Postoperative Changes in Vessel Density according to Macular Hole and Macular Pseudohole Subtypes

Purpose@#To determine the postoperative changes in vessel density according to macular hole and macular pseudohole (MPH) subtypes and to investigate the differences in the mechanisms underlying their development. We also investigated whether changes in vessel density are correlated with changes in the multifocal electroretinogram (mfERG) and best corrected visual acuity (BCVA). @*Methods@#We reviewed the medical records of patients with MPH or a macular hole who underwent pars plana vitrectomy. We included 15 eyes of 15 patients with a full thickness macular hole (FTMH), nine eyes of nine patients with a tractional lamellar macular hole (LMH), eight eyes of eight patients with a degenerative LMH, and nine eyes of eight patients with a MPH. The BCVA, foveal avascular zone (FAZ), foveal and parafoveal vessel density, and mfERG ring 1 and ring 2 P1 amplitudes were analyzed before and 1 and 6 months after surgery. @*Results@#One month postoperatively, the foveal vessel density of patients with a MPH or tractional LMH increased (p = 0.011, p = 0.008). The parafoveal vessel density of patients with a MPH, tractional LMH, and FTMH increased (p = 0.007, p = 0.038, p = 0.031). There was no significant increase in foveal or parafoveal vessel density in patients with a degenerative LMH (p = 0.201, p = 0.171). There was a significant correlation between the change in parafoveal vessel density and that in BCVA 6 months postoperatively in patients with a FTMH (r = -0.543, p = 0.037). @*Conclusions@#By assessing changes in vessel density after vitrectomy, it is possible to estimate the effect of traction according to the type of macular hole. There was a significant correlation between parafoveal vessel density and BCVA in patients with a FTMH. Restoration of the retinal structure and vessel density might improve visual acuity.

Recurrent Ophthalmoplegia Associated with Hyperhomocysteinemia

Purpose@#This report describes a case of recurrent ophthalmoplegia associated with hyperhomocysteinemia due to methylenetetrahydrofolate reductase (MTHFR) gene polymorphism.Case summary: A 40‐year‐old healthy man presented with acute diplopia. He had a history of diplopia due to left sixth nerve palsy 2 years ago. Prism and alternate cover tests revealed left hypertropia in the primary position. Examination of ductions and versions showed mild elevation of the left eye on adduction. Brain and orbit magnetic resonance imaging were normal. Laboratory studies revealed an elevated level of erythrocyte sedimentation rate (ESR) and homocysteine. A diagnosis of left fourth nerve palsy associated with hyperhomocysteinemia was made. Symptoms were completely resolved within 2 weeks. Two years later, the patient again had diplopia associated with esotropia and limited abduction of the right eye. ESR and homocysteine level were normal. Analysis for MTHFR gene polymorphisms, which contribute to variable hyperhomocysteinemia, revealed 677TT homozygote variant. A diagnosis of recurrent paralytic strabismus associated with hyperhomocysteinemia, caused by MTHFR gene polymorphism, was made. Symptoms resolved within 1 month, and the patient did not have any further recurrence in 6 months. @*Conclusions@#Patients with hyperhomocysteinemia may present with ophthalmoplegia. An analysis for MTHFR gene polymorphisms is needed to diagnose hyperhomocysteinemia.

Early Detection of Kawasaki Disease in Infants

BACKGROUND AND OBJECTIVES: This study aimed to investigate the clinical characteristics of infantile Kawasaki disease (KD), and to evaluate early diagnostic features of KD in febrile infants. SUBJECTS AND METHODS: We retrospectively reviewed the medical records of 64 KD patients from January 2010 to October 2014. There was an analysis of the clinical, laboratory data of the infants versus children groups. Furthermore, the clinical and laboratory data of infantile KD patients were compared with 16 infants who were admitted for other acute febrile diseases. RESULTS: A total of 64 patients with KD were identified; 20 (31.3%) were infants; 44 (68.8%) were >1 year old children. Incomplete KD was much more common in infants (n=13, 65.0%) than in children group (n=14, 31.8%) (p=0.013). The infants were characterized by significantly higher rates of inflammatory changes at the Bacille Calmett-Guerin (BCG) inoculation site (p<0.001), but lower rates of changes in the extremities (p=0.029) and cervical lymphadenopathy (p=0.006). The serum levels of platelet after 1 week (p=0.005), C-reactive protein (p=0.038), and N-terminal pro-brain natriuretic peptide (NT-proBNP) (p=0.026) were all significantly higher in the infants group. Comparing the infants with KD versus the other acute febrile diseases, there were significantly higher serum levels of erythrocyte sedimentation rate (p=0.002), C-reactive protein (p=0.046) and NT-proBNP (p=0.001) for the infants with KD group. CONCLUSION: BCGitis and higher levels of NT-proBNP can be helpful for early diagnosis of the incomplete KD in infants, and may be a good predictor of KD in acute febrile infants, when combined with other acute phase reactants.

Alexander Disease

Alexander disease (ALXD) is a rare demyelinating disease of the white matter of the brain that is caused by a mutation in the glial fibrillary acidic protein (GFAP) gene. The overexpression of GFAP in astrocytes induces a failure in the developmental growth of the myelin sheath. The neurodegenerative destruction of the myelin sheath of the white matter is accompanied by an accumulation of abnormal deposits of Rosenthal fibers in astrocytes, which is the hallmark of ALXD. The disease can be divided into four groups based on the onset age of the patients: neonatal, infantile, juvenile, or adult. Early-onset disease is more severe, progresses rapidly, and results in a shorter life span than late-onset cases. Magnetic resonance imaging and genetic tests are mostly used for diagnostic purposes. Pathological tests of brain tissue for Rosenthal fibers are definitive diagnostic methods. Therapeutic strategies are being investigated. Ceftriaxone, which is an enhancer of glial glutamate transporter (GLT-1) expression, is currently in clinical trials for the treatment of patients with ALXD. To date, there are no clinically available treatments. The cause, pathology, pathophysiology, inheritance, clinical features, diagnosis, and treatment of ALXD will be reviewed comprehensively.

Small Bowel Obstruction Caused by an Aberrant Congenital Band in a Child / 대한소아소화기영양학회지

Small bowel obstruction due to congenital band is not only rare in children, but also difficult to diagnose, because common symptoms such as vomiting and abdominal pain are observed in patients. In order to prevent a fatal result, an anomalous congenital band should be considered in the discriminative diagnosis of intestinal obstruction in children who have no previous experience of operation or intraperitoneal inflammation. This report presents a 4-year-old boy who was admitted with abdominal pain and nonbilious vomiting for a day. The initial suspicion was for acute gastroenteritis. However, after further investigation and performance of surgery, the intestinal obstruction associated with a congenital band was confirmed. No recurrence was observed during the 8-month follow-up period. Thus early confirmation based on radiologic study is a crucial factor for the diagnosis of small bowel obstruction caused by a congenital band.

A Case of Langerhans Cell Histiocytosis with Hypothalamic Mass / 대한소아혈액종양학회지

A 14-month-old girl presented with petechial skin lesions and polydipsia was diagnosed as Langerhans cell histiocytosis (LCH) and responded fairly well to multiple chemotherapies using vincristine, cyclophosphamide, and prednisone. 3 years later, relapses were more common with short periods of remissions in spite of using more intensive therapy with vinblatine and etoposide. At age of 4.5, sudden weight gain and abnormal behavior led to MRI study and revealed an hypothalamic mass. Radiation of 1, 800 cGy was given to the mass and followed by a 75% decrease in measuring and remission of the obesity. Although, there was no evidence of tumor progression in the hypothalamus, she died of sepsis due to systemic progression of the disease at age of 5. LCH commonly present with the symptoms of diabetes insipidus, but hypothalamic mass is not common. We report this case with a brief review of literatures.

Prevalence of Ambler Class A Extended-Spectrum beta-Lactamase-Producing Escherichia coli and Klebsiella pneumoniae Isolates in Korea / 대한임상미생물학회지

BACKGROUND: The aim of this study is to determine the nationwide prevalence of Ambler class A extended-spectrum beta-lactamase (ESBL)-producing Escherichia coli and Klebsiella pneumoniae and to characterize genotypes of ESBLs. METHODS: During the period of February through July, 2003, E.coli and K.pneumoniae isolates were collected from 12 hospitals in Korea. Antimicrobial susceptibilities were tested by disk diffusion method, and ESBL-production was determined by the double-disk synergy test. MICs of beta-lactam antibiotics were tested by agar dilution method. Searches for bla TEM, bla SHV, bla CTX-M, bla PER-1, bla VEB, bla IBC, bla GES and bla TLA genes were performed by PCR amplification, and the genotypes of ESBLs were determined by direct nucleotide sequence analysis of amplified products. RESULTS: Resistance rates of E.coli (n=246) and K.pneumoniae (n=239) isolates to ceftazidime were 8.5% and 20.1%, respectively. Most prevalent Ambler class A ESBL genotypes in E.coli isolates were bla CTX-M-15 (n=4) and bla CTX-M-3 (n=3), and each of bla CTX-M-14, bla SHV-12, and bla TEM-52 gene was also found in one isolate. Most prevalent ESBL genotypes in K.pneumoniae were bla SHV-12 (n=30) and bla CTX-M-3 (n=13), and bla CTX-M-14 (n=5). bla SHV-2a (n=3), bla SHV-5 (n=2), bla TEM-52 (n=1), bla GES-3 (n=2) genes were also found. CONCLUSION: CTX-M-type ESBL-producing E.coli and K.pneumoniae isolates are spreading, and a GES-type ESBL has emerged in Korea.

A Case of Transient Symptomatic Zinc Deficiency - in A cows milk - Fed , preterm Infant -

Acrodermatitis enteropathica(AE) which starts in early infancy after weaning is a rare hereditary chronic disorder of zinc absorption. AE is characterized by alopecia, diarrhea and skin lesions localized to periorificial areas and acrally on the extremities. However, recent reports presented Transient Symptomatic Zinc Deficiency(TSZD) in preterm infants. TSZD is clinically similar to AE and skin lesions rapidly heals after zinc supplementation. When the treatment was withheld, no recurrence was seen. We experienced a TSZD case in a cow's milk fed, preterm infant, so We report it with a brief review of literature.