No abstract available.
Sarcoma*

No abstract available.
Endoderm* ; Endodermal Sinus Tumor*

Wilms' tumor is one of the most common primary malignant tumors of the kidney during infancy and childhood and is known to be originated from the primitive cells of metanephric blastema. It presents difficulties when encountered in deciding the presence of anaplasia or in differentiating it from other renal tumors of childhood with different biologic behavior because of its diverse histologic patterns and varying degrees of differentiation. Evaluation of clinical and histopathologic features in terms of prognostication was done of 32 cases of Wilms' tumor which were surgically resected and diagnosed in the period from January 1979 through June 1992. Immunohistochemical reaction for cytokeratin, vimentin, actin and desmin was also analysed on all cases of Wilms' tumor in conjunction with clear cell sarcoma of the kidney(CCSK), malignant rhabdoid tumor of the kidney(MRTK) and congenital mesoblastic nephroma(CMN) to assess the validity of immunohistochemistry in differentiating Wilms' tumor from these renal tumors. Twenty four(75%) cases were diagnosed before the age of 5 and 40.7% were under 2 years old. Mixed type was most common(62.5%), followed by epithelial, blastemal and stromal predominant type in descending order of frequency. Anaplasia was observed in 3 cases(9.4%), two of which were epithelial predominant type and one blastemal predominant type. Treatment modality and presence of anaplasia were significantly correlated with 5 year survival rate of patients. Immunohistochemical stain revealed that all epithelial component of Wilms' tumor were positive for cytokeratin and 56.3% of Wilms' tumor had blastemal component which were positive for both cytokeratin and vimentin. Twenty cases(62.5%) of Wilms' tumor had blastemal component which were positive for cytokeratin with a proportion of more than 5% of reactive cells. Stromal component of Wilms' tumor generally did not show differentiation into the specialized type of tissue and all revealed positive reactions for vimentin among which some revealed positive reactions for actin. Only 3 out of 6 cases with rhabdomyoblastic differentiation were positive for desmin. CCSK, MRTK and CMN which have different biologic behavior and treatment modality compared to Wilm's tumor showed positivity only for vimentin and/or actin. In summary, treatment modality and presence of anaplasia are significantly correlated with patients' survival and the immunohistochemical stain for cytokeratin is very helpful in confirming the presence of blastemal component and useful in the differential diagnosis of Wilms' tumor from other kinds of pediatric renal tumors.
Child ; Male ; Female ; Humans ; Diagnosis, Differential

A pediatric patient with combined primary thrombocytopenic purpura and acquired hemolytic anemia (Evans syndrome), whose condition did not respond to treatment with prednisolone, has enjoyed long-term remission following a period of treatment with 6-Mercaptopurine.
6-Mercaptopurine/therapeutic use* ; Anemia, Hemolytic, Autoimmune/drug therapy* ; Child ; Human ; Male ; Purpura, Thrombocytopenic/drug therapy* ; Syndrome

No abstract available.
Intermittent Positive-Pressure Ventilation* ; Tetanus*

No abstract available.
Rhabdomyosarcoma*

No available abstract.
Leukemia, Myeloid, Acute*

No abstract available.
Heart Septal Defects, Ventricular*

Coagulation involves the regulated sequence of proteolytic activation of a series of proteins to achieve appropriate and timely hemostasis in an injured vessel. In the non-pathological state, the inciting event involves exposure of circulating factor VIIa to extravascularly expressed tissue factor, which brings into motion the series of steps which results in cell based model of coagulation. In the new concepts of coagulation system, initiation, amplification and propagation steps are involved to converse of fibrinogen to fibrin. The precisely synchronized cascade of events is counter-balanced by a system of anticoagulant mechanisms. Developmental hemostasis refers to the age-related changes in the coagulation system that are most marked during neonate and childhood. An understanding of these changes in crucial to the accurate diagnosis of hemostatic abnormalities in neonate and children. This review aims to elucidate the main events within the coagulation cascade as it is currently understood to operate in vivo, and also a short review of the anticoagulants as they relate to this model. Also this paper describes the common pitfalls observed in the clinical data related to the coagulation system in neonate to children.
Anticoagulants ; Child ; Factor VIIa ; Fibrin ; Fibrinogen ; Glycosaminoglycans ; Hemostasis ; Humans ; Infant, Newborn ; Polymethacrylic Acids ; Proteins ; Thromboplastin

PURPOSE: The human chromosome 9p21 region that is a frequent site of deletions and rearrangements in many tumor types including leukemias implied the existence of a tumor suppressor gene within 9p21 which is involved in tumor formation. CDKN2 (p16) gene is located in the same chromosomal region. The loss of CDKN2 function is probably one of the most common genetic alterations and is now thought to play a key role in leukemogenesis. We examined the frequency of the point mutation of CDKN2 gene by analyzing the DNA sequence and demonstrated the prognostic implication of mutations of CDKN2 gene in childhood acute leukemia. METHODS: We investigated the prevalence of the point mutation in thirty patients with 20 cases of acute lymphoblastic leukemia (ALL) and 10 cases of acute myeloid leukemia (AML). The point mutation of CDKN2 gene was analyzed in a PCR generated DNA sequencing technique. RESULTS: There was no point mutation in exon 1 of CDKN2 gene. A missense mutation (G-- Arginine ; Base Sequence ; Chromosomes, Human ; Codon ; Exons ; Genes, p16 ; Genes, Tumor Suppressor ; Glycine ; Humans ; Leukemia* ; Leukemia, Myeloid, Acute ; Mutation, Missense ; Point Mutation ; Polymerase Chain Reaction ; Precursor Cell Lymphoblastic Leukemia-Lymphoma ; Prevalence ; Sequence Analysis, DNA ; T-Lymphocytes