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.

[Iatrogenic disease in hemophilia and Von Willebrand's disease].

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.

[Disseminated intravascular coagulation, 113 cases, including 17 children with fulminant purpura].

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.

[Effect of environmental humidity on the epistaxis of patients with acute thrombocytopenia].

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.

[The clinical problem of hereditary hemorrhagic diseases developing prolonged bleeding time].

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.

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.