Neonatal diabetes mellitus (NDM) is defined as hyperglycemia that presents clinical symptoms within the first month of life and requires insulin therapy to maintain euglycemia. The diabetic condition may be transient, permanent, or transient with recurrence later in life. NDM is a rare clinical disorder. Three cases of NDM have been reported in Korea so far. We experienced two cases of NDM in twin brothers who were born with small for gestational ages. Their HLA typings were DR9 and DR14, and insulin autoantibody, islet cell antibody, GAD-reactive autoantibody, and insulin receptor antibody were not found. The genetic analysis with polymorphic DNA markers for chromosome 6 indicated paternal uniparental isodisomy.
Chromosomes, Human, Pair 6 ; Diabetes Mellitus* ; Genetic Markers ; Gestational Age ; Histocompatibility Testing ; Humans ; Hyperglycemia ; Insulin ; Islets of Langerhans ; Korea ; Receptor, Insulin ; Recurrence ; Siblings* ; Twins* ; Uniparental Disomy

No abstract available.
Animals ; Rats* ; Vitamin A* ; Vitamins* ; Wounds and Injuries*

Multicentric reticulohistiocytosis (MRH) is a rare multisystemic granulomatous disease of unknown cause, characterized by severe debilitating polyarthritis and papular to nodular mucocutaneous lesions. This disease usually manifests as a progressive, destructive polyarthritis with mucocutaneous eruptions consisting of erythematous to brownish-colored papules and nodules. MRH is important, not only because of its disfiguring and disabling manifestations and systemic involvements, but also due to its association with internal malignancy. The histological features of skin and synovial lesions are infiltration of multinucleated giant cells, and histiocytes which contain abundant eosinophilic cytoplasm and have a ground glass appearance. Without the accompanying skin nodules, the patients can be misdiagnosed as having rheumatoid arthritis or psoriatic arthritis, and this inaccurate diagnosis will affect treatment. We report a case of MRH which was misdiagnosed as rheumatoid arthritis.
Arthritis ; Arthritis, Psoriatic ; Arthritis, Rheumatoid* ; Cytoplasm ; Diagnosis ; Eosinophils ; Giant Cells ; Glass ; Histiocytes ; Humans ; Skin

Signet-ring cells are cells in which the nucleus is crescentically compressed with a cellular border, so that the cell looks like a signet-ring. Many cutaneous signet-ring cell neoplasms originate from the stomach, lung and breast, and these appear to be the most common sites of origin. In skin, the appearance of signet-ring cells within a neoplasm can indicate metastatic adenocarcinoma of the stomach, lung and breast. Thus, the primary origin and other metastatic sites should be evaluated. A 44-year-old man presented with a six month history of cutaneous swelling on the left axillary and left supraclavicular area. Histopathologic findings of the axillary lesion showed signet-ring cells in the lymphatics. These clustered cells were PAS-positive, diastase-resistant, and stained with colloidal iron and CEA. During the work-up study to find the primary origin, we discovered advanced gastric cancer and multiple lymph node metastases.
Adenocarcinoma* ; Adult ; Breast ; Colloids ; Edema* ; Humans ; Iron ; Lung ; Lymph Nodes ; Neoplasm Metastasis ; Skin ; Stomach ; Stomach Neoplasms

Inguinal hernia repair can result in paresthesia and/or pain in the inguinal region. Pharmacological and surgical management often yield inconsistent results associated with considerable risks and side effects. Radiofrequency thermocoagulation (RF) is a neuro-destructive treatment for severe pain, but associated with hypoesthesia, neuritis-like reactions, and occasional neuroma formation. Pulsed radiofrequency (PRF), unlike RF, delivers high intensity currents in pulses, is non-neurodestructive, and therefore less painful, without the potential complications. Here we report on PRF in chronic postoperative inguinal pain. A 23-year-old male who received right inguinal hernia repair and complained of right sided groin pain for approximately 10 years underwent PRF at the L1 and L2 dorsal root ganglia (DRG). He then reported a decrease in pain from 80-90/100 mm to 15-30/100 mm on a visual analogue scale (VAS), which lasted for twelve months.
Electrocoagulation ; Ganglia, Spinal* ; Groin ; Hernia, Inguinal ; Herniorrhaphy* ; Humans ; Hypesthesia ; Male ; Neuroma ; Paresthesia ; Spinal Nerve Roots* ; Young Adult

No abstract available.
Toluene*

Calvus, callus, plantar warts and piezogenic papules are well known to cause discomfort and disturbance of gait in ordinary physical and sports activities. Furthermore, calcifying aponeurotic fibroma, whose mass slowly grows on the palms and soles of children and young adults, leads to pain, disturbance of gait, and even to skeletal deformities. A 22-year-old woman noticed an elevated subcutaneous mass on the left plantar surface of her big toe, which was accompanied with pain, tenderness and gait disturbance. The histologic findings showed an ill-circumscribed fibroblastic proliferation with foci of calcification and chondroid differentiation. After total excision of the mass, the patient became free of the above symptoms and the lesion did not recur for 9 months.
Bony Callus ; Child ; Congenital Abnormalities ; Female ; Fibroblasts ; Fibroma* ; Gait* ; Humans ; Sports ; Toes ; Warts ; Young Adult

Lentigo maligna melanoma and lentigo maligna are typically located on photo-exposed sites such as the head and neck, with the cheek being the most common site. Since lentigo maligna melanoma and lentigo maligna are found predominantly on such cosmetically-sensitive areas, it is critical to determine the exact histologic margin for maximal sparing of tissue and complete extirpation of the tumor. Since Mohs micrographic surgery has been recommended for tumors on cosmetically-sensitive locations where the tumor margin is indistinct, it appears to be a reasonable treatment modality for this type of tumor. We describe a case of lentigo maligna melanoma on the cheek, which was resected with Mohs micrographic surgery.
Cheek ; Head ; Hutchinson's Melanotic Freckle* ; Lentigo* ; Melanoma* ; Mohs Surgery* ; Neck

This experiment was designed for the elucidation of relationships between the cell adhesion molecules and synchronous beating rates of cardiomyocytes isolated from ventricles of 3-day-old rats. These cells were grown on the culture vessel coated or non-coated with cardiogel for 1, 3, 5, and 7 days. The synchronous beating rate and morphologic changes of cells were investigated under the inverted microscope. Those changes of cardiomyocytes were observed by transmission elcectron microscopy (TEM). Connexin43, pan-cadherin, and alpha-sarcomeric actin were stained by indirect immunofluorescent (IF) technique. Western blotting were used for identifying unique bands of connexin43 and pan-cadherin in the regions of specific size. The synchronous beating numbers of cardiomyocytes in each group on the dish coated or non-coated with cardiogel were significantly different for 3, 5, and 7 days in culture. The maximum values of synchronized beating in the cells appeared on the day 5. The beating numbers of cells grown on coated dish comparing with non-coated dish were significantly increased on the day 5 and day 7. The proliferation of cardiomyocytes increased markedly in the cardiogel-coated dish on the day 5, while the number of fibroblasts in non-coated dish were obviously increased on the day 7. In indirect IF studies, a normal redistribution of connexin43, pan-cadherin, and alpha-sarcomeric actin of the cells was expressed in day 3 throughout day 5. The reaction intensity of those proteins were increased or decreased in the proportion to the beating numbers. The lack of reaction was appeared in the fibroblasts consisting of monolayer on the day 7. Unique bands of connexin43 and pan-cadherin having a specific size were marked on Western Blotting. The structures such as gap junction, fascia adherence and desmosome compatible with intercellular adhesion in cardiac muscle were abundant on day 3 to 7. In conclusion, the amount or reaction intensity of connexin43 and pan-cadherin in cardiomyocytes were stronger simultaneously with the increase of synchronous beating numbers, particularly for 3 and 5 days. The maximum beating rates of cardiomyocytes reached on the day 5, while the maximum beating rates of them were markedly decreased owing to the proliferation of fibroblasts on day 7. TEM findings consisting of intercalated discs might be explained regardless of coating with cardiogel on why the close relationships between the beating rates and the connexin43 and pan-cadherin of those cells in culture exist.
Actins ; Animals ; Blotting, Western ; Cell Adhesion Molecules ; Connexin 43 ; Desmosomes ; Fascia ; Fibroblasts ; Gap Junctions ; Microscopy ; Myocardium ; Myocytes, Cardiac* ; Rats

Opioids are generally used to treat severe cancer pain. Usually, it is common to increase the dose of opioids to maintain analgesia. Opioid-induced hyperalgesia (OIH) is a paradoxical response to opioid resulting in increased perception of pain rather than antinociceptive effect. A 64-year-old female with pancreatic cancer was suffering from whole abdominal pain. She took massive opioid therapy, however, her pain had been worse and widen in the 3 months. Radiologic imaging was performed to exclude metastatic cancer. The result was negative. We suspected OIH, and reduced the amount of opioids, then, added to adjuvant analgesics. And also we performed celiac plexus neurolysis with the use of alcohol and continuous epidural catheter insertion. Her numeric rating pain scale (NRS) decreased from 9/10 to 3/10. This case suggests that adjuvant analgesics and interventional treatments can resolve a OIH patient with intractable cancer pain.
Abdominal Pain ; Analgesia ; Analgesics ; Analgesics, Opioid ; Catheters ; Celiac Plexus ; Female ; Humans ; Hyperalgesia ; Middle Aged ; Pain Management ; Pancreatic Neoplasms ; Stress, Psychological