CYTOMEGALOVIRUS OOPHORITIS WITH CORTICAL NECROSIS DURING REMISSION OF ACUTE LYMPHOCYTIC LEUKEMIA

Ovarian involvement of cytomegalovirus (CMV) is rarely observed in autopsy and biopsy materials. Cortical necrosis of the ovaries was found in an autopsy case with generalized CMV infection. The patient was an 11-year-old girl in a remission state of acute lymphocytic leukemia. Autopsy revealed several areas showing necrotic change up to 2 mm in size in the cortex of both ovaries. Many cytomegalic cells were found in both the necrotic and intact areas of the cortex. CMV had infected the granulosa, thecal and stromal cells as well as vascular endothelial cells. Oocytes of neither primary nor graafian follicles showed cytomegalic changes, although they were destroyed due to the necrosis. CMV antigen was immunohistologically detected in these cytomegalic cells. Ultrastructurally, herpesvirus-type particles were revealed in the nuclei and cytoplasm of the cytomegalic cells. This case demonstrated that ovarian infection with CMV can potentially induce cortical necrosis and decrease the number of oocytes. ACTA PATHOL JPN 38 : 1069 ∼ 1076, 1988.     

Analysis of mesenteric thickening on computed tomography

Computed Tomography (CT) provides a noninvasive information in the evaluation of abnormalities of the gastrointestinal tract by direct imaging of the bowel wall and adjacent mesentery. Several prior studies have discussed the variable CT appearances of mesenteric abnormalities, such as lymphoma, metastasis, inflammatory disease and edema. Although mesenteric thickening was mentioned in these studies, no study has provided a detailed analysis of the CT appearance of the thickened mesentery. Two characteristic types of mesenteric thickening were identified in 47 patients. Type I is "Intra-mesenteric thickening", which was noted in 25 patients with vascular obstruction, inflammatory disease and edema. Type II is "Mesenteric surface thickening", which was noted in 22 patients with peritonitis carcinomatosa, peritoneal mesothelioma, tuberculous peritonitis and pseudomyxoma peritoneal. An understanding of these two types of mesenteric disease is important in the identification of mesenteric pathology.     

Enflurane reduces the excitation and inhibition of dorsal horn WDR neuronal activity induced by BK injection in spinal cats

The effects of enflurane (0.5%, 1.5% and 2.5%) on the excitation and inhibition of dorsal horn wide dynamic range (WDR) neuronal activity induced by bradykinin (BK) injection was studied in spinal cats. Extracellular activity was recorded in the dorsal horn from single WDR neurons responding to noxious and non-noxious stimuli applied to the cutaneous receptive fields on the left hind paw foot pads of decerebrate, spinal cord transected (L_1–2) cats. When 10µg of BK was injected into the femoral artery ipsilateral to the recording site as the noxious test stimulus, 24 of 26 WDR neurons (92%) gave excitatory responses and 2 (8%) gave inhibitory resposes. On the other hand, when the injection of 10µg of BK into the femoral artery contralateral to the recording site was used as the noxious test stimulus, 7 of 12 WDR neurons (58%) gave inhibitory responses, 3 (25%) gave excitatory responses, and 2 (17%) showed no response. The excitatory neuronal activity in WDR neurons was not depressed by 0.5% or 1.5% enflurane but was depressed significantly by 2.5%. However, the inhibitory neuronal activity in WDR neurons was significantly depressed by 0.5%, 1.5% and 2.5% enflurane. We have found that enflurane reduces the excitation as well as the inhibition of dorsal horn WDR neuronal activity induced by BK injection. These results suggest that the reduction of excitatory and inhibitory responses produced by noxious stimulation is likely to be the fundamental basis of the enflurane-induced anesthetic state in terms of WDR neurons.(Nagasaka H, Nakajima T, Takano Y et al.: Enflurane reduces the excitation and inhibition of dosal horn WDR neuronal activity induced by BK injection in spinal cats. J Anesth 4: 102–109, 1990)     

Comprehensive genetic screening for vascular Ehlers–Danlos syndrome through an amplification-based next-generation sequencing system

Vascular Ehlers–Danlos syndrome (vEDS) is a hereditary connective tissue disorder (HCTD) characterized by arterial dissection/aneurysm/rupture, sigmoid colon rupture, or uterine rupture. Diagnosis is confirmed by detecting heterozygous variants in COL3A1. This is the largest Asian case series and the first to apply an amplification-based next-generation sequencing through custom panels of causative genes for HCTDs, including a specific method of evaluating copy number variations. Among 429 patients with suspected HCTDs analyzed, 101 were suspected to have vEDS, and 33 of them (32.4%) were found to have COL3A1 variants. Two patients with a clinical diagnosis of Loeys–Dietz syndrome and/or familial thoracic aortic aneurysm and dissection were also found to have COL3A1 variants. Twenty cases (57.1%) had missense variants leading to glycine (Gly) substitutions in the triple helical domain, one (2.9%) had a missense variant leading to non-Gly substitution in this domain, eight (22.9%) had splice site alterations, three (8.6%) had nonsense variants, two (5.7%) had in-frame deletions, and one (2.9%) had a multi-exon deletion, including two deceased patients analyzed with formalin-fixed and paraffin-embedded samples. This is a clinically useful system to detect a wide spectrum of variants from various types of samples.     

A case of crossed fused kidney with simple ureterocele

A 32-year-old man consulted Osaka National hospital with chief complaints of dysuria and macrohematuria. DIP and CT revealed that the right kidney deviated to the lower pole of the left kidney and they fused together. The right ureter crossed over the supine. The calcified shadow existed in the lower end of the left ureter with cobra head image. He had no external anomalies. Under diagnosing crossed fused kidney (inverted L shaped) complicated the left ureterocele with a stone, transurethral incision of ureterocele (TUI) was performed. We made transverse incision and extracted stone, 7 mm in size (calcium oxalate 96% and calcium phosphate 4%). Three months later after the operation, IVP, CG and VCG revealed the down-sized ureterocele and no VUR. Crossed renal ectopia complicated many anomalies about 50%. Among them anomalies of the urinary tract was most frequent about 30%. But crossed renal ectopia with ureterocele wasn't reported so far in Japanese literature.     

The effect of midazolam on the memory during cesarean section and the modulation by flumazenil

In 30 patients (ASA-1) for elective Cesarean section who had given informed consent, characteristics of amnesia induced by midazolam (M) and their modulation by flumazenil (F) were examined. Spinal anesthesia was performed with dibucaine. After the delivery, the baby was shown to the mother. Then 21 patients were given bolus intravenous injection of M until the patient got into sleep and the inhalation of nitrous oxide mixed with oxygen was started. At the end of surgery bolus injection of F 0.1 mg was cumulatively repeated until the patient awoke. Nine patients were given only nitrous oxide and oxygen inhalation after the delivery. The remembrance of the baby face and the doses of M and F were compared. In group given M, 14 patients recalled their baby's face whereas 7 did not. The average doses of M and F in patients with memory were 69 micrograms.kg-1 and 2.5 micrograms.kg-1, respectively, whereas the average dose of M and F in patients without memory were 94 micrograms.kg-1 and 4.2 micrograms.kg-1, respectively. In the patients without M injection, all could recall the face of the baby. These results suggest that M could produce retrograde amnesia, when combined with nitrous oxide inhalation, which is not reversed by F.     

Novel chloride channel gene mutations in two unrelated Japanese families with Becker's autosomal recessive generalized myotonia

We investigated the skeletal muscle voltage-gated chloride channel gene (CLCN1) in two unrelated Japanese patients with Becker's myotonia congenita. The non-myotonic parents of each patient were consanguineous. The proband of each family shares generalized myotonia, transient weakness after rest, and leg muscle hypertrophy. However, the disease severity related to the degree of myotonia differed, even in view of the response to long train nerve stimulation tests. CLCN1 gene analysis revealed a novel Ala659Val missense mutation identified to be homozygous in the more severe patient, while a novel Gln445Stop nonsense mutation was present in the other patient. Both mutations were absent in 90 Japanese normal controls. This is the first report of Japanese cases of Becker's myotonia congenita with CLCN1 gene mutations.     

Severe stenosis of the internal carotid artery presenting as loss of consciousness due to the presence of a primitive hypoglossal artery: a case report

BACKGROUND: Symptoms of ischemic attacks in the internal carotid system usually involve focal cerebral dysfunction, i.e., hemiparesis or aphasia. However, an ischemic attack in the vertebrobasilar artery system usually presents with combined symptoms. The variety of manifestations included in the vertebrobasilar profile makes the potential pattern of symptoms considerably more variable and complex than that in the carotid system. Manifestations can include syncope and also vertigo. METHOD AND RESULTS: A 42-year-old woman experienced frequent attacks of faintness with vertigo. Angiography demonstrated severe stenosis of the left internal carotid artery with a persistent primitive hypoglossal artery just distal to the stenosis. The right internal carotid artery was normal and cross circulation through the anterior communicating artery was not well developed. Both vertebral arteries were hypoplastic. The patient underwent carotid endarterectomy and, thereafter the episodes of syncope completely disappeared. CONCLUSION: It was supposed that global ischemia including the brain stem occurred because of stenosis of the left internal carotid artery attributable to the presence of a primitive hypoglossal artery.