Vitamin D deficiency masquerading as pseudohypoparathyoidism type 2.
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Biomedical subjects
Publications and source records attributed to R Aggarwal.
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BACKGROUND/AIM: Laparoscopic Heller cardiomyotomy is an established modality in the surgical treatment of patients having achalasia cardia. We present our initial experience in robot-assisted laparoscopic Heller cardiomyotomy without addition of an antireflux procedure and discuss the relative merits and disadvantages of the procedure. PATIENTS AND METHODS: Five patients underwent robot-assisted laparoscopic Heller cardiomyotomy between August 2001 and October 2002. The diagnosis had been confirmed by radiology and manometry in all patients prior to surgery. RESULTS: All procedures were completed successfully using the robotic system, without conversion to open procedure. Mucosal perforation occurred in 1 patient and was sutured robotically. The average operative time was 114.8 (range 65-160) min which is comparable to laparoscopic procedures. After a mean follow-up period of 9.4 (range 3-17) months, 4 patients remained completely asymptomatic, and 1 patient has benefited from symptomatic improvement. CONCLUSIONS: The enhanced dexterity and the high-quality three-dimensional vision available with robot-assisted surgery make the application of this technology highly suitable for Heller cardiomyotomy. The minimal lateral and posterior dissection due to the wristed instruments avoids the need for an antireflux procedure.
BACKGROUND: The introduction of laparoscopic techniques to general surgery was associated with many unnecessary complications, which led to the development of skills laboratories to train novice laparoscopic surgeons. This article reviews the tools currently available for training and assessment in laparoscopic surgery. METHODS: Medline searches were performed to identify articles with combinations of the following key words: laparoscopy, training, curriculum, virtual reality and assessment. Further articles were obtained by manually searching the reference lists of identified papers. RESULTS: Current training involves the use of box trainers with either innate models or animal tissues; it lacks objective assessment of skill acquisition. Virtual reality simulators have the ability to teach laparoscopic psychomotor skills, and objective assessment is now possible using dexterity-based and video analysis systems. CONCLUSION: The tools are now available for the development of a structured, competency-based, laparoscopic surgical training programme.
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AIM: To assess the effectiveness of expressed breast milk (EBM) in reducing pain due to venepuncture, in term neonates, as measured by behavioural and physiological observations. METHODS: This randomized, placebo-controlled, double-blind trial involved 81 full-term neonates, up to 4 wk of postnatal age, who needed venepuncture for blood investigations. Two minutes before the venepuncture, in the intervention arm, 40 babies received 5 ml of EBM, while 41 babies in control group received 5 ml of distilled water (DW) as placebo. Two observers who were blinded to the intervention recorded the physiological (heart rate and oxygen saturation) and behavioural parameters [duration of crying and modified Neonatal Facial Coding Scores (NFCS)] after the venepuncture. RESULTS: There was no difference in the baseline characteristics of the neonates in the two groups. The duration of crying was significantly shorter in babies fed EBM [median 38.5 s, interquartile range (IQR) 9.5-57.5 s] than in those fed DW (median 90 s, IQR 28-210 s). The mean duration of crying in EBM group was shorter by 70.7 (95% confidence interval 36.6-104.9) s. The modified NFCS at 0, 1 and 3 min was significantly lower (p < 0.01) in the EBM than in the DW group. The change in heart rate and oxygen saturation was significantly lower in the EBM group and returned to baseline values sooner than in the DW group. CONCLUSION: Feeding 5 ml of EBM before venepuncture is effective in reducing symptoms due to pain in term neonates.
The mainstay of treatment of chronic rhinosinusitis is through the administration of topical nasal drugs. The delivery and intranasal distribution of these is therefore of potential clinical significance. Until there is progress in the nasal drug distribution assessment methodology, it will be difficult to improve topical nasal drug delivery, which is known to be suboptimal in many ways. This study reviews intranasal drug delivery assessment methods, the present knowledge and explores future directions for research.
OBJECTIVES: To audit the performance of hospitals in evidence-based prescribing. SETTING: All hospitals in England were invited to participate. The audit was completed in 62 hospitals. SUBJECTS: Prescribing and clinical data were collected on 100 consecutive medical inpatients aged >/= 65 years at each site, enabling evaluation of eight prescribing indicators before and after intervention. The data were collected using a specifically designed database. INTERVENTIONS: The results of the first audit were available immediately from the software and a national report with locally identifiable information was returned to hospitals. Hospitals were encouraged to design and deliver their own intervention strategy. A questionnaire was sent to all hospitals to document prioritization of indicators. RESULTS: Generic names were used for 36 061 (82.6%) in 1999 and 39 188 (86.4)% in 2000. In 1999, 50% (3074) of patients had documentation of allergy status. This increased to 60% (3684) in 2000. For 21.2% of patients prescribed paracetamol in 1999 and 18.1% in 2000, the prescription was written such that it was possible to exceed the maximum recommended dose of 4 g in 24 hours. Long-acting hypoglycaemic drugs were prescribed to 29 patients in 1999 and 20 patients in 2000. Anti-thrombotics were used appropriately for 54% (520/966) of patients in atrial fibrillation in the first audit and 57% (579/1019) in the second audit. The appropriate use of aspirin increased from 91% (595/651) to 94% (725/772) and the appropriate use of benzodiazepines dropped from 49% (537/1088) to 47% (460/966) between the audits. For three indicators, the allocating of a high priority translated into a bigger improvement between the audits. CONCLUSIONS: Local ownership of data and the quality improvement process, and provision of national benchmarking data did not result in a significant improvement in prescribing in the second audit.
Motility abnormalities, common in gastroesophageal reflux disease, are likely to be related to endoscopic esophagitis. We studied pH and manometry parameters in relation to the severity of esophagitis. Forty-seven patients with symptomatic gastroesophageal reflux disease for > 3 months were evaluated by: (i) endoscopy (grading of esophagitis by Savary-Miller classification); (ii) mucosal biopsy; (iii) manometry; and (iv) 24-h pH-metry. We found Savary-Miller's grades of: 0 (9 patients out of 47), I (16/47), II (16/47), III (4/47), IV (2/47). Distal esophageal contraction amplitude was lower in severe (grade II to IV) as compared with mild (grade 0 and I) esophagitis (49 [7-182] versus 83 [27-196] mmHg [P = 0.001]). The length and pressure in the lower esophageal sphincter (LES), duration and velocity of contraction in the body, number of episodes of reflux and long-duration reflux, longest reflux, median pH, per cent of time with pH < 4 and DeMeester scores were not significantly different between the two groups. The area under pH 4 showed a negative correlation with LES pressure and amplitude of distal esophageal contractions. We conclude that higher endoscopic grades of esophagitis are associated with lower amplitude of contraction in distal esophagus. Lower LES pressure and distal esophageal contraction amplitude are associated with greater area under curve for pH below 4.
BACKGROUND: Though most patients with achalasia cardia (AC) respond to pneumatic dilation (PD), one-third experienced recurrence. Long-term follow-up studies on factors associated with various outcomes are scanty. METHODS: In this retrospective study, 126 patients (36.5 +/- 14.6 yr, 76 male) with AC (diagnosed by esophagoscopy, barium esophagogram, and/or manometry) were followed up in person or through mail. The median dysphagia-free duration was calculated by Kaplan-Meier analysis. Factors associated with nonresponse and recurrence after PD were determined using univariate and multivariate analyses. RESULTS: Symptoms were dysphagia (126, 100%), chest pain (21, 17%), regurgitation (61, 48%), weight loss (33, 26%), and pulmonary symptoms (23, 18%); 5 of 126 (4%) had megaesophagus (> or =7 cm). The mean lower esophageal sphincter (LES) pressure was 38.7 +/- 16.8 mmHg. One hundred and fifteen of 126 (91%) patients responded to PD (90 (71%) to first session); 25 of these had recurrence of dysphagia after 15 +/- 17 months. Post-PD chest pain requiring hospitalization occurred in 21 of 126 (17%; one had an esophageal perforation). Post-PD LES pressure, which was assessed in 48 of 126 patients, had decreased by >50% from baseline in 14 of 29 responders, 0 of 11 nonresponders (p= 0.004, chi(2) test), and 5 of 8 relapsers. The median dysphagia-free duration by Kaplan-Meier analysis was 60 months (SE 2.7, 95% CI 54.7-65.3). On univariate analysis, male gender, pulmonary symptoms (nocturnal coughing spell, history of respiratory infection), absence of chest pain, and failure to achieve a reduction in LES pressure >50% after PD were associated with poor outcome; whereas age, grade of dysphagia, regurgitation, megaesophagus, and LES pressure before PD were not. Male gender was associated with poor outcome by multivariate-analysis. CONCLUSIONS: PD is an effective and safe treatment for AC. Post-PD LES pressure measurement may be helpful in assessing response. Male patients have poorer outcomes following PD.
Surgical excellence is traditionally defined in terms of technical performance, with little regard for the importance of interpersonal communication and leadership skills. Studies in the aviation industry have stressed the role of human factors in causing error and, in an attempt to reduce the occurrence of adverse events, led to the organisation of simulation based training scenarios. Similar strategies have recently been employed for the surgical team with the development of a simulated operating theatre project. This enables technical and non-technical performance of the surgeon and circulating staff to be assessed by experts situated in an adjacent control room, and provides an opportunity for constructive feedback. The scenarios have good face validity and junior surgeons can benefit from the process of learning new technical skills in a realistic environment. The effect of external influences such as distractions, new technology, or a crisis scenario can also be defined, with the ultimate aim of reducing the number of adverse events arising in the real operating room.
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Intrafamilial transmission is rare in epidemic hepatitis E; its frequency in sporadic hepatitis E is not known. We followed up 86 household contacts (age range 4-75 years, mean +/- SD 32.4 +/- 15.8; 49 males), who were family members of patients with acute sporadic hepatitis E. Of the 86 contacts, 68 (79%) tested negative for IgG anti-hepatitis E virus antibodies. Four (4.7%) had IgM anti-hepatitis E virus antibodies at the time of diagnosis of hepatitis E in the index case; two of these contacts possibly had hepatitis E virus infection acquired simultaneously with that in the index case, and two could have had intrafamilial transmission. None developed serological evidence of hepatitis E virus infection over a period of 49 +/- 18 days after the diagnosis of index case, although a majority lacked IgG antibodies to hepatitis E virus and were likely to be susceptible. This suggests that person-to-person transmission is uncommon in sporadic hepatitis E.
BACKGROUND AND OBJECTIVES: Hepatitis B virus (HBV) may occasionally be transmitted through transfusion of blood units that are hepatitis B surface antigen (HBsAg) negative but HBV DNA positive. Children with beta-thalassemia are particularly susceptible to HBV because they receive multiple blood transfusions. These children have high infection rates despite vaccination against HBV. Post-vaccination infections may be a result of viruses harbouring surface (S)-gene mutations (e.g. G587A) in a region critical for reactivity to antibody to hepatitis B surface antigen (anti-HBs). The true prevalence of HBV in individuals with beta-thalassemia has not been studied previously. PATIENTS AND METHODS: Seventy patients with beta-thalassemia (median age 6 years; range 8 months to 22 years; 49 male), who had received seven to 623 (median 61) units of blood each and three doses (10/20 micro g) of HBV vaccine (Engerix B) before presentation to us, were included in the study; 50 of the 70 patients had received transfusions prior to vaccination. Enzyme-linked immunoassay for serological markers [HBsAg, antibody to hepatitis B core antigen (anti-HBc) and quantitative anti-HBs] and polymerase chain reaction (PCR) followed by Southern hybridization for molecular detection of hepatitis B, was performed on all samples. The PCR-amplified product was cloned, sequenced and the nucleotide and deduced amino acid sequences for the HBV S and polymerase (P) genes were analysed for mutations. RESULTS: Four of 70 (5.7%) individuals with beta-thalassemia were HBsAg positive and 14 (20%) were anti-HBc positive. The prevalence of serological markers increased with number of transfusions (P < 0.01). Of 70 patients, 53 (75.7%) had an anti-HBs titre of > 10 IU/l following vaccination and 17 (24.3%) were non-responders (< 10 IU/l); 22 (31.4%) of the 70 were DNA positive. The frequency of HBV infection in beta-thalassemia was similar in vaccine responders and non-responders. The virus was of subtype ayw (genotype D) in the five DNA-positive samples in which a 388-nucleotide region of the S gene was sequenced. Mutations occurred at 13 positions in the S gene and at 10 positions in the P gene. Hydrophobicity plots revealed differences in amino acid regions 117-165 and 195-211. Some of these amino acid substitutions coincided with the putative cytotoxic T-lymphocyte epitopes of both S and P proteins. CONCLUSIONS: A high frequency of HBV infection was seen using molecular methods in thalassemic patients. The frequency of infection was similar in vaccine responders and non-responders. A number of mutations were observed in the S gene, which could have implications for viral replication as well as virus-host cell interaction.
BACKGROUND AND OBJECTIVES: In developing countries, caring for the large number of babies in orphanages is very hard work. Whereas the physical needs of most of the children are met, play often gets neglected. Studies have repeatedly shown that babies in such institutionalized settings suffer from severe psychomotor retardation. The aim of this project was to develop an intervention programme of structured play. We hypothesized that such an intervention would result in acceleration of psychosocial development in otherwise healthy institutionalized children. DESIGN: Prospective longitudinal. SETTING: Mother Teresa's Orphanage, run by Missionaries of Charity. SUBJECT AND METHODS: All 30 children in the orphanage aged 6 months-2.5 years, were assessed for their Motor, Mental and Social Quotients, using the Indian adaptation of Bailey's Scale of Infant Development(DASII) and the Vineland's Social Maturity Scale. A structured 'Regime of Play' was then built into the routine of the orphanage. A repeat developmental assessment was performed at the end of 3 months to assess the impact. RESULTS: Out of the original cohort of 30, 19 children were available for post-intervention assessments. The remainder were adopted before their assessments. Their mean Motor Quotient rose from 63.7 to 81.7, mean Mental Quotient rose from 65.8 to 89.6 and the mean Social Quotient rose from 61.9 to 91.3, a gain of 18, 23 and 30 points respectively (p < 0.0001). There was also an overall change in the environment of the orphanage. Children became more active, playful, responsive and independent. Contrary to what caretakers assumed, their workload actually decreased. The responsiveness in the children awakened as a result of play, acted as a positive feedback for caretakers to continue the play sessions. CONCLUSIONS: This study shows that short daily sessions of play can significantly improve the development of children in such institutions. It is vital to remember that children grow 'Not by Bread Alone'.
RATIONALE, AIMS AND OBJECTIVES: This national clinical audit aimed to develop and implement a methodology to assess the appropriateness of prescribing for patients over the age of 65 in hospitals, general practice and nursing homes. METHODS: Organizations providing health care in the National Health Service in these three sectors were recruited into multi-disciplinary and inter-organizational local coalition teams. Prescription data and relevant clinical data were collected electronically on a customized database. The appropriateness of prescribing for specific conditions among the patients sampled was assessed by simple computerized algorithms, and users were provided with feedback to stimulate discussion and change. Use of the software tool was demonstrated to be feasible and its data reliable. Participants were re-audited, after a period of nationally guided and locally driven intervention, to evaluate levels of change. Local efforts to stimulate change and barriers to change were collected qualitatively. RESULTS AND CONCLUSIONS: The investigation revealed encouraging results and demonstrated the ability of audit to improve the quality of clinical services in given circumstances, although a multiplicity of questions relating to cost and methodology remain to be addressed.
Serial subclinical transmission among susceptible humans may serve as a reservoir of hepatitis E virus (HEV) in areas in which HEV is endemic. This hypothesis was investigated in an experimental primate model. Four groups of 4 cynomolgus macaques each were inoculated intravenously with 10(4)-10(5) (group 1), 10-100 (group 2), and 1-10 (group 3) cynomolgus macaque HEV infectious doses. All 4 animals in group 1 had clinical disease marked by alanine aminotransferase (ALT) elevation, fecal virus excretion, viremia, and seroconversion. Of the animals in groups 2 and 3, only 1 had evidence of biochemical hepatitis, although most had virus excretion and viremia (3 animals each in groups 2 and 3), and evidence of seroconversion (1 animal in group 2 and 3 animals in group 3). Viral genomic titers in stool specimens of animals with or without ALT elevation were similar. Infectivity studies confirmed the viability and transmission potential of the virus excreted by animals without ALT elevation. These data suggest that subclinical HEV infection may represent an HEV reservoir.
Apnea, defined as cessation of breathing resulting in pathological changes in heart rate and oxygen saturation, is a common occurrence in sick neonates. Apnea is a common manifestation of various etiologies in sick neonates. In preterm children it may be related to the immaturity of the central nervous system. Secondary causes of apnea should be excluded before a diagnosis of apnea of prematurity is made. Methylaxanthines and Continuous Positive Airway Pressure form the mainstay of treatment of apnea in neonates. Mechanical ventilation is reserved for apnea resistant to above therapy. An approach to the management of apnea in neonates has been described.
Since a universal definition for hypoglycemia is lacking, an operational threshold for initiating therapy has been defined. Hypoglycemia is encountered in a variety of neonatal conditions including prematurity, growth retardation and maternal diabetes. Since hypoglycemia may be asymptomatic, routine screening for this condition in certain high risk situations is recommended. Supervised breast-feeding may be a treatment option in asymptomatic hypoglycemia. However, symptomatic hypoglycemia should always be treated with a continuous infusion of parenteral dextrose. Neonates needing dextrose infusion rates above 12 mg/kg/m should be investigated for refractory causes of hypoglycemia. Hypoglycemia has been linked to poor neuro-developmental outcome and hence aggressive screening and treatment is recommended.