Heme synthesis - note that some reactions occur in the cytoplasm and some in the mitochondrion (yellow)
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Hereditary coproporphyria

The porphyrias are inherited or acquired disorders of certain enzymes in the heme biosynthetic pathway (also called porphyrin pathway). They are broadly classified as hepatic porphyrias or erythropoietic porphyrias, based on the site of the overproduction and mainly accumulation of the porphyrins (or their chemical precursors). more...

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Overview

In humans, porphyrins are the main precursors of heme, an essential constituent of hemoglobin, myoglobin, and cytochrome.

Deficiency in the enzymes of the porphyrin pathway leads to insufficient production of heme. This is, however, not the main problem; most enzymes—even when less functional—have enough residual activity to assist in heme biosynthesis. The largest problem in these deficiencies is the accumulation of porphyrins, the heme precursors, which are toxic to tissue in high concentrations. The chemical properties of these intermediates determine in which tissue they accumulate, whether they are photosensitive, and how the compound is excreted (in the urine or feces).

Subtypes

There are eight enzymes in the heme biosynthetic pathway: the first and the last three are in the mitochondria, while the other four are in the cytosol.

  1. δ-aminolevulinate (ALA) synthase
  2. δ-aminolevulinate (ALA) dehydratase
  3. hydroxymethylbilane (HMB) synthase
  4. uroporphyrinogen (URO) synthase
  5. uroporphyrinogen (URO) decarboxylase
  6. coproporphyrinogen (COPRO) oxidase
  7. protoporphyrinogen (PROTO) oxidase
  8. ferrochelastase

Hepatic porphyrias

The hepatic porphyrias include:

  • ALA dehydratase deficiency
  • acute intermittent porphyria (AIP): a deficiency in HMB synthase
  • hereditary coproporphyria (HCP): a deficiency in COPRO oxidase
  • variegate porphyria (VP): a deficiency in PROTO oxidase
  • porphyria cutanea tarda (PCT): a deficiency in URO decarboxylase

Erythropoietic porphyria

The erythropoietic porphyrias include:

  • X-linked sideroblastic anemia (XLSA): a deficiency in ALA synthase
  • congenital erythropoietic porphyria (CEP): a deficiency in URO synthase
  • erythropoietic protoporphyria (EPP): a deficiency in ferrochelatase

Porphyria variegata

Variegate porphyria (also porphyria variegata or mixed porphyria) results from a partial deficiency in PROTO oxidase, manifesting itself with skin lesions similar to those of porphyria cutanea tarda combined with acute neurologic attacks. It may first occur in the second decade of life; there is a cohort of sufferers living in South Africa descended from a single person from the Netherlands, Berrit Janisz, who emigrated in the 17th century.

Signs and symptoms

The hepatic porphyrias primarily affect the nervous system, resulting in abdominal pain, vomiting, acute neuropathy, seizures, and mental disturbances, including hallucinations, depression, anxiety, and paranoia. Cardiac arrhythmias and tachycardia (fast heart rate) may develop as the autonomic nervous system is affected. Pain can be severe and can, in some cases, be both acute and chronic in nature. Constipation is frequently present, as the nervous system of the gut is affected.

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West faces new allegations; One says mayor asked him out at age 15;
From Spokesman Review, The (Spokane), 5/7/05 by Bill Morlin

(c) The Spokesman-Review

The controversy swirling around Spokane Mayor Jim West grew substantially Friday when two men, now living in Seattle, came forward with new allegations about his conduct in 1980 and as recently as 2001.

West faces allegations of sexual abuse of children dating to the 1970s and more recent allegations of abuse of public power.

Brad Crelia, of Seattle, said he was a 15-year-old Lewis and Clark High School student in 2001 when West, then Senate minority leader, asked him out.

The other man said he was sexually abused in 1980 by David Hahn when Hahn and West - his good friend and fellow Spokane County sheriff's deputy - took a group of Boy Scouts on a 50-mile hike around Mount Rainier. The man said he was 12 at the time.

"I reported the abuse the next day to Jim West and asked to be moved to another tent, and he did nothing about it. He told me to 'forget it,' " said the man, a professional who said he has no criminal record.

To "avoid impacts on my family," he asked that only his first name, Scott, be used in this story. He did, however, provide The Spokesman-Review his full name and work and home telephone numbers for verification. He also provided the newspaper a picture of himself as a boy, as well as the names of his parents, who are now retired and living in Spokane. The man's parents fully corroborated details of his allegations in an interview Friday at their North Side home but asked to remain anonymous.

The man's story is in sharp contrast to West's claims that he had no knowledge Hahn might be sexually abusing boys at his apartment or on Scout overnight camping trips during the late 1970s and early 1980s.

"I didn't know Dave Hahn was abusing kids until many, many years later," West told The Spokesman-Review on Wednesday.

West did not return a message seeking comment Friday night. Earlier in the day, he resigned from the board of directors for Morning Star Boys' Ranch, a position he'd held for 12 years.

As media interest in the West story broadened, the man named Scott came forward for the first time with allegations that he was abused in 1980 by Hahn.

Scott said the abuse occurred while he and other Boy Scouts from Spokane were on a 50-mile hike around Mount Rainier in the Cascades. The group drove to the base of the mountain in Western Washington and spent a week hiking around the mountain, spending nights sleeping in two-man tents.

"I believe it might have been the first or second night," Scott said. "I was sexually molested by David Hahn.

"I didn't say anything" during the molestation. "I was quiet and pretended I was asleep."

Soon thereafter, Scott said, "I told Jim West what had happened and asked if I could move to another tent. I think I was pretty clear about what I told him, and I remember him just dismissing me.

"I knew from talking to him I should just keep quiet," Scott said.

Later, in front of other boys, Scott said West embarrassed him by referring to him as "fag boy."

"It took me so long to even mention it to my parents," he said.

Scott's father is a 73-year-old professional and his mother is 67 and a retired city of Spokane employee.

The couple, who have three other older sons, said they didn't learn Scott was molested by Hahn until years later, but saw a marked change in his personality and behavior immediately after he returned from the hike.

"He was hard to talk to, for one thing," recalled Scott's mother.

She described Scott as "more rebellious and harder to understand" after he returned from what the parents thought would be a positive experience. Instead, he wanted nothing to do with the Boy Scouts and quit Troop 345, which met at Hamblen Elementary School, where Hahn and West were appointed scoutmasters.

When Hahn was told to resign in August 1981 or face a possible criminal investigation for alleged pedophilia, he went to his South Hill apartment and committed suicide, allegedly after abusing one of his young victims one last time. Hahn was 36.

Scott's father said that after Hahn's suicide, he asked his son if something had occurred during the 50-mile hike. "Scott told me Hahn had molested one of the kids and the kid was crying." But Scott didn't say that he was the victim.

Scott said he sought counseling when the childhood abuse caused him emotional and mental problems in the early 1990s while attending Western Washington University. He didn't fully discuss what happened with his parents until August 2003 when he and his wife traveled to see them for a week's vacation on the Pend Oreille River.

"Scott and I were going to get groceries in August 2003 and he told me that he'd been molested by Hahn and had been getting therapy for some time," the mother said. "I was so shocked, to tell you the truth."

She said she clipped a June 8, 2003, Spokesman-Review story identifying three men who said they were abused as boys by Hahn. Scott was not among those identified.

Robert J. Galliher, one of Hahn's alleged victims who is now suing Spokane County, told The Spokesman-Review that he was molested and had a gun pointed at his head moments before Hahn killed himself.

Senior sheriff's officials "wouldn't tell me why he killed himself," West said Wednesday when asked what he knew about Hahn.

Instead of his photo, West recently posted a picture of Mount Rainier on one of two personality profiles he used at the Web site Gay.com, which he has acknowledged using to find young gay men to date.

West used City Hall computer equipment to offer an internship in the mayor's office to one of those young men, a fictional 18-year- old Ferris High School student named "Moto-Brock" who was created last year by a computer consultant hired by The Spokesman-Review.

West admitted the gay chat room conduct, but said allegations from Galliher and Michael G. Grant Jr. that he sexually abused them as boys are "flat lies."

Scott's father said he had "an uneasy feeling about Hahn and West" in 1981, a year after the camping trip and before Hahn killed himself.

"Just the close association of the two always made me wonder," the father said.

When Scott's parents talked with him about this week's revelations about West, they said their son told them he had reported the abuse to West during the campout. Scott offered the same version of events during a telephone interview Friday.

"When Scott told me this, I was furious because I was the one who sent him on this hike," his father said.

It was Hahn who approached the family, the father said, and encouraged them to send their son, who was reluctant to go on the Mount Rainier hike.

"Hahn said, 'We want him to go. He can sleep in my tent.' He told us both that he'd take good care of him," said the father.

Their older son had been on a Little League football team called the Cowboys, coached by West and Hahn, according to the father.

"David Hahn was a very gregarious, persuasive person," the father said. "Jim West was a little more serene and reserved."

"They were both nice-looking young men," he continued. "They were both sheriff's deputies, both war heroes or ex-military. Those were different times. I had no reason to distrust either man."

"This is beyond a 'gay' issue," he said. "These people like Hahn are predators. They are looking for victims at ages when they are ill-prepared to take care of themselves."

Crelia, an openly gay 19-year-old now living on Seattle's Capitol Hill, contacted the newspaper after the stories on West were published Thursday. He was an LC freshman in the 2000-2001 school year, when the high school was temporarily relocated to the Holley- Mason Building downtown.

West, then a state senator, had an office on the sixth floor of the building adjacent to the Local Planet, a weekly newspaper run by Crelia's parents, Connye Miller and Matt Spaur.

That year, Crelia recalled, he'd just told his mother for the first time that he was gay. He also started a club, Spectrum, a gay- straight alliance at LC. He was honored as a Chase Youth Award nominee in 2001 for his efforts.

Crelia said he'd run into West a couple of times while visiting his parents' office.

"The first time was a 'Hi, how are you' kind of thing," Crelia said.

The second time, toward the end of his freshman year, West emerged from his office and asked the teenager out, Crelia recalled.

"He asked if I'd like to go out and get coffee ... just go out and talk ... and I said no thank you," Crelia recalled.

"He then asked, would you want to do anything else, and that's what kind of weirded me out. It was definitely an awkward feeling, because I knew who he was. I don't know what his intentions were, but I definitely felt they were sexual," Crelia said.

"I'd heard things from other people that he'd done that before," Crelia said.

Matt Spaur, Crelia's father and the former Local Planet publisher, said the teenager told his mother, Connye Miller, shortly afterward that he'd been approached by an older man.

Miller died in June 2003 of complications of hereditary coproporphyria, a painful disease that Crelia also has. He said he takes a battery of medicines, including pills for nausea and depression.

It wasn't until the 2003 mayoral campaign that his son confided in him that the man who asked him out was West, Spaur recalled.

"The story around town was that Brad wasn't the only one," Spaur said. He said he considered having the Local Planet investigate West, but lacked the resources.

The incident with his son "indicates that West was willing to look at much younger men and approach them," Spaur said. "He might not have known Brad's age. But I'm 42, and I'd feel very awkward approaching someone that young," Spaur added.

Crelia said he used to visit Gay.com in high school, lying about his age because the Web site requires participants in online chats to be 18. He met men as old as 30 on that site and had sex with some of them, Crelia said.

In the chat room on that Web site, he heard online rumors about West liking men, Crelia said. But he never met West online.

It's a "creepy feeling" to think that Spokane's one-time senator and current mayor is online trying to pick up younger men, Crelia said.

Karen Dorn Steele can be reached at (509) 459-5462 or by e-mail at karend@spokesman.com. Bill Morlin can be reached at (509) 459- 5444 or by e-mail at billm@spokesman.com.

(c) The Spokesman-Review

Copyright c 2005 The Spokesman-Review
Provided by ProQuest Information and Learning Company. All rights Reserved.

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