Eosinophilic fascitis: a rare fibrosing disorder.

Eosinophilic fasciitis is a rare systemic disease characterized by inflammatory thickening of the skin and underlying fascia, peripheral eosinophilia, elevated inflammatory markers and typical biopsy findings. Internal organ involvement is rare unlike in other fibrosing disorders. Though this is usually a benign disease, it is often difficult to diagnose and the course may also be variable. Glucocorticoids are the mainstay of therapy, but in many cases steroid sparing immunosuppressive drugs are required particularly if the diseases has protracted course. The author reports a typical case of this rare disorder which could well be a first report from Nepal.

Clinical profile of patients with systemic lupus erythematosus.

INTRODUCTION: Clinical features of systemic lupus erythematosus vary between different parts of the World; however, this information on Nepalese systemic lupus erythematosus does not exist. METHODS: Patients with systemic lupus erythematosus fulfilling the American College of Rheumatology criteria treated in Patan Hospital were studied by means of retrospective review of their case records. The results were compared with various regional and international studies. RESULTS: The female to male ratio was 36:1 with a mean age at diagnosis 28.67 years. Cutaneous manifestations were the most common 72 (96%) presentations followed by musculoskeletal involvement 70 (93%). Haematological and renal involvements each were present in 33 (44%) of patients. Pulmonary and neuropsychiatric involvements were present in 15 (20%) and 8 (11%) patients respectively. Antinuclear antibody and anti-double stranded deoxyribonucleic acid antibodies were positive in 70 (93%) and 66 (88%) of patients respectively. A quarter of patients had some form of co-morbid conditions. At the time of last follow up, 70 (93%), 66 (90%), and 6 (9%) of patients were taking antimalarials, steroids and immunosuppressive agents respectively, mostly in combinations. Eighty seven percent of patients had their disease activity under control at the time of last follow up whereas four percent still had an active disease. In another four percent died during the course of therapy and the outcome was unknown in another four percent of patients who lost to follow up. CONCLUSIONS: Clinical characteristics and antinuclear antibody pattern of Nepalese lupus patients is more or less similar to those with patients from other countries in the region and in the World.

Behçet's disease: an account of three cases.

Behçet's disease is a chronic relapsing inflammatory condition affecting multiple organ-systems of the body. Patient's with this disease present to different specialists with wide spectrum of clinical features of varying severity. Worldwide, this disease is clustered around certain parts of Asia, previously known as 'Silk route'. Here we present three cases of Behçet's disease who presented to our clinic with ocular, dermatological, and articular manifestations.

The influence of ethnic origin on the skin photoageing: Nepalese study.

Ethnic and genetic differences modify skin structure and function. Skin photoageing is becoming one of the most studied cosmetological topics. However, there are relatively few data available to evaluate the effect of ethnic skin origin on the degree of photoageing, particularly among people inhabiting sunny and hot climate as in South Asia. Two hundred and forty participants, enrolled in the study conducted in Pokhara valley, Nepal, were classified into four different age categories viz. below 30, 30-50, 50-60 and above 60 years. Participants answered the questioner regarding their age, gender, ethnic origin, occupation, skin characteristics, lifestyle factors, type of cosmetic and skin care products used, drug treatment and personal skin condition evaluation. Skin hyperpigmentation and the degree of wrinkling, the two most important parameters related to photoageing, were particularly evaluated in two ethnic groups, namely Aryan-origin and Mongolian-origin participants. The study showed that Aryan-origin ethnic skin was more wrinkled and darker than that of Mongolian. Male skin was found to be darker than the female skin, which might be the result of the use of sun-protective cosmetic products among women. Smoking was found to increase the degree of wrinkling; however, no differences related to ethnic origin in relation to smoking were observed. This study indicates the need to further evaluate the differences in skin response to daily exposure to strong sun among various ethnic-origin inhabitants.

Lemierre's syndrome in a patient with severe lupus nephritis.

Lemierre's syndrome is a rare septic thrombophlebitis of the internal jugular vein with frequent metastatic complications following an acute oropharyngeal infection. Immunocompromised patients are at higher risk of developing this syndrome owing to the decreased host response and increased risk of oropharyngeal sepsis. We herein report a 24-year-old woman with severe lupus nephritis on immunosuppressive therapy, who developed Lemierre's syndrome following a pharyngeal infection despite an adequate and timely antibiotic therapy. We hereby draw an attention to the importance of accurate and timely diagnosis and appropriate management of Lemierre's syndrome in immunocompromised patients so as to achieve a successful prognosis for this deadly infection.

Prevalence of dental caries in 5 - 6 years and 12 - 13 years age group of school children of Kathmandu valley.

INTRODUCTION: Dental caries is one of the most common conditions affecting the general health of children. The present study was carried out among school children of Kathmandu valley to determine the prevalence of dental caries in two age groups. METHODS: The study was conducted from December 2007 to May 2008. The age of the school children of the study was divided into two group: 5 - 6 years and 12 - 13 years. A stratified cluster sampling with proportional allocation was used while grouping the subjects. The dental status examination was done with the help of trained dentists. Decayed, missed and filled teeth index and decayed, missed and filled surfaces index (dmft for primary dentition and DMFT for permanent dentition) were used as the standard tools for the determination of prevalence. RESULTS: A total of 638 students (325 of age group 12 - 13 years and 313 of age group 5 - 6 years) from 30 different schools of the Kathmandu valley were included in the study. The caries status was found higher in the age group of 5 - 6 years than in the 12 - 13 years and it was found to be statistically significant (p < 0.001). The dmfs and caries percentage of the age group 5 - 6 years and the DMFS and caries percent of the 12 - 13 years was found to be 3.79, 69 % and 1.6, 53.23 % respectively. The dmft/dmfs value was found to be significant according to the districts in the 5 - 6 years age group whereas the DMFS was found statistically significant among the sexes of the 12 - 13 years age group. CONCLUSION: The caries percentage was found to be above the recommended level of the World Health Organization. However, the DMFS and DMFT values were within the WHO level.

A diagnosis missed for several years-Wegener's granulomatosis.

Wegener's granulomatosis is a form of systemic vasculitis of small to medium sized vessels and affects upper respiratory tract, lungs and kidneys along with various organs. It causes necrotizing granulomatous inflammation of the affected parts and presents with positive antineutrophil cytoplasmic antibodies in more severe forms. Being a systemic disease with the potential to affect any organ-systems with a wide range of clinical presentations, it is associated with a risk of delay in diagnosis with resultant setback in institution of appropriate treatment. Confusion may arise due to an extent of histological similarity between Wegener's granulomatosis and the more prevalent tuberculosis, both causing granulomatous inflammation of the affected parts. Here, we present two cases of this rare disorder where the diagnosis was missed for several years in the beginning causing a delay in institution of specific therapy which led to the development of complications.

Bone and joint decade 2000-2010 in Nepalese perspective.

The Bone and Joint Decade is a worldwide multi-disciplinary initiative targeting the care of people with musculoskeletal disorders. It focuses on improving the quality of life of these people through advances in the understanding and treatment through education, research, and prevention strategies. Though the epidemiological data in Nepal are very scanty, worldwide statistics have shown that musculoskeletal conditions represent more than half of all chronic diseases, and are the most common cause of severe long-term pain and disability. There is acute shortage of specialist manpower that care for people with these disorders. Furthermore the teaching in musculoskeletal disorders in health institutions is very minimal. All of these problems highlight the need for expanding the teaching of musculoskeletal disorders at all levels of medical education; promotion of clinical research; improving diagnostic, treatment, and rehabilitative facilities; and implementation of preventive strategies to reduce the burden of these chronic debilitating conditions in the society.

Antiphospholipid syndrome in childhood systemic lupus erythematosus.

Antiphospholipid syndrome is an autoimmune disease that presents with recurrent arteriovenous thrombosis, repeated pregnancy loss and elevated titres of antiphospholipid antibodies in the blood. It is a common cause of acquired thrombosis and can manifest within any part of the vascular tree. Inferior Venacava thrombosis at outset, however, is not a common manifestation of systemic lupus erythematosus associated-antiphospholipid syndrome particularly in children. Here, we present a 14-year old girl who developed antiphospholipid syndrome as a presenting manifestation of systemic lupus erythematosus.

The role of the ADC value in the characterisation of renal carcinoma by diffusion-weighted MRI.

The purpose of this study is to evaluate the role of diffusion-weighted imaging (DWI) in combination with T(1) and T(2) weighted MRI for the characterisation of renal carcinoma. The institutional review board approved the study protocols and waived informed consent from all of the patients. 47 patients (32 male and 15 female; age range, 21-85 years; median age, 65 years) who had suspected renal lesions on abdominal CT underwent MRI for further evaluation and characterisation of the lesions from April 2005 to August 2007 in our university hospital. A region of interest was drawn around the tumour area on apparent diffusion coefficient (ADC) maps. Final diagnosis was confirmed by histological examination of surgical specimens from all patients. The ADC value was significantly higher in renal cell carcinoma (RCC) than in transitional cell carcinoma (2.71+/-2.35 x 10(-3) mm(2) s(-1) vs 1.61+/-0.80 x 10(-3) mm(2) s(-1); p = 0.022). While analysing the histological subtypes of RCC, a significant difference in ADC values between clear cell carcinoma and non-clear cell carcinoma was found (1.59+/-0.55 x 10(-3) mm(2) s(-1) vs 6.72+/-1.85 x 10(-3) mm(2) s(-1); p = 0.0004). Similarly, ADC values of RCC revealed a significant difference between positive and negative metastatic lesions (1.06+/-0.38 x 10(-3) mm(2) s(-1) vs 3.02+/-2.44 x 10(-3) mm(2) s(-1); p = 0.0004), whereas intensity on T(1) and T(2) weighted imaging did not reach statistical significance. In conclusion, DWI has clinical value in the characterisation of renal carcinomas and could be applied in clinical practice for their management.

A profile of low vision among the blind school students in Lumbini Zone of Nepal.

INTRODUCTION: There is an increasing awareness about the needs of students with low vision, particularly in developing countries where programs of integrated education are being developed. However, the appropriate low vision services are usually neither available, nor affordable. OBJECTIVES: To study the profile of students with low vision in Lumbini Zone of Nepal. MATERIALS AND METHODS: A cross-sectional study included students with low vision from 8 integrated schools. The reading performance was measured using their own text-books with optical low vision devices prescribed for near reading. The reading rates were measured on a range of print sizes. The Standard Clinical Low Vision Assessment Form and the National Low Vision Program protocol were used. SPSS software was used in data analysis. RESULTS: Of 46 students enrolled, 23 had a mild visual impairment, 18 had severe visual impairment and 5 were blind. Spectacles were needed for 29 (63 %) students. Of the 11 students who had been wearing glasses, the visual acuity improved in 4 with a change in prescription. 91 % of the students had near vision better or equal to 1.5 M with optical low vision services for near reading. The reading rate was 24 WPM at the age of 5 years, whereas at the age of 21 years, it was 55 WPM. CONCLUSION: The majority of the students with low vision need optical low vision services. Accurate refraction is important in these students. The reading rate increases significantly with improved near visual acuity.

Organophosphorus poisoning.

Acute poisoning by organophosphorus (OP) compounds is a major global clinical problem, with thousands of deaths occurring every year. Most of these pesticide poisoning and subsequent deaths occur in developing countries following a deliberate self ingestion of the poison. Metacid (Methyl parathion) and Nuvan (Dichlorovos) are commonly ingested OP pesticides; Dimethoate, Profenofos, and Chlorpyrifos are other less frequently ingested compounds in Nepal. The toxicity of these OP pesticides is due to the irreversible inhibition of acetylcholinesterase (AChE) enzyme leading to accumulation of acetylcholine and subsequent over-activation of cholinergic receptors in various parts of the body. Acutely, these patients present with cholinergic crisis; intermediate syndrome and delayed polyneuropathy are other sequel of this form of poisoning. The diagnosis depends on the history of exposure to these pesticides, characteristic manifestations of toxicity and improvements of the signs and symptoms after administration of atropine. The supportive treatment of OP poisoning includes the same basic principles of management of any acutely poisoned patient i.e., rapid initial management of airways, breathing, and circulation. Gastric lavage and activated charcoal are routinely used decontamination procedures, but their value has not been conclusively proven in this poisoning. Atropine is the mainstay of therapy, and can reverse the life threatening features of this acute poisoning. However, there are no clear cut guidelines on the dose and duration of atropine therapy in OP poisoning. Cholinesterase reactivators, by regenerating AChE, can reverse both the nicotinic and muscarinic effects; however, this benefit has not been translated well in clinical trials. All these facts highlight that there are many unanswered questions and controversies in the management of OP poisoning and there is an urgent need for research on this aspect of this common and deadly poisoning.

Relapsing polychondritis: a rare disease of multisystem involvement.

Relapsing Polychondritis (RP) is a rare connective tissue disease of unclear pathogenesis and may present with multisystem involvement. In this report we describe a case of Relapsing Polychondritis, a rare autoimmune disease of varied presentation, course, and response to therapy.

Twelve episodes of meningitis in the same patient!

Recurrent bacterial meningitis is potentially life-threatening; resultant complications and adverse events during management take their extra toll on the patient. A rare case with 12 consecutive episodes of pyogenic meningitis, probably the maximum number ever reported in the literature, has been presented. A minor head injury but with no subsequent cerebrospinal fluid (SCF) leak during childhood was the index event in this patient. High resolution computerized tomography (HRCT) scan of the base of the skull clearly revealed a bony dehiscence missed on numerous previous imagings. Culprit micro organisms involved in recurrence were Streptococcus pneumoniae and Neisseriae meningitides. Though surgical repair would have been definitive treatment, medical management with pneumococcal and meningococcal vaccination and prophylactic penicillin have been used in this patient to prevent further recurrences.

Hemidiaphragmatic paralysis: a rare complication of cervical herpes zoster.

Herpes zoster, a sequel of the reactivation of the varicella zoster virus, usually presents with cutaneous eruptions associated with intense pain and burning sensation in the affected dermatomes. Motor weakness, however, can sometimes complicate herpes zoster. In this report we present a case that had diaphragmatic motor weakness as a sequel of herpes zoster lesions in the neck.

Paroxysmal nocturnal hemoglobinuria.

Paroxysmal nocturnal hemoglobinuria (PNH) is a rare hemolytic disorder of acquired origin and is clinically manifested by chronic hemolysis, thromboses in various sites, and bone marrow failure. The disease is so rare that the delay in the diagnosis is not uncommon and this bears a tremendous impact on patient management. We present this case to draw attention to this uncommon cause of hemolytic anemia, which should be considered in any patient, of any age, who has signs of chronic hemolysis.

Poisoning : pattern and profile of admitted cases in a hospital in central Nepal.

An analysis of all poisoning cases admitted in medical and pediatric wards of Patan Hospital for one year (1st Jan to 31st Dec 2004) was carried out. A total of 154 cases were admitted which was 0.8% of total hospital admissions. Females outnumbered males and almost two-thirds patients were young adults (15-34 years). Seasonal variation in poisoning was observed with more cases in the summer months. Organophosphorus compounds (42%), drugs (25%), and zinc phosphide (6.5%) were common poisonings in total and in adult populations, whereas kerosene was the most frequent poisoning in pediatric age group. Paracetamol, benzodiazepines, and tricyclic antidepressants were the most frequently used drugs. The circumstances of poisoning were intentional (75%) and accidental (20%); most of the childhood poisonings were accidental in nature. The mean hospital stay for all type of poisoning was 7.5 days; whereas it was 10.2 days for organophosphorus, 2.5 days for paracetamol, and 1.5 days each for zinc phosphide and kerosene ingestion. Intensive care unit (ICU) service was required in 17% of patients; and almost 25% developed complications. Aspiration pneumonia and respiratory failure were the most frequently observed complications. Ninety four percent of admitted patients recovered completely; leaving a mortality rate of 5%.

Delayed presentation of Sheehan's syndrome: a case report.

Sheehan's syndrome, though rare, is still one of the commonest causes of hypopituitarism in developing countries like ours. The clinical presentation is variable with abrupt or insidiously developing pituitary insufficiency after a heavy intrapartum or postpartum haemorrhage. We present an elderly lady with this syndrome who had slowly progressive panhypopituitarism 24 years after a severe haemorrhage associated with the delivery of triplets.