A Case Report of Lethal Multiple Pterygium Syndrome

We report a patient with a lethal multiple pterygium syndrome. The patient was the first child born to a 28-year old mother. The family history was unremarkable; no consanguinity was reported. The patient was the product of a 37-week gestation by cesarian section, and admitted to our intensive care unit after resuscitation. He weighed 2,158 g, and measured 33cm (CHL). The patient had hydrops, cystic hygroma on the back of the neck, hypertelorism, a flat upturned nose, a highly arched cleft palate, micrognathia, low set ears and multiple pterygia. In addition, the finger showed slender and hypoplastic ridges and creases. The feet showed rocker-bottom deformities. Furthermore, the genitalia was normally formed male with bilateral cryptorchism. The spine showed scoliosis and lordosis with vertebral bony fusions, separations and reduced intervertebral spaces. The ribs were 11 pairs and gracile. The lung was hypoplastic, and the heart was small but normal in structure. Chromosomal examination revealed a normal male karyotype (46, XY). The infant died within two hours after birth.
Gillin and Pryse-Davis (1976) described three female siblings with this early lethal disorder. This disorder was separated from other conditions associated with pterygia by Hall et al. (1982). At least 30 cases have been reported. However, this type of case seems to have not been reported in Japan. This disorder is considered autosomal-recessive, but in other report, X-linked recessive inheritance is proposed. Therefore, further studies are necessary in order to make a more precise etiology of this disorder.     

Dynamics of various peptide hormone receptors in human placental cell membranes

The action of peptide hormone receptors on the cell membranes and the cyclic-AMP in its post receptor system dynamics was studied. [Results] Receptors for hCG-LH, hPL-hGH, ACTH and Insulin were found in the placental cell membranes but receptors for FSH and PRL were not found. The possibility was suggested that the cyclic-AMP was the second messenger in the receptor system for hCG-LH, hPL and ACTH. The differences between normal placental cell membranes and hydatidiform mole were studied from the point of view of the receptor level. Receptors on molar trophoblast for hCG-LH, hPL-hGH and ACTH increased about two fold to three fold over those on normal trophoblast. The presence of receptors for hCG-LH, hPL and ACTH was confirmed also in GCH-1 (gestational choriocarcinoma cell line). The possibility is shown that the placenta has an auto regularity system in its own tissue throughout the various peptide hormones receptors such as hCG-LH and hPL.     

A CASE OF FOAMY MYOCARDIAL TRANSFORMATION OF INFANCY

A case of Infantile cardiomyopathy in a year and four months old girl, which was clinically characterized by tachycardia resistent to any drugs and marked hyperplasia of mitochondria in their cytoplasms on electron microscopic observation, was presented. In the present case light and electron microscopical proof of the diagnosis was obtained by right endomyocardial biopsy, while most of this condition has not been recognized prior to autopsy. In Japan this newly recognized infantile cardiomyopathy has not been reported, and we proposed that this condition could be designated as mitochondrial cardiomyopathy caused by unknown etiology. The literatures described previously in the world have been reviewed.     

Resection and reconstruction of sternnum

In case of sternal resection, it is necessary to preserve bone material indispensable for the stability of the anterior chest wall and air tightness of the thoracic cavity, and the support of the chest wall integrity must be restored by some means. Various techniques have been applied to the reconstruction of the chest wall following resection. During the last 10 yers, we have performed reconstructive operation for 6 cases of the chest wall following resection of the sternum in recurrent cases of breast cancer or invaded case of primary breast cancer. In these patients, the chest wall was reconstructed using a rib-latissimus dorsi osteomyocutaneolus flap or a latissimus dorsi myocutaneous flap. The sternum was totally resected in 3 cases, and in all 3 cases, reconstructed using a rib-latissimus dorsi osteomyocutaneous flap. Although postoperative pulmonary function decreased, all cases could be relieved from endotracheal intubation within 17 hours aftr operation, and had no problems in activities of daily living or occurrence of chest flailing or paradoxical movement of the chest. An artificial material (expanded polytetrafluoroethlene patch) was used in only one patient for the reconstruction of the osseous thorax, but this case developed infection during postoperative chemotherapy. After this experience, we used only biological materials for the reconstruction of the chest wall and postoperatively performed radiotherapy and/or chemotherapy on all cases. We have observed no flap infection or detachment since then. One characteristic of using the latissimus dorsi myocutaneous flap is that it is easily elevated and rarely causes serious postoperative esthetic or functional problems. The flap is also easily utilized to reinforce the osseous thorax because ribs immediately below the latissimus dorsi muscle are readily mobilized as a pedicle graft. Reconstruction of the chest wall following resection of the sternum, described in this report, allowed us to perform radiotherapy and/or chemotherapy without serious postoperative complications on the cases relapsing after treatment of breast cance. The 2-year survival rate is 50% and one of these cases survived up to 10 years after resection of the sternum. Thus we prefer to perform resection of the sternum for sternal recurrence of breast cancerif there are no metastatic lesions in other organs.     

Bacterial eradication from root dentine by ultrasonic irrigation with sodium hypochlorite

The study aimed to evaluate intracanal irrigation procedures in eradicating bacteria from surface, shallow and deep layers of root dentine using extracted human teeth. Artificial bacterial smear layer was successfully produced by rubbing a mixture of dental plaque and artificially decalcified dentine or carious dentine on root canal walls. The reservoir holes were 3.5 mm in depth, 1 mm in diameter prepared 1.5 mm apart and parallel to the root canals on the decrowned planes, in which five separate bacterial species were placed ( Actinomyces israelii, Fusobacterium nucleatum, Propionibacterium acnes, Streptococcus mutans and Streptococcus sanguis ). Bacterial eradication after irrigation of the prepared canals was determined by bacterial recovery (i) from the root canal surfaces and shallow layers where bacteria were smeared artificially and (ii) from deeper layers of root canal dentine reservoir holes.
Ultrasonic irrigation with 5.5% and 12% NaOCl eradicated bacteria from artificial smear layer ( P <0.0001), whilst 12% NaOCl irrigation with a syringe was insufficient. Ultrasonic irrigation with water or 15% EDTA failed to eradicate bacteria from smeared surfaces. Ultrasonic irrigation with 12% NaOCl killed A. israelii, F. nucleatum, P. acnes, S. mutans , and S. sanguis placed in reservoir channels, although for F. nucleatum , a very small number of bacteria remained in five samples out of 12. Ultrasonic irrigation with less concentrated NaOCl failed to eliminate bacteria completely from reservoir channels in most samples. Ultrasonic irrigation with 12% NaOCl appeared to eliminate bacteria efficiently from surface, shallow and deep layers of root dentine.     

Restriction fragment-length polymorphism analysis of 16S rDNA from oral asaccharolytic Eubacterium species amplified by polymerase chain reaction

Restriction fragment-length polymorphism (RFLP) analysis of 16S rDNA amplified by polymerase chain reaction was used to generate restriction profiles of the type strains of oral asaccharolytic Eubacterium species, that is, Eubacterium brachy, Eubacterium exiguum, Eubacterium lentum, Eubacterium minutum, Eubacterium nodatum, Eubacterium saphenum, Eubacterium timidum and 33 asaccharolytic Eubacterium strains isolated from oral sites. The 16S rRNA gene sequences from isolated genomic DNA samples were amplified by polymerase chain reaction (PCR). PCR products were purified and characterized by single digestions with 7 restriction endonucleases. Among the 7 endonucleases, Hpall was found to discriminate the respective reference strains. Twenty-three isolates, out of 33, were assigned to one of the reference species, on the basis of their restriction profiles by digestion with Hpall. The remaining 10 isolates could not be assigned to any of the established species and constituted 4 distinct groups, each of which may be a new species.