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Respiratory Insufficiency (respiratory + insufficiency)
Kinds of Respiratory Insufficiency Selected AbstractsPEEP Therapy for Patients With Pleurotomy During Coronary Artery Bypass GraftingJOURNAL OF CARDIAC SURGERY, Issue 3 2000Susumu Ishikawa M.D. We studied the efficacy of intraoperative positive end-expiratory airway pressure (PEEP) therapy for the prevention of postoperative pulmonary oxygenation impairment. A total of 66 patients with solitary CABG procedure were included in this study. The pleural cavity was intraoperatively opened in 44 patients and not opened in 22. PEEP therapy was not used in any patient before May 1996 (referred to herein as the former period) and was used more recently in eight patients with pleurotomy (referred to herein as the latter period). PEEP was initiated immediately after pleurotomy during the harvest of the internal mammary artery graft. Without PEEP therapy, values of PaO2, A-aDO2, and respiratory index (RI) were worse in patients with pleurotomy than in those without pleurotomy. Meanwhile, there were no major differences in these values between patients with or without pleurotomy after the induction of PEEP therapy. Respiratory insufficiency (A-aDO2 > 400 mmHg and RI > 1.5) was detected in six patients with pleurotomy in the former period. Three of these six patients required over 1 week of long-term mechanical respiratory support. No respiratory insufficiency occurred in patients of the latter period. In conclusion, PEEP therapy, which is initiated just after pleurotomy, may prevent oxygen impairment and pulmonary atelectasis after extracorporeal circulation (ECC) and is recommended for patients with pleurotomy, especially for patients with preoperative low respiratory function. [source] Prediction of respiratory insufficiency in Guillain-Barré syndromeANNALS OF NEUROLOGY, Issue 6 2010Christa Walgaard MD Objective Respiratory insufficiency is a frequent and serious complication of the Guillain-Barré syndrome (GBS). We aimed to develop a simple but accurate model to predict the chance of respiratory insufficiency in the acute stage of the disease based on clinical characteristics available at hospital admission. Methods Mechanical ventilation (MV) in the first week of admission was used as an indicator of acute stage respiratory insufficiency. Prospectively collected data from a derivation cohort of 397 GBS patients were used to identify predictors of MV. A multivariate logistic regression model was validated in a separate cohort of 191 GBS patients. Model performance criteria comprised discrimination (area under receiver operating curve [AUC]) and calibration (graphically). A scoring system for clinical practice was constructed from the regression coefficients of the model in the combined cohorts. Results In the derivation cohort, 22% needed MV in the first week of admission. Days between onset of weakness and admission, Medical Research Council sum score, and presence of facial and/or bulbar weakness were the main predictors of MV. The prognostic model had a good discriminative ability (AUC, 0.84). In the validation cohort, 14% needed MV in the first week of admission, and both calibration and discriminative ability of the model were good (AUC, 0.82). The scoring system ranged from 0 to 7, with corresponding chances of respiratory insufficiency from 1 to 91%. Interpretation This model accurately predicts development of respiratory insufficiency within 1 week in patients with GBS, using clinical characteristics available at admission. After further validation, the model may assist in clinical decision making, for example, on patient transfer to an intensive care unit. ANN NEUROL 2010;67:781,787 [source] Bilateral periventricular nodular heterotopia and lissencephaly in an infant with unbalanced t(12;17)(q24.31; p13.3) translocationDEVELOPMENTAL MEDICINE & CHILD NEUROLOGY, Issue 6 2008Salvatore Grosso MD PhD Periventricular nodular heterotopia and Miller-Dieker syndrome are two different disorders of brain development. Miller-Dieker syndrome exhibits classical lissencephaly and is related to defects in the lissencephaly gene (LIS1). Periventricular nodular heterotopia is characterized by aggregates of grey matter adjacent to the lateral ventricle and is mainly linked to mutations in the Filamin A (FLNA) gene. We describe a male infant presenting with facial dysmorphisms resembling those of Miller-Dieker syndrome, neuromotor delay, and drug - resistant infantile spasms. Magnetic resonance imaging of the brain showed periventricular nodular heterotopia overlaid by classical lissencephaly with complete agyria. Cytogenetic and molecular investigations detected a maternally inherited unbalanced translocation involving chromosome arms 17p and 12q. This resulted in partial monosomy of 17p13.3,pter and partial trisomy of 12q24.3,qter No mutation was found in the FLNA gene. The patient died at the age of 22 months from respiratory insufficiency during an infection of the lower respiratory tract. Our observation extends the list of the overlying cortical malformations associated with periventricular nodular heterotopia. It remains to be established whether this peculiar neuronal migration disorder represents a phenotype totally linked to 17q13.3 deletion or results from a combination of gene defects at 17q13.3 and 12q24.3. [source] PRECLINICAL STUDY: Mechanisms of respiratory insufficiency induced by methadone overdose in ratsADDICTION BIOLOGY, Issue 1 2010Lucie Chevillard ABSTRACT Methadone may cause respiratory depression. We aimed to understand methadone-related effects on ventilation as well as each opioid-receptor (OR) role. We studied the respiratory effects of intraperitoneal methadone at 1.5, 5, and 15 mg/kg (corresponding to 80% of the lethal dose-50%) in rats using arterial blood gases and plethysmography. OR antagonists, including intravenous 10 mg/kg-naloxonazine at 5 minutes (mu-OR antagonist), subcutaneous 30 mg/kg-naloxonazine at 24 hours (mu1-OR antagonist), 3 mg/kg-naltrindole at 45 minutes (delta-OR antagonist) and 5 mg/kg-Nor-binaltorphimine at 6 hours (kappa-OR antagonist) were pre-administered. Plasma concentrations of methadone enantiomers were measured using high-performance liquid chromatography coupled to mass-spectrometry. Methadone dose-dependent inspiratory time (TI) increase tended to be linear. Respiratory depression was observed only at 15 mg/kg and characterized by an increase in expiratory time (TE) resulting in hypoxemia and respiratory acidosis. Intravenous naloxonazine completely reversed all methadone-related effects on ventilation, while subcutaneous naloxonazine reduced its effects on pH (P < 0.05), PaCO2 (P < 0.01) and TE (P < 0.001) but only partially on TI (P < 0.001). Naltrindole reduced methadone-related effects on TE (P < 0.001). Nor-binaltorphimine increased methadone-related effects on pH and PaO2 (P < 0.05) Respiratory effects as a function of plasma R -methadone concentrations showed a decrease in PaO2 (EC50: 1.14 µg/ml) at lower concentrations than those necessary for PaCO2 increase (EC50: 3.35 µg/ml). Similarly, increased TI (EC50: 0.501 µg/ml) was obtained at lower concentrations than those for TE (EC50: 4.83 µg/ml). Methadone-induced hypoxemia is caused by mu-ORs and modulated by kappa-ORs. Additionally, methadone-induced increase in TE is caused by mu1- and delta-opioid receptors while increase in TI is caused by mu-ORs. [source] Seronegative myasthenia gravis: disease severity and prognosisEUROPEAN JOURNAL OF NEUROLOGY, Issue 6 2005F. Romi Around 10,20% of myasthenia gravis (MG) patients do not have acetylcholine receptor (AChR) antibodies (seronegative), of whom some have antibodies to a membrane-linked muscle specific kinase (MuSK). To examine MG severity and long-term prognosis in seronegative MG compared with seropositive MG, and to look specifically at anti-AChR antibody negative and anti-MuSK antibody negative patients. Seventeen consecutive seronegative non-thymomatous MG patients and 34 age and sex matched contemporary seropositive non-thymomatous MG controls were included in a retrospective follow-up study for a total period of 40 years. Clinical criteria were assessed each year, and muscle antibodies were assayed. There was no difference in MG severity between seronegative and seropositive MG. However, when thymectomized patients were excluded from the study at the year of thymectomy, seropositive MG patients had more severe course than seronegative (P < 0.001). One seropositive patient died from MG related respiratory insufficiency. The need for thymectomy in seronegative MG was lower than in seropositive MG. None of the seronegative patients had MuSK antibodies. This study shows that the presence of AChR antibodies in MG patients correlates with a more severe MG. With proper treatment, especially early thymectomy for seropositive MG, the outcome and long-term prognosis is good in patients with and without AChR antibodies. [source] Chronic inflammatory demyelinating polyneuropathy, phrenic nerve and respiratory symptomsEUROPEAN JOURNAL OF NEUROLOGY, Issue 1 2005J. Costa Respiratory involvement in chronic inflammatory demyelinating polyneuropathy (CIDP) has been very recently described. Phrenic nerve conduction studies have been described as useful to detect respiratory impairment in these patients. This study describes two patients with CIDP, in whom neurophysiological studies of the respiratory muscles were performed. The first patient had severe respiratory insufficiency, and phrenic nerve studies disclosed no motor responses and electromyography (EMG) of the diaphragm confirmed severe loss of motor units, bilaterally. On treatment, we documented clinical and neurophysiological improvement. In the second patient, phrenic nerve studies showed abnormal results; however, EMG of the diaphragm ruled out loss of motor units. The first case represents the risk of phrenic nerve involvement in this disorder, and the potential recovery on treatment. The second case illustrates that the temporal dispersion of the motor responses can be misleading, and EMG of diaphragm should be performed to confirm the loss of motor units. [source] The use of ITS DNA sequence analysis and MALDI-TOF mass spectrometry in diagnosing an infection with Fusarium proliferatumEXPERIMENTAL DERMATOLOGY, Issue 11 2008Florian Seyfarth Abstract:, Although mycoses are among the most common diseases worldwide, infections with Fusarium spp. occur only rarely. Mostly patients suffering from underlying immune deficiency are infected with this mould, resulting in a considerably decreasing prognosis. In immunocompromised patients, cutaneous manifestations are more often associated with Fusarium sp. than with Candida sp. or Aspergillus sp. We describe one patient with acute lymphoblastic leukaemia, who was first treated with chemotherapy after GMALL protocol 07/03. After relapse, the patient was successfully transplanted in second remission with a human leukocyte antigen (HLA)-matched unrelated peripheral blood stem cell graft. Ten months later, the patient died from respiratory insufficiency and recurrence of leukaemia. Previously, Aspergillus antigen was detected in blood. In the latter course, disseminated papules appeared. One of these was examined histologically and mycologically. Conventional cultural diagnostics led to the diagnosis of a fusariosis, further supported by internal transcribed spacer (ITS) sequencing and matrix assisted laser desorption/ionisation,time-of-flight mass spectrometry (MALDI-TOF) mass spectrometry, both determining the isolated strain as Fusarium proliferatum, which is a very infrequent pathogen within this genus. Our investigations underline the potential of MALDI-TOF MS based identification of Fusarium species as an innovative, time and cost efficient alternative to ITS sequencing. [source] Pulmonary embolism in a patient with severe congenital deficiency for factor V during treatment with fresh frozen plasmaHAEMOPHILIA, Issue 3 2005A. García-Noblejas Summary., Thrombosis is a rare complication in patients with congenital clotting factor deficiencies. In most cases, it is related to inherited procoagulant factors, use of central venous catheters or administration of coagulation factor concentrates. There are only a few case reports about thrombotic events during treatment with fresh frozen plasma (FFP). We report the case of a patient with homozygous inherited factor V deficiency, who developed a pulmonary embolism at a time of treatment with methylene blue treated FFP (MBFFP). The patient had only two other factors predisposing to thrombosis and both were acquired: obesity and bed rest. He started anticoagulant treatment with low molecular weight heparin (LMWH) while the deficient factors were replaced with MBFFP. After 8 days of treatment the patient developed a severe respiratory insufficiency. Pulmonary haemorrhage was considered among the differential diagnosis and LMWH was stopped. An inferior vena cava filter was placed without any further thrombotic complications. To our knowledge, there are no reports about patients with clotting factor deficiencies who developed a thrombotic event during treatment with MBFFP. [source] Severe Legionella pneumophila pneumonia following infliximab therapy in a patient with Crohn's diseaseINFLAMMATORY BOWEL DISEASES, Issue 8 2009Florian Beigel MD Abstract Background: Immunosuppressive therapy with anti-TNF-, antibodies is effective in patients with inflammatory bowel disease (IBD). However, there is an increased risk for infections associated with this therapy. Methods: Here, we report the case of a 58-year-old patient with Crohn's disease (CD) treated with steroids and azathioprine who developed severe Legionella pneumophila pneumonia after 3 infusions of infliximab. The patient presented at our IBD department with severe active CD complicated by inflammatory small bowel stenoses and entero-enteral fistulas despite long-term high-dose steroid therapy. To achieve steroid tapering and control of disease activity, immunosuppressive therapy with azathioprine was initiated. Due to persistent symptoms, infusion therapy with the anti-TNF-, antibody infliximab was started, subsequently leading to significant clinical improvement. However, after the third infliximab infusion the patient was hospitalized with fever, severe fatigue, and syncope. Results: Laboratory findings and chest X-ray revealed left-sided pneumonia; cultural analysis showed L. pneumophila serogroup 1 leading to respiratory insufficiency, which required mechanical ventilation for 2 weeks in the intensive care unit. After discontinuation of all immunosuppressive agents and immediate antibiotic therapy the patient recovered completely. Conclusions: To our knowledge, this is the third case of L. pneumophila pneumonia in an IBD patient treated with infliximab. Similar to other published cases, concomitant treatment of immunosuppressives and anti-TNF agents is a major risk factor for the development of L. pneumophila infection, which should be ruled out in all cases of pneumonia in patients with such a therapeutic regimen. Appropriate prevention strategies should be provided in these patients. (Inflamm Bowel Dis 2009) [source] PEEP Therapy for Patients With Pleurotomy During Coronary Artery Bypass GraftingJOURNAL OF CARDIAC SURGERY, Issue 3 2000Susumu Ishikawa M.D. We studied the efficacy of intraoperative positive end-expiratory airway pressure (PEEP) therapy for the prevention of postoperative pulmonary oxygenation impairment. A total of 66 patients with solitary CABG procedure were included in this study. The pleural cavity was intraoperatively opened in 44 patients and not opened in 22. PEEP therapy was not used in any patient before May 1996 (referred to herein as the former period) and was used more recently in eight patients with pleurotomy (referred to herein as the latter period). PEEP was initiated immediately after pleurotomy during the harvest of the internal mammary artery graft. Without PEEP therapy, values of PaO2, A-aDO2, and respiratory index (RI) were worse in patients with pleurotomy than in those without pleurotomy. Meanwhile, there were no major differences in these values between patients with or without pleurotomy after the induction of PEEP therapy. Respiratory insufficiency (A-aDO2 > 400 mmHg and RI > 1.5) was detected in six patients with pleurotomy in the former period. Three of these six patients required over 1 week of long-term mechanical respiratory support. No respiratory insufficiency occurred in patients of the latter period. In conclusion, PEEP therapy, which is initiated just after pleurotomy, may prevent oxygen impairment and pulmonary atelectasis after extracorporeal circulation (ECC) and is recommended for patients with pleurotomy, especially for patients with preoperative low respiratory function. [source] Group A streptococcal toxic shock syndrome with extremely aggressive course in the third trimesterJOURNAL OF OBSTETRICS AND GYNAECOLOGY RESEARCH (ELECTRONIC), Issue 4 2010Takashi Sugiyama Abstract Group-A-streptococcus-(GAS)-induced toxic shock syndrome (TSS) is uncommon, but carries a high risk of maternal mortality during pregnancy. The onset of gravidic GAS-TSS has been reported mostly during the puerperium. A 16-year-old woman, who was at 37 weeks of gestation, and without obstetrical care during the last 30 weeks, was referred to our hospital. She complained of fever for one day with headache and abdominal pain after the fever developed. On admission, her consciousness was drowsy, intrauterine fetal death was recognized, and she rapidly developed shock status with coma and hypotension, hemolysis, disseminated intravascular coagulation (DIC), and multi-organ failure. Although we had not obtained the results of a bacterial culture, we suspected sepsis with DIC with homolysis and multi-organ failure resulting from an infection. The patient was treated with antibiotics and intubation because of respiratory insufficiency. Twelve hours after admission to the intensive care unit in our hospital, she died. Cultures from blood, subcutaneous tissue, vaginal discharge, and pharynx all revealed GAS bacteria, and therefore she was diagnosed as having GAS-TSS. GAS-TSS in pregnancy is rare. However, once the infection occurs in a pregnant woman, it rapidly develops into sepsis with multi-organ failure. Therefore, early recognition and intensive treatment for GAS during pregnancy is recommended in women with high fever, muscular pain, hemolysis and DIC during pregnancy. [source] Physician attitudes towards ventilatory support for spinal muscular atrophy type 1 in AustralasiaJOURNAL OF PAEDIATRICS AND CHILD HEALTH, Issue 12 2007Nimeshan Geevasinga Background: Without ventilatory support, premature death from respiratory insufficiency is virtually universal in infants with spinal muscular atrophy type 1 (SMA1). With mechanical ventilation, however, long-term survival has been reported from numerous international centres. We aimed to characterize physician attitudes to the various forms of ventilatory support for children with SMA1. Methods: We surveyed neurologists, respiratory physicians, clinical geneticists and intensivists from all major paediatric hospitals in Australia and New Zealand regarding their views on ventilatory management of SMA1. Results: Ninety-two of the 157 (59%) physicians surveyed replied. Respondents included 16 clinical geneticists, 19 intensive care physicians, 28 neurologists and 29 respiratory physicians. Almost half (47%) opposed invasive ventilation of children with SMA1 and respiratory failure precipitated by intercurrent illness. The majority (76%) opposed invasive ventilatory support for chronic respiratory failure in SMA1. In contrast, non-invasive ventilation was felt by 85% to be appropriate for acute respiratory deteriorations, with 49% supporting long-term non-invasive ventilatory support. Most physicians felt that decisions regarding ventilation should be made jointly by parents and doctors, and that hospital Clinical Ethics Committees should be involved in the event of discordant opinion regarding further management. A majority felt that a defined hospital policy would be valuable in guiding management of SMA1. Conclusions: Respiratory support in SMA1 is an important issue with significant ethical, financial and resource management implications. Most physicians in Australian and New Zealand oppose invasive ventilatory support for chronic respiratory failure in SMA1. Non-invasive ventilation is an accepted intervention for acute respiratory decompensation and may have a role in the long-term management of SMA1. Clinical Ethics Committees and institutional policies have a place in guiding physicians and parents in the management of children with SMA1. [source] Pneumonectomy: four case studies and a comparative reviewJOURNAL OF SMALL ANIMAL PRACTICE, Issue 9 2004J. M. Liptak Pneumonectomy is the resection of all lung lobes in either the left right lung field. The surgical technique and postoperative results pneumonectomy for clinical disease have not been reported in companion animals. Pneumonectomy was performed in three dogs and one cat to treat pulmonary or pleural disease, and the postoperative outcome compared with the complications and results reported in the human literature. One dog died immediately postoperatively due to suspected respiratory insufficiency and the remaining three animals survived the perioperative period. Postoperative complications were reported in two animals. Cardiac complications occurred in the cat, with perioperative arrhythmias and progressive congestive heart failure. Gastrointestinal complications were diagnosed in one dog, with mediastinal shift oesophageal dysfunction. Left- and right-sided pneumonectomy is feasible in companion animals, and the postoperative outcome and complications encountered in this series were similar to those reported in humans. [source] Abstracts of the 8th Meeting of the Italian Peripheral Nerve Study Group: 83JOURNAL OF THE PERIPHERAL NERVOUS SYSTEM, Issue 1 2003G Lauria We describe a 64-year-old patient complaining of progressive gait disturbance, referred to the compressive effect of multiple discal protrusions, for about 3 years. At the age of 62 he presented epileptic seizures during a febrile episode. Cerebral MRI showed bilateral frontobasal T2-weighted hyperintensity involving cortex and white matter. Partial seizures reappeared one year later and a MRI revealed a mild frontobasal atrophy. At the moment of our observation, neurological examination showed waddling gait with bilateral foot drop, muscular atrophy and weakness limited to the gluteal muscles and widespread deep tendon areflexia. Nerve conduction studies showed absent F-waves at both upper and lower limb examination, with normal distal sensorimotor nerve conduction. Needle EMG examination detected mild chronic partial denervation, predominant in proximal muscles of lower limbs. Somatosensory evoked potentials recorded from upper extremities showed bilateral increase in early latencies (N9). Overall, neurophysiological findings indicated a widespread radiculopathy. Serum exams revealed positive anti-nucleus (1:640, granular). CSF examination detected increased IgG level and several oligoclonal bands. Chest radiogram was normal. Soon after our first observation, the patient showed symptoms of respiratory insufficiency. A CT scans revealed a thoracic mass compatible with microcytoma, whereas anti-Hu (3 +) antibodies and increased NSE (neuronal specific enolase) titer were found. In the following two weeks, the patient showed a progressive worsening of the general clinical conditions and died. We interpreted this complex neurological picture, which included an atypical limbic encephalitis and a slowly progressive polyradiculopathy, as a paraneoplastic syndrome. The almost complete resolution of the encephalitic process and the subtle chronic involvement of the peripheral nervous system, characterized by a limited, though widespread, radicular impairment, are rather peculiar features. [source] Electrophysiologist-Implanted Transvenous Cardioverter Defibrillators Using Local Versus General AnesthesiaPACING AND CLINICAL ELECTROPHYSIOLOGY, Issue 1 2000ANTONIS S. MANOLIS With the advent of smaller biphasic transvenous implantable Cardioverter defibrillators (ICDs) and the experience gained over the years, it is now feasible for electrophysioiogists to implant them safely in the abdominal or pectoral area without surgical assistance. Throughout the years, general anesthesia has been used as the standard technique of anesthesia for these procedures. However, use of local anesthesia combined with deep sedation only for defibrillation threshold (DFT) testing might further facilitate and simplify these procedures. The purpose of this study was to test the feasibility of using local anesthesia and compare it with the standard technique of general anesthesia, during implantation of transvenous ICDs performed by an electrophysiologist in the electrophysiology laboratory. For over 4 years in the electrophysiology laboratory, we have implanted transvenous ICDs in 90 consecutive patients (84 men and 6 women, aged 58 ± 15 years). Early on, general anesthesia was used (n = 40, group I), but in recent series (n = 50, group II) local anesthesia was combined with deep sedation for DFT testing. Patients had coronary (n = 58) or valvular (n = 4) disease, cardiomyopathy (n = 25) or no organic disease (n = 3), a mean left ventricular ejection fraction of 35%, and presented with ventricular tachycardia (n = 72) or fibrillation (n = 16), or syncope (n = 2). One-lead ICD systems were used in 74 patients, two-lead systems in 10 patients, andan AVICD in 6 patients. ICDs were implanted in abdominal (n = 17, all in group I) or more recently in pectoral (n = 73) pockets. The DFT averaged 9.7 ± 3.6 J and 10.2 ± 3.6 J in the two groups, respectively (P = NS) and there were no differences in pace-sense thresholds. The total procedural duration was shorter (2.1 ± 0.5 hours) in group II (all pectoral implants) compared with 23 pectoral implants of group I (2.9 ± 0.5 hours) (P < 0.0001). Biphasic devices were used in all patients and active shell devices in 67 patients; no patient needed a subcutaneous patch. There were six complications (7%), four in group I and two in group II: one pulmonary edema and one respiratory insufficiency that delayed extubation for 3 hours in a patient with prior lung resection, both probably related to general anesthesia, one lead insulation break that required reoperation on day 3, two pocket hematomas, and one pneumothorax. There was one postoperative arrhythmic death at 48 hours in group I. No infections occurred. Patients were discharged at a mean time of 3 days. All devices functioned well at predischarge testing. Thus, it is feasible to use local anesthesia for current ICD implants to expedite the procedure and avoid general anesthesia related cost and possible complications. [source] Acute pulmonary toxicity following intralesional administration of bleomycin for a lymphovenous malformationPEDIATRIC PULMONOLOGY, Issue 2 2010Khalid Atwa MD Abstract Objective To describe the clinical course and treatment of an infant with acute pulmonary toxicity following intralesional administration of bleomycin for a lymphovenous malformation. Design Case report. Setting A tertiary care University-affiliated hospital. Patient, Intervention, and Results An 8-month-old girl developed acute respiratory distress with profound hypoxemia complicated by pneumothorax and pneumomediastinum 1 day following intralesional administration of bleomycin. She was treated with bilateral chest tube insertion, systemic corticosteroids, pentoxifylline, and supportive care. At the most recent follow-up 5 months after the onset of the respiratory event, the patient is active, walks, and talks without any evidence of pulmonary diseases clinically. This is the youngest infant reported, to date, with acute bleomycin pulmonary toxicity following intralesional administration of bleomycin resulting in acute respiratory insufficiency followed by complete recovery. Conclusions This case illustrates the importance of early recognition and aggressive treatment of acute bleomycin toxicity resulting from intralesional administration of this medication for lymphovenous malformations. Pediatr Pulmonol. 2010; 45:192,196. © 2009 Wiley-Liss, Inc. [source] Partial splenectomy for portal hypertension in cystic fibrosis related liver diseasePEDIATRIC PULMONOLOGY, Issue 12 2007Dominique Louis MD Abstract Aims To review the middle- and long-term effects of partial splenectomy (PS) on portal hypertension (PHT) and its complications in patients with cystic fibrosis (CF) related liver disease risky PHT. Method Over a 20 years period, 19 patients aged 7,23 years underwent partial PS for massive splenomegaly, hypersplenism, and / or severe PHT. Results In all but three cases, PHT and hypersplenism have improved for long periods. Noticeable improvement of hepatic tests occurred simultaneously. In all patients PS resolved abdominal discomfort. Fifteen patients are alive and a stabilization of the liver disease occurred with a follow-up of 1,20 years (mean 7.9). One patient died following respiratory insufficiency 10 years after PS although PHT was stable. Manifestations recurred in 2 patients 5 and 6 years after PS. In two patients, the course of the disease evolved to hepatic insufficiency without recurrence of PHT 3 and 8 years after PS. PS did not give the expected results in three cases only, in which PHT was not modified or reoccurred during the following year. No severe complication was observed. Early (three patients) or late (one patient) eventration required surgical procedure. Conclusions Our results show that PS is a reliable and well-tolerated technique. Therefore, it is a therapeutic option for the management of PHT in CF patients with a preserved liver function. It can prevent and significantly delay a liver transplantation and its constraints. Pediatr Pulmonol. 2007; 42:1173,1180. © 2007 Wiley-Liss, Inc. [source] Familial dysautonomia: A diagnostic dilemma.PEDIATRIC PULMONOLOGY, Issue 6 2001Chronic lung disease with signs of an autoimmune disease Abstract We present an 11-year-old girl with sensory and autonomic neurological dysfunction, and respiratory insufficiency caused by recurrent aspiration. The diagnosis of familial dysautonomia (FD) was confirmed by a missing axonal flare to histamine, miosis in response to conjunctival methacholine and homozygous polymorphic linked markers DS58(18) and DS159(7) on chromosome 9. Ashkenazi Jewish descent could not be ascertained by history. A variety of positive tests for autoantibodies were initially interpreted as evidence for systemic lupus erythematosus vs. overlap syndrome with pulmonary, cerebral, skin, and ocular involvement. The diagnosis of FD was delayed because of the rarity of this disorder in Germany (second case reported). We discuss possible explanations for the misleading immunological findings, including interference by antibodies binding to milk proteins used as blocking reagents in enzyme-linked immunoassays and circulating immune-complexes due to chronic aspiration pneumonitis. Pediatr Pulmonol. 2001; 31:478,481. © 2001 Wiley-Liss, Inc. [source] Detection of Jarcho,Levin syndrome at 12 weeks' gestation by nuchal translucency screening and three-dimensional ultrasoundPRENATAL DIAGNOSIS, Issue 5 2001Andrew D. Hull Abstract Jarcho,Levin syndrome (JLS) or spondylothoracic dysotosis causes early newborn death from respiratory insufficiency. Prenatal diagnosis is possible using fetal ultrasound, but requires a high level of suspicion and definitive diagnosis may be delayed well into the second trimester. We present a case in which a combination of three-dimensional ultrasound and measurement of nuchal translucency thickness allowed successful detection of recurrent JLS at 12 weeks' gestation. Copyright © 2001 John Wiley & Sons, Ltd. [source] Oral dantrolene and severe respiratory failure in a patient with chronic spinal cord injuryANAESTHESIA, Issue 8 2010M. Javed Summary Oral dantrolene is used widely for the treatment of spasticity in patients with spinal cord injury. A 60-year-old patient in the rehabilitation phase following cervical spine injury presented with generalised weakness and deteriorating respiratory function, requiring intensive care admission, tracheal intubation and ventilation. He had bilateral basal lung collapse and a raised diaphragm and was on high-dose oral dantrolene. The cessation of dantrolene resulted in a dramatic recovery of respiratory function within two days. High-dose oral dantrolene can cause severe respiratory insufficiency and may present difficulties in the differential diagnosis of respiratory failure in patients with high cervical spinal cord injuries. [source] Prediction of respiratory insufficiency in Guillain-Barré syndromeANNALS OF NEUROLOGY, Issue 6 2010Christa Walgaard MD Objective Respiratory insufficiency is a frequent and serious complication of the Guillain-Barré syndrome (GBS). We aimed to develop a simple but accurate model to predict the chance of respiratory insufficiency in the acute stage of the disease based on clinical characteristics available at hospital admission. Methods Mechanical ventilation (MV) in the first week of admission was used as an indicator of acute stage respiratory insufficiency. Prospectively collected data from a derivation cohort of 397 GBS patients were used to identify predictors of MV. A multivariate logistic regression model was validated in a separate cohort of 191 GBS patients. Model performance criteria comprised discrimination (area under receiver operating curve [AUC]) and calibration (graphically). A scoring system for clinical practice was constructed from the regression coefficients of the model in the combined cohorts. Results In the derivation cohort, 22% needed MV in the first week of admission. Days between onset of weakness and admission, Medical Research Council sum score, and presence of facial and/or bulbar weakness were the main predictors of MV. The prognostic model had a good discriminative ability (AUC, 0.84). In the validation cohort, 14% needed MV in the first week of admission, and both calibration and discriminative ability of the model were good (AUC, 0.82). The scoring system ranged from 0 to 7, with corresponding chances of respiratory insufficiency from 1 to 91%. Interpretation This model accurately predicts development of respiratory insufficiency within 1 week in patients with GBS, using clinical characteristics available at admission. After further validation, the model may assist in clinical decision making, for example, on patient transfer to an intensive care unit. ANN NEUROL 2010;67:781,787 [source] Mutation in BAG3 causes severe dominant childhood muscular dystrophy,ANNALS OF NEUROLOGY, Issue 1 2009Duygu Selcen MD Objective Myofibrillar myopathies (MFMs) are morphologically distinct but genetically heterogeneous muscular dystrophies in which disintegration of Z disks and then of myofibrils is followed by ectopic accumulation of multiple proteins. Cardiomyopathy, neuropathy, and dominant inheritance are frequent associated features. Mutations in ,B-crystallin, desmin, myotilin, Zasp, or filamin-C can cause MFMs and were detected in 32 of 85 patients of the Mayo MFM cohort. Bag3, another Z-disk,associated protein, has antiapoptotic properties, and its targeted deletion in mice causes fulminant myopathy with early lethality. We therefore searched for mutations in BAG3 in 53 unrelated MFM patients. Methods We searched for mutations in BAG3 by direct sequencing. We analyzed structural changes in muscle by histochemistry, immunocytochemistry, and electron microscopy, examined mobility of the mutant Bag3 by nondenaturing electrophoresis, and searched for abnormal aggregation of the mutant protein in COS-7 (SV-40 transformed monkey kidney fibroblast-7) cells. Results We identified a heterozygous p.Pro209Leu mutation in three patients. All presented in childhood, had progressive limb and axial muscle weakness, and experienced development of cardiomyopathy and severe respiratory insufficiency in their teens; two had rigid spines, and one a peripheral neuropathy. Electron microscopy showed disintegration of Z disks, extensive accumulation of granular debris and larger inclusions, and apoptosis of 8% of the nuclei. On nondenaturing electrophoresis of muscle extracts, the Bag3 complex migrated faster in patient than control extracts, and expression of FLAG-labeled mutant and wild-type Bag3 in COS cells showed abnormal aggregation of the mutant protein. Interpretation We conclude mutation in Bag3 defines a novel severe autosomal dominant childhood muscular dystrophy. Ann Neurol 2008 [source] Cardiac and respiratory failure in limb-girdle muscular dystrophy 2IANNALS OF NEUROLOGY, Issue 5 2004Maja Poppe MD Mutations in the gene encoding fukutin-related protein cause limb-girdle muscular dystrophy 2I. In this multicenter retrospective analysis of 38 patients, 55.3% had cardiac abnormalities, of which 24% had developed cardiac failure. Heterozygotes for the common C826A mutation developed cardiac involvement earlier than homozygotes. All patients initially improved while receiving standard therapy. Independent of cardiac status, forced vital capacity was below 75% in 44.4% of the patients. There was no absolute correlation between skeletal muscle weakness and cardiomyopathy or respiratory insufficiency. These complications are a primary part of this specific type of limb-girdle muscular dystrophy, with important implications for management. Ann Neurol 2004;56:738,741 [source] A DIFFERENT APPLICATION METHOD OF TALC PLEURODESIS FOR THE TREATMENT OF PERSISTENT AIR LEAKANZ JOURNAL OF SURGERY, Issue 8 2006Dalokay Kilic Persistent air leak is a serious problem that may cause empyema, hypoxia, respiratory insufficiency, and other life-threatening complications. Chemical pleurodesis may be carried out for the treatment of persistent air leak if the lung is fully expanded. However, the standard method of chemical pleurodesis entails clamping the chest tube for a period of time after instillation of the agent. In patients with massive air leak, this would result in a tension pneumothorax. Therefore, standard chemical pleurodesis for persistent air leak is not an appropriate treatment for these patients. In this study, we carried out talc pleurodesis in six patients using an inverted U-shaped chest tube elevated to 60 cm that did not result in tension pneumothorax and mediastinal shift. No recurrence was observed during a mean follow up of 16.2 months. [source] Causes and place of death in Italian patients with amyotrophic lateral sclerosisACTA NEUROLOGICA SCANDINAVICA, Issue 3 2010R. Spataro Spataro R, Lo Re M, Piccoli T, Piccoli F, La BellaV. Causes and place of death in Italian patients with amyotrophic lateral sclerosis. Acta Neurol Scand: 122: 217,223. © 2010 The Authors Journal compilation © 2010 Blackwell Munksgaard. Objectives,,, To determine the causes and place of death in a cohort of Italian patients with amyotrophic lateral sclerosis (ALS). A better understanding of the likely causes of death in ALS might improve the palliative care at the end-of-life, whereas knowing the place of death will help to verify the need for highly specialized care services, e.g. hospice and nursing home. Patients and methods,,, Between 2000 and 2008, 182 ALS patients (onset: spinal, 127; bulbar, 55; M/F: 1.6) were followed in a single ALS Tertiary Centre in Palermo, Sicily, Italy until death. Medical data for each individual patient were recorded in a large database throughout the disease course. Information concerning causes and place of death were obtained by consultation with relatives or the family physician. Results,,, Respiratory failure (terminal respiratory insufficiency, pneumonia) was the most frequent cause of death (81.3%), which included six cases (3.3%) who requested a terminal sedation. Sudden death and death during sleep accounted for by 6.0% and 6.6% of all deaths, respectively. Heart-related causes of death were relatively infrequent in our cohort, accounting for by 7.1% of all deaths (i.e. sudden death: 6.0% and myocardial infarct: 1.1%). Patients (85.2%) died at home. Conclusions,,, The leading cause of death in ALS remains the respiratory failure, followed by the sudden death and death during sleep. Most patients in our cohort died at home, a choice that might be only partially driven by cultural factors. These findings might have a great impact on the development of the advanced and end-of-life palliative care and in the planning of specialized care services, as hospice and nursing home. [source] Phrenic paresis,a possible additional spinal cord dysfunction induced by neck manipulation in cervical spondylotic myelopathy (CSM): A report of two cases with anatomical and clinical considerationsCLINICAL ANATOMY, Issue 3 2001Wesley W. Parke Abstract The clinical records of two male subjects with severe cervical spondylotic myelopathy (CSM) who developed respiratory insufficiency after the cervical manipulation involved in preoperative anesthetic intubation were examined. Their cervical imaging was analyzed with respect to the known anatomic relationships of the spinal phrenic nerve nuclei to the spondylotic compressive lesions in an attempt to provide the anatomic and pathologic rationales that may explain this phrenic paresis as a possible traumatic complication of severe CSM. Perusal of extant literature revealed extensive descriptions of CSM symptoms, but none had previously reported an associated neuromuscular weakness of the diaphragm. Magnetic resonance imaging analyses indicated that the existing degree of upper cervical cord compression, when reinforced by the additional posterior and anterior pressures consequent to cervical spinal extension and flexion, could readily account for the functional impairment of phrenic nerve neuron cells and/or their efferent fibers. Thus, the anatomic relations of the phrenic nerve nuclear columns and their efferent tracts predispose them to interference by compressive lesions found in CSM, and undue manipulation of the cervical spine when advanced stenosis is known to be present should be recognized as a possible cause of cervical spondylotic myelopathic,phrenic paresis. Clin. Anat. 1:173,178, 2001. © 2001 Wiley-Liss, Inc. [source] Perinatal risk factors of adverse outcome in very preterm children: a role of initial treatment of respiratory insufficiency?ACTA PAEDIATRICA, Issue 2 2004B Mřlholm Hansen Aim: To investigate risk factors of adverse outcome in a cohort of very preterm children treated mainly with nasal continuous positive airway pressure (CPAP) during the neonatal course. Methods: In Denmark, preterm children are treated with nasal CPAP as a first approach to respiratory support. A national prospective study of all infants with a birthweight below 1000 g or a gestational age below 28 wk born in 1994,1995 was initiated to evaluate this approach. Of the 269 surviving children 164 (61%) were not treated with mechanical ventilation in the neonatal period. A follow-up of the children at 5 y of age was conducted. Data from the neonatal period and the 5-y follow-up were analysed. Results: In multivariate analyses including 250 children, a severely abnormal neonatal brain ultrasound scan was predictive of cerebral palsy (OR= 19.9, CI 95%: 6.1,64.8) and intellectual disability (OR = 6.2, CI 95%: 2.3,16.5). A high Clinical Risk Index for Babies (CRIB) score (OR = 2.4, CI 95%: 1.1,5.5) and chronic lung disease (OR = 2.8, CI 95%: 1.2,6.9) were predictive of intellectual disability. In univariate analyses mechanical ventilation was associated with cerebral palsy (OR = 4.3, CI 95%: 1.7,10.8) and intellectual disability (OR = 2.2, CI 95%: 1.2,4.2), but the associations became insignificant in multivariate analyses including chronic lung disease and a severely abnormal ultrasound scan. Conclusion: The associations between neonatal risk factors and adverse outcome in our cohort were very similar to those found in other cohorts with another initial treatment of respiratory insufficiency. We found no significant adverse effects of mechanical ventilation beyond what could be explained by associations with chronic lung disease and IVH 3,4/PVL. [source] | ||||||||||||||||||||||||||||