Congenital Malformations in Newborns from Diabetic Mothers*

It has been well known that there is a high incidence of congenital malformations in newborns from diabetic mothers when the mothers' diabetes control before and during pregnancy is poor. We treated 438 pregnant diabetics who bore 443 children between February 1964 and June 1992. Among these children, there were 51 cases (11.5%) with congenital malformations, 21 cases with major anomalies (4.7%) and 30 cases with minor anomalies (6.8%). The type of malformations are not related to special organs; heart malformations and cleft lips are relatively frequent compared to other types of malformations. The mechanism of the congenital malformations in newborns from diabetic mothers remains unclear. However, clinically and experimentally it has been found to be due to fuel-mediated teratogenesis. Since October 1978, HbAi has been used as an index of diabetic control and the relationship between congenital malformations and the mother's diabetes control has been observed. 1) There is no difference in the incidence of malformations in children from IDDM and NIDDM mothers. However, there are more severe malformations in the children from IDDM mothers compared to those from NIDDM mothers. 2) Mothers who bore children with major malformations had all made their first visit to our hospital after pregnancy. HbAi in the IDDM mothers who had children with malformations at the first visit was 11%. 3) In the NIDDM mothers, even if HbAi levels are near normal, children with major malformations were born and there was little relationship between congenital malformations and the mothers' diabetes control. These data suggest that there are two kinds of congenital malformations in children from diabetic mothers, fuel-mediated teratogenesis, and malformations as seen in children from non-diabetic mothers.     

Pulmonary thromboembolism in a patient with idiopathic hypereosinophilic syndrome

We report a successful treatment in a patient with idiopathic hypereosinophilic syndrome (HES) presenting with left pulmonary truncal thromboembolism and right pleural effusion. A treatment with urokinase infusion by a Swan-Gants catheter near a left pulmonary thrombus was performed and left pulmonary arterial occlusions were recanalized completely. After corticosteroid therapy was started, right pleural effusion disappeared. Both anticoagulant and corticosteroid therapy was necessary for treatment in HES patients who had thromboembolic episodes.     

Reconstruction of a natural-appearing umbilicus using an island flap: case report.

A case of successful reconstruction of the umbilicus using a depressed scar in an 11-year-old girl with a large and rigid scar spreading around the centre of the abdominal wall is presented. The anatomical umbilicus was replaced by a large scar after a previous surgery for umbilical cord hernia. The difficulty of the conventional method compelled us to use a distant scar dimple as an island flap. In addition, the three-dimensional morphology of the umbilicus could be reproduced by rolling a spindle island flap into a cone. Postoperatively, the reconstructed umbilicus has remained deep and aesthetically well shaped.     

Self‐reported seafood intake and atopy in Japanese school‐aged children

Background: The effects of fish consumption and n‐3 poly‐unsaturated fatty acid (PUFA) levels on atopic disorders are inconsistent in previous reports, but few studies have investigated the effects of both fish and n‐3 PUFA. The aim of the present study was to investigate whether erythrocyte fatty acids and the consumption of fish are associated with atopic diseases in pre‐ and early adolescents. Methods: A total of 135 students with eczema, 136 students with asthma, and 137 healthy control students were selected from fifth and eighth grades in Shunan, Japan. Atopic disorders and dietary intake were evaluated with questionnaires, and total serum IgE was measured using an enzyme‐linked immunosorbent assay. In addition, erythrocyte membrane levels of PUFA were assessed via gas chromatography. Results: Total IgE was significantly elevated in the atopic subjects (P < 0.001). The intake of fatty and dried fish or seafood was significantly associated with eczema (odds ratios of the highest quartiles: 0.46, 95% confidence interval (95%CI): 0.22–0.94; 0.34, 95%CI: 0.16–0.71, respectively). Additionally, only erythrocyte eicosapentaenoic acid (EPA) level had a negative association with eczema (P= 0.048). For asthma, the effect of fish consumption was not significant. Conclusions: Fish consumption was related to a low prevalence of eczema, but not asthma in Japanese pre‐ and early adolescents. EPA may be involved in this mechanism.     

A role for primary cilia in glutamatergic synaptic integration of adult-born neurons

The sequential synaptic integration of adult-born neurons has been widely examined in rodents, but the mechanisms regulating the integration remain largely unknown. The primary cilium, a microtubule-based signaling center, is essential for vertebrate development, including the development of the CNS. We examined the assembly and function of the primary cilium in the synaptic integration of adult-born mouse hippocampal neurons. Primary cilia were absent in young adult-born neurons, but assembled precisely at the stage when newborn neurons approach their final destination, further extend dendrites and form synapses with entorhinal cortical projections. Conditional deletion of cilia from adult-born neurons induced severe defects in dendritic refinement and synapse formation. Deletion of primary cilia led to enhanced Wnt and β-catenin signaling, which may account for these developmental defects. Taken together, our findings identify the assembly of primary cilia as a critical regulatory event in the dendritic refinement and synaptic integration of adult-born neurons.     

Keratomycosis caused by Aspergillus viridinutans: an Aspergillus fumigatus-resembling mold presenting distinct clinical and antifungal susceptibility patterns

We report here a case of fungal keratitis caused by Aspergillus viridinutans, a member of the genus which was found to have clinical and antifungal susceptibility characteristics distinct from the species it most closely resembles, Aspergillus fumigatus. A 26-year-old woman presented with contact lens-associated keratitis, the etiologic agent of which was initially slow growing and found to be resistant to amphotericin B and voriconazole. Therapeutic keratoplasty was performed and an isolate from a corneal scraping was identified as A. viridinutans through partial DNA sequencing of the β-tubulin gene, along with its slow growth 42°C. In contrast with A. fumigatus, A. viridinutans proved to be resistant to a wider variety of antifungal agents. To the best of our knowledge, this is the first case of keratitis caused by A. viridinutans. Clinical isolates of the genus Aspergillus include rarely encountered species such as A. viridinutans. These species may show clinical manifestations distinct from those of A. fumigatus, including clinical resistance to amphotericin B and voriconazole and relatively low susceptibility to other antifungal agents.     

Effects of age and inactivity due to prolonged bed rest on atrophy of trunk muscles

This study investigated the effects of age and inactivity due to being chronically bedridden on atrophy of trunk muscles. The subjects comprised 33 young women (young group) and 41 elderly women who resided in nursing homes or chronic care institutions. The elderly subjects were divided into two groups: independent elderly group who were able to perform activities of daily living involving walking independently (n = 28) and dependent elderly group who were chronically bedridden (n = 13). The thickness of the following six trunk muscles was measured by B-mode ultrasound: the rectus abdominis, external oblique, internal oblique, transversus abdominis, thoracic erector spinae (longissimus) and lumbar multifidus muscles. All muscles except for the transversus abdominis and lumbar multifidus muscles were significantly thinner in the independent elderly group compared with those in the young group. The thicknesses of all muscles in the dependent elderly group was significantly smaller than that in the young group, whereas there were no differences between the dependent elderly and independent elderly groups in the muscle thicknesses of the rectus abdominis and internal oblique muscles. In conclusion, our results suggest that: (1) age-related atrophy compared with young women was less in the deep antigravity trunk muscles than the superficial muscles in the independent elderly women; (2) atrophy associated with chronic bed rest was more marked in the antigravity muscles, such as the back and transversus abdominis.     

Theory of mind impairment in adult-onset myotonic dystrophy type 1

Patients with myotonic dystrophy type 1 (DM 1) exhibit behavioral disorders and distinctive personality traits. We hypothesize that this is due to altered social cognitive function. To investigate the cognitive basis of this impairment in DM 1 patients, we examined their theory of mind (ToM) ability, which is the specific cognitive ability to understand the mental states of others. Nine adult-onset DM 1 patients (4 men and 5 women) performed 2 ToM tasks: "Reading the Mind in the Eyes" test and faux pas recognition test. DM 1 patients were found to be impaired in both ToM tests, but had no difficulty with control tasks designed to test visual and lexical comprehension. The present results indicate that social cognitive impairment in patients with adult-onset DM 1 is associated with ToM dysfunction, which could be due to the brain lesions associated with this disease.     

Identification of cytomegalovirus and human herpesvirus-6 DNA in a patient with corneal endotheliitis

Purpose

To report the case of a patient with unilateral corneal endotheliitis in which both cytomegalovirus (CMV) and human herpesvirus-6 (HHV6) DNA was identified in the aqueous humor.

Case

A 67-year-old man with corneal endotheliitis OD was referred to us for decreased visual acuity. Local corneal stromal edema, pigmented keratic precipitates, a coin-shaped lesion and minimal anterior chamber reaction were observed by slit-lamp biomicroscopy. Cells with owl’s eye appearance in the endothelial cell layer were observed by in vivo laser confocal microscopy. The patient had rheumatoid arthritis, which was treated by oral prednisolone and intravenous abatacept. Polymerase chain reaction analysis of aqueous humor samples detected both CMV and HHV6 DNA, but not other HHVs. Treatment with topical ganciclovir and systemic valganciclovir resulted in a clear cornea.

Conclusions

A patient with corneal endotheliitis had both CMV and HHV6 DNA identified in the aqueous humor. Although both viruses were identified in this case, clinical manifestations resembled CMV corneal endotheliitis, and it was unclear whether HHV6 could affect the clinical course. Systemic abatacept and corticosteroid therapy might play a positive role in cases with both CMV and HHV6 DNA in this corneal endotheliitis.     

Clinicopathological features of 171 cases of primary thyroid lymphoma: a long-term study involving 24553 patients with Hashimoto's disease

There are few large‐scale reports of primary thyroid lymphoma (PTL). This study clinically and pathologically reviewed 171 patients with PTL and 24 553 patients with Hashimoto’s disease at Ito Hospital between January 1990 and December 2004, to investigate the clinical features and the treatment outcomes of PTL. The median age of the patients with PTL was 67 years (range, 27–90 years). The pathological diagnosis of PTL patients included diffuse large B‐cell lymphoma (DLBCL) (n = 74), DLBCL with mucosa‐associated lymphoid tissue (MALT) lymphoma (n = 13), MALT lymphoma (n = 80) and others (n = 4). Of the 167 patients with B‐cell lymphoma, treatment included combined modality therapy (CMT) (n = 95), radiation therapy (RT) alone (n = 60) and chemotherapy alone (n = 6). Information on treatment was not available in six patients. Information on treatment response was available in 154 patients; 149 patients (97%) responded to treatment. According to the institutional treatment strategy of Ito Hospital, 45 of 54 patients with stage IE disease received RT alone, and 87 of 113 stage IIE patients received CMT. The 5‐year overall survival rate was 85% (95% confidence interval, 79–91%). This study demonstrated that PTL showed good response to radiotherapy and chemotherapy and had a favourable prognosis.