigste angeborene Defekt des Harnstoffzyklus. Er wird im Gegensatz zu anderen akut verlaufenden angeborenen Stoffwechselerkrankungen X-chromosomal vererbt. Betroffene Jungen zeigen in der Regel schon in den ersten Lebenstagen infolge einer sich ausbildenden Hyperammon?mie schwere klinische Symptome wie zunehmende Lethargie, Erbrechen, Krampfanf?lle und Koma. Aber auch übertr?gerinnen k?nnen lebensbedrohliche Hyperammon?mien sowie progrediente chronische Enzephalopathiesyndrome entwickeln. Die Notfallbehandlung bei der Erstmanifestation inklusive aller diagnostischer/differentialdiagnostischer Ma?nahmen sowie die Langzeittherapie und Stoffwechselüberwachung dieser Patienten sind in der vorliegenden Arbeit beschrieben. Zus?tzlich wird über den Allopurinoltest berichtet, der zur Erfassung Heterozygoter verwendet wird.      

Kurzer Abriss der Epilepsien und epileptischen Syndrome des Kindesalters

The classification of seizures, epilepsies and epileptic syndromes is complicated and unsatisfying. It is unknown by which mechanisms a person with a disposition to cerebral seizures will develop an epilepsy. The fact that the same child with a stable, non-progressive lesion, for example a cortical dysplasia, will show at different ages totally different types of seizures, with the consequence to classify this very same child to aged dependent different epileptic syndromes did not lead to a better understanding of the ILAE classification system. It was the wish of the editors to give a short cut paper on the whole spectrum of childhood epilepsies and epileptic syndromes to the reader.     

Mikroben überlisten die Immunantwort mit Tricks und Täuschung

Zusammenfassung Die Vermutung, Infekterreger und Abwehr bef?nden sich bei normalen Individuen in sicherem Gleichgewicht, ist eine Illusion. Auch beim Immunkompetenten unterlaufen alle Infekterreger die Immunabwehr. Zum Teil kommen sie „verkleidet durch die Hintertür“: Kapsel, Biofilm, Antigen-Drift und -Shift sind wirksame T?uschungsman?ver. Oder sie „speien Gift und Galle“, um die Torhüter zu l?hmen; d. h. sie rufen eine chemische Blockade der intrazellul?ren Abt?tung, der Antigenverarbeitung und der Antigenpr?sentation hervor. Zu unserem Glück ist es für einen Parasiten meist nicht von Vorteil, seinen Wirt zu t?ten. Bei Parasiten jedoch, die letzlich zum Tod des Wirts führen, müssen wir ?rzte eingreifen und die Infektion verhindern bzw. den Parasiten bek?mpfen.