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PubMedDate not supplied

[On a case of Guillain - Barre's polyradiculoneuritis with arterial hypertension (author's transl)].

A twenty year old man affected by Guillain-Barré's radiculopolyneuritis with tetraparesis had high protein and only few cells in the C.S.F., with no cranial nerve involvement or respiratory difficulty. He had high arterial pressure (both diastolic and systolic) for about three months accompanied by episodes of tachycardia and hyperhydrosis. Investigations of renal functionas well as catecholamine and vanil-mandelic acid urinary excretion were normal. Tilting test and carotid sinus reflex were normal. The patient recovered almost completely eight months after the onset of his illness. The presence of arterial hypertension in the Guillain-Barré's syndrome has been rarely reported with no respiratory involvement; however this condition may be less rare than suspected. The pathogenesis of this complication is not easy to explain. According to the authors this condition may be due, in cases, to impairment of the sympathetic system mostly at peripheral level. In some cases of Guillain-Barré's syndrome renal involvement of immunopathological origin might be the cause of hypertension. The authors emphasize the importance of a correct evaluation of symptoms in the prognosis and in the choice of therapy.

Adult↗
PubMed1984

[Sarcoidosis at the Cochin University Hospital Center from 1975 to 1982].

An analysis of 80 cases of sarcoidosis admitted to the departments of respiratory medicine (36), rheumatology (19) and internal medicine (25) over a 7 year period, revealed a wide range of clinical presentations; there was a higher incidence of associated disease and a greater number of localisations of the disease in patients admitted to the department of internal medicine than in those admitted to the other two departments. On the other hand, bronchial biopsy was more commonly positive in patients admitted to the department of respiratory medicine whose respiratory function was more disturbed than the patients in the other two departments. The patients referred to the departments of rheumatology and internal medicine without radiological respiratory involvement had respiratory function tests and positive alveolar lavages. The prognosis was the same in all three departments; 50 p. 100 were treated with steroids. The recruitment of the patients in this series allows a different evaluation of the disease compared to series reported from more specialised departments.

Adolescent↗
PubMed1978

Sinus histiocytosis with massive lymphadenopathy (Rosai-Dorfman disease): Report of a case with respiratory tract involvement.

The term "sinus histiocytosis with massive lymphadenopathy," introduced in 1969 by Rosai and Dorfman, refers to a newly recognized disease entity characterized by painless cervical lymphadenopathy, fever, leukocytosis, increased erythrocyte sedimentation rate, and hypergammaglobulinemia. The typical course is one of insidious onset, protracted duration of the active disease state, and eventual spontaneous remission, occasionally with subsequent recurrences. Lymph nodes other than those in the cervical area may be involved, and extranodal involvement can occur (such as in the orbit, skin, or respiratory tract). Clinically, this entity may closely simulate malignant lymphoma or nonneoplastic conditions with lymphadenopathy. We present a case in which this disease process extensively involved the respiratory tract and produced obstructive symptoms. The otorhinolaryngologist should be aware of this disease entity and should include it in the differential diagnosis for patients with cervical lymph node enlargement or bronchial obstruction.

Adult↗
PubMedDate not supplied

[Resistance to antibiotics of bacteria involved in respiratory infections (author's transl)].

The present status of resistance to antibiotics of bacteria involved in respiratory infections is reviewed. Schematically it can outlined as follows. Streptococcus B-haemolyticus as well as Pneumococcus did not change their sensitivity to penicillin, but some strains are now resistant to tetracycline. Streptococcus viridans, Enterococcus and H. influenzae did not change substantially their sensitivity to antibiotics. Staphylococcus aureus is the bacterial species that always poses some problems with regard to antibiotic resistance. Due to the selection of strains penicillinase-producing because of the large use of penicillin, the most part of clinical isolates of staphylococci is now resistant to penicillin. In addition an increased number of strains resistant to the other antibiotics has been registered as soon as they has been introduced in therapy. The resistant strains spread in a particularly rapid way in hospital. The introduction in therapy of penicillinase-resistant penicillins constituted a remarkable advance in therapy of staphylococcal infection. However, there is now a growing number of indications about the emergence of methicillin resistant strains of staphylococci. On the other hand it must be recalled that since 1960 a marked reduction of incidence and mortality in severe staphylococcal infections has been noted. Incidence and mortality of respiratory infections due to Gram-negative bacilli is augmented particularly in connection with a larger use of immunosuppressive and antineoplastic therapies, of particular surgical or reanimation procedures, of intensive courses of antibiotic therapy etc. Emergence of Pseudomonas, Proteus, Serratia, Providencia, etc. infections poses many difficult problems of chemotherapy since these species are scarcely sensitive to antibiotics. Carbenicillin, cephalosporins, sisomicin, tobramycin, amikacin are the more recent drugs that alone or in combination may offer some chances of success in this field.

Anti-Bacterial Agents↗
PubMed2026

Respiratory manifestations as clues to inherited metabolic disorders in children: a phenotype-driven diagnostic approach.

UNLABELLED: Inherited metabolic disorders (IMDs) are uncommon but clinically important causes of respiratory morbidity in children. Respiratory involvement may be the first or dominant manifestation, although it may precede, accompany, or follow systemic involvement. Because cough, dyspnea, hypoxemia, recurrent infection, abnormal chest imaging, and ventilatory failure are non-specific, affected children may initially be managed for common respiratory conditions, such as infection, asthma, aspiration, immunodeficiency, or non-metabolic diffuse lung disease, before the underlying IMD is recognized. This narrative mini-review presents a phenotype-driven approach to recognizing IMDs in pediatric respiratory practice. Rather than cataloguing rare disorders by metabolic pathway, it organizes respiratory involvement into practical clinical entry points: diffuse lung disease, pulmonary alveolar proteinosis-like disease, pulmonary vascular disease, recurrent infection or bronchiectasis, upper-airway or thoracic restriction, and neuromuscular respiratory failure and aspiration. For each pattern, we highlight extrapulmonary red flags and first-line biochemical, enzymatic, and genetic tests that may guide early etiological diagnosis. CONCLUSION: Careful recognition of respiratory phenotypes, combined with targeted metabolic and genomic evaluation, may shorten diagnostic delay and allow disease-specific treatment before irreversible pulmonary or neurological injury occurs. WHAT IS KNOWN: • IMDs can involve the respiratory system and may mimic common pediatric respiratory disorders or non-metabolic forms of childhood diffuse lung disease. • Respiratory manifestations may precede, accompany, or follow classical systemic features, and their temporal pattern varies among individual IMDs. WHAT IS NEW: • This mini-review organizes IMD-related respiratory involvement by presenting respiratory phenotype rather than by metabolic pathway. • It links respiratory entry points with extrapulmonary red flags and targeted biochemical, enzymatic, and genetic testing to support earlier diagnosis.

Humans↗
PubMed1992

Unusual manifestation of Stevens-Johnson syndrome involving the respiratory and gastrointestinal tract.

There are many recognized acute complications of Stevens-Johnson syndrome: however, long-term sequelae involving the respiratory and gastrointestinal tracts are exceedingly rare. We present an 8-year-old girl who developed esophageal stricture and chronic lung disease while recovering from Stevens-Johnson syndrome and discuss these complications with possible mechanisms for their occurrence.

Child↗
PubMed1999

[A case of microscopic polyangiitis with severe cardiac and respiratory muscle involvement].

A 66-year-old female was admitted to our hospital in January, 1998, complaining of low grade fever and muscle weakness of her legs. Physical examination revealed muscle weakness of her neck (4/5) and proximal skeletal muscles of her bilateral legs (3/5-4/5). She showed proteinuria and microhematuria. Her serum levels of ureanitrogen, creatinine, aspartate aminotransferase, alanine aminotransferase, creatinekinase, aldolase and myoglobin were all within the normal ranges. Antinuclear antibodies were negative, but her serum levels of pANCA (743 EU) and C reactive protein (18.0 mg/dl) were elevated. Neuroconduction velocity of her left common peroneal nerve was decreased to 40.8 m/sec and electric myograph showed neurogenic changes. Magnetic resonance images (MRI) of her bilateral thigh depicted high signal intensity in quadriceps by T 2 weighed images, but the signals were not enhanced by gadolinium injection. Muscle and renal biopsies revealed necrotizing vasculitis of the small arteries. Crescentic glomerulonephritis was also observed by renal biopsy. These findings supported the diagnosis of microscopic PN. On 16 th admission day, she developed acute cardiac and respiratory failures due to cardiac and respiratory muscle involvements with PN, and was assisted by mechanical ventilation. She was treated with methylprednisolone pulse therapy (500 mg/day, three consecutive days) on 18 th admission day, followed by 40 mg of oral prednisolone daily. However, her symptoms deteriorated, and herserum creatinine levels increased to 2.4 mg/dl. On 24 th admission day, intravenous cyclophosphamide pulse therapy (500 mg/day) was instituted. Her cardiac wall motion on echocardiography and serum creatinine levels gradually improved, but her skeletal and respiratory muscle weakness did not improve. On 38 th admission day, she was complicated with respiratory infection by methicillin resistant Staphylococcus aures. On 62 th admission day, she died of endotoxic shock. This is the first report describing respiratory muscle involvement with PN, and the second report describing MRI findings of muscle involvement by PN. Therefore, our case provides important clinical information for the diagnosis and treatment of the disease.

Aged↗
PubMed2003

[Airway manifestations of relapsing polychondritis: treatment with cyclophosphamide and placement of bronchial stents].

We report the case of a 41-year-old female patient with relapsing polychondritis and severe respiratory involvement. The patient presented with acute respiratory failure requiring endotracheal intubation. Bronchoscopy revealed tracheal collapse and inflammatory stenoses with dynamic collapse of the major airways. We describe a multidisciplinary therapeutic approach, consisting of immunosuppressive treatment, bronchoscopic placement of self-expandable stents into the collapsing bronchi, and tracheotomy. In addition, we report the effectiveness of oral cyclophosphamide for treatment of relapsing polychondritis with severe respiratory involvement after failure of other immunosuppressive agents. The problem of severe respiratory complications in patients with relapsing polychondritis and the need for a multidisciplinary approach is discussed.

Adult↗
PubMed1991

Persistence of respiratory abnormalities four years after the onset of toxic oil syndrome.

We studied a random sample of 436 subjects with TOS aged 18 to 60 years, to assess the prevalence of respiratory involvement four years after onset of the syndrome. Clinical evaluation together with chest roentgenograms, electrocardiogram and functional respiratory tests were carried out. Respiratory involvement occurred in 390 (89.4 percent) individuals and was the most common abnormality detected, followed by neurological disorders in 289 (66.3 percent), osteoarticular symptoms in 171 (39.2 percent), psychiatric disorders in 96 (22 percent), hepatic involvement in 91 (20.9 percent), and sclerodermatous sequelae in 89 (20.4 percent). Among patients with respiratory involvement, dyspnea and cough were the most common complaints. Decreased VC was observed in 151 (34.6 percent) patients and reduced transfer factor of CO in 95 (21.8 percent) patients. Airway obstruction and alveolar hypoventilation were observed only in nine (2.1 percent) patients. Six (1.4 percent) patients suffered from pulmonary artery hypertension.

Adolescent↗
PubMed1980

Lower respiratory tract involvement in Rocky Mountain spotted fever.

A retrospective study of 35 patients with Rocky Mountain spotted fever was undertaken to determine the frequency of respiratory symptoms, as well as to analyze the types of pulmonary problems encountered to hospital patients. Cough, present in only 33% of patients, led to an incorrect initial diagnosis and delay in therapy in eight individuals. Lower respiratory tract involvement (rales, abnormal chest roentgenograms, and abnormal gas exchange) was present in 42% of patients at some point during the illness. The conditions of nine patients deteriorated during the first week of hospitalization. Pulmonary edema (probably noncardiogenic) was the usual explanation for worsening gas exchange. Bacterial pneumonia and hemorrhage were detected in only two patients.

Adult↗
PubMed1999

A second locus for autosomal dominant myopathy with proximal muscle weakness and early respiratory muscle involvement: a likely chromosomal locus on 2q21.

We recently mapped a locus for a new variant of autosomal dominant myopathy (Swedish families) with proximal muscle weakness, early respiratory muscle involvement, and unique muscle biopsy findings to chromosomal region 2q24-31. In this study, a French family with a similar clinical phenotype and pathology (muscle biopsy) was investigated to see whether the disease gene associated with the myopathy is mapped to the same region as the one in the Swedish families; however, chromosomal region 2q24-q31 was completely excluded. In order to localise the disease gene for the French family, a genome-wide scan was performed using polymorphic microsatellite markers. A maximum two-point lod score of 2.11 (the highest lod score that can be achieved in this family) was obtained for the markers in the region between D2S1272 and D2S1260, spanning 4 cM. This result suggests that the gene responsible for the French form is likely to be located on chromosome 2q21.

Chromosome Mapping↗
PubMed1999

Respiratory muscle involvement in Bethlem myopathy.

We report a patient from a previously reported family with autosomal dominant Bethlem myopathy who demonstrated childhood onset, slowly progressive limb-girdle muscle weakness, contractures, and progressive respiratory compromise. Chest x-ray, pulmonary function tests, and electrophysiologic studies suggested respiratory muscle involvement, thus expanding the clinical repertoire in Bethlem myopathy.

Adult↗
PubMed1986

Bronchoscopic and radiologic features of Kaposi's sarcoma involving the respiratory system.

In a group of 61 patients admitted to New England Deaconess Hospital, Boston, with a diagnosis of acquired immune deficiency syndrome (AIDS), 25 were found to have Kaposi's sarcoma involving the skin and mucous membranes. Of these 25 patients, eight had lesions involving the respiratory system. Radiographically, patients with Kaposi's sarcoma had hilar and mediastinal adenopathy with perihilar parenchymal infiltration which progressed to diffuse bilateral infiltrates over a period of months. This pattern and the tempo of its evolution were distinctly different from the diffuse infiltrates seen in patients with Pneumocystis carinii pneumonia. Bronchoscopy was performed in seven of the eight patients, revealing characteristic lesions of Kaposi's sarcoma in the airways. We propose that parenchymal pulmonary Kaposi's sarcoma can be strongly suspected in a patient with AIDS who has the following features: a characteristic radiologic pattern; endobronchial Kaposi's sarcoma at bronchoscopy; and no evidence of opportunistic infection. In this subset of patients, further diagnostic intervention such as open lung biopsy, a procedure with potential morbidity in these ill individuals, may be unnecessary.

Acquired Immunodeficiency Syndrome↗
PubMed1998

An early onset muscular dystrophy with diaphragmatic involvement, early respiratory failure and secondary alpha2 laminin deficiency unlinked to the LAMA2 locus on 6q22.

We present four subjects from one family and one subject (with an affected sibling who had died) from a second, unrelated family, with early onset, Duchenne-like, muscular dystrophy who presented with proximal girdle weakness, calf and generalized muscle hypertrophy, selective wasting of the sternomastoid muscles, rigidity of the spine and contractures of the tendo Achilles. Intellect was normal. Serum creatine kinase was grossly elevated and the muscle biopsies showed a dystrophic picture. All five subjects have developed early respiratory failure due to severe diaphragmatic involvement; two have already died aged 4 and 7 years of age and the remaining three are dependent on night time ventilation. There has been very little deterioration over time in the skeletal muscle function, and the survivors remain ambulant, the oldest being 11 years. Immunocytochemical studies of the muscle biopsy showed a normal pattern for dystrophin and the dystrophin-associated glycoproteins, but a reduction of the laminin alpha2 chain of merosin. Magnetic resonance imaging of the brain was normal. The disease did not link to the LAMA2 locus for laminin alpha2 on chromosome 6q, so that these families seem to represent a new form of autosomal recessive muscular dystrophy with a secondary merosin deficiency. The primary protein deficiency has not yet been identified.

Age Factors↗
PubMedDate not supplied

Rod myopathy with extensive systemic and respiratory muscular involvement.

A patient with both muscle weakness and progressively degenerating respiratory function became increasingly less responsive to therapeutic intervention. The diagnosis of the muscle disease was made through biopsy samples and confirmed as rod myopathy. Through autopsy samples, the patient was found to have had extensive centriacinar emphysema and widespread involvement of rod disease in the skeletal muscles. The most involved of the muscles sampled (anterior tibial, brachioradialis, rectus femoris, psoas, diaphragm, biceps) was the diaphragm. While the influence on diaphragmatic function has been reported in the juvenile form of rod disease, this is the first known report of the extensiveness of involvement in adult-onset disease. The diseased state of the diaphragm was appreciated as a contributing factor to the respiratory insufficiency.

Adult↗
PubMed2003

High levels of hydrolytic enzymes secreted by Candida albicans isolates involved in respiratory infections.

Differences in production of two putative virulence factors of Candida albicans, phospholipase and proteinase, were determined for a large panel of clinical C. albicans isolates (n = 186) obtained from the European SENTRY programme. Seventy-two per cent of isolates produced detectable amounts of phospholipase and 95 % of isolates produced detectable amounts of proteinase. There was no clear correlation between the results of the phospholipase and proteinase assays and the geographical distribution of the isolates. However, isolates that originated from respiratory infections produced significantly higher amounts of phospholipase than isolates obtained from blood, the urinary tract or wounds/skin/soft tissue and also appeared to produce more proteinase. These virulent isolates involved in respiratory infections may originate from the oral cavity. Whether these results are caused by selection for these highly virulent isolates remains to be resolved.

Candida albicans↗
PubMed2005

Upper respiratory tract involvement in the course of diffuse infiltrative lymphocytosis syndrome in HIV-1-infected patients: report of 2 cases.

Diffuse infiltrative lymphocytosis syndrome (DILS) in patients with human immunodeficiency virus (HIV) infection is characterized by persistent CD8(+) lymphocytosis with visceral lymphocytic infiltration. DILS induces a large spectrum of clinical features. We describe 2 HIV-infected patients with upper respiratory tract involvement that occurred during the course of DILS.

Adult↗