Kornel Terplan

Fulminating Gastroenterocolitis caused by Staphylococci. Its Apparent Connection with Antibiotic Medication.

Summary Detailed anatomic reports are presented of 8 cases in which various major surgical operations were performed, accompanied by administration of several broad spectrum antibiotics, including terramycin, Syncrobin (penicillin and streptomycin), and aureomycin. Dosages, which were in general comparatively small, and the combinations of the antibiotics used in some individual cases are presented in Table I. The clinical picture was characterized by nausea, vomiting, and a few loose stools, commencing from the first to the fourth postoperative day and changing rapidly into massive diarrheas, leading to a severe shock-like state. Death occurred from the third to the eleventh postoperative day. The causative agent responsible for the acute inflammation of the gastrointestinal tract was presented by large masses of staphylococci within the desquamated or loosely adherent pseudomembranous exudate covering the mucous coat, particularly in the small intestine (see Fig. 2 to 4). The recent inflammatory reaction was in general restricted to mucosa and submucosa. In five cases, colonies of coagulase positive hemolytic staphylococci were recovered from the intestinal tract and/or from the lungs. The clinical course and pathologic-anatomic findings, based in particular on bacteria stains of the tissue sections from the gastrointestinal tract and including the recent degenerative changes in the adrenals and kidneys, point to a severely toxic state from massive staphylococcic infection of the gastrointestinal tract, but not to a septicemic process. The staphylococci grown on blood agar in the few instances tested were resistant against all the antibiotics used in these cases. In three recent observations, not reported in detail, the same clinicopathologic syndrome was present, with the same rapid fatal outcome and the same pathologic, histologic, bacterioscopic and cultural findings. The staphylococci grown from the intestinal tract and from the lungs in these cases were sensitive only to Erythromycin, but not to the antibiotics administered.

Intraventricular Hemorrhage in Full-term Neonates

The neuropathological and general pathological features of intraventricular hemorrhage are described in 32 neonates who had been born at term. Although the most common site of bleeding was the choroid plexus, a significant minority occurred in the subependymal germinal matrix. Associated neuropathological findings included meningeal venous congestion, periventricular white‐matter necrosis and subarachnoid hemorrhage. Pulmonary hemorrhages, hyaline membranes and congenital heart‐defects were common. Most of the infants had low Apgar scores at birth and lived for less than two days. Significant neurological findings included hypotonia, hyporeflexia, decreased activity level and seizures.

Correlating auditory evoked and brainstem histologic abnormalities in infantile Gaucher's disease

In an infant with Gauchers disease, brainstem auditory evoked responses were abnormal on three separate occasions. Only waves I and II were typically present at standard intensity settings, although a third peak was elicited at 88 dB. Histologic studies demonstrated absence of neurons in the cochlear nuclei and a hypoplastic superior olivary complex. This neurophysiologic-pathologic correlation supports contentions that both brainstem auditory evoked response (BAER) waves I and II arise from the auditory nerve and that it may be capable of generating additional peaks.

Brain changes in newborns, infants and children with congenital heart disease in association with cardiac surgery. Additional observations

SummaryThe histopathologic pattern in the Central Nervous System in 12 out of 21 new-borns and young infants, below 2 months of age, operated for various congenital heart defects, is that of recent necroses in the deep and subcortical white matter of the pallium, seen from a few hours to 8 days following completion of surgery. Type and location of these lesions of ischemic coagulative necrosis, resembling findings in the perinatal respiratory distress syndrome, seem to depend on shock-like states, the low output syndrome of the left heart with hypovolemia and hypotension. The cause of death in all these infants was cardio-respiratory failure. The question to what extent glial scars replacing necroses within the mostly nonmyelinated fiber systems of the pallium will lead to defective maturation of the brain in later infancy and childhood remains a challenge for neurologic and psychological investigation, even if the heart defect has been successfully repaired by the surgeon.Post-mortem data of the CNS in 24 older infants and children below 9 years of age in association with cardia surgery include thrombembolic infarctions, also, after catheterization alone, diffuse or segmental cortical necroses, and cortical scars, especially in children older than 1 year, confirming data recently reported from this laboratory.ZusammenfassungIn 12 von 21 Neugeborenen und 1–2 Monate alten Säuglingen mit angeborenen Herzfehlern fanden sich frische Coagulationsnekrosen im weißen Mark des Großhirns nach Herzoperationen, bei Überlebensdauer von wenigen Stunden bis zu 8 Tagen. Das histologische Bild ist ähnlich dem bei respiratorischen Schwierigkeiten bei der Frühgeburt, wenn auch gewöhnlich weiter ausgebreitet, und ist pathogenetisch bedingt durch ischämische Störungen im Gefolge von Schock mit Verringerung des Blutauswurfs vom linken Herzen (low output syndrome) und arterieller Hypotension. Die Todesursache bei allen 21 Neugeborenen und Säuglingen war Versagen der Herz-Lungen-Funktion mit Ödem, Blutungen und Atelektasen der Lungen. Die Frage, ob und wieweit die gliösen Vernarbungen im noch nicht myelinisierten Mark die Hirnreifung in der späteren Säuglings- und Kleinkinderperiode beeinträchtigen, erfordert sorgfältige neurologische und psychologische Untersuchung aller jungen herzoperierten Säuglinge, auch wenn der kongenitale Herzdefekt erfolgreich operativ beseitig wurde.Die Hirnbefunde im Anschluß an Operationen angeborener Herzfehler von 24 älteren Säuglingen und Kindern unter 9 Jahren bestätigen die Ergenisse früherer Untersuchungen an 200 Kindern und Jugendlichen, besonders bezüglich der Häufigkeit von embolischen Infarkten — auch nach Katheterisierung allein —, bandartiger oder diffuser corticaler Nekrosen und einzelner Rindennarben, dies besonders bei Kindern, die älter als 1 Jahr waren, und nach wiederholten Herzeingriffen.

Histopathology of the nervous system in carnosinase enzyme deficiency with mental retardation

A few years ago, Perry e t al.’ reported a heretofore unknown metabolic disorder associated with progressive motor dysfunction, mental retardation, deafness, and blindness in two siblings who had appeared normal during the first few months of life. In the course of their physical and mental deterioration, which h a d b e e n initiated by seizures, increased amounts of carnosine were found in the urine and an elevated carnosine concentration in the serum and a deficiency of serum carnosinase activity were demonstrated.2 These higherthan-normal levels of serum carnosine persisted even when the children were on diets free of meat products. Recently, two physically and mentally retarded boys were observed in the follow-up studies of infants born a t the Children’s Hospital (in the Child Development Program) with a disease pattern similar t o that described by Perry e t al. The boys were severely mentally retarded; however, their younger sister appeared normal. In all three siblings carnosinuria, carnosinemia, and serum carnosinase deficiency were demonstrated. Extracts of liver, kidney, and spleen obtained after death of the older boy were examined by electrophoresis. There was only one of the two forms of carnosinase normally present in these tissues. In the sera of the parents carnosinase activity was detectable but a t a lower level than normal. (A detailed account of the methods used for the assay of the enzyme carnosinase will be given elsehere.^) We shall report briefly the clinical history of

Abnormal Cerebral Cortical Neurons in a Child With Maternal PKU Syndrome

A child born to a phenylketonuric (PKU) woman not on dietary treatment was microcephalic and had congenital heart and other physical anomalies consistent with the maternal PKU syndrome. After a repeat cardiac catherization at 3 months of age, he suffered an embolic left cerebral infarct and seizures. He was found by his mother dead in bed at 4 months of age; general autopsy revealed Klebsiella sepsis and pneumonia. Neuropathologic studies of the right cerebral hemisphere showed neuronal loss of the third cortical layer; Golgi studies revealed persistently immature cortical pyramidal cell somata and dendritic spines. This is the first report of specific neuronal abnormalities in a child with the maternal PKU syndrome and may, in part, account for the clinical features of microcephaly, mental retardation, and seizures seen in affected children. (J Child Neurol 1987;2:201-204).