Sensory nerve conduction studies in infants, children and teenagers - An update.

Objective: Nerve conduction studies (NCS) in children remain technically challenging and depend on the cooperation of the child. Motor NCS are not compromised by analgosedation but data for sensory NCS are lacking. Here, we ask whether sensory NCS is influenced by analgosedation. We also compare the present data with NCS studies from the 1990s regarding anthropometric acceleration of the contemporary paediatric population. Methods: Sensory NCS of the median nerve and sural nerve were performed in 182 healthy subjects aged 1 to 18 years during general anaesthesia and in 47 of them without analgosedation. Results: Sensory NCS was not influenced by midazolam or propofol. The sensory nerve action potential (SNAP) amplitude and the nerve conduction velocity (NCV) of the sural nerve as well as the SNAP of the median nerve show no significant age dependence in age range 1-18 years. The sensory NCV of the median nerve increased age-dependent. Conclusions: In clinical practice, analgosedation can be used for diagnostic NCS. Sensory NCS data show no relevant secular trend over the last 30 years. Differences due to technical inconsistency predominate. Significance: Analgosedation can improve diagnostic quality of sensory NCS in children. Additionally, we provide sensory NCS values from a large pediatric cohort.

Laparoscopic cholecystectomy for symptomatic cholecystolithiasis (CCL) in "Kasabach-Merritt syndrome" (KMS) (Kaposi-tumor like hemangioendothelioma with case-specific perioperative management).

Objectives: The Kasabach-Merritt syndrome (KMS) is characterized by the occurrence of hemangioendothelioma (giant hemangioma with thrombosis leading to thrombocytopenia), which can be associated with disseminated intravasal coagulation. Specific aim: Based on (i) selective references from the current scientific literature and derived recommendations as well as (ii) own experiences obtained in the diagnostic and perioperative management of a representative case from daily practice in abdominal surgery, the specific case undergoing elective cholecystectomy (CCE) in KMS is to be described by means of scientific case report. Case presentation: (Patient-, finding- and treatment-specific characteristics): - Medical history: 72-years old female patient with a known KMS of the left arm and upper thorax, recurrent thrombophlebitis of the left arm and thoracic veins, previous upper GI bleeding (Mallory-Weiss syndrome in 2006, chronic anemia in lack of vitamin B12, type-A gastritis, former bleeding complications after teeth extraction/open appendectomy 1962/Caesarean section 1968 with need of transfusion [60 red blood cell packages]), intraabdominal adhesions, hypothyreosis, initial liver cirrhosis. - Symptomatology: Characteristic for cholecystolithiasis (CCL). - Diagnostic: Abdominal ultrasound shows CCL, fibroscan does not confirm suspicious cirrhosis. Laboratory parameters showed: Activation of intravasal coagulation with elevated prothrombin fragments, D-dimers and reduced antiplasmin concentration. Accelerated fibrinolysis capacity; currently, no secondary thrombocytopenia or factor-13 decrease. In addition, fibrinogen concentration within normal range, no hint onto the manifestation of an aquired von-Willebrand's syndrome. - Diagnosis: Chronic fibrosing cholecystitis in CCL after former acute cholecystitis (3 months ago) with indication for surgical intervention. - Therapy: Laparoscopic CCE including careful exploration of upper abdominal cavity for KMS manifestation (with no revision of bile duct) and peritoneal adhesiolysis (histological finding, chronic fibrosing cholecystitis with thickening of the wall of the gall bladder but no hint of malignancy) under perioperative prophylaxis with antibiotics and temporary cessation of platelet medication for 7 d preoperatively, "bridging" with low molecular weight heparin (Clexane, 1 × 40 mg s.c.; Sanofi-Aventis, Frankfurt/Main, Germany); 1 h preoperatively, 15-20 mg/kg body weight Cyclocapron i.v. (once again 6-8 h postoperatively; thereafter, 500 mg of Cyclocapron 4×/d until the 3rd postoperative day). - Intraoperatively: Congestion of veins but not at the immediate surgical field (gall bladder, hepatic bed of the gall bladder, Calot's triangle). - Outcome: Uneventful, in particular, no (bleeding) complications. Conclusions: If surgical approach is indicated, the intervention should be thoroughly planned (in particular, under elective circumstances) with regard to hemangioma site and extension as well as distance to the surgical field and possible surgical alternative options (surgical access site, open/laparoscopic approach etc.) to prevent - at the best possible rate - bleeding complications intra-/postoperatively and, thus, to provide adequate patient safety.

Outcome after Hypospadias Repair: Evaluation Using the Hypospadias Objective Penile Evaluation Score.

INTRODUCTION: The Hypospadias Objective Penile Evaluation Score (HOPE-Score) is a concise and reproducible way to describe hypospadias severity. We classified boys undergoing primary hypospadias repair to determine the correlation between the HOPE-Score and the severity of hypospadias first and the outcome after surgery second. MATERIALS AND METHODS: Patients who underwent primary hypospadias repair from 2005 to 2014 were identified. An independent physician assessed retrospectively the HOPE-Score, using photographies of the patients before, after primary surgery, and after all necessary surgeries. The correlation between the HOPE-Score and the severity of hypospadias, on the one hand, and the outcome after surgery, on the other hand, were analyzed. RESULTS: The HOPE-Score was assessed preoperatively for 79 boys, postoperatively for 66, and after all necessary surgeries for 21 patients. Mean HOPE-Score reached 30.2 ± 5.9 before surgery, 42.2 ± 6.1 after primary surgery, and 43.7 ± 3.4 after all necessary surgeries. A significant correlation between the HOPE-Score and the severity of hypospadias before surgery was observed. The boys with glanular hypospadias scored significantly higher (36.3 ± 5.4) than those with distal (29.6 ± 4.4) and proximal hypospadias (21.1 ± 3.5). Furthermore, a significant correlation between the HOPE-Score and the outcome after hypospadias repair was observed. Patients who needed no reintervention after primary hypospadias repair scored significantly higher postoperatively (45.1 ± 5.4) than those who needed a second (40.8 ± 4.2) or more than two surgeries (36.9 ± 7.4). CONCLUSION: The HOPE-Score is a good system to assess the severity of hypospadias and the cosmetic outcome after hypospadias repair.

T Cells of Infants Are Mature, but Hyporeactive Due to Limited Ca2+ Influx.

CD4 T cells in human infants and adults differ in the initiation and strength of their responses. The molecular basis for these differences is not yet understood. To address this the principle key molecular events of TCR- and CD28-induced signaling in naive CD4 T cells, such as Ca2+ influx, NFAT expression, phosphorylation and translocation into the nucleus, ERK activation and IL-2 response, were analyzed over at least the first 3 years of life. We report dramatically reduced IL-2 and TNFα responses in naive CD31+ T cells during infancy. Looking at the obligatory Ca2+ influx required to induce T cell activation and proliferation, we demonstrate characteristic patterns of impairment for each stage of infancy that are partly due to the differential usage of Ca2+ stores. Consistent with those findings, translocation of NFATc2 is limited, but still dependent on Ca2+ influx as demonstrated by sensitivity to cyclosporin A (CsA) treatment. Thus weak Ca2+ influx functions as a catalyst for the implementation of restricted IL-2 response in T cells during infancy. Our studies also define limited mobilization of Ca2+ ions as a characteristic property of T cells during infancy. This work adds to our understanding of infants' poor T cell responsiveness against pathogens.

Surgical Treatment Results In Gastroschisis Based On Preterm Delivery Within The 34th Week Of Gestation By Caesarean Section.

UNLABELLED: The aim of the study was to assess the value of the today's appropriate approach, preterm delivery in the 34th week of gestation by Caesarean section and subsequent surgical intervention at the perinatal center, in daily practice of pediatric surgery with regard to early postoperative and mid-term outcome. MATERIAL AND METHODS: Over the time period of 9 years, all consecutive cases diagnosed with gastroschisis at the perinatal center, University Hospital of Magdeburg, were born by Caesarean section within the 34th week of gestation followed by surgical intervention. The registered data were compared with those published by other groups. RESULTS: Overall, there were 19 cases through the investigation period from 01/01/2006 to 12/31/2014. The mean duration of gestation was 237.9 days. The mean birth weight was 2,276 g. In all individuals, a primary closure with no artificial material was achieved. The duration of postoperative artificial respiration was 2.3 days. Oral uptake could be initiated on the 10th postoperative day on average. The mean hospital stay was 37 days. There was no lethality. As complications, postoperative (iv catheter-associated) sepsis occurred in one case and relaparotomy became necessary in a further case because of no possible completion of enteral nutrition by 20 days after primary closure (complication and relaparotomy rate, 10.5% and 5.26%, respectively). CONCLUSIONS: The data indicate that in case of gastroschisis, primary closure can be more frequently achieved by section within the 34th week of gestation. Under the prediction of an optimal neonatological care, the risks of a preterm delivery by a planned section appear to be manageable.

Meconium-induced periorchitis.

A testicular tumour-like lesion or a solid extratesticular mass are serious postnatal problems with a broad spectrum of inflammatory or tumorous causes and possible differential diagnoses. In this case report, an extraordinary case of a newborn boy with a rare cause of a periorchitis is described.A premature boy infant (25-year-old mother with obesity) was diagnosed as having a disturbance of prosperity. A plain film of the abdomen showed a dilated intestine (no pathological findings in abdominal ultrasound, in particular, no ascites). After 3 postnatal months of clinical observation at the hospital including temporary parenteral nutrition, hydrocele at both sides was diagnosed. Therefore, the boy underwent surgical exploration. Intraoperatively, the processus vaginalis peritonei was surprisingly filled with meconium; because of that, the situs was extensively rinsed. In addition, the boy underwent an operation for the right and left hydrocele. The testes were preserved (on follow-up investigation there were no further problems).Pathohistological investigation confirmed meconium periorchitis, which is a rare postnatal disease and which can only develop in cases of former meconium peritonitis and if the processus vaginalis peritonei is open. The precise details of cause and pathophysiology in this case may significantly help in avoiding unnecessary orchiectomy.

Implantation of human demineralized bone matrix (DBM) for the treatment of juvenile bone cysts.

OBJECTIVE: Definitive bony consolidation in juvenile bone cysts. Prevention of pathologic fractures. Preservation of limb function. INDICATIONS: Juvenile bone cysts at all sites. CONTRAINDICATIONS: Malignant cystic lesions. SURGICAL TECHNIQUE: After opening and curettage, the cyst is packed with human demineralized bone matrix (DBM). POSTOPERATIVE MANAGEMENT: Clinical and radiologic checks after 1, 4, and 6 months, followed by further 6-monthly checks. RESULTS: Over a period of 2 years, nine cysts packed with DBM showed almost totally osteodense images after an average of 8 months, with no other significant changes (follow-up period: 24 months). A typical decrease in cyst transparency on the plain radiographs was already detectable in all patients after 3-4 months. Marked cortical remodeling was visible after 6 months. A significant complication in one cyst in the distal tibial region was a pathologic fracture following distortion trauma; this occurred after 5 months, probably because of insufficient filling of the cyst. The fractured limb was immobilized in a lower-leg cast and healed sufficiently for stable weight bearing after 12 weeks.