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PubMedDate not supplied

Subcutaneous fat necrosis of the newborn associated with anemia.

Subcutaneous fat necrosis (SFN) of the newborn characteristically affects full-term infants who have experienced perinatal distress, such as hypothermia, obstetric trauma, or asphyxia. We report a newborn who had pallor, deep breathing, and severe anemia immediately after birth. She developed SFN on the fourth postnatal day. Her condition improved after blood transfusions and the skin lesions resolved in 6 weeks. This appears to be the first report of SFN associated with anemia.

Anemia, Neonatal↗
PubMedDate not supplied

Ultrastructural alterations and phagocytic function of cryopreserved platelets.

Fresh human platelets and platelets cryopreserved in 4% dimethylsulfoxide were examined ultrastructurally before and after incubation in a suspension of latex particles. Cryopreserved platelets had fewer discoid forms than fresh platelets. The cryopreserved platelets had many sphered platelets containing an increased number of vacuoles; the sphered platelets were more electron-lucent margination and pallor of organelles. Phagocytosis of latex into vacuoles was markedly impaired in the cryopreserved platelets. The morphologic alterations suggested that the freezing, thawing, and washing procedures reduced the functional activity of platelets.

Blood Platelets↗
PubMed1998

Frequency of immediate adverse effects associated with apheresis donation.

BACKGROUND: Apheresis donation is considered safe, but the incidence of adverse effects has not been determined in a large multicenter series of donations with modern instruments. STUDY DESIGN AND METHODS: The Hemapheresis Committee of the American Association of Blood Banks devised a uniform questionnaire that asked about 32 specific adverse effects. Transient paresthesia and mild vasovagal events were excluded. A survey was conducted in 1995; 17 centers returned 19,611 responses concerning 250 to 2,000 consecutive apheresis donations per center. RESULTS: Six hundred adverse effects were reported in 428 donations (2.18% of donations). Pain or hematoma at a venipuncture site was the most common response (1.15% of donations); only 203 donations had other (nonvenipuncture) adverse effects (1.04%). Total and nonvenipuncture rates were, respectively, 4.84 and 2.92 percent for 2,295 first donations and 1.78 and 0.77 percent for 17,303 repeat donations (p < 0.001). Rates of nonvenipuncture symptoms in first and repeat donations were, respectively, citrate-induced nausea and/or vomiting, 0.87 and 0.27 percent; tetany, 0.09 and 0.04 percent; pallor and/or diaphoresis, 1.87 and 0.32 percent; vasovagal nausea and/or vomiting, 0.87 and 0.13 percent; syncope and/or seizure, 0.39 and 0.04 percent; and chills and/or rigors, 0.31 and 0.01 percent. The overall rate of donor unconsciousness was 0.08 percent. Hemolysis was reported twice. Clotting or leakage occurred in 0.08 percent of donations, and inability to return blood occurred in 0.16 percent. No life-threatening adverse effects were reported. Procedure-specific nonvenipuncture rates were 1.05 percent of 17,584 platelet donations, 0.67 percent of 594 white cell donations, and 0.37 percent of 1,354 plasma donations. Center-specific rates varied from 0.32 to 6.81 percent of donations for total adverse effects and from 0.11 to 2.92 percent of donations for nonvenipuncture events. CONCLUSION: Apheresis donation is a safe undertaking, suitable for voluntary blood donors, with a very low risk of serious adverse effects. The risk of unconsciousness is lower than that found in many studies of whole-blood donation.

Blood Component Removal↗
PubMed1999

Frequency of immediate adverse effects associated with therapeutic apheresis.

BACKGROUND: Therapeutic apheresis was found to be reasonably safe in prior studies using instruments that are now largely obsolete. The incidence of adverse effects with current instruments and techniques has not been assessed in a large multicenter study. STUDY DESIGN AND METHODS: A survey was conducted in 1995 using a uniform questionnaire that asked about 32 specific events but excluded transient paresthesia and mild vasovagal events. Eighteen centers returned 3429 responses concerning 125 to 500 therapeutic apheresis procedures per center. RESULTS: Two hundred forty-two adverse events were reported in 163 procedures (4.75% of all procedures; 6.87% of first-time procedures and 4.28% of repeat procedures). The numbers (incidence) of selected specific events were transfusion reaction, 56 (51 in plasma exchange [PE] with plasma replacement) (1.6%); citrate-related nausea and/or vomiting, 41 (1.2%); systolic blood pressure <80 mmHg, 34 (1.0%); vasovagal nausea and/or vomiting, 17 (0.5%); pallor and/or diaphoresis, 16 (0.5%); pulse >120, 14 (0.4%); respiratory distress, 9 (0.3%); tetany or seizure, 9 (0.2%); and chills or rigors, 6 (0.2%). Rates for other specific events were < or =0.1 percent. Vasovagal phenomena were more frequent in procedures done in neurologic patients than in those done in hematology or oncology patients (p = 0.011) or renal or rheumatic patients (p = 0.038). Procedure-specific rates were red cell exchange, 8 (10.26%) of 78; PE (plasma), 89 (7.81 %) of 1140; PE (no plasma), 42 (3.35%) of 1255; leukapheresis, 4 (5.71%) of 70; plateletpheresis, 0 of 18; and autologous peripheral blood progenitor cell collection, 11 (1.66%) of 664. Three deaths were reported; all were attributed to primary disease. CONCLUSION: Therapeutic apheresis procedures are relatively safe, with a 4.75-percent overall incidence of mostly reversible adverse effects. Among the most commonly performed procedures, the risk is higher for blood component exchanges, especially if allogeneic red cell or plasma transfusion occurs, and lower for peripheral blood progenitor cell collection.

Anticoagulants↗
PubMed2001

Clinical characteristics of patients with vasovagal reactions presenting as unexplained syncope.

OBJECTIVE: To describe the clinical characteristics of vasovagal syncope (VVS) in patients presenting to a tertiary referral centre with unexplained syncope, in whom the diagnosis of VVS was confirmed by tilt table testing (HUT) and in whom other causes of syncope excluded. DESIGN: Prospective study of 62 consecutive patients with more than two episodes of syncope in the past year. SETTING: A regional tertiary referral centre for patients with unexplained syncope. PATIENTS: Sixty-two patients, mean age 50 +/- 21 years, 39 female, were studied. Mean duration of symptoms was 5 years. Average frequency of attacks was one episode per week. INTERVENTIONS: Detailed semi-structured questionnaires were completed regarding presenting symptoms. RESULTS: In over one-third of patients, episodes occurred suddenly, with no prodromal features. In those with prodrome, 71% had autonomic symptoms, but 27% had palpitations or dyspnoea and 21% had chest pain. Eleven percent of patients denied known provocative features. In the remainder, the most common were prolonged standing (37%), hot weather (27%) and lack of food (23%). One-fifth had symptoms sitting and 5% whilst driving. Seventy-five percent of patients suffered after effects, the most common being severe fatigue. Over half sustained an injury during syncope, and 13% sustained a fracture. Unwitnessed episodes occurred in 25%. Pallor was reported in half the cases, sweating in 13% and myoclonus in 5%. CONCLUSIONS: Atypical presentations of VVS occur in many patients referred to a tertiary referral centre. Knowledge of the clinical characteristics of unexplained syncope for which VVS is the attributable diagnosis should assist in appropriate management of such patients.

Adult↗
PubMed2001

Retching and vomiting in neurologically impaired children after fundoplication: predictive preoperative factors.

BACKGROUND/PURPOSE: In neurologically impaired children, retching and recurrent vomiting are common after Nissen fundoplication. The aim of this study was to identify whether there are preoperative factors that predict their occurrence. METHODS: Twenty neurologically impaired children (8 boys, 12 girls; age range, 3 months to 8 years) were studied prospectively by taking a detailed history of behaviors and symptoms associated with feeding before and after Nissen fundoplication for gastroesophageal reflux. RESULTS: Preoperatively, children could be classified into 2 groups. Children in group A had symptoms suggestive of only gastroesophageal reflux (effortless "vomiting" or regurgitation), whereas children in group B exhibited one or more features associated with activation of the emetic reflex (pallor, sweating, retching, forceful vomiting). Postoperatively 0 of 8 in group A retched compared with 8 of 12 in group B (P <.005, Fishers Exact test). CONCLUSIONS: Children at high risk of retching, and ultimately vomiting, after antireflux surgery may be identified clinically preoperatively. They have symptoms that are specifically caused by activation of the emetic reflex rather than to gastroesophageal reflux. In these cases, antireflux surgery could be considered inappropriate and hence be avoided.

Central Nervous System Diseases↗
PubMed2001

Evaluation of patients with decreased vision or blindness.

A good ocular examination and careful evaluation of the pupillary light reflexes will localize most lesion(s) producing a complaint of blindness. Lesions of the clear media severe enough to produce blindness will prohibit fundus examination. Retinal, optic nerve, and complete chiasmal lesions will produce dilated, slowly reactive pupils. Retinal blindness is usually associated with dramatic ophthalmoscopic lesions of diffuse altered reflectivity and vascular attenuation, the major exceptions being the sudden acquired retinal degeneration syndrome and some patients with glaucoma. Optic nerve lesions may involve the optic disc where they may be recognized by either pallor or hyperemia and cupping or elevation. Confirmation of optic nerve blindness in practice usually is by ruling out retinal blindness, but a more definitive methodology is to have a normal electroretinogram, slow or absent direct pupillary light reflexes (PLR), and abnormal visual evoked potentials. Central blindness is diagnosed by normal eyes, normal PLR, and often central nervous system (CNS) signs indicative of a diffuse or multifocal CNS disease.

Animals↗
PubMed2002

Digital artery damage associated with closed crush injuries.

This retrospective study describes closed finger crush injuries in seven patients (eight fingers) in which each finger sustained a loss of blood supply. Clinical findings included numbness, decreased two-point and sharp/dull sensation, cyanosis or pallor, and decreased capillary filling. Fractures, especially transverse fractures near the proximal interphalangeal joint or distal interphalangeal joint, were usually present and often showed longitudinal crush fracture lines. Exploration and revascularization were carried out in seven fingers, all of which survived. The only finger not explored progressed to necrosis and amputation. Crush injuries to the fingers, especially those associated with displaced fractures, should be carefully evaluated for symptoms and signs of ischaemia.

Adolescent↗
PubMed2001

[Intrapulmonary tumor cell embolism from cancer of the bladder as the cause of a subacute cor pulmonale].

HISTORY AND CLINICAL FINDINGS: A 61-year-old man had over 3 weeks experienced increasing dyspnoea on effort and loss of appetite. He reported previous chronic pain with a "stiffened hip" treated long-term with diclofenac, and three transurethral surgical interventions. It was only at the latest admission that bladder cancer (first diagnosed 2 years previously) became known as the reason for the interventions. On admission pallor, sinus tachycardia, fever of 38 degrees C and dyspnoea on light exertion were noted, but blood pressure was normal. INVESTIGATIONS: Chest X-ray was normal, but the patient had marked respiratory insufficiency (pO2 30.8 mmHg, pCO2 31.6 mmHg) on room air. Echocardiography showed a dilated right ventricle with paradoxical septal movement. There was no evidence of thrombosis in the leg veins on ultrasound. D-dimers were normal. DIAGNOSIS, TREATMENT AND COURSE: Pulmonary artery embolism of unknown origin was suspected. Despite anticoagulation with low-molecular heparin the patient's condition deteriorated. Spiral computed tomography of the thorax did not show thrombi in the pulmonary arterial vessels. Perfusion scintigraphy demonstrated definite perfusion deficits bilaterally in the upper lobes of the lung, consistent with pulmonary embolism. As his condition gradually worsened rt-PA was started to achieve fibrinolysis, but failed to produce any change in haemodynamic and respiratory functions. He died a few hours later. Microscopic examination at autopsy revealed multiple tumour emboli with intimal fibrosis in the peripheral arteries. A poorly differentiated urothelial carcinoma of the bladder with extensive infiltration of blood vessels was found (carcinomatous haemangiosis). CONCLUSION: A malignant tumour with micro-embolization of tumour cells should be considered as a possible diagnosis when the clinical picture indicates pulmonary embolism of unknown genesis.

Carcinoma, Transitional Cell↗
PubMed2001

Reduction of carotid artery blood flow in pediatric patients with syncope: evaluation with head-up tilt test.

We investigated the effect of postural changes on cerebral circulation by measuring carotid artery blood flow (CABF) in the supine position and during head-up tilt (HUT) test using a Doppler flow meter. Subjects included 10 patients with neurally mediated syncope, 10 patients with orthostatic intolerance, 8 with epilepsy, aged between 8 to 24 years (mean +/- SD, 13.9 +/- 4.1 years). The test caused pallor and dizziness in 16 patients (symptomatic), while no symptoms were recognized in the other 12 patients (asymptomatic). Significant reductions in the mean CABF (Fm), maximum CABF (Fs) and minimum CABF (Fd), components of the CABF waveform, were noticed during HUT compared to before HUT (supine), and these reductions in symptomatic patients were more severe than those in asymptomatic patients (Fm: - 2.8 +/- 2.0 SD vs - 0.7 +/- 1.4 SD; Fs: - 2.7 +/- 1.5 SD vs - 0.9 +/- 1.3 SD; Fd: - 4.5 +/- 2.8 SD vs - 2.2 +/- 2.1 SD). Reductions in Fm, Fs and Fd in the symptomatic patients during HUT lasted longer than those of the asymptomatic patients, being statistically significant for Fm and Fs (Fm: p < 0.01; Fs: p < 0.05). The criteria during HUT for distinguishing neurally mediated syncope (NMS) from others was Fs: < - 4 SD and Fd: < - 5 SD. Our data suggest that reduction of CABF should be carefully evaluated for the diagnosis of neurally mediated syncope in pediatric patients. Such a reduction might be an essential mechanism of syncopal attacks.

Adolescent↗
PubMedDate not supplied

[Multilocular erythema migrans in borreliosis].

BACKGROUND: Borreliosis is the most common vector transmitted disease in childhood. Although the disease manifests with an erythema migrans in 80 % of the patients, multilocular skin manifestations are only observed in 2-18 % of these. Differential diagnoses of erythema migrans include erysipelas, persistent insect bite reaction, and fixed drug eruption, in particular when the clinical history does not reveal a tick bite. PATIENT: We report on a 5-year-old boy showing nine erythemas with central pallor on his face, trunk, arms and legs. He recalled a tick bite 3 weeks before. RESULTS: Serological studies revealed an acute infection with Borrelia burgdorferi. After antibiotic treatment with orally administered amoxicillin skin manifestations resolved within three days. During a follow-up period of six months the patient revealed no signs of persistent borreliosis. CONCLUSION: Multilocular erythema migrans is a possible manifestation of borreliosis and is classified as disseminated early infection which is frequently associated with systemic reactions, including malaise, arthritis, carditis, headache and even meningeal signs. Treatment is based on antibiotics, which should preferably be given intravenously in case of systemic signs.

Animals↗
PubMed2006

[Leber's hereditary optic neuropathy in malnutrition: a case report].

BACKGROUND: We present a case of Leber's hereditary optic neuropathy (LHON) manifested by malnutrition, tobacco and alcohol abuse. HISTORY AND SIGNS: We report on a 36-year-old patient with alcohol and tobacco abuse for years. 5 months before manifestation of LHON, the alcohol abuse was stopped. Because of congenital cataract the reduction in visual acuity, which occurred after alcohol consumption was stopped, was first misinterpreted. Findings at first exam: visual acuity right eye 0.2, left eye 0.05, temporal optic disc pallor left > right, vitamin B12 and folic acid at the lower level. Molecular genetic analysis: LHON mtDNA 11 778 mutation. Therefore, a pure tobacco-alcohol optic neuropathy could be excluded. THERAPY AND OUTCOME: Under high-dose vitamin-B complex substitution the visual acuity did not improve in the next 6 months. CONCLUSIONS: The cause of the manifestation of the LHON is unknown and multifactorial. In this case the manifestation was caused by malnutrition and tobacco abuse.

Adult↗
PubMed1998

Early VEP and ERG evidence of visual dysfunction in autosomal recessive osteopetrosis.

Autosomal recessive (AR) osteopetrosis has a rapid course and manifests in the first months of life. Visual loss occurs because of optic nerve compromise, and more rarely retinal dysfunction (which may be a part of a primary neurodegeneration). The only curative treatment currently available is bone marrow transplantation (BMT). It has been suggested that BMT is contraindicated if AR osteopetrosis is associated with a primary neurodegeneration. Visual impairment tends to be irreversible after BMT. The young age of the patients makes reliable, objective tests of visual function especially important. We have reviewed the flash electroretinograms (ERGs) and flash and pattern visual evoked potentials (VEPs) recorded without sedation from 15 patients with AR osteopetrosis, 11 of whom were recorded longitudinally. The most frequent, early indication of visual dysfunction was a delay in the pattern or flash VEP latency. This first affects the pattern reversal VEP to small checks. Importantly this often preceded fundal changes of optic disc pallor, and evidence of optic nerve compression on neuroimaging. Only two patients had ERG evidence of retinal dysfunction affecting both rods and cones. One of these patients had a distinctive fundal appearance, but did not have evidence of associated neuronal degenerative disease. The other patient was lost to follow-up. In the patients reviewed in this study successful BMT and optic nerve decompression did not result in VEP improvement. Fundoscopy, VEP and ERG testing are indicated when the diagnosis of AR osteopetrosis is suspected and provide a useful means of monitoring visual involvement.

Bone Marrow Transplantation↗
PubMed1997

Massive fetomaternal hemorrhage.

This report describes a full-term newborn with massive fetomaternal hemorrhage. Fetal movements were decreased 48 hr prior to delivery. On the day of delivery, they were absent. The nonstress test was abnormal with low biophysical profile and decreased beat-to-beat variability. The infant presented with extreme pallor, hypotonia, hepatosplenomegaly, and ascites. The initial hemoglobin was 2.2 g/dL, the Kleihauer-Betke stain was 27.6% (highest level ever reported). Right temporal and cerebellar hemorrhages were present. Sequelae include severe developmental delay and asymmetric double hemiplegia.

Adult↗
PubMedDate not supplied

[Cow's milk protein sensitive enteropathy].

Cow's milk protein sensitive enteropathy (CMPSE) is characterized by the following items: 1. The great majority of affected infants have not been breast fed or only for a few days. Additional risks are immaturity, preceding enteritis, trisomy 21, and abdominal operation in the newborn. 2. Half of the patients become ill during the first two weeks after starting cow's milk formula. The main symptoms are watery, mucus containing diarrhea, vomiting, abdominal distension, pallor and rapid weight loss. 3. In CMPSE the small intestinal mucosa shows varying degrees of inflammation and villous atrophy. Bloody stools refer to large bowel affection. 4. CMPSE is always transitory and usually persists for less than one year. Inadequate treatment leads to "severe protracted diarrhea" or "intractable diarrhea" syndrome. Soya-based formula should not be the diet of first choice, since secondary intolerance to soya proteins will frequently develop. Exclusive breast feeding during the first months of life is the best prophylaxis of CMPSE.

Animals↗
PubMedDate not supplied

[Compression syndrome of the upper thoracic outlet (thoracic outlet syndrome)--report on a 13-year-old girl].

The thoracic-outlet-syndrome, a symptom complex consisting of arterial, venous and neural disorders of the upper extremity, is caused by compression of the neurovascular structures between the clavicle and the first rib. Other reasons are congenital abnormalities like a cervical rib, an abnormal first rib, acquired anatomic lesions like fractures and traumata or functional factors. The younger-and middle-age groups are most commonly involved. The symptoms are depending upon the point at which compression occurs and include pain, paresthesias, numbness, fatigue, pallor, coolness or swelling and enlargement of the arms. At physical examination a systolic murmur is heard in the supraclavicular region in 30 percent and the pulse of the upper extremity often is reduced. Diagnostic measurements include roentgenogram of the chest and the cervical spine, pulsed doppler-ultrasonography, nerve conduction-velocity testing and an arterio-venography. Treatment is nonoperative in patients with mild symptoms (shoulder-girdle-exercises) and operative in patients with severe or persistent symptoms. The operative treatment of choice is resection of the first rib by an axillary route. Risks are minimal, and the results are good to excellent in 90% of the cases. Because the thoracic outlet syndrome is very rare in childhood, we report a 13 year old girl which has undergone bilateral operation with excellent result.

Adolescent↗
PubMed1998

[Ocular changes in primary hematologic diseases].

Ocular manifestations can be the presenting symptom of hematological diseases. Ocular changes can be found in up to 90% of the patients depending on the underlying disease. Most patients with ocular manifestations however are asymptomatic. Hematological disorders can manifest in all structures and adnexa of the eye. The most common manifestations are conjunctival pallor and hemorrhages, intraretinal hemorrhages and cotton wool spots. Retinal infiltrates, manifestations in the lids, anterior segment, optic nerve, orbit and adnexa are rare. Different ocular manifestations in anemia, leukemia, malignant Non-Hodgkin lymphoma, Hodgkin's disease, chronic myeloproliferative diseases, plasmocytoma, myelodysplastic syndromes, coagulopathies, and reticulo-histiocytic diseases are described. Not only the underlying disease but also chemotherapy, bone marrow transplantation can lead to ocular complications.

Eye Diseases↗
PubMed1995

[Bilateral posterior ischemic optic neuritis in an adolescent with diabetes mellitus with decompensated blood glucose homeostasis].

BACKGROUND: Diabetogenic loss of visual acuity is very rare in juvenile onset diabetics. PATIENT: A 14-year-old adolescent with type-I diabetes mellitus since 7 years is reported. She experienced an episode of ketoacidotic diabetic coma after one year of very bad glycemic control. Even two weeks before onset of the diabetic coma her visual acuity decreased to 8/200 in both eyes. The perimetry revealed central and paracentral socotomas, the examination of the fundi showed only some microaneurysms and a normal optic disc. Electrophysiological results (normal ERG and EOG, pathologic pattern-VECP) proved irreversible damage of optic nerve or optic tract. RESULTS: Neuritis of optic nerve may not be existent but ischemic damage: the pattern-VECP showed no reproducible answers and the flash-VECP showed normal latency and borderline normal amplitude. There were no pathological findings in neurological and serological tests (like CT, NMR, EEG, cerebrospinal fluid, auto-antibodies or parasites). In the two years after visual loss the visual acuity increased only slightly to 13/200 in the right eye and 20/200 in the left. The optic disc showed temporal pallor in both eyes as sign of descending atrophy of the optic nerve. CONCLUSIONS: The clinical signs in this unusual case of diabetes mellitus point towards the diagnosis of a posterior ischemic optic neuropathy in both eyes caused by the extremely bad diabetic condition.

Adolescent↗