Dismal prognosis of Pneumocystis jirovecii pneumonia in patients with multiple myeloma.

Patients with multiple myeloma (MM) are at high risk for infections, including opportunistic infections such as Pneumocystis jirovecii pneumonia (PJP). We conducted a retrospective analysis of patients with MM developing PJP over a 6-year period between January 2016 and December 2021 at the University Hospital of Würzburg by screening cases of microbiologically documented PJP. A total of 201 positive results for P. jirovecii in respiratory specimens were retrospectively retrieved through our microbiology database. Of these cases, 13 patients with MM fulfilled the definition of probable PJP according to EORTC fungal disease definitions. We observed two peaks in PJP incidence, one after stem cell transplantation during first-line treatment (n = 5) and the other in heavily pretreated patients with six or more prior lines of therapy (n = 6). There was high morbidity with nine (69%) patients admitted to the ICU, seven of whom (78%) required mechanical ventilation, and high mortality (62%, n = 8). Notably, only two of the 13 patients (15%) had received PJP prophylaxis. The main reason for discontinuation of prophylaxis with trimethoprim-sulfamethoxazole was grade IV neutropenia. The observed morbidity and mortality of PJP in MM patients are significant and even higher than reported for patients with other hematologic malignancies. According to most current guidelines, the use of prophylaxis would have been clearly recommended in no more than three (23%) of the 13 patients. This illustrates the need to critically reconsider the indications for PJP prophylaxis, which remain incompletely defined.

Ultrasound examination of paediatric testicles after laparoscopic laser dissection of internal testicular vessels in cryptorchism.

PURPOSE: To evaluate the growth of testicles, their sonographic structure and the recovery of testicular perfusion through collateral vessels after preliminary laparoscopic laser dissection (PLLD) of the internal spermatic artery in cryptorchism. MATERIALS AND METHODS: The postoperative position, the volume increase, the structural and vascular abnormalities and the incidence of epididymal abnormalities were evaluated with ultrasound in 32 children (mean age: 6.5 +/- 1.34 years) after PLLD. The intratesticular blood flow and the flow-in anastomoses from the deferential and cremasteric arteries were assessed using colour and power Doppler US (7.5 MHz transducer). RESULTS: All testicles were permanently positioned in the scrotum and showed growth after surgical treatment. None of the testes showed sonographic signs of atrophy. There were no clear differences in intratesticular perfusion compared to the non-operated testis. A collateral blood flow in the deferential artery was found in 44 cases by colour and/or power Doppler US. A perfusion to the lower testicular pole was detected in 29 cases by power Doppler. Intraoperatively, 12 epididymal inversions were eliminated so that an elongated epididymis was sonographically visualised in three testes. CONCLUSION: Ultrasound can be used for the postoperative control of the testicular structure and the volume increase as well as detection of the flow-in anastomoses. The collateral blood supply after PLLD was sufficient for subsequent growth of testicles in all cases.

[Minimal invasive pediatric surgery: development and progress by innovative technology]. / Minimalinvasive Kinderchirurgie: Entwicklung und Fortschritt durch innovative Technologie. Die ATCS-Klebung.

BACKGROUND: Minimally invasive pediatric surgery (MIPS) has a solid tradition in which technique and technology have made key contributions to an already broad range of indications. This surgical method still has a deficiency with regard to tissue management of large-area defects, however. Sealing techniques can further expand the range of application. METHOD: Technological evaluation provided outstanding data of fleece-bound collagen- and fibrinogen-based sealing systems (TachoComb) on biodegradability, adhesive strength and practicability. A relevant instrument was developed for MIS application and was introduced as the ATCS (AMISA-TachoComb-System). PATIENTS: From 1993-2000, ATCS sealing was carried out in the scope of thoracoscopy (105 procedures) and laparoscopy (53 procedures) and specifically for recurring pneumothorax, traumatic chylothorax and splenic trauma. RESULTS: Pneumothorax: 59 ACTS procedures in 49 patients (mean age: 11.4 yrs) with 6 reoperations (10.2%) and one recurrence (1.7%). The drainage dwell time was reduced (p < 0.05) using a conventional comparison (31.9 hours vs. 17 days) and further relevant parameters were also reduced. Chylothorax: 3 ATCS procedures in 3 patients (mean age: 6.3 years) with reduction in the drainage dwell time (p < 0.05) based on a conventional comparison (35 hours vs. 18 days). Splenic trauma: 17 ATCS procedures in 16 patients (mean age: 8.9 years) with one re-operation (5.9%) for associated liver trauma, organ conservation in each case and no significant drainage volumes. CONCLUSION: The ATCS is an innovative instrument for MIPS and ca be employed for efficient and socio-economic (e.g. DRGs) closure of large-area defects.

Carcinoid tumors of the appendix in children--epidemiology, clinical aspects and procedure.

INTRODUCTION: Incidental detection of a carcinoid tumor of the appendix after appendectomy is often accompanied by uncertainty about the further procedure. The tumor frequency in our own patient population, the further course in these children and a practicable follow-up protocol have to be determined. PATIENTS AND METHODS: All appendectomies performed between January 1, 1982, and December 31, 1996, were retrospectively evaluated with regard to carcinoids, monitoring the clinical course, and follow-up of the patients involved. RESULTS: A total of 4747 appendectomies were performed, 8 children had a histologically manifest carcinoid (0.169%). All patients were symptom-free in the further course, no metastases or signs of a carcinoid were documented during a mean follow-up period of 6.6 years. CONCLUSION: The youngest patient with a metastasizing carcinoid tumor of the appendix reported in the literature was 19 years old. Nevertheless, all younger patients should undergo regular follow-ups; this is done in our department by serum serotonin and chromogranin A determination.

Magnetic resonance imaging of osteoarthritis.

Considering the plethora of imaging protocols, joint-specific orientations, and potential artifacts, the design and interpretation of MR imaging examination is difficult. Like a physical examination, these considerations must be tailored to the specific tissue, joint, and clinical question under consideration. Shortcomings of MR imaging include the lack of consensus among radiologists with respect to which protocols best image articular joints. To date, few prospective studies have been undertaken to assess osteoarthritis by MR imaging. Diagnostic imaging is central to staging the severity of osteoarthritis and assessing the efficacy of therapeutic osteoarthritis. Plain film radiography is insensitive for identifying early changes of osteoarthritis. Tailored MR imaging, producing high spatial and or contrast resolution images, is proving to be an important tool in the early detection and surveillance of osteoarthritis progression. Future therapeutic techniques, such as chondrocyte transplantation, use of growth factors, or cartilage protease inhibitors requires high resolution and volumetric MR imaging studies for accurate identification of focal articular cartilage defects and generalized cartilage loss. Creation of cartilage models by three-dimensional MR image rendering may be helpful for preoperative planning of orthopedic surgical procedures in advanced cases of osteoarthritis. More work needs to be done in high resolution and volumetric MR imaging of articular cartilage. Given the availability of new disease-modifying treatments designed to prevent, delay the progression of, or reverse osteoarthritis, additional prospective MR imaging studies need to be undertaken to improve the reproducibility of MR imaging as a primary outcome measure in the evaluation of osteoarthritis. Interinstitutional standardization of specific MR imaging magnet strengths, surface coils, joint orientations, sequences used, scoring systems and quality assurance methodologies are needed to establish the reproducibility of MR imaging and interpretation for assessment of patients with osteoarthritis.

Pulmonary embolism and myocardial hypoxia during extracorporeal membrane oxygenation.

The treatment of a newborn with severe meconium aspiration by venoarterial extracorporeal membrane oxygenation (ECMO) was complicated by myocardial hypoxia with a marked decrease of myocardial contractility. The onset of the cardiac hypoxia was related to a pulmonary artery embolus. The origin of the embolus was a deep femoral vein thrombosis, caused by a central vein catheter, which was inserted 1 day before ECMO by venous cutdown. The possible pathophysiology of myocardial hypoxia in this patient is discussed, especially with regard to myocardial perfusion, supporting the hypothesis of coronary perfusion occuring with blood from the left ventricle and not from the arterial cannula in the aorta.

[Percutaneous removal of a brachiocephalic vein anchored venous catheter with wire loop and mini-laparoscopy scissors]. / Perkutane Entfernung eines in der Vena brachiocephalica festgenähten Venenkatheters mittels Drahtschlinge und Mini-Laparoskopieschere.

PURPOSE: The use of mini-laparoscopy scissors to remove a central venous catheter inadvertently fixed to the wall of the brachiocephalic vein is described. PATIENT AND METHODS: During a rethoracotomy in a 15-year-old female patient, a central venous catheter preoperatively introduced in the left subclavian vein was inadvertently trapped by a suture and fixed to the wall of the left brachiocephalic vein. The foreign body was removed by use of a transjugularly introduced venous sheath, a catheter wire snare, and mini-laparoscopy scissors. RESULTS: The fixed catheter was freed from the wall of the vein under fluoroscopic control with the help of a mini-laparoscopy scissors. Since the intravasal end of the catheter had already been grasped during mobilization with the wire snare it could be completely removed subsequently without any problem. CONCLUSIONS: The percutaneous, intravascular use of mini-laparoscopy instruments may be considered for foreign body removal in special cases.

Laser treatment of hemangiomas of the larynx and trachea.

BACKGROUND AND OBJECTIVE: Hemangiomas have a typical clinical course and may lead to life-threatening obstruction if the central respiratory tract is involved. STUDY DESIGN/PATIENTS AND METHODS: This was observed in 32 children over a period of 20 years. The radiation parameters and application procedure of Neodymium:Yttrium-Aluminum-Garnet-laser (Nd:YAG-laser) therapy were adjusted for the degree of obstruction and the type of disease. RESULTS: The success rate was 93.8%, of which a maximum of one application was sufficient in 24 children (75.0%). Subglottic cicatricial stenosis was considered a laser-related complication. CONCLUSION: Nd:YAG-laser treatment is a safe and effective therapeutic measure for eliminating respiratory tract obstruction caused by hemangiomas.

Vascular lesions in children: percutaneous MR imaging-guided interstitial Nd:YAG laser therapy--preliminary experience.

PURPOSE: To investigate the use of magnetic resonance (MR) imaging to guide interstitial laser therapy of deep hemangiomas and vascular malformations in children. MATERIALS AND METHODS: Sixteen children aged 3 months to 16 years with symptomatic vascular lesions underwent percutaneous laser treatment. MR imaging guidance of the laser applicator and online thermomonitoring with MR imaging were performed with a 0.2-T open MR system. Follow-up studies were performed 2 days and 6 weeks after thermotherapy. RESULTS: Interactive positioning of the laser applicator was possible in all patients. Online thermomonitoring was possible in 122 of 137 therapy spots (89%). There was a good correlation between volumes of coagulated tissue on intraprocedural T1-weighted images and volumes of coagulated tissue on follow-up T2-weighted images. At 6-week follow-up, MR imaging demonstrated a reduction in lesion size in 10 patients (mean reduction, 72%) and an increase in lesion size in two patients (mean increase, 134%). Clinical symptoms improved in 14 of 16 patients (88%). CONCLUSION: MR imaging-guided laser therapy appears to be a safe and potentially effective minimally invasive treatment for selected children with vascular lesions.

Qualitative and quantitative analysis of synapses in Hirschsprung's disease.

In Hirschsprung's disease (HD), certain intestinal nervous plexuses are absent. Sprouting nerve endings contain different amounts of synaptophysin (SY), a protein and main constituent of acetylcholinesterase (AChE) storage compartments. Due to the lack of specific markers for synapses, a qualitative analysis of nerve endings of intestinal segments affected by HD has not yet been undertaken. For this study, resected colorectal specimens from patients with HD (n = 8, mean age 2.1 years) were investigated in parallel for AChE, SY, and content of small synaptic vesicles by biochemical, immunohistochemical, and electronmicroscopic means. In the microdissected muscular layer, reduced SY (1.4 microgram/mg total protein, normal 24 +/- 0.3) was observed. Immunohistochemistry showed in affected tissues reduced numbers of SY-positive nerve fibers and nerve endings, which in turn were thickened and distorted, in both the muscle proper and the muscularis mucosae. Combining both morphologic and biochemical findings, in HD the number of cholinergic vesicles in the remaining nerve endings seems to be increased as measured by SY, a marker molecule specific for synaptic vesicles. Our data also suggest that nerve endings in HD may contain high concentrations of cholinergic vesicles, paralleling the known high amounts of acetylcholine and AChE found in intestinal segments of patients with HD.

Congenital cystic adenomatoid malformation of the lung and fetal hydrops--a case with favourable outcome.

We present a case of congenital cystic adenomatoid malformation of the lung (CCAM) diagnosed at 23 weeks of gestation with concomitant fetal hydrops. The sonographical picture of CCAM disappeared in the third trimester of pregnancy and fetal hydrops resolved under medication with digitalis to the mother. The neonate showed mild dyspnea; the prenatal diagnosis of CCAM was confirmed by chest X-ray and computed tomography. The affected lung segments were dissected at 5 days of age. The diagnosis of CCAM type III was confirmed histologically.

Multisystem organ failure and capillary leak syndrome in severe necrotizing enterocolitis of very low birth weight infants.

BACKGROUND: Classification systems for necrotizing enterocolitis (NEC) in preterm infants have been developed to define severity grades relevant for treatment and prognosis. Multisystem organ failure (MSOF) and capillary leak syndrome (CLS) also have prognostic value in these patients. The aim of this retrospective study was to investigate the incidence and predictive value of MSOF and CLS according to the classification criteria. METHODS: The records of 1,022 very low birth weight infants admitted from 1982 to 1996 were reviewed for diagnosis of NEC stage IIA or higher (classification of Walsh and Kliegman). Among those patients (n = 50) the incidence of MSOF and CLS was determined, separately for surgical or conservative treatment. RESULTS: Twelve patients were assigned to stage II, 22 to stage IIIa, and 16 to stage IIIb; 31 infants underwent operation. Mortality rate was not influenced by the grade. In eight patients only gastrointestinal symptoms were found, whereas in 23 patients, up to three organ systems and in 19 patients, four or more organ systems were affected. Mortality depended on the number of involved organ systems. CLS occurred postoperatively in 10 of the 31 infants; eight of them died. CONCLUSION: The prognostic values of MSOF and CLS are higher than that of classification criteria in NEC of VLBW infants.

[Laparoscopy in the newborn infant--indications and procedure]. / Laparoskopie des Neugeborenen--Indikation und Durchführung.

UNLABELLED: The indications and technical features of minimal invasive surgery, which is of particular interest in neonates, are being discussed. Thirty-three laparoscopies had been performed as of 1997. The youngest child was 2 days old; the smallest one weighted 2150 g. The most frequent indications were clarification of cholestasis, cystic masses, pyloromyotomies and sigmoid resections. Three conversions were necessary (two choledochal cysts, splenic torsion). Complications were one postoperative incisional hernia and two rectal stenoses after sigmoid resection. CONCLUSION: Laparoscopy can even be performed in newborns and meets the requirements of minimally invasive surgery.

Magnetic resonance imaging: controlled interstitial laser therapy in children with vascular malformations.

BACKGROUND AND OBJECTIVE: Interstitial laser therapy (ILT) is a minimally invasive treatment method for congenital vascular malformations (CVM). This study was intended to show whether or not open magnetic resonance imaging (MRI) offers a means of on-line thermometry and procedure control. STUDY DESIGN/PATIENTS AND METHODS: ILT using a bare fiber and an Nd:YAG laser was applied in 20 children with CVM under open MR control. RESULTS: With the open MR systems, needle placement and advancement was excellent in all cases. On-line thermometry was possible in 90% of the therapy spots. The 6 week MR follow-up revealed a 76% reduction in tumor size in 14 patients. Clinical symptoms improved in 85% of the patients. CONCLUSION: MR-guided ILT could become a safe method to treat selected types or selected regions of CVM.

[Acute appendicitis in the child]. / Die akute Appendizitis beim Kind.

The appendicitis is the commonest cause of an acute abdomen in children older 1 year of age. Only 5% of children with appendicitis are younger than 2 years of age. There is a familial preponderance. The younger the child the faster the symptoms of the disease are increasing in intensity. The symptoms starts with unspecific periumbilical or epigastric pain, followed by nausea, vomiting and restlessness at night. Finally the pain moves to the position of the appendix. The position of the appendix shows a high variation in children thus the pain characteristic is not uniform. Laboratory tests are not reliable but ultrasonography is recommended to exclude other diseases and to try to confirm the diagnoses. With the technique of "Graded compression Sonography" the rate of non identified appendicitis has been reduced under 5%. Laparoscopy is another option. Its use just for diagnostic purposes is limited but is recommended widely for primary therapeutic treatment with laparoscopic performed appendectomy. Laparoscopy has a special advantage against conventional appendectomy in the diagnostic of recurrent unspecific abdominal pain in children and in cases with interval appendectomy. Finally in pseudoappendicitis and pseudoperitonitis in children with immunvasculitis and other extraabdominal diseases. Letality of the acute appendicitis is zero.

[Transcutaneous and laparoscopic laser treatment in extensive retroperitoneal lymphangiomas in childhood]. / Die transkutane und laparoskopische Laseranwendung zur Behandlung ausgedehnter retroperitonealer Lymphangiome im Kindesalter.

Retroperitoneal lymphangiomas are rare congenital vascular malformations. They cannot always be completely excised and are associated with high recurrence, complication and morbidity rates. We therefore utilize an alternative treatment concept in some cases. We excise the cystic types laparoscopically with a Nd: YAG laser (wavelength 1064 nm). Residual tissues are percutaneously managed by interstitial laser therapy under MRI monitoring. The high soft-tissue contrast of the MRI enables exact positioning of the laser fiber. The examination is thermosensitive and provides online and noninvasive demonstration of the interstitial tissue coagulation. We have treated four infants laparoscopically and three other children percutaneously.

In vitro and in vivo MR imaging of hyaline cartilage: zonal anatomy, imaging pitfalls, and pathologic conditions.

Hyaline cartilage plays an essential role in the maintenance of normal synovial joint function by reducing friction and distributing loads. Histologic analysis of hyaline cartilage reveals zonal variation in cellular morphology, proteoglycan concentration, and collagen fiber size and orientation. High-resolution magnetic resonance (MR) imaging reveals an analogous laminar anatomy that is often visible on clinical images obtained with proper attention to technique. In vitro and in vivo pulse sequences show three distinct laminae: a hypointense superficial lamina, a hyperintense intermediate lamina, and a heterogeneous deep lamina that consists of alternating hyperintense and hypointense bands perpendicular to the subchondral bone. Imaging pitfalls include magic angle effects, truncation artifact, partial volume effect, regional anatomic variation, chemical shift, and magnetic susceptibility effects. Pathologic conditions that affect articular cartilage include chondromalacia patellae, osteoarthritis, and localized traumatic lesions. Although detection of early cartilage disease remains elusive, MR imaging can demonstrate intermediate and advanced lesions.

Silymarin retards collagen accumulation in early and advanced biliary fibrosis secondary to complete bile duct obliteration in rats.

Silymarin (SIL), a standardized plant extract containing about 60% polyphenole silibinin, is used as a hepatoprotective agent. Its antifibrotic potential in chronic liver diseases has not been explored. Therefore, we applied SIL to adult Wistar rats that were subjected to complete bile duct occlusion (BDO) by injection of sodium amidotrizoate (Ethibloc). This treatment induces progressive portal fibrosis without significant inflammation. Rats with sham-operation that received SIL at 50 mg/kg/d (n = 10) and rats with BDO alone (n = 20) served as controls, whereas groups of 20 animals were fed SIL at a dose of 25 and 50 mg/kg/d during weeks 1 through 6 or doses of 50 mg/kg/d during weeks 4 through 6 of BDO. Animals were sacrificed after 6 weeks for determination of blood chemistries, total and relative liver collagen (as hydroxyproline [HYP]), and the serum aminoterminal propeptide of procollagen type III (PIIINP). BDO in untreated rats caused an almost ninefold increase in total liver collagen (16.1 +/- 3.1 vs. 1.8 +/- 0.4 mg HYP, P < .001). SIL at 50 mg/kg/d reduced total HYP by 30% to 35%, either when given from week 1 through 6 or from week 4 through 6 after BDO (10.6 +/- 2.7 and 10.2 +/- 3.9 mg HYP, both P < .01 vs. BDO alone), whereas 25 mg/kg/d were ineffective. Because SIL at 50 mg/kg/d also reduced the collagen content per gram of liver tissue, it acted as a true antifibrotic agent. The single value of PIIINP at killing paralleled the antifibrotic activity of SIL with 11.6 +/- 3.8 and 9.9 +/- 3.7 vs. 15.3 +/- 5.2 microg/L in both high-dose groups (P < .05 and P < .01, respectively, vs. rats with BDO alone). Except for a decreased alkaline phosphatase and a lower histological fibrosis score in the groups that received SIL, clinical-chemical parameters were not different among all groups with BDO. We therefore conclude that 1) BDO with Ethibloc is a suitable model to test for pure antifibrotic drugs because it induces progressive rat secondary biliary fibrosis without major inflammation; 2) oral SIL can ameliorate hepatic collagen accumulation even in advanced (biliary) fibrosis; and 3) PIIINP appears to be a suitable serum marker to monitor the inhibition of hepatic fibrogenesis in this model of biliary fibrosis.

A 7-year-old child with primary tumour localisation in the distal duodenum--new imaging procedures for an improved diagnosis.

UNLABELLED: Duodenal gastrinomas in childhood are extremely rare and often missed at first medical consultation. We report on a 7-year-old child with sporadic gastrinoma of primary localisation in the distal duodenum. Small metastases in the liver and regional nodes were detected pre-operatively by somatostatin receptor scintigraphy (SRS) but not by other conventional imaging procedures. Diagnostic procedures include pre-operative SRS, endoscopic ultrasound and intra-operative endoscopic transillumination. CONCLUSION: Gastrinomas are rare abdominal tumours in childhood. Pre-operative tumour-specific diagnosis is possible by 111indium pentreotide SRS.