The May-Hegglin anomaly is a rare autosomal dominant trait characterized by platelet abnormalities in the peripheral blood and large (up to 5mu) pale blue staining inclusions in the cytoplasm of neutrophils, eosinophils, basophils, and monocytes. We experienced a case of May-Hegglin anomaly in a 7 year old male and another case out of his family. His initial complaints at the admission were petechiae and intermittent epistaxis. Diagnosis was confirmed by peripheral blood smear and family study. We report the case with brief review of related literature.
Basophils ; Blood Platelets ; Child ; Cytoplasm ; Diagnosis ; Eosinophils ; Epistaxis ; Humans ; Male ; Monocytes ; Neutrophils ; Purpura

Fluid resuscitation is a basic treatment in hemorrhagic shock. We compared the circulatory responses to fluid resuscitation of 10% pentastarch with those of fresh whole blood and plasmanate in hemorrhagic shock. Eighteen mongrel dogs were bled 24 ml/kg and replaced by equivalent amounts of fresh whole blood(n=6, group B), pentastarch(n=6, group P) and plasmanate(n=6, group PL). Hemodynamic measurements and calculations were performed before and after bleeding and after volume therapy. The decrease of hematocrit and platelet count after volume replacement indicate that hemodilutional effect was maximum 30 min after volume therapy and significantly greater in group P than PL(p<0.05). Central venous pressure(CVP), pulmonary capillary wedge pressure(PCWP) and cardiac index(CI) were increased to 146-189%, 146-172% and 146-175% in group P, respectively during 60 min. These changes were significantly greater than group B and PL(p<0.05). There was delayed recovery of mean arterial pressure in group PL(92% 30 min after volume therapy) compared with group B and P(92% and 93% 5 min). Also group P and PL showed significant prolongation in prothrombin time and partial thromboplastin time during experiment(120 min) and these were significantly more prolonged in group P than PL(P<0.05). And group P showed similar O transport and O extraction ratio to those of group B. The increases in plasma catecholamine were observed after hemorrhage, but no significant changes 5 and 30 min after volume therapy. This suggests that the neurohumoral response to hemodilution was not marked. Mixed venous O2 saturation(SvO2) was directly proportional to CI during experiment(r=0.69, p<0.01), indicating that SvO2 can represent CI during shock and volume therapy. In conclusion, l0% pentastarch is useful as a substitute for fresh whole blood or plasmanate.
Animals ; Arterial Pressure ; Blood Transfusion, Autologous* ; Capillaries ; Dogs* ; Hematocrit ; Hemodilution ; Hemodynamics ; Hemorrhage ; Hydroxyethyl Starch Derivatives ; Partial Thromboplastin Time ; Plasma ; Platelet Count ; Prothrombin Time ; Resuscitation* ; Shock ; Shock, Hemorrhagic*

No abstract available.

No abstract available.
DiGeorge Syndrome*

No abstract available.

It has been suspected that the cannula of the ultrasonic generator became heated during liposuction, and that the heated cannula might possibly damage the soft tissues such as vessels, nerves and muscles. To confirm these suspicions, the actual temperature of the cannula was measured after being switched on, and the influence of the heated cannula on the soft tissues of 30 rabbits was studied macroscopically and microscopically. When the cooling system of ultrasonic generator was not operated, the temperature of a cannula tip increased to 100degrees C in 10 seconds, and the temperature of a cannula shaft did not increase over 40 degrees C. When the cooling system of the ultrasonic generator was operated, the temperature of the cannula tip increased to 70 degrees C in 20 seconds and to 100 degrees C in 1 minute. The stronger ultrasonic power was, the higher the temperature of the cannula tip was. When the heated cannnula tip touched the muscles, vesseles and nerves directly, the arterial and venous walls were perforated in about 20 seconds and 10 seconds, respectively. Gross changes of the muscles, such as color change and depression of the contacted area, were seen in about 30 seconds, and histological changes occurred in about 3 seconds. When adding the Klein solution, an infiltration solution for increasing the destructive effects of the ultrasound, the onset time of tissue damage was significantly shortened in vessels and nerves, but there was no significant difference in muscles. Although there was no finding of damage by the ultrasonic wave itself on the vessels, nerves, and muscles during ultrasonic liposuction, this study confirmed that the heated cannula could damage vessels, nerves, and muscles. Thus we must try to avoid placing the cannula at certain areas for more than 10 seconds douring the ultrasonic liposuction.
Catheters* ; Depression ; Hot Temperature ; Lipectomy* ; Muscles* ; Rabbits ; Ultrasonics* ; Ultrasonography

Muscles from three patients were obtained during continuous ischemic periods and were investigated ultrastructural changes to know the reversible tourniquet time. Tourniquet was applied for periods lasting between 60 minutes and 90 minutes in the human and muscle biopsies were taken from 10 minutes to 90 minutes ischemic period and at the same periods after release of the tourniquet. All specimens were investigated with electron microscopy and the results were as follows: In 10 minutes tourniquet period, loss of cristae in few mitochondria were observed. In 30 minutes tourniquet period, partial swelling, loss of cristae and myelin figure in many mitochondrias and vacuolar dilatation in T-tubule and sarcoplasmic reticulumn were observed. In 60 minutes tourniquet period, above change were observed in nearly all mitochondrias and total number of mitochondrias were decreased. In 90 minutes tourniquet period, above changes were more distinct. 20 minutes after release of 90 minutes tourniquet period, small sized new mitochondria were appeared and abundant electron dense granule in their matrix and sarcoplasmic reticulumn were visible. 60 minutes after release of 90 minutes tourniquet period, the majority of damaged mitochondrias were replaced by small sized new mitochondrias and electron dense granule were abundant in sarcoplasmic reticulumn. In human, the changes on the ultrastructure were reversible for at least a succeeding 90 minutes ischemic periods.
Biopsy ; Dilatation ; Humans ; Microscopy, Electron ; Mitochondria ; Muscle, Skeletal ; Muscles ; Myelin Sheath ; Tourniquets

No abstract available.
Heart* ; Thoracic Surgery* ; Thyroid Gland*

No abstract available.
Heart Neoplasms*

Spontaneous cerebrospinal fluid (CSF) leak is a recognized cause of spontaneous intracranial hypotension (SIH). Subdural hematoma (SDH) is a serious but rare complication of SIH. An autologous epidural blood patch at the CSF-leak site can effectively relieve SIH. We report a case of bilateral SDH with SIH caused by a CSF leak originating at the C1-2 level. A 55-year-old male complained of orthostatic headache without neurological signs. His symptoms did not respond to conservative treatments including bed rest, hydration and analgesics. Magnetic resonance imaging showed a subdural hematoma in the bilateral fronto-parietal region, and computed tomography (CT) myelography showed a CSF leak originating at the C1-2 level. The patient underwent successful treatment with a CT-guided epidural blood patch at the CSF-leak site after trephination for bilateral SDH.
Analgesics ; Bed Rest ; Blood Patch, Epidural* ; Cerebrospinal Fluid ; Headache ; Hematoma, Subdural ; Hematoma, Subdural, Chronic* ; Humans ; Intracranial Hypotension* ; Magnetic Resonance Imaging ; Male ; Middle Aged ; Myelography ; Trephining