Hereditary leukoencephalopathy and palmoplantar keratoderma: a new disorder with increased skin collagen content.

We report a new neurocutaneous syndrome of apparent autosomal recessive inheritance consisting of early-childhood-onset palmoplantar keratoderma followed in adulthood by progressive tetrapyramidal syndrome and cognitive impairment. Of the four affected siblings, two were available for evaluation. Investigation disclosed cerebral white-matter involvement on MRI and arylsulfatase A pseudodeficiency carrier state, which was also identified in clinically unaffected family members. Since skin biopsies showed dermal connective tissue abnormalities, we studied collagens I, III, and VI biosynthesis. Northern blotting of RNA extracted from cultured skin fibroblasts revealed an increased steady-state messenger RNA (mRNA) level of alpha 1(VI) collagen, whereas no differences were detected for pro alpha 1(I), pro alpha 1(III), and tropoelastin mRNAs. The skin content of collagen and total protein was higher in the patients than in controls. We suggest that an extracellular matrix abnormality may be involved in the pathogenesis of this disorder.

Aminophylline versus doxapram in idiopathic apnea of prematurity: a double-blind controlled study.

A double-blind controlled study, in two parts, was undertaken to compare the effectiveness of aminophylline and doxapram therapy in idiopathic apnea of prematurity. In the first part of this study, eight of 15 infants responded to doxapram therapy with complete cessation of apneic spells and six of 11 infants responded similarly to administration of aminophylline. These differences were statistically insignificant. In the second part of the study, assessment was made of whether the addition of doxapram to aminophylline therapy was effective in treatment of apnea of prematurity that had been unresponsive to aminophylline alone. Of ten infants who continued to have apneic spells during treatment with aminophylline, eight responded to the addition of doxapram with complete cessation of apnea. Nine infants received placebo in addition to aminophylline, and none had a reduction in frequency of apnea.

Efficacy of doxycycline and tetracycline in ocular rosacea.

We compared the effects of doxycycline and tetracycline hydrochloride on the subjective symptoms in ocular rosacea. Twenty-four patients with symptomatic ocular rosacea were randomly assigned to two groups and treated with doxycycline 100 mg/day (group 1, 16 patients) or tetracycline hydrochloride 1 g/day (group 2, eight patients). The dosages of each drug were gradually tapered and discontinued according to symptomatic response. At each examination all the manifesting symptoms were scored by the patients. Patients were followed up from six weeks to three years. After six weeks of drug treatment, all patients except one had symptomatic improvement. Although most of the scores of the symptoms were significantly decreased in both groups, greater symptomatic relief occurred in the tetracycline hydrochloride-treated patients (P = .041). However, after three months of treatment there was no significant difference in symptoms between the two groups. Gastrointestinal tract complications occurred in two of the 16 patients (12.5%) in group 1 and in three of the eight patients (37.5%) in group 2. Both tetracycline hydrochloride and doxycycline can control the symptoms of ocular rosacea.

Normalization of the dexamethasone suppression test at discharge from hospital. Its prognostic value.

Fourteen patients with RDC diagnosis of primary, endogenous, major depressive disorder were studied in an inpatient setting. All were non-suppressors on the Dexamethasone Suppression Test on admission to hospital and were retested at discharge. Over 70% of patients continued to be non-suppressors at discharge, when in clinical remission. Four out of 14 patients converted to normal suppression. All 10 of the non-normalizers did poorly on follow-up: 3 patients committed suicide. All normalizers did well. Non-normalization of the DST at discharge from hospitalization may be more common than previously suspected and predicts poor clinical outcome.

The use of doxycycline and tetracycline in ocular rosacea.

The authors compared the effects of doxycycline and tetracycline hydrochloride on subjective symptoms in ocular rosacea. Twenty-four patients with symptomatic ocular rosacea were prospectively treated with doxycycline 100 mg/day (group 1, n=16) or tetracycline hydrochloride 1g/day (group 2, n=8). The dosages of each drug were gradually tapered and discontinued according to symptomatic response. Patients were followed from six weeks to three years. In group 1, 15 patients (94%) had symptomatic improvement (12 patients were asymptomatic and three symptomatically improved), and two patients (13%) had gastrointestinal tract (GIT) complications followed by discontinuation of medication. In group 2, seven of the eight patients (87%) were asymptomatic and one patient (13%) had symptomatic improvement; three patients discontinued the use of tetracycline as a result of GIT complaints. This study indicates that both tetracycline hydrochloride and doxycycline can control the symptoms of ocular rosacea.

Plasma ascorbic acid levels in premature infants.

During gestation the fetus is abundantly provided with vitamin C, but inadequate postnatal provision of the vitamin may lead to early hypoascorbemia. Since premature newborns are often prevented from full alimentation for a considerable length of time, ascorbate deficiency may result. Plasma ascorbate levels of 20 premature newborns were determined during the first two weeks of life. Daily ascorbate intake of less than 5 mg/kg b. w. resulted in a decline of plasma ascorbate level from 1.56 +/- 0.15 mg/100 ml (mean +/- SEM) on the first day of life to a low level of 0.48 +/- 0.05 mg/100 ml by two weeks of age. An increase of daily ascorbate intake to 5--10 mg/kg b.w. resulted in a higher plasma concentration of 0.79 +/- 0.09 mg/100 ml (p less than 0.02) while parenteral supplementation of 50 mg vitamin C daily provided for a high plasma concentration of 2.7 +/- 0.46 mg/100 ml (p less than 0.005) when compared with the other two groups. Maturational and environmental factors may impose a higher demand for vitamin C during early premature life and therefore adequate amounts of the vitamin should be early provided.

Basal cell carcinoma developing in a nevus flammeus.

A 44-year-old man with Sturge-Weber syndrome developed a basal cell carcinoma at the site of a nevus flammeus on his right cheek, which had been treated with a course of grenz rays at six years of age without obvious improvement. He also exhibited erythematous papulovesicular lesions on the upper trunk, which histologically proved to be transient acantholytic dermatosis. The association of nevus flammeus and basal cell carcinoma is extremely rare; to the best of our knowledge, only four similar cases have been previously reported.

[Pharmacists graduated in the first twenty years on the medical faculty of Hungarian Royal University]. / A Magyar Királyi Tudományegyetem orvosi karának elsö húsz évében végzett gyógyszerészek.

The present paper, summarizing the results of original archival researches, gives a survey on the beginnings of the pharmaceutical education in Hungary at the end of the 18th century. While analysing the documents the author calls our attention to some stubborn errors and mistakes of the former historical literature as well. The paper-based on the data of the promotional book-presents a list including names, nationality and religion of those 170 students of pharmacy, who completed their studies during the first 20 years of pharmaceutical education in Hungary. Comparing the dates of tirocinial and those of university examinations respectively, the author proves that sometimes even 40 or 59 years old, respected pharmacists were forced to take their exams at the university. Having made a comparison between the register of students and that of promotions the author concludes that until 1788 the studies took three months or a year only in theory, for candidates actually did not attend university courses at all. In the case when one failed, a short preparation for a make-up exam was also available. Data prove, that the requirements of the royal decree, a centrally made regulation, called Normativum Sanitatis - in spite of the difficulties caused e.g. by the lack of handbooks - could be more or less responded. (The circumstances of the courses are going to be analysed in a further paper.)

Necrotising enterocolitis in the very low birthweight infant: expressed breast milk feeding compared with parenteral feeding.

The incidence of necrotising enterocolitis (NEC) in very low birthweight infants (VLBW less than or equal to 1500 g) was reduced by the delayed onset of enteral feeding. Eight (18%) out of 44 VLBW infants who were in hospital during the first year of the study developed NEC. During the next 12 months 85 similar infants were initially fed by parenteral nutrition only, and then from age 14-21 days with infant formula. During the second year only 3 (3%) patients developed NEC. There were no other relevant changes in management. Throughout the entire study, the onset of NEC in each infant in whom it occurred was after the start of enteral feeding. We recommend avoiding enteral feeding in VLBW infants during the period that they are particularly vulnerable--namely the first 2 or 3 weeks of life.