Yoshie Hashida

Conduction tissue anomalies in absence of the right superior caval vein

The incidence of electrical instability of the heart is high in patients with absence of the right and persistence of the left superior caval vein when the latter connects to the coronary sinus. It has been suggested that a large coronary sinus may influence the susceptibility to arrhythmias. we studied the conduction tissues of 8 hearts from the cardiopathological collection of Childrens Hospital of Pittsburgh. Six of these specimens had a persistent left superior caval vein connecting to the coronary sinus. There were 4 with absence of the right superior caval vein, 3 of which were in the group with persistent left veins. We evaluated the sinus node and the specialized atrioventricular junctional area, comparing them with known normals. The coronary sinus varied in size from through it. The size did not alter the histology of the adjacent conduction tissue. In contrast, the sinus node was abnormal in 3 of the 4 hearts with absent right superior caval vein. This may be the key factor in the development of arrhythmias when the right superior caval vein is absent or abnormal.

Temporal bone histopathologic findings of Waardenburg's syndrome: A case report

A histopathological study of the temporal bones of a 3‐year‐old black girl who had bilateral deafness associated with Waardenburgs syndrome type II showed a similar pattern of pathology in both ears. The most striking findings were an absence of pigmentation in the inner ear and cochleosaccular abnormality. This is, to our knowledge, only the third report on human temporal bone histopathology in Waardenburgs syndrome and the first report of such a case with absence of pigment (melanin) in the inner ear. A possible association of hearing loss with absence of inner ear pigment in this case is discussed.

Renal Histopathology in Alagille's Syndrome

Various renal abnormalities have been reported in Alagilles syndrome (arteriohepatic dysplasia), usually as single case reports. The renal findings at autopsy of four patients with Alagilles syndrome, ranging in age from 4 1/2 months to 7 years, 2 of whom had evidence of renal dysfunction, are described and are compared with kidneys from patients with other cholestatic liver diseases of childhood. Two of the Alagilles patients had histologic findings suggestive of membranous nephropathy and special stains revealed accumulation of lipid in the glomerular and tubular basement membranes. Immunofluorescence of 1 revealed extensive accumulation of IgG and IgM. One patient had medullary cysts and mild interstitial fibrosis, and the fourth had a large subcapsular cyst and mild tubulointerstitial nephritis. All 4 cases, when examined with the electron microscope, revealed varying degrees of basement membrane thickening, splitting, and vacuolation with dense osmiophilic particles, most prominent in the patients with membranous nephropathy. These ultrastructural findings did not correlate with the degree of hyperlipidemia, but rather with the patients age, and were also observed in other cholestatic diseases. The findings suggest that Alagilles syndrome is frequently associated with renal abnormalities, including lipid deposition, which may in some instances, lead to clinically significant renal impairment.

Pancreatic Pathology in Trisomy 13: Specificity of the Morphologic Lesion

Pancreases from 8 patients with trisomy 13 were studied. Ectopic splenic tissue was present in 6: 3 had accessory spleens and ectopic splenic tissue embedded in the pancreas, one had intrapancreatic splenic tissue without an accessory spleen, and one had an accessory spleen without intrapancreatic splenic tissue. The intrapancreatic splenic tissue was multiple, generally poorly or nonencapsulated, and contained pancreatic acini, islets of Langerhans, and ducts lined by tall columnar epithelium with goblet cells. The rest of the pancreas in these and other cases of trisomy 13 had microcyst formation and focal proliferations of small ducts similar to those seen in the splenic tissue. This constellation of features appears to be distinctive of trisomy 13.

Histopathology of Otitis Media in Infants with Cleft and High-Arched Palates

The middle ear and the bony and cartilaginous portions of the eustachian tube (ET) were studied histopathologically in 20 temporal bones from 19 infants, aged 45 minutes to 12 months. Otitis media (OM) was observed in 17 of 20 bones, and 16 bones with OM had an effusion. Otitis media was more severe in the ears of cleft palate infants compared to those with high-arched palates. Inflammation was present in the bony portion of the ET in 15 of 17 temporal bones with OM, but appeared to be less marked than that present in the middle ear. In addition, inflammation of the cartilaginous portion of the ET appeared to be less severe than in its bony portion. Inflammation of the middle ear and the ET appeared to be more pronounced in older infants, particularly in those with cleft palates.

Ultrastructure of the adrenal cortex in Cushing's disease in children

Abstract This paper describes the ultrastructure of the adrenal cortex in two children with Cushings disease. The main ultrastructural changes were observed in the zonae fasciculata and reticularis. The hyperplastic cells seen by light microscopy in diffuse hyperplasia of the adrenal cortex of Cushings disease appeared as electron light cells at the ultrastructural level. Their appearance resulted from dilatation and hyperplasia of the smooth-surfaced endoplasmic reticulum and a decrease in the size of lipid globules. Mitochondrial changes included two types of mitochondria, increased electron density of the matrix, outpouching of the outer membrane, intramitochondrial dense bodies, and tubular cristae. Our observations tend to agree with the findings of others in experimental animals and to differ with a previous report on Cushings disease.

A staged laryngotracheal reconstruction using alloplast (Proplast) in the canine model

Standard laryngotracheal reconstructive procedures are constrained by the availability of grafting materials and the ability to repair large defects. Reconstruction utilizing alloplasts may be ideal, but previous results have generally been poor. We present a two-stage procedure using Proplast to reconstruct large laryngotracheal defects in 6 dogs. A custom-made Proplast implant was inserted into a strap muscle pocket and buccal mucosa was placed in the adjacent peritracheal tissue. The muscle-Proplast composite graft was rotated to repair a large defect (3.25 cm by 2.25 cm). All 6 dogs survived. Endoscopic and histologic studies over 12 months showed continued stabilization and maturation of the implant. We feel that Proplast may be used as an alternative to autograft for reconstructing large laryngotracheal defects when the procedure is staged to allow maximal fibrous ingrowth and the implant is protected from infection.

Experimental Otitis Media Evaluated by Magnetic Resonance Imaging: An in Vivo Model

The use of magnetic resonance imaging in otitis media research is being explored in our laboratory. In this study, we present a new method for studying changes in the middle ear cleft due to an episode of induced otitis media in the chinchilla model. It uses gadolinium-diethylenetriamine pentaacetic acid, a magnetic resonance imaging contrast agent, to examine the uptake and washout characteristics of middle ear mucosa during an inflammatory episode. Parameters such as the time to maximum intensity of the mucosa and the washout rate of the contrast agent from the mucosa were significantly correlated to the duration of the infection.

Histopathologic study of otitis media in individuals with head and neck tumors.

Five temporal bones, each including the eustachian tube, were obtained from five adults with advanced malignant tumors of the head and neck. The specimens were from the side on which the tumor had occurred. Otitis media had been detected clinically in two cases, and was detected histopathologically in the other three. We discuss the possibility that otitis media might have been caused by tumor invasion of the paratubal area, by postoperative inflammation in the nasopharynx, or by an inflammatory reaction of tubal structures to radiotherapy, or that these conditions may have coexisted.

Congenital alopecia, seizures, and psychomotor retardation in three siblings

Three siblings, devoid of hair at birth, had an unusual autosomal recessive disorder characterized by universal congenital alopecia, microcephaly, seizures, psychomotor retardation, and severe growth failure. Metabolic and chromosome studies were normal. Skin biopsies disclosed immature hair follicles, some of which were filled with keratotic material but had no hair shafts. Neuropathologic features included cerebral cortical hypoplasia, neuronal depletion, and microcalcifications. The familial occurrence of universal congenital alopecia conjoined with nonprogressive central nervous system abnormalities in this and other kindreds defines a nosologic group of neurocutaneous disorders in which congenital alopecia is the solitary cutaneous manifestation.