Hb Sinai-Baltimore or alpha 2 beta (2)18(A15)Val->Gly, a silent, mildly unstable beta chain variant detected by isoelectrofocusing and high performance liquid chromatography

Isoelectrofocusing and high performance liquid chromatographic methods were used to study an abnormal hemoglobin present in a Black male infant and his mother. The variant, named Hb Sinai-Baltimore, focused slightly behind Hb A and separated incompletely from Hb A by cation exchange high performance liquid chromatography, while the separation of the beta A and beta X chains by reversed phase high performance liquid chromatography was complete. The variant was identified through an analysis of peptides in a tryptic digest of the isolated beta X chain and by sequencing of amplified DNA which included the beta-globin gene. The Val->Gly replacement at position beta 18 (codon 18; GTG->GGG) or at the last position of the A helix decreases the stability of the variant without affecting the hematological parameters of its carrier. The propositus was a compound heterozygote for Hb Sinai-Baltimore and Hb S; the relative quantities of the two variant chains were somewhat different from those of the beta X and beta A chains in the mother with the simple Hb Sinai-Baltimore heterozygosity. An uncertainty about the alpha-globin gene status in the child prevented a further evaluation of these differences.     

Boriding with a thermally unstable gas (diborane)

Thermally unstable diborane can be used to boride the surface of iron or steel if the proper conditions are maintained. This paper explores the conditions which lead to efficient boriding and shows examples. The use of ion bombardment is suggested as a method to further improve the technique.     

Anesthetic management of a patient with the allergic granulomatous angitis (Churg-Strauss syndrome)

We gave anesthesia 4 times to a patient (19-year-old female) with allergic granulomatous angitis (AGA). She had asthma, myopathy and detrimental side effect in her eyes of steroid therapy for AGA. Two of the 4 operations were emergency laparotomy for peritonitis due to colon perforation, and the other 2 operations were elective eye surgeries. General anesthesia was induced with thiamylal (1st operation) or midazolam (2nd-4th operation). Intraoperative anesthesia was maintained with N2O-O2-isoflurane combined with thoracic epidural anesthesia (1st operation) or N2O-O2-sevoflurane (2nd-4th operation). Asthmatic attack, which AGA accompanies frequently, did not occur during these anesthetic managements. The steroid therapy, which is a fundamental means to control the allergic syndrome, might suppress asthma. However, asthmatic attack and systemic vasculitis should be kept in mind in anesthetic management of AGA.     

Sheep Hb I variant, or beta13(A10)Gly-->Ser, in breeds from Corsica: detection by gene sequencing

Nuclear magnetic resonance (NMR) offers the possibility to determine moisture profiles in porous building materials. Moreover, the relaxation of the nuclear magnetic resonance signal can provide additional information on the water distribution in the microstructure. For mortar, it is shown that the transverse relaxation yields information on the distribution of water in the gel pores and capillary pores. Moisture profiles and relaxation were measured during water absorption. The effect of the drying treatment on the microstructure and the water absorption was investigated.     

A computerized tomography (CT) scan appliance for optimal presurgical and preprosthetic planning of the implant patient

This article describes the technique for using the CT (computerized axial tomography) scans, with specially designed software and an adjunct appliance, for accurate planning of dental implants and implant-supported restorations. The available bone can be evaluated, surgical problems anticipated, and errors in placement avoided. The learning objective of this article is an enhanced knowledge of the extended application of the CT scans and the use of the orientation and immobilization appliance.     

Hb Helsinki: a variant with a high oxygen affinity and a substitution at a 2,3-DPG binding site (beta82[EF6] Lys replaced by Met)

A new haemoglobin, Hb Helsinki, in which beta 82-Lys (EF6) is replaced by Met, was found in a Finnish family. It was associated with familial erythrocytosis, and the oxygen affinity of the blood was higher than normal. The oxygen equilibrium curves of purified Hb Helsinki and HbA from the same haemolysate have been determined under vaious conditions. "Stripped' Hb Helsinki was found to show normal cooperativity, slightly low oxygen affinity and a reduced Bohr effect at physiological pH. However, the organic phosphates, 2,3-diphosphoglycerate (2,3-DPG) and inositol hexaphosphate (IHP) had a very small effect on Hb Helsinki, and the 2,3-DPG binding constant of deoxygenated Hb Helsinki is close to that of oxyhaemoglobin A. Thus, the replacement of Lys by Met at position 82 dramatically changes the nature of the central cavity of the tetramer and the effect of 2,3-DPG on the respiratory function of the molecule.     

The holistic management of the patient with pre-eclampsia

Physiological adaptations of pregnancy are summarised. Maternal consequences of pre-eclampsia, eclampsia and HELLP syndrome are described. Goals of ITU management are outlined. Directions for future management/therapies are included. The need to integrate technological expertise with sensitive nursing care is emphasised.     

Anesthetic management of a patient with Bernard-Soulier syndrome

Bernard-Soulier syndrome (BSS) is a hereditary hemorrhagic disease characterized by prolonged bleeding time due to abnormal platelet aggregation and giant platelets. Transfusion of platelet-rich plasma is the only treatment available for the hemorrhagic episodes in patients with this disease. A 20-year-old female with BBS was scheduled for sagittal osteotomy of the mandibular rami under general anesthesia. Anesthesia was induced with fentanyl and diazepam, and was maintained with nitrous oxide, fentanyl, and 0.5% enflurane. No exacerbation of the bleeding tendency was observed during or after the surgery. We consider that the use of halothane should be avoided in patients with BBS because it may inhibit the aggregation of platelets and prolong the bleeding time.     

Peripartum management of a patient with Isaacs' syndrome

PURPOSE: To describe the peripartum management of a patient with Isaacs' syndrome with specific reference to the anaesthetic implications of the disease process. Associated medical problems included obesity, pregnancy induced hypertension and a difficult airway. CLINICAL FEATURES: This 30-yr-old gravida V para 0 woman presented to the anaesthesia consultation clinic at 37-wk gestation to discuss pain relief options for labour and delivery. She had a history of Isaacs' syndrome (a peripheral motor neuron disorder), congenital heart disease (ASD and VSD), treated Hashimotos thyroiditis, obesity and a family history of haemachromatosis. On the day of consultation, she was hypertensive and peripheral oedema was noted. Her urine showed trace protein. Four days later, she presented to the labour suite and her cervix was 9 cm dilated. An epidural anaesthetic was given without difficulty and she had an uneventful labour and delivery course. There were no subsequent neurological complications. CONCLUSION: Isaacs' syndrome is an extremely rare peripheral motor neuron disorder. This patient was successfully managed with epidural analgesia for labour and delivered a healthy child with no congenital anomalies.     

Anesthetic management of a patient with Cowden syndrome

Cowden syndrome is a rare syndrome of chromosome abnormalities presenting with polyposis of digestive tracts, characteristic skin eruption and neuromuscular disorders. A 56-year-old male patient with Cowden syndrome underwent upper abdominal surgery under general anesthesia followed by post-operative epidural analgesia with buprenorphine. Proposed total gastrectomy was not performed because of massive invasion of carcinoma in the abdominal cavity and gastrojejunostomy was done instead. The anesthesia was satisfactory with inhalation of nitrous oxide and enflurane with intravenous vecuronium. Neuromuscular monitoring with electric twitch-responses of the hand showed normal patterns throughout the anesthesia. The recovery from anesthesia and neuromuscular blockade was prompt. Intermittent epidural buprenorphine, twice a day (0.2 mg of buprenorphine in 9 ml of normal saline for one time) was started just after the recovery of anesthesia and continued for four days. Delirium occurred two days after beginning epidural buprenorphine and disappeared three days after its discontinuation. The patient died 52 days after the operation from obstructive jaundice and sepsis. The delirium, therefore, seems to have been caused by buprenorphine possibly due to its impaired metabolism by the liver. Although we did not experience any abnormal neuromuscular reactions to vecuronium or anesthetic agents, it is important to perform preoperative neuromuscular examinations and peri-operative monitoring in the anesthetic management of a patient with this syndrome.     

Woody hands in a patient with pancreatic carcinoma: a variant of cancer-associated fasciitis--panniculitis syndrome

We report an elderly woman with rapidly progressive painless, woody induration of the hands. Mild diabetes mellitus was demonstrated. Skin biopsy features included broad fibrous bands extending deeply into subcutaneous fat, a mild mononuclear cell infiltrate, and post-thrombotic recanalization of a deep vessel in one specimen. The patient developed uncontrolled haematemesis and was demonstrated at laparotomy to have disseminated pancreatic carcinoma. The unusual clinical features and temporal relationship between the skin changes and the tumour suggest a paraneoplastic eruption. Which appears best classified as an example of cancer-associated fasciitis-panniculitis syndrome.     

Anesthetic management of a patient with hypertrophic cardiomyopathy using propofol, fentanyl and ketamine

A 59-year-old male with hypertrophic cardiomyopathy was scheduled for resection of a maxillary cyst. Metoprolol was discontinued the day before surgery. Thirty min before anesthesia, meperidine 35 mg was administered intramuscularly. After intravenous administration of midazolam 3 mg, a pulmonary catheter was inserted for monitoring hemodynamic parameters. Anesthesia was induced with propofol 75 mg, fentanyl 0.15 mg and ketamine 75 mg. Anesthesia was maintained with continuous infusion of propofol 5 mg.kg-1.h-1 and ketamine 1 mg.kg-1.h-1. Moreover, fentanyl was added as necessary during surgery. Blood pressure (BP), pulmonary arterial pressure (PA), systemic vascular resistance index (SVRI) and pulmonary vascular resistance index (PVRI) were measured using a pulmonary catheter during anesthesia. Since BP decreased after intubation, dopamine 3 micrograms.kg-1.min-1 was administered for 20 min. The hemodynamic state was stable during surgery. However, BP, PA, SVRI and PVRI increased temporally at extubation. His postoperative course was uneventful. In conclusion, total intravenous anesthesia with propofol, fentanyl and ketamine may be useful for anesthetic management of a patient with hypertrophic cardiomyopathy.     

Chiropractic management of a patient with subluxations, low back pain and epileptic seizures

OBJECTIVE: To describe the chiropractic management of a patient presenting with complaints of low back pain and epileptic seizures. The discussion also addresses epilepsy and the current concepts of this disorder; possible mechanisms for the neurological effects of the chiropractic adjustment at sites of subluxation and its therapeutic implications are proposed. CLINICAL FEATURES: A 21-year-old woman with low back pain reported that she had fainted during the night and hit her head. She had been diagnosed since childhood with grand mal (tonicclonic) seizures as well as petit mal seizures. She had a seizure approximately every 3 hr, with a duration between 10 sec and 30 min for each episode. Examination indicated signs of subluxation/dysfunction at the L5-S1, C6-C7 and C3-C4 spinal levels. There was no evidence of cranial nerve involvement or any upper motor neuron lesion. Radiographic analysis revealed retrolisthesis of L5, hypolordosis of the cervical spine and hyperextension of the C6-C7 motion segment. INTERVENTION AND OUTCOME: Chiropractic adjustments using a specific-contact, short-lever arm, high-velocity, low-amplitude maneuver (i.e., Gonstead) were applied to the subluxations at the cervical, thoracic and lumbopelvic region. The patient's reported low back pain and neck complaints improved and her seizure frequency decreased. At 1.5-yr follow-up, the patient reported her low back complaints had resolved and her seizures had decreased (period between seizures as great as 2 months). CONCLUSION: Results encourage further investigation of possible neurological sequalae, such as epileptic seizures, from spinal dysfunction identified as vertebral subluxation complexes by chiropractors and treated by specific spinal adjustments.