Menachem Schlesinger

Hypocomplementemic autosomal recessive hemolytic uremic syndrome with decreased factor H.

Abstract. We describe the clinical course, complement components, and pathological findings of 10 infants with autosomal recessive hemolytic uremic syndrome (HUS). All patients were members of one extended highly inbred Bedouin kindred. The median age of presentation was 2 weeks (range 1–20 weeks). Eight patients died, 2 patients are alive, on dialysis. Renal biopsies revealed thrombotic microangiopathy with a predominant early arteriolar involvement and subsequent development of ischemic glomerular changes. Immunofluorescence was positive for C3 in glomeruli. All patients had low complement components levels during and between relapses, and in some this was evident soon after birth and prior to the onset of symptoms. This deficiency could not be normalized by repeated plasma transfusions. Biosynthetic labelling of patients’ fibroblasts demonstrated normal rates of C3 protein synthesis. Serum factor H levels were greatly decreased or absent in 4 patients tested and moderately decreased in 15 of 23 healthy unaffected siblings and patients. This defect may cause complement activation and consumption, possibly at the endothelial cell level.

Rare jatropha multifida intoxication in two children

Two children were admitted to the Emergency Department (ED) after ingesting a large amount of fruit of a plant identified as Jatropha multifida. They were mildly obtunded, had intractable vomiting, and seemed dehydrated. Intravenous fluid replacement and urine alkalinization were initiated. After stabilization, their 5-day hospital stays were uneventful except for a subclinical rise of liver enzymes. Jatropha species contain the toxalbumin ricin, which causes severe vomiting and diarrhea, dehydration, shock, and renal and hepatic impairment. Ricin also has cardiotoxic and hemolytic effects and several deaths have been documented. Children are attracted by the shape and the color of the Jatropha fruits. Mortality can be prevented by immediate fluid and electrolyte replacement.

Use of a Homeopathic Preparation for “Infantile Colic” and an Apparent Life-Threatening Event

OBJECTIVE: An apparent life-threatening event (ALTE) caused by ingestion of drugs or toxins has been reported rarely among infants. None of these agents was homeopathic medication. We report 11 infants who presented with an ALTE after ingestion of Gali-col Baby, a homeopathic agent indicated for “infantile colic.” METHODS: A retrospective case-control study was conducted. Charts of all infants who were younger than 1 year and were admitted with an ALTE from January 2005 through August 2008 to the pediatric division at the Barzilai Medical Center were reviewed. Age-matched infants who were admitted on the same dates for a reason other than ALTE served as a control group. Information on medications administered before admission was recorded. RESULTS: During the study period, 36 635 children visited the pediatric emergency department of the Barzilai Medical Center. There were 11 057 admissions to the pediatric division during this period, 115 of which were because of an ALTE. Eleven of these infants received Gali-col Baby before the event as opposed to none in the control group (P < .005). Three infants received a significant overdose, compared with the manufacturers recommended dosage. After a thorough investigation, no other presumptive causes for ALTE were found among the 11 infants. CONCLUSIONS: Gali-col Baby is associated with an ALTE in some infants. There are no published controlled trials on the efficacy or safety of its use; therefore, better control and supervision of Gali-col Baby and probably other homeopathic medications are needed to prevent possible serious adverse effects.

A nationwide prospective surveillance study in Israel to document pediatric invasive infections, with an emphasis on Haemophilus influenzae type b infections

BACKGROUND AND METHODS An ongoing nationwide prospective surveillance study was initiated in Israel in October, 1988, to document childhood invasive infections caused by Haemophilus influenzae type b (Hib), Streptococcus pneumoniae and Neisseria meningitidis. This study enabled us to document the effect on childhood invasive Hib disease of the introduction of conjugate Hib vaccines to Israel. RESULTS The incidence of invasive Hib disease before the age of 5 years dropped from 34 per 100000 before initiation of immunization to < 5 per 100000 in 1995 and is projected to be <4 in 1996. After <2 years, when various conjugate vaccines had been available in the private sector alone and had achieved partial coverage only, the Israeli Ministry of Health decided to add Hib conjugate vaccine to the regular infant immunization program, free of charge, effective for all infants born after January 1, 1994. The vaccine chosen was Hib polysaccharide linked to outer membrane protein complex of N. meningitidis B. Vaccine coverage has exceeded 90% of all infants born since January 1, 1994. Efficacy and effectiveness during the first 34 months of the program (January 1, 1994, to October 31, 1996) were 95.4 and 99.7%, respectively, for all invasive Hib disease and 97 and 99.4%, respectively, for Hib meningitis. CONCLUSION The described ongoing surveillance program showed the existence and extent of Hib problems in Israel and documented the success of the immunization program in essentially eliminating the disease in Israel.

Some aspects of the humoral and neutrophil functions in post-comatose unawareness patients

Post-comatose unawareness (PCU) is one of the possible outcomes of severe brain injury. Patients with severe brain injury have an increased susceptibility to severe nosocomial infections for multifactorial reasons, including immune suppression at different levels. We studied different immunological aspects in 11 PCU patients. Impaired humoral immunity was found in 27% of them. Two patients had decreased haemolytic activity of the classical complement pathway, associated with decreased levels of the components C1q, C1r and C4. Another patient had very low levels of IgG2 and IgG4. The neutrophil killing activity was impaired in these three patients, but was completely restored with the addition of a heterologous serum, suggesting a humoral defect. Neutrophils showed normal chemotaxis and random migration, and the superoxide anion release by neutrophils was also found to be normal. Understanding the immunological events in PCU patients contributes to a better and more intensive therapeutic approach, which might accelerate the rehabilitation of these patients.

Hereditary properdin deficiency in three families of Tunisian Jews

Hereditary properdin deficiency is a rare genetic disorder of the complement system. Three propositi and six additional family members with properdin deficiency have been found following analysis of the hemolytic activity of the classical (CH50) and the alternative (AP50) complement pathways in the sera of 101 survivors of meningococcal infections and 59 survivors of severe pneumococcal and Haemophilus influenza infections. All the properdin‐deficient individuals had undetectable levels of properdin by radial immunodiffusion and by Western blotting. They belonged to three non‐related families of Tunisian Jews who came from different parts of Tunisia. Two patients had a meningococcal infection at 15 and 16 years of age, respectively, and one had Haemophilus influenza meningitis at 1.5 years of age. In contrast to the fulminant and fatal course of meningococcal infection which was previously described in some properdin‐deficient patients, our patients had a relatively mild disease. Properdin deficiency may not be as rare as previously thought. Analysis of AP50, in addition to CH50, in sera of patients who had meningococcal infection, will probably disclose many more cases of hereditary properdin deficiency. In addition, our findings indicate that, as in other complement abnormalities, hereditary properdin deficiency may also be associated with the ethnic origin of the patient.

Accessory Nipples: Any Relationship to Urinary Tract Malformation?

Abstract: One hundred two Infants and children age 3 days to 16.5 years, found to have accessory nipples (AN), went enrolled in tills study. They were categorized by ethnic origin, sex, positive family history of AN, and number, site, and shape of AN, to determine factors for Increased risk of anomalies of the urinary tract Physical and ultrasound examinations of the abdomen did not reveal evidence of urinary tract malformation In any of the children. The results of this survey support the contentions that AN are not associated with urinary tract matformatlons, and that no further investigation is required In children with solitary AN.

Cell mediated immunity and effects of “thymic humoral factor” in 15 patients with SSPE

Cell mediated immunity (CMI) in 15 patients with subacute sclerosing panencephalitis (SSPE) was assessed by E-rosette formation, leucocyte migration inhibition factor (LIF) production, and proliferative responses to mitogens. In eleven patients, one or more of these parameters were impaired. These defects varied among the different patients and no consistent or uniform pattern of immune deficiency could be demonstrated. Although no single patient had a generalized reduction of all the T-cell functions, a diminished percentage of E-rosetting cells was the most frequent abnormal parameter (7 out of 15 patients). There was no correlation between the clinical state of the patients and the immune defects. Thymic humoral factor (THF), a thymic hormone, reconstituted at least one CMI impaired function especially the reduced levels of E-rosette forming cells in 7 out of 9 patients. It seems unlikely that a generalized cell-mediated defect is the underlying cause of this disease, but immunomodulatory therapy should be considered in some selected cases.

Pediculosis capitis: why prefer a solution to shampoo or spray?

Abstract: Two hundred and sixty‐eight children with pediculosis capitis took part in a comparative study to test the efficacy of five different pediculocides commonly used in Israel. The preparations used were pyrethrin shampoo, pyrethroid spray, malathion solution, carbaryl shampoo and carbaryl lotion. Carbaryl lotion and malathion solution were much more efficient when compared to the shampoo and spray preparations.

Typhoid Fever Diagnosed by Isolation of S. typhi from Gastric Aspirate

ABSTRACT. The diagnosis of typhoid fever (TF) is usually established by culturing 5. typhi from the blood or by serology. In this report we describe three patients in whom the diagnosis of TF was made by the isolation of 5. typhi from gastric contents, despite negative blood urine and stool cultures. Culture of gastric contents has not previously been recognized as a diagnostic tool in this disease.