Endobronchial leiomyoma: a case report.
Leiomyoma of the lung is very rare in children. The case history is presented of an 8 year old boy with an endobronchial leiomyoma which caused total atelectasis of the left lung.
Biomedical subjects
Publications and source records attributed to N Kiper.
Leiomyoma of the lung is very rare in children. The case history is presented of an 8 year old boy with an endobronchial leiomyoma which caused total atelectasis of the left lung.
Sodium chloride deficiency (SCD) was observed within the 1st year of life in 12 of 46 cystic fibrosis (CF) patients between July 1989 and September 1992. All patients showed sweating, loss of appetite, fever, vomiting, irritation, dehydration, weakness, and cyanosis during an attack. Mean plasma sodium, potassium and chloride levels were 122.9 (range 106-135), 2.5 (range 1.6-3.5), and 73.3 (range 60-90) mEq/l respectively. Alkalosis and elevated plasma renin activity were detected in all patients. Of the patients, 50% showed microscopic haematuria, and hypercalciuria was detected in two out of four patients. Low urinary sodium and high urinary potassium were observed in the four examined patients. Increased creatinine, BUN and uric acid values returned to normal with treatment. All the patients were treated initially with intravenous fluids and electrolyte solutions. All patients were less than 7 months of age during the first attack, five received only breast milk and the others breast milk with formula milk. Their oral salt supplement was 2-4 mEq/kg per day, which is recommended for CF patients, but could be deficient in excessively sweating infants. The genotype of these patients might be cause of high salt losses. F508 is the most common mutation with the frequency of 38% in our CF patients with SCD, but the frequency of unknown mutations is high (54%).
SETTING: In developing countries including Turkey, tuberculosis is still a major problem. Rapid diagnosis and early medical intervention are the two most important considerations in preventing the spread of the disease. OBJECTIVE: This study was carried out to determine the diagnostic value of BCG test in childhood tuberculosis and compare it with tuberculin test in this regard. DESIGN: 50 patients and 20 healthy children without any evidence of previous BCG vaccination and aged 80 days-15 years were simultaneously tested with purified protein derivative (PPD) and BCG vaccine. RESULTS: In pulmonary tuberculosis BCG test was positive in 100% of cases and the PPD test in 44.5%. Similarly, BCG test was positive in 100% of military tuberculosis and tuberculous meningitis cases but PPD test was negative in all of them. Out of 22 patients with malnutrition 18 (82%) had positive BCG test and 4 had positive PPD test. BCG test showed uniformly high positivity in all grades of malnutrition. CONCLUSION: BCG is more reliable and sensitive than the tuberculin test in the diagnosis of tuberculosis. It is still valuable in the diagnosis of tuberculosis especially in developing countries where the disease is still a major public health problem and where sophisticated methods such as rapid culture with BACTEC and demonstration of bacilli with DNA probes are not widely available.
The general characteristics and treatment of childhood tuberculous spondylitis are reviewed. Most of the patients (79 per cent) were under 5 years old with girls being in greater number. Twenty-six patients used classical antituberculous chemotherapy and five were given intermittent chemotherapy. There was no significant difference in the mean period of inactivation of the disease (2.8 v. 2.9 months) or in post-treatment angle of kyphosis (33 degrees v. 37.6 degrees) between the classical and intermittent chemotherapy groups. In addition to drug therapy, plaster jacket and immobilization were adequate in four children whereas 27 underwent surgery with only two requiring radical operations. In children with tuberculous spondylitis, intermittent drug treatment is a good alternative due to effectiveness and convenience of use and radical surgery should be reserved for selected cases.
Seventy-two children with no underlying diseases were treated for empyema. Radical surgical approaches like decortication were necessary for only 3 children. In 66 children tube drainage was applied. Staphylococcus aureus was cultured or was shown in Gram's stain in 32 (44%) and children with this microorganism had longer duration of tube drainage (p < 0.05). The period for normalization of chest X-ray was positively related with the age of the patient (p < 0.05). In 60 of 72 children, chest X-ray was normal after 3 months. Follow-up of the patients 18 months after the infection revealed that pulmonary radiograms were normal in all cases and pulmonary function tests were within normal limits in all of the tested children (n = 25). It is emphasized that avoiding major surgical approaches must be encouraged in childhood empyema.
The general characteristics of 56 childhood cases of cystic hydatid disease were analysed and the results of mebendazole therapy versus surgery were verified. Pulmonary radiograms and ultrasonography were used in the diagnosis. The cysts were localized primarily to the lungs. Twenty seven patients were surgically-treated, with eight having recurrence after a mean period of 3.6 yrs. Thirty patients received regular mebendazole treatment, in a dose of 50 mg.kg-1 with a mean duration of treatment of 11.7 months. Twenty one patients were cured and discontinued the therapy. Nine still use the drug, seven of whom have had dramatic improvement, while the other two have minimal radiographic changes but subjective improvement in general condition. The lung cysts vanished leaving minimal scars, whilst the liver cysts turned into inactive forms. The surgically-treated and drug-treated groups were similar in age, duration and severity of the disease. The recurrence rate of drug-treated children (1 out of 20) was lower than that of the surgically-treated children (8 out of 27); however, this was not statistically significant.
The plasma levels of mebendazole in children with hydatid cyst disease were measured with high pressure liquid chromatography. In 24 children who received mebendazole orally in a dose of 50 mg/kg, the mean (SD) level 4 hours later was 25.76(9.81) ng/ml (87.6(33) nanomole/l). This result was similar to those in most adult series. During more prolonged treatment, the plasma level 4 hours after the dose of the drug rose significantly with respect to the initial level (p < 0.05).
A diphosphonate, disodium etidronate, a compound known to inhibit microcrystal growth of hydroxyapatite was given to a 3.5-year-old girl with pulmonary alveolar microlithiasis (PAM) that was symptomatic. The drug was used for approximately 36 months in a single daily dose of 15 mg/kg. No significant side effects were encountered with somewhat clearing of lung bases in chest radiograms and subjective improvement in general condition. These findings suggest a role of diphosphonates in the treatment of PAM.
A total of 130 children diagnosed as having pulmonary and extrapulmonary tuberculosis who received short course intermittent chemotherapy between 1978-1992 were evaluated retrospectively. One hundred and ten children with tuberculosis were treated with isoniazid (10-15 mg/kg, maximum 400 mg), rifampin (10-15 mg/kg, maximum 600 mg), and streptomycin (30 mg/kg, maximum 1 g) daily, for 15 days. Treatment was completed with similar doses of isoniazid and rifampin twice a week for a period of 9 months. Since 1986, 20 children with tuberculosis were being treated with the same regimen but without streptomycin. The majority of patients in these cases had pulmonary tuberculosis (75%), followed by lymph nodes (9%), pleural (7%), bone and joint (5%), miliary (3%), and abdominal tuberculosis (1%). The clinicoradiologic response to treatment was observed to be excellent. Only one case of relapse was detected, which was the case of a patient with lymph node tuberculosis that occurred 18 months after the completion of treatment. No serious adverse drug reaction was observed in any of the cases mentioned. In conclusion, short-course low-dose intermittent chemotherapy is an effective and economical treatment with minimal side effects for pulmonary and extrapulmonary tuberculosis in childhood.
Cardiac involvement was evaluated by echocardiography in 26 young cystic fibrosis patients. The mean age was 48.4 months (range 3 months to 15 years). The findings were compared with 26 age- and sex-matched children without a history of cardiopulmonary complaints. All patients had normal values of left ventricular ejection fraction and fractional shortening. Interventricular septal and posterior left ventricular wall thicknesses were similar to control group but right ventricular free wall thickness was found greater than in the control group. Abnormal septal motion was documented in six patients. Right ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in two patients and there was a negative correlation with clinical Shwachman scores (r: -0.55). Left ventricular pre-ejection period to ventricular ejection time ratio was found over the upper limit of normal in five patients. For both mitral and tricuspid valves, the mean ratios of peak velocity during passive filling (E) phase of diastole to peak velocity during atrial contraction (A) phase were found significantly lower than in the control group (p < 0.05). Early diastolic peak velocity was similar to that in the control group but late atrial peak velocity was higher in the patient group (p < 0.05). Isovolumic relaxation time was found the same as in the control group. We conclude that cardiac changes in diastolic and systolic functions begin at very young ages in cystic fibrosis patients.
Allergic bronchopulmonary aspergillosis (ABPA) is hypersensitivity to Aspergillus fumigatus which manifests as episodic wheezing, usually refractory to bronchodilator therapy, with fixed and transient pulmonary infiltrates, central bronchiectasis, blood eosinophilia, elevated serum IgE level, immediate skin reactivity to an A. fumigatus antigen and precipitating antibodies to A. fumigatus. It is an unusual complication of asthma and cystic fibrosis (CF). We present two cystic fibrosis patients with ABPA treated successfully with prednisone and, in Case 1 also with itraconazole. The physician should be alert to the possibility of ABPA whenever CF patients present with the new infiltrates, high serum total IgE and other positive parameters of A. fumigatus sensitization. Treatment with systemic steroids should be started in order to prevent irreversible lung damage.
A seven-year-old girl with Turner's syndrome, who suffered from recurrent respiratory system infections since birth, was investigated to determine the etiology of bronchiectasis. Electron microscopy of recurrent nasal biopsy specimens revealed ciliary aplasia. Ciliary aplasia in Turner's syndrome, has not previously been reported.
Miliary tuberculosis is a severe manifestation of tuberculosis. Six children aged between two months and 10 years with the diagnosis of miliary tuberculosis were treated with intermittent antituberculous therapy for six, nine or 12 months. All the patients showed clearance of both clinical and radiological symptoms; there was no drug toxicity or resistance and no relapses were seen in the follow-up period ranging from nine months to nine years. Intermittent therapy is safe and effective in miliary tuberculosis and it may be an alternative therapy because of its minimal toxicity and lower cost.
An ear-nose-throat survey was carried out on 30 children with cystic fibrosis (CF) between two and 17 years of age. There were three (10%) confirmed cases of secretory otitis media. One child had previous surgery for polyps. Ten children had chronic pansinusitis mildly responsive to medical therapy. Hypertrophy of the nasal conchae was detected in eight cases. The data showed that the risk of ear-nose-and-throat disease was increased in CF patients, but audiologic problems are not very common in CF patients. CF should be kept in mind in children who have nasal polyps, hypertrophied turbinates or sinusitis unresponsive to medical therapy.
A 10-year-old girl with yellow dystrophic nails, bronchiectasis, chronic sinusitis and lower-limb lymphedema is presented. The underlying mechanism remains unknown although it has been postulated to be associated with lymphatic abnormalities. To date no causative treatment exists. Our patient was treated with conservative management, including a low-fat diet supplemented with medium-chain triglycerides. Moderate improvement in the lymphedema of the lower extremities was observed. To our knowledge this is the first case of yellow nail syndrome to be treated with diet.
Tuberculosis of the middle ear is currently a rare disease but still occurs and may cause permanent hearing loss. A four-month-old infant with chronic left-ear drainage was diagnosed with tuberculous otitis media by biopsy examination and PPD positivity without BCG. He was treated successfully with antituberculous therapy. Tuberculosis should be considered in the differential diagnosis of chronic ear infection, especially in young infants.
The purpose of this study was to determine the prevalence of anti-tuberculosis drug resistance in children followed at Hacettepe University Ihsan Doğramaci Children's Hospital. Sixty cases with tuberculosis for whom susceptibility testing was available were searched retrospectively. Teh overall drug resistance was 26.7 percent. Resistance to streptomycin (sm) was the most frequent (18.3%), followed by isoniazid (6.7%), rifampicin (6.5%), and ethambutol (4.2%). Strain resistant to more than one drug was present in two cases (3.3%). In summary, excluding SM, both single and multidrug resistance were relatively low in our pediatric patients.
Pulmonary hemosiderosis may rarely be associated with juvenile rheumatoid arthritis or can develop during the course of the disease. We present a three-year-old boy with severe iron deficiency anemia (without any pulmonary symptoms) and arthralgia at the time of diagnosis. Two years after the initial diagnosis he developed pulmonary hemosiderosis and pauciarticular type of juvenile rheumatoid arthritis which progressed to seronegative polyarticular juvenile rheumatoid arthritis. He responded very well to prednisolone and was maintained well on low-dose alternate-day prednisolone and naproxen sodium treatment. This is the only case of association of these two diseases in our experience in both the Pediatric Rheumatology and Pediatric Respiratory Diseases Departments.