[Congenital Intermittent Third-degree Atrioventricular Block Associated with Retinoid Exposure in Pregnancy]. / Konnataler intermittierender AV-Block III° nach Retinoideinnahme in der Schwangerschaft.

BACKGROUND: The retinoid acitretin, which has been approved as an effective therapy for severe keratinization disorders, is highly teratogenic, and exposure in the first trimester of pregnancy is associated with the risk of miscarriage and various malformations, including congenital heart defects. Cardiac conduction system disorders have not been described so far. CASE REPORT: A 24-year-old woman was treated with acitretin for dyskeratosis follicularis until pregnancy was diagnosed at 12 weeks of gestation. The female infant was born after 35 weeks gestation by cesarean section because of intermittent fetal bradycardia. The baby was vigorous at birth (Apgar 9, 10, 10 at 1, 5 and 10 min) but displayed intermittent third-degree atrioventricular block. A search for maternal autoantibodies and viral infections gave negative findings. CONCLUSION: The spectrum of disorders caused by intrauterine retinoid exposure appears to include atrioventricular conduction failure.

Major contributors to hospital mortality in very-low-birth-weight infants: data of the birth year 2010 cohort of the German Neonatal Network.

The German Neonatal Network (GNN) is a prospective cohort study with the focus on long term development of very-low-birth-weight infants. It was the aim of this study to determine detailed information on causes of mortality in the GNN birth cohort 2010.Major contributors to hospital mortality were recorded by the attending neonatologists for the cohort of very-low-birth-weight (VLBW) infants born in centres of the German Neonatal Network (GNN) in 2010. The data quality was approved by on-site monitoring.2 221 VLBW infants were born in GNN centres in 2010, and death occurred in 221 infants. Male infants carried a higher risk than females (58.8% males among non-survivors vs. 51.7% among survivors, p=0.047). In 11 infants, the major contributor to death was not determined by the attending neonatologist. In 25 infants born at the limit of viability, comfort palliative care was primarily initiated and 14 infants had lethal malformations. The majority of non-survivors suffered from inflammatory diseases including sepsis- or necrotizing enterocolitis (NEC)-associated death (n=56). Respiratory pathology was a major contributor to death in 65 infants including 11 infants who died from pulmonary haemorrhage.Potentially preventable complications of preterm birth such as sepsis, NEC and pulmonary haemorrhage predominate the major contributors to mortality in the GNN 2010 cohort. In order to decrease the rate of these associated deaths, future trials should focus on prophylaxis and therapy optimization strategies for these outcomes.

A prospective study with ten years follow-up of two-hundred patients with proximal femoral fracture.

BACKGROUND: The proximal femoral fracture is one of the most common injuries in the elderly. Nevertheless, no results beyond the second year post surgery have been reported in the literature. Therefore, the purpose of this study was to evaluate any revision and mortality within 10 years follow-up as well as the walking ability of still alive patients. METHODS: A total of 200 consecutive patients were included. A prospective database was first used to collect the demographic data. Exactly ten years after the surgery, a final evaluation was conducted by telephone for every patient. Any revision, any contralateral as well as other fractures and the date of death were recorded. For all patients who were still alive, the mobility score according to Parker was also surveyed. RESULTS: The average age was 79.0 years (SD: 12.5); women were affected at higher numbers (73.5%). The total surgical revision rate was 17.5% (35/200), due in particular to hematoma (9×) or infection (7×). A surgical revision later than two years was only needed in three patients (1.5%). The risk of another fracture caused by a fall was 19% (38/200), most often a contralateral femoral fracture (22/200; 11%) that happened on average 51.9 months (1-97) after the initial surgery. The risk of a contralateral femoral fracture was 15.4% (22/143) in patients who survived the first year post surgery. The postoperative mortality was 1, 2, 5 and 10 years or 23.5%, 32.5%, 55% as well as 81.5%, respectively. An average Parker's mobility score of 6.3 points (0-9) was determined for the 37 patients (18.5%) who were still alive at the time of the follow-up. CONCLUSION: The long-term study showed that revision surgery was only required in 3/200 patients (1.5%) beyond the second year of that surgery. On the other hand, more than half of all patients had already passed away five years after the initial surgery. The exact incidence of a contralateral femoral fracture was 11.9%, climbing to 15.4% if the patient survived at least one year. Nearly every fifth patient experienced another fall resulting in a severe fracture requiring treatment during the long-term course.

Direct bladder hernia after indirect hernia repair in extremely low birth weight babies: two case reports and a review of the literature.

BACKGROUND: Inguinal hernia repair is the most common surgical procedure in babies. Despite a meticulous technique, relapses may occur. The occurrence of a direct bladder wall hernia in relapses has never before been reported in the literature. CASE PRESENTATION: Here, we report two cases of direct bladder herniation: a white baby boy born after 25 weeks of gestation and a white baby boy born after 26 weeks of gestation. Both of the formerly extremely low birth weight babies were affected after open bilateral hernia repair. Recurrent hernias developed on the right side, and direct bladder herniation was identified intraoperatively. In one case, laparoscopy was applied to identify a supravesical type of hernia. Immaturity and a difficult postnatal course might have contributed to hernia relapse in these cases. CONCLUSIONS: Misinterpretation of bladder herniation might have disastrous consequences. Laparoscopy is a helpful tool in comparable cases.

Isolated tracheoesophageal fistula versus esophageal atresia - Early morbidity and short-term outcome. A single institution series.

PURPOSE: We compared the postnatal course, morbidity and early results after repair for cases of isolated or "pure" TEF with those for cases of esophageal atresia (EA) with distal tracheoesophageal fistula (TEF). METHODS: Twenty-four consecutive infants were divided into two groups: isolated TEF [TEF group] (n = 5) and EA with distal TEF [EA group] (n = 19). RESULTS: A high rate of prematurity (29%) and major cardiac and other surgically-relevant malformations (0.8 vs. 0.7 per infant) was found in both groups. The median age at surgery was 8 days for the TEF group vs. 1 day for the EA group (p < 0.01). Most infants of both cohorts had stable acid-base and respiratory parameters at admission. Generally, tracheoscopy provided valuable information regarding the position of the TEF. Surgery for isolated TEF was performed via right cervicotomy in 4 cases and via thoracotomy in one. Postoperative thoracostomy tubes were inserted in 3 cases and one emergency gastrostomy was created for acute gastric overextension (exclusively in patients with EA). The duration of postoperative mechanical ventilation (49 vs. 113 h, p = 0.045) and the median length of stay in the pediatric surgery unit (10 vs. 20.5 days, p = 0.003) were shorter for the isolated TEF group. Four EA patients experienced severe events. Total mortality was 8% (0 out of 5 with TEF vs. 2 out of 19 with EA). CONCLUSION: Developmental delay and a high rate of morbidity were found in both groups. More complex surgery increased perioperative morbidity in cases of EA. With early recognition of isolated TEF, a less complicated course can be expected in comparison with esophageal atresia.

[Reduction of the amputation rate in bone and soft tissue sarcoma by interdisciplinary cooperation]. / Senkung der Amputationsrate bei Knochen- und Weichgewebssarkomen durch interdisziplinäres Vorgehen.

On the basis of three patients with bone and soft tissue sarcoma,we would like to illustrate the necessity of a well working inter-disciplinary cooperation of radiologists, internal oncologists, radiation therapists, orthopaedic surgeons and plastic surgeons. Functional extremity preservation in sarcoma patients can be achieved by a good interdisciplinary management without im-pairing the total prognosis of the patients. Patients with sarcoma should be treated in centres in which all specialised divisions are experienced and well trained in the treatment of tumour patients. Only then can a promising approach be achieved.

[Histological and immunohistochemical investigations with capsular contracture after breast augmentation with smooth silicone gel implants]. / Histologische und immunhistochemische Untersuchungen bei Kapselkontraktur nach glatten Brustimplantaten.

INTRODUCTION: A prospective study was performed to analyse the cellular and molecular composition of fibrous capsules around silicone breast implants. The necessity of an exact histological classification for comparing objectively the different findings of capsular contracture is shown. PATIENTS AND METHODS: The prospective study (investigation time 1/2003 to 6/2005) included 24 female patients (average age: 40+/-12 years) with contracture after bilateral cosmetic breast augmentation with smooth silicone gel implants (Mentor). In each patient the baker score was determined preoperatively. Samples of capsular tissue from all patients were evaluated histologically and immunohistochemically and classified according to the histological classification introduced by Wilflingseder and co-workers. RESULTS: All capsules showed the same basic histological structure with a three-layer composition. For the correlation analysis we had to exclude one patient with repeated implant change. There was no correlation between the patient's age, time of implantation, length of implant period, and capsular contracture. Greater amounts of silicone particles were associated with increased degrees of capsular contracture (Baker: r = 0.687, n = 23, p < or = 0.001; Wilflingseder: r = 0.784, n = 23, p < or = 0.001). High silicone amounts were associated with an increased local inflammation (r = 0.489, n = 23, p , 0.05). A moderate to severe local inflammation was found in 23 patients (95.8%). In summary, there was a positive correlation (r = 0.797, n = 23; p , or = 0.001) between the clinical classification (Baker score I to IV) and the histological classification (Wilflingseder score I to IV). CONCLUSIONS: We demonstrated in our study, in spite of using implants with high gel cohesiveness (fourth generation), the presence of vacuolated macrophages with microcystic structures containing silicone and silicone particles in the capsular tissue. Greater capsular thickness was associated with an increased number of silicone particles ans silicone-loaded macrophages in the peri-implant capsule. The histological classification introduced by Wilflingseder and co-workers takes into consideration this pathogenetic mechanism of inflammatory reaction which seems to be one of the major key factors in the development of capsular contracture.

[Training in sports medicine for medical students--Evaluation of a practice-oriented teaching concept]. / Sportmedizinische Ausbildung von Medizinstudenten. Evaluation eines praxisbezogenen Lehrkonzeptes.

INTRODUCTION: Education in Sports Medicine is offered heterogeneously in German medical schools. Efficacy and acceptance among medical students are unknown. The self-image of sports has changed from a traditional, competition-oriented way to adventure, entertainment, wellness and health. Therefore changes of our curricula to a practical oriented way of teaching are required. In this regard a new curriculum was developed. The main focus, besides an interdisciplinary class in sports medicine was to offer practical education in sports to the students. METHODS: Efficacy and acceptance of the class were evaluated using a questionnaire. 90 medical students (58 male, 32 female) were included into the study . RESULTS: The interdisciplinary topics of the class were graded positively. Most of the presentations were regarded as very useful for the future practice. Empathy and commitment of the professor were keystones to good scores. The "fun-factor" was the key for the success of the practical assignment. Practical training was regarded as fundamental for a sports medicine class. DISCUSSION: Evaluation of the new concept as well as a discussion of the current practice to teach sports medicine at medical school confirm the need of a more practical oriented education, in order to meet the requirements of sports medicine in a time with changing self image of sports.

[Modified Pirogoff's amputation]. / Modifizierte Pirogoff-Amputation.

OBJECTIVE: The goal of Pirogoff's amputation of the hindfoot is a weight-bearing stump with minimal loss of limb length and stable soft tissue coverage with preservation of the sensation of the sole of the heel. INDICATIONS: Non-reconstructable forefoot and midfoot after complex trauma, deep bony and soft tissue infection, infected Charcot foot, necrosis or gangrene due to vasculopathy, malignant tumors and deformities. CONTRAINDICATIONS: Possibility for reconstruction of the forefoot and midfoot, minor amputation, loss or irreversible destruction of the sole of the heel. SURGICAL TECHNIQUE: The incision runs from dorsal, 1-2 cm distal of the Chopart joint, to plantar, 5-6 cm distal of the Chopart joint for creation of an adequate plantar skin flap. Exarticulation of the foot from dorsal to plantar through the Chopart joint with preservation of the posteromedial neurovascular bundle. Enucleation of the talus. Minimal resection of the cuboidal and posterior facets of the calcaneus as well as the malleoli inclusive of the distal tibial joint surface. The calcaneus is brought under the tibia and a tibiocalcaneal arthrodesis is performed with two compression screws. POSTOPERATIVE MANAGEMENT: No weight bearing until stable scar formation, early mobilization in a walker. Interim prosthesis after 2-4 weeks and definitive prosthesis after 2-3 months. RESULTS: From January 2010 to December 2014 six patients were treated with a modified Pirogoff's amputation. Primary wound healing was achieved in four patients and in two patients wound healing was impaired. In one patient the wound was conservatively healed and the other patient needed below knee amputation. Early primary prosthetic treatment was possible in four patients. The tibiocalcaneal arthrodesis healed in all five remaining cases. All patients with a healed Pirogoff stump were able to walk for short distances in bare feet without the prosthesis.

[Amputation and exarticulation of the lesser toes]. / Amputation und Exartikulation der Kleinzehen.

OBJECTIVE: Amputations and exarticulations of the toes may be necessary due to several reasons. The goal is to remove necrosis or infection prior to its spread to the midfoot region. From a functional or cosmetic point of view, amputation/exarticulation of a single toe plays no major role. However, this can be different with exarticulation of several toes. INDICATIONS: Necrosis, trauma, infection, tumor, deformity. CONTRAINDICATIONS: Conditions where amputation/exarticulation of a toe is insufficient, e. g., in progressing peripheral arterial disease. SURGICAL TECHNIQUE: The toe can either be amputated through the distal phalanx or exarticulated in the metatarsophalangeal joint. POSTOPERATIVE MANAGEMENT: Orthopedic shoes or orthotic devices are rarely necessary when a single toe is amputated/exarticulated. However, concomitant deformities of the foot have to be thoroughly addressed. If more than one toe is amputated, silicone spacers may be necessary to prevent the remaining toes from deviating. RESULTS: Amputations and exarticulations of the toes are frequent and the procedure is technically simple. However, the complication rate is high due to typical indications making amputation necessary.

[Orthotic Supply after Complex Trauma of the Foot]. / Orthopädische Schuhversorgung nach komplexer Fußverletzung.

BACKGROUND: After complex trauma of the foot, patients frequently need orthotic care. There have been no systematic studies on the quality of care or patient satisfaction. Therefore the goal of this study was to evaluate patient satisfaction and the quality of orthotic care. PATIENT AND METHODS: In a prospective non-randomized, cross-sectional intervention study, the quality of care and patient satisfaction with orthotic devices were evaluated clinically and pedographically. This was followed by a lengthy discussion and recommendation to improve the insoles or shoes. These improvements were followed up by a written/telephone survey. 39 of the 50 recruited patients with complex trauma of the foot (78 %) were supplied with orthotic devices (insoles, modifications of the sole and orthopaedic shoes). RESULTS AND CONCLUSION: Most patients (76 %) were content with their orthotic care. However, shortcomings were noted in 20 patients. These were: insufficient arch support/stabilization, inadequate support of roll-off and unsatisfactory unloading of the forefoot. 23 % of the patients did not use their orthotic devices. Compliance increased after counseling. However, half of the patients had problems in obtaining reimbursement for the modifications recommended. In conclusion, orthotic treatment of patients after complex trauma of the foot is demanding. Modifications of the devices are frequently needed. Interprofessional collaboration plays an important role.

Structure of oligonucleotides with either a strand break or a bulged nucleotide.

We report NMR and molecular modelling studies on a DNA duplex structure which is composed of three oligonucleotides and mimics a strand break. Although it retains a B form conformation our model suggests that it is kinked at the strand break. In the same sequence with an extra bulged adenosine at the centre for the major species this residue is stacked in the helix and a kink is observed in the model.

Heterotopic auxiliary liver transplantation in a 3-year-old boy with acute liver failure and aplastic anemia.

BACKGROUND: Auxiliary liver transplantation offers an alternative method to conventional transplantation in acute liver failure. It is especially challenging for children because lifelong immunosuppression may be avoided. However, experience with this procedure is rare and there is controversy about whether to place the graft orthotopically or heterotopically. METHODS: We present the case of a 3-year-old boy with acute liver failure due to non-ABC hepatitis complicated by aplastic anemia who underwent auxiliary liver transplantation. Segments 2 and 3 of the graft were implanted heterotopically in the right lower abdomen. RESULTS: Good liver function was immediately restored. Aplastic anemia resolved 3 weeks after transplantation. Immunosuppressive therapy was discontinued after 14 months, and the graft was left to atrophy. Thirty-nine months after transplantation the boy is alive and well with normal liver function tests and normal blood cell counts. CONCLUSIONS: Heterotopic auxiliary liver transplantation allowed recovery of the native liver in a child with acute liver failure and aplastic anemia due to non-ABC hepatitis.

Fatal Salmonella enteritidis sepsis acquired prenatally in a premature infant.

BACKGROUND: Before the advent of antibiotic therapy, Salmonella typhi infection during pregnancy was associated with a high incidence of fetal and neonatal death. Little information is available about the risk to the fetus or the newborn of a pregnant woman infected by non-typhoid salmonella, and treatment recommendations do not exist. CASE: We report a case of transplacental infection of a fetus by non-typhoid salmonella in a woman with gastroenteritis. Salmonella enteritidis was cultured from stool of the pregnant woman, who had diarrhea and fever before cesarean was performed at 29 weeks' gestation. The premature girl died 4 hours after birth from septic shock. Salmonella enteritidis was cultured from blood cultures and swabs of the premature infant and from the placenta and uterus. CONCLUSION: This observation argues in favor of antibiotic treatment for non-typhoid salmonella infection in pregnancy because of the risk of transplacental infection of the fetus.

Acute cerebellitis with near-fatal cerebellar swelling and benign outcome under conservative treatment with high dose steroids.

Acute cerebellar swelling is an emergency because of brainstem compression as well as upward or downward cerebellar herniation. Few childhood cases are on record, with fatal outcome in three out of six. We report a girl with probable Epstein-Barr virus-associated cerebellar swelling who recovered completely with steroid treatment after a stormy course. Review of the literature showed that all three patients, including our own, who recovered fully, received high-dose steroids in contrast to none of the four patients who died or survived with sequelae. Neuroimaging and evoked potential studies are useful for early diagnosis and disease monitoring. We conclude that for the time being high-dose steroid treatment is advocated in patients with acute infectious or parainfectious cerebellar swelling.

Randomised clinical trial of two treatment regimens of natural surfactant preparations in neonatal respiratory distress syndrome.

AIMS: To compare treatment regimens of two widely used natural surfactant preparations Curosurf and Survanta in respiratory distress syndrome (RDS). METHODS: The effects of the two treatment regimens on gas exchange, ventilatory requirements, and 28 day outcome in infants with RDS were compared. Seventy five preterm infants (birth weight 700-1500 g) with RDS requiring artificial ventilation with an FIO2 of > or = 0.4, were randomly selected at 1-24 hours of age. One group received an initial dose of Curosurf (200 mg/kg); the other group Survanta (100 mg/kg). Patients who remained dependent on artificial ventilation with an FIO2 of > or = 0.3 received up to two additional doses of Curosurf (each of 100 mg/kg) after 12 and 24 hours or up to three additional doses of Survanta (each of 100 mg/kg) between six and 48 hours after the initial dose. RESULTS: There was a rapid improvement in oxygenation and ventilatory requirements were reduced in both groups. However, infants treated with Curosurf had a higher arterial:alveolar oxygen tension ratio and required a lower peak inspiratory pressure and mean airway pressure at several time points within 24 hours of randomisation (p < 0.05-0.001). The incidences of pneumothorax in the Curosurf and Survanta groups were 6% and 12.5%, respectively; the corresponding figures for grades 3-4 intracerebral haemorrhage were 3% and 12.5%, respectively. Mortality was 3% in the Curosurf group and 12.5% in the Survanta group. However, these differences did not reach significance. CONCLUSION: The Curosurf treatment regimen resulted in a more rapid improvement in oxygenation than Survanta and reduced ventilatory requirements up to 24 hours after start of treatment. This was associated with a trend towards reduced incidence of serious pulmonary and non-pulmonary complications.

Lactic acidosis from thiamine deficiency during parenteral nutrition in a two-year-old boy.

This is a case report of a two-year-old boy who was operated electively for a blind-loop syndrome of the proximal jejunum. Because of the appearance of chylous ascites, parenteral nutrition was carried out postoperatively. The boy developed a severe uncompensated acidosis and paralytic ileus. Relaparotomy on suspicion of ischemic bowel did not explain the cause of the acidosis and ileus. Postoperatively, the child's condition worsened, requiring intensive care. The drastically elevated lactate levels corroborated the eventually suspected diagnosis of a vitamin B1 deficiency syndrome. The administration of thiamine within two hours produced correction of the acidosis without further bicarbonate therapy. In 24 hours circulation was stabilized. Two months post-operatively the boy had completely recovered from the sequelae of his shock event.

Aplasia cutis congenita--etiological relationship to antiphospholipid syndrome?

A case of aplasia cutis congenita is reported. The mother suffered from antiphospholipid syndrome and a possible etiological relationship between this and aplasia cutis congenita is discussed.