Paraneoplastic cushing syndrome due To Wilm's tumor

Paraneoplastic syndromes are rare disorders that are triggered by an altered immune system response to neoplasm. Paraneoplastic syndromes may be the first or the most prominent manifestations of cancer. Wilm's tumor is the most frequent pediatric renal malignancy and usually presents with abdominal mass. Unusual presentations like acquired von Willebrand disease, sudden death due to pulmonary embolism and Cushing syndrome have been described in the literature. Cushing syndrome, as the presenting symptom of a malignant renal tumor in children, is a very rare entity. Few case reports are available in the literature exploring the option of preoperative chemotherapy as well as upfront nephrectomy. We report a rare case of paraneoplastic Cushing syndrome due to a Wilm's tumor. Based on gradual decrease of postoperative weight, blood pressure, serum adrenocorticotropic hormone, and plasma cortisol levels, along with histological confirmation of Wilm's tumor, paraneoplastic Cushing syndrome due to Wilm's tumor was confirmed

Comparison of presentation and outcome in 100 pediatric Hodgkin lymphoma patients treated at children hospital, Lahore, Pakistan and royal Marsden hospital, UK

Objective: To compare differences in demographics and outcomes in childhood Hodgkin lymphoma [HL] presenting at the Children's Hospital Lahore [CHL], and Royal Marsden Hospital [RMH], UK

Study Design: An observational comparative study

Place and Duration of Study: From January 2011 to February 2012 at CH, Lahore and from October 2008 to February 2012 at RMH, UK

Methodology: Consecutive HL patients [50 from each hospital] were inducted. Data regarding age, gender, staging, histopathology and outcome were analysed. Clinical and pathological staging done according to Ann-Arbor and World Health Organization classification. Treatment duration was 6-8 months. They were followed for 6 months post-treatment. Frequencies of variables were noted and compared. Chi-square test was used for determining significance

Results: Patients from Children's Hospital, Lahore were younger [mean 7.9 years] with male predominance [n=42, 84%]. Histopathology showed Mixed Cellularity [MC] in 32 [64%], Nodular Sclerosis [NS] in 5 [10%], Lymphocyte Rich in 4 [8%] and lymphocyte depleted in 1 [2%], nodular lymphocyte predominant [NLP] in 1 [2%] each. Majority presented in stage IV [n=25,50%], or stage III [n=20,40%]. Constitutional B symptoms were present in 37 [74%]. Bone marrow involvement observed in 23 [46%]. Remission was achieved in 42 [84%] patients; 2 [4%] relapsed, 4 [8%] expired and 2 [4%] left against medical advice. In contrast, RMH patients were older [mean 11.8 years.] and 30 [60%] were males. NS [n=40,80%] and NLP [n=6,12%] types were predominant. Two [4%] patients were in stage I, 27 [54%] in stage II, 12 [24%] in stage III and 9 [18%] presented in stage IV. Fourteen [28%] had B-symptoms. None had bone marrow disease. Event free survival was 46 [92%]. Four [8%] patients relapsed. Three responded to second line therapy and one relapsed postautologous transplant

Conclusion: Significant differences were observed in age at presentation, stage, histopathology and extent of bone marrow involvement between the groups. Of interest is the bone marrow involvement in stage IV patients in Pakistan. Delayed diagnosis account for advanced stage but difference in pathological subtype needs further study

Ollier's disease with myelodysplastic syndrome

Ollier's disease also known as enchondromatosis is a rare skeletal disorder that is usually sporadic, non-hereditary, and characterized by abnormal bone development [skeletal dysplasia]. While this disorder may be present at birth [congenital]; it may not become apparent until early childhood with more obvious symptoms, such as deformities or improper limb growth. It carries high risk of skeletal, visceral and brain malignancy seen in approximately 25% of patients. Occurrence of Ollier's disease with myelodysplastic syndrome has never been reported in the literature. The different types of myelodysplastic syndromes are diagnosed based on certain changes in the blood cells and bone marrow characterized by one or more cytopenias despite a relatively hypercellular bone marrow. We hereby report the case of a 14 years boy who presented with painless finger swelling and hepatosplenomegaly. Radiological and bone marrow findings confirmed the diagnosis of Ollier's disease with Refractory Anemia and Excess Blasts [RAEB-1]

Disproportionate short stature with multisystem involvement - Ellis-van Creveld syndrome

Ellis-van Creveld syndrome is a rare form of mesenchymal - ectodermal dysplasia. It is an autosomal recessive disorder characterized by disproportionate short stature, postaxial polydactyly, ectodermal dysplasia and congenital heart defect. This case report presents two cases with classical clinical findings along with some unusual features including rhizomelic limb shortening, global developmental delay and bilateral lens subluxation not reported previously

Comparison of three prognostic scores [PRISM, PELOD and PIM 2] at pediatric intensive care unit under Pakistani circumstances

To compare the performance of the Pediatric Risk of Mortality [PRISM], the Pediatric Index of Mortality 2 [PIM 2] and Pediatric Logistic Organ Dysfunction [PELOD] scores at general pediatric intensive care unit in a developing country setting, investigating the relation between observed and predicted mortality. A contemporary cohort study was undertaken at Pediatric Intensive Care Unit [PICU], Children's Hospital, Institute of Child Health, Lahore, Pakistan. 131 consecutive admissions fulfilling the inclusion criteria were enrolled in the study. PRISM, PIM 2 and PELOD calculations were performed as set out by original articles, using the published formulae. Statistical analysis included Standardized Mortality Rate [SMR], Hosmer Lemeshow goodness of fit test, receiver operating curve [ROC] characteristics and Spearman's correlation test. 139 patients were admitted to PICU. 38 presented exclusion criteria. 29 [28.7%] patients died. Estimated mortality was; PRISM: 19.7[19.5%], PIM: 21.01[20.5%] and PELOD: 18.4[18.3%]. SMR was 1.47 [SD +/- 0.19], 1.4 [SD +/- 0.19] and 1.57 [SD +/- 0.19], respectively. PRISM had better calibration [x[2] = 7.49, p = 0.49] followed by PIM 2 [x[2] = 9.65, p = 0.29]. PIM 2 showed best discrimination with area under ROC = 0.88 [0.81-0.94] followed by PRISM 0.78 [0.67-0.89] and PELOD 0.77 [0.68-0.87]. Spearman's correlation r between PRISM and PIM 2 returned 0.74 [p < 0.001]. PRISM as well as PIM 2 is validated for PICU setting in Pakistani circumstances. PELOD performed poorly. PIM 2 has advantages over PRISM for stratification of patients in clinical trials

Histiocytosis in children: Five years experience at a tertiary care hospital

Histocytosis is a diverse group of illness of monocyte- macrophage cell lineage. Diagnostic criteria and management protocols are not precisely defined due to cases. Study was conducted to review the clinical features at admission and outcome of histiocytosis following chemotherapy [DALHAX 83 protocol was used]. It was a retrospective study, carried out from March 2000 to February 2005, at department of hematology- oncology, The Children's Hospital and the Institute of Child Health, Lahore. From 01-03-2000 to 28-02-2005, total 36 cases with histocytosis disorders were admitted. It comprises 2% of total admissions, class 1 langerhan cell disease was the most common [24/36], followed by class II [8/36] and class III [4/36] cases among this group of illness. Fever and hepatosplenomegaly was present in all classes. Soft tissue swelling, jaundice and lymphadneopathy were also seen in class I, II and III histiocytosis respectively. Nineteen patients died/ left treatment and absconded with incomplete treatment. Seventeen patients responded to chemotherapy again. Histiocytosis class I is a chemosensitive disease. If the functional deterioration of the involved organ has not set in, class I disease has good prognosis

Frequency, risk factors and outcome in admitted Neonates presenting with hypothermia

Neonatal hypothermia is a significant cause of morbidity and mortality, particularly in preterm and low birth weight babies especially in developing countries. This study was performed to describe the frequency, associated risk factors and outcome of neonatal hypothermia in admitted cases. This was an observational study carried out in prospective manner. Study population consisted of 500 neonates admitted to neonatal unit of the Children's Hospital, Lahore during the months of January and February 2006. Various variables were recorded in a predesigned proforma and outcome was analyzed. The overall frequency of hypothermia was 34.8%. It was significantly associated with low birth weight [p<0.05], prematurity [p<0.0.5], shifting from outside Lahore [p<0.05], and history of birth asphyxia [p<0.05]. Mortality in hypothermic neonates was significantly higher than the non hypothermic ones [p<0.05]. The risk of mortality was recognized to be higher in hypothermic than the non hypothermic admitted neonates