Evaluation of catch-up growth in severe pediatric Hashimoto's hypothyroidism.

BACKGROUND: We aimed to evaluate catch-up growth in children with severe Hashimoto's hypothyroidism (HH) after thyroid hormone replacement therapy (HRT). METHODS: A multicenter retrospective study was conducted including children referred for growth slowdown that led to the diagnosis of HH between 1998 and 2017. RESULTS: A total of 29 patients were included, with a median age of 9.7 years (13-172 months). Median height at diagnosis was -2.7 [-4.6; -0.1] standard deviation score (SDS), with a height loss of 2.5 [0.7; 5.4] SDS compared to height before growth deflection (p<0.0001). At diagnosis, the median TSH level was 819.5 mIU/L [100; 1844], the median FT4 level was 0 pmol/L [undetectable; 5.4], and the median anti-thyroperoxidase antibody level was 1601 UI/L [47; 25,500]. In the 20 patients treated only with HRT, there were significant differences between height at diagnosis and height at 1 year (n = 19, p<0.0001), 2 years (n = 13, p = 0.0005), 3 years (n = 9, p = 0.0039), 4 years (n = 10, p = 0.0078), and 5 years (n = 10, p = 0.0018) of treatment but not in the case of final height (n = 6, p = 0.0625). Median final height was -1.4 [-2.7; 1,5] SDS (n = 6), with a significant difference between height loss at diagnosis and total catch-up growth (p = 0.003). The other nine patients were also given growth hormone (GH). They were smaller at diagnosis (p = 0.01); however, there was no difference in final height between those two groups (p = 0.68). CONCLUSION: Severe HH can lead to a major height deficit, and catch-up growth seems to be insufficient after treatment with HRT alone. In the most severe cases, administration of GH may enhance this catch-up.

[The role of gonadal peptides in clinical investigation]. / Intérêt clinique du dosage des peptides gonadiques.

Inhibins, activins, and anti-Mullerian hormone (AMH) are gonadal dimeric peptides produced in ovaries and testes by homologous cells, granulosa cells and Sertoli cells, respectively. The production of inhibins is driven by FSH, that of AMH may indirectly depends on FSH, while it is down regulated, at least in the male, by testosterone. In the past decade, measurements of serum inhibin and AMH have provided useful tools for clinical investigation in gonadal disorders: pseudohermaphroditism, androgen insensitivity, anorchidism, gonadal dysgenesis, disorders of pubertal developpement. Inhibins, activins, and AMH are also reliable markers of gonadal tumors. They are extensively used as indexes of fertility: in the male the production of inhibin B reflects the spermatogenetic activity, in women both inhibin B and AMH levels are correlated with the number of preantral and early antral follicles and reflect the ovarian reserve of follicles available for recruitment.

Scintigraphic imaging of paediatric thyroid dysfunction.

Imaging of thyroid dysfunction is safe and clinically relevant in children. In congenital hypothyroidism (CH), thyroid imaging permits a precise characterization of the aetiology, which is important for genetic counselling and clinical management. CH may be due to thyroid dysgenesis (ectopia, hypoplasia and athyrosis) or occurs in eutopic glands. In the latter, hypothyroidism may be either transient, especially after iodine overload, or due to permanent autosomal recessive dyshormonogenesis. Thyroid scintigraphy (TS) with either 99mTcO4 or 123I will identify ectopic thyroid tissue, which is the commonest cause of CH. However, recent reports favour the use of 123I, which enhances the accuracy of the aetiological classification. In cases of eutopic thyroid, the measurement of 123I uptake before and after perchlorate administration evaluates the organification process. At all ages, colour Doppler ultrasound scanning (CDU) is helpful in assessing thyroid volume, in identifying nodules and in characterizing tissue vascularization. TS and CDU images of most paediatric thyroid dysfunctions are presented.

Effects of an early postnatal treatment of hypogonadotropic hypogonadism with a continuous subcutaneous infusion of recombinant follicle-stimulating hormone and luteinizing hormone.

BACKGROUND: The neonatal-midinfancy surge in pulsatile gonadotropin secretion is attributable to an increase in GnRH pulse amplitude and is associated with a rapid expansion of Leydig and Sertoli cell populations with concomitant surges in testosterone, inhibin, and anti-Mullerian hormone production as well as an increase in testicular volume. Boys with congenital hypogonadotropic hypogonadism (HH) do not activate these processes. A potential cause for azoospermia and infertility in adult life is deficient proliferation of immature Sertoli cells before and during puberty due to the absence of FSH. OBJECTIVE: The objective of the study was to investigate whether early postnatal continuous sc infusion of gonadotropins could mimic the physiological growth of testes and to evaluate responses of the Leydig and Sertoli cells to early gonadotropin replacement. DESIGN AND METHODS: Two neonates (P1 with hypotuitarism and P2 with HH) with micropenis and microorchidism were treated for 6 months with high doses of recombinant LH and FSH (a gift of Luveris and Gonal-F from Serono, Lyon, France) delivered sc with an insulin pump. RESULTS: Gonadotropin continuous sc infusion increased mean serum LH and FSH to normal or supranormal levels. Mean testosterone increased from undetectable levels to 7.6 and 5.2 nmol/liter, respectively, in P1 and P2. Inhibin B and anti-Müllerian hormone increased to normal levels. Mean testicular volume increased from 0.45 to 0.57 ml at birth to 2.10 ml at 7 months. Stretched penile length increased from 8 to 30 mm (P1) and 12 to 48 mm (P2). CONCLUSIONS: The present regimen induced physiological postnatal testes growth and high-normal activation of Leydig and Sertoli cells.

Pathogen-specific risk factors and protective factors for acute diarrheal illness in children aged 12-59 months in São Paulo, Brazil.

Diarrheal diseases are a leading cause of childhood morbidity and mortality in Latin America. Most studies have focused on infants but not on older children. We enrolled 505 children (age, 12-59 months) with diarrhea and age-matched controls in a case-control study in Sao Paulo, Brazil. Independent risk factors for diarrhea included another household member with diarrhea (matched odds ratio [mOR], 8.1; attributable fraction [AF], 0.17; P<.001) and consumption of homemade juice (mOR, 1.8; AF, 0.10; P=.01); protective factors included boiling of the baby bottle or nipple (mOR, 0.60; AF, 0.19; P=.026), childcare at home (mOR, 0.58; AF, 0.12; P=.004), and piped sewage (mOR, 0.58; AF, 0.05; P=.047). Hand washing by the caretaker after helping the child defecate protected against Shigella infection (mOR, 0.35; P<.05). Preparation of rice, beans, or soup in the morning and serving it to children after noon were associated with enterotoxigenic Escherichia coli infection (mOR, 8.0; P<.05). In these poor households, 28% of cases of diarrhea in 1-4-year-old children was attributable to easily modifiable exposures.

Lack of effect of GnRH agonists on final height in girls with advanced puberty: a randomized long-term pilot study.

GnRH agonists improve final height in girls with "true" precocious puberty. To test if a comparable effect can be obtained in older girls, we performed a long-term controlled study in 30 caucasian girls whose puberty started between 8.4 and 10 yr (9.4 +/- 0.1 yr), a variant of normal called "advanced" puberty. At entry into trial, these girls had clinical, biological, and sonographic manifestations of puberty and a bone age greater than 10.9 yr. They were randomized 2:1 to receive 3.75 mg triptorelin im every 4 weeks for 2 yr (n = 20, group I) or no treatment (n = 10, group II). Mean height at inclusion was 135.2 +/- 4.3 cm (+0.6 SDS) in group I, 136.1 +/- 4.2 cm (+0.8 SDS) in group II, with target height 157.6 +/- 4.3 cm (group I) and 157.8 +/- 4.7 cm (group II), and predicted height (Bayley-Pinneau) 154.1 +/- 3.9 cm and 155.2 +/- 3.7 cm. Although GnRH agonists transiently delayed sexual maturation as well as bone age and growth rate, they had no clear-cut long-standing effect, and final height was comparable in treated (157.6 +/- 4.0 cm) and untreated girls (156.1 +/- 5.3 cm) (NS).

Diagnostic sonography of HIV-associated nephropathy: new observations and clinical correlation.

OBJECTIVE: HIV-associated nephropathy is an important cause of morbidity that is characterized clinically by uremia and proteinuria and histologically by focal segmental glomerulosclerosis. In the largest series yet analyzed to our knowledge, we describe new sonographic findings and record the prevalence of other findings. We review the sonographic findings in a large group of HIV-infected patients. MATERIALS AND METHODS: Seventy-six consecutive HIV-infected patients underwent renal sonography. Abnormalities seen on sonography were recorded. RESULTS: Of 152 kidneys imaged, sonography showed that 30 kidneys (20%) were enlarged. Abnormal echogenicity was present in 136 kidneys (89%). Eighty-one kidneys (53%) were globular; 58 (38%) had decreased corticomedullary definition; 74 (49%) had decreased renal sinus fat; and 66 (43%) had heterogeneous parenchyma, some with echogenic striations. CONCLUSION: Our data reveal several sonographic abnormalities that have not previously been described: decreased corticomedullary definition, decreased renal sinus fat, parenchymal heterogeneity, and globular renal configuration. These new findings were found mainly in patients with advanced HIV infection.

The safety and efficacy of adefovir dipivoxil, a novel anti-human immunodeficiency virus (HIV) therapy, in HIV-infected adults: a randomized, double-blind, placebo-controlled trial.

Adefovir dipivoxil is a novel nucleotide analogue with several promising in vitro anti-human immunodeficiency virus (HIV) characteristics. To evaluate the safety and efficacy of adefovir dipivoxil monotherapy, a randomized, double-blind, placebo-controlled study was initiated involving 72 subjects with moderately advanced HIV disease. Subjects were randomly assigned in a 2:1 ratio to receive adefovir dipivoxil or placebo as a once-daily oral dose for 6 weeks, followed by 6 weeks of open-label adefovir dipivoxil. Two dose levels were studied (125 mg and 250 mg). Adefovir dipivoxil was determined to be safe and well-tolerated when administered for 12 weeks. At week 6, changes in absolute CD4 T cell levels and HIV-1 RNA levels were significantly greater with adefovir dipivoxil than with placebo. These effects were sustained through 12 weeks of treatment. As determined by standard RNA sequencing techniques, only 1 of the 24 subjects who received adefovir dipivoxil (125 mg/day) developed any genotypic change from baseline.

Surgical correction of blepharoptosis in oculopharyngeal muscular dystrophy.

Progressive, usually symmetric blepharoptosis with or without dysphagia appears in most instances in the fifth decade in oculopharyngeal muscular dystrophy (OPMD). We review our experience over 20 years of applying Beard's surgical guidelines for correction of ptosis to OPMD patients with satisfactory results. As the disease continues to progress, the rate of recurrence of ptosis among follow-up patients of a 9-year minimum period was 13%.

Geographic variation in the management and outcome of patients with AIDS-related Pneumocystis carinii pneumonia.

Pneumocystis carinii pneumonia (PCP) is one of the most common reasons for the hospitalization of AIDS patients; however, geographic differences in PCP management have not been evaluated previously. Therefore, we abstracted data on socioeconomic characteristics, prior HIV care, severity of illness, timeliness and intensity of in-hospital care, duration of hospitalization, and survival from 1547 randomly selected medical records of patients hospitalized with AIDS-related PCP between 1987 and 1990 at 82 hospitals in Chicago, Los Angeles, Miami, New York City, and Raleigh-Durham, North Carolina. Multivariate regression models were used to assess factors associated with longer hospital stays and increased inpatient mortality. Our results showed that in-hospital mortality ranged from 15% to 27%, bronchoscopy rates from 53% to 70%, and mean length of stay from 14 days to 23 days. Geographic variations in mortality were accounted for by differences in severity of illness at admission, insurance status, and in-hospital patient management. However, significant regional variations in hospital length of stay persisted, even after adjusting for patient demographics, severity of illness, and use of diagnostic and therapeutic care resources.

Gonadotropin-releasing hormone agonist treatment of girls with constitutional short stature and normal pubertal development.

GnRH agonists have been proposed to improve final height in patients with constitutional short stature. We treated 31 girls, aged 11.9 +/- 1 yr (mean +/- SD), with short stature, recent pubertal onset and predicted final height of 155 cm or less with depot triptorelin. During the 23 +/- 4 months of treatment, bone age progression was 0.6 +/- 0.3 bone age yr/yr, and growth velocity declined from 7 +/- 2 to 4 +/- 0.8 cm/yr (P < 0.0001). Height prognosis, calculated by the Bayley-Pinneau method, progressed from 149.6 +/- 3.4 to 151.8 +/- 4 cm at the end of treatment (+2.2 +/- 2.6 cm; P < 0.0001). When treatment was interrupted, growth velocity slightly increased to 4.6 +/- 1.6 cm/yr, and bone age maturation was accelerated: 1.3 +/- 0.4 bone age yr/yr during the first posttreatment year. At the last visit, 26 +/- 9 months after interruption of treatment, bone age was 14.9 +/- 1.3 yr (> or = 13.5 yr in all patients), height was 149.1 +/- 4 cm, and final height prognosis was 150.6 +/- 3.6 cm. Final height prognosis was 1 +/- 2.3 cm greater than pretreatment height prognosis (P < 0.02) and 1.2 +/- 2.2 cm below the height predicted at the end of the treatment (P < 0.01). No major side-effect was observed. Height SD score decreased during treatment with GnRH agonist from -2.3 +/- 0.9 to -2.7 +/- 0.7 SD score (P < 0.0001). We conclude that 2 yr of depot triptorelin-induced pubertal delay has a limited effect on near-final height in girls with constitutional short stature and that the growth benefit observed does not currently justify the use of GnRH agonists, given their cost and potential side-effects.

A university outbreak of Escherichia coli O157:H7 infections associated with roast beef and an unusually benign clinical course.

An outbreak of Escherichia coli O157:H7 infections occurred after a graduation banquet at a university in Wisconsin. Sixty-one (32%) of 193 banquet attendees developed a gastrointestinal illness; 2 were hospitalized, none developed hemolytic-uremic syndrome or thrombotic thrombocytopenic purpura, and none died. The spectrum of illness was unusually mild, with 61% of ill persons reporting nonbloody diarrhea. A strain of E. coli O157:H7, indistinguishable from the outbreak strain by toxin type, plasmid profile, and pulsed-field gel electrophoresis, was isolated from an unopened package of an uncooked round of beef from the original shipment of meat. An investigation suggested that both undercooked roast beef and salad cross-contaminated with beef were vehicles of transmission. These findings demonstrate that meat from beef cattle may transmit E. coli O157:H7, and such infections among young to middle-aged adults may be mild and may often go undetected.

Outbreaks of Salmonella enteritidis infections in the United States, 1985-1991.

The spread of Salmonella enteritidis infections in the United States was tracked to identify potential risk factors and preventive measures. Isolation rates and information regarding outbreaks of S. enteritidis from 1985 through 1991 were determined by reports to the national Salmonella surveillance system and through the foodborne disease outbreak surveillance system. From 1985 through 1991, 380 outbreaks were reported involving 13,056 ill persons and 50 deaths. The proportion of Northeast outbreaks fell from 81% in 1985 to 55% in 1991 as the number of outbreaks in other areas increased. Grade A shell eggs were implicated in 82% of outbreaks. Case-fatality rates in nursing homes and hospitals were 70 times higher than in other settings. Cultures of environmental or animal specimens from all farms tested yielded S. enteritidis. Eggborne S. enteritidis infections are a major public health problem. Preventive measures, including educating consumers about proper handling of eggs, using pasteurized eggs, and controlling infections on egg farms, may stem the impact of this disease.

Correction of blepharoptosis in oculopharyngeal muscular dystrophy: review of 91 cases.

Oculopharyngeal muscular dystrophy is a progressive, hereditary myopathy in which the levator palpebrae and the pharyngeal muscles are selectively involved. Progressive, usually symmetric blepharoptosis with or without dysphagia appears in the fifth decade. The high prevalence of this myopathy in Quebec stems from the immigration of a single couple from France in 1634, whose numerous descendants manifest its autosomal dominant heredity. We review our experience in 91 cases over 20 years. The ptosis was moderate in 83% of the cases at the time of surgery, and the average age was 65 years. Beard's surgical guidelines for correction of ptosis based on the degree of ptosis and residual levator function were applied directly in cases of primary intervention, with consistently satisfactory results. This is in contrast to other types of hereditary myopathic ptosis, in which the guidelines must be modified. The rate of recurrence of ptosis among patients followed for at least 9 years was 13%.

[Immunity to and incidence of measles in a population of Parisian children]. / Immunité antirougeoleuse et incidence de la maladie dans une population d'enfants parisiens.

BACKGROUND: Measles remains prevalent in France despite the development of nationwide vaccination program. This study evaluates measles immunity and correlates it with active immunization. MATERIAL AND METHODS: A total of 250 (131 boys and 119 girls) French children, aged 2 to 15 years, seen as outpatients in our hospital, were studied from 1.01.90 to 1.06.91; 133 had received live measles vaccine during the 2nd year of life, the 117 others were not vaccine-protected. Evaluation included medical records, specially those concerning past-history of measles or measles-like diseases and immunizations. Measles IgG and IgM antibodies (ELISA) were looked for in all 250 children. RESULTS: 102 of the 117 children who were not vaccine-protected had a detectable antibody titer. 42 of these 102 had a history of measles, before the age of 5 years in 30, between 5 and 10 years in 9 and after the age of 10 years in 3. Only 4 of the 70 children aged over 10 years and not vaccine-protected had no detectable antibodies. 131 of the 133 actively immunized children had detectable antibodies. CONCLUSIONS: Measles is probably more frequently subclinical than was believed until now. Its relative frequency in France in children who had not received the live vaccine explains the high percentage of those aged over 10 years who had detectable antibodies and the relatively low incidence of the disease in teenagers and young adults.

Comparison of plasmid profiles, phage types, and antimicrobial resistance patterns of Salmonella enteritidis isolates in the United States.

To evaluate the laboratory techniques for subtyping isolates of Salmonella enteritidis, we compared the plasmid profiles (PP), phage types (PT), and antimicrobial susceptibility patterns (AS) of two nationally representative samples of sporadic human S. enteritidis isolates from 1979 (n = 28) and 1984 (n = 37), 43 isolates from 20 outbreaks of S. enteritidis infections between 1983 and 1987, and 46 animal isolates selected from the U.S. Department of Agriculture Veterinary Services Laboratory in 1986 and 1987. Sporadic and outbreak isolates from humans showed similar rates of resistance to at least one of a panel of antimicrobial drugs (23 and 14%, respectively), PT (91 and 98%, respectively), and PP (97 and 100%, respectively). Sixteen different PP were identified in sporadic, outbreak, and animal isolates; two PP accounted for 76% of sporadic and outbreak isolates. Sporadic human isolates were of PT 8 (42%), of PT 13a (37%), nontypeable (9%), of PT 14b (8%), of PT 9a (3%), and of PT 13 (2%). Outbreak human isolates had similar distributions of PT. PT 8 was associated with poultry: 58% (7 of 12) of the poultry isolates but only 24% (8 of 34) of the isolates from other animals were of PT 8 (P less than 0.04). Although antimicrobial susceptibility patterns do not appear as useful as an epidemiologic marker, PP and PT effectively subtyped S. enteritidis.

International increase in Salmonella enteritidis: a new pandemic?

Over the past 5 years Salmonella enteritidis infections in humans have increased on both sides of the Atlantic ocean. The WHO salmonella surveillance data for 1979-87 were reviewed and show that S. enteritidis appears to be increasing on at least the continents of North America, South America, and Europe, and may include Africa. S. enteritidis isolates increased in 24 (69%) of 35 countries between 1979 and 1987. In 1979, only 2 (10%) of 21 countries with reported data reported S. enteritidis as their most common salmonella serotype; in 1987, 9 (43%) of 21 countries reported S. enteritidis as their most common serotype; 8 (89%) of 9 were European countries. Although the reason for the global increase is not yet clear, investigations in individual countries suggest it is related to consumption of eggs and poultry which harbour the organism.

Lethal paralytic shellfish poisoning in Guatemala.

An outbreak of paralytic shellfish poisoning occurred in Champerico, on the Pacific coast of Guatemala, July-August 1987. Of 187 people affected with characteristic neurologic symptoms, 26 died. A case study implicated a species of clam, Amphichaena kindermani, harvested from local beaches as the vehicle of the neurotoxins (saxitoxins). Children less than 6 years old had a higher fatality rate (50%) than people greater than 18 years of age (7%). The minimum lethal dose for 1 child was estimated to have been 140 mouse units of toxin/kg body weight; thus children may be more sensitive to the saxitoxins than are adults. This is the first large outbreak of paralytic shellfish poisoning recognized in Guatemala.

[Neonatal pneumococcus laryngitis]. / Laryngite néonatale à pneumocoque.

Report of a case of laryngitis beginning within the first 10 hours of life. The epiglottis was red, swollen, and covered by a membrane. Culture of laryngeal swabs isolated Streptococcus pneumoniae. Complete recovery was obtained with antibiotic therapy.

The evaluation of forearm density with axial tomography.

The purpose of the present investigation was to validate axial tomography technique for estimating forearm density. Twenty forearm specimens from six male and four female embalmed human cadavers were used. Segmental mass, volume and density criteria were obtained by weight in air, hydrostatic weight and mass/volume ratio, respectively. Densities of bones and muscles were similarly measured. Segmental density as measured by axial tomography was overestimated by an average of 2.1% and the errors ranged from 0.05 to 4.81%. Results from this study indicated the presence of three density regions in the segment. Moreover, density data were found to be very similar in all zones of the central region of the segment (2/3 of its length) to actual segment density. It is thus actually possible to estimate forearm density in living humans, by taking two scans in the central zone of the segment if data pertaining to tissue densities are available.