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Biomedical subjects

H M Koenig

Publications and source records attributed to H M Koenig.

53 records · Page 3Linked to original sources
PubMed1977

The molecular defects of alpha-thalassemia in the Filipino.

Molecular hybridization with synthetic radioactive DNA (cDNA) complementry to alpha globin mRNA sequences shows that, as in most other Southeast Asian populations, the alpha globin structural genes are deleted in Filipinos affected by the alpha-thalassemia syndromes. Thus, all 4 alpha-globin structural genes are deleted in homozygous alpha-thalassemia with hydrops fetalis, 3 and 2 structural genes are deleted in hemoglobin H disease and alpha-thalassemia-1 respectively.

DNA↗
PubMed1977

Free erythrocyte porphyrin: hemoglobin ratios, serum ferritin, and transferrin saturation levels during treatment of infants with iron-deficiency anemia.

Free erythrocyte porphyrin:hemoglobin (FEP:Hb) ratios were determined on 20 infants with iron-deficiency anemia. FEB:Hb ratios were compared with simultaneously drawn serum ferritin and serum transferrin saturation levels. FEP:Hb ratios dropped steadily during treatment of the anemic infants, but remained elevated compared to age-matched nonanemic infants, until the anemia was corrected. FEP:Hb ratios detected iron deficiency when acute inflammatory disease was present. Serum ferritin levels and transferrin saturation failed to detect iron deficiency after iron therapy was started or when acute inflammatory disease was present. Measurement of FEP:Hb ratios is a reliable and practical way to make a prompt diagnosis of iron-deficiency anemia in infants.

Anemia, Hypochromic↗
PubMed1976

Overdosage with vincristine.

Five patients received overdoses of vincristine ranging from 3.5 to 32 mg. Neurotoxicity accounted for most of the complications observed. Peripheral neuropathies, cranial nerve palsies, paralytic ileus, atony of the bladder, hypertension, hypotension, seizures, inappropriate ADH secretion, and severe bone marrow depression were all encountered. Two patients died within 72 hours of the overdose. Another patient died of sepsis 22 days after the overdose. Two patients recovered and were discharged. The three patients who survived longer than a few days showed improvement in the vincristine-induced neuropathy, and the two long-term survivors had essentially complete recovery. It appears that if a patient can be supported through the critical period following an overdose, he can be expected to recover normal neurologic function.

Adolescent↗
PubMed1976

Classification of microcytic anemia by fluorometric analysis of free erythrocyte porphyrins (FEP).

Microcytic red blood cells (RBC) occur in iron-deficiency anemia, lead poisoning, and the thalassemia syndromes. Micromeasurement of FEP by acid extraction from RBC was performed on RBC of 64 subjects with RBC mean corpuscular volume less than 78 fl as determined on a Coulter S. FEP was also determined on RBC from 25 nonanemic, normocytic subjects for comparison. The 25 nonanemic subjects, 29 subjects with alpha-thalassemia trait and 16 subjects with beta-thalassemia trait had FEP less than 107 mugm/100 ml RBC. Nineteen microcytic subjects with iron-deficiency anemia had FEP of 185--752 mugm/100 ml RBC. Hemolysates from 8 lead intoxication individuals had FEP values similar to those of iron-deficient patients. The fluorescence emission spectra of lysates with high FEP, which were not extracted, were similar in iron deficiency and lead poisoning. The porphyrin that accumulates in these two conditions appears to be zinc protoporphyrin. Micromeasurement of FEP can be used to initially classify microcytic anemias into either a disturbance of globin synthesis or a disturbance in heme synthesis. Iron-deficiency anemia and lead poisoning cause accumulation of identical prophyrin and cannot be distinguished by fluorometric analysis.

Anemia, Hypochromic↗
PubMed1976

In vitro production of platelet-binding IgG in childhood idiopathic thrombocytopenic purpura.

We studied two boys, 9 and 10 years old, respectively, who underwent splenectomy eight months and six weeks after initially presenting as "typical" cases of childhood ITP. Splenic leukocytes from each child were cultured and net synthesis rates of IgG determined. Splenic synthesis rates of IgG were five-and sevenfold greater, respectively, than in spleens of control subjects. A significant portion of the splenic culture-produced IgG from each patient demonstrated specificity for homologous and autologous platelets. Synthesis rates of IgG were determined on serial cultures of bone marrow cells from the 10-year-old boy who failed to respond to splenectomy. His initial marrow synthesis rate of IgG was five times greater than mean control values. His synthesis rate of IgG in marrow decreased to the control range in association with immunosuppressive therapy and intesive platelet transfusions. These data suggest that (1) the spleen was a production site of platelet-binding IgG in the two children, and (2) the bone marrow may have been an additional site of production in our second patient.

Antibodies↗
PubMed1975

Childhood idiopathic thrombocytopenic purpura. Aggressive management of life-threatening complications.

Idiopathic thrombocytopenic purpura (ITP) in childhood is usually a benign, self-limited illness. Life-threatening complications, such as central nervous system (CNS) hemorrhage, occur in less than 1% of cases. We report a case in which the patient failed to respond to splenectomy and high-dose corticosteroid therapy. Immunosuppressive therapy with cyclophosphamide, vincristine sulfate, and corticosteroids in conjunction with frequent transfusions of platelets was associated with a complete remission that persisted after therapy was discontinued.

Age Factors↗
PubMed1975

The micromeasurement of free erythrocyte protoporphyrin as a means of differentiating alpha thalassemia trait from iron deficiency anemia.

Free erythrocyte protoporphyrin levels were measured in 29 individuals with alpha thalassemia trait, 19 with proved iron deficiency anemia, and 25 normal control subjects. Individuals with alpha thalassemia trait and normal iron studies had FEP levels in the normal range despite marked degrees of microcytosis. In contrast, individuals with iron deficiency anemia had marked elevation of FEP levels with similar degrees of microcytosis. Measurement of FEP levels appears to be a useful tool in differentiating alpha thalassemia trait from iron deficiency anemia.

Anemia, Hypochromic↗
PubMedDate not supplied

Use of recombinant human erythropoietin in a Jehovah's Witness.

We report the case of a Jehovah's Witness who bled massively, refused blood transfusion, and survived profound anemia (hematocrit = 5.6%) intact. The patient was treated with recombinant human erythropoietin, parenteral iron, and oxygen. The pharmacology and hematopoietic response to erythropoietin are discussed. We suggest considering this therapy for acutely anemic patients who refuse transfusion to decrease the duration of the most severe anemia.

Adult↗
PubMedDate not supplied

Bispectral Index System (BIS) monitoring reduces time to discharge in children requiring intramuscular sedation and general anesthesia for outpatient dental rehabilitation.

PURPOSE: Pediatric patients who receive both intramuscular (i.m.) sedation and general anesthesia (GA) for oral rehabilitation occasionally experience prolonged sedation and delayed discharge. The Bispectral Index System (BIS) is an EEG monitor that measures the level of sedation. The authors compared discharge times of patients who had BIS monitoring to those who did not to determine if the use of BIS speeded discharge. METHODS: After IRB approval, 20 children were enrolled. BIS was monitored continuously from admission until discharge. Each child received ketamine, midazolam, and glycopyrrolate i.m. Once sedated, the patient was transferred to the operating room, monitored, and i.v. access was established. GA proceeded with sevoflurane, rocuronium, and fentanyl. Randomly, in half the patients, the anesthesiologist knew and maintained the BIS at GA level of sedation by adjusting sevoflurane. In the rest, the anesthesiologist did not know BIS. Time from turning of sevoflurane to discharge was noted and compared. RESULTS: Patients where the BIS was known and used were discharged 60+/-13 minutes after the end of GA. Patients where BIS was unknown were discharged 90+/-11 minutes after the end of GA (P<.001). CONCLUSIONS: Based on the data, the authors recommend the use of BIS to facilitate faster discharge of pediatric patients who require i.m. sedation and GA for oral rehabilitation.

Adjuvants, Anesthesia↗
PubMedDate not supplied

Bispectral index system (BIS) monitoring reduces time to extubation and discharge in children requiring oral presedation and general anesthesia for outpatient dental rehabilitation.

PURPOSE: Pediatric oral rehabilitation patients who receive presedation with oral Versed and general anesthesia (GA) occasionally experience prolonged sedation and delayed discharge. The Bispectral Index System (BIS) is an EEG monitor that measures the anesthesia level. The purpose of this study was to compare the effects of monitoring the BIS to not monitoring the BIS on time from discontinuation of GA to extubation and to discharge. METHODS: Twenty-nine children were enrolled. BIS was monitored from admission until discharge. Each child received 0.7 mg/kg of oral Versed. In the operating room, GA with sevoflurane (IH), rocuronium 1 mg/kg (IV), fentanyl 1 microg/kg (IV), and ondansetron 0.15 mg/kg (IV) was administered. Randomly, in half the patients, the anesthesiologist maintained the level of anesthesia and BIS by adjusting sevoflurane. In the rest, the anesthesiologist did not know BIS. The time from turning off sevoflurane to discharge was compared. RESULTS: Group 1 patients were extubated 5+/-2 minutes sooner than group 2 patients (P=.04). The post-anesthesia care unit stay for group 1 patients was 47+/-17 minutes compared to 63+/-17 minutes in group 2. (p=0.02). CONCLUSIONS: Monitoring anesthesia with BIS promotes earlier extubation and discharge for pediatric dental patients who receive oral Versed and sevoflurane GA.

Administration, Oral↗