A case of systemic lupus erythematosus in a 13-year-old girl]

The test with running on a treadbane showed a 56% increase of working capacity in the control group of male albino mice on the 20th day of training. Oral administration of elton, leveton, phytoton, and adapton, as well as Leuzea and Rhodiola extracts and Schisandra chinensis tincture caused a statistically significant increase in the time of running on the treadbane of animals by the 10th day of medication. The increase in the working capacity of the animals was more marked by the 20th day. In the test of swimming "to the limit" adapton, phytoton, leveton, and elton increased to a greater extent the working capacity of male albino rats in diminishing succession (from 213 to 168%). Schisandra tincture and Rhodiola and Leuzea extracts also increased the swimming time of the animals by 135-159%.     

Slide tracheoplasty: a case report of successful concomitant reconstruction of extensive congenital tracheal stenosis and pulmonary artery sling

An 8-month-old infant presented with an extensive congenital tracheal stenosis with an aberrant left pulmonary artery. The patient was treated successfully by relocation of the left pulmonary artery and tracheal reconstruction with slide tracheoplasty. This patient is the first survivor cited in the literature after concomitant repair using slide tracheoplasty.     

Pulmonary endodermal tumor resembling fetal lung: report of a case in a 14-year-old girl

This report describes the clinical and histologic features of a pulmonary tumor in a 14-year-old girl that is most consistent with a rare entity described in the literature as "pulmonary endodermal tumor resembling fetal lung" (PET). This tumor is composed of glycogen-rich columnar cells forming complex glands with focal festooning and mitotic activity, admixed with solid "morules" of cells with eosinophilic cytoplasm and focal nuclear clearing. Patchy tumor necrosis and a bland stroma were also present. Immunoreactivity for carcinoembryonic antigen (CEA), alpha 1-antichymotrypsin, and 12E7 was present in glandular cells and for human chorionic gondatropin (HCG), alpha 1-antichymotrypsin, and 12E7 in morular cells. Ultrastructural features are those of an epithelial tumor. Related entities have been called "pulmonary blastoma lacking sarcomatous elements" and "adenocarcinoma of fetal lung type." Most cases of PET have occurred in adults, and the histologic features thought to have prognostic significance in small published series are applied to our case, in which the patient remains well and without evidence of tumor recurrence or metastasis for 28 months following local resection as the sole treatment.     

A successful surgical repair of congenital mitral stenosis due to commissural papillary muscle fusion]

A case of congenital mitral stenosis, patent ductus arteriosus, pulmonary hypertension was reported. At one year of age, the patient underwent surgical division of PDA because of persistent left heart failure. She went well after the operation. At seven year of age, she was readmitted to our hospital for easy fatigability. The cardiac catheterization revealed remarkably elevated pulmonary arterial pressure and pulmonary capillary wedged pressure. She underwent a surgical intervention for the mitral stenosis at eight year of age. At operation, the mitral valve exhibited the characteristics of type IIc according to Carpentier's classification: thickened and dysplastic leaflet, extremely short chordae tendanae fused with papillary muscles, obliteration of interchordal space and hypertrophic two papillary muscles. We replaced the valve with 23 mm Carbomedicus prosthetic valve because it seemed to be difficult to repair the native valve satisfactorily without residual stenosis or insufficiency. Her postoperative course was uneventful and the pulmonary arterial pressure and pulmonary capillary wedged pressure decreased remarkably one year after the operation.     

Severe stenosis of the right coronary artery in a 15-year-old girl with type IIa hypercholesterolemia: successful treatment with stent implantation

Coronary artery stenosis with need for therapy is rarely seen in childhood. A 15-year-old girl with hypercholesterinaemia type II a was undergoing lipid aphereses therapy (once or twice a week) since she was 6 years old. The girl was seen in our hospital with stenocardia and depression of the ST-segment in the inferior ECG leads at rest. Myocardial scintigraphy with technetium 99 showed an ischemia of the infero-lateral left ventricular myocardium. During selective coronary angiography a 90% stenosis of the proximal right coronary artery over a distance of approximately 5 mm close to the ostium was found. Post stenotic dilatation of the vessel was obvious. In addition a diffuse 10% stenosis in the proximal and middle part of the right coronary artery was found. The left coronary artery appeared angiographically normal. After balloon dilatation, stent implantation was performed without complications with a 6 mm microstent. Reduction of the stenosis from 90% to 40% could be achieved. Ticlopidin 2 x 250 mg was started for thrombocyte aggregation inhibition. During the following 12 months the patient has been free of symptoms. Lipidaphareses has been continued as before. Stent implantation seems to be a successful treatment for coronary artery stenosis also in young patients.     

A successful repair of infective endocarditis accompanied by a pseudoaneurysm of the left ventricle

The present paper reports a successful surgical treatment of a 47-year-old male with a pseudoaneurysm of the left ventricle. The patient has also been administered Penicillin G for 5 months to treat endocarditis. Cardiac catheterization showed severe aortic stenosis and a pseudoaneurysm of the left ventricle which was dilating in systole. The patient underwent patch closure of the pseudoaneurysm whose ostium was situated at the miral-aortic inter valvular fibrosa followed by aortic valve replacement and direct closure of a right Valsalva sinus aneurysm. His postoperative course was uneventful. The patient had no recurrence of endocarditis nor malfunction of the prosthetic valve for one year postsurgery. This is the first report in Japan of successful surgical treatment of a pseudoaneurysm of the left ventricle due to perforation of the miral-aortic intervalvular fibrosa after endocarditis.     

A case of pulmonary mesenchymal hamartoma in a 9-year-old child

A 14 years old patient, diagnosed in early childhood of type 1 Gaucher's disease is reported to show his unusual genotype and clinical course. The patient is heterozygous for mutation D409H, that in the homozygous patient determines an aggressive disease accompanied by neurological signs, and for a new mutation, which must be considered of mild effects. Early detection of bone involvement is emphasized as a factor to consider when indicating of enzyme replacement therapy.     

Protruded lumbar intervertebral nucleus pulposus in a 12-year-old girl who recovered after nonsurgical treatment: a follow-up case report

Restless legs syndrome affects millions, but most healthcare providers don't know it exists. Its sleep-deprived victims are plagued by indescribable crawling sensations in their legs that occur at night and that are only relieved by moving the legs. Many home healthcare clients, already challenged by their primary illness, are also affected by the persistent symptoms of Restless Legs Syndrome. It is important that home care nurses learn about Restless Legs Syndrome-what it is, how to assess it, and what can be done about it.     

Malignant melanoma in a 7-year-old child. A report of a dramatic case

Malignant melanoma developed on the scalp at the site of a congenital nevus in a 7-year-old girl. At birth, simple observation was proposed, despite the recognized desirable but non-urgent indication for excision, in order to avoid subjecting the infant to general anesthesia. At the first sign of a change in the macroscopic appearance of the nevus, surgical resection was performed, but the short-term outcome was fatal.... This case focuses our attention on malignant progression of even small congenital nevi and emphasizes the need for early preventive and systematic resection even when the patient's age of the patient requires general anesthesia.     

Mechanisms of acute eosinophilic inflammation in a case of acute eosinophilic pneumonia in a 14-year-old girl

The alpha 1-, alpha 2-, alpha 3-, and alpha 4-tubulin genes have been mapped by in situ hybridization to the polytene chromosomes of five species representative of the Drosophila montium subgroup geographical distribution. A lambda phage clone containing alpha 1-tubulin specific sequences was isolated from a genomic DNA library of Drosophila auraria and its restriction endonuclease pattern is presented. Both well-characterized heterologous and homologous probes were used to assess orthogonality of gene members between species groups. The in situ hybridization pattern observed in all species studied is consistent with that of Drosophila melanogaster, since alpha 1-, alpha 2-, and alpha 3-tubulin genes are located on the same polytene arm, and the alpha 4-tubulin gene is found on a different arm. Cross-hybridization was observed among alpha 1-, alpha 2-, and alpha 3-tubulin specific sequences in all species studied, using either heterologous or homologous probes. However, unlike D. melanogaster, in all montium species studied, both alpha 1- and alpha 3-tubulin specific probes hybridize to the same polytene band, indicating a clustered organization of the above genes. The chromosomal organization of this gene family would suggest that taxa within the montium subgroup are closer to their common ancestors than are the taxa in the melanogaster species group. A mode of evolution for this gene family in Drosophila is proposed.     

A case of successful resection of pulmonary arteriovenous fistula with a high shunt ratio

We report on a 25-year-old cyanotic man who was diagnosed as having a pulmonary arteriovenous fistula. His chief complaint had been shortness of breath since childhood. Polycythemia (Hb 21.4 g/dl) was detected during a health checkup at his company. A chest X-ray showed an abnormal mass in the left lung. Blood gas analysis showed severe hypoxia with PaO2 of 38.6 mmHg at room air. Angio-CT showed a large aneurysmal lesion at S6 of the left lung with a large feeding artery and vein. Oxygen saturation was 75.2% in the radial artery and 62.5% in the right atrium. The right-to-left shunt ratio was therefore calculated as 62%. The aneurysmal lesion was resected by segmentectomy of the left S6 following division of A6 and V6. After a successful operation, the patient no longer had shortness of breath or cyanosis and blood gas analysis showed PaO2 as 84.3 mmHg at room air. Pulmonary angiography showed no residual shunt lesion.     

Metastatic retroperitoneal paraganglioma in a 16-year-old girl. Case report, molecular pathological and cytogenetic findings

Hypoxic-ischemic encephalopathy is still a very important cause of neonatal mortality and morbidity. Recently, platelet-activating factor (PAF) has been accused of being responsible for the neuronal damage in hypoxic-ischemic brain. We investigated tissue PAF concentrations in hypoxic-ischemic brain injury in immature rats. Endogenous PAF concentration in brain tissue showed a marked increase in hypoxic-ischemic pups (85.6 +/- 15.5 pg/mg protein) when compared to that of control (9.05 +/- 3.1 pg/mg protein). In addition, we examined the effects of flunarizine, a selective calcium channel blocker, and Ginkgo biloba extract (EGb 761) on endogenous PAF concentration in hypoxic-ischemic brain injury. Endogenous PAF concentrations in both flunarizine-pretreated (16.6 +/- 4.8 pg/mg protein) and EGb 761-pretreated (33.5 +/- 8.9 pg/mg protein) pups were significantly lower than the untreated group. These results indicate that PAF is an important mediator in immature rat model of cerebral hypoxic-ischemic injury. The suppressor effect of flunarizine and EGb 761 on PAF production may open new insight into the treatment of hypoxic-ischemic brain injury.     

CT and MRI in a girl with late-onset ornithine transcarbamylase deficiency: case report

We report CT and MRI findings in a girl with late-onset ornithine transcarbamylase deficiency, who presented with progressive somnolence. Both imaging methods showed signs of an acute cerebral ischaemia with new defects on follow-up. Despite an unusual clinical presentation, laboratory studies led to the diagnosis of this rare inherited metabolic defect.     

Pulmonary atresia with intact ventricular septum: long-term results of "one and a half ventricular repair"

BACKGROUND: Between 1982 and 1984, we successfully performed "one and a half ventricular repair" using a Glenn shunt for 3 patients with pulmonary atresia with intact ventricular septum. Here we review the 10-year follow-up results. METHODS: In these patients, the preoperative Z scores of the tricuspid valve diameters ranged from -5.2 to -6.5. Right ventricular outflow tract reconstruction combined with a Glenn shunt were performed in all patients. Cardiac catheterization was done at least 10 years post-operatively. RESULTS: All 3 patients have maintained New York Heart Association functional class I status for more than 10 years. Angiography in 2 patients confirms sufficient left pulmonary artery pressure with pulsatile blood flow and good right ventricular contraction. A pulmonary arteriovenous fistula has developed in 1 patient. CONCLUSIONS: Although the lower limits of the tricuspid valve diameter for "one and a half ventricular repair" using a cavopulmonary shunt have not yet been determined, we successfully performed this procedure in 3 patients with severely hypoplastic right ventricles and tricuspid valve diameter Z scores of less than -5.0. The results up to 10 years postoperatively are acceptable.