rare case of Kikuchi-Fujimoto Disease associated with systemic lupus erythematosus

Kikuchi-Fujimoto Disease [KFD], also known as histiocytic necrotizing lymphadenitis, is a self-remitting, immune-mediated rare disorder having unique histopathological characteristics which is commonly seen in young Asian females, but can occur in all ethnicities. There is a strong association between KFD and Systemic Lupus Erythematosus [SLE]. We present a case of a young Pakistani boy who presented with cervical lymphadenopathy, fever, blackish discoloration of finger tips, and Raynaud's phenomenon. His lymph node biopsy was suggestive of KFD. The American Rheumatology Association diagnostic criteria were not met as no other features of SLE were present. His autoimmune workup including Anti-Nuclear Antibodies [ANA] and Anti-Double Stranded DNA [Anti-Ds DNA] antibodies were positive and supported the diagnosis of SLE. He improved clinically with steroid therapy and nifedipine with resolution of symptoms

Pheochromocytoma: a rare cause of childhood hypertensive encephalopathy

Pheochromocytomas are rare neuroendocrine tumours of chromaffin tissues. They are catecholamine secreting tumours which cause severe hypertension and other systemic disturbances. Of all the causes of childhood hypertension, pheochromocytoma constitutes less than 1%. We report the case of a 12 years old child who presented with hypertensive encephalopathy, confirmed histologically to be secondary to pheochromocytoma, and cured with meticulous critical care and surgical resection

Role of biopsy in pediatric lymphadenopathy

To determine the role of lymph node biopsy in the diagnosis of lymphadenopathy and to find out the pattern of different diseases in relation to age, gender, and the site of lymph nodes involved. This retrospective study was carried out at the Histopathology Department of the Children's Hospital and The Institute of Child Health, Lahore, Pakistan, over a period of 9 years, from January 1999 to December 2007. Tissue samples were collected from 898 children presenting with lymphadenopathy, and the diagnosis was confirmed on histology and through various specific tests. The clinical data of the patients were collected from computerized hospital records. Among the total 898 consecutive lymph node biopsies, the most common pathology encountered was reactive hyperplasia in 356 children [39.6%], followed by tuberculosis in 262 [29.1%] and malignant lymphomas in 132 children [14.6%]. The rest of the lesions include; 72 cases of granulomatous lymphadenitis [8%], 13 of histiocytosis X [1.4%], 44 [4.9%] of metastatic tumors, 16 of chronic inflammation [1.8%], and 3 cases of Kikuchi's disease [0.3%]. The cause of lymphadenopathy was found to be significantly associated with age, gender, and site of the lymph nodes involved. Lymphadenopathy is a relatively common condition in the pediatric age group. Although 39.6% of children had reactive hyperplasia of unknown etiology, 60.3% children presented with a specific diagnosis

Clinico-morphological pattern of bone lesions in children

The present study was performed to find the spectrum of bone diseases in children and to correlate with age, sex, and site of origin. This was a retrospective study and consists of 95 cases of hone biopsies which were diagnosed in the Department of Pathology at the Institute of Child Health and Children's Hospital, Lahore during a period of six years [Jan 2003-Dec 2008]. These biopsies were evaluated on haematoxylin and eosin stained sections from paraffin embedded tissue blocks. Special stains were performed whenever required. Pyogenic osteomyelitis [OM] accounted for 35.78% of all bone pathologies followed by tuberculous osteomyelitis in 22.10%. Osteosarcoma [OS] was the most frequent [primary tumour of bone followed by Ewing's sarcoma [ES]. One case of metastatic bone tumour was also recorded. Osteochondroma, osteoma, aneurysmal bone cyst, fibro-osseous dysplasia, chondroma and simple bone cyst were also found in children. The bone tumours are relatively more prevalent in males

Sonographic evaluation of the triangular cord sign in the diagnosis of biliary atresia, confirmed with needle liver biopsy

Neonatal cholestasis continues to present a diagnostic challenge for pediatric surgeons. Various diagnostic protocols are available, often involving invasive procedures which are generally not available in the district general hospitals. The objective of this study is to evaluate the role of Triangular Cord sign [TC sign] on ultrasound for the prompt and easy diagnosis of biliary atresia in infants. A prospective study was planned. Patients with positive TC sign underwent needle liver biopsy for confirmation. Infants with neonatal cholestasis were referred from the gastroenterology department. Abdominal ultrasound was performed with 3-6MHz convex and 6-10MHz linear transducers on Core Vision, Toshiba by a single paediatric radiologist and results were correlated with needle biopsy of the liver interpreted by the histopathologist. Thirty nine Infants with cholestatic jaundice underwent ultrasound and biopsy. Twenty patients were diagnosed on ultrasound as biliary atresia on the basis of positive triangular cord sign and 18 of them were confirmed on biopsy

Morphological pattern and frequency of intracarnial tumours in children

The objective of the present study was to observe the histopathological pattern of intracranial tumours and to provide a comprehensive data about its frequency in children less than 16 years and to correlate the site of lesion with the histological diagnosis. Design: A descriptive study. Place and Duration of Study: The study was carried out at the Department of Histopathology, Children's Hospital and Institute of Child Health, Lahore and King Edward Medical College, Lahore over a period of three years. [January 1999 - December 2002]. Subjects and The histopathological data of 116 brain tumours of all the patients less than 16 years was collected and compared with the findings reported from centres in other parts of the country. The initial histopathological evaluation of these lesions was performed on H and E stained sections of paraffin embedded tissues. Special stains and immunohistochemistry were performed whenever indicated. Autolyzed biopsies and previously reported biopsies were not included. The ages ranged from 5 days to 16 years with male to female ratio of 3:1. Glial tumours comprised the largest category 75.8% and among all the glial tumours astrocytomas were the commonest comprising 44.8% of all intracranial neoplasms. Medulloblastoma 15.5% ranked the second most common brain tumour followed by ependymoma, 10.3%. Out of 116 cases, 51 [44%] were supratentorial and 65 [56%] infratentorial in location. In infratentorial region 38% of the tumours were in the cerebellum, 9.5% in the brain stem and 8.5% in the fourth ventricle. The majority of these tumours were astrocytomas 32 cases [49.2%], WHO I/II, medulloblastoma 18 cases [27.6%] and ependymoma 6 cases [9.2%]. In supratentorial region out of 51 cases the most common tumour was astrocytoma, 20 cases [39.2%], high grade WHO III/IV, ependymoma, mixed glial tumours and PNET 5.1% each. The less common tumours included craniopharyngiomas, choroid plexus papilloma and ganglioneuroblastoma 3.4% each. Choroid plexus carcinoma, haemangioma, non Hodgkin's lymphoma, oligodendroglioma and ganglioneuroma 1.7% each. Infratentorial location was more common between the age of 3-11 years [58.2%], by contrast in the under 3 years old a supratentorial location was more frequent. Paediatric brain tumours are more common in infratentorial region and astrocytomas are the most common tumours. Supratentorial astrocytomas are more likely to be high grade than infratentorial. Males are affected more than females

Intra-abdominal tumors in children

The objective of the present study was to observe the histopathological pattern of intra-abdominal tumors in children less than 16 years. Design: This is a descriptive study. Place and Duration of Study: The study was carried out at the Department of Histopathology, Children's Hospital and the Institute of Child Health and King Edward Medical College, Lahore, over a period of 5 years, from January 1997 to December 2002. Subjects and The histopathological and demographic data of 264 intra-abdominal tumors of both the sexes under 16 years of age was collected and analyzed to determine the various morphological types of intra-abdominal tumors in relation to age and sex. Neuroblastoma was the most common tumor constituting 29.6% of all cases, followed by Wilms' tumor [25.1%]. Others were non-Hodgkins lymphomas [15.5%], germ cell tumors and hepatoblastoma [9%] each, rhabdomyosarcoma [4.4%], hepatocellular carcinoma [1.4%] and miscellaneous [6%]. Majority of the patients [77.2%] were under 5 years of age. The male to female ratio was 1.1:0.9. Intra-abdominal tumors are more common in males. Most of the tumors were noted in children less than 5 years of age

Analysis of the liver biopsies: a histopathological study

To evaluate the spectrum of childhood liver diseases. Design: A department-based retrospective study. Place and Duration of Study: It was carried out at the department of Pathology, Children's Hospital and the Institute of Child Health, Lahore, over a period of two years [October 1998 to October 2000]. Subjects and The histopathological data of total 50 percutaneous needle biopsies, wedge biopsies and partial hepatectomy specimens of both the sexes between 1 month - 16 years age were collected and compared with the findings reported from centers in other parts of the country. The commonest cause of hepatomegaly was hepatic inflammatory disorder in 43 cases [29%] followed by biliary atresia in 40 cases [27%]. Other causes of hepatomegaly were hepatic neoplasia 11 cases [7.3%], cirrhosis 10 cases [7%], glycogen storage disease 10 cases [7%] and fatty change in 8 cases [5.3%]. Hepatic diseases are quite common in paediatric population in Pakistan and percutaneous needle liver biopsy is an important and helpful diagnostic procedure in undiagnosed cases of hepatomegaly