Acrodermatitis Enteropathica

and Emanual Maverakis3



(1)
University of British Columbia, Vancouver, British Columbia, Canada

(2)
New York Medical College, Valhalla, NY, USA

(3)
University of California Davis, Sacramento, CA, USA

 




11.1 Synopsis of Study


AE (Acrodermatitis Enteropathica) is an autosomal recessive disorder affecting the uptake of zinc which causes reduction of all body zinc levels, including blood serum. Similar symptoms of severe zinc deficiency have often been observed in patients who underwent a prolonged intravenous nutrition. Common symptoms of AE include the appearance of perleche, skin lesions, erythematous patches, plaques of dry and scaly skin, and eczematous plaques on the face, scalp, and genital area. It is interesting to note that AE was frequently observed in Europe, particularly Northern Europe; and cases of AE have also been reported every year in Iran since 1970. AE is considered a minor and treatable disorder as long as it is diagnosed and treated promptly. It can be treated by supplementing the patient’s diet with necessary amounts of zinc which would raise the level of zinc in blood plasma to normal levels.

Being a recessive autosomal disorder, which presents clinical symptoms if an individual of any gender is homozygous for the disease-related-gene, AE has become more common in Iran since it was first reported in 1970. It is interesting to note that Iran contains high numbers of consanguineous marriage especially in rural areas and tribes. Furthermore, malnutrition and dietary zinc deficiency contribute to the manifestation and severity of the clinical symptoms of this disorder. Many rural populaces of Iran are reported to have malnutrition, particularly neonatal and school-aged childhood. As a result, number of reported AE cases has been increasing in Iran. Therefore, conducting more research studies on this disorder and its complications is of great significance, especially in a country like Iran. The opportunity of conducting various studies in this field seems endless. AE has rarely been studied which may be due to the very few number of diagnosed and reported cases. The disorder is occasionally reported in patients who have been hospitalized for a long time and undergo intravenous nutrition.

With regards to the complications of AE, such as cutaneous, trichological, as well as neurological disorders, which are presented by reference texts and articles, further research studies are required to endorse or reject the symptoms and conditions as well as provide further information about AE. The present study has carried out by using the files of AE patients that were referred to Razi Hospital during the years 1999 to 2004.


11.2 Purpose of Study


The purpose of the study was to determine blood zinc levels and the symptoms of patients with Acrodermatitis Enteropathica, who were referred to Razi Hospital during the years 1999 to 2004. More specifically, the study aimed to determine the range of blood zinc levels of the mentioned patients as some AE cases have been reported to have normal blood zinc levels. Furthermore, the relationship between blood zinc level and the disorder’s complications was investigated as well.

Alternate purposes:



  • Indicate the relationship between blood zinc level and gastrointestinal symptoms in AE patients1


  • Indicate the relationship between blood zinc level and alopecia in AE patients2


  • Indicate the relationship between blood zinc level and cutaneous symptoms in AE patients3


  • Indicate the relationship between blood zinc level and psychological symptoms in AE patients4


11.3 The Considered Hypotheses




1.

Blood zinc level is less than normal in majority of AE patients.

 

2.

There are some patients diagnosed with AE, whose blood zinc levels are normal.

 

3.

Hair zinc level is less than normal in majority of AE patients.

 

4.

Most AE patients suffer from gastrointestinal disorders (diarrhea, vomiting, and cramps).

 

5.

The lower the blood zinc level is, the more serious the AE symptoms are.

 

6.

All AE patients demonstrate cutaneous manifestations.

 

7.

Most AE patients show psychological disorders such as moodiness, lethargy, anorexia, and photophobia.

 

8.

The first symptom of the disorder is mucocutaneous lesions in most AE patients.

 

9.

The first sign of recovery in most AE patients is the disappearance of cutaneous manifestations.

 

10.

Most cases that are fed breast milk show the symptoms of the disorder as soon as they discontinue it.

 

11.

Majority of the AE patients suffer from iron deficiency anemia and their blood ferritin and alkaline phosphatase levels are below normal.

 

12.

There may be a correlation between zinc levels and iron levels if patients are iron deficiency anemia and blood ferritin and alkaline phosphatase levels return to normal by the prescription of zinc supplementation.

 


11.4 Introduction


Being a recessive autosomal disorder, which presents clinical symptoms if an individual of any gender is homozygous for the disease-related-gene, Acrodermatitis Enteropathica has become more common in Iran since it was first reported in 1970. It is interesting to note that Iran contains high numbers of consanguineous marriage especially in rural areas and tribes. Furthermore, malnutrition and dietary zinc deficiency contribute to the manifestation and severity of the clinical symptoms of this disorder. Many rural populaces of Iran are reported to have malnutrition, particularly neonatal and school-aged childhood. As a result, number of reported AE cases has been increasing in Iran. Therefore, conducting more research studies on this disorder and its complications is of great significance, especially in a country like Iran.

The main purpose of the study was to determine blood zinc levels and the symptoms of 27 infant patients with Acrodermatitis Enteropathica, who were referred to Razi Hospital in Tehran, Iran during the years 1999 to 2004. More specifically, the study aimed to determine the range of blood zinc levels of the mentioned patients as some AE cases have been reported to possess normal serum zinc levels.

Furthermore, the secondary purpose of this study was to determine the relationship between blood zinc levels and the appearance and severity of symptoms, such as gastrointestinal symptoms, alopecia, cutaneous and psychological symptoms in AE patients.


11.5 Methods


The article is presented as a retrospective cross sectional study. Patients who had referred to the Razi Hospital Clinic or were sent to Razi Hospital by other medical clinics during 1999 to 2004 because of Acrodermatitis Enteropathica were considered and examined in this study: 27 infant patient cases were examined, of which 11 were male and 16 were female. The location of study was at Razi Hospital’s Dermatology Section located on Vahdat Eslami St. Tehran, Iran.

The study was also carried out by referring to the medial records of the considered patients that were available in the hospital archives. In cases where there was inadequate patient information available in the hospital archives or more information was needed, the patient was contacted and besides to ordering appropriate tests, an interview was set with the parents of the patient in order to complete the medical documents. Patients who were either not available and had incomplete hospital records or did not give their consent to be included in the clinical research were excluded from the study.

In order to obtain the zinc blood levels of the patients the following procedure was carried out. The blood sample was collected early in the morning since the normal circadian variation in plasma zinc levels is at its lowest and most precise during early mornings. The patients’ parents were also told that their infant should be fasting before the blood test so that the test results would be accurate.

Furthermore, the patient medical records were used in order to complete the survey tool and patients were asked to come in for an interview if any further information was required or if there were questions that could not be answered based on the data from the medical documents. Geographically speaking, the patients were from many different parts of the country, such as Meshkin City, Kermanshah, Ghazvin, Marand, Ghaem City, Zanjan, Tehran, Boushehr, Shiraz, Tonekabon, and Fouman. Table 11.1 illustrates the types of variables that were measured in this study including a brief description about each one.


Table 11.1
Types of the measured variables



















































































Row

Name of variable

Type of variable

Nominal or comparative/continuous or discrete

Dependent or independent

Description

scale

1

Age

Quantitative

(discrete)

Discrete

Independent

Patient’s age is stated based on the month and the year of birth

Year

2

Weight

Quantitative

(continuous)

Continuous

Independent

Patient’s weight is stated based on kg

Kilogram

3

Sex

Qualitative

(nominal)

Nominal

Independent

Patient’s sex

Male/female

4

Place of birth

Qualitative

(nominal)

Nominal

Independent

Place of birth is stated based on the name of the city and the state

City name

5

Father’s age

Quantitative

(discrete)

Discrete

Independent

Father’s age is stated based on the year of birth

Year

6

Mother’s age

Quantitative

(discrete)

Discrete

Independent

Mother’s age is stated based on the year of birth

Year

7

Parent’s relationship

Qualitative

(nominal)

Nominal

Independent

Parent’s relativity is first degree, second degree or without relativity

First degree/second degree/no relativity

8

Cutaneous lesions

Qualitative

(nominal)

Nominal

Dependent

The lesions are eczematous and erythematous, with scales around areas like the nose, eyes, genital and rectal areas

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May 9, 2017 | Posted by in Dermatology | Comments Off on Acrodermatitis Enteropathica
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