Nine Japanese patients with immotile-dyskinetic cilia syndrome: an ultrastructural study using tannic acid-containing fixation.

Respiratory cilia and sperm flagella of nine Japanese patients with immotile-dyskinetic cilia syndrome were studied ultrastructurally by using a tannic acid-containing fixative. Respiratory cilia from two female patients with Kartagener's syndrome and one male patient with situs inversus and sinobronchitis were completely immotile and lacked both dynein arms. However, approximately 30% of the spermatozoa from the male patient were weakly motile. In four patients with immotile cilia syndrome without Kartagener's triad, immotile respiratory cilia generally lacked the inner dynein arms. Two clinically unusual cases, an 11-year-old boy and a 29-year-old woman with prolonged saccharin test, recurrent bronchitis, and bronchiectasia, possessed motile respiratory cilia. Ultrastructurally, both dynein arms were normal, but numerous defective central pairs (more than 50% and 70%, respectively) were seen, and the defect in the second case was similar to the transposition of microtubules reported by Sturgess et al (N Engl J Med 303:318-322, 1980). However, defects in the first case were unique and may be congenital. We propose a new type of dyskinetic cilia syndrome with defective central pairs. Additionally, nasal cilia from a 35-year-old man with immotile cilia syndrome contained excess large singlets within ciliary axonemes consisting of 17 protofilaments.

[Tuberculous meningitis in Japanese children between 1980-1991].

We evaluated the clinical and laboratory findings of tuberculous meningitis admitted to Tokyo Metropolitan Children's Hospital from 1980 through 1991 retrospectively. They consisted of 26 patients (14 boys and 12 girls), and their age ranged from 4 months to 11 years. Seventeen patients were under 3 years of age. Mortality rate was 4% (1/26), and there were 13 patients with sequelae on discharge from the hospital, and 12 patients without any sequelae. The period to recover normal CSF findings was relatively long. Some cases which did not seem to respond well to chemotherapy at the initial stage, recovered from meningitis without sequelae. This suggests that it is not necessary to change the chemotherapy at the initial few weeks of therapy. The risk factors of poor prognosis were; age less than 2 years, decreased level of consciousness on admission, convulsion, CSF protein more than 70 mg/dl, and CSF glucose less than 20 mg/dl. Tryptophan reaction was not always positive. Chloride in CSF was not so important to diagnose tuberculous meningitis in children. For the proper diagnosis of tuberculous meningitis in children one should consider several factors such as tuberculin skin test, family history, chest X-ray findings and CSF study. BCG was inoculated in six children (three patients were under one year old and the rests were older than 4 years). In three patients under one year old BCG was seemed to be inoculated after Mycobacterium tuberculosis infection, and apparently not effective to prevent tuberculosis meningitis. We conclude that BCG vaccination is necessary in early infancy to prevent tuberculous meningitis.

[Active tuberculosis in children who received INH chemoprophylaxis].

Twelve children who developed active tuberculosis even after receiving isoniazid (INH) chemoprophylaxis were seen at Tokyo Metropolitan Children's Hospital from 1982 through 1991. All cases received INH more than 9 mg/kg/day, except for one case in which the amount of INH administered at the referring hospital was unknown and Streptomycin was administered together with INH. The age of starting INH prophylaxis ranged from 2 months to 13 years, and the age at which clinical symptoms and/or laboratory evidences of active tuberculosis were first manifested ranged from 4 months to 18 years. Five patients developed active tuberculosis after the completion of chemoprophylaxis and patients during chemoprophylaxis, with the first presentation ranging from primary complex (seven), chronic pulmonary tuberculosis (two), tuberculous meningitis (two), and tuberculous pleuritis (one). None of the Mycobacterium tuberculosis resistant to INH was isolated. Reviewing these patients, eleven cases had at least one of the following factors: (1) age less than two years old (2) infectious sources expectorated more Mycobacterium tuberculosis (3) delay in starting INH. Above factors should be considered in initiating INH chemoprophylaxis and subsequent follow-up of the patients.

Ultrastructure of respiratory cilia of WIC-Hyd male rats. An animal model for human immotile cilia syndrome.

The WIC-Hyd rat is a mutant from the Csk: Wistar-Imamichi rat, with spontaneous hydrocephalus. In male rats, the hydrocephalus is severe and about one half of hydrocephalic male littermates possess situs inversus totalis. Ependymal cilia in these animals are immotile, and this defect is regarded as a mechanical cause of hydrocephalus. This paper presents the ultrastructural features of respiratory cilia in these rats in comparison with those in human immotile cilia syndrome. The respiratory cilia in these rats also are immotile and the dynein arms are missing, as in human cases. Previously only eight dogs with immotile cilia syndrome and a mutant hydrocephalic-polydactyl mouse were reported with respect to these phenomena. However the WIC-Hyd rat is the first useful animal model for human immotile cilia syndrome, and further studies may serve to clarify the genetic background of this condition.

Longitudinal follow-up of pulmonary function after lobectomy in childhood - factors affecting lung growth.

We examined the changes in pulmonary function values in 27 patients who underwent a lobectomy due to cystic lung disease and compared the results regarding such factors as disease type, age at operation, and preoperative infections. Percent vital capacity (%VC) decreased immediately after lobectomy, but recovered to normal values within 2 postoperative years and remained within or above the normal range. The ratio of residual volume to total lung capacity (RV/TLC) rose temporally with the increase in %VC, but then remained normal after 2 postoperative years. There was no difference in %VC and RV/TLC between diseases, while bronchial atresia showed a significantly lower correlation with percent of forced expiratory volume at 1 s. The older group operated upon at over 4 years of age and the group that had infections before operation showed relatively low %VC and high RV/TLC. Some patients demonstrated extremely low %VC along with funnel chest deformities. Our study suggests that overinflation of the remaining lung compensates VC in the early period after lobectomy while subsequently alveolar multiplication occurs. Factors affecting compensatory lung growth were considered to be operation later than 4 years of age, preoperative infection, and a thoracic deformity.