Sclerema neonatorum is an uncommon and fatal disease which encountered in premature & debilitated newborn infants. Recently we experienced a case of sclerema neonatorum in premature infant with congenital heart disease which was confirmed by autopsy. We present a case with a brief review of literatures.
Autopsy ; Heart Defects, Congenital ; Humans ; Infant, Newborn ; Infant, Premature ; Sclerema Neonatorum*

A case of pycnodysostosis in 12 years old girl was presented with a brief review ofliterature. This patient was admitted to our pediatric dept. of N.P.H. with chief complaints of loss of appetite and respiratory difficulty. She was characterized by a peculiar face with anterior bossing, exophthalmos, opened anterior fontanel and coronal suture and dwarfism. So bone series was performed and showed character of pycnodysostosis such as opened anterior fontanel and coronal suture, receding mandibular angle, inoreased bone density with narrowing bone marrow and acrcosteolytica on distal phalanx. But she died on the 25 th hospital day due to chronic renal failure.
Appetite ; Bone Density ; Bone Marrow ; Child ; Cranial Fontanelles ; Dwarfism ; Exophthalmos ; Female ; Humans ; Kidney Failure, Chronic ; Pycnodysostosis* ; Sutures

BACKGROUND: Nerve conduction study is an objective and quantitative diagnostic method for the diagnosis and follow-up study of polyneuropathy. Sometimes it is hard to interpret the nerve conduction study, because there are many segmental variables to test and uneven distribution of abnormalities in polyneuropathy. And there can be some interpersonal differences in the interpreting the results. It is obvious that one objective and quantitative value that represents the many segmental variables is helpful for the clinical interpretation and follow?up of polyneuropathy. METHODS: We evaluated 242 normal subjects and 71 patients with demyelinating polyneuropathy to find out a standardized representative value, distinguishing patients from normal subjects. The standardized representative value was made by combination of the some standardized segmental variables which showed marked differences between two groups. We evaluated the clinical usefulness of this value by comparing the sequential changes of this value with the clinical course. RESULTS: Statistically significant differences were present in each segmental value of the nerve conduction study between the groups of patients and normal persons. The diagnostic sensitivity of the segmental value was in range of 25.7%-81.8% in case of 2SD criteria. The diagnostic sensitivity of each functional standardized value - average of the segmental standardized values of same nature - was in range of 54.1%-89.9% in case of 2SD criteria. Using 3SD criteria the sensitivity was reduced to 20.9%-81.1%, more in sensory and mixed nerve than motor nerve. By combination of these functional standardized values, the diagnostic sensitivity could increase up to 79.1%-93.2% on 3SD criteria. CONCLUSION: The best combination which represents the NCV study is the average of the motor nerve terminal latency, the motor nerve conduction velocity, and the ratio of conduction block with 93.2% sensitivity and 100% specificity. This representative value well reflects the clinical course of patient in follow-up studies.
Diagnosis* ; Follow-Up Studies* ; Humans ; Neural Conduction* ; Polyneuropathies* ; Sensitivity and Specificity