Melanotic schwannoma. An unusual case of an ossified soft tissue mass

A case of a melanotic schwannoma presenting as a soft tissue mass of the abductors of the hip is reported. The radiographic findings suggested myositis ossificans, but several subtle findings raised the concern for something else. A bilobed appearance, lack of classic zoning pattern of the ossification, and atypical pain pattern should alert the physician. Biopsy should be considered if the radiographic and clinical presentations are not classic for myositis ossificans.     

Development of a miniature undulation pump for the distributed artificial heart

Research of the distributed artificial heart is important not only to acquire the means of individual organ perfusion but also to clarify the characteristics of the organ and the mechanism of blood distribution. To investigate the distributed artificial heart, the miniature undulation pump was developed. The outer diameter and the thickness of the developed pump were 38 mm and 11 mm, respectively. The priming volume of the pump was 3.2 ml. The total size including the motor unit was 38 mm in diameter and 32 mm in length. The total weight was 67.5 g. The total volume was 27.5 ml. The pump was driven with pulse width modulation by using a 1 chip motor controller. More than 5 L/min of continuous output could be obtained. The results showed that the developed miniature undulation pump system had enough performance for individual organ perfusion.     

Mutational Analysis of Androgen Receptor (AR) Gene in 46,XY Patients with Ambiguous Genitalia and Normal Testosterone Secretion: Endocrinological Characteristics of Three Patients with AR Gene Mutations

The prevalence of abnormalities in androgen receptor gene (AR) among patients with ambiguous genitalia is unknown. Moreover, endocrinological data from prepubertal patients with AR mutation are very limited. Thus, the aim of this study was to examine the prevalence of abnormalities in AR among patients with both ambiguous genitalia, which was defined as a combination of two or more genital abnormalities (i.e. hypospadias, microphallus (penile length < 25 mm), hypoplastic scrotum, bifid scrotum, undescended testis) in this study, and normal to elevated T levels. We also compared the endocrinological data of prepubertal patients with AR mutation and ambiguous genitalia with that of those without the AR mutation. We screened 26 Japanese prepubertal 46,XY patients (five from three families were included) with both ambiguous genitalia and normal to elevated T levels. Mutations in AR were found in three (two of the three were related). Among the 23 patients without mutation in AR, the steroid 5-alpha-reductase 2 gene (SRD5A2) was also examined in eight patients with elevated T/dehydrotestosterone ratio after the hCG (>10) or with undervirilized family members. No mutation in SRD5A2 was found. Characteristics of the three patients with mutation in AR were compared with the 23 patients without mutation. In two patients, basal T levels (0.3, 0.2 ng/ml) and peak T levels after the hCG tests (8.3, 8.5 ng/ml) tended to be higher, and the peak LH/ peak FSH ratios after the GnRH tests (4.6, 4.0) were higher than in patients without mutation, at the ages of 1 yr and 9 mo and 3 yr and 8 mo, respectively. In conclusion, an abnormality in either AR or SRD5A2 was not common among patients with ambiguous genitalia and normal testosterone secretion. Elevated peak LH/peak FSH ratio (≥4) after the GnRH test in addition to detectable basal T levels and elevated peak T levels after the hCG test may infer AR abnormality in prepubertal patients with ambiguous genitalia at the age of one and over, although further study is needed, because our data were limited.     

PAX5 alterations in an infant case of KMT2A‐rearranged leukemia with lineage switch

Lineage switch is a rare event at leukemic relapse. While mostly known to occur in KMT2A‐rearranged infant leukemia, the underlying mechanism is yet to be depicted. This case report describes a female infant who achieved remission of KMT2A‐MLLT3‐rearranged acute monocytic leukemia, but 6 months thereafter, relapsed as KMT2A‐MLLT3‐rearranged acute lymphocytic leukemia. Whole exome sequencing of the bone marrow obtained pre‐post lineage switch revealed two somatic mutations of PAX5 in the relapse sample. These two PAX5 alterations were suggested to be loss of function, thus to have played the driver role in the lineage switch from acute monocytic leukemia to acute lymphocytic leukemia.     

An Analytical Model / Emotional Intelligence Quotient and QOL in Mothers with Infants in Japan

Objective: The purpose of this study was to examine the relationship betweenthe emotional intelligence quotient and health-related quality of life using structuralequation modeling.Methods: A self-administered questionnaire survey was conducted among 1,911mothers who visited the Health Center for an infant medical examination. A hypotheticalmodel was constructed using variables of the emotional intelligence quotient, socialsupport, coping, parenting stress, and perceived health competence.Result: There were a total of 1,104 valid responses (57.8%). Significantstandardized estimates were obtained, confirming the goodness of fit issues with themodel. The emotional intelligence quotient had a strong impact on physical andpsychological quality of life, and showed the greatest association with coping. This studydiffered from previous studies in that, due to the inclusion of social support andexplanatory variables in coping, an increase in coping strategies was more highlyassociated with emotional intelligence quotient levels than with social support.Conclusion: An enhanced emotional intelligence quotient should be considereda primary objective to promote the health of mothers with infant children.     

Retroperitoneal hemorrhage due to spontaneous rupture of adrenal myelolipoma

BACKGROUND: A very rare case of retroperitoneal bleeding due to spontaneous rupture of a large adrenal myelolipoma in a 62-year-old woman is reported. METHODS/RESULTS: She consulted the emergency room of the Nagano Red Cross Hospital with a complaint of sudden left flank pain. A computerized tomography (CT) scan revealed a tumor with areas of fat density and hematoma in the left retroperitoneal space. After her general condition improved, an operation was performed. The tumor strongly adhered to the left kidney and a left nephrectomy with the tumor was curative. Histologic diagnosis was adrenal myelolipoma. No blood transfusion was required. CONCLUSIONS: A CT scan is very useful in the pre-operative diagnosis of adrenal myelolipoma with retroperitoneal hemorrhage. Watch and wait treatments before operation and nephrectomy with adhered tumor are safe and curative.     

Changes of body balance before and after total knee arthroplasty in patients who suffered from bilateral knee osteoarthritis

Purpose

It is still controversial whether simultaneous or staged total knee arthroplasty (TKA) is most desirable for patients with bilateral knee osteoarthritis. We retrospectively evaluated changes in balance among patients with bilateral osteoarthritis who underwent staged TKA using a gravicorder.

Methods

Patients were stratified into two groups: the unilateral group (UG) (22 patients) consisted of patients who did not undergo a second TKA within 24 months of the first TKA, and the bilateral group (BG) (20 patients) were those who had a second TKA within 12 months after initial TKA.

Results

The mean gravity center position (GCP), which indicates the translation of GCP in the mediolateral direction between pre- and post-TKA shifted to the operative side in both groups after initial surgery. While the GCP was maintained on the same side in UG over 2 years follow-up, in BG it moved to the opposite side and approached a central position after the second TKA. Locus length of GCP (LG), which indicates postural control function by proprioceptive reflex showed significant improvement after initial TKA in UG, while BG showed significant improvement after the second TKA.

Conclusions

The degree of LG improvement after initial TKA may indicate the necessity of a second TKA for patients with bilateral osteoarthritis. The current study suggests that simultaneous bilateral TKA is not always necessary for patients with bilateral knee arthritis, and that properly performed rehabilitation such as improving postural sway after initial TKA might attenuate the timing for the second TKA.     

Bispectral index monitoring in fast track pediatric cardiac surgery

BACKGROUND: We evaluated the changes in the bispectral index (BIS) as a potential indicator of level of consciousness in infants and children undergoing fast track cardiac surgery. METHODS: Twenty-one children undergoing fast track cardiac surgery were recruited into this study. Anesthesia was maintained with inhaled sevoflurane and intravenous fentanyl 10 microg x kg(-1). Cardiopulmonary bypass (CPB) with mild hypothermia and an immediate tracheal extubation protocol were used. BIS was recorded throughout the operation. RESULTS: In average, BIS was kept almost under 70 with 0.5-3.0% of sevoflurane. During rewarming from mild hypothermia, BIS increased temporarily over 70 in about a half of children. We, therefore, treated them by increasing sevoflurane concentration. Nineteen children were extubated in the operating room, and two patients were extubated in ICU within three hours after surgery. CONCLUSIONS: BIS was kept within the level of adequate sedation during surgery. However, since the increase in BIS during the rewarming phase could reflect light anesthesia, caution should be taken around this phase.