Ependymal changes in the human fetal brain.
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Biomedical subjects
Publications and source records attributed to J G Chi.
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Microvessels in the hippocampus of aged SAM-P/10 (14 months old) showed the following ultrastructural changes compared with those of young-mature controls (3 months old): (1) the majority of capillaries had lost the smooth contours typical of young cases; (2) the luminal surface of capillaries showed irregularity; (3) the endothelial cytoplasm was thicker; (4) vesicles appeared more frequently in the endothelium; (5) interendothelial tight junctions and basement membranes, however, seemed to show no significant abnormalities; (6) pericytes, especially those of arterioles and venules, contained many enlarged cytoplasmic inclusions with honeycomb-like vacuoles; (7) the area of glial perivascular end feet was greater. These morphological findings raise the possibility of impaired blood-brain barrier function and microhemodynamic disturbances in aged SAM-P/10 hippocampus.
A retroperitoneal teratoma was studied with CT and magnetic resonance (MR) imaging. The images were correlated with the pathologic findings. The tumor was composed of five portions: adipose tissue, bone, hair, sebum, and loose edematous fibrofatty tissue with skin. Computed tomography and MR both demonstrated a well defined mass with separate cystic and solid components and all five tissue elements of the tumor. Magnetic resonance was superior to CT in delineating the relationship of blood vessels to the tumor.
We present the MR findings of hepatic choriocarcinoma in a 2-week-old infant. Two masses of heterogeneous signal intensity were evident; one was an extensive mass on the left side of the liver and the other was a "daughter" nodule in the right lobe. Peripheral portions of the masses were hyperintense on T1-weighted imaging (T1WI) and T2-weighted imaging (T2WI) suggesting subacute hemorrhage. Central portions of the masses were hypointense on T1WI and bright on T2WI. T1-weighted postgadolinium imaging showed peripheral enhancement of both tumor nodules. The infant's mother had normal serum beta-human chorionic gonadotropin levels and pelvic CT.
We report CT and ultrasound (US) features of adenoma malignum of the uterine cervix in a patient with Peutz-Jeghers syndrome (PJS) in whom bilateral ovarian mucinous cystadenomas and sex cord tumors with annular tubules were associated. Adenoma malignum was shown as a hyperechoic mass mixed with multiple cysts on US and a low attenuated endocervical mass on CT. We think that imaging demonstration of an endocervical mass is important for the correct diagnosis of adenoma malignum in a female with PJS.
Cerebrocostomandibular syndrome is a potentially lethal developmental disorder characterized by mental handicap, palatal defects, micrognathia, and severe costovertebral defects. We report a 3-day-old male neonate who died of respiratory difficulty that began at birth. Micrognathia, glossoptosis, high-arched palate, and hypoplasia of the lower half of the face were present. Multiple posterior rib defects and a narrow rib cage were associated with pulmonary hypoplasia. The rib gaps were filled with fibrovascular tissue. A facial bone study showed multifocal growth retardation involving the septal cartilage, vomer, and mandibular condyle, indicative of maxillomandibular growth arrest. The tongue had an abnormal genioglossus muscle and papillae.
We report a 12-year-old girl who had pigmented villonodular synovitis with extensive bony erosion around the left sacroiliac joint. The lesion mimicked a primary bone tumor clinically and radiologically, but was a typical pigmented villonodular synovitis (PVNS). The sacroiliac joint is an unusual site for PVNS, especially in the pediatric age group.
We have studied the ultrastructural characteristics of the pigments in the macrophages of liver and lymph nodes from three children with chronic granulomatous disease (CGD). The pigments represent lipofuscin bodies and appear to be formed from lysosomes. Characteristic structures are believed to represent transitional stages between lysosomes and mature pigment granules. It is thought that the residual lipids undergo progressive oxidation secondary to deficient lipolytic activity, overloading the lysosomes. It was also noted that liver biopsy can be a valuable tool in confirming the diagnosis of CGD, particularly when the disease is clinically suspected but the results of the nitroblue tetrazolium test are equivocal.
Neonatal choriocarcinoma associated with a seemingly normal gestation is rare. A total of 20 cases of either primary or metastatic choriocarcinoma in infancy have been reported until 1992. We report an additional case of a huge choriocarcinoma of the liver in a 2-month-old boy, who died of tumor hemorrhage. This baby was born to a 19-year-old unmarried mother. The placenta was thought to be normal and was not examined histologically. The liver mass was first noted 2 weeks after birth, and he was admitted because of poor feeding and pallor. Before any therapy was instituted, he died of massive tumor bleeding. Autopsy revealed a huge hemorrhagic tumor mass with massive necrosis in the left lobe of the liver. Several nodular metastases were found only in the lung. The remainder was unremarkable except for bilateral cleft lip and palate. It is presumed that the choriocarcinoma in the liver could be either a primary tumor in the absence of any primary focus in the mother or the infant or a metastatic lesion from an occult choriocarcinoma of the placenta.
Oligomeganephronia is a rare congenital renal hypoplasia that is characterized histologically by a reduction in the number of reniculi with compensatory hypertrophy of the glomeruli and proximal renal tubules. Oligomeganephronia has generally been regarded as a congenital but not genetic disease. Kusuyama et al. first suggested that oligomeganephronia might be associated with a chromosomal anomaly, namely 4p monosomy syndrome. Their assumption originated from the fact that external anomalies of their cases of oligomeganephronia are very much like those described in 4p monosomy syndrome. We have experienced two autopsy cases of oligomeganephronia associated with multiple congenital anomalies that are seen in 4p deletion syndrome. Chromosome studies performed in both cases revealed 4p deletion and 4p ring, respectively. There were remarkable similarities between these cases. We suggest that there are two types of oligomeganephronia; one is a solitary sporadic type with no associated anomaly, and the other is a syndromic type that is a part of a complex anomaly of 4p deletion syndrome and possibly other related chromosomal deletion syndromes.
The authors present a retroperitoneal fetus in fetus in a 3-month-old girl. A 15-cm cystic mass with a monstrous fetuslike structure surrounded by a complete sac containing serous fluid was removed from the left retroperitoneal space. The draining vessel of the cystic mass was connected to the right renal vein of the host. The included fetus weighed 380 g. It had a well-developed umbilical cord, four extremities, head, buttock, and vertebral bodies with a meningomyelocele. The thoracic cavity of the included fetus had only a saclike foregut structure, but the abdominal cavity revealed a full length of intestine with a Meckel diverticulum, bilateral ovaries, urinary bladder, and cloaca with external opening. The cephalic end was composed of well-developed tooth germs, tongue and buccopharynx, mandible, maxilla, sphenoid bone, and salivary glands. Chromosomal study showed 46,XX with a normal G banding pattern. We report this case as an example of fetus in fetu with a complete umbilical cord and fetal membrane.
The hyaloid vascular system of developing human eyeballs is studied to elucidate the relationship to the retinal vascular patterns. The process of the regression of the hyaloid vascular system in fetuses consisted of gradual shrinkage of the wall of the vessels with lessening of the lumen and eventually, thread-like acellular strands that remained as a consequence. The intercapillary bridge in the retinal vessel appeared to be a remnant of the degenerated vessel as in the hyaloid vascular system. At the ninth month of intra-uterine life, the regression of the hyaloid vascular system was almost complete.
Choroid plexus papilloma (CPP) arising in the temporal horn is rare in adult population, and to the best of our knowledge, there has been no report of such a case with temporal lobe epilepsy (TLE). The authors describe a unique case of a 27-year-old woman who was diagnosed as TLE and was found to have a CPP in the temporal horn. Choroid plexus papilloma of the temporal horn, even though rare, can be found in adult population and be causally related to temporal lobe epilepsy.
Intestine is seldom a site of clinical manifestation of malignant hypertension, particularly in childhood. This report deals with a case of malignant nephrosclerosis superimposed on benign nephrosclerosis which probably resulted from a unilateral obstructive uropathy and chronic pyelonephritis. Clinical features included severe hypertension, neuroretinopathy with retinal exudate and hypertensive encephalopathy. An acute abdomen due to transmural infarction of the ileum caused by multiple thrombotic occlusion of necrotizing arteritis involving bowel wall and the mesentery was noted.