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PURPOSE OF REVIEW: The essential role of the lymphatic system in fluid homeostasis, nutrient transport, and immune trafficking is well-recognized, however there is limited understanding of the mechanisms that regulate lymphatic function, particularly in the setting of critical illness. The lymphatics likely affect disease severity and progression in every condition, from severe systemic inflammatory states to respiratory failure. Here, we review structural and functional disorders of the lymphatic system, both congenital and acquired, as they relate to care of the pediatric patient in the intensive care setting, including novel areas of research into medical and procedural therapeutic interventions. RECENT FINDINGS: The mainstay of current therapies for congenital and acquired lymphatic abnormalities has involved non-specific medical management or surgical procedures to obstruct or divert lymphatic flow. With the development of dynamic contrast-enhanced magnetic resonance lymphangiography, image-directed percutaneous intervention may largely replace surgery. Because of new insights into the mechanisms that regulate lymphatic biology, pharmacologic inhibitors of mTOR and leukotriene B4 signaling are each in Phase II clinical trials to treat abnormal lymphatic structure and function, respectively. SUMMARY: As our understanding of normal lymphatic biology continues to advance, we will be able to develop novel strategies to support and augment lymphatic function during critical illness and through convalescence.

Background Infants with critical congenital heart disease ( CCHD ) are more likely to be small for gestational age (GA). It is unclear how this affects mortality. The authors investigated the effect of birth weight Z score on 1‐year mortality separately in preterm (GA CCHD . Methods and Results Live‐born infants with CCHD and GA 22 to 42 weeks born in California 2007–2012 were included in the analysis. The primary predictor was Z score for birth weight and the primary outcome was 1‐year mortality. Multivariable logistic regression was used. Results are presented as adjusted odds ratios and 95% confidence intervals ( CIs ). The authors identified 6903 infants with CCHD . For preterm and full‐term infants, only a Z score for birth weight Z score 0–0.5, adjusted odds ratio, 2.15 [95% CI , 1.1–4.21] and adjusted odds ratio, 3.93 [95% CI , 2.32–6.68], respectively). In contrast, in early‐term infants, the adjusted odds ratios for Z scores CI , 1.93–6.04), 1.78 (95% CI , 1.12–2.83), and 2.03 (95% CI , 1.27–3.23), respectively, versus the reference group. Conclusions GA seems to modify the effect of birth weight Z score on mortality in infants with CCHD . In preterm and full‐term infants, only the most severe small‐for‐GA infants ( Z score Z score

Infants with critical congenital heart disease (CCHD) are more likely to have fetal growth restriction and subsequently be small for gestational age (SGA) at birth. While SGA infants with CCHD are known to have poor postnatal outcomes, birth weight (BW) has only been evaluated as a dichotomous

Cholangiocarcinoma is a rare disease, accounting for less than 2% of all human malignancies. 1 Although the entire biliary tree is potentially at risk, tumors involving the biliary confluence or the right or left hepatic ducts (hilar cholangiocarcinoma) are most common and account for 40% to 60% of all cases. 2–6 Meaningful clinical experience in managing hilar cholangiocarcinomas has been limited to a few referral centers because of the infrequency with which they are encountered. Although resection has long been recognized as the most effective therapy for hilar cholangiocarcinoma, 7 the importance of partial hepatectomy and the willingness of surgeons to use it routinely are relatively recent developments. 2,8–10 Many clinical series extend over a prolonged period, often greater than 20 years. 4,5,9,11,12 As a result, these reports lack a uniform approach to diagnosis, assessment of disease extent, and resection, and the results are therefore difficult to interpret. Further, most studies originate from surgical departments and tend to focus on surgical findings and results and often do not provide a full accounting of all patients seen. Long-term survival in patients with hilar cholangiocarcinoma depends critically on complete tumor resection. 2,10 In the absence of widespread disease, the likelihood of achieving a complete resection requires examination of all factors related to local tumor extent, which increasingly has become possible with noninvasive imaging studies. 13,14 Tumor location and extent within the biliary tree, as provided by the Bismuth-Corlette classification system, 15,16 is only one component. Additional factors that must be addressed relate to radial tumor growth and its impact on adjacent structures, specifically portal venous involvement and consequent hepatic lobar atrophy. Both the modified Bismuth-Corlette and the American Joint Committee on Cancer 17 staging systems fail to account for all of these local, tumor-related factors, which frequently influence therapy. We have shown previously that a preoperative staging system that accounts fully for local tumor-related factors accurately predicts resectability and correlates with survival. 2 The present study represents an analysis of all patients with hilar cholangiocarcinoma seen and treated at a single institution during a 9-year period. The organizational structure of the hepatobiliary program at Memorial Sloan-Kettering Cancer Center (MSKCC) allows a multidisciplinary review of all patients, regardless of disease stage. Thus, a full accounting of all patients, including those with unresectable tumors, is possible. The relatively short time interval ensures homogeneity with respect to disease assessment and surgical approach. This study also proposes a preoperative staging system, based on imaging data and modified from a previous report, 2 that stratifies patients into treatment groups with predictable resectability rates and survival.

The prevalence and significance of free cancer cells in the peritoneal cavity of clinically M0 gastric cancer patients is unknown. We reviewed our results with peritoneal washings to determine (1) the prevalence of positive cytology in M0 and M1 disease and (2) the influence of positive cytology on the pattern of failure and survival.Laparoscopic washings were obtained from 127 patients with gastric cancer at Memorial Sloan-Kettering Cancer Center from December 1, 1990 to August 1, 1996. Cytology was performed by the Papanicolau technique.The prevalence of positive cytology was as follows: 0% (0/45) in T1/T2 M0 disease; 10% (3/31) in T3/T4 M0 disease; and 59% in M1 disease. The three M0 patients with positive cytology recurred intra-abdominally (median follow up of 8.5 months). Survival was significantly less compared with stage-matched controls with negative cytology resected for cure (P.03), and the same as those patients with stage IV disease.Patients with positive lavage cytology are stage IV, even in the absence of macroscopic peritoneal disease. Laparoscopic lavage cytology is a rapid technique for identifying the subset of M0 patients who are unlikely to benefit from resection alone. Such patients require additional treatment strategies to improve survival.

Background: Palliating the effects of biliary obstruction is a major goal of therapy in patients with cancer at the hepatic duct confluence. This study was undertaken to evaluate the effectiveness of intrahepatic biliary-enteric bypass to either the segment III duct or the right sectoral hepatic ducts in patients with unresectable hilar cholangiocarcinoma or gallbladder carcinoma. Methods: From December 1991 to October 1996, 55 consecutive bypass procedures were prospectively evaluated in patients with unresectable hilar cholangiocarcinoma or gallbladder cancer. Patients were divided into three groups based on the primary tumor and the type of bypass performed: group 1A, cholangiocarcinoma/segment III bypass (n = 20); group 1B, cholangiocarcinoma/right sectoral hepatic duct bypass (n = 14); group 2, gallbladder cancer/segment III bypass (n = 21). Results: Mean hospital stay (14 ± 2 days) and mean blood loss (629 ± 84 mL) were similar among the three groups. Perioperative death occurred in 6 patients (11%): 0 in group 1A, 3 each in groups 1B and 2. All survivors had relief of jaundice and pruritis after bypass. Complications occurred in 25 patients (45%). Preoperative transhepatic biliary drainage, performed in 14 patients prior to referral, was associated with a higher incidence of contaminated bile, greater operative blood loss, and postoperative biliary leak that was less likely to resolve spontaneously. Median survival in patients with cholangiocarcinoma (groups 1A and 1B) was 52 weeks and was unaffected by the type of bypass performed. By contrast, median survival in patients with gallbladder cancer (group 3) was 20 weeks; all but 3 died within 32 weeks of surgery. In patients with cholangiocarcinoma, the 1-year bypass patency was 80% in group 1A (segment III bypass) and 60% in group 1B (right sectoral hepatic duct bypass). Overall, there were 9 late bypass failures (18%) requiring reintervention. Conclusions: Intrahepatic biliary-enteric bypass effectively relieves symptoms due to malignant hilar obstruction. In patients with cholangiocarcinoma, segment III bypass provides excellent palliation with relatively few late complications and can be performed with minimal morbidity and mortality. Bypass to the right sectoral hepatic ducts, on the other hand, is associated with significant procedure-related morbidity and mortality and more late complications. Patients with gallbladder cancer, because of their poor survival, are probably better palliated by percutaneous biliary stenting.

In 1963 the American Medical Association (AMA) and the American Academy of Pediatrics (AAP) jointly established the Jacobi Award named after Dr Abraham Jacobi, acknowledged as the founder of pediatrics. He introduced pediatrics as a specialty in the opinions of medical historians. The first recipient was Dr Alexis F. Hartman, Sr, Professor of Pediatrics at Washington University in St Louis. This tribute to the efforts of Dr Abraham Jacobi reminds us of the very significant dedication that characterized Dr Jacobi's life. He was a most remarkable man of boundless energy and strong convictions. One year ago, Dr Robert Haggerty gave a very scholarly presentation of Jacobi's personal life and his professional life. Dr Haggerty referred to Jacobi as “a respectable rebel,” in my opinion, a very appropriate description of this remarkable man. Rather than covering many facets of Jacobi's life and career as did Dr Haggerty, it is appropriate that we touch on some aspects of Dr Jacobi's character as a reminder to keep us focused on the development of our specialty, the problems it presented, and also the problems we face today. Dr Jacobi was born in Hartum, Westphalia, Germany, in 1830. His family was of very limited means; consequently, there were struggles and sacrifices to provide him with his education. His MD was obtained from the University of Bonn in 1851.1 Jacobi's professional life could have been predicted from his outspoken strong opinions and intolerance of heavy-handed rules. His life was characterized by struggles against the prejudices of new ideas, injustices, and absurdities beginning in his student years. As a “zealous democrat” he was involved in the cause of the revolutionary activities going on in the 1840s in Germany. For this he was accused of high treason and was confined in prisons in Cologne and Minden for … Address correspondence to: Edmund C. Burke, MD, Mayo Clinic, 200 First St NW, Rochester, MN 55905.

This study evaluated the effect of locally delivered methylprednisolone on the systemic and local immune response in the sponge matrix allograft model. Polyurethane sponges were coated with peritoneal exudate cells from DBA/2 (H-2d) or C57Bl/6 mice (H-2b) and placed subcutaneously in C57BL/6 recipients 24 hr later. Each mouse received both a syngeneic and an allogeneic sponge graft. Local immunosuppression was effected by placement on day 0 of cellulose/matrix pellets containing a preparation of increasing quantity of controlled release MP or by daily intrasponge injection of MP. Local immunosuppression was demonstrated by decreased cytotoxicity on day 12 in the allogeneic sponge receiving MP directly, as compared with the groups that received MP in the opposite syngeneic sponge or no MP. This effect was noted at a specific MP dose (0.5 mg/kg/day). Absolute numbers of precursor cytolytic cells and mature cytolytic cells (determined by limiting dilution analyses) infiltrating the allografts were also decreased in the sponges receiving MP directly, relative to the groups receiving MP in the opposite syngeneic sponge. Maintenance of systemic (in contrast to local) immunity was also demonstrated. Animals treated with local MP into a simultaneously placed syngeneic sponge or in whom no MP was delivered became sensitized to a synchronous DBA/2 sponge and rejected a subsequent DBA/2 skin graft in second-set fashion. Conversely, animals that received local MP into their synchronous DBA/2 sponge rejected a subsequent DBA/2 skin graft or a third-party graft with first-set kinetics. The presence of local MP at the initial graft site prevented the animals from becoming sensitized to the presented alloantigen but did not keep the animal from developing a rejection response to a third-party skin graft with first-set kinetics. It appears that delivery of MP locally to the site of antigen is an effective method to modulate alloreactivity. In addition, the sponge matrix allograft appears to be a useful model for studying local immunosuppression.

Background The indications for preoperative biliary stenting in patients with obstructive jaundice are controversial. We evaluated the effect of preoperative biliary stenting on bacterobilia and infectious complications following surgical treatment of proximal cholangiocarcinoma. Design A retrospective review was performed of all patients undergoing surgical treatment of proximal cholangiocarcinoma. Setting A metropolitan cancer surgery service. Patients and Methods Seventy-one patients underwent palliative biliary bypass or curative resection of proximal cholangiocarcinoma from March 1, 1991, to April 1, 1997, and were entered into a prospective database. Forty-one patients underwent preoperative biliary intubation and stent placement. We analyzed patient, nutritional, laboratory, and operating room factors. Statistical evaluation was performed using Studentttest and χ2analysis. Main Outcome Measure Data were recorded for a history of cholangitis, operative time, amount of blood loss, incidence of intraoperative bacterobilia, proportion of patients with postoperative infectious and noninfectious complications, and length of hospital stay. Results: All patients (n=14) with a history of preoperative cholangitis had been subjected to previous endoscopic retrograde cholangiopancreatography and/or percutaneous transhepatic biliary drainage. Groups were equivalent for risk for comorbidity, proportion undergoing curative vs palliative procedures, time spent in the operating room, and amount of blood loss. Patients with stents had a significantly lower bilirubin level (P=.005). Patients with stents had a significantly increased risk for bacterobilia (P=.001) and infectious complications (P=.03). Bacterobilia was present in 11 (100%) of 11 patients undergoing endoscopic stenting and in 15 (65%) of 23 patients undergoing percutaneous stenting. There was no increased risk for noninfectious complications, length of hospital stay, or mortality in patients with stents. In 10 (59%) of 17 patients with postoperative infectious complications and positive findings of intraoperative bile culture, the organism was synonymous. Conclusions Preoperative biliary stenting in proximal cholangiocarcinoma increases the incidence of contaminated bile and postoperative infectious complications. Endoscopic stents frequently do not relieve jaundice in high biliary obstruction and are rarely indicated, especially in light of their high contamination rate.

OBJECTIVES: To determine the resectability rate for hilar cholangiocarcinoma, to analyze reasons for unresectability, and to devise a presurgical clinical T-staging system. METHODS: Ninety patients with hilar cholangiocarcinomas seen between March 1, 1991, and April 1, 1997, were evaluated. Accurate patterns of disease progression and therapy were evaluable. Disease was staged in 87 patients using extent of ductal tumor involvement, portal vein compromise, and liver atrophy. RESULTS: In 21 patients, disease was deemed unresectable for cure at presentation. In 39 patients, disease was found to be unresectable at laparotomy, 23 secondary to nodal (N2) or distant metastases. Unresectability was the result of metastases in 52% and of locally advanced disease in 28%. Thirty patients (33%) had resection of all gross disease, and 25 of these (83%) had negative histologic margins. Twenty-two patients underwent partial hepatectomy. The 30-day mortality rate was 7%. Projected survival is greater than 60 months in those with a negative histologic margin, with a median follow-up of 26 months. A presurgical T-staging system allows presurgical selection for therapy, predicts partial hepatectomy, and offers an index of prognosis. CONCLUSIONS: In half the patients, unresectability is mainly the result of intraabdominal metastases. Presurgical imaging predicts unresectability based on local extension but is poor for assessing nodal metastases. In one third of patients, disease can be resected for cure with a long median survival. Curative resection depends on negative margins, and hepatic resection is necessary to achieve this. The T-staging system correlates with resectability, the need for hepatectomy, and overall survival.

OBJECTIVE: The authors determined the accuracy of laparoscopy in detecting metastatic disease in patients with gastric adenocarcinoma. SUMMARY BACKGROUND DATA: The majority of patients with gastric adenocarcinoma in the United States present with advanced disease. They are at high risk for intraabdominal metastatic spread. METHODS: One hundred eleven patients with gastric adenocarcinoma underwent laparoscopy at Memorial-Sloan Kettering Cancer Center from December 1991 to December 1995. All were judged to be free of intra-abdominal metastatic disease on preoperative computed tomographic scan imaging. RESULTS: Laparoscopic exploration was successful in 110 of 111 patients and accurately staged 94% of the patients with respect to metastatic disease with a sensitivity of 84% and a specificity of 100%. The prevalence rate of metastatic disease was 37%. Twenty-four patients underwent laparoscopy only and were discharged in an average 1.4 days versus 6.5 days in patients undergoing exploratory laparotomy without resection (p < 0.05). No patients undergoing laparoscopy only have returned for palliative surgery. CONCLUSIONS: Laparoscopy should be performed in nonobstructed, nonbleeding patients with advanced gastric cancer in the United States. More than one third of these patients have unsuspected metastatic disease at time of operation. Laparoscopy is highly accurate in detecting occult metastases and identifies a unique population of stage IV patients who may benefit from newer induction chemotherapeutic approaches while avoiding unnecessary laparotomy.

• Medical records of 52 human immunodeficiency virus (HIV)-infected patients who underwent a total of 80 anorectal operations from January 1985 to January 1990 were retrospectively reviewed to determined whether anorectal surgical wounds healed in HIV-infected patients and the mean survival time of these patients after surgery. Twenty-four operations were performed in asymptomatic HIV-infected patients, 19 in HIV-infected patients with persistent lymphadenopathy, and 37 in patients with acquired immunodeficiency syndrome. Wounds healed in 49 patients (94%). The mortality rate 30 days after surgery was 2%. There were no major complications. The mean survival time of HIV-infected patients after surgery was 15 months. We conclude that anorectal surgical wounds heal in most HIV-infected patients and that the survival time after surgery of HIV-infected patients with anorectal disease justifies appropriate surgical treatment. ( Arch Surg. 1991;126:1267-1271)

The clinical and pathologic findings of 2 infants and 7 older children with polyarteritis nodosa who were autopsied are reported. The most frequent clinical features included prolonged high fever, skin rash, abdominal symptoms, leukocytosis, proteinuria, and signs of either cardiac or renal failure. The 2 infants died of cardiac arrest, whereas renal or neurologic involvement was the most common cause of death in the older children. A consistent finding at autopsy was arteritis of the epicardial coronary arteries.

Familial metaphysial dysostosis has been described as it occurred among 18 members of a family in seven generations. The roentgenologic changes were very similar to those seen in vitamin D resistant rickets. No biochemical defect could be detected. Studies of renal function with special reference to tubular reabsorption of phosphorus gave normal values. The treatment of choice is osteotomy, after epiphysial closure has occurred, to correct the pronounced bowing of the legs. Administration of vitamin D in high doses has no effect on the course of this disease.

The clinical course of 41 patients who had systemic lupus erythematosus complicated by nephritis with symptoms beginning before they were 15 years old was reviewed. The retrospective analysis showed a definite trend toward prolonged survival among the patients receiving high doses of steroids, compared to those receiving no or low doses. We conclude that it is no longer justified to withhold high-dose steroid therapy from children with lupus nephritis, but it should be emphasized equally that this does not apply to children with discoid lupus erythematosus, rheumatoid arthritis with a positive LE clot test, or drug-induced lupus erythematosus. Our present plan of management is outlined.

Isotope renograms (ortho-iodohippurate-I were obtained from 30 children who had no evidence of renal disease. Determinations were made by a standardized technique with the subjects in a state of relative dehydration; in 15 children the renogram was repeated under conditions of hydration (14 children) or further dehydration (1 child). Values for the point of initial peak (point A), point of maximal radioactivity (point B), and the differences between these points (B-A) were calculated. These values were plotted arithmetically against surface area to obtain regression lines and estimates of the variability of the observations about the line. The time from appearance of radioactivity to peak radioactivity was calculated and found to fall into a relatively narrow range. Similar attempts to correlate mathematically the height of timed segments of the decreasing slope of the renogram with surface area were successful in children with a body surface area of 1.15 m2 or greater. The state of hydration appeared to influence the excretory slope of the renogram; this effect was most pronounced in children whose total body surface area was less than 1.15 m2.

Daily water metabolism of 21 patients was studied for 3 days after intracardiac surgery and whole-body perfusion. The average obligatory water losses of these patients were approximately 500, 750, and 750 ml. per M. 2 of body surface per day on the first, second, and third postoperative day, respectively. A simple system, which combines knowledge of the estimated postoperative water requirement with the known daily change in body weight, was devised to manage accurately water metabolism after intracardiac surgery.

Each of 21 patients was noted to have a low concentration of water in the serum immediately after whole-body perfusion. This indicated a loss of water either by the patient or the pump-oxygenator. Investigation suggested that the patient, and not the pump-oxygenator, was the source of water loss. Two of the 21 patients had a profuse, short-lived solute diuresis in the immediate postperfusion period. These phenomena are apparently benign and they require no specific treatment.

A survey of the case records of children seen at the Mayo Clinic from 1935 to 1965 revealed that 77 had nephro-ureterolithiasis and 6 had nephrocalcinosis. Calculous disease occurred twice as frequently in boys as in girls; the incidence did not change with age between the ages of 1 and 15 years. Urologic lesions, urinary infections, or both were present in 49 children and cystinuria was present in 5. In 8 children, renal stones were considered causally related to hypercalciuria, whereas in 14 children no etiologic associations could be found. Chemical analysis of calculi, which was performed in 36 cases, revealed that most stones consisted of calcium oxalate or phosphate; no uric acid stones were found.

The clinical and hemodynamic features are described in a 7½-year-old boy who had a patent ductus arteriosus and pulmonary hypertension without a murmur. Values for oxygen saturation of the blood and dye-dilution curves demonstrated that the flow through the ductus was mainly from pulmonary artery to aorta. This was reversed when the patient breathed 99.6 per cent oxygen. Blood shunted from right to left through the ductus could be detected at the right radial artery. Thus retrograde movement of blood occurred in the aortic arch. Clamping of the ductus caused an increase in pulmonary arterial pressure, a decrease in systemic arterial pressure, an increase in arterial oxygen saturation and a change in the arterial dye-dilution curve toward normal. Division of the ductus was followed by clinical improvement.

A case is described in which oxalosis occurred in an 11-year-old boy. The patient had experienced the formation of numerous calculi and multiple operations for removal of the calculi. The clinical, pathologic and histochemical findings in the case are presented. The unusual features of the case are extensive deposits of calcium oxalate crystals in the kidneys, bone marrow, spleen, liver, blood vessels, and adrenal cortex. Calcium oxalate, a relatively inert substance, appears to effect renal damage by virtue of its deposition in the renal tissue. While many aspects of oxalate metabolism remain obscure, it is generally presumed that oxalosis represents an as yet undefined metabolic defect.

Radioisotope renograms with orthoiodohippurate-I131 were obtained for 41 children who had known renal or renovascular disorders. (Two patients had hypertension but no evidence of renal disease.) Values determined for various components of the renogram curves were considered abnormal when they exceeded two standard deviations from the mean values for normal children of similar body surface area or when time B (from injection to the point of maximal radioactivity) exceeded 6.5 minutes or differed from the contralateral kidney by more than 1 minute. The renogram was clearly abnormal in all 17 patients who had unilateral or predominantly unilateral renal disorders. Abnormalities associated with bilateral renal disorders varied with the disease and its severity. The clinical severity of impairment of renal function could be estimated from the renogram. The isotope renogram proved to be a simple, reproducible technique for determination of function of the separate kidneys.

Five children in two families, three boys in one family and two girls in another, had the onset of nephrotic syndrome in early infancy. The proteinuria and edema formation were not altered by steroid therapy. Growth retardation, renal osteodystrophy, and tetany appeared at an early age. Glycosuria, aminoaciduria, and renal tubular acidosis developed during the course of illness, and nephrocalcinosis was noted. A renal biopsy specimen from a child in each family showed glomerular proliferative changes, glomerular scarring, interstitial inflammation, and microcalcinosis, together with extensive tubular atrophy. Three of the five children have died so far, all at about 8 years of age. The similarity of the clinical course suggests that the children in each of these families has a distinct inherited disease, a form of familial nephrotic syndrome.

Summary An 18-month-old boy was brought to the Mayo Clinic because of gangrene of one toe and generalized edema. The clinical course and pathologic findings indicated the presence of thrombosis of the renal veins and the nephrotic syndrome. Necropsy revealed thrombi in the inferior vena cava and both renal veins. Only four cases of the nephrotic syndrome and thrombosis of the renal veins occurring in children have been reported in the literature heretofore and these are reviewed briefly. A theory as to the possible mechanism for the pathogenesis of edema has been developed. However, the cause of thrombosis of the renal veins remains unknown.

Although most patients with coarctation of the aorta survive to early adult life, it has been adequately demonstrated that some patients die in infancy and early childhood as a result of their coarctation. In selected cases, then, operation is advisable at a very early age. Cardiac failure in a 10 week old infant necessitated operation at this age in the case reported.

Current information relating to the clinical significance of proteinuria in children through adolescence was reviewed. Proteinuria may be transient, orthostatic, or fixed. The data indicate that only fixed proteinuria—that is, proteinuria on multiple urine examinations without pos tural exaggeration—may indicate serious glomerular disease. The patient should be followed with periodic but infrequent examinations, avoiding unnecessary, complicated, and hazardous investigations.

The method of collection of urine for bacteriologic investigation is of utmost importance. Catheterization and midstream collection* are both acceptable and widely employed methods of obtaining urine from the female child for colony counts. Nonetheless, neither is practical nor possible in many instances. Often urine must be collected by having the child void into a sterile container after thorough cleansing of the area. Frequently, in such instances, evidence of bacteriuria is found which may be puzzling in the absence of symptoms or other evidence of urologic disease. Contamination in such cases is a likely source and we wish to point out what is considered a significant observation that may have important influence on the method of collection of urine.

Follow-up survival and health information were obtained, after a median of 27.5 years, from 132 patients who had been seen originally as children with nephrotic syndrome between 1951 and 1967. Ninety seven patients were alive. Recurring edema or proteinuria, or both, persisted in 15 percent of those still alive. Eight of 11 parous women reported relapses during pregnancy. There was no apparent increase in malignancies, atopic diseases, clinical defects in cell-mediated immunity, or cardiovascular diseases. Twenty two patients (17%) died of renal causes between 3 months and 8 years after the onset of nephrotic syndrome. Steroid resistance was the presenting feature universally predictive of a poor outcome; nine of the 11 such patients died and the other two are now receiving hemodialysis. Hematuria was present initially in 41 percent of the patients who died of renal causes, compared with 14 percent of those still alive. Hypertension was noted on the first examination in 22 percent of those who died of renal causes, compared with 10 percent of those alive.

In a study of a family of four children and the parents it was found that three of the four children suffered from mental retardation, spastic diplegia and sensitivity to flickering light. The sensitivity to light experienced by the children in this family is compared with similar conditions described as “hereditary light sensitive epilepsy”. The hypothesis is offered that while in some instances sensitivity to light results from diffuse neuronal disease, in others it stems from a more discrete defect in subcortical systems.

Of six patients with diffuse glomerulonephritis and associated infected ventriculoatrial shunt, three had improved renal function after removal of the infected shunt, and three died from Central-nervous-system disease. The renal disease probably resulted from an immunologic response to the staphylococcal infection rather than from direct bacterial embolization of the kidney.

SummaryThe carbazole and naphthoresorcinol methods for hexuronic acid determination have been used to characterize the partially purified acid mucopolysaccharides obtained from urine of normal persons, from patients with gargoylism and from their relatives. The results, expressed as a ratio of carbazole to naphthoresorcinol hexuronic acid, demonstrated a similar abnormal qualitative excretion of chondroitin sulfate B by patients with gargoylism. by one or both parents, and by some of their other relatives. Occurrence of an abnormal qualitative excretion of chondroitin sulfate B in the clinically normal relatives of patients with gargoylism probably indicates the heterozygous carrier state of the disease.

THIS report is concerned with a familial renal disease associated with nerve deafness. Familial renal disease without associated nerve deafness, which has been adequately reviewed by Eason,1 Rinkoff2 and Reyersbach3 and their associates, is not discussed further here. We are of the opinion that the three following criteria are necessary for the diagnosis of this syndrome: a family history of progressive renal disease in males affected; the presence or history of hematuria, which may be intermittent; and nerve deafness in most of the males and in some of the females affected. We have thus termed this entity "hereditary hematuria, nephropathy . . .

IN 1839 Frohlichfirst described the clinical entity now referred to as the prunebelly syndrome. At that time, although it was evident that the abdominal musculature was absent, the associated urinarytract anomalies were not recognized. The thin skin and omental covering of the abdomen appeared wrinkled and wizened like a dried prune, hence the appellation "prune-belly" (Fig 1). Parker, in 1895, called attention to the accompanying hydronephrosis, hydro-ureter, megalocystis, and undescended testes as comprising this syndrome. Although 146 cases of this syndrome have been reported in the literature,1-13including the more recent report of Williams and Burkholder7in August 1967, none has detailed the survival data of these children. We wish to add our 19 cases to this total, thus bringing the overall number of cases to 165, and to discuss the clinical findings and survival in thissyndrome. Our Series Since 1945, 19 patients with prune-belly syndrome have been seen at

GROSS HEMATURIA in a child is both unusual and alarming. In a previously healthy child without a history of trauma, the differential diagnosis in these cases includes glomerulonephritis, tumors of the urogenital system, hematologic diseases, inflammatory conditions, and reticuloendothelial diseases. Rarely is hydronephrosis considered a cause of gross hematuria. We recently saw an 8-year-old boy who had sudden onset of gross painless hematuria with the passage of clots. The anomaly and the hydronephrosis coexisting and the satisfactory surgical results were unusual enough to be reported. Report of a Case An 8-year-old boy was admitted to the hospital on April 8, 1966, because of painless gross hematuria of 12 hours' duration. At the onset of the hematuria, a few clots were present in the voided specimens. These were passed after fleeting and colicky pains in the right flank. The boy's past health had been good and there had been no previous