Histopathological patterns in childhood steroid resistant nephrotic syndrome

To determine the frequency of various histopathological lesions in children with steroid resistant nephrotic syndrome [SRNS] presenting to the Children's Hospital and the Institute of Child Health, Multan. Retrospective observational study. This study was conducted at the Department of Paediatric Nephrology, The Children's Hospital and The Institute of Child Health, Multan, Pakistan from October 2005 to December 2012. Medical record of 152 children with SRNS, who were biopsied was reviewed all SRNS patients, both initial steroid resistant and late non-responders were included in the study out of the total 152 patients, 98[64.5%] were males and 54[35.5%] females, with a male to female ratio of +/- 1.8: 1. Mean age and standard deviation of patients was micro 8.11 +/- 3.58 years with age range of 1 to 15 years. Histopathological spectrum showed focal segmental glomerulosclerosis [FSGS] as the commonest [59; 38.81%] lesion followed by mesangioproliferative glomerulonephritis [MesPGN] [40; 26.31%], minimal change disease [MCD] [35; 23.02%] and mesangiocapillary glomerulonephritis [MCGN] [13; 08.55%]. Four [2.63%] patients had membranous nephropathy. One patient of renal amyoidosis was also diagnosed on renal biopsy. Overall FSGS was the commonest lesion followed by MesPGN, MCD, and MCGN. IgMN was an associated finding in 25% cases of MesPGN. FSGS was significantly more common among children >10 years. MCD was significantly more common among children 1-5 years. MesPGN and MCGN were significantly more common among children >5 years

Significance of bone marrow biopsy in diagnosis of pediatric diseases; one year experience at a single pediatric hematology/oncology center

Despite the recent advances in the field of hematology in the form of molecular studies and immunophenotyphing, morphological study of bone marrow remains a corner stone in the diagnosis of pediatric hematological diseases. It is also helpful in the diagnosis of many non-hematological diseases. This study is unique in a sense that bone marrow biopsy procedure and morphology reporting were done by a pediatrician trained in clinical hematology. To describe the indications of bone marrow biopsy and frequency of pediatric hematological and non-hematological diseases on morphological basis. This study was conducted at the Pediatric Hematology/Oncology Department, The Children Hospital and the Institute of Child Health Multan from January 2010 to December 2010. This study was conducted on children whether admitted in hematology / oncology ward or referred from various departments of this hospital. A Performa was filled for each patient including detailed history, clinical examination, base line investigation reports and provisional diagnosis. All bone marrow biopsies were performed from posterior iliac spines according to standard protocol for this procedure. Biopsy samples were stained with Leishman stain for morphological study. Bone marrow biopsy report was issued with detailed morphology, morphological diagnosis and suggestion for further investigations e.g. immunophenotyping. Patients age range was 3 months to 13 years with Male: Female = 1:1. Out of 100 bone marrow biopsy reports, disease distribution was acute lymphoblast leukemia [ALL] 30%, acute myeloid leukemia [AML] 7%, lymphoma infiltration 3%, aplastic anemia 18%, idiopathic thrombocytopenic purpra [ITP] 7%, storage disorders 11%, hemolytic anemia 5%, congenital dyserythropoitic anemia [CDA] 2%, red cell aplasia [RCA] 2%, refractory anemia with excessive blasts [RAEB] 2%, nutritional anemia 3%, malaria 3%, reactive changes 5% and normal morphology 2%.In children, acute leukemia is a leading hematological disease on bone marrow morphology followed by aplastic anemia and various non-hematological diseases. Despite availability of advanced diagnostic facilities, bone marrow biopsy is still a useful diagnostic test in many childhood diseases

Outcome of childhood acute lymphoblastic leukaemia after induction therapy-3 years experience at a single paediatric oncology centre

Acute lymphoblastic leukaemia [ALL] is the most common paediatric malignancy. It represents 25% of all childhood cancers and approximately 75% of all cases of childhood leukaemia. A sharp peak of ALL incidence is observed at 2-5 years of age. Objective was to see the bone marrow remission pattern at the end of induction therapy in paediatric ALL patients in our setup. It was a Descriptive case series and conducted at Paediatric Oncology Department, Children Hospital complex Multan from December, 2005 to December, 2008. Thirty-eight paediatric ALL patients were included in the study. Diagnosis was based on history, examination, blast cells count on peripheral blood film and bone marrow biopsy and immunophenotyping on peripheral blood/bone marrow aspirate. According to UK ALL 2003 protocol all patients were given 4-drug induction therapy, i.e., vincristine, prednisolone/dexamethasone, L-aspiragenase and daunomycin. Bone marrow biopsy was repeated at day 28 of induction therapy and remission pattern was seen. Out of 38 Patients, 26 [68%] were males. Age range was between 2-12 years [Mean 5.4 years]. Bone Marrow Biopsy was done in 38 [100%] and Immunophenotyping in 34 [89%] patients. At day 28 of induction therapy, 28 [74%] patients went into complete remission [<5% blast cells in bone marrow], 2 [5%] into partial remission [5-25% blast cells in bone marrow] and 1 [3%] was not in remission [>25% blast cells in the bone marrow]. Seven [18%] patient died due to febrile neutropenia and sepsis during the course of induction therapy. ALL in children is curable with effective chemotherapy. Remission can be achieved in most of these patients after induction therapy. However outcome can be improved with effective control of infections