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Are there differences in clinical and laboratory parameters between children and adults with American visceral leishmaniasis?

A prospective study on 23 patients with American visceral leishmaniasis (VL), comparing clinical and laboratory parameters of 14 children (mean age of 3.85+/-3.39 years) to nine adults (27.4+/-10.90 years) was performed in São Luís, Maranhão, Brazil, between August 2000 and July 2002. Data were collected at entrance (day 0), end of treatment, as well as 120 and 210 days after treatment using a protocol chart containing patient identification, clinical and laboratory data. N-Methylglucamine antimonate administered at the dose of 20mg/Sb5+/kg/day for 20-30 days was successfully used in all patients. Patients were followed for 1 year after treatment, and no relapses were observed. A prolonged duration of the disease, lymphadenopathy and bleeding predominated in adult patients, while hepatomegaly and skin-mucosal pallor were more frequent in children. Disease was longer and more severe in adults than in children. Although both groups exhibited a trend toward normalization of hematological and biochemical parameters, more children returned sooner to normal values than adults. Difference in clinical or laboratory parameters between children and adults did not indicate the need for different clinical or therapeutic approaches.

Adolescent↗

Anatomic and visual outcomes after indocyanine green-assisted peeling of the retinal internal limiting membrane in idiopathic macular hole surgery.

PURPOSE: To report anatomic and visual outcomes after vitrectomy and adjunctive retinal internal limiting membrane (ILM) peeling with and without intravitreal indocyanine green for idiopathic macular hole repair. DESIGN: Retrospective comparative study of consecutive case series. METHODS: Three consecutive groups of idiopathic macular hole cases underwent modifications of surgical technique. Group I (48 eyes of 47 patients) underwent a standard vitrectomy, fluid/gas exchange, and 1 week's face-down positioning, group II (21 eyes of 21 patients) an adjunctive ILM peeling without use of indocyanine green, and group III (28 eyes of 28 patients) an adjunctive peeling of ILM stained with intravitreal application of 0.1 to 0.2 ml of 0.5% indocyanine green dye. RESULTS: Three groups of patients had comparable clinical characteristics as to age, gender, estimated duration of macular hole, preoperative visual acuity, and follow-up time. The rate of macular hole closure after a single surgery, as determined by optical coherence topography was 85.4% in group I, 85.7% in group II, and 100% in group III. Groups I and II showed a statistically significant visual improvement, but group III did not show significant visual acuity improvement as the mean logarithm of the minimal angle of resolution visual acuity was from 0.767 (20/120) preoperatively to 0.691 (20/100) postoperatively (P =.342). Eight cases in group III developed within a few postoperative months of optic disk pallor and irreversible peripheral visual field loss, predominantly affecting the nasal field. CONCLUSIONS: Intravitreal indocyanine green-assisted ILM peeling improves anatomic success in macular hole surgery, but it may potentially lead to unfavorable visual acuity outcome and peripheral visual field loss.

Adult↗

Clinical features associated with lesions other than pituitary adenoma in patients with an optic chiasmal syndrome.

PURPOSE: Pituitary adenomas are the most common cause of an optic chiasmal syndrome, and treatment of these lesions is considerably different from the treatment of most of the other lesions in this region. Although the diagnosis of a pituitary adenoma is usually inferred from the results of neuroimaging, lesions other than pituitary adenomas can have an appearance that suggests an adenoma. The objective of our study was to determine whether there are clinical findings that suggest a lesion producing a chiasmal syndrome is something other than a pituitary adenoma. DESIGN: Retrospective, case-controlled, analysis of medical record data. METHODS: The records of the Neuro-Ophthalmology Unit of the Wilmer Eye Institute were searched for patients with a chiasmal syndrome who had been evaluated before treatment and for whom pathologic or laboratory confirmation of the etiology was available. Presenting clinical features of these patients were recorded, and analyses with both a single variable and multiple variables were performed to determine whether there were any features that could identify with a high degree of probability the etiology of the lesion producing the syndrome. RESULTS: The search revealed 149 patients who met the inclusion criteria, including 90 patients with pituitary adenomas and 59 patients with other lesions. Variables that were highly suggestive of an etiology other than pituitary adenoma included symptomatic visual loss, younger age, unilateral optic disk pallor, a relative afferent pupillary defect, and an absolute or a complete visual field defect or one was greater inferiorly than superiorly. CONCLUSION: Although no single clinical feature can be used to determine the specific nature of a lesion that produces an optic chiasmal syndrome, certain features are highly suggestive of an etiology other than pituitary adenoma. When these features are present, the likelihood that a suprasellar lesion is a pituitary adenoma is much lower, regardless of the appearance on neuroimaging.

Adenoma↗

Bilateral visual loss in craniodiaphysial dysplasia.

PURPOSE: To report a rare case of craniodiaphysial dysplasia (CDD) that resulted in a profound loss of vision in both eyes. DESIGN: Observational case report. METHODS: A 2-year-old girl presented with midfacial anomaly. Marked thickening and sclerosis in the calvaria and facial bones were detected on the plain x-rays, which were compatible with CDD. Two years later, she visited our clinic because of visual loss in both eyes. RESULTS: The visual acuity was light perception in both eyes. The optic disk swelling with temporal pallor was observed in her both eyes. Orbital computed tomography scan revealed near-total obliteration of the optic canal in both eyes. CONCLUSIONS: CDD is a severe bone disorder characterized by massive generalized hyperostosis and sclerosis, especially involving the facial bones. Bony encroachment on the cranial foramina causes optic nerve compression, and this may lead to progressive visual impairment and ultimately to blindness.

Blindness↗

Cases from the Osler Medical Service at Johns Hopkins University.

A 47-year-old white woman with a history of stage III squamous cell carcinoma of the anus was transferred to Johns Hopkins Hospital for further evaluation of renal failure, hemolytic anemia, and thrombocytopenia. The patient was first diagnosed with squamous cell carcinoma of the anus 1 year before admission. She was treated with external beam radiation of the pelvis and two cycles of mitomycin C-based chemotherapy (a cumulative dose, 34 mg/m(2)). Her clinical course was complicated by Clostridium difficile colitis and myositis successfully treated with prednisone. Three months before admission, the patient developed dysuria. Her creatinine increased from normal to 1.7 mg/dL, and microscopic hematuria was present. A renal ultrasound and an abdominal computed tomographic scan showed no abnormalities or obstruction. One month before admission, she underwent a cystoscopy, which showed only radiation-induced changes in the bladder. Two weeks before admission, the patient became delirious and was taken to a hospital, where she was found to be anemic, with a hematocrit level of 23.7%, and thrombocytopenic with a platelet count of 110,000/mm(3). Her creatinine level was 5.9 mg/dL. Previous values of hematocrit, platelet count, and serum creatinine were normal. On admission at Johns Hopkins Hospital the patient had no complaints. She was afebrile on physical examination and had normal vital signs. Head, neck, chest, cardiovascular, and abdominal examinations were normal. There was skin pallor, but no echymoses or petechiae. She was alert and oriented with normal mental status. Her neurologic examination was normal. Laboratory data showed a white blood cell count of 6390/mm(3), a hematocrit level of 26.5%, and a platelet count of 26,000/mm(3). Her blood urea nitrogen level was 57 mg/dL, creatinine level was 4.0 mg/dL, and lactate dehydrogenase was 550 U/L (reference, 115 to 275 U/L). Urinalysis showed innumerable red blood cells and large protein. A peripheral blood smear showed fragmented red blood cells, schistocytes, no abnormal white blood cells, and few platelets. There was no radiographic or clinical evidence of relapse of her squamous cell carcinoma. What is the diagnosis?

Anemia, Hemolytic↗

[Ischaemia of the lower limbs: anaesthesia and intensive care].

OBJECTIVES: To appreciate the severity of a patient with acute limb ischaemia, to know how to manage these patients during the perioperative period. DATA SOURCES: References were obtained from PubMed data bank (http://www.ncbi.nlm.nih.gov/entrez/query.fcgi) using the following keywords: acute limb, ischaemia, prognosis, complications, rhabdomyolysis, hyperkalaemia, compartment syndrome, fasciotomy. DATA SYNTHESIS: Ischaemia of the lower limbs is a medico-surgical emergency. The ischaemia implies a decrease of cellular energetic stocks and an increase in intracellular calcium. During reperfusion, the calcium paradox is exacerbated and ROS formation produces membrane damage. Tissue oedema and a local and general inflammatory syndrome occur. Clinical symptoms of acute ischaemia include pallor, pulselessness, decrease of temperature and pain. Occurrence of neurological symptoms is a sign of severity. Prognosis of patients relates directly to preexisting collateral circulation, aetiology of the occlusion (thrombosis vs embolus), duration of ischaemia, topography of the occlusion (severity of proximal occlusions as the acute aortic occlusion), and co-morbidity (renal failure, heart failure). The temperature of the ischaemic limb, quality of the downstream circulation, extension of the thrombus, arterial pressure and association to a venous thrombosis are other prognostic factors of lower limb ischaemia. The first treatment to be initiated is high doses of heparin. Once the diagnosis is made, the number of preoperative tests will be as small as possible because of the urgency of revascularization. Arteriography will be performed only when really needed and when its realization will not delay revascularization and will not alter the patient's prognosis. Where general anesthesia is required, the choice of anaesthetic agents will be based on their haemodynamic stability. During severe acute limb ischaemia, monitoring of invasive pressure is recommended, as well as regular dosages of potassium, arterial gases and CPK. Preoperatively in case of severe ischaemia, (proximal occlusion lasting more than 6 hours), preventive treatment, including controlled reperfusion with heparinized serum is indicated. Surveillance and prevention of a rhabdomyolysis and renal failure are imperative. Immediately after reperfusion, a dosage of potassium must be performed; moreover that hyperkalaemia is favoured by acidosis or renal failure. Postoperative haemodialysis is performed in case of hyperkalaemia or renal failure. Occurrence of compartment syndrome has to be checked and fasciotomy must be performed in case of a doubt on the microcirculation integrity.

Acute Disease↗

[Rhytidectomy and Raynaud's phenomenon: about two cases].

Raynaud's phenomenon is a currently vascular syndrome (8 to 10% of women and 3 to 5% of men). It was defined as episodic ischaemia of the fingers, toes, nose, ears and nipples, which presents clinically as pallor, cyanosis, and often rubor of the skins, in response to cold, emotional stimuli and vasoconstriction agents. The phenomenon is caused by a vasoconstriction of arterials skin. In severe forms of the phenomenon, we can see ulcerations and necrosis. We report here two cases of rhytidectomy flap necrosis in Raynaud's Phenomenon. After review of literature, we explain the elements of physiopathology whose can explain these complications and we try to establish recommendations to these complications.

Female↗

[Ghosal haematodiaphyseal dysplasia: a new case].

Ghosal-type hematodiaphyseal dysplasia has been first described in 1986, as a steroid-dependent anemia with endosteal broadening of the long bone's diaphyses and metaphaphyses, which makes a distinction with the periosteal reaction in Camuratti-Engelmann's disease and with Caffey's disease. Extreme pallor is first noticed and leads to search for palpable thick long bones that are not always clinically obvious. The transmission of this rare entity seems to be autosomal recessive, with a common racial background from the Middle East and the Indian subcontinent. Anemia is considerably improved by steroids, which allow to avoid blood cells transfusions, but a minimum maintenance dose is necessary to keep the hemoglobin at a normal level probably during the whole life, involving iatrogen complications. The mechanism of anemia and the genetic cause of the disease are yet unknown.

Adolescent↗

[Severity assessment of acute diarrhoea].

Clinician should recognize any life-threatening causes of diarrhoea, such as intussusceptions, surgical abdomen, and haemolytic uraemic syndrome. The following clinical features should alert: abdominal pain with tenderness, with or without guarding, pallor, jaundice, oligo-anuria, bloody diarrhoea, systemically unwell out of proportion to the level of dehydration, shock. The risk of dehydration is related to age (highest in young infants<6 months), and frequency of watery stools (>8/day) and vomiting (>2/day before 1 year and >4/day after 1 year), but these historical points have a moderate sensitivity. The severity of dehydration is rarely estimated with accuracy in terms of weight loss (third sector with full colon, absence of accurate baseline pre-dehydration weight). Combinations of examination signs perform markedly better than any individual sign in predicting dehydration (poor rate agreement, clinically unhelpful likelihood ratio). The presence of at least three signs better correlate with dehydration. Laboratory tests are not helpful. New studies are mandatory to validate severity scoring systems.

Acute Disease↗

Space motion sickness: incidence, etiology, and countermeasures.

Space motion sickness is experienced by 60% to 80% of space travelers during their first 2 to 3 days in microgravity and by a similar proportion during their first few days after return to Earth. Space motion sickness symptoms are similar to those in other forms of motion sickness; they include: pallor, increased body warmth, cold sweating, malaise, loss of appetite, nausea, fatigue, vomiting, and anorexia. These are important because they may affect the operational performance of astronauts. Two hypotheses have been proposed to explain space motion sickness: the fluid shift hypothesis and the sensory conflict hypothesis. The fluid shift hypothesis suggests that space motion sickness results from the cranial shifting of body fluids resulting from the loss of hydrostatic pressure gradients in the lower body when entering microgravity. The cranial fluid shifts lead to visible puffiness in the face, and are thought to increase the intracranial pressure, the cerebrospinal-fluid pressure or the inner ear fluid pressures, altering the response properties of the vestibular receptors and inducing space motion sickness. The sensory conflict hypothesis suggests that loss of tilt-related otolith signals upon entry into microgravity causes a conflict between actual and anticipated signals from sense organs subserving spatial orientation. Such sensory conflicts are thought to induce motion sickness in other environments. Space motion sickness is usually treated using pharmaceuticals, most of which have undesirable side effects. Further studies elucidating the underlying mechanism for space motion sickness may be required for developing new treatments.

Antiemetics↗

Pure red cell aplasia--a rare disease with multiple causes.

Pure red cell aplasia (PRCA) is a relatively rare disease although multiple factors are implied in the pathogenesis of its development. A slow progressive normocytic-normochromic anemia and reticulocytopenia, without leukopenia and thrombocytopenia in a patient who, except pallor, does not show abnormal findings on physical examination, should arise the suspicion that he has PRCA. Search for underlying diseases or infections and intake of drugs may help for the establishment of the diagnosis of acquired PRCA. Lack of erythroblasts in the bone marrow with normal development of the other hemopoietic series, as well as high level of serum erythropoietin are important clues for the diagnosis. Elimination of potentially causative factors, administration of immunosuppressive agents and/or recombinant erythropoietin, preferably epoetin beta, may induce remission and complete recovery.

Adrenal Cortex Hormones↗

Merosin-negative congenital muscular dystrophy: magnetic resonance spectroscopy findings.

Congenital muscular dystrophies (CMD) are heterogeneous group of muscle disorders with autosomal recessive inheritance. Merosin deficiency has been identified in some patients with CMD all of whom also had white matter abnormalities on MRI. In postmortem studies, the brain showed extensive myelin pallor with a spongy appearance of white matter and moderate astrocytosis or demyelination. Direct assessment of neuropathologic aspects of MN-CMD such as demyelination is possible with MR spectroscopy (MRS). Although previous reports have described several neuro-imaging findings of this disease, MRS findings have not been reported in literature. In this case, we report MRS features of a 4-year old girl with MN-CMD. MRS of brain demonstrated that N-acetylaspartate (NAA)/Creatine (Cr) ratio was normal. Increased Choline (Cho)/Cr and Myo-inositol (MI)/Cr ratios were obtained. These findings were interpreted as demyelination and gliosis of white matter.

Brain↗

Relation between motion sickness susceptibility and vasovagal syncope susceptibility.

Motion sickness is common in the population, especially in children, but its physiopathology is only partially understood and the true nature of the particular susceptibility of certain subjects remains completely unknown. Some symptoms of motion sickness, like pallor and cold sweating, are of an autonomic nature and the role of the autonomic nervous system in vasovagal syncope is well known. Our aim was therefore to study the relationship between motion sickness susceptibility and vasovagal syncope susceptibility. Questionnaires about susceptibility to motion sickness and to vasovagal syncope or presyncope in adulthood and childhood, filled in by 899 students (20.4 +/- 2.1 years, 405 men), were analysed. Motion sickness susceptibility in childhood was 31.1% and in adulthood 7.9% (p < 0.001). Vasovagal syncope susceptibility in childhood was 36.4% and in adulthood 33.9% (NS). A relationship between motion sickness susceptibility in adulthood and vasovagal syncope susceptibility in childhood and adulthood (p = 0.004 and 0.005, respectively) was found. Despite the limitations of a retrospective study this relationship between motion sickness susceptibility and vasovagal syncope susceptibility may indicate that a common mechanism exists, explaining the particular susceptibility of some subjects to both disorders. This paradigm may prove useful in better understanding the true nature of motion sickness and vasovagal syncope.

Aging↗

Lymphomatosis cerebri as a cause of white matter dementia.

Primary central nervous system lymphoma most often presents as a solitary, isolated lesion in immunocompetent patients. Rarely, the disease presents as a diffuse, infiltrating condition without formation of a cohesive mass, a pattern called lymphomatosis cerebri. We present 3 immunocompetent individuals who developed rapidly progressive dementia. Magnetic resonance imaging features mimicked other disorders of white matter and prompted preoperative diagnoses of Binswanger's disease (subcortical ischemic vascular dementia), unknown leukoencephalopathy, viral infection, or infiltrating glioma. Neuropathologic examination at biopsy (Poon T, Matoso I, Tchertkoff V, Weitzner I Jr, Gade M. CT features of primary cerebral lymphoma in AIDS and non-AIDS patients. J Comput Assist Tomogr . 1989;13:6-9) and autopsy (Schwaighofer BW, Hesselink JR, Press GA, Wolf RL, Healy ME, Berthoty DP. Primary intracranial CNS lymphoma: MR manifestations. Am J Neuroradiol . 1993;10:725-9) demonstrated nonnecrotic, diffusely infiltrating, large-cell B-cell lymphoma of white matter, with relative sparing of gray matter, and without significant leptomeningeal involvement or bulky periventricular disease at autopsy. Microglial and astrocytic reactions, but only subtle myelin pallor, were evident as individual tumor cells permeated the entire brain and spinal cord, albeit with considerable variation in cell density. Individual tumor cells could be identified from the optic nerve to spinal cord, documenting the "whole-brain" nature of the disease. CD20 immunostaining was necessary to fully appreciate the extent of individual lymphoma cell percolation through the white matter. The neurobehavioral deficits manifested by these patients demonstrate that lymphomatosis cerebri is an additional neoplastic cause of white matter dementia and can be added to the growing list of disorders responsible for this syndrome.

Aged↗

Takayasu's arteritis: results of a university hospital of 45 patients in Turkey.

BACKGROUND: Takayasu's arteritis (TA) is a rare disease which appears to be most common in East Asia. However, the disease has been reported to be worldwide. The clinical features of the disease can show variations in different geographical areas. The aim of this study is to evaluate clinical, laboratory and radiological features and the outcome of patients with TA in our hospital. METHODS: The hospital files of patients who were followed with the diagnosis of TA between the years 1973 and 2003 in Hacettepe University Hospital were retrospectively evaluated. RESULTS: Male/female ratio was 5/40, and the mean age was 34 years (18-59). Constitutional symptoms were present in 71% of the patients. Claudication and pallor of the extremity, decreased extremity pulsations, arterial hypertension, and arterial bruits were present in 44%, 56%, 58%, and 27% of the patients, respectively. Aortic valvular insufficiency, abdominal aortic aneurism, and cardiomegaly were present in four, one, and four patients, respectively. The initial complaint of six patients was cerebrovascular events. The distribution of the patients according to the angiographic findings was as follows, 56% Type I, 18% Type II, 22% Type III, and 4% Type IV arteritis. The need for vascular surgical interventions were significantly less common in patients who were treated with immunosuppressives plus alternate dose steroids (6%) compared to patients who were treated only with antiaggregant agents (33%). CONCLUSIONS: The demographic and angiographic findings of our patients were similar to previous observations from Japan and Italy, and disclose distinct clinical features in comparison to other Asian countries. Alternate-day glucocorticoids plus cytotoxic drugs may be beneficial and safe in patients with TA.

Adolescent↗

Multiple branch retinal arteriolar occlusions associated with smallpox vaccination.

We describe a rare combination of multiple multiple branch retinal arteriolar occlusions (BRAO) and encephalopathy, which developed in an adult male 10 days after smallpox vaccination. A 53-year-old physician presented with sudden temporal visual field loss in the left eye. Ten days earlier he had received a smallpox vaccination. Hyperbaric oxygen was administered because of suspected BRAO. Examination on day 3 revealed a decrease in visual acuity and loss of visual field. Fundoscopy of the left eye disclosed a retinal infarct in the inferior macula and multiple cotton wool spots over the entire posterior pole. Fluorescein angiography showed delayed arterial filling and multiple non-perfused areas. Magnetic resonance imaging of the brain disclosed white matter focal lesions. Neurological examination was normal. Audiological examination revealed mild hearing loss caused by an old phonic trauma. A combination of intravenous methylprednisolone and immunoglobulins, recommended for the treatment of Susac syndrome, was administered. At the follow-up visit 3 months later, visual acuity in the left eye improved, fundoscopy showed resolution of the cotton wool spots and temporal disc pallor, and the visual field was substantially restored.

Anti-Inflammatory Agents↗

Evaluation of irregular menses in perimenarcheal girls: a pilot study.

Acyclic vaginal bleeding in girls within three years of menarche is most commonly attributed to an immature hypothalamic-pituitary-ovarian axis. Assuming this diagnosis may preclude the practitioner from performing more definitive studies and thereby diagnosing other, treatable causes of menstrual irregularities. A retrospective chart review of 178 girls presenting to an inner-city hospital-based adolescent clinic within three years of menarche was performed. Personal and family medical and menarcheal history was assessed, and findings on physical and laboratory examination performed were evaluated. Of the 178 girls still perimenarcheal at presentation, 47 were the focus of this study. Of these, 39 had no significant findings on physical examination, while 3 had signs of functional ovarian hyperandrogenism (FOH) including obesity, hirsutism, and moderate acne with corresponding LH/FSH ratios>3, although pelvic ultrasound examination revealed normal ovaries. Four of the 39 patients with normal physical exams had LH/FSH testing done, and 1 of the 4 had an abnormal LH/FSH ratio, indicating possible FOH. Two of the 47 patients were pregnant. Other laboratory abnormalities included microcytic, hypochromic anemia in patients, and an elevated Erythrocyte Sedimentation Rate in a patient later diagnosed with a rheumatologic disorder. Those perimenarcheal girls presenting with irregular menses and findings including obesity, acne, or pallor, were likely to have treatable causes of menstrual irregularities. In one of the four girls with a normal physical examination, hormonal testing indicated possible FOH, thus suggesting that hormonal evaluation of perimenarcheal girls with menstrual irregularities may be justified, as it may reveal previously unsuspected pathology.

Acne Vulgaris↗

Gastric volvulus in children.

PURPOSE: The aim of the study was to review the records of all children who presented with gastric volvulus in the past 10 years. METHODS: The study group consisted of 21 children with an age range from 0.2 months to 4.3 years who were operated for gastric volvulus from 1992 to 2003. RESULTS: Initial symptoms included acute abdominal pain after meals, vomiting, and in 8 cases, acute apnea associated with pallor, cyanosis, and hypotonia. After the first episode, barium studies revealed an organoaxial gastric volvulus in all cases. The surgical procedure was an anterior gastropexy with reinforcement of the esophagogastric angle performed by laparoscopy in 13 cases and by laparotomy in 8 (1 converted laparoscopy). An associated antireflux fundoplication was done in 3 patients. All children received postoperative antireflux medication for at least 1 month. The follow-up ranged from 4 months to 4.8 years. Two children in the laparotomy group required reoperation (Toupet fundoplication) for persistent gastroesophageal reflux disease. All children are currently symptom-free and without treatment. CONCLUSIONS: Gastric volvulus is a clinical and radiological reality, which can be treated by a gastropexy. Initial fundoplication is not mandatory. The laparoscopic gastropexy is a good option and allows a repeat laparoscopic procedure if needed.

Acute Disease↗