Surgical outcome of spelenectomy in Thalassemia major in children

Objective: To determine the surgical outcome of splenectomy in children with thalassemia major

Methods: It is an observational and descriptive study conducted in Department of Paediatric Surgery in collaboration with hematology, radiology, anesthesia and paediatric intensive care department at The Children's Hospital and the Institute of Child Health, Multan during the period of September 2007 to September 2013. A total of 50 patients suffering from thalassemia major already diagnosed and under management reffered from haematology department for splenectomy were included in this study. After admission, patients were assessed on the basis of history, clinical examination, and necessary investigations before surgery and later on follow-up. Investigations carried were CBC, PT, APTT, Viral markers, ECG, X-ray Chest, abdominal ultrasonography and ECHO if necessary. Splenectomy was performed after prophylactic vaccination against post splenectomy infections. Follow up was performed for at least two years. Blood transfusion requirements and number of hospital visits per annum before and after splenectomy were calculated and results analyzed statistically using SPSS-20

Results: Fifty patients were included in this study. Out of these fifty, 43 [86%] male and 7[14%] were female with a mean age of 9 years. Average blood transfusion requirement was 250 ml/kg/year, interval of blood transfusion was two weeks and twenty five visits per year before splenectomy. After splenectomy, requirement of blood transfusion reduced to 125ml/kg/year, interval between transfusion increased to one month and hospital visits reduced up to twelve per year

Conclusion: Blood transfusion requirement and number of hospital visits per year are decreased and interval between transfusions is increased after splenectomy. Splenectomy should not be delayed when indicated. Preoperative vaccination decreases the chance of post splenectomy infection

Granuloma formation in discoid lupuserythematosus: a novel microscopic feature

Lupus erythematosus is an autoimmune disease with marked pleotropism. If several systems are involved then the disease is named as systemic lupus erythematosus [SLE] and if skin is exclusively involved the term discoid lupus erythematosus [DLE] is used. One of the several histopathological features of DLE includes periappendageal inflammation. This may at times ; completely wipe out sebaceous glands forming sebaceous granulomas. To determine the frequency of sebaceous granulomas formation in discoid lupus erythematosus. In this prospective observational study was conducted at the Departments of Dermatology and Pathology, Pakistan Institute of Medical Sciences, Islamabad. 100 cases of DLE spanning over two years and with the age range of 3 years to 70 years were examined for the presence of sebaceous granuloma. Other features of DLE like hyperkeratosis, follicular plugging, epidermal atrophy, basal layer vacuolization, basement membrane deposits, pigmentary incontinence, perivascular inflammation, periappendageal inflammation, and collagen damage were also noted., Out of these 100 cases, 8 cases contained sebaceous granulomas. These granulomas were,, . composed of epithelioid cells, foreign body giant cells containing partially digested sebaceous material and a few lymphoctytes. Sebaceous granulomas formation was seen in 8% cases of DLE cases. This feature must be recognized both by dermatologists and pathologist so that diagnosis of DLE may not be distracted and erroneous diagnosis due to presence of granulomas may not be rendered

Response to treatment of cutaneous leishmaniasis with intralesional chloroquine vs intralesional meglumine antimoniate

In Pakistan, cutaneous leishmaniasis [CL] is mostly caused by Leishmania major. For simple lesions which are few in number and where there is no risk of disfigurement or joint mobility restriction, topical application or local treatment e.g. intralesional antimony compounds are valuable. To compare the effect of intralesional chloroquine with meglumine antimoniate in the treatment of CL. In this quasi experimental study, 60 patients of CL with 1 to 3 lesions and aged >3years were divided into 2 treatment groups to receive either intralesional chloroquine [treatment group] or meglumine antimoniate [control group]. Both drugs were used Ice per cm[2] of lesion, once weekly for 8 weeks [8 injections]. 8 more injections were given to those who showed partial response. Both treatments showed 100% response; however, greater number of injections was required with meglumine antimoniate [p<0.05]. Both drugs were well tolerated. Intralesional chloroquine appears to be an effective, safe and cheap alternative to meglumine antimoniate in the treatment of CL

Missed abortion; misoprostol versus cervical catheter as cervical priming agent

To compare the effectiveness of extra-amniotic cervical catheter and vaginal misoprostol as a cervical priming agent prior to surgical evacuation in first trimester missed abortions. Randomized clinical trial. At lady Willingdon hospital gynecology unit 1 from 1 st March 2008 to 28th February 2009. 100 patients with diagnosis of missed abortions of up to 12 weeks + 6 days were studied. The primigravida were included. They were divided in two groups. In group. A [50] patients 400 microgram [2 tablet] misoprostol were placed high up in posterior vaginal fornix while in group B [50] patients extra-amniotic. Foleys catheter was placed aseptically 6 hour before surgical intervention as priming agent. All multigravida, patients sensitive to prostaglandin, and with disturbed coagulation were excluded. The main out comes are 1, cervical dilatation and effacement 2, complications like fever, pain lower abdomen, headache and vomiting 3, amount of bleeding in ml after application of agent. Cervical catheter proved to be good cervical priming agent comparable to misoprostol. Cervical dilatation was significantly better in misoprostol [> 10 mm 44%, > 8mm 30%, > 5mm 20%] as compared to Foleys group [>10 mm 24%, > 8mm 38%, > 5mm 20%] while in 3 [6%] dose of misoprostol was repeated and in 10 [20%] patient in Foleys group has no effect. The side effects occurred in both groups but systemic effects were more in misoprostol, pain lower abdomen 42% VS 46%, backache 18% VS 26%, fever 10% VS nil, headache 16% VS nil and no side effect 14% VS 28% as compared to Foleys catheter. Systemic effects were absent in Foleys catheter due to inert nature. Bleeding occurred in all patients with misoprostol while it was absent in 48% cases in Foleys group [>60ml 42% VS 8%, <40ml 32% VS 14% < 20ml 26% VS 30%]. 48% cases had no bleeding in Foleys group. Cervical catheter proved to be good priming agent due to lesser systemic side effects as compared to misoprostol

Sebaceous granulomas: a novel feature of discoid lupus erythematosus

To determine the frequency of sebaceous granulomas formation in discoid lupus erythematosus, Retrospective observational study. Departments of Dermatology and Pathology, Pakistan Institute of Medical Sciences, Islamabad. 100 cases of Discoid Lupus Erythematosus [DLE] spanning over two years and with the age range of 3 years to 70 years were examined for the presence of Sebaceous Granuloma. Other features of DLE like hyperkeratosis, follicular plugging, epidermal atrophy, basal layer vacuolization, basement membrane deposits, pigmentary incontinence, perivascular inflammation, periappendigeal inflammation, and collagen damage were also noted. Lupus erythematosus is an immune complex, type Ill hypersensitivity disease where antibodies are formed against native Deoxyribose Nucleic Acid [DNA]. The immune compexes are deposited in various organs and various sites causing marked pleotropism. If several systems are involved then the disease is named Systemic Lupus Erythematosus [SLE] and if skin is exclusively involve the term Discoid Lupus Erythematosus [DLE] is used. One of the several features of DLE includes periappengeal inflammation. This may at times completely wipe out sebaceous glands forming sebaceous granulomas. Out of these 100 cases 8 cases contained sebaceous granulomas. These granulomas were composed of epithelioid cells, foreign body giant cells containing partially digested sebaceous material and a few lymphoctytes. Sebaceous granulomas formation was seen in 8% cases of discoid lupus erythematosus. This feature must be recognized both by dermatologists and pathologist so that diagnosis of DLE may not be distracted and erroneous diagnosis due to presence of granulomas may not be rendered

Management of viable sigmoid volvulus by mesosigmoidoplasty

Mesosigmoidoplasty is a non-resective procedure for viable sigmoid volvulus. It corrects the main pathogenetic factor, the narrow but long mesosigmoid. This study was conducted to know the outcome of mesosigmoidoplasty in cases of viable sigmoid volvulus. This prospective study was conducted from July 2004 to June 2008 at District Headquarter Teaching Hospital D.I.Khan, on patients admitted with large gut obstruction due to viable sigmoid volvulus. After initial investigations and resuscitation, non-operative decompression was tried in all cases. Successfully decompressed patients underwent elective laparotomy and the rest had emergency laparotomy and mesosigmoidoplasty. After their discharge from hospital, patients were followed up for 6 months. Thirty-nine patients presented with viable sigmoid volvulus; 35 males and 4 females, with male to female ratio of 8.7:1. Age range was 48-70 years. Thirteen [33%] patients had successful decompression. Two patients refused surgery after successful non-operative decompression and were dropped from the study. Out of the remaining 37 patients 11[30%] patients had mesosigmoidoplasty on elective list and 26[70%] had emergency operation for mesosigmoidoplasty. Average hospital stay was 4 days [2-6 days]. Post-operative complications were wound infection in 1[2.7%] and paralytic ileus in 3[8%] cases. During 6 months follow-up, only 1[2.7%] patient had recurrence. Mesosigmoidoplasty is a definitive procedure for viable sigmoid volvulus with low rates of mortality, morbidity

Experience in management of fournier's gangrene: a review of 19 cases

Fournier's gangrene is a polymicrobial necrotizing fasciitis of the perineal, perirectal or genital area. The purpose of this study was to report our experience and results in the management of Fournier's gangrene. Retrospective review of charts of 19 consecutive patients of Fournier's gangrene treated between January 2005 and December 2008 in surgical unit, District Headquarter Teaching Hospital, D.I.Khan. 19 male patients were identified [mean age 54.7 years, range 39-68 years]. Etiology included anorectal in 21%, urogenital in 26.3%, cutaneous in 31.6% where as in 21% of cases the cause remained undetermined. Comorbid conditions were identified in 57.9% cases and included diabetes mellitus, chronic renal failure, malnutrition, cirrhosis and chronic steroid therapy. The most common organisms found on culture were Bacteroides fragilis, E coli, Streptococci and Staph aureus. The treatment plan was resuscitation, broad spectum intravenous antibiotics and multiple debridements [mean 2.9, range 1-8] per hospital stay [mean 26 days, range 10-52 days]. Diversion procedures were also performed, which included cystostomy [15.8%] and colostomy [5.3%]. Reconstructive measures included local skin flaps in 2 cases and split thickness skin grafts in 3 cases. The mortality rate was 10.5% [2 patients died due to severe sepsis]. Early recognition of the pathology and aggressive surgical debridement are the mainstay of the management of Fournier's gangrene. Additional strategies to improve wound healing and increased patient survival are also needed

Follicular thyroid carcinoma presenting initially with skull metastasis

Follicular thyroid carcinoma very rarely present initially with metastasis to skull. This report describes an otherwise asymptomatic 52 years old female patient with follicular thyroid carcinoma, who initially presented with soft mass in right occipito-parietal region and a multinodular goiter. Radiology showed an osteolytic lesion with overlying soft tissue component. Histopathology revealed metastasis from a well differentiated follicular carcinoma of thyroid gland

Comparison of fine needle aspiration with biopsy in the diagnosis of cutaneous leishmaniasis

Leishmaniasis is a zoonotic infection caused by the protozoa belonging to the genus Leishmania .Demonstration of Leishman-Donovan [LD] bodies on histopathological examination of biopsy specimen is considered to be the definitive diagnostic modality. This study was designed to assess the diagnostic value of fine needle aspiration and to compare it with full thickness biopsy. This was randomized, open label comparison study conducted in department of dermatology, Pakistan Institute of Medical Sciences, Islamabad from 1 [st] June 2007 to 31 [st] July 2007.15 patients, 15 years of age or older were enrolled in the study. Selection of patients was made on the basis of clinical features. Patients with the lesions having typical clinical features of cutaneous leishmaniasis-like erythematous crusted plaques or nodules were enrolled. Demographic characteristics including age, sex residential address site, size, shape and duration of lesions was noted Informed consent was taken. Procedure and its pros and cons were explained to the patient. Procedure was performed in the FNA room of pathology department under strict aseptic conditions. Local anaesthesia was not given as patients tolerated the pain well. Biopsy specimen was taken from the same lesion under local anaesthesia after doing FNA. Slides were processed and examined. The diagnosis was confirmed on the basis of finding giant cells or Leishman-Donovan bodies on FNA of the lesion. Out of 15 patients 12 patients had positive FNA and three patients had negative FNA report. While the biopsy specimen showed typical histopathological features of cutaneous leishmaniasis in 14 out of 15 patients and one turned out to be a case of ruptured inclusion cyst. So out of 3 negative cases of FNA one had ruptured inclusion cyst. The diagnostic rate was 80%for FNA and 93.3%for biopsy. Fine needle aspiration seems a reasonably good diagnostic modality for cutaneous leishmaniasis.Its sensitivity is comparable to that of full thickness biopsy

Eosinophilic granuloma: a rare presentation of Langerhans cell histiocytosis in a young female

Histiocytosis encompasses a group of diverse disorders that have in common, as a primary event, the accumulation and infiltration of monocytes, maerophages, and dendritic cells in the affected tissues. Langerhans cell histiocytosis [LCH] can be local and asymptomatie, as in isolated bone lesions, or it can involve multiple organs and systems with significant symptomatology and consequences. Thus, the clinical manifestations depend on the site of the lesions and organs and systems involved and theft functions. We report a case of a 35-year-old lady who presented with skin lesions in presternal area mimicking very closely to those of scrofuloderma [a form of skin tuberculosis]. She had been treated with antituberculous therapy thrice with no benefit. There was no evidence of systemic involvement. Diagnosis of LCH was confirmed on histopathological findings of skin lesion and markers. Patient was treated with oral methttrexate with resolution of lesions

Ivermectin in the treatment of scabies

Ivermectin is structurally similar to the macrolide antibiotics, but does not have antibacterial activity. It is, however, active against number of ecto- and endoparasites. It has been extensively employed in veterinary medicine, and in humans it is used to treat filarial diseases, principally onchocerciasis. A clinical trail was conducted for the first time in Pakistan to determine the efficacy of oral ivermectin in patients with scabies and its comparison with the most effective standard treatment available until now i.e. permethrin applied locally. This non randomized, open-label comparison study was conducted in department of dermatology, Pakistan Institute of Medical Sciences, Islamabad, from January, 2007 to March, 2007. 30 patients, 12 years of age or older were enrolled in the study. Diagnosis was made on the basis of clinical features, including history and clinical examination with typical lesions and sites of involvement. 15 patients received orally administered ivermectin, two doses of 200 micro g/kg body weight separated by one week. Remaining 15 patients received local treatment with permethrin 5%, according to the standard method, in which it is applied for 12 hours and was repeated after one week. Pretreatment complete physical examination was done along with necessary laboratory investigations in all cases. They were repeated after 2 weeks at the end of treatment to note any abnormality developing. Groups were almost matched in terms of age, sex and weight. The efficacy was evaluated by the relief of symptoms and disappearance of the lesions. All patients completed therapy without any complication. Patients recovered completely with relief of symptoms and complete healing of skin lesions. The cure rate was 100% in both groups, at the end of treatment period. Ivermectin seems to be a safe and effective alternative to permethrin. The striking advantage of ivermectin being its benefit of oral administration and low cost

Chloroquine in cutaneous leishmaniasis

Chloroquine is an antiprotozoal drug that has shown a favorable therapeutic index for cutaneous leishmaniasis. A clinical trial was conducted to determine the efficacy of oral chloroquine in patients with cutaneous leishmaniasis and its comparison with the most effective standard treatment, pentavalent antimony compounds. This non randomized, open-label comparison study was conducted in department of dermatology, Pakistan Institute of Medical Sciences, Islamabad, from August 2006 to January 2007. 30 patients, 12 years of age or older were enrolled in the study. Diagnosis was made on the basis of clinical features and histopathological examination. 15 patients received orally administered chloroquine 250mg tds for 20 days and remaining 15 patients received injectable pentavalent antimony 20mg/kg for 28 days. Pre-treatment complete physical examination was done along with necessary laboratory investigations in all cases. They were repeated after 2 weeks and then at the end of treatment to note any abnormality developing. Groups were almost matched in terms of age, duration of lesions and number of lesions. The efficacy was evaluated by the appearance of the lesion and healing with or without scar formation. Patients were followed up for 3 months. All patients completed the study without any complication. Lesions healed completely with only minimal scarring or post-inflammatory hyperpigmentation at the site of lesion. The cure rate at 3-month follow-up was 100% in group A [chloroquine] and 93% in group B [antimony compound] at the end of treatment period. Chloroquine seems to be a safe and effective alternative to antimony compounds. The striking advantage of chloroquine being its benefit of oral administration, and low cost along with easy availability which is of particular importance in underdeveloped countries like Pakistan

Griscelli's syndrome in a young adult - a case report with the review of literature

Griscelli syndrome is a rare autosomal recessive disorder characterized by partial albinism with variable immunodeficiency. Silvery grey hair with large, clumped melanosomes on microscopy of hair shafts is diagnostic. The commonest complication leading to mortality includes lymphohistiocytic proliferation in various organs, including the brain. There are three types of this syndrome described. We present a young adult with classic clinical features and confirmatory findings of clumped melanosomes on microscopy of hair shaft

Degos disease a rare disorder

Malignant atrophic papulosis [Degos

Tuberous sclerosis in a young adult

Tuberous sclerosis is a genetic disorder of hamartoma formation in many organs, characterized by angiofibroma, Shagreen patch, periungual fibroma, ash-leaf macule, although not invariably, seen in association with epilepsy and mental retardation. We document a case of tuberous sclerosis in a young adult without CNS manifestations

Acute mastoiditis in perspective

Thirty cases of acute mastoiditis with sub periosteal abscess were treated at Federal Government Services Hospital over a period of 8 years. The salient feature in these cases was the presence of extensive cholesteatoma in the mastoid presenting mainly in the paediatric age group. These children belonged to poor communities, were malnourished and attended hospital in a state of emergency. The presentation and course of these cases confirms the aggressive and potentially serious nature of the pathology requiring early medical attention in order to avert impending complications. Significantly all patients had a history of ear discharge prior to abscess formation. It appears that in our environment most cases of acute mastoiditis with abscess formation develop from chronic otitis media with cholesteatoma

Plasmacytoma of the nasal cavity

A case reported of 50 year old male with repeated epistaxis and mass in right nasal cavity. It was approached through lateral rhinotomy which revealed its attachment to lateral wall of the nose but there was no bony erosion. Excision biopsy confirmed it to be a plasmacytoma