Childhood-onset systemic lupus erythematosus: a cohort study

Objective: To determine the clinical and immunological characteristics and short-term outcome of children with systemic lupus erythematosus [SLE]. Study Design: A descriptive cohort study. Place and Duration of Study: Paediatric Rheumatology Clinic, The Aga Khan University Hospital, Karachi, from January 2011 to December 2015

Methodology: Clinical and immunological profile and short-term outcome of children less than 16 years of age admitted in the paediatric ward, with the diagnosis of SLE was studied. Demographic data, clinical presentation, laboratory findings, immunological profile and treatment regimens of these children were evaluated

Results: Thirty-two children, satisfying the criteria of American College of Rheumatology [ACR] for SLE, were enrolled during the study period of five consecutive years. A female predominance was observed with 28 [87.5%] patients being female [F:M 7:1]. Mean age at symptom onset was 10.5 +2.7 years; and 8.8 +2.1 years in females and males, respectively. The mean age at diagnosis was 11.3 +2.8 years in females and 9.4 +1.9 years in males. Prolonged fever was the most common non-specific symptom found in 27 [84%], followed by pallor in 13 [41%] patients. Twenty-two [69%] children were found to be anemic and 18 patients [56%] having signs of arthritis at presentation. Renal involvement was observed in 15 [47%] patients. The most common laboratory finding was anemia, found in 22 [69%] of cases. The most common immunological markers were serum anti-neutrophil antibodies [ANA], positive in 28 [88%] patients, followed by anti double-stranded DNA antibodies, raised in 26 [81%] of cases. Out of 32, 12 patients were lost to follow-up. Of the remaining 20 children who were followed for four years, ten [50%] went into remission

Conclusion: Childhood-onset SLE encompasses a wide variety of manifestations with a female preponderance. Fever, arthralgia and pallor are the most frequent clinical manifestations among the children. Hemolytic anemia [HA] is the most common laboratory abnormality, with ANA and anti ds-DNA antibodies positivity in the majority of padiatric patients

Moyamoya: an uncommon variant of stroke in children

Moyamoya is a rare cause of stroke in children. It is an infrequent cerebrovascular disorder of unknown etiology. We are reporting a case of a 7-year-old girl who presented with an acute history of left-sided weakness. On imaging she was diagnosed with Moyamoya disease. CT Angiogram revealed an occlusion of the right middle cerebral artery typical of Moyamoya disease. The child improved with conservative therapy

Prerequisites for histopathological evaluation of reduction mammoplasty specimens: Recommendations based on an audit in a tertiary care hospital

Objective: To recommend prerequisites for the histo-pathological evaluation of reduction mammoplasty [RM] specimens based on an audit in a tertiary care hospital

Methods: All reduction mammoplasty specimens received at department of pathology, in a tertiary care hospital over a 3 years period were re-evaluated retrospectively. Medical records were checked for age, family history of breast cancer, indication for surgery and radiological evaluation. Pathology reports were reviewed for number of blocks sampled and diagnosis. A prerequisites protocol was developed based upon deficiencies and impediments noted

Results: We received a total of 26 cases of reduction mammoplasty. Only 2 [7.69%] specimens were from males. The age of the patients ranged from 26 to 50 years. Indication for surgery was provided in all [100%] of cases, with macromastia being most prevalent indication. Family history of breast carcinoma and radiological evidence was absent or not provided in all cases. The number of blocks prepared were in range of <4 in 17 [73.91%], 5-8 blocks in 5 [19.23%] and 8-12 blocks in 4 cases [15.38%]. The main histopathological finding was fibrocystic change, [n = 12, 46.15%]. Specimen radiography was not conducted in any case

Conclusion: There is a need to stratify RM cases as high, moderate and low risk of breast cancer based on family history of breast carcinoma, clinical and radiological evaluation. The high risk cases should be oriented, with margins inked and extensive sampling done. Specimen radiography should be carried out in younger patients in which mammography is not recommended

The myomatous erthrocytosis syndrome; a case report and review of literature

Uterine leiomyomas are the most common benign pelvic tumours in females. Rarely they are associated with symptoms of ectopic hormone production. One of these syndromes is the myomatous erythrocytosis syndrome, which is a triad of polycythaemia, fibroid[s] and resolution of the polycythaemia after hysterectomy. The case presented is of a post-menopausal lady found to have a Hb of 21 during workup of a pelvic mass. A diagnosis of secondary polycythemia due to ectopic erythropoietin production by the fibroid was made. After five sessions of phlebotomiesa target Hb of 15 was obtained for surgery. The haematological values restored to normal three days post-operatively. It has been postulated that all fibroids produce erythropoietin. Recognition and proper management of this condition is important to differentiate between primary and secondary polycythaemia as primary polycythemia is a thromboembolic condition, to avoid un-necessary investigations and to prevent any complications secondary polycythaemia may have per-operatively

Molecular classification of breast cancer: an overview with emphasis on ethnic variations and future perspectives

Morphologically identical breast cancers can display divergent clinical outcomes and responses to therapy. This can predominantly be attributed to molecular class differences that exist amongst histologically similar cancer types. Consequently, molecular classification can be more powerful than histopathology as a predictive factor for the different treatments. This article reviews the molecular classification of breast cancer and emphasizes that ethnic variations may exist in molecular class prevalence patterns. It also highlights key insights into the currently defined molecular classes as provided by ongoing research on primary breast cancers using recent state-of-the-art technology. Such research is revealing that significant molecular heterogeneity may exist within the molecular classes themselves. More diverse ethnic variations may also be unraveled. The results of ongoing and upcoming research may provide more precise prognostic and predictive information about breast cancer and perhaps a breakthrough step toward "personaliziation" of breast cancer treatment. Forty-one relevant articles [2000-2012] extracted through PubMed and Google advanced searches and at our institute's library were utilized to prepare the article, along with results of published and ongoing research hby the authors

Indeterminate cell tumor [histiocytosis]

Indeterminate cell tumor [ICT; histiocytosis] is a rare disorder characterized by accumulation of histiocytes that do not fulfill the phenotypic criteria designated for Langerhans cells [LC]. The cells classified as "indeterminate" exhibit overlapping features between dendritic cells and histiocytic cells by showing variable reactivity for CD1a and positivity for S-100 protein and CD68. Ultrastructurally, absence of Birbeck granules, a feature consistent with LC, epitomizes the lesional cells. Herein, we report a case of ICT in a new born emphasizing its histogenesis and clinical, morphologic, immunohistochemical, and ultrastructural features

Frequency and characteristics of skin cancers diagnosed at Ayub Medical College, Abbottabad Pakistan from 1995-2003

Our histopathology laboratory serves mountainous area of Hazara Division in Northern Pakistan. This study is an analysis of the records of skin cancers reported over a nine years period to develop local statistics about frequency and characteristics of skin cancers. Data from 1995-2003 record of cancers reported by Department of Pathology, Ayub Medical College, Abbottabad were analysed to calculate cumulative and year wise frequency of skin cancers and their major types. Frequency distribution with reference to gender and age was also calculated. Skin cancers were 220 [1.04%] out of the total 21075 cancers recorded during this period. Squamous cell carcinoma was diagnosed in 118 [53.6%], Basal cell carcinoma in 88 [40%] and Malignant melanoma in 14 [6.4%] cases. The mean age for all skin cancers considered together was 58.75 +/- 15.21. Non Melanoma Skin Cancer [NMSC] were seen in all age groups [minimum 15, maximum 103 years], while no malignant melanoma patient was under 35 years of age. Gender difference in distribution was negligible with slight male preponderance [23:21]. Malignant melanoma was significantly [p<0.05] more in males [71.4%], while distribution of both the types of NMSC was non-significantly in favour of males. We found no significant rise or fall in total skin cancers or any type of skin cancer in this 9 years period. At present the frequency of skin cancer is stable at around 1% of total cancers reported at Ayub Medical College, reflecting a stable very low incidence rate of skin cancer in Hazara Division of Pakistan

HbsAg and HCV: Increasing test requests and decreasing frequency of positive tests at clinical laboratory of Ayub teaching hospital, Abbottabad

This audit was carried out to assess the frequency of positive results out of the total test requests made for HBsAg and HCV at our laboratory. The frequencies for three years were compared for significance. We have reported monthly total test requests and frequency of positive tests for each of these years in this article. This study is an audit of all HBsAg and HCV test requests received at clinical laboratory of Ayub Teaching Hospital, Abbottabad during a three year period from 2002-2004. Both the tests were made using Rapid device [immunochromatographic kits] method. Frequencies of positive results were calculated from the total test requests for each month of these three years. Cumulative frequencies were compared for statistical significance of difference. Total HBsAg tests requested for years 2002, 2003 and 2004 were 2058, 2563 and 5207 respectively. The frequencies of HBsAg positive cases out of these were 5.53%, 4.36% and 2.68% respectively. The number of test requests for HBsAg increased significantly from 2002 to 2003 and 2004, while the frequency of positive tests decreased significantly [p<.001] during this period. Total HCV requests for years 2002, 2003 and 2004 were 1261, 1671 and 4638 respectively. The frequencies of HCV positive cases out of these were 14.19%, 8.79% and 5.84% respectively. Just like HBsAg, number of test requests for HCV increased significantly from 2002 to 2003 and 2004, while the frequency of positive tests decreased significantly [p<.001] during this period. Conclusions: The test requests have considerably increased in the last few years, probably as a result of increasing awareness of clinicians and public. However most of these tests are negative. In view of high prevalence of both HBsAg and HCV in Pakistan, this overcautious attitude of clinicians is understandable although this is a burden on the pocket of patients and hospital resources

Types of brain tumors in hazara division and northern areas of Pakistan

Background: Early diagnosis and management of brain tumors is important to reduce morbidity and mortality. The objective of this study was to calculate frequency of different types of brain tumors in patients operated and managed at Ayub Teaching Hospital, Abbottabad

Methods: This study was carried out at Neurosurgical Department of Ayub Teaching hospital, Abbottabad from September 1999 to October 2003. Records of patients who consented for surgery after CT scan evidence of brain tumors, which were proved on biopsy later on, were included in the study. The tissue biopsy was taken by open craniotomy or by computerized stereotactic method. Pattern of different brain tumors was studied during this period

Results: Our study revealed predominantly tumors arising from neuroepithelium [49.2%] while tumors arising from meninges were relatively low [23.3%]. Nerve sheath tumors [4.2%], germ cell tumors, mal-developmental origin [2.5%], blood vessel tumors [5.8%] and miscellaneous tumors [6.6%] were found relatively in a very low frequency

Conclusion: Evaluation of brain tumor frequency will help in future management plan that may be in the form of open craniotomy, stereo tactic radiosurgery, whole brain radiotherapy or gamma knife surgery