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Sample records for adysplasia pulmonary hypoplasia

  1. Hereditary renal adysplasia, pulmonary hypoplasia and Mayer-Rokitansky-Küster-Hauser (MRKH syndrome: a case report

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    Acién Maribel

    2010-04-01

    Full Text Available Abstract Background Hereditary renal adysplasia is an autosomal dominant trait with incomplete penetrance and variable expression that is usually associated with malformative combinations (including Müllerian anomalies affecting different mesodermal organs such as the heart, lung, and urogenital system. Case report A case showing pulmonary hypoplasia, hip dysplasia, hereditary renal adysplasia, and Mayer-Rokitansky-Kuster-Hauser syndrome in adulthood is reported here. The i.v. pyelography showed right renal agenesis with a normal left kidney and ureter. Ultrasound and Magnetic Resonance Imaging also showed right renal agenesis with multicystic embryonary remnants in the right hemipelvis probably corresponding to a dysgenetic kidney. An uretrocystoscopy showed absence of ectopic ureter and of the right hemitrigone. She was scheduled for a diagnostic laparoscopy and creation of a neovagina according to the McIndoe technique with a prosthesis and skin graft. Laparoscopy confirmed the absence of the uterus. On both sides, an elongated, solid, rudimentary uterine horn could be observed. Both ovaries were also elongated, located high in both abdominal flanks and somewhat dysgenetics. A conventional cytogenetic study revealed a normal female karyotype 46, XX at a level of 550 GTG bands. A CGH analysis was performed using a 244K oligoarray CGH detecting 11 copy number variants described as normal variants in the databases. The 17q12 and 22q11.21 microdeletions described in other MRKH patients were not present in this case. Four years after operation her evolution is normal, without symptoms and the neovagina is adequately functional. The geneticists have studied her family history and the pedigree of the family is shown. Conclusions We suggest that primary damage to the mesoderm (paraaxil, intermediate, and lateral caused by mutations in a yet unidentified gene is responsible for: 1 skeletal dysplasia, 2 inappropriate interactions between the bronchial

  2. Unilateral pulmonary hypoplasia

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    Albay S

    2008-10-01

    Full Text Available Pulmonary hypoplasia represents a broad range of malformations characterized by incomplete development of lung tissue. The severity of the lesion depends on the appearance time of the malformation during the timeline of lung development, and the presence of further anatomic anomalies. In this report, we present a case of pulmonary hypoplasia in a 27-year old woman.

  3. Hemifacial microsomia with pulmonary hypoplasia

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    Panigrahi, Inusha; Das, Rashmi Ranjan; Marwaha, Ram Kumar

    2010-01-01

    Hemifacial microsomia (HFM) is the second most common facial anomaly after cleft lip and palate. HFM is characterised by facial asymmetry, microtia, preauricular tags, macrostomia and cardiac defects. The majority of cases are sporadic, although autosomal dominant and recessive modes of inheritance have been reported. Here, the case of an 11-month-old boy with HFM and pulmonary hypoplasia, which is a rare association, is described. Pulmonary hypoplasia was detected during investigation of the...

  4. Aberrant left pulmonary artery associated with right pulmonary hypoplasia

    International Nuclear Information System (INIS)

    Aberrant left pulmonary artery (ALPA), or pulmonary artery sling, is an uncommon vascular malformation that is frequently associated with obstructive disorders of the tracheobronquial tree. In newborns, it produces severe respiratory problems. In contrast, in adults, it is usually discovered by change. ALPA has been associated with right pulmonary hypoplasia (RPH) in a small number of cases. We present a new case of ALP associated with right pulmonary hypoplasia in an adult woman, diagnosed by CT and MR. 12 refs

  5. Causes of congenital unilateral pulmonary hypoplasia

    International Nuclear Information System (INIS)

    A review of the roentgenograms and clinical records of 33 children with primary congenital underdevelopment of one lung showed that 9 patients had simple pulmonary hypoplasia, 8 had anomalous venous return to the right atrium or the inferior vena cava (scimitar syndrome), 7 had an absence of ipsilateral pulmonary artery, 7 had an accessory diaphragm, and 2 had a pulmonary sequestration adjacent to a small diaphragmatic hernia. (orig.)

  6. Evaluation of fetal pulmonary hypoplasia by MR signal intensity

    International Nuclear Information System (INIS)

    Magnetic resonance (MR) imaging plays an important role in prenatal diagnosis and perinatal treatment. We correlated low intensity of the fetal lung after 25 weeks' gestation and neonatal respiratory distress in 35 fetuses. We employed MR imaging using the half-Fourier acquisition, single-shot, turbo spin-echo (HASTE) sequence. In all fetuses, signal intensity was lower in the lung than in the liver, and all infants subsequently had respiratory distress at birth. In 9 autopsy cases, pulmonary hypoplasia was established as the cause of death. Despite the small number of cases examined in this study, low signal intensity in the fetal lung may be a predictor of respiratory distress requiring mechanical ventilation at birth. (author)

  7. Down-Regulation of Sonic Hedgehog Expression in Pulmonary Hypoplasia Is Associated with Congenital Diaphragmatic Hernia

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    Unger, Sharon; Copland, Ian; Tibboel, Dick; Post, Martin

    2003-01-01

    The pathogenesis of pulmonary hypoplasia associated with congenital diaphragmatic hernia (CDH) is unknown. The sonic hedgehog (Shh) cascade is crucial for the patterning of the early respiratory system in mice. To establish whether Shh plays a role in the pathogenesis of lung hypoplasia in CDH, we investigated the gestation-specific expression of Shh in normal rat and human lungs using in situ hybridization and immunohistochemistry. The expression pattern was compared with that of age-matched...

  8. Abnormal fetal movements, micrognathia and pulmonary hypoplasia: a case report. Abnormal fetal movements

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    Morokuma Seiichi

    2010-08-01

    Full Text Available Abstract Background Micrognathia is a facial malformation characterized by mandibular hypoplasia and a small, receding chin that fails to maintain the tongue in a forward position. We previously reported a system of prenatal screening that we developed to identify fetuses with compromised central nervous system function by observing fetal behavior. In this paper we report the case of a preterm infant with micrognathia and pulmonary hypoplasia who presented abnormal fetal movements. Case presentation A 27-year-old Japanese primigravida at 33 weeks of gestation was referred to our hospital. Ultrasonographic examination revealed clinical polyhydramnios. Micrognathia was evident on midsagittal and 3 D scan. The lung area was less than the mean -2.0 standard deviations for the gestational age. The infant had mandibular hypoplasia and glossoptosis. After emergency cesarean delivery for non-reasuring fetal status, required immediate tracheostomy and cardiopulmonary resuscitation with mechanical ventilatory support. However, the infant's cardiopulmonary condition did not improve and she died 21 hours after birth. Conclusions The findings of our ultrasound exam are suggestive of brain dysfunction. The observation of fetal behavior appears to be effective for the prediction of prognosis of cases with micrognathia.

  9. EXTRAPULMONARY SEQUESTRATION WITH PULMONARY HYPOPLASIA AND MULTICYSTIC RENAL DYSPLASIA : A RARE CASE REPORT

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    Anita

    2015-05-01

    Full Text Available Pulmonary sequestration is a rare anomaly which consists of the presence of pulmonary tissue that is not attached to the rest of the lung and does not communicate with the trachea. [1] It could be intrapulmonary or extrapulmonary. We report a case of extrapulmonary sequestration with brief review of literature. A 22 years old primigravida underwent an ultrasonography at 24 weeks of gestation which revealed a single live fetus with bilateral pleural effusion, fetal hydrops and the fetal thorax showed mediastinal shift to the right. A hyperechoic mass was present in the left thoracic cavity with a systemic blood supply to it. Termination of pregnancy was advised as the findings were incompatible with life and the fetus autopsied. Significant gross findings were a hypoplastic left lung, a grey - white spongy mass adjacent to the left lung but no t attached to it and present outside the pleural cavity which derived its blood supply via a branch from the thoracic aorta and caused a shift in the mediastinal structures to the right. Both kidneys showed multiple cystic spaces. Microscopically the mass showed multiple cystically dilated alveolar spaces and ducts lined by cuboidal to tall columnar epithelium, the left lung showed features of pulmonary hypoplasia and the microscopic findings in both the kidneys were suggestive of multicystic renal dysplasi a. Hence, it was reported as a case of left sided extrapulmonary sequestration with hypoplastic left lung and bilateral renal cystic dysplasia.

  10. Clinical results of combined palliative procedures for cyanotic congenital heart defects with intractable hypoplasia of pulmonary arteries

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    FAN Xiang-ming; ZHU Yao-bin; SU Jun-wu; ZHANG Jing; LI Zhi-qiang; XU Yao-qiang; LI Xiao-feng

    2013-01-01

    Background Congenital heart defects with intractable hypoplasia of the pulmonary arteries without intercourse or with intercourse stenosis is unsuitable for surgical correction or regular palliative procedures.We reported our experience with combined palliative procedures for congenital heart defects with intractable hypoplasia pulmonary arteries.Methods From 2001 to 2012,a total of 41 patients with cyanotic congenital heart defects and intractable hypoplasia of the pulmonary arteries underwent surgical procedures.From among them,31 patients had pulmonary atresia with ventricular septal defect (VSD) and the other 10 cases had complicated congenital heart defects with pulmonary stenosis.Different kinds of palliative procedures were performed according to the morphology of the right and left pulmonary arteries in every patient.If the pulmonary artery was well developed,a Glenn procedure was performed.A modified Blalock-Taussi9 shunt or modified Waterston shunt was performed if pulmonary arteries were hypoplastic.If the pulmonary arteries were severely hypoplastic,a Melbourne shunt was performed.Systemic pulmonary artery shunts were performed bilaterally in 25 cases.A systemic-pulmonary shunt was performed on one side and a Glenn procedure was performed contralaterally in 16 cases.Major aortopulmonary collateral arteries were unifocalized in six cases,ligated in two cases and interventionally embolized in two cases.There was one early death because of cardiac arrest and the hospital mortality was 2.4%.Results Five patients suffered from postoperative low cardiac output syndrome,three had perfusion of the lungs,and two pulmonary infections.Systemic pulmonary shunts were repeated after the original operation in three cases due to the occlusion of conduits.The mean follow-up time was 25 months.The pre-and the post-operation left pulmonary indices were (8.13±3.68) vs.(14.9±6.21) mm2/m2.The pre-and post-operation right pulmonary indices were (12.7±8.13) vs.(17.7±7

  11. Hereditary renal adysplasia: new observations and hypotheses.

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    Moerman, P; Fryns, J P; Sastrowijoto, S H; Vandenberghe, K; Lauweryns, J M

    1994-01-01

    Renal agenesis and dysplasia are frequently regarded by pathologists, even pediatric pathologists, as sporadic malformations. We report six fetal autopsy cases of hereditary renal adysplasia (HRA): two pairs of siblings, one case with paternal unilateral renal agenesis, and one case with an autosomal balanced 6p/19q translocation. The main purpose of this paper is to emphasize that nonsyndromal renal agenesis and dysplasia are pathogenetically related and often inherited as an autosomal dominant trait with incomplete penetrance and variable expression. A subsidiary purpose is to present a case of bilateral multicystic dysplasia with a balanced 6p/19q translocation. This observation further supports the assignment of one of the loci for HRA to chromosome 6p. PMID:8065999

  12. Cerebellar Hypoplasia

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    ... disorders that begin in early childhood, such as ataxia telangiectasia. In an infant or young child, symptoms of a disorder that features cerebellar hypoplasia might include floppy muscle tone, developmental or ...

  13. Bilateral cystic dysplasia of the rete testis with renal adysplasia.

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    Bouron-Dal Soglio, Dorothée; Harvey, Isabelle; Jovanovic, Mubina; Oligny, Luc L; Fournet, Jean-Christophe

    2006-01-01

    Cystic dyplasia of the rete testis (CDRT) is an uncommon, generally unilateral lesion characterized by anastomosing cystic spaces lined by a flattened simple cuboidal epithelium in the rete testis. In the literature this lesion often is associated with an ipsilateral urogenital lesion such as renal agenesia or multicystic dysplasia of the kidney, in order of frequency. The pathogenesis is explained by some authors by their common embryologic origin. We are reporting the finding of bilateral CDRT associated with ultrasound-diagnosed renal adysplasia in a 20-week gestational age fetus with oligohydramnios. Although CDRT has been referred to as being associated with multicystic renal dysplasia or renal agenesis, the present case appears to be unique in combining all the malformations together. PMID:16822083

  14. Pulmonary hypoplasia on preterm infant associated with diffuse chorioamniotic hemosiderosis caused by intrauterine hemorrhage due to massive subchorial hematoma: report of a neonatal autopsy case.

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    Yamada, Sohsuke; Marutani, Takamitsu; Hisaoka, Masanori; Tasaki, Takashi; Nabeshima, Atsunori; Shiraishi, Mika; Sasaguri, Yasuyuki

    2012-08-01

    A male infant born prematurely at 31 weeks of gestation weighed 789 g and had mildly brown-colored oral/tracheal aspirates at delivery. The amniotic fluid was also discolored, and its index was below 5. The patient died of hypoxemic respiratory and cardiac failure 2 hours after birth. The maternal profiles showed placenta previa and intrauterine growth restriction (IUGR) at 22 weeks of gestation, and revealed recurrent episodes of antenatal and substantial vaginal bleeding and oligohydramnios, indicating chronic abruption-oligohydramnios sequence. The thickened placenta, weighing 275 g, grossly displayed unevenness and diffuse opacity with green to brown discoloration in the chorioamniotic surface, and revealed chronic massive subchorial hematomas (Breus' mole) with old peripheral blood clot, circumvallation, and infarction. Microscopically, diffuse Berlin-blue staining-positive hemosiderin deposits were readily encountered in the chorioamniotic layers of the chorionic plate, consistent with diffuse chorioamniotic hemosiderosis (DCH) due to Breus' mole, accompanied by diffuse amniotic necrosis. At autopsy, an external examination showed several surface anomalies and marked pulmonary hypoplasia, 0.006 (less 0.012) of lung:body weight ratio. Since Breus' mole has a close relationship with intrauterine hemorrhage, resulting in DCH, IUGR, and/or pulmonary hypoplasia of the newborn, the present features might be typical. PMID:22827763

  15. Inherited neuroaxonal dystrophy in dogs causing lethal, fetal-onset motor system dysfunction and cerebellar hypoplasia

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    Fyfe, John C.; Al-Tamimi, Raba' A.; Castellani, Rudy J.; Rosenstein, Diana; Goldowitz, Daniel; Henthorn, Paula S.

    2010-01-01

    Neuroaxonal dystrophy in brainstem, spinal cord tracts, and spinal nerves accompanied by cerebellar hypoplasia was observed in a colony of laboratory dogs. Fetal akinesia was documented by ultrasonographic examination. At birth, affected puppies exhibited stereotypical positioning of limbs, scoliosis, arthrogryposis, pulmonary hypoplasia, and respiratory failure. Regional hypoplasia in the central nervous system was apparent grossly, most strikingly as underdeveloped cerebellum and spinal cor...

  16. Vermian aplasia and hypoplasia

    International Nuclear Information System (INIS)

    This paper reports vermian aplasia or hypoplasia unrelated to Dandy-Walker complex or Joubert syndrome. Vermian hypoplasia is most commonly associated with the malformations of Dandy-Walker cyst or variant. There have been few reports of familial vermian anomaly (Joubert syndrome) with episodes of polypnea and apnea. Five patients with vermian hypoplasia or absence were prospectively discovered among 1,130 children undergoing brain MR imaging. Ages ranged from 2 mo to 15 y at the time of discovery: The abnormalities were poorly defined in three patients who had previously undergone CT. There was no common link in these patients with a variable number of other systemic, brain, or facial abnormalities. No unifying symptoms were identified. Two patients had the MR imaging appearance of Joubert syndrome, but none had the cyclical respiratory symptoms usually associated with this syndrome

  17. Cerebral white matter hypoplasia

    International Nuclear Information System (INIS)

    This paper demonstrates the MR imaging findings in children with cerebral white matter hypoplasia (CWMH). The MR studies of four children, aged 3-7 y (mean age, 2.3 y) with a diagnosis of CWMH were reviewed. In all cases multiplanar T1-weighted and T2-weighted spin-echo images were obtained. All children had similar histories of severe developmental delay and nonprogressive neurologic deficits despite normal gestational and birth histories. In two cases there was a history of maternal cocaine abuse. Autopsy correlation was available in one child. The MR images of all four children demonstrated diffuse lack of white matter and enlarged ventricles but normal-appearing gray matter. The corpus callosum, although completely formed, was severely thinned. There was no evidence of gliosis or porencephaly, and the distribution of myelin deposition was normal for age in all cases. Autopsy finding in one child correlated exactly with the MR finding

  18. Biliary hypoplasia in Williams syndrome

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    O'Reilly, K; Ahmed, S F; Murday, V.; McGrogan, P

    2006-01-01

    Neonatal hepatitis and biliary hypoplasia are not recognised features of Williams syndrome. A case of Williams syndrome, presenting with neonatal conjugated hyperbilirubinaemia leading to an initial misdiagnosis is reported.

  19. Optic nerve hypoplasia

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    Savleen Kaur

    2013-01-01

    Full Text Available Optic nerve hypoplasia (ONH is a congenital anomaly of the optic disc that might result in moderate to severe vision loss in children. With a vast number of cases now being reported, the rarity of ONH is obviously now refuted. The major aspects of ophthalmic evaluation of an infant with possible ONH are visual assessment, fundus examination, and visual electrophysiology. Characteristically, the disc is small, there is a peripapillary double-ring sign, vascular tortuosity, and thinning of the nerve fiber layer. A patient with ONH should be assessed for presence of neurologic, radiologic, and endocrine associations. There may be maternal associations like premature births, fetal alcohol syndrome, maternal diabetes. Systemic associations in the child include endocrine abnormalities, developmental delay, cerebral palsy, and seizures. Besides the hypoplastic optic nerve and chiasm, neuroimaging shows abnormalities in ventricles or white- or gray-matter development, septo-optic dysplasia, hydrocephalus, and corpus callosum abnormalities. There is a greater incidence of clinical neurologic abnormalities in patients with bilateral ONH (65% than patients with unilateral ONH. We present a review on the available literature on the same to urge caution in our clinical practice when dealing with patients with ONH. Fundus photography, ocular coherence tomography, visual field testing, color vision evaluation, neuroimaging, endocrinology consultation with or without genetic testing are helpful in the diagnosis and management of ONH. (Method of search: MEDLINE, PUBMED.

  20. Optic nerve hypoplasia.

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    Kaur, Savleen; Jain, Sparshi; Sodhi, Harsimrat B S; Rastogi, Anju; Kamlesh

    2013-05-01

    Optic nerve hypoplasia (ONH) is a congenital anomaly of the optic disc that might result in moderate to severe vision loss in children. With a vast number of cases now being reported, the rarity of ONH is obviously now refuted. The major aspects of ophthalmic evaluation of an infant with possible ONH are visual assessment, fundus examination, and visual electrophysiology. Characteristically, the disc is small, there is a peripapillary double-ring sign, vascular tortuosity, and thinning of the nerve fiber layer. A patient with ONH should be assessed for presence of neurologic, radiologic, and endocrine associations. There may be maternal associations like premature births, fetal alcohol syndrome, maternal diabetes. Systemic associations in the child include endocrine abnormalities, developmental delay, cerebral palsy, and seizures. Besides the hypoplastic optic nerve and chiasm, neuroimaging shows abnormalities in ventricles or white- or gray-matter development, septo-optic dysplasia, hydrocephalus, and corpus callosum abnormalities. There is a greater incidence of clinical neurologic abnormalities in patients with bilateral ONH (65%) than patients with unilateral ONH. We present a review on the available literature on the same to urge caution in our clinical practice when dealing with patients with ONH. Fundus photography, ocular coherence tomography, visual field testing, color vision evaluation, neuroimaging, endocrinology consultation with or without genetic testing are helpful in the diagnosis and management of ONH. (Method of search: MEDLINE, PUBMED). PMID:24082663

  1. MR findings in pontocerebellar hypoplasia

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    Uhl, M.; Pawlik, H.; Laubenberger, J.; Langer, M. [Department of Radiology, Division of Paediatric Radiology, University Hospital of Freiburg, Freiburg (Germany); Darge, K. [Department of Paediatric Radiology, Children`s Hospital of the University of Heidelberg, Heidelberg (Germany); Baborie, A. [Department of Neuropathology, Neurozentrum, University of Freiburg, Freiburg (Germany); Korinthenberg, R. [Department of Neuropaediatrics, Children`s Hospital, University of Freiburg, Freiburg (Germany)

    1998-07-01

    We present four cases with combined hypoplasia of the cerebellum and the ventral pons - pontocerebellar hypoplasia (PCH). PCH represents an autosomal recessive neurodegenerative disorder with fetal onset. The disease is rare, with less than 20 cases having been reported. The main findings of PCH and the inclusion criteria for our cases can be summarised as progressive microcephaly from birth, pontocerebellar hypoplasia documented by MRI and marked chorea, which may change, later in childhood, to more dystonic patterns. The cerebral cortex becomes progressively atrophic. Motor and mental development are delayed, and epilepsy, mainly tonic-clonic seizures, is frequent. The MRI features in all of our cases were: (1) Hypoplastic cerebellum situated close to the tentorium. The hypoplastic cerebellum has a reduced number of folia, in contrast to the normal number of thin folia in simple cerebellar atrophy. (2) The cerebellar hemispheres are reduced to bean-like or wing-like structures. The cerebellar hemispheres appear to `float` in the posterior fossa. (3) Markedly hypoplastic ventral pons. (4) Slight atrophy of the supratentorial gyral pattern. (5) Dilated cerebromedullary cistern and fourth ventricle. (6) Delayed myelination of the white matter. (7) No significant disorganisation of brain architecture and no severe corpus callosum defect. (orig.) With 3 figs., 2 tabs., 13 refs.

  2. Hypoplasia of exocrine pancreas with myocardial necrosis

    Institute of Scientific and Technical Information of China (English)

    Xiang Sheng Zhang; Xin Xiu Xu; Yan Zhang; Sbu Hua Wu

    2000-01-01

    AIM To study the clinical and pathological features of hypoplasia of exocrine pancreas with myocardialnecrosis.METHODS One ease of hypoplasia of exocrine pancreas with myocardial necrosis was autopsied. Theclinical signs and pathological changes were analyzed.RESULTS A 15-month-old boy with hypoplasia of exocrine pancreas was reported. The main clinicalfeatures were steatorrhea and marked underdevelopment. He died of acute heart failure afterhospitalization. Autopsy showed that there were aplasia of exocrine portion and fatty metaplasia ofpancreas, the myocardium revealed focal necrosis and sear formation.CONCLUSION Atrophy of exocrine pancreas and myocardial necrosis exist at the same time, suggestingthat there may be some relationship between them. It was likely that the damaged pancreatic tissue releasedsome active materials that may harm the myocardium or decrease pancreatic juice that results in lack ofnutrient and myocardial necrosis.

  3. THE SYNDROME OF AUTOSOMAL RECESSIVE PONTOCEREBELLAR HYPOPLASIA, MICROCEPHALY, AND EXTRAPYRAMIDAL DYSKINESIA (PONTOCEREBELLAR HYPOPLASIA TYPE-2) - COMPILED DATA FROM 10 PEDIGREES

    NARCIS (Netherlands)

    BARTH, PG; BLENNOW, G; LENARD, HG; BEGEER, JH; VANDERKLEY, JM; HANEFELD, F; PETERS, ACB; Valk, J.

    1995-01-01

    The syndrome of autosomal recessive pontocerebellar hypoplasia, microcephaly, severely impaired mental and motor development, and extrapyramidal dyskinesia is a distinct system degeneration, previously designated pontocerebellar hypoplasia type 2 (PCH-2). To further characterize its clinical and neu

  4. Enamel hypoplasia: challenges of esthetic restorative treatment.

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    Ruschel, Vanessa Carla; Araújo, Élito; Bernardon, Jussara Karina; Lopes, Guilherme Carpena

    2016-01-01

    Enamel defects, such as white or yellow-brown spots, usually cause problems that are more esthetic than functional. Enamel hypoplasia may be the result of hereditary, systemic, or local factors. Dental trauma is a local etiologic factor. It is relatively common in the primary dentition and can cause defects on the surface of permanent successors. Treatment for such defects can differ, depending on the depth of the spots. For deeper white-spot lesions, a composite resin restoration may be necessary. This is an excellent mode of treatment, due to both its low cost and its conservation of healthy tooth structure. The objective of this case report is to describe composite resin restoration of a maxillary central incisor affected by enamel hypoplasia. PMID:27599287

  5. Bilateral hind limb hypoplasia in a foal

    International Nuclear Information System (INIS)

    Hypoplasia affecting both hind limbs was observed in a neonatal foal. Scintigraphy aided in establishing diagnosis and prognosis. Radiography revealed an abnormally shaped middle phalanx and lack of the distalphalanx and navicular bone in the right hind limb, with an abnormally shaped distal phalanx and navicular bone in the left hind limb. Scintigraphic findings were closely correlated with the radiographic findings. Radiographic and scintigraphic findings were confirmed at postmortem examination

  6. Radicular Dentin Dysplasia Associated with Enamel Hypoplasia

    International Nuclear Information System (INIS)

    The author observed a rare case of radicular dentin dysplasia associated with enamel hypoplasia in a 11-year-old boy with a complaint of gum boil formation. 1. Clinically, yellowish-brown colored teeth with severe attrition and several gum boils were observed. 2. Radiographically, obliteration of pulp chamber and root canal, multiple periapical radiolucencies without obvious cause and blunt roots were observed. 3. Systemically, scalp hair and eyebrows were loose and short. And saddle nose could be also seen.

  7. Rate of enamel formation and hypoplasia timing

    OpenAIRE

    Al-Shorman, Abdulla; Alrousan, Mohammad; Khwaileh, Ali

    2014-01-01

    Linear Enamel Hypoplasia (LEH) is a decrease in enamel thickness resulting from a temporary disruption in enamel formation during times of dental development. To establish a statistical relationship between the timing of enamel formation and the length of the crown, the published data of Reid and Dean (2006) were statistically analyzed using SPSS. The results indicate that the relationship between the crown length and the days of enamel formation is nonlinear as proposed by previous studies b...

  8. Soft palate hypoplasia in a horse

    International Nuclear Information System (INIS)

    Several imaging techniques were used to diagnose hypoplasia of the soft palate in a horse. The absence of the caudal soft palate, hypertrophied lymphoid tissue and the formation of a pseudouvula were observed endoscopically. Plain and contrast radiography were used to demonstrate a soft palate remnant and to identify structures rostral to the epiglottis. Retrograde endoscopy of the pharynx via a tracheotomy incision is described

  9. Pulmonary Vein Stenosis in a Newborn: A Commonly Overlooked Diagnosis

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    Nathalie Jeanne Magioli Bravo-valenzuela; Guilherme Ricardo Nunes Silva; Marcela Pinto Varella

    2015-01-01

    The diagnosis of primary pulmonary vein stenosis is often overlooked because its symptoms overlap lung diseases and pulmonary arterial hypertension. Its diagnosis may be difficult because the condition is progressive and associated with other defects. We present a case of pulmonary vein stenosis in a newborn with stenosis of the left-sided common pulmonary vein, diffuse hypoplasia of the superior right pulmonary vein, and atresia of the inferior right pulmonary vein.

  10. Pontoneocerebellar hypoplasia: definition of MR features

    International Nuclear Information System (INIS)

    We report five patients with pontoneocerebellar hypoplasia. Because the issue of cerebellar malformations is a difficult subject, we tried to define criteria for diagnosis on MRI: a thin flat pons with disappearance of the anterior curve, a small cerebellum with predominant flattening of the hemispheres and shortened cerebellar fissures, in contrast to atrophy. The posterior fossa is not enlarged. We emphasize the probable late onset of the disease in fetal life because of the demonstration of the abnormalities at US during the last trimester of the pregnancy in one patient. Prenatal diagnosis is important because of possible autosomal recessive transmission. (orig.)

  11. Spectral domain optical coherence tomography and microperimetry in foveal hypoplasia

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    Swakshyar Saumya Pal

    2011-01-01

    Full Text Available A case of foveal hypoplasia associated with ocular albinism with anatomic and functional changes by various techniques using spectral domain optical coherence tomography (SD-OCT, microperimeter and confocal scanning laser ophthalmoscope is described. This case highlights the importance of microperimeter in detecting the functional abnormalities of vision and SD-OCT in identifying the retinal laminar abnormalities in foveal hypoplasia.

  12. Classification, diagnosis and potential mechanisms in pontocerebellar hypoplasia

    NARCIS (Netherlands)

    Y. Namavar; P.G. Barth; B.T. Poll-The; F. Baas

    2011-01-01

    Pontocerebellar Hypoplasia (PCH) is group of very rare, inherited progressive neurodegenerative disorders with prenatal onset. Up to now seven different subtypes have been reported (PCH1-7). The incidence of each subtype is unknown. All subtypes share common characteristics, including hypoplasia/atr

  13. Brainstem hypoplasia presenting with mirror movement

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    Burcu Ekmekçi

    2015-09-01

    Full Text Available 20 years old female patient, who had operated from congenital syndactyly on her left hand at five age, admitted to neurology policlinic with involuntary movement on her hands. We saw mirror movement (MM when she writing, catching with her left hand. This movement is had low amplitude in the right hand than left. Cervical MRG revealed no abnormality. Brain MRG revealed right middle, inferior cerebellary peduncle, olive and pyramid hypoplasia. Mirror movement shows homolog muscle activity which simulating contralateral movement, during a spesific task. This movement is seen usually upper extremity especially in the hand. Corticospinal tract dysfunction is often considered in the pathogenesis. MM may present as part of cervico medullary junction abnormality, cerebral palsy, cerebrovasculary disease, Parkinson disease. We wanted to discuss the patogenesis of MM in our patient with syndactyly and MRG abnormality.

  14. Brain stem hypoplasia associated with Cri-du-Chat syndrome

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    Hong, Jin Ho; Lee, Ha Young; Lim, Myung Kwan; Kim, Mi Young; Kang, Young Hye; Lee, Kyung Hee; Cho, Soon Gu [Dept. of Radiology, Inha University Hospital, Inha University School of Medicine, Incheon (Korea, Republic of)

    2013-12-15

    Cri-du-Chat syndrome, also called the 5p-syndrome, is a rare genetic abnormality, and only few cases have been reported on its brain MRI findings. We describe the magnetic resonance imaging findings of a 1-year-old girl with Cri-du-Chat syndrome who showed brain stem hypoplasia, particularly in the pons, with normal cerebellum and diffuse hypoplasia of the cerebral hemispheres. We suggest that Cri-du-Chat syndrome chould be suspected in children with brain stem hypoplasia, particularly for those with high-pitched cries.

  15. Brain stem hypoplasia associated with Cri-du-Chat syndrome

    International Nuclear Information System (INIS)

    Cri-du-Chat syndrome, also called the 5p-syndrome, is a rare genetic abnormality, and only few cases have been reported on its brain MRI findings. We describe the magnetic resonance imaging findings of a 1-year-old girl with Cri-du-Chat syndrome who showed brain stem hypoplasia, particularly in the pons, with normal cerebellum and diffuse hypoplasia of the cerebral hemispheres. We suggest that Cri-du-Chat syndrome chould be suspected in children with brain stem hypoplasia, particularly for those with high-pitched cries.

  16. Enamel hypoplasia in the middle pleistocene hominids from Atapuerca (Spain).

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    Bermúdez de Castro, J M; Pérez, P J

    1995-03-01

    The prevalence and chronology of enamel hypoplasias were studied in a hominid dental sample from the Sima de los Huesos (SH) Middle Pleistocene site at the Sierra de Atapuerca (Burgos, northern Spain). A total of 89 permanent maxillary teeth, 143 permanent mandibular teeth, and one deciduous lower canine, belonging to a minimum of 29 individuals, were examined. Excluding the antimeres (16 maxillary and 37 mandibular cases) from the sample, the prevalence of hypoplasias in the permanent dentition is 12.8% (23/179), whereas the deciduous tooth also showed an enamel defect. No statistically significant differences were found between both arcades and between the anterior and postcanine teeth for the prevalence of hypoplasias. In both the maxilla and the mandible the highest frequency of enamel hypoplasias was recorded in the canines. Only one tooth (a permanent upper canine) showed two different enamel defects, and most of the hypoplasias were expressed as faint linear horizontal defects. Taking into account the limitations that the incompleteness of virtually all permanent dentitions imposes, we have estimated that the frequency by individual in the SH hominid sample was not greater than 40%. Most of the hypoplasias occurred between birth and 7 years (N = 18, X = 3.5, SD = 1.3). Both the prevalence and severity of the hypoplasias of the SH hominid sample are significantly less than those of a large Neandertal sample. Furthermore, prehistoric hunter-gatherers and historic agricultural and industrial populations exhibit a prevalence of hypoplasias generally higher than that of the SH hominids. Implications for the survival strategies and life quality of the SH hominids are also discussed. PMID:7785727

  17. Inhalation of nitric oxide as a treatment of pulmonary hypertension in congenital diaphragmatic hernia

    DEFF Research Database (Denmark)

    Henneberg, Steen Winther; Jepsen, S; Andersen, P K; Pedersen, S A

    1995-01-01

    Congenital diaphragmatic hernia (CDH) still has a mortality risk of around 40%. The concomitant pulmonary hypoplasia and the persistent pulmonary hypertension are of major prognostic importance. The use of a selective pulmonary vasodilator may revert this vicious circle that is fatal to many chil...

  18. Unilateral segmental odontomaxillary hypoplasia: an unusual case report

    Energy Technology Data Exchange (ETDEWEB)

    Pandey, Sushma; Pai, Keerthilatha M; Nayak, Ajay G; Vineetha, Ravindranath [Manipal College of Dental Sciences, Manipal (India)

    2011-03-15

    Facial asymmetry is not an uncommon occurrence in day to day dental practice. It can be caused by various etiologic factors ranging from facial trauma to serious hereditary conditions. Here, we report a rare case of non-syndromic facial asymmetry in a young female, who was born with this condition but was not aware of the progression of asymmetry. No relevant family history was recognized. She was also deficient in both deciduous and permanent teeth in the corresponding region of maxilla. Hence, the cause of this asymmetry was believed to be a segmental odontomaxillary hypoplasia of left maxilla accompanied by agenesis of left maxillary premolars and molars and disuse atrophy of corresponding facial musculature. This report briefly discussed the comparative features of segmental odontomaxillary hypoplasia, hemimaxillofacial dysplasia, and segmental odontomaxillary dysplasia and justified the differences between segmental odontomaxillary hypoplasia and the other two conditions.

  19. Persistent pulmonary hypertension of the newborn

    OpenAIRE

    Sharma, Vinay; Berkelhamer, Sara; Lakshminrusimha, Satyan

    2015-01-01

    Persistent pulmonary hypertension of the newborn (PPHN) is characterized by elevated pulmonary vascular resistance resulting in right-to-left shunting of blood and hypoxemia. PPHN is often secondary to parenchymal lung disease (such as meconium aspiration syndrome, pneumonia or respiratory distress syndrome) or lung hypoplasia (with congenital diaphragmatic hernia or oligohydramnios) but can also be idiopathic. The diagnosis of PPHN is based on clinical evidence of labile hypoxemia often asso...

  20. Unilateral Glenoid Hypoplasia: A Case Report and Review of the Literature

    OpenAIRE

    Ashish Suryawanshi; Amber Mittal; Snehal Dongre; Neeti Kashyap

    2012-01-01

    Glenoid hypoplasia is a relatively rare alteration that in most cases involves the pectoral girdle in a bilateral and symmetrical manner. In general, glenoid hypoplasia is associated with skeletal changes such as hypoplasia of the humeral head or changes in the morphology of the acromion and of the coracoid. We describe a rare case of unilateral glenoid hypoplasia without instability and not involving humeral head. The patient was managed effectively with nonoperative measures that featured s...

  1. Two novel prenatal treatment strategies for experimental left ventricular hypoplasia.

    Czech Academy of Sciences Publication Activity Database

    Sedmera, David; Dealmeida, A.; McQuinn, T. C.

    Vienna: ESC, 2007, s. 103-104. [Annual Congress of the European Society of Cardiology 2007. Vienna (AT), 01.09.2007-05.09.2007] Institutional research plan: CEZ:AV0Z50450515 Keywords : hypoplasia Subject RIV: FA - Cardiovascular Diseases incl. Cardiotharic Surgery

  2. Implantable Ultralow Pulmonary Pressure Monitoring System for Fetal Surgery

    OpenAIRE

    Etemadi, Mozziyar; Heller, J. Alex; Schecter, Samuel C.; Shue, Eveline H; Miniati, Doug; Roy, Shuvo

    2012-01-01

    Congenital pulmonary hypoplasia is a devastating condition affecting fetal and newborn pulmonary physiology, resulting in great morbidity and mortality. The fetal lung develops in a fluid-filled environment. In this paper, we describe a novel, implantable pressure sensing and recording device which we use to study the pressures present in the fetal pulmonary tree throughout gestation. The system achieves 0.18 cm H2O resolution and can record for 21 days continuously at 256 Hz. Sample tracings...

  3. Palatoglossal fusion with cleft palate and hypoplasia of cerebellar vermis

    Directory of Open Access Journals (Sweden)

    Shailesh Solanki

    2016-01-01

    Full Text Available A new-born male presented within 12 h of birth with respiratory distress. On examination and workup, he had palatoglossal fusion, cleft palate and hypoplasia of the cerebellar vermis. A 2.5 Fr endotracheal tube was inserted into the pharynx through nostril as a nasopharyngeal stent, following which his respiratory distress improved. Once child was optimised, then feeding was started by nasogastric tube and feeds were tolerated well. Elective tracheostomy and gastrostomy were done, followed by release of adhesions between the tongue and palate at a later stage. Review of literature suggests that palatoglossal fusion is uncommon and presents as an emergency. Mostly, these oral synechiae are associated with digital and/or cardiac anomaly. Other disorders associated with intra-oral synechiae include congenital alveolar synechiae, van der Woude syndrome, popliteal pterygium syndrome and oromandibular limb hypogenesis syndrome. The authors report a hitherto undescribed association of palatoglossal fusion with cleft palate and hypoplasia of the cerebellar vermis.

  4. Lung hypoplasia and patellar agenesis in Ehlers-Danlos syndrome.

    Science.gov (United States)

    Pradhan, P; Deb, J; Deb, R; Chakrabarti, S

    2009-12-01

    A 22-year-old male patient was admitted with severe cough associated with purulent expectoration, left-sided chest pain and breathlessness. There was a history of recurrent respiratory ailments since childhood. The patient appeared younger than his chronological age. His face and ears were both dysmorphic. Clinically, the patient was diagnosed with Ehlers-Danlos syndrome (EDS). Computed tomography of the thoracic region revealed hypoplasia of the left lung and hyperplasia of the right lung. Both the patellae were absent. However, ultrasonography of his abdomen, echocardiography and other routine blood and urine examination showed no gross abnormalities. Although other respiratory tract abnormalities with EDS are not uncommon, unilateral lung hypoplasia and patellar agenesis in EDS make this case unique. PMID:20087544

  5. Optical coherence tomographic findings in optic nerve hypoplasia

    Directory of Open Access Journals (Sweden)

    Daruchi Moon

    2013-01-01

    Full Text Available We investigated a case of unilateral optic nerve hypoplasia using spectral domain optical coherence tomography (SDOCT. Optical coherence tomography was done on both eyes using 5-line Raster scan for the fovea to analyze the retinal nerve fiber layer thickness, inner retinal layer thickness, outer retinal layer thickness, and optic disc cube scan for the disc. Retinal nerve fiber layer thickness, inner retinal layer thickness, and outer retinal layer thickness were manually measured at 21-points of each five lines, and results were compared between both eyes. Retinal nerve fiber layer thickness and inner retinal layer thickness of optic nerve hypoplasia were significantly thinner than the opposite eye, but there was no significant difference in the thickness of the outer retinal layer between both eyes.

  6. Multiple myeloma presenting with coexisting severe marrow hypoplasia.

    Science.gov (United States)

    Medhi, K; Kalita, Dipti; Chopra, Anita; Anand, Mona; Raina, Vinod; Kumar, Rajive

    2008-01-01

    A 68-year-old man was referred to us with clinical and bone marrow (BM) features compatible with aplastic anemia. The correct diagnosis, hypoplasia of the BM coexisting with multiple myeloma, became apparent after noting rouleaux in the peripheral blood (PB) and approximately 50% plasma cells in the touch imprint of one of the two BM biopsies done. As standard therapy was precluded, the patient was put on dexamethasone but died within 4 days. This first case of the coexistence of untreated myeloma with aplastic BM shows that even apparently straightforward hypoplasia seen on the BM biopsy should be interpreted in conjunction with the PB smear and the BM touch imprint findings. Among other things, the BM biopsy and imprint should be repeated if the PB has findings such as rouleaux that do not fit with straightforward aplastic anemia. The combination of myeloma and BM aplasia precludes standard therapy and is rapidly fatal. PMID:19008591

  7. Multiple myeloma presenting with coexisting severe marrow hypoplasia

    Directory of Open Access Journals (Sweden)

    Medhi K

    2008-10-01

    Full Text Available A 68-year-old man was referred to us with clinical and bone marrow (BM features compatible with aplastic anemia. The correct diagnosis, hypoplasia of the BM coexisting with multiple myeloma, became apparent after noting rouleaux in the peripheral blood (PB and approximately 50% plasma cells in the touch imprint of one of the two BM biopsies done. As standard therapy was precluded, the patient was put on dexamethasone but died within 4 days. This first case of the coexistence of untreated myeloma with aplastic BM shows that even apparently straightforward hypoplasia seen on the BM biopsy should be interpreted in conjunction with the PB smear and the BM touch imprint findings. Among other things, the BM biopsy and imprint should be repeated if the PB has findings such as rouleaux that do not fit with straightforward aplastic anemia. The combination of myeloma and BM aplasia precludes standard therapy and is rapidly fatal.

  8. Oral rehabilitation for a patient with oligodontia and maxillary hypoplasia

    OpenAIRE

    Chung, Da-Woon; Vang, Mong-Sook; Park, Sang-Won; Lim, Hyun-Pil; Yang, Hong-So

    2009-01-01

    An 18 year old female with oligodontia and maxillary hypoplasia was treated using an interdisciplinary team approach involving orthodontists, maxillofacial surgeons and prosthodontists. Full mouth one-piece fixed partial dentures were the final restoration. The fixed partial dentures fabricated for the maxilla and mandible using the concept of a shortened dental arch resulted in improved esthetics and the masticatory function. This paper describes the treatment procedures for an oligodontia p...

  9. Aesthetic approach for anterior teeth with enamel hypoplasia

    OpenAIRE

    Josué Martos; Andréa Gewehr; Emanuele Paim

    2012-01-01

    Enamel hypoplasia is a developmental defect of the enamel that is produced by a disturbance in the formation of the organic enamel matrix, clinically visible as enamel defects. Disorders that occur during the stages of enamel development and maturation reduce the amount or thickness of the enamel, resulting in white spots, tiny grooves, depressions and fissures in the enamel surface. The complexity and intensity of the dental deformity lesions will conduct the ideal treatment-associating cons...

  10. Optic nerve hypoplasia and astigmatism: a new association.

    OpenAIRE

    Zeki, S. M.

    1990-01-01

    Thirty-one patients with optic nerve hypoplasia (ONH) or septo-optic dysplasia and two patients with segmental ONH underwent retinoscopy. The results were compared with those of 20 normal subjects. There was a higher prevalence of astigmatism in the patients than in the controls. Two patients with segmental ONH had no evidence of astigmatism. The association of astigmatism with ONH has not hitherto been reported. The close association of ONH with astigmatism highlights the importance of perfo...

  11. Lissencephaly-pachygyria and cerebellar hypoplasia in a calf

    OpenAIRE

    Bianca Lemos dos Santos; Maria Cecília Florisbal Damé; Ana Carolina Barreto Coelho; Plínio Aguiar de Oliveira; Clairton Marcolongo-Pereira; Ana Lucia Schild

    2016-01-01

    ABSTRACT: A case of lissencephaly-pachygyria and cerebellar hypoplasia diagnosed in a Charolais x Tabapuã calf is described. The calf presented since birth, clinical signs characterized by apathy, prolonged recumbency, tremors of the head and neck, ataxia, hypermetria, difficulty walking, blindness and swelling of the joints of the four limbs. Due to the unfavorable prognosis, the animal was euthanized and necropsied at 34 days of age. At necropsy, a rudimentary development of the brain folds...

  12. Multiple myeloma presenting with coexisting severe marrow hypoplasia

    OpenAIRE

    Medhi K; Kalita Dipti; Chopra Anita; Anand Mona; Raina Vinod; Kumar Rajive

    2008-01-01

    A 68-year-old man was referred to us with clinical and bone marrow (BM) features compatible with aplastic anemia. The correct diagnosis, hypoplasia of the BM coexisting with multiple myeloma, became apparent after noting rouleaux in the peripheral blood (PB) and approximately 50% plasma cells in the touch imprint of one of the two BM biopsies done. As standard therapy was precluded, the patient was put on dexamethasone but died within 4 days. This first case of the coexistence of untre...

  13. Classification, diagnosis and potential mechanisms in Pontocerebellar Hypoplasia

    Directory of Open Access Journals (Sweden)

    Poll-The Bwee

    2011-07-01

    Full Text Available Abstract Pontocerebellar Hypoplasia (PCH is group of very rare, inherited progressive neurodegenerative disorders with prenatal onset. Up to now seven different subtypes have been reported (PCH1-7. The incidence of each subtype is unknown. All subtypes share common characteristics, including hypoplasia/atrophy of cerebellum and pons, progressive microcephaly, and variable cerebral involvement. Patients have severe cognitive and motor handicaps and seizures are often reported. Treatment is only symptomatic and prognosis is poor, as most patients die during infancy or childhood. The genetic basis of different subtypes has been elucidated, which makes prenatal testing possible in families with mutations. Mutations in three tRNA splicing endonuclease subunit genes were found to be responsible for PCH2, PCH4 and PCH5. Mutations in the nuclear encoded mitochondrial arginyl- tRNA synthetase gene underlie PCH6. The tRNA splicing endonuclease, the mitochondrial arginyl- tRNA synthetase and the vaccinia related kinase1 are mutated in the minority of PCH1 cases. These genes are involved in essential processes in protein synthesis in general and tRNA processing in particular. In this review we describe the neuroradiological, neuropathological, clinical and genetic features of the different PCH subtypes and we report on in vitro and in vivo studies on the tRNA splicing endonuclease and mitochondrial arginyl-tRNA synthetase and discuss their relation to pontocerebellar hypoplasia.

  14. Pulmonary damage in childhood: Evaluation with pulmonary scintigraphy

    International Nuclear Information System (INIS)

    Introduction: There are a number of pulmonary diseases during infancy that predispose to chronic respiratory conditions at the adults age. Bronchiectasis, malformations, bronchogenic cysts, atelectasis and sequels of adenovirus infection are frequent factors for recurrent respiratory diseases, that can produce a permanent pulmonary damage. The pulmonary radioisotopic studies allow a reliable pre-operatory evaluation in order to determinate the global and segmental pulmonary function. Chronic hypoventilated territories shown secondary hypoperfusion, giving as a result a match defect in the ventilation/perfusion scintigraphy. Goal: To determinate if the perfusion scintigram alone is enough in order to evaluate the pulmonary function in pediatric population with chronic pulmonary diseases. Materials and Methods: All ventilation/perfusion scintigraphic studies done in children with chronic pulmonary diseases derived to our center between 1999-2001 were analyzed. A total of 58 children (25 boys and 33 girls, in a age range from 1 month to 17 years) were studied. The most frequent indications the study were: bronchiectasis, pulmonary hypoplasia, several bronchitis and sequels of adenovirus respiratory infection. This last one represents the 31% of all cases. The number and location of each perfusion and ventilation defects where correlated. Results: Twelve out of the 58 children had normal lung scintigraphics studies (21%), 44 showed mixed defects (76%), in 1 child only one perfusion defect was found (1.5%) and in other child only a single ventilation alteration was seen (1.5%). Conclusion: According to the results in our group, we observed that in chronic pulmonary diseases most cases have matched ventilation/perfusion defects, because of readecuation of the pulmonary blood flow. The perfusions scintigraph was diagnosed in 98.5% of the studied cases. This allows us to suggests that in the evaluation of pulmonary damage in children with chronic pulmonary diseases, the

  15. Unilateral Glenoid Hypoplasia: A Case Report and Review of the Literature

    Directory of Open Access Journals (Sweden)

    Ashish Suryawanshi

    2011-01-01

    Full Text Available Glenoid hypoplasia is a relatively rare alteration that in most cases involves the pectoral girdle in a bilateral and symmetrical manner. In general, glenoid hypoplasia is associated with skeletal changes such as hypoplasia of the humeral head or changes in the morphology of the acromion and of the coracoid. We describe a rare case of unilateral glenoid hypoplasia without instability and not involving humeral head. The patient was managed effectively with nonoperative measures that featured specific rehabilitation exercises for the shoulder.

  16. Unusual Anatomic Variations Associated With Bilateral Ulnar Artery Hypoplasia.

    Science.gov (United States)

    Ro, Hyung-Suk; Roh, Si-Gyun; Shin, Jin Yong; Lee, Nae-Ho; Yang, Kyung-Moo

    2016-05-01

    Variations and anomalies of upper extremities have been commonly reported in routine dissection, clinical practices, and cadaver studies. Despite ongoing research on arterial variations of upper extremities, the absence of bilateral ulnar artery is extremely rare with only 3 patients reported. As the authors are presenting a successfully treated patient, initially prepped for radial forearm osteocutaneous free flap for treatment on oromandibular defect after a wide resection of head and neck cancer lesion, being confirmed to have bilateral ulnar artery hypoplasia and due to this, the patient had to change her surgical plan to fibular osteocutaneous free flap. PMID:27100648

  17. Hypoplasia of the thumb. Clinical presentation and reconstruction

    International Nuclear Information System (INIS)

    In the genesis of the partial or total absence of the thumb they are genetic, environmental factors or a combination of both. It is take part of a syndrome or to be isolated and frequently associated with problems of radial longitudinal deficiency of the forearm. Objective. The purpose of this study is shown the experience, the focus of the processing and the results obtained since the point esthetic and functional view. The most it accepted classification is the proposal by Blauth that helps to determine the forecast and the processing. Materials and methods. it is a work type series of cases in 22 children with hypoplasia of the thumb, with a minimum of 12 months, (average 28 months). In 15 cases there were association of radial dysplasia or another anomaly among them 4 patients with VATER, and the 7 remaining they corresponded to hypoplasia of the thumb as only entity. We carried out tendon transfer, with opening of the first comisure in 2 patients with hypoplasia type II. In 3 patients, with hypoplasia type III A, one carries out corner opening, transfer of the superficial flexor of the 4 finger to correct instability of the articulation MF and opposition of the thumb, and transfer for extension of the thumb. In 17 cases one carries out politicization of the index. Results. The outcome was evaluated in: non pinch, lateral pinch and fingertip pinch; the grade of opposition like good, minimal and non opposition, and the aesthetic result according to the satisfaction of the parents in bad, regular and good. The five children reconstructed with transfers of tendons and comisure opening had good result. In 17 children with politicization one patient had a necrosis, of the 16 remaining a good or acceptable result was obtained. Discussion. It is not easy to follow a good system of measure of the functional results. We find that a practical way to evaluate was the clip, opposition and aesthetics. Previous to the surgeries it is required to evaluate alterations

  18. Optic nerve hypoplasia in fetal alcohol syndrome: an update.

    Science.gov (United States)

    Pinazo-Duran, M D; Renau-Piqueras, J; Guerri, C; Strömland, K

    1997-01-01

    Optic nerve hypoplasia was detected in up to one half of a group of Swedish children born to alcoholic mothers. Using an experimental model of pre- and postnatal alcohol exposure in rats fed a liquid diet, reduced optic nerve size from gestational day 21 (294 +/- 26 x 10(2) microns2 vs 502 +/- 16 x 10(2) microns2; n = 6; p alcohol levels achieved in dams and their offspring. Altered glial cells and degenerating and atrophic optic axons, myelin sheaths and ganglion cells were frequent in the alcohol-exposed optic nerves. Smaller optic nerve (1.918 +/- 61 x 10(2) microns2 vs 2.195 +/- 40 x 10(2) microns2; n = 4; p alcohol exposure. In summary, alcohol as a major teratogenic agent may induce dysmorphogenesis and irremediable damage to the retina and optic nerve, which frequently manifests itself as hypoplastic optic nerve. PMID:9352281

  19. Rare combination of congenital heart disease and pulmonary alveolar proteinosis.

    Science.gov (United States)

    Tanaka, Yuki; Miyamoto, Takashi; Yoshitake, Shuichi; Naito, Yuji; Kobayashi, Tomio

    2015-10-01

    Here, we describe a case of total anomalous pulmonary venous return with coarctation of the aorta that was diagnosed as pulmonary alveolar proteinosis at autopsy in a male infant. Surgical repair was performed at 1 day of age, but the infant died on postoperative day 51 due to respiratory insufficiency without any evidence of pulmonary venous obstruction. He had been unexpectedly diagnosed with pulmonary alveolar proteinosis and pulmonary hypoplasia on autopsy. Congenital pulmonary alveolar proteinosis is a serious condition with a high mortality rate, which should be considered in the differential diagnosis in patients with a clinical picture of pulmonary venous obstruction, because most patients are unable to survive without proper treatment. In this report, we address specific issues that should be discussed in such cases based on our recent experience. PMID:26310609

  20. Radiological features of Goltz syndrome: Focal dermal hypoplasia. A report of two cases

    Energy Technology Data Exchange (ETDEWEB)

    Boothyrod, A.E.; Hall, C.M.

    1988-10-01

    Two female infants with Goltz syndrome (focal dermal hypoplasia) were recently investigated for severe feeding problems and failure to thrive. Both demonstrated severe skeletal malformations and marked gastrooesophageal reflux with laxity of the hiatus. One child (case 1) exhibited nasal regurgitation during feeding. Interestingly, both children had undergone surgery; Case 1 or a right parasagittal abdominal hernia associated with focal dermal hypoplasia of the abdominal wall and Case 2 for an exomphalos also associated with dermal hypoplasia. This observation suggests more widespread mesodermal abnormality. (orig./GDG).

  1. Angiographic study of systemic-pulmonary collateral in pulmonary atresia with ventricular septal defect

    International Nuclear Information System (INIS)

    Objective: To evaluate the angiography in demonstrating the development of systemic-pulmonary collateral vessels and pulmonary arteries, and its clinical significance in pulmonary atresia with ventricular septal defect (PA + VSD). Methods: The findings of angiography of the right ventricle and aorta arch and upper portion of the descending thoracic aorta in 98 cases with PA + VSD were retrospectively studied including 63 additional selective angiography of collateral vessels. The types of systemic-pulmonary collateral vessels and the development of left and right native pulmonary arteries with or without fusion were emphatically analyzed, etc. Results: Among those 98 cases, the pulmonary arteries were supplied by patent ductus arteriosus in 14(14%), 11 of them were infants and young children; and by direct aortopulmonary collaterals in 43(44%); by multiple origin of systemic-pulmonary artery collaterals in 41(42%). 60 of 98 cases were visualized the presence of left and right native pulmonary arteries through collateral vessels. Among them, the hypoplasia of left and/or right pulmonary arteries was found. Conclusion: Angiography of right ventricle and aorta combined with necessary selective angiography of collateral vessels was still an indispensable method in the demonstration of systemic-pulmonary collateral vessels and development of pulmonary arteries, and provided the main basis for the selection of indications for interventional/surgical therapy

  2. Implantable Ultralow Pulmonary Pressure Monitoring System for Fetal Surgery

    Science.gov (United States)

    Etemadi, Mozziyar; Heller, J. Alex; Schecter, Samuel C.; Shue, Eveline H.; Miniati, Doug; Roy, Shuvo

    2015-01-01

    Congenital pulmonary hypoplasia is a devastating condition affecting fetal and newborn pulmonary physiology, resulting in great morbidity and mortality. The fetal lung develops in a fluid-filled environment. In this paper, we describe a novel, implantable pressure sensing and recording device which we use to study the pressures present in the fetal pulmonary tree throughout gestation. The system achieves 0.18 cm H2O resolution and can record for 21 days continuously at 256 Hz. Sample tracings of in vivo fetal lamb recordings are shown. PMID:22801521

  3. Pulmonary edema

    Science.gov (United States)

    ... congestion; Lung water; Pulmonary congestion; Heart failure - pulmonary edema ... Pulmonary edema is often caused by congestive heart failure . When the heart is not able to pump efficiently, blood ...

  4. Hypoplasia of the odontoid with atlanto-axial subluxation in Hurler's syndrome

    International Nuclear Information System (INIS)

    There appears to be an increased incidence of hypoplasia of the odontoid in Hurler's syndrome. As this predisposes to atlanto-axial subluxation, it should be sought in this mucopolysaccharidosis, as well as in Morquio's syndrome. (orig.)

  5. Hypoplasia of bone marrow and cytopenia in non-hemic diseases

    International Nuclear Information System (INIS)

    Common (stereotype) and different links in pathogenesis of hemodepressions pronounced in the form of cytopenia and/or hypoplasia of hemopoiesis are considered. Pathogenesis and signs of the diseases, as well as their diagnosis, are studied in detail

  6. Genetics Home Reference: intrauterine growth restriction, metaphyseal dysplasia, adrenal hypoplasia congenita, and ...

    Science.gov (United States)

    ... abnormal side-to-side curvature of the spine ( scoliosis ) or thinning of the bones ( osteoporosis ). Adrenal hypoplasia ... health conditions: Diagnostic Tests Drug Therapy Surgery and Rehabilitation Genetic Counseling Palliative Care Related Information How are ...

  7. Circular Enamel Hypoplasia: A Rare Enamel Developmental Disturbance in Permanent Teeth

    OpenAIRE

    Sandhu, Meera; Gulia, Shweta; Nagpal, Mehak; Sachdev, Vinod

    2014-01-01

    Circular enamel hypoplasia, a type of enamel hypoplasia is an extensive enamel disturbance that results in a demarcating line surrounding the crown of the injured teeth visible both clinically and radiographically that most frequently occurs as a result of trauma in children around the age of two years. Clinical features include poor aesthetics, dentin sensitivity, increased susceptibility to dental caries and malocclusion. Early radiographic diagnosis of such teeth is important for timely in...

  8. Association between Enamel Hypoplasia and Dental Caries in Primary Second Molars: A Cohort Study

    OpenAIRE

    Hong, L.; Levy, S.M.; Warren, J.J.; Broffitt, B.

    2009-01-01

    The purpose of this study was to assess the longitudinal relationships between enamel hypoplasia and caries experience of primary second molars. The study sample was 491 subjects who received dental examinations at both age 5 and 9 by the calibrated examiners. Four primary second molars (n = 1,892) were scored for the presence of enamel hypoplasia for each participant. Caries presence and number of decayed and filled surfaces (dfs) were determined at age 5 and 9. The relationships between ena...

  9. Pulmonary growth and development after repair of congenital diaphragmatic hernia

    International Nuclear Information System (INIS)

    Congenital diaphragmatic hernia (CDH) is generally associated with pulmonary hypoplasia that determines survival. To evaluate the degree of this hypoplasia, we examined pulmonary growth and development in 26 clinical cases who had undergone surgical repair of CDH, using chest X-rays and pulmonary perfusion scintigrams. Seven cases developed their symptoms more than 24 h after birth (Group I) and 19 cases within 24 h after birth (Group II), which included 11 cases with persistent fetal circulation (PFC). The affected lung in group I expanded immediately after surgery as showed by X-rays. On the other hand, one-eight weeks were required for expansion of the affected lung in group II. The perfusion to the hernia side was normal in all cases of group I, but reduced in 15 of 19 cases of group II. Especially, the perfusion was significantly decreased in cases with PFC. These results indicate a slow lung growth on chest X-rays and hypoperfusion in the affected side in most of group II, suggesting a vascular pulmonary hypoplasia. (author)

  10. Enamel hypoplasia and its role in identification of individuals: A review of literature.

    Science.gov (United States)

    Kanchan, Tanuj; Machado, Meghna; Rao, Ashwin; Krishan, Kewal; Garg, Arun K

    2015-01-01

    Identification of individuals is the mainstay of any forensic investigation especially in cases of mass disasters when mutilated remains are brought for examination. Dental examination helps in establishing the identity of an individual and thus, has played a vital role in forensic investigation process since long. In this regard, description on the role of enamel hypoplasia is limited in the literature. The present article reviews the literature on the enamel hypoplasia and discusses its utility in forensic identification. Enamel hypoplasia is a surface defect of the tooth crown caused by disturbance of enamel matrix secretion. Enamel defects can be congenital or acquired. In cases of mass disasters, or when the body is completely charred, putrefied and mutilated beyond recognition, the unique dental features can help in identification of the victims. PMID:26097340

  11. X-linked congenital adrenal hypoplasia associated with hypospadias in an Egyptian baby: a case report

    Directory of Open Access Journals (Sweden)

    Metwalley Kotb

    2012-12-01

    Full Text Available Abstract Introduction X-linked congenital adrenal hypoplasia is a rare developmental disorder of the human adrenal cortex and is caused by deletion or mutation of the dosage-sensitive sex reversal adrenal hypoplasia congenita critical region of the X chromosome, gene 1 (DAX-1 gene. Most affected children present with failure to thrive, salt wasting and hypoglycemic convulsions in the first months of life. Hypospadias affects approximately one in 250 live male births. Mutations in the mastermind-like domain-containing 1 (MAMLD1 gene have been implicated as one of the causes of hypospadias in children. To the best of our knowledge, an association between congenital adrenal hypoplasia due to a DAX-1 mutation and hypospadias due to mutation of the MAMLD1 gene has not previously been reported in the literature. Case presentation A 35-day-old male Egyptian baby was referred to our institution for the evaluation of a two-week history of recurrent vomiting associated with electrolyte imbalance. On examination, our patient was found to have hypotension and dehydration. A genital examination showed distal penile hypospadias with chordee and normal testes. He had hyponatremia, hyperkalemia, hypoglycemia and metabolic acidosis. Endocrinological investigations revealed low levels of cortisol, 17-hydroxyprogesterone and aldosterone, with a high level of adrenocorticotrophic hormone. A provisional diagnosis of congenital adrenal hypoplasia associated with hypospadias was made. A molecular genetics study confirmed the diagnosis of X-linked congenital adrenal hypoplasia due to DAX-1 mutations and hypospadias due to MAMLD1 mutation. He was started on hydrocortisone and fludrocortisone treatment. After three weeks of treatment, his symptoms improved and his blood sugar, sodium, potassium and cortisol levels normalized. Conclusions We report the case of an Egyptian baby with an association of congenital adrenal hypoplasia due to DAX-1 mutation and hypospadias due

  12. The helical three-dimensional CT in the diagnosis of torticollis with occipitocondylar hypoplasia

    International Nuclear Information System (INIS)

    Congenital anomalies of the atlanto-occipital and atlantoaxial joints are rare. Those most commonly reported are atlantoaxial instability, basilar impression, anomalies of the odontoid process, laxity of the transverse atlantal ligament and atlanto-occipital fusion. Occipital condylar hypoplasia is infrequent and difficult to recognise. We recently diagnosed it using helical 3D CT in association with torticollis in two patients. The first patient had a several year history of torticollis. The second patient had acute cervical lymphadenitis associated with post-operative torticollis. 3D CT distinctly revealed atlantoaxial subluxation with hypoplasia of the occipital condyles in both cases. (Copyright (c) 1998 Elsevier Science B.V., Amsterdam. All rights reserved.)

  13. The helical three-dimensional CT in the diagnosis of torticollis with occipitocondylar hypoplasia

    Energy Technology Data Exchange (ETDEWEB)

    Ilkko, E.; Tikkakoski, T.; Pyhtinen, J. [Department of Radiology, Oulu University Hospital, Kajaanintie 50, 90220 Oulu (Finland)

    1998-11-01

    Congenital anomalies of the atlanto-occipital and atlantoaxial joints are rare. Those most commonly reported are atlantoaxial instability, basilar impression, anomalies of the odontoid process, laxity of the transverse atlantal ligament and atlanto-occipital fusion. Occipital condylar hypoplasia is infrequent and difficult to recognise. We recently diagnosed it using helical 3D CT in association with torticollis in two patients. The first patient had a several year history of torticollis. The second patient had acute cervical lymphadenitis associated with post-operative torticollis. 3D CT distinctly revealed atlantoaxial subluxation with hypoplasia of the occipital condyles in both cases. (Copyright (c) 1998 Elsevier Science B.V., Amsterdam. All rights reserved.)

  14. Turner's hypoplasia and non-vitality: A case report of sequelae in permanent tooth

    OpenAIRE

    2010-01-01

    Hypoplasia is the result of disruption in the process of enamel matrix formation, which in turn causes defect in quality and thickness of enamel. Four cases of Turner′s hypoplastic teeth with a previous history of trauma/infection in their primary predecessors at the age of 2-3 years have been reported. These hypoplastic teeth had turned non-vital without any carious insult, cavitation or further trauma. This article thereby stresses the importance of early detection of enamel hypoplasia and ...

  15. Diagnosis of abdominal aortic hypoplasia by state-of-the-art MR angiography

    International Nuclear Information System (INIS)

    Abdominal aortic hypoplasia is a rare vascular variant with possible major clinical sequelae when the renal arteries are also involved. The condition is thought to result from embryonic overfusion of the two dorsal aortas. The diagnosis may be considered in patients presenting with hypertension in the neonatal period and severely reduced or absent arterial pulses in the groin. We present contrast-enhanced state-of-the-art magnetic resonance angiography imaging findings in an 8-year-old boy with abdominal aortic hypoplasia. (orig.)

  16. Management of pulmonary hypertension in infants with congenital diaphragmatic hernia.

    Science.gov (United States)

    Gien, J; Kinsella, J P

    2016-06-01

    In infants with congenital diaphragmatic hernia (CDH), a posterolateral diaphragmatic defect results in herniation of abdominal contents into the chest and compression of the intrathoracic structures. In the most severe cases, hypoplasia of the ipsilateral and contralateral lungs, severe pulmonary hypertension (PH) and left ventricular (LV) hypoplasia/dysfunction all contribute to increased mortality. The management of PH in CDH is complicated by structural and functional changes in the heart, pulmonary vasculature, airways and lung parenchyma; consequently, determining optimal management strategies is challenging. Treatment of PH in patients with CDH changes as the underlying pathophysiology evolves in the days and weeks after birth. During the early transition, the use of pulmonary vasodilators is limited by LV structural and functional abnormalities, and pulmonary vasodilators such as inhaled nitric oxide (iNO) may have a limited role (for example, stabilization for extracorporeal membrane oxygenation (ECMO), treatment of marked preductal desaturation and treatment of PH as LV performance improves). In contrast, subacute treatment of PH in CDH with iNO has an important role in recurrent or persistent PH and potentially improves survival. Chronic PH and vascular abnormalities may persist into childhood in patients with CDH, contributing to late mortality. It is unclear how pulmonary vasodilator therapies, such as iNO, sildenafil and bosentan, will modulate late outcomes in CDH with late/chronic PH. PMID:27225962

  17. Cystic lung changes in a thin section CT in an asymptomatic young adult with unilateral pulmonary vein atresia: A case report

    Energy Technology Data Exchange (ETDEWEB)

    Lee, Seung Choul; Yi, Jeong Geun; Park, Jeong Hee [Konkuk Univ. Medical Center, Seoul (Korea, Republic of)

    2012-07-15

    Unilateral pulmonary vein atresia is a rare anomaly, usually associated with symptoms of recurrent hemoptysis and pneumonia in early childhood. Only one report of an asymptomatic adult patient can be found in the literature. We present the case of an asymptomatic patient with unilateral right pulmonary vein atresia in a 20 year old man. Chest radiograph and multidetector computed tomography showed not only pulmonary vein atresia, pulmonary artery hypoplasia, but also cystic lung changes on thin section CT, along with septal and bronchovascular bundle thickening, and ground-glass opacity. Unilateral pulmonary vein atresia could be another disease which can show cystic lung changes on thin section chest CT.

  18. Cystic lung changes in a thin section CT in an asymptomatic young adult with unilateral pulmonary vein atresia: A case report

    International Nuclear Information System (INIS)

    Unilateral pulmonary vein atresia is a rare anomaly, usually associated with symptoms of recurrent hemoptysis and pneumonia in early childhood. Only one report of an asymptomatic adult patient can be found in the literature. We present the case of an asymptomatic patient with unilateral right pulmonary vein atresia in a 20 year old man. Chest radiograph and multidetector computed tomography showed not only pulmonary vein atresia, pulmonary artery hypoplasia, but also cystic lung changes on thin section CT, along with septal and bronchovascular bundle thickening, and ground-glass opacity. Unilateral pulmonary vein atresia could be another disease which can show cystic lung changes on thin section chest CT

  19. Breast hypoplasia following irradiation of the female breast in infancy and early childhood

    International Nuclear Information System (INIS)

    The occurrence and the degree of breast hypoplasia were studied in 129 women irradiated with ionizing radiation in infancy or childhood for hemangioma located in the breast region. The patients were born between 1934 and 1943 and were treated at Radiumhemmet before 4 years of age. Dosimetry was based on treatment data. The mean absorbed dose to the breast anlage was 2.3 Gy. Breast asymmetry was estimated by a mailed questionnaire to all patients and by a clinical examination of 53 patients living in Stockholm Country. Breast hypoplasia on the treated side was reported by 57% of the patients and on the contralateral side by 8%. A breast hypoplasia exceeding 10% was found in 53% of the clinically examined patients on the treated side and in 9% on the contralateral side. The frequency and the severity of impaired breast development increased with the absorbed dose. The possibility of a threshold dose for the occurrence of breast hypoplasia could neither be established nor ruled out. (orig.)

  20. Concurrent occipital hypoplasia, occipital dysplasia, syringohydromyelia, and hydrocephalus in a Yorkshire terrier

    OpenAIRE

    Cagle, Laura

    2010-01-01

    Magnetic resonance imaging of a 7.5-year-old neutered male Yorkshire terrier with mild generalized ataxia and intermittent neck scratching led to a diagnosis of caudal occipital malformation and syringohydromyelia. Surgical exploration led to a diagnosis of occipital dysplasia with concurrent occipital hypoplasia. Following a dorsal laminectomy of the first cervical vertebra there was no progression or improvement a month later.

  1. Prenatal evaluation of the middle ear and diagnosis of middle ear hypoplasia using MRI

    International Nuclear Information System (INIS)

    Analysis of the middle ear with fetal MRI has not been previously reported. To show the contribution of fetal MRI to middle ear imaging. The tympanic cavity was evaluated in 108 fetal cerebral MRI examinations (facial and/or cerebral malformation excluded) and in two cases, one of Treacher Collins syndrome (case 1) and the other of oculo-auriculo-vertebral (OUV) spectrum (case 2) with middle ear hypoplasia identified by MRI at 27 and 36 weeks' gestation, respectively. In all 108 fetuses (mean gestational age 32.5 weeks), the tympanic cavity and T2 hypointensity related to the ossicles were well visualised on both sides. Case 1 had micro/retrognathia and bilateral external ear deformity and case 2 had retrognathism with a left low-set and deformed ear. MRI made it possible to recognize the marked hypoplasia of the tympanic cavity, which was bilateral in case 1 and unilateral in case 2. Both syndromes are characterized by craniofacial abnormalities including middle ear hypoplasia, which cannot be diagnosed with US. The middle ear cavity can be visualized with fetal MRI. We emphasize the use of this imaging modality in the diagnosis of middle ear hypoplasia. (orig.)

  2. Bilateral mandibular distraction in micrognathism or hypoplasia of mandible, hazrat fatemeh hospital

    Directory of Open Access Journals (Sweden)

    Mohammad-Esmaiil Hassani

    2014-01-01

    Conclusion: The Bilateral Distraction Oseogenesis method can bring forwards the retruded mandible effectively and its results has no difference with older methods. It is an effective and reliable procedure for hypoplasia of Mandible and the results are comparable with older methods.

  3. Implantable ultra-low pulmonary pressure monitoring system for fetal surgery.

    Science.gov (United States)

    Etemadi, Mozziyar; Heller, J Alex; Schecter, Samuel C; Shue, Eveline H; Miniati, Doug; Roy, Shuvo

    2012-11-01

    Congenital pulmonary hypoplasia is a devastating condition affecting fetal and newborn pulmonary physiology, resulting in great morbidity and mortality. The fetal lung develops in a fluid-filled environment. In this work, we describe a novel, implantable pressure sensing and recording device which we use to study the pressures present in the fetal pulmonary tree throughout gestation. The system achieves 0.18 cm H2O resolution and can record for twenty one days continuously at 256 Hz. Sample tracings of in vivo fetal lamb recordings are shown. PMID:22801521

  4. Interstitial pulmonary emphysema in ventilated neonates

    International Nuclear Information System (INIS)

    106 (15.7%) of 675 artificially ventilated newborn developed interstitial pulmonary emphysema (PIE). Basic lung diseases were: IRDS, neonatal pneumonia, shock lung, meconium aspiration, hypoplasia of the lungs and other miscellaneous disorders of the chest. PIE developed in 68% of patients within 8 hours following artificial respiration. At the beginning of PIE both lungs were concerned in 41.5% of patients, one lobe of both lungs was affected in 32.1%. PIE was located in one lung in 8.5% and in only one lobe in 17.9%. Maximum of PIE was seen within 5 days after initiating respiration in 76.7% of the patients. Persistent PIE developed in 28.7% of the patients. Persistent PIE of both lungs was seen in 11 cases, PIE of one lung in 8 cases and persistent lobar emphysema in another 8. Pulmonary pseudocysts developed in 22 (20.8%) of the patients. (orig.)

  5. Pulmonary Rehabilitation

    Science.gov (United States)

    ... programs are certified by the American Association of Cardiovascular and Pulmonary Rehabilitation. These programs can be found on the AACVPR ... at 1-800-586-4872. American Association of Cardiovascular and Pulmonary Rehabilitation ... ...

  6. Pulmonary Fibrosis

    Science.gov (United States)

    Pulmonary fibrosis is a condition in which the tissue deep in your lungs becomes scarred over time. This tissue ... may not get enough oxygen. Causes of pulmonary fibrosis include environmental pollutants, some medicines, some connective tissue ...

  7. Pulmonary edema

    Science.gov (United States)

    Pulmonary edema is an abnormal buildup of fluid in the lungs. This buildup of fluid leads to shortness of ... Pulmonary edema is often caused by congestive heart failure . When the heart is not able to pump efficiently, blood ...

  8. Ischemic stroke in patient with existing congenital hypoplasia of the middle cerebral artery

    International Nuclear Information System (INIS)

    Presented is a clinical case of a woman 29 years old with ischemic stroke (IS), which has developed abruptly in existing congenital hypoplasia and occlusion of the middle cerebral artery. There are no other well or less well documented risk factors for cerebrovascular disease. In family history noted that the father of the patient died suddenly at the age of 45 years from stroke, also without evidence of vascular disease. On magnetic resonance imaging (MRI) of the brain is found high signal zone in the left nucleus lentiformis. We discussed the possibilities for implementing conventional angiography and eventually surgical procedures unfortunately rejected due to the high risk to the patient. Key words: Ischemic Stroke. Magnetic Resonance Imaging. Hypoplasia

  9. Congenital porencephaly with cerebellar hypoplasia in a Holstein calf: a case report

    International Nuclear Information System (INIS)

    We describe the case of a nine-day-old female Holstein calf which had cheiloschisis, a moderate dome-shaped head, ataxia and opisthotonus since birth. No significant findings except the dome-shaped head were observed on survey radiography of the skull. Computed tomography (CT) images showed bilateral lateral ventriculomegaly, cerebellar hypoplasia and a cyst-like lesion communicating with the right lateral ventricle. Post-mortem examination revealed a cerebral defect in the frontoparietal lobe, which communicated with the right lateral ventricle, and cerebellar hypoplasia. CT provided a characteristic finding of porencephaly and was helpful for diagnosing the accompanying anomalies. We suggest that porencephaly should be included as a specific anomaly in the differential diagnosis of congenital brain malformation

  10. Regional hypoplasia of somatosensory cortex in growth-retarded mice (grt/grt).

    Science.gov (United States)

    Sawada, Kazuhiko; Saito, Shigeyoshi; Sugasawa, Akari; Sato, Chika; Aoyama, Junya; Ohara, Naoko; Horiuchi-Hirose, Miwa; Kobayashi, Tetsuya

    2016-07-01

    Growth-retarded mouse (grt/grt) is a spontaneous mutant that is known as an animal model for primary congenital hypothyroidism caused by resistance to TSH signaling. The regional pattern of cerebral cortical hypoplasia was characterized in grt/grt mice. Ex vivo computed tomography (CT)-based volumetry was examined in four regions of the cerebral cortex, i.e., prefrontal, frontal, parietal and occipito-temporal regions, which were demarcated by structural landmarks on coronal CT images. A region-specific reduced volume of the parietal cortical region covering most of the somatosensory cortex was noted in grt/grt mice rather than in both heterozygous (grt/+) and wild-type (+/+) mice. We concluded that the cortical hypoplasia in grt/grt was seen in identical cortical regions corresponding to human congenital hypothyroidism. PMID:26915353

  11. The pathogenesis of renal dysplasia. I. Quantification of hypoplasia and dysplasia.

    Science.gov (United States)

    Schwarz, R D; Stephens, F D; Cussen, L J

    1981-09-01

    In order to assess the relative effects of abnormal ureteric orifice position and abnormal urodynamics on the morphogenesis of hypoplasia and dysplasia in kidneys obtained from infants, we devised a method of quantifying the renal structures. The method was based on radial glomerular counts which ranged from zero to normal (seven to nine), a score for dysplastic structures, and the ratio of normal to abnormal tissues present. These three values, when plotted against each other, correlated closely. The glomerular count, with occasional minor adjustment for inconsistencies, was the best parameter of hypodysplasia. Severe to moderate grades of hypodysplasias fell in the low and middle ranges and hypoplasia through to normal in the highest range. By grading kidneys in this way, we were able to compare the effects of ureteral ectopy and abnormal urinary dynamics on the developing kidney. PMID:7024184

  12. Subdural effusions and lack of early pontocerebellar hypoplasia in siblings with RARS2 mutations.

    Science.gov (United States)

    Kastrissianakis, Katherina; Anand, Geetha; Quaghebeur, Gerardine; Price, Sue; Prabhakar, Prab; Marinova, Jasmina; Brown, Garry; McShane, Tony

    2013-12-01

    Mutations in the recently described RARS2 gene encoding for mitochondrial arginyl-transfer RNA synthetase give rise to a disorder characterised by early onset seizures, progressive microcephaly and developmental delay. The disorder was named pontocerebellar hypoplasia type 6 (PCH6) based on the corresponding radiological findings observed in the original cases. We report two siblings with the RARS2 mutation who displayed typical clinical features of PCH6, but who had distinct neuroimaging features. Early scans showed marked supratentorial, rather than infratentorial, atrophy, and the pons remained preserved throughout. One sibling also had bilateral subdural effusions at presentation. The deceleration in head growth pointed to an evolving genetic/metabolic process giving rise to cerebral atrophy and secondary subdural effusions. RARS2 mutations should be considered in infants presenting with seizures, subdural effusions, decelerating head growth and evidence of cerebral atrophy even in the absence of pontocerebellar hypoplasia on imaging. PMID:24047924

  13. Pulmonary rehabilitation

    OpenAIRE

    Spruit, Martijn A.

    2001-01-01

    Pulmonary rehabilitation is a therapy that offers benefits to patients with chronic obstructive pulmonary disease that are complementary to those obtained by pharmacotherapy. The main objective of pulmonary rehabilitation is to restore muscle function and exercise tolerance, reverse other nonrespiratory consequences of the disease, and help patients to self-manage chronic obstructive pulmonary disease and its exacerbations and symptoms. To do so, a multidisciplinary program tailored to the pa...

  14. Pulmonary Embolism.

    Science.gov (United States)

    Rali, Parth; Gandhi, Viral; Malik, Khalid

    2016-01-01

    Pulmonary embolism covers a wide spectrum of presentation from an asymptomatic individual to a life-threatening medical emergency. It is of paramount importance to appropriately risk stratify patients with pulmonary embolism, particularly with those who present without hypotension. Right ventricular dysfunction can evolve after a patient has received a diagnosis of pulmonary embolism, necessitating aggressive measures rather than simple anticoagulation. In this review, we discuss definition, risk stratification, pathogenesis, diagnostic approach, and management, with particular focus on massive pulmonary embolism. PMID:26919674

  15. Combination esthetic treatment of anterior teeth affected by idiopathic enamel hypoplasia: a case report.

    Science.gov (United States)

    Roberts, Donald L; Warner, Ben F; Bentley, Dan A; Quock, Ryan L

    2016-01-01

    The esthetic management of enamel dysplasia presents an opportunity for the practitioner to integrate expertise in diagnosis, biomaterials, and technique. In addition to considerations of appearance, careful attention must be paid to occlusal function and restorative material properties. This case report highlights a combined approach of conservative lithium disilicate indirect restorations and composite resin direct veneers to electively restore the anterior teeth of a patient diagnosed with idiopathic enamel hypoplasia. PMID:27148656

  16. Foveal hemorrhage in an eye with foveal hypoplasia associated with albinism

    Directory of Open Access Journals (Sweden)

    Masuda N

    2014-09-01

    Full Text Available Naonori Masuda, Taiji Hasegawa, Mariko Yamashita, Nahoko Ogata Department of Ophthalmology, Nara Medical University, Nara, Japan Abstract: Oculocutaneous albinism is a group of congenital disorders caused by alterations of melanin biosynthesis. We report our findings in a patient with oculocutaneous albinism who presented with foveal hypoplasia and a foveal hemorrhage. A 48-year-old man noted a dark spot in the middle of the visual field of his right eye. He had depigmented skin, white hair, white eyebrows, and white cilia. He also had horizontal nystagmus and depigmented irides. His best-corrected visual acuity was 2/100 with -14.0 diopters in the right eye and 3/100 with -5.0 diopters in the left eye. Ophthalmoscopy showed diffuse depigmentation in both eyes and a foveal hemorrhage in the right eye. Optical coherence tomography showed the absence of a foveal pit in both eyes and a subretinal hyperreflective lesion corresponding to the foveal hemorrhage in the right eye. Fluorescein angiography showed that the retinal and choroidal vessels were relatively hypofluorescent because of the lack of a blocking effect of the pigments in the retinal pigment epithelium. Fluorescein angiography and indocyanine green angiography did not show any evidence of choroidal neovascularization in either eye. The foveal hemorrhage in the right eye spontaneously regressed and finally resolved at 3 months after onset. At the final examination, the patient reported that his vision had recovered. A foveal hemorrhage is a rare condition in an eye with foveal hypoplasia associated with albinism. The hemorrhage may be related to high myopia and also to the hypoplasia of the fovea associated with albinism. Keywords: albinism, foveal hemorrhage, foveal hypoplasia, simple hemorrhage

  17. A case of superior segmental optic hypoplasia accompanied by a glaucomatous optic neuropathy

    OpenAIRE

    Ohguro, Hiroshi

    2008-01-01

    Ikuyo Ohguro, Hiroshi OhguroDepartment of Ophthalmology, Sapporo Medical University School of Medicine, Sapporo, Hokkaido, JapanAbstract: This is the first case report of a bilateral superior segmental optic hypoplasia (SSOH) accompanied by a glaucomatous optic neuropathy (GON). A 47-year-old man incidentally diagnosed as having bilateral SSOH, simultaneously disclosed glaucomatous optic disc appearances, including enlargements of the cup of the optic nerve heads and a thinning of the infero-...

  18. Tigroid pattern of the white matter: a previously unrecognized MR finding in lissencephaly with cerebellar hypoplasia

    Energy Technology Data Exchange (ETDEWEB)

    Kono, Tatsuo [Dokkyo University, Department of Radiology, Mibu, Shimotsuga, Tochigi (Japan); Moriyama, Nobuko [Ibaraki Children' s Hospital, Department of Paediatrics, Mito, Ibaraki (Japan); Tanaka, Ryuta [University of Tsukuba, Department of Paediatrics, Tsukubu, Ibaraki (Japan); Iwasaki, Nobuaki [Ibaraki Prefectural University of Health Sciences, Department of Paediatrics, Ami, Ibaraki (Japan); Arai, Jun-ichi [Ibaraki Children' s Hospital, Department of Neonatology, Mito, Ibaraki (Japan)

    2008-10-15

    Brain MR images of a 14-month-old boy with lissencephaly and cerebellar hypoplasia showed numerous radiating linear structures in the white matter. This finding was identical to the tigroid or leopard-skin pattern that is seen in Pelizaeus-Merzbacher disease or metachromatic leukodystrophy and represents the perivascular white matter spared from demyelination. We speculate that mutations of the reelin gene, expressed both in the cortex and in the white matter, may play an important role in its development. (orig.)

  19. Foveal hemorrhage in an eye with foveal hypoplasia associated with albinism

    OpenAIRE

    Masuda N; Hasegawa T; Yamashita M; Ogata N

    2014-01-01

    Naonori Masuda, Taiji Hasegawa, Mariko Yamashita, Nahoko Ogata Department of Ophthalmology, Nara Medical University, Nara, Japan Abstract: Oculocutaneous albinism is a group of congenital disorders caused by alterations of melanin biosynthesis. We report our findings in a patient with oculocutaneous albinism who presented with foveal hypoplasia and a foveal hemorrhage. A 48-year-old man noted a dark spot in the middle of the visual field of his right eye. He had depigmented skin, white hair...

  20. Learn About Pulmonary Fibrosis

    Science.gov (United States)

    ... www.lung.org > Lung Health and Diseases > Lung Disease Lookup > Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary fibrosis is ... while processing XML file."); } }); } } --> Blank Section Header Lung Disease Lookup Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, ...

  1. Bilateral coronoid hypoplasia and complex odontoma: a rare concurrence of developmental pathology and odontogenic tumour of the mandible.

    Science.gov (United States)

    Dar, Mohd Arif; Alaparthi, Ravikiran; Yalamanchili, Samatha; Santosh, Arvind Babu Rajendra

    2015-01-01

    We present a rare case of concurrent bilateral coronoid hypoplasia and complex odontoma in the mandible, with replacement of missing posterior teeth in both sides of the lower jaw. A 20-year-old woman was diagnosed with bilateral occurrence of coronoid hypoplasia and unerupted complex odontoma after radiographic and histopathological examination. The patient was surgically treated with complete removal of the unerupted complex odontoma and prosthetic replacement of the missing teeth. PMID:26464410

  2. Neuropathological features in a female fetus with OPHN1 deletion and cerebellar hypoplasia.

    Science.gov (United States)

    Rocas, Delphine; Alix, Eudeline; Michel, Jessica; Cordier, Marie-Pierre; Labalme, Audrey; Guilbert, Hélène; Till, Marianne; Schluth-Bolard, Caroline; de Haas, Pascale; Massardier, Jérôme; Portes, Vincent des; Edery, Patrick; Touraine, Renaud; Guibaud, Laurent; Vasiljevic, Alexandre; Sanlaville, Damien

    2013-05-01

    We report the case of a 33-year-old pregnant woman. The third-trimester ultrasound scan during pregnancy revealed fetal bilateral ventricular dilatation, macrosomia and a transverse diameter of the cerebellum at the 30th centile. A brain MRI scan at 31 weeks of gestation led to a diagnosis of hypoplasia of the cerebellar vermis without hemisphere abnormalities and a non compressive expansion of the cisterna magna. The fetal karyotype was 46,XX. The pregnancy was terminated and array-CGH analysis of the fetus identified a 238 kb de novo deletion on chromosome Xp12, encompassing part of OPHN1 gene. Further studies revealed a completely skewed pattern of X inactivation. OPHN1 is involved in X-linked mental retardation (XLMR) with cerebellar hypoplasia and encodes a Rho-GTPase-activating protein called oligophrenin-1, which is produced throughout the developing mouse brain and in the hippocampus and Purkinje cells of the cerebellum in adult mice. Neuropathological examination of the female fetus revealed cerebellar hypoplasia and the heterotopia of Purkinje cells at multiple sites in the white matter of the cerebellum. This condition mostly affects male fetuses in humans. We report here the first case of a de novo partial deletion of OPHN1, with radiological and neuropathological examination, in a female fetus. PMID:23416624

  3. Enamel hypoplasia in the deciduous teeth of great apes: variation in prevalence and timing of defects.

    Science.gov (United States)

    Lukacs, J R

    2001-11-01

    The prevalence of enamel hypoplasia in the deciduous teeth of great apes has the potential to reveal episodes of physiological stress in early stages of ontogenetic development. However, little is known about enamel defects of deciduous teeth in great apes. Unresolved questions addressed in this study are: Do hypoplastic enamel defects occur with equal frequency in different groups of great apes? Are enamel hypoplasias more prevalent in the deciduous teeth of male or female apes? During what phase of dental development do enamel defects tend to form? And, what part of the dental crown is most commonly affected? To answer these questions, infant and juvenile skulls of two sympatric genera of great apes (Gorilla and Pan) were examined for dental enamel hypoplasias. Specimens from the Powell-Cotton Museum (Quex Park, UK; n = 107) are reported here, and compared with prior findings based on my examination of juvenile apes at the Cleveland Museum of Natural History (Hamman-Todd Collection; n = 100) and Smithsonian Institution (National Museum of Natural History; n = 36). All deciduous teeth were examined by the author with a x10 hand lens, in oblique incandescent light. Defects were classified using Fédération Dentaire International (FDI)/Defects of Dental Enamel (DDE) standards; defect size and location on the tooth crown were measured and marked on dental outline charts. Enamel defects of ape deciduous teeth are most common on the labial surface of canine teeth. While deciduous incisor and molar teeth consistently exhibit similar defects with prevalences of approximately 10%, canines average between 70-75%. Position of enamel defects on the canine crown was analyzed by dividing it into three zones (apical, middle, and cervical) and calculating defect prevalence by zone. Among gorillas, enamel hypoplasia prevalence increases progressively from the apical zone (low) to the middle zone to the cervical zone (highest), in both maxillary and mandibular canine teeth

  4. Tricuspid atresia and pulmonary atresia in a child with Rubinstein-Taybi syndrome.

    Science.gov (United States)

    Loomba, Rohit S; Geddes, Gabrielle

    2015-01-01

    Rubinstein-Taybi syndrome is a well-characterized condition causing distinctive physical characteristics, intellectual disability, and multiple congenital malformations. Cardiac abnormalities are found in a third of individuals with this condition and usually consist of isolated septal defects or patent ductus arteriosus, although more complex congenital lesions have been described. We present the first reported case of tricuspid atresia and pulmonary atresia with hypoplasia of the right ventricle in the setting of Rubenstein-Taybi syndrome. PMID:26085772

  5. Tricuspid atresia and pulmonary atresia in a child with Rubinstein-Taybi syndrome

    OpenAIRE

    2015-01-01

    Rubinstein-Taybi syndrome is a well-characterized condition causing distinctive physical characteristics, intellectual disability, and multiple congenital malformations. Cardiac abnormalities are found in a third of individuals with this condition and usually consist of isolated septal defects or patent ductus arteriosus, although more complex congenital lesions have been described. We present the first reported case of tricuspid atresia and pulmonary atresia with hypoplasia of the right vent...

  6. Tricuspid atresia and pulmonary atresia in a child with Rubinstein-Taybi syndrome

    Directory of Open Access Journals (Sweden)

    Rohit S Loomba

    2015-01-01

    Full Text Available Rubinstein-Taybi syndrome is a well-characterized condition causing distinctive physical characteristics, intellectual disability, and multiple congenital malformations. Cardiac abnormalities are found in a third of individuals with this condition and usually consist of isolated septal defects or patent ductus arteriosus, although more complex congenital lesions have been described. We present the first reported case of tricuspid atresia and pulmonary atresia with hypoplasia of the right ventricle in the setting of Rubenstein-Taybi syndrome.

  7. Evolving hybrid approaches: the preservation of the 'neglected' pulmonary valve function in patients with congenital heart disease.

    Science.gov (United States)

    Vida, Vladimiro L; Bacha, Emile; Stellin, Giovanni

    2016-07-01

    The involvement of the hemodynamic expertise in the operating room led to the development of new strategies aimed to improve both early and long-term outcome of patients with congenital heart disease. During the last decade, with the aim of preserving the pulmonary valve function, we embarked on a new surgical approach, which combines surgical and interventional techniques, which are performed in the operating room. We believe that the preservation of the pulmonary valve function can be extended to any patients with classic tetralogy of Fallot and other selected patients with congenital pulmonary valve hypoplasia and dysfunction. PMID:26950198

  8. A Rare Cause of Persistent Pulmonary Hypertension Resistant to Therapy in The Newborn: Short-Rib Polydactyly Syndrome

    Directory of Open Access Journals (Sweden)

    Nihat Demir

    2015-01-01

    Full Text Available Short-rib polydactyly syndrome is an autosomal recessively inherited lethal skeletal dysplasia. The syndrome is characterized by marked narrow fetal thorax, short extremities, micromelia, cleft palate/lip, polydactyly, cardiac and renal abnormalities, and genital malformations. In cases with pulmonary hypoplasia, persistent pulmonary hypertension of the newborn can develop. In this paper, we present a term newborn with persistent pulmonary hypertension of the newborn, which has developed secondary to short-rib polydactyly syndrome and was resistant to therapy with inhaled nitric oxide and oral sildenafil.

  9. IMAGe association: report of two cases in siblings with adrenal hypoplasia and review of the literature.

    Science.gov (United States)

    Phillips, Katherine; Arroyo, May R; Duckworth, Lizette Vila

    2014-01-01

    We report the postmortem findings of two siblings with gross and microscopic features consistent with IMAGe association (Intrauterine growth retardation, Metaphyseal dysplasia, Adrenal hypoplasia congenita, and Genital anomalies) with an emphasis on the histopathology of the adrenal gland in this rare syndrome. The first sibling was an 8-week old male diagnosed postnatally with primary adrenal insufficiency. There was no deletion of the DAX1 gene by FISH. Examination at autopsy revealed dysmorphic features including frontal bossing, epicanthal folds, flat philtrum, cryptorchidism, penile chordee, overriding fourth toe, and height and weight below 3rd percentile. Grossly, the adrenal glands were not identified; however, microscopic examination of the suprarenal soft tissue revealed a 3 mm focus of disorganized fetal adrenal cortex with distended "cytomegalic" cells with abundant pink eosinophilic cytoplasm, vesicular nuclei, and cytoplasmic vacuolization. A minute focus of permanent adult cortex was also seen, but no adrenal medulla was identified. An autopsy of the sibling, who died 12 years previously at day 9 of life, revealed dysmorphic facial features with cryptorchidism and a large phallus. The adrenal glands were grossly hypoplastic (11 mm). Histologically, the adrenal glands showed disorganized fetal cortex with cytomegalic cells, a larger amount of permanent adult cortex, and bizarre nuclei with numerous pseudoinclusions. While there is currently limited information regarding the histopathologic adrenal findings in IMAGe association, our small case series suggests overlapping features between X-linked recessive congenital adrenal hypoplasia (cytomegalic cells with lack of permanent adult cortex) and autosomal recessive congenital adrenal hypoplasia (diminished permanent adult cortex without cytomegalic cells). PMID:24617583

  10. Living with Pulmonary Fibrosis

    Science.gov (United States)

    ... www.lung.org > Lung Health and Diseases > Lung Disease Lookup > Pulmonary Fibrosis Living With Pulmonary Fibrosis What to Expect ... while processing XML file."); } }); } } --> Blank Section Header Lung Disease Lookup Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, ...

  11. [Aplasia and hypoplasia of the side of the nose in the Mocha Period and today].

    Science.gov (United States)

    Pirsig, W

    1989-01-01

    In earthenware vessels from the Moche period (200-700 A.D.) pathological findings of nasal deformities have been depicted in a realistic manner. By means of two recent casuistics and of two portrait vessels of Old Peru it is demonstrated how exactly the nasal pathology was observed by the artists of the Moche period. The first example shows a unilateral hypoplasia of the nasal ala due to injury in early childhood, the second example shows a bilateral aplasia of the nasal alae due to an intrauterine growth disturbance. PMID:2690796

  12. Bilateral multicystic renal dysplasia with potter sequence: A case with penile agenesis

    International Nuclear Information System (INIS)

    Hereditary renal adysplasia (HRA) is rare autosomal dominant condition. Patients have several other anomalies including Potter facies, thoracic, cardiac, and extremity deformities. The case present dysmorphic facial features such as hypertelorism, prominent epicanthic folds, a flat and broad nose, choanal stenosis, low set ears and a receding chin. He had femoral bowing, hypoplastic right tibia and agenesis of the right foot. He had rich and thin skin. He had also a dysplastic empty scrotum, penile agenesis, and anal atresia. The autopsy revealed pulmonary hypoplasia, ventricular septal defect, bilateral multicystic renal dysplasia, agenesis of ureter and bladder, intraabdominal testicles, and a single umbilical artery. The penile agenesis was first reported, and including the consanguinity in the parents might further delineate the bilateral multicystic HRA. Vater/caudal regression anomalies, Mullerian duct/aplasia, unilateral renal agenesis, and cervicothoracic somite anomalies association and Coloboma, heart anomaly, choanal atresia, retardation, retardation, genital and ear anomalies syndrome has been considered in differential diagnosis. (author)

  13. Pulmonary studies

    International Nuclear Information System (INIS)

    Radionuclide studies of the lung are described, as regards perfusion studies, ventilation studies and physiological considerations. The four principal applications for radionuclide studies of the lungs are outlined and the uses of these discussed in relation to particular entities including pulmonary embolic disease, congestive heart failure, asthma, acute, nonasthmatic, bronchial obstruction, chronic pulmonary disease and cancer. (Auth./C.F.)

  14. Potential risk factors in systemic hypoplasia and dental caries at odontogenesis stage

    Directory of Open Access Journals (Sweden)

    Kovach I.V.

    2013-03-01

    Full Text Available Lesion of hard dental tissue with caries process and lesions not connected with caries present overwhelming majority of dental diseases. Causes leading to damage of enamel integrity and dentin with progressing demineralization are known; they allow to choose justified method of primary prophylaxis. Along with it, etiology and pathogenesis of enamel lesions with demineralization signs remains unexplored. Epidemiologic study of prevalence, incidence and severity of lesion of hard dental tissues in 330 pupils, who constantly live on endemic iodine-defficient territory, of three zones – mountain zone, foothill and valley. For comparison, schoolchildren migrated to this territory 2-3 years after birth, and children – residents of favorable as for iodine level in drinking water were examined, 30 children in each group. It was established, that overwhelming number of schoolchildren of endemic zone suffer from endemic goiter of various severity, which sufficiently impacts on protein-mineral ratio of the organism, bone skeleton and teeth including. It was also established, that residents of three various levels of iodine consumption are prone to development of multiple dental caries and systemic enamel hypoplasia. Children, residents of mountain zone suffer the most, with simultaneous lesion of thyroid gland. The role of endemic goiter in systemic dental enamel hypoplasia and multiple caries, which changes protein matrix, negatively impacts on odontogenesis and mineralization degree is underlined.

  15. Chronic hepatitis C infection in a patient with bone marrow hypoplasia

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    Chronic hepatitis C virus (HCV) infection is associated with multifarious extra-hepatic manifestations; the most described and discussed being mixed cryoglob- ulinemia which is strongly related to B-cell lympho- proliferative disorders (LPDs). We present a case of chronic HCV infection and mixed cryoglobulinemia, with minimal liver involvement. The case is a 53-year- old patient who was diagnosed as having bone marrow hypoplasia at the age of three. She received several blood transfusions to normalize her haemoglobin. At the age of 31, she was diagnosed with rheumatoid ar- thritis on account of her diffuse joint pain and inflam- mation, elevated rheumatoid factor (RF) and Raynaud's phenomenon. Twenty years later, monoclonal gam- mopathy of IgG Lambda (one year later, changed to IgM Kappa) was detected during a routine examina- tion. A bone marrow biopsy showed hypoplasia, Kappa positive B-lymphocytes and low-grade malignant lym- phoma cells. PCR of the bone marrow aspirate was not contributory. No treatment was initiated owing to her poor bone marrow function and she is under regular follow-up.

  16. Managing elevated intraocular pressure in a patient with optic nerve hypoplasia

    Directory of Open Access Journals (Sweden)

    Karina Marcovitch

    2009-10-01

    Full Text Available Karina Marcovitch, Joseph SowkaNova Southeastern University, College of Optometry, Davie, FL, USABackground: Optic nerve hypoplasia (ONH is a congenital optic disc anomaly, often manifesting with visual deficits.Case: A 51-year-old woman with a history of bilateral amblyopia was referred for glaucoma evaluation due to elevated intraocular pressure. The patient demonstrated the classic nerve head appearance of bilateral ONH with “double ring sign” and indistinguishable cupping. Despite demonstrating functional and structural measurements consistent with glaucomatous optic neuropathy, it was felt that these deficits were more likely longstanding and secondary to ONH. Additionally, in the absence of any amblyogenic factors, it was also concluded that the patient’s bilateral “amblyopia” was the result of ONH. In that the patient presented with a significant and modifiable risk factor for glaucomatous development and pre-existing retinal nerve fiber layer and visual field defects, the patient was treated with topical glaucoma medications. Conclusion: Diagnosing glaucoma in patients with concurrent anomalies is a clinical conundrum. This report provides a review of ONH with emphasis on the common misdiagnosis of amblyopia in these patients as well as presenting a strategy for diagnosing and managing glaucoma in patients with preexisting, confounding conditions. Keywords: optic nerve hypoplasia, glaucoma, visual field loss, scanning laser polarimetry, threshold automated perimetry

  17. Restoration of incisal half with edge-up technique using ceramic partial crown in turner's hypoplasia: A case report.

    Science.gov (United States)

    Hegde, Shreya; Kundabala, M

    2014-01-01

    This case report describes a rare treatment modality for Turner's hypoplasia done with a very conservative approach for the esthetic and functional problem of the defect. Diagnosis was made as Turner's hypoplasia of upper two central incisors with proximal caries. Treatment planning was done after considering many factors such as conservation of tooth structure, esthetics, occlusion and economy. Tooth preparation was done to receive Edge-up, all ceramic partial crowns for both the upper central incisors,using pressable all ceramic material and cemented with resin cement. PMID:24554869

  18. Pulmonary hypertension due to obstructive sleep apnea in a child with Rubinstein-Taybi syndrome

    Directory of Open Access Journals (Sweden)

    Hyung Soon Choi

    2012-06-01

    Full Text Available Rubinstein-Taybi syndrome (RTS is characterized by peculiar facies, mental retardation, broad thumbs, and great toes. Approximately onethird of the affected individuals have a variety of congenital heart diseases. They can also have upper airway obstruction during sleep, due to hypotonia and the anatomy of the oropharynx and airway, which make these patients susceptible to obstructive sleep apnea (OSA. In our case, pulmonary hypertension was caused, successively, by congenital heart defects (a large patent ductus arteriosus and arch hypoplasia and obstructive sleep apnea during early infancy. The congenital heart defects were surgically corrected, but persistent pulmonary hypertension was identified 2 months after the operation. This pulmonary hypertension was due to OSA, and it was relieved by nasal continuous positive airway pressure. This case is the first report of pulmonary hypertension from OSA in a young infant with RTS.

  19. Pulmonary hypertension due to obstructive sleep apnea in a child with Rubinstein-Taybi syndrome.

    Science.gov (United States)

    Choi, Hyung Soon; Yu, Jeong Jin; Kim, Young-Hwue; Ko, Jae-Kon; Park, In-Sook

    2012-06-01

    Rubinstein-Taybi syndrome (RTS) is characterized by peculiar facies, mental retardation, broad thumbs, and great toes. Approximately one-third of the affected individuals have a variety of congenital heart diseases. They can also have upper airway obstruction during sleep, due to hypotonia and the anatomy of the oropharynx and airway, which make these patients susceptible to obstructive sleep apnea (OSA). In our case, pulmonary hypertension was caused, successively, by congenital heart defects (a large patent ductus arteriosus and arch hypoplasia) and obstructive sleep apnea during early infancy. The congenital heart defects were surgically corrected, but persistent pulmonary hypertension was identified 2 months after the operation. This pulmonary hypertension was due to OSA, and it was relieved by nasal continuous positive airway pressure. This case is the first report of pulmonary hypertension from OSA in a young infant with RTS. PMID:22745646

  20. Prostaglandin E2 decreases fetal breathing movements, but not pulmonary blood flow, in fetal sheep.

    Science.gov (United States)

    Savich, R D; Guerra, F A; Lee, C C; Kitterman, J A

    1995-04-01

    Fetal breathing movements are vital for normal fetal lung growth. Inhibition of these fetal breathing movements is associated with pulmonary hypoplasia. Pulmonary hypoplasia also occurs subsequent to alterations in other factors, such as a significant decrease in pulmonary blood flow. The prostaglandin system is known to have profound effects on both fetal breathing movements and on the pulmonary vascular system. We studied six late-gestation chronically instrumented fetal sheep by using an electromagnetic flow transducer around the left pulmonary artery to determine whether a decrease in fetal breathing movements, subsequent to a continuous infusion of prostaglandin E2 (PGE2), is associated with a decrease in pulmonary blood flow. A continuous PGE2 infusion of 0.88 +/- 0.11 microgram.kg-1.min-1 over 120 min led to a significant decrease in fetal breathing movements (control 40.5 +/- 3.6%, infusion 3.3 +/- 1.6%; P < 0.001). In contrast, the PGE2 infusion had no effect on mean left pulmonary artery blood flow (control 27.7 +/- 9.3 ml.min-1.kg-1, infusion 23.8 +/- 7.0 ml.min-1.kg-1. The PGE2 infusion demonstrated central effects in the percentage of time the fetus was in high-voltage electrocortical activity (control 41.9 +/- 2.5%, infusion 56.5 +/- 5.4%; P < 0.05) and in the amount of time spent in low-voltage electrocortical activity without fetal breathing movements (control 17.5 +/- 2.7%, infusion 40.2 +/- 4.8%; P < 0.05). A significant decrease in the fetal heart rate during the infusion was seen with no effect on either the systemic or pulmonary blood pressure.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:7615458

  1. Pulmonary Atresia

    Science.gov (United States)

    ... to repair the defect. Return to main topic: Congenital Heart Disease See on other sites: MedlinePlus https://medlineplus.gov/ency/article/001091.htm Pulmonary atresia American Heart Association www. ...

  2. [Pulmonary rehabilitation].

    Science.gov (United States)

    Senjyu, Hideaki

    2016-05-01

    Pulmonary rehabilitation commenced in Japan in 1957. However, the development of pulmonary rehabilitation took a long time due to the lack of the necessary health and medical services. Pulmonary rehabilitation is a comprehensive intervention based on a thorough patient assessment followed by patient-tailored therapies that include, but are not limited to, exercise training, education, and behavior change, designed to improve the physical and psychological condition of people with chronic respiratory disease and to promote the long-term adherence to health-enhancing behaviors. The benefits of pulmonary rehabilitation include a decrease in breathlessness and an improvement in exercise tolerance. It is important that the gains in exercise tolerance lead to an increase in daily physical activity. PMID:27254948

  3. Pulmonary sequestration

    International Nuclear Information System (INIS)

    Pulmonary sequestration is a congenital affection consisting in the presence of a cystic mass of no-functional pulmonary tissue without an obvious communication with tracheobronchial tree and that receives all or most of its bloodstream of the anomalous vessels from systemic circulation. Taking into account that presentation of this affection is rare compared to other pulmonary affections (between the 1% and the 2% of all pulmonary resections) and that also the more usual is its definitive treatment before adulthood. The case of man aged 44 is presented coming to consultation due to frequent episodes of pneumonias from more 10 years ago diagnosed as a bronchiectasis. The more significant facts of embryology origin of this affection including: anatomical and pathological features, imaging diagnosis, surgical treatment details, and postoperative course. (author)

  4. PULMONARY SCEDOSPORIOSIS

    Directory of Open Access Journals (Sweden)

    SEVERO Luiz Carlos

    1998-01-01

    Full Text Available A case of a solitary pulmonary nodule due to Scedosporium apiospermum (Pseudallescheria boydii is related. A review of the pertinent literature was done and, in addition, similar lesions caused by other opportunistic fungi are commented.

  5. Pulmonary Edema

    OpenAIRE

    Tanser, Paul H.

    1981-01-01

    The physician who deals with pulmonary edema from a pathophysiologic basis will seldom make a diagnostic or therapeutic error. Recent additions to preload and afterload therapy have greatly helped in the emergency and ambulatory treatment of pulmonary edema due to left heart failure. Careful follow-up and patient self-monitoring are the most effective means of reducing hospitalization of chronic heart failure patients.

  6. Pulmonary hypertension

    OpenAIRE

    Anton Vonk Noordegraaf; Joanne A. Groeneveldt; Harm Jan Bogaard

    2016-01-01

    In 2015, more than 800 papers were published in the field of pulmonary hypertension. A Clinical Year in Review article cannot possibly incorporate all this work and needs to be selective. The recently published European guidelines for the diagnosis and treatment of pulmonary hypertension contain an inclusive summary of all published clinical studies conducted until very recently. Here, we provide an overview of papers published after the finalisation of the guideline. In addition, we summaris...

  7. Discrepancy in compliance between the clinical and genetic diagnosis of choroidal hypoplasia in Danish Rough Collies and Shetland Sheepdogs

    DEFF Research Database (Denmark)

    Fredholm, Merete; Larsen, R. C.; Jönsson, M.; Söderlund, M. A.; Hardon, T.; Proschowsky, H. F.

    2016-01-01

    Collie eye anomaly (CEA) is a congenital, inherited ocular disorder which is widespread in herding breeds. Clinically, the two major lesions associated with CEA are choroidal hypoplasia (CH) and coloboma, and both lesions are diagnosed based on ophthalmological examination. A 7.8-kb intronic...

  8. Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery

    Energy Technology Data Exchange (ETDEWEB)

    Greenberg, S.B.; Crisci, K.L.; Koenig, P.; Robinson, B.; Anisman, P.; Russo, P. [St. Christopher`s Hospital for Children, Front Street at Erie Avenue, Philadelphia, PA 19134 (United States)

    1997-12-01

    Background. Abnormalities of the pulmonary arteries following palliative or corrective surgery for tetralogy of Fallot (TOF) are common. Our purpose was to compare the usefulness of magnetic resonance imaging (MRI) and echocardiography in the post- operative evaluation of the pulmonary arteries in children with TOF. Objective. Our hypothesis was that MRI is more sensitive than echocardiography in the detection of branch pulmonary artery abnormalities in children with TOF. Materials and methods. Pulmonary artery MRI and echocardiography were performed in 20 children following palliative and/or corrective surgery for TOF. MRI and echocardiography were compared in their ability to detect abnormalities of the pulmonary arteries. Angiographic or surgical correlation was available in 15 children. A perfusion scan for confirmation of pulmonary artery patency was available in one additional child. Results. Abnormalities of the branch pulmonary arteries identified by MRI included: absence or occlusion (2), focal stenosis (15), hypoplasia (2), aneurysm (1), and non-confluence (1). Echocardiography could not adequately visualize the right and left branch pulmonary arteries in eight and ten children, respectively. Echocardiography missed stenosis in 13 branch pulmonary arteries, patency of hypoplastic pulmonary arteries in two children, non-confluence of the pulmonary arteries in one child, and a left pulmonary artery aneurysm in one child. Abnormalities identified by MRI were confirmed in 16 children by angiography, surgery or perfusion scan. Conclusion. MRI is more sensitive than echocardiography for the evaluation of branch pulmonary artery abnormalities in children following surgery for TOF. (orig.) With 2 figs., 3 tabs., 11 refs.

  9. Magnetic resonance imaging compared with echocardiography in the evaluation of pulmonary artery abnormalities in children with tetralogy of Fallot following palliative and corrective surgery

    International Nuclear Information System (INIS)

    Background. Abnormalities of the pulmonary arteries following palliative or corrective surgery for tetralogy of Fallot (TOF) are common. Our purpose was to compare the usefulness of magnetic resonance imaging (MRI) and echocardiography in the post- operative evaluation of the pulmonary arteries in children with TOF. Objective. Our hypothesis was that MRI is more sensitive than echocardiography in the detection of branch pulmonary artery abnormalities in children with TOF. Materials and methods. Pulmonary artery MRI and echocardiography were performed in 20 children following palliative and/or corrective surgery for TOF. MRI and echocardiography were compared in their ability to detect abnormalities of the pulmonary arteries. Angiographic or surgical correlation was available in 15 children. A perfusion scan for confirmation of pulmonary artery patency was available in one additional child. Results. Abnormalities of the branch pulmonary arteries identified by MRI included: absence or occlusion (2), focal stenosis (15), hypoplasia (2), aneurysm (1), and non-confluence (1). Echocardiography could not adequately visualize the right and left branch pulmonary arteries in eight and ten children, respectively. Echocardiography missed stenosis in 13 branch pulmonary arteries, patency of hypoplastic pulmonary arteries in two children, non-confluence of the pulmonary arteries in one child, and a left pulmonary artery aneurysm in one child. Abnormalities identified by MRI were confirmed in 16 children by angiography, surgery or perfusion scan. Conclusion. MRI is more sensitive than echocardiography for the evaluation of branch pulmonary artery abnormalities in children following surgery for TOF. (orig.)

  10. A case of optic-nerve hypoplasia and anterior segment abnormality associated with facial cleft

    Directory of Open Access Journals (Sweden)

    Miyake T

    2016-07-01

    Full Text Available Tomoko Miyake,1 Shota Kojima,1 Tetsuya Sugiyama,2 Mari Ueki,1 Jun Sugasawa,1 Hidehiro Oku,1 Kensuke Tajiri,1 Yuka Shigemura,3 Koichi Ueda,3 Atsuko Harada,4 Mami Yamasaki,4 Takumi Yamanaka,4 Hidetsuna Utsunomiya,5 Tsunehiko Ikeda1 1Department of Ophthalmology, Osaka Medical College, Takatsuki City, 2Nakano Eye Clinic of Kyoto Medical Co-operative, Kyoto, 3Department of Plastic and Reconstructive Surgery, Osaka Medical College, Takatsuki City, 4Department of Pediatric Neurosurgery, Takatsuki General Hospital, Takatsuki City, 5Department of Radiological Science, International University of Health and Welfare, Graduate School, Fukuoka, Japan Introduction: The incidence of facial cleft is rare and ranges between 1.43 and 4.85 per 100,000 births. To date, there have been few reports of detailed ophthalmologic examinations performed in cases of facial cleft. Here, we report a case of optic-nerve hypoplasia and anterior segment abnormality associated with facial cleft. Case report: A 9-day-old female infant was delivered by cesarian section at 34 weeks of gestational age (the second baby of twins and weighed 2,276 g upon presentation. She had a facial cleft and ectrodactyly at birth. Right eye-dominant blepharophimosis was obvious. Examination of the right eye revealed inferior corneal opacity with vascularization, downward corectopia, and optic-nerve hypoplasia. The corneal diameter was 8 mm in both eyes, and tonometry by use of a Tono-Pen® XL (Reichert Technologies, Depew, NY, USA handheld applanation tonometer revealed that her intraocular pressure was 11–22 mmHg (Oculus Dexter and 8 mmHg (Oculus Sinister. B-mode echo revealed no differences in axial length between her right and left eyes. When she was 15–16 months old, we attempted to examine her eyes before she underwent plastic surgery under general anesthesia. She had a small optic disc in both eyes and the right-eye disc was tilted. After undergoing canthotomy, gonioscopy and ultrasound

  11. Focal dermal hypoplasia due to a novel mutation in a boy with Klinefelter syndrome.

    Science.gov (United States)

    Alkindi, Said; Battin, Malcolm; Aftimos, Salim; Purvis, Diana

    2013-01-01

    A boy was born with multiple anomalies, including right hemifacial microsomia, eye abnormalities, syndactyly, right hand ectrodactyly, hypoplastic nails, omphalocele, bladder exstrophy, renal dilatation, and splayed symphysis pubis. The skin was also abnormal, with atrophic skin plaques and areas of telangiectasia along the lines of Blaschko. The karyotype was 47,XXY (Klinefelter syndrome). He was found to have a heterozygous mutation in the PORCN gene. He exhibited the classical features of focal dermal hypoplasia. Fewer than 15% of reported cases are male when it is thought to be due to postzygotic mutation and thus mosaic. This is the first reported boy to have heterozygous mutation for Goltz syndrome who survived due to the extra X chromosome. PMID:23131169

  12. Hypodontia and hypoplasia of mandible: A rare defect caused by combination therapy

    Directory of Open Access Journals (Sweden)

    Senthil Kumar

    2013-01-01

    Full Text Available Hypodontia is defined as the developmental absence of one or more tooth and it can present in varying degrees of severity and severe hypodontia has been defined as the absence of six teeth, excluding third molars. Radiation plays an important role in the treatment of head and neck cancer, in spite of its benefits, radiation has several side-effects in the head and neck region. This article highlights a case report, where hypodontia, hypoplasia of mandible, stunded permanent teeth roots, microdontia, inversion of tooth bud were observed in 19 years old patient who was diagnosed with Langerhans cell histiocytosis at her age of 3 years she had received a combination of radiotherapy and chemotherapy for a period of 1 year and radiation of 50 Gy was used in the head and neck region. Radiographs were taken and it revealed abnormal changes in growth and development of bone and teeth.

  13. Rare Case of Unilateral Hypoplasia of Lung with Associated Ventricular Mass in an Adult

    Science.gov (United States)

    Alam, Azad; Iyer, Aparna; Kutty, Jayalakshmi Thelapurath

    2016-01-01

    Hypoplasia of the lung is a rare congenital condition which can be: a) primary i.e. no apparent cause is found; or b) secondary i.e. associated with other congenital anomalies that are implicated in its pathogenesis. These anomalies may involve the diaphragm, cardiovascular, central nervous, urogenital and musculoskeletal system. Patients usually present in neonatal, infancy or childhood period and very rarely in adulthood. Our patient was an adult having a unilateral hypoplastic lung associated with a ventricular mass and to our knowledge this rare combination has never been reported in the English literature; though there are reports of prenatal or newborns with hypoplastic lung and rhabdomyoma of ventricle who did not survive.

  14. Management of Zinner’s Syndrome Associated with Contralateral Seminal Vesicle Hypoplasia: A Case Report

    Directory of Open Access Journals (Sweden)

    Mehdi Kardoust Parizi

    2013-01-01

    Full Text Available A 27-year-old man presented with chronic hematospermia, painful ejaculation, and primary infertility. Physical examination, transrectal ultrasonography, and pelvic magnetic resonance imaging (MRI demonstrated left seminal vesicle cyst, left renal agenesia, and contralateral seminal vesicle hypoplasia. Hormone workup (LH, FSH, prolactin, and testosterone was normal. Sperm analysis showed oligoasthenozoospermia and low ejaculate volume. We performed transurethral resection of the ejaculatory duct (TUR-ED using methylene blue vasography guidance without surgical-related complications. Hematospermia and painful ejaculation completely improved at 2-month followup, and the patient’s wife experienced a missed abortion thereafter. This patient was considered as a rare variant of Zinner’s syndrome and was managed effectively with a less invasive treatment modality (TUR-ED.

  15. Types of Pulmonary Hypertension

    Science.gov (United States)

    ... from the NHLBI on Twitter. Types of Pulmonary Hypertension The World Health Organization divides pulmonary hypertension (PH) ... are called pulmonary hypertension.) Group 1 Pulmonary Arterial Hypertension Group 1 PAH includes: PAH that has no ...

  16. Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children

    International Nuclear Information System (INIS)

    Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by ∼50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5–13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2–8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 ± 11.3 to 10.8 ± 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 ± 0.5 to 4.0 ± 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 ± 53.6 to 65.7 ± 9.6 μmol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia

  17. Staged Transcatheter Treatment of Portal Hypoplasia and Congenital Portosystemic Shunts in Children

    Energy Technology Data Exchange (ETDEWEB)

    Bruckheimer, Elchanan, E-mail: elchananb@bezeqint.net; Dagan, Tamir [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel); Atar, Eli; Schwartz, Michael [Schneider Children' s Medical Center Israel, Section of Radiology (Israel); Kachko, Ludmila [Schneider Children' s Medical Center Israel, Section of Anesthesiology (Israel); Superina, Riccardo; Amir, Gabriel [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel); Shapiro, Rivka [Schneider Children' s Medical Center Israel, Section of Gastroenterology (Israel); Birk, Einat [Schneider Children' s Medical Center Israel, Section of Pediatric Cardiology (Israel)

    2013-12-15

    Purpose: Congenital portosystemic shunts (CPSS) with portal venous hypoplasia cause hyperammonemia. Acute shunt closure results in portal hypertension. A transcatheter method of staged shunt reduction to afford growth of portal vessels followed by shunt closure is reported. Methods: Pressure measurements and angiography in the CPSS or superior mesenteric artery (SMA) during temporary occlusion of the shunt were performed. If vessels were diminutive and the pressure was above 18 mmHg, a staged approach was performed, which included implantation of a tailored reducing stent to reduce shunt diameter by {approx}50 %. Recatheterization was performed approximately 3 months later. If the portal pressure was below 18 mmHg and vessels had developed, the shunt was closed with a device. Results: Six patients (5 boys, 1 girl) with a median age of 3.3 (range 0.5-13) years had CPSS portal venous hypoplasia and hyperammonemia. Five patients underwent staged closure. One patient tolerated acute closure. One patient required surgical shunt banding because a reducing stent could not be positioned. At median follow-up of 3.8 (range 2.2-8.4) years, a total of 21 procedures (20 transcatheter, 1 surgical) were performed. In all patients, the shunt was closed with a significant reduction in portal pressure (27.7 {+-} 11.3 to 10.8 {+-} 1.8 mmHg; p = 0.016), significant growth of the portal vessels (0.8 {+-} 0.5 to 4.0 {+-} 2.4 mm; p = 0.037), and normalization of ammonia levels (202.1 {+-} 53.6 to 65.7 {+-} 9.6 {mu}mol/L; p = 0.002) with no complications. Conclusion: Staged CPSS closure is effective in causing portal vessel growth and treating hyperammonemia.

  18. Hypoplasia of the nose and eyes, hyposmia, hypogeusia, and hypogonadotrophic hypogonadism in two males.

    Science.gov (United States)

    Bosma, J F; Henkin, R I; Christiansen, R L; Herdt, J R

    1981-01-01

    Two males, 9-11 and 29-31 years of age, with severe hypoplasia of the nose, hypoplasia of the eyes, sensory abnormalities of taste and smell, and hypogonadism were studied. The nasal septum, cribriform plates and foramina of the vomeronasal (vn) nerves were demonstrated in both; the capsule of the vn organ was shown in one. Their nasal skeleton, demonstrated by tomoradiography, had grown in early embryological form. The nose was not patent in either patient. In both, the cranial vaults, orbits, epipharynges, and oral cavities were indented toward the hypoplastic nasal composite and the peripheral dimensions of their faces were normal for their respective ages. Each patient had impaired visual function with cataracts and colobomata. Each was unable to recognize the smell of any vapor (Type I hyposmia), and had severe impairment of recognition of any tastant (recognition hypogeusia); detection of vapors and of tastants were in appropriate anatomical areas. Each was unable orally to recognize standard plastic forms (astereognosis) though each could recognize the forms manually. Each patient had bilateral inguinal hernias, one or two undescended testes, and hypogonadotrophic hypogonadism. These patients do not fall within the spectrum of arrhinencephaly because of the presence of medial structure of attachment of the falx cerebri and because of their normal intelligence. Distinction of patients with this pattern of abnormalities from arrhinencephaly is important by reason of their potentiality of normal mental development. We hypothesize that their abnormalities resulted from an embryological disruption that occurred in the first trimester of pregnancy. The embryogenesis of the nasal composite is presumed to have been adequate for reciprocal induction of the anlagen of the forebrain. Development of their faces to normal peripheral dimensions indicates that the nasal composite is not essential for gross facial enlargement. PMID:6802865

  19. Pulmonary embolism

    International Nuclear Information System (INIS)

    Pulmonary embolism is a common medical problem whose incidence is likely to increase in our aging population. Although it is life-threatening, effective therapy exists. The treatment is not, however, without significant complications. Thus, accurate diagnosis is important. Unfortunately, the clinical manifestations of pulmonary embolism are nonspecific. Furthermore, in many patients the symptoms of an acute embolism are superimposed on underlying chronic heart or lung disease. Thus, a high index of suspicion is needed to identify pulmonary emboli. Laboratory parameters, including arterial oxygen tensions and electrocardiography, are as nonspecific as the clinical signs. They may be more useful in excluding another process than in diagnosing pulmonary embolism. The first radiologic examination is the chest radiograph, but the clinical symptoms are frequently out of proportion to the findings on the chest films. Classic manifestations of pulmonary embolism on the chest radiograph include a wedge-shaped peripheral opacity and a segmental or lobar diminution in vascularity with prominent central arteries. However, these findings are not commonly seen and, even when present, are not specific. Even less specific findings include cardiomegaly, pulmonary infiltrate, elevation of a hemidiaphragm, and pleural effusion. Many patients with pulmonary embolism may have a normal chest radiograph. The chest radiograph is essential, however, for two purposes. First, it may identify another cause of the patient's symptoms, such as a rib fracture, dissecting aortic aneurysm, or pneumothorax. Second, a chest radiograph is essential to interpretation of the radionuclide V/Q scan. The perfusion scan accurately reflects the perfusion of the lung. However, a perfusion defect may result from a variety of etiologies. Any process such as vascular stenosis or compression by tumor may restrict blood flow. 84 references

  20. Pulmonary imaging

    International Nuclear Information System (INIS)

    Studies in pulmonary imaging predominantly depict the distribution of pulmonary perfusion or the ventilation-perfusion balance for the detection of pulmonary embolism, obstructive airway disease, and lung carcinoma. Much work has been done using 133Xe to study both regional ventilation and blood flow. This work has led to a greater understanding of regional blood flow and ventilation, of lung anatomy and morphometry, of the mechanical factors that influence lung function, and of the biochemical and other nonrespiratory functions of the lung. The recent development of emission tomography and the potential for receptor- and function-oriented radiopharmaceuticals have allowed several important regional physiologic processes to be measured. The physical parameters measured in radionuclide emission studies are the temporal and spatial relations of the distribution of tracer in the lungs. Tracers that are diffusible will wash into and out of the lungs at a rate proportional not only to flow but also to membrane permeability. If the radionuclide label is attached to microspheres or macro-aggregates, the amount lodging in any portion of the lung can be related to relative flow in the lung. Metabolism of the lung is reflected by the rate of extraction and disappearance of various labeled radiopharmaceuticals. The problems encountered in assessing the value of ventilation-perfusion scans for the diagnosis of pulmonary embolism are concerned not only with improving specificity, but also with better estimation of the prior probability of pulmonary embolism in the patient being evaluated

  1. Treatment of severe maxillary cleft hypoplasia in a case with missing premaxilla with anterior maxillary distraction using tooth-borne hyrax appliance

    Directory of Open Access Journals (Sweden)

    Akshai Shetty

    2015-01-01

    Full Text Available Cleft orthodontics generally poses a challenge and a missing premaxilla adds to the difficulty in managing them. The lack of bone support and anterior teeth in a case with missing premaxilla accounts not only for difficulty in rehabilitation but also in increasing the maxillary hypoplasia. This article presents a case report where planned orthodontic and surgical management using distraction has helped treat a severe maxillary hypoplasia in a patient with missing premaxilla. The treatment plan and method can be used to treat severe maxillary hypoplasia and yield reasonably acceptable results for such patients.

  2. Pulmonary histiocytosis

    Directory of Open Access Journals (Sweden)

    Milenković Branislava

    2013-01-01

    Full Text Available Introduction. Langerhans cell histiocytosis encompasses a group of disorders of unknown origin with different clinical presentations and outcomes. It is characterized by infiltration of the involved tissues by large numbers of Langerhans cells, often organized into granulomas. The accumulation of these cells causes the classic lytic bone lesions, skin rashes, lymphadenopathy, splenomegaly, and dysfunction of organ such as the pituitary gland, lungs, liver, and bone marrow. Pulmonary histiocytosis. Adult pulmonary Langerhans cell histiocytosis is a rare disorder of unknown etiology. It occurs predominantly in male smokers, with an incidence peak between 20 and 40 years of age. High-resolution computed tomography of the chest can show nodules, cavitated nodules, and thickand thin-walled cysts. The definite diagnosis of pulmonary Langerhans cell histiocytosis requires identification of Langerhans’ cell granulomas infiltrating and destroying distal bronchioles, which is usually achieved by lung biopsy at a site selected by chest high-resolution computed tomography. Treatment. Treatment options for adults have never been clarified by a clinical trial. The published literature provides minimal data on the comparative efficacy of various treatment options which include surgery/curettage, steroids, radiation, and various chemotherapy regimens. The improved understanding of the mechanisms involved in the pathogenesis of pulmonary Langerhans cell histiocytosis should help in the development of specific therapeutic strategies and effective treatment.

  3. MICrocephaly, disproportionate pontine and cerebellar hypoplasia syndrome: A clinico-radiologic phenotype linked to calcium/calmodulin-dependent serine protein kinase gene mutation

    Directory of Open Access Journals (Sweden)

    Rashid Saleem

    2013-01-01

    Full Text Available MICrocephaly, disproportionate pontine and cerebellar hypoplasia (MICPCH syndrome, a rare X-linked disorder, generally seen in girls, is characterized by neurodevelopmental delay, microcephaly, and disproportionate pontine and cerebellar hypoplasia. It is caused by inactivating calcium/calmodulin-dependent serine protein kinase (CASK gene mutations. We report a 2-year-old girl with severe neurodevelopmental delay, microcephaly, minimal pontine hypoplasia, cerebellar hypoplasia, and normal looking corpus callosum, with whom the conventional cytogenetic studies turned out to be normal, and an array-comparative genomic hybridization (a-CGH analysis showed CASK gene duplication at Xp11.4. Our case highlights the importance of using clinico-radiologic phenotype to guide genetic investigation and it also confirms the role of a-CGH analysis in establishing the genetic diagnosis of MICPCH syndrome, when conventional cytogenetic studies are inconclusive.

  4. Idiopathic abdominal cocoon syndrome with unilateral abdominal cryptorchidism and greater omentum hypoplasia in a young case of small bowel obstruction

    Science.gov (United States)

    Fei, Xiang; Yang, Hai-Rui; Yu, Peng-Fei; Sheng, Hai-Bo; Gu, Guo-Li

    2016-01-01

    Abdominal cocoon syndrome (ACS) is a rare cause of intestinal obstruction due to total or partial encapsulation of the small intestine by a fibrocollagenous membrane. Idiopathic ACS with abdominal cryptorchidism and greater omentum hypoplasia is even rarer clinically. We successfully treated a 26-year-old male case of small bowel obstruction with acute peritonitis. He was finally diagnosed with idiopathic ACS with unilateral abdominal cryptorchidism and greater omentum hypoplasia during exploratory laparotomy. He then underwent enterolysis, cryptorchidectomy, and appendectomy. He recovered gradually from the operations and early postoperative inflammatory ileus. There has been no recurrence of intestinal obstruction since the operation, and he is still in follow-up. We analyzed his clinical data and retrospectively reviewed the literature, and our findings may be helpful for the clinical diagnosis and treatment on ACS. PMID:27239122

  5. Thyroid Hypoplasia as a Cause of Congenital Hypothyroidism in Monozygotic Twins Concordant for Rubinstein-Taybi Syndrome.

    OpenAIRE

    Akın, Mustafa Ali; Güneş, Tamer; Akın, Leyla; Çoban, Dilek; Kara Oncu, Sena; Kiraz, Aslıhan; Kurtoğlu, Selim

    2011-01-01

    Rubinstein-Taybi syndrome (RSTS), a genetic disorder characterized by growth retardation, mental deficiency, dysmorphic face, broad thumbs and large toes, generally affects monozygotic twins concordantly. Thyroid hypoplasia (TH) is a common cause of congenital hypothyroidism (CH) and often accompanies dysmorphic syndromes. A pair of female twins were admitted to our neonatology unit 16 hours after delivery. They were born at 35 weeks of gestation. Both twins had an unusual dysmorphic facial a...

  6. Revisiting the craniosynostosis‐radial ray hypoplasia association: Baller‐Gerold syndrome caused by mutations in the RECQL4 gene

    OpenAIRE

    Van Maldergem, L; Siitonen, H. A; Jalkh, N.; Chouery, E; Roy, M.; Delague, V; Muenke, M; Jabs, E W; J. Cai; Wang, L. L.; Plon, S E; Fourneau, C.; Kestilä, M.; Gillerot, Y; Mégarbané, A

    2005-01-01

    Baller‐Gerold syndrome (BGS) is a rare autosomal recessive condition with radial aplasia/hypoplasia and craniosynostosis (OMIM 218600). Of >20 cases reported so far, a few appear atypical and have been reassigned to other nosologic entities, including Fanconi anaemia, Roberts SC phocomelia, and Pfeiffer syndromes after demonstration of corresponding cytogenetic or molecular abnormalities. Clinical overlap between BGS, Rothmund‐Thomson syndrome (RTS), and RAPADILINO syndrome is noticeable. Bec...

  7. CT volumetry of lumbar vertebral bodies in patients with hypoplasia L5 and bilateral spondylolysis and in normal controls

    Energy Technology Data Exchange (ETDEWEB)

    Wilms, Guido E.; Demaerel, Philippe; Keyzer, Frederik de [UZ Leuven, Campus Gasthuisberg, Department of Radiology, Leuven (Belgium); Willems, Endry [ZOL, Department of Radiology, Genk (Belgium)

    2012-08-15

    To examine the feasibility and results of calculating the volume of lumbar vertebral bodies in normal patients and patients with suspected hypoplasia of L5. Lumbar multi-detector CT was performed in 38 patients with bilateral spondylolysis and hypoplasia of L5 and in 38 normal patients. Lumbar vertebral body volume of L3, L4 and L5 was measured by CT volumetry with a semi-automated program, created with MeVisLab. In the control group, the average vertebral body volume (in cubic centimeters) of L3 was 35.93 ({+-}7.33), 36.34 ({+-}7.13) for L4 and 34.63 ({+-}6.88) for L5. In patients with suspected hypoplasia L5 the average body volume (in cubic centimeters) of L3 was 36.85 ({+-}7.37), 36.90 ({+-}6.99) for L4 and 33.14 ({+-}6.57) for L5. The difference in mean vertebral body volume for L3, L4 and L5 between both groups was statistically not significant. However, there was a statistically significant difference of the ratio L5/L4 (P < 0.001) between both groups: the mean ratio L5/L4 in the control group was 95.3 {+-} 3.9%, the ratio for the hypoplastic L5 group was 89.9 {+-} 6.3%. There was no significant difference in the vertebral body volume for L3, L4 and L5 between both groups due to inter-patient variability. However, the relation between the body volume of L5 and L4 is significantly different between both groups. The volume of the vertebral body of L5 proved to be on average 10.2% smaller than the volume of L4 in the group with hypoplasia L5 versus 4.7% in the control group. (orig.)

  8. Clinical distinction between nasal optic disc hypoplasia (NOH and glaucoma with NOH-like temporal visual field defects

    Directory of Open Access Journals (Sweden)

    Hiroshi Ohguro

    2010-06-01

    Full Text Available Hiroshi Ohguro, Ikuyo Ohguro, Midori Tsuruta, Maki Katai, Sachie TanakaDepartment of Ophthalmology, Sapporo Medical University School of Medicine, JapanPurpose: To report on the clinically important differences between nasal optic hypoplasia (NOH and glaucoma with NOH-like temporal visual field defect (VFD.Method: Five NOH (four bilateral and one unilateral patients, three unilateral NOH patients with glaucoma, and two glaucoma patients with NOH-like temporal VFD were clinically characterized. Superior segmental optic nerve hypoplasia was also associated with glaucoma in one eye of a bilateral NOH case and the NOH eye of a unilateral NOH patient. Ocular manifestations including refractive errors, size, and appearances of the optic discs, retinal nerve fiber thickness (NFLT ascertained by optical coherence tomography (OCT, and VFD were examined.Results: Ophthalmic examinations revealing NOH showed high myopia at more than -5.0D, a small disc with nasal double-ring appearance, significantly decreased NFLT by OCT, and retinal nerve fiber layer defect in the corresponding nasal sector. Stationary temporal VFD varied from a slight depression of the peripheral isopters to wide sector defects. In contrast, two glaucoma patients with NOH-like temporal VFD showed several different clinical features, including mild myopia less than -5D, a normal size with glaucomatous disc cupping; a slight decrease in nasal NFLT and progression of temporal and other glaucomatous VFD.Conclusion: Careful evaluation of optic disc appearance and measurement of NFLT using OCT may help to distinguish between NOH and glaucoma with NOH-like temporal VFD.Keywords: nasal optic disc hypoplasia, glaucoma, temporal visual field defects, optical coherence tomography, superior segmental optic nerve hypoplasia

  9. Genitopatellar syndrome: a new condition comprising absent patellae, scrotal hypoplasia, renal anomalies, facial dysmorphism, and mental retardation

    OpenAIRE

    Cormier-Daire, V.; Chauvet, M.; Lyonnet, S; Briard, M.; Munnich, A; Le Merrer, M

    2000-01-01

    We report on the association of absent patellae, genital and renal anomalies, dysmorphic features, and mental retardation in seven children (six boys and one girl) belonging to five unrelated families. Flexion deformities of the knees and hips with club feet and absent patellae were consistently observed and scrotal hypoplasia and cryptorchidism were present in all boys (6/6). Dysmorphic features included a coarse face, a large nose with a high nasal bridge, and microcephaly. Other features i...

  10. Association between enamel hypoplasia and dental caries in primary second molars and permanent first molars: A 3-year follow-up study

    OpenAIRE

    Sakeenabi Basha; Roshan Noor Mohamed; Hiremath Shivalinga Swamy

    2016-01-01

    Context: Enamel hypoplasia is a defect caused by disturbances during enamel formation. These defects in the enamel present important clinical significance as they predispose a tooth to dental caries. Aim: The aim of this study was to assess the longitudinal relationships between enamel hypoplasia and caries experience of primary second molars and permanent first molars. Materials and Methods: The study sample consisted of 765 subjects who underwent dental examinations at both ages 6 and 9 by ...

  11. Restoration of incisal half with edge-up technique using ceramic partial crown in turner′s hypoplasia: A case report

    OpenAIRE

    Shreya Hegde; Kundabala, M

    2014-01-01

    This case report describes a rare treatment modality for Turner's hypoplasia done with a very conservative approach for the esthetic and functional problem of the defect. Diagnosis was made as Turner's hypoplasia of upper two central incisors with proximal caries. Treatment planning was done after considering many factors such as conservation of tooth structure, esthetics, occlusion and economy. Tooth preparation was done to receive Edge-up, all ceramic partial crowns for both the upper centr...

  12. Anterior maxillary segmental distraction in the treatment of severe maxillary hypoplasia secondary to cleft lip and palate

    Science.gov (United States)

    Li, Hongliang; Dai, Jiewen; Si, Jiawen; Zhang, Jianfei; Wang, Minjiao; Shen, Steve Guofang; Yu, Hongbo

    2015-01-01

    Anterior maxillary segmental distraction (AMSD) is an effective surgical procedure in the treatment of maxillary hypoplasia secondary to cleft lip and palate. Its unique advantage of preserving velopharyngeal function makes this procedure widely applied. In this study, the application of AMSD was described and its long-term stability was explored. Eight patients with severe maxillary hypoplasia secondary to CLP were included in this study. They were treated with AMSD using rigid external distraction (RED) device. Cephalometric analysis was performed twice at three time points for evaluation: before surgery (T1), after distraction (T2), and 2 years after treatment (T3). One-way analysis of variance was used to assess the differences statistically. All the distractions completed smoothly, and maxilla was distracted efficiently. The value of SNA, NA-FH, Ptm-A, U1-PP, overjet and PP (ANS-PNS) increased significantly after the AMSD procedure (P 0.05). Changes of palatopharyngeal depth and soft palatal length were insignificant. AMSD with RED device provided an effective way to correct maxillary hypoplasia secondary to CLP, extended the palatal and arch length, avoided damage on velopharyngeal closure function and reduced the relapse rate. It is a promising and valuable technique in this potentially complicated procedure. PMID:26629107

  13. Early Developmental Perturbations in a Human Stem Cell Model of MODY5/HNF1B Pancreatic Hypoplasia

    Directory of Open Access Journals (Sweden)

    Adrian Kee Keong Teo

    2016-03-01

    Full Text Available Patients with an HNF1BS148L/+ mutation (MODY5 typically exhibit pancreatic hypoplasia. However, the molecular mechanisms are unknown due to inaccessibility of patient material and because mouse models do not fully recapitulate MODY5. Here, we differentiated MODY5 human-induced pluripotent stem cells (hiPSCs into pancreatic progenitors, and show that the HNF1BS148L/+ mutation causes a compensatory increase in several pancreatic transcription factors, and surprisingly, a decrease in PAX6 pancreatic gene expression. The lack of suppression of PDX1, PTF1A, GATA4, and GATA6 indicates that MODY5-mediated pancreatic hypoplasia is mechanistically independent. Overexpression studies demonstrate that a compensatory increase in PDX1 gene expression is due to mutant HNF1BS148L/+ but not wild-type HNF1B or HNF1A. Furthermore, HNF1B does not appear to directly regulate PAX6 gene expression necessary for glucose tolerance. Our results demonstrate compensatory mechanisms in the pancreatic transcription factor network due to mutant HNF1BS148L/+ protein. Thus, patients typically develop MODY5 but not neonatal diabetes despite exhibiting pancreatic hypoplasia.

  14. Detection of increased frequency of thyroid hypoplasia in subjects irradiated in utero as the results of Chernobyl catastrophe

    Energy Technology Data Exchange (ETDEWEB)

    Drozd, V.; Danilova, L.; Lushchyk, M.; Leonova, T.; Platonova, T. [International Fund Arnica, Minsk (Belarus); Grigorovich, A.; Sivuda, V. [Brest Regional Endocrinological Dispensary, Brest (Belarus); Branovan, I. [Chernobyl Project, New-York (United States); Biko, I.; Reiners, C. [Clinic and Policlinic of Nuclear Medicine, University of Wurzburg, Wursburg (Germany)

    2012-07-01

    For the 24 years passed after the Chernobyl catastrophe a significant experience in estimation of medical consequences of thyroid irradiation among Belarus patients had been accumulated. The aim of our screening of ultrasonic examination was the detection of the thyroid hypoplasia prevalence in the regions affected with radionuclide fallout. Since 2004 to 2007 thyroid ultrasound with volume estimation was performed in 3311 Belarus subjects, living on the areas of Brest region with the different contamination rate density. Examined subjects were divided in 3 groups: 1) irradiated at the age of 1 to 3 years old at the moment of Chernobyl catastrophe, 2) irradiated in utero, and 3) born after the catastrophe. It was revealed that thyroid hypoplasia was detected in 3% of group 1 (out of 1876 persons), in 5, 8% of group 2 (out of 503 persons, P<0.05) and in 1, 7% of the third group (out of 932 persons). The separation of the irradiated in utero subjects (group 2) to subgroups in dependence of the gestation period, showed the highest prevalence of thyroid hypoplasia among the irradiated in the first trimester of gestation: 7, 7% (P<0.05), in the second trimester: 5, 3%, in the third trimester: 4, 7%

  15. Detection of increased frequency of thyroid hypoplasia in subjects irradiated in utero as the results of Chernobyl catastrophe

    International Nuclear Information System (INIS)

    For the 24 years passed after the Chernobyl catastrophe a significant experience in estimation of medical consequences of thyroid irradiation among Belarus patients had been accumulated. The aim of our screening of ultrasonic examination was the detection of the thyroid hypoplasia prevalence in the regions affected with radionuclide fallout. Since 2004 to 2007 thyroid ultrasound with volume estimation was performed in 3311 Belarus subjects, living on the areas of Brest region with the different contamination rate density. Examined subjects were divided in 3 groups: 1) irradiated at the age of 1 to 3 years old at the moment of Chernobyl catastrophe, 2) irradiated in utero, and 3) born after the catastrophe. It was revealed that thyroid hypoplasia was detected in 3% of group 1 (out of 1876 persons), in 5, 8% of group 2 (out of 503 persons, P<0.05) and in 1, 7% of the third group (out of 932 persons). The separation of the irradiated in utero subjects (group 2) to subgroups in dependence of the gestation period, showed the highest prevalence of thyroid hypoplasia among the irradiated in the first trimester of gestation: 7, 7% (P<0.05), in the second trimester: 5, 3%, in the third trimester: 4, 7%

  16. Iron as the Key Modulator of Hepcidin Expression in Erythroid Antibody-Mediated Hypoplasia

    Directory of Open Access Journals (Sweden)

    J. C. Fernandes

    2014-01-01

    Full Text Available Erythroid hypoplasia (EH is a rare complication associated with recombinant human erythropoietin (rHuEPO therapies, due to development of anti-rHuEPO antibodies; however, the underlying mechanisms remain poorly clarified. Our aim was to manage a rat model of antibody-mediated EH induced by rHuEPO and study the impact on iron metabolism and erythropoiesis. Wistar rats treated during 9 weeks with a high rHuEPO dose (200 IU developed EH, as shown by anemia, reduced erythroblasts, reticulocytopenia, and plasmatic anti-rHuEPO antibodies. Serum iron was increased and associated with mRNA overexpression of hepatic hepcidin and other iron regulatory mediators and downregulation of matriptase-2; overexpression of divalent metal transporter 1 and ferroportin was observed in duodenum and liver. Decreased EPO expression was observed in kidney and liver, while EPO receptor was overexpressed in liver. Endogenous EPO levels were normal, suggesting that anti-rHuEPO antibodies blunted EPO function. Our results suggest that anti-rHuEPO antibodies inhibit erythropoiesis causing anemia. This leads to a serum iron increase, which seems to stimulate hepcidin expression despite no evidence of inflammation, thus suggesting iron as the key modulator of hepcidin synthesis. These findings might contribute to improving new therapeutic strategies against rHuEPO resistance and/or development of antibody-mediated EH in patients under rHuEPO therapy.

  17. Enamel hypoplasias and physiological stress in the Sima de los Huesos Middle Pleistocene hominins.

    Science.gov (United States)

    Cunha, E; Rozzi, F Ramirez; Bermúdez de Castro, J M; Martinón-Torres, M; Wasterlain, S N; Sarmiento, S

    2004-11-01

    This study presents an analysis of linear enamel hypoplasias (LEH) and plane-form defects (PFD) in the hominine dental sample from the Sima de los Huesos (SH) Middle Pleistocene site in Atapuerca (Spain). The SH sample comprises 475 teeth, 467 permanent and 8 deciduous, belonging to a minimum of 28 individuals. The method for recording PFD and LEH is discussed, as well as the definition of LEH. The prevalence of LEH and PFD in SH permanent dentition (unilateral total count) is 4.6% (13/280). Only one deciduous tooth (lower dc) showed an enamel disruption. Prevalence by individual ranges from 18.7-30%. The most likely explanation for the relatively low LEH and PFD prevalence in the SH sample suggests that the SH population exhibited a low level of developmental stress. The age at occurrence of LEH and PFD was determined by counting the number of perikymata between each lesion and the cervix of the tooth. Assuming a periodicity of nine days for the incremental lines, the majority of LEH in the SH sample occurred during the third year of life and may be related to the metabolic stress associated with weaning. PMID:15386251

  18. Hypoplasia of the sphenoid sinuses as a diagnostic tool in cystic fibrosis

    International Nuclear Information System (INIS)

    Purpose: To measure and compare the size of the sphenoid sinuses in patients with cystic fibrosis (CF) to patients with inflammatory sinonasal disease, and to correlate the size with number of CF mutations in each patient. Material and Methods: Ninety-six CF patients aged 5-47 years (median 19 years) and 130 control patients aged 7-51 years (median 32 years) were examined using coronal CT of the paranasal sinuses. In each patient, the CT image with the largest coronal area of the sphenoid sinuses was scanned into a Macintosh computer with image processing and analysis software. Largest coronal area and largest circumference of the right and left sphenoid sinuses were automatically measured. Additionally, antero-posterior extension of the sphenoid sinuses was calculated from the lateral scanograms. CF patients were grouped according to number of confirmed mutations (CF-0, CF-1, or CF-2). Results: CF patients generally had small sphenoid sinuses. The largest difference for all parameters were observed between the CF-2 and the control groups (p<0.0001). No CF-2 patient had pneumatization beyond the presphenoid. The CF-0 and CF-1 groups consisted of two populations, one overlapping the CF-2 group and another overlapping the control group. Conclusion: Hypoplasia of the sphenoid sinuses is a characteristic finding in CF patients. When pneumatization of the basisphenoid is present, the existing CF diagnosis should be questioned. (orig.)

  19. Clinical and immunologic outcome of patients with cartilage hair hypoplasia after hematopoietic stem cell transplantation.

    Science.gov (United States)

    Bordon, Victoria; Gennery, Andrew R; Slatter, Mary A; Vandecruys, Els; Laureys, Genevieve; Veys, Paul; Qasim, Waseem; Waseem, Qasim; Friedrich, Wilhelm; Wulfraat, Nico M; Scherer, Franziska; Cant, Andrew J; Fischer, Alain; Cavazzana-Calvo, Marina; Cavazanna-Calvo, Marina; Bredius, Robbert G M; Notarangelo, Luigi D; Mazzolari, Evelina; Neven, Benedicte; Güngör, Tayfun; Tayfun, Güngör

    2010-07-01

    Cartilage-hair hypoplasia (CHH) is a rare autosomal recessive disease caused by mutations in the RMRP gene. Beside dwarfism, CHH has a wide spectrum of clinical manifestations including variable grades of combined immunodeficiency, autoimmune complications, and malignancies. Previous reports in single CHH patients with significant immunodeficiencies have demonstrated that allogeneic hematopoietic stem cell transplantation (HSCT) is an effective treatment for the severe immunodeficiency, while growth failure remains unaffected. Because long-term experience in larger cohorts of CHH patients after HSCT is currently unreported, we performed a European collaborative survey reporting on 16 patients with CHH and immunodeficiency who underwent HSCT. Immune dysregulation, lymphoid malignancy, and autoimmunity were important features in this cohort. Thirteen patients were transplanted in early childhood ( approximately 2.5 years). The other 3 patients were transplanted at adolescent age. Of 16 patients, 10 (62.5%) were long-term survivors, with a median follow-up of 7 years. T-lymphocyte numbers and function have normalized, and autoimmunity has resolved in all survivors. HSCT should be considered in CHH patients with severe immunodeficiency/autoimmunity, before the development of severe infections, major organ damage, or malignancy might jeopardize the outcome of HSCT and the quality of life in these patients. PMID:20375313

  20. Adrenal hypoplasia congenita: a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism

    Directory of Open Access Journals (Sweden)

    Marta Loureiro

    2015-09-01

    Full Text Available Primary adrenal insufficiency is defined by the impaired synthesis of adrenocortical hormones due to an intrinsic disease of the adrenal cortex. Determining its etiology is crucial to allow adequate long-term management and genetic counseling. We report the case of a male adolescent that presented in the neonatal period with adrenal crisis and received replacement therapy for primary adrenal insufficiency. During follow-up, adrenal hypoplasia congenita (AHC was suspected given his persistently raised adrenocorticotropic hormone levels, with markedly low 17-OH progesterone and androstenedione levels. DNA sequence analysis revealed a mutation in NR0B1 gene (c.1292delG, confirming the diagnosis. Delayed puberty and persistent low levels of gonadotropins led to testosterone replacement therapy. X-linked AHC is a rare cause of primary adrenal insufficiency and hypogonadotropic hypogonadism, related to mutations in NR0B1 gene. Despite its rarity, AHC should be considered in patients who present with primary adrenal failure, low levels of 17-OH progesterone and hypogonadotropic hypogonadism.

  1. Pulmonary aspergillosis.

    Science.gov (United States)

    Chabi, M L; Goracci, A; Roche, N; Paugam, A; Lupo, A; Revel, M P

    2015-05-01

    Aspergillosis is a mycotic disease usually caused by Aspergillus fumigatus, a saprophytic and ubiquitous airborne fungus. Aspergillus-related lung diseases are traditionally classified into four different forms, whose occurrence depends on the immunologic status of the host and the existence of an underlying lung disease. Allergic broncho-pulmonary aspergillosis (ABPA) affects patients with asthma or cystic fibrosis. Saprophytic infection (aspergilloma) occurs in patients with abnormal airways (chronic obstructive pulmonary disease, bronchiectasis, cystic fibrosis) or chronic lung cavities. Chronic necrotizing aspergillosis (semi-invasive form) is described in patients with chronic lung pathology or mild immunodeficiency. Invasive aspergillosis (angio-invasive or broncho-invasive forms) occurs in severely immuno-compromised patients. Knowledge of the various radiological patterns for each form, as well as the corresponding associated immune disorders and/or underlying lung diseases, helps early recognition and accurate diagnosis. PMID:25753544

  2. Pulmonary Hypertension

    OpenAIRE

    Newman, John H.

    2005-01-01

    The modern era in cardiopulmonary medicine began in the 1940s, when Cournand and Richards pioneered right-heart catheterization. Until that time, no direct measurement of central vascular pressure had been performed in humans. Right-heart catheterization ignited an explosion of insights into function and dysfunction of the pulmonary circulation, cardiac performance, ventilation–perfusion relationships, lung–heart interactions, valvular function, and congenital heart disease. It marked the beg...

  3. What Causes Pulmonary Hypertension?

    Science.gov (United States)

    ... from the NHLBI on Twitter. What Causes Pulmonary Hypertension? Pulmonary hypertension (PH) begins with inflammation and changes in the ... different types of PH. Group 1 pulmonary arterial hypertension (PAH) may have no known cause, or the ...

  4. Pulmonary Arterial Hypertension

    Science.gov (United States)

    Pulmonary Arterial Hypertension What Is Pulmonary Hypertension? To understand pulmonary hypertension (PH) it helps to understand how blood ows throughout your body. While the heart is one organ, it ...

  5. Pulmonary manifestations of malaria

    International Nuclear Information System (INIS)

    We report on the two different types of pulmonary manifestations in acute plasmodium falciparum malaria. The more severe variant shows long standing interstitial pulmonary infiltrates, whereas in the more benign courses only short-term pulmonary edemas are visible. (orig.)

  6. Pulmonary Fibrosis Foundation

    Science.gov (United States)

    ... most current news and updates from the Pulmonary Fibrosis Foundation. Life with PF Education & Support About PF ... LEARN MORE We Imagine a World Without Pulmonary Fibrosis The Pulmonary Fibrosis Foundation mobilizes people and resources ...

  7. Pulmonary manifestations of malaria

    Energy Technology Data Exchange (ETDEWEB)

    Rauber, K.; Enkerlin, H.L.; Riemann, H.; Schoeppe, W.

    1987-05-01

    We report on the two different types of pulmonary manifestations in acute plasmodium falciparum malaria. The more severe variant shows long standing interstitial pulmonary infiltrates, whereas in the more benign courses only short-term pulmonary edemas are visible.

  8. Idiopathic Pulmonary Fibrosis

    Science.gov (United States)

    ... Explore Idiopathic Pulmonary Fibrosis What Is... How the Lungs Work Other Names Causes Signs & Symptoms Diagnosis Treatments Living With Clinical Trials Links Related Topics How the Lungs Work Lung Transplant Pulmonary Hypertension Pulmonary Rehabilitation Respiratory Failure ...

  9. How Is Pulmonary Hypertension Treated?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is Pulmonary Hypertension Treated? Pulmonary hypertension (PH) has no cure. However, ... Types of Pulmonary Hypertension." ) Group 1 Pulmonary Arterial Hypertension Group 1 pulmonary arterial hypertension (PAH) includes PH ...

  10. Ophthalmologic manifestations of focal dermal hypoplasia (Goltz syndrome): A case series of 18 patients.

    Science.gov (United States)

    Gisseman, Jordan D; Herce, Honey H

    2016-03-01

    Focal Dermal Hypoplasia (FDH) or Goltz syndrome is a rare multi-system disorder with cutaneous, ocular, dental, and skeletal anomalies due to dysplasia of mesoectodermal derived tissues. It is an X-linked inheritance syndrome caused by mutations in the PORCN gene. This study is aimed to investigate the ocular findings in patients with Goltz syndrome. To date, there have been a limited number of case reports on the ocular manifestations of FDH. This is a prospective, non-consecutive, observational case series. Prospective ophthalmologic evaluation was performed on 18 patients with confirmed genetic testing for FDH, Goltz Syndrome, as a component of a larger multi-subspecialty study to better characterize the findings of this condition. Special attention was placed on evaluating the incidence of anophthalmia, microphthalmia, colobomas (iris, optic nerve, and/or retinal), cataracts, nystagmus, and strabismus. A complete ophthalmologic exam was done on all the patients. The mean patient age was 12.8 years (1-55 years). Eighty-nine percent were female and 77% (14/18) of patients had some form of an ophthalmologic manifestation of the condition. Ophthalmological findings included chorioretinal colobomas (61%), iris colobomas (50%), microphthalmia (44%), anophthalmia (11%), cataracts (11%), and conjunctival and eyelid papillomas (5%). Nystagmus was present in 33% and strabismus in 22% of the patients. Visual acuity ranged from 20/20 to no light perception. This study demonstrates a higher incidence of ophthalmologic manifestations as previously reported (77% vs. 40%). To our knowledge, this is the largest case series reported in the literature with 18 patients. © 2016 Wiley Periodicals, Inc. PMID:27001926

  11. Pulmonary Hypertension in Pulmonary Langerhans Cell Granulomatosis

    OpenAIRE

    Matthias Held; Philipp Schnabel; Arne Warth; Berthold Jany

    2012-01-01

    Introduction. Pulmonary Langerhans cell granulomatosis is a rare disease with a variable course. In pulmonary Langerhans cell granulomatosis pulmonary hypertension is frequent and has an independent prognostic impact. A vasculopathy which ist not related to ventilatory disturbance and fibrosis has been identified. An arteriopathy and even a venulopathy have been described. Due to this possible venulopathy vasodilators carry a significant risk for pulmonary congestion and edema. No drugs have ...

  12. [Pulmonary embolism].

    Science.gov (United States)

    Söffker, Gerold; Kluge, Stefan

    2015-01-01

    Acute pulmonary embolism is an important differential diagnosis of acute chest pain. The clinical signs are often non-specific. However, diagnosis and therapy must be done quickly in order to reduce morbidity and mortality. The new (2014) European guidelines for acute pulmonary embolism (PE) focus on risk-adapted diagnostic algorithms and prognosis adapted therapy concepts. According to the hemodynamic presentation the division in a high-risk group (unstable patient with persistent hypotension or shock) or in non-high-risk groups (hemodynamically stable) was proposed. In the high-risk group the immediate diagnosis is usually done by multidetector spiral computed tomography (MDCT) and primarily the medical therapy of right ventricular dysfunction and thrombolysis is recommended.In the non-high-risk group, this is subdivided into an intermediate-risk group and low-risk group, the diagnosis algorithm based on the PE-pretest probability--determined by validated scores. Moreover, the diagnosis is usually secured by MDCT--the new gold standard in the PE-diagnosis, scores, or it can be primarily ruled out due to the high negative predictive value of D-dimer determination. To improve the prognostic risk stratification in non-high-risk group patients the additional detection of right ventricular dysfunction (MDCT, echocardiography), cardiac biomarkers (troponin, NT proBNP) and validated scores (e.g. Pulmonary Embolism Severity Index) is recommended. Therefore, the intermediate-risk group can be further subdivided. For treatment of non-high-risk group patients, the initial anticoagulation (except those with severe renal insufficiency) using low molecular weight heparin/fondaparinux and conversion to vitamin-K antagonists or alternatively with direct oral anticoagulants (DOAK) is recommended. Hemodynamically stable patients with right ventricular dysfunction and myocardial ischemia (Intermediate-high-risk group patients) but with clinically progressive hemodynamic

  13. Contrast-enhanced MR angiography of pulmonary venous abnormalities in children

    International Nuclear Information System (INIS)

    Echocardiography and X-ray angiography have been considered as gold standards for evaluation of pulmonary venous abnormalities. However, each technique has its own limitations, such as limitation in visualization of the pulmonary veins within the lungs by echocardiography, and the invasive nature of and use of ionizing radiation in X-ray angiography. Contrast-enhanced MR angiography (MRA) is a fast noninvasive method of visualization of the vessels including the pulmonary arteries and veins. To evaluate the utility of contrast-enhanced MRA in the evaluation of pulmonary venous abnormalities in pediatric patients and to compare its diagnostic accuracy with that of transthoracic echocardiography. In 30 pediatric patients 31 contrast-enhanced MRA studies were performed for evaluation of pulmonary venous abnormalities. Each of 124 pulmonary veins was evaluated for site of connection, course within the lung, presence of obstruction, and topographic relationship with the adjacent structures. The findings of MRA were compared with echocardiographic findings for 116 veins in 29 studies in 28 patients. Contrast-enhanced MRA visualized 99% (123 of 124) of the pulmonary veins investigated, while echocardiography visualized 89% (103 of 116). Exact agreement was found between the two methods in 72% of the veins with a weighted kappa of 0.60 (0.47-0.73, 95% CI). Echocardiography failed to diagnose an abnormal connection in 2 of 15 pulmonary veins, a discrete stenosis in 2 of 19 veins, and diffuse hypoplasia in 10 of 14 veins. In 29% of patients, MRA made the uncertain echocardiographic findings clear. In another 29%, MRA provided a new diagnosis. Contrast-enhanced MRA is a powerful, safe, and accurate fast-imaging technique for the anatomical evaluation of pulmonary venous abnormalities. MRA may obviate the need for conventional X-ray angiography. Cardiac catheterization may be reserved for those patients in whom pulmonary vascular resistance needs to be determined. (orig.)

  14. Moebius syndrome with macular hyperpigmentation, skeletal anomalies, and hypoplasia of pectoralis major muscle in an Egyptian child

    Directory of Open Access Journals (Sweden)

    Rabah M. Shawky

    2015-07-01

    Full Text Available We report a 4 month old female infant, 3rd in order of birth of the first cousin consanguineous parents. The patient has congenital right facial nerve palsy, with asymmetry of facial expression during crying and difficulty in swallowing. Associated anomalies include abnormal facial features, bilateral finger anomalies, bilateral talipes equinovarus, kyphoscoliosis, hypotonia, high frequency hearing loss. Bilateral macular hyperpigmentation was detected in our patient on fundus examination which was not reported previously in Moebius syndrome cases. In addition there is hypoplasia of the right pectoralis major muscle.

  15. [Pulmonary circulation in embolic pulmonary edema].

    Science.gov (United States)

    Sanotskaia, N V; Polikarpov, V V; Matsievskiĭ, D D

    1989-02-01

    The ultrasonic method was used in acute experiments on cats with open chest under artificial lung ventilation to obtain blood flow in low-lobar pulmonary artery and vein, the blood pressure in pulmonary artery, as well as the left atrial pressure in fat (olive oil) and mechanical (Lycopodium spores) pulmonary embolism. It is shown that pulmonary embolism produces the decrease in the blood flow in pulmonary artery and vein, the increase of the pressure in pulmonary artery and left atria, the increase of lung vessels resistance. The decrease is observed of systemic arterial pressure, bradycardia, and extrasystole. After 5-10 min the restoration of arterial pressure and heart rhythm occur and partial restoration of blood flow in pulmonary artery and vein. In many experiments the blood flow in vein outdoes that in the artery--it allows to suppose the increase of the blood flow in bronchial artery. After 60-90 min there occur sudden decrease of systemic arterial pressure, the decrease of the blood flow in pulmonary artery and vein. The pressure in pulmonary artery and resistance of pulmonary vessels remain high. Pulmonary edema developed in all animals. The death occurs in 60-100 min after the beginning of embolism. PMID:2923969

  16. Gene expression signature of cerebellar hypoplasia in a mouse model of Down syndrome during postnatal development

    Directory of Open Access Journals (Sweden)

    Vitalis Tania

    2009-03-01

    population that is thought responsible for the cerebellar hypoplasia in Down syndrome, a global destabilization of gene expression was not detected. Altogether these results strongly suggest that the three-copy genes are directly responsible for the phenotype present in cerebellum. We provide here a short list of candidate genes.

  17. Apelin and pulmonary hypertension

    DEFF Research Database (Denmark)

    Andersen, Charlotte Uggerhøj; Hilberg, Ole; Mellemkjær, Søren;

    2011-01-01

    Pulmonary arterial hypertension (PAH) is a devastating disease characterized by pulmonary vasoconstriction, pulmonary arterial remodeling, abnormal angiogenesis and impaired right ventricular function. Despite progress in pharmacological therapy, there is still no cure for PAH. The peptide apelin...... vasoconstriction, and has positive inotropic and cardioprotective effects. Apelin attenuates vasoconstriction in isolated rat pulmonary arteries, and chronic treatment with apelin attenuates the development of pulmonary hypertension in animal models. The existing literature thus renders APLNR an interesting...

  18. Pulmonary veno-occlusive disease

    Science.gov (United States)

    Pulmonary vaso-occlusive disease ... common among children and young adults. As the disease gets worse, it causes narrowed pulmonary veins, pulmonary artery hypertension , and congestion and swelling ...

  19. Giant infantile pulmonary hemangioma

    International Nuclear Information System (INIS)

    We present a very unusual case of giant infantile pulmonary hemangioma presenting as a large solitary pulmonary mass. This was successfully managed with surgical resection. Histological examination revealed that the mass was positive for GLUT-1 receptor, a marker for infantile hemangioma. To our knowledge only a few cases of pulmonary hemangioma have been described previously in the literature. Pulmonary hemangiomas are very rare lesions, most of them presenting as a pulmonary mass. This case emphasizes the fact that this rare lesion should be considered in the differential of an enhancing pulmonary mass in an infant. (orig.)

  20. Pulmonary function in advanced pulmonary hypertension.

    OpenAIRE

    Burke, C. M.; Glanville, A R; Morris, A J; Rubin, D.; Harvey, J. A.; Theodore, J; Robin, E. D.

    1987-01-01

    Pulmonary mechanical function and gas exchange were studied in 33 patients with advanced pulmonary vascular disease, resulting from primary pulmonary hypertension in 18 cases and from Eisenmenger physiology in 15 cases. Evidence of airway obstruction was found in most patients. In addition, mean total lung capacity (TLC) was only 81.5% of predicted and 27% of our subjects had values of TLC less than one standard deviation below the mean predicted value. The mean value for transfer factor (TLC...

  1. Peran kalsium sebagai prevensi terjadinya hipoplasia enamel (The role of calcium on enamel hypoplasia prevention

    Directory of Open Access Journals (Sweden)

    Soegeng Wahluyo

    2013-09-01

    Full Text Available Background: Fluoride is a trace element found in many natural and commonly consumed by humans in the form of fluoride salts such as Sodium Fluoride (NaF. The impacts that are most often caused by the intake of fluoride is a damage of enamel tooth/enamel hypoplasi or fluorosis. The manifestations of these effects are defects in teeth with whitish colour, brown to black colour effected on the aesthetic. So that the prevention of fluorosis is required. Purpose: The aim of this study was to analyze the effect of calcium as prevention against tooth enamel fluorosis in Wistar rats caused by exposure to fluoride through indicators of Amelogenin, Calbindin- 28kDa protein expression, the matrix of tooth enamel density and distance between ameloblast cell. Methods: This was an experimental studies, post-test only control group design. This study used three groups of rats. Group 1 (Control was induced by sterile destilled water, group 2 (treatment 1 was induced by fluoride and group 3 (treatment 2 was induced by combination of fluoride and calcium. Each induction was done through sonde for 28 days. results: The results showed that the induction of fluoride causes the increased expression of Amelogenin protein; decreased expression of Calbindin-28kDa protein; a decrease in the density of the enamel matrix and widen the distance between cells ameloblast, while the result of the combination induced by fluoride and calcium showed increased protein expression of Calbindin-28kDa and increased density of the enamel matrix. Conclusion: Calcium can be used as an alternative preventive against the occurrence of enamel hypoplasia due to exposure of fluoride in Wistar rats.latar belakang: Fluorida adalah salah satu trace element yang ada dialam dan sering dikonsumsi manusia dalam bentuk garam fluorida yaitu sodium fluoride (NaF. Paparan fluorida biasanya berkaitan dengan asupan fluorida yang dapat membahayakan enamel gigi yaitu terjadinya hipoplasia enamel atau

  2. Quantitative pulmonary perfusion imaging at 3.0 T of 2-year-old children after congenital diaphragmatic hernia repair: initial results

    International Nuclear Information System (INIS)

    To investigate whether dynamic contrast-enhanced MR imaging of the lung following congenital diaphragmatic hernia repair is feasible at 3.0 T in 2-year-old children and whether associated lung hypoplasia (reflected in reduced pulmonary microcirculation) can be demonstrated in MRI. Twelve children with a mean age 2.0 ± 0.2 years after hernia repair underwent DCE-MRI at 3.0 T using a time-resolved angiography with stochastic trajectories sequence. Quantification of lung perfusion was performed using a pixel-by-pixel deconvolution approach. Six regions of interest were placed (upper, middle and lower parts of right and left lung) to assess differences in pulmonary blood flow (PBF), pulmonary blood volume (PBV) and mean transit time (MTT) while avoiding the inclusion of larger pulmonary arteries and veins. The difference in PBF and PBV between ipsilateral and contralateral lung was significant (P < 0.5). No significant differences could be detected for the MTT (P = 0.5). DCE-MRI in 2-year-old patients is feasible at 3.0 T. Reduced perfusion in the ipsilateral lung is reflected by significantly lower PBF values compared with the contralateral lung. DCE-MRI of the lung in congenital diaphragmatic hernia can help to characterise lung hypoplasia initially and in the long-term follow-up of children after diaphragmatic repair. (orig.)

  3. Pulmonary Hyalinising Granuloma: A rare pulmonary disorder

    International Nuclear Information System (INIS)

    Pulmonary hyalinising granulomas are rare, non-infectious fibros lesions of the lung which can mimic metastatic disease. It was first described in literature by Engleman et al in the year 1977. Its etiology is unknown but they may be caused by an exaggerated immune response. The patient typically presents with cough, chest pain, dyspnoea or haemoptysis in association with multiple bilateral parenchymal nodules. We report the case of a 20 years old male who presented with a 12-month history of worsening dry cough. His plain chest radiograph and subsequent CT scan revealed bilateral pulmonary nodules. A CT guided biopsy of the pulmonary lesions was consistent with Pulmonary Hyalinising Granuloma [PHG].

  4. Pulmonary arteriovenous fistula

    Science.gov (United States)

    Pulmonary arteriovenous fistula is an abnormal connection between an artery and vein in the lungs. As a result, blood passes ... Pulmonary arteriovenous fistulas are usually the result of abnormal development of the blood vessels of the lung. Most occur in ...

  5. Pulmonary alveolar proteinosis

    Science.gov (United States)

    Alveolar proteinosis; Pulmonary alveolar phospholipoproteinosis ... In some cases, the cause of pulmonary alveolar proteinosis is unknown. In others, it occurs with lung infection or an immune problem. It also can occur with cancers of the blood system, ...

  6. Hantavirus Pulmonary Syndrome (HPS)

    Science.gov (United States)

    ... this page: About CDC.gov . Hantavirus Share Compartir Hantavirus Pulmonary Syndrome (HPS) Severe HPS. Image courtesy D. ... the workers showed evidence of infection or illness. Hantavirus Pulmonary Syndrome (HPS) Topics Transmission Where HPS is ...

  7. Extensive pulmonary alveolar microlithiasis

    OpenAIRE

    Rittayamai, Nuttapol; Muangman, Nisa; Ruangchira-Urai, Ruchira

    2013-01-01

    Pulmonary alveolar microlithiasis is a rare pulmonary disorder that is caused by abnormal sodium-dependent phosphate co-transporter from the mutation of SLC34A2 gene, leading to accumulation of microliths in the alveoli. We report the extensive pulmonary alveolar microlithiasis in an elderly woman who presented with progressive dyspnea for 2 months. Chest radiograph revealed diffuse pulmonary calcification. Tissue histopathology from open lung biopsy demonstrated widespread intra-alveolar lam...

  8. The pulmonary extracellular lining.

    OpenAIRE

    George, G; Hook, G E

    1984-01-01

    The extracellular lining of the lungs is reviewed. The pulmonary extracellular lining is a complex mixture of phospholipids, proteins and carbohydrates which is absolutely essential for the maintenance of normal pulmonary functions such as gas exchange. Without the lining the lungs would collapse. Alterations in the pulmonary extracellular lining may underlie some disease conditions induced by toxic agents, especially those which interfere with the formation of pulmonary surfactant. The extra...

  9. Symptoms, Diagnosis and Treatment for Pulmonary Fibrosis

    Science.gov (United States)

    ... www.lung.org > Lung Health and Diseases > Lung Disease Lookup > Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, Causes & Risk Factors Pulmonary ... while processing XML file."); } }); } } --> Blank Section Header Lung Disease Lookup Pulmonary Fibrosis Learn About Pulmonary Fibrosis Pulmonary Fibrosis Symptoms, ...

  10. Cerebellar and brainstem hypoplasia in a child with a partial monosomy for the short arm of chromosome 5 and partial trisomy for the short arm of chromosome 10

    NARCIS (Netherlands)

    Arts, W F M; Hofstee, Y; Drejer, G F; Beverstock, G C; Oosterwijk, J C

    1995-01-01

    A child with hypoplasia of the cerebellum and brainstem in association with an unbalanced translocation, resulting in a partial deletion of the short arm of chromosome 5 and a partial trisomy of the short arm of chromosome 10, is described. A balanced translocation was present in his mother and mate

  11. Idiopathic pulmonary hemosiderosis

    OpenAIRE

    Sunilkumar, B M; Sathishkumar, K M; Somashekhar, A R; P.P. Maiya

    2010-01-01

    Recurrent or chronic pulmonary hemorrhage is rare in children. Idiopathic pulmonary hemosiderosis (IPH) manifests as hemoptysis, diffuse parenchymal infiltrates on chest radiographs and microcytic hypochromic anemia. The hemoptysis present may be mistaken for more common diseases, delaying the diagnosis and further management. Idiopathic pulmonary hemosiderosis is a disorder of unknown etiology. Treatment of IPH includes immunosuppressive drugs along with supportive measures.

  12. Esthetic and endosurgical management of turner′s hypoplasia; a sequlae of trauma to developing tooth germ

    Directory of Open Access Journals (Sweden)

    Bhushan B

    2008-12-01

    Full Text Available Turner′s hypoplasia usually manifests as a portion of missing or diminished enamel, generally affecting one or more permanent teeth in the oral cavity. A case report of 8 year old girl who met with trauma at 2 years of age leading to primary incisors being knocked out, reported after 6 years with complaint of pain and discharge in her anterior malformed teeth is discussed in this article. The permanent incisors erupted with dilacerated crown, root malformations and missing enamel. Further, patient developed sinus, lateral root pathology, tooth mobility and malocclusion in relation to affected teeth which were managed by esthetic, functional, endodontic and surgical procedure. Root canal treatment along with palatal contouring and esthetic restoration by light cure composite was performed on the tooth with crown dilaceration and sinus, where as surgical management was considered for the tooth with root malformation.

  13. EXOSC8 mutations alter mRNA metabolism and cause hypomyelination with spinal muscular atrophy and cerebellar hypoplasia

    Science.gov (United States)

    Boczonadi, Veronika; Müller, Juliane S.; Pyle, Angela; Munkley, Jennifer; Dor, Talya; Quartararo, Jade; Ferrero, Ileana; Karcagi, Veronika; Giunta, Michele; Polvikoski, Tuomo; Birchall, Daniel; Princzinger, Agota; Cinnamon, Yuval; Lützkendorf, Susanne; Piko, Henriett; Reza, Mojgan; Florez, Laura; Santibanez-Koref, Mauro; Griffin, Helen; Schuelke, Markus; Elpeleg, Orly; Kalaydjieva, Luba; Lochmüller, Hanns; Elliott, David J.; Chinnery, Patrick F.; Edvardson, Shimon; Horvath, Rita

    2014-01-01

    The exosome is a multi-protein complex, required for the degradation of AU-rich element (ARE) containing messenger RNAs (mRNAs). EXOSC8 is an essential protein of the exosome core, as its depletion causes a severe growth defect in yeast. Here we show that homozygous missense mutations in EXOSC8 cause progressive and lethal neurological disease in 22 infants from three independent pedigrees. Affected individuals have cerebellar and corpus callosum hypoplasia, abnormal myelination of the central nervous system or spinal motor neuron disease. Experimental downregulation of EXOSC8 in human oligodendroglia cells and in zebrafish induce a specific increase in ARE mRNAs encoding myelin proteins, showing that the imbalanced supply of myelin proteins causes the disruption of myelin, and explaining the clinical presentation. These findings show the central role of the exosomal pathway in neurodegenerative disease. PMID:24989451

  14. Reduced ventral cingulum integrity and increased behavioral problems in children with isolated optic nerve hypoplasia and mild to moderate or no visual impairment.

    Directory of Open Access Journals (Sweden)

    Emma A Webb

    Full Text Available OBJECTIVES: To assess the prevalence of behavioral problems in children with isolated optic nerve hypoplasia, mild to moderate or no visual impairment, and no developmental delay. To identify white matter abnormalities that may provide neural correlates for any behavioral abnormalities identified. PATIENTS AND METHODS: Eleven children with isolated optic nerve hypoplasia (mean age 5.9 years underwent behavioral assessment and brain diffusion tensor imaging, Twenty four controls with isolated short stature (mean age 6.4 years underwent MRI, 11 of whom also completed behavioral assessments. Fractional anisotropy images were processed using tract-based spatial statistics. Partial correlation between ventral cingulum, corpus callosum and optic radiation fractional anisotropy, and child behavioral checklist scores (controlled for age at scan and sex was performed. RESULTS: Children with optic nerve hypoplasia had significantly higher scores on the child behavioral checklist (p<0.05 than controls (4 had scores in the clinically significant range. Ventral cingulum, corpus callosum and optic radiation fractional anisotropy were significantly reduced in children with optic nerve hypoplasia. Right ventral cingulum fractional anisotropy correlated with total and externalising child behavioral checklist scores (r = -0.52, p<0.02, r = -0.46, p<0.049 respectively. There were no significant correlations between left ventral cingulum, corpus callosum or optic radiation fractional anisotropy and behavioral scores. CONCLUSIONS: Our findings suggest that children with optic nerve hypoplasia and mild to moderate or no visual impairment require behavioral assessment to determine the presence of clinically significant behavioral problems. Reduced structural integrity of the ventral cingulum correlated with behavioral scores, suggesting that these white matter abnormalities may be clinically significant. The presence of reduced fractional anisotropy in the optic

  15. Pulmonary vascular diseases.

    Science.gov (United States)

    Mélot, C; Naeije, R

    2011-04-01

    Diseases of the pulmonary vasculature are a cause of increased pulmonary vascular resistance (PVR) in pulmonary embolism, chronic thromboembolic pulmonary hypertension (CTEPH), and pulmonary arterial hypertension or decreased PVR in pulmonary arteriovenous malformations on hereditary hemorrhagic telangiectasia, portal hypertension, or cavopulmonary anastomosis. All these conditions are associated with a decrease in both arterial PO2 and PCO2. Gas exchange in pulmonary vascular diseases with increased PVR is characterized by a shift of ventilation and perfusion to high ventilation-perfusion ratios, a mild to moderate increase in perfusion to low ventilation-perfusion ratios, and an increased physiologic dead space. Hypoxemia in these patients is essentially explained by altered ventilation-perfusion matching amplified by a decreased mixed venous PO2 caused by a low cardiac output. Hypocapnia is accounted for by hyperventilation, which is essentially related to an increased chemosensitivity. A cardiac shunt on a patent foramen ovale may be a cause of severe hypoxemia in a proportion of patients with pulmonary hypertension and an increase in right atrial pressure. Gas exchange in pulmonary arteriovenous malformations is characterized by variable degree of pulmonary shunting and/or diffusion-perfusion imbalance. Hypocapnia is caused by an increased ventilation in relation to an increased pulmonary blood flow with direct peripheral chemoreceptor stimulation by shunted mixed venous blood flow. PMID:23737196

  16. Multidetector computed tomography pulmonary angiography in childhood acute pulmonary embolism

    International Nuclear Information System (INIS)

    Pulmonary embolism is a life-threatening condition affecting people of all ages. Multidetector row CT pulmonary angiography has improved the imaging of pulmonary embolism in both adults and children and is now regarded as the routine modality for detection of pulmonary embolism. Advanced CT pulmonary angiography techniques developed in recent years, such as dual-energy CT, have been applied as a one-stop modality for pulmonary embolism diagnosis in children, as they can simultaneously provide anatomical and functional information. We discuss CT pulmonary angiography techniques, common and uncommon findings of pulmonary embolism in both conventional and dual-energy CT pulmonary angiography, and radiation dose considerations. (orig.)

  17. Multidetector computed tomography pulmonary angiography in childhood acute pulmonary embolism

    Energy Technology Data Exchange (ETDEWEB)

    Tang, Chun Xiang; Zhang, Long Jiang; Lu, Guang Ming [Medical School of Nanjing University, Department of Medical Imaging, Jinling Hospital, Nanjing, Jiangsu (China); Schoepf, U.J. [Medical School of Nanjing University, Department of Medical Imaging, Jinling Hospital, Nanjing, Jiangsu (China); Medical University of South Carolina, Department of Radiology and Radiological Science, Charleston, SC (United States); Medical University of South Carolina, Department of Pediatrics, Charleston, SC (United States); Chowdhury, Shahryar M. [Medical University of South Carolina, Department of Pediatrics, Charleston, SC (United States); Fox, Mary A. [Medical University of South Carolina, Department of Radiology and Radiological Science, Charleston, SC (United States)

    2015-09-15

    Pulmonary embolism is a life-threatening condition affecting people of all ages. Multidetector row CT pulmonary angiography has improved the imaging of pulmonary embolism in both adults and children and is now regarded as the routine modality for detection of pulmonary embolism. Advanced CT pulmonary angiography techniques developed in recent years, such as dual-energy CT, have been applied as a one-stop modality for pulmonary embolism diagnosis in children, as they can simultaneously provide anatomical and functional information. We discuss CT pulmonary angiography techniques, common and uncommon findings of pulmonary embolism in both conventional and dual-energy CT pulmonary angiography, and radiation dose considerations. (orig.)

  18. Mechanisms responsible for pulmonary hypertension

    Institute of Scientific and Technical Information of China (English)

    2008-01-01

    @@ Pulmonary hypertension is a pathophysiologic process characterized by progressive elevation of pulmonary vascular resistance and right heart failure, which is a common complication of many diseases. Pulmonary hypertension with no apparent causes (unknown etiology) is termed primary pulmonary hypertension or, more recently, idiopathic pulmonary arterial hypertension (IPAH).

  19. Pulmonary arterial hypertension : an update

    NARCIS (Netherlands)

    Hoendermis, E. S.

    2011-01-01

    Pulmonary arterial hypertension (PAH), defined as group 1 of the World Heart Organisation (WHO) classification of pulmonary hypertension, is an uncommon disorder of the pulmonary vascular system. It is characterised by an increased pulmonary artery pressure, increased pulmonary vascular resistance a

  20. Pulmonary Extramedullary Hematopoiesis Involving the Pulmonary Artery

    OpenAIRE

    Varun Monga; Margarida Silverman

    2015-01-01

    Extramedullary hematopoiesis (EMH) occurs as a complication of hematologic disorders such as myelofibrosis, sickle cell anemia and thalassemia. The extramedullary tissue usually involves liver, spleen and lymph nodes, less frequently the chest. We present a recent case of a man with myeloproliferative neoplasm who developed pulmonary hemorrhage secondary to EMH in the lung and pulmonary artery. Radiation therapy was considered the best approach, but it didn’t work and the patient died a week ...

  1. Effect of pulmonary hypertension on outcome of pulmonary tuberculosis

    OpenAIRE

    Majid Marjani; Parvaneh Baghaei; Majid Malekmohammad; Payam Tabarsi; Babak Sharif-Kashani; , Neda Behzadnia; Davood Mansouri; Mohammad Reza Masjedi; Ali Akbar Velayati

    2014-01-01

    Background: This study performed at the National Research Institute of Tuberculosis and Lung Disease, Tehran, Iran, aimed to evaluate the effect of concomitant pulmonary hypertension on the outcome of pulmonary tuberculosis. Methods: New cases of pulmonary tuberculosis were recruited for the study. Pulmonary hypertension was defined as systolic pulmonary arterial pressure ≥35 mm Hg estimated by transthoracic Doppler echocardiography. We assessed the relationship between pulmonary hypertensi...

  2. Surgically treated pulmonary stenosis

    DEFF Research Database (Denmark)

    Nielsen, Eva A; Hjortdal, Vibeke E

    additional four patients died after hospital discharge; therefore, the long-term mortality was 5%. The maximum follow-up period was 57 years, with a median of 33 years. In all, 16 patients (20%) required at least one re-intervention. Pulmonary valve replacement due to pulmonary regurgitation was the most...... re-intervention at the present stage. CONCLUSION: Surgical relief for pulmonary stenosis is efficient in relieving outflow obstruction; however, this efficiency is achieved at the cost of pulmonary regurgitation, leading to right ventricular dilatation and tricuspid regurgitation. When required......, pulmonary valve replacement is performed most frequently >20 years after the initial surgery. Lifelong follow-up of patients treated surgically for pulmonary stenosis is emphasised in this group of patients, who might otherwise consider themselves cured....

  3. Cystic pulmonary hydatidosis

    Science.gov (United States)

    Sarkar, Malay; Pathania, Rajnish; Jhobta, Anupam; Thakur, Babu Ram; Chopra, Rajesh

    2016-01-01

    Cystic echinococcosis (CE) is a zoonotic parasitic disease caused by the larval stages of the cestode Echinococcus granulosus. Worldwide, pulmonary hydatid cyst is a significant problem medically, socially, and economically. Surgery is the definitive therapy of pulmonary hydatidosis. Benzimidazoles may be considered in patients with a surgical contraindication. This review will focus on pathogenesis, lifecycle, clinical features, and management of pulmonary hydatid disease. PMID:27051107

  4. How Is Pulmonary Hypertension Diagnosed?

    Science.gov (United States)

    ... from the NHLBI on Twitter. How Is Pulmonary Hypertension Diagnosed? Your doctor will diagnose pulmonary hypertension (PH) ... To Look for the Underlying Cause of Pulmonary Hypertension PH has many causes, so many tests may ...

  5. Liver Disease and Pulmonary Hypertension

    Science.gov (United States)

    Liver Disease Pulmonary & PH Hypertension Did you know that if you have liver disease, you are at risk for pulmonary ... to the liver without cirrhosis. How does liver disease relate to pulmonary hypertension? Liver disease can cause what is known ...

  6. Solitary pulmonary nodule

    Science.gov (United States)

    ... as aspergillosis , coccidioidomycosis , cryptococcosis , or histoplasmosis Primary lung cancer is the most common cause of cancerous (malignant) pulmonary nodules. This is cancer that starts in the ...

  7. Limb mammary syndrome: a new genetic disorder with mammary hypoplasia, ectrodactyly, and other Hand/Foot anomalies maps to human chromosome 3q27.

    OpenAIRE

    Van Bokhoven, H.; Jung, M; Smits, A P; van Beersum, S.; Rüschendorf, F; Van Steensel, M; Veenstra, M; Tuerlings, J.H.; Mariman, E.C.; Brunner, H.G.; Wienker, T. F.; Reis, A.; Ropers, H H; Hamel, B C

    1999-01-01

    We report on a large Dutch family with a syndrome characterized by severe hand and/or foot anomalies, and hypoplasia/aplasia of the mammary gland and nipple. Less frequent findings include lacrimal-duct atresia, nail dysplasia, hypohydrosis, hypodontia, and cleft palate with or without bifid uvula. This combination of symptoms has not been reported previously, although there is overlap with the ulnar mammary syndrome (UMS) and with ectrodactyly, ectodermal dysplasia, and clefting syndrome. Al...

  8. Screening of TYR, OCA2, GPR143, and MC1R in patients with congenital nystagmus, macular hypoplasia, and fundus hypopigmentation indicating albinism

    OpenAIRE

    Markus N. Preising; Forster, Hedwig; Gonser, Miriam; Lorenz, Birgit

    2011-01-01

    Background A broad spectrum of pigmentation of the skin and hair is found among patients diagnosed with ocular albinism (OA) and oculocutaneous albinism (OCA). Even though complexion is variable, three ocular features, i.e., hypopigmentation of the fundus, hypoplasia of the macula, and nystagmus, are classical pathological findings in these patients. We screened 172 index patients with a clinical diagnosis of OA or OCA based on the classical findings, to evaluate the frequency of sequence var...

  9. Dental enamel Hypoplasia. Investigations on the Bones Exhumed from the Medieval Necropole of Lozova (Republic of Moldova, XIVth–XVth Centuries

    Directory of Open Access Journals (Sweden)

    Robert Daniel Simalcsik

    2014-10-01

    Full Text Available Dental hypoplasia is a developmental anomaly based on perturbations of amelogenesis. Hypoplasia defects are part of the unspecific quantitative indicators for the state of health and / or nutritional state during the formation of the dental buds. It is a response of the human organism to physiological stress. The incidence of this dysplasia in a past population can indicate its biological frailty in its attempt to adapt to the environmental changes. The osteological material was excavated in the interval 2010 – 2011 by archaeologists from the Archaeology Centre in Chisinau, from the Medieval cemetery of Lozova (Straseni County, Republic of Moldova, dated for the XIVth and XVth centuries. Fifty one skeletons from 50 inhumation graves have been excavated and analyzed so far. Only 40 individuals had most of their teeth present. The enamel hypoplasia is of linear transversal type, located on the labial surface of the dental crowns, in the median third. The canine is the most affected tooth, followed by the incisors. The incidence of dental enamel hypoplasia at population level (based on the data collected and on the number of graves excavates so far, which does not illustrate the entire population of the cemetery is 7.5%. The incidence of dental caries is 23.53%, of cribra orbitalia – 11.75%, and of cribra cranii externa – 1.96%. The results obtained for a relatively small rural community illustrate a good adaptation to the stressing environmental factors. The possible malnutrition and illness episodes suffered during early childhood were recovered along the growth and development processes.

  10. Three-Dimensional Endo-Cardiovascular Volume-Rendered Cine Computed Tomography of Isolated Left Ventricular Apical Hypoplasia: A Case Report and Literature Review.

    Science.gov (United States)

    Hong, Sun Hwa; Kim, Yang Min; Lee, Hyun Jong

    2016-01-01

    We report multidetector computed tomography (MDCT) and cardiac magnetic resonance (CMR) findings of a 34-year-old female with isolated left ventricular apical hypoplasia. The MDCT and CMR scans displayed a spherical left ventricle (LV) with extensive fatty infiltration within the myocardium at the apex, interventricular septum and inferior wall, anteroapical origin of the papillary muscle, right ventricle wrapping around the deficient LV apex, and impaired systolic function. MDCT visualized morphologic and also functional findings of this unique cardiomyopathy. PMID:26798219

  11. Case Series: Fetal Pulmonary Vein A-Wave Reversal: An Early Marker of Left-Sided Cardiac Anomalies?

    Directory of Open Access Journals (Sweden)

    Aldo L. Schenone

    2015-04-01

    Full Text Available Background - Improvements in congenital heart disease (CHD screening are needed based on the lack of sensitivity of current screening methods and the understanding that the early detection of certain CHDs may improve outcomes. Fetal venous circulation has caught medical attention, and two studies demonstrated that it is feasible to register pulmonary vein flow velocity waveforms (FVWs during early gestation. Meanwhile, the latter study proposed pulmonary vein A-wave reversal as a marker of cardiac anomaly. Methods - We report a series of six consecutive fetuses with confirmed cardiac anomalies that underwent first-trimester screening, including pulmonary vein FVWs, at our center during 2013. CHD was confirmed by late pregnancy echocardiography, and in three cases fetal autopsies were performed. Result/Discussion - The ductus venosus (DV and nuchal translucency (NT predicted 50% of CHD cases, whereas the combination of markers identified 66.6% of CHD cases. When adding pulmonary vein assessment, the rate of detection rose to 83.3%. Total five of six cases of CHD had reversal of pulmonary vein A-wave during early pregnancy. The sixth case with CHD and nonreversal of A-wave was described as right ventricle hypoplasia with type 1 tricuspid atresia and persistent ductus arteriosus. Conclusion This is the first series reporting pulmonary vein end-diastolic reversal as a CHD screening add-on during early pregnancy. The addition of pulmonary vein FVW assessment to the current CHD screening bundle could increase the rate detection of cardiac anomalies. This pilot study suggests that pulmonary vein end-diastolic flow reversal favors detection of left-sided CHD over the right-sided ones.

  12. Mutations in the very low-density lipoprotein receptor VLDLR cause cerebellar hypoplasia and quadrupedal locomotion in humans

    Science.gov (United States)

    Ozcelik, Tayfun; Akarsu, Nurten; Uz, Elif; Caglayan, Safak; Gulsuner, Suleyman; Onat, Onur Emre; Tan, Meliha; Tan, Uner

    2008-01-01

    Quadrupedal gait in humans, also known as Unertan syndrome, is a rare phenotype associated with dysarthric speech, mental retardation, and varying degrees of cerebrocerebellar hypoplasia. Four large consanguineous kindreds from Turkey manifest this phenotype. In two families (A and D), shared homozygosity among affected relatives mapped the trait to a 1.3-Mb region of chromosome 9p24. This genomic region includes the VLDLR gene, which encodes the very low-density lipoprotein receptor, a component of the reelin signaling pathway involved in neuroblast migration in the cerebral cortex and cerebellum. Sequence analysis of VLDLR revealed nonsense mutation R257X in family A and single-nucleotide deletion c2339delT in family D. Both these mutations are predicted to lead to truncated proteins lacking transmembrane and signaling domains. In two other families (B and C), the phenotype is not linked to chromosome 9p. Our data indicate that mutations in VLDLR impair cerebrocerebellar function, conferring in these families a dramatic influence on gait, and that hereditary disorders associated with quadrupedal gait in humans are genetically heterogeneous. PMID:18326629

  13. Homozygosity mapping and targeted genomic sequencing reveal the gene responsible for cerebellar hypoplasia and quadrupedal locomotion in a consanguineous kindred

    Science.gov (United States)

    Gulsuner, Suleyman; Tekinay, Ayse Begum; Doerschner, Katja; Boyaci, Huseyin; Bilguvar, Kaya; Unal, Hilal; Ors, Aslihan; Onat, O. Emre; Atalar, Ergin; Basak, A. Nazli; Topaloglu, Haluk; Kansu, Tulay; Tan, Meliha; Tan, Uner; Gunel, Murat; Ozcelik, Tayfun

    2011-01-01

    The biological basis for the development of the cerebro-cerebellar structures required for posture and gait in humans is poorly understood. We investigated a large consanguineous family from Turkey exhibiting an extremely rare phenotype associated with quadrupedal locomotion, mental retardation, and cerebro-cerebellar hypoplasia, linked to a 7.1-Mb region of homozygosity on chromosome 17p13.1–13.3. Diffusion weighted imaging and fiber tractography of the patients' brains revealed morphological abnormalities in the cerebellum and corpus callosum, in particular atrophy of superior, middle, and inferior peduncles of the cerebellum. Structural magnetic resonance imaging showed additional morphometric abnormalities in several cortical areas, including the corpus callosum, precentral gyrus, and Brodmann areas BA6, BA44, and BA45. Targeted sequencing of the entire homozygous region in three affected individuals and two obligate carriers uncovered a private missense mutation, WDR81 p.P856L, which cosegregated with the condition in the extended family. The mutation lies in a highly conserved region of WDR81, flanked by an N-terminal BEACH domain and C-terminal WD40 beta-propeller domains. WDR81 is predicted to be a transmembrane protein. It is highly expressed in the cerebellum and corpus callosum, in particular in the Purkinje cell layer of the cerebellum. WDR81 represents the third gene, after VLDLR and CA8, implicated in quadrupedal locomotion in humans. PMID:21885617

  14. Discrepancy in compliance between the clinical and genetic diagnosis of choroidal hypoplasia in Danish Rough Collies and Shetland Sheepdogs.

    Science.gov (United States)

    Fredholm, M; Larsen, R C; Jönsson, M; Söderlund, M A; Hardon, T; Proschowsky, H F

    2016-04-01

    Collie eye anomaly (CEA) is a congenital, inherited ocular disorder which is widespread in herding breeds. Clinically, the two major lesions associated with CEA are choroidal hypoplasia (CH) and coloboma, and both lesions are diagnosed based on ophthalmological examination. A 7.8-kb intronic deletion in the gene encoding non-homologous end-joining factor 1 (NHEJ1) has been reported to be the causative mutation underlying CH when present in the homozygous state. In this study, we have investigated the compliance between the clinical and genetic diagnosis of CH in the Danish Rough Collie and Shetland Sheepdog populations. Our results show that the deletion in NHEJ1 is not predictive for CH in the Danish Rough Collie population, whereas the clinical and genetic diagnosis is in accordance with each other in the Shetland Sheepdog population. Based on these results, it can be concluded that the intronic deletion in NHEJ1 is not the causative mutation but, rather, a marker linked to the locus underlying the trait in some populations but linked to both the wild-type and CH-causing locus in most dogs in the Danish Rough Collie population. PMID:26732749

  15. Revisiting the craniosynostosis‐radial ray hypoplasia association: Baller‐Gerold syndrome caused by mutations in the RECQL4 gene

    Science.gov (United States)

    Van Maldergem, L; Siitonen, H A; Jalkh, N; Chouery, E; De Roy, M; Delague, V; Muenke, M; Jabs, E W; Cai, J; Wang, L L; Plon, S E; Fourneau, C; Kestilä, M; Gillerot, Y; Mégarbané, A; Verloes, A

    2006-01-01

    Baller‐Gerold syndrome (BGS) is a rare autosomal recessive condition with radial aplasia/hypoplasia and craniosynostosis (OMIM 218600). Of >20 cases reported so far, a few appear atypical and have been reassigned to other nosologic entities, including Fanconi anaemia, Roberts SC phocomelia, and Pfeiffer syndromes after demonstration of corresponding cytogenetic or molecular abnormalities. Clinical overlap between BGS, Rothmund‐Thomson syndrome (RTS), and RAPADILINO syndrome is noticeable. Because patients with RAPADILINO syndrome and a subset of patients with RTS have RECQL4 mutations, we reassessed two previously reported BGS families and found causal mutations in RECQL4 in both. In the first family, four affected offspring had craniosynostosis and radial defect and one of them developed poikiloderma. In this family, compound heterozygosity for a R1021W missense mutation and a g.2886delT frameshift mutation of exon 9 was found. In the second family, the affected male had craniosynostosis, radial ray defect, poikiloderma, and short stature. He had a homozygous splice site mutation (IVS17‐2A>C). In both families, the affected offspring had craniosynostosis, radial defects, and growth retardation, and two developed poikiloderma. Our results confirm that BGS in a subgroup of patients is due to RECQL4 mutations and could be integrated into a clinical spectrum that encompasses RTS and RAPADILINO syndrome. PMID:15964893

  16. Autosomal-Recessive Mutations in the tRNA Splicing Endonuclease Subunit TSEN15 Cause Pontocerebellar Hypoplasia and Progressive Microcephaly.

    Science.gov (United States)

    Breuss, Martin W; Sultan, Tipu; James, Kiely N; Rosti, Rasim O; Scott, Eric; Musaev, Damir; Furia, Bansri; Reis, André; Sticht, Heinrich; Al-Owain, Mohammed; Alkuraya, Fowzan S; Reuter, Miriam S; Abou Jamra, Rami; Trotta, Christopher R; Gleeson, Joseph G

    2016-07-01

    The tRNA splicing endonuclease is a highly evolutionarily conserved protein complex, involved in the cleavage of intron-containing tRNAs. In human it consists of the catalytic subunits TSEN2 and TSEN34, as well as the non-catalytic TSEN54 and TSEN15. Recessive mutations in the corresponding genes of the first three are known to cause pontocerebellar hypoplasia (PCH) types 2A-C, 4, and 5. Here, we report three homozygous TSEN15 variants that cause a milder version of PCH2. The affected individuals showed progressive microcephaly, delayed developmental milestones, intellectual disability, and, in two out of four cases, epilepsy. None, however, displayed the central visual failure seen in PCH case subjects where other subunits of the TSEN are mutated, and only one was affected by the extensive motor defects that are typical in other forms of PCH2. The three amino acid substitutions impacted the protein level of TSEN15 and the stoichiometry of the interacting subunits in different ways, but all resulted in an almost complete loss of in vitro tRNA cleavage activity. Taken together, our results demonstrate that mutations in any known subunit of the TSEN complex can cause PCH and progressive microcephaly, emphasizing the importance of its function during brain development. PMID:27392077

  17. Bilateral meandering pulmonary veins

    Energy Technology Data Exchange (ETDEWEB)

    Thupili, Chakradhar R.; Udayasankar, Unni [Pediatric Imaging, Imaging Institute Cleveland Clinic, Cleveland, OH (United States); Renapurkar, Rahul [Imaging Institute Cleveland Clinic, Thoracic Imaging, L10, Cleveland, OH (United States)

    2015-06-15

    Meandering pulmonary veins is a rare clinical entity that can be mistaken for more complex congenital syndromes such as hypogenetic lung syndrome. We report imaging findings in a rare incidentally detected case of bilateral meandering pulmonary veins. We briefly discuss the role of imaging in diagnosing this condition, with particular emphasis on contrast-enhanced CT. (orig.)

  18. Pulmonary hypertension CT imaging

    International Nuclear Information System (INIS)

    Full text: The right heart catheterization is the gold standard in the diagnosis and determines the severity of pulmonary hypertension. The significant technical progress of noninvasive diagnostic imaging methods significantly improves the pixel density and spatial resolution in the study of cardiovascular structures, thus changes their role and place in the overall diagnostic plan. Learning points: What is the etiology, clinical manifestation and general pathophysiological disorders in pulmonary hypertension. What are the established diagnostic methods in the diagnosis and follow-up of patients with pulmonary hypertension. What is the recommended protocol for CT scanning for patients with clinically suspected or documented pulmonary hypertension. What are the important diagnostic findings in CT scan of a patient with pulmonary hypertension. Discussion: The prospect of instantaneous complex - anatomical and functional cardiopulmonary and vascular diagnostics seems extremely attractive. The contrast enhanced multislice computed (CT ) and magnetic resonance imaging are very suitable methods for imaging the structures of the right heart, with the possibility of obtaining multiple projections and three-dimensional imaging reconstructions . There are specific morphological features that, if carefully analyzed, provide diagnostic information. Thus, it is possible to avoid or at least reduce the frequency of use of invasive diagnostic cardiac catheterization in patients with pulmonary hypertension. Conclusion: This review focuses on the use of contrast-enhanced CT for comprehensive evaluation of patients with pulmonary hypertension and presents the observed characteristic changes in the chest, lung parenchyma , the structures of the right half of the heart and pulmonary vessels

  19. Pulmonary alveolar microlithiasis

    OpenAIRE

    Gayathri Devi, H.J.; K N Mohan Rao; Prathima, K. M.; Das, Jayanth K.

    2011-01-01

    Pulmonary alveolar microlithiasis is a rare disease of unknown cause. We report a case in a young boy who presented with history of failure to thrive and chest X-ray finding suggestive of miliary mottling. Open lung biopsy revealed pulmonary alveolar microlithiasis.

  20. Pediatric postprimary pulmonary tuberculosis

    International Nuclear Information System (INIS)

    Heading AbstractBackground. Postprimary pulmonary tuberculosis (TB) is not commonly seen in children.Objective. The purpose of this study was to determine the radiographic findings and patient characteristics of pediatric postprimary pulmonary TB.Materials and methods. We reviewed the clinical charts and chest radiographs in six patients.Results. The radiographic findings of pediatric postprimary pulmonary TB include upper-lobe consolidation and cavitation, multifocal ill-defined airspace opacities, evidence of prior pulmonary TB, and apical pleural thickening. Pleural effusions and lymphadenopathy are not commonly present. Although postprimary disease typically does not affect young children, five of the children in this series were less than ten years of age at the time of presentation.Conclusion The possibility of postprimary TB should be considered in pediatric patients at risk for this disease who present with upper-lobe pulmonary consolidation and cavitation. These patients are highly infectious and early recognition and treatment can limit transmission of TB. (orig.)

  1. Extralobar pulmonary sequestration

    Directory of Open Access Journals (Sweden)

    Ulys A

    2011-04-01

    Full Text Available Albertas Ulys, Narimantas Evaldas Samalavicius, Saulius Cicenas, Tadas Petraitis, Mantas Trakymas, Dmitrij Sheinin, Leonid GatijatullinInstitute of Oncology, Vilnius University, Santariskiu, Vilnius, LithuaniaAbstract: Prevalence of pulmonary sequestration accounts for up to 6.4% of all congenital pulmonary malformations. We report on a 40-year-old woman who underwent excision of an aberrant solid retroperitoneal mass in the left subdiaphragmatic area. The mass was identified to be an extralobar pulmonary sequestration. The diagnosis could be made without surgery by percutaneous tissue biopsy and imaging. We encourage keeping in mind pulmonary sequestration anomaly presenting as an aberrant retroperitoneal mass. The aim of this case report is to increase awareness about the condition and review the criteria for its definitive diagnosis and treatment.Keywords: retroperitoneal aberrant mass, extralobar pulmonary sequestration

  2. Nuclear medicine pulmonary diagnosis

    International Nuclear Information System (INIS)

    Scintigraphic recording of regional ventilation and perfusion with 99mTc-Aerosol and 99mTc-MAA remain in the foreground of nuclear medicine pulmonary diagnostics. The most important indication for ventilation scintigraphy is the prediction of postoperative pulmonary function, which is still performed in many hospitals with perfusion scintigraphy, and with which, in turn, intrapulmonary right-left shunts can be simply and also semiquantitatively recorded. Combined ventilation/perfusion scintigraphy offers a very high degree of sensitivity in the proof of acute pulmonary embolism, is therefore exceptionally well suited for exclusion diagnostics, while specificity compared to pulmonary angiography and spiral CT still needs some clarification. The self-cleaning mechanism of the lung can be quantitatively examined using mucociliary and resorptive clearance. The clinical areas of application are limited for methodical reasons. Primary diagnostics of bronchial carcinoma and dignity differentiation of solitary pulmonary nodules, preferably with 18F-FDG PET are gaining steadily in importance. (orig.)

  3. Genetic analysis of NR0B1 in congenital adrenal hypoplasia patients: identification of a rare regulatory variant resulting in congenital adrenal hypoplasia and hypogonadal hypogonadism without testicular carcinoma in situ.

    Science.gov (United States)

    Walker, A P; Fowkes, R C; Saleh, F; Kim, S-H; Wilkinson, P; Cabrera-Sharp, V; Talmud, P J; Humphries, S E; Looijenga, L H J; Bouloux, P M G

    2012-01-01

    There have been few testicular histology reports of adult patients with congenital adrenal hypoplasia/hypogonadal hypogonadism (AHC/HH), but Leydig cell hyperplasia has been observed, an indicator of the possibility of malignant transformation. We aimed to define the basis of AHC/HH in 4 pedigrees of different ethnic backgrounds. One patient was elected to have testicular biopsy which was examined for evidence of carcinoma in situ (CIS). NR0B1 mutation analysis was performed by sequence analysis. NR0B1 expression was investigated by RT-PCR. Testicular biopsy sections were stained with HE or immunostained for OCT3/4, an established marker of CIS. We identified NR0B1 variants in the 4 AHC pedigrees: pedigree 1 (United Arab Emirates), c.1130A>G predicting p.(Glu377Gly); pedigree 2 (English Caucasian), c.327C>A predicting p.(Cys109*); pedigree 3 (Oman), a 6-bp deletion of a direct repeat, c.857_862delTGGTGC predicting p.(Leu286_Val287del); pedigree 4 (English Caucasian), c.1168+1G>A, a regulatory variant within the NR0B1 splice donor site. This last male patient, aged 30 years, presented with evidence of HH but incomplete gonadotrophin deficiency, following an earlier diagnosis of Addison's disease at 3 years. Hormonal therapy induced virilisation. Testicular biopsy was performed. The c.1168+1G>A variant abrogated normal splicing of testicular mRNA. Histological examination showed poorly organised testicular architecture and absence of spermatozoa. Morphological analyses and the absence of immunohistochemical staining for OCT3/4 excluded the presence of malignant germ cell cancer and its precursor lesion, CIS. These studies add to the knowledge of the types and ethnic diversity of NR0B1 mutations and their associated phenotypes, and provide insight into the assessment and interpretation of testicular histology in AHC and HH. PMID:23018754

  4. Pulmonary mycoses among the clinically suspected cases of pulmonary tuberculosis

    OpenAIRE

    Tshering Ongmu Bhutia; Luna Adhikari

    2015-01-01

    Background: This study was carried with the main objectives: (1) to find out the occurrence of pulmonary mycoses in clinically suspected pulmonary tuberculosis cases at central referral hospital, Tadong, Sikkim. (2) To find out the various fungi causing pulmonary mycoses in clinically suspected pulmonary tuberculosis cases. Methods: 200 clinically suspected pulmonary tuberculosis cases who visited the department of microbiology for the diagnostic microscopic examination of sputum sample f...

  5. Pulmonary arteriovenous malformation in chronic thromboembolic pulmonary hypertension

    Science.gov (United States)

    Sharma, Arun; Gulati, Gurpreet S; Parakh, Neeraj; Aggarwal, Abhinav

    2016-01-01

    Chronic thromboembolic pulmonary hypertension is a morbid condition associated with complications such as hemoptysis, right heart failure, paradoxical embolism, and even death. There is no known association of chronic thromboembolic pulmonary hypertension with pulmonary arteriovenous malformation. Possible hypothesis for this association is an increased pulmonary vascular resistance leading to the compensatory formation of pulmonary arteriovenous malformation. We present one such case presenting with hemoptysis that was managed with endovascular treatment.

  6. Pulmonary endocrine cells in plexogenic pulmonary arteriopathy associated with cirrhosis.

    OpenAIRE

    Gosney, J R; Resl, M.

    1995-01-01

    A clear association has been described between numbers of pulmonary endocrine cells and the migration and/or proliferation of myofibroblasts which is thought to underlie the vascular changes seen in plexogenic pulmonary arteriopathy due to cardiac shunts and primary pulmonary hypertension. In contrast, the pulmonary endocrine system in a subject with florid pulmonary plexogenic arteriopathy associated with cirrhosis was entirely normal, suggesting possible differences in its pathogenesis.

  7. Variation in perikymata counts between repetitive episodes of linear enamel hypoplasia among orangutans from Sumatra and Borneo.

    Science.gov (United States)

    Skinner, Mark F

    2014-05-01

    The goal of this study is to evaluate whether repetitive linear enamel hypoplasia (rLEH) in apes is ecologically informative. LEH, which appears as grooves of thinner enamel often caused by malnutrition and/or disease, is a permanent record of departures from developmental homeostasis in infant and juvenile apes. Orangutans were selected for the study as they are a threatened species, have a remarkably high prevalence of rLEH, and because Sumatra is deemed a better habitat for orangutans than is Borneo, facilitating an ecological comparison. Objectives are to determine: a) whether periodicity of rLEH in orangutans corresponds to monsoon-mediated cycles in precipitation or food; and b) whether patterning of rLEH supports the view that Borneo is an inferior habitat. This study compares the counts of perikymata between adjacent LEH from 9 Sumatran and 26 Bornean orangutans to estimate the periodicity of rLEH. A total of 131 nonredundant inter-LEH perikymata counts were transformed to natural log values to reveal clusters of counts in a multiplicative series. Using a value of 10 days to form one perikyma, rLEH tends to recur semiannually in both populations. However, Sumatran orangutans show significantly fewer semiannual intervals and more annually recurring episodes. Bornean orangutans show mostly semiannual intervals and are more variable in inter-LEH perikymata counts. It is concluded that: a) developmental conditions for infant orangutans in Sumatra protect them somewhat from seasonal and environmental variation; b) temporal patterning of rLEH indicates that Borneo is the poorer habitat for orangutans; and c) the study of rLEH can be ecologically informative. PMID:24500972

  8. Low estriol levels in the maternal marker screen as a predictor of X-linked adrenal hypoplasia congenita: Case report

    Directory of Open Access Journals (Sweden)

    Durković Jasmina

    2014-01-01

    Full Text Available Introduction. X-linked adrenal hypoplasia congenita (AHC is a rare cause of adrenocortical insufficiency. Early postnatal diagnosis may prevent severe hypoglycemia, Addisonian crises and death. Low maternal estriol (E3 levels in the second trimester of pregnancy could indicate the possibility that the fetus suffers from a disorder that causes adrenal insufficiency. Suspicion is based on the fact that E3 originates from dehydroepiandrosterone (DHEA synthesized in the fetal adrenals. In case of adrenal insufficiency, the impaired production of fetal DHEA leads to a subsequent reduction of E3 concentrations in maternal serum. There are only a few reports of AHC suspected prenatally due to low maternal E3 levels. Case Outline. We describe two brothers with adrenal insufficiency due to AHC. The older brother was admitted to the hospital at the age of 33 days due to failure to thrive, vomiting, and dehydration. Genetic analysis revealed a hemizygous mutation in DAX-1 gene, thus confirming the diagnosis of ACH. The same mutation was detected in his mother. In the second pregnancy, E3 concentrations were determined from maternal serum. Estriol levels during the second trimester were extremely low suggesting the diagnosis of AHC. The diagnosis was confirmed during the neonatal period by genetic testing, and replacement therapy was started at the age of 10 days. This boy never experienced an adverse episode such as hypoglycemia or adrenal crises. Conclusion. Since determination of E3 is a simple, sensitive, noninvasive and cheap method, its use as an obligatory prenatal screening test should be accepted as a standard practice in Serbia.

  9. PULMONARY CIRCULATION AT EXERCISE

    OpenAIRE

    R. Naeije; CHESLER, N

    2012-01-01

    The pulmonary circulation is a high flow and low pressure circuit, with an average resistance of 1 mmHg.min.L−1 in young adults, increasing to 2.5 mmHg.min.L−1 over 4–6 decades of life. Pulmonary vascular mechanics at exercise are best described by distensible models. Exercise does not appear to affect the time constant of the pulmonary circulation or the longitudinal distribution of resistances. Very high flows are associated with high capillary pressures, up to a 20–25 mmHg threshold associ...

  10. Pulmonary manifestations of leptospirosis

    Directory of Open Access Journals (Sweden)

    Sameer Gulati

    2012-01-01

    Full Text Available Leptospirosis has a spectrum of presentation which ranges from mild disease to a severe form comprising of jaundice and renal failure. Involvement of the lung can vary from subtle clinical features to deadly pulmonary hemorrhage and acute respiratory distress syndrome. Of late, it has been identified that leptospirosis can present atypically with predominant pulmonary manifestations. This can delay diagnosis making and hence optimum treatment. The purpose of this review is to bring together all the reported pulmonary manifestations of leptospirosis and the recent trends in the management.

  11. PULMONARY EMBOLISM: DIAGNOSIS BY FOUNDATIONS

    OpenAIRE

    Грабовський, Ю. В.

    2015-01-01

    Pulmonary embolism (PE) – this obstruction pulmonary arterial clot (embolus or) formed in the venous system, the right atrium and right ventricle of the heart, or other material that ended up in the pulmonary circulation (droplets of fat, bone marrow, tumor cells, air, parasites, fragments of catheters etc.), resulting in lung parenchyma preryvayetsya circulation. This leads to the development of hypertension, pulmonary circulation and compensated or decompensated pulmonary heart. This is one...

  12. Pulmonary arterial hypertension: an update

    OpenAIRE

    Hoendermis, E.S.

    2011-01-01

    Pulmonary arterial hypertension (PAH), defined as group 1 of the World Heart Organisation (WHO) classification of pulmonary hypertension, is an uncommon disorder of the pulmonary vascular system. It is characterised by an increased pulmonary artery pressure, increased pulmonary vascular resistance and specific histological changes. It is a progressive disease finally resulting in right heart failure and premature death. Typical symptoms are dyspnoea at exercise, chest pain and syncope; furthe...

  13. Partial anomalous pulmonary venous return in patients with pulmonary hypertension

    International Nuclear Information System (INIS)

    Anomalous pulmonary venous return is an uncommon congenital malformation, and may be partial or total. Partial anomalous pulmonary venous return (PAPVR) is more common than total anomalous pulmonary venous return, and is often associated with other congenital cardiac anomalies. Whilst many patients with PAPVR remain asymptomatic, some may present in later age with symptoms related to left-to-right shunt, right heart failure and pulmonary hypertension. We report two cases of PAPVR detected on Computed Tomography Pulmonary Angiogram (CTPA) for the work up of pulmonary hypertension. The cases demonstrate that, although uncommon, partial anomalous pulmonary venous return can be a contributing factor to pulmonary hypertension and pulmonary veins should be carefully examined when reading a CTPA study.

  14. Pulmonary Hypertension in Scleroderma

    Science.gov (United States)

    ... Revatio ® . Sildenafil has been shown to improve exercise capacity, pulmonary artery pressure, and functional class in patients with ... Riociguat has been shown to significantly improve exercise ... TRANSPLANTATION Lung transplantation is reserved for patients with ...

  15. Reperfusion pulmonary edema

    International Nuclear Information System (INIS)

    Reperfusion following lower-torso ischemia in humans leads to respiratory failure manifest by pulmonary hypertension, hypoxemia, and noncardiogenic pulmonary edema. The mechanism of injury has been studied in the sheep lung lymph preparation, where it has been demonstrated that the reperfusion resulting in pulmonary edema is due to an increase in microvascular permeability of the lung to protein. This respiratory failure caused by reperfusion appears to be an inflammatory reaction associated with intravascular release of the chemoattractants leukotriene B4 and thromboxane. Histological studies of the lung in experimental animals revealed significant accumulation of neutrophils but not platelets in alveolar capillaries. The authors conclude that thromboxane generated and released from the ischemic tissue is responsible for the transient pulmonary hypertension. Second, it is likely that the chemoattractants are responsible for leukosequestration, and third, neutrophils, oxygen-derived free radicals, and thromboxane moderate the altered lung permeability

  16. Pulmonary mycosis in AIDS

    International Nuclear Information System (INIS)

    We retrospectively reviewed our series of 35 pulmonary mycosis in patients with AIDS, observed from 1987 to 1999, to correlate the imaging and pathologic findings. We further evaluated the frequency of fungal pneumonia before and after the use of a highly active antiretroviral therapy (HAART). Early recognition of pulmonary mycosis is imperative in these patients and improved survival can be achieved with early CT detection and prompt institution of high-dose antifungal therapy

  17. Pulmonary mycosis in AIDS

    Energy Technology Data Exchange (ETDEWEB)

    Busi Rizzi, Elisa; Schinina, Vincenzo; Bellussi, Angelo; De Santis, Andrea; Mazzuoli, Giovanna; Giosue, Sandro; Bibbolino, Corrado

    2001-01-01

    We retrospectively reviewed our series of 35 pulmonary mycosis in patients with AIDS, observed from 1987 to 1999, to correlate the imaging and pathologic findings. We further evaluated the frequency of fungal pneumonia before and after the use of a highly active antiretroviral therapy (HAART). Early recognition of pulmonary mycosis is imperative in these patients and improved survival can be achieved with early CT detection and prompt institution of high-dose antifungal therapy.

  18. Neonatal Pulmonary Hemosiderosis

    OpenAIRE

    Boris Limme; Ramona Nicolescu; Jean-Paul Misson

    2014-01-01

    Idiopathic pulmonary hemosiderosis (IPH) is a rare complex entity characterized clinically by acute or recurrent episodes of hemoptysis secondary to diffuse alveolar hemorrhage. The radiographic features are variable, including diffuse alveolar-type infiltrates, and interstitial reticular and micronodular patterns. We describe a 3-week-old infant presenting with hemoptysis and moderate respiratory distress. Idiopathic pulmonary hemosiderosis was the first working diagnosis at the Emergency De...

  19. ROCKing pulmonary fibrosis

    OpenAIRE

    Sheppard, Dean

    2013-01-01

    Clinical vignette: A 76-year-old man consults you for increasing shortness of breath over the past two years and an increasing requirement for home oxygen. A video-assisted thoracoscopic lung biopsy shows findings of usual interstitial pneumonitis, and he has no identifiable cause for pulmonary fibrosis, so he is considered to have idiopathic pulmonary fibrosis (IPF). His diffusing capacity for carbon monoxide (DLCO) is 45% of predicted, and his total lung capacity is 40% of predicted. Becaus...

  20. Pulmonary Rehabilitation in Emphysema

    OpenAIRE

    Ries, Andrew L.; Make, Barry J; Reilly, John J.

    2008-01-01

    Pulmonary rehabilitation is an established treatment for patients with chronic lung disease. Benefits include improvement in exercise tolerance, symptoms, and quality of life, with a reduction in the use of health care resources. As an adjunct to surgical programs, such as lung volume reduction surgery, pulmonary rehabilitation plays an important role not just in preparing patients for surgery and facilitating recovery but also in selecting patients and ensuring informed choices about treatme...

  1. Hemorrhagic pulmonary leptospirosis

    International Nuclear Information System (INIS)

    Leptospirosis is an infectious disease characteristic of humid eastern countries. It is relatively uncommon in the West. it usually presents with either hepatorenal or pulmonary involvement, two forms which generally overlap to a certain degree. We report a case of severe onset hemorrhagic pulmonary leptospirosis in a man who, during the course of the disease, presented multi systemic embolism (spleen, kidney and central nervous system). (Author) 11 refs

  2. Pulmonary manifestations of leptospirosis

    OpenAIRE

    Sameer Gulati; Anu Gulati

    2012-01-01

    Leptospirosis has a spectrum of presentation which ranges from mild disease to a severe form comprising of jaundice and renal failure. Involvement of the lung can vary from subtle clinical features to deadly pulmonary hemorrhage and acute respiratory distress syndrome. Of late, it has been identified that leptospirosis can present atypically with predominant pulmonary manifestations. This can delay diagnosis making and hence optimum treatment. The purpose of this review is to bring together a...

  3. Pulmonary alveolar proteinosis

    OpenAIRE

    Crestani, B; Epaud, R.; Aubier, M.; M-C. Dombret; Taille, C.; M-P. Debray; Danel, C.; R. Borie

    2011-01-01

    Pulmonary alveolar proteinosis (PAP) is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF) receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alveolar macrophages. Auto-immune alveolar proteinosis is the most frequent form of PAP, representing 9...

  4. Primary pulmonary alveolar proteinosis

    OpenAIRE

    Šarac Sanja; Milić Rade; Zolotarevski Lidija; Aćimović Slobodan; Tomić Ilija; Plavec Goran

    2012-01-01

    Introduction. Pulmonary alveolar proteinosis is an uncommon disease characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired disease can be idiopathic (primary) and secondary. The prevalence of acquired pulmonary alveolar proteinosis is about 0.37 per 100,000 persons. Common symptoms are dyspnea and cough. Chest X-ray shows bilateral perihilar infiltrates. Open-lung biopsy is the gold standard for the diagnosis. Treatment includes w...

  5. Congenital Pulmonary Alveolar Proteinosis

    OpenAIRE

    Saber Hammami; Khaled Harrathi; Khaled Lajmi; Samir Hadded; Chebil Ben Meriem; Mohamed Néji Guédiche

    2013-01-01

    Pulmonary alveolar proteinosis (PAP) is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been defined as congenital PAP which is fatal and a late-onset PAP which is similar to the adult form and less severe. The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole-lung lavage is the curr...

  6. Pulmonary interstitial emphysema.

    OpenAIRE

    Greenough, A; Dixon, A K; Roberton, N R

    1984-01-01

    Forty one of 210 preterm infants ventilated for respiratory distress syndrome in a three year period had radiological evidence of pulmonary interstitial emphysema. The development of this condition was significantly associated with malpositioning of the endotracheal tube in a main bronchus and the use of high peak pressure ventilation. Pulmonary interstitial emphysema was associated with a significant increase in the number of pneumothoraces, intraventricular haemorrhages, and the need for pr...

  7. Reexpansion Pulmonary Edema

    OpenAIRE

    Yasemin Işık; İsmail Katı; Onur Palabıyık; Uğur Göktaş

    2011-01-01

    Reexpansion pulmonary edema is a rare but life threating complication which is occurring during the treatment of lung collapse secondary to pleural effusion, pneumothorax or atelectasis. We presented a 68 year-old case with hypertension, heart failure, cerebrovascular disease and diabetes mellitus who had developed reexpansion pulmonary edema three hours after the application of unilateral thoracentesis (Journal of the Turkish Society of Intensive Care 2011; 9: 26-9)

  8. Gestational pulmonary arterial hypertension

    OpenAIRE

    Moll, Matthew; Payne, Julie G.; Tukey, Melissa H.; Farber, Harrison W.

    2015-01-01

    Pulmonary arterial hypertension (PAH) is a progressive disease marked by the irreversible pulmonary vascular changes of vasoconstriction, thrombosis, and proliferation of smooth muscle and endothelial cells. The untreated clinical course is characterized by progressive dyspnea and a median survival of less than 3 years. Many of these patients are of child-bearing age; however, pregnancy leads to physiologic changes that are particularly poorly tolerated in PAH, conferring a 30%–56% mortality....

  9. Idiopathic pulmonary arterial hypertension

    OpenAIRE

    Firth, Amy L.; Mandel, Jess; Yuan, Jason X.-J.

    2010-01-01

    Despite improved understanding of the pathobiology of pulmonary arterial hypertension (PAH), it remains a severe and progressive disease, usually culminating in right heart failure, significant morbidity and early mortality. Over the last decade, some major advances have led to substantial improvements in the management of PAH. Much of this progress was pioneered by work in animal models. Although none of the current animal models of pulmonary hypertension (PH) completely recapitulate the hum...

  10. Diagnosing pulmonary embolism

    Directory of Open Access Journals (Sweden)

    Khosla Rahul

    2006-01-01

    Full Text Available Pulmonary embolism (PE is a common, treatable, highly lethal emergency, which despite advances in diagnostic testing, remains an under diagnosed killer. The mortality rate of diagnosed and treated pulmonary embolism ranges from 3-8%, but increases to about 30% in untreated pulmonary embolism. PE is a part of the spectrum of venousthromboembolic disease and most pulmonary emboli have their origin from clots in the iliac, deep femoral, or popliteal veins. Nonspecific clinical signs and symptoms with low sensitivity and specificity of routine tests such as arterial blood gas, chest roentgenogram and electrocardiogram make the diagnosis of PE very challenging for the clinician. Pulmonary angiography is the gold standard diagnostic test, but this technique is invasive, expensive, not readily available and labor intensive. Diagnostic strategies have revolved around establishing clinical probabilities based on predictive models, then ruling in or ruling out the diagnosis of PE with various tests. The aim of this article was to review the literature and present an evidence- based medicine approach to diagnosis of pulmonary embolism.

  11. CT findings of pulmonary aspergillosis

    Energy Technology Data Exchange (ETDEWEB)

    Cheon, Jung Eun; Im, Jung Gi; Goo, Jin Mo; Kim, Hong Dae; Han, Man Chung [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1995-10-15

    The fungus aspergillus can cause a variety of pulmonary disorders. Aspergilloma is a noninvasive aspergillus colonization of virtually any type of preexisting pulmonary cavity or cystic space. Invasive pulmonary aspergillosis is serious, usually fatal infection in patients being treated with immunosuppressants or who have chronic debilitating disease. Allergic bronchopulmonary aspergillosis is characterized clinically by asthma, blood and sputum eosinophilia and positive immunologic reaction to aspergillus antigen. Awareness of the radiographic and CT findings of pulmonary aspergillosis is important in making the diagnosis of aspergillus-caused pulmonary disorders. In this pictorial essay, we illustrated various radiological findings of pulmonary aspergillosis focused on CT findings correlated with gross pathologic specimens.

  12. Bilateral renal agenesis/hypoplasia/dysplasia (BRAHD: postmortem analysis of 45 cases with breakpoint mapping of two de novo translocations.

    Directory of Open Access Journals (Sweden)

    Louise Harewood

    Full Text Available BACKGROUND: Bilateral renal agenesis/hypoplasia/dysplasia (BRAHD is a relatively common, lethal malformation in humans. Established clinical risk factors include maternal insulin dependent diabetes mellitus and male sex of the fetus. In the majority of cases, no specific etiology can be established, although teratogenic, syndromal and single gene causes can be assigned to some cases. METHODOLOGY/PRINCIPAL FINDINGS: 45 unrelated fetuses, stillbirths or infants with lethal BRAHD were ascertained through a single regional paediatric pathology service (male:female 34:11 or 3.1:1. The previously reported phenotypic overlaps with VACTERL, caudal dysgenesis, hemifacial microsomia and Müllerian defects were confirmed. A new finding is that 16/45 (35.6%; m:f 13:3 or 4.3:1 BRAHD cases had one or more extrarenal malformations indicative of a disoder of laterality determination including; incomplete lobulation of right lung (seven cases, malrotation of the gut (seven cases and persistence of the left superior vena cava (five cases. One such case with multiple laterality defects and sirelomelia was found to have a de novo apparently balanced reciprocal translocation 46,XY,t(2;6(p22.3;q12. Translocation breakpoint mapping was performed by interphase fluorescent in-situ hybridization (FISH using nuclei extracted from archival tissue sections in both this case and an isolated bilateral renal agenesis case associated with a de novo 46,XY,t(1;2(q41;p25.3. Both t(2;6 breakpoints mapped to gene-free regions with no strong evidence of cis-regulatory potential. Ten genes localized within 500 kb of the t(1;2 breakpoints. Wholemount in-situ expression analyses of the mouse orthologs of these genes in embryonic mouse kidneys showed strong expression of Esrrg, encoding a nuclear steroid hormone receptor. Immunohistochemical analysis showed that Esrrg was restricted to proximal ductal tissue within the embryonic kidney. CONCLUSIONS/SIGNIFICANCE: The previously unreported

  13. [Primary pulmonary sarcomas].

    Science.gov (United States)

    Jakubcová, T; Jakubec, P

    2009-01-01

    Primary pulmonary sarcomas are rare diseases unlike lung carcinomas. The occurence of these sarcomas is between 0.013-0.40% of all malignant lung tumours. There are malignant mesenchymal tumours. They are flowing from the soft tissue of lung. The pulmonary sarcomas are heterogenic group with various biological behaviour. Their morfologic structure does not digger from the sarcomas of soft tissue. The primary pulmonary sarcomas occur more often in childhood and in young people unlike lung carcinomas. Radiation and some toxic substances are noted risk factors. Some gene mutations, infectious pathoghens and contraception have a possible impact on the origin of some types of the sarcomas. The current hypothesis is, that most of the sarcomas, if not all sarcomas, stem from primitive multipotent mesenchymal cell by malignant transformation in one or more lines. The diagnostic standard is biopsy from tumour with histologic and immunohistochemistry examination of a sample. The basic diagnostic problem is exclusion of a secondary origin of sarcomatic cells in the lung, because pulmonary metastasis of extrapulmonary sarcomas are more often than the primary pulmonary involvement.The optimal treatment is a resection of the tumour.The other therapeutic modalities are radiotherapy and chemotherapy, but results of these modalities are unsatisfactory. There are various chemotherapeutic regimes, monotherapy or combination regimes. The basic cytostatics are doxorubicine, iphosphamide, dacarbazine. Problems of the chemotherapy are high toxicity and relatively low curative effect about 20%.The first studies with biological treatment of the sarcomas of soft tissue have been published recently.This types of drugs could be a part of the complex management of these primary pulmonary tumours in the future. The primary pulmonary sarcomas have mostly aggresive course and often recur. Their prognosis is usually not very good. The survival median is 48 months and 5-years survival ranges

  14. Predictors of Pulmonary Infarction.

    Science.gov (United States)

    Miniati, Massimo; Bottai, Matteo; Ciccotosto, Cesario; Roberto, Luca; Monti, Simonetta

    2015-10-01

    In the setting of acute pulmonary embolism (PE), pulmonary infarction is deemed to occur primarily in individuals with compromised cardiac function.The current study was undertaken to establish the prevalence of pulmonary infarction in patients with acute PE, and the relationship between infarction and: age, body height, body mass index (BMI), smoking habits, clot burden, and comorbidities.The authors studied prospectively 335 patients with acute PE diagnosed by computed tomographic angiography (CT) in 18 hospitals throughout central Italy. The diagnosis of pulmonary infarction on CT was based on Hampton and Castleman's criteria (cushion-like or hemispherical consolidation lying along the visceral pleura). Multivariable logistic regression was used to model the relationship between covariates and the probability of pulmonary infarction.The prevalence of pulmonary infarction was 31%. Patients with infarction were significantly younger and with significantly lower prevalence of cardiovascular disease than those without (P < 0.001). The frequency of infarction increased linearly with increasing height, and decreased with increasing BMI. In logistic regression, the covariates significantly associated with the probability of infarction were age, body height, BMI, and current smoking. The risk of infarction grew with age, peaked at approximately age 40, and decreased afterwards. Increasing body height and current smoking were significant amplifiers of the risk of infarction, whereas increasing BMI appeared to confer some protection.Our data indicate that pulmonary infarction occurs in nearly one-third of the patients with acute PE. Those with infarction are often young and otherwise healthy. Increasing body height and active smoking are predisposing risk factors. PMID:26469892

  15. Pulmonary thromboembolism in children

    Energy Technology Data Exchange (ETDEWEB)

    Babyn, Paul S.; Gahunia, Harpal K. [Hospital for Sick Children, Department of Pediatric Diagnostic Imaging, Toronto, ON (Canada); Massicotte, Patricia [Stollery Children' s Hospital and University of Alberta, Departments of Pediatric Hematology and Cardiology, Edmonton, AB (Canada)

    2005-03-01

    Pulmonary thromboembolism (PTE) is uncommonly diagnosed in the pediatric patient, and indeed often only discovered on autopsy. The incidence of pediatric PTE depends upon the associated underlying disease, diagnostic tests used, and index of suspicion. Multiple risk factors can be found including: peripartum asphyxia, dyspnea, haemoptysis, chest pain, dehydration, septicemia, central venous lines (CVLs), trauma, surgery, ongoing hemolysis, vascular lesions, malignancy, renal disease, foreign bodies or, uncommonly, intracranial venous sinus thrombosis, burns, or nonbacterial thrombotic endocarditis. Other types of embolism can occur uncommonly in childhood and need to be recognized, as the required treatment will vary. These include pulmonary cytolytic thrombi, foreign bodies, tumor and septic emboli, and post-traumatic fat emboli. No single noninvasive test for pulmonary embolism is both sensitive and specific. A combination of diagnostic procedures must be used to identify suspect or confirmed cases of PTE. This article reviews the risk factors, clinical presentation and treatment of pulmonary embolism in children. It also highlights the current diagnostic tools and protocols used to evaluate pulmonary embolism in pediatric patients. (orig.)

  16. Pulmonary edema in renal failure

    International Nuclear Information System (INIS)

    Forty-nine cases of pulmonary edema in nephropatic patients were studied. The most frequent radiologic findings are discussed. The unreliability of a precise differentiation between ''cardiac'' and ''renal'' patterns of pulmonary edema in nephropatic patients is emphasized

  17. Radiographic signs of pulmonary diseases

    International Nuclear Information System (INIS)

    The first part of the radiographic interpretation in pulmonary radiography is the description of radiographic signs. They are presented in terms of: (i) changes in pulmonary volume and (ii) changes in pulmonary density. Changes in density should be precisely located and characterized. A protocole for the description of pulmonary changes is therefore proposed. A few words are then said on how to interprete these radiographic signs

  18. Genetics Home Reference: pulmonary veno-occlusive disease

    Science.gov (United States)

    ... Understand Genetics Home Health Conditions pulmonary veno-occlusive disease pulmonary veno-occlusive disease Enable Javascript to view the ... Disease Control and Prevention: Pulmonary Hypertension Fact Sheet Disease InfoSearch: Pulmonary venoocclusive ... pulmonary venoocclusive disease Orphanet: Pulmonary ...

  19. Primary pulmonary Hodgkin's lymphoma

    International Nuclear Information System (INIS)

    Two cases of primary pulmonary Hodgkin's lymphoma (PPHL) are presented, a male aged 21, and a female aged 32 years. Symptoms included non-productive cough, shortness of breath, low grade fever, wheezing and weight loss. Duration of illness varied between 6 weeks in the male patient to female patient. Both patients were given an empirical trial of antibiotics and anti-cough measures with no response. Radiological studies carried out after failure to respond to medical treatment, revealed the presence of pulmonary parenchymal masses in both patients. Cytology, bronchoscopic and transbronchial biopsies were not diagnostic, which led to opened wedge resections. Finally, the diagnosis of primary pulmonary Hodgkin's disease was reached after supportive immunohistochemical staining (CD 30 and CD 15 both positive in Rs cells). Both patients were regarded as stage I extranodal (IE) after exhaustive measures failed to demonstrate involvement of other body cites. (author)

  20. [Diffuse Pulmonary Ossification].

    Science.gov (United States)

    Avsar, K; Behr, J; Morresi-Hauf, A

    2016-04-01

    Diffuse pulmonary ossification (DPO) represents an uncommon condition usually associated with different underlying pulmonary and extrapulmonary diseases. In this work, we discuss eleven patients of our clinic with the diagnosis of a diffuse pulmonary ossification. We focus on histological changes in the surrounding lung tissue. Clinical and radiological findings were analysed. The aim of the study is to collect data for a better understanding of this condition, especially in association with interstitial lung disease.Three patients with interstitial lung disease had histological findings of UIP. The follow-up data of these patients showed a benign course of the disease.The analysis of the clinical data yielded a very heterogenous group. Regarding these fact we assume, that DPO is not an own entity, but a pathological epiphenomenon in the context of different conditions, possibly with pathogenetic overlap. PMID:26829606

  1. Neonatal pulmonary hemosiderosis.

    Science.gov (United States)

    Limme, Boris; Nicolescu, Ramona; Misson, Jean-Paul

    2014-01-01

    Idiopathic pulmonary hemosiderosis (IPH) is a rare complex entity characterized clinically by acute or recurrent episodes of hemoptysis secondary to diffuse alveolar hemorrhage. The radiographic features are variable, including diffuse alveolar-type infiltrates, and interstitial reticular and micronodular patterns. We describe a 3-week-old infant presenting with hemoptysis and moderate respiratory distress. Idiopathic pulmonary hemosiderosis was the first working diagnosis at the Emergency Department and was confirmed, 2 weeks later, by histological studies (bronchoalveolar lavage). The immunosuppressive therapy by 1 mg/kg/d prednisone was immediately started, the baby returned home on steroid therapy at a dose of 0,5 mg/kg/d. The diagnosis of idiopathic pulmonary hemosiderosis should be evocated at any age, even in the neonate, when the clinical presentation (hemoptysis and abnormal radiological chest images) is strongly suggestive. PMID:25389504

  2. Neonatal Pulmonary Hemosiderosis

    Directory of Open Access Journals (Sweden)

    Boris Limme

    2014-01-01

    Full Text Available Idiopathic pulmonary hemosiderosis (IPH is a rare complex entity characterized clinically by acute or recurrent episodes of hemoptysis secondary to diffuse alveolar hemorrhage. The radiographic features are variable, including diffuse alveolar-type infiltrates, and interstitial reticular and micronodular patterns. We describe a 3-week-old infant presenting with hemoptysis and moderate respiratory distress. Idiopathic pulmonary hemosiderosis was the first working diagnosis at the Emergency Department and was confirmed, 2 weeks later, by histological studies (bronchoalveolar lavage. The immunosuppressive therapy by 1 mg/kg/d prednisone was immediately started, the baby returned home on steroid therapy at a dose of 0,5 mg/kg/d. The diagnosis of idiopathic pulmonary hemosiderosis should be evocated at any age, even in the neonate, when the clinical presentation (hemoptysis and abnormal radiological chest images is strongly suggestive.

  3. Pulmonary lymphatics and radiation

    International Nuclear Information System (INIS)

    Knowledge of the anatomy and physiology of the respiratory system has been more difficult to acquire than that of other organ systems owing to the complexity of the respiratory function of the lungs and to the technical difficulties involved. This is especially true of the lymphatics of the lung and is illustrated by the fact that the first measurement of pulmonary lymph flow was in 1942 by Warren and Drinker. A review of the literature reveals that few experiments have been designed to study the pulmonary lymphatics per se in relation to the effects of external radiation or after the inhalation of radioactive particles. However, the documented involvement of hilar lymph nodes implies that the lung lymphatics have a role in transporting particles from the alveoli or malignant cells from the parenchyma. Information from clinical and experimental sources, though scattered, is fairly abundant and of value in assessing the role of the pulmonary lymphatics. Our method for collecting pulmonary lymph is presented. Studies on the pulmonary lymph flow in normal dogs and in dogs with experimental congestive heart failure are described. We irradiated (4000 to 5000 R) the medial one-third of both lungs of a series of dogs. The lymph flow of the lungs was measured immediately after the course of irradiation and after a period of about 5 months. Although lung biopsies showed characteristic radiation pneumonitis in many areas, alterations in the lung parenchyma were not quantitatively reflected in the pulmonary lymph flow either in the acute stage or after fibrosis had time to develop

  4. Pulmonary hypertension in chronic obstructive pulmonary disease

    International Nuclear Information System (INIS)

    Pulmonary hypertension (PH) is a relatively common complication of chronic obstructive pulmonary disease (COPD). Its appearance during the course of COPD is associated with a worsened prognosis, due to reduced life expectancy and greater use of health care resources. Although a well-defined lineal relationship has not been shown, the prevalence of PH in patients with COPD is higher in cases characterized by greater obstruction and severity. PH is infrequent in cases of mild and moderate COPD. In cases of COPD, PH is generally mild or moderate, and seldom impairs right ventricular function. In many cases it is not apparent during rest, and manifests itself during exercise. PH can be severe or out of proportion with the severity of COPD. In this situation, the possibility of associated conditions should be explored, although COPD might be the only final explanation. There is scarce knowledge about the prevalence and behavior of PH in patients with COPD residing at intermediate and high altitudes (>2.500 meters above sea level), which is a common situation in Latin America and Asia. PH in COPD is not exclusively related with hypoxia/hypoxaemia and hypercapnia. The mechanical disturbances related with COPD (hyper inflation and high alveolar pressure) and inflammation may prevail as causes of endothelial injury and remodeling of pulmonary circulation, which contribute to increased pulmonary vascular pressure and resistance. The appearance of signs of corpulmonale indicates advanced PH. This condition should therefore be suspected early when dyspnoea, hypoxaemia, and impairment of diffusion are not in keeping with the degree of obstruction. PH is confirmed by Doppler echocardiography. Right heart catheterization may be justified in selected cases. Long-term oxygen therapy is the only intervention proven to be temporarily useful. Conventional vasodilators do not produce medium- or long-term improvement and can be detrimental to the ventilation-perfusion relation. Neither

  5. Pulmonary lymphangioleiomyomatosis: case reports

    International Nuclear Information System (INIS)

    We reviewed the radiologic, physiologic, and pathologic features of biopsy-proved pulmonary lymphangioleiomyomatosis (LAM) in 2 patients. Chest radiographs showed diffuse increased interstitial lung markings with normal or slightly increased lung volume. The prominent computed tomographic (CT) feature was thin-walled cysts throughout the lungs. Pulmonary function test showed a mixed obstructive and restrictive pattern in one and a mainly restrictive pattern in another, which was somewhat different from that of the reported cases. Proliferation of smooth muscle cells in the lymphatics, alveolar septae, vessels, and bronchioles was the pathologic finding

  6. Reexpansion pulmonary edema

    Directory of Open Access Journals (Sweden)

    Genofre Eduardo Henrique

    2003-01-01

    Full Text Available Reexpansion pulmonary edema (RPE is a rare, but frequently lethal, clinical condition. The precise pathophysiologic abnormalities associated with this disorder are still unknown, though decreased pulmonary surfactant levels and a pro-inflammatory status are putative mechanisms. Early diagnosis is crucial, since prognosis depends on early recognition and prompt treatment. Considering the high mortality rates related to RPE, preventive measures are still the best available strategy for patient handling. This review provides a brief overview of the pathophysiology, diagnosis, treatment, and prevention of RPE, with practical recommendations for adequate intervention.

  7. Congenital Pulmonary Alveolar Proteinosis

    Directory of Open Access Journals (Sweden)

    Saber Hammami

    2013-01-01

    Full Text Available Pulmonary alveolar proteinosis (PAP is a rare syndrome characterized by pulmonary surfactant accumulation within the alveolar spaces. It occurs with a reported prevalence of 0.1 per 100,000 individuals. Two clinically different pediatric types have been defined as congenital PAP which is fatal and a late-onset PAP which is similar to the adult form and less severe. The clinical course of PAP is variable, ranging from spontaneous remission to respiratory failure. Whole-lung lavage is the current standard treatment for PAP patients. We report a new congenital case of PAP.

  8. Canine pulmonary angiostrongylosis

    DEFF Research Database (Denmark)

    Koch, Jørgen; Willesen, Jakob Lundgren

    2009-01-01

    Canine pulmonary angiostrongylosis is an emerging snail-borne disease causing verminous pnemonia and coagulopathy in dogs. The parasite is fund in Europe, North and South America and Africa, covering tropical, subtropical and temperate regions. Its distribution has been characterised by isolated...... larvae may not reflect what happens under field conditions. There is insufficient understanding of the spread of infection and the dynamic consequences of this parasite in the canine population. This review discusses the biology, epidemiology, clinical aspects and management of canine pulmonary...

  9. When a pulmonary embolism is not a pulmonary embolism: a rare case of primary pulmonary leiomyosarcoma

    OpenAIRE

    Muganlinskaya, Nargiz; Guzman, Amanda; Dahagam, Chanukya; Stephen R. Selinger

    2015-01-01

    Arterial leiomyosarcomas account for up to 21% of vascular leiomyosarcomas, with 56% of arterial leiomyosarcomas occurring in the pulmonary artery. While isolated cases of primary pulmonary artery leiomyosarcoma document survival up to 36 months after treatment, these uncommon, aggressive tumors are highly lethal, with 1-year survival estimated at 20% from the onset of symptoms. We discuss a rare case of a pulmonary artery leiomyosarcoma that was originally diagnosed as a pulmonary embolism (...

  10. Alteration in pulmonary perfusion due to iatrogenic pulmonary vein stenosis: A mimicker of pulmonary embolism

    OpenAIRE

    Ostwani, Wesam; Arabi, Mohammad

    2011-01-01

    Iatrogenic pulmonary vein stenosis (PVS) is a known, yet rare, complication of atrial radiofrequency ablation. Alterations in pulmonary perfusion may mimic massive pulmonary embolism on a ventilation/perfusion (V/Q) scintigraphy. This is particularly important due to the overlap in presenting clinical symptoms. The present case illustrates the functional significance of PVS and the changes in perfusion in response to angioplasty.

  11. Radiological diagnosis of pulmonary hypertension

    International Nuclear Information System (INIS)

    In 43 patients with obstructive and restrictive lung disease a catheterisation of the right heart with measurement of pulmonary artery pressure was performed. In a retrospective study several radiological parameters of pulmonary hypertension were evaluated on the chest radiographs of these patients. Considering those parameters on the p.a. and lateral chest radiograph, the diagnosis of pulmonary hypertension in patients with elevated pulmonary artery pressure at rest can be made with great accuracy. When pulmonary artery pressure is elevated only during exercise, the accuracy of radiological diagnosis is much lower. (orig.)

  12. Pulmonary haemosiderosis and gluten.

    OpenAIRE

    Reading, R; Watson, J.G.; Platt, J. W.; Bird, A. G.

    1987-01-01

    A child with idiopathic pulmonary haemosiderosis for three years required three monthly transfusions. Circulating avian, gliadin, and reticulin antibodies suggested the diagnosis of gluten enteropathy, and jejunal biopsy showed subtotal villous atrophy. During 15 months on a gluten free diet his growth and behaviour improved and he required no transfusions.

  13. Pulmonary nuclear medicine

    International Nuclear Information System (INIS)

    This article reviews the contribution made by nuclear imaging to the assessment, diagnosis and monitoring of patients with respiratory disease. It focuses on several specific areas including the diagnosis of pulmonary embolism, the investigation of intrapulmonary infection and neoplasm and the role positron emission tomography (PET) scanning. (orig.)

  14. Primary pulmonary alveolar proteinosis

    Directory of Open Access Journals (Sweden)

    Šarac Sanja

    2012-01-01

    Full Text Available Introduction. Pulmonary alveolar proteinosis is an uncommon disease characterized by the accumulation of surfactant proteins and phospholipids within the alveolar spaces. Acquired disease can be idiopathic (primary and secondary. The prevalence of acquired pulmonary alveolar proteinosis is about 0.37 per 100,000 persons. Common symptoms are dyspnea and cough. Chest X-ray shows bilateral perihilar infiltrates. Open-lung biopsy is the gold standard for the diagnosis. Treatment includes whole-lung lavage, application of granulocyte-macrophage colonystimulating factor and lung transplantation. Case report. We reported a 51 year-old man with primary form of the disease. It was the second case of this extremely rare disease in the past 30 years in our clinic. The symptoms were longlasting dry cough, fever and physical deterioration. Chest Xray revealed bilateral pulmonary infiltrates; computed tomography showed patchy ground-glass opacification with interlobular thickening. The diagnosis was established by open lung biopsy. Additional tests were performed to exclude secondary form of the disease. Conclusion. We presented a rare clinical entity with typical clinical features and clinical and radiological course of the disease, in order to improve differential diagnostic approach to patients with bilateral lung infiltrations. In patients with pulmonary alveolar proteinosis timely diagnosis and adequate treatment can improve a prognosis.

  15. Pulmonary alveolar proteinosis

    OpenAIRE

    Chan, Adrian Kwok Wai; Takano, Angela; Hsu, Ann Ling; Low, Su Ying

    2014-01-01

    We report a 68-year-old female who presented with chronic cough and progressive dyspnoea. Computed tomography of the thorax and subsequent bronchoscopy confirmed the diagnosis of pulmonary alveolar proteinosis (PAP), which was treated with whole lung lavage. This case is reported in view of the low incidence of PAP.

  16. Imaging pulmonary fibrosis

    International Nuclear Information System (INIS)

    Localized fibrosis of the lung is usually scar tissue while diffuse pulmonary fibrosis is more often a sign of active disease. Chronic infiltrative lung disease may be classified into four categories: idiopathic pneumonitis, collagen diseases, granulomatosis (sarcoidosis), and caused by known diseases (pneumoconiosis, hypersensitivity pneumonitis, drug-induced lung disease, radiation). (authors)

  17. Pulmonary langerhans cell histiocytosis

    Directory of Open Access Journals (Sweden)

    Suri Harpreet S

    2012-03-01

    Full Text Available Abstract Pulmonary Langerhans Cell Histiocytosis (PLCH is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events resulting in enhanced recruitment and activation of Langerhans cells in small airways. Bronchiolar inflammation may be accompanied by variable lung interstitial and vascular involvement. While cellular inflammation is prominent in early disease, more advanced stages are characterized by cystic lung destruction, cicatricial scarring of airways, and pulmonary vascular remodeling. Pulmonary function is frequently abnormal at presentation. Imaging of the chest with high resolution chest CT scanning may show characteristic nodular and cystic abnormalities. Lung biopsy is necessary for a definitive diagnosis, although may not be required in instances were imaging findings are highly characteristic. There is no general consensus regarding the role of immunosuppressive therapy in smokers with PLCH. All smokers must be counseled on the importance of smoking cessation, which may result in regression of disease and obviate the need for systemic immunosuppressive therapy. The prognosis for most patients is relatively good, particularly if longitudinal lung function testing shows stability. Complications like pneumothoraces and secondary pulmonary hypertension may shorten life expectancy. Patients with progressive disease may require lung transplantation.

  18. Hantavirus Pulmonary Syndrome

    Centers for Disease Control (CDC) Podcasts

    2011-07-14

    Dr. Adam MacNeil, epidemiologist with Viral Special Pathogens Branch at CDC, discusses hantavirus pulmonary syndrome.  Created: 7/14/2011 by National Center for Emerging Zoonotic and Infectious Diseases (NCEZID).   Date Released: 7/18/2011.

  19. Pulmonary function in space

    Science.gov (United States)

    West, J. B.; Elliott, A. R.; Guy, H. J.; Prisk, G. K.

    1997-01-01

    The lung is exquisitely sensitive to gravity, and so it is of interest to know how its function is altered in the weightlessness of space. Studies on National Aeronautics and Space Administration (NASA) Spacelabs during the last 4 years have provided the first comprehensive data on the extensive changes in pulmonary function that occur in sustained microgravity. Measurements of pulmonary function were made on astronauts during space shuttle flights lasting 9 and 14 days and were compared with extensive ground-based measurements before and after the flights. Compared with preflight measurements, cardiac output increased by 18% during space flight, and stroke volume increased by 46%. Paradoxically, the increase in stroke volume occurred in the face of reductions in central venous pressure and circulating blood volume. Diffusing capacity increased by 28%, and the increase in the diffusing capacity of the alveolar membrane was unexpectedly large based on findings in normal gravity. The change in the alveolar membrane may reflect the effects of uniform filling of the pulmonary capillary bed. Distributions of blood flow and ventilation throughout the lung were more uniform in space, but some unevenness remained, indicating the importance of nongravitational factors. A surprising finding was that airway closing volume was approximately the same in microgravity and in normal gravity, emphasizing the importance of mechanical properties of the airways in determining whether they close. Residual volume was unexpectedly reduced by 18% in microgravity, possibly because of uniform alveolar expansion. The findings indicate that pulmonary function is greatly altered in microgravity, but none of the changes observed so far will apparently limit long-term space flight. In addition, the data help to clarify how gravity affects pulmonary function in the normal gravity environment on Earth.

  20. Pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis

    International Nuclear Information System (INIS)

    Objectives: Patients with idiopathic pulmonary fibrosis (IPF) have an increased risk of pulmonary tuberculosis. However, detecting pulmonary tuberculosis may be difficult due to the underlying fibrosis. The aim of this report is to describe the radiological and clinical findings of pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis. Materials and methods: We reviewed 143 consecutive patients in whom IPF was diagnosed by either the histological or radio-clinical criteria. Among them, nine patients were histologically (n=2) or bacteriologically (n=7) confirmed to have active pulmonary tuberculosis. The location and patterns of pulmonary tuberculosis were examined on a thin section CT scan. Results: The most common thin section CT findings were subpleural nodules (n=6; mean diameter, 3.2 cm) and a lobar or segmental consolidation (n=3). The lesions were located most commonly in the right lower lobe (n=4). The incidence of tuberculosis in patients with idiopathic pulmonary fibrosis was more than five times higher than that of the general population. Conclusion: The atypical manifestation of pulmonary tuberculosis is common in patients with idiopathic pulmonary fibrosis, which may mimic lung cancer or bacterial pneumonia

  1. Pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis

    Energy Technology Data Exchange (ETDEWEB)

    Chung, Myung Jin; Goo, Jin Mo E-mail: jmgoo@plaza.snu.ac.kr; Im, Jung-Gi

    2004-11-01

    Objectives: Patients with idiopathic pulmonary fibrosis (IPF) have an increased risk of pulmonary tuberculosis. However, detecting pulmonary tuberculosis may be difficult due to the underlying fibrosis. The aim of this report is to describe the radiological and clinical findings of pulmonary tuberculosis in patients with idiopathic pulmonary fibrosis. Materials and methods: We reviewed 143 consecutive patients in whom IPF was diagnosed by either the histological or radio-clinical criteria. Among them, nine patients were histologically (n=2) or bacteriologically (n=7) confirmed to have active pulmonary tuberculosis. The location and patterns of pulmonary tuberculosis were examined on a thin section CT scan. Results: The most common thin section CT findings were subpleural nodules (n=6; mean diameter, 3.2 cm) and a lobar or segmental consolidation (n=3). The lesions were located most commonly in the right lower lobe (n=4). The incidence of tuberculosis in patients with idiopathic pulmonary fibrosis was more than five times higher than that of the general population. Conclusion: The atypical manifestation of pulmonary tuberculosis is common in patients with idiopathic pulmonary fibrosis, which may mimic lung cancer or bacterial pneumonia.

  2. Spectrum of pontocerebellar hypoplasia in 13 girls and boys with CASK mutations: confirmation of a recognizable phenotype and first description of a male mosaic patient

    Directory of Open Access Journals (Sweden)

    Burglen Lydie

    2012-03-01

    Full Text Available Abstract Background Pontocerebellar hypoplasia (PCH is a heterogeneous group of diseases characterized by lack of development and/or early neurodegeneration of cerebellum and brainstem. According to clinical features, seven subtypes of PCH have been described, PCH type 2 related to TSEN54 mutations being the most frequent. PCH is most often autosomal recessive though de novo anomalies in the X-linked gene CASK have recently been identified in patients, mostly females, presenting with intellectual disability, microcephaly and PCH (MICPCH. Methods Fourteen patients (12 females and two males; aged 16 months-14 years presenting with PCH at neuroimaging and with clinical characteristics unsuggestive of PCH1 or PCH2 were included. The CASK gene screening was performed using Array-CGH and sequencing. Clinical and neuroradiological features were collected. Results We observed a high frequency of patients with a CASK mutation (13/14. Ten patients (8 girls and 2 boys had intragenic mutations and three female patients had a Xp11.4 submicroscopic deletion including the CASK gene. All were de novo mutations. Phenotype was variable in severity but highly similar among the 11 girls and was characterized by psychomotor retardation, severe intellectual disability, progressive microcephaly, dystonia, mild dysmorphism, and scoliosis. Other signs were frequently associated, such as growth retardation, ophthalmologic anomalies (glaucoma, megalocornea and optic atrophy, deafness and epilepsy. As expected in an X-linked disease manifesting mainly in females, the boy hemizygous for a splice mutation had a very severe phenotype with nearly no development and refractory epilepsy. We described a mild phenotype in a boy with a mosaic truncating mutation. We found some degree of correlation between severity of the vermis hypoplasia and clinical phenotype. Conclusion This study describes a new series of PCH female patients with CASK inactivating mutations and confirms that

  3. Pulmonary arterial hypertension.

    Science.gov (United States)

    Montani, David; Günther, Sven; Dorfmüller, Peter; Perros, Frédéric; Girerd, Barbara; Garcia, Gilles; Jaïs, Xavier; Savale, Laurent; Artaud-Macari, Elise; Price, Laura C; Humbert, Marc; Simonneau, Gérald; Sitbon, Olivier

    2013-01-01

    Pulmonary arterial hypertension (PAH) is a chronic and progressive disease leading to right heart failure and ultimately death if untreated. The first classification of PH was proposed in 1973. In 2008, the fourth World Symposium on PH held in Dana Point (California, USA) revised previous classifications. Currently, PH is devided into five subgroups. Group 1 includes patients suffering from idiopathic or familial PAH with or without germline mutations. Patients with a diagnosis of PAH should systematically been screened regarding to underlying mutations of BMPR2 gene (bone morphogenetic protein receptor type 2) or more rarely of ACVRL1 (activine receptor-like kinase type 1), ENG (endogline) or Smad8 genes. Pulmonary veno occusive disease and pulmonary capillary hemagiomatosis are individualized and designated as clinical group 1'. Group 2 'Pulmonary hypertension due to left heart diseases' is divided into three sub-groups: systolic dysfonction, diastolic dysfonction and valvular dysfonction. Group 3 'Pulmonary hypertension due to respiratory diseases' includes a heterogenous subgroup of respiratory diseases like PH due to pulmonary fibrosis, COPD, lung emphysema or interstitial lung disease for exemple. Group 4 includes chronic thromboembolic pulmonary hypertension without any distinction of proximal or distal forms. Group 5 regroup PH patients with unclear multifactorial mechanisms. Invasive hemodynamic assessment with right heart catheterization is requested to confirm the definite diagnosis of PH showing a resting mean pulmonary artery pressure (mPAP) of ≥ 25 mmHg and a normal pulmonary capillary wedge pressure (PCWP) of ≤ 15 mmHg. The assessment of PCWP may allow the distinction between pre-capillary and post-capillary PH (PCWP > 15 mmHg). Echocardiography is an important tool in the management of patients with underlying suspicion of PH. The European Society of Cardiology and the European Respiratory Society (ESC-ERS) guidelines specify its role

  4. Pulmonary lymphangioleiomyomatosis as a pulmonary manifestation of tuberous sclerosis - a case report-

    International Nuclear Information System (INIS)

    Pulmonary lymphangioleiomyomatosis is a very rare disease mainly arising in reproductive-aged women. Pulmonary lymphangioleiomyomatosis as a pulmonary involvement of tuberous sclerosis is found in only 1 out of 100 patients. Pulmonary involvement in pulmonary lymphangioleiomyomatosis itself and that as a pulmonary manifestation of tuberous sclerosis has been considered very similar with regard to clinical, radiologic, and pathologic manifestations. We report 1 case of pulmonary lymphangioleiomyomatosis as a pulmonary manifestation of tuberous sclerosis in a 39-year-old Korean woman

  5. When a pulmonary embolism is not a pulmonary embolism: a rare case of primary pulmonary leiomyosarcoma

    Science.gov (United States)

    Muganlinskaya, Nargiz; Guzman, Amanda; Dahagam, Chanukya; Selinger, Stephen R.

    2015-01-01

    Arterial leiomyosarcomas account for up to 21% of vascular leiomyosarcomas, with 56% of arterial leiomyosarcomas occurring in the pulmonary artery. While isolated cases of primary pulmonary artery leiomyosarcoma document survival up to 36 months after treatment, these uncommon, aggressive tumors are highly lethal, with 1-year survival estimated at 20% from the onset of symptoms. We discuss a rare case of a pulmonary artery leiomyosarcoma that was originally diagnosed as a pulmonary embolism (PE). A 72-year-old Caucasian female was initially diagnosed with ‘saddle pulmonary embolism’ based on computerized tomographic angiography of the chest 2 months prior to admission and placed on anticoagulation. Dyspnea escalated, and serial computed tomography scans showed cardiomegaly with pulmonary emboli involving the right and left main pulmonary arteries with extension into the right and left upper and lower lobe branches. An echocardiogram on admission showed severe pulmonary hypertension with a pulmonary artery pressure of 82.9 mm Hg, and a severely enlarged right ventricle. Respiratory distress and multiorgan failure developed and, unfortunately, the patient expired. Autopsy showed a lobulated, yellow mass throughout the main pulmonary arteries measuring 13 cm in diameter. The mass extended into the parenchyma of the right upper lobe. On microscopy, the mass was consistent with a high-grade primary pulmonary artery leiomyosarcoma. Median survival of patients with primary pulmonary artery leiomyosarcoma without surgery is one and a half months, and mortality is usually due to right-sided heart failure. Pulmonary artery leiomyosarcoma is a rare but highly lethal disease commonly mistaken for PE. Thus, we recommend clinicians to suspect this malignancy when anticoagulation fails to relieve initial symptoms. In conclusion, early detection and suspicion of pulmonary artery leiomyosarcoma should be considered in patients refractory to anticoagulation, prompting initiation

  6. When a pulmonary embolism is not a pulmonary embolism: a rare case of primary pulmonary leiomyosarcoma

    Directory of Open Access Journals (Sweden)

    Nargiz Muganlinskaya

    2015-12-01

    Full Text Available Arterial leiomyosarcomas account for up to 21% of vascular leiomyosarcomas, with 56% of arterial leiomyosarcomas occurring in the pulmonary artery. While isolated cases of primary pulmonary artery leiomyosarcoma document survival up to 36 months after treatment, these uncommon, aggressive tumors are highly lethal, with 1-year survival estimated at 20% from the onset of symptoms. We discuss a rare case of a pulmonary artery leiomyosarcoma that was originally diagnosed as a pulmonary embolism (PE. A 72-year-old Caucasian female was initially diagnosed with ‘saddle pulmonary embolism’ based on computerized tomographic angiography of the chest 2 months prior to admission and placed on anticoagulation. Dyspnea escalated, and serial computed tomography scans showed cardiomegaly with pulmonary emboli involving the right and left main pulmonary arteries with extension into the right and left upper and lower lobe branches. An echocardiogram on admission showed severe pulmonary hypertension with a pulmonary artery pressure of 82.9 mm Hg, and a severely enlarged right ventricle. Respiratory distress and multiorgan failure developed and, unfortunately, the patient expired. Autopsy showed a lobulated, yellow mass throughout the main pulmonary arteries measuring 13 cm in diameter. The mass extended into the parenchyma of the right upper lobe. On microscopy, the mass was consistent with a high-grade primary pulmonary artery leiomyosarcoma. Median survival of patients with primary pulmonary artery leiomyosarcoma without surgery is one and a half months, and mortality is usually due to right-sided heart failure. Pulmonary artery leiomyosarcoma is a rare but highly lethal disease commonly mistaken for PE. Thus, we recommend clinicians to suspect this malignancy when anticoagulation fails to relieve initial symptoms. In conclusion, early detection and suspicion of pulmonary artery leiomyosarcoma should be considered in patients refractory to anticoagulation

  7. Current practice for pulmonary hypertension

    Institute of Scientific and Technical Information of China (English)

    Toru Satoh

    2014-01-01

    Objective To investigate the current practice of pulmonary hypertension including current epidemiology,diagnosis and treatment.Data sources The review was based on data obtained from the published articles and guidelines.Study selection Articles with high level of evidence or current best evidence in each issue were selected to be reviewed.Results Overall prevalence of pulmonary hypertension was 0.3% to 6% with left heart disease occupying the most proportion,followed by pulmonary disease,pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension.In diagnosis,a flow diagram of diagnosis of pulmonary hypertension,differential diagnosis of pulmonary hypertension and how to determine the severity of pulmonary hypertension are explained including recent development of magnetic resonance imaging and gene abnormality study on bone morphogenetic protein receptor Ⅱ.In treatment,newlydeveloped pulmonary vasodilators and the way to use them are shown to treat pulmonary hypertension.Conclusion Safer and more effective treatment algorithm and basic researches and clinical trials are warranted to be explored.

  8. Pneumococcal Vaccine and Patients with Pulmonary Diseases

    OpenAIRE

    Mirsaeidi, Mehdi; Ebrahimi, Golnaz; Allen, Mary Beth; Aliberti, Stefano

    2014-01-01

    Chronic pulmonary diseases describe chronic diseases that affect the airways and lung parenchyma. Examples of common chronic pulmonary diseases include asthma, bronchiectasis, chronic obstructive lung disease, lung fibrosis, sarcoidosis, pulmonary hypertension and cor pulmonale. Pulmonary infection is considered a significant cause of mortality in patients with chronic pulmonary diseases. Streptococcus pneumoniae is the leading isolated bacteria from adult patients with community-acquired pne...

  9. Pulmonary histiocytosis X - imaging aspects of pulmonary involvement

    International Nuclear Information System (INIS)

    Pulmonary histiocytosis X is an idiopathic disease which is and uncommon but important cause of pulmonary fibrosis in young adults. Chest radiographs and high resolution computed tomographic (HRCT) scans of the lungs of 7 patients diagnosed as pulmonary histiocytosis X were examined retrospectively. The authors reviewed the pathologic, clinical and radiographic features of pulmonary histiocytosis X, focusing on differential diagnosis and disease progression. Pulmonary histiocytosis X can be suspected on the basis of chest radiographic findings; predominantly upper lobe nodules and cysts present an increased sensitivity and are virtually pathognomonic of this disorder. Chest HRCT allows good assessment of the evolution of pulmonary histiocytosis X and is also valuable in distinguishing histiocytosis from other disorders that produces nodules or cysts. (author)

  10. Solitary pulmonary nodule by pulmonary hematoma under warfarin therapy

    International Nuclear Information System (INIS)

    Pulmonary hematoma is a rare cause of a pulmonary nodule. Mostly it results from penetrating or blunt chest injuries. The case of a patient is reported, whose chest X-ray showed a pulmonary nodule suspected of malignancy. This patient was maintained permanently on anticoagulants (warfarin derivates) after cardiac valve replacement with a prosthesis. A definite diagnosis could not be established by non-invasive methods. A needle biopsy of the lung was impracticable because of the location of the pulmonary lesion; an exploratory thoracotomy could not be carried out due to a general indication of nonoperability. Control examinations showed that the pulmonary nodule had vanished completely within four months. In consideration of the patient's clinical situation it can be concluded that the pulmonary lesion was caused by a hematoma of the lung. (orig.)

  11. Pulmonary Impedance and Pulmonary Doppler Trace in the Perioperative Period.

    Science.gov (United States)

    Tousignant, Claude; Van Orman, Jordan R

    2015-09-01

    Pulmonary hypertension and associated vascular changes may frequently accompany left-sided heart disease in the adult cardiac surgical population. Perioperative assessment of right ventricular function using echocardiography is well established. In general, understanding the constraints upon which the right ventricle must work is mostly limited to invasive monitoring consisting of pulmonary artery pressures, cardiac output, and pulmonary vascular resistance. The latter 2 measurements assume constant (mean) flows and pressures. The systolic and diastolic pressures offer a limited understanding of the pulsatile constraints, which may become significant in disease. In normal physiology, pressure and flow waves display near-similar contours. When left atrial pressure and pulmonary vascular resistance are increased, changes in pulmonary arterial compliance will result in elevated impedance to right ventricular ejection. Pressure reflections, the result of strong reflectors, return more quickly in a noncompliant system. They augment pulmonary artery pressure causing a premature reduction in flow. As a result, pressure and flow waves will now be dissimilar. The impact of vascular changes on right ventricular ejection can be assessed using pulmonary artery Doppler spectral imaging. The normal flow velocity profile is rounded at its peak. Earlier peaks and premature reductions in flow will make it appear more triangular. In some cases, the flow pattern may appear notched. The measurement of acceleration time, the time from onset to peak flow velocity is an indicator of constraint to ejection; shortened times have been associated with increased pulmonary vascular resistance and pressure. Understanding the changes in the pulmonary arterial system in disease and the physics of the hemodynamic alterations are essential in interpreting pulmonary artery Doppler data. Analyzing pulmonary artery Doppler flow signals may assist in the evaluation of right ventricular function in

  12. Persistent diffuse pulmonary interstitial emphysema mimicking pulmonary emphysema

    OpenAIRE

    Demura, Y; Ishizaki, T; Nakanishi, M; Ameshima, S; Itoh, H.

    2009-01-01

    A 69-year-old male non-smoker with a history of atopic asthma presented with symptoms suggestive of chronic obstructive pulmonary disease and this appeared to be corroborated by lung function testing and a chest radiograph. However, a chest CT showed no evidence of pulmonary emphysema and instead demonstrated free air along the bronchovascular sheaths indicative of pulmonary interstistial emphysema, possibly caused by repeated prior exacerbations of asthma. His lung function tests and symptom...

  13. Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology

    OpenAIRE

    Humbert, M.

    2010-01-01

    Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are two of the key subgroups of pulmonary hypertension. They are characterised by different risk factors. PAH can be associated with mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2), HIV infection, congenital heart disease, connective tissue disease (such as systemic sclerosis), and exposure to particular drugs and toxins including fenfluramine derivatives. In cont...

  14. Molecular Mechanisms of Pulmonary Vascular Remodeling in Pulmonary Arterial Hypertension

    Science.gov (United States)

    Leopold, Jane A.; Maron, Bradley A.

    2016-01-01

    Pulmonary arterial hypertension (PAH) is a devastating disease that is precipitated by hypertrophic pulmonary vascular remodeling of distal arterioles to increase pulmonary artery pressure and pulmonary vascular resistance in the absence of left heart, lung parenchymal, or thromboembolic disease. Despite available medical therapy, pulmonary artery remodeling and its attendant hemodynamic consequences result in right ventricular dysfunction, failure, and early death. To limit morbidity and mortality, attention has focused on identifying the cellular and molecular mechanisms underlying aberrant pulmonary artery remodeling to identify pathways for intervention. While there is a well-recognized heritable genetic component to PAH, there is also evidence of other genetic perturbations, including pulmonary vascular cell DNA damage, activation of the DNA damage response, and variations in microRNA expression. These findings likely contribute, in part, to dysregulation of proliferation and apoptosis signaling pathways akin to what is observed in cancer; changes in cellular metabolism, metabolic flux, and mitochondrial function; and endothelial-to-mesenchymal transition as key signaling pathways that promote pulmonary vascular remodeling. This review will highlight recent advances in the field with an emphasis on the aforementioned molecular mechanisms as contributors to the pulmonary vascular disease pathophenotype. PMID:27213345

  15. Molecular Mechanisms of Pulmonary Vascular Remodeling in Pulmonary Arterial Hypertension

    Directory of Open Access Journals (Sweden)

    Jane A. Leopold

    2016-05-01

    Full Text Available Pulmonary arterial hypertension (PAH is a devastating disease that is precipitated by hypertrophic pulmonary vascular remodeling of distal arterioles to increase pulmonary artery pressure and pulmonary vascular resistance in the absence of left heart, lung parenchymal, or thromboembolic disease. Despite available medical therapy, pulmonary artery remodeling and its attendant hemodynamic consequences result in right ventricular dysfunction, failure, and early death. To limit morbidity and mortality, attention has focused on identifying the cellular and molecular mechanisms underlying aberrant pulmonary artery remodeling to identify pathways for intervention. While there is a well-recognized heritable genetic component to PAH, there is also evidence of other genetic perturbations, including pulmonary vascular cell DNA damage, activation of the DNA damage response, and variations in microRNA expression. These findings likely contribute, in part, to dysregulation of proliferation and apoptosis signaling pathways akin to what is observed in cancer; changes in cellular metabolism, metabolic flux, and mitochondrial function; and endothelial-to-mesenchymal transition as key signaling pathways that promote pulmonary vascular remodeling. This review will highlight recent advances in the field with an emphasis on the aforementioned molecular mechanisms as contributors to the pulmonary vascular disease pathophenotype.

  16. Pulmonary sarcoidosis: management.

    Directory of Open Access Journals (Sweden)

    Sharma O

    2002-04-01

    Full Text Available During the last two decades many advances have been made in the field of sarcoidosis. The disease is now recognised as a multisystem disorder occurring in patients with a genetic predisposition and an exposure to yet unknown transmissible environmental agent/s. The diagnosis is based on a compatible clinical and/or radiological picture, histological evidence of non-caseating granuloma and exclusion of other diseases capable of producing a similar clinical or histological picture. Treatment primarily consists of administration of corticosteroids, although there are valuable alternative drugs. Treatment should be considered in symptomatic patients with evidence of radiologic or lung function deterioration. The patients with extra-pulmonary involvement particularly with ocular, myocardial, and neuro-sarcoidosis almost always need treatment. For asymptomatic pulmonary sarcoidosis patients no therapy is needed.

  17. Pulmonary alveolar proteinosis

    International Nuclear Information System (INIS)

    Since the original description of pulmonary alveolar proteinosis (PAP) as bilaterally symmetric, confluent, perihilar infiltrates, no large series evaluating the relative frequency of radiographic findings has been published. The authors reviewed 53 histologically proved cases and found a predominantly confluent pattern in 77.4%; of these, 61% had a nodular component. A predominantly nodular pattern was seen in 22.6% often with some coalescence or reticular shadows. Distribution was perhilar 56.6%, diffuse in 26.4%, basilar in 26.4%, and asymmetric in 35.8%. The classic pattern was seen in 18.9%. The authors conclude that the spectrum of patterns in pulmonary alveolar proteinosis is wider than generally appreciated

  18. Obstructive pulmonary disease

    International Nuclear Information System (INIS)

    Although /sup 133/xenon ventilation studies and aerosol inhalation images have been shown to be more sensitive than other measurements of pulmonary function in detecting early airway disease, they fall short as ideal screening tests. In addition they do not distinguish between the different diseases of air flow obstruction. The diagnosis of the conditions discussed in this paper does not require studies of regional lung function. It is doubtful if further refinements in instrumentation or the introduction of other radioisotopes would really help in this matter. Chronic bronchitis, emphysema, and bronchial asthma are the major obstructive pulmonary disease. Cystic fibrosis and bronchiectasis, which are less common, are also accompanied by airway obstruction. Local obstruction of a bronchus may also occur as a result of a tumor or foreign body or a mucous plug

  19. [Pulmonary Langerhans cell histiocytosis].

    Science.gov (United States)

    Popper, H H

    2015-09-01

    Pulmonary Langerhans cell histiocytosis is regarded as a reactive proliferation of the dendritic Langerhans cell population stimulated by chronic tobacco-derived plant proteins due to incomplete combustion but can also occur in childhood as a tumor-like systemic disease. Currently, both these forms cannot be morphologically distinguished. In the lungs a nodular proliferation of Langerhans cells occurs in the bronchial mucosa and also peripherally in the alveolar septa with an accompanying infiltration by eosinophilic granulocytes and destruction of the bronchial wall. Langerhans cells can be selectively detected with antibodies against CD1a and langerin. In the reactive isolated pulmonary form, abstinence from tobacco smoking in most patients leads to regression of infiltration and improvement of symptoms. In high-resolution computed tomography (HRCT) the small star-like scars can still be detected even after complete cessation of tobacco smoking. PMID:26289803

  20. Pulmonary interstitial glycogenosis

    Energy Technology Data Exchange (ETDEWEB)

    Lanfranchi, Michael [Creighton University Medical School, Children' s Hospital and Medical Center, Omaha, NE (United States); Allbery, Sandra M.; Wheelock, Lisa [Children' s Hospital and Medical Center, Department of Radiology, Omaha, NE (United States); Perry, Deborah [Children' s Hospital and Medical Center, Department of Pathology, Omaha, NE (United States)

    2010-03-15

    Although bronchopulmonary dysplasia (BPD) is a common cause of interstitial lung disease in chronically intubated premature neonates, other interstitial lung disease in nonintubated infants is rare. We present a case of pulmonary interstitial glycogenosis that developed in a nonintubated, 31-week gestation infant in whom infectious etiologies had been excluded. The infant was well initially and then developed respiratory distress at 18 days of life. Radiographs at first day of life were normal, but CT and radiographic findings at 18 days of life showed severe interstitial lung disease, mimicking BPD. Lung biopsy showed pulmonary interstitial glycogenosis. This entity is not well described in the pediatric radiology literature and is important to consider, as the condition is responsive to a course of corticosteroids. (orig.)

  1. Isolated Pulmonary Valve Endocarditis

    Directory of Open Access Journals (Sweden)

    Mohammad Ali Hatamizadeh

    2009-06-01

    Full Text Available Infective endocarditis is one of the most severe complications of parenteral drug abuse. The outstanding clinical feature of infective endocarditis in intravenous drug abusers is the high incidence of right-sided valve infection, and the tricuspid valve is involved in 60% to 70% of the cases. We herein report a case of isolated pulmonic valve infective endocarditis with a native pulmonary valve.

  2. Thromboembolic chronicle pulmonary Hypertension

    International Nuclear Information System (INIS)

    The thromboembolic chronicle pulmonary Hypertension, also well known as chronic lung thromboembolism not resolved; it is a form not very common of lung thromboembolism. Until very recently was considered a rare curiosity of autopsy, but as the methods of diagnoses have improved and our attitude has changed, the incidence of this nosological entity has experienced a notable increment, but the most excellent in this illness is maybe that it is a form of lung hypertension, potentially recoverable

  3. Primary pulmonary cryptococcosis

    OpenAIRE

    Asadi Gharabaghi, Mehrnaz; Allameh, Seyyed Farshad

    2014-01-01

    Here, we report a case of primary cryptococcal pneumonia in a 25-year-old woman who presented with several weeks’ history of cough, dyspnoea and night sweating. These symptoms started in the third trimester of her pregnancy. She was being treated for infertility and got pregnant with in vitro fertilisation. On chest imaging, there were bilateral air space consolidation and cavitary lesions. Fungal pulmonary infection was diagnosed after surgical lung biopsy. She received fluconazole 400 mg pe...

  4. Idiopathic Pulmonary Haemosiderosis

    OpenAIRE

    Gaurav Vishal; Ekta Dalal; Neelam Raval

    2013-01-01

    We report a case of idiopathic pulmonary hemosiderosis (IPH) in a 8year old female who presented with anaemia, breathlessness, puffiness of face and grade 4 clubbing. She had been investigated a number of times at multiple hos-pitals and had received multiple blood transfusions considering iron deficiency anaemia. Diagnosis of IPH was sus-pected on basis of clinical history of persistent respiratory distress, hypoxemia, repeated anaemia episodes and diffuse bilateral infiltrates on chest X ra...

  5. Diagnostics for pulmonary tuberculosis

    Science.gov (United States)

    Cudahy, Patrick

    2016-01-01

    Tuberculosis (TB) remains a leading cause of human suffering and mortality despite decades of effective treatment being available. Accurate and timely diagnosis remains an unmet goal. The HIV epidemic has also led to new challenges in the diagnosis of TB. Several new developments in TB diagnostics have the potential to positively influence the global campaign against TB. We aim to review the performance of both established as well as new diagnostics for pulmonary TB in adults, and discuss the ongoing challenges. PMID:27005271

  6. Pulmonary remodeling in asthma

    OpenAIRE

    Lieberman, Phil

    2010-01-01

    The inflammatory and immunologic processes responsible for asthma can produce permanently fixed obstructive lung disease unresponsive to medical therapy. This can be manifested clinically by the failure of a childhood asthmatic to reach full expected lung capacity at adulthood and by an accelerated decline in pulmonary capacity in adults. Recent studies have furthered our insight into the pathologic processes underlying these changes and the potential effects of therapy to prevent them.

  7. Chronic obstructive pulmonary disease

    OpenAIRE

    NR Anthonisen

    2007-01-01

    The global prevalence of physiologically defined chronic obstructive pulmonary disease (COPD) in adults aged >40 yr is approximately 9-10 per cent. Recently, the Indian Study on Epidemiology of Asthma, Respiratory Symptoms and Chronic Bronchitis in Adults had shown that the overall prevalence of chronic bronchitis in adults >35 yr is 3.49 per cent. The development of COPD is multifactorial and the risk factors of COPD include genetic and environmental factors. Pathological changes in COPD are...

  8. Diagnostics for pulmonary tuberculosis

    OpenAIRE

    Cudahy, Patrick; Shenoi, Sheela

    2016-01-01

    Tuberculosis (TB) remains a leading cause of human suffering and mortality despite decades of effective treatment being available. Accurate and timely diagnosis remains an unmet goal. The HIV epidemic has also led to new challenges in the diagnosis of TB. Several new developments in TB diagnostics have the potential to positively influence the global campaign against TB. We aim to review the performance of both established as well as new diagnostics for pulmonary TB in adults, and discuss the...

  9. Pulmonary alveolar proteinosis

    OpenAIRE

    Patel, Sandeep M; Sekiguchi, Hiroshi; Jordan P Reynolds; Krowka, Michael J.

    2012-01-01

    Pulmonary alveolar proteinosis (PAP) is a disease of alveolar accumulation of phospholipoproteinaceous material that results in gas exchange impairment leading to dyspnea and alveolar infiltrates. There are three forms of PAP: congenital, acquired and idiopathic; of which the latter two are predominant in the adult population. Previous case studies have found that the acquired form can be secondary to various autoimmune, infectious, malignant and environmental etiologies. Recent advances in t...

  10. Pulmonary Alveolar Proteinosis

    OpenAIRE

    Patel, Sandeep M; Hiroshi Sekiguchi; Jordan P Reynolds; Krowka, Michael J.

    2012-01-01

    Pulmonary alveolar proteinosis (PAP) is a disease of alveolar accumulation of phospholipoproteinaceous material that results in gas exchange impairment leading to dyspnea and alveolar infiltrates. There are three forms of PAP: congenital, acquired and idiopathic; of which the latter two are predominant in the adult population. Previous case studies have found that the acquired form can be secondary to various autoimmune, infectious, malignant and environmental etiologies. Recent advances in t...

  11. Pulmonary langerhans cell histiocytosis

    OpenAIRE

    Suri Harpreet S; Yi Eunhee S; Nowakowski Gregorz S; Vassallo Robert

    2012-01-01

    Abstract Pulmonary Langerhans Cell Histiocytosis (PLCH) is a relatively uncommon lung disease that generally, but not invariably, occurs in cigarette smokers. The pathologic hallmark of PLCH is the accumulation of Langerhans and other inflammatory cells in small airways, resulting in the formation of nodular inflammatory lesions. While the overwhelming majority of patients are smokers, mechanisms by which smoking induces this disease are not known, but likely involve a combination of events r...

  12. Transcatheter Pulmonary Valve Replacement

    OpenAIRE

    Ghawi, Hani; Kenny, Damien; Hijazi, Ziyad M.

    2012-01-01

    Transcatheter pulmonary valve replacement (tPVR) has evolved into a viable alternative to surgical conduit or bioprosthetic valve replacement. This procedure has paved the way for a more advanced approach to congenital and structural interventional cardiology. Although many successes have been noted, there are still a number of challenges with this procedure, including large delivery systems, the need for a conduit or a bioprosthetic valve as a landing zone for the valve, optimal timing of th...

  13. Pulmonary lymphangiomyomatosis. Pulmonale Lymphangiomyomatose

    Energy Technology Data Exchange (ETDEWEB)

    Fischbach, R.; Neufang, K.F.R. (Koeln Univ. (Germany, F.R.). Inst. und Poliklinik fuer Radiologische Diagnostik); Deutsch, H.J. (Koeln Univ. (Germany, F.R.). Klinik 3 fuer Innere Medizin); Feaux de Lacroix, W. (Koeln Univ. (Germany, F.R.). Pathologisches Inst.)

    1991-01-01

    The case of a woman of 48 years of age suffering from pulmonary lymphangiomyomatosis is presented. Chest radiographs showed the reticular infiltrate of diffuse interstitial lung disease, whereas high resolution CT ilustrated multiple, well-defined cystic lesions distributed diffusely through the lungs without fibrotic changes or nodular densities. The patient underwent open lung biopsy and pleurodesis because of recurrent pneumothorax. Treatment with medroxy-progesterone was initiated. (orig.).

  14. Pulmonary pseudotumours and asbestos

    International Nuclear Information System (INIS)

    Ten patients are described in whom a localized reaction in the visceral pleura developed comparatively rapidly, involving parts of the underlying lung parenchyma, forming an atelectatic pseudotumour. The lesions were mistaken for pulmonary malignancy, leading to thoracotomy in 5 patients. It is suggested that these lesions can occur many years after exposure to asbestos and that they are sequelae of benign pleurisy. Computer tomography is of great value in these cases and may obviate thoracotomy. (Auth.)

  15. Pulmonary arterial hypertension.

    OpenAIRE

    Montani, David; Günther, Sven; Dorfmüller, Peter; Perros, Frédéric; Girerd, Barbara; Garcia, Gilles; Jaïs, Xavier; Savale, Laurent; Artaud-Macari, Elise; Price, Laura; Humbert, Marc; Simonneau, Gérald; Sitbon, Olivier

    2013-01-01

    Pulmonary arterial hypertension (PAH) is a chronic and progressive disease leading to right heart failure and ultimately death if untreated. The first classification of PH was proposed in 1973. In 2008, the fourth World Symposium on PH held in Dana Point (California, USA) revised previous classifications. Currently, PH is devided into five subgroups. Group 1 includes patients suffering from idiopathic or familial PAH with or without germline mutations. Patients with a diagnosis of PAH should ...

  16. Digital angiography in pulmonary embolism

    International Nuclear Information System (INIS)

    Pulmonary digital subtraction angiography was diagnostic in 98.3% of patients with possible acute pulmonary embolism. The procedure was well tolerated even in severely ill patients. A large image intensifier made simultaneous imaging of both lungs possible reducing the number of contrast injections necessary. Small volumes of low iso-osmolar concentration of modern contrast media were used. There was no need for catherization of the pulmonary artery. Theoretical considerations and our limited experience indicate that this will reduce the number of complications compared with conventional pulmonary angiography. The procedure is rapidly performed and the diagnostic accuracy high. This makes digital subtraction angiography cost effective. Digital pulmonary angiography can be recommended as the primary diagnostic method in most patients with possible pulmonary embolism. (orig.)

  17. Pulmonary Artery Denervation Reduces Pulmonary Artery Pressure and Induces Histological Changes in an Acute Porcine Model of Pulmonary Hypertension

    OpenAIRE

    Rothman, A.M.K.; Arnold, N D; Chang, W.; Watson, O.; Swift, A J; Condliffe, R; Elliot, C A; Kiely, D. G.; Suvarna, S K; Gunn, J.; Lawrie, A.

    2015-01-01

    Background— Pulmonary arterial hypertension is a devastating disease with high morbidity and mortality and limited treatment options. Recent studies have shown that pulmonary artery denervation improves pulmonary hemodynamics in an experimental model and in an early clinical trial. We aimed to evaluate the nerve distribution around the pulmonary artery, to determine the effect of radiofrequency pulmonary artery denervation on acute pulmonary hypertension induced by vasoconstriction, and to de...

  18. Pulmonary emboli risk reduction.

    Science.gov (United States)

    Epley, D

    2000-06-01

    Pulmonary embolism is a major cause of death in hospitalized patients in the United States. Significant morbidity is a characteristic of this phenomenon and its common antecedent, deep venous thrombosis. Research has shown that pulmonary embolism is rarely a consequence of superficial venous thrombosis. Because the signs and symptoms of these disorders are often nonspecific and not readily apparent, prevention is the goal of patient care. Nurses play a critical role in the prevention process. The recognition of persons at risk, the assessment of these patients for early signs and symptoms, and the institution of prophylactic measures to minimize the effect of any existing factors or to deter their development are essential components of nursing care. The administration of anticoagulation therapy, with the concomitant monitoring of its effectiveness, and the education of persons regarding precautions associated with the indicated medications and relevant lifestyle modifications are also fundamental aspects of care. This article provides a review of risk factors associated with pulmonary embolism and deep venous thrombosis, particularly prolonged immobility and surgery involving the abdomen or lower extremities, as well as a review of the respective etiologies, most notably those encompassing Vichow's triad. In addition, current modalities to diagnose, treat, and prevent these allied disorders are discussed, as well as the associated nursing management. PMID:11249288

  19. Pulmonary fungal infections.

    Science.gov (United States)

    Smith, Jeannina A; Kauffman, Carol A

    2012-08-01

    This review details some of the advances that have been made in the recent decade in the diagnosis, treatment and epidemiology of pulmonary fungal infections. These advances have occurred because of increasing knowledge regarding the fungal genome, better understanding of the structures of the fungal cell wall and cell membrane and the use of molecular epidemiological techniques. The clinical implications of these advances are more rapid diagnosis and more effective and less toxic antifungal agents. For example, the diagnosis of invasive pulmonary aspergillosis, as well as histoplasmosis and blastomycosis, has improved with the use of easily performed antigen detection systems in serum and bronchoalveolar lavage fluid. Treatment of angioinvasive moulds has improved with the introduction of the new azoles, voriconazole and posaconazole that have broad antifungal activity. Amphotericin B is less frequently used, and when used is often given as lipid formulation to decrease toxicity. The newest agents, the echinocandins, are especially safe as they interfere with the metabolism of the fungal cell wall, a structure not shared with humans cells. Epidemiological advances include the description of the emergence of Cryptococcus gattii in North America and the increase in pulmonary mucormycosis and pneumonia due to Fusarium and Scedosporium species in transplant recipients and patients with haematological malignancies. The emergence of azole resistance among Aspergillus species is especially worrisome and is likely related to increased azole use for treatment of patients, but also to agricultural use of azoles as fungicides in certain countries. PMID:22335254

  20. Pulmonary thromboembolism diagnosis algorithms

    Energy Technology Data Exchange (ETDEWEB)

    Kasai, Takeshi; Eto, Jun; Hayano, Daisuke; Ohashi, Masaki; Yoneda, Takahiro; Oyama, Hisaya; Inaba, Akira [Kameda General Hospital, Kamogawa, Chiba (Japan). Trauma and Emergency Care Center

    2002-01-01

    Our algorithm for diagnosing pulmonary thromboembolism combines ventilation/perfusion scanning with clinical criteria. Our perfusion scanning criterion states that high probability defines 2 segmental perfusion defects without corresponding radiographic abnormality and indeterminate probability defines less than 2 segmental perfusion defects (low probability: less than one segmental perfusion defect; intermediate: perfusion defects between high and low probability). The clinical criterion is divided into 7 items related to symptoms and signs suggestive of pulmonary thromboembolism. More than 4 items are defined as a highly suspicious clinical manifestation (HSCM), and less than 4 are considered a low suspicious clinical manifestation (LSCM). In 31 cases of high probability, 18 of HSCM did not include pulmonary angiograhy (PAG), and 13 of LSCM included PAG (positive: 11; negative: 2). In 12 cases of indeterminate probability, 7 of LSCM were observed without PAG and 5 of HSCM with PAG (positive: 4; negative: 1). PAG performance thus decreased to 41.9%. The positive prediction of high probability is 93.5%, which is very high, compared to indeterminate probability at 33.3%. (author)

  1. Pulmonary alveolar microlithiasis

    International Nuclear Information System (INIS)

    Pulmonary alveolar microlithiasis (PAM) is a rare disease characterized by the diffuse and bilateral presence of calcium phosphate microlite in the alveolar spaces. The progression of this potentially lethal disease is show and most of the patients remain asymptomatic during years or decades, resulting in a show deterioration of the pulmonary function. The typical finding of the sand storm in the chest X-ray is characteristic of this entity. Mutations in the SLC34A2 gene that does the coding for the type II co-transporter of sodium phosphate were identified as responsible for this disease. Of the almost 600 cases, only 6 have been reported in Colombia. We are presenting a case of pulmonary alveolar microlite in a 27 year old man, with progressive respiratory distress whose diagnosis was made by the X-ray findings and confirmed by trans bronchial biopsy. In the 2 years follow-up, shows evolution towards deterioration of his respiratory function making him a candidate for lung transplantation.

  2. Radiologic diagnosis of pulmonary dirofilariasis

    Energy Technology Data Exchange (ETDEWEB)

    Akimoto, Manabu; Takashima, Tsutomu; Kamimura, Ryoichi; Itoh, Hiroshi; Suzuki, Masayuki; Arakawa, Fumitaka; Watanabe, You; Miyata, Samon.

    1988-01-01

    Radiologic evaluation of pulmonary dirofilariasis was made in five pathologically verified cases. In all cases, the lesion was shown to be well-defined, non-calcified solitary pulmonary nodule less than 2 cm in diameter, with smooth margin in subpleural location. The pulmonary vessels toward the tumor were well shown on tomogram and CT without convergence. These findings are fairly characteristic of the disease, but it is still difficult to make a definitive differentiation from the malignant lesion by radiologic study alone.

  3. Pulmonary edema: radiographic differential diagnosis

    International Nuclear Information System (INIS)

    To evaluate the feasibility of using chest radiography to differentiate between three different etiologies of pulmonary edema. Plain chest radiographs of 77 patients, who were clinically confirmed as having pulmonary edema, were retrospectively reviewed. The patients were classified into three groups : group 1 (cardiogenic edema : n = 35), group 2 (renal pulmonary edema : n = 16) and group 3 (permeability edema : n = 26). We analyzed the radiologic findings of air bronchogram, heart size, peribronchial cuffing, septal line, pleural effusion, vascular pedicle width, pulmonary blood flow distribution and distribution of pulmonary edema. In a search for radiologic findings which would help in the differentiation of these three etiologies, each finding was assessed. Cardiogenic and renal pulmonary edema showed overlapping radiologic findings, except for pulmonary blood flow distribution. In cardiogenic pulmonary edema (n=35), cardiomegaly (n=29), peribronchial cuffing (n=29), inverted pulmonary blood flow distribution (n=21) and basal distribution of edema (n=20) were common. In renal pulmonary edema (n=16), cardiomegaly (n=15), balanced blood flow distribution (n=12), and central (n=9) or basal distribution of edema (n=7) were common. Permeability edema (n=26) showed different findings. Air bronchogram (n=25), normal blood flow distribution (n=14) and peripheral distribution of edema (n=21) were frequent findings, while cardiomegaly (n=7), peribronchial cuffing (n=7) and septal line (n=5) were observed in only a few cases. On plain chest radiograph, permeability edema can be differentiated from cardiogenic or renal pulmonary edema. The radiographic findings which most reliably differentiated these two etiologies were air bronchogram, distribution of pulmonary edema, peribronchial cuffing and heart size. Only blood flow distribution was useful for radiographic differentiation of cardiogenic and renal edema

  4. Genetics Home Reference: pulmonary arterial hypertension

    Science.gov (United States)

    ... Home Health Conditions pulmonary arterial hypertension pulmonary arterial hypertension Enable Javascript to view the expand/collapse boxes. ... All Open All Close All Description Pulmonary arterial hypertension is a progressive disorder characterized by abnormally high ...

  5. Sickle Cell Disease and Pulmonary Hypertension

    Science.gov (United States)

    Sickle Cell Disease Pulmonary & PH Hypertension Did you know that if you have Sickle Cell Disease you are at risk for Pulmonary ... or PH, is a complex and often misunderstood disease. Pulmonary hypertension means high blood pressure that is located ...

  6. Multimodality imaging of pulmonary infarction

    International Nuclear Information System (INIS)

    Highlights: • A plethora of pulmonary and systemic disorders, often associated with grave outcomes, may cause pulmonary infarction. • A stereotypical infarct is a peripheral wedge shaped pleurally based opacity but imaging findings can be highly variable. • Multimodality imaging is key to diagnosing the presence, aetiology and complications of pulmonary infarction. • Multimodality imaging of pulmonary infarction together with any ancillary features often guide to early targeted treatment. • CT remains the principal imaging modality with MRI increasingly used alongside nuclear medicine studies and ultrasound. - Abstract: The impact of absent pulmonary arterial and venous flow on the pulmonary parenchyma depends on a host of factors. These include location of the occlusive insult, the speed at which the occlusion develops and the ability of the normal dual arterial supply to compensate through increased bronchial arterial flow. Pulmonary infarction occurs when oxygenation is cut off secondary to sudden occlusion with lack of recruitment of the dual supply arterial system. Thromboembolic disease is the commonest cause of such an insult but a whole range of disease processes intrinsic and extrinsic to the pulmonary arterial and venous lumen may also result in infarcts. Recognition of the presence of infarction can be challenging as imaging manifestations often differ from the classically described wedge shaped defect and a number of weighty causes need consideration. This review highlights aetiologies and imaging appearances of pulmonary infarction, utilising cases to illustrate the essential role of a multimodality imaging approach in order to arrive at the appropriate diagnosis

  7. Multimodality imaging of pulmonary infarction

    Energy Technology Data Exchange (ETDEWEB)

    Bray, T.J.P., E-mail: timothyjpbray@gmail.com [Department of Radiology, Papworth Hospital NHS Foundation Trust, Ermine Street, Papworth Everard, Cambridge CB23 3RE (United Kingdom); Mortensen, K.H., E-mail: mortensen@doctors.org.uk [Department of Radiology, Papworth Hospital NHS Foundation Trust, Ermine Street, Papworth Everard, Cambridge CB23 3RE (United Kingdom); University Department of Radiology, Addenbrookes Hospital, Cambridge University Hospitals NHS Foundation Trust, Hills Road, Box 318, Cambridge CB2 0QQ (United Kingdom); Gopalan, D., E-mail: deepa.gopalan@btopenworld.com [Department of Radiology, Papworth Hospital NHS Foundation Trust, Ermine Street, Papworth Everard, Cambridge CB23 3RE (United Kingdom)

    2014-12-15

    Highlights: • A plethora of pulmonary and systemic disorders, often associated with grave outcomes, may cause pulmonary infarction. • A stereotypical infarct is a peripheral wedge shaped pleurally based opacity but imaging findings can be highly variable. • Multimodality imaging is key to diagnosing the presence, aetiology and complications of pulmonary infarction. • Multimodality imaging of pulmonary infarction together with any ancillary features often guide to early targeted treatment. • CT remains the principal imaging modality with MRI increasingly used alongside nuclear medicine studies and ultrasound. - Abstract: The impact of absent pulmonary arterial and venous flow on the pulmonary parenchyma depends on a host of factors. These include location of the occlusive insult, the speed at which the occlusion develops and the ability of the normal dual arterial supply to compensate through increased bronchial arterial flow. Pulmonary infarction occurs when oxygenation is cut off secondary to sudden occlusion with lack of recruitment of the dual supply arterial system. Thromboembolic disease is the commonest cause of such an insult but a whole range of disease processes intrinsic and extrinsic to the pulmonary arterial and venous lumen may also result in infarcts. Recognition of the presence of infarction can be challenging as imaging manifestations often differ from the classically described wedge shaped defect and a number of weighty causes need consideration. This review highlights aetiologies and imaging appearances of pulmonary infarction, utilising cases to illustrate the essential role of a multimodality imaging approach in order to arrive at the appropriate diagnosis.

  8. Invasive hemodynamic assessment of pulmonary hypertension

    OpenAIRE

    Pagnamenta, Alberto

    2015-01-01

    The diagnosis of pulmonary hypertension requires an invasive confirmation of an elevated mean pulmonary artery pressure during a right heart catheterization. The present thesis reviews the invasive hemodynamic approaches to assess the functional state of the pulmonary circulation and its impact on right ventricular function in pulmonary vascular diseases. Pulmonary vascular resistance is better characterized by multi-point pressure/flow measurements. The occlusion analysis of the pulmonary ar...

  9. Eosinofilia pulmonar Pulmonary eosinophilia

    Directory of Open Access Journals (Sweden)

    Luiz Eduardo Mendes Campos

    2009-06-01

    Full Text Available As formas de eosinofilia pulmonar constituem um grupo heterogêneo definido pela presença de um ou dois critérios: infiltrado pulmonar com eosinofilia sanguínea e/ou eosinofilia tissular caracterizada por eosinófilos demonstrados na biópsia pulmonar ou no lavado broncoalveolar. Embora o infiltrado inflamatório seja composto de macrófagos, linfócitos, neutrófilos e eosinófilos, a presença de eosinofilia é um marcador importante para o diagnóstico e tratamento. A apresentação clínica e radiológica pode revelar eosinofilia pulmonar simples, pneumonia eosinofílica crônica, pneumonia eosinofílica aguda, aspergilose broncopulmonar alérgica e eosinofilia pulmonar associada à doença sistêmica, como na síndrome de Churg-Strauss e na síndrome hipereosinofílica. A asma está frequentemente associada, podendo ser um pré-requisito, como na aspergilose broncopulmonar alérgica e na síndrome de Churg-Strauss. Nas doenças com acometimento sistêmico, a pele, o coração e o sistema nervoso são os órgãos mais comprometidos. A apresentação radiológica pode ser considerada como típica, ou pelo menos sugestiva, para três formas de eosinofilia pulmonar: pneumonia eosinofílica crônica, aspergilose broncopulmonar alérgica e pneumonia eosinofílica aguda. A etiologia da eosinofilia pulmonar pode ser de causa primária (idiopática ou secundária, compreendendo causas conhecidas, como drogas, parasitas, infecções por fungos e micobactérias, irradiação e toxinas. A eosinofilia pulmonar pode também estar associada a doenças pulmonares difusas, doenças do tecido conectivo e neoplasias.Pulmonary eosinophilia comprises a heterogeneous group of diseases defined by eosinophilia in pulmonary infiltrates (bronchoalveolar lavage fluid or in tissue (lung biopsy specimens. Although the inflammatory infiltrate is composed of macrophages, lymphocytes, neutrophils and eosinophils, eosinophilia is an important marker for the diagnosis

  10. Unexplained childhood anaemia: idiopathic pulmonary hemosiderosis.

    Science.gov (United States)

    Siu, K K; Li, Rever; Lam, S Y

    2015-04-01

    This report demonstrates pulmonary haemorrhage as a differential cause of anaemia. Idiopathic pulmonary hemosiderosis is a rare disease in children; it is classically described as a triad of haemoptysis, pulmonary infiltrates on chest radiograph, and iron-deficiency anaemia. However, anaemia may be the only presenting feature of idiopathic pulmonary hemosiderosis in children due to occult pulmonary haemorrhage. In addition, the serum ferritin is falsely high in idiopathic pulmonary hemosiderosis which increases the diagnostic difficulty. We recommend that pulmonary haemorrhage be suspected in any child presenting with iron-deficiency anaemia and persistent bilateral pulmonary infiltrates. PMID:25904566

  11. Genetics Home Reference: pulmonary alveolar microlithiasis

    Science.gov (United States)

    ... Genetics Home Health Conditions pulmonary alveolar microlithiasis pulmonary alveolar microlithiasis Enable Javascript to view the expand/collapse boxes. Print All Open All Close All Description ...

  12. Pulmonary MRA: Differentiation of pulmonary embolism from truncation artefact

    Energy Technology Data Exchange (ETDEWEB)

    Bannas, Peter [University of Wisconsin-Madison, Department of Radiology, Madison, WI (United States); University Hospital Hamburg-Eppendorf, Department of Radiology, Hamburg (Germany); Schiebler, Mark L.; Motosugi, Utaroh; Francois, Christopher J. [University of Wisconsin-Madison, Department of Radiology, Madison, WI (United States); Reeder, Scott B. [University of Wisconsin-Madison, Department of Radiology, Madison, WI (United States); University of Wisconsin-Madison, Department of Biomedical Engineering, Madison, WI (United States); University of Wisconsin-Madison, Department of Medical Physics, Madison, WI (United States); University of Wisconsin-Madison, Department of Medicine, Madison, WI (United States); Nagle, Scott K. [University of Wisconsin-Madison, Department of Radiology, Madison, WI (United States); University of Wisconsin-Madison, Department of Medical Physics, Madison, WI (United States); University of Wisconsin-Madison, Department of Pediatrics, Madison, WI (United States)

    2014-08-15

    Truncation artefact (Gibbs ringing) causes central signal drop within vessels in pulmonary magnetic resonance angiography (MRA) that can be mistaken for emboli, reducing diagnostic accuracy for pulmonary embolism (PE). We propose a quantitative approach to differentiate truncation artefact from PE. Twenty-eight patients who underwent pulmonary computed tomography angiography (CTA) for suspected PE were recruited for pulmonary MRA. Signal intensity drops within pulmonary arteries that persisted on both arterial-phase and delayed-phase MRA were identified. The percent signal loss between the vessel lumen and central drop was measured. CTA served as the reference standard for presence of pulmonary emboli. A total of 65 signal intensity drops were identified on MRA. Of these, 48 (74 %) were artefacts and 17 (26 %) were PE, as confirmed by CTA. Truncation artefacts had a significantly lower median signal drop than PE on both arterial-phase (26 % [range 12-58 %] vs. 85 % [range 53-91 %]) and delayed-phase MRA (26 % [range 11-55 %] vs. 77 % [range 47-89 %]), p < 0.0001 for both. Receiver operating characteristic (ROC) analyses revealed a threshold value of 51 % (arterial phase) and 47 % signal drop (delayed phase) to differentiate between truncation artefact and PE with 100 % sensitivity and greater than 90 % specificity. Quantitative signal drop is an objective tool to help differentiate truncation artefact and pulmonary embolism in pulmonary MRA. (orig.)

  13. Pulmonary comorbidities associated with chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    Fabrizio Luppi

    2012-12-01

    Full Text Available Introduction Chronic obstructive pulmonary disease (COPD is characterized by a relatively irreversible airflow limitation caused by chronic inflammation, in most cases tobacco-related. The impact of COPD on morbidity and mortality at the single-patient level depends upon the severity of COPD symptoms and the existence of other types of systemic and/or pulmonary disease, also known as co-morbid conditions.Materials and methods This review examines the pulmonary diseases commonly associated with COPD, in terms of their prevalence, clinical features, pathogenic mechanisms, prognoses, and implications for management of COPD.Results The incidence and prevalence of various pulmonary diseases are significantly increased in patients with COPD. These conditions include symptomatic bronchiectasis, combined pulmonary fibrosis and emphysema, lung cancer, sleep-related respiratory disorders, and pulmonary embolism. Some of these concomitant respiratory diseases have an independent negative impact on the prognosis of COPD patients, and their presence has important implications for treatment of these patients.Conclusions Physicians treating patients with COPD need to be aware of these coexisting pulmonary diseases. All patients with COPD should be carefully evaluated to identify pulmonary comorbidities, since they not only influence the prognosis but also have an impact on disease management. The treatment of COPD is no longer restricted exclusively to inhaled therapy. The therapeutic approach to this disease is becoming increasingly multidimensional in view of the fact that successful management of comorbidities might positively affect the course of COPD itself.

  14. Pulmonary MRA: Differentiation of pulmonary embolism from truncation artefact

    International Nuclear Information System (INIS)

    Truncation artefact (Gibbs ringing) causes central signal drop within vessels in pulmonary magnetic resonance angiography (MRA) that can be mistaken for emboli, reducing diagnostic accuracy for pulmonary embolism (PE). We propose a quantitative approach to differentiate truncation artefact from PE. Twenty-eight patients who underwent pulmonary computed tomography angiography (CTA) for suspected PE were recruited for pulmonary MRA. Signal intensity drops within pulmonary arteries that persisted on both arterial-phase and delayed-phase MRA were identified. The percent signal loss between the vessel lumen and central drop was measured. CTA served as the reference standard for presence of pulmonary emboli. A total of 65 signal intensity drops were identified on MRA. Of these, 48 (74 %) were artefacts and 17 (26 %) were PE, as confirmed by CTA. Truncation artefacts had a significantly lower median signal drop than PE on both arterial-phase (26 % [range 12-58 %] vs. 85 % [range 53-91 %]) and delayed-phase MRA (26 % [range 11-55 %] vs. 77 % [range 47-89 %]), p < 0.0001 for both. Receiver operating characteristic (ROC) analyses revealed a threshold value of 51 % (arterial phase) and 47 % signal drop (delayed phase) to differentiate between truncation artefact and PE with 100 % sensitivity and greater than 90 % specificity. Quantitative signal drop is an objective tool to help differentiate truncation artefact and pulmonary embolism in pulmonary MRA. (orig.)

  15. Chronic obstructive pulmonary disease

    Directory of Open Access Journals (Sweden)

    V K Vijayan

    2013-01-01

    Full Text Available The global prevalence of physiologically defined chronic obstructive pulmonary disease (COPD in adults aged >40 yr is approximately 9-10 per cent. Recently, the Indian Study on Epidemiology of Asthma, Respiratory Symptoms and Chronic Bronchitis in Adults had shown that the overall prevalence of chronic bronchitis in adults >35 yr is 3.49 per cent. The development of COPD is multifactorial and the risk factors of COPD include genetic and environmental factors. Pathological changes in COPD are observed in central airways, small airways and alveolar space. The proposed pathogenesis of COPD includes proteinase-antiproteinase hypothesis, immunological mechanisms, oxidant-antioxidant balance, systemic inflammation, apoptosis and ineffective repair. Airflow limitation in COPD is defined as a postbronchodilator FEV1 (forced expiratory volume in 1 sec to FVC (forced vital capacity ratio <0.70. COPD is characterized by an accelerated decline in FEV1. Co morbidities associated with COPD are cardiovascular disorders (coronary artery disease and chronic heart failure, hypertension, metabolic diseases (diabetes mellitus, metabolic syndrome and obesity, bone disease (osteoporosis and osteopenia, stroke, lung cancer, cachexia, skeletal muscle weakness, anaemia, depression and cognitive decline. The assessment of COPD is required to determine the severity of the disease, its impact on the health status and the risk of future events (e.g., exacerbations, hospital admissions or death and this is essential to guide therapy. COPD is treated with inhaled bronchodilators, inhaled corticosteroids, oral theophylline and oral phosphodiesterase-4 inhibitor. Non pharmacological treatment of COPD includes smoking cessation, pulmonary rehabilitation and nutritional support. Lung volume reduction surgery and lung transplantation are advised in selected severe patients. Global strategy for the diagnosis, management and prevention of Chronic Obstructive Pulmonary Disease

  16. Pulmonary arteriovenous malformations.

    Science.gov (United States)

    Shovlin, Claire L

    2014-12-01

    Within the past decade, pulmonary arteriovenous malformations (PAVMs) have evolved from rare curiosities to not uncommon clinical states, with the latest estimates suggesting a prevalence of ~1 in 2,600. PAVMs provide anatomic right-to-left shunts, allowing systemic venous blood to bypass gas exchange and pulmonary capillary bed processing. Hypoxemia and enhanced ventilatory demands result, although both are usually asymptomatic. Paradoxical emboli lead to strokes and cerebral abscesses, and these commonly occur in individuals with previously undiagnosed PAVMs. PAVM hemorrhage is rare but is the main cause of maternal death in pregnancy. PAVM occlusion by embolization is the standard of care to reduce these risks. However, recent data demonstrate that currently recommended management protocols can result in levels of radiation exposure that would be classified as harmful. Recent publications also provide a better appreciation of the hematologic and cardiovascular demands required to maintain arterial oxygen content and oxygen consumption in hypoxemic patients, identify patient subgroups at higher risk of complications, and emphasize the proportion of radiologically visible PAVMs too small to treat by embolization. This review, therefore, outlines medical states that exacerbate the consequences of PAVMs. Chief among these is iron deficiency, which is commonly present due to concurrent hereditary hemorrhagic telangiectasia: iron deficiency impairs hypoxemia compensations by restricting erythropoiesis and increases the risk of ischemic strokes. Management of periodontal disease, dental interventions, pulmonary hypertension, and pregnancy also requires specific consideration in the setting of PAVMs. The review concludes by discussing to what extent previously recommended protocols may benefit from modification or revision. PMID:25420112

  17. Hyperthyroidism and pulmonary hypertension.

    Science.gov (United States)

    Marvisi, M; Brianti, M; Marani, G; Del Borello, R; Bortesi, M L; Guariglia, A

    2002-04-01

    In recent years, many authors have described several cases revealing an association between hyperthyroidism and pulmonary hypertension (PH). This observational study was designed to evaluate the incidence of PH in hyperthyroidism and was set in a department of internal medicine and pulmonary diseases with an out-patients department of endocrinology. Thirty-four patients, 25 women and nine men, with a mean age of 38 +/- 15 SD years participated. Twenty had Graves' disease and 14 had a nodular goitre. The patients were divided into two equally matched groups: those with a recently diagnosed hyperthyroidism, taking no drugs (group 1; n = 17) and those in a euthyroid state taking methimazole (group 2; n= 17). Transthoracic Doppler echocardiography was performed and systolic pulmonary artery pressurements of (PAPs) was determined by the tricuspid regurgitation method using the Bernoulli equation. Measurements of triiodothyronine, tetraiodothyronine, free thyroxine (Ft4), thyroid-stimulating hormone (TSH) and antithyroglobulin and antimicrosomal antibodies were also taken. We found a mild PH in seven patients of group 1 and in none of group 2. The mean +/- SD systolic pulmonaryartery pressurewas 28.88 +/- 6.41 in group 1 and 22.53 +/- 1.84 ingroup 2 (P<0.0001). A correlation was found between the TSH value and PAPs (r = -082;P < 0.001) and Ft4 and PAPs (r = 0 85; P < 0.001) in group 1. These findings indicate the presence of a frequent association between PH and hyperthyroidism. We suggest that hyperthyroidism be included in the differential diagnosis of PH. PMID:11999999

  18. Pulmonary function in microgravity

    Science.gov (United States)

    Guy, H. J.; Prisk, G. K.; West, J. B.

    1992-01-01

    We report the successful collection of a large quantity of human resting pulmonary function data on the SLS-1 mission. Preliminary analysis suggests that cardiac stroke volumes are high on orbit, and that an adaptive reduction takes at least several days, and in fact may still be in progress after 9 days on orbit. It also suggests that pulmonary capillary blood volumes are high, and remain high on orbit, but that the pulmonary interstitium is not significantly impacted. The data further suggest that the known large gravitational gradients of lung function have only a modest influence on single breath tests such as the SBN washout. They account for only approximately 25% of the phase III slope of nitrogen, on vital capacity SBN washouts. These gradients are only a moderate source of the cardiogenic oscillations seen in argon (bolus gas) and nitrogen (resident gas), on such tests. They may have a greater role in generating the normal CO2 oscillations, as here the phase relationship to argon and nitrogen reverses in microgravity, at least at mid exhalation in those subjects studied to date. Microgravity may become a useful tool in establishing the nature of the non-gravitational mechanisms that can now be seen to play such a large part in the generation of intra-breath gradients and oscillations of expired gas concentration. Analysis of microgravity multibreath nitrogen washouts, single breath washouts from more physiological pre-inspiratory volumes, both using our existing SLS-1 data, and data from the upcoming D-2 and SLS-2 missions, should be very fruitful in this regard.(ABSTRACT TRUNCATED AT 250 WORDS).

  19. Imaging of pulmonary tuberculosis

    International Nuclear Information System (INIS)

    Tuberculosis, more than any other infectious disease, has always been a challenge, since it has been responsible for a great amount of morbidity and mortality in humans. After a steady decline in the number of new cases during the twentieth century, due to improved social and environmental conditions, early diagnosis, and the development of antituberculous medication, a stagnation and even an increase in the number of new cases was noted in the mid-1980s. The epidemiological alteration is multifactorial: global increase in developing countries; minority groups (HIV and other immunocompromised patients); and elderly patients due to an altered immune status. Other factors that may be responsible are a delayed diagnosis, especially in elderly patients, incomplete or inadequate therapy, and the emergence of multidrug-resistant tuberculosis. The course of the disease and its corresponding clinicoradiological pattern depends on the interaction between the organism and the host response. Classically, pulmonary tuberculosis has been classified in primary tuberculosis, which occurred previously in children, and postprimary tuberculosis, occurring in adult patients. In industrialized countries, however, there seems to be a shift of primary tuberculosis towards adults. Furthermore, due to an altered immunological response in certain groups, such as immunocompromised and elderly patients, an atypical radioclinical pattern may occur. The changing landscape, in which tuberculosis occurs, as well as the global resurgence, and the changed spectrum of the clinical and radiological presentation, justify a renewed interest of radiologists for the imaging features of pulmonary tuberculosis. This article deals with the usual imaging features of pulmonary tuberculosis as well as the atypical patterns encountered in immunodepressed and elderly patients. (orig.)

  20. Drug-induced pulmonary disease

    Science.gov (United States)

    ... medlineplus.gov/ency/article/000104.htm Drug-induced pulmonary disease To use the sharing features on this page, ... take longer to improve. Some drug-induced lung diseases, such as pulmonary fibrosis, may never go away. Possible Complications Complications ...

  1. Thrombolytic therapy in pulmonary embolism.

    LENUS (Irish Health Repository)

    Nagi, D

    2010-01-01

    Massive pulmonary embolism carries a high mortality. Potential treatment includes anticoagulation, thrombolytic therapy and embolectomy. We report a case of deep vein thrombosis leading to progressive massive pulmonary embolism despite appropriate anticoagulation, where thrombolysis with IVC filter placement resulted in a successful outcome.

  2. Tobacco smoking and pulmonary tuberculosis

    OpenAIRE

    Kolappan, C.; Gopi, P

    2002-01-01

    Background: The prevalence of tuberculosis in adult men in India is 2–4 times higher than in women. Tobacco smoking is prevalent almost exclusively among men, so it is possible that tobacco smoking may be a risk factor for developing pulmonary tuberculosis. A nested case control study was carried out to study the association between tobacco smoking and pulmonary tuberculosis.

  3. [Idiopathic Pulmonary Fibrosis].

    Science.gov (United States)

    Prasse, A

    2015-10-01

    Idiopathic pulmonary fibrosis (IPF) is the most common idiopathic interstitial pneumonia and a disease of the elderly. Cigarette smoking and longterm exposure to substances harming alveolar epithelial cells are risk factors for the development of IPF. There is also evidence for a genetic susceptibility. IPF is defined as the idiopathic variant of Usual Interstitial Pneumonitis (UIP). Diagnosis of IPF is complex and based on the exclusion of other diseases associated with an UIP pattern. The only cure is lung transplantation. In the last years there was a breakthrough in the treatment of IPF. With pirfenidone and nintedanib there are now two compounds approved for the treatment of IPF. PMID:26444136

  4. Idiopathic Pulmonary Haemosiderosis

    Directory of Open Access Journals (Sweden)

    Gaurav Vishal

    2013-06-01

    Full Text Available We report a case of idiopathic pulmonary hemosiderosis (IPH in a 8year old female who presented with anaemia, breathlessness, puffiness of face and grade 4 clubbing. She had been investigated a number of times at multiple hos-pitals and had received multiple blood transfusions considering iron deficiency anaemia. Diagnosis of IPH was sus-pected on basis of clinical history of persistent respiratory distress, hypoxemia, repeated anaemia episodes and diffuse bilateral infiltrates on chest X ray. [Natl J Med Res 2013; 3(3.000: 292-293

  5. Microarray analysis in pulmonary hypertension.

    Science.gov (United States)

    Hoffmann, Julia; Wilhelm, Jochen; Olschewski, Andrea; Kwapiszewska, Grazyna

    2016-07-01

    Microarrays are a powerful and effective tool that allows the detection of genome-wide gene expression differences between controls and disease conditions. They have been broadly applied to investigate the pathobiology of diverse forms of pulmonary hypertension, namely group 1, including patients with idiopathic pulmonary arterial hypertension, and group 3, including pulmonary hypertension associated with chronic lung diseases such as chronic obstructive pulmonary disease and idiopathic pulmonary fibrosis. To date, numerous human microarray studies have been conducted to analyse global (lung homogenate samples), compartment-specific (laser capture microdissection), cell type-specific (isolated primary cells) and circulating cell (peripheral blood) expression profiles. Combined, they provide important information on development, progression and the end-stage disease. In the future, system biology approaches, expression of noncoding RNAs that regulate coding RNAs, and direct comparison between animal models and human disease might be of importance. PMID:27076594

  6. Congenital pulmonary lymphangiectasia

    Directory of Open Access Journals (Sweden)

    Campisi Corradino

    2006-10-01

    Full Text Available Abstract Congenital pulmonary lymphangiectasia (PL is a rare developmental disorder involving the lung, and characterized by pulmonary subpleural, interlobar, perivascular and peribronchial lymphatic dilatation. The prevalence is unknown. PL presents at birth with severe respiratory distress, tachypnea and cyanosis, with a very high mortality rate at or within a few hours of birth. Most reported cases are sporadic and the etiology is not completely understood. It has been suggested that PL lymphatic channels of the fetal lung do not undergo the normal regression process at 20 weeks of gestation. Secondary PL may be caused by a cardiac lesion. The diagnostic approach includes complete family and obstetric history, conventional radiologic studies, ultrasound and magnetic resonance studies, lymphoscintigraphy, lung functionality tests, lung biopsy, bronchoscopy, and pleural effusion examination. During the prenatal period, all causes leading to hydrops fetalis should be considered in the diagnosis of PL. Fetal ultrasound evaluation plays a key role in the antenatal diagnosis of PL. At birth, mechanical ventilation and pleural drainage are nearly always necessary to obtain a favorable outcome of respiratory distress. Home supplemental oxygen therapy and symptomatic treatment of recurrent cough and wheeze are often necessary during childhood, sometimes associated with prolonged pleural drainage. Recent advances in intensive neonatal care have changed the previously nearly fatal outcome of PL at birth. Patients affected by PL who survive infancy, present medical problems which are characteristic of chronic lung disease.

  7. Pulmonary alveolar proteinosis

    Directory of Open Access Journals (Sweden)

    B. Crestani

    2011-06-01

    Full Text Available Pulmonary alveolar proteinosis (PAP is a rare pulmonary disease characterised by alveolar accumulation of surfactant. It may result from mutations in surfactant proteins or granulocyte macrophage-colony stimulating factor (GM-CSF receptor genes, it may be secondary to toxic inhalation or haematological disorders, or it may be auto-immune, with anti-GM-CSF antibodies blocking activation of alveolar macrophages. Auto-immune alveolar proteinosis is the most frequent form of PAP, representing 90% of cases. Although not specific, high-resolution computed tomography shows a characteristic “crazy paving” pattern. In most cases, bronchoalveolar lavage findings establish the diagnosis. Whole lung lavage is the most effective therapy, especially for auto-immune disease. Novel therapies targeting alveolar macrophages (recombinant GM-CSF therapy or anti-GM-CSF antibodies (rituximab and plasmapheresis are being investigated. Our knowledge of the pathophysiology of PAP has improved in the past 20 yrs, but therapy for PAP still needs improvement.

  8. Pulmonary alveolar proteinosis

    Directory of Open Access Journals (Sweden)

    Hadice Selimoğlu Şen

    2011-06-01

    Full Text Available Pulmonary alveolar proteinosis is a rare disease characterized by the accumulation of lipoproteinosis material within the alveoli. A 36 age male patient applied to our hospital with dyspnea that began 5 months ago. Bilateral diffuse infiltration on postero-anterior chest graphy was seen. Arterial blood gas measurements (ABG was: pH:7.44, pCO2: 36 mmHg, pO2: 49 mmHg, SaO2: 85%, HCO3: 24 mmol/L. High resolution computed tomography (HRCT reported as bilateral diffuse ground glass areas and interlobuler septal thickening on this areas. Asido resistant bacillus was negative in sputum at three times and was also negative in post bronchoscopic sputum and bronchoalveoler lavage material. Wedge resection was made by video-assisted thoracoscopic surgery. Histopathology report of biopsy material revealed “Pulmonary alveolar proteinosis (PAP”. Massive lung lavage under general anesthesia was planned for patient. Clinical improvement was seen in patient beginning from admission and ABG measurements entered the recovery trend in follow-up. Partial pressure of oxygen was increased to 65 mmHg and patient was followed without lung lavage. Control chest radiography and HRCT showed significant radiological improvement. After three months, radiological lesions had completely regressed at control HRCT. As a result, in patients with symptoms and radiologically bilateral diffuse infiltration physicians should consider PAP as a rare disease in the differential diagnosis.

  9. Pulmonary emphysema and smoking

    International Nuclear Information System (INIS)

    We assessed the relation between PE and smoking in 1,563 cases (1,068 men and 495 women) who underwent CT scaring for suspicion of respiratory disease on chest radiograph or some respiratory complaints. PE was diagnosed by the existence of low attenuation areas in CT scan and not by pulmonary function tests. CT was performed with 10 mm collimation in a standard algorithm. There were 2 subtypes of pulmonary emphysema: centrilobular and paraseptal emphysema. PE, regardless of the grade, was seen: in 189 out of 348 (54.3%) cases in males smokers and in only 2 out of 63 (3.2%) cases in male non-smokers; and in 5 out of 25 (20.0%) in female smokers and in 4 out of 203 (2.0%) in female non-smokers. PE was observed in more than half of male smokers. High incidence of PE was also observed in even younger generation, and severity would progress with advancing age and smoking. Both types of emphysema progress with age and amount of cigarette smoking. (author)

  10. Pulmonary Langerhans cell Histiocytosis.

    Science.gov (United States)

    Harari, S; Comel, A

    2001-10-01

    Pulmonary Langerhans cell Histiocytosis is a rare granulomatous disease affecting both sexes, with greater incidence in the second and third decades of life; smoking appears to be the most important risk factor. Its etiology is unknown, although there are data indicating an uncontrolled immune response as possible cause, sustained by the Langerhans cells, antigen presenting cells for T lymphocytes, and their accumulation in the distal bronchioles; these cells express on their surface the CD1a and CD1c antigens, and the B7 molecule, essential for activating quiescent T lymphocytes. In its evolution the granuloma is characterized by the progressive reduction in the LC number, with the increase of fibrosis, surrounding and destroying the bronchiolar lumen; the remaining of the lumen, or the traction exerted by fibrous tissue on the adjacent alveolar spaces leads to the cyst development. Vascular involvement occurs frequently, and may explain the onset of pulmonary hypertension in advanced cases of the disease. The disease may be asymptomatic, or it may present with aspecific respiratory signs and symptoms, and has characteristic radiological findings, being included in the group of cysticaerial parenchymal alterations. The diagnosis could be suggested by the finding of a number of LC in BAL greater than 5%. Different therapies have been proposed, but it seems that the most important measure is smoking cessation. PMID:11587096

  11. Pulmonary manifestation of leptospirosis

    International Nuclear Information System (INIS)

    Authors analysed and present chest X-ray findings of serologically proven leptospirosis from Seoul National University Hospital, either admitted or referred for serological verification, during recent 2 years. Radiological findings were correlated with the lung specimen findings of experimentally induced leptospirosis in guinea pig. The results are as follows: 1. 24 cases (56%) showed positive X-ray findings. 2. Predominant radiological patterns of involved lung were tiny dot, small nodule, rosette density in 11 cases, massive confluent consolidation in 4 cases, and diffuse ill-defined velly increased density in 9 cases. 3. Distribution of pulmonary lesions were bilateral (100%), non-lobar, non-segmental (95%), and there were conspicuous tendency of peripheral lung predominance. 4. Extrapulmonary manifestation, such as pleural effusion or cardiomegaly was rate. 5. Pulmonary lesions resolved completely usually 5 to 10 days after their appearance. 6. From the gross and microscopic findings of serially sacrificed guinea pig's lung and a case of autopsy, authors concluded that fine dot-like density in chest X-ray was due to paleolithic hemorrhage in intraalveolar space at initial stage, growing up to ressette density or confluent consolidation as the pathetic extends to surrounding lung forming purpura and coalescent hemorrhage.

  12. Pulmonary manifestation of leptospirosis

    Energy Technology Data Exchange (ETDEWEB)

    Im, Jung Gi; Yeon, Kyung Mo; Han, Man Chung; Kim, Chu Wan; Lee, Jung Sang; Kim, Suhng Gwon; Han, Yong Chol; Chang, Woo Hyun; Chi, Je Geun [Seoul National University College of Medicine, Seoul (Korea, Republic of)

    1986-02-15

    Authors analysed and present chest X-ray findings of serologically proven leptospirosis from Seoul National University Hospital, either admitted or referred for serological verification, during recent 2 years. Radiological findings were correlated with the lung specimen findings of experimentally induced leptospirosis in guinea pig. The results are as follows: 1. 24 cases (56%) showed positive X-ray findings. 2. Predominant radiological patterns of involved lung were tiny dot, small nodule, rosette density in 11 cases, massive confluent consolidation in 4 cases, and diffuse ill-defined velly increased density in 9 cases. 3. Distribution of pulmonary lesions were bilateral (100%), non-lobar, non-segmental (95%), and there were conspicuous tendency of peripheral lung predominance. 4. Extrapulmonary manifestation, such as pleural effusion or cardiomegaly was rate. 5. Pulmonary lesions resolved completely usually 5 to 10 days after their appearance. 6. From the gross and microscopic findings of serially sacrificed guinea pig's lung and a case of autopsy, authors concluded that fine dot-like density in chest X-ray was due to paleolithic hemorrhage in intraalveolar space at initial stage, growing up to ressette density or confluent consolidation as the pathetic extends to surrounding lung forming purpura and coalescent hemorrhage.

  13. [Pulmonary sarcomatoid carcinoma].

    Science.gov (United States)

    Antoine, Martine; Vieira, Thibault; Fallet, Vincent; Hamard, Cécile; Duruisseaux, Michael; Cadranel, Jacques; Wislez, Marie

    2016-01-01

    Pulmonary sarcomatoid carcinomas are a rare group of tumors accounting for about one percent of non-small cell lung carcinoma (NSCLC). In 2015, the World Health Organization classification united under this name all the carcinomas with sarcomatous-like component with spindle cell or giant cell appearance, or associated with a sarcomatous component sometimes heterologous. There are five subtypes: pleomorphic carcinoma, spindle cell carcinoma, giant cell carcinoma, carcinosarcoma and pulmonary blastoma. Clinical characteristics are not specific from the other subtypes of NSCLC. Epithelial to mesenchymal transition pathway may play a key role. Patients, usually tobacco smokers, are frequently symptomatic. Tumors are voluminous more often peripherical than central, with strong fixation on FDG TEP CT. Distant metastases are frequent with atypical visceral locations. These tumors have poorer prognosis than the other NSCLC subtypes because of great aggressivity, and frequent chemoresistance. Here we present pathological description and a review of literature with molecular features in order to better describe these tumors and perhaps introduce new therapeutics. PMID:26778815

  14. HIPOPLASIA CEREBELAR E PORENCEFALIA EM BOVINOS CHAROLÊS NO SUL DO RIO GRANDE DO SUL CEREBELLAR HYPOPLASIA AND PORENCEPHALY IN CHAROLAIS CATLLE IN SOUTHERN BRAZIL

    Directory of Open Access Journals (Sweden)

    Ana Lucia Schild

    2001-02-01

    Full Text Available Descrevem-se dois casos de hipoplasia cerebelar congênita observados em terneiros da raça Charolês, pertencentes a um estabelecimento localizado no município de Capão do Leão. Um dos animais apresentava sinais clínicos cerebelares ao nascimento, caracterizados por severa incoordenação motora, hipermetria e tremores de intenção, principalmente da cabeça e do pescoço. O outro animal não conseguia manter-se em estação e apresentava opistótono acentuado. As lesões macroscópicas caracterizaram-se por hipoplasia cerebelar, observando-se apenas remanescentes das regiões do flóculo e do paraflóculo cerebelares e uma cavidade preenchida por líquido e recoberta pelas leptomeninges, que estavam aderidas aos resquícios das folhas cerebelares. Ao corte do encéfalo, havia hidrocefalia discreta e a presença de cavidades císticas (porencefalia. São discutidas as possíveis etiologias, sugerindo-se a possibilidade de que a enfermidade tenha sido causada pelo vírus da diarréia viral bovina-doença das mucosas, apesar dos resultados negativos da imuno-histoquímica realizada em fragmentos do cerebelo dos animais doentes para detecção desse vírus.Two cases of cerebellar hypoplasia in Charolais calves from a herd of a farm located in the municipality of Capão do Leão, Southern Brazil are described. One calf with cerebellar clinical signs at birth, showed incoordination, hypermetria and intention tremors, mainly in the neck and head. The other calf was recumbent with opisthotonos. The gross lesions were characterized by cerebellar hypoplasia, with only remnants of cerebellar floculus and parafloculus and an expanded fluid-filled space covered by leptomeninges. Mild hydrocephalus and porencephaly were observed in the cerebrum. It was discussed the possible etiologies and suggested the possibility of the disease has been caused by bovine viral diarrhea-mucosal disease (BVD-MD virus despite of negative results of imuno

  15. Pulmonary Hypertension and Computed Tomography Measurement of Small Pulmonary Vessels in Severe Emphysema

    OpenAIRE

    Matsuoka, Shin; George R. Washko; Yamashiro, Tsuneo; Estepar, Raul San Jose; Diaz, Alejandro; Silverman, Edwin K.; Hoffman, Eric; Fessler, Henry E.; Criner, Gerard J.; Marchetti, Nathaniel; Scharf, Steven M; Martinez, Fernando J; Reilly, John J.; Hatabu, Hiroto

    2009-01-01

    Rationale: Vascular alteration of small pulmonary vessels is one of the characteristic features of pulmonary hypertension in chronic obstructive pulmonary disease. The in vivo relationship between pulmonary hypertension and morphological alteration of the small pulmonary vessels has not been assessed in patients with severe emphysema.

  16. Pulmonary alveolar microlithiasis with calcified pleural plaques

    OpenAIRE

    Malhotra Balbir; Sabharwal Raghu; Singh Mandeep; Singh Amarjeet

    2010-01-01

    Pulmonary alveolar microlithiasis (PAM) is a rare disease. Herein we report a case of pulmonary alveolar microlithiasis who was suspected to have the disease on chest X-ray and was confirmed on high resolution CT and transbronchial lung biopsy. These investigations showed characteristic features of pulmonary alveolar microlithiasis with diffuse interstitial pulmonary fibrosis.

  17. Celiac disease with pulmonary haemosiderosis and cardiomyopathy

    OpenAIRE

    Işikay, Sedat; Yilmaz, Kutluhan; Kilinç, Metin

    2012-01-01

    Celiac disease or pulmonary haemosiderosis can be associated with several distinguished conditions. Pulmonary haemosiderosis is a rare, severe and fatal disease characterised by recurrent episodes of alveolar haemorrhage, haemoptysis and anaemia. Association of pulmonary haemosiderosis and celiac disease is extremely rare. We describe a case of celiac disease presented with dilated cardiomyopathy and pulmonary haemosiderosis without gastrointestinal symptoms of celiac disease. In addition, vi...

  18. Unusual pulmonary findings in mucolipidosis II.

    Science.gov (United States)

    Ishak, Marleine; Zambrano, Eduardo V; Bazzy-Asaad, Alia; Esquibies, Americo E

    2012-07-01

    We report undescribed pulmonary findings in a child with mucolipidosis II (ML-II). Children with ML-II bear significant pulmonary morbidity that may include extensive pulmonary fibrosis, persistent hemosiderosis as well as pulmonary airway excrescences as they reach preschool age. PMID:22162509

  19. Pulmonary alveolar microlithiasis with calcified pleural plaques

    Directory of Open Access Journals (Sweden)

    Malhotra Balbir

    2010-01-01

    Full Text Available Pulmonary alveolar microlithiasis (PAM is a rare disease. Herein we report a case of pulmonary alveolar microlithiasis who was suspected to have the disease on chest X-ray and was confirmed on high resolution CT and transbronchial lung biopsy. These investigations showed characteristic features of pulmonary alveolar microlithiasis with diffuse interstitial pulmonary fibrosis.

  20. Non-congenital heart disease associated pediatric pulmonary arterial hypertension

    OpenAIRE

    Ivy, D. D.; Feinstein, J.A.; Humpl, T; Rosenzweig, E.B.

    2009-01-01

    Recognition of causes of pulmonary hypertension other than congenital heart disease is increasing in children. Diagnosis and treatment of any underlying cause of pulmonary hypertension is crucial for optimal management of pulmonary hypertension. This article discusses the available knowledge regarding several disorders associated with pulmonary hypertension in children: idiopathic pulmonary arterial hypertension (IPAH), pulmonary capillary hemangiomatosis, pulmonary veno-occlusive disease, he...

  1. Pulmonary complications in renal transplantation

    International Nuclear Information System (INIS)

    To evaluate the radiographic and CT findings of pulmonary complications other than pulmonary edema arising from renal transplantation. Among 393 patients who had undergone renal transplantation at our hospital during a previous ten-year period, 23 with pulmonary complications other than pulmonary edema were included in this study. The complications involved were infection caused by CMV (n=6), bacteria (n=4), fungus (n=4), tuberculosis (n=2), varicella (n=1) or chlamydia (n=1), and malignancy involving lung cancer (n=4) or Kaposi's sarcoma (n=1). Two chest radiologists reviewed all images. The complications manifesting mainly as pulmonary nodules were lung cancer (4/4), tuberculosis (1/2), and Kaposi's sarcoma (1/1). Pulmonary consolidation was a main feature in bacterial infection (4/4), fungal infection (3/4), tuberculosis (1/2), chlamydial infection (1/1), and varicellar pneumonia (1/1). Ground-glass attenuation was a main CT feature in CMV pneumonia (4/6), and increased interstitial making was a predominant radiographic feature in CMV pneumonia (2/6). The main radiologic features described above can be helpful for differential diagnosis of the pulmonary complications of renal transplantation

  2. Diagnosis of Transverse Sinus Hypoplasia in Magnetic Resonance Venography: New Insights Based on Magnetic Resonance Imaging in Combined Dataset of Venous Outflow Impairment Case-Control Studies: Post Hoc Case-Control Study.

    Science.gov (United States)

    Han, Ke; Chao, A-Ching; Chang, Feng-Chi; Hsu, Hung-Yi; Chung, Chih-Ping; Sheng, Wen-Yung; Chan, Lung; Wu, Jiang; Hu, Han-Hwa

    2016-03-01

    In previous studies of transverse sinus (TS) hypoplasia, discrepancies between TS diameter measured by magnetic resonance venography (MRV) and contrast T1-weighted magnetic resonance (contrast T1) were observed. To investigate these discrepancies, and considering that TS hypoplasia is associated with neurological disorders, we performed a post hoc analysis of prospectively collected data from 3 case-control studies on transient global amnesia (TGA), transient monocular blindness (TMB), and panic disorders while retaining the original inclusion and exclusion criteria. Magnetic resonance (MR) imaging of 131 subjects was reviewed to evaluate TS diameter and the location and degree of venous flow stenosis and obstruction.MRV without contrast revealed that TS hypoplasia was observed in 69 subjects, whom we classified into 2 subgroups according to the concordance with contrast T1 observations: concordance indicated anatomically small TS (30 subjects), and discrepancy indicated that the MRV diagnosis is in fact flow-related and that TS is not anatomically small (39 subjects). The latter subgroup was associated with at least 1 site of venous compression/stenosis in the internal jugular vein (IJV) or the left brachiocephalic vein (BCV) (P < 0.001), which was significantly larger in patients than controls. Compensatory dilatation of contralateral TS diameter was only observed with MRV, not with contrast T1 imaging.The clinical implication of these results is that using MRV only, IJV/BCV compression/stenosis may be misdiagnosed as TS hypoplasia. And contralateral TS have no compensatory dilatation in its diameter in contrast T1 imaging, just compensatory increased flow volume. PMID:26962781

  3. Idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Noble Paul W

    2008-03-01

    Full Text Available Abstract Idiopathic pulmonary fibrosis (IPF is a non-neoplastic pulmonary disease that is characterized by the formation of scar tissue within the lungs in the absence of any known provocation. IPF is a rare disease which affects approximately 5 million persons worldwide. The prevalence is estimated to be slightly greater in men (20.2/100,000 than in women (13.2/100,000. The mean age at presentation is 66 years. IPF initially manifests with symptoms of exercise-induced breathless and dry coughing. Auscultation of the lungs reveals early inspiratory crackles, predominantly located in the lower posterior lung zones upon physical exam. Clubbing is found in approximately 50% of IPF patients. Cor pulmonale develops in association with end-stage disease. In that case, classic signs of right heart failure may be present. Etiology remains incompletely understood. Some environmental factors may be associated with IPF (cigarette smoking, exposure to silica and livestock. IPF is recognized on high-resolution computed tomography by peripheral, subpleural lower lobe reticular opacities in association with subpleural honeycomb changes. IPF is associated with a pathological lesion known as usual interstitial pneumonia (UIP. The UIP pattern consists of normal lung alternating with patches of dense fibrosis, taking the form of collagen sheets. The diagnosis of IPF requires correlation of the clinical setting with radiographic images and a lung biopsy. In the absence of lung biopsy, the diagnosis of IPF can be made by defined clinical criteria that were published in guidelines endorsed by several professional societies. Differential diagnosis includes other idiopathic interstitial pneumonia, connective tissue diseases (systemic sclerosis, polymyositis, rheumatoid arthritis, forme fruste of autoimmune disorders, chronic hypersensitivity pneumonitis and other environmental (sometimes occupational exposures. IPF is typically progressive and leads to significant

  4. Pulmonary hypertension in POEMS syndrome

    OpenAIRE

    Jian LI; Tian, Zhuang; Zheng, Hao-Yi; Zhang, Wei; Duan, Ming-Hui; Liu, Yong-Tai; Cao, Xin-Xin; Zhou, Dao-bin

    2013-01-01

    POEMS syndrome is a rare clonal plasma cell disease. Patients with POEMS syndrome are at risk of developing pulmonary hypertension, but the data on its incidence and impact on outcome are limited. We reviewed records of 154 POEMS syndrome patients with complete duplex echocardiography data for estimation of pulmonary artery systolic pressure (sPAP) at the time of diagnosis. Forty-two (27%) of 154 patients with pulmonary hypertension (estimated sPAP ≥50mmHg) were identified. Median age was 46 ...

  5. Proapoptotic Bid is required for pulmonary fibrosis

    OpenAIRE

    Budinger, G.R. Scott; Mutlu, Gökhan M.; Eisenbart, James; Fuller, Alyson C.; Bellmeyer, Amy A.; Baker, Christina M.; Wilson, Mindy; Ridge, Karen; Terrence A. Barrett; Lee, Vivian Y.; Chandel, Navdeep S.

    2006-01-01

    The molecular mechanisms of pulmonary fibrosis are poorly understood. Previous reports indicate that activation of TGF-β1 is essential for the development of pulmonary fibrosis. Here, we report that the proapoptotic Bcl-2 family member Bid is required for the development of pulmonary fibrosis after the intratracheal instillation of bleomycin. Mice lacking Bid exhibited significantly less pulmonary fibrosis in response to bleomycin compared with WT mice. The attenuation in pulmonary fibrosis w...

  6. Amiodarone-induced pulmonary toxicity mimicking acute pulmonary edema.

    Science.gov (United States)

    Fabiani, Iacopo; Tacconi, Danilo; Grotti, Simone; Brandini, Rossella; Salvadori, Claudia; Caremani, Marcello; Bolognese, Leonardo

    2011-05-01

    Amiodarone is a highly effective antiarrhythmic drug. Its long-term use may, however, lead to several adverse effects, with pulmonary toxicity being the most serious. The article presents the case of a 78-year-old woman with a history of cardiac surgery, who after 2 years of amiodarone therapy for prophylactic treatment of atrial fibrillation developed amiodarone pneumonitis mimicking an acute pulmonary edema. The patient failed to respond to diuretic therapy and several courses of anti-infective therapy. Differential diagnosis of different causes of pulmonary infiltrates did not demonstrate any other abnormality. Lung biopsy findings were consistent with the diagnosis of amiodarone pneumonitis. Given the widespread use of amiodarone as an antiarrhythmic agent, pneumologists and cardiologists should consider this important adverse effect as a differential diagnosis of pulmonary distress refractory to therapy in all patients treated with amiodarone who present with respiratory symptoms and pneumonia-like illness. PMID:19924000

  7. A Family with Mental Retardation, Epilepsy and Cerebellar Hypoplasia Showing Linkage to Chromosome 20p11.21-q11.23

    Directory of Open Access Journals (Sweden)

    Fatih Bayrakli

    2014-01-01

    Full Text Available Background: Cerebellar hypoplasia (CH is a rare malformation caused by various etiologies, usually manifesting clinically as nonprogressive cerebellar ataxia with or without mental retardation. The molecular pathogenesis of the autosomal recessive cerebellar ataxias has a wide range of mechanisms. Differential diagnosis and categorization of the recessive cerebellar ataxias, however, need more specific, biochemical and genetic investigation. Methods: This study applied whole-genome linkage analysis to study a family with nonprogressive cerebellar ataxia and additional mental retardation, epilepsy, and facial dysmorphic features. Genotyping and linkage analysis was done using the GeneChip Mapping 250K NspI Array (Affymetrix Inc., Santa Clara, Calif., USA for genome-wide linkage analysis of the genotyping data from the affected children and their parents. Results: Allegro software version 1.2 was used for multipoint linkage analysis. We assumed an autosomal recessive inheritance pattern and assigned a penetrance of 0.999. Single-nucleotide polymorphism allele frequencies were estimated from the Affymetrix data of the Caucasian family studied. Using these parameters, a theoretical maximum logarithm of the odds score of 2.69 was identified at chromosome 20p11.21-q11.23. Conclusions: This chromosomal locus is unprecedented in autosomal recessive and nonprogressive ataxia disorder. Further investigation might reveal a new causative gene generating the CH phenotype.

  8. Nuclear scan of pulmonary hemorrhage in radiopathic pulmonary hemosiderosis

    International Nuclear Information System (INIS)

    Idiopathic pulmonary hemosiderosis, a disease of unknown etiology most often occuring in children, is characterized by recurring episodes of alveolar consolidation. Exacerbations of pulmonary hemorrhage coincide with episodes of alveolar filling; repeated episodes lead to progressive interstitial fibrosis and eventually to corpulmonale. Serial nuclear scans of the lungs after injection of radiolabeled red blood cells should parallel the pathologic and radiographic findings. We observed the accumulation of radiolabeled red blood cells in the lungs on scan images, a finding not previously reported

  9. Mycobacterial Pulmonary Infections in Patients with Idiopathic Pulmonary Fibrosis

    OpenAIRE

    Park, Sung-Woo; Song, Jin Woo; Shim, Tae Sun; Park, Moo-Suk; Lee, Hong-Lyeol; Uh, Soo-taek; Park, Choon-Sik; Kim, Dong Soon

    2012-01-01

    Patients with idiopathic pulmonary fibrosis (IPF) have an increased risk for developing tuberculosis (TB). However, no studies have been reported regarding the development of nontuberculous mycobacterium (NTM) lung disease (NTMLD). We reviewed 795 patients with IPF from five university hospitals who were diagnosed by histological or radio-clinical criteria. In the 795 patients with IPF, pulmonary infections with mycobacterium tuberculosis (MTB) and NTM were found in 35 (4.4%) and 16 patients ...

  10. Changes in Large Pulmonary Arterial Viscoelasticity in Chronic Pulmonary Hypertension

    OpenAIRE

    Wang, Zhijie; Lakes, Roderic S.; Golob, Mark; Eickhoff, Jens C.; Chesler, Naomi C.

    2013-01-01

    Conduit pulmonary artery (PA) stiffening is characteristic of pulmonary arterial hypertension (PAH) and is an excellent predictor of mortality due to right ventricular (RV) overload. To better understand the impact of conduit PA stiffening on RV afterload, it is critical to examine the arterial viscoelastic properties, which require measurements of elasticity (energy storage behavior) and viscosity (energy dissipation behavior). Here we hypothesize that PAH leads to frequency-dependent change...

  11. Endogenous pulmonary antibiotics.

    Science.gov (United States)

    Gibbons, M A; Bowdish, D M; Davidson, D J; Sallenave, J M; Simpson, A J

    2006-05-01

    The human lung produces a variety of peptides and proteins which have intrinsic antimicrobial activity. In general these molecules have broad spectra of antimicrobial activity, kill micro-organisms rapidly, and evade resistance generated by pathogens. In recent years it has become increasingly apparent that the antimicrobial peptides (AMPs) simultaneously possess immunomodulatory functions, suggesting complex roles for these molecules in regulating the clearance of, and immune response to, invading pathogens. These collective properties have stimulated considerable interest in the potential clinical application of endogenous AMPs. This article outlines the biology of AMPs, their pattern of expression in the lung, and their functions, with reference to both antimicrobial and immunomodulatory activity. We then consider the biological importance of AMPs, before concentrating on the potential to use AMPs to therapeutic effect. The principles discussed in the article apply to innate immune defence throughout the body, but particular emphasis is placed on AMPs in the lung and the potential application to pulmonary infection. PMID:16722137

  12. [Immunodepression and pulmonary infections].

    Science.gov (United States)

    Yao, N A; Ngoran, N; de Jaureguiberry, J P; Bérard, H; Jaubert, D

    2002-11-01

    The acquired immunosuppressed states are increasingly numerous. Pneumopathies are a frequent, serious complication and etiologic diagnosis is often difficult. The nature of the micro-organism in question is a function of the immunizing type of deficiency. In neutropenias, the infections are primarily bacterial, their potential gravity being correlated with the depth of the deficiency into polynuclear, or fungic, especially in prolonged neutropenias. The aspleened states are responsible for a deficit of the macrophage system and contribute to the infections with encapsulated germs (pneumococci, klebsiellas...). The organic grafts imply an attack of cell-mediated immunity, in the particular case of the auxiliary T lymphocytes (CD4)), with a special predisposition for viral and fungic infections. During VIH infection, the immunizing deficit of CD4 lymphocytes worsens with time. At the early stage, the infections are especially bacterial. At the more advanced stages, the pulmonary pneumocystosis and tuberculosis dominate. At the late stage, finally, deep immunosuppression allows emerging of the atypical mycobacteries. In the deficiencies of humoral immunity (congenital hypogammaglobulinemias, lymphoid hemopathies B), the germs to be mentioned are the pneumococcus, Haemophilus influenzae, the salmonellas and the legionellas. Immunosuppressed pneumopathies are characterized by radio-clinical pictures of very variable gravity, ranging from focused acute pneumopathy to bilateral diffuse pneumopathy with acute respiratory distress syndrome, with phases of atypical tables with respiratory symptomatology larval or absent. The highlighting of the micro-organisms in question requires urgent complementary investigations: hemocultures, bronchiolo-alveolar washing. In certain cases, it will be possible to resort to the transtracheal puncture or transthoracic puncture guided by tomodensitometry, and if necessary to pulmonary biopsy under videothoracoscopy. Emergency of the anti

  13. [Pulmonary function in patients with infiltrative pulmonary tuberculosis].

    Science.gov (United States)

    Nefedov, V B; Popova, L A; Shergina, E A

    2007-01-01

    Vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), FEV1/VC%, PEF, MEF25, MEF50, MEF75, TLC, TGV, pulmonary residual volume (PRV), R(aw), R(in),, R(ex), DLCO-SB, DLCO-SS, PaO2, and PaCO2 were determined in 103 patients with infiltrative pulmonary tuberculosis. Pulmonary dysfunction was detected in 83.5% of the patients. Changes were found in lung volumes and capacities in 63.1%, impaired bronchial patency and pulmonary gas exchange dysfunction were in 60.2 and 41.7%, respectively. The changes in pulmonary volumes and capacities appeared as increased PRV, decreased VC and FVC, and decreased and increased TGV and TLC; impaired bronchial patency presented as decreased PEF, MEF25, MEF50, MEF75, FEV1/VC% and increased R(aw) R(in), and R(ex); pulmonary gas exchange dysfunction manifested itself as reduced DLCO-SB, DLCO-SS, and PaO2 and decreased and increased PaCO2. The magnitude of the observed functional changes was generally slight. Significant disorders were observed rarely and very pronounced ones were exceptional. PMID:17915466

  14. [Pulmonary function in patients with disseminated pulmonary tuberculosis].

    Science.gov (United States)

    Nefedov, V B; Shergina, E A; Popova, L A

    2007-01-01

    Vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), FEV1/VC%, PEF, MEF25%, MEF50%, MEF75%, TLS, TGV, pulmonary residual volume (PRV), Raw, Rin, Rex, DLCO-SB, DLCO-SS, PaO2, and PaCO2 were determined in 29 patients with disseminated pulmonary tuberculosis. Pulmonary dysfunction was detected in 93.1% of the patients. Changes were found in lung volumes and capacities in 65.5%, impaired bronchial patency and pulmonary gas exchange dysfunction were in 79.3 and 37.9%, respectively. The changes in pulmonary volumes and capacities appeared as increased PRV, decreased VC, FVC, and TLS, decreased and increased TGV; impaired bronchial patency presented as decreased PEF, MEF25%, MEF50%, MEF75%, and FEV1/VC% and increased Raw, Rin, and Rex; pulmonary gas exchange dysfunction manifested itself as reduced DLCO-SS and PaO2 and decreased and increased PaCO2. The observed functional changes varied from slight to significant and pronounced with a preponderance of small disorders, a lower detection rate of significant disorders, and rare detection of very pronounced ones. PMID:18041129

  15. Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology

    Directory of Open Access Journals (Sweden)

    M. Humbert

    2010-03-01

    Full Text Available Pulmonary arterial hypertension (PAH and chronic thromboembolic pulmonary hypertension (CTEPH are two of the key subgroups of pulmonary hypertension. They are characterised by different risk factors. PAH can be associated with mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2, HIV infection, congenital heart disease, connective tissue disease (such as systemic sclerosis, and exposure to particular drugs and toxins including fenfluramine derivatives. In contrast, CTEPH can be associated with anti-phospholipid antibodies, splenectomy and the presence of a ventriculo-atrial shunt or an infected pacemaker. The first-line therapies used to treat PAH and CTEPH also differ. While medical therapy tends to be used for patients with PAH, pulmonary endarterectomy is the treatment of choice for patients with CTEPH. However, there are possible common mechanisms behind the two diseases, including endothelial cell dysfunction and distal pulmonary artery remodelling. Further research into these similarities is needed to assist the development of targeted pharmacological therapies for patients with inoperable CTEPH and patients who have persistent pulmonary hypertension after endarterectomy.

  16. Pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension: pathophysiology.

    Science.gov (United States)

    Humbert, M

    2010-03-01

    Pulmonary arterial hypertension (PAH) and chronic thromboembolic pulmonary hypertension (CTEPH) are two of the key subgroups of pulmonary hypertension. They are characterised by different risk factors. PAH can be associated with mutations in the gene encoding bone morphogenetic protein receptor type II (BMPR2), HIV infection, congenital heart disease, connective tissue disease (such as systemic sclerosis), and exposure to particular drugs and toxins including fenfluramine derivatives. In contrast, CTEPH can be associated with anti-phospholipid antibodies, splenectomy and the presence of a ventriculo-atrial shunt or an infected pacemaker. The first-line therapies used to treat PAH and CTEPH also differ. While medical therapy tends to be used for patients with PAH, pulmonary endarterectomy is the treatment of choice for patients with CTEPH. However, there are possible common mechanisms behind the two diseases, including endothelial cell dysfunction and distal pulmonary artery remodelling. Further research into these similarities is needed to assist the development of targeted pharmacological therapies for patients with inoperable CTEPH and patients who have persistent pulmonary hypertension after endarterectomy. PMID:20956167

  17. A Case of Pulmonary Varix Associated with Superior Pulmonary Vein Occlusion

    OpenAIRE

    Tajiri, Sakurako; Koizumi, Jun; Hara, Takuya; Kamono, Masahiro; Hayama, Naoki; Kobayashi, Ichiro; Kondo, Yusuke; Kondo, Tetsuri; Asano, Koichiro; Abe, Tadashi

    2012-01-01

    A pulmonary varix is a localized dilatation of a pulmonary vein, which is usually asymptomatic presented as a mass on a chest roentgenogram, and diagnosed with pulmonary angiography. We encountered a case of 55 year-old man, in whom incidentally identified was a dilated blood vessel that passed through the minor fissure and returned to the inferior pulmonary vein, which we diagnosed as pulmonary varix. This vascular anomaly was accompanied by the occluded superior pulmonary vein, highly sugge...

  18. Massive Pulmonary Hemorrhage from Dual Circulation Pulmonary Arteriovenous Malformations in Hereditary Hemorrhagic Telangiectasia

    OpenAIRE

    Sharma, Krishna B.; Lutz Forkert

    2004-01-01

    Pulmonary arteriovenous malformations (AVMs) are commonly supplied by the pulmonary arterial system and rarely by the systemic bronchial circulation. The authors outline the case of a young woman with pulmonary AVMs as part of hereditary hemorrhagic telangiectasia with the uncommon presentation of massive hemoptysis. Management of her recurrent, life-threatening pulmonary hemorrhage was complicated by pulmonary AVMs that were supplied by both the pulmonary and systemic bronchial arterial circ...

  19. Decreased time constant of the pulmonary circulation in chronic thromboembolic pulmonary hypertension

    OpenAIRE

    MacKenzie Ross, Robert V.; Toshner, Mark R.; Soon, Elaine; Naeije, Robert; Pepke-Zaba, Joanna

    2013-01-01

    This study analyzed the relationship between pulmonary vascular resistance (PVR) and pulmonary arterial compliance (Ca) in patients with idiopathic pulmonary arterial hypertension (IPAH) and proximal chronic thromboembolic pulmonary hypertension (CTEPH). It has recently been shown that the time constant of the pulmonary circulation (RC time constant), or PVR × Ca, remains unaltered in various forms and severities of pulmonary hypertension, with the exception of left heart failure. We reasoned...

  20. Anticoagulation in Pulmonary Arterial Hypertension.

    Science.gov (United States)

    Robinson, Jeffrey C; Pugliese, Steven C; Fox, Daniel L; Badesch, David B

    2016-06-01

    Pulmonary arterial hypertension (PAH) is characterized by molecular and pathologic alteration to the pulmonary circulation, resulting in increased pulmonary vascular resistance, right ventricular failure, and eventual death. Pharmacologic treatment of PAH consists of use of a multitude of pulmonary vasodilators, sometimes in combination. PAH has been associated with increased thrombosis and disrupted coagulation and fibrinolysis, making anticoagulation an attractive and frequently employed therapeutic modality. Observational studies have provided some insight into the therapeutic potential of anticoagulation in idiopathic PAH, but there is a distinct lack of well-controlled prospective trials. Due to the conflicting evidence, there is a large amount of heterogeneity in the application of therapeutic anticoagulation in PAH and further well-controlled prospective trials are needed to clarify its role in treating PAH. PMID:27137522

  1. Pulmonary involvement in tuberous sclerosis

    International Nuclear Information System (INIS)

    The authors present a case of tuberous sclerosis with marked pulmonary involvement, confirmed by both radiological and pathological studies. The radiological manifestations and basic pathology of this rare condition are reviewed with emphasis on differential diagnosis. (orig.)

  2. COPD (Chronic Obstructive Pulmonary Disease)

    Science.gov (United States)

    ... page from the NHLBI on Twitter. What Is COPD? Español COPD, or chronic obstructive pulmonary (PULL-mun- ... can clog them. Normal Lungs and Lungs With COPD Figure A shows the location of the lungs ...

  3. Pulmonary ventilation/perfusion scan

    Science.gov (United States)

    V/Q scan; Ventilation/perfusion scan; Lung ventilation/perfusion scan ... A pulmonary ventilation/perfusion scan is actually two tests. They may be done separately or together. During the perfusion scan, a health ...

  4. Traumatic lesions of pulmonary parenchyma

    International Nuclear Information System (INIS)

    Five cases of post-traumatic pulmonary lesions (contusion, laceration and hematoma) are presented. The pathophysiology, radiological aspects and differential diagnosis are reviewed. The benign evolution showing the absorption in short time, without medical interference is emphasized. (Author)

  5. Drug-induced pulmonary disease

    Science.gov (United States)

    ... mediastinitis ) Abnormal buildup of fluid in the lungs ( pulmonary edema ) Buildup of fluid between the layers of tissue that line the lungs and chest cavity ( pleural effusion ) Many ... as bleomycin, cyclophosphamide, and methotrexate Illegal drugs

  6. Etiopathogenesis of neurogenic pulmonary edema

    Czech Academy of Sciences Publication Activity Database

    Šedý, Jiří

    2010-01-01

    Roč. 160, 5-6 (2010), s. 152-154. ISSN 0043-5341 Institutional research plan: CEZ:AV0Z50390512 Keywords : neurogenic pulmonary edema * intracranial pressure * sympathetic system Subject RIV: FH - Neurology

  7. Preventing Hantavirus Pulmonary Syndrome (HPS)

    Science.gov (United States)

    ... page: About CDC.gov . Hantavirus Share Compartir Preventing Hantavirus Pulmonary Syndrome (HPS) Eliminate or minimize contact with ... Pathogens Branch 1600 Clifton Rd Atlanta, GA 30333 Hantavirus Hotline (877) 232-3322 (404) 639-1510 800- ...

  8. Chronic cough and pulmonary infiltrates

    International Nuclear Information System (INIS)

    Case of chronic cough and pulmonary infiltrates, in patient feminine of 66 years who she consults for scheme of cough with mucous expectoration that it increases with the exhibition to the powder and the cold

  9. Serum calcium in pulmonary tuberculosis

    OpenAIRE

    Subhash C. Sharma

    1981-01-01

    Serum calcium was studied serially in 94 patients with active pulmonary tuberculosis. An equal number of age- and sex-matched patients with chronic obstructive pulmonary disease were controls. Seventy patients in the study group were normocalcaemic and 10 were hypercalcaemic. These 10 were on a higher supplement of vitamin D than the 70 normocalcaemic patients. There was a positive correlation between the daily vitamin intake and the degree and duration of hypercalcaemia. None of the controls...

  10. New Mechanisms of Pulmonary Fibrosis

    OpenAIRE

    Strieter, Robert M.; Mehrad, Borna

    2009-01-01

    The understanding of the pathogenesis of pulmonary fibrosis continues to evolve. The initial hypothetical model suggested chronic inflammation as the cause of pulmonary fibrosis, whereas a subsequent hypothesis posited epithelial injury and impaired wound repair as the etiology of fibrosis without preceding inflammation. Over the past decade, several concepts have led to refinement of these hypotheses. These include the following: (1) the importance of the integrity of the alveolar-capillary ...

  11. Epidemiology of idiopathic pulmonary fibrosis

    OpenAIRE

    Ley, Brett

    2013-01-01

    Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of it...

  12. Epidemiology of idiopathic pulmonary fibrosis

    OpenAIRE

    Ley B; Collard HR

    2013-01-01

    Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its...

  13. Pulmonary Surfactant: An Immunological Perspective

    OpenAIRE

    Chroneos, Zissis C.; Sever-Chroneos, Zvjezdana; Shepherd, Virginia L.

    2009-01-01

    Pulmonary surfactant has two crucial roles in respiratory function; first, as a biophysical entity it reduces surface tension at the air water interface, facilitating gas exchange and alveolar stability during breathing, and, second, as an innate component of the lung's immune system it helps maintain sterility and balance immune reactions in the distal airways. Pulmonary surfactant consists of 90% lipids and 10% protein. There are four surfactant proteins named SP-A, SP-B, SP-C, and SP-D; th...

  14. Pulmonary lymphangioleiomyomatosis in a male.

    OpenAIRE

    Kang, H. W.; Kim, C. J.; Kang, S K; Lee, K. S.; C. S. LEE; Kim, Y.H.(Center for Underground Physics, Institute for Basic Science (IBS), Daejon, 305-811, Korea)

    1991-01-01

    Pulmonary lymphangioleiomyomatosis has been observed almost exclusively in women, usually in their reproductive years. Exacerbations with pregnancy and after hormonal manipulation have been documented, and it has been suggested that its pathogenesis is due to the influence of hormonal(estrogenic) stimulus. The clinical, roentgenographic, and histopathologic features of this case of pulmonary lymphangioleiomyomatosis in a 22-year-old male are all characteristic of those described in prior repo...

  15. Pulmonary Hypertension Secondary to COPD

    OpenAIRE

    Adil Shujaat; Abubakr A. Bajwa; Cury, James D.

    2012-01-01

    The development of pulmonary hypertension in COPD adversely affects survival and exercise capacity and is associated with an increased risk of severe acute exacerbations. Unfortunately not all patients with COPD who meet criteria for long term oxygen therapy benefit from it. Even in those who benefit from long term oxygen therapy, such therapy may reverse the elevated pulmonary artery pressure but cannot normalize it. Moreover, the recent discovery of the key roles of endothelial dysfunction ...

  16. Pulmonary function in Parkinson's disease.

    OpenAIRE

    Hovestadt, A.; Bogaard, J. M.; Meerwaldt, J D; van der Meché, F G; Van Stigt, J.

    1989-01-01

    Pulmonary function was investigated in 31 consecutive patients with relatively severe Parkinson's disease. Clinical disability was assessed by Hoehn and Yahr scale, Northwestern University Disability Scale and Websterscore. All patients were on levodopa substitution therapy and used anticholinergics. Pulmonary function was investigated by spirography, determination of a maximal inspiratory and expiratory flow-volume curve and, when possible, maximal static mouth pressures were determined. Pea...

  17. Cardiovascular Function in Pulmonary Emphysema

    OpenAIRE

    Dina Visca; Marina Aiello; Alfredo Chetta

    2013-01-01

    Chronic obstructive pulmonary disease (COPD) and chronic cardiovascular disease, such as coronary artery disease, congestive heart failure, and cardiac arrhythmias, have a strong influence on each other, and systemic inflammation has been considered as the main linkage between them. On the other hand, airflow limitation may markedly affect lung mechanics in terms of static and dynamic hyperinflation, especially in pulmonary emphysema, and they can in turn influence cardiac performance as well...

  18. Occupational chronic obstructive pulmonary disease

    DEFF Research Database (Denmark)

    Omland, Oyvind; Würtz, Else Toft; Aasen, Tor Brøvig;

    2014-01-01

    Occupational-attributable chronic obstructive pulmonary disease (COPD) presents a substantial health challenge. Focusing on spirometric criteria for airflow obstruction, this review of occupational COPD includes both population-wide and industry-specific exposures.......Occupational-attributable chronic obstructive pulmonary disease (COPD) presents a substantial health challenge. Focusing on spirometric criteria for airflow obstruction, this review of occupational COPD includes both population-wide and industry-specific exposures....

  19. Diseases of Pulmonary Surfactant Homeostasis

    OpenAIRE

    Jeffrey A Whitsett; Wert, Susan E.; Weaver, Timothy E.

    2015-01-01

    Advances in physiology and biochemistry have provided fundamental insights into the role of pulmonary surfactant in the pathogenesis and treatment of preterm infants with respiratory distress syndrome. Identification of the surfactant proteins, lipid transporters, and transcriptional networks regulating their expression has provided the tools and insights needed to discern the molecular and cellular processes regulating the production and function of pulmonary surfactant prior to and after bi...

  20. Balloon pulmonary angioplasty: a treatment option for inoperable patients with chronic thromboembolic pulmonary hypertension

    Directory of Open Access Journals (Sweden)

    Aiko eOgawa

    2015-02-01

    Full Text Available In chronic thromboembolic pulmonary hypertension, stenoses or obstructions of the pulmonary arteries due to organized thrombi can cause an elevation in pulmonary artery resistance, which in turn can result in pulmonary hypertension. Chronic thromboembolic pulmonary hypertension can be cured surgically by pulmonary endarterectomy; however, patients deemed unsuitable for pulmonary endarterectomy due to lesion, advanced age, or comorbidities have a poor prognosis and limited treatment options. Recently, advances have been made in balloon pulmonary angioplasty for these patients, and this review highlights this recent progress.

  1. Lung imaging in pulmonary disease

    International Nuclear Information System (INIS)

    Although it has been recognized for several years that chronic obstructive pulmonary disease (COPD) can cause lung perfusion defects which may simulate pulmonary embolism, relatively little use has been made of either the radioxenon or the radioaerosol inhalation lung imaging procedures until the last few years as a means of distinguishing pulmonary embolism (P.E.) from COPD is reported. Recent experience is reported with the use of both of these procedures in comparison with pulmonary function tests for the early detection of COPD in population studies and also in P.E. suspects. Equal emphasis is given to simultaneous aerosol ventilation-perfusion (V/P) imaging in the differential diagnosis of P.E. Finally, this paper is concerned with new developments in regional lung diffusion imaging following the inhalation of radioactive gases and rapidly absorbed radioaerosols. Their experimental basis is presented and their potential clinical applications in pulmonary embolism are discussed. As a result of these investigations, a functional (V/P) diagnosis of pulmonary embolism in patients may be possible in the near future with a sequential radioaerosol inhalation procedure alone

  2. Pulmonary Function Measures Predict Mortality Differently in Idiopathic Pulmonary Fibrosis versus Combined Pulmonary Fibrosis and Emphysema

    OpenAIRE

    Schmidt, Shelley L.; Nambiar, Anoop M.; Tayob, Nabihah; Sundaram, Baskaran; Han, MeiLan K.; Gross, Barry H.; Kazerooni, Ella A.; Chughtai, Aamer R.; Lagstein, Amir; Myers, Jeffrey L.; Murray, Susan; Toews, Galen B.; Martinez, Fernando J; Flaherty, Kevin R.

    2010-01-01

    The composite physiologic index(CPI) was derived to represent the extent of fibrosis on high resolution computed tomography, adjusting for emphysema in patients with idiopathic pulmonary fibrosis(IPF). We hypothesized longitudinal change in CPI would better predict mortality than forced expiratory volume in 1 second(FEV1), forced vital capacity(FVC), or diffusing capacity for carbon monoxide(DLCO) in all patients with IPF, and especially in those with combined pulmonary fibrosis and emphysema...

  3. Pulmonary interstitial glycogenosis within a discrete pulmonary lesion mimicking congenital pulmonary airway malformation

    OpenAIRE

    Robert L. Ricca; Goldin, Adam B.; Deutsch, Gail H.; Kimberly J Riehle

    2015-01-01

    Interstitial lung diseases (ILD) are a heterogeneous group of pulmonary disorders that are relatively rare in the pediatric population. These diseases are characterized by impaired gas exchange and typically manifest with diffuse infiltrates on radiographs. Pulmonary interstitial glycogenosis (PIG) has recently been identified as an ILD affecting neonates and infants that manifests diffusely throughout the lungs by imaging, has non-specific clinical features, and usually has a favorable outco...

  4. The diagnosis value of pulmonary perfusion/ventilation imaging for pulmonary embolism: in comparison with pulmonary artery angiography

    International Nuclear Information System (INIS)

    Objective: To evaluate the value of the pulmonary perfusion/ventilation imaging in diagnosis of pulmonary embolism (PE). Methods: Forty-five patients undergone pulmonary radionuclide imaging and pulmonary artery angiography in Fuwai Hospital were analyzed retrospectively. Results: Twenty-six patients were diagnosed as with PE by pulmonary angiography. For the pulmonary segments with complete filling defect on pulmonary artery angiography, the coincidence rate was 92.7% between the angiography and radionuclide pulmonary imaging, while the pulmonary segments with partial filling defect, the coincidence rate was 73.2% (P<0.01). The sensitivity and specificity were 92.3% and 84.2%, respectively. Both the positive predictive value and negative predictive value were 88.9%. Out of 26 patients with PE, 23 patients had also pulmonary ventilation imaging. The results showed mismatch between pulmonary perfusion and ventilation imaging in all of the 23 patients. Conclusion: The pulmonary perfusion plus pulmonary ventilation imaging plays an important role in diagnosing PE

  5. Coronary to pulmonary fistula as the primary source of pulmonary blood supply in pulmonary atresia with ventricular septal defect

    Directory of Open Access Journals (Sweden)

    Isman Firdaus

    2004-12-01

    Full Text Available A communication between the coronary and pulmonary arteries, so called coronary to pulmonary fistula, is a rare source of pulmonary supply in pulmonary atresia (PA with ventricular septal defect (VSD. A 4 year old girl referred to National Cardiovascular Center Harapan Kita, Jakarta with symptoms and signs of increased pulmonary blood flow since infancy and was confirmed by the chest x-rays. Heart examination revealed normal first heart sound with single loud second heart sound and an ejection systolic murmur at the pulmonary area. ECG demonstrated sinus rhythm with normal axis and biventricular hypertrophy. Echocardiography was performed and truncus arteriosus (TA type I was suspected with perimembranus VSD, overriding of the aorta, and dilated main pulmonary artery. But on cardiac catheterization studies, a non obstructive fistula was found between the left coronary and main pulmonary artery coexisted with PA and VSD. A successful surgery was performed subsequently and confirmed the above diagnosis. Although there were episodes of pulmonary hypertension crisis during early post operative course, she was then discharge from the hospital in a good condition. Since irreversible pulmonary vascular disease may develop in a non restrictive coronary to pulmonary fistula, early recognition of this anomaly is very important for better surgical result. (Med J Indones 2004; 13: 237-40Keywords: coronary to pulmonary fistula, pulmonary atresia, ventricular septal defect

  6. REACTIVE OXYGEN AND NITROGEN SPECIES IN PULMONARY HYPERTENSION

    OpenAIRE

    Tabima, Diana M.; Frizzell, Sheila; Gladwin, Mark T.

    2012-01-01

    Pulmonary vascular disease can be defined as either a disease affecting the pulmonary capillaries and pulmonary arterioles, termed pulmonary arterial hypertension, or as a disease affecting the left ventricle, called pulmonary venous hypertension. Pulmonary arterial hypertension (PAH) is a disorder of the pulmonary circulation characterized by endothelial dysfunction, as well as intimal and smooth muscle proliferation. Progressive increases in pulmonary vascular resistance and pressure impair...

  7. Chronic obstructive pulmonary disease.

    Science.gov (United States)

    Barnes, Peter J; Burney, Peter G J; Silverman, Edwin K; Celli, Bartolome R; Vestbo, Jørgen; Wedzicha, Jadwiga A; Wouters, Emiel F M

    2015-01-01

    Chronic obstructive pulmonary disease (COPD) is a common disease with high global morbidity and mortality. COPD is characterized by poorly reversible airway obstruction, which is confirmed by spirometry, and includes obstruction of the small airways (chronic obstructive bronchiolitis) and emphysema, which lead to air trapping and shortness of breath in response to physical exertion. The most common risk factor for the development of COPD is cigarette smoking, but other environmental factors, such as exposure to indoor air pollutants - especially in developing countries - might influence COPD risk. Not all smokers develop COPD and the reasons for disease susceptibility in these individuals have not been fully elucidated. Although the mechanisms underlying COPD remain poorly understood, the disease is associated with chronic inflammation that is usually corticosteroid resistant. In addition, COPD involves accelerated ageing of the lungs and an abnormal repair mechanism that might be driven by oxidative stress. Acute exacerbations, which are mainly triggered by viral or bacterial infections, are important as they are linked to a poor prognosis. The mainstay of the management of stable disease is the use of inhaled long-acting bronchodilators, whereas corticosteroids are beneficial primarily in patients who have coexisting features of asthma, such as eosinophilic inflammation and more reversibility of airway obstruction. Apart from smoking cessation, no treatments reduce disease progression. More research is needed to better understand disease mechanisms and to develop new treatments that reduce disease activity and progression. PMID:27189863

  8. Pulmonary Alveolar Proteinosis

    Directory of Open Access Journals (Sweden)

    Sandeep M Patel

    2012-01-01

    Full Text Available Pulmonary alveolar proteinosis (PAP is a disease of alveolar accumulation of phospholipoproteinaceous material that results in gas exchange impairment leading to dyspnea and alveolar infiltrates. There are three forms of PAP: congenital, acquired and idiopathic; of which the latter two are predominant in the adult population. Previous case studies have found that the acquired form can be secondary to various autoimmune, infectious, malignant and environmental etiologies. Recent advances in the understanding of the pathophysiology of PAP demonstrate that the idiopathic form is due to antigranulocyte macrophage-colony stimulating factor antibodies. Therapeutic targets that replace granulocyte macrophage colony stimulating factor or remove these antibodies are being actively developed. The current standard of care is to perform whole lung lavage on these patients to clear the alveolar space to help improve respiratory physiology. A case of PAP is reported, followed by a literature review on the diagnosis and management of this rare condition with the aim of increasing awareness among physicians when treating patients who present with alveolar infiltrates.

  9. Pulmonary thromboembolism in cats.

    Science.gov (United States)

    Schermerhorn, Thomas; Pembleton-Corbett, Julie R; Kornreich, Bruce

    2004-01-01

    Pulmonary thromboembolism (PTE) is rarely diagnosed in cats, and the clinical features of the disease are not well known. PTE was diagnosed at postmortem examination in 17 cats, a prevalence of 0.06% over a 24-year period. The age of affected cats ranged from 10 months to 18 years, although young (10 years) cats were more commonly affected than were middle-aged cats. Males and females were equally affected. The majority of cats with PTE (n = 16) had concurrent disease, which was often severe. The most common diseases identified in association with PTE were neoplasia, anemia of unidentified cause, and pancreatitis. Cats with glomerulonephritis, encephalitis, pneumonia, heart disease, and hepatic lipidosis were also represented in this study. Most cats with PTE demonstrated dyspnea and respiratory distress before death or euthanasia, but PTE was not recognized ante mortem in any cat studied. In conclusion, PTE can affect cats of any age and is associated with a variety of systemic and inflammatory disorders. It is recommended that the same clinical criteria used to increase the suspicion of PTE in dogs should also be applied to cats. PMID:15320593

  10. Longitudinal Evaluation of the Hypothalamic-Pituitary-Testicular Function in 8 Boys with Adrenal Hypoplasia Congenita (AHC) Due to NR0B1 Mutations

    Science.gov (United States)

    Galeotti, Caroline; Lahlou, Zineb; Goullon, Domitille; Sarda-Thibault, Hélène; Cahen-Varsaux, Juliette; Bignon-Topalovic, Joëlle; Bashamboo, Anu; McElreavey, Ken; Brauner, Raja

    2012-01-01

    Background Boys carrying mutations in the NR0B1 gene develop adrenal hypoplasia congenita (AHC) and impaired sexual development due to the combination of hypogonadotropic hypogonadism (HH) and primary defects in spermatogenesis. Methods We analysed the evolution of hypothalamic-pituitary-testicular function of 8 boys with AHC due to NR0B1 mutations. Our objective was to characterize and monitor the progressive deterioration of this function. Results The first symptoms appeared in the neonatal period (n = 5) or between 6 months and 8.7 years (n = 3). Basal plasma adrenocorticotrophic hormone (ACTH) concentrations increased in all boys, whilst cortisol levels decreased in one case. The natremia was equal or below 134 mmol/L and kaliemia was over 5 mmol/L. All had increased plasma renin. In 3 of 4 patients diagnosed in the neonatal period and evaluated during the first year, the basal plasma gonadotropins concentrations, and their response to gonadotropin releasing hormone (GnRH) test (n = 2), and those of testosterone were normal. The plasma inhibin B levels were normal in the first year of life. With the exception of two cases these concentrations decreased to below the normal for age. Anti-Müllerian hormone concentrations were normal for age in all except one case, which had low concentrations before the initiation of testosterone treatment. In 3 of the 8 cases the gene was deleted and the remaining 5 cases carried frameshift mutations that are predicted to introduce a downstream nonsense mutation resulting in a truncated protein. Conclusions The decreases in testosterone and inhibin B levels indicated a progressive loss of testicular function in boys carrying NR0B1 mutations. These non-invasive examinations can help to estimate the age of the testicular degradation and cryopreservation of semen may be considered in these cases as investigational procedure with the aim of restoring fertility. PMID:22761912

  11. X-linked adrenal hypoplasia congenita and hypogonadotropic hypogonadism: Identification and in vitro study of a novel small indel in the NR0B1 gene.

    Science.gov (United States)

    Yu, Tingting; Wang, Jian; Yu, Yongguo; Huang, Xiaodong; Fu, Qihua; Shen, Yiping; Chen, Fuxiang

    2016-05-01

    DAX1 is an orphan nuclear receptor that has a key role in the development and function of the adrenal and reproductive axes. Mutations in NR0B1, the gene encoding DAX1, result in X-linked adrenal hypoplasia congenita (AHC) and hypogonadotropic hypogonadism (HHG). A Chinese pedigree with X-linked AHC and HHG was investigated in the present study. Sequence analysis identified a novel small indel variant, c.195_207delinsTG, in the NR0B1 gene. To determine the effect of this variant on DAX1 expression, reverse‑transcription quantitative PCR and western blot assays were performed. The mRNA expression levels in carriers of mutant NR0B1 were significantly reduced (62% decrease) compared to those in individuals with wild-type NR0B1 (WT). The c.195_207delinsTG mutation was demonstrated to lead to various truncated DAX1 proteins, including the C‑terminal truncated DAX1, which was only detected in the cytoplasm, and the N‑terminal truncated DAX1, which was present in the cytoplasm and nucleus. A luciferase assay was then performed to assess the repressor function of DAX1 in modulating steroidogenic factor 1 (SF‑1)‑mediated transactivation. WT DAX1 significantly suppressed the SF‑1‑mediated promoter activity of the steroidogenic acute regulatory protein by 35.5±1.9%. In contrast to other known pathogenic mutations which abolish the repressor function of DAX1, the c.195_207delinsTG mutant proxkduced a higher repressor activity, demonstrating a 49.9±2.6% reduction of promoter activity. These findings suggested that the mutation of NR0B1 in X‑linked AHC with HHG enhanced the function of DAX1 to repress SF-1 activation, while DAX1 is expected to have additional roles in the pathological mechanism. PMID:27035099

  12. Managing chronic thromboembolic pulmonary hypertension: pharmacological treatment options

    OpenAIRE

    I.M. Lang

    2009-01-01

    Chronic thromboembolic pulmonary hypertension (CTEPH) is a life-threatening condition in which organised thrombi obstruct the pulmonary vessels, causing increased pulmonary vascular resistance, progressive pulmonary hypertension (PH) and right heart failure. The treatment of choice is pulmonary endarterectomy, which restores pulmonary haemodynamics with acceptable periprocedural mortality rates in the majority of suitable patients. However, CTEPH may be inoperable owing to surgically inaccess...

  13. Pulmonary dysanapsis and diving assessments.

    Science.gov (United States)

    Ong, Lin Min; Bennett, Michael H; Thomas, Paul S

    2009-01-01

    Airway obstruction is a relative contraindication to diving. Dive candidates are assessed clinically, and lung function tests evaluate variables such as forced vital capacity (FVC), forced expiratory volume in one second (FEV1) and the FEV1/FVC ratio. A small number of individuals have a normal FEV1, but a disproportionately large lung capacity, or pulmonary dysanapsis. These individuals have a decreased FEV1/FVC ratio, suggesting airway obstruction, which may affect their dive medical assessments. Three cases of pulmonary dysanapsis presented for fitness-to-dive assessment. Case 1, a 29-year-old male had an FEV1: 3.52 L (85% predicted), FVC: 5.31 L (108.5% predicted), giving a FEV1/FVC of 66%. Case 2, a 25-year-old male with an FEV1: 4.55 L (95% predicted), FVC: 7.0 L (121% predicted) and a FEV1/FVC of 66%. Albuterol produced an FEV1 increase of 11%, but his hypertonic saline challenge was negative. Case 3, a 61-year-old man had an FEV1: 3.49 L (126% predicted), FVC: 7.06 L (216% predicted), and a FEV1/FVC of 49%. This report highlights pulmonary dysanapsis which may be confused with obstructive airway disease and applicants deemed unfit to dive. While pulmonary dysanapsis may increase the risk of airway hyperresponsiveness, there is no evidence of an association with diving-related pulmonary barotrauma. PMID:20112528

  14. Aerobic exercise in pulmonary rehabilitation

    Directory of Open Access Journals (Sweden)

    Thiago Brasileiro de Vasconcelos

    2013-01-01

    Full Text Available The aim of this study was to conduct a literature review on the usefulness of aerobic exercise in pulmonary rehabilitation. This is an exploratory study of literature through the electronic databases Medline, Lilacs, Scielo, Pubmed and Google Scholar, published between 1996 and 2012, conducted during the period February to May 2012 with the following keywords: COPD, pulmonary rehabilitation, aerobic exercises, physical training, quality of life. The change in pulmonary function and dysfunction of skeletal muscles that result in exercise intolerance and reduced fitness and may cause social isolation, depression, anxiety and addiction. The training exercise is the most important component of the program of pulmonary rehabilitation where the aerobic training provides consistent results in clinical improvement in levels of exercise tolerance and decreased dyspnea generating more benefits to the body, reducing the chance of cardiovascular disease and improves quality and expectation of life. We demonstrated that the use of aerobic exercise in pulmonary rehabilitation program, allows an improvement of motor skills, decreased muscle fatigue and deconditioning, reducing sedentary lifestyle; however, has little or no effect on the reduction of strength and atrophy muscle.

  15. Pulmonary agenesis and pulmonary sling anomaly in an infant with Down syndrome.

    Science.gov (United States)

    Takci, S; Yigit, S; Haliloglu, M; Boduroglu, K; Kiper, N

    2013-01-01

    Pulmonary agenesis is a rare congenital anomaly presenting with normal karyotype in most of the cases. Rarely pulmonary agenesis is associated with chromosomal abnormalities and other genetic disorders such as Oculo-auriculo-vertebral spectrum, VACTERL association and velo-cardio-facial syndrome. This report presents a patient with pulmonary agenesis, pulmonary sling anomaly and Down syndrome. PMID:24341150

  16. Answers about Lung Transplantation for Pulmonary Hypertension

    Science.gov (United States)

    ... the page. Answers about Lung Transplantation for PULMONARY HYPERTENSION Part One: Overview From the development of epoprostenol ... decades, expansion of medical treatment of pulmonary arterial hypertension (PAH) has improved survival and quality of life ...

  17. Lymphangioleiomyomatosis and tuberous sclerosis with pulmonary involvement

    International Nuclear Information System (INIS)

    We present two cases of pulmonary lumphangioleiomyomatosis and one case of tuberous sclerosis with pulmonary involvement describing the most characteristic features according to plain chest X-ray and high-resolution computed tomography (HRCT). (Author) 14 refs

  18. Genetics Home Reference: idiopathic pulmonary fibrosis

    Science.gov (United States)

    ... idiopathic pulmonary fibrosis. N Engl J Med. 2007 Mar 29;356(13):1317-26. Citation on PubMed ... PJ, Garcia CK. Effect of telomere length on survival in patients with idiopathic pulmonary fibrosis: an observational ...

  19. Peripheral airway obstruction in primary pulmonary hypertension

    OpenAIRE

    Meyer, F; Ewert, R.; Hoeper, M; Olschewski, H.; Behr, J.; Winkler, J.; Wilkens, H.; Breuer, C.; Kubler, W; Borst, M

    2002-01-01

    Background: As there is controversy about changes in lung function in primary pulmonary hypertension (PPH), lung mechanics were assessed with a focus on expiratory airflow in relation to pulmonary haemodynamics.

  20. Hereditary Hemorrhagic Telangiectasia (HHT) and Pulmonary Hypertension

    Science.gov (United States)

    Hereditary Hemorrhagic Telangiectasia (HHT) Pulmonary & PH Hypertension Did you know that if you have HHT, you are at risk for pulmonary ... options for patients in the future. Hereditary Hemorrhagic Telangiectasia-Associated PH, or HHT-Associated PH My doctor ...

  1. Acute pulmonary embolism following air travel

    OpenAIRE

    Ledermann, J. A.; Keshavarzian, Ali

    1983-01-01

    Three cases of pulmonary embolism following long air flight are described. There was no previous history of venous disease. The symptoms were transient in one and severe in two. The occurrence of pulmonary embolism immediately after air travel is emphasized.

  2. Diurnal Variations in Human Pulmonary Function

    OpenAIRE

    Medarov, Boris I.; Pavlov, Valentin A.; Rossoff, Leonard

    2008-01-01

    Pulmonary function has circadian modulations. Variations in human pulmonary function during the daytime hours (diurnal variations) remain to be well characterized. Discerning these variations will contribute to better understanding the relationship between biorhythms and lung physiology and to improving clinical management of pulmonary diseases. The aim of this study was to determine the magnitude of pulmonary function variability during the usual daytime hours in a population of patients ref...

  3. An Update on Pulmonary Arterial Hypertension

    OpenAIRE

    Wapner, Joanna; Matura, Lea Ann

    2015-01-01

    Pulmonary arterial hypertension (PAH) is a progressive disease that ultimately leads to right heart failure and death. PAH is defined as a mean pulmonary arterial pressure ≥ 25 mm Hg with a pulmonary capillary wedge pressure ≤ 15 mm Hg at rest. The diagnosis of PAH is one of exclusion; diagnostics include an extensive history, serology, chest radiograph, pulmonary function tests, ventilation/perfusion scan, transthoracic echocardiogram, and right heart catheterization. Treatment and care of p...

  4. Pulmonary hypertension in chronic interstitial lung diseases

    OpenAIRE

    Antonella Caminati; Roberto Cassandro; Sergio Harari

    2013-01-01

    Pulmonary hypertension (PH) is a common complication of interstitial lung diseases (ILDs), particularly in idiopathic pulmonary fibrosis and ILD associated with connective tissue disease. However, other lung diseases, such as combined pulmonary fibrosis and emphysema syndrome, pulmonary Langerhans cell histiocytosis, and lymphangioleiomyomatosis, may also include PH in their clinical manifestations. In all of these diseases, PH is associated with reduced exercise capacity and poor prognosis. ...

  5. How to Measure Peripheral Pulmonary Vascular Mechanics

    OpenAIRE

    Chesler, Naomi C; Argiento, Paola; Vanderpool, Rebecca; D’Alto, Michele; Naeije, Robert

    2009-01-01

    Pulmonary hypertension (PH) is initially a disease of the small, peripheral resistance arteries. Changes in these vessels are best assessed by measurement of pulmonary artery pressure at several levels of flow to generate multi-point pressure-flow curves. This approach is superior to the traditional single-point measurement of pulmonary vascular resistance (PVR) because it allows a flow-independent definition of the resistive properties of that portion of the pulmonary vascular bed and also p...

  6. Pulmonary Venous Obstruction in Cancer Patients

    OpenAIRE

    Chuang-Chi Liaw; Hung Chang; Tsai-Sheng Yang; Ming-Sheng Wen

    2015-01-01

    Background. We study the clinical significance and management of pulmonary venous obstruction in cancer patients. Methods. We conducted a prospective cohort study to characterize the syndrome that we term “pulmonary vein obstruction syndrome” (PVOS) between January 2005 and March 2014. The criteria for inclusion were (1) episodes of shortness of breath; (2) chest X-ray showing abnormal pulmonary hilum shadow with or without presence of pulmonary edema and/or pleural effusion; (3) CT scan demo...

  7. Group B streptococcal phospholipid causes pulmonary hypertension

    OpenAIRE

    Curtis, Jerri; Kim, Geumsoo; Wehr, Nancy B.; Levine, Rodney L

    2003-01-01

    Group B Streptococcus is the most common cause of bacterial infection in the newborn. Infection in many cases causes persistent pulmonary hypertension, which impairs gas exchange in the lung. We purified the bacterial components causing pulmonary hypertension and identified them as cardiolipin and phosphatidylglycerol. Synthetic cardiolipin or phosphatidylglycerol also induced pulmonary hypertension in lambs. The recognition that bacterial phospholipids may cause pulmonary hypertension in new...

  8. HIF and pulmonary vascular responses to hypoxia

    OpenAIRE

    Shimoda, Larissa A.; Steven S Laurie

    2013-01-01

    In the lung, acute reductions in oxygen lead to hypoxic pulmonary vasoconstriction, whereas prolonged exposures to hypoxia result in sustained vasoconstriction, pulmonary vascular remodeling, and the development of pulmonary hypertension. Data from both human subjects and animal models implicate a role for hypoxia-inducible factors (HIFs), oxygen-sensitive transcription factors, in pulmonary vascular responses to both acute and chronic hypoxia. In this review, we discuss work from our laborat...

  9. Mechanisms of Dysfunction in Senescent Pulmonary Endothelium

    OpenAIRE

    Jane-wit, Daniel; Chun, Hyung J.

    2012-01-01

    Age-dependent changes in pulmonary endothelium contribute to worsened clinical outcomes in elderly individuals. Due to altered pulmonary endothelial responses, older participants have increased vulnerability to infection-related sequelae, higher prevalence of pulmonary hypertension, mitigated DNA repair mechanisms, and attenuated parenchymal healing. Aberrant signaling in pulmonary endothelium undergird these clinical processes. In this review, we provide an overview of the work that has eluc...

  10. Pulmonary angiography with lopamidol 370

    International Nuclear Information System (INIS)

    Fifty-one consecutive patients who underwent pulmonary angiography with iopamidol, 370 mg/ml, were studied prospectively. Patients were asked to grade any heat or pain felt on injection, and were observed for coughing. Right atrial and pulmonary artery pressures were obtained before the first and after the final contrast agent injection. Pressure changes were compared with those in a consecutive group of 25 patients who underwent examinations performed with Na-meglumine diatrizoate, 370 mg/ml. Film quality was evaluated for patient motion. Iopamidol generated marked discomfort in only three patients. Pressure changes were largely unremarkable. In no case was there any significant motion artifact secondary to coughing. Iopamidol is a safe, well-tolerated contrast agent for pulmonary angiography. It improves image quality by nearly eliminating coughing

  11. Pulmonary alterations in Behcet's disease

    International Nuclear Information System (INIS)

    Purpose: This study aims to demonstrate pulmonary alterations (PA) in patients with Behcet's disease by using CT. Materials and methods: CTs of 50 patients with Behcet's disease and 20 others in a control group have been evaluated retrospectively for PA (septal, reticular, nodular, atelectatic opacities). Results: Eight out of 50 patients (16%) with Behcet's disease showed PA. Three out of 20 (15%) in the control group showed PA. No differences were observed between Behcet's disease patients and the control group regarding pulmonary alterations (p = 0.917). No differences were observed in the disease duration, ages and sex in either group in those with and without PA. Conclusion: Pulmonary alterations can be seen in patients with Behcet's disease, but these alterations are not significant.

  12. Primary pulmonary neoplasms in children

    International Nuclear Information System (INIS)

    Primary pulmonary tumors are extremely uncommon in children, so individual experience in their medical management is limited. Our paper shows the manifestations, the diagnostic procedures used and the evolution of 17 patients that were assisted in our hospital through years 1991-2000. Radiology enables us to evaluate the presence of a pulmonary mass. US showed if it was solid, cystic or mixed and the presence or absence of pleural fluid. CT showed better the localization, extension and presence of metastasis, essential knowledge for planning and performing surgery. In a pediatric patient with a pulmonary mass and also in patients with spontaneous pneumothorax or hemo thorax a primary tumor must be considered as a possibility, and diagnostic procedures with US, CT and endoscopy performed. Congenital cystic lesions can become malignant, so they must always be treated by surgery. (author)

  13. Epidemiology of idiopathic pulmonary fibrosis

    Directory of Open Access Journals (Sweden)

    Ley B

    2013-11-01

    Full Text Available Brett Ley, Harold R Collard Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of California San Francisco, San Francisco, California, USA Abstract: Idiopathic pulmonary fibrosis is a chronic fibrotic lung disease of unknown cause that occurs in adults and has a poor prognosis. Its epidemiology has been difficult to study because of its rarity and evolution in diagnostic and coding practices. Though uncommon, it is likely underappreciated both in terms of its occurrence (ie, incidence, prevalence and public health impact (ie, health care costs and resource utilization. Incidence and mortality appear to be on the rise, and prevalence is expected to increase with the aging population. Potential risk factors include occupational and environmental exposures, tobacco smoking, gastroesophageal reflux, and genetic factors. An accurate understanding of its epidemiology is important, especially as novel therapies are emerging. Keywords: idiopathic pulmonary fibrosis, epidemiology, incidence, prevalence, mortality, risk factors

  14. [Pulmonary hemorrhage complicating transthoracic puncture].

    Science.gov (United States)

    Febvre, M; de Fenoyl, O; Capron, F; Vadrot, D; Rochemaure, J

    1989-01-01

    Fine needle transthoracic aspiration (FNTA) of pulmonary opacities under guidance of computerized tomography (CT) is a simple procedure yielding conclusive results in many patients with malignant lung tumours. The small caliber of the needles utilized and the CT control make this increasingly popular procedure both accurate and safe. Yet a number of complications have occurred; most of them were benign (e.g. pneumothorax) but some were potentially dangerous (e.g. pulmonary haemorrhage), so that the pros and cons of the procedure must carefully be weighed. We report the case of a patient in whom FNTA was complicated by copious haemoptysis and a pulmonary haematoma clearly visible at CT. The literature concerning the potential complications of transthoracic puncture is reviewed. PMID:2633295

  15. Pulmonary hypertension in antiphospholipid syndrome.

    Science.gov (United States)

    Zuily, Stéphane; Wahl, Denis

    2015-01-01

    Pulmonary hypertension (PH) is a rare but life-threatening condition in antiphospholipid syndrome (APS) patients with or without systemic lupus erythematosus (SLE). The definition of PH is based on hemodynamic parameters estimated by transthoracic echocardiography (TTE) and confirmed by right heart catheterization (RHC). New evidence suggests that antiphospholipid antibodies (aPL) in SLE patients increase the risk of PH; however, studies yield conflicting results. Hypotheses regarding the impact of aPL on PH include large vessel and microvascular thrombosis, and endothelial remodeling. Natural history of PH is progressive worsening mainly due to recurrent pulmonary embolism. The management in APS patients includes anticoagulation; patients undergoing pulmonary endarterectomy need to be closely monitored because of an increased risk of thrombotic complications. PMID:25604574

  16. Nitric oxide treatment for fulminant pulmonary hypertension.

    OpenAIRE

    Allman, K G; Young, J D; Stevens, J E; Archer, L N

    1993-01-01

    A 3 year old child with known pulmonary haemosiderosis suffered acute circulatory collapse secondary to raised pulmonary vascular resistance. Nitric oxide inhalation produced a profound improvement in circulatory parameters and gaseous exchange. Nitric oxide may have a therapeutic role in acute pulmonary hypertensive crisis.

  17. Lutembacher Syndrome and Rheumatic Pulmonary Stenosis

    Institute of Scientific and Technical Information of China (English)

    Jufang Chi; Hangyuan Guo; Biao Yang

    2008-01-01

    We discdbed a case of a 55-yr-old woman diagnosed with Lutembacher syndrome and rheumatic pulmonary stenosis.Congenital atrial septal defect was found in age 7 and rheumatic fever in age 34.As the patient developed pulmonary hypertension with calcified mitral valve leaflet and pulmonary stenosis so surgery was not indicated.So the patient was managed by medical therapy alone.

  18. High altitude pulmonary edema: case report

    International Nuclear Information System (INIS)

    Acute pulmonary edema is characterized by the accumulation of liquid in the pulmonary interstice, the alveoli, the bronchi and bronchioles; it is from the excessive circulation from the pulmonary vascular system towards extra vascular and the respiratory spaces. The Liquid filters first at the interstitial space to soon perivascular and peri bronchial and, gradually, towards the alveoli and bronchi

  19. Pulmonary complications of upper abdominal surgery.

    Directory of Open Access Journals (Sweden)

    Deodhar S

    1991-04-01

    Full Text Available Pulmonary complications encountered in 67 patients undergoing upper abdominal surgery in our unit in one year period are analysed. Pulmonary function tests and their post-operative reduction, as also the risk factors are discussed. Pathophysiology responsible for pulmonary complications is outlined.

  20. Making pulmonary rehabilitation a success in COPD.

    Science.gov (United States)

    Bourbeau, J

    2010-01-01

    A truly successful pulmonary rehabilitation entails implementing physical activity maintenance. This article reviews the current knowledge on pulmonary rehabilitation and the expected benefits, the setting, the relationship between self-management and pulmonary rehabilitation, in order to develop and implement clinically-effective physical activity maintenance interventions. The effectiveness of pulmonary rehabilitation is well-established. However, access to pulmonary rehabilitation is limited. Home-based pulmonary rehabilitation has been shown to be an effective, equivalent alternative to outpatient pulmonary rehabilitation in COPD patients. The opportunity to offer different pulmonary rehabilitation settings tailored to individual needs should improve accessibility to this intervention. Sustained long-term physical activity remains the most important challenge for COPD patients. We need a dependable system of coordinated health care interventions and communication, and components that include self-management support. Self-management should be an integrated part of pulmonary rehabilitation and remain long after the pulmonary rehabilitation is completed. By early identification of patients who may have difficulty maintaining exercise and implementing appropriate self-management interventions during and after the rehabilitation program, it may be possible to promote better long-term involvement in physical activity. Pulmonary rehabilitation should not stand alone; the best program is that which can be maintained to translate into a continuous increase in the activities of daily living. Future research should evaluate the effect of self-management interventions combined with pulmonary rehabilitation to improve long-term activity and exercise maintenance. PMID:20809435

  1. Asymptomatic Pulmonary Hypertension in Systemic Lupus Erythematosus

    OpenAIRE

    Kamel, Shereen R.; Omar, Gihan M.; Darwish, Ayman F.; Asklany, Hany T.; Abdou S. Ellabban

    2011-01-01

    Introduction: Pulmonary arterial hypertension (PAH) is a serious and often fatal complication of systemic lupus erythematosus (SLE). Because the diagnosis of PAH often is made years after symptom onset, early diagnostic strategies are essential. Doppler echocardiography currently is considered the noninvasive screening test of choice for evaluating pulmonary hypertension. Aim: Screening for asymptomatic pulmonary hypertension in systemic lupus erythematosus patients using Doppler echocardiogr...

  2. Meandering right pulmonary vein associated with severe and progressive "idiopathic-like" pulmonary hypertensive vascular disease.

    Science.gov (United States)

    Cuenca, Sofia; Bret, Montserrat; Del Cerro, Maria Jesus

    2016-03-01

    Congenital anomalies of the pulmonary veins are rare. Meandering right pulmonary vein, considered a part of the Scimitar syndrome spectrum, is often an incidental finding during chest imaging. We present the case of a 4-year-old girl diagnosed with meandering pulmonary vein, who developed pulmonary hypertensive disease with an aggressive course, in spite of absence of hypoxia or elevated pulmonary wedge pressure. PMID:26374461

  3. Chronic Thromboembolic Pulmonary Hypertension after Pulmonary Embolism, Thrombolysis, Catheter Fragmentation, and Embolectomy

    OpenAIRE

    Wiedenroth, Christoph B.; Guth, Stefan; Rolf, Andreas; Mayer, Eckhard

    2014-01-01

    A 21-year-old male patient with massive acute pulmonary embolism was treated by thrombolysis, interventional thrombus fragmentation, and surgical pulmonary embolectomy. Within the following 2 years, the patient developed progressive dyspnea at exertion. Chronic thromboembolic pulmonary hypertension was diagnosed by right-heart catheter, VQ scan, magnetic resonance, and conventional pulmonary angiography. A normalization of the patient's exercise capacity and pulmonary hemodynamics could be ac...

  4. Pulmonary capillary blood volume in patients with probable pulmonary Kaposi's sarcoma.

    OpenAIRE

    Camus, F.; de Picciotto, C.; Gerbe, J; S Roy; Bouchaud, O.; E. Casalino; Perronne, C

    1996-01-01

    BACKGROUND: An increase in pulmonary capillary blood volume secondary to angiogenesis has been described in Kaposi's sarcoma. The value of the pulmonary capillary blood volume as an early marker of pulmonary Kaposi's sarcoma was evaluated. METHODS: In a prospective study 45 HIV positive patients (nine asymptomatic for Kaposi's sarcoma, 29 with cutaneous or mucocutaneous Kaposi's sarcoma, and seven with pulmonary Kaposi's sarcoma), underwent pulmonary function tests and determination of transf...

  5. Acute effects of riociguat in borderline or manifest pulmonary hypertension associated with chronic obstructive pulmonary disease

    OpenAIRE

    Ghofrani, Hossein A.; Staehler, Gerd; Grünig, Ekkehard; Halank, Michael; Mitrovic, Veselin; Unger, Sigrun; Mueck, Wolfgang; Frey, Reiner; Grimminger, Friedrich; Ralph T. Schermuly; Behr, Juergen

    2015-01-01

    Riociguat is the first oral soluble guanylate cyclase stimulator shown to improve pulmonary hemodynamics in patients with pulmonary arterial hypertension and chronic thromboembolic pulmonary hypertension (PH). This pilot study assessed the impact of a single dose of riociguat on hemodynamics, gas exchange, and lung function in patients with PH associated with chronic obstructive pulmonary disease (COPD). Adults with COPD-associated borderline or manifest PH (pulmonary vascular resistance > 27...

  6. Pulmonary vascular mechanics: Important contributors to the increased right ventricular afterload of pulmonary hypertension

    OpenAIRE

    Wang, Zhijie; Chesler, Naomi C

    2013-01-01

    Chronic hypoxia causes pulmonary vasoconstriction and vascular remodeling, which lead to hypoxic pulmonary hypertension (HPH). HPH is associated with living at high altitudes and is a complication of many lung diseases, including chronic obstructive pulmonary disease, cystic fibrosis, and obstructive sleep apnea. Pulmonary vascular changes that occur with HPH include stiffening and narrowing of the pulmonary arteries that appear to involve all vascular cell types and sub-layers of the arteria...

  7. Pulmonary vascular wall stiffness: An important contributor to the increased right ventricular afterload with pulmonary hypertension

    OpenAIRE

    Wang, Zhijie; Chesler, Naomi C

    2011-01-01

    Pulmonary hypertension (PH) is associated with structural and mechanical changes in the pulmonary vascular bed that increase right ventricular (RV) afterload. These changes, characterized by narrowing and stiffening, occur in both proximal and distal pulmonary arteries (PAs). An important consequence of arterial narrowing is increased pulmonary vascular resistance (PVR). Arterial stiffening, which can occur in both the proximal and distal pulmonary arteries, is an important index of disease p...

  8. Pulmonary Complications due to Esophagectomy

    Directory of Open Access Journals (Sweden)

    Yashar Talebi

    2011-08-01

    Full Text Available Introduction: Esophageal carcinoma is the scourge of human beings. Pulmonary compli-cations in patients who have undergone operation are common (20-30% of cases and there are no suitable tools and ways to predict these complications. Methods: During a period of 10 years, from March 1998 to February 2007, 200 patients (150 male and 50 female underwent Esophagectomy due to esophageal carcinoma in thoracic surgery ward retrospectively. Complications include the length of hospitalization, mechanical ventilation, morbidity and mortality. Patients’ risk factors include age, preoperative chemo-radiotherapy, stage of the disease and preoperative spirometry condition. Results: We grouped our patients into three categories: Normal (FEV1 ≥ 80% predicted, mildly impaired (FEV1 65% to 79% predicted, more severely impaired (FEV1 < 65% predicted.Although almost all patients had radiographic pulmonary abnormalities, significant pulmonary complications occurred in 40 patients (20% which underwent Esophagectomy. Pleural effusion and atelectasia in 160 patients (80%. 24 patients needed chest-tube insertion. 20 patients (10% developed ARDS. 14 patients (7% developed chylothorax. 20 patients (10% of patients died during their postoperative hospital stay. 30 patients (15% required mechanical ventilation for greater than 48 hours. Conclusion: We reviewed a number of preoperative clinical variables to determine whether they contributed to postoperative pulmonary complications as well as other outcomes. In general, age, impaired pulmonary function especially in those patients with FEV1 less than 65% predicted was associated with prolonged hospital length of stay (LOS. In fact pulmonary complications rate after Esophagectomy are high and there was associated mortality and morbidity.

  9. Hypoxic pulmonary vasoconstriction in humans.

    Science.gov (United States)

    Ariyaratnam, Priyadharshanan; Loubani, Mahmoud; Morice, Alyn H

    2013-01-01

    Hypoxic pulmonary vasoconstriction is the elegant theory put forward more than six decades ago to explain regional variations in perfusion within the lung in certain animal species in response to localised restrictions in oxygenation. Although considerable progress has been made to describe the phenomenon at the macroscopic level and explain it at the microscopic level, we are far from a universal agreement about the process in humans. This review attempts to highlight some of the important evidence bases of hypoxic pulmonary vasoconstriction in humans and the significant gaps in our knowledge that would need bridging. PMID:24024204

  10. Systemic to pulmonary vascular malformation.

    Science.gov (United States)

    Pouwels, H M; Janevski, B K; Penn, O C; Sie, H T; ten Velde, G P

    1992-11-01

    A case is reported of life-threatening haemoptysis as a result of an anomalous communication between a bronchial artery and pulmonary vein, demonstrated by angiography. The patient recovered following bilobectomy of the right lower and middle lobes. When a systemic artery is involved in an arteriovenous malformation of the lung, haemodynamics are different compared with those present in malformations fed by the pulmonary artery. This implicates other clinical features, options for surgical intervention and prognosis. In reviewing the literature, a relationship with Rendu-Osler-Weber disease is absent in these specific malformations. PMID:1486979

  11. Reexpansion pulmonary edema following thoracentesis

    Directory of Open Access Journals (Sweden)

    Ansuman Mukhopadhyay

    2016-01-01

    Full Text Available Reexpansion pulmonary edema is an uncommon complication of the treatment of lung atelectasis, pleural effusion or pneumothorax and pathogenesis is unknown. An elderly male patient presented to us with right-sided pleural effusion. 2 h after thoracentesis, he felt chest discomfort and increased breathlessness. His chest examination showed right-sided crackles. Chest radiograph showed right-sided heterogeneous opacity in right lower zone consistent with unilateral pulmonary edema. He was managed conservatively along with bilevel positive airway pressure ventilator support. His condition improved gradually and was discharged successfully after 2 days.

  12. Pulmonary complications of cystic fibrosis

    International Nuclear Information System (INIS)

    The life expectancy of patients with cystic fibrosis (CF) has steadily increased over recent decades with a corresponding increase in the frequency of complications of the disease. Radiologists are increasingly involved with managing and identifying the pulmonary complications of CF. This article reviews the common manifestations of CF lung disease as well as updating radiologists with a number of less well-known complications of the condition. Early and accurate detection of the pulmonary effects of CF are increasingly important to prevent irreversible lung damage and give patients the greatest possibility of benefiting from the new therapies becoming available, which correct the underlying defect causing CF

  13. LUNG CANCER AND PULMONARY THROMBOEMBOLISM

    OpenAIRE

    Cukic, Vesna; Ustamujic, Aida

    2015-01-01

    Introduction: Malignant diseases including lung cancer are the risk for development of pulmonary thromboembolism (PTE). Objective: To show the number of PTE in patients with lung cancer treated in Clinic for pulmonary diseases and TB “Podhrastovi” in three-year period: from 2012-2014. Material and methods: This is the retrospective study in which we present the number of various types of lung cancer treated in three-year period, number and per cent of PTE in different types of lung carcinoma,...

  14. Contribution of live heartworms harboring in pulmonary arteries to pulmonary hypertension in dogs with dirofilariasis.

    Science.gov (United States)

    Kitagawa, H; Sasaki, Y; Ishihara, K; Hirano, Y

    1990-12-01

    To investigate whether adult heartworms harboring in the pulmonary arteries contribute to pulmonary hypertension, we determined the cardio-pulmonary values immediately before and after removal of heartworms from the pulmonary arteries and before and after insertion of live worms in their place. In 10 heartworm-infected dogs, 8 to 46 worms were removed. The mean pulmonary arterial pressure fell significantly from 24.5 +/- 7.9 mmHg to 16.3 +/- 4.9 mmHg (p less than 0.01) immediately after removal. The right cardiac output decreased in 7 of the 10 cases. The total pulmonary resistance and right ventricular stroke work index also decreased. At 24 hours after removal, live heartworms were put back into the pulmonary arteries of their host dog. The mean pulmonary arterial pressure elevated significantly (p less than 0.01) immediately after insertion. The right cardiac output further decreased in 7 of the 10 dogs, and the total pulmonary resistance and right ventricular stroke work index increased. Separate from this, 12 to 42 heartworms were transplanted into the pulmonary arteries of 5 heartworm-free dogs. Immediately after transplantation, the pulmonary arterial pressure did not show any significant change. However, the stroke volume decreased, and the total pulmonary resistance increased. These facts suggest a contribution of live heartworms to the pulmonary hypertension, although there is a complicated interaction among the presence of heartworms, the pulmonary lesions and the pulmonary hypertension. PMID:2287128

  15. Acute exacerbations and pulmonary hypertension in advanced idiopathic pulmonary fibrosis.

    LENUS (Irish Health Repository)

    Judge, Eoin P

    2012-07-01

    The aim of this study was to evaluate the risk factors for and outcomes of acute exacerbations in patients with advanced idiopathic pulmonary fibrosis (IPF), and to examine the relationship between disease severity and neovascularisation in explanted IPF lung tissue. 55 IPF patients assessed for lung transplantation were divided into acute (n=27) and non-acute exacerbation (n=28) groups. Haemodynamic data was collected at baseline, at the time of acute exacerbation and at lung transplantation. Histological analysis and CD31 immunostaining to quantify microvessel density (MVD) was performed on the explanted lung tissue of 13 transplanted patients. Acute exacerbations were associated with increased mortality (p=0.0015). Pulmonary hypertension (PH) at baseline and acute exacerbations were associated with poor survival (p<0.01). PH at baseline was associated with a significant risk of acute exacerbations (HR 2.217, p=0.041). Neovascularisation (MVD) was significantly increased in areas of cellular fibrosis and significantly decreased in areas of honeycombing. There was a significant inverse correlation between mean pulmonary artery pressure and MVD in areas of honeycombing. Acute exacerbations were associated with significantly increased mortality in patients with advanced IPF. PH was associated with the subsequent development of an acute exacerbation and with poor survival. Neovascularisation was significantly decreased in areas of honeycombing, and was significantly inversely correlated with mean pulmonary arterial pressure in areas of honeycombing.

  16. [Pulmonary function in patients with focal pulmonary tuberculosis].

    Science.gov (United States)

    Nefedov, V B; Popova, L A; Shergina, E A

    2008-01-01

    Vital capacity (VC), forced vital capacity (FVC), forced expiratory volume in 1 second (FEV1), FEV1/VC%, PEF, MEF25, MEF50, MEF75, TLC, TGV, pulmonary residual volume (PRV), Raw, Rin, Rcx, DLCO-SB, DLCO-SS/VA, PaO2, and PaCO2 were determined in 40 patients with focal pulmonary tuberculosis. Changes were found in lung volumes and capacities in 75%, impaired bronchial patency and pulmonary gas exchange dysfunction were in 57.5 and 25%, respectively. The lung volume and capacity changes appeared mainly as increased TGV and PRV; impaired bronchial patency presented as decreased MEF50, MEF75, and FEV1/VC%; pulmonary gas exchange dysfunction manifested itself as reduced DLCO-SB, PaO2, and PaCO2. The magnitude of the observed functional changes was generally slight. TGV and PRL increased up to 148-187 and 142-223% of the normal values, respectively; MEF50, MEF75, FEV1/VC%, and DLCO decreased to 59-24, 58-26, 78-57, and 78-67% of the normal values and PaO2 and PaCO2 did to 79-69 and 34-30 cm Hg. PMID:18450075

  17. SERIES "NOVELTIES IN PULMONARY REHABILITATION" New tools in pulmonary rehabilitation

    NARCIS (Netherlands)

    Wijkstra, P. J.; Wempe, J. B.

    2011-01-01

    In patients with more severe chronic obstructive pulmonary disease ( COPD), the benefits of rehabilitation might not be clear and, therefore, new treatment options have been developed to increase the benefits of rehabilitation. This review provides an overview of new approaches being developed as an

  18. Pulmonary embolism and pulmonary infarction; Lungenembolie und Lungeninfarkt - pathologische Anatomie

    Energy Technology Data Exchange (ETDEWEB)

    Mueller, K.M.; Mueller, A.M. [Berufsgenossenschaftliche Kliniken Bergmannsheil, Bochum (Germany). Inst. fuer Pathologie]|[Bochum Univ. (Germany). Universitaetsklinikum

    1998-03-01

    Radiological and nuclear medical evaluation of pulmonary embolisms and their consequences is often problematic, since parenchymal alterations in the form of possible pulmonary infarctions occur in only 10-15% after vessel obliteration. Small embolisms rather frequently cause hemorrhagic pulmonary infarctions, which can clinically be demonstrated by radiological and nuclear medical methods, after obliteration of the pre-capillary arterio-arterial anastomoses type I. In pre-existing chronic lung diseases with often markedly developed bronchial artery systems and additional anastomoses hemorrhagic pulmonary infarctions are extremely rare. Thus, today, radiological and nuclear medical studies, such as spiral computer scanning, have to rely largely on the results of thrombembolic vessel obstruction and transitory perfusion deficits and less on parenchymal infiltration patterns. (orig./MG) [Deutsch] Die radiologischen und nuklearmedizinischen Begutachtungen von Lungenembolien und deren Folgen sind oft problematisch, weil Parenchymveraenderungen in Form moeglicher Lungeninfarkte in nur 10-15% nach der Gefaessverlegung entstehen. Kleine Embolien fuehren haeufiger zu haemorrhagischen Lungeninfarkten, die mit radiologischen und nukelarmedizinischen Verfahren fassbar werden. Bei vorbestehenden chronischen Lungenerkrankungen mit meist verstaerkt ausgebautem Bronchialartheriensystem und zusaetzlichen Anatomosen sind haemorrhagische Lungeninfarkte besonders selten. Die radiologische und nuklearmedizinische Diagnostik muss sich daher heute nach vielversprechenden Studien, z.B. unter Einsatz der Spiralcomputertomographie, wesentlich auf die Befunde der thrombembolischen Gefaessobstruktion und transitorische Perfusionsausfaelle und weniger auf parenchymatoese Infiltratmuster stuetzen. (orig./MG)

  19. Analysis of perfusion defects by causes other than acute pulmonary thromboembolism on contrast-enhanced dual-energy CT in consecutive 537 patients

    International Nuclear Information System (INIS)

    Objective: To assess causes, incidence and patterns of perfusion defects (PDs) on dual-energy perfusion CT angiography (DECTA) in clinically suspected acute pulmonary thromboembolisms (PTE). Materials and methods: Consecutive 537 patients who underwent DECTA for suspicion of PTE were retrospectively reviewed. After excluding patients with possible PTE or unsatisfactory perfusion map quality, 299 patients with 1697 lobes were included. The DECTA (Somatom Definition, Siemens) was performed at 140 kV and 80 kV. Color-coded perfusion images were obtained with a lung PBV application of the workstation software (Syngo Dual Energy). The presence, incidence, three patterns of PDs (wedge-shaped, heterogeneous, and regionally homogeneous), pulmonary diseases, and the matchedness between the PD and the disease extent were studied. Results: 315 of 1697 lobes (18.6%) in 156 of 299 patients (81.3%) showed PDs. Among them, 51 (3%), 257 (15.1%), and 7 (0.4%) lobes had PDs due to vascular, nonvascular, and unidentifiable causes, respectively. Vascular causes include: pulmonary arterial (PA) hypertension (0.7%), extrinsic occlusion of PA by fibrosis (0.6%), PA hypoplasia (0.6%), vasculitis (0.5%), cancer mass compressing PA, venous occlusion, AVM, and pulmonary angiosarcoma. Most of PDs were wedge-shaped and well-matched. Nonvascular causes include: mosaic attenuation (4.1%), emphysema (3.2%), interstitial fibrosis (1.6%), bronchitis (1.4%), GGO (1.2%), cellular bronchiolitis (1%), bronchiectasis, airway obstruction, compensaroty lung hyperinflation, air trapping, cor-pulmonale, bronchopneumonia, physiologic decreased ventilation, and segmental bronchial atresia. Most of PDs showed heterogeneous pattern and were not matched. Conclusions: Various vascular and nonvascular diseases cause PDs on DECTA. Each disease shows different pattern of PD depending on pathophysiology and physiologic compensation.

  20. Pulmonary embolism in adolescents

    Institute of Scientific and Technical Information of China (English)

    MA Qing-bian; YAO Wan-zhen; CHEN Jian-ming; GE Hong-xia; LI Shu; ZHENG Ya-an

    2012-01-01

    Background Pulmonary embolism (PE) is rare and seldom considered in adolescent patients; however it occurs with a greater frequency than is generally recognized,and it is a potentially fatal condition.The aim of the current study was to understand its epidemiology,clinical features and the cause of delay of its diagnosis in adolescents.Methods A retrospective analysis of nine adolescents with acute PE admitted to the Peking University Third Hospital over the past 16-year period was performed.The epidemiology,clinical features and risk factors of the adolescents were described and compared with those of adults and elderly patients.The time to diagnosis and misdiagnosed diseases were analyzed.Pretest probability of PE was assessed retrospectively by the Wells score and revised Geneva score.Results The incidence of PE was 43.6 per 100 000 hospitalized adolescents in our hospital.The incidence of PE in adolescents was much lower than that in adults and PE is diagnosed in about 1/50 of elderly people.The clinical features in adolescents were similar to those in adults.But fever and chest pain were more common in adolescents (P<0.05).The major risk factors included surgery,systemic lupus erythematosus (SLE),thrombocytopenia,long-term oral glucocorticoids and trauma.The mean diagnostic time was (7.8±8.4) days.Six cases had a delayed diagnosis.The mean delay time from symptom onset to diagnosis was (11.0±8.8) days.The time of presentation to diagnosis in patients initially admitted to the emergency department was less than one day,and was much shorter than the time in outpatients,(9.4±7.5) days.Most of the patients were initially misdiagnosed with a respiratory tract infection.Most patients' values of Wells score or revised Geneva score were in the moderate or high clinical probability categories; 88% by Well score vs.100% by revised Geneva score.Conclusions PE was seldom considered in the adolescent patients by physicians,especially outpatient physicians,so the

  1. Both rare and de novo copy number variants are prevalent in agenesis of the corpus callosum but not in cerebellar hypoplasia or polymicrogyria.

    Directory of Open Access Journals (Sweden)

    Samin A Sajan

    Full Text Available Agenesis of the corpus callosum (ACC, cerebellar hypoplasia (CBLH, and polymicrogyria (PMG are severe congenital brain malformations with largely undiscovered causes. We conducted a large-scale chromosomal copy number variation (CNV discovery effort in 255 ACC, 220 CBLH, and 147 PMG patients, and 2,349 controls. Compared to controls, significantly more ACC, but unexpectedly not CBLH or PMG patients, had rare genic CNVs over one megabase (p = 1.48×10⁻³; odds ratio [OR] = 3.19; 95% confidence interval [CI] = 1.89-5.39. Rare genic CNVs were those that impacted at least one gene in less than 1% of the combined population of patients and controls. Compared to controls, significantly more ACC but not CBLH or PMG patients had rare CNVs impacting over 20 genes (p = 0.01; OR = 2.95; 95% CI = 1.69-5.18. Independent qPCR confirmation showed that 9.4% of ACC patients had de novo CNVs. These, in comparison to inherited CNVs, preferentially overlapped de novo CNVs previously observed in patients with autism spectrum disorders (p = 3.06×10⁻⁴; OR = 7.55; 95% CI = 2.40-23.72. Interestingly, numerous reports have shown a reduced corpus callosum area in autistic patients, and diminished social and executive function in many ACC patients. We also confirmed and refined previously known CNVs, including significantly narrowing the 8p23.1-p11.1 duplication present in 2% of our current ACC cohort. We found six novel CNVs, each in a single patient, that are likely deleterious: deletions of 1p31.3-p31.1, 1q31.2-q31.3, 5q23.1, and 15q11.2-q13.1; and duplications of 2q11.2-q13 and 11p14.3-p14.2. One ACC patient with microcephaly had a paternally inherited deletion of 16p13.11 that included NDE1. Exome sequencing identified a recessive maternally inherited nonsense mutation in the non-deleted allele of NDE1, revealing the complexity of ACC genetics. This is the first systematic study of CNVs in congenital brain malformations, and

  2. Pulmonary Hypertension in Pregnancy: Critical Care Management

    Directory of Open Access Journals (Sweden)

    Adel M. Bassily-Marcus

    2012-01-01

    Full Text Available Pulmonary hypertension is common in critical care settings and in presence of right ventricular failure is challenging to manage. Pulmonary hypertension in pregnant patients carries a high mortality rates between 30–56%. In the past decade, new treatments for pulmonary hypertension have emerged. Their application in pregnant women with pulmonary hypertension may hold promise in reducing morbidity and mortality. Signs and symptoms of pulmonary hypertension are nonspecific in pregnant women. Imaging workup may have undesirable radiation exposure. Pulmonary artery catheter remains the gold standard for diagnosing pulmonary hypertension, although its use in the intensive care unit for other conditions has slowly fallen out of favor. Goal-directed bedside echocardiogram and lung ultrasonography provide attractive alternatives. Basic principles of managing pulmonary hypertension with right ventricular failure are maintaining right ventricular function and reducing pulmonary vascular resistance. Fluid resuscitation and various vasopressors are used with caution. Pulmonary-hypertension-targeted therapies have been utilized in pregnant women with understanding of their safety profile. Mainstay therapy for pulmonary embolism is anticoagulation, and the treatment for amniotic fluid embolism remains supportive care. Multidisciplinary team approach is crucial to achieving successful outcomes in these difficult cases.

  3. Pulmonary Hypertension in the Intensive Care Unit.

    Science.gov (United States)

    Jentzer, Jacob C; Mathier, Michael A

    2016-07-01

    Pulmonary hypertension occurs as the result of disease processes increasing pressure within the pulmonary circulation, eventually leading to right ventricular failure. Patients may become critically ill from complications of pulmonary hypertension and right ventricular failure or may develop pulmonary hypertension as the result of critical illness. Diagnostic testing should evaluate for common causes such as left heart failure, hypoxemic lung disease and pulmonary embolism. Relatively few patients with pulmonary hypertension encountered in clinical practice require specific pharmacologic treatment of pulmonary hypertension targeting the pulmonary vasculature. Management of right ventricular failure involves optimization of preload, maintenance of systemic blood pressure and augmentation of inotropy to restore systemic perfusion. Selected patients may require pharmacologic therapy to reduce right ventricular afterload by directly targeting the pulmonary vasculature, but only after excluding elevated left heart filling pressures and confirming increased pulmonary vascular resistance. Critically-ill patients with pulmonary hypertension remain at high risk of adverse outcomes, requiring a diligent and thoughtful approach to diagnosis and treatment. PMID:25944777

  4. How Is Pulmonary Embolism Diagnosed?

    Science.gov (United States)

    ... are available shortly after the scan is done. Lung Ventilation/Perfusion Scan A lung ventilation/perfusion scan , or VQ scan, uses a radioactive substance to show how well oxygen and blood are flowing to all areas of your lungs. This test can help detect PE. Pulmonary Angiography ...

  5. Radiological case: idiopathic pulmonary hemosiderosis

    OpenAIRE

    Nascimento , J.; Morais, L; Macedo, F.

    2012-01-01

    ABSTRACT We present a case of a four year-old child with a second episode of iron deficiency anaemia. Following fever, cough and hemoptysis a chest X-ray and chest CT were performed. Bilateral and patchy areas of ground glass and consolidation were noted in the setting of intrapulmonary hemorrhage. The final diagnosis was idiopathic pulmonary hemosiderosis.

  6. Pulmonary Metastasis from Pseudomyxoma Peritonei

    Directory of Open Access Journals (Sweden)

    Toshiyuki Kitai

    2012-01-01

    Full Text Available Pseudomyxoma peritonei (PMP is a rare clinical condition, where copious mucinous ascites accumulate in the peritoneal cavity due to dissemination of mucin-producing tumor. Because of this disseminating, yet nonmetastasizing, behavior, PMP attracts much interest from surgical oncologists in that aggressive locoregional therapy can give the opportunity of long survival and even cure. Although extra-abdominal metastasis is exceptionally rare, the lung is the most likely site in such a case. In this paper, the clinical findings and treatment of eleven cases with pulmonary metastasis from PMP were reviewed, including ten cases in the literature and one case which we experienced. The clinical features of PMP cases with pulmonary metastasis were similar to cases without pulmonary metastasis. The histological type was low-grade mucinous neoplasm in most cases. Pulmonary lesions were resected in seven cases in which abdominal lesions were controlled by cytoreductive surgery and hyperthermic intraperitoneal chemotherapy or another therapeutic modality. Disease-free state was maintained in five cases at the end of the follow-up period. However, it should be noted that rapid progression after resection was seen in two cases, suggesting that biological features may have changed by surgical intervention.

  7. Management of pulmonary arterial hypertension.

    LENUS (Irish Health Repository)

    Judge, Eoin P

    2013-02-01

    Pulmonary arterial hypertension (PAH) is a complex disease with a high mortality. Management of this disease is underpinned by supportive and general therapies delivered by multidisciplinary teams in specialist centres. In recent years, a number of PAH-specific therapies have improved patient outcomes. This article will discuss the management of PAH in the context of relevant recently published studies in this area.

  8. Pulmonary Testing Laboratory Computer Application

    OpenAIRE

    Johnson, Martin E.

    1980-01-01

    An interactive computer application reporting patient pulmonary function data has been developed by Washington, D.C. VA Medical Center staff. A permanent on-line data base of patient demographics, lung capacity, flows, diffusion, arterial blood gases and physician interpretation is maintained by a minicomputer at the hospital.

  9. MRI of the pulmonary parenchyma

    International Nuclear Information System (INIS)

    Imaging of the pulmonary parenchyma represents a unique challenge for MRI. Limited signal is caused by low proton density, susceptibility artifacts, and physiological motion (cardiac pulsation, respiration). Recently, further improvements in MRI techniques have widened the potential for investigations of pulmonary parenchymal disease. These include very short echo times, ultrafast turbo-spin-echo acquisitions, projection reconstruction technique, breathhold imaging, ECG triggering, contrast agents (perfusion imaging, aerosols), sodium imaging, hyperpolarized noble gas imaging, and oxygen enhancement. By using widely available techniques, MRI is helpful in the assessment of (a) acute alveolitic processes in chronic infiltrative lung disease, (b) detection and characterization of pulmonary nodules, (c) detection, characterization, and follow-up of pneumonia, (d) differentiation of obstructive atelectasis from non-obstructive atelectasis and infarctions, and (e) measurements of lung water content. Chronic bronchitis, bronchiectasis, and emphysema are not readily assessable by routine MRI techniques. More sophisticated techniques are under investigation for MR imaging of pulmonary ventilation and perfusion. They represent the beginning of functional MR imaging of the lung which will be established in the future. (orig.)

  10. MRI of the pulmonary parenchyma

    Energy Technology Data Exchange (ETDEWEB)

    Kauczor, H.U.; Kreitner, K.F. [Mainz Univ. (Germany). Klinik und Poliklinik fuer Radiologie

    1999-07-01

    Imaging of the pulmonary parenchyma represents a unique challenge for MRI. Limited signal is caused by low proton density, susceptibility artifacts, and physiological motion (cardiac pulsation, respiration). Recently, further improvements in MRI techniques have widened the potential for investigations of pulmonary parenchymal disease. These include very short echo times, ultrafast turbo-spin-echo acquisitions, projection reconstruction technique, breathhold imaging, ECG triggering, contrast agents (perfusion imaging, aerosols), sodium imaging, hyperpolarized noble gas imaging, and oxygen enhancement. By using widely available techniques, MRI is helpful in the assessment of (a) acute alveolitic processes in chronic infiltrative lung disease, (b) detection and characterization of pulmonary nodules, (c) detection, characterization, and follow-up of pneumonia, (d) differentiation of obstructive atelectasis from non-obstructive atelectasis and infarctions, and (e) measurements of lung water content. Chronic bronchitis, bronchiectasis, and emphysema are not readily assessable by routine MRI techniques. More sophisticated techniques are under investigation for MR imaging of pulmonary ventilation and perfusion. They represent the beginning of functional MR imaging of the lung which will be established in the future. (orig.)

  11. Aspiration-related pulmonary syndromes.

    Science.gov (United States)

    Hu, Xiaowen; Lee, Joyce S; Pianosi, Paolo T; Ryu, Jay H

    2015-03-01

    Aspiration of foreign matter into the airways and lungs can cause a wide spectrum of pulmonary disorders with various presentations. The type of syndrome resulting from aspiration depends on the quantity and nature of the aspirated material, the chronicity, and the host responses. Aspiration is most likely to occur in subjects with a decreased level of consciousness, compromised airway defense mechanisms, dysphagia, gastroesophageal reflux, and recurrent vomiting. These aspiration-related syndromes can be categorized into airway disorders, including vocal cord dysfunction, large airway obstruction with a foreign body, bronchiectasis, bronchoconstriction, and diffuse aspiration bronchiolitis, or parenchymal disorders, including aspiration pneumonitis, aspiration pneumonia, and exogenous lipoid pneumonia. In idiopathic pulmonary fibrosis, aspiration has been implicated in disease progression and acute exacerbation. Aspiration may increase the risk of bronchiolitis obliterans syndrome in patients who have undergone a lung transplant. Accumulating evidence suggests that a causative role for aspiration is often unsuspected in patients presenting with aspiration-related pulmonary diseases; thus, many cases go undiagnosed. Herein, we discuss the broadening spectrum of these pulmonary syndromes with a focus on presenting features and diagnostic aspects. PMID:25732447

  12. [Choriocarcinoma causing a pulmonary embolus.

    DEFF Research Database (Denmark)

    Theliade, J.E.; Skovby, A.M.; Kirk, V.; Parvaiz, I.; Holmvang, L.

    2008-01-01

    A 32 year-old women experienced dyspnea and thoracic pain that persisted with variable intensity over a course of eight months until acute worsening necessitated admission. A CT scan demonstrated a central pulmonary embolus. Subsequent surgical embolectomy produced a grained substance that was hi...

  13. Rifampicin pulmospheres for pulmonary delivery

    Directory of Open Access Journals (Sweden)

    Morde M

    2009-01-01

    Full Text Available Dry powder inhalation formulations of rifampicin were prepared. Spray drying was used to prepare pulmospheres and their physicochemical characteristics were evaluated. Spray dried pulmospheres containing rifampicin were mixed with inhalable lactose for preparing dry powder inhalation formulations. These formulations were further characterized to evaluate the feasibility of developing effective treatments for pulmonary tuberculosis.

  14. Pulmonary ventilation/perfusion scan

    Science.gov (United States)

    ... Critical Care, Perelman School of Medicine, University of Pennsylvania, Philadelphia, PA. Also reviewed by David Zieve, MD, MHA, Isla Ogilvie, PhD, and the A.D.A.M. Editorial team. Related MedlinePlus Health Topics COPD Lung Diseases Nuclear Scans Pulmonary Embolism Browse the Encyclopedia A.D. ...

  15. Hantavirus Pulmonary Syndrome, French Guiana

    OpenAIRE

    Matheus, Séverine; Djossou, Félix; Moua, David; Bourbigot, Anne Marie; Hommel, Didier; Lacoste, Vincent; Dussart, Philippe; Lavergne, Anne

    2010-01-01

    A systematic serological survey of patients suffering from symptoms suggestive of Hantavirus pulmonary syndrome allowed us to identify a native case in French Guiana. Partial molecular characterization of the implicated hantavirus revealed its close relationship with the Bolivian Rio Mamore virus. We tentatively named it Maripa virus.

  16. Drugs induced pulmonary arterial hypertension.

    Science.gov (United States)

    Seferian, Andrei; Chaumais, Marie-Camille; Savale, Laurent; Günther, Sven; Tubert-Bitter, Pascale; Humbert, Marc; Montani, David

    2013-09-01

    Pulmonary arterial hypertension (PAH) is a rare disorder characterized by progressive obliteration of the pulmonary microvasculature, resulting in elevated pulmonary vascular resistance and premature death. According to the current classification, PAH can be associated with exposure to certain drugs or toxins, particularly appetite suppressant drugs, such as aminorex, fenfluramine derivatives and benfluorex. These drugs have been confirmed to be risk factors for PAH and were withdrawn from the market. The supposed mechanism is an increase in serotonin levels, which was demonstrated to act as a growth factor for the pulmonary arterial smooth muscle cells. Amphetamines, phentermine and mazindol were less frequently used but are also considered as possible risk factors for PAH. Dasatinib, a dual Src/Abl kinase inhibitor, used in the treatment of chronic myelogenous leukaemia was associated with cases of severe PAH, in part reversible after its withdrawal. Recently several studies raised the potential endothelial dysfunction that could be induced by interferon, and few cases of PAH have been reported with interferon therapy. Other possible risk factors for PAH include: nasal decongestants, like phenylpropanolamine, dietary supplement - L-Tryptophan, selective serotonin reuptake inhibitors, pergolide and other drugs that could act on 5HT2B receptors. Interestingly, PAH remains a rare complication of these drugs, suggesting possible individual susceptibility and further studies are needed to identify patients at risk of drugs induced PAH. PMID:23972547

  17. The Pulmonary Circulation and Exercise Responses in the Elderly

    OpenAIRE

    Taylor, Bryan J.; Johnson, Bruce D.

    2010-01-01

    Aging is associated with a progressive deterioration in the structure and function of the pulmonary circulation. Remodeling of the pulmonary vasculature occurs from maturity to senescence that is characterized by an increase in pulmonary vascular stiffness, pulmonary vascular pressures, and pulmonary vascular resistance along with increased heterogeneity of alveolar ventilation and pulmonary perfusion and decreased pulmonary capillary blood volume and membrane diffusing capacity that is consi...

  18. Bifurcating stents in the pulmonary arteries: A novel technique to relieve bilateral branch pulmonary artery obstruction.

    Science.gov (United States)

    Narayan, Hari K; Glatz, Andrew C; Rome, Jonathan J

    2015-10-01

    Balloon angioplasty and stent placement in close proximity to the bifurcation of the branch pulmonary arteries can be challenging. Multiple approaches have been previously described, though none of these approaches both treats bilateral proximal branch pulmonary artery stenosis and provides an anchor for a transcatheter pulmonary valve replacement. We report a novel approach that involves serial stent placement and balloon dilation through the struts of the stent in each pulmonary artery, along with balloon expansion of the proximal portion of the stents to the diameter of the main pulmonary artery. In the two cases we describe, this strategy resulted in significant relief of branch pulmonary artery obstruction without compromising the anatomy of the main pulmonary artery segment. This technique can be an effective way to alleviate stenoses of the bilateral proximal branch pulmonary arteries and provides a landing zone for a future transcatheter pulmonary valve. PMID:26256829

  19. Inhaled Therapies for Pulmonary Hypertension.

    Science.gov (United States)

    Hill, Nicholas S; Preston, Ioana R; Roberts, Kari E

    2015-06-01

    The inhaled route has a number of attractive features for treatment of pulmonary hypertension, including delivery of drug directly to the target organ, thus enhancing pulmonary specificity and reducing systemic adverse effects. It can also improve ventilation/perfusion matching by dilating vessels supplying ventilated regions, thus improving gas exchange. Furthermore, it can achieve higher local drug concentrations at a lower overall dose, potentially reducing drug cost. Accordingly, a number of inhaled agents have been developed to treat pulmonary hypertension. Most in current use are prostacyclins, including epoprostenol, which has been cleared for intravenous applications but is used off-label in acute care settings as a continuously nebulized medication. Aerosolized iloprost and treprostinil are both prostacyclins that have been cleared by the FDA to treat pulmonary arterial hypertension (PAH). Both require frequent administration (6 and 4 times daily, respectively), and both have a tendency to cause airway symptoms, including cough and wheeze, which can lead to intolerance. These agents cannot be used to substitute for the infused routes of prostacyclin because they do not permit delivery of medication at high doses. Inhaled nitric oxide (INO) is cleared for the treatment of primary pulmonary hypertension in newborns. It is also used off-label to test acute vasoreactivity in PAH during right-heart catheterization and to treat acute right-heart failure in hospitalized patients. In addition, some studies on long-term application of INO either have been recently completed with results pending or are under consideration. In the future, because of its inherent advantages in targeting the lung, the inhaled route is likely to be tested using a variety of small molecules that show promise as PAH therapies. PMID:26070575

  20. A rare case of human pulmonary dirofilariasis with a growing pulmonary nodule after migrating infiltration shadows, mimicking primary lung carcinoma

    OpenAIRE

    Akira Haro; Sadafumi Tamiya; Akira Nagashima

    2016-01-01

    Introduction: Pulmonary dirofilariasis is a rare pulmonary parasitic infection by the nematode Dirofilaria immitis. It is characterized by an asymptomatic pulmonary nodule usually seen on chest X-ray. The differential diagnosis of pulmonary dirofilariasis includes other pulmonary diseases, primary lung carcinoma and metastatic lung tumor. Case presentation: Pulmonary dirofilariasis was diagnosed in a woman who presented with interstitial pneumonia. Growth of the pulmonary nodule was detect...

  1. Diagnosis and quantitative estimation of pulmonary congestion or edema by pulmonary CT numbers

    International Nuclear Information System (INIS)

    Pulmonary computed tomography (CT) was performed in 25 patients with left heart failure and 10 healthy persons to diagnose pulmonary congestion or edema associated with left heart failure. In an analysis of histogram for pulmonary CT numbers obtained from CT scans, CT numbers indicating pulmonary edema were defined as -650 to -750 H.U. This allowed pulmonary edema to be quantitatively estimated early when abnormal findings were not available on chest X-ray film or pulmonary circulation studies. Histograms for CT numbers could be displayed by colors on CT scans. (Namekawa, K.)

  2. Pulmonary Venous Obstruction in Cancer Patients

    Directory of Open Access Journals (Sweden)

    Chuang-Chi Liaw

    2015-01-01

    Full Text Available Background. We study the clinical significance and management of pulmonary venous obstruction in cancer patients. Methods. We conducted a prospective cohort study to characterize the syndrome that we term “pulmonary vein obstruction syndrome” (PVOS between January 2005 and March 2014. The criteria for inclusion were (1 episodes of shortness of breath; (2 chest X-ray showing abnormal pulmonary hilum shadow with or without presence of pulmonary edema and/or pleural effusion; (3 CT scan demonstrating pulmonary vein thrombosis/tumor with or without tumor around the vein. Results. Two hundred and twenty-two patients developed PVOS. Shortness of breath was the main symptom, which was aggravated by chemotherapy in 28 (13%, and medical/surgical procedures in 21 (9% and showed diurnal change in intensity in 32 (14%. Chest X-rays all revealed abnormal pulmonary hilum shadows and presence of pulmonary edema in 194 (87% and pleural effusion in 192 (86%. CT scans all showed pulmonary vein thrombosis/tumor (100% and surrounding the pulmonary veins by tumor lesions in 140 patients (63%. PVOS was treated with low molecular weight heparin in combination with dexamethasone, and 66% of patients got clinical/image improvement. Conclusion. Physicians should be alert to PVOS when shortness of breath occurs and chest X-ray reveals abnormal pulmonary hilum shadows.

  3. Discrimination between invasive pulmonary aspergillosis and pulmonary lymphoma using CT

    Energy Technology Data Exchange (ETDEWEB)

    Kawel, Nadine, E-mail: nadine.kawel@gmx.de [Department of Radiology, Kantonsspital Graubuenden, Loestr. 170, 7000 Chur (Switzerland); Department of Radiology, University Hospital Basel, Petersgraben 4, 4031 Basel (Switzerland); Schorer, Georg M., E-mail: gmschorer@hotmail.com [Institute of Diagnostic Radiology, University Hospital of Zurich, Raemistr. 100, 8091 Zurich (Switzerland); Stiftung Zuercher Blutspendedienst SRK, Ruetistrasse 19, 8952 Schlieren (Switzerland); Desbiolles, Lotus, E-mail: lotus.desbiolles@usz.ch [Institute of Diagnostic Radiology, University Hospital of Zurich, Raemistr. 100, 8091 Zurich (Switzerland); Seifert, Burkhardt, E-mail: seifert@ifspm.uzh.ch [Biostatistics Unit ISPM, University of Zurich, Hirschengraben 84, 8001 Zurich (Switzerland); Marincek, Borut, E-mail: borut.marincek@usz.ch [Institute of Diagnostic Radiology, University Hospital of Zurich, Raemistr. 100, 8091 Zurich (Switzerland); Boehm, Thomas, E-mail: thomas_boehm@gmx.net [Department of Radiology, Kantonsspital Graubuenden, Loestr. 170, 7000 Chur (Switzerland); Institute of Diagnostic Radiology, University Hospital of Zurich, Raemistr. 100, 8091 Zurich (Switzerland)

    2011-03-15

    Objective: The purpose was to assess the characteristic CT features of invasive pulmonary aspergillosis (IPA) and pulmonary lymphoma (PL) and to analyze the potential to distinguish the two entities using CT. Methods: The CT images of 70 patients with either proven IPA (n = 35) or PL (n = 35) were evaluated retrospectively and independently by two radiologists (reader 1 [R1] and reader 2 [R2]), analyzing images for presence, number and characteristics of pulmonary nodules and masses, ground-glass opacities, consolidations and other interstitial changes. Results: Interreader agreement was moderate (4/33 CT features), good (9/33) or excellent (20/33). Pulmonary nodules (P = 0.045 [R1], P = 0.001 [R2]), nodules with spiculated outer contours (P < 0.001 [R1], P = 0.001 [R2]), nodules with a halo sign (P < 0.001 [R1+R2]), nodules with homogeneous (P = 0.030 [R1], P = 0.006 [R2]) and inhomogeneous (P = 0.001 [R1], P < 0.001 [R2]) attenuation patterns, nodules with cavitation (P = 0.006 [R1], P = 0.003 [R2]) and wedge-shaped, pleural-based consolidations (P < 0.001 [R1 + R2]) occurred significantly more often in patients with IPA, while masses without a halo sign (P = 0.03 [R1], P = 0.01 [R2]), lobar consolidations with bronchogram (P = 0.02 [R1 + R2]) and consolidations with homogeneous attenuation patterns (P < 0.001 [R1 + R2]) were found significantly more frequent in PL-patients. Conclusions: Those CT features can therefore be considered suggestive for either IPA or PL. However, in most cases the diagnosis cannot be made based on CT findings solely because no single feature gained a high sensitivity and specificity concomitantly. Furthermore, the logistic regression did not show a combination that was significantly better than the best univariate predictor.

  4. Bilateral multiple pulmonary tuberculous nodules mimicking metastatic disease

    International Nuclear Information System (INIS)

    We present CT findings of a young woman who has bilateral pulmonary nodules mimicking metastases. Clinical presentation with active multiple pulmonary macronodules without cavitation responsive to treatment is an atypical manifestation of pulmonary tuberculosis. We reviewed the causes of multiple pulmonary nodules, role of radiological findings in differential diagnosis and parenchymal manifestations of pulmonary tuberculosis in this report

  5. Diagnosing chronic thromboembolic pulmonary hypertension: current perspectives

    Directory of Open Access Journals (Sweden)

    Hadinnapola C

    2014-09-01

    Full Text Available Charaka Hadinnapola, Deepa Gopalan, David P Jenkins Papworth Hospital National Health Service Foundation Trust, Papworth Everard, Cambridge, United Kingdom Abstract: Chronic thromboembolic pulmonary hypertension is a rare and relatively poorly understood disease. It remains underdiagnosed and is often not recognized in primary and secondary care, as its symptoms are nonspecific and there are few clinical signs until late in the disease process. However, pulmonary endarterectomy (PEA offers a potential cure for patients with this type of pulmonary hypertension; therefore, it is important that they are identified and diagnosed in a timely manner. PEA is associated with a 2.2%–5% risk of significant morbidity and mortality, even in experienced PEA centers. Therefore, once chronic thromboembolic pulmonary hypertension is diagnosed, further assessment of operability and patient selection is crucial. Assessment of operability involves determining the distribution and burden of chronic thromboembolic disease, assessing pulmonary hemodynamics, and assessing the functional impairment of the patient. Ventilation perfusion scintigraphy is of value in screening for the presence of chronic thromboembolic disease. However, computer tomography pulmonary angiography and magnetic resonance pulmonary angiography are now increasingly used to image the vascular occlusions directly. This allows assessment of the surgically accessible disease burden. Some centers still advocate conventional selective pulmonary angiography for the latter. Right-heart catheterization remains the gold standard for assessing pulmonary hemodynamics. Higher pulmonary vascular resistances are associated with poorer outcomes as well as increased risks at the time of surgery. This is in part because of the presence of more distal chronic thromboembolic material and distal pulmonary artery remodeling. However, in experienced centers, these patients are being operated on safely and with good

  6. Interruption of pulmonary arterial flow with inadequate ventilation leads to pulmonary infection.

    Science.gov (United States)

    Urano; Shibayama, Y; Fukunshi, K; Nariyama, K; Ohsawa, N

    1996-03-01

    We examined the effect of interruption of pulmonary arterial flow and inadequate ventilation on the development of pulmonary infarction in rats. Pulmonary arterial flow was blocked by the injection of agar into the inferior vena cava and inadequate ventilation was produced by obstructing the left main bronchus with a polypropylene tip. Histological and angiographic examination of the lung demonstrated that: pulmonary artery embolism alone does not induce pulmonary infarction; obstruction of a bronchus does not induce significant changes, but that pulmonary infarction develops when pulmonary artery embolism and obstruction of a bronchus occur simultaneously. It has been thought that pulmonary infarction is caused by acute obstruction of a pulmonary artery, however, the alveolar walls are supplied with oxygen by both the pulmonary circulation and by ventilation. Interruption of pulmonary arterial flow alone is probably not sufficient to induce pulmonary infarction, which is probably caused by deficiency of oxygen supply to the alveolar walls by a synergy between interruption of pulmonary arterial flow and inadequate ventilation. PMID:8605572

  7. Pulmonary infections in immunocompromised patients

    International Nuclear Information System (INIS)

    Full text: Introduction: The lung is one of the most commonly affected organs in immunocompromised patients. Primary complication is pulmonary infection which is associated with high morbidity and mortality. Although radiography and CT, as main diagnostic tools are reliable and credible methods, often there is difficulty with the correct diagnose. The reasons for this are that immunocompromised patients are potentially susceptible to infection by various microorganisms and that the radiographic findings are rarely specific for detecting a particular pathogen. What you will learn : Our objective is to present general nosological classification of pulmonary infections in immunocompromised patients, and to evaluate and analyze new imaging methods and discuss their correlation with the clinical setting, which aims to facilitate the diagnosis and to take a decision for the treatment. The experience indicates that a clinical environment conducive the immunocompromised patients to infection with certain pathogens, thereby changing the frequency of their occurrence. The most commonly cited fungal infections, cytomegalovirus infections, Pneumocystis carinii pneumonia and Pulmonary tuberculosis (PTB) of which convincing is the Imaging diagnosis primarily in fungal infections, and Pneumocystis carinii pneumonia and less accurate - in bacterial and viral infections. Discussion: The term 'immunocompromised' describes a subject with an increased risk for life-threatening infection as a result of congenital or acquired abnormalities of the immune system. Over the past few decades, the number of immunocompromised patients has grown considerably, reflecting the increased use of immunosuppressive drugs, and the syndrome of acquired immunodeficiency. Given the high incidence of pulmonary infections in immunocompromised patients (lung is one of the most commonly affected organs, such as lung infection is about 75% of pulmonary complications), rapid and accurate diagnosis is important

  8. Quantitative Computed Tomography of Pulmonary Emphysema and Ventricular Function in Chronic Obstructive Pulmonary Disease Patients with Pulmonary Hypertension

    OpenAIRE

    Huang, Yu-Sen; Hsu, Hsao-Hsun; Chen, Jo-Yu; Tai, Mei-Hwa; Jaw, Fu-Shan; Chang, Yeun-Chung

    2014-01-01

    Objective This study strived to evaluate the relationship between degree of pulmonary emphysema and cardiac ventricular function in chronic obstructive pulmonary disease (COPD) patients with pulmonary hypertension (PH) using electrocardiographic-gated multidetector computed tomography (CT). Materials and Methods Lung transplantation candidates with the diagnosis of COPD and PH were chosen for the study population, and a total of 15 patients were included. The extent of emphysema is defined as...

  9. Naloxone-induced pulmonary edema.

    Science.gov (United States)

    Schwartz, J A; Koenigsberg, M D

    1987-11-01

    We present the case of a 68-year-old woman with acute pulmonary edema secondary to the administration of naloxone to reverse an inadvertent narcotic overdose. The patient presented following a 12-hour history of increasingly bizarre behavior and confusion. A total IV dose of 1.6 mg naloxone was administered in an attempt to reverse the suspected overconsumption of a codeine-containing cough suppressant. She immediately became agitated, tachycardic, and diaphoretic; a clinical diagnosis of acute pulmonary edema was made. Following treatment with furosemide, nitroglycerin, and morphine sulfate, the patient recovered completely without further incident. Although naloxone is thought to be a safe drug with few complications, it should not be used indiscriminantly, and the smallest doses necessary to elicit the desired response should be used. PMID:3662194

  10. Uncommon presentation of pulmonary aspergilloma

    Directory of Open Access Journals (Sweden)

    Baradkar V

    2009-01-01

    Full Text Available Cases of pulmonary aspergilloma without any predisposing factors are rarely reported. Clinical presentation varies from case to case. Here, we report a case of pulmonary aspergilloma in a 60-year-old male patient who was admitted to the Intensive Respiratory Care Unit with spontaneous pneumothorax. The patient had a history of dyspnea on exertion since 9 months and mild haemoptysis since the last 6 months. A computerised tomographic scan of the lungs showed a lesion in the left main bronchus along with obstructive emphysema of the right lung, moderate pneumothorax and mediastinal emphysema. Bronchoscopy was performed and the biopsy samples were processed for histopathological examination and culture on Sabouraud′s dextrose agar, which yielded growth of Aspergillus flavus. Repeat sputum samples also yielded the growth of A. flavus . The patient responded to intravenous liposomaamphotericin B and intercostal drainage.

  11. Pulmonary changes in cystic fibrosis

    International Nuclear Information System (INIS)

    Pulmonary abnormalities in cystic fibrosis result from the obstruction of small bronchi by highly viscous mucus. Chronic obstructive lung disease and recurrent pulmonary infections result in a typical radiographic pattern later in the disease. Most patients can now be expected to survive into adulthood. The radiologist must make a careful comparison of serial films in order to detect complications early. By far the most important imaging modality is the conventional chest radiograph. CT is more sensitive for detection of structural abnormalities of the lung. Bronchography is a dangerous procedure and can lead to rapid deterioration of lung function. Lung scanning is a very sensitive method for demonstrating regional disturbances of ventilation and may reveal abnormalities earlier than conventional radiographs. In severe hemoptysis, selective bronchial arteriography with embolization of the bleeding vessel can be a life-saving procedure. (orig.)

  12. Pulmonary retention of coal dusts

    Energy Technology Data Exchange (ETDEWEB)

    Morrow, P.E.; Gibb, F.R.; Beiter, H.; Amato, F.; Yuile, C.; Kilpper, R.W.

    1980-01-01

    The principal objectives of this study were: to determine, quantitatively, coal dust retention times in the dog lung; to test the appropriateness of a pulmonary retention model which incorporates first order rate coefficients obtained from in vitro and in vivo experiments on neutron-activated coal; to acquire a temporal description of the pulmonary disposition of the retained coal dust, and to compare the behavior of two different Pennsylvania coals in the foregoing regards. The principal findings include: retention half-times for both coals of approximately 2 years following single, hour-long exposures; a vivid association of the retained coal dust with the pulmonic lymphatics; and a general validation of the retention model.

  13. Cytologic diagnosis of pulmonary lesions.

    Science.gov (United States)

    Rossi, Esther Diana; Mulè, Antonino; Maggiore, Claudia; Miraglia, Antonella; Lauriola, Libero; Vecchio, Fabio Maria; Fadda, Guido

    2004-01-01

    The major types of cytologic preparations used in most laboratories to detect the lesions of the lower respiratory tract (LRT) are examined. These methods include sputum, bronchial washing, bronchial brushing, bronchoalveolar lavage (BAL) and fine-needle aspiration biopsy (FNAB). Sputum represents the simplest and most cost-effective sampling method even though fiberoptic bronchoscopy and radiologic guided FNAB are superseding it as the first diagnostic choice in most cases. There are advantages and disadvantages associated with each technique:bronchial brushing and FNABs tend to preserve both the cellular details and their architectural arrangement whereas sputum and bronchial washing often cause a variable degree of cellular degeneration and fragmentation. As a result, most pulmonary lesions may be detected and correctly diagnosed if multiple techniques are used to acquire diagnostic material. CT-guided FNAB represents the most effective method to achieve a correct diagnosis in pulmonary tumors. PMID:15852720

  14. Pulmonary candidiasis presenting as mycetoma

    Directory of Open Access Journals (Sweden)

    Bachh Arshad

    2008-01-01

    Full Text Available Candida is a saprophytic yeast that is frequently recovered from the respiratory tract. Most mycetoma lesions are due to Aspergillus species growing inside an existing cavity. The saprophytic nature of the Candida species in the human respiratory tract obscures diagnosis of Candida pulmonary infections. Only a few cases of mycetoma due to Can-dida have been reported. We report a case of mycetoma caused by Candida albicans in a diabetic immunocompromised tuberculous patient. Diagnosis was confirmed by biopsy and certain points strongly favoured the diagnosis. The patient was immunocompromised due to uncontrolled diabetes mellitus. Candida albicans was grown from bronchial and repeatedly from sputum samples and Candida antigen was positive from blood in high titre. There was a good clinical as well as radiological response to antifungal therapy and Candida antigen became negative. We emphasize that in the immunosuppressed host, a mycetoma-like lesion may be caused by Candida pulmonary infection.

  15. Pulmonary complications in patients with chronic obstructive pulmonary disease following transthoracic esophagectomy

    OpenAIRE

    Jiao, Wen-Jie; Wang, Tian-You; Gong, Min; Pan, Hao; Liu, Yan-Bing; Liu, Zhi-Hua

    2006-01-01

    AIM: To investigate the incidence of various types of postoperative pulmonary complications (POPCs) and to evaluate the significance of perioperative arterial blood gases in patients with esophageal cancer accompanied with chronic obstructive pulmonary disease (COPD) after esophagectomy.

  16. Prevalence, predictors, and survival in pulmonary hypertension related to end-stage chronic obstructive pulmonary disease

    DEFF Research Database (Denmark)

    Andersen, Kasper Hasseriis; Iversen, Martin; Kjaergaard, Jesper;

    2012-01-01

    The prevalence, prognostic importance, and factors that predict the presence and degree of pulmonary hypertension (PH) diagnosed with right heart catheterization (RHC) in patients with end-stage chronic obstructive pulmonary disease (COPD) remain unclear....

  17. 基底动脉发育不良与后循环梗死的相关性%Association between basilar artery hypoplasia and posterior circulation infarction

    Institute of Scientific and Technical Information of China (English)

    张志勇; 刘尊敬; 田朝晖; 唐文雄; 刘玮; 薛爽; 焦劲松

    2014-01-01

    其卒中防治工作。%Objective To explore the relationship between basal artery hypoplasia ( BAH ) and posterior circulation ischemic stroke and its clinical characteristics to improve the understanding of BAH. Methods A total of 328 hospitalized patients from April 2012 to April 2014 were enrolled retrospectively. With normal course and regular shape of basilar artery on brain magnetic resonance angiography ( MRA) , other causes of posterior circulation ischemic stroke were excluded.They were divided into BAH ( n=48 ) and non-BAH (n=280) groups according to the morphology and diameter of basilar artery on head MRA. We compared the general information and intracranial vascular variations between two groups, especially the incidence rate of posterior circulation infarction and mean blood flow velocity ( Vm ) of basal artery by analyzing clinical information and MRI findings.Meantime, their clinical outcomes were observed through follow-ups.And detailed clinical features were discussed for the patients with posterior circulation infarction in the BAH group.Results ( 1 ) The concurrent lesions included vertebral artery intracranial segment hypoplasia (n=24, VAH), fetal type posterior artery (n=18, FTPA), persistent trigeminal artery (n=1) and giant fenestration variation on vertebral artery ( n=1 ) in the BAH group.In comparison, it was more liable to cranial vascular variations in the BAH group ( P BAH group ( P<0.05).(3) these cases with stroke in two groups had no mortality during a follow-up period of 4-28 months.There were 3 cases with recurrent posterior circulation stroke in the non-BAH group.The number of cases with mRS scoring 2 points or less in the BAH group was more than that in the non-BAH group at discharge, 30 or 90 days after discharge ( P<0.05 ) .( 4 ) these cases with posterior circulation stroke in the BAH group often presented as lacunar syndrome (9/17), paramedian infarction in pons (9/17) and bilateral VAH plus unilateral FTPA (8/17).Conclusion As a relatively rare disease, BAH

  18. Ozone and Pulmonary Innate Immunity

    OpenAIRE

    Hollingsworth, John W.; Kleeberger, Steven R.; Foster, W. Michael

    2007-01-01

    Ambient ozone (O3) is a commonly encountered environmental air pollutant with considerable impact on public health. Many other inhaled environmental toxicants can substantially affect pulmonary immune responses. Therefore, it is of considerable interest to better understand the complex interaction between environmental airway irritants and immunologically based human disease. The innate immune system represents the first line of defense against microbial pathogens. Intact innate immunity requ...

  19. Pulmonary surfactant and lung transplantation

    OpenAIRE

    Erasmus, Michiel Elardus

    1997-01-01

    Pulmonary surfactant lowers the surface tension at the air-water interface inside the alveolus. This is achieved by adsorption of surfactant phospholipids at the air-water interface, a process controlled by surfactant-associated proteins, such as SP-A. In this way, surfactant prevents collapse of the alveolus at end expiration, prevents formation of alveolar edema and increases the compliance of the lung. In chapter 1a an overview is given how the normal function of surfactant can be affected...

  20. Pulmonary Scintigraphy for Tumour Diagnosis

    International Nuclear Information System (INIS)

    Scanning of the lungs with albumin aggregates is one of the most useful methods for studying variations in pulmonary circulation and diagnosing embolisms and infarcts. However, its utilization for the diagnosis of tumours is barely in the trial stage. Using scintillation scanning, the authors have centered their attention on the study of patients with primary pulmonary tumours and secondary tumours at-other sites, and have observed that the tracer distribution pattern differs fundamentally according to the type of tumour. With metastatic tumours, the repercussion of the lesions in scintillation scanning is slight and is entirely dependent on the volume of the zone of condensation. The tumorous nodules behave entirely like inactive zones within a mass of active functional parenchyma and their effect on the scanning image depends solely on the size of the tumorous region and the amount of healthy parenchyma intervening between it and the detector. With primary pulmonary tumours and more especially those located in the hilar region, relatively small lesions give rise to defects in uptake in extensive areas of the lung, and these may affect various segments, a lobe or even the entire lung. This decrease in uptake cannot be entirely explained by the image of the tumour or by the associated zones of atelectasia, but must be due to serious alteration in the haemodynamics of the lung affected. Various kinds of phenomena associated with tumorous development may cause vascular changes affecting even the periphery of the lung: changes in breathing conditions accompanied by a reduction of oxygen tension, leading to change in haemodynamic conditions; pulmonary hypertension through compression of the veins in the lungs which are less resistant than the arteries; and direct nervous stimulation due to irritation of the bronchial plexi as a result of the growth of the tumour and, associated reactive. phenomena. (author)