Managing the hair and skin of African American pediatric patients.

In Africa, the ancestral home of most African Americans, hair is viewed as the epitome of beauty. However, when Africans were brought to America as slaves, they were unable to care for their hair and skin adequately and were exposed to the predominant white culture, which valued straight hair and light skin. As a result, many African Americans lost self-esteem because of the characteristics of their hair and skin. In this article we examine the anatomic and physiologic features of African American hair and skin and typical African American hair and skin care practices. Common African American hair and skin disorders and their management are discussed. The goal of this article is to help primary care providers understand the special hair and skin care required for African American children (as well as other dark-skinned patients). With good patient education, understanding one's own hair and skin characteristics can also support positive self-esteem.     

Physicochemical hair conformation of patients with Sanfilippo disease type IIIA

Sanfilippo disease type IIIA is an inborn error of metabolism with a deficiency in the heparan sulfamidase. Besides severe psychomotor retardation hair changes are obligatory. Hair is found to be coarse like a brush. We applied X-ray diffraction and infrared spectroscopy to characterize the conformation of hair samples of Sanfilippo patients. In healthy subjects as well as in the affected hair samples we found the wave numbers of structural relevance 1450, 1500, 1630, 1730, the pair 2337 and 2362, the quadruplet 2850, 2870, 2917, 2930 and 3080 cm-1. Also on X-ray diffraction analysis no differences could be detected. Though morphological-macroscopically and microscopically-changes were described for Sanfilippo hair samples, we could not find any change in supramolecular structure. The physical properties of coarseness of those hair specimen seems to be due to differences in the structural assembly of hair fibres and storage of heparan sulfate.     

Hair loss in children and adolescents

No other body system is more exposed to the public eye than the skin and the hair. Pediatric nurse practitioners (PNPs) frequently encounter patients in their practice settings with the complaint of "hair loss." The most often diagnosed causes of hair loss include tinea capitis, alopecia areata, traction alopecia, and trichotillomania. This article defines these conditions, explores the causes for them, and describes the incidences. Emphasis is placed on the PNP's role on making a differential diagnosis based on the presenting clinical manifestations, patient history, and laboratory testing. Treatment for these conditions as well as nursing goals and PNP interventions for management and family education also are discussed.     

Urethroscopic holmium:YAG laser epilation of urethral diverticular hair follicles following hypospadias repair

Urethral hair and diverticula are well described long-term complications following urethroplasty or hypospadias repair. Endoscopic laser ablation is an uncommonly reported treatment option for a symptomatic hair-bearing urethra, and holmium laser hair epilation has not been previously reported in the literature. We report the first case of urethroscopic holmium laser ablation of urethral diverticular hair follicles and the outcome.     

Steroid profiles in serum by liquid chromatography-tandem mass spectrometry in infants with genital hair

BACKGROUND: In recent years, an increasing number of infants have been seen with fine hair in the genital area and no other signs of androgen excess, but the hormonal basis of this finding is unknown. AIM: To compare steroid profiles in infants with genital hair with age-matched control infants, using liquid chromatography-tandem mass spectrometry (LC-TMS) to measure eight steroids (cortisol, 11-deoxycortisol, progesterone, 17-hydroxyprogesterone, testosterone, androstenedione, DHEA, and DHEA-S). PATIENTS: Samples were obtained between 1/04 and 12/05 from infants with genital hair, and for comparison, a group of 5-9 year-olds with premature adrenarche, as well as control children of similar ages being seen for thyroid problems or short stature. RESULTS: Steroid profiles in infants with genital hair were similar to those in control infants, except that DHEA-S levels were somewhat higher (17.5 vs. 7.6 microg/dl [476 vs. 207 nmol/l]; p = 0.067), and six of 12 had levels >15 microg/dl (408 nmol/l) vs. one of 12 controls. Testosterone levels were low (<10 ng/dl [<350 pmol/l) in nearly all infants with pubic hair; the main exception was a girl whose father used topical testosterone (31 ng/dl [1076 pmol/l]). Genital hair disappeared in two patients over time but persisted for 6 months to 2 years in most. CONCLUSIONS: No pathological increase in steroid levels was found in infants with genital hair vs controls, though a mild elevation of DHEA-S was seen in about half. This suggests that pubic hair in infancy may represent a mild and early onset variant of premature adrenarche, with a benign clinical course.     

Cortisone in hair of elementary school girls and its relationship with childhood stress

Children may be exposed to stressful situations with adverse effects on their physiological and psychological health. As cortisone may be a useful additional biomarker for stress research and as it has been shown to be detectable in human hair, this study measured physiological concentrations of hair cortisone in 223 elementary school girls and explored its relationship with child-reported estimates of stress, more specifically questionnaires on major life events (i.e., Coddington Life Events Scale for Children), emotions (i.e., anger, anxiety, sadness, and happiness), and coping strategies (i.e., emotion- versus problem-focused coping). Cortisone concentrations were positively correlated with the overall life event score for the past 6 months (rho?=?0.223, p?=?0.004), as well as with the negative event score for this period (rho?=?0.227, p?=?0.003; N?=?165). Cortisone did not correlate with emotions or coping styles reported by the children. Conclusion: Despite its exploratory nature, this study may suggest elevated hair cortisone concentrations under psychosocial stress in young children. Although the observed findings should be interpreted with prudence, this study may encourage further research elucidating the potential importance and relevance of hair cortisone analysis as an additional or substituting stress biomarker for hair cortisol.     

Untersuchung der LH-, Östrogen- und Testosteronausscheidung im Vergleich zur Testesgröße

In 60 boys aged 6 to 15 years testicle size and the stages in the development of the genitalia as well as of pubic and axillary hair were correlated with the urinary excretion of LH, estrogens and testosterone. An increase in testicle size and in estrogen excretion was measured from 9 to 10 years onward, 1 year later testosterone excretion increases. At age 11 to 12 years development of the genitalia commences, followed 1 year later by that of pubic and axillary hair. From 12 to 13 years a real increase in LH excretion could be measured with our method.     

Trichothiodystrophy with severe cardiac and neurological involvement in two sisters

Trichothiodystrophy or sulphur-deficient brittle hair is a clinical marker for several autosomal recessive neurocutaneous syndromes. The typical hair abnormality is frequently associated with many alterations affecting the skin, nervous system, eyes and bones as well as the immune, gonadal and endocrine systems. We report the first cases of dilated cardiomyopathy in two sisters with trichothiodystrophy, leading to cerebral infarction in the younger one. In addition, both suffer from severe hearing impairment, osteosclerosis, and psychomotor retardation with central hypomyelination. CONCLUSION: Severe cardiac involvement and stroke may be associated features of trichothiodystrophy.     

Pubertal stages 1980 of Stockholm schoolchildren

In March 1980 a cross-sectional study of the pubertal development of a sample of Stockholm schoolchildren was conducted. Breast development (B1-B5), pubic hair (PH1-PH5) and axillary hair (AH1-AH3) were rated in 138 girls, and genitalia development (G1-G5), pubic hair (PH1-PH5) and axillary hair (AH1-AH3) were rated in 116 boys according to the Tanner method. The girls'and the boys'ratings were done by one female and one male medically qualified expert respectively. When relating social class to age of pubertal stage no significant differences were found. Median ages for entry to successive pubertal stages as well as centiles were calculated by logistic regression. Girls were somewhat earlier in their breast and pubic hair development than Stockholm girls investigated in earlier studies; however, somewhat later in their development of axillary hair. The boys entered G2 earlier than boys studied in 1970 but entered G5 at exactly the same age, 15.1 years. Boys entered PH2 somewhat later than 1970 boys, but again reached PH5 at exactly the same age, 15.5 years. The present study is based on a more recent and somewhat larger sample of children drawn from a wider area of Stockholm than the earlier studies. It is therefore suggested that these values be used in the proposed new height and weight reference values for youth in Sweden.     

The tay syndrome (congenital ichthyosis with trichothiodystrophy)

We report a 5-year-old boy affected with the Tay syndrome, and give a review of 12 pertinent cases previously reported under various designations. The Tay syndrome is a distinct type of congenital ichthyosis characterized by a peculiar anomaly of hair growth which has been termed trichothiodystrophy. The hair shafts are extremely brittle, and they show alternating light and dark banding when examined microscopically between polarizing filters. Other features of this syndrome are low birth weight, short stature, mental retardation, delayed neuromuscular development and other CNS anomalies, dysplasia of nails, hypoplasia of subcutaneous fatty tissue, prematurely aged facial appearance, hypogonadism, cataracts, osteosclerosis, dysphonia, and increased susceptibility to infections. The syndrome is inherited as an autosomal recessive trait. We delineate the criteria for distinguishing this gene defect from other types of congenital ichthyosis associated with disturbed hair growth, as well as from other types of trichothiodystrophy which are not associated with ichthyosis.     

Clinical longitudinal standards for height, weight, height velocity, weight velocity, and stages of puberty

New charts for height, weight, height velocity, and weight velocity are presented for clinical (as opposed to population survey) use. They are based on longitudinal-type growth curves, using the same data as in the British 1965 growth standards. In the velocity standards centiles are given for children who are early- and late-maturing as well as for those who mature at the average age (thus extending the use of the previous charts). Limits of normality for the age of occurrence of the adolescent growth spurt are given and also for the successive stages of penis, testes, and pubic hair development in boys, and for stages of breast and pubic hair development in girls.     

Clinical longitudinal standards for height,weight, height velocity,weight velocity,and stages of puberty.

New charts for height, weight, height velocity, and weight velocity are presented for clinical (as opposed to population survey) use. They are based on longitudinal-type growth curves, using the same data as in the British 1965 growth standards. In the velocity standards centiles are given for children who are early- and late-maturing as well as for those who mature at the average age (thus extending the use of the previous charts). Limits of normality for the age of occurrence of the adolescent growth spurt are given and also for the successive stages of penis, testes, and pubic hair development in boys, and for stages of breast and pubic hair development in girls.     

Cartilage‐hair hypoplasia associated with isolated hypoganglionosis: A case report

Cartilage‐hair hypoplasia is a rare metaphyseal chondrodysplasia characterized by diverse clinical manifestations and a high incidence of Hirschsprung disease. We present a male patient with cartilage‐hair hypoplasia associated with severe intestinal obstruction. Genetic analysis of ribonuclease mitochondrial RNA‐processing complex gene identified compound heterozygous mutations consisted with previously reported mutations: n.‐14_3dupGAAGCTGAGGACGTGGT and n.183G > T. First, we considered that intestinal obstruction was due to an extensive type of Hirschsprung disease, but it was later confirmed as isolated hypoganglionosis. Isolated hypoganglionosis is rare and its therapeutic strategies are not well established. In cases of cartilage‐hair hypoplasia associated with severe intestinal obstruction, the differential diagnosis of not only Hirschsprung disease, but also isolated hypoganglionosis, should be considered.     

Hypertrichosis cubiti (hairy elbow syndrome): a clue to a malformation syndrome

Hypertrichosis cubiti (HC) or hairy elbow syndrome (OMIM # 139600) consists of a localised form of long vellus hair on the extensor surfaces of the distal third of the upper arm and the proximal third of the forearm bilaterally, or occasionally on other parts of the body. In the 28 cases reported in the literature so far the elbow hair abnormality was either isolated or associated with short stature or other physical abnormalities. Most of these cases were sporadic, but autosomal dominant as well as autosomal recessive inheritance patterns have been postulated. We report on three unrelated girls (aged 7 to 11 years) of whom one presented with excess hair in the elbows alone and the other two had associated abnormalities including short stature, dysmorphic facial features and mental retardation. The literature on this subject has been reviewed and the authors focus on cases of HC with associated anomalies. A pathogenic explanation by somatic mosaicism is proposed.     

Zinc status in well nourished Bangladeshi children suffering from acute lower respiratory infection

This study was done to investigate the association of zinc status of well nourished Bangladeshi children with Acute Lower Respiratory Infection (ALRI). In this case control study, simultaneous estimation of serum and hair zinc was performed in 35 well nourished children, aged 6-60 months suffering from ALRI and 38 normal matched controls. Serum zinc was low in children suffering from ALRI as compared to control (90 +/- 51 pg/dL vs 176 +/- 98 pg/dL, OR: 6, 95% CI: 1.83, 19.66;P <0.05) children. Hair zinc was also found significantly low in children suffering from ALRI as compared to control (158 + 48 1g/g vs 247 154 pg/g, OR: 3, 95% CI: 1.46, 10.04, P < 0.05). We conclude that Bangladeshi children suffering from pneumonia, have decreased levels of serum and hair zinc.     

Determination of hair trace elements in childhood celiac disease and in cystic fibrosis.

Applying proton-induced X-ray emission authors investigated the hair trace element contents in 10 children with acute celiac disease after 3 to 6 and 12 months long gluten-free diet; in 9 children with cystic fibrosis and in a control group (6 children) of the same age. There was no difference in Cu, Fe, Ca, Cl values between the examined groups. The Zn contents of the hair are significantly low in the group with acute celiac disease after a short-term diet and also in the group with cystic fibrosis, the data approach the normal range only after a year's diet. The significant rise of hair potassium contents is well indicated in patients with acute celiac disease and this rise may be due to the destruction of cell cuticles. In case of cystic fibrosis there is no significant rise of hair potassium value.     

An Approach to Hair Loss in Pediatric Primary Care

Pediatric hair loss is a cause of concern for patients and families. Early diagnosis and treatment are crucial, as delays in care for certain diagnoses can cause permanent scarring alopecia. The evaluation of hair loss includes a thorough history, physical examination, and other potential tests. The causes of hair loss can be classified as either acquired versus congenital. Acquired causes of hair loss can be subdivided into scarring and nonscarring; however, some conditions may present as nonscarring and progress to scarring alopecia. Recommendations for the evaluation and treatment of pediatric hair loss for the primary care practitioner will be summarized.     

Near strangulation as a result of hair tourniquet syndrome

Hair tourniquet syndrome has been a recognized clinical entity for many decades. In this syndrome, hair becomes tightly wrapped around an appendage, usually that of an infant. Body parts involved include the finger, toe, penis, clitoris, and uvula. The end result of necrosis is also well documented. In this report, an 11-month-old girl with hair tourniquet syndrome causing signs and symptoms of an acute strangulation is presented.     

Zinc nutriture in type I diabetes mellitus: relationship to growth measures and metabolic control

Zinc concentrations in plasma, hair, and urine from children and young adults with insulin-requiring or Type I diabetes mellitus were significantly correlated with height, weight, and age, as well as with indices of metabolic control, i.e., fasting serum glucose, percent glycosylated hemoglobin (HbA1), and 24-h urine glucose and insulin excretion. Urinary zinc excretion was greater in subjects than in controls (p less than 0.0001) and significantly correlated with urine glucose (p less than 0.004, r = 0.35) and volume (p less than 0.0007, r = 0.40). Urinary zinc and volume were not correlated in controls. Hyperzincuria in the subjects was not secondary to hyperinsulinuria, although zinc and insulin excretion were significantly correlated in controls (p less than 0.03, r = 0.63). Zinc in insulin preparations could not explain the excessive zinc excretion. Mean fasting plasma zinc was significantly higher than in controls, and positively correlated with height for age, while being inversely correlated with age, duration of diabetes, HbA1, urine volume, and glucose excretion. Both the mean and range of hair zinc concentration in the subjects were not different from controls. Male subjects with diabetes had a significantly lower hair concentration when compared with female subjects with diabetes (p less than 0.0009). Zinc homeostasis appears to be altered as a consequence of glucose intolerance in diabetes. Continued urinary zinc losses over time may result in a zinc deficiency state not demonstrable by altered zinc concentrations in plasma and hair.     

Embryology of the hair follicle.

A study of hair follicle development has enabled us to differentiate four stages by relating them to corresponding gestation ages. Stage I of the hair bud is characterized by an epiblastic proliferation penetrating the subjacent mesenchyma at the extremity of which mesenchymal cells accumulate. This appears, at the level of the lower lip, before the 11th week of intrauterine life. Stage II of the hair bulb is reached on the 12th-13th week of intrauterine life, as soon as the distal extremity of the hair bud, which has extended, becomes depressed at the mesenchymal papilla level. Stage III is defined by the observation of hair cone and rough sebaceous glands on the 15th week of intrauterine life. Finally, stage IV shows a differentiated sebaceous gland with a hair which comes through the skin surface on the 18th week of intrauterine life.