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We report the case of a 6-year-old male who underwent a hemicraniectomy after a right-sided middle cerebral arterial infarct. This is the youngest patient reported to have undergone this procedure for stroke-associated brain swelling. This case illustrates that hemicraniectomy for stroke can be performed safely in pediatric patients, with potential brain- and lifesaving benefits., (Copyright 2003 S. Karger AG, Basel)

Objective: Overexpression of pro-inflammatory cytokines such as tumor necrosis factor-alpha (TNF-alpha) can contribute to multiple organ dysfunction syndrome and septic shock in critically ill patients. We previously found that glutamine (GLN) can attenuate cytokine expression, induce heat shock protein 72 (HSP 72), and protect against endotoxin-induced mortality and organ injury in an in vivo rat model. However, data on the effect of GLN on direct attenuation of cytokine release and HSP 72 expression in human peripheral blood polymorphonuclear cells (PBMCs) is lacking., Methods: In this study, we assessed the effect of GLN on TNF-alpha and HSP 72 expression in human PBMCs. After treating with various doses of GLN, human PBMCs were stimulated with lipopolysaccharide (LPS). TNF-alpha release was analyzed via enzyme-linked immunosorbent assay and HSP 72 via western blot., Results: GLN at doses greater than 4 mM decreased TNF-alpha release at 4 and 24 h after LPS stimulation. Sublethal heating of PBMCs before LPS also markedly decreased TNF-alpha after LPS. Doses of GLN greater than 2 to 4 mM led to an increase in HSP 72 expression after LPS., Conclusion: These results indicate that GLN, which may improve outcomes in critically ill patients, can directly attenuate pro-inflammatory cytokine release in PBMCs. This effect may be related to enhanced HSP 72 expression.

The medical and surgical management of patients with multiple muscular ventricular septal defects (VSDs) is associated with morbidity and mortality. Three children with Swiss cheese VSDs were treated with transcatheter occlusion of their multiple defects using the Amplatzer muscular VSD occluder. Seventeen defects were closed in five catheterization procedures. One patient had three devices placed in two procedures, the second had five defects closed in one procedure, and the third had nine defects closed in two procedures. Two patients had previously been treated with pulmonary artery banding and required subsequent surgical band removal. There was immediate reduction in the left-to-right shunting and clinical improvement in all patients. Complications included the need for blood transfusion during the two longest procedures and tricuspid valve regurgitation in one. Transcatheter occlusion of multiple VSDs is an acceptable alternative or adjunct to surgical therapy for these complex patients., (Copyright 2002 Wiley-Liss, Inc.)

Recent physiological studies have implicated theta - a high-amplitude 4-8 Hz oscillation that is prominent in rat hippocampus during locomotion, orienting and other voluntary behaviors - in synaptic plasticity, information coding and the function of working memory. Intracranial recordings from human cortex have revealed evidence of high-amplitude theta oscillations throughout the brain, including the neocortex. Although its specific role is largely unknown, the observation of human theta has begun to reveal an intriguing connection between brain oscillations and cognitive processes.

Clinical trials have demonstrated that glutamine (GLN) supplementation can decrease infectious morbidity and improve survival in a number of settings of critical illness. The mechanism of this protection remains unclear. The objective of this study was to evaluate the effect of GLN on cytokine release, organ injury, and survival from endotoxin-induced septic shock. Endotoxemia was induced in Male Sprague-Dawley rats by intravenous administration of 5 mg/kg Escherichia coli lipopolysaccharide (LPS). Concomitantly, animals were fluid resuscitated with a lactated ringers (LR) solution and given GLN (0.75 g/kg i.v.) or LR alone. Blood samples were obtained at multiple time points post-LPS injury for cytokine analysis. Survival rates were monitored for 72 h. Organ injury was evaluated in a separate set of animals via pathologic exam of tissues harvested 6 h post-LPS injury. A single dose of GLN significantly attenuated the release of TNF-alpha at 2 h (P < 0.005) and IL-1 beta at 4 h (P < 0.0001). This attenuation of cytokine release was associated with a significant decrease in mortality (P < 0.003). Pathologic exam demonstrated significant protection of both lung and small bowel tissue by GLN. Blood gas values 6-h post-LPS injury showed increased PaO2 and bicarbonate concentration in GLN treated animals. These data indicate that GLN can significantly attenuate pro-inflammatory cytokine release, protect against end-organ damage, and decrease mortality from endotoxemia. GLN confers protection even when administered at the onset of endotoxemia, rather then as pre-treatment. Thus, one explanation for the clinical benefits observed from GLN-supplementation may be related to the attenuation of pro-inflammatory cytokines.

Hebbian heteroassociative learning is inherently asymmetric. Storing a forward association, from item A to item B, enables recall of B (given A), but does not permit recall of A (given B). Recurrent networks can solve this problem by associating A to B and B back to A. In these recurrent networks, the forward and backward associations can be differentially weighted to account for asymmetries in recall performance. In the special case of equal strength forward and backward weights, these recurrent networks can be modeled as a single autoassociative network where A and B are two parts of a single, stored pattern. We analyze a general, recurrent neural network model of associative memory and examine its ability to fit a rich set of experimental data on human associative learning. The model fits the data significantly better when the forward and backward storage strengths are highly correlated than when they are less correlated. This network-based analysis of associative learning supports the view that associations between symbolic elements are better conceptualized as a blending of two ideas into a single unit than as separately modifiable forward and backward associations linking representations in memory.

This study examined the association between two common polymorphisms, the dopamine D4 receptor (DRD4) gene and the serotonin transporter promoter (5-HTTLPR) gene and temperament in 61 infants aged 12 months. Twenty-two infants had a least one copy of the 6-8 repeat DRD4 alleles (L-DRD4) and 39 had two copies of the 2-5 repeat allele (S-DRD4). Twenty infants were homozygous for the short form (s/s) of 5-HTTLPR while 41 were either heterozygous for the short and the long form (l/s) or were homozygous for the long form (l/l). The infants were observed in a series of standard temperament episodes that elicited fear, anger, pleasure, interest, and activity. L-DRD4 infants showed less interest in a structured block play situation and more activity in a free play situation. They also displayed less anger in an episode of mild physical restraint. Infants with s/s 5-HTTLPR showed less fearful distress to stranger approach and less pleasure in a structured play situation than infants with l/l or l/s 5-HTTLPR. Duration of looking during block play was affected by a significant interaction between DRD4 and 5-HTTLPR. Shortest duration of looking was associated with the L-DRD4 and s/s 5-HTTLPR genotypes. The implications and limitations of these findings are discussed.

We examine how oscillations in the intracranial electroencephalogram (iEEG) relate to human maze learning. Theta- band activity (4-12 Hz in rodents; 4-8 Hz in humans) plays a significant role in memory function in rodents and in humans. Recording intracranially in humans, we have reported task-related, theta-band rhythmic activity in the raw trace during virtual maze learning and during a nonspatial working memory task. Here we analyze oscillations during virtual maze learning across a much broader range of frequencies and analyze their relationship to two task variables relevant to learning. We describe a new algorithm for detecting oscillatory episodes that takes advantage of the high signal-to-noise ratio and high temporal resolution of the iEEG. Accounting for the background power spectrum of the iEEG, the algorithm allows us to directly compare levels of oscillatory activity across frequencies within the 2- to 45-Hz band. We report that while episodes of oscillatory activity are found at various frequencies, most of the rhythmic activity during virtual maze learning occurs within the theta band. Theta oscillations are more prevalent when the task is made more difficult (manipulation of maze length). However, these oscillations do not tend to covary significantly with decision time, a good index of encoding and retrieval operations. In contrast, lower- and higher-frequency oscillations do covary with this variable. These results suggest that while human cortically recorded theta might play a role in encoding, the overall levels of theta oscillations tell us little about the immediate demands on encoding or retrieval. Finally, different patterns of oscillations may reflect distinct underlying aspects of memory function.

Enhanced expression of heat shock protein (HSP) has been shown to be protective against laboratory models of septic shock. Induction of HSPs to improve outcome in human disease has not been exploited because laboratory induction agents are themselves toxic and not clinically relevant. In this study, we demonstrate that a single dose of intravenous glutamine causes a rapid and significant increase in HSP25 and HSP72 expression in multiple organs of the unstressed Sprague-Dawley rat. With the utilization of a fluid-resuscitated rat model of endotoxemia, mortality was dramatically reduced by glutamine administration concomitant with the endotoxin injury. Endotoxin-treated animals given glutamine exhibited dramatic increases in tissue HSP expression and marked reduction of end-organ damage. These data suggest glutamine may protect against mortality and attenuate end-organ injury in endotoxemic shock via enhanced HSP expression. Furthermore, glutamine confers protection when administered at the initiation of sepsis, rather than as pretreatment. Thus glutamine appears to be a clinically viable enhancer of HSP expression and may prove beneficial in the therapy of sepsis and sepsis-induced organ injury.

Electrode grids on the cortical surface of epileptic patients provide a unique opportunity to observe brain activity with high temporal-spatial resolution and high signal-to-noise ratio during a cognitive task. Previous work showed that large-amplitude theta frequency oscillations occurred intermittently during a maze navigation task, but it was unclear whether theta related to the spatial or working memory components of the task. To determine whether theta occurs during a nonspatial task, we made recordings while subjects performed the Sternberg working memory task. Our results show event-related theta and reveal a new phenomenon, the cognitive "gating" of a brain oscillation: at many cortical sites, the amplitude of theta oscillations increased dramatically at the start of the trial, continued through all phases of the trial, including the delay period, and decreased sharply at the end. Gating could be seen in individual trials and varying the duration of the trial systematically varied the period of gating. These results suggest that theta oscillations could have an important role in organizing multi-item working memory.

The authors examined the effects of intraserial repetition on multitrial serial learning of random consonant lists, analyzing both learning rates and perfect trial interresponse times (IRTs). Lists varied along 3 dimensions: list length, presence or absence of a repeated element, and lag between repeated elements. After achieving a forward-recall criterion on a given list, participants (N = 20) attempted backward recall. At small lags, IRTs between the repeated elements were very short (compared with IRTs from identical positions in nonrepetition lists). At larger lags, the IRT to recall the second repeated item was substantially longer than in control lists. These results reveal a latency analogue of the Ranschburg pattern seen in accuracy data. A Ranschburg pattern was also found in participants' learning rates. These results both generalize the Ranschburg phenomenon and present further challenges to theories of serial order memory.

It is well known that multitrial free recall is accompanied by increased organization of output over learning trials, even when the order of presentation is randomized. We compared the relation between learning and organization in 30 young and 30 older adults as they learned categorized materials to a criterion of 100% recall. The importance of this age manipulation was that it allowed us to examine, using two groups that differ significantly in their learning ability, whether organization and learning follow the same function. As was expected, older adults showed less organization on any given learning trial. However, when equated for degree of learning, the older adults showed approximately the same level of organization as the young. This finding suggests that the organization-learning relation remains invariant in the face of significant differences in participants' mnemonic abilities.

Self-movement through an environment generates optic flow, a potential source of heading information. But it is not certain that optic flow is sufficient to support navigation, particularly navigation along complex, multi-legged paths. To address this question, we studied human participants who navigated synthetic environments with and without salient optic flow. Participants used a keyboard to control realistic simulation of self-movement through computer-rendered, synthetic environments. Because these environments comprised series of identically textured virtual corridors and intersections, participants had to build up some mental representation of the environment in order to perform. The impact of optic flow on learning was examined in two experiments. In experiment 1, participants learned to navigate multiple T-junction mazes with and without accompanying optic flow. Optic flow promoted faster learning, mainly by preventing disorientation and backtracking in the maze. In experiment 2, participants found their way around a virtual city-block environment, experiencing two different kinds of optic flow as they went. By varying the rate at which the display was updated, we created optic flow that was either fluid or choppy. Here, fluid optic flow (as compared with choppy optic flow) enabled participants to locate a remembered target position more accurately. When other cues are unavailable, optic flow can be a significant aid in wayfinding. Among other things, optic flow can facilitate path integration, which involves updating a mental representation of place by combining the trajectories of previously travelled paths [corrected].

In immediate free recall, words recalled successively tend to come from nearby serial positions. M. J. Kahana (1996) documented this effect and showed that this tendency, which the authors refer to as the lag recency effect, is well described by a variant of the search of associative memory (SAM) model (J. G. W. Raaijmakers & R. M. Shiffrin, 1980, 1981). In 2 experiments, participants performed immediate, delayed, and continuous distractor free recall under conditions designed to minimize rehearsal. The lag recency effect, previously observed in immediate free recall, was also observed in delayed and continuous distractor free recall. Although two-store memory models, such as SAM, readily account for the end-of-list recency effect in immediate free recall, and its attenuation in delayed free recall, these models fail to account for the long-term recency effect. By means of analytic simulations, the authors show that both the end of list recency effect and the lag recency effect, across all distractor conditions, can be explained by a single-store model in which context, retrieved with each recalled item, serves as a cue for subsequent recalls.

Theta oscillations (electroencephalographic activity with a frequency of 4-8 Hz) have long been implicated in spatial navigation in rodents; however, the role of theta oscillators in human spatial navigation has not been explored. Here we describe subdural recordings from epileptic patients learning to navigate computer-generated mazes. Visual inspection of the raw intracranial signal revealed striking episodes of high-amplitude slow-wave oscillations at a number of areas of the cortex, including temporal cortex. Spectral analysis showed that these oscillations were in the theta band. These episodes of theta activity, which typically last several cycles, are dependent on task characteristics. Theta oscillations occur more frequently in more complex mazes; they are also more frequent during recall trials than during learning trials.

Neonatal neurosurgery patients have specific considerations throughout the perioperative course in addition to the usual care of neonates undergoing other surgical procedures. Prematurity, with its associated comorbidity, temperature, and glucose control are important topics to consider in this age group. This article addresses practical aspects of preoperative assessment, intraoperative management, and postoperative care. Because CSF shunting and myelomeningocele repair are common neonatal procedures, these specific procedures are the focus of this article.

I present a new method for analyzing associative processes in free recall. While previous research has emphasized the prominence of semantic organization, the present method illustrates the importance of association by contiguity. This is done by examining conditional response probabilities in the output sequence. For a given item recalled, I examine the probability and latency that it follows an item from a nearby or distant input position. These conditional probabilities and latencies, plotted as a function of the lag between studied items, reveal several regularities about output order in free recall. First, subjects tend to recall items more often and more rapidly from adjacent input positions than from remote input positions. Second, subjects are about twice as likely to recall adjacent pairs in the forward than in the backward direction and are significantly faster in doing so. These effects are observed at all positions in the output sequence. The asymmetry effect is theoretically significant because, in cued recall, nearly symmetric retrieval is found at all serial positions (Kahana, 1995; Murdock, 1962). An attempt is made to fit the search of associative memory model (Raaijmakers & Shiffrin, 1980, 1981) with and without symmetric interitem associations to these data. Other models of free recall are also discussed.

Objective: To identify common characteristics among infants with breastfeeding malnutrition in a region with an increasing incidence of breastfeeding malnutrition., Design: Retrospective case series., Setting: A 361-bed regional tertiary care children's hospital in a 1.7 million population metropolitan area., Case Series: five infants with severe breastfeeding malnutrition and hypernatremia admitted to a tertiary care children's hospital over a 5-month period. Retrospective case review: 166 infants admitted between 1990 and 1994 with the diagnosis of dehydration, hypernatremia, or malnutrition., Main Outcome Measures: Maternal characteristics, age at presentation, percent loss from birth weight, serum sodium, average age at birth hospital discharge, neurologic, or cardiovascular complications., Results: Five infants were admitted to a children's hospital over a 5-month period with severe breastfeeding malnutrition and hypernatremia. The average weight loss at time of readmission was 23% (+/- 8%) from birth weight. The average presenting sodium was 186 +/- 19 mmol/L. Three suffered significant complications. From 1990 through 1994, there was a statistically significant (P < .05) annual increase in the number of infants admitted with breastfeeding malnutrition and hypernatremia., Conclusions: While breastfeeding malnutrition and hypernatremia is not a new problem, this cluster of infants represents an increase in frequency and severity of the problem and could be a consequence of several factors, including inadequate parent education about breastfeeding problems and inadequate strategies for infant follow-up.

Background: Several studies have documented cutaneous findings in neonates of various racial groups. Our purpose was to determine the frequency of birthmarks in Israeli neonates of Jewish and Arabic origin., Methods: A cohort of 1672 newborn infants under 96 hours of age were examined for the presence of birthmarks. Of these 841 (50.3%) were Jewish and 831 (49.7%) were Arab. The Jewish group was further subdivided into various ethnic groups according to parental ancestry., Results: Melanocytic brown lesions (Mongolian spots, congenital nevi, and café-au-lait spots), were more common in Arab infants. The vast majority of Jewish infants with Mongolian spots were of Asian or African ancestry. On the other hand, congenital melanocytic nevi were found only in Jewish infants of European ancestry. Vascular lesions (salmon patch and port-wine stain) in Arab neonates exhibited a female preponderance., Conclusions: Our data suggest that the prevalence of birthmarks in Israeli neonates is similar to the prevalence reported by others in white neonates.

Objective: To determine if early serum hCG levels are predictive of implantation outcome in patients undergoing IVF-ET., Design: Retrospective study of IVF cycles using receiver operator characteristic curve (ROC) analysis., Setting: Tertiary-care, university hospital-affiliated IVF program., Patients: Three hundred fifty-one conception cycles were studied., Interventions: None., Main Outcome Measure: Implantation failure, defined as chemical pregnancies, ectopic gestations, and first trimester abortions, or implantation success, defined as delivered singleton and multiple pregnancies, and second trimester abortions., Results: For each post-ET day 14 to 20, mean hCG levels of the implantation success group were significantly greater than implantation failure outcomes (P < 0.0001). Using ROC curve analysis, hCG cutoff values for each post-ET day were calculated for optimal discrimination of implantation failure from implantation success cycles. A patient with an hCG measurement greater than the calculated cutoff value had a > or = 90% chance of having an implantation success after IVF-ET., Conclusion: Discriminatory hCG cutoff values may be useful in predicting implantation outcome in IVF-ET cycles and may guide clinicians in identifying those pregnancies at risk for adverse outcomes and instituting more intensive surveillance in this population. This information also may be useful in providing counseling to IVF patients regarding pregnancy prognosis and result in cost savings.

Discrimination studies suggest that two, and only two, channels encode relative spatial phase shifts in compound gratings (Bennett & Banks, 1991; Field & Nachmias, 1984). The more sensitive channel consists of even-symmetric filters and responds best to cosine phase shifts (e.g., 0 degree-180 degrees); the other consists of odd-symmetric filters and responds best to sine phase shifts (e.g., 90 degrees-270 degrees). The present experiments investigated whether the two-channel model generalizes to suprathreshold perceptual tasks. Experiment 1 examined classification learning of compound gratings, consisting of a fundamental (f) and second harmonic (2f), that differed in 2f contrast and relative phase. Experiments 2 and 3 measured the perceived similarity of f + 2f gratings. The results of Experiment 1 were broadly consistent with the predictions of the two-channel model. Specifically, the classification data were best explained by assuming that classification was based on the responses of differentially sensitive even- and odd-symmetric filters. In Experiments 2 and 3, two-dimensional multidimensional scaling solutions provided a good account for the similarity judgments. In Experiment 2, Dimension 1 was strongly correlated with cosine phase, and Dimension 2 was moderately correlated with sine phase. In Experiment 3, cosine phase was again strongly related to Dimension 1, whereas the absolute value of sine phase was strongly related to Dimension 2. Overall, these results suggest that the two-channel model of phase discrimination provides a useful framework for interpreting classification and similarity judgments of compound gratings.

Although the recommendations to evaluate possible cervical spine injuries in patients with isolated head injuries are identical to those with multiple trauma, to date, no such study has confirmed that risk factors are the same for both injuries. We reviewed the charts of 268 pediatric patients with isolated head injuries admitted to the Intensive Care Unit at Children's Hospital Medical Center (1985-1990) to determine which risk factors were present. In this retrospective study, we divided the patients into two groups: low risk and high risk. The low-risk patients (n = 135) were those capable of verbal communication who did not report cervical discomfort. The high-risk patients (n = 133) either were incapable of verbal communication (preverbal or physically unable due to head injury) or reported neck pain. All patients under 2 years of age were considered preverbal and at high risk. The patients in both groups were indistinguishable by age, sex, mechanism of injury, and type of injury sustained. No patient in the low-risk group suffered cervical spine injury. Cervical spine trauma was present in 10 (7.5%) high-risk patients. Using the method of adjusted odds ratio, we found that high-risk patients had 23 times the likelihood of neck injury when compared with low-risk patients (p = 0.003, 95% confidence limit). Our results indicate that cervical spine X-rays (i.e., anteroposterior, odontoid, lateral views) are indicated only in high-risk pediatric patients with head injuries who either complain of neck pain or cannot voice such complaints because of significant head injury or preverbal age.(ABSTRACT TRUNCATED AT 250 WORDS)

There have been major advances in the treatment of multiple myeloma in the past 20 years, but for the individual patient the prognosis still remains uncertain. As the length of survival varies from several months to over 10 years, definition of prognostic parameters at the time of diagnosis, and early detection of disease activity are most important. In our study, median survival was 42 months with very good quality of life. Factors not helpful in prognosis were sex, WBC and platelet counts, BUN, serum M protein type, extent of osteolytic lesions, percentage of plasma cells in bone marrow and plasma cell asynchrony. However, age, hemoglobin, calcium, uric acid, Bence-Jones proteinuria and polyclonal Ig concentrations had a certain degree of prognostic importance. Due to more sensitive and more specific laboratory methods, peripheral blood findings are lately gaining in importance. With new "salvage" protocols, the detection of additional prognostic parameters and sensitive indicators of disease activity may be most important for further improvement in the survival of patients with multiple myeloma.

Painful piezogenic pedal papules were observed in 10 of 29 patients with Ehlers-Danlos syndrome. The diagnosis was made by examining each patient while he or she stood with full body weight on the heels and by observing the appearance of the painful papules on the medial, posterior, and lateral aspects of both heels. Biopsy specimens of papules demonstrated a thickened and dense dermis. The subcutaneous fibrous trabeculae were thin, with resultant poor compartmentalization of the fat. Painful piezogenic pedal papules are due to herniation of subcutaneous fat into the dermis, which is possibly due to structural defects of the connective tissue. The high prevalence of painful piezogenic pedal papules in our group of patients (34.5%) makes its direct association with the Ehlers-Danlos syndrome highly feasible, most probably because of the connective tissue defect that occurs in patients with Ehlers-Danlos syndrome.

Seven cases of Israeli Moslem men with hyperkeratotic nodular callosities of the legs and forehead are reported. These patients were randomly encountered over a two-year period in our dermatology clinic. The nodules were attributed to repeated trauma to the legs and forehead sustained during Moslem prayer. This article familiarizes the clinician with the morphologic characteristics of these lesions, which may occur in some Moslem men.

In vitro adherence of Candida albicans to human corneocytes and the effect of a chitin-soluble extract (CSE) on the adherence reaction were studied. Adherence of the yeasts to cells obtained from different individuals was variable. However, repeated adherence tests with pooled corneocytes of 2 individuals from this group showed that the adherence parameters did not differ greatly throughout these tests. CSE at the concentration of 50 mg/ml had a significant inhibitory effect on the attachment of C. albicans to the corneocytes, most probably by blocking their receptors of attachment. The data indicate that the preparation may be useful in the prophylactive management of recurrent cutaneous candidiasis in susceptible individuals.

A case of transient acantholytic dermatosis (TAD) in pregnancy is reported. The eruption appeared in the third month of the pregnancy and completely resolved two months later. Reports concerning the occurrence of this disorder in young people are rare, and, to the best of our knowledge, this is the first patient in whom onset of the disease has been detected during pregnancy.

Six cases of dermatophytosis of the diaper area due to either Epidermophyton floccosum or Trichophyton rubrum are described herein. The clinical and laboratory features of these patients and those reported in the literature are highlighted. Dermatophytosis should be considered in the differential diagnosis of rashes in the diaper area. Most standard remedies for diaper rash will not be effective or may exacerbate diaper dermatophytosis. Recognition of this condition by the clinician will lead to earlier rational treatment and may avoid the delay caused by therapy.

A 19-year-old white woman with pityriasis rotunda in whom no diagnosable disorder could be demonstrated is reported on herein. Pityriasis rotunda has been described in Oriental and black patients, usually in association with certain serious systemic diseases. Our patient appeared to have the second reported case of pityriasis rotunda in white persons. In both cases the dermatosis occurred in an otherwise healthy individual. Physicians should be alert to the possible occurrence of pityriasis rotunda in white patients. The literature on pityriasis rotunda is reviewed.

A 5-year-old girl with Ehlers-Danlos syndrome developed painful piezogenic pedal papules. She had suffered from pain in her heels for the previous two years and had undergone extensive orthopedic examinations that revealed no abnormalities. On admission, she had typical signs associated with piezogenic pedal papules (PPP), with pain induced by standing and disappearance of the lesions and the pain on relief of pressure. These papules were due to herniation of subcutaneous fat into the dermis, possibly because of structural defects of the connective tissue. To the best of our knowledge, this is the first reported case of painful PPP occurring in childhood. We suggest that the herniation of subcutaneous fat in our patient was most probably due to the connective tissue defect that occurs in the Ehlers-Danlos syndrome.

Renal biopsy specimens from eight patients with cryoglobulinemic nephropathy were examined by light, electron, and immunofluorescence microscopy. The light microscopic features were not diagnostic. Characteristic ultrastructural findings were observed in all except one specimen and consisted of glomerular electron-dense deposits, which had a coarse, granular appearance at low-power magnifications and revealed tubular, annular and in a few cases, also fibrillar structures at high-power magnifications. Subendothelial deposits were a constant finding. The presence of intravascular thrombi (deposits), osmiophilic bodies, and crystals within the cytoplasm of the glomerular cells are also helpful for the diagnosis of this entity. Because of these specific findings, cryoglobulinemic nephropathy should be recognized as a clinicopathologic entity.

Two patients with primary sclerosing cholangitis developed perforating lesions in the skin of the extremities. Clinically and histologically, the cutaneous condition showed the features of perforating folliculitis with superimposed lesions of prurigo nodularis. The course of the eruption paralleled the severity of the biliary disease. A theory of pathogenesis for the development of the perforating folliculitis in these patients is suggested. To the best of our knowledge, this is the first report of the coexistence of both conditions.