Biomedical subjects
Publications and source records attributed to H A Cohen.
During a mumps outbreak between 1987 and 1988, six children presented with symptoms of acute cerebellar involvement as the sole neurologic complication. The neurologic symptoms followed parotitis and lasted for up to 3 weeks. Serologic evidence was suggestive of recent mumps infection. Symptoms spontaneously resolved in all patients without any residual signs or symptoms during 2- to 2.5-year follow-up.
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A recessively inherited syndrome of blepharophimosis and ptosis with weakness of extraocular and frontal muscles is reported in six members of three related kindreds. Prognathism, synophrys and thick eyebrows added to a typical facial appearance. Additional findings included short stature, borderline head circumference and toe syndactyly. Borderline mental retardation and anosmia were found in one patient. The clinical features and the mode of inheritance distinguish this syndrome from other blepharophimosis and ptosis syndromes.
Recurrent parotitis in children is a well described but rare condition of unknown cause. The clinical features of 11 children with recurrent parotitis are described.
The possible association of urinary tract infection (UTI) with ritual circumcision on the eighth day of life was studied by analyzing the epidemiology of urinary tract infections during the first year of life in 169 children with UTI (56 males and 113 females) born in Israel from 1979 to 1984. Forty-eight percent of the episodes of UTI occurring in males appeared during the 12 days following circumcision, and the increased incidence during that period was highly significant. The median age of the males at the time of the UTI was 16 days, compared with seven months in females. Ritual Jewish circumcision as practiced in Israel may be a predisposing factor for UTI during the 12-day period following that procedure.
OBJECTIVE: To report two cases of fixed drug eruption induced by methylphenidate. CASE SUMMARY: Two children with attention deficit disorder treated with methylphenidate as a simple drug developed fixed drug eruption of the scrotum. Drug discontinuation was followed by a complete resolution of the skin eruption. Rechallenge resulted in the same drug rash. Macrophage migration-inhibiting factor (MIF) assay with methylphenidate was positive. DISCUSSION: The pathogenesis of fixed drug eruption and the role of MIF assay in the diagnosis of adverse drug reaction is discussed. CONCLUSIONS: Fixed drug rash induced by methylphenidate is a possible but rare phenomenon.
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Two cousins and an unrelated patient, all offspring of consanguineous parents, presented with Peters anomaly, unusual facial appearance, disproportionate short stature, retarded skeletal maturation, and a variable degree of mental retardation. Variable digital, cardiac, CNS, and urogenital anomalies were present. The inheritance is probably autosomal recessive. The condition is a distinct clinical entity for which we suggest the eponym Krause-Kivlin syndrome. Peters anomaly is thought to result from abnormal migration of neural crest cells. A similar mechanism was implicated in the pathogenesis of other disorders of the anterior chamber. The presence of Peters anomaly, and possibly of other corneal endothelial disorders in a newborn infant, should alert the clinician to the possibility of this syndrome. Communicating hydrocephalus (or brain atrophy) and polyhydramnios were documented in two patients, potentially allowing prenatal diagnosis in secondary familial cases.
Helicobacter pylori was found in a gastric biopsy specimen of a child with protein-losing gastropathy. Through erythromycin therapy, clinical and ultrasonographic recovery was associated with the disappearance of the pathogen on repeated biopsy. The association of H. pylori with protein-losing gastropathy has been reported only once in children, to our knowledge. Although the causative correlation between the pathogen and the disease has not been proven yet, we suggest that future cases of protein-losing gastropathy be studied for the presence of H. pylori and followed up by ultrasonographic investigation and urea breath test. Appropriate treatment should be given to suspected cases.
The ability of equilibrium gated radionuclide ventriculography to detect segmental left ventricular (LV) wall motion abnormalities was determined in 26 patients undergoing cardiac catheterization. Multiple gated studies obtained in 30 degrees right anterior oblique and 45 degrees left anterior oblique projections, played back in a movie format, were compared to the corresponding LV ventriculograms. The LV wall in the two projections was divided into eight segments. Each segment was graded as normal, hypokinetic, akinetic, dyskinetic, or indeterminate. Thirteen percent of the segments in the gated images were indeterminate; 24 out of 27 of these were proximal or distal inferior wall segments. There was exact agreement in 86% of the remaining segments. The sensitivity of the radionuclide technique for detecting normal versus any abnormal wall motion was 71%, with a specificity of 99%. Equilibrium gated ventriculography is an excellent noninvasive technique for evaluating segmental LV wall motion. It is least reliable in assessing the proximal inferior wall and interventricular septum.
Monocytes derived from the peripheral blood of psoriatic patients demonstrated a significantly higher phagocytic capacity (36 to 40%) for both 125I-labeled Shigella flexneri and 125I-labeled Staphylococcus albus compared with monocytes from healthy subjects. Monocytes from psoriatic patients showed a 2-to-4fold increase in bactericidal capacity against S. albus when compared with normal monocytes. However, the bactericidal capacity of monocytes from diphylline-treated patients did not differ from that of the control subjects. The antibody-dependent cellular cytotoxicity (ADCC) activity against EL4 tumor cells was found to be similar in both psoriatic patients and control subjects. It is postulated that these abnormalities of monocyte function in psoriasis are caused by a decreased cAMP/cGMP ratio similar to the decreased cAMP/cGMP ratio found in the lesional epidermis of this disease. It seems therefore, that the psoriatic abnormality is not confined to only one type of cell, the epidermal cell.
6 patients with mycosis fungoides in different stages of the disease were successfully treated with intralesional injections of 1% mineral oil emulsions of killed BCG and cord factor, and/or topical application of an ointment containing killed BCG and cord factor.
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Herpes simplex virus type 2 was isolated and identified from the vesicular border of pyoderma gangrenosum lesions on the genital region of a patient with chronic lymphatic leukemia. Dramatic relief of pain as well as quick disappearance of the vesicular margin of the lesions and of the inflammatory halo around them occurred as a result of local treatment with a solution of zinc acetate. A careful search for a viral agent should be done in every case of pyoderma gangrenosum occurring in a patient with a hematological malignancy or/and impaired immunity, especially if the lesions are on the face or in the genital region.
The delayed hypersensitivity against the leishmania parasite was restored to a patient who had suffered from diffuse cutaneous leishmaniasis (DCL) for 26 years, by the application of an ointment containing heat-killed and lyophilized BCG and cord-factor (trehalose-6-6'-dimycolate) after stripping the affected and adjacent areas with scotch-tape.
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A patient, suffering for 42 years from the late tuberculoid-type of leishmaniasis located in his face, was successfully treated with intralesional injections of emetine hydrochloride. Previous treatments, which included intralesional injections of steroids and concomitant intramuscular injections of antimonials, flagyl, fluorocytosine, infusions of amphotericin B--with and without concomitant treatment by steroids systemically and/or intralesionally--and amphotericin B intralesionally, were altogether ineffective. In addition, the patient underwent five operations in a plastic surgery department.