Acrodermatitis enteropathica is a rare autosomal recessive disorder affecting early infancy. This syndrome is characterized by acral and periorificial dermatitis, alopecia and intractable diarrhea. Whereas hereditary acrodermatitis enteropathica is caused by mutation affecting intestinal zinc absorption, transient acrodermatitis enteropathica is caused by inadequate supply or malabsorption of zinc in the premature infant. We report a case of transient acrodermatitis enteropathica in a 5-month-old, breast-fed premature infant.
Absorption ; Acrodermatitis* ; Alopecia ; Dermatitis ; Diarrhea ; Humans ; Infant ; Infant, Newborn ; Infant, Premature ; Zinc

BACKGROUND: Infrequent restriction site PCR (IRS-PCR) is a recently described DNA fingerprinting technique based on selective amplification of restriction endonuclease-cleaved fragments. We applied of IRS-PCR to clinical isolates of Vibrio parahaemolyticus associated with diarrhea. METHODS: IRS-PCR assay was performed with adaptors for XbaI and HhaI restriction sites. A total of 35 strains of V. parahaemolyticus which were isolated from clinical specimens of patients with diarrhea were analyzed. The isolates were collected from different geographic areas of Seoul (n=12), Incheon (n=21) and Gwangju (n=2) during 1998-2000 in Korea. RESULTS: In IRS-PCR, amplifed DNA fragments between 50 and 400 bp were found to be the most reproducible in this study. When V. parahaemolyticus isolates were amplified with AH1 and PX-G as primers, 35 isolates could be grouped into five IRS-PCR patterns: A (n=16), B (n=4), C (n=6), D (n=5) and E (n=4). The patterns were subdivided into 15 subtypes: A1, A2, B1, B2, B3, B4, C1, C2, C3, D1, D2, D3, E1, E2 and E3. The IRS-PCR patterns of V. parahaemolyticus did not show any relationship with serotype or geographic origin, but the isolates from same outbreak produced a same pattern(A1). CONCLUSION: The results provide evidence of the discriminatory power of the IRS-PCR method as it applies to V. parahaemolyticus.
Diarrhea ; DNA ; DNA Fingerprinting ; Gwangju ; Humans ; Incheon ; Korea ; Polymerase Chain Reaction* ; Seoul ; Vibrio parahaemolyticus* ; Vibrio*

Lafora' disease is an autosomal recessive, fatal, generalized polyglucosan storage disorder that occurs in childhood or adolescence with stimulus sensitive epilepsy (resting and action myoclonias, grand mal, and absence), dementia, ataxia and rapid neurological deterioration. We present a 19-year-old, 32 kg woman with lafora's disease was scheduled for tonsillectomy under general anesthesia. Her preanesthetic examination revealed extreme muscle atrophy and dementia. Grand mal, myoclonic seizures, and upper airway obstruction were frequent. General anesthesia and tracheal intubation with sevoflurane and nitrous oxide provided safe anesthesia. The intraoperative course was uneventful and the emergence of anesthesia was smooth.
Adolescent ; Airway Obstruction ; Anesthesia ; Anesthesia, General ; Ataxia ; Dementia ; Epilepsy ; Female ; Glucans ; Humans ; Intubation ; Methyl Ethers ; Muscular Atrophy ; Nitrous Oxide ; Seizures ; Tonsillectomy ; Young Adult

PURPOSE: The purpose of this study was to compare the curative effect of Proximal Femoral Nail Antirotation (PFNA) with a Proximal Femoral Nail (PFN) for the treatment of intertrochanteric fracture of the femur. MATERIALS AND METHODS: This study compared 58 cases of 57 patients who were treated by PFNA from June 2007 to February 2009 with 60 cases of 58 patients who were treated by PFN from July 2005 to May 2007. The mean duration of follow-up was 17.2 months (range: 12-31 months). All the fractures were classified according to the AO/ASIF systems. The operative time, the average number of days of hospitalization, the amount of bleeding, the incidence of complications, the union time as assessed on radiologic examinations, the tip apex distance (TAD), the outcome according to the Cleveland index, the change of the neck shaft angle and the amount of sliding at the end of follow-up were compared between the two groups. The clinical outcomes were compared according to the mobility score of Parker, Palmer, Jensen. The results were analyzed using the Student T-test and chi-square tests. RESULTS: There was no significant difference in blood loss during surgery, the number of hospitalization days, radiographic bone union and TAD (p>0.05). Compared with PFN, the operation time, the postoperative sliding and the neck shaft angle change were significantly less in the PFNA group (p<0.05). For the postoperative complications, there was 1 case of cutting out and 1 case of superficial infection in the PFNA group, and there were 4 cases of cutting out, 2 cases of back out, 2 cases of varus collapse, 1 case of nonunion and 1 case of superficial infection in the PFN group. CONCLUSION: Using the PFNA had relatively satisfying clinical results for the treatment of intertrochanteric fracture, as compared with those of using PFN.
Follow-Up Studies ; Hemorrhage ; Hip Fractures ; Hospitalization ; Humans ; Incidence ; Nails ; Neck ; Operative Time ; Postoperative Complications

A 60-year-old healthy man was consulted with dark brown eschars and purulent discharges on the grafted skin of a burn scar on the posterior aspect of both lower extremities which had developed two weeks after the skin graft. Aspergillus fumigatus was isolated by repeated fungal cultures from pus and tissue pieces. Also septate hyphae branching at an acute angle are seen in the dermis in PAS-stained section. After two weeks therapy with fluconazole and itraconazole, the patient accepted skin graft again. The skin lesion was improved after prophylactic itraconazole treatment for three weeks.
Aspergillosis* ; Aspergillus fumigatus ; Burns* ; Cicatrix ; Dermis ; Fluconazole ; Humans ; Hyphae ; Itraconazole ; Lower Extremity ; Middle Aged ; Skin* ; Suppuration ; Transplants*

A 61-year-old healthy breeder presented with three punched out, purulent ulcers surrounded by erythematous indurated plaque on the burn scars of the dorsum of the right upper arm and hand which were developed at six months prior to visit. Aspergillus niger was isolated from the pus and tissue pieces in three consecutive cultures. After eight weeks therapy with oral itraconazole, purulent ulcers were healed leaving fibrotic scars. We report a rare case of primary cutaneous aspergillosis by A. niger in a healthy man.
Arm ; Aspergillosis ; Aspergillus niger* ; Aspergillus* ; Burns* ; Cicatrix* ; Hand ; Humans ; Itraconazole ; Middle Aged ; Niger ; Suppuration ; Ulcer

Pilomatricoma is a tumor with differentiation toward hair cortex cells. There have been a few cases of multiple familial pilomatricomas in association with myotonic muscular dystrophy. We report two members of the family(brother and sister) with association of multiple pilomatricomas and myotonic muscular dystrophy. Multiple familial pilomatricomas may be a cutaneous manifestation of the myotonic syndrome.
Hair ; Humans ; Muscular Dystrophies* ; Pilomatrixoma* ; Siblings*

PURPOSE: Return of spontaneous circulation (ROSC) is closely related to a favorable treatment outcome in out-of-hospital cardiac arrest and is also a reliable treatment target in clinical trials. The aim of this study is the identification of major factors affecting field ROSC by analyzing the updated data encompassing the pre-hospital treatment processes. METHODS: This is a retrospective review of cardiopulmonary resuscitation (CPR) cases performed by 119 rescuers before hospital arrival from January 2012 to December 2014 in one province. Cases with traumatic cardiac arrest, unnecessary CPR, arrest occurred during transport, under age 14 years, and incomplete medical records were excluded. RESULTS: Of 1,832 patients enrolled in the study, ROSC was achieved in 99 cases (5.4%). Among them, 50 (50.5%) had ROSC at the event field and others achieved ROSC during the transport process, respectively. Total cases were divided into two groups based on ROSC before arrival at the hospital. Age, underlying cardiovascular illnesses, the event place, presence of a witness, bystander CPR, application of an automated external defibrillator (AED), the initial shockable rhythm, defibrillation, advanced airway, hydration, the arrival time of rescuer CPR, application time of AED, and defibrillation showed significant statistical differences. In multivariate analysis, age, defibrilOf 1,832 patients enrolled in the study, ROSC was achieved in 99 cases (5.4%). Among them, 50 (50.5%) had ROSC at the event field and others achieved ROSC during the transport process, respectively. Total cases were divided into two groups based on ROSC before arrival at the hospital. Age, underlying cardiovascular illnesses, the event place, presence of a witness, bystander CPR, application of an automated external defibrillator (AED), the initial shockable rhythm, defibrillation, advanced airway, hydration, the arrival time of rescuer CPR, application time of AED, and defibrillation showed significant statistical differences. In multivariate analysis, age, defibrillation, hydration, bystander CPR, and the call-to-rescuer CPR interval appeared to be correlated with field ROSC. CONCLUSION: To improve the survival rate through field ROSC, a public campaign to improve bystander CPR, prompt recognition of cardiac arrest, and rapid application of pre-hospital treatment and political support of the public institution are mandatory.
Cardiopulmonary Resuscitation ; Defibrillators ; Emergency Medical Services ; Heart Arrest ; Humans ; Medical Records ; Multivariate Analysis ; Out-of-Hospital Cardiac Arrest* ; Retrospective Studies ; Survival Rate ; Treatment Outcome

Creeping eruption is a syndrome characterized by the penetration in human skin of larvae from hookworms of various animals, mainly cats and dogs. A 26-year-old male presents with pruritic erythematous serpiginous linear skin lesion on the left lower leg for 2 weeks. The skin lesion was developed after walking in bare feet along the beach of an island in Philippines. Histopathologic finding showed a burrow containing numerous eosinophils and a few mononuclear cells in the epidermis. No parasite was found in multiple histologic section.
Adult ; Ancylostomatoidea ; Animals ; Cats ; Dogs ; Eosinophils ; Epidermis ; Foot ; Humans ; Larva ; Larva Migrans* ; Leg ; Male ; Parasites ; Philippines ; Skin ; Walking

Mesenchymal chondrosarcoma is a rare tumor occurring in both bone and soft tissues and exhibits characteristic of a malignant nature. The authors experienced a case of mesenchymal chondrosarcoma occurring in a 23-year-old woman which had invaded the cervical spine. The patient presented with severe both shoulder pain, left upper extremity weakness(Grade IV) and paresthesia at admission. Radiologic studies of the cervial spine showed an aggressive osteolysis of C4 vertebral body, pedicle and lamina with compression of the spinal cord posteriorly on C3, C4, C5 levels. The tumor was totally removed by a combined anterior and posterior approach. The removed vertebral body was replaced with autogenous bone and stabilized by Codman locking plate symtem. The pathological examination showed characteristic of mesenchymal chondrosarcoma.previous symptoms well improved postoperatively. The authors present a case of mesenchymal chondrosarcoma with review of literature.
Chondrosarcoma, Mesenchymal* ; Female ; Humans ; Osteolysis ; Paresthesia ; Shoulder Pain ; Spinal Cord ; Spine* ; Upper Extremity ; Young Adult