Congenital proptosis secondary to orbital teratoma. Clinicopathological study.

CASE REPORT: A newborn male with right proptosis secondary to a retroocular mass. Ophthalmological examination also showed corneal ulcer and perforation, iris hernia, total ophthalmoplegia, chemosis and eyelid retraction. The histopathology diagnosis was mature teratoma. DISCUSSION: Teratomas are tumors composed of a mixture of mature tissues consisting of 3 germ layers. Congenital teratomas of the orbit are very rare and should be included as a possibility in cases with a primary tumor in the orbit.

Juvenile osteopetrosis: effects on blood and bone of prednisone and a low calcium, high phosphate diet.

Four children with juvenile osteopetrosis are described who were treated with a combination of prednisone and a low calcium, high phosphate diet. One of the children, treated as a neonate, achieved complete clinical and radiological remission from the disease after nine months, at which point treatment was stopped. There have been no signs of recurrence for two years. Two who did not start treatment until over 24 months of age have shown a good clinical and radiological response but have remained on treatment for six years. The fourth child started treatment at 6 months and showed a good clinical response, but x ray films showed no change nine months later. He was then lost to follow up, stopped treatment, and died two years later of a septicaemia. These patients provide further evidence for the efficacy of steroids in juvenile osteopetrosis, and the combination with the low calcium, high phosphate diet described offers a potentially effective alternative treatment to marrow transplantation, both for the haematological and skeletal complications of the disorder.

[Iatrogenic disease in hemophilia and Von Willebrand's disease]. / Yatrogenia en hemofilia y enfermedad de Von Willebrand.

From a group of 115 children with hereditary haemorrhagic disease, nine suffered avoidable accidents or incidents during their treatment, these nine patients represent 8.6% of the cases. The observed complications included a giant cervical hematoma and hemomediastinum after a puncture of the internal jugular vein; an encephalic lesion associated with descompressive craneotomy; a hemophilic pseudo cyst associated with inappropriate treatment of a tibial fracture; acute bleeding and shock after surgery for tonsillectomy and circumcision; subdural hygroma after a subdural puncture; giant hematoma and acute anemia secondary to a venous dissection; permanent dyslexia after inappropriate puncture; giant hematoma and acute anemia secondary to a venous dissection; permanent dyslexia after inappropriate management of intracranial bleeding; bleeding and acute anemia after surgical drainage of a prepucial hematoma and a joint hematoma of the left knee after synovectomy and application of a prosthesis.

Garcia's disease. Cyclic thrombocytopenic purpura in a child and abnormal platelet counts in his family.

After a critical study, a splenectomy was performed in a 6-year-old boy with chronic thrombocytopenic purpura. Failure of surgery and immunosuppressive therapy prompted new investigations which led to the discovery of a cyclic thrombocytopenic purpura related to a periodic variation in maturity of megakaryocytes. The patient's platelets were morphologically and functionally normal and it was not possible to demonstrate neither immunological mechanism, nutritional deficiency, influence of the environment nor consumption or excessive destruction of platelets. Cyclic thrombocytopenia was detected in the father and also cyclic variations in platelet counts from normal values to over 1,000 x 10(9)/1 in 4 of 9 siblings. In view of these findings. The abnormal condition in this family was named Garcia's disease.

[Disseminated intravascular coagulation, 113 cases, including 17 children with fulminant purpura]. / Coagulación intravascular diseminada. 113 casos que incluyen 17 niños con púrpura fulminante

Between 1964 and 1971, 113 children with the syndrome of disseminated intravascular coagulation were studied at the Hospital Infantil de México, including 17 cases with the diagnosis of fulminant purpura. Comparison was established with reports from foreign institutions. The following conclusions are offered: Fulminant purpura appears after a period of latency following the causal disease. It is not caused by septicemia; shows a clear picture in infants, preschool and school children, with letality index of 17.7%. In Mexico, DIC was caused by infection in 88% of the cases and in 68% of them, the infection started in the digestive tract. The diagnosis of fulminant purpura is based on the clinical picture, while in DIC, the diagnosis must also be based on laboratory tests unless one half of the diagnoses be missed.

[Effect of environmental humidity on the epistaxis of patients with acute thrombocytopenia]. / Efecto de la humidificación del ambiente sobre las epistaxis en pacientes con trombocitopenia grave

The effect of humidification of the environment was studied on the frequency and severeness of spistaxes in a group of 16 children. A control group was composed of 18 children with the idea that they were similar, but the hematologic conditions of the latter group were more unfavorable. A decrease of epistaxis was not found; there was a minimal prolongation of the days between one and the next nosebleeding picture. A definite decrease in the severeness of bleeding was found in the second group as judged by a lesser necessity of transfusions.

[The clinical problem of hereditary hemorrhagic diseases developing prolonged bleeding time]. / El problema clínico de las enfermedades hemorrágicas hereditarias que evolucionan con tiempo de sangrado prolongado

Thirty-two patients with hereditary hemorrhagic diseases and a platelet functional abnormality were set apart from our group of patients with hereditary hemorrhagic diseases, and their symptoms, signs and hematological examinations were collected; the initial events and the age of the patients when they were obsserved, the main hemorrhagic manifestations during their clinical course, the clinical severity of the disorders, the survival of the patients and the laboratory test for hemostasis useful to make the diagnosis, were evaluated. In reference to bleeding time, thirty patients had abnormal bleeding time, but the other two had normal bleeding time.

Topography of the sex chromatin in vaginal histiocytes.

Routine vaginal smears with good numbers of histiocytes with kidney shaped morphology of their nuclei were selected for the study of sex chromatin topography. A variable distribution of the sex chromatin was found in 125 nuclei studied: polar in 57.4 percent, ventral in 21.9 percent and dorsal in 20.7 percent of the nuclei. No relationship was found between sex chromatin and cytoplasmic morphology and phagocytic activity.

Unique phenotypic expression of glucosephosphate isomerase deficiency.

Studies of a Mexican kindred present evidence for a unique phenotype of erythrocyte glucosephosphate isomerase, GPI Valle Hermoso. The proband was apparently the homozygous recipient of a mutant autosomal allele governing production of an isozyme characterized by decreased activity, marked thermal instability, normal kinetics and pH optimum, and normal starch gel electrophoretic patterns. Unlike previously known cases, leukocyte and plasma GPI activities were unimpaired. This suggested that the structural alteration primarily induced enzyme instability without drastically curtailing catalytic effectiveness, thereby allowing compensation by cells capable of continued protein synthesis. Age-related losses of GPI, however, were not evident by density-gradient fractionation of affected erythrocytes.