Total bile acids levels as a stratification tool for screening portopulmonary hypertension in patients with decompensated cirrhosis.

AIM: Echocardiography is necessary for portopulmonary hypertension diagnosis, and identifying patients with cirrhosis who require it is challenging. In this study, we aimed to investigate the utility of the total bile acid (TBA) levels as a screening tool for identifying patients with decompensated cirrhosis who should undergo echocardiography for portopulmonary hypertension diagnosis. METHODS: We evaluated 135 patients with decompensated cirrhosis who underwent liver transplantation. Subsequently, factors contributing to tricuspid regurgitation pressure gradient (TRPG) elevation (≥30 mmHg) were analyzed using preoperative data, including the TBA levels. RESULTS: The median age of patients was 58 years (61 women), and 45 and 90 patients had Child-Turcotte-Pugh grades of B and C, respectively. The median TRPG level was 21 mmHg, and 17 patients (12.6%) showed TRPG elevation. Multiple logistic regression analysis revealed that elevated TBA (odds ratio 4.322; p = 0.013) and main pulmonary artery diameter ≥33 mm (odds ratio 4.333; p = 0.016) were significantly associated with TRPG elevation. The TBA cut-off value (167.7 µmol/L) showed a high diagnostic performance, with 70.6% sensitivity and 64.4% specificity. Ursodeoxycholic acid (UDCA) administration increased the TBA levels dose-dependently. Analysis stratified by UDCA use revealed that in patients not taking UDCA (n = 59), elevated TBA levels and younger age significantly contributed to TRPG elevation. However, in those taking UDCA (n = 76), this contribution disappeared, suggesting that UDCA consumption reduced TBA levels' efficiency in diagnosing TRPG elevation. CONCLUSIONS: The TBA levels may be a potential screening tool for TRPG elevation; however, caution is warranted when interpreting cases treated with UDCA.

The importance of the palatine bone for endoscopic endonasal skull base surgery.

Endoscopic endonasal skull base surgery is increasingly prevalent, with its scope expanding from pathogens in the midline region to those in the paramedian region. Maximizing anterior sphenoidectomy is important for the median approach, and lateralizing the pterygopalatine fossa is crucial for the paramedian approach. Maximizing the surgical corridor in the nasal cavity and minimizing damage to neurovascular structures are vital for establishing a surgical field with minimal bleeding, ensuring safe, precise, and gentle procedures. However, the relationship between the maxillofacial and skull base bones in endoscopic endonasal skull base surgery is difficult to understand because these bones are intricately articulated, making it challenging to visualize each bone's outline. Understanding important bones and their related neurovascular structures is essential for all skull base surgeons to maximize the surgical corridor and minimize iatrogenic injury to neurovascular structures. This study aimed to elucidate the role of the palatine bone from a microsurgical anatomical perspective. Three dry skulls were used to demonstrate the structure of the palatine bone and its relationship with surrounding bones. A formalin-perfused cadaveric head was dissected to show the related neurovascular structures. The arteries and veins of the cadaveric heads were injected with red- and blue-colored silicon. Dissection was performed using a surgical microscope and endoscope. In addition, the utilization of the palatine bone as a landmark to identify neurovascular structures, which aids in creating a wider surgical field with less bleeding, was shown in two representative cases. The palatine bone consists of unique complex structures, including the sphenoidal process, ethmoidal crest, pterygopalatine canal, and sphenopalatine notch, which are closely related to the sphenopalatine artery, maxillary nerve, and its branches. The ethmoidal crest of the palatine bone is a well-known structure that is useful for identifying the sphenopalatine foramen, controlling the sphenopalatine artery and nerve, and safely opening the pterygopalatine fossa. The sphenoidal process of the palatine bone is a valuable landmark for identifying the palatovaginal artery, which is a landmark used to safely and efficiently expose the vidian canal. The sphenoidal process is easily cracked with an osteotome and removed to expose the palatovaginal artery, which runs along the pharyngeal groove, just medial to the vidian canal. By opening the pterygopalatine canal (also known as the greater palatine canal), further lateralization of the periosteum-covered pterygopalatine fossa contents can be achieved. Overall, the sphenoidal process and ethmoidal crest can be used as important landmarks to maximize the surgical corridor and minimize unnecessary injury to neurovascular structures.

Loss of function of the Pad-1 aminotransferase gene, which is involved in auxin homeostasis, induces parthenocarpy in Solanaceae plants.

Fruit development normally occurs after pollination and fertilization; however, in parthenocarpic plants, the ovary grows into the fruit without pollination and/or fertilization. Parthenocarpy has been recognized as a highly attractive agronomic trait because it could stabilize fruit yield under unfavorable environmental conditions. Although natural parthenocarpic varieties are useful for breeding Solanaceae plants, their use has been limited, and little is known about their molecular and biochemical mechanisms. Here, we report a parthenocarpic eggplant mutant, pad-1, which accumulates high levels of auxin in the ovaries. Map-based cloning showed that the wild-type (WT) Pad-1 gene encoded an aminotransferase with similarity to Arabidopsis VAS1 gene, which is involved in auxin homeostasis. Recombinant Pad-1 protein catalyzed the conversion of indole-3-pyruvic acid (IPyA) to tryptophan (Trp), which is a reverse reaction of auxin biosynthetic enzymes, tryptophan aminotransferases (TAA1/TARs). The RNA level of Pad-1 gene increased during ovary development and reached its highest level at anthesis stage in WT. This suggests that the role of Pad-1 in WT unpollinated ovary is to prevent overaccumulation of IAA resulting in precocious fruit-set. Furthermore, suppression of the orthologous genes of Pad-1 induced parthenocarpic fruit development in tomato and pepper plants. Our results demonstrated that the use of pad-1 genes would be powerful tools to improve fruit production of Solanaceae plants.

Anatomy of small canals around the jugular foramen: Special reference to Jacobson's and Arnold's nerves.

The jugular foramen harbors anatomically complex bony, venous and neural structures. It is closely associated with small canals including the mastoid, tympanic, and cochlear canaliculi, and the stylomastoid foramen. The minute intraosseous branches of Arnold's and Jacobson's nerves (<1 mm in length) remain difficult to study with current imaging techniques, and cadaveric dissection is the most reliable approach. Our aim was to examine the variations of Jacobson's and Arnold's canaliculi and nerves and to provide detailed cadaveric graphics. To reveal the anatomical structures of small canals around the jugular foramen, 25 sides of dry skulls and 14 sides of cadaveric heads were examined. Intraosseous branches varied more in Arnold's nerve than Jacobson's nerve. In our cadaveric dissection, all specimens formed a single canal for Jacobson's nerve connecting the jugular foramen to the tympanic cavity. The intraosseous course of Arnold's nerve varied in its communication with the facial nerve. A descending branch crossing the facial nerve was identified in five of 14 sides, an ascending branch in 13. In two specimens, an ascending branch clearly reached the base of the stapedius muscle. Classical anatomical studies of cadavers remain a supplementary tool for analyzing these tiny structures. The present study confirms Gray's findings of 1913. Variations of these nerves could be even more complex than previously reported. Our study provides additional information regarding the anatomy of Jacobson's and Arnold's nerves.

Cleidocranial dysplasia with growth hormone deficiency: a case report.

BACKGROUND: Cleidocranial dysplasia (CCD) is a rare skeletal disorder with autosomal dominant inheritance that is characterized by hypoplastic clavicles, delayed closure of the cranial sutures, dental abnormalities, and short stature, among other features. The responsible gene for CCD is RUNX2 located on the short arm of chromosome 6p21. In general, there are intrafamilial variations in height among CCD patients. Few studies have reported data on recombinant human growth hormone (rhGH) treatment for patients with CCD; thus, it remains to be elucidated whether rhGH treatment can improve short stature. Here, we report a case of a 6-year-old girl with CCD who has growth hormone deficiency (GHD) and a novel mutation of RUNX2. CASE PRESENTATION: At 5 years of age, this patient was diagnosed with GHD and rhGH treatment was initiated. Thereafter, she was diagnosed with CCD due to the presence of hypoplastic clavicles and an open fontanelle, which was also observed in her mother and brother. She responded well to rhGH treatment; her height improved from - 3.2 SD to - 2.4 SD after 13 months. CONCLUSION: A detailed patient history and physical examination are necessary for the early diagnosis of CCD. Similarly, to ascertain the effect of rhGH treatment, careful evaluation of the patient's final height post-therapy is needed.

[Unruptured Fronto-Orbital Artery Aneurysm Anomalously Originating from the Proximal Segment of the Anterior Cerebral Artery(A1)Concurrent with Tuberculum Sellae Meningioma:A Case Report].

We report a case of an unruptured aneurysm arising from the bifurcation of the fronto-orbital artery originating from the proximal segment of the left anterior cerebral artery(A1)concurrent with a tuberculum sellae meningioma. A 46-year-old woman presented with a right visual disturbance and left temporal hemianopsia. Preoperative neuroradiological examination showed a heterogeneously enhanced extra-axial suprasellar tumor expanding to the right optic canal and 3-mm diameter aneurysm arising from the bifurcation of the fronto-orbital artery originating from the proximal segment of the left anterior cerebral artery(A1). She underwent total removal of the tumor and neck clipping of the aneurysm via a right pterional approach without any additional deficits. Postoperative MRI and 3D CTA showed total tumor removal and complete obliteration of the aneurysm. The patient was discharged 21 days after surgery without new or worsened postoperative neurological deficits. Cerebral aneurysm concurrent with meningioma is extremely rare. Preoperative diagnosis and appropriate assessment of both the meningioma and unruptured aneurysm are warranted to prevent severe complications.

Prophylactic Opening of the Pleural Cavity for Postoperative Drainage is a Risk Factor for Prolonged Pleural Effusion After a Fontan Operation.

Postoperative prolonged pleural effusion (PPE) remains a confounding problem after a Fontan operation. We aimed to describe the risk factors for PPE after a Fontan operation and to clarify the impact of prophylactic opening of the pleural cavity (POPC) for drainage tube insertion on PPE. We retrospectively reviewed the medical charts of 50 consecutive patients who underwent a Fontan operation at our institution. POPC for postoperative drainage was performed based on each surgeon's preference. Patients were divided into three groups for analysis: group A (n = 12), no opening; group B (n = 14), unilateral opening; and group C (n = 24), bilateral opening. At the time of surgery, the median age of our patient group was 26 months, with a median body weight of 10.5 kg. The volume of pleural effusion tended to be lower in group A than in groups B and C (p = 0.08). The median duration of drainage was significantly shorter (p = 0.03) in group A (3 days) than in group B (4 days) or C (5 days). Overall, 12 patients required chest tube drainage for ≥ 7 days. Multivariate analysis revealed POPC (p = 0.01) and postoperative water balance (p = 0.03) as independent predictors of PPE. POPC and postoperative water balance are risk factors for PPE after a Fontan operation. Therefore, avoiding POPC for postoperative drainage may reduce the risk of postoperative pleural effusion and morbidities associated with PPE after a Fontan operation.

Scattering pulse-induced temporal contrast degradation in chirped-pulse amplification lasers.

Herein, a theory for modeling the problem of scattering pulse-induced temporal contrast degradation in chirped-pulse amplification (CPA) lasers is introduced. Using this model, the temporal evolutions of the scattering and signal pulses were simulated, the temporal contrasts for different cases were compared, and finally the theoretical prediction was verified by an experimental demonstration. The result shows that the picosecond and the nanosecond temporal contrast is mainly determined by the scattering pulses generated in the stretcher and the compressor, respectively. In addition, the B-integral accumulation will further degrade the temporal contrast, especially the picosecond temporal contrast. We believe it is helpful for solving the problem of the picosecond pedestal contrast (i.e., noise limit). With reference to these results, some suggestions for the temporal contrast improvement are presented.

Mechanisms underlying increased vascular smooth muscle contractility in the rabbit basilar artery following subarachnoid hemorrhage.

Increased vascular contractility plays an important role in the development of cerebral vasospasm following subarachnoid hemorrhage (SAH). Here, we summarize our current knowledge regarding molecular mechanisms that contribute to increased smooth muscle contractility of rabbit basilar artery following SAH. Our studies demonstrated that upregulation of receptor expression, impairment of feedback regulation of receptor activity, and enhancement of myofilament Ca²âº sensitization might lead to increased smooth muscle contractility following SAH.

[Effect of weekly paclitaxel followed by 5-fluorouracil, epirubicin, and cyclophosphamide as neoadjuvant treatment for patients with triple-negative and luminal-type breast cancer - a multicenter study].

This study examined the pathological complete response (pCR )rate and safety of induction chemotherapy with 12 cycles of weekly paclitaxel (80 mg/m²) followed by 4 cycles of 5-fluorouracil (500 mg/m²), epirubicin (100 mg/m²), and cyclo- phosphamide (500 mg/m²). The study medication was administered to female patients (n=31)with a mean age of 51 years, diagnosed with stage II A (n=18), II B (n=11) and III A (n=2) disease and with an estrogen receptor positive rate of 65% (20/31). No patient was HER2-IHC [human epidermal growth factor receptor 2 (HER2) immunohistochemistry (IHC)](3+) or HER2-FISH (fluorescence in situ hybridization) positive. Twenty-eight patients completed the treatment regimen. Treatment was halted in 2/31 patients due to progression of disease in one patient and a Grade 3 non-hematological adverse effect of skin eruption and itching in the other patient. A third patient died of causes unrelated to the study medication. Central review ascertained a pCR in 6 patients. In patients with triple-negative disease we observed a pCR rate of 67% (6/9). In patients with the Luminal (A+B) subtype, 0% (0/19) had a pCR. Grade 3/4 toxicity included leucopenia (58%), neutropenia (58%), febrile neutropenia (26%), fatigue (10%), and ALT elevation (7%). In terms of pCR, patients presenting with triple-negative disease and manageable safety profiles appear to respond well to this treatment regimen, while only a modest response was observed in patients with Luminal subtype disease.

Clinical, anatomical, and histological features of the rhomboid lip and considerations for surgery using a retrosigmoid approach: a retrospective study.

OBJECTIVE: The rhomboid lip is a neural tissue encountered during cerebellopontine angle surgery, with differing shape and extent among individuals. This study aimed to investigate the variation of rhomboid lips during posterior fossa surgery. METHODS: In this retrospective study, we examined posterior cranial fossa surgeries performed using a retrosigmoid approach. Rhomboid lips were classified according to thickness, extent, and appearance, with some subjected to histological analysis. T2-weighted magnetic resonance imaging (MRI) of rhomboid lips was conducted. RESULTS: Among 304 surgeries, rhomboid lips were observed in 75 patients who underwent schwannoma or meningioma resection, facial spasm-related neurovascular decompression, and other surgeries (37, 2, 32, and 4 patients, respectively). Rhomboid lips were categorized based on apparent thickness: thin membranous type, resembling an arachnoid membrane, and thick parenchymal type. Rhomboid lip extension was classified by position relative to the choroid plexus: non-extension, lateral extension, and jugular foramen (41, 22, and 12 patients, respectively). Veins were observed on the rhomboid lip surface in 37 cases. The rhomboid lip was visible in only one case (parenchymal jugular foramen type) on MRI. Histologically, the rhomboid lip comprised an ependymal cell layer, a glial layer, and connecting tissue. The glial layer thickness determined the rhomboid lip thickness, which was greater in the parenchymal type than in the membrane type. In 42 patients, the rhomboid lip was dissected, with no complications observed. CONCLUSIONS: Morphological classification of the rhomboid lip and understanding of its anatomical details contribute to safe surgical field development for neurosurgeons.

Are transmembrane 6 superfamily member 2 gene polymorphisms associated with steatohepatitis after pancreaticoduodenectomy?

Aim: After pancreaticoduodenectomy, 20-40% of patients develop steatotic liver disease (SLD), and steatohepatitis can be a problem. Although patatin-like phospholipase domain-containing 3 protein (PNPLA3) and transmembrane 6 superfamily member 2 (TM6SF2) polymorphisms are involved in SLD and steatohepatitis development, whether this is the case after pancreaticoduodenectomy is unclear. Methods and Results: Forty-three patients with pancreatic cancer who underwent pancreaticoduodenectomy at our hospital between April 1, 2018, and March 31, 2021, were included. We extracted DNA from noncancerous areas of residual specimens after pancreaticoduodenectomy and determined PNPLA3 and TM6SF2 gene polymorphisms using real-time polymerase chain reaction. SLD was defined as a liver with an attenuation value of ≤40 HU or a liver-to-spleen ratio of ≤0.9 on computed tomography. We defined high hepatic fibrosis indexes (HFI) instead of steatohepatitis as a Fibrosis-4 index of ≥2.67 or nonalcoholic fatty liver disease fibrosis score of ≥0.675 in patients with SLD. The cumulative incidence of SLD (P = 0.299) and high HFI (P = 0.987) after pancreaticoduodenectomy were not significantly different between the PNPLA3 homozygous and minor allele groups. The incidences of high HFI at 1 year after pancreaticoduodenectomy were 16.8% and 27.0% in the TM6SF2 major homozygous and minor allele groups, respectively, with a significant difference in the cumulative incidence (P = 0.046). Conclusion: The TM6SF2 minor allele may contribute to steatohepatitis development after pancreaticoduodenectomy.

[Successful treatment using recombinant thrombomodulin for disseminated intravascular coagulation associated with recurrent prosthetic valve endocarditis].

Prosthetic valve endocarditis(PVE)occasionally evokes sepsis and disseminated intravascular coagulation(DIC). A 46-year-old man developed relapsing active PVE with an annular abscess and suffered from exacerbating sepsis and DIC. Despite the administration of antibiotics, his DIC score increased. Anti-DIC treatment with recombinant thrombomodulin (rTM) was initiated, and his DIC was remarkably resolved. Accordingly, the abscess cavity was closed by using a homograft anterior mitral leaflet, and the aortic root was replaced with the homograft. He is doing well without an evidence of recurrent endocarditis 18 months after the operation. rTM is a new and promising drug for the treatment of DIC with infective endocarditis.

Midline Suboccipital Unilateral Trans-Cerebellomedullary Fissure Approach for Clipping of Ruptured VA-PICA Aneurysm: Two-Dimensional Operative Video.

The vertebral artery-posterior inferior cerebellar artery (VA-PICA) aneurysm poses a technical challenge for microsurgical clipping due to its anatomical complexity, which requires dissection of lower cranial nerves. Endovascular treatment is regarded as a feasible first-line therapeutic option for VA-PICA aneurysm because it has an acceptable aneurysm occlusion rate and is less invasive. However, microsurgical clipping remains an effective treatment option. We present the case of a 62-year-old man who presented with subarachnoid hemorrhage (SAH) due to a ruptured VA-PICA aneurysm. Neuroradiologic examination revealed a 2-3 mm medially pointing left VA-PICA aneurysm with acute obstructive hydrocephalus due to massive SAH in the posterior cranial fossa. As the patient had acute obstructive hydrocephalus and a relatively small aneurysm, we selected clipping over endovascular treatment. Because the aneurysm was located close to the midline and anterolateral to the medulla oblongata, we approached it from the midline. A midline suboccipital craniotomy, C1 laminectomy, and drilling of the left condylar fossa were performed; a unilateral cerebellomedullary fissure opening was added; and the aneurysm was clipped. Postoperative neuroradiologic examinations revealed complete obliteration of the aneurysm. As shown in this video, unilateral cerebellomedullary fissure opening combined with adequate removal of the condylar fossa provides a wide operative field in the cerebellomedullary cistern while avoiding strong retraction of the cerebellum. We believe that this technique makes VA-PICA aneurysm clipping safe and successful. Patient consent was obtained to perform the surgery and to publish the surgical video (Video 1).

Bilateral lung transplant with pulmonary artery reconstruction using donor aorta for pulmonary hypertension with a giant pulmonary arterial aneurysm.

OBJECTIVES: Standard bilateral lung transplantation (BLT) is not feasible for patients with pulmonary arterial hypertension (PAH) complicated with a giant pulmonary arterial aneurysm (PAA). This study aimed to describe the outcomes of BLT with pulmonary artery reconstruction (PAR) using donor aorta for such patients. METHODS: This is a retrospective single-centre study reviewing PAH patients with a PAA who received BLT with PAR using donor aorta from January 2010 through December 2020. We compared the characteristics and short- and long-term outcomes of recipients receiving PAR (PAR group) with those who had no PAA and received standard BLT (non-PAR group). RESULTS: Nineteen adult PAH patients underwent cadaveric lung transplantation during the study period. Among them, 5 patients with a giant PAA (median pulmonary artery trunk diameter, 69.9 mm) underwent BLT with PAR using donor aorta and the others received standard BLT. Although the operation time tended to be longer in the PAR group compared with the non-PAR group (1239 vs 958 mins, P = 0.087), 90-day mortality (PAR group: 0% vs non-PAR group: 14.3%, P > 0.99), and 5-year survival rate (PAR group: 100% vs non-PAR group: 85.7%, P = 0.74) was comparable between the groups. No dilatation, constriction or infection of the aortic grafts were recorded during the study period with a median follow-up time of 94 months in the PAR group. CONCLUSIONS: Lung transplantation with PAR using donor aorta is a valid surgical option for PAH patients complicated with a giant PAA.

Comparison of the novel Franseen needle versus the fine-needle aspiration needle in endoscopic ultrasound-guided tissue acquisition for cancer gene panel testing: A propensity score-matching analysis.

Background and Aim: Reports have indicated that a surface area of 4 mm2 or more of collected tissue sections could provide the recommended total DNA for the OncoGuide NCC Oncopanel system, which is a cancer gene panel test developed in Japan. We wished to compare the percentage of tissue sections collected by endoscopic ultrasound-assisted tissue acquisition (EUS-TA) with surface areas of ≥4 mm2 between a conventional needle, namely the EZ Shot 3 Plus (Olympus Medical Japan, Tokyo, Japan) (EZ3), and the recent SonoTip TopGain (MediGlobe, Rohrdorf, Germany) (TopGain). Method: From April 2010 to December 2021, among 693 EUS-TA cases, EZ3 was used in 390 cases and TopGain in 45. The EZ3 and TopGain groups were matched in a 1:1 ratio with a tolerance of 0.2, with 35 patients each matched using propensity score analysis. Results: The TopGain group had a significantly higher percentage of cases with a tissue area of ≥4 mm2 than the EZ3 group (42.9% vs 68.6%, P = 0.030). Multivariate analysis revealed an association between TopGain and tissue areas of ≥4 mm2 (odds ratio 2.996, 95% confidence interval 1.068-8.403, P = 0.037). Conclusions: EUS-TA using TopGain significantly collected more ≥4 mm2 tissue area compared with EZ3, suggesting its usefulness for cancer gene panel testing.

Roles and regulation of cytokinins in tomato fruit development.

Cytokinins (CKs) are thought to play important roles in fruit development, especially cell division. However, the mechanisms and regulation of CK activity have not been well investigated. This study analysed CK concentrations and expression of genes involved in CK metabolism in developing tomato (Solanum lycopersicum) ovaries. The concentrations of CK ribosides and isopentenyladenine and the transcript levels of the CK biosynthetic genes SlIPT3, SlIPT4, SlLOG6, and SlLOG8 were high at anthesis and decreased immediately afterward. In contrast, trans-zeatin concentration and the transcript levels of the CK biosynthetic genes SlIPT1, SlIPT2, SlCYP735A1, SlCYP735A2, and SlLOG2 increased after anthesis. The expression of type-A response regulator genes was high in tomato ovaries from pre-anthesis to early post-anthesis stages. These results suggest that the CK signal transduction pathway is active in the cell division phase of fruit development. This study also investigated the effect of CK application on fruit set and development. Application of a synthetic CK, N-(2-chloro-pyridin-4-yl)-N'-phenylurea (CPPU), to unpollinated tomato ovaries induced parthenocarpic fruit development. The CPPU-induced parthenocarpic fruits were smaller than pollinated fruits, because of reduction of pericarp cell size rather than reduced cell number. Thus, CPPU-induced parthenocarpy was attributable to the promotion of cell division, not cell expansion. Overall, the results provide evidence that CKs are involved in cell division during development of tomato fruit.

HLA-class II and class I genotypes among Japanese children with Type 1A diabetes and their families.

OBJECTIVE: To determine the HLA-DRB1, DQB1, DPB1, A, C, and B genotypes among Japanese children with autoimmune type 1 diabetes. METHODS: Four hundred and thirty patients who were GADAb and/or IA-2Ab-positive (Type 1A) were recruited from 37 medical centers as part of a nationwide multicenter collaborative study. DNA samples from 83 siblings of the children with Type 1A diabetes and 149 parent-child trios were also analyzed. A case-control study and a transmission disequilibrium test (TDT) were then performed. RESULTS: The susceptible and protective DRB1 and DQB1 alleles and haplotypes were confirmed. DPB1 alleles unique to the Japanese population and those common to multiple ethnic groups were also present. A linkage disequilibrium (LD) analysis showed both susceptible and protective haplotypes. The TDT did not reveal any alleles that were transmitted preferentially from the mother or father to children with Type 1A. Homozygosity for DRB1-09:01-DQB1-03:03 and heterozygosity for DRB1-04:05-DQB1-04:01 and DRB1-08:02-DQB1-03:02 were associated with an extremely high risk of Type 1A. A comparison of children with Type 1A and their parents and siblings suggested a dose effect of susceptible DRB1-DQB1 haplotypes and an effect of protective alleles on immunological pathogenesis. DRB1-09:01 appeared to be strongly associated with an early onset in preschool children with Type 1A diabetes. CONCLUSIONS: This study demonstrated the characteristic association of HLA-class II and class I genes with Type 1A diabetes among Japanese children. A TDT did not reveal the genomic imprinting of HLA-class II and class I genes in Type 1A diabetes.

Lateral Suboccipital Infrafloccular Approach with Extensive Arachnoid Dissection for Vertebral Artery-Associated Hemifacial Spasm: Two-Dimensional Operative Video.

Hemifacial spasm (HFS) is generally caused by compression of the root exit zone (REZ) of the facial nerve by the anterior and posterior inferior cerebellar arteries and occasionally the vertebral artery (VA). Owing to its large caliber and high stiffness, microvascular decompression (MVD) for VA-associated HFS is considered more difficult, and the result is worse than for HFS not associated with the VA.1,2 Therefore, a safer, more reliable MVD is required for VA-associated HFS. In Video 1, we demonstrate our MVD technique in a 57-year-old woman who presented with left HFS owing to facial nerve compression by a dolichoectatic VA. A lateral suboccipital infrafloccular approach with extensive arachnoid dissection was performed. Arachnoid dissection was started from the cisterna magna and continued from the caudal to the rostral direction. This extensive arachnoid dissection provided access to the facial nerve REZ through the infrafloccular route with gentle retraction of the flocculus in the caudorostral direction, while avoiding strong retraction of cranial nerve VIII and the cerebellum. In addition, we were able avoid damaging the neurovascular structures in the operative field. This is mandatory to make the operative field bloodless and facilitate identifying the relationship between the facial nerve REZ and the offending vessels. MVD of the facial nerve REZ was achieved. The patient's postoperative course was uneventful, and her HFS resolved postoperatively. Patient consent was obtained to perform the surgery and to publish the surgical video.