Bradycardia with sevoflurane in siblings with Branchio-oto-renal syndrome

Branchio-oto-renal syndrome (BOR, Melnick-Fraser syndrome, MIM#113650) refers to a rare autosomal dominant disorder characterized by branchial cysts or fistulas, hearing loss, external ear malformation, preauricular pits and renal abnormalities. The authors present three episodes of significant bradycardia in two siblings diagnosed with BOR syndrome during the sevoflurane general anesthesia. There is no published experience of anesthesia with this syndrome. Bradycardia occurred variously at induction, maintenance and immediately prior to emergence and required surgical stimulation, atropine, or epinephrine to treat. We seek to raise awareness of the potential for bradycardia during the procedures in patients with this syndrome requiring volatile anesthesia, especially sevoflurane.     

Increases in BMD correlate with improvements in bone microarchitecture with teriparatide treatment in postmenopausal women with osteoporosis.

Increases in BMD are correlated with improvements in 2D and 3D trabecular microarchitecture indices with teriparatide treatment. Therefore, improvements in trabecular bone microarchitecture may be one of the mechanisms to explain how BMD increases improve bone strength during teriparatide treatment. INTRODUCTION: Bone strength is determined by BMD and other elements of bone quality, including bone microarchitecture. Teriparatide treatment increases BMD and improves both cortical and trabecular bone microarchitecture. Increases in lumbar spine (LS) BMD account for approximately 30-41% of the vertebral fracture risk reduction with teriparatide treatment. The relationship between increases in BMD and improvements in cortical and trabecular microarchitecture has not yet been studied. MATERIALS AND METHODS: The relationship between increases in BMD and improvements in cortical and trabecular microarchitecture after teriparatide treatment was assessed using data from a subset of patients who had areal BMD measurements and structural parameters from transiliac bone biopsies in the Fracture Prevention Trial. 2D histomorphometric and 3D microCT parameters were measured at baseline and 12 (n = 21) or 22 (n = 36) mo. LS BMD was assessed at baseline and 12 and 18 mo, and femoral neck (FN) BMD was measured at baseline and 12 mo. Pearson correlation was performed to assess the relationship between actual changes in BMD and actual changes in microarchitectural parameters. RESULTS: Changes in LS BMD at 12 mo were significantly correlated with improvements in trabecular bone structure at 22 mo: 2D bone volume (r = 0.45, p = 0.02), 2D mean wall thickness (r = 0.41, p = 0.03), 3D bone volume (r = 0.48, p = 0.006), 3D trabecular thickness (r = 0.44, p = 0.01), 3D trabecular separation (r = -0.37, p = 0.04), 3D structural model index (r = -0.54, p = 0.001), and 3D connectivity density (r = 0.41, p = 0.02). Changes in LS BMD at 18 mo had similar correlations with improvements in bone structure at 22 mo. Changes in FN BMD at 12 mo were significantly correlated with changes in 2D mean wall thickness (r = 0.56, p = 0.002), 3D bone volume (r = 0.51, p = 0.004), 3D trabecular thickness (r = 0.44, p = 0.01), 3D trabecular separation (r = -0.46, p = 0.01), and 3D structural model index (r = -0.55, p = 0.001). CONCLUSIONS: Increases in BMD are correlated with improvements in trabecular microarchitecture in iliac crest of patients with teriparatide treatment. Therefore, improvements in trabecular bone microarchitecture may be one of the mechanisms to explain how BMD increases improve bone strength during teriparatide treatment.     

Pyloroplasty in association with Nissen fundoplication in children with neurologic disorders

Previous studies have suggested that delayed gastric emptying occurs in severely mentally retarded patients with gastroesophageal reflux. Based on this data, pyloroplasty was employed in such patients. A retrospective analysis of 99 consecutive patients who underwent primary fundoplication for GER was performed. Gastric emptying, as measured by successful removal of the nasogastric tube or elevation of the gastrostomy tube, was studied. Children with neurologic disorders had no clinically significant difference in gastric emptying after fundoplication (3.31 days) when compared with neurologically normal patients (2.21 days). When added to Nissen fundoplication, pyloroplasty did not hasten the return of gastrointestinal function in the severely impaired patients (4.91 days). A prospective study employing gastric isotope bolus feedings before and after Nissen fundoplication will determine if pyloroplasty improves gastric emptying when used in conjunction with Nissen fundoplication for patients with severe neurologic disorders.     

Chemotherapy in patients with teratoma with malignant transformation

OBJECTIVE: Germ-cell tumours (GCTs) with a non-GCT malignant component are a unique and rare phenomenon called teratoma with malignant transformation (TMT). The only published series of patients with TMT treated with chemotherapy comprised 10 patients. We report here our experience in treating 14 patients with TMT. PATIENTS AND METHODS: Sarcoma was identified in 10 of 14 patients, with rhabdomyosarcoma ranking first (n=4). Other histological types included adenocarcinoma (n=3) and bronchoalveolar carcinoma (n=1). Immunohistochemistry was performed to help in identifying the malignant non-GCT component. RESULTS: Primary treatment consisted of surgery alone in 4 patients. The remaining 10 patients received first-line cisplatin-based chemotherapy with resection of residual masses (n=5): 4 patients had a complete response and 5 had a partial response. Overall, 9 patients developed a relapse with a median time of 84 mo (range: 6-168). At relapse, 8 patients received a chemotherapy regimen directed to the non-GCT component. Four of these patients achieved a partial response. With a median follow-up of 59 mo (range: 3-180), 4 of 14 patients are alive, including 3 who are disease-free. CONCLUSION: To our knowledge, this is by far the largest reported European series of chemotherapy in TMT. Although TMT has a poor prognosis compared to GCT, its management may be improved by adapted chemotherapy associated with surgical resection of residual masses.     

Bradycardia with sevoflurane induction in patients with trisomy 21

The authors present a series of three paediatric patients with trisomy 21 who developed significant bradycardia during inhalation induction with sevoflurane. All three were undergoing adenotonsillectomy. The possible association of such problems with trisomy 21 and treatment options are reviewed. Although the occurrence of bradycardia in these three patients may have been a random occurrence, given its occurrence in three of five consecutive patients with trisomy 21, a causal relationship may be involved and future observation of these patients appears warranted.     

Atherosclerosis in patients with analgesic nephropathy treated with haemodialysis

SUMMARY: Accelerated atherosclerosis was reported to be associated with chronic analgesic consumption, but most studies were retrospective, and individual findings have almost never been controlled with regard to other atherosclerotic risk factors. Ten haemodialysis patients with analgesic nephropathy (group I) and 19 haemodialysis patients where renal failure was not caused by analgesic nephropathy (group II) were included in the study. All patients were female without diabetes. Using B-mode ultrasonography, we compared intima-media thickness (IMT) in the carotid arteries and plaque occurrence, and their thickness in group I with that in group II. the possible differences in atherosclerotic risk factors in both groups were also investigated. In group I, the average age was 60.2 years, and the average dialysis treatment was 55.7 months. In group II, the average age was 54.6 years, and the average duration of dialysis treatment was 50.4 months. We found no statistically significant difference in the age and duration of dialysis treatment between groups I and II. the IMT values of the carotid arteries (0.97 vs 0.78 mm; P = 0.027) were significantly higher in group I. More patients had plaques in group I (90 vs 57.9%), and the number of plaques ( P = 0.037) and their thickness ( P = 0.043) were significantly higher in this group. There was no statistically significant difference in the atherosclerotic risk factors between groups I and II. the results indicate that patients with analgesic nephropathy treated with haemodialysis showed advanced atherosclerosis compared with other haemodialysis patients, despite no difference being found in the atherosclerotic risk factors between these patients.     

Experience with tacrolimus in children with steroid-resistant nephrotic syndrome

Children with steroid-resistant nephrotic syndrome (SRNS) are at risk of developing renal failure. We report here the results of a single-center retrospective observational study of the remission rate in pediatric patients with SNRS receiving tacrolimus. Serial renal biopsies from children on tacrolimus therapy were evaluated for tubulointerstitial fibrosis and transforming growth factor-β immunostaining. Of the 16 children with SRNS, 15 went into complete remission after a median of 120 days of therapy. Nine children were able to stop steroids, while the others were on tapering doses. Forty-seven percent had relapses, most of which were steroid-responsive. Serial renal biopsies were obtained from seven children after a median treatment duration of 24 months; two of these children had increased tubulointerstitial fibrosis and four showed increased transforming growth factor-β tissue staining. Children with worsening histological findings were younger. There was no significant association between tacrolimus exposure and biopsy changes, although the average trough level was higher in those children with worsening histological findings. In conclusion, tacrolimus may be a safe and effective alternative agent for inducing remission in children with SRNS. However, caution needs to be taken when prescribing this agent due to its narrow therapeutic index. Serial renal biopsies are necessary to check for subclinical nephrotoxicity, especially in younger children and those with higher trough levels.     

Satisfaction with upper extremity surgery in individuals with tetraplegia

Objective

To supplement the scant information available regarding the satisfaction of patients with tetraplegia following upper extremity reconstructive surgery for such individuals with spinal cord injury (SCI).

Study design

Retrospective study with questionnaire follow-up.

Setting

The Danish Spinal Cord Injury Centers.

Material and methods

In the initial review period, 119 upper extremity surgeries were performed on patients with tetraplegia (n = 49). Seven died and the remaining 42 were invited to complete a follow-up questionnaire with a five-level scale ranging from strongly agree to strongly disagree regarding satisfaction. Forty patients completed the questionnaire.

Results

Median time from first surgery was 13 years (2–36). Sixty-five percent of the sample had a C5–C6 SCI, with 64% experiencing complete injury. Initially, 76% of the sample expressed general satisfaction with life, but only 28% of the sample reported that hand appearance improved after surgery. Interestingly, those having surgery from 1991 to 2008 reported significantly greater satisfaction (P < 0.001) and were significantly more satisfied with activities of daily living (ADL) (P < 0.001) than those having surgery between the years 1973 and 1990. In particular, gain of independence was obtained with pinch/specific hand surgery compared to triceps activation. Accordingly, the pinch/specific hand surgery group was significantly more satisfied than the triceps group on the ADL (P = 0.027), and the independence questions (P < 0.001).

Conclusion

Overall satisfaction with upper extremity surgery is high. It can have a positive impact on life in general, ability to perform ADL, as well as supplying an increased level of independence.     

Glycopyrrolate compared with atropine in association with ketamine anaesthesia

Atropine and glycopyrrolate given intravenously before the induction of a ketamine anaesthesia to diminish salivary secretion were compared for their effect on psychotomimetic side-effects, awakening time and heart rate. Though atropine is a tertiary amine that crosses the blood-brain barrier, which glycopyrrolate as a quaternary ammonium compound does not, it did not increase the incidence of psychotomimetic side-effects nor did it significantly prolong the awakening time after ketamine anaesthesia. During intubation the increase in heart rate was significantly higher following atropine than following glycopyrrolate.     

Long-term therapy with enalapril in patients with nephrotic-range proteinuria

The effect of enalapril on urinary protein excretion and renal function was studied in six paediatric patients with various renal diseases causing nephrotic-range proteinuria. In three younger children (aged 7 – 9 years) with steroid-resistant nephrotic syndrome, enalapril at a dose of 0.5 mg/kg per day given for 24 months yielded a temporary reduction of proteinuria in one child, a moderate and steady decrease in another and a complete disappearance of proteinuria in the third. Three adolescents, aged 17 years, took enalapril for 24 months at a dose of 20 mg/day. We observed no effect on proteinuria in one patient with Alport syndrome, a complete disappearance of urinary protein in one patient with membranoproliferative glomerulonephritis and a moderate decrease in the third patient who had idiopathic steroid-resistant nephrotic syndrome. Enalapril therapy resulted in an important reduction of proteinuria in two patients and a moderate decrease in three others. However this therapy was accompanied by a fall in glomerular filtration in all subjects, which was very marked in two patients. This fall in glomerular filtration may, however, simply reflect the natural course of the disease. Received June 2, 1995; received in revised form and accepted March 7, 1996     

Tomographic studies of rCBF with99mTc-HM-PAO SPECT in comparison with PET in patients with primary brain tumors

^99mTc-hexamethyl-propyleneamine-oxime (HM-PAO) was developed as a radiotracer of regional cerebral blood flow (rCBF) with SPECT. THe purpose of this study was to investigate if HM-PAO is able to trace rCBF in primary brain tumors. In 10 patients with glioblastoma grade IV the intracerebral distribution of HM-PAO was studied in comparison with C¹⁵O₂ steady state inhalation technique and PET for rCBF evaluation. In all instances the cerebral HMPAO distribution was comparable with rCBF pattern as confirmed by a significant correlation of tumor to cortex ratios. The results indicate the clinical usefulness of HMPAO for tracing rCBF in brain tumors.     

Anticoagulation with argatroban in a parturient with heparin-induced thrombocytopenia

Unfractionated heparin and low-molecular-weight heparin are currently the anticoagulants of choice for the prevention of recurrent thromboembolic disease during pregnancy. However, heparin-induced thrombocytopenia contraindicates the use of unfractionated heparin and low-molecular-weight heparin. We describe a patient who was admitted to our hospital with deep vein thrombosis at 18 weeks of gestation and who developed heparin-induced thrombocytopenia during her antenatal care. Therapeutic anticoagulation was initially achieved with argatroban, then changed to fondaparinux. During early labor, fondaparinux was discontinued and intravenous argatroban was substituted. Argatroban was discontinued during transition to active labor. After return of a normal partial thromboplastin time, combined spinal-epidural analgesia was induced for routine completion of labor and vaginal delivery. We discuss the decisions made in the maintenance of this patient’s anticoagulation during the peripartum period as well as timing of her neuraxial labor analgesia.