Simultaneous onset of alopecia areata and idiopathic thrombocytopenic purpura: A potential association?

Alopecia areata (AA) has been associated with a variety of autoimmune diseases. Immune thrombocytopenic purpura (ITP) is an autoimmune disease marked by isolated thrombocytopenia. We present a young girl with the simultaneous onset of AA and ITP. To the best of our knowledge, this association has not previously been reported. We hypothesize that ITP may be one of many autoimmune diseases associated with AA via antibodies that cross-react between platelet membrane glycoproteins and follicular glycoproteins.

Porcine factor VIII (Hyate: C®): in vitro stability, microbiological safety and effect of heparin.

The in vitro stability of porcine factor VIII (PF VIII) was evaluated when it was reconstituted with sterile water (PF VIIISW ) to ≈ 30 U PFVIII mL(-1) , as per the manufacturer's recommendations, and stored in plastic syringes at room temperature, with and without heparin and at four different dilutions. PF VIII was prepared antiseptically without laminar airflow and remained sterile at room temperature for 1 week. PF VIIISW retained at least 88% of baseline activity for 48 h and 74-86% for 72 h. Addition of heparin 1 unit mL(-1) solution resulted in a decrease in the stability of PF VIIISW to 72-74 % of baseline values by 24 h. Reconstituted PF VIIISW , further diluted with normal saline to 10-24 U PF VIII mL(-1) , retained 98% of baseline activity for 48 h and 84% of baseline for 72 h. PF VIII diluted to 6 U mL(-1) , however, retained 100% baseline activity for only 24 h, and declined to 71% and 64 % of baseline by 48 and 72 h, respectively. PF VIII reconstituted with normal saline, instead of sterile water, retained 90% or more of baseline activity for a minimum of 4 days. Once reconstituted, PF VIII appeared to be more stable at room temperature than when stored in the refrigerator. These in vitro stability studies confirm that PF VIII (30 U mL(-1) ) can be given effectively by prolonged continuous infusion, since it retains 88% baseline activity at room temperature in a plastic syringe for a minimum of 48 h, remains sterile and will maintain baseline PF VIII levels when further diluted with saline.

Epidural hematoma of a cauda equina in a child with hemophilia A.

PURPOSE: The presenting signs, treatment, and outcome of an epidural hematoma of the cauda equina in a child with severe hemophilia are reported for the first time. PATIENTS AND METHODS: A 20-month-old boy with severe hemophilia A (factor VIII <0.01 U/ml) presented with a 12-day history of refusal to stand and constipation of 5-7 days duration. He had normal deep tendon reflexes with normal sensation and withdrawal to pinprick of his lower extremities bilaterally. He stood on his right leg, but had inversion of his left foot and refused to bear weight on his left leg. MRI revealed an epidural hematoma of the cauda equina and a distended bladder. Factor VIII replacement therapy and lumbosacral laminectomy with evacuation of the hematoma resulted in recovery of a normal gait, but bladder dysfunction persisted for 11 months. Clean intermittent catheterization (CIC) was required until bladder function returned. RESULTS: Complete neurologic recovery occurred 11 months after presentation CONCLUSION: This case demonstrates the following points: (a) an epidural hematoma of the cauda equina in a child with severe hemophilia can present with neurologic findings that are as subtle as those seen in normal children; (b) CIC can be performed safely over an extended period without factor VIII replacement; and (c) complete recovery is possible, despite prolonged bladder dysfunction and a 12-day interval between the onset of symptoms and treatment.

Congenital leukemia: report of two cases.

Congenital leukemia is a rare disease in which a leukemic process is present at birth or immediately thereafter. The majority of cases presented in the literature were reported prior to the availability of contemporary immunophenotyping methods, and lineage assignment was often made on the basis of morphology alone. Congenital leukemias may be of various lineages, although, historically, monocytic and myelomonocytic congenital leukemias appear to be the most prevalent. We present two cases of congenital leukemia with detailed immunophenotypic and cytochemical characterization. One case is of the lymphoid lineage, and the second is of myelomonocytic lineage. Neither patient displayed trisomy 21.

Spontaneous hemorrhage associated with accidental brodifacoum poisoning in a child.

A 36-month-old child had spontaneous hemorrhage from her nose, mouth, and urinary tract, and a fall in hemoglobin of 20 gm/L (2 gm/dl). The prothrombin time and partial thromboplastin time were markedly prolonged with a decrease in the vitamin K-dependent factors. The child had ingested brodifacoum, a long-acting rodenticide. Prolonged follow-up and treatment with vitamin K were necessary.

Multiple chromosome aberrations in an infant with acute monoblastic leukemia.

A male infant was diagnosed at age 16 months with acute monoblastic leukemia. At diagnosis, studies of bone marrow revealed multiple chromosome aberrations: 48,XY,+8,+19,t(4;11). Chromosome studies have been repeated at remission and relapse over the course of his disease. To our knowledge, this combination of chromosome abnormalities has not been previously reported.

Chemotherapy-associated eccrine hidradenitis: neutrophilic eccrine hidradenitis reevaluated: the role of neutrophilic infiltration.

Two cases of chemotherapy-associated eccrine hidradenitis are presented. Previous cases have been labeled "neutrophilic eccrine hidradenitis" (NEH). Both cases reported herein involve neutropenic patients and are notable for the absence of neutrophils on histologic examination. Oncologists need to be aware of this newly described process, and this rash must be added to the differential diagnosis of the neutropenic oncology patient. The role of neutrophils in this disorder is discussed, and a new term, "chemotherapy-associated eccrine hidradenitis," is suggested for this unusual process.

Brainstem auditory evoked potentials in fullterm and preterm newborns with hyperbilirubinemia and hypoxemia.

Brainstem auditory evoked potentials (BAEP) were studied in 93 newborns (49 preterm and 44 fullterm) admitted to the neonatal intensive care unit. One group of 42 infants (28-42 wk CA) were considered at low risk for perinatal complications and served to establish the normal ranges of the BAEP parameters (wave I peak latency and wave I-V interwave interval). Two groups of high risk infants were studied during or shortly following clinically significant hyperbilirubinemia (31 infants) or hypoxemia (20 infants). Though statistically significant BAEP abnormalities were found in the high risk infants, these consisted predominantly of wave I latency prolongations in hypoxemic infants (p less than 0.05) indicating dysfunction in peripheral auditory processes. No significant increases in the wave I-V interval (central conduction time) were found in the high risk infants to suggest central auditory dysfunction in the brainstem. These findings are discussed in relation to previous studies of hyperbilirubinemic and hypoxemic infants.

Sequential changes in red cell glycolytic enzymes and intermediates and possible control mechanisms in the first two months of postnatal life in lambs.

The sequential changes in several glycolytic enzymes, glucose-6-phosphate dehydrogenase, glycolytic intermediates, and adenosine triphosphate, as well as intracellular pH and plasma inorganic phosphorus were followed simultaneously in eight lambs from birth to 2 months of age. The activities of all glycolytic enzymes and glucose-6-phosphate dehydrogenase were elevated at birth. The 2,3-diphosphoglycerate concentration increased markedly postnatally and was associated with a simultaneous increase in the concentrations of red cell glucose-6-phosphate and total triose phosphate and a decrease in intracellular pH. Inorganic phosphorus also increased and correlated with the 2,3-diphosphoglycerate concentration in the first 10 days of postnatal life. The content of red cell 3-phosphoglycerate, 2-phosphoglycerate, phosphoenolpyruvate, and ATP increased slightly. These results suggested increased glycolytic flux through the diphosphoglycerate mutase reaction which resulted in net synthesis of 2,3-diphosphoglycerate. The red cell total triose phosphate peaked and fell initially, followed by glucose-6-phosphate and 2,3-diphosphoglycerate suggesting inhibition of phosphofructokinase activity and a decrease in glycolysis secondary to decreased red cell intracellular pH. After 10 days of postnatal life all glycolytic intermediates fell simultaneously, which correlated with a decrease in activity of the glycolytic enzymes.

Red cell glycolytic intermediates and adenosine triphosphate in preterm infants on the first day of life.

Red cell glycolytic intermediates and ATP were evaluated in 47 appropriate for gestational age preterm infants on the 1st day of life who were divided into three groups on the basis of gestational age: 28-30, 31-33, and 34-36 wk. The results were compared to those previously obtained in term infants. The concentrations of glucose-6-phosphate, total triose phosphates, and ATP were significantly higher than in term infants but appeared to be appropriately elevated for the young mean age of the red cell population. The concentration of red cell 2,3-diphosphoglycerate (2,3-DPG) was significantly decreased when compared to term infants and was lowest at 28-30 wk gestation. The content of red cell 3-phosphoglycerate was increased in term infants and was inappropriately elevated for the age of the red cell population at 28-30 wk gestation. This pattern of glycolytic intermediates was suggestive of a young red cell population metabolizing at an increased glycolytic rate with increased flow through the phosphoglycerate kinase step rather than the 2,3-DPG bypass in "normal" preterm infants. Two preterm infants of 28-30 wk gestation with low red cell intracellular pH were also evaluated and had markedly decreased concentrations of red cell 2,3-DPG and ATP and all phosphorylated intermediates distal to the phosphofructokinase reaction, indicative of a cross-over at the phosphofructokinase step secondary to acidosis.(ABSTRACT TRUNCATED AT 250 WORDS)

Fetal erythropoiesis in juvenile chronic myelocytic leukemia.

Red cell enzymes, 2,3-diphosphoglycerate (2,3-DPG) and adenosine triphosphate (ATP), were evaluated in a 23-mo-old boy with juvenile chronic myelocytic leukemia (JCML) at the onset of his illness and 6 mo later during the accelerated phase. The activities of the age-dependent red cell enzymes, hexokinase, aldolase, pyruvate kinase, and glucose-6-phosphate dehydrogenase, were elevated, as were the concentrations of red cell 2,3-DPG and ATP, consistent with a young red cell population metabolizing at an increased glycolytic rate. The activities of the non-age-dependent enzymes, glyceraldehyde-3-phosphate dehydrogenase (G3PD), phosphoglycerate kinase, and enolase, were also increased to levels similar to or greater than those observed in term infants. As the illness progressed, the activity of red cell G3PD increased further, and phosphoglucose isomerase activity increased markedly. These results are consistent with the prior suggestion that JCML represents a reversion to "fetal" erythropoiesis.

Sickle cell disease and bronchial asthma.

A six-year-old girl with sickle cell disease and severe asthma was treated for status asthmaticus on several occasions. Sickle cell crises appeared to be precipitated by the asthmatic episodes. The interactions between these two disease processes are discussed and a treatment protocol for the rare patient with both of these diseases is proposed.

Red cell enzymopathies in the newborn. I. Evaluation of red cell metabolism.

In order to evaluate properly red cell metabolic data obtained in newborns with congenital hemolytic disorders, the unique metabolic characteristics and normal developmental changes that occur prenatally and postnatally are presented. The age-dependent red cell glycolytic enzymes (hexokinase, aldolase, pyruvate kinase) and glucose-6-phosphate dehydrogenase and most glycolytic intermediates are elevated at birth and at 11 to 12 months of age, consistent with the presence of a young red cell population the entire first year of life. However, certain red cell enzymes are elevated out of proportion to the age of the red cell population [phosphoglucose isomerase. glyceraldehyde-3-phosphate dehydrogenase, phosphoglycerate kinase (PGK), and enolase (ENO)] whereas others are decreased [phosphofructokinase (PFK), glutathione peroxidase, carbonic anhydrase, and others]. These metabolic characteristics are felt to be unique and representative of "fetal erythropoiesis." Activities of PGK and ENO decrease the PFK increases toward normal adult values beginning at eight to nine weeks of age. The concentration of glucose-6-phosphate steadily increases after birth and peaks at three to four weeks of age, at a time when PFK activity remains relatively unchanged, suggesting a relative block in glycolysis at the PFK step secondary to an enzyme with both decreased activity and altered kinetic properties (a "fetal" isozyme). Thus, evaluation of red cell enzyme and glycolytic intermediate data obtained in the first year of life should be related to the knowledge that a young red cell population is present and the characteristic unique metabolic red cell alterations described in cord blood persist beyond the immediate neonatal period.

Red cell metabolic alterations in postnatal life in term infants: possible control mechanisms.

Red cell glycolytic intermediates and enzymes in term infants in the first year of life were correlated with the fetal hemoglobin concentration (%F), intra- and extracellular venous pH, plasma inorganic phosphorus (Pi) and pyruvate kinase (PK) activity. Changes in the non-age-dependent enzymes phosphoglycerate kinase, enolase, and phosphofructokinase correlated most significantly with the postnatal decline in %F (P less than 0.001), not the age of the red cell population, as reflected in PK activity. The age-dependent enzymes, hexokinase and glucose-6-phosphate dehydrogenase, however, correlated well with PK activity (P less than 0.001). The concentration of glucose-6-phosphate did not correlate significantly with the postnatal decline in %F (P greater than 0.05) or PK (P greater than 0.10), but correalted significantly with the plasma Pi concentration (P less than 0.001). "Total triose phosphate" and 2,3-diphosphoglycerate did not correlate with Pi. It appears from these studies that an extracellular factor, Pi alters the pattern of glycolytic intermediates in term infants and that the postnatal changes in phosphoglycerate kinase, enolase, and phosphofructokinase are unique to the "fetal" red cell and reflect passage from fetal to "adult" erythropoiesis.

Red cell metabolic alterations in postnatal life in term infants: glycolytic intermediates and adenosine triphosphate.

Red cell glycolytic intermediates and adenosinetriphosphate were evaluated in term infants from birth to on year of age and compared to values obtained from normal adults and subjects wit a population of a similar mean cell age. The concentration of glycolytic intermediates, with the exception of phosphoenolypyruvate were elevated at birth when compared to normal subjects, consistent with a young mean red cell population. The mean levels of red cell glucose-6-phosphate, fructose-6-phosphate, and "total triose phosphate" were elevated on days 1 and 4 of life when compared to both red cells from normal adults and subjects with a similar young mean red cell age. Glucose-6-phosphate steadily increased in concentration, peaked at 3 to 4 wk of age, and then progressively decreased in value. Total triose phosphate declined to a mildly elevated concentration by 3 to 4 wk of age. The mean concentrations of 2,3-diphosphoglycerate and adenosine triphosphate were normal on day 1, increased on day 4, and then declined by 3 to 4 wk to normal values, until 5 to 6 months when both increased. The mean phosphoenolpyruvate concentration was decreased on day 1 of age when compared to red cells of a similar mean age, but this decrease was not significant (P greater than 0.05). The mean concentrations of 3-phosphoglycerate increased at 3 to 4 wk of age and remained elevated for cell age at 11 to 12 months but this increase was no statistically significant (P greater than 0.05). 3-Phosphoglycerate levels did not change significantly throughout the first year of life. At one year of age, all red glycolytic intermediates and adenosine triphosphate were elevated when compared to red cells from normal adults, but were comparable to those observed in subjects with a red cell population of a similar mean cell age, consistent with the persistence of a young red cell population throughout the first year of life.

Red cell metabolic alterations in postnatal life in term infants: glycolytic enzymes and glucose-6-phosphate dehydrogenase.

The activities of red cell enzymes enolase (ENO), phosphoglycerate kinase (PGK), phosphofructokinase (PFK), glucose-6-phosphate dehydrogenase (G-6-PD), hexokinase (HK), aldolase (ALD), and pyruvate kinase (PK) were followed sequentially in term infants from birth to one year of age. At birth, red cell PGK and ENO activities were disproportionately elevated when compared to both red cells with a similar mean cell age and those with a younger mean cell age; red cell PFK was significantly decreased. There was a progressive full in PGK and ENO activities and rise in PFK levels toward normal values in the first year of life. The most significant changes in PGK, ENO, and PFK appeared to begin at 8 to 9 wk of age. ENO and PFK activities stabilized at approximately 5 to 6 months of age at values compatible with mean cell age; mean PGK levels remained mildly elevated at 11 to 12 months. The age-dependent enzymes G-6-PD, PK, ALD, and HK were all elevated in term newborns. G-6-PD and ALD progressively decreased in activity during the first year of life. PK and HK decreased in activity until 8 to 9 wk when there was a secondary rise in mean activity. Mean red cell G-6-PD, PK, ALD, and HK levels remained mildly to moderately elevated at 11 to 12 months of life, suggesting the persistence of a relatively young red cell population throughout the first year of life.

Erythroid aplasia in juvenile rheumatoid arthritis.

A 9-year old girl had clinical manifestations of juvenile rheumatoid arthritis that were further complicated by severe anemia. Subsequent studies showed the anemia to be due to erythroid aplasia. Hematologic remission was induced with prednisone, 60 mg/day.