Ectrodactyly: A rare anomaly of limbs.

Split-hand/split-foot malformation (SHFM) is a rare congenital malformation of the limbs with median clefts of the hands and feet and aplasia/hypoplasia of the phalanges, metacarpals and metatarsals. When present as an isolated anomaly, it is usually inherited as an autosomal dominant form. We report a case of nonsyndromic form of ectrodactyly because of its rarity.

Outcomes of Snakebite Envenomation in Children.

Introduction: Snakebite is a medical emergency, and is considered to be one of the major public health hazards in the Terai and inner-Terai regions of Nepal.Very few studies have been conducted so far in Nepal to highlight the epidemiology of snakebite in children. Aim: To review the pattern and characteristics of snakebites in children, focusing primarily on the outcomes, fatality and risk factors for death. Settings: An emergency department of a 136-bedded secondary care-referral hospital (Lumbini Zonal Hospital) situated at Butwal in the Western Nepal. Methodology: The study was carried out in 152 children aged < 15 years old, who got anti-snake venom (ASV) over a period of 48 months. Diagnosis of snakebite envenomation was based on clinical ground. Results: Children over 5 years of age constituted the highest number (87%) of cases. Peak incidence of poisoning (71%) was observed during the months of Asadh- Bhadra (June-September). Most of the bites (61%) were by unidentified snakes, most commonly (91%) on the extremities. Snakebite envenomation occurred more frequently (52%) during night time. 85% of children had local or systemic complications, commonest being respiratory paralysis(92/152).Case fatality rate (CFR) was 28%. Risk factors for death were: age < 5 years; bites by unidentified snakes and kraits and bites on ears & unknown sites. Conclusion: Compared with adults, children with snake envenomation have higher morbidity and mortality, which can be minimized by early diagnosis, appropriate treatment and close monitoring of children on ventilation for the timely management of complications. Emphasis should be given on developing a standard management protocol in children.

Clinical Profile and Sensitivity Pattern of Salmonella Serotypes in Children: A Hospital Based Study.

Introduction: Enteric fever is a systemic infection caused by the bacteria, Salmonella enterica serovar Typhi (S.typhi) and Salmonella enterica serovara Paratyphi (S. paratyphi A, B and C). Most of the burden of the disease is limited to the developing world and the disease still has the issues like wide spectrum of clinical presentation and multidrug resistance. Objectives: This study was done to analyze the clinical profile and antibiotic sensitivity pattern in the cases of culture positive enteric fever. Methods: A prospective cross-sectional study was conducted in Civil Service Hospital from February 2010 to January 2011 in the paediatric population in the age group of 2 to 14 years. Children with Salmonella species isolated in blood culture were included in the study. Results: Out of the 40 children with culture positive enteric fever, male to female ratio was 1.3:1 with common age group between 11-14 years. S typhi was isolated in 25 cases while S. paratyphi in 15 cases. Clinical features of S. typhi and S. paratyphi were indistinguishable. Both S.typhi and S. paratyphi were found to be 100% sensitive to drugs like Ceftriaxone, Cefotaxime, Cefixime and Chloramphenicol. Sensitivity to Ofloxacin was 100% in S. paratyphi and 92% in S.typhi. Similarly sensitivity of Azithromycin was 92% and 93% for S.typhi and S. paratyphi respectively. Conclusion: Salmonella serotype is still 100 % sensitive to third generation cephalosporin. Some percentage of resistance is seen with Ofloxacin in S. typhi and with Azithromycin in both S.typhi and S. paratyphi.

Two-hit hypothesis and multiple organ dysfunction syndrome.

A severe insult in the form of infection or trauma primes the host immune system so that a subsequent, relatively trivial insult produces a markedly exaggerated host immune response, which can lead to multiple organ dysfunction syndrome (MODS) and death. This forms the basis of the "two-hit hypothesis" (THH), which is being increasingly recognised as an important cause of morbidity and mortality following severe injury and sepsis, particularly in intensive care settings. Appreciation of the impact of repeated insults and pathophysiology of MODS is vital in the prevention of this serious complication. We describe a case which illustrates the concept of THH and MODS and present a review of literature on this subject.

Pre-emptive renal transplantation: optimum treatment for end-stage renal disease?

Renal transplantation (RT) prior to having dialysis or pre-emptive renal transplantation (PRT) has been controversial because of the paucity of clinical evidence to clarify the benefits and risks of PRT. The recent emergence of evidences from major transplant centres in the United Kingdom and United States have confirmed the survival advantages for both renal allografts and RT recipients. Hence an increasing number of transplant centres are adopting the practice of PRT. This article discusses the advantages and disadvantages of PRT and highlights the clinical evidences in support of PRT.

Colonic diverticular disease: current perspectives.

Diverticular disease is a common condition affecting large intestine in the western world, which, although remains asymptomatic in majority of people, can present with abdominal pain, acute diverticulitis, perforation, haemorrhage, intestinal obstruction and fistulation through neighbouring organs. Complicated colonic diverticular disease (CDD) is associated with significant morbidity and mortality. Increasing urbanisation globally with intake of diet deficient in fibres has led to CDD as a significant problem; hence awareness of CDD is paramount for its prevention and appropriate management. The purpose of this article is to provide a comprehensive review of the epidemiology, pathophysiology, clinical presentations, and current management of diverticular disease of the colon and its complications.

Factors influencing long-term outcomes following renal transplantation: a review.

Despite continuing advances in immunosuppressive and supportive therapies, the success of renal transplantation is impacted by factors present in the donor and recipient pre- and post-transplantation. The pre-transplant factors influencing the long-term graft function in the donor include source, age, sex, and HLA mismatches; and in the recipient include age, duration of dialysis and sensitisation. After transplantation, a number of events may lead to progressive deterioration of renal function and graft loss, which include delayed graft function, acute rejection, viral infections, recurrent disease, drug nephrotoxicity, non-compliance and chronic allograft nephropathy. Modulation of individual factor is mandatory to preserve satisfactory renal function in long-term. In this review, each factor is discussed in the context of current transplant practice and an up to date review of literature is presented.

Childhood Neurocysticercosis: Clinico-Radiological Profile and Outcome.

Abstract: Neurocysticercosis (NCC) is a major cause of neurological illness worldwide. It is the most common identifiable cause of partial seizure especially in the children of developing world. There is insufficient information about NCC in children in Nepal. This study was, therefore, conducted to evaluate the clinical, neuro-radiographic and therapeutic aspects of NCC in children. Material and Methods: 68 children with this neurocysticercosis were studied prospectively in 20 months in the Lumbini Zonal Hospital (LZH), a secondary-level-referral hospital in the Western Nepal. The diagnosis of NCC was based primarily on the neuro-imaging (CT/MRI) findings. Results: The patients were predominantly females (nearly 65%) with age ranging from 2 to 14 years. Preschool-age children constituted 10% of the patients. The three common manifestations were seizures (91%), headache and or vomiting (38%) and hemi and or monoparesis (15%). CT/MRI demonstrated a single parenchymal ring or nodular enhancing lesion (REL) in 84% of cases with perilesional oedema in nearly 90% of cases. A large majority of patients were treated only with the anticonvulsant drugs (ACDs) for 9 months. Follow-up with repeat CT after 6 months showed a complete resolution of NCC in most of the cases without the need for cysticidal treatment. Conclusion: NCC should be considered first in the differential diagnosis of new-onset seizure among the children of developing countries, where taeniasis is endemic. Most of the patients with NC do not need anticysticercal therapy.

Patient information leaflets in surgical practice: a contemporary review.

Undergoing any surgical procedure, however minor this may be, generates a certain degree of anxiety amongst the patients, which is primarily related to the possible postoperative course and complications. Introduction of Patient Information Leaflets (PIL) in routine surgical practice has helped ameliorate this problem significantly and therefore has become a standard practice in the United Kingdom and elsewhere. This review highlights the evidence available in support of the use of PIL and outlines the process of development of PIL in surgical practice.

Behcet's Disease : report of a case and review of the literature.

Behcet's disease (BD) is an autoimmune disease of unknown aetiology with multisystem involvement presenting with relapsing orogenital ulcers and uveitis, involvement of central nervous system, joints, lungs, gastrointestinal tract and major blood vessels leading to myriads of presentations, which requires a multidisciplinary approach for satisfactory outcome. We describe a patient with BD, highlight its pathophysiology and management aspects, and present review of pertinent literature.

Helicobacter pylori infection, glandular atrophy, intestinal metaplasia and topography of chronic active gastritis in the Nepalese and Japanese population: the age, gender and endoscopic diagnosis matched study.

BACKGROUND: The incidence and mortality from gastric cancer is high in Japanese but extremely low in Thailand. It is different among Asian countries. The aim of this study is to investigate the difference of peptic ulcer disease, glandular atrophy, intestinal metaplasia and topography of chronic active gastritis between the Nepalese and Japanese population. MATERIALS AND METHODS: Nepalese patients were paired with Japanese patients by age, gender and endoscopic diagnosis in order to compare the prevalence of H. pylori infection (N=309) and the difference of H. pylori related peptic ulcer disease (N=48). Glandular atrophy and intestinal metaplasia scores were also compared between the Nepalese and Japanese population in H. pylori positive cases (N=152) and negative cases (N=145) using paired cases by age, gender and endoscopic diagnosis. Paired H. pylori-positive Nepalese and Japanese population were also used to compare the ratio of corpus gastritis to antrum gastritis (C/A ratio) (N=152). RESULTS: Among peptic ulcer diseases, gastric ulcer was frequent in Japanese and duodenal ulcer was frequent in Nepalese. The prevalence of H. pylori infection in the Nepalese and Japanese population were similar. Glandular atrophy and intestinal metaplasia scores in the H. pylori positive Japanese were significantly higher than those of Nepalese in all positions according to triple site biopsy. Furthermore, there were significant differences in glandular atrophy and intestinal metaplasia scores between in the H. pylori-negative Nepalese and Japanese population except intestinal metaplasia score in the greater curvature of the upper corpus. Japanese C/A ratio was significantly higher than that of Nepalese. Corpus predominant gastritis (C/A ratio>1.00) was characteristic in the elderly Japanese. Nepalese was antrum predominant (C/A ratio<1.00) in every age group. CONCLUSIONS: Gastric ulcer was a common disease in Japanese, in contrast duodenal ulcer was common in Nepalese. H. pylori infected Japanese patients showed severe atrophic and metaplastic gastritis in comparison with Nepalese. These results may be associated with the high incidence of gastric cancer in Japanese. Corpus predominant gastritis was found in the elderly Japanese and antrum predominant gastritis was found in every age Nepalese.

Simultaneous pancreas and kidney transplantation for end-stage renal failure secondary to diabetic nephropathy : principles and practice.

Diabetic nephropathy is the second most common cause of renal failure in the United Kingdom where majority of the patients were managed by renal transplantation and insulin therapy in the past. Over the last three decades, increasing number of patients are undergoing simultaneous pancreas and kidney transplantation (SPKT) because of its advantages, which renders the patient both dialysis and insulin-independent, halts the progression of complications of diabetes, thereby improves the quality of life, survival and has proven to be cost-effective. This article presents a review on the principles and contemporary practice of SPKT worldwide and highlights the future directions.

Vacuum-assisted closure (VAC) therapy in the management of wound infection following renal transplantation.

OBJECTIVES: Wound infection in the setting of immunosuppressed state such as renal transplantation (RT) causes significant morbidity from sepsis, prolongs hospital stay and is expensive. Vacuum-assisted closure (VAC) therapy is a new technique of management of wound based on the principle of application of controlled negative pressure. The aim of this study was to assess the efficacy of VAC therapy in the management of wound infection following RT. MATERIALS AND METHODS: This is a prospective study of a cohort of 180 consecutive RTs performed over a period of 4 years, where the data were retrieved from a prospectively maintained computerised database and case-notes. RESULTS: 9 of 180 (5%) patients developed wound infection following RT which led to cavitations and dehiscence with copious discharge, and refused to heal with conventional treatment. All 9 cases were treated with VAC therapy. The VAC system was removed after a median of 9 (range 3-30) days when discharge from the wound ceased. Four patients were discharged home with portable VAC device and managed on an outpatient basis, where the system was removed after a median 5.5 (range 3-7) days. The median hospital stay after initiation of VAC therapy was significantly shorter (5, range 2-12 days) than on conventional treatment prior to VAC therapy (11, range, 5-20 days) (p=0.003). Complete healing was achieved in all cases. CONCLUSIONS: The use of VAC therapy is an effective and safe adjunct to conventional and established treatment modalities for the management of wound infection and dehiscence following RT. Key words: Renal transplantation, wound infection, vacuum-assisted closure therapy.

Ureteric complications following renal transplantation: an eight years experience.

OBJECTIVES: Ureteric complications (UCs) following renal transplantation (RT) cause significant morbidity and ureteric stents are employed to bridge the vesico-ureteric anastomosis with a view to preventing these complications. The purpose of this study was to examine the incidence of UCs and outcomes following RT in both stented (STG) and non-stented groups (NSTG) of RT patients. METHODS: This is a retrospective study of a cohort of 650 consecutive RTs [STG (N=267; 41%) and NSTG (N=383; 59%)] performed over a period of 8 years, where the data were retrieved from a prospectively maintained computerised database and case-notes. RESULTS: The overall incidence of UCs was 6.5% (42/650), which consisted of ureteric obstruction (UO) in 4.3% (28) and ureteric leak (UL) in 2.2%(14) of patients. The incidence of UO was significantly high in the NSTG compared to the STG (6.3% vs.1.5%; P=0.002). However, the incidence of UL (3.4% vs.1.3%; P=0.1) and post-transplant urinary tract infection (UTI) (44% vs.41%; P=0.57) were not significantly different between the STG and NSTG groups. UO and UL were associated with significantly high incidence of UTI (P=0.001 and 0.01, respectively). All UCs were managed successfully without allograft loss. CONCLUSIONS: Routine stenting of ureteric anastomosis resulted in reduced incidence of UO without concomitant increased risk of UTI.

A comparison of 25 gauge Quincke spinal needle with 26 gauge Eldor spinal needle for the elective Caesarian sections: insertion characteristics and complications.

OBJECTIVE: The study was designed to compare the insertion characteristics and incidence of PDPH between 25 gauge Quincke needle and 26 gauge Eldor needle for spinal anaesthesia in elective c/s. METHOD: 60 pregnant women (aged 19-35 yrs and weighing 58 -67 kg) undergoing elective caesarean section were randomized into group A (Quincke spinal needle group) or group B (Eldor spinal needle group). Spinal anaesthesia was performed with 2.9 ml 0.5% heavy bupivacaine using 25 gauge Quincke spinal needle in group A and 26 Gauge Eldor spinal needle in group B. Onset, time of first identification of backflow of CSF, number of attempts, level of sensory and motor blockade, failure of anaesthesia, inadequate anaesthesia and incidence of PDPH were recorded. RESULT: Quincke spinal needle was found easy at insertion, first attempt was successful in 90% of cases, whereas Eldor spinal needle was successful at first attempt in only 60% of cases. Early identification of CSF was seen in Eldor spinal needle group in 3.5 seconds vs. 5.2 seconds in Quincke spinal needle group. Blood mixed CSF was seen in 8 Quincke spinal needle group vs. none in Eldor spinal needle group. Onset was similar between both groups i.e. in 6 minutes. Failure of anaesthesia was none in Eldor spinal needle group vs. 2 in quincke spinal needle group. Height of sensory block achieved was T4 level in 26 parturients,T6 in 1 ,T8 in 1 and no anaesthesia at all in another 2 parturient as compared to T4 level in 29 and T3 in 1 parturient in Eldor spinal needle group. The degree of motor block with the use of Bromage criteria showed a motor score of 1 or 2 in 26 parturients in Quincke spinal needle group vs. same in all cases in Eldor spinal needle group. The total incidence of PDPH was 8.3 % (5 out of 60 parturient) which occurred all in Quincke spinal needle group. 2 parturient who developed severe PDPH required epidural blood patch. CONCLUSION: 26 gauge Eldor spinal needle was found to be better than 25 gauge Quincke spinal needle for caesarian sections to decrease the incidence of PDPH, though not all insertion characteristics were in favour of the Eldor needle.