Comorbidities of alopecia areata in infancy and childhood. A small descriptive study in a tertiary hospital in Greece

Sir, A small descriptive study in a tertiary hospital in Greece was conducted on the comorbidities of alopecia areata in infancy and childhood. Alopecia areata is a non-scarring alopecia of autoimmune origin linked also to genetic and environmental factors [1], affecting 2% of the general population and is considered a disease of young adults. Attempts have been made to detect the comorbidities in infants and children suffering from alopecia areata. Sorell Jennifer et al. established a strong association of alopecia areata with atopy, psoriasis thyroid disease, and juvenile idiopathic arthritis [2]. More recently, Comiz et al. [3] added anemia, obesity, vitamin D deficiency, hypothyroidism, vitiligo, psoriasis, hyperlipidemia, and depression to the list of the comorbidities detected in the pediatric population with alopecia areata. The purpose of the study was to detect the comorbidities in infants and children with alopecia areata in an outpatient dermatology clinic during a period of six years from 2013 through 2019. All those examined as outpatients and those hospitalized for several reasons in the pediatric ward who were diagnosed with alopecia areata were included in the study. Laboratory tests, a full blood count, and vitamin D, IgE, and thyroid tests were performed in the laboratories of our hospital. During these seven years, 71 patients were diagnosed with alopecia areata and 7 (approx. 10%) were children. Four (57.1%) were males, and the rest three were females. The males were aged 23 months, and 6-, 7-, and 11-years. The females were 2-, 7-, and 11-year-old. Clinical atopy confirmed by high levels of IgE in the serum was detected in two males and in all three females. Thyroid dysfunction, hypothyroidism, was only detected in one infant associated with atopy; this was in a 23-month-old who at the time of the diagnosis of alopecia areata was hospitalized with severe asthma. Vitamin D deficiency was found in one male patient. A family history of alopecia areata was found in only one male patient. A family history of atopy was reported in only one boy, aged 7 years. A family history of thyroid dysfunction was detected in two males 28%: The 23-month-old infant whose father suffered from hyperthyroidism and the 12-year-old male whose both parents suffered from hypothyroidism. A family history of rheumatoid arthritis was found in one female patient. All patients presented with a mild disease limited to the scalp at the time of diagnosis. No nail pitting was observed, and neither clinical signs of psoriasis, nor of vitiligo. Folliculitis of the scalp preceded the onset of alopecia areata in one of the females (Table 1). Although males comprised 57.1% of our cases, most studies have found a preponderance of females in the pediatric population with AA. Atopy was the most frequent comorbidity (5/7; 70%) and was more frequent in females; all three girls were atopic. The second most frequently found comorbidity was thyroid dysfunction, hypothyroidism., detected in one patient (14%). Vitamin D deficiency was noted in one (14%) patient. A family history of AA was found in one patient as well as a family history of atopy. A family history of thyroid dysfunction was found in two patients (28%). The precipitating factor in our case was staphylococcal infection of the scalp. Staphylococcus, probably acting as a super antigen, was observed in only one patient (14%). Both atopy and thyroid dysfunction should be sought for in pediatric patients with AA in this order.

Recent advancement in the treatment of boys and adolescents with hypogonadism

Clinical manifestations and the need for treatment varies according to age in males with hypogonadism. Early foetal-onset hypogonadism results in disorders of sex development (DSD) presenting with undervirilised genitalia whereas hypogonadism established later in foetal life presents with micropenis, cryptorchidism and/or micro-orchidism. After the period of neonatal activation of the gonadal axis has waned, the diagnosis of hypogonadism is challenging because androgen deficiency is not apparent until the age of puberty. Then, the differential diagnosis between constitutional delay of puberty and central hypogonadism may be difficult. During infancy and childhood, treatment is usually sought because of micropenis and/or cryptorchidism, whereas lack of pubertal development and relative short stature are the main complaints in teenagers. Testosterone therapy has been the standard, although off-label, in the vast majority of cases. However, more recently alternative therapies have been tested: aromatase inhibitors to induce the hypothalamic-pituitary-testicular axis in boys with constitutional delay of puberty and replacement with GnRH or gonadotrophins in those with central hypogonadism. Furthermore, follicle-stimulating hormone (FSH) priming prior to hCG or luteinizing hormone (LH) treatment seems effective to induce an enhanced testicular enlargement. Although the rationale for gonadotrophin or GnRH treatment is based on mimicking normal physiology, long-term results are still needed to assess their impact on adult fertility.

Indications and long-term results of enteral feeding in infancy and childhood

Enteral feeding is more favorable than parenteral one because it can significantly intervene against the colonization of bacteria and preserve gut functions. However, this necessitates the presence of a good-functioning gastrointestinal tract. Young infants, critically ill children, and patients with neurological disabilities are the most probable candidates to perform enteral feeding. In the present literature review, we have discussed the indications and long-term results of enteral feeding in pediatric settings. Our results show that modality is a safe and efficacious modality in these settings with favorable outcomes and fewer adverse events and complications. Many indications were reported for the modality, and in general, children that usually suffer from severe weight deficit, weight faltering, and growth retardation are indicated to receive enteral nutrition. Some contraindications were also reported in the literature, and in general, conditions affecting the function and health status gastrointestinal tract should recommend against conducting approaches of enteral feeding. Different complications were reported, including mechanical, metabolic, infectious, gastrointestinal, and drug-related complications that might lead to worsened prognosis and can significantly impact the long-term outcomes of these patients. Therefore, paying adequate attention should be considered in these cases to prevent the development of these complications, and provide all the necessary procedures to potentially manage the expected adverse events.

Epigenome-Wide Association Study of Infant Feeding and DNA Methylation in Infancy and Childhood in a Population at Increased Risk for Type 1 Diabetes

We assessed associations between infant diet (e.g., breastfeeding and introduction to solid foods) and DNA methylation in infancy and childhood. We measured DNA methylation in peripheral blood collected in infancy (9–15 months of age) in 243 children; and in a subset of 50 children, we also measured methylation in childhood (6–9 years of age) to examine persistence, and at birth (in cord blood) to examine temporality. We performed multivariable linear regression of infant diet on the outcome of methylation using epigenome-wide and candidate site approaches. We identified six novel CpG sites associated with breastfeeding duration using an EWAS approach. One differentially methylated site presented directionally consistent associations with breastfeeding (cg00574958, CPT1A) in infancy and childhood but not at birth. Two differentially methylated sites in infancy (cg19693031, TXNIP; cg23307264, KHSRP) were associated with breastfeeding and were not present at birth; however, these associations did not persist into childhood. Associations between infant diet and methylation in infancy at three sites (cg22369607, AP001525.1; cg2409200, TBCD; cg27173510, PGBD5) were also present at birth, suggesting the influence of exposures other than infant diet. Infant diet exposures are associated with persistent methylation differences in CPT1A, which may be one mechanism behind infant diet’s long-term health effects.

Eccrine Angiomatous Hamartoma: A Rare Presentation in Late Adulthood

Eccrine angiomatous hamartoma is a tumor characterized by benign malformation of eccrine and vascular elements, occurring generally at birth or during early infancy and childhood. The rarity with which it is encountered in adults and the elderly makes it a diagnostic challenge in such age brackets. We report an interesting case of eccrine angiomatous hamartoma presenting in late adulthood confirmed by histopathology. Keywords: Cisplatin, concurrent chemotherapy, daily, weekly, three weekly, carcinoma cervix

Concluding Discussion to Part I

In this concluding discussion, evidence is brought together from the different categories of dress objects considered to examine further how people were assisted by objects in achieving social goals, and how these objects contributed to daily experience. Initially, the artefacts are examined from a life course perspective, in order to further illuminate the important roles and functions of dress objects at particular life course stages, a theme already evident in previous chapters. Particular types of dress objects associated with the life course stages of infancy and childhood, puberty up to menopause, and post-menopause, and their roles in life course rituals and identity construction are discussed. For infants and children, socialization and protection are important functions, while for adults, identities relating to fertility and married status are key. It is suggested that older women wore less jewellery than younger ones, and that gifting of jewellery within families was important in relation to life course stages and rituals. The various functions of dress objects in cementing and facilitating wider family and community relationships are also discussed.

Genetic pathogenesis, diagnosis, and treatment of short-chain 3-hydroxyacyl-coenzyme A dehydrogenase hyperinsulinism

Congenital hyperinsulinism (CHI), a major cause of persistent and recurrent hypoglycemia in infancy and childhood. Numerous pathogenic genes have been associated with 14 known genetic subtypes of CHI. Adenosine triphosphate-sensitive potassium channel hyperinsulinism (KATP-HI) is the most common and most severe subtype, accounting for 40–50% of CHI cases. Short-chain 3-hydroxyacyl-coenzyme A dehydrogenase hyperinsulinism (SCHAD-HI) is a rare subtype that accounts for less than 1% of all CHI cases that are caused by homozygous mutations in the hydroxyacyl-coenzyme A dehydrogenase (HADH) gene. This review provided a systematic description of the genetic pathogenesis and current progress in the diagnosis and treatment of SCHAD-HI to improve our understanding of this disease.

A study of clinical analysis and management of lnguinoscrotal swelling in paediatric age group

Background: Inguinoscrotal swellings are one of the commonest congenital problems in infancy and childhood. Delay in diagnosis and treatment leads to loss of testis, ovaries or portion of bowel to incarceration or strangulation. This study was undertaken for clinical evaluation of age, sex, sidewise distribution management and management of complications.Methods: Retrospective observational study done from April 2011 to March 2019. Children with age from 1 month to 12 years presenting with inguinoscrotal swelling Results: In Total 174 children had inguinoscrotal swelling which include 155 (89%) male children and 19 (11%) female children. Most of the patients presented in 2 to 5 years (41%) age group out of 114 case of inguinal hernia include 63 on right,47 on left and 4 bilateral and 60 case of hydrocele 48 on right,10 on left and 2 bilateral.0ut of 174 children 16 were discharge on same day of surgery. Out of 174 children 1 patient had taken as emergency operation due to irreducible hernia. Conclusions: Inguinal hernia and hydrocele in children remain one of the most common congenital problems observed by surgeons. The childhood inguinoscrotal swellings are generally more predominant on the right side and this has been attributed to the delay in descent of the right testis. Regarding the sex prevalence, males are more commonly affected. Inguinal herniotomy in children is a safe and effective operation done as day care procedure.

Plexiform Cellular Schwannoma in Infancy and Childhood: A Clinicopathological Study of Seven Cases of an Underrecognized Nerve Sheath Tumor with a Tendency Toward Local Recurrence

Plexiform cellular schwannoma (PCS) is very rare, and it is not completely understood. We present our experience with 7 additional cases of PCS in infancy and childhood to further characterize its distinctive clinicopathological features. There were 5 females and 2 males with a mean age of 28 months (ranging, 2 months to 8 years). The involved sites included the left forearm ( n = 2), sacrococcygeal region ( n = 2), retroperitoneum ( n = 1), thoracic spinal canal and thoracic cavity ( n = 1), and neck ( n = 1). Tumor sizes ranged from 3 to 13 cm in maximum diameter (mean, 7.1 cm). Histologically, all tumors consisted of abundant spindle cells arranged in a multinodular or plexiform growth pattern, possessing elongated, hyperchromatic nuclei and pale eosinophilic cytoplasm with indistinct cell margins. Mitotic figures were easily identified, with a mean count of 4 per 10 consecutive high power fields (HPF). Immunohistochemically, all tumors were strongly and diffusely positive for S100 protein, SOX10 and H3K27me3. The Ki-67 index ranged from 5% to 30% (mean, 15%). Follow-up (available in 6 cases) revealed that 5 patients experienced local recurrence and were treated by re-excision. There was no evidence of recurrence and metastasis in 3 patients, and the other 2 were alive with the disease. In conclusion, PCS is an uncommon nerve sheath tumor predominantly occurring in infants and children, featuring a plexiform or multinodular growth pattern and exhibiting a tendency toward local recurrence. PCS is easily mistaken as malignant peripheral nerve sheath tumor (MPNST) due to its locally aggressive behaviors and worrisome features, including hypercellularity, hyperchromatism and high proliferative activity. Increased awareness of its potential occurrence and greater familiarity with its characteristic features are helpful for both clinicians and pathologists to avoid misdiagnosis and unnecessary overtreatment.

Kindler syndrome: A rare case report

Kindler Syndrome (KS) is a rare hereditary disorder characterized by acral blistering of infancy and childhood, photosensitivity, progressive poikiloderma, and cutaneous atrophy. We report this case of KS in a 4 year old female child on account of its rarity Keywords: Kindler syndrome; acral blistering; pokiloderma; photosensitivity