A 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.

Etiology and genetic factors in clefts of lip and/or palate reported at children's hospital, Lahore, Pakistan.

The etiology of cleft lip (CL) and/or cleft palate (CP) has been extensively studied in industrialized countries and is suggested to be heterogeneous with increasing evidence that both genetic and environmental factors are operating. To evaluate this assertion in a developing country like Pakistan, a case finding cross-sectional study was completed from 1(st) July 2010 to 31(st) May 2011 for 100 cases of CL and/or CP referred to the Genetic Clinic of the Children's Hospital, Lahore, Pakistan. A clinical examination followed by necessary diagnostic work-up was completed for each case. The cause of CL and/or CP was clear in 18% of the children (n = 18). Environmental causes were found in 6 children (four mothers developed hyperthermia during the 2(nd) month of gestation, one mother was diabetic, and one mother was a known case of epilepsy and took sodium valproate throughout her pregnancy). Six children were suffering from known genetic malformation syndromes (each with Jarcho-Levin syndrome, Oral-Facial-Digital syndrome type XI, Oral-Duplication syndrome, Kabuki syndrome, Fronto-nasal dysplasia and Nager syndrome). Novel chromosomal aberrations were identified in 2 children. In 82% of the children (n = 82) the cause of oro-facial clefts remained unknown. Impact of gender and consanguinity on the development of CL and/or CP was also studied. Prevalence of CP was significantly more among female children as compared to that in males (P < 0.05). Associated anomalies were present in 18% of the cases, anomalies of the craniofacial region being the most common. These findings were compared with regional and international studies.

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.

Hepatic tumours in childhood: an experience at the Children Hospital and Institute of Child Health, Lahore.

OBJECTIVES: In this report, we share our experience about the common types of childhood hepatic tumours during 10 years (2001-2010) and compare them with other studies. METHODS: During 10 years (2001-2010), all the hepatic tumours of childhood received at Pathology Department of the Children Hospital and Institute of Child Health, Lahore Pakistan are recorded. This includes both resected specimens and biopsies. All the slides were reviewed and the pathologic diagnosis was confirmed. RESULTS: We diagnosed 48 liver tumour cases in children (below 18 years of age). Among these tumours, 39 (81.25%) were malignant. Male to female ratio was 2:1. Hepatoblastoma was the most common liver tumour in this age group accounting for 69.23% of all malignant tumours (27 cases). The second most common primary tumour was hepatocellular carcinoma diagnosed in six patients (15.38%). Other malignant tumours were undifferentiated embryonal sarcoma and biliary rhabdomyosarcoma. Benign tumours included mesenchymal hamartoma, infantile haemangioendotheloima, hemangioma and benign cyst. There were also three metastatic tumours during these 10 years. In one case there was tumour necrosis only and as such no definitive diagnosis was rendered. CONCLUSION: The spectrum of hepatic tumours in children is different from that found in the older age group and most of them are malignant.

Lymph nodal infarction simulating acute appendicitis.

A number of diseases can present as acute right iliac region pain. Lymph node infarction, located adjacent to the cecum, mimicking acute appendicitis in a 13-year-old boy is presented here.

Role of biopsy in pediatric lymphadenopathy.

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: 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.

An updated audit of fine needle aspiration cytology procedure of solitary thyroid nodule.

This study was conducted to see the sensitivity, specificity, and accuracy of fine needle aspiration cytology (FNAC) for solitary thyroid nodules and to compare our experience with that of other regions of the world. It was a prospective cross sectional study conducted on 76 thyroid samples submitted and reported at the Department of Pathology, King Edward Medical University, Lahore. There were 65 (85.5%) females and 11 (14.5%) males. Male to female ratio was 1:5.9. Ages of the patients ranged from 10 to 60 year with mean age 35.37 +/- 12.17. Thirty-three (43.42%) samples were indeterminate. There were 30 cases (39.47%) of benign lesions, comprising of colloid goiter, follicular adenoma, and diffuse hyperplasia. The final reports of these cases were almost the same. On FNAC, 13 cases were declared as malignant (6 cases) or suggestive of malignancy (3 cases) or suspicious for malignancy [4 cases (5.26%)]. Only 9 cases (11.84%) were clearly committed as malignant lesions, comprising of papillary carcinoma, anaplastic carcinoma and suggestive of follicular carcinoma. Comparison of malignant cases on histopathology (14 cases) was close to that of FNAC (13 cases). After comparison of FNAC results with histopathology, overall sensitivity of FNAC was found to be 90%, specificity 87.5%, and accuracy 87%, while positive predictive value (PPV) was 93% and negative predictive value (NPV) was 79.5%. In conclusion, we recommend this procedure in the light of views of other experts as a primary investigation of thyroid lesions. We strongly recommend the suggestion that in a patient with one or more thyroid nodule, FNAC should be advised for every patient for exclusion of cancer. We will also encourage the clinicians to embrace this procedure in the initial management of patients. As FNAC is inexpensive, sensitive, specific, and an accurate procedure it should be adapted as an initial investigation of thyroid diseases in all tertiary hospitals in developing countries like Pakistan.

Intra-abdominal tumors in children.

OBJECTIVE: 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 METHODS: 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. RESULTS: 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. CONCLUSION: Intra-abdominal tumors are more common in males. Most of the tumors were noted in children less than 5 years of age.

Morphological pattern and frequency of intracranial tumours in children.

OBJECTIVE: 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 METHODS: 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&E stained sections of paraffin embedded tissues. Special stains and immunohistochemistry were performed whenever indicated. Autolyzed biopsies and previously reported biopsies were not included. RESULTS: 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. CONCLUSION: 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.