A case of tubulointerstitial nephritis and uveitis syndrome with severe immunologic dysregulation

Idiopathic tubulointerstitial nephritis and uveitis (TINU) syndrome is an uncommon condition, characterized by acute tubulointerstitial nephritis (TIN) with a favorable course and uveitis with a chronic relapsing course. The pathogenesis remains unclear, but a lymphocyte-mediated immune mechanism has been suggested. A 9-year-old boy was evaluated for fatigue and 2 kg of weight loss. Renal glucosuria, elevated urine ₂-microglobulin (MG), progressive renal dysfunction, polyclonal hypergammaglobulinemia, various autoantibodies and abnormal lymphocyte phenotypes were found. A renal biopsy revealed acute TIN. After 2 months of treatment with prednisolone, renal function and polyclonal hypergammaglobulinemia were normalized. While tapering prednisolone, anterior uveitis developed, which was improved with topical steroid. But abnormal lymphocyte phenotypes and autoantibodies persisted on low-dose prednisolone. Uveitis became aggravated, and urine ₂-MG increased again. The second renal biopsy (7 months later) was normal except for minimal focal interstitial fibrosis. Uveitis was not responsive to systemic steroids, but improved with additional cyclosporin. Abnormal lymphocyte phenotypes improved, and most autoantibodies disappeared. We report a rare case of idiopathic TINU syndrome with severe immunologic dysregulation, which correlated with the clinicopathological and biochemical parameters. The information about lymphocyte phenotypes and autoantibodies may provide more insight into the pathophysiology and the clinical course of uveitis in this rare disorder.     

General anaesthesia in a patient with Brugada syndrome

The successful administration of a combined general and epiduralanaesthetic to a patient with Brugada syndrome is reported.A review of the literature is presented. Br J Anaesth 2002; 89: 788–91     

Unusual presentation of long QT syndrome

We describe the case of a 9-yr-old child with undiagnosed longQT syndrome who experienced an intraoperative cardiac arrestafter accidental intravascular injection of bupivacaine withepinephrine via a misplaced epidural catheter. Br J Anaesth 2003; 90: 804–7     

Acute postoperative biventricular failure associated with antiphospholipid antibody syndrome

Antiphospholipid syndrome is probably the most common acquiredhypercoagulable state, but information on perioperative managementis sparse. Minor alterations in anticoagulant therapy, infection,or a surgical insult may trigger widespread thrombosis. Theperioperative course of a 31-yr-old woman with primary anticardiolipinantiphospholipid antibody syndrome requiring a mitral valvereplacement is described. Postoperatively, she developed acuteglobal biventricular failure requiring extracorporeal membraneoxygenation support and plasmapheresis. The management of anticoagulationand cardiac surgery in this condition is reviewed. Br J Anaesth 2004; 92: 748–54     

Anaesthesia in an adult with Rubenstein-Taybi syndrome using the ProSeal laryngeal mask airway

We report the anaesthetic management of an adult with Rubenstein–Taybisyndrome. This rare congenital syndrome is characterized bysevere learning difficulties, cardiac abnormalities, gastrooesophagealreflux, and cranio-facial abnormalities with the likelihoodof difficult intubation. A ProSeal laryngeal mask airway wasused to ventilate the patient for eye surgery. Br J Anaesth 2002; 89: 786–7     

Anaesthesia for Kartagener's syndrome

Kartagener’s syndrome is a hereditary syndrome involvinga combination of dextrocardia (situs inversus), bronchiectasisand sinusitis, transmitted as an autosomal recessive trait.We describe a patient who had three anaesthetics over a periodof a few months. Discussion relates to anaesthetic considerationsin the syndrome and to recent findings relating to the molecularmechanisms of left-right development. Br J Anaesth 2000; 85: 919–21     

Tubulointerstitial nephritis and uveitis in association with Epstein-Barr virus infection

 The case of a 13.5-year-old girl with acute tubulointerstitial nephritis and uveitis (TINU syndrome) is presented. The etiology of this rare syndrome, which in most cases involves female adolescents and usually regresses spontaneously, is still unknown. An infection-triggered pathological immune reaction has been considered to play a role in the pathogenesis of this disorder. Here we report for the first time the association of TINU syndrome and Epstein-Barr virus infection. Received: 4 May 1998 / Revised: 20 August 1998 / Accepted: 26 August 1998     

Complex regional pain syndrome

Br J Anaesth 2001; 87: 99–106     

Another feature of TURP syndrome: hyperglycaemia and lactic acidosis caused by massive absorption of sorbitol

Endoscopic transurethral resection of the prostate (TURP) canbe complicated by absorption of a large volume of irrigationfluid. The clinical features of this complication are referredas the TURP syndrome. We report a case where hyperglycaemiaand lactic acidosis complicated the TURP syndrome caused bythe massive absorption (approximately 15 litres) of a sorbitol–mannitol irrigation solution. The proposed mechanism is a typeB lactic acidosis related to the metabolism of sorbitol. Br J Anaesth 2001; 87: 316–19     

Xenon anaesthesia for laparoscopic cholecystectomy in a patient with Eisenmenger's syndrome

There are few reports on anaesthesia for patients with Eisenmenger’ssyndrome requiring non-cardiac surgery and none of the use ofxenon. We describe the use of xenon with a closed- circuit systemin a patient with Eisenmenger’s syndrome having a laparoscopiccholecystectomy. Br J Anaesth 2001; 86: 882–6     

Quantification of atelectatic lung volumes in two different porcine models of ARDS

Background. Cyclic recruitment during mechanical ventilationcontributes to ventilator associated lung injury. Two differentpathomechanisms in acute respiratory distress syndrome (ARDS)are currently discussed: alveolar collapse vs persistent floodingof small airways and alveoli. We compare two different ARDSanimal models by computed tomography (CT) to describe differentrecruitment and derecruitment mechanisms at different airwaypressures: (i) lavage-ARDS, favouring alveolar collapse by surfactantdepletion; and (ii) oleic acid ARDS, favouring alveolar floodingby capillary leakage. Methods. In 12 pigs [25 (1) kg], ARDS was randomly induced,either by saline lung lavage or oleic acid (OA) injection, and3 animals served as controls. A respiratory breathhold manoeuvrewithout spontaneous breathing at different continuous positiveairway pressure (CPAP) was applied in random order (CPAP levelsof 5, 10, 15, 30, 35 and 50 cm H₂O) and spiral-CT scans of thetotal lung were acquired at each CPAP level (slice thickness=1mm). In each spiral-CT the volume of total lung parenchyma,tissue, gas, non-aerated, well-aerated, poorly aerated, andover-aerated lung was calculated. Results. In both ARDS models non-aerated lung volume decreasedsignificantly from CPAP 5 to CPAP 50 [oleic acid lung injury(OAI): 346.9 (80.1) to 96.4 (48.8) ml, P<0.001; lavage-ARDS:245 17.6) to 42.7 (4.8) ml, P<0.001]. In lavage-ARDS poorlyaerated lung volume decreased at higher CPAP levels [232 (45.2)at CPAP 10 to 84 (19.4) ml at CPAP 50, P<0.001] whereas inOAI poorly aerated lung volume did not vary at different airwaypressures. Conclusions. In both ARDS models well-aerated and non-aeratedlung volume respond to different CPAP levels in a comparablefashion: Thus, a cyclical alveolar collapse seems to be partof the derecruitment process also in the OA-ARDS. In OA-ARDS,the increase in poorly aerated lung volume reflects the specificinitial lesion, that is capillary leakage with interstitialand alveolar oedema. This study contains parts of the doctoral thesis of ChristinaHartmann.     

Post-dural puncture headache in young adults: comparison of two small-gauge spinal catheters with different needle design

Background. To reduce the risk of post-dural puncture headache(PDPH) in continuous spinal anaesthesia, small-gauge spinalcatheter systems with different techniques of dural perforationhave been developed. Methods. Two systems, the catheter through-needle technique(MicroCatheter, Portex, UK) and the catheter over-needle technique(22G Spinocath®, B. Braun, Germany), were used in 18 younghealthy volunteers (age 18–30 yr), who were enrolled ina neuroendocrinological investigation for analysis of neuropeptidesin cerebrospinal fluid (CSF). After intermittent sampling ofCSF (17x0.5 ml over 4 h), the catheter was removed and the developmentof PDPH and pain intensity were documented prospectively bythe subjects in a standardized headache assessment (11-pointnumerical rating scale [NRS]). Results. The study revealed a high overall incidence of PDPH(78%) with no significant differences between groups (P=0.26).However, the over-needle group showed a significantly shorterduration of PDPH (2.4 [SD 2.3] vs 5.1 [3.1] days, P=0.050) andlower maximum pain intensity (3.1 [2.9] vs 7.3 [3.4] NRS, P=0.014)than the through-needle group. Conclusions. The results demonstrate a potential benefit ofthe catheter over-needle technique for the reduction of theduration and intensity of PDPH.     

A case of adult-onset tubulointerstitial nephritis and uveitis (“TINU syndrome”) associated with sacroileitis and epstein-barr virus infection with good spontaneous outcome

The authors report a case of tubulointerstitial nephritis and uveitis (TINU syndrome) in a 48-year-old woman. The patient's ocular symptoms (relapsing bilateral uveitis) began 4 months before the renal disease was diagnosed and were treated successfully with local steroid therapy. The main baseline laboratory findings were anemia, a rapid sedimentation rate, and a decreased renal function. Urinalysis results showed mild proteinuria and some hyaline and hyaline-granular casts. Immunoglobulin (Ig) G and IgM antibodies to Epstein-Barr virus (EBV) were present. The renal biopsy showed interstitial lymphocytes and infiltration by rare plasma cells, tubular atrophy without granulomas, and slight expansion of the mesangium; electronic microscopy showed rare electron-dense deposits in the mesangium; no vascular alterations were seen, and immunofluorescent staining was uniformly negative. X-ray of the pelvis showed bilateral sacroileitis, which has been previously described in only 1 case of TINU syndrome; human leukocyte antigen B27 was negative. After 6 months without any therapy, all laboratory findings were normal; after 30 months, renal function was still normal, uveitis had not relapsed, but sacroiliac involvement was still present; EBV-viral capsid antigen (VCA) IgM were still high (28 UA/mL), and the EBV IgG titers were increased (VCA>170, EBV-nuclear antigen 108 UA/mL). This case confirms that this rare entity, originally observed in children, may occur and have a favorable spontaneous renal outcome also in the adult; EBV may play a role, as previously suggested. This is, to the authors’ knowledge, the first reported case of TINU syndrome with the association of a well-documented bilateral sacroileitis.     

Tubulointerstitial nephritis and uveitis: an immunological disorder?

A 14-year-old boy with tubulointerstitial nephritis and uveitis (TINU syndrome) is described. Nephropathy improved without systemic cortisone treatment, whereas uveitis relapsed and was treated with topical steroids. Blood cell immunological analysis and serum analysis revealed signs of cytotoxic T-cell, macrophage and granulocyte activation, which declined as the clinical symptoms improved. This may be interpreted as an indication of their significance as markers in the pathogenesis of this syndrome or as part of a microbial-triggered immune response.     

Incremental spinal anaesthesia for elective Caesarean section in a patient with Eisenmenger's syndrome

We describe a new approach to anaesthesia for elective Caesareansection in a woman with Eisenmenger’s syndrome. Incrementalregional anaesthesia was performed using a microspinal catheterand haemodynamic monitoring included transthoracic bioimpedancecardiography. This approach allowed the disadvantages of generalanaesthesia and invasive cardiac output monitoring to be avoided. Br J Anaesth 2001; 86: 723–6     

Tracheostomy in a patient with severe acute respiratory syndrome

The coronavirus which causes severe acute respiratory syndrome(SARS) is a virulent and highly contagious organism. Of the1755 SARS patients in Hong Kong, over 400 were healthcare workers.Meticulous attention to infection control and teamwork are essentialto minimize cross-contamination and prevent staff from contractingthe illness. These points are especially pertinent when anaesthetizingSARS patients for high-risk procedures such as tracheostomy.We describe the management of such a case. Br J Anaesth 2004; 92: 280–2     

Long QT syndrome and anaesthesia

Br J Anaesth 2003; 90: 349–66     

Does the opioid-sparing effect of rectal diclofenac following total abdominal hysterectomy benefit the patient?

Background. The aim of this prospective, double-blind, randomized,placebo-controlled clinical trial was to investigate the opioid-sparingeffects of rectal diclofenac following total abdominal hysterectomy. Methods. Forty ASA I–II patients, aged 20–60 yr,were randomized to receive identical-looking suppositories ofeither diclofenac 75 mg or placebo, twice daily. All patientswere given a standardized anaesthetic, with intravenous morphinevia a patient-controlled analgesia device and either diclofenacor placebo for postoperative analgesia. Results. The median 24 h morphine consumption (interquartilerange) was significantly higher (P=0.02) in the placebo group[59 (45–85) mg] than in the diclofenac group [31 (14–65)mg]. In comparison with the placebo group, there were significantreductions in total pain score in the diclofenac group at rest(P=0.04) and on movement (P<0.01). Total (SD) sedation scorewas significantly lower (P=0.04) in the diclofenac group [90(73) mm] than in the placebo group [148 (89) mm]. Total (interquartilerange) nausea score was significantly lower (P<0.01) in thediclofenac group [14 (0–53) mm] than in the placebo group[64 (30–109) mm]. There was no significant differencebetween the two groups of patients in episodes of vomiting ornumber of rescue antiemetics. Conclusions. Rectal diclofenac reduces morphine consumption,improves postoperative analgesia, and reduces the incidenceof adverse effects such as sedation and nausea. Br J Anaesth 2002; 88: 714–16     

Haemodynamic effects of haemorrhage during xenon anaesthesia in pigs

Background. It was hypothesized that xenon would stabilize meanarterial pressure (MAP) in haemorrhagic shock, recovery, andvolume resuscitation, because a higher MAP has been observedwith xenon, when compared with isoflurane anaesthesia. The responsesto haemorrhage and subsequent volume replacement were thereforecompared between xenon and isoflurane anaesthesia, in pigs. Methods. Pigs were randomized to anaesthesia with xenon 0.55MAC (group Xe, n=9) or isoflurane 0.55 MAC (group Iso, n=9),each with remifentanil 0.5 µg kg^–1 min^–1.MAP, heart rate, cardiac output (CO), and left ventricular fractionalarea change (FAC) were collected at control (1), after haemorrhage(20 ml kg^–1) (2), after 10 min of recovery (3), aftervolume replacement (4), and 30 min later (5). Data were analysedby two-way repeated measures ANOVA. Results. Blood loss decreased MAP (Xe: 103 [21] to 53 [24] mmHg; Iso: 92 [18] to 55 [14] mm Hg) and CO (Xe: 4.1 [0.8] to2.6 [0.5] litre min^–1; Iso: 5.1 [1.1] to 3.8 [1.2] litremin^–1), in spite of significant tachycardia. MAP and COrecovered to about 75% of control, and subsequent volume replacementcompletely reversed symptoms in both groups, but increased FAConly with xenon. Conclusion. Haemodynamic response to acute haemorrhage appearedfaster with xenon/remifentanil than with isoflurane/remifentanilanaesthesia. In particular MAP decrease and short-term recoverywere more marked with xenon (P<0.02). In the xenon group,volume replacement increased FAC compared with control and isoflurane(P<0.02).