"Craniofacial and dental manifestations of a case of Proteus syndrome"

Proteus syndrome, which is a reference to the ancient Greek god Proteus, the Polymorphous, was called to the attention of pediatricians. Recognition of this syndrome has been difficult because of the variability of the syndrome's manifestations and because of its rarity. We describe a rare case of Proteus syndrome and we discuss its differential diagnosis. Our case presents with hemi facial hypertrophy, scrotal tongue, enamel hyperplasia as well as differential in the size of the dentition on both sides of the arch.     

Assessment of tracheal intubating conditions in children using remifentanil and propofol without muscle relaxant

BACKGROUND: Tracheal intubation in children can be achieved by deep inhalational anaesthesia or an intravenous anaesthetic and a muscle relaxant, suxamethonium being widely used despite several side-effects. Studies have shown that oral intubation can be facilitated safely and effectively in children after induction of anaesthesia with propofol and alfentanil without a muscle relaxant. Remifentanil is a new, ultra-short acting, selective mu-receptor agonist that is 20-30 times more potent than alfentanil. This clinical study was designed to assess whether combination of propofol and remifentanil could be used without a muscle relaxant to facilitate tracheal intubation in children. METHODS: Forty children (5-10 years) admitted for adenotonsillectomy were randomly allocated to one of two groups to receive remifentanil 2 microg.kg(-1) (Gp I) or remifentanil 3 microg.kg(-1) (Gp II) before the induction of anaesthesia with i.v. propofol 3 mg.kg(-1). No neuromuscular blocking agent was administered. Intubating conditions were assessed using a four-point scoring system based on ease of laryngoscopy, jaw relaxation, position of vocal cords, degree of coughing and limb movement. Mean arterial pressure (MAP) and heart rate (HR) measured noninvasively before induction of anaesthesia to 5 min after intubation (seven time points). RESULTS: Tracheal intubation was successful in all patients without requiring neuromuscular blocking agent. Intubating conditions were clinically acceptable in 10 of 20 patients (50%) in Gp I compared with 18 of 20 patients (90%) in Gp II (P < 0.05). MAP and HR decreased in both groups after induction of anaesthesia (P < 0.01). Both HR and MAP were significantly lower in Gp II compared with Gp I after tracheal intubation (P < 0.01). No patient in the present study developed bradycardia or hypotension. CONCLUSIONS: We conclude that remifentanil (3 microg.kg(-1)), administered before propofol (3 mg.kg(-1)) provides acceptable tracheal intubating conditions in children, and completely inhibited the increase in HR and MAP associated with intubation.     

DETECTION OF EUBACTERIA IN INTERSTITIAL CYSTITIS BY 16S rDNA AMPLIFICATION

Objective

To determine what role non-culturable microorganisms play in the etiology of interstitial cystitis (IC).

Materials and Methods

Thirty patients fulfilling NIH criteria for the diagnosis of interstitial cystitis and sixteen control patients with culture negative urine gave written informed consent and underwent bladder biopsy. Polymerase chain reaction (PCR) using two sets of universal primers for bacterial 16S rDNA was performed on urine from the cystoscope and on a cold cup bladder biopsy specimen. Of the PCR positive bladder biopsies, three patients with interstitial cystitis and three controls were randomly selected and cloned. Ten clones from each were sequenced and putative taxonomic assignments made.

Results

12/26 (46%) IC and 5/12 (42%) control urine specimens and 16/30 (53%) and 9/15 (60%) bladder biopsies were PCR positive, respectively. The bacterial populations in the two patient groups tested appeared to be different based upon analysis of the 16S rRNA sequences.

Conclusions

Both IC and control patients had non-culturable bacteria in their bladders. A random sampling of the two populations revealed that the bacterial populations are different, suggesting a possible link between one or more bacterial species and IC.     

Hepatitis C Screening and Treatment versus Liver Transplantation

Objectives: To: (i) calculate the number needed to screen (NNS) and community effectiveness of combination treatment (interferon-α plus ribavirin) compared with its efficacy of 28 to 45%; (ii) compare the cost of opportunistic hepatitis C virus (HCV) screening and combined treatment to prevent 1 high risk patient developing cirrhosis with the cost of liver transplantation; and (iii) put a cash value on how much purchasing authorities should bid in a commissioning round for new HCV treatments. Design: (i) A financial option appraisal of liver transplantation versus opportunistic HCV screening and treatment; (ii) a financial/commissioning model from a purchasers’s perspective using the latest national and international standards/guidelines and audit reports. Setting and participants: West Kent Health Authority (963 000 residents and 9 primary care groups). All patients at high risk of HCV presenting to health services in 1998/1999 who had an anti-HCV test. Results: Using current National Institute of Clinical Excellence (NICE) guidance (combined treatment for moderate to severe liver changes), about 113 479 high risk persons need to be screened and medically treated, costing about 50 947 pounds sterling (£) to prevent 1 patient developing cirrhosis in 10 to 20 years. The community effectiveness therefore is 0.00088%.Thenetpresent value (NPV) of HCV screening and combined treatment is ?£32 471 to ?£25 407 to prevent 1 patient developing cirrhosis compared with liver transplantation; and the marginal cost to fund current volumes is £54 000. In contrast, if current international standards were used (which advocates combined treatment for mild, moderate and severe liver changes), the NNS is 118 to prevent 1 high risk patient developing cirrhosis; the community effectiveness is 0.847%; the NPV is ?£6479 to £584; and the variable cost to fund current volumes is £796 850. Conclusion: The community effectiveness of combined treatment, at best (i.e. using the international model), is 33 to 53 times less than its efficacy; HCV screening and drug treatment is more expensive than liver transplantation. Furthermore, the international model is a far more technically efficient pathway for delivering HCV care than that recommended by NICE guidance.     

Free oxygen radicals in acute renal failure

OBJECTIVE: To assess the levels of free oxygen radicals in acute renal failure and their predictive value in clinical outcome. DESIGN: Prospective. SETTING: Intensive care unit. METHODS: Study was conducted in 50 children (25 with acute renal failure and 25 age and sex matched controls). Blood urea, serum creatinine, serum protein, uric acid and free oxygen radical markers were estimated in both groups. Superoxide dismutase (SOD), glutathione peroxidase(GPx) and lipid peroxide (LPO) were estimated in blood by standard techniques. RESULTS: Hemolytic uremic syndrome (HUS) was a major cause of acute renal failure (52%), rest were due to acute glomerulonephritis (AGN), septicemia and renal venous thrombosis. In the renal failure group 56% of the patients were dialyzed (peritoneal) and the mortality was 28% (7/25). The levels of SOD, GPx and LPO were significantly raised in renal failure group. Higher values of LPO, SOD and GPx were documented in subjects who expired. The most important independent variable for predicting clinical outcome was LPO with a sensitivity of 89.4%, specificity of 93%, positive predictive value of 95%. CONCLUSION: Levels of free oxygen radicals (SOD, LPO and GPx) are raised in acute renal failure and these enzymes can be used as marker of renal injury. LPO levels are highly sensitivity and specific for predicting the clinical outcome     

Evaluation of free radical status in CSF in childhood meningitis

The study was undertaken to investigate the possible role of free radicals and antioxidants in childhood meningitis. Sixty children suffering from acute bacterial meningitis (ABM) or tuberculous meningitis (TBM) according to their clinical and laboratory findings were enrolled in the study. The production of superoxide anions (O2.-), hydrogen peroxide (H2O2) and malondialdehyde (MDA) and the activities of xanthine oxidase (XO), superoxide dismutase (SOD) and glutathione peroxidase (GPx) were monitored in the study groups and findings compared with those in 20 age-matched controls. Children with ABM and TBM who died registered significant increases in the production of O2.- and MDA and in the activities of SOD and CPK compared with survivors. The rate of production of oxidants and MDA and the activities of XO, SOD and CPK were of a much higher magnitude in deceased ABM and in ABM survivors than in fatal TBM and survivors, respectively. The abnormalities in most of the biochemical parameters investigated were more marked in the children with ABM than in TBM and controls (p < 0.001). Increased MDA production and creatine phosphokinase (CPK) activity of different magnitudes in the two study groups suggest varying degrees of tissue damage. The alterations observed in 20 children who died (14 from ABM, 6 from TBM) revealed elevated levels of oxidants, antioxidants and toxicity markers, particularly in ABM patients, which suggests the possibility that natural or synthetic antioxidants might prevent disease progression and tissue damage in childhood meningitis.     

Prothrombotic factors in nephrotic syndrome

Nephrotic syndrome is a hypercoagulable state with variable prevalence of clinical thrombosis. The role of platelet aggregation, fibrinogen and antithrombin III and protein S levels in the pathogenesis of hypercoagulable state in these patients is controversial. Since no study on Indians is available, the clinical and laboratory profile of 22 patients of nephrotic syndrome (age 18-35 years with an MF ratio of 4:3), have been studied. The coagulation profile revealed a prolonged APTT in 12 patients (54.5%), and a prolonged TT in four (18.1%). In the rest APTT and TT were normal. PT was raised in two patients. Fibrinogen, an acute phase reactant was raised in five patients (22.7%). Antithrombin III levels were reduced in 19 patients (86.4%), normal in one and raised in two patients. Free Protein S levels were high in 12(54.5%), normal in seven and decreased in three patients. Platelet aggregation with adrenaline and adenosine diphosphate was raised in 6 patients. Ultrasonographically detected deep vein thrombosis was seen in one patient only (4.5%) who had ATIII levels of 48%. This low incidence can be explained by elevated protein S levels which was found to be raised in 12(54.5%) cases, protein S being an anticoagulant factor. This low level of clinical thrombosis in Indian patients of nephrotic syndrome may be an ethnic variable factor. It is thus concluded that although patients with nephrotic syndrome have a hypercoagulable state, clinical thrombosis is rarely seen in Indian patients with nephrotic syndrome.     

EARLY CARCINOMA TONGUE – SURGICAL OPTIONS

A retrospective analysis of 127 surgically treated cases of T-1, T-2 carcinoma of oral tongue during the period 1987-1990 was undertaken. 68.5 per cent (87) underwent hemiglossectomy and 31.5 per cent (40) underwent wide excision. There were loco-regional recurrences in 22 per cent (27). In the hemiglossectomy group 9 per cent (8 of 87) had local recurrences compared to 25 per cent (10 of 40) of wide excision group, (P = 0.01). Mean disease free survival was 40 months and 33 months for hemiglossectomy group and wide excision group respectively, (P = 0.006). It is seen that local recurrences are significantly less for the hemiglossectomy group compared to the wide excision group.KEY WORDS: Disease free survival, Early cancer, Recurrence, Surgery, Tongue     

Anaesthesia and the Sturge-Weber syndrome

We report a series of 13 patients with Sturge-Weber syndrome anaesthetised on 17 occasions. Anaesthesia management varied depending on the clinical manifestations which ranged from localized, superficial skin lesions to extensive systemic involvement. These patients tolerate anaesthesia well but anaesthetic management includes evaluation for associated anomalies. Difficulty with intubation may occur due to angiomas of the mouth and upper airway. Anaesthesia should be planned to avoid trauma to the haemangiomata and increases in intraocular and intracranial pressure. Nous rapportons une série d’observations concernant des porteurs du syndrome de Sturge-Weber anesthésiés à 17 occasions. L’anesthésie a varié selon les manifestations cliniques qui allaient de la lésion superficielle localisée à l’atteinte systémique grave. Ces patients tolèrent bien l’anesthésie mais celle-ci nécessite une recherche des anomalies associées pour fin d’évaluation. La présence d’angiomes de la bouche et des voies respiratoires supérieures peut rendre l’intubation difficile. La planification de l’anesthésie doit inclure la prévention du traumatisme aux hémangiomes et de l’augmentation de la tension intraoculaire et cérébrale.