Pediatric radiological diagnostic procedures in cases of suspected child abuse.

Advanced and specialized radiological diagnostic procedures are essential in cases of clinically diagnosed injuries to the head, thorax, abdomen or extremities of a child, especially if there is no case history or if the reporting of an inadequate trauma suggests battered child syndrome. In particular, these diagnostic procedures should aim at detecting lesions of the central nervous system (CNS), so that the treatment can be immediately initiated. If the diagnostic imaging reveals findings typically associated with child abuse, accurate documentation constituting evidence, which will stand up in court, is required to prevent any further endangerment of the child's welfare.

[Pediatric radiological diagnostics in suspected child abuse]. / Kinderradiologische Diagnostik bei Verdacht auf Kindesmisshandlung.

Advanced and specialized radiological diagnostics are essential in the case of clinical suspicion of pediatric injuries to the head, thorax, abdomen, and extremities when there is no case history or when "battered child syndrome" is assumed on the basis of inadequate trauma. In particular, the aim of this sophisticated diagnostic procedure is the detection of lesions of the central nervous system (CNS) in order to initiate prompt medical treatment. If diagnostic imaging shows typical findings of child abuse, accurate documented evidence of the diagnostic results is required to prevent further endangerment of the child's welfare.

Preliminary report on elective preterm delivery at 34 weeks and primary abdominal closure for the management of gastroschisis.

INTRODUCTION: We aimed to critically evaluate elective preterm delivery and immediate abdominal wall closure and other techniques for the management of gastroschisis, hypothesizing that the advantages of an elective preterm delivery outweigh possible complications related to prematurity at birth. PATIENTS AND METHODS: 13 gastroschisis patients were enrolled in the elective preterm delivery program (Group 1) since 1999. Patients were delivered by cesarean section in the 34th gestational week, with immediate primary closure of the defect. Data regarding parameters at and after birth were compared with a historical control group of 10 patients conventionally managed for gastroschisis in a similar period (1994 - 1999) (Group 2). The primary endpoints of this study were the initiation of oral feeding and the length of hospital stay. RESULTS: There was a significantly faster initiation of oral feeding (p = 0.0012) and a shorter hospital stay (p = 0.0160) in Group 1. The postoperative outcome was excellent in all patients. Acute and late complications were fewer and less severe in Group 1 and none were related to prematurity. CONCLUSIONS: Elective preterm delivery appears to be an effective method for the management of gastroschisis, and a method whose advantages thus far have outweighed the possible complications due to prematurity.

[Basic diagnostic approach to suspected allergic asthma]. / Näichtliche Atemnot und typische Pfeifkonzerte. Basisdiagnostik bei Verdacht auf allergisches Asthma.

Anamnestic factors of importance are, in particular, acute attacks of shortness of breath, dry cough and symptoms of concomitant rhinoconjunctivitis. Wheezing and other rhonchi are the typical findings on auscultation. Of decisive importance for the diagnosis is spirometry showing reduced values for the one-second/forced vital capacity and the Tiffeneau index. After administration of a beta sympathomimetic, an obstruction can be at least partially reversed. An absent primary obstruction should be inducible by unspecific provocation. On the basis of symptoms and lung function, bronchial asthma is divided into four stages. For establishing the allergic genesis of the illness, additional anamnestic data, in particular concerning the nature of possible allergens, are needed before sensitization is proven by the prick test and the detection of specific IgE antibodies. An equivocal situation can be clarified with the aid of specific nasal--more rarely also bronchial--provocation.

[Accidents in children and adolescents. Analysis of the accident event]. / Unfälle von Kindern und Jugendlichen. Analyse des Unfallgeschehens.

BACKGROUND: Accidents are the most frequent cause of death in children and adolescents. The aim of this study was to determine factors, which affect injury severity and to compare the absolute number of accidents with exposition data. PATIENT AND METHODS: From 1 January 1999 to 31 December 2001 a school survey of 2325 pupils was carried out. The 3645 accidents sustained by children and adolescents aged between 6 and 17 years treated at the surgical emergency department of the University Hospital Dresden were analyzed. RESULTS: Of the 3645 patients, 620 (17%) were admitted to hospital and 3025 (83%) were treated as outpatients. The most frequent diagnosis of the hospitalized patients was commotio cerebri; 83% of 36 severely injured patients had a head injury. 55.5% (297 of 536) of children between 6 and 9 years were using a protective helmet. In contrast only 14% of adolescents carried a protective helmet. None of 50 injured bicycle drivers with helmet had an AIS for head injuries >2. Twenty-for of 233 injured bicycle drivers without helmet had an AIS for head injuries >2. CONCLUSIONS: Head injuries are the main cause of hospitalization in traumatized children and adolescents. However, the use of a protective helmet becomes significantly less frequent with increasing age.

[Management of multiple endocrine neoplasia syndrome type 2 families in association with rare germline mutations of the RET proto-oncogene]. / Management von Familien mit einem multiplen endokrinen Neoplasie-Syndrom Typ 2 in Assoziation mit seltenen Keimbahnmutationen des RET-Protoonkogens.

INTRODUCTION: Heredity of MEN2 syndromes is caused by a heterozygous germline mutation in the RET proto-oncogene. This study describes families with rare noncysteine codon 790/791 mutations and discusses the genotype-phenotype correlation plus the therapeutic options. PATIENTS AND METHODS: Forty-five patients with a putative sporadic MTC were screened for RET germline mutations by direct DNA sequencing. Family members of identified index cases underwent genetic analysis. Gene carriers were examined clinically and biochemicaly and underwent prophylactic thyroidectomy. RESULTS: Five index patients were identified. In the kindreds three L790F and one Y791F carriers were detected. The thyroid gland histology of L790F carriers revealed MTC in 2 patients and C-cell hyperplasia in 2 additional patients. The Y791F carrier had a normal histology. CONCLUSIONS: Codon 790/791 mutations had diverse penetrance: prophylactic thyreoidectomy in children is a justifiable approach for codon 790 mutation carriers, but should depend on the clinical course of codon 791 carriers.

Within-gene interaction between c.135 G/A genotypes and RET proto-oncogene germline mutations in HSCR families.

Hirschsprung disease (HSCR) is considered a model for a complex inheritance disorder. Several genes, including the major HSCR-susceptibility RET proto-oncogene, play an aetiological role in the development of HSCR. Genetic linkage analysis in familial HSCR with both long- and short-segment phenotypes has demonstrated a tight linkage to the RET locus, while the phenotype within a HSCR family is characterised by an incomplete penetrance or a variable extension of the aganglionosis. Therefore, additional genetic alterations of RET are postulated in the aetiology or modification of the HSCR phenotype. In this study, the coding region of all 21 exons of the RET proto-oncogene, including the flanking intronic sequences, were investigated by direct DNA sequencing in a HSCR population. We genotyped the c.135 G/A polymorphism and resolved haplotypes comprising the mutation locus and the c.135 G/A polymorphism. Twenty different mutations were detected in 18 of 76 HSCR patients. In ten families the mutations were inherited from the parents, while only four patients had a positive family history for the disease. Moreover, in all ten families an incomplete penetrance of the HSCR phenotype was observed. We have investigated the effect of the non-mutated wild-type allele as well as the c.135 G/A polymorphism on the phenotype within the HSCR families. Our findings support the notion that both RET alleles are involved in the pathogenesis of a subgroup of HSCR patients in a dose-dependent fashion. Additionally, we have shown a modifying effect of the c.135 G/A polymorphism on the HSCR phenotype within HSCR families.

Rifampin-impregnated silicone catheters: a potential tool for prevention and treatment of CSF shunt infections.

BACKGROUND: Infection continues to be one of the major complications of cerebro-spinal fluid shunting procedures. Recent insights in the pathophysiological mechanism of these foreign body infections have elucidated the difficulty of achieving successful treatment without device removal. The development of a rifampin-impregnated silicone catheter yielded excellent results in infection prevention and treatment in vitro as well as in an animal model. PATIENTS AND METHODS: Here, we describe the application of this device in two patients with a complicated course of shunt infection. RESULTS: In one patient the rifampin-impregnated shunt system was implanted after external drainage to prevent further infection. The shunt infection of the second patient was treated by replacement of the infected shunt system with a rifampin-impregnated device. Both patients recovered immediately without any signs of adverse effects and the two shunt systems have now been working properly for more than 36 and 21 months, respectively. CONCLUSION: These results suggest that rifampin-impregnated silicone catheters could become a valuable tool in the treatment and prevention of shunt infections.

[Definition of shock types]. / Definitionen der Schockformen.

Definitions of shock types. Hypovolaemic shock is a state of insufficient perfusion of vital organs with consecutive imbalance of oxygen supply and demand due to an intravascular volume deficiency with critically impaired cardiac preload. Subtypes are haemorrhagic shock, hypovolaemic shock in the narrow sense, traumatic-haemorrhagic shock and traumatic-hypovolaemic shock. Cardiac shock is caused by a primary critical cardiac pump failure with consecutive inadequate oxygen supply of the organism. Anaphylactic shock is an acute failure of blood volume distribution (distributive shock) and caused by IgE-dependent, type-I-allergic, classical hypersensibility, or a physically, chemically, or osmotically induced IgE-independent anaphylactoid hypersensibility. The septic shock is a sepsis-induced distribution failure of the circulating blood volume in the sense of a distributive shock. The neurogenic shock is a distributive shock induced by generalized and extensive vasodilatation with consecutive hypovolaemia due to an imbalance of sympathetic and parasympathetic regulation of vascular smooth muscles.

Developing a European Internet and kiosk-based health information system.

A consortium of partner organisations (universities, health care organisations and information technology companies) from Northern Ireland, Germany, Portugal and Italy have collaborated to develop a multi-lingual, multi-media Internet and kiosk-based health information system in cardiology and skin cancer. The project, CATCH II (Citizens Advisory System based on Telematics for Communication and Health), has been funded by the European Commission under the Fourth Framework Research and Development TELEMATICS Applications Program (TAP), Health Care Sector. In this paper we provide an overview of the system and the methodological approach adopted. Key characteristics with respect to the technical architecture and flexible customisation of different web and kiosk-based versions will be presented. In particular, the development of dedicated software for the procurement, structuring and management of the information knowledge-base is illustrated. Some of the most interesting findings from a cross-national study of health information needs on the internet are presented along with information on the validation of the system by the general public, content providers and health care authorities.

[The reconstruction of rectal atresia. Diagnostics, therapy and prognosis of anorectal malformations]. / Rekonstruktionsverfahren bei Rektumatresien - Diagnostik, Therapie und Prognose von anorektalen Agenesien -

The reconstruction of rectal atresia. Diagnostics, therapy and prognosis of anorectal malformations. The reconstructive operations of anorectal malformations are aimed on the establishment of anatomical correct position of the anus and the elimination of existing urethral or cutaneous fistulas resulting in a regular fecal continence. The favourite surgical method of operation is the posterior sagittal approach by Pena, in high-type anorectal malformations the endorectal pull-through by Rehbein can also be recommended. The assessment of fecal continence of the low-type forms is satisfactory good compared to high-type. It mainly depends on the localisation of these defects and on the experience of the surgeon. With the exception of not accurate placement of the neorectum respectively neoanus secondary surgical intervention to improve the fecal continence should be used only after applying all conservative methods, like perineal gymnastics, electrostimulation and biofeedback training.

Effect of PEEP and suction via chest drain on functional residual capacity and lung compliance after surgical repair of congenital diaphragmatic hernia: preliminary observations in 5 patients.

BACKGROUND/PURPOSE: Congenital diaphragmatic hernia (CDH) is associated with pulmonary hypoplasia that limits survival. The authors' knowledge on lung mechanics and lung volumes in these patients with hypoplastic lungs is still limited. Therefore, the authors performed measurements of functional residual capacity (FRC), compliance of the respiratory system (CRS), and tidal volume in 5 full-term infants (gestational age, 38 to 40 weeks; birth weight, 2,800 to 3,530 g) before and after surgical repair of neonatal CDH. METHODS: The authors studied the influence of different levels of positive end-expiratory pressure (PEEP) and suction via inserted ipsilateral chest tube connected to a water seal on lung volume and lung mechanics. A computerized tracer gas (SF6) washout method was used for serial measurements of FRC. Compliance of the respiratory system was determined according to insufflatory method. RESULTS: The authors found a preoperative compliance between 1.5 and 3.9 mL/kPa/kg and a preoperative FRC between 9.1 and 12.9 mL/kg indicating severe hypoplasia of the lungs in all patients. Immediately after surgical repair of CDH, compliance decreased to 85% (78% to 91%) of preoperative value, and FRC increased to 132% (110% to 150%) of preoperative value under mechanical ventilation while at 4 cm of water of PEEP and at -10 cm of water of suction via chest drain with the need of high fraction of inspired oxygen. After reduction of PEEP from 4 to 2 or 1 cm of water and lowering suction from -10 cm of water to -2 or 0 cm of water FRC decreased to 103% (80% to 122%) of preoperative value and compliance, and tidal volume improved to 135% (110% to 147%) of preoperative value resulting in increased alveolar ventilation, correction of acidosis and improvement in oxygenation. During the first days after surgery inadequate high PEEP or strong suction via chest tube drainage resulted in increase in FRC paralleled by decrease in compliance indicating overdistension of these hypoplastic lungs. CONCLUSIONS: The data show that overdistension of hypoplastic lungs in infants with CDH can be detected and excluded by repeated measurements of FRC and compliance in these critical ill infants. These data might help setting appropriate ventilator parameters, adequate suction via chest drain, and thereby improve gas exchange and outcome.

[Selenium administration in children with SIRS]. / Selensubstitution bei Kindern mit SIRS.

PATIENTS AND METHOD: At the Clinic for Paediatric Surgery of the University of Dresden, in a time period ranging from 5/1994 to 12/1996, all patients aged between 1 and 16 years with severe inflammatory surgical diseases or extended scalded skin, were given an adjuvant selenium substitution. As control group, all patients with the same diagnosis and age treated during the months 1/1997 to 12/1998, did not receive this adjuvant selenium substitution. All these patients fulfilled the criteria of "Systemic Inflammatory Response Syndrome" (SIRS). The selenium-therapy group consisted of 34 patients and the control group without substitution consisted of 31 patients. The following laboratory parameters were measured on the 1st, 2nd, 3rd, 6th and last treatment day: white blood cell count, interleukin 6, C-reactive protein, fibrinogen, malondialdehyde, activity of glutathione peroxidase in plasma and level of selenium in plasma and whole blood. RESULTS: The initially high interleukin 6 rates declined significantly in both groups from the 2nd day on. The acute phase proteins, i.e. the C-reactive protein and fibrinogen, normalized in both groups after the 3rd day of treatment. The initial low rates of selenium in plasma and blood gained more rapidly a normal level in the therapy group than in the control group. On the 1st day of therapy the glutathione peroxidase activity in plasma was in both groups at the inferior limit of norm range and remained at this level in the control group for the whole observation period. In the selenium-substitution group on the contrary, these initial low values raised to the double as an expression of an elevated cell membrane protection. The initial significant elevated malondialdehyde rates in both groups, expressing a raised lipidperoxidation, fell down to a normal level in the selenium-substitution group, whereas they remained at their initial high level in the control group during the whole observation period. CONCLUSION: The substitution of selenium in children with SIRS is a supportive therapy.

[Recurrent hemorrhage from a duodenal ulcer in a 5-year-old healthy boy. A case report]. / Rezidivierende Blutung aus einem Ulcus duodeni bei einem 5jährigen gesunden Knaben. Ein Fallbericht.

Peptic ulcers in infants are rare. We report a 5-year-old boy who was admitted with recurrent bleeding from a huge duodenal ulcer. There were no concomitant disease and no preceding symptoms discovered. The only clinical symptom was bloody stool of light red color what led to diagnostic problems. Upper gastrointestinal bleeding was not considered initially. Short transit time through the gut may suggest a bleeding source within the lower intestine. Gastroscopy was performed delayed. Injection therapy of the ulcer once using fibrin sealant was followed by definite cessation of bleeding. Helicobacter pylori was not found. Hormone producing tumors could be excluded. There was a psycho-social situation of stress recognizable for the infant. The pathogenic mechanism of peptic ulceration due to psycho-social stress is unknown and somewhat doubtful at all. Peptic ulcer disease in infants and children should more often be considered when dealing with diffuse abdominal pain or with gastrointestinal bleeding.

[Popliteal artery entrapment syndrome. Case report of an 11-year-old boy]. / Popliteal-artery-entrapment-Syndrom. Kasuistik eines 11jährigen Knaben.

The popliteal entrapment syndrome arises due to a compression of the popliteal artery by tendomuscular structures often combined with an anomal position of the artery. Mostly young men are complaining of this disease. We report about an eleven-year old boy, who had an interview with us because of acute ischaemic symptoms in the left shank. We ensured a popliteal entrapment syndrome type I by Kogel. By a dorsal approach to the fossa poplitea we performed the myotomy and the restoration of the artery into the normal position. Eight month postoperative the boy is without any complaint. In doppler-scan we record an normal arterial flow.

[Does MRI compared with ultrasound examination add therapy-relevant knowledge in anorectal abnormalities?]. / Bringt die MRT-gegenüber der sonographischen Untersuchungstechnik einen therapierelevanten Erkenntniszuwachs bei anorektalen Fehlbildungen?

The topographical situation of the blind end of the recto-anal atresia to the musculus levator ani is the main question to ask when deciding on any operative procedure and for the prognosis of continence. In neonates, the use of preoperative perineal ultrasound is sufficient rather than magnetic resonance imaging in establishing the surgical way. In older incontinent children after pullthrough procedure, magnetic resonance imaging has benefits over endorectal ultrasound, especially in presenting muscular and cicatricial relationships.

[Selenium administration in severe inflammatory surgical diseases and burns in childhood]. / Selensubstitution bei schweren entzündlichen chirurgischen Krankheitsbildern sowie Verbrennungen und Verbrühungen im Kindesalter.

PATIENTS AND METHOD: Substitution of selenium was performed in the University Clinic of Paediatric Surgery in Dresden in the time from 1994 to 1996 in 34 children aged 1 to 16 years with severe inflammatory surgical diseases as well a s widespread burns. Seven further patients have been examined within this time who have not received substitution of selenium as preliminary comparison group. All these patients fulfilled the criteria of "Systemic Inflammatory Response Syndrome" (SIRS). The following paraclinical parameters were examined: white cell count, interleukin 6, C-reactive protein, fibrinogen, malondialdehyde, activity of glutathione peroxidase in plasma and level of selenium in plasma and whole blood. RESULTS: Patients with initially low level of selenium who received substitution of selenium reached normal ranges more quickly than patients without substitution. Originally partly elevated values of malondialdehyde as sign of increased peroxidation of lipids were normalized under substitution of selenium. Initially low activity of selenium level in plasma showed a clear increase under substitution of selenium as sign of increased protection of the cell membrane. CONCLUSION: The substitution of selenium in children with SIRS is a supportive therapy.

Structured data collection and knowledge-based user guidance for abdominal ultrasound reporting.

This paper describes a system for structured data collection and report generation in abdominal ultrasonography. The system is based on a controlled vocabulary and hierarchies of concepts; it uses a graphical user interface. More than 17,000 reports have been generated by 43 physicians using this system, which is integrated into a departmental information system. Evaluations have shown that it is a well accepted tool for the fast generation of reports of comparatively high quality. The functionality is enhanced by two additional components: a hybrid knowledge-based module for "intelligent" user guidance and an interactive tutoring system to illustrate the terminology.